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A 59-year-old man, without any particular medical history, presented at our department of neurology at the military hospital with a progressive clinical picture, 10 days following a flu, made of initial ascending paresthesia attending rapidly the upper limbs in <24 hours followed after 2 days by weakness in the lower then the upper limbs, and a discrete dysphagia with frequent choking to liquids. He was admitted to our hospital 8 days after the beginning of the symptoms on 11 April 2016. During hospitalization, he developed 3 days after his admission, respiratory weakness with worsening of the dysphagia, and facial diplegia on the 14th day of evolution. No autonomic dysfunction was noted. Physical examination showed flaccid tetraplegia graded 3 in upper limbs and 2 in lower limbs according to MRC scale, with absence of all reflexes, hypoesthesia in glove and stocking distribution, a positive Romberg sign, and facial diplegia. The EMG (Figures , and ) showed sensory and motor demyelinating polyradiculoneuropathy with decreased motor conduction velocities, prolonged distal latencies of ulnaris, medianus, tibilais, and peroneus, prolonged F-wave latencies in the four limbs, with decreased sensory conduction velocities of the right musculocutaneous and left ulnaris. CSF analysis highlighted albumino-cytological dissociation with 0 white cell and CSF protein level at 0.7 mg/dL. The patient was initially diagnosed as GBS and received an immunoglobulin therapy. His symptoms improved then progressively, and he was discharged on 25 April 2016, after the total regression of the respiratory signs and the improvement of the dysphagia, facial diplegia, and muscle weakness which was graded 4+ at his discharge. Two weeks later, he presented with worsening of the dysphagia and a sensory ataxia. He was readmitted to our department on 10 May 2016. Physical examination showed sensory ataxia, positive Romberg sign, loss of deep sensation at right, absence of all reflexes, with the same muscle strength grade 4 at his discharge, a weak nausea reflex, and a discrete facial diplegia. This was considered as a treatment-related fluctuations (TRF), and the patient received a second immunoglobulin therapy with a complete regression of the dysphagia and the sensory ataxia. After his discharge, he continued to improve with a clear regression of symptoms and recovery of all reflexes, only a discrete proximal muscle weakness persisted with few paresthesia of extremities which responded well to pregabalin. One year later, on April 2017, the patient manifested recurrent symptoms with flaccid tetraplegia graded 4, absence of all reflexes, and a positive Romberg sign noted at the physical examination. He was then differentially diagnosed with A-CIDP according to Ruts et al criteria. He started taking methylprednisolone daily with a total recovery. The evolution was marked by steroid dependence, leading to the association of an immunosuppressive agent (azathioprine) with stability of the disease. He only kept a discrete proximal muscle weakness in lower limbs. |
A 15-year-old previously healthy female was admitted to our tertiary care pediatric intensive care unit (PICU) from a community emergency room with unresponsiveness. She was found by her parents in an unresponsive and tremulous state on the morning of admission. Emergency rescue was called and upon their arrival, the patient was intubated at the scene for significant respiratory depression. The patient was then transported to a local emergency department, and en route she was given one dose 1 mg lorazepam (0.02 mg/kg) for tremulousness, suspected to be seizure-related, with no response. Upon arrival to the outside emergency room, she was unresponsive, normothermic, tachycardic with a heart rate of 150 bpm and with mild hypertension and the blood pressure of 130/84 mmHg. She had symmetric and reactive pupils on examination. She received an additional 1 mg of lorazepam for ongoing tremulous movements with no response. She had an unremarkable brain computerized tomography scan and cerebrospinal fluid studies. She had normal electrolytes and liver function test. In addition, urine toxicology screen, blood alcohol, acetaminophen, and aspirin level were negative. She received a normal saline bolus, and a dose of ceftriaxone was then transferred to our PICU with a diagnosis of status epilepticus and possible meningitis. Just prior to transport, she was started on propofol infusion at 20 mcg/kg/min. At admission to the PICU, her physical examination was notable for normothermia, normotension, and tremulous motor activity. She had no response to painful stimuli but had a normal pupillary examination. Her propofol infusion was discontinued immediately and on follow-up assessment, she was able to localize to pain. Her repeat electrolytes, blood gas, chest X-ray, and electroencephalogram were normal, and electrocardiogram revealed sinus tachycardia with normal PR or QTc intervals. Over the next 10 hours, her neurological status remained unchanged. She was continued on invasive mechanical ventilation and intravenous fluids. Despite her negative toxicology screen, and her family’s denial of any history drug use in the past or significant potential sources of mood-altering drugs in the house, we considered a trial of ILE therapy secondary to the possibility of a drug overdose. She was started on intravenous 20% lipid emulsion at a dose of 1 mL/kg over one hour. By the end of lipid infusion, the patient had such a dramatic improvement in her sensorium, she was extubated, and became quite verbal admitting that she ingested about forty 350 mg of each tablet of carisoprodol (14 gm), which was used by her father for chronic lower back pain. |
Our patient is a 76-year-old lady with chronic obstructive pulmonary disease, peripheral vascular disease, and smoking as past medical history, presenting with 2 months of a nonproductive cough. The patient was found to have a left upper lobe nodule on a plain chest radiograph and further evaluation with a computed tomography (CT) of the chest delineating a 1.2 cm spiculated nodule (). Following a PET-CT that showed pathologic FDG uptake of the lesion, the patient underwent elective left thoracotomy with upper lobe lobectomy and mediastinal lymph node resection. Pathology showed a 1.5 × 0.6 cm papillary predominant adenocarcinoma with no lymph node metastases.\nThe immediate postoperative course was uncomplicated and the patient was transferred to the cardiothoracic unit for observation. Follow-up chest radiography on postoperative day-1 showed a small left lower lobe opacity with the patient complaining of mild pain at the surgical site. On postoperative day-2 the patient developed atrial fibrillation with rapid ventricular rate associated with hemoptysis and worsening left sided pleuritic chest pain. Plain chest radiography showed worsening left lung opacity with bronchial cutoff (). A CT of the chest revealed diffuse ground glass attenuation of the left lower lobe with proximal bronchial obliteration (). The patient underwent elective intubation to undergo flexible bronchoscopy that revealed a tight and obstructed orifice of the left lower lobe (). She was brought to the operating room emergently for high suspicion of lobar torsion, and a left thoracotomy showed a dark purple appearing lung (). Following detorsion the lung did not appear viable; however considering the patient's baseline marginal FEV1, 24 hours was given for the lung to recover. The patient showed no clinical improvement and underwent completion of left lung pneumonectomy the next day. Postoperative recovery was uneventful and patient was discharged after 7 days. The patient was seen in the outpatient office 4 months following discharge reporting no complications. |
An 11-year-old male Rwandan, a known patient of multiple hereditary exostoses (MHE), presented to our hospital with a 6-month history of unrelenting bone pain despite treatment with NSAIDs; he later developed (two weeks prior to admission) a dull persistent aching pain and swelling of his left lower thigh that was worse at night and unresponsive to morphine. His parents noticed that he had also lost weight and appetite. There was no history of trauma or a fall. He was diagnosed with MHE at age 3 and had been living a relatively normal life and attending school. None of his siblings or any other member of his family had MHE. His past surgical history was unremarkable. On general examination, he was well nourished, had mild pallor of the mucus membranes, no jaundice, lymphadenopathy, or skin rash. All the other parameters were normal. Examination of the musculoskeletal system revealed normal stature except for the curving deformity of the left leg. There were multiple palpable bony swellings bilaterally on the upper humeri and lower femurs. The lesion on the left distal femur was markedly enlarged and tender, with induration, reddening, and limited range of motion of his knee joint (). Repeat X-rays confirmed the presence of bony outgrowths (exostoses) on the medial and lateral aspects of the distal femurs bilaterally and left metaphyseal widening common in this condition as had been previously identified when he was diagnosed at age 3, and further investigations of the left distal femur swelling () with magnetic resonance imaging (MRI) revealed a distinct enhancing lesion in the distal aspect of the left thigh at the site of intense swelling and pain. MRI showed a lesion with hypointense signals on T1WI sequences and has heterogeneous signal intensities with moderate and heterogeneous enhancement on T1WI postcontrast study and on T2WI sequences; the lesion had heterogeneous signal intensities (Figures –). At surgery, there was a necrotic lesion, and excision biopsy at the site of the left distal femur exostosis was taken (Figures and ). Hematological evaluation was normal except for a mild leukocytosis. The results of the serum biochemical tests were also normal. We did not do genotyping for EXT-1 and EXT-2 due to lack of facilities.\nHistopathological examination revealed a characteristic fibrous cartilaginous cap with a broad base (1.293 mm thick), covering a layer of normal appearing marrow and bone below which was a tumor-forming osteoid, an osteoblastic tumor as evidenced by presence of numerous bone spicules of varying maturity. There was also marked cellular atypia, grossly pleomorphic osteoblasts in the marrow with frequent mitoses. These features were those of a high-grade osteoblastic osteosarcoma. In conclusion, histopathological revealed an osteochondroma with an underlying high-grade osteoblastic osteosarcoma involving the marrow cavity (Figures –).\nFor staging purposes, CT scans of the chest, abdomen, and pelvis were done to investigate any presence of metastatic lesions (Figures and ). There were no metastatic deposits in the lungs, abdomen, and pelvis, and this was confirmed with PET scan in India where the patient was referred for specialized bone tumor treatment including limb salvage therapy. He was reevaluated, and the diagnosis of MHE and osteoblastic osteosarcoma confirmed. Whole body PET scan showed metabolically active disease in the distal left femur 8.6 × 8.1 × 16 cms in dimension with features consistent with osteosarcoma, multiple hereditary exostoses with evidence of skip lesions, loco-regional lymph node involvement, and no distant metastases. Histopathological review confirmed the earlier diagnosis of osteochondroma with osteoblastic osteosarcoma. The conclusion was that the patient had clinically localized disease. The following treatment plan was proposed and instituted: initiation with neoadjuvant chemotherapy followed by limp salvaging surgery and finally adjuvant chemotherapy. He received doxorubicin 35.5 mgs/m2 per day (day 1 and day 2), cisplatin 60 mgs/m2 for 7 days (days 1 and 2), methotrexate 12 gms/m2 per day, etoposide 100 mgs/m2/day for 5 days, and ifosfamide 2.8 gms/m2/day for 5 days followed by Ifosfamide 3 gms/m2/day for 3 days and subsequently pegylated Interferon-α2b 0.5 mcg/kg - 1mcg/kg. He completed a total of six cycles of chemoimmunotherapy.\nOur patient is still alive and back at school with no evidence of disease after 11 months of treatment and follow-up and continues to be followed up by the oncologist. |
A 27-year-old female with primary infertility was seen in our unit in March 2011. She had regular periods and her body mass index was 25 kg/m2. Her baseline investigations were normal and ultrasound report revealed polycystic ovaries. Her husband's semen-analysis report revealed azoospermia. His clinical and biochemical findings were suggestive of obstructive azoospermia. After counseling regarding the treatment options available, a diagnostic percutanous epididymal sperm aspiration was performed which revealed presence of motile sperms. The couple decided to go ahead with ART.\nCycle programming was done using combined oral contraceptive pills and controlled ovarian hyperstimulation was started after onset of menstruation using with recombinant follicle stimulating hormone (FSH) and multiple dose flexible antagonist (0.25 mg) regimen was followed. Injection human chorionic gonadotrophin (hCG) 5000 international units (IU) intramuscular was administered once more than three follicles reached a diameter of 17 mm. Serum estradiol (E2) on the day of hCG trigger was more than 3800 pg/mL and expected number of follicles was 14-15. She underwent ultrasound guided transvaginal oocyte retrieval and a total of 13 metaphase II oocytes were retrieved. She received 100 ml infusion of human albumin (20% solution) over 4 h, prophylactically after oocyte retrieval. Intracytoplasmic sperm injection was performed using surgically retrieved sperms and 10 fertilized oocytes were obtained the next day. On day 2, three grade 2 cleavage embryos were transferred.[] Supernumerary embryos were cultured and eventually two blastocysts were cryopreserved.\nPatient received luteal support in the form of daily vaginal micronized progesterone 400 mg twice daily and was given intramuscular injection of progesterone 100 mg weekly twice. The patient was advised to take high protein diet and to maintain the total fluid intake/output records which was evaluated once in 3 days for signs and symptoms of OHSS.\nOn post-retrieval, day 9, the patient presented with complaints of severe abdominal discomfort, vomiting, difficulty in breathing and decreased urine output. Her abdominal girth had increased from 72 cm to 84 cm. Her blood test revealed hemoconcentration (hematocrit was 54%), raised liver enzymes, and low sodium (122 mEq/L). An ultrasound of the abdomen revealed bilateral enlarged ovaries (right ovarian size was 11 × 10 × 9 cm and left ovarian size was 10 × 8 × 8 cm) with significant ascitis []. Chest X-ray (an abdominal shield was used) showed bilateral pleural effusion. The patient was diagnosed with severe OHSS and admitted to the intensive care unit. Intravenous fluid administration, analgesics, anti-emetics, and prophylactic anti-coagulation with low molecular weight heparin 2500 IU subcutaneously were initiated. After stabilization, patient underwent therapeutic pleural tap and ultrasound-guided abdominal paracentesis. A total of 2.5 L of ascitic fluid was drained. The patient's symptoms were relieved temporarily. The patient's clinical and biochemical parameters were monitored on a daily basis. The patient was advised to wear elastic stockings and high protein diet intake. Infusion of human albumin was also given once daily.\nHowever, within 2 days the patient had recurrence of abdominal discomfort and difficulty in breathing due to collection of ascitic fluid. A repeat paracentesis was carried out and further 2.5 L of fluid was drained out. Barring low albumin levels (2.1 g/dL) rest of the biochemical parameters were stabilizing (hematocrit 34%). However, despite a high protein diet and daily infusion of albumin, the albumin levels kept deteriorating. Further she developed severe anaphylactic reaction after human albumin infusion on day 13 post-retrieval, which necessitated discontinuation of infusion. Despite all prophylactic measures prompt recognition and early aggressive treatment of OHSS, the patient's condition did not show any signs of improvement. She had rapid re-accumulation of ascitic fluid and due to worsening of symptoms, we had to resort to repeated paracentesis in order to relieve her of her discomfort. However, the serum albumin levels had fallen to alarming levels (1.7 g/dL) partly due to ascitic fluid tapping. The patient developed signs and symptoms hypoalbuminemia in the form of generalized puffiness of the body, face and limbs, and developed vulval edema. Her serum beta HCGs levels were 30 mIU/L (day 10, post-retrieval). With early pregnancy, worsening late OHSS, and severe hypoalbuminemia, rapid re-accumulating of ascites and drainage lead to loss of protein rich fluid, inadequate replacement of protein lead to further lowering of albumin, further contributing to rapid re accumulation of ascitis. Abdominal paracentesis was done 6 times and on day 20, after consulting the medical oncologist, who was familiar with treatment of rapidly accumulating ascitis, as a last resort we decided to transfuse the patient with thawed plasma.\nPatient was transfused four units of thawed plasma in 48 h. The transfusion volume was calculated (10-15 mL/kilo body weight). This resulted in a dramatic improvement in the patient's condition as reflected by significant increase in urine output and stabilization of abdominal girth (indicating no clinically significant re-accumulation of ascitic fluid). Most importantly her serum albumin level started rising (2.5 g/dL immediate post-transfusion). No further paracentesis was required and patient was discharged day 26 post-retrieval.\nThe patient's serum beta HCG levels on day 18 after oocyte retrieval was 522.4 mIU/L and after 14 days the transvaginal ultrasound revealed twin intrauterine gestation sacs with a yolk sac and a fetal pole in each sac but no cardiac activity []. Subsequently, a repeat ultrasound was done a week later and the patient was diagnosed with missed abortion and the pregnancy terminated. The patient was discharged and planned for frozen embryo transfer at a later date. |
A 68-year-old Asian man presented to our out-patient department with lower back and bilateral leg pain for 1 year. The symptom was described as a “tingling sensation,” and the pain in both his thighs was aggravated by walking for more than 20 minutes. The diagnosis was neurogenic intermittent claudication, which was not relieved by analgesics, muscle relaxants, and other medications. Lumbar spine magnetic resonance imaging showed degenerative spondylolisthesis and severe degenerative central canal and bilateral foraminal stenosis (Fig. ).\nAt first, we performed a transforaminal nerve root block, but his symptoms did not improve. We discussed his condition with a specialist from our anesthesiology department to formulate a plan for managing his symptoms. We decided to perform percutaneous epidural neuroplasty with a Racz catheter. If his symptoms and signs were not relieved with neuroplasty, a surgical procedure would be considered.\nAfter obtaining our patient’s informed consent, we transferred him to our operation room, and he was placed in a prone position. Following sterile preparation and draping, we inserted a 16-gauge RX Coudé needle through his sacral hiatus. We performed epidurography with 10 ml of water-soluble contrast media. Before the epidural catheter was inserted, we checked its integrity by injecting 1 ml of sodium chloride to flush the catheter. The Racz neuroplasty catheter was inserted through the needle under continuous fluoroscopy. Undue resistance or abnormality did not occur during the insertion of the catheter and injection of materials. We injected 1500 units of hyaluronidase in 10 ml of normal saline. In addition, 9 ml of 0.125 % bupivacaine and 4 mg of dexamethasone were injected. After the injections, the anesthesiologist tried to pull out the catheter in the epidural space. During the procedure, our patient experienced severe burning pain on both buttock areas and suddenly moved his back and legs on the surgical table. We made him relax by injecting analgesics. Following this event, our patient complained of radiating pain on his left buttock. The anesthesiologist felt resistance while removing the catheter. Under fluoroscopy, the catheter could not be removed because it was trapped in the left foraminal portion of the vertebra. The distal tip of the sheared catheter was exposed and was palpable in the subcutaneous layer of the puncture site. As the anesthesiologist pulled the end portion of the catheter, the wire of the epidural catheter stretched out of shape and was not removed.\nDuring the procedure, the epidural catheter was finally broken. The anesthesiologist continuously tried to remove the broken catheter in the coccyx but was unsuccessful. Subsequently, our patient experienced more severe radiating pain in his left leg. Lumbar spine three-dimensional computed tomography (CT) revealed that the epidural catheter tip was located at the left neural foraminal inlet at the lumbar vertebral body 5 (L5) to sacral vertebral body 1 (S1) level (Fig. ). We explained the errant issue and its possible complications to our patient. We decided to perform surgical removal of the broken neuroplasty catheter with his consent. He was moved to our operating room and placed in a prone position under general anesthesia.\nA total punch laminectomy was performed, starting from the lower margin of the lamina of lumbar vertebral body 4 (L4). The catheter was confirmed to be present in the inlet area of the left L5 to S1 facet and was removed by retraction. The size of the retained catheter was 12 cm (Fig. ). After identifying the nerve root of L4, we performed foraminotomy. The nerve root of L5 was trapped in the bony structure. The facet joints on L4 to L5 were totally removed. Subsequently, both L4 nerves were released. While the thecal sac and nerve root were retracted, discectomy was performed at the L4 to L5 level. We performed lumbar interbody fusion with cages and transpedicular screw fixations on L4 to L5 and then closed the surgical wound. Our patient’s symptoms subsided after the surgery. There was no evidence of infection at the operation site. During a 12-month postsurgical follow-up period, our patient experienced no symptoms and no neurological deficits. |
This 14-year-old girl was referred to our hospital from a peripheral gynecologic unit. She was mentally dull and noncommunicative. The mother stated that for the past 18 months the patient used to cry every month for a few days with abdominal pain. She was found to hit her abdomen with the pain in the recent months. Initially, the pain was relieved with oral analgesics, but later she required parenteral analgesics for pain relief. Prior to her referral to the hospital, she was reviewed by a urologist in another hospital who found a right-sided hydronephrosis and hydroureter and treated her with a double J-stent which relieved her loin pain.\nThe mother noticed gradual abdominal enlargement in her daughter who had never menstruated.\nOn abdominal examination in our unit, a pelviabdominal mass reaching the xiphisternum was found which was tense and tender with a smooth contour.\nAn ultrasound scan of the pelvis and abdomen revealed a blind upper vagina and bicornuate uterus with the right horn containing the hematometra and fundus reaching the epigastrium. The left horn was rudimentary and noncommunicating with the endometrial cavity. There was a right hemorrhagic tuboovarian mass 7 cm × 7 cm with multiple cysts in the ovary. The left ovary appeared normal.\nBoth the kidneys and ureters were normal. Pelviabdominal magnetic resonance imaging (MRI) confirmed the above findings. Intravenous pyelography was within normal limits.\nA decision to undertake a laparotomy was taken to relieve her pain caused by the hematometra. This was explained to the parents, and written consent was obtained for possible hysterectomy.\nExamination under anesthesia revealed a blind lower vagina that measured 1.3 cm. On rectal examination the pelvis was tense and filled with the lower end of the hematometra. As vaginal drainage of the hematometra was impossible, the abdomen was opened by a vertical midline incision reaching up to 3 cm above the umbilicus. Manual exploration of the mass revealed a bicornuate uterus with a rudimentary horn on the left side and a normal ovary. The right cornu was massively distended reaching the xiphisternum. There was gross hematosalpinx on the right side glued together with an enlarged ovary forming a tuboovarian mass measuring approximately 8 cm × 8 cm.\nAs it was not possible to deliver the uterus, it was decided to drain the hematometra by a midline bore metallic suction drain which was inserted in the upper anterior uterine wall. At least 2 liters of tarry fluid was drained. This shrunk the conical uterine mass to one-third its original size and could then be delivered through the abdominal incision (). No cervix and vaginal could be palpated.\nRoutine hysterectomy along with the left rudimentary horn and right salpingo-oophorectomy was carried out (). The left ovary was conserved. The patient had an uneventful postoperative recovery. She was allowed home on the 7th day after operation. Histopathology reported the right ovarian mass to be mucinous cystadenoma (8 cm × 7 cm) and a rudimentary left horn of the uterus with nonfunctioning endometrium. |
A 68 year old man with stiff knee due to long standing osteoarthritis of underwent primary TKA (LCCK, Legacy Constrained Condylar Knee Nexgen, Zimmer, Warsaw,USA) (Figures and ). Preoperatively, he had flexion contracture of 30 degrees with only 10° of further flexion possible. There was a varus deformity of 10 degrees with no noticeable instability. Apart from long standing hypertension of 15 years, there was no other co morbidity. Due to stiffness of the joint, extensive soft tissue releases including excision of all fibrotic bands over patellofemoral and suprapatellar areas, debridement of medial and lateral gutters of the knee joints, excision of the lateral aspect of the prepatellar fat pad and quadriceps snip were performed to facilitate eversion of patella and exposure of the joint. Lateral retinaculum was left intact. There were no intraoperative complications and the alignment and stability of the prosthesis was satisfactory. Tourniquet was inflated before cementing for a total duration of 20 minutes. Before closure, tourniquet was deflated and hemostasis was achieved. Immediate postoperative period was uneventful and one unit blood was transfused to compensate for intra operative losses. Drain was removed 24 hrs after surgery. Static quadriceps exercises were started followed by knee mobilization on third day. On fourth post operative day, patient complained of increasing pain and swelling over the knee joint. The swelling was warm, tender, non pulsatile and any passive or active motion aggravated the pain. There was no neural deficit with good posterior tibial and dorsalis pedis pulses and normal capillary refill. Blood chemistry and coagulation profile were normal. Initially the limb was elevated and cold compresses were applied. Within the next two hours, swelling rapidly increased in size extending into distal thigh (Figure ). Urgent Doppler ultrasound was performed which revealed pseudo aneurysm of size 3.5 × 2.3 cm2 arising from a vessel on lateral aspect of knee joint but its vessel of origin could not be identified. A subsequent angiogram revealed pseudo aneurysm originating from superior lateral geniculate artery (Figure ). Using a micro catheter, a sub selective catheterization of the superior lateral genicular artery was performed and the artery was embolized by means of 3 mm coils (Figure ). The patient experienced immediate relief from pain and swelling gradually decreased in size. The limb was immobilized for next 24 hours after which range of motion exercises were started as tolerated by the patient. Repeat Doppler 24 hr later showed no aneurysm with normal arterial and venous refill. There was a marked improvement in functional capabilities with range of motion of 5-100° and no evidence of recurrent bleeding at follow up. |
A 63-year-old previously healthy man presented to our institution with a 4-year history of continuous progressive hip pain and local numbness of right thigh in January 2018. Upon examining and questioning, the patient stated he has been experiencing a gradual increase in his hip pain, as well as worsening numbness and swelling of the right thigh. In the medical journal of his current illness, the pain in his right hip can reach 4–5 points using the visual analogue scale and cannot be alleviated with rest and hot compresses. One month ago, the patient felt that the above symptoms were aggravated, especially during sleeping and sitting. He denied history of injury and any other underlying diseases. No pertinent family history was identified, including hypertension and cancer.\nPhysical examination showed a mass sized 12 × 24 cm in the proximal thigh, which was hard in texture, unclear in boundary, adherence to surrounding tissues, poor mobility, and no tenderness. However, low skin temperature and varicose vein were not found on the surface of the mass. Routine laboratory studies were almost within normal range, except that the tissue polypeptide specific antigen was significantly elevated to 101.55 U/L (normal: <80 U/L). Plain radiographs showed irregular shadow of a soft tissue mass in his right thigh (Fig. A and B). Computed tomography (CT) showed multilocular cystic-solid mass in the right thigh root, with high suspicion of malignancy (Fig. A–E). Magnetic resonance imaging (MRI) of the hip revealed the irregular mass in his right thigh mimicking a parosteal sarcoma (Fig. A–I).\nConsidering the large volume of the mass and possibility of malignancy, surgical exploration and complete tumor resection were performed according to the designed surgical procedure. After successful anesthesia, the patient was placed in a supine position, with the right buttock being cushioned high. During the operation, multiple cystic masses in the deep muscles were seen, with a size of about 24 × 15 × 10 cm extending from the upper middle of the thigh to the right hip (Fig. A–C). Each capsule contains clear yellowish and reddish liquids, which are filled with a large number of round, tough, oval-shaped, white translucent, cartilage-like granules with a diameter of about 0.5 to 2.0 cm. The chondroid granules in the capsule were cleaned completely, the wall of the capsule was separated from the surrounding tissues, and the capsule was excised completely and sent for pathological examination. The incision was closed. Intraoperative blood loss was approximately 900 mL, thus we used erythrocyte 2U. The postoperative pathology confirmed the diagnosis of synovial osteochondromatosis of the thigh (Fig. A–F). Pathological result was positive for Vimentin and S-100. Biopsy samples were negative for AE1/AE3 and EMA, with 5% Ki-67 positive nuclei.\nOne week after the operation, the patient's symptoms improved significantly compared to the preoperative status. Postoperatively, visual analogue scale score of his hip pain improved to 0–1 points compared to the preoperative status, 4–5 points. At a 1-year follow-up visit, the patient was doing well, with no local recurrence or new symptoms. There were no complications associated with the operation during the follow-up period. |
A 29-year-old male with a history of right tibial shaft and lateral malleolus fractures following a fall injury underwent open reduction and internal fixation with a locking plate for the fibula fracture and minimally invasive percutaneous osteosynthesis for the tibial shaft fracture at another hospital. Postoperatively, the patient was followed up at our hospital for 1 year due to residential issues, and the removal of the plate was scheduled after confirmation of bone union. According to the previous hospital’s medical records, a 9-hole locking plate (APIS®, Gwangju, Republic of Korea) was used for the distal tibia, with three 5.0-mm locking screws for proximal fixation and six 3.5-mm locking screws for distal fixation. Under spinal anesthesia, a plate removal surgery was performed, following which the fibular plate was easily removed. However, five of the six distal locking screws were damaged, and they could not be removed with a screwdriver. This situation was explained to the patient during the operation, and we could confirm that the patient wanted to remove the plate irrespective of the amount of time required. At the time, a tourniquet was used for approximately 1 h, and screw removal was attempted using a conical extraction screw, but only one of the six screws could be removed. Previously proposed techniques requiring additional skin incisions were not considered because there were multiple damaged screws that could not be removed, these techniques would require extended amount of time for removal, and the surrounding soft tissues could be damaged. Hence, we decided to use a screw extractor (IRWIN®, Huntersville, NC, USA), a non-medical instrument, following sterilization with ethylene oxide. In brief, 6-mm drill bit of the extractor was used, four screw heads were drilled, the locking screws and locking plate were separated, and the plate was removed. Of the remaining four screws, one was removed using a vice grip; the remaining three screws could not be removed with the vice grip owing to a short extruded portion. A hollow reamer could not be used because the three screws were adjacent to each other and the reamer could cause a large bone defect; hence, we created sufficient space around the screws by forming small holes around them with a 1.8-mm K-wire, following which all screws could be removed using the vice grip. After saline irrigation, debridement of the soft tissues contaminated with metal debris was performed. The sizes of the metal debris were found to be relatively large, which facilitated easier debris removal. With 2-h use of a tourniquet, all implants could be removed and the skin could be closed (). Postoperatively, no complications were noted during physical and radiological examinations. Remarkably, no problems were noted at the final outpatient visit at 3 months after discharge; therefore, follow-up was terminated. The patient had not visited an outpatient clinic for more than a year since the last follow-up, thus we thought that he had no specific complications including infection and re-fracture. |
A 64-year-old female patient presented to her general practitioner with a 2-month history of left upper quadrant pain. There had been no history of trauma or any specific precipitating event. No change in bowel habit was documented. On examination, she was a well-looking lady with mild left upper quadrant tenderness. No other significant findings were elicited at this time. Following a non-conclusive ultrasound, the general practitioner referred the patient to a general surgeon for further investigation.\nAfter review of the history and examination, the general surgeon’s differentials were diverticulosis, inflammatory bowel disease and colon cancer. The patient was investigated with a computed tomography (CT) scan of the abdomen. Limited biochemical tests were ordered, mainly urea and electrolytes, which were essentially normal. Helical CT images were acquired from the lung bases to the symphysis pubis with multiplanar reformats, post intravenous and oral contrast administration ( and ).\nGiven the concern for a possible colonic adenocarcinoma, the patient was taken to theatre for surgical exploration. Drainage of the collection and a hemicolectomy was performed. Intraoperatively, a distal transverse colon ‘tumour’ with an abscess was identified. There were adhesions between the colon and jejunal loops in the left upper quadrant with an associated collection. The collection was drained and the resected bowel was sent for histological analysis. The patient had an uneventful recovery and was discharged home after a week.\nUpon opening the large bowel, gross pathology indicated a mass lesion (). The cut surface was variegated and an abscess cavity was noted. There was no extension of the lesion into the adherent small bowel.\nSince the histologic findings did not correlate with the clinical diagnosis, the specimen was re-examined to exclude a sampling error. A small opening was identified in the bowel wall and further sectioning revealed a fish bone ( and ).\nThis article followed all ethical standards for carrying out research. |
A 27-year-old male with no known co-morbidity sustained penetrating injuries to the perineum, rectum, bladder neck, and bowel following a fall on a sharp object. After initial resuscitation, he underwent surgical repair of his injury with ileostomy and primary repair of bladder neck injury. The perineum was contaminated with rectal content. After debridement and hemostasia, a delayed closure was planned. Patient had a stormy course in the intensive care unit (ICU), with the delayed abdominal closure being followed by wound dehiscence, ventilator-associated pneumonia and adult respiratory distress syndrome (ARDS). He was managed with ventilator support, antibiotics, parenteral nutritional, and thromboprophylasix with low molecular weight heparin (LMWH). Despite his stormy course, he was recovering from ARDS and healing perineal wound; In the 3rd week of his ICU admission, he had sudden bleeding from the bladder neck manifesting as hematuria through Foleys catheter and pericatheter bleeding from the urethra. The bleeding was managed with surgical exploration of perineal wound and direct compression. He was transfused with two unit of packed red blood cell (PRBC), and 1 gm of TA was ordered. But, by mistake, the patient received 10 gm of TA as bolus, followed by 1 gm infusion for 8 hrs. Patient was stabilized and was doing well on a ventilator until the attending nursing staff noticed pallor and coldness of the right lower limb after 6-8 hours of TA administration. Suspecting acute arterial occlusion, an urgent contrast angiography was done, which revealed arterial thrombosis involving right common iliac artery extending from the bifurcation of aorta up to the proximal femoral artery [].\nConsultation of a vascular surgeon was sought; the patient was systemically heparinized as per the hospital protocol to keep activated partial throboplastin time (aPTT) 2-2.5 times the control value. A few hours later, there was increasing pallor of the limb with loss of arterial pulsations in the entire lower limb with associated swelling. Immediate decompressive fasciotomy was done to prevent further ischemic injury. In view of worsening ischemic changes, the patient was planned for aorto-femoral bypass grafting. But, before he was prepared for the operating room, there was return of blood flow, skin color and arterial pulsations to the lower limb. Surgical intervention was postpone. Eventually, the patient recovered well from the arterial thrombosis and also from ARDS and was discharged from the ICU after about 10 days from the arterial thrombosis on LMWH prophylaxis. |
A 23-year-old female presented to our institution with abdominal pain, nausea, vomiting, fever, constipation, jaundice, and swelling of the abdomen. Considering her past medical history, the patient's symptoms had started after a complicated laparoscopic cholecystectomy 3 weeks ago, where during surgery, the patient suffered a massive bleeding due to porta hepatis injury, which led to the decision to change to open surgery (open cholecystectomy) and as a result the bleeding could be controlled. Physical examination revealed jaundice, palpitation, paroxysmal nocturnal dyspnea, mild cough with white sputum, polyarthralgia, bruising around the umbilicus, and there was no lymphadenopathy. The patient had an abdominal drainage, and about 200 mL of biliary secretion was coming out through it per day. Abdomen multislide computed tomography showed a cut injury in the right portal vein and right hepatic artery, with an abscess in the right hepatic lobe associated with necrotic areas in liver segments 5-6-7-8 (hepatic infraction), and there were clips in the right branch of porta hepatis (right portal vein and right hepatic artery) (Fig. , ). The patient was prepared for endoscopic retrograde cholangiopancreatography to locate the injury and possibly treat it. Endoscopic retrograde cholangiopancreatography showed that the duodenum and papilla were normal, the common bile duct was reflected, and it was interrupted at the level of the gallbladder clip. Intrahepatic bile ducts were not drawn. A sphincterotomy was performed, a catheter was inserted in the papilla, and the common bile duct was tied at the bifurcation. Despite the sphincterotomy, biliary secretions continued to come out through the abdominal drainage. So, based on clinical and radiological findings, we decided to do a laparotomy. Intraoperatively, we found a massive adhesion because of the last operation in addition to necrotic tissue and light local biliary peritonitis. Because of a fragile tissue, our very careful dissection in the porta hepatis in order to identify the right branches of the hepatic artery and the portal vein was unfortunately useless, so we removed the necrotic tissue and put drainage in the space. We decided to finish the procedure at this point to avoid any further injury and to reduce the postoperative morbidity. The patient did not need any intraoperative blood transfusion. Except for a biliary fistula, the postoperative period was uneventful. The patient was followed up for 7 months and she was in good general condition, and the biliary fistula stopped after 2 months. |
A 3.5-year-old girl was referred to a tertiary care hospital in Riyadh, Saudi Arabia from one of the Ministry of Health Hospitals, for neuromuscular evaluation of congenital contractures on August 6, 2011. The patient's mother confirmed a full term of a normal pregnancy with normal fetal movements which is a sign that the fetus is growing in size and strength. Cesarean section was recommended for delivering the patient due to prolonged labor. Although there were no perinatal problems reported, the patient was born with contractures particularly affecting her fingers, knees, and feet. Over time, those contractures improved with periodic physiotherapy sessions particularly her thumbs and 4th and 5th fingers which were often flexed in the palm. According to her parents, the patient is cognitively intelligent, and both her speech and language are matching the 99th percentile. On the contrary to the negative progression of this condition, there was a significant improvement where she was able to crawl and stand on her knees at an early age. Moreover, she is not on any medications, has no known allergies, and has never been hospitalized nor had any surgery.\nThe patient is followed regularly by orthopedics for consideration of soft tissue release particularly with current talipes more obvious on the left side. She also has pterygium webbing of the knee joints limiting them from the extension. Whereas ES has an unknown incidence, it is more common among children from consanguineous relationships. Therefore, the family pedigree was checked, and it was found that the parents are double first-degree cousins, and they have 2 girls and 1 boy who are healthy with no similar family history reported.\nUpon arrival to the hospital and after documenting the patient's medical history, physical examination showed the following: temperature 37.1°C, respiratory rate 20 breaths/min, pulse rate 96 beats/min, blood pressure 110/60 mmHg, and blood oxygen saturation 96%. Furthermore, weight was 9.5 kg, head circumference was 45 cm, and cranial nerve examination was unremarkable. She has, however, some distinct dysmorphic facial features (ie, triangular face with micrognathia and prominent forehead, narrow palpebral fissures, deep-set eyes, and low-set ear). There was no tongue fasciculation. On the neuromuscular assessment, she showed isometric strength around the shoulder, the elbows, and relatively preserved strength with finger flexion. The hands, however, showed mild deformity of the 4th and 5th fingers and the creases present with no obvious hypothenar muscle eminence. In the lower extremity, a clear thinning of the leg muscles was detected compared to the thigh associated with pterygium behind both knees limiting knees extension. In addition, the Achilles tendon shortening was noticed more on the left side with inversion, whereas limited ankle dorsiflexion appeared on the right side with weakness distally. Reflexes were difficult to elicit, but responses were +1 around the knees and the biceps level. Clinical examination of the patient's back showed subtle scoliosis but when pulled to a sitting position the head was lagging and the patient was not able to hold the head up for longer periods, however, she was not very cooperative according to her parents.\nComplete blood count and routine chemistry profiles were in normal ranges. White blood cells and differential count (lymphocytes) study was also normal, including Human leukocyte antigen, blastogenesis normal responses, ratios, CD4/CD8 ratios, Antihuman B lymphocyte serum, and T-helper cells. At the genomic level, an array comparative genomic hybridization using microarray was used to detect any chromosomal copy number changes on the genome which appeared normal without imbalance. Also, the patient's Karyotype was normal. The skeletal survey showed normal bone density, dextrocardia with left-sided aortic arch and stomach, and no signs of skeletal dysplasia (Fig. ). The anteroposterior radiographs of the pelvis showed normal articulation with slight breakage of the Shenton line (Fig. ). Abdominal ultrasound showed normal liver, gallbladder, spleen, and pancreas. Multiple bilateral renal cysts were observed in both right and left kidneys measuring 4.9 and 5.7 cm, respectively. Knees magnetic resonance imaging (without contrast) showed bilateral knee flexion contracture deformities of the posterior fibrotic bands/pterygia with the more posterior location of the neuron bundle. Echocardiography showed dextrocardia with situs solitus and persistent left superior vena cava to the coronary sinus. The very small patent ductus arteriosus producing right-to-left shunt and patent foramen ovale causing left-to-right shunt were confirmed. Electrocardiogram showed normal sinus rhythm with dextrocardia (Fig. ).\nThe patient was followed in the orthopedic clinic and underwent an operative intervention to correct the bilateral fixed flexion deformity at her knees which was related to the posterior bilateral fibrotic bands/pterygia. Surgical realignment was rendered, and a complete extension was obtained. Post-operative, the patient used cast and extension night splint. During follow-up, the patient had no complications, and she was compliant to the regular orthopedics appointments and the periodic physiotherapy sessions. |
A 50-year-old male smoker presented in the emergency unit with acute generalized abdominal pain and guarding in the epigastric and right upper quadrant region. The patient complained of abdominal pain for the last 12 hours with two episodes of vomiting in the last five hours and complete constipation for two days. The patient is a known regular user of over-the-counter nonsteroidal anti-inflammatory drugs (NSAIDS) for more than 10 years for his osteoarthritis and myalgias.\nThe patient had a distended centrally inverted abdomen with thoraco-abdominal respiratory movements. A dull percussion note was present at the flanks with decreased bowel sounds. No visceromegaly was noted on physical examination. The patient was febrile with a temperature of 100℉ though the rest of his vitals were stable with a heart rate of 87 per minute, respiratory rate of 17 per minute and blood pressure of 130/90 mmHg. A pre-rectal examination showed a collapsed rectum, normal prostate palpation and tenderness on deep bimanual palpation.\nAfter a brief history and physical examination in the emergency room, the patient was admitted and a thorough workup panel was requested. The complete blood panel showed neutrophilic leucocytosis, though renal function tests, liver function tests, urine complete analysis, serum electrolytes and erythrocyte sedimentation rates were all within normal range. Further workups for hepatitis B antigen and antibody for hepatitis C showed no viral antigenicity. Serology for helicobacter pylori was also negative.\nA differential diagnosis of gastritis and duodenal perforation was made owing to the symptoms and long usage of NSAIDs. Pancreatitis, biliary pathologies and bacteremia were considered second options in finalizing the diagnosis. The patient was sent for a radiological consult where his radiographs, abdominal ultrasound, and computerized tomography were done (Figures -).\nAfter making the final diagnosis, the surgical team decided to treat the patient with a unique and non-invasive approach. To start the management, a nasogastric tube was passed and all the gastric contents were removed. This step requires special expertise as an improper removal of gastric contents will hamper this method. An intravenous administration of a proton pump inhibitor, Risek™ (omeprazole) 40 mg over 24 hours and H2 blocker, Zantac™ (ranitidine) 150 g over 24 hours were initiated. Along with these anti-gastric acid therapies, an intravenous injectable antibiotic Tanzo™ (tazobactam sodium) was given every eight hours. The patient was closely monitored for addressing any signs and symptoms. A surgical team was prepared as a backup for any invasive procedure in case of failure of this conservative management. During the course of 24 hours, the patient’s symptoms were gradually alleviated and a drastic drop in white blood cells was also observed. This approach brought acceptable results.\nThe abdominal girth was measured and regular palpations were done to check for any reoccurrence of the symptoms. The patient passed flatus on the third day and passed stool on the fourth day of admission. The patient graded 0/10 on the pain scale on the fifth day. Ultrasound was repeated and no signs of free air or fluid were seen. The patient was advised to consume semisolid food items and was discharged on the ninth day of admission. An anti-ulcer therapy with a proton pump inhibitor, oral tablet Risek™ (omeprazole) was prescribed for the next six weeks. |
A 36-year-old woman, had undergone in vitro fertilization and embryo transfer, was referred to our institution for evaluation of fetal hepatosplenomegaly at 37 weeks' gestation. The index pregnancy was otherwise uncomplicated. Her past history was uneventful, and the results of antenatal laboratory tests were normal. A fetal heart rate was about 150 beats per minute. Fluctuations in the fetal heart rate are called variability were normal without distress sign. A transabdominal ultrasonography with 3.5-5 MHz transducer (Voluson 730 pro, GE, Seoul, Korea) revealed a single, live fetus with measurements corresponding to the gestational age except abdominal circumference, due to hepatosplenomegaly. Findings of fetal hydrops also were not observed. A total amniotic fluid index measured 17.5 cm which is normal range. Hepatomegaly means that the liver reaches into the fetal pelvis adjacent to the fetal bladder. Length of liver in this period is normally varies from 40 to 60 mm. It can be measured from the dome of the diaphragm to the tip of the right lobe parallel to the long axis of the fetal trunk []. Splenomegaly is diagnosed by three findings. First, the stomach is displaced anteriorly and to the midline by the enlarged spleen. Second, the spleen is very close to the anterior abdominal wall. Third, color Doppler imaging depicts multiple ramifications of the splenic artery and vein. Length of spleen in this period is normally measured from 45 to 60 mm []. Echogenecity of both organs is usually isoechogenic. However, in this case, size of liver and spleen were above 95th percentile of the upper limit and also the shows both organs adjacent to bladder, which indicates fetal hepatosplenomegaly. Although limited clinical information was available to discuss the ideal mode of delivery with the parents, we recommended cesarean delivery next day to prompt evaluation of rapid progression of fetal hepatosplenomegaly. At delivery, the newborn, a male, weighted 3,070 g was born and Apgar scores were 5 and 7 at 1 and 5 minutes, respectively. When the newborn was physically examined, it revealed unstable respiration with tachypnea and desaturation due to abdominal distension. Intubation was immediately performed and the baby was mechanically ventilated in the neonatal intensive care unit. The abdomen was moderately distended and liver and spleen were palpable 4 and 2 cm below the costal margins. No skin lesions were present (e.g., twenty five to thirty percent of infants with CL have specific cutaneous infiltrates; leukemia cutis which usually appear as firm blue or red nodules called Blueberry Muffin) [].\nOn laboratory tests performed at birth, the hemoglobin concentration was 13.3 g/dL, hematocrit was 42.9%, platelet cells count was 228×103/UL, and white blood cells count was 28.18×103/UL. A differential cell count revealed 25% neutrophils, 44% lymphocytes, 4% monocytes, 3% eosinophils, 0% basophils, 1% myelocytes, and 23% atypical lymphocyte. Few atypical cells were observed on peripheral blood smear and initial peripheral blood chromosome study reveals normal karyotype. Also, serology studies for TORCH syndrome, including serum antibody titers against toxoplasmosis, cytomegalovirus, rubella, and herpes simplex virus and venereal disease research laboratory tests were all negative ruling out infectious condition. Another work-up for septic condition and tandem mass for metabolic disorder were all negative. While further evaluation and monitoring were performed, aspects of hepatic failure were consistently shown with elevated total bilirubin (7.38 mg/dL) in spite of exchanged transfusion for prevention of kernicterus. On peripheral blood test performed on 28 days after birth, the hemoglobin concentration was 10.7 g/dL, hematocrit was 33.4%, platelet cells count was 123×103/UL, and white blood cells count was 67.94×103/UL. A differential cell count revealed 20% neutrophils, 6% lymphocytes, 22% monocytes, 19% eosinophils, 3% basophils, 0% myelocytes, and 30% atypical lymphocyte. Increased numbers of atypical cells which are large-sized immature blasts were seen in a peripheral blood smear (), therefore bone marrow aspiration was performed. A bone marrow aspirate smear showed a moderately cellular marrow with abnormal proliferation of immature blasts (≥35%) and granulocytic precursors and negative for myeloperoxidase, Sudan black B and periodic acid Schiff stain. The erythroid precursors and megakaryocytes were decreased in number (). Immunophenotyping revealed that the 75% of blasts were positive for myeloid leukemic marker of CD33+. The final diagnosis was acute myeloid leukemia. Work-up for detecting metastasis of leukemic cell was done, and there were no specific finding except small ventricular septal defect in computed tomography angiography.\nTo figure out the cause of CL, especially for recurrent genetic abnormalities in de novo AML, fluorescence in situ hybridization (FISH) for core-binding factor subunit beta, myeloid lymphoid lineage, AML1/ETO gene rearrangement were done. Although recurrent genetic abnormalities were not detected, 94% of three AML1 gene signals which could be interpreted as trisomy 21 were detected in this bone marrow specimen. The AML1 (CBFA2) gene, located in the chromosomal band 21q22, has recently attracted a lot of interest in terms of its role in leukemogenesis []. Retrospectively, to investigate the three AML1 signals on initial peripheral blood specimen shown normal karyotype, we performed the FISH, AML1/ETO gene rearrangement. FISH results showed 50% three AML1 signals on initial peripheral blood specimen. Based on the presence of three AML1 signals with normal karyotype in initial study, this patient was thought to have a clonal change of cryptic three AML1 gene signals carrying normal karyotype, and in other words, this result can confirm the presence of CL with AML1 gene duplication. Three AML1 clonal progressions were noted in bone marrow specimen (). Therefore, this patient had very low possibility of Down syndrome except the case of very low level of mosaic Down syndrome.\nEven though the infant was treated with chemotherapy (idarubicin, cytarabine, etoposide, and daunorubicin), ventilator care and inotropic agents, the baby developed increasing respiratory distress and hypotension. He died of respiratory distress with pneumonia at 6 weeks after birth. |
Our case is a 4 years and 8-month-old Syrian Arabic girl with a birth weight of 4 kg, who is the sixth of seven siblings born to healthy consanguineous parents. She followed normal psychomotor development and experienced no remarkable illness until the age of 4 years and 2 months when her parents noticed a rapid weight gain (about 1 kg every 10 to 15 days) due to excessive eating (she required six to seven big meals/day). Later, they observed her to have alterations in body secretion (decreased tears when crying, decreased nasal discharge, unfavorable body odor, and decreased sweating) in addition to blue cold extremities, diarrhea alternated with constipation, polyuria, and polydipsia. Two months after the onset of obesity she had urinary incontinence during night sleep. Then, significant behavioral changes developed including: mood alteration, anxiety episodes, rage attacks, nervousness, and aggressive behavior, in addition to recurrent fatigue, social withdrawal, prolonged periods of sleep (12 hours continuously), and difficulty staying awake during the day. These complaints grew to be a serious concern to the family so she was admitted to Damascus Children Hospital, endocrinal department, at the age of 4.5 years, for further investigations.\nFor three generations, the family history was negative for similar presentations, obesity, or psychiatric disorders. It was noteworthy that an older sister of our patient had died at the age of 12 years with a diagnosis of acute myeloid leukemia (AML). Her sister’s malignancy was not accompanied by any of the signs or symptoms our patient had.\nOn physical examination, general obesity was noticed without striae or altered skin pigmentation. No dysmorphic features were observed, neither were there any minor or major congenital malformations. Her weight was 25 kg (above 97% percentile) while her length and head circumference measured 110 cm (at 90% percentile) and 52 cm respectively. Her body mass index (BMI) was equal to 20.1 (Figs. and ).\nAn ophthalmic examination revealed left exotropia with normal fundus and normal papillary response. The performed laboratory analyses did not indicate any significant abnormality. Her complete blood count (CBC), blood glucose, sodium (Na), chlorine (Cl), potassium (K), calcium (Ca), alanine aminotransferase (ALT), aspartate aminotransferase (AST), triglycerides (TG), cholesterol, urine density, creatinine, and urea were all within normal limits. Her thyroid-stimulating hormone (TSH), free thyroxine (T4), morning cortisol, adrenocorticotropic hormone (ACTH), prolactin, growth hormone (GH), insulin-like growth factor-1 (IGF1), follicle-stimulating hormone (FSH), and luteinizing hormone (LH) were also within normal limits.\nHer abdominal and pelvic ultrasound was normal. Her bone age was slightly advanced and it fitted 5 to 5.5 years old. Brain magnetic resonance imaging (MRI) was normal; it showed no cortical atrophy. A homogenous mild enlargement of her pituitary gland was observed on both T1-weighted and T2-weighted images with normal hyperintense posterior lobe on T1-weighted images (Figs. and ).\nSince the endocrine function tests were normal, her symptoms had been explained on the basis of psychotropic origin, and she was discharged with antipsychotic medication (risperidone 1 mg orally per day) and the recommendation to follow up at the out-patient psychiatric clinic after 3 months of prescribed treatment. After 3 months of discharge, when she was 4 years and 8 months old, she developed dyspnea that worsened with exertion and during sleep, continuous snoring, and recurrent chest pain. The respiratory symptoms lasted for 5 days after which she had two episodes of cyanosis and obstructive apnea which lasted for 10 minutes with improvement via oxygen mask. As a result of progressive respiratory distress (respiratory rate 35/min, expiratory grunting, flaring of nostrils, wheezing with prolonged expiration, and remarkably loud snoring) she was readmitted to Damascus Children Hospital, intensive care unit (ICU). Investigations showed hypoxemia, hypercapnia, and respiratory acidosis accompanied by radiological evidence of a big round opacity in her right lung (Fig. ).\nShe was intubated 24 hours after admission and attached to a mechanical ventilator because of severe respiratory distress with altered consciousness: blood gases were pH 7.27, partial pressure of carbon dioxide (PaCO2) 79 mmHg, partial pressure of oxygen (PaO2) 38 mmHg, and oxygen saturation 79%. Her laboratory results were within normal limits except for elevated values of Na (up to 162) and C-reactive protein (77 mg/L). During this admission, her weight was recorded as 40 kg (>97th percentile).\nA chest computed tomography (CT) scan demonstrated a 6 cm round mass filling most of her right lung and pushing her heart and mediastinum to the left with infiltration and consolidation of the right lower lobe of her right lung (Fig. ).\nSubsequently, when her health stabilized, a complete resection was performed of a 10×10 cm solid round mass from the posterior wall of her chest. It was a mature ganglioneuroma (Fig. ).\nHer unexplained rapid gain of weight and the presence of chest ganglioneuroma brought attention to ROHAAD syndrome as a diagnosis of her situation and helped to exclude other illnesses that could be considered in the differential diagnosis. Familial obesity and Prader–Willi syndrome were both excluded since her family history did not support the first, and with the absence of mental retardation and congenital abnormalities there was no indication to investigate the latter.\nA good improvement in consciousness was achieved by stopping sedative drugs, so she was able to speak, move her limbs, and interact with others. Nevertheless, she required prolonged ventilation because of respiratory instability and she had myoclonic seizures. Three attempts at extubation failed without a clear reason. A brain MRI was ordered which revealed a generalized cortical atrophy of her brain with the same mild pituitary gland enlargement observed in a previous MRI (Fig. ).\nA month after her admission to the ICU, a tracheostomy was performed because of lack of spontaneous breathing. One week later, she had cardiorespiratory arrest and died. A timeline of her signs and symptoms is shown in Fig. . |
A 61 year old, completely edentulous male patient reported to the Oral and Maxillofacial Surgery Department of K.M. Shah Dental College and Hospital, Piparia, India, with a complaint of inability to get dentures constructed due to reduced mouth opening. The patient had history of paan masala (mixture of areca, slaked lime and tobacco) chewing for about 10 years duration. He had noticed a gradual decrease in mouth opening since the last 3—4 years. He reported to have discontinued the habit due to severe burning sensation of the oral mucosa and inability to eat hot and spicy food, since the past 2 years. There was no significant medical history. The patient was diagnosed as having Grade IVA OSMF according to Khanna et al.[]\nImpressions of both arches were made with customized plastic perforated trays. Study casts were then made and occlusal splints were constructed by the heat cure method so that the splints had adequate strength while delivering the inter-occlusal forces. The splints were made such that they had an occlusal table on which the working ends of the mouth gag could be placed. Grooves were made on the splints to facilitate fixation of the splints with wires. Additional holes were drilled in the occlusal table to accommodate the free ends of the twisted wires.\nThe surgery was performed under general anesthesia with nasal intubation. The standard incision was made with a surgical knife. Standard dissection was performed with the help of an artery and finger dissection.\nThe maxillary occlusal splint was placed and secured with per alveolar wiring followed by fixation of the mandibular occlusal splint with circum mandibular wiring. The mouth was then forced open with a mouth gag to achieve a splint to splint distance of 35 mm []. This was considered to be adequate mouth opening for future prosthetic rehabilitation. Bilateral buccal defects of 3.5 cm × 2.0 cm were covered by BFP grafts using the standard technique[] [].\nThe patient was hospitalized for 15 days post-operatively. Ryle's tube feeding was instituted for 1 week post-operatively to maintain oral-hygiene while the grafted buccal fat pad was being epithelized. The post-operative mouth-opening exercise was started as soon as the patient was hemo-dynamically stabilized []. A mouth opening of 30 mm was maintained. By the second post-operative week the patient was trained and motivated to perform mouth opening exercises with the help of the Heister's mouth gag. Parentral medications were given for 7 days post-operatively followed by oral medications for 5 days. Daily intra-oral irrigation was performed with normal saline. The occlusal splints were removed after 4 weeks of surgery. Mouth opening after 4 weeks is shown in . After that, the patient was referred for construction of complete dentures. |
We present the case of a 13-year-old boy admitted to our hospital in September 2018 for severe respiratory distress, a global motor deficit with the lower limbs more affected than the upper limbs, motor regression, axial hypotonia with poor control of the head, muscle strength of 2–3/5 on the Medical Research Council’s scale (MRC) for the upper limbs and 2/5 on the MRC scale for the lower limbs, generalized severe muscular atrophy, retractions of the elbow and knees, more obvious in the distal segments.\nThe patient comes from a non-consanguineous twin pregnancy with term birth and no hypoxic events. He has a 19-year-old healthy brother.\nIn the first year of life, the patient was hypotonic and had a moderate motor delay, sat after 1 year, and walked without support after 2 years. He never achieved running, climbing stairs, or jumping on one foot. His lower limbs have always been more affected than his upper limbs. He had his first neurological examination at the age of 4, where the lack of deep tendon reflexes, tongue fasciculations, neurogenic changes on electromyography, motor deficit with the lower limbs being more affected than the upper limbs and muscular atrophy were observed. Biopsy revealed neurogenic changes raising the suspicion of a motor neuron disease. Genetic testing for 5q SMA – multiplex ligation-dependent probe amplification (MLPA) and Sanger sequencing – was negative. Between 5 and 10 years of age, the evolution was slowly progressive. The proximal weakness became severe, and the patient developed severe generalized muscle atrophy, swallowing dysfunction and ventilatory restriction, severe contractures at all levels, progressive scoliosis, and a lack of deep tendon reflexes. Gastrostomy was performed in 2019. After the age of 10, he became a wheelchair user, and his motor regression was dramatic. In March 2021, spine surgery was performed, after which muscular atrophy became extreme and the weakness was generalized. The patient lost head control and only presented a few active movements in the upper limbs. He presented paradoxical breathing without requiring noninvasive ventilation or oxygen. He had no deep tendon reflexes and presented a dysphonia with a hoarse voice. The patient also had retraction of the mandibular joint with very limited movements leading to the incomplete opening of the mouth, subcutaneous nodules around the interphalangeal joints, with the upper limbs more affected than the lower limbs. He had no paroxysmal events such as myoclonus or other types of seizures.\nElectroencephalography (EEG) showed frequent generalized slow waves with a duration of 1–2 seconds, rarely focalized, with the left side more affected than the right one, without any clinical manifestations. Electroneurography (ENG) showed mildly reduced amplitude of Compound Motor Action Potential (CMAP) but with an increased area (due to the reinnervation process). Sensory Nerve Action Potential (SNAP) had normal amplitude and velocities. Electromyography (EMG) showed chronic denervation changes with very large, polyphasic motor unit potentials (MUP) and incomplete recruitment.\nNo liver enlargement was seen following the abdominal ultrasound. Spinal X-ray showed severe thoracal scoliosis (Cobb angle >100 degrees) with thoracic deformity and restrictive respiratory dysfunction.\nIn order to elucidate the diagnosis, a dried blood spot (DBS) card was sent for sequencing and copy number variation (CNV) assessment (using MLPA) of the SMN1 gene. No clinically relevant variant was identified following SMN1 sequencing and MLPA.\nIn the presence of a negative SMN1 test, we continued the testing using the whole-exome sequencing method. Genetic testing identified three heterozygous mutations in the ASAH1 gene. The first identified mutation (NM_004315.5:c.458_459del) was a two-base pair deletion in position 458 of the coding region in exon 6 generating the substitution of tyrosine in position 153 of the protein to a stop codon – p.(Tyr153Ter), or p.(Tyr153*), leading to a premature stop codon and thus, to a truncated protein. This variant is reported as pathogenic (class 1) according to the American College of Medical Genetics and Genomics (ACMG) guidelines.\nThe second identified mutation (NM_004315.5:c.1226T>C) was the T to C substitution of nucleotide 1226 of the coding region in exon 14, generating the substitution of isoleucine in position 409 of the protein to threonine – p.(Ile409Thr). This variant has been reported as a variant of unknown significance according to ACMG.\nThe third identified mutation (NM_004315.5:c.35G>C) was the G to C substitution of nucleotide 35 of the coding region in exon 1, generating the substitution of arginine in position 12 of the protein to a stop codon – p.(Arg12Pro). This variant is reported as a variant of uncertain significance according to ACMG.\nThe patient received supportive treatment in our Center, including respiratory assessment. Currently, he only needs cough assist 3–4 times/day, does not need oxygen or noninvasive ventilation. He receives daily physical therapy, respiratory and general, daily occupational therapy, neurocognitive evaluation, and psychotherapy. He has no need for anticonvulsant drugs. A severe evolution was noted, and the patient’s prognostic is poor due to rapid motor regression with extremely severe, generalized muscular atrophy and retractions, almost without any active movements, together with respiratory involvement (chronic respiratory failure) and swallowing difficulties (bulbar involvement). |
A 56-year-old female with a past medical history of hypertension and hyperlipidemia was diagnosed ~15 years ago with RA in Honduras. She first established care in our rheumatology clinic ~4 years ago. She endorsed ongoing symptoms of joint pains, joint tenderness and morning stiffness. Her outpatient physical exam was consistent with swelling, tenderness and limited mobility of multiple joints. Her labs were significant for an elevated level of anti-citrullinated peptide antibody, an elevated level of C-reactive protein and an elevated erythrocyte sedimentation rate. Her rheumatoid factor was negative. She also had radiographic evidence of bony erosions on X-ray. In addition, she was evaluated as not having a diagnosis of systemic lupus erythematosus (SLE). Her RA diagnosis met the 2010 American College of Rheumatology diagnostic criteria for the diagnosis of RA.\nThe patient was initially treated with methotrexate, but after failure to achieve remission, methotrexate was stopped and she was started on Adalimumab at a dose of 40 mg subcutaneously every 2 weeks. She continued to have recurrent symptoms of joint pain and her dose of Adalimumab was subsequently increased to Adalimumab 40 mg subcutaneously every week. Approximately 2 weeks after increasing her dose of Adalimumab, she presented to the rheumatology clinic with a facial rash, oral ulcers, bilateral shoulder pain, bilateral knee pain and suicidal ideations. She was sent to the emergency room for further evaluation. She described no previous suicidal ideations or suicidal attempts and stated that she started to develop suicidal ideations over the last few days. On presentation, she was tachycardic with a heart rate of 108 beats per minute and afebrile. Her examination was notable for a facial rash, oral ulcers, mild bilateral shoulder and bilateral knee pain on palpation. Her laboratory results were significant for an elevated anti-nuclear antibody titer of 1:1,280 (normal is <1:80), a strongly positive anti-histone antibody level of 2.6 (normal is <1 Unit) and a borderline positive anti-double-stranded DNA antibody (anti-dsDNA ab) level of 33 IU/mL (normal is <30 IU/mL). She was admitted for an ATIL episode and a psychiatric consultation. The assessment made by the psychiatric consultation team was that while the patient had suicidal ideations, there was no history of a current plan for suicide and her suicidal ideations might be an associated symptom of her current ATIL episode or possibly secondary to her chronic pain and debilitation from her RA. The recommendation from the psychiatry team was close follow-up with better control of the patient’s RA symptoms and to continue to treat the active ATIL episode.\nThe patient’s Adalimumab was stopped at the start of her hospitalization and she was treated with a course of intravenous glucocorticoid. Her joint pains resolved, rashes improved and she was no longer having suicidal ideations. She was subsequently started on Abatacept and closely followed up in the outpatient setting. After a few weeks, her anti-dsDNA abs were repeated and were negative. Remission of her RA was also gradually achieved with no further episodes of suicidal ideations. |
A 40-year-old woman referred to Imam Khomeini Cancer Institute was complaining of intense lower facial pain and progressive swelling for the period of the last six months. Although she has not had a considerable problem in her past medical history, she has also given us a history of her left second premolar and first molar teeth root canal therapy in which she had not noticed any change in pain and swollen area size. She had also undergone incisional biopsy in another health care center with pathologic results designated as a “malignant Pindborg tumor.”\nOn clinical examination, diffuse swelling causing lower lip tenderness and paresthesia with extension to the left posterior part of the mandible was detected. On examination, a mass that measures approximately 4 cm with a rubbery consistency was found. The overlying skin tissue was intact; however, oral mucosa was ulcerated. No palpable subcutaneous cervical lymph node was found. There was no difficulty in swallowing, dysphagia, trismus, or dysphonia.\nOn panoramic and cone-beam computed tomography (CBCT), the lesion with a well-defined border and internal focal radiopacities was seen that extended from the periapical area of the mandibular left canine to the mandibular left second molar in an anteroposterior dimension and from the alveolar crest to the inferior border of the mandible in a superoinferior aspect (). Buccal and lingual cortical plate perforation was also detected. The inferior alveolar canal (IAC) was involved by the lesion, which confirmed the patient's history of pain and paresthesia (). After multidisciplinary evaluations and elucidation of the lesion destructive nature in radiographs, left hemimandibulectomy was performed for the patient ().\nMicroscopic examinations showed odontogenic epithelial neoplasm composed of sheet and island of epithelial cells with malignant features including cellular pleomorphism, hyperchromatism, few atypical mitotic figures, central necrosis, and perineural invasion in a collagenized stroma. At the periphery of the islands, tall columnar cells resembling ameloblast cells with nuclear palisading, reverse polarity, and subnuclear vacuolization are evident. The particular feature of this tumor was the presence of intercellular eosinophilic amorphous materials in extensive areas that had been transformed into mineralized materials in some parts. The lesion was superficially covered by ulcerated stratified squamous epithelium (). Besides, Ki-67 immunostaining was performed, and its expression was noticeable (around 45% in HPF), especially at the peripheral cells and occasionally at the central cells of the tumor islands (). |
SM. A 48-year-old white female presented for evaluation of possible laser vision correction.
Descriptions of her eye findings: 20/20 best corrected vision, normal intraocular pressure, simple myopia, normal cornea thickness, normal pupil size, and normal topography. The significant ocular findings: chronic bilateral optic disk edema for ten years.
In the consideration for the selection of either PRK or LASIK as the procedure for this patient, it is necessary to search relevant evidences to support the decision.
PRK and LASIK are laser eye surgery procedures intended to correct a person's vision to reduce dependency on glasses or contacts.
In PRK, the surgeon removes the outer layer of epithelium with various techniques, then an excimer laser ablates and reshapes corneal tissue. Anesthetic drops are used to reduce pain. LASIK uses a microkeratome device or a femtosecond laser under suctioning to increase intraocular pressure to about 65 mmHg up to 100 mmHg for 45 second to cut epithelium and stroma to a thickness of 100–180 microns, and then, an excimer laser ablates corneal tissue under the epithelial/corneal tissue flap [].
PRK is an excellent alternative when LASIK is not an option. It is suitable for those with less corneal tissue, because it can preserve more corneal tissue. There will be no complications of stromal flap in PRK. Unlike LASIK, it does not require pressure suction on the eye ball. Nevertheless, the drawbacks are more discomfort than LASIK in the first 24 hours after procedure, it requires more healing time than LASIK, trauma may cause complications after surgery, and long-term outcomes are not as well established as older corrective procedures such as LASIK [].
LASIK is appropriate for people who have more corneal tissue. The benefits are less discomfort than PRK, almost no pain, 20/20 vision or better is typically achieved by the first day, corneal haze is very rare, immediate clear vision, and follow |
A 62-year-old male lifelong non-smoker was referred to the head and neck oncology clinic with a five-month history of enlarging right neck mass associated with neck pain, dysphagia, and dysphonia. He was otherwise healthy, aside from type II diabetes mellitus and a distant history of sarcoidosis. Physical examination, as well as contrast-enhanced CT scan of the neck, showed a large base of tongue mass that crossed the midline and projected into the suprahyoid epiglottis. There was a 4.6 × 3.4 cm mass representing a necrotic confluence of lymph nodes in right level IIA and IIB. Biopsy confirmed non-keratinizing, HPV-OSCC. Immunohistochemistry p16 testing was used for HPV diagnosis. PET/CT scan demonstrated bilateral level III lymphadenopathy, but did not show evidence of distant metastases. The patient was diagnosed with a cT4aN2c HPV-OSCC and referred to the institutional tumor board where a recommendation for definitive chemoradiation was made. Staging was according to the American Joint Committee on Cancer Seventh Edition. The patient received 70 Gy in 35 fractions to the primary site and involved nodes, with 56 Gy to the uninvolved regional nodes bilaterally, including the bilateral lateral retropharyngeal nodes. Due to the patient’s comorbidities the concurrent chemotherapy consisted of intravenous cetuximab given as a loading dose of 400 mg/m2 one week prior to the initiation of radiotherapy, followed by seven weekly doses of 250 mg/m2 concurrent with radiotherapy. During and soon after completing chemoradiotherapy, the patient experienced the expected toxicities and had evidence of favourable cancer response to treatment. A contrast enhanced CT scan of the neck performed five weeks after completion documented good anatomic response to treatment with resolution of all disease with the exception of a lymph node within right level II, which was much decreased in size, measuring 2 cm in greatest dimension.\nTwo months after completing chemoradiotherapy, the patient presented to medical attention with onset of binocular vertical diplopia. He was found to have subtle evidence of a new pupil-sparing, left-sided cranial nerve III palsy. His extraocular movement deficits progressed rapidly over one and a half weeks and upon repeat examination he was found to have a new right-sided cranial nerve VI palsy. Differential diagnosis included ischemia, neuromuscular disorder, metastasis, unrelated compressive lesion, and bilateral aneurysm. He was admitted to hospital for investigations. Electromyography showed no evidence of myasthenia or myopathy. Magnetic Resonance Imaging of the sella revealed two separate enhancing cavernous sinus lesions; one tumor in the left cavernous sinus that encased the internal carotid artery with extension into the sella (Fig. ) and another involving the right cavernous sinus and clivus at the level of Dorello’s canal. PET/CT scan demonstrated resolution of his original primary tumor and cervical lymphadenopathy, suggesting complete metabolic response within the chemoradiotherapy field (Fig. ). However, intense focal FDG uptake was demonstrated within the aforementioned two cavernous sinus lesions (Fig. ) as well as FDG uptake within a new 1 cm hypodensity within segment 4A of the liver.\nThe patient underwent Endoscopic Image Guided Transsphenoidal biopsy of the sellar tumor which showed a malignant basaloid tumor consistent with non-keratinizing p16-positive squamous cell carcinoma, likely of oropharyngeal origin (Fig. ) and consistent with early distant metastases from his HPV-OSCC. Discussion with both radiation oncology and medical oncology was held, outlining the poor prognosis associated with metastatic head and neck cancer. The patient began palliative chemotherapy with carboplatin and paclitaxel, and has tolerated this well, with mild improvement in his symptoms in the form of improved extraocular eye movement and return of left eye vision. |
A 13-year-old girl attended our hospital with mild low back pain and a left-sided primarily lumbar scoliosis of 28° Cobb angle. Brace treatment was started, but unfortunately the curves progressed in time. At the age of 15 the Cobb angle was over 50°. An anterolateral correction and fusion from Th12 to L3 was performed by left lumbotomy, using Monarch (Depuy Spine) instrumentation and iliac crest bone graft (Fig. ). After a quick and uncomplicated recovery and a correction up to less than 20° she was discharged from the hospital in 5 days.\nAt routine intervals of 3 months she attended the outpatient department, the rehabilitation program was undisturbed and the curves remained unchanged.\nHowever, 1 year after surgery, she returned somewhat earlier than expected, with a recurrent scoliosis, a significant left sided lateral deviation and flexion of the spine as well as a flexion contracture of the left hip. Pain was not the primary issue. Neither she nor we were able to redress the severe curve. BSE, CRP and WBC were within normal range. Besides this recurrent scoliosis below the instrumented level on plain X-rays and CT-scan, no complications were seen. The instrumented curve and screw position were unchanged and the fusion was solid.\nOn MRI a thickened psoas muscle was seen at the left side with significant oedema due to either infection or neoplasm. CT-guided cultures were taken, but no microorganisms were isolated. Histology reported non-specific inflammation, but not malignancy.\nOver a period of time the general condition of the girl got worse with progression of the curve, lateral deviation and pain. Repeated blood tests now showed a raised BSE, CRP and WBC. Finally, almost 2 years after initial surgery, another MRI-scan revealed a large abscess around the screws and rods as well as in and underneath the left psoas muscle (Fig. ). The hardware was removed, the abscess was drained and a thorough debridement was performed. This time the cultures showed S. aureus for which intravenous antibiotics were administered (Flucloxacilline 1,000 mg, 6 times daily).\nShe recovered very well, without wound healing problems and the infection parameters returned to normal values. The serious derangement of the posture resolved completely. |
A 72 years old woman proceeded to the Emergency Department complaining of acute lower right abdominal pain, mild fever, mild weight loss and constipation. The past medical history of the patient was free.\nThe patient was presented with severe lower right abdominal pain, with signs of local peritonitis and a palpable mass in the same region. The body temperature was 37.2–37.4°C, while arterial blood pressure and cardiac rate 150/90 mmHg and 90/min respectively. The signs of local peritonitis combined with the palpable mass of the lower right abdominal area suggested perforation of the appendix and abscess formation. The laboratory examinations of the patient showed leucocytosis with white blood cell count (WBC) 19000 (with macrophage prevalence: 89%). Colonoscopy revealed obstruction of the right colon. Biopsies were not acquired because the colon lumen was obstructed and the endoscope could not approach the lesion. Computerized tomography (CT) scans of the abdomen (Figure ) revealed a soft lobular mass, measuring 5 × 5 cm, attached to the ascending colon and cecum, compatible with a tumor. The most possible diagnosis was that of a perforated colonic tumor. The patient underwent explorative laparotomy and right hemicolectomy with an end-to-end ileocolonic anastomosis. Thorough exploration of the abdominal cavity revealed no other pathologic findings.\nThe surgical specimen consisted of a 10 cm length of the terminal ileum and the whole of the right colon. The serosa was very heavily covered by suppurative exudates and fibrotic tissue.\nMicroscopic examination of the surgical specimen revealed thickening of the ascending colon wall with neutrophilic infiltration. Numerous polymorphonuclear leukocytes within the muscularis and a fibro-purulent reaction over the serosa with actinomycotic "sulfur granules" in it, were found in high power magnification (Figure ).\nUpon receiving the pathology report, systemic intravenous penicillin treatment was initiated. Therapy continued for 10 days and then followed by oral penicillin for 6 months. No postoperative complications were observed and the patient was discharged the 14th day. |
A 28-year-old man presented at a traumatic surgery unit in an outlying hospital, where an external rotation of the left leg and a patellar dislocation were detected. Examination also revealed clinical signs of a neurofibromatosis-1 (Figure ). The patient had stumbled doing his work as a cook and had fallen on his flexed left knee. Conventional radiography of the knee demonstrated an osteochondral flake near the medial patellar margin, whereas the femoral fracture remained unnoticed (Figure ). After reposition of the patellar dislocation, the joint was stabilized with an orthosis, and the patient was sent home.\nTwo days later, because of increasing pain and swelling of the left knee, his family doctor arranged further radiographic examinations with MRI and CT scans, revealing the Hoffa fracture. The patient was then referred to a medical center for traumatic surgery. Surgical treatment consisted of diagnostic arthroscopy, open reduction of the fracture, and internal fixation with three 40-mm headless compression screws (Figure ). The screws were placed in posterior to anterior and caudal to cranial directions. The anterior horn of the lateral meniscus was fixed to the joint capsule using FiberWire 2-0, and the ruptured retinaculum was fixed to the medial patellar margin with two Mitek anchors. A 3 × 3 cm tumor close to the articular capsule turned out to be a lipoma and not, as initially suspected, a neurofibroma.\nAfter surgery, the knee was stabilized with a knee orthosis, and partial weight-bearing was prescribed for 10 weeks. Fracture healing was regular. In <4 months after the accident, the patient returned to work without any orthopaedic therapeutic appliances. In this case, the occurrence of a femoral fracture after a low-velocity trauma was probably influenced by reduced bone quality because of an underlying NF1, but NF1 did not influence fracture healing. Two years after the operation, the range of motion of both knees was unlimited, and no osteoarthrotic changes were noticed. |
A 64 years old male with NF1 had multiple hospital admissions with abdominal and back pain between 2003 and 2004. He had lost significant amount of weight. As a part of work up he had MRI scan of the abdomen and pelvis. His scans showed a large pre-sacral mass in left retroperitoneal region with possible infiltrative characteristics. This mass extended up to left lesser trochanter and also had some calcification in it ().\nA diagnosis of plexiform neurofibroma was made. In 2008 he had acute abdominal pain following trauma. There was possibility of a haematoma in the retroperitoneal mass on imaging. He had surgery with drainage of haematoma and debulking of retroperitoneal mass. He bled heavily during the surgery and required blood transfusion. The mass removed surgically showed diffuse involvement of nerve segment and its branches with tortuous expansion. Histology revealed spindle cell tumour infiltrating adjacent fat and entrapping nerve bundles ( and ). Tumour cells were positive for S100 protein immunostains. These features were consistent with malignant peripheral nerve sheath tumours. He was reviewed in genetic endocrine clinic. He appeared depressed and was overweight with a BMI of 29kg/m2. He had extensive bruising of skin on his arms and legs and his blood pressure was 160/100mmHg. He had no abdominal striae or proximal muscle weakness. His 24 hours urinary metanephrines and 5 HIAA were normal. His serum potassium, renal function and renal MRI scan was normal. He had a 2 cm enhancing mass in the wall of the second part of the duodenum representing neurofibroma. His gut peptides, chromgranin A and B were normal. His octreotide scan was negative.\nHe had overnight dexamethasone suppression test and his cortisol failed to suppress. He had low dose dexamethasone suppression test (LDDST) combined with CRH stimulation. At baselne he had measurable ACTH of 29ng/L with cortsiol of 689nmol/L. His cortisol was 427nmol/L on day three of low dose dexamethasone and his ACTH was 10ng/L consistent with ACTH dependent Cushing’s. After 100mcg of CRH there was no increase in cortisol or ACTH level. His ACTH precursors were high at 126 pmol/L (). A CT scan of his adrenals and MRI scan of the pituitary gland was normal. Patient did not have inferior petrosal sinus sampling in view of normal pituitary MRI scan, multiple neurofibromas and previous history of bleeding on surgery.\nA diagnosis of Cushing’s syndrome due to ectopic ACTH was made. The source of ectopic ACTH was most likely his large retroperitoneal plexiform neurofibroma with MPNST. This patient was a poor surgical risk for further debulking of the plexifrom neurofibroma. He was treated with Imatinib mesylate at a daily dose of 75 mg/kg for 4 weeks.\nHe had repeat LDDST with CRH stimulation 4 months after the treatment. His cortisol suppressed to 27 nmol/L and ACTH was < 5ng/L on day 3. There was no response of cortisol or ACTH to CRH (). His MRI scan of the abdomen one year after treatment with imitinib showed no increase in the size of plexiform neurofibroma but there is a definite decrease in the density of plexiform mass. Immunochemical staining of the tumour did not show positive staining for ACTH. |
A 23-year-old Yamani woman presented to our emergency department with a case of abdominal pain and distension. Examination and investigations found an uncomplicated umbilical hernia, liver cirrhosis and ascites. The patient was admitted by the medical team, who investigated the patient for two weeks, eventually diagnosing a case of hepatitis B infection and decompensated liver cirrhosis (Child-Pugh grade B). After two weeks under medical care and multiple ascitic tapping, the patient responded with a sudden gush of clear fluid and spontaneous evisceration of the omentum from the umbilical hernia (Figure ). The patient was referred from the medical team as a case of spontaneous umbilical hernia rupture and leaking ascitic fluid with omentum evisceration.\nAn urgent laparotomy was performed after preparation of the skin with an antiseptic solution. Excision of the umbilicus and eviscerated omentum was done. Aspiration of all ascitic fluid and samples were sent for cytology, biochemical, and microbiology testing. All abdominal viscera were examined, but no evidence of the recanalization of the umbilical vein was found.\nAfter placement of an abdominal drain, a blunt dissection was done between the posterior rectus sheath and the rectus abdominus to facilitate placement of the polypropylene mesh in a sublay position. The posterior rectus sheath was closed using 1/0 non-absorbable polypropylene stitch. The mesh was placed over the posterior rectus sheath and below the rectus abdominus. The anterior rectus sheath was closed using 1/10 polypropylene stitch, and the skin was closed using clips.\nPostoperatively, the patient stayed in the ICU for five days and was then moved to a regular ward. She received prophylactic broad spectrum antibiotics and albumin. Her abdominal drain output ranged from 1000-2000 ml clear ascitic fluid per day for one week, then decreased gradually over the next week to around 30 ml. The drain was removed, and the patient was discharged home. During follow-up in the outpatient clinic after two weeks, the patient was doing well with no wound complications. Consequently, the patient was referred to a tertiary center for further management. |
A 56-year-old male was admitted to our hospital with a 6-month history of shortness of breath and palpitation following physical exertion. Transthoracic echocardiography revealed a giant right SVA. The aortic valve was tricuspid with trivial aortic regurgitation and the ascending and descending aorta were within normal limits. The bi-dimensional images confirmed the presence of a right coronary sinus aneurysm with maximum dimensions of 56.82 mm×49.67 mm (). A computed tomography (CT) scan showed the giant right SVA. The proximal right coronary artery (RCA) was not visible, whereas the middle and distal RCA were clearly visible (-). Electrocardiogram was normal.\nSurgery was performed through a median sternotomy. Cardiopulmonary bypass was established by ascending aorta, superior vena cava, and inferior vena cava cannulation with left heart venting through the atrial septum. A giant SVA was observed at the right sinus (). After cross-clamping the ascending aorta, cardioplegia solution was infused through the ascending aorta. The ascending aorta was opened at the border of the SVA and normal ascending aorta. The SVA involved the total right sinus, protruded forward and downward, and the orifice of the RCA could not be found inside (). The proximal RCA on the SVA was occluded and the proximal RCA on the atrioventricular groove had a small lumen and a very thin wall. The middle RCA on the atrioventricular groove was normal. Coronary artery bypass to the middle RCA was performed immediately with greater saphenous vein (GSV) grafting, and cardioplegia solution was infused through the GSV graft (). The right sinus was reconstructed with a vascular prosthesis patch ranging from the aortic annulus to the normal ascending aorta and from commissure to commissure (). The proximal GSV graft was anastomosed to the normal ascending aorta. The cardiopulmonary bypass time was 148 min and cross-clamp time was 108 min.\nThe duration of mechanical ventilation support was 27.25 h. The patient’s duration of stay in the intensive care unit was 89.83 h. The patient’s recovery was uneventful. He was discharged from the hospital 6 days after surgery. A postoperative CT scan showed good aortic root morphology (, ). |
A 51-year-old female was admitted to our hospital with a 3-month history of tonic-clonic seizures lasting up to 20 minutes each, and left-sided limb weakness. Her family reported that she did not sustain any head injuries during her seizures, and always recovered completely without fever, headache, or cognitive dysfunction.\nPrior to admission to our hospital, she had been admitted to another hospital on several occasions. Following her first seizure, which was 20 minutes in duration, she was assessed at a clinic. At presentation, her vital signs were normal, and head CT, MRI, and electroencephalography were normal. She was diagnosed with epilepsy. As no abnormalities were detected on imaging studies and she had no previous history of seizures, she was not started on prophylactic anticonvulsive medication. She was asymptomatic until 25 days later, when she had a tonic-clonic seizure lasting approximately 10 minutes. She was assessed at the emergency department. Head CT showed a low-density lesion in the right frontal lobe, and MRI showed an area of abnormal signal in the right frontal lobe. However, magnetic resonance angiography of the head was normal. Full body positron emission tomography demonstrated decreased uptake of fluorodeoxyglucose in the right frontal lobe.\nShe was admitted to hospital. Her vital signs were normal. Serum electrolyte, blood urea nitrogen, creatinine, glucose, albumin, bilirubin, alkaline phosphatase, aspartate aminotransferase, cholesterol, triglyceride, and low-density lipoprotein cholesterol levels, and red blood cell and platelet counts, were normal. She was diagnosed with epilepsy and treated with intravenous fluids, mannitol, and anticonvulsant medication (sodium valproate). She improved significantly over the course of her admission. Although she still had occasional seizures, she was discharged home on the 7th day after admission on sodium valproate 0.2 mg, three times daily.\nFor the first 14 days after discharge, she was seizure-free, but gradually developed left-sided limb weakness. On the 15th day, she could no longer move her left arm and leg, and was readmitted to the same hospital. Her blood pressure was 142/98 mmHg (1 mmHg = 0.133 kPa) and her other vital signs were normal. Head CT showed enlargement of the right frontal lobe lesion. Brain MRI showed a right frontal lobe lesion with mixed low and intermediate signal intensity on T1-weighted images, high signal intensity on T2-weighted images, massive edema, insignificant compression of the right lateral ventricle, and no enhancement (Figures –). Lumbar puncture showed a normal cerebrospinal fluid (CSF) opening pressure. CSF analysis showed normal white blood cell count, red blood cell count, protein, glucose, herpes simplex virus and Epstein-Barr virus polymerase chain reaction, toxoplasma titer, and oligoclonal bands. She was still thought to have epilepsy. She was treated with intravenous fluids and mannitol, and sodium valproate was continued. After 7 days, her limb weakness had resolved and she could walk without assistance. She was discharged 15 days after admission. Five days after discharge, her left-sided hemiplegia returned, and she complained of a mild to moderate right-sided headache. She was readmitted to the same hospital. Head CT showed massive edema in the right frontal lobe, with nodular hemorrhage (Figures , ). Brain MRI showed enlargement of the right frontal lobe lesion, and also showed a new lesion with low signal intensity on both T1- and T2-weighted images and diffuse small hemorrhages in the juxtacortical areas, with significant compression of the right lateral ventricle and deviation of the midline to the left (Figures –). She was still thought to have epilepsy, and was treated with mannitol and sodium valproate. However, her symptoms did not resolve.\nApproximately 3 months after the first seizure, she was transferred to our hospital. On reviewing her past history, we learned that she was a farmer, and had a history of type II diabetes mellitus and hypertension, both diagnosed 5 years previously. Both her blood glucose level and blood pressure had been well controlled. She denied the use of tobacco, alcohol, or illicit drugs. Her last menstrual period was 2 years ago, and she did not use any contraceptive medication. She denied any similar symptoms in other family members.\nOn physical examination, her blood pressure was 150/90 mm Hg, and other vital signs were normal. She was alert and oriented, and her speech was fluent. Her pupils were equal, round, and reactive to light. Fundus examination revealed bilateral edema and hemorrhage. Examination of the other cranial nerves was normal. Muscle strength was grade 2 in the left arm and grade 0 in the left leg. She had a positive Babinski sign on the left side. The remainder of the neurological examination was within normal limits.\nThe differential diagnosis included primary angiitis of the central nervous system, tumor, infection, and venous sinus thrombosis. Autoimmune markers (erythrocyte sedimentation rate, rheumatoid factor, antineutrophil cytoplasmic antibody, antiphospholipid antibody, antinuclear antibody), tumor-related antibodies (carcinoembryonic antigen, CA-125, CA-199, CA-724), and coagulation protein levels (antithrombin III, protein C, protein S, lupus anticoagulant, factor V) were all normal. Serological tests for human immunodeficiency virus, Borrelia, and syphilis were negative.\nLumbar puncture showed a CSF opening pressure of 304 kPa. CSF analysis showed a total cell count of 10 × 106/L, white blood cell count of 6 × 106/L, and elevated protein level of 63 mg/dL (normal: 15–45 mg/dL). CSF glucose and chloride levels, oligoclonal bands, 24-hour IgG synthesis rate, and cytology were all normal, and CSF virus antibody testing was negative.\nElectrocardiography and echocardiography were normal, and brain magnetic resonance venography was normal. Our laboratory and imaging findings did not indicate a diagnosis of isolated cortical vein thrombosis.\nAfter the lumbar puncture and blood draw, she was treated with mannitol. Sodium valproate 0.2 mg, three times daily, was continued. There was no further deterioration. Two weeks after admission, surgical excision of the right frontal lobe lesion was performed under general anesthesia.\nPostoperative histological examination of the surgical specimen showed destruction of brain parenchyma with infiltration of macrophages and proliferation of reactive astrocytes and small vessels. There were foci of hemorrhage in the lesion (). Further examination found that a number of small vessels in both the subarachnoid space and brain parenchyma were filled with thrombus, some of which was organized. Elastic fiber staining showed that the obstructed vessels were veins.\nThe patient recovered gradually after surgery. She had residual mild left-sided hemiparesis and occasional tonic-clonic seizures. After 1 month of rehabilitation, she was able to live on her own. DSA at 2 weeks after surgery was normal. At her 9-month follow-up appointment, she still had mild left-sided hemiparesis. She continued taking sodium valproate (0.2 mg, three times daily) and had experienced one tonic-clonic seizure since discharge. All the routine tests that were performed during her initial presentation were repeated, and the results were normal. Brain MRI did not show any new lesions. |
A 38-years-old male from the Indian sub-continent presented to the emergency department with sudden onset of dysphonia followed by dysphagia for both solids and liquids for one week. The patient also reported 5 kg of unintentional weight loss over two months. He did not give a history of eating any spoiled food in the recent past. His voice had become nasal in quality, and drinking more water frequently led to regurgitation of the liquid through the nose, resulting in cough. His symptoms started abruptly without any associated limb weakness. On examination, he was vitally stable, the power in all four limbs was normal, and the reflexes were intact. His planters were bilaterally down-going, and there were no cerebellar signs. On cranial nerve examination, the patient had a deviation of the uvula towards the left side, and there was some palatal droop. The rest of the exam was unremarkable except for the presence of palpable lymph nodes in the right inguinal region.\nBasic metabolic panel at the initial encounter revealed mild hypercalcemia (corrected calcium 2.62 mmol); however, the rest of the electrolytes were within the normal limits. The intact parathyroid hormone (PTH) level was 6.6 picograms per milliliter (reference range: 15 - 65) with low vitamin D (Table ).\nChest and neck X-ray did not elucidate any anatomical pathology as a possible explanation. The initial neurological impression was of bulbar palsy. Due to the presence of neurologic symptoms, stroke was thought of, which was ruled out by a computed tomography (CT) head, followed by a magnetic resonance imaging (MRI) of the head, which showed some hyperintense non-specific lesions in the frontal and the parietal lobe (Figure ). They were considered nonspecific because the patient was hypertensive for the past seven years. Neurologic tuberculosis was another possibility, as the patient originally belonged, and had frequent travels, to a region with high endemicity. Tuberculosis was excluded by a negative QuantiFERON TB test (Cellestis Limited, Carnegie, Victoria, Australia). A negative antinuclear antibody (ANA) and antineutrophil cytoplasmic antibody (ANCA) test which were done after the initial blood investigations were inconclusive; it came back negative, making a diagnosis of connective tissue disease and vasculitis less likely.\nVideo-assisted modified barium swallow (done to assess the swallowing capacity of the patient) showed that the patient had mild palatal abnormality with the partial inability of the epiglottis to cover the respiratory tract, leading to micro aspirations which prompted the insertion of the nasogastric tube to prevent aspirations (Figure ). The patient’s voice became hoarser and nasal over the course of his hospital stay, and his calcium levels were rising gradually. A subsequent contrast-enhanced thoracic CT scan which was ordered while keeping lymphomas as a possible cause of his symptomatology, showed multiple lymph nodes in the paratracheal and hilar regions (Figure ).\nA contrast-enhanced CT scan of the abdomen and pelvis elucidated multiple para-aortic, iliac, and superficial and deep inguinal lymph nodes (Figures -). Inguinal lymph nodes, being sizeable for an excisional biopsy, were sent for histopathological examination, revealing a granulomatous inflammation without caseation with a negative acid-fast bacillus smear and polymerase chain reaction (PCR) (Figures -). Given the exclusion of other possible conditions and suggestive biopsy findings, the diagnosis of sarcoidosis was established at this point. Although asymptomatic, the patient’s calcium level was still elevated, which was managed by intravenous normal saline.\nPulse steroid therapy was started with intravenous methylprednisolone for five days. On the third day of pulse steroids, the patient’s calcium levels returned to baseline, as seen in Table ; however, that patient’s voice and swallowing did not improve even after the completion of the steroid therapy. At this point, a trial of intravenous immunoglobulin (IVIG) at 0.4 g/kg/body weight was started. After two doses of the IVIG, the patient showed significant improvement in his swallowing capacity and dysphonia, and his calcium levels remained normal.\nA repeat video fluoroscopy showed marked improvement in his swallowing capabilities, which led to the removal of the nasogastric tube. The patient completed the course of IVIG for five days and was discharged from the hospital asymptomatic with a follow-up appointment (Figure ).\nFollow-up visits\nOne month after discharge from the hospital, the patient followed up with the pulmonology team. The patient was completely asymptomatic. He was continued on regular steroids with tapering over the next few months. On a subsequent visit with pulmonology after another month, the patient was asymptomatic and did not complain of change in voice or dysphagia. He had a follow-up visit with the neurology four months after his initial discharge, where a complete neurological assessment was done and found within the normal range. The patient had stopped taking steroids at this point according to the schedule; he was kept on regular follow up. |
We present the case of a 48-year-old man, complaining of a bilateral knee injury and functional disability. The patient fell about two meters down an embankment one hour before the presentation and was unable to stand up due to pain, so he was brought to our hospital by ambulance.\nClinical examination revealed a marked bilateral swelling of both knees, severe pain at the passive mobilization of the knee joints, pain-limiting active flexion to less than 30°, and an inability to actively extend the knee joints or to perform an active straight leg raise bilaterally. Additionally, weight-bearing had hardly been possible. There was a loss of fullness and a palpable deficit at the inferior poles of his patellae. Testing of the ligamentous knee joint stabilizers was significantly limited by guarding due to the severe sharp pain. The patient's medical history revealed that a couple of years earlier, he had a right knee sprain resulting in an acute rupture of the anterior cruciate ligament (ACL), which was treated conservatively. The patient does not take any drugs regularly, and we note that he is allergic to levofloxacin. The weight and height of the patient were recorded at the physical examination as 107 kilograms and 180 cm, with a body mass index (BMI) of 33.\nPlain radiographs of his knees showed bilateral knee effusions with patella alta (high-riding patellae) on both anteroposterior and lateral views. Insall-Salvati ratios measured 1.6 and 1.47 for right and left knee, respectively () (normal values range from 0.8 to 1.2; patella alta > 1.2 and patella baja < 0.8). Moreover, the irregularity and incongruity of the patellar tendons on the lateral radiographs were additional signs suggestive of the extensor mechanism's rupture and consistent with the tendons' rupture from the lower pole of the patellae bilaterally.\nOur patient was operated under general anesthesia 48 hours after the accident. He was placed in the supine position. The clinical examination under anesthesia of both knees demonstrated a full instability of the MCL with a valgus stress test at both 0 and 30° of knee flexion. No laxity was demonstrated in the remaining ligaments of the knee joint. The lower extremities were prepared and draped together in the usual sterile fashion. The intervention was performed without the use of a tourniquet.\nOur ligamentous reconstruction was approached by an anterior longitudinal midline incision. Dissection was carried down through the skin and subcutaneous tissues to the level of the patellar and quadriceps paratenon, which were carefully preserved. The patellar tendon rupture was identified near the proximal osteotendinous junction bilaterally (). The hemarthrosis was evacuated, and the joint was copiously irrigated. A chronic tear of the right ACL was identified, with the proximal portion of the ligament missing and only scar tissue remaining with the ACL stump adhering to an intact Posterior Cruciate Ligament (PCL). A complete proximal (femoral) MCL tear was identified bilaterally, confirming our clinical suspicion. The medial and lateral retinacula, which were involved as well, were identified for later repair.\nAt first, with the same incision, the MCL tear was approached. Proximal reinsertion at the level of the medial femoral condyle using a DePuy Mitek super QuickAnchor™ Plus DS® was done to ensure the stability of the medial motion plane. Then, after debridement of the tendinous tissue and visualization of the inferior pole of the patella, three DePuy Mitek super QuickAnchor™ Plus DS® were screwed into the medial, middle, and lateral thirds of the patella in the proper coronal plane. The purchase of the anchors was tested as we were able to deliver the patella to the distal extent of the incision by pulling on the anchor sutures. Then, a circumferential 1.2 mm thick stainless steel wire was passed through the center of the thickness of the patella superiorly and the tibial tuberosity inferiorly. Gradual tension was applied on the metallic wire to obtain the optimal patellar height, confirmed by an intraoperative lateral X-ray. One suture in each anchor was used to create a running Krackow stitch distally through the tendon, ensuring that full-thickness bites were obtained.\nThe second limb of each suture was passed in a locked fashion through the proximal free tendon and tied within the substance of the tendon. The additional suture within each anchor was incorporated into the repair for reinforcement in a simple continuous fashion. The reconstruction was further protected by a strip of quadriceps tendon measuring 10 × 1 cm, long enough to cover the patellar tendon, which was harvested and turned down. The edges of the turned down quadriceps tendon were fixed to the underlying patellar tendon using slowly absorbable interrupted sutures (Vicryl 2.0). Next, the medial and lateral retinacula tears were repaired using interrupted No. 2 PDS sutures (Ethicon; Somerville, New Jersey, USA). The strength of the repair was tested bilaterally through a gentle range of motion; a flexion up to 130° was possible.\nPostoperatively, the legs were placed in knee immobilizer braces, with the knees locked in full extension. The postoperative course was uneventful, and radiographic control was satisfactory (). On the second postoperative day, the patient began ambulation with a walker while keeping the extension knee braces. Full weight-bearing was permitted as tolerated, along with isometric quadriceps-strengthening exercises. The rest of the protocol is as follows: knee flexion exercises limited to 45° were started at the second postoperative week. He had no pain and reached 45° of active bilateral knee flexion. He had an active flexion of 80° at the sixth week, and the knee braces were discontinued. In the eighth postoperative week, the patient achieved a bilateral active complete knee extension and could walk without crutches. As part of his daily physiotherapy program, he was allowed full knee flexion along with a focus on muscle strengthening exercises. Twelve weeks after surgery, the patient presented 100° maximum bilateral knee flexion and returned to work.\nUpon examination seven months after surgery, the patient showed an adequate range of motion of both knees (135° flexion, 0° extension) (). Quadriceps muscle, the primary contributor to knee joint stability, had a good strength with no clinical signs of muscular atrophy or extensor lag. The patient denied any sense of instability, and, consequently, he returned to his recreational sports activities. In addition, he reported feeling that his knees were as strong as they were before the accident. At the final follow-up 12 months after the injury, the patient was symptom-free and extremely satisfied as he recovered completely. |
A 54-year-old man was referred to our institution, he complained pain on his left thigh for 2 years. The pain was felt in the midthigh region. There were no other symptoms from the anamnesis. Patient previously had pathological fracture 3 years ago on the left femoral shaft. It occurs after the patient was slipped on the bathroom and fell. The patient had undergone curettage, open reduction and internal fixation at district general hospital. Histopathological examination after first surgery suggested fibrous dysplasia. On physical examination, the lump was palpable on his postero-medial thigh. The lumps were firm and non-tender on palpation, fixated, and the size was around 14 × 8 × 11 cm ().\nThe patient underwent diagnostical procedures, including a blood test, plain radiograph, and Magnetic Resonance Imaging (MRI). Laboratory findings were within normal ranges. A shows the pre-fracture condition 3 years ago, while B shows the fracture line in the diaphyseal of the left femur. C shows femoral radiograph that revealed lytic geographic lesion at femoral diaphysis, cortical disruption, interrupted periosteal reaction, soft tissue mass, and internal fixation attached to the femoral bone. Femoral MRI indicated a tumor on femoral diaphysis that measured 20 × 10 × 14 cm and displayed low to intermediate signal intensity on T1-weighted images. T2-weighted images displayed a heterogenous lesion, containing areas of low to hyperintense area. Some area showed metal artifacts due to metallic implants (). The histopathological result from core biopsy suggested chondrosarcoma.\nThe patient underwent total femur resection with wide surgical margin. He was placed on supine position. The surgical incision started 4 cm proximal to the greater trochanter, it is brought curved distally to the medial thigh until anteromedial aspect of tibial tuberosity. Superficial femoral artery and vein were identified. Rectus femoris and some parts of vastus lateral muscle were preserved. The tumor was removed with wide surgical margin (). Reconstruction after total femur resection was done using total femur megaprothesis (D). The remaining hip capsule was sutured tightly with mersilene tape. Psoas muscle was tenodesed to the anterior hip capsule. External rotator muscles were sutured to the posterior hip capsule. Remaining abductor tendon is attached to the lateral aspect of the prothesis. Sartorius was sutured to the rectus femoris muscle.\nThe surgery took 7 h. The amount of bleeding during total femur resection was 1300 cc. There was no complication during or after surgery. We mobilized the patient with non-weight bearing and after one month of surgery we change it to partial weight bearing as tolerated. The length of stay in the hospital was 7 days.\nThe histopathology examination from the resected tumor showed cartilage matrix and hypercellular chondrocyte. The chondrocytes were varied in shape, atypical, larger in size, and hyperchromatic nuclei. There were large number of binucleation with mitosis, and same myxoid changes. The conclusion from the histopathology examination was chondrosarcoma grade II ().\nAt the 12 months follow up, the patient was in good condition. Patient had Trendelenburg gait and weak extensor knee muscles. Patient needed one crutch for walking. MSTS score evaluated functional outcomes, where the patient scored 16 or 53%. The patient did not complain of any pain. The patient also showed no sign of recurrence 12 months after the surgical procedure.\nAt follow up, the patient was in good condition and could work normally. |
A 23-year-old woman was diagnosed with AA by the end of 2010. In recent years, she has not had any immune system-related serious illnesses and conditions, such as asthma, allergies, and atopic dermatitis. Because of her condition, she experienced psychological changes in the workplace. She was initially treated with mometasone furoate, a topical corticosteroid, and oral cepharanthine to enhance blood flow. Despite compliance to treatment, AA progressed to diffuse AA. Initially, she was treated with intralesional triamcinolone acetonide (40 mg) five times per week between January and February 2011; triamcinolone acetonide has a potency that is five times that of methylprednisolone. However, her symptoms persisted.\nShe was referred to the dermatology department of our university hospital in June 2012. The result of the pull test at the margin of the alopecic lesions was positive. As further treatment to resolve alopecia, narrowband ultraviolet B phototherapy and squaric acid dibutylester (SADBE) as a local autoimmune treatment were administered as an outpatient for four courses between June 2011 and March 2013. Her response was erratic. Therefore, systemic corticosteroid pulse therapy was administered (). The first course of 1,500 mg corticosteroid therapy in the hospital was administered in May 2013. The first course comprised 500 mg methylprednisolone administered intravenously for 3 days. However, the clinical recurrence of extensive AA, nearly alopecia universalis (), was observed 3 months after the first course. She was administered repeated corticosteroid pulse therapy until the end of 2013. A second course of 1,500 mg corticosteroid therapy was administered for 3 days, but her symptoms did not improve. After the third course of the same corticosteroid pulse therapy, extensive alopecia finally improved to small diffuse alopecia. In March 2014, SADBE treatment was started again as an outpatient. By September 2015, SADBE treatment was replaced by administration of 25 mg/day prednisolone, which was gradually tapered down to 5 mg/day. Her alopecia was resolved through the multidisciplinary approach to the treatment of AA.\nIn February 2016, she complained of right groin pain immediately after sustaining injury in the right leg after almost falling off her bicycle. She was referred to our department for orthopedic evaluation 1 month after the incident. Steroid-induced ONFH was suspected because of prior repeated systemic corticosteroid pulse therapy. Physical examination and MRI scan of the bilateral hip joints showed abnormal signs. The range of motion of the right hip joint was restricted in the direction of flexion, abduction, and internal rotation. Pain in the right groin increased when standing or climbing up the stairs. The T1-weighted MRI of the hip showed the characteristic band pattern on the right and a large diffused low signal area in the left femoral head. Based on these findings, she was diagnosed with stage three postcollapse ONFH on the right and stage one precollapse ONFH on the left (). Thereafter, she complained of continuous and severe right hip joint pain. THA was scheduled and performed for the right ONFH in March 2017. For the left ONFH, she wanted a joint-preserving surgery, wherein core decompression and autologous bone graft were performed (). One year postoperatively, three clinical scores in her hip improved (University of California, Los Angeles [UCLA] activity score, 4; Oxford hip score, 19 points; and Harris hip score [HHS], right: 90.0 points, left: 85.5 points) compared with those preoperatively (UCLA activity score, 2; Oxford hip score, 41 points; HHS, right: 36.0 points, left: 46.0 points). The postoperative course went well, and the patient became ambulatory with a single cane and was discharged after rehabilitation therapy. At the Department of Dermatology, 5 mg/day corticosteroid was administered as maintenance dose for AA. The patient was carefully followed up as an outpatient if the symptoms on the left side worsened. |
In December 2006, a 31-year-old woman was referred to a neurologist because of consciousness disorder and fainting. Her main problems were obesity, snoring and waking up with a feeling of suffocation in the middle of sleep. The intraoral examination showed a large soft palate (). The soft palate was scored as class III according to the Mallampati classification (visualization of the soft palate and the base of the uvula) []. The electroencephalogram (EEG) showed focal dysrhythmia during hyperventilation with scattered sharp waves (). The patient was depressed and had sleep disorders such as sleep apnea and myoclonus, especially at the onset of sleep. She had experienced several occurrences of complete loss of consciousness during swimming and at work. The patient was on anticonvulsants and antidepressants (at first, she had been prescribed with Lamotrigine for 5 months, but later she was given 500mg Sodium valproate per day).\nOne of the best treatments for snoring during sleep is UUUP. The success rate of this type of surgery is reported to be between 16% and 83% [].\nWe chose a minimally invasive surgical procedure for the present case since the patient had a proper facial profile and a large soft palate (class III according to the Mallampati classification) [].\nIn May 2007, after analyzing the lateral cephalogram, we evaluated the craniofacial and pharyngeal airway morphology before the surgery. Under general anesthesia, 1cm of the soft palatal mucosa, from the right tonsil to the left tonsil, was removed. The patient’s tonsils were also removed during the surgery, and the anterior and posterior tonsillar pillars were sutured together ().\nThe symptoms were significantly decreased after the recovery. The patient no longer had sleep apnea, and antidepressants and antiepileptic drugs were discontinued. After the surgery, sharp waves were detected on the EEG at the level of the trachea (), but the patient was clinically asymptomatic. The 10-year follow-up showed no symptoms of sleep apnea or seizure. The patient did not lose any weight during the follow-up period. |
A 50-year-old man has been suffering from nasal obstruction and snoring for 2 months and was admitted to our hospital. His medical history revealed that he had total thyroidectomy and an operation for nasal septum deviation 30 years ago. He did not complain about nasal discharge, nasal itching, or sneezing. On physical examination, there was a serous secretion on nasal mucosa and nasal septum deviated to the right in the posterior portion. A small biopsy was taken and sent to the pathology. Brown-black coloured and soft specimen was measured 0.7 × 0.4 × 0.2 cm in size. In microscopic evaluation, several dilated blood vessels in a loose stroma were observed (). A few cells with foamy cytoplasm were seen and were interpreted as histiocytes and they were reported as vascular rich lesion suggesting hemangioma. A few months later, the patient was readmitted to the hospital with similar complaints and additionally with a swelling of left half of his face. On sinonasal MRA, a heterogeneous mass was seen in maxillary sinus, extending to the nasal cavity and infiltrating nasal conchae. The patient underwent an operation. The maxillary sinus was curetted, and several biopsies were taken from sinus mucosa. On histologic examination, largely dilated, thin-walled vessels were observed in a loose stroma similar to the first biopsy. However, between blood vessels, few histiocyte-like cells with bland nucleus and foamy/clear cytoplasm stood out without forming a distinctive pattern (). Although the patient does not have any complaint of flank pain or hematuria, metastatic renal cell carcinoma was suspected and urgent urologic consult was asked for. CD10, EMA, PanCK, and Vimentin immunohistochemistry were applied to determine the origin of the clear cells. These cells were stained positively with all these antibodies. (See Figures and ; for Vimentin and CD10 staining, resp.) In addition to that, on abdominal CT, a heterogeneous exophytic mass was observed. It was 12 × 12 × 10 cm in size, located in middle-to-upper portion of left kidney, and had cystic and necrotic areas. The sinonasal biopsy was diagnosed as metastatic renal cell carcinoma. Surgical and medical treatment of the patient was made in another hospital. One year later, the lesion recurred and the patient came up again in ear-nose-throat clinic with similar signs and symptoms. On physical examination, there was a polypoid mass extending to inferior nasal concha of left nasal cavity. Total maxillectomy was made and specimen was reported again as metastatic renal cell carcinoma. Any solid organ metastasis was not detected in this time. |
A 75-year-old female patient presented at the surgical consultation for perianal hitching and bleeding. The patient's complaints started 12 months prior, with progressive worsening. Her past medical history included hypothyroidism, glaucoma, and hemorrhoidectomy 30 years previously. Her family medical history included hepatic cirrhosis (father) and coronary artery disease (mother) but no cancer. Physical examination showed a 3 cm plaque-like eczematous lesion, circumferentially involving the perianal skin and extending to the anal verge (). A biopsy of the perianal mass demonstrated a well-differentiated mucinous adenocarcinoma. Serum carcinoembryonic antigen level and other laboratory tests were within normal range. A pelvic MRI showed that the lesion had invaded the skin tissue without involvement of perirectal structures and anal sphincters (). An endoanal ultrasound confirmed the absence of invasion into the anal canal and anal sphincters. A colonoscopy showed two polyps in the ascending colon that were both removed and histologically examined; they were both tubular-villous adenomas with low-grade dysplasia. A total-body CT scan did not show any associated visceral malignancy or lymphadenopathy. The patient was discussed within a multidisciplinary team including surgeons, oncologists, radiotherapists, radiologists, and pathologists. Since no anal canal involvement was identified and because of the patient's refusal of a permanent colostomy, a wide local excision was planned with a flap to close the perianal defect, and a protective colostomy. A biopsy of the inguinal sentinel node was also scheduled to rule out inguinal lymph node metastasis.\nThe day before the operation, a lymphoscintigraphy was performed which showed uptake in the right inguinal region. The patient underwent surgery the following day under general anesthesia. A sentinel inguinal lymph node was retrieved at the right groin region. A laparoscopic lateral colostomy was performed on the sigmoid colon. The patient was then placed in a jackknife position. The neoplasia was resected with 1 cm of free margins over the skin and the anal verge, including the dermis and the subcutaneous tissue. Once the excision was completed, multiple biopsies inside the anal canal were taken to exclude invasion. Then, stiches were placed at the four cardinal points on the mucosa of the anal canal. A double V marking was drawn, and the flaps were dissected with vascular pedicle preservation, based on random inferior gluteal perforator vessels and peripheral skin undermining (). The flaps were extended medially until complete coverage of the defect was attained and were then sutured tension free to the mucosa of the anal canal. The subcutaneous tissue was approximated, and the dermis was closed with separate sutures of reabsorbable material.\nThe pathological report was mucinous adenocarcinoma of the perianal skin involving the subcutaneous tissue with pagetoid spread with negative resection margins (). The lesion presented the following immunohistochemical pattern: CK7+, CK20+, CDX2+, CEA+, and GCFDP15-, suggestive of secondary Paget's disease (Figures –). No metastasis on the inguinal sentinel node was demonstrated. The postoperative course was uneventful, and the patient was instructed to avoid recumbent or sitting position for the first 4 days and was discharged on postoperative day 12.\nAfter 3 months (), random biopsies were taken in the flaps, in the skin proximal to the flaps, and in the anal canal, and the histopathological examination showed no sign of disease. The colostomy was then closed 2 months later. She was followed up at 3-month intervals; at the last follow-up, 14 months after surgery, the patient was fully continent and did not show any sign of recurrence. |
A 53-year old female was admitted to the Intensive Care Unit of Kingston General Hospital in Kingston, Ontario, Canada with diabetic ketoacidosis (DKA). She had a past medical history of type 1 diabetes with recurrent DKA and end stage renal disease secondary to diabetic nephropathy on intermittent hemodialysis. Prior to admission, she was being dialyzed through a left arm arteriovenous fistula.\nShortly after admission, her left arm fistula thrombosed, and a right internal jugular (IJ) tunneled double-lumen catheter was inserted for dialysis along with a peripherally inserted central catheter (PICC) via right basilic vein for intravenous access. The patient had a previous failed right arm AV fistula so the left arm was avoided in hopes of future recovery of the left AV fistula.\nTwo weeks later during the course of her admission, she continued to have episodes of DKA, and inadvertently her PICC line was dislodged, requiring removal. The patient had poor peripheral intravenous (IV) access and multiple attempts to insert peripheral IVs under ultrasound guidance were unsuccessful. Ultrasound examination of the patient's left internal jugular showed significant narrowing. The patient declined an attempt femoral venous access because she had previous lower extremity venous grafting for her AV fistula and she was told to avoid cannulization of any of those vessels. A 7 French, 16cm triple-lumen CVC was placed into the right internal jugular vein with sonographic guidance without difficulty. The puncture site for the CVC insertion was significantly distal to the tunneled dialysis catheter. Post-procedure, all three lumens were able to draw blood and flush saline. A chest X-ray confirmed placement of the catheter adjacent to the tunneled dialysis catheter () close to the cavoatrial junction. The patient did not have any immediate complaints or discomfort. Approximately six hours later, the on-call resident was called by nursing staff to assess the patient for new neck discomfort and jaw pain which had begun two hours prior.\nAt the time of assessment, the patient's heart rate was 95 beats per minute and regular. Her blood pressure was 188/84, respiratory rate was 18 and oxygen saturation was 99% on room air. She was afebrile. On examination, the patient had distended neck veins, pronounced facial edema and plethora (). She also had bilateral upper-extremity edema primarily manifesting in her digits. The rest of her cardiorespiratory examination was unremarkable.\nAll three lumens of the CVC were withdrawing blood and flushing without difficulty.\nPoint of care ultrasound of the right internal jugular vein revealed no thrombus at the site of CVC entry or distal to it. The SVC was not visualized directly. The CVC was removed promptly. There was no visible clot on the catheter, and no clot was pulled along with the catheter. Within an hour, the patient's facial swelling and plethora began to resolve, and her neck pain began to improve. The next morning, pain and swelling had resolved completely ().\nA retrospective chart review revealed a fistulogram performed months prior that showed significant stenosis at the left subclavian vein and mild narrowing at the junction where the two brachiocephalic veins merge to form the SVC (). At that time, angiography was attempted to alleviate the stenosis. However, the patient did not tolerate this, and the procedure did not lead to a significant change in the degree of stenosis.\nThe events surrounding the SVC syndrome and the previously identified SVC stenosis were disclosed to the patient. In order to prevent a similar event from occurring in the future, a note was made in the chart that would be more visible should the need for further CVCs arise. |
A 72-year-old female patient was referred to our unit having undergone complicated laparoscopic mesh repair of a large hiatal defect. A polyester composite mesh was fixed to the diaphragm using polyester sutures. Postoperatively, the patient developed an oesophageal leak with mediastinitis and pleural empyema. This required referral to another institution for oesophageal stenting, pleural lavage and decortication.\nAt follow-up, the patient complained of significant dysphagia and progressive post-prandial vomiting. A gastroscopy, performed 9 months postoperatively, revealed a distal oesophageal stricture with significant inflammatory change, associated with intraluminal foreign material suggestive of mesh. A computed tomography was performed and confirmed no recurrence of hiatus hernia but showed some mild thickening of the gastro-oesophageal junction.\nTen months following the patient's original surgery, she was referred to our institution for ongoing care. We were able to ameliorate the patient's symptoms temporarily with bougie dilatation and partial endoscopic mesh retrieval; but, following symptom relapse, the decision was made to attempt removal via intragastric laparoscopy.\nAt operation, a laparoscopy was performed, and a 12-mm camera port and two 5-mm balloon ports were introduced transperitoneally into the gastric lumen []. An endoscopy was performed, and an endoscopic snare was used to grasp the mesh. It was then delivered from the oesophageal lumen into the gastric lumen, where laparoscopic forceps could grasp it []. The mesh was trimmed using laparoscopic scissors, back to the oesophageal mucosa. The mesh was retrieved endoscopically.\nPost-operative barium swallow was normal and the patient was resumed on oral intake. Symptomatic relief was immediate, and the patient was tolerating all foods including bread and meat. The post-operative course was complicated by minor port site wound infection.\nAt most recent follow-up, 3 years postoperatively, imaging has revealed a recurrent hiatal defect. The patient does experience some mild epigastric discomfort but is able to tolerate small meals, and no further intervention has been required to date. |
A 64-year-old female patient presented to the emergency department after a train derailment. Upon arrival, she was nonresponsive, and Advance Trauma Life Support protocol was initiated. She was intubated, and on further workup, she was found to have a right upper extremity- (RUE-) comminuted displaced fracture of proximal radius and ulna Gustilo grade IIIc open fracture with large degloving injury and gross contamination. illustrates the large bony and subcutaneous tissue defect, and shows the X-ray of bony loss after application of an external fixator device while is the X-ray obtained in the trauma bay on initial arrival.\nThe patient was taken urgently to the operating room (OR) for right upper extremity washout, elbow-spanning external fixation, proximal ligation of the radial artery with angiogram secondary to substantial damage to the artery from the initial injury complex, right forearm fasciotomy, decompression of the carpal tunnel, and placement of wound VAC. Intraoperative angiogram demonstrated patent ulnar artery flow with an intact arch and backflow to the radial artery in the wrist. The soft tissue injury measured 15 cm × 10 cm, as seen in , which shows the original debridement soft tissue and bony defect. demonstrates the bony defect with antibiotic bead placement. On the first postoperative day, the patient was alert enough to undergo a neurovascular exam, where a posterior interosseous nerve (PIN) injury was noted.\nThe patient was taken back to the OR for multiple additional washouts and antibiotic bead exchange. On postoperative day (POD) 8, the patient had placement of ulnar intramedullary nail secondary to the only remaining skin bridge which was along the ulnar shaft and decision was taken to preserve the remaining skin bridge. Wound cultures from the initial surgery grew Citrobacter fruendii, Serratia marcescens, and Enterococcus faecalis, for which antibiotic regimen was adjusted based on susceptibilities, and the patient was treated for 6 weeks with amoxicillin and ciprofloxacin.\nThe patient's resultant defect consisted of a 5.5 cm radial epiphysis loss with an intact radial head and neck at the level of the elbow, a 20 × 15 cm overlying soft tissue defect, nearly circumferential, of the forearm involving her antecubital fossa. A repeat angiogram demonstrated flow through the ulnar artery with profuse flow to the midupper palmar arch and retrograde flow to the distal radius via the superficial palmar arch. She also had occlusion of the interosseous artery from its origin into the mid forearm with reconstitution in the distal forearm via collaterals.\nIn a multidisciplinary approach, she underwent an open reduction internal fixation (ORIF) of radius with a vascularized fibular osteocutaneous free flap to replace the proximal radius and overlying soft tissue. Radial head arthroplasty was performed at the proximal end of the fibular graft because the short segment of the native proximal radius remains.\nThe osteocutaneous fibular harvest was dissected in a standard fashion from the left lower extremity with a planned overlying skin paddle under sterile tourniquet control. The proximal osteotomy was completed so that the fibular would be reamed to fit the radial arthroplasty system while the flap was still vascularized. The fibular length after distal osteotomy was 6 cm. The radial head arthroplasty was done after dissecting the peroneal vessels proximal to bifurcation. The construct was placed in the joint and plated to the radial diaphysis. The capsule was closed after the arthroplasty. Fluoroscopy was used to verify the placement and range of motion (ROM).\nThe saphenous vein was harvested for the microvascular anastomosis secondary to lack of pedicle length to obtain anastomosis without undue stretch. The interposition graft was sewn end to side with the brachial artery above its bifurcation. The brachial artery was ischemic for a total of 30 minutes after which the flow was restored and confirmed by Doppler flow cytometry of the hand and through anastomosis. Couplers were used to anastomose the vein graft to the venae comitantes as well as the vein graft to the flap. The peroneal artery was sewn end to end with the saphenous graft. Both the skin and bone flaps showed appropriate blood flow. Two implantable Doppler probes were utilized distal to the arterial anastomosis beyond the bifurcation point to the skin paddle and bone. The skin flap was inserted to cover the bone and hardware. The remainder of the soft tissue defect was covered with a split thickness skin graft. The lower left leg was closed primarily, and the external fixator was reapplied.\nThe patient was transferred to the inpatient rehabilitation center within our institution on POD 7 from the osteocutaneous free flap transfer. On POD 30, the external fixator was removed. The patient had follow-up with both plastic surgery and orthopedic surgery. She had volar skin defect that closed secondarily of her right upper extremity. On POD 33, she was seen in clinic, observed passive ROM with limited restrictions on extension, flexion, pronation, and supination. With repeat X-ray of her extremity, she was shown to have appropriate interval consolidation with no loosening of hardware and good radial head placement. On POD75, the patient had difficulty extending her thumb and her fingers and the metacarpophalangeal joints secondary to the initial PIN injury. The range of motion around the elbow was 50 degrees for pronation/supination and 25–130 for flexion/extension. Her X-rays on POD 75 showed appropriate interval consolidation without loosening of hardware, good positioning of radial head, and appropriate interval healing of the ulnar fracture around the IMN nail, as seen in . The Mayo Elbow Performance Index was 20 before the surgery, while it was 85 on POD 75. The patient had been stabilized to transfer back to her native country, so follow-up exams were limited. We had correspondence with her orthopedic surgeon abroad to convert the IMN of the ulna to a compression plate with bone grafting to encourage healing at the ulna nonunion site. |
A 62-year-old healthy male with severe back pain was diagnosed with an acute aortic dissection (Stanford Type A). Emergency surgery was performed with an 8.0-mm ID endotracheal tube. Neither significant findings of the trachea nor any complications related to the intubation were described in the anesthetic records. Total arch replacement via a median sternotomy in the supine posture was performed with no complications. Postoperative intubation was required for ventilation support until the 15th postoperative day (POD). The patient was discharged from the hospital on POD 30. Beginning on POD 45, he started to experience throat discomfort without dyspnea. Although an expectorant (carbocysteine, 1500 mg/day) was prescribed by his primary doctor, symptomatic improvement was not identified. On POD 50, he developed dyspnea, and the symptoms exacerbated quickly. He was brought to our hospital by ambulance.\nUpon arrival, he complained of severe dyspnea with inspiratory stridor in his throat. Computed tomography revealed a mass arising from the posterior wall of the trachea (Fig. ). Bronchoscopy revealed a white subglottic tumor occupying 80% of the tracheal diameter (Fig. ). We performed an emergency tracheostomy under topical anesthesia, and the symptoms improved dramatically. For 5 days after the tracheostomy, hydrocortisone was prescribed; however, no tendency toward reduction of the mass could be identified during follow-up bronchoscopy. To shorten the treatment period, we performed a resection of the tumor under bronchoscopy 6 days after the tracheostomy.\nThe operation was performed with general anesthesia via the tracheostomy tube. The tumor was cauterized with an argon beam coagulator (ERBE VIO-S/APC®) via the bronchoscope, which was inserted from a bite block in his mouth. During the abrasion process, we found that the tumor had a pedicle which could not have been identified by preoperative computed tomography or bronchoscopy. The tumor was resected easily with abrasion of the pedicle. The tracheostomy tube prevented the resected tumor from falling into the distal airway (Fig. ). Little bleeding was experienced. The total length of the operation was 20 min.\nThree days after the resection of the tumor, the tracheostomy tube was removed. To prevent recurrence, an inhaled steroid was prescribed. He was discharged from the hospital 16 days after the tracheostomy. The inhaled steroid was continued until 3 months after the operation. No recurrence of the tumor was identified by bronchoscopy at 12 months after the resection. The pathological findings of the tumor revealed a granuloma including many lymphocytes, without any malignant component. |
The patient is a 26-year-old female who is G3P1. She had a cesarean section followed by D and C for a miscarriage. She was an otherwise well woman with no other medical or surgical history. The most recent pregnancy, unfortunately, also resulted in a miscarriage, for which she underwent her second D and C. She represented with bleeding 11 days later and was diagnosed with retained products of conception and underwent another D and C, this time under ultrasound guidance by a senior level registrar. Both procedures were uncomplicated, and the histopathology demonstrated only products of conception.\nApproximately 3 months later, she presented to our unit with chronic intermittent pelvic pain. As part of a diagnostic workup, an ultrasound was performed, which demonstrated omentum embedded into the myometrium, suggestive of a previous uterine perforation [].\nShe underwent hysteroscopy and diagnostic laparoscopy to further investigate the US findings. On hysteroscopy, filmy intrauterine adhesions were seen, which were divided. Tissue that resembled omentum was also seen protruding into the uterine cavity at the level of the fundus through a two-centimeter full-thickness defect.\nOn laparoscopy, omentum could be seen adherent to the fundal defect and the anterior abdominal wall []. The rest of the pelvis was normal. Instrumentation and curette of the uterus were only done under laparoscopic guidance after the omentum was released. The amount of energy used during dissection was limited as much as possible to minimize thermal injury to the myometrium. The full-thickness defect in the fundus could be seen where the uterine manipulator was visible laparoscopically. The defect was repaired in one layer with 1-polydioxanone sutures [ (This video is also available at )]. This suture material was chosen because it is monofilament, absorbable and lasts longer, and given the long-standing fistulous tract. To prevent secondary intra-uterine adhesions, a Foley's catheter was inserted into the uterus, and she was commenced on a regimen of estrogen and progesterone postoperatively. Histopathology results confirmed omental tissue.\nA sonohysterogram was performed 11 weeks later, which demonstrated that the defect had healed, with no evidence of a persisting fistula. However, there were some thick intrauterine adhesions in the right cornua, which were distorting the cavity shape. As she is planning further pregnancies in the near future, we plan to take her back to the theater to divide these adhesions in due course. Should she conceive in future, she has been advised to undergo an elective cesarean section at term (37–38 weeks) given the fundal full-thickness nature of the uterine defect. |
A 4-h-old male newborn weighing 3,400 g was referred to our hospital with an imperforate anus. There was no orifice in the perineal region. A white median raphe cyst, 4 mm in diameter, was present on the ventral side of the penis. Auscultation of the heart and lungs was normal. The patient’s family history was unremarkable. After 24 h, the color of the middle raphe cyst turned dark green. The cyst was incised at bedside, and meconium passed from it. Insertion of a soft catheter showed a deep fistula running parallel to the urethra (Fig. ). Urination was normal, with no urine passed from the fistula. Urethrography and fistulography, performed 3 and 4 days after birth, respectively, showed a long fistula running parallel to the urethra from the rectal pouch to the penis (Fig. ). The distance between the rectal pouch and the anal dimple was 1 cm. During urethrography, a small amount of contrast retrograde had flowed into the rectal pouch (Fig. ), suggesting a communication between the fistula and the urethra. X-rays and ultrasound did not show any anomalies in the sacrum and spinal cord. No other anomalies could be identified.\nOne-stage limited posterior sagittal anorectoplasty (PSARP) was performed on the fourth day after birth. With the urethral catheter indwelling, the patient was placed in the prone position, and a sagittal incision was made. The posterior rectal wall was opened in the midline and extended distally, ending directly at the fistula, which was 2 mm in diameter. The dissection continued between the rectum and the urethra until the two structures were completely separated from each other. The distal end of the fistula was ligated to the remaining part of the corpus spongiosum penis (Fig. ). The muscles were repaired and anorectoplasty was performed. The urethral catheter was removed on the seventh postoperative day and there was no difficulty in urination. Patient recovery was uneventful and he was discharged on the eighth postoperative day. Follow-up for eight months has shown no evidence of secretion passing from the opening of penile fistula, and the patient has been doing well. Bowel control could not be evaluated owing to patient age. |
A 70-year-old woman was referred to our hospital because of right knee dislocation after TKA was performed on her right knee due to severe varus deformity and flexion contracture (figure ). Before TKA, the patient had a 45° flexion contracture and severe varus deformity, according to the patient's records. The patient's knee had dislocated when she first tried to stand and walk on the second day after surgery. The day after the dislocation, the patient was referred to our institute (figure ). We attempted closed reduction of the dislocated knee in the operating room, but this was unsuccessful. In the first operation, size 5 femoral and size 4 tibial components (Genesis II, Smith & Nephew) of a non-posterior-stabilized prosthesis with a size 11 liner were inserted. Paramedian arthrotomy was performed via the previous longitudinal incision, and the knee was exposed. Eversion and lateral dislocation of the patella were attempted; however, the surrounding soft tissues were very tight and further proximal dissection and patellofemoral ligament resection was required to enable patellar eversion and knee flexion to 90°. The liner was then removed. After placing the retractors in the appropriate sites, we observed that the lateral collateral ligament, arcuate ligament, and popliteus tendon were severely torn, with inadequate release of the medial structures, including the medial collateral ligament (MCL) and intact medial sleeve with pes anserine tendons. The tibia dislocated laterally, even in the absence of a laterally applied force. First, a periosteal elevator was used to strip the superficial MCL from the proximal tibia, up to the diaphyseal region. Subsequently, the insertion of the pes anserine tendon was released with a cautery. The posterior capsule was released from the top of the tibia and the back of the posteromedial femur with an electrocautery and the curved tip of the osteotome, respectively. A second incision was made, extending from the lateral femoral condyle to the head of the fibula. Since grade III acute injury of the posterolateral corner of the knee was present, we preferred direct repair of the injured structures []. Upon incision of the fascia between the iliotibial tract and the biceps femoris, midsubstance tears of the lateral collateral ligament, arcuate ligament, and popliteus tendon were revealed. These were treated by performing an end-to-end repair with Bunnell-type crossing sutures []. The popliteus tendon was also found to be avulsed from the femur, and transosseous sutures were used to attach the tendon to its proximal insertion site. Posterolateral capsular repair and plication were performed, and the tendon of the lateral gastrocnemius muscle was sutured to the adjacent capsule. Subsequently, a size 15 deep-dish liner was inserted in order to optimize soft tissue tension. Rotational alignments of the tibial and femoral components were normal so they were not revised; further, there was no malalignment of the limb after the reduction of the knee prosthesis.\nDuring knee flexion, the patella subluxated laterally; hence, lateral retinacular release was performed. Furthermore, 2 suction drains were applied for 4 days until hematoma drainage ceased, and the patient's knee was kept in a brace for 2 months. Culture of the synovial fluid that was collected during the second operation revealed methicillin-resistant Staphylococcus aureus (MRSA); therefore, parenteral teicoplanin (Targocid®) treatment was started. Antibiotic therapy was continued for 1 month, and the eradication of the infection was confirmed clinically and with the help of laboratory tests. During this period, the patient was enrolled in a physical therapy program that included. Only isometric quadriceps exercises with no flexion and walking without weight bearing for 1 month. After 1 month, range of movement (ROM) exercises was begun, and the patient started to walk with full weight bearing and was able to achieve full knee extension. After the end of 14 postoperative months, the patient had a knee flexion of 100° and no varus or valgus instability (Figure ). |
The patient is a 75-year-old woman with a past medical history of hypertension presented to the office with a 9 × 9 cm fungating left thigh mass that was reported to be slowly growing over the course of two years (). She became concerned when it began to drain purulent fluid. She reported a 20 Lb. weight loss over the last few months attributed to decreased appetite. The patient was seen by a general surgeon who performed a wide-margin resection of her tumor. The wound was closed primarily and the patient recovered well from surgery. Pathology returned as Clear Cell Hidradenocarcinoma exhibiting extensive necrosis, hyalinization, focal ossification, and frequent mitoses. All pathologic margins were negative. Immunohistochemical stains were positive for D2-40, p63, CK 5/6, and vimentin. CEA was positive in the ductal component. PAX-8 was negative ( and ).\nPET/CT scan was performed after final pathology returned to evaluate for distant metastatic disease. PET/CT demonstrated mildly avid (Max SUV 2.7) 1.1 cm left inguinal and 0.6 external iliac adenopathy. These findings were corroborated with MRI of the pelvis and sonography demonstrating characteristically benign appearing inguinal lymph node. Although this likely represented typical postoperative changes, ultrasound-guided FNA and core needle biopsies of a 2.2 cm inguinal lymph node (without abnormal morphology) were obtained to rule out lymph node metastasis. No other abnormal lymph nodes were identified on ultrasound. Pathology failed to demonstrate any malignant cells on cytology, and immunohistochemistry returned as benign with normal lymph node architecture.\nAfter discussion in multidisciplinary tumor board, the patient was referred to a radiation oncologist and treated utilizing intensity-modulated radiation therapy to a total dose of approximately 5000 cGy in 25 fractions to the lymph nodes and 6400 cGy in 32 fractions to the tumor bed. The patient remains under close surveillance with a follow-up scheduled PET/CT. There is no evidence of recurrence to this date. |
A 23-year-old female arranged for a telephone consultation with a primary care facility in the northeast of England with the primary complaint of vertigo. Her dizziness was intermittent, and she described it as a feeling of “the world spinning around her.” This developed gradually over the course of four days prior to the consultation and was severe enough to set her balance off. By the time of the appointment, she was experiencing these symptoms four to five times a day. Each episode lasted 20 minutes on average, with the longest one lasting about an hour. These symptoms were often triggered by a change of posture, particularly after standing from a sitting position. Associated with the dizziness was a feeling of nausea; however, she did not experience any vomiting as a result of this.\nIn addition to vertigo, she also complained of aural fullness with the right ear affected slightly more than the left. This was accompanied by bilateral hearing loss, described as “muffled hearing,” as well as the ringing of the ears (tinnitus).\nShe tested positive for COVID-19 by RT-PCR 14 days prior to the appointment. She was initially prompted to test for COVID-19 due to a dry cough and runny nose for two days before testing. During the course of this illness, she also developed anosmia, ageusia, and shortness of breath. As dictated by local guidelines, she remained in isolation for ten days without feeling the need to seek medical help for her illness and therefore did not receive any medication for her COVID-19 infection. The symptoms of cough and runny nose had resolved completely; however, she continued to experience residual shortness of breath, and loss of senses of smell and taste at the time of this consultation. The symptoms of vertigo developed nine days after testing positive for COVID-19, and 11 days after the onset of her first COVID-19-related symptoms. A repeat RT-PCR three days prior to the appointment was now negative for COVID-19 (ten days after the initial positive result).\nThere was no history of headache, weakness, slurred speech, visual disturbances, or facial drooping. Her past medical history was otherwise unremarkable, and she was not on any regular medication. She denied any use of recreational drugs.\nIn light of the above history, she was called in for a face-to-face appointment on the same day for further evaluation. She was hemodynamically stable with a heart rate of 86 beats per minute, a blood pressure of 129/71 mmHg, a respiratory rate of 16 breaths per minute, saturating at 98% on room air, and was apyrexial. She was able to walk comfortably without aid into the clinic and was in no obvious distress at the time. She was fully oriented in time, place, and person. There were no motor or sensory deficits found after a thorough neurological and cranial nerve examination.\nBoth ears were examined and were found to be negative for any signs of infection, obstruction, or trauma. Tuning fork tests were conducted; Rinne tests were positive in both ears, and the Weber test did not lateralize to any particular side, suggesting bilateral sensorineural hearing loss. A Dix-Hallpike maneuver was performed which was negative. A head impulse test positively elicited compensatory eye movements in the horizontal plane. There was no observed nystagmus, and ocular movements were otherwise intact bilaterally. No gait abnormalities were identified and tandem walking was normal. Romberg's sign was also negative.\nAfter a full assessment, a diagnosis of labyrinthitis secondary to the recent COVID-19 infection was made. She was treated symptomatically with prochlorperazine thrice a day for up to four weeks. She was given a leaflet for information about viral labyrinthitis, its self-limiting characteristic, and asked to therefore watch and wait for symptoms to resolve on their own within four weeks. She was warned about symptoms such as headache, visual disturbances, weakness, or slurred speech. The advice was to contact the clinic immediately if developing any of the above red-flag symptoms, or earlier than four weeks if her condition continued to deteriorate after one week despite the therapy prescribed, or in case of concerns.\nShe eventually made a full recovery within three weeks of the encounter with no residual symptoms persisting from either labyrinthitis or COVID-19. |
A 20-year-old Saudi female was diagnosed with severe acne vulgaris at our dermatology clinic in December 2019. On her routine clinic visit in October 2020, the decision was made to start treatment with systemic isotretinoin (Roaccutane®) with a dose of 20 mg once daily for one month. A baseline platelet count was requested before starting the medication, the results of which are shown in Table .\nAfter one month (November 2020), the patient returned to the clinic for her routine visit. A new complete blood count was requested to identify any hematological abnormalities induced by isotretinoin. The laboratory results revealed thrombocytosis, with no other abnormalities detected in the complete blood count, as shown in Table . A reticulocyte count and a peripheral blood smear were not done.\nAccordingly, the medication was discontinued.\nThe patient also reported other non-hematological side effects. During treatment, she had recurrent episodes of seizures as diagnosed by a neurologist, who confirmed the diagnosis through an electroencephalogram (EEG); however, she did not start any anti-epileptic medication even though she had a positive family history of epilepsy.\nA thorough medical history was obtained from the patient after the occurrence of these side effects. She denied any history of malignancy, chronic diseases, or any drug ingestion, other than isotretinoin, that may lead to thrombocytosis. She was not feverish during the physical examination, and we did not observe leukocytosis or any signs of infection. Also, she did not have any known drug allergies. The dermatological examination demonstrated mild nodulocystic acne in her forehead and both cheeks.\nOne month after the discontinuation of isotretinoin (December 2020), the patient came for her routine clinic visit. A new complete blood count was requested. The results showed that the platelet count had returned to the normal range, as shown in Table . However, the seizure episodes continued to occur even after one month of isotretinoin discontinuation. The patient was referred to a neurologist to initiate anti-epileptic therapy. |
Our patient was a 47-year-old Caucasian man with a remote history of head injury and long-term PEG feeding in a nursing home who had developed increasing agitation and confusion. The patient had a closed head injury from a motor vehicle accident sustained 23 years previously. Since then he has had chronic but stable cognitive impairment and resides in an extended care facility. At baseline, he was able to talk and make simple decisions, but not complex ones such as those regarding his care. He is totally unable to perform his activities of daily living (ADLs) and is transferred using a ceiling lift and a two-person assist. He required tracheostomy for breathing and was fed via a PEG tube with Isosource® HN formula (Nestlé Nutrition™, Highland Park, MI, USA) without carnitine (Table ). He had been stable on tube feeds for many years. His calculated body mass index (BMI) was 24kg/m2, and his caloric intake via enteral feeds was 1800kcal/day. His other medical problems included a chronic foot ulcer without active infection and diabetes mellitus. He did not have any history of rhabdomyolysis, cardiac disease, liver disease, or renal insufficiency that would have suggested an underlying genetic defect in fatty acid oxidation. An unexplained acute change in his cognitive and/or psychological function and behavior occurred when he became increasingly confused and drowsy. As the patient had been on chronic phenytoin therapy for years, his phenytoin level was checked and found to be above the therapeutic range at 143μmol/L (therapeutic range 40 to 80μmol/L). His phenytoin dose was adjusted such that, days later, the level was 63μmol/L. Despite the achievement of therapeutic phenytoin concentration, his cognitive changes did not return to baseline. He continued to be delirious with a reversed sleep cycle. The phenytoin was further adjusted such that the level was subtherapeutic at 14μmol/L without improvement in his cognitive status. He did not return to his baseline level of function, being oriented only to person and unable to answer questions.\nWe evaluated him for other causes of confusion, which showed that his hematological profile, electrolytes, and renal function were normal. The glucometer reading was 6.6mmol/L at the time when he was first assessed for his confusion (hemoglobin A1C unknown). His liver biochemistry and International Normalized Ratio remained normal, and the serum total bilirubin was 8μmol/L (normal <18μmol/L ). The serum albumin level was low (29g/L) without any manifestation of hypoalbuminemia. Evidence of myopathy was difficult to assess clinically, given the neurologic compromise due to his remote accident. Creatine kinase and myoglobinuria were not measured at the time. His serum ammonia level, however, was high on multiple measurements taken under ideal conditions, ranging from 75 to 100μmol/L (normal <35μmol/L). The patient was not on any medication associated with hyperammonemia. His enteral feed, Isosource® HN, did not contain supplemental carnitine, and his serum carnitine and plasma amino acid concentrations were measured. His total carnitine was low at 20.5μmol/L (normal 30 to 63μmol/L), and his free carnitine was 17.2μmol/L (normal 22 to 59μmol/L). Acyl carnitine levels in urine were measured before and after carnitine supplementation, and they did not show findings suggestive of an underlying genetic defect of fatty acid oxidation. Plasma concentrations of ornithine (71μM), citrulline (34μM), and arginine (68μM) were normal, so there was no evidence of an undiagnosed urea cycle defect or other aminoacidopathy. Supplementation with carnitine 500mg twice daily via his PEG tube was initiated. His diet was otherwise the same without protein being removed from the diet. The patient’s confusion subsequently improved, and he was restored to his baseline neurological state, free of confusion, agitation, or behavioral disturbances within 1 month of the carnitine supplementation. Repeat serum ammonia concentration after carnitine supplementation decreased promptly to 53 to 60μmol/L. |
A 41-year-old male with a past history of cerebellar hemangioblastoma complained of vision loss in the left eye for 6 years, with reduced vision acuity in the right eye for 3 months. His vision acuity in the left eye started to deteriorate 6 years ago without other discomforts. CT scans at that time showed a mass at cerebellum. He underwent a resection surgery but the left eye still went blind several months later. Postoperative pathological examination confirmed the diagnosis of hemangioblastoma. Two years ago, MRI revealed a newly occurring mass adjacent to the left optic nerve which was asymptomatic. Unfortunately, the follow-up visits showed that it had been progressing gradually without treatment.\nThree months ago, his right eye had a significant reduction in vision acuity from 20/20 to 20/25 and was referred to our hospital. The visual field test and fundoscopy revealed no abnormality in the right eye. MRI revealed a mass occupying from the left orbit apex to the anterior cranial fossa, with hypointensity on T1-weighted images and hyperintensity on T2-weighted images (). Apparent and homogenous enhancement of the mass was observed on the gadolinium-enhanced MRI scans (), while the surrounding area showed no enhancement. The mass showed evident hyperintensity compared to brain parenchyma on ADC maps () but not on DWI (). The surrounding area along the left optic nerve was hyperintense on DWI (), and was considered severe peritumoral edema. Also, bilateral optic tracts were thickened on MRI due to swelling. MRA revealed no abnormity (Supplementary Material). Taken together, these imaging results highly suggested glioma. Hemangioblastoma was also suspected considering his past history. The latest MRI showed another mass in the left cerebellum, which was hyperintense on T2-weighted imaging (T2WI) and hypointense on T1-weighted imaging (T1WI) and DWI, with significant enhancement on contrast scans. These MRI findings and the location of posterior fossa strongly supported the possibility of hemangioblastoma. Combining these imaging clues and his past history, the recurrence of hemangioblastoma at both cerebellum and optic nerve was highly suspected.\nThe patient then received a left frontotemporal-approach craniotomy to resect the mass. At surgery, a reddish tumor adherent to the left optic nerve was observed. The attached tortuous vessels originated from ophthalmic artery indicated its rich blood supply. The tumor was excised and measured 1.5 × 0.8 × 0.8 cm. Postoperative MRI (\n) showed a complete excision of the mass. The edema has receded postsurgery but not completely. Changes in vision or visual fields have not been observed during the 2-week hospital admission after surgery.\nMacroscopically, the mass was solid, soft, and had a taupe surface. Histopathological results revealed that the tumor consisted of vacuolated stromal cells and numerous capillary vessels. The stromal cells are NSE(+) and GFAP(+), and the vessels are CD34(+), CD31(+), and F8-R(+). Interspersed CD68(+) monocytes and macrophages were also observed. CgA and Syn were both negative among the whole tissue (). Therefore, the diagnosis of hemangioblastoma was confirmed by the typical pathological characteristics. Considering the frequent recurrence of hemangioblastoma in this patient, VHL syndrome was probably associated with his clinical manifestations. Unfortunately, the patient refused genetic testing. |
A 65-year-old man presented with swelling of his right breast of 6 weeks duration. The swelling was insidious at onset but increased rapidly in size. There was no history of pain or fever associated with the swelling. He was known to have familial lipomatosis with multiple other swellings on his body which had been present for several years. Examination revealed a non-tender, firm 15 cm hemispherical lump occupying the whole of the right breast with peau d'orange appearance of the overlying skin and distortion of the nipple. The left breast was normal. There was no palpable axillary lymphadenopathy. There were multiple non-tender, soft solid subcutaneous lumps (lipomas) in the trunk and there was also a large right sided hydrocele (Fig. ). Based on the clinical findings of the breast swelling, it was thought to be a malignant tumour.\nRoutine baseline investigations, including chest X-ray, were within normal limits. Ultrasound scan of the breast revealed that it was an encapsulated mass containing areas of decreased echo pattern suggestive of sarcomatous changes in a lipoma. Magnetic resonance imaging of the breast swelling detected mixed signals with some areas of enhancement similar to those seen in soft tissue sarcomas. Computerised tomography ruled out any pulmonary metastasis. Mammography showed an enlarged dense right breast with a well-circumscribed soft tissue mass occupying almost the whole of the breast leaving a rim of normal breast tissue. Image guided core biopsy was inconclusive and the histological appearances were compatible with a wide differential diagnosis including fibromatosis, fasciitis, myofibroblastoma, dermatofibrosarcoma protuberance, leiomyoma or peripheral nerve sheath tumour. The patient underwent a mastectomy of the right breast. The mastectomy specimen contained a greatly enlarged male breast of 22 × 16 × 15 cm3, weighing 2255 g. Slicing of the specimen revealed a spherical, soft, degenerated 15 cm mass occupying almost the entire specimen. The cut surface of the tumour varied in appearance, mostly brown and soft with a firm white area. The tumour was well circumscribed and could be 'shelled out' from the surrounding breast tissue.\nMicroscopic sections (Figure ) revealed a tumour arising from breast connective tissue with areas which varied in cellularity. The cellular areas consisted of closely packed small uniform spindle cells showing relatively little dysplasia or pleomorphism and separated by varying amounts of collagen. Elsewhere myxoid degeneration was present. The white area noted macroscopically was of low cellularity, consisting of hyalinised collagen, which widely separated the tumour cells. The cells in this area showed greater pleomorphism and atypia. Mitotic figures were infrequent with no atypical mitoses seen. Although there was degeneration, no true necrosis was seen.\nThe tumour was extremely well circumscribed and had no infiltration to the adjacent breast tissue. The resection margin was free of tumour. Immunocytochemistry revealed that it was strongly positive for connective tissue markers vimentin and CD 34 and smooth muscle cell marker desmin, and negative for smooth muscle actin, protein S-100 (a marker for tumours of neural and fat origin) and epithelial marker MNF 116. Based on the histopathology and immunocytochemistry analysis, it was diagnosed as a myofibroblastoma of the breast. The stains for oestrogen and androgen receptors were not carried out. Following mastectomy, the patient made an uneventful recovery and, 5 years later, he remains well with no evidence of recurrence. |
A 17-year-old girl was referred to the Department of Periodontics with the chief complaint of gingival overgrowth and mobility in her teeth for the last 2 years. She reported that the problem has begun in lower anterior and progressed involving both the arches. Patient appeared fit and indicated no current or previous systemic disease during her medical history interview.\nIntraorally, severe diffuse gingival enlargement of both the arcades (more pronounced on the right side) was observed covering almost all the surfaces of teeth and projecting into the vestibule [Figure –]. Gingiva was bright red, friable, fibrous as well as edematous in consistency. The erythema was disproportionate to the amount of plaque and calculus remaining on the dentition panoramic radiograph demonstrated generalized alveolar bone loss []. Probing depth ranged from 10 mm to 12 mm with an attachment loss of 7-9 mm and was more in the right molar region. Grade 2 mobility was present around the mandibular and maxillary first and second molars and grade 1 around the mandibular right premolars.\nLaboratory tests revealed no evidence of any systemic disease such as leukemia, scurvy and hormonal disorders.\nInternal bevel gingivectomy was performed in the maxillary arch and left mandibular region[]. Adjunctive amoxicillin (500 mg, 3 × 1) plus metronidazole (400 mg, 3 × 1) for 7 days was prescribed to treat AgP. Patient could not continue her treatment for personal reasons and when she reported after 1 year, the recurrence of gingival enlargement was noticed [Figure –]. Periodic panoramic radiographic examination revealed progressive alveolar bone loss, which was more pronounced on the right lower molars []. Biopsy showed hyperplastic stratified squamous epithelium along with ulceration of epithelium at places. Sub-epithelial tissue revealed a large number of normal plasma cells, few eosinophils and inflammatory granulation tissue [Figure and ]. A diagnosis consistent with PCG was made. To clarify whether this enlargement was due to a hypersensitivity reaction, the screening for the various antigenic substances was done. The patient was questioned about the habitual use of chewing gum, mouthwash and toothpaste. The only relevant history given by the patient was the use of herbal toothpaste (Babool, Dabur Oral Care Products, India) for the last 3-4 years. The patient was advised to discontinue the use of herbal toothpaste, which dramatically reduced the severity of enlargement within 2 weeks [Figure –]. Moreover, the patient herself switched on to other herbal toothpaste (Meswak, Dabur Oral Care Products, India) after discontinuing Babool toothpaste. Thus, it was highly probable that babul extract from the Babul tree “Acacia Arabia” was responsible for the gingival enlargement in the present case as all other constituents of both the herbal toothpastes are similar.\nBlood test (enzyme-linked immunosorbent assay) for allergy of toothpaste components disclosed very high level of allergen specific antibody and symptoms relation for Acacia (105 kUA/L; normal value < 0.35 kUA/L), which confirmed “Acacia” as causative allergen.\nRight mandibular second molar with hopeless prognosis was extracted. Although pocket depth reduced significantly from 10-14 mm to 6-8 mm, but clinical attachment level was not reduced significantly. Surgical procedures were carried out on right molar region to treat AgP and residual enlargement. Patient was advised to continue with regular toothpaste.\nPatient was recalled at 1, 3, 6 months and after that she was recalled at 6 months interval. No recurrence of gingival enlargement was noticed in both arches and probing depth ranged from 3 mm to 5 mm []. Panoramic radiograph showed no further progression of bone loss []. |
A 63-year-old male with a past medical history of non-ischemic cardiomyopathy (ejection fraction 10%-15%), hypertension, chronic kidney disease stage G3A, and severe mitral valve regurgitation status post valve repair initially presented for an upgradation of his biventricular pacemaker to biventricular implantable cardioverter defibrillator (BIV-ICD) and replacement of non-functional right atrial lead. The pacemaker pocket was exposed and identified and the atrial and ventricular leads were pulled back under fluoroscopic guidance. A new left coronary sinus lead was placed. Next, right atrial (RA) and right ventricular (RV) leads were placed and access was obtained via the left coronary sinus as the left subclavian vein was found to be occluded with formation of collaterals. The leads were sutured in fascial planes via silk suture. The pacing and sensing parameters were confirmed. BIV-ICD was placed in the pocket, and the pouch was closed via vicryl sutures. The patient had electrocardiogram (EKG) (Figure ) and chest X-ray (Figure ) performed after the procedure. He had developed small pneumothorax from the procedure, which resolved on its own.\nThe patient was asymptomatic for the next seven months and then he developed increasing shortness of breath. At that point, the EKG (Figure ) revealed loss of appropriate pacing function. It was discovered that the patient kept massaging his pacemaker pocket site in the last seven months but he denied manipulating the device itself. He had also been moving his arm up and down frequently, which would have led all his device leads to be pulled up towards the device in a ratchet-like fashion. The patient was scheduled again for revision of BIV-ICD and on incision of the generator pocket, the left ventricular lead was found coiled up in the pocket beside the generator (Figure ). A new left ventricular lead was placed in the coronary sinus and then access was obtained via the right subclavian vein for right-sided leads. His right atrial and right ventricular leads were also found pulled up. A decision was made to place new right atrial and right ventricular leads. It is to be noted that all his device leads were undamaged and his device was found to be in the pocket in normal orientation. After securing the leads via sutures, the pacing and sensing function was analyzed. The pocket was revised and closed with vicryl sutures and the device was sewn to the pectoral fascia. A chest X-ray (Figure ) and EKG (Figure ) were obtained after the procedure to ensure the absence of pneumothorax and to document the appropriate functioning of the device, respectively. |
A 47-year-old Caucasian man presented to our center for oncological evaluation with an intra-abdominal mass, 23 months after right eye enucleation for choroidal melanoma. Physical examination was unremarkable. Full-body skin examination did not reveal any lesions of concern. Laboratory values, including liver function tests and tumour markers, were within normal limits. Computerized tomographic (CT) scan demonstrated a 5 cm abdominal mass located at the liver hilum, and displacing the body of the pancreas (Figures and ). Positron emission tomography (PET-CT) showed marked 18-FDG avidity in the area of concern (). A core biopsy was obtained under sonographic guidance. Histopathological examination proved it to be metastatic melanoma (Figures and ). The patient received dimethyl triazeno imidazole carboxamide (DTIC) chemotherapy for stage IV disease [–]. Because of the presence of a single metastatic focus, relatively long disease free interval, and threat of mechanical interference with portal structures, should the disease progress; we favoured surgical clearance of the disease in the hope of achieving durable palliation and potentially prolonging disease-free survival (DFS). Moreover, harvesting melanoma cells would allow preparation of autologous cell vaccine to be combined with interleukin-2 therapy, to further increase the chances of successful treatment []. At surgery, there was a conglomerate of enlarged hard lymph nodes encasing portal structures of the hepatoduodenal ligament. The liver was normal in size and appearance. There was no gross evidence of additional metastatic disease. All grossly positive lymph nodes were surgically removed, without need of vascular inflow reconstruction or bile duct excision. Histopathologic examination of the resected specimen showed lymph nodes replaced by metastatic melanoma (Figures , , and ). The patient tolerated the procedure well, but subsequently developed severe necrotizing pancreatitis with pancreatic leak and bleeding from a hepatic artery pseudoaneurysm. He required reoperation for necrotising pancreatitis and hepatic artery angiographic stenting. He ultimately convalesced and was discharged in stable condition. He is now six months after surgery and will start adjuvant chemotherapy combined with autologous melanoma-cell vaccine. |
A 66-year-old woman presented at a hospital with chest pain. Her history included diabetes, hypertension, and hyperlipidemia. On examination, the patient had a pulse of 100 beats/minute and blood pressure of 150/80 mmHg. Her electrocardiogram, echocardiogram, and blood test results were normal. Multislice computed tomography (CT) showed a saccular LMCA aneurysm and significant stenosis in the LAD artery (Fig. ). Coronary angiography revealed a saccular LMCA aneurysm measuring 9.8 × 7.5 mm with 75% stenosis in the proximal portion of the LAD artery. The operation was performed under general anesthesia. A median sternotomy was performed, and after a longitudinal pericardial opening was made, the heart was inspected. The LITA was removed from the inner chest wall in a skeletonized fashion using electric cautery. A distal segment of 1.5–2 cm was procured and reserved for use as a patch repair. Before aortic cannulation, the ascending aorta was dissected from the pulmonary artery. Under cardiopulmonary bypass, coronary artery bypasses of the left internal thoracic artery to the LAD artery were constructed in the beating heart. After aortic cross-clamping, the LMCA saccular aneurysm was exposed without main pulmonary artery transection. The saccular LMCA aneurysm was carefully dissected and completely excised. There was no thrombus in the lumen. Then, the LITA was longitudinally divided and trimmed to fit the incised LMCA. The small internal thoracic artery patch was sutured to the normal and firm lateral coronary arterial wall with a continuous 7–0 Polypropylene suture. Resection of the saccular aneurysm and closure using a small internal thoracic artery patch was then complete. The aortic cross-clamp time was 120 min, and the CPB time was 147 min. The patient had an uneventful hospitalization and was discharged on aspirin therapy. Follow-up multislice CT 10 days after the operation revealed the complete disappearance of the aneurysm and a successful repair with no luminal stenosis by the internal thoracic artery patch. The LITA graft was also found to be patent (Fig. ). The patient has been followed up yearly since 2009. Fortunately, at the 9-year follow-up, the patient was still asymptomatic, and there were no changes in the ECG and UCG. The patient included in the follow-up had preserved preoperative left ventricular function, and there was no coronary incompetence. Pathology of the aneurysm revealed that the aneurysm wall was very thin due to a lack of trilaminar arterial structure from the remarkable atherosclerotic changes (Fig. ). |
A 21-year-old male presented to us with bilateral hip pain right more than left for 2 years and difficulty to walk for the past 6 months. 2 years back, the patient experienced pain in both hips insidious in onset gradually progressive aggravated while walking relived on rest. Then, the patient was walking with support, and for the past 6 months, the patient was not able to bear weight and pain was severe on the right side. The patient is a known case of polyostotic fibrous dysplasia was diagnosed around 10 years of age. The patient was initially able to walk with the help of walker, unable to do activities of daily living, unable to sit cross-legged, and unable to squat. There is no history of trauma or any constitutional symptoms. Clinical findings were consistent with coxa vara deformity of bilateral hips. There was no evidence of any endocrine disturbance or altered pigmentation or precocious puberty. On imaging, it was concluded that the patient suffered from polyostotic fibrous dysplasia with bilateral severe shepherd’s crook deformity of the proximal femur with measured neck-shaft angle of 15° on the right side and 55° on the left side (). Whole body bone scan showed multiple areas of increased tracer uptake (). Computerized tomography of the pelvis showed features consistent with fibrous dysplasia of the proximal femur with bilateral shepherd’s crook deformity, also bilateral iliac wing, sacral ala, L3,4,5, S1, and 2 are also involved (). All the blood and serum biochemical investigations of the patient such as the hemoglobin, total and differential white cell counts, erythrocyte sedimentation rate, C-reactive protein, calcium, phosphorus, and alkaline phosphatase levels, and all the hormonal studies were within normal limits. It was decided to do correction of the deformity to realign the head, neck, and shaft and to achieve valgus at the neck shaft region, in one stage, each side at a time. On the right side, lateral closing wedge osteotomy was done at the subtrochanteric level to achieve alignment of the shaft with the proximal femur and fixed with a 135° dynamic Richard’s screw with a six-holed long side plate. 3 months later on the left side, lateral closing wedge osteotomy was performed at the subtrochanteric level to align the distal shaft with the proximal femur. Fixation was done using a 135° dynamic Richard’s screw with a six-holed long side plate to span osteotomy site. Postoperatively, we achieved a neck-shaft angle of 140° on the right side and 135° on the left side ( and ). The patient was started on active and passive quadriceps strengthening exercises, and after 6 weeks of immobilization, toe touch weight-bearing was started followed gradually by partial and complete weight-bearing mobilization. Follow-up imaging showed union at both the osteotomy sites bilaterally. At present, at 12-month postoperatively (), the patient is walking full weight-bearing without support and there are no signs of recurrence of lesions of fibrous dysplasia or the deformity ( and ). |
A previously healthy 13-year-old girl was admitted to the hospital due to 5 weeks of worsening lower back pain. Her pain was exacerbated by any movement such as walking or bending over, and the pain had been waking her up during the night in the days leading up to admission. Her pain was initially attributed to muscle strain, as a result of which she was seen by a physical therapist. She had also been taking ibuprofen with minimal relief. There was no obvious trauma noted although the patient recalled a minor abrasion to her back which happened during soccer practice. She reported no fever, cough, night sweats, joint pain, skin rash, or weight loss. Her immunizations were up to date, and she had not recently travelled nor had any contact with cats. In the emergency room, the patient was afebrile and her vital signs were stable. Her physical examination was unremarkable with the exception of mild lower back tenderness. Her laboratories revealed normal white blood cell counts (WBCs) of 9.2 × 103/mm3 with 58% neutrophils. She had elevated inflammatory markers; C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were 1 mg/dL (normal is less than 0.3) and 52 mm/h (normal is less than 15), respectively. A plain radiograph of her back () showed bony irregularity at the inferior aspect of T11 and superior aspect of T12. A magnetic resonance imaging (MRI) of her spine was consistent with vertebral osteomyelitis and discitis of T11 and T12 (). A blood culture was obtained and she was started on intravenous (IV) vancomycin and ceftriaxone empirically. The antibiotic regimen was changed in 2 days to IV ceftaroline fosamil 400 mg q 8 h. The possibility of tuberculosis was ruled out by a negative interferon-gamma release assay. The orthopaedic surgeon was consulted and did not recommend surgical intervention. The patient’s back pain improved, her blood culture showed no bacterial growth, and she was discharged home after 3 days of hospital stay. She had a central venous catheter placed, and she completed a total of 2 weeks of IV antibiotics and 6 weeks of oral antibiotics, sulfamethoxazole/trimethoprim and cefdinir, to empirically cover for S. aureus and Streptococcus species, respectively. She followed up with the infectious disease clinic which monitored her condition closely and followed her CRP, which normalized in 3 weeks. The patient had a full recovery, and she returned to her normal daily activities. |
A 46-year-old woman (gravida 2, para 2) was referred to our hospital complaining of a lower abdominal mass and pain. Her medical history was unremarkable. She was initially diagnosed with a uterine leiomyoma by transcervical needle biopsy. CT revealed a large heterogeneous tumor occupying the pelvic cavity and an intravascular tumor within the dilated left internal iliac and ovarian veins (Figures and ). Her preoperative cervical cytology results were negative for intraepithelial lesions and malignancy. The endometrial cytology and needle biopsy results were also negative. Thus, the preoperative diagnosis was IVL, with extension of the tumor into the left internal iliac and ovarian veins.\nIntraoperatively, multiple myomas were found within the uterine corpus and cervix, and the tumor extended to the parametrium and paracolpium. Detachment of the tumor from the left ureter and vaginal wall was very difficult. Intravenous tumors in the left internal iliac and ovarian veins could be palpated. The left internal iliac vein forming the common iliac vein was transected at the bifurcation region. In addition, TAH and bilateral salpingo-oophorectomy (BSO) were performed, resulting in the complete surgical resection of the tumor (operative time, 11 hours; blood loss, 8462 g). The resected uterus and adnexa weighed 897 g (Figures and ). There was no residual tumor detected in the venous resection stump.\nThe nodule resected from the uterus and the internal iliac and ovarian veins consisted of a proliferation of spindle cells. There was no nuclear atypia and the mitotic index was low. In addition, vessel endothelium cells and a vascular smooth muscle layer covered the IVL (Figures and ). The tumor cells stained positive for Alcian blue (pH = 2.5) and the staining disappeared after hyaluronidase digestion. However, compared to that in Case 1, the intensity of the staining was weaker and less diffuse (Figures and ). Similar findings for hyaluronan expression were obtained using the sample retrieved from the preoperative needle biopsy.\nThe histopathological diagnosis of the uterine and intravascular tumors was IVL. There has been no evidence of IVL recurrence, with the most recent follow-up at 38 months postoperatively. |
A 58-year-old woman who had a history of mitral (14 years ago) and tricuspid (7 years ago) valve replacement operations was admitted to our clinic with exertional dyspnea, palpitation, abdominal tenderness, and right-sided inguinal pain. She had been followed up with irregular intervals. It had been more than a year since her last echocardiographic evaluation, and the mechanical valves were reported to be functioning normally at that time. In her medical history, there was an attempt for coronary angiography which was performed via right femoral arterial access ten months ago. Her coronary angiography was reported to be normal. Femoral cannulation was not performed at her cardiac operations.\nBeginning four months ago, she had complaints of exertional dyspnea, palpitation, right-sided abdominal tenderness, and pain at the right groin area. On physical examination, any cardiac murmur was not noticed. She had hepatomegaly and mild peripheral pitting edema. Physical examination of lower extremity revealed continuous bruit on the right groin with palpable thrill. Lower extremity peripheral pulses were weakened at the right side. Branham's sign was present.\nLaboratory evaluation did not reveal any abnormality but mildly elevated hepatic enzymes. Echocardiography was performed; mitral and tricuspid valves were reported to be functioning normally, and there was neither stenosis nor insufficiency. Left ventricular systolic and diastolic functions were normal. There was biatrial dilatation without any thrombus formation.\nWith the provisional diagnosis of peripheral AVF leading to a new onset right-sided heart failure, Doppler ultrasonography was performed and revealed right superficial femoral artery to common femoral vein fistulisation. Contrast enhanced lower extremity computerized tomography was performed afterwards, which revealed the connection between superficial and deep femoral arteries to common femoral vein and the early phase contrast enhancement in iliac veins and inferior venae cavae ().\nThe patient was operated under general anesthesia. Right vertical groin incision was performed. Superficial femoral artery (SFA) and deep femoral artery (DFA) were explored. A narrow-based AVF between DFA and common femoral vein (FV), 3 cm distal to DFA origin, was detected. A second connection with a broad-based neck was located between SFA and FV, at the fourth centimeter of SFA (). Both were occluded temporarily, and the thrill ceased. The narrow-based AVF between DFA and FV was ligated and divided at arterial and venous ends. For the second AVF, SFA at the broad-based junction was resected. The venous site was sutured from within the artery. After resection of a ten millimeter segment of artery, SFA was repaired with end to end anastomosis. An autologous arterial wall patch was secured on half distance of the anastomosis. After the procedure thrill was totally ceased, both SFA and DFA pulses were present. |
A 64-year-old woman was admitted to our hospital with a pulsatile mass swelling over the left cervical region and right hemiparesis after cough for 1 day. She had a history of fibromatosis, but no previous history of trauma, operation or inflammation in this region. On physical examination, a 5-cm large pulsatile swelling with blowing bruit was found over the left cervical region and multiple fibromas were found in regions of face, neck, and trunk. Neurological examination revealed muscular strength of grade IV according to the ‘manual muscle test (MMT)' grading system and hypoesthesia of right limbs, including touch and pain sensation. Computed tomography angiography (CTA) and DSA revealed a left high-flow internal carotid-jugular fistula at the first cervical level and twisted left internal carotid artery (ICA) (Figures ). Blood flow from the vertebrobasilar artery and right ICA via the circle of Willis supplied the left anterior cerebral artery and middle cerebral artery and drained backwards into the petrosal segment of left ICA (Figures ).\nWe chose embolization of both the fistula and parental artery, because no appropriate covered stent could be used to pack the fistula in the condition of reservation of left ICA. The purpose of first endovascular treatment was to isolate the fistula, by blocking both the backward blood flow from right ICA and the forward blood flow from left ICA. A Headway-21 stent catheter was selectively inserted into the distal part of fistula in the segment of carotid cavernous sinus, meanwhile an Echelon-10 microcatheter was placed in the distal part of stent catheter. One LVIS 5.5*30 mm stent, with its characteristic relatively compact mesh, was used to cover the distal part of fistula to avoid coils being pushed into internal jugular vein and heart (Figure ). However, the 3.5 cm fistula could not be covered by the LVIS stent. Therefore, 10 coils were additionally used one by one, to block the fistula from the distal to proximal part through an Echelon-10 microcatheter (Figure ). When considering the fast-backward blood flow from the right ICA after embolization, we could not guarantee the safety of blocking by Onyx. We chose one detachable balloon to block the ICA near the proximal part of fistula to stop the forward blood flow, and the backward blood flow would be stopped in the second treatment (Figures ).\nThe blowing bruit was reduced significantly after the first treatment, but became worse after 2 months later. The patient was admitted to our hospital again, and DSA revealed residual blood flow in the fistula from the left ICA and premature balloon deflation (Figures ). It was fortunate that LVIS stent blocked the balloon into the internal jugular vein. An additional 13 coils were used to pack the fistula with double-microcatheter techniques, under the multi-angle DSA projection, to avoid the coil protruding into the internal jugular vein (Figures ). Afterwards, two detachable balloons were used to block the proximal part of left ICA again (Figure ). DSA revealed the forward blood flow disappeared, the low-flow backward blood from right ICA still supplied the fistula, and a normal ipsilateral jugular vein (Figure ). Because the fistula was mostly blocked, and the low-flow backward blood may promote the formation of thrombosis in the distal part of fistula, we stopped the second endovascular treatment and planed a DSA examination to evaluate the effect of embolization and to determine further treatment. After this treatment, the patient did not feel the blowing bruit or any other discomfort.\nHowever, the patient complained of swelling and pain in the left occipitocervical region 2 days later. Physical examination revealed a large subcutaneous mass, with volatility and severe tenderness. Emergent CT revealed a subcutaneous hematoma in left occipitocervical region, without abnormalities in brain (Figures ). DSA in the hybrid operation room showed the coils in the fistula were stable without no forward blood flow in the proximal part of fistula. However, there was still a little backward blood flow through the right ICA and vertebrobasilar artery into right internal jugular vein, via the distal part of fistula. The lower part of fistula was not shown, because the blood drained into the right internal jugular vein through the sigmoid and transverse sinus reversely (Figures ). It was suggested that the occipitocervical subcutaneous hemorrhage was caused by poor ipsilateral jugular drainage, which was the result of the formation of thrombosis in fistula extending to the left internal jugular vein, thus blocking forward and backward reflow. We inserted an Echelon microcatheter through the left vertebrobasilar artery-posterior communicating artery, then selectively to petrosal segment of the left ICA, where 3 coils were put in, and then a 1.2 ml Onyx-18 was injected slowly. DSA examination after this procedure revealed that the backward blood flow distal to the fistula disappeared (Figures ). An open operation to remove the hematoma was preformed after the interventional operation. Multiple capillary hemorrhages were found from behind the sternocleidomastoid muscle after removing clots. The operation to stop bleeding was successful, and the postoperative course was uneventful. A 6-month follow-up DSA demonstrated a totally occluded fistula allowing the patient to engage in light manual labor with normal neurological functioning after the operation (Figures ). |
A 41-year-old man was hospitalized with severe scar contracture due to flame burn injury to neck and chest three years ago. After undergoing several surgeries, his contracture got worse. He developed severe scar contractures involving neck, face, anterior chest, and both shoulders including upper arms leading to severe deformity. His chin, chest, and both the upper arms were fused together by thick hard scars. Because of the contractures, he had to stay in a stooped posture with no neck flexion or extension. The cervicomental and the mentosternal angles were completely obliterated by thick stiff scars, and the anterior neck structures, including the larynx, the trachea, and the carotid arteries, were not identifiable or palpable ().\nWe consulted an otolaryngologist for airway evaluation, but endoscopic examination of the airway could not be performed because of the narrow external nares and the small oral opening. Preoperative X-rays and a computed tomography (CT) scan for assessing his airway passage and related structures revealed marked thickening of the soft tissues around the neck, without distortion or stenosis of the upper airway and the trachea.\nIn preoperative airway examination, the width from upper incisor to lower teeth was less than 1 cm. Because of the restricted mouth opening, we could not perform modified Mallampati test (mMT). It was difficult to perform face mask ventilation because of nearly fixed neck movement and regressed mandible. For the same reason, conventional laryngoscopic intubation was thought to be impossible. Since the diameter of nares was about 0.5cm, it would be impossible to intubate the patient by nasal approach. So we considered several adjunctive methods, such as fiberoptic bronchoscopy, laryngeal-mask airway, lightwand or a GlideScope® Video laryngoscope, which might be used concomitantly. But there was no available noninvasive approach except orotracheal fiberoptic intubation while the patient is awake, or surgical tracheostomy.\nThe day before the surgery, a peripheral intravenous catheter was placed. Glycopyrrolate 0.2 mg was delivered to the patient via intramuscular injection for reduction of oral secretions just before transferring to operating room. He was placed in the supine position on the operating table with standard monitor. The patient's vital signs were stable and he lay down comfortably on his back by stuffing the gap with pillow and folded sheets. The first attempt of awake fiberoptic intubation was performed after applying topical lidocaine spray to the oral cavity and pharynx. However, it was impossible to intubate with endotracheal tube through restricted mouth opening. Surgical tracheostomy wasn't possible either because thick scar made it impossible to identify the anatomy of the neck. So we decided to perform rapid surgery for release of the neck and mouth contractures under local anesthetic lidocaine (totally 400 mg) to improve his mouth opening by freeing the mandible from the scar. There were no systemic effects such as hypotension, bradycardia, arrhythmia, and seizure.\nInitial scar releasing operation of the mouth and neck was done with intermittent administration of fentanyl (totally 200 mcg). In spite of dissecting nearly the whole circumference of the neck, the mouth opening was only enough to allow passage of the blade of laryngoscope ().\nSince it was impossible to identify glossopharyngeal nerve or superior laryngeal nerve even after releasing the contractures, we could not perform nerve block. After applying topical lidocaine spray to the oral cavity and pharynx, orotracheal intubation with a cuffed endotracheal tube (7.0 mm ID) was performed with the use of fiberoptic bronchoscopy under GlideScope® Video laryngoscope guidance (). There was no difficulty during the intubation. We confirmed the tube passing through the vocal cord and then it was fixed at mouth angle.\nAfter the surgery, the patient remained with endotracheal tube and was transferred to surgical intensive care unit.\nHe received continuous infusion of midazolam and vecuronium for sedation and mechanical ventilation was maintained. Postoperative day 2, sedation was stopped and extubation was accomplished. After it, his spontaneous breathing was regular, chest movements were good. There was no hoarseness. Postoperative day 3, the patient was moved to the general ward. |
A 64 year-old man of weight 70 kg and height 1.65 m underwent a total hip arthroplasty (THA) in January 2003, due to severe osteoarthritis of his left hip following avascular osteonecrosis (figure ). The patient was active and performed strenuous manual labour that required him to ascend and descend stairs frequently. A SEM3 type (Science et Médecine, Montrouge, France) cementless forged Ti 6Al V4 alloy, with femoral stem size (12) coated with hydroxyapatite on the proximal third, with a metallic head of a diameter 28 mm, and an ultra-high molecular weight polyethylene (UHMWPE) insert (liner) with a metal acetabular cup (50 mm) was inserted. Four years after his original operation (in January 2007), the patient experienced severe pain in the left hip while walking. He was admitted to hospital some days after this episode, with pain in the left hip and inability to bear weight. There was no history of trauma. Radiographic examination revealed a neck fracture of the femoral component without bone loss in the proximal femur (figure ). For the extraction of the fractured femoral stem a specially designed extractor (patent pending) was invented. Six days after his admission, the patient underwent a revision THA. The elapsed time was needed for the construction of the extractor. At retrieval an extensive amount of bone was adherent to the device, and the fractured implant was well fixed. At revision the same stem design and the same size (12) of the femoral stem was inserted. The metal acetabular cup was not revised but only a new UHMWPE insert was applied because it exhibited areas of attrition due to friction of the fractured stem. The postoperative course was uneventful. The patient was mobilized on crutches for 6 weeks. At follow-up examination, seven months postoperatively the patient remains independently mobile and pain free with a good range of motion. The fractured stem and the neck carrying the head were sent off for stereomicroscope examination with magnifications up to ×40. Images were captured through digital camera (Nikon Coolpix 5400 5 M Pixels or Sony DCR TRV80E 2 M Pixels) and further investigation with scanning electron microscope was decided. The head-side of the neck was thus examined with scanning electron microscope (Quanta 200, FEI Company) and images were taken at magnifications from ×10 to ×110.\nThe examination of the stem and neck (figures and ) shows that this model has a neck machined for a better range of motion and impingement avoidance, with material removed mainly on the medial side, but machining extends also to the posterior and anterior aspects. Apparently, the fracture occurred at the smallest section of the neck, where the diameter is reduced to approximately 9.8 mm, corresponding to a surface area of 75 mm2. The laser etched markings lie on the anterior aspect of the stem, when used in a left hip. Additional laser etched markings are made on the neck, giving the indication relative to the characteristics of the cone (SEM 8. 00' and SpD2 = 12,45). These markings lie also on the anterior aspect, when the stem is implanted on a left hip. Scanning electron microscope figures of the fracture surface of the part supporting the head clearly show that the fracture has ended on the posterior side. Typical beach marks can be seen in the middle of the surface (arrows on figure ). This beach marks are characteristic of the beginning of intergranular fracture, after a first stage of fatigue crack propagation. The second stage of the fracture consists in an intergranular fracture, located in the region marked a on figure , and shown more clearly in figure . The very last stage consists in a shear lip (marked region b in figure and figure ), oriented to 45° relative to the plane of intergranular fracture, and characteristic of the very end of the fracture due to shear stresses. Except for the intergranular fracture, which is very clear, the other fracture surfaces are smeared and polished from abrasive contact in vivo as enhanced by biological lubrication (see fragment contact in figure ). Examination of the edges of the surface with a stereomicroscope reveals marks of tools, probably iatrogenically caused during retrieval of the implant. Unfortunately, these marks lie on the anterior aspect just in the vicinity of the laser markings, and interfere with possible other marks of fracture. Scanning electron microscope examination of the edge of the surface near the laser markings cannot show any clear evidence of initiation of fatigue fracture, mainly because of the tool marks and of the abraded surface in this region (figures and ). No typical striations could be found near the edge of the surface. The marks shown in figure (near the "O") are more probably due to attrition because there are several such marks in this region, with different orientations. |
A 68-year-old male presented with chest pain similar to acute coronary syndrome. The patient had felt a substernal pain from a period of four days prior to examination, which had begun while carrying out farm work in a squatting position. The severity of the chest pain had fluctuated, and then the patient felt intolerable chest pain that lasted for two hours. An electrocardiogram showed normal sinus rhythm, and cardiac enzymes were normal. However, compared with another chest X-ray four months earlier (), a second chest X-ray revealed left diaphragmatic hernia with a markedly distended stomach and passive atelectasis of the left lower lung (). An abdomen computed tomography showed that the herniated stomach was significantly distended by ingested air due to severe pyloric compression. The diaphragmatic defect was approximately 5 cm in diameter ().\nWe performed an emergency operation because the patient suffered from continuing chest pain, which suggested a strangulation of the stomach. A left lateral thoracotomy was performed through the eighth intercostal space after intubation with a double-lumen endotracheal tube and insertion of a nasogastric tube for decompressing the stomach. We carefully inspected the stomach, which was entirely displaced into the thoracic cavity, including the greater omentum (), and we then reduced the stomach into the peritoneal cavity. A diaphragmatic defect was identified on the highest central tendon and had a round shape, half of which had a well-demarcated margin (). The diaphragm was thinned and unevenly rough owing to senile change. The remaining tissue of the fragile margin was completely excised () and was closed primarily using continuous running sutures and multiple interrupted sutures (). The patient was extubated before leaving the operating room. The chest X-rays before discharge showed complete reduction of the stomach and full re-expansion of the left lung (). The patient was uneventfully discharged and resumed a normal diet 10 days after the operation. |
A 41-year-old Indian female first presented to the outpatient clinic complaining of a tender lump over her right parieto-occipital region. A computed tomography (CT) scan of her head was done, revealing a small, well defined lytic lesion of the skull extending from the inner table to the outer table. The initial impression was that the lump was consistent with sinus pericranii [].\nShe presented to an outside hospital 1 year later with left shoulder pain weakness and numbness of both lower extremities. On examination, power in both lower limbs was 4 out of 5 but there were no motor deficits in her upper limbs. There was mild sensory deficit to light touch and pain over her left cervical segment 8 (C8) and thoracic segment 1 (T1) dermatomes. There were no cerebellar signs or higher mental function deficits. No lymphadenopathy was clinically evident and biochemically there were no abnormalities. A contrast magnetic resonance imaging (MRI) scan of her cervical spine was done, which showed an extradural lesion extending from cervical segment 7 (C7) to thoracic segment 2 (T2) occupying the left posterolateral spinal canal. She underwent a C7 to T2 laminectomy, excision of lesion and instrumentation of the spine. The histological diagnosis was that of RDD.\nShe subsequently presented to our institute 2 months after surgery with numbness extending from her left mid torso to her left leg. A contrast MRI scan of the brain and spine was done. It showed enhancement in the first and second thoracic segments’ left lateral recesses and neural foramina likely representing postsurgical changes in the absence of residual tumor. It also revealed the previously noted osteolytic lesion involving the right posterior parietal bone, which now had extradural and extracalvarial enhancing soft tissue components []. However, she was asymptomatic from the cranial lesion, erthyrocyte sedimentation rate (ESR) was not elevated, there was no lymphocytosis, and gamma-globulin levels were normal. In addition, the lesion was isolated and small, thus conservative management was adopted and she was placed on outpatient observation with close follow-up.\nA follow-up MRI of her brain and spine was done 4 months later, which showed resolution of the enhancement seen at the region of the spinal lesion. The brain lesion showed marginal increase in size but as the patient continued to remain asymptomatic, we elected to continue observation with close follow-up and interval imaging.\nHer subsequent follow-up MRI brain at 6 months now showed a significant interval increase in the size of the right parieto-occipital extraaxial dural-based enhancing soft-tissue lesion. Dimensions were approximately 2 × 3.2 × 1.7 cm in maximum orthogonal dimensions. It also showed an interval increase in protrusion into the underlying right cerebrum with significant interval development of vasogenicedema in the right parieto-occipital lobe, extending to reach the right atrial subependyma. The lesion was transcalvarial with a subgaleal component, which also shows interval increase in size []. Biochemically, she had markedly raised lactate dehydrogenase levels (LDH) (1198, reference: 222-454 U/L) and lymphocytosis.\nThe patient was due for outpatient review a week after the MRI brain, but in view of the finding, she was quickly contacted to assess her clinical state. She had, however, presented to the emergency department with headaches, neck stiffness, increasing drowsiness, and nausea, and was thus admitted for treatment. She was treated with dexamethasone 4 mg three times a day and analgesics. Her symptoms subsequently improved. We decided to take her for surgery because of the increasing size of the lesion, vasogenic cerebral edema secondary to the lesion as evident on her scan, and evidence of raised intracranial pressure (ICP). She underwent a craniotomy and excision of the lesion. The tumor was found to be an intradiploic tumor eroding into surrounding soft tissue superficially and deep with dural involvement and extension into the brain. The lesion was excised en-bloc and the calvarial defect was closed with an acrylic cranioplasty. The patient made an uneventful recovery. On last follow-up, a month after surgery, a first time positron emission tomography-computed tomography (CT-PET) was done to assess for residual disease or recurrence and also screen for systemic disease. This showed successful removal of the intracranial lesion with no other systemic lesions.\nHistopathology showed the lesion to be involving both the inner and outer tables of the skull as well as the dura mater. There was nodular proliferation of foamy histiocytes, with admixture by lymphocytes, plasma cells and neutrophils, with numerous foci of sclerosedstroma. Immunohistochemistry staining of the histiocytes was positive for S-100 and CD68 and negative for CD1a. In the dura, the histiocytic proliferation showed intervening foci of sclerosedstroma []. These findings were highly diagnostic of RDD. |
72-year-old male patient presented with a history of the left buttock pain radiating to the left lower limb for the past 6 months, which aggravated over the past 2 days (visual analog scale 9/10). On examination, the patient had no nerve root tension signs, and bilateral straight leg raising test was negative. Extensor hallucis longus (EHL) and plantar flexion were Grade 3 power by Medical Research Council (MRC) grading on the left side. The patient also reported sensory deficit to both crude and fine touch over L5 and S1 dermatome in the left lower limb. There were no bowel or bladder symptoms. The patient was evaluated by MRI examination that revealed total six lumbar vertebrae and a prolapsed intervertebral disc between L5 and L6 vertebra with left side lateral recess and foraminal stenosis and a possible posterior epidural migration of the disc fragment [Figures and ]. Patient was advised urgent surgical decompression by L5 and L6 laminotomy and microdiscectomy in view of neurological deterioration. Routine surgical steps for microdiscectomy were followed after confirmation of the level. L5 laminotomy was performed. The disc space was explored after securing shoulder of exiting nerve root. To our surprise, the extruded disc fragment was not seen, and both the exiting and traversing nerve roots were absolutely free of any compression/tension with adequate mobility. We looked both at the level as well as above and below the L5–L6 disc level to find out the missing disc material. MRI was repeatedly checked and compared with X-ray images to ascertain the level of discectomy. Intraoperative C-arm images were taken to reconfirm the level of discectomy. After extensively searching for the disc as well as going through the radiology images repeatedly, it was contemplated that the disc material might have got sucked through the suction tip. The suction fluid was filtered through a surgical mop used as a sieve, and the material collected was sent for histopathological study []. The roots and epidural space anteriorly were checked again with sweeping movements using Watson Cheyne retractor and nerve hook retractor under direct vision. After repeated confirmation, the incision was closed in layers. Postoperatively, the patient had relief from radicular pain (postoperative VAS for back and leg pain 3/10), and the motor power was improved (Grade 4/5 MRC). The biopsy report confirmed the material filtered was intervertebral disc material []. |
A 71-year-old male with a history of left ventriculoperitoneal (VP) shunt for hydrocephalus from bacterial meningitis, Y grafting for abdominal aortic aneurysm, and bilateral above-knee amputation was referred to our hospital. Enhanced computed tomography (CT) revealed a saccular aneurysm measuring 68 mm in diameter in the middle of the descending aorta, which was a candidate for TEVAR (). The patient was severely obese, with an ascending aorta of 45 mm in diameter and a calcified external iliac artery of less than 5 mm (). His left VP shunt passed subcutaneously from the left thoracic side to the abdominal region. Therefore, surgery with left thoracotomy and laparotomy was extremely difficult.\nNot only conventional descending aortic replacement with left thoracotomy, but also TEVAR via iliofemoral artery, abdominal aorta, ascending aorta, and left ventricular apex was considered challenging. Among the neck vessels, the brachiocephalic artery and left subclavian artery were too steep to deliver the stent graft to the descending aorta. Conversely, the LCCA, which was leading to the descending aorta (), was considered as an alternative access site for TEVAR. However, there was a possibility that the patient would not be able to tolerate the long duration of LCCA clamp due to the hypoplasia of the right vertebral and right middle cerebral arteries. Furthermore, his LCCA was 6.0–8.0 mm in diameter and showed atheromatous change, and thus was not large enough to allow the insertion of a delivery sheath with an outer diameter of 7.7 mm; doing so could possibly result in LCCA damage and cerebral embolization of debris after the LCCA declamping. Conversely, LCCA stenosis was a potential long-term complication. Therefore, left Ax–CA bypass before LCCA access was planned for TEVAR, with the aim to shorten the LCCA clamp time, followed by LCCA ligation after the procedure.\nThe procedure was done under general anesthesia in an operating room equipped with a Siemens Arcadis Avantic mobile angiography system (Siemens Medical, Munich, Germany). The left axillary and left carotid arteries were exposed through a small left subclavicular and anterolateral neck incision. The LCCA stump pressure at the time of LCCA clamping was 57 mmHg, which was equal to the diastolic systemic blood pressure. Simple clamping of the LCCA was performed because an LCCA stump pressure above 50 mmHg is considered to be generally safe for procedures requiring common carotid artery clamping such as carotid endarterectomy. Bypass grafting from the left axillary artery to the distal portion of the LCCA was performed using a Gore-Tex Intering Vascular Graft (diameter, 7 mm; W. L. Gore and Associates, Flagstaff, AZ, USA). Proximal anastomosis was end-to-side, distal anastomosis was end-to-end, and the LCCA was ligated proximal to the anastomosis.\nA guidewire was advanced with a pig tail catheter from the proximal portion of the ligated LCCA to the descending aorta, captured with a snare catheter introduced from the left femoral artery, and pulled through the left femoral artery. The access route was stretched and linearized easily by tugging the guidewire carefully, indicating that stent-graft insertion could proceed safety. Despite the presence of a very small saccular aneurysm in the distal portion of the aortic arch, it protruded less than 7 mm into the lumen, with no tendency to expand; therefore, rupture of the aneurysm or dissection of the aortic arch was deemed unlikely. After the incision of the LCCA, a Zenith TX2 Pro-Form stent graft (outer diameter 7.7 mm; ZTEG-2P-30-140-PF; Cook Medical, Bloomington, IN, USA) was delivered smoothly to the descending aorta with the tug-of-wire technique and deployed to the distal side of the aneurysm (). Similarly, a second Zenith TX2 Pro-Form stent graft (outer diameter 7.7 mm; ZTEG-2P-32-140-PF; Cook Medical Bloomington, IN, USA) was delivered and deployed to the proximal side of the aneurysm. TAA was successfully excluded (). After removal of the delivery system, the LCCA was ligated proximal to the incision ().\nThe patient was discharged without any complications after a 10-day hospital stay. Postoperative CT scan confirmed the correct positioning of the stent graft, exclusion of the aneurysm with no endoleaks, and a patent left Ax–CA bypass graft (). At follow-up 5 years after the surgery, TAA was found to have shrunk, the Ax–CA bypass graft was patent with aspirin treatment, and the patient had no neurological symptoms. |
A 21-year-old Nigerian female with hemoglobin SS presented to a community hospital with a two-day history of left-sided headache and expressive aphasia. Her past medical history was notable for painful crises that occurred approximately once every 5 months and were managed at home with oral analgesics. Physical examination was remarkable for mild pallor and a severe expressive aphasia. She was transfused 2 units of packed red blood cells. The initial CT was normal, although an MRI showed an acute ischemic stroke in the distribution of the left middle cerebral artery. She was transferred to the Johns Hopkins Hospital for further evaluation and management. A complete blood count showed a hemoglobin of 9.9 g/dL, hematocrit of 27.4%, and reticulocyte count of 9.3%. Shortly after admission, she underwent an automated exchange transfusion to achieve a hemoglobin S concentration of 30%. The percent of hemoglobin S after the procedure was 17.0%.\nA transthoracic echocardiogram and bubble study with agitated saline showed bidirectional flow consistent with a PFO. A CT angiogram showed evidence of moyamoya disease. Chronic hypertransfusion therapy was recommended, although the patient did not return for transfusion therapy. She was also placed on daily oral aspirin and folate.\nFour months after the first stroke, the patient was readmitted with complaints of general malaise, weakness, intermittent confusion, and slurred speech noted four days before presentation. Physical examination was notable for a right facial droop, dysarthria, and a deviation of the tongue to the right. A complete blood count showed a hemoglobin of 11.5 g/dL, hematocrit of 32.5%, and reticulocyte count of 4.8%. The percent of hemoglobin S at presentation was 89.4%. She underwent another exchange transfusion to achieve a hemoglobin S of 30%. The day after the exchange transfusion, a doppler ultrasound of the lower extremities was performed to determine if a thrombus was present that could have provided a source for paradoxical embolus. This study demonstrated a thrombus in the common femoral vein at the site of the erythrocytapheresis catheter that had been placed the previous day for the exchange transfusion. The catheter had been removed after the exchange transfusion, and there was no leg swelling or other signs or symptoms of deep venous thrombosis (DVT). It was thought that the thrombus most likely developed in association with the catheter, and the patient was started on enoxaparin (60 mg subcutaneously every 12 hours) in addition to warfarin (5 mg orally each day). The enoxaparin was discontinued when she became therapeutic on warfarin. A hypercoagulable evaluation was done 4 days after the exchange transfusion showed a normal antithrombin III (98%) and protein C (117%). The protein S was slightly low (53%) at this time; a repeat study was recommended. There was no evidence for factor V Leiden or the prothrombin 20210 mutation. An evaluation for lupus anticoagulants and antiphospholipid antibodies (dilute Russell's viper venom test, anticardiolipin, and beta-2 glycoprotein antibodies) was negative.\nThis patient was managed with chronic transfusion therapy. In consultation with hematology, neurology, and cardiology, the decision was made not to repair the PFO given the small size of the PFO and the presence of significant cerebral vascular disease with moyamoya. In sickle cell disease, moyamoya is generally irreversible and often associated with ischemic strokes. She continued warfarin and was started on 40 mg of rosuvastatin for possible atherosclerotic cerebral vascular disease. She received transfusion therapy for five months with no known complications; she subsequently moved to another state and was lost to followup. |
We describe a case of a 36-year-old black African woman with two previous live births by cesarean section and two previous miscarriages who was referred in her fifth pregnancy after 6 weeks of amenorrhea. Her serum quantitative β-human chorionic gonadotropin (bHCG) was 16,124 mIU/ml. However, an intrauterine or extrauterine pregnancy could not be located on a transabdominal ultrasound scan.\nA copper intrauterine contraceptive device had been removed 2 months prior to her last menstrual period. She reported using one cycle of clomiphene 50 mg with the hope of achieving a twin pregnancy.\nShe had delivered twice by cesarean section for failure to progress. Her last two pregnancies had been first-trimester miscarriages; one was managed expectantly, and the other was surgically evacuated, though the actual procedure was unknown to the patient. She did not have any chronic medical illness and was not receiving any medication prior to this presentation. She stayed in a city suburb that was well serviced. She was a school principal in her second marriage with no children in the current relationship. She did not smoke or drink alcohol.\nOn examination, she had a normal blood pressure of 113/70 mmHg and a pulse rate of 98 beats/min. Her body temperature was 37.5 °C. On examination, her cardiorespiratory and neurological systems were normal. Her abdomen was soft and not tender. The result of her pelvic examination was normal. TVUS showed a gestational sac of 13 mm with irregular margins and a visible yolk sac located on the anterior isthmic portion of the uterus, raising suspicion of a cesarean section scar ectopic pregnancy. She declined a Doppler ultrasound evaluation scheduled for the next day. She was scheduled to have serial bHCG evaluations every 48 h.\nA repeat serum quantitative bHCG done 48 h after the initial test revealed a level of 21,521 mIU/ml, a 33% rise. She defaulted follow-up until 1 week later, when she presented with pelvic pain of increasing intensity for 5 days. An urgent transvaginal scan was performed. A fetal pole with active cardiac activity (crown-rump length 0.9 cm) in a gestational sac was located in the anterior low myometrium. The sac traversed the full width of the anterior myometrium, with the posterior margin of the sac abutting the anterior margin of endometrium and the anterior margin of the sac extending to a subserosal location in a fairly exophytic fashion. There was evidence of trophoblastic circulation on Doppler examination. There was no endometrial fluid or free pelvic fluid (Fig. ).\nShe was immediately admitted for a diagnostic laparoscopy/hysteroscopy and possible excision of the scar pregnancy if confirmed. A preoperative complete blood count showed hemoglobin 13.4 g/dl, white blood cells 7.2 × 103/μl, and platelet count 243 × 103/μl. The patient’s kidney function was normal with sodium 135 mmol/L, potassium 4.9 mmol/L, urea 3.5 mmol/L, and creatinine 65 μmol/L. The patient’s liver function test results were also normal. She had a negative result in a blood test for human immunodeficiency virus. Urinalysis did not show abnormalities. The patient’s random blood sugar was 5.6 mmol at admission. At laparoscopy, the bladder was adherent high on the anterior uterine wall, and the ectopic pregnancy was not visualized (Fig. ). At hysteroscopy, there were extensive adhesions within the lower endometrial cavity, which obscured visibility. There was no active intracavitary bleeding ruling out a threatened or inevitable miscarriage. We could not visualize any obvious bulge in the cervical canal suggestive of a cervical ectopic pregnancy. Because of the uncertainty of the location of the pregnancy due to adhesions, excision was postponed. Postoperatively, the patient became unstable with low blood pressure, systolic pressure range of 82 to 95 mmHg and diastolic pressure range of 40 to 55 mmHg, and a pulse rate range of 64 to 73 beats/min, but without active vaginal bleeding or use of medications inducing persistent hypotension. Anesthesia had been induced with etomidate 16 mg and suxamethonium 100 mg, and maintenance was initiated with isoflurane 0.8–1.5%. Intra- and perioperative analgesia was induced with fentanyl 200 mg intravenously (IV), indomethacin 100 mg rectally, and paracetamol 1 g IV. Antibiotic prophylaxis was with ceftriaxone 1 g IV and metronidazole 500 mg IV. This prompted us to order an urgent MRI scan to map the location of the pregnancy in the immediate postoperative period. MRI confirmed the TVUS findings of a cesarean section ectopic scar extending to the serosa (Fig. ).\nAn emergency laparotomy was then performed on the same day. The abdomen was entered through a Pfannenstiel incision along the old skin scar. A transverse incision was made in the upper uterine segment just above the adherent bladder. The products of conception were removed with forceps, and the gap in the anterior myometrium at the old scar was seen and felt. There was massive bleeding from the implantation site. Twenty milliliters of vasopressin (20 U diluted in 100 ml) in normal saline was administered into the bleeding myometrium edges. The edges were apposed in layers with VICRYL suture (Ethicon, Somerville, NJ, USA) to repair the defect. Estimated blood loss was 2000 ml. The patient was transfused with 1 U of packed cells intraoperatively. She was continued on the same intravenous antibiotics and analgesia that had been commenced after the laparoscopy. Her hemoglobin count on day 1 postoperatively was 8 g/dl, and she declined any further transfusion. Oral iron and folic acid supplementation was commenced.\nThe patient’s postoperative recovery was uneventful, and she was discharged on day 4 after surgery. Histology confirmed the presence of decidua and chorionic villi. The patient wanted a child because she was in a new relationship, but she was no longer sure of her future fertility plans after the ectopic pregnancy. A levonorgestrel implant was inserted 2 weeks postoperatively. The patient last attended physical review at 6 weeks, and she was well with no problems related to the surgery at a telephone review at 3 months postoperatively. |
An 85-year-old female, with a history of falls at home, presented to us with pain in the right hip. On radiographs, a communited unstable intertrochanteric fracture was noted. The patient was planned for PFNA on a traction table.\nThe patient was positioned on a fracture table, and a closed reduction is done. Once a satisfactory reduction was achieved, the incision for standard PFNA was made. Once the nail was passed, the lateral incision for the blade was made. The bone was drilled with an appropriate drill, and keeping the tip-apex distance to be <25mm, a screw of size 90cm was chosen. On passing the blade, it was seen on the c-arm that the blade was greater than expected and would lead to joint penetration and hence, needed to be revised to a smaller size. For this, the blade was removed and a new blade of size 80cm was taken and driven along the same path. The surgeon felt resistance while reaching the end of the blade, and on seeing in the c-arm, a part of the previous blade was found to be inside the head and had been pushed further across the joint, due to the force by the new blade (Figure ).\nThe surgeon checked the part that had been removed earlier and found out that it was only the sleeve that had been removed, and the helical blade had broken and was left in situ. The surgeon decided to use a Watson Jones approach to open the fracture and expose the head and remove the helical blade with a nibbler (Figure , ). Once removed, the fracture was reduced, and the nail was reinserted and a new blade of size 80cm was put in (Figure ). The wound was closed in layers with a drain in situ, and the patient was kept non-weight bearing for 6 weeks.\nPost-operative course\nThe patient was kept non-weight bearing for 6-8 weeks till there were signs of new bone formation. This was warranted by the operating surgeon due to the highly osteoporotic nature of the bone, and fear of screw cut out on early weight-bearing, even though the tip-apex distance was appropriate. The patient was subsequently started on toe-touch weight-bearing with a walker and slowly converted to partial and then full weight-bearing. The patient continued to have pain and difficulty in weight-bearing even at 4 months postoperatively. The patient expired at 8 months from surgery due to natural causes. |
A 32-year-old male, addicted to heroin for 3 years, was found in a semiconscious, unresponsive state in his room and brought in a state of shock with gasping respiration by family members to the emergency room. He was hypothermic, hypotensive with blood pressure 70/45 mmHg, a heart rate of 128 per min, shallow respiration with a rate of 12/min, and hypoxemic with oxygen saturation 88% on 10 liter per min oxygen. Initial evaluation was suggestive of an acute opioid overdose as the patient was a known heroin addict; there were pinpoint pupils with hypoventilation and hypothermia. A blood sample was sent to the toxicology laboratory. After 0.4 mg of intravenous naloxone in increments of 0.1 mg boluses, patient's sensorium level improved and respiration became more normal with an improvement in oxygen saturation, but he remained hypotensive. Fluid resuscitation was continued, vasopressor (noradrenaline infusion) added, and an infusion of naloxone was started. The patient was shifted to the ICU for further observation and treatment. Blood gas analysis showed persistent metabolic acidosis. Hematological parameters were normal but biochemistry revealed signs of acute rhabdomyolysis with a marked rise in the creatinine kinase level, hyperkalemia, and high blood urea nitrogen (BUN) and creatinine. Aggressive hydration with alkalinazation and forced diuresis was started. Despite the aggressive therapy for about 3 h, the patient went into anuria with a marked rise in creatinine. The patient started to agitate, and become restless with a complaint of generalized body and joint pain and his respiration became labored; auscultation revealed bilateral diffuse crepitation. The patient was intubated and connected to a mechanical ventilator with propofol and remifentanil infusion as sedative-analgesic. An urgent chest X ray confirmed acute pulmonary edema. Nephrology consultation was sought for urgent dialysis; naloxone infusion was stopped. Although the metabolic parameters improved after urgent dialysis, the ventilatory parameters worsened requiring higher amounts of oxygen and a higher level of positive end expiratory pressure (PEEP). Echocardiographic evaluation revealed a normal cardiac function. The lung condition worsened further over the next couple of days with severe hypoxemia consistent with ARDS. The patient was managed with a lung protective ventilatory strategy with the infusion of methyl prednisolone 1 mg/kg/day along with other supportive care in the form of regular dialysis, antibiotics, nutritional support, chest physiotherapy, etc. In view of the prolonged ventilatory support, the sedation-analgesia regime was changed into midazolam and morphine. After about 10 days, respiratory parameters were gradually improved, and the kidney function started to recover. The patient was successfully extubated and liberated from the ventilator when he passed the spontaneous breathing trial (SBT) on day 15. Few hours after extubation, the patient started to show signs of opioid withdrawal with agitation, generalized aches and pain all over the body, lacrymation, increasing restlessness, tachypnea, tachycardia, and hypertension. The trial of intravenous haloperidol failed to control his agitation. He was reloaded with a short-acting opioid, remifentanil, to which he responded; psychomimetic and other physical symptoms were modestly controlled with 400 μg/h of remifentanil, but he required increasing doses of remifentanil and was restless and agitated on the slightest reduction of remifentanil infusion. The trial of midazolam and propofol infusion failed to reduce the remifentanil requirement. On day 18, dexmedetomedine infusion was added after a loading dose of 50 μg over 30 min, and remifentanil infusion was successfully tapered off over 4 h. The patient was managed with a continuous infusion of dexmedetomidine 0.7 μg/kg/h, and lorazepam was added for night comfort. On day 5 of dexmedetomidine infusion, we gave oral clonidine 25 μg every 4 h to the patient and slowly tapered off dexmedetomidine over the next 2 days. He remained conscious, oriented, and calm throughout the dexmedetomidine treatment. Finally, he was shifted to a rehabilitation center on oral clonidine. |
A 57-year-old man with an extensive life-time history of sun exposure and long-term smoking was evaluated for a recurrent cutaneous chest wall keloid. The keloid initially developed secondary to a varicella zoster infection as a child. At age 9, the keloid was treated with low-dose external beam irradiation and essentially resolved. Many years later and over a period of eighteen months prior to clinician reevaluation in 2009, the lesion began to grow in size. Corticosteroid injections failed to induce regression and a punch biopsy was performed.\nPathology from the punch biopsy revealed a primary desmoplastic melanoma, at least 5.0 mm thick with positive margins arising within a keloid. The patient's review of systems revealed vague muscle and rib pains, right-sided headaches, abdominal pain, and a forty-five-pound weight loss. Staging workup was completed to investigate these symptoms and revealed no evidence of metastatic disease.\nClinically, it was impossible to differentiate keloid from desmoplastic melanoma. Thus, a two-stage surgical procedure was performed with initial resection of the primary site including the entire keloid with 2 cm margins and bilateral axillary sentinel node biopsy (Figures , , and ). Temporary coverage of the primary resection defect was performed with porcine dermal substitute (not pictured) to ensure a margin free resection prior to reconstruction, since it was difficult to determine how deep the melanoma had invaded macroscopically.\nFinal pathology revealed desmoplastic melanoma occurring in the keloid with clear radial margins and a deep margin within 0.1 cm of the resection (Figures , , and ). Thus, one week after the initial surgery, the patient returned to the operating room and a new deep margin was obtained, which was negative for malignancy. Bilateral sentinel lymph nodes were negative for metastasis. Partial thickness skin graft reconstruction was then performed to cover the previously created 145 cm2 defect. The partial thickness skin graft had a 100% take (). The patient was offered adjuvant immunotherapy but declined. He remained recurrence free from melanoma at his thirteen month follow-up evaluation. However, two months after this follow-up appointment, he was diagnosed with a primary squamous cell carcinoma of the lung, which rapidly progressed causing his death, just three months later. |
A 17-year-old-male patient reported to the Department of Dental Sciences with a gross asymmetry over the right anterior region of the mandible. The patient gave a history of loosening of teeth since 1 year. Intra-oral examination revealed a bony hard swelling in the right mandibular region extending from central incisor to first molar []. Right mandibular lateral incisor, canine, first premolar, and second premolar were mobile and gave no response on electrical pulp testing. On palpation the swelling was non-tender, irregular in shape, and measuring 2 cm × 3 cm. Orthopantomogram showed a large well-circumscribed radiolucency extending from right mandibular central incisor to the mesial root of first molar []. Due to the expansive growth there was deviation of the roots of right mandibular canine and first premolar. Root resorption was also evident in relation to the first premolar, second premolar, and mesial root of first molar on the right side. Based on these features, a differential diagnosis of odontogenic cysts and tumors (like odontogenic keratocyst, lateral dentigerous cyst, AOT, calcifying epithelial odontogenic tumor) was given. Other differential diagnosis included central giant cell lesions, lateral periodontal cyst, lateral radicular cyst, and the much rarer central benign mesenchymal neoplasms. The lesion was operated under local anesthesia and the specimen was sent for histopathologic examination. The mobile teeth, that is, mandibular lateral incisor, canine, premolars, and the first molar were removed along with the lesion []. Grossly, the lesion was white to tan, nodular tissue with cystic spaces containing yellowish brown semisolid material []. Microscopic examination revealed extremely vascular encapsulated lesion showing multivariate patterns of cellular arrangements ranging from sheets of polygonal cells arranged in ductal pattern, rossetes to solid sheets of cells []. In the center of these ducts, eosinophilic amyloid-like material was also seen. The solid lobular masses showed numerous spindle to columnar hyperchromatic cells with interspersed deposits of eosinophilic hyaline-like material. Mitotic figures were also seen in the lobules [Figures and ]. The above features were consistent with the diagnosis of AOT. |
A 56-year-old female presented with bilateral otalgia and hypoacusis gradually progressing for the past two weeks and left sided facial palsy significantly increasing within two days. She has been previously twice unsuccessfully treated with antibiotics in another hospital due to chronic otitis media. She also had a history of psoriasis and hyperthyreosis. AAV may occur with antithyroid drug therapy. However, the patient was treated only one month before the blood test; thus the possibility of drug-induced AAV is low.\nOtoscopic examination revealed bilaterally thickened and reddish eardrums. There was a subtotal perforation of the right tympanic membrane and an anterior perforation of the left tympanic membrane with effusion. Left facial nerve palsy was categorized as grade IV according to House-Brackmann. Nose examination was normal. The pure tone audiometry showed severe bilateral mixed hearing loss on the level of 80–100 dB with air bone. On the right the threshold is on the level of 95–100 dB with air bone gap of 50 dB. On the left the threshold gap is 70 dB. Chest X-ray revealed signs of bronchitis. Urine analysis was normal.\nHigh resolution computed tomography scans of the temporal bones showed bilateral sclerosing mastoiditis and opacification of the right tympanic cavity with an air-fluid level. No signs of bony destruction within the ossicular chain, the internal ear, or facial canal were present (). Magnetic resonance imaging (MRI) with the use of T2-weighted, T1-weighted, and contrast enhanced images was performed and showed normal appearance of cerebral structures and mastoid cells filled with fluid on both sides. Hypertrophic pachymeningitis sometimes shows in the GPA patients wit hear involvement. In this case there were no signs of pachymeningitis on MRI examination. The diagnosis of acute otitis media with peripheral facial nerve paralysis was made and intravenous antibiotics treatment was started. As there was no response to drug therapy, the patient was referred to myringoplasty and antromastoidectomy with facial nerve decompression. During surgery, granulation in the mastoid cavity was found. There was no granulation on the facial nerve canal. The facial nerve was pale and swollen during decompression. After surgery otalgia and hearing on the left side improved slightly, but facial paralysis did not show any improvement. The patient was discharged and sent home with prescribed antibiotic treatment.\nAfter two months, the patient's condition deteriorated significantly as she presented with bilateral facial paresis (House-Brackman grades IV-V), horizontal nystagmus directed to the right ear, severe hearing loss (70–80 dB), otalgia, and otorrhea. Blood tests showed WBC of 12,93 thousand/μL (normal range: 4–10) and CRP of 147,76 mg/L (normal range: 5,0). Laboratory tests showed hyperthyreosis with positive results for antibody to thyroglobulin: A-TG of 180 U/L (normal range: 0,0–60). Renal function tests and abdominal ultrasound were normal. Intravenous antibiotic treatment was administered.\nChest X-ray showed bilateral hilar mass lesions. Chest CT scans revealed mediastinal nodules and parenchymal consolidations in both lungs (). Head MRI showed no pathological changes except for complete opacification of mastoid cells and middle ears bilaterally. Ophthalmic evaluation revealed bilateral scleritis.\nDue to the suspicion of lungs metastatic disease, the patient was referred to thoracotomy. The histopathology tests of pulmonary lesion suggested tuberculosis, which resulted in introduction of tuberculostatic treatment. With no improvement after tuberculostatic treatment and suspicion of GPA, serological ELISA tests were performed and c-ANCA tests were positive. Subsequently the patient was transferred to Rheumatology Department, where the diagnosis of GPA was confirmed. Control laboratory tests showed WBC of 14,9 thousand/μL (normal range: 4–10), markedly increased value of c-ANKA > 150 U/L (>8,0 U/mL positive result), and abnormal urine analysis with the presence of protein and red blood cells. Follow-up head CT showed chronic inflammatory changes with polyps in paranasal sinuses.\nThe patient was commenced on systemic steroid therapy in the form of Solu-Medrol 2,0 g per day and cyclophosphamide (Endoxan) 600 mg per day with the protection of Uromitexan. The total dose of Endoxan was 5,4 g. In the course of treatment, the patient's condition improved concerning hearing and nystagmus. Facial nerve palsy regressed from 5th to 2nd grade bilaterally in House-Brackman scale.\nUnfortunately, the patient's renal function deteriorated gradually and she died 2 years after the initial otological symptoms due to progressive renal disease. |
A 60-year-old lady presented with a one-week history of shortness of breath, low-grade fever and right-sided pleuritic chest pain. She had been treated with azithromycin for presumed bronchitis with no improvement. She had RA factor positive rheumatoid arthritis diagnosed about 3 years prior and was doing well with hydroxychloroquine and low dose prednisone. Her other medical problems included osteopenia, gastroesophageal reflux disease and hypercholesterolemia for which she was on weekly risedronate, calcium and vitamin D supplements, omeprazole and atorvastatin respectively.\nOn examination her vital signs were stable, she was afebrile and her oxygen saturation was 95% on room air. She appeared tired but not in any distress. Auscultation of the lungs revealed wheezes bilaterally and few crackles at the right base. The remainder of her examination was unremarkable. She did not have any synovitis in the joints at this time and range of motion was normal in all joints with no muscle wasting. Skin examination showed some old hypopigmented macules over her neck and face. There were no skin nodules.\nHer white count was 7.8 × 109/l with neutrophils of 84%. The ESR by Westergren's method was 46 mm at the end of one hour. A comprehensive metabolic profile was normal and urine analysis did not show any sediment. Her chest radiograph showed streaky infiltrates at the right base. A high resolution CT scan showed three lung nodules (Fig ). An FDG PET scan was done which showed avid uptake in all the nodules with an SUV value of 8 (Fig ). A CT-guided lung biopsy of the right lung nodule was performed. The lung biopsy showed fibrosis with acute and chronic inflammation and necrotizing vasculitis (Fig ). Biopsy specimen examined for aerobic and anaerobic bacteria, acid fast bacilli and fungus, was negative on smear and culture. A complete vasculitis panel was obtained which included antinuclear antibodies, anti-dsDNA, anti-Smith, anti-SSA and SSB, anti-histone antibody, anti-Jo-1, anti-centromere antibody, anti Scleroderma -70, anti-proteinase 3 and anti-myeloperoxidase antibodies along with the antineutrophil cytoplasmic antibodies (ANCA). The only positive tests were an elevated cANCA in a titer of 1: 640 (Normal less than 1: 20) and anti-proteinase 3 antibodies of 118 units (Normal 0 – 20 units).\nA screening CT scan of the sinuses was normal. A two-dimensional echocardiogram showed a left ventricular ejection fraction of 55% and normal pulmonary artery pressures. Based on the biopsy and the serological findings, a diagnosis of limited Wegener's granulomatosis presenting as lung nodules was made.\nMethotrexate was started and the low-dose prednisone was continued. Her symptoms improved dramatically with these and a repeat CT scan of the chest obtained one month after treatment showed regression in the size of the nodules and one done 3 months later showed complete resolution of the same. She will be followed closely for development of kidney or upper respiratory tract involvement and monitored for methotrexate toxicity. |
An elective pancreatoduodenectomy was scheduled for a 35-year-old woman with a pancreatic mass suspected to be a solid pseudopapillary neoplasm (). Her medical history included two cesarean sections and moderately increased blood pressure (160/80 mm Hg), which was managed with ramipril. The blood pressure was 145/100 mm Hg on the day of surgery. During induction of anesthesia (with extradural injection of bupivacaine and epinephrine) and after application of mild pressure to the abdomen, the blood pressure increased suddenly to 266/167 mm Hg and, after abdominal incision, increased further to 288/180 mm Hg. Tissue preparation was stopped, and severe hypotension (blood pressure: 50/20 mm Hg) developed, which was managed with intravenous catecholamines. The operation was discontinued, and the patient recovered in the intensive care unit.\nAfterwards, the patient was referred to our department for further diagnostic evaluation. A detailed medical history revealed episodes of generalized weakness, shortness of breath, palpitations, and skin pallor, lasting approximately 2 minutes, which had occurred once or twice monthly during the previous year. Symptoms were worsened by sitting and improved by walking and deep breathing. Moreover, we learned that the patient had previously been hospitalized with increased blood pressure during pregnancy. The workup showed marked elevations in urinary excretion of methylated catecholamines (). Based on previously obtained abdominal computed tomography and magnetic resonance imaging, a team of radiologists, internists, and surgeons agreed that the pancreatic tumor resembled a pheochromocytoma extending from the left adrenal gland (). Thus, the patient was scheduled for lateral transperitoneal laparoscopic adrenalectomy.\nThe patient received preoperative doxazosin at a final dose of 4 mg four times daily for a 1-month period, which normalized blood pressure (110–120/70–80 mm Hg). Because the tumor was well vascularized, we used a harmonic scalpel for the adrenalectomy. Immediately prior to ligation of the adrenal vein, blood pressure increased to 310/120 mm Hg, which was managed with intravenous sodium nitroprusside. The operation lasted for 3 hours, and the excised tumor measured 5.7 cm × 4.5 cm × 5.0 cm (). After the operation, the patient was in good condition, with normal blood pressure, and was discharged after 5 days (). Symptoms did not recur, and urinary excretion of methylated catecholamines was normal 6 months after the adrenalectomy (). Pathological examination revealed a pheochromocytoma that did not infiltrate the adrenal capsule or local blood vessels.\nEthical approval: The research related to human use has been complied with all the relevant national regulations, institutional policies and in accordance the tenets of the Helsinki Declaration, and has been approved by the authors' institutional review board or equivalent committee.\nInformed consent has been obtained from patient included in this study. |
A 64-year-old African American male was referred to the pulmonary clinic for evaluation of abnormal imaging. His primary care physician had obtained a chest radiograph after the patient presented with two weeks of a productive cough. The patient otherwise was asymptomatic. He had been diagnosed with chronic obstructive pulmonary disease, diabetes, allergic rhinitis and benign prostatic hypertrophy. His medications which adequately controlled his medical problems included inhaled triamcinolone, albuterol, metformin and tamsulosin. He had been an active smoker until 8 years ago and had a 60 pack-year history of smoking prior to that. He did not drink alcohol and never used illicit drugs. He worked in an automobile factory for about twenty five years. However, for the last fifteen years his only job was as a steel welder where he was involved in welding the steel frames for the automobiles. During that time, it was the only task that he was doing in the factory. He did use eye protective shields and a face mask which were provided to him. The part of the building he worked in was small and enclosed, and the exhaust system was also not optimal. There were no employee health surveillance programs implemented by the employer until about ten years ago. However, these were done infrequently and patient was not subjected to one while he was working.\nWhen patient was seen in the pulmonary clinic, he still complained of occasional productive cough, but otherwise denied having fevers, night sweats, chest pain, shortness of breath, orthopnea or unintentional weight loss. On physical examination, his vitals revealed a blood pressure of 123/73, pulse of 68/minute, respiratory rate of 17/minute and temperature of 97.8 Fahrenheit. Pulmonary examination did not reveal any crackles or wheezing. The remainder of the examination was also within normal limits. Spirometric assessment revealed an FEV1 of 2.59 liters (104% of predicted) and FVC of 3.83 liters (113% of predicted). However FEV3/FVC ratio was reduced suggesting mild obstruction in the mid-flow range. His chest radiograph (CXR) showed a pattern of reticular-nodular opacities bilaterally, with lower lung field predominance (Figure ). A computed tomographic scan (CT) of the chest showed multiple small reticulonodular opacities without lymphadenopathy (Figure ). As the patient was having recurrent episodes of productive cough, in order to evaluate for underlying infection, he underwent a bronchoscopy with bronchoalveolar lavage (BAL) and transbronchial biopsies. The bacterial, viral, fungal and acid fast bacilli cultures from the BAL fluid were negative. The biopsy specimens showed dense nodular interstitial fibrosis containing clusters and sheets of macrophages having cytoplasmic iron pigment (Figure ). This was also confirmed with Prussian Blue staining. The diagnosis of pneumosiderosis (Welder's lung) was made. Ferritin levels were not measured in the BAL as the diagnosis was easily established by the biopsy specimen. The patient was advised to change his job, as even after preventive measures he developed Welder's lung from significant iron dust exposure. His intermittent bouts of productive cough were treated with bronchodilators and steroids.\nThe patient retired from his job after his initial evaluation. His respiratory symptoms, which first appeared after about fifteen years of the initial exposure to iron dust, resolved within a month with supportive therapy. One year after the initial presentation, he is doing well without any pulmonary complaints. |
A 73-year-old Japanese man was admitted for OPCAB due to symptomatic angina. His medical history included hypertension, chronic kidney disease, and asymptomatic bilateral internal carotid artery (ICA) stenosis. Carotid ultrasonography showed 94.0% of stenosis in the right ICA area with a peak velocity of 283.2 cm/s. The left ICA showed a peak velocity of 595.2 cm/s, although the stenosis rate of area could not be counted. Coronary angiogram showed 99% stenosis of the left anterior descending coronary artery (LAD), 99% stenosis of the distal segment in the right coronary artery (RCA), and 90% stenosis in the proximal segment of the left circumflex coronary artery (LCX). Left ventricular angiography showed severe hypokinesis of the posterior wall with an ejection fraction of 40%. Although carotid ultrasonography demonstrated bilateral ICA stenosis, preoperative CT of the brain showed no ischemic stroke with any neurological symptoms. On the basis of these findings, there was no indication for preceding carotid endarterectomy or carotid artery stenting, and revascularization was selected performing OPCAB to avoid perioperative stroke. OPCAB was performed, using the left internal thoracic artery to graft the LAD, the saphenous vein graft (SVG) for the posterior descending artery of the RCA, and the SVG for the posterior lateral of the LCX. The anesthesia and operation times were 6 h 11 min and 4 h 11 min, respectively. He was hemodynamically stable during and after surgery. Four hours after the operation in intensive care unit (ICU), he regained consciousness from the anesthesia, demonstrating left-sided hemiparesis with sensory disorder. We suspected a perioperative stroke because of his bilateral ICA stenosis. His National Institutes of Health Stroke Score (NIHSS) was 10 out of 42 points at that time. A CT of the brain indicated a slightly low density of the right frontal and insular cortex (Figure ). Additional CT with a contrast agent as 3D-CTA of the neck and brain was performed and exhibited multiple large-vessel occlusions: the origin of the bilateral ICA, the origin of the right vertebral artery, and the M2 segment of right middle cerebral artery (MCA; Figure ). We believed that there was a small ischemic core and that the brain tissue would be largely salvageable by recanalization of the right ICA and MCA; hence, endovascular thrombectomy was suggested. An initial injection of the right common carotid artery indicated occlusion of the right cervical ICA (Figure ). Manual aspiration with a syringe was performed with the balloon inflated to arrest the flow of the right ICA. Sizable clots were removed. An injection of the right ICA showed completely recanalized ICA (Figure ), with moderate stenosis at the origin and occluded M2 segment of MCA (Figure ). Endovascular thrombectomy using a Solitaire (Medtronic) stent retriever was performed for occlusion of the MCA, and a subsequent ICA injection demonstrated excellent reperfusion of the MCA with a modified thrombolysis in cerebral infarction score of 2A (Figure ). Although the occlusion of the left ICA was detected (Figure ), it was not the responsible vessel of these neurological deficits. The recanalization was not performed because there was a high risk of distal embolism, which might have caused new deficits, or bleeding due to duration of angiography after open-heart surgery. Since recanalization of the responsible vessel was most important first of all, the routine four vessel angiograms were not performed. After recanalization, followed-up CT of the brain showed no intracerebral hemorrhage and slightly low density of the right frontal and insular cortex (Figure ). His left-sided hemiparesis improved immediately after the thrombectomy. We administered 100 mg of aspirin daily for this acute ischemic stroke and ICA stenosis. Followed-up CT of the brain at day 7 revealed ischemic edema of the frontal operculum and temporal pole (Figure ). His neurological examination found improvement gradually, and he left the ICU at day 9. He continued rehabilitation for his residual neurological deficits. Finally, his left-sided hemiparesis persisted only slightly, and the NIHSS was improved two out of 42 points. The patient was discharged from the hospital on day 46. He has been followed up as an outpatient. Subsequently, carotid artery stenting was performed for his remaining right ICA stenosis 6 months later. He exhibited no recurrence of symptomatic ischemic stroke or angina for 1 year with his medical treatment. |
A 66-year-old man was referred to our clinic for an asymptomatic AAA. Computed tomography (CT) urography revealed a 6.6 cm infrarenal AAA and a HSK with a wide parenchymatous isthmus, and CT angiography showed multiple renal arteries (). Four of the accessory renal arteries arose from the aneurysm body, and supplied the inferior poles and isthmus. Two of these, arising from the upper part of the aneurysm were relatively large with diameters of 2.5 mm and 3.1 mm. Initially, the patient had normal renal function with a serum creatinine of 1.00 mg/dL.\nOpen surgical repair was decided due to short common iliac arteries, angulation of the neck greater than 60 degrees, and the presence of two relatively large accessory renal arteries. The operation was performed through a long midline incision. Transperitoneal dissection along the Treitz ligament revealed the aneurysm with its upper third being shielded by the isthmus of the HSK. The infrarenal aorta, external and internal iliac arteries were dissected for clamping and accessory renal arteries were carefully isolated (). The aneurysm sac was opened and a Y-shaped Dacron graft (18-9 mm, Hemashield; Maquet Corporation, Rastatt, Germany) was placed under the isthmus for reconstruction. Both common iliac arteries were spared from aneurysmal change, but were heavily calcified and short, and thus, distal anastomoses were performed to the right iliac bifurcation, and to the left external iliac artery. The larger two accessory renal arteries were reimplanted to the graft with aortic patches. The accessory renal arteries were clamped for 30 and 50 minutes, respectively. One hundred mL of 15% mannitol and 10 mg of furosemide were infused before clamping for renal protection. The other two small arteries were ligated. The operation took 6 hours and 10 minutes. Estimated amount of blood loss was 2,100 mL and 4 pints of blood was transfused during the operation. After 1 day of intensive care unit stay, he was moved to the general ward. He was discharged at postoperative 7th day.\nAlthough occlusion of one of the implanted arteries with infarction of the isthmus was noticed on CT taken at 6 days postoperatively, renal function was stable with a serum creatinine level of 0.90 mg/dL. CT angiography at 18 months postoperatively showed the renal infarct had remained unchanged without any progression, and that both the aortic graft and left accessory renal artery were patent (). Currently, the patient is clinically well at 29 months postoperatively. |
A 32-year-old female patient was referred to a dentist for evaluation of a painful expansion in her mandible []. Intraoral examination revealed a diffuse swelling in the posterior area of the left mandible. The overlying mucosa was erythematous, ulcerated and necrotic []. The panoramic radiograph showed a radiolucent lesion with ill-defined borders extending from the second premolar to second molar []. The incisional biopsy was performed, and the general pathologist's diagnosis was ameloblastoma. Other consulting reports from other General Pathology Departments were ameloblastic fibroma (AF), osteosarcoma and mesenchymal chondrosarcoma. Due to the first microscopic diagnosis (ameloblastoma), en bloc resection from the second premolar to the third molar was done and reconstruction was performed after 6 months using autogenous iliac bone graft stabilized using titanium mesh and plate. Two years later, she came back with a huge recurrent lesion and obvious facial asymmetry. Therefore, she was referred to an oral and maxillofacial pathology center for more investigation, and her primary biopsy specimens and previous histopathological slides were reviewed. Two slides (number 1, 2) of the primary lesion were present. Careful evaluation of slide number 1 showed scattered benign ameloblastic islands and dental papilla-like stroma with tumoral giant cells [Figures and ]. Hyalinization was seen around these benign islands []. Dentinoid material was also found near some odontogenic epithelial nests []. Slide number 2 revealed a highly cellular mesenchymal tissue with hyperchromatic and bizarre pleomorphic cells without any epithelial islands. The malignant counterpart was similar to a poorly differentiated round-cell sarcoma. Some eosinophilic materials, most likely dysplastic dentin, were also found in this malignant counterpart [Figures and ]. The eosinophilic material was osteoid-like, and in these areas, the histopathologic feature was very similar to fibroblastic osteosarcoma. These varying microscopic features justified the different and unusual pathologic reports of the lesion as ameloblastoma, AF, osteosarcoma and mesenchymal chondrosarcoma. However, due to the histopathologic features of the lesion, the diagnosis of AFOS was confirmed. No cervical lymphadenopathy was present and the laboratory data were unremarkable. Computed tomography scan of the abdomen, pelvic sonography and chest X-ray were normal, and the patient was referred for surgical treatment. As well, adjuvant chemotherapy consisting of fluorouracil and cyclophosphamide was carried out. Unfortunately, 12 months after surgery and in spite of local tumor control, pulmonary, mediastinal and axillary lymph node metastases arose, indicating a progressive systemic disease. Therefore, she was referred to the oncologist for palliative chemotherapy. |
We present a 57 year old gentleman with CKD 5 who had an autogenous brachiocephalic fistula 4 months prior to presentation to us. During their last surgical clinical visit, the fistula was noted to be poorly maturing and then referred to interventional radiology for fistulogram and possible endovascular intervention to assist with fistula maturation.\nThe patient had a fistulogram which demonstrated a high grade juxta-anastomotic stenosis which was successfully balloon dilated. After a 6 week follow up clinic visit the fistula was still immature and a duplex scan, a second fistulogram with possible intervention were requested.\nFistulogram was performed via an antegrade approach from an access just proximal to the swing point. There was an “apparent” stenosis (Fig. ) which was angioplastied then followed by severe spasm (Fig. ). which was perceived by the operator to be recalcitrant stenosis. In the light of this perceived recalcitrant stenosis, a decision to stent the area was taken. After measuring the vessel diameter based on the immediate post-plasty images a 6 mm diameter × 5 cm length Viabahn stent (Gore & Associates, Flagstaff, AZ) was selected and deployed in the standard fashion.\nFollowing stent deployment, the stent migrated and stopped at the confluence of the cephalic vein and the subclavian vein (Fig. ). At the time the operator thought the stent was stable and unlikely to cause harm to the patient in this position. However, after reviewing the images with colleagues including vascular surgeons a decision was made to attempt to retract the stent into the arm which would be easier for the surgeon to retrieve the stent surgically, if required. The patient was subsequently brought back 24 h later to the interventional radiology suite.\nInitial fluoroscopic image of the left shoulder region demonstrated the stent was absent from the final position documented the previous day indicating the stent had migrated further (Fig. ). Fluoroscopic scanning of the chest identified the stent to overlie the left lower lobe (Fig. ).\nSubsequent Pulmonary angiogram confirmed the stent to lie within a segmental pulmonary artery of the left lower lobe (Fig. ).\nAfter discussion of the options, risks and benefits with the patient and a multidisciplinary team, a decision to attempt stent retrieval was made versus leaving the stent in situ.\nAfter appropriate informed written consent, the right groin was prepped and in the standard fashion. Right common femoral vein access was then upsized to accept a 16 F sheath (Cook, Bloomington. IN USA). Main pulmonary access was then performed with an APC pulmonary catheter (Cook, Bloomington. IN USA). The APC catheter was then removed over a Storq wire (Cook, Bloomington. IN USA) wire and subsequently a 12 F 70 cm braided sheath was advanced into the main pulmonary artery and then left lower lobe pulmonary artery. Pulmonary angiograms performed identified the optimal projection to identify the vessel to access. After accessing the appropriate vessel the 12F sheath was advanced just to the origin of the branch above the stent. Subsequently a 15 mm Amplatz Gooseneck snare (ev3, Plymouth MN, USA) was manipulated until the stent was lassoed at about half way along the stent. Given the flexibility and potential collapsibility of the Viabahn stent it was over-sheathed carefully collapsing and gently retracting the captured stent to minimize potential vessel injury (Fig. , Additional file ). Once the stent had been totally ensheathed, the 12F sheath was retracted through the outer 16F sheath coaxially. The stent was retrieved intact (Fig. ).\nThe procedure was performed under moderate sedation using Fentanyl and midazolam with continuous monitoring of the patient’s vitals by a dedicated nurse. Throughout the procedure the patient remained hemodynamically stable with normal respiratory function. Post procedure the patient was observed for 6 h post procedure before being discharged home in a stable condition.\nThe patient has so far been followed up for 3.5 years and has not developed any adverse pulmonary or cardiac condition. Interval CTPA done at an outside facility showed normal pulmonary vasculature with no evidence of pulmonary vessel injury. |
A 6-month-old male child presented with feeding difficulty and intraoral swelling noticed at 2 months of age. The swelling was progressively increasing in size. No history of fever, cold and cough. The child was immunized adequately for the age with no delay in developmental milestones. General examination was unremarkable except for the presence of mild pallor.\nOn local examination, the swelling was well-defined measuring 3 cm × 2 cm, firm in consistency with smooth overlying surface. The swelling was nontender and nonpulsatile. The swelling did not bleed on touch. No abnormality was found on systemic examination. A plain radiograph of skull revealed a lytic lesion in the maxilla. On CT scan, an expanded soft-tissue swelling in the right maxillary antrum was noticed. Expansion of the involved bone was seen. Chest radiograph was normal. In view of the above findings, 24 h urine sample was sent for the estimation of VMA, which was found to be normal. Wide local excision of the tumor was done and sends for histopathological examination.\nGrossly, the specimen received measures 4 cm × 3 cm × 2 cm and on the serial section, cut surface showed tumor measures 2 cm × 2 cm × 1.5 cm with attached tooth measures 0.6 cm × 0.5 cm × 0.5 cm. The cut surface of tumor showed heterogeneous gray-black appearance. On microscopic examination, the tumor was composed of cells arranged in alveolar pattern separated by fibrovascular stroma []. Two distinctive types of cells were seen – large cells arranged peripherally with abundant cytoplasm, round vesicular nucleus and brown pigment; and small cells with scanty cytoplasm and hyperchromatic round nuclei were seen in the center []. Histological features were consistent with a small round cell tumor favoring melanotic neuroectodermal tumor of infancy. Immunohistochemical studies were done for confirmation. Cytokeratin, [] MELAN-A and HMB45 [] were positive in large cells and negative for small cells. Neuron-specific enolase (NSE) [] and synaptophysin [] were positive in small round cells and negative in large cells. S100, CD99, LCA were negative in large and small cells. Thus, a final diagnosis of MNTI was made by correlating HPE and immunohistochemistry (IHC) findings. This case was followed up for the next 6 months without any recurrence. |
A 74-year-old female came to our emergency department (ED) with fever and dysuria of 3 days duration. She was in Peru a week prior to presentation and had similar complaints. She was seen by a local physician there and was told that she had a urinary tract infection. She received a one-time injection of an unknown medication after which she felt much improved. She then travelled to the United States and had been here for 3 days after which her symptoms recurred.\nHer medical history was notable for hypertension. She had no prior illness that required hospitalization and led a relatively healthy lifestyle. She had an unremarkable family history including no history of sudden death.\nIn the ED, she was noted to have a temporal temperature of 39°C, tachycardia at 101 bpm with a blood pressure of 129/49 mm Hg; her physical exam was unremarkable. Laboratory data were notable for leukocytosis with bandemia and pyuria. She was to be admitted for sepsis from a urinary tract infection. Within an hour she complained of increasing chills, and a repeat rectal temperature was 41°C. Prior to administration of antipyretics she developed a generalized tonic–clonic seizure. A computed tomography scan of the head was unremarkable. She was not on a cardiac telemetry at the time of the seizure.\nAn ECG was obtained that showed sinus tachycardia at 106 bpm with coved ST segment elevation in leads V1 and V2 (more pronounced in V2), characteristic of the Brugada ECG pattern (). Echocardiogram showed normal left ventricular systolic function with a left ventricular ejection fraction of 55% to 60% and no segmental wall motion abnormalities or valvular abnormalities. Her blood work was notable for mild elevation of troponin I at 1.5 ng/mL (normal reference range = 0.0-0.5 ng/mL), which was attributed to demand ischemia. Repeat ECGs during the febrile period continued to show the same precordial ST segment changes. Telemetry revealed no significant events. She had no further seizures. A neurologist was consulted who recommended no further workup for her first and one-time seizure episode.\nAfter 48 hours of intravenous antibiotics, she showed clinical improvement and was no longer febrile. A repeat ECG with normal temperature demonstrated reversibility of her classic Brugada type 1 changes with clinical defervescence (). She had no history of prior syncope, seizure, or palpitations. She refused to undergo any interventional studies; therefore, a cardiac catheterization and an electrophysiological study were not performed despite our recommendation.\nAt the time of discharge she was advised to have prompt intervention with antipyretics for any future febrile episodes and also to avoid certain medications (see Discussion). At 2-month follow-up, she remains well and free of cardiac events. |
An 8-year-old African boy, a primary school pupil, from rural Kenya presented to our unit with a 1-year history of cough, difficulty in breathing and dyspnea on exertion that were relieved by rest. His difficulty in breathing was worse at night and there were no reported known relieving factors. His dyspnea was progressive in spite of antibiotics, inhalers and treatment for pulmonary tuberculosis (PTB) for 6 months. His cough was dry and associated with constitutional symptoms; he had no history of hemoptysis, chest pain or foreign body inhalation. Our patient had no features of cardiac failure, no dysphagia or odynophagia. He had been treated for PTB at the district hospital without improvement.\nThere was no history of similar illness nor was there a history of tumors in the family. Our patient was well prior to the onset of these symptoms with no history of previous hospital admission. A chest X-ray was done after completion of the anti-tubercular medication since there was no improvement. After the X-ray, the patient was referred to our facility. When we examined our patient, he was tachypneic at 40 breaths/minute, with flaring of alae nasi and no cyanosis. He had wheezing with symmetrical chest expansion and his trachea deviated to the left with reduced air entry on the right and rhonchi on auscultation. The results of his cardiovascular and abdominal examinations were unremarkable. His neck had no visible swelling nor was a mass palpable.\nThe chest X-ray done at the district hospital after no improvement on anti-TB medication showed a widened mediastinum without features of PTB (Fig. ). His hemogram and erythrocyte sedimentation rate were normal. A chest computed tomography (CT) scan was done upon admission to our unit. It showed a cystic mass in the superior mediastinum. It appeared to be arising from the neck at the level of the thyroid cartilage deep to the sternocleidomastoid muscle and extending into the mediastinum to the level of the carina. The mass displaced the major neck vessels (Fig. ). The lung parenchyma and the heart were reported as normal, with no pleural effusion. The radiologist reported a differential diagnosis of branchial cleft cyst and thymoma.\nOur patient was prepared for surgery after the basic preoperative laboratory tests i.e. total blood count, urea electrolytes and creatinine were done and found to be normal. Median sternotomy was performed combined with neck dissection. Intraoperative findings included a right cystic mass arising from the angle of the right jaw extending into the superior mediastinum; there was no extension into the anterior mediastinum. The right common carotid artery was displaced anterolaterally, the superior vena cava anteriorly. The cyst was dissected from the surrounding vessels and the other adjacent structures within its vicinity in the chest. Extension into the neck was noted as the cyst stalk and excised en bloc. The stalk ended abruptly at the angle of the jaw. Its end could not be traced into the pharynx due to the above finding. Postoperatively, our patient was admitted in our intensive care unit from where he was discharged to the general ward after 2 days. He was discharged from the hospital 10 days after the surgery following an uneventful postoperative course. Histopathology of the specimen reported a fibrous cyst wall lined with low columnar and cuboidal cells. These features are consistent with a branchial cyst.\nOur patient was seen 2 weeks after discharge in our clinic and all his symptoms had cleared. |
A 62-year-old Chinese man was admitted to hospital on July 25, 1985, because of recurrent palpitations and shortness of breath. On examination, there was an increased respiratory rate. A raised jugular venous pressure was noted, together with hepatomegaly and marked peripheral edema. His pulse was 94 beats per minute and regular. Blood pressure was 90/10 mmHg. The apex beat was displaced laterally. Auscultation revealed a grade IV/IV systolic murmur, a harsh low-pitched diastolic murmur at the apex and fine rales at the lung bases. An electrocardiogram showed sinus rhythm. A chest radiograph revealed cardiomegaly and pulmonary venous congestion. Serum potassium, sodium, calcium and magnesium were normal. The patient was diagnosed as congestive heart failure, rheumatic heart disease: mitral stenosis and insufficiency, and was treated with inhalation of oxygen, vasodilator, diuretics and antibiotics. Two days later, his condition improved. On the third hospital day, the patient's palpitation recurred. Heart rate was 180 beats per min. The electrocardiogram revealed ventricular tachycardia. After administration of 100 mg of lidocaine in 40 ml of 25% glucose intravenously, the ventricular tachycardia was reverted to sinus rhythm. Two hours later, the ventricular tachycardia recurred (Figure ). Heart rate increased up to 200 beats per min. Even though, 100 mg of lidocaine in 40 ml of 25% glucose being intravenously administered for four times at interval of 10 minutes, it failed to convert ventricular tachycardia to sinus rhythm. Blood pressure dropped to zero. Intravenous infusion of dopamine and normal saline were administered to maintain blood pressure because no other antiarrhythmic agents and defibrillator were available in the hospital. After blood pressure had returned to 90-100/50-60 mmHg, ventricular tachycardia persisted. Twelve and half hours later, the patient suddenly felt cold and chill, the intravenous infusion was immediately stopped, and 25 mg promethazine and 5 mg dexamethasone were intramuscularly injected at once. Thirty minutes later, the symptom was released. Heart rate reduced to 88 beats per min and the electrocardiogram revealed sinus rhythm.\nPyrogens were detected in the solutions, which caused the reaction in the patients. |
A 10-year-old girl visited our affiliated hospital, complaining of pudendal deformity. The patient was born at 39 weeks of gestation by normal delivery as the second child, and the birth weight was 3,144 g. There was no disorder in the course of pregnancy in her mother. Intake of androgenic medicine or the disorders of ovaries and uterus was not observed by a periodic medical examination. No pudendal deformity was clearly observed at birth, but lateral asymmetry of the pudendal region was noticed at about 4 years old. A child care worker pointed out that she pressed her heel to the crotch while sitting on her folded legs. After 5 years old, her mother confirmed that her clitoral hood clearly hypertrophied. After entering primary school, a teacher in charge pointed out that she pressed her crotch to a chair or bar, and the mother told her to stop it, but she repeated this behavior every day. When she strongly wanted to be absent from an overnight school trip at 9 years old, the mother brought her to the Pediatric Department. Various tests were performed suspecting disorders of sex development (DSD). On the first visit, the height was 132 cm and the body weight was 26 kg. The clitoral hood was enlarged. The appearance was similar to the vulva in children with congenital adrenal hyperplasia, and the clitoris size was 8 x 5 mm. Labial fusion or adhesion was not detected, and the urinary tract and vagina were open at the normal positions. No masculinization, such as acne and polytrichosis, was noted (). Intake of androgenic medicine or the disorders of prepuce was not observed. In the blood test, the sex chromosome was 46, XX. The blood count, blood chemistry, and hormonal test were normal (). On abdominal ultrasonography, the uterus and ovaries were present. Abdominal CT and MRI examinations showed no tumorous lesion.\nBased on the above examination and test findings, DSD was considered negative. The patient was diagnosed with clitoral hood enlargement and referred to our department to undergo clitoral hood reduction. For surgery, a longitudinal incision was designed for the dorsal side in order to resect the clitoral hood by cut and try. In the clitoral region, the clitoral hood and corpus cavernosum were dissected through an inverted V-shape incision. The volume of the exposed corpus cavernosum clitoridis was reduced while conserving the neurovascular bundle, following the Marberger method []. The clitoral hood was resected into a triangle shape and used for labial formation. On histopathological examination, lymphedema and venous tasis in a grade consistent with the influence of foreskin excision were observed. No abnormality was noted in the corpus cavernosum. Her postoperative course was uneventful. As of 10 months after surgery, favorable improvement of the appearance was noted (). |
The patient was a boy aged 11 years and 11 months and was admitted to our hospital due to abdominal pain and headache. His abdominal pain was localized to the right lower abdomen and was defined as paroxysmal and unbearable. He also had sporadic oral ulcers but no fever, rash, joint swelling, or joint pain. He underwent appendectomy due to appendicitis in the initial stage of abdominal pain. He also underwent surgery to remove his perianal abscess. He was the only child in his family, and his parents were healthy and had no similar medical history.\nUnfortunately, his abdominal pain and oral ulcers persisted. He was admitted to the hospital again and underwent electronic colonoscopy () and pathological examination (), whereby Crohn's disease (CD) was diagnosed. His treatment course included mesalazine, infliximab, and briefly thalidomide. During the course of the treatment, he developed an anal fistula and continued to have abdominal pain.\nOne week before admission, he developed paroxysmal headache, without convulsions, blurry vision, tinnitus, weak limbs, vomiting, abdominal pain, diarrhea, fever, and other manifestations of NBD. The supplementary medical history revealed that he was diagnosed with uveitis in the right eye and received intraocular lens implantation in another hospital 3 years ago due to blurred and decreased vision in the right eye. Thus, the total duration of the disease was estimated to be 3 years.\nPhysical examination indicated that he had a splitting headache without any other positive signs, such as the Babinski sign, stiff-neck, and other neurological manifestations. The ocular fundus revealed that the cornea of the right eye was deformed. Nevertheless, the anterior chamber was still clear, the crystal membrane was white and cloudy, the intraocular lens was normal, the optic papilla was pale, and the macular area was yellow and disordered (C/D = 0.7) ().\nLaboratory tests revealed increased C-reactive–protein level, white–blood-cell number, and erythrocyte sedimentation rate. The count of Th1 and Th2 helper T-cell subsets indicated that IL-6, IL-2, IL-4, and IFN-γ levels were elevated (). Results from tests for hepatic, renal, and coagulation functions were normal. There was no abnormality in cerebrospinal fluid.\nThe MRI and MRV of the brain showed many filling defects in the superior sagittal, left sigmoid, and bilateral transverse sinuses, later diagnosed as cerebral venous sinus thrombosis (CVST). No abnormal signal shadow was observed in the brain parenchyma, ventricle, brainstem, or cerebellum (). |
A 44-year-old Japanese man presented to the Maxillofacial Surgery Clinic at the Tokyo Medical and Dental University, Tokyo, Japan, with reports of a hard mass and dull pain in the left mandible. His medical history was generally unremarkable, though his mandible had been accidentally smashed against his young son’s head at the age of 40. No fracture was detected at the time, and no treatment was received.\nClinical examination revealed slight swelling in the left lower molar region and swelling of the left mandible. There was no disturbance of sensitivity in the left lower lip or chin, and he could move his lips normally. Radiographic examination showed large, irregular, multilocular radiolucency of the left area of mandible extending to the premolar and molar area, with no evidence of any impacted tooth (Fig. ). A computed tomography (CT) image revealed a 45 × 30 × 30 mm multilocular cystic mass in the mandible (Fig. ). An axial CT image showed thinning or partly resorption site of the buccal cortical plate. The coronal section images of the CT scan showed the mass lesion displacing the mandibular canal downward, near the inferior border. The roots of the adjacent teeth were resorbed, changing their shapes (Fig. ). The location of the teeth was not changed.\nThe initial clinical impression was an ameloblastoma, myxoma, keratocystic odontogenic tumor or another tumor type lesion.\nThe lesion was removed surgically with the buccal cortical bone and buccal mucosa in contact with the mass of the tumor. The mass fully excised intraorally under general anesthesia, and the inferior alveolar nerve in contact with the mass was preserved.\nThe resected specimen of the primary tumor was a soft, fragile, yellowish-white mass. Microscopically, tumor tissues were composed of plexiform or cord-like nests of tumor cells with round to oval nuclei and eosinophilic cytoplasm in a matrix with prominent myxoid change (Fig. ). On the other hand, the component of solid sheets of tumor cells was limited (Fig. ). In some areas, tumor cells surrounded small blood vessels (Fig. ). Moreover, tumor cells manifested a uniform cell morphology, poor dysplasia, and inconspicuous mitosis.\nImmunohistochemical staining was positive for vimentin (Fig. ), muscle-specific actin/HHF35 (Fig. ), and Calponin (Fig. ), a protein responsible for binding the actin-binding protein. A hairline-shaped area of positive staining for type IV collagen surrounding the tumor cells was also observed (Fig. ). VEGF and D2–40 was also focally positive in tumor cells. In contrast, staining for alpha-SMA, cytokeratin (AE1/AE3), cytokeratin (CAM5.2), CK19, CD34, CD68, p63, S-100, Factor VIII, and desmin, CD56, chromogranin A, synaptophysin was all negative in the tumor cells. Moreover, CD31 was also negative, but we observed many blood cells intervened between tumor nests. The Ki-67 labeling index was almost 1%. No necrosis or tumor invasion into the neurovascular channel was observed. The primary tumor was diagnosed as a glomus tumor based on these findings.\nThe patient remained symptom-free and manifested no signs of recurrence. However, a recurrent tumor was detected in a panoramic radiograph during an 8-year follow-up. The panoramic radiographs taken earlier, after excision of the primary tumor, showed normal healing process, bone regrowth, and increased radiopacity. The follow-up panoramic radiograph 8 years later depicted the recurrence as a radiolucent expansion in the lower area of the mandible. A CT exam showed an expanding lesion exiting the lower site of the mandible and a thinning buccal cortical bone in contact with the tumor (Fig. , ). In magnetic resonance imaging (MRI), the recurrent lesion showed a lower or compatible signal intensity compared to the muscle in T1-weighted images and a low-to-high inhomogeneous signal intensity in T2-weighted images. The apparent diffusion coefficient in the glomus tumor area was 2.0 × 10− 3 mm2/sec, suggesting a low cellular density (Fig. -).\nThe recurrent tumor mass and related teeth were removed under general anesthesia, and the bone surface was shaved. The recurrent tumor had the same microscopic, morphologic, and immunohistochemical features as the primary tumor (Fig. ). The tumor invaded the medullary cavity of the mandible, involving spongiosa and resorbing compressively the cortex bone around tumor.\nThe patient has no symptoms of recurrence as of this writing, 2 years after the second operation, and will be followed for the long term to promptly detect any signs of new tumor growth.\nThe patient is satisfied that the glomus tumor was regulated without resecting the mandibular region. He is pleased that there was no functional deterioration that he was worried about before treatment and there was no aesthetic damage.\nTimeline of patient diagnosis and treatment.\n1. 4 years before the first visit, the patient had been smashed against his young son’s head.\n2. At first visit, the symptom was slight swelling of the left mandible. There was no disturbance of sensitivity. The examinations were performed with X-lay and CT.\n3. First surgery was performed and started clinical follow-up after the surgery.\n4. 8 years later from first surgery, the recurrence was found by panoramic radiograph. The examinations were performed with CT and MR.\n5. Second surgery was performed, and it was passed 2 years with no recurrence from second surgery. |
Patient 1 is an active 59-year-old man with a body mass index (BMI) of 34. His femur showed Dorr A bone type and a high canal-flare index of 5.8 (Figure ). He underwent uncomplicated primary DA THA with a size 10 Corail collared stem with a high-offset neck and a 36 mm +5 mm head. Postoperative radiographs showed neutral coronal and sagittal alignment of the stem and a canal fill ratio of 0.91 (Figure ). He progressed rapidly in the early recovery period. He was walking 5 miles a day and back to full-time work four weeks following surgery. At eight weeks postoperatively, he reported new symptoms of thigh pain, swelling, and weakness. Radiographs showed development of 3-5 mm thick RLLs in zone 1 coronally and zones 8 and 14 laterally (Figure ). Despite conservative measures including a cane and activity restriction, his symptoms continued to escalate, and he was ultimately revised to an S-ROM 16 x 11 x 36 + 6 stem through a posterior approach (Figure ). The modular fixed bearing liner was revised to a dual-mobility construct for enhanced stability. Postoperatively, the patient was placed on restricted weight bearing with limited activity. He returned to work part time after four weeks and resumed full weight bearing and full-time work eight weeks following revision.\nOf note, this patient recovered well and returned to our office for treatment of advanced arthritis in his opposite hip. After careful consideration of stem options and surgical approaches, we proceeded with a Corail stem through a DA approach. However, for this hip we reamed the distal canal in an effort to get improved metaphyseal fixation with a larger stem. By widening the diaphysis, we were able to increase the stem size from a 10 to a 12. A KLA collared stem with a +1.5 head was used to restore leg length and offset (Figure ). Postoperatively, the patient was allowed to progress to full weight bearing with activity restriction. He was back to work full time without activity restriction at eight weeks. At one year following surgery, the patient continues to do well. |
An 11-year-old girl presented with painless swelling in left frontoparietal region of scalp from 8 months and progressively increasing in size. The mass was detected incidentally when she had a fall. There was no history of headache, vomiting, fever, and seizure or any focal neurological deficit. Local examination revealed a hard mass of 50 × 48 mm in size with normal overlying skin. She has no sensory and motor deficit. Her general and systemic examination was normal. Blood investigations were normal. CT scan shows a well-defined lytic lesion in left frontoparietal bone. On MRI, a lytic lesion in left frontoparietal calvarium was seen with sun ray periosteal reaction, measuring 60 × 53 mm in size with a subgaleal and epidural component causing bulking of parenchyma (). The patient was operated and excision of left frontal tumor with reconstruction of skull was done. Intraoperative frozen section revealed monomorphic small round cells arranged in clusters and scattered singly. Diagnosis of a malignant round cell tumor was made.\nThe tumor was sent for histopathological examination. Grossly, the specimen consists of one large grayish brown soft tissue attached to a flat bony fragment measuring 7.0 × 7.0 × 4.5 cms. External surface of the soft tissue was smooth and partially encapsulated. Cut surface of the soft tissue was gray white to yellowish gelatinous and showed few hemorrhagic areas also (). On microscopic examination, section revealed a monomorphic round cell tumor arranged in lobular, trabecular, and micro- and macrofollicular pattern with eosinophilic secretion in the lumen (). Tumor cells had round nuclei with stippled chromatin, prominent nucleoli, and thick nuclear membrane. Cytoplasm was moderate in amount and vacuolated. Connective tissue septae with fine blood vessels were seen throughout the tumor. Mitosis was 0–2/hpf. There was also infiltration of tumor cells in the surrounding fibroadipose tissue. Section examined from bone showed bony trabeculae and bone marrow revealing marked fibrosis and infiltration by tumor cells. On Periodic Acid Schiff (PAS) stain, tumor cells were negative. On immunohistochemistry, tumor cells were immunopositive for MIC-2. Keeping in view the morphological and immunohistochemical profile, a final diagnosis of Ewing's sarcoma of bone was made. |
The patient referred for invasive coronary angiography was a 42-year-old male, active duty US Army soldier, without significant cardiac history, admitted to Brooke Army Medical Center with chest pain. On the day of admission, the patient noted severe substernal chest pain along with lightheadedness, flushing, and nausea. Symptoms persisted for approximately 45 minutes and resolved spontaneously soon following presentation. Electrocardiogram obtained on arrival revealed diffuse ST segment depressions with an initial serum troponin T reported at 0.02 ng/mL. The patient was hemodynamically stable and free from chest pain at the time of admission. The patient was admitted to the Cardiology service for management of his NSTEMI, with plans to pursue an early invasive strategy. The patient was given aspirin 325 mg orally, clopidogrel 600 mg orally, and atorvastatin 80 mg orally and a continuous heparin infusion was initiated. Troponin T peaked at 0.42 ng/mL twelve hours following admission.\nThe following morning, the patient was sent for left heart catheterization with coronary angiography with arterial access via the right radial artery. The patient was normotensive and in normal sinus rhythm on arrival to the catheterization laboratory. Midazolam 1 mg and fentanyl 25 mcg were administered intravenously, with appropriate conscious sedation achieved. After standard preparation of the site and subcutaneous infusion of 3 cc of 1% lidocaine, arterial access was obtained without difficulty via modified Seldinger technique and a hydrophilic Terumo 6 Fr Glidesheath Slender was advanced into the vessel followed by administration of intra-arterial verapamil 2.5 mg and nitroglycerin 400 mcg. Continuous intravenous heparin drip was also started prior to initiation of the procedure. A 5 Fr pigtail catheter was advanced into the left ventricle over a J-wire in the standard fashion for evaluation of LVEDP prior to ventriculography.\nImmediately following catheter placement, the patient experienced probable acute radial artery spasm, preventing either antegrade or retrograde movement of the catheter. Manipulation of the catheter was discontinued for 5–10 minutes to allow for spontaneous resolution of the spasm; however, catheter entrapment persisted. Additional conscious sedation with fentanyl 50 mcg and midazolam 1 mg was administered intravenously, warm compresses were applied, and supplemental intra-arterial infusions of nitroglycerin and verapamil were provided, again without resolution of catheter entrapment. Escalation of vasodilator therapy was then pursued with initiation of a nicardipine infusion at a rate of 12.5 mg/hour. After sufficient time was expended for medication effect to occur, attempt at catheter removal was again unsuccessful. Local subcutaneous administration of additional lidocaine and nitroglycerin was ineffective as well. Right femoral access was then obtained and the procedure resumed, revealing normal coronary angiography and left ventricular hemodynamics ().\nWith multiple traditional interventions for relief of radial spasm exhausted, consultation with anesthesiology was pursued to assist with escalation of sedation. The patient was converted by the anesthesia team to general sedation, first with propofol infusion followed by a dexmedetomidine infusion. Catheter entrapment nonetheless persisted. At this time, surgical consultation with vascular surgery and cardiothoracic surgery was obtained, as surgical intervention appeared a distinct possibility for catheter removal.\nA final attempt at nonsurgical intervention was collectively decided upon, with suggestion made to attempt regional axillary nerve block as a means of reducing sympathetic tone attributing to the severe vasoconstriction. Under ultrasound guidance, 40 cc of 1.5% mepivacaine without epinephrine was infused slowly in the location of the right axillary nerve. Right upper extremity venous engorgement and vasodilation were appreciated on ultrasound during infusion. Approximately seven minutes after completion of the infusion, gentle traction was placed on the radial catheter, resulting in atraumatic removal of both the sheath and catheter.\nThe patient was monitored as an inpatient for 24 hours. He regained full function of his right arm without notable neurovascular deficiency. He has been followed up routinely as an outpatient without development of such deficiencies. |
In March 2012, a caucasian 63-year-old man was seen in our institution for a painful swelling of the right knee joint present since 2 years. Pain was exacerbated by digital pressure. This patient resulted affected by D-TGCT that had been surgically treated with multiple synovectomies in 1987, 1990 and 1996, respectively. Histologic review of the original specimens of the primary tumor and of all recurrences was performed. All the specimens exhibited overlapping morphology by showing a combination of hyperplastic synovia, a mononuclear cell proliferation organized in a nodular pattern of growth, scattered hemosiderin-laden macrophages, xanthoma cells and multinucleated giant cells. These findings met the diagnostic criteria for D-TGCT and we therefore confirmed the primary diagnosis. Because of pain and persistent joint effusion, the patient underwent another synovectomy at our institution, and the histologic evaluation of the specimen confirmed the diagnosis of D-TGCT without atypical features. In contrast with previous samples a greater amount of fibrosis was seen.\nTwo years later the patient presented again with painful swelling of the right knee associated with pain localized in the right hip. Loss of weight and functional limitation of the right leg was observed. Magnetic nuclear resonance imaging demonstrated extra-articular soft tissue multilobular masses around the posterior part of the knee joint, with erosion of the lateral femoral condyle. Extension into the patello-femoral joint and the proximal tibia with a low signal intensity on T2-weighted image was present. In T1-weighted images, dark signal nodules, compatible with hemosiderin deposition, were readily identified (Figure a). Clinical examination showed recurrent tumour in the knee whereas computed tomography imaging revealed pelvic lymph nodes enlargement. In consideration of the extensive destruction with consequent functional compromise of the knee joint the patient underwent an “above the knee” amputation. During surgical procedure frozen examination of one inguinal lymph node was performed that showed a sub-capsular proliferation of mononucleated cells, associated with multinucleated giant cells and siderophages consistent with lymph node seeding of D-TGCT (Figure a, b).\nMacroscopically, the evaluation of the knee disarticulation revealed a multinodular lesion of 30 cm × 25 cm × 21 cm, that was extensively infiltrating both the leg and the thigh with massive destruction of the knee joint. Alternation of white, yellowish and brownish areas with hematic-cystic lesions was seen (Figure b). Histological analysis of the lesion showed the same morphological features as the previous samples and confirmed the diagnosis of D-TGCT. In particular, the lesion showed synovial-like mononuclear cells lacking significant cytologic atypia, admixed with multinucleated giant cells (Figure c, d). Foci of ischemic necrosis were also evident. The mitotic rate was lower than two figures per 10 high power field. Ki67 proliferative index did not exceed 5% of neoplastic cells.\nNo areas of malignant transformation were identified in the specimen. Four months later a CT scan of the pelvis revealed a mass located in the soft tissue of the contralateral buttock, associated with inguinal lymph nodes swelling without evidence of lung metastases. In January 2015, the patient started systemic treatment with imatinib. |
A 65-year-old lady with a past medical history significant for chronic obstructive pulmonary disease (COPD), hypertension, and hypothyroidism presented to our hospital for evaluation of dizziness. The patient was recently discharged from our hospital after being treated for COPD exacerbation. A computed tomography (CT) scan of her chest done at that time had revealed new-onset mediastinal lymphadenopathy and the presence of a moderate pericardial effusion (Figure ). She had undergone bronchoscopy and biopsy of the mediastinal mass and was being followed by pulmonology and cardiology as an outpatient.\nUpon presentation this time, the patient complained of dizziness for a week, which was worse on standing. She also described multiple near-fainting spells. She denied other neurological symptoms. She also denied chest pain, dyspnea, palpitations, or tinnitus. Her vital signs on presentation were within normal limits, and her examination did not reveal any positive findings. Initial lab tests including blood counts, metabolic profile, thyroid-stimulating hormone, cardiac enzymes, arterial blood gas, and electrocardiogram were unremarkable. Imaging tests including chest X-ray and CT scan of the head also did not reveal any acute abnormalities. Her orthostatic vitals were significantly positive. Given her recent diagnosis of pericardial effusion and history suggestive of presyncope, a limited echo was ordered to assess any increase in the size of the effusion. The repeat echo revealed that the effusion had significantly increased in size (Figure ), along with findings suggestive of pre-tamponade physiology.\nInterventional cardiology was consulted, and the patient was taken to the cardiac catheterization lab for immediate pericardiocentesis under fluoroscopy guidance. A subxiphoid approach was used, and approximately 600 mL of bloody fluid was drained from the pericardial space. A catheter was also left in place to drain overnight. Overnight, the patient started becoming progressively short of breath and hypoxic. She was given breathing treatments and placed on 6 L supplemental oxygen via a nasal cannula. She subsequently became hypotensive, pale, and tachycardic, and there was a sudden gush of around 200 cc blood in the pericardial drain. The patient was given IV fluid boluses and started on norepinephrine infusion. Around 1.5 L of hemorrhagic fluid was drained via the pericardial catheter, but she remained hypotensive. A bedside echo showed a large pericardial effusion (Figure ) with near collapse of the ventricles signifying pericardial tamponade.\nWe suspected a cardiac perforation, and she was immediately taken to the operating room (OR) for mediastinal exploration and control of bleeding. In the OR, it was revealed that she had transection of the distal portion of the LIMA, which was causing the bleeding. The pericardial sac was also opened revealing a large amount of blood that was drained. The accompanying cardiac structures such as the right and left ventricles, the left and right atriums, the pulmonary veins, and ascending aorta were inspected and no obvious damage was noted. The LIMA was clipped and ligated at several points to control the bleeding, following which her blood pressure improved and we were able to wean her off norepinephrine. The patient had an uneventful rest of the hospital stay and was soon discharged to a skilled nursing facility. |
A 60-year-old woman had been followed elsewhere for over 5 years for an incidentally found lipoma at the craniocervical region. She was then referred to us due to progressing motor weakness in her right extremities, associated with paresthesia for 3 months. Her neurological examination revealed mild hemiparesis in the right extremities and disturbance of deep perception. Bladder and bowel function were normal. Computed tomography (CT) scans showed a low-density mass compressing the dorsal aspect of the medulla and the spinal cord (Fig. ). There was no abnormality of the vertebral bony components (Fig. ). The mass showed a high-intensity signal on T1-weighted magnetic resonance imaging (MRI) (Fig. ), which was consistent with lipoma by fat suppression sequence. This lipoma was detected 5 years ago; however, the previous MRIs at the initial hospital were not available. Surgery was planned to decompress the medulla and the C1–C2 spinal cord by opening of the foramen magnum, performing a laminectomy of the C1 arch and debulking the lipoma.\nThe patient was placed in the prone position under general anesthesia. Motor evoked potentials were monitored during the surgery. A suboccipital midline skin incision was made from the inion to the C3 level. A standard suboccipital craniotomy with opening of the foramen magnum and a C1 laminectomy were performed. The dura mater of the posterior fossa and the spinal cord looked normal. Opening of the dura mater revealed a yellowish mass covered by the arachnoid membrane, indicating a lipoma (Fig. ). The mass was dissected from the cerebellar tonsils and the medulla at the upper pole, then it was partially removed (Fig. ). It was difficult to establish a dissection plane on the lateral and inferior aspects of the medulla and the spinal cord (Fig. ). Internal decompression was performed with an ultrasound aspirator for the main mass towards the caudal pole. The mass was hemorrhagic, which could be controlled by bipolar coagulation. The incised arachnoid membrane was sutured to avoid later adherent tethering of the cord (Fig. ). An expansile duraplasty was not performed as sufficient subdural space was obtained after the decompression. Histological examination confirmed that the mass consisted of mature adipocytes, confirming a benign lipoma. The postoperative course was uneventful. No neurological deterioration was noted. The right hemiparesis and deep perception improved within a month after surgery. The postoperative CT scans showed adequate decompression of the medulla and the spinal cord (Fig. ). A follow-up MRI taken 12 months after the surgery showed no recurrence or regrowth of the lipoma (Fig. ). Her neurological deficit subsided completely at the follow-up examination. |
A 64-year-old man, who had undergone laryngectomy to treat laryngeal cancer 12 years ago, was referred to our clinic with severe pain at right inguinal region preventing him from walking. He did not report any radiation of his pain to other areas. The pain was progressive and started from 1 year ago. It was exacerbated by walking. He mentioned that his pain was not alleviated with rest and does not allow him to sleep. He had been evaluated by different specialists. He had taken many analgesics; however, none of them had relieved his chief complaint. His family and social history were insignificant. During physical examination, he walked with difficulty. In supine position, he tended to keep his right hip in 30 degrees of hip flexion. His right hip extension in 0-30 flexion was painful. His right knee range of motion was normal. No sign of scar, mass, erythema, or tenderness was detected in his right inguinal area. No tenderness was detected palpating his right sacroiliac joints, posterior superior iliac spine, iliac crest, anterior superior iliac spine, or symphysis pubis. Meanwhile, during spine examination, tenderness on the patient's spine at thoracolumbar junction was noted. His previous workup included pelvic X-ray (AP), right hip X-ray, and MRI, all of which were insignificant. Due to his spinal tenderness, a lumbosacral plain radiography, lumbosacral computed tomography (CT) [Figure ], and magnetic resonance imaging (MRI) [Figure ] scan were performed. On spine plain X-ray, the right pedicle at the level of L1 was not visible (winking owl sign). CT scan revealed a lytic lesion in the posterior half of the L1 vertebral body and the L1 right pedicle. The lesion involved the inferior body cortex. On MRI, the lesion turned out to be a mass in the posterior L1 body and its right pedicle obliterating the right intervertebral foramen. It was hyposignal on T1 and hypersignal on T2. On bone scan, foci of hyper absorption on several of his ribs and lumbar vertebrae were present.\nAs a result, our patient was managed with L1 vertebral laminectomy and root decompression. The mass obliterated the right L1 intervertebral foramen. Thus, we had to excise his right L1 pedicle and right L1 superior facet in order to visualize the right L1 intervertebral foramen and decompress the right L1 root through its course. The spinal lesion was biopsied and sent for pathology. As the L1 vertebrae were unstable after excising its right pedicle and superior facet, posterior instrumentation was performed to enhance the spinal stability [Figure ]. Interestingly, the day after surgery inguinal pain was relieved significantly, and the patient succeeded to go out of bed with a thoracolumbosacral orthosis (TLSO). The pathology reported metastatic adenocarcinoma showing papillary configuration with pulmonary origin. Our patient was referred to a radiotherapist and oncologist for further radiotherapy/chemotherapy treatment. After referring him to the oncologist, it was revealed that he had also foci of lung metastases and managed with chemotherapy. He visited our clinic regularly for follow-up at 2 weeks, 6 weeks, 12 weeks, 24 weeks, and 1 year postoperatively. After 1 year postoperatively, our patient ambulated independently, and his inguinal pain was relieved and had no operative complication. |
A 64-year-old woman was admitted to our hospital with a pulsatile mass swelling over the left cervical region and right hemiparesis after cough for 1 day. She had a history of fibromatosis, but no previous history of trauma, operation or inflammation in this region. On physical examination, a 5-cm large pulsatile swelling with blowing bruit was found over the left cervical region and multiple fibromas were found in regions of face, neck, and trunk. Neurological examination revealed muscular strength of grade IV according to the ‘manual muscle test (MMT)' grading system and hypoesthesia of right limbs, including touch and pain sensation. Computed tomography angiography (CTA) and DSA revealed a left high-flow internal carotid-jugular fistula at the first cervical level and twisted left internal carotid artery (ICA) (Figures ). Blood flow from the vertebrobasilar artery and right ICA via the circle of Willis supplied the left anterior cerebral artery and middle cerebral artery and drained backwards into the petrosal segment of left ICA (Figures ).\nWe chose embolization of both the fistula and parental artery, because no appropriate covered stent could be used to pack the fistula in the condition of reservation of left ICA. The purpose of first endovascular treatment was to isolate the fistula, by blocking both the backward blood flow from right ICA and the forward blood flow from left ICA. A Headway-21 stent catheter was selectively inserted into the distal part of fistula in the segment of carotid cavernous sinus, meanwhile an Echelon-10 microcatheter was placed in the distal part of stent catheter. One LVIS 5.5*30 mm stent, with its characteristic relatively compact mesh, was used to cover the distal part of fistula to avoid coils being pushed into internal jugular vein and heart (Figure ). However, the 3.5 cm fistula could not be covered by the LVIS stent. Therefore, 10 coils were additionally used one by one, to block the fistula from the distal to proximal part through an Echelon-10 microcatheter (Figure ). When considering the fast-backward blood flow from the right ICA after embolization, we could not guarantee the safety of blocking by Onyx. We chose one detachable balloon to block the ICA near the proximal part of fistula to stop the forward blood flow, and the backward blood flow would be stopped in the second treatment (Figures ).\nThe blowing bruit was reduced significantly after the first treatment, but became worse after 2 months later. The patient was admitted to our hospital again, and DSA revealed residual blood flow in the fistula from the left ICA and premature balloon deflation (Figures ). It was fortunate that LVIS stent blocked the balloon into the internal jugular vein. An additional 13 coils were used to pack the fistula with double-microcatheter techniques, under the multi-angle DSA projection, to avoid the coil protruding into the internal jugular vein (Figures ). Afterwards, two detachable balloons were used to block the proximal part of left ICA again (Figure ). DSA revealed the forward blood flow disappeared, the low-flow backward blood from right ICA still supplied the fistula, and a normal ipsilateral jugular vein (Figure ). Because the fistula was mostly blocked, and the low-flow backward blood may promote the formation of thrombosis in the distal part of fistula, we stopped the second endovascular treatment and planed a DSA examination to evaluate the effect of embolization and to determine further treatment. After this treatment, the patient did not feel the blowing bruit or any other discomfort.\nHowever, the patient complained of swelling and pain in the left occipitocervical region 2 days later. Physical examination revealed a large subcutaneous mass, with volatility and severe tenderness. Emergent CT revealed a subcutaneous hematoma in left occipitocervical region, without abnormalities in brain (Figures ). DSA in the hybrid operation room showed the coils in the fistula were stable without no forward blood flow in the proximal part of fistula. However, there was still a little backward blood flow through the right ICA and vertebrobasilar artery into right internal jugular vein, via the distal part of fistula. The lower part of fistula was not shown, because the blood drained into the right internal jugular vein through the sigmoid and transverse sinus reversely (Figures ). It was suggested that the occipitocervical subcutaneous hemorrhage was caused by poor ipsilateral jugular drainage, which was the result of the formation of thrombosis in fistula extending to the left internal jugular vein, thus blocking forward and backward reflow. We inserted an Echelon microcatheter through the left vertebrobasilar artery-posterior communicating artery, then selectively to petrosal segment of the left ICA, where 3 coils were put in, and then a 1.2 ml Onyx-18 was injected slowly. DSA examination after this procedure revealed that the backward blood flow distal to the fistula disappeared (Figures ). An open operation to remove the hematoma was preformed after the interventional operation. Multiple capillary hemorrhages were found from behind the sternocleidomastoid muscle after removing clots. The operation to stop bleeding was successful, and the postoperative course was uneventful. A 6-month follow-up DSA demonstrated a totally occluded fistula allowing the patient to engage in light manual labor with normal neurological functioning after the operation (Figures ). |
A 1-day-old boy, born of a full term normal vaginal delivery, was referred to us with a defect in lower anterior abdominal wall. The boy had a low lying umbilicus with an exposed bladder plate that was getting soaked with urine, which was being passed in intermittent squirts from a tiny orifice on the bladder plate. He had a well formed phallus and was also passing clear urine per-urethrally in good stream. On insertion of a catheter per-urethrally, the tip of the tube was not seen on the exposed bladder plate. There was no pubic diastasis clinically and on pelvic radiograph (Fig. 1). Hernial orifices, testis and anus were normal. The baby also had polydactyly and thirteen ribs on chest radiograph. Clinically, the differential diagnoses were superior vesical fissure or duplicate exstrophy (antero-posterior). However, the fact that the per-urethral catheter did not come out through the bladder plate went against the diagnosis of superior vesical fissure and a wet bladder plate went against the diagnosis of duplicate exstrophy. A renal ultrasound showed bilateral kidneys normal with a well distended bladder within. Thus a diagnosis of duplicate exstrophy was made. However, there was still a doubt whether one ureter was opening onto the exposed bladder plate.\nThe patient was prepared for cystourethroscopy and surgery. On cystoscopy, there was a smooth walled good capacity bladder showing both ureteric orifices ectopically placed, very close to the bladder neck. The left orifice was oval and gaping. On surgical exploration, the visible bladder mucosa on the surface of the abdominal wall was separate from an intact bladder deep to it. The median umbilical ligament was going to the umbilicus which was superior to the duplicate bladder plate. The duplicate bladder plate appeared to have a very tiny communication with the intact native bladder but the communication could not be identified clearly during surgical excision of the bladder plate. Both ureters were draining into the intact bladder. The abdomen could be closed easily without much tension. Per-urethral catheter was removed on post-operative day two and child passed urine in good stream. |
An 80-year-old female patient with a prior history of hypertension, hyperlipidaemia, diabetes, and ischaemic heart disease with a prior inferior MI and PCI with drug-eluting stent placement to LCX, 3 years prior to her current admission. She was admitted with respiratory failure due to pulmonary oedema and pneumonia.\nThe patient was conscious, intubated, and ventilated with remarkable tachypnoea, her blood pressure was 80/50 mmHg and her pulse 100 b.p.m. There were decreased lung sound in both bases, heart sounds were irregular and fast, periphery was cold and dry.\nInitial TTE upon admission showed normal size LV with normal global systolic function, severe ischaemic MR (posteriorly directed wall jet), and no stenosis. After a 10-day period with improvement of infectious status, but no improvement in her CHF signs and inability to wean from mechanical ventilation due to continuous pulmonary congestion, a decision was made to perform urgent mitral clipping as salvage therapy. Prior to the procedure, TOE was performed that showed severe MR with a posteriorly directed jet, severe malcoaptation mainly resulting from posterior leaflet immobility, and no evidence of papillary muscle rupture or new VSD. Mitral valve measurements were based on TOE prior to the procedure: average annular diameter was 40 mm and average coaptation surface length was 2.9 mm. An IABP was inserted 3 days prior to the procedure as means of haemodynamic support and aim of altering LV geometry to optimize the chances of success of MC by improving leaflet coaptation surface length and annular diameter. Transoesophageal echocardiography after insertion of IABP showed an average annular diameter of 36 mm and average coaptation surface length was 5.2 mm. Two MCs were implanted (one medially and one laterally to the prominent calcification). Mitral regurgitation improved from severe to moderate and atrial V-wave decreased from 60 to 25 mmHg, with no immediate post-procedural complications.\nFollowing the procedure there was an initial improvement in in her respiratory condition, the IABP was kept in situ to allow better haemodynamic and vulemic support. Unfortunately 7 days after the procedure, she developed ventilator associated pneumonia and sepsis with multiorgan failure, she died 10 days following the procedure. |
A 76-year-old woman with diabetes mellitus visited our hospital for routine follow-up. Physical examination revealed no abnormalities. Except for her glucose metabolism, no abnormalities were observed on routine laboratory examinations, including blood biochemistry for serum tumor markers such as carcinoembryonic antigen and carbohydrate antigen 19-9. A laterally spreading soft lesion was detected in the cecum (Figures –). Observation using colonoscopy showed a small nodule with adhesion of some blood (approximately 5 mm in diameter) and a whitish laterally spreading soft lesion (approximately 25 mm in diameter) (Figures –). On narrow-band imaging (NBI), a pattern with a central dark area surrounded by a clear lighter area, that is, a circular pattern with dots, and an invisible capillary vessel, were observed as hyperplastic features, whereas a central light area surrounded by a dark outer area, that is, a round-oval pattern, was observed as an adenomatous feature in a small nodule (Figures –). Therefore, biopsy samples were obtained from the nodule presenting with an adenomatous feature (), and the lesion was eventually diagnosed as an SSA/P with cytological dysplasia, considering the endoscopic observations including the NBI features. The potential risk of progression to cancer was explained to the patient, because of the SSA/P features, that is, location in a right-side colon, being of large size, and being with cytological dysplasia. Endoscopic submucosal dissection was thought to be feasible but was not performed at the patient's request. After 11 months, follow-up colonoscopy was performed. At the same site in the cecum, an elevated lesion with a central depression was observed, where a small nodule had been identified earlier on the basis of the similar appearance and location of the nodule in both the cancer and SSA/P (). Biopsy samples were obtained from the central depression site, and the elevated lesion was eventually diagnosed as a moderately differentiated adenocarcinoma. Based on these endoscopic findings, an invasive adenocarcinoma arising from the SSA/P was suspected. Endoscopic resection could not be performed and the lesion was removed surgically. The pathological diagnosis was a moderately differentiated adenocarcinoma extending 2700 μm into the submucosal layer with a mucin pool, lymphatic infiltration (Figures and ), in which the most aggressive deep invasion was observed in a central depression (Figures and ), and a remaining SSA/P with/without cytological dysplasia component that was detected at the periphery of the invasive cancer (Figures and ). Neither recurrence nor metastasis of colon cancer has been detected in the patient till now. |
A 3-month-old boy, delivered in eutocic birth, with good height-weight evolution and irrelevant personal and familial past diseases, presented with a complex partial seizure, preceded by a loud scream and ocular infraversion. Spontaneous recovery occurred in few minutes. The patient was admitted in a pediatric emergency room. He was apyretic, hemodynamically stable, eupneic with good peripheral oxygen saturations, flat, and pulsating fontanelle. He had normal blood and urinary laboratory tests. No signs of traumatic brain injury were seen. Fifteen minutes after admission, he suffered another seizure. A postictal period with no reaction to pain, bradycardia, and a hard and bulging fontanelle was noticed. A cranial computed tomography (CT) scan was performed ().\nThe patient was transferred to a pediatric intensive care unit, and treatment with phenytoin 15 mg 12/12 h, mannitol, sedoanalgesia with midazolam, and fentanyl was started. He was intubated and connected to mechanical ventilation. An external ventricular drain was urgently placed through the left external angle of the anterior fontanelle.\nA magnetic resonance imaging (MRI) and angiography (MRA) were performed in the first 24 hours after the hemorrhage (). A suprasellar lesion with 26 mm diameter compatible with a giant aneurysm of the anterior communicating artery complex was documented. A slight filling of the suspected aneurysm and an endoluminal thrombus were visualized.\nA diagnostic cerebral angiography () was done three days after MRI and MRA. No therapeutic endovascular procedure was performed once there was no filling of the suspected aneurysm, at this point. A spontaneous thrombosis was assumed to have occurred. No other lesions were identified.\nThese features were considered to be consistent with an intracranial hemorrhage secondary to the rupture of an anterior communicating artery complex giant aneurysm, with subsequent spontaneous thrombosis.\nOn the 5th day of treatment, assisted ventilation and sedation were suspended and the patient was extubated. A CT scan was repeated 11 days after the initial hemorrhage, which demonstrated persistence of the hydrocephalus. A medium-low pressure ventriculoperitoneal shunt was surgically inserted at that time.\nWith two weeks of treatment the electroencephalogram reported low paroxysmal activity, bilaterally, on the parietal region. Progressive phenytoin suspension and its substitution for carbamazepine were endeavored. The MRA was repeated at this point ().\nAt this early stage a clinically significant improvement was achieved. The child recovered to his baseline neurological condition and had no more seizures. After 26 days of hospitalization, he was discharged from hospital, without neurological deficits.\nThe patient was followed up in neuropediatric and pediatric neurosurgery outpatient clinic. He totally recovered and repeated the MRA when he was 6-month old, which disclosed further volume reduction of the thrombosed aneurysm and good cerebral vascularization (). No neurological symptoms due to mass effect were detected.\nFurther angiographic studies were performed at 6 and 12 months and subsequently with yearly intervals until 5 years after aneurysm rupture. The last MRA showed no recanalization of the anterior communicating artery aneurysm and a permeable right ACA (). At five-year follow-up, the child remains neurologically intact, living without restrictions. Seizures ceased, allowing the suspension of the prescribed medical therapy. |
A 47-year-old male with a past medical history notable for hypertension on metoprolol succinate, morbid obesity, and pre-diabetes presented to the emergency department (ED) with a chief complaint of generalized weakness. The patient tested positive for COVID-19 and exhibited mild unspecified respiratory symptoms. He was subsequently discharged home to recover in isolation per the CDCs COVID-19 response protocols. One week later, the patient returned to the ED for ongoing symptoms and was admitted requiring supplemental oxygen for hypoxia. Upon admission to the hospital, the patient was noted to have difficulty standing and ambulating. Two days into his inpatient stay, the patient developed urinary retention issues requiring intermittent catheterization. Three days later, he developed facial weakness and numbness. The clinical diagnosis of GBS was suspected and subsequent EDX studies reported AIDP. The patient was treated with a five-day course of intravenous immune globulin (IVIG). Three days after the completion of his IVIG treatment, the patient noted improvement with right upper extremity anti-gravity strength.\nThe patient was transferred to inpatient rehabilitation with significant proximal lower extremity weakness. The bilateral upper extremities demonstrated slight weakness. The bilateral lower extremities demonstrated a significant loss of strength, 1/5 dorsiflexion, and 3/5 plantar flexion bilaterally. The patient also reported diminished sensation to light touch in bilateral upper extremities in all dermatomes. His blood pressure upon rehabilitation admission was 110/73 mmHg.\nPrior to hospitalization, the patient was independent with mobility and all activities of daily living. Upon evaluation in the inpatient rehabilitation, the patient was at a significant functional decline from baseline, requiring dependent assistance with toileting hygiene, showering, upper body dressing, lower body dressing, footwear management, rolling left and right, and all transfers. The patient was unable to ambulate due to his level of impairment. In inpatient rehabilitation, the patient completed three hours total of physical, occupational, and speech therapy per day five days a week with exercises aimed to improve balance, mobility, activities of daily living, fine motor skills, cognition, and breath support.\nAfter completing four weeks of inpatient therapy, the patient was independent with supine to sit, upper and lower body dressings, and rolling right and left in bed. He required minimal assistance to lift the left lower extremity into the bed and moderate assistance with wheelchair-to-bed transfers and bed mobility. He was able to stand with the assistance of his spouse and by using significant reliance on bilateral upper extremity support on a walker for stability and offloading lower extremities. He required a power wheelchair for mobility and was unable to transfer in and out of the car thereby requiring a wheelchair van for transportation. Final muscle strength grading was not documented. His blood pressure remained stable throughout his stay on metoprolol succinate and was 121/65 mmHg on discharge. |
Patient 1 was a 38 years-old male. In May 2010, this patient was diagnosed with glioma soon after an episode of seizures. MRI showed intra-axial expansive and infiltrative lesions that were cortical and subcortical, and which affected the anterior half of the right temporal lobe and extending from the pole to the Sylvian fissure superiorly and to the right parahippocampal gyrus, posteriorly, and medially. Partial surgical resection was performed in August 2010 and the first pathologic diagnosis was astrocytoma grade II. He underwent chemotherapy with TMZ at a dose of 2,000 mg with cycles every 28 days for 5 days in the years 2011–2013, with no tumor regrowth until the beginning of 2015. At this time, he underwent MRI, which was used to compare the discrete extension of the signal alteration areas, especially the subinsular regions. In March 2015, he resumed chemotherapy with TMZ at a dose of 100 mg/day and the patient then lost 12 kg of body weight, which was associated with anorexia, insomnia, and depression. In May 2015, he suffered a seizure requiring hospitalization. In June 2015, the patient resumed the old chemotherapy regimen with TMZ (2,000 mg every 28 days for 5 days), and a follow-up with MRI; however, the tumor size continued to increase. In January 2016, the neuro-oncology team decided to discontinue treatment with TMZ considering the risk/benefit and planned a surgical re-approach. This was followed by chemoradiation and lasting 6 cycles of PCV associated with CBD. The CBD dosage was ranging from 300 to 450 mg/day.\nDuring chemoradiation, the patient had an excellent clinical performance, practiced sports and had few symptoms of fatigue and/or nausea.\nAt 1 month after the end of chemoradiation, control MRI (Figure ) was characterized by exacerbation and the ultra-precocious phenomenon of PSD with increased edema and inflammatory disease characterized by extensive areas of contrast enhancement associated with tissue hypoperfusion (not shown). MRI controls demonstrated the progressive reduction of these findings.\nThe result of a pathological study after the first surgery was astrocytoma grade II with Ki67 staining of 5%. After the second surgery, he progressed to GBM grade IV (Figure ), related to increased cellularity, frequent mitosis, presence of micronecrosis, microvascular proliferation/endothelial, Ki67 staining of 30%, and loss of ATRX expression. Biomolecular marker analysis indicated IDH-1 mutated and MGMT methylated. |
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