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A 58-year-old woman was admitted with a headache and dizziness that had persisted for one week. Upon neurological examination, she demonstrated left hemifacial numbness due to left fifth cranial nerve palsy and diplopia at left gaze due to left sixth cranial nerve palsy. The patient did not exhibit ataxic gait, and her brain stem reflex and cerebellar signs were intact. She did not complain of tinnitus and did not exhibit nystagmus. She did not have histories of hypertension or diabetes mellitus.\nA computed tomographic (CT) scan revealed a 43×21 mm round mass in the retroclival and prepontine region. Abnormal findings were not observed in the clivus or surrounding bony structure and the posterior cortical lining of the clivus was intact (). Magnetic resonance imaging (MRI) showed heterogeneous high signal intensity of the mass in T2-weighted images (WI) and iso-signal intensity in T1WI (). The mass revealed heterogeneous enhancement with gadolinium (, ). The pons was severely compressed by the mass and contained a cystic lesion showed by high signal intensity in T2WI and iso-signal intensity in T1WI (). The basilar artery was not surrounded by the mass and was displaced to the right side. We did not detect a vascular lesion in MR angiography.\nWe performed surgery via the left retro-labyrinthine transpetrosal approach. After petrosectomy and incision of the petrosal dura and tentorium, a grayish mass covered by arachnoid membrane was exposed between the cranial nerves (). The left fifth and sixth cranial nerve were displaced laterally by the mass, but the other cranial nerve was not. The mass was very friable and easily removed by suction tools. The mass consisted of yellowish, dark material that resembled old hematoma was first removed and then decompressed internally. The sixth cranial nerve was then released and easily detached from the mass capsule. The sixth cranial nerve did not adhere to the mass capsule. The extension of the mass to the pons was safely separated and the pontine surface in contact with the mass was founded intact by mirror. Although the last piece of the mass was attached to the clival dura of a tiny surface, it was easily separated from the dura and there was no penetration or invasion (). The basilar artery was not encased by the tumor, and the cranial nerves on the opposite side were well separated from the mass. The mass was completely removed without injury to the cranial nerves or pons. The final pathological diagnosis was confirmed to be typical chordoma with nuclear atypia (). Immunohistochemical stains demonstrated positive keratin AE1/AE3 and epithelial membrane antigen but negative Ki 67 index (). There was an absence of mitoses and necrosis.\nPostoperatively, the patient gradually recovered from her cranial nerve palsy, diplopia and left hemifacial numbness. No other neurological deficits were found. Twelve months after the operation, we detected no recurrence of the mass on MR images and the cystic lesion in the pons had decreased in size. |
The patient is a 34 year old healthy primigravida with spontaneous dichorionic diamniotic twins and likely bicornuate uterus, with one fetus in each horn, initially diagnosed at 12 weeks gestation by two-dimensional ultrasound. Magnetic resonance imaging at that time reported bicornuate unicollis uterine anatomy, with symmetrical myometrial thickness in both horns. The left cervical canal was noted to communicate with the right lower uterine segment and not directly with the vagina. A thin amniotic band was noted in the superior left uterine horn. Bilateral normal maternal kidneys were noted.\nThe patient subsequently presented at 17 weeks gestation with new onset significant pelvic pain. An MRI at an outside hospital demonstrated a thin myometrial wall (thickness not reported) in the left uterine horn without myometrial disruption, but with moderate pelvic free fluid (Fig. ). The patient’s hematocrit was noted to decline from 32 to 26%, and she was transferred to our tertiary care center for further management. Three-dimensional ultrasound at our institution revealed two separate uterine cavities, each with a live appropriately-grown fetus with normal amniotic fluid. The anatomy of the cervices was difficult to delineate, but a vascular connection was noted between the medial surfaces of each horn. The myometrium over the left horn was noted to be “markedly thinned” superiorly (Fig. ). Moderate hemoperitoneum was documented, with a 3.6 cm clot adherent to the thinnest portion of the myometrium of the left horn. Concern was raised for rupture of a rudimentary uterine horn. A severe cardiac anomaly was incidentally diagnosed in the fetus in the right unicornuate uterine horn.\nGiven these imaging findings and the patient’s ongoing pain, the decision was made to proceed with diagnostic laparoscopy and left hemi-hysterectomy with fetus in situ. The patient underwent ultrasound-guided selective reduction of the fetus in the left uterine horn using intracardiac potassium chloride. Immediately after this procedure, the patient underwent laparoscopy, at which point she was noted to have a right unicornuate uterus with a rudimentary left uterine horn, with 2 cm rupture on the superior surface with amnion protruding (Fig. ), and moderate hemoperitoneum. Due to intraoperative bleeding and suspected amniotic rupture during examination of the left horn, the procedure was converted to an abdominal hemi-hysterectomy. During this procedure, a fibrous but narrow band was noted between the uterine horns, which was ligated and transected using bipolar energy. The procedure and the patient’s recovery were uncomplicated.\nThe pregnancy in the right uterine horn continued postoperatively. At 19 weeks of gestational age, in the setting of a severe fetal cardiac anomaly and high likelihood of preterm delivery complicating surgical correction, the patient underwent laminaria placement and uncomplicated ultrasound-guided dilation and curettage of the right uterine horn.\nThe patient subsequently spontaneously conceived two singleton pregnancies. Normal anatomical surveys and fetal echocardiograms were noted in each. Both pregnancies were complicated by preterm contractions without cervical change. Eighteen months after her surgery, the patient delivered a healthy, small for gestational age (2,580 g) female infant, followed by a healthy 2,722 g male the following year. |
A 55 years old woman who was suffering from a slow progressive spinocerebellar ataxia over the last 20 years participated in this study. Her last MRI showed cerebellar atrophy. She reported that she was a frequent faller due to her poor balance. She believed that one of the major contributors to her falls was having spasticity in her lower limbs.\nSince the PoNS™ is not registered in Australia, it was used with approval from the Therapeutic Goods Administration (TGA) of Australia and the Melbourne Health Human Research Ethics Committee. The patient provided informed consent.\nIntervention consisted of 2 physiotherapy sessions per day, 1.5 h per session (3 h between sessions) for 2 weeks (18 sessions in total plus pre- and post- intervention assessment sessions). Each session was structured in three blocks of 20 min, each focusing on gait performance, balance control and a relaxation/meditation period in the same order. During each block the patient used the PoNS™ device at a comfortable intensity which could be adjusted by the patient or therapist. At least 5 min of rest was given between stimulation blocks. Since the PoNS™ mouthpiece sits on the tongue, the patient was allowed to remove the device in case of excessive salivation to swallow or clean up.\nClinical assessment of gait and balance was performed using the miniBEST, which is a 14-item test scored on a 3-level ordinal scale with a maximum of 28 points. It focuses on anticipatory postural adjustments, reactive postural control, sensory orientation and gait []. The Depression Anxiety Stress Scale (DASS) and cognitive function using the CogLog were also used to assess negative emotional states and memory functioning and executive skills, respectively [, ].\nGait assessment was performed using a set of 4 inertial sensors sampling at 128 Hz (APDM, Portland, OR, USA) placed at the ankles, sacrum and chest, from which spatiotemporal and ranges of motion (RoM) measures were obtained over 40 gait cycles. Included walking measures were: speed (pace of walking), stride length (distance between any two successive points of heel contact of the same foot), cadence (number of steps per unit time), trunk RoM (the full movement potential of trunk) and percentages of stance (when the foot remains in contact with the ground), swing (when the reference foot is not in contact with the ground and swings in the air) and double support (both feet are in contact with the ground) time.\nPosturography was used to assess balance using the same APDM system during double support (DS), tandem (TS) and single-leg (SL) stance for 30 s.\nThe patient completed the 2-week intervention with no adverse effects related to the intensity of the intervention or the use of PoNS device. In the miniBEST, improvements were calculated as the percentage of the possible improvement points, that is: post- minus pre-intervention score as a percentage of 28 (maximum score) minus baseline score. Her score increased by 12 of 19 potential improvement points (63%). Interestingly, most of the improvements occurred in the dynamic (gait) section of the test, which assesses gait speed, turns and obstacle negotiation. She also showed considerable improvement in the anticipatory tasks, being able to stand from a chair independently, rising on toes for more than 3 s, and single-leg standing, as well as in the reactive balance tasks (Table ). In addition, the patient showed a decrease in DASS and CogLog scores.\nGait assessment showed that most spatiotemporal measures remained similar after intervention with a slight increase in the cadence. However, greater changes were observed on arm (20% increases in RoM) and trunk motion (22% more horizontal and 22% less frontal RoM) during walking. Furthermore, in the symmetry measures, asymmetry of stride length and velocity (> 35%) was decreased (Table ).\nPosturographic measures of balance showed large reductions in jerk, sway area and mean velocity for the DS and TS conditions (> 77%). Interestingly, mean velocity in the anteroposterior (AP) direction was increased (> 25%) but decreased in the mediolateral (ML) direction (> − 76%) for the same conditions (DS and TS) (Fig. ). These changes were less pronounced in the SL conditions, which were the most challenging. Other non-linear measures of balance, e.g. mean frequency, also showed important reductions. |
An otherwise healthy 33-year-old woman, gravida 3, para 2, from a Sephardic Jewish origin, was initially referred to our institution at 30.6 weeks of gestation due to a large neck mass found on prenatal ultrasonography (US). Her previous two pregnancies were uncomplicated. The fetal sonogram showed a 10 by 8 cm mass on the right side of the neck, which was not present in detailed scans taken at 14 and 22 weeks. The mass was composed of a cystic portion and a solid portion containing blood vessels and was growing rapidly in subsequent ultrasound studies. A significant polyhydramnios with amniotic fluid index (AFI) of 50 suggested an upper gastrointestinal obstruction and a highly possible airway obstruction as well. Findings were confirmed by fetal magnetic resonance imaging (MRI). In anticipation of the difficulty in establishing a secured airway at birth and the potential complicated resection of the giant tumor after birth, the mother was referred to our hospital for consultation.\nThe parents were in consultation with the maternal fetal team, neonatologist, anesthesiologist, pediatric surgeon, and otolaryngologist. The parents were presented with a guarded prognosis but insisted that the pregnancy continue with maximal efforts during delivery and during the neonatal period.\nTherefore, a planned EXIT procedure, which provides the best chance to establish a patent airway, was offered to our patient, presenting the risks []. Specifically, we informed the parents about the risks for the mother, including significant hemorrhage from the uterus due to the uterine relaxation necessary to avoid placental separation, with a possible uterine resection in the case of a life-threatening hemorrhage.\nKnowing the risk of an unplanned preterm delivery due to polyhydramnios and uterine contractions, we scheduled our patient for a planned cesarean delivery at 34 weeks organizing and preparing a multidisciplinary team ready to perform the EXIT procedure.\nA multidisciplinary team including obstetricians, anesthesiologists, neonatologists, otolaryngologists, pediatric surgeons, pulmonologists, cardiologists, and nursing staff participated. A combined epidural and general anesthesia was planned. Our patient’s blood pressure was monitored continuously to detect and treat a possible event of maternal hypotension in order to maintain good fetal perfusion. After epidural catheter placement while lying on her left side, the parturient returned to lie on her back. Immediately, a severe hypotension (65/30 mmHg) with tachycardia (150 beats/minute) appeared. We related this complication to the polyhydramnios causing a significant decrease in the vena cava flow. After left uterine displacement and bolus of phenylephrine, her blood pressure and heart rate returned to normal. General anesthesia with rapid sequence induction was induced with succinylcholine (100 mg) and propofol (150 mg). During the EXIT procedure an appropriate uterine relaxation was maintained to prevent placenta expulsion. We used a high dose of inhaled anesthetics and minimal nitroglycerine intravenous drip. No bleeding occurred during the procedure.\nA classical uterine incision was made and only the fetal head and upper chest with the cervical mass were delivered through the uterine incision. The rest of the body and the cord were left in situ to avoid placental separation. The amniotic fluid was slowly drained to avoid an abrupt drainage of the fluid and an early separation of the placenta.\nThe multidisciplinary team had planned and rehearsed the following escalating step-by-step scenarios for establishing a secured airway: (1) direct laryngoscopy and intubation attempt by a pediatric anesthesiologist with the aid of a neonatologist, (2) rigid bronchoscopy by an otolaryngologist and possible aid of flexible bronchoscopy by a pediatric pulmonologist, (3) if laryngoscopy and bronchoscopy failed, a tracheostomy was planned by an otolaryngology team. As the tumor was highly vascularized, any debulking procedure was impractical and would have imminently put both the mother and newborn at the risk of death. Analgesia for the newborn using intramuscular fentanyl was prepared in advance.\nAfter the head of the newborn emerged, a direct laryngoscopy was attempted; however, the larynx was not visible as the tumor obstructed the pharynx. Attempts at direct intubation were abolished after 1 minute. Next, a rigid bronchoscopy was performed and only the tip of the epiglottis was visualized. At 11 minutes from delivery, endotracheal intubation was successfully performed. From the time of delivery to the time of intubation, the presence of a good heart rate of the fetus was monitored by echocardiography. After the airway was secured, the female newborn was delivered, the cord was clamped, and the placenta extracted. The arterial cord pH was 7.01 with CO2 of 71 mmHg and lactate of 9.5 mmol/L. Immediately after delivery, a computed tomography (CT) scan was performed under general anesthesia that demonstrated that a tracheostomy could be performed without interfering with the tumor. During the tracheostomy, a biopsy was taken from the tumor that demonstrated both mature and immature teratoma (Fig. ).\nThe birth weight of the newborn girl was 3 kg including the tumor. A giant submental mass that protruded from the right side of her neck was noted. The tumor was covered with a thin skin layer with a large cystic and solid appearance. There were occasional bleedings from several lacerated areas on the tumor surface (Fig. a). A CT study demonstrated the abundant blood supply of the tumor including the fact that the right carotid artery was supplying this tumor (Fig. b). During the first 2 days of life, before the next procedure, the tumor continued to grow significantly, probably partially due to internal bleeding. Her heart function was normal but prerenal azotemia evolved due to loss of large amounts of serotic fluid as well as blood from the lacerated mass.\nBecause resection of the giant, highly vascularized, cervical teratoma could be a fatal procedure due to massive bleeding, we used endovascular embolization a day prior to the tumor resection. The embolization was done by a pediatric cardiologist and an interventional neuroradiologist. Cervical angiography via a femoral line catheter demonstrated the highly vascularized tumor supplied by the external right carotid artery (Fig. a). A carotid occlusion test was performed and showed good blood supply from the contralateral internal carotid artery (ICA). Next, the origin of the external carotid was occluded using detachable platinum coils. Since resection of the tumor implied the sacrifice of all cervical carotid branches it was mandatory to also occlude the cervical ICA and common carotid artery to achieve minimal blood loss during surgery (Fig. b). The procedure was done under general anesthesia.\nUpon withdrawal of the angiography catheter a sudden deterioration of our patient was noted. Fluoroscopy and echocardiography demonstrated cardiac tamponade, probably from a small laceration in the aorta. An urgent pericardiocentesis retrieving 3 ml of blood from the pericardial space allowed fast and full hemodynamic recovery (Fig. c, d). There was no re-accumulation of pericardial blood.\nLess than 24 hours after the embolization, the surgical team, which included a head and neck surgeon, pediatric otolaryngologist, and a plastic surgeon, performed the surgery with a pediatric anesthesiologist. Using LigaSure™ Sealer/Divider (Medtronic), the tumor was dissected including a section of the lacerated skin. Because the main blood supply of the tumor was embolized, the surgery was performed from its distal part along our patient’s mandible towards the proximal part at the junction of her neck and thorax. The tumor impinged into her pharynx, and her larynx and epiglottis were identified and preserved. Her mandible and neck muscles were preserved; however, her vagal nerve was surrounded by the tumor and was sacrificed with the carotid artery. The tumor weighted 800 grams and the overall blood loss was 300 ml. After tumor resection and closure of the skin (Fig. ), the tracheostomy tube was changed. This was followed by a pneumothorax that required a chest tube insertion.\nGradually, after the tumor resection, our patient was weaned from mechanical ventilation. She was fed initially using an orogastric tube and gradually learned to feed orally. After the resection, a right vocal cord paralysis was observed using a flexible fiberoptic laryngoscopy; therefore, we decided to leave the tracheal tube in place. We attributed this finding to an injury of the recurrent laryngeal nerve during the resection. Another neurological sequela was an abduction weakness of her right shoulder which slowly recovered. Neck teratomas can arise from and completely replace the thyroid tissue []. Thyroid function tests demonstrated hypothyroidism and she started receiving thyroid replacement therapy. A follow-up ultrasound of her head was normal. A brain MRI done a month after the final surgery demonstrated normal brain appearance. She was discharged to her home at 3 months of age (Fig. ). |
A 41-year-old male was admitted to our hospital due to gross hematuria for two and a half months. Twelve years previous, the patient underwent transurethral resection, suprapubic cystostomy, and urethral sounds to cure a right seminal vesicle cyst with multiple stones and obstruction of the right ejaculatory duct. At that time, a biopsy diagnosed the seminal vesicle cyst as an epidermal cyst, a benign cyst lined with a thin layer of squamous epithelium. A week after his current admission, perineal and scrotal pains newly developed. Digital rectal examination detected tenderness with a hard and highly elevated posterior prostate compressing the rectal wall. No prostate-specific antigen elevation was detected and urine cytology was negative for malignant cells. Computed tomography (CT) urography revealed hypoplastic change in the right kidney, a 4.9-cm-sized right seminal vesicle cyst with a thickened wall, and benign prostate hyperplasia (). The patient was diagnosed with Zinner syndrome with a seminal vesicle cyst. Transrectal sonography detected a 3.8-cm-sized hypoechoic lesion at the left transitional zone of the prostate showing a bulging contour and prominent vascularity, which favored chronic prostatitis over malignancy. Treatment included not only palliative medication but also aggressive procedures such as nerve blocking for pain control. Six months after the initial onset of gross hematuria, another transrectal biopsy was done and the specimen was pathologically diagnosed as poorly differentiated carcinoma. CT urography and magnetic resonance imaging demonstrated a bulging mass at the left prostate gland, which had increased in size compared with images taken three months previous (). The mass showed signs of internal necrosis and anorectal adhesion, but did not show anorectal invasion. A 4.7-cm-sized seminal vesicle cyst at the right side and necrotic lymph nodes at the left external iliac area were also observed. Through surgery, the urinary bladder, prostate, and bilateral seminal vesicles were removed en bloc. On gross examination, the specimen consisted of an 11.0×6.0×5.0-cm-sized multinodular mass with a tan-white cut surface accompanied by hemorrhage and necrosis. Adjacent to the tumor, the seminal vesicle cyst had a smooth mucosa measuring 5.0×3.0 cm. Under the microscope, squamous metaplasia was observed in the seminal vesicle cyst lining (). Microscopically observed, the tumor was a poorly differentiated carcinoma (). Immunohistochemical analysis of the tumor was positive for p63 () and negative for cytokeratin 7 (), cytokeratin 20 (), and carcinoembryonic antigen (). Although the specimen displayed some immunoactivity for vimentin (), the staining was positive in macrophages, as reported in murine seminal vesicle carcinoma []. Histochemical studies (periodic acid–Schiff [PAS] and mucicarmine) were positive for mucin (). The tumor was pathologically diagnosed as poorly differentiated squamous cell carcinoma arising from the seminal vesicle cyst. The involvement of carcinoma was detected in rectal and right ureter tissues, but no lymph node metastasis was observed. The postoperative course had no further events. The patient was discharged and scheduled for adjuvant concurrent chemoradiation therapy. |
This 2 year-old Hispanic male has a past medical history of hypoplastic left heart syndrome (HLHS) that required multiple cardiothoracic surgeries including a Norwood procedure with a Sano shunt after birth, a bidirectional Glenn procedure at 4 months of age, and two subsequent tricuspid valve repairs. He had a right middle cerebral artery (MCA) stroke at 18 months of age manifesting as left face and arm weakness. At that time, a heterozygous prothrombin G20210A mutation was diagnosed causing thrombophilia. The stroke was medically managed. He ultimately made a full neurologic recovery and was discharged home with warfarin for the thrombophilia.\nHe was most recently admitted for acute left hemiplegia. At 1430 on the day of presentation, his mother noted that he was demonstrating some left-sided weakness that progressed to complete hemiplegia. At 1800 in the emergency department, his mentation and speech were appropriate, but the left hemiplegia persisted and his modified Rankin scale (mRS) was 4. His anticoagulation was subtherapeutic with an INR of 1.6. Suspecting a stroke, a head CT scan was obtained demonstrating an area of hypodensity within the inferior right frontal lobe suggestive of a recent infarct. To obtain more information, the patient was electively intubated and an emergent MRI was obtained (), demonstrating a complete occlusion of the proximal right M1 segment. An area of restricted diffusion was noted deep to the insular cortex involving the right lentiform nucleus and posterior limb of the internal capsule. Patchy areas of restricted diffusion were demonstrated in right frontal region and posterior temporo-occipital junction without corresponding hypointensity on the ADC map indicating subacute areas of infarction.\nGiven the findings of the MRI, the patient was urgently transferred to the endovascular suite for intervention. He remained intubated and under general anesthesia while access was obtained in the right common femoral artery with a 4F sheath. A 65 cm-length, 4F angled Glidecath (Terumo Medical Corporation, Somerset, NJ), was navigated into the right internal carotid artery (ICA) over a 0.035 inch Glidewire (Terumo Medical Corporation, Somerset, NJ) without difficulty given the history of aortic arch reconstruction. An angiogram obtained from a right ICA injection () confirmed the occluded proximal M1 segment. A Trevo Pro 14 microcatheter (Stryker, Kalamazoo, MI) was navigated through the guide catheter into the right ICA over a Synchro 14 micro-guidewire (Stryker, Kalamazoo, MI). The microguidewire was then carefully advanced through the M1 segment thrombus followed by the microcatheter over the guidewire and an angiogram was obtained through the microcatheter after the guidewire was withdrawn (), confirming position of the catheter. A 3 × 20 mm Trevo XP ProVue Retriever stent (Stryker, Kalamazoo, MI) was then deployed into the occluded M1 segment (). After approximately 3 minutes, the stent retriever and microcatheter were withdrawn together as a unit through the 4F guide catheter in the ICA. An angiogram obtained after the pass () revealed that the M1 segment was still occluded and a thrombus fragment had migrated into the right anterior cerebral artery (ACA).\nAfter the failed first pass of the stent retriever, a larger 4 × 20 mm Trevo XP ProVue Retriever stent was deployed into the right M1 segment using a Trevo Pro 18 microcatheter that was first passed through the M1 thrombus over a Synchro 14 microwire (). After deployment of the stent, the microcatheter was carefully withdrawn out of the groin over the stent pusher wire, leaving only the stent retriever and 4F guide catheter in place. Gentle aspiration was then applied to the guide catheter after 3 minutes while the stent retriever was carefully withdrawn from the M1 segment into the 4F catheter in the ICA. A subsequent angiogram showed partial recanalization of the M1 segment. This procedure was repeated for a third pass in the M1 segment. A 4 × 20 mm stent retriever was once again deployed into the M1 clot and the microcatheter was withdrawn. Aspiration was applied to the 4F guide catheter as the stent was withdrawn, resulting in a TICI 2b recanalization of the right MCA territory ().\nNext, attention was turned to the right ACA thrombus (). Using the same technique, a 4 × 20 mm stent retriever was deployed through the ACA thrombus after a microcatheter was passed through the clot over a microwire (). The microcatheter was then withdrawn, leaving only the guide catheter and stent retriever in place. The stent was allowed to incorporate into the thrombus over a period of approximately 3 minutes, and then aspiration was applied to the 4F guide catheter in the ICA as the stent retriever was withdrawn. The resulting cerebral angiogram showed a partial reperfusion in the right MCA territory and complete reperfusion in the right ACA territory (). The puncture to recanalization time for the procedure was 55 minutes and time from symptom onset to full recanalization was 7 hours. The guide catheter and sheath were removed and pressure was held to the groin for 15 minutes.\nThe patient was extubated at the end of the procedure and admitted to the pediatric intensive care unit. At that time, he was moving all of his extremities, but was weaker on the left side. An MRI obtained on the following day did show an increased area of restricted diffusion deep to the insular cortex. He was placed on a heparin infusion as a bridge while warfarin was restarted with a goal INR of 2.0-2.5. The strength in his left arm and leg continued to improve over the course of his hospitalization and was discharged home after a week. At a 30 day return outpatient clinical visit, he was walking normally with only a mild motor deficit in his left hand and a slight facial droop. His mRS at that time was 1. |
A 13-year old boy presented to our hospital with a history of fall onto his right outstretched dominant hand after being involved in a motor vehicle accident. He complained of pain, immediate swelling, and restricted range of motion of the right elbow with loss of sensation over the right ring and little fingers following trauma. Physical examination revealed deformity and generalized swelling of the elbow joint with tenderness at the medial joint line on palpation. Both active and passive elbow joint range of motion were restricted.\nRadiograph of the right elbow showed posterolateral elbow dislocation with intra-articular entrapment of the medial humerus epicondyle avulsion fracture fragment (). Closed manipulative reduction under sedation was attempted and the elbow joint was successfully reduced. However, the medial humerus epicondyle fracture fragment remained entrapped in the elbow joint () and sensory ulnar neuropraxia persisted. Four days after the injury, the patient underwent open reduction and internal fixation of the medial humerus epicondyle avulsion fracture with ulnar nerve decompression through medial elbow approach.\nIntraoperatively, it was noted that the medial humerus epicondyle fracture fragment was entrapped in the elbow joint and impinged on the ulnar nerve distal to the Struther’s ligament (). The fracture fragment was retrieved from the joint and fixed using two 4.0 mm cannulated cancellous screws directed perpendicular to the fracture line and anteriorly to avoid from entering the olecranon fossa. The ulnar nerve was decompressed but not transposed. It was covered with surrounding soft tissue to avoid irritation from the hardware ().\nPost-operatively the elbow was supported in a splint in 90 degree flexion. The wound healed well, and range of motion exercise was started two weeks after surgery. At six weeks follow up, fracture union was noted on radiography () and the patient achieved full active elbow range of motion. Complete recovery of the ulnar sensory neuropraxia occurred six months after surgery. |
Patient 2 was a 31-year-old woman of Salvadoran descent, who delivered three children. At 4 years of age, the patient first presented episodes of seizures and abnormal neurological findings and was diagnosed with HHH syndrome. The patient had developmental disability, manifested by expressive language and attention problems determined upon neuropsychiatric evaluation, and significant myopia. The patient was prescribed a diet consisting of protein restriction to 1.5 g/kg/d during childhood. The patient's weight was at the 25th percentile with height less than the 3rd percentile.\nThe patient's first pregnancy occurred at 18 years old. She had episodes of nausea and dizziness. She was maintained on a low-protein diet in the first trimester, but mild hyperammonemia was detected during the 11th and 12th week of pregnancy; as a result, protein intake was further restricted to 40 g/d. It was unclear if the patient was compliant or not. At 22 weeks gestation, the patient developed seizures, and was initiated with carbamazapine. Brain imaging showed multiple small calcifications of unreported cause, with normal cerebral angiography. The child was born at full term, via Cesarean-section. The baby girl had intrauterine growth restriction (weight <3rd percentile). Although the baby girl remained small, follow-up recorded a normal development at her 2 years of age.\nVery little is described for the course of her second and third pregnancy. The second child was born via Caesarian section. A moderately elevated ornithine concentration (302 μM/L) was measured in the cord blood, but the levels decreased to normal in the newborn's blood 24 hours after birth. The patient's blood ammonia levels increased 24 hours postpartum and were given a treatment consisting of oral sodium benzoate and intravenous arginine. The patient responded well to the treatment. Follow-up showed normal growth and development for the child at 10 months of age.\nThe third child was born via repeat Cesarean section and had transient respiratory distress which required mechanical ventilation. No further information was given regarding the outcome of this child.\nUpon follow-up, the patient was noted to have been prescribed more anticonvulsants, and had significant weight loss from 95 to 50 lb. The patient died suddenly at the age of 31 years and 9 months, with an autopsy revealing two firm nodules in the brain, one each in the left parietal and frontal lobes with no determined cause of death. |
A 77-year-old Caucasian male with a past medical history of insulin-dependent diabetes mellitus, dyslipidemia, hypertension, and chronic kidney disease stage IIIa presented to the emergency department due to bilateral lower extremity pain. The patient was diagnosed and treated for lower extremity cellulitis. Due to pain of his lower extremities with ambulation, the patient requested an indwelling urinary catheter. Therefore, urinary catheter placement was performed on the patient's request. Prior to discharge to the skilled nursing facility, discontinuation of the indwelling urinary catheter was recommended multiple times to the patient, but he refused the catheter's removal. He was discharged to the skilled nursing facility after having completed his antibiotic course. He was to continue furosemide, gabapentin, and also nystatin cream for prophylactic prevention of fungal infection on his feet bilaterally.\nNearly three weeks after discharge to the skilled nursing facility, the patient was brought to the emergency department by emergency medical services due to signs of a possible stroke observed at the facility. He was demonstrating dysarthria, right-sided facial droop, and right-sided weakness. These symptoms were first noted about 2.5 hours prior to arrival. Computed tomography head showed no acute abnormality. The patient was not a candidate for alteplase due to an elevated INR.\nInitial emergency department vitals showed a temperature of 38.3. After blood cultures were drawn, a single dose of one gram of ceftriaxone was given to the patient. Later that day, the patient's antibiotics were broadened to vancomycin and cefepime. On his physical exam, the patient's speech remained dysarthric, and he also exhibited a right-sided facial droop with right-sided weakness. The indwelling urinary catheter from his previous admission was still in place. His labs were remarkable for a creatinine level of 3.50, a blood urea nitrogen of 105, a potassium level of 5.2, and a serum bicarbonate of 17. His white blood cell count was within normal limits. Computed tomography angiography of the head and neck vessels was performed in the emergency department even with his acute on chronic kidney injury with a creatinine of 3.50. The computed tomography scans revealed a 50% to 70% stenosis of the left carotid artery and a less than 50% stenosis of the right carotid artery at the bifurcation. The patient underwent magnetic resonance imaging of the brain the following day, which revealed no signs of an acute stroke.\nUpon returning to his hospital room after his magnetic resonance imaging scan, the patient was observed to be unresponsive with no palpable pulses. Cardiopulmonary resuscitation was started. Once return of spontaneous circulation was achieved, the follow-up electrocardiogram did not reveal any ST-segment or T-wave changes concerning for ischemia or infarction. Transthoracic echocardiogram with a bubble study revealed an ejection fraction estimated at 50% to 55% without any wall motion abnormalities and a negative bubble study. Alarmingly, the echocardiogram reported “a possible vegetation attached to the tricuspid valve with a separate possible vegetation attached to the Eustachian valve.” It also reported mild-to-moderate tricuspid regurgitation.\nAt this time, the patient's blood cultures returned positive for fungal growth. Urine cultures were drawn following the blood culture results, and those cultures also demonstrated fungal growth. The patient's blood and urine cultures were finalized as Candida lusitaniae, and the patient continued on micafungin 150 mg daily. As this organism is highly uncommon, multiple blood cultures were drawn. However, on 3 different days, the blood cultures resulted positive for C. lusitaniae.\nAs the days progressed, the patient displayed findings consistent with an anoxic brain injury. Therefore, the decision was made with the family to withdraw care, and the patient expired soon after. |
An 18-year-old male patient reported with a chief complaint of a recurrent swelling and dull aching pain in upper left back region of the jaw since 1 mo.\nPatient was apparently alright 1 mo back until he experienced dull aching pain in upper left posterior region of the jaw.\nPatient was otherwise healthy 4 years back until he noticed a swelling in upper left region of jaw which slowly increased to a large size. He visited a hospital at his native place and was operated twice one year apart for the same swelling. The swelling reduced in size but did not disappear completely. So, the patient reported to the Department of Oral Pathology and Microbiology with the chief complaint of recurrent swelling and dull pain in upper left region of the jaw. The past medical history of the patient was not contributory.\nNot contributory.\nExtraoral examination revealed a diffuse swelling of approximately 7 cm × 5 cm in size on the left side of face extending antero-posteriorly from left ala of nose to the anterior border of the ramus and supero-inferiorly from infraorbital rim to the corner of the mouth. Skin over the swelling was normal (Figure ). On palpation, the swelling was found to be bony hard in consistency. Temperature over the swelling was slightly raised. A single, left submandibular lymph node was palpable approximately 2 cm × 2 cm in size. Intraorally, a single, smooth, ovoid swelling, extending antero-posteriorly from distal of 22 to mesial of 26 and supero-inferiorly from vestibular depth to marginal gingival was noticed (Figure ). On palpation, it was bony hard and slightly tender with fixity to underlying bone.\nComputed tomography (CT) scan showed a mixed hypodense hyperdense lesion in maxillary left region extending antero-posteriorly from the distal aspect of 21 up to 26 regions and supero-inferiorly from alveolar ridge upto the floor of orbit. The lesion had a well defined, partly corticated periphery (Figure ). It had predominantly hypodense internal structure with multiple intermittent hyperdense flecks present within. Expansion was evident on the buccal aspects of the alveolus, anterior and lateral walls of the left maxillary sinus with thinning and perforation evident at multiple sites. Thinning of the floor of left orbit with invagination of the lesion was noticed (Figure ).\nBased on the clinical and radiographic findings, provisional diagnosis of benign odontogenic tumor was arrived at. Due to the extent of the lesion and history of recurrence, CCOT was considered.\nFollowing routine blood investigations, patient was referred to the department of oral surgery for incisional biopsy of the lesion.\nCareful histopathological examination of Hematoxylin and Eosin (H and E) stained sections showed the following features: cystic lumen was lined by odontogenic epithelium of variable thickness. Basal cells of the epithelium were tall columnar with polarized hyperchromatic nuclei. Stellate reticulum like cells could be noted above the basal cells. The superficial layers showed groups of pale eosinophilic ghost cells. The connective tissue wall was predominantly fibrous with dense bundles of collagen fibres and devoid of inflammation (Figure ). Many active odontogenic rests were also seen in the connective tissue. One or two areas demonstrated globular areas of calcifications.\nThe histopathological diagnosis of the incised biopsy specimen was given as COC.\nSegmental resection was carried out in this case (Figure ) and the specimen was subjected to H and E staining. The histopathological examination revealed a connective tissue wall with odontogenic epithelium. At few places, the epithelium was proliferating with stellate reticulum like cells surrounded by spindle shaped cells. Aggregates of eosinophilic ghost cells surrounded by irregular calcifications could be noted towards the lumen. Large areas of dentinoid were conspicuously present in the subjacent connective tissue (Figure ). At places, active odontogenic rests and metastatic bone was seen.\nSpecial staining with van Gieson’s stain identified dentinoid which stains pinkish red and ghost cells which appear yellow in colour (Figure ). |
A previously healthy 89-year-old female presented to the emergency department complaining of midsternal chest discomfort that radiated to her back. Her chest discomfort began the day prior to presentation, but she initially attributed it to indigestion and thus waited to seek medical attention. Her chest pain persisted however, which prompted her to seek evaluation in the emergency department. Upon arrival, she was given aspirin with resolution of her symptoms. Laboratory analysis revealed an elevated troponin I level of 0.319 and initial creatine kinase (CK) of 12.7. Brain natriuretic peptide level on presentation was not checked. Electrocardiogram (ECG) was notable for diffuse T-wave inversions demonstrating inferior, as well as anterolateral ischemia, and a prolonged QTc of 503 ms (). Echocardiogram demonstrated moderate left ventricular dysfunction (ejection fraction of 35%-40%) with mid to distal anteroseptal, anterolateral, and apical akinesis (). The patient was taken to the catheterization suite and underwent emergent left heart catheterization. Angiography revealed 90% stenosis in the mid right coronary artery (RCA) which was believed to be the culprit lesion. There were no significant obstructive lesions noted in the left anterior descending or left circumflex arteries. She underwent percutaneous coronary intervention (PCI) with successful stent placement to the RCA (). She was then started on appropriate guideline-directed medical therapy and observed in the intensive care unit where her chest pain resolved. Repeat laboratory analysis revealed that CK had decreased to 6.4. ECG obtained following PCI revealed interval improvement of the inferior T-wave inversions, with sustained T-wave abnormalities in the anterolateral leads (). Cardiac magnetic resonance imaging is not available at our facility and was not performed. The remainder of her hospitalization proceeded without incident, and she was discharged home in stable condition three days later.\nThe patient was evaluated two weeks after discharge in an outpatient clinic and was feeling well. She again denied any stressors prior to the onset of pain but reported that the development of chest pain caused her a great deal of emotional distress. Repeat echocardiogram was obtained which revealed normal left ventricular systolic function (). |
Clinical Presentation and Assessment
A 57-year-old male was admitted to the emergency department 12 hours after sudden onset of left lower extremity weakness. Past medical history was relevant for ischemic stroke six months prior to admission with residual left upper extremity weakness, deep vein thrombosis and inferior vena cava filter placement. Baseline modified Rankin score (mRs) was 1. At examination, there was complete hemianopia, facial droop, left lower extremity decreased sensation and weakness. National Institutes of Health Stroke Scale (NIHSS) was 6. He was supposed to be on anticoagulation therapy but had not been taking warfarin for the past two weeks.
Initial assessment confirmed that he was out of therapeutic window (international normalized ratio [INR] was 1.1). Computerized tomography perfusion (CTP) was positive for a small acute cerebral infarction of middle cerebral artery cerebral artery territory (Figure ). Cardiac source of embolism was unlikely after negative ECG for atrial fibrillation and no obvious thrombus displayed on echocardiogram.
Computerized tomography angiography (CTA) of the head and neck revealed an intraluminal thrombus arising from a hypodense plaque in the proximal segment of the right ICA, resulting in 54% of lumen stenosis, according to the NASCET criteria (Figure ). Low attenuation of the plaque imaging was suggestive of soft lipid-rich material, which is known to be an unstable feature. Final diagnosis was acute ischemic stroke and symptomatic moderate carotid stenosis of atherosclerotic etiology.
Management and Outcome
The patient was out of time window to benefit from intravenous tissue plasminogen activator administration. Endovascular or surgical intervention at this point was not considered due to the nonocclusive nature of the thrombus, as well as a high risk of further distal embolization. Decision was then made to manage the case conservatively. He was started on heparin drip, warfarin and aspirin, and was admitted to the neurocritical care unit for close monitoring. Within three days from presentation, the patient’ |
A 44-year-old female factory worker presented to our clinic complaining of a 1 year history of repeated, temporal and parietal headache characterized by bursting sensations. She had previously presented to a local hospital 6 months prior, in April 2016. The patient’s initial brain magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) were normal. CSF studies revealed 68 white cells per microliter and a pressure of 310 mmH2O. Viral encephalitis was suspected and she received acyclovir intravenously (500 mg Q8H for 7 days), but symptoms showed no signs of relief.\nThe patient visited the local hospital again 3 months later, in July 2016, complaining of an aggravated headache and paroxysmal numbness of her left face and arm. She was diagnosed with an ischemic stroke of the right thalamus and administered oral aspirin 100 mg and atorvastatin calcium 20 mg daily (Fig. ). Her numbness improved while the headache showed no improvement.\nShe visited the local hospital for the third time 5 days prior to presenting to our hospital due to the severity of her headache on October 26, 2016. Lumbar puncture was remarkable for a CSF pressure greater than 400 mmH2O, white cell count of 40 per microliter, and a protein level of 1171 mg/L. Cerebral magnetic resonance venography (MRV) revealed underdevelopment of her right lateral sinus and sinus thrombosis was considered as a possible etiology. She was treated with low molecular heparin and her headache was slightly relieved. She was then admitted to our hospital for further treatment.\nThe patient had no significant past medical history, denied a history of blood transfusions and consumption of raw food. Her physical examination was unremarkable, and no abnormal neurologic signs apart from horizontal nystagmus was noted.\nLaboratory tests revealed the proportion of eosinophils to be slightly elevated (3.35%) while other indices were within normal limits. HIV, syphilis, and Herpes simplex virus-1 and 2 antibodies were absent. In addition, the T-Cell spot test, purified protein derivative test for Mycobacillus Tuberclosis and Latex agglutination test for the cryptococcal capsular antigen were all negative. She underwent three lumbar punctures after admission to our clinic and results revealed a high intracranial pressure and an increased white cell count (Table ).\nNo abnormalities were found on cerebral MRA, while MRV imaging suggested underdevelopment of the right lateral sinus. Digital subtraction angiography (DSA) revealed the arterial system to be normal and confirmed dysplasia of the right lateral sinus (Fig. ). Cerebral MRI with contrast revealed several small, hyperintense lesions involving the right cerebellar hemisphere and bilateral occipital lobe, with gadolinium ring enhancement (Fig. ). NGS of CSF sample was further carried out and suggested several possible pathogens including parasites and virus (Table ). Serum was subsequently evaluated for presence of cysticercal antibodies and CSF also confirmed positivity, but negative for Echinococcus granulosus. Review of the patient’s previous imaging records revealed several additional anomalies: her pulmonary CT revealed multiple densities in chest wall soft tissue (Fig. ), and her chest radiographs take 6 months prior to admission to our hospital revealed multiple calcifications on the left side of the neck and right chest wall (Fig. ). A diagnosis of cerebral cysticercosis was therefore finally established. Oral praziquantel (2.5 mg/Kg/d × 7 days for one procedure) was administered thrice. Her headache was recovered markedly. Three months after she was discharged, her secondary cranial MR scan revealed persistence of the aforementioned ring-enhancing lesions (Fig. ). She underwent a repeat lumber puncture that revealed a CSF pressure of 220 mmH2O and 10 white blood cells per microliter. Meanwhile, CSF cysticercal antibodies remained positive. Repeat CSF NGS revealed that the T. solium index dropped considerably compared to initial NGS readings. On follow-up 6 months later, she reported no persisting symptoms.\nCSF was collected via standard procedures. DNA was extracted from 300 μL CSF samples using the TIANamp Micro DNA Kit (DP316, Tiangen Biotech, Beijing, China) and sonicated to a size of 200–300 bps fragments (Bioruptor Pico protocols). DNA libraries were then constructed via end-repaired adaptation added overnight and application of polymerase chain reaction (PCR) amplification to extracted DNA. An Agilent 2100 Bio-analyzer (Agilent Technologies, Santa Clara, CA) in combination with quantitative PCR was utilized to quantify DNA libraries. Sequencing was performed using the BGISEQ-100 platform (BGI-Tianjin, Tianjin, China) [].\nMost high-quality sequencing data were generated and computational subtraction of human host sequences was performed using a Burrows- Wheeler Alignment tool []. Remaining sequencing data were aligned with Microbial Genome Databases, which is composed of 2700 whole genome sequences of viral taxa, 1494 bacterial genomes or scaffolds, 73 fungi and 47 parasites associated with human infectivity. The total number of reads from different samples was standardized as 20 M for comparison. After alignment, previously obtained files were filtered and duplicates removed. ThMmapped data were further processed and with the depth and coverage of each species calculated using Soap Coverage (). A control sample from a non-infected patient was obtained and subjected to the aforementioned procedures. The DNA of CSF samples before and after drug treatment was extracted to construct a complementary DNA (cDNA) library for sequencing. The number of reads from the cDNA library of the patient’s CSF was 37,781,754 (before drug treatment) and 19,043,412 (after drug treatment) respectively. Those of the control sample were 25,962,600. As a result of pathogen detection, T.solium was identified as the most predominant parasite with 3.495% coverage of the T.solium genome in the first detection; however this reduced to 0.078% in the second detection after specific drug therapy. No reads of T.solium were detected from control samples. Reads of viruses were also detected in the CSF samples, later identified as human herpesvirus 4 (HHV-4). After drug therapy, the number of HHV-4 reads decreased approximately 20-fold. The results of CSF NGS prior to and after praziquantel therapy are shown in Table . |
In January 2014, a 55-year-old Caucasian woman was admitted to our Emergency Unit with paroxysmal malignant hypertension accompanied by headache, vertigo, tachycardia, lacrimation, nausea and altered mental status. Her medical history included a caesarean section (1984), an abdominal surgery due to mechanical ileus, a laparoscopic cholecystectomy (1995), gastro-oesophageal reflux and a total thyroidectomy due to a benign non-toxic multi-nodular goiter, later requiring thyroid hormonal substitution. In 2008, an elevated fasting glucose level pointed to an underlying type 2 diabetes mellitus. Hypertension and sinus tachycardia was first diagnosed in 2003. Consequently, the patient underwent several examinations in various hospitals but no underlying organic causes of her complaints could be detected. Endocrinological disorders - especially pheochromocytoma - were excluded on several occasions. Her altered mental status during the hypertensive crises raised the possibility of neurological deficiency, but no signs of intracranial tumor, cerebral vascular lesion, hemorrhage or even epilepsy were detectable. Moreover, cardiac arrhythmias and ischemic heart disease were also excluded. Eventually, in 2004, after numerous diagnostic procedures, panic syndrome was diagnosed; therefore anxiolytic and antidepressant medications were initiated. Between 2004 and 2013 the patient was treated with this combination of psychopharmacological agents. During a thorough psychiatric follow-up period the frequency of the paroxysms dropped noticeably, but the patient seemed to be addicted to the psychopharmacological regimen so the medications were withdrawn in another center. During the following year the patient did not show any clinical symptoms of paroxysmal hypertension. At the time of the current admission to our clinic the patient’s medical therapy consisted of metoprolol 100 mg twice daily, esomeprazole 40 mg daily, levothyroxine 100 μg daily, allopurinol 100 mg daily, and insulin therapy (glulisine insulin 3 times a day and glargine insulin once a day).\nThe patient presented herself at the Division of Rare Diseases for further etiologic examinations. During the first clinical evaluation a very severe attack could be observed. The patient became unconscious and her blood pressure rapidly rose to 230/100 mmHg, with a regular heart rate of 160 to 180/minute (Figure ). Moreover, focal muscle twitching appeared on her left face, and excessive lacrimation and flushing could also be observed. Because of her unstable clinical condition she was immediately admitted to our ICU. Before the administration of additional medications, the patient’s severe clinical condition improved significantly without assistance. By the end of the paroxysm the frequency of sinus rhythm decreased to 90/minute and her blood pressure was also normalized. After the crisis, no signs of arrhythmia or long-standing neurological defects could be observed. No other significant clinical abnormalities could be found during further clinical examinations. Afterwards, during the first week of hospitalization she had attacks two to four times a day. These paroxysmal hypertensive crises lasted for three to five minutes and then disappeared spontaneously without any medical interventions. Between the paroxysms the patient was asymptomatic. Because of the repeated attacks, a combination of alpha- and beta-adrenoceptor blockers was given, which was able to lower her blood pressure and heart rate during the paroxysms, but not the frequency of the attacks. Holter electrocardiography recordings and twelve-lead surface electrocardiograms revealed sudden onset of episodic sinus tachycardia without any signs of further atrial or ventricular arrhythmias (Figure ). Renal Doppler ultrasound examination was performed to exclude renovascular disease. It revealed physiological blood flow in both renal arteries, with no significant difference regarding resistive indices (0.67 versus 0.7, respectively). Although previous examinations were not able to prove any endocrinological background of the paroxysms, a repeated laboratory testing of pheochromocytoma and carcinoid was performed. Laboratory results of our patient are shown in Table . An elevated serum chromogranin A level appeared, but it proved to be a false positive result due to concomitant proton-pump inhibitor (PPI) therapy (after cessation of the PPI, the chromogranin A level was in the normal range). Surprisingly, an adenoma could be detected in the left adrenal gland during computed tomography. Due to the repeated severe clinical symptoms, we were obliged to start treatment of the pheochromocytoma; thus, a cardio-selective beta-blocker (bisoprolol 5 mg twice daily) in combination with an alpha-adrenoceptor-blocker (doxazosine 4 mg once daily) was initiated. During this time, urine concentrations of 5-hydroxyindoleacetic acid (5-HIAA), metanephrine, normetanephrine and dopamine were found to be normal. Although, we measured slightly elevated serum concentrations of noradrenalin and dopamine during an attack, the levels did not fulfill the criteria for pheochromocytoma (Table ). To ensure the safe exclusion of pheochromocytoma, an iodine-131-metaiodobenzylguanidine (131I-MIBG) scan was also performed, which did not reveal any abnormalities relating to adrenal gland dysfunction (Figure ). Furthermore, hyperaldosteronism as a very rare cause of paroxysmal hypertension could also be excluded. Further laboratory tests helped to exclude any hormonal abnormalities; thus the aforementioned adrenal gland adenoma was regarded as an ‘incidentaloma’. Thyroid laboratory tests showed the effective hormonal substitution of hypothyroidism secondary to the previous thyroidectomy. After excluding the possibility of endocrine disorders we focused on anxiolytic medication. For this reason psychiatric examination was performed and alprazolam was re-administered in a daily dose of 1 mg (0.5 mg twice daily). We could demonstrate an immediate clinical improvement; furthermore, the daily dose of alpha- and beta-blockers could also be decreased. During the administration of alprazolam at a daily dose of 1 mg, sleepiness and fatigue occurred, therefore we decreased the daily dose to 0.5 mg. Consequently, the paroxysmal increase in blood pressure reappeared so further therapy of 1 mg was necessary for maintenance. After a four-week period on the ICU, the patient was discharged though still with mild symptoms but with an improved quality of life. The systolic and diastolic blood pressures and heart rate collected after the discharge of our patient were inserted into the manuscript (Table ). |
Our patient was a 15-year-old Iranian male who presented to the Oral and Maxillofacial Medicine Department of School of Dentistry, complaining of a painless swelling on his tongue from 1 month earlier, which was growing fast (Figure ). He did not recall any history of trauma or tongue biting. His medical history was unremarkable, and he was not using any medication.\nHe did not have any symptoms such as pain, paresthesia, altered sense of taste, or mouth dryness. Intraoral examination revealed an exophytic nodular sessile mass with an intact surface with the same color as the surrounding mucosa, measuring 1.5 × 1.5 cm in size. It had a firm consistency and was located in the posterolateral surface of the tongue, extending to the ventral surface. The lesion was freely moving beneath the covering mucosa. The patient had no lymphadenopathy.\nBased on the patient's history and clinical findings, a magnetic resonance image (MRI) of the tongue without contrast was requested for the patient, which revealed a tongue mass with well-defined borders measuring 15 × 12 mm, which was hypersignal on T2-weighted, and hyposignal on T1-weighted images.\nBased on the clinical findings (intact mucosal surface and no attachment to the epithelium) and the information acquired from the MRI, benign mesenchymal tumors were considered in the list of differential diagnosis. The fast growth rate of the tumor in our case was concerning. After obtaining written informed consent from the patient and his parents, the patient underwent excisional biopsy under local anesthesia by an oral and maxillofacial surgeon. The mass was submucosal, and after elevating a mucosal flap, a pedunculated yellow-color mass with a smooth surface appeared, which measured 1 × 1 cm, and was easily resected with blunt dissection (Figure ). The specimen was sent to the Pathology Department in formalin. The pathology report was as follows:\nMicroscopic examination revealed a benign neoplasm with a clear capsule composed of cells with spindle-shaped nuclei, forming an Antoni A pattern. The tumoral cells did not show any cellular atypia or mitosis (Figure ).\nBecause of the benign nature of the lesion, no additional treatment was indicated. After the postoperative period, the patient's complaints, including the swelling, were resolved. The patient had no signs of recurrence in the first follow-up session scheduled 1 year postoperatively (Figure ). |
A 71 year-old male presented for an urgent biopsy of a lesion of Corpus Callosum on a background of clinically diagnosed intra-operative anaphylaxis for the same procedure 4 days prior, which was abandoned. Symptoms of the initial presentation, which brought the patient under the care of the Neurosurgical team, included impaired ability to self-care and mild cognitive dysfunction. Surgical and Medical teams deemed the histo-pathological diagnosis essential due to the need for tailored subsequent medical management including potential urgent administration of chemotherapy and radiation.\nThe specialised allergy testing was not feasible due to the brief 4 day timeline proposed between the two procedures. Decision was made to proceed with surgery due to the urgent need for the identification of the nature of the lesion.\nBoth patient and the prior care episode were evaluated. Patient had a background of treated hypertension, with no other underlying illnesses. Prior to this, he had undergone uneventful surgical procedures and anaesthesia.\nHistory, records of the procedure, and tests for mast cell mediators, were reviewed by the attending anaesthetist. During the first care episode patient was given a single dose of midazolam, followed by remifentanil and propofol total intravenous anaesthesia, rocuronium and cephazolin in quick succession. The laryngoscopic view was a Grade 3 Cormack and Lehane, with direct laryngoscopy utilising a Macintosh 4 Blade. CMAC® Blade 4 Videolaryngoscope demonstrated an equivalent Grade 3 Cormack and Lehane view. Following successful airway management, patient developed intractable hypotension, concomitant bronchospasm and was diagnosed with clinical anaphylactic episode. The patient was treated with an adrenaline infusion, procedure was abandoned and he was admitted with an endotracheal tube in situ to Intensive Care Unit where he recovered uneventfully. The patient re-presented 4 days later for a repeat procedure. Specific allergen testing was unavailable and deemed not to be feasible at this point in time.\nDuring the repeat care episode, decision was made to secure the airway via an awake flexible endoscopic intubation prior to administering general anaesthesia. Airway was topicalised generously using a mix of 4% nebulised xylocaine, 10% local anaesthetic sprays to the back of the pharynx, and topical co-phenylcaine nasal spray. In addition to the above, Disposable devilbis atomiser was used to a total of 9 mg.kg− 1 of xylocaine. Remifentanil infusion at the dose of 0.05 mcg.kg− 1.min− 1 was used during the intubation and airway was secured uneventfully through the nose with a flexible Storztm 5.1 mm video fiber-optic bronchoscope. After connecting the breathing circuit, confirmation of CO2 was obtained and patient was administered 250 mg of thiopentone. Remifentanil infusion was increased to 0.2 mcg.kg− 1.min− 1 and maintained at this level through the case. Anaesthesia was maintained with Sevoflurane at a total dose of less than 1 MAC. For infection prophylaxis, 600 mg of clindamycin was administered. Procedure was completed uneventfully, patient extubated and taken to recovery. Using the above technique, the only medication in common between the two episodes of care was remifentanil. |
A 32-year-old multiparous woman was referred for abdominal pain, bloody discharge, and an absence of fetal movement at the 37 weeks of her pregnancy. Absence of fetal heartbeat and placental abruption were diagnosed by ultrasound. As labor began spontaneously, we augmented it by amniotomy. The patient delivered a dead female infant weighing 2,928 g, and simultaneously delivered a placenta with large blood clots in a short time. Thereafter, she had profuse hemorrhage refractory to manual compression of the uterus, total 30 units of intravenous oxytocin, and 0.4 mg of methylergometrine.\nThe laboratory data showed severe anemia and DIC in which the hemoglobin level and the platelet count had dropped from 10.3 to 5.8 g/dL and 72,000 to 36,000/μL, respectively, in 3 hours, and the blood fibrinogen was below the limit of detection. In spite of rapid transfusion with packed red cells and fresh frozen plasma, the bleeding continued and the total blood loss amounted to more than 5,000 mL.\nSince she fell into hypotension with systolic blood pressure as low as 69 mmHg and oliguria at that time, we attempted our modified UBT to achieve hemostasis after verbal informed consent. The intrauterine balloon catheter wrapped with TXA-soaked gauze was inserted into the uterus and inflated with 120 mL of sterile water until the lowest part of the balloon was fitted to internal uterine os. The vagina was then packed with gauze. Transfusions amounted to 20 units of packed red cells, 20 units of fresh frozen plasma, and 5 units of platelet concentrates. Uterotonics and antibiotics were also given. The patient was strictly monitored with the balloon in place.\nAfter confirming immediate and complete hemostasis, both the balloon and gauze were removed on the following morning after 10 hours of indwelling. She recovered well and her hematological data also returned to normal. She was discharged in stable condition on the fourth postpartum day. |
A 33-year-old female patient with no particular underlying disease was referred for right cheek swelling 4 days after blunt trauma on the face. When she visited the emergency room, there was hematoma on the right cheek and the patient was unable to open the right eye due to severe swelling. We removed the hematoma on the cheek and bacterial examination was done. She was also consulted to an ophthalmologist but there were limitations in examination due to excessive periorbital swelling.\nSix years ago, she was operated for fractured craniofacial bone which the right side fronto-orbital, zygomaticomaxillary complex due to a traffic accident. She underwent surgery with consultation through the neurosurgery department at that time. The midface was reconstructed with autologous bone and fixated with metal plates, whereas the supraorbital rim and orbital roof were reconstructed with artificial bone. After that, the reduction site was still vulnerable to sinus-orbit-brain connection after surgery ().\nThe next day after treatment in the emergency room, the patient came as an outpatient. The cheek swelling still remained with proptosis, and pus was recognized in the conjunctiva. So, we suspected periorbital infection and checked the periorbital area using an enhanced computed tomography (CT). In the CT image, it showed severe sinusitis in the right side as well as a diffuse infectious aspect in the orbit ().\nThus, we planned an emergency operation and conducted a consultation to the otorhinolaryngology department for evaluation of the sinusitis. In the operation room, diffusely spread pus was observed inside the orbit. The bacterial examination was performed in the orbit and in the sinus respectively. Five days later, the same bacteria which Prevotella oralis and methicillin-resistant Staphylococcus epidermidis (MRSA) were identified in both areas.\nInitially, we used experimental intravenous antibiotics such as 3rd generation cephalosporin and aminoglycoside to cover wide spectrum bacteria. But, antibiotics were changed to vancomycin and quinolone after bacterial identification. Medical and surgical treatment, blood test, and radiologic test were regularly performed for more than a month.\nAs a result, the intraorbital infection was resolved, but blindness and extraocular movement limitation were inevitable (). In radiological evaluation, it revealed right intraorbital diffuse enhancement and paranasal sinusitis in initial magnetic resonance image (MRI) initially, but it showed the improvement in 4-month follow-up MRI image (). One interesting thing was that eosinophilia gradually became worse during the treatment period, but it showed a normal level after improvement (). |
A 70-year-old man smoker with a previous history of a transient ischemic attack, was referred to the emergency department of our hospital because of a pain to the left shoulder and arm that had started 4 d ago and was unresponsive to analgesics.\nUpon admission, the electrocardiogram showed anteroseptal ST elevation myocardial infarction (Figure ) and transthoracic echocardiography revealed left ventricular hypertrophy, that was more pronounced at the interventricular septum, compatible with hypertrophic cardiomyopathy. Additional findings were an apical aneurysm, and moderate attenuation of systolic function. High sensitivity troponin I was elevated to 11037 ng/L. The patient was transferred to the coronary care unit and the next day coronary angiography revealed left anterior descending artery occlusion at the mid-level (Figure ). Subsequently, he was submitted to balloon angioplasty with placement of a drug-eluting stent (Resolute Integrity, 3 mm × 18 mm, Figure ). The patient remained asymptomatic and was discharged under optimal medical treatment including aspirin, clopidogrel, simvastatin, metoprolol, furosemide, lisinopril and eplerenone.\nFour days later and about 20 min after taking his evening medication that was metoprolol and simvastatin and during ingestion of Greek rice pudding made of sheep milk, rice and sugar, the patient started gradually to develop lip swelling and itching followed by erythematous rash in all over his body. Within, approximately, 15 min he complained of chest pain and discomfort spreading to the left shoulder and arm. He was immediately transferred to the emergency department of our hospital. On arrival, the patient was covered in all his body with rash accompanied by itching and angioedema of the lips. The electrocardiogram showed ST elevation in V1-V4 leads (Figure ). Hydrocortisone and dimetindene maleate was given intravenously together with oral desloratadine and he was transferred to the catheterization laboratory, where coronary angiography revealed stent thrombosis with left anterior descending coronary artery occlusion (Figure ). The patient underwent thrombus aspiration that was followed by an additional stent placement (stent in stent procedure, drug eluting stent 3 mm × 16 mm, Figure ). However, mild chest pain remained for about 2 h and was attributed to “no reflow” phenomenon. Transthoracic echocardiography revealed, apart from hypertrophic cardiomyopathy with asymmetrical septal hypertrophy, also thrombus formation in an apical aneurysm (Figure and F) necessitating heparin infusion. Contrast-echocardiography with Sonovue-Sulfur hexafluoride microbubbles revealed sessile apical thrombus (Figure ). Tryptase was elevated confirming an allergic reaction. The patient had an uneventful recovery and was scheduled for discharge after an allergology investigation.\nIn order to identify what had triggered the allergic reaction, skin prick tests were performed, under strict hemodynamic monitoring, for simvastatin and metoprolol that were the drugs the patient was taking after the first episode, and were inconclusive. Subsequently, it was decided to proceed to an oral food challenge test, again under strict hemodynamic monitoring, using the rice putting ingredients that were sheep milk, rice and sugar according to the protocol described previously[]. Following an initial amount of 0.6 g of sheep milk given slowly and 15 min after swallowing of 5 g of sheep milk the patient suddenly felt unwell, dizzy, started sweating, developed urticarial rash and complained of severe dyspnea. Soon after, he became disorientated and sleepy. On examination, he was pale with bronchospasm accompanied by hypoxemia (SpO2 82%), and sinus tachycardia (110/60 mmHg, 125 bpm), feeling itchy but without electrocardiographic changes. He was immediately treated with 250 mg hydrocortisone intravenously, 4 mg dimetindene maleate intravenously and 5 mg desloratadine orally with improvement in signs and symptoms. He gradually became stable and asymptomatic. Blood examinations, 10 min after onset of symptoms were performed for troponin, IgE antibodies and tryptase. Troponin was not increased, eosinophils were 70/μL, but IgE levels were increased to > 1000 IU/mL (normal values: 1-183 IU/mL). We did not proceed to challenge the patient with rice or sugar on ethical grounds, while the patient recalled that he was apprehended to sheep milk in the past.\nHe had an uncomplicated hospital follow-up and was discharged with the advice neither to eat rice pudding nor to drink sheep’s milk again. |
A 68-year-old man presented to our institution. He previously underwent laparoscopic intersphincteric resection of the rectum with diverting loop ileostomy for lower rectal cancer at the age of 56. The immediate postoperative course was uneventful. A pathological examination revealed that the patient had T1, N1a, M0, Stage IIIA rectal adenocarcinoma. He did not receive adjuvant chemotherapy, and there was no recurrence of cancer during the follow-up period. However, soon after surgery, he developed an anastomotic stenosis requiring repeated endoscopic balloon dilatation.\nHis loop stoma was not reversed because these treatments were unsuccessful. Therefore, the surgeon proposed surgical resection of the anastomosis, but the patient did not agree to undergo permanent colostomy. Consequently, he had lived with loop ileostomy since undergoing surgery.\nTwelve years postoperatively, he felt swelling and pain in the scrotum. He was seen by a local physician and diagnosed with a perineal abscess. He was referred to a urologist at our hospital for treatment. Computed tomography (CT) scans revealed a low-density area and air-fluid level in the perianal region (Fig. ), which was consistent with the signs of a perianal abscess. Drainage was performed, and the abscess was resolved. The urologist was concerned about the cause of the perineal abscess, and anastomotic complications were considered to be associated with the abscess. The patient was then referred to the department of surgery for further evaluation and treatment.\nDuring examinations, we found that the patient did not have a significant medical history, including inflammatory bowel disease. He denied having any allergies. He had undergone surgery for an inguinal hernia and duodenal ulcer. He quit smoking after undergoing rectal cancer surgery. He took oral antibiotics after the perineal abscess was drained; otherwise, he denied taking any routine medications.\nHe was 161 cm in height and 50 kg in weight, and his body mass index was 19 kg/m2. A physical examination revealed a well-healed scar on his abdomen and a loop stoma in the right lower quadrant. A digital examination revealed severe stenosis at 2 cm from the anal verge. The patient was afebrile and did not have pain in the scrotum or anus. Given his stable medical condition, we judged that urgent surgical intervention was unnecessary. Close observation with a radiological evaluation was planned.\nOne month later, the patient developed skin ulcers in the right ankle (Fig. ) and around the stoma (Fig. ), with a fever over 39 °C. He also presented with bloody discharge per anus. Laboratory data showed there was an elevated C-reactive protein level (11.1 mg/dL) and white blood cell count (7710/mL), while the hemoglobin and albumin levels were decreased (9.4 and 2.6 g/dL, respectively). Regarding the skin ulcers, the culture studies were negative for bacteria, whereas a pathological examination of the biopsy specimen revealed pyoderma gangrenosum. These findings suggested that the patient’s acute illness may have reflected an inflammatory response rather than infectious disease.\nColonoscopy revealed that the bowel lumen had narrowed at 2 cm from the anal verge and extended to 10 cm from the anal verge, where a fistula was incidentally found (Fig. ). Moreover, edematous and easily hemorrhagic mucosa was observed throughout the proximal colon (presumably the transverse colon). The endoscopic examination was terminated soon after we detected a mucosal laceration in the transverse colon that was possibly caused by air insufflation (Fig. ). A pathological examination of a biopsy specimen of the colon revealed inflammatory mucosa with infiltration of neutrophils, eosinophils, and lymphocytes. These findings were interpreted as nonspecific colitis and did not indicate a definitive diagnosis of a condition such as UC, DC, or infectious colitis. Contrast-enhanced abdominal CT did not show signs of cancer recurrence or bowel ischemia.\nA contrast enema confirmed that the distal bowel was narrowed with a fistula, whereas the remnant colon appeared to be consistent with the “lead pipe” phenomenon (Fig. ). We observed active and diffuse mucosal inflammation that indicated UC; pyoderma gangrenosum of the skin is well-known to be an extra-intestinal feature of UC. Given the findings of the physical, laboratory, and imaging studies, we hypothesized that the pathogenesis of the disease was either UC in the diverted colon or DC mimicking UC.\nAfter reviewing the patient’s clinical episodes and extensively discussing the case with gastroenterologists, surgeons, stoma nurses, and the patient, we proposed total colectomy with end ileostomy. Then, the patient finally agreed to undergo this procedure.\nWith the patient under general anesthesia in the lithotomy position, we performed total colectomy with open laparotomy and transperineal resection of the rectum, in accordance with the abdominoperineal resection. The loop ileostomy was taken down and the end ileostomy was reconstructed at the same stoma site. The operative time was 267 min and the estimated blood loss was 210 mL.\nThe postoperative course was uneventful, and he was discharged home on the 11th postoperative day.\nA macroscopic view of the resected specimen is shown in Fig. . The coloanal anastomosis had significant stenosis. There was a fistula proximal to the anastomosis. There was atrophic mucosa, and haustra had disappeared from the distal colon. In contrast, the mucosa appeared to be reddened, edematous, and rather dilated in the proximal colon. The pathological findings included erosion and ulceration in the edematous wall, a crypt abscess, and inflammatory infiltration into the mucosa throughout the colon (Fig. –), without any evidence of dysplasia or carcinoma. The pathological diagnosis was consistent with UC. Given that he developed these inflammatory mucosal alterations after the surgery requiring fecal diversion, we concluded that DC mimicking UC was appropriate for the final diagnosis.\nThe skin ulcers in the right ankle and around the stoma healed over time. Six months after the last surgery, the ulcer areas were significantly reduced (Fig. –). Three years after the last surgery, the skin ulcers were completely healed with some scar tissue (Fig. –).\nWe reported the rare case of a man with acute and severe DC mimicking UC with extra-intestinal manifestations, and he was successfully managed with surgical treatment. His clinical symptoms and histological findings were consistent with the findings of UC, suggesting there is a possible pathogenetic link between DC and UC.\nDC is a frequently seen consequence of the interrupted fecal stream and is characterized by nonspecific mucosal inflammation. In patients who undergo fecal diversion surgery, 70–91% have the endoscopic diagnosis of DC, and 70–100% of those have some histological changes [, , , ]. Typically, the altered bacterial flora decreases luminal SCFA in the diverted intestine, and this may play a role in the development of DC [, ]. Despite the high incidence of DC, most patients are asymptomatic [, ]. Surgical re-anastomosis is the most favorable treatment, whereas some medications are also available [–]. However, acute and severe colitis may occur [, ], and a careful assessment with a prompt decision about the treatment is required in clinical settings.\nIn our case, the decision-making process was as follows. Initially, we considered nonsurgical, conservative treatment targeting UC. However, an enema or oral agents could not reach the diseased colon directly, and steroids or immunomodulators may have provoked a secondary infection and/or recurrent perineal abscess. The presence of a fistula also prohibited the use of these agents. There does not appear to be sufficient evidence supporting cytapheresis for the treatment of DC.\nSecond, the friability of the colonic mucosa represented by insufflation-induced lacerations required urgent and effective treatment. Although the patient underwent diversion, he was nearly at risk of experiencing colonic perforation. Moreover, imaging studies had already shown the presence of a mucosal fistula.\nThird, pyoderma gangrenosum of the skin had worsened rapidly. These features were the patient’s chief complaints, and delayed or prolonged treatment would have diminished his quality of life. Finally, we considered reversing the fecal stream with loop ileostomy closure. However, we deemed it impossible because of the anastomotic stenosis and colonic fistula. Thereafter, we decided to perform surgical resection of the residual colon, including the coloanal anastomosis.\nTo diagnose DC, it is necessary to exclude other bowel disorders such as IBD, radiation colitis, infectious colitis, and nonsteroidal anti-inflammatory drug-associated colitis. A histological examination is paramount for the diagnosis; however, a comprehensive assessment of the patient’s disease, social, and medical histories is also required to determine the treatment strategy.\nDC is regarded as a multifactorial disease []; the causative factors include alterations in intestinal bacterial flora [, , , ], overproduction of oxygen free radicals, impairment of butyrate oxidation [, ], defects in the transport of SCFA, and immunological factors []. Regarding immunological factors, some intestinal mucosal changes or disruption may produce inflammatory or immunoregulatory cytokines, and they induce predominant T helper cell type 1 (Th1). A previous report demonstrated that the Th1 phenotype might be the pathogenesis of DC []. Others indicated that tumor necrosis factor alpha, interleukin 1 beta, interleukin 6, and transforming growth factor beta are important in the treatment of DC [–].\nThe endoscopic features of DC include erythema, friability, and edema, and in severe cases, ulcers, filiform and inflammatory polyps, and strictures are found [–]. The most common pathological features include expanded lymphoid aggregates and inflammation in the lamina propria with lymphocytes and plasma cells [, ].\nIn some cases, follicular lymphoid hyperplasia with crypt distortion and basal lymphoplasmacytosis were observed in DC [, , ]. In such cases, the crypts may appear atrophic and short and are displaced by lymphoid infiltrates. Lymphoid hyperplasia, in which the germinal centers of B and T cell lymphocytes are enlarged, is found in pediatric patients but not in all adult patients with DC [, , , , ]. The microscopic abnormalities found in those with DC may also occur in those with UC, and these diseases cannot be distinguished from one another []. One study hypothesized that the histological resemblance of DC to active UC suggests there is a pathogenetic link between the two diseases [].\nImmunological factors could play important roles in the pathogenesis of both DC and UC. In our case, the differential diagnoses of DC and UC remain unclear. The acute illness of this patient was represented by a perineal abscess, high-grade fever, bloody discharge per anus, and skin ulcers, which could be interpreted as UC with extra-intestinal manifestations. The colonic fistula may indicate chronic inflammation in the diverted colon. These findings suggest that DC may mimic UC, and acute progression of the disease can eventuate in a long-term period in patients without a pre-existing IBD.\nThis report has some limitations. There is still controversy about how to distinguish DC and UC, and there are concerns about total colectomy in patients with acute and severe diseases. Larger case series are required to compare symptomatic and asymptomatic patients with DC and should include periodic histological examinations of the diverted colon. This will help to elucidate the pathogenesis of DC. |
A 59-year-old man initially presented with mild back pain located in the projection of the lumbosacral (LS) spine, radiating down the right leg. The pain had first occurred a year before the initial hospitalization. The first magnetic resonance imaging (MRI) of the LS spine showed moderate degenerative changes with mild disc protrusions that did not indicate neurosurgical treatment, but required physiotherapy. However, 3 months later, the patient developed paresthesias in the right leg and lower abdomen. He also showed signs of motor weakness in the right leg, forcing him to use a crutch when walking. Another cycle of physiotherapy was recommended, resulting in no signs of improvement. Repeated MRI of the LS spine showed the same findings as the first scan. Finally, the patient presented with paresis, Gross Motor Function Classification System (GMFCS) IV, increased muscle tone, hypotrophic muscles, and vivid muscle reflexes in the right leg (GMFCS IV), as well as hypoesthesia and hypoalgesia along the T8 dermatome.\nMRI of the thoracic spine was performed, showing an oval intramedullary lesion located at the T4/5 intervertebral level []. The lesion did not show any change at 1-month follow-up MRI. Due to the hyperintensity seen on the T1-weighted sequence, the lesion was interpreted as a subacute intramedullary hematoma. Gadolinium contrast was not used. No signs of arteriovenous malformations or arteriovenous fistulas were found on these MRI scans.\nDue to progression of neurological symptoms, a neurosurgical intervention was indicated. The procedure was done under general anesthesia, and the patient was placed in the prone position. After accessing and removing the T4 and T5 laminae, the dura was exposed and intraoperative ultrasound was used to verify the location of the lesion. Using microsurgical technique, a linear incision of the dura and midline myelotomy were done. The intramedullary process was exposed and macroscopic evaluation revealed it to be a soft yellowish tumor [] which did not resemble a hematoma, but showed characteristics typical of a lipoma. Samples for histopathological examination were obtained, and maximal reduction of the process was performed using the cavitron ultrasonic surgical aspirator. During the procedure, the patient's somatosensory-evoked potentials (SSEPs) and motor-evoked potentials (MEPs) were monitored. The SSEPs did not show pathological changes throughout the procedure, whereas MEPs were absent from the onset and could not be monitored.\nHistopathological examination revealed lobes of mature fat cells [], thus confirming the diagnosis of intramedullary spinal cord lipoma. Postoperatively, the patient's motor function temporarily deteriorated and a repeat MRI of the thoracic spine was done []. A significant reduction in size of the intramedullary lipoma was revealed and postoperative complications were excluded. After physiotherapy, the patient's motor function recovered. |
A 19-year-old man presented to our division for the management of severe right lower quadrant pain. His previous history was remarkable for juvenile rheumatoid arthritis starting at 12 years of age, involving the sacroiliac and mandibular joint as well as the right ankle and hip. He was tested positive for HLA-B27, but tests for antinuclear antibodies and rheumatoid factor proved to be negative. At 16 years of age etanercept 50 mg s.c. was started which initially relieved the symptoms, so the dose was reduced to every 2 weeks after 1 year of treatment. However, 1 year later the joint pain recurred and was refractory to etanercept dose escalation (weekly) and add-on naproxen. Two months before the onset of abdominal pain the treatment regime was changed to adalimumab 40 mg s.c. every 2 weeks and the patient remained free of rheumatologic problems for the next year.\nDuring evaluation for abdominal pain, lower right quadrant tenderness and a palpable tumor were noted. Blood work revealed systemic inflammation (C-reactive protein 46 mg/l and leukocytosis with 13.7 × 109 leucocytes per liter with 84% neutrophils). Ultrasound and computed tomography suggested terminal ileitis and abdominal lymphadenopathy. Stool tests for microorganisms including Clostridium difficile were negative. Colonoscopy demonstrated an ulcerated ileocecal valve with a narrow opening that could not be intubated. Histology confirmed granulomatous inflammation and acute inflammation, and all tests for infectious diseases, including acid-fast staining, mycobacteria cultures and immunohistochemistry for cytomegalovirus, were negative.\nSince the patient's presentation was consistent with new-onset CD, we continued adalimumab treatment and started budesonide at 9 mg/day. Subsequently, abdominal pain improved; however, surveillance of small bowel inflammation by abdominal ultrasound continued to show wall thickening >6 mm, consistent with active inflammation, and budesonide was increased to 15 mg/day. Due to recurrent abdominal symptoms treatment was switched to infliximab 5 mg/kg body weight, but abdominal pain only temporarily improved and recurred only 3 months after starting infliximab. The patient was now complaining of constant abdominal pain and had lost 12 kg of body weight (body mass index 16 kg/m2). Magnetic resonance imaging revealed chronic terminal ileitis and a complex fistula system. In hospital parenteral nutrition was started, infliximab was stopped, and 9 months after the onset of abdominal pain, right-sided colectomy with an ileocolic anastomosis was performed (fig. ). The immediate postoperative course was unremarkable; the patient remained free of symptoms and body weight increased.\nThree months after surgery, joint inflammation recurred in the hips, shoulders, right foot, lumbar spine and sacroiliac joint. Due to limited therapeutic options for the treatment of ileosacral arthritis, etanercept 50 mg s.c. weekly was restarted after careful discussion with the patient. Etanercept immediately relived joint pains, but 14 days after restarting the TNF inhibitor abdominal pain recurred, accompanied by an increase in calprotectin levels. Colonoscopy revealed multiple ulcers at the ileocolic anastomosis and histology confirmed ulceration. Abdominal pain slowly resolved after etanercept had been discontinued. The clinical course is summarized in figure . |
An 82-year-old woman presented to our institution for evaluation of her right TKA, which was performed in 2009 at an outside hospital. Her past medical history was significant only for rheumatoid arthritis (RA) for which she usually took adalimumab biweekly, methothrexate and celecoxib but reported this was well controlled.\nPer her outside operative records, implants used at the initial surgery were a cemented Triathlon® CR femoral component, a 9 mm size 3 X3® CS insert, a size 3 Triathlon® universal tibial baseplate, and a 10 mm asymmetric Triathlon® X3® patella.\nThe patient was initially referred by the primary surgeon to our oncology clinic in February 2019 due to findings of a distal femur lesion in her right knee discovered on follow-up X-rays () and better seen in a noncontrast MRI. At that time, the patient was pain-free but reported feelings of instability. The physical exam showed a well-healed midline surgical incision for TKA without complication. No obvious instability with varus/varus testing or drawer testing was found.\nMRI with IV contrast was performed to further characterize this mass in the right distal femur and rule out a malignant lesion (). The MRI report showed “postsurgical changes of right TKA with findings typical for osteolysis secondary to particle disease and knee joint effusion with synovitis.” The orthopedic oncologist then referred the patient to our adult reconstruction clinic with very low suspicion for a neoplastic process.\nThe patient was assessed again in April 2019 and mentions occasional knee buckling but without pain. Her X-rays showed a focal well-circumscribed lucent lesion with thin geographic mineralized margin along the distal diametaphysis and deep to the cemented femoral component. There was thinning and weakening of the cortices in several areas. At that time, we recommended surgery due to the fracture risk. The patient declined but was agreeable to close surveillance. She was assessed two months later and did not want to consider surgery, again and refused X-rays due to absence of pain. She returned back a month later with mild knee pain and opted to proceed with a revision TKA for the diagnosis of massive femoral osteolysis and impending femoral fracture. The patient was later seen preoperatively, and the pain and X-rays remained stable ().\nWe utilized the prior incision and entered the join through a medial parapatellar arthrotomy. Clear synovial fluid was seen and sent for culture. The patella and tibia were mobilized and found to be well fixed, although the tibia had some osteolysis. The polyethylene was found to have minor oxidation, but no significant wear was found. We found no third bodies or other debris. We removed the insert and found no backside wear or any other explanation for this massive osteolysis. There was visual appearance of diffuse in vivo oxidation. Based on the extent of the osteolysis, we decided to revise the femur with a stem to bypass the defect. The femoral component was then removed with minimal bone loss. There were massive cystic changes with some debris that we sent for pathology (). After refreshing the cuts, we proceeded with reconstruction. The femoral reconstruction was performed using a size A Tritanium® metaphyseal cone and Triathlon® femoral component with a 15 mm × 100 mm cemented stem extension and a 16 mm posterior stabilized X3® insert.\nAll the cultures resulted negative. Microscopic evaluation () of the periprosthetic tissue revealed features typically observed when implant failure has taken place. Numerous multinucleated foreign body giant cells can be seen filled with debris from breakdown products. Some of the giant cells had empty spaces where engulfed methyl methacrylate cement was present but dissolved during tissue processing. Other foreign body giant cells contained shards of refractile debris and fragmented refractile particles. Macrophages also infiltrated the tissue and are filled with phagocytosed microparticles. Chronic inflammatory cells were present but there was no acute inflammation supporting that this was an aseptic process.\nAt one-year clinical follow-up, the patient was noted to be doing well with no changes in her radiographic examination (). Her incision healed uneventfully. The patient was pain free and ambulating without walking aids. At the last follow-up, her right knee range of motion was 0-130°. |
A 56-year-old man with a history of alcohol abuse presented at an outpatient clinic with a 1-month history of progressive cough, hemoptysis, and dyspnea on exertion. He had been diagnosed with type 2 diabetes and hypertension, 2 years ago and 3 months ago, respectively. He was a heavy alcoholic, with a drinking history of two bottles of Soju daily for more than thirty years. There were no abnormal findings on physical examination. Chest X-ray on admission showed increased haziness in the right middle and right lower lobes. Laboratory evaluation at presentation revealed; a serum white blood cell count of 8.51×103/µL, hemoglobin, 8.2 g/dL, and a platelet count of 292×103/µL. Because the patient had only respiratory symptoms, other specific lab data, such as amylase or lipase levels were not checked at that time. A computed tomography (CT) scan of chest was performed to evaluate hemoptysis and findings suggestive of mediastinitis with abscess and esophageal perforation were obtained. Increased opacity and a ground glass pattern were observed in the middle and the lower lobes of the right lung (). A sputum examination returned negative findings for acid fast bacillus (AFB) smear, sputum cytology, sputum gram staining and culture. Gastrofiberoscopy was performed to check for gastroesophageal perforation, and bronchofiberoscopy to check for the presence of an endobronchial lesion that might have caused hemoptysis, but no abnormalities were found in both. Furthermore, bronchial washing cytology showed no evidence of malignancy, and esophagography also presented no specific findings. In addition, endoscopic ultrasonography failed to depict any mediastinal lesion, and fine needle aspiration revealed only the presence of some red blood cells and inflammatory cells.\nAlthough there was no evidence of esophageal perforation, assuming that esophageal microperforation may attribute to vessel erosion with mediastinal abscess or mediastinitis, the patient was managed conservatively with intravenous antibiotics and hemostatic agents for fifteen days, and was discharged when his symptoms resolved after 20 days of medical treatment. However, two days after discharge, cough, hemoptysis, and dyspnea re-developed, and the patient was readmitted. On second admission, vital signs were stable (blood pressure 110/70 mm Hg, heart rate 78 per minute, respiratory rate 18 per minute, temperature 36.5℃, and oxygen saturation 96% in room air). Breathing sounds were decreased but resonance was elevated on the right lung field. His sputum was foamy and brown-colored, suggestive of hemoptysis, and simple chest radiography revealed pneumothorax of the right lung. Complete blood count findings were normal, except for a hemoglobin level of 9.5 g/dL; other blood test results were C-reactive protein 1.44 mg/dL and erythrocyte sedimentation rate 62 mm/hr.\nChest CT revealed air bubbles from the lower mediastinum to the upper pancreas and a large amount of pneumothorax on the right side (), and thus, a chest tube was inserted in the right thoracic cavity.\nConsidering chest CT findings, we assumed that there may be a pancreatic problem. Therefore, amylase and lipase levels were also checked in the serum and pleural fluid. Blood exam results were serum amylase 810 IU/L and lipase 657 IU/L. Pleural fluid analysis revealed bloody exudate with increased amylase and lipase levels (>15,000 IU/L and >3,000 IU/L, respectively) and with pleural fluid red blood cells 330,000/mm3, white blood cells 3,100/mm3 (polymorphonuclear neutrophil leukocytes 47%, lymphocyte 10%), protein 3.5 g/dL, adenosine deaminase 41.3 IU/L, and carcinoembryonic antigen 3.3 ng/mL.\nThen abdominal CT was performed for further evaluation, and showed a 2.0×1.4-cm-sized air-containing cavitary lesion in the pancreatic body ().\nAFB smear and culture findings and tuberculosis polymerase chain reaction findings of pleural fluid were nonspecific.\nThe patient was managed conservatively with parenteral nutrition, a hemostatic agent (tranexamic acid) and intravenous antibiotics, but no improvement was observed. ERCP performed on the fifteenth day of hospitalization, demonstrated main pancreatic duct disruption with dye leakage (), and an endosopic nasopancreatic drainage (ENPD) tube was then placed from the nose to the pancreatic duct. On the sixteenth hospital day, the amount of hemoptysis decreased, and on the eighteenth hospital day, frothy non-bloody sputum was observed. Later, ruptured pancreatic pseudocyst and the presence of pancreaticothoracic fistula were confirmed by MRCP (). Two weeks after ENPD insertion, second ERCP showed that partial disruption of main pancreatic duct and pancreatic juice leakage persisted.\nENPD removal was then performed and an endoscopic retrograde pancreatic drainage tube was inserted on hospital day twenty-nine. At this time serum amylase and lipase levels decreased to 258 IU/L and 178 IU/L, respectively. Finally, the patient was discharged in an asymptomatic state, excepting a cough and sputum production, after 34 days of inpatient management. Follow up ERCP was performed in an outpatient clinic at 38 days after discharge, and showed resolution of the main pancreatic duct disruption, and a pancreatic body with a main pancreatic duct benign stricture (). |
A 61-year-old Japanese woman presented with declining vision in her left eye. She was diagnosed with BRVO in her left eye and underwent photocoagulation of the retinal periphery five years before this event. She had no history of systemic disease except for hypertension, controlled by medications. She developed retinal schisis extending to the macula and was referred to Chiba University Hospital for treatment. Her visual acuity was 20/20 in the right eye and 20/50 in the left eye at the initial presentation. The intraocular pressure was 12 mmHg in the right eye and 11 mmHg in the left eye. The axial length was 21.53 and 21.75 mm in the right and left eyes, respectively. The retinal schisis was observed at the inferior temporal retina with white vessels. Optical coherence tomography (OCT) showed macular traction and schisis. The posterior vitreous detachment was incomplete. The fluorescein angiogram showed retinal neovascularization in the nonperfused retina (). She underwent phacovitrectomy with implantation of intraocular lens. During the surgery, the epiretinal membrane and internal limiting membrane were removed from the macula using Brilliant Blue G. The posterior hyaloid was separated except for the inferior temporal area where the vitreous is firmly attached to the retina. After the surgery, the decrease in the schisis cavity was observed with stable visual acuity between 20/50 and 20/40 (). Two and a half years after the initial surgery, she presented with macula-involving bullous retinal detachment (). Her visual acuity decreased to counting fingers. The outer retinal break was suspected posterior to the equator in the retina with BRVO. She underwent PPV with inner retinectomy at the BRVO area to reduce vitreous traction because the hyaloid was impossible to be separated from retina. The retinectomy encompassed the inferotemporal peripheral retina and extended posteriorly into the macula, almost to the fovea (); note that the inferotemporal arcade was truncated by the retinectomy. Subretinal fluid was drained through the outer retinal break, and the retina was attached with silicone oil tamponade. The retina remained attached after the removal of silicone oil and the addition of the 7 mm width encircling buckle to support the peripheral retina. Her visual acuity was 20/60, and intraocular pressure was 10 mmHg at one and a half years after the last surgery (). The defect in her visual field slightly enlarged after the inner retinectomy (). |
The patient under consideration was a 12-year-old boy who had been under the supervision of different ophthalmologists for 4 years. During this period, the patient had undergone various ophthalmologic and neurological examines. However, no noticeable organic reason was put forth for the disorder. Finally, with the possibility of a psychological reason, the patient was referred to a clinical psychologist. After the different psychological evaluations, he was considered to be a patient afflicted with functional neurological symptom disorder-conversion disorder by a clinical psychologist and a psychiatrist.\nThe examinee was the first child of the family. The parents of the child had divorced when he was three and half years old. The father had married again and during this period, the child had lived with a father. It seems that the problems of child vision problems had intensified after the divorce and, in particular, when the second wife of his father gave birth to a new child.\nThe patient underwent CAT treatment plan. Single-case design (A/B) was used to assess the effects of an intervention. Before and after intervention set of tools were done. The intervention includes twelve sessions of CAT and four sessions of follow-up. And during the treatment, intensity and weakness of conversion symptoms were observed. Also, the long-term effects of interventions during the treatment period were evaluated.\nZung's self-rating anxiety scale (SAS), beck depression inventory, second edition (BDI-II) and visual acuity test (VA) have been used for evaluation before and after the intervention. BDI-II used for measuring symptoms and intensity of depression which includes 21 items. Each item has a score between 0 and 3 and it is one of the valid depression measurement scales in a clinical trial.[] SAS, introduced by Zung, has been widely used in research and in clinical practice for the detection of anxiety.[] In this study, in order to evaluate the vision power of the examinee, the VA test was used by an ophthalmologist.\nThe cognitive-analytical intervention in psychogenic myopia had a significant effect on the increase of VA. Although, had a significant effect on the decrease of depression and anxiety. A raw score of depression based on BDI-II was 44 in the pretest and after 5-month follow-up it become 15. However row score of anxiety based on SAS was 47 in pretest and after 5-month follow-up it become 23, and so on VA increased as it comes in below table in both eyes [ and ]. Moreover, based on continues clinical interview and reports of family there was a significant improvement in complaints of the case. |
A 61-year-old African American male with past medical history of hypertension and schizophrenia presented to the emergency room following 2 episodes of syncope.\nHe reported 3 month history of progressive neck mass. Physical examination revealed a temperature of 37.7 degrees, blood pressure of 130/87 mmHg, pulse of 92 bpm, and respiratory rate of 17 bpm. There was extremely large left sided neck mass extending into the left axilla and multiple palpable left and right cervical lymph nodes. The lungs were clear to auscultation, and there was a 2/6 systolic ejection murmur heard best at the bases. The abdomen was soft and nontender, without palpable organomegaly. There was a 5x2 cm right gluteal non-tender, non-mobile mass with central ulceration and also a 2x2 cm spherical mass at the left upper back with small central ulceration. There was 2+ pitting edema of the lower extremities bilaterally. There was differential swelling of left upper extremity. Electrocardiogram showed normal sinus rhythm, left axis deviation, low voltage and mild t wave inversion in V2–V4 ().\nInitial CT scan of the abdomen and pelvis showed diffuse metastatic disease of the visualized lower chest, abdomen and pelvis including superficial soft tissues, left kidney, and probable right kidney. There was diffuse confluent adenopathy and massive right inguinal lymphadenopathy, along with pronounced diffuse anasarca. There are multiple renal masses. There are solid masses arising off of the lateral aspect of the left kidney involving the upper, middle and lower lobe (). The left kidney mass was described as an exophytic lesion. There was a mass along the anterior aspect of the heart that measures approximately 5 cm but incompletely visualized. CT head was negative.\nTransthoracic echocardiogram revealed a large mass measuring 4.8 cm x 3.0 cm extending from the apex to the mid RV cavity, and extending into the RV outflow tract stopping just short of the pulmonic valve (). There was deformation of the RV free wall suggesting invasion of the myocardial wall and a malignant growth. There was mild RV enlargement, with normal function of the segments not involved in mass. There RA was mildly dilated with no mass seen, and there was no evidence of thrombus in the IVC. There was a small pericardial effusion without echocardiographic evidence of tamponade. A dedicated CT of the thorax revealed a mildly enlarged heart, and a filling defect in the RV measuring 6 cm extending to the apex and suspicious for malignancy ().\nA core biopsy of the right superficial gluteal mass revealed a tumor arranged in solid nests having abundant eosinophilic cytoplasm, central nucleus and conspicuous nucleoli, with areas of coagulative tumor necrosis, and individual tumor cell exhibiting high-grade atypia with pleomorphism ().\nOn immunohistochemistry, the tumor cells expressed pacytokeratin, PAX 8, vimentin and CD 10 (weakly). The tumor cells are negative for S 100, Melan-A and RCC. Immunostains for CD 117 and CK 7 were performed, however were uninterpretable due to tissue depletion. Morphology and immunohistochemical profile favor a metastatic poorly differentiated carcinoma of likely renal origin, with a possibility of an unclassified RCC. Oncology was consulted and they suggested a diffuse metastatic malignancy of primary renal origin with poor prognosis. Oncology advised that the patient should follow up in outpatient cancer clinic. Due to extent and burden of metastasis, patient and family members agreed to conservative management and patient was placed in hospice and comfort care with no further aggressive management. He was discharged from the hospital to hospice and to follow-up with the cancer clinic, although there were no records that he kept the appointments and he was lost to follow-up. |
The 33-year-old Caucasian female was first admitted to our hospital in 2003. At that time, the patient had a history of two spontaneous abortions followed by amenorrhea and galactorrhea, and also a history of migratory arthralgia of the large joints. She suffered from nonspecific symptoms including lower back pain accompanied by mild fever. Despite a lack of neurological manifestations, the patient underwent several investigations including brain computerized tomography (CT) and MRI, which surprisingly showed a mass-like lesion in the right temporal lobe with cystic and infiltrative portions that partially enhanced following intravenous (IV) gadolinium administration. Additionally, a smaller lesion was detected in the hypothalamus ( and ). Glioma was suspected based on the imaging results, and stereotactic biopsy of the lesion was performed. Histological analyses revealed granular, foamy, periodic acid–Schiff (PAS)-positive macrophages with no signs of tumor. WD was suspected and then confirmed at a referential histopathological laboratory (Bonn, Germany) using PAS staining technique. For WD confirmation, the patient underwent a thorough gastroenterological evaluation including repeated duodenal biopsy; all biopsies were deep; histology was done with correct PAS staining and the specimens examined by experienced pathologists were found to be negative. The diagnosis of primary CNS WD was postulated and was supported by spinal tap, which showed polymerase chain reaction (PCR)-positive results for WD. Antibiotic treatment was initiated, first with IV ceftriaxone 2 g bid for 2 weeks followed by oral trimethoprim–sulfamethoxazole 160/800 mg bid. The fever subsided with treatment; however, the patient still suffered from lower back pain.\nIn 2005, the patient developed diplopia despite ongoing oral antibiotic treatment. Neurological investigation revealed vertical gaze paresis. Brain MRI was then performed, which showed partial regression of the temporal lesion, no significant change of the hypothalamic lesion, and two new lesions. One small lesion was situated in the right thalamus with discrete, central postgadolinium enhancement (). The second new lesion had an infiltrative appearance and surrounded the cerebral aqueduct in the mesencephalon; discrete dot-like enhancement was also apparent (). Antibiotic treatment remained unchanged and the patient’s symptoms improved after a few days.\nIn 2006, the patient suffered from recurrent diplopia and paresthesia of the right arm. Vertical gaze palsy again was observed, and additionally, paresis of the left abducens nerve was also detected. Brain MRI showed a new mass-like lesion in the brain stem with small ring-like, central, postgadolinium enhancement ( and ). The lesion previously observed in the right thalamus as well as hypothalamic infiltration had completely regressed. Remarkable regression of the initial mass-like lesion in the right temporal lobe was also noted. Spinal tap was again performed, and although the cerebrospinal fluid (CSF) was PCR-negative for WD, no other cause was found. Antibiotic treatment therefore remained unchanged (oral trimethoprim–sulfamethoxazole 160/800 mg bid).\nA few months later, the patient stopped visiting our outpatient clinic and we had no contact with her for 8 years. Her next visit took place in October 2014. At that time, she reported that she had stopped taking antibiotics. She denied any worsening of her neurological status in the previous period and was able to attend work as an office clerk. She still suffered from vertical gaze palsy and sixth cranial nerve paresis on the left side. MRI from November 2014 found no new lesions; the previous lesions had partially regressed. Resumption of antibiotic treatment was suggested but was refused by the patient.\nIn December 2014, the patient was found unconscious by her family. Upon admission to the hospital, she was severely dehydrated with hyperosmolarity, metabolic acidosis, and hypotension. After rehydration and correction of the acid–base dysbalance, she regained consciousness. Right-sided abducens paresis in addition to her previous ocular palsies and severe cognitive impairment was found. She suffered from short- and long-term memory loss and had retrograde amnesia spanning several months prior to the new episode. MRI showed diffuse brain atrophy and severe atrophy of the right hippocampus with hippocampal sclerosis (). All enhancing lesions on MRI had regressed, only postinflammatory gliosis in the right temporal lobe and a small periaqueductal pseudocyst were found. Oral antibiotic treatment with trimethoprim–sulfamethoxazole 160/800 mg bid was reestablished. Unfortunately, her neurological status thus far (April 2015) remains the same. The patient signed the informed consent and agreed with publishing medical data in scientific literature in anonymous form. This case report was approved by the ethics committee of Na Homolce Hospital, Prague, Czech Republic. |
A 28-year-old primigravida was referred to our hospital at 13 weeks of pregnancy. She had been diagnosed with Ebstein's anomaly and an associated atrial septal defect at 3 years of age. She had been asymptomatic without medication. At age 25, she underwent cardiac catheterization for hemodynamic assessment anticipating a subsequent pregnancy. It revealed that her left arterial oxygen saturation (SaO2) was 93%, with good left ventricular function (). Surgical intervention was recommended due to severe tricuspid regurgitation and a remarkably enlarged, atrialized right ventricle. She declined to undergo surgery and became pregnant at the age of 28. At the first medical examination conducted at our hospital, she had no respiratory failure and had good percutaneous oxygen saturation (SpO2) of 93%. A chest X-ray confirmed cardiomegaly with a cardiothoracic ratio (CTR) of 54% (), and echocardiography revealed a dilated right atrium, a septal leaflet displaced by 50 mm from the tricuspid annulus, moderate tricuspid regurgitation (Carpentier's type C), and an ostium secundum atrial septal defect (). Left ventricular systolic function was preserved, though the interventricular septum was displaced by the enlarged, atrialized right ventricle (). At 22 weeks' gestation, her SpO2 decreased to 89%, and oxygen was administered by nasal cannula for cyanosis along with anticoagulation therapy (heparin) to prevent occult embolism. She was admitted at 27 weeks' gestation with dyspnea on exertion. Echocardiography revealed worsening tricuspid regurgitation and right side volume overload, but left ventricular systolic function seemed to be maintained. Although fetal growth was appropriate, elective cesarean section was performed at 33 weeks' gestation due to her respiratory failure and progressive desaturation with worsening cardiomegaly (). Her baby, weighing 1943 g (appropriate for date), was delivered with respiratory distress syndrome and required surfactant treatment. Pathological examination of the placenta was appropriate for gestational age. Two months after delivery, the mother's cardiac condition worsened to the level of NYHA class IV. A chest radiograph confirmed progressive cardiomegaly with a CTR of 70% and decreased pulmonary blood flow (). Urgent cardiac catheterization revealed SaO2 was 77% even with O2 administration, and a right-to-left shunt of 39%. Left ventricular function was severely impaired (). She underwent surgical repair with tricuspid valve replacement using a bioprosthetic valve (Epic Stented Tissue Valve, St. Jude Medical Inc, St Paul, MN) and ASD closure. A pacemaker was implanted (Reply DR, Sorin, Saluggia, Italy) due to complete AV block. During the postoperative period, diuretics (furosemide and aldactone), an angiotensin converting enzyme inhibitor (imidapril) and a beta-blocker (bisoprolol) were initiated. She was discharged in good hemodynamic condition with an ejection fraction of 57%, and she has remained well with an improved of CTR of 48% (). |
A 50-year-old Thai male presented with three episodes of generalized seizures and right-sided hemiparesis for 6 h before arrival. He had no previous seizures. He had a history of well-controlled diabetes mellitus and hypertension for 20 years and took metformin 1000 mg/day and diltiazem 60 mg/day. His past medical history revealed progressive slowness in thinking and walking, memory impairment, sleep-wake disturbance and mood disorder, which had slowly progressed for the past 20 years; however, it had rapidly worsened during last year of his life. He was diagnosed with organic mood disorder 5 years before this presentation and treated with risperidone 0.5 mg/day, sertraline 50 mg/day, and trihexyphenidyl 1 mg/day. Even with this treatment, his symptoms had been progressively worsening for the past 1 year to the point that he could not perform daily living activities, such as taking the correct medications. He was the fifth of seven children. His sister had a history of unexplained hearing loss, cognitive decline, and slowness of movement starting at the age of 20. When she was 40 years old, she developed visual and auditory hallucinations as well as recurrent transient ischaemic attacks with full recovery. The patient’s father and mother died at the ages of 70 and 78, respectively, and had no history of cognitive impairment or stroke. A mental status examination showed a good level of consciousness; however, he was mute and slow to respond to commands. A motor examination showed right-sided weakness (grade 2/5 for arm and grade 0/5 for leg) and generalized hyperreflexia except for right leg hyporeflexia and no sensory impairment. There was also mild right facial weakness. A CT of the brain showed diffuse white matter abnormalities, old multiple lacunar infarctions in the bilateral basal ganglia, thalamus, and left pons. The initial diagnosis was acute ischaemic stroke with seizures. He was prescribed 300 mg/day of aspirin and usual stroke care. Phenytoin was prescribed for seizure control. An MRI of the brain was performed on day 12 after admission. The results showed acute infarction of the left pons (Fig. ), several old lacunar infarcts surrounded by minimal gliosis in the bilateral putamen and thalamus as well as few scattered small, old infarcts surrounded by minimal gliosis in the bilateral frontal-parietal periventricular white matter without anterior temporal lobe lesion (Fig. a, b, and c). Surprisingly, a large number of microbleeds were found throughout the brain (Fig. d, e and f). The total numbers of cerebral microbleeds was 214 and 136 in lobar areas according to the Microbleeds Anatomical Rating Scale (MARS) []. After a comprehensive review of vascular risk factors, his HbA1C was 5.5%, and his serum LDL was 79 mg/dL. His blood pressure was well-controlled. None of these risk factors explained his symptoms and MRI findings. He had a history of unexplained cognitive impairment and mood disorder. In addition, his sister had a history of cognitive decline, psychiatric symptoms and transient ischaemic attack; therefore, a genetic condition, such as CADASIL, was suspected and confirmed by molecular genetic testing, which revealed a homozygous known pathologic variant, c.1672C > T (p. Arg558Cys), in the NOTCH3 gene. His clinical symptoms deteriorated, and he died of tracheobronchitis with secretion obstruction. |
An 82-year-old man presented to the emergency with the chief complaints of progressively worsening upper abdominal pain for three days and multiple episodes of coffee-coloured vomiting for the past eight hours. The pain was constant in nature and no previous episodes were reported. He had 3-4 bouts of coffee-coloured vomiting, but bowel habits were otherwise normal. He gave no history of blood in stools or loose stools. There was no significant past medical history, nor did he have any recent trauma or surgery. On physical examination, the patient had tachycardia and hypotension. Reduced air entry was noted in the right lung base along with the presence of bowel sounds. On per abdomen examination, there was tenderness and guarding over the epigastric region, but no palpable lumps were found. The rectal examination was within normal limits. Blood investigations revealed a total leucocyte count of 14,500 and haemoglobin of 12% with a haematocrit of 40%. Liver enzymes and renal function tests were within normal limits. An arterial blood gas test revealed metabolic acidosis with serum lactate levels of 3.2 mmol/L. Other haematological investigations were normal. A posteroanterior chest radiograph showed the right dome of the diaphragm as being raised, with a non-homogenous opacity rising towards the right axilla (Figure ).\nComputed tomography (CT) of the chest and the abdomen was performed, with findings suggestive of diaphragmatic herniation of the omentum, pylorus, colon, and suspected duodenum into the thoracic cavity. The gastroesophageal junction was noted to be normal (Figure ).\nDue to his worsening clinical condition and the onset of peritonitis, the patient underwent an emergency exploratory laparotomy (Figure ).\nAt surgical exploration, a defect of size 6 x 5 cm was identified just behind xiphisternum through which part of the stomach, transverse colon, and omentum had herniated into mediastinum. After confirming viability by inspection, the contents were reduced back into abdomen. The rest of the gut, intra-abdominal structures, and organs were found to be normal. The diaphragmatic rent was repaired and plication of diaphragm done with non-absorbable suture. The post-operative course of the patient was uneventful, and the patient was discharged on post-operative Day 5. He has had complete resolution of symptoms and has had no recurrence. An upper gastrointestinal endoscopy done after three weeks was normal. |
HM is a 53-year-old African American male who presented to the emergency department with complaint of right-sided abdominal pain. The patient has a past medical history significant for hypertension controlled with clonidine and amlodipine, as well as polysubstance abuse. He denied alcohol abuse or any significant familial history of malignancy. The patient stated that he was in his normal state of health when he experienced rapid onset of intense abdominal pain and nausea which was worsened by eating, with no associated fevers, chills, vomiting, or diarrhea. However, patient did admit to intermittent heroin use and smoking crack cocaine on a daily basis.\nInitial laboratory analysis revealed that the patient had a serum lipase level greater than 2000 u/L, alkaline phosphatase level of 53 IU/L, AST level of 34, ALT level of 25, total bilirubin level of 1.9 mg/dL, hemoglobin level of 12.9, and creatinine level of 4.0 mg/dL. He was admitted to the medical intensive care unit (MICU) with the diagnosis of acute pancreatitis and acute renal failure.\nAn abdominal ultrasound revealed a normal sized common bile duct of 3.7 mm, without evidence of cholelithiasis or biliary sludge. A lipid panel was done and showed a serum total cholesterol and triglyceride level of 107 mg/dL and 122 mg/dL, respectively. The patient did not have any other risk factors for pancreatitis; therefore it was concluded that the likely etiology of the patient's pancreatitis was secondary to his crack-cocaine abuse.\nThe patient remained in the MICU hemodynamically stable and afebrile for four days until being transferred to the medical floor with a hemoglobin level of 9.5 gm/dL. One day following transfer the patient had a single large volume melenic bowel movement and was noted to be lethargic and in acute respiratory distress. He was subsequently transferred back to the intensive care unit and found to have a hemoglobin level of 4.8 gm/dL and diagnosed with hypoxic respiratory failure secondary to gastrointestinal bleeding and acute blood loss.\nEndoscopic evaluation via esophagogastroduodenoscopy (EGD) revealed several large ulcers of various sizes, ischemic in appearance throughout the stomach and duodenum. Given the fact that the patient did not have any risk factors for peptic ulcer disease and his history of cocaine abuse, it was thought that the patient's ulcers were likely ischemic in nature. At no time prior to the acute blood loss event secondary to gastric ulcer bleeding was the patient ever hypotensive or intubated. Nonetheless, a serum gastrin and H. pylori serology were sent and were negative. The patient was placed on intravenous pantoprazole drip, transfused, and remained in the ICU for further monitoring.\nHM was eventually discharged to out-patient followup with the gastroenterology team as well as drug counseling service. He returned in approximately one month, substance-free and devoid of any further complications; however, he failed to return for follow-up endoscopic exam. |
A forty-five year old Caucasian female was referred to the Royal National Orthopaedic Hospital, Stanmore, UK, due to an eight week history of lumbar back pain after lifting a bag onto an overhead luggage rack. The pain did not respond to analgesia or physiotherapy. During this time, the patient experienced no fevers, sweats or weight loss. The patient recalled no urinary symptoms, had never had any other back pain or injury, no epidural anaesthetic, no skin conditions, no sinusitis or otitis media, no gastrointestinal issues and no other significant co-morbidities, such as diabetes. The patient had undergone surgery as a teen to remove varicose veins in the leg due to Klippel–Trenaunay syndrome; a congenital vascular disorder where one limb may be affected by port wine staining, varicose veins or too much bone and soft tissue growth. She had received no antibiotics in recent memory. Some years previously, the patient had had a dental cavity filling and some subsequent dental hygienist procedures, but she had good oral health. She had a copper intrauterine device in situ since 2009.\nThe patient was born and raised in Australia, and moved to the United Kingdom twenty years previously, where she resided in London working as a consultant in finance. Recent travel history included visiting her father in hospital in 2014 in Manila in the Philippines. The patient was a smoker (fifteen cigarettes a day) and drank approximately fourteen units of alcohol per week. Other than the aforementioned, the patient was healthy and well.\nThe patient was referred to our specialist orthopaedic hospital based on abnormal outpatient imaging of the spine. The patient’s C-reactive protein (CRP) at the time of admission was <10 mg l−1 and her white cell count was within the normal range. T1-weighted sagittal magnetic resonance imaging (MRI) of the thoracolumbar spine showed reduced signal intensity of the T12 and L1 vertebral bodies that indicated marrow oedema, and destruction of the central and posterior aspect of the T12–L1 intervertebral disc and endplates (). Short T1 inversion recover (STIR) sagittal imaging also showed a small posterior extension of disc space fluid, elevating the posterior longitudinal ligament (). These images demonstrated discitis of the twelfth thoracic and first lumbar vertebrae. |
A 69-year-old male who had undergone left-sided hemi-hepatectomy for HCC was diagnosed with multifocal hepatic recurrence of the tumor 1 year later. His cardiovascular risk profile consisted of smoking (38 pack years), arterial hypertension, insulin-dependent diabetes, obesity, and dyslipoproteinemia. His medical history additionally comprised bilateral carotid artery disease and stenting of the right internal carotid artery, chronic renal impairment, and chronic obstructive lung disease. Chronic occlusion and good collateralization of his left anterior descending artery (LAD) had been described 5 years previously when he underwent PCI for myocardial infarction with stent implantation in the RCX.\nAfter recurrence of the HCC, he suffered a posterior NSTEMI and underwent stenting of a subtotal stenosis of the RCA. In addition to the chronically occluded LAD, coronary angiography now showed 80 and 90% stenosis of the distal RCX and a posterolateral branch, respectively, which were not addressed in the course of the intervention.\nIn the weeks after the coronary intervention, he suffered from increasing angina and developed ventricular arrhythmia. Echocardiography, MRI (Siemens Magnetom Prisma, 3 Tesla), and CT (256 Multisclice Philips ICT) showed a tissue mass of 72 × 45 × 56 mm infiltrating the myocardium of both ventricles and the interventricular septum (Fig. a, b). When his cardiac symptoms became so debilitating that they limited his quality of life more than the cancer and he expressed an urgent desire for treatment despite his dismal prognosis, he was referred to our department to evaluate surgical treatment options.\nAs the RCA lesion had already been addressed, the LAD had been occluded with good collateralization for at least 5 years, and we agreed with the cardiologist that it was highly unlikely that such severe symptoms were caused by the remaining RCX lesions alone, we assumed the metastatic tumor to contribute to the patient’s deterioration by stealing perfusion from, compressing, or infiltrating coronary vessels. The imaging performed by this date, however, had not shown large tumor vessels communicating with the coronary arteries so that these phenomena were supposed to be caused by a multitude of small tumor vessels rather than large communicating vessels that would be amenable to interventional treatment such as embolization.\nAssuming that a sufficient reduction of the tumor size would not only palliate the patient’s angina but also reduce the frequency and severity of his ventricular arrhythmia, and given both the surgical risk of an aggressive procedure (Euroscore 14.02 for a combined procedure including CABG) and his palliative situation and limited life expectancy, he was offered a restrictive surgical approach consisting of debulking of the metastatic tumor with an option for subsequent coronary intervention, should the surgical procedure not achieve sufficient palliation of his symptoms.\nAfter median sternotomy and opening of the pericardium, the apex of the heart appeared plump and enlarged with an even surface that showed no visible tumor growth. The large metastasis seen on MRI and CT was palpable on and infiltrating the apex. Cardiopulmonary bypass (CPB) was established in customary fashion because exposition of the apex was not possible without provoking severe arrhythmia and hemodynamic instability. Felt strips were placed along the edges of the palpable tumor mass using 12 pledgeted 4.0 prolene sutures (Fig. c) that were snugged down after application of French glue. Without entering the ventricular cavities, a portion of the tumor measuring 27 × 27 × 12 mm was removed in toto (Fig. d). Additionally, an approximately equal quantity of fragmented tumor tissue was removed so that only those sections of the tumor that had grown deep into the ventricular walls and septum were left. This resulted in considerable bleeding from a multitude of small vessels within the tumor. After careful hemostasis, the edges of the crater that was left within the area delimited by the felt strips were approximated by tying down two pledgeted 2.0 prolene sutures that were spanned across the crater through two of the felt strips. Following this, the remaining 4.0 sutures were tied down, and the defect was finally closed by a mattress suture followed by an over-and-over suture. The patient remained hemodynamically stable throughout the procedure, and weaning from CPB, decannulation, and sternal closure were performed in customary fashion.\nHistopathologic examination of the removed tissue showed a mostly solid tumor consisting of medium-sized to large cells with a wide eosinophilic cytoplasm surrounding enlarged vesicular nuclei with prominent eosinophilic nucleoli. Further findings consisted of atypical mitotic figures and local invasion of blood vessels. Immunohistochemical examination yielded a profile that was also compatible with a diagnosis of metastatic HCC (co-expression of Hep-Par1, positive for CK8 at least in some sections, cytoplasmatic positivity for TTF1, negative for CK7).\nThe patient was extubated 5 h after the procedure and discharged from the ICU on postoperative day 2. He took an uneventful further course and was discharged on postoperative day 9 with his symptoms palliated. One and a half months later, he presented for a follow-up examination, this time complaining not of cardiac symptoms but of headaches. MRI of the head showed no cerebral metastases. MRI of the heart showed a mass of felt strips and organized hematoma on the apex as well as the change in configuration of the apical region brought about by the surgical procedure. This made it difficult to assess the size of the tumor which appeared, however, to have regained much of its previous size. Nevertheless, the patient survived five and a half months after the procedure with his cardiac symptoms alleviated.\nThe patient provided written approval of the publication of his case including any pertinent images for scientific purposes. |
The patient was a 48-year-old Caucasian man, with a history of smoking, alcoholism, tracheostomy, diagnosed with laryngeal moderately differentiated squamous cell carcinoma, and had not previously received tumor treatment. In November 2016, the patient received high-dose cisplatin monochemotherapy (100 mg/m2 every 21 days) along with 70 Gy of radiotherapy into 35 sessions in the head and neck region, as a therapeutic schedule. The only comorbidity was gastroesophageal reflux, and home therapy consisted of omeprazole 20 mg and dexamethasone 4 mg daily, and acetaminophen 500 mg if necessary for pain. On the day of cisplatin administration (total dose of 178 mg), the patient received vigorous hydration (3 L of saline solution; 0.9%), diuretics (125 mL of mannitol; 20%), electrolytes (20 mL of potassium chloride, 19.1%; and 10 mL of magnesium sulphate), and prophylaxis for acute emesis (20 mg of dexamethasone plus 24 mg of ondansetron). From days 2–6 following the first infusion the patient received 10 mg metoclopramide and 100 mg dimenhydrinate every 6 h, and 8 mg ondansetron every 12 h orally to prevent nausea and vomiting. Nevertheless, the patient had nausea during the five days following chemotherapy, decreasing his food intake. He did not have diarrhea, vomiting, or fever. Furthermore, the patient did not report any previous episodes of allergy or anaphylaxis.\nAt 12 days following the first cisplatin infusion and after 8 sessions of radiotherapy (total of 16 Gy), the patient presented with acute onset of palpable purpura in the lower limbs (Fig. ). On the 13th day following chemotherapy, the patient was hospitalized with suspected meningococcemia and began an antibiotic therapy with intravenous 1000 mg vancomycin every 12 h and 2000 mg cefepime every 8 h. On the 14th day after chemotherapy, the patient underwent a cranial computed tomography scan and cerebrospinal fluid (CSF) analysis that refuted the meningococcemia hypothesis, thus the antibiotic therapy was discontinued. The CSF analysis included adenosine deaminase activity, bacterioscopy, biochemistry, cytology, culture, gram bacterioscopy, IgG dosage, and screening for antitoxoplasma antibodies, mycobacteria, antineoplastic cells, fungi, and anti-cardiolipin antibody. All of the tests had normal or negative results. At the 14th day following the first dose of chemotherapy, a skin biopsy was performed, which revealed neutrophilic exudates coating the walls of the vessels of the superficial dermis, with marked apoptosis of inflammatory cells (leukocytoclasia). There was spilling of red blood cells and degenerative changes in the dermal collagen. The diagnosis of leukocytoclastic vasculitis was therefore performed (Fig. ). The direct immunofluorescence test revealed mild and granular deposits of IgM and C3 on the wall of some upper dermis vessels. Moreover, blood tests were negative for cryoglobulin, anti-neutrophil cytoplasmic antibody (ANCA), antinuclear antibodies (ANA), hepatitis B and C, and for human immunodeficiency virus.\nAt the 15th day following chemotherapy, a blood test showed marked leukopenia (Table ); therefore, the patient received 300 mg subcutaneous filgrastim, blood was taken for culture and he restarted prophylactic antibiotic therapy at reduced doses (500 mg vancomycin and 1000 mg cefepime). He also began a regimen of 40 mg daily subcutaneous enoxaparin. At the 16th day following chemotherapy initiation, the patient underwent a urine test, and therapy with 1 mg/g topical triamcinolone daily was initiated owing to radiodermatitis, and therapy with 4 mg oral dexamethasone daily was restarted. At the 18th day, a therapy with 20 mg oral omeprazole daily was also restarted. At the 19th day, the patient received 5 mg/mL (20 mL) fenoterol inhalation therapy every 6 h, and the antibiotic regimen was altered to 500 mg ciprofloxacin every 12 h and 500 mg amoxicillin + 125 mg clavulanate orally every 8 h. Another urine test was performed on the 20th day, and a 24-h urine collection was performed on the 21th day owing to worsening of renal function, as evidenced by increased serum creatinine and blood urea nitrogen (BUN) (Table ). Discharge from the hospital occurred at the 23th day, once the purpura lesion had recovered and the patient’s renal function started to improve. Figure shows the sequence of pharmacotherapy and laboratory tests.\nAll the results of blood tests done prior to (basal) and following cisplatin administration are described in Table (hematological tests) and Table (biochemical and other blood tests). We observed that the majority of hematological parameters (hemoglobin, leukocytes, neutrophils, lymphocytes, monocytes, and platelets) decreased after cisplatin chemotherapy and during the hospitalization, and began to increase on day 16 and 17, due to filgrastim administration. Eosinophils and basophils increased after cisplatin infusion; this was observed even on the blood test performed prior to the appearance of purpura lesion (on day 7). Regarding the renal parameters, creatinine and BUN increased primarily on day 16 (3 days after hospitalization) probably due to vancomycin administration, since these parameters began to decrease after the change to the antibiotic regimen used. The hepatic parameters were slightly altered after hospitalization only in relation to albumin and total proteins. The international normalized ratio parameter, a global standard for prothrombin time, was within the reference values. The erythrocyte sedimentation rate (ESR) and c-reactive protein (CRP) levels were altered in the test performed 14 days after cisplatin administration (2 days after appearance of the first purpura lesion). The rheumatoid factor showed no marked changes. The levels of complement components C3 and C4 were below the reference value in the test performed on day 14.\nDuring the hospitalization, the general condition of the patient was good, with a fever only present on days 15 and 16, ranging from 100.22 °F to 100.76 °F, and no presence of arthralgia on any day. The palpable purpura occurred only in the lower limbs, and did not spread to any other site.\nTwenty-five days after cisplatin administration and 2 days after discharge from hospital, the chemotherapy regimen was changed to carboplatin AUC 5 (total dose of 375 mg) owing to impaired renal function and the possibility of pharmacodermia (leukocytoclastic vasculitis due to cisplatin administration). After carboplatin administration, the patient had no more episodes of purpura. |
This is a 26-year-old man who experienced right lower limb weakness for 2 years and the weakness exacerbated in last half year. He visited the second affiliate hospital of the Zhejiang University School of Medicine. A physical examination indicated the result of the straight leg raising test was positive and also muscle atrophy. The muscle power of the right lower limb had decreased to grade 3. There was no sensory impairment. Magnetic resonance imaging revealed an intraspinal extradural tumor over T10 to L3 (Fig. ). It appeared to be a spinal extradural arachnoid cyst (SEAC). To confirm whether the fistula existed between the subarachnoid space and arachnoid cyst, and to localize the position of the fistula, we performed a real-time technique. First, we injected contrast medium into the cyst under fluoroscopy. After 1 h the follow-up computed tomography (CT) was administered, and it revealed there was no contrast-infiltration into the subarachnoid space (Fig. ). We then extracted about 20 mL of fluid from the cyst. The follow-up magnetic resonance imaging on the same day indicated the cyst did not become smaller (Fig. ). A “one-way valve” fistula was suspected such that cerebral spinal fluid could pass into the cystic space from the subarachnoid space but could not flow in the opposite manner. Therefore we designed a procedure to localize the fistula. We penetrated two needles into the cyst and subarachnoid space separately in the L3/L4 level under digital subtraction angiography (Fig. ). Pending the fluid drained through those two needles, we injected 10 mL of contrast medium slowly into the subarachnoid space and a little contrast medium infiltrated into the cystic space horizontally at the T12/L1 level. We then administered high resolution computed tomography (HRCT) immediately to confirm the position in the axial plane. The HRCT revealed a funnel-shaped enhancement between the lower edges of the T12 body and the left nerve root, and this is the accurate position of the fistula (Fig. ).\nAfter general anesthesia and endotracheal intubation, the patient was placed in prone position. A fluoroscopy was used to determine the correct operative level, and a longitudinal incision was made 5 cm off midline at the T12 level. Muscle was dissected layer by layer, and a spinal process and left lamina were exposed. The left lamina was partially ground by cutting burr and then removed along with ligamentum flavum by Kerrison punch. After that, the cyst was exposed. Under a microscope, the cystic wall was fenestrated and it then drained off clear cystic fluid (cerebral spinal fluid). After partial excision of the cyst and evacuation of cystic fluid, a spinal endoscope (SPINENDOS, Germany) was maneuvered into the space and the fistula was detected (Figs. , ). The fistula was detached from the arachnoid membrane and was ligated with a 7–0 Vicryl purse string suture. Regional leakage from the repaired site was noted. An anastoclip was then used to close the fistula (Fig. ). Pulmonary pressure was elevated by ventilator (valsalva maneuver) to check the leakage and there was no more leakage. Hemostatic matrix and gel [Fibrin sealant kit (human), (Shanghai RAAS Blood Products Co, Ltd., Shanghai, China)] were utilized at the local region. The postoperative diagnosis was a thoracolumbar extradural arachnoid cyst. The pathological report revealed an arachnoid cyst (Fig. ). The symptoms improved on postoperative day 2. His lower limbs regained strength with limited dorsiflexion of the right foot.\nThrough the 3-month outpatient department follow-up, there was no more numbness or weakness of his right lower limb. The motion of dorsiflexion also improved. The 3-month follow-up magnetic resonance imaging revealed no recurrence of the previous lesion and no spinal cord compression (Fig. ). |
A 44-year-old male patient reported to the Department Of Prosthodontics with a complaint of severe sensitivity pertaining to his posterior teeth and generalized discoloration of his teeth. The patient's history did not reveal any habits pertaining to his complaints, but his spouse reported about night grinding habits. Extraorally no loss of vertical dimension was noted [ and ]. On intraoral examination, severe attrition of posterior teeth was noted. Mild attrition was noted in the anterior teeth [Figure , and ]. This case was diagnosed according to Turner and Misserlian classification[] as category 2. The patient was referred to the Department Of Endodontics for opinion on sensitivity and discoloration. As per patient's request, a more conservative method of management was preferred initially. Hence, root canal for the sensitive teeth and bleaching of all the anterior teeth was done to improve esthetics. The patient reported with a severe sensitive condition in his posterior teeth 3 months later and was dissatisfied with the bleaching. By then, the rate of attrition had not changed as measured at 36 and 13. Root canal for the posterior teeth and subsequent crowns were thought to be a more appropriate treatment plan. The loss of vertical dimension was assessed. Although there was no clinical evidence of volume of distribution (VD) loss, the patient had a freeway space of 4 mm but restoring posterior teeth alone would have resulted in a more esthetic anterior open bite. Hence, it was decided to prepare anterior teeth as well. Face bow transfer was done, and the diagnostic casts were articulated. Then a diagnostic wax up was done for provisional restoration at existing vertical dimension [Figure -]. Tooth preparation was done, retraction cords were placed and an optical impression was made with Cerec 3 system (sirona dental systems) [Figure and ]. Centric relation was recorded using extra oral tracing. Zirconia copings were fabricated with CAD/CAM designing and milling and tried in the patient's mouth [Figure and ]. After verifying the fit of the copings, full tooth morphology was created using D’sign ceramic material (Ivoclar-Viva). Apreglaze verification was done to evaluate esthetics and occlusion. Final glazing was done and the crowns were cemented with a Rely-X 3M resin cement [Figure and ]. A soft splint was advised postinsertion as a precautionary measure and the patient followed up for 1 year. At 1-year posttreatment, the patient had no complaints of sensitivity and was satisfied with the esthetic crowns |
A 61-year-old male who presented with a 1-year history of recurrent chest discomfort and palpitation was referred to our hospital. Examinations found no positive results except that Holter electrocardiography monitor revealed frequent ventricular premature contractions. He underwent the RFCA procedure since pharmacologic treatment did not show any significant improvement. After RFCA, the patient suddenly experienced dizzy, tight in the chest with cold sweat, bradycardia, and hypotension (89/50 mm Hg). Since chest X ray showed no sign of hydropericardium, vagal reflex was considered first. There was no improvement of blood pressure after rapid fluid infusion, and 2 mg atropine was administered intravenously. Fluoroscopy again showed increased heart shadow, and transthoracic echocardiography revealed hydropericardium. Protamine was administrated intravenously to neutralize heparin. The patient then underwent pericardiocentesis drainage immediately, and 800 mL blood was drained off. Afterward, the chest tightness got better and the blood pressure increased (120/75 mm Hg). Since the patient remained hemodynamically stable for 40 minutes in the catheterization room, he was transferred to the ward and observed carefully. Two hours later, he felt tight in the chest again, blood pressure 95/70 mm Hg, heart rate 85 bpm, and auscultation of the heart found a far low blunt sound. We aspirated the indwelling catheter in the pericardium again and drained off 750 mL blood. Volume expansion and dopamine were administrated to sustain the blood pressure in normal. However, the patient had circulatory unstability again 1 hour later. The transthoracic echocardiography showed a left ventricular wall rupture and a hematoma in the left ventricular wall, which was smaller during systole period due to the contraction of the myocardium (Fig. ).\nThe patient was transferred to the operating room for surgical exploration quickly due to active bleeding of the heart. Median sternotomy was performed. The pericardium cavity was filled with a large amount of blood and clot. After evacuating the pericardium cavity carefully, fresh blood was noted to be coming out of the left side of the heart, and the motion of the left ventricular free wall was hypokinetic. CPB was established with cannulation of the ascending aorta and right atrium quickly. After the cardioplegic arrest, further exploration of the heart revealed a 6 × 8 cm2 area of contusion in the posterolateral wall. A 5-cm tear of the epicardium was found at the center of the contusion near the apex, and 1 cm of the tear penetrated into the whole wall. Teflon-buttressed sutures were used to close the tear. Furthermore, a pericardium patch of sufficient size was applied to the contusion region using continuous sutures around and bioglue inside (Fig. ). The patient was discontinued from CPB uneventfully with excellent hemodynamic status. Postoperatively, he recovered well without any complication and was discharged from the hospital on the 15th postoperative day. A month after discharge, cardiac magnetic resonance showed that the injury region of the left ventricular wall had healed (Fig. ).\nThe case study was approved by the Ethic Committee of Shanghai East Hospital, Tongji University School of Medicine, Shanghai, China. Informed consent was obtained from the patient before data collection. |
A 19-year-old young female presented with a painless, palpable bony swelling over her lower back region (Figure ). It was progressively increasing in size for the last 4 years and had grown to such an extent that she was uncomfortable in lying down on her back for the last 4 months. On examination, a hard non-tender globular mass of size 8 x 5 x 5 cm was palpable in the midline of the S2-3 region, more towards left, with a smooth margin, and the overlying skin was non-adherent. The overlying skin was thick and showed black discoloration. There was no neural deficit, and the bowel/bladder functions were normal.\nRadiograph of the lumbosacral spine showed a bony outgrowth in the posterior aspect of the S2-3 region (Figure ). On skeletal survey, no similar lesion was found in any other part. Computed tomographic (CT) scan was performed to delineate bony details (Figures and 4). MRI showed a bony outgrowth of size 81 x 51 x 54 mm arising from posterior elements of the S2-3 region in the midline (more towards left side). It was well circumscribed by soft tissue envelope. The lesion showed a hypointense rim with a heterogeneously hyperintense center on T1- and T2-weighted images, indicating marrow suggestive of osteochondroma (Figure ). After a detailed discussion with the patient and her relatives, excisional biopsy was planned for local tumor control as well as for diagnostic purpose.\nThe tumor was approached through a posterior midline incision. The whole tumor was exposed, and en bloc excision was performed from the base of the tumor along with the cartilaginous cap from the conjoint lamina of the S2-3 vertebra keeping a healthy margin (Figure ). Care was taken in the whole procedure to prevent damage to the neurovascular structures. The wound was closed in layers with a negative suction drain. Postoperative radiograph showed complete excision of the bony mass, and the patient got relief from the pressure symptoms.\nHistopathology report showed a benign tumor with mature bone trabeculae and endochondral ossification with a cap of hyaline cartilage, confirming the diagnosis of osteochondroma (Figure ). On follow-up at 6 weeks, 3 months, 6 months, 12 months, and 24 months, there was no clinical and radiological recurrence (Figure ) of the disease, and no neurological deficit was detected. |
A 75-year-old male with no significant medical history, presented to the emergency department with right upper jaw pain after he had a tooth extraction a week before his presentation. After the tooth extraction, the patient was complaining of worsening pain in the upper jaw, and he started experiencing fever and chills as well. On review of systems, the patient endorsed night sweats and weight loss for the last two months. On physical examination, he was febrile with a temperature of 103 F, the rest of his vital signs were stable. Computed tomography (CT) scan of the face showed concerns of right maxillary osteomyelitis with soft tissue swelling (Figure ). The patient was admitted for further workup and intravenous antibiotics.\nOromaxillofacial surgery evaluated the patient and took him to the operative room, where he underwent irrigation and debridement, a bone biopsy of the maxilla, and extraction of multiple teeth. Infectious disease team recommended starting the patient on intravenous ampicillin-sulbactam while waiting for the culture results. His bone culture grew pan-sensitive Raoultella planticola. Based on the above findings, the teams put a plan to discharge the patient on levofloxacin and metronidazole to finish six weeks of antibiotics.\nSurprisingly, his bone biopsy revealed an unexpected finding, as he was found to have diffuse large B-cell lymphoma affecting the same site of the osteomyelitis. Magnetic resonance imaging (MRI) of the face revealed a large infiltrative soft tissue lesion with extensive surrounding enhancement involving the right side of the face, especially the maxilla, and it was extending intracranially (Figure ). The patient underwent staging imaging, and he was found to have stage IV DLBCL with metastasis to the liver (Figure ). The oncology team evaluated the patient, and they recommended to start chemotherapy. Eventually, the patient was started on rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) chemotherapy. The patient completed a total of six cycles with minor complications secondary to chemotherapy such as nausea, vomiting, or anemia requiring transfusions. Fortunately, he had an excellent response to chemotherapy, and he continued to follow up with the oncology clinic. |
A 35-year-old African American man was initially sent to our emergency room to evaluate a possible head trauma after a witnessed fall. He complained of frequent falls and leg weakness for 3–4 months. The weakness appeared to be progressive and persistent without episodic worsening. He had to use a cane to compensate but was still able to ambulate. His family members also endorsed cognitive defects over the past few months before admission. These impairments consisted mainly of apathy and withdrawal from social interactions; he used to be fully independent but now stayed at home most of the time. His medical history was only significant for alcohol abuse (three to four cans of beer and red wine daily for 2 years), and his family history was unremarkable.\nPhysical examinations showed an age-appropriate, alert, and oriented man. He was able to engage in conversations with a paucity of speech and flat affect; he also made eye contact and followed all commands during our evaluations.\nThere was bilateral weakness with spasticity in all lower extremity muscle groups. The deep tendon reflex was hypoactive with an upward response of the plantar reflex on the left side. He also had a wide-based unsteady gait and poor limb coordination. The remainder of the neurological examination and a general examination were unremarkable.\nInitial brain computed tomography (CT) in our emergency room demonstrated a mass lesion occupying the left caudate and extending into his frontal lobe (Fig. ). A brain MRI showed bilateral and symmetric hyperintense signals in the corpus callosum, periventricular white matter, and internal capsule (Fig. ) with an axial fluid-attenuated inversion recovery (FLAIR) sequence with gadolinium enhancement (Fig. ). A cervical spine MRI was negative for cord compression and abnormal signals.\nRoutine blood tests, as well as human immunodeficiency virus (HIV), syphilis, and thyroid function tests, were unremarkable, and his serum vitamin B12 level was borderline (289 picogram per milliliter). A cerebral spinal fluid sample revealed a mildly elevated protein level (120 mg per deciliter) with a normal cell count (two cells per milliliter) and glucose level (79 mg per deciliter). The sample was negative for cytology, oligoclonal bands, culture, and Epstein–Barr virus DNA polymerase chain reaction (PCR). His urine toxicology panel was negative for common recreational drugs.\nThe initial impression was an acquired demyelinating white matter disease due to nutritional deficiency. A trial vitamin supplement (500 mg administered intravenously three times a day and 1000 μg of cyanocobalamin administered intramuscularly daily) was given based on his alcohol abuse history, but no symptom improvements were seen during the following week. An inherited condition was thus suspected given the negative workup, the non-response to treatment, and the symmetric involvement in the brain MRI. This suspicion was validated by his peroxisomal fatty acid profile (Table ), and a diagnosis of X-ALD was confirmed according to the identification of a pathogenic mutation in the ABCD1 gene: c.1489 2A>G (g.153005544). Adrenocortical insufficiency was not identified during the evaluation. No seizure activity was witnessed or shown on video-electroencephalography.\nHis neurological condition deteriorated rapidly. He progressed into a non-verbal, quadriplegic patient dependent on a ventilator within 5 months of the initial encounter, and after 1 year of follow up he had degenerated into a vegetative state. Genetic counseling was offered to his family, and all of his siblings were negative for the ABCD1 gene mutation. |
A 17-year-old male was referred to the emergency department of our institute due to multiple traumas following a car accident. At the time of admission, with Glasgow Coma Score of 9, the patient was sleepy and had mismatched pupil sizes. Physical examination revealed that intense pain, gross swelling, and considerable deformity in the 4th finger (ring finger) of the right hand in addition to multiple scalp lacerations. Then, a comprehensive emergency imaging was rendered to the patient, including cranial, thorax, and abdomen. Furthermore, extensive radiographs of the right hand were obtained ( and ). A temporary casting was immediately employed to immobilize the fracture-dislocation. Our first decision tomanage patient’s orthopedic issue was that closed reduction and percutaneous pinning for either lesion after the neurosurgery. After a complete emergency evaluation, subdural hematoma was displayed on the cranial computed tomography (CT) in addition to massive alveolar hemorrhage on the thorax CT. With the exception of above findings, no additional pathologies were recognized. With the diagnosis of subdural hematoma, the patient was promptly taken to operating theater by neurosurgeons, to provide decompression. After the operation, due to the lung and hand pathologies, no neurosurgeons and traumatologists allowed surgical intervention for the hand fracture-dislocation. Therefore, our decision converted from surgery to non-surgicaltreatment. At the intensive care unit, closed reduction and short arm splint in the intrinsic plus position was applied with a single attempt. The reduction was confirmed through the control plain radiography. The patient was followed up with 2 daily clinic visits for 3 weeks at the intensive care unit. At the 4th week of the follow-up, the X-ray displayed a complete union in CMC fracture dislocation ( and ). For that reason, the splint was removed. A satisfactory range of motion (ROM) was observed at the time of the last follow-up. There was no limitation of movement, and full ROM was accomplished1 year postoperatively, DASH score was evaluated as 13.5 (, , ). |
The 83-year-old patient presented herself at a neurological emergency department with bilateral blindness and a history of severe headache, which she described the day before. An urgent computed tomography (CT) scan of the brain had been arranged, and a massive pituitary adenoma with hemorrhage was observed, and admission to the neurosurgical department immediately followed. When arriving at the hospital the hemodynamic parameters were stable (BP 130/90, 65 BPM); Glasgow coma score was 14/15. Her medical history included chronic renal failure (III°) as well as cardiovascular disease with hypertension, chronic atrial fibrillation, coronary heart disease with percutaneous transluminal coronary angioplasty, and stent. Therefore, dual oral anticoagulation with clopidogrel and acetylsalicylic acid was administered. Multiple electrode aggregometry did not detect effects of both anticoagulants. The neuro-ophthalmic examination revealed no perception of light in both eyes. Fundoscopy was normal without pallor of the optic nerves, no meningeal signs, and no overt clinical signs of hormone imbalance, but the measured pituitary hormone profile had a slight elevated prolactin level and suppression of sex hormones and thyroid profile (Table ). The blood tests revealed normal electrolytes and infection parameters. A magnetic resonance imaging (MRI) demonstrated a large 22 mm x 18 mm x 15 mm enhancing hemorrhagic pituitary macroadenoma with suprasellar extension and compression of the optic chiasm (Figure ). Emergency surgery was indicated. A transsphenoidal approach was carried out under general anesthesia. The pituitary tumor was identified, though it was not characteristic of a hemorrhagic pituitary macroadenoma. The tumor was tough and yellow colored, and debulking with a sharp curettage was difficult. Acute hemorrhage ceased after resection of tumor. Dopamine agonist was not used due to the patient experiencing complete blindness. After decompression, the surgery was concluded. After a period of cardio-pulmonary stability in the ICU, the patient was extubated. In this case, the transsphenoidal decompression of the optic chiasm provided an improvement in both eyes. Her visual disturbance improved with visual acuity from 0 to 5/100 for both eyes postoperatively. Counting fingers, identifying persons, and color vision were possible. On the first postoperative day, she was anxious and complained of gradual increase in shortness of breath. A non-ST elevation myocardial infarction was diagnosed. Additionally, to that, she developed an acute renal failure requiring temporary hemodialysis. After a short period, kidney function recovered, and cardiovascular stability was observed. The pathological examination confirmed a pituitary adenoma, most of it undergoing infarction. |
A 23-year-old Caucasian male with a history of illicit substance use self-presented to the emergency department (ED) of a community hospital with somatic complaints of feeling “the cells of his brain disappearing” and abdominal pain due to “feeling his gall bladder disappear.” He had visited the ED three additional times the same month for various complaints. He reported suicidal ideations with plans to overdose during this ED visit. While in the ED, the patient reportedly drank hand sanitizer. Nevertheless, the patient sought assistance with detoxification from alcohol and substance use. His reported alcohol consumption recently increased to include drinks one to five beers every day. He also began using phenibut, purchased online, in the past month. He stated that he would consume four to five grams per day. Upon attempting to stop consumption of these substances on his own, he reported experiencing symptoms of withdrawal, such as cold sweats, anxiety, difficulty sleeping, and visual and auditory hallucinations.\nIn addition, the patient reported recent symptoms of depression (sad mood, low energy level, anhedonia, feelings of worthlessness and helplessness), poor sleep, poor concentration, poor appetite, and weight loss. He admitted currently experiencing anxiety with ruminative worry, restlessness, racing thoughts, and panic attacks. His history is significant for recurrent suicidal thoughts since age 10 and a suicide attempt in which he stabbed himself several times in the neck with scissors while intoxicated during the previous year of this ED visit where he was hospitalized for a week following the episode. His history is also positive for a hospitalization and admissions to mental health treatment programs. His social history indicated a history of physical abuse by his stepfather at a young age and aggressive behavior.\nThe history was negative for psychotic and manic symptoms although a long pattern of illicit drug use remains a confounding factor in this interpretation. The patient admitted to experimentation with numerous drugs since age 13 including alcohol, cocaine, lysergic acid diethylamide (LSD), dimethyltryptamine (DMT), mushrooms (psilocybin), ketamine, ecstasy, opioids, inhalants, benzodiazepines, synthetic marijuana, amphetamines, and crystalline methamphetamine. In one instance, he had reportedly set himself on fire while under the influence.\nUpon admission to the ED, the patient’s vitals and laboratory results were obtained. His vital signs, complete blood count, and basic metabolic panel were all within normal limits. The urine drug screen showed straw and clear-colored urine and was positive for cannabis. His salicylate level was less than 0.3 mg/dL, acetaminophen level was less than 5 mg/dL, and blood alcohol level was less than 10 mg/dL.\nThe results of administration of a mental status exam revealed cooperation during the interview; regular psychomotor activity; normal speech rate and volume; and logical, organized, and goal-directed thinking. He was alert and oriented to time, place, and person. The patient’s mood was reported to be depressed. His affect was anxious and labile, and new involuntary movements were noted. There were no suicidal or homicidal thoughts, plans, or intent at the time. He denied currently having auditory or visual hallucinations and reality testing was intact, but admitted to occasional auditory hallucinations where he hears ambulance sirens. There was no thought withdrawal or thought broadcasting. The patient’s intelligence was estimated as average and fund of knowledge appeared to be adequate. His insight and judgement were deemed to be limited with poor impulse control. His language was fluent. The patient was able to move all limbs with normal muscle tone and strength. MRI revealed no structural abnormalities that would result in abnormal behavior (Figure ).\nThe patient was admitted to the Behavioral Health Unit of the hospital and the Clinical Institute Withdrawal Assessment protocol was utilized. Benzodiazepines were given for withdrawal symptoms of alcohol and phenibut. The patient was given Wellbutrin, 150 mg, by mouth daily for his depression symptoms and continued on Seroquel, 50 mg, at bedtime for further mood stabilization. Further, he was started on gabapentin, 100 mg, by mouth three times a day for impulsivity, anxiety, and protracted withdrawal symptoms. |
A 23-year-old Croatian man without any significant past medical history was admitted to our intensive care unit (ICU) due to a parapharyngeal abscess on the left side of the neck with spreading of infection in the mediastinum and the left side of the thorax. Urgent surgery was performed with incision of the parapharyngeal abscess, neck dissection, and left side thoracotomy with incision and drainage of the mediastinum and thorax. In the postoperative period in the ICU, the patient was sedated and mechanically ventilated with antibiotic therapy according to microbiological findings (blood, urine, bronchoalveolar lavage, and tissue sample taken during surgery). After the first surgery, Streptococcus mitis was isolated from the parapharyngeal abscessus. In the second surgery, Staphylococcus sp. was isolated from the mediastinal and neck swab wounds. From the beginning (upon admission to the ICU), the patient was treated with meropenem and linezolid. During the second week of treatment, he started to develop a nosocomial infection of the lung caused by multiresistant Pseudomonas aeruginosa, which was treated with ceftolozane/tazobactam 3 × 3 g intravenously. In the next 10 days, control computed tomographic (CT) scans of the neck and chest showed progression of mediastinal infiltrates and the formation of organized pleural effusion on the left side that required additional surgery. Due to atelectasis of the left lung that persisted during the second week of treatment in the ICU, bronchoscopy with bronchoaspiration and recruitment maneuvers were performed several times. Residual pleural effusions were drained several times before and after weaning procedures with a small-bore pleural catheter and Seldinger technique. On the 30th day of the patient’s illness, sedation was stopped, and the patient awoke promptly. He was hemodynamically stable and ready for weaning, which was successfully done in the next 24 hours. When the patient was extubated and started to breathe spontaneously, we enhanced his active physical therapy in combination with a cough assist device (CoughAssist E70; Philips Respironics, Hamburg, Germany). After the weaning procedure, the patient was fully conscious and hemodynamically stabile with blood pressure 115/70 mmHg, heart rate of 86 beats/minute, and body temperature of 36.7 °C. The patient was spontaneously breathing with oxygen saturation (SpO2) of 94%, fraction of inspired oxygen (FiO2) of 50%, partial pressure of oxygen (PaO2) of 9.19 kPa, carbon dioxide pressure (pCO2) of 5.7 kPa, and PaO2/FiO2 ratio of 138, but almost no rising of the left side of the thorax and no breath sounds on the same side.\nWe performed lung ultrasound, which showed atelectasis of the major part of the left lower lobe and the posterior part of the upper lobe on the 34th day of the patient’s stay in the ICU. We used electrical impedance tomography (Dräger PulmoVista 500; Dräger, Lübeck, Germany) as additional diagnostic support for the ultrasound, which confirmed reduced air entrance in the major part of the left lung. Finally, according to local protocol, we performed a CT scan of the chest, which confirmed previous findings of complete atelectasis of the left lower lobe and major atelectasis of the left upper lobe (Fig. ).\nWe decided to apply a new method whereby we used different experiences of single-lung ventilation respecting all basic principles of various types of mechanical ventilation. Our trial, which we named “targeted segmental recruitment,” was one of the last attempts to avoid the proposed life-threatening surgical reduction of the lung parenchyma in our young patient. His Acute Physiology and Chronic Health Evaluation II (APACHE II) score upon admission was 7, and his Sequential Organ Failure Assessment (SOFA) score upon admission was 6. On the day before we performed the segmental recruitment maneuver, his APACHE II score was 12, and his SOFA score was 6. His procalcitonin level on the day before segmental recruitment maneuver was 0.171 μg/L.\nThe patient was analgosedated, intubated with the Univent 8.5-mm tube (tube with integrated endobronchial blocker; Vitaid, Lewiston, NY, USA), and mechanically ventilated. We removed the endobronchial blocker and replaced it with a pulmonary artery (PA) catheter. With the help of a bronchoscope, using a loop through the working channel of the bronchoscope, the PA catheter was placed in the entrance to the lower left bronchus, and the PA catheter balloon was inflated to achieve bronchial closure. Using the appropriate connectors, we connected the PA catheter to the second ventilator (Dräger Evita 2) and applied 30 cmH2O of pressure of the 30% oxygenated air in CPAP ventilation mode for 30 minutes. The decision for the pressure of 30 cmH2O was made on the basis of the fact that pressures higher than 35 cmH2O are associated with barotrauma and the clinical appearance of pneumothorax []. The right lung and the upper lobe of the left lung were ventilated the entire time by controlled mechanical ventilation with protective ventilation parameters using the Dräger Evita XL ventilator. After 30 minutes, we removed the PA catheter from the lower left bronchus and placed it in the upper left bronchus with the described procedure and repeated the CPAP maneuver. Again, the right lung and the lower lobe of the left lung were simultaneously ventilated by controlled mechanical ventilation with protective ventilation parameters. Within the next 12 hours of the performed procedure, the patient was awakened and extubated. After the targeted segmental recruitment, he was breathing spontaneously with improved clinical parameters as well as better rising of the left side of the thorax with audible respiratory sounds on the left side. The PulmoVista 500 monitoring was applied continuously, and better ventilation of the left lung was noted in the first hours after the procedure. In the next 48 hours, we applied the CoughAssist device to the patient, and he was encouraged to cough. After 48 hours (36th day of treatment), a CT scan of the thorax was performed, indicating significantly better ventilation of the left lung (Fig. ). During and after the procedure, SpO2, end-tidal carbon dioxide (EtCO2), invasive blood pressure, and pulse were monitored. The patient did not have any significant decrease in SpO2 or increase in EtCO2, nor did he have hemodynamic instability or changes in heart rhythm. Before and after the procedure, arterial blood gas analyses were performed as part of routine laboratory processing (Table ). The patient was discharged to home 49 days after admission, and he was mobile on his own with stabile hemodynamic and respiratory status and without signs of infection. |
A one-year-old male was referred to Dermatology with nonhealing ulcerated areas on the right side of his neck and axilla corresponding to sites of recent removal of central and arterial lines, respectively. The working diagnosis from the referring doctors was contact allergic eczema to the dressing used to secure the lines. At the time of referral, he was intubated and ventilated in paediatric intensive care receiving intravenous (IV) antibiotic therapy for pneumonia. Sputum was positive for metapneumovirus. He had a significant past medical history including a bicuspid aortic valve, visual impairment, and chromosomal duplication disorder with significant developmental delay. On examination he had deep ulcerated areas with a violaceous undermined border on the right side of the neck () and right axilla in keeping with PG. A skin biopsy () supported this demonstrating a neutrophilic infiltrate in the upper dermis around the dermal blood vessels, in the deeper dermis and the subcutaneous fat. A small number of mononuclear cells were present. There was no evidence of vasculitis. Blood results revealed a marked leucocytosis (41.4 × 109/L); predominantly neutrophils (32.4 × 109/L); elevated C reactive protein 145 mg/L; anaemia; and thrombocytopenia. Blood film examination revealed a myeloid left shift and toxic granulation in neutrophils. Neutrophil integrin expression was normal. IgG and IgA were both elevated at 23 and 4.86 g/L, respectively. Specialist input from Immunology and Haematology was sought and it was felt that both the hyperglobulinaemia and blood film findings were secondary to inflammation and sepsis. Topical therapy with clobetasol propionate was commenced with a dramatic improvement within 24 hours (). Skin swabs were taken from line insertion sites and grew Candida albicans; this was felt to be of clinical insignificance as the lesions showed a dramatic clinical improvement within 24 hours of applying topical clobetasol propionate. No antifungal therapy was required and subsequent swabs were negative. Interestingly, similar lesions were noted a few days later upon resiting the central line to the left side of his neck. These cleared rapidly with the same treatment. One month later he was readmitted with pneumonia requiring a further course of IV antibiotics but fortunately did not develop any PG lesions. The clinical findings supported by histological features and the dramatic response to potent topical corticosteroid therapy favour a diagnosis of PG induced by pathergy. Furthermore, these lesions healed by forming cribriform scarring which is typical of PG. |
A 59-year-old Caucasian woman underwent laparoscopic cholecystectomy at the age of 45 years. During the procedure, the choledochal duct was injured, requiring direct suturing. Postoperatively, a slight leakage persisted and a stenosis of the choledochal duct at the site of the injury and a choledochocutaneous fistula developed. Because conservative measures did not close the fistula, a high Roux-Y latero-lateral choledochojejunostomy (biliodigestive anastomosis) was set up 5 months after cholecystectomy. Despite this procedure, recurrent cholangitis developed, which responded favorably to antibiotics at each episode. As the frequency of cholangitic episodes increased 2 years after cholecystectomy (patient age, 47 years), the choledochojejunostomy was revised, the jejunum was partially resected, an adhesiolysis was performed, and intrahepatic gallstones were removed. Eight years after cholecystectomy, cholangitis recurred, multiple liver abscesses developed, and blood cultures grew Streptococcus intermedius, Pseudomonas aeruginosa, and E. coli. Twelve years after cholecystectomy, cholangitis relapsed owing to the recurrence of bile duct stenosis at the site of the previous injury, which was dilated during an ERCP without complications. Despite this measure, cholangitis recurred 2 weeks later but responded to appropriate treatment. Three months later, cholangitis recurred and blood cultures grew ESBL. Stenosis at the site of the anastomosis was dilated and, for the first time, a plastic stent was deployed by means of ERCP. Three months later, cholangitis recurred. On ERCP, the previously placed plastic stent was no longer visible and a new one was deployed into the right hepatic duct without complications. Cholangitis recurred again 6 and 7 months later. During consecutive ERCP, a liver abscess was detected, and as the previous plastic stent was no longer visible, a 10-mm-long Wallstent (metal stent) was deployed.\nTwo weeks after ERCP, the patient was readmitted because of fever and tenderness of the right upper quadrant. Results of blood chemical investigations during hospitalization are presented in . During ERCP on hospital day (hd) 1, the old Wallstent was replaced with a new one. During a further ERCP under sedation with propofol 1% (10 mL) and midazolam (2.5 mg), 7 days later (hd8), the previously deployed Wallstent was removed. Because the patient did not wake up after the procedure, flumazenil (0.25 mg) was given, with no effect. Additionally, low blood pressure and respiratory insufficiency developed, requiring intubation and artificial ventilation. A computed tomography (CT) scan of the thorax showed air in the right atrium and ventricle, and pericardium (). Abdominal CT showed no bile duct stent; a widened choledochal duct; and air within the bile duct system, the liver, and the subcapsular veins (). Magnetic resonance imaging of the brain showed multiple hypointense lesions in the subcortical white matter, which were interpreted as being septic emboli or air emboli (). A CT scan of the cerebrum confirmed the presence of multiple air emboli within the parenchyma. Transesophageal echocardiography failed to detect a patent foramen ovale (PFO). Hyperbaric therapy was not available. A cerebral CT scan because of mydriasis, 1 day later (hd9), showed massive cerebral edema, with a shift to the left, and ongoing herniation of the brain stem (). Antiedematous therapy was ineffective. The patient died on the same day without regaining consciousness. At autopsy, the entrance of air into the venous system could not be detected neither inside nor outside of the liver; however, a PFO was found. |
A 17-year-old adolescent nulligravid girl whose last menstrual period was four days back referred with the diagnosis of low lying myoma after being transfused with four units of blood. She presented to the Gynecology Outpatient Department on 22 July 2018, with history of irregular vaginal bleeding of 2-year duration.\nShe gives history of lower abdominal swelling which was small initially and progressively enlarged to attain the current size for the last 13 months. She is sexually active but not married. She has no history of abdominal pain, urinary, or bowel complaint, has no bleeding from other sites, and has no significant past medical and surgical history noted.\nOn examination, patient was conscious, coherent with blood pressure of 100/60 mmHg, pulse 80 /min, and temperature normal and has pale conjunctiva, cardiovascular, and respiratory systems normal. Abdominal examination revealed 20-week sized nontender, firm, irregular, fixed, and lower border unreachable mass. On vaginal examination, a firm huge nontender growth attached to the anterior vaginal wall. It was difficult to reach at the cervix. A clinical diagnosis of cervical myoma was made.\nHer blood investigations on arrival showed hemoglobin of 9.7 gm/dL and were transfused one unite blood and the hemoglobin elevated to 10.7 gm/dL. Ultrasonography showed empty uterus with normal size echotexture and pushed up in to the abdomen. There is 10 by 12 cm hypoechoic mass arising from the cervix filling the vaginal canal with these the diagnosis of cervical myoma was made. The diagnosis necessitated Computed Tomography (CT) scan of the abdomen and pelvis which helped to know the extent of the mass and reported hypodense contrast enhancing mass seen on the uterus arising from the cervical region measures about 10.3 cm by 14.4 cm. The mass grows down in to the vagina and concluded with the diagnosis of contrast enhancing cervical mass likely myoma ().\nUnderstanding the complexity of her surgery and possible postoperative morbidity and mortality, surgical, anesthesiology, nursing, and recovery room teams was assembled with gynecologic oncology team. She was prepared for elective laparotomy the day before the surgery. On 18 August 2018, the patient was taken to operating room, after the general anesthesia given; she was positioned in a supine position. The abdomen cleaned with povidone iodine and draped with sterile towels and midline vertical incision was made. Intraoperatively, bilateral fallopian tubes and ovaries, the uterus, and urinary bladder grossly look normal; there is 10 cm by 15 cm sized firm mass between the vagina and the lower uterine segment (). Vesicouterine peritoneum incised the bladder reflected away from the lower uterine segment and upper vaginal wall, about 4 cm vertical incision was made on proximal anterior vaginal wall, and dissection of the mass away from the anterior vaginal wall was tried but it was difficult to have a clear cleavage line to excise the whole mass. Then another 6 cm longitudinal incision was made on the posterior vaginal wall; sharp and blunt dissection were made to separate the bulk of mass away from the vaginal wall; finally excision of the mass from the base within 1 cm of normal vaginal tissue was performed to carefully remove a 15 × 10 × 7 cm encapsulated mass (). The anterior vaginal wall, extension of incision on the cervix, and posterior vaginal wall closed separately with vicryl number 0 in two layers (). Hemostasis was secured, correct count was reported, the fascia and skin were closed using delayed absorbable stitch. The surgery was completed after 2hrs and 20 minutes.\nThe excised mass was sent for histopathological examination; the patient recovered completely and discharged on 6th postoperative day. The final pathological diagnosis of the mass was vaginal angiofibroma (). |
A 71-year-old Caucasian gentleman with a past medical history of chronic obstructive pulmonary disease (COPD) was admitted with shortness of air and productive cough. A CT angiogram of the chest was obtained due to concerns for pulmonary embolism and showed spiculated nodules in bilateral lower lobes; right nodule measured 1.1 cm x 0.9 cm while the left measured 1.5 cm x 1.2 cm. Also noted was right hilar lymphadenopathy with the largest lymph node measuring 2.9 cm x 2.1 cm; no mediastinal or axillary lymphadenopathy was noted. Emphysematous changes of the lungs were also seen. A biopsy of the left lung nodule and a right lymph node fine needle aspiration (FNA) were performed. Pathology results revealed the left lung nodule to be poorly to moderately differentiated nonkeratinizing squamous cell carcinoma. Cytology studies on the right lymph node sample showed highly atypical large lymphocytes with 95% monoclonal B-cells on flow cytometry, concerning for large B-cell lymphoma. After stabilization from respiratory standpoint, the patient was discharged home on supplemental oxygen therapy via nasal cannula at three liters/minute (L/min). His case was discussed in a multi-disciplinary tumor board. Cardio-thoracic surgery team felt he was not a candidate for lobectomy and would need radiation therapy; a referral to radiation oncology was made. A lymph node excision biopsy was planned to establish a definite diagnosis of lymphoma.\nApproximately eight weeks following his initial admission, the patient presented with worsening dyspnea with productive cough. He was hemodynamically stable with no increase in supplemental oxygen requirements; however, he was noted to have facial swelling, jugular venous distention, diffuse wheezing with bilateral basal crackles and bilateral pitting pedal edema. Treatment was started for acute COPD exacerbation. Intravenous (IV) furosemide was given as well as given peripheral edema along with prednisone 50 mg daily. A CT chest with contrast was performed; this showed a large, centrally necrotic 18.1 cm x 8.7 cm x 9.7 cm bilateral lobulated infiltrating mediastinal mass involving the entire mediastinum and the right hilum, causing mass-effect upon the heart, superior and inferior vena cava and pulmonary arteries and deforming the airway including trachea and right main bronchus. Significant narrowing of the SVC, the right main and right upper lobe pulmonary arteries was seen as well as of the left brachiocephalic vein. Multifocal consolidations were also noted. Bilateral lung nodules were re-visualized but were stable in size in comparison to prior CT scan. Small pericardial effusion was seen (Figure ).\nBlood and sputum cultures were obtained; broad spectrum antibiotics including vancomycin, cefepime, and levofloxacin were initiated for pneumonia in setting of recent hospitalization. The mediastinal mass was presumed to be rapidly enlarging lymphoma. Bone marrow and left supra-clavicular lymph node biopsy was obtained by interventional radiology (IR). Over the next 24 hours, he was observed to have increasing tachypnea; oxygen requirement increased from 3 L/min by nasal cannula (NC) to 10 L/min via a non-rebreather (NRB) mask. On examination, the patient was noted to have audible stridor. He acutely became hypotensive; systolic blood pressure (SBP) dropped from 110 mmHg and above up until this point to below 90 mmHg. The deterioration in his hemodynamic and oxygenation status is depicted in Figure .\nHe was transferred to the intensive care unit (ICU) and was subsequently intubated. Inotropic support was initiated. An arterial line waveform was suggestive of pulsus paradoxus. Electrocardiogram (EKG) showed sinus tachycardia at a rate of 110 beats per minute (bpm) with low voltage QRS (Figure ).\nA trans-thoracic echocardiogram (TTE) was immediately performed which showed moderate-sized pericardial effusion with signs concerning for impending cardiac tamponade including right ventricle diastolic collapse. The patient was urgently taken for diagnostic and therapeutic pericardiocentesis; 160 milliliters (mL) of pericardial fluid was drained followed by placement of a pigtail catheter in the pericardial sac. Following the procedure, the patient became increasingly hypotensive and inotropic support was intensified with addition of epinephrine followed by vasopressin to an already maximum rate of norepinephrine. The IV fluids in periodic boluses were given with temporary improvement in blood pressure each time. A repeat TTE showed only mild pericardial effusion. Over the next few hours, the patient developed worsening swelling of his face, upper chest, and upper extremities. He was also noted to have worsening abdominal distention. Eventually, the patient's urine output declined. Pericardial fluid analysis showed bloody appearance with 71% mononuclear cells; malignant lymphoma cells were noted on cytology. His recent lymph node biopsy results were reviewed and a preliminary read was consistent with aggressive DLBCL. Dose adjusted etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin (DA-EPOCH) were started promptly by the oncology team who felt that the lymphoma was chemo-sensitive and a rapid response to treatment could be anticipated. Prior to chemotherapy initiation, a renal panel was obtained, revealing metabolic acidosis (bicarbonate levels 14.2 mmol/L, pH 7.022 on arterial blood gas), hyperkalemia (potassium 6.0 mEq/L), hyperphosphatemia (phosphorus 6.3 mg/dL), and hypocalcemia (calcium 6.2 mg/dL). Nephrology was consulted for initiation of continuous renal replacement therapy (CRRT) in setting of oliguria and concerns for tumor lysis syndrome (TLS). His family requested for comfort care measures to be pursued at this point; the patient was extubated palliatively and expired within a few minutes. Later, the patient’s respiratory cultures grew Acinetobacter baumannii which was only intermediately sensitive to cefepime, meropenem, and levofloxacin (Table ). |
A 25-year-old man presented to our epilepsy center for evaluation of seizures. He was born at term without any developmental delays and had no risk factors for epilepsy including traumatic brain injury, brain surgery, febrile seizures, central nervous system infections, or family history of seizures and no significant past medical or psychiatric comorbidities. Three years prior to his presentation he had his first seizure. He did not remember the event, but while attending basic training in the Army, he was reportedly found in the shower confused by his fellow soldiers. There was no tongue bite or urinary incontinence, but he was disoriented afterward for much of that day. He had another episode within the same month while he was performing physical training exercises, whereby he collapsed and remained confused for hours, but no report of witnessed convulsions. An evaluation at that time was unrevealing. He had 12 episodes in the next 3 years. They were all similar, some associated with lateral tongue laceration suffered during the event. He was seizure-free for 6 months and then began to have spells at least monthly. He denied an aura or premonition preceding his seizures. His wife reported at night that he would “cry” at the onset and then appears to have clonic jerking bilaterally and symmetrically, up to 3 minutes in duration. He was reported to be distressed for a few minutes after the episodes. Brain MRI was reportedly normal and EEG abnormal, but the reports were unavailable. He had been taking levetiracetam 3000 mg daily with topiramate 50 mg daily. He had also tried valproic acid but reportedly had abnormal labatory studies so this was discontinued. At his appointment, it was determined that he would continue his current regimen of levetiracetam, and topiramate was increased to 100 mg total daily. A presumptive diagnosis of epilepsy was made upon clinical grounds though the classification included focal epilepsy localized to the frontal head region or genetic generalized epilepsy manifest as recurrent nocturnal generalized tonic-clonic seizures. At his follow up appointment, a high-resolution 3-T brain MRI was performed and was normal without intracranial abnormalities. EEG demonstrated 3–4 Hz generalized polyspike-and-wave discharges supporting a clinical diagnosis of genetic generalized epilepsy. The patient and his wife had recorded a video of his habitual seizures, which was reviewed an epileptologists (WOT). As noted in the video, he appears agitated and combative and is thrashing his extremities in a non-rhythmic and discontinuous manner with side to side head movements with eyes closed (). He and his wife were clear that this was the semiology of his typical seizure. The side to side head movements, eye closure, and discontinuous nonrhythmic hypermotor activity suggested FS . He was subsequently admitted to the epilepsy monitoring unit for LTVEM for differential diagnosis and classification of recurrent events. During the admission, EEG redemonstrated interictal generalized spike and polyspike and slow wave complexes noted previously. He had one seizure with clinical semiology suggesting a focal to bilateral tonic-clonic seizure due to head version, yet lateralized and focal seizures are known to occur in genetic generalized epilepsies . Despite the appearance of focal features, the ictal EEG demonstrated a generalized seizure onset. Immediately following a definitive diagnosis of epilepsy with electroclinical support from a electroclinical bilateral tonic-clonic seizure, he exhibited the exact same post-ictal behavior that was witnessed in clinic while reviewing the smartphone video. This behavior observed on the smartphone video was therefore able to be linked to his habitual postictal state with violent thrashing that simulated a FS (). In discussion with the patient and his wife, the difference between his seizure and a postictal state with confusion and combativeness was underscored to define a sequence of events rather than separate events. LTVEM was therefore able to establish a diagnosis of genetic genealized epilepsy despite the history suggesting focal epilepsy and the smartphone video suggesting a FS. |
A 22-year-old man was referred to our hospital with complaints of left eye redness and swelling for more than a month. He had no history of nausea or vomiting, but he also complained of mild blurred vision, double vision and occasional headache for more than a week.\nOn further questioning, the patient revealed a history of a trauma. He was involved in a motor vehicle accident and received a head injury that involved basilar skull fractures and resulted in a subarachnoid haemorrhage and epidural haematoma. The patient received conservative treatment and was discharged from a local hospital following the alleviation of symptoms. However, the patient developed symptoms in the left eye 4 months after the injury. These symptoms included blurred vision, swelling, and hyperaemia of the left eye.\nHe denied a history of diabetes and hypertension. There was no history of pneumonia, tuberculosis, or any other infectious diseases. There was also no history of fever, sickness or any surgery. There was no loss of appetite or loss of weight. He was a non-smoker with no allergies to any medications.\nOn examination, the visual acuity and intraocular pressure in the right eye of the patient were 6/5 and 17 mmHg, respectively, and the corresponding values for the left eye were 4/5 and 25 mmHg. On physical examination, there was no eyelid swelling, exophthalmos, ptosis or visual decrease of the right eye, and this eye was almost normal except for slight hyperaemia (Fig. ). Extraocular muscle movement showed no limitation in the right eye (Fig. ). However, the left eye exhibited eyelid swelling, mild ptosis, exophthalmos, chemosis, and corkscrew hyperaemia centred on the cornea (Figs. and ). Furthermore, there were some limitations of eye movement, and abduction and elevation of the left eye was − 1, yet movement on adduction and depression were normal (Fig. ). The left anterior chamber was slightly shallow and quiet, but the right anterior chamber was normal. The cornea was clear with intact corneal sensation in both eyes, and there was no relative afferent pupillary defect and no anisocoria noted. The vitreous and lens were clear. Fundus examinations did not show disc swelling, obvious vascular dilatation or tortuosity, cotton wool spots or haemorrhages of either eye. The resident doctor previously doubted the presence of glaucoma, and he was admitted to our hospital. Optical coherence tomography (OCT) and field of vision tests did not reveal any abnormalities. By contrast, the MRI of the periorbital region revealed a broadening of the left superior ophthalmic vein, slight thickening of the left lateral rectus muscle, and an expansion of the left cavernous sinus, yet the right superior ophthalmic vein, extraocular muscles and cavernous sinus were almost normal (Fig. ). These results aroused suspicion of the left CCF, so the patient was transferred to the neurosurgery department. The neurological examination was normal with the exception of a periorbital bruit on the left side. Thus, a tentative diagnosis of a left CCF was made. Surprisingly, cerebral angiography revealed a crevasse in the inner side of the intracavernous segment of the right internal carotid artery, the right cerebrovascular (CVA) filling delay, and arterial blood traversing the intercavernous sinus to reach the contralateral cavern, which resulted in the dilatation of the left ophthalmic vein (Fig. ). Therefore, the patient was ultimately diagnosed with right CCF. Embolization surgery was suggested, but no additional treatment for the eyes was mentioned since most studies show that symptoms in the eyes could be completely relieved after aetiological treatment.\nDetachable balloon catheter embolization surgery was performed after several days. The surgery was successful, and the patient recovered well. All symptoms, including the redness and swelling of the left eye, the blurred vision, and the double vision, were resolved. On physical examination prior to the patient’s discharge, the visual acuity was improved to 5/5, and the intraocular pressure was 18 mmHg in the left eye, which was within the normal range. Additionally, the exophthalmos, chemosis and hyperaemia of the left eye were significantly relieved (Fig. ). |
A 7-year-old male presented with a 1 month-long history of nausea and vomiting. A head CT was performed for concern for increased intracranial pressure and demonstrated a large cystic frontal mass that crossed the midline, with perilesional edema. His past medical history was significant for a diagnosis of “lazy eye” 1 year previously and short stature (later determined to be due to growth hormone deficiency). Family history was unremarkable.\nMRI brain demonstrated a large cystic sellar/suprasellar mass, most consistent with craniopharyngioma (). MRI volume measures as with Case 1 demonstrated primary cystic disease with minimal solid tumor component (, graph). Initial surgical management included stereotactic cyst catheter placement with concomitant drainage of ~50 mL of “machine oil”-like cyst fluid. His initial post-operative course was uneventful.\nThe patient returned to the outpatient clinic for weekly cyst aspiration. Three months after catheter placement, MRI demonstrated an increase in cyst size (), and repositioning of the catheter was undertaken. Resumption of scheduled cyst aspiration again led to a decrease in the cyst size (, graph). However, due to the need for continued cyst aspiration, and the position of the tumor, which appeared amenable to gross surgical excision, craniotomy for tumor removal was undertaken. At surgery, the cyst wall was densely adherent to the anterior cerebral vessels, and subtotal resection was achieved. The patient was treated with focal radiation (54 Gy in 30 fractions) but was noted to have rapid cyst recurrence during radiation. Following completion of radiation, a cyst catheter was replaced. The patient was treated with intracystic bleomycin, which resulted in an initial decrease in cyst size. However, after 1 month, the patient developed new onset lethargy, and imaging demonstrated right frontal lobe edema adjacent to the cyst wall (). He was treated with dexamethasone and cessation of bleomycin with resolution of his symptoms.\nThe patient was offered systemic tocilizumab (12 mg/kg IV q2 weeks) on a compassionate use basis. After 6 months of therapy, the cyst exhibited a partial response with measurements improved from 26 × 28 × 27 mm (AP × TRV × CC) to 18 × 24 × 18 mm (AP × TRV × CC) (). He has had no significant adverse effects, no new neurologic sequela and no hospitalizations. Therapy was discontinued after seven months, and he continues on an MRI monitoring plan. |
A 55-year-old man with a history of extensive alcohol and tobacco use presented with a 2-month history of a progressively enlarging, 5-cm ulcerative, and painful midline tongue lesion extending to the floor of mouth resulting in an anterior tongue cleft. This lesion was associated with unintentional weight loss, left otalgia, and submandibular swelling. Bilateral nontender palpable lymphadenopathy in levels I, II, and III were present.\nPrior biopsies by an otolaryngologist in private practice did not demonstrate carcinoma but were otherwise inconclusive with reactive inflammatory changes. However, a positron emission tomography scan revealed hypermetabolic lesions of the anterior tongue as well as lymph nodes bilaterally in levels I, II, and III suggestive of malignancy (). The assessment was a likely T3/4N2cM0 tongue squamous cell carcinoma. He underwent a panendoscopy with biopsies and a physical examination under anesthesia for surgical planning. Biopsy results again did not yield carcinoma but showed granulomatous inflammation without organisms present.\nA multidisciplinary tumor board recommended a rheumatologic workup and an excisional lymph node biopsy. Lymph node biopsy results demonstrated noncaseating granulomatous inflammation with no malignancy (), while laboratory results demonstrated markedly elevated ACE (angiotensin converting enzyme) levels. These findings suggested an atypical case of sarcoidosis, and the patient was referred for pulmonary consultation. The patient had developed scattered erythematous macules involving the extremities, palms, soles, and trunk. Laboratory evaluation demonstrated T pallidum immunoglobulin G antibodies present with a reflex RPR (rapid plasma reagin) titer of 1:512. The lymph node biopsy sample was then analyzed with immunohistochemistry (IHC) revealing spirochetes (). The patient was diagnosed with tertiary syphilis and started on doxycycline 100 mg twice daily for 30 days because of a severe penicillin allergy. After receiving treatment, the patient came back to our clinic with his tongue lesion healing well but with a persistent anterior tongue cleft (). The patient has not obliged with further laboratory evaluations to recheck a RPR titer after antibiotic therapy. |
A 51-year-old male patient visited a local hospital because of dyspepsia and epigastric pain. Ultrasonography revealed a 4.5 cm sized cystic mass near the CBD and pancreatic head (Figure ). He was transferred to our hospital for further evaluation of the cystic mass. His medical history and laboratory findings were unremarkable. Tumor markers such as alpha-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen 19-9 were within normal limits. Contrast-enhanced abdominal computed tomography (CT) showed a homogeneous low-density cystic mass with thin, smooth walls next to the CBD, and there were suspicions of a communication between the two structures (Figure ). Under the impression that the lesion was a type II choledochal cyst, which is a discrete diverticulum of the extrahepatic bile duct, magnetic resonance (MR) imaging and endoscopic ultrasound (EUS) were done. The cystic mass showed low signal intensity on the T1-weighted MR image and high SI on the T2-weighted MR image with nearly imperceptible walls and there was no evidence of an enhancing solid portion in the cyst (Figure ). EUS also revealed a 4.5 cm sized cystic mass which seemed to be connected with the CBD, and gastroscopy showed irregular mucosal thickening with hyperemic change in the second portion of the duodenum (Figure ). Based on these findings, the patient underwent Whipple’s operation under the impression the lesion was a type II choledochal cyst with extrinsic compression of the duodenum, and the possibility of combined duodenal malignancy due to the mucosal lesion in the duodenum. An examination of the resected specimen revealed a duodenal cancer in the second portion of the duodenum 2.5 cm proximal to the ampulla of Vater, and the cystic mass did not show communication with the CBD (Figure and B). The histological diagnosis was mucinous adenocarcinoma of the duodenum with cystic metastasis and subpyloric lymph node metastasis (Figure and D). The postoperative course of the patient was uneventful. The patient was disease-free 12 mo after the initial diagnosis. However, the patient died 18 mo after the recurrence. |
A 36-year-old healthy female presented to our Emergency Department with a progressive swelling on the left side of her neck that had started 2 days before presentation. There was no history of trauma, fever, cough, coagulopathies, recent medical procedures, medication intake or thyroid problems. The patient complained of pain and dysphagia on the side of the swelling.\nThe initial evaluation revealed a sick-looking nonetheless stable patient who was not in dyspnea or in respiratory distress. The neck examination revealed a well-defined soft cystic lesion confined to the left side of the neck anteriorly, measuring around 4 cm × 4 cm, extending from the left thyroid lobe levels III-IV. It was tender to the touch and moved with deglutition. Preliminary fiberoptic flexible scope examination of the larynx was normal.\nLaboratory investigations showed a white blood cell count of 10.42 × 10 × 9/L, hemoglobin of 115 g/L and calcium levels of 2.32 mmol/L, which were all within the normal range. The thyroid function test was also within the normal range with elevated antithyroglobulin antibodies.\nA preliminary ultrasound study of the neck showed posterior inferior left lobe hypoechoic thyroid nodule measuring 3 cm with ill-defined lateral border and echogenicity suggesting turbid fluid collection in the left neck spaces [].\nA computer tomography scan showed diffused inflammatory changes of the superficial and deep facial planes mainly on the left side of the neck; however, they were confined to the infrathyroid region within the muscular and visceral facial planes of the intermediate deep cervical fascia [Figure and ].\nThree hours after the initial assessment, the patient's condition suddenly changed. The swelling on the neck became engorged with increased tenderness. However, the patient remained clinically stable with no evidence of airway compromise or respiratory distress. Laryngeal flexible fiberoptic scope was repeated and revealed a left aryepiglottic submucosal hematoma compressing the airway and shifting the patient's laryngeal inlet to the right [Figure and ].\nAccording to the findings of the flexible fiberoptic scope, the focus was on securing the airway. Therefore, the patient was electively intubated then transferred to the intensive care unit for observation. The patient was managed conservatively with intravenous antibiotics and steroids (dexamethasone). The following day, a, direct flexible fiberoptic scope was repeated which showed regression of the left sided submucosal swelling, and laryngeal inlet patency was visualized. The patient was then extubated and transferred from the intensive care unit to the ward where she gradually improved and discharged on the following day.\nThyroid ultrasound-guided fine needle aspiration (FNA) cytology showed normal sized thyroid gland with evidence of an oblong shaped, fairly well-defined hypoechoic nodule in the left thyroid lobe. Cytology was reported as Thy1 (Bethesda class I, nondiagnostic or unsatisfactory). In relation to previous imaging studies and cytology report, a diagnosis of ruptured thyroid cyst and hematoma was established. |
Our 31 year old male patient was born in a rural village in Myanmar with limited access to medical care. He recalls being frequently ill during his childhood, but otherwise had normal development. He had been diagnosed with malaria due to frequent flu-like symptoms. He also had episodes when he was unable to move properly. The duration of these episodes is unclear, likely weeks. He had one episode at four years old where he could not speak, followed by a full recovery. His childhood progressed with these episodes until the age of 18 years. At this time, he developed difficulty writing with his right hand and switched to writing with his left. Within a year he developed dysarthria, described as trouble pronouncing words and phonating, as if “only air came out.” He also had a mildly abnormal gait without tripping or falling. Up until this time he was taught by missionaries and learned how to read, write, and speak English, which he did well despite his symptoms. However, his symptoms progressed and by 21 years of age he was unable to talk, walk, or write. It is unclear if his symptoms were due to dystonia and/or rigidity and bradykinesia at this time, though both are possible. He was brought to the nearest city for hospitalization, during which time he was tried on multiple medications. It is unknown what was tried initially, except for trihexyphenidyl; he had no response to any of them. He was then started on carbidopa/levodopa with profound improvement in his motor and speech abilities. He was discharged without a clear diagnosis but continued on carbidopa/levodopa functioning near normal.\nAt the age of 24 years he fled Myanmar to Malaysia. Doctors evaluated him there and advised him to stop carbidopa/levodopa as his exam was normal. After about 1 month off the medication he started to develop difficulty walking and talking. He restarted carbidopa/levodopa and these symptoms improved. He tried to discontinue carbidopa/levodopa a few more times but with each attempt his symptoms would return within several weeks.\nAt the age of 29 years he moved to the United States and was diagnosed clinically with dopa-responsive dystonia. He had head imaging and blood work that were reportedly unremarkable. He was on 450 mg/day of levodopa at the time. His symptoms had no diurnal variation, were relatively consistent throughout the day, and only seemed to worsen when he was tired. Trihexyphenidyl 4 mg/day was added with little benefit.\nHe first presented to us in October 2016 at the age of 31 years. At this time he was on carbidopa/levodopa 10/100 mg 1.5 tablets three times per day and trihexyphenidyl 2 mg twice per day. On exam he had dystonia in his neck and trunk that was subtle at rest and became prominent with walking. He displayed posterior shift and flexion in the neck, and mild extension in the trunk. He exhibited a sensory trick with improvement in his posturing when touching his hand to his chin; this also occurred but to a lesser degree when he imagined touching his chin without actually touching it, and to an even lesser degree when the examiner touched his chin. He had mild improvement when walking backwards. While walking forward he also had increased arm swing with mild choreic movements in his fingers, right side greater than the left. The remainder of his general and neurological exam was normal, with no parkinsonism and no evidence of dystonia in his limbs at rest or with action (including writing) or in his face. His exam can be seen in the included video (additional files , , , , and ).\nHe was referred for genetic testing to further elucidate the etiology of his dystonia. A 16-gene dystonia panel was sent through Invitae, including ANO3 (DYT23), ATP1A3 (DYT12), GCH1 (DYT5A), GNAL (DYT25), PARK2 (AR juvenile parkinsonism), PNKD (DYT8), PRKRA (DYT16), PRRT2 (DYT10), SGCE (DYT11), SLC2A1 (DYT9 and 16), SLC6A3, SPR (DYT5B), TH (DYT5B), THAP1(DYT6), TOR1A (DYT1), and TUBB4A (DYT4). This revealed a homozygous missense mutation in the TH gene (p.Thr494Met, transcript NM_199292.2) leading to a diagnosis of autosomal recessive tyrosine hydroxylase deficiency. This mutation is likely pathogenic and has been previously reported in patients with more severe phenotypes [, ].\nHe has no known family history of neurological or medical disease, nor of consanguinity. He knew his mother, father, older sister, and three younger brothers well until the age of 21 years when he left his village for medical care. He has had little to no contact with them since. |
A 40-year-old man presented to the University of Alabama at Birmingham Medical Center emergency department with depression. He was admitted to the hospital and started on oral fluoxetine 20 mg daily, titrating to 40 mg daily after the initial 3 days. The patient then gave written informed consent to participate in an investigational study examining rapid reduction of depressive symptoms in patients with major depressive disorder with active suicidal ideation with intent. At the time of writing this case report, esketamine nasal spray has been approved only for the management of treatment-resistant depression (TRD). He met the trial eligibility criteria and was randomized to esketamine nasal spray, which was initiated on day 1 at a dose of 84 mg administered using 3 nasal spray devices (28 mg/device) twice per week for 4 weeks. After the initial dose, the patient reported dysgeusia almost immediately and began intermittently retching, which delayed the administration of the subsequent dose. He continued retching intermittently for 14 min, and the bad taste persisted for approximately 3 h.\nAfter the third dose was administered on the first day, the patient was encouraged to rinse out his mouth with water and spit it out without swallowing. However, the patient retched within 2 min and the bad taste persisted for several hours. On the second dosing day, rinsing with water was attempted again, with a similar lack of effect. After 20 min of intermittent retching, a nurse suggested a fruit punch–flavored powdered drink (Crystal Light Fruit Punch) mixed with water to mask the taste, which the patient drank 25 min after the initial spray. The bad taste resolved within 5 min (30 min after the report of the adverse event). During the third dosing day, dysgeusia was reported again, but after drinking the punch-flavored beverage, the time to resolution decreased to 24 min after the adverse event was reported. This strategy was employed for all subsequent doses, and the patient reported only one more instance of dysgeusia on the final day of dosing. Again, time to resolution was within 30 min of the report of the adverse event. A punch-flavored drink has been used successfully to reverse or prevent dysgeusia with other patients at our center. |
A 29-year-old man presented to the orthopedics department with progressive right wrist pain. He sustained a blunt injury caused by assault 3 days ago and initially evaluated in the emergency department. Radiographs of the wrist were obtained, and he was diagnosed with wrist sprain. He was prescribed with nonsteroidal anti-inflammatory and muscle relaxants drugs, and a volar short arm splint was applied. He was referred to the orthopedics clinics when his pain did not respond to treatment. The splint was removed, and inspection showed wrist edema along with loss of the dorsal ulnar styloid prominence ().\nThe wrist was tender to palpation, and the ulnar styloid process was not palpable at the dorsal aspect of the joint. He had mildly limited dorsiflexion and palmar flexion, and no forearm rotation. His previous X-rays, obtained 3 days ago in the emergency department, revealed an obvious UVD ().\nClosed reduction was attempted under local anesthesia, however failed due to extreme pain. A computerized tomography (CT) was taken for possible locked dislocation. Axial views and volume-rendered images of the DRUJ revealed volar dislocation of the ulnar head engaged with the sigmoid notch ().\nThe patient was taken to the operating room and under general anesthesia, the ulnar head was reduced by forceful pronation. The DRUJ was stable during passive forearm rotation. A long arm cast was applied with the forearm in semi-prone position and kept for 3 weeks. Magnetic resonance imaging (MRI) was obtained to inspect possible injuries to the stabilizing soft tissues and showed incomplete triangular fibrocartilage complex (TFCC) tear and bone impaction injury caused by engagement of the ulnar head to the sigmoid notch (). The cast was removed at the 3 weeks, and rehabilitation was initiated. The 3-stage wrist rehabilitation program was prescribed for the patient: Progression to each stage was guided by pain response. The stages of rehabilitation were described as pain control and ROM, regaining wrist stability with ROM, axial loading on the wrist and neuromuscular control. The patient performed isometric strengthening of the DRUJ stabilizers, PQ, and extensor carpi ulnaris (ECU). Isometric exercises were applied to the ECU and PQ muscles. Once the patient was able to complete two sets of 10 repetitions of isotonic strengthening exercises without significant increase in pain, a variety of balance, weight-bearing, and coordination activities was performed. After 3 weeks, he reached full range of forearm supination and pronation without pain. At the last follow-up, more than 24 months after closed reduction, he was doing well without any pain or limitations in range of motion (). Since the hospital outpatient service visits were limited due to the COVID-19 pandemic, the patient evaluated with a quick dash score (0) through online interview. |
Patient 2 was a 31-year-old woman of Salvadoran descent, who delivered three children. At 4 years of age, the patient first presented episodes of seizures and abnormal neurological findings and was diagnosed with HHH syndrome. The patient had developmental disability, manifested by expressive language and attention problems determined upon neuropsychiatric evaluation, and significant myopia. The patient was prescribed a diet consisting of protein restriction to 1.5 g/kg/d during childhood. The patient's weight was at the 25th percentile with height less than the 3rd percentile.\nThe patient's first pregnancy occurred at 18 years old. She had episodes of nausea and dizziness. She was maintained on a low-protein diet in the first trimester, but mild hyperammonemia was detected during the 11th and 12th week of pregnancy; as a result, protein intake was further restricted to 40 g/d. It was unclear if the patient was compliant or not. At 22 weeks gestation, the patient developed seizures, and was initiated with carbamazapine. Brain imaging showed multiple small calcifications of unreported cause, with normal cerebral angiography. The child was born at full term, via Cesarean-section. The baby girl had intrauterine growth restriction (weight <3rd percentile). Although the baby girl remained small, follow-up recorded a normal development at her 2 years of age.\nVery little is described for the course of her second and third pregnancy. The second child was born via Caesarian section. A moderately elevated ornithine concentration (302 μM/L) was measured in the cord blood, but the levels decreased to normal in the newborn's blood 24 hours after birth. The patient's blood ammonia levels increased 24 hours postpartum and were given a treatment consisting of oral sodium benzoate and intravenous arginine. The patient responded well to the treatment. Follow-up showed normal growth and development for the child at 10 months of age.\nThe third child was born via repeat Cesarean section and had transient respiratory distress which required mechanical ventilation. No further information was given regarding the outcome of this child.\nUpon follow-up, the patient was noted to have been prescribed more anticonvulsants, and had significant weight loss from 95 to 50 lb. The patient died suddenly at the age of 31 years and 9 months, with an autopsy revealing two firm nodules in the brain, one each in the left parietal and frontal lobes with no determined cause of death. |
It is a case report of a 13-year-old girl appeared to the outpatient department with the history of swelling over her right hand for 5 months. It was gradually progressive in nature and situated over the dorsum and medial aspect of the hand. There was no history of trauma or such type of lesion elsewhere in the body or the in the family. There were no any associated features, i.e. pain, fever, or any sign or symptoms influencing her general health. On examination, there was a swelling, which was firm in consistency and occupying the dorsal and inner side of the fifth metacarpal. Local temperature was not raised and the skin was mobile and there was no any feature suggestive of inflammatory pathology. On deep palpation, it was tender and the range of movement was restricted.\nThe routine hematological examination was within normal limit. The radiology revealed that there was an osteolytic fusiform expansible lesion involving to the whole distal 2/3rd of the fifth metacarpal and the articular surface too. The cortical is was paper thin, breached, inflated, and without the periosteal reaction () and the tumor radiograph had “soap bubble” appearance. Hence, the provisional (clinicoradiological) diagnosis of aneurysmal bone cyst and GCT was conceded. The chest X-ray was also sought and it was within the normal limit. The core-cut biopsy sent and it conferred the diagnosis of GCT ().\nOur technique is free osteoarticular metatarsal transfer, described by the Maini etal. The dorsal approach was used for the enbloc resection. Incision also included the previous biopsy track. The fifth metatarsal was removed except the base of it along with the partial resection of surrounding muscles (). While removing the mass in enbloc, the capsule and collateral ligament of the fifth metacarpophalangeal joint left. The fourth metatarsal (same side) was harvested from the foot along with its capsule and collateral ligament of the metatarsal-phalangeal joint. The required length of the metatarsal was measured preoperatively, and it was osteotomized out from the base (), and the capsuloligamentous of the metatarsal sutured to the counter capsuloligamentous structure at the recipient site to reconstruct the metacarpal-phalangeal joint. The metatarsal was fixed with the leftover base of the metacarpal by the K-wires and the volar slab applied (). At the 14thpost-operative day, the sutures removed and the exercise started gradually.\nAt each follow-up, the clinical and radiological assessment was done. The Union at the junction of the metatarsal and the base of the leftover metacarpal occurred in the 6weeks and no obvious changes noticed at the transferred metatarsal. Initially, the movements had both extension and flexion lag, so the meanwhile electric stimulation given. At the end of the 6months of follow-up, the movements are painless and almost up to normal except the terminally restricted at the flexion. It ranges from 0° to75° flexion at the metacarpophalangeal joint ( and ). The patient was able to grasp any object and has pretty good grip strength.\nAfter the 2years of follow-up, after the surgery, the procedure is fulfilling our expectations and corroborates the reliability of this method. During the initial follow-up, the patient had the mild-to-moderate pain over his foot while walking and unable to dorsiflex his fourth toe. However, now, she is free from pain or any complaint such asdeformity or difficulty in walking. However, there is still slight weakness of fourth toe’s dorsiflexor. Finally, she is happy and has no any complaints. |
A 39-year-old pregnant Caucasian woman presented to the Obstetric Emergency Room reporting vertigo, vomiting, nystagmus, dizziness, and hindrance in the execution of fine movements of the right arm. The maternal parameters on admission are regular: pulse 98 beats per minute, pressure 110/68 mmHg, and temperature of 36.2 °C. She had an obstetric history of a first-trimester spontaneous abortion and a medical history of tension headache. She is married and graduated. She has a high socioeconomic status and is employed as an engineer.\nThe ongoing pregnancy coursed physiologically until that moment. The fetus was screened for aneuploidy with a noninvasive prenatal test (NIPT), while second- and third-trimester ultrasounds for the study of malformations were both normal. The oral glucose tolerance test at 24 weeks was negative. At 38 + 4-week gestation, the patient was hospitalized due to suspected vestibular neuritis diagnosed by an otolaryngology (ORL) specialist. Following diagnosis, the patient started therapy with corticosteroids, including prednisone 5 mg two times per day and levosulpiride 25 mg two times per day, that continued for 3 days.\nOn day 2, the patient developed diplopia and worsening of vomiting and dizziness, with improvement of symptoms in left lateral decubitus. The ORL revaluation excluded peripheral vestibulopathy and progressed to an urgent brain computed tomography (CT) scan without contrast that excluded ischemic or hemorrhagic brain lesions.\nOn day 3, due to further worsening of symptoms, urgent neurological counseling was performed. Viral examinations of herpes simplex I and II, herpes zoster, and herpes virus VI were negative. The neurologic examination showed the left eye adducted and elevated, vertical diplopia, and presence of rotatory nystagmus accentuated on the right gaze and dyssynergia in the cerebellar maneuvers of the right upper limb. After a detailed medical history, the patient stated for the first time that she had undergone cervical spine manipulations by an osteopathic specialist in the days preceding the beginning of the symptoms. Magnetic resonance imaging (MRI) of the brain was urgently performed, showing a punctate lesion hyperintense on diffusion-weighted imaging (DWI) (Fig. A–B), characterized by a reduction of apparent diffusion coefficient (ADC) on the colorimetric maps. This finding was suggestive of ischemia in the posterolateral right medulla oblongata, which is consistent with the symptoms of Wallenberg syndrome, although the patient did not have the full spectrum of symptoms. On MR angiography, the intracranial V4 segment was normal, but the right posteroinferior cerebellar artery (PICA) was not present (Fig. E). Urgent thrombolytic therapy or emergency revascularization was not deemed necessary by the neurologist colleague. Acetylsalicylic acid (ASA), 100 mg, therapy was then prescribed. Subsequently, echo-color Doppler ultrasound of the supra-aortic trunk detected no alterations of the cervical vessels, and transthoracic echocardiogram with exclusion of patency of the foramen ovale was performed. During this observation period, the patient was continuously monitored with noninvasive blood pressure monitor and pulse oximeter to detect hypotensive state and/or desaturation episodes early. Considering the clinical condition of the patient, the term gestational age, and initial onset of prodromal contractions, an elective cesarean section (CS) under subarachnoid anesthesia was performed, given the inability of the patient to deliver vaginally because of the ischemic cerebral event and obligatory left lateral decubitus, diplopia, and dizziness. The intraoperative and postoperative courses were uneventful. Six hours after the CS, prophylaxis with low-molecular-weight heparin was prescribed.\nThe neonate showed good adaptation to postnatal life with an Apgar score of 9 at 1′, 10 at 5′, and 10 at 10′; blood gas analysis was regular both in the artery and vein. Normal anthropometric parameters were present: 3250 g (52nd centile), length of 49 cm, and cranial circumference of 36 cm.\nOn day 4, for better study suspected dissection on small vertebral vessels, the patient underwent CT angiography of the neck, which showed a focal dissection at the V2 distal segment of the right vertebral artery (Fig. C–D). The puerperium course was normal, and the midwife helped the patient during breastfeeding because of the difficulty of standing up and walking due to the diplopic symptom. Psychological support was offered during the hospitalization, with daily physiotherapy rehabilitation and orthoptic evaluation. Congenital and acquired thrombophilia tested negative. After 12 days of rehabilitation, the patient was discharged with continued complaints of diplopia and a walker for mobility. After the VAD diagnosis and for the entire length of hospitalization, the patient was treated with Cardioaspirin 100 mg/day and prophylactic enoxaparin 4000 UI/day subcutaneous injection for 60 days. At the subsequent neurological evaluation, during the follow-up of 2 and 4 months, the patient showed persistence of vertical diplopia and a circumspect and wide gait, and life-long ASA was prescribed. A follow-up MRI was scheduled for 6 months after the stroke, which confirmed the signs of the previous ischemic lesion on the posterolateral right medulla oblongata. The remaining findings are unchanged.\nEthical approval was obtained, and the patient gave written informed consent to publish this case and any accompanying images. |
A 42-year-old Hispanic female presented to the emergency room with a one-day history of epigastric abdominal pain, nausea and vomiting. The pain was mostly in left upper quadrant of abdomen, sharp, intermittent, without any alleviating or aggravating factors. It was associated with multiple episodes of nausea and bilious vomiting. Patient denied any fevers, chills, diarrhea, constipation and prior similar episodes. Her relevant past surgical history included Laparoscopic Roux-en-Y gastric bypass (LGBP) 13 years ago and recent robotic assisted Total abdominal hysterectomy with bilateral salpingo-oophorectomy (TAH BSO) 10 days ago for bleeding uterine fibroids. Patient had low grade fever with tachycardia on presentation. Her abdominal examination revealed mild distension with tenderness and fullness in the epigastrium and left hypochondriac regions of the abdomen. Rectal examination was unremarkable. Her vital signs and blood work were as shown below (Tables and ). She was admitted, with plans for bowel rest, intravenous hydration, blood work and urgent CT scan of abdomen and pelvis with soluble contrast.\nCT scan of her abdomen and pelvis revealed findings of previous gastric bypass with gastric pouch severely dilated and air-fluid levels measuring up to 17 cm. The excluded portion of the stomach was noted to be decompressed as a result of dilated gastric pouch. The proximal to mid small bowel was fluid-filled and severely dilated to approximately 4 cm. Distal small bowel and colon were decompressed. There was no evidence of pneumoperitoneum. Based on these findings initial diagnosis was presumed to be gastric outlet obstruction with dilated gastric pouch. Fluid hydration and nasogastric tube decompression was continued. Patient was prepared to be taken to the operation room for emergent diagnostic laparoscopy.\nThe intraoperative findings were not consistent with the radiological findings (Figs –). As shown in the CT scan of abdomen and pelvis; multiple loops of dilated small bowel were seen, more predominantly dilated and matted bowel complex just above the spleen. The dilated bowel complex was decompressed using needle aspiration to help reduction of the bowel loops. The ileo cecal junction was then identified and traced back to the perisplenic hilar region. The trifurcation of three bowel loops (Roux limb, biliopancreatic limb and common limb) were identified with identification of ligament of Treitz. The prior gastric bypass was of ante colic- ante gastric type and Peterson’s defect was not closed. The above described bowel trifurcation had sunken postero- inferior to the spleen and had herniated through a potential space underneath the spleen and torsed around the splenic vessels and hilum. This picture was consistent with perisplenic small bowel volvulus. As noted earlier there was a massively dilated proximal small bowel segment above the spleen, displacing it caudally. Careful adhesiolysis was performed inferior to the spleen to release the segment of the trifurcation. The bowel segments once reduced were assessed and appeared viable. Of note, the spleen appeared better perfused after reduction. The remnant hernia defect of size 2 × 3 × 2.5 cm around the hilar region was then approximated using interrupted non-absorbable sutures. Enterotomy at the decompression site was closed with non absorbable suture. All potential sites of IH were re-examined and there was no evidence of any other internal herniation. Coelomic cavity was thoroughly irrigated and operation concluded successfully. Postoperative hospital stay was uneventful, and the patient was discharged home after two days. |
A 12-months old boy was hospitalized because of a mass in the sacrococcygeal region. The boy was born full term by normal vaginal delivery with a birth weight of 3050 g. The sacrococcygeal mass was detected by prenatal US at 34 week’s gestational age. There was no family history of twins. The patient had no other congenital anomalies. On a physical examination, the boy was well-nourished, except for a lower limb-like structure that measured 35 × 25 cm, with a scrotum like structure in the lower back and buttock of the boy (Fig. ). A complete blood count, kidney-liver function test, and tumor markers including β-HCG, AFP and CEA were within reference range. CT showed a large, complex mass with a bony structure resembling sacrococcygeal bone, hip bone and the femur (Fig. ). The mass had cystic and solid components with blood supply from the aorta of the boy (Fig. ). Spinal dysraphisms of the sacrum of the host were observed and adipose tissue in the epidural space was continuous with mass (Fig. ). MRI showed the mass was connected to the dilated sacral canal of the boy. This resulted in a tethered cord and the conus medullaris was terminated at the level of the fourth lumbar vertebra. The filum terminale was thickened and had been moved to the sacral canal (Fig. ). MRI also showed the mass had bony structure, cystic lesions, soft tissue components similar to skeletal muscle around joints and bones and abundant percutaneous adipose tissue. No anomalies in other systems including the urinary system, the cardiac system and the bowels were detected from abdominal CT, ultrasound, echocardiography and a Barium enema examination. No obvious mass effect was found because the mass was almost exophytic. A preoperative diagnosis of FIF was made and surgery was performed to remove the mass. Lysis of the tethered cord and sacral canal plasty were also performed. The patient’s parents agreed to perform the treatment that the boy received. On gross examination, the mass had malformed trunk, buttock, intestine and 1 lower limb with 1 toe in the foot. Upon incision, bones, soft tissues, malformed intestinal tissues were observed. Histopathological examination of the mass showed skin, nerves, bone and bone marrow, adipose tissue, skeletal muscle and intestinal tissue. The postoperative period was uneventful. The child was in good health during a follow-up of half a year and there was no evidence of recurrence. |
A 43-year-old man was referred to our hospital reporting several months of abdominal discomfort. He was a heavy smoker with 30 pack/years. His medical history included an ischemic stroke, helicobacter pylori gastritis and severe hypertension complicated by cardiac hypertrophy. He was prescribed chlorthalidone, barnidipine, lisinopril, nebivolol, clopidogrel, simvastatin and ranitidine. His family history was remarkable for multiple aortic aneurysms and coronary artery disease in his father, who died at a young age of a ruptured aneurysm. His mother had been treated for systemic hypertension. On physical examination he was hypertensive with a blood pressure reading of 211/130 mmHg and a heart rate of 55 bpm. During auscultation of the abdomen a murmur was detected. Palpation of the abdomen was not painful. Peripheral pulsations were present in both arms and legs. Neurological examination was normal.\nLaboratory results showed severe renal insufficiency with an estimated glomerular filtration rate (eGFR) of 14mL/min and a creatinine level of 418 μmol/L. A CT angiography revealed a large suprarenal aortic aneurysm with diffuse circular atherosclerosis and extensive circumferential aortic wall thrombosis (). The celiac trunk, superior mesenteric artery and right renal artery were occluded and the right kidney was atrophic. The left renal artery was critically stenosed. The patient underwent a left renal artery percutaneous transluminal angioplasty (PTA), which was complicated by complete occlusion of the vessel. To rescue the left kidney and treat the abdominal angina a bifurcated Dacron bypass was made from the right external iliac to the left renal artery and common hepatic artery. Collateral flow via a well-developed gastroduodenal artery ensured adequate perfusion of the superior mesenteric arterial network. We were hesitant to initiate anticoagulant treatment because of the patient’s high bleeding risk due to the recent major surgery, severe hypertension and renal insufficiency. Therefore, an MRDTI scan was performed to assess the age of the thrombus; this showed a low signal intensity of the aortic thrombus, indicative of chronic rather than acute thrombosis (). High signal intensity was found only in the aortic wall at the level of the left renal artery where the PTA had caused acute occlusion and bypass surgery had been performed ().\nBecause no acute thrombosis was identified, anticoagulant treatment was not started and antiplatelet therapy was continued. Abdominal ultrasonography after the bypass surgery showed open bypasses. The patient’s renal function gradually improved, allowing him to be discharged from hospital in good health. In view of the premature aortic thrombosis in the presence of an AAA, genetic testing was performed but this was negative for both connective tissue disorders and for syndromic thoracic aortic aneurysm. The patient was kept under close outpatient surveillance because of chronic persistent renal insufficiency, characterized by an eGFR of 60 mL/min. In the first year after presentation, there were no thrombotic or bleeding complications. |
We here report a 25-month-old male child born by full-term vaginal delivery presented to us with sudden onset of left-sided hemiparesis lasting 2 hours, not preceded by constitutional symptoms or seizure activity. He had similar episodes of transient hemiparesis on either side in past which relieved spontaneously without any neurological deficit. First episode of right-sided focal seizures occurred at age of 4 months. Frequency of seizures increased and child was started on antiepileptic drugs. Since age of 11 months, child had recurrent episodes of transient hemiparesis on either side with or without associated seizures, lasting 2-3 days and relieved by itself. There was history of developmental delay noticed after the age of 4 months and presently he could only walk with support corresponding to developmental age of 11 months. On examination he was afebrile with normal vitals. There was port-wine stain of 4 × 1 cm in an atypical site involving midline of forehead extending over nose []. His modified GCS was 13/15, had UMN type left facial nerve palsy with left-sided hemiparesis. Rest neurological and systemic examination was unremarkable. Contrast CT scan of brain showed linear hyperdense area in right high parietal lobe with gyriform enhancement along with enlargement of right choroid plexus and left frontal atrophy [Figures and ].\nEEG done urgently revealed no ictal activity, excluding postictal hemiparesis. PET scan revealed hypoperfusion and decreased metabolism in cortex surrounding the leptomeningeal angioma. Therefore the hemiparesis was presumed to be of vascular in origin. Child was started on aspirin to prevent further recurrences of these thrombotic events. On 3rd day of admission, child developed right-sided tonic clonic seizures with facial twitching lasting 15 minutes, and loss of consciousness followed by right-sided hemiparesis. Meanwhile his hemiparesis on left side improved. A concurrent EEG revealed epileptic activity with focus on left side, suggesting hemiparesis of postictal origin. Seizures were controlled with antiepileptic drugs. Child remained seizure-free thereafter until discharge and his residual deficits resolved within 24 hours. On 6th day of admission, child was discharged on anticonvulsants and aspirin prophylaxis. On follow-up up to 1 year, both of these hemiparetic episodes and seizure frequency decreased. Features suggestive of Sturge–Weber syndrome in this child were facial port-wine stain, focal seizures, hemiparesis or paroxysmal vascular events and neuroimaging findings. |
A 7-year-old girl was referred to Mofid Children’s Hospital with a large necrotic ulcer on his left upper chest, and generalized lymphadenopathy since two months ago ().\nThe ulcer was begun with a small pruritic papule on the left upper chest, enlarged and extended to the neck, and then ulcerated.\nShe had a history of toxoplasmosis presenting with cervical lymphadenopathy, and positive IgM and IgG anti-toxoplasma antibodies, at least 10 months before this ulcer appearance. An excisional biopsy was performed at that time, with result of preserved nodal architecture, follicular hyperplasia and no malignant cells and positive PCR for Toxoplasma gondii infection, so, she underwent following up until the skin ulcer appeared, and then, she was referred for more extensive work up.\nOn physical examination she had a large necrotic ulcer (20 × 10 Cm) with a noma appearance on the chest, and a hyperpigmented plaque (2 × 1 cm) on the abdomen, along with generalized lymphadenopathy and mild splenomegaly.\nAfter debridement of the chest wall ulcer and biopsies of abdominal skin and cervical lymph node, she was treated with ceftriaxone and clindamycin.\nSeveral aerobic and anaerobic microorganisms such as Staphylococcus saprophyticus, Lactobacillus spp., enterococci spp., Staphylococcus aureus and Peptococus niger were isolated from the ulcer culture.\nFurther investigation showed normal bone survey and bone marrow aspiration.\nThoracic computed tomography (CT) without contrast revealed a large lobulated mass in the left anterior chest wall, with extension to the axillary area and several cervical and left supra clavicular lymphadenopathies. (The CT scan was not available in this article). Abdominal and pelvic CT scan revealed mildly enlarged spleen and para-aortic lymphadenopathies.\nCT scan of the neck showed multiple extensive adenopathies predominantly in the left side of the neck and significant soft tissue swelling of the lower neck and chest wall. Brain CT scan was normal.\nRoutine laboratory tests were within normal ranges. Specific anti-toxoplasma antibody was detected by indirect immunofluorescent assay (IFA), shown a titre of 1:6400.\nHIV test was negative. Biopsies of cervical lymph node, abdominal skin plaque and chest wall ulcer debridement were compatible with anaplastic large cell lymphoma (). Immunohistochemical staining of cervical lymph node biopsy revealed tumour cells to be positive for leukocyte common antigen (LCA), ALK, CD30, CD3 and weakly positive for epithelial membrane antigen (EMA) (). The second time PCR for Toxoplasma gondii was positive in the lymph node specimens. The patient underwent chemotherapy according to oncologist recommendations. Two years follow up has shown no relapse or major complications. |
A 32-year-old woman with history of Hashimoto's thyroiditis and migraines presented in September 2019 with complaints of elevated heart rate. An episode of supraventricular tachycardia was captured on electrocardiogram, and she was referred to electrophysiology for ablation. She underwent a slow pathway ablation for AV node reentrant tachycardia in January 2020. Post ablation, there were no inducible arrhythmias. However, post ablation, she complained of elevated heart rate into the 150s. In April 2020, she underwent slow pathway ablation once more and an implantable cardiac monitor was placed. This implanted monitor revealed inappropriate sinus tachycardia, for which she was symptomatic. There was no workup done to assess for POTS. She was then prescribed ivabradine for inappropriate sinus tachycardia, which improved her symptoms. Unfortunately, she also experienced blurred vision associated with ivabradine, a side effect that she could not tolerate due to her work demands as an airfield controller.\nIn July 2020, she underwent a partial sinus node ablation, after her two slow pathway ablations. She remained symptomatic with an elevated heart rate. It was then recommended by her electrophysiologist that she undergo a complete sinus node ablation with pacemaker placement due to the nature of her vocation (which prohibits her from taking medications that could impair her performance).\nShe sought a second opinion from a general cardiologist on September 21, 2020. On evaluation, the increase in her heart rate from 108 bpm while supine to 125 bpm while standing was suggestive of a POTS diagnosis []. Furthermore, her supine norepinephrine was 677 pg/mL and her standing norepinephrine level was 855 pg/mL, suggestive of hyperadrenergic POTS (). After shared decision making, the patient elected to maximize medical management once more with metoprolol and ivabradine, as well as a supervised exercise program. She took a leave of absence from her work while on these medications and was monitored via a non-real-time heart monitor for two weeks.\nOf note, the patient tested positive for SARS-CoV-2 by PCR on September 24, 2020. She wore her heart monitor from October 6 through October 19, 2020. The results of her non-real-time heart monitor became available on November 10, 2020, and demonstrated bradycardia as low as 31 beats per minute and Mobitz II second-degree AV block (). Given this reading, she was advised to present to the emergency department, and she was subsequently admitted to the cardiology service.\nAt the time of admission, the patient reported poor compliance with ivabradine and metoprolol, only taking these medications occasionally in the four weeks prior to her admission. Her event monitor reported 92 episodes of Mobitz II second-degree AV block lasting a total of 6 minutes and 32 seconds over the course of two weeks ().\nDuring her admission, the patient was evaluated by the electrophysiology service and was noted to have dropped beats on telemetry but was asymptomatic during these. Because she had two slow pathway AVNRT ablations, her fast pathway likely had a longer refractory period and the dropped beats were likely secondary to transient AV block. Because she had normal PR and QRS intervals, malignant AV nodal and infranodal pathology was thought to be unlikely. Pacemaker placement was deemed unnecessary. Metoprolol and ivabradine were not felt to be the cause of her bradycardia as she reported poor compliance with these medications prior to her admission.\nAfter discharge, she was seen in the outpatient cardiology clinic on November 13, 2020, and her POTS was reevaluated. Her heart rate was 86 bpm while supine and 97 bpm standing. Further, her supine and standing norepinephrine levels had markedly decreased to 203 pg/mL and 419 pg/mL, respectively. The patient also reported subjectively feeling better since discharge. Heart monitoring in November 2020 recorded mostly sinus rhythm, with occasional periods of Type II AV Block, both Mobitz I and Mobitz II second-degree AV block. Since discharge, the patient has remained stable from a cardiac standpoint without requiring any chronotropic medications. |
A 63-year-old male patient with large anterior mediastinal mass was referred to our hospital for treatment. The patient was pathologically diagnosed as cervical schwannoma and underwent surgical resection twelve years ago. He had re-operation because of the recurrent neck tumor four years ago. No specific neural, cardiovascular and respiratory disfunction and neoplasms his history contained as well as his family history. The patient suffered from chest oppression and shortness of breath for four months, and these symptoms gradually became worse. The Preoperative CT confirmed that the patient was diagnosed as TM and large anterior mediastinal mass (Fig. ) Due to occasion of severe airway overreaction during the process of his endoscopy, fiber bronchoscopy was not finished.\nConsideration of potential risk from serious TM, the patient was intubated with guidance of fibreoptic bronchoscopy in the supine position, then underwent median sternotomy and tumor resection followed by tracheal suspension. The prime procedures of this surgery were briefly depicted by hand drawings (Fig. a: tumor site exposure; Fig. b: further sculpture of removed autogenous rib cartilage; Fig. c: anchoring malacial tracheal rings and membrane by fresh graft) and details of surgical procedure were as follows:\nStep 1: Tumorectomy. After medisection of sternum followed by opening pretracheal fascia, upper principal bronchus and frontage of cervical schwannoma were revealed. Along the line between the tumor and its adjacent tissue,the tumor was underwent entire resected.\nStep 2: Fabrication of scaffold. Partial autogenous rib cartilage was removed from 5th rib, and its top and bottom parts were penetrated with flexible steel needle to form two channels available for thread, which could manufactured a scaffold to anchor the extensive malacial tracheal rings and membrane (Fig. ).\nStep 3: Tracheal suspension. Free rib cartilage graft, fixed with bilateral tracheal rings, were deposited in front of malacial trachea by silk thread across the channels to cover the collapsed tracheal wall, so the malactic tracheal rings and membrane were elevated and pulled for enlarging the diameter of cartilaginous ring (Fig. a).\nAfter living a short period of mechanical ventilation with positive airway pressure, the patient was successfully extubated within 12 h after surgery. During his hospital stay, major postoperative complications didn’t occur, but mild pneumonia happened. The patient was discharged on the 16th day postoperatively. In the follow-up,the images showed that either cross section of intraluminal stenosis or collapsed segment of airway was remarkably relieved, and scaffold made by autogenous rib cartilage clinged to extratrachea stably. It was surprising that the graft finally integrated with tracheal wall (Fig. b). |
A previously healthy 69-year-old man presented with a history of vague left sided abdominal pain with vomiting for one-day duration. His past medical and surgical history was unremarkable. He was afebrile and did not have any gastrointestinal or urinary symptoms on admission. On the second day of his illness, he experienced worsening of abdominal pain focused on the right upper quadrant with tenderness. His white blood cell count (6.16 × 109/l) and C-reactive protein (less than 5 mg/dl) levels were normal. He had low platelets of 60 × 109/l. His serum electrolytes and renal and liver functions were within normal limits. The abdominal ultrasound scan did not show any significant findings. He was managed symptomatically and was kept under observation.\nHowever, on day 4, his clinical condition worsened with associated fever, jaundice, and reduced urine output. There was a tender hepatomegaly of 5 cm below the right costal margin. He also developed haemodynamic instability with a pulse rate of 126 per minute and systolic blood pressure of 90 mmHg. He developed acute respiratory distress syndrome and acute kidney injury with raised inflammatory markers, liver enzymes, and direct hyperbilirubinaemia. He was resuscitated, started on intravenous broad spectrum antibiotics, and was admitted to the intensive care unit for supportive management.\nThe repeated abdominal ultrasonography revealed a small subhepatic fluid collection and hepatomegaly. There was a hypoechoic lesion in segment V. Contrast-enhanced computed tomography of the abdomen and chest revealed moderate hepatomegaly with a 3 × 3 × 2 cm ill-defined, nonenhancing, hypodense area in segment V, compatible with a liver abscess. The lesion was communicating with the fundus of the gall bladder. There was pericholecystic fluid suggestive of acute cholecystitis. There were no gall bladder calculi, and the biliary tree was normal. There was bilateral moderate pleural effusion with basal lung consolidation. Therefore, the findings were compatible with an intrahepatic abscess secondary to type II gall bladder perforation following acute acalculous cholecystitis (Figures and ).\nHe recovered with supportive management, and as the abscess was small and he was clinically improving, the abscess was managed conservatively. His blood culture and culture of the pleural fluid were negative. Intravenous antibiotics were continued for 4 weeks after consulting the microbiologist. The repeated ultrasonography showed considerable reduction in the size of the abscess. He was hospitalized for a total period of 5 weeks. Although he was offered an elective laparoscopic cholecystectomy, he later defaulted follow up. |
A 70-years old right hand-dominant woman affected by Osteogenesis Imperfecta type 1 came to our emergency department complaining of severe left shoulder pain, swelling and functional limitation after a domestic fall. Before the trauma, the patient was used to live alone and was able to perform activities of daily living, with some limitation due to her genetic pathology. As a teenager, she was diagnosed with OI type I due to blue sclerae, several fractures (more than 30 reported events) that never required surgery. The patient has many other comorbidities including blindness secondary to glaucoma, juvenile cataract, retinal detachment, right-convex dorsal scoliosis and osteoporosis treated with annual infusion of zoledronic acid.\nClinical examination failed to show any neurovascular deficits or other injuries. Plain radiographs of the shoulder detected a 4-fragments proximal humerus fracture (). A CT scan was performed and confirmed the displaced multi-fragmented fracture of the proximal humerus, with subluxation of the humeral head ().\nA reverse shoulder arthroplasty was preferred over a syntesis because of the fragmentation and the poor quality of the bone stock. Appropriate informed consent was obtained. The patient was placed in beach-chair position and a delto-pectoral approach was performed. The main cephalic fragment was identified and removed without the need of further humeral head osteotomy. A small size metallic glenoid component fixed with two screws and a 40 mm glenosphere were implanted. A preventive bone cerclage on the proximal humeral diaphysis was performed to avoid further rim fracture propagation. A medium size cementless SMR reverse shoulder arthroplasty prosthesis (Lima Corporate, San Daniele del Friuli, Italy) was implanted as our experience leads to prefer (). Postoperative plain radiographs were obtained ().\nPostoperative course was uneventful with the patient discharged at home three days after the operation without complications. The involved arm was hold in brace during rest for three weeks with early passive and assisted active mobilization allowed.\nAfter one month, the patient was able to achieve 40° active abduction and flexion, 10° active extra-rotation and internal rotation at the trochanteric region with pain. At 6-months follow-up, the patient improved the range of motion with decreased pain intensity. She was able to perform 75° active abduction and 85° active flexion, 30° active extra-rotation and intra-rotation to lumbar spine. Usual radiographic controls showed good positioning of the implant components without signs of osteolysis or mobilization (). |
We present a case of a 50-year-old Caucasian male who presented to the emergency department with complaints of lower abdominal pain, fever and sweating. On examination, the patient had tenderness to palpation in the right iliac fossa, with significant rebound tenderness and guarding. Body temperature was recorded at 38.5°C.\nThe patient's past medical history was significant for an incident of similar pain six months prior to presentation. He was diagnosed with sigmoid diverticular disease confirmed by computed tomography (CT) scan and managed conservatively. A subsequent colonoscopy confirmed the diagnosis, and did not reveal any other colonic pathology.\nFurther investigations revealed a raised white blood cell count of 16,000 per microliter. CT scan of the abdomen and pelvis disclosed evidence of extensive free gas under the right dome of the diaphragm confirming suspicion of a perforation. Fluid-filled prominent loops of small bowel were noted. However, none of them were dilated to suggest obstruction. Mild bowel thickening was also noted around the cecum. Pneumatosis coli suggestive of ischemic bowel, extending from the cecum to the proximal ascending colon was seen. A blind ending structure with calcifications was also seen (Figure ). Other significant findings on the scan included a liver cyst, consistent with the patient’s previous CT scan, and consolidation at the base of the right lung.\nSubsequently, the patient underwent an emergency laparotomy and a diffuse four-quadrant peritonitis was seen. A giant perforated MD, 80 cm proximal of the ileocecal valve was identified as the cause of the peritonitis. The giant MD measured approximately 10 cm in length and 2.5 cm in width (Figure ). A small perforation at the tip of the MD was observed. No other intraabdominal pathology was identified, in particular, there was no evidence of colonic ishchaemia. Resection of the segment of small bowel bearing the MD was performed with a side-to-side stapled anastomosis.\nPathologic examination showed a T-shaped length of bowel, 6 x 5 x 3 cm in maximal dimension, with a 3 x 5 x 6 cm portion of mesentery attached. The presumed diverticulum was 5 cm long with a maximal inner circumference of 5 cm. The perforation measured 0.2 cm and the lumen of the specimen was stained green. No heterotropic tissue was identified.\nPost-operative recovery was uneventful and the patient was discharged on oral antibiotics. |
A 32-year-old African American man was admitted to the hospital with bilateral lower extremities weakness and inability to walk for 2 days. The patient states that he had progressive bilateral lower extremities weakness for the last 3 months, which is getting worse to the point that he is unable to walk. He also complained about slurred speech and deviation of the left eye for the past 2 weeks. The patient had a history of hypothyroidism and acquired immune deficiency syndrome with CD4+ T-lymphocyte cell (CD4) count <20 and plasma HIV RNA (viral load) of 206,000 6 months before the admission. Physical examination was remarkable for dysarthria, horizontal nystagmus bilaterally, dysmetria on finger-to-nose examination, and unsteady gait. Patient initial laboratory findings have been mentioned in Table .\nThe patient underwent MRI, which showed abnormal non-enhancing hyperintense T2 hypointense T1 signal extending from central cerebellar white matter, superior cerebellar peduncles, near entirety of the pons, cerebral peduncles, and left cortical spinal tract. The patient underwent lumbar puncture. The cerebrospinal fluid analysis was unremarkable.\nThe patient became hypoxic, hypotensive, and developed altered mental status on day 5 of admission. The patient was intubated and started on broad-spectrum antibiotics. The patient was transferred to the ICU for further management of acute hypoxic respiratory failure and septic shock. VRE peri-rectal surveillance cultures were sent at the time of arrival to ICU and were found to be negative. The patient failed multiple weaning attempts and underwent percutaneous tracheostomy and percutaneous endoscopic gastrostomy tube placement.\nThe patient developed severe watery diarrhea during the third week of hospitalization. The patient's stool was sent for further analysis. C. difficile glutamate dehydrogenase and toxin B tests came back positive, and the patient was started on oral vancomycin for 14 days. Patient diarrhea persisted despite completing a 14-day course of antibiotic. Patient developed bleeding per rectum on the last day of antibiotic course. Digital rectal exam showed solid brown stool with specks old blood, no fresh blood. The patient was scheduled for a colonoscopy. Colonoscopy showed a diffuse area of severely altered vascular, congested, erythematous, friable with contact bleeding; hemorrhagic, inflamed, nodular, and ulcerated mucosa was found in the sigmoid colon, in the descending colon, and in the transverse colon. This has been shown in Figures , . Biopsies were taken with cold forceps for histology.\nThe patient was started on oral and rectal vancomycin with metronidazole for a total of 14 days. The patient's blood culture and urine culture showed no growth of any organism. Tissue culture grew VRE. An echocardiogram showed no vegetation. Infectious disease was consulted, and the patient was advised to continue with oral and rectal vancomycin with metronidazole. The patient's clinical condition improved, and his diarrhea resolved. The patient was discharged to a nursing home with a follow-up appointment. |
A 29-year-old pregnant woman visited the local hospital to confirm her pregnancy. On ultrasound the pregnancy was confirmed (6 weeks), and a pelvic mass was incidentally detected without any symptoms. The patient was transferred to our tertiary hospital for evaluation of the incidentally detected pelvic mass. Ultrasound revealed that there was an about 8 weeks sized embryo in the uterus and a 10 cm sized hypoechoic mass on the left, posterior to the uterus. But it was difficult to distinguish the mass from an adnexal mass, leiomyoma (intraligamentary), or other tumor by the ultrasonographic findings. We checked her serum tumor marker, and performed magnetic resonance imaging. Serum CA-125, CA-19-9, CA-72-4, and carcinoembryonic antigen were within normal range. A pelvic MRI without contrast media was checked at 11 weeks of pregnancy. Magnetic resonance imaging (MRI) revealed an about 10×10 cm sized mass in the retroperitoneum, and an anteriorly displaced uterus and rectum. This mass had high signal intensity on a T2-weighted image, and there was extension to the central spinal canal through the S3 left neural foramen. The MRI suggested a benign retroperitoneal neurogenic tumor (). After we consulted with the urology and neurosurgery departments, we decided to remove the retroperitoneal mass after delivery or at the time of the cesarean section. The patient underwent routine antenatal care without complications or symptoms, and there was no growth of the retroperitoneal mass on ultrasonography. The patient delivered a male baby with body weight of 2,940 g by cesarean section at 38 weeks of gestation and the retroperitoneal mass was excised with assistance from the urology team. The intraoperative frozen biopsy suggested a ganglioneuroma. Grossly, the resected mass was multiply fragmented, measuring 10×7×6 cm in the largest piece, partly encapsulated, and showed a whitish yellow and gelatinous cut surface. The histology report confirmed the ganglioneuroma (). We could not completely resect the deepest portion due to the possibility of injury to normal tissues (nerves and vessels). The patient was discharged on the 7th postoperative day in good condition. We planned regular follow-up without additional treatment. She will be checked with a computed tomography three months later. |
A 13-year-old Caucasian female student reported to the oral and maxillofacial surgery clinic with the chief complaint of purulent discharge oozing just distal to the upper right second molar, with a bad taste and foul odour, that started two weeks before seeking treatment. Upon clinical examination, the patient had no intraoral or extraoral swelling, and there was a full complement of teeth on that arch except for teeth #18 and #28. All teeth were firm, vital and non-carious. A panoramic radiograph revealed ectopic eruption of the right maxillary third molar in the maxillary sinus with hyperdense lesion surrounding its crown and obliterating the sinus cavity (\n).\nFurther radiographic investigation using cone beam computed tomography (CBCT) determined the exact location of the maxillary molar and the lesion extension since the molar was seen in close proximity to the infraorbital rim. CBCT showed the ectopic third molar with incompletely formed roots located in the posterosuperior aspect of the right maxillary sinus with a close approximation to the orbital floor superiorly and pterygoid plates posteriorly (\n). The third molar was surrounded by a well-defined corticated hyperdense lesion measuring 23×36×35mm, occupying almost the whole cavity of the right maxillary sinus and causing mediolateral expansion of the alveolar ridge. The CBCT also revealed the destructive effect of the associated pericoronal lesion on the right maxillary sinus floor and buccal cortical plate, distal to tooth #17, causing oroantral communication, which explains the reason for the purulent discharge, the chief complaint of the patient (\n). Differential diagnosis of the detected lesion was dentigerous cyst, odontogenic keratocyst or Gorlin cyst. Based on the clinical and radiographical examination, the surgical removal of the ectopic third molar along with cyst enucleation was planned through an intraoral approach under general anaesthesia induced via nasopharyngeal intubation. Induction of anaesthesia was achieved with propofol 2mg/kg, fentanyl 1µgm and atracurium 0.5 mg/kg, then maintained with propofol infusion 200µgm/kg/min, fentanyl 1µgm/kg/hour and atracurium 0.1 mg/kg every 20 minutes.\nAn oral and maxillofacial surgeon with 15 years of experience performed lateral sinus antrostomy, utilizing a standard Caldwell-Luc approach, with a bony window created in the anterolateral wall of the maxillary sinus (\n). An incision was made through the Schneiderian membrane to enter the maxillary sinus. The cystic lining was identified, and the pus was drained prior to the complete removal of the cystic lining and extraction of the ectopic maxillary third molar (\n). The antrum was thoroughly irrigated, and the cystic lining was placed in a 10% buffered formalin solution for subsequent histopathological examination and final diagnosis. The flap was closed with chromic catgut sutures with good approximation of edges. Postoperative antibiotics were given to the patient in form of 500 mg of amoxicillin every eight hours for a minimum of five days with an analgesic (325mg acetaminophen every four hours per day), also one dose of betamethasone 4 mg was given to prevent postoperative edema of the cheek, and the patient was instructed not to blow her nose for two weeks.\nHistopathologic examination reported a cystic cavity lined by a thin non-keratinized stratified squamous epithelium. Part of the epithelial lining showed hyperplasia due to inflammation and long-standing lesion, and a connective tissue wall infiltrated with chronic inflammatory cells and composed of fibroblasts, collagen fibres and blood vessels, suggesting an infected dentigerous cyst (\n).\nA panoramic radiograph and CBCT were performed three months after the surgery. CBCT axial cuts revealed some bone formation in the mediolateral dimension when compared to the preoperative radiographs, which indicates that the bone is in the healing process (\n).\nHealing appeared to be better clinically than radiographically, the bone requiring longer time to form and be detected radiographically. There is improved bone healing in younger patients, and so we expect a quick healing process with our patient.\nPostoperative CBCT scans showed opacification and mucosal lining thickening of the right maxillary sinus, as well as continued discontinuity of the posterolateral floor and part of the anterior wall of the right maxillary sinus (\n). |
A 72-year-old postmenopausal woman presented to the emergency room with complaints of acute abdominal pain and purulent vaginal discharge. The patient had 2-month history of brown purulent vaginal discharge accompanied with a foul smell; otherwise there was no fever or abdominal pain.\nShe stated that the abdominal pain started suddenly without any warning earlier in the evening, and she had never experienced abdominal pain or discomfort similar to this previously. In the past, she denied any significant change in bowel or bladder habits. Past medical history was significant for hypertension and without any distinct surgical history. She denied any tobacco, alcohol, or drug use, and she did not have any obvious occupational exposures or chronic family illnesses.\nOn physical examination, she was groaning with extreme pain, and her vitals were as follows: temperature of 36.7°C, blood pressure 101/70 mm Hg, heart pulse of 110 beats/min, respiratory rate of 20 breaths/min, and oxygen saturation of 97% without the addition of supplemental oxygen. Vaginal examination only showed rotten discharge without cervical and vaginal anomalies. Initial laboratory results showed a white blood count of 9.1 × 109/L and C-reaction protein 116.4 mg/L.\nThere was a lot of purulent discharge on her vaginal examination, with cervix lifting pain. Because of the muscular tension with obvious pain, palpation of atrophic uterine was not clear. Transvaginal ultrasound and the abdominal computed tomography (CT) showed generalized fluid and free air in abdominal cavity.\nThe patient was in a septic condition, and was taken to the operating room emergency for exploratory laparotomy, where, upon entering the peritoneal cavity, there was 500 mL of purulent fluid encountered, and 50 mL pus was sent for culture and sensitivity. The small bowel and large bowel were examined in their entirety without significant pathology noted. Thorough examination of the pelvis revealed a hyperemic uterine fundus with associated necrosis; about 1.5 × 2 cm crevasse was present on posterior wall of the uterus, which was close to the fundic uteri (Fig. ). Both parametriums were thickened and inflammatory changes were present. Both fallopian tubes and ovaries were normal. Consequently, the patient was underwent total hysterectomy with bilateral salpingo oophorectomy, then peritoneal toileting was done over and over again, and finally, 1 intra-abdominal drain was kept. The patient received imipenem anti-infective therapy for 6 days. Culture of the pus showed growth of streptococcus pharyngitis, sensitive to imipenem. On the eighth day after operation, the patient's incision presented with fat liquefaction; then the dressing was changed twice a day during more than 20 days and finally the incision healed well without suture. Histopathological studies revealed suppurative endometritis, myometritis, and abscess formation, with no evidence of malignancy. The patient was discharged more than 1 month postoperative day.\nThis report did not involve any ethics. However, the patient's informed consent was obtained, and the corresponding data were analyzed retrospectively. |
Here we report a 48-year-old male case of EAML of the kidney which is a relatively newly described renal neoplasm and is closely related to the more common classic AML of the kidney and perivascular epithelioid cell neoplasm of extra-renal sites [].\nThe ethical committee of our institute gave us permission for the current publication. The patient gave informed consent and is aware of this case report.\nThe patient arrived at our institute, complaining of severe pleuritic chest pain for approximately three months. He was not a smoker and did not consume any illicit drug or alcohol. He had never experienced urinary problems. His past medical history was unremarkable. He had no history of previous surgery.\nOn physical examination, a huge abdominal mass was palpated in the left flank. Incredibly, he did not feel this enormous mass earlier which may suggest the rapid growth of the lesion.\nThere were no abnormal findings in laboratory studies (Including complete blood count, urine analysis and stool examination) except for a slightly elevated Erythrocyte Sedimentation Rate (ESR). Renal and liver function tests were within normal limits. He was admitted for further diagnostic evaluations. The chest Computed Tomography scan (CT-scan) revealed a massive pleural effusion in the left hemithorax. Abdominopelvic CT-scan showed two infrarenal heterogeneous masses arising from the medial pole of the left kidney with significantly increased vascularity. Both masses contained adipose tissue, which was suggestive of either AML or a well-differentiated liposarcoma ().\nPurified Protein Derivatives (PPD) skin test and cytopathologic analysis of pleural fluid were done in order to rule out tuberculosis and malignancy respectively. The aspirated fluid only contained inflammatory materials.\nThen, he underwent a radical left nephrectomy which is the treatment of choice for huge AML of the kidney based on the literature. Surgery was performed by an experienced urologist, under general anesthesia, and via a midline incision. After exploring the abdominal cavity, two huge masses attached to the middle pole of the left kidney were observed (). Massive intra-operative bleeding led us to infuse one unit of homologous cross-matched packed cells. Masses were measuring 32*22*8 cm and 2*1*0.5 cm, with lobulated surfaces and covered by a thin capsule. On cut sections, both masses contained soft, creamy yellow tissue comprising a growing adipose tissue. The smaller mass was separately located into perinephric fat while the greater one, was invaded renal sinus fat. After the operation, he was admitted to the Intensive Care Unit for 4 days and then to the urology ward for a week. The post-operative period was uneventful and the scar healed perfectly.\nThe specimen was sent for histopathologic evaluations. The larger mass contained necrotic tissue and atypical epithelioid cells with the mitotic rate of 0–1 IN 10 HPF. The atypical mitotic figure was not identified. These findings completed the gross characteristics to confirm the diagnosis of EAML, possibly associated with tuberous sclerosis. ()\nLastly, by the consult of an expert dermatologist, the association with Tuberous Sclerosis Complex (TSC) which presents with multi-system progressive tumors, intractable epilepsy, and mental retardation, was ruled out. He had no skin lesion suggestive of sclerotic changes or a history of neurological manifestations of TSCs.\nNo specific concurrent treatment was applied for him except antibiotic (1 g of Cefazolin every 6 h) and analgesia (when required) for two weeks postoperatively. He was under our close observation during his stay at the hospital. Renal Function Test and Complete blood count were evaluated every other month. His abdominal pain was completely relieved after the surgery. At a two-year follow-up by CT-scan. There has been neither metastasis nor recurrence during the 24-month period since resection (). |
A 14-year-old boy presented to the emergency department with a painful left wrist following a fall with his bike after a low-velocity collision with a car. The exact mechanism of trauma could not be described by the boy, but most of the impact was received on the left hand and wrist. On clinical examination, there was swelling but no gross deformity of the left wrist. The wrist was diffusely tender on palpation and range of motion (ROM) was limited by pain. There were no neurovascular deficits and the skin was intact. Anteroposterior and lateral X-rays of the left wrist showed a displaced Salter-Harris type IV fracture of the distal ulna (Figures and ). Because of this uncommon fracture type with displacement of the fragment, subsequent computed tomography (CT) was performed. CT showed a displaced fracture through the epiphysis and metaphysis on the volar side of the distal ulna with an intraarticular step of 3 mm (). The patient's wrist was immobilized in a below-the-elbow plaster at the emergency department. Reduction and stabilization were, for practical reasons, planned 5 days later.\nThe procedure was performed under general anaesthesia. First, an attempt at closed reduction was made, with pressure applied to the volar aspect of the wrist under radioscopic control. This resulted in a partial but insufficient reduction of the fracture, which led to conversion to an open reduction and fixation procedure. The fracture was approached through an ulnovolar incision. The fragment was reduced under radioscopic control using Kirschner pins to manipulate and lever the fracture fragments. Three Kirschner pins were placed parallel to the physeal plate. Two pins were placed in the coronal plane with one through the proximal fragment and one through the distal fragment. A third pin was drilled in the anteroposterior plane through the distal fragment (). The patient was immobilized in an above-the-elbow cast for two weeks.\nFollow-up after two weeks showed a favourable clinical evolution with maintained position of the fracture fragments on X-ray (). To encourage mobilization of elbow and fingers, a wrist brace had to be worn for three more weeks. Rotations had to be avoided. One K wire was removed after six weeks and automobilization was started. Evaluation four weeks later showed a good range of motion in flexion-extension and rotations of 45°. To improve supination, physiotherapy was added. Three months later, the second pin was removed because of migration.\nAt follow-up 6 months after injury, there was a full range of motion of the wrist compared to the other side with a similar grip strength on both sides. X-ray of the wrist showed the fracture was fully healed with no evidence for premature fusion of the physis or growth arrest (). X-ray 1 year postoperatively showed complete closure of the physis with development of a negative ulnar variance (). Further follow-up with radiographic control will be provided until skeletal maturity. |
A 40-year-old nulliparous woman with no past medical history, other than endometriosis, presented to the emergency room with severe chest tightness of one day duration. She described chest tightness while exercising on a bicycle after 10 minutes. The next day she developed a severe hacking cough at work during a conference call. The cough was associated with disorientation and severe chest tightness. This prompted her to present to the emergency department in February 2018. The patient is a pharmacist, nonsmoker, and denies illicit drug use or recent travel. She had endometrial laser ablation with myomectomy in 2006. Hormonal contraceptives have been used since 2004 and were stopped three months before presentation in hopes of conception. Her last menstrual period was four days before the onset of symptoms. On physical examination, she had chest tightness localizing to the right side and decreased right sided breath sounds. All the routine laboratory work and vital signs were normal. The CXR showed a large right spontaneous pneumothorax with what approved to be a 5.6 cm pleural mass at the right lung base ().\nFollowing the pneumothorax diagnosis, the patient underwent emergent right thoracostomy with pigtail catheter placement. A repeat CXR revealed marked re-expansion of the lung but persistence of the right cardiophrenic opacity of unclear etiology. A follow-up CTof chest showed a 33 mm diaphragmatic defect with a 5.8 x 4.6 x 3.9 cm area of herniated liver corresponding to the presumed pleural mass ().\nFollowing complete thoracic imaging the patient underwent video-assisted thoracoscopic surgery (VATS), mechanical pleurodesis, and open repair of the right diaphragmatic defect by Dr. Emily Cassidy (Figures and ). Intraoperatively, the lungs appeared grossly normal. An obvious diaphragmatic defect was noted in the posteromedial portion of the central tendon of the diaphragm with a sizable protrusion of liver in to the chest cavity. There was an attempt to dissect liver adhesions from the diaphragm. Extensive liver adhesions forced conversion of the right VATS to a posterior lateral muscle sparing thoracotomy. Electrocautery and blunt dissection were used to separate the liver and diaphragm. Once the liver hernia was completely reduced, the diaphragm was repaired using multiple interrupted prolene pledgeted horizontal mattress sutures. Later, right parietal pleurectomy was performed by scoring the pleura posteriorly, anteriorly throughout the entire chest cavity. At the apex as well as the base, where pleurectomy was difficult, mechanical pleurodesis using a Bovie scratch pad was perfomred. A 24-french chest tube was placed at the apex of the chest cavity and 24-french blake was placed at the level of diaphragm.\nIntraoperatively, an endometrial implant (blue berry spot) was noted on the chest wall (), but the endometrial implant was not successfully biopsied for pathology evaluation due to the extent of liver adhesions and due to conversion of procedure from VATS to open thoracotomy.\nOn postoperative day three, the patient began her menstrual cycle. She was evaluated by a gynecologist consultant who recommended hormonal therapy, leuprolide a Gonadotropin releasing hormone (GnRH) analogue, to begin 2-3 weeks postoperatively for a period of 6-12 months for hormonal suppression to reduce the risk of recurrent pneumothorax. GnRH analogs are highly effective at suppressing ovarian hormone production and inhibition of growth of endometrial tissue. Due to a persistent air leak, the patient's chest tube was transitioned to a Heimlich valve to facilitate home discharge. We believed that the persistent air leak indicated there was some minor defect in the visceral pleura that was too small to identify intraoperatively. The patient was discharged on postoperative day eight and was seen as an outpatient by the cardiothoracic surgeon. At this time, there was resolution of air leak and removal of the chest tube. Patient was also seen after two months in a primary care clinic for follow-up visit with no issues. |
A 58-year-old woman was admitted to the hospital with severe back pain accompanied with nausea and vomiting. Because of the back pain, she underwent a selective nerve root block in the lumbar spine at another hospital. After the spinal procedure, the patient felt more severe back pain, newly developed pain, and weakness in her right leg. She had intact voiding sensation and no difficulty voiding at the time of admission. The paraparesis was checked during a neurologic examination. In the right lower extremity, ankle dorsiflexion (Gr III/IV+) and toe flexion and extension (IV-/IV+) weaknesses were prominent compared with those of the left lower extremity. Deep tendon reflex was absent on the right knee, and the "extensor toe sign" was negative bilaterally, without clonus. Sensory impairment to the pain was noted on the L5 dermatome of the right calf.\nAn MRI revealed a ventrally located intradural mass extending from T10 to L2; the mass was iso- to hypointense on T1-, and the hyperintense portion on the T2-weighted images was compatible to the bleeding inside the tumor. The lesion was heterogeneously enhanced with contrast administration ().\nOne day after her admission, the patient experienced worsening of her neurological status with weakness of the lower extremities and the loss of anal tone and voiding sensation. She underwent an emergency operation with high-dose methylprednisolone therapy.\nA laminectomy from the T10 to L2 levels was performed using a posterior midline approach. After the dura was opened, the mass was exposed to be dark and oil-colored, and a chocolate-colored subdural hematoma was observed above and below the mass (). The mass was not connected to the nerve roots. However the mass was adhered to the ventral dura. Therefore, the mass was removed en bloc under an operating microscope, with coagulation of the dural attachment. A frozen section biopsy of the mass was performed, and under microscopic examination, it was compatible with a meningioma. The dura was closed in a watertight fashion with bleeding control. Although no wave was observed on intraoperative monitoring at the beginning of the surgery, some wave was observed at the end of surgery. The pathologic report confirmed the meningioma was a fibrous type WHO grade I/III tumor.\nDuring the postoperative course, the status of motor and sensory was improved. The patient felt bladder fullness and anal contraction after 2 postoperative days. She was transferred to the department of rehabilitation for an intense rehabilitation program. Two months post-surgery, the patient was freed from the Foley catheter, and her urinary symptoms recovered without urinary incontinence, frequency, residual urine sensation, and urgency. |
An 86-year-old multiparous Japanese woman with an unremarkable medical history was transferred to our hospital after a traffic accident (Table ). She was hit by a car while walking at a crosswalk. On presentation to our emergency department, she complained of pain in her buttock. Her Glasgow Coma Scale score was 15/15. Her blood pressure was 100/53 mmHg, heart rate was 93 beats/minute, respiratory rate was 15 breaths/minute, and oxygen saturation was 100% while breathing 2 L/minute of oxygen. A whole-body computed tomography scan revealed fractures of her left pubic bone and sacrum and a hematoma with contrast extravasation in front of the sacrum (Fig. ). Her blood pressure then suddenly dropped to 67/38 mmHg secondary to hemorrhagic shock. Rapid resuscitation with fluids and blood was performed. We attempted to perform transcatheter arterial embolization (TAE). Based on angiographic findings (Fig. ), bilateral internal iliac artery embolization was performed with gelatin sponge particles. She received 560 ml of packed red cells, 480 ml of fresh frozen plasma, and 200 ml of platelets, and she became hemodynamically stable.\nAfter admission to our intensive care unit, she developed shaking chills and a high fever. She was hemodynamically stable the following day. However, reddish urine was observed. Her serum lactate dehydrogenase level was extremely high, and fragmented red blood cells were present on peripheral blood smears. On the third day of hospitalization, despite fluid challenges and the use of diuretics, she became anuric and thus underwent hemodialysis. However, she developed severe delirium and was intubated under sedation. She was not diagnosed as having TTP at this point because her platelet count was not reduced despite the worsening of her hemolysis. Her prothrombin time and activated partial thromboplastin time were normal, and her fibrin degradation products were returning toward the normal concentration within 3 days of admission; therefore, disseminated intravascular coagulation (DIC) was excluded.\nOn the fifth day of hospitalization, her platelet count, measured by a different hemocytometer, was very low. Her fragmented red blood cell concentration measured by visual judgment based on the International Council for Standardization in Haematology (ICSH) reference method was 28.8% (Fig. ). We finally confirmed the diagnosis of TTP based on the classic pentad of TTP and began plasma exchange.\nAfter retrospectively checking her platelet count, we found that it had decreased to 55 × 109/L on the second day of hospitalization (Fig. ). The presence of many fragmented red cells is often associated with a spurious increase in the platelet count because the fragmented red cells are erroneously measured as platelets by automated blood cell counters.\nPlasma exchange was continued for 5 consecutive days. Her clinical course dramatically improved in just a few days, and her platelet count increased. She was weaned from hemodialysis on the 15th day of hospitalization. She recovered fully and was discharged from our hospital on the 31st day of hospitalization. The ADAMTS13 activity measured by an enzyme immunoassay on the third hospital day was not reduced (65%), but a direct Coombs test was negative and the complement factor level was normal. |
Case 4 was a 58-year-old female living in rural Moshi who started ART in 2012 and was enrolled in the RTMM arm. According to RTMM, her level of adherence during the first 2 study months was 47%. After the nurses showed her this level of adherence during a feedback session, she said,\nI never missed the medication intakes. I think the RTMM was not working properly. Therefore, I want to be provided with a new device.\nThe study nurse exchanged the old device with a new one. In the next feedback session, her level of adherence according to RTMM was still low, at 20%, despite her insistence that she never missed a medication intake.\nDuring the interview with the study investigator, the participant mentioned that the device was easy to use, but charging it was a challenge because it did not have an alarm to indicate a low battery. At the beginning of the study, this participant lived in a rural area, where her adherence level was low, according to RTMM. After 10 months, she moved to an urban area and the device started to send daily signals, showing device openings indicative of a high level of adherence. The participant explained,\nI have never missed my medication intakes; therefore, I was surprised why the device was not recording the openings. After I moved from my old house in the village, the device started to record the openings, so I thought the problem was network coverage.\nThe participant was happy to be monitored in real time, as it made her feel cared for. Privacy concerns were not an issue for this participant as she had disclosed her HIV status to her family.\nFor this participant, the main reason for the discrepancy between self-reported adherence and adherence generated by the device seemed to be related to adequate power, charging, and availability of network coverage. |
A Folliculotropic Mycosis Fungoides was diagnosed in a 58-year-old male patient in 1997 and treated with local chlormethine between 1998 and 2006.\nIn 2006, MF progressed toward a tumoral form with infiltrating plaques and nodules all over his body, the most important being an exophytic one arising from the nasal region. No Sezary cell was noted in the blood smear. A biopsy of cutaneous tumor was performed and the pathologist confirmed a localization of tumoral nontransformed MF.\nBetween 2006 and 2014 the patient received several systemic treatment lines including methotrexate, PUVA therapy, pegylated liposomal doxorubicin, polychemotherapy, histone deacetylase inhibitors, and anti-CCR4 monoclonal antibody. All these drugs were without long-lasting effect and tumoral lesions progressed including the tumoral lesion of the nose (Figures and ).\nThe extent, progression, and resistance of his skin lesions gave a palliative intent to his treatment project. The patient reported that the aspect of his nose refrained him from interacting with people, which led him progressively to get socially isolated. He reported difficulties in interacting with his family members especially with his young grandchildren. Histology from the nasal lesion was obtained and showed classical Mycosis Fungoides of granulomatous type without transformation.\nHe was then referred to our radiotherapy unit in August 2014. We opted for a conventional radiotherapy with 12 MeV electrons and 6 MV and 18 MV photons. The patient received 36 Gy in 18 fractions (2 Gy per fraction, 5 fractions per week).\nLesions disappeared completely within a few weeks (Figures and ). The patient presented acute grade I radiodermatitis (NCI CTCAE Version 4.03) which resolved spontaneously. No clinical relapse had been noted 3 years after the treatment.\nAs the physical appearance of the irradiated nose got better the patient reported a psychological relief. The recovery of the normal aspect of his nose helped him resume some of his social activities, use public transportation, and better interact with friends and family members. |
A 65-year-old man was admitted to our hospital for elective high tibial osteotomy after the diagnosis of right knee osteoarthritis (Figure ). No other medical history was found. Surgery was performed under general and spinal anesthesia the day after admission. Intraoperatively, the medial osteotomy wedge was opened to 20°, and the desired correction was achieved. Postoperatively, non–weight-bearing mobilization was advised during 4-week rehabilitation. The patient was permitted to walk on crutches and was not required to take anticoagulants.\nOn postoperative day 12, he developed bilateral arm paresthesia. Urgent brain CT was performed, and no evidence of stroke was found. The patient did not appear to be dyspneic. Symptoms persisted until the next day, and reexamination was performed. The patient's oxygen saturation was 89% on room air, with normal breath sounds. His heart rate was 110 beats per minute. Blood pressure was not obtainable in either upper limb and was 130/80 mmHg in the lower limbs. The radial artery pulse was not palpable. Electrocardiogram revealed sinus tachycardia. Enhanced CT revealed emboli in the bilateral pulmonary and subclavian arteries and deep vein thrombosis (DVT) in the left lower limb (Figure ). In addition, magnetic resonance imaging revealed a tiny brain infarction (Figure ).\nPhysical examination and diagnostic imaging suggested that the patient's bilateral arm paresthesias were caused by arterial emboli in the bilateral subclavian arteries. An inferior vena cava filter device was inserted to prevent future emboli, and anticoagulation with intravenous heparin and urokinase was initiated. The levels of proteins C and S, antithrombin III, factor V Leiden, lupus anticoagulant, cardiolipin antibody immunoglobulins, prothrombin, and homocysteine were examined to assess the possibility of a hypercoagulable state, but all were normal. Intravenous anticoagulation was continued for 10 days with urokinase and 12 days with heparin, based on the follow-up results of enhanced CT. A direct oral anticoagulant was initiated when all intravenous drugs were discontinued.\nOn postoperative day 30, transesophageal echocardiography revealed a PFO during the Valsalva maneuver. On the following day, CT showed no arterial or venous emboli, and bilateral radial pulses could be palpated, and symptoms had completely resolved. The patient recovered uneventfully and was discharged from our hospital on postoperative day 44. |
A female patient aged 1 year 8 months old presented to the emergency department after falling on an outstretched hand. On radiograph imaging (Figure ), a supracondylar fracture Gartland type III was diagnosed. On her first neurovascular survey, no abnormalities were noticed. The patient was operated on the next day. After closed reduction, two 1.4-mm Kirschner wires in a cross-configuration were placed to stabilize the fracture. On the lateral side, the wire was inserted percutaneously, and the medial pin was placed under direct bone vision. Good and stable fracture reduction was achieved (Figure ). After surgery, the patient was placed in a posterior splint with 60° flexion at the elbow. A neurovascular examination was done, but no neurologic deficit (motor or sensory) was identified, although no documentation of specific sensation testing is found. Active finger movements were observed. The child was released from the hospital on the second day after surgery, with an outpatient clinic follow-up visit programmed 3 weeks after surgery.\nNo motion was allowed at the elbow joint for all this period. The pins were removed after 3 weeks at the outpatient clinic visit (Figure ).\nAbout 6 weeks after surgery, the child began oral antibiotic treatment (Augmentin), which was prescribed by a physician in the community because of a skin infection on the fifth fingertip, on the fractured side. The mother saw the child bite her finger continuously. After a few days of treatment, the child was referred to the hospital because of lack of response to the antibiotic treatment. On her admission to the pediatric orthopaedics unit, amputation of the distal phalanx was noted, and radiograph imaging confirmed the diagnosis (Figures and ). A child abuse workup (total body examination and a social worker inquiry) that was done after admission ruled out this option. We started treatment with systemic intravenous antibiotics (Cefamezin 30 mg/kg) and topical treatment (Synthomycin).\nOn her second day in the hospital, a consultation was held with a pediatric neurologist to rule out a systemic disease. Our clinical suspicion was of an ulnar nerve injury sensory only without any motor deficit. Nerve conduction velocity (NCV) under light sedation was performed, which confirmed partial ulnar nerve injury.\nThe wound infection signs improved, and the wound began to heal under intravenous antibiotic treatment. An extra protection to the hand was done with a bulky bandage that the patient could not remove. The child was discharged from the hospital after 6 days of treatment, and she wore a mitten for about 1 month, 24 hours a day. She was kept under close clinical follow-up at the outpatient clinics once weekly. The wound healed. A second NCV was done after 3 months, and full nerve recovery was noticed. The patient did not bite her finger anymore.\nAt 1-year follow-up, the stump was not infected and was free of any tenderness or irritation. The child did not show any signs of ulnar nerve irritation. |
A 40-year-old nulliparous woman with no past medical history, other than endometriosis, presented to the emergency room with severe chest tightness of one day duration. She described chest tightness while exercising on a bicycle after 10 minutes. The next day she developed a severe hacking cough at work during a conference call. The cough was associated with disorientation and severe chest tightness. This prompted her to present to the emergency department in February 2018. The patient is a pharmacist, nonsmoker, and denies illicit drug use or recent travel. She had endometrial laser ablation with myomectomy in 2006. Hormonal contraceptives have been used since 2004 and were stopped three months before presentation in hopes of conception. Her last menstrual period was four days before the onset of symptoms. On physical examination, she had chest tightness localizing to the right side and decreased right sided breath sounds. All the routine laboratory work and vital signs were normal. The CXR showed a large right spontaneous pneumothorax with what approved to be a 5.6 cm pleural mass at the right lung base ().\nFollowing the pneumothorax diagnosis, the patient underwent emergent right thoracostomy with pigtail catheter placement. A repeat CXR revealed marked re-expansion of the lung but persistence of the right cardiophrenic opacity of unclear etiology. A follow-up CTof chest showed a 33 mm diaphragmatic defect with a 5.8 x 4.6 x 3.9 cm area of herniated liver corresponding to the presumed pleural mass ().\nFollowing complete thoracic imaging the patient underwent video-assisted thoracoscopic surgery (VATS), mechanical pleurodesis, and open repair of the right diaphragmatic defect by Dr. Emily Cassidy (Figures and ). Intraoperatively, the lungs appeared grossly normal. An obvious diaphragmatic defect was noted in the posteromedial portion of the central tendon of the diaphragm with a sizable protrusion of liver in to the chest cavity. There was an attempt to dissect liver adhesions from the diaphragm. Extensive liver adhesions forced conversion of the right VATS to a posterior lateral muscle sparing thoracotomy. Electrocautery and blunt dissection were used to separate the liver and diaphragm. Once the liver hernia was completely reduced, the diaphragm was repaired using multiple interrupted prolene pledgeted horizontal mattress sutures. Later, right parietal pleurectomy was performed by scoring the pleura posteriorly, anteriorly throughout the entire chest cavity. At the apex as well as the base, where pleurectomy was difficult, mechanical pleurodesis using a Bovie scratch pad was perfomred. A 24-french chest tube was placed at the apex of the chest cavity and 24-french blake was placed at the level of diaphragm.\nIntraoperatively, an endometrial implant (blue berry spot) was noted on the chest wall (), but the endometrial implant was not successfully biopsied for pathology evaluation due to the extent of liver adhesions and due to conversion of procedure from VATS to open thoracotomy.\nOn postoperative day three, the patient began her menstrual cycle. She was evaluated by a gynecologist consultant who recommended hormonal therapy, leuprolide a Gonadotropin releasing hormone (GnRH) analogue, to begin 2-3 weeks postoperatively for a period of 6-12 months for hormonal suppression to reduce the risk of recurrent pneumothorax. GnRH analogs are highly effective at suppressing ovarian hormone production and inhibition of growth of endometrial tissue. Due to a persistent air leak, the patient's chest tube was transitioned to a Heimlich valve to facilitate home discharge. We believed that the persistent air leak indicated there was some minor defect in the visceral pleura that was too small to identify intraoperatively. The patient was discharged on postoperative day eight and was seen as an outpatient by the cardiothoracic surgeon. At this time, there was resolution of air leak and removal of the chest tube. Patient was also seen after two months in a primary care clinic for follow-up visit with no issues. |
A 48-year-old lady was discovered to have multiple pulmonary nodules on chest x-ray (Fig. ) which was taken in view of desaturating levels of oxygen post-operatively. The patient had undergone elective left leg varicose vein surgery. An urgent CT scan was subsequently booked and showed innumerable soft tissue lesions throughout both lung fields including a sub-pleural individual lesion measuring 3.5 cm in the lingula (Fig. ). One lesion in the right upper lobe contained a small amount of air due to possible local erosion into an adjacent airway. Furthermore, a para-tracheal mass was seen on the right side measuring 5.6 cm with appearances suggestive of pleural metastases. Mediastinal nodes were reported as slightly prominent on the scan with nodes at the right hilum up to 12 mm in size.\nA CT guided fine needle aspirate (FNA) was carried out ~1 month after her initial surgery. Histopathology showed abnormal smooth muscle proliferation within lung parenchyma (Fig. ). The smooth muscle cells were positive for smooth muscle Actin, Vimentin and Desmin protein (Fig. ). This is suggestive of a smooth muscle tumour (which in the lung is most likely to be metastatic) or lymphangioleiomyomatosis. The immunohistochemistry favoured the diagnosis of a smooth muscle tumour.\nThe patient has a history of a total abdominal hysterectomy carried out at the age of 42 for polymenorrhagia and a large multiple fibroid uterus. Histopathology on this occasion confirmed multiple benign fibroids with no sinister features. This was in keeping with a diagnosis of BMLs of the lung.\nThe patient’s symptoms improved with the assistance of chest physiotherapy after the varicose vein stripping. This case is different in view of the large number of widespread metastases present in the lung making metastasectomy a non-viable option. On discussion with the patient a decision was made to adopt a conservative approach in her management and she is currently being followed up as an outpatient. |
A 68 year old female, known case of hypertension and osteoporosis presented to the emergency department with complaints of absolute constipation for five days, abdominal distension for three days and vomiting for one day. Abdominal distention was gradual in onset and generalized. Vomitus was feculent and large in quantity. Apart from an uneventful caesarian section 35 years prior, she had no other past surgical history. On examination, the abdomen was tense, tympanatic and mildly tender on the right side on deep palpation. DRE revealed collapsed ano-rectal canal with no fecal staining. Her Xray abdomen supine shown below () demonstrated dilated small bowel and cecal distension with fecal loading and no gas under the diaphragm was seen in the chest x-ray (). She had history for similar complaints one year back, for which she was managed conservatively. She was on medications for osteoporosis, and had family history of Chron’s disease.\nCT scan with IV contrast demonstrated marked diffuse dilatation of small bowel loop and the cecum with abrupt point of transition at the junction of cecum and the ascending colon. Mild ascites was seen in the peri-hepatic region, in the pericecal region and the pelvis (, ).\nBased on radiology, no proper cause of intestinal obstruction could be identified. The patient was offered colonoscopy before laparotomy but she chose to get operated. Surgery was performed by consultant surgeon and senior registrar. Patient was counselled for right hemicolectomy, diversion and stoma formation.\nOn laparotomy, around 200 ml reactionary fluid was aspirated. The cecum and small bowel were distended but healthy looking. Cecum was found rotated anteriorly upon itself (). Distal to the caecum, the large bowel was collapsed. There was no other obvious pathology observed. Liver and peritoneum were normal. Cecectomy was done with end to end ileo-colic anastomosis.\nPost operatively, the patient was kept in the general ward, and she developed ileus (). She was given chewing gum 2nd post op day, and allowed sips 3rd post op day. Her potassium was replaced, glycerin suppositories given. Her ileus resolved on 7th post op day and she was discharged the next day.\nPatient was called for follow-up after two weeks in which she reported uneventful post discharge recovery. |
The patient's mother was 36 years old, with a total of 5 pregnancies and 1 live birth. Eleven years ago, she gave birth to a healthy boy via cesarean section. She previously had 2 artificial abortions and 1 spontaneous abortion, with no family history of hereditary diseases. Ultrasound test result was normal at gestational week 6. At gestational week 12, nuchal translucency of 1.3 mm was observed, with no positive findings. Non-invasive DNA analysis showed low trisomy 21 risk, low trisomy 18 risk, and low trisomy 13 risk. At gestational week 24+5, ultrasound examination showed abnormal development of the long bones of the limbs (the length of the long bones was shorter than 1% controlled to the same gestational week of normal fetus), thick metaphysis in the right lower limb, irregular vertebral arrangement, and a narrow and small thorax (Fig. A–D). The patient's parents decided to terminate the pregnancy at 27 weeks of gestation considering this as a lethal skeletal dysplasia.\nAfter the termination of pregnancy at week 27, the gross examination of the fetus showed a flat face and nose bridge, short limbs, asymmetric short lower limbs, and bilateral clubfeet with bilateral ankle joint contracture (Fig. A, B). The autopsy report indicated that the left humerus bone was 2.5 cm long; the right humerus bone, 2.0 cm; the left femur, 3.0 cm; and the right femur, 2.2 cm; all were significantly shorter than 4SD measured at the same gestational age. In addition, the shapes of T3 to L3 were abnormal, the vertebral bodies appeared fused, the thorax was small with a maximum circumference of 16 cm, and the abdominal circumference was 26 cm. The autopsy also found subcutaneous edema in the head and face and thick metaphysis in the lower limbs. X-ray imaging showed short femur and humerus bones, a narrow and small thorax, thick metaphysis with a thick “splashed paint”’ pattern, and asymmetric short lower limbs (Fig. A–C).\nThe karyotype of the fetus was 46, XX. No unusual single-nucleotide polymorphisms was detected. Whole exome analysis showed that the fetus was heterozygous for the EBP mutation (NM_006579.2; C.440G>A p.Arg147His), which must have occurred de novo because the parents were non-carriers (Fig. ). Thus, CDPX2 was confirmed. |
A 19-year-old Chinese man involved in a motor vehicle accident was admitted to the emergency department presenting with a Glasgow Coma Scale (GCS) of 3 and right eye mydriasis. A computed tomography (CT) scan of his brain revealed right-side ASDH and a midline shift of 14.5 mm with severe brain swelling (Figs. and ). He was hemodynamically stable, with no clotting dysfunction according laboratory tests. He was subsequently transferred to the operating room and underwent a right decompressive craniectomy. The ASDH was caused by a ruptured lateral fissure vein. The brain exhibited slowly progressing swelling after the hematoma and inactivated brain tissue were evacuated. Consequently, immediate augmentation duraplasty using artificial dura mater was performed. On examination, the patient exhibited bilateral mydriasis. Given the slowly progressing brain swelling, this was possibly due to delayed contralateral ASDH or EDH. A CT scan of the brain was performed immediately thereafter, which revealed the emergence of contralateral DEH (Fig. ). Owing to a mass effect from the DEH and severe brain swelling, the patient was immediately taken to the operating room to undergo a left hematoma evacuation and decompressive craniectomy. During surgery, it was determined that the DEH was caused by the rupture of the middle meningeal artery branch, with temporal and occipital bone fractures (Fig. ). Following surgery, the patient was transferred to the neurological intensive care unit, where the left and right pupil sizes were measured to be 2.0 mm and 5.0 mm, respectively.\nOn postoperative day 1, approximately 5 h after the second surgery, the primary nurse found that the size of the patient’s left pupil gradually increased from 2.0 mm to 3.5 mm. An immediate brain CT scan revealed evidence of left-side DEH of the posterior cranial fossa (Fig. ). Owing to mass effect of the DEH, a third surgery was offered. He underwent left posterior cranial fossa hematoma evacuation and decompressive craniectomy. During the third surgery, it was determined that the DEH was caused by a ruptured transverse sinus. A brain CT following the third surgery was performed (Fig. ). The patient recovered to a GCS of 7 within 40 days after surgery, and was transferred to the rehabilitation hospital. He was ultimately discharged from the rehabilitation hospital with a Glasgow Outcome Score of 4. He underwent cranioplasty 1 year later and has since recovered well. |
A 65-year-old Chinese woman presented with a history of abdominal pain for 3 days. The patient developed paroxysmal abdominal pain in the past 3 days, accompanied by nausea, bloating and acid regurgitation. The pain was limited to the middle and upper abdomen, and anal exhaust and bowel movements were normal. The symptom did not become aggravated during the 3 days. She denied the symptoms of steatorrhea. The patient has had type 2 diabetes for more than 20 years and used a drug regimen to control the DM, but the patient complained that her fasting blood glucose was still poorly controlled. She had no other diseases. Her family medical history was unremarkable.\nNo positive signs were found in the physical examination. The results of the hematological examinations were normal. Only the blood sugar (11.7 mmol/L) and triglycerides (4.3 mmol/L) were abnormal in the blood biochemical examination, and no signs of infection were found. No abnormal results were observed from the electronic gastroscopy and electronic colonoscopy, which were performed 2 months ago. An enhanced abdominal CT scan revealed slightly increased gastrointestinal contents and no signs of acute abdominal (obstruction, perforation, etc.) or celiac vascular disease but found that the patient did not have the body and tail of the pancreas (Fig. a-b).\nSo the preoperative diagnosis is gastrointestinal dysfunction, agenesis of the dorsal pancreas, and pancreatic exocrine insufficiency were not excluded. Mosapride and trimebutine were used to improve gastrointestinal function, and 0.6 g pancreatin enteric-coated capsules were given to the patient before each meal continuously to supplement her pancreatic exocrine function. And Intermediate effect insulin were used to help control the fasting blood glucose.\nThe patient returned to the gastrointestinal surgery clinic 1 week later, complaining that although the symptoms were significantly relieved, but there was still mild postprandial bloating. Here fasting blood glucose fluctuates from approximately 5-8 mmol/L. The physician continued to give the patient the above medications. |
A 45-year-old female presented with a 1-month history of severe lower back pain and pain radiating to the left leg. No ‘café au lait’ spots or neurofibromas were present and the patient confirmed there was no family history of NF1. The neurological examination revealed a decreased sensation to a pin prick in the left L4 area. There was no obvious motor weakness of the leg, and bladder and bowel dysfunction was evident. Plain radiographs of the lumbar spine showed an osteolytic lesion at the L4 vertebral body. Computed tomography revealed compression of the spinal canal resulting from destruction of the posterior elements of L4 (). Magnetic resonance imaging (MRI) showed a destructive lesion and extradural tumor at the L4 level with dural compression. The lesion extending from the L4 vertebral body to bilateral pedicles showed intermediate signal intensity on T1-weighted images (), and it was heterogeneous with mixed high and intermediate signal intensities on T2-weighted images (). No other tumors were identified.\nAs the paralysis progressed rapidly, decompressive laminectomies and extradural tumor resection were performed. At the same time, posterior spinal fusion with instruments including a percutaneous pedicle screw system (Mantis, Stryker Japan Co., Tokyo, Japan) was performed for maintenance of spinal stability and prevention of tumor dissemination. The tumor appeared slightly adherent to the dura. The tumor mass was not obviously connected with the bilateral L4 spinal nerve roots or the dura. The boundary between the tumor and the L4 vertebral body was unclear. Histologically, the tumor consisted of coagulation necrosis and sheets of tumor cells with alternating areas of hyper- and hypocellularity. The majority of the tumor cells were atypical spindle cells. The spindle cells exhibited mitotic figures and pleomorphism. Immunohistochemically, the spindle cells were positive for vimentin and smooth muscle actin and negative for S-100, epithelial membrane antigen (EMA) and cluster of differentiation 34 (CD34). The histological diagnosis was undifferentiated pleomorphic sarcoma. Postoperatively, the symptoms of the patient were dramatically relieved. However, they recurred two weeks following the surgery, and the patient presented with progressive hypoesthesia and motor weakness in the legs. A total en bloc spondylectomy was performed according to a previous study () (). Briefly, all posterior elements of the spine (the spinous process, the superior and inferior articular processes, the transverse process and the pedicle) were removed in one section using a posterior approach. Subsequently, the anterior elements of the spine (the vertebral body and psoas muscle) were removed en bloc at the coincident level of the posterior halves using an anterior midline transperitoneal approach. To maintain stability of the tumor resection, posterior and anterior instrumented fixation was performed.\nAlthough multidrug adjuvant chemotherapy (30 mg/m2 doxorubicin, 1.5 g/m2 ifosfamide and 300 mg/m2 dacarbazine; 3 days] was administered following the second surgery (), the patient succumbed to intramedullary dissemination and carcinomatous meningitis 8 months following the initial consultation.\nThe second postoperative histopathological examination revealed a densely cellular area composed of spindle cells with a fascicular growth pattern and coagulation necrosis. Each of the spindle cells exhibited irregular contours with abundant gross cellular atypia, mitotic figures and pleomorphism (). On immunohistochemical staining, the tumor cells were positive for vimentin and bcl-2, focally positive for EMA, and negative for AE1/AE3, desmin, S-100, CD99 and CD34.\nFISH was performed on an unstained, paraffin-embedded tissue section using the Dual Color, Break Apart Rearrangement Probe (Kreatech Diagnostics, Amsterdam, Netherlands) according to the manufacturer’s instructions. Hybridization signals were assessed in 200 interphase nuclei with strong, well-delineated signals and distinct nuclear borders by two individuals, as previously described (). Dual color FISH analysis involving chromosome 17q showed that >10% of the cells from the tumor showed the deletion signal pattern of one red (NF1 region probe on 17q11) and two green [myeloperoxidase (MPO) gene region on 17q22 as the control probe), demonstrating a deletion of the NF1 gene (). These histopathological and cytomolecular findings confirmed the diagnosis of MPNST with focal epithelioid features. |
The patient is a female in her 40s who underwent a repeat renal transplantation that was complicated by acute tubular necrosis, delayed graft function, and Pseudomonas sepsis with a right upper lobe pneumonia (Figure ). The patient was admitted and treated with an extended course of piperacillin-tazobactam for the Pseudomonas infection. She continued to experience dyspnea following the completion of treatment and had a persistent right upper lobe opacity identified on chest X-ray (Figure ) and the subsequent computed tomography (CT) scan (Figures -). CT scans demonstrated a large opacity (10 cm x 4 cm) with adjacent areas of mixed ground glass opacity, airspace opacity, and thickening of the interlobular septa. The combination of the ground-glass attenuation with superimposed interlobular septal thickening and reticular thickening-specific opacifications was consistent with a “crazy paving” pattern. Given the history of immunosuppression, infectious, inflammatory, and neoplastic lesions were considered in the differential. A follow-up CT scan two months later demonstrated a persistent lesion with little change from the previous imaging. Given the failure of this lesion to resolve, the patient underwent a surgical lung biopsy to exclude an atypical infectious or neoplastic lesion.\nA video-assisted thoracoscopic surgery (VATS) was performed and an abnormal right upper lobe with a nodular and cheesy appearance was identified. A representative wedge biopsy was sent for intraoperative consultation. The cryosections showed interstitial pneumonitis with calcification and no evidence of malignancy. Given that this appeared to be a benign process, a lobectomy was not performed, but additional wedge biopsies were sent for permanent sections along with material for culture. As shown in Figures , , , , sections of the lung biopsy were composed of extensive multifocal calcifications, highlighted by a von Kossa stain (Figures , , , ) . The calcifications ranged in size from small stippled foci to larger irregular deposits with scattered foreign body giant cell reactions. The calcifications were localized within the alveolar septa and were associated with collagenous fibrosis and focal fibroblastic proliferation (Figures -). Many of the small blood vessels showed marked subendothelial myxoid intimal thickening with associated calcifications (Figures -). In contrast, the larger veins and arteries were relatively spared from calcification. The cultures were negative and Grocott's methenamine silver stain (GMS) and Ziehl-Neelsen (ZN) stains on tissue sections were negative for fungal organisms and acid-fast bacteria, respectively.\nFollowing the biopsy, the PTH was found to be mildly elevated at 8.1 pmol/L and associated with a slightly elevated calcium of up to 2.7 mmol/L. In a follow-up six months after the resection, the patient had normal pulmonary function testing, and the X-ray shown in Figure showed a significantly reduced opacity in the right upper lobe of the lung with no new consolidations or lesions. |
A 51-year-old Japanese woman was admitted for evaluation of proteinuria in 2011. She had a history of appendicitis at 12 years of age, cholelithiasis at 30 years of age, myoma at 46 years of age, and at least a 5-year history of dyslipidemia. Proteinuria (1+) had been first identified during a medical checkup 3 years prior and was ignored until increasing to 3+ in 2011. Her parents and brothers suffered from dyslipidemia but had no history of renal disease.\nOn admission, her height and weight were 161 cm and 49 kg, respectively, and her blood pressure was 137/94 mm Hg. On physical examination, slight pitting edema was observed; however, there were no xanthomas. Urinary protein excretion was 4.12 g/g Cre, and the serum triglyceride level was high. She did not exhibit nephrotic syndrome. The other laboratory findings are shown in Table . A computed tomography scan showed neither atrophy nor enlargement of the kidneys.\nA renal biopsy specimen was processed in August 2011 using standard methods, and in the sections for light microscopy, the renal cortex was found to contain 11 glomeruli, 2 of which showed sclerosis. The remaining glomeruli were enlarged by distinct dilatation of the capillary lumina, which were filled with thrombus-like substances that stained slightly with periodic acid-Schiff. Some glomeruli exhibited moderate mesangial cell proliferation and increased mesangial matrix, and double contours of the capillary wall were partially observed. No crescents or adhesion in glomeruli were found (Fig. –c). A direct immunofluorescence showed no deposition of IgG, IgA, IgM, C1q, C3, or fibrinogen in glomeruli. Staining with Oil red O showed lipid droplets in the capillary lumina, which were stained positive for apoE (Fig. , e). Electron microscopic findings showed capillary dilation and occlusion with numerous lamellated lipid granules. Thin basement membranes with swollen endothelial cells were also observed. The histological findings and the results of apoE analysis (described below) satisfied the diagnostic criteria for LPG [].\nTreatment with fenofibrate and irbesartan was initiated. Two weeks later, serum triglycerides and urinary protein excretion were reduced to 200 mg/dL and 1 g/g Cre, respectively. Two months later, the former was within the normal range, and the latter was reduced to less than 0.5 g/g Cre. Eight months later, urinary protein excretion ceased and did not recur thereafter (Fig. ).\nIn a second biopsy in August 2013, the specimen contained 22 glomeruli, 4 of which were obsolescent. Two of the glomeruli were filled with thrombi, but thrombi were absent in the other glomeruli (Fig. ). |
A previously healthy 12-year-old girl was referred to our hospital with a right common femoral vein deep venous thrombosis (DVT) complicated with pulmonary embolism. Three days after a mild blunt trauma to the right thigh, she presented to the referring hospital with fever, right thigh pain, difficulty breathing, and chest pain. Doppler ultrasound of the right thigh showed DVT in the right common femoral vein. Magnetic resonance imaging of the right thigh and hip showed pyomyositis of the anterolateral muscle compartment without signs of hip joint septic arthritis or femur osteomyelitis (Figure ). A spiral chest computed tomography scan with angiography demonstrated multiple intramural filling defects in the left lower lobe segmental branch of the pulmonary artery with multiple emboli in both lungs and a mass in the right atrium (a suspected thrombus). Therefore, the patient was administered low-molecular-weight heparin (LMWH) and transferred to our institution.\nThe medical histories of the patient and her family were unremarkable. The patient was fully immunized, and her developmental and growth histories were likewise unremarkable.\nA physical examination in the pediatric intensive care unit indicated that the patient was overweight (weight: 53 kg, height: 139 cm, body mass index: 27.4). The patient was conscious and alert, with a blood pressure of 122/82 mmHg, mean arterial pressure of 91 mmHg, pulse of 137 beats/min, respiratory rate of 25 breaths/min, and oxygen saturation of 93% on two-liter oxygen via a nasal cannula. Chest examination revealed decreased air entry on the right side with bilateral crepitation. Cardiovascular and abdominal assessments revealed no abnormalities. Lower limb evaluation showed diffuse right thigh swelling associated with mild tenderness and a decreased range of motion at the hip joint with good perfusion. Examination of the left leg revealed normal results.\nAn initial laboratory work up showed a white blood cell (WBC) count of 24.6 × 109/L (normal range: 4-11 × 109/L) with 90% neutrophils, hemoglobin level of 8.4 g/dL (normal range: 11-15 g/dL), mean corpuscular volume of 78 fL (normal range: 80-100 fL), C-reactive protein level of 207 mg/L (normal range: 0-10 mg/L), erythrocyte sedimentation rate of 150 mm/h (normal range: 3-13 mm/h), and D-dimer concentration of 1.47 μg/mL (normal value: <0.4 μg/mL). Chest radiography showed bilateral pleural effusion, which was more on the right side. A transthoracic echocardiogram demonstrated an echogenic, non-obstructive mass in the right atrium at the inferior vena cava and the right atrium junction, suggesting an intracardiac thrombus.\nAfter 12 hours of incubation, the blood culture grew MRSA that was susceptible to vancomycin with a minimal inhibitory concentration (MIC) of 1 μg/mL (automated VITEK®2; BioMerieux, Marcy l’Etoile, France) and 1.5 μg/mL by E-Test (BioMerieux). However, the isolate was resistant to other anti-MRSA agents (i.e., clindamycin with an MIC of >4 μg/mL, trimethoprim-sulfamethoxazole with an MIC of >4/76 μg/mL, and erythromycin with an MIC of >8 μg/mL). The antimicrobials were adjusted according to the blood culture results. The vancomycin dose was increased to 15 mg/kg/dose every six hours with a targeted trough therapeutic level of 15-20 mg/L.\nUltrasound-guided aspiration of intramuscular fluid and a muscle biopsy revealed 60 mL of turbid hemorrhagic fluid. Pathological examination showed necrotic muscle tissue with heavy infiltration of neutrophils, consistent with septic myositis. All cultures from the aspirate were negative.\nThe maximum vancomycin trough level reached in this patient was 11 mg/L after changing the vancomycin dosing regimen to 30 mg/kg as a loading dose followed by 20 mg/kg every eight hours on day four of treatment.\nDespite a negative blood culture after 48 hours, the patient continued to be highly febrile (39-40°C) and her respiratory condition worsened as the respiratory support increased to non-invasive positive pressure ventilation. The C-reactive protein level increased to 348 mg/L. Pleural effusion on the right side increased and required chest tube insertion. Pleural fluid analysis showed pleocytosis (WBC 43/μL, 90% neutrophils) with low glucose and high protein levels. However, the pleural fluid culture results were negative. Chest computed tomography showed progression to necrotizing pneumonia in the right middle and lower lobes with empyema and new septic emboli in both lungs (Figure ). Therefore, vancomycin was replaced with intravenous linezolid (600 mg every 12 hours) after five days of vancomycin therapy.\nApproximately 48 hours after starting linezolid, the fever subsided. The patient’s respiratory condition gradually improved over the next seven days. The patient was transferred to the pediatric floor with two-liter oxygen via a nasal cannula while maintaining the LMWH and linezolid.\nThe patient’s respiratory condition continued to improve. She completed four weeks of linezolid treatment. Blood cell counts, including the WBC count, normalized and remained stable throughout the course. A follow-up chest X-ray performed one week after linezolid discontinuation showed markedly improved lung parenchyma with remaining large pneumatoceles (Figure ). Repeated thigh magnetic resonance imaging conducted after six weeks showed resolution of the DVT and considerable pyomyositis improvement. Additionally, a repeated transesophageal echocardiogram showed resolution of the intracardiac thrombus. The patient was discharged in good condition after six weeks of hospital stay. The pneumatoceles resolved spontaneously after three months on the follow-up chest radiograph. |
A 37-year-old male patient reported to the Department of Periodontology Government Dental College, Calicut with a chief complaint of bleeding from gums and bad breath of 3 months duration. On clinical examination, generalized moderate to deep periodontal pockets and bleeding on probing was noticed. Radiographic examination revealed generalized bone loss. Based on the history, clinical and radiographic findings, the patient was diagnosed with chronic generalized periodontitis. Six weeks after performing thorough scaling and root planning and following a strict plaque control regime, the case was reevaluated. Since the pocket depth was 7 mm distal to 36, it was decided to proceed with flap surgery [].\nAfter giving block anesthesia in the left mandibular posterior area, internal bevel incision was given, and a full thickness flap was raised. Meticulous defect debridement and root planing was done to remove subgingival plaque, calculus, and inflammatory granulation tissue. A deep two wall defect was observed distal to first molar, heavy ledges were noticed extending bucally from second premolar to the second molar area []. First it was decided to remove the ledges as a part of osteoplasty procedure and then utilize the removed bone to fill the two wall defect. For this purpose, a sharp sickle scaler (PDT Cruise Scaler U15-33 R113, PDT Inc., US) [] was used in a scrapping motion with sufficient force such that the bone was essentially shaved off []. The harvested bone was carefully collected in a dappen dish and then transferred to the defect with a periosteal elevator [Figures and ]. The flap was repositioned, single interrupted sutures were placed and periodontal dressing was given [Figures and ]. The patient was reviewed after 1-week, the healing was found to be uneventful. The sutures were removed, and the area was irrigated with saline. The patient was put on the maintenance phase with oral prophylaxis performed every 3 months. Radiographs were taken after 1-year. Clinically, there was a reduction in probing pocket depth from 7 mm to 3 mm []. Radiographs revealed radio opacity in the site distal to first molar, suggestive of bone fill in the defect []. |
A 67-year-old female patient reported to the Department of Periodontology and Oral Implantology with the chief complaint of having missing teeth in right and left side of the posterior maxilla. There was no significant medical history. On enquiring about previous dental treatment, it was found out that the tooth was lost because of caries and periodontal disease. Preoperative computerized tomography (CT) scans were performed to obtain an accurate measurement of the bone before surgery. Immediate and 8 months postoperative CT scans were performed to check the proper placement and success rate of the implants, bone formation, and sinus membrane position. Patient had maxillary posterior bone - 1.49 mm on the right side and 1.47 mm on the left side between the alveolar crest and maxillary sinus as seen in .\nPresurgical preparation included medical, dental, and computerized axial tomography (CAT) scan radiographic evaluations and basic dental therapy to alleviate preexisting medical-dental problems. Prior to implant surgery, informed consent for bone graft and sinus lifting of the implant, CAT scan consent was obtained from the patient. Patient received 625 mg augmentin (amoxicillin and clavulanate potassium) twice in a day before surgery. The patient was treated under oral sedation or intravenous sedations. Patient was treated with 5 mg of triazolam orally and then draped with sterile surgical barrier. Before a surgical procedure start, a full mouth prophylaxis was done on surgery patient. The posterior quadrant of the maxilla was anesthetized via local anesthesia injection 2% lidocaine HCL and epinephrine 1:100,000 in a 30-gauge needle. A direct, full thickness midcrestal incision with a #15 blade was made through the mucoperiosteum to the crest of the ridge. Full thickness reflection of buccal and palatal tissues exposed the alveolar ridge. To reflect the flap, molt elevator was used. The implant position was marked on the alveolar crest with a small trephine drill (Ø 2.0 mm). After locating the implant position, the preparation was widened with two sizes internally irrigated trephine (Ø 3.5 mm and Ø 4.25 mm) drill. Minimal pilot drilling (Ø 2.0 mm) was performed to a depth approximately 1 mm away from the sinus floor boundary. The osteotomy site was gently tapped with mallet and osteotome number three or four. Intra oral radiograph of the osteotomy site was taken to determine the position of the sinus membrane as seen in .\nAt the same time, a 4-5 vials of blood was collected from the patient's vein, and blood is spun by a special machine called a centrifuge and spun for approximately 12 min. The PRF obtained is mixed with bone graft material. Pieces of PRF are made with scissor. The drops of clindamycin and cefazolin are added into the pieces of the PRF clot. These small pieces of the membrane are placed inside the osteotomy socket as a cushion during sinus lifting. A mixture of the MFDBA were taken in the separate container saturated it with saline. After 10 min, the excess fluid was drained, and clindamycin and cefazolin powder added into it. The bone particles and pieces of membrane were placed inside the osteotomy site. The osteotomy site with membrane and bone was gently tapped with osteotomes and mallet. Autogenous bone grafts from maxillary tuberosity area or bone removed during the site preparation from trephine were used to fill out the osteotomy site. The membrane and bone particles protect sinus membrane from perforation. Osteotome site was packed with bone and membrane after gradually adding bone particles and tapping it with osteotome and mallet. Bleeding from the osteotome site provides sign regarding the perforation of the sinus membrane. If bleeding does not occur from the site, it shows that perforation happened in the sinus membrane. Trial implant is placed inside the osteotome site to check adequate width of osteotome site. After checking with trial implant, actual size implant was placed in the osteotomy site. All the implants achieved primary stability. Ten to twelve small holes were made with surgical quarter round bur on the buccal surface of the posterior maxilla to initiate fast healing at the implant site. Bone from the maxillary tuberosity is taken and crushed with a bone crusher forceps. Crushed bone pieces and the rest of MFDBA particles are placed around the implant mainly on the buccal surface. Implant is covered with healing collar or cover screw. Placement of healing collar or cover screw is dependent upon occlusal clearance. Occlusal clearance between maxillary ridge bone and mandibular teeth is <5 mm than cover screws are placed on the implant. PRF is placed in around healing collar for excellent healing of soft tissues. After repositioning the soft tissues, primary closure was attained using 4-0 chromic gut suture. The site was allowed to heal for 3 months. After 4 months, abutments were placed on the implants and restorative procedure was initiated. |
The present case report is about a 21-year-old female patient who had a scald burn injury at 1.5 years old was admitted to our department for her breast deformation due to burn contracture at the inferior pole of the right breast [Figure and ]. According to the patient's history, burn wounds were treated with conventional burn dressing. On physical examination, we defined that development of the volume of the right breast was equal to the left, the inferior pole of the right breast was flattened due to contracture, and the nipple was projected to the inferior pole. We found that the inframammary sulcus of the right breast was 2 cm lower than that of the left; the distance of the nipple to inframammary sulcus was 4.7 cm, whereas the areola to inframammary sulcus was 2 cm [Figure and ]. In addition, there was minimal wideness at the base of the right breast due to contracture. The size of the left breast was nearly ideal: The sternal notch-nipple distance was measured at 18 cm and the nipple-inframammary sulcus distance was 7 cm. In addition, the base of the right breast was minimally expanded due to contractures of the lower pole of the breast. In the measurement of the left breast, the sternal notch-nipple distance was 18 cm and the nipple-inframammary sulcus distance was 7 cm — the accepted ideal breast size measurements.\nWe had three aims when we were planning the surgery:\nMoving the nipple-areola complex to the same level as the other breast, Moving the inframammary sulcus to 2 cm above, and Releasing the lower pole contracture of the breast.\nWe could achieve the first two of these aims with a superior pedicle technique of reduction mammoplasty with minimal tissue excision. In addition, Z-plasty was planned to release the contracture. A new nipple-areola complex level was identified according to the left breast's level. Medial and lateral lines were planned to merge inferiorly at 2 cm above the inframammary sulcus [Figure and ]. According to this technique, the diameter of the medial and lateral vertical will determine the length of the nipple to inframammary sulcus [].\nThis technique is also a bit narrow in the breast base. Z-plasty was planned to relieve the contracture of the lower pole as the third objective.\nA superior pedicle carrying the nipple-areola was de-epithelialised. A lower parenchymal V flap was transposed superiorly and attached to the pectoral muscle. Inferior parts of the lateral and medial glandular flaps were minimally excised to form a new inframammary sulcus. The desired laxity of skin at the lower pole was obtained by performing a new Z-plasty between the lateral and medial flaps. |
The patient is a 34 year old healthy primigravida with spontaneous dichorionic diamniotic twins and likely bicornuate uterus, with one fetus in each horn, initially diagnosed at 12 weeks gestation by two-dimensional ultrasound. Magnetic resonance imaging at that time reported bicornuate unicollis uterine anatomy, with symmetrical myometrial thickness in both horns. The left cervical canal was noted to communicate with the right lower uterine segment and not directly with the vagina. A thin amniotic band was noted in the superior left uterine horn. Bilateral normal maternal kidneys were noted.\nThe patient subsequently presented at 17 weeks gestation with new onset significant pelvic pain. An MRI at an outside hospital demonstrated a thin myometrial wall (thickness not reported) in the left uterine horn without myometrial disruption, but with moderate pelvic free fluid (Fig. ). The patient’s hematocrit was noted to decline from 32 to 26%, and she was transferred to our tertiary care center for further management. Three-dimensional ultrasound at our institution revealed two separate uterine cavities, each with a live appropriately-grown fetus with normal amniotic fluid. The anatomy of the cervices was difficult to delineate, but a vascular connection was noted between the medial surfaces of each horn. The myometrium over the left horn was noted to be “markedly thinned” superiorly (Fig. ). Moderate hemoperitoneum was documented, with a 3.6 cm clot adherent to the thinnest portion of the myometrium of the left horn. Concern was raised for rupture of a rudimentary uterine horn. A severe cardiac anomaly was incidentally diagnosed in the fetus in the right unicornuate uterine horn.\nGiven these imaging findings and the patient’s ongoing pain, the decision was made to proceed with diagnostic laparoscopy and left hemi-hysterectomy with fetus in situ. The patient underwent ultrasound-guided selective reduction of the fetus in the left uterine horn using intracardiac potassium chloride. Immediately after this procedure, the patient underwent laparoscopy, at which point she was noted to have a right unicornuate uterus with a rudimentary left uterine horn, with 2 cm rupture on the superior surface with amnion protruding (Fig. ), and moderate hemoperitoneum. Due to intraoperative bleeding and suspected amniotic rupture during examination of the left horn, the procedure was converted to an abdominal hemi-hysterectomy. During this procedure, a fibrous but narrow band was noted between the uterine horns, which was ligated and transected using bipolar energy. The procedure and the patient’s recovery were uncomplicated.\nThe pregnancy in the right uterine horn continued postoperatively. At 19 weeks of gestational age, in the setting of a severe fetal cardiac anomaly and high likelihood of preterm delivery complicating surgical correction, the patient underwent laminaria placement and uncomplicated ultrasound-guided dilation and curettage of the right uterine horn.\nThe patient subsequently spontaneously conceived two singleton pregnancies. Normal anatomical surveys and fetal echocardiograms were noted in each. Both pregnancies were complicated by preterm contractions without cervical change. Eighteen months after her surgery, the patient delivered a healthy, small for gestational age (2,580 g) female infant, followed by a healthy 2,722 g male the following year. |
A 27-year-old nullipara presented to her local hospital at 33 weeks' gestation with decreased fetal movement, uterine contractions, and possible leakage of fluid from the vagina. Testing confirmed rupture of membranes; therefore, the patient was started on antibiotics to increase latency and was given betamethasone to hasten fetal lung maturity. An ultrasound at the community hospital showed polyhydramnios and a fetal double bubble sign consistent with duodenal atresia. The mother was transferred to Penn State Milton S Hershey Medical Center for anticipation of preterm delivery in a fetus that would require postnatal surgery.\nAfter transfer, fetal monitoring showed normal fetal heart rate variability with accelerations and occasional decelerations related to contractions. An ultrasound confirmed the double bubble sign and polyhydramnios. Blood was observed in real-time swirling into the amniotic fluid from the umbilical cord (). The patient was taken to the operating room and an emergent cesarean section was performed. The amniotic fluid was grossly bloody. A viable male infant weighing 2295 grams was delivered with Apgar scores of 7 and 8 at one and five minutes, respectively.\nThe umbilical cord overall length was 29.5 cm and there were 13, 0.5- 1 cm exposed segments of the umbilical arteries spiraling along the length of the cord (). A discrete area from which the hemorrhage emanated was not identified. The placental disk was of normal weight and appeared grossly normal. Histopathologic examination of the cord showed absence of Wharton's jelly covering the umbilical artery, extreme attenuation of the media in the portion of the vessel exposed to the amniotic fluid, and degeneration of the overlying amnion (Figures and ).\nThe infant had no stigmata of Down syndrome. He appeared pale at birth and an initial hematocrit was 29.4% that was treated by a blood transfusion upon admission to the neonatal intensive care unit. Postnatally the abdominal X-ray revealed air in the stomach and first portion of the duodenum with the remainder of the abdomen appearing gasless, consistent with duodenal atresia. A nasogastric tube was placed and 37 ml of bloody secretions were suctioned. The infant underwent laparotomy on the second day of life where an atresia was noted in the third portion of the duodenum and duodenojejunostomy was performed. He did well postoperatively and was discharged on day 30 of life. |
An 18-year-old Xhosa-speaking South African male was referred to the Livingstone Hospital renal unit for evaluation. He first presented at the age of 17 years to a nearby hospital emergency unit with a headache. He was not on any chronic or over-thecounter medications, did not consume liquorice, ethanol or traditional medications and was a non-smoker. He was noted to be hypertensive, with a blood pressure of 216/114 mmHg and hypokalaemic, with a serum potassium level of 2.9 mmol/l (see ), but he left without treatment.\nEight months later he was seen at the same unit with a similar presentation and was admitted for further investigations and treatment. Despite multiple antihypertensive interventions, his blood pressure remained uncontrolled. At discharge he was commenced on the following medications: enalapril 10 mg 12 hourly, amlodipine 10 mg daily, furosemide 40 mg twice daily, atenolol 25 mg daily, hydrallazine 50 mg twice daily and hydrochlorothiazide 25 mg daily.\nHe was referred to our unit, and one month later his blood pressure was 179/118 mmHg in the left arm and 182/113 mmHg in the right arm, despite adherence to the treatment regime. He looked well, had a regular pulse rate of 59 beats/min and weighed 68 kg. The patient gave a history of experiencing frequent headaches associated with muscle fatigue, but no myalgias. The muscle fatigue was worse at times when the headache was present. He also described exertional dyspnoea but no spells suggestive of phaeochromocytoma. Both his parents were hypertensive and his father had died of an uncertain cause before the age of 50 years.\nOn cardiovascular examination, all pulses were present. There Division of Nephrology and Hypertension, Livingstone was no radiofemoral delay or any bruits. The cardiac apex beat was forceful and laterally displaced with an associated fourth heart sound. He had normal secondary sexual characteristics, and retinal examination revealed markedly reduced arteriolar diameter and arteriovenous nicking. The urine dipstick was normal as was an ultrasound of the kidneys, ureters and bladder.\nChest radiography showed a bulky heart shadow and electrocardiogram confirmed left ventricular hypertrophy with a Sokolow–Lyon score of 88 mV. Laboratory investigationsconfirmed the hypokalaemia and his serum potassium level wasnow 2.7 mmol/l, associated with a supressed renin level of 6.0 mIU/l and aldosterone of 48.6 pmol/l (). Unfortunately, arterial blood gas analysis was not performed prior to treatment.\nBased on this presentation, genetic testing for the locally prevalent c.1815G>A (p.R563Q) Liddle’s syndrome-associated genetic variant in the SCNN1B gene was requested, and his treatment was intensified to include doxazosin as no amiloridecontaining medications were available at the time. His blood pressure remained uncontrolled and the c.1815G>A (p.R563Q) variant screen was negative.\nDue to his persistent hypokalaemia, hypertension and supressed renin and aldosterone levels, sequencing of exon 13 of the beta-chain of the epithelial sodium channel was pursued. A novel heterozygous 11bp deletion in the SCNN1B gene was detected (, case and , control). The mutation causes a frame shift in exon 13 of the gene, resulting in a premature stop codon and truncated protein product.\nHis family was called in for screening. We have been unable to see his mother who lives in a rural area distant from our clinic. He has two brothers and one sister who are from the same parents. His younger brother (11 years) and his older sister (21 years) were both well and had normal blood pressures. His older brother (25 years) was found to have a raised blood pressure of 141/77 mmHg on ambulatory monitoring but was overweight with signs of insulin resistance and he had a body mass index of 35.1 kg/m2. All three siblings tested negative for the mutation seen in this patient and had potassium, renin and aldosterone levels in the normal range.\nAs amiloride is not registered in South Africa and is only available in combination with hydrochlorothiazide (5 mg amiloride; 50 mg hydrochlorothiazide) (Amiloretic, Aspen Pharmacare, South Africa), he was started on the latter. Over the next few months his blood pressure improved significantly, with office readings of 162/91, 139/84, 190/113 and 142/100 mmHg. He still requires concomitant treatment with enalapril 10 mg daily, amlodipine 10 mg daily, atenolol 50 mg daily, doxazosin XL 8 mg daily and potassium replacement. On one occasion, after running out of tablets for three days, his blood pressure was 221/157 mmHg.\nHis creatinine level has remained elevated but he has had no further headaches. However, potassium levels remain low and we suspect that higher doses of amiloride are needed. This will be introduced, pending local regulatory approval. |
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