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A 75-year-old man was transferred to our hospital by ambulance early in the morning because of a severely impaired consciousness level with a Glasgow Coma Scale of 7. For nearly 5 years at a clinic close to his home, he had uneventfully received twice weekly HD due to diabetic nephropathy. He had been free of serious hypoglycemic episodes for several years. Approximately 40 days before the emergency transportation, he began receiving teneligliptin at a dose of 20 mg/d because of worsening glycemic control with a glycoalbumin (GA) level of 27.1%. Despite having eaten a comfortable meal on the previous night, his blood test results on his arrival at the hospital revealed a severe hypoglycemic state: 23 and 45 mg/dL in the venous and capillary blood, respectively. Teneligliptin, a DPP-4 inhibitor, was the only ongoing antidiabetic medication, which he had last taken the previous evening. His other medications and their daily dose were as follows: 75 mg of clopidogrel, 60 mg of nifedipine, 2 mg of doxazosin, 240 mg of furosemide, 20 mg of olmesartan, 5 mg of zolpidem, 3 g of calcium carbonate, and 300 mg of ursodeoxycholic acid. At the time of dialysis, he also received intravenously 6000 units of epoetin alfa, 5 µg of maxacalcitol, and 1 g of levocarnitine chloride. The patient informed us that he had taken these medications the previous evening and taken them exactly as prescribed. Thus, an ordinary cause of severe hypoglycemia was not determined, includng the prescription. He became fully conscious shortly after an intravenous injection of glucose. However, the decision to hospitalize him was determined primarily because of significant fluid retention, which was coincidentally revealed by chest computed tomography imaging (). At the time of admission, the GA level was reduced to 22.6%.\nThe problem of anasarca was settled by near-daily HD with little difficulty. His dry weight reduced from 55.0 to 51.0 kg. Thereafter, HD frequency was increased to 3 times weekly. In marked contrast, severe hypoglycemia reemerged immediately following recovery from hypoglycemia and required frequent glucose injections (), despite his consuming full meals. Teneligliptin was immediately discontinued, although its causal association with his hypoglycemia was not determined at the time of hospitalization. Doxazosin and nifedipine were subsequently stopped on the following day and 5 days later, respectively, to avoid a blood pressure drop due to the removal of excessive fluid by frequent HD. Zolpidem was disused throughout the hospitalization so that the patient’s conscious level could be precisely evaluated and because the patient did not call for a hypnotic agent. Incidentally, calcitriol was used as a substitute for maxacalcitol during the hospitalization because of the availability at our facility. After the following afternoon, his overt hypoglycemia eventually disappeared. After 9 days of admission, he was discharged with preprandial blood glucose levels of approximately 120 mg/dL, and no significant hyper- or hypoglycemia. |
Our patient was a 37-year-old woman, who worked as an English teacher at a local high school. She fell down the stairs of the height of three meters and sustained a head injury. She exhibited a partial loss of consciousness scored 12 at (E3V4 M5) on Glasgow Coma Scale after the injury and was therefore transported to the emergency hospital. CT revealed a left acute subdural hematoma, which was treated using the craniotomy hematoma exclusion method. The patient's consciousness status improved after surgery, and she was transferred to our hospital for rehabilitation. On admission, the patient presented with a right homonymous hemianopsia, mild memory and attention disorders, and left hemiparesis. Although conventional MRI revealed a lesion in the left occipital lobe, no apparent cause of the left hemiparesis could be identified. The patient subsequently participated in a rehabilitation program which sufficiently improved her cognitive disorders to allow for a return to work 1 year later. Unfortunately, the left hemiparesis remained. The patient's left hemiparesis was scored as follows, according to the Brunnstrom staging: upper extremity IV, finger V, and lower extremity IV. Thus, she required a short leg brace and a cane for walking. Despite the stable cognitive status which allowed for a return to work, the patient never regained control of her left upper extremity and fingers and never regained a normal degree of locomotion. Two years after the injury, we performed additional MRI which revealed the same occipital lesion noted earlier, but no evidence could be determined regarding right cerebral hemisphere or brainstem abnormalities, which were speculated to be responsible for the left hemiparesis (). Therefore, DTI and FT were used to identify the cause of the patient's left hemiparesis.\nMRI was performed using a 1.5 Tesla imager (Signa Horizon LX CV/I, GE Medical Systems, Milwaukee, WI) and a conventional head coil to obtain DTI data. The DTI scan conditions were similar to those described previously [, ]. The DTI data obtained were transferred to an offline Windows PC with an Intel (R) Core (TM) i7 CPU processor (1.73 GHz) and 2046 MB memory. The data were then analysed using the diffusion TENSOR Visualizer (dTV) and VOLUME-ONE software (free software provided by the University of Tokyo Hospital, Tokyo, Japan; ).\nAt first, FA map images were obtained using dTV to measure the FA values. The FA values were measured using the region of interest (ROI) technique. Several sphere ROIs with a radius of 2.0 voxels were delineated on an FA map of the corticospinal tract (CST), which included the cerebral peduncle, the posterior limb of the internal capsule, and the corona radiata. After measuring the mean FA value of each ROI, we compared these to the corresponding values from five age-matched controls (average age, 35.2 years; range, 30–39 years). The Statistical Package for the Social Sciences (SPSS v. 11.0; SPSS Inc., Chicago, IL, USA) was used for statistical analysis, and a P value < 0.05 was considered statistically significant.\nWe conducted the CST evaluation by fibre tracking. The seed area was defined as a region of interest drawn around the cerebral peduncle, and the target area was defined as a region of interest drawn around the precentral gyrus. The stop criteria were set at FA < 0.18. The FT anisotropy information was colour-coded: red indicates high anisotropy, whereas orange indicates low anisotropy.\npresents the results of the comparison outlined above. The mean FA values of the patient were significantly decreased as compared with those of healthy controls only on the right side of each ROI (P < 0.001); however, there were no significant differences on the left side. Further, bilateral comparison of the mean FA values from each ROI revealed the right-side values as significantly decreased in the cerebral peduncle (P = 0.001), the posterior limb of the internal capsule (P = 0.015), and the corona radiata (P < 0.001).\nOn FT evaluation, the right CST of the patient with DAI was depicted less clearly (tracked line, 118; drawn line, 6) compared with that of the right CST of healthy controls (tracked line, 242; drawn line, 34; ). Furthermore, the depiction of the patient's right CST (tracked line, 118; drawn line, 6) was poor in comparison with that of the left CST (tracked line, 252; drawn line, 34; ). |
A 45 year old female with the diagnosis of 10 years' progression of definite () bilateral type I Menière's Disease, according to the Lopez-Escamez classification (), with recurrent vertigo and hearing loss attacks symptomatically controlled with sulpiride (50 mg) on demand, was referred to the cochlear implants unit of a tertiary hospital center as a possible candidate for cochlear implantation, because of profound hearing loss (1997 BIAP) in the left ear and fluctuating moderate-severe (1997 BIAP) hearing loss in the right ear. The most recent vertigo episode occurred 10 days prior to her visit to the cochlear implant unit. No visual impairment or other alterations were found on otoneurotologic examination; normal ocular movements, normal smooth pursuit, and saccadic movement, absence of ocular misalignment on skew deviation test and no spontaneous nystagmus were also found, despite the recency of the last vertigo attack. Other neurological examinations and standard cerebral MRI were also normal for this patient.\nIn addition to the same tests as for case I, because of the bilateral MD diagnosis, the patient had received 3 months prior a 3 Tesla MRI examination of the inner ear using the HYDROPS MRI sequence (): this sequence is based on the digital subtraction of images produced by the different time diffusion of gadolinium along the inner ear fluids. This MRI showed bilateral cochlear and vestibular endolymphatic hydrops with left side hydrops predominating, as can be observed in Figure .\nThe instrumental vestibular testing was performed by the same senior neurotologist using a vHIT ICS Impulse™ USB hardware version with software version 4.1: this device was a different unit from the device used in case I. Figure shows enhanced vHIT eye velocity responses for both sides, with an aVOR gain value of 1.14 on right horizontal canal function test and 1.05 for left side. In this case, the vHIT calibration was repeated four times with similar enhanced gain values obtained, and a fifth calibration was done with the default system calibration parameters, also yielding similar aVOR gain values. The VVOR test was also performed on this patient using the vHIT ICS Impulse™ device, finding an enhanced eye velocity response during the VVOR test with a measured VVOR gain () value of 1.39 for left side and 1.35 for right side.\nFor both cases presented in this paper, the patients gave written consent to publish the results obtained from their clinical examinations and instrumental tests. |
A 14-year-old previously healthy girl presented 2 weeks after an uncomplicated laparoscopic appendectomy for non-perforated acute appendicitis in a regional hospital. The girl complained of gradually reducing urinary frequency to twice per day and prolonged hesitancy. The micturition stream was initially weak and slow before becoming interrupted. Straining did not produce stronger urinary stream. She had never suffered from urinary tract infections (UTIs) or constipation and opened her bowels daily.\nFollowing an episode of acute cystitis 2 months later, she completely lost her ability to void. She was put on indwelling Foley urinary catheter, and her cystitis was successfully treated with antibiotics. After every attempt to remove the urinary catheter, she had to be catheterized again with 300 to 1200 mL of urine volume registered. She noted loss of urge to urinate and felt only dull pain in suprapubic region and right iliac fossa on extreme bladder distention. The girl was kept on indwelling urethral urinary catheter and referred to a tertiary center to determine the etiology of her urinary retention.\nShe was examined with normal clinical findings and no obvious pathology on abdominal and pelvic ultrasound scan (USS). A pediatric neurologist found nothing abnormal, and magnetic resonance imaging (MRI) of the brain and spine, electromyography (EMG) of the lower extremity, somatosensory-evoked potentials (SEP) of tibial nerve, electroencephalogram (EEG), and lumbar puncture were with no pathology. On USS, the gynecologist described multiple follicular cysts on ovaries bilaterally and found no pathology explaining her urinary retention.\nOur pediatric urologist performed an examination under general anesthesia including a free calibration of the urethra up to 26F followed by normal findings on cystoscopy. Videourodynamic study (VUDS) showed an asensitive and hypotonic bladder. The bladder filling had to be stopped at 360 mL due to the patient's discomfort. Maximum intravesical pressure achieved 11 cmH\n2\nO. When pulling the urodynamic catheter out of the bladder manually, the maximum urethral pressure measured was 120 cmH\n2\nO. On vesicocystourethrogram (VCUG), there was no vesicoureteral reflux, a smooth bladder wall and closed bladder neck (\n).\nPsychologic and psychiatric evaluation identified no major problem. During the following 2 years of repeated admissions to several regional and university hospitals, many of the tests described above were repeated, including an MRI of brain and spine with identical conclusions.\nClean intermittent catheterization (CIC) was recommended to the patient. However, because of poor tolerance of CIC due to frequent macroscopic hematuria and pain, a suprapubic catheter was placed. Thereafter, she suffered recurrent symptomatic afebrile UTIs caused by multi-resistant bacterial strains, e.g.,\nKlebsiella\n,\nPseudomonas\n, or\nEscherichia\n. Finally, after 2 years, based on the history, symptoms, and urodynamic findings, she was diagnosed with Fowler's syndrome (FS).\nFor the treatment of FS, the patient was indicated for S3 neurostimulation. The implantation of two Medtronic S3 neurostimulators, type Interstim II, bilaterally in the upper gluteal region was performed under general anesthesia in two phases. The first phase was a transcutaneous implantation of the electrodes into S3 foramina and their connection to externalized neurostimulators. The first procedure took 30 minutes. As the patient restored her voiding completely back to normal when switching on the neurostimulators and experienced no side effects, she could undergo the second phase 4 weeks later—permanent subcutaneous implantation of the neurostimulators (\n). The second procedure took 15 minutes under general anesthesia.\nWith a transcutaneous remote control, she was able to modify the intensity of stimulating current to avoid any discomfort (\n). On the last follow-up, 4 months after the implantation, she voided four to six times per day with post-void residuals up to 50 mL on USS. Unfortunately, she suffered two prolonged episodes of burning on micturition even after the operation. On both occasions, she was diagnosed with acute cystitis by\nE. coli\n107 that was treated with antibiotics after sensitivity testing. |
A 52 years old Caucasian male with a history of hypertension presented to the emergency department with severe pressure-like left-sided chest pain of 1-hour duration which radiated to the ipsilateral arm. He was administered 0.4 mg of sublingual nitroglycerin which improved the pain significantly. Vital signs showed a blood pressure of 100/72 mmHg, heart rate of 71/min, respiratory rate of 19/min with a saturation of 96% on room air, and a temperature of 97.8 F. His cardiopulmonary examination was within normal limits. An electrocardiogram was obtained which reflected a new ST segment elevation in leads V2-V5 (). The cardiac catheterization laboratory was activated, and the patient was given loading doses of aspirin 325 mg, ticagrelor 180 mg, and atorvastatin 80 mg. Meanwhile, laboratory investigation was significant for troponin-I of more than 80.00 ng/mL. While in the emergency room, the patient started complaining of palpitations and he lost consciousness. Upon examination, there was no palpable pulse and the cardiac monitor showed ventricular fibrillation. A code blue was called and cardiopulmonary resuscitation was initiated. Immediate successful intubation was performed. In total, the patient received 9 rounds of 1 mg intravenous (IV) epinephrine every 3-5 minutes, 300 mg IV amiodarone bolus, 1 gram of 10% calcium chloride, 2 doses of 50 mEq sodium bicarbonate, and 5 biphasic defibrillator shocks of 200 joules (J) each. The cardiac monitor still showed VF.\nConsidering it as a resuscitation failure, double sequential defibrillation was performed and he received 4 additional shocks of 400 J each. Despite this, the patient was still in an electrical storm and at this moment intravenous esmolol 500 mcg/kg (35 mg) IV bolus was administered. The patient subsequently experienced an immediate return of spontaneous circulation. The total resuscitation time was 50 minutes. The therapeutic hypothermia protocol was initiated, and the patient was urgently transferred to the cardiac catheterization laboratory. A loading dose of tirofiban IV 25 mcg/kg and bivalirudin IV 0.75 mg/kg was administered, and a tirofiban maintenance infusion was continued for 12 hours. Emergent catheterization revealed a 100% occlusion of the left anterior descending artery (LAD) (Video ) with subsequent placement of two drug-eluting stents (DES) in the LAD (Video ).\nThe patient was then promptly transferred to the coronary care unit. Therapeutic hypothermia was stopped after 24 hours and meanwhile echocardiography revealed an ejection fraction of 30% with anterior and apical wall akinesis confirming a diagnosis of ischemic cardiomyopathy. The patient was successfully extubated after 4 days, and his neurological function was at his baseline. His hospital stay was complicated by an isolated episode of atrial fibrillation with rapid ventricular response and aspiration pneumonia. The former responded to beta-blockade for rate control. Aspiration pneumonia was treated with 7 days of intravenous ceftriaxone and metronidazole as per the sensitivity results of sputum culture which grew Haemophilus influenzae. The patient was discharged from the hospital on day 12 on aspirin 81 mg once daily, ticagrelor 90 mg two times a day, metoprolol succinate extended-release tablet 150 mg once a day, lisinopril 5 mg once a day, and a life vest. He was followed up within a week at our cardiac rehabilitation clinic where he was found to be asymptomatic and compliant with medications. |
Case 1 is a 57-year-old woman (weight = 77 Kg) who was admitted to hospital with an acute manic episode. She had a previous history suggestive of a depressive episode, for which she was prescribed escitalopram 10 mg by her family physician about 2 months prior to current admission. At admission, escitalopram was discontinued and divalproex sodium EC 500 mg daily was initiated, the dose of which was increased to 1000 mg daily at bedtime within a week. The steady state serum level was 452 mmol/litre (therapeutic range 350–700). Risperidone (1.5 mg hs) was added to address some unresolved paranoid symptoms. The patient improved clinically enough in 4 weeks to be discharged from hospital. During a subsequent ambulatory appointment one month later, she presented with depressive symptoms including a lack of motivation, low energy, and tearfulness. She complained of “grogginess” with risperidone; henceforth the dose was tapered to 1 mg at bedtime and then 0.5 mg after another month. Three months after her discharge she continued to complain about ongoing depressive symptoms. Her serum valproate level on the 1000 mg dose was 784 mmol/L. Her dose of valproate was then reduced down to 750 mg at bedtime. At her next presentation in six weeks, she was assessed to be euthymic and the serum level of valproate was 362 mmol/L. She continued to remain stable for subsequent 6 months and then experienced another depressive episode. At that point, her serum valproate level was 528 mmol/L whilst on a valproate dose of 750 mg. She was also continuing risperidone 0.5 mg at bedtime. Escitalopram 10 mg was added for management of symptoms of depression and risperidone was discontinued for feelings of “fogginess” on the medication. At the time of reporting the case, the patient has been in remission for about three years on a combination of valproate 750 mg and escitalopram 10 mg. |
A 28-year-old, Jamaican female, gravida 7 para 3 presented to the clinic at 8-week gestation for her first prenatal visit. Her first pregnancy was complicated with uncontrolled hypertension resulting in a term primary cesarean delivery. Prior to this pregnancy she was normotensive. As this pregnancy occurred in Jamaica, medical records regarding the details of her care were not available. The indication for the cesarean delivery was not known to the patient. However, the patient reported that her blood pressure normalized after this delivery. This was followed by an uncomplicated pregnancy and repeat cesarean delivery at term three years later. Over the next three years, she had three elective terminations of pregnancy. A year thereafter, she had another pregnancy that was complicated by proteinuria and elevated blood pressure that resulted in a fetal demise at 29-week gestation. After delivery, her blood pressure normalized and she remained asymptomatic. The presumptive diagnosis by the medical team was early onset severe preeclampsia, and therefore no renal biopsy or subsequent workup was undertaken at the time.\nA year after the last pregnancy she emigrated to the United States and presented to our clinic for prenatal care at 8-week gestation. She denied any medical problems and was not using any medications. She smoked one pack of cigarettes per day and denied the use of alcohol or other illicit drugs. Her initial blood pressure was 96/60 mmHg. Prenatal labs, initial complete blood count, and BUN and creatinine levels were normal ( and ).\nAt 12-week gestation, she returned to clinic for follow-up. Physical examination was positive for 1+ bilateral lower extremity edema. Her blood pressure was 102/64 mmHg. A baseline 24-hour urine collection revealed 5047 g of protein excretion. Subsequent workup with renal ultrasound, microscopic urine analysis, urine electrolytes, and a rheumatology panel consisting of anti-nuclear antibody (ANA), CRP, anti-double stranded DNA, anti-JO-1 antibody, Sjogren's antibody, anti-DNA antibody, anti-cardiolipin antibody, complement C3 and complement C4, thyroid antibody, and anti-smooth muscle antibody was undertaken.\nBy 13-week gestation, the patient gained five pounds over one week, her bilateral lower extremity edema increased 2+, and she now complained of mild shortness of breath. Blood pressure was 110/60. Her rheumatology workup was negative, except for ANA which was positive with a nucleolar pattern. She was started on a sodium restricted diet and jointly managed with a nephrologist and rheumatologist.\nFollow-up at 15-week gestation showed worsening of her lower extremity edema and another five-pound weight gain. She was continued on a sodium restricted diet (2000 mg daily) and started on furosemide 20 mg daily. A week later, there was no improvement in her edema and an additional five-pound weight gain was noted. The dose of furosemide was increased to 40 mg daily. Laboratory results at this time revealed the following: 24 hr urine protein 8020 g, BUN 10 mg/dL, creatinine 0.4 mg/dL, and albumin 0.8 mg/dL.\nBetween 16-week gestation and 23-week gestation the patient was closely monitored with weekly weights, urine protein collection, and blood pressure and for fetal wellbeing. Based on the worsening of her proteinuria range (11 g/day–18 g/day), severe hypoalbuminemia, and bilateral lower extremity edema, she was diagnosed with nephrotic syndrome.\nAt 24-week gestation, patient presented to the labor and delivery triage unit. Examination revealed +4 bilateral lower extremity edema with fluid tracking up to the abdomen. The patient stated that she was unable to ambulate. Her 24-urine protein had increased to 13,000 g. Her BUN was 7 mg/dL, creatinine was 0.7 mg/dL, and blood pressure was 111/67 mmHg. The patient was admitted to the hospital and subsequently started on 25% intravenous albumin and 40 mg furosemide twice a day for diuresis.\nRenal biopsy was done at 25 weeks. On light microscopy (), the glomeruli were enlarged, and 3/17 glomeruli were globally sclerosed. The mesangium showed an increase in cellularity. There was no endocapillary proliferation, crescents, or fibrinoid necrosis present. The capillary loops were thickened and showed spikes on sliver methenamine stain. The tubules showed focal signs of acute tubular injury with vacuolation, blebbing, dilatation, and nuclear dropout. There was not vasculitis or vascular necrosis present. PLA2R was negative. On direct immunofluorescence () there was granular staining in capillary loops for IgG (2+), IgA (1+), IgM (trace), C3 (trace), C1q (trace), kappa (1+), lambda (2+), and fibrinogen (trace). There was no significant glomerular staining for albumin, nor significant staining in the tubular basement membrane or vessel walls. Electron microscopy () revealed global thickening of the glomerular basement membrane due to subepithelial and intramembranous immune type electron dense deposits. There were no subendothelial or mesangial immune type deposits noted.\nThe patient was started on Tacrolimus 2 mg daily and 10 mg oral prednisone at 27 weeks. She lost 5 pounds in the following two weeks. Fetal growth continued to be appropriate for gestational age and she denied any headache, visual disturbances, or elevated blood pressure.\nShe represented in preterm labor at 30-week gestation. Her vital signs were stable with BP: 110/77, HR: 80, and proteinuria of 18 g. She subsequently delivered a 2 lbs 8 oz female infant with Apgar scores of 3 and 6 at 1 and 5 minutes, respectively. The patient symptoms improved significantly after delivery. She had lost 70 pounds of fluid weight by 3 weeks postpartum and continued on the regimen of Tacrolimus and prednisone. |
63-year-old woman who had suffered from the right medial knee pain for 5 years and was not responsive to conservative treatment was admitted to our clinics. 30° varus-valgus stress X-ray indicated that the patient had an intact MCL and LCL. After the detailed physical examination and reviewing of X-ray images, it was decided that UKR would be the most suitable option for the patient with anteromedial knee osteoarthritis. After spinal anesthesia application and sedation, the UKR surgery was performed with a standard minimal invasive midline vertical incision and a medial parapatellar approach; the patella was removed laterally but not dislocated or everted. The patient received a medial partial knee implant with a mobile-bearing insert (medium size with 4 mm thickness; Oxford®, Zimmer Biomet Inc., Warsaw, IN, USA). Following the UKR surgery (), weight bearing was allowed as tolerated by the patient and a standard postoperative physiotherapy was started on the first postoperative day. The patient was discharged at postop 2nd day when she met the following criteria: independent ability to get dressed, to get in and out of the bed, and to sit and rise from a chair/toilet; independence in personal care; and mobilization with crutches. After discharge, a home-based exercise program was given to the patient. At postoperative follow-up, our patient acquired a full knee RoM in the postop 1st month and returned to independent daily activities without any external support in the postop 3rd month.\nAt postoperative 1st year after first UKR application, the patient fell down while getting on a public bus; this caused that the right knee of the patient was exposed to the valgus force vector. After that moment, the patient heard a pop sound and felt an incredible pain that prohibited the flexion and/or extension of the medial side of the right knee. And then she was admitted to our emergency department. The first evaluation was performed, and the patient was diagnosed with a grade 3 MCL rupture and the UKR insert dislocation (). Having completed the preoperative preparations, the patient was operated on the same day. After anesthetic administration, a surgery with a standard minimal invasive midline vertical incision and a medial parapatellar approach (to a previous incision site) was performed to change the mobile-bearing insert with the same size (medium-sized mobile-bearing insert with 4 mm thickness; Oxford®, Zimmer Biomet Inc., Warsaw, IN, USA). After having changed the mobile-bearing insert, the MCL structures were repaired and anchored to its femoral origin with a 5 mm titanium anchor. Following the surgery, weight bearing and full RoM with a hinged knee brace were allowed as tolerated by patient and a standard postoperative physiotherapy was started on the first postoperative day. Crutches were recommended for 2 to 3 weeks to enable the patient to regain a normal gait. The brace was used continuously for 4 weeks and thereafter during the day for 2 weeks. After the physiotherapy program administration, the patient was discharged at postop 1st day.\nThe patients were evaluated regarding pain intensity (Numeric Pain Rating Scale (NPRS)), active range of motion (RoM), and quality of life (Short-Form 12 Health Survey (SF-12 Health Survey)). Functional capacity was evaluated using the Iowa Level of Assistance Scale (ILAS), Iowa Ambulation Velocity Scale (IAVS), Hospital for Special Surgery (HSS) knee score, and Timed Up and Go (TUG) test. Rehabilitation program and outcome evaluation were conducted by one clinical physiotherapist at preoperative period (before the first UKR application), at discharge (postop 2nd day after the first UKR surgery), and at postop 2nd year (after 2 years from the MCL repair and the insert change). The evaluation results are shown in . |
A 60-year-old man with a past surgical history of C5–C7 anterior arthrodesis for cervical fracture 17 years ago was found on the floor after an unwitnessed ground-level fall, resulting in a head strike. He was taken to the local hospital in an Aspen collar with a GCS of 15 and no neurologic deficits. Routine ECG and laboratory evaluation was unremarkable except for elevated serum alcohol level. Full cervical spine computed tomography (CT) scan was performed which showed an Anderson and Montesano classification (20) type III left occipital condyle fracture (), a Levine and Edwards classification (21) type III (Jefferson) fracture with mild lateral subluxation of bilateral C1 masses (), and an asymmetric widening and slight anterior subluxation of the right AOJ (). A head CT was performed which showed no acute intracranial abnormalities.\nThe patient was immediately transferred to a level I trauma center for further management. In the emergency department, he reported severe midline neck pain, worse with movement, and unchanged paresthesia to bilateral upper extremities which he states is his baseline. Physical exam was unremarkable except for a right forehead hematoma with overlying abrasions and tenderness to palpation over the midline posterior neck. He exhibited full strength and unchanged baseline sensation to all extremities. He denied bowel or bladder incontinence and had strong rectal tone and intact perianal sensation. Through his clinical course, he became increasingly altered and agitated, refractory to medication. He was unable to maintain spinal precautions due to his agitation, and in an attempt to protect his cervical spine, he underwent endotracheal intubation.\nMRI of the cervical spine was performed and showed ligamentous injury at the craniocervical junction (CCJ) (Figures and ), asymmetric left odontoid-lateral mass widening, widening and subluxation of the right AOJ (), and edema of the paravertebral soft tissues around the fracture. CT and MRI established the diagnosis of OCD in a neurologically intact patient. Due to the unstable nature of the fracture, the patient was taken to the operating room for emergent occiput-C2 posterior instrumentation and fusion ().\nAn Aspen collar was in place at all times until surgical stabilization. Immediately, after removing the collar, Mayfield tongs were applied. Prior to patient positioning, baseline somatosensory evoked potentials (SSEPs) were obtained. Using a Jackson table turning frame, the patient was rotated into prone position. Fluoroscopic imaging was used during manual manipulation of the Mayfield apparatus to ensure no further displacement of the fracture.\nOcciput to C2 posterior instrumented arthrodesis was performed. Neuromonitoring remained stable during the procedure. The patient was discharged to home five days after presentation. |
A 44-year-old woman with a history of metastatic triple negative breast cancer and lung metastases presented with a six-month history of recurrent haemoptysis. She had no other significant medical history. She was initially managed for her right breast cancer with a wide local incision and adjuvant chemoradiotherapy in 2014; however, her malignancy recurred two years later. She had positive margins on subsequent right mastectomy and proceeded to excision of the right pectoralis major and overlying dermis. Six months later she was found to have bilateral pulmonary metastases and underwent initial diagnostic bronchoscopy identifying a bleeding mass in the medial segment of the right middle lobe (RB5), which was subsequently treated with topical adrenaline and biopsied – confirming metastatic disease. Her malignancy progressed despite palliative chemotherapy with epirubicin and cyclophosphamide, during which time she developed worsening haemoptysis of ~1/2 cup (~120 mL) daily. A multidisciplinary decision was then made to perform therapeutic bronchoscopy due to excessive distress caused to the patient because of haemoptysis. She underwent bronchoscopy using a therapeutic video bronchoscope (Olympus BF-TH190, Olympus Corporation, Tokyo, Japan) introduced via a rigid bronchoscope, which provided secure airway access. Endobronchial survey revealed the source of bleeding in the distal right middle lobe, although the actual bleeding source was not directly visible. A volume of 2 mL of TISSEEL was injected into the right middle lobe bronchus via a catheter followed by deployment of a size 6 Spiration (Redmond, WA, USA) IBV to add stability and prevent expectoration (Fig. ). A further 1 mL of TISSEEL was then applied over the valve (Fig. ). The procedure abolished the patient's haemoptysis instantly.\nTwo weeks later the patient developed recurrent haemoptysis; however, repeat bronchoscopy showed a different source of bleeding in the right lower lobe, with the existing combination TISSEEL and IBV still in place in right middle lobe and maintaining haemostasis. To control the new bleeding TISSEEL was injected in the right lower lobe bronchus distal to the opening of RB6, followed by deployment of a size 9 IBV. Further injection of TISSEEL was then applied and haemostasis was achieved. Unfortunately the patient was found to have brain metastases and died of her malignancy 10 weeks later, without recurrence of haemoptysis. |
A 50-year-old woman presented with iatrogenic habitual dislocation of left patella from childhood and pain from past 3 years. She recalled a childhood surgery over middle third of left thigh (incision and drainage). Few years after the surgery, she started noticing the giving way of patella and lived for 38 years without much disability. She was carrying out her daily activities without much pain or symptoms, but she was never symptom free during this period.\nPhysical examination did not reveal general joint laxity or malalignment of the lower extremity. There was a healed surgical scar over middle and lower third lateral aspect of the left thigh. Although the patella was in the femoral trochlear groove in extension, it dislocated laterally with 25 degrees of knee flexion. She complained slight discomfort by an apprehension test, but was not very painful even when dislocated. The passive lateral patellar tilt test was negative since the soft tissue attachment to the lateral border of the patella was diffusely stiff and tight. Radiological examination revealed evidence of patella alta as defined by Insall and Salvati,[] and Q angle was 18 degrees. Clinically and radiologically (Grade III Kellgren and Lawrence classification[]) tricompartmental degenerative knee arthritis was appreciated []. Preoperative Kujala score[] was 49.\nWith patient in supine position under epidural anesthesia, an anterior midline knee incision was used with paramedian arthrotomy. The lateral retinaculum was thickened while the medial retinaculum was thinned. The lateral tether is released by dividing the fibrous adhesions, which extended along the lateral intermuscular septum. The lateral patellar retinaculum and the synovium were divided and vastus lateralis was detached from the quadriceps tendon, leaving a rim of tendon for suturing.\nDue to shortening of the extensor mechanism, the patella could not be reduced. Quadriceps tendon was lengthened using V Y plasty at aponeurotic junction. Tracking was augmented with MPFL reconstruction using semi-tendinosus tendon and patellar end was fixed with 2.8 mm FASTak titanium anchor on the medial border of patella and over near isometric point on medial epicondyle of femur, which was checked under fluoroscopy. Patellar maltracking was persistent and so, it was decided to proceed with medial and distal transfer of tibial tuberosity.\nA long-leg hinged knee brace was applied with the knee in 20° of flexion and partial weight bearing with crutches for four weeks was advised, during which the patient was encouraged to do vigorous static quadriceps strengthening exercises. Knee mobilization and full weight bearing was started after four weeks. Initially patient had extensor lag of 15 degrees as she was immobilized in flexion. But after vigorous quadriceps exercises, patient improved without any extensor lag.\nAt final followup two years postoperatively, the patient was pain-free. There was no evidence of patellar maltracking or instability on detailed physical examination. Radiographs showed well reduced patella in both lateral, anteroposterior view and skyline view []. The patient's kujala knee society score 89 [] and range of motion was 0° to 90° on the left side []. Quadriceps strength was rated 4 (MRC grade). |
A 55-year-old non-diabetic, non-hypertensive male with a history of recurrent colicky left lumbar pain presented with acute urinary retention. Catheterization was attempted; however, it was unsuccessful. Ultrasound revealed an overdistended urinary bladder with a normal-sized prostate and scarring and focal caliectasis in the left kidney (). The right kidney was normal and no calculi were seen on either side on ultrasound. Serum electrolyte, renal and liver functions were normal. The haemogram revealed neutrophilia. The prostate-specific antigen was within normal limits. A rigid urethroscopy was performed owing to suspicion of a left urethral calculus and a 11-mm size calculus was removed from the posterior urethra. The patient was discharged and had no difficulty with micturition for 2 weeks thereafter. The patient subsequently developed burning micturition with hesitancy and induration in the perineal region. The urine was turbid and microscopy revealed the presence of Escherichia coli. A perineal ultrasound revealed an abscess in the perineum, which extended to the proximal parts of the corpus spongiosum (). This abscess was drained under saddle block. The patient subsequently developed a discharging sinus at the operative site () leaking purulent fluid. A retrograde urography revealed periurethral extravasation of the injected contrast material with a lytic lesion in the left pubic ramus (). A repeat perineal ultrasound revealed a linear hypoechoic tract leading from the skin surface to the corpus spongiosum (). A retrograde CT urography was performed to look for the extent and ramifications of the abscess. On the non-contrast CT scan, a lytic lesion was seen involving the left inferior pubic ramus (). The pubic symphysis and bodies of both pubic bones were normal. A proximal femoral nail was noted in situ on the left side, which was inserted 10 years before for fracture of the proximal shaft of the femur owing to accidental trauma. On injecting iodinated contrast into the urethra, there was extravasation of the contrast in the periurethral region in the soft tissues surrounding the bulbar and the posterior penile urethra. An extension of the contrast through the external anal sphincter into the intersphincteric plane () with inflammatory stranding in the ischioanal fossae was seen. The contrast also extended into the lytic lesion present in the left inferior pubic ramus (). The contrast also extravasated through the cutaneous opening in the perineum and the natal cleft (). E. coli were isolated on pus culture. The patient was treated with intravenous antibiotics and suprapubic cystostomy was performed. The patient is presently being considered for elective urethroplasty. |
An 86-year-old man was referred for echocardiography with a 2-month history of anginal chest pain of New York Heart Association (NYHA) class II without hospital admissions. The patient had a history of cigarette smoking for 20 years and systemic hypertension for 10 years. In addition, he had atrial fibrillation rhythm.\nOn examination, the patient showed no signs of cyanosis or clubbing, with O2 saturation of about 90% in air room. Chest radiography showed a globe-shaped heart, with normal vascularity and a cardiothoracic ratio of 0.5. Echocardiography revealed features of Ebstein’s anomaly with mild-to-moderate tricuspid regurgitation () and right ventricular and atrial dilation. The function of the right ventricle was preserved. The ratio between the area of the functional right ventricle and the combined area of both the right atrium and the atrialized right ventricle was 42%. The anterior leaflet of the tricuspid valve was enlarged but pliable with mild tethering, and the septal and posterior leaflets were significantly displaced (about 14 mm/m2 for the septal and 16 mm/m2 for the posterior leaflet; ). The left ventricle was of normal size and thickness, and the ejection fraction was estimated at 45-50% due to wall motion abnormalities in the septal, lateral, and anterior segments. There was a small patent foramen ovale, seen in contrast study (). Other interesting echocardiographic findings were bicuspid aortic valve () with no stenosis and only mild regurgitation and dilated coronary sinus because of persistent left superior vena cava. The size of the ascending aorta and aortic arch was normal with no coarctation. Coronary angiography revealed a long left main with 50% distal stenosis and significant proximal lesions in the proximal left anterior descending coronary artery and obtuse marginal.\nThe patient was candidated for coronary artery bypass grafting surgery, but he refused cardiac surgery. He was, therefore, discharged on medical anti-ischemic therapy (Aspirin, beta blocker, Statin, angiotensin-converting enzyme inhibitor) and anticoagulation therapy with a target international normalized ratio (INR) of 2-2.5 and recommended to have regular follow-ups. |
A 51-year-old woman underwent hysterectomy and pelvic lymph node dissection for uterine cancer when she was 48 years old, and lymphedema developed in the left leg soon after the operation. She had one episode of cellulitis. Despite wearing elastic stockings, lymphedema worsened, and she visited our institution at the age of 49 years. She was diagnosed with lymphedema based on lymphoscintigraphic finding. There was a development of collateral lymphatic vessels and dermal backflow in bilateral lower leg (See figure, Supplemental Digital Content 1, which displays lymphoscintigraphic findings. Collateral lymphatic vessels were observed in the bilateral lower legs. Lymphatic function in the left thigh was impaired, ). She had no allergies or other pertinent medical histories.\nLVA was performed at the age of 50 years. The postoperative course was uneventful, and lymphedema improved. However, lymphedema worsened again at 1 year postoperatively after taking a long flight, although she wears elastic stockings daily. A second LVA was planned (Fig. ).\nPreoperative indocyanine green (ICG) lymphography showed a linear pattern in the right leg. Dermal backflow was observed in the left thigh and lower leg. There was no linear pattern in the area (left lower leg) where the lymphatic thrombus was found afterward. Preoperative echography showed 2 hypoechoic circles measuring about 0.5 mm in diameter that did not collapse with pressure from the probe, although the veins collapsed with pressure (Fig. ). Compared with lymphatic vessels, veins usually collapse more easily under pressure, because the inner pressure of the lymphatic vessels is higher than that of the veins. In this case, the 2 circles did not collapse under pressure, and we surmised that the inner pressure prevented collapse. The hypoechoic circles extended proximal and distally and did not have flow with on color Doppler mode.\nDuring LVA, we identified 2 parallel white vessels beneath the superficial fascia. Two vessels were in close contact. We diagnosed these as lymphatic vessels because of the location and appearance and the fact that they ran in parallel, which is not usually observed with other vessels or nerves. When we incised the vessels, white material was extruded (Fig. ). A diagnosis of lymphatic thrombosis was made, and we concluded that the vessels did not collapse with pressure from the probe during echography because of thrombus. Intraoperative echography revealed the same findings, that is, a hypoplastic circle without collapse by probe pressure, 15 cm distal from the incision, which indicated that there was lymphatic thrombus in at least 15 cm. We ligated the lymphatic vessels, closed the wound at this site, and performed LVA at other sites (4 sites in the left and 1 site in the right leg). Though the postoperative course was uneventful, the patient’s lymphedema did not improve postoperatively. This may be partially because the patient gained weight after LVA, and there is another possibility that postoperative thrombus formed within the anastomosis site had harmful effect for lymphedema, although it is difficult to confirm.\nHistopathological examination showed a thickened smooth muscle layer (tunica media) in the lymphatic vessels (See figure, Supplemental Digital Content 2, which displays histopathological findings of the lymphatic vessel and the lymphatic thrombus. (a) The lymphatic vessel (a) and the lymphatic thrombus (b) (×20, H&E). Thickened smooth muscle layer (tunica media) in the lymphatic vessels is observed. (c) The lymphatic vessel (×100, D2-40). The endothelial cells of the vessel were negative for D2-40, ). Fibrous thickening of the tunica intima was observed, and the inner lumen was narrow. The inner layer of the vessel was negative for D2-40, which is a marker to stain the lymphatic endothelial cells. In the thrombus, hyperplasty of fibroblasts and organization were found (Fig. ). We did not observe hyperplasty of the lymphatic endothelial cells, which are positive for D2-40 within the thrombus. |
A 67-year-old male with prior history of diaphragmatic trauma eight years earlier and subsequent diaphragmatic hernia approached through thoracotomy, requiring open thoracoabdominal aortic reconstruction in 2017 due to a suprarenal abdominal aortic aneurysm; reconsults six months later to our emergency department with coffee ground vomit, general paleness and moderate dehydration. At his arrival, non-variceal upper gastrointestinal bleeding was suspected, undergoing an esophagogastroduodenoscopy without evidence of active bleeding and a chest x-ray that identified an image of a lower left pulmonary consolidation. A chest CT-scan was performed, illustrating a left diaphragmatic hernia with protrusion of the gastric body into the ipsilateral hemithorax.\nGiven the findings, an abdominal CT-scan was performed identifying a seven centimeter left hemi-diaphragmatic defect associated with herniation of the fundus, body and gastric antrum, along with the spleen (A and B). Due to the patient’s severe malnutrition, parenteral nutritional repletion was necessary since the passage of an advanced nutrition probe was not possible due to the anatomical alteration generated by the hernial defect.\nFifteen days after admission, the patient underwent abdominal laparoscopic surgery with evidence of a large left diaphragmatic hernia with protrusion of half the stomach, omentum and the posterior aspect of the spleen. Additional findings were the spleen had a sub capsular tear, as well as a severe adhesion syndrome on the chest wall and diaphragm with entrapment of the inferior lobe of the left lung. A hernial defect of seven by five centimeters was found, with preservation of the hiatus. The reduction of the herniated organs was achieved through abdominal laparoscopy, however a complete dissection was not possible due to the splenic interposition (A–C). The Thoracic Surgery group, through a thoracoscopic approach, performed extensive adhesiolysis and decortication, achieving the extraction of the protruding sac without damaging the spleen thus enabling a primary repair without tension (D–F).\nTwenty-four hours postoperatively, the patient tolerated the liquid diet, presenting an uneventful recovery, being discharged on the fifth postoperative day with normal food intake. Six months later he was reviewed with an abdominal CT-scan showing a satisfactory diaphragmatic hernial correction (A-B). |
A 40-year-old man (Caucasian, M.Sc., working as a fund manager, not married, not religious) reported that he had been suffering from sleep-related painful erections (SRPE) for 6 years. He awoke several times a night with a painful erection. The erection and the pain could be relieved by physical exercise or cooling the penis for about 3 min. Remarkably, he reported that his SRPE only occurred after having sexual intercourse, including ejaculation, the evening before. He did not have SRPE when intercourse and ejaculation had taken place during other times of the day. The duration of the intercourse did not make any difference; if he ejaculated during intercourse before he went to sleep, SRPE would occur in any case. After masturbation, he experienced no problems at all.\nBecause of these SRPE, the patient experienced significant problems in his romantic relationships. His former partner felt dissatisfied with the diminished frequency of intercourse, and, partly due to this, their relationship ended. The patient still experienced SRPE in his current relationship, which began 3 years after his former one ended. Because of his SRPE, the patient decided to stop having intercourse for a number of weeks. This led to slight relationship problems and caused his partner to advise him to consult the general practitioner (GP). He asked his GP whether he could rule out medical abnormalities and whether he could recommend a non-pharmacological therapy to remedy his complaints. The patient underwent a biopsychosocial evaluation by his GP, ruling out medical conditions, drug abuse, psychological issues, and major relationship problems. Physical examination, a neurological examination, examination of the external genitalia, digital rectal examination, blood pressure and pulse also did not show any abnormalities. Because the patient wanted to be sure there were no other medical abnormalities, he requested a referral to a urologist. Subsequent visits to the urologist provided the following information: Doppler ultrasound of his penile vessels performed after a night without SRPE was normal; nocturnal penile tumescence registration (NPTR) with a RigiScan device (Dacomed Corporation, Minneapolis, MN, USA) showed five periods of SREs, the last one with a duration of about 90 min. After the urologist visits, the patient was referred back to his GP. The patient refused any proposed pharmacological treatment, because of “not wanting to be a patient at his age.” Because his SRPE only occurred after having sexual intercourse the previous evening, his GP suggested the patient should have intercourse at other times of the day. As a result, the patient no longer experienced SRPE and this effect remained during the follow-up period of 3.5 years.\nUnfortunately, despite this result, his current relationship ended for other reasons than shifting the time of sexual intercourse. In the last 3 years, he has had some short-lived relationships. He now is a schooled practitioner of tantric sex (defined by him as practices in which slow, mindful, non-orgasmic sexual union, or masturbation creates a path to the experience of spiritual ecstasy) and aims to eliminate all orgasms, both during intercourse and masturbation. Only a few times, in the last 3.5 years, he had sexual intercourse, including ejaculation in the evening. Without exception, these were followed by awakenings several times a night with a painful erection. |
An 80-year-old man was admitted to our hospital after presenting with high fever and right subcostal pain. He had no history of trauma, bacteremia, or risk factors for cardiovascular disease such as hypertension, diabetes mellitus, or hyperlipidemia. Physical examination showed that tenderness was present in the right hypochondriac region and a pulsatile mass was palpable in the periumbilical region. Laboratory tests revealed elevated hepatobiliary enzymes, a high serum C-reactive protein (CRP) concentration of 22.1 mg/L, a high white blood cell (WBC) count of 10,100/µL, and a high erythrocyte sedimentation rate of 29 mm/h; however, blood cultures were negative. Additionally, the patient had a normal serum immunoglobulin G4 concentration and negative autoantibodies, which include antinuclear antibody and antineutrophil cytoplasmic antibody (ANCA) serology. Systemic contrast-enhanced computed tomography (CT) revealed enlargement of the gallbladder with wall thickening without stones and a 36-mm dilatation of the SMA. No other vascular entities were detected. Based on these clinical and imaging findings, the patient was diagnosed with acute cholecystitis and SMAA. The SMAA was located 11 cm distal to the origin of the SMA and ended at the origin of the ileocolic artery. No signs of irregular or lobulated arterial walls, soft tissue inflammation or masses around vessels, perivascular fluid collections, or aneurysms with intramural air were noted. The SMAA contained a large intramural thrombus with no findings of occlusion throughout the SMA (). Although we considered that infection might have caused the SMAA, CT images showed no typical findings of a mycotic aneurysm. Therefore, we scheduled a surgical intervention for the SMAA after improvement of the cholecystitis, and the patient immediately underwent percutaneous transhepatic gallbladder drainage and intravenous antibiotic therapy. Additionally, since these treatments started, we had performed abdominal CT every 2 or 3 days for following up of cholecystitis and SMAA while strictly monitoring the laboratory data, including WBC count, CRP levels, and fever. After 2 weeks, his condition and clinical findings had remarkably improved. Laboratory tests showed a normal WBC count and serum CRP concentration of 2.68 mg/L. His symptoms, such as high fever and subcostal pain, were diminished. During this period, no growth of the SMAA was found on CT images. Thus, we performed an elective surgical intervention for the SMAA.\nThe patient was in supine position during surgery, and the SMAA was exposed through an upper median abdominal incision. The surface of the aneurysm was white and glistening, and severe adhesion between the aneurysm and surrounding tissues was present. Although unswollen, the gallbladder had a thickened wall, and there were slight adhesions around it. Tissues between the gallbladder and SMAA were intact. After clamping the proximal and distal sides of the aneurysm, we assessed the viability of the intestinal tract based on intestinal color changes. The aneurysm was subsequently opened. The aneurysm wall was very thick, and a red-brown thrombus was found. A small amount of backflow from the peripheral SMA was present. We resected the aneurysm and interposed a great saphenous vein graft taken from the left groin within the SMA (). There were no signs of intestinal tract ischemia before or after reconstruction.\nThe cultures of the aneurysm and adjacent tissue were negative. Histological examination of the aneurysmal wall showed dense infiltration of inflammatory cells, which mainly comprised lymphocytes that were predominantly located in the media and adventitia. Adventitial fibrosis was also observed, and the tunica media exhibited partially decreased, fractured, and absent elastic fibers (). Immunohistochemical staining was negative for immunoglobulin G4. Intraoperative and histopathological findings indicated an immunoglobulin G4-unrelated inflammatory aneurysm. The patient’s postoperative course was uneventful. Postoperative CT revealed good patency of the interposed graft. At 2-year follow-up, the patient was alive and well, and contrast-enhanced CT showed no evidence of the occlusion. |
The patient, a term male at 37 weeks’ gestational age born from a primigravida 26-year-old mother, was admitted to our NICU soon after birth for presenting signs of respiratory distress and severe intrauterine restriction.\nPrenatal screening of third trimester despite normal amniotic fluid revealed that the fetus was suffering intrauterine growth restriction. The newborn was a symmetrical, small for gestational age (SGA) with birth weight 2285 gr (P 3rd-10th), length 44 cm (P 3rd), and head circumference 32 cm (P 3rd-10th). He was born with cesarean section, due to fetal distress with Apgar score of 7 and 8 at 1 and 5 minutes, respectively. Maternal history revealed depression and anorexia nervosa since 18 years of age. AN was intensified during the second and third trimester of pregnancy. Mother's body weight at first and third trimester was 52 and 43 kg, respectively; with a height of 162 cm BMI fell from 19, 8 kg/m2 to 16, 3 kg/m2. An arterial blood analysis from the umbilical cord within normal range according to his gestational age and day of life. Maternal showed metabolic alkalosis, hypokalemia, hypochloremia, and hyponatremia, which were also confirmed in subsequent measurements. It was confirmed that the infant had no episodes of vomiting, diarrhea/multiple stools and was not on any drug administration. Urine analysis for hypercalciuria, chloride concentration, and ultrasonography of the kidneys were normal. In view of the intrauterine growth restriction, the infant was evaluated for intrauterine infection that had normal findings. The arterial blood pressure of the baby was blood analysis revealed similar findings (Table ). Total parenteral nutrition started on 1st day of life (dol), while enteral nutrition on 2nd dol with electrolyte supplementation. Alkalemia and electrolyte disorders were progressively corrected within the first week of life. The newborn had no signs of respiratory distress after 12 hours admission in NICU, and with an uneventful NICU hospitalization, he was finally discharged at 10 dol.\nAt 6 months of life, the baby had a normal physical and neurolodevelopmental examination. |
We detail the case of a 48-year-old man, never-smoker and non-drinker, who was referred to the Clinic of Oral and Craniomaxillofacial Surgery at the University Hospital Basel, Switzerland, by his dentist, for further evaluation of a solitary ulcer on the lower left retromolar buccal plane. The patient reported swelling of the left cheek since 5 days, mild pain while chewing, and a swelling on the buccal mucosa related to the left lower jaw since about 1 year. Fever, night sweat, and weight loss were denied. Due to his obsessive-compulsive disorder, the patient has been under psychiatric medication treatment in the past (Deroxat®, paroxetine, selective serotonin reuptake inhibitor (SSRI)), but at the point of consultation, he did not take any medication. The patient reported on his allergy to penicillin, with erythematous rash episodes in the past. The data set includes the medical record of our TUGSE case including the corresponding photo material. No risk for third parties existed. Informed consent was obtained from the patient for case description and photo material.\nExtraoral examination showed a slight swelling of the cheek on the left side without redness. Palpation of the cervical lymph nodes revealed a slightly enlarged submandibular lymph node at level Ib on the left side, with a 2 × 1.5 cm diameter. Palpation of the immovable lymph node was painful. The nuchal, the axillary, the cubital, and the inguinal lymph nodes were not swollen. The diameter of the intraoral lesion on the lower left retromolar buccal plane was approximately 4 × 3 cm; the lesion presented as indurated base with a central superficial ulceration of 2 × 1 cm (Fig. ). The rapid growth of the ulcer over the last 5 days was potentially indicative for a malignant process, wherefore we performed an incisional biopsy to obtain histopathological diagnosis after we had obtained informed consent from the patient. Tests for EBV, lues, HIV, hepatitis B, and hepatits C turned out to be negative. Nine days after the biopsy, the lesion showed a tendency towards regression.\nHistologically, the ulceration showed an expanding, infiltrative, and vaguely granulomatous morphology, involving the superficial mucosa and the fatty tissue, and extended between the deep striated muscle fibers. The lesion was rich in lymphocytes, histiocytes, and eosionophils intermingled with activated T-blasts without phenotypic abnormalities (co-expressing CD2, CD3, CD5, and CD7, and lacking CD30) (Fig. ). Although TUGSE was already presumed at that point, further molecular analyses were performed due to the large size of the lesion, its destructive and deep-penetrating nature, and the presence of large lymphoid forms with eosinophilic nucleoli. Deoxyribonucleic acid (DNA) was extracted from the mucosal biopsy. Using multiplex polymerase chain reaction and high-resolution fragment length analysis of the DNA, B-, and T-cell clonality as well as chromosomal translocations t(11;14) and t(14;18) were assessed []. A biclonal T-cell receptor rearrangement of type V-I and V-III was detected, but there was neither evidence of clonal B-cells nor of the translocations t(11;14) and t(14;18). TUGSE was then diagnosed on the basis of the phenotype (especially the lacking expression of CD30, the retained T-cell phenotype and the absence of EBV), the clinical presentation, and the morphology.\nNine days after biopsy, the patient was pain-free and the ulceration showed significant regression (Fig. ). We performed a standard orthopanthomography (Fig. ), which revealed no other pathological finding than the clinical evident buccal angulation of tooth 38 which might have caused the microtrauma of the ulceration. We therefore recommended the prophylactic extraction of the respective tooth. However, the patient decided against it. We saw the patient in a regular follow-up program, quarterly within the first half year, every 6 months within the first year, and annually for 2 years. Twenty-six months after diagnosis, no recurrence of the ulceration was seen, even though the mucosa still showed signs of microtrauma with punctate hemorrhage (Fig. ). |
A 6-year-old, previously healthy boy, presented to the Pediatric department because of a strongly positive tuberculin skin test (34 mm) and mild chronic swelling with a skin fistula in the left submandibular area, which developed 3 months prior to admission. He was initially treated with oral and then intravenous broad spectrum antibiotics for 3 weeks with the presumptive diagnosis of cervical adenitis with no response at another hospital. After evaluation by an oral and maxillofacial surgeon, the diagnosis of a submandibular abscess originating from the decayed primary second lower left molar was made (Fig. ). The patient underwent incision and drainage of the abscess extraorally and extraction of the involved tooth. Postoperatively, the symptoms improved, and he was discharged. Routine bacterial cultures were negative. The following 2 months, he had persistent moderate swelling, and a fistula developed at the incision site.\nThe patient had no history of contact with a case of active tuberculosis, and investigation of family contacts was negative. He lived in an urban area and had no contact with cattle or history of consumption of unpasteurized dairy products. The child was born in Greece and never travelled abroad. Both grandparents and parents were born in the former Soviet Union and had immigrated to Greece 20 years ago. The patient was not immunized with Bacillus Calmette–Guérin vaccine.\nPhysical examination on admission revealed a well-nourished boy in good clinical condition. A left submandibular firm, erythematous swelling measuring 3 × 3 cm, was palpated with a draining sinus tract. Laboratory studies were unremarkable. Blood tests revealed a white blood cell count of 15,200/mm3 with 74% polymorphonuclears, 18% lymphocytes, and 8% monocytes; erythrocyte sedimentation rate was 50 mm/h, and C-reactive protein was 77 mg/L. Measurement of serum immunoglobulins, determination of lymphocyte subpopulations by flow cytometry, and nitroblue tetrazolium test were performed and were normal. Serologic testing ruled out human immunodeficiency virus (HIV) 1 and 2 (enzyme-linked immunosorbent assay method), toxoplasmosis, cytomegalovirus, Epstein–Barr virus, and Bartonella henselae infection. Furthermore, chest radiography and abdominal ultrasound were normal, while culture of gastric aspirate was negative. QuantiFERON-TB Gold In-tube (QFT-IT) test was positive. A computed tomography of the region revealed the presence of chronic osteomyelitis of the mandibular body adjacent to the extraction site with osteoblastic subperiosteal activity and thickening around the lower mandibular border (Fig. ).\nAntituberculous treatment was initiated with isoniazid, rifampicin, and pyrazinamide (PZA), and the patient underwent surgical excision of the submandibular mass and the draining sinus. The histopathological examination revealed no lymphoid but only fatty and fibrous tissue with granulomatous caseating infection. Acid fast bacilli were visualized by Ziehl–Neelsen stain. Two months later, mycobacterial growth was evident on Lowenstein–Jensen medium. The culture isolate was identified as M. bovis subsp. bovis by means of the GenoType Mycobacterium tuberculosis complex (MTBC) assay (Hain Lifescience, Germany). This assay is based on the DNA-STRIP technology and permits the genetic differentiation of species belonging to the Mycobacterium complex on the basis of gyrase B polymorphisms []. Our finding was subsequently verified in the central laboratories of the kit manufacturer. Susceptibility test showed resistance to PZA, as expected. Based on these results, PZA was discontinued, and ethambutol was added to the therapeutic regimen. By the end of a 6-month therapy, the lesion had healed and showed complete resolution. The patient has been followed for 2 years and had no recurrence. |
A 73-year-old Asian woman with an underlying anxiety disorder, functional headache, and hypertension was prescribed escitalopram and lorazepam when she presented with progressively worsening headaches to her primary care doctor. Her symptoms did not improve with the medications, and she was unable to eat well and required bed rest. She was transported to our hospital 4 days later after developing chest and back pain with altered consciousness. She was a housekeeper, had no allergies, and had no alcohol or tobacco smoking history. On arrival, her Glasgow Coma Scale score was 3/15 (E1V1M1); both pupils were approximately 4 mm in diameter and reactive. Her blood pressure was too low to be measured, her carotid artery pulse was palpable, her heart rate was 112 beats/minute, and her respiratory rate was 30 breaths/minute. Her conjunctiva was pale. An auscultation of breath sounds did not reveal upper and lower airway obstructions and was within normal limits. Her abdomen was soft and flat without tenderness. She had no skin abnormalities (such as rash). Both legs had no edema. Echocardiography on arrival was performed as point of care ultrasound and revealed a hypercontractile left ventricle with an eliminated left ventricular cavity and a collapsed inferior vena cava without right ventricular dilation. There was no pericardial effusion or obvious large regurgitant jet observed on color Doppler. In response, we immediately inserted a peripheral venous catheter and began introducing fluid resuscitation; however, she developed PEA. Conventional CPR according to the adult advanced cardiovascular life support guidelines (including adrenaline) was initiated and a return of spontaneous circulation (ROSC) occurred. However, her blood pressure was unstable and PEA returned, prompting repeated CPR with immediate administration of fluids and three adrenaline injections. Venoarterial extracorporeal membrane oxygenation (VA-ECMO) was initiated for refractory PEA. Whole-body contrast-enhanced computed tomography was unremarkable, and the admission laboratory results were also unremarkable, except for anemia (Table ). Her hemoglobin level decreased from 7.1 g/dL to 3.5 g/dL 1 hour later without obvious signs of gastrointestinal hemorrhage. Therefore, 8 units of packed red blood cells were transfused for 1 day, after which her hemodynamic status stabilized. She was in a coma without sedatives; thus, targeted temperature management at 34 °C was initiated on admission to an intensive care unit. Echocardiography in the intensive care unit showed a thickened interventricular septum (which was 12.8 mm), prolonged anterior mitral valve, and contact between the bodies of the anterior and posterior mitral valves, suggesting that the left ventricular obstruction could have potentially occurred through this redundant anterior mitral valve. VA-ECMO was terminated on day 3, and after stabilizing her hemodynamics, transthoracic echocardiography showed a sigmoid septum with normal left ventricular function (ejection fraction, 75%) (Fig. ). On day 26, dobutamine-infused (30 μg/kg per minute) Doppler echocardiography revealed a significant outflow gradient (236 mmHg) accompanied with chest pain (Fig. ) and intermittent systolic anterior motion (SAM) of the mitral valve; continuous monitoring during Doppler echocardiography showed a Brockenbrough–Braunwald sign (Fig. ), which is a fall of arterial blood pressure after premature ventricular contraction; these findings confirmed a diagnosis of latent LVOTO due to a sigmoid septum. The significant LVOTO was not dependent on SAM but might have occurred due to the greatly thickened interventricular septum. As a result, carvedilol was initiated with gradual increment up to 10 mg/day on day 35. In addition, verapamil (120 mg/day) was administered on day 29. A follow-up dobutamine-infused Doppler echocardiography on day 40 showed a reduction of the outflow gradient to 14 mmHg, indicating a successful medical therapy.\nThe worsening anemia that was identified at admission was suspected to be hemolytic anemia (HA) based on results of the blood test. The results of the laboratory examination, including total bilirubin, direct bilirubin, lactate dehydrogenase, reticulocyte count, and haptoglobin, and a direct Coombs test on day 1 are presented in Table . Her drug lymphocyte stimulation test was positive, and agglutination occurred when her serum reacted with lorazepam. Therefore, lorazepam-induced immunological HA was diagnosed. Her anemia improved following discontinuation of lorazepam. Her hemoglobin level was 11.9 g/dL on day 64, and she was eventually discharged on day 68 without any sequelae. |
A previously healthy 6-year-old boy presented to a pediatric hospital with a 3-week history of torticollis. He had symptoms of an upper respiratory tract infection four weeks prior and had 2 days of documented fever at home during that time. He had been treated with a 7-day course of amoxicillin by the primary care physician for suspected streptococcal pharyngitis. Four days into the course of antibiotics, he woke up from sleep with pain on the left side of his neck. Despite taking ibuprofen and acetaminophen, he presented to the Emergency Department 3 weeks later due to persisting torticollis. Pain was worse with movement. There was no history of head/neck trauma. At the time of presentation, the infectious symptoms had resolved. Some fatigue was noted but he remained generally active, continuing to play hockey. There was no history of rash, peripheral joint pain, or weight loss. Past medical history and family history were unremarkable.\nOn examination, the patient was afebrile with normal blood pressure for age and a maximum heart rate of 110 beats per minute. The patient's head was tilted to the right with chin rotation to the left. No lymphadenopathy or masses were noted on palpation of the neck. There was no tenderness to palpation of bilateral sternocleidomastoid muscles. There was a limited range of motion in all planes of rotation of the neck secondary to pain, particularly in lateral flexion. Bilateral injected conjunctivas were present. The oropharynx was normal with no erythema or mucus membrane changes. Cardiovascular exam revealed normal peripheral pulses, a quiet precordium with normal heart sounds, and no murmur. Respiratory exam was normal. The abdomen was soft with no distension, tenderness, or hepatosplenomegaly. There were no bruits heard on auscultation of major vessel regions. There were no rashes or desquamation of the skin. Neurological exam was normal.\nAt the time of presentation, laboratory investigations revealed an elevated white blood cell count of 17.4 × 109/L with a neutrophil count of 14.1 × 109/L. Hemoglobin was normal for age at 110 g/L. Inflammatory markers were elevated including platelet count of 860 × 109/L and CRP of 38.5 mg/L. Renal function (BUN and creatinine) and liver function (ALP and ALT) were normal for age. Because of the unexplained elevated white blood cell count and evidence of inflammation, a chest X-ray was performed which revealed normal lung fields but an enlarged cardiac silhouette. X-ray of the cervical spine was normal with no atlantoaxial rotary subluxation demonstrated. Ultrasound of the neck revealed mild thickening of the left sternocleidomastoid muscle and no lymphadenopathy. Abdominal ultrasound with Doppler was normal.\nAdditional investigations included a normal throat swab for group A streptococci and a negative anti-streptolysin O antibody titer. High-sensitivity troponin was elevated to 176 ng/L. Creatinine kinase was normal. ANCA was normal. Electrocardiogram showed normal sinus rhythms without evidence of chamber hypertrophy. The patient underwent an echocardiogram to further characterize the enlarged cardiac silhouette identified on the chest X-ray. This revealed massive ectasia and aneurysmal dilatation of the right coronary artery, left main artery, left anterior descending artery, and circumflex arteries, as seen in . Left ventricular function was normal. The aortic arch was normal as were the proximal neck vessels.\nBecause of the dilated coronary aneurysms, the patient was diagnosed with KD. Despite lack of fever, given the evidence of ongoing inflammation and initial presence of bilateral nonsuppurative conjunctivitis, in addition to the coronary artery changes, the patient was treated with high-dose IVIG (2 g/kg) and started on daily low-dose aspirin. Low-molecular-weight heparin was started as antithrombotic therapy and once stabilized, daily atenolol was initiated. Activity was restricted as much as possible.\nInflammatory markers were followed. Platelets revealed a peak of 952 × 109/L and CRP a peak of 54.6 mg/L. After treatment, both platelet and CRP levels normalized.\nThe patient's neck pain and the limited range of movement resolved immediately after treatment, as did the bilateral conjunctivitis. The patient was stable and appeared well at time of discharge. His aspirin, low-molecular-weight heparin, and atenolol were continued. The CT angiogram performed after discharge revealed massively dilated and aneurysmal coronary arteries, as shown in .\nIn follow-up cardiology and rheumatology clinics, he has been doing well with no further neck pain or stiffness. He did not develop desquamation during follow-up, and the repeat echocardiogram one month after discharge was unchanged. He will continue long-term anticoagulation therapy with low-dose heparin with a target level greater than 0.5 IU/ml. He will also continue low dose aspirin and atenolol. His family was advised to have the annual influenza vaccine. |
An 18 year old woman was referred to our outpatient clinic of Endocrinology, University-Hospital of Naples Federico II because of hypothyroidism due to Hashimoto's thyroiditis. Blood samples showed high levels of thyroperoxidase and thyroglobulin antibodies and normal calcitonin serum levels. The patient was euthyroid with normal serum levels of thyroid-stimulating hormone (TSH), free triiodothyronine (FT3), and free thyroxine (FT4) during replacement therapy with L-T4. At physical examination, a palpable nodule of ~2 cm in size was detected in the isthmus of the thyroid. There were no palpable cervical lymph-nodes. An US evaluation confirmed an isolated lesion located in the isthmus, showing an isoechoic solid nodule with smooth margins; its size was 18 × 13 × 6 mm with intra and perilesional vascularity (Figure ). Therefore, a FNA was performed and cytological results revealed a TIR3A lesion. The cytological specimen showed an increased cellularity with some microfollicular structures in the background of scant colloid (Figure ). Thus, we assessed the risk factors associated with the isolated TIR 3A nodule of our patient. According to the ATA guidelines we repeated the FNA which confirmed the same result (TIR3A). The second US (after 6 months) showed that there were no clear signs suggesting malignancy such as microcalcifications or taller than wide-shaped nodules. However, we found a small hypoechoic cranial component in the nodule with blurred margins and elastography revealed an increased stiffness in this cranial component. No nodules were detected in the contralateral lobes by US; cervical lymph nodes were normal. Among the possible risk factors, our patient referred a familial history of thyroid cancer. Her mother was submitted to total thyroidectomy for a follicular variant of PTC twenty years ago; our subsequent evaluation showed that she was disease free at the moment.\nOn this basis, we decided that a surgical treatment was indicated for our patient and assessed the risk/benefit of total thyroidectomy vs. isthmusectomy. |
A 55-year-old male arrived at the ER, a large tree having fallen across his pelvis. The patient was hypotensive at the scene. He received over 1500 ml of crystalloid in route to the emergency room. Upon arrival to St. Mary’s Medical Center, the patient had a systolic pressure of 70. His heart rate was in the 130 s. The patient was awake and alert. A left subclavian central line was placed and he was transfused with two units of O-blood. The patient’s abdomen was soft and nontender. The patient had blood at his meatus. The patient’s pelvis was tender to palpation. His left leg was cold, mottled and pulseless. There was no femoral pulse on the left side. The chest x-ray was normal, however, the pelvis film revealed extreme widening and displacement of the symphysis pubis (Fig. ). A bedsheet was wrapped around the patient’s pelvis and another two units of O-packed red blood cells were administered. Additionally, he was given four units of fresh frozen plasma. The patient was started on norepinephrine to control his persistent hypotension. His systolic blood pressure rose into the mid-90s and the patient was taken to the CT scanner. The CT scan revealed that the patient had an acute occlusion of his left iliac artery, as well as a large left iliac wing fracture with a massive associated hematoma. A right iliac wing fracture was located near the sacroiliac joint. There was pubic diastasis, as well as a left superior rami fracture with a modest associated hematoma. There was no extravasation of dye (Figs and ). The patient was taken directly to the operating room because he was developing significant abdominal distention in the face of continued hypotension. Anesthesiologists placed a second large-bore central line for fluid resuscitation. An exploratory laparotomy was performed, revealing an intraperitoneal bladder rupture. The patient was bleeding freely from his pelvic hematoma. Multiple attempts were made to pack the pelvis tightly with laparotomy pads, but efforts were unsuccessful. Because of the distorted anatomy obtaining proximal and distal control of the iliacs was extremely difficult. The patient suffered massive retroperitoneal venous bleeding, which we were unable to control. The patient exsanguinated on the operating room table; despite having received over 30 units of packed red blood cells, 30 units of fresh frozen plasma, several 10 packs of platelets, cryoprecipitate and factor VII A. |
A 29-year-old man presented to our department with the chief complaints of dysfunction of elbow flexion, wrist extension, and finger extension. He was right-handed. One year earlier, his right limb was caught in machinery. The forearm was pulled forward by a conveyor belt, and his distal clavicle hit a metal pole. Since rupture of the right axiallary artery and pectoralis major muscle and fractures of the clavicle and scapula were diagnosed, revascularization and open reduction with internal fixation of the clavicle were performed immediately. However, the right upper limb palsy remained.\nThe skin scar was on the anterior side of the shoulder from the coracoid process to the axilla. The British Medical Research Council (BMRC) grading scale was 1 for biceps, 3 for triceps, 1 for brachioradialis, 1 for wrist extensors, 1 for finger extensors, and 5 for deltoid. Active range of motion (ROM) of the right shoulder was nearly full flexion and abduction (, which displays active motion of the shoulder, elbow, wrist, and finger before surgery). (, which displays active motion of the elbow, wrist, and fingers at one and a half years after the LD transfer).\nOn MR imaging, no pseudomeningocele was observed. Biceps and brachialis showed denervation-re-innervation changes on electromyography.\nBased on these findings, it was concluded that the cause of the palsy, which involved dysfunction of elbow flexion, as well as wrist and finger extension, was a terminal nerve level injury of the brachial plexus, especially the musculocutaneous nerve and part of the radial nerve. Since electromyography of the LD and the muscles innervated by the median nerve showed a normal pattern, transfer of LD for elbow flexion and subsequent tendon transfer for wrist and finger extension were scheduled. Intraoperative exploration using a nerve stimulator was not planned.\nBipolar transfer of LD for reconstruction of elbow flexion was performed based on the method of Zancoli et al. With the patient in the left-lateral position, disinfection with povidone-iodine solution was carried out. After performing a right anterior longitudinal humerus incision, the coracoid process and insertion of the biceps were exposed. The LD flap was designed with a size of 9 cm × 30 cm and elevated as a pedicled myocutaneous flap (Fig. ). After passing the insertion of LD (humeral end) deep to the pectoralis major tendon, this end of LD was fixed to the coracoid process with a suture anchor, and additional suture was performed for reinforcement. After trimming of the LD origin (caudal end), the flap length was adjusted so that the elbow joint remained spontaneously at 100 degrees with supination of the wrist. The insertion of the biceps was wrapped by the origin of LD and sutured. Four drains were placed to manage the dead spaces, and the incision was closed (Fig. ). The elbow was fixed with a 2-mm Kirschner wire penetrating the ulna and humerus at 90 degrees, followed by splint fixation.\nAfter 3 weeks, the Kirschner wire was removed, and active and passive ROM training for the elbow were started. Active elbow flexion was 130 degrees after 5 months.\nTendon transfer based on the method of Riordan with a slight modification was performed for the wrist and finger reconstruction as secondary surgery 5 months after primary surgery.\nAfter splint fixation in the extension position of the wrist and finger for 3 weeks, active and passive ROM training began.\nOne and a half years after the primary surgery, the BMRC grade was 4 for elbow flexion, 4 for wrist extension, and 4 for finger extension. The active ROM of the right elbow was 0/135 (, which displays active motion of the elbow, wrist, and fingers at one and a half years after the LD transfer). The disabilities of the arm, shoulder, and hand score was 26. |
A 27-year-old female (46,XX) patient with normal cognition visited the Endocrinology Clinic of 1st affiliated hospital of China Medical University (Shenyang) for amenorrhea with an unknown etiology. The patient had labial fusion when she was born and underwent plastic surgery afterward. During puberty, the patient underwent another surgery for an ovarian cyst. The patient was treated in another hospital as PCOS for several times resulting with mild remission of amenorrhea and recurrence of ovarian cysts. The patient was suspected of having 21-OHD due to increased 17OHP levels and came to our hospital for a genetic test for CYP21A2 mutations. The patient did not have fatigue, loss of appetite or other symptoms and was the only child in the family. Her parents appeared to be normal. She was born by vaginal delivery, and the mother had no complaints of abnormal manifestations during the perinatal period or history of reproductive system diseases. Her parents are nonconsanguineous. Physical examination of the patient revealed the following characteristics: a height of 165 cm and weight of 55 kg; no hyperandrogenism symptoms like hirsutism or acne; and no purple stripes. The patient complained of difficulty of bending the metacarpophalangeal joints from childhood (Fig. ). There were no obvious abnormalities in the genitals.\nThe ultrasound showed the presence of a 1.2 × 1.4 cm fluid area in the left ovary, and there was a 9.5 × 6.3 × 4.3 cm cyst in the right ovary behind the uterus. Digital radiography (DR) suggested there were no bone abnormalities (Fig. ). The adrenal CT scan showed there were no obvious hyperplasia changes (Fig. ). The laboratory test results of the patient and her parents are shown in Table . The levels of progesterone and 17-hydroxy progesterone of the patient were significantly increased. Androgen levels were not increased, and the basal level of cortisol in the morning was within the lower limit of the normal level. Cortisol was stimulated by the ACTH stimulation test. There were no abnormalities in the other measurements or in those of the parents.\nCYP21A2 (NM_000500) was tested first due to the suspicion of 21-OHD, but no mutations were found in the patient. Then the patient’s complaint about a mild difficulty of bending the metacarpophalangeal joints reminded us to consider PORD, which usually presents with skeletal deformities and sexual dysfunction, so POR (NM_000941) gene was tested secondly. Exon 11 of PORharbored a homozygous mutation (c.1370G > A) which leads to a conversion of arginine at amino acid position 457 to histidine (R457H). The patient’s parents were both heterozygous carriers of this variant (Fig. ). Since the disease-causing homozygous mutations in POR gene were found, no further genetic analysis was performed.\nOur patient once underwent surgery to remove a large ovarian cyst; however, since the diagnosis was unclear and hormone replacement therapy was not given, the ovarian cyst soon recurred. The patient was given low-dose corticosteroids twice a day (hydrocortisone 5 mg in the morning and 2.5 mg in the afternoon) and estrogen/progesterone sequential therapy in our hospital, and her ovarian cysts gradually decreased in size (the biggest ovarian cyst shrank to 3.5 × 3.3 × 2.3 cm) with regular menstruation in the following visits. The serum levels of ACTH, LH, 17OHP and P were declined accordingly (Table ). No adverse effect of corticosteroids was found in the follow-up. |
A 36-year-old Chinese woman was hospitalized on 28 December 2018 with a complaint of anal pressing pain and an anal mass. Anorectal physical examination showed no redness or ulcers in the perianal skin. Preoperative digital anal examination showed moderate anal tightness, a smooth rectum wall, and no swelling or mass. There were also no significant changes in the rectal wall when the mass was pulled. The perianal skin and lower rectal mucosa were smooth, and no ulcers, fistulas, or purulent secretion were found under anoscopy. The tumor was palpable 6 cm from the anal margin at a 7 o'clock direction, with a range of about 5 cm × 5 cm (). Perianal ultrasound suggested an inflammatory mass, with no significant relationship between the mass and the rectum (). The clinical diagnosis was a perianal mass for further examination. The decision was made to perform excision and biopsy. Under general anesthesia, the perianal area was prepped and draped in a sterile manner and the anal mass was identified. Local anesthesia with 25% macaine was injected into the skin around the mass, and an elliptical incision was made around the mass, about 0.5 cm from the edge of the mass. The subcutaneous tissue was taken down by electrocautery. During surgery, the tumor was found to be spreading subcutaneously, with unclear boundaries. It had a hard texture, no capsule, and no fluctuation (indicating a solid mass) and was sent for pathology. The skin was closed using interrupted subcuticular stitches and Monocryl.\nGross examination showed a gray-yellow mass measuring 4 cm × 3 cm × 3 cm (). Hematoxylin and eosin staining revealed nests of mononuclear cells with abundant eosinophilic granular cytoplasm, small central nucleoli, and a small number of vascular structures in the tumor tissues, with no hemorrhage or necrosis. Immunohistochemical examination showed notable and strong positivity for NSE, vimentin, and S-100. The immunostaining pattern also showed slight positive expression of Ki-67. However, cytokeratin AE1/AE3, epithelial membrane antigen, synuclein, chromogranin A (CgA), CD34, smooth muscle actin, desmin, and neurofilament proteins were all negative (). These findings were compatible with a GCT. The patient was followed-up for 19 months after the operation, with no recurrence to date.\nThe study was approved by the ethics committee of Beijing Dongfang Hospital and conducted in accordance with the provisions of the Declaration of Helsinki. All patient details have been de-identified, and the patient provided written informed consent for treatment and publication of this report. |
A 52-year-old multiparous Japanese woman was referred to our hospital with a complaint of a feeling of abdominal fullness. She had uterine leiomyoma that had been observed for 9 years, and a history of chronic thyroiditis. Aside from the earlier observed leiomyoma, a previously unnoticed soft mass at the uterine cervix was palpable on pelvic examination. T1-weighted magnetic resonance imaging with fat suppression revealed a swollen uterine corpus with leiomyoma, and suggested a uterine cervical lesion with a low signal intensity (Fig. ). Circulating levels of CA 125 and CA 19–9 were within the reference range at 16.3 and 11 U/mL, respectively. Hysterectomy was performed with a clinical diagnosis of multiple leiomyomas and an unknown cervical tumor. Postoperatively, the patient underwent adjuvant chemotherapy (gemcitabine plus docetaxel) and did well for the next 3 months, with neither local recurrence nor distant metastasis on chest and abdominal computed tomography imaging.\nThe enlarged uterus was 8 × 14 × 8.5 cm in size. Several elastic, hard, whitish masses were found in the uterine corpus, consistent with leiomyoma. In addition, an elastic, soft gray-white hemorrhagic mass measuring 8 × 6 × 5 cm was observed at the anterior wall of the uterine cervix (Fig. ).\nHistologically, the cervical mass was below the squamous epithelium and consisted of a nest-like proliferation of less cohesive, epithelioid tumor cells that had rounded nuclei and eosinophilic cytoplasm. The tumor cells had no marked nuclear pleomorphism and prominent nucleoli, and a coarse chromatin pattern was found in the nucleus. Mitotic figures were moderately frequent (4 per high-power field) but atypical mitoses were not present (Fig. ). The tumor showed a confluent growth pattern and individual nests of tumor cells were compartmentalized by intricate vasculature (Fig. ). There was no apparent involvement of myometrial smooth muscle cells. Foci of extensive necrosis and hemorrhage were found in some areas. A low-grade ESS component, showing uniform cells with round to spindle-shaped nuclei, which were whorled around arteriole-type vessels, was not coexistent in the lesion.\nImmunohistochemically, the tumor cells were diffusely positive for vimentin (clone V9, DAKO, Glostrup, Denmark), α-smooth muscle actin (αSMA; clone 1A4, DAKO; Fig. ), muscle-specific actin (clone HHF-35, DAKO), and heavy caldesmon (clone h-CD, DAKO), with moderate positivity for c-kit (DAKO; Fig. ), estrogen receptor (ER; clone SP1, Ventana, Tucson, AZ; Fig. ), and progesterone receptor (PgR; clone 1E2, Ventana). In contrast, the cells were negative for pan-cytokeratin (clone AE1/AE3, DAKO), desmin (clone D33, DAKO), CD10 (clone 56C6, Novocastra, Newcastle upon Tyne, United Kingdom), CD34 (clone QBEnd10, DAKO), p16 (clone E6H4, Roche, Basel, Switzerland), DOG1 (clone K9, Leica Biosystems, Wetzlar, Germany) and cyclin D1 (Biocare, Concord, CA). The Ki-67 (clone 30–9, Roche) labeling index of the neoplastic cells was 19.5%. These histological and immunohistochemical findings suggested a differential diagnosis of non-epithelial tumor of the uterus, particularly high-grade ESS, epithelioid leiomyosarcoma, or uterine GIST.\nFISH was performed for further molecular biological observations. To detect YWHAE rearrangement derived from chromosomal translocation of t(10;17)(q22;p13)—we used a custom dual-color, split-signal YWHAE probe set for the YWHAE locus on chromosome 17p13 (Chromosome Science Lab, Inc., Sapporo, Japan). The probe set consisted of a 333-kilobase (kb) sequence labeled with SpectrumGreen (telomeric [RP11-143 L7 and RP11-22G12]) and a 303-kb sequence labeled with SpectrumOrange (centromeric [RP11-100 F18 and RP11-60C18]). Rearrangements in the NUTM2A and NUTM2B genes at 10q23 and 10q22, respectively, were characterized using a custom break-apart probe design. The probes were synthesized using oligo-based SureFISH technology (Agilent Technologies, Santa Clara, CA) and labeled with FITC (5’ probe) and Cy3 (3′ probe) fluorophores. The analysis was performed with formalin-fixed, paraffin-embedded specimens sectioned into 3-μm-thick slices, as described previously []. The split-signal rate of YWHAE, NUTM2A and NUTM2B was 18%, (Fig. ), 0 and 4%, respectively, when 50 nuclei were counted. In contrast, a JAZF1 split, which is a major gene translocation in low-grade ESS, [] was not detected.\nTo investigate KIT and PDGFRA gene mutations, direct sequence analysis was performed. Exons 9, 11, and 13 of the KIT gene and exon 18 of the PDGFRA gene were amplified by polymerase chain reaction using the primers KITexon9F: GGC TTT TGT TTT CTT CCC TTT A, KITexon9R: ATG GTA GAC AGA GCC TAA ACA, KITexon11F: GAT CTA TTT TTC CCT TTC TCC C, KITexon11R: AGC CCC TGT TTC ATA CTG AC, KITexon13F: GCT TGA CAT CAG TTT GCC AG, KITexon13R: GCA GCT TGG ACA CGG CTT T, PDGFRAexon18F: CAG ATG GCT TGA TCC TGA GT, and PDGFRAexon18R: GAG GAT GAG CCT GTC CAG T. The amplified products were purified and sequenced. The sequences of KIT exons 9, 11, and 13 were wild type. In PDGFRA exon 18, substitution of nucleotide C to T, known as a single-nucleotide variant (rs2228230) was detected in one allele. The variant does not cause amino acid substitution. Thus, it was considered that there was no oncogenic mutation in KIT and PDGFRA genes in this tumor. |
A 66-year-old female patient first presented to our hospital in 2005 with lower abdominal pain and macroscopic hematuria. The patient was diagnosed with cervical cancer (FIGO stage IVa), and histological analysis determined squamous cell carcinoma. Routine computed tomography (CT) did not detect any apparent lymph node or distant metastases. Primary concurrent chemoradiotherapy (CCRT) consisting of whole-pelvis and paraaortic external beam radiotherapy of 50 and 45 Gy/25 fractions, respectively, was conducted with high-dose-rate intracavitary brachytherapy consisting of 25 Gy/5 fractions. The chemotherapy protocol consisted of intrauterine arterial administration of cisplatin (70 mg/m2) every 3 weeks but was only conducted once due to the development of grade 4 neutropenia. Weekly intravenous (IV) administration of cisplatin with radiotherapy had been the standard mode of administration for several years, because there were no randomized trials that supported the superiority of intra-arterial (IA) infusion of cisplatin with radiation. However, with regard to the primary treatment of stage IVa cervical cancer, IA infusion of cisplatin, followed by radical hysterectomy for local control, DFS, and overall survival (OS), was superior to IV administration in the neoadjuvant setting of our series of 3 phase II clinical trials. This infusion technique was also examined at another institution during CCRT; therefore, we adopted the IA infusion of cisplatin with radiotherapy in this case after obtaining written informed consent. Four months after completion of the primary treatment, the patient complained of slight right inguinal pain. Skeletal scintigraphy revealed multiple pelvic bone metastases (A) while magnetic resonance imaging (MRI) revealed a tumor that was not observed at the time of completion of the primary treatment (B). Laboratory tests showed elevated serum alkaline phosphatase (ALP) levels of 357 mIU/ml and N-telopeptide (NTx) levels of 26.7 nM BCE/L. As the pelvic metastatic lesions were located in the previous radiation field, concurrent chemotherapy with BP was conducted. The chemotherapy regimen consisted of intravenous nedaplatin, a cisplatin analogue, at a dose of 80 mg/m2 every month for 6 cycles. Because zoledronic acid (ZOL) was not available in Japan at the time, weekly intravenous treatment with pamidronate (15 mg) was administered for 12 months beginning on the first day of nedaplatin treatment. The right inguinal pain was relieved 2 months after the initiation of the treatment. Follow-up skeletal scintigraphy showed no abnormal lesions, and the serum levels of ALP and NTx declined to the normal range 41 months after the last administration of pamidronate (B). The patient is currently alive without any recurrence 89 months after the first diagnosis of bone metastases. |
Medical history and demographics
A 30-year-old man was referred to the endocrine outpatient clinic to initiate testosterone replacement in view of worsening symptoms of tiredness, lethargy and erectile dysfunction. He was diagnosed with Klinefelter syndrome during childhood but had not had any testosterone replacement for up to 10 years due to poor adherence to treatment. He had a medical history of mild asthma. He did not have cryptorchidism in childhood. He was a smoker and occasional drinker. He had not fathered a child before. He was not married but had a partner and both expressed their desire to have children in the future. He had no family history of testicular pathology.
On physical examination, he had gynecomastia and sparse body hair over his arms and legs. However, he had normal pubic hair and normal penile size. His both testes were very hard with irregular surfaces: the right testes measuring 25 ml in size and the left testes measuring 8-10 ml in size. He weighed 83.6 kg with a height of 1.93 m (BMI 22.4).
Investigations
His laboratory tests showed very low serum testosterone levels, elevated luteinizing hormone and follicle stimulating hormone levels, which were consistent with primary gonadal failure (hypergonadotropic hypogonadism). Testicular tumor markers such as alpha-fetoprotein and serum beta human chorionic gonadotropin (beta HCG) were normal. Thyroid function test, renal function test, liver function test, prostate specific antigen, vitamin D, calcium and prolactin levels were all within normal limits (Table ).
An ultrasound scan of his testes demonstrated bilateral solid testicular masses with no blood flow seen within the lesions. There was very little identifiable normal testicular tissue seen within the right testis, which was replaced by a large mass measuring 3.4 cm x 2.7 cm x 2.3 cm. The left testis contained an echogenic, well defined mass measuring 0.9 cm x 0.65 cm x 0.5 cm (Fig |
The patient was a 41-year-old female administrative assistant with a history of coldness and pain in the right upper limb with onset 1 year previously and progressive deterioration. Complaints worsened with movement of the upper limb, primarily abduction, limiting the patient’s activities. She reported no comorbidities, local trauma, or smoking. On physical examination, the right hand was pale and cold and capillary refill time was elevated. The right brachial pulse was weaker than its contralateral counterpart and right radial and ulnar pulses were absent, with biphasic flow on duplex imaging. The brachial-brachial index was 0.64. The contralateral upper limb had strong pulses. There was no murmur in the right supraclavicular region. Adson, hyperabduction (), and costoclavicular postural maneuvers were positive. The Ross maneuver was negative. There were no neurological changes or muscular atrophy. X-rays of the chest and cervical spine showed a cervical rib joined to the first rib on the right (). Computed tomography angiography of the chest with hyperabduction of the upper limbs showed compression of the right subclavian artery by the cervical rib and thickening of the artery wall, without significant stenosis. There was no aneurysm (). Having diagnosed aTOS, surgical decompression was indicated.\nUnder general anesthesia, a right supraclavicular approach was obtained, with anterior and medial scalenectomy, followed by total resection of the cervical rib and partial resection of the first rib. The subclavian artery wall was slightly thickened, although without reducing pulsation (). Immediately after decompression, the patient’s right brachial pulse was strong and unchanged by abduction of the upper limb. Since the structural change to the artery was discrete and without effect on pulsation, we decided not to resect the injured segment. The patient’s recovery was uneventful and she was prescribed analgesics and motor physiotherapy. She was discharged on the fourth postoperative day. At the 30-day follow-up consultation, she reported no limitation of activities. Her capillary refill times and brachial pulses were symmetrical. Postural maneuvers were negative and her brachial-brachial index was 0.91. She had no wound complications and no motor disorders in the right upper limb. She described mild paresthesia in the fingertips, with progressive improvement after motor physiotherapy. An ultrasound scan at 3 months showed good flow through the right subclavian artery. |
A 53-year-old Caucasian British, right-hand dominant woman was involved in a road traffic accident while driving a car, involving a head-on collision with another vehicle at approximately 30 miles/hour. She sustained a closed injury to the left forearm against the steering wheel, resulting in obvious clinical deformity. No neurovascular deficit was evident.\nRadiographs revealed a displaced and angulated trifocal fracture of the radial shaft in combination with a displaced two-part mid-shaft ulna fracture (Figure ). Within 24 hours an open reduction and internal fixation of the fracture was performed.\nUnder general anesthesia, using a direct subcutaneous approach to the ulna, the ulna was reduced and fixed with a seven-hole titanium dynamic compression plate (DCP; Figure ); 1 mm compression was applied.\nThe radius was exposed using Henry's approach. The distal radius fracture was fixed using a five-hole titanium DCP while applying 1 mm compression. The proximal three radius fragments were fixed with a nine-hole DCP (Figure ). Careful handling of the soft tissues was paramount and extra care was taken to avoid devascularising any of the bone fracture segments. We also applied 1 mm compression to the proximal fracture. The middle fracture was bridged because of inherent comminution.\nAfter wound closure, an above-elbow back slab was applied with the elbow held in 90 degrees of flexion. The forearm was held elevated in a sling and our patient was monitored for signs of compartment syndrome. Our patient was discharged from hospital after 48 hours of observation in a broad arm sling; there were no immediate post-operative complications.\nAfter two weeks, the back slab and the skin staples were removed. There was no neurovascular deficit; only a minor but improving subjective altered sensation over the dorsal first web space. The range of active supination was slightly reduced, but otherwise a good range of movement was demonstrated. Our patient was left free of a cast and advised to mobilize her forearm.\nAt six weeks follow-up, our patient showed further functional improvement. A weakened power grip was noted and physiotherapy initiated. Results as seen on radiographs were satisfactory.\nAfter three months, our patient returned to work as a cashier. She was pain free but reported a weakness in the left forearm and occasional paresthesia over the dorsal first web space.\nOur patient completed the Disabilities of the Arm, Shoulder and Hand (DASH) questionnaire and scored 49.1 (measures scaled on a zero to 100 scale: a higher score indicates greater disability). She was finding lifting tasks difficult and did not yet feel able to drive. She had good and equal active and passive range of movement of both wrist and elbow. Grip and pincer strength were measured and values revealed an objective weakness on the left, although this was confounded by dominant limb strength variation.\nOur patient's final review took place six months after the initial injury. She had made a complete functional recovery with a full range of movement of elbow and wrist joints, equal on both sides. The altered sensation over the first dorsal web space of the left hand had continued to improve over time. She had resumed driving, remained pain free and her grip strength had been restored.\nRadiographs revealed that the fractures had united (Figure ) and our patient was subsequently discharged. No plan was made to remove the plates in the future. |
A 64-year-old man with a history of smoking and rheumatoid arthritis (RA) presented with left hemiparesis, slurred speech, and left facial droop. His NIHSS score was 9. Initial CTA showed occlusion of the extracranial right ICA and a tandem occlusion of the right MCA (Figure ).\ntPA was administered intravenously, and the patient was transferred to the interventional neuroradiology suite. Biplane digital subtraction angiography revealed a complete occlusion of the cervical right ICA at the vessel’s origin due to extensive atherosclerotic disease (Figure ).\nThe right MCA and right anterior cerebral arteries (ACAs) could not be visualized, and multifocal embolic filling defects were detected within the cavernous and supraclinoid ICA segments on microcatheter contrast injection past the carotid origin occlusion (Figure ).\nGiven the previous administration of tPA, a decision was made to attempt balloon angioplasty without carotid stenting of the extracranial ICA. A 6 mm x 20 mm noncompliant balloon was positioned across the occluded proximal ICA and inflated until adequate luminal patency was restored. The thromboemboli in the supraclinoid ICA and MCA were subsequently removed by suction aspiration using an ACE 68 reperfusion catheter (Penumbra, Inc., Alameda, CA) and a direct aspiration first pass (ADAPT) technique []. Post-procedural right common carotid angiography demonstrated complete recanalization of the right middle cerebral and right anterior cerebral arteries without angiographic evidence of complicating features (Figure ).\nAngiography of the right common carotid artery demonstrated approximately a 40% residual stenosis at the ICA origin with no delay in the transit of contrast into the intracranial vasculature. A thrombolysis in cerebral infarction (TICI) score of three was achieved at the conclusion of the operation. Subsequent head computed tomography (CT) and magnetic resonance imaging (MRI) of the brain showed scattered infarcts in the right middle cerebral artery territory with multifocal petechial hemorrhages without frank hematoma. Compared to his initial NIHSS score of nine at presentation, he was discharged home three days later with an NIHSS of zero and no residual neurological deficits. |
In late 2016, a 61 year old non-smoking female presented with exertional shortness of breath, mild pedal edema, distended neck veins, and a recent weight gain of 15 lbs over the prior 2 weeks. It was initially suspected that the patient was experiencing pulmonary embolism based on her clinical presentation and elevated D-dimer, but further testing revealed that she was experiencing cardiac tamponade with mild pulmonary hypertension and heart failure. Pericardiocentesis was performed without definitive diagnosis. In February 2017, the patient exhibited worsening respiratory symptoms without fever, hemoptysis, sputum production, B symptoms, or extremity edema. Baseline chest PET-CT images were reviewed by radiologists at both UCLA Oncology and Texas Tech University Health Sciences Center, demonstrating a large middle mediastinal mass encasing the main pulmonary artery, with pericardial and left pleural effusion (Figure & Figure ). Scattered pulmonary nodules and hypodense lesions were identified in the right lobe of the liver, consistent with a diagnosis of metastatic disease (Figure ). Biopsy of the mediastinal mass revealed a high grade undifferentiated malignant neoplasm composed of highly proliferative (ki67 staining ~50%) pleomorphic anaplastic epithelioid malignant cells with large areas of necrosis and fibrosis. Immunohistochemistry revealed strong antigenicity for CD31 and CD34, and weak antigenicity for D2-40 and Factor VIII indicating a diagnosis of angiosarcoma that was corroborated at both UC San Diego Health and MD Anderson.\nConventional treatment options were recommended, however the patient declined these based on low reported survival rates, and instead, requested the non-selective beta blocker propranolol as a single agent therapy. In May 2017, 40 mg/kg propranolol was administered daily and PET-CT scans were performed at regular intervals to assess the response of the tumor to propranolol. Assessment of tumor response was based on 18F-fluorodeoxyglucose (FDG) tracer uptake and measurements/assessments of the primary tumor and distant metastases. After 12 months of propranolol as a single agent therapy, significant debulking and decreased size of the residual mediastinal mass was observed on PET-CT scans, with resolution of pericardial effusion (Figure & Figure ). Pulmonary nodules were stable to regressed, and the nodules in the right lobe of the liver had completely resolved (Figure ). There was no evidence of residual hyper-metabolic activity based on FDG measurements in the primary lesion or in metastatic sites in the chest, abdomen, or pelvis on PET-CT. |
A 73-year-old man with diabetes mellitus, hypertension and hypercholesterolemia, and a previous history of coronary artery bypass surgery presented for evaluation of dizziness and episodes of syncope. Bilateral cervical bruits, a difference in blood pressure 45–50 mmHg between both arms, as well as barely discernable radial, ulnar and branchial arterial pulses on the right upper limb prompted color duplex which revealed bilateral internal carotid artery stenosis of 75% at each side, retrograde flow in his right vertebral artery (subclavian-vertebral steal) and retrograde flow in his right carotid artery during the midsystolic phase of the cardiac cycle (subclavian-carotid steal), while an antegrade blood flow was seen during the rest of the cardiac cycle (Figure ). Further investigation with selective catheterization of the origin of the right subclavian artery and digital subtraction angiography demonstrated occlusion of the innominate artery (Figure ) while catheterization of the left subclavian artery confirmed the subclavian-vertebral steal phenomenon on the right side (Figure ). Due to bilateral external and common-external (on the left) iliac artery occlusions (the patient was a 200 meters claudicant), we had to perform upper extremity catheterization and full aortic arch aortography. Initially we performed a left brachial catheterization but we did not pass the aortic arch due to a dissecting plaque at the origin of the left subclavian artery (< 50%). We only performed selective catheterization of the left subclavian artery (Figure ). Catheterization through the impalpable right brachial artery was the option for catheterization of the right subclavian artery, the wire passed the innominate lesion (possibly through) the subendothelial route. Clinical neurologic evaluation and brain computed tomography (CT) scan did not reveal focal ischemic brain lesions, while no myocardial ischemic symptoms and an ejection fraction of 40% was seen after regular post-coronary artery bypass grafting (using saphenous veins) (CABG) follow-up. We had to provide the most safe and effective clinical solution to this 73-year-old man with very extensive and severe arterial disease and a previous CABG. Permanent dizziness and many episodes of syncope with acceptable cardiac function was the indication for doing something invasively. The priorities for the patient were: a) reestablishment of antegrade arterial flow through the right carotid and right vertebral arteries; b) treatment of the bilateral internal carotid artery stenosis; c) treatment of the left subclavian dissecting lesion; and d) treatment of bilateral iliac artery occlusions.\nThe operation was conducted under general anesthesia and included a staged bilateral approach with a left cervical incision along the anterior border of the left sternocleidomastoid muscle employed to expose the left common carotid artery and to perform the endarterectomy with a Dacron patch angioplasty and the proximal end to side polytetrafluoroethylene (PTFE) graft-arterial anastomosis with the use of a shunt, while a limited right cervical incision was used to expose the origin of the right common carotid artery and to perform the peripheral end to side graft-arterial anastomosis passing anteriorly to the trachea under the staple muscles. We preferred left carotid to right carotid bypass instead of left subclavian to right carotid bypass, to avoid double length of graft and dysfunction in neck movements, besides in our case left subclavian had an ostial < 50% stenosis. Concerning the internal carotid stenosis, we treated the left one since it was more ulcered and our policy is to treat the left lesion first and later the right one, after close follow-up. When performing treatment of the right internal carotid stenosis we tried an effective solution to its coiling. Endovascular treatment of the innominate occlusion was an option with specific disadvantages: a) the lesion could possibly be passed with a wire but then the angioplasty would be performed rather in a subendothelial route or, in an extremely eccentric way in a vessel which is known to be fragile and the patient having had a previous sternotomy, rendering the emergent operation in case of rupture, a catastrophe; b) the possibility of having an alternative second endovascular approach (second wire) through femoral or left subclavian arteries was deemed neither efficient nor safe; c) the feasibility of innominate endovascular angioplasty is known to have a high risk of distal embolization. In this case the operator had to put one or two filters for protection of embolization (carotid and subclavian); d) the insertion of a filter in the right common carotid, at least, was technically very difficult or unfeasible before innominate angioplasty, as the only route was the right subclavian and the angioplasty had to be done in a retrograde way in the innominate artery; and e) the medium and long term results of subclavian angioplasty are inferior to operative ones, especially for innominate occlusions or sub-occlusions and we are unaware of a series with medium or long term results.\nWe have an excellent series of 0% death-stroke rate of carotid endarterectomies (70 cases) and we have not yet adopted the questionable carotid angioplasty combined with stenting (CAS). The patient remains symptom free after one year of follow-up, while the right internal carotid artery stenosis remains stable. |
A 14 years old girl was brought to our pediatric emergency unit because of severe hypocromic microcytic anemia detected by her primary care physician who had required blood tests to investigate the cause of reported asthenia and weight loss. Her mother reported that the patient had experienced early satiety and abdominal pain for several months. No history of fever or trauma as well as no dysuria, gross hematuria or recent travel abroad. Reported regular menstrual cycles until 2 months before admission.\nWith regards to the past medical history, a neurosurgical intervention for cranial meningocele of the bregmatic region at the age of 6 years and laparoscopic cholecystectomy for gallstones at the age of 13 years have been reported. At the time of cholecystectomy, hemoglobin was normal as well as hemoglobin electrophoresis. Initially, parents denied that their daughter had any history of eating disorder such as PICA, though alopecia was reported.\nAt physical examination: anorexic habitus, pulse rate of 88 beats/min, respiratory rate of 28/min, very pale skin and mucosae, no evident bleeding nor icterus. Her abdomen was rigid under palpation with a non- compressible mass in the left upper quadrant.\nLaboratory tests, including erythrocyte morphology, confirmed a severe IDA, with normal Serum electrolytes, amylase, thyroid and liver function tests and vitamin B6, B12 and folate at the lower range of norm. Fecal occult blood test was positive.\nAbdominal ultrasound (US) showed perihepatic free fluid and mild splenomegaly (10.5 cm diameter) and computed tomography (CT) scan reported severe gastric dilatation due to organic matter accumulation. The mass completely filled the gastric fundus and antrum and extended into duodenum. Also, small bowel dilatation and air-fluid levels were present (Fig. ). A gastric bezoar was therefore suspected.\nAfter appropriate transfusion of erythrocytes and plasma, endoscopist evaluation was requested. In the light of the large size of the mass, it was thought that endoscopy could likely fail to remove the mass (Fig. ). Thus, the patient was referred for surgical evaluation. The patient underwent surgical intervention by means of a median sovraumbilical laparotomy and anterior gastrotomy; a 1360 g trichobezoar was extracted (Fig. ) and sent to histological examination which concluded for gastric trichobezoar, with vegetable fibers and amorphous matter.\nThe post-operative course was characterized by the onset of fever. A CT scan of the abdomen and thorax revealed perisplenic and pleural effusion with pulmonary consolidation which required a broad-spectrum antibiotic therapy and the abdominal placement of a drainage tube which was removed 6 days later. Feeding was gradually reintroduced, first by means of total parenteral nutrition and then orally with supplementation of iron, vitamin B complex, folate and other micronutrients. During this period, the patient was carefully monitored for post surgical complications.\nAt the time of hospital discharge, blood values were normalized and we observed an initial catch-up weight; pulmonary and abdominal US didn’t show any residual free fluid collection.\nThe patient underwent both psychiatric and psychological evaluations before discharge. Both concluded for PICA disorder, on the basis of history of reported and chronic ingestion of small stones which started during primary school attendance and subsequent hair ingestion that the patient was pleased to eat because she found them to be tasty. According to DSM-V (Diagnostic and Statystical Manual of Mental Disorders) [], PICA is characterized by an appetite for substances that are largely non-nutritive, such as ice (pagophagia), hair (trichophagia), paper (papyrophagia), drywall or paint, metal (metallophagia), stones (lithophagia) or earth (geophagia), glass (hyalophagia) or feces (coprophagia). To be considered as PICA, these actions must persist for more than 1 month at an age where eating such objects is considered developmentally inappropriate, not part of culturally sanctioned practice and sufficiently severe to warrant clinical attention. As our patient met all the diagnostic criteria, she has been included into our follow up program for patients affected by Eating Disorders in order to prevent possible recurrence of PICA. We didn’t report bezoar recurrence in our 2 years follow up. |
A 55-year-old female with a past medical history of cocaine use and hypertension was admitted for a large right basal ganglia hemorrhage with extension into all four ventricles requiring right decompressive craniotomy. Following a fever on day 23 of hospitalization, a head CT scan was obtained which revealed development of 3 new distinct parenchymal hemorrhages within the prior right cerebral hemorrhages. At this time, the patient's fever prompted initiation of empiric vancomycin and ceftazidime. On day 26 of hospitalization, the parenchymal hemorrhages were managed with neurosurgical debridement of the hemorrhagic brain with partial temporal lobectomy and duraplasty with titanium mesh placement since the patient failed to improve with medical management alone. A repeat head CT scan obtained on day 28 of hospitalization identified an 18 mm subgaleal fluid collection overlying the duraplasty noted in . Given the persistent fevers, the subgaleal fluid collection was aspirated on day 30 of hospitalization which subsequently grew 2+ Enterobacter cloacae complex susceptible to TMP-SMX, gentamicin, ciprofloxacin, and ertapenem. Resistance patterns of the patient-specific E. cloacae isolated are described in . Cell count of the cerebrospinal fluid (CSF) identified 17,300 nucleated cells/μL with 98% granulocytes, 35,000 red cells/μL, 980 mg/dL of protein, and an undetectable glucose level.\nGiven the susceptibilities, the patient was transitioned from vancomycin and ceftazidime to SMX-TMP (5 mg/kg of TMP) every 8 hours IV and metronidazole 500 mg IV every 12 hours. Since the patient continued to have persistent fevers, a repeat head CT scan was performed which identified an enlarging subgaleal fluid collection measuring 104 × 23 mm as noted in . The neurosurgical team believed the risk of removal of the titanium mesh was much greater than the benefit given her poor surgical candidacy attributed to comorbidities. Furthermore, a future procedure would be needed following removal. Repeat subgaleal collection aspirations performed on day 35 of hospitalization remained persistently positive for E. cloacae prompting placement of a lumbar drain. Daily intrathecal gentamicin at a dose of 4 mg was started at day 36 of hospitalization and was administered daily through the lumbar drain. A follow-up head CT scan was performed and revealed no significant change in the subgaleal abscess size. Given the lack of improvement on imaging, repeat cultures of the subgaleal fluid collections were obtained and remained positive for E. cloacae. Repeat CSF cell count revealed 139,000 nucleated cells/μL with 95% granulocytes, 351,000 red cells/μL, 1,910 mg/dL of protein, and an undetectable glucose level. Given the persistently positive cultures and worsening CSF profile, after 11 days of SMX-TMP and metronidazole, the patient was transitioned to ertapenem 1 g IV daily. A CSF culture obtained 3 days after the initiation of ertapenem was found to be sterile. CSF cell count from this specimen revealed improvement and identified 112 nucleated cells/μL with 83% granulocyte predominance, 7 red cells/μL, 449 mg/dL of protein, and a glucose level of 32 mg/dL. An MRI of the brain obtained 7 days after ertapenem was initiated revealed a decrease in the subgaleal fluid collection from 18 mm to 8 mm as noted in .\nOn day 50 of hospitalization, intrathecal gentamicin was stopped. The lumbar drain was removed after 2 weeks of therapy with ertapenem given achievement of a drainage rate of less than 10 mL/hour. After 16 days of ertapenem, the patient was transitioned to SMX-TMP for prolonged therapy given the presence of the titanium mesh. She was ultimately discharged to a rehabilitation facility after 77 days of hospitalization. |
A 36-year-old male presented to the outpatient department with a history of low backache for the past one year, associated with typical claudication symptoms, left-sided unilateral numbness, and paresthesia of the foot. He gave no history of radicular pain, but he complained of progressive reduction in claudication distance. He had experienced a sudden deterioration in gait along with urinary incontinence, following a trivial traumatic fall, one week before presentation. There was no history of constitutional features. On examination, he had a bilateral high-stepping gait due to foot drop. Symmetrical weakness of both the L4 and L5 roots (MRC grade 0/5) and partial weakness of the S1 root (MRC grade 3/5) was noted with nondermatomal sensory disturbances. Deep tendon reflexes of the lower limbs were absent bilaterally. Though anal tone was normal, saddle anesthesia was present. Postvoidal ultrasonogram of the urinary bladder revealed a residual urine volume of 250 ml, thus confirming a neurogenic bladder.\nPlain radiography of the lumbar spine did not show any gross feature of instability and was inconclusive (). Magnetic resonance imaging (MRI) of the lumbar spine revealed a solitary intraspinal posterior epidural lesion of 1.8 × 1.5 × 0.5 cm at the L2-L3 level with heterogeneous signal intensities and adjacent epidural fatty hypertrophy contributing to severe canal stenosis (3 mm). The cauda equina was severely compressed and was almost not visible (). Owing to the heterogeneous signal intensities, computerized topography (CT) was performed which revealed the presence of an osseous lesion attached to the right L2 inferior articular process causing severe secondary canal stenosis (). Considering the recent-onset neurological deficit, the patient was advised surgical decompression and excision biopsy at the earliest. The patient was positioned prone on a Relton Hall frame under general anesthesia. A standard midline posterior approach was employed and L2 and L3 lamina were exposed. The spinous process was removed and using a motorized burr, a rectangular trough was created surrounding the lesion. The lamina was thinned out using a burr to avoid further insult to the dural sac, and then using a Kerrison ronguer, laminectomy was completed all around the lesion under microscopic guidance (). A small osteotome was used to remove the attachment on the right side which required partial removal of the facet joint. The lesion was then held, and the adherent soft tissues beneath the lesion were removed, resulting in en bloc removal and complete decompression of the cauda equina. Fusion was performed, as the procedure involved partial facet joint resection. The lesion was sent for histopathological examination. There were no adverse events or postoperative complications. The patient was mobilized with bilateral orthoses, and bladder training was initiated.\nAt 4 weeks, there was an improvement in his urinary symptoms, and by 12 weeks partial neurological recovery (MRC grade 3/5 motor power) in bilateral L4 and L5 was observed. The S1 root power increased by one grade (MRC grade 4/5). His gait improved thereafter, and he was able to return to his normal activities by 6 months with further neurological improvement by 1 grade in all roots.\nThe gross specimen measured 2 cm × 1.5 cm × 1.5 cm. It was greyish white in color and firm to hard in consistency (). It had a well-defined capsule and had the feel of particulate materials on cut sections. Histopathological examination revealed the presence of bone, cartilage and ligamentous tissue, and zones abutting all these composed of mature adult-type encapsulated adipose tissue (Figures and ). This confirmed the diagnosis of benign osteolipoma. |
A 51-year-old male with a permanent IVC filter that had been inserted approximately 20 years ago when the patient developed a DVT during a hospitalization for severe non-ischaemic cardiomyopathy, was transferred to our medical intensive care unit for shock and acute renal failure. Following the IVC insertion, he had been treated with warfarin for one year and had been on anti-platelet therapy since.\nHe had been admitted to the hospital three days prior to transfer after presenting with progressive bilateral lower extremity pain and decreased sensation in his gluteal region. Acute bilateral DVTs involving the common femoral and popliteal veins were diagnosed. Over 48 h, despite receiving unfractionated heparin, he developed anuric renal failure and shock. Placement of a right internal jugular dialysis catheter was complicated by airway compromise due to a retropharyngeal haematoma necessitating endotracheal intubation. The heparin infusion was discontinued and the patient was transferred to our hospital.\nOn arrival, his mean arterial pressure was 71 (104/53) mmHg while on norepinephrine, vasopressin, and phenylephrine. Arterial blood gas analysis showed a pH of 7.06, partial pressure of carbon dioxide (PaCO2) of 28 mmHg, partial pressure of oxygen (PaO2) of 312 mmHg, and a lactate of 16 mmol/L. The platelet count was 31 K/μL. Examination was notable for tense bilateral lower extremity oedema. Dorsalis pedis pulses were detectable with Doppler ultrasound. An abdominal computed tomography (CT) showed dilation of the distal IVC suggesting thrombosis (Fig. A). Transthoracic echocardiography showed a 25% ejection fraction with no right ventricular dilation or strain. The IVC was collapsible proximal to the hepatic veins. Lower extremity ultrasound confirmed acute bilateral DVTs involving the external iliac and femoral veins. Laboratory evaluations excluded thrombophilia, heparin-induced thrombocytopenia and thrombotic thrombocytopenic purpura. Infusion of 5 L of isotonic fluid and continuous renal replacement therapy led to a reduction in the vasopressor requirement, a reduction in lactate to 2.0 mmol/L, and pH/PaCO2 normalization. However, the lower extremity oedema progressed with development of bullae and purple skin discolouration (Fig. B). Dorsalis pedis pulses became undetectable, consistent with compartment syndrome due to PCD.\nCatheter-directed thrombolysis, surgical thrombectomy, and fasciotomy were deemed to be contraindicated due to ongoing shock, severe cardiomyopathy, the retropharyngeal haematoma, and persistent thrombocytopenia thought to be the consequence of platelet consumption. Unfractionated heparin was restarted and, within 24 h, lower extremity pulses were again palpable. However, there was a progressive rise in creatinine phosphokinase to 44,000 IU/L and an increase in lactate to 5.8 mmol/L despite continued vasopressor support and continuous dialysis. His family decided to pursue palliation and withdrawal of life-supportive measures. Post-mortem examination confirmed an occluding thrombus at the level of the IVC filter with extension to the internal and external iliac veins (Fig A, B). The autopsy did not identify an underlying malignancy. |
The patient is a 50-year-old African American female with a history of bilateral breast reduction twelve years ago, iron deficiency anemia, and obesity, who presented to the surgeon's office complaining of tenderness of her right breast. The patient reported that recently she had been developing keloids along the scar of the right breast with some areas having a blue hue; her left breast was unremarkable. She noticed that after wearing a sports bra there was increased pressure and abrasions to the keloid, leading to cellulitis and edema. She was previously treated with two courses of antibiotics for what was presumed to be an infected keloidal scar of her right breast but with minimal improvement. On exam, she had a large 10 cm diameter keloidal region on the inferior and lateral aspect of the right breast with edema and cellulitis. The keloidal area had no palpable fluctuance; she exhibited no nipple discharge or palpable adenopathy of the right axilla ().\nThe patient had a benign-appearing mammogram 8 months prior, and all of her screening mammograms since her breast reduction have been without signs of malignancy. Another mammogram was ordered but was not performed due to patient discomfort. An ultrasound of the breast was preformed and suggested marked edema and skin thickening suggestive of infection but no definitive fluid collection or underlying suspicious mass was observed.\nThe patient underwent a right breast partial mastectomy for cosmesis and resection of the infected keloidal area. Intraoperatively, the mass was highly vascular, firm, but not fixed to the chest wall. Postoperatively, the pathology revealed a high-grade primary angiosarcoma of the breast with negative margins.\nPatient underwent a computed tomography of the chest, abdomen, and pelvis, which did not show any evidence of gross metastatic disease. The patient then underwent completion mastectomy and scheduled for adjuvant chemotherapy with combination gemcitabine and Taxotere, followed by radiation. |
A 14-year-old right-handed female fell from a 10th floor apartment, and presented to our emergency department with multiple trauma. She had no history of any previous illness or medication before the injury. She complained of pain in the abdomen, right elbow, pelvic area, and low back area. She underwent iliac artery embolization and exploratory laparotomy to repair the iliac artery and a liver laceration at another hospital, and was subsequently transferred to our emergency center for additional surgical intervention involving the pelvic bone, spine and anterior trans-olecranon fracture dislocation (). Fortunately, computed tomography (CT) confirmed that she had no brain injury and no systematic neurologic symptoms, although there was a chance fracture of the T10 vertebra. She was subjected twice to a closed reduction of the injured elbow joint, once at the first hospital, and then in our emergency room. According to the Mayo classification, the olecranon fracture was diagnosed as type IIIB. Sixteen days after the initial trauma, the anterior trans-olecranon fracture dislocation of the right elbow joint was treated by open reduction and internal fixation by applying a pre-contoured olecranon plate (Zimmer, Warsaw, IN, USA) using the posterior approach incised along the triceps, followed by application of a long arm splint ().\nAfter surgery, the elbow was immobilized in a long arm splint at 90 degrees of flexion and neutral rotation. Starting from the 3rd postoperative day, the patient was allowed gentle exercise of the right elbow joint, but developed aggravated pain during rehabilitation at 2 weeks post-surgery. The HO was first noted in radiographs, at 3 weeks. At 10 weeks post-surgery, she had increased difficulty in moving her right elbow joint, and had reduced range of motion. The size of HO had progressed and was clearly revealed in radiographs. Physical examination revealed total ankylosis of the right elbow at 45 degrees of flexion, at post-operative 6 months. However, since the radioulnar joint was intact, pronation and supination of the forearm showed the full range of joint motion. Standard anteroposterior and lateral radiographs of the affected elbow revealed an unusually large mass connecting the distal humerus to the ulna (, ). CT scan images revealed the progress of mineralization from the outer margins towards the center area (). After 6 months maturation of the heterotopic mass, surgical excision was carried out using the posterior approach through the previous incision scar. Skin, subcutaneous tissue, and deep fascia were incised in line with the skin incision. The ulnar nerve was identified and retracted. The extent of the mass was exposed adequately and was excised completely from the distal humerus and ulna (). Intraoperative elbow range of motion flexion 0 to 140 degree was checked.\nPostoperative radiographs showed no trace of HO (). One day after the operation, a 1-time dose of radiation therapy (800 cGy) was provided to prevent recurrence. Four days after surgery, the elbow range of motion exercise was started, as tolerated.\nAt 30 months after surgery, patient had no pain in the elbow; radiographs revealed no further development of the HO, and a residual flexion deformity of 10 degrees with flexion up to 145 degrees, and supination and pronation of the forearm were achieved with full range of motion (, ). The patient was permitted to return to play, without any restrictions.\nThe Patient was given an opportunity to review the manuscript and consented to its publication. |
A 71-year-old man presented with acute low back pain and radiating pain in the right leg, with a positive straight-leg raising sign at 30°. He had a history of minor trauma suggestive of a lumbar sprain. His symptoms had gradually worsened, and he was unable to walk due to aggravation of pain in the back and leg upon standing or walking. On manual muscle testing, muscle weakness was not detected in the extremities. His patellar tendon reflexes were normal. On neurological examination, L5 nerve root compression was identified. On plain lumbar spine radiographs, spinal abnormalities, except for spondylolisthesis, were difficult to detect. Slight disc space narrowing at L4-5 was noted in lateral view (). Computed tomography showed no definite bone abnormalities. MRI revealed disc herniation at both root exits at the L4-5 intervertebral disc level (). Based on these observations, we diagnosed the patient with spondylolisthesis and lumbar disc herniation at L4-5.\nIntraoperatively, disc sequestration was noted, and the herniated disc was found to be incarcerated to the adjacent conjoined L5 nerve roots. Upon further investigation, we discovered that the L5 nerve root originated from the caudal level of the L5 pedicle and was conjoined with the S1 nerve root (). The disc herniation at L4-5 migrated bilaterally and was found beneath the abnormal conjoined nerve roots. After removal of the disc herniation and unroofing of the nerve root, we did not observe any obvious mobility of the conjoined nerve root. After surgery, the patient's leg pain immediately disappeared and no muscle weakness was recorded. Upon retrograde imaging review, we observed several signs of conjugated nerve roots on routine MRI images (): the "sagittal shoulder sign," a vertical structure connecting two consecutive nerve roots and the overlying herniated disc on the parasagittal MRI which represents a combination of a protruded or extruded disc adjacent to a conjoined nerve root; "corner sign," asymmetric structure of the anterolateral corner of the dural sac with one side being angulated compared with the other; "fat crescent sign," the presence of extradural fat between the conjoined nerve root and the asymmetric dural sac; and "parallel sign," an unusual course of the entire nerve root at the disc level, running parallel to the disc plane. |
A 50-year-old male patient was a known case of diabetes mellitus and was being treated for that since 1989. He was; however, quite irregular with this treatment and his diabetes was often out of control.\nIn November 2005, he presented with complaints of fever, cough with expectoration and weakness. The fever was low grade and there was no history of hemoptysis, dysuria and hematuria. Routine laboratory investigations showed high White Blood Cell count which was 59,000/mm3, and there were premature leucocytes on peripheral smear. He also had elevated platelet count which was 726,000/mm3. His diabetes was out of control with FBS level 264 mg/dl and PPBS value 372 mg/dl.\nA provisional diagnosis of CML with uncontrolled diabetes mellitus was made and he was further investigated.\nHis routine urinalysis showed glycosuria. Renal Function Tests and Liver Function Tests were within normal limits. Test for HIV infection was non-reactive. Chest X-ray did not reveal any abnormality. Bone marrow aspiration study was consistent with the diagnosis of CML. Test for Philadelphia chromosome was not carried out.\nHe was given hydroxyurea 500 mg 3 times a day and subsequently the doses were titrated with reference to WBC count.\nDuring the follow-up that spanned over next several months, he experienced occasional seasonal attacks of respiratory infection. For this, he was treated with antibiotics and insulin which controlled the infection promptly.\nIn October 2010, he complained of occasional fever, cough with slight expectoration and loss of appetite, weakness and weight loss. He was diagnosed to have pulmonary tuberculosis on the basis of his clinical picture and positive TB ELISA test (IgM) for which he received anti tubercular treatment for next 10 months.\nIn February 2011, he complained of retrosternal burning sensation while walking which was relieved by rest. He also experienced tightness around the chest. The pain did not radiate but was associated with the mild breathlessness. ECG did not reveal any abnormality. The Treadmill Test was strongly positive for exercise induced coronary insufficiency.\nAs his symptoms and stress test suggested coronary heart disease, he was referred to a cardiac center for further evaluation. 2D-echo was carried out which showed echo-concentric LVH, normal resting systolic function, presence of diastolic dysfunction, no regional wall motion abnormalities, and normal PA pressure. Coronary angiography was done on 1st March 2011 which showed LVEF 50% and triple vessel disease. Revascularization was contemplated; however, his routine investigation showed a positive HIV test; hence no further interventional treatment was carried out.\nThis new development necessitated further investigations. Philadelphia chromosome was tested which showed a positive result. CD3, CD4 and CD8 count results were 2940 cells/mm3, 393 cells/mm3 and 2477 cells/mm3 respectively. Viral load was tested on 5th November 2011and it showed 43356 HIV-1 RNA copies/ml.\nHe was advised to start anti-retroviral therapy along with Imatinib, however, patient declined to take this treatment due to lack of resources. He was therefore continued on hydroxyurea.\nIn May 2012, the retroviral disease tests were repeated. The results of both the tests are given in .\nPatient continues to take hydroxyurea regularly and in spite of advanced HIV he has not developed any major complications of HIV or CML during his follow-up period. |
A 50-year-old, non-smoking female patient with a medical history of diabetes mellitus (DM) and hypertension (HTN) presented to the Urology Department of King Fahad General Hospital with a three-month history of hematuria. She denied any history of flank pain, dysuria, urgency, fever, or weight loss, or appetite. Clinical examination was normal, laboratory findings were within the normal range, urine analysis showed >40 red blood cells (RBCs) per high power field, and urine culture showed no bacterial growth. Bladder smooth muscle cell markers (smooth muscle actin (SMA), desmin, and calponin) were positive. B-catenin and CD117 were negative. An abdominal and pelvic CT scan with contrast was done and showed a large submucosal urinary bladder mass. An abdominal/pelvic MRI was performed and revealed a large submucosal well-defined round mass with a slightly lobulated smooth margin originating from the submucosal layer of the left anterolateral wall of the urinary bladder. It measured 5 x 7 x 4.5 cm and was isointense to the T1 weighted image's muscle and heterogenous with the cystic component on the T2 image. No suspicious lymph nodes in the pelvis and no infiltration into the adjacent organs and tissues were found. The finding suggests bladder leiomyoma and solitary fibrous tumor (Figure ).\nAn ultrasound-guided biopsy sample was taken and sent for histopathological examination and revealed a spindle cell tumor. On cystoscopy, a normal urinary bladder wall with no obvious exophytic masses and both ureteric orifices were seen, and a urine jet was present, but there was a massive indentation in the left posterolateral site of the urinary bladder extending to the dome of the bladder. The patient then underwent an open partial cystectomy (Figure ). A specimen of 7 x 5 x 3.5 cm was obtained and preserved in formalin for histopathological examination. A well-encapsulated white firm mass measuring 3 x 2.5 cm was found and the histopathological examination showed spindle cells in sheets which confirm the diagnosis (Figure ).\nA cystogram was done in post-operative day 8 and confirmed no urine leak. The Foley catheter was removed, and the patient was discharged in good condition without any complains. In four months follow-up, cystoscopy showed normal both ureteric orifices with normal bladder wall and no obvious bladder masses. CT abdomen and pelvis with IV contrast in delayed images for follow-up after one and half year showed contrast in the urinary bladder with no definite filling defects (Figure ). In the three, six months and one-year follow-ups, the patient was evaluated, and no hematuria or other complains were recorded. |
A 70-year-old woman presented in November 2017 to the Emergency Department at Skåne University Hospital, Sweden, due to the rapid onset of fever, shivers, and a suspected skin infection. She had a previous medical history of left-sided ductal breast cancer with lymph node involvement in 1999, which was treated chronologically with neoadjuvant chemotherapy, partial mastectomy, axillary lymph node dissection, and radiation therapy. In addition, in 2001, a right-sided localised ductal breast cancer in situ was identified and was treated surgically with a partial mastectomy. Secondary to her lymph node dissection, she developed lymphoedema of her left arm, which had been continuously treated with compression stockings. The patient was on treatment with an ACE inhibitor and a beta-blocker due to hypertension, and in addition, she had a known systolic murmur, characterized as physiological, as transthoracic echocardiographs in 2011 and 2017 were normal. Since her surgery in 1999, on a total of six occasions prior to her last and seventh visit, of which the first episode occurred in 2008, she had been treated for erysipelas in her left upper arm. The presentation had always been sudden with spiking fever and erythema spreading in approximately the same localisation. Interestingly, on all three out of the three occasions where a blood culture has been drawn on presentation with erysipelas, the cultures have shown growth of a bacterium belonging to the S. mitis group. These first two isolates also had similar MIC values for penicillin of 0.064 and 0.125 mg/L, for vancomycin of 0.25 and 0.5 mg/L, and for gentamicin of 2 and 2 mg/L (). In addition, they were both sensitive to clindamycin.\nOn the present visit, she once again had a sharply demarcated, warm, swollen, and painful erythema measuring approximately 7 × 15 cm in the lymphoedematous area on her left upper arm. No local portal of bacterial entry was found. Vital parameters showed a temperature of 38.0°C, respiratory rate of 16 breaths/min, O2 saturation of 96% on room air, heart rate of 80 beats/min, and blood pressure of 120/70 mmHg. On physical examination, a grade II systolic murmur was heard with punctum maximum I2 dexter. She had no signs of septic emboli, oral examination showed no signs of infection, and examination of lymph nodes was normal. Possibly due to her quick presentation, that is, less than 6 hours from the onset of symptoms, her laboratory results were normal with a white blood cell count of 8.4 ∗ 109/L, platelets of 263 ∗ 109/L, and hemoglobin of 147 g/L. Her CRP was 12 mg/L. She was clinically diagnosed with erysipelas, and due to previous bacteraemia with the S. mitis group in relation to erysipelas and the presence of a systolic murmur, blood cultures were drawn and she was treated with one dose of intravenous penicillin (3g≈5 million IU) followed by an oral penicillin (1g≈1.6 million IU) three times daily, for seven days. Once again, now for the third time, the two blood cultures showed growth of a bacterium belonging to the S. mitis group. The MIC value for penicillin was 0.125 mg/L, for vancomycin 1 mg/L, and for gentamicin 16 mg/L (). Similar to the two previous isolates, it was also sensitive to clindamycin. Her treatment was prolonged for 10 days, and a follow-up visit was arranged. Repeat blood cultures were drawn 14 days after discontinuation of antibiotics and they were negative. To prevent further infections, she has once again been referred to the lymphoedema outpatient clinic as well as to the dentist office. On follow-up, thereafter, the patient had no sequelae to her infection, and she gave informed consent for this case report to be published.\nThe three blood isolates, one analysed in 2015 and two in 2017 (15 and 8 months apart), were initially subgrouped to S. mitis/S. oralis/S. pseudopneumoniae of the S. mitis group by combining the MALDI-TOF MS results (MALDI Biotyper, Bruker) with the information that the three stains were resistant to optochin. To allow a more detailed comparison, the three stored isolates were reanalysed and now ethanol/formic acid extractions were performed on the strains, and the updated and improved Bruker MALDI Biotyper database (DB-7311 MSP Library) was used for the MALDI Biotyper analysis. In addition to the standard log (score), weighted list (scores) was also calculated []. S. mitis was the best match for both the first and second isolates when both log (score) and list (score) were calculated. For the third isolate, the best match was S. oralis for both types of scores (). Next, the mass spectra of the three isolates were inspected manually. All three strains showed the specific peak 6839.1 m/z which is associated with S mitis and S. oralis strains, but only the third isolate showed the specific peak 5822.5 m/z which is associated with S. oralis () []. In addition, no peak profiles typical for S. pneumoniae and S. pseudopneumoniae could be detected in the three isolates [, ]. These results further support that the first two isolates are S. mitis and the third isolate is S. oralis. Many differences were seen in the mass spectra of the third isolate (S. oralis) compared to the first two (S. mitis). On the other hand, no clear differences in the spectra between the first and second isolate could be seen, and one can therefore not exclude that they belong to the same clone. |
A 52 -year old female with intense pain (Visual Analogue Scale- VAS 10) [] in the left maxillary first molar was referred to a private dental clinic for any treatment possible to relieve the pain. Her face was pale, and the severity of the pain made her sleepless the night before. She said that she was diagnosed as having Non Hodgkin's Lymphoma (NHL) 5 months earlier and had been under anti lymphoma chemotherapeutic treatments since then. Clinical examinations of the involved tooth did not show decay or past crown restoration[]. She had a good oral hygiene and did not have any periodontal problems. The tooth did not show abrasion or attrition lesion, and no gingival recession was seen around it. The sensation due to lack of cementum, in Cemento Enamel Junction (CEJ) area was ruled out as well. Vertical and horizontal percussion and also vestibular palpation tests caused severe tooth ache. In addition, the tooth was very sensitive to cold water and air stimulation. Following careful radiographic and clinical examinations, vertical root fracture and cracks in crown were also ruled out. After interviewing the patient, and consulting her oncologist, it was revealed that the tooth did not have any symptoms of pain, tenderness or discomfort before the administration of chemotherapeutic drugs. After consulting her oncologist and further observation of the applied anti lymphoma protocol of the patient, it was found that the pain had been commenced after administration of Cyclopho-sphamide chemotherapy drugs and had intensified in the last cycles of its administration. Due to the severity of the pain, a through root canal treatment was performed on the tooth after obtaining the patient's agreement.\nThe tooth was desensitized by infiltration of 2% Epinephrine-Lidocain solution (Daroupakhsh-Iran). Moreover, an emergency pulpotomy treatment was performed on the tooth at this session. Then, the root canal pulp was extirpated by a broach (Maillferr -Swiss) which significantly relieved the tooth pain, and a temporary sedative restoration was applied on the treated tooth. Furthermore, another appointment was arranged for the patient to complete the root canal treatment, using Mtwo nickle-titanium rotary system (VDW-Munich- Germany) []. |
A 16-year-old female teenager reported that she began to present with edema of her right leg at the age of three at which time she sought treatment; however, the cause of the swelling was not diagnosed. At ten years of age, she also observed that her left leg was swollen, and at the age of 13, a lymphoscintigraphy was performed which detected lymphedema of all four limbs, arms, and legs. At 16 years of age, after erysipelas, the edema of the lower limbs worsened and clinically identifiable lymphedema of the right arm was observed, especially the hands, so she sought the Clínica Godoy for treatment. A volumetric evaluation was performed of all four limbs, and an intensive 8-hour treatment program was proposed. The adolescent was submitted to manual (Godoy & Godoy technique) [] and mechanical lymphatic therapy (RA Godoy) [] of the lower limbs, cervical lymphatic therapy (cervical stimulation) [], and the continuous use of a grosgrain stocking [].\nA reduction in the size of all four limbs was observed at the end of the 5-day treatment program without using any therapies specifically to treat the arms (). Maintenance of the results also used the grosgrain stocking and mechanical lymphatic therapy (RA Godoy) of the legs at the patient's home.\nIn the maintenance phase, the patient was advised about the need of skin care. Moreover, she performed myolymphokinetic exercises of both the legs and arms, prioritizing activities that require little effort and did not involve impact or repetitive movements that could aggravate the edema. The patient was asked to return for monitoring by the care team every three months; however, she did not return. Only after four years, she returned to the service to treat an outbreak of erysipelas of the legs and the accompanying worsening of the lower limb lymphedema. At this time, she did not present with edema of the arms (). Thus, the initial intensive treatment for leg lymphedema was sufficient to treat the arm lymphedema.\nThis case report was approved by the Research Ethics Committee of FAMERP (# CAAE: 27810214.6.0000.5415). |
A 42-year-old male patient with history of hypertension, dyslipidemia, diabetes mellitus, and previous myocardial infarction (MI) presented to the emergency department with acute coronary syndrome. His past coronary event was an inferior MI which was treated with thrombolysis six years ago within 7 hours of onset. Catheterization at that time revealed anomalous right coronary artery originating from the left Valsalva sinus with moderate proximal obstruction and the patient received medical treatment. He had recently performed a clinical evaluation with a normal treadmill test and normal echocardiogram in another hospital.\nHe presented to the emergency department of a non-24/7 primary PCI medical centre with acute onset (2 hours) of severe chest pain and diaphoresis. On admission, he was anxious, his blood pressure was 80/50 mmHg, and he had normal heart and lung auscultation. His first electrocardiogram () showed ectopic atrial rhythm, inferior inactive zone, and ST elevation in precordial leads V1–V3 (there were no previous electrocardiogram tracings available for comparison). He was treated with IV hydration, acetylsalicylic acid 300 mg, clopidogrel 600 mg, and thrombolysis with Tenecteplase 40 mg with improvement of peripheral perfusion and blood pressure. However, due to persistent chest pain, emergent coronary angiography was performed four hours later. Left main and circumflex were free of significant obstruction and left anterior descending artery had a moderate systolic dynamic compression caused by intramyocardial bridging in the mid segment with normal TIMI flow (). Right coronary artery had its anomalous origin close to the left coronary sinus and was occluded in the proximal segment ().\nPercutaneous coronary intervention was performed in the right coronary artery through right radial approach using Amplatz 1 (6 French) guiding catheter. He received 8,000 U of unfractioned heparin. The lesion was easily crossed with a Runthrough 0.014 guidewire and predilated with a 2,5 × 20 mm balloon with flow restoration. A Zotarolimus eluting stent (Endeavor) 2,75 × 24 mm was implanted at 12 atm and postdilated with a 3,0 mm balloon at 20 atm with clinical and angiographic success. At the end of the procedure, the patient was asymptomatic and ECG showed resolution of ST elevation.\nThe patient had in-hospital course free of complications and predischarge evaluation revealed normal left and right ventricular function (ejection fraction 59%) and complete resolution of precordial electrocardiogram abnormalities and return to normal sinus rhythm (). The patient was discharged six days after admission taking acetylsalicylic acid 100 mg, clopidogrel 75 mg, atorvastatin 80 mg, metoprolol 100 mg BID, and enalapril 10 mg BID. After an uneventful one-month follow-up, the patient was asymptomatic. |
An 87-year-old woman presented to our outpatient clinic with a 7-month history of vivid visual hallucinations. These symptoms started as simple images and sparkling lights and progressed to complex hallucinations including objects and people moving in her room. They occurred 4–6 times a day and lasted 5–10 minutes each. Although she was aware of the nature of these symptoms, the hallucinations became more persistent and were disturbing to her. She had good insight into her condition. There were no associated auditory hallucinations or delusions, and she had no history of any psychiatric disorder.\nShe denied any headaches or trauma. Her medical history included hypothyroidism and open angle glaucoma. Her medications included l-thyroxine and anti-glaucoma eye drops. Surgical history was significant for bilateral cataract surgeries a few years prior to current treatment.\nHer visual acuity was 6/60 in the right eye and finger counting at 1 m in the left eye. The scores were 6/60 in both eyes 2 years prior to her presentation. Mental status examination revealed an appropriately dressed woman with no psychomotor agitation or retardation. Her thoughts were logical and goal directed. Affect was euthymic with spontaneous emotional reactivity. Mini-Mental State Examination revealed normal cognitive function including normal orientation and intact recall and attention abilities.\nLaboratory tests included a normal complete blood count, blood sugar, kidney and liver function, thyroid stimulating hormone, vitamin B12, and calcium levels. Magnetic resonance imaging of the brain revealed age-related atrophic changes with small vessel disease.\nThe diagnosis of typical CBS was made based on the presence of visual hallucinations with visual impairment in a cognitively normal patient without psychiatric illness. The patient was initially treated with quetiapine 25 mg/day, which was later increased to 50 mg/day. After 1 month, the hallucinations were still present, and the patient was distressed. Hence, the treatment was changed to risperidone, 0.5 mg/day. After 3 days, the patient had cessation of the visual hallucinations lasting until her next visit, 6 weeks later. The patient’s risperidone was then decreased to 0.25 mg/day; however, the hallucinations reoccurred. The dose was then returned to 0.5 mg/day, and the hallucinations disappeared again. |
A 51-year-old man presented with recurrent cough following pyrethroids spraying in an enclosed area for eradication of flea. Twenty four hours after the beginning of symptoms he admitted in first medical center and partially treated for 2 days with impression of respiratory poisoning. He discharged seemingly healthy, so that he could walk and breathe normally at the time of discharge. The same night symptoms recurred and he referred to second medical center and admitted for one and a half day and underwent indeterminate treatment. Four days after beginning of symptoms the patient referred to our tertiary university hospital with complaints of ptosis and dyspnea. Within 5-6 days of admission limb weakness, dysphagia, dysarthria, blurred vision, diplopia, tremor and respiratory distress added to previous symptoms. The patient was admitted to the ICU, intubated and mechanical ventilated. There was no history of diarrhea or respiratory infection in preceding 4 weeks.\nIn first physical examination the Blood pressure was 110/80, Temperature = 37.4, Respiratory rate = 16, Pulse rate = 80. He was conscious aware, obey and oriented. The pupils were of normal size and reactive. There was no evidence of gaze preference and nistagmus. The ophthalmoscopic examination was normal. Cranial nerves were intact and no gaze palsy was detected. In sensory examination, there was no stocking and gloving sensory loss and no sensory level. The position and vibration sensation was intact. In motor exam, there was no hypotonia or spasticity and rigidity, without any evidences of abnormal movements.\nDeep tendon reflexes were 2+/and symmetric in upper and lower limbs and a reduced manual muscle force of 4/5 on the Medical Research Council (MRC) grading was detected symmetrically in upper limbs.\nElectrocardiogram, chest X-ray and brain MRI were normal. No botulinium neurotoxin was detected in serum or feces. Lumbar puncture revealed normal CSF analysis without cytoalbuminemic dissociation.\nIn first electrodiagnostic study (EDX), 13 days after admission, distal motor latency (DML) of bilateral median and ulnar nerves was mildly prolonged in axonal range []. Other parameters of motor nerve conduction studies (NCS) and F-wave latencies were within normal limits. Examination terminated prematurely due to sudden deterioration of respiratory function and hemodynamic status of the patient during EDX study. In the second study, 10 days later, compound muscle action potential (CMAP) amplitude was reduced in upper limbs in comparison to the first study []. Distal sensory latency (DSL) was prolonged in bilateral median and ulnar nerves []. In repetitive nerve stimulation (RNS), 10.4% decremental response in Nasalis muscle and up to 34.1% decremental response in abductor digiti minimi (ADM) were detected [Tables and ]. After exercise for 10 seconds post activation facilitation was seen in the ADM muscle []. In needle electromyography (EMG), reduced recruitment of normal-shaped motor unit action potentials (MUAPs) and fibrillations and positive sharp waves in facial and upper limb muscles were detected []. Slight polyphasia in deltoid was unremarkable.\nWe followed the patient conservatively without any immunomodulation: Keeping the patient in ICU intubated and mechanically ventilated, checking CBC, electrolitis and liver enzymes, controlling intake and output, feeding with nasogastric tube and DVT and bedsore prophylaxis. After the first week of ICU admission, condition of the patient improved but respiratory failure persists. During the first month several attempts of weaning was unsuccessful and the patient confined again on mechanical ventilation due to recurrence of respiratory distress. After 3 months he completely recovered without use of any antidote, plasma exchange or IVIG and discharged in good general health. In follow-up visits after 6, 12 and 24 months the patient was strong and healthy without any residual weakness. The 23-year-old patient's son developed milder but similar symptoms of ptosis and dyspnea that remitted after 2 weeks without any hospitalization. |
An 81-year-old man with a history of rectal bleeding for the previous one month was referred to our clinic. He presented bright blood which was not mixed with the stool. The patient who was otherwise healthy, had no previous history of urologic intervention. Digital rectal examination found a firm, round solid submucosa nodule in the anterior rectum, it was 1cm in size and 5cm above the anal verge. After administration, systemic examination showed normal results with normal level of serum cancer embryo antigen (CEA 2.38 ng/ml) and prostate specific antigen (free PSA 0.8 ng/ml, total PSA 2.38 ng/ml). Colonoscopy confirmed the nodule was 1cm in diameter and in the anterior rectum 5cm above the anal verge with an otherwise normal look of the surface rectal mucosa (). Magnetic resonance imaging (MRI) demonstrated a well-circumscribed and submucosa nodule measuring 1cm in diameter at the level of 5cm above the anal verge protrude to the rectal lumen (), with relatively low intensity on T1-weighted images and high intensity on T2-weighted images, however it was enhanced on the contrast imaging. The border between the nodule and the prostate was clear. After the diagnosis for a nodule in the submucosa rectum was made with the clinically suspicious of carcinoid tumor, local excision of the nodule en bloc with 5mm normal mucosa around the nodule and some superficial muscularispropriawas performed.\nDuring the procedure a well-encapsulated round nodule was found in the submucosa. The nodule was 1cm in diameter, and could be separated from the surrounding submucosa tissue. Postoperative days were uneventful, and the patient was discharged on the second postoperative day. Under the microscope, the histologic sections revealed a well-encapsulated nodule of dilated glandular structures. The nodule was full of dilated glandular structures which was located between the severe burnt rectal mucosa and muscularispropria. The dilated glandular structures were surrounded by a dense fibrovascularstroma, which contained bland, spindled and ovoid cells without mitotic activity. Within the glandular epithelium, a range of morphologic appearances was evident and which showed the prostatic nature. The glands of interest had both a luminal layer and a basal layer of cuboidal to columnar cells. The typical prostatic acini possessed papillary infoldings of the luminal epithelium and occasional cribriforming of the glands, some degree of corpora amylacea was present (). Immunohistochemical staining for PSA strongly revealed positive staining of the epithelium (), confirming the prostatic nature of the nodule, while the stromal cells showed strong staining for actin and smooth muscle actin. No evidence of malignancy was observed. This patient has no evidence of recurrent disease during four years follow-up. |
A 30-year-old female patient reported to the outpatient department of oral diagnosis with a complaint of recurrent swelling all over the gums since 2 years, which was gradual in onset and increased in size since 2 months. Along with this, there was difficulty in mastication, bleeding gums while brushing the teeth and bad breadth since 2 months. Patient reported similar swelling 2 years ago, for which surgical management in form of gingivectomy was carried out. The medical history was not contributory. Family history was also void of any genetic disorders.\nOn extra-oral examination, the patient had incompetent lips and convex profile. There was mild asymmetry of the face with fullness over both right and left side of face. On palpation, the swelling was soft to touch. Single, left submandibular lymph node was palpable, which was tender, soft and movable.\nIntra-oral examination revealed massive generalized, diffuse, nodular enlargement of the gingiva involving both maxillary and mandibular arch []. It was firm and leathery in consistency. The gingiva appeared pink in color with generalized loss of stippling. Grade III mobility was seen with 17, 25, 26, 31, 32, 35, 37, 38, 41, 42 and Grade II mobility was observed with 34 and 43. Bleeding on probing and pus discharge was seen in association with majority of teeth. Posterior teeth were barely visible as the enlarged gingiva covered occlusal 1/3rd of crown [Figures and ]. Based upon clinical features, a provisional diagnosis of chronic inflammatory generalized gingivitis with advanced periodontitis was made.\nRadiographic examination revealed presence of all permanent teeth except 16 and 36 and severe generalized vertical and horizontal bone loss in maxillary and mandibular posterior teeth. The remaining teeth showed migration with spacing. Few carious teeth were noted [].\nBlood chemistry was nonsignificant except for mild lymphocytosis and low hemoglobin level (9 g/dL). Red blood cell indices were within normal limits. Differential count showed mild lymphocytosis. No abnormality in morphology of cells was reported.\nExcisional biopsy of gingiva was performed and subjected to histopathological examination. Histological features showed parakeratinized stratified squamous epithelium with elongated rete ridges at places. Connective tissue showed collagen fibers arranged in bundles and coalescing pattern. Marked chronic inflammatory cells were present [].\nOn the basis of medical, family, drug history, clinical, radiographic and histopathological findings, a final diagnosis of recurrent idiopathic fibromatosis with aggressive periodontitis was given.\nTreatment plan included oral prophylaxis followed tetracycline irrigation and administration of chlorhexidine mouth wash and extraction of Grade III mobile teeth. Gingivectomy with flap surgery was planned subsequently for remaining teeth. |
A 59 year old man with no prior medical history, presented to a peripheral hospital emergency department with sharp pain retrosternally after eating fish the previous day. He was able to swallow fluids and soft diet but with odynophagia. A cardiac cause was ruled out and a barium swallow was organised as an outpatient. The patient was discharged home. Day five after presentation he had frank haematemesis and some malaena. He was haemodynamically stable with a haemoglobin at 130 g/L and admitted to the general surgical ward. The next day he had another large haematemesis and proceeded to urgent upper gastrointestinal (GI) endoscopy where a fish bone was seen protruding from an ulcerated area in the oesophagus, 24 cm from the teeth. There was some active bleeding after the fish bone was removed. A Sengstaken-Blakemore tube (SBT) was inserted and the oesophageal and gastric balloons inflated. This controlled the bleeding and the patient was admitted to the intensive care unit (ICU) with intravenous (IV) antibiotics as well as an ongoing blood transfusion. Overnight there was no further bleeding but the patient had evidence of sepsis with a high fever and hypotension despite no evidence of blood loss. He required inotropic support.\nThe following morning, the patient was transferred to the ICU at our centre. The SBT balloons were deflated and patient observed. There was no evidence of blood loss over the ensuing 24 hours so the SBT was removed. Five hours later, he had a massive haematemesis causing hypovolaemic shock with cardiac arrest requiring reinsertion and reinflation of the SBT, massive transfusion and cardiopulmonary resuscitation. Once stabilized, a computed tomography (CT) angiogram was performed. This demonstrated an aortoesophageal fistula located 2 cm distal to the origin of the left subclavian artery on the descending aorta (Fig ). In the vascular intervention suite under image intensifier control, an endoluminal stent-graft (Zenith® TX2™ Thoracic TAA Endovascular Graft – one piece Thoracic Body Extension TBE 30–80, William A. Cook Australia Pty. Ltd, Brisbane, Australia) was inserted via the left femoral artery (Fig ). A small endoleak was noted after deployment but was controlled by partial inflation of the SBT oesophageal balloon. The patient returned to ICU with Vancomycin, Timentin and Fluconazole antibiotic cover and his coagulopathy was corrected. A repeat CT angiogram after 24 hours showed no leak after deflation of oesophageal balloon of the SBT. There was significant haematoma within the mediastinum. The SBT was removed the next day without complication. An upper GI endoscopy, five days later revealed a deep oesophageal ulcer at 24 cm with superficial ulceration extending to 29 cm circumferentially. A naso-jejunal feeding tube was inserted at that time.\nSubsequently, a percutaneous endoscopic gastrostomy (PEG) was performed. Repeat endoscopy at one month, showed an improvement in the superficial ulceration but the aortic stent was visible through the wall at 24 cm. The patient was maintained on broad spectrum antibiotics delivered via the PEG tube whist discussions were held between him and his family with respect to the management options. However, 51 days after the aortic stent-graft insertion the patient developed a spiking pyrexia and general malaise. Blood cultures were positive for a methicillin-resistant staphylococcus aureus (MRSA) and he was recommenced on IV Vancomycin. Repeat CT angiogram revealed no mediastinal gas, fluid levels or significant soft tissue changes to suggest mediastinitis. It was felt the bacteraemia was related to the infected aortic stent-graft.\nAfter multidisciplinary surgical discussion, a plan for definitive treatment was made. Firstly, the patient had a cervical oesophagostomy with the site of division guide by the CT scan to ensure no dissection close to the fistula. A left hemithyroidectomy was needed to allow a tension-free stoma. Two days later, when an aortic allograft became available, the aortoesophageal fistula was repaired. Initially, a left trapezius muscle flap was raised so that this could be brought through the posterior thoracic wall via a window created from partial resection of the fourth and fifth left ribs. After raising the flap, the patient was placed supine and the mediastinum was accessed via a clam-shell incision. We went on cardio-pulmonary bypass with access via left common femoral artery, ascending aorta and right atrium. Retroplegia and left ventricular vent were inserted, the latter via left superior pulmonary vein. The patient was placed into deep hypothermic arrest to 20°C. The aorta was opened from distal arch to 1 cm beyond the distal extent of the endoluminal stent. The descending aorta was cross-clamped and antegrade cerebral perfusion re-established. The stent-graft was removed and sent for microbiological assessment along with involved aorta including the proximal left subclavian artery. The aortic homograft was anastomosed proximally to the isthmus of the arch. The trapezius muscle flap was pulled through from behind. The aortic side branches under-run and the homograft positioned and anastomosed distally. Following re-warming and the patient coming off cardio pulmonary bypass the remaining oesophagus was resected to the level of the azygous vein out of the operative field. Drains were placed in both pleural spaces and in the mediastinum with vacuum suction drains to flap site. Parentral antibiotic cover consisted of Meropenum for two weeks and Vancomycin for four weeks. Cultures from the stent and aortic tissues grew MRSA.\nTen weeks after the aortic repair, the patient was readmitted for reconstitution of the gastrointestinal tract. The colon was mobilised on the ascending branch of the left colic vascular pedicle. During the development of a retrosternal tunnel there was substantial venous bleeding related to adhesion of the right ventricle to the posterior sternum. A median sternotomy was performed and two tears in the right ventricular wall were repaired with bovine pericardium. Because of dense adhesions retrosternally, the use of a retrosternal tunnel was abandoned and a subcutaneous tunnel created. Due to tension from the skin closure on the interposed colon, the colon was returned to the abdomen and the subcutaneous tunnel was stretched overnight with the aid of a half filled litre bag of saline used as a tissue spacer. The following day, the colonic interposition graft was taken to the neck via the subcutaneous tunnel and anastomosed to the proximal oesophageal remnant. There were no complications and he was commenced on oral fluids at day nine after a contrast swallow showed no leak and good passage through the conduit. He was discharged home one week later tolerating normal oral diet. The patient is well with normal activity, 19 months after the original diagnosis of aortoesophageal fistula with minor gastro-oesophageal reflux being his only symptom. |
A 68-year-old woman had a history of pars plana vitrectomy with ILM peeling and silicone oil tamponade for the treatment of concommitant rhegmatogenous retinal detachment and FTMH in her left eye before 6 years. She had her retinal detachment repaired, but the macular hole persisted despite long term tamponade with silicone oil. A year after the initial one, she had a second surgery using short term gas tamponade with sulfur hexafluoride gas (SF6), but the hole failed to close. A third attempt to close the macular hole was carried out a year after, with carbon hexafluoride gas (C3F8). Despite her strict obedience to face down positioning, the hole failed to close.\nAfter three operations that failed, the patient did not prefer to have further interventions and remained on regular follow-up visits for about 4 years. During this time period no significant anatomical and functional change occurred in her left eye.\nIn her last visit, she had a best corrected visual acuity (BCVA) of counting fingers (CF) in her left vitrectomized eye which had a chronic large macular hole with a base daimeter of 1697 micrometers. Upon proposal, she has accepted a fourth surgery with autologous PRF application.\nThe surgical technique included four ports pars plana approach for the application of a round button of PRF to the macular hole, followed by fluid-air and air-SF6 gas exchange. The patient was ordered to have face down positioning for three days following the operation.\nThe PRF button was cut with scissors from a PRF membrane which was prepared with Choukrun's procedure described elsewhere [, ]. In short, this procedure consisted of centrifuge of autologous blood drawn immediately before the operation, followed by removal of the fibrin clot from the test tube and its compaction with a metal press board. The PRF button was slightly larger than the macular hole, permitting complete plugging of the hole (see video, Supplementary , which summarizes surgical steps).\nThe week after the operation, the hole was shown to be completely sealed by the PRF button on optical coherence tomographic (OCT) images. The button was resorbed slowly until the third postoperative month, and replaced by neuroglial proliferation which closed the hole completely ().\nDespite the chronic course of the pathology and low initial visual acuity, the Snellen BCVA improved to 0.16 in the last control visit. No complication occurred during or after the procedure. |
The patient is a 30-year-old male who was born in Turkey and is of Kurdish descent. He moved to Switzerland in 2009 at the age of 19. In 2010, he married a Swiss woman with whom he has one child. They divorced in 2016 and he has been living alone in a rented apartment since.\nHe first presented in our outpatient clinic at the age of 22 in late 2012. He reported that he had lost his driving license due to cocaine use while driving and that the relationship with his wife had ended. At this time, he was unemployed, smoked cannabis 2-3 times a week and used alcohol regularly. The Beck depression inventory (BDI) showed a moderate depression with a score of 26. Over the following years, he was hospitalized 30 times in our clinic, voluntarily as well as involuntarily (see ). He was diagnosed with cannabis use disorder (2014), mixed personality disorder with narcissistic and antisocial traits (2015), cocaine use disorder (2016) and schizophrenia (2018) according to ICD-10.\nHe first admitted himself to our clinic in 2014. He wished to cease cannabis consumption (about five joints a day) and reported depressive symptoms. In 2015 he was incarcerated for criminal assault. During his imprisonment, he committed a suicide attempt (self-administered lacerations at his neck and sternum). Consequently, the patient was admitted to a psychiatric forensic department, where he removed a lightning bulb in the isolation room and injured himself on the left wrist. In 2016, at the age of 25, he reported to suffer from acoustic hallucinations for the first time (imperative voices telling him to consume drugs) and that he had experienced these symptoms for 4 years. It was unclear whether this first documented psychotic episode was caused by substance use alone and he did not meet the criteria for paranoid schizophrenia according to ICD-10 at the time. During treatment, he stated that the use of cannabis would positively affect his hallucinations. He also reported to gamble a lot, losing up to CHF 9,000 in a single month. Additionally, he repeatedly expressed suicidal thoughts, like the idea of ending his life with a drug overdose. From 2016 onwards, he continued to admit himself or was referred to our clinic through his practicing psychiatrist. He alternately presented psychotic and depressive symptoms, besides the wish for detoxification and withdrawal-treatment due to continued heavy cocaine (1–2 g/day), alcohol (5–8 liters beer/day), and cannabis (3–5 joints/day) use. In 2018, the psychotic symptoms persisted despite the patient's abstinence from cannabis and cocaine, hence the diagnosis of paranoid schizophrenia was made. Due to the severity of his psychotic symptoms inpatient treatments focused on psychoeducation and the establishment of pharmacotherapy with atypical antipsychotics. In between these hospitalizations, the patient repeatedly stopped his medication (see ) and resumed cannabis, cocaine, and alcohol use.\nAt the end of 2019, he was involuntarily committed after shouting and rampaging in public, attacking the arresting police officers, and smashing his head against the police station's wall. He was taken to the hospital, where he had to be sedated with propofol, as his head injury could not be examined otherwise. After that, he was transferred to our psychiatric clinic, where he stated that he could not remember the events that led to his committal. He reported that he had not used cocaine within the last few months but corrected himself after a toxicologic serum screening showed positive for cocaine, cannabis, and alcohol.\nIn May 2020, the patient wanted to admit himself to our clinic yet again. When he was told that an immediate inpatient admission was not possible at the time, he took 20 mg of lorazepam and 55 mg of aripiprazole presumably to force his admission. As sedation wore off, the patient became increasingly aggressive and eventually attacked and injured a staff member, which led to two police-assisted isolation. The next day he apologized for his actions but was subsequently banned from entering the clinic for a month for attacking and injuring a staff member.\nIn January 2021, he was involuntarily committed (29th hospitalization) after getting involved in a brawl. At this time, he was intoxicated, psychotic, and did not cooperate with our staff. Again, he had to be sedated with propofol as otherwise a cranial computer tomography to rule out head injuries would not have been possible. During his hospitalization, a knife with a blade length of 15 cm was found in his room by our staff. The confiscation led to aggressive behavior and threats from the patient, leading to another ban from our clinic.\nIn his most recent involuntary hospitalization (30th), the police reported that the patient went on a rampage, damaging cars in the city center. He was intoxicated (alcohol) but refused to give a urine sample for further toxicological examination. He was delusional and paranoid at the time of admission (fear of getting poisoned). Throughout the hospitalization, the patient started damaging the inventory of our department. An application for compulsory treatment authorization was granted as per article 434, Swiss civil code. After switching from oral olanzapine to oral aripiprazole, the patient agreed to an intramuscular depot medication with aripiprazole. However, he continued to use cocaine and cannabis during hospitalization. Therefore, after the patient gave his informed consent, methylphenidate 100 mg/d was prescribed off-label to support cocaine abstinence.\nAt the same time, we initiated treatment with CBD-rich cigarettes (Manufacturer: Koch & Gsell; Products: “Heimat” Pure Hemp, “Heimat” Tobacco+Hemp; approx. 10% of CBD, <1% of THC) as adjunctive inpatient therapy. A week later in the following therapeutic session, the patient stated that he could now also abstain from illicit cannabis use. He replaced his former habit of smoking cannabis joints (illicit cannabis and standard tobacco) with CBD-rich cigarettes (approx. 20 cigarettes per day, approx. 400 mg of CBD per day). Interestingly, the patient managed to reflect on his cannabis use and its negative impact on his schizophrenia for the first time. He began to realize the relation between worsening of psychotic symptoms and continued use of cannabis containing high THC contents. Additionally, he claimed that “street cannabis would destroy my therapy with aripiprazole.” He maintained cocaine and illicit cannabis abstinence until discharge from our ward. The urine test for cocaine showed a negative result at the time of discharge, 2 weeks after initiation of methylphenidate. THC showed positive as our tests were not able to distinguish between illicit cannabis and CBD-rich cigarette consumption. For the assessment of the patient's compliance regarding the cessation of illicit cannabis use, we relied on our clinical impression and self-reports.\nWe established a controlled daily dispense of methylphenidate and monthly intramuscular injections of aripiprazole (400 mg/28 days) via a local pharmacy. Once weekly psychotherapeutic outpatient contacts were scheduled, and he received weekly rations of CBD cigarettes. The cantonal social department approved to cover the costs of a CBD product for half a year. For financial reasons, we suggested the purchase of “Alplant Skywalker Lemon OG Outdoor, 50 grams” (CBD-rich hemp <7% CBD, <0.2% THC) which was used by the patient to roll his cigarettes. The patient receives a package of this product every week. He continues to appear regularly at the weekly outpatient appointments. Furthermore, he reported significantly reduced illegal cannabis use but admitted some relapses. When reflecting upon the cannabis use, he started to see the link between psychotic symptoms and substance use. Due to this realization, the patient now prefers CBD rather than ordinary cannabis. He refrained from using cocaine since establishing the off-label therapy with methylphenidate. A 6-week and a 12-week follow-up urine test resulted negative for cocaine. No further psychiatric hospitalization was required since discharge. Aided by our clinic's social workers, a supportive long-term therapeutic setting is now being planned. However, his psychotic symptoms (delusion and paranoia) did not completely remit. |
A 25 year-old young man was injured in a motor vehicle accident (inability to control the vehicle due to violation of the speed limit). When his car reached the U-turn, it slid off the road and flipped over. Possible mechanism of injury was dorsiflexion pronation of the left side and dorsiflexion supination of the right side. The patient was brought in conscious and hemodynamically stable at the time of admission; he presented with severe bilateral ankle swelling, numerous bilateral ecchymoses and blisters without any open wounds. On physical exam, toe circulation was mildly delayed but the extremities were warm and no evidence of ischemia could be found. In the systemic review, no serious head, spinal or abdominal trauma requiring emergent measures could be noted. Ankle and plantar radiographs were obtained which demonstrated left ankle Hawkin type 4 open talar neck fracture with subtalar and talonavicular dislocation and a 3 cm open wound (). On the right side, Hawkin type 4 open and comminuted fracture of the right talar body and lateral process with subtalar and talonavicular dislocation, and 3 cm open wound was noted (, ). In spinal radiography there were T10 and T11 vertebral body fractures.\nIn the right lower extremity, multiple fragmented talar body fracture and posterior medial tubercle were opened via an anteromedial approach. Chevron osteotomy of the medial malleolus was performed. After intraarticular irrigation and minimal soft tissue manipulation, reduction was performed by three partial thread cannulated and cancellous screws (4 mm). Consequent intraarticular irrigation was performed and the location of malleolar osteotomy was fixed by 2 malleolar pins (). In the left extremity, the talus neck fracture with medial wall comminution and lateral malleolar tip avulsion was opened by simultaneous anteromedial and lateral approach for a better view, after which intraarticular irrigation and debridement took place. Neck reduction was performed using 2 cannulated cancellous screws (4 mm), and lateral Malleolus fracture was fixed using two pins(number 1.1) (). Antibiotic prophylaxis was continued up to 48 hours after the operation. The patient was discharged with short splints on both lower extremities without any wound complications. The patient’s condition after 2 years follow up was 20 degrees plantar flexion, zero dorsiflexion with moderate pain and AVN and collapse in talar body on the right side. The Foot and Ankle Disability Index (FADI) score was 58/7 and 40 degrees plantar flexion 20 dorsiflexion no pains no AVN on the left side. The Foot and Ankle Disability Index (FADI) score was 82/7 (). The patient`s quality of life was good based on the SF-36 score. |
A 37-year-old healthy man was referred to the University Dental Hospital of Sharjah for the management of a soft tissue mass on the left anterior part of maxillary gingiva, being there for a duration of six months. According to the patient, the swelling enlarged gradually during that period, with frequent episodes of recurrent bleeding without any history of trauma. The patient also presented with a congenital port-wine stain on the left facial skin along the distribution of the maxillary division of the trigeminal nerve ().\nIntraoral clinical examination revealed a labial painless bluish red mass, about 3 × 4 cm in diameter, which was a firm, pedunculated, nontender, and nonpulsatile lesion, extending from the upper right central incisor to the upper left canine ().\nThe oral mucosa overlying the lesion showed some degree of keratinization with multiple superficial ulcerations that bleed easily on a slight touch. The neighboring upper left lateral incisor was nonvital, mobile, and crowned, whereas the upper left canine responded to the vitality test. Finally, the upper right central incisor was firm. The patient had poor oral hygiene and suffered from generalized chronic periodontitis. No regional lymphadenopathy was present, and the facial port-wine stain extended intraorally to involve the upper left labial mucosa, the buccal gingiva, and the whole left side of the hard and soft palates ().\nA panoramic radiograph showed a well-demarcated periapical radiolucency in the alveolar region extending from the root of tooth number 11 till tooth number 23, with nonvital tooth number 22. A supernumerary tooth was detected distal to the root of tooth number 21 within the radiolucent lesion, and an impacted right maxillary canine was present, yet it was irrelevant to the lesion. The nasal cavity and maxillary sinus cavity appeared normal and free from the lesion ().\nThe clinical differential diagnosis included hemangioma, pyogenic granuloma, peripheral giant cell granuloma, and peripheral ossifying fibroma. |
We herein report the case of a 76-year old menopaused multiparous female with a history of three normal vaginal deliveries, well-controlled hypertension and Type 2 diabetes mellitus, who presented to the emergency department for acute obstructive bilateral pyelonephritis having complained of sustained high-grade fever, left flank pain and severe acute storage low urinary tract symptoms. Physical exam revealed a high-grade fever with a grade 4 anterior and apical compartment prolapse with no urine leakage on prolapse reduction testing, and bilateral costovertebral angle tenderness.\nThe patient had undergone a laparoscopic sacrohysteropexy in early 2018 as a cure for her grade 4 debilitating anterior and apical compartment prolapse, also accountable for a bilateral hydronephrosis at the time. She started complaining a year later from a clinical relapse of her cystocele manifesting as a complete vaginal eversion and intermittent self-limiting macrohematuria.\nHer past surgical history also includes a left quadrantectomy and axillary lymph node dissection for a luminal A left breast cancer in 2008, followed by adjuvant radiotherapy, chemotherapy according to an AC-T regimen: 4 cycles of combined doxorubicin and cyclophosphamide followed by 4 cycles of paclitaxel (Taxol) and Tamoxifen-based hormonotherapy.\nFurthermore, she was treated for recurrent upper and lower urinary tract infections (UTIs). The last infectious episode occurred in January 2020 when she was treated for a community acquired nonobstructive pyelonephritis, incriminating a penicillin-resistant Escherichia coli. A renal and pelvic ultrasound performed in the aforementioned context showed no hydronephrosis and a 170 ml postvoid residue. A therapeutic trial by once daily tamsulosin was initiated in an attempt to improve voiding as a mean to reduce the frequency of UTIs. The patient was also instructed to perform clean intermittent catheterization of the bladder once daily but was never compliant.\nAbdominal and pelvic computed tomography (CT) scan without injection of intravenous contrast showed severe bilateral hydronephrosis (A), associated with bilateral hydroureters extending to the urogenital hiatus (B & C), where both of the ureters seem to be directly compressed between the uterine fundus and the pelvic diaphragm (D) due to POP (Stage 4 Ba prolapse and associated Stage 2 C prolapse according to POP-Q classification) (E). Diffuse thickening of the bladder wall could be seen as well, with no evidence of bladder overdistension. CT imaging was not in favor of the eventuality of a ureteral stone migration and revealed no peri-renal fat stranding, nor indirect signs of inflammation.\nBlood work revealed a white blood cell count of 8.0 × 103/dl, a mild anemia, a creatinine level of 0.81 mg/dl, C-reactive protein (CRP) level was 24.6 mg/l. Urinalysis showed combined hematuria and pyuria with urine culture demonstrating the growth of >10∧6 Klebsiella pneumoniae with extended-spectrum betalactames secretion. The patient was treated empirically then according to bacterial sensitivity with intravenous piperacillin-tazobactam (4.5 g intravenously perfused over 6 h, four-times daily). The patient was reluctant to undergo a bilateral emergent nephrostomy and thus 1 g of amikacin was administered once daily, intravenously, for 2 consecutive days due to the sustained high-grade fever with a preserved stable hemodynamic state. Vaginal pessary insertion was proposed but the patient could not find the prescribed device and, therefore, the POP was only reduced manually in a temporary fashion.\nFor the current context, a specialized infectious diseases consultation was sought and piperacillin-tazobactam was started empirically then adjusted according to culture. The patient was apyretic on day 2 of her admission. Due to the rapid recurrence of the prolapse, in the year following her laparoscopic sacrohysteropexy, and due to the patient’s preference, a shared decision was made to perform a transvaginal cure of the prolapse on day 3 of her admission. The surgery consisted of a vaginal hysterectomy, with cystocele repair through an anterior colporrhaphy (consisting of a suture-plication of the vesico-vaginal Halban's fascia – using nonabsorbable multifilament braided separate sutures) associated with Richter’s sacrospinous ligament fixation of the prolapsed vaginal vault using a vaginal flap for prevention of a subsequent vaginal vault prolapse.\nVaginal packing was left overnight, and the Foley bladder catheter was removed 48 h later. PVR was 300 ml, measured by ultrasound testing and the patient left with instructions to perform clean intermittent catheterization twice daily and ertapenem intramuscular injections for a total antibiotic treatment of 14 days.\nThe patient had no PVR on short term follow-up but manifested a new-onset stress urinary incontinence (SUI) and is posted for urodynamic evaluation. |
A 47-year-old previously healthy Sinhala female's right foot was bitten by a snake near the back door of her home in the Kegalle district, Sri Lanka. Within seconds, she felt burning pain ascending along that limb, and there was heavy bleeding from the site of bite. Within a couple of minutes, she felt dizziness, nausea, and numbness of the whole body, had profuse sweating and frothy salivation, and was screaming in pain from the site of bite. On the way to the nearby hospital, she started to clench her jaw tightly and limbs became rigid; she was frothing and was not responding for about 5 minutes, indicating a generalized seizure. She arrived at the hospital within 30 minutes. The doctor at the outpatient department decided to administer ASV and directed the patient to an internal medicine ward for that. Physical examination findings at the ward were a pulse rate of 100/minute and blood pressure of 150/90 mmHg, and lungs were clear to auscultation bilaterally with an arterial oxygen saturation of 95% whilst breathing air with no neurological deficit. By this time, the killed snake was brought in and doctors identified it as a HNV; thus, antisnake venom (ASV) was not administered. Even though there was bleeding at the site of the bite even on admission to the hospital, her 20-minute whole blood clotting time, platelet count, prothrombin time and international normalized ratio, and activated partial thromboplastin time and liver function tests were all normal. Urine sample obtained via the catheter showed 50–55 red cells per high-power field, arterial blood gases indicated a compensated metabolic acidosis, and serum sodium and potassium levels were normal. Her urine output was <100 ml for the first 24 hours and serum creatinine rose from 80 μmol/l to 277 μmol/l. She was transferred to the Teaching Hospital, Kandy, on day 2 for further management.\nOn day 2, a bulla developed at the site of the bite, and there was an edema and warmth at the right foot. Complete (full) blood count demonstrated neutrophilic leucocytosis, and the CRP level of the following day was 261 mg/l. Intravenous antibiotics was started to cover the wound infection. Serum creatinine was 377 μmol/l with oliguria on day 2. Serum sodium and potassium levels remained within the normal range from day 1–5. On the day 5, creatine kinase was 75.1 U/l. Regular hemodialysis every other day from day 2 to day 24 and fluid management were started. Oral sodium bicarbonate was started, and management of her acute kidney injury with collaboration of nephrology team continued.\nOn day 3, her blood pressure rose to 160/90 mmHg, and it was controlled by prazosin and nifedipine SR; however, it generally remained on or above 140/90 mmHg until her discharge. She developed bilateral lung crepitations on day 3 that remained for 7 days. She developed bilateral parotid swelling and edema of the right leg on day 3, and it lasted 3 days. Edema below her right knee persisted another 10 days. Her blood picture on day 2 did not show hemolysis and was suggestive of bacterial infection but blood picture on day 5 showed evidence of microangiopathic hemolytic anemia (MAHA), and same changes were there in a blood film taken on day 11, as depicted in .\nHer day 2 hemoglobin level of 10.8 g/dl dropped to 8.4 g/dl on day 5. On day 2, her platelet count was 104 × 109/l and that dropped to nadir of 29 × 109/l in day 6 and was <150 × 109/l until day 20. A consultant in transfusion medicine has assessed her, and blood transfusion and plasmapheresis was performed on day 7. Another four cycles of plasmapheresis followed. Local edema at the site of the bite increased with necrosis (); thus, wound debridement was done on day 7 and followed up by regular wound toilets.\nWe did an electroencephalogram (EEG) on this patient on the earliest available day (day 11) and that was normal. The 2D echocardiogram done on day 17 was also normal.\nThe offending snake's carcass was taken to the Peradeniya University, and an expert on HNV, Dr. Kalana Maduwage, has confirmed it as a Hypnale hypnale. is a photo of the offending snake.\nAs her daily urine output improved to >1000 ml, she was discharged on day 30 and asked to come for a review in five days. She defaulted treatment and was on alternative medication. After developing progressive bilateral ankle edema and exertional dyspnea, she came back again on day 46, and hemodialysis and supportive therapy were restarted at the nephrology unit. On day 49, she had an anterolateral non-ST-elevation myocardial infarction (non-STEMI), and she was managed at the cardiology unit. She had progressive impaired vision of the left eye starting from a few days after the snakebite and could not count fingers held 30 cm in front of that eye on the 46th day. She was referred to the eye unit, there was bilateral optic disc edema more on the left, the patient was diagnosed of left anterior ischemic optic neuropathy (AION), and steroid therapy was started. Her erythrocyte sedimentation rate and contrast-enhanced computed tomography (CECT) brain done on day 53 were normal. is a photograph of fundi of this patient.\nShe had two episodes of seizures on day 76, and we suspected a possible relationship to her envenomation. The opinion of the neurology team regarding three seizures was obtained. Repeated EEG and CECT brain were normal. Despite being on calcium carbonate 500 mg plus 0.25 μg 1-alpha-hydroxycholecalciferol daily from day 46, her serum calcium level was low (1.8 mmol/l). Last two seizures were attributed to hypocalcemia due to chronic kidney disease following HNV envenomation, and daily calcium carbonate dose was increased to 500 mg thrice daily. After three months, she was diagnosed of end-stage renal disease by nephrology team and on hemodialysis once in four days and was searching for a kidney donor at six months. |
A-17-year-old previously healthy female presented to THP with a history of fever for 2 days associated with body aches and nausea. She didn’t have any abdominal pain, bleeding manifestations or postural symptoms. On examination, she was flushed and febrile but was not pale or icteric. She was mildly dehydrated. Blood pressure was 100/70 mmHg, pulse rate 100 beats/min and capillary refilling time (CRFT) was less than 2 s. On abdominal examination, there was no free fluid. Lung fields were clear on respiratory system examination. Other systems examination was normal.\nHer NS1 antigen was positive and serotype was identified as DEN1. She was managed as dengue fever with continuous monitoring. On the 3rd day of fever, she complained of retrosternal chest pain and undue tiredness. At that time her cardiovascular system examination was normal and electrocardiogram (ECG) showed acute T wave inversion in V2-V5 leads. Troponin I was negative and 2D echo showed global left ventricular hypokinesia and mild impairment of LV function. Ejection fraction was 40–45%. She was treated as having dengue fever complicated by myocarditis. Intravenous hydrocortisone 200 mg 8 hourly was administered for 2 days to reduce myocardial inflammation. On the 4rd day following admission, she complained of abdominal pain and ultrasound scanning revealed free fluid in hepato-renal pouch. Blood pressure was 100/70 mmHg, pulse rate 70 bpm, and CRFT was less than 2 s. She was taken to High Dependency Unit (HDU) and was managed as having DHF complicated with myocarditis with continuous monitoring and with careful administration of fluid to avoid fluid overload. She was discharged on day 7 of illness after recovering from critical phase of dengue fever. She was advised on limiting physical activities. During the follow up on day 14 of the illness, ECG showed reversal of T inversions. Echocardiogram showed improvement of left ventricular function with an ejection fraction of 55%. |
A 69-year-old man developed a sudden epigastric pain. He was presented at this hospital as an emergency outpatient. Six years earlier, he underwent laryngoesophagopharyngectomy, bilateral lymph node dissection for hypopharyngeal cancer, and esophageal reconstruction with a free jejunum flap. On physical examination, the abdomen was flat and soft with tenderness in the epigastric region, but no sign of peritoneal irritation. Blood biochemistry findings revealed elevated values: creatinine, 1.16 mg/dl; lactate dehydrogenase, 364 U/l; and creatine phosphokinase, 622 U/l.\nAbdominal contrast computed tomography (CT) revealed twisted mesentery with the small intestine around the point of torsion (whirl sign) and the superior mesenteric artery as the axis. Contrast enhancement was weakened in the same area of the small bowel (Fig. ). Given this information, we suspected small bowel volvulus and performed emergency surgery on the same day.\nA 5-mm camera port was placed in the umbilicus and 5-mm ports in the lower and right lower abdomen. During laparoscopic examination, the upper jejunum adhered to the small bowel close to the terminal ileum with overlapping of the small bowel. The entire part from the upper jejunum to the terminal ileum was twisted clockwise with the superior mesenteric artery and vein as the axes and the adhesion site as the starting point. There were areas of poor color enhancement throughout the twisted section of the small bowel (Fig. ). We laparoscopically separated the adhesion between different sections of the intestinal tract and traced the bowel from the small bowel in the region of the ligament of Treitz toward the anus to confirm the absence of adhesions or torsion up to the terminal ileum. The color of the small bowel improved; hence, the surgery was completed without resecting any part of the intestine.\nPostoperatively, the patient made good postoperative recovery, resumed oral intake on day 2, and was discharged on day 5 after surgery. No recurrence has been reported 1 year postoperatively. |
A 17-year-old male patient with no known medical history presented to a rural community ED with reports of seizure activity and altered mental status. The patient had sudden onset headache while having intercourse at home, which progressed to seizure activity witnessed initially by the patient’s girlfriend and then by his mother. These were described as tonic-clonic jerking, lasting only seconds. The patient has no known history of seizures. Of note, the patient does not currently take any medications nor does he have any allergies to medications. He has no known medical problems; however, both mother and daughter confirmed the patient's remote history of amphetamine use. On presentation, his vitals were as follows: heart rate, 102 beats per minute; blood pressure (BP), 145/83 mmHg; respiratory rate, 16 breaths per minute; and SpO2 of 98% on room air.\nThe patient presented to the ED via emergency medical services with an acute-onset seizure and altered mental status. An initial exam revealed an obtunded patient without response to verbal or painful stimuli but maintaining his airway with spontaneous respirations. Neurological exam was concerning for a left upward eye gaze with associated bilateral, non-fatiguing nystagmus. Initial differential diagnosis was broad with considerations of metabolic and electrolyte abnormalities, infection, and toxic ingestion; however, given the patient's abnormal neurological examination, intracranial pathology was the top initial concern. Blood was collected and sent to the laboratory with pertinent findings presented in Table .\nDue to ongoing short episodes of seizure-like activity, the patient was administered nasal midazolam and was immediately sent for a non-contrasted head computed tomography (CT) scan (Figures -). This showed a significant ICH with SAH with concern for developing hydrocephalus and possible temporal horn entrapment. Given the findings of ICH with SAH and concern for status epilepticus, the patient was intubated and started on a levetiracetam drip for seizure control and a propofol drip for both sedation and seizure control. Despite the propofol use, he remained hypertensive and was initiated on labetalol for targeted ICH BP management. Ultimately, this allowed for safe transfer to a tertiary care facility with neurosurgery capability.\nHospital course\nUpon arrival at the tertiary facility, the patient remained intubated for airway protection. As noted above, the urine drug screen did return positive for amphetamines, which is postulated as the culprit for the spontaneous hemorrhage. During the patient’s stay, bilateral external ventricular drains (EVD) were placed to allow for simultaneous cerebrospinal fluid drainage and intrathecal tissue plasminogen activator administration. Extensive imaging and procedures were completed in hopes of identifying the etiology, including CT angiography and four-vessel angiography. Neither CT angiography nor invasive four-vessel angiography found an identifiable source of hemorrhage, as no aneurysms or vascular malformations were identified.\nClinically, the patient progressed from a comatose state on initial presentation to fully alert, oriented, and able to follow commands. On day eight of his hospital stay, he was successfully extubated. On day 14, which is known as the last day of the vasospasm window in the neurocritical care setting, the patient had the EVDs removed and was transferred to a neurocritical care step-down unit where he underwent physical therapy. He was discharged on day 28 without any focal neurological deficits. Follow-up non-contrast head CT revealed no further ICH. |
A 72-year-old woman presented with pain, swelling, and limited movement of her left knee 4 h after injury.\nThe patient had a 14-year history of osteoporosis and 10-year history of bilateral medial knee joint pain with limited movement due to anteromedial OA. On January 10, 2019, the patient underwent medial Oxford UKA of the right knee at our department. About 1 year after the operation, her pain in the right knee completely resolved, but pain in the left knee persisted and gradually increased. She was supposed to undergo left medial Oxford UKA at our department after January 2020. Owing to the coronavirus disease 2019 (COVID-19) pandemic, she could not come to the hospital. On the morning of February 9, 2020, she fell down and injured her left knee; she had severe pain in her knee and had limited movement. She was brought to our hospital. After X-ray examination, she was diagnosed with a comminuted fracture of the left patella. After COVID-19-related pneumonia was excluded, she was admitted to our department.\nThe patient had a history of hypertension with regular medication and no heart disease or diabetes.\nThe patient neither smoked nor drank and she denied any family history.\nThe results of the right knee surgery were excellent. Clinical examination of her left knee revealed local swelling without skin abrasion. There was obvious tenderness over the medial joint line and the surface of the patella, and the fracture space could be palpated. Passive ROM testing exacerbated the pain. The patient’s Oxford Knee Score before patellar fracture was 29 and body mass index was 20.5 kg/m2.\nThe laboratory test results were normal.\nRadiographs showed a comminuted fracture of the left patella and confirmed OA of the medial knee compartment with osteophytosis and diffuse grade 4 cartilage defects. The lateral compartment was intact. The varus stress film of the left knee showed a “bone on bone” sign in the medial knee compartment (Figure ). |
A 40-year-old male presented to our clinic with a history of holocranial headaches for the past 16 years that had gradually worsened. For the 1 year prior to presentation, he was troubled on an almost daily basis with severe headaches that also caused frequent nocturnal awakenings. He had been treated elsewhere for migraine headaches without significant symptomatic improvement.\nHe had noticed acromegaloid changes in the form of coarsening facial features and an increase in the size of his hands and feet during the prior 10 years. However, he did not seek medical attention for these changes, as they seemed to cause no harm. The patient had no history of seizures, loss of consciousness, recurrent vomiting, or episodes of transient visual obscurations. He denied a history of hypertension or diabetes mellitus. Two months before the current presentation, he had an episode of severe headache that he described as the worst headache of his life. The episode lasted for 4 to 5 hours, and he was treated with intravenous analgesics and fluids. No nausea, vomiting, dizziness, blurring of vision, diplopia, or any other focal neurologic deficit was associated with the episode. He denied a history of head trauma, anticoagulant use, prior surgical intervention, cranial irradiation, and pituitary dynamic testing.\nOn examination, the patient's pulse rate was 84/min, and blood pressure was 128/76 mmHg. His height, weight, and body mass index were 175 cm, 74 kg, and 24.1 kg/m2, respectively. The clinical features of acromegaly were confirmed on physical examination; however, no signs of active disease were noted (, , and ). Visual acuity was 6/6 assessed using a Snellen chart, and field testing done by confrontation perimetry was normal. The remainder of the patient's general and systemic examination was unremarkable.\nLaboratory evaluation () revealed normal complete blood count, liver, and renal function tests. Endocrine profile included normal serum free thyroxine, thyroid-stimulating hormone, 8 am cortisol, testosterone, prolactin, and insulin-like growth factor 1. Growth hormone suppression test with 75 g glucose revealed nadir growth hormone of <1 μg/L (normal). These investigations suggested normal pituitary function with no evidence of growth hormone hypersecretion. MRI showed enlarged pituitary fossa, a 12-mm macroadenoma in the right half of the pituitary gland, and evidence of hemorrhage within the mass ().\nThe differentials of acute severe headache include subarachnoid hemorrhage, pituitary apoplexy, hypertensive encephalopathy, acute cerebral ischemia, intracranial hypotension (spontaneous or post lumbar puncture), idiopathic intracranial hypertension, posttraumatic headache, substance use or withdrawal-related headache, migraine, cluster headache, and idiopathic thunderclap headache. Our patient had severe acute headache with clinical features suggestive of acromegaly and neuroimaging evidence of pituitary macroadenoma with hemorrhage, suggestive of pituitary apoplexy. However, clinical and biochemical markers of acromegaly activity were normal, suggestive of inactive disease. The patient had no history of transsphenoidal surgery or cranial irradiation. Pituitary apoplexy causing spontaneous remission of acromegaly was therefore diagnosed.\nBecause the laboratory evaluation did not reveal any endocrine abnormalities, no specific treatment was initiated. The severity of headache reduced significantly during the course of the patient's hospital stay, and he was advised to take oral analgesic paracetamol when needed. He was counseled regarding the need for close follow-up to detect hypopituitarism and was discharged with a plan to repeat pituitary function and neuroimaging at an interval of 3 to 6 months. |
A 52-year-old woman presented at a local hospital’s emergency department after being discovered by her family with sudden-onset vomiting and loss of consciousness. Head CT showed bilateral basal ganglia hemorrhages (data not obtained). The patient was immediately transferred to our hospital. Vital signs on arrival were unremarkable except for markedly elevated blood pressure (180/100 mm Hg). The patient had a 2-year history of hypertension without any history of diabetes, trauma, surgery, or taking oral anticoagulant drugs. Her Glasgow Coma Scale (GCS) score was 6 (E1V1M4). Neurologic examinations revealed coma, quadriplegia, and bilateral positive Babinski sign. Bilateral pupils were 3 mm but sluggish to light. Head CT showed bilateral basal ganglia hemorrhages of about 18 mL on the right and 27 mL on the left side (). Hematoma volume was calculated using the ABC/2 method. Chest CT showed bilateral mild pleural effusion and infection in bilateral lungs. Laboratory tests were within normal ranges including complete blood cell counts, bleeding time, activated partial thromboplastin time, prothrombin time, liver and renal function, and blood glucose level. We decided to remove the hematomas by aspiration to reduce intracranial pressure (ICP). We pasted CT markers on the frontal and temporal areas of the patient’s head for head CT to indicate the puncture points for aspiration (). The patient later underwent MIS for ICH under general anesthesia. The neurosurgeon performed hematoma catheter injections using a standard sterile technique. The catheter diameter was 4.8 mm. Frontal and temporal catheters were placed, and 10 and 5 mL of blood clots were aspirated from the left and right sides, respectively. Immediate postoperative head CT confirmed the catheter track and residual hematomas ( and ). The patient was sedated and transferred with an endotracheal tube and mechanical ventilation to the Neurosurgical Intensive Care Unit.\nUrokinase injection and clot aspiration were performed for the residual hematomas. On postoperative day 1, the 2 catheters were clamped after infusion of urokinase (20,000 U dissolved in 3 mL normal saline) for 2 h. However, at 1.5 h, the patient exhibited the Cushing reflex and bilateral pupils were 5 mm and unresponsive to light. We opened the catheter valve, which alleviated the patient’s signs and symptoms. GCS score improved, and the patient started responding to commands starting from postoperative day 2. We infused urokinase every 12 h up to 4 doses instead of performing a single injection, and the patient was stable during the process. On postoperative day 3, head CT showed a residual clot (10 mL on the right and 2 mL on the left side) and the patient was extubated ( and ). Human albumin and furosemide were administered to control ICP and cerebral edema. The patient’s pneumonia continued to worsen after the operation, requiring assisted ventilation. She underwent tracheotomy by general anesthesia on postoperative day 10. On postoperative day 19, the ventilator was removed and head CT revealed absorption of the intracranial hematoma ( and ). Over the 2 years of follow-up, there was no recurrence of ICH. The non-fluent aphasia improved but speech did not return to normal. The patient suffered mild disorder of the left and right limb muscles, but she was able to walk unassisted. The patient’s modified Rankin Scale score was 3 on postoperative day 90 and 2 at the 2-year follow-up. This case was approved by the medical ethics committee of the First Affiliated Hospital of China Medical University to publish the case details. Written, informed consent was obtained from the patient for publication of case details and accompanying images. |
Our patient was a 4-year-old Japanese female who underwent an urgent innominate artery transection under general anesthesia due to a suspected TIF. She was born at 37 weeks’ gestation with a natural delivery. Her birth weight was 2542 g and the APGAR scores were 7 and 8 at 1 and 5 min, respectively. At birth, the patient was immediately intubated due to forced breathing and hypoxemia. Her craniofacial anomalies including exophthalmos, prominent forehead, blue sclera, and micrognathia indicated the possibility of one or more complications of a genetic disease. Radiographic examinations demonstrated advanced skeletal maturation, and she was diagnosed MSS at 2 months old. She underwent tracheostomy for tracheomalacia at 3 months old, followed by gastrostomy at the age of 2 years, but not required ventilator support. Gastrostomy was performed under inhalation anesthesia via tracheostomy tube under spontaneous breathing in combination with regional anesthesia.\nOne month before the patient’s admission to our hospital, intermittent tracheal bleeding was observed and managed by a change in the smaller cannula size and shorter length. Two weeks later, the patient needed hospitalization at a primary medical clinic because of acute tonsillitis and pneumonia-induced hypoxemia. Intermittent tracheal bleeding reappeared, and granulation at the distal site of the cannula without bleeding was detected by bronchoscopy. Contrast-enhanced computed tomography revealed extremely thin connective tissue between the innominate artery and the trachea, suggesting TIF and she was transferred to our hospital (Fig. ).\nAfter admission, the patient’s vital signs were stable; blood pressure 96/57 mmHg, heart rate 143 bpm, and SpO2 97% (room air attached with heat and moisture exchangers). Her height and body weight were 81 cm and 8 kg, respectively. A 4.0 mm ID uncuffed tracheostomy tube of a length that was customized for the patient was used, and air leakage during bag valve ventilation was limited. There was a risk of surgical site infection and postoperative mediastinitis due to the short distance between the cannula and surgical site, but we decided not to change the tracheal cannula because the patient’s hypersalivation and craniofacial abnormality predicted difficult oral intubation, and there was a possibility of rebleeding due to inadvertent granulation stimulation.\nAnesthesia was induced and maintained with midazolam, ketamine, and fentanyl. Rocuronium was administered to prevent coughing during surgery. In addition to the standard ASA monitoring, the invasive blood pressure (left radial artery), central venous pressure (CVP) (right femoral vein), and regional cerebral oxygen saturation (rSO2) at the sites of the head and back were monitored during anesthesia. No specific response occurred during the induction of anesthesia; blood pressure 80/43 mmHg, heart rate 145 bpm, SpO2 100%, CVP 5 mmHg, rSO2 values 95/53, and pharyngeal temperature 36.5 °C. Bronchoscopy after the induction of anesthesia showed a white ulcer lesion at the anterior trachea (Fig. ).\nThe patient was first placed on pressure-controlled ventilation, but the end-tidal CO2 increased to around 60 cmH2O after rocuronium administration because of air leakage through the vocal cords. Although a tube exchange to a cuffed tube was considered, there was no cuffed tracheostomy tube that would fit her, and it was difficult to perform even the oral bronchoscopy due to the limited oral space. We thus placed oral pharyngeal gauze to decrease the air leakage, and the patient's end-tidal CO2 then gradually decreased to around 45 cmH2O.\nMedian sternotomy was performed followed by clamming of the innominate artery at the proximal and distal sites in contact with the trachea. Although the regional cerebral oxygen saturation temporarily decreased by 10% from the control value when the innominate artery was clamped, other vital signs were nearly stable and the innominate artery was transected without any graft replacement.\nA bronchoscopy after the operation revealed no evidence of active bleeding in the patient's trachea, and she was transferred to the intensive care unit (ICU). To prevent surgical-site bleeding and unexpected movement, 0.2 mg/kg/h midazolam and 0.4 μg/kg/h dexmedetomidine were administered until postoperative day 1. Spontaneous respiration was detected 1 h after admission to the ICU, and the pharyngeal gauze was then removed. After discontinuation of the sedatives, consciousness recovered to the preoperative level and the ventilation management was also ended. Although frequent aspiration was required due to hypersalivation, no additional treatment for hypoxemia was needed. The patient was discharged to a general ward on postoperative day 2, and she was transferred to her previous clinic on postoperative day 8. |
A 32 year-old woman with a history of hypothyroidism and pre-eclampsia initially presented to an outside hospital with acute onset dense left hemiplegia, right gaze preference, and left-sided neglect. Her initial National Institute of Health Stroke Scale (NIHSS) was 14 and she had an admission Glasgow Coma Scale (GCS) of 10. A computed tomography (CT) angiogram of her neck revealed complete occlusion of the right cervical internal carotid artery (ICA). She was outside the time window for intravenous thrombolysis; however, she underwent mechanical thrombectomy using a stent retreiver device and aspiration (Penumbra System®, Alameda, CA). Immediately after the procedure, there was a successful restoration of the blood flow to the distal ICA, proximal middle cerebral artery (MCA), and to the anterior cerebral artery (ACA), with residual distal M2 occlusion. She was intubated for the procedure and was extubated in the following days. Her left-sided weakness persisted and a repeat CTA showed re-occlusion of the right cervical ICA. No further intervention was done and she was treated with aspirin and statin for secondary stroke prophylaxis. The stroke was deemed cryptogenic after work-up for a potential source was negative including an echocardiogram which demonstrated a normal ejection fraction, normal left atrial size, and negative bubble study. A workup for prothrombotic and hypercoagulable states were negative as well. Magnetic resonance imaging (MRI) of the brain was done which showed a large area of diffusion restriction with corresponding decreased apparent diffusion coefficient (ADC) and T2 hyperintensity in the right frontal, parietal, temporal lobes and in the basal ganglia with areas of hypointensities on gradient echo sequencing, which suggested infarction in these areas with some areas of hemorrhagic conversion (Figure ).\nSubsequently, she was discharged to an inpatient rehabilitation center. While at the rehabilitation center, about four weeks after her stroke, she developed moderate to severe insidious onset headache. A repeat MRI, done four days after the onset of headache, showed diffusion restriction (with corresponding decreased ADC) and a ring-enhancing lesion in the right basal ganglia which involved part of the previous ischemic stroke. An extensive area of T2 hyperintensity was seen around this lesion consistent with vasogenic edema (Figure ).\nWith a recent invasive procedure along with the MRI findings, the possibility of an abscess was entertained, even though she did not have systemic signs of an infection (afebrile, WBC count 7800/mm3, negative blood cultures). She was empirically started on broad-spectrum antibiotics (vancomycin, cefepime, and metronidazole) and admitted to our institute for further management. On day three of admission to our hospital, she developed a high-grade fever and had an acute deterioration in her mentation that progressed to coma. An MRI was repeated to evaluate for any progression of the disease and to obtain stereotactic images for drainage. In addition to the previously mentioned ring-enhancing lesion, the post-contrast sequences now demonstrated enhancement of the right lateral ventricular wall which was suggestive of ventriculitis (Figure ).\nShe underwent a stereotactic drainage of the lesion, which aspirated purulent material. The patient was continued on broad-spectrum antibiotics. Vancomycin was discontinued after 10 days. Cefepime was switched to ceftriaxone, which along with metronidazole, was continued for a total of six weeks. An extensive laboratory workup was done which did not reveal a potential source of infection or immunocompromised state. Due to the high suspicion for an abscess and the purulent aspirate, a bacterial DNA probe was carried out on the aspirate, which revealed the presence of Fusobacterium necrophorum. Since Fusobacterium necrophorum is the implicated organism in Lemierre's syndrome, a surveillance of signs were carried out on the patient but failed to reveal neck pain or thrombosis of the internal jugular vein (imaged with an ultrasound of the neck). On post-drainage day one, her mental status improved and she progressed to her baseline prior to her discharge from the hospital. |
A 29 year old woman was referred with steroid dependent ulcerative colitis diagnosed 2 years earlier. She was treated with prednisolone 40 mg PO daily, azathioprine 150 mg PO daily, mesalazine tablets 2 g PO twice daily and mesalazine foam enemas 4 g PR daily. She was also taking folate 0.5 mg PO daily as she was attempting to conceive. Her TPMT (thiopurine methyltransferase) activity before therapy was normal at 8.5U/mL (7.0 – 14.5). In evaluating her steroid dependency her 6TGN was found to be 134 with 6MMP 5560, giving a 6MMP:6TGN ratio of 41, consistent with shunting. It was judged that an increased azathioprine dose would have lead to a large 6MMP rise (with such a ratio) and probable toxicity, so co-therapy was decided on. The azathioprine dose was reduced to 25 mg PO daily and allopurinol 100 mg PO daily was dosed concomitantly. She declined treatment with a biological agent or entry into a clinical drug trial as she was attempting to conceive.\nShe conceived 15 months later on the allopurinol & azathioprine co-therapy. The prednisolone dose was reduced to 7.5 mg PO daily; the lowest dose she had been on. To avoid a flare during pregnancy the decision was to maintain her at low dose prednisolone. At the time, her 6TGN was 254 and 6MMP was < 500 (6MMP:6TGN ratio ~2). Allopurinol, azathioprine and prednisolone were continued for the duration of the pregnancy and her ulcerative colitis was well controlled.\nA healthy baby boy was delivered by caesarean section at term due to breech position. She elected not to breast feed for personal reasons. Sixteen months after delivery of her first child she suffered a miscarriage at 6.5 weeks pregnancy. Foetal chromosomal studies were not performed. She plans to fall pregnant again in the following year before entering a clinical trial to manage her ulcerative colitis. She continues on combination azathioprine and allopurinol. |
A 21 year-old female undergraduate student presented with a 2 year history of spinal, pelvic and distal thigh pains with an insidious onset. There were no reports of neurogenic-type pain or sensory changes. See Fig. for the body chart.\nThe patient reported a slight weakness negotiating a flight of stairs but she was unsure if she felt weakness in her lower extremities or her trunk. Aggravating factors included walking up more than down stairs, sit to stand transfers and stepping up curbs. In sitting, no symptoms were evident.\nThere was no history of weight change, night sweats or recent infections/systemic illness. Early morning stiffness was described as minimal but lasted 40 min. There was no family history of any significance. The patient recalled always being last at running races as a child but no missed milestones or paediatric input was highlighted. Medication included naproxen and paracetamol as required, taken a few times per week with mild relief. No other medical, drug or mental health history was noted. Attendance at University had not been interrupted. No change to social activities or family relationships were reported due to the symptoms.\nPast treatment included recent private physiotherapy and the patient’s interpretation was that “core exercises” had been initiated. These had been performed twice a day for 2 weeks. Subjectively, pain severity and the sense of strength when negotiating stairs had improved by 10% since starting the exercises. Care was transferred to the authors NHS service as is typical in the UK to enable free provision of assessment and treatment.\nA 9-question tool called The Subgroups for Targeted Treatment (STarT) back screening tool questions patients on matters such as catastrophising, fear, anxiety and depression []. The tool has been advised to identify back pain patients who may have modifiable aspects to their presentation that may benefit from cognitive-behavioural approaches [].\nThe initial STarT back score was 7 with a subscore of 4 (see Additional file ) indicating a high risk factor for spinal pain-related disability []. The score indicates significant psychosocial risk factors for a poor prognosis [] and stratification to physiotherapy services to deal with psychosocial elements would be advised [–]. The EuroQol 5D (EQ. 5D) questionnaire gives health related quality of life and psychometric analysis that has demonstrable construct validity and reliability []. The patients EQ. 5D responses demonstrated severe pain, functional impact and anxiety/depression scores (see Additional file ).\nSubtle spinal rotation and thoracic scoliosis concave to the right was noted on standing. The gait pattern was entirely normal including rope walking tasks. The sit to stand movement pattern was abnormal. A wide base of support was achieved by abducting the hips. The lower limbs were internally rotated and the upper limbs were used to assist rising from the chair.\nDermatomes, reflexes and tone were deemed normal. There were no Babinski or Hoffmann’s reflexes. There was no clonus at the feet. The Romberg’s test was also negative and there was no dysdiadochokinesis. Myotomal examination revealed no focal weakness when examined in isolation, but weakness was apparent during sit to stand and bridging tasks. Single leg stance demonstrated a good ability to maintain pelvic and trunk posture with no Trendelenburg. There was no visible muscle atrophy but a mildly raised BMI may have limited the ability to detect a loss of muscle bulk.\nLumbar spine movements into extension and side flexion were normal and pain free. Flexion was relatively reduced (fingers to mid-tibia) and some spinal pain was reported at the end of range. Rising from flexion involved bracing of the lumbar spine and movement generation from the thoracic region and hips. Neurodynamic tests were unremarkable. Spinal palpation was pain free. The thoracic spine moved well and gave no pain responses. The hips moved well and quadrant testing was normal. Distal joint screening revealed no evidence of inflammation or synovitis.\nAtypical movement patterns were evident but no focal neural concerns on testing in clinic, pointing to either central cord or nerve root pathology, were detected. The initial plan was to monitor and continue exercise-based physiotherapy input especially in light of recent gains from exercise.\nPhysiotherapy focused on functional movement quality with a trunk and lower limb strengthening programme. No formal myotome weakness was detected but the functional challenge when standing and the effortful nature of bridging was enough to warrant a programme to strengthen these tasks. Bridging was the main focus of the physiotherapy programme that involved review and progression of an exercise programme. Sit to stand exercises from a raised position were also encouraged. Reassurance was provided regarding the negative examination findings in clinic as evident fear and anxiety had been displayed in clinic and on the questionnaire responses. A review with the initial Extended Scope Practitioner (ESP) was advised if no further gains were made or any regressions materialised.\nSome subjective gains were reported but objectively there were no gains after four sessions of treatment over a 2 month period. The ESP reviewed again. Isolated trunk and limb strength was re-assessed using myotome testing as well as isometric hip strength testing into abduction and extension. This again proved negative. Bridging remained effortful but the sit to stand movement was the main evident limitation.\nAn MRI of the entire spine was ordered by the ESP to assess the cord/neural health. If this test proved negative the next suggestion was to explore a neurology referral. The MRI hoped to differentiate or rule out the following possible origins to the bilateral lower limb weakness: spinal cord pathology such as compression from intervertebral discs, inflammation or intrinsic tumour, or more rarely a parasaggital brain lesion. The lack of upper motor neuron signs suggested a lower probability of any brain lesions though. Anterior horn cell disease could be ruled out and was low on the hypothesis list as the patient was young and did not have any fasciculations. Spinal canal stenosis and cauda equina change could also be examined but there was no bladder/sphincter symptoms or saddle changes so again the level of concern was low. A primary muscle disorder was another possible origin to be considered.\nBlood tests ordered by the general practitioner had shown no significant change to inflammatory markers, urea, electrolytes or liver function. Repeat tests were scheduled by the general practitioner but did not include creatine kinase. |
A healthy 20-year-old male presented to the musculoskeletal oncology department after incidental diagnosis of a cystic lesion located in the left femoral head (A). The lesion was found incidentally after the patient underwent an MRI of the lower abdomen for suspected appendicitis. Six months after the incidental finding the patient began to complain of left groin pain.\nThe patient had hip pain with flexion abduction and external rotation and a negative FADDIR test. CT scan demonstrated a lobular lesion in the epiphyseal region of the femoral head sparing the articular surface (B). The lesion measured 2.3 × 15 × 1.6 cm. Due to continued symptoms and the subchondral location at the weight bearing zone of the femoral head, hip arthroscopy including biopsy samples, debridement and curettage was suggested. Due to the size and location of the lesion bone substitute was used to fill the cavity.\nHip arthroscopy was first performed with traction for evaluation of possible articular penetration of the lesion. A lateral portal was established under fluoroscopy and then a mid-anterior portal [] was created under arthroscopic visualization with the aid of fluoroscopic guidance. Capsulotomy between the two entry points was performed with a hooked radiofrequency probe (VAPR; DePuy Mitek, Raynham, MA, USA) to facilitate instrument maneuverability. The femoral head was probed and appeared to be intact (C). Traction was then released and the peripheral compartment was inspected. The location of the lesion was identified using fluoroscopy (D). The capsule was further divided in a T shape. A small window on the lateral cortex of the femoral head was created. The lesion was excised using a curette and a shaver to the underlying bleeding bed of bone (E). The cavity was than filled with an osteo-conductive (HA) Bone Paste (BoneSource BVF, Stryker, Mahwah, NJ, USA) (F). The capsule was closed at the end of the procedure and the pathology report confirmed benign enchondroma. Post-operative radiographs at 18 months after surgery showed marked increased density of the femoral head (A and B). The patients MHHS and Visual Analogue Scale score were improved from 74 and 6 pre-operatively to 95 and 0 post-operatively with 30 months follow up. |
A 39-year-old African-American male was admitted (day 1) from a nursing home for a chief complaint of weakness and decreased appetite for several days. He was initially admitted to the medical Intensive Care Unit for suspected sepsis of unknown origin and was started empirically on vancomycin and meropenem. At that time, his urine cultures grew >100,000 colony-forming units (CFU)/mL of Candida glabrata (sensitivities not performed); however, no antifungal therapy was added to his antimicrobial regimen as this was thought to be colonization secondary to his chronic indwelling Foley catheter for urinary retention. He was stabilized and transferred to the general medical floor on day 6 for further management of his sepsis. Subsequent workup on days 10–13 revealed osteomyelitis due to a Stage IV sacral decubitus ulcer with exposed bone. The patient had negative blood cultures, but urine cultures continued to remain positive now yielding 15,000 CFU/mL of Candida albicans (sensitivities not performed) on day 28. This organism was thought to be a colonizer and no treatment was received. The patient was treated for a total of 45 days for his osteomyelitis with a combination of vancomycin and various Gram-negative therapies including meropenem, ertapenem, and ceftazidime.\nConcurrently to the patient's infectious workup, he received a workup for failure to thrive (body mass index 14.7 kg/m2) on day 2 and consequently received a percutaneous endoscopic gastrostomy tube placement on day 12. The patient experienced elevated alkaline phosphatase, aspartate aminotransferase, and alanine aminotransferase throughout his hospital course, primarily attributed to failure to thrive and poor nutritional status. The patient was also found to have extremely brittle diabetes with occurrences of multiple hypoglycemic episodes correctable with 50% dextrose boluses. The patient was started on insulin glargine 2 units every evening, but this produced large fluctuations in blood sugars ranging from 18 to 460 mg/dL, with an average of 233 mg/dL. The patient was switched to neutral protamine Hagedorn (NPH) insulin on day 51 at 2 units twice daily in an attempt to avoid hypoglycemia. It was also found that when the patient was not allowed to eat or drink, his blood sugars would drop to <50 mg/dL unless a 5% dextrose infusion was administered. Glycemic goals for this patient were to avoid hypoglycemia and diabetic ketoacidosis, which was extremely difficult despite switching from insulin glargine to NPH insulin.\nDespite broad-spectrum antimicrobial treatment for osteomyelitis, the patient continued to have leukocytosis. Subsequent blood and urine cultures were drawn on day 34. On day 36, an abdominal computed tomography (CT) scan was ordered to identify other potential sources of infection. The scan demonstrated right-sided pyelonephritis and left-sided hydronephrosis and hydroureter. The patient's antimicrobial therapy was expanded to include micafungin. Urology was consulted on day 37 for further management. A repeat abdominal CT scan on day 38 demonstrated pyelonephritis in both kidneys, as well as filling defects (left >right) and mild debris along the bladder base, indicative of fungus balls/mycetomas. On day 40, urine cultures returned growing 35,000 CFU/mL of C. glabrata (fluconazole with minimum inhibitory concentration [MIC] of 8 and caspofungin MIC of 0.5 mg/L) and C. albicans (fluconazole MIC of 4 mg/L and caspofungin MIC of ≤0.25 mg/L). Blood cultures also returned on day 40 and revealed C. glabrata with a fluconazole MIC of 2 mg/L. The patient was switched from micafungin to high-dose fluconazole based on susceptibilities. In addition, urology recommended drainage of the fungal balls and subsequent irrigation with AmBd via bilateral nephrostomy tubes. A nephrostomy tube was placed in his left kidney on day 45 in an attempt to drain the fungal ball and in preparation for AmBd irrigation. After 6 days of systemic antifungal therapy, the blood cultures cleared; however, a renal ultrasound performed on day 54 was still concerning for continued presence of fungal balls. A lack of improvement in renal impairment was observed, indicated by an estimated glomerular filtration rate of 10-30 mL/min/1.73 m2, which was comparable to prenephrostomy tube placement. The patient eventually required intermittent hemodialysis on day 56. On day 59, the patient was taken to interventional radiology for possible placement of the right nephrostomy tube; however, this placement failed. The decision was then made to discontinue fluconazole and initiate systemic AmBd at 0.7 mg/kg/day (29.3 mg) to treat the renal fungal balls. On day 61, the patient's LFTs significantly worsened []. This was attributed to the AmBd (Naranjo adverse drug reaction probability scale score of 5). The patient's LFTs returned almost to near baseline, suggesting amphotericin B as the culprit of acute liver injury.\nAfter it was discussed with the patient that all medical treatment options had been thoroughly exhausted, the patient decided to change his code status to do not resuscitate. The patient's condition continued to decline, and his daughter decided to stop the 5% dextrose infusion that was required to keep his blood glucose normal. The patient expired after 66 days of hospital admission. |
A three-year-old male patient was admitted with a two-month history of abdominal pain, intermittent fever, asthenia, and adynamia. During physical examination, he presented jaundice, II/IV tricuspid murmur, and generalized swelling. Blood cultures isolated Corynebacterium diphtheriae. Echocardiography revealed a deformed and enlarged tricuspid valve with multiple vegetation in both anterior and posterior leaflets. One of them was as big as to prolapse the right ventricle, as it had a 12 mm diameter and III/IV grade insufficiency with pulmonary systolic pressure of 42 mmHg.\nThe pediatric cardiovascular surgical service opted for performing urgent surgical intervention, given the sepsis persistence and risk for an embolic event due to the vegetation. Surgery was performed in extracorporeal circulation with low hypothermia (32ºC), conducting bypass with Alpha-Stat. Arterial cannulation of ascending aorta and both cavae was done. Del Nido blood-based cardioplegia was utilized for heart arrest. The valve was found to be completely compromised by the infectious process, thus it had to be resected and samples were sent for further examination.\nA tricuspid neovalve was tailored with two rectangular segments of autologous pericardium. It measured approximately half of the tricuspid annulus in width and 1.3 times the distance between papillary muscles and valvular annulus in length. The patches were tacked into the papillary muscles with separated polypropylene 5/0 sutures, then in the tricuspid annulus with a 6/0 polypropylene continuous suture ( and ). To avoid stenosis and promote ventricular filling, four fenestrations as big as 1/3 of the length of the patches were made in the inferior part of both patches. A cold saline solution delivered through the right ventricle demonstrated a competent tricuspid valve.\nOnce the repair was finished, clamp was removed from the aorta. No further rhythm dysfunction ensued, and hemodynamic state was positive. After two weeks of surgery, neither neurological nor cardiovascular complications occurred. An echocardiogram revealed a well-functioning neoprosthesis of autologous pericardium, as well as a satisfactory biventricular function with no presence of abscess or vegetations (). No sepsis or cardiac failure were detected on follow-up after a 12-week postoperative course. |
A 34-year-old woman, 1 week postpartum, presented to our dermatology clinic with an intensely pruritic generalized rash. Two days after delivery of her child, the patient developed an itchy rash on the abdomen. On discharge, she was instructed to follow up with the dermatology department if the rash did not resolve. After leaving the hospital, she reported that the eruption had progressively spread to the buttocks and legs and the itching seemed to be worse. The patient's prenatal course was uneventful. She gained 13 kg during pregnancy, with a prepregnancy weight of 72 kg. A healthy male neonate was delivered by caesarean section at 38 weeks' gestation without complication. The patient's medical history was unremarkable. She was currently not taking any medications, and she reported no known drug allergies. On physical examination, the patient was afebrile and her blood pressure was normal. Examination of the skin revealed erythematous papules and urticarial plaques involving the abdominal striae with periumbilical sparing. Similar lesions were noted on the legs and buttocks (Fig. ). The face, palms, and soles were uninvolved. No vesicles or pustules were noted. Based on the characteristic clinical presentation and disease course, she was diagnosed with PUPPP. She was informed of the safety profile and potential benefits of medications but remained reluctant to use medications during lactation, despite her severe symptoms. AWB injection was then considered for her treatment. Venous blood of 10 mL was drawn from the patient, followed by intramuscular injection of 5 mL of the blood on each side of her buttock. Seven days later, both subjective and objective improvements of symptoms were noticed and she received 1 more session of AWB injection (Fig. ). On follow-up after 12 days, all subjective symptoms had improved, leaving only postinflammatory hyperpigmentation (Fig. ). No complications such as infection, abscess formation, or hematoma were observed at the injection site. |
The patient was a 74-year-old African American female with a past medical history of hypertension, smoking, sarcoidosis, and fibromyalgia. She presented with a painful swelling in her left neck and the symptoms did not improve with antibiotics and oral steroids for 4 months. She was referred for head and neck surgical evaluation. Physical examination demonstrated a 2-cm mobile tender mass inferior to the left earlobe. The overlying skin was unremarkable. Facial movement was intact and no cervical lymphadenopathy was identified. Contrasted CT of the neck revealed a 2.5×1.6 cm oval-shaped, cystic mass with irregular borders within the left parotid gland. The mass demonstrated peripheral enhancement and central hypo-attenuation. The top differential diagnoses by imaging included malignancy and infection. A fine needle aspiration was performed at the outside hospital and inconclusive.\nThe patient was transferred to the Veterans Affairs San Diego Healthcare System for surgery, due to the suspicion of a salivary neoplasm with malignant potential. Left parotidectomy was performed, during which a relatively well-demarcated cystic tumor was encountered within the superficial lobe of the parotid gland. There was no clinical evidence of extra-parenchymal extension, and no difficulty was encountered in dissecting the tumor from the facial nerve branches. The tumor and surrounding superficial parotid lobe were removed en bloc. Several slightly enlarged lymph nodes from Level II were also biopsied for histologic examination. No frozen section examination was requested intraoperatively.\nMacroscopically, the tumor measured 2.8×1.5×1.2 cm in size with an ill-defined boundary. There were cystic and hemorrhagic changes within the tumor. The vast majority of the tumor were sampled and submitted in six tissue blocks, in addition to one block of the uninvolved parotid gland. Microscopically, the tumor had an infiltrative growth pattern with centrally cystic areas and focal necrosis (). The neoplastic cells were exclusively oncocytic, characterized by abundant eosinophilic granular cytoplasm, uniformly round nuclei, and variably-sized nucleoli (). There were rare mucocytes, comprising less than 1% of the neoplasm (). No discernible populations of squamoid (epidermoid), or intermediate cells were noted. A focus of perineural invasion was present as shown in . No mitotic figures or significant lymphoid infiltrates were identified within the neoplasm. The uninvolved salivary parenchyma showed scattered small foci of chronic inflammation and otherwise unremarkable morphology. There were two lymph nodes identified with no involvement by the tumor cells.\nThe infiltrative pattern suggested a malignant neoplasm and the differential diagnoses included oncocytic carcinoma and oncocytic variants of other salivary malignancies, including OMEC. Mucicarmine stain confirmed the presence of rare mucocytes (). p63 immunostaining (Clone: BC4A4, monoclonal, BioCare Medical, Concord, CA, USA) revealed moderate-to-strong nuclear positivity in the oncocytes with a diffuse staining pattern (). p63 were negative in mucocytes (not shown). FISH was performed to detect the MAML2 translocation using a break-apart probe flanking the MAML2 gene at 11q21 locus (Leica DM 6000B, Mayo Clinic, Rochester, MN, USA). The result was nuc ish (MAML2x2)(5MAML2 sep 3MAML2x1) [89/200], i.e. 44.5% of the cells were positive for the MAML2 translocation (). Thus, the overall findings supported the diagnosis of OMEC with exclusive oncocytic morphology. Because of necrosis and perineural invasion, this case was graded as intermediate grade according to Armed Forces Institute of Pathology (AFIP) method.\nBased on the pathologic diagnosis of an intermediate grade MEC and the presence of perineural invasion, the patient received adjuvant radiation therapy postoperatively with a total of 6600 centiGray (cGy) to the parotid bed and 5040 cGy to the ipsilateral neck via volumetric-modulated arc therapy. She remained disease-free 16 months after the surgery. |
A 58-year-old Caucasian man with an extensive history of injection drug use was hospitalized with a diagnosis of infective endocarditis (IE); eight months earlier, he had experienced an episode of IE that was treated surgically with a bioprosthetic valve. Blood cultures taken during the current admission revealed methicillin-resistant Staphylococcus aureus infection. Transesophageal echocardiography revealed a 2 cm linear vegetation on the prosthetic aortic valve and a possible ring abscess without evidence of aortic insufficiency. Therapy with intravenous ciprofloxacin, gentamicin and levofloxacin was initiated.\nOn the third day of hospitalization, our patient experienced an acute onset of left upper quadrant abdominal pain. No history of recent trauma could be elicited. Our patient's vital signs were as follows: temperature 40°C, blood pressure 85/48 mmHg, heart rate 104 beats/minute, respiratory rate of 20 breaths/minute, and an oxygen saturation of 99% breathing room air. On physical examination our patient was found to be in mild distress and diaphoretic with dry mucous membranes. The skin of the upper and lower extremities was indurated at sites of frequent subcutaneous injections, but without erythema, exudate or abscess. No petechiae of the skin or nail beds were identified. A cardiac examination revealed a III/VI crescendo/de-crescendo systolic ejection murmur radiating to the apex and to the carotid arteries. An abdominal examination revealed a distended abdomen and tenderness to palpation in the left upper quadrant. A computed tomography (CT) scan of the abdomen and pelvis revealed an enlarged and fractured spleen with surrounding hematoma, but no evidence of liver laceration, infarct or intra-abdominal abscess (Figure ).\nOur patient underwent an emergency splenectomy. A subcostal incision revealed approximately 2L of blood inside the abdominal cavity, an enlarged spleen with grade IV disruption (Figure ), and an aneurysmal splenic artery. The spleen was freed at its attachments and the arteries and veins at the hilum were ligated. The aneurysmal splenic artery was dissected medially for sufficient proximal exposure and was then ligated. Abdominal exploration revealed no further gross pathological findings. A histopathological evaluation of other organs for evidence of amyloidosis was not performed. Our patient's early post-operative recovery was uneventful. However, he later died from complications of endocarditis and ring abscess.\nWhen the gross spleen was sectioned along its short axis in the operating room (Figure ) no abscess was found. Microbiological examination revealed few polymorphonuclear leukocytes (PMNs), many red blood cells, and rarely S. aureus bacteria. A pathological examination revealed very few PMNs and none of the acute inflammation expected in cases of splenic infarction. The splenic red pulp was almost totally replaced by plasma cells. Staining for amyloid was strikingly positive (Figure ). Characteristic talc crystals were observed inside the splenic parenchyma (Figure ). |
A 59-year-old European man crashed his car into a concrete dam (Fig. ). Bystanders attending to the accident found him in cardiac arrest and started cardiopulmonary resuscitation (CPR) immediately. Sufficient CPR efforts were continued until the emergency services had arrived. The first recorded heart rhythm was ventricular fibrillation (VF). On inspection, no signs of injury were immediately visible and no skid marks were found. CPR was continued by physician-staffed emergency medical services (EMS) according to the current advanced life support (ALS) guidelines []. Return of spontaneous circulation (ROSC) was achieved after 30 minutes. He remained unconscious without any sign of muscular activity. He was intubated, mechanically ventilated, and treated with catecholamines during and post CPR.\nAlthough the car was severely damaged, the prehospital physician deemed a traumatic cause for out-of-hospital cardiac arrest (OHCA) unlikely. Based on findings indicative of myocardial ischemia in a post-ROSC electrocardiogram (ECG), acute coronary syndrome was suspected as the etiology of cardiac arrest. After telephone consultation with the trauma leader of the regional trauma center, the patient was transported to the trauma center with percutaneous coronary intervention (PCI)-capability primarily within 120 minutes of the accident.\nOn arrival at the trauma center, the patient appeared clinically stable. His heart rate was 65 per minute, systolic blood pressure was 150 mmHg, oxygen saturation measured by pulse oximetry was 94%, and body temperature was 34.2 °C. Signs of myocardial ischemia were found in the ECG (Fig. ). His pupils were found to be equal, round, and reactive to light.\nAfter primary evaluation in the emergency room a whole-body CT scan revealed findings listed in Table . An MRI scan (Fig. ) of his head and neck was obtained immediately due to the severity of the CT findings. Additional findings in the MRI scan are summarized in Table .\nThe medical and social history of our patient were provided by his family. Subjective overall health assessment found the married man, who was a father and grandfather, to be in good health. He had suffered a fall leading to a fractured scapula 8 years before this accident, which was treated non-operatively. Two years ago, he was assessed for suspected coronary heart disease by a specialist in cardiology, who could not substantiate this suspicion.\nHe was transferred to the intensive care unit (ICU) for further treatment. Halo fixation was installed because only ligamentous structures were disrupted in this case. This procedure is common and adequate in AOD when no cervical spine fractures are present [].\nDue to several episodes of severe bradycardia, transient transvenous pacing was conducted. Cardiac diagnostics showed an ischemic cardiomyopathy with recurrent episodes of ventricular tachycardia. Assessment via echocardiography was performed in the trauma room, 3 weeks and 2 months after the accident and revealed akinesia of the left anterior descending coronary artery (LAD) region and hypokinesia of the inferior wall after a suspected myocardial infarction and VF. Early coronary angiography could not be performed due to severe brain injuries.\nAlthough he was initially assessed to have a poor neurological prognosis from the perspective of the neurologists and neurosurgeons because of his severe brain injuries, he could be discharged from the ICU after 23 days; he was responding to verbal contact and was able to move all his extremities.\nAfter 23 days of treatment at the trauma center he was transferred to a hospital close to his home. Further in-patient treatment was continued by local protocol for further 33 days (timeline in Table ).\nHe was discharged to a neurological rehabilitation facility, where care and rehabilitation efforts were continued with great success. Three months after the incident the tracheostomy was surgically closed.\nCoronary angiography was performed 4 months after the primary event and revealed no coronary artery disease. Subsequently, he had to wear a life vest due to arrhythmia. He was defibrillated once by the LifeVest® 3 months after the trauma during his stay at the neurological rehabilitation facility. Finally, 6 months after wearing the life vest an implantable cardioverter-defibrillator (ICD) was installed.\nSix months after the trauma, he was fully conscious, spontaneously breathing, independent of help in everyday life, and mobile with walking crutches. However, he was unable to swallow granular feed due incomplete bilateral paresis of the hypoglossal nerve. His neurologic status is continuously improving; treating neurologists attested a high potential of restitution. |
We present the case of a 48-year-old male, who was evaluated by the medical genetics service because he had noticed weakening of his voice with a high pitch since age 35, associated with premature graying since his 30s and skin lesions since about the age of 40. At the age of 32, bilateral cataracts were diagnosed and at 44 he was diagnosed with diabetes mellitus, currently on oral hypoglycemic agents. Additionally, he has hypothyroidism and hypertriglyceridemia in management and calcification of the Achilles tendon. Patient endorses lack of an early adolescent growth spurt; however, final stature is similar to his other 3 siblings (164 cm). Patient reports he had no child by choice.\nPatient is product of the union of consanguineous parents (second cousins) and has a 49-year-old brother with similar clinical characteristics, including voice changes since the age of 28, bilateral cataracts at age 29 (subsequently presents complications from corneal ulceration and is currently legally blind), and premature graying since age 33, moreover, scleroderma-like skin changes since his 30s and diagnosis of type 2 diabetes mellitus at age 35. His brother also endorses no child by choice. No other complications such as atherosclerosis, dyslipidemia, hypertension, osteoporosis, or tumors were reported.\nUnfortunately, patient's brother and parents declined genetic testing. There are no other relatives with clinical suspicion of WS.\nPatient states maternal aunt has unspecified type leukemia and father with a history of acute myocardial infarction at age 65 and a diagnosis of melanoma at age 85. Maternal uncle diagnosed with lung cancer at age 72 and maternal grandfather with prostate cancer diagnosed at age 73.\nOn initial physical examination, he appeared much older than his age with “bird-like” facial appearance, beak-shaped nose, and bilateral cataracts, his voice was high-pitched and his hair and eyebrows were scarce and markedly gray. He had thin upper limbs with decreased subcutaneous fat and truncal obesity (). Moreover, we found short stature, hypogenitalism, lower limbs with markedly atrophied skin and subcutaneous fat, abnormal pigmentation of the skin and hyperkeratosis, and flat feet (Figures and ).\nWRN gene sequencing identified the homozygous variant NM_00553.4: c.2581C>T (NP_000544.2: pGln861Ter). WRN gene sequencing report can be found in Supplementary . This variant generates a stop codon at position 861 and has been classified as pathogenic and previously described in homozygous status in a Caucasian patient from the United States in 2006 [].\nLaboratory findings included normal renal function, high blood glucose (164 mg/dl), elevated glycosylated hemoglobin (9.4%), and elevated triglycerides (324.6 mg/dl) with normal cholesterol (162.4 mg/dl). EKG showed an elevation of the J point by early repolarization. Abdominopelvic CT-scan showed bilateral renal cysts, small umbilical hernia, and no fatty liver. Testicular ultrasound showed decreased bilateral testicular volume mainly left side.\nRegular screening for malignancies is recommended for patients with WS, due to the high risk of early-onset neoplasms. Also, it is very important to rule out cardiovascular and metabolic diseases during the follow-up of these patients. Our patient is still under periodic clinical observation and follow-up. Currently, he is on treatment with oral hypoglycemic agents for DM2 with adequate glucose control and in treatment of hypertriglyceridemia. Until now no signs of atherosclerosis or cardiovascular disease have been detected. However, he was recently diagnosed with refractory cytopenia with multilineage dysplasia, a form of myelodysplastic syndrome, which has required multiple transfusions.\nAccording to a clinical history, the patient's brother is being monitored for inadequate control of diabetes mellitus and severe skin lesions that have been difficult to treat, but no cancer has been documented. |
A 43-year-old male patient reported to the Department of Prosthodontics in our dental college in Chennai, India with a chief complaint of missing teeth in the right upper back tooth region for a duration of six months. He also complained of difficulty in mastication as well as esthetic problem. There is no relevant past medical history and past dental history revealed that the patient had undergone extraction of the badly carious tooth in left maxillary first premolar and first molar three months back. On intraoral examination we found missing left maxillary first premolar and first molar with second premolar acting as a pier abutment (Figure ).\nDifferent treatment options were discussed with the patient, and with the patient’s consent, we decided to rehabilitate the edentulous space using nonrigid connector in the distal aspect of the pier abutment. The following clinical steps were carried out for oral rehabilitation. The patient preferred metal ceramic restoration. Hence tooth preparation was done on left maxillary canine, second premolar, and second molar (Figure ) with equigingival margins and shoulder finish line incorporated in the preparation for better outcome of the restoration.\nThe gingival retraction was done with gingival retraction cord and final impression was made using elastomeric impression material with two-stage putty wash technique. Interocclusal record was made using bite registration material to obtain good occlusion of the patient. Provisional temporary restoration was given using tooth colored auto polymerizing resin and was cemented using noneugenol temporary cement. Type IV dental stone was used to pour cast. After the material was completely set, it was retrieved and die cutting was done and the die pins were placed. Master cast was then mounted on an articulator with the help of interocclusal record.\nWax pattern (Figure ) was fabricated in the laboratory on the maxillary left canine, first premolar, and second premolar with a female prefabricated attachment on the distal aspect of pier abutment. The pattern is invested, burned out, and cast. After the casting has been cleaned and pickled, any part of the keyway portion of the attachment that protrudes above the occlusal surface is carefully cut off.\nSurveying was done using Ney surveyor which helps in checking the position and parallelism of female and male component placed within the contours of the pier abutment. Male pattern was later retrieved from the female pattern, so that the female pattern is free of wax in the recess area. After the casting is recovered from the investment, the mandrel and any excess on the top portion of the key are carefully reduced so the key and keyway were in flush with each other.\nAfter casting, metal trimming was done and extension of female pattern was cut accordingly. Later metal try-in of the anterior segment (female part) was done in the laboratory (Figure ) in the working cast to check the marginal fit of the casted metal units.\nThe posterior segment (male pattern) was seated in the casted female portion, then wax pattern was fabricated for left maxillary first molar and second molar. Similar casting procedures were carried out (Figure ) and the marginal fit was checked in the laboratory for the posterior segment also.\nBoth male and female portions were placed and the metal fit was checked in the lab (Figure ).\nMetal try-in of both anterior and posterior segment was done clinically to verify properly the marginal fit of the restoration (Figure ). Then it was subjected to ceramization after shade selection.\nCompleted five-unit FPD is shown in Figure . Anterior segment with female portion (keyway mortise) and posterior segment with male portion (key tenon) were assembled together in the working cast completing the laboratory procedure (Figure ).\nClinically, three-unit segment containing the left maxillary canine, first premolar, and second premolar (pier abutment) with keyway on its distal aspect was cemented (Figure ) first followed by cementation of posterior two-unit segment containing left maxillary first molar and second molar with key on the mesial contour of the first molar (Figure ). Cementation was done using Type I glass ionomer cement.\nThen the excess cement is removed and the occlusion was verified using articulating paper. The patient was advised to use dental floss regularly to maintain good oral hygiene and interdental brush if needed. The patient was reviewed after one week (Figure ) to further evaluate the occlusion and oral hygiene and the restoration was a successful one. |
A 56-year-old right-handed man with secondary education was admitted to our hospital complaining of visual deficit that had gradually progressed over 6 years. Initial presentation 6 years prior included misplacement of his belonging such as keys and wallet; however, as his daily life activities were not affected and he maintained independence, he continued driving and working full time. The symptoms were not sufficiently severe for the patient to seek treatment at that time. Four years ago, he started experiencing confusion at home, which led him to enter the wrong rooms; he urinated anywhere as he was unable to locate the bathroom. As the symptoms worsened, he could no longer find a cup on the table immediately in front of him and gradually developed difficulty in writing in a given area on a piece of paper. He could not track sentences in the newspaper and started listening to the radio instead of watching television. He subsequently experienced problems in performing calculations, dressing himself, and recognizing faces. He had to cease working and driving because of difficulties in identifying common objects. On the other hand, his ability to recognize different sounds and voices was preserved, and he did not experience any hallucinations, weakness, extrapyramidal symptoms, or ataxia. There were no significant events in his medical history. By probing his family history, it was revealed that his mother suffered from mild cognitive decline at 80 years old, while his father did not have any neurologic dysfunction.\nNeurologic examination revealed no cranial nerve, motor, or cortical sensory impairment. The patient's performance in neuropsychologic tests () showed visuospatial deficits, prosopagnosia, color agnosia, and simultanagnosia, memory deficits, dyscalculia, agraphia, and alexia. The patient had difficulty completing some tests such as the trail making, line bisection, and word reading tests, as he was unable to recognize the figures. Language and executive functions were less affected. The patient was aware of his visual deficits and became silent and withdrawn as the disease progressed.\nRoutine laboratory test showed that blood and urine parameters, serum chemistry, and liver and renal functions were within normal limits. Lumbar puncture revealed normal cerebrospinal fluid pressure and biochemistry. Ophthalmologic examination found no abnormalities in the fundus or in intraocular pressure, while visual field and visual acuity tests were not completed because of non-cooperation. Brain MRI showed marked diffuse cortical and subcortical atrophy in bilateral parietal and occipital lobes. [18F]FDG PET revealed significant hypometabolism in diffuse regions of bilateral temporal, parietal, and occipital lobes, prominently in occipital lobes, and no metabolic changes were observed in frontal lobes, basal ganglia, and thalami; [18F]PI2620 PET showed increased tracer uptake throughout the brain, especially in temporal, parietal, and occipital lobes, while standardized uptake value ratios (SUVRs) were 3.8, 4.0, and 4.3, respectively, using cerebellum as the reference region, in close correspondence with the regions that showed the most prominent hypometabolism; [18F]AV45 PET was positive with amyloid deposited throughout the cortices (). Whole-exome sequencing identified the p.Pro354Leu (c.1061C>T) variant of the microtubule-associated protein tau (MAPT) gene. The apolipoprotein E genotype was ε3/ε4. No other variant was detected in genes associated with early-onset AD [amyloid precursor protein (APP), presenilin 1 (PSEN1), and PSEN2]; frontotemporal dementia and parkinsonism linked to chromosome 17 [granulin (GRN), fused in sarcoma (FUS), TAR DNA-binding protein (TARDBP), charged multivesicular body protein 2b (CHMP2B), valosin-containing protein (VCP), etc.]; and other degenerative diseases. There was no expansion detected in Chromosome 9 open reading frame 72 [C9ORF72]. Genetic testing and neurologic evaluation were also proposed to the patient's parents, but they refused consent.\nThe patient was diagnosed with PCA and received treatment with donepezil 10 mg/day and memantine 10 mg/day; additionally, psychoeducation and home-based cognitive retraining tasks were assigned to the patient and his family members, but the symptoms deteriorated.\nThe clinical study protocol and informed consent forms were approved by the ethics committee of Xuanwu Hospital of Capital Medical University, China. Written informed consent was obtained from the patient and his legal guardian for [18F] PI2620 PET, genetic testing, and the publication of this case report (including all data and images). |
A 59-year-old white female with chronic hepatitis C presented to our endoscopy suite for a percutaneous liver biopsy. The patient had been diagnosed with hepatitis C virus genotype 1a 5 years prior and had initially been treated with a 48-week course of pegylated interferon alfa-2a (Pegasys; Genentech USA, Inc.) and ribavirin. Unfortunately she had relapsed within 6 months of stopping antiviral therapy. A percutaneous liver biopsy at that time had demonstrated active chronic hepatitis. The patient had failed a second course of antiviral therapy with pegylated interferon and ribavirin. She had subsequently developed diffuse arthralgias attributed to hepatitis C virus-related cryoglobulinemia. After discussing the risks and benefits of protease inhibitor-based antiviral therapy, the decision was made to perform a surveillance percutaneous liver biopsy for restaging of her disease.\nThe patient's home medications included valsartan 160 mg daily for hypertension, lansoprazole 30 mg every morning for dyspepsia, simvastatin 10 mg daily for hyperlipidemia, and lorazepam as needed for anxiety. She was also taking trazodone 100 mg at bedtime for insomnia and duloxetine 60 mg daily for depression. She reported stable control of her symptoms with these medications for several years without any adverse drug interactions. She had medication allergies to sulfa-containing compounds, acetaminophen and erythromycin. Before the procedure the patient was without any noted complaint. She was afebrile with normal vital signs (blood pressure of 120/78 mm Hg and pulse of 71 beats per minute). A percutaneous liver biopsy was performed without immediate complication using sterile technique and ultrasound guidance. The patient's vital signs remained stable during the procedure, and she was sent to the recovery suite for continued observation.\nApproximately 10 min post-procedure, the patient developed intense right-sided abdominal and chest pain. On re-evaluation, her cardiopulmonary and abdominal examinations were benign. Chest radiography showed no evidence of pneumothorax or free air, and laboratory testing was unremarkable for acute anemia, renal insufficiency, acidemia or transaminitis. She was medicated with 50 μg of intravenous fentanyl, which did not alleviate her pain. She was then administered an additional 50 μg of fentanyl divided in two separate doses. Approximately 5 min after receiving the fentanyl the patient became agitated, diaphoretic and hypertensive. She displayed pressured speech and was in a moderate amount of distress. She was breathing spontaneously at 25 breaths per minute with a blood pressure of 198/106 mm Hg, a pulse of 82 beats per minute and an oxygen saturation of 98% on room air. Serial physical examination revealed profound flushing, diaphoresis, a soft abdomen with increased bowel sounds and mild rigidity in all four extremities. 2 mg of intravenous lorazepam was administered with marked improvement in her vital signs and symptoms. She was admitted to the medical intensive care unit for close observation where she was made nil per os, and computed tomography imaging of her chest, abdomen and pelvis demonstrated no complications from the biopsy. She received intravenous fluids, anxiolytics and pain control with hydromorphone with good effect.\nThe patient remained afebrile during her brief hospital stay, with gradual resolution of her agitated state and widespread pain within 24 h. Her blood pressures demonstrated improvement, but did not return to baseline. She was resumed on valsartan and tolerated advancement of her diet. Although no specific etiology for her abdominal and chest pain was found, review of the patient's medications suggested a possible interaction between the recently administered fentanyl and her antidepressants duloxetine and trazodone. Considering the presentation, a diagnosis of serotonin syndrome was made, and fentanyl was added to her medication allergy list. Since her depression and insomnia had been stable on duloxetine and trazodone without any previous adverse interactions, she was discharged from the hospital with instructions to restart these antidepressants. The patient was seen in follow-up 1 week after her hospital discharge and her physical exam revealed normal vital signs and no neurologic findings. Her liver biopsy results eventually showed mild inflammatory activity with fibrosis. |
A 60-year old man from Sri Lanka presented with a history of sudden onset breathlessness and right sided pleuritic chest pain. 5 days before he had developed a painful swelling of his right leg while he was being treated in the hospital for recurrent episodes of ENL. 3 years ago he was diagnosed to have lepromatous leprosy following a positive slit skin smear for mycobacterium leprae. He was commenced on World Health Organization (WHO) multi-drug therapy (MDT) regimen for multi-bacilary (MB) leprosy, which was complicated by type II lepra reaction (ENL) with right sided panuveitis and treated with oral prednisolone 30 mg/day with gradual tapering to 10 mg/day. He continued to develop recurrent episodes of ENL reactions since the completion of MDT-MB which was managed only with prednisolone. 3 months before the current admission he was started on a second course of MDT-MB treatment for new skin nodules with a positive slit skin smear for mycobacterium leprae. 3 weeks before he was commenced on thalidomide 100 mg twice daily in addition to oral prednisolone of 15mg per day for refractory ENL. He did not have recent major surgery, prolonged immobilization or recent diagnosis of any malignancy. There was no history suggestive of past venous or arterial thrombosis and tendency to develop thrombosis in his family.\nOn examination he was dyspnoeic at rest without central cyanosis. He had leonine facies and generalized erythematous skin with multiple nodules. His right leg was swollen with pitting ankle oedema (figure ) while having normal arterial pulses. His respiratory system examination revealed a respiratory rate of 30 per minute and signs of right lower zone pleural effusion. He had tachycardia with a rate of 110 beats per minute, blood pressure of 110/70 mm Hg and a normal jugular venous pressure. Neurology revealed a glove and stocking sensory peripheral neuropathy. There was no organomegally noted on abdominal examination.\nDuring initial evaluation a venous doppler ultrasound of the lower limb confirmed a deep vein thrombosis in the right femoral vein. Subsequently a computed tomography pulmonary angiography (CTPA) confirmed pulmonary emboli in the right and left main pulmonary arteries with right lower lobe lung collapse and a mild pleural effusion (figure ). His initial blood counts showed a mild anaemia, a normal white cell and differential count and thrombocytopenia of 90k/mm3 which normalized following withdrawal of thalidomide. His liver and renal function tests were within normal limits.\nHe was started on enoxaparin and warfarin concomitantly and the target INR of 2.5 was achieved on day 7. Thalidomide and anti leprosy therapy were stopped temporarily while low dose corticosteroid being continued. After one week his dyspnoea and general condition improved. |
A 40-year-old woman presented with a slowly growing asymptomatic mass over the scalp in the right parieto-occipital region. Clinical history revealed that there was no trauma or fracture in the past. There was no history of headache or convulsion. The patient did not have weight loss or loss of appetite. Her menstrual cycles were normal. The mass had been progressively increasing in size over the last 4 years. On examination, the swelling measured approximately 2 cm × 2 cm, was firm, slightly fixed, nontender, and covered by normal skin []. It was non-fluctuant. There was no discharge or punctum associated with the swelling. There was no evidence of cervical lymphadenopathy on palpation. Further systemic and bilateral breasts examination revealed no abnormality in any of the systems.\nX-ray of the skull showed bony calvaria with no evidence of any bony abnormality or fracture. Laboratory investigations revealed normal hematological parameters such as complete blood counts and peripheral smear examination. Biochemical tests such as thyroid, kidney, and liver function tests, as well as lipid profile of the patient were normal.\nFNAC was performed and blood mixed cellular aspirate was obtained. Papanicolaou and Giemsa stains were applied. Smears prepared showed high cellularity comprising of clusters, sheets, whorls, and singly scattered epithelial cells. These cells showed mild pleomorphism and were round to oval with eccentric nuclei, fine granular chromatin, and moderate amount of cytoplasm [Figure ]. Few of the cells showed intranuclear inclusions []. Many tight whorls of such cells were also noted [, inset]. No necrosis or any mitotic figures were seen, and the background showed blood. Finally, an impression of cutaneous meningioma was made based on the cytomorphological features. The patient was advised radiological investigations and neurosurgical intervention.\nCECT brain showed evidence of soft tissue enhancing swelling over the right parieto-occipital region []. Bony calvaria showed underlying erosion in the corresponding region, which was not detectable on X-ray of the skull. Underlying brain parenchyma, bilateral gangliocapsular region, posterior fossa, and falx midline all appeared normal. Based on the radiological findings, surgical excision of the mass was planned and pre-anesthetic checkup was advised. The tumor was surgically excised and sent for histopathological examination. The mass was completely excised with the intact capsule, and there was no intraoperative bleeding or any other complication.\nGrossly, a single circular fibrofatty firm soft tissue mass measuring 3.5 × 3 × 0.3 cm was received. Multiple sections showed fibroadipose and fibrocollagenous tissue with interspersed whorls, nests, and syncytial sheets of meningothelial cells [Figure and ]. An occasional focus of microcalcification was also noted [, inset]. Immunohistochemical examination revealed positivity for vimentin, epithelial membrane antigen (EMA), and S-100 [Figure –].\nBased on the radiological, cytomorphological, and histological features in conjunction with immunohistochemical examination, a diagnosis of cutaneous meningioma (cutaneous meningothelial heterotopia) was made. |
A 55-year-old Caucasian woman presented to our hospital with a new onset dyspnea on exertion, lower extremity swelling, and orthopnea. Her electrocardiogram (ECG) showed sinus rhythm and a complete LBBB. She underwent a transthoracic echocardiogram (TTE) that revealed left ventricular (LV) enlargement and severe LV systolic dysfunction. She had no history of hypertension or alcohol abuse, and the results of her serology evaluation were negative for Chagas. A multislice computed tomography scan showed no lesions in the coronary arteries. She was diagnosed with idiopathic dilated cardiomyopathy.\nPharmaceutical treatment was initiated and optimized to reach the target doses of carvedilol, enalapril, spironolactone, digoxin, and furosemide. While the patient showed clinical improvement, she remained symptomatic after several months of optimized doses of these treatments. She subsequently experienced clinical worsening, remaining in New York Heart Association (NYHA) functional class III to IV. She was then admitted for implantation of a biventricular pacemaker.\nDuring hospitalization, she showed rapid clinical improvement after reversal of the LBBB. An ECG showed sinus rhythm and a QRS complex interval of 110 ms. The medical team opted for not implanting the pacemaker at that time.\nHowever, the patient returned 10 days later, presenting with clinical deterioration in NYHA functional class III. The ECG showed LBBB once more. She also brought the TTE performed on the day of discharge revealing LV enlargement with LV end-diastolic dimension of 67 mm, LV end-systolic dimension of 61 mm, LV end-systolic volume of 155 ml, an ejection fraction (EF) of 19% (by Simpson’s method), and Grade III diastolic dysfunction. We also noted a wide QRS complex in the ECG signal obtained during the TTE. Implantation of a biventricular pacemaker was scheduled.\nThe implantation of the biventricular pacing was performed uneventfully, with the LV lead positioned through the coronary sinus in the posterolateral wall. During the procedure, we observed alternating narrow QRS complex intervals and LBBB. This allowed the electrode positioned in the posterolateral wall of the LV to capture the ECG of a narrow QRS sinus rhythm of 110 ms and LBBB (i.e., the QRS complex time was 160 ms). We documented an increase in the LV electrical delay. The interval time was defined as the time from the first deflection on a surface ECG to the local intrinsic activation at the LV stimulation site (Q-LV) after the installation of LBBB (Figure ).\nThe patient showed clinical improvement after the pacemaker implantation, remaining in functional NYHA class II. We performed an echocardiogram to measure various parameters under three conditions: biventricular pacing (with a heart rate of 65 beats/minute), sinus rhythm with a narrow QRS complex, and LBBB pattern (Table ).\nThe sinus rhythm with a narrow QRS is the underlying rhythm, which we obtained after inhibiting the pacemaker. The LBBB pattern was obtained after atrial pacing at a rate of 75 beats/minute (the LBBB was rate-dependent).\nThe LVEF remained unchanged for each condition measured. There was a clear increase of the pre-ejection period (PEP), isovolumetric contraction time (ICT), and myocardial performance index in the presence of LBBB when compared to biventricular pacing and narrow QRS. We also observed abnormal interventricular septum motion during LBBB, which reversed at biventricular pacing or sinus rhythm with narrow QRS (Figure ).\nThe patient presented good clinical function and echocardiographic response after the intervention. An echocardiogram done eight months later showed an increase in LVEF to 34% compared to the value of 19% before pacemaker implantation and an end-systolic volume reduction to 141 mL (before pacemaker implantation=155 ml). These parameters have been measured by Simpson’s method. |
A 38-year-old man visited our hospital complaining of anterior chest pain. He had no significant medical or family history, and the vital signs were stable. Ischemic events were not observed in electrocardiography, but chest X-ray and computed tomography (CT) showed a cystic lesion (6.0 × 7.0 × 10.0 cm) in the anterior mediastinum (Fig. a). Although the cystic capsule demonstrated contrast enhancement, its fluid component had low radiation absorbance. Based on these findings, we suspected the mass to be a thymic cyst. Blood tests indicated the presence of inflammation (white blood cell count 11,200/μL and C-reactive protein 3.38 mg/dL).\nTwo days after hospitalization, the patient developed dyspnea and his chest pain worsened. Subsequent chest CT showed that the cystic lesion had become inhomogeneous and the radiation absorbance of the cyst’s fluid component had increased (Fig. b). The cyst wall became thickened, and bilateral effusion was observed. Blood tests indicated that hemoglobin levels had decreased from 15.8 to 12.8 g/dL, and levels of inflammatory markers had increased, with the fever exceeding 38.5 °C. Needle aspiration biopsy and tumor wall biopsy with a small skin incision were performed; however, we could not obtain a diagnosis. One week after admission, general condition and laboratory data of the patient gradually improved. A chest CT on day 13 showed that the tumor had become small in size with a thickened wall (Fig. c). The effusion on the right side had decreased and that on the left side had disappeared.\nThe patient had recovered enough to undergo surgery; the tumor was resected by sternotomy on day 18. The tumor was found to be encased in a smooth, yellow, and elastic coat. The tumor was densely adhered to the junction of the left brachiocephalic vein and superior vena cava, and it was required to detach the tumor from the dense adhesion site carefully. The right phrenic nerve was preserved, and the right pleural effusion was serous. The tumor and thymic tissue were resected en bloc. The operative time was 288 min, and the estimated blood loss was 521 mL. The resected tumor was covered with a thick, fibrous capsule, and the lumen was filled with necrotic tissue and hemorrhagic material (Fig. a, b). The postoperative course was uneventful, and he was discharged on day 26.\nThe pathological findings showed a fibrotic cyst wall; the cyst was filled with necrotic tissue. The slight proliferation of lymphocytes was confirmed in the necrotic tissue and around the cyst wall (Fig. a, b). The tumor was diagnosed as type B1 cystic thymoma (Fig. c). As the tumor did not appear to have spread beyond the capsule, it was determined to be at Masaoka stage I. Nevertheless, the dense adherence of the tumor to its surrounding tissue indicated the possibility of invasion, and postoperative radiotherapy (50 Gy) was administered.\nTwo years after the surgery, recurrent metastasis of the tumor was found on the right pleura and the left upper lobe of the lung. The patient was treated with chemotherapy, radiotherapy, and local resection. The patient remains alive 12 years after the first surgery. Following an analysis of the tissue obtained from the resected recurrent tumor, the pathological diagnosis was changed to type B3 thymoma. |
A 34-year-old man was admitted to the Surgery Department in our centre in July 2016 with diffuse abdominal pain, dyspnea, general fatigue, and weakness. In his medical history, he had been admitted to another centre two months ago for an episode of acute pancreatitis.\nAbdominal ultrasonography revealed a heterogeneous area of 5 cm in size in the body of the pancreas, peripancreatic fluid, gallstones with thickness in the gallbladder wall, and multiple cysts in the left kidney. Abdominal computed tomography (CT) showed heterogeneous collection of fluid with a thick wall of 12 × 4 cm in size along the body of the pancreas and left colic angle (often an abscess or a pseudocyst) with infiltration of adipose tissue around it and mild thickness at the wall of the colon. A simple renal cyst was also reported in the left kidney ().\nLaboratory investigations were within normal levels except an elevation in C-reactive protein value (18.1 mg/dl) and amylase (765 U/L). The hepatic tests were within the normal range (total bilirubin (TB) was 0.64 mg/dl, alanine aminotransferase (ALT) was 20 IU/L, and aspartate transaminase (AST) was 19 IU/L). The patient was treated as an episode of acute pancreatitis.\nOther investigations were performed; upper gastrointestinal endoscopy (UGI endoscopy) demonstrated an esophagitis (grade A) at the lower esophagus, incompetence of the lower esophageal sphincter (LES), diffuse congestion of the mucous membrane of the stomach, and aphthous ulcer at the fundus of the stomach. Lower GI endoscopy was normal until the terminal ileum.\nA month later, the CT scan for thorax and abdomen data were similar to the previous finding, and the pancreatic cyst measuring 13.5 × 7 cm stretched down through the peritoneal cavity in front of the mesenteric vessels.\nThe laboratory values were normal, and a primary diagnosis of pancreatic pseudocyst was probable, and the decision of surgical intervention was decided.\nIntraoperatively, an extreme oedema in the pylorus, the transverse mesocolon, the head and body of the pancreas, and the hepatoduodenal ligament was found. A cholecystitis required cholecystectomy. After entering the lesser sac, a large mass of 35 × 20 × 15 cm in size was found, located in the space between the tail of the pancreas, spleen, left colic angle, left kidney, stomach, and diaphragm. The mass was hard to dissect from the neighboring structures. In puncture, a clear pure liquid was aspirated proposing the existence of a hydatid cyst. Cyst fenestration was performed, and multiple daughter cysts were evacuated; the endocyst membrane was removed (). A Foley catheter was placed in the residual cavity. The simple renal cyst needs no intervention according to the urologist. The final diagnosis was pancreatic hydatid cyst.\nThe patient had another episode of acute edematous pancreatitis after a month of surgery, and the amylase level was over 1000 U/L. The development of local retroperitoneal abscess required puncture and drainage by CT; the patient also developed a deep venous thrombosis and was treated by anticoagulants.\nDuring 18 months of follow-up, the patient was well with no episodes of recurrence or other complications. |
A 40-year-old male patient reported to our hospital with a chief complaint of facial wound on the left cheek region for past one month, which was initially smaller and increased progressively to the present large size, with appearance of worms in the wound. The patient was mentally challenged and revealed a medical history of epilepsy from childhood.\nOn extraoral examination, the ulcer was 5 cm × 4 cm in size on the left chin region, including lower lip and infiltrating the underlying tissues with an everted and erythematous border. Orocutaneous fistula was present at a point in the base of the ulcer. The wound was tender and firm. Maggots were seen burrowed deep in the wound []. The affected region was swollen, and the swelling extended to the middle of the upper lip. On the left side of the neck, two submandibular lymph nodes were palpable. On palpation, the ulcer was tender and indurate.\nOn intraoral examination, hard tissue revealed partially edentulous space and poor oral hygiene with severe deposits of calculus and stains. On soft tissue examination, a small fistula was seen in the left lower vestibule at the corner of the mouth.\nConsidering the patient's mental status, history of epilepsy, and poor oral hygiene, it was provisionally diagnosed as an ulcer infested with maggots (oral myiasis).\nAbout 15–20 maggots were removed with a tweezer following the application of turpentine oil [Figures and ]. The area was irrigated with saline and betadine solution. The removed maggots were placed in a container, sealed tightly, and disposed off. Surgical debridement of the wound was carried out under local anesthesia []. The patient was prescribed antibiotic (cefotaxime 200 mg bd), and analgesic (ibuprofen) to prevent further infection and to control pain. When the patient was reviewed after a week, the swelling had subsided and wound healing was observed to be satisfactory []. |
A 90-year-old woman was admitted to the emergency department of our hospital because of a syncope episode. A routine blood test showed severe anaemia (Hb 6.6 gr/dl) and physical examination showed no abnormalities but traces of melaena. She had a previous history of nicotinism and ischaemic heart disease with hyperkinetic arrhythmia. She was hospitalized on a medical ward to receive blood transfusions and to undergo more diagnostic examinations. A cardiology consultation showed no abnormalities, and upper gastrointestinal endoscopy was performed. A bleeding endoluminal gastric mass was found arising from the antrum, extending to the angulus and involving both the anterior and posterior gastric wall. An adrenalin injection (1:10000) was executed on the site of the bleeding to control the haemorrhage and biopsies were collected. A surgical consultation was requested and the patient was transferred to the division of general and emergency surgery. The pathological report from the biopsies revealed a moderately differentiated gastric adenocarcinoma (G2). A computed tomography (CT) scan was also performed and confirmed the presence of a gastric mass with perigastric adenopathies (Figure ). No evidence of metastastic activity was found. After an anaesthesiological consultation and subsequent fluid therapy to achieve a good balance, surgical intervention was scheduled. Given her age, the extent of the tumour and general clinical conditions, we decided to perform a gastric resection with D1 lymphadenectomy, and reconstruction using a Billroth II Hofmeister-Finsterer retrocolic loop with mechanical sutures. We inserted a nasogastric tube to obtain gastric decompression and a surgical drain next to the anastomosis. The pathological report disclosed a mixed type adenocarcinoma of the stomach, with signet ring cells, cellular elements typical of the Lauren's intestinal type and undifferentiated cells (pT3 N0 Mx G3 R0) with surgical rim with no evidence of pathological findings. The clinical course was normal except for haemoserous output from the abdominal drain (daily output about 300 cc). This haemoserous output transformed to enteric fluid on the 12th postoperative day, with a medium output of 500 cc/day. Her clinical condition was stable and there were no indications for further surgical intervention. We put the patient on total parenteral nutrition, antimicrobial therapy and somatostatin. We medically treated the fistula and achieved a prompt clinical response from the patient with reduction of enteric fistula output. The enteric fistula developed from the duodenal stump, as showed by radiological examinations and a CT scan performed after the appearance of enteric fluid on drainage. The patient retained the abdominal drain until the 60th postoperative day when she was discharged in good health. Oncological follow-up was planned, but the patient refused any treatment. Twelve months after discharge she was readmitted to our emergency department with a diagnosis of subacute bowel obstruction and transferred to our surgical department. Physical examination defined the patient to be in poor clinical condition with an erythematous cutaneous swelling (diameter 6 cm) on the right side of her abdomen at exactly the same location as the drain was previously inserted to control the enteric fistula (Figure ). Medical therapy with fluids and a nasogastric tube for stomach decompression was performed in order to improve her clinical condition. In addition, biopsies were collected on the erythematous lesion. The bowel obstruction resolved in 2 days with the reprise of flatus. A pathological report showed the presence of signet ring cells and neoplastic cells coming from a primary adenocarcinoma of the stomach. These cells characterized the lesion as a cutaneous metastasis from an adenocarcinoma of the stomach. Given the patient's age and her poor general condition, we decided not to remove the lesion. Her clinical condition improved in 7 days, and she was transferred to the geriatric unit of our hospital for further care. |
A 67-year-old man was admitted to our hospital because of liver dysfunction during a screening examination. Enhanced abdominal computed tomography (CT) revealed a hypervascular mass of 35 mm in diameter in the descending portion of the duodenum (Fig. ), and the left three sections of the liver were occupied by multiple cystic tumors with contrast enhancement of the cystic wall, 13 cm in diameter (Fig. ). A duodenal tumor was identified on gastrointestinal endoscopy (Fig. ), and a biopsy revealed a NET. The serum levels of insulin, gastrin, and glucagon were within normal ranges. CT did not initially reveal evidence of pancreatic invasion between the tumor and the pancreas; however, irregularities of the duodenal wall and swelling of the lymph nodes around the pancreatic parenchyma were observed. Thus, the patient was diagnosed with non-functional duodenal NET with multiple liver metastases, T2N1M1 stage IV (UICC 8th). In addition, CT revealed the anatomical variation of the CHA, which branched from the SMA and ran fully through the head of the pancreatic parenchyma (Fig. , Additional file Figure S1). The CHA branches into the left hepatic artery (LHA), the middle hepatic artery (MHA), and the right hepatic artery (RHA) (Fig. a, b). Furthermore, a developed gastric arterial arcade, 4 mm in diameter, was found between the left gastric artery (LGA) and the right gastric artery (RGA). The RGA was branched from a distal portion at a distance of 10 mm from the root of the LHA (Fig. ). Incidentally, we did not observe stenosis of the celiac axis due to compression by the median arcuate ligament. We planned PD and left trisectionectomy with caudate lobectomy combined resection of the tp-CHA with the preservation of the gastric arterial arcade in order to maintain arterial flow of the remnant liver, preserving the route of the celiac artery to the right posterior hepatic artery (RPHA) via the gastric arterial arcade from the LGA to the RGA, LHA, and RHA. If the hepatic arterial flow could not be maintained by this route, the preservation of the tp-CHA by separating from pancreatic parenchyma or arterial reconstruction using radial artery graft between CHA and RHA was planned. Four weeks after percutaneous transhepatic portal embolization, surgery was carried out.\nAfter laparotomy, the gastric arterial arcade was exposed and encircled, and the LHA, RHA, and proper hepatic artery (PHA) were encircled (Fig. ). The LHA was divided at the distal side of the origin of the RGA. The MHA and the right anterior hepatic artery (RAHA) were also divided. The left portal branch and the right anterior portal branch were divided (Fig. ). The liver was transected, and the left hepatic duct and right anterior hepatic duct were divided. The left trisections and caudate lobe were anatomically resected. After clamping the PHA, the hepatic arterial signals of the RPHA via the gastric arterial arcade were confirmed by intraoperative Doppler ultrasonography (Fig. ). After trisectionectomy and caudate lobectomy, PD was performed. The pancreatic head was dissected from the SMA after the upper jejunum was divided. The pancreas was divided in front of the SMV. Finally, the specimen was only connected by the tp-CHA and the common hepatic duct (CHD) (Fig. ). The hepatic arterial signals of the RPHA was maintained after clamping the PHA. The PHA and the origin of CHA were divided, and the tp-CHA was taken out with the pancreatic head (Fig. ). The CHD was divided, and the specimen was removed (Fig. ). Reconstruction was performed via modified Child’s method. The operative time was 1072 min and the intraoperative blood loss was 3052 ml, and red blood cell transfusion was performed (1680 ml).\nPostoperatively, the patient developed pancreatic fistula (Clavien-Dindo IIIa) and biliary leak (Clavien-Dindo IIIa), and these complications were treated conservatively. There were no signs of hepatic ischemia. The patient was discharged on postoperative day 39. The pathological diagnosis was duodenal neuroendocrine tumor G2 with multiple liver metastases. The Ki-67 labeling index was < 20%, and staining for chromogranin A and synaptophysin were positive. There was no evidence of invasion of the pancreatic parenchyma; however, the duodenal tumor was confined to the MP layer, and one of the 25 examined lymph nodes was positive, and moderate lymphovascular invasion was observed. The final diagnosis was pMP, med, INFa, ly1, v2, pPM0, pDM0, and pEM0. The patient has shown no recurrence in the 22 months since the operation. Enhanced abdominal CT at 4 months after surgery revealed the blood flow of the RPHA via the gastric arcade (Fig. ).\nOver the years, several authors have described variations in the hepatic arterial anatomy; a CHA arising from the SMA—called the hepatomesenteric type—is a rare clinical entity. Yang et al. and Hiatt et al. reported that this condition was observed in only 31 of 1324 patients and 15 of 1000 patients, respectively [, ]. A CHA passing through the pancreatic head parenchyma, tp-CHA, is even rarer; Yang et al. [] reported that among 31 patients with the hepatomesenteric type, only 3 had this condition.\nWhen PD is scheduled in such patients with tp-CHA, it is important to maintain the arterial supply to the liver. Surgeons should preoperatively determine whether to preserve or perform combined resection of the tp-CHA. Tp-CHA preservation was selected in several previous reports [, , ]. This surgical procedure is technically feasible; however, there is a risk of a positive surgical margin or insufficient lymph node dissection and a tendency for increased intraoperative blood loss during the separation of the pancreatic parenchyma. If the tp-CHA is resected, reconstruction is usually necessary in order to maintain the hepatic arterial flow. Previous reports [, , ] have described successful arterial reconstruction after CHA resection during PD; however, such procedures are associated with an increased risk of thromboembolism, which can lead to a fatal outcome, especially in HPD []. In contrast, when collateral circulation develops, surgeons can perform combined resection of the tp-CHA, preserving the collateral circulation without arterial reconstruction. Several reports have recommended preoperative embolization of CHA in order to maintain the hepatic arterial flow through enlarged collateral arteries []. Although preoperative embolization can increase the liver arterial flow through collateral arteries, it is not routinely recommended because of the risk of complications, which includes the migration of embolic material [, ].\nA developed gastric arcade or pancreaticoduodenal arcade is frequently seen in patients with the stenosis of the CHA due to factors such as compression by the median arcuate ligament []. There are only a few cases in which the hepatomesenteric trunk and the tp-CHA and the association between the tp-CHA and the development of a gastric arterial arcade have not been reported. On the other hand, Miyamoto et al. reported the case of a patient with pancreatic head cancer with a CHA arising from the SMA who underwent radical PD combined with the resection of the CHA, in which the hepatic arterial flow was maintained via the gastric arterial arcade []. In this report, the patient did not have a developed gastric arterial arcade; however, the hepatic arterial flow via the gastric arterial arcade was sufficient and hepatic ischemia was not detected after the operation. Considering this case, even if the patients with tp-CHA do not have a developed gastric arterial arcade, surgeons may be able to preserve hepatic arterial flow via the gastric arterial arcade alone. If the hepatic arterial flow via the gastric arterial arcade alone is adequate after clamping the PHA, the combined resection of the tp-CHA can be considered, even if the gastric arcade is not developed before surgery. In cases in which the hepatic arterial flow is not adequate, the preservation of the tp-CHA or arterial reconstruction should be considered.\nWhen performing HPD, a PD-first procedure before hepatectomy is generally performed, as this approach is anatomically rational []. However, in the present case, performing hepatectomy after PD carried a risk of the arterial supply to the liver being reduced during hepatectomy. Had we chosen a PD-first procedure and the hepatic arterial flow not been maintained after CHA resection, it would have been necessary to perform arterial reconstruction before liver transection. This method is associated with a risk of injury to the reconstructed artery and thrombosis during liver transection. Given the above, we opted to perform hepatectomy before PD in our patient with a tp-CHA undergoing HPD.\nIn the procedure for separating the tp-CHA from the pancreatic parenchyma entirely, the surgeon should be concerned about the increasing rate of hemorrhage, surgery time, and the risk of injury to the tp-CHA. The surgical reconstruction of the hepatic artery when performing HPD is also associated with a high degree of risk. The association between tp-CHA and gastric arterial arcade was recognized on preoperative CT scans; the development of this collateral circulation may have the potential to prevent ischemia-related liver complications. From these points of view, the preoperative identification of the developed arcade of the gastric arteries helps in planning an appropriate operative procedure, and this procedure seems to be a viable and simple option. To our knowledge, this is the first report of PD combined with resection of a tp-CHA without preoperative embolization. Furthermore, this is also the first report of HPD for a patient with a tp-CHA. The preoperative identification of the developed arcade of the gastric arteries helps in planning the appropriate operative procedure when PD is scheduled for patients with a tp-CHA. |
An 84-year-old female patient was referred to our department after aborted sudden cardiac death due to ventricular fibrillation. The patient had experienced severe first time chest pain during the last hours prior to admission. Shortly before her arrival to our hospital, she developed ventricular fibrillation that was successfully converted to normal rhythm after application of a 200 J electrical shock by the emergency physician. On admission in our intensive care unit, she was awake, oriented, and hemodynamically stable. The patient's history revealed mild hypertension treated with a low-dose ACE-Inhibitor. No other cardiovascular risk factors were identified and no history of angina pectoris or exercise induced dyspnoea was reported. The ECG showed a sinus rhythm with T-wave inversion in the precordial leads (V1–V4). A bed-side echocardiography identified a severely reduced ejection fraction with an apical ballooning appearance of the LV. No signs of psychologic or physical stress were reported. The laboratory tests identified on admission elevated troponin levels (high sensitivity troponin = 102.7 ng/L) and increase white blood cells (13,000/μl) with normal C-reactive protein. No other pathological changes in laboratory tests were found on admission. A coronary angiography was performed within the same day that revealed a moderate stenosis of the left circumflex coronary artery (dashed arrow in ) as well as a localized spasm of the proximal LAD (white arrow in ), which was reversible after intracoronary injection of 0.2 mg nitroglycerin (white arrow in ).\nThe patient was treated with aspirin, statin, selective ß-blocker (bisoprolol), nitrate, and calcium antagonists. In addition, intravenous therapy with diuretics was initiated. Repeated echocardiographic examinations showed a slowly improving LV function with persistent hypokinesia of the apex. At one week, the LV ejection fraction was moderately reduced (ejection fraction of 42%). During the monitoring period, no ventricular arrhythmias were noted. Two weeks after the coronary angiography, a cardiac MRI was performed. The result of the examination was suggestive of the diagnosis of takotsubo cardiomyopathy with moderately reduced LV ejection fraction (Ejection fraction 44%), presence of myocardial edema in the apical region, and the presence of subendocardial late gadolinium enhancement in the apical region (Figures and ). The patient recovered well and could be discharged after 21 days in stable condition and without clinical symptoms such as angina or dyspnea. Due to the episode of ventricular fibrillation and incomplete recovery of the LV function she was discharged with a life-vest. The subsequent Holter-ECG monitoring did not show any ventricular arrhythmia and the 1-month follow-up echocardiographic exam exhibited normal LV function (ejection fraction 61%) without wall motion abnormalities. Thus, the life-vest was removed and the ICD implantation could be deferred. The patient is in stable condition and without cardiac symptoms on three months of follow-up. |
A 55-year-old male with impaired neck mobility and difficult in swallowing after tongue lesion resection with lymphadenectomy on June 23, 2011 was aware of paresthesia on the surface of his neck, and aggravated symptoms gradually. In February 2011, the patient was admitted to the First Affiliated Hospital of Guangzhou University of Chinese Medicine with recurrent oral ulcer. Physical examination revealed 2 ulcerated areas, 1 cm × 1 cm and 0.5 cm × 0.5 cm in size, on the left side of the tongue. The oral ulcer areas were biopsied and pathologically suspected as highly differentiated tongue squamous carcinoma. Postoperative pathology after left hemiglostectomy on February 19, 2011, showed (1) highly differentiated oral squamous cell carcinoma on the left tongue, with a mass diameter of approximately 0.8 cm and a depth of invasion of approximately 0.3 cm; and (2) leukoplakia on the left tongue without tumor invasion in the matrix or anterior, posterior, inner, or outer boundaries of the mass (Fig. ).\nAnother mass was also found on the right side of the neck 4 months after surgery, and physical examination showed that there was no new mass noted in the residual tongue, but the lymph nodes under the right jaw were enlarged, with a size of approximately 2 cm × 2 cm and clear boundaries. Thus, cervical lymph node metastasis was considered. Pathological examination after neck lymphadenectomy on June 23, 2011, showed\nhighly differentiated metastatic squamous cell carcinoma in the right and left submaxillary lymph nodes;\nreactive hyperplasia in the right submaxillary lymph nodes;\nabsence of cancer metastasis (0/8) in the left neck lymph nodes; and\ncancer metastasis (2/4) in the right neck lymph nodes (Fig. ).\nThe patient visited our characteristic Traditional Chinese Medicine (TCM) clinic in November 2018. Physical examination revealed a 15 cm-long curved surgical incision with numerous surrounded scar tissues in the neck region, and muscular dysplasia in the sternocleidomastoid muscle and the scalenus muscle (Fig. ). The Vancouver Scar Scale (VSS) was as follows: color (M): 1; vascular distribution (V): 0, thickness (H): 2, and flexibility (P): 4, with a total of 7 points. The range of neck movement in all directions was as follows: flexion: 30.67°± 7.87°; extension: 38.83°± 7.25°; right lateral side-bending: 27.83°± 3.66°; left lateral side-bending: 26.00°± 2.97°; right rotation: 54.83°± 9.09° and left rotation: 53.67°± 10.82° (Table ). These previous data indicated that the patients neck movement limitation and difficult in swallowing were related to the postoperative subcutaneous adhesion and scar hyperplasia in the neck region which induced localized circulation disturbance and limited range of motion of the neck. FSN treatment was performed to release the affected muscle and dissociate tissue adhesions.\nThe patient relaxed his body naturally during the FSN treatment. The physician selected the muscle that felt cold, stiffness, numbness, or painful to the patient as the affected muscle, so called “tightened muscle (TM)”, including the bilateral sternocleidomastoid muscles, pectoralis major muscles, trapezius muscles, rectus abdominis muscles, diaphragm muscles, and erector spinae muscles. The entry point was located around the affected muscles, and needling was performed from the upper, lower, left, or right side or diagonally, whichever was the most convenient for the physician to operate. The needle was inserted into the muscle belly from far to near and mostly parallel or perpendicular to the muscle fibers. The main entry points were\nsternocleidomastoid muscle: 3 to 5 cm above 1/3 of the clavicle or 3 cm below the Tiantu point (Ren 22);\ntrapezius muscle: at the Jianjing point (GB 21);\nrectus abdominis muscle: 2 cm from the linea mediana ventralis; and\ndiaphragm muscle: 2 to 4 cm from the linea mediana ventralis and inside the cartilages of the 7th to 12th ribs.\nThe needle feeder for FSN was placed on the skin, and the angle between the needle, and the size of the entry point was maintained as small as possible. After the skin was disinfected with iodide, a disposable needle for FSN (Nanjing Paifu Medical Technology Co., Ltd.; Batch No. 20152270832) was inserted in parallel from the entry point into the loose subcutaneous connective tissues. If the physician obviously perceived resistance or the patient felt sore and swollen when the needle was inserted into the affected muscle, the needle was slightly pulled back to the subcutaneous layer.\nThe inserted needle was fixed to the skin with the thumb. The middle finger served as a support, and the index finger and the ring finger held the needle in turn to sway in a fan-shaped pattern at an angle of 40° with a frequency of 100 times per min for 2 minutes each, called “swaying movement. While the physician was swaying for FSN in tandem with reperfusion approach, the patient actively or passively contracted the affected muscle, forming a resistance to the physicians actions. The main reperfusion techniques were as follows.\nFor the sternocleidomastoid muscle, the patient lay down on a supine position, with the sternocleidomastoid fully exposed by turning his head to the normal side at 30° to 40°. With the entry point taken 3 cm above 1/3 of the clavicle, the needle tip was pushed toward the neck region, swaying in a fan-shaped pattern. The patients head was turned to the affected side, while the physician used his palm to hold the patients head and face and applied force to the opposite direction, forming an ipsilateral head-turning resistance (Fig. ). Alternatively, the patient in the supine position had a fully exposed manubrium, and the entry point taken was 3 cm below the Tiantu point (Ren 22). The needle tip was pushed toward the neck region, swaying in a fan-shaped pattern. The patient might raise his head to the greatest extent, so the physician used his palm to hold the patients forehead and applied force to the opposite direction, forming a supine head-raising resistance.\nFor the trapezius muscle, the patient was in an orthopnea position, and the shoulder back on the affected side was fully exposed. The needle tip was pushed toward the neck region. The patient shrugged his shoulder on the affected side toward the same-side ear, and the physician held the shoulder on the affected side and applied force to the opposite direction, forming an ipsilateral shrugged shoulder resistance. Alternatively, the patient laterally bent his head toward the affected side, and the physician used his palm to hold the head and face on the affected side, forming an ipsilateral lateral bending head resistance.\nFor the rectus abdominis muscle, the patient was in a supine position, and his abdomen was fully exposed. The needle tip was pushed toward the linea mediana ventralis, and needling was performed in a direction perpendicular to the rectus abdominis muscle, thereby swaying in a fan-shaped pattern. The patient kept both lower extremities together, straight, and bent his body up at the hips with an angle of 30° or performed sit-ups with both upper limbs straightened and lifted off the bed at the same time.\nFor the diaphragm muscle, the patient was in a supine position with the chest and abdomen exposed and the needle tip pushed toward the xiphoid. As the patient took a deep breath and huffed, the physician used his palm to hold the patients abdomen and push.\nFor the cicatricose, the patient was in a supine position. For the physicians convenience, the entry point was taken 2 to 3 cm from the area (attachment point) where scar tissues were tightly connected, and the needling direction was parallel to the cicatricose. The needle tip was inserted into the connective tissues beneath the scars at the smallest possible angle, and the physician swept at the maximum angle and at the same frequency and duration as the affected muscle (Fig. ), thereby forming an ipsilateral head-turning resistance and an ipsilateral lateral bending head resistance. Reperfusion approach lasted approximately 10 seconds each, with an interval of more than 10 seconds. The stiffness of the affected muscle was examined after reperfusion approach was repeated 3 times.\nThe patients condition should be carefully observed during FSN treatment, and the physicians approached technique, including swaying movement and reperfusion approach, should be smooth and soft. Upon the completion of the procedure, the physician should instruct the patient to move his body for a few minutes with the soft casing tube retained and observe improvement in the patients symptoms. If no dizziness, nausea, chest distress, palpitation, or any other adverse reaction was found, the disposable soft casing tube was removed.\nFSN treatment was administered 2 to 3 times every week for 1 month until the patient felt a remarkable improvement in neck movement, and soreness, stiffness, cold, and tingling were greatly reduced. The VSS was M1, V0, H2, and P2, with a total of 5 points. The range of neck movement in all directions was as follows: flexion: 38.83°± 3.82°; extension: 41.83°± 7.33°; right lateral side-bending: 33.33°± 2.50°; left lateral side-bending: 28.33°± 1.63°; right rotation: 58.33°± 9.00° and left rotation: 62.00°± 6.54°. Stata11.0 was used to process data, and t-test for paired data was performed for statistical analysis, whilst P < .05 indicated statistically significant difference. A significant difference was observed in neck mobility in all directions except flexion and right rotation (P > .05) before and after treatment. Thus, neck movement was improved after the patient underwent FSN (Table ). |
A fifteen year old male presented to the Emergency Department (ED) at Cork University Hospital (CUH), a level one trauma centre serving the southeast of Ireland, after being crushed under the front wheel of a tractor on a farm. There was no significant past medical or surgical history.\nInitial management followed standard Advanced Trauma Life Support (ATLS) protocol [], and Orthopaedic Association Audit standards for Trauma (BOAST) guidelines for pelvic fracture [].\nOn admission Glasgow Coma Scale (GCS) was 10, the patient was intubated by the Emergency team after deterioration of his concousness level. We applied a chest tube after we had done needle decompression for left side tension pneumothorax. He underwent emergency embolization of the internal iliac artery. He responded well and the haemodynamic stability was achieved. On examination the left leg was noted to be internally rotated. Dorsalis pedis pulses were preserved bilaterally. Plain films demonstrated fractures of the left sacrum and iliac bone, a locked symphysis pubis () and fracture of the distal left femur. Computed tomography (CT) scan of the pelvis () demonstrated multiple fractures; displaced fracture of the left iliac bone, commuted left sacral ala fracture, minimally displaced right-sided sacral ala fracture with plastic deformation of the posterior crescent fracture configuration which was not fully extended through the iliac wing, and complete rupture of the pubic symphysis with LSP. CT scan of the lumbosacral spine (), showed a complete burst fracture of the L3 and L4 vertebral bodies and minimally displaced transverse process fractures of the right L1-L4 vertebrae. The surgical treatment for this young patient was a challenge. The patient was haemodynamically unstable on admission. We arranged a selective coil embolisation of the internal iliac artery by using multiple 6 mm pushable Azur CX coils () to stop the bleeding after failure of all measurements of resuscitation.\nA multidisciplinary team (MDT) including National Spine Injuries Unit and pelvic trauma specialist were consulted and the patient was transferred to the National Spine Injuries Unit, Mater Misericordae Hospital Dublin for definitive management.\nThe decision for a closed reduction to unlock the LSP by using the external fixator (Hoffman 2) as a lever arm for reduction was made as the best option for a skeletally immature haemodynamically unstable patient. The Illiosacral Joint (ISJ) was fixated with two illiosacral cannulated screws.\nThe patient was in the supine position on the orthopaedic table. An external fixator was applied to the pelvis and used as a lever arm for closed reduction of the LSP. This involved applying an anterior and lateral pulling force directly on the pubic bone via the external fixator with a simultaneous external rotation force applied on the left iliac wing (). The bilateral SIJ fracture was fixed with two fully threaded cannulated screws () using the method described by Routt et al. []. The distal femur was fixed with external fixators (). The patient's position was then changed to prone for posterior spinal stabilisation L1 to L5 with two rods and ten pedicular screws ().\nPost-operative management included analgesia, thromboembolic prevention commenced 48 h post operatively with subcutaneous enoxaparin, bed rest for 48 h with 30 degrees inclination. The post-operative period was uneventful. We allowed partial weight bearing for eight weeks. We removed the external fixator after eight weeks from the initial surgery after clinical and radiological confirmation of fractures healing by plain radiograph (, ).\nAfter one year from the initial injury the patient was fully weight bearing with no limping. We assessed his quality of life and mobility by SF-12 an SF-32 outcome scores []. His SF-12 was 42 and SF-32 was 97. His updated radiographs are illustrated in (). |
A 66-year-old female with a history of hypertension, depression, previous MRSA infection, gastric bypass, and gastroesophageal reflux disease sustained a complicated work-related injury, which included right wrist and humerus fracture managed by open reduction and internal fixation. Post-operative day 10, she developed a deep shoulder joint abscess with MSSA (susceptible to all antibiotics tested) requiring a second surgery with irrigation and debridement and hardware removal. She was treated initially with a 6-week course of intravenous vancomycin followed by oral clindamycin. Her shoulder infection showed improvement after 5 weeks of clindamycin, but then she was found to have developed a massive left thigh hematoma that required surgical evacuation with debridement of necrotic tissue and wound vacuum placement. She was continued on oral clindamycin for an additional 7 weeks due to suspected staphylococcal infection. She required multiple surgical debridements, and antibiotics were stopped since the wound bed appeared to be healing with no signs of infection. After 4 weeks of being off antibiotics, the patient’s leg wound appeared to have increasing induration and drainage, thus clindamycin was reinitiated. MSSA was isolated from a wound culture obtained at this time, and susceptibilities now revealed clindamycin resistance. Oritavancin was chosen to treat her deep tissue infection since the patient reported allergies to cephalexin (anaphylaxis), tetracyclines (anaphylaxis), and sulfa antibiotics (hives), which limited antibiotic treatment selection. She received one dose of oritavancin after 20 weeks of clindamycin and tolerated the dose without difficulty. On follow-up, she was deemed to have a suboptimal response to her first dose of oritavancin by evidence of continued wound drainage and elevated erythrocyte sedimentation rate (ESR), thus a second dose was administered, which was 19 days after receiving the first dose. During a follow-up visit, the patient reported new-onset hearing loss and was referred for audiology testing. She had received her final dose of oritavancin 14 days after her second dose for a total of three doses in a 33-day period. Evaluation by audiology was conducted after she received her third dose where the patient was diagnosed with moderate high frequency sensorineural hearing loss. At outpatient follow-up 3 weeks after her third oritavancin dose, she was clinically cured and her inflammatory markers had normalized. The patient reported some mild improvement in hearing loss but still had tinnitus. |
A 6-year-old girl presented with a history of road traffic accident 4 years ago, causing trauma to the spinal cord. Soon after the trauma, paralysis of both lower limbs and hands was noticed by her parents. The child underwent extensive rehabilitation leading to complete recovery of her upper limbs, but developed a plateau in her recovery phase below the level of injury. On detailed assessment prior to the cell therapy, she exhibited neurological features like hypotonia and hyporeflexia in bilateral lower limbs. Strength of grade 5 in bilateral upper limbs and grade 0 in bilateral lower limbs was recorded. Total sensory loss below D10 level was present. Urinary incontinence was reported with poor urine control. Magnetic resonance imaging (MRI) showed focal myelomalacia from C7 to D1 in the form of focal atrophy of the cord extending from C7 to D1 with altered signals at these levels; see . Functionally, she was partially dependent on her mother for activities of daily living (ADL), mainly mobility. She was able to stand and walk with a walker and Hip Knee Ankle Foot Orthosis (HKAFO) with difficulty and many compensatory strategies. Trunk control was poor in standing. The patient underwent two doses of cell therapy with a gap of 6 months between the two. She scored 82 out of 126 on Functional Independence Measure (FIM). On American Spinal Injury Association (ASIA) scale, she was at level A.\nBased on inclusion criterion as per the World Medical Associations Helsinki Declaration, the patient was selected for intervention. The treatment protocol is approved by the Institutional Committee for Stem Cell Research and Therapy (IC-SCRT). Prior to admission, a signed informed consent was obtained from the parents. Granulocyte-Colony Stimulating Factor (G-CSF) (300 mcg) injections were administered subcutaneously, 48 hours and 24 hours prior to bone marrow aspiration. Autologous bone marrow mononuclear cells (MNCs) transplantation was done for the child. With the patient in supine position, local anesthesia was given in the region of the right anterior superior iliac spine with sedation. Following this, using a bone marrow aspiration needle, 100 mL of bone marrow was aspirated and collected in heparinized tubes and transported to the laboratory. In the laboratory, the MNCs were separated by the density gradient method. The cells were sent for CD34 counts. Total cell count was 83 × 106, out of which 96% cells were viable. CD34 count was 1.23%. The cells were transported back to the OT in a sterile cool container. The patient was positioned in lateral position and, using a lumbar puncture needle and cerebrospinal fluid (CSF) drainage set, the thecal sac was punctured in the L4-L5 space. A catheter was introduced into the thecal sac and the cells were injected through the catheter. The catheter was then withdrawn after the cells were injected. Methylprednisolone 500 mg in 100 mL saline was given intravenously simultaneously during the injection.\nAfter the cellular therapy, patient underwent rigorous physiotherapy and occupational therapy.\nOne week after cell therapy, a reassessment was conducted which showed some clinical improvements. Sensory improvement was seen in both of the lower limbs, mainly touch in both of the soles of her feet and patchy areas in the legs as compared to no sensations previous to the cell therapy. She also exhibited improvement in her urinary control, as she was able to hold urine for one and half hours as compared to poor control previously. With respect to ambulation, her gait was significantly improved using HKAFO. Improved strength of back extensors and abdominals was also seen. In view of improvements after cell therapy and neurorehabilitation, the patient underwent 2nd dose after a span of 6 months. During these 6 months, the mother continued her exercises at home.\nThe patient underwent the same procedure of cell therapy.\nAfter 2nd dose of cell therapy, patient underwent physiotherapy and occupational therapy for one week. After one week reassessment was done. Urinary control improved; thus she could hold for 2 to 2 and 1/2 hours and could pass voluntarily. There was a significant improvement in her gait, but with increased lordosis. She showed minimal control of bowel. Use of diapers was restricted only when outdoors, thus reducing her dependency on them. Her sitting and standing balance improved. She was now able to do tasks like bending down and picking up objects from floor in standing position, which was difficult for her earlier. ASIA grade remained unchanged at level A. She now scored 101 out of 126 on FIM as compared to 82 previously with training for stair climbing, which was significant.\nThe child showed increased duration in her urinary control from 1 (1/2) to 2 (1/2) hours, thus reducing her dependency on diapers. As a result of improved back extensors and abdominals, there was a significant improvement in her gait. Her static and dynamic balance improved. Antigravity positions and activities were easier for her, which was reflected in her FIM scores. |
A previously healthy 10-month-old boy was admitted with one week of fever, rash, and malaise. He appeared lethargic with a fever of 102 degrees F and a heart rate of 180 beats per minute. He had a purpuric petechial rash over his trunk and extremities and an erythematous bulging tympanic membrane on the right. His liver was palpable 4 cm below the costal margin and he had no splenomegaly. His initial white blood cell count was 3.9 with 68% lymphocytes and 13% atypical lymphocytes. His hemoglobin was 8.1 mg/dL and platelet count 36,000. His AST, ALT, and LDH were all elevated at 152, 146, and 2041, respectively. Epstein-Barr virus (EBV) serologies indicated acute infection and viral load demonstrated persistent viremia. His immunoglobulins were normal.\nHe was treated with ceftriaxone for acute otitis media and possible bacteremia; over the next several days he improved clinically with less lethargy and improved appetite. However, his pancytopenia worsened necessitating red blood cell and platelet transfusions. His bilirubin rose to 8, ALT to 1890, and AST to 7300. His triglycerides were elevated at 319 and fibrinogen low at 107 (). A bone marrow biopsy revealed hypocellular marrow with no evidence of hemophagocytosis.\nHe was treated with high dose IVIG, acyclovir, steroids, and chemotherapy according to the HLH-94 protocol []. Three weeks into his illness he developed respiratory distress and was intubated. He developed progressive liver failure with coagulopathy and appeared septic. He died 24 days after the onset of nonspecific viral symptoms consistent with acute EBV infection.\nGene sequencing performed on a peripheral blood sample at the University of Washington identified a point mutation in exon 1 resulting in a new splice site and the deletion of 22 base pairs, frame shift, and early termination of SH2D1A, confirming a diagnosis of XLP. His mother is a carrier of the mutation. His soluble interleukin-2 (sIL-2R) receptor level was 9311 U/mL and perforin studies were normal. Natural killer cell function was not able to be performed.\nX-linked lymphoproliferative disease (XLP) is a rare genetic disorder which affects less than one in one million people, usually previously healthy males in their first decade of life. The defective gene responsible for this disease is found on the X chromosome at Xq25 and encodes the protein SAP (signaling lymphocyte activation molecule or SLAM-associated protein, also called DSHP or SH2D1A), an important mediator of signal transduction in natural killer (NK) and T cells that ultimately lead to lymphocyte activation [, ]. More than 50 heterogeneous mutations of SH2D1A have been reported, although none are identical to that found in our patient. Deficiencies of this protein alter the function of NK and T cells and decrease cytokine production, subsequently affecting B cell proliferation and differentiation. This abnormal response renders the immune system unable to destroy cells infected with EBV [].\nSAP also appears to enhance apoptosis in B and T cells. Owing to the uncontrolled proliferation of T cells in SAP deficient patients, the majority of patients with XLP will progress to fulminant infectious mononucleosis with extensive hepatic necrosis and bone marrow failure leading to death within one month of onset of the disease. Survivors will typically exhibit residual cellular and humoral immunodeficiency, and are at increased risk for additional lymphoproliferative disorders and malignancies, usually of B cell origin [].\nXLP classically presents as fever, marked lymphadenopathy, malaise, pharyngitis, and hepatosplenomegaly. The diagnosis should be suspected in young males with an abnormal or exaggerated immune response to EBV, to include prolonged clinical course of greater than 1-2 weeks, marked cytopenias, viremia, or in those with a history of fatal EBV infection in maternal male relatives. Initial laboratory tests may reveal pancytopenia or lymphocytosis on the CBC, atypical lymphocytes on peripheral blood smear, positive Monospot or EBV titers, low fibrinogen, and elevation of bilirubin, triglycerides, and liver transaminases. Immunoglobulin levels can also be helpful, as hypogammaglobulinemia occurs in one-third of patients. Serial labs may demonstrate progressively worsening pancytopenias and liver function tests. Subsequent evaluation with neuroimaging, coagulation studies, and bone marrow biopsy can aid in determining the extent of disease. Definitive diagnosis is made by undergoing genetic testing for mutation in SH2D1A.\nXLP can have a similar clinical picture to hemophagocytic lymphohistocytosis (HLH) and in series of HLH patients mutations in SAP are frequently found. Thus, SAP mutation analyses should be considered in patients with HLH without apparent cause [–]. HLH can be familial in origin, or associated with various infections and autoimmune disorders. NK function and perforin studies can often be helpful in distinguishing these two entities; patients with HLH will exhibit decreased NK cell activity and perforin levels, while in XLP patients perforin levels are usually normal. A diagnosis of HLH is confirmed when analysis of bone marrow aspirate or lymph node tissue reveals hemophagocytosis [].\nThe differential diagnosis of XLP also includes common variable immunodeficiency (CVID), which affects males and females equally and is characterized by frequent respiratory infections and low levels of IgG, IgA, and IgM []. Transient hypogammaglobulinemia of infancy can occur in infants without other complications and levels often normalize after 24 months of age. Griscelli syndrome is an autosomal recessive disease that features immunodeficiency, cytopenias, partial albinism, neurologic deficits, and viral induced HLH []. Chediak-Higashi is another autosomal recessive syndrome featuring partial albinism and immunodeficiency, but these patients also exhibit platelet dysfunction and can enter a lethal phase of disease initiated by viral infection (often EBV) leading to lymphohistiocytic infiltration of organs and death []. It is important to distinguish these entities from XLP as treatment approach and prognosis vary greatly.\nUnfortunately, individuals with XLP who enter the fulminant stage of disease have over 90% mortality, but some improvement has been shown with the HLH-94 protocol consisting of steroids and cytotoxic drugs such as cyclosporine and etoposide. Rituximab has reportedly been effective and IVIG has been used in the acute setting with minimal benefit. The only curative treatment for patients with XLP is an allogeneic bone marrow transplant, which ideally should be performed prior to EBV exposure. Thus, perinatal testing with chorionic villus sampling or amniocentesis is helpful in pregnancies of a known carrier [].\nPrimary care physicians should be aware of XLP and suspect it in boys who develop complications with acute EBV infections. With suspected cases, hematology and immunology should be consulted for help in management and diagnosis. While treatment for affected children is still largely ineffective, it is essential to make a diagnosis so mothers who are carriers can receive appropriate genetic counseling and other family members may be screened. |
An exceptionally active 45 year-old-male activated United States (US) Army Reservist with no past medical history presented for review after sustaining a laceration to his right knee.\nThe patient was conducting marijuana abatement when he accidentally lacerated his right leg just proximal and lateral to the knee joint resulting in a superficial soft tissue injury. He was examined in the emergency department (ED) later that day (day 0). He received 10 sutures, tetanus immunization, and was prescribed cephalexin 500mg to be taken orally four times daily for seven days. He had excellent healing of the wound with rapid advancement in his mobility and remained without constitutional symptoms, associated erythema, or significant pain. On day +7 after the laceration, he fell down stairs onto his right knee. There was mild pain at that time with no popping or tearing sensation. However, over the next 72 hours, he noted increased pain and swelling of the right knee. Post-injury day +10 was also the first time he noticed an appreciable impairment in mobility due to pain. He had a second fall which worsened his knee pain further. The swelling and pain did not improve by day +35 so he presented again to the ED for reevaluation. His vital signs were normal and he was afebrile. His knee was warm, swollen, but without erythema. Range of motion was full, but painful with extremes of motion. Laboratory data revealed an elevated C-reactive protein (CRP) level of 5.4 mg/dL (reference <0.5 mg/dL), but other blood tests did not reveal any abnormalities. Arthrocentesis was advised, however, the patient declined the procedure. Meniscus injury was atop the differential diagnosis by the ED team and orthopedics due to his repeated falls; infection was believed to be unlikely due to the rapid initial healing and long temporal distance between injury and re-presentation to the ED. No antibiotics were prescribed and he was scheduled for magnetic resonance imaging (MRI) of the knee as an outpatient on day +49.\nRight knee MRI with and without contrast revealed findings concerning for osteomyelitis within the lateral femoral condyle (A) and the medial tibial plateau osteomyelitis with abscess formation (2.3 × 1.3 × 0.3cm; B). A joint effusion and thickened enhancing synovium was present consistent with septic arthritis. The MRI also demonstrated enhancement of the popliteus musculature compatible with myositis and a longitudinal tear of the medial meniscus. The patient underwent urgent arthroscopic washout and debridement on day +50. He was initially treated empirically with intravenous ceftriaxone and vancomycin. Numerous serum based microbiologic diagnostics were negative and bone cultures from the tibial sequestrum yielded no growth. Pathologic analysis of bone revealed lymphoplasmacytic necrosis and osteomyelitis without the identification of bacterial, mycobacterial, or fungal elements. Due to concerns for difficult-to-grow bacteria, mycobacteria, or fungi as cause of infection, bone biopsy under fluoroscopic guidance was then pursued on day +57 for repeat biopsy of the tibial plateau sequestrum and sample processing for direct molecular based testing. At discharge the patient was receiving oral linezolid, rifampin, and moxifloxacin as empiric coverage for most bacterial pathogens and non-tuberculous mycobacteria. Universal broad-range 28S ribosomal polymerase chain reaction (uPCR) assay revealed the presence of Scedosporium dehoogii DNA. The patient's antimicrobials were then transitioned to dual antifungal therapy with oral voriconazole (VCZ) and oral terbinafine (TBF). He first received two 400mg loading doses of oral VCZ 12 hours apart, followed by oral voriconazole 300mg twice daily. VCZ levels were acquired every 14 days with a target dosing range between 2 and 6 mg/L. Oral TBF was dosed 250mg once daily.\nWhile maintained on VCZ and TBF, his CRP normalized and average VCZ levels were 4 mg/L for the next several months. Repeat MRI on day +195 revealed ongoing osteomyelitis in the lateral femoral condyle and extensive post-debridement changes in the medial tibial plateau (C and D). He underwent a repeat arthroscopic debridement on day +242 for continued severe debilitating knee pain and continued radiographic advancement of disease in the tibia, but unfortunately the procedure did not successfully improve his symptoms. He had requested an amputation due to the severe debilitating pain despite appropriate antifungal treatment and multiple attempts at arthroscopic debridement. He was indicated for a two stage total knee arthroplasty (TKA). He had an en bloc resection of the affected osteomyelitic medial tibial plateau and an articulating VCZ spacer with a 15mm medial augment was placed on day +463. The resected bone from the medial tibia was sent for fungal cultures and uPCR after the first stage of knee arthroplasty and remained negative. Dual antifungal therapy was uninterrupted until he was placed on a 4 week antifungal holiday starting on day +533 with continued demonstration of low level inflammatory assays. He underwent a second stage reconstruction on injury day +561 with a revision Zimmer Persona TKA. The day after completion of the surgery, the patient was restarted on VCZ and TBF at the same previous doses, including the same VCZ loading dose regimen as when he first started antifungals. On evaluation of day +576, the patient confirmed appreciable reduction in his knee pain with no infection complications identified. Follow-up X-rays of the patient's right knee on day +621 confirmed appropriately maintained hardware alignment (A and B). Long term VCZ and TBF for at least six months after his second stage TKA was recommended despite fingernail discoloration and peripheral neuropathy symptoms to reduce his risk of treatment failure. |
A 2.5-month-old male infant presented on ventilatory support with history of stridor since the neonatal period and episodes of apnea. The patient was a full-term baby with a birth weight of 3 kg, born by normal vaginal delivery. He was hospitalized at 1.5 months and 2 months of age with respiratory tract infections. During the second admission, he had two episodes of apnea for which he required ventilatory support. Chest X-ray showed bilateral hyperinflation with patchy consolidation. It did not show any evidence of common causes of stridor, like lobar emphysema, bronchogenic cyst or mediastinal mass. Because most common causes for stridor in the neonatal period include laryngeal and tracheal diseases, e.g. laryngomalacia, tracheomalacia, laryngeal and tracheal cysts, webs, stenosis and mucus plugs fibreoptic laryngoscopy and bronchoscopy were carried out. Bronchoscopy revealed external pulsatile compression on trachea. Hence, suspecting vascular ring, imaging was performed using 64-slice MDCT to confirm the diagnosis and determine further details. MDCT with three-dimensional (3D) reconstruction showed double aortic arch forming a vascular ring, encircling and compressing the trachea and esophagus [Figure and ]. The right aortic arch was the dominant arch with right subclavian and right common carotid arteries arising from the right arch and left common carotid and left subclavian arteries arising from the left arch. Echocardiography did not show any evidence of double aortic arch because of suboptimum suprasternal acoustic window. However, it helped to rule out any associated intracardiac anomaly.\nThe infant underwent an emergency surgery through left posterolateral thoracotomy. Before the anomalous arch was removed, pulling endotracheal tube proximally from the site of constriction led to increase in airway pressure, which suggested that the anomalous arch led to constriction of trachea and the resultant symptoms. During surgery, the left aortic arch after the origin of the left common carotid and left subclavian arteries was removed and both ends were oversewn. Additionally, the ligamentum arteriosum was also divided. After removal of the anomalous left arch, the airway pressure did not rise on pulling the endotracheal tube proximally. This was used as an intraoperative method to check whether the constricting arch is effectively removed. Mediastinal pleura was left open unlike in other thoracic vascular surgeries, which prevented fibrosis and possible reconstriction on trachea.\nIn the post-operative period, the patient was extubated after 2.5 days of ventilatory support. Gavage feeding was started from the third and oral feeds from the sixth post-operative day. He was kept in the intensive care unit for 10 days and in the pediatric ward for 1 week during which he became completely free of stridor. At the 6-month follow-up, he was completely symptom free. |
A 29-year-old female with a complaint of abdominal distention was referred to our hospital. She had been treated with antibiotics for acute pyelonephritis three times when she was 6, 20, and 28 years old. Blood test results including renal function and urine test were normal. Abdominal computed tomography (CT) scan demonstrated markedly dilated left calyceal structures, a pelvis with thinning renal parenchyma, and left ureter from the proximal to the distal end (). Retrograde pyelography revealed the absence of anatomical malformation of the ureteral orifice and the absence of vesicoureteral reflux (). Urinary cytology of the left ureter was negative. Split function of the left kidney function was less than 10% in renal scintigraphy. The diagnosis was megaureter due to stricture of the vesicoureteral junction. The patient hoped for surgery before becoming pregnant. Laparoscopic nephroureterectomy was performed by an intraperitoneal approach. Prior to the laparoscopic procedure, an open-ended 6Fr left ureteral stent was placed. During surgery, the patient was initially positioned in the right flank position and four trocars were placed. The pneumoperitoneum pressure was 10 mmHg. The first 12 mm trocar (A: camera port) was inserted in the umbilicus. The second 12 mm trocar (B) was located in the anterior axillary line above the ilial crest. The third 5 mm trocar (C) was located in the midclavicular line on the left. The fourth 5 mm trocar (D) was placed on the upper lateral site of the third trocar for assisting instruments ().\nWe approached the retroperitoneum from the lateral aspect of the descending colon. To facilitate dissection around the kidney, the ureter was emptied by sucking urine through a placed ureteral stent. After circumferential dissection of the kidney, we cut the renal artery and vein with polymer ligating clips. Then, the patient was positioned in a Trendelenburg position and a 5 mm trocar was inserted in the right lower umbilicus (E). The surgeon changed his right-hand trocar from (B) to (E) and his left-hand trocar from (C) to (B). The sigmoid colon was reflected medially and the dilated ureter was dissected down to the bladder. Then, the ureteral stent was removed and we resected the distal end of the ureter with polymer ligating clips. We extracted the resected specimen from the first trocar extending the skin incision to 30 mm using a 15 mm endocatch bag. The specimen weighed 576 g.\nThe total operative time was 262 minutes. Circumferential dissection of the left kidney in the right flank position took 129 minutes and resection of the distal ureter and removal of the specimen in a head-down position took 83 minutes. The total pneumoperitoneum time was 212 minutes. The estimated blood loss was minimal. The urine volume sucked from the inserted ureteral catheter was 900 mL.\nOperative and pathological findings showed that the kidney size was 12 × 8 × 6 cm, renal pelvis was remarkably dilated, and, in some parts of the renal parenchyma, interstitial fibrosis was recognized. The patient was discharged from our department without complications and was satisfied with her postoperative wound (). |
A 75-year-old man was referred to our hospital for abdominal fullness and nausea since 2 months. He had a medical history of hypertension and hyperlipidemia and a surgical history of the right inguinal hernia. The patient's laboratory findings were within normal limits. Abdominal computed tomography (CT) revealed a well-demarcated oval isodensity mass of 25 mm at the tip of his appendix. Contrast-enhanced CT revealed a lesion with gradual homogeneous contrast enhancement from the arterial phase to the equilibrium phase (). No abnormal findings were found in the root to the middle of the appendix. Abdominal ultrasonography (US) revealed a well-demarcated hypoechoic tumor. The tumor size was 22 mm × 18 mm × 18 mm, with some cystic area and blood flow (). Colonoscopy findings were normal. The patient's symptoms naturally alleviated during examination period.\nPreoperative diagnosis indicated appendiceal neuroendocrine tumor (NET) G1 or gastrointestinal mesenchymal tumors, such as GIST. Malignancy could not be ruled out; therefore, laparoscopic ileocecal resection with D3 lymph node dissection was recommended. Intraoperative findings revealed a well-demarcated tumor at the tip of the appendix, with no invasion into the surrounding tissue. This observation was similar to the preoperative imaging findings. According to another intraoperative finding, dissecting the adhesion between the terminal ileum and the peritoneum, which was the effect of the past herniorrhaphy, was necessary. The operation time was 167 min, and the amount of blood loss was 100 ml.\nPathological findings revealed a well-demarcated tumor originating from the muscular layer at the tip of the appendix and spindle-shaped heterotypic cells proliferating in a bundle. Vascular invasion and lymph duct invasion were not detected. No tumor cells were found in the dissected lymph node. Immunohistochemical studies revealed negative values for KIT and CD34 and positive values for S-100 protein (), which confirmed the schwannoma of the appendix. The patient was discharged on the 9th day after surgery without any complication requiring medical treatment. The patient is presently doing well without any evidence of recurrence at 3 months after surgery. |
We describe the case of a 34-year-old gravida II para l woman, with a gestational age of 26 + 3 weeks at admission, who had a relatively healthy 4-year-old child with her 40-year-old husband of non-consanguineous marriage. She had been on injectable contraception for 2 years and had regular menses for 6 months before the pregnancy. She had antenatal care at a local health center and was vaccinated with tetanus toxoid once and supplemented with iron for 3 months. She was screened for retroviral infection, hepatitis, and syphilis and it was documented nonreactive. She had no anatomic scan at early gestation. She came to Felege Hiwot Referral Hospital with the chief complaint of severe and persistent headache of a day’s duration which was occipital in location associated with blurred vision and generalized body swelling of 1 week’s duration. She had no other danger signs in pregnancy. Her past gynecologic history, medical history, and surgical history were uneventful. She is Amhara by ethnicity. She had no known family history of hereditary or chromosomal disorders.\nHer blood pressure at admission was 180/120 mmHg and pulse rate was 84 beats per minute; her respiratory rate was 22 breaths per minute and she was afebrile. She had pink conjunctiva and non icteric sclera, 24 weeks-sized gravid uterus, no abdominal tenderness, no organomegaly, no sign of fluid collection in her abdomen, and the fetal heart beat was positive. She had no vaginal bleeding or discharge. She had pedal and pretibial edema. She was conscious and oriented to person, place, and time. Her deep tendon reflex was +2 and her motor and sensory examinations showed no motor or sensory problems. Other parts of systemic examinations were normal.\nHer hypertension was controlled with intravenously administered hydralazine 5 mg two doses at our emergency department. In her complete blood count her white blood cells were 7300 cells/micL, hemoglobin of 13.4 g/dl, and platelet count was 169,000 cells/micL. Urine protein dipstick was +2, and liver and renal function tests were done: serum glutamic pyruvic transaminase (SGPT) 89 IU/L (elevated), serum glutamic oxaloacetic transaminase (SGOT) 102 IU/L (elevated), alkaline phosphatase (ALP) 229 IU/L, and lactate dehydrogenase (LDH) 288 IU/L. Total bilirubin was 0.24 mg/dl, albumin was 3.49 g/dl, blood urea and nitrogen was 12 mg/dl, serum creatinine was 0.69 mg/dl, and oral glucose tolerance test was in the normal range. Obstetric ultrasound showed a singleton, alive, intrauterine pregnancy with average gestational age of 26 weeks, there was a single large ventricle with partially formed midline structure (see Fig. ), amniotic fluid index was 13.4 cm, placenta was located anteriorly at the body of the uterus, and the presentation was breech; the fetus had normal four chambers of heart with normal outflow tract.\nAfter blood pressure was controlled (it took 2 hours), she was admitted with the diagnosis of late second trimester pregnancy and preeclampsia with severity feature plus semilobar HPE. Seizure prophylaxis for preeclampsia was given (magnesium sulfate according to World Health Organization guideline), methyldopa 500 mg orally every 8 hours was added, and she was counselled about options of management; the high incidence of associated anomalies, severe morbidities of survivors, and poor prognosis were discussed. Termination was decided and done with misoprostol 100 microgram every 3 hours at the third dose with outcome of 1.1 kg male, alive neonate. On examination of the neonate, there was cebocephaly, hypotelorism, single patent nostril which enabled nasogastric tube 6F, micropenis (8 mm), and unilateral right hand polydactyly with agenesis of middle phalanges of the fifth finger. There was rigidity involving all extremities which resisted extension and flexion (see Figs. , and ).\nAfter basic neonatal care was given (cord tied, airway cleaned, and newborn dried), he was transferred to our neonatal intensive care unit (NICU) but he died 20 minutes after admission to NICU. Immediate cause of death was not known. Following his death, further investigations were not possible for cultural reasons. At third postpartum day, maternal blood pressure was 130/90 mmHg, pulse rate was 78 beats per minute, and respiratory rate was 20 breaths per minute. Her complete blood count showed white blood cells of 12,000 cells/micL, hemoglobin was 11 g/dl, and platelet count was 122,000 cells/micL. Liver function tests showed SGPT of 35 IU/L, SGOT of 12 IU/L, ALP of 359 IU/L, and LDH of 254 IU/L; total bilirubin was 0.56 mg/dl, blood urea and nitrogen was 22 mg/dl, and serum creatinine was 0.8 mg/dl. After she was counselled to have preconception care and prenatal screening in next pregnancy, she was sent home relatively healthy. She was well at postpartum visits and methyldopa was discontinued at seventh postpartum day. |
A 41-year-old female with significant past medical history of cannabis use, tobacco use, bipolar disorder, chronic obstructive pulmonary disease, hypertension, and reflux esophagitis, but no alcohol use, presented to the emergency department for evaluation of recurrent excruciating epigastric pain accompanied by severe nausea and vomiting. Previously, she has had a similar but milder symptom for which she was evaluated. Previous esophagogastroduodenoscopy revealed no evidence of Barrett's esophagus or sprue; however, reflux esophagitis was detected for which she was started on a proton pump inhibitor.\nThe patient had been relatively symptom-free over the course of the two years until recently when she started experiencing severe epigastric pain with retching and nausea. She was then given pantoprazole and was discharged to follow up as an outpatient. The patient returned to the emergency department a few days later with similar epigastric pain with nausea and vomiting. Electrocardiogram (EKG) was without any significant ST or T wave changes ruling out acute coronary artery syndrome. CT scan abdomen and pelvis was performed and was negative for any acute pathology. The patient was treated with pantoprazole and pain medications and was later discharged after stabilization. The next day, the patient returned to the emergency department complaining of severe crampy abdominal pain with severe nausea and intermittent vomiting. The patient described her pain as being more severe with a rating of 10/10, twisting in nature, and some sensations of “butterflies” going up in the chest. Her pain was worse with eating, and she had been unable to tolerate any solid or liquid diet. She reported no relief with pantoprazole. Her review of systems was negative for heart burn or reflux-type symptoms, diarrhea, melena, or hematochezia. She also denied headache or history of migraine.\nA detailed abdominal examination was also done which was negative for guarding or rebound tenderness. The patient, however, had moderate tenderness to palpation in her epigastrium. Other workups including EKG, complete blood count (CBC), electrolytes, urinalysis, and kidney and liver functions were all within normal limits. The urinary pregnancy test was also negative.\nThe patient was then admitted to the hospital with initial differential diagnosis of gastritis, severe gastroesophageal reflux, cyclic vomiting syndrome, and peptic ulcer disease. She was given intravenous fluids, antiemetics, and initial one dose of morphine to decrease the pain and anxiety. The next morning, the patient's symptoms were unchanged. A urine drug screen was ordered which came back positive for marijuana. The patient admitted to its chronic use with increasing use over the past 2 weeks.\nConcerns for cannabis hyperemesis syndrome were raised given the repeated bouts of abdominal pain, nausea, and vomiting with benign abdominal exam in the setting of recent increased use of marijuana. The patient was continued on pantoprazole with addition of metoclopramide for nausea control but there was no improvement in her symptoms. Esophagogastroduodenoscopy was repeated that was evident only for mild gastropathy that could not fully explain her symptoms. After discussion with the patient and her spouse, a trial of capsaicin 0.1% topical cream, one application 3 times daily, was ordered with which the patient had dramatic relief with near complete resolution of her symptoms. |
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