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A 69-year-old woman was referred to our hospital by a dentist at another hospital because of swelling in her left maxilla. There was the mobility of the left maxillary second premolar and first molar (FDI #25 and #26) and swelling of the surrounding gingiva. Computed tomography (CT) images revealed maxillary bone expansion with a ground glass appearance (). The patient had begun steroid therapy for polymyalgia rheumatica 4.5 years earlier. Since then she had been taking alendronate, one of the oral BPs, for the prevention of glucocorticoid-induced osteoporosis.\nThe clinical diagnosis was acute periodontitis in #25 and #26 parts with FD of the left maxillary bone. After an administration of clarithromycin, #25 and #26 were extracted, and then small bone fragments were collected from the sockets for a histopathological examination. The finding of not FD but the sequestrum predicted the occurrence of BRONJ.\nAt 1 year after the extractions, a purulent discharge and exposed bone with gingival fistulas were observed in the extraction sites (). CT images revealed separated sequestrums in the left maxilla and bone resorption in the periapical area of the left maxillary first premolar (FDI #24) (). With the permission of the patient’s attending physicians, the alendronate was withdrawn. After exposed bone had been observed for 8 weeks, the patient was clinically diagnosed with Stage 2 BRONJ accompanying FD of the left maxillary bone. Under general anesthesia, we first performed extraction of #24 and sequestrectomy. We then performed a partial resection of the FD because necrotic-like bone fragments were collected by curettage of the marginal bone (). Finally, the surgical wound was completely closed by sutures. The histopathological diagnosis was chronic osteomyelitis with FD (). At 2.5 years after the operation, the healing is uneventful although residual FD bone exists (). Although local conditions permit the resumption of alendronate, it remains discontinued at the attending physicians’ discretion. |
A 20-year-old elite collegiate football player (starter Division I Pac-12 conference) was in the middle of a play when he planted his foot and sustained a noncontact hyperextension injury to his right knee, resulting in a traumatic posterior knee dislocation. He was emergently reduced and immobilized on the field and then taken to the visiting team's trauma center for further evaluation. On initial presentation, good Doppler flow at the foot was noticed; however, over the next few hours, an arterial injury was highly suspected. The athlete was then taken to the operating room for angiography and arterial repair. Intraoperatively, acute occlusion of the distal popliteus artery at the bifurcation of the anterior tibial and tibioperoneal trunks was observed. A double interposition bypass with great saphenous vein using a posterior approach was ultimately performed, returning excellent flow distally. Finally, a long posterior splint was used to protect the bypass and stabilize the knee.\nOnce the patient had stabilized at the visiting team's hospital, he was referred to our outpatient facility. He was seen 5 days later for definitive management of his MKI. Physical examination was notable for decreased sensation in the CPN distribution, grade 4 weakness with ankle dorsiflexon and eversion, and moderate effusion with ecchymosis. Lachman's was positive as well as gross varus laxity. Review of the MRI revealed a KD III L MKI, complete anterior cruciate ligament (ACL) tear, partial posterior cruciate ligament (PCL) tear, lateral collateral ligament (LCL) fibular avulsion, and bicep femoris avulsion from the fibula. To assist with repair and clearance, vascular and plastic surgery were consulted. Once the team was established, it was decided that reconstruction would be performed in two stages. The first stage would be an open exploration of the CPN to determine the extent of injury and possible repair with plastic surgery, followed by repair versus reconstruction of the LCL and bicep femoris, with vascular surgery on standby. The second stage would be an ACL reconstruction using autograft. The plan was discussed extensively with the athlete and his family, and the decision to proceed was made.\nSeventeen days after initial injury and vascular interposition bypass graft, a right-knee open LCL and bicep femoris repair with common peroneal neuroplasty was performed. Using a lateral incision, careful dissection was carried down to the lateral complex and CPN. Neuroplasty of the CPN was then performed from the thigh to the superficial and deep branches within the lateral compartment. It was noted that the CPN had some contusion at the tibial neck but was otherwise intact. Attention was then turned to the lateral complex, and the LCL and fibular biceps femoris attachment were repaired back to the fibular head using two Mitek Lupine suture anchors at 30° to 40° of knee flexion. Doppler was performed at the end of the case with excellent flow, followed by a long posterior splint with medial and lateral struts.\nPostoperatively, the patient was kept non–weight bearing. At 2 weeks post-op, he was converted to a hinged-knee brace locked at 30 to 40 flexion. At 4 weeks post-op, he began physical therapy to work on active range of motion (ROM) with the goal of zero to 120° and continued non–weight bearing. At this time, he began to show improvement in CPN function in both sensation and strength. During the 6-week follow-up, it was decided to proceed with an ACL reconstruction using bone-patellar tendon-bone autograft and was scheduled for 10 weeks status post-index procedure. During the same follow-up, the knee ROM was 5° to 110° of extension and flexion, respectively. Quadricep and hamstring strength as well as ankle dorsiflexion and eversion were near normal.\nThe second stage of the MKI reconstruction commenced 10 weeks after the athlete's index procedure. Examination under anesthesia revealed a positive Lachman and pivot shift tests with negative dial test at 30° and 90°. Posterior offset of 6 to 8 mm with a firm end point on his posterior drawer and very minimal laxity of the lateral complex at only 20° was present. The rest of the examination under anesthesia was otherwise normal. During diagnostic arthroscopy, the medial and lateral menisci and articular surfaces were normal. A femoral-sided PCL injury that had healed was indicated. The ACL reconstruction using bone-patellar tendon-bone autograft was then completed through a two-incision approach. The reconstruction was uneventful, with excellent Doppler pulses before and after surgery.\nThe athlete's university postoperatively followed Multicenter Orthopaedic Outcomes Network ACL rehab protocol with the addition of no varus stress. At 5 months after LCL and bicep femoris repair with CPN neuroplasty and 3 months after ACL reconstruction, active ROM was symmetric to the contralateral side. Strength was decreased in the quadriceps, hamstrings, and great toe extension. Ankle dorsiflexion and eversion were excellent, and sensation had returned to normal. The knee was stable to varus/valgus stress and anterior/posterior translation. Finally, after extensive rehabilitation with team trainers and 13.5 months from injury, the patient had full recovery of the CPN as well as normal strength and ROM compared with the contralateral side, with excellent ligamentous stability in all planes. At this time, he was cleared to return to sport with use of a custom ACL knee brace for protection against varus and to avoid hyperextension mechanisms of injury. |
A 52-year-old male patient without a significant medical history was admitted to a local hospital reporting palpitations, lightheadedness, and presyncope symptomatology that firstly occurred one week before his admission. The electrocardiogram (ECG) demonstrated tachycardia–bradycardia syndrome, and the 24-hour ECG Holter monitoring revealed a sinus rhythm, with frequent episodes of paroxistic atrial fibrillation, a prolonged sinus pause of 2.97 seconds following the termination of tachycardia, and then the restoration of a sinus rhythm with severe sinus bradycardia before reaching the baseline heart rate of 55 beats per minute (A).\nConsidering the definite correlation between the symptoms and the electrocardiographic findings, the diagnosis was of sinus node disease, brady–tachy form, with symptomatic intermittent sinus bradycardia, prolonged sinus pauses, and a Class IB indication for cardiac pacing []. The patient was therefore referred to our cardiology clinic for a dual-chamber, permanent pacemaker implantation.\nThe physical examination revealed a 4/6 systolic murmur with a maximum of intensity on the left sternal border. No diastolic murmur was present. The ECG performed in our clinic demonstrated a sinus rhythm, a benign early repolarization pattern, and an increased voltage of the QRS complex, which was interpreted as a normal feature considering the physical constitution of the patient, who was very tall and thin. However, subtle secondary repolarization changes in lead V6 and the inferior leads could suggest an early stage of left ventricle hypertrophy (B). Transthoracic echocardiography showed a subaortic thin membrane located at 9 mm below the aortic valve (A). The subaortic membrane caused moderate subaortic stenosis with a mean gradient of 37.3 mmHg and mild aortic regurgitation (see ). The cardiac cavities dimensions were normal, and the left ventricle had normal contractility and systolic function (See ). No other abnormalities were initially observed. Transesophageal echocardiography offered a better visualization of the subaortic membrane and demonstrated the systolic fluttering of the aortic cusps (B; see ). Transesophageal echocardiography further excluded the presence of a possibly associated atrial or ventricular septal defect.\nLeft subclavian access was initially used, but fluoroscopic evaluation revealed that the guidewire had an abnormal trajectory in the left paravertebral region, which raised the suspicion of a venous anomaly. Therefore, venography was performed and demonstrated a PLSVC draining in the right atrium via the coronary sinus but without the possibility to clearly assess the status of the right SVC (see ). Because the team encountered difficulties in advancing the lead, they switched to the right side. Surprisingly, the venography performed from the right subclavian access demonstrated the absence of the SVC on the right side (see ). Though demanding, the ventricular electrode was eventually advanced through the left SVC and the coronary sinus. After entering the right atrium, it formed a loop in the atrial cavity in order to facilitate the passage through the tricuspid orifice. The electrode was then actively fixed in the right ventricle (see ). However, the right atrial lead could not be implanted due to the difficult access to the right atrial appendage and the risk of the destabilization of the ventricular lead position. Postprocedural careful transthoracic echocardiography revealed a dilated coronary sinus that measured 24/26 mm and contained the pacemaker lead (C). The flow through the coronary sinus was normal and unimpaired by the pacemaker lead. Subsequently, computed tomography angiography excluded other venous or cardiac anomalies (D).\nAfter cardiac pacing, the patient became asymptomatic with a normal exercise tolerance. Considering the absence of symptoms, with only moderate subaortic obstruction and mild aortic regurgitation, there was no strong recommendation for the surgical removal of the subaortic membrane according to the European Guidelines for the management of grown-up congenital heart disease []. Furthermore, the patient refused to take heart surgery into consideration. He was discharged with the recommendation to follow beta-blocker and direct oral anticoagulant therapy, considering the high risk of the reoccurrence of the supraventricular tachyarrhythmia. A medical reevaluation was performed after one and six months following the pacemaker implantation, and it revealed the absence of symptoms, adequate ventricular pacing, and the stationary echocardiographic parameters of the subaortic stenosis. Further reassessment is expected, close monitoring being of foremost importance to identify the optimal moment for the surgical intervention.\nThis research was performed in accordance with the Declaration of Helsinki of 1975 and revised in 2013. After extensive consultation, the patient gave verbal and written informed consent and fully authorized the authors to use his medical data for research purposes, as stated in the “Patient Informed Consent” (Order 1410/2016, issued by the Romanian Ministry of Health), signed by the patient. |
A 47-year-old male presented with the complaint of his right eye having gradually become blurred over the past 4 months followed by blurred vision in his left eye 1 week before the initial visit. He reported a 10-year history of diabetes mellitus treated with metformin 3 × 500 mg and a blood glucose level controlled at around 100–200 mg/dL. On ophthalmological examination, his visual acuity showed light perception in the right eye and 20/400 in the left eye. IOP was high in both eyes (right eye 57 mm Hg and left eye 50 mm Hg) with mid-dilated pupil, corneal edema, and a bullous appearance on the right eye. Rubeosis iridis was also prominent in both eyes. Funduscopic examination of the left eye showed a slightly enlarged cup-disk ratio (0.4–0.5) with neovascularization on the disk, dot blot hemorrhage, and exudates in all quadrants of the retina. Gonioscopic findings in the right eye showed closed angle with neovascularization of the angle in all quadrants, while the angles of the left eye were still open with neovascularization of the angle in three quadrants. The glucose level was found to be 300 mg/mL. The patient was diagnosed as having NVG in both eyes as a result of PDR and uncontrolled diabetic mellitus. He was admitted for Ahmed valve implantation with intraoperative intravitreal 1.25 mg/50 µL bevacizumab injection in the left eye. Surgical intervention was required for the severe condition of the right eye. Timolol maleate and brinzolamide eye drops together with oral carbonic anhydrase were prescribed. The patient was also referred for consultation with an endocrine specialist.\nOne day following Ahmed valve implantation, the IOP of the left eye was found to be reduced to 6 mm Hg, and marked improvement of visual acuity (20/100) with absence of hyphema was observed. PRP was initially planned for the patient but could not be performed due to poor visualization caused by lens opacity. Phacoemulsification with intraocular lens insertion was then performed and immediately followed by PRP. BCVA improved to 20/20. One month after PRP, the patient's IOP had gradually increased to 37 mm Hg with flat Ahmed implant bleb and persistent rubeosis iridis, thus necessitating a 5-fluorouracil needling procedure combined with bevacizumab intracameral injection. The IOP gradually decreased to <20 mm Hg with administration of antiglaucoma eye drops (brinzolamide and timolol maleate). The second PRP was performed in the following month. The patient's left eye BCVA was consistent at 20/20 and IOP was 9 mm Hg at 15 months follow-up after the second PRP. He was instructed to have bi- or trimonthly visits as rubeosis iridis persisted; however, funduscopic appearance was good and further careful monitoring was advised. The patient's blood glucose level was maintained at <200 mm/dL.\nThe last clinical appearance is shown in Figure and Figure . Despite persisting rubeosis, the patient presented with consistent BCVA at 20/20 and controlled IOP. Optical coherence tomography of the optic disk (Fig. ) showed good retinal nerve fiber layer thickness. Optical coherence tomography of the macula (Fig. ) showed slight macular edema. Visual field examination with Humphrey test showed mild to moderate peripheral visual field defect and preserved central visual field in the left eye. |
A 15-year-old female patient (bodyweight 50 kg, height 160 cm) presented with syncope. Severe arterial hypertension was diagnosed (178/147 mmHg left arm, 102/83 mmHg left leg). She reported previous episodes of exercise independent headaches and nose bleeds. Auscultation revealed no cardiac murmurs, but an accentuated second heart sound and reduced pulses in the lower limbs. There were no clinical signs to suggest Alagille or William’s syndrome or clinical evidence of neurofibromatosis. Blood tests showed normal inflammatory markers and normal creatinine. The urine dip showed no markers for blood or protein. No regular medication was taken at the time she presented. The electrocardiogram showed normal sinus rhythm, no significant signs of left ventricular (LV) hypertrophy or abnormal repolarization. Echocardiography detected a severe concentric LV hypertrophy (14 mm diastolic septal diameter, 15 mm diastolic diameter of the LV posterior wall) and a long hypoplastic segment in the abdominal aorta with massive arterial collateralization. Cardiac catheterization confirmed the diagnosis of a midaortic syndrome with a minimum diameter of <1.5 mm and a gradient of 50 mmHg between the aortic arch and the femoral arteries (Figure ). The narrow part began directly under the diaphragm above the truncus coeliacus and continued down to the bifurcation of the arteriae iliacae including the renal arteries, with reversed flow in the caudal aorta up to the renal arteries. Because of the extraordinary length of the hypoplastic segment of 23 cm, we decided for a stepwise interventional therapy with a paclitaxel covered balloon. No additional computed tomography scan or magnetic resonance imaging (MRI) was performed. Consultation with our surgeon, supported the interventional approach. The proposed procedure was reviewed and approved by our institutional review board, extensively discussed with the patient’s family, and informed consent was obtained before proceeding.\nAfter balloon interrogation of the long stenotic abdominal aorta using a 6 × 20 mm Tyshak balloon (NuMED Inc., Hopkinton, NY, USA) to differentiate between rigid stenosis and hypoplastic parts, serial balloon dilation with a 7 × 40 mm paclitaxel covered In.Pact balloon (Medtronic, Minneapolis, MN, USA) (3 µg/mm2 Paclitaxel on the balloon) was performed (Figure ) from end to end of the stenosis. Planned redilations, to stepwise improve the aortic compliance and reduce the risk of dissection, were performed after 2, 4, and 8 months with Elutax balloons ranging from 8 mm to 12 mm diameter (Aachen Resonance, Düsseldorf, Germany) (2 µg/mm At the end of the last interventional procedure, a mild endothelial lesion at the former narrowest point of the aorta was noticed (Figure ). This lesion healed and after 2.7 years MRI showed a stable and adequate result (Figure ). Today the girl is in unrestricted physical condition. The blood pressure has markedly improved (139/64 mmHg right arm and 127/65 mmHg right leg) under therapy with atenolol and amlodipine.\nThe patient and patient’s family consented to the publication of this case’s history and the images presented. |
A 40-year-old male not known to have any chronic medical illness, presented complaining of epigastric and left upper quadrant pain for 1 month, associated with intermittent nausea and vomiting, and aggravated by fatty meals, with no other associated symptoms. He had frequent visits to the emergency department where he was managed with analgesia and antacids with mild symptomatic improvement. Clinical examination was unremarkable with no evidence of jaundice or abdominal tenderness. His blood test results showed a normal complete blood count, kidney function, and liver function. Chest X Ray revealed dextrocardia with stomach fundic gas shadow on Right side (). Abdominal ultrasonography revealed transpositioning of the solid organs with a left sided liver and gallbladder with a solitary stone and mild wall thickening. We elected to perform a Magnetic Resonance Cholangiopancreatography to delineate the anatomy and to rule out any anomalies within the biliary tree. It confirmed the previously noted findings, showed no evident anomaly within the biliary tree, and confirmed the diagnosis of situs inversus totalis (, ). The patient was Scheduled for an elective laparoscopic cholecystectomy.\nThe Operating room equipment arrangement was adjusted as Mirror Image of Routine Laparoscopic cholecystectomy (). The Monitor was placed on left side of the patient. The surgeon with the camera assistant were on right side of the patient and the first assistant was on left side of the patient. The abdomen was scrubbed and draped in the standard aseptic technique. The first infraumbilical 11 mm trocar introduced and pneumoperitoneum induced using the open technique. Three 5 mm trocars were placed, at the xiphisternum which was used for the surgeon’s left hand, at the left midclavicular line 2 cm below the costal margin which was used as working port for the surgeon’s right hand and at left anterior axillary line 5 cm from the costal margin which was used for retraction of the gallbladder fundus by the second assistant, respectively. Inspection of the abdominal cavity confirmed the presence of situs inversus totalis, with the liver and the gallbladder positioned in the left side (). The Calot’s triangle was identified. The peritoneum overlying the gallbladder infundibulum was then incised and the cystic duct and cystic artery identified and circumferentially dissected, till the critical view was obtained. The cystic duct and cystic artery were then doubly clipped and divided, through the subcostal port using the right hand. The gallbladder was dissected from its peritoneal attachments using electrocautery and was retrieved using Endoscopic bag through the infraumbilical port. The total operative duration was 80 min, which was longer than the conventional laparoscopic cholecystectomy performed in patient without underlying anatomical variation. It can be attributed to the modification in the technique required to adjust to the mirror image anatomy.\nThe patient had an uneventful postoperative course and was discharged on postoperative day 1. Pathological examination of the gallbladder confirmed the presence of gallstones with chronic cholecystitis. No postoperative complications were noted during his follow up in the outpatient department. |
A 70-year-old female patient visited our pain clinic. She had a previous medical history of hypertension and tension headache for 10 years. Seven years ago, the patient had undergone L3-S1 posterolateral spinal fusion surgery after being diagnosed with spinal stenosis and spondylolisthesis. The surgery, however, led to no improvement in the patient's lumbar back pain and lumbar radiculopathy. In order to treat the continuing low back pain, pelvic pain, and spread of radicular pain to both sides of the lower leg, several epidural and caudal blocks were performed. Although the lumbar back pain improved for 7 months with treatment, the patient continued to experience pelvic pain on both sides, corresponding to a score of 9 on the visual analogue scale (VAS) ranging from 0-10 (0 = no pain, 10 = most intense pain ever). Physical examination showed no signs of erythema, or infection. There was tenderness upon palpating both SIJs, but not on the hip joint. Patrick's and Gaenslen's tests were positive on both sides. We decided to perform SIJB for diagnostic and therapeutic purposes. We injected 0.5% mepivacaine 2 cc and triamcinolone acetate 10 mg into the SIJs. During the SIJB, we confirmed the referred pain the patient had complained of. Two hours after the procedure, the patient's pain level decreased to a VAS score of 0 and she was discharged.\nThree months later, the patient was re-admitted with a chief complaint of bilateral pelvic pain corresponding to a VAS score of 6. Physical examination showed the same results as previously. Recurrence of SIJD was suspected, and SIJB was planned. We performed SIJB on the left SIJ first to diagnose and treat the patient. After placement of non-invasive blood pressure and pulse oximetry monitors, the patient was placed on the fluoroscopy table in the prone position and both the hips and the pelvic region around the sacroiliac joints were disinfected widely with povidone iodine solution, after which we waited for 10 minutes. The physician cleaned his hands using a 62% ethanol gel, wore radiation protective gloves and disposable sterile gloves, and draped the patient. An anteroposterior fluoroscopic image of the SIJ was obtained. The fluoroscope was angled caudad, placing the posterior superior iliac spine and iliac crest to overlay the joint image, and was oblique to the contralateral side, placing the anterior and posterior aspects of the joint in line. A 23 gauge 6 cm disposable needle was advanced under coaxial technique and intermittent fluoroscopy until the joint was entered. After injection of 0.5 cc of contrast media, we confirmed that the needle was positioned in the capsule by the spread of the contrast media in the SIJ. A mixture of 0.5% mepivacaine 2 ml and triamcinolone acetonide 10 mg was injected. Following the same procedure, SIJB was then performed on the right side. The patient complained of ordinary referred pain on both sides at the time of the contrast media injection. After the injection of the mixture of 0.5% mepivacaine 2 cc and triamcinolone acetonide 10 mg, the pelvic pain on both sides decreased to a VAS score of 0. The patient did not complain of any other symptoms and her blood pressure was within normal limits. She was stabilized in bed for one hour and then discharged.\nThe patient was admitted to the emergency room two days after the final SIJB with chief complaints of left pelvic pain corresponding to a VAS score of 9 and fever (39.0℃). During the physical examination, the patient showed tenderness in the left hip. Blood pressure and heart rate were within normal limits. Laboratory tests showed the white blood cell count to be within normal limits, at 7.68 k/µl, but the neutrophil count was increased to 93.6%. Both ESR, at 35 mm/hr, and C-reactive protein (CRP), at 15 mg/dl, showed an increase. There were no specific findings on chest computed tomography (CT) or abdominal and pelvic CT, with the exception of L3-S1 posterior fusion. CT showed osteophytes and erosion in both SI joints and irregularity in the joint spaces, but there were no particular changes compared to the previous CT. Following admission, the patient was started on vancomycin hydrochloride 1 g intravenous (IV) every 12 hours. Pelvic magnetic resonance imaging (MRI) performed 3 days after the procedure showed high signal intensity edema and enhancement in a T2-weighted image at the left iliacus muscle, leading to a suspected diagnosis of myositis. There was no suspicion of infection or inflammation at the sacroiliac joint (). Methicillin-sensitive staphylococcus aureus (MSSA) was discovered in the blood culture taken 7 days after the procedure. Following this blood culture result, the patient's antibiotic therapy was changed from vancomycin to cefazolin sodium 2 g IV every 8 hours. After 17 days of continuous antibiotic therapy, the patient's ESR increased to 99 mm/hr and her CRP increased to 8.8 mg/dl. Taking these findings into consideration, the antibiotics were changed to ciprofloxacin 400 mg IV every 12 hours and rifampicin 450 mg P.O. every 12 hours. 27 days after the procedure, the patient's left pelvic pain decreased to a VAS score of 3. However, the patient's ESR and CRP remained elevated, at 93 mm/hr and 8.57 mg/dl, respectively, and she maintained a fever of at least 38.0℃ . Forty one days after the procedure, prosthetic infection at the site of the spinal fusion surgery performed 7 years ago was suspected, and 67Ga SPECT/CT was performed. At the fusion site, 67Ga SPECT/CT showed only mild uptake, which appeared to be inflammatory change. In addition, abnormal uptake was seen at the left SIJ, and septic sacroiliitis was suspected (). 58 days after the procedure, CRP normalized to 0.29 mg/dl and ESR decreased to 60 mm/hr, and the patient had no fever. Her pain decreased to a VAS score of 2 with a fentanyl 4.2 mg/10.5 cm patch and oral acetaminophen. The infection was thought to be under control, so the patient was discharged and placed on antibiotics and analgesics. She was directed to follow up as an outpatient. |
A 75-year-old woman underwent PCNL for partial staghorn stone in the left kidney, causing moderate hydronephrosis (). The procedure was performed by experienced faculty urologists under spinal anesthesia, using antibiotic prophylaxis. After retrograde catheterization with a 7F ureteral catheter in the lithotomy position, the patient was turned prone and percutaneous access was established under fluoroscopic guidance after contrast injection through the ureteral stent. Access to the collecting system was achieved by single puncture of posterior lower calix under C-arm X-ray guidance, the site of puncture was 1 cm below 12th rib and 3 cm medial to posterior axillary line to avoid colonic injury (CT scan shows partly retrorenal colon) (). After tract dilatation using coaxial serial Teflon-coated dilators, a 24F Amplatz sheath was placed and a slender nephroscope 17F (Karl Storz) was used for the procedure.\nDuring inspection by nephroscope of the lower calix, there was intense venous bleeding from the injured part of the lower calix and we followed the tract through luminal structure and we suspected the possibility of injury to the structures surrounding the left kidney. Through the nephroscope, contrast material was injected to the abnormal tract, and the left renal vein with two tributaries and inferior vena cava were seen (), consequently direct injury and penetration to the left renal vein was confirmed.\nIntraoperatively, the bleeding was controlled by placing the Amplatz sheath over the bleeding tract. The patient was hemodynamically stable throughout the whole procedure. The procedure continued and the stones were fragmented and removed; stone-free state was documented with C-arm and Double-J stent was inserted.\nAt the end of the procedure, 20F Foley catheter was inserted and used as a nephrostomy tube, its balloon was inflated with 3 mL fluid inside the renal pelvis and pulled back with light pressure to the lower calix to press the site of injury to the renal vein tributaries and then was closed for 24 hours.\nThe patient was kept in the ICU and she remained hemodynamically stable, with clear urine through both urethral and nephrostomy catheter; renal function and hemoglobin were normal. Antegrade pyelography was done on the second postoperative day; there was distally patent ureter with no extravasation, neither contrast leak to renal vein. At third postoperative day, urethral catheter was removed and the patient was discharged home, with nephrostomy catheter in place. |
A 79-year-old male was referred in March 2003 with a lump on the left side of his neck. The patient had noted a sudden increase in the size of the lump over the preceding two months. He did not report any neck pressure symptoms, weight loss or anorexia. His past history included right partial thyroidectomy for a solitary nodule (colloid goitre) in 1978 and repair of abdominal aortic aneurysm in 1994. He had suffered myocardial infarction in 1995 and had an episode of acute coronary insufficiency in January 2003. His recent coronary angiograms showed an occluded left anterior descending artery and echocardiogram revealed good left ventricular function. He was a non-smoker and consumed alcohol in moderation. He had been taking warfarin, diltiazem MR, lisinopril, uniphyllin, glyceryl trinitrate tablets and buccal suscard.\nOn examination he had left sided goitre extending superiorly into the posterior triangle and inferiorly into the retrosternal space, with variable consistency. The trachea was deviated to the right and there was cervical lymphadenopathy on the left side. Systemic examination was unremarkable and fine needle aspiration of thyroid gland showed presence of Hürthle cells. Computerised tomographic (CT) scan with contrast enhancement (figure &) of the neck and thorax revealed large left sided thyroid goitre with significant mediastinal extension. It showed mixed attenuation with foci of calcification peripherally. There was a 3 cm complex mass on the left side of the neck, posterior to the carotid sheath structures and deep to the sternomastoid, indicative of lymph node metastases. Thyroid profile and routine blood investigations were unremarkable.\nBased on the above findings radical surgery was planned. On exploration of the neck we confirmed left goitre with intrathoracic extension and enlarged lymph nodes under the sternocleidomastoid close to the jugulodigastric muscle and surrounding the carotid sheath. There was no remnant thyroid tissue seen on the right side following the previous thyroid surgery. Left hemithyroidectomy with modified neck dissection (lymphadenectomy, preserving all vessels and nerves) was performed. Macroscopic examination of the thyroid lobe showed a well defined solid pale brown mass approximately 8 cm in maximum dimension, surrounded by a narrow rim of preserved thyroid tissue. The lymph node specimen comprised of several nodules of partly necrotic tissue. Microscopic examination showed the thyroid lobe containing a Hürthle cell neoplasm, which was mostly encapsulated, with foci of capsular and vascular invasion. The two lymph nodes revealed metastatic Hürthle cell carcinoma. [pT3, N1a, Mx], (Figure &).\nThe patient had adjuvant therapy with oral radioiodine 131 (3060 MBq Sodium Iodine). He was put on a daily dose of 100 mcg of thyroxine. This was to be followed by a second dose of 5911 MBq of radioactive iodine six months from the time of the first dose. |
The patient is a 69-year-old male with a medical history of diabetes, hypertension, and a remote history of several bouts of pancreatitis who presented to clinic with a 6-month history of steatorrhea and an unintentional 20 lb weight loss. During this visit, the patient reported an operative history of an open cholecystectomy by a right paramedian incision, and an appropriate scar was noted on physical examination. No formal records were available. Workup with CT imaging revealed a 2.1 × 7.0 cm multicystic lesion with mural nodularity in the body of the pancreas () and diffuse pancreatic ductal dilation throughout (). Endoscopic ultrasound and the fine needle aspiration (FNA) measured the duct at 12.6 mm in the head, 14 mm in the neck, and 20 mm in the body, and after endoscopic tissue retrieval, the pathology returned as a mucinous neoplasm with focal atypia. A working diagnosis of IPMN was made. The patient's serum CA-19-9 was borderline elevated at 37 U/mL.\nThe patient described met both Sendai and Fukuoka criteria and so was taken to the operating room for definitive treatment. In the operating room, the pancreas was found to be grossly abnormal with diffuse distortion of the duct throughout the head, body, and tail, thus warranting a pylorus-preserving total pancreatectomy and en-bloc splenectomy to safely remove all of the neoplastic tissue. During the procedure, a Roux-en-Y limb was discovered that had previously been brought up to the head of the pancreas in the antecolic position ( left). Intraoperative discussion among the surgical team concluded that this had presumably been performed to drain what may have been a pancreatic pseudocyst identified at the time of the patient's prior recurrent pancreatitis attacks. This was later confirmed upon postprocedural discussion of findings with the patient.\nIn the operating room, the decision was made to divide the Roux-en-Y limb preserving most of its length and its mesentery. The required pylorus-preserving total pancreatectomy was then safely performed. The cranial portion of the Roux-en-Y limb was used to perform the standard biliary–enteric reconstruction as an end-to-side hepaticojejunostomy, and alimentary reconstruction was completed by standard end-to-side duodenojejunostomy ( right) to the proximal most jejunum in an antecolic manner. The patient tolerated the procedure well.\nFinal surgical pathology revealed a 5.5 cm IPMN with low-grade dysplasia and surrounding chronic pancreatitis, as well as a 0.4 cm well-differentiated Ki-67 positive pancreatic endocrine tumor with Ki-67 proliferative index <1%. The patient's postoperative course was uneventful and he was discharged home on postoperative day 5. He was seen in the outpatient setting at a routine 1-month follow-up visit, at which time he was found to be in excellent condition and spirits, and overall, doing well. |
A 40-year-old male not known to have any chronic medical illness, presented complaining of epigastric and left upper quadrant pain for 1 month, associated with intermittent nausea and vomiting, and aggravated by fatty meals, with no other associated symptoms. He had frequent visits to the emergency department where he was managed with analgesia and antacids with mild symptomatic improvement. Clinical examination was unremarkable with no evidence of jaundice or abdominal tenderness. His blood test results showed a normal complete blood count, kidney function, and liver function. Chest X Ray revealed dextrocardia with stomach fundic gas shadow on Right side (). Abdominal ultrasonography revealed transpositioning of the solid organs with a left sided liver and gallbladder with a solitary stone and mild wall thickening. We elected to perform a Magnetic Resonance Cholangiopancreatography to delineate the anatomy and to rule out any anomalies within the biliary tree. It confirmed the previously noted findings, showed no evident anomaly within the biliary tree, and confirmed the diagnosis of situs inversus totalis (, ). The patient was Scheduled for an elective laparoscopic cholecystectomy.\nThe Operating room equipment arrangement was adjusted as Mirror Image of Routine Laparoscopic cholecystectomy (). The Monitor was placed on left side of the patient. The surgeon with the camera assistant were on right side of the patient and the first assistant was on left side of the patient. The abdomen was scrubbed and draped in the standard aseptic technique. The first infraumbilical 11 mm trocar introduced and pneumoperitoneum induced using the open technique. Three 5 mm trocars were placed, at the xiphisternum which was used for the surgeon’s left hand, at the left midclavicular line 2 cm below the costal margin which was used as working port for the surgeon’s right hand and at left anterior axillary line 5 cm from the costal margin which was used for retraction of the gallbladder fundus by the second assistant, respectively. Inspection of the abdominal cavity confirmed the presence of situs inversus totalis, with the liver and the gallbladder positioned in the left side (). The Calot’s triangle was identified. The peritoneum overlying the gallbladder infundibulum was then incised and the cystic duct and cystic artery identified and circumferentially dissected, till the critical view was obtained. The cystic duct and cystic artery were then doubly clipped and divided, through the subcostal port using the right hand. The gallbladder was dissected from its peritoneal attachments using electrocautery and was retrieved using Endoscopic bag through the infraumbilical port. The total operative duration was 80 min, which was longer than the conventional laparoscopic cholecystectomy performed in patient without underlying anatomical variation. It can be attributed to the modification in the technique required to adjust to the mirror image anatomy.\nThe patient had an uneventful postoperative course and was discharged on postoperative day 1. Pathological examination of the gallbladder confirmed the presence of gallstones with chronic cholecystitis. No postoperative complications were noted during his follow up in the outpatient department. |
Patient IM, a 43-year-old French-speaking and right-handed female first consulted a neurologist due to headaches with neck pain. The magnetic resonance imaging (MRI) scan performed in 2005 showed lesions in the left parietal-occipital junction and in the left superior cerebellar presumptively of vascular origin. At the time of testing, patient IM took anticoagulants and medication for arterial hypertension. Patient KV was a 60-year-old female and retired employee at the time of testing. She was bilingual in French and Dutch and a forced right-hander (she wrote with the right hand since childhood). She suffered from a cerebral anoxia in 2007 (cardiac arrest during a thyroidectomy) which affected the left frontal and the bilateral parieto-occipital regions. At the time of testing, she took an antidepressant as well as thyroid and insulin medication. The two patients had no history of drug or nicotine use or alcohol abuse. The two patients had no known common medical history prior to their neurological incident. The neuropsychological profile of both patients is presented in .\nDuring the first clinical interviews after their cerebral damage (more than 5 years prior to our testing), the two patients complained mainly of difficulties in memory and fatigue. Their spontaneous complaints, although still present, were significantly reduced at the time of testing (as corroborated by the improvement in the neuropsychological tests, ). They had no spontaneous complaints about their social interactions and they reported healthy relationships with their family, friends, etc. Their global scores at a questionnaire assessing cognitive and affective empathy (French translation of the Questionnaire of Cognitive and Affective Empathy, a QCAE from ) was within the norms both for the cognitive empathy subscale (patient IM = 57; patient KV = 68; controls’ mean = 59.42, s.d. = 7.59) and for the affective empathy subscale (patient IM = 42; patient KV = 39; controls’ mean = 36.76, s.d. = 5.06). Note that these were only self-reported measures (we were not able to collect data from heteroevaluation). When asked more specific questions about their daily life social interactions, both patients reported however more frequent misunderstandings of what other people meant or intended to do since their neurological incident. Such misunderstandings were reported when the meaning or intention was implicit rather than explicit. |
A 38-year-old Caucasian male patient presented to our dental office with a 1 year history of recurrent swelling on the buccal aspect of lower right mandibular region. He reported pain/tenderness on chewing over the past year. Medical history was unremarkable and there was no contraindication to dental treatment. There was no history of trauma to the teeth and jaw; a previous dental root treatment in the lower right region was reported.\nClinical examination revealed that the first right lower molar was tender to percussion and had a large coronal restoration; the adjacent premolar and molar teeth were vital and intact with no signs of caries or anomalies such as occlusal tubercle. The buccal soft tissue of lower molar teeth was normal. The probing depths were within normal limits. The molar tooth had no mobility. Radiographic examination showed a large circumscribed radiolucency at the apex the mesial root of first mandibular molar (). However, lamina dura surrounding the root of second premolar was apparent circumferentially. Further history revealed that the molar tooth had been endodontically treated 5 years ago and restored with an amalgam restoration. The obturation was deemed inadequate and a diagnosis of persistent AP due to inadequate root canal filling of lower right first molar was ascertained. After full explanation the patient consented to nonsurgical endodontic retreatment, followed by coronal restoration with amalgam.\nFirst, patient was asked to rinse with 0.2% chlorhexidine mouthwash. After administering local anesthesia, and isolation with rubber damn, the amalgam restoration was removed using a water-cooled high-speed fissure bur and straight line access to the root canals was made. Working length was determined radiographically, confirmed by electronic apex locator and using hybrid instrumentation (mixed technique) the canals were shaped and cleaned; copious irrigation with 5.25% NaOCl solution was carried out. Canals were dried and obturated by lateral condensation technique with gutta-percha and endodontic sealer (Roth's Grossman Type 801, Roth International LTD., Chicago, IL, USA). Post-treatment occlusal reduction and adjustment did not carry out and access cavity was temporized with Cavit (). Next day the patient suffered from severe pain/swelling and analgesics/antibiotics were prescribed. The patient was asymptomatic after one week and the tooth was permanently restored with amalgam.\nThe patient returned 6 weeks later complaining of episodes of pain and local buccal abscess particularly in the last three weeks. Clinical examination revealed that the buccal soft tissue of mandibular right region was inflamed/swelled and tender to palpation. After investigation with thermal and electrical tests, the pulp of lower right second premolar was deemed nonvital. A test cavity in the absence of local anesthesia confirmed dental pulp death. One-visit nonsurgical endodontic treatment was carried out similar to the previous case (). Antibiotics were not prescribed. At the 7-day follow-up, signs and symptoms completely resolved.\nRadiographic examination at the 6-month recall demonstrated gradual reduction of the periradicular radiolucency (). At the 12-month recall examination, the tooth was asymptomatic, and the region of periapical radiolucency was remarkably reduced (). Two-year follow-up revealed healthy periodontal tissue with normal probing depths, no signs or symptoms of inflammation and infection. Radiographically, the periapical lesion was completely healed (), signifying endodontic treatment success. |
A 75-year-old woman was admitted to our intensive care unit for clinical monitoring after right parotid gland removal; she was diagnosed with a colliquative tumoral parotid gland mass in a peripheric hospital and then moved to the local otolaryngological surgical department to undergo surgery.\nHer past history included hypertensive cardiomiopathy, with episodes of heart failure, and atrial fibrillation.\nOn admission to our unit she underwent a chest radiograph that showed a wrong positioning of the CVC (arrow 7 Fr, 3 lumen, and 16 cm length): as shown in the tip projected over the left side of the descending aorta, at the level of carina, creating an angle with the spine greater than 40° []. Moreover, we noticed saline leak from the insertion site and suspected a catheter rupture. In addition we performed a vascular ultrasonography that showed a complete thrombotic occlusion of the left internal jugular vein.\nFor these reasons and for the timing of catheterization (28 days before) we decided to remove it.\nThe patient was mechanically ventilated with assisted mode, and she was placed in the head-down position before removing the catheter. Resistance was met during the removal of the catheter; after its dislodgement, local pressure using a gauze was performed to avoid bleeding. At inspection the catheter was whole, and a brownish scab on its distal tip compatible with a blood clot was found.\nAfterwards she developed hemodynamic instability (blood pressure 86/40 mmHg, heart rate 100 bpm) that required adding inotropic support (dopamine 6 mcg/Kg/min) and deterioration of ventilation (PaO2/FiO2 146).\nDue to no evidence of recovery, the next day we performed an echocardiogram and noticed air microbubbles in the right heart chambers (); these were synchronous with breaths and were apparently coming from superior vena cava. On the hypothesis of a vein-to-dermis fistula made while removing the catheter, the insertion site was sealed with air occlusive dressing with no more evidence of air bubbles within the heart.\nA neck and thorax CT scan was performed, showing total thrombotic occlusion of the left internal jugular vein extending to the innominate vein and partially involving the left subclavian vein, with air bubbles within it (). A diagnosis of venous air embolism associated with removal of central venous catheter was then made.\nAt a second vascular ultrasonography we noticed the presence of a tract between the vein and the dermis (), responsible for air embolism, which was still visible more than 24 hours after air occlusive dressing placement. |
A right-hand-dominant male patient aged 61 years with insulin-dependent diabetes (height 177.8 cm; weight 104 kg; body mass index 33.4) was referred by an infectious disease specialist (JDT) to our clinic for the surgical management of a recurrent deep infection of the left shoulder. The patient worked as a commercial property developer and had only recently started using insulin for diabetes. Poor glycaemic control was clear from his self-reported history and a high haemoglobin A1C (12.8%; normal is <5.7%). He denied having any other medical problems and he did not drink alcoholic beverages or smoke. As detailed below, about 4 months prior the patient had two arthroscopic debridement procedures and completed an 8-week course of antibiotics for a P. acnes infection of his left shoulder region. When he was first seen in our clinic for the recurrent infection, 2 months had passed from the completion of the 8-week course of intravenous and oral antibiotics.\nHis left shoulder problems started approximately 11 years prior. At that time he was not diabetic and had a successful and uncomplicated open repair of a torn supraspinatus tendon. In September 2015, he had a second left shoulder surgery for impingement symptoms, and this included an arthroscopic acromioplasty with debridement of suture material from the prior cuff repair. Around that time, metformin was prescribed for new-onset diabetes, but he was not compliant with taking this medication. A subacromial corticosteroid injection was given 7 months (April 2016) later for persistent pain and progressively reduced motion. Three weeks after the injection, a large abscess had formed and it extended from the glenohumeral joint and subacromial space to the subcutaneous tissue over the acromion. His blood glucose was 340 (normal: 65–99). An aspiration of purulent fluid grew P. acnes in fortified thioglycolate broth culture in anaerobic conditions. In addition to two surgical debridement procedures, antibiotic treatment by his orthopaedic surgeon initially included a 10-day course of intravenous vancomycin and oral levofloxacin (750 mg/day). An infectious disease specialist (JDT) who was then consulted discontinued these antibiotics and started a 6-week course of intravenous daptomycin. Although penicillin is often the antibiotic treatment for this type of infection, it was not used because he was allergic to it. |
An 84-year-old postmenopausal white female (gravida 3, para 2) was referred to our urogynecology clinic for evaluation and treatment of a persistent rectal bulge () (). She had noticed this bulge for over 10 years and initially thought that it was a hemorrhoid. She had been evaluated by a colorectal surgeon 10 years prior to presentation who recommended an abdominal resection rectopexy. The patient elected to not pursue surgery at that time. She described a bulge at the anus with the size of a lemon which worsened with physical activity and reported accidental leakage of bowel contents consisting of pellet-formed stool. She was sometimes unable to differentiate between the bulge and stool. She reported significant adverse impact on her quality of life, including impairment of her ability to mow her lawn or to exercise. Additionally, she reported anal laxity, constipation, and occasional bright red blood per rectum. She denied any urinary symptoms or vaginal prolapse.\nPelvic exam revealed an atrophic vagina with no significant anterior or posterior vaginal wall prolapse. Her cervix was absent surgically, and her pelvic organ prolapse quantification was as follows: GH 2, PB 2, Aa -3, Ba -3, Ap -3, Bp -3, C -7, D n/a, and TVL 7. Rectal exam showed normal perianal skin and sphincter tone. With Valsalva, she demonstrated significant anterior rectal wall prolapse with palpable contents consistent with small bowel enterocele in the rectovaginal space. Prolapse was approximately 6 cm in size with the anterior rectal wall extending outside the anal verge and no evidence of posterior or circumferential rectal prolapse. Endoanal ultrasound showed normal levators, intact external anal sphincter circumferentially, and attenuation of the internal anal sphincter. There were no discernible masses, but the patient was unable to produce her rectal prolapse with the ultrasound transducer in place. Transperineal ultrasound was used to dynamically evaluate the perineal and rectal anatomy and demonstrated a large enterocele prolapsing between the vagina and the rectum with apparent fat or small bowel contents. Prolapse at the anterior rectal wall was directly associated with this enterocele. Defecography and dynamic MRI were not available at our facility.\nThe patient was evaluated by a colorectal surgeon for opinion on treatment plans. This surgeon indicated that a rectopexy would be acceptable, but it would be reasonable to attempt to repair the enterocele first to see if her symptoms would resolve and to potentially spare the patient from a more morbid procedure. The patient indicated a desire to proceed with an attempt at repair with the vaginal approach.\nThe patient was admitted to the hospital and anesthetized for surgery. A vertical posterior vaginal incision was made, extending into the perineal body. The vaginal epithelium was separated from the underlying endopelvic fascia, and dissection was performed superiorly and laterally to the level of the ischial spines and levator ani. A large enterocele was identified, opened, ligated with two purse-string sutures of delayed absorbable monofilament suture, and then excised (Figures and ). A suture capturing device was used to pass a polypropylene suture through the levator tendon immediately distal to the ischial spine bilaterally, following which these sutures were attached superiorly and laterally to the rectovaginal septum completing the iliococcygeus suspension in a tension-free manner (Figures and ). Small site-specific rectovaginal septal defects were repaired. The vaginal epithelium was then closed, and the perineal body was reconstructed.\nPostoperatively, the patient recovered well and was discharged home on the first postoperative day with complete resolution of her rectal prolapse symptoms. She was seen at 1 month and 3 months postoperatively and by 3 months had resumed her normal activities of daily living with complete symptom resolution and normal defecation without incontinence. |
A 39-year-old Caucasian woman presented with a new and persistent cough. Radiographic imaging of her chest demonstrated a mass in the upper lobe of the right lung. Fine-needle aspirate biopsy demonstrated adenocarcinoma. Genomic testing showed the patient’s tumor to be EGFR-mutation positive with exon 19 deletion. She was referred to a comprehensive cancer center for treatment. Her initial computed tomography (CT) scan suggested possible mediastinal invasion at the level of the right brachiocephalic vein. Therefore, she was treated with neoadjuvant afatinib therapy prior to possible surgical intervention. The patient agreed to participate and was explained the nature and objectives of this study, and informed consent was formally obtained. No reference to the patient's identity was made at any stage during data analysis or in the report.\nThe patient began developing small papules within 24 hours of starting afatinib and by the fourth day of therapy was experiencing severe dermatologic toxicity. Lesions initially appeared on the face, neck, and chest. Individual lesions became confluent and involved 80% of the affected areas. The patient noted her lesions to be extremely pruritic. Her oncologist prescribed oral doxycycline 100 mg twice daily, topical treatment to her skin lesions with clindamycin 1% gel twice daily, and hydrocortisone 2.5% cream.\nThe patient’s cutaneous symptoms and lesions continued to progress rapidly during the next several days. She was severely debilitated to the point of considering discontinuation of afatinib therapy. Therefore, her oncologist referred her to the dermatology clinic for evaluation and treatment of the drug-associated skin toxicity. After obtaining written consent from the patient, photos of all of the affected areas were taken.\nCutaneous examination after starting afatinib revealed diffuse erythema with individual and confluent papules and pustules on the forehead, face, neck, chest, upper abdomen, and upper back (Figures -).\nHer forehead lesions also had superficial scaling, and the lesions on the nose were crusted (Fig ). There was also diffuse involvement of the scalp. No other hair, nail, or other mucocutaneous lesions were observed.\nTherapeutic intervention included oral dexamethasone 4 mg daily for seven days and doxycycline 100 mg twice daily. The clindamycin gel was discontinued since it had elicited severe irritation and pain; topical triamcinolone 0.1% ointment was applied to the face and neck twice daily, and clobetasol propionate 0.05% cream was applied to the chest and back twice daily. To alleviate her pruritis, she received oral fexofenadine 180 mg each morning as well as hydroxyzine 50 mg each evening. During the seven-day course of dexamethasone, she stopped afatinib for three days and then resumed therapy at a 30 mg dose--a 25% reduction from the initial dose of 40 mg.\nFollow-up examination one week later showed significant improvement in both skin symptoms and lesions. Her skin pain and pruritis had resolved. There were both a resolution of the pustules and a flattening of the papules (Figures -).\nErythema persisted on the affected skin areas. Forehead scaling had also resolved, and the crusted lesions on her nose had cleared (Figure ).\nImprovement of the cutaneous toxicity allowed the patient to complete the four-week course of afatinib therapy. A repeat CT scan following the EGFR inhibitor therapy showed a dramatic decrease in the size of the tumor. Additionally, there was no longer any vascular involvement of the right brachiocephalic vein.\nSurgical excision of the upper lobe of the right lung and multiple lymph nodes was performed. Pathology demonstrated complete removal of the tumor and showed absence of tumor in any lymph nodes. Within one month of discontinuing afatinib, the patient’s skin lesions completely resolved, and she was able to discontinue all oral and topical therapy.\nThe patient’s case was reviewed by the cancer center tumor board; the benefit of adjuvant chemotherapy following the successful resection of her tumor was found to be less than 2%. Therefore, the tumor board recommended observation. She will be closely monitored with re-staging every three months. |
A 64-year-old male presented with a two month history of difficulty urinating and was found to have a fungating penile mass involving 50% of his penis. The mass was hard and fixed and extended from the glans proximally up the shaft. He also had bilateral palpable inguinal lymphadenopathy. There were no associated constitutional symptoms. Given there was a high suspicion for malignancy, the patient underwent partial penectomy within a month of presentation. Biopsy results confirmed a pT2 tumor with invasive keratinizing squamous cell carcinoma, poorly differentiated, and tumor size of 5 × 4 × 2.5 cm, with corpus spongiosum and lymphovascular involvements.\nFollowing the procedure, the patient had PET-CT for staging, and imaging revealed enlarged hypermetabolic bilateral axillary lymph nodes concerning for metastatic disease. In addition, there was a large centrally necrotic lymph node conglomerate in his left groin that had increased FDG avidity. The patient had left inguinal and bilateral pelvic lymph node dissections revealing metastatic squamous cell carcinoma in multiple lymph nodes. The left inguinal mass was also found to be metastatic well-differentiated SCC. His diagnosis was staged at T2N3M0.\nAfter his surgical procedures, patient was started on adjuvant chemotherapy. He began first line chemotherapy with paclitaxel, ifosfamide, and cisplatin (TIP). He underwent 4 cycles of TIP but eventually developed disease progression on repeat imaging. At this point, the patient was started on cetuximab given EGFR amplification on tumor analysis with the FoundationOne testing platform. However, the patient had an allergic reaction to cetuximab, so his treatment was changed to panitumumab. The patient had stable disease and a progression-free survival of 6 months with anti-EGFR treatment, which is clinically significant given that this treatment was given in the second-line setting for an aggressive tumor type that other than chemotherapy there is no other approved drug to date.\nThe patient was ultimately started on the PD-1 inhibitor nivolumab. He had initial response to immunotherapy followed by stable disease, so he had a disease control rate of an additional 6 months with this investigational agent at that time. Ultimately, he was placed on hospice and passed away two years from the the time of diagnosis. |
A 38-year-old Caucasian male with Goltz syndrome presented for elective surgery for removal of a left thigh papillomatous mass. He was born with webbed fingers and toes, as well as “splotchy skin.” He was diagnosed with Goltz syndrome with skin manifestations as an infant. In his late 20s, he started developing warts and skin tags over his body, and, later in his 30s, he started developing a papillomatous mass on his left inner thigh. His wife reported that the patient has been snoring for about 4 years; however, his snoring had significantly improved in the last 6 months. Other past medical history included obesity with a BMI of 43 and uncontrolled hypertension. Surgical history included a tonsillectomy as a teenager with no mention of anesthetic problems. He denied dysphagia and shortness of breath. Upon airway examination, he had a Mallampati score of 1 and full range of motion of his neck and mandible. The patient's physical examination was significant for a scattered, reticular rash across his chest, as well as a large papillomatous skin mass on his left inner thigh. Induction of anesthesia was accomplished with fentanyl, lidocaine, propofol, and rocuronium. A two-handed mask ventilation technique was required due to his body habitus, though no signs of airway obstruction were noted. Oxygen saturation during mask ventilation was maintained at 100%. Direct laryngoscopy was attempted with Macintosh 4 blade, a grade 2 laryngeal view was obtained, and a mass at the base of the tongue was visualized pulling the vocal cords to the right and obstructing the view of the vocal cords. A glidescope was then used in order to better visualize the mass. With optimal visualization, a friable, large, papillomatous mass was noted at the right base of the tongue (Figures and ). The trachea was intubated with an 8 mm endotracheal tube. General anesthesia was maintained with sevoflurane, and the otolaryngology service was consulted intraoperatively for evaluation of the tongue mass. A small biopsy was taken, and then the surgical removal of the soft tissue mass on his thigh was performed. Upon completion of the thigh mass removal, the otolaryngology serviced returned for debridement of the tongue lesion to obtain a more clear view of the larynx and vocal cords. Upon completion of the debridement, the epiglottis and surrounding soft tissues were noted to be edematous. It was therefore decided to admit patient to the surgical intensive care unit and leave the trachea intubated overnight to safely allow the swelling to subside. After completion of three doses of dexamethasone 8 mg in the surgical intensive care unit, the trachea was uneventfully extubated and the patient was discharged home on postoperative day 2.\nThe pathological report for the debrided mass was interpreted as papillary lymphoid hyperplasia. The patient presented back to the operating room three weeks later for an elective removal of the remaining obstructing lesions []. He was electively extubated the next day in the surgical ICU, just as the previous procedure, to allow for airway edema recovery. At a follow-up clinic visit with otolaryngologist one week later, the patient was breathing well, with no areas of concern for residual or recurrent pharyngeal disease. |
A 60-year-old female known case of hypertension and diabetes on treatment had undergone bilateral total knee replacement in 2002. The patient was treated with oral bisphosphonate therapy for osteoporosis, 70mg alendronate once a week orally from 2003 to 2007 (4 years) and then from 2009 to 2013 (4 years). The patient presented to the hospital in January 2013 with pain in the right side of the jaw and pain during mastication since 6 months. From her history, clinical examination and radiological findings she was diagnosed to have osteonecrosis of her right posterior body mandible (). This was treated by way of sequestrectomy and saucerization in January 2013 () and stoppage of Alendronate. There was no history of the patient having any dental procedure while on alendronate. In March 2013, she was admitted with complaints of pain and swelling over the right thigh following a history of trivial fall, and after clinical examination and history taking, X-rays were done which showed an atypical right displaced subtrochanteric fracture through the focally thickened lateral cortex of proximal femur (). The fracture was managed surgically with proximal femoral nailing (). The knee replacement was unrelated to the sub-trochanteric fracture. For the subtrochanteric fracture of femur, regular follow-ups were done postoperatively with serial X-rays. Blood investigations were done, and serum calcium was 9.5mg/dl, and serum Vitamin D3 was 20ng/ml which was optimized with 6,00,000 IU of intramuscular Vitamin D3 and maintained by oral Vitamin D3 medications. At 3 months, the fracture was in delayed union (), and hence, the patient was started on injection Teriparatide 20mcg subcutaneous for a period of 18 months. The patient was kept non-weight bearing. The 6 months X-ray showed further progression in fracture healing (), and the patient was allowed partial weight bearing. In October 2013, 10 months after stopping Alendronate, she again came with the complaint of pain in the right side of the jaw; X-rays showed relapse of ONJ () sequestrectomy was done on the right mandible. In February 2014, 13 months after stopping Alendronate, the patient came with the increased pain of the right mandible and was diagnosed as a fracture of the right mandible; the fracture was operated on and fixed with a plate in February 2014 (). Meanwhile, the patient continued injection Teriparatide. At 1year postoperatively, the atypical femoral subtrochanteric fracture had healed () and the patient was allowed full weight bearing. 24 months postoperatively, X-rays confirmed solid union of the atypical subtrochanteric femoral fracture (). On recent review, the patient walks full weight bearing without the need of any support, with a normal gait and is completely asymptomatic as regards the femur as well as the jaw. |
An 86-year-old female presented with a swelling over the back in left lateral aspect since 5 years, which was initially small in size, reducible, painless, and gradually increased. The swelling had become irreducible and associated with pain since last 2 months. There was no other significant history in the form of trauma, previous surgery, and chronic infections like tuberculosis or altered bowel habits. On examination at admission, there was 15 × 12 × 10 cm, non-tender, non-pulsatile, irreducible swelling, soft in consistency in the left lumbar region with an expansile cough impulse. There was congenital right scoliosis [].\nExamination of right flank, rest of the abdomen and other hernial orifices was normal. Plain radiograph of the abdomen revealed a gas shadow within the swelling. Ultrasonography revealed well-defined, elongated and rounded mixed echogenic masses of about 3.7 × 2.2 × 1.4 cm and about 2 cm diameter in the left lumbar region, which appeared most probably to be herniated bowel loops. Computed tomography (CT) scan of the abdomen revealed 3 cm defect in the left lumbar region at the level of L3 vertebra, with a hernial sac containing large bowel loops, confirming the presence of left lumbar hernia containing large bowel. A diagnosis of irreducible left lumbar hernia was made and the patient was taken up for the surgery after she was examined for anesthetic fitness. The patient was placed in the right lateral decubitus position and the operating table was angulated to achieve the same effect as a kidney rest. The left flank incision was given over the swelling for exploration. The sac was herniating through the superior lumbar triangle and was covered by the muscles forming the boundaries of the triangle []. There were dense adhesions around the sac which had made it irreducible. There were no features of strangulations.\nThe dense adhesions were released meticulously and the sac was reduced completely and intact. A sheet of polypropylene mesh was fashioned as inlay prosthesis and was placed in the extraperitoneal space by umbrella placement technique through the defect in the muscle layer [Figures and ]. The defect was repaired with prolene continuous suturing and the rest of the posterior abdominal wall was closed in layers.\nThe immediate postoperative period was uneventful. Oral sips were started after 4 hours of operation. The patient was discharged after removal of sutures on 10th postoperative day, with a healed scar. On follow-up visit after 2 weeks, the patient was absolutely asymptomatic with a well-healed operation scar. |
A fifty-one year old woman presented with weight loss and vague epigastric pain. Three years previously, she had undergone a wide local excision and axillary dissection for a right sided breast lump. Postoperative histology showed a completely excised grade II invasive carcinoma (T2) with associated intermediate grade DCIS. On immunohistochemistry, the tumour was found to be positive for both oestrogen and progesterone receptors but negative for E-cadherin, suggesting a predominantly lobular tumour type; all eight excised lymph nodes were clear of metastases (N0). She had subsequently received adjuvant radiotherapy to the breast. As she was unable to tolerate either tamoxifen or anastrozole due to side effects (persistent hot flushes), surgical oophorectomy had been performed. She required tibolone to treat menopausal symptoms. She had been regularly reviewed in breast clinic without any clinical evidence of recurrence.\nShe was initially investigated with an upper gastrointestinal endoscopy. This demonstrated only small antral polyps, which were biopsied (Figure ). Histology revealed gastric mucosa infiltrated by poorly differentiated adenocarcinoma of a signet-ring pattern, with immunostaining negative for oestrogen, progesterone receptors and Her-2. Due to the unexpected nature of these findings, a further upper gastrointestinal endoscopy was performed with repeat biopsies from both proximal and distal stomach which, again, were consistent with signet-ring type adenocarcinoma (Figure ). Computed tomography (CT) could not define a definite primary gastric cancer; there was no evidence of lymph node or metastatic spread (Tx N0 M0).\nThe patient was counselled for radical curative surgery in view of an apparent localised primary gastric cancer. A D2 total gastrectomy with Roux-en-Y reconstruction was performed and the patient made an uneventful postoperative recovery. Post operative histology revealed a poorly differentiated adenocarcinoma of the stomach, invading into the duodenum, with 40 out of 41 lymph nodes involved (pT4 N3 Mx). Immunohistochemistry performed on the stomach and lymph nodes was positive for CK7 and GCDFP (Gross Cystic Disease Fluid Protein) (Figure ) and negative for CK20, E-cadherin and Her-2. The tumour in the lymph nodes, but not the stomach, was strongly positive for both oestrogen and progesterone receptors. Consequently, the diagnosis was revised to metastatic breast cancer. The patient was considered for further hormonal therapy. Subsequently she developed widespread bony metastases. She is currently receiving bisphosphonates and palliative care. |
A 68-year-old Japanese man with a history of distal partial gastrectomy for gastric cancer 10 years earlier was admitted for surgical treatment of intrathoracic esophageal cancer (T3, N2, M0, stage III). He underwent subtotal esophagectomy with three-field lymph node dissection and removal of the remnant stomach with the abdominal lymph nodes. The alimentary continuity was reconstructed with a pedicled jejunal limb through the antethoracic route. When we separated the diaphragm from the esophagus and removed xiphoid surgically to prevent a pedicled jejunal limb injury, the pericardium was opened. Anastomosis of the esophagus and jejunum was carried out instrumentally with a circular stapler. A postoperative enteral contrast examination showed smooth passage and no deformity of the reconstructed jejunum. The patient was discharged about 4 weeks postoperatively. About 6 months after the esophagectomy, the patient was readmitted to our hospital because of abdominal discomfort and vomiting. His vital signs were stable and unremarkable. He was thin, with a body mass index of 18.6 kg/m2. Physical examination revealed that the pedicled jejunum through the antethoracic space was markedly dilated and the abdomen was soft and flat (Fig. ). Laboratory data showed only leukocytosis with no other remarkable findings. A chest roentgenogram revealed an increased cardiothoracic ratio of 70%, and an enteral contrast study showed a bird’s beak deformity. The swallowed barium remained static in the reconstructed jejunum. Computed tomography of the thoracoabdominal region showed the reconstructed jejunum within the pericardium anterior to the heart (Fig. ). We diagnosed the patient with IPDH after esophagectomy and performed an emergency laparotomy. Upon opening the abdominal cavity, we found that the reconstructed jejunum had herniated into the pericardium through an approximately 4-cm-diameter diaphragmatic defect without a hernia sac (Fig. ). The herniation was easily reduced, and the congestion of the incarcerated jejunum resolved. There was no evidence of bowel ischemia. Primary closure of the diaphragmatic defect was accomplished using vertical mattress sutures with 1–0 silk (Fig. ). Because the diaphragm adhered to the pericardium around the diaphragmatic defect, we closed these together. Moreover, to reinforce the closure of the diaphragmatic defect, we used a graft harvested from the rectus abdominis posterior sheath. Interrupted sutures with 3–0 nylon were placed to fix the 8 × 6 cm graft of the rectus abdominis posterior sheath to the diaphragmatic defect, preventing recurrence of the hernia (Fig. ). Postoperatively, an upper gastrointestinal study confirmed free flow of contrast medium from the cervical esophagus into the intra-abdominal jejunum through the pedicled jejunum. The postoperative course was uneventful, and the patient was discharged on the seventh postoperative day. |
A 61-year-old Caucasian female with a prior diagnosis of PBC was diagnosed with T3N1M0 stage III, grade 2 left breast invasive ductal carcinoma in October 2011. The tumor was 7 × 4 cm, and a palpable left axillary lymph node was noted on examination. The tumor was positive for estrogen receptor, progesterone receptor, and HER-2 oncogene overexpression (fluorescence in situ hybridization ratio 9.91). Interestingly, her twin sister also had a diagnosis of PBC; other family history included lymphoma in maternal cousin and colon cancer in maternal uncle. Her case was presented at our institution's tumor board and neoadjuvant chemotherapy was recommended.\nDue to the patient's underlying PBC, she had multiple baseline abnormalities of liver function tests, alkaline phosphatase [two times the upper limit of normal (ULN)] and grade 2 cytopenia from splenomegaly. Therefore, anthracycline-based regimens were avoided due to a concern for decreased clearance of doxorubicin leading to increased cardiotoxicity, as the patient would require trastuzumab as well. Neoadjuvant chemotherapy with 6 cycles of docetaxel, carboplatin, and trastuzumab (TCH) was given followed by bilateral skin-sparing total mastectomy and left axillary lymph node dissection with immediate reconstruction. Docetaxel was initiated with a 25% dose reduction and required a subsequent 25% dose reduction on carboplatin due to cytopenia. Postoperatively, pathology demonstrated a decrease in the tumor mass from 7 × 4 to 2.5 × 2 cm, but 4 of 20 axillary lymph nodes were positive for invasive cancer with extracapsular extension (T2N2a). Adjuvant radiation therapy was given to the chest wall and axilla (5,000 Gy). She then completed trastuzumab maintenance for a total of 52 weeks in October 2012 and started adjuvant endocrine therapy with anastrozole.\nIn February 2013, 3 months after the completion of trastuzumab maintenance therapy, our patient presented with skin nodules, and the clinical examination was remarkable for 2 erythematous left upper chest wall nodules measuring 0.5 cm. Restaging positron-emission tomography-computed tomography demonstrated an increased activity in the anterior mediastinum and subcutaneous tissue along with medial left breast reconstruction area concerning for tumor recurrence. There was no evidence of distant/metastatic disease except for underlying cirrhosis of liver, portal hypertension, and splenomegaly. Punch biopsies of the skin nodules revealed invasive adenocarcinoma significant for a small area of lymphovascular invasion. Immunohistochemical stains for AE1-AE3 were positive, highlighting tumor cells, and CD31 was positive, highlighting the lymphovascular invasion. Cutaneous metastases were estrogen receptor negative, progesterone receptor negative and HER-2 positive. She required a bone marrow biopsy due to worsening of cytopenia, which revealed no evidence of breast cancer or other hematological malignancies.\nThe patient's unique presentation and complex comorbidities were discussed at the multidisciplinary tumor board. Based on the team's consensus, the patient was started on docetaxel, pertuzumab, and trastuzumab for 6 cycles, followed by pertuzumab and trastuzumab as maintenance therapy. Docetaxel was re-initiated at a 25% dose reduction (65 mg/m2). The patient's bilirubin was within normal limits; however, the patient's alkaline phosphatase was 1.6 times ULN, warranting a dose reduction. Repeat imaging was negative for systemic disease, except for the primary recurrence of cutaneous metastases. Unfortunately, she experienced a second recurrence of cutaneous metastasis while she was on anti-HER-2 therapy, for which she underwent surgical excision followed by radiation therapy (6,000 Gy). Our patient experienced multiple cutaneous recurrences over the period of the next 24 months. Due to abnormalities in liver function tests (bilirubin was over 2.5 times ULN), multiple anti-HER-2-based regimens were contraindicated, including trastuzumab emtansine (TDM1). Treatment with capecitabine, along with trastuzumab, was therefore initiated. The capecitabine dose was reduced by 25% based on the elevated bilirubin and anticipated patient tolerance, which was subsequently discontinued due to side effects and disease progression. Therapy was then changed to vinorelbine, along with trastuzumab. Vinorelbine was given with a 60% dose reduction (12.5 mg/m2) due to its extensive hepatic metabolism by cytochrome P450 enzymes. The patient's disease had progressed after 2 cycles of vinorelbine and it was discontinued.\nThe patient then developed a left breast implant infection and recurrent metastasis around the port and skin area, requiring removal of bilateral tissue expanders, full thickness excision of necrotic skin on the right chest wall, and antibiotic therapy. Right abdominal fasciocutaneous flap histology was negative for cancer. The patient's bilirubin was 2–2.5 times the ULN; subsequently, nab-paclitaxel at a 50% dose reduction was initiated every 3 weeks for 5 cycles along with trastuzumab, which resulted in complete resolution of the cutaneous metastasis. Our patient requested a drug holiday and decided to hold off chemotherapy temporarily due to severe fatigue but agreed to continue trastuzumab every 3 weeks (6 mg/kg). One year later, she reported 2 small axillary nodules on the left upper chest wall and axilla concerning for recurrence. Unfortunately, her bilirubin was markedly elevated at 5 times ULN (6.0 mg/dl), limiting her ability to receive nab-paclitaxel. |
A 49-year-old male visited our pain clinic with the chief complaint of burning pain and sensory loss in the right temporal area and tongue for the previous 2 months. The pain intensity was 10 out of 10 on the numeric rating scale (NRS). The patient had felt a burning sensation on the tongue, as though tasting something hot, for one year before visiting our clinic. During a previous dental clinic visit, the patient had been diagnosed with temporomandibular joint disorder and put on treatment with daily ibuprofen (2,400 mg). Despite this medical treatment, he did not experience symptom relief. Therefore, the patient was transferred to our pain clinic.\nWith regard to medical history, the patient had experienced an episode of herpes zoster at the T4 dermatome 10 months earlier. On physical examination, the patient had neuropathic pain and allodynia in the distribution of the right mandibular nerve (V3). Flexion of the neck and compression of the occipital area induced heavy pain in the patient's head and neck, causing an inability to sleep in the supine position. However, there were no vesicles on his skin and no tenderness in the temporal area. The symptoms corresponded with trigeminal neuritis rather than classical TN. Thus, we first diagnosed the patient as having atypical TN and prescribed carbamazepine (CBZ) 100 mg bid, alprazolam 0.25 mg bid, and nortriptyline 10 mg daily. In addition, enhanced MRI was performed to identify the cause of neuritis.\nAn MRI of the internal auditory canal (IAC) showed a mass in the right parapharyngeal space through the foramen ovale. For further evaluation, an MRI of the paranasal sinus (PNS) with contrast was performed, revealing a 2.3 × 3 × 5.1 cm-sized, ill-defined, heterogeneously enhancing mass in the right parapharyngeal space and the right masticator space (). We had a high suspicion that the mass was a neurogenic tumor. The whole-body fluorodeoxyglucose (FDG) fusion PET showed suggestive of malignancy in the right parapharyngeal space and the right masticator space, while other areas showed no abnormal FDG uptake suggesting malignancy (). For the relief of TN, a right V3 root block via an infrazygomatic approach was performed in our pain clinic (), and then an otolaryngologist performed an endoscopic transpterygoid biopsy for pathologic evaluation.\nCBZ was very effective in treating the patient's TN, as the NRS value decreased from 10 to 3. However, a skin rash on the entire body developed suddenly 20 days later, which was thought to have been induced by CBZ. Thus, we stopped CBZ and started the patient on gabapentin 300 mg tid and oxycodone 5 mg tid. After 3 days, the skin rash disappeared, and the pain was well-controlled, with the NRS value maintained at 1 or 2. The patient was scheduled for radiation and chemotherapy. |
A 10-year-old white girl with right-sided SWS was diagnosed with right anisometropic amblyopia at age of 6 years and responded to glasses with a stabilized visual acuity of 20/100 in the right eye. She underwent laser treatment of the cutaneous nevus flammeus of the right side of the face with a good cosmetic result. There was no history of glaucoma. Magnetic resonance imaging of the brain excluded intracranial involvement. She presented with decreased vision in the right eye to over a period of 6 months with a hyperopic shift from 4.00 D to 6.00 D. At presentation, her best-corrected visual acuity (BCVA) was hand motions right eye and 20/20 left eye. Intraocular pressures were normal without evidence of iris neovascularization or abnormal conjunctival or scleral vessels. Ophthalmoscopic evaluation revealed a tomato ketchup red appearance of the right fundus compared with the left and a serous retinal detachment (RD) of the right eye (). The entire inferior retina of the right eye was detached with involvement of the macula. B-scan ultrasound and optical coherence tomography (OCT) () confirmed the presence of a diffuse choroidal hemangioma with thickening of the choroid and associated RD. Fluorescein angiography showed diffuse early hyperfluorescence. The intense early hyperfluorescence persisted through the laminar venous and full venous phases and only faded in the late angiogram phase. There was no fluorescein leakage from the retinal circulation. The patient underwent PDT with parameters typically used in treatment of choroidal neovascularization. Two sessions of PDT where the tumor was thicker were planned: the macula area and the area next to inferotemporal arcade. Verteporfin was infused at a concentration of 6 mg/m2, and an 83-second treatment was conducted with a 689 nm Zeiss laser that was delivered at 50 J/cm2 with an intensity of 600 mW/cm2. Firstly, four spots of 5300 μm were applied to the diffuse choroidal hemangioma in the macular area. OCT was used to monitor treatment response. Two weeks after treatment, there was a decrease in the thickness of the choroidal tumor. However, because the amount of subretinal fluid (mainly in lower quadrants) has not changed (), an inferotemporal choroidotomy for drainage of subretinal fluid was created. This procedure was combined with an intravitreal injection of gas (SF6) and cryoapplication in the temporal quadrants. No complications were noted. Forty days later, a second session of PDT treatment was applied. Five spots of 5200 μm were applied out of the inferotemporal arcade. The further PDT session resulted in the total elimination of the subretinal fluid and the overlying RD (). One year after the treatment, the RD has not recurred and BVCA improved to 20/125 right eye. No side effects were noted. |
The patient is a 24-year-old male with past medical history significant for obesity, anxiety and chronic omphalitis. He developed omphalitis following an abdominoplasty for excess skin after weight loss. Therefore, he underwent wide excision of umbilical stalk, direct lipectomy and placement of wound VAC (vacuum-assisted closure). He continued to have poor healing at the site and tunneling of the wound edges. There was concern for ongoing wound infection at follow-up, and he received an antibiotic course followed by local wound care.\nHe presented to our hospital for complex abdominal wall closure. He underwent sinus tract excision, recreation of the umbilicus and layered closure of the fascia followed by skin closure with drains. Postoperatively patient was started on lovenox dose 30 mg twice a day (BID) for deep vein thrombosis (DVT) prophylaxis, with the first dose administered 3 h after the surgery. Patient developed hemorrhage at the surgical site and was taken back the next day for washout and closure. Approximately two units of blood clots were removed per operative notes. Patient was noted to have diffuse oozing rather than bleeding from a single or multiple vessels consistent with a clotting abnormality rather than a anatomic etiology. His hemoglobin did drop from 11 g/dL at midnight of surgery to 9 g/dL by the next morning.\nHematology was consulted due to concern for excessive bleeding. The patient reported that he had multiple surgeons who had told him in his lifetime that he had increased intra and postoperative bleeding but he had no workup done for the same. He denied any family history of bleeding disorders. He also denied any history of purpura, epistaxis, gum bleeding, or hemarthrosis. He did have dental extraction without any complications. Patient was a non-smoker and non-alcoholic.\nInitial laboratory results were notable for chronic normocytic anemia with hemoglobin and hematocrit of 9 g/dL and 27% respectively. Prothrombin time (PT) was 15.1 s (reference: 11.8 - 14.3 s), internationalized normal ratio (INR) of 1.2, and plasma thromboplastin time was high normal at 31 s (reference: 23 - 34 s). Further workup showed normal factor VIII, IX, XIII activities and normal von-Willebrand’s antigen and activity. Thromboelastography was normal except low R-time of 4.2. Closure time and thrombin time were noted to be normal. Finally, factor XI activity was noted to be low at 0.28 U/mL (reference: 0.44 - 1.43 U/mL). He didn’t have any subsequent episodes of bleeding. He was educated on his increased risk of bleeding and potential need for fresh frozen plasma (FFP) infusions or factor XI infusions prior to future surgical procedures, and was asked to follow-up with the local hemophilia center to create a plan for any future surgeries that he may undergo. |
A 30-year-old woman first noticed double vision 2 years prior to hospitalization. It had been present periodically, mostly in the evenings, and did not disturb the patient sufficiently to seek medical attention. Three months later she noticed leg weakness with walking difficulties, especially when climbing and walking down stairs, and walking instability resulting in consecutive falls. Six months later weakness of the arms appeared that caused difficulties in washing and combing her hair. After seven months she noticed difficulties in swallowing and nasal speech after prolonged talking. All of these symptoms had a fluctuating course and were more pronounced in the evenings.\nA neurological examination revealed diplopia due to the weakness and fatiguability of the rectus superior and rectus lateralis muscles of the left eye as well as of the rectus inferior of the right eye, moderate weakness of the facial muscles, mild dysphagia with nasal speech, mild weakness of neck muscles, and weakness and fatiguability of proximal limb muscles. Very mild active and percussion myotonia were observed, but the patients had not been aware of it prior to hospitalization. The patient's mother had been diagnosed with DM2 at the age of 48 years and her maternal grandfather had cataracts.\nA diagnosis of MG was confirmed in our patient by clear positive results in the neostigmine test and decremental responses in the repetitive nerve stimulation test (RNST) (); these were 43% and 26% in the deltoid and nasal muscles, respectively. Anti-acetylcholine receptor (AChR) antibody titer was elevated (8.8 nmol/L, normal <0.2 nmol/L). A chest CT scan showed a persistent thymic tissue in the upper anterior mediastinum, and a pathohistological examination of the thymus after thymectomy revealed thymic hyperplasia. Electromyography during relaxation demonstrated diffuse episodes of myotonic discharges () in multiple muscles, while during voluntary muscle activity a mild myopathic lesion was revealed by short-duration motor-unit action potentials and early recruitment (). Genetic testing confirmed the presence of DM2. The patient improved remarkably after treatment with anticholinesterases, corticosteroids, and azathioprine. |
JA was a 6-month-old female patient who was the second baby of a consanguineous parents. Her elder sibling is apparently healthy 5-years-old girl. JA was born by normal spontaneous vaginal delivery after full term pregnancy and her birth weight was 3 kg. Her mother had gestational diabetes mellitus and she was on diabetic diet plan. No history of neonatal intensive care unit admission.\nAlthough parents were well educated with some physicians being family members and despite, they noticed that their baby is not developing normal and is being hypotonic, but they denied this fact until the age of 4 months.\nAt the age of 4 months, their baby started to have respiratory distress with desaturation and was admitted as a case of acute bronchiolitis. Her assessment revealed; global developmental delay, hypotonia with head lag, microcephaly and micrognathia. There was no associated hypopigmented skin or hair. Her abdominal examination was normal with normal female external genitalia.\nDuring hospital stay, the patient was noticed to have weak sucking and swallowing with recurrent chocking. Gastroesophageal reflux disease was investigated with no abnormalities. On the 8th day of hospitalization, the patient developed frequent tonic-clonic seizures with up rolling of both the eyes. Her electroencephalography revealed an abnormal focal epileptic discharge and levetiracetam was started.\nHer routine laboratory investigations were normal, and her immunological work up revealed normal immunoglobulin level. Ophthalmological examination revealed bilateral congenital cataract. Brain magnetic resonance imaging showed brain atrophy with corpus callosum agenesis (Fig. ) and poor grey and white matter differentiation with a scarce myelin sheath. Her echocardiography showed hypertrophic cardiomyopathy with mitral regurgitation, tricuspid regurgitation, and pulmonary hypertension (Fig. ). Chromosomal studies revealed a normal karyotype (46XX).\nBased on the constellation of findings in the history, physical examination and investigations; the diagnosis of VICIS was suspected.\nMolecular genetic testing with whole exon sequencing identified the pathogenic homozygous variant c.4751T>A p. (Leu1584∗) on exon 27 of the EPG5 gene and confirmed the diagnosis (Fig. ).\nGenetic counseling for the patients parents was carried out and the family was tested to the detected mutation which identified that both parents are heterozygous and that her sister was normal. Parents were advised for pre-conception counseling and to consider in vitro fertilization treatment.\nThe patients terminal event was at the age of 9 months when she presented to the emergency room in critical condition and was shifted to the pediatric intensive care unit as a case of refractory septic shock with cardiac failure. Resuscitative measures were done but the patient did not revive and declared dead. |
A 69-year-old male was referred to Emory Eye Center in June 2010 for herpetic stromal keratitis in the left eye. His past ocular history was notable for central retinal vein occlusion (CRVO) in the left eye 3 years earlier. He underwent multiple intravitreal injections of bevacizumab and triamcinolone for the treatment of CRVO-associated macular edema. He subsequently developed a suspected herpetic keratouveitis resulting in a dense central stromal scar and visual acuity of count fingers despite aggressive topical and oral antiviral therapy.\nThe patient underwent penetrating keratoplasty (PK) with extra-capsular cataract extraction and intraocular lens placement in July 2010. Pathologic examination of the corneal transplant showed microsporidial organisms. Visual acuity improved to 20/80 with use of rigid gas permeable contact lens, and the graft remained clear until March of 2013 when the patient presented with signs and symptoms of early graft rejection. Topical corticosteroids were utilized with no improvement, so repeat PK was performed in October 2014. Pathologic examination of the repeat PK specimen again showed microsporidia despite the absence of intraocular inflammation, hypopyon, or any other signs of clinical infection.\nFive weeks post-operatively, the patient presented with new onset floaters that had been present for 2 weeks. Visual acuity was light perception, and intraocular pressure was 17 mmHg. Anterior segment examination showed trace haze at the cornea graft-host interface, 1+ anterior chamber cells, a normal iris with a round and reactive pupil, and a well-positioned posterior chamber intraocular lens. There was no view of the posterior pole. B-scan ultrasound showed a presumed vitreous hemorrhage. The patient was lost to follow-up and presented again on post-operative week 9. Examination showed keratic precipitates, trace anterior chamber cell, and neovascularization of the iris. There was no view of the posterior segment due to a dense yellow opacity over the pupil (Fig. ). B-scan ultrasound showed vitreous opacities, and the patient was diagnosed with presumed endophthalmitis. A vitreous tap and injection with vancomycin 1.0 mg/0.1 mL and ceftazidime 2.25 mg/0.1 mL were performed. A diagnostic and therapeutic pars plana vitrectomy was performed as the patient failed to improve. Examination of vitreous specimen and iris tissue biopsied at the time of vitrectomy showed microsporidia, consistent with a diagnosis of microsporidial endophthalmitis. Bacteria and fungi did not grow in culture. The patient was lost to follow-up and therefore did not undergo testing for the human immunodeficiency virus (HIV). To our knowledge, he was otherwise healthy and without underlying systemic disease. |
A 12-year-old girl reported to our department with a complaint of missing lower teeth and an unaesthetic facial appearance with progressive loss of hearing over a period of 3 months. Patient complained that only few permanent teeth had erupted and she had difficulty in chewing and was unable to eat. Past dental history revealed extraction of upper left permanent molar by a private dentist because of gross decay. There was no relevant medical history or family history. Her general examination revealed short stature and she was well oriented with normal intelligence. Extraoral examination revealed hypertelorism (increased intercanthal distance of eyes), frontal bossing with brachycephalic skull, depressed and flattened nasal bridge, malar hypoplasia, and an underdeveloped maxilla with a prognathic mandible and concave facial profile []. Hand showed hypoplastic distal phalanges [], and brachydactyly of the fourth toe [] was seen. The thorax was narrow and cone shaped, and spinal abnormalities like mild scoliosis and kyphosis were appreciated []. On palpation, a depression over the sagittal suture with presence of metopic suture of the frontal bone was felt and the patient could approximate her shoulders in midline []. Intraoral examination revealed normal mouth opening and jaw movements. Erupted upper right permanent first molar and retained deciduous teeth were seen with high arched and narrow palate [Figure , ] with erupted right permanent first molar and retained deciduous teeth [] were seen. Panoramic radiograph revealed few impacted teeth, multiple retained deciduous teeth, and underdeveloped maxillary sinuses []. AP view of pelvis demonstrated a widened pubic symphysis and triradiate cartilage []. Skull AP and lateral views showed the presence of multiple wormian bones with open fontanelles []. Chest radiograph showed bell-shaped rib cage and hypoplastic clavicles []. Based on the clinical and radiographic findings, a diagnosis of CCD was made. She was referred to otolaryngologist for opinion and audiometry revealed conductive deafness of right side of the ear. Multidisciplinary approach with dental and medical team of doctors will aid in the treatment of this rare CCD. |
A 550 Kg, 20 years old pregnant mare was presented at the equine teaching hospital due to severe hindlimbs lameness. A heart rate of 56 beats per minute, a respiratory rate of 16 breaths per minute and clear signs of pain were present at the clinical examination. A radiological study revealed a high-grade, chronic tendinopathy of the suspensory branches on the right and left side. Since foal delivery was expected within 2 months, the horse was hospitalised for pain and supportive therapy. During the hospitalization, pain could not be easily controlled, despite the adoption of a multimodal systemic analgesic plan. A pain score [] was used in order to monitor the analgesic efficacy of the attempted treatments. Due to the inefficacy of a combination of methadone, non-steroidal anti-inflammatory agents and gabapentin in controlling pain over the first week, an epidural catheter placement was planned, in order to provide repeated morphine administrations into the epidural space. The sacro-coccigeal area was clipped and aseptically prepared, and a Tuohy needle was inserted targeting the sacro-coccigeal space, with the needle opening pointing cranially. The hanging drop technique with sterile saline was used to confirm the needle tip location into the epidural space. An epidural catheter was advanced through the Tuohy needle, for a length previously calculated in order to reach the L4-L5 vertebra. A bacterial filter was applied to the injection port and the catheter was secured to the skin with adhesive material and covered with adhesive dressing. The systemic analgesic treatment was stopped. An initial epidural dose of 0.1 mg/kg of morphine (Morphin HCl Sintetica, Sintetica S.A., Switzerland) every 8 h was started, with immediate improvement of the clinical condition. After each drug injection, the epidural catheter was always flushed with a volume of 6 ml of sterile saline. An abdominal ultrasound examination was repeatedly performed in order to check the clinical condition of the foal, with no abnormalities detected until delivery. On day 16 (from now on, days are reported as “days after starting the epidural analgesic treatment”), a strong excitatory phase with increased locomotor activity, dysphoria and photophobia occurred and lasted for few hours. Even if it could not be correlated to any particular injection or time interval after injection, a morphine overdose was suspected. Its administration was stopped and epidural methadone (0.1 mg/kg) (Methadone Sintetica, Sintetica S.A., Switzerland) was given instead. Two days later, due to stable clinical conditions, methadone was stopped and morphine restarted. During the excitatory period, the horse was moved to an isolated and quiet stable and the windows were covered with black light-reflecting material. On day 21, due to a weaning of the analgesic effect of morphine within one hour before administration, shorter intervals (6 h) were adopted and ketamine (Ketasol, Graeub AG, Switzerland) was added (0.1 mg/kg every 6 h). On day 42, a second but milder excitatory episode occurred; also in this case, it could not be correlated to any particular injection or time interval after injection. Morphine was interrupted for 24 h and it was substituted by epidural methadone (0.1 mg/kg). The day after, due to stable clinical conditions, methadone was stopped and morphine restarted at half of the dosage. On day 49, the foal was delivered and morphine dose was further reduced to 0.025 mg/kg. On day 53, epidural administration was stopped and morphine (0.025 mg/kg) and ketamine (0.5 mg/kg) were injected intramuscularly, every 8 h. On day 56 the epidural catheter was removed. A bacterial examination of the catheter tip was performed and no contamination was found. Finally, on day 57, the mare and the foal were discharged from the hospital in stable clinical conditions and the analgesic therapy of the mare was continued at home by the private veterinarian. During the whole hospitalization period, reduction in faeces production occurred but no signs of colic were noticed.\nAfter obtaining owner consent, the plasmatic levels of morphine ant its metabolites (M3G and M6G) were evaluated to titrate the analgesic treatment over time. Moreover, blood samples were taken from the foal (in concomitance with already needed blood withdrawal for routine tests) in order to rule out morphine overdosing. On day 36, a sequential blood sampling (SBS) from the mare was performed, at 30 min before and 1, 3 and 5 h after morphine administration. Another sample was taken on day 42, one hour and a half after morphine injection, during the episode of excitation and increased locomotor activity. Further blood samples were taken on day 49, 50 and 51 (day of delivery and 1 and 2 days post-delivery). For each blood collection, 10 ml of blood were withdrawn and put aside, 10 ml were then collected in an EDTA syringe and the previous blood administered back to the horse. The blood was immediately centrifuged (3000 rotation per minute for 10 min at 20 °C) and the plasma successively stored at − 80° in special cryotube (CryoPure Tube, Sarstedt, Germany). Withdrawal of blood from the foal was performed on day 0 (after delivery, before first meal), on day 1 and on day 2. In this case, 1.5 ml of blood was taken and transferred in an EDTA tube; then, the same centrifugation and storages process as for the mare was applied. Measurement of morphine, M3G and M6G plasmatic concentration were performed using liquid chromatography-tandem mass spectrometry. Results are presented in Tables and . |
A 74-year-old female was initially evaluated for a headache and right eye peripheral vision loss. MRI brain with and without contrast was performed, revealing a 5.5 cm heterogeneously T2 hyperintense lesion with thick irregular nodular enhancement in the left parietal-occipital region (Fig. ). The patient underwent a gross total resection of the mass that was diagnosed as GBM, IDH-wildtype, WHO grade IV, MGMT promoter methylation not detected. Fresh specimen in multiple sectors was processed for tumor culture. Following resection, she received hypofractionated concurrent chemoradiation with temozolomide followed by four cycles of adjuvant temozolomide (TMZ). Six months later, the patient developed multifocal GBM recurrence in the right temporal and frontal lobes, for which she underwent single fraction 18 Gy stereotactic radiosurgery to the right frontal lesion and five fractions of 2250 cGy to the right temporal lesion.\nThree months following SRS, the patient began to experience falls associated with hip pain and difficulty walking. MRI of the right hip demonstrated a pathologic hip fracture, which was thought to be due to metastatic disease from an undiagnosed second primary cancer. The patient then developed altered mental status and right-sided upper motor neuron facial weakness. A full metastatic imaging work-up was performed, revealing a 3.9 cm left breast mass, multiple lytic lesions of the pelvic bones, and multiple pulmonary and hepatic nodules. Core biopsies were obtained from the left breast and the left pubic bone, both of which demonstrated metastatic GBM.\nThe patient’s mental status deteriorated as the right temporal recurrence rapidly progressed, and she underwent right temporal craniotomy for debulking of the tumor eleven months after her initial diagnosis of GBM (Fig. ). Histologically, this secondary tumor was identical to the primary. 2 × 105 freshly dissociated cells from the right temporal recurrence were orthotopically transplanted directly into the striatum of SCID mice with preserved microglial activity (IcrTac:ICR-Prkdc strain) to assess the cells’ ability to generate a patient-derived xenograft (PDX). Following surgery, the patient stabilized neurologically, but opted for palliative care and was transferred to hospice where she expired one month later.\nWhile the patient was alive, consent was obtained for a rapid autopsy, which was ultimately performed within four hours of death. Gross and histological evaluation confirmed numerous GBM metastases. The extent of metastatic disease was widespread, including bilateral lungs, heart, liver, thyroid, left breast, small bowel, omentum, peritoneum, left pelvic bone, and hilar lymph nodes (Fig. ). Notably, there were extensive metastatic lesions involving the abdominal cavity.\nThe autopsy revealed residual GBM in the original site of occurrence (left occipital lobe). On histological examination of pre- and post-mortem samples, the metastases appeared identical to the primary tumor and temporal recurrence. All sites demonstrated the classic appearance of GBM on H&E staining, including nuclear atypia, microvascular proliferation, and pseudopalisading necrosis. Sarcomatous transformation was not identified on histological review of the tissue sections, and was confirmed absent by reticulin-staining performed on representative sections from occipital lobe, temporal lobe (Fig. e), left breast, heart, lung, liver, lymph nodes, and omentum.\nTo evaluate the molecular phenotype of the primary, recurrent, and metastatic lesions, two different next-generation-sequencing (NGS) panels were employed (see Additional file ). The Ion AmpliSeq Hotspot Cancer NGS Panel v2, covering 50 genes and 207 amplicons, was performed on all pre-mortem (left occipital brain primary, right temporal lobe recurrence, and left breast metastases) and select post-mortem (paratracheal lymph node and omentum) tissue specimens. The Ion Torrent Oncomine Comprehensive Assay v3, covering 161 cancer driver genes, was performed on the remaining post-mortem samples (left occipital lobe, right temporal lobe, left breast, lungs, and liver) collected during rapid autopsy.\nThe AmpliSeq panel revealed single nucleotide variants (SNVs) in PIK3CA, SMARCB1, BRAF, and TP53. Notably, five different SNVs were detected for TP53. There were differences in TP53 mutations between the primary tumor, metastases, and among the metastases themselves (Fig. ). The more comprehensive Oncomine panel revealed only one non-silent SNV common to all specimens: BRCA1 p.I571T. A large number of private mutations were detected in the temporal lobe recurrence, left breast, lung, and liver metastases. Among the extracranial metastatic sites, only the left breast, liver, and omentum specimens were found to share any mutations other than the one identified at BRCA1.\nCells cultured from the left occipital primary site at the time of initial diagnosis, and the right temporal site at the time of recurrence, expanded readily in serum-free conditions and were all found to harbor the BRCA1 p.I571T mutation (see Additional file ). Both mice, orthotopically xenotransplanted with cells from the temporal recurrence, developed fatal malignant gliomas after 5 weeks, confirming the tumor’s aggressive behavior in both human and rodents (Fig. ). Necropsy analysis of the lungs, intestines, liver, and spleen in these mice did not reveal the presence of any peripheral metastases as seen in the patient. Tumor cells from this aggressive GBM were isolated from the primary PDX and were subsequently propagated in culture and used to generate a reliable PDX model which forms within 3–4 weeks post implantation and, importantly, recapitulates both the rapid growth and the malignant infiltrative spread of human GBM. Mutational hotspot analysis of the PDX confirmed the presence BRCA1 p.I571T in all cells, and multiple inactivating mutations of ARID1A in a significant subpopulation. ARID1A mutations were also identified in the left breast autopsy specimen (Fig. ).\nTo investigate whether the relatively high number of SNVs in all secondary sites compared to the primary could have resulted from mismatch repair (MMR) deficiency, we performed a mutational spectrum analysis; results confirmed a high proportion of C:G to T:A transitions, which is typical of the mismatch repair deficiency described in the setting of temozolomide-treated recurrent GBM (Fig. ). The integrity of the mismatch repair pathway was evaluated using immunohistochemistry. All metastatic specimens exhibited positive staining for anti-MLH1, anti-MSH2, anti-MSH6, and anti-PMS2, indicating microsatellite stability (MSS). MSS was further confirmed with PCR of established microsatellite loci [] (see Additional file ). In addition, we evaluated DNA polymerase epsilon (POLE) using PCR, which showed no alterations at mutational hotspots within the coding region of the gene. |
A boy aged 5 years and 10 months was admitted to the hospital on 13 October 2012 with recurring symptoms of hematuria for more than 1 month and weight loss of 1 kg. No urinary irritation symptoms, chills, fever, or rashes were observed. In the neonatal period, the boy had been well nourished. A physical examination revealed no enlargement of the tongue, umbilical hernia, iris loss, genitourinary malformation, or mental dysplasia. The kidneys did not seem to be swollen, and there appeared to be no pain when percussed or pressed. Similarly, pressing on the bladder area induced no pain, and no mass was found. There was no family history of nephroblastomas.\nBlood and liver function tests revealed no obvious abnormalities. However, a routine urine test showed proteinuria and hematuria. A computed tomography (CT) scan revealed a 2.9 cm in diameter shadow of round, soft tissue in the mid pole of the left kidney, with an uneven density. Scattered calcifications were also detected (). Color ultrasound imaging confirmed the presence of a hyperechoic mass (2.2×1.5 cm) in the mid pole of the kidney, with an intense blood signal (). Emission computed tomography (ECT) pointed to the presence of space-occupying lesions in the left kidney, in which the blood supply was increased, suggesting a malignant tumor.\nThe patient underwent resection of the tumor on 22 October 2012. In the process of the resection, the tumor was seen to be located in the mid pole of the left kidney. The papillary tumor invaded the renal parenchyma and pelvis, but the kidney capsule and ureter were tumor free. Further, lymphadenectasis was not found surrounding the renal pedicle.\nGross examination showed a grayish-white irregular 2.5×2.5×2 cm sized mass in the left kidney, with a definite boundary. The surface of the mass was partly adhered to the kidney capsule. The cut surface showed that the tumor had spread from the renal cortex to the medulla and finally to the renal pelvis, with formation of several cysts. Some large cysts included grayish yellow papillary excrescences (). Under a microscope, the tumor consisted of a papillary RCC and WT. The papillary RCC was located in the cyst, and the cancer cells were arranged in a papillary shape. The thin fibrovascular cores could be observed in these papillae, and some of the tumor tissue invaded the cyst wall (). The majority of the WT tissue was epithelium, and well-differentiated tiny tubes coexisted with poorly differentiated original tubular structures (). Loose connective tissues () and immature mesenchymal tissues were discovered between the WT and papillary RCC (). The surgical margins were clear. As shown by immunohistochemical staining, the papillary RCC region was positive for cytokeratin 7 (CK7), alpha-methylacyl coenzyme A racemase, and cluster of differentiation 10 but negative for Wilms tumor gene 1 (WT1). The immunoprofile of WT region was contrary to the former (). The pathological diagnosis was an epithelial WT coexisting with a papillary RCC in the left kidney.\nAccording to the criteria of the National Wilms’ Tumor Study, the WT was classified as Stage I. Based on the pathological grading, the RCC was classified as Grade 2, and the TNM staging was T1aN0M0. Therefore, the treatment provided was in accordance with that for a Stage I WT (ie, radical nephrectomy of the left kidney, with complete resection of the tumor). After the operation, the patient recovered quickly. One month after the operation, the patient received vincristine plus dactinomycin (EE-4A regimen) as chemotherapy and underwent regular CT and B-mode ultrasound examinations. At the 69-month follow-up, there was no recurrence or metastasis.\nThis study was approved by the medical ethics committee of the First Affiliated Hospital of Guangxi Medical University. Written informed consent has been provided by the legal guardian of the patient to have the case details and any accompanying images published. |
A 49-year-old woman presented with a 3-month history of repeated ptosis of both eyelids and oral ulcers and erosions. Physical examinations revealed scattered ulcers and erosions in the mouth (Fig. ). Laboratory examinations showed that CA 125 was elevated (51.6 U/ml), while other tumor markers, including CA199, 153, CEA, and AFP, were normal. Autoimmune antibodies, including anti-CENP-B antibody, ANCA, anti-AchR antibody, and ANA, were all positive.\nThe patient was first diagnosed with an oral aphthous ulcer and ocular myopathy myasthenia gravis. She was treated with gentamycin and dexamethasone spray inhalation to improve her oral lesions and pyridostigmine to cure muscle weakness. However, the oral ulcers improved slightly and the myasthenia gravis persisted. An abdominal ultrasound showed a hypoechoic mass in the left adrenal gland. A further CT examination showed a 6 × 5 cm, well-defined round solid mass with central necrosis in the pancreatic tail. There was no calcification detected in the mass. The solid part of the mass had slight enhancement in the arterial phase with many serpentine feeding arteries, moderate enhancement with a draining vein around the tumor in the portal venous phase and persistent enhancement in the delayed phase (Fig. a-e). The fundus of the stomach was compressed by the mass. The boundary between the mass and the splenic artery and vein was not clear, and swollen lymph nodes were not observed in the posterior peritoneum. The mass was initially considered to be a neuroendocrine tumor in the pancreatic tail.\nAt the same time, the patient’s symptoms worsened. She could not swallow, and she felt severe pain in her mouth. She also developed a cough and expectoration. A chest CT revealed infection in the lower lobes of both lungs. Streptococcus was detected from a throat swab. Levofloxacin was administered to fight the infection, methylprednisolone to fight the inflammation, and thalidomide to alleviate the vascular inflammatory reaction in addition to pyridostigmine and immunomodulatory therapy. However, 3 days later, the patient progressed to severe dyspnea, wheezing and difficulty with expectoration. Emergency intubation and mechanical ventilation were administered. Aspergillus was detected after bronchoalveolar lavage. Immunoglobin and voriconazole were given. Four days later, the symptoms resolved and the intubation was detached. Most of the infections in the lungs were resolved according to a chest CT. The pain in the mouth was also alleviated.\nAfter a multidisciplinary discussion, the patient’s tentative diagnosis was paraneoplastic pemphigus and the myasthenia symptoms caused by the pancreatic tumor. Myasthenia gravis, in turn, led to the patient’s inability to excrete sputum. If the pancreatic tumor could not be removed, the symptoms would not completely remit, and the symptoms due to myasthenia gravis would also continue to aggravate, finally leading to the occlusion of the respiratory tract. Therefore, the patient was transferred to general surgery. During the surgery, a 4 × 5 cm dark-red tumor with medium texture and clear boundaries was detected in the pancreatic tail (Fig. ). The tumor was close to the splenic artery and vein, and the spleen was normal. Postoperative pathology confirmed the tumor was a follicular dendritic cell sarcoma with immunohistochemistry showing CD21(+), CD23(+), CD138(+), SMA(+), Des(+), CD117(−), DOG-1(−), S-100(−), CD34(−), CK(−), EBER and EBV(−)(Fig. a-c).\nThe patient continued to be treated with antifungal and anti-infection therapy. Twelve days after surgery, the patient developed sudden heart palpitation, discomfort and difficulty in breathing. Mechanical ventilation was again administered. However, the patient died of inability to excrete sputum and occlusion of the respiratory tract. |
An eighteen month old Nigerian female presented to our health facility with a history of noisy breathing since birth, associated with intermittent mucoid rhinorrhea, especially from the right nasal cavity. Protrusion of a pale mass in the right nasal cavity was observed by the parents prior to presentation. There was no epistaxis, fever or difficulty with feeding. The child was the product of a term pregnancy and delivery was said to be uneventful. The developmental milestones were within normal expectations.\nExamination revealed a well-fed young girl with noisy respiration, afebrile and not pale. The external nasal pyramid appeared splayed; the right nasal cavity was completely obliterated by a lobulated pale mass which was firm, sensitive to touch, non-hemorrhagic and attached to the nasal septum. It was partly covered by mucoid secretions. Test aspiration of the mass was dry. The left nasal cavity, ears, throat and neck appeared normal. A nasogastric tube was easily passed through the left nasal cavity. No lesion was seen in any other parts of the body.\nX-ray (occipito-frontal and post nasal space views) revealed radio-opacity of the right nasal cavity, extending posteriorly into the nasopharynx. There was no evidence of calcification of the soft tissue shadow or bony erosion of the nasal bony framework. The plain radiographs of the soft tissue neck and the chest were within normal limits. Complete blood and platelet counts were within normal range.\nExamination under general anesthesia (via oro-tracheal intubation) revealed that the right nasal mass could neither be delivered via the anterior nares nor the post nasal space, prompting a right lateral rhinotomy for adequate exposure of the mass (Figure ). The stalk which was attached at the mid portion of the cartilaginous septum was rocked and completely excised and the rest of the mass delivered. Hemostasis was achieved and wound closed in two layers.\nThe excised mass appeared irregular, but smooth-surfaced, measuring 6 × 5 × 3 cm and weighing 27 g. The cut surface showed a lobulated tan colored appearance. Histological examination revealed haphazardly arranged heterogeneous tissues, including cysts lined by epidermis with associated hair follicles and sweat glands, gastrointestinal epithelium, respiratory epithelium and columnar epithelium reminiscent of mammary glands. Lobules of mature adipose tissue and fibrocollagenous connective tissue were also present (Figure ). These histological features were consistent with a mature teratoma.\nThe postoperative course was uneventful and the patient was discharged on the seventh day post surgery. Follow-up for the past 31 months has been symptom free. |
On July 23rd 2015, a 27-year-old gravida 3, para 2 woman with a 2-week history of intermittent abdominal pain, especially aggravated during sexual intercourse, was referred to Amana Regional Referral Hospital in Dar es Salaam. The patient was amenorrheic for 4 months and had last normal vaginal delivery 7 years ago. Vital signs and initial laboratory parameters were nomal. On physical examination, her abdomen was distended with a fundal height corresponding to approximately 20 weeks, which distends more than it should. Vaginal examination showed the presence of a single cervix. She had been irregularly using oral contraceptive pills after her second pregnancy, and had a history of sexual transmitted infection in December 2014.\nIn June 2015, she was diagnosed with HIV and started antiretroviral therapy. Her HIV regimen includes Atripla and Septrin for opportunistic infections. For accurate diagnosis an ultrasound examination was thus performed. Transabdominal sonography revealed a single live fetus appearing to be extrauterine with transverse lie, which is very close to the anterior abdominal wall. The fetal biparietal diameter of 2.6 cm and crown-rump length of 6.7 cm were measured and corresponded to the 13+ weeks mean and fetal cardiac activity was over 100 beats/min. There was no demonstrable uterine myometrium around the fetus and cramped fetal parts with reduced to no amniotic fluid (). Because an empty uterus can easily be missed on a routine transabdominal ultrasound scan, transvaginal ultrasound scan was used for clearer visualization of the uterine cavity. Transvaginal ultrasound scan revealed an empty uterus and a moderate amount of free fluid in the Pouch of Douglas.\nOn July 27th 2015, to confirm the presumptive diagnosis of abdominal pregnancy, a subsequent abdomen-pelvis magnetic resonance imaging was performed at Muhimbili National Hospital in Dar es Salaam, and it showed no intrauterine gestational sac while a fetus was visualized in right abdomen with the placenta in the right iliac fossa ().\nOn July 29th 2015, the patient suddenly developed intensified abdominal pain, her blood pressure fell to 80/40 mmHg and hemoglobin of 6.5 g/dL. Emergency laparotomy was performed. Under general anesthesia, a midline incision above the umbilicus was made. On opening the abdomen, about 1,500 mL of blood associated with clots was found and removed from the peritoneal cavity. The placenta was attached between the anterior and posterior leaves of right broad ligament and also implanted over right adnexal and ileocolic region. The right fallopian tube and ovary were severely distorted which is highly suggestive of a tubal pregnancy that ruptured and resulted in secondary implantation in the peritoneal cavity, and they were neither well visible because of extensive pelvic adhesion. The left fallopian tube and ovary were normal. The uterus was found to be empty and small.\nA gestational sac lying on the right side of the abdominal cavity superior to the uterus was seen and part of the fetus was protruding from it. The gestational sac was incised, the fetus was extracted, and the umbilical cord was clamped and ligated at its base. Large portions of placenta were adhered to segments of the large bowel, omentum, and right adnexal region of the uterus and there was significant bleeding from the partially detached placenta, which prompted removal of about 80% of the placenta to facilitate hemostasis. The rest of the placenta was left in situ. Unilateral adnexectomy and partial omentectomy were also performed. One unit of blood was transfused intraoperatively and another unit of blood was given postoperatively. The patient recovered without any other complications and was released from hospital on the 5th postoperative day, and no abnormality was detected on subsequent ultrasound examination.\nOn November 16th 2015, the patient tested for CD4 count and the result was 502 cells/mm3, a good sign that antiretroviral therapy is working effectively. On December 4th 2015, 4 months after the termination of pregnancy, she also tested for beta-human chorionic gonadotropin and the level had completely regressed to less than 1 mIU/mL. |
A 17-year-old man was involved in a road accident in which he suffered the open fractures of the right femur and tibia. At the arrival to the Emergency Dept (ED), he was alert and hemodynamically stable and the Glasgow Coma Scale (GCS) was 15; the initial alignment of the fractured ends was performed in the ED with a gentle traction performed under sedation with iv. ketamine; a total body CT did not demonstrate other injuries. Approximately two hours after the admission the patient was taken to the surgical theatre for the external fixation of the fractured bones; at entering the operating room, the GCS was 8, the arterial pressure was 115/80 mm Hg, the heart rate was 115 bpm, and the arterial oxygen saturation (SPO2) was 85 at room air; the procedure was performed under general iv anesthesia with propofol and remifentanyl; the standard monitoring included the ECG, the noninvasive arterial pressure, the SPO2, and the end-tidal CO2 (ETCO2); during the intervention, the SPO2 rose to 100% at a FIO2=40% and all the other variables remained stable throughout the procedure after the 3-hour-long intervention in which the complete alignment of the bony ends was achieved; the patient was transferred to the Intensive Care Unit (ICU) still intubated and mechanically ventilated; the iv anaesthetics were gradually tapered until the complete suspension. Two hours later, the SpaO2 and the ETCO2 slightly decreased and anisocoria was observed; and an urgent CT scan of the head demonstrated a diffuse cerebral edema and the herniation of the cerebellar tonsils (Figures and , respectively). At this time, the pupils became bilaterally mydriatic and the EEG was almost isoelectric; due to the severity of the conditions, a MR scan was considered unnecessary. On the basis of the clinical and radiologic findings repeated boluses of iv. mannitol and steroids were given in the following hours aiming to reduce the intracranial pressure. An echocardiogram demonstrated a severe right ventricular depression with an ejection fraction of 20%. On the following day, the patient was declared brain dead according to the current Italian law.\nAt the autopsy, the cerebral microvascular network appeared diffusely plugged with BME (Figures –) and ischemia-related microcalcifications were scattered throughout the brain (); other organs were less extensively involved; no PFO was demonstrated. |
A 60-year-old female housewife was admitted to the Urology Clinic of Sina Hospital, Tehran (capital of Iran) eight months before admission (winter of 2013) with pain in the suprapubic area (). The patient had been treated by antibiotic therapy since eight months ago following intense suprapubic pains accompanied by dysuria and frequency symptoms and had been diagnosed as simple cystitis. In spite of partial recovery and continuation of long-term treatment with antibiotics, non-steroid anti-inflammatory drugs, sedative drugs and alpha-blocker, the symptoms were recurred with severe and mild periods. The pain was persistent and spread towards the perineal area. Along with the pain, the patient had frequency, nocturia and dysuria, which resulted in inability to pass urine completely. The pain intensified when the bladder was full and the patient also had occasional flank pain on both sides. In abdominal physical examination, slight tenderness in the suprapubic area was found, but the rest of examination had normal findings. In paraclinical tests, urine culture test had normal results, and urine analysis showed normal findings regarding the values of white blood cell, red blood cells, sugar and protein. Urine cytology had negative results regarding malignancy. Abdomen and pelvic sonography and the liver and kidney function test had normal results.\nIn cystoscopy report, the size of bladder was slightly decreased and there was a mild inflammation of the epithelial lining of the bladder. No tumoral lesion or glomerulation was observed. Bladder biopsy and hydro distention were performed. Bladder biopsy had negative results for malignancy, but chronic inflammation was reported. The results of the examination of the genitalia system in physical examination and dilation and curettage (D&C) had normal findings.\nThe patient reported diabetes mellitus type 2 for three years and used metformin daily. FBS was 120 mg/mL. The digestive problems of the patient were a persistent bloat for many years accompanied by pain in the epigastrium and the umbilical area, constipation, and hemorrhoid without bleeding. The musculoskeletal problems of the patient were chronic osteoarthritis in both knees and chronic low back pain, which had reduced the physical activities of the patient to a sedentary life. This patient was chosen because of presence of both symptoms of BPS and “reeh”. Positive “reeh” signs and symptoms included:\nAbdominal distention with boring quality, urinary symptoms, and non-localized and shifting pains in the abdomen with sudden onset relived easily, absence of feeling heaviness in the pain position, severity of the pain with consumption of flatulent foods like peas.\nICSI Score, numeric pain rating scale (NPRS) (which scales the pain from one to ten) and Impact of symptoms/quality of life (QOL) (a questionnaire with negative scaling of quality of life) score results were recorded in each examination session before starting the treatment (, ). ICSI assesses urinary symptom (urgency, frequency and nocturia). These were used as indicators of the symptoms before the treatment and a tool for keeping track of the treatment process. The patient took the infusion of two grams of dried horsemint before lunch and dinner. The dose was very lower than the toxic dosage. New reports of her condition were prepared every 14 days, after either visiting her or making phone calls. Measuring variable was performed by taking history, clinical examination and using the questionnaires by supervising an expert urologist. |
A 79-year-old Caucasian male, with a past medical history of atrial fibrillation on warfarin and metoprolol, and coronary artery disease on atorvastatin with previous coronary artery bypass grafting and placement of a dual-function pacemaker/ implantable cardioverter defibrillator (ICD), was on a motor boat in a remote location. The patient’s boat went over a wake of a larger boat passing by. He bounced off his seat in a vertical direction and subsequently landed on his tailbone. After the high impact fall, he complained of both immediate lower back and diffuse abdominal pain but did not seek out urgent medical help.\nTwo days after the initial incident, he started to become pale and diaphoretic; additionally, his ICD delivered three shocks over a 30-min period. He presented via ambulance service to a local community hospital in hemorrhagic shock with a blood pressure of 63/22 and heart rate of 118 beats/min. A primary survey was pertinently positive for hemodynamic instability and diffuse abdominal and lower thoracic spine tenderness.\nHe was resuscitated with 1 L of normal saline leading to an improvement of his pressure to 106/88. Initial laboratory investigations included a hemoglobin of 95 g/L, lactate of 6.1 mmol/L, creatinine of 129, and a supratherapeutic INR of 8.8. An initial non-contrast CT abdomen and pelvis showed moderate hemoperitoneum with sentinel clot in the left upper quadrant and pericolic gutter, as well as the area adjacent to the posterior wall of the stomach. An additional finding of a severely comminuted, minimally displaced burst fracture of the T10 vertebral body was noted (). Further interventions included INR reversal with 3 mg of Vitamin K and 3000 units of prothrombin complex concentrate, and administration of 2 units of packed red blood cells and 2 L of normal saline. Based on clinical severity, the patient was transferred to the trauma service at a tertiary-care Level 1 trauma center.\nPrimary survey revealed a protected airway, spontaneous and bilateral air entry, and hemodynamic stability with a blood pressure of 100/60 and a heart rate of 88 beats/min. His abdomen continued to be mildly distended and tender without peritoneal signs, however the patient reported it had improved since his original presentation to the local hospital. Repeat laboratory investigations revealed a stable hemoglobin of 94 g/L, and correction of his INR to 1.2. Given his stable condition, he underwent a CT RIPIT (Rapid Imaging Protocol in Trauma) [] and CT angiogram (CTA) of the abdomen and pelvis. His imaging revealed pseudoaneurysms of the left gastric artery measuring up to 6 mm with another 9 mm rounded area of increased attenuation along the lesser curve of the stomach (, ). No extravasation was seen. Decision was made to monitor the patient closely with serial abdominal exams and repeat imaging in 72 h, or sooner if the patient exhibited any signs of deterioration.\nOver the next 72 h, the patient’s vital signs and abdominal exams, improved and his hematological profile remained stable. Given the patient’s CHADS score of 2, it was decided to hold therapeutic anticoagulation until the patient’s bleeding risk decreased, however deep venous thrombosis prophylaxis was initiated. A repeat CTA was performed to follow the evolution of the two pseudoaneurysms. The imaging study revealed unchanged pseudoaneurysms but noted the distal aspect of the left gastric artery was attenuated in keeping with a focal dissection and intramural thrombus. Secondary to the dissection, the patient was started on 81 mg of aspirin daily. Over the next few days the patient continued to improve clinically, and was discharged home. The patient lived outside of the local area, and arrangements for close follow up were made. |
On 24 June 2002, a 67-year-old Japanese woman presented to our department with the chief complaint of a swollen left nasal alar base. She had restrictive lung impairment and was treated for gastric cancer at 60 years. There was no history of any intranasal or intraoral discharge or of oral and maxillofacial trauma, nor were there any signs of perinasal inflammation. There were no noteworthy matters in her social, environmental, and family history.\nAn extraoral examination revealed a diffuse swelling of her left nasal alar base without tenderness (Fig. ). Her left nasolabial fold was less distinct than the right nasolabial fold, and her left ala was deformed because of the markedly elevated alar base.\nAn intraoral examination revealed a swollen left upper gingivolabial sulcus (Fig. ). The mucosal surface was normal, and the swelling fluctuated upon palpation but was not tender. No swelling was observed at the right upper gingivolabial sulcus.\nA panoramic radiograph showed no abnormal findings (Fig. ); however, apical periodontitis of her left upper central incisor was observed. Computed tomography (CT) findings revealed a well-circumscribed oval lesion, approximately 19×14×12 mm in size, inferior to her left nasal alar base (Fig. ). The lesion was relatively homogeneous and showed a lower density than the muscle. Absorption of the maxilla and nasal septum was not clearly identifiable on the CT images. Although an area of high density was observed adjacent to the right piriform aperture, it was difficult to confirm the presence of a lesion.\nBecause she declined further evaluation and treatment, a wait-and-see approach was adopted. Although an increase in the swelling was noted in 2005, it remained untreated. Because of the recurrence and further aggravation of the swelling, she finally requested surgical removal of the lesion on 19 January 2010. On 7 May 2010, magnetic resonance imaging (MRI) revealed a cyst-like lesion, measuring approximately 25 mm in maximum diameter, at her left nasal alar base and another cyst-like lesion, measuring approximately 12 mm in maximum diameter, at her right nasal alar base. Both lesions showed high signal intensity on T1-weighted images and low signal intensity on T2-weighted short inversion time inversion recovery images (Fig. ). On the basis of a clinical diagnosis of bilateral nasoalveolar cysts, the cysts were extirpated under general anesthesia on 19 May 2010. The cysts adhered relatively strongly to the mucosa of her nasal cavity floor and the levator labii superioris alaeque nasi muscle. No maxillary reabsorption was observed on either side. Her postoperative course was uneventful, and no recurrence was observed 1 year after surgery.\nA histopathological examination showed that both cysts were lined with single-layered or multi-layered non-keratinized squamous epithelia, which comprised several goblet cells. The cyst walls were composed of a fibrous connective tissue with mild inflammatory cell infiltration and contained seromucous glands in the deeper layers (Fig. ). The histopathological diagnosis was bilateral nasoalveolar cysts. |
A previously healthy 25-year-old man with no known risk factors (no smoking history) visited the emergency room with abdominal and loin pain of 3 h’ duration. The patient's vital signs were stable and physical examination showed no abnormality except tenderness in the right lumbar region of the abdomen and in the paravertebral muscles at L4–L5. Apart from a leukocyte count of 23.79 × 109/L, haemoglobin of 10.8 g/dL and neutrophil ratio of 90.2%, haematological and laboratory values were within normal limits. Normal saline IV infusion and dezocine were administered, and the patient was kept under observation.\nFour hours later, a change in consciousness and cyanosis of the lips and extremities were observed; heart rate and blood pressure could not be obtained. Bedside ultrasound showed accumulation of fluid with a hyperechoic mass in the abdominal cavity. Cardiopulmonary resuscitation (CPR), cardiac stimulants and vasopressors were administered, but the heart rate and blood pressure remained undetectable. The patient died despite resuscitation attempts. Because of the unclear circumstances of death, a medical-legal autopsy was performed.\nAutopsy was performed on the day after death. No obvious abnormalities were observed on external examination apart from mild postmortem lividity. No unusual findings were noted in the head and neck region. With the exception of sternal fracture with surrounding soft tissue haemorrhage caused by CPR, there were no specific findings in the thoracic cavity. Five hundred millilitres of fluid blood and 1 100 g of clotted blood were present within the abdominal cavity. A 2.5-cm longitudinal tear was found in the intimal layer of the right common iliac artery, forming a thrombosed false lumen that involved the abdominal aorta proximally and the left common iliac artery ((A)). The dissection extended 7 cm along the longitudinal axis of the abdominal aorta and 4.5 cm along the left common iliac artery ((B)). The source of bleeding was a 0.7-cm adventitial rupture of the abdominal aortic dissection, located 2.5 cm above the aortic bifurcation ((C,D)).\nGrossly, no atheromatous plaques were found in the aorta or iliac arteries. The heart showed slight hypertrophic change (390 g) with no valvular degeneration or coronary artery anomaly. Other organs had no pathological changes or injuries aside from pallor.\nAll tissue samples were fixed in formalin and embedded in paraffin. Aorta, vertebral arteries, celiac trunk, superior mesenteric artery, renal arteries and common iliac arteries were stained with haematoxylin–eosin (HE) stain, Masson's trichrome stain and elastica van Gieson (EvG) stain. Other organ sections were stained with HE.\nWith the exception of slight intimal thickening, the intact part of the aortic wall showed no histopathological change ((A)). In contrast, a ruptured adventitial and mural thrombus was observed at the aortic dissection site ((B,C)), with evident thickening of the intimal layer and attenuated elastic fibres ((D)). Compared with the aortic dissection site, the intimal rupture site in the right common iliac artery showed hyperplasia of the intimal layer and depletion of smooth muscle cells, which were replaced with maloriented elastic fibres and fibrous connective tissue ((A–F)). There was lack of continuity of the internal elastic lamina. The smooth muscle nuclei at the rupture site showed mild enlargement and hyperchromatism, and had a block-like shape. Smooth muscle cells lay within a characteristic loose myxoid stroma of increased ground substance.\nConsidering the typical histopathological characteristics of FMD, sections of the vertebral arteries, celiac trunk, superior mesenteric artery and renal arteries were submitted for microscopy. With the exception of the fairly normal structures of the vertebral arteries, all arteries showed mild intimal thickening and internal elastic lamina discontinuity. Thickening of the intima, fibroplasia with disorganization of smooth muscle cells and loss of elastica in the muscularis layer were observed in the renal arteries ((A–C)). The left anterior descending artery, left circumflex artery and right coronary artery showed similar changes, with intimal thickening and internal elastic lamina discontinuity ((A)). Furthermore, a regional decrease in myocytes and interstitial fibrosis with capillary proliferation were observed microscopically in the left ventricle and the ventricular septum ((B)); these findings may be related to previous ischemic events.\nThe cause of death was severe blood loss resulting from spontaneous rupture of the abdominal aorta and bilateral common iliac artery dissection attributed to FMD. |
A 35 years old male patient presented with a lump on the dorsum of the right hand of 8 months duration. The swelling recurred twice after the previous excision. The histopathological diagnosis was a giant cell tumor. After the second recurrence, he was offered amputation, through the wrist by the first surgeon and an onco surgeon as well. Patient sought online consultation in the USA and the UK. The suggestion was for ray amputation. On presentation, there was swelling on the dorsum of the hand []. X-ray showed a thin shadow of subchondral bony rim of the third metacarpal head [].\nThe tumor consisting of soft tissue between the adjacent second and fourth metacarpal was excised. The cartilaginous shell of the head of the third metacarpal was excised as well. This exposed the base of the proximal phalanx of the affected middle finger []. A corticocancellous bone graft was interposed between the remaining unstable proximal phalanx and the adjacent proximal phalanx of the ring finger []. Kirschner wires are passed through adjacent proximal phalanges []. The wires were clamped with an external fixator. The wires in the second metacarpal were for added stability only [Figures and ].\nThe proximal half of the proximal phalanges is normally within the skin of the web spaces []. The graft is placed within this skin pocket of the web. Flexion at the metacarpophalangeal joint of the connecting rod of the spanning fixator prevented stiffness at the joint [].\nX-ray in the year 2012 [] and in October 2014 [Figure –], which was 7 years after surgery, showed graft maturation, integration without absorption. The range of motion (ROM) is shown in Figures –. The accompanying Tables and show the ROM, grip strength, and pinch strength. He can play cricket, drive car, can do personal care and office work painlessly. Quick DASH score is 0. Flexion and extension of the proximal phalanges of long and ring fingers at metacarpophalangeal (MP) joints occur together. Similarly, there is ROM of 30°–90° of the affected long finger at the MP joint. Full flexion at the MP joint is noteworthy! Abduction and adduction of the long and ring fingers occur together but do not affect his activities. There is radial deviation of the ring finger at the MP joint level. Wider graft would have kept the digits wider apart and may have been more beneficial. There was also inability of the index to actively extend fully, though fully correctable passively. |
A 27-year-old Caucasian woman was admitted to the Emergency Department of our Institution because of bloody diarrhoea – up to 10 bowel movements per day – during the last month, 3 weeks after quitting smoking. Physical examination showed no abnormalities but confirmed haematochezia on digital rectal examination. Colonoscopy showed continuous severe colonic inflammation with small ulcers from the anus to the descendent colon, classified as grade 3 in Mayo endoscopic sub-score and 3 points in Ulcerative Colitis Endoscopic Index of Severity (UCEIS); complete examination was not performed because of the risk of perforation. Empirical antibiotic treatment with ciprofloxacin and metronidazole, as well as oral and rectal mesalamine were started and partial symptomatic improvement was achieved. Venous thrombosis prophylaxis with subcutaneous enoxaparin, 40 mg per day, was started. At admittance, haemoglobin, white cell count, platelets, fibrinogen and C reactive protein (CRP) were within the reference range. Stool cultures were negative. Cytomegalovirus (CMV) infection was also ruled out in colonic biopsies (polymerase chain reaction – PCR – and, later, immunohistochemistry). As bloody diarrhoea persisted 48 h later, and histopathological examination of colonic biopsies showed crypt distortion, a mixed inflammatory infiltrate of the lamina propria and crypt abscesses suggesting the diagnosis of UC, intravenous methylprednisolone (1 mg per kg of weight, daily) was started. After 3 days of corticosteroids the patient achieved partial clinical response (6 bowel movements per day, Edinburgh index 2 points, CRP within the normal range); nevertheless, 2 weeks later infliximab therapy (5 mg/kg of weight) was started due to sustained clinical activity, with 10 bloody bowel movements per day and a progressive increase of CRP levels, up to 10 mg/dL. Three days after the first dose of infliximab, the patient presented a massive lower bleeding with haemodynamic instability and severe anaemia; CT scan showed active arterial haemorrhage from ascendant colon; a subsequent arteriography demonstrated active arterial bleeding from a colic branch of the superior mesenteric artery; selective transcatheter embolization with platinum microcoils (MicroNester©, Cook Medical) was performed with immediate technical success; nevertheless, the patient persisted with rectal bleeding 2 days after embolization, requiring laparoscopic subtotal colectomy and ileostomy. Pathological evaluation of the colon confirmed the diagnosis of UC. Eight days after surgery the patient was discharged. |
A 67-year-old lady with a long-standing history of dyspnea on exertion and cerebellar abscess 8 years back that was managed conservatively underwent right total knee replacement under spinal anesthesia for severe osteoarthrosis from a nearby center. She had no prior history of vascular risk factors or ischemic heart disease. The patient was well in the immediate postoperative period. On the second postoperative day, she was noted to have acute onset weakness of the left leg followed by right upper limb and was shifted to our center for further management.\nOn arrival, the patient was restless, drowsy with tachypnea and was having grade 0 power in the right upper limb and left lower limb with bipyramidal signs, without features of intracranial hypertension, cranial nerve dysfunction, fever or meningeal signs. She was evaluated with magnetic resonance imaging, which showed fluid attenuation inversion recovery (FLAIR) hyperintensities in the anterior and posterior circulation territories, some showing restricted diffusion suggestive of infarcts of varying ages [Figure –]. Vessel imaging was normal. Her chest radiograph showed multiple shadows, which was initially interpreted as secondary to aspiration []. Arterial blood gas analysis showed type 1 respiratory failure (on O2by mask 6l/mt-p O242.7, p CO231 hco3 26, p H 7.54). She was intubated and ventilated in view of altered sensorium, respiratory distress and hypoxemia.\nPresentation in the early postoperative period following orthopedic surgery with focal neurological deficits, multiple cerebral infarcts and respiratory failure made us consider pulmonary embolism and postoperative stroke as the first possibility. There were no features to suggest fat embolism. Lower limb venous Doppler did not show any deep venous system thrombosis; however, the pelvic veins could not be studied. Her cardiac evaluation, including transthoracic echo and 24-h ECG, were normal.\nThe patient showed improvement in sensorium and weakness by the next week, but was having persistent hypoxemia (on ventilator SIMV, PS 12, PEEP 5, Fi O250% p O253, p CO226, Ph 7.45, with normal lung compliance) and had a few bouts of hemoptysis. A review of her chest radiographs showed nodular opacities in both lung fields appearing contiguous with pulmonary artery shadows, raising a suspicion of PAVF []. Therefore, she underwent computed tomography (CT)-pulmonary angiogram, which showed multiple high-flow PAVF – a total of six in number – with arterial feeders from the right superior, middle and inferior as well as the left inferior pulmonary artery thus explaining her persisting hypoxemia and route for multiple cerebral embolism []. She was not cooperative for transesophageal echo, but we performed a transcranial Doppler with agitated saline contrast, which showed high-intensity transient signals in the middle cerebral artery suggesting the presence of a right to left shunt. There were no other clinical features or family history of hereditary hemorrhagic telengectasia (HHT). However, genetic studies could not be done.\nSubsequently, the patient was extubated and initiated on rehabilitation. She made a good recovery of her motor power (mRS = 2 at discharge) and is doing well on follow-up. |
A 64-year-old male farmer presented to our outpatient clinic with a 2-day history of foot drop and tenderness in the region of the left fibular head. He complained of mild pain radiating from the knee to the ankle and numbness affecting the dorsal aspect of his left foot. The patient reported no history of trauma. In his examination, there was no evidence of degenerative disc disease of the lumbar spine. Careful physical examination revealed nebulous soft swelling below the fibular head. There were no signs of tibialis anterior or peroneal muscle atrophy. However, there was weakness in foot eversion, ankle dorsiflexion, and great toe extension. Plantar flexion and foot inversion were normal. We also found diminished sensation in the first web space of the left foot and positive Tinel’s sign near the fibular head.\nElectromyography (EMG) findings demonstrated denervation of the anterior tibial and peroneus muscles. No abnormality was found in the conduction velocity of the tibial and sural nerves. Radiographs of the knee showed normal findings. Ultrasonography (USG) revealed the 38 × 21 × 16 mm cystic mass localized between the muscle structures. Magnetic resonance imaging (MRI) revealed the presence of a fluid-filled mass, likely presenting a ganglion cyst, around the fibular neck, which compressed the common peroneal nerve ().\nSurgery was performed with a lateral approach under a pneumatic tourniquet. The cyst was seen to arise from the anterior aspect of the proximal tibiofibular joint with a distinct pedicle. It was compressing the peroneal nerve at the level of the fibular neck (). Careful dissection was carried out under loupe magnification to preserve all nerve branches, and the cyst was completely removed after its pedicle was ligated (). The histopathologic evaluation confirmed the diagnosis of a ganglion cyst.\nPostoperatively, the patient was mobilized with an antifoot-drop peroneus splint and treated with intensive physiotherapy. In the early days after surgery, he had no complaints of pain. Sensory loss and motor function were almost fully recovered 2 months after operation. Two years after the operation, there was no recurrence. |
A 49-year-old woman presented with a lump in her neck, which had gradually increased in size during the previous two months. Physical examination revealed a firm nodular mass in the right lobe of the thyroid gland. Her past medical history was unremarkable. Thyroid function tests were within normal range. Ultrasonography revealed a round, relatively well-defined, mixed echoic nodule in the right lobe. The nodule had a markedly hypoechoic portion in the lateral side and had a slightly hypoechoic portion in the medial side. Color Doppler imaging showed increased blood flow within the markedly hypoechoic portion of the nodule ().\nThe fine needle aspiration in this nodule exhibited clusters of cuboidal cells with features of papillary carcinoma. The smear also contained spindle cell groups with non-cohesive arrangement among the tumor cells of PTC. The spindle cells were in a pale to eosinophilic background with associated lymphocytic infiltrate. They had vesicular nuclei, small nucleoli, and indistinct cytoplasmic membrane. Neither significant atypia nor mitotic figures were identified (). Total thyroidectomy with central lymph node dissection was performed.\nGrossly, the right lobe of the thyroid gland showed a well-circumscribed yellow to white, partially encapsulated mass, measuring 2.3×2.0×1.8 cm. Histologically, the tumor consisted of two distinct components: stromal and epithelial. The stromal component consisted of spindle-shaped cells arranged in interlacing fascicles and an abundant fibromyxoid matrix accompanied by thick collagen fibers. The stromal cells had neither nuclear atypia nor mitotic figures. Occasional lymphocytic infiltration or extravasated red blood cells were also identified in the center and periphery of the tumor stroma. Epithelial components had features of PTC (). The tumor was confined to the thyroid parenchyma. Metastatic tumor tissue was detected in four of five resected central lymph nodes without stromal components. Immunohistochemically, the spindle cells exhibited focal cytoplasmic staining with desmin and diffuse cytoplasmic stain with smooth muscle actin. In contrast, they showed negative staining with cytokeratin and thyroglobulin. These findings are consistent with previous reports of PTC-NFS.\nWe further performed immunohistochemical analyses of β-catenin, transforming growth factor-β (TGF-β), Smad-2 and -4, and matrix metalloproteinase (MMP)-3 and -9, which have been reported to be useful in distinguishing FM from NF. The immunostaining results for the stromal spindle cells are summarized in . The spindle cells showed nuclear and cytoplasmic β-catenin expression and cytoplasmic TGF-β expression. Smad-2 and -4 were also localized in the nuclei of the spindle cells. In contrast, the spindle cells lacked MMP-3 expression and showed scant cytoplasmic staining with MMP-9 (). |
A sixty-two-year-old Caucasian female presented to the emergency room with complaints of acute right-sided vision changes for the past 12 hours. She reported image distortion, blurriness, and loss of central vision with “flashing lights.” She also experienced mild injection of her right conjunctiva and stated that she has experienced episodic blurry vision accompanied by headaches for the past several months.\nSignificant medical histories included CVID and ulcerative colitis. Her initial immunologic evaluation revealed a low IgG level at 223 mg/dL, normal IgA, and IgM values. The patient was placed on daily sulfamethoxazole-trimethoprim prophylaxis in the setting of recurrent upper respiratory tract infections including sinusitis and bronchitis. The patient failed the prophylactic antibiotic therapy as she continued to have recurrent, persistent sinusitis. The patient was eventually placed on immunoglobulin therapy, and her infections subsided. In addition, the patient has notably complained of intermittent blurry vision in the right eye for several years. Acutely, the patient displayed worsened right eye vision distortion and was evaluated in the emergency room.\nIn the emergency room, the physical exam was significant for mildly injected right conjunctiva and visual acuity of 20/70 in the right eye and 20/25 in the left eye. The right intraocular pressure (IOP) was measured at 34 mmHg and 12 mmHg in the left. Color vision was intact bilaterally with reactive and equal pupils. No signs of trauma, photophobia, eye drainage, or hyphema were noted. Extraocular eye movements were intact, and no other focal deficits were noted on neurological exam.\nThe ophthalmology slit lamp examination showed 4-5 trace cells in anterior chamber of right eye confirming anterior chamber uveitis. There were no keratic precipitates, and the iris appeared normal on direct illumination bilaterally. The left eye exam did not show any cells in the anterior chamber or evidence of corneal edema. Gonioscopy and dilated fundus examination were unremarkable. Cup to disc ratio was within normal limits, and no afferent pupillary defects were noted.\nThe patient was acutely treated with three rounds of dorzolamide/timolol and 0.2% brimonidine with a subsequent decrease in the right eye's IOP to 24 mmHg. She was discharged with brimonidine 0.2% and dorzolamide/timolol two times a day in the right eye. After four days of the initial presentation, a follow-up IOP revealed 41 mmHg of the right and 18 mmHg of the left eye. There was poor foveal reflex in the right eye, and 0-1 cells were noted in the anterior chamber. The patient was diagnosed with Posner-Schlossman syndrome given the clinical findings of elevated right unilateral ocular pressure and anterior segment inflammation with right blurry vision. |
A 53-year-old Caucasian woman was referred to our institution because of the suspicion of peritoneal carcinomatosis, raised by the findings of ascites at a transvaginal ultrasound performed as a yearly routine exam; a pre-surgical staging exam with computed tomography (CT) scan show thickening of the gastric walls, multiple omental nodules and ascites ().\nHer previous personal history was unremarkable and she denied any clinical symptom or cancer history.\nAt our hospital, she underwent an esophagogastroduodenoscopy and colonoscopy, with results negative for gastric/colon cancer.\nHer comprehensive metabolic profile revealed mild liver dysfunction with an alanine transaminase of 77 U/L and aspartate transaminase of 71 U/L. When tumour markers were assessed, CA125 demonstrated increased levels of 290 U/mL (normal values <35 U/mL), whereas carcinoembryonic antigen, CA 19.9 and other immunohistochemical markers were within the normal ranges. Serological assessment of HIV, hepatitis C virus and hepatitis B virus were negative.\nTen days later, the patient underwent an ultrasound-guided biopsy () with a diagnosis of suspicious carcinoma from the an unknown primary site.\nAfter 2 weeks, the patient received a CT scan of the thorax (to complete pre-operative staging), demonstrating a spontaneous (with no therapy) dimensional and numerical reduction of peritoneal lesions in the upper abdomen, partially included in the chest CT scan, as well as resolution of peri-hepatic and peri-splenic ascites ().\nSince there was no evidence of primary cancer at pre-operative examinations and the second CT scan revealed a partial resolution of peritoneal implants and ascites without therapy, the suspicion of an infectious disease was raised.\nThe pathological evaluation of the biopsies performed on the omentum and peritoneum revealed the presence of lymphoid aggregates with a central core of epithelioid cells with large eosinophilic cytoplasm, without atypia or mitosis () and with no immunohistochemical marker of oncologic malignancy. Due to the presence of necrotic nodules with histiocytes and giant cells, a Ziehl–Neelsen stain was performed to identify bacilli (), whose presence was then confirmed with molecular assays.\nBecause of the uncertain result of the biopsy and the conflicting results of the 2 CT scans, in order to rule out malignancy with certainty, the patient underwent a laparoscopic surgery in 2 weeks.\nDefinitive histological diagnosis excluded the presence of malignant cells and reported a necrotising inflammation caused by non-tuberculous mycobacteria. Hence, the patient was sent to a hospital with expertise in infectious diseases.\nA follow-up CT scan performed 1 year later confirmed a complete recovery of peritoneal findings. |
A 68 year old Pakistani male was referred to the outpatient oncology clinic at our center with a three month history of a self-discovered, progressively increasing palpable lump in the left breast. He had sought medical attention for increasing size of the lump with recent development of pain. His past history was significant for type 2 diabetes and long term ethanol use. There was no family history of breast disease.\nPhysical examination revealed bilateral gynaecomastia along with a 3 × 3 cm sized, relatively well circumscribed subareolar mass which was firm and mildly tender. The overlying skin appeared normal. There was evidence of palpable lymphadenopathy in the ipsilateral axilla.\nBreast ultrasonogram confirmed bilateral gynaecomastia along with an irregular, hypoechoic, non-compressible mass in the subareolar region of the left breast with associated ipsilateral axillary adenopathy. The breast nodule was subjected to fine needle aspiration biopsy (FNAB) which revealed atypical cells, following which the patient underwent baseline blood workup and complete staging with computed tomographic (CT) imaging. His preoperative blood workup was essentially normal although liver function tests did reveal a marginally elevated alanine transaminase (ALT) at 64 IU/L. Serological tests for hepatitis B and C were negative and ultrasound of the liver showed mild fatty infiltration. After a core biopsy confirmation of malignancy, he underwent a modified radical mastectomy (MRM) with axillary lymph node dissection. His postoperative course was uneventful except mild seroma formation at the surgical site (Figure ).\nGross histopathology of the surgical specimen revealed a firm greyish white vaguely circumscribed lesion just beneath the nipple measuring 2.8 × 2.5 × 2 cm. Microscopically, an infiltrative tumor was identified which was composed of discohesive sheets of pleomorphic cells. Tumor cells showed moderate to marked nuclear pleomorphism along with moderate cytoplasm (Figure ). There was absence of an intraductal component whereas gynaecomastia was evident in the surrounding breast tissue characterized by breast ducts lined by double layer of epithelium with mild ductal hyperplasia. Breast acini or lobules were not identified. The lesion was reported as grade III on a scale of I to III according to modified Bloom Richardson grading system with invasion of the dermal lymphatics. Surgical margins were negative and six of the eleven dissected axillary lymph nodes demonstrated evidence of disease. The tumor was pathologically staged as pT2pN2Mx.\nTumor cells were negative for E-cadherin immunostain (Figure ) and thus based on morphology and immunprofile a diagnosis of invasive lobular carcinoma, pleomorphic variant was rendered. The neoplastic cells were strongly positive for estrogen receptor expression but only displayed weak positivity for expression of the progesterone receptor. Her2-Neu staining by immunohistochemistry revealed a score of +1 and the Ki-67 proliferative index was 20-25%.\nThe patient was recommended adjuvant chemotherapy to be followed by chest wall and axillary irradiation and hormonal therapy. He completed 4 cycles of adjuvant chemotherapy without any evidence of recurrence and was subsequently lost to followup. |
A 65-year-old man with severe chronic obstructive pulmonary disease (COPD) was admitted with a four-day history of chest pain and worsening shortness of breath. He explained the chest pain started suddenly when he tried to reach out for something on his computer table. It was located on the right anterior chest, sharp in nature, 7/10 in intensity, pleuritic, and worse with coughing and deep breathing. He had a past medical history of severe COPD with frequent exacerbations recently necessitating multiple antibiotics and steroid courses, coronary artery disease, gastroesophageal reflux disease (GERD), and hypertension. He had a 30-pack per year smoking history and quit about 10 years ago. He was a retired fireman living with his family.\nVitals signs in the emergency department (ED) were stable; he was breathing on ambient air. The physical examination demonstrated decreased breath sounds bilaterally without any wheezing or Ronchi. Moderate tenderness was present in the mid-axillary line in the fifth intercostal space, but no other abnormalities were noticed. Laboratory investigations were negative for any leukocytosis, troponin, or any other abnormalities. The electrocardiogram (EKG) showed a normal sinus rhythm. Computed tomography (CT) angiography was done to rule out pulmonary embolism (PE). The CT was negative for PE but showed mild peribronchial infiltrate in the right middle lobe and posterolateral lung herniation between the seventh and eighth ribs, with minimal subcutaneous emphysema along the right chest wall (Figure ).\nThe patient was admitted to the hospital and managed conservatively on broad-spectrum antibiotics, including vancomycin, levofloxacin, and 40 mg per day of prednisone. Two days later, his face swelled up suddenly with a change in the quality of his voice while he was eating dinner. An examination showed a swelling in the neck, diffuse crepitations on his body involving the face, all the way down to the buttocks. Repeat CT chest and neck showed extensive subcutaneous emphysema in the face, neck, chest, and mediastinum with a right-sided pneumothorax at the level of the previous lung herniation (Figures -).\nThe prevertebral and retropharyngeal air was demonstrated as compressing the oropharynx (Figure ). His oxygen requirement went up to 6 liters nasal cannula. A blowhole incision was made on the anterior chest wall, and he was observed in the medical intensive care unit (MICU). Surgery to close the defect was deferred due to his other comorbidities and the higher risk of post-operative complications. He improved gradually over the course of the next few days, completed the course of antibiotics with steroids, and was discharged to a rehabilitation center and did well post-discharge. |
A male patient, 46 years old, 192 cm height and 85 kg weight, in January 2016 comes to our observation in relation to the appearance of an acute pain in the anterior superior region of the left shoulder which radiated to the lateral cervical and deltoideal region with an increase of the painful symptomatology at night.\nThe CARE reporting guidelines were adopted for the presentation of this case report.[\nThe patient reported not having had any direct trauma or being affected by systemic, metabolic, or endocrine disorders. The patient had practiced rest for a week, local intermittent application of ice, and oral NSAID intake under medical indication, without substantial improvements in the painful symptomatology. At our observation, the clinical evaluation of the left shoulder highlighted: lack of presence of swelling, reddening of the skin, or alterations of surface temperature; severe functional limitation during passive and active movements of the shoulder on all the range of motion planes; and intense pain during palpation of the acromioclavicular joint.\nThe patient reported a pain value of 8/10 measured with the visual-analogical spatial (VAS) scale. A radiographic examination and an echotomographic examination of the shoulder were performed sequentially. Both instrumental examinations showed the presence of a calcific deposit inside the acromioclavicular joint (Figs. and 2) with the absence of significant alterations of the bone structures of the joint and of the rotator cuff tendons, neither the presence of a reactive bursitis.\nIn particular, the echotomographic examination, compared to the radiographic examination, better highlighted the exact intra-articular localization of the calcification in the left acromioclavicular joint. The calcium deposit had a hyperechoic appearance, homogeneous overall in the absence of a posterior shadow, thus creating an ultrasound picture of a fluid-soft calcific image (nubecular appearance) with a maximum diameter of 12.2 mm. The calcification also appeared surrounded by diffuse and inhomogeneous hypoechogenicity in relation to the associated intra-articular inflammatory reaction (Fig. ).\nIn relation to the clinical characteristics of the pathology and of the data obtained from the imaging, a diagnosis of an intra-articular calcification in reabsorption phase, of the left acromion-clavicular joint, was postulated.\nA therapeutic guideline for the treatment of intra-articular calcifications of the acromion-clavicular joint has still not been described. However, based on the experience gained by our team in the treatment of calcific tendinopathy of the shoulder and the results described by other research groups for this latter type of pathology,[ it was decided to use the same procedure of a UGPT of a calcific tendinopathy of the shoulder. After informing the patient about the technique and the therapeutic options and after obtaining the informed consent, the UGPT of the intra-articular calcification was performed. Additionally, the patient has provided informed consent for publication of the case.\nThe patient was placed on an examination bed in a supine position with the left upper limb in a neutral position.\nThe equipment used was prepared as follows:\nan 8-mL syringe with 2% lidocaine hydrochloride,\ndifferent 10 mL syringes with about 6 mL of physiological solution,\na 5-mL syringe with 1 mL of methylprednisolone acetate 40 mg/mL,\na 16-gauge (G) needle,\na 21-G needle,\nsterile gloves,\nsurgical cutaneous disinfectant (10% iodopovidone),\nbactericidal disinfectant (chlorhexidine),\ndry ice,\nultrasound probe, and\nsterile polyurethane probe cover.\nAfter the echotomographic evaluation to identify the exact location of the calcification and the best access route for the introduction of the needle, an accurate and wide disinfection of the shoulder, with surgical cutaneous disinfectant, and of the ultrasound probe with 70% alcohol solution, with subsequent placement of a sterile polyurethane probe cover were carried out.\nThe following procedure was adopted to treat the calcific formation:\nSkin disinfection and target centering: A new skin disinfection of the region of interest with chlorhexidine was performed. The probe was then positioned in such a way as to allow an optimal visualization of the area to be treated.\nLocal anesthesia: Through the 21 G needle, 8 mL of lidocaine hydrochloride was injected, under ultrasound guidance, along the path of the needle, from the skin to the pericalcific level to anesthetize the area. The needle was then removed and 2 minutes were awaited to allow the anesthetic to make its local effect.\nIntroduction of the needle under ultrasound guidance within the calcification: A 16-G needle fitted with a 10-mL syringe partially filled with the saline solution was inserted along the short side of the probe. The needle was ecographically followed along its entire journey, from the area of its cutaneous entry to the target (intra-articular calcification) (Fig. ). For a better visualization of the needle, it is advisable to maintain a trajectory as parallel as possible to the long axis of the probe.\nSuction Procedure: In this phase, the alternate compression and release of the syringe plunger have been applied so that the physiological solution was injected and resuctioned inside it. This impulse was applied until the calcific amorphous material was completely aspirated inside the syringe, which will result in a modification of the color of the physiological solution due to the presence of calcium fragments. Such process may be repeated with different syringes, but without removing the needle from inside the joint, until the calcific content is totally expelled.\nTreatment with cortisone: Without removing the needle from inside the articular location, a new 5-mL syringe was placed and 1 mL of methylprednisolone acetate was injected inside the joint.\nPosttreatment ultrasound check: Once the needle has been extracted, the skin is again disinfected with chlorhexidine and after a few minutes, an ultrasound check of the treated part was performed to rule out any local complications. After that, the entry point was covered with sterile gauze and dry ice was applied for about 15 minutes.\nThe patient reported no pain throughout the treatment procedure.\nAfter being clinically rechecked about 30 minutes after the end of the treatment, the patient was discharged with the following indications: absolute functional rest for the first 3 days; relative functional rest for further 15 days, taking care to avoid elevation and abduction movements of the joint above 90°, and avoid the use of the left upper limb to move heavy loads; ultrasound clinical examination after 3 weeks from the treatment; and application of ice packs during the first 3 hours after the intervention and intake of analgesic drugs only if needed.\nNo local complications after the UGPT were detected. The patient did not need local or systemic therapy for pain management after treatment.\nThe clinical and instrumental check carried out 3 weeks after the treatment highlighted the disappearance of nocturnal pain, a significant pain reduction, referring to a VAS scale of 1/10 and a recovery of the entire range of movement (ROM) of the left shoulder on all movement planes during both active and passive mobilizations.\nThe ultrasound evaluation showed the presence of small intra-articular hyperechogenic fragments with disappearance of the intra-articular inflammatory reaction (Fig. ).\nWhen the patient was contacted about 3 years after the treatment, he reported that during this period he had not experienced any particular or functional disorder to the treated shoulder. He also reported that he had recommenced the sporting activities that he had discontinued previously (basketball/tennis) due to his injury. The UGPT we performed as a treatment of a calcification of the acromion-clavicular joint has been a valid and nonpainful therapeutic treatment, of rapid execution, low cost, minimally invasive, which did not show complications and of easy repeatability. |
A 38-year-old male patient with a 40-pack-year history of smoking initially presented with sudden-onset right-sided facial nerve palsy which was diagnosed as idiopathic Bell's palsy and was treated with prednisolone. Three weeks later, the patient developed a new left-sided facial palsy in addition to his unrecovered right-sided facial palsy. The patient denied any systemic symptoms except for a mild headache. His physical examination showed bilateral lower motor neuron facial nerve palsy. Outside of the facial nerve paralysis, he was neurologically intact, and his physical examination revealed no abnormalities. Further workup was carried out to rule out autoimmune, metabolic, infectious, and vasculitic causes and revealed no abnormality. A chest X-ray showed a right upper lobe opacity which prompted a computed tomography (CT) scan of the chest which showed a well-defined, suspected soft-tissue lesion in the right upper lobe with extensive hilar and ipsilateral mediastinal lymphadenopathy (Fig ).\nAt this point, the differential diagnosis included sarcoidosis and primary lung cancer given the presenting complaint was bilateral facial nerve paralysis. Because the intrapulmonary lesion was too distal to be reached by flexible bronchoscopy, video-assisted mediastinoscopy was performed and revealed gross right-sided mediastinal invasion. Multiple biopsies from lymph nodes were taken and revealed metastatic poorly differentiated adenocarcinoma of the lung. Accordingly, positron emission tomography (PET)/CT and magnetic resonance imaging (MRI) of the brain were performed to stage the cancer. Brain MRI with contrast showed normal enhancement of the seventh and eighth cranial nerves with neither brain metastasis nor leptomeningeal metastasis. The PET/CT showed a metabolically active right upper lobe lesion with extension into the mediastinum and active ipsilateral mediastinal lymph nodes but no distant metastasis. Due to our high clinical suspicion of leptomeningeal involvement, a lumbar puncture was performed. Cerebrospinal fluid (CSF) cultures and cytology were all negative. Additionally, a paraneoplastic panel in his serum and CSF was negative as well. At this point, his clinical stage was considered cIIIB; cT4 (extension) N2 M0. He underwent chemoradiation as his definitive therapy. The patient received platinum-based doublet chemotherapy with a total of 60 Gray of radiation. He tolerated the chemotherapy and radiation well but had no resolution of his facial paralysis. Nine months later, the patient presented to the emergency department with severe headache and generalized tonic-clonic seizures. On physical examination, he was cachectic and still had bilateral facial weakness. A brain CT revealed acute hydrocephalus. An urgent external ventricular drain was inserted. A new brain MRI showed no new changes. Cytology of the withdrawn CSF was positive for metastatic adenocarcinoma. An abdominal and pelvic CT scan was obtained which showed newly developed liver metastases as well. Given the patient's disease progression and deteriorating condition, palliative care was offered, and the patient succumbed to his disease. |
A 31-year-old man with no allergies attended another otolaryngology clinic for a left submandibular swelling and was diagnosed with submandibular gland stone. Intraoral removal of the stone was attempted at under local anesthesia, but the stone migrated deeply and could not be removed. The stone remained in its deep location over 4 months of follow-up. Therefore, he was referred to our department for sialendoscopy. Upon presentation to us, he had no past medical history except for obesity (body mass index: 26). Computed tomography (CT) showed a stone (4.4×4.0×3.9 mm) located in hilar region of left submandibular gland (). We performed outpatient sialendoscopy of the palpable stone under local anesthesia, but the sialendoscope could not reach the stone due to a ductal stricture. Therefore, several weeks later, we performed intraoral removal of the stone under general anesthesia. After retraction of the tongue using a tongue retractor, a 3-cm mucosal incision was made on the lingual aspect of the retromolar region. The exposed sublingual gland was retracted laterally, and Wharton’s duct and the lingual nerve were identified. After careful dissection along Wharton’s duct to the hilum of the submandibular gland, the submandibular gland was raised with extraoral finger pressure and the medial aspect of the submandibular gland was exposed. An incision was made on the hilum after palpation of the stone, and the stone was removed. The hilum was sutured to the oral mucosa, and an orifice was made for salivary flow. Operation time was 28 minutes and bleeding was negligible. The patient was discharged 1 day after surgery without complications; he had no lingual nerve paralysis nor dyspnea. However, that night he experienced dyspnea when supine and was transported by ambulance back to our hospital. The left sub-mandibular region was moderately swollen, the oral floor was slightly swollen, and there was no bleeding from the wound. CT showed pharyngolaryngeal edema from the left submandibular gland to the epiglottis (). The fiberscopic image showed edematous swelling from the epiglottis to the left lateral arytenoid cartilage (). After discussion with anesthesiologists and otolaryngologists, we intubated and mechanically ventilated the patient and admitted him to the intensive care unit. He was given antibiotics and steroids. Although the wound was released by suture removal after intubation, there was no bleeding or hematoma. Follow-up CT and fiberoptic nasal endoscopy 4 days later showed that the pharyngolaryngeal swelling had mostly resolved, and he was extubated and discharged. |
A 64-year-old Caucasian male with a medical history of hypertension and symptomatic bradycardia status-post pacemaker implantation presented to the emergency department with the chief complaint of swelling of the right upper extremity for three weeks. It progressed to a generalized swelling of the right shoulder and right side of the neck, restricting his arm movements. He also voiced the concern of worsening shortness of breath which started with the swelling. He did not report any personal or family history of thrombophilia. There was no previous history of any excessive upper extremity exertion or catheterization in the neck. Vital signs on presentation were a blood pressure of 127/61 mmHg, pulse rate of 79/minute, good volume, regular rhythm with no radio-radial or radio-femoral delay, respiratory rate of 19/min with a saturation of 93% on 3-liters nasal cannula, and temperature of 97.5oF. Physical examination revealed right upper extremity swelling without any sensory or motor deficits, right-sided neck swelling, and right-sided facial plethora. Examination of the contralateral arm, as well as the cardiovascular and respiratory systems, was normal. Biochemical investigations were within normal limits. The patient underwent a right upper extremity duplex ultrasonography which revealed an acute non-occlusive thrombus in the proximal right internal jugular vein (Figure ) and right subclavian vein (Figure ) at the cephalic vein confluence. At this point, Factor V Leiden, anti-thrombin III, protein C, and protein S levels were ordered which were normal. The patient was admitted to the medical ward and an intravenous heparin infusion was initiated. Chest radiography did not reveal any cervical rib, and computed tomography (CT) pulmonary angiography showed no evidence of pulmonary embolism. The swelling improved over the course of two days, and the patient was switched to oral apixaban for anticoagulation. He was discharged on apixaban for six months, and a complete resolution of his signs and symptoms was noted at his three-month follow-up examination. |
The patient was a 24-year-old woman, who was diagnosed with CdCS in infancy. She had grade 1 intellectual disability and abnormal gait due to neuromotor malfunction. General anesthesia was advised, because oral examination was not possible owing to the intellectual disability.\nThe caregiver stated that internal organ malformations were absent. She was 150 cm tall and weighed 42 kg. Only partial airway evaluation and parental interviews were performed, since preoperative examinations including blood tests were not possible because of poor coordination. Although the American Society of Anesthesiologists (ASA) physical status classification could not be made accurately, it was estimated to be ASA 2, because the patient appeared physically was healthy and had no other medical condition disease. No abnormalities were noted in with the aperture and cervical spine; However, she had a difficult airway, which was caused by class 2 malocclusion and micrognathia, with a broad nasal bridge and epicanthal folds, which are characteristic of CdCS.\nDental examinations and treatments were planned under outpatient general anesthesia, and the following procedure was used for inducing general anesthesia.\nThe patient refused to sit on the dental chair, but her parents managed to soothe her and mask induction was started with inhalation of 50% nitrous oxide and oxygen and sevoflurane (4 vol%), followed by sedation. Airway obstruction occurred as the patient calmed down. Thus, positive pressure ventilation was performed using the oropharyngeal airway. Patient monitors for evaluating blood pressure in a noninvasive manner, temperature, pulse oximetry, and electrocardiography were attached simultaneously. An intravenous line was obtained and rocuronium 20 mg was injected. A cotton swab soaked with epinephrine (Bosmin®R) (1 mg/1 cc) was applied to both nasal cavities to facilitate smooth nasotracheal intubation.\nNasotracheal intubation was performed after sufficient neuromuscular blockade. First, the endotracheal tube (ETT) was passed through the left nostril, using a laryngoscope with a size 3 Macintosh curved blade. However, intubation was impossible due to a Cormack-Lehane grade of 4, i.e., the vocal cord was not exposed (). After immediate removal of the instrument, oxygen supply and anesthesia depth were maintained using 100% oxygen and 4 vol% of sevoflurane. Subsequently, video laryngoscopes (AceScope, Acemedical Co., Korea) were prepared, and the vocal cord was easily exposed using the same laryngoscope blade ().\nThe ETT was too thick to pass through the vocal cord but could pass through the nostrils, even though an ETT with an inner diameter (ID) of 6.0 mm and outer diameter (OD) of 8.6 mm was selected after considering patient's age, sex, and height. Intubation was possible with an ETT with an ID of 5.5 mm, although it was difficult due to the wider OD of the anterior part of the balloon (). An inflation pressure of 30 cmH2O was applied without inflating the balloon after intubation. Thus, we decided to replace the ETT with one with a smaller OD because absolutely no air leaked was observed. Nonetheless, we proceeded with the intubated tube, since the expected treatment time was short and repeated intubation attempts were considered to be detrimental to the patient. Dexamethasone 10 mg was administered to prevent post-intubation stridor. Desaturation or bleeding were not observed during the induction process.\nThe ETT was placed at a depth of 23 cm from thenares. Anesthesia was maintained with 50% nitrous oxide, oxygen, and 2 vol% of sevoflurane. The peak airway pressure was between 15 and 17 cmH2O when the partial pressure of end tidal carbon dioxide was maintained at approximately 30 mmHg using the volume control mode. A dentist from the department of preventive dentistry performed the oral examination. The patient's oral hygiene was very poor: extensive calculus deposition was observed. Oral prophylaxis was performed with an ultrasonic scaler. The follow-up visit was planned after 18 months.\nA neuromuscular-blocking reversal agent was administered after the procedure. Extubation was performed with a positive pressure of 30 cmH2O, following recovery from anesthesia and confirmation of spontaneous breathing and eye opening. Vital signs were observed within approximately 3 min while providing 100% oxygen, and the patient was shifted to the recovery room. For the dental treatment procedure required 95 min and 130 min for anesthesia. No complications were observed during recovery and the patient was discharged. |
An 11-month-old boy was referred to a tertiary center for failure to thrive, poor muscle tone, short neck, kyphosis, and unusual spacing between teeth. He was diagnosed with infantile HPP after repeated low ALP activity test results and radiographic assessment of severe rickets-like skeletal changes and tongue-like lucencies projecting into the metaphyses. He was hospitalized multiple times for pneumonia likely related to musculoskeletal manifestation of HPP, which required treatment with intravenous antibiotics. At age 9 years, he developed persistent headaches; a magnetic resonance imaging (MRI) cranial scan confirmed craniosynostosis with Chiari malformation and cerebellar tonsillar herniation. He underwent craniovertebral decompression, with removal of the posterior arch of C1; a ventriculoperitoneal shunt was inserted to relieve intracranial pressure. He subsequently underwent 2 shunt revisions.\nThe patient experienced multiple fractures, starting at age 17 years, when he sustained bilateral femoral fractures when jumping off a wall; this required bilateral intramedullary rod insertion. At age 18 years, he sustained a right tibial fracture while jumping. Bone healing was delayed, but the fracture eventually healed satisfactorily. At age 20 years, he sustained bilateral femoral fractures when he rolled off his bed during a seizure and required rehabilitation for approximately 8 months.\nOver the first 18 years of life, the patient was hospitalized 8 times for a total of 43 days (Table ). Of these hospitalizations, 5 separate admissions required a stay of ≥5 days; the stay for insertion of a ventriculoperitoneal shunt was 14 days.\nOutpatient specialist visits represented a significant proportion of the healthcare resource utilization by this patient (Fig. ). Most of the outpatient specialist visits required consultation with providers in 12 specialties, including pediatric dentist (38 visits), pediatric endocrinologist (32 visits), neurosurgeon (27 visits), and general pediatrician (19 visits; Table ). Outpatient management consisted of diagnostic imaging procedures (Table ). The most frequent procedures were radiography of the limbs and spine, performed on 18 and 12 occasions, respectively; the patient also underwent 14 MRI cranial scans. Dental surgery and tooth extraction were performed as day case procedures for management of dental carries and malocclusion on 3 occasions. |
An 8-year-old white girl with no family history of skin cancer except for skin cancer in her grandfather complained of 4 weeks of pressure-like headaches, mostly in the posterior occipital area, and intermittent nausea with several episodes of emesis in June of 2012. A physical examination was significant for decreased sensation to light touch over the right side of her face and her right upper extremity. Magnetic resonance imaging (MRI) of her brain revealed a large heterogeneous skull base mass. Chordoma was suspected over chondrosarcoma, and the diagnosis was confirmed by pathology after tissue was obtained during an endonasal surgical resection. Resection of the chordoma left residual tumor in her lateral skull base at the hypoglossal canal and jugular foramen. She was referred to a proton facility where she received fractionated proton and photon beam therapy: 77.4 Gy total, 59.4 Gy (cobalt gray equivalent) in 33 fractions with protons and 18 Gy in 10 fractions with photons. She was returned to our care afterwards. Scheduled MRIs showed decreasing lesion size through postoperative year 2. However, she experienced acute cranial nerve 6 palsy approximately 2.5 years after surgical resection. An MRI showed a 3×1.5×4 cm mass in the resection cavity with signal change suspicious for dedifferentiation. A subsequent biopsy obtained during a retrosigmoid craniotomy confirmed high-grade sarcoma. She has since completed 17 cycles of ifosfamide and etoposide alternating with vincristine, doxorubicin, and cyclophosphamide with the last round in April 2016. She began maintenance capecitabine on 26 April 2016. The most recent MRI, approximately 14 months after her second surgery, showed a mild interval decrease in tumor size.\nMRI of our patient’s brain with and without contrast at her initial presentation showed a large heterogeneous mass emanating intracranially from the base of her clivus and extending through the foramen magnum to C1–C2. A small portion was anterior to C1–C2 in her nasopharyngeal soft tissues with extension through her left hypoglossal canal. Posteriorly, the lesion displaced her pons and medulla. Multiple cranial nerves were not well seen and were either displaced or encased by the mass as it filled the right cerebellomedullary angle. The lesion was T1 isointense to muscle (Fig. ), and only mildly enhanced in portions (Fig. ). The lesion had a predominantly very bright T2 signal (Fig. ). There was restricted diffusion with bright diffusion-weighted imaging and dark apparent diffusion coefficient signals, indicating hypercellularity (Fig. , ).\nFollow-up MRI 2.5 years after the initial MRI, and after surgery and proton beam therapy, showed local recurrence. The recurrent tumor was centered at the rightward aspect of her clivus and extended superiorly to the dorsum sella and inferiorly to the level of the occipital condyle. The signal intensity of this mass was intermediate to dark, isointense to gray matter on T2-weighted images in contrast to the very T2 hyperintense signal like cerebral spinal fluid as seen on the initial presentation (Fig. ). In addition, on follow-up the lesion was avidly enhancing post-contrast on T1-weighted imaging (Fig. ) as compared to the initial lesion where there was only mild enhancement.\nThe biopsy from the initial lesion showed classic chordoma histology with physaliphorous cells with multivacuolated cytoplasm mixed with epithelioid cells arranged in anastomosing cords, clusters, and chains. There was a myxoid matrix and occasional fibrous septa (Fig. ). No immunohistochemical stains were performed as histology provided a definitive diagnosis.\nHematoxylin and eosin staining of the recurrent tumor obtained during our patient’s second surgery, however, showed a high-grade anaplastic spindle cell neoplasm. There were areas of geographical necrosis with no particular architectural pattern visible at low magnification (Fig. ). In the higher power images, the cells were very pleomorphic with epithelioid and spindle shapes, and there were mitotic figures present (Fig. ). Myofibroblastic tumor and fibrosarcoma, leiomyosarcoma, and rhabdomyosarcoma were ruled out with immunohistochemistry. Vimentin was positive, consistent with sarcoma in general. Ki-67, a proliferation index that is normally very low in a chordoma, was scored at 80 %, indicating rapid cellular division. Epithelial membrane antigen (EMA), cytokeratin, and brachyury were negative, indicating full dedifferentiation. |
An 86-year-old man was admitted to our hospital with a 3-day history of acute abdominal pain. The patient had no previous medical history. A physical examination revealed marked right upper quadrant pain with normal bowel sounds. Murphy’s sign was positive. His vital signs were within the normal range. Abdominal ultrasonography revealed an enlarged gallbladder with surrounding fatty tissue inflammation. The blood biochemistry was essentially normal, including C-reactive protein (1.9 mg/dL) and total bilirubin (1.4 mg/dL) levels. An enhanced computed tomography examination revealed an enlarged gallbladder and incarcerated gallstone. Gallbladder wall enhancement was discontinued, and the fundus of the gallbladder was located centrally beyond the round ligament (Fig. ). The round ligament was attached to the right umbilical portion, which was associated with the anomaly of the intrahepatic portal vein system (Fig. ). Magnetic resonance cholangiopancreatography demonstrated the root of the cystic duct, while the middle portion of the cystic duct was unclear (Fig. ).\nWith the preoperative diagnosis of grade II acute gangrenous cholecystitis according to the 2018 Tokyo guidelines or gallbladder torsion, LC was planned. The first port was inserted into the umbilicus, and an enlarged and reddish gallbladder was observed. The gallbladder was swollen; however, torsion was not detected intraoperatively. The second port was placed in the epigastric area, while others were at the right hypochondriac and right lumbar regions. The gallbladder was attached to the left side of the hepatic round ligament (Fig. ). The cystic duct and the cystic artery were located in the normal positions. Severe inflammation and the narrow working space between the epigastric port and the gallbladder made it difficult to dissect Calot’s triangle; however, the cystic duct and the cystic artery were resected after the critical view of safety was confirmed. Due to the severe inflammation, a subtotal cholecystectomy was finally performed. The operative time was 178 min, and intraoperative blood loss was 50 mL. The pathological diagnosis was acute cholecystitis with a mucosal ulcer. The patient was discharged on the fifth day after surgery without postoperative complications. |
A 59-year-old man visited a local hospital with a 3-year history of occult blood and mucus in the feces, and with a long history of intermittent episodes of diarrhea and constipation. The patient had no history of steroid use, chronic liver disease, viral hepatitis, autoimmune diseases, or neoplasm. Physical examination was unremarkable except for a lump palpable on the anterior wall of the rectum 5 cm from the anal verge. Occult blood was detected in his feces. Other laboratory studies were normal, and carcinoembryonic antigen was not elevated. A subsequent endoscopy of the rectum revealed a 5-cm ulcerative nodule in the anterior wall of the rectum, 5 cm from the anal verge (Figure ). Initial endoscopic biopsy was performed in the local clinic and histopathologic finding was a rectal adenoma with low-grade dysplasia. After three months, a repeated colorectal endoscopy showed an ulcerative rectal mucosa and granulation tissue with no evidence of malignancy. However, the findings of abdominal magnetic resonance imagining (MRI) revealed thickening of the mucosa-submucosa, raising suspicion of rectal carcinoma (Figure ). In addition, clinical symptoms persisted despite medical treatment. Therefore, surgical resection of the rectum was performed. The final pathology report was no malignancy. Thus, the patient was referred to our hospital for the pathology consultation.\nGrossly, a well-demarcated, superficial ulcer with polypoid mucosa involved almost the entire circumference of the rectum 5 cm from the anal dentate line. The ulcerative nodular lesion measured 5 cm × 4 cm in length and width (described in the pathological report at the local clinic).\nHistological examination revealed fibromuscular obliteration in the lamina propria combined with a superficial ulcer, which is surrounded by a large amount of serrated crypts (Figure ). These irregular glands showed a saw-toothed pattern or a diamond-shaped architecture involving the base of crypts (Figure ). The epithelial cells lined in the crypts were characterized by mucin hypersecretion and slight nuclear stratification (Figure ), which is consistent with the pathological characteristics of SSA/p. Around the SSA/p area, micro-vesicular hyperplastic polyps (MVHPs) were found, showing the earliest lesions of serrated dysplasia (Figure ).\nImmunostaining for HES1 showed loss of expression in the serrated glands, while nuclear staining was observed in the normal adjacent epithelium and interstitial inflammatory cells (Figure ). Similarly, there was superficial loss of MLH1 expression in the crypts with serrated architectures (Figure ). However, proteins encoded by other mismatch repair genes, such as MSH2, MSH6 and PMS2, were nuclear positive in all cells. The staining for β-catenin was diffusely positive along the cell membranes, and P53 showed focal nuclear positivity. Ki-67 staining increased in the crypts of SSA/p (Figure ), compared with the basal staining in glands of MVHPs (Figure ). |
This patient is a 14-year-old male, who felt a popping sensation and significant right knee pain while jumping and colliding with another player during a basketball game the previous day. Following the injury, he was evaluated in an outside emergency department, where anterior, posterior, and lateral radiographs obtained in the emergency department demonstrated a tibial fracture consisting of two primary components (). He was placed in a cast and sought a second opinion regarding findings and management.\nUpon presentation to the clinic the following day, he reported mild pain (3/10) and noted no normal function of his leg. A physical exam was performed but was limited due to pain. Following the review of radiographic imaging, an MRI was performed, which demonstrated a type IIIB tibial tubercle avulsion fracture and complete tear of the patellar tendon from its distal attachment site, as well as a hematoma at the fracture site (). After discussing the findings with the family, the patient was scheduled to undergo open reduction internal fixation of a type IIIB fracture and repair of the patellar tendon three days following the initial injury.\nAn 8-centimeter anterior incision was made at the superior aspect of the tibial tubercle and extended distally. At the patellar tendon insertion site on the tibia, the tendon was noted to be completely avulsed from the bone cortex distally, while proximally, the tendon remained attached to the displaced tubercle. The tendon remained attached to the inferior pole of the patella. The anterior tibial plateau fragment was anatomically reduced using two fully threaded noncannulated screws (Arthrex, Naples, FL), while the tibial tubercle fragment was reduced via bicortical fixation with a 50 mm fully threaded 3.5 mm cortical screw (Arthrex, Naples, FL).\nThe distal patellar tendon was completely avulsed through two-thirds of its length. To restore the native footprint of the patellar tendon, a 4.5 mm PEEK (polyetheretherketone) corkscrew anchor (Arthrex, Naples, FL) was placed slightly lateral to the anatomic insertion site to avoid a stress riser on the anterior tibial cortex. The anatomic repair of the patellar tendon was completed with two mattress sutures and tied.\nIn addition to the avulsion of the patellar tendon and periosteum, it was noted that fascial tissue with tibialis anterior muscle belly avulsed through the injury site causing subacute extensive bleeding within the anterolateral compartments (). This scenario raised concern for impending compartment syndrome, and an anterolateral compartment release was planned.\nThree 3-centimeter incisions were made along the anterolateral aspect of the leg. The first was located 3 centimeters distal to the neck of the fibula, the second was located 10 centimeters above the distal fibula tip, and the third was located at the midpoint between the two. Under endoscopic visualization, the intramuscular septum was identified and Metzenbaum scissors were used to cut through the fascial compartment beginning in the anterior compartment and extending proximally then distally to the midtibia (). The fascial incision was extended posteriorly into the peroneal compartment and then was extended proximally and distally to the midtibia. These steps were repeated for the midpoint and distal incision sites. Distally, the course of the superficial perineal nerve was identified and the nerve itself was protected during the distal release of the anterior compartment. It was believed that the impending compartment syndrome occurred due to damage to the surrounding bony and muscular tissue. A medium Hemovac drain was placed along the length of the lateral compartment, exiting in the posterolateral proximal leg. The patient was placed in a hinged knee brace which was locked in extension. He was discharged home later that day.\nOn postoperative day number two, the patient's Hemovac drain was removed by a family member. The patient was seen 1 week postoperatively and noted moderate pain (6/10) and 0% normal function. On physical examination, incisional sites were clean, dry, and intact and a small fracture blister was noted on the posterior aspect of the knee—which was cleaned and redressed. Radiographic imaging revealed well-positioned screws, no evidence of new fractures or foreign bodies, and early evidence of callus formation. Two and a half weeks after surgery, the patient presented to the clinic for evaluation. He reported that he had no pain (0/10) and had 5% of his normal function at this time. On physical examination, he noted no tenderness to palpation of the knee joint, and he had 40 degrees of knee flexion. Anterior-posterior and lateral X-rays were taken which showed evidence of callus formation in the bone (). At this time, it was recommended that the patient begin gentle active range of motion exercises with extension and light flexion. He was also encouraged to become full weight-bearing with the brace until its removal two months postoperatively.\nFive months postoperatively, the patient reported no pain (0/10) and possessed 95% of his normal function at this time. On physical examination, he was nontender to palpation along the joint line. There was no laxity with varus or valgus stress. He demonstrated 5/5 quadriceps strength with no evidence of an extensor lag. He had an active range of motion from 0 to 130 degrees of flexion, and there was no lag with straight leg raise. Repeat anterior-posterior and lateral X-rays demonstrated a well-reduced tibial tubercle fracture as well as well-positioned and nondisplaced hardware (). |
A 24-year-old Caucasian man presented with a several month history of muscle pain, fatigue and insidious onset of pitting edema to his lower extremities. His symptoms progressed to include bilateral arm swelling, muscle pain to the thenar eminence of both hands, and paresthesias to his hands. He did not have a rash, joint pain, Raynaud phenomenon, oral ulcers, fever, hardening of the skin or weight loss, and he denied any cardiac, respiratory, genitourinary, or gastrointestinal symptoms. Prior to symptom onset, he had traveled to South America and reported possible ingestion of undercooked meat as well as swimming in a river and lake. He had frequent visits to the Northeastern United States and had recently spent an extended period of time outdoors in Rhode Island. Medical, surgical, and family histories were unremarkable, and he was not taking any medications. He had a history of mild alcohol intake, no history of smoking, and some marijuana use. On examination, his vital signs revealed a blood pressure of 104/57 mm Hg, pulse of 56 bpm, and normal temperature. He had no significant findings on head, neck, cardiovascular, respiratory, or abdominal exam. He had no cervical, axillary, or inguinal lymphadenopathy. He had significant pitting edema on his feet and legs extending up to his knees as well as non-pitting edema on the dorsum of both hands. Although there was edema, the skin was soft without any significant hardening and was without any overlying erythema. There were no signs of skin dimpling or grooves. His neurological examination revealed normal strength.\nHis initial laboratory work was significant for a mild eosinophilia of 700 with a normal white blood cell count, hemoglobin, and platelets. He had normal calcium, creatinine, and electrolyte levels. His alanine aminotransferase was slightly elevated and total protein slightly low, but he had normal albumin and bilirubin. His thyroid-stimulating hormone was slightly increased, but his free T4 and total T3 were normal. His urinalysis was normal. He had a normal level of creatine phosphokinase, sedimentation rate, and C-reactive protein. Further workup revealed a positive anti-nuclear antibody with a titer of 1:160 with a speckled pattern. His extractable nuclear antigen panel was negative, including Scl-70. His ANCA, myeloperoxidase and proteinase-3 serum studies were also negative. His ACE level and complement levels were normal. Infectious workup revealed negative stool studies for culture, ova, and parasites. Antibodies for HIV, CMV, and Trichinella were negative. Serum for histoplasmosis, cryptosporidium, coccidiomycosis, and interferon gamma release assay were negative. A blood smear for parasites was negative. An ELISA IgG/IgM test for Lyme disease was positive with subsequent testing with Western blot strongly positive for IgG (eight out of ten bands positive) and also positive for IgM (two out of three bands positive).\nThe patient had extensive imaging done with a normal CT scan of his neck, chest, abdomen, and pelvis. An echocardiogram was also normal. Due to a previous negative workup along with persistent pain and swelling, an MRI of his right lower extremity was performed (see Figure ). Imaging demonstrated extensive circumferential edema with enhancement of the superficial soft tissues, superficial fascia, and, to a lesser extent, deep fascia of the lower leg. Taking into account the mild peripheral eosinophilia and the imaging findings, a diagnosis of eosinophilic fasciitis was considered. To complete the workup, a biopsy of the fascia, muscle, and adipose tissue of the left calf was taken (see Figure ). The biopsy did not include the dermis. Surprisingly, there was no evidence of eosinophilic fasciitis. Instead, the specimen illustrated a striking granulomatous fasciitis and vasculitis. The fascia showed exuberant granulomatous inflammation (Figure A) with an inflammatory infiltrate that was made up predominately of histiocytes and CD3-positive T cells with very rare eosinophils (Figure B). The granulomatous inflammation centered primarily on small- to medium-sized blood vessels and was non-necrotizing. While the vessels did not display overt fibrinoid necrosis, they did appear damaged with loss of endothelial cells confirmed with CD31 immunostaining. The inflammatory infiltrate was seen extending into adipose tissue and particularly around blood vessels within the fat. The adjacent skeletal muscle also showed perivascular inflammation and vasculitis in both the perimysial and endomysial compartments. There was no endomysial fibrosis, fatty infiltration, or inflammation surrounding muscle fibers. AFB (acid-fast bacteria) and Wade-Fite stain were negative for mycobacterial organisms. GMS (Grocott-Gomori's methenamine silver) stain was negative for fungal organisms. Due to the positive Western blot for Lyme, a Warthin-Starry silver nitrate stain was performed to evaluate for spirochetes; however, no definitive organisms were seen. A Borrelia PCR analysis of the tissue was performed as well, but no DNA was detected.\nThe patient was given a diagnosis of granulomatous fasciitis along with a diagnosis of Lyme disease. He was first treated with doxycycline for 42 days straight due to initial Lyme serology being positive. The calf biopsy revealing fasciitis was not performed until the patient was 3 weeks into the doxycycline course. Once fasciitis was diagnosed, he was started on a prednisone taper starting at 1 mg/kg/day for a week with taper by 10 mg every 2 weeks. After 2 months of treatment with prednisone, the patient had near resolution of symptoms. A repeat MRI was performed 82 days after the initial MRI with the previously seen changes consistent with fasciitis nearly completely resolved with only a thin sliver of edema over the superficial fascia. On re-evaluation of the patient, he reported skin changes to his left upper arm at 20 mg of prednisone per day and skin changes to his left forearm at 7.5 mg per day. Examination of the upper arm revealed an atrophic patch with some overlying erythema and examination of the forearm showed indurated, bound-down, tense skin with a slight groove and minimal overlying hyperpigmentation. The rest of the dermatological examination was within normal limits. Skin biopsy revealed marked septal thickening with sclerosis, sparse lymphoplasmacytic infiltrate along the dermal subcutaneous junction, and swollen, homogenized collagen fibers with diminished spaces between the fibers (see Figure ). The clinical examination along with pathology revealing dermal and subcutaneous sclerosis was consistent with a diagnosis morphea profunda. Lyme serology was repeated, but Western blot for IgG was negative. A scleroderma antibody panel was negative as well. He was started on methotrexate and a higher dose of prednisone. |
A 16-year-old Kosovar male, of Albanian ethnicity referred to our department with the chief complaint of painless swelling on the right cheek. The patient described painless swelling 3 months before visit as the initial observation, but has shown the enlargement in the last few weeks. Medical history data of the patient revealed no systemic disease or other health problems.\nFacial asymmetry was present on the right side on clinical extraoral examination.\nThe skin overlying the swelling was normal. The extraoral swelling was well circumscribed, painless and approximately 5 × 5 cm in size. The consistency was hard and without fluctuation.\nIntraoral examination revealed a painless swelling in the right maxillary vestibule extending from the maxillary right central incisor to the maxillary right first molar. The intraoral swelling was firm, non tender, covered with normal mucous membrane.\nEgg shell cracking was present buccally but not palatally (Fig. ). Aspiration revealed thick juicy yellow liquid and cholesterol crystals were visible. Panoramic radiograph revealed a unilocular radiolucent lesion extending from the maxillary right central incisor to the maxillary right first molar, in contact with the roots of the teeth present inferiorly, and to the maxillary sinus superiorly. Maxillary right canine tooth was displaced posteriorly most probably by the cystic pressure and the unerupted lateral incisor was present inside the radiolucency (Fig. ). The vitality of teeth 11, 14, 15, with roots in close relation to the lesion was positive.\nPreoperative diagnosis of the lesion was made as dentigerous cyst based on the age of the patient, location of the swelling, impacted right canine and unerupted lateral incisor located inside the lesion, aspirated thick juicy yellow liquid and visible cholesterol crystals, but the UA was also taken into consideration. The surgical operation including total enucleation of the cystic lesion together with impacted tooth was made (Figs. , ). After removing the lesion along with the impacted lateral incisor tooth and after measuring it, the lesion was approximately 4 cm in length (Fig. ). The wound was tamponated with gauge which was removed periodically for 3 days from the postoperative second day. The specimen was sent for pathological examination.\nThe pathological examination revealed UA, mural form. Infiltrating islands of atypical basaloid cells with peripheral palisading were present. Separation artifact of peritumoral stroma was evident (Fig. ).\nThe nature of the tumor was explained to the patient and we advised the patient to regard regular follow-up visits. There were no signs of recurrence since 2 years after the operation. |
The patient was a 22-year-old nulliparous Japanese woman. Her medical and family histories were noncontributory. She had no previous history of smoking, habitual alcohol drinking, or drug abuse. She was referred to our hospital for fetal anatomical evaluation because of severe oligohydramnios at 13 weeks and 6 days of gestation. The gestational age was calculated from the first day of her last menstrual period. At her 10 weeks and 6 days' visit to the previous clinic, the crown-rump length of the fetus was 34.7 mm and no remarkable abnormalities of amniotic fluid volume were recorded. However, at 13 weeks and 4 days of gestation, a remarkable decrease in the amniotic fluid volume was found. At the first visit to our hospital, we carried out an antenatal ultrasonographic scan and a single live fetus was noted. The crown-rump length, biparietal diameter, and nuchal translucency of the fetus were 66.1 mm, 23.6 mm, and 2.1 mm, respectively. Because of severe oligohydramnios (), ultrasonographic images were not very clear, and no clear 3D images were obtained. As far as we could check, no structural abnormalities were found in the fetal cranium, brain, upper extremities, thorax, and heart. However, the lower extremities remained in the fixed extension, but all long bones were visible (). The lower extremities stayed in the same position, and no individual movements of the two legs were seen, which suggested the fusion of the two lower extremities. Color Doppler ultrasound revealed a single umbilical artery (). Neither kidneys nor the urinary bladder was visualized. Therefore, the diagnosis of type I sirenomelia was made prenatally.\nThe couple was informed about the findings and the suspected poor prognosis of the fetus. They then opted to have a termination of pregnancy, which was performed at 15 weeks and 1 day of gestation. On the postnatal examination, the baby weighed 60 grams and the external examination revealed the fusion of both lower extremities (). The external genitalia and anal structures of the baby were ambiguous. Postmortem imaging studies with the radiography and CT scans revealed a single lower limb containing two femurs, two tibiae, and two fibulae, and none of them were fused (). Agenesis of the lower lumbar and sacral spines was also noted ().\nA 75 g oral glucose tolerance test (OGTT) was performed 2 days postpartum, which showed a mildly impaired glucose tolerance pattern of the mother (). At 17 weeks postpartum, another OGTT was carried out and the blood glucose levels were in the normal range (). |
A 28-year-old man was transferred from another institution with uncontrolled intermittent epistaxis. He had a history of road traffic accident 9 months earlier with mild traumatic brain injury associated with multiple skull base fractures, from which he was stabilized and recovered completely. Two weeks prior to arrival, he developed torrential nasal bleeds that stopped spontaneously the first time, but required nasal packing the next time it occurred a few days later. The bleeds were becoming more frequent and heavier, thus he was transferred for urgent intervention. His evaluation included a CT of the brain that showed multiple areas of encephalomalacia (). The sphenoid sinuses showed complete opacification with hyperdensity on both sides denoting hemosinus (). Multiple non-displaced skull fractures were seen with a small bony defect at the superior aspect body the sphenoid bone on the left side ().\nThe CT angiogram of the brain demonstrated the presence of a large arterial out-pouching arising from the cavernous part of the left internal carotid artery protruding through a defect at the left superior-lateral aspect of the sphenoid sinus representing pseudoaneurysm, with its tip projecting well into the right side of the sphenoid sinus measuring 16 x 8 mm (). He was shifted to the angiographic suite for embolization. The procedure was performed under general anesthesia and endotracheal intubation to protect the airway in case of rebleeding during the procedure.\nA left internal carotid artery cerebral angiogram was performed using the standard projections, as well as oblique projections, and showed an aneurysm arising from the paraophthalmic segment of the internal carotid artery that projects anteriorly and medially into the sphenoid sinus (). The aneurysm was multilobulated with multiple daughter sacs. It had a small neck to dome ratio. We decided to proceed with primary coiling. The aneurysm was cannulated using Echelon 14 microcatheter (eV3, Irvine, MN, USA) over 0.014” Silverspeed microguidewire (eV3, Irvine, MN, USA). Multiple detachable bare metal coils were deployed into the aneurysm to pack it tightly in sequential “baskets” distal first then proximal using 9 mm diameter coils for each to obviate the need for an adjunctive device. A completion angiogram showed near complete obliteration of the pseudoaneurysm with small residual flow into the neck with preservation of the origin, and flow into the ophthalmic artery (). He tolerated the procedure and was extubated upon completion. There was no immediate complications and he received the post coiling heparinization protocol for 24 hours as is the usual practice at our institution when the coil mass is close to the origin of an important vessel, in this case, the ophthalmic artery. He was discharged from the hospital few days later. He was then followed up clinically at the outpatient and seen in the outpatient clinic with no further episodes of nasal bleeding or new neurologic deficit. A one-year follow up showed a stable coil mass with no evidence of recanalization. |
A 58-year-old woman presented with a one-day history of malena and light-headedness. She did not have any known co morbidities. Apart from being anaemic she was haemodynamically stable and her general physical and abdominal examination unremarkable. Hematological examination revealed low hemoglobin levels of 7.3 g/dl with normal clotting profile. After initial resuscitation, an esophagogastroduodenoscopy (OGD) was performed which excluded any cause of upper gastrointestinal bleed. This was followed by colonoscopy, which although did confirm the presence of blood within the gastrointestinal tract but did not localize the site of bleeding. However, the patient's hemoglobin level continued to fall over the next two days, which was treated with a transfusion of six units of packed red cell.\nSubsequently, she underwent selective superior mesenteric angiography, which demonstrated a large hyper vascular mass with an extensive contrast blush, near the terminal ileum in the right iliac fossa (Figure ). It was supplied by a large feeding artery and drained via a solitary large vein into the portal venous system (Figure , ). No extravasation of contrast was seen suggesting that the lesion was not actively bleeding at the time of the study. Inferior mesenteric and coeliac axis angiography revealed no synchronous lesion or evidence of any vascular deposit in the liver.\nShe thereafter underwent laparotomy through midline incision, which identified vascular solid tumor along the anti-mesenteric border of the ileum about 30 cm from the ileo-caecal junction, measuring 7 × 7 cm (Figure ). A segmental small bowel resection was performed, and intestinal continuity established with an end-to-end small bowel anastomosis. The patient had an unremarkable postoperative recovery; her hematocrit remained stable and she was discharged from the hospital on the 7th postoperative day.\nThe histopathology of the resected tumor showed a submucosal tumor compressing the mucosal lining causing ulceration. Sectional analysis of the tumour showed thick bands of muscle with vascular spongy stroma. Microscopic examination (Figure ) showed thick bands of smooth muscle staining positive for smooth muscle antigens using immunochemistry. Immunochemistry was negative for gastrointestinal stromal tumors (C-Kit antigens) and sarcomas. Interspersed between these muscle fibers were a variety of vascular channels ranging in size from dilated capillaries to a small and medium sized arteries and veins (Figure ). The smooth muscle appeared to arise from the muscularis propria of the small bowel. There was no significant mitotic activity in the tumor and no evidence of tumor necrosis. The tumor was well circumscribed with clear excision margins. All these findings were consistent with the diagnosis of angioleiomyoma. |
A 59-year-old gentleman with end-stage kidney disease due to hepatitis C with focal segmental glomerulosclerosis, on hemodialysis for seven years, underwent deceased donor renal transplantation. The donor kidney had a KDPI of 60%. There was a 4 antigen HLA mismatch with Class II panel reactive assay (PRA) of 62.31%. Class I PRA was 0%. His donor specific antibody testing was positive for an antibody to DQ7. The donor had died as a result of anoxic brain injury following cardiopulmonary arrest resulting from anaphylactic shock. The cold ischemic time was 12 hours, 7 minutes and the warm ischemic time was 51 minutes. Basilixumab was used for immunosuppression induction and the patient underwent early steroid withdrawal. His maintenance immunosuppression was tacrolimus (targeting trough levels 5-8) and mycophenolate sodium 720 mg BID.\nThe patient's postoperative course was complicated by delayed graft function, necessitating continuation of renal replacement therapy. He was hemodynamically stable throughout his hospital course. A renal allograft ultrasound showed good blood flow to the entire kidney with a resistive index of 0.64. A repeat flow crossmatch was negative, but he remained positive for a persistent low-level donor specific antibody to DQ7 (1000 MFI). He underwent a renal transplant biopsy on his 4th day postoperatively. This showed extensive acute tubular necrosis with associated peritubular capillaritis and interstitial nephritis (). Oxalate crystals were seen in several tubules. One large caliber artery showed active endothelialitis, but no tubulitis or glomerulitis seen. C4d staining was negative in the peritubular capillaries. Electron microscopy revealed minimal effacement of podocyte foot processes. The patient received methylprednisolone 500 mg x 3 doses to treat moderate acute cellular rejection. His tacrolimus dose was also optimized as his trough levels had been running low at between 3.5 and 6. He continued to take mycophenolate sodium at a dose of 720 mg BID. A decision was made not to treat for antibody-mediated rejection given that no glomerulitis was seen and that there was minimal capillaritis with a negative C4d stain.\nHe remained dialysis dependent with minimal urine output for three weeks after transplantation. A urine protein : creatinine ratio was elevated to 1070 mg/g when assessed after hospital discharge when his urine output started to gradually improve. His urine microalbumin : creatinine ratio was 450 mg/g. A decision was ultimately made to readmit the patient on day 12 after transplantation for IV thymoglobulin 1.5 mg/kg to treat his Banff Type IIa T cell mediated rejection, given his lack of response to pulsed IV steroid treatment. Given lack of improvement in renal function and urine output by day 16 after transplantation, a second renal allograft biopsy was performed. This revealed residual acute tubular necrosis with associated mild peritubular capillaritis and interstitial nephritis (). Extensive oxalate crystals were now visible in many tubules. His plasma oxalate level was concurrently elevated to 19.3 μmol/L (reference range ≤ 1.9 μmol/L). No signs of persistent acute antibody- or cell-mediated rejection were seen. Again, there was minimal segmental effacement of the podocyte foot processes seen on electron microscopy and no evidence of recurrent focal segmental glomerulosclerosis present.\nHe commenced calcium citrate along with dietary oxalate restriction to manage his hyperoxaluria and his serum creatinine improved to a nadir of 1 mg/dl (88 μmol/L), with a concomitant gradual reduction in his proteinuria and microalbuminuria to undetectable levels. Of note, the patient had no history of malabsorptive intestinal disease and denied any GI symptoms throughout this time period. He had never suffered from renal calculi. At three weeks after transplantation, his urine output and creatinine clearance had recovered sufficiently to enable him to become dialysis independent. A decision was made to continue low-dose oral steroids in the long-term given the presence of early acute cellular rejection on his first allograft biopsy. An interval renal biopsy performed 2 months later showed no ongoing evidence of oxalate deposition, tubular necrosis or cellular rejection. A concurrent repeat plasma oxalate level showed interval reduction to the normal range. |
A 65-year-old man presented to the Outpatient Department of Elpis Hospital, for evaluation of a chronic bacterial prostatitis. He reported mild irritative voiding difficulties such as urgency, frequency, nocturia, dysuria, a mild low back pain and perineal discomfort. Although there was no urethral discharge, the patient complained of local burning sensation on urination and an itching sensation in the urethra. The symptoms lasted for fifteen days.\nA complete review of systems was unremarkable as well as his medical and family history. The patient's hemogram was normal without leucocytosis. The midstream urine showed microscopic hematuria, pyuria but no bacteriuria indentifying the organism infecting the urinary tract was present. The PSA values were elevated 7,6 ng/ml with a free PSA/total PSA ratio 39%. The transrectal ultrasonography revealed a cystic mass in the transitional zone of the prostate with a solid and a nodular inner portion.\nOn rectal examination, the prostate was normal in size and consistency. The prostatic secretions obtained by prostatic massage showed a number of inflammatory cells (more than 30 white cells per high-power field) but no causative infectious agent was found by culture or other means. Several eggs were observed amongst the inflammatory cells measured 51–59×26–29 μm with one convex side (Figure ). The size and shape of the eggs identified them as a group of Enterobius vermicularis. On examination of the prostatic secretions we found a soft palpable material which was 5 mm diameter in size and spherical shape. Palpation gave the impression of a tissue than a stone. An incision was performed in this material and a 4 mm long living worm was found. (Figure ). The microscopic examination identified the worm as Enterobius vermicularis.\nAlthough there was a speculation that the infection was sexually transmitted, the patient denied it. The patient did not experience any symptoms from the gastrointestinal tract or itching sensation in the anus. The cello-tape test was performed and a microscopic examination identified Enterobius vermicularis eggs. Although the patient had a negative cello-tape test and no irritative symptoms he was treated with mebendazole 100 mg PO bid for 3 days followed by two more courses with 3 week time interval. At six month following evaluation of the patient, there has been no recurrence of enteroparasitosis.\nAfter informing the patient, his three grandsons were examined using the cello-tape anal swab technique (one smear per child), for the presence of Enterobius vermicularis eggs. All three samples were positive for parasite eggs and the children were treated with the same antihelmintic regimen. |
Here, we report a 57-year-old Caucasian female, with an unremarkable past medical history, who was referred to our neurosurgical department after she presented with an incidentally found lesion on magnetic resonance imaging (MRI) of her lower spine. On physical examination the patient did complain about discomfort in her pelvic area when sitting, left greater than right. However, she did not have any related neurologic complains such as back pain or leg pain, nor did she display any weakness, numbness or tingling sensation or other signs of radiculopathy. The mass was incidentally discovered on lumbar MRI, which was obtained for evaluation of her atypical pelvic pain.\nRoutine MRI was obtained demonstrating a right-sided sharply demarcated, poorly and heterogeneously contrast enhancing paraspinal lesion at the junction of the lumbar and sacral spine. The mass was localized immediately adjacent to the posterior elements (lamina and spinous process) and was deeply seeded in the right multifidus muscle adjacent to the L5 inferior articular process. It measured approximately 3.0 × 3.0 × 3.5 cm in size on T1 postcontrast images \n[]. Based on radiographic criteria alone, the differential diagnosis included: Schwannoma, lipoma or liposarcoma, fibroma, myoma, sarcoma or metastatic disease among rare other entities. A full systemic work up was performed and included a contrast enhanced computed tomography (CT) of the chest, abdomen, and pelvis as well as a mammogram and were negative for any other focus of systemic disease. As the lesion was concerning for a neoplasm, the patient was counseled and advised to pursue a histopathological diagnosis to direct further treatment options. She opted for an open surgical approach aiming for an excisional biopsy and she was consented and taken electively to surgery.\nPreoperative repeat MRI was performed within a 4 week window after initial presentation and revealed an unchanged right-sided paraspinal lesion at the level of the lower posterior lumbosacral spine. The lesion measured again approximately 3.5 cm in diameter and remained heterogeneously contrast enhancing and appeared somewhat lobulated. It continued to display sharp demarcation to the surrounding soft tissue. It did not show signs of erosion or invasion of the osseous parts of the L5 posterior elements.\nSurgery was performed under general anesthesia. The patient was positioned prone on a Wilson frame and a standard midline incision was performed after application of local anesthesia. The subcutaneous layers were split and the thoracolumbar fascia was divided. Dissecting into the soft tissue, we immediately identified a well encapsulated lesion deep in the paraspinal muscles, but the mass was adherent to the periostium of L5 and S1. An unremarkable dissection was performed and the lesion was resected in toto with a small soft tissue margin. Intraoperative fresh frozen analysis revealed a myxoid spindle cell tumor without significant atypia. Since the mass was abutting the osseous posterior elements of L5 and S1, we carefully coagulated the surface of the entire bony area that had been contacted to diminish the chances for any local recurrence. The wound was closed in layers and the patient had an unremarkable postoperative course. She was discharged to home the second postoperative day.\nThe entire specimen with a small clean margin was received for pathological examination and measured ca. 5 cm. Cut surfaces revealed a thinly encapsulated, well-circumscribed lesion with a soft white, gelatinous appearing cut surface. The specimen was entirely submitted for histopathological examination. Routine hematoxylin and eosin stains were performed on formalin-fixed, paraffin-embedded sections and showed a relatively well-circumscribed tumor composed of prominent myxoid stroma with a distinct presence of stellate-shaped bland cells with pyknotic nuclei and virtual absence of blood vessels []. In approximately 20% of the tumor specimen, areas of increased cellularity and vascularity were noted []. However, no mitotic activity, necrosis, or increase in nuclear atypia was noted in these foci. The tumor cells stained negatively for S100, actin, desmin, EMA, and cytokeratin cocktail. Based on the histopathological and immunophenotypical profile, a diagnosis of cellular myxoma was made.\nAs recurrence is very uncommon for such tumors after complete excision, no other therapeutic intervention was recommended. Due to the rather rare diagnosis of cellular myxoma in this locale – with overall dearth of evidence-based clinical recommendations – a close and frequent follow-up surveillance imaging schedule was established for this patient in spite of this entity being described as a benign tumor. To date, 42 months after the surgery, the patient remains neurologically intact, feels well and no recurrence has been observed on sequential imaging [Figure ]. |
A 64-year-old male presented with a two month history of difficulty urinating and was found to have a fungating penile mass involving 50% of his penis. The mass was hard and fixed and extended from the glans proximally up the shaft. He also had bilateral palpable inguinal lymphadenopathy. There were no associated constitutional symptoms. Given there was a high suspicion for malignancy, the patient underwent partial penectomy within a month of presentation. Biopsy results confirmed a pT2 tumor with invasive keratinizing squamous cell carcinoma, poorly differentiated, and tumor size of 5 × 4 × 2.5 cm, with corpus spongiosum and lymphovascular involvements.\nFollowing the procedure, the patient had PET-CT for staging, and imaging revealed enlarged hypermetabolic bilateral axillary lymph nodes concerning for metastatic disease. In addition, there was a large centrally necrotic lymph node conglomerate in his left groin that had increased FDG avidity. The patient had left inguinal and bilateral pelvic lymph node dissections revealing metastatic squamous cell carcinoma in multiple lymph nodes. The left inguinal mass was also found to be metastatic well-differentiated SCC. His diagnosis was staged at T2N3M0.\nAfter his surgical procedures, patient was started on adjuvant chemotherapy. He began first line chemotherapy with paclitaxel, ifosfamide, and cisplatin (TIP). He underwent 4 cycles of TIP but eventually developed disease progression on repeat imaging. At this point, the patient was started on cetuximab given EGFR amplification on tumor analysis with the FoundationOne testing platform. However, the patient had an allergic reaction to cetuximab, so his treatment was changed to panitumumab. The patient had stable disease and a progression-free survival of 6 months with anti-EGFR treatment, which is clinically significant given that this treatment was given in the second-line setting for an aggressive tumor type that other than chemotherapy there is no other approved drug to date.\nThe patient was ultimately started on the PD-1 inhibitor nivolumab. He had initial response to immunotherapy followed by stable disease, so he had a disease control rate of an additional 6 months with this investigational agent at that time. Ultimately, he was placed on hospice and passed away two years from the the time of diagnosis. |
An 11-year-old girl presented with the gradual onset over one day of sensory dysesthesia of her left forearm followed the day of admission by severe monoparesis of the ipsilateral forearm, increasing dysesthesia, and dysphagia. She recalled that the day the symptoms developed, she swam faster and for a longer duration than usual. She was referred to our hospital one week from the onset because her symptoms had not resolved. Her past history was significant for hydrocephalus from aqueductal stenosis that was diagnosed and treated when she was aged 1 year. Her ventriculomegaly and symptoms at that time resolved completely following endoscopic third ventriculoscopy. CM-I was diagnosed by radiographic criteria. During the subsequent 10 years, she received annual MRI and neurological examinations that documented persistent tonsillar ectopia but no neurological deficits.\nOn admission, her physical examination revealed spastic paresis of left upper limb, with the limb assuming a peculiar posture at rest, with the wrist in extension and the elbow joint in flexion []. The other elements of neurological examination, mental status, cranial nerves, motor, sensory, cerebellar, and deep tendon reflexes were normal except for decreased gag reflex. Besides, she had neither headache, neck pain nor motor deficits of lower extremities, and denied experiencing any Valsalva-induced symptoms. There was no known history of head trauma.\nMRI a week from the onset showed cerebellar tonsillar herniation of 8 mm below McRae's (basion-opisthion) line, which was the same amount as on previous imaging. No recurrence of hydrocephalus was demonstrated [Figure –]. There was no evidence of syringomyelia or abnormal T2 signal in the spinal cord [Figure and ]. Sagittal fast imaging employing steady state acquisition (FIESTA) MRI clearly showed that compared with the previous in detail, the tonsil changed into beaked shape and was asymmetry []. Clinical signs and symptoms pointed to dysfunction of the cervicomedullary junction despite stable imaging findings. Surgical treatment consisting of FMD, C1 laminectomy and duraplasty was performed without complication.\nAfter the operation, dysesthesia and monoparesis of left forearm and dysphagia improved steadily. She was discharged on the 14th postoperative day with complete neurological recovery. At the follow-up 6 months after FMD, she maintained her recovery. Postoperative MRI demonstrated an ascent of the cerebellar tonsil of 4 mm compared with before FMD []. |
A 13-year-old male patient with T-cell acute lymphocytic leukemia underwent a sibling matched allogeneic HCT in February of 2010. As for conditioning regimen, he received total body irradiation, cytarabine, and cyclophosphamide. Neutrophil engraftment was achieved on posttransplant day 10. His pretransplant varicella zoster virus immunoglobulin (Ig) G was positive and he was on acyclovir prophylaxis and cyclosporine was given for GVHD prophylaxis.\nOn posttransplant day 12, he developed erythematous papular rash on face and trunk with pruritus. His white blood cell count was 16,670/μL (neutrophil 75%, lymphocyte 6%, eosinophil 0%). Grade 1 skin graft versus host disease (GVHD) was suspected. Until posttransplant day 21, skin lesions persisted and partial crusted lesions were observed (). He received multiple antihistamine agents and topical steroid but complained of severe itching sensation.\nOn posttransplant day 28, skin biopsy was performed after a consultation with dermatologists. Skin punch biopsy showed perivascular chronic inflammatory cell infiltration and cytoid body of basal layer (). Conservative treatment for pruritus and management for GVHD was recommended. Systemic corticosteroid, gabapentin, and lorazepam were added.\nOn posttransplant day 42, a consultation was requested to the pediatric infectious diseases physicians for the evaluation of persistent intense itching sensation and any possible skin infection. When the infectious diseases team visited the patient and questioned, his mother and grandmother reported that they also developed papular rash with itching sensation. An urgent request for an additional evaluation was requested to a dermatologist. A contagious skin infection was suspected and direct smear from his finger web revealed numerous mites (). He was diagnosed with classic scabies and immediately discharged from the hospital because he was otherwise stable except the skin lesions. Crotamiton ointment was applied for treatment at home. Currently, crotamiton is not recommended by U.S. Food and Drug Administration with safety concerns.\nAfter a week, pruritus subsided and skin lesions continued to improve during the 6 weeks of outpatient clinic follow-up visits.\nAn investigation was performed in all potentially exposed individuals in the hospital. Infection control intervention and environmental disinfection were also performed to prevent the institutional outbreak. There were no other scabies cases from this nosocomial exposure. |
A 15-year-old female patient (bodyweight 50 kg, height 160 cm) presented with syncope. Severe arterial hypertension was diagnosed (178/147 mmHg left arm, 102/83 mmHg left leg). She reported previous episodes of exercise independent headaches and nose bleeds. Auscultation revealed no cardiac murmurs, but an accentuated second heart sound and reduced pulses in the lower limbs. There were no clinical signs to suggest Alagille or William’s syndrome or clinical evidence of neurofibromatosis. Blood tests showed normal inflammatory markers and normal creatinine. The urine dip showed no markers for blood or protein. No regular medication was taken at the time she presented. The electrocardiogram showed normal sinus rhythm, no significant signs of left ventricular (LV) hypertrophy or abnormal repolarization. Echocardiography detected a severe concentric LV hypertrophy (14 mm diastolic septal diameter, 15 mm diastolic diameter of the LV posterior wall) and a long hypoplastic segment in the abdominal aorta with massive arterial collateralization. Cardiac catheterization confirmed the diagnosis of a midaortic syndrome with a minimum diameter of <1.5 mm and a gradient of 50 mmHg between the aortic arch and the femoral arteries (Figure ). The narrow part began directly under the diaphragm above the truncus coeliacus and continued down to the bifurcation of the arteriae iliacae including the renal arteries, with reversed flow in the caudal aorta up to the renal arteries. Because of the extraordinary length of the hypoplastic segment of 23 cm, we decided for a stepwise interventional therapy with a paclitaxel covered balloon. No additional computed tomography scan or magnetic resonance imaging (MRI) was performed. Consultation with our surgeon, supported the interventional approach. The proposed procedure was reviewed and approved by our institutional review board, extensively discussed with the patient’s family, and informed consent was obtained before proceeding.\nAfter balloon interrogation of the long stenotic abdominal aorta using a 6 × 20 mm Tyshak balloon (NuMED Inc., Hopkinton, NY, USA) to differentiate between rigid stenosis and hypoplastic parts, serial balloon dilation with a 7 × 40 mm paclitaxel covered In.Pact balloon (Medtronic, Minneapolis, MN, USA) (3 µg/mm2 Paclitaxel on the balloon) was performed (Figure ) from end to end of the stenosis. Planned redilations, to stepwise improve the aortic compliance and reduce the risk of dissection, were performed after 2, 4, and 8 months with Elutax balloons ranging from 8 mm to 12 mm diameter (Aachen Resonance, Düsseldorf, Germany) (2 µg/mm At the end of the last interventional procedure, a mild endothelial lesion at the former narrowest point of the aorta was noticed (Figure ). This lesion healed and after 2.7 years MRI showed a stable and adequate result (Figure ). Today the girl is in unrestricted physical condition. The blood pressure has markedly improved (139/64 mmHg right arm and 127/65 mmHg right leg) under therapy with atenolol and amlodipine.\nThe patient and patient’s family consented to the publication of this case’s history and the images presented. |
A 30-year-old female with a past medical history significant for protein C deficiency and a history of pulmonary embolism comes with an urticarial rash associated with arthralgia. She did not have any history of fever or joint pain at that time. The episode started recently and was associated with high fever and significant joint pain and stiffness. She was seen in urgent care and treated with prednisone 20 mg. Her rash remained stable but she still had swelling and tenderness of bilateral knee and ankle joints and bilateral wrist and elbow joints.\nShe was seen for the same complaints in the emergency department. An X-ray of the bilateral knee was unremarkable; she was given morphine for pain and was sent for follow-up in the clinic. Meanwhile, suspecting a broad differential of autoimmune etiology, workup was done, including complete blood count, basic metabolic profile, liver function test, urine analysis, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), ANA, complements, rheumatoid factor, hepatitis panel, human acquired immunodeficiency virus (HIV) rapid antigen test, and blood cultures. She was admitted to the hospital for increasing pain and fever of 101 F. Her white blood cell (WBC) count at that time was 30.1, with a neutrophilic predominance and significant bandemia; the liver function test was abnormal. Bone marrow biopsy was not performed because of no lab or physical findings of any hematological malignancy. Suspecting infectious etiology she was started on broad-spectrum antibiotics, vancomycin, and cefepime. Her blood cultures and urine cultures were negative. Suspecting infectious endocarditis, echo was done, which was again negative for vegetations, with preserved ejection fraction and normal valves and wall motion. With the given amount of tachycardia, she was also having an elevation of D-dimer. Computed tomography (CT) chest with contrast was done, pulmonary embolism (PE) was ruled out, and the lungs were unremarkable for any pathology. She still had persisting spiking fevers every day and with each spike of fever, she had a worsening rash and joint pains. The rash was papulosquamous and present over extremities more than axial as shown in Figure and Figure .\nInfectious disease was involved, and investigations were done to rule out infectious as well as autoimmune causes. Chlamydia, gonorrhea, HIV, Hep B, Hep C, Epstein-Barr Virus (EBV), cytomegalovirus (CMV), Lyme’s serology, Borrelia Burgdorferi, and COVID-19 were negative. ESR and CRP were elevated with values of 98 and 298, respectively. Lactic acid was normal. ANA titers were 1:80 speckled pattern and the C2, C3, and C4 complements and C1Q levels were normal. Thyroid-stimulating hormone (TSH) was normal. The X-ray of the knee joints was unremarkable. She had a troponin elevation of 0.12, for which cardiac cath. was done, which showed clean coronaries. With all the infectious and autoimmune diseases ruled out, ferritin and transferrin were done, suspecting Still’s disease since she had temperature spikes, associated rashes, and mild troponin and ANA elevation, Ferritin was significantly elevated at 32,661 and transferrin was 115. With the clinical findings, four major criteria, and one minor Yamaguchi criteria along with the ferritin value, she was diagnosed to have adult-onset Still's disease and started on high-dose steroids when her rashes and joint pains decreased. She was given methylprednisolone 125 mg for three days and sent home with prednisone 60 mg. On follow-up after a week, she reported decreased symptoms. A plan was made to continue steroids for six to eight weeks and start on DMARDs after tapering the steroids. |
The patient was a 46-month-old Saudi boy. He was delivered via cesarean section in view of his large head detected through prenatal ultrasound at term. He weighed 4.63 kg, measured 55 cm in length, and had a head circumference of 41 cm. He was discharged with his mother without admission to the neonatal intensive care unit (NICU). The mother had no antenatal or postnatal problems, and she had no prior medical illness. The head circumference was very large at birth, above the 98th centile. The patient was being reviewed regularly by ultrasound due to the large head size, which was normal initially in the neonatal period. There was no consanguinity and no family history of a similar condition.\nAt the age of six months, magnetic resonance imaging (MRI) showed intracranial hypotension with engorgement of the venous sinus and mild flattening of the pons. Tonsillar descent, third ventricle dilation, and white matter changes were also noted (Figure ). Two months later, the patient underwent a whole spine MRI, which was incomplete, as the patient was fully awake during the study. The study redemonstrates the significant descent of the cerebellar tonsils through the foreman magnum to the left of C2. Also, the visualized part of the dural venous sinus at the posterior fossa was significantly distended (Figure ). The abdominal ultrasound (US), which was done at the age of eight months to rule out organomegaly, was normal (Table ). Parents of the patient kept following up with the neurology, orthopedic, metabolic, and genetics teams in King Abdulaziz Medical City in Jeddah.\nAt the age of 11 months, the patient was sent for computed tomography (CT) to assess the need for a ventriculoperitoneal (VP) shunt. However, the CT showed decreased hydrocephalus, so there was no need for a VP shunt (Figure ). The patient was also seen by the ophthalmologist who confirmed that the optic disc was normal, with no pupillary edema.\nAt the age of 28 months, the patient underwent orchidopexy to bring down the left undescended testis from the intracanalicular level to the mid-scrotal level. He was discharged with no complications, and he was on a course of oral acetaminophen (24 mg/ml) (250 mg) (qid) for 10 days. A follow-up abdominal US was performed to reassess organomegaly, which revealed an increase in liver and spleen span with no evidence of a focal lesion (Table ). Full metabolic workup and other laboratory tests were done and were normal (Table ).\nAt the age of 41 months, MRI showed bifrontal and perisylvian polymicrogyria with areas of prominent Virchow Robin spaces and unchanged cerebellar tonsil ectopia with crowding of the posterior fossa. Bilateral peri-regional high T2 signal intensity was noted (Figures -). Later, he was diagnosed by whole-exome sequencing (WES) as having changes in the PIK3CA gene, which results in PROS, namely, MCAP and mosaic confirmation. He had speech, language, and developmental delay and macrocephaly. The central nervous system (CNS) examination showed mild joint hyperlaxity, mild hypotonia in the upper and lower limbs, normal power, normal reflexes, and normal cranial nerves examination. Moreover, the patient was not found to have a headache, photophobia, vision problems, loss of consciousness, abnormal movements, seizure, or signs of increased intracranial pressure. The cardiac examination showed normal first and second heart sounds with no murmurs. On examination, there was no skin hyper/hypopigmentation nor were there any masses of growth. His head circumference had increased in size: 60 cm. He also had multiple dental caries and underwent full dental clearance with no complications. There was no evidence of regurgitation, choking, or feeding problems. The patient was started on the overgrowth protocol for PIK3CA, which includes alpha-fetoprotein and an abdominal US every three months until the age of eight years. The patient has followed up with the genetic, neurology, developmental, and general pediatrics clinics.\nAt the age of 43 months, another abdominal US was done to check for organomegaly, and it was unremarkable (Table ). He was diagnosed with iron deficiency anemia and was prescribed ferrous sulfate 6 mg/kg/day. He was vitally stable, active, and playful. Regarding the milestones, he could not climb the stairs or eat by himself. He was able to walk with help. He preferred to watch things on his iPad and was on diapers. The mother noticed that when he got happy and excited, his hands fluttered. He had poor eye-to-eye contact with his mother or teacher at school. On examination, he was looking well; not in any pain or distress. He had positive subtle dysmorphic features in the form of low -set ears, macrocephaly with frontal bossing, depressed nasal bridge, and bilateral esotropia. His head circumference was 60 cm (above the 99th percentile). He had an unsteady gait but normal tone and power.\nAt the age of 46 months, the patient was reviewed in the developmental clinic. He started to say "ma" and "ba" only with unspecified one-syllable sounds. He could walk alone without support but could not run. He could go up and down the stairs without support. The patient could drink using glasses and cups but could not feed himself using spoons. He could not wave “bye-bye.” He liked repetitive actions, e.g., opening and closing doors multiple times and turning toy cars around multiple times. His mother was concerned that he lacked an awareness of danger. The mother said that he became more disciplined after attending one semester at a special center for speech therapy, physiotherapy, and occupational therapy. He is still on the overgrowth protocol for PIK3CA. |
A 50-year-old female patient was admitted due to cough that had persisted for several weeks. Her past medical and surgical history was unremarkable. Laboratory data, chest X-ray, and chest CT did not reveal any significant findings. Abdominal US incidentally identified a 42 × 38 × 35-mm multilocular cystic lesion surrounded by the liver, stomach, left kidney, and pancreas (Fig. ). Multiphasic contrast-enhanced CT imaging revealed that the cystic lesion was unilocular, without separation or mural nodules, and it was located in the lesser curvature of the stomach (Fig. ). A thin cyst wall had no contrast enhancement. A multilobulated cyst with a thin internal septum spread outward from the pancreatic tail on MR images, but did not appear to communicate with the main pancreatic duct (Fig. ). Endoscopic US showed a multilocular cystic lesion along the pancreatic tail with a thin multiple septum and mural nodules (Fig. ). These findings could not confirm that the cystic lesion was derived from the pancreas, and a mucinous cystic neoplasm was a possibility considering her age and sex. Therefore, she provided written, informed consent to undergo laparoscopic resection. Laparoscopy confirmed the multilocular cyst in the lesser curvature of the stomach (Fig. ). The gastrocolic ligament was divided, and the body and tail of the pancreas was exposed in the omental bursa. Further observation revealed that the cystic lesion was derived from the lesser omentum. Although the cyst was located directly beside the left gastric artery, enucleation proceeded without sacrificing the artery. Macroscopically, the 4.8 × 4.5-cm cyst had multiple septae (Fig. ). Microscopically, the inner surface of the cyst was lined by a single layer of flattened cells without atypia and focal smooth muscle (Fig. ). The lesion was diagnosed as cystic lymphangioma. The patient was discharged on postoperative day 4 without complications. The patient was free of recurrence after 1 year of follow-up.\nInformed written consent was obtained from the patient for publication of this case report and accompanying images. |
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 62-year-old male patient presented with the history of diabetes mellitus and hypertension along with abdominal pain and a palpable pulsatile epigastric mass. The patient had undergone bilateral total hip replacements, which subsequently got infected. He was initially treated with levofloxacin, teicoplanin, and vancomycin. The implants were removed and joint spacers were implanted. Blood and tissue cultures demonstrated methicillin-resistant Staphylococcus aureus (MRSA) and proteus mirabilis. Although all prosthetic materials were removed, the patient continued to develop fever for several weeks. Computed tomography (CT) demonstrated a septic spleen without other pathologies. A splenectomy was performed.\nTwo months later, the patient showed clinical signs of recurrent sepsis with a white blood count of 12,000/mm3. CT angiography (CTA) revealed a 60-mm paravisceral aortic pseudoaneurysm at the origin of the celiac artery (CA) (Fig. .\nThe patient was transferred to our tertiary care vascular surgical center. Written consent was obtained from the patient. Placement of a covered stent into the CA was attempted to prevent aneurysm rupture but this was technically unsuccessful.\nThrough a midline laparotomy, the paravisceral aorta was exposed through the lesser sac and the infrarenal aorta was exposed distal to the pancreas (Fig. . The crura of the diaphragm were divided to facilitate aortic clamping. The aorta was clamped proximal to the CA and below the renal arteries. Then, the pseudoaneurysm was opened and all infected tissues and necrotic aortic wall were removed. The reconstruction of the CA was not feasible due to the necrotic tissues and the infected environment. The patient had a well-developed gastroduodenal arcade to the common hepatic artery from the superior mesenteric artery (SMA) and the splenic artery was ligated earlier during splenectomy; therefore, we ligated the distal CA. The pseudoaneurysm was excised to healthy-appearing aortic tissue. The aorta proximal to the SMA was repaired with a 4 × 2 cm cryopreserved human aortic allograft patch, after thorough debridement (Fig. . The aortic cross clamp time was 34 min.\nPostoperatively, the patient stayed in the intensive care unit for 2 days. Blood cultures repeatedly confirmed MRSA infection, but tissue culture from the aortic wall was free from bacteria. He was dismissed from the hospital to a rehabilitation facility with meropenem on postoperative day 7. At 1-year follow-up, the patient remained free of infection without antibiotics; control CTA showed patent SMA and renal arteries without pseudoaneurysm or any symptom of infection. |
A 49-year-old woman presented with a 3-month history of repeated ptosis of both eyelids and oral ulcers and erosions. Physical examinations revealed scattered ulcers and erosions in the mouth (Fig. ). Laboratory examinations showed that CA 125 was elevated (51.6 U/ml), while other tumor markers, including CA199, 153, CEA, and AFP, were normal. Autoimmune antibodies, including anti-CENP-B antibody, ANCA, anti-AchR antibody, and ANA, were all positive.\nThe patient was first diagnosed with an oral aphthous ulcer and ocular myopathy myasthenia gravis. She was treated with gentamycin and dexamethasone spray inhalation to improve her oral lesions and pyridostigmine to cure muscle weakness. However, the oral ulcers improved slightly and the myasthenia gravis persisted. An abdominal ultrasound showed a hypoechoic mass in the left adrenal gland. A further CT examination showed a 6 × 5 cm, well-defined round solid mass with central necrosis in the pancreatic tail. There was no calcification detected in the mass. The solid part of the mass had slight enhancement in the arterial phase with many serpentine feeding arteries, moderate enhancement with a draining vein around the tumor in the portal venous phase and persistent enhancement in the delayed phase (Fig. a-e). The fundus of the stomach was compressed by the mass. The boundary between the mass and the splenic artery and vein was not clear, and swollen lymph nodes were not observed in the posterior peritoneum. The mass was initially considered to be a neuroendocrine tumor in the pancreatic tail.\nAt the same time, the patient’s symptoms worsened. She could not swallow, and she felt severe pain in her mouth. She also developed a cough and expectoration. A chest CT revealed infection in the lower lobes of both lungs. Streptococcus was detected from a throat swab. Levofloxacin was administered to fight the infection, methylprednisolone to fight the inflammation, and thalidomide to alleviate the vascular inflammatory reaction in addition to pyridostigmine and immunomodulatory therapy. However, 3 days later, the patient progressed to severe dyspnea, wheezing and difficulty with expectoration. Emergency intubation and mechanical ventilation were administered. Aspergillus was detected after bronchoalveolar lavage. Immunoglobin and voriconazole were given. Four days later, the symptoms resolved and the intubation was detached. Most of the infections in the lungs were resolved according to a chest CT. The pain in the mouth was also alleviated.\nAfter a multidisciplinary discussion, the patient’s tentative diagnosis was paraneoplastic pemphigus and the myasthenia symptoms caused by the pancreatic tumor. Myasthenia gravis, in turn, led to the patient’s inability to excrete sputum. If the pancreatic tumor could not be removed, the symptoms would not completely remit, and the symptoms due to myasthenia gravis would also continue to aggravate, finally leading to the occlusion of the respiratory tract. Therefore, the patient was transferred to general surgery. During the surgery, a 4 × 5 cm dark-red tumor with medium texture and clear boundaries was detected in the pancreatic tail (Fig. ). The tumor was close to the splenic artery and vein, and the spleen was normal. Postoperative pathology confirmed the tumor was a follicular dendritic cell sarcoma with immunohistochemistry showing CD21(+), CD23(+), CD138(+), SMA(+), Des(+), CD117(−), DOG-1(−), S-100(−), CD34(−), CK(−), EBER and EBV(−)(Fig. a-c).\nThe patient continued to be treated with antifungal and anti-infection therapy. Twelve days after surgery, the patient developed sudden heart palpitation, discomfort and difficulty in breathing. Mechanical ventilation was again administered. However, the patient died of inability to excrete sputum and occlusion of the respiratory tract. |
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 59-year-old gentleman with end-stage kidney disease due to hepatitis C with focal segmental glomerulosclerosis, on hemodialysis for seven years, underwent deceased donor renal transplantation. The donor kidney had a KDPI of 60%. There was a 4 antigen HLA mismatch with Class II panel reactive assay (PRA) of 62.31%. Class I PRA was 0%. His donor specific antibody testing was positive for an antibody to DQ7. The donor had died as a result of anoxic brain injury following cardiopulmonary arrest resulting from anaphylactic shock. The cold ischemic time was 12 hours, 7 minutes and the warm ischemic time was 51 minutes. Basilixumab was used for immunosuppression induction and the patient underwent early steroid withdrawal. His maintenance immunosuppression was tacrolimus (targeting trough levels 5-8) and mycophenolate sodium 720 mg BID.\nThe patient's postoperative course was complicated by delayed graft function, necessitating continuation of renal replacement therapy. He was hemodynamically stable throughout his hospital course. A renal allograft ultrasound showed good blood flow to the entire kidney with a resistive index of 0.64. A repeat flow crossmatch was negative, but he remained positive for a persistent low-level donor specific antibody to DQ7 (1000 MFI). He underwent a renal transplant biopsy on his 4th day postoperatively. This showed extensive acute tubular necrosis with associated peritubular capillaritis and interstitial nephritis (). Oxalate crystals were seen in several tubules. One large caliber artery showed active endothelialitis, but no tubulitis or glomerulitis seen. C4d staining was negative in the peritubular capillaries. Electron microscopy revealed minimal effacement of podocyte foot processes. The patient received methylprednisolone 500 mg x 3 doses to treat moderate acute cellular rejection. His tacrolimus dose was also optimized as his trough levels had been running low at between 3.5 and 6. He continued to take mycophenolate sodium at a dose of 720 mg BID. A decision was made not to treat for antibody-mediated rejection given that no glomerulitis was seen and that there was minimal capillaritis with a negative C4d stain.\nHe remained dialysis dependent with minimal urine output for three weeks after transplantation. A urine protein : creatinine ratio was elevated to 1070 mg/g when assessed after hospital discharge when his urine output started to gradually improve. His urine microalbumin : creatinine ratio was 450 mg/g. A decision was ultimately made to readmit the patient on day 12 after transplantation for IV thymoglobulin 1.5 mg/kg to treat his Banff Type IIa T cell mediated rejection, given his lack of response to pulsed IV steroid treatment. Given lack of improvement in renal function and urine output by day 16 after transplantation, a second renal allograft biopsy was performed. This revealed residual acute tubular necrosis with associated mild peritubular capillaritis and interstitial nephritis (). Extensive oxalate crystals were now visible in many tubules. His plasma oxalate level was concurrently elevated to 19.3 μmol/L (reference range ≤ 1.9 μmol/L). No signs of persistent acute antibody- or cell-mediated rejection were seen. Again, there was minimal segmental effacement of the podocyte foot processes seen on electron microscopy and no evidence of recurrent focal segmental glomerulosclerosis present.\nHe commenced calcium citrate along with dietary oxalate restriction to manage his hyperoxaluria and his serum creatinine improved to a nadir of 1 mg/dl (88 μmol/L), with a concomitant gradual reduction in his proteinuria and microalbuminuria to undetectable levels. Of note, the patient had no history of malabsorptive intestinal disease and denied any GI symptoms throughout this time period. He had never suffered from renal calculi. At three weeks after transplantation, his urine output and creatinine clearance had recovered sufficiently to enable him to become dialysis independent. A decision was made to continue low-dose oral steroids in the long-term given the presence of early acute cellular rejection on his first allograft biopsy. An interval renal biopsy performed 2 months later showed no ongoing evidence of oxalate deposition, tubular necrosis or cellular rejection. A concurrent repeat plasma oxalate level showed interval reduction to the normal range. |
The patient is a 41 year old middle-eastern female who underwent abdominoplasty, liposuction (arms and back), and buttock augmentation by fat grafting in the private setting. Her inpatient stay was uneventful and was discharged home 2 days postoperatively. She returned for follow-up on day 4 postoperatively, with concerns regarding a red, hot, and tender swelling in the left gluteal region. She was then admitted to the hospital for 2 days where intravenous ceftriaxone was started. She underwent surgical debridement of necrotic subcutaneous tissue the next day. She continued to attend her outpatient clinic appointments for a few days but was readmitted with similar complaints. Intravenous ceftriaxone was administered and she underwent further debridement of necrotic tissues. Postoperatively, intramuscular chymotrypsin and daily dressings were commenced. Clinically, her condition continued to deteriorate and she became febrile with peripheral temperature readings of 38 °C and started to develop gangrenous wounds. On day 6 postadmission, the patient was then transferred to the operating theater where further necrotic tissue was debrided. On day 17 postcosmetic surgery, she was transferred to the plastic surgery department with a specialist public hospital.\nThe patient was previously healthy with unremarkable past medical, surgical, and family histories. Her concomitant medications were related to her initial surgery and included analgesics, antibiotics, and omeprazole. She has no known drug allergies. She denies alcohol intake, smoking, and recreational drug use. She only traveled to Austria, Germany, and Netherlands 3 months prior to undergoing cosmetic surgery. She denies other risk factors such as being exposed to any environmental sources of infection such reconstruction/demolition sites, animal/insect bites, or any form of penetrating injury.\nOn admission, the patient's vital signs and systemic examination were unremarkable. The gluteal regions were severely affected, there was a 20 cm by 15 cm wound at the left buttock with extensive necrotic skin and subcutaneous tissue. The right buttock was indurated and swollen and other operative sites were unremarkable. Laboratory investigations showed hemoglobin levels of 8.1 (g/dL), white cell count of 46. 69 (109/L), and procalcitonin levels of 1. 46 (ng/mL), other tests were unremarkable (Fig. ).\nThe patient's management involved regular analgesics, empirical intravenous Meropenem, and surgical debridement of her wounds. Despite this, she continued to be febrile with temperatures of >39 °C and her inflammatory markers (including procalcitonin levels) continued to rise. Upon dressing the following day, new areas of necrosis have developed. She continued to undergo regular extensive surgical debridement, almost twice weekly, to the extent that portions of the sacrum and head of femur became exposed. Vacuum-assisted closure therapy (V.A.C ATS®/GranuFoam Silver®, KCI San Antonio, TX) was applied and adjusted to 100 mm Hg continuous mode following debridement. Regular tissue samples were sent to histopathological and microbiological analysis. At 2 weeks postadmission, preliminary microbiological results showed filamentous fungus, prompting the initiation of intravenous voriconazole empirically at 450 mg (6 mg/kg) twice daily for 24 hours followed by 300 mg (4 mg/kg) every 12 hours thereafter. This was continued for 2 weeks and was changed accordingly upon receiving the full mycology report stating the presence of A elegans. This was also confirmed by histopathology samples. Liposomal amphotericin B (AmB) was then started accordingly at a dose of 375 mg (5 mg/kg) per day for a further 3 weeks (Fig. ).\nAfter commencing the appropriate antifungal therapy and several debridement procedures, the patient's general condition, vital signs, investigations, and wounds have gradually improved. She underwent a total of 10 surgical interventions. Her final wounds had healthy granulation tissue with no signs of infection and were deemed suitable for surgical reconstruction (Fig. ). |
A 55-year-old Caucasian male, with a past medical history significant for tobacco abuse (41 pack-years), presented with shortness of breath accompanied by chest and back pain for two months. Blood workup showed a WBC count of 68,400 cells/µL, with an AEC of 27,360 cells/µL. A computed tomography (CT) pulmonary angiogram was performed, as he was hypoxic, and revealed a 3.6-cm speculated mass within the anterior right upper lobe, partially invading the anterior chest wall. It also revealed mediastinal and hilar adenopathy, an extensive osseous lesion (including compression fracture at T7), and a small pericardial effusion (Figure ). A CT of the abdomen and pelvis with contrast was performed and revealed a diffuse metastatic disease involving the liver, adrenal glands, spleen, and the bones. Magnetic resonance imaging (MRI) of the thoracic spine did not reveal spinal cord compression, but it did show the compression fracture at T7 and multilevel thoracic spondylosis. An MRI of the brain revealed a 5-mm lesion in the left occipital lobe, without edema or mass effect.\nThe hematology-oncology team was consulted for an evaluation of the metastatic disease and the eosinophilia. A core needle biopsy was obtained from a liver lesion and the result came back as poorly differentiated adenocarcinoma of the lung (cytokeratin 7, TTF1, and napsin-A were positive, while cytokeratin 2 and CDX2 were negative). Given his functional status, the decision was made to hold on systemic therapy and start on palliative radiation to the spine for pain control. The plan was to complete radiation sessions and then evaluate his functional status before starting systemic therapy.\nHe continued to have a high WBC count during the admission (Figure ). Therefore, a bone marrow biopsy was performed to rule out a hematologic malignancy and it revealed metastatic adenocarcinoma of the lung with no evidence of a myeloproliferative disorder. The flow cytometry from the bone marrow showed a CD5-positive clonal B-cell population, which was similar to the blood flow cytometry. Blood tests, including tests for Janus kinase 2 (JAK-2), calreticulin (CALR), MPL, BCR-ABL, and platelet-derived growth factor receptor (PDGFRA), were negative. The blood smear showed microcytic anemia with leukocytosis with absolute neutrophilia and eosinophilia. The serum immunoglobin E (IgE) was high at 377 IU/ml, and the tryptase level was low at 1.8 µg/L. Given these findings, his eosinophilia was related to a paraneoplastic process rather than a primary bone marrow disease.\nDuring the following days, the patient completed 13 sessions of radiation without improvement in his functional status, pain, or breathing. The case was discussed with the patient and his family; he decided that he would go with comfort measures, so he was discharged to the hospice facility. |
A sixty-four-year-old woman was referred to the department of cardiovascular surgery for the management of ischemic cerebral stroke suspected of cardiac etiology. During the preceding four months, she experienced one episode of transient ischemic cerebral attack. Physical examination was consistent with a monoparesis of left upper extremity and left facial paralysis. No more sign occurred. Cerebral CT scan revealed embolic lesion in the right sylvian artery area. Medical history showed a high blood pressure and hypercholesterolemia. The patient had no prior history of arrhythmia. Electrocardiogram (EKG) and Holter EKG were in sinusal rhythm. Investigations in search of a potential cause of cerebral embolism included a Doppler ultrasound of the carotid arteries which was normal. Transthoracic echocardiography (TTE) () showed an 8 × 8 mm mobile mass attached to the left atrium wall. Transesophageal echocardiography (TEE) () showed a second mass attached to free wall of left atrium (4.3 mm).\nThe tumors were excised under normothermic cardiopulmonary bypass using ascending aorta and bicaval cannulation. Anterograde blood cardioplegia was used. The left atrium was exposed by extended vertical transatrial septal incision (Guiraudon). Two tumors were found: a 10 mm friable translucide mass was attached between the two pulmonary veins and a 6 mm tumor located in left atrium between right inferior pulmonary vein and the base of anterior mitral leaflet. The left atrial appendage was closed. Mitral leaflet and subvalvular apparatus were inspected and were free of tumors. After bypass arrest a TEE confirmed a left atrium cavity with no tumor. Histological examination showed a papillary fibroelastoma including few fibroblasts, collagenous tissue, and elastic fibers surrounded by mucopolysaccharide acid. The patient had uncomplicated perioperative course and was discharged from hospital on postoperative day 8. At the 6-month followup, the patient was well. She has not experienced any neurological event. Transthoracic and transesophageal echocardiography did not find any recurrent tumour at 6-month followup. |
A 52-year-old female patient reported to our outpatient department with a chief complaint of pain in the back teeth region of the right lower jaw for the past 6 months. The patient underwent extraction of the lower right back tooth 2 years ago and developed pain in that region for the past 6 months. The pain was dull, intermittent, radiating in nature and relieved with medication. Intraoral examination revealed clinically missing 46 and the overlying mucosa appeared normal []. Orthopantamogram revealed a well-defined radiopaque mass measuring about 1.5 cm × 2 cm in the right mandible extending from the distal root of 45 to the mesial root of 47 []. On the basis of clinical and radiographic findings, a provisional diagnosis of complex odontoma was given.\nSurgical excision of the lesion was done and 11 small fragments of hard-tissue specimen were submitted for histopathological evaluation []. The specimens were subjected to decalcification and ground section procedures.\nGround section was done by manual grinding method in two steps: first with rough carborundum stone till a section of 2–3 mm was obtained and then on static carborundum stone with hand till a thickness of 1 mm was obtained. Grinding was further done using fine carborundum stone till a section thickness of 0.25 mm was achieved. The ground section was then cleaned, dried and mounted on the slide using dibutylphthalate polystyrene xylene (DPX) and viewed under a microscope.[]\nDecalcification was done using 10% aqueous solution of formic acid, as it is gentler in action unlike nitric acid or hydrochloric acid and less likely to interfere with nuclear staining. After decalcification, extensive washing in tap water was done to remove residual acids from the specimen followed by the standard tissue processing and staining procedure.[]\nOn microscopic examination, the ground section revealed large areas of tissue resembling bone with osteons and central Haversian canals [].\nThe hematoxylin and eosin-stained decalcified sections revealed delicate fibrocellular connective tissue stroma with collagen fibers, spindle-shaped fibroblasts and numerous vascular spaces. Within the connective tissue stroma were seen numerous basophilic masses of material resembling bone with osteocytes and globules resembling cementum [Figures –].\nBased on the histopathological findings and correlating with clinical and radiographic features, a final diagnosis of “Focal cemento-osseous dysplasia” was made. |
A 76-year-old male patient presented with a history of progressive dysphagia for solids for a year, which evolved to dysphagia and odynophagia for liquids in 5 months. The patient was referred from a basic health unit to the General Surgery Service of “Santa Casa de Ribeirao Preto” with esophageal obstruction and suspicion of neoplasia. The patient had a slim figure, as he lost 20 kg in 5 months. His current weight was 39 kg, his height was 1.63 m and his BMI was 14.67 kg/m2. The patient confirmed smoking habits (two packs of cigarettes a day during 50 years) and claimed he was a social drinker; the patient also denied any comorbidity. He then went through an upper gastrointestinal endoscopy (UGE), and the endoscope only entered 22 cm below the upper dental arcade, which discarded the possibility of a biopsy, which was needed for the hypothesis of neoplasia. Thoracic and abdomen CT scan showed a thickening of the gastric walls, especially at the esophagogastric junction. The CT scan also revealed an abrupt straightening of the proximal third of the esophagus (), suggesting esophageal neoplasia. As he was unable to receive oral diet, we opted to provide his diet through a Witzel’s jejunostomy in order to improve his nutritional gains. The neoplasia hypothesis indicated the need to perform a biopsy to analyze the tissue, and if positive for neoplasia, refer the patient to the oncology service for neoadjuvant therapy. The patient, then, underwent through another UGE, but this time, the endoscope passed through the obstruction and identified the presence of a Zenker’s diverticulum. Next, he underwent through an esophagogram that identified a large esophageal diverticulum (, ). Thus, the prognosis changed, and it indicated an endoscopic surgical repair of the Zenker’s diverticulum, as it is the standard procedure. Due to his low BMI and his nutritional conditions, it was opted to delay surgery. |
A 44-year-old Japanese woman with a 6-year history of insulin-dependent diabetes mellitus and an 11-year history of central diabetes insipidus presented with a pain in the genital area worsening over 2 weeks, general fatigue, and loss of appetite. Two months earlier, patient underwent a urinary catheter insertion as a management for urinary frequency, but it was removed during the previous hospital stay, a month before her recent hospitalization, for possible urinary infection. She has had frequent hospital admissions (6 times/year) and was hospitalized 3 months before her recent admission because of edema of the pelvic area and lower limbs. The patient developed diabetes mellitus after undergoing total pancreatectomy for nesidioblastosis, a surgical procedure which involved the removal of patient’s pancreas including the spleen and gallbladder. Her sister was also diagnosed with idiopathic central diabetes insipidus; thus, a family etiology was suspected. The patient had a surgery for suspected tongue cancer 2 years ago and was also suspected of non-alcoholic steatohepatitis with episodes of hepatic encephalopathy. Although she was on multiple medications including subcutaneous insulin injections and desmopressin tablets, her glycemic and hydration status were poorly controlled.\nFour days prior to the present admission, she visited a gynecologist for her inguinal pain. No uterine tenderness or exudate was observed, and she was prescribed gentamicin and lidocaine ointments for possible local infection. She developed edema in the pelvic area with loss of appetite, and her home doctor consulted the university department 1 day before the present admission.\nUpon admission, the patient appeared weak but was alert and had low-grade fever (37.4 °C) under a regular use of acetaminophen (1500 mg/day) and diclofenac (75 mg/day). Her blood pressure was not significantly different from previous measurements (88/42 mmHg) but a sinus tachycardia (heart rate 125/min) was noted. She complained of continuous abdominal pain and tenderness in all four quadrants. No abdominal guarding or rigidity was observed, but she had severe edema in the pelvic and bilateral femoral areas without necrotic skin discoloration. Laboratory investigations revealed a white cell count of 16,310/μL with neutrophilia (90.8%), elevated C-reactive protein of 22.18 mg/dL, and no serum sodium or potassium abnormalities. Serum aspartate aminotransferase and alanine aminotransferase were elevated at 466 U/L and 148 U/L, respectively. The patient’s international normalized ratio was high (2.26), but disseminated intravascular coagulation score did not meet the criteria. The patient’s HbA1c level was 8.8%, and blood sugar at admission was 316 mg/dL. She had low serum albumin concentration (1.7 g/dL), elevated serum ammonia concentration (154 μg/dL), and elevated lactate level (10.3 mmol/L). No ketonuria was noted, but significant pyuria was observed.\nThe abdominal ultrasound was unrevealing; thus, an intravenous treatment with ceftriaxone (1 g every 8 h) was initiated empirically after obtaining the blood and urine culture samples. A CT scan performed the following morning revealed the presence of air in the soft tissue of the inguinal and pelvic areas, such as pectineal and psoas major muscles (Fig. ). Immediate infectious and surgical consultations were made, and the antibiotics were changed to meropenem (1 g every 8 h), vancomycin (1 g every 12 h), and clindamycin (600 mg every 8 h). Gram-positive cocci and gram-positive rods were found in the initial blood cultures. In the evening of hospital day 2, a surgical debridement of the extraperitoneal pelvic tissue with colonostomy was performed, and the CT image after the operation suggested a complete resection of the affected tissue. However, hypernatremia (a serum Na concentration of 160 to 170 mEq/L) ensued as the use of nasal desmopressin could not effectively control the patient’s central diabetes insipidus after the operation.\nNo bacteria could be cultured from the debridement tissues (Table ). The blood culture isolates were finally identified as Streptococcus constellatus using superoxide dismutase A sequencing and C. ramosum by 16S ribosomal DNA sequencing []. The minimum inhibitory concentrations (MICs) of various antibiotics were tested using Etest for C. ramosum [] and the broth microdilution method [] except imipenem and meropenem (Etest) for S. constellatus (Table ). These results were interpreted using the Clinical & Laboratory Standards Institute M11-A8 document [] for C. ramosum and M100-S24 document for S. constellatus. Both strains were susceptible to penicillin, meropenem, and clindamycin. Despite the continued use of susceptible antibiotics and intensive care, disseminated intravascular coagulation and pancytopenia developed, and the patient died on hospital day 8. |
A 17-year-old boy with history of fall from height of approximately 15 meters presented to our institution after receiving primary care at another hospital. At presentation, he was conscious, hemodynamically stable maintaining oxygen saturation at 98% on room air with no visible signs of respiratory distress. His Glasgow coma scale (GCS) was 15, and was able to move all four limbs. The patient had sustained open fracture both bone left leg along with fracture right ankle.\nChest radiograph showed no intrathoracic injury with normal lung parenchyma. Computed Tomography showed burst fracture of fifth lumbar vertebra with canal compromise () and ruled out any injury to head, cervical spine, thorax and abdomen. After primary care, patient was admitted to the orthopedic ward for spine stabilization surgery and surgery for lower limb fracture.\nOn day four post-admission, an emergency consultation call was sent to our intensive care unit (ICU) in view of patient’s deteriorating status. When seen, he was grossly pale and febrile at 101 F, pulse rate of 140 per minute, systolic blood pressure of 80 mmHg and respiratory rate of 32 per minute maintaining oxygen saturation around 90% on oxygen face-mask. Patient’s GCS was 15. Chest auscultation revealed bilateral diffuse coarse crepitation and he was immediately transferred to the ICU. Initial arterial blood gas (ABG) showed partial pressure oxygen (pO2) of 49mm Hg on oxygen by face mask. Patient was intubated, sedated, paralyzed and put on mechanical ventilation with initial settings of volume assist control and high positive end expiratory pressure (PEEP). Central venous catheter was secured in right internal jugular vein under ultrasound guidance. An arterial line was secured in right radial artery for invasive blood pressure and arterial blood gas analysis. Chest radiograph showed bilateral fluffy opacities (). Preliminary blood investigations were mostly unremarkable except for hemoglobin of 6.8 mg/dl and raised ESR of 44. Fundus exam specific for FES was normal and there was no petechial rash on general examination. Urine for fat globules was positive. Over the next few hours, patient’s hypoxemia worsened requiring higher fraction inhaled oxygen (FiO2) of up to 0.8 and PEEP of 16 cm water. A decision was made to turn the patient into prone position after discussion with the orthopedic surgery team in view of unstable lumbar spine fracture. Patient’s family were informed about the specific risks and benefits of prone positioning, particularly in a patient with pre-existing unstable lumbar spine fracture and a written consent for the same was obtained. Positioning was done with the help of 5 trained ICU staff, using logrolling technique for turning the patient lateral followed by a 6th member placing the spinal board under the patient. After securing the patient on the spinal board, he was then shifted to one edge of the bed while the head gel support, chest and pelvic roll were placed in position. Patient was then shifted to prone position and spinal board was removed. Patient’s hands were abducted and placed next to the head and all the pressure points were padded using pillows and cotton rolls.\nOver the next 16 hours, we kept our patient sedated and paralyzed in prone position during which he received targeted fluid therapy and remained hemodynamically stable. Wake up test and pupil examination were done at regular intervals. Serial ABG’s showed dramatic improvement with Fio2 requirement decreasing to 0.4 and PEEP of 8 following which patient was repositioned supine. Two units packed red cells were transfused while the patient was prone. Chest radiograph showed resolving lung infiltrates and we decided to electively mechanically ventilate our patient for next 24 hours in supine position. Weaning was started next day and patient was extubated a day after and transferred back to ward 24 hours later.\nThe patient later on went on to have multiple surgical procedures for injuries to spine and bilateral lower limbs. He was then followed for 8 weeks in the out-patient department following discharge during which he recovered well without any neurovascular deficit. |
On May 20, 2019, a 69-year-old woman, gravida 3, para 3, underwent robotic radical cystectomy, total abdominal hysterectomy, bilateral salpingo-oophorectomy and bilateral pelvic lymph nodes dissection for bladder urothelial carcinoma. The vaginal cuff was closed with continuous stitches of absorbable sutures in a two-layer fashion. The postoperative course was uneventful, and the patient was discharged 11 days after surgery. The pelvic MRI on Sep 1, 2019 showed normal wound healing including the vaginal stump ().\nFive months after the surgery, the patient returned due to bilateral hydronephrosis, suspecting that tumor recurrence was involved. She also complained about a protruding vaginal mass noted after the latest sexual intercourse two weeks ago. The pain gradually increased and the mass was painful upon touching. Speculum examination revealed a 4x3x3cm tubular soft tissue lesion protruding from the anterior aspect of the vaginal apex (). Considering the patient's history, recurrence of bladder cancer was a possible cause. According to the patient, she had the first postoperative sexual intercourse four months after the surgery without any discomfort. Vaginal tumor excision was arranged 1 week after the patient's return visit, and the vaginal mass shrank to 3 × 2 × 2cm. During the operation, the vaginal tumor was easily removed by a Kelly grasper without the need of cold knife conization or electrocauterization from the vaginal approach. After the soft tissue mass, more likely of prolapsed epiploica (), was removed, the vaginal stump ruptured spontaneously. Examination of the vagina showed normal bowel movement and no active bleeding. The dehisced vaginal cuff was closed by interrupted sutures on the first layer and continuous sutures on the second layer with 1-0 Vicryl (polyglactin 910). The total blood loss of the surgery is minimal and no complication is noted. The patient recovered quickly and was able to leave the hospital 24 hours after the operation. The final pathology revealed adipose tissue with inflammatory response which is compatible with the diagnosis of epiploica. On the 30-day post-operative follow up clinic, the vaginal stump is well healed and the patient denied any discomfort in her daily life or during intercourse. |
The authors present the case report of a 69-year-old male patient with several cardiovascular risk factors (hypertension, hyperlipidaemia, and previous smoking) and history of coronary artery bypass grafting (CABG) 6 years before when the left internal mammary artery (LIMA) was grafted to the left anterior descending artery (LAD), saphenous vein conduit was grafted to posterior descendent artery and left radial artery grafted to the intermediary branch. No medical imaging of the aortic arch and its branches was performed before cardiac surgery.\nHe had recurrence of angina following the surgery (CCS class II) mainly when exerting the upper limbs. His therapy was adjusted and remained only mildly symptomatic until 2011 when he was referred to for coronary angiography because of gradually worsening exertional angina with no response to medical therapy. There were no neurological or claudication complaints. Physical examination only showed an II/VI systolic murmur. Basal EKG and blood analysis were unremarkable. On the transthoracic echocardiogram, there was only mild aortic sclerosis and good global and segmental systolic left ventricle function.\nHe had a positive exercise electrocardiographic stress test, showing a 1.5 mm ST segment depression on anterior and inferior leads accompanied by chest pain on 2nd stage Bruce treadmill protocol.\nThe coronary angiography showed no new lesions in native-vessel circulation or the bypass grafts, except for the occlusion of the saphenous venous conduit grafted to the posterior descendent artery and partial retrograde filling through the LIMA graft from the LAD ().\nSubclavian angiography showed a proximal severe stenosis (83%: quantitative coronary angiography) with slow distal flow; there were no significant stenosis in the carotid arteries ().\nIt was performed an ad hoc, left main percutaneous angioplasty, using a drug-eluting stent 4,0/12 mm (Promus Element) without trifurcation involvement, with final angiographic success (). In a second procedure it was performed a proximal left subclavian artery angioplasty with a balloon-expanded stent (Invatec Scuba 9.0 × 30 mm—10 atm) (). The procedure was uneventful. After one year of clinical followup the patient remained asymptomatic. |
A 57-year-old male patient (weight: 62 kg) reported with a chief complaint of bleeding gums from the upper back tooth region for 1 month. On examination, a generalized probing depth of 6–7 mm with the moderate horizontal bone loss was observed. The oral hygiene of the patient was fair with a moderate amount of supragingival and subgingival deposits. The patient was systemically healthy with no history of any cutaneous skin lesions, drug intake, or allergic reaction to any medication or irritant. A nonsurgical phase one therapy comprising of full mouth oral prophylaxis and root surface debridement was initiated. Ibuprofen 200 mg was prescribed post-treatment in case any pain or discomfort was experienced. The patient was recalled after 2 weeks for re-evaluation and follow-up. However, the patient reported back within 1 h, with the development of a sudden, localized, soft, edematous, non-fluctuant, and nonmovable lobular swelling on the frontal and temporal part of his forehead. The swelling was tender on palpation with slight erythema on its outer surface []. The patient also reported the development of a mild rise in body temperature, lassitude, and malaise.\nBased on the patient's history and the timing of onset of allergic response, ibuprofen-induced hypersensitivity reaction was suspected. The patient was questioned regarding the onset of any similar incidents in the past. The patient then reported the onset of a similar swelling a few years back (etiology unknown). The patient was immediately referred to a physician, and blood was withdrawn to check for the total and differential blood count delete was done. The blood reports showed increases in the eosinophils (absolute eosinophils count = 9.67 × 103/μL (H); differential cell count = 16.5%) suggestive of an allergic reaction []. A local prick skin test with ibuprofen was also performed to confirm the hypersensitivity reaction []. To exclude the cross-reactivity and tolerance to other chemically unrelated NSAIDS, an oral challenge with other COX 1 inhibitors (aspirin) was done. The patient was immediately administered 10–20 mg intravenous injection of pheniramine maleate to control the reaction. A drastic reduction in the temporal swelling was observed within 24 h. The patient was kept under observation and was prescribed 10 mg of pheniramine maleate twice a day for 3 days. The patient was instructed not be take any nonselective NSAIDs in the future. The complete resolution of the swelling and urticaria was observed within 72 h.\nSince the allergic response to ibuprofen was immediately observed in the absence of any previous history of cutaneous lesion, bronchial asthma, respiratory disorders along with cross-reactivity to two or more NSAIDs, a diagnosis of NSAID-induced urticaria/angioedema (NIUA) was established based on the European Academy of Allergy and Clinical Immunology)/WAO nomenclature [].[] |
The patient was first referred to our Sleep Research Centre in 2005, when he was 71.5 years old, because he had presented with a 12-year history of repeated nocturnal episodes of violent and automatic complex motor behaviors clearly reflecting dream enactment with frequent dream recall (war scenes or aggression). During such episodes, the patient often screamed, could fall from the bed, and could provoke lesions to the bed partner. The episodes were reported to be initially rare but had subsequently become more and more frequent with a recurrence of 3-4 times per night at the moment of our evaluation.\nIn addition, the patient reported infrequent episodes of weakness of the lower limbs elicited by emotions and perception of intense sounds when falling asleep, together with excessive daytime somnolence and tendency to fall asleep easily during the day and some isolated short episodes of uncontrollable but refreshing sleep, often accompanied by dream mentation. Finally, the patient also reported a mild deficit of the episodic memory.\nNeurological examination was normal and no extrapyramidal signs were detected, in particular; also psychological clinical examination was normal with a score of 29 on the minimental state examination (MMSE) [] and a score of 22 on the Epworth sleepiness scale []. Nocturnal laboratory polysomnography disclosed a sleep latency of 5.5 min, REM latency of 26.5 min, sleep efficiency of 85.4%, and a normal representation of the different sleep stages; periodic leg movement during sleep (PLMS) index was 47.5/hour, with a relatively low periodicity index (0.34) []; leg movements were more frequent but less periodic in REM sleep than in NREM sleep and did not show a clearly decreasing trend through the night. The sleep respiratory pattern was normal (apnea/hypopnea index 3.8/hour). Finally, during REM sleep an excessive amount of tonic and phasic chin EMG activations was evident with a moderate decrease of the REM sleep atonia index (0.82) [, ]. During the subsequent MSLT, the patient fell asleep in all of the 5 sessions with a mean sleep latency of 5 min and 36 s; no sleep-onset REM episodes were recorded.\nCerebral magnetic resonance imaging (MRI) showed a mild cortical atrophy and a small calcification of the falx. Genetic evaluation found the patient to carry the human leukocyte antigen (HLA) DQB1*0602 genotype. Thyroid hormones were within the normal limits and, among the numerous laboratory blood tests performed, a mild abnormality was found only for creatine phosphokinase (251 U/L, normal range 24–204). The patient refused the lumbar puncture procedure for the assessment of the level of hypocretin-1 in the cerebrospinal fluid.\nAfter a careful consideration of the therapeutic possibilities with the patient and his spouse, an agreement was reached and clonazepam was started at a dosage of 0.5 mg at bedtime which was followed by an excellent and sustained beneficial effect on the nocturnal dream enactment episodes which became very rare; sleepiness was reported to be fluctuating, with short but repeated periods of worsening, interspaced by periods of lower levels of daytime somnolence. At the age of 73 years, systemic blood hypertension was diagnosed in another clinical service and an adequate and effective therapy was started and maintained chronically (telmisartan 40 mg/day). Subsequently, at the age of 76 years, the patient was referred to us again for the subjective worsening of the memory function. The psychological clinical examination disclosed the presence of a very mild short-term memory, but the MMSE score was 30. At neurological examination, a very mild hypomimic face was observed together with an olfactory deficit. The score at the Epworth sleepiness scale was 12. Electrocardiography showed sinus bradycardia and a repeat cerebral MRI study substantially confirmed the previous findings.\nA new video-polysomnographic (v-PSG) recording was obtained which disclosed a sleep latency of 18.5 min, REM latency 64 min, and reduced sleep efficiency (71.5%) with a percentage of wakefulness after sleep onset of 25.2% (calculated over sleep period time); PLMS index was 2.8/hour and the sleep respiratory pattern was normal (apnea/hypopnea index 1.8/hour). Finally, during REM sleep the excessive amount of tonic and phasic chin EMG activations persisted (REM sleep atonia index 0.87).\nA new careful consideration of the therapeutic possibilities was carried out and discussed with the patient and his spouse and clonazepam was continued at the same dosage of 0.5 mg at bedtime with a subsequent continuation of the excellent clinical effect on the nocturnal dream enactment episodes.\nThe patient came back to our Sleep Research Centre at the age of 78 years because of the persistence of excessive daytime somnolence and tendency to fall asleep also in inappropriate situations. At neurological examination, hypomimic face and movement slowing were noticed again and the patient reported anosmia. MMSE score was 30. A routine electroencephalogram showed a mild slowing of the resting state alpha rhythm (7.5–8 cycles/s) and sine bradycardia was again found at electrocardiography. A third cerebral MRI again showed only a mild cortical atrophy and a small calcification of the falx (). Nocturnal laboratory v-PSG was again performed which showed a sleep latency of 7 min, REM sleep latency of 8 min, sleep efficiency of 88.5%, and a normal representation of the different sleep stages; PLMS index was 1.8/hour and the sleep respiratory pattern was normal (apnea/hypopnea index 3/hour). During REM sleep an excessive amount of tonic and phasic chin EMG activations was still evident (REM sleep atonia index 0.86). The v-PSG was followed by a MSLT; the patient fell asleep in all of the 5 sessions with a mean sleep latency of 7 min and 12 s; no sleep-onset REM episodes were recorded. The patient gave his consent for the lumbar puncture procedure and hypocretin-1 was found to be undetectable. Dopamine transporter imaging with ¹²³I-2β-carbomethoxy-3β-(4-iodophenyl)-N-(3-fluoropropyl)-nortropane (¹²³FP-CIT) SPECT was also carried out which showed reduced striatal binding with a reduction in both the putamen and caudate nuclei to occipital cortex uptake ratios ().\nFinally, over the last four months the patient has started presenting swollen, warm, painful and stiff hand, and wrist joints; these symptoms are particularly evident in the morning on waking or following prolonged inactivity. Morning stiffness is reported to last for long time. For this problem, the patient has consulted a rheumatologist who has reached the diagnosis of rheumatoid arthritis with the additional information gathered by laboratory testing and X-rays (data not available) and a pharmacological treatment was started with methotrexate, calcium folinate, methylprednisolone, indometacin, lansoprazole, and sucralfate. |
An 86-year-old man was admitted to our hospital with a 3-day history of acute abdominal pain. The patient had no previous medical history. A physical examination revealed marked right upper quadrant pain with normal bowel sounds. Murphy’s sign was positive. His vital signs were within the normal range. Abdominal ultrasonography revealed an enlarged gallbladder with surrounding fatty tissue inflammation. The blood biochemistry was essentially normal, including C-reactive protein (1.9 mg/dL) and total bilirubin (1.4 mg/dL) levels. An enhanced computed tomography examination revealed an enlarged gallbladder and incarcerated gallstone. Gallbladder wall enhancement was discontinued, and the fundus of the gallbladder was located centrally beyond the round ligament (Fig. ). The round ligament was attached to the right umbilical portion, which was associated with the anomaly of the intrahepatic portal vein system (Fig. ). Magnetic resonance cholangiopancreatography demonstrated the root of the cystic duct, while the middle portion of the cystic duct was unclear (Fig. ).\nWith the preoperative diagnosis of grade II acute gangrenous cholecystitis according to the 2018 Tokyo guidelines or gallbladder torsion, LC was planned. The first port was inserted into the umbilicus, and an enlarged and reddish gallbladder was observed. The gallbladder was swollen; however, torsion was not detected intraoperatively. The second port was placed in the epigastric area, while others were at the right hypochondriac and right lumbar regions. The gallbladder was attached to the left side of the hepatic round ligament (Fig. ). The cystic duct and the cystic artery were located in the normal positions. Severe inflammation and the narrow working space between the epigastric port and the gallbladder made it difficult to dissect Calot’s triangle; however, the cystic duct and the cystic artery were resected after the critical view of safety was confirmed. Due to the severe inflammation, a subtotal cholecystectomy was finally performed. The operative time was 178 min, and intraoperative blood loss was 50 mL. The pathological diagnosis was acute cholecystitis with a mucosal ulcer. The patient was discharged on the fifth day after surgery without postoperative complications. |
An 80 year old female came to the emergency department of our hospital complaining of abdominal pain of recent onset. She related a history of frequent episodes of non-bloody diarrhea of approximately one month's duration, unresponsive to loperamide. Her past medical history included surgical correction of rectal prolapse six months previously, implantation of a pacemaker, anticoagulation, osteoporosis, and hypothyroidism treated with levothyroxine.\nHer temperature was normal. Physical examination revealed lower abdominal tenderness but was otherwise unremarkable. The white blood cell count was 8.1 ×109/L (normal: 4.5-10.8 ×109/L). The patient was admitted to hospital.\nDue to chronic renal failure unenhanced abdominal CT scanning was performed (Fig. ). This showed a 15 cm length of colon at the splenic flexure with significant parietal thickening and fat stranding, as well as pneumatosis and free air in the surrounding mesocolon. Ischemic colitis was diagnosed based on location in a watershed area and signs of necrosis and perforation.\nThe following day the white blood cell count was 20.4 ×109/L. In light of this and the CT scan findings, an exploratory laparotomy was performed which revealed a small amount of ascites. A 16 cm segment of thickened bowel from the splenic flexure was resected.\nThe postoperative course was uneventful. The patient was discharged on the eighth post-operative day. However, the next day she was readmitted to hospital with abdominal pain accompanied by at least ten episodes of non-bloody diarrhea occurring that day. An abdominal CT scan with rectal contrast showed no evidence of anastomotic leak or other abnormality. Stool culture and testing for Clostridium difficile toxin were negative. The patient improved with conservative management. Given the diagnosis of collagenous colitis (see pathologic findings below), treatment with oral budesonide was started and she was discharged on the eleventh day of her hospital stay. One month later she reported feeling well and having one to two stools of normal consistency per day.\nMacroscopic examination (Fig. ) showed normal appearing mucosa but a markedly edematous and thickened wall measuring up to 1.5 cm.\nMicroscopic examination (Fig. ) showed the characteristic features of severe collagenous colitis throughout the segment of colon with areas of ulceration and abscess formation. The latter were transmural in at least two sites, compatible with perforation. Of note, subepithelial histiocytes, often multinucleated, were a prominent feature in the collagen band, but true multinucleated giant cells characteristic of the giant cell form of collagenous colitis [] were not found. There was no evidence of acute ischemic colitis or inflammatory bowel disease.\nReview of the prior rectal prolapse resection specimen confirmed the microscopic findings characteristic of prolapse. Histologic features of collagenous colitis were absent. |
A 16-year-old male patient reported with a chief complaint of swelling on the right side of the face for the past 2 months []. The swelling was initially noticed as a small lump which gradually increased to the present size. Medical and family histories were noncontributory. Examination revealed swelling in the right side of the face about 1.5 × 2 cm in size in the region of angle of mandible. There was no draining sinus and the skin over the swelling was clinically normal.\nIntraorally the swelling was present in the buccal aspect of right mandibular second molar measuring about 1.5 cm × 1 cm in dimension. Mucosa over the swelling was clinically normal. Buccal cortical bone expansion was seen along with obliteration of buccal sulcus. On palpation the swelling was firm, nontender and extended superiorly to involve the anterior border of ramus of the mandible.\nCT scan revealed an osteolytic lesion about 2.5 cm × 2 cm in dimension, extending from the distal aspect of 47 to the ramus of the mandible. Destruction of buccal cortical plate was evident. The clinical and radiologic findings were suggestive of odontogenic tumor [Figures –].\nSubsequently, an incisional biopsy was performed under local anesthesia and the specimen was sent for histopathologic examination. Microscopic evaluation of the sections revealed interlacing fascicles of spindle-shaped cells arranged in a biphasic pattern set in a collagenous stroma. Spindle cells were seen with oval, round and tapering nuclei with pale eosinophilic cytoplasm. These cells were seen alternating with closely packed cells with small rounded nuclei and eosinophilic cytoplasm []. Vascular spaces mimicking the hemangiopericytoma pattern were also observed []. Cellular atypia was not noted. Based on these findings we arrived at a diagnosis of myofibroma.\nImmunohistochemical staining was carried out for vimentin, S100, αSMA, CD68, and desmin. Positive immunoreactivity was observed for vimentin and αSMA [Figures and ] and negative immunoreactivity for S100, desmin, and CD68, thus confirming the myofibroblastic nature of the tumor. Further medical and radiographical examination confirmed the solitary nature of the lesion, thus excluding myofibromatosis. Hence local-wide excision of the lesion was performed under general anesthesia. Histopathologic findings of the postsurgical specimen also confirmed the diagnosis of myofibroma.\nCurrently the patient is on third-year follow-up without any evidence of tumor recurrence. |
A 27-year-old male patient without significant past medical or surgical history presented to the outpatient clinic department with incidental finding of splenic cyst on ultrasonography which he had undergone few days back for vague pain abdomen. He could not recall any abdominal trauma in the past. Physical examination was unremarkable except for a non-tender splenomegaly up to 3 cm from the left subcoastal margin. A CT scan [] was done which showed splenomegaly with non-enhancing cystic lesion of size 14 cm × 11 cm. Haematological including ELISA for hydatid serology and biochemical tests was normal. Laparoscopic splenectomy was planned for the patient because of the large size and possibility of pressure symptoms. He was given a course of antihelminthics and vaccinated against Streptococcus pneumoniae, Haemophilus influenzae and Neisseria meningitidis was given 2 weeks before surgery.\nOn the operating table, the patient was placed in the right lateral decubitus position, left side up at a 45°–60° angle in reverse Trendelenburg position with the left arm positioned as for a left lateral thoracotomy. Operating table was flexed 30° at the flank to increase the distance between the lower rib and the iliac crest. 5 ports were used, with one 10 mm camera port at supra-umbilical area and rest being 5 mm ports. Intraoperatively, the cyst was causing pressure atrophy of the residual splenic parenchyma and also had multiple perisplenic and pericystic adhesions. The cyst fluid was aspirated which was clear in colour and around 1.5 L in volume. Dissection was started from inferior pole and Splenocolic along with lienorenal was divided. Further medial mobilisation was done by dissecting the gastrosplenic ligament. Splenic vein and artery were ligated at hilum as shown in . Finally, splenophrenic ligament was divided which lead to completely mobilised spleen. Spleen along with cyst was removed in to in endobag by dilating 10 mm right hand working port. Drain was inserted in the operative area. Fluid aspirated was sent for cytological examination and specimen extracted [] was sent for histopathological examination. Cytology smears showed predominantly degenerated neutrophilic infiltrate with no parasites. Histopathology of the specimen showed fibro-collagenous tissue lining the cyst wall with no definite cyst wall lining with no evidence of cellular atypia suggesting pseudocyst of spleen.\nPostoperatively, the patient was started on a liquid diet on the same day and semisolid diet on next day. Patient's recovery was uneventful and was discharged on the 2nd post-operative day. The patient has been asymptomatic for 6 months and engaged in his routine activities. |
The patient was an 18-month-old 10-kg girl suffering from large multiple hepatoblastomas. At 13 months after birth, abdominal distension was detected, and workup studies confirmed the diagnosis of hepatoblastoma (). She underwent systemic chemotherapy, and the tumor size was gradually reduced, but some parts of the tumors remained, being partially responsive to systemic chemotherapy (). Thus, we decided to perform LDLT to remove the tumor completely.\nThe tumors encroached the retrohepatic IVC (); thus we decided to replace the IVC during the LDLT operation. After we prepared a cold-stored fresh iliac homograft, which was recovered from a deceased organ donor, we performed the LDLT operation shortly after the living-donor workup.\nA 220-g left lateral section liver graft was harvested from her 37-year-old mother (). The diameter of the recipient's native retrohepatic IVC was 12–14 mm and that of the common iliac vein homograft was approximately 18 mm; thus we unified the common iliac vein homograft with the graft hepatic vein orifice to make a conjoined opening as in the normal whole liver graft, instead of implanting the graft hepatic vein in the iliac vein homograft in an end-to-side fashion. The iliac vein homograft included the common iliac vein portion and external iliac vein portion; so the 4-cm-long common iliac vein portion was first excised to use for IVC replacement (). The remaining portion of the homograft was used for a hepatic vein patch and portal vein interposition. The graft hepatic vein orifice was incised () and an iliac vein patch was applied to widen the orifice (). After size matching (), the common iliac vein homograft was attached to replace the retrohepatic IVC ().\nDuring right liver mobilization, the tumor-invaded right diaphragm was excised and repaired primarily. The recipient's native liver, including the anterior portion of the retrohepatic IVC, was removed (). The recipient portal vein was enlarged by interposition of the external iliac vein homograft (). The double-lumen outflow orifice of the left lateral section graft with IVC interposition was anastomosed with the recipient's widened suprahepatic IVC stump (). The infrahepatic IVC stump was anastomosed with the interposed iliac vein homograft after length matching (). The graft portal vein was anastomosed with the interposed external iliac vein homograft (). One left hepatic artery was reconstructed, and Roux-en-Y hepaticojejunostomy was done. Since the liver graft was not large for the size of the recipient abdomen, the abdominal wall was repaired primarily.\nThe resected liver specimen showed multiple viable hepatoblastomas without lymph-node metastasis (). No vascular complication developed after LDLT operation (). This patient recovered uneventfully (), and has been doing well for six months to date. She has been undergoing scheduled adjuvant chemotherapy. |
A 42-year-old male patient presented with mild dyspnea and significant dysphagia since 8 months. Gentle exertion and daily routine activities lead to shortness of breath. These symptoms had become worse around the time of his visit and were accompanied by dysphonia, weight loss, and intermittent aspiration. He complained of foreign body sensation. Various physicians failed to make an accurate diagnosis and the symptoms of the patient continued to deteriorate progressively.\nThe patient had no significant past surgical or medical history other than being a smoker for the past 20 years. There was no abnormality of the tongue and oral cavity including hypopharynx on examination by an otorhinolaryngologist. Clinical examination including neurological examination was normal with no evidence of dysarthria.\nNo intracranial pathology was seen on magnetic resonance imaging (MRI) brain, done to rule out intracranial pathology. A barium swallow showed that osteophytes were severely protruding at the anterior portion of the 4th–6th cervical spine and displaced the lower pharynx and the proximal esophagus anterosuperiorly. In the oral phase, there was no abnormality other than premature bolus loss. In the pharyngeal phase, esophagus was displaced anterosuperiorly with constriction anterior to C5 vertebra. X-ray and computerized tomography (CT) scan of the cervical spine were done to confirm anterior cervical osteophytes and revealed the formation of large osteophytes anteriorly from 4th to 6th cervical vertebrae [Figures and ]. Osteophyte of the 5th cervical spine was the most prominent. It protruded 11 mm beyond the vertebral border. C-spine MRI was consistent with anterior herniation of the intervertebral disc with ruptured anterior longitudinal ligament. A T2-weighted image revealed high signal intensity with irregular enhancement. This could be a result of retropharyngeal irritation and inflammatory changes.\nThe patient had developed severe anxiety and sleep disturbances probably due to increasing severity of symptoms and lack of diagnosis. The patient underwent surgical removal of the osteophyte through Smith–Robinson approach. It included removal of the anterior osteophytes, C4–C5, C5–C6 cervical discectomy, interbody stand-alone polyetheretherketone cage as well as bone graft. Complaints of dysphagia were significantly decreased in postoperative period [Figures and ]. He was started on normal diet 1 day after surgery. He also noticed a change in voice back to normal by 7 days. On further follow-up of 1-year, the patient was symptom free. |
A 79-year-old Caucasian male, with a past medical history of atrial fibrillation on warfarin and metoprolol, and coronary artery disease on atorvastatin with previous coronary artery bypass grafting and placement of a dual-function pacemaker/ implantable cardioverter defibrillator (ICD), was on a motor boat in a remote location. The patient’s boat went over a wake of a larger boat passing by. He bounced off his seat in a vertical direction and subsequently landed on his tailbone. After the high impact fall, he complained of both immediate lower back and diffuse abdominal pain but did not seek out urgent medical help.\nTwo days after the initial incident, he started to become pale and diaphoretic; additionally, his ICD delivered three shocks over a 30-min period. He presented via ambulance service to a local community hospital in hemorrhagic shock with a blood pressure of 63/22 and heart rate of 118 beats/min. A primary survey was pertinently positive for hemodynamic instability and diffuse abdominal and lower thoracic spine tenderness.\nHe was resuscitated with 1 L of normal saline leading to an improvement of his pressure to 106/88. Initial laboratory investigations included a hemoglobin of 95 g/L, lactate of 6.1 mmol/L, creatinine of 129, and a supratherapeutic INR of 8.8. An initial non-contrast CT abdomen and pelvis showed moderate hemoperitoneum with sentinel clot in the left upper quadrant and pericolic gutter, as well as the area adjacent to the posterior wall of the stomach. An additional finding of a severely comminuted, minimally displaced burst fracture of the T10 vertebral body was noted (). Further interventions included INR reversal with 3 mg of Vitamin K and 3000 units of prothrombin complex concentrate, and administration of 2 units of packed red blood cells and 2 L of normal saline. Based on clinical severity, the patient was transferred to the trauma service at a tertiary-care Level 1 trauma center.\nPrimary survey revealed a protected airway, spontaneous and bilateral air entry, and hemodynamic stability with a blood pressure of 100/60 and a heart rate of 88 beats/min. His abdomen continued to be mildly distended and tender without peritoneal signs, however the patient reported it had improved since his original presentation to the local hospital. Repeat laboratory investigations revealed a stable hemoglobin of 94 g/L, and correction of his INR to 1.2. Given his stable condition, he underwent a CT RIPIT (Rapid Imaging Protocol in Trauma) [] and CT angiogram (CTA) of the abdomen and pelvis. His imaging revealed pseudoaneurysms of the left gastric artery measuring up to 6 mm with another 9 mm rounded area of increased attenuation along the lesser curve of the stomach (, ). No extravasation was seen. Decision was made to monitor the patient closely with serial abdominal exams and repeat imaging in 72 h, or sooner if the patient exhibited any signs of deterioration.\nOver the next 72 h, the patient’s vital signs and abdominal exams, improved and his hematological profile remained stable. Given the patient’s CHADS score of 2, it was decided to hold therapeutic anticoagulation until the patient’s bleeding risk decreased, however deep venous thrombosis prophylaxis was initiated. A repeat CTA was performed to follow the evolution of the two pseudoaneurysms. The imaging study revealed unchanged pseudoaneurysms but noted the distal aspect of the left gastric artery was attenuated in keeping with a focal dissection and intramural thrombus. Secondary to the dissection, the patient was started on 81 mg of aspirin daily. Over the next few days the patient continued to improve clinically, and was discharged home. The patient lived outside of the local area, and arrangements for close follow up were made. |
A 64-year-old Caucasian male with a medical history of hypertension and symptomatic bradycardia status-post pacemaker implantation presented to the emergency department with the chief complaint of swelling of the right upper extremity for three weeks. It progressed to a generalized swelling of the right shoulder and right side of the neck, restricting his arm movements. He also voiced the concern of worsening shortness of breath which started with the swelling. He did not report any personal or family history of thrombophilia. There was no previous history of any excessive upper extremity exertion or catheterization in the neck. Vital signs on presentation were a blood pressure of 127/61 mmHg, pulse rate of 79/minute, good volume, regular rhythm with no radio-radial or radio-femoral delay, respiratory rate of 19/min with a saturation of 93% on 3-liters nasal cannula, and temperature of 97.5oF. Physical examination revealed right upper extremity swelling without any sensory or motor deficits, right-sided neck swelling, and right-sided facial plethora. Examination of the contralateral arm, as well as the cardiovascular and respiratory systems, was normal. Biochemical investigations were within normal limits. The patient underwent a right upper extremity duplex ultrasonography which revealed an acute non-occlusive thrombus in the proximal right internal jugular vein (Figure ) and right subclavian vein (Figure ) at the cephalic vein confluence. At this point, Factor V Leiden, anti-thrombin III, protein C, and protein S levels were ordered which were normal. The patient was admitted to the medical ward and an intravenous heparin infusion was initiated. Chest radiography did not reveal any cervical rib, and computed tomography (CT) pulmonary angiography showed no evidence of pulmonary embolism. The swelling improved over the course of two days, and the patient was switched to oral apixaban for anticoagulation. He was discharged on apixaban for six months, and a complete resolution of his signs and symptoms was noted at his three-month follow-up examination. |
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