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A 12-year-old otherwise healthy boy was referred from an urgent care center to our emergency department for fever and back pain. Per telephone report from the referring facility, the child had had a few days of leg pain and lumbar spine pain and was febrile to 102°F. A rectal exam was performed during which the child was reportedly incontinent of “bloody-brown stool.”\nThe patient arrived by ambulance to our emergency department in no acute distress. He complained of bilateral lower leg pain over the past week that had started laterally in his left hip, was achy, radiated to his lower legs but not to his feet, and was worse with movement and better somewhat with rest. His mom reported that he had abnormal gait. By the day of evaluation, he had developed focal lower back pain that was achy, sometimes sharp, worse with movement and sitting but better when supine. He had also been febrile to 101°F at home prompting his mother to bring him to urgent care. He had had no urinary retention associated with his back pain although he did have the above reported bowel incontinence. The patient stated that he could not feel the rectal exam at the urgent care center nor sense that he had been incontinent of stool until he was told that he had been so. Of note, he emphasized that his leg pain had almost completely resolved after digital rectal examination but that his back pain, although improved, was still present. He had had some loose stools in the past two days.\nAfter being triaged to an acute care room, the patient's initial vital signs were Temp 37.1°C, HR 95, BP 103/57, RR 12, O2 sats 96% on room air. He was generally well-appearing and thin. His head/eyes/ears/nose/throat exams were normal. He had no neck pain, stiffness, or meningismus, and he had normal cardiovascular and pulmonary exams. His abdomen was soft and nontender. He had no costal-vertebral angle tenderness to palpation but did complain of midline low back pain to palpation. His arms and legs were without acute abnormality. He had no abnormal skin findings. His neurologic examination was notable for increased tone in his quadriceps bilaterally although he was able to relax with significant coaching. Otherwise, he had a normal cranial nerve exam, normal gait, normal toe and heel walking, 5/5 motor strength proximally and distally throughout, normal sensation to light touch throughout, normal speech, no dysmetria.\nWhen the patient rolled to his side for a digital rectal exam, he was found to have copious amounts of stool-like, oozing, thick, brownish-red, malodorous fluid exuding from his anus. After cleaning him, his external anal exam was normal-appearing. A digital exam was performed and was negative for mass although he was unable to sense the exam or the fluid exuding from his anus. He was able to bear down.\nAn IV was placed and blood work was obtained. His laboratory studies were notable for mild leukocytosis to 13,000 without left-shift and complete metabolic panel without acute abnormality. “Stool” was sampled and found to be guaiac positive. Urine studies were normal and postvoid residual volume was less than 100 cc.\nAn MRI of the lumbar spine was obtained and was negative for spinal abnormality but notable for perirectal phlegmon (). Together with our physical exam findings and the child's history of fever, this was felt to be diagnostic for a perirectal abscess. Interestingly, the pain from the fluid collection had apparently been relieved by digital rectal which had likely caused spontaneous drainage and decompression.\nUltimately, the child was taken to the operating room with pediatric surgery for exam under anesthesia due to recurrence of pain. The abscess, draining rectally, was confirmed by exam. The residual fluid collection was drained and a 10-French Malecot catheter was placed, flushed to ensure functionality and sutured in place. On discharge, two days after initial evaluation, the child was eating, drinking, and ambulating well. He is to follow up with pediatric surgery subsequently in the outpatient setting. |
A 13-year-old girl diagnosed the previous year with CVID in the setting of pneumonia, low serum immunoglobulin levels, and absent antibody responses to immunizations, presented with worsening dyspnea. Whole exome sequencing did not reveal an underlying genetic explanation for the immune deficiency. A chest CT scan at the time of CVID diagnosis revealed only mediastinal lymphadenopathy. She was placed on monthly IgG replacement therapy and did well until she reported gradual worsening of dyspnea over 3 months. The dyspnea first manifested during competitive sports and progressed to an inability to walk up a single flight of stairs. Pulmonary function testing results () showed a restrictive pattern, and she was unable to complete the maneuvers for diffusing capacity of the lungs for carbon monoxide (DLCO). A follow-up chest CT scan revealed mediastinal and hilar lymphadenopathy, peripheral interlobular septal thickening, peripheral consolidation, and ground glass opacities (). Bronchoalveolar lavage obtained by bronchoscopy did not show any evidence of infection. Specific testing included bacterial, fungal, mycobacterial, and viral cultures along with PCR assays for influenza, respiratory syncytial virus, parainfluenza, human metapneumovirus, adenovirus, cytomegalovirus, Epstein-Barr virus, human herpes virus-8, and Pneumocystis jirovecii. She underwent a right lower lobe wedge resection biopsy via thoracoscopy. The biopsy () showed non-caseating granulomatous inflammation with aggregates of small lymphocytes, scattered multinucleated giant cells, scattered foci of organizing pneumonia, interstitial fibrosis focally in the subpleural space but not prominent or diffuse, and airway luminal compromise from adjacent lymphoid hyperplasia, confirming the diagnosis of GLILD.\nShe was treated with 4 doses of rituximab 375 mg/m2 given 4–6 months apart, based on clinical symptoms and pulmonary function testing, and azathioprine 50 mg daily for 18 months as IgG replacement therapy was continued. She responded well to the new therapy regimen with complete resolution of exercise intolerance and normalization of pulmonary function testing parameters (). She also had remarkable improvement of the CT scan abnormalities () with the follow up CT scan after completion of therapy demonstrating resolution of all abnormalities. |
A 54-year-old male visited the urology outpatient clinic with left flank pain experienced for 1 week. He had no history of urinary stones and a 5-year history of hypertension with medication. He complained of sharp pain in the left flank and iliac crest areas (). The pain was rated at 10/10 on the Numeric Rating Scale (NRS). Initially, he visited a local clinic and was administered medication for pain control. However, he was referred to our hospital as there was no significant improvement in his symptoms. To evaluate the potential intraurinary causes, he was admitted to the department of urology. Several tests, including routine urine analysis, blood tests, and X-ray were performed. Although there was no evidence of common intraurinary lesions, such as a urinary stones or urinary tract infection, he constantly complained of intense pain. He underwent abdominopelvic computed tomography (APCT), which indicated severe stenosis in the proximal portion of the left renal artery (). There were no abnormal findings in the spinal discs, facet joints and paraspinal muscles in APCT. Renal scan (Tc-99m DTPA [diethylenetriamine-pentaacetic acid]) was performed to evaluate his kidney function. It revealed that the glomerular filtration rate of left kidney was decreased (). The patient underwent percutaneous transluminal angioplasty, wherein a stent was inserted into the left renal artery. Pain improved by 5/10 on the NRS, after intervention. Two days later he was discharged home.\nHowever, the day after he was discharged, he returned to the emergency room with severely aggravated pain of 8/10 on the NRS and was readmitted to the cardiology department. Flank pain worsened again despite stenting having been performed on the last admission, which was considered the cause of pain. The physician considered a musculoskeletal disorder.\nA consultation with the rehabilitation department was conducted. During the physical examinations, tenderness was observed at each level of the left deep paravertebral muscles (DPVM) at the T10–T12 vertebrae. Local hyperalgesia on the skin over the left flank and iliac crest was identified when a fold of skin and subcutaneous tissue was pinched and rolled. Based on these findings, flank pain was thought to be the result of TLJ syndrome.\nTo correct the dysfunction of the TLJ segment, injections targeting the multifidus were administered, which plays the biggest role in the DPVM. Each needle was placed 1–1.5 cm lateral to the spinous process at the depth of the lamina. After touching the lamia, the physician pulled the needle approximately 5 mm backward and injected 3.5 mL of 0.25% lidocaine at each level. One area was injected per muscle and each of 3 levels were injected. There was no risk of blood vessel or nerve damage as the injection was completed after contact with the lamina bone. The average injection depth was approximately 3.5 cm. The next day the patient reported significant pain relief and a decreased pain score from 8 to 3 on the NRS. A second injection had the same way. Following the second treatment, the patient was discharged home. When he visited the outpatient clinic 9 days after the second injection, the flank pain had resolved almost completely. As there was remaining tenderness in the left T10–T12 DPVM, an additional treatment was performed at these levels. Five days after the third injection, flank pain was completely resolved, and no recurrence was detected at the regular checkup at the cardiology department after several months.\nPatient has provided informed consent for publication of the case. |
A 57-year-old female patient presented to the emergency department of our hospital with sudden-onset symmetric paralysis of bilateral lower limbs. The power was grade 0 with associated sensory loss as well. The patient had no other contributory history including that of fever, acute bouts of cough, coagulopathy, or any other chronic medical or surgical co-morbidity. She was referred for magnetic resonance imaging (MRI) of the spinal cord to evaluate the cause of paraplegia on a 1.5 Tesla whole body magnet (Siemens Medical Systems, Erlangen, Germany). An examination was performed on a 1.5 T scanner [] which revealed a mixed intensity complex multilobulated lesion in the anterior epidural space with convex posterior margin, adjacent to the C7-D2 vertebral bodies. The lesion was seen to cause gross compression and displacement of the spinal cord in the region with altered cord signal intensity (extending from C3 to D3 levels) suggestive of cord edema. This raised a suspicion of small anterior epidural hematoma which, however, could not explain the gross compression of cord along with the length of the edematous cord segment. The MRI also showed multiple dilated tortuous vascular channels in the epidural space along with the hematoma, as well as in the paravertebral space. This prompted a computed tomography (CT) angiography [] which confirmed the presen ce of extensive epidural, paravertebral, and chest wall collateral vessels, along with a hypertrophied anterior spinal artery compressing the spinal cord in the cervicodorsal region. The imaging was performed on a 64 Slice MDR CT scanner (GE Medical Systems, Milwaukee, USA). The collaterals were connected to bilateral internal thoracic, vertebral, and anterior spinal arteries. In addition, the long segment cord edema was probably due to vascular congestive state in the region as a result of slow flow and compression of perimedullary veins by the intraspinal space-occupying lesion hence formed. An interesting finding emerging at this stage was severe post-ductal coarctation of aorta, which could explain the genesis of all above features. No evidence of any abnormal spinal vascular malformation was noted. The patient was referred for further management to the cardiovascular surgery unit, where she was counseled for a possible shunt surgery, in the event of a cardiac emergency. Surgical therapy is associated with a low risk of restenosis and recurrence, whereas endovascular therapy, consisting of percutaneous balloon angioplasty and endovascular stent repair, has much higher incidence of restenosis, but is a safe and acceptable modality in poor surgical candidates.[] As for the present episode, she was managed conservatively with supportive hospital care and showed satisfactory improvement in grade of power, sensations and gait. She was discharged after 2 weeks once she became ambulatory. |
JS, a 25 year old female, presented to a chiropractic teaching clinic with a chief complaint of neck and upper back pain. Following the initial work-up, a diagnosis of neck pain of mechanical origin with an associated myofascial syndrome was made and a course of SMT, STT and exercises for the deep neck flexors undertaken. JS's symptoms were resolving well. During the course of a standard office visit it was noted that JS was being investigated for gastric ulcers because of a 3.5 year history of daily mild to severe and at times disabling abdominal pain with associated throat tightness. The pain would reach 8/10 in intensity and last for several hours. Repeated investigations including abdominal ultrasound, endoscopy, and tests for helicobacter pylori were all negative. JS's medical practitioner (GP) made a presumptive diagnosis of gastric ulcer disease and prescribed Cimetidine, then Lansoprazole and later Esomeprazole to no avail. JS was then advised by her GP to cease all medications and diagnosed functional dyspepsia. With persisting symptoms, the GP opted to prescribe an anti-depressant, Fluoxetine hydrochloride. JS's symptoms persisted and, in addition, she began to experience adverse effects to the Fluoxetine hydrochloride and so this was ceased. It was at this time that one of the authors (JKS) examined JS and discovered that both abdominal and throat symptoms were reproducible by direct pressure over the lateral aspect of her xiphoid process.\nA diagnosis of xiphodynia was established and a course of treatment involving 2 minutes of low-level laser therapy (LLLT) to an area of approximately 4 cm2 surrounding the xiphoid undertaken. A therapeutic trial of two laser treatments per week for up to four weeks was recommended. Progress was good. At the end of the third week JS reported significant decrease in frequency, duration and intensity of her abdominal pain. JS experienced days without any pain at all. In addition, the intensity of her pain was now reported as 3/10 maximum with duration of 30 minutes. Palpation of the xiphoid no longer reproduced abdominal pain although some throat tightness was still experienced. Two additional LLLT treatments were undertaken and JS was reviewed six weeks later at which time there was no tenderness over the xiphoid and JS reported infrequent very mild abdominal symptoms and no throat tightness. After reflecting upon her abdominal symptoms JS recalled that they began when she was working as a fruit-picker and had repeatedly performed awkward lifts of heavy crates. In order to lift and move the crates JS arched backward and used her abdomen to support the lift. This brought the edge of the crate into contact with her xiphoid process. The abdominal pain and throat tightness began the day following a particularly strenuous workday. |
A 40-year-old male admitted to another hospital with fever for half a month and pain in lower limbs for 1 week. Ultrasound results revealed deep vein thrombosis of the lower extremity, and laboratory examination revealed a high level of troponin I.\nThe patient was transferred to our hospital. Electrocardiography revealed ST elevation in leads II and III, AVF and V1–3, Q wave was found in leads II and III and AVF. Troponin I level was 4.402 ng/mL. Therefore, a diagnosis of myocardial infarction was confirmed. However, the blood cell count revealed an extremely high white blood cell (WBC) count (81.9×109/L) and low platelet count (50×109/L) that indicated leukemia. The patient underwent bone marrow aspiration that confirmed the diagnosis of acute myelogenous leukemia (M4).\nThe patient then underwent percutaneous coronary angiography, which revealed an occlusion of the proximal right coronary artery (). After the guidewire crossing, there was still no blood flow in the right coronary artery (). Intravascular ultrasound was performed, which showed multiple thrombi in the right coronary artery from distal to proximal. The endarterium was complete without significant atherosclerosis (). After repeated thrombus aspiration, many thrombi were aspirated, but there were still many thrombi that could not be aspirated. There are still serial stenoses in the right coronary artery ( and 1d). To improve blood flow, balloon dilatation was applied in the middle of the right coronary artery, the site of most serious stenoses (). After balloon dilatation, the blood flow partially improved (). Because of the unusual appearance of the aspirated thrombi () and the history of leukemia, the thrombi were sent for histological examination. The histological examination revealed abundant blast cells in coagulative necrosis (), according to the examination of the bone marrow. It indicated that the coronary artery was occluded by a leukemic clot.\nThe patient then received chemotherapy with idarubicin and cytarabine, but he finally died due to Staphylococcus epidermidis septicopyemia. |
A 24-year-old white man, a middle-distance runner (800 m) competing at national level (seasonal best/personal best of 1 minute 52 seconds), developed severe left heel pain in the pre-season in March 2013. His maximum perceived pain intensity was 10 cm on a visual analog scale (VAS) that ranged from 0 to 10 cm, with 10 cm expressing the worst perceivable pain; the athlete had to interrupt all running activity, and severe pain was perceived even when walking or standing. He continued training with aqua jogging and cycling. He got personalized hand-crafted orthopedic gel peads. Two months later he was attended by an orthopedic surgeon, who additionally prescribed oral intake of nonsteroidal anti-inflammatory drugs (NSAIDs) for 8 weeks. The athlete could continue his training but was not free from pain. When discontinuing medication in July 2013, pain returned immediately, and perceived pain intensity during walking was 10 cm on a VAS (range 0–10 cm). Eight sessions of ESWT were thus added to his treatment plan, and were conducted at a German Olympic center. He did not feel better after the treatment and reported a high level of frustration. An MRI was performed in January 2014 showing a calcaneal spur, signs of inflammation at the calcaneal tubercle, and structural changes of the plantar fascia, surrounded by a large edema (see Fig. ). In February 2014 he underwent an open plantar fasciotomy. Four weeks later he was allowed to perform the first units of regenerative running. Pain returned after approximately 1 week of training. An X-ray revealed no pathology and he was recommended to continue with soft training sessions. He received a peppering injection that reduced pain for 12 hours, and NSAIDs were re-prescribed. His running performance remained at a remarkably low level in comparison to his non-injured state, despite regular personalized training sessions. He presented himself at our out-patient clinic in July 2014 (for timeline see Fig. ).\nAn examination identified pain to palpation at the medial calcaneal tubercle and along the medial band of the plantar fascia. Thickening and enlargement of the proximal one-third plantar fascia was noted. Full and pain-free range of motion was noted to his ankle and foot. Standing caused moderate (VAS score, 5 cm) pain; walking caused severe (VAS score, 10 cm) pain. Latent myofascial trigger points could be found in the surrounding muscles: gastrocnemius medialis and lateralis, and tibialis posterior. Apart from these symptoms no abnormalities in his medical or family history which may have been relevant to the medical case were reported and he presented himself in a good mental condition. The diagnosis based on these findings was chronic plantar fasciitis (calcaneal spur syndrome).\nHe was treated with neural therapy (that is, injection of < 1 ml procaine 1%, which is a local anesthetic) of the surgical scar and along the surgical puncture channel. He lay in a supine position on a treatment table. Sessions took approximately 5 minutes. In total, three sessions (at baseline, at week 1, and after 4 weeks) were performed.\nAt the first treatment (March 2015), he described a slurping noise, like “if something filled up the pain origin.” Afterwards he could stand pain-free and walking (not running) was subjectively improved. After the third session the pain had been completely eliminated (VAS = 0 cm). He could return to sports at the former level. Since March 2015 no recurrence of the problem could be observed. No adverse events were observed. |
A 12 year old female presents to the emergency department with a complaint of abdominal pain. She has a past medical history of Hirschsprung's disease with a staged repair. At day four of life she underwent colostomy with resection of the affected colon from the mid transverse colon to the junction of the sigmoid and descending colon. She returned two months later for the second stage of the repair where she underwent a Soave endorectal pull thru procedure with incidental appendectomy. Since that time she has had a good recovery without constipation or diarrhea and with normal bowel function. She had recurrent tonsillitis and underwent tonsillectomy. She takes no medications. The patient complains of a sharp bilateral lower abdominal pain for the past two days that is greatest in the suprapubic region. She has had three episodes of emesis. She denies a change in bowel habits. She does report a low grade fever to 101. She denies dysuria, frequency or hematuria. She denies any history of sexual activity. Her first menstrual period was six weeks ago and her second menstrual period was two weeks ago. She complains of a new watery vaginal discharge for less than one day.\nUpon arrival her vitals are temperature 37.2 degrees Celsius by mouth, pulse 125 beats per minute, blood pressure 124/64 mm/Hg, and pulse ox 96% on room air. She weighs 45 kg. She is in obvious moderate distress due to her pain. Her bowel sounds are normal. Her abdomen is non distended and firm with voluntary guarding. It is diffusely tender, but worse in the bilateral lower quadrants without rebound tenderness. There is no CVA tenderness. On pelvic exam, there are normal external genitalia Tanner stage II-III with intact hymen from six o'clock to nine o'clock position. There are no obvious perineal or vaginal lacerations. A watery blood tinged discharge is present. The rest of her physical exam is unremarkable.\nInitial labs showed a normal metabolic panel. The complete blood count had a normal hemoglobin and hematocrit with a white blood cell count of 14.5 thou/cu mm. There were 64 percent neutrophils and 18 percent lymphocytes with 11 percent bands. Her urinalysis had 219 red blood cells and 85 white blood cells with a large amount of squamous epithelial cells. It was nitrite negative and had large leukocyte esterase. Urine PCR for gonorrhea and chlamydia was negative. The urine pregnancy test was negative. CT scan of the abdomen and pelvis with IV and oral contrast showed normal lung bases, liver, spleen, pancreas, gallbladder, kidneys, and adrenal glands. There were no bowel obstruction noted. Bilateral dilated tubular structures were noted in the lower quadrants, adnexal regions, with wall enhancement and surrounding inflammatory changes consistent with bilateral pyosalpinx. There were no distinct drainable abscesses seen.\nSee Figures , , , and : CT scan of the abdomen and pelvis with intravenous and oral contrast showing bilateral dilated fallopian tubes with pronounced wall enhancement\nShe received IV fluids and morphine for pain control, and she was admitted to gynecology service for IV antibiotics. In the hospital she received IV Ampicillin, Gentamicin, and Flagyl for four days until she was afebrile for forty eight hours and had a normal white count. She was discharged on a ten day course of Doxycycline and Flagyl with Motrin for pain control. At six month telephone follow up she denies any recurrence of her symptoms. |
A 60-year-old man presented with an incidental mass that had been firstly noticed for microscopic hematuria during evaluation of lower urinary tract symptoms. A hyperechoic cystic lesion was firstly detected with ultrasound at proximal to dilated left renal pelvis. Cystic lesion, originated from renal pelvis, containing multilocular cystic structure divided by contrasted septate, and covered with calcified wall in 7 × 6 × 6 cm size (Bosniak-IIF) was displayed on contrast-enhanced computerized tomography ().\nPartial nephrectomy was planned because of suitable size in spite of location at expanding to the renal hilum. The inadvertent minimal opening on the cyst wall caused a change in the decision about operation plan as radical nephrectomy. We prevented any leakage from content of cyst during radical nephrectomy by clamping the two sides of tear on the wall.\nGrossly, the tumor was a multilocular cystic mass filled with chocolate color mucinous fluid (). This morphologic appearance called to mind metastatic spread of ovarian or appendiceal mucinous neoplasia. Although this cyst had morphologically looked like mentioned possibility, patient's gender and medical history, who was male and appendectomised, were not proper to suggested theories.\nMicroscopically, renal parenchymal thinning, multiloculated and mucoid content, and partially papillary structures were defined in this cystic lesion. Histologically, multilayered tall columnar mucinous epithelium forming the cyst wall and resembling intestinal and endocervical epithelium, was seen gaining gradual malignancy potential with formation of glands of different sizes and shapes lined by columnar cells having hyperchromatic and pleomorphic nuclei, and eosinophilic-to-vacuolated cytoplasm (). Also nuclear atypia and stromal invasion which are characteristic features of malignant type of MC were obviously seen. Positive immunohistochemical staining with CEA, CK7, and EMA and negative staining of CK20 fortify diagnosis.\nThe patient did well postoperatively and his followup during 28 months after operation was troubleless. Contrast-enhanced abdominal CT and thorax X-ray film were negative for any local or metastatic invasion at postoperative 24th month. |
A five-year-old Hispanic (non-indigenous) boy was admitted to our hospital with a history of intermittent fever, and was found to have elevated muscle enzymes.\nHis mother reported that he had been taken to a doctor five days earlier and the apparent diagnosis was an intestinal infection, for which he was started on trimethoprim sulfamethoxazole and acetaminophen. The fever was associated with productive cough. After no apparent improvement, she decided to bring her child to our emergency department.\nThe child had been healthy four months before. He had been admitted to our hospital two months prior due to dyspnea and hypoxia, and was diagnosed with a pericardial effusion that caused a tamponade on an echocardiogram, causing right atrial and ventricular collapse (Figure ). Emergency percutaneous pericardial catheter drainage was placed, draining 200 mL of serous clear fluid. A culture from the pericardial effusion was reported sterile; cytology reported no microorganisms or leukocytes, but an increased level of proteins (3400 mg/dL). Acid-fast stain results were negative, as were the results of adenosine deaminase testing. Rheumatoid tests were also performed: anti-double-stranded DNA results were negative and serum complement (C3 and C4) levels were within normal limits. HIV rapid test and serum antibody (IgM) tests for cytomegalovirus and Epstein Barr virus were negative as well. Coxsackie serology was not available. Hypoalbuminemia was also noted at 2 g/dL (reference range 3.4 to 4.8 g/dL) at admission, associated with proteinuria. Tests were inconclusive and the pericardial effusion was considered to be viral. Because of his overall health improvement, he was discharged from hospital one month later, with follow-up planned.\nOur patient did not have any important family, traumatic, or surgical history. He had had viral hepatitis three years previously. No allergies or medication taking were reported. He had normal psychomotor development, and vaccinations had been given on schedule.\nOn physical examination, his vital signs were within normal limits and he was not febrile. His weight was 13.6 kg and his height 98 cm, considered low stature for his age (Z = -3.9). A thyroid palpation revealed no goiter, nodules or pain. Heart sounds were rhythmic and no pericardial rub was heard. An anterior mid-thoracic scar was noted where the percutaneous pericardial catheter had been placed. He had hepatomegaly that was later confirmed on an ultrasound. Results of an anterior chest X-ray were normal.\nHematology tests on admission revealed a total leukocyte count of 6.83 K/UL, hemoglobin level of 12.5 g/dL and a platelet count of 310 K/μL. His blood chemistry results revealed elevated muscles enzymes: lactate dehydrogenase (LDH) of 1117 (reference range 240 to 480 IU/L), a creatine phosphokinase (CPK) of 2874 U/L (reference range 38 to 170 U/L) and creatine kinase MB (CK-MB) of 60 U/L (reference range 0 to 24 U/L). His renal function tests were within normal limits. Hypertrigliceridemia was also noted at 408 mg/dL (reference range 30 to 200 mg/dL). Urine test results showed 500 mg/dL proteinuria and microscopic analysis showed five to eight erythrocytes per field. During the time our patient was hospitalized, his CPK levels were moderately increased (2435 to 4615 U/L). Our patient did not report muscle pain or weakness, and results of a neurological examination were normal. A repeat echocardiogram was performed due to his history, showing normal systolic and diastolic ventricular function with no pericardial effusion.\nConsidering his recent history of cardiac tamponade, thyroid tests were ordered. The results were diagnostic of primary clinical hypothyroidism: free T3: < 1.1 pg/mL (reference range 1.1 to 3 pg/mL), total T3: 34.9 ng/dL (reference range 100 to 200 ng/dL), free T4: 0.2 ng/dL (reference range 0.7 to 1.85 ng/dL), total T4: 1.04 μg/dL (reference range 4.5 to 12 μg/dL) and thyrotropin 299.380 μIU/mL (reference range 0.490 to 4.670 μIU/mL). A thyroid scan with technetium (Figure ) and a thyroid ultrasound were performed showing a normally located and absorbing thyroid gland without nodules (Figure ). Anti-peroxidase (APO) and anti-thyroglobulin antibody test results were negative. Levothyroxine was started at a dose of 9 μg/kg per day. Bone age was also calculated with a radiograph of the left hand and was reported delayed by two years.\nAn electromyogram reported a myophatic inflammatory process involving mainly proximal muscles compatible with myositis. A muscle biopsy was performed that revealed colliquative necrosis without inflammatory cell infiltration (Figures and ). An anti-Jo-1 antibody test result was negative. Rhabdomyolisis was, however, considered to be autoimmune and steroids were given. Three weeks after the thyroid hormones and one week after steroids had been started, muscle enzyme levels (CPK, CK-MB, and LDH) had returned to normal levels. |
A 55-year-old man with no significant past medical history presented for evaluation of worsening chest pain. He reported the symptoms of chest pain and heaviness for two weeks prior to presentation, initially on exertion, which later progressed to occur at rest. On presentation, blood pressure was 110/68 mmHg, and heart rate was 73 bpm. Physical examination was within normal limits. Electrocardiogram showed nonspecific T wave inversion in inferior leads. A limited bedside transthoracic echocardiogram showed a normal left ventricular ejection fraction of 55%, hypokinesis of basal to mid inferior myocardium, and a dilated aortic root of 5.3 cm at the level of sinuses of Valsalva with evidence of only mild aortic regurgitation. Laboratory data, including complete blood count and metabolic panel, were unremarkable, and Troponin levels were normal. Given concern for unstable angina, the patient underwent invasive coronary angiogram via a transfemoral route, which showed no evidence of coronary artery disease in the left circulation. Collaterals were noted filling the right coronary artery (RCA) from the left system (Figure ). The RCA could not be engaged despite the use of multiple sizes and types of catheters; hence the possibility of “flush” occlusion of the ostium of the RCA was entertained. An aortic root angiography was then performed with a 6 Fr pigtail catheter but failed to show the ostium of RCA or any antegrade flow in the RCA. Additionally, there was a significantly dilated aortic root and an unclear linear hypodensity in the right coronary cusp (Figure ). The patient tolerated the procedure well without any remarkable intra-procedural events.\nCardiac computed tomography angiography (CCTA) was performed the next day to avoid contrast-induced nephropathy (Figure ). It revealed a discrete transverse dissection flap visualized in the right sinus of Valsalva, extending 25 mm superiorly. The ostium of the RCA was not communicating with the right coronary cusp. The aortic root was aneurysmal and measured 5.5 cm at the level of sinuses of Valsalva.\nThe patient was sent for emergent cardiovascular surgery. Cardioplegia was delivered retrogradely through the coronary sinus. Intra-operative findings showed bicuspid aortic valve morphology with aortic root aneurysm. There was complete occlusion of the RCA, and the ostium was noted to be arising from the false lumen of the chronic aortic dissection. The patient underwent aortic root replacement and modified Bentall procedure with a 25 mm St. Jude valve conduit with re-implantation of the left coronary artery and single saphenous coronary artery bypass graft to the right coronary artery. The patient had an unremarkable postoperative course. |
A two month old female infant presented to the orthopedic examination room with a palpated mass on the anteromedial side of the proximal left tibia. On the physical examination the lesion was found to be oval in shape, soft, compressible and painless. Anteroposterior and lateral plain radiographs demonstrated swelling of the soft tissue at this part of the tibia without signs of bony erosion (Fig. ). The ultrasound (Doppler sonography) detected a cystic lesion with blood flow, measured 12 mm × 4 mm, while the integrity of the bone cortex was confirmed. The most possible diagnosis was hemo-lymphangioma. In the following six months, despite the fact that the size of the mass increased, the patient remained asymptomatic. A new ultrasound was performed (Fig. ) and measured the mass 23 mm × 6 mm; the origin remained cystic. In the yearly follow-up the lesion's size increased to 44 mm × 37 mm.\nDue to the continuous augmentation of the mass, surgical excision was decided. An MRI (Fig. ) was performed, pre-operatively, in order to establish the extent of the tumor and define the relationship to the surrounding structures. A well-defined extra-articular cystic malformation with a maximum diameter of 45 mm was viewed, located on the anteromedial side of the left tibia. The lesion had fine adhesions to the surrounding tissues and the cortex of the bone was found intact.\nEn bloc resection was performed releasing the specimen from the adhesions to the subcutaneous tissues and the medial head of the gastrocnemious. Macroscopically, it was found oval in shape with harsh features and spotty surface (Fig. ).\nHistological examination described the resected tumor as a fibro-lipomatous mass containing dense fibrous conjunctive tissue with vascular areas of lymphatic cells and vessels filled in with red blood cells (Fig. &). The definitive histological diagnosis was hemo-lymphangioma.\nTen days post-operatively the trauma area was swelling; the seroma formed was aspirated and gave 20 ml of sterile, bloody fluid.\nA week later, the swelling recurred; surgical exploration and lavage was performed, while a drainage was placed. Intravenous Netilmicin and Cefamandile Nafate were administered for a total period of ten days, whereas the leg was immobilized on an above knee splint. The swelling was progressively decreased. A week after, the trauma area was examined by the ultrasound and 1,58 ml's of fluid were demonstrated. A full plaster was placed with the knee joint flexed in 90° for three weeks. At the end of that period of time the volume of the fluid was less that 1 ml. After two months of follow-up by clinical and Ultrasonography examination, the quantity minimized to zero, while in the yearly follow-up no recurrence was observed. |
A 68-year-old, 65 kg male, fruit hawker by occupation was admitted with a diagnosis of bilateral inguinal hernia and was planned for bilateral inguinal hernioplasty under combined spinal epidural anesthesia. He was a known case of hypertension since 4 years; on regular treatment, tablet cilnidipine 10 mg + metoprolol 25 mg once a day. After confirming NBM status and written informed consent, the patient was taken for surgery. Under all aseptic precautions, an 18 gauze, 80 mm Tuohy needle (PORTEX Epidural Minipack System 1) was used to access the epidural space in L3–L4 intervertebral space with loss of resistance technique in the sitting position. The epidural space was encountered 5 cm from skin through a midline approach, and an 18-gauze radiopaque close-ended multihole epidural catheter advanced up to 9 cm after which a resistance was felt. Hence, the whole assembly both needle and catheter were simultaneously withdrawn and a fresh prick taken with the same needle through a paramedian approach in the same intervertebral space (L3–L4). The epidural space was encountered 6 cm from skin and the catheter was advanced cephalad and fixed at 12 cm. The placement of catheter in the epidural space was confirmed by meniscus sign, and no resistance was encountered while injecting the test dose. Furthermore, we did not find cerebrospinal fluid (CSF) or blood during aspiration. Subarachnoid block was then given by a 25-gauze spinal needle through a midline approach in L3–L4 intervertebral space with 3cc of 0.5% heavy bupivacaine and 60 μg injection buprenorphine as an additive after confirming free flow of CSF and negative aspiration of blood. Trendelenberg position was given to the patient, and after achievement of sensory level of up to T8 surgery was started which lasted 2 h. Surgery was uneventful, and patient was vitally stable throughout the procedure. While removing epidural catheter at the end of surgery, it was found that the catheter was broken from almost 15 cm mark []. Immediately, operating surgeons were informed about the event, and an urgent neurosurgery reference was sent for. The patient and his relatives were well informed and counseled regarding the complication. Patient had complete motor recovery after 4 h of spinal anesthesia. He remained asymptomatic in the postoperative period, and there was no feature of local infection, sepsis, or any neurological deficit. No abnormality was detected on neurological examination. It was not possible to detect the retained fragment on digital X-ray of thoracolumbar spine both in anteroposterior and lateral position. Computed tomography (CT) scan of dorsolumbar spine with sagittal and coronal reconstruction was done which revealed a 2–3 cm radiopaque fragment of the catheter near the L4 vertebral lamina outside the canal []. No catheter was seen at any other vertebral level or in the canal or in skin, subcutaneous, and muscle plane. However, since the part of the catheter which was suspected to be inside the patient's body was large (almost about 15 cm) and can be cause of infection in the epidural space, foreign body removal was scheduled by neurosurgeons next morning with exploratory laminectomy under general anesthesia in prone position. A 5 cm incision was taken at the site of insertion of epidural (L3–L4 space). The surgeons gently removed the epidural catheter after dissecting through the layers of subcutaneous tissue, and catheter was found lying in paraspinal muscles []. A total length of 17 cm of epidural catheter was retrieved []. After surgery, the patient was discharged without significant neurological complications. |
A 47-year-old man was admitted to the emergency department of the hospital with left shoulder pain, which was ongoing and exacerbating for 5 days. There were over-sensitivity, swelling and minimally elevation of skin temperature on palpation. Range of motion of the left shoulder was 50 degrees of forward flexion, 40 degrees of abduction and 40 degrees of internal rotation. Blood examination revealed the WBC count to be 5110/mm, CRP 19.02 mg/L and body temperature 36.7°C. Radiographic examination of the left shoulder showed destruction of the joint () and there was dislocation of the joint with a Hill-Sachs lesion on the computerized tomography (). Prior diagnosis was septic arthritis according to these findings. It was also recognized that the patient was diabetic, had similar symptoms on the opposite shoulder 6 years ago, had several surgeries on the right shoulder and had ongoing discharge from a sinus. This history alerted us and a MRI examination of the left shoulder was made which displayed Osteoarthropathic changes and extensive fluid. 250 mL hematoma was depleted by puncture of the joint (). Direct microbiological examination revealed no bacteria and the culture was negative. Thus the prior diagnosis of septic arthritis was cleared off. The elevation of CRP was attributed to discontinuous discharge from the opposite shoulder. The process was considered to be chronic as the temperature of the body and WBC count were normal. When the case history was detailed, it was recognized that he had neck pain and had a diagnosis of syringomyelia. He was out of routine control for several years. A MRI of the cervical vertebrae detected the syrinx (). Consequently, a final diagnosis of Charcot osteoarthropathy of the left shoulder due to syringomyelia was accomplished. The patient was discharged and referred to Neurosurgery for the follow-up of the syrinx and Physical Therapy and Rehabilitation for the rehabilitation of the left shoulder. |
A 39-year-old white American man with a past medical history of hypertension, hyperlipidemia, and major depressive disorder presented to our facility after being transferred from another facility following an attempted suicide. He presented to our intensive care unit; he was intubated due to respiratory distress and hypoxic-anoxic brain injury, secondary to complications of sharp foreign object ingestion as a means of suicide attempt. According to collateral information retrieved from the sending facility, he was seen aspirating blood while undergoing esophagogastroduodenoscopy (EGD) thus requiring emergency intubation. On admission to our facility, an abdominal X-ray showed no evidence of foreign object ingestion. Another EGD was performed at our facility to investigate upper gastric bleeding which revealed a 2 cm ulcer at the base of his esophagus, which was cauterized resulting in control of the hemorrhage. An abdominal axial computed tomography (CT) scan performed on hospital day 18 showed evidence of a foreign object in his descending colon which was later expulsed via bowel movement on day 20. Per transfer records received at our facility, medications included amlodipine 5 mg orally daily, simvastatin 10 mg orally daily, gemfibrozil 600 mg orally twice a day, and niacin 500 mg daily. According to the history obtained from members of his family, there were no reported medication allergies and no past non-prescribed substance use history.\nWithin hours of admission to our intensive care unit, his mental status progressively declined due to hypoxic-anoxic brain injury reaching a Glasgow Coma Score (GCS) of 6. On physical examination there was no eye opening response, no verbal response, and he only merely withdrew from painful stimuli. There was neither decorticate nor decerebrate posturing at the time of examination. Lung auscultation revealed decreased breath sounds in bilateral lung bases. His abdomen was distended, obese, with decreased bowel sounds in all four quadrants and tympanic to percussion. His white blood cell (WBC) count was elevated at 14.9 (103/uL), with 83.6 % neutrophil predominance. A chest X-ray showed bilateral pleural effusion. A sputum culture was positive for Klebsiella pneumonia. His clinical picture was consistent with aspiration pneumonitis with severe hypoxia which later decompensated to ventilator-associated pneumonia with methicillin-resistant Staphylococcus aureus (MRSA) pneumonia (as seen on sputum culture), which thus made him ventilator dependent due to decreased respiratory drive. His treatment was a combination of levofloxacin 750 mg administered intravenously daily, piperacillin/tazobactam 3.375 mg every 6 hours, and vancomycin 1 gram daily. On day 8, a maximum core temperature (Tmax) of 39.6 °C (103.3 °F) was recorded rectally. He had no motor response to painful stimuli, no eye opening, nor verbal response despite being off all sedating agents. His blood culture at this time was positive for Candida albicans and, as a result, micafungin 100 mg administered intravenously daily was initiated. While on this regimen, on day 9, a temperature of 41.6 °C (106.9 °F) was recorded with a GCS of 3. Acetaminophen 650 mg was administered rectally; cooling blankets and multiple ice packs were applied to his groin, axillae, and neck regions resulting in a decrease in his body temperature to 37.8 °C (100.1 °F) within 8 hours of intervention. He continued to have fever on a daily basis with no improvement in cognitive functioning as he remained comatose. On hospital day 11, he had become flaccid and was considered to be in a vegetative state as evidenced by an electroencephalogram (EEG) study, which showed diffuse slowing consistent with encephalopathy with very poor prognosis. His WBC count increased to 27.9 (103/uL) and he remained in a state of septicemia. Due to multiple failed attempts to wean him off the ventilator, a tracheostomy was performed on day 14 and a percutaneous endoscopic gastrostomy (PEG) tube was placed on day 17. Given his neurological decline, a lumbar puncture was considered for cerebrospinal fluid (CSF) analysis. However, he was already on broad spectrum antibiotics, therefore, the benefits from CSF analysis would have been undermined by antibiotic coverage.\nAfter concerted deliberations over the trial of levetiracetam as a neuroprotective and antiepileptic agent, the decision was made on day 29 to administer 500 mg of the medication via a PEG tube at bedtime. Within 48 hours of administration, he was awake and alert but remained disoriented to person, place, and time. His GCS improved from 3 to 14 and he was able to respond to painful stimuli from his lower extremities up to his knees. Amantadine 50 mg/ml via a PEG tube every morning was started for neurocognitive stimulation as he was noted to have decreased cognitive speed measured grossly during conversation evidenced by increased latency of response when questioned. On hospital day 38 (9 days after levetiracetam 500 mg initiation), he was able to communicate verbally, although with difficulties with phonation even with a Passy-Muir valve. There was orientation to person and place but not to time. His body temperature was 37.3 °C (99.1 °F) without antipyretic agents, while his WBC remained elevated at 20.9 (103/uL) showing persistence of infection.\nThe finding of a neurological examination was consistent with critical illness polyneuropathy, as he had no motor function below the clavicle after surviving the profound neurological impairment induced by hyperpyrexia. His pain sensation remained intact, he responded to sharp and dull touch sensations, and he responded to vibrations. He was noted to have decreased cognitive speed; therefore, he required neurocognitive stimulation. Amantadine was initiated on day 34, resulting in improved cognitive functioning with noted increased cognitive speed during conversation. Amantadine was increased to 100 mg/ml via a PEG tube for enhancement of neuroprotection on day 41.\nHe was weaned off a ventilator, then transferred to our medical floor and eventually discharged. Extensive rehabilitation was recommended for neuromusculoskeletal strengthening in all his extremities. A CT scan showed diffuse brain atrophy disproportionate to his chronological age; thus consistent with hypoxic ischemic brain injury.\nFollowing cognitive recovery, he was awake, alert, and oriented to time, place, person, and situation. He remained with a motor strength of 0/5 bilaterally in upper and lower extremities proximally and distally, secondary to critical illness polyneuropathy; however, he had intact temperature and pain sensation to all modalities in the upper and lower extremities, chest, abdomen, and face. He was seen on follow-up evaluation 6 months following initial encounter and he remained cognitively intact. |
An 83-year-old woman presented with acute and progressive weakness of the right-hand extensors with painless swelling in the proximal part of the right forearm. Interestingly, she noticed that weakness after a fall on the outstretched left hand leading to fracture of left distal radius. She was treated conservatively in a cast. Six weeks later, and during routine follow-up, she mentioned that she noticed her right-hand weakness only when she started to use it excessively. She denied any injury to her right-hand or elbow. Examination revealed a swelling 3 × 3 cm in the antero-lateral aspect of the right forearm in the region of supinator muscle. The swelling was soft in consistency and decreased in size on flexion with supination of the forearm indicating that the lump was deep to the brachioradialis muscle. Extension of the metacarpophalangeal joints of all fingers in her right-hand was weakened. Also, there was slight weakness in the wrist joint extension. There was no sensory deficit in any of the dermatomes of the hand or the forearm.\nRadiographs of the right elbow showed soft-tissue density closely related to the proximal radius with normal bone appearance. Electromyography of the posterior interosseous nerve and muscles demonstrated active denervation, but nevertheless there were 1 or 2 recruited motor units confirming continuity of these motor fibres. Magnetic resonance imaging (MRI) of the right forearm revealed a multilobulated mass with hypointensity signal on a T1-weighted sequence which is pathognomonic of a lipoma. The precise anatomical location was in the lateral and anterior aspects of the right radius neck and extending distally for about 3 cm as shown in Figures and . The lesion was explored through anterior approach under general anaesthesia. The radial nerve and its two branches were identified. PIN was traced distally to the level of the proximal margin of volar supinator muscle (arcade of Frohse). There was focal constriction of the nerve at the level of the proximal edge of the supinator muscle. The nerve was slightly atrophied distal to this constriction. The attachment of the supinator muscle was dissected from the radius. The lesion was deep to supinator and was encapsulated and firmly attached to the proximal radius. The lesion then was excised with stripping of the attached periosteum. Histology confirmed the diagnosis of lipoma which was totally encapsulated except at the site of its attachment to bone.\nPostoperatively, with intensive physiotherapy, she noticed improvement in hand and wrist extension. The final check, 6 months later, confirmed that elbow, wrist, and hand function were similar in both sides. |
A 34-year-old male patient consulted the Department of Oral Medicine and Radiology, with a complaint of swelling on his upper left back gums since 3 months []. The swelling had an insidious onset, gradually and constantly increasing in size. There was no preceding pain in the regional teeth. Occasional bleeding and mobility of the upper left back teeth was reported. Associated symptoms of loss of weight and appetite were present.\nMedical history was significant in that he was diagnosed with pulmonary tuberculosis for which he was under treatment.\nPersonal history was significant as well in that he had visited commercial sex workers, repeatedly.\nIntraorally maxillary left buccal gingiva with respect to the teeth 22, 23, 24, 25, showed a sessile swelling, measuring 6 × 3 cm in size, having a fiery red color with superficial ulcerations and a multinodular appearance. It was nontender to palpation and firm in consistency and did not induce any discharge. The swelling had also extended onto the maxillary palatal gingiva in relation to tooth 21, 22, 23. The teeth 21, 22, 23, 24, 25 exhibited grade II mobility. Periodontal pockets were not detected in the region of complaint [].\nThe mid dorsum of the tongue showed an irregular area of depapillaton with diffuse erythema. The hard palate demonstrated diffuse erythema with no evidence of scrapable or nonscrapable white lesions in association, the clinical impression of which was chronic erythematous candidiasis. Rest of the oral mucosa appeared normal.\nThis clinical picture raised a strong suspicion of an underlying immunocompromised status for which HIV-(Western Blot) test was advised, and the patient was found to be reactive for HIV 1 and 2.\nPanoramic radiograph [] and IOPARs taken in the region of complaint showed loss of supporting alveolar bone, with no evidence of any central pathology. A chest radiograph showed the presence of diffuse radiolucencies involving the right upper lobe of the lung. Complete hemogram revealed macrocytic anemia and raised ESR (32 mm/hr).\nAn incisional biopsy was performed from the gingiva. The lesion on H and E showed ulcerated stratified squamous epithelium with underlying connective tissue showing diffuse infiltration by large round cells with vesicular nuclei, single prominent eosinophilic nucleus, and scanty cytoplasm. Increased mitotic activity was evident in the cells. Areas of necrosis and pyknotic cells were also evident. The impression was that of intermediate grade NHL []. Immunohistochemistry was performed with CD 20 marker that showed diffuse positivity in the cells and confirmed the diagnosis of B-cell NHL [].\nThe sections from the gingiva were also stained for AFB, which turned out to be negative.\nFinal diagnosis was that of HIV-associated NHL of maxillary gingiva.\nThe patient was referred to a regional oncology center for the management of NHL as well as to a physician for the treatment of TB and for ART. The patient did not report to either of the treatment centers even on repeated reminders and ultimately succumbed 4 months after the diagnosis. |
The patient was a 61-year-old man with multiple left rib fractures (1–6 ribs), left pneumothorax, left lung contusion, and left thoracic subcutaneous emphysema due to a fall injury. The examination showed a partial depression in the left front rib and abnormal breathing (see Fig. ).\nAdmission chest CT examination: 1–6 rib fractures on the left side (of which 3, 4 ribs are long comminuted fractures (see Fig. )); left pneumothorax, left traumatic wet lung; a small amount of liquid pneumothorax on the left side.\nPatient was given early chest straps, multiparametric monitoring, analgesia, and oxygen therapy. The chest pain was still severe. The visual analogue scale scored 7–8 points for the pain at rest and 9 points for the cough.\nPhysical examination revealed that the left chest wall was recessed and abnormally breathed. The CT scan of the rib showed a long comminuted fracture of 3 and 4 ribs. The key to successful operation was the reduction and fixation of these two rib fractures. A preoperative CT scan was performed to reconstruct the 3D model based on the scan results (see Fig. ), and 3D printing technology was used to prepare 3 and 4 rib models (see Fig. ). The three D print models of each fracture segment of the two ribs were adherently reconstructed.\nThe two rib metal plates were separately shaped according to the reconstruction model (see Figs. and ).\nThe patient is scheduled to have a open reduction and internal fixation of 3–6 rib fracture. After general anesthesia, right side lying position, small incision about 8 cm was performed under the edge of 4th rib underarm. The skin was sequentially incised and the subcutaneous tissue was freed layer by layer. The front of the latissimus dorsi muscle and the anterior serratus were exposed. The tunnel was established on the 3rd and 4th rib surfaces from the back of the chest and small muscles to the back of the scapula. The special long hooks lifted the scapula and exposed the scapular operation space. With assistance of endoscope, the electrocautery is useful to expose 3 cm outside the outermost fracture lines of the 3 and 4 ribs. The locking plate was molded on the surface of the third rib before operation, and the broken end of the non-fracture at the anterior and posterior portions of the third rib was well fitted. The distance between the two ends of the metal bone plate exceeded the fracture line to 3 nail holes distance. Under the thoracoscope, the metal plate and the ribs were temporarily fixed with long-angled forceps. The MIPO system was used to drill the holes. Two screws were implanted and locked at both ends to firmly fix the metal plate. In turn, each fracture segment was reset and drilled and secured to a metal plate. The fourth rib is fixed in the same way. Intraoperative image (Figs. , and ). 5, 6 rib fractures given to fix the ribs, not the content of this article, not elaborated. Sufficient to stop the bleeding, the wound was given to leave a negative pressure drainage tube. After a routine thoracoscopic probe of the chest cavity, a closed thoracic drainage tube was placed posterior to the 7th intercostal space and the incision was closed layer by layer. After the chest wall is well-shaped. Three days after surgery review the map (Fig. ). |
A 59-year-old woman with breast cancer, under Everolimus medication, was brought to the clinic complaining of a chronic swallowing impairment (dysphagia) and pain sensation with a feeling of generalized hot oral mucosa. The patient signed a written informed consent before her engagement in the study. The patient was under 5 mg/day Everolimus (Afinitor) for 2 months. According to the patient, the symptoms appeared the first month of medication intake and persisted. The clinical examination revealed the presence of erythema and ulcers, but these did not interfere with the patient’s diet. According to a meticulous examination of the oral cavity, the patient was diagnosed with oral mucositis grade II of the national cancer institute scale (NCI) (). A speech therapy specialist diagnosed the patient with a chronic dysphagia. The NCI assessment scale for oral mucositis and the functional outcome swallowing scale for staging oropharyngeal dysphagia (FOSS) () [] were used in order to assess the severity of the complication before the treatment and after 24 h of each treatment. According to the FOSS scale, the patient showed a compensated abnormal function manifested by significant dietary modifications and prolonged mealtime with a stable weight and occasional cough with an absent aspiration—therefore a stage II of the FOSS scale []. According to the oral mucositis assessment scale for OM, the patient was diagnosed with a stage 2. The treatment of choice was the therapeutic use of photobiomodulation therapy. For the management of oral mucositis, diode laser 635 nm (smart M Pro, Lasotoronix, Poland) was intraorally applied at energy density of 3 J/point and a time of 30 s, output power of 100 mW, in a continuous and contact mode on four points on the tongue and two on the oropharynx (). Extraoral application of diode laser was conducted with a wavelength of 635 nm, energy density of 3 J/point, output power of 100 mW, and a time of 30 s per point on the following areas: Lips, cutaneous surface corresponding to the buccal mucosae, and bilateral cervical lymphatic chain (). For the management of dysphagia, the parameters were as follows: Diode laser 635 nm (smart M Pro, Lasotronix, Poland) 3 J/cm2 for 30 s on each point, output power of 100 mW, continuous and contact mode. One session of PBM was conducted each 24 h for five days. The intraoral irradiated surfaces were bilaterally: Four points on the soft palate and four points on the oropharynx. The extraoral irradiated surfaces were lateral and ventral pharynx and larynx, midline neck, and lateral neck anterior to sternocleidomastoid muscle (). After treatment, a significant reduction of dysphagia (from stage II to Stage 0) was noted and a significant reduction of the oral mucositis was noted (). Therefore, PBM therapy successfully treated the cancer therapy-induced dysphagia. |
A 58-year-old man presented few natural teeth in the upper jaw (dental elements 1.2, 1.1, and 2.2) and long-term complete mandibular edentulism (). Due to scarce retention of the removable dentures, the patient had not worn them for several years and reported difficulties in eating and speaking, besides aesthetic problems. The patient presented mild hypertension under treatment and antiaggregant therapy and was not a smoker. The primary patient request was a nonremovable rehabilitation. The remaining superior teeth appeared not to be suitable for supporting a fixed rehabilitation, both for their position and for the periodontal attachment loss, and were considered hopeless.\nIn order to plan an implant-supported oral rehabilitation, a preliminary cone beam computed tomography (CBCT) was prescribed to better evaluate the bone volume of both jaws.\nPreliminary radiographic examination through CBCT revealed the presence of sufficient bone volume in the anterior maxilla and poor bone volume in the lower jaw except for the interforaminal area; no periapical radiolucency was discovered in correspondence of the remaining teeth ().\nThe treatment planned consisted in a fixed full-arch rehabilitation of maxilla supported by 5 implants after the extraction of remaining teeth, and a fixed full-arch rehabilitation on 4 implants in the mandible. Both interventions were scheduled to be conducted through a flapless approach after computer-aided planning in order to reduce intraoperative and postoperative morbidity. The strong request of the patient to try everything possible to avoid a provisional mobile restoration led to carefully planning not only implant insertion but also prosthetic rehabilitation. Thus, the patient was informed about the possibility of applying immediate fixed provisional prosthesis if at the time of implant insertions; most of the implants had showed an insertion torque higher than 45 N·cm, as reported by Cannizzaro et al. [].\nThe patient was informed about the treatment and a written consensus was obtained according to local legislation.\nInitially, a biphasic impression in vinyl polyether silicone (EXA'lence Putty and EXA'lence Light Body, GC Europe) of both arches was obtained to have initial stone models. After having simulated maxillary teeth extraction on the upper working model, both stone models were put in a medium-values articulator to realize the diagnostic wax-up in order to previsualize the aesthetic result, also considering the extraction of the residual teeth ().\nAs the remaining teeth showed no pathological mobility and their position could not affect the insertion of sufficient number of implants, they could be used for supporting the surgical guide. However, their extraction would be performed on the same day of implant insertion, as they were not necessary for the definitive restoration.\nThe stone model of the upper jaw was sent to the laboratory, where it was scanned through a model scanner equipped with a computer (7 Series, Dental Wings, Montreal, Canada) to generate STL (STereo Lithography interface format) files. Then, DICOM (Digital Imaging and Communications in Medicine) files derived from preliminary CBCT of the maxilla and STL files from maxilla model scanning were coupled using 3Dyagnosis software (3Diagnosys 4.2, 3DIEMME srl, Italy) so as to be perfectly superimposed, thus obtaining the 3D image that allows the implementation of the intervention planning. Thanks to the presence of the remaining teeth, it was possible to directly couple STL files from the model scan and DICOM files from CBCT using the teeth themselves as reference points, thus avoiding the production of an acrylic guide with radiopaque markers.\nThen, a virtual simulation of the rehabilitation was performed. Once having digitally reproduced the diagnostic wax-up, the position of the implants was planned, considering the bone availability and in a prosthetically driven approach in order to have a favourable emergence of the prosthetic screws (). Once the implant position was planned, the project was sent to a CAM center (3DIEMME srl, Italy), and the guided surgical template, being supported by the remaining teeth, was printed by stereolithography in biocompatible material (class I CE). Together with the template, a model with implant analogue holes was provided. In laboratory, the implant analogues were inserted into the model, and a metal-reinforced acrylic provisional prosthesis—1.5 mm diameter steel bar reinforcement—(Acry Pol LL, Ruthinium Group, Badia Polesine, Italy) to be relined on the abutments in the patient's mouth after the implant positioning was produced based on the diagnostic wax-up ().\nThe following preintervention drug therapy was prescribed:Antibiotic prophylaxis with amoxicillin 2 g 1 hour before surgery Rinse with chlorhexidine 0.20% for 1 minute before surgery\nAt the time of surgery, local anesthesia was administered through articaine with epinephrine 1 : 100000, and implant insertion was performed through a flapless approach. After checking the correct seating of the teeth-supported surgical template, soft tissue plugs in correspondence of the sites of implant insertion were removed with the help of a soft tissue punch through the surgical guide. Then, a sequence of calibrated drills with increasing diameters (RealGUIDE surgical kit, 3DIEMME srl) was used to prepare the implant sites under abundant irrigation with refrigerated physiological solution using an implant motor (i-Surge+, Satelec Acteon, France) at a speed of 800 rpm, while implants were inserted mechanically at 25 rpm with no irrigation and under torque measurement control. The implants inserted had external hexagon connection, diameter of 4.2 mm, and length of 11 or 13 mm (MIS Lance Standard Platform; MIS Implant Technologies Ltd., Karmiel, Israel). All the implants showed an insertion torque higher than 45 N·cm, so the remaining teeth could be removed (), postextractive alveoli were filled with collagen sponges, and screwed provisional prosthesis was delivered.\nIn brief, five temporary abutments (Temporary Cylinder Standard Platform, MIS Implant Technologies Ltd.), which height had been previously studied in laboratory in order not to protrude occlusally from the provisional prosthesis, were screwed on the implants, and metal-reinforced resin provisional prosthesis was directly relined and fixed on the abutments with resin (Splintline, Lang Dental Mfg. Co. Inc., Wheeling, IL) and then refined and polished in the dental laboratory and finally placed in the mouth (). The provisional prosthesis was screwed on the implants and tightened at 25 N·cm, and then the holes for screws were filled with light-curing resin. Resorbable 4.0 sutures were necessary only at the sites of teeth extraction.\nFinal orthopantomography (OPG) was performed to control implant insertion and prosthesis fitting (). Postintervention drug therapy consisted inamoxicillin 1 g twice a day for 5 days, ibuprofen 400 mg 2–4 times a day to be taken where necessary, rinses with chlorhexidine 0.2% for 1 minute 3 times a day for 2 weeks.\nAbout one month later, impressions of the edentulous mandible and of the maxillary provisional rehabilitation were collected, and a radiological acrylic template with five radiopaque markers reproducing the lower diagnostic wax-up and in occlusion with the maxillary provisional prosthesis was produced. The patient underwent a CBCT of the mandible wearing the radiological template, biting into the established position of centric occlusion. DICOM data from CBCT were processed with the 3Dyagnosis software together with STL files derived from stone model and template scans. The implant insertion was virtually planned as previously described ().\nMoreover, the insertion of three anchor pins for the rigid stabilization of the mucosa-supported guide into its appropriate position during the surgical drilling phase was also planned. Files deriving from the project were processed, and the surgical template, the acrylic model, and the provisional prostheses were produced as described above ().\nIn order to provide the correct positioning of the surgical template in the patient's mouth, a silicon index (Occlufast Rock, Zhermack, Badia Polesine, Italy) was realized after seating the surgical guide on the mandible model and putting it in the appropriate three-dimensional relationship to the maxillary model, according to the centric occlusion registered previously ().\nOn the day of surgery, after local anesthesia was administered, the correct seating of the surgical template was checked in mouth and, asking the patient to bite the previously obtained silicon index, it was stabilized in its correct intermaxillary relationship through three anchor pins: a 1.2 mm diameter drill was passed through the three vestibular pin holes of the surgical template under irrigation with refrigerated physiological solution using the implant motor at 1000 rpm and the anchor pins inserted. Once the surgical template was stabilized, the silicon index was removed and four external hexagon dental implants, diameter of 4.2 mm and length of 11 or 13 mm (MIS Lance Standard Platform), were inserted following the same procedure as above. No sutures were necessary. As all implants showed an insertion torque higher than 45 N·cm, the patient could receive provisional prosthesis at the same time of surgery, following the same protocol as for the maxilla (). Minor occlusal adjustments were performed as required, and the access holes were filled. Preintervention and postintervention drug therapy was administered as described above, and the patient was advised to chew only lightly during the first six to eight weeks after intervention. The patient underwent periodical controls to evaluate mucosal healing and to perform occlusal adjustments to the provisional prosthesis. About four months after the second intervention, biphasic impressions in vinyl polyether silicone (EXA'lence Putty and EXA'lence Light Body) were obtained through individual trays and using the provisional prosthesis as implant transfer, according to the pick-up technique. Moreover, bite registration of the provisional prosthesis was also obtained through a silicon base material (Occlufast Rock).\nImpressions were immediately poured to obtain stone models, which were fixed in a medium-values articulator and put in their correct intermaxillary relationship by means of the provisional prosthesis and of the bite registration obtained. Then, articulated stone models were scanned (7Series, Dental Wings), the definitive prostheses virtually designed by the use of a planning software (DWOS, Dental Wings), and related files were transferred to the milling machine.\nDefinitive PMMA monolithic prosthesis was realized from 98 × 20 mm three-layered PMMA blocks (VIPI BLOCK TRILUX®, VIPI Industria, Pirassununga, SP, Brazil) through a 3D milling machine (DWX-50, Roland DG Mid Europe S.R.L., Acquaviva Picena, Italy), according to the manufacturer's instruction. After milling, the milled pieces were removed from the block by sectioning with a diamond disc, the connection peduncles, and mechanically finished and polished using rubbers and gloss, respectively.\nAfter checking the prosthesis adaptation to the stone models, they were delivered to the patients and connection screws tightened at 25 N·cm; screw accesses were filled with light-curing restoration material ().\nThe patient was instructed about oral hygiene behaviors to follow at home, and periodic controls for in-office hygiene were scheduled every four months. After one year follow-up, the patient was satisfied with the result, with good functional and aesthetical integration of the rehabilitation. Only a few accumulations of bacterial plaque were found on the prosthesis between one control visit and another. Good clinical and radiographic results could be reported, with no sign of soft tissue inflammation and limited bone resorption around the implant necks (). |
A 60-year-old man with a past history of RCC (clear cell type, G2, T1b N0 M0 Stage I) treated by a right nephrectomy in June 2015 was required to have a follow-up examination at 6-month intervals after surgery, without the use of an anticancer agent. In January 2018, a routine gastrointestinal endoscopy found an ulcerative lesion of approximately 10 mm diameter in the greater curvature of the gastric body (Fig. ). An endoscopic ultrasonography (EUS) of this lesion showed the first three sonographic layers were blurred, which suggested submucosal invasion. An endoscopic biopsy of the lesion exhibited clear cytoplasm with prominent nucleoli, which was histologically compatible with metastasis to the stomach of the patient’s known RCC. On the other hand, computed tomography (CT) incidentally detected a well contrast-enhancing round-shaped mass in the fundus of the gallbladder (Fig. ). Additional ultrasonography revealed a sessile polypoid lesion, and gallbladder stone and wall thickening were not observed. Although these findings were lacking conclusive evidence of diagnosis whether the gallbladder tumor was primary or metastatic, the circumstantial evidence potentially pointed to the tumor as a metastasis from the patient’s known RCC. 18F-Fluoro-deoxyglucose positron emission tomography combined with CT (FDG-PET/CT) was performed as a preoperative workup to detect other possible remote metastasis. However, specific FDG uptake was not shown, even in the gastric and gallbladder tumors. The blood examination was unremarkable.\nIn February 2018, a gastric wedge resection via laparoscopic and endoscopic cooperative surgery (LECS) technique was applied to the gastric tumor, and laparoscopic cholecystectomy to the gallbladder tumor was simultaneously performed (Fig. ). The operation lasted 190 min with little intraoperative blood loss. Intraoperative pathologic diagnosis was not performed in this case. The hospitalization period after surgery was not eventful, and the patient was discharged on postoperative day 7. Histological examination confirmed that the tumors of the stomach and gallbladder were both metastatic RCC. Immunohistochemical staining was strongly positive for CAM 5.2 and vimentin, supporting the diagnosis. Macro- and microscopic findings are shown in Fig. . Thereafter, the patient required examination every 3 months without the use of anticancer agents and has survived without relapse to 12 months after the surgery. |
In a work-up of a 40-year-old female with a history of low back pain, a routine MRI of the lumbar spine identified an incidental lesion. The axial T\n1 weighted image showed a 21-mm round, homogeneous lesion of low signal intensity interforaminally in the left S2 segment of the sacrum. The axial short tau inversion-recovery image showed a well-defined lesion with an inhomogeneous signal intensity throughout the lesion with high peripheral rim intensity. The axial T\n1 image with fat saturation after contrast media injection showed moderate enhancement throughout the lesion and in the peripheral rim (). A radiograph of the pelvis was performed to further characterize the lesion. On clinical examination, the sacral area was not painful to palpation. Owing to the atypical appearance of the lesion and the non-specific nature of the MRI signal pattern, a bone scan was performed that showed a solitary lesion on the left side of the sacrum with increased metabolic activity. For further characterization, and in the search of a potential primary tumour, positron emission tomography (PET)-CT was performed, which showed a well-defined sclerotic lesion with mild fludeoxyglucose (FDG) avidity (average standardized value 2.5) in the S2 segment and no other abnormalities (). Owing to the metabolic activity of the lesion, the patient was referred to the orthopedic oncology department for image-guided biopsy of the lesion. The biopsy specimen consisted of a few small fragments of bone marrow, some skeletal muscle, fibroadipose tissue and blood clots. Infiltration of the otherwise normal bone marrow with scattered small groups of big foamy cells was identified (). The foamy cells had vacuolated cytoplasm and small centrally located nuclei. The cells were negative for cytokeratin AE1/AE3, CD68, barchyury, Melan A, HMB 45, desmin and smooth muscle actin but positive for S100 protein (). The cells contained multiple lipid droplets and numerous large mitochondria; the existence of the latter was exhibited with antimitochondrial marker (). A pathological diagnosis of hibernoma was made in correlation with the imaging findings.\nOwing to the clinical assessment in correlation with the history of bilateral lumbago, worse after physical activity, the patient was diagnosed with chronic lower back pain. At 1-year follow-up, the patient was being managed with analgesics and physical therapy. |
A 69-year-old Italian Caucasian woman arrived at the emergency department presenting with sudden onset of lower left limb pain. The patient reported no dyspnea, chest pain, weight loss, nausea, abdominal pain, hematuria, myalgias, or arthralgias. Her medical history was notable for type 2 diabetes mellitus and chronic atrial fibrillation. She had no history of coronary heart disease, hypertension, or dyslipidemia, and she did not smoke. Her medications included digoxin and repaglinide, but not anti-thrombotic drugs.\nThe patient's physical examination revealed that she appeared to be in good condition, but she reported discomfort due to pain in the left leg. The patient's blood pressure was 125/85 mmHg, and her heart rate was 80 beats/minute and irregularly irregular. Her respiratory rate was 22 breaths/minute, her oxygen saturation was 98% while the patient was breathing ambient air, and her body temperature was 36°C.\nIn her left leg, femoral, popliteal, and tibial pulses were absent, with pale and cool feet and with slow capillary return. Her right leg was absent of tibial pulse. Her Ankle Brachial Pressure Index was 0 on the left and 0.4 on the right. The motor and sensory functions of the legs were preserved.\nA transesophageal echocardiogram revealed enlargement of the left atrium with a thrombus in its appendange and enlargement of the right atrium. She had no signs of atrial shunt or detectable atherosclerosis in the proximal aorta. A diagnosis of embolizing non-valvular atrial fibrillation was confirmed, and the patient underwent lower left limb Fogarty thromboembolectomy, that successfully restored distal blood flow. The patient was discharged after two days to a low molecular weight heparin regimen as bridging therapy for a warfarin regimen.\nAfter one week, the patient returned to the emergency department with acute ischemic symptoms in her left leg. Angiography was performed, which showed an apparently normal wall of the abdominal aorta, right iliac and femoral artery branches, obstruction of the left external iliac artery and total obstruction of the left femoral artery branches, and obstruction of the right popliteal artery and of the tibial-peroneal axes on both sides. Urgent embolectomy was again performed with subsequent restoration of blood flow, and the patient was discharged under warfarin therapy.\nAfter four weeks, the patient returned with left leg pain at rest. A search for a hypercoagulable state revealed normal thrombin, pro-thrombin, and partial thromboplastin times; normal anti-thrombin III, protein S, and protein C antigens; and a normal protein C pathway, activated protein C resistance, and homocysteine level. Screening for anti-cardiolipin antibodies and lupus anti-coagulant were negative, and her anti-nuclear antibody test was negative. Her erythrocyte sedimentation rate was 8 mm/hour. Her blood cell count revealed normal amounts of red and white cells, and her platelet count was 850,000/μl.\nTransesophageal echocardiography showed resolution of the atrial thrombus; however, an intra-luminal pyramidal lesion (1.4 cm×1.5 cm in size) with an irregular surface was seen on the anterior wall of the descending aorta (Figure ). Computed tomography (Figure ) with contrast medium confirmed the presence of a mural aortic thrombus in the anterior wall of the descending aorta extending 1.5 cm distally, a mural thrombus in the left pulmonary artery localized in close proximity to the aortic thrombus, a lung infarction in the inferior left lobe, an infarction in the spleen, sparse atherosclerotic plaques in the abdominal aorta and the right femoral artery, occlusion of the left external iliac artery, and occlusion of the right internal iliac artery. There were no signs of venous thrombosis.\nThe patient underwent surgical treatment consisting of an endovascular exclusion of thrombus with a Zenith 32 mm×80 mm endoprosthesis (Cook, Inc., Bloomington, IN) implanted just after the emergence of the left anonym subclavian artery, a subsequent thromboembolectomy of the left external iliac artery, and a femoro-popliteal bypass. The patient was discharged after a few days under a low molecular weight heparin regimen as bridging therapy for a warfarin regimen, as well as aspirin. Additional therapy included β-blockers and hydroxyurea, an anti-neoplastic drug acting on nucleic acid synthesis to reduce her platelet count. Her platelet count decreased to 300,000/μl.\nTwo months later an echocardiographic Doppler study revealed the patency of the bypass and the left femoral axis, which previously had been treated by embolectomy. Two months later the patient presented with left leg pain while she was being treated with warfarin. An echocardiographic Doppler scan showed obstruction of the popliteal bypass. Therapy with aspirin, clopidogrel, and warfarin was initiated. Out-patient follow-up was arranged, and eight months later the patient did not report any further embolization. |
A previously healthy 38-year-old white man presented with left leg tightness and soreness. He had no significant past medical history but did have an extensive international travel history from his military service. His family history was notable for a maternal history of bladder cancer; a social history disclosed a remote 13-year chewing tobacco history with current cigar smoking accruing to approximately 1.3 cigar-years.\nOver a span of approximately 6 months, his left leg tightness and soreness progressed to include his left low back along with sensory changes along his left lateral foot and heel that rendered him with intermittent physical limitation. A review of his symptoms was otherwise unrevealing with no constitutional symptoms. A physical examination was notable for “shotty” supraclavicular lymphadenopathy.\nAn initial evaluation at an outside institution was ultimately inconclusive. Serial magnetic resonance imaging (MRI) and computed tomography (CT) of his chest, abdomen, and pelvis showed progressive and aggressive-appearing mixed sclerotic-lytic lesions in his iliac and sacral regions. One sacral lesion extended into the left S1 neural foramen. There was associated widespread lymphadenopathy involving the supraclavicular, mediastinal, abdominal, and pelvic regions. Multiple tiny pulmonary nodules up to 10 mm were also present. All imaging appeared consistent with multifocal osteomyelitis although a malignant or infectious process could not be ruled out.\nThe results of cursory laboratory tests were unremarkable and notable for only a mildly elevated sedimentation rate of 39 mm/hour. Rheumatic serologies and hematologic workup were unrevealing. Extensive infectious studies yielded no identifiable microorganism.\nMultiple biopsies were sought. A left axillary lymph node excisional biopsy disclosed reactive lymphadenopathy with scattered pigment-laden macrophages and extensive fatty replacement. Right and left iliac bone marrow biopsies showed marrow fibrosis with focally prominent chronic inflammatory infiltrates. There was no evidence of mycobacterial or fungal organisms. Bone marrow flow cytometry showed no monoclonal B cell population. All biopsies were without evidence of lymphoma or any other neoplastic processes.\nA provisional diagnosis of non-bacterial chronic recurrent multifocal osteomyelitis (CRMO) was given. He was treated with opioids, and then transitioned to non-steroidal anti-inflammatory drugs (NSAIDs) and a course of physical therapy. His symptoms improved on this treatment regimen. He received no glucocorticoid during this time.\nBy the time of presentation to our institution, his clinical course had significantly improved over several months but had not resolved. A repeat positron emission tomography (PET)-CT scan showed hypermetabolic foci throughout his torso involving lymph nodes, bones, and lungs as pictured in Fig. . The largest lymph nodes measured up to 1.6×2.0 cm at the left supraclavicular fossa, 2.0 cm at the celiac chain, and 3.1×1.8 cm at the anterior aortocaval chain. Availability of prior study measurements were lacking for comparison. Lytic-sclerotic lesions were re-demonstrated and involved his sacrum, left posterior iliac, right supra-acetabular iliac wing, and left anterior tenth rib. Multiple sub-centimeter lung nodules were re-demonstrated in the upper lobes of his left and right lungs. No splenomegaly was present.\nAn open bone biopsy of his left iliac crest was then performed and finally revealed a diagnosis of classical Hodgkin lymphoma after 13 months of diagnostic uncertainty. Histologic study demonstrated fibrosis and an inflammatory infiltrate. Newly noted were collections of mummified and lacunar cells with abundant basophilic cytoplasm. Also observed were large multilobed nuclei consistent with Reed–Sternberg and Hodgkin cells as seen in Fig. .\nHe was started on a doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) chemotherapy regimen without radiation therapy. Significant clinical and radiologic responses were seen after two cycles. A whole body PET-CT revealed significant reductions in metabolic activity with resolution of his presenting symptoms. After completion of six cycles, the PET-CT was consistent with complete remission. |
A 35-year-old male patient presented with swelling in the left side of the neck. USG and magnetic resonance imaging (MRI) revealed a well-marginated mass lesion measuring 3.8 cm × 2.8 cm at the left carotid bifurcation [] splaying the proximal internal and external carotid arteries, with circumferential area of contact with ICA of 180°-270°, consistent with the imaging diagnosis of Shamblin stage 2 CBT. Subadventitial excision of CBT was done after retraction of the carotids. No special technique was used to visualize the tumor and the excision was simple. Proximal and distal control of the internal, external, and common carotid arteries was done over vascular loops and the vessel loops were used to retract the external and internal carotid artery bifurcation. There were no arterial or nerve injuries. No preoperative embolization was planned. The postoperative course was uneventful and the patient was given intraoperative heparin based on body weight. No neurological deficits were observed at recovery or in the immediate postoperative period. The patient developed right-sided dense hemiplegia on the morning of the first postoperative day, with a score of 29/48 according to the Scandinavian Stroke scale.[] Plain computed tomography (CT) of the brain [] revealed a recent infarct involving the left capsuloganglionic region and corona radiata (middle cerebral artery territory). Color Doppler of the carotid arteries [] revealed partial thrombosis of the left ICA with approximately 50% reduction in vessel lumen and hypoechoic hematoma around the proximal ICA, consistent with a diagnosis of postoperative dissection. Patient was managed conservatively with low molecular weight (LMW) heparin. In view of ICA thrombus, oral warfarin was started and LMW heparin was stopped after 5 days. Two weeks later, a follow-up carotid Doppler study [] showed a pseudoaneurysm measuring 2.3 cm × 1.5 cm in the medial aspect of proximal left ICA with a central patent lumen of size 10 mm × 8 mm and a mural thrombus around it. Neck of the pseudoaneurysm measured 2 mm. ICA thrombus had significantly resolved with good distal flow. Carotid angiography done on the 17th postoperative day [] showed a pseudoaneurysm measuring 2.3 cm × 1.8 cm, medial to the proximal left ICA filling through a small neck of 2 mm []. Intimal irregularity was noted in the lateral wall of the left ICA, suggesting arterial injury/dissection. The neck of the pseudoaneurysm was very narrow, so a 0.018-inch guide wire could not be negotiated and attempts to close the pseudoaneurysm were not successful. Microcatheters and stent grafts were not available in our hospital, as it was a primitive hospital set-up. Non-invasive methods were avoided in this patient because he developed stroke on the first postoperative day, mandating anticoagulation. In view of the ICA pseudoaneurysm and no significant intraluminal thrombus in ICA on angiogram, he was started on oral antiplatelets (aspirin) on the 18th postoperative day and oral anticoagulant was stopped after 2 days. But the pseudoaneurysm had spontaneously thrombosed with no demonstrable flow into the aneurysm in follow-up Doppler done after a week of stopping anticoagulants. There was minimal hypoechoic intimal thickening and irregularity of the proximal ICA with no significant lumen narrowing, thrombus, or false lumen. The patient was continued on oral antiplatelets for 6 months, and is on regular follow-up for 5 years. His neurological weakness has improved significantly with regular physiotherapy. |
A eighty-one-year-old African American female patient presented to the hospital with the complaints of dysphagia and loss of appetite for past few weeks. She had a past medical history of right RCC, clear cell carcinoma subtype with bilateral adrenal metastasis (T3b, N0, and M1) diagnosed in May 2006. At that time, she was treated with right radical nephrectomy and right adrenalectomy followed by radiofrequency ablation of left adrenal metastasis and systemic chemotherapy with sunitinib. She tolerated the treatment well with adequate control of her malignant disease for 11 years. Recently, in June 2017, she presented with the complaints of dysphagia predominantly for solid food, loss of appetite, and generalized fatigability. Physical examination was unremarkable. Esophagogastroduodenoscopy with endoscopic ultrasound showed a distal esophagus mass causing high-grade stricture extending up to the adventitia with no mediastinal lymphadenopathy. Metallic esophageal stent was placed in, and biopsies were obtained which showed adenocarcinoma of esophagus (T3N0M0). Computed tomography (CT) of the chest and abdomen showed distal esophageal thickening along with incidental findings of solid mass lesion involving the inferior pole of the left kidney measuring 3.4 × 2.6 cm in the largest dimension () and a well-defined hypodense nodule in the right lobe of the thyroid gland measuring 2.1 × 2.8 cm in the largest dimension (). Biopsy of renal mass was done which showed neoplastic cells with clear cytoplasm arranged in nests and mitotic figures suggesting clear cell carcinoma (). Fine-needle aspiration (FNA) from the thyroid nodule also identified neoplastic clear cells on cytology raising possibility of metastasis from RCC (). This was confirmed with immunohistochemical stain, which showed atypical cells to be positive for PAX8 () and CAIX while negative for TTF-1. Positron emission tomography (PET) scans showed increased uptake in the distal esophagus, left renal mass, and right thyroid nodule. A diagnosis of concurrent distal esophageal adenocarcinoma along with left renal RCC recurrence and thyroid metastasis was made. The patient was planned for left nephrectomy and thyroidectomy. However, the patient wished to opt out for any surgical intervention or aggressive medical therapy and preferred to be treated with comfort care measures. The patient was treated with palliative intention and died 2 months later. |
A 53-year-old man with a history of opium addiction was admitted at the emergency ward with decreased level of consciousness (GCS=3) resembling methadone poisoning. Then, the patient was admitted to the Intensive Care Unit (ICU) and associated work-up such as brain CT scan was performed.\nSpiral brain CT scan with IV contrast reported a 57×47 mm heterodense mass with cystic and hemorrhagic components and severe peripheral edema with pressure effect on the right lateral ventricle in the right temporoparietal lobe, which enhanced obviously with contrast. Midline shift to left side was also evident. As a result of imaging findings, the patient was transferred to the neurosurgery ward for surgical treatment ().\nIntraoperatively, an ill-defined lesion was seen with foci of hemorrhage suspicious for ICH or hemorrhagic brain tumor. Some parts of the mass had firm to hard consistency. Incisional biopsy was performed due to the large size of tumor and infiltrative borders.\nThe specimen received in the pathology ward consisting of multiple creamy brown fragments of brain tissue in total measuring 2.5×2×1.5 cm. The paraffin block and slides were prepared and stained with hematoxylin and eosin. On histological evaluation, brain tissue was seen with an infiltrative neoplastic growth, which showed a biphasic pattern, composed of malignant glial and mesenchymal components. The glial component showed increased cellularity with cells arranged in diffuse infiltrating sheets, having pleomorphic hyperchromatic nuclei arranged in fibrillary matrix. Many cells with bizarre nuclear atypia interspersed with areas of pseudopalisading necrosis. Microvascular proliferation was not seen. The mesenchymal component contained spindle cells arranged in fascicles and sheets, along with nuclear atypia and mitoses. Areas of well-differentiated cartilaginous elements containing atypical chondrocytes with bizarre oval to spindle nuclei lying in lacunae and some of them containing more than one nuclei in one lacunae, surrounded by chondroid stroma, were seen. Some small areas also showed epithelial differentiation as adenoid formations (-)\nReticulin stain showed increased deposition of collagen within the mesenchymal areas (). On immunohistochemistry, the glial component was GFAP positive. The mesenchymal component was positive for vimentin and negative for GFAP. S100 was positive in both glial and mesenchymal components (). EMA showed positive reaction in the adenoid epithelial component. IDH1 had negative result in both glial and mesenchymal components. P53 and Ki-67 show 50% and 30% reaction in tumor cells, respectively ().\nOn the second day of surgery, cardiac arrest happened and the patient died. |
A 38-year-old female with an unremarkable past medical history initially presented to an outside facility with acute-onset low back pain. MRI showed a fluid-filled appendix, and a subsequent CT scan raised concern for acute appendicitis. A laparoscopic appendectomy was performed on the 11th of August 2015 with intraoperative findings of a swollen appendix without any evidence of rupture. Frozen section of the specimen revealed at least low-grade dysplasia with negative margins, and the procedure was terminated at that point. Final pathology revealed invasive adenocarcinoma with evidence of perforation and normal mesoappendix making this a T4Nx tumor. Peritoneal washings revealed tumor cells present.\nThe patient was then referred to our institution for consideration of CRS/HIPEC. After multidisciplinary tumor board discussion, the patient underwent 3 months of XELOX therapy with plans for interval CRS/HIPEC. In January 2016, the patient underwent laparoscopic right hemicolectomy, omentectomy, and HIPEC. Prior to incision, 1 g of ertapenem was given per routine. No gross disease was appreciated on exploration. The placement of the HIPEC cannulae was performed through the laparoscopic extraction site of the colon and omentum, essentially facilitating a minimally invasive HIPEC. Per institutional practice, HIPEC involved administration of 40 mg MMC with a target intraperitoneal temperature of 41 °C for 90 min. Per institutional protocol, mitomycin C is given at a fixed dose and not dosed by body surface area. Thirty milligrams is administered at time 0 min, and 10 mg administered at time 60 min. Total perfusate is 3 L of normal saline.\nDuring the operation, the patient received 2.2 L of total fluid, primarily lactated Ringer’s solution, over the course of 5 h. No blood products were given, and the patient made 265 mL of urine during the case with an estimated blood loss of 50 mL. The patient’s fluid balance over the next 24 h was essentially even, 2.5 L of saline and 1.8 L of urine output recorded. No blood products were given postoperatively either.\nOn postoperative day 2, the patient developed acute respiratory distress with increasing oxygen requirements. She was febrile to 39.5 ° C and acutely tachycardic, with a heart rate of 143 bpm. She was placed on a partial rebreather and transferred to the surgical ICU. A CT scan was obtained, which ruled out pulmonary embolism but showed marked edema and infiltration of the lungs (Fig. ). The patient was started on vancomycin/piperacillin-tazobactam/azithromycin for presumed pneumonia. She was given 20 mg furosemide intravenously with excellent diuresis. She received an additional dose on postoperative day 3. Nasal swab for respiratory syncytial virus and Legionella and Strep pneumoniae urine antigen studies were sent and ultimately returned as negative, and WBCs were within normal limits throughout this event. Additional studies including sputum and blood cultures were all negative.\nOn postoperative day 4, the patient’s respiratory status continued to worsen despite the use of intermittent bilateral positive airway pressure (BiPAP), ultimately requiring intubation. Phenylephrine was also administered for blood pressure support. At this time, chest X-ray showed increasing pulmonary opacities (Fig. ). The pulmonology service was consulted on postoperative day 5, and their team concluded that this patient had ARDS of uncertain etiology given negative infectious workup to date. Bronchoscopy was recommended if the patient failed to improve.\nHowever, following intubation, the patient rapidly improved and was extubated by postoperative day 7. Though she remained essentially afebrile from postoperative day 2 until discharge (Tmax no greater than 38 C), empiric antibiotics were continued until discharge, with discontinuation of azithromycin on postoperative day 7. Repeated chest X-ray was obtained and showed marked improvement (Fig. ). The patient continued to improve with no complications following extubation and was discharged home on postoperative day 10 without need of supplemental oxygen, tolerating a regular diet, and with return of bowel function.\nAt no point during the postoperative course was there ever any evidence of abdominal sepsis to explain the pulmonary findings. Throughout her postoperative course, her abdomen remained appropriately soft; she had an early return of bowel function, with no clinical evidence of peritonitis. Furthermore, an abdominal CT scan done at the onset of respiratory insufficiency on postoperative day 2 showed normal postsurgical changes, but no fluid collection or other evidence of anastomotic leak, making an abdominal source for her pulmonary toxicity unlikely.\nThe patient experienced mild neutropenia 5 days following the onset of respiratory distress, which resolved within a few days. Prior to her operation, the patient had a white blood cell count of 3.9 × 109 cells/L, which increased within the normal range to 7.9 × 109 at the onset of respiratory insufficiency. Her white blood cell count then progressively decreased to a mild neutropenia, reaching 2.6 × 109 by postoperative day 7. At the time of her discharge her neutropenia had resolved, with a count of 8.0 × 109.\nAt the patient’s most recent follow-up appointment, over 1 month postoperatively, she reported no shortness of breath or chest pain, with an oxygen saturation of 99 % on room air with a respiratory rate of 16. Overall, she is doing well from a respiratory standpoint. She is scheduled to restart systemic chemotherapy. |
A 51-year-old female presented with a history of breast lump for past 6 months. There was a history of rapid increase in size with pain and ulceration for 1 month. She had lost 6 kg of weight in 2 months. There was no history of breast malignancy in the family. On examination, left breast showed a huge foul smelling fungating mass with absent nipple and areola, occupying almost entire left breast. Axillary lymph nodes were palpable. Trucut biopsy elsewhere reported as phyllodes tumor. Mammogram was not done as the tumor was ulcerated. Positron emission tomography scan revealed large hypermetabolic mass in the left breast and few hypermetabolic lymph nodes in the left axilla. There was no evidence of metastasis elsewhere in the body. With the diagnosis of phyllodes tumor probably malignant with skin ulceration, mastectomy with axillary dissection was done.\nSpecimen revealed a large tumor ulcerating and erupting out through the skin. Nipple and areola were absent. The tumor measured 15.5 cm × 13 cm × 7 cm with lobulated margins. An another firm tumor measuring 2.5 cm with spiculated margin was seen at the periphery of large tumor, abutting it []. Histology of the larger tumor showed stromal predominance occupying a low power field [], increased cellularity, spindle cells with moderate atypia, and increased mitosis [] of 13/10 high power field (HPF). Scattered ducts in clefts and leaf like pattern were seen with double layered epithelium. Tumor was ulcerating the skin with surface necrosis. At its periphery, an epithelial tumor is seen without transition zone in between [], consists of cells in tubules and trabeculae with stromal desmoplasiar []. Moderate atypia increased mitosis, and lymphovascular emboli were noted. Eleven axillary lymph nodes were involved by invasive carcinoma. A diagnosis of malignant phyllodes tumor with ulceration with coexisting invasive carcinoma of no special type was rendered. She was currently on chemotherapy and radiotherapy. |
A 69-year-old Caucasian male was referred to our hospital with 3 weeks of abdominal distension and worsening right lower quadrant pain. He was diagnosed with IgG kappa multiple myeloma four years prior to presentation. He was initially treated with bortezomib/dexamethasone with monthly zolendronic acid with good response initially; however, a year after diagnosis, he was found to have disease progression which manifested as a right radius fracture. His regimen was switched to lenalidomide with dexamethasone with good response and clinically depressed levels of paraproteins. After completion of 9 months of therapy, he underwent autologous stem cell transplant with high-dose melphalan. 7 months after bone marrow transplant, his disease progressed with involvement of pericardial fluid. Salvage therapy was initiated with pomalidomide, bortezomib, and dexamethasone which was discontinued a year later due to peripheral neuropathy; however, at the end of treatment, there was no evidence of ongoing disease.\nWhen the patient presented to our hospital, he had an acute abdomen. Initial blood work revealed a normocytic anemia with hemoglobin of 8.4 g/dl and elevated ESR of 44. He also had acute kidney injury with creatinine of 3 mg/dl (baseline of 1.9 mg/dl). CT scan of the abdomen and pelvis revealed extensive stranding seen throughout the abdomen within the peritoneal space with edema in the mesentery ().\nHe underwent an exploratory laparotomy which revealed induration of the entire base of the mesentery and retroperitoneum. He had an IgG level of 4407 units with predominantly kappa light chains whose level was 4833 units (kappa to lambda ratio 540). Pathology revealed extensive mesenteric infiltration by kappa restricted plasma cells positive for CD138 on immunohistochemistry, without evidence of amyloidosis. Bone marrow biopsy revealed a 30% involvement by plasma cells (Figures –). Cytogenetics showed 1q22 duplication, trisomy 7 and 15, and gain of 8q24.1. The skeletal survey revealed lytic lesions in the left femur and skull (Figures and ).\nHe was started on carfilzomib and dexamethasone therapy for relapsed multiple myeloma. Unfortunately, he died within one day of start of the chemotherapy from surgical complications of bowel obstruction. |
A 42-year-old male, nonsmoker, with medical condition significant for hypertension presented to the emergency department after a fall followed by two episodes of seizures. On presentation physical examination was notable for altered level of consciousness and mild symmetrical decrease in power of 4/5 in all four limbs. Laboratory workup including complete blood count, electrolytes, coagulation panel, lipid profile, urine, and serum drug screen was unremarkable. CT scan head revealed a 1.5 cm left temporoparietal lobe intraparenchymal hemorrhage with surrounding edema as shown in (). As part of the diagnostic workup, an ECG was also performed on admission which was normal. The patient was admitted to the neurointensive care unit (NICU) for further management. A computerized tomography angiogram was performed, which showed early draining veins at the site of the lesion, suspicious for an underlying vascular malformation. Subsequently a cerebral angiogram was performed which confirmed the presence of an AVM underlying the hemorrhage (). A partial embolization of the AVM was performed, and the patient was boarded for surgical resection ().\nOn day 3 of admission, the patient complained of sudden-onset chest pain. He described it as left sided, retrosternal, sharp, nonradiating pain, worsened when lying down on left side, lasted 2-3 minutes and then resolved spontaneously. It did not recur however prompted an ECG which showed sinus rhythm with nonspecific ST segment elevation in leads V3-V6 (). Cardiology was consulted who deemed the ECG changes as J point elevation suggestive of benign early repolarization and not a true acute coronary event. A high sensitivity cardiac troponin assay done immediately and repeated two times at 6 hours and 12 hours from the onset of symptoms remained negative (<0.017 ng/ml; normal value <0.057 ng/ml). A transthoracic echocardiogram (TTE) performed later that day revealed no regional wall motion abnormalities or left ventricular dysfunction. The next day, patient was taken for craniotomy and surgical resection of the AVM (). The surgery was uneventful. A follow-up ECG on the postoperative day 1 revealed pronounced ST elevation with new T wave inversions (in leads V2-V6) highly suggestive of acute STEMI (). The patient was completely asymptomatic with no chest pain or other cardiac symptoms. Serial estimation of high sensitivity cardiac troponin was again negative (<0.017 ng/ml) and a repeat TTE was unremarkable. Given these findings and the absence of the symptoms, no intervention was done and he was monitored in the NICU.\nThe patient did not have any further untoward event(s) and continued to do well postoperatively with normalization of his ECG changes over the next 48 hours (). He improved neurologically and was transferred out of the ICU on day 7. He was subsequently discharged on day 12 with home health physical therapy, neurosurgery, and cardiology follow-up appointments. An exercise stress test was eventually performed 3 months' after discharge which did not reveal any evidence of coronary artery disease. |
A 25-year-old North-Indian male presented to our center with history of recurrent painful swelling below the right lower lid for the past 1 year with associated watering. He also gave history of chronic sinusitis for the past 10 years. There was no history of purulent or serosanguineous discharge. He was diagnosed elsewhere as a case of chronic dacryocystitis of the right lacrimal sac and treated with systemic antibiotics with no resolution in symptoms. A computerized tomography (CT) scan was done 1 year back which reported a small oval swelling in the right medial canthal region with deviated nasal septum, bilateral small maxillary sinus polyps, and concha bullosa.\nOn examination, vision was 6/6 N6 in both eyes. Anterior and posterior segment examinations of both eyes revealed normal findings. There was fullness of the right lower lid with erythema, induration, and tenderness. Regurgitation on pressure on the lacrimal sac (ROPLAS) was negative and syringing showed the lacrimal drainage system to be bilaterally patent. A clinical diagnosis of right partial nasolacrimal duct obstruction with preseptal cellulitis was made. An otolaryngology opinion was sought. Nasal endoscopic evaluation revealed a deviated nasal septum to the left with giant concha bullosa with tenderness and congestion over the lacrimal sac area. The patient was started on systemic antibiotic and nonsteroidal anti-inflammatory agents. However, there was no clinical improvement even after 1 month. Since his clinical features were suggestive of idiopathic orbital inflammatory disease (IOID), a trial of systemic steroids was given. The patient reported symptomatic relief after 2 days.\nMeanwhile, the patient underwent right conchoplasty under general anesthesia by the otolaryngologist based on the history of recurrent episodes of pain and diagnostic nasal endoscopic findings. During surgery, area around the lacrimal sac was found to be inflamed and a tissue biopsy was taken. It revealed multiple sporangia and sporocysts of R. seeberi with chronic inflammation.\nIn view of the diagnosis, it was decided to excise the lacrimal sac after counseling the patient about postoperative epiphora. Right dacryocystectomy was performed under local anesthesia. There was brisk intraoperative bleeding. The sac was removed completely along with nasolacrimal duct with wide excision and generous electrocauterization of the surrounding margins. The wall of nasolacrimal duct was also cauterized. The lacrimal sac fossa was copiously irrigated with 5% povidone-iodine solution.\nOn histopathological examination, the lacrimal sac showed fibrocollagenous tissue with dense lymphocytic infiltration. Multiple vascular channels were seen with areas of hemorrhage. Numerous sporocysts in various stages of maturation were seen just underneath the stratified squamous epithelium of the lacrimal sac [].\nThe patient has been counseled about the chances of recurrence of infection and advised periodic checkup. |
The 54-year-old male patient was diagnosed with bipolar disorder for 39 years, with a past history of duodenal ulcer, but no other digestive system disease, metabolic syndrome or food poisoning. He was admitted to the first hospital in October 2017 for recurrent abnormally elevated arousal energy level for a week, where he received 1500 mg sodium valproate, 800 mg quetiapine and 0.75 g lithium carbonate each day. At the beginning of treatment, routine examinations indicated that the level of amylase was 82 U/L, while other laboratory indices and abdominal ultrasound scan were normal. All of a sudden, the patient felt nausea and vomited. The physical examination revealed no skin or scleral icterus. However, the clinician found that the level of amylase increased to 770 U/L, with the blood sodium valproate concentration of 113.0 mg/ml (normal: 50–125 μg/ml). The patient therefore was discharged and referred to the second hospital for possible acute pancreatitis. In the second hospital, further tests were carried out. The results of computed tomography demonstrated an increased pancreatic volume and swollen peripancreatic fat tissue and the contrast-enhanced computed tomography scans on the next day showed similar results. By now the patient was diagnosed with acute pancreatitis. He was to start a fasting diet, trypsin inhibition and gastric mucosa protection in addition to the bipolar disorder medication prescribed at the first hospital. Two days later, however, the patient refused to go to the second hospital to continue with his medications. To keep on with his treatment for acute pancreatitis as well as to stabilize his mood swing, his sister brought him to our hospital on December 2017. He was admitted and routine examinations were carried out. The results of blood tests indicated that serum and urine amylase were 428 U/L (normal range: 0–220 U/L) and 1316 U/L (normal range: 0–620 U/L), respectively, whereas blood cholesterol (normal range: 3.10–5.70 mmol/L), blood triglycerides (normal range: 0.45–1.95 mmol/L), blood calcium, glutamic-oxalacetic transaminase, glutamic-pyruvic transaminase, urea nitrogen, creatinine and lithium carbonate (0.7 mmol/L) were normal, upon which we continued his treatment plan for bipolar disorder that set out in the first hospital and acute pancreatitis in the second hospital, while the serum levels of amylase were routinely examined.\nFor the first few days of treatment, the level of serum amylase was reduced to 220 U/L, thus we considered the withdrawal of acute pancreatitis treatment. The patient was allowed on a clear liquid diet. However, had been symptom-free for several consecutive days, abdominal pain and diarrhea re-emerged unexpectedly and the blood level of amylase increased to 320 U/L. Considering that the patient exhibited no history of gallstone or other reasons that would induce pancreatitis, we believed that sodium valproate was the medication that held responsible so we decided to change his treatment plan, where dosage of lithium carbonate was increased to 1.0 g/day. As a result, his abdominal pain disappeared, the blood amylase level dropped to 208 U/L, and the concentration of lithium carbonate was found to be 0.89 mmol/L. A few days later, without any symptoms, his blood amylase level elevated to 362 U/L. However, since he had no more symptoms of acute pancreatitis and his mood was stable, his sister asked for him to be discharged. Although we have explained the patient was not clear from acute pancreatitis and we would not recommended him to go home without full recovery, the patient’s sister insisted. We then followed up the patient by phone. At the time of this case presentation, his sister reported that he slept well at home, went back to work, and the blood amylase level was 194 U/L. |
A 79-year-old white man arrived by ambulance to our emergency department with chief complaints of lower abdominal pain and profuse diarrhea. On examination, general signs of poor health were observed. His blood pressure was 150/60 mmHg and pulse rate 90 beats per minute (bpm). His temperature was 37.3 °C and he did not appear to be in distress. A physical examination did not reveal any bowel obstruction. His laboratory findings were within normal range except for an elevated white blood cell count (14.5 × 109/L), slightly low red blood cell count (3.12 × 1012/L), and slightly raised level of creatinine (124 μmol/L). His medical history included recent completion of radiotherapy treatment for a Gleason score 9 prostate adenocarcinoma, previous cerebrovascular event, asbestosis, atrial fibrillation, and chronic lymphoid leukemia. His abdominal surgical history included only an open appendectomy 40 years prior. He is a retired marine engineer and lives with his wife. He reported that he did not drink alcohol and had quit tobacco smoking 30 years ago. His family history is noncontributory.\nComputed tomography (CT) of his abdomen and pelvis was performed and demonstrated a peritoneal recess containing multiple small bowel loops over the anterior side of the intraperitoneum lying between peritoneal lining and the transverse abdominis fascia consistent with a pseudoherniation of a preperitoneal subtype (Fig. ). There was no mass or dilatation of bowel loops and no mass to his upper abdominal organs or retroperitoneum.\nIn view of CT abdomen findings, a surgical opinion was sought. As there was no evidence of bowel compromise, he was managed non-operatively. Fecal polymerase chain reaction (PCR) tested positive for Salmonella and our patient improved on antibiotics with resolution of abdominal pain. He was discharged and followed up 2 weeks later at out-patient clinic and had no abdominal discomfort; therefore, no elective hernia repair was planned and further follow-up was not indicated. |
A 53-year-old otherwise healthy man presented with fevers, joint pain and abdominal pain of several weeks duration. He was found to have an elevated bilirubin of 1.8, aspartate aminotransferase of 171, alanine aminotransferase of 265 and alkaline phosphatase of 466. An abdominal ultrasound showed a thickened gallbladder wall without obvious cholelithiasis and a normal common bile duct. CT scan did not show any significant findings (Fig. ). By hospital day number two, the patient developed a leukocytosis of 15 000 and it was felt that he had clinical acute cholecystitis and surgery recommended. Given the lack of dilated biliary system on ultrasound and computed tomography (CT), the decision was made to forego magnetic resonance cholangiopancreatography (MRCP) and proceed to surgery with intra-operative biliary evaluation via cholangiogram. Endoscopic ultrasound is not available in our institution. A laparoscopic cholecystectomy with intra-operative cholangiogram was performed. The cholangiogram showed no evidence of choledocholithiasis (Fig. ). The gallbladder pathology showed only chronic cholecystitis without gallstones and was without evidence of lymphoma in the gallbladder tissue. Postoperatively, the patient's bilirubin continued to climb to a peak of 10.2, a white blood cell count of 16 000 and hemoglobin <9 within 4 days of surgery. Additionally, the patient had daily intermittent fevers to over 101 F after surgery. A post-operative CT showed no acute complications (Fig. ) and MRCP showed normal biliary drainage without damage to the bile ducts or residual choledocholithiasis. Given the normal MRCP, the gastroenterology service recommended a liver biopsy rather than endoscopic retrograde cholangiopancreatography. The liver biopsy revealed IVLBCL. A lumbar puncture with flow cytometry and magnetic resonance imaging scan of the brain were negative for lymphoma involvement. A Positron emission tomography (PET) scan showed only increased activity in the liver. The IVLBCL seemed isolated to the liver only and R-CHOP chemotherapy was given along with prophylactic central nervous system (CNS) coverage with high-dose methotrexate. After six cycles of chemotherapy, the patient has normal bilirubin, normal lactate dehydrogenase (LDH) and no uptake on PET scan (Fig. ). He continues to have no evidence of active disease at follow-up of 9 months. |
The patient was a 67-year-old male who had inserted a foreign body into his rectum 7 hours before the hospitalization at the Emergency Room (ER). The patient had a second-degree hemorrhoid and was undergoing conservative treatment. He heard that a hemorrhoid could be improved if he used a massager to massage around the anal area. The patient massaged the perianal area with a massager to which differently shaped tips could be attached. The tip was unintentionally separated from the massager body inside the patient's rectum. The patient tried to remove the tip by himself, but came to the ER after feeling the foreign body move inside.\nHis past medical history showed a right hemicolectomy 2.5 years earlier because of ascending colon cancer. According to the pathologic results at that time, adjuvant chemotherapy was not performed because of the tumor was T2N0M0, stage Ib. He was going through a periodic follow-up and did not have any other significant medical history.\nThe patient did not have abdominal pain, but felt discomfort and minor pain around the perianal area. The patient's vital signs were stable, with a pulse rate of 70 per minute, a respiratory rate of 12 per minute, a blood pressure of 130/80 mmHg, and a temperature of 36.5℃. According to the physical examination, the abdomen was soft and flat, the bowel sound was normal, and no abdominal pain was being experienced. Upon digital rectal examination, the distal edge of the foreign body, which was located approximately 8 cm from the anal verge, was palpable. The foreign body was hard and had a smooth surface. There were no blood, no tar-colored rectum discharge, and no foul odor.\nOn a simple abdominal X-ray image, the foreign body was observed to be a radio-opaque object located in the pelvic cavity (). According to the laboratory test, the white blood cell count was 10,440/µL (neutrophils, 84%; normal range, 8,770/µL), which showed an increase in white blood cell count and neutrophil count. There were no other abnormalities on the blood test.\nSince there was a possibility of anal sphincter damage if the foreign body was removed at the ER, removal after anesthesia was decided upon. After spinal anesthesia, a digital rectal examination was done in the lithotomy position. Unlike the first digital rectal examination in the ER, the object moving a bit distally was located 5-6 cm from the anal verge. With careful palpation so as not to move the object inside, a hole could be felt in the center of the foreign body, and a Kelly forcep was used to hold the object and to remove it from the rectum (). The surgery was finished after using anoscopy to check for mucosal damage on the distal rectum and the anal canal.\nThe removed foreign body was a gray-colored hard-rubber-based object which was 7 cm long and 3 m in diameter (). After making sure that there were no anal bleeding and no abnormality on the physical examination of the abdomen for 24 hours, the patient was discharged. Currently, he is undergoing periodic follow up for colon cancer. |
Case 1. A 47-year-old male presented to the emergency department after falling backwards while trying to put his motorcycle in the rear of his pickup truck. He sustained an injury to his left hip and thigh. The patient was initially capable of weight bearing but as time progressed he experienced severe swelling and pain in the region that radiated to his groin. The swelling in the left thigh progressively worsened during the patient's time in the ED; however, no neurological or vascular compromise of the distal left lower extremity was noted. Past medical history was significant for bipolar 1 disorder and left foot fracture. Surgical history was positive for an inguinal hernia repair. The patient's current medications were quetiapine fumarate and NSAIDs (nonsteroidal anti-inflammatory drugs). Vitals and labs at presentation to the ED were essentially within normal limits. His hemoglobin was 12.7 and hematocrit was 36.6.\nImaging initially included a chest and pelvic X-ray which showed no gross abnormality. A CT angiogram of the left lower extremity was performed for increasing swelling which revealed a large soft tissue hematoma along the left hemipelvis and upper thigh with active extravasation likely emanating from a small branch of the left profunda femoris (focus of contrast blush) (Figures and ).\nThe patient was subsequently treated by endovascular means. The right common femoral artery access was used with a micropuncture technique under ultrasound guidance. A standard guidewire was then placed followed by a 5-French sheath. Following this, an Omni flush catheter and LLT guidewire were used to traverse the aortic bifurcation and position the catheter within the left external iliac artery. Digital angiography was performed and a site of extravasation was noted within a small peripheral branch of the profunda (Figures and ). The Omni flush was then exchanged for a 4-French straight catheter that was positioned within the proximal left profunda. Angiography was performed and, perplexingly, no active extravasation was noted. A Progreat microcatheter was then introduced in a coaxial fashion into the first perforating artery. Repeat angiography identified extravasation arising from a left distal profunda branch artery. The vessel proximal and distal to the area of active bleeding was then embolized using three 0.5 mm straight hilar microcoils. Repeat angiography demonstrated successful embolization and no further extravasation ().\nAll catheters were removed and hemostasis was secured. The patient was discharged home on oral analgesics on hospital day 3. |
We present the case of a 73-year-old female with a history of lung carcinoid tumor as well as squamous cell skin cancers who presented to our clinic for evaluation and management of a non-healing ulcer on the left lower extremity. Initial biopsy showed a squamous cell carcinoma in situ and she was treated with electrodessication and curettage. One year later, after having failed multiple chemotherapeutic and targeted agents for treatment of lung carcinoid tumor, she was started on sunitinib in combination with lanreotide and was doing well. Another year passed and the patient presented to our clinic with concerns for recurrence of the squamous cell carcinoma in situ of the left lower leg as she had again a non-healing ulceration. This ulcer was biopsied two times showing scar and pigmented macrophages. Wound culture grew Serratia marcescens and Corynebacterium. However, the skin failed to improve with conventional therapies including oral antibiotics including Bactrim and doxycycline, topical antibiotics including mupirocin and silver sulfadiazine, and regular alginate dressing changes. Sunitinib was stopped with the idea that this medication could have been preventing wound healing. The ulcer improved with almost complete resolution, and sunitinib was restarted. She presented again for follow-up examination at which time she had a recurrent 2.9 cm × 1.8 cm ulcer with beefy red edges and an overlying black eschar, draining serosanguinous fluid without gross purulence (). A third skin biopsy was then performed and showed epidermal hyperplasia, ulceration, and dense acute inflammation with neutrophils and crusting ( and ). Methenamine silver, Fite stain, acid-fast bacilli (AFB), and periodic acid Schiff (PAS) were negative. There was no malignancy identified. Imaging ruled out bone involvement. The patient was restarted on oral antibiotic doxycycline. Due to concerns for drug-induced PG as reported in the literature, sunitinib was stopped. The ulceration improved and she was taken off of antibiotics. She had almost complete resolution of the ulcer ().\nDrug-induced PG is a rare but serious adverse drug reaction that has been reported in the literature. Clinicians should have a high level of suspicion when caring for the skin of oncologic patients undergoing this therapy, as a change in their treatment plan can lead to resolution of this adverse cutaneous drug reaction. |
A 26-year-old male patient from Myagdi presented with pain abdomen for 8–9 days which was intermittently acute and spasmodic. Pain was progressive in nature and radiating towards the flanks. Pain was associated with fever at times. There was history of passage of loose stool mixed with blood every 10 min, which relieved the spasmodic pain. On inquiry, he revealed similar on and off incidences for the past 12 years . In the past he had undergone colonoscopy examination in other centers and was diagnosed as ulcerative colitis. He was on medical treatment for ulcerative colitis for the past 10 years which did not improve the condition. He had a history of weight loss of more than 15 kg in past 6 years.\nClinically he was pale with poor general condition. He had gross ascites and rectal prolapse. Other systems were within normal limits.\nAs a fresh investigation, repeat colonoscopy and biopsy were carried out. On colonoscopy examination the provisional diagnosis was ulcerative colitis (Fig. ) but the biopsy was reported as nonspecific colitis. No evidence of ulcerative colitis was seen in the biopsy. USG and CT scan of abdomen showed diffuse circumferential wall thickening in the rectosigmoid region of bowel and narrowing of lumen (Fig. ). Wall also showed multiple small ‘anechoic cystic spaces’ on USG. A provisional diagnosis of ulcerative colitis was suggested.\nConsidering the colonoscopy picture, radiological impression, condition of the patient and lack of response to medical treatment, subtotal colectomy was planned. Preoperative routine investigations showed Hb 6 gm/dl, platelet 76 000/ cu mm, with microcytic hypochromic anemia . All other results were within normal limit. Hartman procedure for subtotal colectomy was carried out (Fig. ). During surgery, massive blood transfusion (10 pints) was necessary.\nSubtotal colectomy specimen measuring 70 cm was received (Fig. ). Externally dilated and congested blood vessels were noted. Distal portion measuring upto 24 cm from distal resected margin showed marked edematous elevated brownish mucosa (Figs and ). Focal areas of mucosal ulceration were also noted. Cut section showed markedly thickened wall with several dilated spaces involving the whole wall thickness (Fig. ) . The cystic spaces measured upto 2 cm in maximum diameter.\nSection showed mucosal surface ulceration. Underlying wall showed several ectatic anastomosing vascular spaces lined by endothelial cells. Several spaces show congestion and some with thrombosis and recanalization (Fig. ). The vascular spaces were distributed from mucosa to subserosa (Fig. ). Surrounding stroma showed dense chronic inflammation and lymphoid aggregate. There was no evidence of cryptitis, crypt abscess, granuloma and atypia.\nHistopathologically diagnosis of Cavernous Vascular Malformation—Diffuse infiltrating (expansive) type was given.\nPatient developed jaundice, deranged liver function test and subsequently chest infection which aggravated due to the past long history of immunosuppressive therapy. He expired on 15th post-operative day. |
A 6-week old male Belgian Blue cross-breed calf was examined for respiratory distress at the clinic for ruminants of Utrecht University. The calf was reportedly born without complications but showed marked respiratory distress after delivery which improved only mildly in the first hours of life, although it never resolved entirely. Aside from the respiratory distress the calf was otherwise alert. During the first few weeks of life the animal maintained a good appetite and strong suckle reflex, but would tire quickly which meant that it had to interrupt meals repeatedly. Although the growth rate was decreased compared to other calves on the farm the main complaint from the owner was the ongoing labored breathing and rapid exhaustion after excitement.\nAt the time of presentation to Utrecht University the calf was bright, alert and responsive and in good body condition, although smaller than normal for an animal of its age and breed. The peripheral pulse was strong and equal, with a rate of 140 pulses per minute (reference range 80 - 110). The pulse rate matched the heart rate. Tachypnea (56 breaths per minute, reference range 30-50) and dyspnea were noted. No abnormalities were noted on auscultation of the lungs. Heart auscultation revealed a pansystolic murmur with punctum maximum over the tricuspid valves (V/VI) on the right side of the thorax, and punctum maximum over the pulmonic valves (IV/VI) on the left side of the thorax. A thrill was palpable over the heart that was of equal intensity on the left and right side of the chest. The jugular veins were not distended nor was a jugular pulse present. The mucous membranes were cyanotic. A thorough neurologic exam of the patient using established guidelines [] did not show any abnormalities other then strong exercise intolerance.\nHematology revealed a PCV of 35% (reference range, 21-37%) and a hemoglobin concentration of 11.9 g/dL (reference range, 8.0 - 15.0 g/dL). Leukogram results were within normal limits. Arterial blood was obtained from an ear artery for blood gas analysis, revealing normocapnia (paCO2 = 42 mm Hg, reference range 35 - 44 mm Hg) but marked hypoxemia with an oxygen saturation of 41.1% (reference range > 97%) and a paO2 of 23 mm Hg (reference range >79 mm Hg). Similar results were obtained from arterial blood collected from the femoral artery. A blood sample obtained from the jugular vein at the same time showed mild hypercapnia and a compensated respiratory acidosis with a pH of 7.44 (reference range, 7.35 - 7.45) and a pCO2 of 53 mm Hg (reference range 35 - 44 mm Hg). The oxygen saturation and pO2 in venous blood were 30.6% and 19 mm Hg (reference range 30-40 mm Hg) respectively. Blood lactate concentrations were measured repeatedly at different times in arterial and venous blood and ranged between 0.87 and 1.22 mmol/L (reference range: 0.55-2.22 mmol/L) with venous blood lactate concentrations ranging between 0.1 and 0.3 mmol/L above arterial blood lactate concentrations.\nAlthough the clinical examination and arterial blood gas analysis clearly indicated the presence of a cyanotic heart defect, a Tetralogy of Fallot or an Eisenmenger's complex were considered unlikely differentials as hypoxemia encountered in patients with these conditions are much milder than determined in this animal [-].\nEchocardiographic examination revealed an enlargement of the right ventricle, flattening of the interventricular septum and the presence of a VSD (Figure and ). The VSD was subvalvular and approximately 1.5-2 cm in diameter. Also, an abnormal position of the left and right ventricular outflow tract was detected, both being slightly off-center. At the level of the heart base, short axis views showed the centrally located artery originating from the right ventricle. At this level the artery originating from the left ventricle could be followed to its bifurcation into two arteries (Figure ). The diameter of this artery increased a few centimeters after its origin from the left ventricle, resembling a post-stenotic dilatation, and decreased again before its bifurcation (Figure ). Based on these findings the presence of a functional left outflow tract obstruction was considered.\nColor and spectral Doppler revealed bi-directional, turbulent flow through the VSD during systole. Small tricuspid and mitral valvular insufficiencies were also detected. No turbulent flow was detected in the arteries originating from either ventricle. No abnormal flow was detected between the atria. The echocardiograhic examination therefore suggested a complete transposition of the aorta and pulmonary artery. Furthermore a subvalvular VSD with bidirectional blood flow and mild bilateral atrioventricular valve insufficiencies were diagnosed. The presence of a stenosis of the pulmonary artery was considered but could not be confirmed.\nElectrocardiographic examination was conducted using a standard bipolar monitor lead (base-apex) revealing a sinus tachycardia as the sole anomaly.\nSeveral weeks after admission, cardiac catheterization using a Swan-Ganz catheter was performed in order to obtain blood from the cranial vena cava, right atrium and right ventricle for blood gas analysis. proper location of the catheter tip was determined based on typical pressure wave tracings for the atrium, ventricle and great artery. Jugular venous blood had a pO2 of 15 mm Hg (reference range 35 - 44 mm Hg), an oxygen saturation of 19.8% and a pCO2 of 44 mm Hg (reference range 35 - 44 mm Hg). In the right atrium pO2 was 16 mm Hg with an oxygen saturation of 21.2%. As the catheter was advanced into the right ventricle a sudden increase of the pO2 to 21 mm Hg and of the oxygen saturation to 36.6% was observed.\nAfter completely inserting the catheter (90 cm) into the jugular vein a characteristic arterial pressure curve tracing indicating that the tip of the catheter was located in the great artery exiting the right ventricle was recorded. A wedge pressure tracing identifying the position of the catheter tip in a pulmonary artery branch could not be obtained. Blood sampled from the artery exiting the right ventricle had a pO2 of 19 mmHg and an oxygen saturation of 35.2%. Blood collected from the ear artery at the time of cardiac catheterization yielded a pO2 of 23 mmHg and an oxygen saturation of 38.9%. These results suggested that arterialization of the blood of the systemic circulation occurred in the right ventricle and that blood from the right ventricle was unlikely to pass through the pulmonary circulation as the oxygen saturation of blood in the right ventricle and arterial blood were similar.\nAt the time of catheterization the PCV was 48% (reference range 21 - 37%) and hemoglobin concentration was 16.5 g/dL (reference range 8.0 to 15.0 g/dL) revealing a marked increase when compared to values measured at admission.\nThe calf remained in the clinic until 19 weeks of age when it was humanely euthanized due to a sudden worsening of exercise intolerance and cyanosis over a period of a few days. At the time of euthanasia the calf weighed 94 kg. A normally developed 4-month old Belgian Blue bull calf would be expected to weigh between 170 and 210 kg. A PCV of 65% (reference range 21 - 37%) and a total protein concentration of 6.4 g/dL (reference range 5.9 - 7.5 g/dL) were determined at this time.\nOn post mortem examination the calf was found to be in moderate body condition and showed marked cyanosis of all mucous membranes. Complete transposition of both great vessels was noted, with the aorta exiting from the right ventricle and the pulmonary trunk exiting from the left ventricle (Figures and ). The width of the aorta at its base was 3.5 cm, and it expanded to 4 cm at its widest point. Coronary arteries were clearly visible arising from around the base of the aorta (Figure ). The pulmonary artery outflow was 2.5 cm which quickly dilated to 6.5 cm before splitting into the left and right pulmonary branches (Figures and ). The presence of a morphologic left outflow tract obstruction could not be confirmed on post mortem examination. There was a subvalvular VSD which measured 1.2 × 1.3 cm and the right ventricular wall was markedly hypertrophied with a 1:1 ratio between the left and right free ventricular walls (Figure ). The ductus arteriosus and foramen ovale were both closed. All other organs were unremarkable. Histology of lung tissue revealed a mild multifocal lymphocytic perivasculitis and moderate multifocal lymphocytic interstitial pneumonia. Lung tissue did not show pulmonary edema, thickening of the wall of pulmonary vessels or any other signs of chronic pulmonary hypertension. Histology of brain tissue did not reveal any abnormalities.\nComplete transposition of the great arteries has repeatedly been reported in cattle and is thought to be the result of an abnormal truncus arteriosus development during cardiogenesis with possible genetic or teratogenic etiology [,,]. The incidence is over 95% higher among males than females []. Since this malformation causes the systemic and pulmonary circulation to run in parallel it is compatible with life only in combination with at least one more cardiac or extracardial congenital abnormality allowing blood to shunt bidirectionally between the two otherwise completely separated circuits. A left-to-right shunt is required to allow oxygenated blood to enter the systemic blood circuit, whereas a concomitant and volumetrically identical right-to-left shunt is needed to maintain an adequate blood volume in the pulmonary circuit [].\nIn the case presented here a VSD was identified as sole connection between pulmonary and systemic circuit. In human patients with TGA a VSD is the second most common concomitant cardiac malformation after a persistent ductus arteriosus with an incidence of approximately 45% []. Color and spectral Doppler echocardiography conducted in the patient presented here confirmed the bidirectional turbulent blood flow through the VSD, which obviously allowed sufficient amounts of oxygenated blood to enter the systemic circulation to assure survival, while at the same time maintaining the volume in the pulmonary circulation. A turbulent rather than a unidirectional flow during the systole is suggestive of equal systolic pressures in left and right ventricle. Similar pressure in systemic and pulmonary circulation is commonly observed in human patients with TGA and a large VSD through which pressure at or near the systemic level is transmitted to the pulmonary circuit [,].\nTricuspid regurgitation as diagnosed in our patient is also commonly observed in human patients with TGA. Although atrioventricular regurgitation could be caused by a congenital valve abnormality, in most human cases it is considered to be secondary to annular dilatation from right ventricle dysfunction []. In the calf presented here all cardiac valves were found to be morphologically normal on post mortem examination, whereas the enlargement of the right ventricle diagnosed echocardiographically would support the hypothesis of ventricular dysfunction and ensuing annular dilatation.\nThe criterion established in the literature to confirm the diagnosis of TGA is the visualization of the aorta arising from the right ventricle and the pulmonary artery from the left ventricle, either by angiocardiography or echocardiography, as done in the present study [,]. Cardiac catheterization in combination with arterial blood gas analysis also provided strong evidence to support the diagnosis of TGA in the case reported here. Whereas the sudden rise of oxygen tension and oxygen saturation in the right ventricle confirmed the presence of a VSD with functional left-to-right shunt, the similar oxygen tension and oxygen saturation in the right ventricle and the ear artery indicated that arterial blood originated from the right ventricle and arterialization solely occurred through pulmonary venous blood shunting from the left to the right ventricle. The inability to obtain a wedge pressure tracing that is normally obtained as the tip of the Swan-Ganz catheter with inflated balloon is advanced into a pulmonary artery branch via the right ventricle further indicated that the catheter was not located in the pulmonary artery.\nIn the present case extremely low arterial oxygen saturation and paO2 values were measured, indicating marked hypoxemia. In humans with acute episodes of hypoxemic hypoxia, as might occur during decompression or at high altitude, an arterial pO2 between 40 and 30 mmHg is associated with loss of judgment, euphoria and obtundation whereas values below 25 mmHg, as measured in this patient are associated with coma []. However in this calf neurologic deficits such as ataxia, or abnormal reflexes potentially indicating the presence of diffuse cerebral hypoxia were not observed. Histology also showed no abnormalities. Furthermore neither elevated plasma lactate concentrations suggestive of marked hypoxia and ensuing enhanced anaerobic glycolysis were found These findings indicate that neurological tissues can adapt in the presence of congenital chronic severe anoxia.\nIn an animal with congenital TGA it might be expected that at the time of parturition, and the cessation of perfusion by the umbilical vessels, the animal would suffer from acute hypoxia. However, results from studies looking at oxygen saturation in near-term and neonatal calves suggest differently. For instance, Comline and Sliver [] reported that, in late-term bovine fetuses, the pO2 of the umbilical vein was around 37.6 ± 1.5 mmHg, while Bleul et al. [] reported that, at the time of birth, the paO2 and oxygen saturation values in the arterial blood of calves were 45.3 ± 16.0 mmHg and 64.2% respectively.\nConsidering that at the time of first examination this patient had a paO2 of 23 mmHg and arterial oxygen saturation of 41% it is probable that the change in blood oxygenation at the time of interruption of the blood flow through the umbilical vessels was rather small, explaining the absence of clinical signs commonly observed in patients with acute hypoxemic hypoxia.\nThe calf in this report appeared to be stable during the first weeks of life, but respiratory distress, exercise intolerance and cyanosis worsened markedly in the last days before euthanasia. However, it did remain bright and alert with a good appetite, without showing any neurologic deficits. The PCV rose from 35% at the time of admission to 48% and later to 65%, while total protein concentration remained constant. This finding is consistent with excessive erythrocytosis observed in patients with chronic hypoxemia as occurs during high altitude disease. In the present case chronic hypoxemia obviously triggered marked erythrocytosis without improving blood oxygenation. The increased amount of unsaturated hemoglobin in blood not only exacerbates cyanosis but also increases blood volume and blood viscosity thereby affecting pulmonary blood flow and thus the pulmonary ventilation-perfusion relationship []. This vicious circle is likely to have contributed considerably to the demise of the patient.\nIn human patients with TGA systemic ventricular dysfunction is a common sequel with advancing age [] and the most common cause of death in untreated children older than one week []. Despite the inherent vulnerability of the morphologic right ventricle to failure, severe hypoxemia as well atrioventricular regurgitation considerably increases the risk of congestive heart failure in these patients.\nIn cases of TGA combined with a large VSD pulmonary hypertension is another common complication resulting from continuous exposure of the pulmonary circulation to the systemic blood pressure and is likely to contribute to the demise of untreated children having survived the neonatal period. Presence of a pulmonary outflow tract obstruction decreases the risk of pulmonary vascular disease by protecting the pulmonary capillary bed from excessive pressure []. Although the presence of a pulmonary outflow tract obstruction could not be ruled out with certitude, lung histology did not show any tissue damage consistent with persistent pulmonary hypertension. It is difficult to assess how much of an affect the interstitial pneumonia seen histologically will have affected this patient clinically. |
A 55-year-old female referred to our hospital with a 10 month history of repeated episodes of headache associated with vomiting that had worsened over the past 1 week, followed by one episode of generalized tonic clonic convulsions. There was fever on and off and there was no visual complaints or limb weakness. On examination, she was conscious and cooperative. Cranial nerves examination revealed left eye nystagmus, normal visual field and acuity. There was no sensory or motor deficit. Fundoscopy revealed bilateral papilloedema. Magnetic resonance imaging (MRI) carried out elsewhere reported as partly thrombosed giant right internal carotid artery aneurysm. She was referred for computed tomography angiogram of the cerebral vessels.\nPlain CT brain revealed a midline, well defined round and heterogeneous mass in the region of foramen of Monro showing patchy areas of peripheral hyperdensity with the mean Hounsfield Unit 70 []. There was obstructive hydrocephalus due to pressure effect on foramen of Monro, accompanied by dilatation of the bitemporal horns of lateral ventricles. Posterior fossa and fourth ventricle were unremarkable. The lesion showed minimal enhancement and anterior displacement of anterior cerebral artery (ACA) and its callososeptal branches without encasement [].\nReview of the MRI Brain revealed a large well-defined lobulated cystic mass lesion in midline in the region of foramen of Monro with extension in to the frontal lobe. The mass was predominantly hyperintense on T1 [], heterointense on T2 weighted images and was not suppressed in Fluid attenuated inversion recovery (FLAIR) []. The lesion revealed nodular rim enhancement on administration of gadolinium. The third ventricle appears to be displaced inferiorly and fourth ventricle appears normal. The major part of the lesion showed restricted diffusion with low-apparent diffusion co-efficient (ADC) values [], and peripheral blooming in gradient []. The flow voids of bilateral internal carotid arteries were normal. Magnetic resonance (MR) angiogram was normal and hence the possibility of thrombosed aneurysm was ruled out. MR spectroscopy revealed prominent peaks centered at 1-1.5 ppm, which probably corresponded to lipid/cholesterol peaks []. |
Our patient was a 48-year-old lady who was a local politician and who was carrying out her duties satisfactorily for the past several years. About a year and a half prior to her presenting to us, she developed symptoms characterized by low mood, anhedonia, easy fatigability, decreased concentration, hopelessness, and decreased sleep and appetite. These symptoms developed following a minor stressor at work and were clearly disproportionate to the stress. The symptoms had developed abruptly and the patient had never experienced such symptoms earlier. She had been an emotionally strong and confident lady earlier and was herself surprised at her current condition. She also developed prominent suicidal ideations and tried to kill herself by setting herself on fire, but was incidentally found and rescued by her maid. These symptoms continued for 4-5 days and were spontaneously remitted. Similar episodes occurred on almost a monthly basis for the next 18 months, each lasting 3-5 days. The interval varied between 15 and 40 days. They were unrelated to her menstrual cycles. There was no history of mania or psychosis. In the inter-episodic period, the patient was fully functional and carried out all her duties satisfactorily. Initially, the patient did not realize that it was a psychiatric illness, but after several such episodes, she consulted a private psychiatrist who started her on escitalopram (10 mg). It was continued for 4 months with no improvement. She changed psychiatrists and was tried on mirtazapine (15 mg) irregularly for 3 months without improvement. She did not follow-up with one psychiatrist and therefore full doses were not tried. She eventually presented to us during an episode and was off all psychotropics at that time. We made a diagnosis of RBDD as per ICD 10 and started her on paroxetine (10 mg). She scored 24 on Hamilton depression rating scale[] at the time of presentation. Although her symptoms resolved in about 4 days time, the dose was gradually increased to 25 mg/day, but, in the following month, she again had an episode lasting for about 4 days. She scored 20 on Hamilton depression rating scale this time and had somewhat less frequent suicidal ideation. Paroxetine dose was further increased to 50 mg. About 25 days later, the patient again developed depressive symptoms in the form of mild fatigue and some decrease in self-confidence, which lasted for 3-5 days. She now scored only 7 on Hamilton depression rating scale during the episode. There were no ideas of hopelessness, helplessness, worthlessness, or suicidal ideation. However, the lack of confidence still prevented her from carrying out her duties in those 3-5 days, although otherwise she was symptomatically much better. However, with one session of supportive counseling, she managed to get back to work as well. In the subsequent month, although she complained of mild fatigue, there were no other symptoms and she started doing her duties satisfactorily. The patient was continued on the same dose and is on regular follow-up. Thereafter, for the subsequent 9 months, she maintained the full symptomatic improvement. |
A 35-year-old female of Indian origin presented with complaint of depression over left parietal area noticed accidentally about four years back. At that time the size is about a coin. It is slowly and gradually progressive in nature. At present it is about 4 × 4 cm in size. There were no other symptoms at this time duration. There was no history of any trauma. Her neurological examination was normal. Local examination shows a well-defined nontender rounded left parietal depression of 4 × 4 cm in size without any soft tissue mass with normal overlying scalp. Plain x-ray skull was showing a well-demarcated rounded lytic area in parietal region with normal margin. (Figure ) There is no other abnormality seen in plain x-ray. CT scan revealed a well-defined skull defect with complete disappearance of the central bone matrix and no evidence of new bone formation (Figure ). The soft tissue widows showed no associated soft tissue mass in or around the calvarial defect. MRI brain showing calvarial defect to be filled with thin soft tissues that were hypointense on both T1-weighted and T2-weighted (Figure &). Tc-99 m MDP bone scintigraphy reveals no bone uptake in the resorbed site as well as around the margin. Whole-body bone scan was undertaken, which revealed no similar bony lesion any where in the body. (Figure ). The preoperative differential diagnosis included Paget disease at destructive stage, eosinophilic granuloma, Brown tumor, and osteolytic metastasis but none of them correlated well with the clinical and radiologic features.\nA left parietal craniectomy was performed, a bony defect of 4 × 4 cm with a very thin remainder of the inner table was found. There is involvement of overlying galea and subgaleal tissue in form of thickening and increased vascularity was found. (Figure ) The inner table was removed and bone specimens taken from the edges. (Figure ) Dura was normal in texture colour and vascularity.(Figure ) Cranioplasty done with bone cement. (CMW-1 Johnson's and Johnson's) (Figure ). Histopathological examination of bone pieces showed intraosseous angiomatosis with mixed patterns of bone destruction, fibrous connective tissue replacement by highly vascularised collagenous tissue, and abnormal small vessel proliferation (Figure ). The combination of clinical, imaging, and pathologic findings strongly suggested the diagnosis as Gorham disease. The patient had an uneventful postoperative course and was discharged on 7th postoperative day. |
A 43-year-old woman underwent urgent laparotomy for spontaneous massive rupture of a subcapsular liver hematoma associated with HELLP syndrome.\nThe right lobe of the liver was entirely replaced by a large hematoma and areas of necrosis, and there were deep ruptures in both lobes. After establishing that neither conservative surgical treatments nor partial resections would have been effective; a total hepatectomy was performed to control the hemorrhage.\nAfter isolating the hilar structures, the bile duct and hepatic artery were ligated and divided. The portal vein (PV) was clamped and divided at the bifurcation level. After division of all ligamentous attachments, the liver was progressively mobilized to the left until the retrohepatic vena cava was completely exposed. All the accessory hepatic veins encountered were ligated and divided, and the right hepatic vein was clamped, divided, and oversewn. Rotating the liver towards the right, the left and middle hepatic veins were isolated for 3-4 cm within the hepatic parenchyma and divided permitting the removal of the liver. After suturing the stump of the left hepatic vein, it was noted that the orifice of the stump of the middle hepatic vein matched the orifice of the portal vein in length and diameter. A temporary end-to-end porto-middle hepatic vein anastomosis was created using a running suture (Prolene 5/0; Ethicon, Somerville, NJ), instead of the standard TPCS ().\nThe patient was taken back to the ICU for hemodynamic and metabolic monitoring and management. Twelve hours after placing the patient on the liver transplant waiting list with a Status 1 designation, a liver became available.\nThe patient returned to the operating room for a LT which was performed in a piggyback fashion. The recipient IVC was cross-clamped tangentially, occluding approximately half of its lumen, but leaving the TPMHV open and untouched due to its position above the caval clamp. After a longitudinal incision of the recipient IVC anterior wall, the graft was placed orthotopically and rotated to the right, and an end-to-side anastomosis between donor and recipient IVC was performed ().\nNext, the TPMHV was taken down, and an end-to-end PV anastomosis was completed. After removing the IVC and portal vein clamps, the liver perfused well, and the patient remained hemodynamically stable.\nArterial and biliary anastomoses were constructed utilizing a standard technique.\nThe patient was discharged 47 days after the liver transplant with normal hepatic, renal kidney, and respiratory functions. She remains in healthy condition after ten-month followup. |
A 21-year-old male patient reported to the Department of Oral and Maxillofacial Surgery with the chief complaint of swelling on the lower lip of 10 days' duration. The patient gave a history of an increase in swelling in the morning and before meals and the swelling usually subsided by evening. On general physical examination the patient was well built and nourished with no relevant past medical history. None of the family members of the patient had reported a similar lesion.\nOn intraoral examination, a fluctuant swelling measuring approximately 1 × 1 cm in diameter was seen on the lower lip. On palpation, the swelling was nontender, mobile, and soft in consistency. Based on the clinical presentation, a provisional diagnosis of mucocele was rendered. The lesion was surgically excised under local anesthesia and sent for histopathological examination as three soft tissues, white to brown in colour, smooth to rough surface, fluctuant to firm in consistency, and collectively measuring approximately 1.4 × 1.2 × 0.4 cms ().\nMicroscopic examination in a low power revealed the presence of numerous ectatic, variable-sized ducts interspersed in a fibrocellular connective tissue stroma (). On higher magnification, the ductal/cystic spaces showed mucin pooling with abundant muciphages (). Associated mucous minor salivary glands showed acinar atrophy with squamous metaplasia of the excretory duct. Areas of hemorrhage were also seen overlying the lesional tissue.\nConsidering the location and the benign clinical appearance of the lesion, the differential diagnosis was narrowed down to four possible lesions: namely, mucocele, lipoma, sclerosing polycystic adenosis, and polycystic dysgenetic disease in order of their frequency of occurrence. These can be easily distinguished histopathologically. Mucocele is unicystic in nature with watery spinnbarkeit nature of material in aspirate, lipoma presents as an encapsulated mass of mature adipocytes, and sclerosing polycystic adenosis exhibits fibrosis and hyperplastic ductal and acinar epithelial elements. Thus, this classical histopathological picture aided in diagnosing the case as a “polycystic dysgenetic disease of minor salivary glands.”\nThe patient underwent assessment of all glandular tissues in the whole body including other salivary glands, liver, kidney, and pancreas and no significant findings were elicited. The lesion in the lower lip healed uneventfully and a review at 18 months showed no recurrence. |
This patient is a 65-year-old male with a history of chronic postural headache for 13 years. The headaches started after sustaining a ground level fall in 1997 shortly after having a L4-L5 laminectomy in 1997 for spinal stenosis. He was eventually seen by a specialist in low pressure headaches and was subsequently diagnosed with spontaneous intracranial hypotension. Computed tomographic melography (CTM) demonstrated a likely CSF leak at L4-L5. The headaches were initially managed conservatively with bed rest, caffeine, increase oral intake, intravenous fluid, and an abdominal binder. These measures provided only minimal temporary relief. Multiple interlaminar epidural blood patches were performed but none of them were effective. The patient also underwent C6–C8 rhizotomy as well as multiple C2-C3 epidural steroid injections. Discouraged and not wanting to consider surgical intervention the patient decided to simply try and cope with the pain. He continued to use acetaminophen, ibuprofen, and oxycodone 40 mg q12 hrs but continued to have daily headaches. Unable to tolerate the headaches any longer the patient once again sought medical intervention in 2010. At that time he was referred to the current authors for evaluation and potential nonsurgical intervention.\nAt the time of consultation the patient complained of daily dull, achy frontal headache with some radiation to the neck that was significant worse when sitting or standing and resolved when lying supine. His pain was reported to be 9/10 with verbal numeric rating scale (VNRS). The headaches are frequently associated with recent nausea, vomiting, and photophobia. On physical exam he was found to be afebrile, normotensive, and with no gross neurological deficits. Heavily T2-weighted magnetic resonance myelography (MRM) was performed which showed a CSF collection in the posterior epidural space at the level of L5 presumably representing the site of CSF leak. MRM was chosen to help located the exact site of CSF leak because addition lumbar puncture for intrathecal contrast for a CTM could exacerbate the patient's symptoms []. The case was discussed with the patient's neurosurgeon and the decision was made to attempt an EBP by entering the bilateral intervertebral foramen. The potential risks and benefits were explained to the patient in great detail. |
The case was a 66-year-old man with unremarkable family and previous disease histories. He complained of dysuria in January 2015. Pelvic magnetic resonance imaging suggested a malignant mass in the prostate, and he underwent transurethral resection of the prostate in February 2015. The pathological results showed acinar adenocarcinoma of the prostate with a Gleason score of 3 + 3. In subsequent years, radiological examination showed no recurrence and his prostate-specific antigen level was normal.\nThe patient complained of lower abdominal pain in January 2017. A mass was found in his sigmoid colon by enteroscopy examination. Positron emission tomography/computed tomography examination showed a mass in the sigmoid colon with local lymph nodes invasion and a mass in the right upper lobe of the lung. In March 2017, the patients underwent biopsy of the 2 masses. Biopsy of the colon showed moderately differentiated adenocarcinoma with Kirsten rat sarcoma viral oncogene homolog exon 2 mutation, and biopsy of the lung showed moderately differentiated adenocarcinoma with epidermal rowth factor receptor exon 21 mutation. In April 2017, the patient underwent surgical resection of the right upper lobe of the lung, and postoperative staging showed T1bN0M0 (stage IA). Since the colon cancer presented as unresectable stage III, from May 2017, we gave him 8 cycles of chemotherapy with modified FOLFOX6 regimen (oxaliplatin 85 mg/m2, leucovorin 400 mg/m2, 5-fluorouracil 400 mg/m2 on day 1, followed by 5-fluorouracil 2400 mg/m2 intravenous infusion over 46 hours every 2 weeks) to convert it to resectable colon cancer. After 8 cycles of chemotherapy, the patient underwent radical resection of colon cancer in September 2017. Figure shows all the pathological tissues. The patient finished the remaining 4 cycles of modified FOLFOX6 regimen chemotherapy in November 2017. Now the patient takes examination every 3 months, and the results show no recurrence.\nTo find out whether genetic factors were involved in carcinogenesis, the patient's plasma was examined using next-generation sequencing (NGS), which showed DNA methyltransferase 3A (DNMT3A) and telomerase reverse transcriptase (TERT) mutations. |
A 45-year-old male patient visited our clinic in May 2005 due to detachment of a porcelain-fused-to-metal crown on the left maxillary central incisor, which had been connected at another clinic in 2000.\nThere were no special findings in his physical and extraoral conditions. On intraoral examination, mesiodistal fracture lines were found on the remaining tooth structure. A swelling and slight redness were observed on the peripheral soft tissue, but there were no acute symptoms such as purulence or pain [Figure and ].\nRadiographic examination showed no inflammatory symptoms, such as an increased periapical space, bone resorption, or periapical radiolucency, thus, the inflammation was judged confined almost exclusively in the gingival tissue. A nasopalatine foramen was found adjacent to the apex of the maxillary left central incisor [].\nThe maxillary left central incisor had a root fracture and required extraction. After a careful consultation about various treatment options, the patient desired implant treatment. Because there existed no obvious signs of acute inflammation or any other symptoms of the soft and hard tissue, postextraction immediate implant placement was planned. Prior to the implant treatment, the patient was subjected to meticulous scaling of all the remaining teeth and oral hygiene instruction session.\nIn May 2005, the maxillary left central incisor was extracted. After careful curettage of the extraction site, the mucoperiosteal flap was reflected. There was no resorption of the labial bone and an implant (TiOblast, Astra Tech, Mölndal, Sweden, ø 4.5 mm, length 13 mm) was placed into the fresh extraction socket with good primary stability. The osteotomy was made so that the implants would be located centrally in relation to the socket; however, careful drilling was conducted in order to preserve the buccal wall. The gap between the fixture and the socket wall was filled with autogenous bone particles collected during the surgery. The wound was closed after a releasing incision. On the periapical radiograph taken immediately after implant surgery, the implant appeared to interfere with the nasopalatine canal [].\nThe implant was restored with the definitive crown in November 2005. No abnormality was found during quarterly maintenance visits after the implant surgery, however, at the maintenance visit in November 2007, the patient complained about swelling on the palate. Clinical examination detected pulsation on the palate and an oval-shaped radiolucency having unclear boundary around the apex of the dental implant detected on the periapical radiograph. A computed tomography (CT) scan confirmed the radiolucency of a size of 10 mm × 10 mm × 9 mm, which surrounded the apical portion of the implant, reaching to the nasopalatine canal [Figure –]. Although the patient had no spontaneous pain, the lesion seemed to be enlarging and it was judged that early removal of the cyst was necessary. Because the implant was immobile, with no signs of peri-implant bone resorption, it was decided not to remove the entire implant but to resect the apical portion of the implant lodged inside the cyst, and remove the entire lesion. The surgery was performed in November 2007. Flap incision was designed to be away from the expected lesion area so that the incision will not overlap with the bone defect after the cyst removal. Mucogingival flap was elevated and resorption of the labial bone was observed. After removing the thinned cortical bone and detaching the cyst wall on the palatal side, the apical portion of the implant (approximately 3 mm) lodged inside the cyst was resected using a carbide bur. Thereafter, the entire cyst, including the resected implant, was removed en bloc. It was evident that the nasopalatine neurovascular bundle was incorporated into the cyst []. The wound was closed by repositioning the mucoperiosteal flap, and no bone grafting materials were used to fill the defect.\nThe histopathological findings showed that the wall of the cystic lesion comprised of cuboidal, ciliated columnar and stratified squamous epithelium with underlying connective tissue. The cysts that form adjacent to oral mucosa of the incisal foramen was lined mainly by stratified squamous epithelium, whereas the lining close to the incisal foramen was lined by cuboidal and ciliated columnar epithelium, and there were no signs of inflammatory cell localization within the observed biopsy []. Thus, based on the clinical and histological findings, the lesion was diagnosed as nasopalatine duct cyst developed in association with the dental implant placement.\nAfter the cyst removal, the patient has been carefully followed by clinical, radiographic examinations (both periapical and CT) during maintenance visits scheduled every 4 months. The current status after 28 months after the cyst removal shows no signs of implant mobility, marginal gingival recession, or peri-implant inflammation. Compared with the radiographic findings before the cyst removal, the radio-opacity of the original lesional area seemed to have increased. The bone defect measured on the CT after the cyst removal is 4 mm × 3 mm × 4 mm, comparably smaller to the preoperative radiograph, suggesting a favorable healing []. |
In January 2012, a 32-year-old lady was referred to our department for evaluation of a resistant prolactinoma. She presented to a peripheral hospital initially in February 2010 with a history of galactorrhoea and menstrual irregularity of 6 months duration. She complained of occasional episodic headache, not associated with vomiting, seizures, altered sensorium, and visual field defects. There was no history to suggest thyroid dysfunction, systemic or psychiatric ailment, and drug intake. She denied features to suggest a disturbance of any other hormonal axes or similar history in family members. Her initial evaluation revealed normal vital parameters with no evidence of goiter. Systemic examination was normal except for galactorrhoea. Her neurological examination was normal including visual fields and fundus examination. Hormonal profile revealed hyperprolactinemia (serum prolactin 289 ng/ml) with normal thyroid, adrenal, gonadal, and growth axes evaluation. Magnetic resonance imaging (MRI) sella revealed multiple, small, focal, well-defined, abnormal areas of signal intensity in the anterior pituitary on both sides []. Lesions appear homogeneously hypointense on T2 weighted images with minimal enhancement after contrast. Dynamic mean curve analysis shows slow uptake of contrast with low peak by the lesions suggesting a diagnosis of multiple pituitary microadenomas. There was no pressure effect on surrounding structures. The patient was treated with cabergoline 0.5 mg biweekly and continued for another 6 months. She had regular menstrual cycles and galactorrhoea subsided after 2 months of therapy.\nIn July 2010, the patient had conceived leading to withdrawal of cabergoline therapy. The patient did not have any features of raised intracranial tension throughout pregnancy and neuroimaging was not repeated for monitoring. She had an uneventful pregnancy and delivered a healthy baby at term. Nine months after delivery she presented with headache and occasional vomiting. Serum prolactin was elevated (158 ng/ml) and the patient underwent repeat neuroimaging. On dynamic and contrast enhanced MRI, pituitary gland appears heterogeneously bulky macroadenoma of 12 × 14 × 15 mm predominantly involving the right half of the anterior pituitary []. The lesion had a mixed pattern of contrast uptake and had no pressure effects on the surrounding tissue. The repeat hormonal panel revealed the normal function of anterior and posterior pituitary. Visual field examination by perimetry was normal and other hematological/biochemical parameters were normal including lipid profile. She was diagnosed to be a case of macroprolactinoma and was treated with cabergoline 0.5 mg weekly. The prolactin level normalized after 3 months with no recurrence of galactorrhea. However, repeat neuroimaging after 6 months did not show any resolution in the size of the pituitary macroadenoma. She continued to receive cabergoline and had no clinical features of hyperprolactinemia or pressure symptoms. |
A 50-year-old Caucasian female, with no previous smoking history or pancreatic cancer family history, presented to our clinic for surgical evaluation of a biopsy-proven PDA noted at the junction of the body and tail of the pancreas. The patient had previously undergone a classic pancreaticoduodenectomy (Whipple) procedure with concurrent hemicolectomy 6 years prior (in 2011) for a T3N0M0 adenocarcinoma of the pancreatic head that had invaded the mesentery of the proximal transverse colon. After the 2011 surgery, pathology revealed a poorly differentiated PDA along with a PanIN grade 2. The final pathology showed negative surgical margins, positive perineural spread, and 0/33 specimen lymph node involvement. From June 2012 to October 2012, the patient underwent and completed adjuvant chemotherapy with gemcitabine, capecitabine, and radiation at an outside hospital. She was carefully followed by her medical oncology team with serial CA 19-9 monitoring and abdominal MRIs on an ongoing basis. Due to her young age, in 2011, she underwent genetic screening and no germline mutations were identified. Since the time of the primary resection, the patient had been high functioning and healthy, with the exception of some problems of early satiety and recurrent cholangitis. These sequelae were attributed to close proximity of the gastrojejunostomy and hepaticojejunostomy, with possible reflux of intestinal contents up the afferent limb, all partially managed by diet changes.\nIn 2017, a biannual screening MRI with intravenous contrast showed a new pancreatic lesion measuring 2.3 × 2.2 cm in the tail of the pancreas (). Esophagogastroduodenoscopy and endoscopic ultrasound-guided biopsy identified it as a poorly differentiated adenocarcinoma. From 2011 to 2017, she had had close followup with serial CA 19-9, and a measurement of this marker after identification of the lesion on MRI showed an elevation, which was confirmed on repeat testing (61 and 55 U/mL; normal <37 U/mL). This was the first instance of two consecutive CA 19-9 measurements outside of the normal range since resection of the primary cancer 6 years prior. When the patient presented to our institution a few months later, the CA 19-9 had returned to normal at 32 U/mL (), and there was a moderate increase in CEA (16.9 ng/mL; normal <3 ng/mL). The patient received a second MRI of the abdomen and pelvis with contrast to identify distant disease, which showed the lesion to be confined to the pancreas. Along with the MRI of the abdomen, a CT of the chest was preformed, which showed no gross metastatic lesions. A completion pancreatectomy was scheduled with revision and lengthening of the jejunal limb proximal to the gastrojejunostomy to resect the tumor and treat her episodes of early satiety and recurrent cholangitis.\nIntraoperatively, the patient's three Whipple anastomoses were noted to be grossly intact and there was no evidence of metastatic disease. First, the stomach was divided approximately two centimeters proximal to the prior gastrojejunostomy. The jejunostomy was closed, and gastrocolic and gastrosplenic ligaments were divided. The splenic artery was ligated, and the splenocolic ligament was divided. The spleen and pancreas were mobilized out of the retroperitoneum. The jejunum was divided between the pancreaticojejunostomy (PJ) and hepaticojejunostomy. The proximal jejunum, prior PJ, remaining pancreas, and spleen were removed. The tumor was noted to be grossly confined to the pancreas. The distal end of the stomach was delivered through the mesocolon and a retrocolic gastrojejunostomy was undertaken 60 cm downstream from the hepaticojejunostomy. The anatomy before and after this operation is shown in .\nThe patient tolerated the procedure well and had an uncomplicated hospital course. The patient was closely followed postoperatively and did well. She completed two cycles of adjuvant chemotherapy with gemcitabine and capecitabine from January 2018 to April 2018, but did have some neutropenia at the end of her adjuvant therapy requiring pegfilgrastim. Her intermittent fevers, early satiety, and abdominal pain resolved after the surgery. She became an obligate insulin-dependent diabetic after the completion pancreatectomy procedure and now requires exogenous pancreatic enzymes to support her nutrient absorption.\nThe specimen was found to be consistent with a poorly differentiated invasive adenocarcinoma. Resection margins were negative, and 2 of 17 lymph nodes were positive for metastatic cancer. We sent representative slides of the patient's 2017 tumor and 2011 tumor to Perthera (McLean, VA) for next-generation sequencing (NGS) and histological analysis, which tested for mutations in a total of 315 genes and stained for various predictive biomarkers (). Both lesions showed the same mutations in KRAS (G12R), CDKN2A (splice site 151-1 G to A), and TP53 (Y220C). In addition, her 2011 tumor had a mutation in ACVR1B (S4) that was not present in the 2017 tumor, and the 2017 tumor had amplifications of MYC and mutant KRAS that were not present in the 2011 tumor (). Upon histological analysis, staining for MLH1, MSH2, MSH6, PMS2, pAKT, and HER2 was similar in both samples, but in the 2017 tumor, there was 60% increased staining for RRM1 and 20% increased staining for ERCC1, which changed the classification from low to high staining for ERCC1. |
A 35 year old female with past medical history significant for anxiety, hypertension, and colorectal carcinoma presented for exploratory laparotomy and placement hepatic arterial infusion pump for treatment of disease recurrence with metastases to liver and lung. Because of aberrant hepatic vasculature requiring extended dissection time and intraoperative vascular surgery consultation, the total operating time was a protracted 8 h. Methylene blue was injected by the surgeon directly into the liver to delineate hepatic vasculature and help in confirming hepatic pump placement. A total of 85 mL of 0.5% methylene blue was administered by the surgeon. Methylene blue vials were ordered and dispensed from pharmacy with dose recommendation of up to 7 mg/Kg or 560 mg (112 mL of 0.5% methylene blue) []. The surgeon documented that patient was receiving SSRI preoperatively, but it is unclear if surgeon was aware of the dangers of concomitant administration of methylene blue and SSRI. In addition, fentanyl was administered intraoperatively, and serotonin toxicity has also been described with concurrent use of MAO-inhibitors and phenylpiperidine series opioids [,]. Because the patient had also been on SSRIs and received an extremely high dose of methylene blue during surgery, she was monitored closely in the PACU postoperatively for symptoms of serotonin syndrome. While her recovery was initially uneventful, on postop day 1, she became increasingly lethargic with tachypnea, tachycardia, hyperreflexia, and myoclonus was elicited. Due to the severity and progression of symptoms, care was escalated and the patient was evaluated by the critical care service and psychiatry. Cyproheptadine was started and all serotonergic agents were discontinued. While a rare occurrence, cyproheptadine causing hepatitis and acute liver failure has been reported []. Hence, liver function tests were monitored during her treatment with cyproheptadine. The patient’s symptoms improved over the next couple of days and she was restarted on SSRI and discharged home uneventfully on postoperative day 7. |
The 70-year-old male patient was admitted to the Department of Neurology with isolated left upper extremity weakness and clumsiness. He had wrist drop and was unable to grip with the fingers. He complained of no sensory loss or numbness. His past medical history included long-term oral anticoagulation due to recurrent lower extremity deep vein thrombosis, glaucoma, and a non-significant aortic valve stenosis, with vascular risk factors including a 5-year history of treated hypertension, hypercholesterolemia, and a history of non-significant stenosis of the left anterior descendent coronary artery. On admission, no abnormality could be detected by physical examination, apart from the neurological signs, which included a wrist drop on the left side with decreased grip strength (Fig. ). Pronation and wrist dorsiflection were lost, as well as the ability to form a ring with the thumb and the index. The reflexes of the left upper extremity were slightly brisk, with not pathological reflexes present and no sensory deficits. Laboratory parameters were without abnormal findings except for a slightly elevated fasting blood glucose level. Cranial CT revealed contrast-enhancing, irregularly shaped lesions with diameters of 7, 10, and 9 mm (in the temporal, parietal, and frontal lobes, respectively), surrounded by perifocal edema. Carotid duplex ultrasonography did not demonstrate signs of focal atherosclerotic plaques, circulatory disturbance or stenosis on either side. In accordance with the findings of the CT scan, the cranial MRI confirmed the tumor in the right precentral gyrus, corresponding to the ‘hand knob’, accompanied by further similar lesions posteriorly in the parietal and the temporal lobes, and in the left frontal lobe (Fig. ). Electromyography and nerve conduction studies did not support a peripheral origin underlying the paresis. The neuropsychological examination revealed a deficit in the Luria three-step test as a single alteration, which performance could, however, be remarkably improved by verbal clues. Chest X-ray performed as part of the search for primary tumor revealed no abnormality. Non-contrast and contrast-enhanced chest CT detected an irregularly shaped mass in segment 10 of the left lung, with inhomogeneous enhancement of the contrast agent and multiple associated lymph node conglomerates, suggesting lung cancer as the primary tumor. Abdominal ultrasonography did not detect malignancy. The patient received palliative steroid therapy, with no improvement in the paresis during the observation period. Based on the recommendation of the tumor board, the patient was transferred to the Department of Pulmonology for bronchoscopic tissue sampling. The verification of the diagnosis by this means, however, was unsuccessful. The diagnostic process had to be suspended due to an acute bleeding duodenal ulcer, and the patient received blood transfusion and local hemostatic treatment in the Department of Surgery. In association with the antibiotic treatment, the patient developed pseudomembranous colitis caused by Clostridium difficile. In addition to the persisting colitis, fever occurred accompanied by hemoptysis and coughing. The subsequent chest X-ray did not confirm pneumonia. Despite the applied antibiotic and oxygen therapy, respiratory failure developed, and the patient passed away 2 months after the onset of the neurological symptom. The autopsy and the histopathological analysis identified an adenocarcinoma (mucinous tubular adenocarcinoma with solid anaplastic parts) both in the brain and the lung; (Fig. ). |
A young female, 30 years old, presented in our clinic suffering a right side unilateral vestibular hypofunction (UVH) due to vestibular neuritis (VN) []. At the time of diagnosis, she reported sudden and severe rotatory vertigo with associated autonomic symptoms over the previous 48 hours. Pure tone audiometry (PTA) in both ears was 5 dBHL; she had no tinnitus. The patient denied any previous history of related problems, although her mother was suffering from migraine headache. The patient was hospitalized three days, where she received intravenous steroids and antiemetics. Inner ear and cerebellar magnetic resonance imaging (MRI) were normal. She began vestibular rehabilitation treatment, with a progressive exercise regimen. Initially, she performed gaze stability and balance exercises 3–5 times a day in her home, for a total stimulus time of 20–40 min daily. The gaze stability exercises included the x1 and x2 paradigms for both near and far target distances. The balance exercises were provided to improve her use of vestibular information to maintain balance. We progressed these exercises by reducing the base of support, altering vision, and proprioceptive input (eyes open or closed; standing on a firm or soft surface). The gait exercises included walking in tandem, with eyes closed, with cephalic movement in the sagittal and horizontal planes. Habituation exercises were indicated based on the result of the 16 movements from the Motion Sensitivity Quotient []. The habituation movements included 4 repetitions, 4 times a day, until the exercises did not generate any symptoms for 48 hours, at which time the patient suspended them. Videonystagmography (VNG) showed unilateral weakness of the right ear at 78%. At the time of discharge, her Dizziness Handicap Inventory (DHI) was 66. The patient continued with this rehabilitation treatment for 9 months, after which the final DHI improved to 36. Although she said that she did all the prescribed exercises, she reported blurred vision and a permanent dizzy sensation. She decided to discontinue treatment but returned to the clinic after three months with the same symptoms. Additionally, she now reported a new onset of periodic headaches that did not fulfill migraine or vestibular migraine criteria []. A repeated VNG exam showed spontaneous nystagmus towards the left, with a slow phase velocity (SPV) of 7°/sec. At that moment DHI was 54; the Motion Sensitivity Quotient (MSQ) was 11.81 points; Functional Gait Assessment (FGA) was normal; and modified Clinical Test of Sensory Interaction and Balance (mCTSIB) was 120/120. She restarted vestibular rehabilitation and after 10 sessions her DHI was not better (64 points). Laboratory studies showed normal levels for FAN (Antinuclear Factor), folic acid, Anti-DNA, ionogram, magnesium, calcium, proteinogram, VDRL, and vitamin B12. However, she reported that her headaches had become premenstrual and they now fulfilled the criteria for a migraine diagnosis. She was started on 12.5 mg oral amitriptyline daily and prescribed dietary measures. Subsequently this patient reported less blurred vision and dizziness and no headaches for two months. A new MRI and an angiocerebral MRI were normal. Nevertheless, the patient returned two months later complaining of persistent dizziness, blurred vision, and no benefits from the amitriptyline in spite of the fact that the dosage had been raised to 50 mg daily. We modified her treatment to start vestibular rehabilitation again (her initial DHI was 40) and she was started on 25 mg oral topiramate daily. The patient did not tolerate this medication and discontinued its use. Next, she was prescribed 10 mg of flunarizine daily with good tolerance. Repeated VNG showed spontaneous left nystagmus with a SPV of 3°/sec. Her DHI was now 34, but her clinical dynamic visual acuity was abnormal showing a 6-line difference from static visual acuity. During the next 4 months, the patient did not come to the clinic, after which period of time she came in and was treated with 25 mg of oral venlafaxine daily (provided by another clinic). At this point, we tested with the vHIT (ICS impulse 1085 Otometrics®), which showed a gain of 0.57 in the right horizontal canal with overt and covert saccades (). The other semicircular canals had normal gains. Electrocochleography was normal, Brainstem Auditory Evoked Response was normal, a new MRI was normal, and PTA was normal. A 4th VNG showed spontaneous horizontal nystagmus to the right, with a SPV of −3°/sec (was interpreted as a recovery nystagmus), and an orthoptic evaluation was normal. Though she said she still suffered from blurred vision and dizziness, she reported fewer headaches. We prescribed 24 mg daily of betahistine (8 mg every 8 hours) to improve compensation. While the patient experienced an improvement, she reported that the dizziness persisted throughout the day. She began working again but had to stop 1 month later due to blurred vision, headaches, and dizziness. Once again, she began vestibular rehabilitation plus psychological therapy. This combination of therapies resulted in some improvement, though her symptoms persisted during head movement, especially in the dark. Her constant blurred vision made it difficult for her to read. Her 5th VNG showed that a spontaneous nystagmus to the left was 1.8°/sec, while the vHIT was the same as before. Based on persistent symptoms and lack of improvement with traditional vestibular rehabilitation, we elected to treat her using passive and predictive, yaw head impulses towards the affected side only. This was done for five consecutive days. Video HIT was used to ensure that the velocity of impulse was correct to stimulate her right horizontal canal in the field of fast movements. When the patient did the head impulse exercises, she sat in front of a solid black circle of 10 mm in diameter on a white background placed at one meter. This circle was positioned at the same level or height as her occipitonasal axis. Stimulation of the affected semicircular canal was done with 10 series of 15 passive head impulses (done by the therapist) with 30 seconds of rest between each series. The initial head position was such that the patient's gaze was centered on the point in front of her with ±2° between the horizontal and vertical planes []. The head impulses were small and fast, with a peak amplitude of 15 degrees, a peak velocity of 150°/sec, and peak acceleration of 3000°/sec; the return to the initial position was slow. Video HIT equipment was used to monitor the velocity and amplitude of movements, which were corrected when necessary.\nAfter this treatment, the patient reported resolution of blurred vision and dizziness, with no premenstrual migraines or vertigo. Her final vHIT showed a gain of 0.71 for the right horizontal semicircular canal with covert saccades (). The VOR gain remained normal in the vertical canals. Her final DHI was 12. The clinical horizontal dynamic visual acuity test was now within 2 lines of her static visual acuity (normal). After 6 and 12 months, she returned for follow-up to report that she had no more vestibular symptoms. Her premenstrual migraines persisted and she continued to have a spontaneous nystagmus beating towards the left at 1.8°/sec SPV, in the dark (). |
A 60-year old woman was admitted to the outpatient department of our hospital due to deep pain of the pelvis and distal lumbar spine. Progressive pain of the posterior right leg also developed. Neurological examination revealed a reduction of strength of the right leg and incomplete paresis of the right tibial and common peroneal nerve (both branches of the sciatic nerve). The patellar reflexes were evocable on both sides, the Achilles tendon reflex was positive on the left side but absent contralaterally. Additionally, a hypoaesthesia of the S1 dermatome was detected on the right leg. The clinical picture was therefore that of a partial impairment of the right sciatic nerve. Initially, a local irritation of the sacral spinal nerve 1 was suspected, however a magnetic resonance imaging (MRI) showed the presence of a massive tumor in the right pelvis. An extension of the MRI to the whole pelvis revealed a lipomatous appearance and the presence of septa (Figure ). The diameter of the lesion was 18 × 17 × 8 cm. The enormous tumor displaced adjacent anatomic structures, for example the right obturator and piriformis muscles were displaced to the right, the uterus and the rectum were displaced to the left. The sciatic foramen was completely occupied. The neoplasm spread out to the gluteal region, with displacement of the right gluteal muscles (minimus, medius, and maximus).\nThe lesion presented high signal intensities on T1-weighted sequences (Figure ) and a complete signal loss on fat-saturated T1-weighted images (Figure ). The fat equivalent density was verified by computed tomography (CT), with measured densities of -100 HE (Figure ). There was no pathological enhancement of contrast media (Figure ) nor any signs of bone destruction (Figure ). The CT and MRI findings, and especially the signal loss on fat-saturated T1-weighted images, indicated a lipomatous tumor, e.g. a lipoma. In addition, the huge size of the lesion and the presence of broad fibrous septa (Figure ) were considered indicative of a well-differentiated liposarcoma. The lesion was successfully resected.\nAfter surgical removal of the tumor, the histopathology confirmed the diagnosis of a well-differentiated liposarcoma (Figure ). Nearly all neurological symptoms disappeared postoperatively. Only the hypoaesthesia of the S1 dermatome of the right leg remained. |
A 66-year-old African American female with a past medical history of refractory immunoglobulin G (IgG) lambda MM, essential hypertension, and chronic kidney disease presented to the emergency department with five days of right upper quadrant pain.\nHer MM was diagnosed one year prior when she presented with altered mental status, uremia, hypercalcemia, hypoalbuminemia, and paraproteinemia. A skeletal survey at that time revealed multiple thoracic spinal lytic lesions and an eroding soft tissue mass at the level of T10. Further evaluation revealed a very high IgG level, elevated M protein band, and a kappa/lambda ratio <0.01 (normal 0.26-1.65). A biopsy from the soft tissue mass revealed a plasmacytoma. Radiation therapy was initiated for 10 days. She received three cycles of bortezomib and dexamethasone followed by two cycles of bortezomib, dexamethasone, and lenalidomide. Her disease progressed, and a subsequent bone marrow biopsy revealed hypercellular bone marrow with 70% atypical plasma cells. The patient subsequently received seven cycles of carfilzomib, lenalidomide, and dexamethasone. She was not a candidate for bone marrow transplantation given the high plasma cell burden.\nOn her current presentation, the pain was sudden in onset, intermittent, worse with eating, and without radiation. The pain was associated with nausea and anorexia, but she was without any change in bowel habits. She denied any previous similar episodes. Upon physical exam, the patient was in distress but remained alert and oriented. Her vital signs were all stable. She exhibited right upper quadrant abdominal tenderness without rebound or guarding. Her initial labs are presented in Table . The patient was admitted to the hospital for further evaluation of her abnormal labs and supportive treatment.\nThe patient was started on intravenous hydration and was made nil per os. An abdominal ultrasound revealed a distended GB with sludge (Figure ). The GB wall was thickened up to 9.5 mm, and the sonographic Murphy sign was positive. The common bile duct and common hepatic duct measured 6.3 mm and 3 mm, respectively. The liver measured 18.3 cm and exhibited normal echogenicity. There were no intraparenchymal masses or fluid collections. The portal and hepatic veins were patent.\nThe patient was diagnosed with acute cholecystitis. Intravenous piperacillin-tazobactam 3.37 g every eight hours was initiated, and the patient was referred for open cholecystectomy given her overall condition and lactic acidosis. Intra-operatively, the GB was thickened and firm but not overly distended or perforated. The GB was dissected from the liver edge, and a liver biopsy was performed successfully.\nThe pathology report from the cholecystectomy revealed chronic cholecystitis with involvement of the GB submucosa and serosa by abnormal plasma cells with lambda light chain restriction (Figure ). Subsequently, Congo red stain of the GB sections revealed apple-green birefringence throughout the submucosal areas consistent with amyloid deposits. The liver biopsy exhibited abnormal plasma cells in periportal locations with lambda light chain restriction as well; Congo red stain was not done on the liver sample.\nThe patient declined any further treatment for MM and decided to proceed with hospice care. She was discharged home with comfort measures. |
A 51-year-old lady was admitted to our hospital in February 2009 with left-sided pleuritic chest pain. She had a history of left upper lobectomy in December 2000 for a primary carcinoid tumour of the lung (chromogranin positive; no mitotic activity; Ki67 labelling index of 2%; clear margins). The patient had been discharged from followup in 2005 with no evidence of recurrence. On representing in 2009, a CT scan showed evidence of local recurrence with left-sided pleural lesions and an enlarged left hilar lymph node, as well a solitary 4.7 cm splenic lesion (). The patient was further investigated with Octreotide SPECT and Gallium 68 whole body PET studies, which showed uptake in the pleural lesions, no uptake of the splenic lesion, and normal appearance of the abdominal and pelvic viscera. However, increased activity was demonstrated in the left acetabulum, which was thought to represent bony metastasis. An ultrasound guided percutaneous biopsy of the splenic lesion diagnosed a low-grade neuroendocrine carcinoma in keeping with metastasis of the resected lung primary (chromogranin, cam 52, panCK, MFF116, synaptophysin, CD56 positive: no necrosis; no mitoses; Ki67 labelling index < 5%). After a multidisciplinary discussion, a funding application for Lutetium 177 DOTATATE therapy was made. Prior to the commencement of this therapy, a repeat CT scan in September 2009 showed a decrease in the size of the patient's pleural deposits, but an increase in the splenic lesion to 5.8 cm. In view of the isolated progression of the splenic lesion, it was decided that Lutetium therapy would be more effective following a reduction in the tumour load by excision of the splenic mass. As a result, the patient underwent a laparoscopic splenectomy in November 2009.\nThe patient was placed in the right lateral decubitus position, and 4 ports were placed in the left flank. The colonic attachments were divided, and the short gastric vessels were controlled with the Ligasure vessel sealing system (Covidien, Valleylab, Boulder, CO, US) to allow isolation of the splenic hilum (). The splenic artery was then dissected and ligated with a hemostatic clip (). Finally, the splenic hilar vessels were dissected and divided using a vascular linear stapler (Ethicon Endo-Surgery, Inc. Cininnati, OH, US) (). Following complete splenic mobilisation (), the spleen was placed in a leak-proof bag and exteriorised to the abdominal wall. Histological analysis of the specimen confirmed a metastatic carcinoid tumour (chromogranin, cam 5.2, MFF116, synaptophysin, NSE and CD56 positive; no necrosis; Mitotic count 2 per HPF; Ki67 labelling index <5%). The patient made a good postoperative recovery and was discharged on the second postoperative day.\nSince discharge the patient has been commenced on Lutetium 177 DOTATATE therapy. Prior to commencement, repeat PET scan in April 2010 showed the formation of several liver metastases. The patient's pleural disease had also increased whilst her bony acetabular lesion was unchanged in appearance. The patient has since completed 3 cycles of Lutetium therapy and her latest Gallium PET scan from October 2010 shows little disease progression. Clinically, the patient has no symptoms of carcinoid syndrome and but suffers from chronic pain at the sights of her left pleural deposits and left acetabular metastasis. She is currently being considered for external beam radiotherapy to her left hip. |
In February 2007, a 61-year old male was referred by his local dentist for the chief complaint of a swelling at site of tooth 14, where an implant was present. In December 2002, the patient’s maxillary right first premolar was extracted. A Nobel Biocare TiUnite dental implant was used to replace the missing tooth in February 2003. Since the time of implant placement, the patient noted swelling in the buccal gingival area where the implant was placed. He could feel it with his tongue and fingers. He had always been slightly uncomfortable with this. He preferred that his gingival tissue be returned to its previous form.\nIntraoral clinical examination revealed an implant supported porcelain fused to metal crown replacing the maxillary right first premolar. A peri-apical radiograph of the implant revealed no signs of peri-implant bone loss or radiolucency. Periodontal examination revealed stability of the implant with probing depths ranging from 2-3 mm. There were no clinical signs of peri-implantitis. There was, however, a 5 × 5 mm raised area in the non-keratinized soft tissue facial to the implant []. There were no signs of erythema or infection. Upon digital palpation, there was no soft tissue drainage and the raised area was found to be firm and screw threads could be detected. The patient did not experience any discomfort upon percussion. Hard and soft tissue examination of the rest of the mouth was normal. We recommended surgical exploration and modification of the protruding implant. The patient consented to the treatment plan\nThe surgery was performed under local anesthesia. Sulcular incisions were made extending from the mesio-buccal of the right maxillary first molar distally to the mesio-labial of the right maxillary canine. A vertical releasing incision was also made at the mesio-labial aspect of the right maxillary canine. A full thickness buccal flap was raised. The area of the implant and surrounding alveolar bone was exposed. It was apparent that the apical portion of the dental implant had perforated cortex of the alveolar bone []. The implant was osseo integrated. There was no necrotic bone surrounding the implant. A high speed bur was used to remove the portion of the implant protruding through the buccal plate of bone. A round diamond bur was used to flatten the implant and try to make it flush with the buccal plate of bone []. Copious irrigation was utilized. The facial flap was then repositioned and secured with 4-0 gut suture []. The patient was seen at four weeks [], three months [] and six months []. The area healed uneventfully. |
A 6-year-old boy was brought to the neurology clinic with a 6-month history of inability to walk. Onset was gradual and the problem progressed slowly until the patient was bedridden. The boy was a product of a full-term uneventful pregnancy who was delivered through spontaneous vaginal delivery. There was no history of trauma, fever, weight loss or any developmental delay during infancy. Dietary history was significant in that he was drinking only milk during the last 3 years. His past medical and surgical history was unremarkable. He was conscious but irritable and resisted examination, lying on the couch with his legs flexed at hips and knees (). There were no rashes, petechiae, hematoma, or gingival bleeding. During the neurological examination, power in the lower limbs could not be assessed as he refused to exert at all. Reflexes were found to be exaggerated and plantars were bilaterally flexors. Upper limbs were normal. Cardiovascular examination was normal and there was no organomegaly. Respiratory system examination was normal, but examination of the chest cage revealed pectus excavatum with scorbutic rosaries ().\nX-ray of the lower limbs, upper limbs and pelvis showed the classical appearance of scurvy with a thin cortex and scurvy line. A vitamin C assay showed a markedly low level of 0.5 mg/L (normal, 4-20 mg/L). Other investigations, including a complete blood count, liver function tests, renal profile, calcium profile, thyroid function tests and vitamin D level were normal. CT of the brain was normal. The patient was started on a vitamin C supplement, 250 mg twice daily with a vitamin C rich diet. His condition improved dramatically and pain subsided. He started to move his legs and mobilized. Within 10 days he was back on his legs and walking with no support. Vitamin C supplementation was continued for 3 months and a follow-up vitamin C level showed that it was back to normal. The parents were educated regarding proper diet, and they started to follow a healthy well-balanced diet. |
A 57-year-old male was admitted to our hospital with a 5-day history of severe dizziness, mild chest pain and dyspnea. On admission, he had a pulse rate of 115 bpm, a blood pressure of 80/60 mmHg, and a respiratory rate of 24. He had a history of hypertension and no diabetes mellitus. On physical examination, there were no pathological signs for heart sounds, but some wet rales were detected in the lower lung region. There was minimal bilateral leg edema. Cardiac troponin T levels were clearly normal. The electrocardiogram (ECG) did not show any new myocardial infarction (MI) sign. When checking patient history, we discovered that he had suffered STEMI four weeks prior. In the coronary angiography performed, an occlusive lesion was detected in the distal circumflex artery and he was discharged with medical treatment (). There was a right type of coronary dominance. Transthoracic echocardiography showed a 3-cm wide wall defect on the posterior side of the left ventricle, a 57-mm pericardial effusion and the left ventricular ejection fraction (LVEF) was 40% (). The operation was performed immediately via standard median sternotomy. Adherence of the pericardium was at an advanced level. The anterior part of the pericardium was dissected carefully for cannulation. We used cold potassium-based cardioplegia (Plegisol®, Baxter, Chicago, USA) for cardiac arrest and continued with cold blood cardioplegia. After a standard cardiopulmonary bypass (CPB) procedure, we separated the entire pericardium from the heart. On the posterior region we detected a formation like pseudo-aneurysm which was a 2-mm thick membrane formed by adhesion. It was almost completely covered the posterior face of the left ventricle. After opening this membrane, the myocardial defect was seen (). We considered that it was a subacute situation because of the non-existence of fragile necrotic tissue. The defect was sutured with eight separated and teflon felt supported 3-0 prolene sutures ( and ). Tissue glue was applied on the sutured line. Finally, the residual capsule existent from adhesion was closed with a 3-0 prolene continuously (). There was no need for a concomitant CABG according to angiography and intraoperative examination. No bleeding occurred from suture line or anywhere else. Weaning from CPB was performed with the support of an intra-aortic balloon pump and dopamine medication. While aortic cross clamp time was eighty-seven minutes, total CPB time was one hundred and twenty one minutes. Postoperative transthoracic echocardiography showed normal left and right ventricular size (LVEF of 45%) without pseudo-aneurysm and minimal pericardial effusion (). Intra-aortic balloon pump was removed on the second postoperative day. He was discharged without any follow up events at postoperative day seven from our hospital. |
A 44-year-old male, manual worker, non – smoker, presented to our outpatient orthopaedic department for chronic pain in the volar side of the right wrist. He was of Greek origin. His weight was 82 kg and height 1, 78 m. He had no past medical history and was social alcohol drinker. He did not reported any major family history. There was no history of trauma and no previous operation in the upper limb. Physical examination revealed tenderness on palpation of the area over the triquetrum. There was no sensory deficit involving the median nerve and no other pathological signs. Antero – posterior and lateral x-rays were performed and the co-existence of ulnar and radial exostoses together with 4th and 5th short metacarpal bones and two sesamoid ossicles in the wrist area were found (Fig. ). X-ray examination of the left hand was normal. Hence, the symptoms were attributed to the presence of the one sesamoid ossicle over the triquetral bone at the volar aspect of the wrist. Pain alleviate medications were prescribed and the patient was advised to avoid heavy manual work.\nThe exostoses arose from the distal metaphysis of the radius and ulna (Fig. ). The radial one emerged from the ulnar side of the distal metaphysis and had a sideway and proximal direction. The length was measured to be around 2.3 cm. The ulnar exostosis emerged from the radial side, had a triangular contour and a size of about 0.5 cm.\nThe sesamoids were 2, both in the volar aspect of the wrist (Fig. and ). The first one was located anterior to the triquetral bone; its diameter was measured to be 0.55 cm and considered to be the cause of pain. The second one was located anterior to the hamate bone and the base of the 4th metacarpal bone and its diameter was measured to be 0.48 cm. There was no pain on palpation in this area. |
A 39-year-old woman, who had been taking medication of quetiapine as an antipsychotics for depression, experienced mild dysarthria and visited the department of neurology in our hospital. Her symptom was diagnosed as drug-induced lip dyskinesia, which disappeared in a week. Screening head magnetic resonance imaging (MRI) at this time revealed stenosis of the left MCA with no brain parenchymal lesions (), why she was consulted to our department. We performed angiography, confirming moderate M1 portion stenosis (Figures and ). SPECT showed no apparent laterality in CBF, thereby we decided to observe her with no treatment.\nSeven months later, the patient experienced mild weakness and numbness in her right hand and visited our department. Although MRI showed no apparent ischemic change in her brain, arterial spin labelling (ASL) of MRI detected the decrease of CBF in the left cerebrum (), which was thought to well correspond for her symptoms. She was admitted and treated with an antiplatelet agent. Two weeks later, she still complained of numbness in her right hand; thereby, we decided to perform left STA-MCA anastomosis to prevent deterioration of her symptoms. Preoperative SPECT showed no apparent laterality in CBF (). On operation, left temporal craniotomy was performed, and the parietal branch of the STA was anastomosed with the M4 portion on the temporal lobe (Figures and ). The intraoperative course was uneventful, and the patient recovered from anesthesia without any new neurological symptoms\nPostoperatively, her speech was normal until postoperative day 1 (POD1). On POD2, she exhibited mild speech disturbance, which worsened day by day finally resulting in complete motor aphasia on POD6. Her comprehension was kept normal. On POD3, generalized convulsion occurred, which ceased quickly by diazepam, and levetiracetam was initiated. On the same day, she presented with mild weakness of right upper extremity, which improved gradually and disappeared on POD7. MRI and CT showed no ischemic or hemorrhagic changes, but ASL and SPECT revealed remarkable increase of CBF in the left cerebrum (Figures and ), by which the symptoms were diagnosed as CHPS. Despite the treatment with strict blood pressure and the administration of edaravone and minocycline, complete motor aphasia remained unchanged on POD21. MRI showed no abnormality except slightly hypointense changes on T2 weighted images and FLAIR (). At this point, the patient was discharged partly because of the request from the patient, and we continued to follow her in outpatient visit. One month after the surgery, the patient started to utter words that were not fluent, when SPECT and MRI showed normalization (). Thereafter, the improvement of her speech was slow, and totally more than three months was taken for full recovery after the surgery. |
A 75-year-old female presented acutely with a history of sudden onset paraesthesia and ischemic rest pain involving the left foot and ankle. Motor function was retained, capillary and superficial venous return were reduced, the foot was cold, and there were no palpable pedal pulses. The calf remained soft to palpate. There was no history of palpitations or arrythmia, and the patient was in sinus cardiac rhythm both clinically and on electrocardiography. Plain chest radiography and computed tomography angiography (CTA) excluded a proximal arterial source of embolism. Furthermore, there was no previous trauma, (local or systemic) infection or inflammation, collagen, connective tissue or genetic syndromes, or arteritis; nor was there a family history of aneurysmal disease. Positive cardiovascular risk factors included hypercholesterolaemia and hypertension. Baseline haematological and biochemical analyses were normal, as were the renal and liver function. The creatinine kinase was within normal limits. The right foot was well perfused with a palpable PTA pulse.\nCTA demonstrated no significant stenosis in the supragenicular vessels bilaterally. On the symptomatic left side, the tibioperoneal trunk (TPT) and anterior tibial artery (ATA) were patent proximally, with the ATA and peroneal artery occluding in the distal calf. The dorsalis pedis was occluded; however, the plantar arteries reconstituted. Dominant flow to the foot was via the PTA, which occluded in the distal calf (). These findings were considered reflective of embolic disease within the left-sided tibial arteries. The asymptomatic right leg demonstrated dominant flow to the ankle via the PTA. The ATA and peroneal arteries occluded in the midcalf.\nDue to the acute nature of the patient's symptoms, emergent thromboembolectomy of the popliteal and tibial arteries was performed via a medial approach to the distal popliteal artery. Selective thromboembolectomy using Fogarty catheters for each tibial artery was unsuccessful. Urokinase was also injected into each tibial artery without effect. Subsequent exposure of the dominant PTA at the ankle demonstrated a thrombosed 10mm diameter aneurysm. A PTA thrombectomy was performed and subsequent angiography demonstrated flow to the foot via the aneurysmal PTA (). The aneurysm was opened and demonstrated a mixture of old, organised, and fresh thrombus (). The PTA aneurysm was excised and thrombectomy established adequate inflow and backflow to the excised PTA segment. The PTA was repaired using an interposition long saphenous vein graft (). Postoperatively, the PTA pulse was present, the foot had brisk capillary refill, and motor and sensory function normalised. Follow-up at 16 months demonstrated the patient to have a well perfused left foot with a palpable PTA pulse.\nAlthough the patient's contralateral foot was asymptomatic, she opted to have this repaired electively considering the aneurysmal right PTA aneurysm was the single arterial supply to the foot. Repair was performed via excision of the 8mm PTA aneurysm and a spatulated primary end-to-end PTA anastomosis. Completion angiography was satisfactory, and the right foot was well perfused with a palpable PTA pulse postoperatively. Histopathology of the right-sided PTA aneurysm demonstrated findings consistent with atherosclerotic degenerative aneurysmal disease. Specifically, there was intimal thickening, extensive lamina disruption, and broad areas of media replaced by collagenous fibrous tissue, with calcification of the media. There was no inflammation or mural necrosis seen. |
A 28-year-old man presented to the emergency department with right iliac fossa pain, which had been present for one week and worsened over the last day. On examination, he was afebrile, blood pressure was 115/65 mm Hg, heart rate was 89 beats per min, and oxygen saturation was 98% at ambient air. His abdomen was soft with a palpable 5-cm mass in the right lumbar region. Palpation of the mass elicited pain over the lateral aspect of his right knee. Results of an initial laboratory evaluation (Table ) were normal as well.\nAn abdominal radiograph performed on admission did not reveal any abnormality. A computed tomography (CT) scan of his abdomen (Figure , , Figure ) was done, given the palpable mass; the CT revealed an ovoid heterogeneous mass with intralesional enhancement and calcification in the right iliac fossa. It appeared to have a tail superior-posteriorly and was located along the course of the right lateral femoral cutaneous nerve, raising the possibility of a schwannoma. Magnetic resonance imaging (MRI) of the abdomen was done for further evaluation and revealed an ovoid mass measuring approximately 4.6 cm x 4.2 cm x 5.7 cm at the level of L3 - L4 with a tail-like appendage to the proximal and distal parts with features highly suggestive of an ancient schwannoma (Figure ). A CT-guided biopsy was performed, which confirmed the diagnosis of schwannoma. The histopathology of the mass revealed the schwannoma to be benign. The mass showed cores of spindle cell lesions with moderate cellularity. The spindle cells were bland-looking and arranged in vague fascicles, set in a collagenous stroma. They exhibited wavy nuclei with focal nuclear palisading. No mitosis was seen. Tumor cells were diffusely and strongly positive for S100, while being negative for CD117 and DOG1.\nPain over the right knee was due to nerve compression. Treatment options were discussed with the patient. The patient was not keen for surgical resection of the tumor due to financial problems and as the surgical resection could possibly result in residual numbness over the leg and may affect his gait. Therefore, we planned to manage the patient conservatively with analgesia. His pain was controlled well with gabapentin, 300 mg every night. |
A two-year-old boy with no past medical history presented to our pediatric ED brought by his mother due to worsening left ear redness and swelling that started two days prior. The mother believed it started after an insect bite to the auricle of the left ear, causing the patient to frequently scratch the ear. There was no reported fever, otorrhea, impaired hearing, previous history of otitis externa, otitis media, skin infections, or other symptoms. On physical exam, the left ear appeared erythematous and swollen in the helix and antihelix area (Figure ), with swelling best noted on the posterior aspect of the auricle (Figure ).\nOn palpation, there was tenderness of the auricle, warmth and fluctuant swelling in the affected area. There was sparing of the earlobe and tragus. The mastoid area was not tender or swollen. The ear canal was clear without evidence of otitis externa, and the tympanic membrane was normal. The remainder of the patient’s physical exam, including vital signs, was normal.\nThe diagnosis was made by history and physical exam findings. Important features that help distinguish this case from an expanding otitis externa or otitis media were the sparing of the ear lobule and normal ear canal and tympanic membranes. Other differential diagnoses considered included malignant otitis externa, mastoiditis, and auricular hematoma, but each of these has distinctive features that are not present in perichondritis and are well described in the literature, therefore will not be discussed in this case report.\nThe patient was treated with ibuprofen 10 mg/kg, one dose of intravenous (IV) piperacillin/tazobactam 100 mg/kg (piperacillin component), and a single dose of dexamethasone 4 mg IV. After discussion via phone call with the hospitalist and ENT specialist, we decided to admit the patient to the pediatric inpatient unit for close monitoring and evaluation by ENT during the next 24 hours. The discussion of whether to use a fluoroquinolone to cover Pseudomonas came up, but it was decided to continue IV piperacillin/tazobactam 100 mg/kg every eight hours, based on local antibiogram guidance.\nThe patient was evaluated by ENT the next day and was found to have significantly improved. He did not have any fluctuance, which ruled out the initial suspicion of a developing abscess or suppurative perichondritis. The patient was discharged home at the end of admission day one, after discussing with the patient's mother the need to have outpatient follow-up within that same week and to continue giving oral antibiotics at home as prescribed by the ENT specialist. The patient received a total of four doses of IV piperacillin/tazobactam 100 mg/kg during admission and was discharged with a prescription for oral cefdinir 7 mg/kg every 12 hours for 10 days. The patient’s mother was contacted via phone call and reported that the patient’s symptoms resolved completely on approximately day four of treatment and the patient completed a full 10-day course of antibiotic. She did not attend the follow-up visit with ENT or pediatrician as the patient was feeling well. On a three-month post-ED visit phone call, the mother denied perichondritis recurrence and reported no other complications or cosmetic sequelae. |
A 33-year old truck driver man was admitted to the hospital emergency department with a history of one week headache and acute diplopia. He stated that during recent week, he had a transient decreased level of consciousness resulting in a minor car accident. No important finding was reported in his past medical and drug history. At admission, his general appearance and consciousness level was normal; in addition to, vital signs including body temperature, blood pressure, pulse rate and respiratory rate were stable. No cervical stiffness or focal neurologic signs were found. Papillary edema was found in funduscopic examination.\nThe peripheral blood white cell count was 15000/μL with a neutrophil left shift (PMN = 80%). Blood culture was negative. There was no other positive laboratory finding. Chest X-ray and electrocardiogram (ECG) were normal.\nThe brain CT scan without contrast revealed a large mass lesion in left parieto-occipital area with prominent vasogenic edema and midline shift (). We requested magnetic resonance imaging (MRI) with and without contrast for more investigation (). There was a mass with size of 5*4*5 centimeter with ring enhancement in favor of a high grade glioma metastasis or brain abscess.\nThe patient underwent a left temporoparietal craniotomy. After opening of dura, we used transsulcal approach. During surgery, a purulent mass was observed. Complete drainage of purulent discharges and then irrigation was performed. Duraplasty was done to avoid potential complications of brain edema. Histopathology of biopsy specimen revealed a pyogenic abscess. Direct observation of purulent fluid under a microscope indicated a large amount of PMN cells. Furthermore, the cultures were negative.\nTo find the source of brain abscess, we requested CT-Scan of paranasal sinuses and mastoid part of temporal bone. There was no evidence of otitis media, paranasal sinusitis or mastoiditis.\nConsidering a hematogenous source, we did transthoracic echocardiography. It showed mild right ventricular (RV) and right atrial (RA) enlargement, significant left to right shunt (QP/QS=2), normal left ventricular (LV) and RV function with normal pulmonary artery pressure (PAP).\nWe requested transesophageal echocardiography (TEE) to determine the location of shunt and probable vegetation. Contrast study showed bidirectional shunt but predominant phase was left to right. In addition to, a moderate size superior sinus venosus type atrial septal defect (SVC type ASD) with partial anomalous right upper pulmonary vein connection was detected in TEE. There was no vegetation ().\nPatient received intravenous vancomycin, metronidazole and ceftriaxone for a total of 6 weeks and was cured. His headache was gradually resolved. At the time of discharge from the hospital, no lesion was detected in brain CT-scan. No physical and mental symptom was reported in 6 months follow up. In the control MRI which was provided 6 months after surgery no positive finding except mild arrested hydrocephaly was reported.\nThe patient did not accept our suggestion to undergo surgery for closure of ASD. |
A 61-year-old woman presented with two supernumerary nipples located along the milk line on each side of the upper abdomen. During a few months before referral, the patient had noticed a firm palpable mass in close relations to the supernumerary nipple on the right side (). She had no other symptoms. Bilateral mammogram and ultrasound revealed normal breast parenchyma. Ultrasound of the supernumerary nipple on the right side confirmed a small mass in relation to this nipple, presenting as a hypoechoic, well-defined area, measuring approximately 10 mm in diameter ().\nUpon clinical examination, the supernumerary nipple on the right side presented with inversion and a palpable firm mass underneath this nipple. Ultrasound-guided needle core biopsy (16G) was performed (). The pathology report described elongated epithelial islands composed of cubic cells, with a small centrally located lumen with focally identifiable cuticles. The epithelial structures were surrounded by a dense fibrous stroma. The microscopic analysis indicated the possibility of SyT and the lesion was categorized as a B3-lesion (a lesion with uncertain malignant potential) ().\nFollowing a Multidisciplinary Team Conference, it was decided to recommend resection with a 5 mm rim of normal tissue, which was subsequently performed ().\nMacroscopically the tumour measured 7 x 5 x 5 mm and was described as a firm and grey-white tumour, in close relations to the supernumerary nipple. Microscopically the tumour measured 13 mm in diameter and was localized in the deep part of dermis and underlying subcutaneous tissue with no relation to the epidermis. The tumour was composed of solid trabecular and glandular imitating formations with focal cysts. The epithelium consisted of cells with slightly irregular nuclei and indistinct nucleoli, surrounded by an eosinophilic cytoplasm. The glandular structures were lined by cuboidal cells. The tumour was in close association with the lactiferous ducts and smooth muscle of the nipple (). Foreign body giant cell reactions, due to ruptured cysts, were identified.\nImmunohistochemical analysis showed positive reaction for CK5, CK14, and P63 () in association with the epithelium presenting as solid cords, whereas the glandular luminal cells showed positive reaction for CK7 (). Thus, the immunohistochemical analysis demonstrated the complex nature of this lesion. The diagnosis was SyT based on the pathology report.\nA re-excision was performed due to insufficient rim of normal tissue in the cranial direction in order to minimize the risk of recurrence. |
A-68-year-old male with no significant past medical history presented to our hospital with acute respiratory distress and inspiratory stridor, necessitating emergency intubation and mechanical ventilation. Two months earlier, he had been operated upon in our hospital for diverticulitis of the large intestine and had made an uneventful recovery. In the intensive care unit, fiberoptic laryngoscopy carried out to determine the cause of his stridor revealed immobile vocal cords in the paramedian position. Neurology consultation was requested to rule out the possibility of vocal cord paralysis due to involvement of the lower cranial nerves. Magnetic resonance imaging of the brain and spinal cord was normal and cerebrospinal fluid studies excluded infectious or inflammatory pathology. Tracheostomy was under consideration to ease the management of secretions. The next day, while been re-examined by one of us (PKS), mild drooping of the left eyelid was noticed with positive fatigability. Neurological examination was limited as the patient was intubated, but there was no definite clinical weakness of the proximal musculature and forward arm abduction time was within normal limits (power 5/5 medical research council grade). We were unable to clinically assess the bulbar musculature and muscles of mastication on account of the patient’s intubated state. To identify myasthenia gravis, the Tensilon (edrophonium) test was performed, which briefly relieved eyelid weakness. During this procedure, the patient was carefully monitored because edrophonium can cause significant bradycardia, heart block and asystole. Therefore, atropine and cardiopulmonary resuscitation equipment was made available at the bedside. Single-fiber electromyography and repetitive nerve stimulation (RNS) studies to assess for increased jitter and a decremental response, respectively, were not performed as they were unavailable on an emergent basis. Injection neostigmine 3 mg intravenously under continuous cardiac monitoring every 6-hourly was initiated and, within 48 h, the patient was able to be weaned off the ventilator. The total dose administered was 24 mg over 48 h. Acetylcholine receptor antibodies (anti-AChR) came back negative. Anti-MuSK antibodies were not sent due to the non-availability of a testing facility. |
We describe the case of a 56-year-old Caucasian woman who presented acutely with left arm numbness and pain lasting for nearly eight hours. She had had similar episodes of numbness a few days previously, but these had lasted for only five to 10 minutes each time.\nShe smoked 20 cigarettes/day. Her heart rate was regular, although her blood pressure was lower in the left arm than in the right arm (103/80 mmHg vs. 170/80 mmHg, respectively). Her left arm looked pink but cool, with no palpable brachial, radial or ulnar pulse. There was decreased sensation over the forearm, though there was no motor deficit.\nHer chest, cardiovascular and abdominal examinations showed no other abnormalities. She underwent urgent computed tomography, which showed an acutely thrombosed left subclavian artery.\nShe was put on an intravenous heparin infusion and magnetic resonance angiography was arranged (Figure ). Initial angiograms obtained through the femoral artery in the groin showed a tight stenosis which was right at the origin of the subclavian artery. As a result, a guidewire could not be passed through the groin puncture despite several attempts. The brachial route was therefore chosen. A guidewire was passed using a left brachial artery approach through the narrowing. A 5 mm × 4 cm stainless steel stent Genesis (Cordis Endovascular, Warren, NJ, USA) was subsequently deployed and, when ballooned, although it clearly had eliminated the atherosclerotic lesion, the diameter was less than the diameter of the native normal vessel. To improve conformity, the stent was ballooned to 6 mm, which improved the conformity. A good, brisk flow through the stent was confirmed, and the procedure was subsequently completed (Figure ).\nUnfortunately, the patient continued to smoke heavily and was soon re-admitted with recurrent symptoms. The duplex repeat angiogram confirmed an occlusion of the left subclavian stent. The occlusion was successfully traversed, and a 6 cm-long, 7 mm S.M.A.R.T. Nitinol Stent System (Cordis Corporation, Miami Lakes, FL, USA) was deployed through the original stent with a good result (Figure ).\nUnfortunately, the stent blocked again for the second time, and a decision made to carry out a bypass rather than perform repeat radiological re-intervention. She therefore underwent a carotid to axillary bypass.\nIntra-operatively, the left common carotid artery was approached through a longitudinal incision along the medial aspect of the left sternocleidomastoid (SCM). The artery was exposed and controlled. The left axillary artery was approached through an infraclavicular incision parallel to the clavicle. The artery was exposed and controlled. A total of 3000IU of heparin were given intravenously. A 6 mm ringed PTFE graft was tunneled under the SCM and over the clavicle and anastomosed using a 5-0 Prolene suture (Figure ). Good pulses were established after the procedure. The patient had an uneventful recovery, and her claudication symptoms settled completely. At her one-year follow-up examination, the graft was still patent and she was asymptomatic. |
A 54-year-old previously healthy Caucasian female with otherwise unremarkable past medical history presented to emergency department with one-day history of hematochezia and abdominal pain. The patient described crampy left lower quadrant pain with no aggravating or relieving factors. She had a total of five bowel movements since symptom onset with the first bowel movement containing stool mixed with bright red blood followed by predominantly bloody stools. She took no medications on a regular basis and denied having a screening colonoscopy for colorectal cancer at age 50. She reported symptoms of upper respiratory tract infection (cold, sneeze, and cough) for which she took three doses of 120 mg pseudoephedrine purchased from a local grocery store for 1 day prior to symptom onset. Her maternal grandfather had prostate cancer but there was no significant gastrointestinal tumor history in the family. She was a nonsmoker and reported drinking socially (roughly one standard drink) once a week.\nHer admission vitals were within normal limits. Physical examination was consistent with mild tenderness on the left side of abdomen and hypoactive bowel sounds. Rectal examination showed bright red blood without any stool in the rectal canal. Her laboratory values were significant for mild anemia with hemoglobin of 11.5 mg/dl, hematocrit of 34.5%, erythrocyte sedimentation rate 31 mm/hr, and C-reactive protein 2.15 mg/dl. A computed tomography scan revealed mild to moderate mural thickening of the descending/sigmoid colon consistent with colitis without pericolonic abscess, ascites, or free air (). An infectious workup was obtained including blood cultures, stool cultures, gastrointestinal panel for Clostridium difficile, and gastrointestinal viruses but was negative. She was resuscitated with intravenous fluids.\nThe patient underwent colonoscopy which demonstrated segmental moderate inflammation in the sigmoid colon, descending colon and splenic flexure along with internal and external hemorrhoids. There was evidence of submucosal hemorrhages with mild edema in the aforementioned segments of the colon (). Endoscopic findings were highly suspicious of ischemic colitis. Several biopsies were obtained from the inflamed areas which exhibited focal lamina propria eosinophilic change with mild crypt attenuation and loss of goblet cells consistent with mild ischemic changes. There was no evidence of chronic inflammation.\nShe was observed in the hospital for 3 days and her diet was progressed slowly. Her bloody bowel movements ceased after 1 day in the hospital and patient was counseled and educated regarding avoidance of pseudoephedrine and over the counter medications for symptomatic management. |
A 45-year-old man with a history of hypertension, dyslipidemia, and habitual drinking visited our hospital with complaints of severe chest pain, back pain, bilateral shoulder pain, and vomiting. He fell ill after heavily consuming alcohol for a day. Three and a half hours prior to his arrival, he experienced discomfort in his chest followed by vomiting. Two hours and 20 min prior to his arrival, he felt nauseated, describing a feeling of sudden stiffness throughout his entire upper body. On arrival, he was alert, his blood pressure was 139/98 mmHg, pulse rate was 64/min and regular, body temperature was 36.5 °C, respiratory rate was 20/min, and oxygen saturation was 95%. He was 173 cm tall and weighed 73.1 kg. No abnormalities other than cold sweat and epigastric tenderness were noted on physical examination. Chest radiography of the lateral view (seated position) showed several areas of free-air just below the diaphragm (Fig. ). Contrast-enhanced computed tomography (CT) showed free-air, bilateral pleural effusion, and a dilated esophagus and stomach (Fig. ). Based on these findings, an esophageal rupture with mediastinitis was the definitive conclusion and he was thus diagnosed with Boerhaave syndrome. A subsequent esophagogram further confirmed the diagnosis. Nine hours after the onset of the rupture, an emergency operation was performed as follows. Through a left thoracoabdominal incision, closure of the perforation and drainage of both the mediastinum and thoracic/abdominal cavities were performed in succession. The penetrating laceration was approximately 3 cm in length vertically on the left posterior wall of the distal esophagus and was patched with the patient’s omentum. Although the patient had a complication of anastomotic insufficiency on the 16th day, it was ultimately resolved. The patient was then administered a liquid diet on the 35th day, and he was discharged on the 41st day. Upper gastrointestinal endoscopy was performed on the 98th day, and a scar due to the suture was observed in the same location as the previous perforation (Fig. ). |
A 50-year-old woman who had been in a vegetative state for two months due to a spontaneous intracerebral and intraventricular hemorrhage was transferred to our hospital. A brain computed tomography (CT) scan revealed severe hydrocephalus without intracerebral hemorrhage and intraventricular hemorrhage. Five days after admission, a VP shunt was implanted. Fifteen days later, the patient had a temperature over 38℃ and a chest X-ray showed patchy consolidation with partial collapse in the right lung. A sputum culture isolated Pseudomonas aeruginosa and Staphylococcus aureus. Cerebrospinal fluid (CSF) and blood cultures were negative.\nThe patient received an initial regimen of intravenous antibiotics (piperacillin/tazobactam and fluconazole), which was shifted to ciprofloxane due to a sustained high fever until the fever subsided. However, the patient became febrile again 6 days later. Liver function tests yielded an alanine aminotransferase level of 115 U/L and an alkaline phosphatase level of 702 IU/L. Fortunately, a chest X-ray showed gradual improvement of the right lung compared to the previous study. At that time, an ultrasonography of the liver revealed two 6-cm-sized hypoechoic masses. A subsequent abdominal CT showed multiple irregular-walled and diverse-sized cysts, including two large ones, which were not enhanced (). The long segment of the distal shunt tube was found going through the subcapsular portion of the right hepatic lobe and the tip of the tube was embedded in the lower large cyst (). Ultrasonography-guided needle aspiration of the upper lesion and percutaneous catheter drainage of the lower lesion resulted in continuous gushing of yellowish pus. Culture of the abscess isolated Staphylococcus capitis (S. capitis), which was sensitive to vancomycin. The patient became afebrile with the administration of intravenous vancomycin at a dose of 500 mg every 12 hours for several days. The patient was unremarkable neurologically and neuroradiologically. A subsequent abdominal CT showed a marked shrinkage of the liver abscesses. A VP shunt revision was then performed with the distal shunt tube extracted uneventfully from the liver, which was enveloped in yellowish pus. The result of CSF culture was negative. Follow-up examinations have been unremarkable, with normal shunt function without the recurrence of liver abscesses. |
A 68-year-old male was admitted to hospital with a history of dyspnea at rest for 2 d, a cough producing mucus, loss of appetite and diarrhoea. Pneumonia was suspected, and the patient received intravenous antibiotic treatment. During the patient's second night in hospital, he was found groggy and walking near his room by the nurses. They took him back to his room and connected a new antibiotic dose to his venous catheter. A few hours later, he was found unconscious, lying on the floor next to his bed. His peripheral venous catheter was connected to a nasal cannula delivering O2 from the wall, with a flow rate of approximately 2 L/min, and the antibiotic dose was found in the garbage can. As post-mortem lividity was present, no resuscitation attempts were performed by the clinicians and the death was pronounced. The body was immediately brought to the University Center of Legal Medicine (Lausanne, Switzerland).\nBefore any manipulation of the corpse, a native CT scan was carried out at around 10 h post-mortem using an eight-row CT unit (CT LightSpeed 8, GE Healthcare, Milwaukee, WI). All scanning parameters are detailed in . A forensic pathologist immediately viewed the native CT images. To evaluate the distribution of gas because of physiological changes in the body after death, the radiological alteration index (RAI) was used as proposed by Egger et al. []. The RAI was based on the analysis of samples from seven sites (heart cavities, liver parenchyma and vessels, left innominate vein, abdominal aorta, kidney parenchyma, L3 vertebra and the subcutaneous pectoral tissues).\nAll images were interpreted in a consensus reading by one board-certified radiologist and one forensic pathologist who were trained in forensic imaging. A post-mortem radiological report was prepared and described all findings from the native CT scan. The native CT scan revealed the presence of subcutaneous, intramuscular and intravascular gas, a pneumoperitoneum, a bloated heart with the right cavities filled with gas and a left pneumothorax ((a–d)). The maximum RAI score is 100, and RAI scores greater than 50 are usually seen in cases of severe changes []. Therefore, given the short post-mortem interval (<12 h), the elevated RAI obtained in this case (75) strongly suggests an exogenous source of gas in the tissues.\nCT-guided gas samples were immediately taken from multiple sites (carotid artery, jugular vein, right auricle, thoracic aorta, pectoral muscle, thoracic cavity, abdominal cavity, scrotum and gluteal soft tissues, (e,f)) according to the protocol for gas analysis described by Varlet et al. [].\nThe external examination revealed massive subcutaneous emphysema with audible crepitations on the entire surface of the body. Additionally, there was a bruise on the left part of the forehead, a small contusion near the left eyebrow surrounded by a purplish-blue bruise and some bruises of different ages on the inferior part of the thorax and on the legs. On the left arm, a venous catheter was still in place.\nSamples for toxicological and biochemical investigations (blood and urine) were collected in S-Monovette® tubes with sodium fluoride or ethylenediaminetetraacetic acid as a preservative (Sarstedt, Nümbrecht, Germany). Biological samples were collected as soon as possible on arrival of the body at the morgue (vitreous humor) and during autopsy (femoral blood, pericardial fluid and urine). During sampling of femoral blood by incision with a scalpel, multiple gas bubbles were visible in the blood ((a)).\nToxicological analysis of the collected samples revealed acetone at a physiological level in the blood, as well as caffeine and paracetamol in both blood and urine. Post-mortem chemistry analysis of the serum samples collected during the autopsy revealed elevated values of C-reactive protein (175 mg/L), consistent with an inflammatory state and a procalcitonine level compatible with a bacterial infection (0.69 μg/L). The results also revealed signs of cardiac dysfunction with a very high level of N-terminal prohormone of brain natriuretic peptide (>35 000 ng/L) and cardiac necrosis with a very high troponin-T level (>56 000 ng/L).\nThe autopsy was performed by one board-certified forensic pathologist and one forensic pathologist in training. Opening of the thoracic cavity revealed numerous gas bubbles in the fatty tissue covering the heart. The pericardium was opened and filled with water, and the heart floated. An incision was made in the right ventricle with a scalpel, and blood with gas bubbles escaped. This technique was performed mainly for training purposes and not for gas sampling, as the gas from the cardiac cavities had already been sampled during CT imaging. The autopsy revealed the presence of numerous gas bubbles throughout the vascular system, even in the small vessels of the brain ((b)). Additionally, changes to the pulmonary parenchyma were consistent with pneumonia. No other major findings were made.\nAn Agilent 6890N GC (Agilent Technologies, Palo Alto, CA) combined with a headspace gas autosampler and equipped with an Agilent Select Permanent Gases column arrangement was used. This column arrangement is specially designed for gas analysis and contains a molecular sieve 5 Å PLOT capillary column (10 m × 0.32 mm i.d.) and a Porabond Q column (50 m × 0.53 mm i.d.) in parallel, which allows for separation of carbon dioxide (CO2). The column temperature was maintained at 45 °C for 13 min. The injector temperature was 100 °C, and the injection was conducted in splitless mode. Helium was used as the carrier gas at a constant flow rate of 8 mL/min. The gas detection and quantification were performed with a thermal conductivity detector set at 150 °C. The system was calibrated for each gas with standard gases of H2S (Multigas, Domdidier, Switzerland), O2 and N2 (from laboratory air), and CH4 and CO2 (Carbagas, Lausanne, Switzerland). With this system, all the gases could be detected in the same run. The gas compositions for the different intracadaver sampling sites are displayed in .\nSamples of the brain, heart, lungs, liver and kidneys were taken during autopsy and stained following a standard haematoxylin and eosin protocol. The heart samples were taken from both ventricles, and the interventricular septum exhibited epicardial vessels deprived of red cells and small intramyocardial haemorrhages. The lung samples taken from each lobe contained numerous clusters of leucocytes, mainly neutrophils, and, to a lesser extent, some macrophages, predominantly in the left lower and right upper lobes. These findings are consistent with the pneumonia suspected on admission to the hospital. There were no significant findings from the other organ samples. Samples of the heart were prepared and analysed for deposition of the plasma antigen fibronectin and the terminal complement complex C5b-9 to look for early cardiac damage, especially the right ventricular ischemia. None of the samples showed reaction for C5b-9, and only small groups of cells stained for fibronectin. Based on these results, cardiac ischemia was not likely in this case.\nIn light of the different results, the cause of death was attributed to a fatal O2 embolism. This was caused by the infusion of pure O2 from the wall into the vascular system through a venous catheter connected to a nasal cannula. Although from a medico–legal point of view the circumstances of the death (accident, suicide and homicide) remained unclear, police investigations led to the suspicion that the patient had connected the nasal cannula to the venous catheter. The wound on the forehead and the bruises observed during the external examination could be explained by a fall from or next to the hospital bed, as suggested by the police. |
The patient is a 14-year-old Caucasian male born full term with no complications. He developed lymphadenopathy at age 3 leading to an excisional biopsy of an axillary node, which showed normal lymph node tissue. His mother denied noticeable persistent lymphadenopathy. At age 9, he developed autoimmune anemia and thrombocytopenia responsive to IVIg and steroids. Lymphadenopathy was not documented at this time. This led to a work up for autoimmune lymphoproliferative syndrome (ALPS). He was found to have normal numbers of double negative alpha/beta T cells with no B220+ DNT cells. He had two subsequent episodes of autoimmune hemolytic anemia. The most recent occurred at age 11 and he was treated with a 4-week course of rituximab. Between episodes, he was asymptomatic and was taking no medications. The patient had no history of significant or severe infections.\nAt age 13, the patient developed back pain, fever, shortness of breath, and cough. He did not improve despite multiple rounds of antibiotics for presumed pneumonia. He was admitted for IV antibiotic treatment. A chest CT showed mediastinal adenopathy and irregular nodular pulmonary infiltrates. A sputum culture was positive for Candida and the patient was treated with antifungals. In total, he received 29 days of amphotericin and micofungin. A bronchoscopy culture was negative for bacteria and fungus. A lung biopsy showed an atypical interstitiital lymphoplasmacytic infiltrate, suggesting an underlying lymphoproliferative process. Bacterial and fungal cultures of the lung were also negative. During his admission he was noted to have IgG < 155 mg/dL (low), IgM 354 mg/dL (high), and IgA 8.7 mg/dL (low). His hypogammaglobinemia was treated with IVIg. He gradually improved and was discharged.\nHe was then referred to our institution for further work up and treatment. Written and informed consent was obtained from the patient and his family members. This study was approved by the Institutional Review Board of the National Institutes of Health. At presentation he reported being in his normal state of health. He was greater than 50th percentile for height and weight. He has no history of arthritis. He reported shortness of breath while playing baseball. His oxygen saturation was 100% at rest but decreased to 92% during a 6-minute walk test. His CT scan showed multiple nodules throughout the lung (Figure ). Of note, many larger lesions seen in prior CT scans had decreased in size or resolved and lesions in new locations were appreciated. Lymphadenopathy and splenomegaly were also noted.\nLaboratory studies showed WBC of 10.36 K/dL, hemoglobin 12.6 g/dL, and platelet count of 386 K/μL. CMV and EBV were not detected by PCR of the blood. The patient did have abnormalities with decreased IgA (5 mg/dL) and IgG (135 mg/dL), elevated IgM (484 mg/dL), and IgE level was <1.0 IU/mL. Analysis of B cells showed normal total (13.4%, 256/μL) and switched memory (CD20+, CD27+, IgM−, 0.3%, 6/μL) B cells, and undetectable unswitched memory B cells (CD20+, CD27+, IgM+). B12 level was 921. At the time of this analysis, he had last received IVIg 3 months prior and rituximab treatment was more than 2 years prior. Laboratory analysis further revealed decreased regulatory T cells with decreased CD25 expression (Figure ). There were variable responses of phosphorylation of STAT (pSTAT) proteins to cytokine stimuli, which did not lead to a clear pattern (Figure ) ().\nTargeted genetic testing using a panel of more than 150 known and predicted immunity-related genes, including CTLA4, LRBA, STAT3, PIK3CD, PIK3R1, FOXP3, AIRE, FAS, FASLG, and CASP10, yielded only one rare variant of interest. A novel, heterozygous missense mutation in STAT3 c.1255G>C, p.G419R was found in the DNA binding domain just a few amino acids away from several other reported STAT3 GOF mutations and confirmed by Sanger sequencing. This variant was predicted to be deleterious by modeling with CADD phred score of 25.3 (). Inheritance is unknown. GOF activity was confirmed using a luciferase assay, as previously reported (Figure ) (). |
A 32-year-old man presented himself at the hospital with a complaint of numbness, causalgia, and paresthesia in the lateral region of his left ankle. Three years ago, his ankle was hit by a steel pipe, and the symptoms developed three months after open reduction and internal fixation of an ankle fracture. Based on the patient's history, traumatic partial peroneal nerve injury was suspected, and conservative treatment was started. Despite the treatment, the pain worsened and the patient received the adhesiolysis of the peroneal nerve a year before his visit to the hospital. However, the pain kept worsening, and spread below his ankle. Radiofrequency thermocoagulation and epidural patient control analgesia were performed without any improvement, so the patient presented himself again at the hospital. Based on the patient's history, he was receiving neuropsychiatric treatment for panic disorder, but he had no particular disease. When he first visited this hospital, he complained of the following subjective symptoms: pain similar to a knife cutting into his left ankle; a hot, burning feeling; an electric shock-like pain that radiated to his calf or the dorsum of his foot; a cold, tingling sensation; and decreased sensation in the regions below his knees. Based on the visual analog scale (VAS), his pain score was 9. Allodynia, hyperalgesia, body temperature asymmetry, change in sweating, and a reduced range of motion were observed in his left ankle. A three-phase bone scan revealed no particular findings, but an electromyography showed neuropathy of his superficial and deep peroneal nerve, and a digital infrared thermographic image showed that the painful site was up to 2.67℃ lower in temperature than the healthy areas of the patient's body. Pregabalin (150 mg), nortriptyline (15 mg), and Ultracet® were administered daily, and a lumbar epidural block, epidurography, and continuous ketamine infusion were performed. As the patient did not appear to respond to these treatments, we decided to try the spinal cord stimulation. A guide needle for an electrode was tunneled through the epidural space between the 1st and 2nd lumbar vertebrae, and the first electrode was placed in the middle of the 9th vertebral body. After the induced paresthesia was confirmed at the painful site, the lead and the extension cables were connected, fixed at the supraspinatus ligament while leaving a margin to avoid tension on the fixing area, and then guided out through the subcutaneous tunnel. After a test period of seven days, the pain decreased by more than 50%, and the dosage of Ultracet® was also decreased from three times to once daily, which allowed for the transplant of a permanent battery. The outpatient follow-up showed the VAS of 2-4, and a decreased dose of pregalin to 150 mg and of nortriptyline to 10 mg. One month after the operation, however, the patient reported a sudden cessation of stimulation, and accordingly, he was advised to visit the hospital. A simple lumbar X-ray was taken, and disconnection of the electrode was suspected (). The spinal cord stimulator was removed and inspected, which confirmed the disconnection (). As the patient wanted re-transplantation of the stimulator after a discussion, the lead was replaced. The patient reported a VAS score of 3 with sufficient electric stimulation and the stimulator has worked normally without particular problems as of now, four months after the operation. |
A 46-year-old male presented in January 2011 with visual impairment in the right eye and without endocrine-related symptoms. MRI revealed a macroadenoma (23 × 21 × 16 mm) that had invaded the right cavernous sinus (Figure ). TSS was performed in January 2011 with a subtotal resection of the tumor, leading to visual improvement. Pathological test results indicated that the Ki-67 index was greater than 3%. In April 2011, the patient again experienced hemianopsia in the right eye, and MRI scan showed that the tumor had recurred with infiltration of the cavernous sinus (22 × 20 × 17 mm) (Figure ). A second surgical intervention was performed to partially resect the tumor using a transcranial approach (Figure ), resulting in visual improvement. Pathological tests revealed that the Ki-67 index was greater than 10% (Figure ). However, five months after the second surgery, MRI revealed regrowth of the residual tumor with invasion of the suprasellar cistern and left cavernous sinuses (Figure ). In January 2012, the patient presented with typical Cushing syndrome, and his ACTH and serum cortisol levels had increased to 119 pg/mL and 49.5 μg/dL, respectively. Tumor recurrence (30 × 25 × 23 mm) was detected by MRI scans in April 2012 (Figure ). In October 2012, a third TSS was performed and the tumor was partially resected (Figure ). Pathology revealed strong ACTH-positive immunostaining in 90% of the cells and a Ki-67 labeling index (LI) of greater than 10%, and weak immunoreactivity for p53 was observed in almost 5% of tumor cells (Figure ). Although the patient underwent fractionated stereotactic irradiation of the residual tumor in December 2012, he began to experience headaches, visual impairment in both eyes, and blepharoptosis in the right eye, and MRI scans in January 2014 revealed that rapid tumor regrowth had occurred with infiltration of the right cavernous sinus (30 × 25 × 27 mm) (Figure ). A fourth subtotal tumor resection surgery was performed in April 2014 using a transcranial approach (Figure ), and resulted a transient improvement in headache and visual impairment symptoms. However, the patient presented with hydrocephalus in May 2014 (Figure ); a ventriculoperitoneal shunt was implanted, which initially improved his hydrocephalus (Figure ). However, the tumor continued to grow rapidly, and the patient died of severe hydrocephalus in October 2014.\nThe clinical characteristics of the remaining refractory pituitary adenomas examined here are presented in Table . Growth rates were determined by calculating the velocity of tumor volume increases using a stereological method based on the Cavalieri principle []. |
A 77-year-old woman with a 1-year history of lower abdominal pain and nausea was admitted to our hospital. Blood examination showed evidence of an inflammatory response (Table ), and abdominal computed tomography revealed a sigmoid tumor with perforation (Fig. ). We suspected sigmoid cancer with perforation, and the patient underwent emergency surgery. Open laparotomy revealed an extensive mass involving the sigmoid colon with surrounding contamination (Fig. ). The abdominal mass was removed en bloc, including resection of the sigmoid colon. The abdomen was flushed to remove contamination. An artificial anus was made.\nHistopathologic examination showed that the oval mass was composed of a diffuse proliferation of plasma cells (Fig. ). At the concavity of the site of the perforation showed the tumor cell infiltrated into the subserosa and necrosis of tissue. But we were unable to identify the site of the perforation pathologically. The surgical margins were free from tumor cells. Immunohistochemical examination revealed positivity for CD79a (Fig. ), immunoglobulin G, and lambda light chain (Fig. , ). Other markers (CD10, CD20, and kappa light chain) were negative (Fig. ). Pathological examination led to a diagnosis of plasmacytoma of the colon. The patient underwent bone marrow biopsy and bone imaging to exclude associated multiple myeloma. Her peripheral blood smear, serum protein electrophoresis, and urine immunoelectrophoresis for Bence-Jones protein were normal.\nPostoperatively, the patient was discharged without any complications. She did not undergo postoperative adjuvant chemotherapy and has had no recurrence in 14 months of regular follow-up.\nExtramedullary plasmacytoma accounts for only 3 to 5% of all plasma cell diseases. These tumors may be solitary or may precede, accompany, or follow the onset of multiple myeloma. Solitary extramedullary plasmacytoma has rarely been reported, and its natural history and diagnosis are unclear. Diagnosis of solitary extramedullary plasmacytoma requires the exclusion of associated multiple myeloma, which is determined by the absence of Bence-Jones protein in the urine, normal serum electrophoresis, and normal bone marrow biopsy []. Our present case met these criteria.\nAlexiou et al. [] reported that extramedullary plasmacytoma most often occurs in the nasopharynx or upper respiratory tract (82.2%). Only 17.8% of cases involve the gastrointestinal tract. The stomach and small intestine are the most commonly involved sites in the gastrointestinal tract. Primary isolated extramedullary plasmacytoma of the colon is extremely rare, occurring in only 0.028% of cases []. Therefore, its clinical features and prognosis are not well known.\nThe clinical presentation of extramedullary plasmacytoma of the colon is variable and may include abdominal pain, intestinal bleeding, and diarrhea. Gabriel and Savu [] reported a rare case in which an extramedullary plasmacytoma was found with ileocecal junction perforation secondary to colonoscopic injury. This is the only previous report to describe extramedullary plasmacytoma with gastrointestinal perforation (Table ).\nIn the present case, we were unable to determine the cause of the perforation by pathologic examination. We consider that the tumor was necrosed and perforated; otherwise, as the tumor grew, the intestinal internal pressure increased, resulting in perforation of the sigmoid colon.\nPostoperative chemotherapy has no effect on the course of extramedullary plasmacytoma. Our patient did not undergo postoperative adjuvant chemotherapy, and she has had no relapse to date. However, careful follow-up is required.\nBecause primary isolated extramedullary plasmacytoma in the colon is very rare, the clinical course, treatment guidelines, and prognosis remain unclear. Further study of the clinical features of primary isolated extramedullary plasmacytoma of the colon is necessary to ensure that adequate treatment is administered. |
A 64-year-old woman with unresectable squamous cell carcinoma of the mid esophagus was treated with definitive chemoradiotherapy, including 4500 cGy of external beam radiotherapy and two cycles of cisplatin and a continuous venous infusion of 5-fluorouracil. Three months later, she was admitted to the hospital with new onset of choking with both solid and liquid foods. Her past medical history included generalized anxiety disorder and hypercholesterolemia. She had an extensive smoking history, but no known history of cardiac disease. A barium esophagram on admission revealed a tracheo-esophageal fistula for which she underwent a successful endoscopic stent placement with a self-expanding metallic stent.\nOne day after the procedure, the patient developed substernal chest pain. Serial electrocardiograms revealed ST elevation in the anterior and lateral leads. Troponin measurements rose to 12.3 ng/ml; serum creatine kinase MB peaked at 10.6 ng/ml. Echocardiography revealed severely reduced global left ventricular systolic function and normal basal systolic function. Regional wall motion abnormalities were noted in the left anterior descending, left circumflex and right coronary artery distributions. The patient developed clinical signs of left-sided heart failure, including acute hypoxemic respiratory failure, and required intubation and mechanical ventilation. An emergency cardiac catheterization revealed normal, patent coronary arteries. A left ventriculogram revealed apical dilation of the left ventricle with akinesis of the whole ventricle except for the anterior and posterior base (Figure ). Cardiology consultants felt that the patient's clinical and echocardiographic features met the diagnostic criteria for takotsubo cardiomyopathy []. The patient was managed conservatively with diuresis and had a rapid clinical improvement; she was extubated after 2 days. She was found to have marked improvement in regional wall motion and left ventricular systolic function on a repeat echocardiogram 6 days later (Figures and ). She had no clinical signs of congestive heart failure during follow-up 2.5 weeks later, making an ischemic or radiation induced irreversible cardiomyopathy unlikely. |
A 45 y/o man with a history of asthma and migraines presented to the neurology clinic in 2000 complaining of a 10-year history of progressive muscle cramps that initially affected his jaws and forearms but subsequently involved his neck and all of four limbs. He also reported a 10-year history of tremors in his upper extremities that also worsened in severity over time. Family history was positive for tremors in his maternal aunt and 2 of his 3 brothers, and cramps in his third brother. Examination at the time of presentation was remarkable for obvious gynecomastia, mild periorbital facial weakness, possible wasting of the sternocleidomastoid muscles, fasciculations in multiple muscles of the face and arms, and a 10 Hz postural tremor in the arms. Of note, mental status, reflexes, strength, sensation and gait were all normal. Curiously, electrodiagnostic testing showed evidence of a generalized sensory neuropathy with absent left sural, median, ulnar and dorsal ulnar cutaneous nerve sensory responses and a slightly reduced superficial radial nerve sensory amplitude and conduction velocity. Electromyo graphy showed evidence of acute denervation in the left lateral gastrocnemius and extensor digitorum brevis and evidence of chronic denervation in multiple muscles of the left upper and lower extremities. DNA testing for Kennedy’s disease confirmed the diagnosis revealing an expansion of 47 CAG repeats (normal being <33). The patient declined treatment for his tremor.\nThe patient continued to be seen in clinic on an annual basis, with slowly progressive disease with increasing difficulty climbing stairs and squatting. He was still able to continue working full time, however. At age 54 the patient reported new hoarseness and mild dysphagia which slowly worsened over the next year. He denied any heartburn or regurgitation. Videofluoroscopic evaluation of his swallowing revealed mild oropharyngeal dysphagia as well as evidence of esophageal reflux, but no laryngeal penetration or frank tracheal aspiration. Follow up swallowing evaluations were recommended every 3 to 6 months. The patient was subsequently seen at age 55 for an annual follow up visit, reporting increased difficulty climbing ladders and stairs. He additionally reported hoarseness after speaking for 5-10 minutes, persistent dysphagia and diffuse cramps.\nPast medical history included mild intermittent asthma; hay fever with rhinitis and conjunctivitis during the summer; tonsillectomy; and migraines. No history of smoking. Medications were sumatriptan and acetaminophen/hydrocodone as needed and fluticasone nasal spray as needed during allergy season.\nOn physical exam he had fasciculations in the hand and tongue and with exertion in the face and platysmus muscles. There was temporal wasting and moderate gynecomastia noted. Voice was coarse, slightly nasal and weak, with moderate bilateral facial weakness present and cranial nerves otherwise intact. Reflexes were absent in the biceps, rest were 2+. Strength was 4/5 in the tongue and hip flexors; 4±/5 in the deltoids, biceps, triceps; rest 5/5, and gait was relatively normal. Body mass index was 24.5 kg/m.\nThe patient returned for an urgent visit, 3 months later, during allergy season. He reported a recent URTI with development of nasal congestion, rhinorrhea, severe post nasal drip, frequent throat clearing, globus pharyngeus, cough, dyspnea and wheezing. He had also experienced several episodes of sudden severe difficulty breathing, with throat tightness and audible stridor, which were very frightening and prompted two visits to the emergency department, where he was sent home after some improvement with prednisone and bronchodilators.\nThe patient was seen 2 weeks later at pulmonary clinic. He was feeling better, dyspnea and wheezing had resolved, but still reported mild residual nasal congestion, globus and frequent throat clearing. He was given a prescription for daily nasal fluticasone spray and cetirizine, with albuterol inhaler as needed. Pulmonary function tests (PFT) were ordered.\nPFT values were within normal limits: forced expiratory volume in 1 second (FEV) 3.66 L (81%), forced vital capacity (FVC) 2.92 L (89%), FEV/FVC ratio 80. Flow volume loop was abnormal, consistent with fixed airway obstruction (). The patient developed laryngospasm with loud stridor and dyspnea during maximal expiratory pressure maneuvers. He was referred to Ears, Nose and Throat Clinic for airway inspection. Flexible fiberoptic nasolaryngoscopy showed no airway lesions or masses. Moderate erythema throughout the glottis and supraglottic larynx was noted, along with diminished abduction of the vocal cords. He was suspected of having laryngopharyngeal reflux and treated with esomeprazole, 40 mg daily, along with behavior modification. He was seen 10 weeks later. Globus pharyngeus and throat clearing were almost gone, and the prior baseline chronic dysphagia had resolved and hoarseness had improved. He was eating more with increased appetite and was very satisfied with the response to therapy. |
A previously healthy 62 years old man who had experienced reduced exercise capacity for the last 6 months was admitted to the local hospital after 2 weeks of increasing dyspnoea. Echocardiography revealed biventricular dilatation, reduced wall thickness, asynchronous left ventricular (LV) contraction and left ventricular ejection fraction (LVEF) of 10%. ECG showed left bundle branch block (QRS width 170 msec). The clinical condition deteriorated rapidly into a cardiogenic shock. Multiorgan failure developed including hepatic dysfunction and renal impairment. The following day, he was transferred to our hospital for LVAD therapy. An Impella LP 2.5® was percutaneously deployed, and the mean arterial pressure immediately improved from 50 mmHg to 70 mmHg and the vasopressor drugs could be stopped. Coronary angiography showed normal coronary arteries. The patient clinically improved and INR and s-creatinine normalized during the first three days.\nAfter five days LVEF was still only 10% and blood pressure could not be sustained without LVAD support. Due to refractory decompensated heart failure and severe asynchronous LV contraction with left bundle branch block, a CRT-D (Medtronic Insync Sentry 7298) was implanted on vital indication. The procedure was complicated by pericardial tamponade not responding to pericardiocentesis. Sternotomy was required to repair a perforation of the right atrium with direct suture. In order to permit prolonged LAVD support and increase pump delivery, Impella LP2.5® was on day 6 after admission replaced through a surgical incision with an Impella LP 5.0® with a maximum flow rate of 5.0/min (Figure ). Ventilator treatment and LVAD support were continued for a total of 22 days. Transient infections were treated with antibiotics. There were no signs of renal impairment, central neurological deficits or mental impairment. The CRT-D was optimized by adjustments of the atrioventricular delay and interventricular timing of pacing guided by echocardiography. At outpatient control after four months the patient was in New York Heart Association (NYHA) functional class IIb with LVEF of 22% and maximal oxygen uptake during exercise was 13.9 ml/kg/min. |
The patient is a 51 year old male who was struck by an automobile at 35 miles per hour while riding a bicycle. There was loss of consciousness in the field and he arrived to our level II trauma center in full spine precautions, as a tier one trauma code. His primary survey was intact and his initial vital signs were; BP 115/80, HR 84, RR 30, O2 saturation 89% on room air which improved to 98% on a non-rebreather mask at 100%. Pertinent findings on secondary survey revealed bilateral chest wall tenderness to palpation, diminished breath sounds bilaterally, upper thoracic spine tenderness to palpation, a complete loss of motor function in his lower extremities, a loss of sensory function below the level of T4 and a Glascow Coma Scale (GCS) of 15. His American Spine Injury Association Motor Score was 50. He also had a loss of his cremasteric reflex, and bulbar cavernous reflex, and had no sacral tone.\nWhile observing strict spine precautions, the patient had chest and pelvis x-rays taken, and was then transported for computed tomography scans of the head, cervical spine and torso. Positive findings from these studies revealed multiple bilateral rib fractures with associated hemothoraces (Figure ). He also sustained fractures and subluxation at the third and fourth thoracic levels (Figure ). The patient was started on spinal dose steroids and strict spine precautions were maintained for anticipated surgical stabilization. Bilateral chest tube thoracostomies were placed for the hemothoraces and a arterial blood gas was then obtained which documented adequate oxygenation and ventilation given this patient's significant pulmonary injury; (pH 7.33 pCO2 42 PaO2 91 HCO3 21, O2 saturation 97 BD-4, 2 liters nasal cannula).\nThe initial drainage from the left chest tube was 500 milliliters (ml) of blood and on his second hospital day it was noted that the chest tube output was 400 ml of milky white fluid suspicious for chyle. Biochemical analysis of the pleural fluid revealed triglycerides of 287 milligrams/decilitre (mg/dL), total protein of 2600 mg/dL, and LDH of 2823 units/L. These results confirmed a diagnosis of chylothorax.\nDue to the complexity of the case, a multidisciplinary team approach was taken to develop the appropriate treatment regimen for this patient. The decision to attempt treatment of the chyle leak with dietary manipulation was agreed upon and the patient was started on a very-low-fat oral diet consisting of mainly fresh fruits, vegetables and whole grains. The patient was also given a semi-elemental formula, Peptamen AF, 1 can with each meal which provided additional kilocalories, protein, and medium chain triglyceride (MCT) oil in order to facilitate wound healing. Two scoops of protein powder (beneprotein) were added to each meal as well. The patient was also started on octreotide, 200 mcg subcutaneous every 8 hours to aid in the reduction of lymph production. The patient tolerated the diet well and these measures led to a dramatic decrease in the chest tube output to less than 100 ml/day of serous fluid by the time he had operative repair and stabilization of his thoracic spine on hospital day seven. After the surgical procedure there was a transient increase in output from the chest tube to 200 ml per day which declined to 35 ml on hospital day 14. The chest tube was then removed without consequence, he was then started on a regular diet and follow up chest x-rays did not reveal any recurrent pleural effusions. The patient was discharged to an inpatient rehabilitation facility and was seen approximately two months after his injury in our clinic. He still had complete motor paralysis of the lower extremities with a T2 sensory loss. His upper extremity function remained unchanged from admission with his motor function intact. His pulmonary status remained stable as he had no ongoing acute pulmonary issues and saturated 98-100% on room air. |
A 46-year-old male patient reported to the Department of Periodontology, Faculty of Dental Sciences, Sri Ramachandra University, Chennai, India with an intraoral swelling in relation to the lower left first and second premolars for the past 8 months associated with a dull pain. The swelling was initially small and gradually increased in size. He was a known asthmatic, but not under any systemic medication. He gave a history of smoking cigarettes (2 packets/day) for the past 30 years and keeping the tobacco quid on the left side of the vestibular area, but had stopped the habit because of burning sensation. On clinical examination, a single sessile erythematous swelling with a fungating whitish mass in the center was seen []. On palpation, it was firm in consistency measuring about 1.5 cm (apicocoronally) and 2.0 cm (mesiodistally) extending from the mesial aspect of tooth 34 to the mesial aspect of tooth 36. Both the premolars exhibited grade 1 mobility with tenderness on vertical percussion and no attachment loss. A single submandibular lymph node was palpable on the left side.\nPatient was counseled regarding the cessation of the smoking habit. Scaling was performed on the first visit and selective grinding of the opposing cusp in occlusion was carried out. At the recall visit, the surface necrosis had disappeared (which could have probably been due to withdrawal of tobacco quid usage).\nComplete hemogram, intraoral periapical radiograph and orthopantomograph were taken. Except for an increase in the eosinophil count, all the other blood parameters were within normal range. No significant alterations were evident in the radiographs []. Based on the clinical findings, a provisional diagnosis of SCC was given.\nAn incisional biopsy of the growth was performed under local anesthesia. The tissue specimen was immediately transferred into 10% buffered formalin solution, sent for routine histopathological examination and were further subjected to immunohistochemical (IHC) analysis.\nHistologically, the tumor showed an ulcerated parakeratotic stratified squamous epithelium [] with a focus of epithelial pearl formation []. The underlying connective tissue stroma revealed richly cellular pleomorphic spindle cells arranged in fascicles resembling a herring bone pattern characteristic of a fibrosarcoma []. The sections were further subjected to IHC for cytokeratin, vimentin and CD34 expression. IHC analysis revealed spindle shaped cells that showed positive reactions for cytokeratin [] and vimentin [] but negative for CD34 []. Antibody to podoplanin, a molecule expressed in lymphatic endothelial cells was used. Positive membrane immunoreactivity was observed in the basal cell layer and connective tissue stroma with a relatively increased lymphatic network complexity as compared with the tumor free zone [].\nA whole body plain and contrast computed tomography (CT) view was performed. CT view of mandible with contrast revealed a 17.8 mm × 4.7 mm sized soft-tissue density lesion in the buccal aspect of the body of the mandible on the left side with no evidence of bony erosion []. Few sub centimeter sized lymph nodes were present in the left submandibular [] and bilateral deep cervical (level II) regions [].\nPatient was referred to an oncologist. Following chemotherapy, the patient was subjected to surgical management that involved a segmental resection of the mandible with supraomohyoid neck dissection followed by radiation therapy. |
A 23-year-old man with a healthy appearance (170 cm, 57 kg) was referred to our clinic for persistent bacteriuria unresolved with prolonged use of antibiotics. He was symptom-free and unremarkable on the physical examination at his 1st visit to our clinic. He had a history of intermittent pain in the right lower abdomen with recurrent UTI for 15 years and had been treated with antibiotics repeatedly. He had a history of hospital admission 10 years ago but he could not hear any underlying cause identified. He had not suffered any other medical or surgical disease. Laboratory tests were normal except for pyuria and growth of Escherichia coli in his urinary samples. Cystoscopy was performed and a small slit-like opening was found on the right lateral wall of the bladder dome without a definite fistulous tract (). Voiding cystography revealed a diverticulum on the right lateral wall of the bladder with a suspicious linear fistula tract heading in the 10 o'clock direction (). We found some air within the bladder and a suspicious communicating tract between the appendix and bladder on the CT scan.\nWith an impression of appendicovesical fistula, laparoscopic surgery was performed through three abdominal ports. A supraumbilical port (10 mm trocar), a suprapubic port (5 mm trocar), and a right upper quadrant port (12 mm trocar) were placed. The distal tip of the appendix was firmly adhered to the right lateral side of the bladder with thick fibrosis. The body and base of the appendix looked healthy without surrounding fibrosis (). The base of the appendix was ligated with 2-0 Vicryl and the bladder was resected with an Endo-GIA stapler (Ethicon Endo-Surgery, Cincinnati, OH, USA) under simultaneous intravesical monitoring of the bladder with a flexible cystoscope. A urethral catheter was inserted for urinary drainage and was removed 7 days after the surgery. The postoperative course was uneventful. The surgical specimen showed a 7.5×1.0 cm sized appendix attached to a 3.0×2.5 cm sized bladder wall, and a small caliber fistulous tract with some fecalith was identified between them. |
Our patient is a male infant born via cesarean section at 29 weeks’ gestational age to a 24-year-old mother for worsening preeclampsia. The infant’s birth weight was 995 g (20th percentile) and head circumference was 25 cm (10th percentile). The initial physical examination revealed a 1 cm × 1 cm neural tube defect in the lumbosacral area consistent with a myelomeningocele that was not detected prenatally. He underwent primary closure of the defect at 1 week of life. The initial and subsequent head ultrasounds did not identify hydrocephalous, intraventricular hemorrhage, or Chiari II malformation. The head circumference grew along the 10th percentile throughout his hospital course. He had normal, symmetric movement of his 4 extremities with increased tone and normal urination and stooling patterns. He had no sensory deficits noted on the dermatomal evaluation of the lower extremities, especially in the sacral region, before or after repair. He passed the auditory brain stem response-hearing screen and had no retinopathy of prematurity.\nThe infant had poor weight gain (<15 g/d from birth to day of life 100), extreme irritability, hypertonicity, and significant feeding intolerance (vomiting and arching with feeds) that was out of proportion for prematurity. At 42 weeks postmenstrual age, he was still requiring feedings via a nasogastric tube. The diagnostic workup for his gastrointestinal and neurological manifestations of pain and irritability, including brain and spine magnetic resonance imaging, and modified barium/follow through speech evaluation did not reveal any pathology.\nAt 43 weeks postmenstrual age, with no identifiable source for the irritability and feeding intolerance, manifested as poor oral intake and lack of weight gain, a diagnosis of visceral hyperalgesia was considered and the infant was trialed on gabapentin. The dose was titrated to effect by administering gabapentin at 5 mg/kg/d by mouth at night on days 1 and 2, 10 mg/kg/d divided twice daily on days 3 and 4, and 15 mg/kg/d divided 3 times daily from day 5 onward. The regimen was adopted from the previous literature, and gabapentin was initially administered via the nasogastric tube. His nasogastric tube was removed after 3 days of starting the medicine, at which time the medicine was switched to oral administration, and he gained an average of 28 g/d until his discharge at 46 weeks postmenstrual age. His tone improved and irritability dissipated. He was discharged home on gabapentin, and the dose and interval were slowly weaned over 3 months by his pediatric neurologist. Adverse clinical events associated with initiation, maintenance, and discontinuation of gabapentin were closely monitored but never experienced by this patient (). |
A Japanese man, who had been followed since 53 years of age by the respiratory department of our Medical Center due to bronchial asthma, was hospitalized for progression of asthma at 63 years old. A suspected diagnosis of HCC in his right liver lobe was confirmed by abdominal computed tomography (CT). An extended right lobectomy was performed after three months in our hospital. Pathologic examination revealed an HCC, composed of a necrotic tumor that measured 10.5 × 9 × 11 cm. In addition, there were three daughter nodules with diameters of less than 1 cm each. The residual nodular tumor without necrosis was Edmondson grade II to III with nuclear atypia, and was moderately differentiated. Invasion of lymphatic and vascular channels was not obvious. Exposure to the surface of the liver capsule was not found. A single lung metastasis and a single mediastinal lymph node metastasis were found in a chest CT scan performed 18 months after the liver lobectomy (Figures and ). Final diagnosis was made based on the elevated levels of two tumor markers for HCC: α-fetoprotein (AFP) (4869 ng/mL) and protein induced by vitamin K absence or antagonists II (PIVKA-II) (>20,000 mAU/mL). Trans-catheter arterial embolization for the mediastinal tumor was attempted, however the risk of spinal artery embolism resulted in the decision to only examine the tumor at that time.\nOur patient was admitted to our Department of Radiation Oncology for irradiation. His Karnofsky performance status score was 90% to 100%. He complained of a moderate cough and moderate bloody sputum but denied any dyspnea or chest pain. To relieve these clinical symptoms, external-beam irradiation, with a dose of 2.25 Gy per fraction, was performed using an antero-posterior parallel-opposed technique (total dose, 60.75 Gy) (Figures and ). The energy was 10 megavolts. The radiation field was set for gross tumor volume plus a margin of 1 cm. The field size was 8 × 10 cm. Radiation therapy was given four times a week. After 40 Gy, the radiation field was changed to left-right parallel-opposed beams to spare the spinal cord (Figure ). The lung metastasis that had induced no clinical symptoms was not treated with radiation therapy, and was located outside the radiation field of the left-right opposing beams (Figure ). A CT scan was performed after the radiation therapy, which showed a remarkable reduction in the mediastinal lymph node. Additionally, shrinking of the tumor in his right lower lobe, outside of the radiation field, was observed (Figures and ). No chemotherapy had been given during this period.\nDuring follow-up as an out-patient, our patient was observed to have dyspnea (Hugh-Jones 1-2), slight cough and slight sputum. AFP levels had decreased to 23 ng/mL, and PIVKA-II to 13 mAU/mL.\nA CT scan performed four years after the radiation therapy showed a lymph node swelling with a diameter of 3.5 cm in the area of origin of the left gastric artery. At this time, AFP and PIVKA-II were elevated to 1990 ng/mL and 1990 mAU/mL respectively, but with no pathologic evidence of recurrence. Stereotactic body radiotherapy for the lesion was performed, with 30 Gy in three fractions. The recurrent tumor disappeared. Six and a half months after the stereotactic body radiotherapy, no obvious recurrent disease was found. |
A 65-year-old male patient was admitted to the neurological care unit because of presyncope suspicious for a cerebrovascular ischemic accident. During the monitoring phase the patient developed a rapid sustained ventricular tachycardia mimicking again neurological symptoms. Structural heart disease was present in form of ischemic cardiomyopathy with an ejection fraction of 0.40 and a scar in the inferior region. There was a history of coronary artery bypass revascularisation 14 years ago, but no signs of acute coronary syndrome. Recent coronary angiography demonstrated severe three vessel disease with a patent arterial graft to the left anterior descending artery, an open vein graft to the circumflex artery, an occluded right coronary artery with an occluded single vein graft, but no need for further revascularization. A week before hospital admission the patient underwent surgery for rupture of the left sided tendon of the subscapularis muscle and a surgical revision due to wound hematoma.\nFor secondary prevention of ventricular tachyarrhythmias a dual-chamber ICD (St. Jude Medical Current DR RF, 2207-36; atrial lead SJM 1888TC/52; ventricular lead SJM Durata 7170) was implanted from the right side without any problems. The operation was performed in the usual way. The greater pectoral muscle was divided between its subclavian and thoracic part in order to form a pocket beyond the pectoralis muscle. The ventricular and atrial leads were introduced after direct puncture of the subclavian vein and adequate sensing, pacing and impedance values were confirmed (R wave amplitude 5.7 mV, threshold 0.9 V at 0.5 ms, impedance 872 Ohm; P wave amplitude 3.9 mV, threshold 0.8 V at 0.5 ms, impedance 764 Ohm). The leads were secured with non-absorbable double sutures around the sleeves. The wound was closed after placement of the ICD generator into the subpectoral pocket. Defibrillation testing was performed successfully with a single 17 Joule shock. At prehospital discharge control the patient claimed about intermittent stimulation of the diaphragm. Testing of ventricular threshold revealed an increase to 3.0 V at 0.5 ms while impedance and sensing values remained normal (). The pacing mode was changed to AAI stimulation to prevent the unpleasant diaphragmatic stimulation. Within the first three months after implantation the patient received a total of 8 adequate antitachycardia pacing therapies and 3 defibrillator discharges at maximum output for treatment of ventricular fibrillation. Despite the life saving effect the patient was worried by the painful therapies of the ICD.\nAt 3-months follow-up the patient demonstrated a further increase of the ventricular stimulation threshold up to 5.0 V and a complete loss of capture of the atrial lead with diminished atrial amplitude during sensing (). Interestingly, no changes were noted with respect to the impedance values of the atrial, ventricular and high-voltage shock lead (). The local aspect of the ICD pocket was unremarkable. A chest X-ray revealed retraction of the atrial lead up to the superior caval vein and extreme coiling of the leads near the header (). The patient underwent a complete revision of the ICD system. Upon opening the pocket the surgeon found an atrophy of the pectoralis muscle and heavily tangled leads without isolation defect (). After uncoiling the leads the ventricular stimulation threshold was within the normal range again. However, both atrial and ventricular leads were extracted and replaced by new ones for safety reasons. Before closing the pocket the generator was secured with a suture to the pectoralis muscle. |
A 12-year-old boy with progressive trunk deformity and a huge subcutaneous tumor was referred to our department. At the age of three, the subcutaneous tumor was found in the back, and biopsy of the tumor indicated a diagnosis of cavernous hemangioma. During that biopsy, a massive hemorrhage occurred, and the patient required a blood transfusion (3000 ml). Scoliosis was diagnosed at age 11, and the patient underwent brace treatment. However, the scoliosis worsened progressively, and the patient was finally referred to our department. Upon physical examination, we found significant protrusion of the right back ribs. On the left back was a huge subcutaneous tumor, measuring approximately 15 cm in diameter, and a scar from a previous surgical wound that was approximately 5 cm long (Figure ). The mass was not tender or throbbing, and no neurological abnormalities were observed.\nFull-length, standing radiographs demonstrated a scoliosis of 85° at T6-L1 and a kyphosis of 58° at T4-T10. The Risser sign was grade zero, and the triradiate cartilages were open (Figure ). Traction radiography showed a correction rate of 6%, indicating extremely low flexibility. On the axial CT images, vertebral body atrophy was recognized at T7-T10, where the vertebral bodies were surrounded by the cavernous hemangioma (Figure ). No congenital deformity of the vertebral bodies was observed. MR images revealed that the hemangioma extended from the subcutaneous region to the paraspinal muscles and the retroperitoneal space at T6-L1, primarily on the left side. Invasion of the hemangioma into the spinal canal and compression of the dura by the hemangioma were recognized at the T8-T10 level (Figure ).\nThe diagnosis of severe progressive scoliosis associated with cavernous hemangioma was made. Prior to the surgery, we decided against the use of radiotherapy, which may reduce the size of the tumor or blood circulation through it, because irradiation of the spine can lead to pseudoarthrosis. In planning the surgery, an anterior approach was considered first, since the hemangioma was mainly located on the concave side of the curve. However, we abandoned this idea because damage to the hemangioma during disc removal or screw placement would have required us to manage the hemorrhage from inside the narrow thoracic cage, which might not have been successful. Although bleeding from hemangioma was inevitable if we used the posterior approach, we had the option of stopping the bleeding by the tamponade effect of closing the wound. Therefore, we used the posterior approach for correction surgery and fusion with pedicle screws at T2-L3.\nA midline skin incision was made on the patient's back. Soon after the incision was made, the hemangioma hemorrhaged massively, and hemostasis by coagulation failed using bipolar forceps, electrocautery, and ligation. Thus, the surgery was continued with gauze packing at the bleeding sites. The soft tissues were carefully detached from the spinous processes or laminae subperiosteally using a spatula to minimize damage to the pseudocapsule of the hemangioma, and consequently, the hemorrhage greatly decreased. Pedicle screws were placed segmentaly, except from T8 to T10, where the hemangioma invaded the spinal canal, to avoid spinal cord injury from intracanalar hemorrhage of the hemangioma. Since we encountered massive hemorrhage at T11, 12 and L1, we could not place pedicle screws at these levels, where we had hoped to place them to increase the number of anchor points. The correction force was applied carefully to avoid screw failure, since fewer screws could be placed than were needed and the flexibility of the curves was quite low. After the correction, abundant bone grafting was performed using the iliac crest and local bone. No drainage tube was placed inside the wound, since it could have increased the risk of postoperative hemorrhage by penetrating or damaging the surrounding hemangioma (figure ) during insertion of the tube. After surgery, the scoliosis was corrected to 59°, and kyphosis to 45°, with correction rates of 31% and 22%, respectively (Figure ). The intraoperative time was 314 minutes, and the intraoperative blood loss was 2800 ml.\nThe patient's hemodynamics stabilized with blood hemoglobin level of 9.2 mg/dl when the surgery finished at 7 pm after the transfusion of 400 ml of preoperative donated autologous blood and 1350 ml of intraoperative and postoperative cell saver autologous blood. The patient was admitted to intensive care unit at 8 pm. At 11 pm (four hours after surgery), his blood pressure was 92/52 mmHg with stable hemodynamics, and his blood hemoglobin level was 8.5 mg/dl. However, seven hours after surgery, after the administration of diazepam to control agitation, his blood pressure suddenly decreased to an unmeasurable level, and the patient became unconscious with apnea. At that time, the hemoglobin level was 5.9 mg/dl. Endotracheal intubation was immediately performed, along with blood transfusion, which resulted in his recovery from hypovolemic shock. The following day, however, the patient developed disseminated intravascular coagulation (fibrinogen degradation products [FDP] 32.3 μg/ml, platelet count 95,000/μl, prothrombin time international normalized ratio [PT-INR] 2.28), and received fresh frozen plasma and gabexate mesylate. Seven days after the surgery, when his general condition had stabilized, the patient was extubated. However, as the patient became conscious, a language disorder became apparent, and a head MRI revealed ischemic lesions in the bilateral frontal and temporal lobes. Sensory and conduction aphasia was diagnosed, caused by cerebral hypoxia during the hypovolemic shock on the day of the surgery (Figure ). The patient gradually recovered from the aphasia, and the ischemic lesions became smaller on MRI (Figure ). At present, two years after the surgery, although the patient has completely recovered from the aphasia. |
A 14-year-old boy presented with double vision following an assault by a friend 10 days before presenting to our clinic. Further questioning revealed that his friend had hit him in his right eye with a pen. The patient denied any significant trauma to the eye. When examined, the patient had a left face turn. The visual acuity was 20/20 in both eyes. There were no signs of injury in the external right eyelid []. However, there was a conjunctival tear inferolaterally. There was a firm mass palpable along the inferotemporal orbital fossa between the globe and the orbital rim. There was less than 15 degrees of hypotropia on Hirschberg's test, while prism bar cover test revealed hypotropia of 20-prism diopter in the right eye []. Extraocular movements were restricted in upgaze as well as downgaze. Diplopia charting showed vertical diplopia in primary gaze, upgaze and downgaze. Hess chart findings were suggestive of maximum underaction in the area of the right inferior oblique as well as underaction in the area of the right inferior rectus, with overaction in the left eye. Force duction test in the right eye revealed restriction of elevation and depression. Anterior segment and fundus examination was normal with a brisk pupillary reaction.\nA computed tomography (CT) scan showed a well-defined tubular translucent object which was indenting the sclera in the right eye inferolaterally and passed inferomedially and posteriorly. There was a defect of the floor of the right orbit with the foreign body extending inferiorly into the maxillary antrum across the fracture site [].\nExploration of the right orbit via trans-conjunctival approach was planned. After making an incision in the conjunctiva, a solid structure was appreciated. This was a plastic transparent tip of a ball point pen []. It was removed without damaging the inferior oblique or inferior rectus muscle fibers. The tip of the pen entered the maxillary sinus posteriorly, where a circumscribed defect was noticed in the orbital floor. As there was no entrapment at the fracture site, no plating was done. The patient was given systemic oral steroids (prednisolone acetate; 1 mg/kg body weight in tapering dose) postoperatively for one month.\nOn one month follow-up there was small residual hypotropia. However, he was able to fuse and therefore had no complaint of diplopia in primary gaze []. |
The patient is a 63-year-old Hispanic woman with a history of peptic ulcer disease with dyspepsia and was treated with a proton pump inhibitor (PPI). She was diagnosed with Helicobacter pylori infection and received a triple drug therapy. Subsequently she experienced weight loss of approximately 14 kg over 2 months (BMI = 18.4; previous weight 64 kg, BMI = 23.5), and her dyspepsia did not improve. Colonoscopy and esophagogastroduodenoscopy (EGD) were performed and revealed a 4 cm pyloric ulcer and 70% pyloric stenosis; biopsies were taken at the time of the EGD. The biopsy demonstrated adenocarcinoma in signet ring cells. She was referred to the General Ignacio Zaragoza Regional Hospital Surgery Department for further evaluation. Social history was significant for an 18-pack-year smoking history. Her family history was significant for first-degree relatives with a history of cancer: one with bladder carcinoma and the other with prostate carcinoma. Her physical examination was within normal limits. Laboratory tests demonstrated normal hemoglobin and hematocrit levels, normal liver enzymes, and normal renal function; however, urinalysis was significant for both proteinuria and hematuria, and her blood type is A positive. Carbohydrate antigen (CA)125 and CA19-9 were normal. Her chest X-ray was normal for her age. A computed tomography (CT) scan of her abdomen and pelvis showed a tumor in her left kidney of approximately 140 × 97 × 85 mm with homogeneous enhancement in its upper pole. No distant metastases were evident. Surgical intervention with an exploratory laparotomy was undertaken, where a partial gastrectomy was performed with gastrojejunal anastomosis with omega of Braun reconstruction and left radical nephrectomy. Interoperative cryosections were evaluated by pathology. She had an estimated blood loss of 850 cc, with an estimated surgical time of 210 minutes. The postsurgical course was without complications. The histopathological report describes the left kidney with a size of 100 x 80 mm with a tumor in the upper and middle pole of 70 x 50 mm. The tumor type was clear cell renal cell carcinoma with a histological grade II on the Fuhrman scale with extension beyond Gerota’s capsule. The clinical stage was T4 N0 M0. The gastric tumor with dimensions of 10 × 10 mm in the antrum and pylorus showed diffuse type adenocarcinoma with signet ring cells with little differentiated histological grade and pathological stage T2 N0 M0. The patient is currently under active surveillance with monthly monitoring by the departments of surgery and medical oncology; to date her CT scan and laboratory tests demonstrate no evidence of relapse or distant metastases. |
A 30-year-old male patient presented to our emergency with bomb blast injury to his right eye. The incidence took place at a countryside liquor shop which allegedly housed a crude bomb-making unit. The spoons were being used to fill in the shell with explosive mixtures.\nVision at presentation was reduced to light perception. Emergency room examination revealed a pulse rate of 68 per min, blood pressure 100/60 mm of Hg with Glassgow Coma Scale score of 13. A deep laceration was noted over his right temple which extended to the right upper eye lid and globe. Careful examination showed some metallic plate-like object impacted in his right eye from the temporal side []. The lids were intact except at the temporal margin. Attempted ocular examination after prying the lids apart revealed deformed eyeball with metallic plate of the spoon securely fixed in the orbit with its handle in the laceration line at the temple. A sclerocorneal laceration was noted which extended from the temporal limbus to the medial canthus through the pupillary axis. There was extrusion of the ocular contents. An X-ray of the skull and orbit was obtained. The radiograph [] revealed that the handle of the steel spoon was lying deep in the laceration line on his temple with its plate entering into the globe through the right upper eye lid. The plate of the spoon was angulated at its junction with the handle. There was no fracture of the bony architecture along the course of soft tissue injury. The patient gave information that something had hit him from his right side at the time of explosion. After proper resuscitation with intravenous fluids and cross-matched blood transfusion, assessment of possible damage was done. The spoon was removed from his temple and orbit under general anesthesia the next morning. Sclerocorneal laceration was repaired with 6-0 Vicryl and 10-0 monofilament nylon. Upper lid laceration was repaired with the 6-0 Vicryl sutures after removal of residual infected ocular tissue. Patient was maintained on intravenous ceftriaxone (1 g every 12 h) and intravenous gentamicin (80 mg every 12 h) systemically along with topical fortified cefazolin (50 mg/ml) and fortified tobramycin (14 mg/ml). At one-week follow-up patient had visual acuity of hand movement close to face. Ultrasonography B scan at one week revealed vitreous hemorrhage and total retinal detachment. Pars plana vitrectomy with retinal reattachment was contemplated, however, poor wound integrity led us to postpone the surgery to a later date. Resolving vitreous hemorrhage was noted at next follow-up visit at one month. Visual acuity persisted at hand movement close to face mainly due to corneal opacity which precluded fundoscopy. |
A 74-year-old male patient underwent left total knee arthroplasty (TKA) at our institution for degenerative knee osteoarthrosis. He had an uneventful postoperative course with no history of delayed wound healing or persistent drainage. Four years later, he presented with a 2-month history of a gradually developing painless swelling over the anterior aspect of the operated knee; the swelling was associated with a small sinus that was extruding a straw-coloured fluid. He had no history of fever, decreased appetite, or weight loss. He had no other musculoskeletal, respiratory, or systemic symptoms of note. He had no history of antecedent trauma, recent travel, or contact with infectious diseases. The patient is a known hypertensive, but the blood pressure was well controlled with treatment, and he is otherwise healthy. He is a retired teacher with no history of involvement in activities requiring excessive kneeling. He is ambulatory in his community and can walk comfortably with the assistance of a cane.\nThe patient's general physical examination results were within normal limits; positive physical findings were limited to the involved knee. There was an anterior knee swelling involving mainly the prepatellar area, approximately 7 cm in diameter, fluctuant, and not tender to palpation, with minimal surrounding erythema; the erythema was present mainly at the punctum. The punctum was draining a yellowish discharge on pressure (). There was no bony tenderness at the patella, distal femur, or proximal tibia. There was no detectable knee effusion, instability, or crepitus. The range of motion was well preserved (5–110°), as it was a prosthetic knee. It was only painful at the end of flexion as this movement compressed the prepatellar bursa.\nPlain radiographs of the knee showed a prepatellar soft tissue swelling (). There were no obvious bony changes, osteolysis, or loosening at the bone-prosthesis interface. Needle aspiration of the prepatellar bursa yielded 50 mL of slightly turbid straw-coloured yellowish fluid that was sent as per our protocol for cell count determination, microcrystal analysis, Gram staining, and Ziehl–Neelsen (ZN) staining for acid-fast bacilli (). Cultures for aerobic and anaerobic bacteria, Mycobacterium tuberculosis, Brucella, and fungi were requested.\nConsidering the patient's age, chronic presentation, and the presence of sinus, he was admitted with a diagnosis of infected prepatellar bursitis. The primary aims of admission were wound dressing, awaiting aspiration results, and further work-up. Aspiration results revealed normal cell counts, no crystals, and no organisms on Gram stain. To our surprise, ZN stain revealed acid-fast bacilli consistent with typical tuberculous infection; the bacterium was confirmed on culture 6 weeks later as Mycobacterium tuberculosis-sensitive to rifampicin, isoniazid, ethambutol, and streptomycin. Cultures for bacteria, Brucella, and fungi were negative. Aspiration was repeated and yielded similar results. The blood work-up showed normal total and differential white blood cell count and slight elevation of both erythrocyte sedimentation rate (ESR) (80 mm/hr; normal: 30–70 mm/hr) and C-reactive protein (CRP) (8 mg/L; normal: 0–4 mg/L). Tuberculin skin test revealed a negative result (<5 mm induration at 72 hrs), and further work-up including chest radiograph and echocardiogram revealed no evidence of systemic disease. A triple three-phase bone scan displayed normal uptake both at the bone-prosthesis interface and at the patella. An infectious disease consult was obtained. The patient was started on rifampicin (600 mg/day), isoniazid (300 mg/day), pyrazinamide (1500 mg/day), and ethambutol (800 mg/day) for 2 months and continued on the same dosage of rifampicin and isoniazid to complete a 6-month course.\nThree weeks later, the swelling significantly subsided in size and the sinus healed; therefore, the patient was discharged. He was reviewed at 6-week intervals at both the orthopaedic and infectious disease clinics for clinical progression and for any side effects of the medication. At the most recent 6-year follow-up, he was doing well with no evidence of local recurrence or prosthetic loosening (). |
An 11-year-old girl, born of full-term pregnancy which was complicated by prolonged labor, who had delayed speech, impairment in domains of communication, self-care, work, academic, interpersonal and social skills since early childhood presented with a history characterized by difficulties with sustained attention, distraction, poor compliance with instruction, loosing things, jumping and hopping around, not waiting for her turn during play, and interrupting parents when they would be interacting, since the age of 4 years. These symptoms increased over the years and were thought to be more than the appropriate mental age and led to marked dysfunction in social and school functioning. At the age of 6 years, she was diagnosed as having ADHD, combined type (as per DSM-IV), with moderate mental retardation (intelligence quotient of 46) at a tertiary care center and the parents were advised to follow the behavioral measures. There was no family or personal history suggestive of head injury, epilepsy, psychosis, affective disorders or substance abuse.\nAt the index assessment at the age of 11 years, the child fulfilled the diagnosis of ADHD, combined type (as per DSM-IV), with moderate mental retardation. Her body weight was 26 kg and physical examination did not reveal any abnormality. On investigation, her magnetic resonance imaging of brain, electroencephalogram, hemogram, serum electrolytes, renal function and liver function tests were found to be within normal limits. In view of the marked psychosocial dysfunction and failure to respond to behavioral measures in the past, she was prescribed methylphenidate 10 mg/day, which she tolerated well. After 1 week, the dose of methylphenidate was increased to 15 mg/day in divided doses. However, on the 4th day of increase in dose, she was found to be restless (more than the past) with constant fidgeting, started running around, shouting, screaming and would bite family members if any attempt was made to calm her down. She spoke loudly at faster pace, hummed tunes and demanded for various food items and toys. However, she was able to recognize the parents and was aware of place and there was no history of abnormal involuntary movements. Also, she did not sleep throughout the night. Next day she was brought to the psychiatry OPD in a state of extreme agitation, running all over the places, opening tap, with socially disinhibited behavior. It was difficult to control her. There was no history of grandiosity, delusions or hallucinations. In view of the extreme agitation, she was admitted, methylphenidate was stopped and she was given Inj. lorazepam to control agitation. Her investigations were repeated including electroencephalogram and computerized tomography of brain, which were normal. For the initial 3 days, lorazepam was given in the dose of 4-8 mg/day intravenously, but there was no improvement in her symptoms. Following this, she was started on sodium valproate 600 mg/day in two divided doses along with olanzapine 5 mg/day. With these medications, by the 6th day of admission, her symptoms started reducing and by the 9th day, she improved significantly and was discharged on the 10th day when she could interact in a meaningful way. Within a period of 1 month, both olanzapine and sodium valproate were withdrawn and the patient has been maintaining well. |
An 85-year-old Caucasian man was admitted to hospital following three collapse episodes with transient loss of consciousness at home. Each episode was short-lived lasting several minutes. Apart from mild abdominal generalized discomfort, there were no other symptoms. There was no history of recent trauma. He had no history of similar episodes but was known to have severe aortic stenosis, type 2 diabetes, paroxysmal atrial fibrillation, hypertension and a previous duodenal ulcer bleed.\nAt that time, he was taking aspirin, bisoprolol, omeprazole and ramipril. He lived with his daughter and was independent with his activities of daily living. He had not smoked for 35 years and his alcohol consumption was minimal.\nOn examination, he was apyrexial, oxygen saturation was 100% on air. His blood pressure was 80/40 mmHg. He was persistently hypotensive despite aggressive fluid resuscitation. There was an ejection systolic murmur on cardiac auscultation. His venous pressure was not elevated and there was no leg edema. The lungs were clear on auscultation. Upper and lower limb pulses were equal bilaterally. Examination of his abdomen revealed mild epigastric discomfort, but there was no rebound or peritonism and bowel sounds were present. Per rectal examination was normal.\nInitial blood results showed a hemoglobin of 11.3 (13-18 g/dL), white cell count of 11 (4-11 × 109/L), and platelets of 136 (150-450 × 109/L). Coagulation profile, renal function and liver function tests were within normal limits. His chest radiograph was normal and his electrocardiogram showed left ventricular hypertrophy. At admission, he was taken to the coronary care unit for cardiac monitoring because of the history of collapse with loss of consciousness which was thought to be related to his aortic stenosis. An urgent echocardiogram was performed which showed evidence of aortic stenosis, but no evidence of critical stenosis with good ejection fraction > 55% and good biventricular contraction.\nA repeat full blood count showed that his hemoglobin had fallen to 4.9 g/dL and he was transfused with red cells, platelets and cryoprecipitate. The impression was that this patient had a possible dissecting thoracic aneurysm that was possibly extending into the abdomen. He was transferred to the intensive care unit.\nIn view of the differential diagnosis of a possible dissection, an urgent chest and abdomen CT scan was performed which showed normal appearances of the thoracic and abdominal aorta with no evidence of aneurysm or dissection. However, the scan revealed a large amount of free intra-peritoneal fluid with areas of low attenuation in the right lobe of the liver. The appearances were concluded to be of metastatic disease within the liver (Figures , , . No primary tumor was identified. A diagnostic peritoneal tap was performed and frank blood was aspirated confirming that there was hemoperitoneum. An acute intra-abdominal bleed from the liver metastatic disease was diagnosed.\nOur patient had an esophageal gastro-duodenal endoscopy as he had been taking aspirin and had a past history of a duodenal ulcer. This did not show any evidence of bleeding. A rigid sigmoidoscopy was also normal.\nWhilst on the intensive care ward, our patient's blood pressure subsequently improved and he did not require inotropic support. He had no further hypotensive episodes and improved during his stay on the ward. Given the advanced nature of his hepatic metastases, he did not wish to have further investigations to identify the primary source of the metastases and decided on conservative supportive treatment, as advised by the oncologists. He was referred to the Macmillan and Hospital Palliative Care Team. Subsequently, he died three months later. A post-mortem was not performed. |
A 28-year-old man was admitted to our hospital because of a motorcycle trauma involving his right upper limb. Three years earlier, the patient had reported a type-1 floating elbow injury to the same limb from a motorcycle accident. The former lesion was treated at a different hospital by ORIF of both humerus and forearm fractures. In details, the comminuted intercondylar fracture was treated by two reconstructive plates on the medial and lateral columns of the distal humerus, along with interfragmentary screw fixation. The radial shaft fracture was treated by a six-hole plate with screws, whereas a tension band wiring was performed to stabilize the ulnar fracture. Radial head resection was also carried out (Fig. ). Following this procedure, the patient obtained a painless elbow although the range of motion (i.e., 40° of extension, 90° of flexion, and 40° of forearm’s pronation/supination) and limb strength were reduced.\nThe patient refused further operations to improve articular range of motion, thus living with this function for 3 years prior to the second high-energy trauma to the elbow.\nWhen the patient was admitted at our emergency room, the physical examination showed bruising, severe soft tissue swelling, and gross deformity of the right elbow and forearm. He complained of tingling in his forearm and inability to carry out active movement of his right hand. The neurological examination showed severe tactile hypoesthesia and paresis of the muscles in the radial and ulnar nerve territories. No anomalies in the arterial pulses were detected. The patient also reported facial soft tissue injury and non-nasal midfacial fractures. The radiographic examination showed a supracondylar fracture of the humerus and midshaft fractures of the radius and ulna. Thus, according to Simpson and Jupiter [], a type-3 floating elbow injury was diagnosed. In detail, a fracture of the humerus close to the most proximal screw occurred, the ulna had a fracture distally to the former fracture with an intermediate third fragment, while the radius sustained a fracture at the site of the most proximal screw (Fig. a, b). A CT scan (Aquilion 64-slice CT, Toshiba Corporation, Japan) showed integrity of the intercondylar bone, with the implants of the former operation stable (Fig. c).\nThe treatment of fractures was delayed by 3 days because of tissue swelling. A dissection of an inverted V-shaped flap of the triceps aponeurosis that was then reflected distally was the approach used for the reconstruction of the fractured distal humerus. The ulnar nerve was therefore mobilized and allowed to remain in its normal anatomical position, but it was carefully protected until the end of surgical procedure. The radial nerve was also dissected and fully mobilized (Fig. ) to avoid its improper stretching caused by muscle spreading at the time of plate positioning. The radial and the ulnar nerves were intact, and no signs of compression by the fracture fragments were noted. A dorsolateral approach to the radius and a lateral approach to the ulna were chosen for the surgical exposure of the forearm. All previous metal implants except two interfragmentary screws and two screws fixing the lateral plate to the humerus were removed. The removal of these screws was unfeasible, and therefore, the lateral plate was cut with metal-cutting saw. In the operative theater, the fractures that had occurred during the first floating elbow injury appeared completely healed. An ORIF of the humeral and forearm fractures using one Y-plate and two straight plates was respectively performed. Cable wires were used to stabilize the third ulnar fragment. The postoperative period was uneventful, and the patient was encouraged to start exercising the shoulder and the hand the day after surgery. The elbow was immobilized in a plaster cast for 3 weeks to limit active movements. Rehabilitation of the elbow was then begun. Clinical and roentgenographic follow-up controls were performed at 1, 3, 6, and 18 months. A functional assessment was made using the Liverpool Elbow Score (LES) that had been previously validated and tested for its internal consistency []. The LES includes a nine-item patient-answered questionnaire and six surgeon-oriented items, with a total score ranging from 0 (worst) to 10 (best). The patient’s fractures partially consolidated within 3 months and radiological evaluation 1 year after surgery showed the union of all fractures (Fig. ). At 18-month follow-up, the LES score was 5.72 and the patient had reduced but painless elbow range of motion compared to the opposite side (Fig. ). The elbow showed 90° of flexion and 45° of extension, the pronation and supination of the forearm were 55° and 45°, respectively, and the palmar grip strength, when measured with a static strength tester (CSD 300 Chatillon—Ametek Inc., Florida), was 50% with respect to the uninjured side. Electroneuromyography revealed progressive improvement in the radial and ulnar nerves since the sixth-month follow-up examination, but at the final evaluation, residual reduction of electrophysiological parameters compared to normal values was recorded in the ulnar nerve territory. The patient was satisfied with the functional result and refused to undergo further surgery to improve the elbow motion. For the present case report, the patient was informed that data concerning the case would be submitted for publication and gave his consent. |
A 46-year-old female patient with a BMI of 25 was referred to our tertiary center (Cerrahpasa Medical Faculty, Gynecological Oncology Unit) in August 2016 with the diagnosis of platinum-resistant ovarian cancer. She had undergone primary cytoreductive surgery 6 months ago. Her medical history was unremarkable except for treatment for epithelial ovarian carcinoma. Positron emission tomography and computed tomography (CT) revealed implants on the right diaphragmatic surface after first-line adjuvant chemotherapy. She has never had any thoracic or abdominal symptoms and no history of major trauma. Initial preoperative evaluation of the patient revealed no significant sign of diaphragmatic hernia. Her physical examination was normal, including chest auscultation sounds and chest X-ray. Thoracic CT did not reveal any abnormal image that could be evaluated as a diaphragmatic hernia. Secondary cytoreductive surgery and HIPEC were planned. She was placed in flank position, and, under general anesthesia, laparotomy was performed. An oval defect with the dimensions of 3 × 4 cm was seen in the left posterolateral site of the diaphragm (Fig. ). The most distal portion of the inferior lobe of the left lung was visible through the defect during inspiration and expiration movements. There was no herniation or strangulation of abdominal components. In addition, the right diaphragm was completely stripped because of a tumor implant. Meanwhile, a 6 × 3 cm iatrogenic diaphragmatic defect was detected (Fig. ). Drainage chest tubes were inserted bilaterally via the 6th intercostal spaces to avoid leakage of the chemotherapeutic agents across repaired diaphragm and postoperative massive pleural effusion. The tissue around the congenital left-sided defect was excised circularly and the defect itself was repaired with interrupted nonabsorbable monofilament sutures. The iatrogenic defect was also repaired with interrupted nonabsorbable monofilament sutures. After complete cytoreduction had been achieved, hyperthermic intraperitoneal chemotherapy with doxorubicin 35 mg/m2 and paclitaxel 175 mg/m2 for 60 min at 42.5°C was delivered. No leakage via drainage tubes from the thoracic cavity was noted during the procedure. During the surgery, the patient was managed by standard monitoring, which involved continuous monitoring of electrocardiography, invasive blood pressure, central venous pressure, pulse oximetry, body temperature and hourly urine output. Arterial samples were drawn at regular periods for the determination of electrolytes, acid base status and hematocrit. Finally, the operation lasted 4 h. At the end of the surgery, the patient remained intubated and was transferred to the intensive care unit for monitoring ongoing resuscitation along with fluid and electrolyte management. The patient was extubated 10 h after completion of surgery, and was transferred to the service on the 2nd postoperative day. Prophylactic enoxaparin 4,000 IU was started 6 h after surgery. On the 3rd and 4th postoperative day, excessive sleepiness throughout the day and dyspraxia was noted. Bilateral anterior thalamic infarction caused by emboli that block the thalamosubthalamic artery was demonstrated on brain CT (Fig. ). The dose of enoxaparin was changed from 4,000 IU once a day to 800 IU twice a day. The patient was discharged on the 12th day after surgery. The patient's symptoms improved within 3 weeks. Follow-up at 4 months after surgery showed no evidence of recurrence and no symptom regarding thalamic infarct. |
Patient 4 (PA-4) experienced premature loss of deciduous teeth since he was one and half years old. The physical examination was unremarkable, other than the absence of the upper and lower anterior incisor and canine teeth (Fig. m). Serum ALP activity was 42 U/L at diagnosis when he was 4 years old. Panoramic X-ray showed a reduced alveolar bone of whole dentition (Fig. n). His wrist and knee X-ray revealed normal growth plates without any evidence of defective bones (Fig. o and p). Patient 4 was diagnosed as odonto HPP at 4 years old (Table ).\nIn order to confirm the diagnosis of HPP and correlate the phenotype with specific genotype, mutational analyses of the ALPL gene were performed in all four patients and their parents except the mother of patient 1 was not involved in this study. The ALPL variants in patient 1 and patient 3 were identified using next generation sequencing and confirmed with Sanger sequencing, whereas the variants in patient 2 and patient 4 were identified and confirmed by direct Sanger sequencing. A 3D structural modeling of the TNSALP constructed based on its sequence homology to the placental isozyme (PDB ID: 1EW2), was used to locate the missense mutation [].\nSix different variants were identified in the ALPL gene in our four patients, including five missense variants and one small insertion variant (Table ). All variants were inherited from their unaffected parents except patient 1 (Fig. ). As the most severe and lethal infantile HPP in the present study, patient 1 carried a novel homozygous variant of c.359G > C (p.G120A) at exon 5 of the ALPL gene, which was predicted to be disease-causing by in-silico analysis of bioinformatics software. His father demonstrated a heterozygous status, while his mother was not involved in this study. To further investigate the effect of the novel missense mutation of c.359G > C, a 3D structure modeling of TNSALP was used to locate the residue, indicating that the residue p.G120A was located in the secondary structure of the TNSALP homodimer interface. |
A 55-year-old woman was referred to the Department of Oral and Maxillofacial Surgery, Aalborg University Hospital, Denmark, due to a long-lasting increasing dull pain from the left side of her face. There was no previously history of facial trauma and her medical history did not contain any known pathology of the intestines.\nExtraorally, a discrete swelling was observed anterior to the left temporomandibular joint. There were no signs of inflammation. The temporalis and masseter muscles were tender on palpation, with normal range of motion. Intraorally, the patient was edentulous with well-functioning removable prosthesis. A panoramic radiographic exam showed the presence of a pedicled osseous lesion originating from the left zygomatic arch (Fig. ). A cone beam computed tomography confirmed the presence of a 1.2 X 1.4 cm well-circumscribed radiopaque structure located on the lateral border of the left zygomatic arch (Fig. ). On the basis of the clinical and radiographic findings, a working diagnosis of peripheral osteoma of the zygoma arch was made. Due to increasing pain and cosmetic reasons, the decision was made to surgically remove the tumor.\nIn general anaesthesia, a preauricular incision through the skin and subcutaneous connective tissue was performed. The tissue was reflected by blunt dissection above the zygomatic arch to the level of the superficial layer of the temporalis fascia. The superficial temporal vessels were retracted anteriorly with the skin flap and nerve stimulator was used during the surgical procedure to detect branches of the facial nerve. After incision of the superficial temporal fascia over the zygomatic arch and dissection of the periosteum from the lateral portion of the zygomatic arch, a complete view of the lesion was obtained and the temporal branch of the facial nerve was carefully protected with a retractor within the superficial layer of the temporalis fascia. The pedunculated bony structure was removed with bur and chisel (Fig. ). Finally, surgical recontouring of the zygomatic arch was performed (Fig. ). Healing was uneventful. Histopathologic examination revealed a well-circumscribed mass composed of dense lamellar bone, compatible with osteoma.\nThere was no clinical or radiographic evidence of recurrence on the one year follow-up examination (Fig. ). The patient had normal neurovascular function, but minor pain from her temporomandibular joint and muscles persisted. |
A 68-year-old woman presented to the Department of Oral and Maxillofacial Surgery at Nagoya Ekisai Hospital (Nagoya, Japan) with a chief complaint of malaise and a 7-month history of swelling of the left buccal mucosa. The patient had no congenital swelling of the left buccal mucosa at birth and no history of systemic disease or relevant family history. The patient had undergone maxillary molar restoration treatment 2 years earlier, after which she reported biting regularly on her buccal mucosa. On most occasions, the wound had healed within a week, so she had not sought medical treatment. An extraoral examination revealed no facial swelling or asymmetry. However, an intraoral examination revealed an area of diffuse swelling on the left buccal mucosa measuring about 15 mm × 30 mm and containing a papillary lesion with multiple red, blue, and clear pebble-like vesicles (Figure ). On palpation, the lesion was nontender and soft. The swelling had not expanded to the veins and was pulsatile. An orthopantomogram confirmed that the adjacent bone was intact. Magnetic resonance imaging revealed a soft tissue mass with a clearly distinguishable outline of the buccinator muscle (Figure ). The lesion was surgically excised under local anesthesia with a margin of 3 mm and a depth of 2 mm via the inside surface of the fascia of the buccinator muscle. The outcome was favorable. Pathologic examination of the specimen revealed expanded lymphatic vessels lined by thin endothelial cells and containing lymphatic fluid. A diagnosis of lymphatic malformation was confirmed on histopathology and immunohistochemical studies. Immunohistochemistry was negative for vascular markers such as CD31 and CD34, and the lymphatics stained specifically for D2-40 (podoplanin) (Figures , , ). On follow-up, the wound was found to have healed with no evidence of trismus or recurrence. The patient continued to be recurrence-free at her 2-year follow-up. Informed consent was obtained from the patient, and the procedures were in accordance with the Helsinki Declaration. |
A 33-year-old woman presented to our electrophysiology clinic for evaluation of 1st degree atrioventricular (AV) delay. She had been diagnosed with this finding 2 years prior while undergoing biannual fitness testing in the Navy. At that time, she had undergone Holter monitoring and treadmill stress echocardiography that did not show malignant conduction disease, arrhythmias, or structural heart disease.\nAt this electrophysiology clinic visit, she reported mild exercise intolerance and a sense that her “heart was not keeping up,” which she attributed to physical deconditioning. Her electrocardiogram (ECG) was notable for sinus rhythm at 71 beats per minute with a PR interval of 214 milliseconds and was otherwise unremarkable (). She underwent repeat treadmill stress testing to assess her heart rate response and AV conduction; she achieved 78% of the maximum predicted heart rate and had superiorly directed premature ventricular contractions (PVCs) and slow accelerated idioventricular rhythm during recovery. Cardiac magnetic resonance imaging, performed to evaluate for infiltrative disease, showed normal biventricular function, normal chamber structure and dimensions, and no late gadolinium enhancement. She was diagnosed with high vagal tone.\nAt her next follow-up two years later at age 35, she was 22 weeks pregnant with her first child. She reported becoming easily dyspneic with exercise in the context of a 10-15-pound weight gain and intermittent palpitations. Her ECG showed sinus bradycardia, a competing junctional rhythm, occasional PVCs, and poor R wave progression—all of which were new (). Review of her family history revealed that her father died at age 47 while awaiting heart transplantation for presumed end-stage ischemic cardiomyopathy. Given these findings, a 30-day ambulatory arrhythmia monitor was ordered, and within the first two weeks, sinus node dysfunction and frequent multifocal ventricular arrhythmias were captured (). In the setting of both her tachy- and bradyarrhythmias and concerning family history, a genetic disorder was suspected. She underwent urgent and successful dual-chamber implantable cardioverter defibrillator (ICD) implantation at 25 weeks gestation, was started on metoprolol, and established care with our Maternal-Fetal Medicine program.\nShe was referred to our inherited cardiovascular disease center for genetic counseling and testing. Her three-generation family history was notable for cardiomyopathy in her father and heart issues in her paternal grandfather (). She underwent genetic sequencing of 121 genes associated with cardiomyopathy and arrhythmia through GeneDx (Gaithersburg, Maryland) and was found to be heterozygous for two different pathogenic variants—a frameshift variant in LMNA (c.1174_1178delAGCCC; p.Ser392TyrfsX32) and a missense variant in MYBPC3 (c.1504C>T; p.Arg502Trp). This specific LMNA variant had not previously been reported but was predicted to result in either protein truncation or loss of protein product through nonsense-mediated mRNA decay and was therefore felt to be the likely cause of her arrhythmias. The MYBPC3 (c.1504C>T) variant had previously been reported in multiple individuals in association with hypertrophic cardiomyopathy. Cascade screening of first-degree relatives was recommended.\nAt 32 weeks gestation, device interrogation and manual review of reported events showed several prolonged episodes of atrial fibrillation. After extensive discussion regarding the risks and benefits of therapeutic anticoagulation, she was started on prophylactic enoxaparin with the plan to undergo induction of labor at term (37 weeks gestation). She underwent an uncomplicated low transverse cesarean section due to nonreassuring fetal heart tones during her induction. She was discharged on therapeutic enoxaparin for thromboprophylaxis given limited data on the use of direct oral anticoagulants during breastfeeding. |
A 54-year-old male presented to our outpatient clinic with a 3-week history of low back pain radiating down to his right leg. The patient's medical history was unremarkable except for an operation of cholecystectomy 5 years previously. The patient's physical examination was unremarkable. Neurogenic claudication and lumbar radiculopathy were detected in his neurological examination. Grade 1 spondylolisthesis, multiple discopathies, and lumbar spinal stenosis were detected on his lumbar magnetic resonance imaging (MRI) scan.\nAfter completing the appropriate preoperative evaluation, the patient was taken to the operation theater and placed on the operating table in the prone position under general anesthesia. As routinely done, silicon pads and pillows were placed to pressure points to provide pressure relief. The patient underwent a 2-level lumbar micro-discectomy, 4-level decompression, and 4-level transpedicular screw fixation surgery. The total duration of the operation was 270 min. The patient's blood pressure remained within normal limits (110/70 mmHg) except for 30 min of 90/70 mmHg during the surgery. Total blood loss was 500 ml and urine output was 300 ml. The replacement was performed with 1000 ml of normal saline with no need for blood transfusion. The lowest hematocrit level detected during the surgery and in the early postoperative period was 42%.\nImmediately after the surgery, the patient complained of having no vision in his left eye. In ophthalmologic examination, visual acuity was 20/20 in the right eye and he had no vision and no light perception in the left eye. There was relative afferent pupil defect in his left eye. In the fundoscopic examination, the right retina was normal and the left retina was completely pale [Figure and ]. Upon consultation with the department of ophthalmology, the intraocular pressures of both eyes were measured and detected within normal limits (18 mmHg). In the biomicroscopic examination, the anterior segment structures of both eyes were normal. There was no problem in the functions of the extraocular muscles. Based on the suggestion of the department of ophthalmology, the patient was started on carbonic anhydrase inhibitor as the first-line treatment. The brain MRI and diffusion MRI of the patient were normal. In fundus fluorescein angiography (FFA), there was no filling defect in the retinal vascular structures in the left eye []. On computed tomography angiography, there was no filling defect in the vertebral artery, internal carotid artery, ophthalmic artery, and retinal arteries. Anterior chamber paracentesis was performed at the 20th h of the surgery. The patient was discharged with a loss of vision in the left eye on the 5th day of operation. The patient was followed up at regular intervals at outpatient clinic. However, the patient's vision and light reflex did not improve during the 1st month of operation.\nDuring the examination of the patient at the 4th week, visual acuity was recorded as at the level of hand movements in the left eye. In fundoscopic examination, the left optic disc was pale, the vascular structures were highly thinned, and the left retina was atrophic []. In FFA performed on the same day, the vascular structures were thinned, there was a delay in their filling, and there were hypofluorescence areas secondary to retinal atrophy on the left optic disc []. No further improvement was achieved in the vision in the 3rd month, the 6th month, and 1 year of follow-up. |
A 61-year-old female patient who had been diagnosed with MM at the skull and rib areas by the Department of Hematology and Oncology in May 2009 received HSCT in January 2010. She was observed to be in a systemically stable state. In December 2010, she complained of edemas and pain in the left mandibular buccal gingiva and the buccal mucosa; thus, she was referred to our department. She had a history of extraction of the left lower first molar tooth due to pain and tooth mobility at the relevant sites 2 months before her visit to the Department of Oral and Maxillofacial Surgery. An edema in the left facial area was found during the first examination, and clinical symptoms such as spontaneous hemorrhage of blue-reddish color tones were observed.() No specific features were noted in the panoramic view. There were no abnormal results except decrease of hemoglobin and hematocrit from the complete blood cell count test.() Monoclonal gammapathy was observed with 5.29 g/dL when the M-protein level increased since June 2010, with the IgA level increasing considerably in the serum immunoglobulin counts (2,314 mg/dL, normal range=70-400 mg/dL). There were no osteolytic lesions in the mandible from the CT scan. Since the patient had a history of existing MM, the test results seemed to be related to hematological diseases, and no changes in bones were observed from the CT scan; our department judged the existing MM to have been manifested into the oral soft tissue area and temporarily diagnosed the patient in detail with extramedullary plasmacytoma. Strong standardized uptake value (SUV) uptake (SUVmax=4.3) was found at the relevant site from a positron emission tomography-CT (PET-CT).()\nThus, our department carried out a biopsy, and tissue specimens were sent to the Department of Pathology at Samsung Medical Center. After the results of the biopsy determined the disease to be plasmacytoma, chemotherapy using lenalidomide, dexamethasone, and radiotherapy (25 Gy) were performed. Healing of the oral lesions was observed later(); some aspects of improvement, including decrease of IgA level, were found from the blood test carried out in March 2011 (740 mg/dL); the M-protein level also shifted to a constant decrease (1.9 g/dL in March 2011), and there was no uptake observed from a follow-up PET-CT scan.()\nDuring the follow-up in August 2011, pain and edemas at the buccal gingiva of the left maxillary posterior teeth and palatal gingiva and pain, edemas, and spontaneous hemorrhage at the left mandibular lingual and buccal sites were found.() Such was considered a recurrence of the existing lesions. In addition, osteolytic lesion with diameter of up to 1 cm and sclerotic rim was found in plain film and CT scans. Consequently, biopsy and radiotherapy were performed again, with the patient hospitalized after consulting the Department of Hematology and Oncology for observation with chemotherapy (dexamethasone, thalidomide, cyclo-pho sphamide, doxorubicin, cisplatin, and etoposide). Note, however, that cytopenia, azotemia, and pleural effusion of the lungs were observed due to development of the lesions; the patient was discharged in September 2011 due to the aggravation of general condition owing to the lesions. |
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