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Patient n. 2 was a 60-year old female, diagnosed with suffering from POAG for ten years, under treatment with hypotensive drugs and regularly controlled IOP twice a year which appeared maintained within normal ranges. During a control visit in January 2016, the patient had reported irritating symptoms of eye discomfort, described as burning, itchiness, and feeling sand, mainly in her right eye (RE), over the last six months. The situation had been managed with the use of several types of tear substitutes, none of them successful in symptom relief. Symptoms were scored in the RE as OSDI of 74 out of 100 and a VAS score of pain of 85 mm out of 100. In the left eye (LE) the symptoms were defined by the patients as light and acceptable: OSDI score was 22 out of 100 and VAS 21 mm out of 100. Slit lamp evaluation had not shown epithelial damage, and there were no signs of inflammation, but only a reduced Tear Film Break-Up Time (TFBUT) of 4 seconds in RE and 8 seconds in LE had been recorded. A therapy with hyaluronic acid (HA) based tear substitute to be administered 4 times/day in both eyes was prescribed.\nIn March 2016, the patient was proposed to receive in her RE a treatment with topical CBS, as a compassionate unconventional therapy, with the aim to reduce the pain symptoms for which the previous therapeutic attempts had turned to be unsuccessful. The therapy for the contralateral LE was maintained with HA based tear substitutes.\nThe patient signed the specifically designed informed consent and started administration of the CBS eye drops in April 2016, with the posology of 0.4 ml (8 drops) in RE, each day for a total of two months. In the GF dosages determined for the two CBS lots were administered during the first and second month.\nOn September 2016, the patient reported a significant relief from subjective symptoms of discomfort; in RE the OSDI was 28 out of 100, with VAS: 32 mm out of 100. Also in LE a reduction was observed, with OSDI score determined as 16 out of 100 and VAS 15 mm out of 100. During this visit, also the IOP was measured, which resulted in the normal range in both eyes and the analyses of the visual field tests performed since 2006.\nIn the mean deviation (MD) values recorded over several visits performed from December 2006 through September 2016 were graphed. A significant progressive lowering in MD values was observed which was followed by an important amelioration in correspondence with the period of treatment with CBS, in the figure highlighted with the arrow. It has to be noted that the increase in MD values was recorded either in the treated right eye or in the untreated left eye. Moreover, the MD values recorded in September 2016, four months after the end of the CBS eye drop administration, showed in both eyes a further amelioration.\nThe improvement is also demonstrated by the analysis of the PSD shown in . A progressive worsening in PSD values had been observed over ten years, whereas a rapid change was recorded in correspondence with the CBS eye drop treatment, either in the treated right eye or in the untreated left eye.\nIn the central 30-2 visual field tests before ((a): right eye RE; (b): left eye LE), at the end (c, d) and after four months (e, f) from the end of the CBS eye drop treatment were shown. An amelioration of the defect was observed in all the four quadrants in both the treated RE and the untreated LE.\nTaking together these observations, a positive effect also in the left untreated eye could be recorded, which suggests a neural cross-talk mechanism between the eyes. |
A 59-year-old woman presented in July 2017 with extensive bleeding from her ileostomy site. Her history included locally advanced bladder cancer for which she had undergone pelvic exenteration and ileal conduit formation in November 2015. At that time, she had a known primary lung adenocarcinoma as well, but had no known liver metastases or other liver disease. Intravenous contrast-enhanced CT of the abdomen and pelvis performed in January 2016 raised the possibility of cirrhosis; however this was not biopsy-proven. In April 2016, she began to notice intermittent bleeding from her stoma which was initially thought to be mechanical tissue breakdown from the stomal flange. Concern for hepatic encephalopathy was raised when she had her first episode of confusion in December 2016. At that time CT of the abdomen and pelvis demonstrated strong radiographic suspicion for cirrhosis together with prominent vessels surrounding the urinary diversion site suspicious for portal hypertension. Despite not having a tissue biopsy, she was diagnosed clinically with cryptogenic cirrhosis in May 2017 during a hospitalization for fatigue, anasarca, and altered mental status. An upper endoscopy performed in June 2017 demonstrated portal hypertensive gastropathy but no esophageal varices.\nUpon presentation to the Emergency Department in July 2017 she had significant hemorrhage from her stoma resulting in hemodynamic instability. She was anemic with a hemoglobin of 8.3 g/dL that improved to 9.4 g/dL after blood transfusion, but gradually fell to 8.2 g/dL by the time of the procedure. Her MELD score was retrospectively calculated to be 19 at presentation, with an INR of 1.5 and total bilirubin of 4.3 mg/dl. She was emergently taken to the interventional suite for embolization with or without portal venous decompression via portosystemic shunt formation. A review of intravenous contrast-enhanced CT imaging showed extensive venous varices around the stoma involving the abdominal wall with a large draining varix arising from the portal system, likely the inferior mesenteric vein []. Also visualized was a variceal connection to the right common femoral vein. The portal and mesenteric veins were noted to be patent. Multiple approaches were considered for this patient. The transjugular intrahepatic portosystemic shunt (TIPS) and transjugular transhepatic approach with portosystemic shunt creation offered the benefit of portal decompression; however, the patient's recurrent hepatic encephalopathy was felt to be a relative contraindication. Transjugular transhepatic approach without formation of a permanent portosystemic shunt was also considered, since it would eliminate the risk of progressive hepatic encephalopathy. Percutaneous transhepatic approach would also eliminate the risk of progressive hepatic encephalopathy but was believed to pose increased risks of hepatic injury and bleeding. Transsplenic venous access to the portal venous system was considered as a viable, albeit technically challenging, option. The superficial nature of the abdominal wall stomal varix presented a less challenging and seemingly more time-efficient approach for access and was chosen as the target.\nUsing a micropuncture kit, the peristomal varix was directly accessed under ultrasound guidance and a micropuncture sheath was placed. Venography was performed and showed a large variceal collateral conglomerate around the stoma with variceal anastomosis with the right common femoral vein []. A wire was advanced and a 5F sheath was secured over the wire. A Kumpe catheter was introduced and advanced into the distal intra-abdominal aspect of the large draining varix. Catheter position was confirmed with repeat venography, and multiple coils were deployed []. This was followed by Gelfoam embolization. Postembolization venography showed sluggish flow in the draining varix with multiple filling defects within the visualized collaterals consistent with embolization []. The coils remained well-situated after placement and there was no evidence of migration. To ensure that there was no filling from the systemic venous system, the right superficial femoral vein was then accessed with a micropuncture kit and a femoral-iliac venogram and IVC venogram were both performed. These demonstrated brisk flow from the right common femoral vein through the iliac system and into the IVC. There was no filling of the stomal variceal collaterals visualized []. Hemostasis was thereby achieved, and the patient became hemodynamically stable shortly thereafter. At 6-month follow-up time no further imaging had been performed and the patient had not had any further episodes of hemorrhage from the ileostomy site |
A 15-year-old female was seen in neurology clinic after several episodes of transient paralysis that were started when she was one year old. Her mother stated that during these episodes she was fully conscious, but half of her body was paralyzed, and she had difficulty in speaking. The paralysis was characterized by recurrent episodes of transient hemiplegia alternating in laterality or affecting both sides simultaneously. There was no associated or preceding vomiting, fever, seizures, loss of consciousness, or headache. The episodes lasted from few hours to few days and recurred every 10–30 days without leaving any residual deficit. The paralysis disappeared after a good sleep and reappeared within half an hour after awakening. She was dysarthric when the episode was unilateral and mute when it was bilateral. The child became ataxic with deterioration of cognitive and intellectual functions after seven years of disease onset. She also lagged developmentally behind her chronological age. She was a product of a nonconsanguineous marriage at 39 weeks of gestation following a spontaneous vaginal delivery. The pregnancy and neonatal period were uneventful. There was no family history of a similar disease including migraine. Her neuroimaging was normal on several occasions. At the age of 14, she developed sudden onset of a partial motor seizure of the face with secondary generalization and status epilepticus. The seizures were difficult to control, lasted several hours, and necessitated an admission to the intensive care unit with intubation and mechanical ventilation. The final diagnosis was AHC with status epilepticus and catastrophic cortical necrosis due to severe hypoxic brain insult. is showing her magnetic resonance imaging (MRI) few months following discharge from the hospital on multiple antiepileptic drugs. She was mute, quadriparetic, and psychotic. In a follow-up visit to the clinic, three years following discharge from the hospital, she showed some improvement in power and speech although continued to be fully dependent on the family for daily activities. She was also on multiple medications for her psychotic symptoms. |
A 6-year-old boy presented with pain and swelling in right upper back teeth region since a month in 2009. He gave a history of loose tooth in the same region for which the upper right permanent first molar was extracted a month back by a doctor in his place. After the extraction, swelling grew rapidly in size and was also evident extraorally since 20 days. On extraoral examination a solitary, diffuse swelling in the right middle third of face was seen, measuring approx 6x5 cm (). There was also a slight superior displacement of right eye ball. Bilateral submandibular lymph nodes were palpable, soft and tender.\nOn intraoral examination a solitary, exophytic, irregular growth was seen in the teeth 54, 55 region measuring approximately 4 × 4 cm, obliterating the buccal vestibule and extending palatally. It was fragile, soft in consistency, tender and readily bled on touch.\nOn radiographic examination a lytic lesion was present in the right maxillary sinus, with the erosion of the lateral wall and displacement of tooth 17 distally and an altered level of right infraorbital margin (). Features like rapid growth at the extraction site of 16, palatally displaced premolar clinically, radiographical presence of displaced 17 with erosion of lateral wall and floor of the sinus made us provisionally diagnose the lesion as giant cell lesion or a malignant lesion. Considering the age of the patient and bleeding tendency of the growth, fine needle aspiration cytology (FNAC) was preferred to a biopsy. The smear showed small round cells arranged in small clusters. The cells showed high nuclear to cytoplasmic ratio with scanty cytoplasm, giving an impression of small round cell tumor. Based on FNAC findings, an incisional biopsy was performed under general anesthesia which showed aggregates of blast cells with a thin rim of eosinophilic cytoplasm and a large hematoxyphilic nucleus which was suggestive of small round cell tumor, probably, Ewing’s sarcoma (ES). Following incisional biopsy, the patient’s parents were informed about the aggressiveness of the lesion and advised immediate surgical treatment.\nTransiently we lost the patient’s follow up for 4 months, during which patient got the lesion excised twice elsewhere. It was diagnosed as xanthogranuloma/pigmented epulis. After each excision lesion grew rapidly and started bleeding intermittently, indicating an aggressive clinical course. These tissues had been subjected to immunohistochemical (IHC) analysis at a higher center; a diagnosis of embryonal rhabdomysarcoma was given by them.\nThe patient presented to our hospital after 4 months with an extensively exaggerated growth in the same site (). A repeat radiographic examination revealed a lytic lesion of maxillary sinus with thinning out of zygoma (). Based on the clinical course and the investigations performed, the patient was treated by excising the tumor from the walls of the maxillary sinus and closing the defect primarily (). Chemotherapy was also advised during the initial phase of recovery. A 1 year follow-up was made which showed no clinical or radiographic evidence of tumor recurrence.\nThe histopathology of excisional biopsy revealed areas showing neural/glial differentiation (), primitive neuroepithelium, myxoid change, isolated areas of mucous metaplasia (), and ductal differentiation (), surplus squamous differentiation () cartilaginous () and osteoid differentiation. Apart from the above features, sheets of small round cells tumors showing vague rosettes were also appreciated (). The tumor tissues were subjected to few IHC markers. Sheets of round cells showed membranous CD-99 staining of round tumor cells (), focal positivity of tumors cells for S-100 and epithelial membrane antigen (EMA) positivity was seen in areas of squamous differentiation.\nHistological presence of sheets of round cells with vague rosettes and membranous CD-99 positivity depicted primitive neuroectodermal tumor. The presence of surplus squamous, mucous and ductal differentiation characterized ectodermal elements, along with the cartilaginous and osseous differentiation typified the mesodermal/neuroectodermal elements, leading to the final diagnosis of oral teratoma with PNET. |
A 55-year-old healthy woman was referred to our institution with a two-year history of progressive dysphagia to solids (). She reported a recent episode of solid food getting stuck in her throat, which prompted presentation to an outside endoscopist. The patient reported no alcohol use. She was a former smoker with a 15 pack-year history, but had quit over 20 years prior. The patient had a past medical history of gastroesophageal reflux disease, for which she was taking omeprazole, and hypothyroidism. She had no known history of any esophageal dysmotility disorder. There was a history of diabetes mellitus in her mother and son.\nPhysical exam and laboratory testing were unremarkable. Esophagography demonstrated a filling defect in the upper thoracic esophagus. Computed tomography (CT) demonstrated an 8 cm mass. Endoscopic ultrasound (EUS) demonstrated a pedunculated mass with a submucosal origin beginning at 20 cm from the incisors on the right side of the neck (). The lesion was felt to have the characteristic appearance of a FVP and the patient elected to proceed with resection.\nThe exploration began via a right cervical approach. The recurrent laryngeal nerve was identified and the cervical esophagus was mobilized. The mass was palpable on the posterior esophageal wall at the thoracic inlet. Upon a short myotomy, no stalk was identified and the mass could not be delivered to the neck. The cervical incision was closed and a right thoracotomy was performed. The mass was seen extending from the level of the azygos vein to the thoracic inlet. The esophageal muscular layer was intact. Following myotomy, the soft mass, which was densely adhered to the mucosa, was visualized and dissected from the underlying mucosa. It became evident that the mass maintained its attachment to a portion of the mucosa. Complete mobilization revealed the mass to be a lipoma at the tip of a large midesophageal diverticulum traveling in a submucosal plane. Repeat endoscopy demonstrated an ostium in the esophageal wall opening into a blind-ending pouch. The diverticulum was fully mobilized and resected using a stapler (). Mucosal closure was reinforced with overlying muscle and a pleural flap.\nThe patient was diagnosed with a large midesophageal diverticulum with a lead point lipoma. The patient’s postoperative course was uncomplicated. A postoperative esophagogram demonstrated no esophageal leak or obstruction. Pathology demonstrated a 7.5 cm diverticulum with a 4.5 cm lipoma without malignancy. At follow-up on the nineteenth postoperative day, the patient was tolerating a diet without dysphagia. |
We present a three-year old boy who presented to the Emergency Department at Mater Dei Hospital in Malta with respiratory distress and abdominal distension on a background of a one-month history of cough. On assessment, he was tachypnoeic with decreased air entry and dullness to percussion on the right side of the chest. Abdominal examination revealed a left sided flank mass. A chest radiograph showed opacification of the entire right lung field with mediastinal shift to the left and a possible retrocardiac mass ().\nEchocardiography revealed a heart that was significantly displaced (posteriorly and leftwards) by the intrathoracic mass. intracardiac structures and connections were normal. There was resultant distortion of the course of the great vessels, with the abdominal aorta and inferior vena cava being displaced anteriorly, navigating their way around the mass. This circumvented course hindered full interrogation of the vessels, and it was difficult to assess patency or presence of tumour invasion in the proximal segments of these vessels. The right atrium was externally compressed by the intrathoracic mass, rendering the right atrium to a slit-like shape, without any obvious obstruction to venous inflow. The significant cardiac displacement did not allow correct measurements of cardiac function such that indices relying on correct angulation could not be used ().\nAfter histological confirmation of nephroblastoma of the left kidney, chemotherapy with intravenous Vincristine, Actinomycin D and Doxorubicin was commenced according to the Clinical Management Guidelines Wilms Tumour by the Children’s Cancer and Leukaemia Cancer Group.\nThe clinical condition improved and the respiratory distress gradually resolved. Magnetic resonance imaging (MRI) with venous studies performed just two weeks after admission showed a significant reduction in the size of the renal primary and the right pleural based and mediastinal mass with resolution of the mediastinal shift, with no inferior vena cava or left renal vein thrombus. Chest radiography performed three weeks after admission showed improved aeration of the right lung and reduction in the size of the pleural effusion. Repeat echocardiography four weeks after admission showed improved findings with resolution of the previously documented cardiac distortion. Normal function could now be documented clearly in view of correct angles of measurement and normal acoustic windows. ().\nOur patient underwent an uneventful nephrectomy, histology being compatible with Stage I intermediate risk regressive nephroblastoma. He continues to receive post-operative chemotherapy as per the Clinical Management Guidelines Wilms Tumour by the Children’s Cancer and Leukaemia Cancer Group. |
A 73-year-old Chinese man was found a cervical mass for 1 month. He had a history of coronary heart disease for 2 years, but no relevant personal or family history of malignancy. Physical examination showed bilateral neck asymmetry, the trachea was deflected to the left, the carotid pulse was normal, approximately 6 × 5 cm solid masses were felt in the right lobe of the thyroid gland. The tumor mass border was unclear with smooth surface, and the tumor moved up and down with swallowing. The left lobe of the thyroid gland had no palpable mass and the neck had no palpable swollen lymph nodes. Accessory examination of neck color Doppler ultrasound at our hospital showed increase in size of the right lobe of the thyroid gland, which had abnormal shape. The upper right pole of the thyroid gland had an approximately 5.6 × 6.0 × 4.2 cm sized mass, with unclear margin, and multiple cystic nodules in the thyroid. The CT of thyroid showed increase in size of the right lobe of the thyroid gland, and an approximately 5.6 × 6.0 × 4.2 cm sized mass, the trachea was compressed and deflected to the left, the thyroid cartilage was compressed, deformed and reached the right subglottic region. The right thyroid lump had multiple calcifications, indicative of a tumor (Fig. ). Electronic laryngoscope examination showed ventricular bands thickening, the right ventricular bands compartment showed a rice-like projection, arytenoid region movement was poor (Fig. ). Thyroid function test was 5.07 mIU/L. The patient underwent subtotal thyroidectomy and excisional biopsy of neck mass under general anesthesia. Intraoperative findings indicated a hard mass in the thyroid gland area, the lateral border and the lower bound were closely related to the surrounding soft tissues, but there was an extremely close relationship between the medial border, the thyroid cartilage, and cricoid cartilage, with no gap between them. An ENT doctor was invited for intraoperative consultation, who suggested that some of the tumors should be removed and frozen. The rapid pathology of the tumor (thyroid right lateral lobe) indicated chondroma. The ENT doctor suggested laryngeal chondroma resection and tracheotomy under general anesthesia. Intraoperative findings indicated that the right lower half of the thyroid cartilage and the right half of the cricoid cartilage were chondroma, and complete resection of the tumor was performed. Postoperative pathology examination showed (right lobe of thyroid gland and larynx) chondroma (Fig. ). Given the advanced age of the patient, long surgical duration and poor cardiorespiratory function, the patient suffered sudden cardiac death after the operation. |
Mr. BH, 39 years old, right-handed, presented with a Bennett fracture of the first right metacarpal following a work accident. The fracture was initially treated in another institution by reduction and percutaneous pinning using Iselin’s technique. Three weeks later, the patient developed an inflammatory reaction around the entry point of the distal pin, at the second metacarpal. A purulent discharge led to early removal of the distal pin. The isolated organism was a methicillin-sensitive Staphylococcus aureus. Regular topical treatment was given, and antibiotic therapy was initiated for five weeks based on laboratory results. Bone healing of the Bennett fracture was obtained in the sixth week after the accident, and the proximal pin was removed.\nThree months later, the patient was referred to us by his doctor because of persistent pain at the second right metacarpal, accompanied by attacks of inflammation and intermittent purulent discharge. Standard hand X-ray demonstrated the presence of an area of osteolysis surrounded by osteocondensation at the second metacarpal, indicating a focus of osteomyelitis along the path of the distal pin (). An MRI was performed on the hand. It confirmed the diagnosis, revealing the extent of the focus of osteomyelitis, the path of the fistula, and the inflammatory extension into neighboring soft tissues ().\nSurgical treatment called for the induced membrane technique.\nThe first stage included a segmental resection of the area of osteomyelitis through a dorsal approach with excision of the reformed tissue while preserving the index finger extensor tendon.\nThe second metacarpal was stabilized with a mini external fixator, and a cement spacer was interposed ().\nAntibiotic therapy was initiated for six weeks based on sensitivity testing. The external fixator was then removed, and the hand temporarily immobilized with a splint until there was healing along the path of the inserts.\nThe second stage was performed in the eighth week. The cement spacer was removed and the biological chamber was filled with a spongy bone graft taken from the ipsilateral iliac crest. Internal fixation was performed with a mini plate while preserving the induced membrane. The postoperative period was uneventful. Bone healing was observed three months later () with complete disappearance of pain. The patient has regained normal use of the hand with a Quick DASH score of 11 and complete mobility (. We are currently at the 36-month follow-up. X-ray of the hand shows incorporation of the graft and evidence of corticalization (). |
We present the case of a 56-year-old female with history of syncope due to third degree atrioventricular heart block presenting initially with onset of stroke symptoms six days after pacemaker placement and two days after hospital discharge. At 5 PM she developed abrupt onset of left facial droop along with left upper and lower extremity weakness. The patient was initially treated at an outlying hospital and received alteplase at 6:35 PM for treatment of acute ischemic stroke.\nA chest radiograph performed at the outlying hospital prior to alteplase administration demonstrated an enlarged cardiac silhouette when compared to prior radiographs showing only borderline cardiomegaly. Upon administration, the patient reported mild chest pain and was given nitroglycerine and morphine. Her chest pain resolved and she was transferred to our comprehensive stroke center for admission. The patient presented to our emergency department at 10:10 PM with a heart rate of 122 beats per minute (bpm) and a blood pressure of 109/41 millimeters of mercury (mmHg).\nAt 11:20 PM the patient went for a computed tomography angiogram (CTA) after an initial assessment by the emergency physician in consultation with the stroke-team attending physician. After CTA at 10:28 PM, she was documented to have a blood pressure of 49/25 mmHg and heart rate of 109 bpm. She was returned to the resuscitation bay for re-evaluation. Cardiac tamponade was suspected due to the extreme hypotension in the setting of thrombolytic administration after recent pacemaker placement.\nOn reassessment, the patient had become confused with a Glasgow Coma Scale of 14. The emergency physician performed a POCUS, which demonstrated a pericardial effusion with features of cardiac tamponade including diastolic collapse of the right ventricle (). At that point the diagnosis of cardiac tamponade was made. The patient was alert and responsive, so an intravenous bolus of normal saline was given while a stat surgical consult was obtained. The surgical team evaluated the patient at the bedside within minutes and was able to review the POCUS findings. As the patient was conscious, they elected to take her immediately to the operating room rather than perform a bedside pericardiocentesis.\nWhile in the operating room, approximately 400 milliliters of coagulated blood were evacuated from the pericardial sac with 150 milliliters of surgical bleeding. The operative report notes resolution of tachycardia following this intervention with heart rate trending down to a range of 80–90 bpm with concomitant improvement in blood pressure. She was discharged two days post-operatively with a pericardial catheter in place. Echocardiogram performed on day of discharge noted a small, residual pericardial effusion. |
Patient n. 2 was a 60-year old female, diagnosed with suffering from POAG for ten years, under treatment with hypotensive drugs and regularly controlled IOP twice a year which appeared maintained within normal ranges. During a control visit in January 2016, the patient had reported irritating symptoms of eye discomfort, described as burning, itchiness, and feeling sand, mainly in her right eye (RE), over the last six months. The situation had been managed with the use of several types of tear substitutes, none of them successful in symptom relief. Symptoms were scored in the RE as OSDI of 74 out of 100 and a VAS score of pain of 85 mm out of 100. In the left eye (LE) the symptoms were defined by the patients as light and acceptable: OSDI score was 22 out of 100 and VAS 21 mm out of 100. Slit lamp evaluation had not shown epithelial damage, and there were no signs of inflammation, but only a reduced Tear Film Break-Up Time (TFBUT) of 4 seconds in RE and 8 seconds in LE had been recorded. A therapy with hyaluronic acid (HA) based tear substitute to be administered 4 times/day in both eyes was prescribed.\nIn March 2016, the patient was proposed to receive in her RE a treatment with topical CBS, as a compassionate unconventional therapy, with the aim to reduce the pain symptoms for which the previous therapeutic attempts had turned to be unsuccessful. The therapy for the contralateral LE was maintained with HA based tear substitutes.\nThe patient signed the specifically designed informed consent and started administration of the CBS eye drops in April 2016, with the posology of 0.4 ml (8 drops) in RE, each day for a total of two months. In the GF dosages determined for the two CBS lots were administered during the first and second month.\nOn September 2016, the patient reported a significant relief from subjective symptoms of discomfort; in RE the OSDI was 28 out of 100, with VAS: 32 mm out of 100. Also in LE a reduction was observed, with OSDI score determined as 16 out of 100 and VAS 15 mm out of 100. During this visit, also the IOP was measured, which resulted in the normal range in both eyes and the analyses of the visual field tests performed since 2006.\nIn the mean deviation (MD) values recorded over several visits performed from December 2006 through September 2016 were graphed. A significant progressive lowering in MD values was observed which was followed by an important amelioration in correspondence with the period of treatment with CBS, in the figure highlighted with the arrow. It has to be noted that the increase in MD values was recorded either in the treated right eye or in the untreated left eye. Moreover, the MD values recorded in September 2016, four months after the end of the CBS eye drop administration, showed in both eyes a further amelioration.\nThe improvement is also demonstrated by the analysis of the PSD shown in . A progressive worsening in PSD values had been observed over ten years, whereas a rapid change was recorded in correspondence with the CBS eye drop treatment, either in the treated right eye or in the untreated left eye.\nIn the central 30-2 visual field tests before ((a): right eye RE; (b): left eye LE), at the end (c, d) and after four months (e, f) from the end of the CBS eye drop treatment were shown. An amelioration of the defect was observed in all the four quadrants in both the treated RE and the untreated LE.\nTaking together these observations, a positive effect also in the left untreated eye could be recorded, which suggests a neural cross-talk mechanism between the eyes. |
A 65-year-old right-handed male patient presented with an unremitting pain in his right shoulder over a period of 34 years (). He was involved in an accident in 1983 when his right arm was pulled into a press machine while working in a factory. Severe crushing injury in the right arm led to the amputation of the entire arm at the shoulder joint (shoulder disarticulation). About a year later, a tingling pain developed in the phantom hand and fingers of the amputee. The tingling pain persisted throughout his phantom hand and fingers, and intermittent electrical shock-like pain superimposed every few seconds. His phantom hand and finger pain was very severe and lasted day and night and was not controlled by anticonvulsants and painkillers. The pain was so uncontrollable that he was admitted to a university hospital twice and examined, but no specific treatment was advised.\nAs the decade passed, the right phantom limb disappeared as it gradually shrank in the severed shoulder area (telescoping phenomenon). Although the right phantom limb disappeared as it become smaller, but the continuous and paroxysmal pain did not disappear and continued in the shoulder area of the amputation. Opiates did not work and have severe side effects, so he lived with pain for 20 years while taking 600 mg per day of pregabalin.\nUpon examination, there was no right arm in the right shoulder due to shoulder disarticulation caused by the accident in 1983 (). There was no mass in the stump of the right shoulder and no tenderness was observed. The skin over the stump looked healthy. The sensation over the right shoulder stump was normal and movement of the right scapula was possible. He denied any phantom sensation and phantom pain. The pain pattern was the same as when it first occurred. With the presence of continuous tingling pain, additional pain such as electric shock occurred once every few seconds. His pain was mainly distributed in the area of the amputation stump. It was persistent and did not worsen in any particular instance, such as emotional or physical activity. However, if he stayed in one position for a long time during sleep, he would wake up from a sudden attack of shoulder pain and could not sleep until the pain is reduced for 2 or 3 hours. He rated his pain as 7 out of a range from 0 to 10 on the NRS-11 scale.\nBecause his pain, along with typical continuous tingling pain, included intermittent electric shocks, a cause of BPA was suspected. Myelographic computed tomography (CT) and magnetic resonance imaging (MRI) of the cervical spine with myelographic MRI were requested. On the myelographic CT scan, ventral and dorsal roots were absent from C7 to the T1 level (). Pseudomeningocele was not observed. In the MRI, the cervical spinal cord was distorted and a slightly high signal was observed along the dorsal horn of the avulsed segments of the cervical spinal cord (). Some widening and elongation of the dorsal root sleeve of the right C7/T1 dural sleeve were noted.\nAfter confirming a diagnosis of chronic neuropathic pain of BPA, microscopic coagulation of the DREZ (microsurgical DREZ-otomy) was performed. After performing right-sided hemilaminectomy from C5 to T2, the dura was opened. Intraoperative neurophysiologic monitoring of the motor evoked potential (MEP) and the somatosensory evoked potential (SEP) of ipsilateral and contralateral arms and legs were performed. The avulsed posterolateral sulcus (C7–T1) was identified under microscopic vision. After opening the pia mater of the avulsed sulcus, coagulation of grayish-yellow-colored gliotic dorsal horn was performed with fine-tipped, bipolar coagulation forceps specially designed for the application (). During the coagulation, the SEP and the MEP of right lower extremity and left extremities were stable ().\nWithin 2 days after the microsurgical DREZ-otomy, the patient's recurrent paroxysmal pain disappeared completely and his continuous tingling pain decreased significantly. There were no neurologic complications postoperatively. His pain attacks did not recur until a year after the operation. He assessed that pain was 80% less than before surgery and was tolerable. His medication was 150 mg of pregabalin a day. |
Mr. BH, 39 years old, right-handed, presented with a Bennett fracture of the first right metacarpal following a work accident. The fracture was initially treated in another institution by reduction and percutaneous pinning using Iselin’s technique. Three weeks later, the patient developed an inflammatory reaction around the entry point of the distal pin, at the second metacarpal. A purulent discharge led to early removal of the distal pin. The isolated organism was a methicillin-sensitive Staphylococcus aureus. Regular topical treatment was given, and antibiotic therapy was initiated for five weeks based on laboratory results. Bone healing of the Bennett fracture was obtained in the sixth week after the accident, and the proximal pin was removed.\nThree months later, the patient was referred to us by his doctor because of persistent pain at the second right metacarpal, accompanied by attacks of inflammation and intermittent purulent discharge. Standard hand X-ray demonstrated the presence of an area of osteolysis surrounded by osteocondensation at the second metacarpal, indicating a focus of osteomyelitis along the path of the distal pin (). An MRI was performed on the hand. It confirmed the diagnosis, revealing the extent of the focus of osteomyelitis, the path of the fistula, and the inflammatory extension into neighboring soft tissues ().\nSurgical treatment called for the induced membrane technique.\nThe first stage included a segmental resection of the area of osteomyelitis through a dorsal approach with excision of the reformed tissue while preserving the index finger extensor tendon.\nThe second metacarpal was stabilized with a mini external fixator, and a cement spacer was interposed ().\nAntibiotic therapy was initiated for six weeks based on sensitivity testing. The external fixator was then removed, and the hand temporarily immobilized with a splint until there was healing along the path of the inserts.\nThe second stage was performed in the eighth week. The cement spacer was removed and the biological chamber was filled with a spongy bone graft taken from the ipsilateral iliac crest. Internal fixation was performed with a mini plate while preserving the induced membrane. The postoperative period was uneventful. Bone healing was observed three months later () with complete disappearance of pain. The patient has regained normal use of the hand with a Quick DASH score of 11 and complete mobility (. We are currently at the 36-month follow-up. X-ray of the hand shows incorporation of the graft and evidence of corticalization (). |
A 31-year-old woman with no significant past medical history presented to our emergency department complaining of a constant headache for the previous 4 days. The headache had begun approximately 6 h after receiving epidural anesthesia for labor. The documentation from the anesthesia service that day reported the use of a 17-gauge Touhy needle to enter the subdural space in the lower lumbar spine and the placement of a 19-gauge epidural catheter. No complications were reported with the procedure, and specifically, there was no mention of inadvertent dural puncture. The patient had an unremarkable delivery of a healthy infant at 38-weeks gestation later that day.\nThe patient described the headache as constant and occipital with some radiation to the frontal area. The headache was worse when upright and partially relieved in the supine position. She reported taking acetaminophen/butalbital/caffeine and ibuprofen with little relief. She had no associated vomiting, fever, or changes in her hearing or vision. She denied any photophobia or focal weakness or numbness. She was afebrile on physical exam, with pulse and blood pressure within the normal range. Her exam was notable for a normal neurologic exam including cranial nerves and no neck stiffness. The patient was tentatively diagnosed with a PDPH. After evaluation by the anesthesia service, she was admitted for pain control and possible placement of an epidural blood patch the next day. A computed tomography (CT) scan of her head was obtained prior to admission to evaluate for other possible causes of the headache (Fig. ). This CT identified bilateral parafalcine subdural hematomas measuring 7 mm in thickness on the left and 3 mm thickness on the right. There was no associated mass effect.\nThe patient was admitted to the intensive care unit and started on levetiracetam for seizure prophylaxis. Neurosurgical consultation advised observation, and a repeat CT scan of the head the next day showed no significant change in the hematomas. The patient also received an epidural blood patch the next day with no improvement in the headache. A head CT performed on hospital day 3 showed a decrease in the size of the hematomas, and the patient was discharged on levetiracetam for seizure prophylaxis for 1 week.\nISH occurring after dural puncture is extremely rare. Only sporadic case reports and a few small case series have described this condition [–]. Any procedure that results in spinal dural puncture will theoretically predispose to the development of an ISH. ISH has been described following epidural and spinal anesthesia, as well as lumbar puncture, myelography, epidural steroid injection, and after implantation of an intrathecal drug delivery device and a spinal cord stimulator [–]. The incidence of ISH specifically caused by epidural anesthesia used in obstetric practice has been estimated to be 1:500,000 [].\nThe same mechanism has been postulated for both PDPH and ISH []. The leakage of cerebral spinal fluid (CSF) from the dural puncture site may continue for several weeks, causing reduction in CSF volume []. This results in lower intraspinal and intracranial pressure, leading to relative ventricular collapse and caudal movement of the spinal cord and brain. As a consequence, the dura, pain-sensitive structures, cranial nerves, and subdural bridging veins are stretched. This may ultimately result in a tear of the bridging veins and consequently an ISH. Risk factors associated with ISH after dural puncture include excessive CSF leakage from multiple punctures in large needle use, pregnancy, coagulopathy, cerebral vascular abnormalities, dehydration, brain atrophy, and alcoholism [–].\nThe duration of time from dural puncture to the diagnosis of ISH ranges widely from 4 h to 29 weeks []. In one case series, 37% of cases were diagnosed within 1 week of dural puncture, and 85% were diagnosed within 1 month []. A headache, most often diagnosed as PDPH, is the main presenting symptom [, –]. Other reported symptoms and signs present at the time of diagnosis are listed in Table [, , ]. Reported rates of surgical intervention for ISH after dural puncture vary from 9 to 80% [, , ]. In general, surgical intervention for ISH is indicated if the hematoma thickness exceeds 10 mm, there is a midline shift of greater than 5 mm, or there is neurologic deterioration []. Furthermore, some have advocated for the use of epidural blood patching in the treatment of ISH caused by dural tears resulting in chronic CSF leaks [, ]. A full recovery is reported in over 80% of patients, with death reported in 7–10% of cases [–].\nHeadache in the postpartum period is common, occurring in 39% of women []. The majority of these headaches are benign primary headaches, such as migraine and tension type []. Secondary headaches in the postpartum period are typically due to obstetric or anesthetic complications, or the hypercoagulable state after delivery (Table ). Our patient was initially misdiagnosed as having PDPH, similar to many previous reports of this condition. PDPH is defined as a headache that develops within 5 days of dural puncture that significantly worsens soon after sitting upright or standing and improves after lying horizontally []. PDPH is more likely to occur in young women of low body mass as compared with other patients []. An epidural blood patch is considered the gold standard for treatment of PDPH, with a success rate of 70–90% []. Over 85% of patients report resolution of PDPH within 6 weeks regardless of treatment [].\nThe incidence of ISH after dural puncture is probably underreported since many of these patients are treated as PDPH with the eventual resolution of their symptoms. When to obtain brain imaging studies in the assessment of a likely PDPH is unclear. A reasonable approach would be to consider imaging in patients that (1) have a postural headache lasting more than 1 week, (2) do not improve or have worsening of their headache after an epidural blood patch, (3) report a change in the headache from postural to non-postural, or (4) develop other neurologic signs or symptoms with the headache []. |
A 71 year old post-menopausal, caucasian woman was admitted under the care of the general surgeons. She was a teetotal pensioner but smoked fifteen cigarettes per day. She had three previous normal vaginal deliveries and her cervical smear history was normal.\nShe presented with a three day history of feeling generally unwell and left iliac fossa pain. She had no bowel or urinary symptoms. She did however report a generally poor appetite and had lost two stone in weight over the previous six months.\nOn examination she was febrile and a mass was palpable in the left upper quadrant. A computerized tomography (CT) scan was performed. This showed a 7 cm × 7 cm × 8 cm anomaly on the left side of the abdomen. Diverticuli were also noted in the adjacent bowel.\nA colonoscopy was arranged which confirmed the presence of diverticular disease in the sigmoid colon. The clinical impression of the anomaly was that it was a peri-colic abscess and a decision was taken to treat the patient with antibiotics. The patient's symptoms improved and she was discharged home.\nA follow up CT scan was requested a few months later but the patient did not attend. She did however attend for surgical out-patient review six months after she first presented. At which time she complained of a greenish vaginal discharge.\nShe was then referred to the gynaecologists who carried out a pipelle endometrial biopsy and a transvaginal ultrasound scan. This showed a large fluid filled cavity suggesting a pyometra. An urgent hysteroscopy was performed and a large pyometra was confirmed and drained. Endometrial currettings were also obtained. Although the patient had been started on intravenous antibiotics, she subsequently became unwell and febrile.\nAt this point further imaging was requested. Her CT scan showed a diverticular sigmoid, with a 7 cm collection lying adjacent to the uterus (on the left). It was postulated that there was a fistula between the bowel and the uterus.\nThe patient's condition continued to deteriorate and she was taken to theatre for an urgent laparotomy. At operation the sigmoid colon mass was found to be adherent to the uterine fundus, with adjacent pus. A subtotal hysterectomy, bilateral salpingo-oophorectomy, left hemi-colectomy and left colostomy were performed by a joint gynaecological – colorectal surgical team. The patient was transferred to the intensive care unit and subsequently made a good post-operative recovery.\nHistology showed a moderately differentiated adenocarcinoma of the perforated colon. The uterus showed only a pyometra of uncertain origin and there was absolutely no evidence of a fistula or of a uterine perforation. |
In 2014, a 42-year-old woman was referred to our hospital for complete resection of a right subclavian tumor. She had originally undergone right axillary, subclavian, and Rotter’s lymph node resection for right occult breast cancer in 2009. After resection, she was administered chemotherapy and actinotherapy. In 2011, she returned with recurrence of a tumor in her right subclavian area. At that time, surgical removal of the tumor was attempted but abandoned because the tumor had tightly adhered to the subclavian artery. However, she did have a complete response to chemotherapy and actinotherapy. The tumor shrank in size, and she was considered to be in a state of complete tumor response (CR).\nWhen she was referred to us, computerized tomography revealed a poorly marginated and enhanced tumor that had occluded the subclavian vein and adhered to the subclavian artery at the infraclavicular lymph node (). Various other examinations determined that she did not have any other metastases. The patient was relatively young and desired to have the tumor removed. We made the decision to perform a complete resection of the tumor together with the sections of the subclavian vein and artery involved. Based on the imaging results, we expected that it would be difficult to control bleeding and to prevent subclavian arterial decortication during the tumorectomy. One of our surgical team suggested that we performed subclavian artery stenting prior to tumor resection to minimize bleeding risk, and we attempted this.\nAfter obtaining informed consent from the patient and approval from the Hospital Ethics Committee, we decided to place stents (Niti-S ComVi stent 8 mm × 8 cm; Taewoong Medical, Seoul, Korea) in the subclavian artery. We placed two stents so that their overlap reached the middle of the tumor, and the landing length was 3 cm from the right brachiocephalic artery.\nSurgery was performed 1 week after stent placement. We removed the right clavicle to allow access to the tumor. We identified and controlled bleeding from the right subclavian artery and the axillary artery, which came from a small incision in the axillary artery. We removed the tumor with the arterial wall including intima, and we were able to detect the placed stent. Surgical bleeding was not observed (). To prevent the stent from moving and causing bleeding, we wrapped the entire length of the stent with an artificial blood vessel (8 mm Advanta™ VXT with ring; Atrium Medical Corporation, Ethicon, USA) and fixed the artificial vessel to the proximal and distal sides of the native artery with a running suture with 6-0 prolene (). We excised the subclavian vein, which strongly adhered to the tumor, en bloc. The tumor was 21 mm × 13 mm, and pathological analysis showed that it was an adenocarcinoma, which was the same as the patient’s previous tumor. Therefore, the tumor was diagnosed as metastatic adenocarcinoma. We did not detect malignancy in the resected subclavian vein. Cancer had invaded the adventitia of the subclavian artery, but the media and intima of the artery were not invaded.\nWe considered the tumor to be completely resected. The postoperative course was good, and postoperative neurological impairment was not observed. The patient received an oral antiplatelet drug after surgery.\nOur institution does not require ethics approval for reporting individual cases. Our institution does not require informed consent to be obtained for reporting individual cases. |
In August 2005, a 25-year-old woman on hemodialysis was referred to our renal clinic with a positive pregnancy test based on serum beta HCG. She had end-stage renal disease secondary to type 1 diabetes and had started dialysis 9 months prior to her visit. Her last menstrual period was 6 weeks prior to the visit. She had had two previous pregnancies, the first resulting in spontaneous abortion in the first trimester and the second resulting in the delivery of a baby at 30 weeks gestation. The second pregnancy was in 2001. She was not told that she had kidney disease during her previous pregnancies. Her past medical history was significant for diabetes since age 9 with retinopathy and nephropathy. She was not aware of any kidney disease until she reached the point of needing dialysis in September 2004 and had been on thrice weekly dialysis since then. Her medications included insulin, RenaGel, PhosLo, folic acid and MVI. On exam, her BP was 112/67 mm Hg and she did not have any edema. Her beta HCG levels were 13 270 mIU/mL at the time of her pregnancy test and increased to 30 232 mIU/mL within 3 days. The plan was to increase her dialysis regimen to more than 20 h per week and she was referred to the care of high-risk obstetrics. She had a transvaginal ultrasound scan, which showed an intra-uterine gestational sac of 5.3 weeks, but no fetal heart tone could be detected. Given that she had HCG levels consistent with 7 or more weeks gestation without visible cardiac activity, she was thought to have a non-viable pregnancy and surgical termination was contemplated once her blood sugars were well controlled. Beta HCG levels were followed with the expectation that they would drop in the setting of a non-viable pregnancy. Her beta HCG levels are charted below:\nA transvaginal ultrasound was repeated in 10 days and showed an intra-uterine pregnancy consistent with a gestational age of 7 weeks and with good fetal heart tones at a rate of 128 bpm. She continued with her pregnancy and was started on 5 × weekly dialysis. Although, in the second trimester, she had an abnormal triple antigen screening test for fetal abnormalities, she delivered a healthy baby weighing 3.5 kg at 32 weeks without any further complications. |
A 64-year-old male visited the emergency room because he was experiencing aggravated dyspnea during exercise and edema on his right leg over the preceding 3 days. Although he had undergone percutaneous coronary intervention for his 2-vessel coronary artery disease 3 months before his visit, he was still experiencing chest discomfort. He weighed 77 kg when he was admitted to the hospital while his healthy weight was 72 kg. He had diabetes. An echocardiogram revealed that he had RWMA in the area supplied by his right coronary artery and left circumflex coronary artery, his LA and LV were enlarged, and his LV had a reduced systolic function with an EF of 12%. Diuretics were prescribed to promote urination. On the 9th day of hospitalization, a stent was inserted in his left circumflex coronary artery under coronary angiography and a previously inserted stent at his right coronary artery was confirmed to be intact. He was discharged from the hospital on HOD#10. He weighed 65 kg when he was discharged.\nAt the end of the group session on HOD#6, a clinical dietitian evaluated his nutritional status. Over 3 months before hospitalization, the dyspnea he experienced during exercise caused him to decrease his food consumption by half. He would eat 3 meals/day of rice and kimchi and ate some fruit between meals. He did not consume meat, other vegetables, milk, or sweetened or high-fat bread or chips. He reported that he would frequently eat out or order delivery foods, including Korean soups and stews. His initial overall NQ was 49.2, which was low and his diversity and dietary behavior category scores were particularly low. His diagnosis was inadequate food and beverage intake as a result of his dyspnea as evidenced by the fact that his estimated calorie consumption was 50.7% of the recommended amount and his estimated protein consumption was 45.1% of the recommended amount. The clinical dietitian encouraged him to eat more by consuming small, frequent meals and taking an oral nutritional supplement. First follow-up session was 2 weeks after his group session. His estimated calorie and protein consumption increased to 1,840 kcal/day and 87 g/day, which were 92% and 102% of the recommended amounts, respectively. However, his increased food consumption resulted in his estimated sodium consumption increasing to more than twice the recommended amount. He ate out for 2 meals/day. He would eat noodles or Korean soup for lunch and grilled beef or bulgogi for dinner. A clinical dietitian taught him how to choose foods that would lower his sodium intake and how to analyze nutrition information and provided him with low-salt kimchi recipes. His second follow-up session was held 4 months after his first follow-up session. During his second session, he said that he was eating more balanced foods, such as bibimbap, kimbap, and grilled pork with vegetables. An analysis of his 3-day food diary showed that his estimated calorie and protein consumption had decreased slightly to 1,740 kcal/day and 61 g of protein/day, respectively. His BMI was 21.9 kg/m2, which was within the normal range. He said that he was not experiencing any more respiratory symptoms, including dyspnea, and an echocardiogram showed that there were no interval changes in his pre-existing RWMA, enlarged LA and LV, and his reduced LV systolic function with an EF of 28%. |
A 55-year-old male patient complaining of a painless mass in the left preauricular area that had been present for 3 months was admitted to our clinic. There was no other symptom and his general physical examinations were completely normal. Physical examination showed a 35 × 30 mm mass in left parotid gland that is partially mobile and painless and covered with normal skin. The facial nerve functions were normal on clinical evaluation. Fine-needle aspiration cytology (FNAC) result was nondiagnostic and complete blood count was normal.\nIn magnetic resonance imaging (MRI) a homogeneous 30 × 20 mm lesion was detected in superficial and deep lobes of left parotid gland spreading to anterior of masseter muscle which is hypointense in T1A sections and hyperintense in T2A sections and with intense contrast enhancement (Figures and ).\nA parotidectomy was planned for the patient. During the operation firstly the main trunk of facial nerve was identified. The main part of the tumor was a 20 × 20 mm reddish, bright lesion with a thin capsule around the temporofacial branch of facial nerve anterosuperior to main trunk. The lesion was encircling the temporofacial branch of the facial nerve and advancing forward through the peripheral branches. At this point we could not follow the nerve, so we referred to frozen section. The result was reported as “mesenchymal lesion.” When we stimulated the lesion with the nerve stimulator we got an answer from the muscles that were innervated by that branch and because of the frozen section result we thought that the lesion might be a tumor of the nerve itself. In this occasion we cut the temporofacial branch of the nerve from the main trunk and excised the tumor totally with branches (zygomatic, temporal, and buccal branches) surrounding the mass medially (Figures , , and ). A weakness was detected in left eyelid postoperatively. The patient was able to close his eye with effort so no additional intervention was considered afterwards. The weak conservation of eyelid function despite the sacrificing of temporofacial branch could be explained by the compensation from collateral peripheral branches located in front of the resected tumor.\nThe histopathologic result was reported as “schwannoma.” The cells that build the tumor were fusiform nucleated spindle cells and had eosinophilic cytoplasms with ill-defined boundaries. In these cells there was palisade forming hypercellular areas and wavy structure of the fibers in the stroma in some places. There was no necrosis, mitosis, or atypia. The neoplastic cells were vimentin and S-100 positive immunohistochemically (Figures and ). |
An 86-year-old woman was referred for a screening colonoscopy because she complained of loss of weight associated with diarrhea and anemia. The patient was in regular general condition, and a physical examination revealed pain on deep palpation of the lower right abdomen. The family reported that the patient had undergone a subtotal gastrectomy to resect an early adenocarcinoma of the gastric antrum 22 years earlier and reported no prior family history of colorectal cancer. The family indicated that the patient started taking cholinesterase inhibitors for the treatment of initial Alzheimer's disease beginning about two years earlier. The colonoscopy identified left-side diverticular disease and the presence of a vegetating lesion protruding into the cecal lumen from the appendicular ostium. The lesion was reddish with a fine nodular surface that spread to the wall of the cecum, with a length of 1.5 cm around the ostium of the appendix (Figures and ).\nChromoscopy with nebulization of indigo carmine showed that the mucosal surface of the cecum was uneven and that there was no clear demarcation regarding its limits. The glands had star-shaped pits similar to conventional type II pits but, in focal areas, this pattern of crypts changed to type IV of Kudo classification []. The ostium of the appendix was located in the central portion of the lesion, which was covered with a thin layer of mucus, and had no apparent signs of inflammation or obstruction. Abdominal computed tomography could not identify the appendicular mass or any lymph node enlargement in the abdominal cavity.\nBased on a suspicion of an adenoma of the vermiform appendix, fragments were collected for a histopathology study. Microscopic analysis showed the presence of a tubular adenoma with low-grade dysplasia (Vienna 3) []. Due to the characteristics and location of the lesion, which did not allow safe endoscopic removal during colonoscopy, the patient was referred for laparoscopic resection. The laparoscopy showed that the tumor began in the proximal third of the appendix, and nearly was implanted in the cecum, without compromising the serosa of the appendix and cecum and without any lymph node enlargement. Considering the advanced age of the patient, a resection of the appendix and part of the cecal wall was performed with a single firing of a laparoscopic mechanical linear stapler (). The surgical specimen was removed out of the abdominal cavity inside a plastic bag.\nThe entire surgical specimen was subjected to a histopathological study. The macroscopic examination showed the presence of a circumferential vegetating mass located in the vermiform appendix, with 2 cm of length that protruded into the cecal ostium of the appendix. The lesion compromised 1.5 cm of the cecum wall. The histological sections showed the vermiform appendix with a sessile polypoid lesion characterized by the proliferation of the epithelial lining with a lush serrated architecture. The tissue slices showed the presence of dilated crypts compressing the lamina propria at the base of the lesion. The epithelial cells presented eosinophilia of the cytoplasm and light atypia with tubular glands that were intensely stained, elongated, and slightly stratified. In focal areas, there were glandular structures lined by epithelial cells with marked atypia and complex architectural changes. The margins of surgical specimen were free of neoplastic cells. These findings led to a diagnosis of intramucosal carcinoma originating in a sessile serrated adenoma of the appendix (). After surgery, the patient showed an uneventful postoperative course and was discharged on the fourth day. One year after the surgical procedure, the patient had recovered from the weight loss and anemia. |
A previously healthy 7-year-old girl was admitted to the hospital after waking up with acute onset expressive aphasia. The patient had no family history of cardiac or neurological disease and was not taking any medication. During a vacation, 3 years ago, the patient experienced an episode where she suddenly became confused and could not express herself coherently. She was assessed at a local hospital where neither cerebral imaging or ECG were performed. It was concluded that the patient had suffered a cerebral concussion despite no history of trauma. The patient had complete remission of all symptoms within a few hours.\nUpon presentation at our hospital, the patient was awake, alert and fully orientated. Clinicians determined the presence of expressive aphasia and a mild central facial palsy on the right side, which regressed and disappeared within the same day. The patient did not complain of any cardiac symptoms, had normal heart sounds and a normal blood pressure of 106/51 mmHg. Her peripheral pulse was irregular with an average heart rate (HR) of 60 beats per minute (BPM).\nCerebral magnetic resonance imaging (MRI) showed a recent infarction in the left frontal lobe and anterior insula cortex consistent with an occlusion of the frontal branch of the left middle cerebral artery. Furthermore, signs of earlier infarctions were seen in the parietal and temporal regions of the right hemisphere ().\nAn initial 12-lead ECG showed a slow nodal escape rhythm with an HR of 54–83 BPM (). A second ECG, done later the same day, showed atrial ectopic tachycardia (AET) with alternating 1:1 and 2:1 conduction with an HR of 180 BPM, probably originating from an ectopic focus in the left atria in the region of the pulmonary vein insertion. The ECG was repeated while intravenous adenosine was administered and showed an atrial rhythm with an HR of 210–220 BPM with positive P-waves in leads II and III, suggesting that the patient’s arrhythmia was either a slow atrial flutter or AET. Transthoracic echocardiography (TTE) was normal apart from the discovery of a small patent foramen ovale (PFO). Transoesophageal echocardiography (TEE) confirmed the PFO and showed a normal left atrium with a normal auricle without any thrombus.\nDC-cardioversion with a voltage of 20 J was attempted, resulting in asystole. Advanced cardiopulmonary resuscitation was begun with return of spontaneous circulation after 3 min. A temporary ventricular pacing (VVI) pacemaker (PM) was inserted and later replaced with a permanent epicardial VVI PM with a backup rate of 80 BPM.During the procedure, it was not possible to pace the right atrium, despite attempts from several positions and maximum output. Pacing on the left atrium was not attempted. No atrial contraction was observed on TEE during atrial or ventricular pacing ().\nIt was concluded that the presenting cerebral insult originated from mural thrombus formation in the left atrium secondary to atrial standstill. Subsequently, the patient started oral anticoagulation. It was decided not to close the PFO.\nHolter monitoring carried out 2 months after discharge showed a predominantly V-paced rhythm of approximately 80 BPM. Holter monitoring also revealed multiple episodes of narrow complex tachycardia with an irregular HR of 160–200 BPM, each lasting under 1 min. The patient was subsequently treated with metoprolol, 1 mg/kg once daily. Once on medication, Holter monitoring showed a predominant V-paced rhythm with an HR up to 112 BPM and an occasional nodal escape rhythm.\nAt 12 months follow-up, the patient was in good condition without any complaints of fatigue, palpitations or other neurological or cardiac symptoms and was physically active several times a week on the same level as her peers. Holter monitoring showed 99% V-pace with approximately 80 BPM without episodes of tachycardia. TTE showed a left ventricle with normal dimensions and normal systolic function. The PFO was not visible. The patient still received metoprolol and warfarin treatment. |
A 31-year-old lady came to us with evidence of spontaneous abortion at 14 weeks of her pregnancy, which was conceived following in vitro fertilization. An ultrasound scan done showed an empty uterine cavity, indicating a complete abortion. She had fever at the time, and hence a course of antibiotics was given. Her hemoglobin levels were low, for which she was given a unit of packed red blood cells.\nShe was a booked case with us and had a past history of two episodes of ascites (OHSS) following the embryo transfer. The first episode was within 12 days of embryo transfer, and the second episode was at 9-10 weeks of gestation. Both episodes were diagnosed as OHSS and treated symptomatically with albumin infusion. At 14 weeks of gestation, she had fever and recurrence of ascites. Ascites did not subside even with albumin and Cabergoline; hence other causes of ascites were evaluated by Mantoux test and chest X-ray, which were negative for tuberculosis.\nHer bleeding per vaginum persisted, for which a scan was done again which showed some retained products of conception for which she underwent dilatation and evacuation. The tissue obtained was sent for histopathology examination and came back as only degenerated products of conception and negative for mycobacterium tuberculosis by PCR.\nWhen the ascites did not disappear after the regular treatment, the ascitic fluid was tapped thrice. It was green in color, leading to suspicion of presence of bile salts or pigments in it though her liver function tests were normal. When analyzed for the same, there were no bile salts or pigments found. Upon culturing it, no growth was found after 7 days. The ascitic fluid was negative for malignancy, and Ca 125 was normal. Her hematocrit persisted at the same values (19.8%) as before. She had lost significant weight within two weeks. The ascitic fluid was further investigated with acid fast staining, which showed no acid fast bacilli. A PCR sent for mycobacterium tuberculosis came back negative. Further evaluation showed its Adenosine DeAminase (ADA) level was 78 IU/L and rose to 110 IU/L in 3 days (normal: <39 IU/L). Based on the ADA levels, she was started on antitubercular treatment with HRZE (Isoniazid + Rifampicin + Pyrazinamide + Ethambutol), which finally resolved the ascites within a week. |
A 12-year-old male patient reported to the Department of Pedodontics and Preventive Dentistry with a chief complaint of broken tooth in the upper front tooth region due to trauma, 1 week back. The patient gave a history of prediagnosed case of MMD with hemiplegia of the right side.\nAt the age of 1½ years, the patient suffered from pulmonary tuberculosis and completed anti-tuberculosis treatment for the same. At the age of 4 years, the patient experienced sudden onset of pain and weakness in the right lower limb, which was associated with difficulty in holding objects with the right hand and raising the right arm above the head. The MRI of brain showed focal right frontal infarct with left cerebral atrophy and an attenuated appearance of intracanal arteries suggestive of effects of MMD. Symptomatic medical treatment was provided to the patient. The patient had no past dental treatment exposure. There is no related family history.\nThe patient appeared underweight with a lean body type. The general physical examination revealed hemiplegia of the right side, affecting both upper and lower limbs ().\nThe extraoral examination revealed a symmetrical face with competent lips and normal mouth opening. On intraoral examination, permanent dentition was present with Angle's Class I malocclusion. Ellis Class I fracture was present in the upper right central incisor whereas Ellis Class II fracture was present in the upper left central incisor ().\nThe dental treatment plan focused on providing preventive as well as restorative care to the patient while simultaneously providing proactive counseling to both the parents and the patient. Behavior management was an integral part of our management. Oral hygiene measures were instructed to the patient and modified angled toothbrush with fluoridated toothpaste was prescribed due to the lack of manual dexterity. Along with this, diet modification was done by recommending intake of noncarious dietary food items such as fibrous food, nuts, and fruits. The medical consent was obtained from the pediatrician following which oral prophylaxis and composite restoration of upper central incisors were done (). |
A 15-year-old girl presented with sudden pain and sense of giving way of the right knee for 1 week. She underwent total meniscectomy for the lateral meniscus at another hospital 8 years ago. Physical examination showed mild effusion, no joint line tenderness, and negative McMurray test. Her knee was stable and showed a full range of motion. Radiographs and MRI showed a displaced, large osteochondritis dissecans in the lateral femoral condyle. Autogenous osteochondral grafting using multiple plugs was performed immediately, and lateral meniscal transplantation was undertaken 5 years after the osteochondral grafting.\nFor meniscal transplantation, the patient was positioned supine on the operating table and the right knee was placed in the figure-of-4 position. The size of the meniscal allograft was measured using the radiographic method proposed by Pollard et al. After thawing the lateral meniscal allograft, redundant soft tissues of the allograft were excised carefully and a 9-mm bony bridge connecting the anterior and posterior horns of the lateral meniscal allograft was prepared. A 1-mm wire was bent along the medial border of the bony bridge and the periphery of the lateral meniscal allograft (). The prepared wire was introduced through the lateral mini-arthrotomy while the patellar tendon was retracted medially. The straight part of the wire was placed along the lateral tibial spine (). However, some portion of the round part of the wire was extruded from the anterolateral margin of the lateral tibial plateau. The straight part of the wire was moved more medially and rotated internally. The round part of the wire was placed accurately along the anterolateral margin of the lateral tibial plateau (). The direction of the straight portion of the wire was marked with electrocautery. After removal of the wire, a bony trough was made along the marked line using a U-shaped chisel. The rest of the transplantation procedure of the lateral meniscal allograft was done in the usual manner.\nAt 1 year after surgery, she had no pain or effusion or pain during McMurray test on the right knee. Her Lysholm score was 86 and Tegner activity scale was 4. Follow-up MRI showed low-signal intensity of the midbody of the allograft that was positioned adequately without extrusion (). |
A 2-year, 4-month-old Afro-Caribbean boy presented to our clinic with a history of abnormal gait and a size discrepancy in the legs noticed by the mother. He was born at term by normal spontaneous vaginal delivery following an uneventful pregnancy. No abnormalities were noted at birth, and he had achieved normal developmental milestones. From the age of 6 months, the patient’s mother noticed that his left leg appeared different to the right; as he grew, it appeared that the right leg grew faster than the left. This difference became more apparent when the child started walking, and the mother noticed the gradual onset of a painless limp and leaning towards the left side. She also noticed a difference in the size of the feet when the child started using footwear, with the left shoe always being too big for the left foot. At this stage, the mother noticed that the child was occasionally tripping when walking or running and that the lower left leg was much thinner than the right.\nOn review in our clinic, the patient was walking and running appropriately. There were no tripping, functional nor balance difficulties. Leg lengths, foot lengths and thigh circumferences were equal; however, there was an obvious asymmetry of the calves, with the left smaller than the right. We found a 4-cm difference in calf circumference measured at a fixed point (4 cm) below the lower pole of the patella. Neurological, vascular and cutaneous examination of the lower limbs was normal. There were no abnormalities of upper limbs or spine.\nRadiographs of the lower limbs revealed normal bony architecture; renal ultrasound was normal, as was magnetic resonance imaging (MRI) of his brain and spine. MRI of his lower limbs (Figs. , , ) confirmed complete absence of the soleus and medial gastrocnemius, with a rudimentary lateral gastrocnemius arising from the semimembranosus to form the Achilles tendon. The MRI also revealed hypertrophy of the deep posterior compartment and peroneal compartment muscles on the left side. Creatine kinase was mildly raised at 193 IU/L (normal 0–170 IU/L), but all other blood markers and genetic testings were normal. Static and dynamic gait analysis revealed no significant difference between feet, including foot phasing, calcaneal stance angles and rearfoot/forefoot angles (Table ). We counseled the parents and reassured them that no intervention was required. At latest follow-up, the child was 5 years and 4 months old and progressing well clinically. Although there remains an obvious difference in calf size (Figs. , ), there are no functional difficulties. The patient’s parents gave informed consent prior to our writing this report. |
A 68-year-old female with a long history of intermittent back pain had a relatively acute onset of severe low back pain radiating down the posterior left buttocks and left leg for three weeks. She denied any trauma or other possible inciting factors. The pain had been getting progressively worse, and she also had developed numbness in the left S1 and S2 dermatomal distributions. An epidural steroid injection performed at a local facility provided partial relief and lasted for approximately 24 hours. The pain was described as intense, electrical pain different from any pain she had ever felt in terms of distribution and severity. Due to the exacerbating factors including standing and walking, thus, she presented to clinic in a wheelchair and preferred to lay in the fetal position. She had presented to the emergency department two days prior to neurosurgery consultation due to continued worsening of pain and numbness.\nA thorough neurological examination was negative except for a positive straight leg raise on the left.\nLumbar spine X-ray did not reveal any abnormal alignment or transitional anatomy (). Lumbar spine MRI (Figures and ) revealed a cystic mass with similar intensity to cerebrospinal fluid (T2 hyperintense/T1 hypointense) located on the dorsolateral left side of the sacral spinal canal inferior to the left S1 pedicle. It was causing significant compression on the exiting S1 and traversing S2 nerve roots. Sacral arachnoid or Tarlov cyst, cystic schwannoma, and synovial cyst were all included in the differential. She was referred to neurosurgery for urgent evaluation of her severe pain in the setting of an intraspinal sacral mass. After obtaining a clinical history from the patient and further reviewing of the MRI, the imaging diagnosis was questioned. A synovial (or juxtafacet) cyst was thought possible even though it was not in close proximity to the facet joint and was located in the sacral spinal canal. Further, the cyst contents were slightly heterogeneous and suggestive of small hemorrhage, and the acute clinical history was atypical for an arachnoid cyst or schwannoma. There was no connection to a facet joint noted on the imaging.\nThe patient was taken to surgery for a left S1 and partial left S2 hemilaminectomy. An epidural, partially hemorrhagic cyst inferior to the left S1 pedicle was dissected off the dura (). The cyst was causing severe compression of the exiting and traversing nerve roots as seen on the preoperative MRI. There was no obvious connection to the left L5-S1 facet joint. Pathology revealed synovial cyst. The patient's left leg pain was significantly improved, and she was dismissed, on postoperative day two, neurologically stable. |
A 38-year-old female had suffered from congenital insensivity to pain with anhydrosis since birth. At age 27, she was admitted to our hospital due to swelling of the right hip joint without a cause. A fracture of the right femoral head fracture was observed in a hip X-ray, and she was diagnosed as having a Charcot hip joint because the collapse of the right femoral head worsened. We followed up this patient carefully, with a conservative treatment, because it was possible for her to walk on one crutch. When she was 29 years old, she suffered from Charcot spine; spinal fusion was performed six times due to collapse of the spinal joint. She was admitted to our hospital again because she could no longer stand, due to limb length discrepancy.\nClinical examination revealed a swelling of the right hip joint and limping due to a shortening of the right hip joint, but the hip was almost pain free. A hip X-ray revealed the disappearance of the right femoral head, destructive changes in the right acetabulum, and a 36 mm shortening of the right hip joint ().\nWe used a posterolateral approach, performed in the lateral decubitus position. On the acetabular side, we used medial protrusion technique because acetabular bone defect of the weight bearing portion was so severe []. The cementless acetabular cup was press fit in accordance with the preoperative plan. In addition, we inserted dome screws into the cementless acetabular cup in order to achieve initial fixation. On the femoral side, we performed a gentle rasping technique to prevent femoral diaphyseal fracture and inserted the cementless femoral implant into the femoral canal. After inserting the implant, we performed an impingement test to prevent bone-to-implant or bone-to-bone impingement. Finally, the short external rotator muscles separated from the proximal femoral bone were completely repaired to their original position. The postoperative rehabilitation schedule was full weight bearing after the operation; a hard abduction brace was used to prevent postoperative dislocation.\nTwo weeks after surgery, although she had been put in a hard abduction brace, postoperative posterior dislocation occurred when she pulled on her trousers and twisted her right hip joint. After closed reduction, we supervised the posture of dislocation and kept the patient in a hard abduction brace continuously. However, posterior dislocation occurred 5 times in the six months after surgery. Afterwards, with continuous use of the hard abduction brace, the posterior dislocation did not recur, and it was possible for her to walk with a lofstrand cane. Today, 9 years after surgery, the patient can walk with the lofstrand cane without causing implant dislocation, although it had been impossible for her to walk before surgery; X-rays revealed no clinical signs of implant loosening compared with the postoperative X-rays (). |
A 78-year-old male presented with a gradually increasing painful swelling over left side of the neck since 8 years. Physical examination revealed a mass in the lateral region of the neck behind the sternocleidomastoid muscle. The mass measured 7 cm in diameter and was tender and non-mobile. The overlying skin was unremarkable. Facial nerve function and the remainder of head and neck examination were normal.\nFine needle aspiration cytology (FNAC) was performed, which was suggestive of a cystic lesion of the salivary gland. Computed tomography (CT) scan showed centrally necrotic rounded mass lesion arising from parotid gland []. The lesion was almost completely replacing the outer lobe of left parotid gland and the inner lobe was normal. Anteriorly the lesion was seen abutting the masseter muscle and fat planes. CT findings suggested neoplastic lesion of the left parotid gland with involvement of bilateral submandibular nodes and left level II lymph nodes. Patient was taken up for surgery. Intra-operatively a 7 × 6 cm mass was seen involving the left parotid gland and all the branches of the facial nerve except the cervical branch. The facial nerve along with its branches was traced anteriorly. The entire tumor along with the parotid gland and the facial nerve was excised en block. The cervical branch of facial nerve was preserved.\nHistopathological examination revealed a tumor composed of spindle shaped cells with elongated hyperchromatic nuclei, arranged in long and short fascicles []. At places cells were arranged around hyaline bands in the form of cords and at places they were arranged in whorls. Some of the tumor cells had epitheloid appearance, while some cells had marked nuclear pleomorphism with tumor giant cells. In some areas, chondroid differentiation was seen. The stroma showed extensive areas of necrosis and hemorrhage. The blood vessels showed hyalinized walls. Mitotic figures were more than five per high-power field and areas of compressed parotid gland were present at the periphery of tumor.\nImmunohistochemically, the tumor cells showed diffuse and strong positivity for S-100, (Dako), however, were negative for Pan-cytokeratin (Dako, AE1/AE3), Desmin (Dako, D33), and Human Melanoma Black (HMB) 45 (Dako) [] and Vimentin (Dako). With the above features, a diagnosis of MPNST was made.\nPost-operatively, the patient had facial palsy [] and distant metastases were not found on whole body scan after 6 months of follow-up. |
In October 2012, a 46-year-old woman was referred to our center for the presence of a large mass (50 × 70 mm) in the superior lobe of the left lung with homolateral pleural effusion. The patient was never smoker, without family history of cancer and without comorbidity. The cytological diagnosis was made using a CT-guided fine needle aspiration of the primary tumor and revealed an adenocarcinoma of the lung (TTF1+, CK7+) with the EGFR ex19del mutation. A PET-CT demonstrated the presence of liver and bone metastases and a nodule in the right breast, confirmed as a metastasis by fine needle aspiration. The patient received zoledronic acid 4 mg every 28 days and gefitinib 250 mg daily since November 2012 obtaining a partial response (PR). In August 2013, a disease progression (PD) was documented, with an increase in size of the primary tumor and size and number of liver metastases. A brain MRI revealed the presence of two cortical nodules, which were treated with stereotactic radiotherapy. The patient was enrolled in the IMPRESS trial and received 6 cycles of cisplatin and pemetrexed plus gefitinib obtaining again a PR that lasted until June 2014. Thereafter, a new lung metastasis appeared in the superior lobe of the left lung and the mammary nodule increased in dimensions. From June 2014 to December 2014 the patient received erlotinib 150 mg daily obtaining an initial stabilization of the disease (SD); however, within 6 months, she experienced again a PD with the increase of the mammary nodule and the appearance of a new bone metastasis in the sacrum. In December 2014, EGFR ex19del and T790M mutations were detectable in a new needle biopsy of the primary tumor; only at this time a digital PCR-based method was available for the analysis of circulating tumor DNA (ctDNA). Briefly, the method was optimized in order to recover a suitable amount of ctDNA for molecular analysis from 3 ml of plasma using the QIAmp Circulating Nucleic Acid Kit (Qiagen®, Valencia, CA). ctDNA was examined using the Prime PCR Probe Assay on a QX100™ Droplet Digital™ PCR System (BioRad®, Hercules, CA) for EGFR mutations (ex19del, T790M, and C797S) []. The ctDNA sample was considered as EGFR mutant when at least one droplet was above the fluorescence intensity threshold of 3000 and results were reported as copies/ml. The first plasma specimen was obtained in December 2014 and confirmed the presence of ex19del and T790M mutations (480 and 260 copies/ml, respectively; Fig. ). The patient was treated with atezolizumab from March to May 2015 and received stereotactic radiotherapy on the lung primary tumor and on metastases of the left superior lobe, breast and bone (sacrum and D10). Due to PD, the patient was given afatinib 40 mg daily from June 2015 to September 2015, but she experienced a further PD with an increase in size of the primary tumor and lung nodules, the appearance of new multiple bilateral lung metastases, lymphangitic infiltration and liver metastases. At this time, the presence of ex19del and T790 M was again demonstrated in a biopsy of a liver metastasis. Since osimertinib was available, the patient was enrolled in the ASTRIS trial and given 80 mg daily starting from October 2015 with an immediate clinical benefit. At the first evaluation a PR was documented with disappearance of most of the lung nodules and lymphangitic infiltration, reduction of the primary tumor and of liver metastases as well. A disappearance of ex19del or T790M was demonstrated in ctDNA in a blood sample obtained in October 2016. However, in April 2017, ex19del appeared again (400 copies/ml) and in July 2017 it increased to 1000 copies/ml, while T790M was also detectable (330 copies/ml, Fig. ). Then, ex19del and T790 M continued to increase to, respectively, 1600 and 510 copies/ml in October 2017, 2100 and 550 copies/ml in November 2017, and 6900 and 1900 copies/ml in December 2017.\nA radiological progression was demonstrated with increase in size and number of liver metastases in December 2017. The patient underwent a new liver biopsy that confirmed the PD and the presence of ex19del and T790M, whereas the ctDNA showed also the appearance of C797S mutation (170 copies/ml), in addition to ex19del and T790M. The patient started chemotherapy with carboplatin and pemetrexed and in February 2018 she obtained an SD associated with a drop of ex19del (800 copies/ml), T790 M (380 copies/ml), and C797S (90 copies/ml) and then a PR in May 2018, with disappearance of C797S and reduction of ex19del (760 copies/ml) and T790M (90 copies/ml). In July 2018, however, ex19del strongly increased to 2200 copies/ml, even though T790M and C797S were undetectable. Finally, in October 2018, when a PD was documented, ex19del increased to 5100 copies/ml, while T790M and C797S appeared again in plasma with 600 and 180 copies/ml, respectively (Fig. ). At this time a NextSeq 550 NGS platform (Illumina®, San Diego, CA) was available to analyse ctDNA by the AVENIO ctDNA Expanded Kit (Roche®, Pleasanton, CA). A plasma sample collected in December 2018 and analysed by NGS confirmed the presence of the ex19del, T790M and C797S and found, in addition, EGFR and c-MET amplifications, which were not present in tissue in the last re-biopsy of December 2017. |
A 31-year-old woman with no significant past medical history presented to our emergency department complaining of a constant headache for the previous 4 days. The headache had begun approximately 6 h after receiving epidural anesthesia for labor. The documentation from the anesthesia service that day reported the use of a 17-gauge Touhy needle to enter the subdural space in the lower lumbar spine and the placement of a 19-gauge epidural catheter. No complications were reported with the procedure, and specifically, there was no mention of inadvertent dural puncture. The patient had an unremarkable delivery of a healthy infant at 38-weeks gestation later that day.\nThe patient described the headache as constant and occipital with some radiation to the frontal area. The headache was worse when upright and partially relieved in the supine position. She reported taking acetaminophen/butalbital/caffeine and ibuprofen with little relief. She had no associated vomiting, fever, or changes in her hearing or vision. She denied any photophobia or focal weakness or numbness. She was afebrile on physical exam, with pulse and blood pressure within the normal range. Her exam was notable for a normal neurologic exam including cranial nerves and no neck stiffness. The patient was tentatively diagnosed with a PDPH. After evaluation by the anesthesia service, she was admitted for pain control and possible placement of an epidural blood patch the next day. A computed tomography (CT) scan of her head was obtained prior to admission to evaluate for other possible causes of the headache (Fig. ). This CT identified bilateral parafalcine subdural hematomas measuring 7 mm in thickness on the left and 3 mm thickness on the right. There was no associated mass effect.\nThe patient was admitted to the intensive care unit and started on levetiracetam for seizure prophylaxis. Neurosurgical consultation advised observation, and a repeat CT scan of the head the next day showed no significant change in the hematomas. The patient also received an epidural blood patch the next day with no improvement in the headache. A head CT performed on hospital day 3 showed a decrease in the size of the hematomas, and the patient was discharged on levetiracetam for seizure prophylaxis for 1 week.\nISH occurring after dural puncture is extremely rare. Only sporadic case reports and a few small case series have described this condition [–]. Any procedure that results in spinal dural puncture will theoretically predispose to the development of an ISH. ISH has been described following epidural and spinal anesthesia, as well as lumbar puncture, myelography, epidural steroid injection, and after implantation of an intrathecal drug delivery device and a spinal cord stimulator [–]. The incidence of ISH specifically caused by epidural anesthesia used in obstetric practice has been estimated to be 1:500,000 [].\nThe same mechanism has been postulated for both PDPH and ISH []. The leakage of cerebral spinal fluid (CSF) from the dural puncture site may continue for several weeks, causing reduction in CSF volume []. This results in lower intraspinal and intracranial pressure, leading to relative ventricular collapse and caudal movement of the spinal cord and brain. As a consequence, the dura, pain-sensitive structures, cranial nerves, and subdural bridging veins are stretched. This may ultimately result in a tear of the bridging veins and consequently an ISH. Risk factors associated with ISH after dural puncture include excessive CSF leakage from multiple punctures in large needle use, pregnancy, coagulopathy, cerebral vascular abnormalities, dehydration, brain atrophy, and alcoholism [–].\nThe duration of time from dural puncture to the diagnosis of ISH ranges widely from 4 h to 29 weeks []. In one case series, 37% of cases were diagnosed within 1 week of dural puncture, and 85% were diagnosed within 1 month []. A headache, most often diagnosed as PDPH, is the main presenting symptom [, –]. Other reported symptoms and signs present at the time of diagnosis are listed in Table [, , ]. Reported rates of surgical intervention for ISH after dural puncture vary from 9 to 80% [, , ]. In general, surgical intervention for ISH is indicated if the hematoma thickness exceeds 10 mm, there is a midline shift of greater than 5 mm, or there is neurologic deterioration []. Furthermore, some have advocated for the use of epidural blood patching in the treatment of ISH caused by dural tears resulting in chronic CSF leaks [, ]. A full recovery is reported in over 80% of patients, with death reported in 7–10% of cases [–].\nHeadache in the postpartum period is common, occurring in 39% of women []. The majority of these headaches are benign primary headaches, such as migraine and tension type []. Secondary headaches in the postpartum period are typically due to obstetric or anesthetic complications, or the hypercoagulable state after delivery (Table ). Our patient was initially misdiagnosed as having PDPH, similar to many previous reports of this condition. PDPH is defined as a headache that develops within 5 days of dural puncture that significantly worsens soon after sitting upright or standing and improves after lying horizontally []. PDPH is more likely to occur in young women of low body mass as compared with other patients []. An epidural blood patch is considered the gold standard for treatment of PDPH, with a success rate of 70–90% []. Over 85% of patients report resolution of PDPH within 6 weeks regardless of treatment [].\nThe incidence of ISH after dural puncture is probably underreported since many of these patients are treated as PDPH with the eventual resolution of their symptoms. When to obtain brain imaging studies in the assessment of a likely PDPH is unclear. A reasonable approach would be to consider imaging in patients that (1) have a postural headache lasting more than 1 week, (2) do not improve or have worsening of their headache after an epidural blood patch, (3) report a change in the headache from postural to non-postural, or (4) develop other neurologic signs or symptoms with the headache []. |
A 30-year-old female presented following a fall during a workout session, when her leg “gave out.” As an avid runner, she noted progressive worsening of her daily activities and exercise intolerance due to pain, numbness, and a burning sensation in her right lower extremity, symptoms that had been present for 12 years. She worked as a physical therapist and while assessing her pulse a decreased ankle-brachial index was noted. She had a history of a tarsal tunnel release 14 years ago for presumed tarsal tunnel syndrome followed by a four-compartment fasciotomy for supposed chronic exertional compartment syndrome (CECS) 10 years ago.\nPhysical exam of the right leg demonstrated a normal femoral pulse, while the popliteal pulse was absent with diminished posterior tibial and dorsalis pedis pulses. Tenderness to palpation was elicited overlying the right tibia.\nRadiographs showed no evidence of a fracture. A duplex ultrasound demonstrated normal triphasic waveforms extending from the right superficial femoral artery caudally to the proximal popliteal artery. Distally, the native popliteal artery was not clearly identified. Multiple collaterals within the popliteal fossa were visualized (). CT angiography (CTA) of the lower extremities showed complete occlusion of the proximal right popliteal artery with significant reconstitution of flow distally from robust collateral vessels (). A band of soft tissue density was visualized anterior to the right popliteal artery just distal to the occlusion, suggestive of a fibrous band contributing to the occlusion (). A 3D volume reconstruction obtained from axial source CTA images showed occlusion of the right popliteal artery with adjacent collaterals (). The imaging and clinical findings correlated with PAES type IV.\nBased on these findings, the entrapment was not amenable to endovascular repair with minimally invasive techniques. Vascular surgery recommended right popliteal artery entrapment release coupled with popliteal-to-popliteal artery bypass and right lower extremity venous mapping. The two heads of the gastrocnemius muscles were split to expose the distal aspect of the popliteal artery. Small fibrous bands were removed off the popliteal artery followed by insertion of a bypass graft (). Flow to the distal limb was excellent demonstrated by palpable pedal pulses on exam. Follow-up three weeks later showed palpable dorsalis pedis and posterior tibial pulses bilaterally, and a follow-up Duplex ultrasound of the right lower extremity revealed a patent bypass graft. The patient noted resolution of her original symptoms of lower extremity burning and numbness. |
A 59-year-old woman presented in July 2017 with extensive bleeding from her ileostomy site. Her history included locally advanced bladder cancer for which she had undergone pelvic exenteration and ileal conduit formation in November 2015. At that time, she had a known primary lung adenocarcinoma as well, but had no known liver metastases or other liver disease. Intravenous contrast-enhanced CT of the abdomen and pelvis performed in January 2016 raised the possibility of cirrhosis; however this was not biopsy-proven. In April 2016, she began to notice intermittent bleeding from her stoma which was initially thought to be mechanical tissue breakdown from the stomal flange. Concern for hepatic encephalopathy was raised when she had her first episode of confusion in December 2016. At that time CT of the abdomen and pelvis demonstrated strong radiographic suspicion for cirrhosis together with prominent vessels surrounding the urinary diversion site suspicious for portal hypertension. Despite not having a tissue biopsy, she was diagnosed clinically with cryptogenic cirrhosis in May 2017 during a hospitalization for fatigue, anasarca, and altered mental status. An upper endoscopy performed in June 2017 demonstrated portal hypertensive gastropathy but no esophageal varices.\nUpon presentation to the Emergency Department in July 2017 she had significant hemorrhage from her stoma resulting in hemodynamic instability. She was anemic with a hemoglobin of 8.3 g/dL that improved to 9.4 g/dL after blood transfusion, but gradually fell to 8.2 g/dL by the time of the procedure. Her MELD score was retrospectively calculated to be 19 at presentation, with an INR of 1.5 and total bilirubin of 4.3 mg/dl. She was emergently taken to the interventional suite for embolization with or without portal venous decompression via portosystemic shunt formation. A review of intravenous contrast-enhanced CT imaging showed extensive venous varices around the stoma involving the abdominal wall with a large draining varix arising from the portal system, likely the inferior mesenteric vein []. Also visualized was a variceal connection to the right common femoral vein. The portal and mesenteric veins were noted to be patent. Multiple approaches were considered for this patient. The transjugular intrahepatic portosystemic shunt (TIPS) and transjugular transhepatic approach with portosystemic shunt creation offered the benefit of portal decompression; however, the patient's recurrent hepatic encephalopathy was felt to be a relative contraindication. Transjugular transhepatic approach without formation of a permanent portosystemic shunt was also considered, since it would eliminate the risk of progressive hepatic encephalopathy. Percutaneous transhepatic approach would also eliminate the risk of progressive hepatic encephalopathy but was believed to pose increased risks of hepatic injury and bleeding. Transsplenic venous access to the portal venous system was considered as a viable, albeit technically challenging, option. The superficial nature of the abdominal wall stomal varix presented a less challenging and seemingly more time-efficient approach for access and was chosen as the target.\nUsing a micropuncture kit, the peristomal varix was directly accessed under ultrasound guidance and a micropuncture sheath was placed. Venography was performed and showed a large variceal collateral conglomerate around the stoma with variceal anastomosis with the right common femoral vein []. A wire was advanced and a 5F sheath was secured over the wire. A Kumpe catheter was introduced and advanced into the distal intra-abdominal aspect of the large draining varix. Catheter position was confirmed with repeat venography, and multiple coils were deployed []. This was followed by Gelfoam embolization. Postembolization venography showed sluggish flow in the draining varix with multiple filling defects within the visualized collaterals consistent with embolization []. The coils remained well-situated after placement and there was no evidence of migration. To ensure that there was no filling from the systemic venous system, the right superficial femoral vein was then accessed with a micropuncture kit and a femoral-iliac venogram and IVC venogram were both performed. These demonstrated brisk flow from the right common femoral vein through the iliac system and into the IVC. There was no filling of the stomal variceal collaterals visualized []. Hemostasis was thereby achieved, and the patient became hemodynamically stable shortly thereafter. At 6-month follow-up time no further imaging had been performed and the patient had not had any further episodes of hemorrhage from the ileostomy site |
A 2-year-old boy reported to the Pedodontic Emergency following trauma due to self-fall and with the complaint of loss of upper front teeth. A thorough history was taken and medical examination was done to rule out any head and neck injuries and other complications. Dental examination revealed the de-gloving injury in the region of maxillary anterior teeth with severe intrusion of 51, 52, and 61 []. Radiographic examination confirmed the intrusion of 51, 52, and 61 with 61 impinging on the developing permanent tooth which was in Nolla's stage V (crown almost completed) []. On examination of the region under relative analgesia (conscious sedation with 60% NO2 and 40% O2), multiple vertical fractures in the labial cortical plate were noticed, which extended from the socket crest up to the nasal notch []. The challenge in treating this condition was to bring about favorable healing of the fractured fragments without any damage to the underlying permanent tooth buds. With such severe and multiple fractures of the labial cortex, the developing tooth buds were left unsupported and literally floating in the mass of blood clot and surrounding damaged connective tissue. Reduction of the fragments could well be done, but immobilization of these fragments to promote healing without deformities was a difficult task. Further complications could be because of the differential healing of the tissues. The connective tissues would heal fast and the bone would take a longer time to heal. The resulting situation would be that the fast healing connective tissue would occupy the spaces between the fractured fragments and result in mal-union and oro-facial deformities.\nExtraction of 51, 52, and 61 was planned as the supporting periodontium was severed. Reduction of the fractured socket walls was done under cautious digital pressure. Placement of mini plates and screws were contraindicated due to the presence of permanent tooth buds. Therefore, for relative immobilization and to promote favorable healing, a resorbable membrane was planned to be placed. The GTR membrane was the best choice which would meet both the requirements. The GTR membrane (PerioCol® -GTR: Sterile collagen periodontal membrane: Eucare Pharmaceuticals (Pvt., Ltd.), India) was trimmed to match the anatomy of the region [] and was placed between the reduced fracture fragments and the injured gingiva []. This was followed by the placement of sutures and periodontal dressing (Reso Pac® Hager Werken GmbH & Co. KG, Germany). Parents were instructed to maintain meticulous oral hygiene. The healing was uneventful and at the end of 10 days the sutures were removed.\nThirty days follow-up showed favorable healing of the maxillary anterior labial cortex and the overlying soft tissue []. Postoperative radiograph shows favorable healing []. There was a need of a space maintainer following teeth loss. Cooperation of the patient was compromised as the child was in precooperative stage. Hence, an indirect Ribbond supported fixed space maintainer was planned []. |
This case is of a 55-year-old female who initially presented with a palpable left neck mass. The mass had been noted by the patient 8 years ago and had progressively grown in size. A computed tomography (CT) of the neck was obtained, which demonstrated a 3.5 × 2.3 × 4.6 cm lesion deep to the left sternocleidomastoid (SCM) muscle in addition to a smaller 1 × 1 × 1.8 cm left posterior neck-enhancing mass. A fine needle aspiration (FNA) had been performed 2 years prior at an outside hospital with pathology indicating a low-grade spindle cell proliferation. She was thus referred to our institution for further evaluation. At the time of initial presentation to our group, the patient had noted dysphagia, left ear ache and tinnitus, and neck pain localized to the two masses. A positron emission tomography (PET) CT was performed, which demonstrated increased fluoro-2-deoxy-\nd\n-glucose uptake in the high cervical mass and, to a lesser degree, in the mass located within the posterior triangle of the neck (\n). The outside tissue blocks were reviewed at our institution and felt to be consistent with a peripheral nerve sheath tumor compatible with schwannoma from both lesions. Because of the patient's ongoing symptoms and because malignancy could not be completely excluded based on the results of the prior FNA, the decision was made to proceed with surgical excision of both lesions. Prior to proceeding, a magnetic resonance neurogram was obtained (\n). Tractography demonstrated that the two lesions appeared to originate from the spinal accessory nerve and that apparent diffusion coefficient values were elevated in both masses, supporting the diagnosis of a less aggressive tumor (\n).\nThe patient was taken to the operating room for surgical resection. The patient was positioned with her head turned slightly to the right with the neck extended (\n). The smaller lesion was approached first through the posterior triangle of the neck. Stimulation mapping of the tumor was conducted. The tumor was found to be located on the distal spinal accessory (cranial nerve XI) nerve, with evidence of trapezius activation with nerve stimulation (\n). After identifying no overlying nerve fibers, the tumor was removed en bloc. A separate incision was made in the upper cervical region to approach the larger second mass located lateral and deep to the SCM muscle, which was reflected medially (\n). During dissection of the tumor away from the nerve, motor evoked potentials (MEPs) to the trapezius were lost. As the tumor was of significant size, view of the proximal aspect of the afferent nerve was initially obstructed. Distally, the tumor was mapped, and the fascicle of origin was identified, which appeared to activate the SCM. After significant debulking of the mass, the proximal fascicle of origin was identified but did not provide any muscle activation after stimulation. The tumor was therefore removed in its entirety. All parameters for brachial plexus monitoring remained stable.\nPathology for both lesions was consistent with schwannoma without malignant features. Next-generation sequencing analyzing the coding regions of 479 cancer genes as well as select introns of 47 genes using the UCSF 500 Cancer Gene Test revealed a small in-frame insertion at codon p.R177 of the Sox 10 gene. There were no identifiable alterations in NF1, NF2, LZTR1, SMARCB1, and TRAF7 genes. Despite the change in MEPs, the patient was noted to be full strength in all muscle groups in the left upper extremity including shoulder shrug and head turning immediately postoperatively. At follow-up, her neck pain and prior dysphagia had improved significantly. |
A 41-year-old North African woman with a history of chronic anemia with iron supplementation intake presented to our emergency department. She had massive rectal bleeding.\nShe had an iron deficiency anemia. She did not complete the investigations so the cause was not yet diagnosed. She had no chronic bleeding, neither gynecologic nor digestive. She was treated by oral iron with no regular follow up. She had no past surgical interventions, and no other medical condition. She had no contact with animals. There were no medical conditions in her family, no cancers and no current infections. No one in her family or neighborhood was diagnosed with tuberculosis.\nIn our admission unit, her hemodynamic parameters showed that she was in shock: her blood pressure was 80/40 mmHg and her pulse was up to 120 beats per minute. She was pale, her conjunctivas were discolored and her limbs were cold. While examining her, we found profuse rectal bleeding with clots. A quick abdominal and pelvis examination including proctologic examination appeared normal. She was transferred to our intensive care unit and monitored.\nNoradrenaline was administered because the crystalloid fluids and the transfusion were not sufficient to compensate for her blood loss. Several blood tests were conducted, including blood count and clotting tests. Her hemoglobin was 8 g/dl and hematocrit was 29 %. The rest of the formula and the clotting tests returned normal results as shown in Table .\nShe first underwent an esogastroduodenoscopy, to identify the source of the bleeding. No abnormality was detected in the said procedure: the esophageal and gastric mucosa were normal, no ulcerations or traces of blood were found, and the duodenal mucosa was also explored and appeared normal. A colonoscopy could not be carried out because of the profuse rectal bleeding. She underwent a computed tomography (CT) of her abdomen which revealed a wall thickening in the terminal ileum with hypervascularization and regional adenomegalies (Figs. , , and ). This aspect suggested either a tumor or an inflammatory bowel disease.\nBecause her bleeding and her hemodynamic collapse persisted, it was decided to proceed with surgery with the aim of diagnosis and treatment. She underwent exploratory laparotomy immediately. It confirmed the wall thickening in her ileocecal junction. Her appendix was inflamed. An ileocecal resection was performed with an ileocolic anastomosis, removing 10 cm from her ileum. The specimen was sent for histological examination. Gross examination showed a round ulcer 5 cm from the ileal end, next to another ulcerative lesion; eight lymphatic nodes were found. The blood vessel under the deep ulcer was the origin of the profuse bleeding.\nThe histopathological examination showed an inflammatory ulcerative reaction with microabscesses (Figs. , and ). A granulomatous necrotic reaction was revealed in the examination of all eight nodes (Fig. ). The pathologist’s report concluded with diagnosis of an infection due to Yersinia enterocolitica.\nHer immediate postoperative outcome was favorable, the bleeding stopped, and her hemodynamic parameters remained stable. She had a normal transit on the fifth day after the intervention. After receiving the pathologist’s report, it was decided to treat her with fluoroquinolones 500 mg twice daily without discontinuing the iron supplementation, and a clinical control was scheduled. At the control 3 weeks later, she had no clinical symptoms, her diarrhea and bleeding had ended, and signs of anemia were diminishing. Antibiotherapy was discontinued. |
A 35-year-old left-handed man who works as a mechanic and firefighter presented to our institution with hand pain and cold intolerance for 3 months. He was referred by a local vascular surgeon who had made the diagnosis of hypothenar hammer syndrome (HHS) following an angiogram of the right upper extremity showing complete occlusion of the ulnar artery (). On initial examination, Doppler signals of the ulnar artery, superficial palmar arch, and ring/small digital arteries were absent in the right hand. Subsequently, the patient developed an ulcer on his right long fingertip after minor trauma that had not healed over a 4-week period. He suffered ischemic hand pain and severe cold intolerance. After conservative management failed, the decision was made for operative intervention. We proposed using an arterial graft versus a venous graft in order to improve long-term patency. Our plan was to utilize the descending branch of the lateral circumflex femoral artery (DLCFA) as an arterial graft.\nIntraoperatively, Doppler ultrasound revealed a segmental thrombotic defect extending from the superficial palmar arch, across the Guyon canal, extending 10 cm proximally in the forearm. The ulnar artery was resected back to healthy bleeding pulsatile flow, resulting in a segmental defect of 12 cm. Final pathology of the submitted ulnar artery segment revealed organizing thromboembolus in the lumen, which at the time of resection was adherent to the lumen.\nThe DLCFA was harvested, with care taken to prevent injury to the lateral femoral cutaneous nerve. A 12-cm segment of the artery was circumferentially dissected, as well as approximately 2 cm of 2 branching vessels at the distal end of the segment that would be used to reconstruct the superficial palmar arch and common digital arteries with one graft. With the arterial graft completely dissected, it was ligated both distally and proximally.\nThe arterial graft and recipient vessels were examined under the operative microscope. The proximal anastomosis was preformed first. The two branches at the distal end of the arterial graft were anastomosed to the superficial palmar arch stump, and common digital artery to the ring and small finger, respectively. A bolus of 5000 units of heparin was given intravenously before release of the microvascular clamps. Adequate pulsatile flow was achieved though the graft and confirmed with Doppler ultrasound to each finger ( and , Video 1). The hand was then irrigated, and the ulnar nerve was explored to ensure that no injury had taken place. The skin was closed, and the patient's hand was placed into a dorsal splint.\nThe patient remained in the hospital overnight for monitoring and was discharged the following morning on oral aspirin as the only anticoagulant. On follow-up examination 2 days later, strong Doppler signals were elicited at the ulnar artery, palmar arch, and radial and ulnar aspect of each digit. At 1 month, Doppler signals remained strong and the ulcer previously present on the right long fingertip had completely resolved (, Video 2). During this visit he reported to be asymptomatic and has had complete resolution of the cold intolerance and burning pain of his right hand. |
A 16-year-old girl presented with a 6-month history of intermittent pain and swelling over the anterior medial aspect of the distal diaphysis of her left tibia. The pain was exacerbated by activity and released by rest. The bony mass grew into 3 × 6 cm in size when she was administrated into our hospital. A palpable, immobile, and mild tender mass was noted 5 months prior to administration. The local skin appeared normal without redness and venous engorgement. The radiographs of the distal tibia shaft demonstrated a mass on the bone surface, which contained radiolucent and sclerotic regions. The radiographs also showed thickened underlying diaphyseal cortex and perpendicular periosteal reaction extending into the soft tissue mass (Fig. ). Computed tomography was taken, and the lesion was about one half of the circumference of the tibia in width without obvious medullary involvement (Fig. ). According to the radiological features, parosteal and periosteal osteosarcoma were considered. A needle biopsy was carried out. The hematoxylin-eosin-stained results revealed the lobules of neoplastic cartilage with myxoid matrix, which implied periosteal osteosarcoma.\nMarginal resection of the periosteal osteosarcoma was performed. At the time of surgery, the lesion margin was firstly identified based upon the CT findings. The lesion was approached by way of an anteromedial incision. Meticulous dissection was performed to preserve sufficient protective margins of tissue. The tumor was exposed and appeared as thickened and enlarged sclerotic bone without adjacent soft tissue mass. Surgical margin of the tumor was finally defined according to the CT images and gross inspection. The bone was excised more than 2 cm away from the margins of the tumor. The lesion and surrounding normal bone were removed. The bone block, about three fifths of the circumference in width and 12 cm in length of the affected tibia, was excised. A large bone defect was left. The retained tibia was about two fifths of the circumference in width at the level of bone defect, which maintained the nature continuity with that superior and inferior to the bone defect. Preliminary evaluation of the surgical margin and intramedullary cavity was performed immediately after removal of the tumor. The bony resection margins were judged clear, and the intramedullary aspect of the lesion was assessed to be uninvolved by gross observation.\nA fibular autograft was harvested from the right lower leg to reconstruct the bone defect of the left tibia. A straight incision about 18 cm in length was made from the point 10 cm above the lateral malleolus along the posterior border of the fibula. The fibula was reached via the posterolateral approach. An 18-cm long fibular bone block was resected. Both ends of the fibular graft were trimmed, and the medullary canal of the tibia was reamed. The fibular graft was firmly impacted into the proximal and distal medullary canal of the left tibia. The wound was closed in order, and a plaster cast was applied to stabilize the calf, the knee, and ankle joints.\nIncisional biopsy tissues were gained postoperatively from multiple sites of the resected tumor and along the surgical margins for histopathological analysis. The hematoxylin-eosin-stained results confirmed the preoperative diagnosis of periosteal osteosarcoma (Fig. ), grade 2 according to the staging system of Enneking []. The histopathological examination showed that the margin of the specimen was clear from tumor cells, and no medullary involvement was identified. Postoperative radiographs of the left lower leg were taken, which demonstrate the retained tibia and the bone defect reconstructed with fibular autograft and stabilized using a plaster cast (Fig. ). The patient received chemotherapy, a combination of cisplatin and doxorubicin, as would be used for conventional osteosarcoma [–].\nThe postoperative course was uneventful. The patient was encouraged to perform isometric muscle exercises of the affected lower extremity and full range of motion of the hip joint as soon as pain could be tolerated. Non-weight-bearing crutch walking was allowed after soft tissue healing. The cast was removed at 3 months postoperatively, and partial weight bearing was permitted, progressing to full weight bearing at 6 months. Follow-up was conducted at 1, 3, 6, and 12 months after the operation and every year thereafter. Radiological and clinical evaluation was performed at each follow-up. Excellent bony healing of the tibia and fibula graft was observed on the radiographs of the left lower limb at 6 months, and the patient regained normal walking function without a crutch. The retained tibia at the bone defect level grew and gradually wrapped the fibula graft. The tibia incorporated well with the fibula graft at 36 months postoperatively. The radiographs at 60 months of follow-up demonstrated that the fibula graft was almost absorbed. The reconstructed tibia nearly regained the diameter as the contralateral unaffected tibia (Fig. ). At the latest follow-up, 11 years after the operation, the radiographs and CT scan of the bilateral lower extremities were taken, showing good remolding of the retained tibia and fibular autograft (Figs. and ). The muscle strength of the bilateral lower limbs were evaluated both as normal (grade 5) according to the Manual Muscle Testing Grading System. Single-legged hop tests [, ] were conducted, and the patient was classified as having self-reported normal function of the bilateral knees. The patient can now conduct daily activities and manual work with a score of 100 according to the Activities of Daily Living (ADL) questionnaire. During the follow-up period of 11 years after surgery, no local recurrence or distant metastasis occurred in the patient.\nIn the current study, we treated a young female patient with periosteal osteosarcoma by marginal resection with retained tibia at the same level of bone defect and reconstruction using the autologous fibula graft. Chemotherapy was received postoperatively. The retained tibia incorporated with the graft fibula well, and excellent limb function was achieved. Local recurrence or distal metastasis was not reported at the 11-year follow-up.\nPeriosteal osteosarcoma was described in 1976 by Unni and his colleagues []. Histologically, it consists of a large chondroblastic tissue with smaller areas of osteoid formation []. Radiographically, it appears as a well-defined, essentially radiolucent mass probably with cortical thickening, extrinsic scalloping of the cortex, and periosteal reaction. CT and magnetic resonance (MR) imaging can reveal the extent of the soft tissue mass. The large chondroid tissue is demonstrated as low attenuation at CT and high signal intensity at T2-weighted MR imaging. There is only occasional involvement of the underlying cortex. The involvement of the medullary cavity is significantly rare. Pathologically, periosteal osteosarcoma is an intermediate-grade chondroblastic osteosarcoma (grade 2 or 3) [–].\nSurgery is required to cue periosteal osteosarcoma, and wide surgical excision is regarded as an appropriate therapy [, , ]. However, a large bone defect is often a leftover after wide resection. There are no autologous bone grafts with proper size to bridge the gap in such cases. Massive allografts are prone to infection []. Alternatively, marginal section is also an option taking the low malignance of periosteal osteosarcoma into consideration. Since local recurrence has a tendency to occur in case of incomplete resection, the bony resection margins and the intramedullary aspect of the lesion should be confirmed as clear at the time of surgery.\nPeriosteal osteosarcoma occurs most often in adolescents and has a predilection for the diaphysis of the tibia or the femur []. The young patients have high demand of the limb function. Limb-salvage surgery should be performed in these patients. The limb-salvage procedure of a malignant tumor in the distal tibia is a great challenge to orthopedic surgeons. The distal half of the diaphysis of the tibia has a few direct muscle attachments, and only part of posterior tibialis posterior, flexor digitorum longus, and extensor digitorum longus are originated from the distal half of the tibia. The limb salvage presents unique difficulties [].\nTo reduce the difficulty encountered in the limb-salvage surgery and preserve the limb function to the utmost extent, we treat the periosteal osteosarcoma by marginal resection. The lesion and its surrounding normal bone was excised in the affected segment of the tibia. Part of the tibia at the same level of bone defect was retained to maintain the continuity of the cortex. The retained tibia served as the structure supporter of the lower limb and preserved the limb length. The bone defect was reconstructed with a long autograft fibula, which was trimmed and inserted into the intramedullary cavity. The fibula graft had the following two main functions: improving the osteogenesis and providing structure support. No implant was needed. Accordingly, implant-related complications or implant removal was avoided. The tibia incorporated well with the autograft fibula at 36 months after surgery as demonstrated on CT. The fibula graft was almost absorbed, and the reconstructed tibia was nearly as thick as the contralateral unaffected tibia. The muscle strength of the left lower extremities was evaluated according to the Manual Muscle Testing Grading System, and functional recovery of the left knee joint assessed using single-legged hop tests were both normal. The patient can now conduct daily activities and manual work without any difficulty. Compared with limb-salvage surgery using prosthesis, the novel technique is easy to perform. It is also economy-friendly, especially in the developing country.\nAt the latest follow-up, no local recurrence or distal metastasis was reported. Two factors may contribute to these issues. At the time of surgery, the bony resection margins and the intramedullary aspect of the lesion were judged clear. Postoperative histopathological examination confirmed the intraoperative judgment. In addition, the cisplatin and doxorubicin were applied in the case. Controversy remains as to whether chemotherapy is necessary in the management of periosteal osteosarcoma [, , , ]. Previous studies have produced mixed results in this consideration. In the report of Bertoni et al., a marginal excision was done in eight patients with periosteal osteosarcoma, and all but one was affected by a local recurrence []. Chemotherapy was not used in the initial treatment in their study. Grimer and his colleagues also reported that the use of chemotherapy was not shown to be a prognostic factor, but the chemotherapy was used in two thirds of the patients (80 patients) in their study, and doxorubicin and cisplatin were used in 75 patients (93.8 %) []. Revell and his colleagues claimed 100 % survival in patients who have undergone adequate chemotherapy and surgery []. While in our study, pre- and postoperative chemotherapy were used, and no recurrence was found during the follow-up period of 11 years.\nAlthough the presented case demonstrated excellent functional recovery without local recurrence or distal metastasis for a long term of follow-up, this procedure should be restricted to low-grade periosteal osteosarcoma without medullary involvement. Larger numbers and a longer follow-up are needed to verify the efficacy of this kind of reconstruction fairly. |
A 34-year-old gentleman was first referred to orthopaedic services with a history of persistent knee pain, located over the right femoral condyle near the origin of the lateral collateral ligament. Eleven years prior to presentation, the patient was involved in a road traffic accident where he sustained a fracture of the right femoral neck and ipsilateral shaft. Initial management consisted of open reduction, fixation of the femoral neck fracture with cannulated screws and the ipsilateral shaft fracture with plating. The patient later had a revision of the plate to a femoral nail. Union was subsequently achieved with the femoral shaft fracture; however, a significant external rotation deformity was noted, and discomfort to the knee.\nThe patient was referred on to our tertiary referral orthopaedic centre for femoral de-rotation surgery. An initial CT scan performed revealed an external rotation deformity of 45 degrees. The patient procedure included removal of femoral nail, osteotomy with de-rotation surgery, and subsequent exchange nail. The femoral nail was fixed proximally and locked into place distally, following the corrective 45 degrees of internal rotation achieved to the distal femur during osteotomy, performed under intraoperative radiographic guidance. The post-operative rehabilitation regime consisted of initial touch weight bearing only, with gradual increments in weight bearing status. At six months follow-up, the patient complained of distal lateral femur pain. A repeat CT scan was performed, revealing a delayed femoral union. This was initially thought to be the cause of the patient’s symptoms. The patient was admitted for dynamization of the femoral nail with an injection of bone graft substitute, and iliac crest graft. Despite eventual union, the patient continued to complain of pain at the distal lateral femur, with a cracking sensation on movement now noted.\nRepeat clinical examination revealed a palpable crepitus over the distal iliotibial band with a snapping sensation, as it appeared to catch. A palpable small, solid swelling was noted at the posterolateral right knee. Ultrasound and CT revealed intra-articular loose bodies lying within the lateral para-patellar gutter. The patient subsequently underwent arthroscopy with removal of loose bodies. Despite this, at six-months follow-up to arthroscopy, the patient continued to complain of posterolateral knee pain. MRI showed no evidence of abnormality to the popliteus tendon or muscle, but highlighted the fabella embedded within the lateral head of gastrocnemius at the posterolateral corner of the knee. (Image ). The fabella was also notable on radiographs (Image ) and CT (Image ). Ultrasound scan confirmed the fabella as mobile on movement and associated with tenderness. The diagnosis of Fabella Syndrome was made, and the patient planned for a Fabellectomy procedure.\nAn initial arthroscopy was performed to review the knee joint, and to potentially assist in surgical excision of the fabella. A complete diagnostic arthroscopy was performed, with the fabella unable to be identified through the posterior capsule. After arthroscopy, open fabellectomy was performed. In view of the patient’s extensive scarring from prior orthopaedic procedures, a short 2cm incision was made directly over the palpable fabella (Image ). The common peroneal nerve was identified and spared. Subsequent incision left a cuff of biceps femoris to protect the nerve. Gastrocnemius was split and incised directly over the fabella, which was removed in entirety (Image ). Histopathology confirmed the diagnosis.\nThe patient was followed-up at two and ten months postoperatively. At both follow-ups, he described the complete resolution of his posterolateral knee pain. |
In May 2008 a 56-year-old man presented at our outpatient clinic with a two month history of increasing anterior knee pain of the left leg without previous trauma. The patient did not perform any sports on a regular basis. Physical examination revealed a full active and passive range of motion of the left knee, but maximal flexion was painful. There was no effusion or swelling but moderate tenderness on palpation of the inferior pole of the patella. Physiotherapy with stretching exercises of the quadriceps muscle did not improve symptoms. Magnetic resonance imaging (MRI) was performed and showed a hyperintense lobulated mass with small fluid-filled cavities at the inferior pole of the patella (Figure ). An additional CT scan revealed an osteolytic lesion with endosteal scalloping and cortical thinning (Figure ). In June 2008 the patient was referred to a musculoskeletal tumor centre where a biopsy according to the guidelines of the musculoskeletal tumor society [] was performed. Histology showed bony fragments with fibrohistiocytic proliferations, loosely arranged spindle cells, and several multinuclear giant cells. The cavernomatous spaces were lined with endothelial cells and contained red blood cells (Figure ). Although the typical fluid-fluid levels on the MRI were not visible the tumor was diagnosed as a benign primary aneurysmal bone cyst (ABC). Curettage with burring and bone grafting was recommended but the patient deferred the surgery to September 2008 for occupational reasons.\nBecause of the long interval we performed another CT scan of the patella that revealed a significant progression of growth from initially 2.5 ml (Figure ) to approximately 9 ml (Figure ) with cortical breakthrough. Surgery revealed a lobulated cavity filled with a mixture of blood and solid red tissue as well as a fracture of the cortex at the inferior patellar pole, with the covering cartilage still intact. We performed intralesional curettage through a lateral approach with additional high-speed burring and lavage with hydrogen peroxide. Due to the aggressiveness of the lesion we decided to fill the cavity with bone cement instead of bone graft to reduce the likelihood of recurrence. Histology confirmed the initial diagnosis of benign ABC. The further treatment consisted of full weight-bearing of the left leg and physiotherapy to regain full range of motion.\nAt last follow up in February 2010 the patient was free of complaints and had a full range of motion. The standard radiographs showed regular placement of the bone cement without evidence of local recurrence (Figure ). After 17 months the inferior cortex is remodelled (Figure ), but there are also degenerative changes in the femoropatellar joint (Figure ). However this did not cause clinical symptoms. The knee society score, the Lysholm score and the WOMAC osteoarthritis index showed excellent results with 100 points each. |
A 63-year-old man presented to the Accident and Emergency department with a history of mild frontal headache and progressive right-sided weakness. He was on bendrofluazide and atenolol for hypertension which was controlled.\nOn examination, he was fully conscious and haemodynamically stable with a pulse of 65 beats per minute and blood pressure of 105/72. Systemic examination was normal. Power was 3/5 in the right arm and leg with the right plantar reflex upgoing. There was progression of neurological signs 24 hours after admission, until the power was 0/5 in the affected limbs with mild slurring of speech 72 hours later. There was no evidence of fluctuating neurological signs. His higher mental functions were intact throughout his hospital admission. The electrocardiogram confirmed sinus rhythm at 60 beats per minute. The chest X-ray was unremarkable.\nHe was admitted and underwent an urgent computed tomography (CT) scan of the brain. The initial CT (Figure ) was performed 6 hours after the collapse. The hospital had access to a magnetic resonance imaging (MRI) scanner once a week and the next available slot was 6 days later. During the weekend following admission he was transferred to the stroke unit and reviewed twice daily. His neurological deficits deteriorated stepwise with 2/5 power in his right arm and leg at 36 hours progressing to 0/5 with mild slurring of speech around 72 hours post admission on Monday. There were no features suggestive of raised intracranial pressure. He had no altered sensorium. His case was discussed with the radiologist and the medical physician on call during the weekend. It was felt there was no need for an urgent repeat scan and all agreed the progression was likely to be due to the stroke. The radiologist reviewed the initial scan over the weekend and had no concerns about the calcified area, which was judged less likely to be a bleed, although this could not be ruled out. The decision was to perform a scan on Monday morning to confirm that the deterioration was due to the stroke and the suspected calcified area was not a bleed.\nA repeat CT brain scan was performed after the weekend on the third day after admission (Figure ). |
A 16-year-old boy was involved in a car accident as a front seat passenger. The patient had no previous or current medical history. In the initial phase he was hospitalized in the intensive care unit because of multiple injuries. He suffered from bleeding in the abdominal cavity, pneumothorax, and lesions of the spleen but experienced no direct head trauma or loss of consciousness during the accident. There were no signs of pancreatitis. He underwent abdominal surgery with laparotomy and tube thoracostomy. After a couple of days his general condition was stabilized and he was transferred to the Pediatric Department at our hospital. Ten days after the trauma he complained about blurred vision on the right eye, and was therefore referred to the Department of Ophthalmology.\nHis presenting corrected visual acuity was 0.03 (Snellen equivalent) on the right eye and 1.0 on the left eye and was unchanged at one-month examination. Six months later, the visual acuity improved to 0.16 on the right eye and was still normal on the left eye. Anterior segment findings and intraocular pressure were normal. Ophthalmoscopy and fundus photo revealed an ischemic white posterior pole with cotton wool spots and intraretinal hemorrhages mainly in the macula and nasally to the optic disc. The white lesions and bleeding decreased already at the one-month visit (), and after 6 months, the white lesions were almost resolved.\nOCT showed hyperreflective and thickened inner retinal layers, a sign of ischemia in the inner retinal circulation. (). At follow-up visits the edema decreased significantly on OCT with disruption of the inner retinal layers but also seemingly disrupted ellipsoid zone. In addition, the thickness of the retina was reduced, from 427 microns at onset to 207 microns at 6 months. A manual segmentation of the OCT layers was conducted in order to ensure correct layer identification and thus thickness calculation. Testing of the central visual field by microperimetry showed a central scotoma with decreased sensitivity in the fovea ().\nOCTA () revealed extensive nonperfusion in the macular area in both the superficial () and the deep capillary plexus of the right eye (). OCTA of the left eye was with normal capillary plexus and normal foveal avascular zone (Figures and ). |
A 48-year-old woman, gravida 1, para 1, visited the internal medicine department at another hospital with a complaint of abdominal fullness and weight loss of 10kg during the last year. A huge abdominal mass was palpated, and she was referred to the gynecology department to search for a tumor of uterine origin. She was premenopausal and had no significant past medical history. Physical findings revealed a large elastic hard mass extending from the xiphoid to the pubic bone. The magnetic resonance imaging (MRI) examination revealed a huge tumor on the uterine corpus, and a number of dilated vessels were observed between the tumor and the myometrium. Therefore, the tumor was suspected to derive from the uterus. The tumor showed an uneven signal on T2-weighted sagittal section (), and the enhanced MRI study showed that the tumor edge but not the center was enhanced (). As such, necrosis was suspected to have occurred in the center of the tumor. Uterine sarcoma was primarily suspected due to the large size, degeneration, and necrosis on MRI imaging. Computed tomography (CT) examination showed no lymph node swelling or distant metastasis. Preoperative laboratory testing revealed anemia (hemoglobin level, 5.6g/dl). We transfused 18 units of RCC before surgery. CT examination and ultrasonography on lower extremities indicated an absence of thrombosis. Preoperative serum levels of CEA, CA 19-9, CA 125, and LDH were within normal limits. A biopsy of the endometrium was not collected as the sounding examination of the endometrium was unsuccessful due to a deviated uterine cervix. At this point, preoperatively, we suspected the tumor was a leiomyosarcoma or leiomyoma with degeneration.\nThe patient underwent laparotomy, where we identified a huge tumor occupying a space from the pelvis to the diaphragm. The tumor surface was smooth and hard with many dilated veins (). A massive tumor with a diameter of 30 cm was observed arising from the posterior uterine wall with a smooth contour and invaded the retroperitoneal cavity under the mesentery. The tumor was firmly adhered to both the mesentery and right ovary. There were no findings of extra-uterine dissemination. The intraoperative frozen section report for the uterine tumor was of degenerated myoma with no findings indicating malignancy. A total abdominal hysterectomy (TAH) and right salpingo-oophorectomy (RSO) were performed. The operation duration and blood loss were approximately 216 minutes and 1000 ml, respectively. The excised specimen weighed 7600 g.\nMacroscopic findings of the tumor revealed a well-circumscribed tumor showing extensive continuity with the posterior wall of the uterus, measuring 28 × 23 cm (). On the sliced surface of the tumor, an obvious heterogeneous pattern was recognized within the mixture of a whitish homogeneous area, suggesting benign uterine fibroids, and a vulnerable area, due to bleeding and necrosis ().\nFor the intraoperative frozen section, we examined three areas, namely, a white homogenous part, a necrotic part, and a cystic part, of which all were findings of a leiomyoma. In the permanent histological examination, 10 additional sections were collected from the tumor. The basic histological findings of all the sections were the same. The tumor was comprised of spindle-shaped cells, homologous to smooth muscle cells, which were arranged in bundles with areas of hyalinization, consistent with a degenerated leiomyoma. The tumor was mostly comprised of degenerated uterine leiomyoma. However, enlarged blood vessels were observed within an area of approximately 2 cm × several mm, and proliferation of atypical cells showing a fine meshwork microvascular structure was observed in the blood vessel cavity (). These atypical cells consisted of various contours, such as cubic, polygonal, and short spindle shape. The nucleus was circular with a high degree of vacuolar enlargement and pleomorphism. Abnormal mitotic figures were also interspersed (). A tumor derived from a blood vessel was thus considered, and malignancy was suggested by the presence of nuclear atypia and abnormal mitosis.\nImmunohistochemical analysis revealed the atypical tumor cells to be positive for ERG, CD31, and AE1/3 (Figures and ), partially positive for Factor VIII, and negative for α-SMA, desmin, H-caldesmon, EMA, CD34, and D2-40. From the above, the atypical tumor cells were of epithelial origin and the final diagnosis was epithelioid angiosarcoma arising in a degenerated uterine leiomyoma.\nThe efficacy of postoperative adjuvant therapy for angiosarcoma has not been demonstrated and there is currently no established chemotherapy regimen. In this case, because the atypical tumor was observed in the blood vessel cavity, we thought it could have been spread hematogenously throughout the body. Hence, we selected adjuvant chemotherapy rather than adjuvant radiotherapy. Six courses of combination adjuvant chemotherapy with paclitaxel (150mg/m2), epirubicin (50mg/m2), and carboplatin (area under the curve = 4) were administered in the present case, following referral to previous reported cases. No recurrence has been observed 10 months after the primary surgery. |
A 44-year-old woman with a history of gastroesophageal reflux disease and hypertension presented with worsening shortness of breath. She was found to have pneumonia and required intubation. She developed multiple complications, including sepsis, adult respiratory distress syndrome, acute renal failure, intensive care neuropathy, cardiac arrhythmias, and cardiac arrest. Approximately three weeks after hospitalization, a tracheostomy tube was placed. She then developed intra-abdominal abscesses after a gastrostomy tube became dislodged. She underwent exploratory laparotomy with abscess drainage and closure of the gastrostomy and was maintained on total parenteral nutrition.\nFour months after admission to the hospital, a nasogastric tube was placed and enteral nutrition was started. Sixteen days later, she experienced an episode of substantial bleeding, and it was not clear whether this came from the airway or oropharynx. Her hematocrit was 20 percent, platelets were 396,000, and blood urea nitrogen was 13 mg/dL. She was resuscitated with packed red blood cells, platelets, cryoprecipitate, and fresh frozen plasma; selective carotid arteriography of the head and neck, direct laryngoscopy, and bronchoscopy were unremarkable.\nThree days later, she again experienced severe bleeding, which was initially felt to have originated from her airway. She was taken to the operating room by the vascular surgery team with the preoperative impression that she had a tracheoinnominate fistula. Median sternotomy revealed a right carotid artery arising directly from the aortic arch with no innominate or right subclavian artery in the anterior mediastinum. No artery to trachea fistula was identified and a provisional diagnosis of aberrant right subclavian artery with subclavian to esophageal fistula was made given the observation of the absence of the subclavian and innominate arteries in the usual location. The sternotomy was closed and a CT of the chest was performed, which demonstrated the anomalous right subclavian artery without aneurysmal degeneration (Figures and ). She was taken back to the operating room for endovascular covered stent grafting of the segment of the subclavian artery in continuity with the esophagus. An angiogram was performed () and three covered stents were deployed to span the artery-esophageal fistula via a right brachial artery approach (). Successful control of the bleeding was accomplished, but she experienced a cardiopulmonary arrest and died despite resuscitative efforts. |
A 37-year old homeless male, with a past medical history of peripheral vascular disease, type 1 diabetes mellitus, hypertension, and depression, presented to the emergency department with intermittent chest pain and progressive shortness of breath for a few weeks. The patient denied intravenous drug use, although admitted to the use of recreational marijuana. The patient’s vitals on admission were stable except for low saturation on a pulse oximetry of 86% on room air. On physical examination, the patient was tachypnic and had fine crackles in the bilateral lung fields on auscultation. Laboratory results on admission showed a normal complete blood count and basic metabolic panel, but a urine drug screen test was positive for opioids. Because of persistent hypoxia, a D-Dimer was checked and came back highly elevated. The patient underwent a computed tomography angiography (CTA) of the chest to rule out pulmonary embolism. The CTA was negative for pulmonary embolism, however, it displayed extensive miliary densities throughout the bilateral lung fields (). The patient was admitted to the floor with a differential diagnosis of military tuberculosis versus fungal infection. Human immunodeficiency virus (HIV), fungal, and Quantiferron testing were negative. The cardiac work up and autoimmune serology were also unremarkable. The patient was then started on intravenous steroids and inhaled albuterol, although no improvement was seen. The patient remained hypoxemic despite therapy, and, therefore, underwent a bronchoscopy with a lung biopsy to find out the etiology of the disease process. The lung biopsy showed alveolated lung tissue with a miliary pattern of perivascular foreign body histiocytes containing refractory material suggestive of microcrystalline cellulose material (). There was no evidence of malignancy and there were no fungal or acid fast bacilli organisms identified on special stains. The histological features suggested intravenous injection of foreign material and upon further questioning the patient admitted to injecting oral opiates. The patient was started on intravenous steroids, although his clinical condition continued to decline. The patient developed hypercapnic respiratory failure, which required intubation, and eventually suffered from a cardiopulmonary arrest and passed away. |
A 67-year-old man presented to the emergency department with syncope.
Seven months prior to this presentation, he was diagnosed with pT3 NX M0 stage III high-grade urothelial carcinoma of the right renal pelvis with peri-neural and peri-vascular invasion. He underwent laparoscopic right nephro-ureterectomy with bladder cuff. Four days after surgery, patient presented to the emergency room with acute severe shortness of breath and hypotension that responded to fluids. Computed tomography angiography (CTA) of the chest revealed extensive filling defects demonstrated in both the distal main pulmonary arteries extending into multiple bilateral upper and lower segmental pulmonary arteries, and low likelihood of right heart strain. Bilateral lower extremity Doppler ultrasonography revealed no evidence of deep vein thrombosis (DVT) in lower extremities. He was started on systemic anticoagulation followed by warfarin bridge. Patient was relatively well until 2 months later, when he developed another episode of acute severe shortness of breath. He was then diagnosed to have new pulmonary embolism while being on warfarin therapy. Patient elected to have inferior vena cava (IVC) filters placed even though there was no Doppler evidence for DVT and anticoagulation was transitioned from warfarin to rivaroxaban. His echo at that time revealed mild pulmonary hypertension without evidence of right heart strain. From the cancer perspective, he was considered to have no evidence of residual disease based on imaging and plan was to manage conservatively with follow-up imaging in 3 months due to intermediate to high risk of recurrence. Patient is a former smoker and quit 20 years prior to this presentation. His brother died of bladder cancer at the age of 50.
Patient presented with another episode of shortness of breath 1 month after starting rivaroxaban. His CTA showed new pulmonary embolus in the right main pulmonary artery with evidence of right heart strain. Due to recurrent pulmonary emboli, his anticoagulation was changed to fondaparinux and aspirin of 325 mg. Patient unfortunately presented 1 month later with worsening shortness of breath, palpitations with |
The first case was a 59-year-old African American male with a past medical history notable for schizoaffective disorder, depression, and substance abuse who was brought in to the emergency room for disorganized behavior and agitation in the community. At the time of admission the patient demonstrated disorientation, repetitive motor behavior, and an alternation between agitation and psychomotor retardation. He had poor response to communication and tactile stimuli. A suspicion of altered mental status due to organic causes was suspected with the possibility of catatonic excitement and retardation. He was admitted to the medical floor, with a work-up revealing a positive toxicology screen for cocaine and opioids. The patients CBC and BMP were within normal limits except for his ammonia level which was 80 mg/dl. The patient was initially treated with Chlorpromazine Hcl 50 mg orally daily for his agitated behavior as well as Naltrexone 50 mg orally daily for his opiate intoxication.\nThe patient exhibited incoherent thought process in addition to mumbled speech that made a significant portion of his assessment evaluation difficult. During evaluation, he displayed abnormal movements of his arms and face, with tremors and restlessness. His affect was flat. He did not display any perceptual disturbances or delusions. An assessment for cognitive impairment was noncontributory during his most recent admission. The patient received Mirtazapine 45 mg orally at bedtime and Olanzapine 10 mg orally daily in his treatment and by day three of admission had shown improvement in his disorganized behavior with supportive care. The patient demonstrated more effort to directly communicate with house staff after treatment began.\nThe patient reported a past history of psychiatric illness that was late in onset. His first presentation at the age of 51 years was significant for depressed mood, paranoid delusions, and auditory hallucinations for which he was diagnosed with a major mood disorder. His symptoms responded poorly to medications including antidepressants. His disease course involved increasing periods of impulsive behavior and agitation. He became noncompliant with his prescribed medications. He was later admitted to the medical floors at the age of 54 years for “repetitive behavior” during which he was found moving from his bed to the bathroom repeatedly as if he wanted to use the bathroom all the time. He also showed some abnormal rocking movements during this time period. A medical work-up for seizure was negative. He was discharged with a presumptive diagnosis of a psychotic disorder. Thereafter, at the age of 56 years he had an episode of property destruction in the community and it was noted that he had “abnormal body movements” in addition to lability of mood. His diagnosis was revised to schizoaffective disorder and he was treated for mood lability at the time with risperidone.\nGiven the late onset of his neuropsychiatric symptoms, a computed tomography scan (CT) of his brain was done during his presentation, as seen in . Reviewing his chart, it was noted that the calcifications were apparent in his first head CT taken in January of 2012 with no changes to the current CT in January of 2019. |
A 58-year-old male presented with fever and breathlessness 10 days after laparoscopic sleeve gastrectomy (LSG). Investigations revealed leukocytosis with left-sided hydropneumothorax. An emergent pigtail insertion into pleural cavity was done and frank pus was drained. The esophageal contrast study revealed a large lower esophageal perforation with contrast leaking into the left pleural cavity. An upper gastrointestinal endoscopy revealed a large defect in the lower esophagus measuring about 4 cm in length. The patient was started on intravenous antibiotics and after an informed consent, an FcSEMS (SX-Ella, Ella CS, Czech Republic; stent body diameter 20 mm and throats diameter of 25 mm with length of 11 cm) was placed across the GEJ. The patient was kept nil orally and parenteral nutrition was given. In spite of these measures, the fever persisted and the drain output was > 200 mL/day. The chest X-ray done on the fourth day revealed distal migration of the stent into the stomach. Using grasping forceps, the stent was repositioned into the esophagus. There was no decrease in the daily drain output and a repeat contrast study was done on the fourth day after repositioning of the stent. There was no passage of contrast across the distal end of the stent and because of this obstruction, it was found to be seeping through the upper end of the stent into the leak (). An endoscopy revealed the blockage of the lower end of the stent by the prolapsed gastric mucosa (). The prolapsed gastric mucosa was pushed by the endoscope and an NJT was placed through the SEMS (). Following this patient had gradual improvement with decreasing daily drain output and he was started on enteral feeding through the NJ tube. The contrast study done on the sixth day of NJT placement revealed free passage of the contrast into the residual stomach with no leakage. The pigtail was removed 4 weeks later and the stent was removed 6 weeks after insertion using the Ella extractor. Endoscopy revealed complete healing of the esophageal defect and there was no leakage on contrast esophagogram. |
A 20-year-old woman presents with chronic dyspnea on exertion and exercise intolerance that was attributed to and treated as presumed exercise-induced asthma since childhood. She experienced worsening left-sided chest heaviness and was subsequently referred to our institute. She has no coronary risk factors and no family history of premature coronary artery disease or congenital heart condition. Physical examination was normal except a subtle continuous murmur at apex and lower right sternal border. ECG showed sinus rhythm without ST or T wave changes (Supplemental Figure 1 available at doi:10.1155/2012/471759). Chest X-ray showed no cardiomegaly (Supplemental Figure 2). Serial cardiac enzymes were negative. In the light of cardiac murmur, an echocardiogram was obtained and revealed anomalous coronary arteries with a large left coronary artery to main pulmonary artery fistula. There was mild left ventricular enlargement with preserved left ventricular contractile function and an ejection fraction of 65%. The appearance of the left ventricle and left atrium was consistent with a systemic to pulmonary vascular shunt with increased stroke volumes in the left heart. There were no structural abnormalities of the aortic, mitral, tricuspid, or pulmonic valves, and there was very mild mitral regurgitation by Doppler. A subsequent cardiac catheterization confirmed the diagnosis of ALCAPA with retrograde filling through collaterals arising from an enlarged right coronary artery (12 mm) (), pronounced left-to-right shunting from the left main coronary artery into the left main pulmonary artery trunk; and the right coronary artery gives rise to collaterals to a large left anterior descending artery which has ectasia in its proximal segment into a smaller circumflex artery. The patient underwent surgical treatment by the creation of a pulmonary artery tube graft from the aorta to the left coronary artery and reconstruction of the main pulmonary artery with a bovine pericardial patch. She had no major complications or ischemic symptoms 18 months after operation. A follow-up echocardiogram at that time showed normalized stroke volume and left atrial and ventricular size secondary to reversed left-to-right shunt, preserved LV EF at 55%, and absence of valvular abnormalities. |
A 17-year-old Sardinian young woman underwent an initial examination at the dentist’s office during a routine checkup in 2010. After obtaining informed consent from her parents, clinical evaluation was performed, during which some caries were detected and also the need for an orthodontic consultation. The presence of an atrophy of filiform papillae with circinate erythematous ulcer-like lesions of the dorsum and the lateral border of the tongue was observed.\nDuring the anamnesis, neither the patient nor her parents reported any history of systemic disease or, in particular, any gastrointestinal symptoms. Talking with the patient and her parents, it was possible to verify that the tongue affection had started when she was five years old with no other symptoms and with intermittence. During her past medical history, no one had ever investigated these tongue lesions.\nWith these elements, the clinician identified the tongue condition as an AG and started with a more detailed analysis of systemic and local conditions related to this pathology. The patient, as highlighted by the anamnesis, did not show any other symptoms or signs related to pathologies typically linked to an AG, apart from her short stature []. She was only 145 cm tall but this condition had previously gone unnoticed because her parents were also short. Considering the presence of short stature with an AG, the problems could be related to a nutritional dysfunction such as vitamin B12 deficiency. Given that Sardinia is an area with a high frequency of CD, gluten enteropathy became the first suspect as a possible cause for the eventual vitamin B12 deficiency.\nAs a first step, a blood sample was requested to determine the vitamin deficiency and to perform AGA and tTG antibody tests and the EmA test. Hematologic tests showed a vitamin deficiency with positive results for antibody tests (Table ) so a small intestine biopsy was performed during a gastroenterological consultation [].\nA diagnosis of CD was made based on the findings from the biopsy sample; such as: the characteristic changes in intraepithelial lymphocytosis, crypt hyperplasia and a Marsh type IIIc villous atrophy. She was treated by excluding gluten-based-food from her diet (gluten-free diet). After five months she repeated the intraoral examination where it was possible to verify remission of the AG. |
A G2P1 32-year-old woman with a history of hyperthyroidism was referred to our perinatology clinic for second trimester ultrasonographic and fetal echocardiographic screening at 26-weeks gestation. The family history was negative for congenital heart defects, chromosomal abnormalities, or unexpected newborn death shortly after birth. There were no other malformations outside the heart of the fetus (Samsung Ultrasound H60). Fetal echocardiography showed that visceroatrial situs solitus with normal systemic and pulmonary venous connections and normal four-chamber view. Fetal cardiac findings were as follows: the aortic arch and ductal arch were located on the right side of the trachea and four vessels on the 3VT view (). Although the right PA arose from the pulmonary trunk, the origin of the LPA could not be seen clearly. To evaluate the anatomy in the transverse plane, Doppler ultrasonography was used, sweeping from inferior to superior and tracking the LPA from the hilum of the left lung, and anterior the trachea revealed its origin from around the vicinity of the ascending aorta closed to the brachiocephalic artery (). The thymus was seen normally behind sternum at the level of the 3 vessels trachea view. The parents were consulted for genetic diseases but cordocentesis for karyotyping and analysis of 22q11 deletion was refused by the patient. At 40 weeks of gestation, a female infant of 3,565 g was born after an uncomplicated vaginal delivery with APGAR scores of 9 and 10 at 1 and 5 minutes, respectively. The physical examination of the baby was normal without any dysmorphic features and arterial oxygen saturation was normal. Neonatal echocardiography revealed LPA origin in the posterior aspect of ascending aorta, midsegment stenosis in the LPA with a peak pressure gradient of 30 mm Hg. Right-sided aortic arch, right-sided patent ductal arteriosus, and mild tricuspid regurgitation were seen in the postnatal echocardiography. Two days after birth, we performed CT angiography and confirmed the fetal diagnosis showing the described left pulmonary hemitruncus. The newborn was discharged at 3 days after birth without cardiac or respiratory symptoms. The baby was doing clinically well in her last clinic visit at 4 months of age. |
A 33-year-old Tibetan woman, gravida 2, para 1, was admitted because of the abnormal location of one of the two gestational sacs of a twin pregnancy; she had previously undergone transverse lower segment cesarean section 7 years earlier. The patient underwent IVF-ET and 3 embryos were transferred to the uterus; a positive pregnancy test was noted 14 days after embryo transfer. Four weeks after embryo transfer, transabdominal ultrasonography revealed 1 intrauterine gestational sac. However, the patient did not adhere to the physician’s suggestion of a follow-up 1 week later.\nSixty days after embryo transfer, transvaginal ultrasonography revealed a dichorionic twin pregnancy with normal cardiac activity and 1 gestational sac situated in the uterine fundus; the other was located lower, immediately over the cesarean section scar, with a thin myometrium of 4 mm in thickness (Fig. ). The patient manifested no abdominal pain and/or vaginal bleeding. A diagnosis of heterotopic cesarean scar pregnancy was then made, but the couple refused medical intervention.\nA follow-up was pursued 7 days later, with both fetuses showing normal fetal cardiac activity and crown–rump length measurements (55 and 48 mm, respectively) that were in accordance with a fetus at 12 + 1 weeks of gestation; 1 fetus was in the upper fundus, whereas the second was still located at the level of the internal os. The ectopic placenta covered the internal cervical os and the cesarean section scar, which was in close proximity to the maternal bladder and which showed the presence of peritrophoblastic vascularity upon Doppler examination.\nWhen the patient was transferred to our department, because of the potential of uterine rupture and catastrophic hemorrhage, we extensively counselled the couple regarding the condition, its risks, and the management options for heterotopic pregnancy. We suggested to the couple the use of selective reduction of the heterotopic cesarean scar pregnancy as soon as possible. The couple, however, refused and preferred expectant management; close monitoring was then performed without patient bleeding and lower abdominal pain. At 16 + 3 weeks of gestation, transabdominal ultrasonography revealed a thin myometrial layer of the cesarean section scar (3.8 mm thick) and complete placenta previa (Fig. ). Massive bleeding and/or uterine rupture during an ongoing pregnancy with expectant management was discussed again with the couple, and the patient opted for selective termination of the abnormally located fetus; this was then accomplished at 16 + 4 weeks of gestation by ultrasound-guided intrathoracic injection of 1 mL of 10% KCl using a 20-G needle inserted transabdominally under local anaesthesia with lidocaine. Upon follow-up, ultrasonography was repeated every two weeks. Although fetal biometric parameters of the ongoing pregnancy were normal, the ectopic placenta still showed total placenta previa and placenta accrete. At 37 weeks’ gestation, a written informed consent was obtained from the couple undergoing MRI to evaluate the placenta. MR image showed heterotopic complete placenta previa and placenta accreta, marked focal thinning of myometrium at the region of cesarean scar. The internal cervical os was covered completely by placenta and dead fetus (Fig. ).\nAt 37 + 6 weeks of gestation the baby was delivered by elective cesarean section. Before the operation, we extensively counseled the couple about the possibility of CS hysterectomy due to total placenta previa and placenta accreta. We noted intraoperatively that profuse vascularization covered the lower uterine segment, that the bladder adhered to the anterior lower segment, and that the mass of HCSP was palpated at the lower uterine segment and bulged toward the vesicoperitoneal reflection. A healthy male baby weighing 2890 gm was delivered through a transverse incision 1 cm above this mass, with Apgar scores of 10 and 10 at 1 and 5 min, respectively. When the dead fetus and placental mass of the HCSP were removed, profuse bleeding due to placenta accreta ensued, and bleeding was controlled by partial excision of the anterior lower uterine segment along with myometrial sutures and uterine packing with gauze. Three-and-one-half units of packed red blood cells were then transfused. The postoperative period was uneventful and the patient and the baby were discharged 6 days after the operation. The pathologic results of the excised anterior lower uterine segment revealed placenta accreta, showing chorionic villi in direct contact with myometrial smooth muscle fibers. |
A 45-year-old male with a previous history of tympanoplasty and functional endoscopic sinus surgery with septoplasty 10 years earlier presented to the ear, nose, and throat (ENT) clinic with several months of left moderate-to-severe otalgia and a sensation of ear blockage in his left ear accompanied by ipsilateral hearing loss. He gave a history of multiple failed ear wax removal in his left ear that had been performed at several ENT clinics, despite the use of alkaline ear drops.\nOn examination, the patient was comfortable and afebrile, and his vital signs were stable. Otoscopic examination of the left ear revealed impacted left ear wax covering the tympanic membrane, which could not be assessed. Otoscopic examination of the right ear also demonstrated mild ear wax, and the tympanic membrane was unremarkable. Oropharynx examination was unremarkable, the lymph nodes of the neck were not palpable, and all cranial nerves were intact upon examination. Nasal endoscopy revealed no pathologies. Ear wax removal under suction was attempted and failed. Another trial of removal after using alkaline ear drops for several days was also attempted but was unsuccessful. However, the surgeon became suspicious that the patient had KO rather than impacted ear wax because the wax was thick, had the appearance of keratin plugs, and was hard to remove after several attempts, despite the use of ear alkaline drops.\nBlood test results of the patient were within normal limits. The patient was planned for microscope-guided examination of the ears under general anesthesia. The examination revealed that the left ear was full of wax that was accumulating in the skin and contained a thick keratinous plug that had dilated the external auditory canal (EAC) with pockets and bone remodeling. Furthermore, the patient ear canal was circumferentially distended with a normal annulus. The tympanic membrane became visible and was intact. The keratinous plug was removed, and a diagnosis of KO was established (). An ear pack was draped with antibiotics and placed in the left ear. The patient was extubated, shifted to the ward without any complications, and discharged the same evening with the ear pack, which was removed after 3 weeks in the outpatient clinic. The patient was started on ciprofloxacin ear drops and analgesia for 1 week.\nIn the follow-up, the ear pack was removed, his hearing returned to normal level, and the pain disappeared. Pathological analysis of the removed plug revealed acellular lamellated keratin flakes and keratinous material (Figures and ), which confirmed our diagnosis. |
A 13-year-old girl with no prior medical history was admitted to the hospital after resuscitation from cardiac arrest. The patient had collapsed during physical exercise in school, and cardiopulmonary resuscitation (CPR) was initiated immediately by school personnel. Pulseless electrical activity was the first observed prehospital rhythm. The patient was intubated by the ambulance staff, and return of spontaneous circulation was achieved after 35 minutes through CPR and adrenaline administration.\nUpon arrival to our emergency department, the patient was hemodynamically unstable with a mean arterial pressure (MAP) of 60–70 mmHg and heart rate of 120 bpm. Arterial blood gases showed a severe combined metabolic and respiratory acidosis with a pH of 6.7, PaCO2 of 83 mmHg (11 kPa), and a blood lactate of 15 mmol/L on mechanical ventilation. Bedside echocardiography revealed a dilated right ventricle and a mass in the right ventricular outflow tract suggesting a venous thrombus. Computed tomography (CT) with pulmonary angiography showed multiple peripheral emboli in both lungs, and CT of the abdomen and pelvis revealed an aneurysm of the inferior vena cava as the likely source of the emboli. CT of the brain showed no evidence of cerebral edema or infarction (). However, based on the prolonged resuscitation and decreased level of consciousness, an ICP transducer was inserted to enable detection of cerebral edema during the impending deep sedation. Initial ICP readings were normal (4 mmHg).\nThe patient was transferred to the cardiothoracic intensive care unit (ICU) and a cerebral perfusion pressure >60 mmHg was maintained with infusion of adrenaline and noradrenaline. Targeted temperature management aiming at 36°C for 24 hours was initiated, and the patient was started on high-dose unfractionated heparin due to the pulmonary emboli. Failed attempts to cannulate both femoral arteries resulted in bilateral hematoma formation and continued bleeding from the right femoral artery despite compression necessitated surgical exploration with repair of the artery and fasciotomy.\nThe day after admission, the patient had been hemodynamically stabilized and weaned off vasopressors. Sedation was gradually diminished and finally turned off. The patient demonstrated eye opening upon stimulation, pupils that were equal and reactive to light, spontaneous breathing, and a normal swallowing reflex. No spontaneous movements were observed. ICP was slightly elevated at 10–17 mmHg depending on stimulation and closely related to MAP. Laboratory analyses revealed rising levels of creatinine, urea, and potassium, and diuresis was low despite stimulation with furosemide and metolazone. Myoglobin and creatine kinase were also significantly elevated, suggesting rhabdomyolysis due to hypoxia, compartment syndrome of the right thigh, or both.\nOn the evening of day 3, the patient had a P-creatinine of 5.17 mg/dL (457 μmol/L), P-urea of 99 mg/dL (35.4 mmol/L), and a P-potassium of 6.0 mmol/L despite infusion of glucose and insulin. The patient was visibly hypervolemic with an estimated cumulated fluid balance of +12.5 liters. ICP was stable but slightly elevated at 16–19 mmHg, and the patient was lightly sedated with remifentanil infusion. It was decided to initiate continuous renal replacement therapy (CRRT), and a double lumen dialysis catheter was placed in the right internal jugular vein. The following dialysis settings were used: Continuous venovenous hemodiafiltration, ST100 dialyzer (Gambro; surface area 1 m2, KUF 25 mL/(h·mmHg)), blood flow 120 mL/min, predilution flow 1000 mL/h (Prismocitrate, Gambro), dialysate flow 1000 mL/h (Prism0cal B22, Gambro), and postdilution flow 200 mL/h (Phoxillium, Gambro).\nApproximately seven hours after start of CRRT, ICP had increased to 38 mmHg (); the patient had developed diverging eye axes and become unresponsive to pain (Glasgow Coma Score 3). On suspicion of cerebral edema or infarction, CRRT was stopped, 50 mL of hypertonic saline (1 mmol/mL) was administered, and the patient was sedated with propofol. CT of the brain was performed and revealed diffuse cerebral edema (). ICP decreased to 20 mmHg within a few hours. Due to the neurological deterioration and unstable ICP, the patient was transferred to the neurological ICU (NICU) and sedated with thiopentone, midazolam, and fentanyl.\nOn day 4, an external ventricular drain (EVD) was inserted stereotaxically based on a predicted need for further hemodialysis, upon which ICP decreased from 10 to 3 mmHg. A window of cardiovascular stability and low ICP was used as an opportunity to start careful intermittent hemodialysis. Hemodialysis was chosen over peritoneal dialysis due to hyperkalemia and the need for removal of large volumes of fluid, as well as the possibility of separating ultrafiltration and dialysis. P-creatinine was 5.92 mg/dL (523 μmol/L) and P-urea 95 mg/dL (34.1 mmol/L) before start of dialysis. The patient completed 3.5 hours of isolated ultrafiltration, with ultrafiltration rate gradually increasing from 250 mL/h to 1000 mL/h and a total fluid removal of 2.5 L. Subsequently, the patient underwent 1 hour of hemodialysis without complications and with no clinically significant changes in ICP. Dialysis settings were modified to reduce the dialysis dose in an attempt to prevent DDS and were as follows: H6 dialyzer (Gambro; surface area 0.6 m2, KUF 33 mL/(h·mmHg)), dialysate sodium 148 mmol/L, potassium 2 mmol/L, bicarbonate 38 mmol/L, calcium 1.5 mmol/L, magnesium 0.5 mmol/L, and low dialysate flow at 300 mL/min and blood flow 150 mL/min with concurrent flows to reduce dialysis efficiency. On day 5, ultrafiltration and hemodialysis were repeated with similar settings. However, ICP gradually increased from 0 to 10 mmHg and the treatment was stopped prematurely. Similar problems were encountered on the following days, necessitating frequent pauses or early cessation of hemodialysis.\nOn day 8, an inferior vena cava filter was placed in order to prevent future episodes of pulmonary embolization. From day 10 and onwards, the patient was able to tolerate hemodialysis without increases in ICP. Hemodialysis was discontinued on day 21, as kidney function was rapidly returning. The patient was gradually weaned off sedation and mechanical ventilation; autonomic dysfunction ensued and was treated with baclofen and propranolol. On day 51 the patient was in a minimally conscious state (MCS) and was discharged from the NICU and transferred to a neurorehabilitation facility. Upon follow-up three months after discharge, she remained in MCS with signs of slow improvement. |
A 30-year-old woman was referred to our center one week after an open heart surgery for an ASD closure with a pericardial patch. Routine postoperative transesophageal echocardiography illustrated a large pedunculated and mobile mass (thrombosis) at the left atrial side of the interatrial septum at the level of the implanted pericardial patch ( and ). The patient's rhythm was normal sinus rhythm, and there were no factors predictive of atrial fibrillation. Extensive laboratory investigation as regards thrombophilic disorders revealed no pathologic values.\nAfter providing an informed consent, the patient was transferred to the operating room and placed on the operating room table in supine position. Upon the induction of general endotracheal anesthesia and placement of indwelling arterial and venous monitoring lines, the patient was prepped and draped in the usual sterile fashion from chin to groins, and a full midline vertical skin incision was performed in the sternum. The dissection was carried through the deeper planes until the sternum was scored and divided. With an oscillating saw, a small portion of the anterior pericardium was procured for the patch closure of the segment of the ASD during the procedure. Purse strings were deployed on the ascending aorta on the right and the atrial appendage. After systemic heparinization, central aortobicaval cannulation was done for CPB. Both caval veins were encircled with surgical tapes. After mild hypothermic CPB and cross-clamping of the aorta, intermittent cold cardioplegic infusion was administrated in an antegrade fashion into the aortic root. Through a standard right atriotomy, the old pericardial patch was opened and the left atrium explored for thrombosis. The thrombosis was attached via a stalk to the raw surface of the interatrial septum underneath the suture line at the site of the resection of the residual cribriform of the primary septum. The old pericardial patch and thrombosis were removed, and the ASD was closed with a new pericardial patch. De-airing was performed through the aortic root and cardiac apex before aortic declamping. The right atriotomy was closed in two layers with running 4-0 Viline sutures. Venous decannulation was followed by aortic decannulation and administration of protamine sulfate. All the cannulation sites were oversewn with 4-0 Viline sutures, and the cannulation sites were hemostatic. With the patient having good hemodynamics and hemostasis, the sternum was then closed with stainless steel wires. The subcutaneous tissues were closed in layers with reabsorbable monofilament sutures. The patient was transferred in very stable condition to the adult intensive care unit. |
A 62-year-old man with a history of hypertension initially presented with progressive development of gait dysfunction, urinary incontinence, and encephalopathy over the course of two weeks following four days of a gastrointestinal illness. Upon presentation to an outside hospital, he was unable to speak and due to his depressed mental status required intubation. He underwent an MRI of the brain on the first hospital day (HD) that revealed extensive supratentorial white matter hyperintensities extending from the periventricular region to subcortical fibers without contrast enhancement (see Figures and ). Thus he was started on 250 milligrams (mg) of intravenous (IV) methylprednisolone every 6 hours for presumed ADEM. He also underwent a lumbar puncture on HD #1 prior to administration of IV steroids that revealed an opening pressure of 20, with cerebrospinal fluid (CSF) containing 0 red blood cells, 47 white blood cells of which 85% were lymphocytes, glucose of 52, and protein of 114. He was empirically treated with acyclovir and ceftriaxone for a total of 5 days, which were discontinued after CSF bacterial cultures were negative. Blood cultures were also negative and a transthoracic echocardiogram was negative for evidence of vegetation to suggest endocarditis. Various viral titers including herpes simplex virus (HSV), Epstein-Barr virus (EBV), varicella zoster virus (VZV), and cytomegalovirus (CMV) were negative, as were serologies for CSF Lyme and Cryptococcus neoformans. In addition, CSF oligoclonal bands and myelin basic protein were negative. Serum inflammatory markers including ANA, C reactive protein, and sedimentation rate were not elevated. EEG showed diffuse slowing suggestive of moderate diffuse cerebral dysfunction without evidence of seizures or epileptiform activity.\nDespite treatment with high dose steroids for approximately 6 days at the outside hospital, his mental status did not improve and thus he was transferred to our facility to the neurological intensive care unit for further management. Upon arrival, the patient's GCS was 10T, with spontaneous eye opening, ability to localize to pain in the left upper extremity, and intact brain stem reflexes. Additionally, he displayed triple flexion in his bilateral lower extremities and decorticate posturing in his right upper extremity. A repeat lumbar puncture performed on HD #8 demonstrated a normal opening pressure, with CSF containing 0 red blood cells, 9 white blood cells of which 94% were lymphocytes, glucose of 79, and protein of 100. CSF bacterial cultures were negative, along with no evidence of active HSV or EBV infection. CSF cytology revealed the presence of white blood cells but was negative for malignancy. RPR was also negative. Two subsequent MRIs of the brain were performed on HD #13 and HD #21 showing no significant change in the appearance of the prior white matter lesions and no new contrast enhancing lesions. In addition, an MRI of the cervical and thoracic spine with contrast performed on HD #22 did not show any prior or new enhancing white matter lesions. With continued treatment with high dose IV steroids at 250 mg of IV methylprednisolone every 6 hours for another 7 days, his mental status improved and thus he was safely extubated on HD #10 and continued on a taper of oral prednisone. He underwent a brain biopsy on HD #13 that revealed multiple small foci of macrophage accumulation and widespread white matter inflammation secondary to demyelination (see Figures \n(h)). He was subsequently discharged on HD #63 to acute rehab on a prolonged oral steroid taper. His physical exam on discharge was notable for a GCS of 15, fully oriented, full strength on the left, and a residual right hemiparesis. Upon outpatient follow-up four months later, he made further improvements, now ambulating without assistance, cognitively back to his baseline, and independent in his activities of daily living, with a modified Rankin score (mRS) of 0. He underwent a follow-up brain MRI five months later that showed overall improvement in the diffuse white matter changes previously seen on initial presentation (see Figures and ). |
A 34-year-old gentleman was first referred to orthopaedic services with a history of persistent knee pain, located over the right femoral condyle near the origin of the lateral collateral ligament. Eleven years prior to presentation, the patient was involved in a road traffic accident where he sustained a fracture of the right femoral neck and ipsilateral shaft. Initial management consisted of open reduction, fixation of the femoral neck fracture with cannulated screws and the ipsilateral shaft fracture with plating. The patient later had a revision of the plate to a femoral nail. Union was subsequently achieved with the femoral shaft fracture; however, a significant external rotation deformity was noted, and discomfort to the knee.\nThe patient was referred on to our tertiary referral orthopaedic centre for femoral de-rotation surgery. An initial CT scan performed revealed an external rotation deformity of 45 degrees. The patient procedure included removal of femoral nail, osteotomy with de-rotation surgery, and subsequent exchange nail. The femoral nail was fixed proximally and locked into place distally, following the corrective 45 degrees of internal rotation achieved to the distal femur during osteotomy, performed under intraoperative radiographic guidance. The post-operative rehabilitation regime consisted of initial touch weight bearing only, with gradual increments in weight bearing status. At six months follow-up, the patient complained of distal lateral femur pain. A repeat CT scan was performed, revealing a delayed femoral union. This was initially thought to be the cause of the patient’s symptoms. The patient was admitted for dynamization of the femoral nail with an injection of bone graft substitute, and iliac crest graft. Despite eventual union, the patient continued to complain of pain at the distal lateral femur, with a cracking sensation on movement now noted.\nRepeat clinical examination revealed a palpable crepitus over the distal iliotibial band with a snapping sensation, as it appeared to catch. A palpable small, solid swelling was noted at the posterolateral right knee. Ultrasound and CT revealed intra-articular loose bodies lying within the lateral para-patellar gutter. The patient subsequently underwent arthroscopy with removal of loose bodies. Despite this, at six-months follow-up to arthroscopy, the patient continued to complain of posterolateral knee pain. MRI showed no evidence of abnormality to the popliteus tendon or muscle, but highlighted the fabella embedded within the lateral head of gastrocnemius at the posterolateral corner of the knee. (Image ). The fabella was also notable on radiographs (Image ) and CT (Image ). Ultrasound scan confirmed the fabella as mobile on movement and associated with tenderness. The diagnosis of Fabella Syndrome was made, and the patient planned for a Fabellectomy procedure.\nAn initial arthroscopy was performed to review the knee joint, and to potentially assist in surgical excision of the fabella. A complete diagnostic arthroscopy was performed, with the fabella unable to be identified through the posterior capsule. After arthroscopy, open fabellectomy was performed. In view of the patient’s extensive scarring from prior orthopaedic procedures, a short 2cm incision was made directly over the palpable fabella (Image ). The common peroneal nerve was identified and spared. Subsequent incision left a cuff of biceps femoris to protect the nerve. Gastrocnemius was split and incised directly over the fabella, which was removed in entirety (Image ). Histopathology confirmed the diagnosis.\nThe patient was followed-up at two and ten months postoperatively. At both follow-ups, he described the complete resolution of his posterolateral knee pain. |
A 21 year old male presented with a history of 6 months swelling in the right inguinal region. The swelling was noticed by the patient after a trivial fall while playing football. Since then the swelling was gradually increasing in size. The swelling was associated with dull aching pain which was localised to the inguinal region with no radiation of the pain. There was no weight loss.\nOn physical examination there was a palpable bony hard mass over the right inguinal region which was fixed to the underlying bone. The right femoral and other peripheral pulses were normal. There was no local warmth and erythema. The movement of the hip was free. Power and sensation of the right lower limb was normal.\nPlain radiography of the pelvis showed a huge expansile lytic lesion of the right superior pubic ramus (). A contrast computed tomography of the pelvis was done which demonstrated an expansile lytic lesion with cavities arising from the right superior pubic ramus. It approximately measured 5cm x 6cm (). CT angiography was done which showed compression and displacement of the femoral vessels. Reduced flow was seen in the femoral artery ().\nPreoperatively several differential diagnosis were kept in mind like primary ABC, ABC secondary to giant cell tumor or a malignant tumor like chondrosarcoma.\nWith the help of cardiovascular-thoracic surgeons anterior approach was taken to reach the tumor site (). The diagnosis of ABC was established by frozen section intraoperatively. Then complete excision of the tumor was done after sectioning the inguinal ligament to get fuller access to the tumor, which was later reconstructed(by suturing with vicryl no.2 and supplemented with rectus sheath as double breasting) The operative specimen consisted of a large osseous structure and many curetted fragments of the soft tissue. The tumor approximately weighed 542gm and size being 11.2cmx 6cm x 5cm(). The diagnosis was confirmed by histo-pathology (). The patient did well post operatively and till date (2 years since surgery) there is no recurrence (with persistence of bone gap). |
A 23-year-old girl reported to the department of oral medicine and radiology with progressive, nonpainful swelling of the gingiva on the labial aspect of the upper and lower anterior teeth with 6 months' duration. There was a history of evening rise in temperature and weakness over the last 3 months. The patient also had a loss of appetite over the last 4 months and a weight loss of about 4.5 kg during the last 8 months. There were no systemic problems, no cough with expectoration and no history of dental trauma or surgery in the affected area. Extraoral examination revealed no cervical lymphadenopathy. Intraoral examination revealed diffuse enlargement of the upper and lower gingiva on the labial surface of anterior maxillary and mandibular teeth []. On palpation, the swelling was slightly tender and firm. The rest of the OC was normal except for the few deep carious teeth. Differential diagnoses were enlargement due to drugs, infection and hematologic malignancy. The possibility of drug-induced enlargement was ruled out based on medical history. The biochemical tests were within normal limits, except for a marginal rise in leukocyte count (13 × 10 9/L) and an elevated ESR of 56 mm/h (Westergren method), which ruled out leukemia-associated enlargement and raised the possibility of one of the common causes of high ESR, TB. An incisional biopsy was carried out under LA in relation to the gingiva of the mandibular right central incisor, in collaboration with the department of periodontics and implantology. Histopathological examination was carried out that revealed clusters of epithelioid cells surrounded by a chronic inflammatory type of infiltrate. There was no evidence of caseating necrosis, but numerous Langhans giant cells were visible in the clusters of epithelioid cells suggestive of a “hard tubercle” [Figure and ]. To eliminate the possibility of localized granulomatous changes superimposed on an area of gingival enlargement, incisional biopsy was repeated in the remaining three quadrants. Histopathology showed similar granulomatous changes in all tissue specimens examined. The tuberculin (Mantoux) test was positive, suggesting tubercular infection. Chest radiography (posteroanterior view) revealed no abnormalities []. A computed tomography scan of the head and neck region was also performed to determine the status of the underlying maxilla and mandible. The scan did not reveal any bone abnormalities. A culture of the sputum, obtained by forceful coughing, was negative for M. tuberculosis. Special staining of formalin-fixed, paraffin-embedded tissue specimens for Mycobacteria, i.e., Ziehl–Neelsen and auramine–rhodamine stain, was negative. An immunologic test to detect antibodies against Mycobacterium in the patient's serum (ELISA) was positive. Polymerase chain reaction (PCR) assay was also carried out using six 5-μm sections of paraffin-embedded tissue to identify specific sequences of M. tuberculosis complex, with adequate controls. The DNA was used as an amplifying target for the sequence IS-6110, which is specific for M. tuberculosis. Positive PCR results confirmed the presence of M. tuberculosis in the tissue samples. In view of these findings, a final diagnosis of primary tuberculous gingival enlargement was made. In consultation with the patients' physicians, antitubercular therapy (ATT) was initiated. During this period, the patient was instructed not to undergo any ultrasonic scaling and polishing or surgical procedure within the OC and was warned about the chance of transmitting the disease to others via aerosol and salivary contamination. After completion of a 6-month regimen of basic periodontal therapy, which included scaling and root planning, oral hygiene instructions were instituted under CDC-issued guidelines. This resulted in significant regression of the enlarged gingivae in both the arches. Gingivectomy and gingivoplasty were performed to shape and contour the residual enlargement under universal aseptic conditions []. No recurrence of lesion occurred during 1-year follow-up []. |
A 53-year-old Hispanic man presented with dorsal laceration to his third and fourth DIPs with bleeding and loss of mobility. The patient was right-hand dominant, and just prior to arrival he sustained a crush/laceration injury at work to this hand. His right hand had become trapped in the blades of an industrial vacuum machine. The patient’s only complaint was pain to the third and fourth digits of his right hand. The patient had no past medical history, no prior surgeries and took only aspirin (81mg daily), folic acid, glucosamine and garlic tablets. His last tetanus shot was one year prior. The patient also denied tobacco use.\nOn physical examination; the patient was alert, ambulatory and talking, but in mild distress. He had a Glasgow coma scale score of 15. The only abnormal physical exam findings were as follows: Third digit of the right hand with a crush injury and avulsion of the nail bed. This was associated with complete extensor tendon deficit, with the tendon exposed on exam and a moderate amount of bleeding. The fourth digit was swollen and tender to palpation with a smaller skin defect over the dorsum of the finger with a puncture wound. There was no damage to the nail or nail bed of the fourth finger, but ecchymosis was noted to the finger pad.\nA radiograph showed a mallet injury to the third finger on the right hand without associated fracture. The fourth finger demonstrated an open fracture of the distal phalynx with DIP extensor distruption ().\nThe patient was given two grams of cefazolin intravenously. A digital block was performed with 1% lidocaine and a tourniquette applied to the base of both injured digits. Percutaneous fixation of the fourth finger was performed first to stabilize the distal fracture fragment using manual pressure and a standard 18-gauge needle. A similar procedure was then tried on the third finger to splint the digit in extension and allow for healing of the macerated tissue overlying the dorsum of the DIP, but the needle could not be advanced through the bony tissue with manual pressure. Verbal consent was obtained from the patient for use of an alternative fixation device. The needle driver for the EZ-IO® system was placed inside of a sterile glove by an assistant and was used to place the inner stylet of the IOVAD needle through the tip of the finger to achieve splint fixation in extension. Percutaneous needle fixation was recommended after phone consultation with the on-call orthopedic surgeon. Both needles were placed by the emergency physician caring for the patient, using simple tactile and visual guidance.\nThe patient was discharged home on cephalexin for 10 days and pain medicine with a bandage in place to protect the fingertips and exposed needles. Post-fixation films demonstrating alignment and needle comparison are shown in .\nThe patient followed up in the orthopedic clinic for needle removal and later physical therapy. He regained normal use of both digits. |
A 23-year-old primigravida at 36th week of gestation with singleton pregnancy was referred to our institute with severe hypertension, headache, and dizziness for past two days. She was hospitalized and diagnosed with severe preeclampsia, with a blood pressure of 210/150 mmHg on admission. However, there was no history of blurring of vision, vomiting or epigastric pain. Her antenatal checkups were irregular though she took iron and calcium tablets in the last fourth month of gestation but had neither undergone screening for aneuploidy nor any anomaly. During her late second trimester she was started with oral labetolol 100 mg tablets twice daily after the diagnosis of gestational hypertension was made. The patient had no personal or family history of hypertension. Her past medical information before gestation showed that she was healthy and without any similar symptoms. All her blood tests like liver enzymes, creatinine, and platelet counts were in normal range except for urine sample which showed proteinuria. The combination of proteinuria with hypertension on admission made the diagnosis of severe preeclampsia overt, so she was started with intravenous labetolol with gradual escalation of doses. Magnesium sulphate as per Pritchard regimen and analgesic for headache were added to her regimen. Even with these measures, her blood pressure continued to be at a higher level so we added calcium channel blocker to her treatment. Keeping in view the risks involved to mother and fetus, a decision for termination of pregnancy was taken and she delivered a live healthy baby weighing 2200 grams by emergency cesarean section under regional anesthesia.\nWe were surprised to notice the development of tremors, palpitation, and sweating with uncontrolled hypertension on seventh postoperative day which directed us to rethink other possible causes. Hence, we decided to go for further evaluation of the patient. On further investigations, her 2D echo test was unremarkable, thereby ruling out any hypertension of cardiogenic origin. However, her abdominopelvic ultrasound revealed a solid mass with fine cystic components of size 7.9x6.8x4.2 cm above her right kidney with a high suspicion of a tumor of adrenal origin (). With these findings, we proceeded with an abdominopelvic contrast-enhanced computed tomography which showed an arterial phase hyperenhancing lesion with dimensions of 5.4x6.3x6.4 cm in the right suprarenal location suggestive of pheochromocytoma (). We sought for the endocrinologist’s opinion who advised for evaluation of a 24-hour urine metanephrine, normetanephrine, and vanillylmandelic acid levels which were raised beyond the normal range which was highly suggestive of pheochromocytoma (). Results of endocrinology and surgical oncology consultations were obtained for optimization of the treatment before surgery, and she was started on an alpha -blocker followed by a beta blocker for adrenal suppression. After two weeks of adrenal suppression with controlled hypertension and with the help of multidisciplinary team comprising of surgeons, anesthesiologist, and endocrinologist, she underwent an uncomplicated laparoscopic adrenalectomy (). Her hemodynamic condition was monitored in the intensive care unit for 24 hours and remained stable. Histologically, the tumor was confirmed to be a pheochromocytoma (). During the follow-up, her urinary normetanephrine excretion remained normal as well as her vital signs. |
A 64-year-old female who had hypertension was admitted with a growing asymptomatic mediastinal mass that was detected incidentally through a medical checkup. The mass had increased in size and appeared to be about twice as large as it had been 2 years earlier according to the chest radiographs. Chest radiography showed a round mass in the middle mediastinum abutting with the left cardiac border (). The blood pressure was 125/79 mmHg and the heart rate was 80 beats/min without any cardiac murmurs and with clear breathing sounds. Laboratory tests including a complete blood cell count, electrolytes, chemicals, and coagulation tests were unremarkable. Her electrocardiography showed a normal sinus rhythm. On 2-dimensional echocardiography, the large cystic mediastinal mass (5.0×4.7 cm) was adjacent to the main pulmonary artery. Computed tomography (CT) showed a 5-cm well defined homogenous enhancing mass attached to the anterolateral wall of the left ventricle and mildly compressing the main pulmonary artery (). Although the left anterior descending coronary artery was displaced posteriorly by the mass, the reconstructed CT image revealed that it had no significant narrowing (). Magnetic resonance imaging (MRI) showed a mildly enhancing cystic lesion with heterogeneous signal intensity on tumor 1 (T1), and tumor 2 (T2) images suggesting the possibility of a neurogenic tumor such as schwannoma with hemorrhagic degeneration ().\nA median sternotomy was used and we could see the mass was attached to the epicardium of the right ventricle after pericardiotomy (). Dissection of the mass was difficult because of severe adhesion and proximity to the left anterior descending coronary artery. We decided to apply cardiopulmonary bypass because of a risk of perforation of the right ventricle. The ascending aorta and single right atrial cannulation was used. Except for the right ventricular adhesion, excision of the mass along the layer was simple. The cyst was connected to the epicardium through a feeding vessel. During dissection of the mass, the feeding vessel was cut accidentally, and the mass collapsed immediately. The mass was filled with a blood-like fluid. After ligation using double silk tying and clipping, the cystic mass was removed.\nThe gross pathologic specimen was a unilocular cyst (5.5×5×2.8 cm) of which the outer surface was yellowish white with multifocal hemorrhage and the inner surface was yellowish white with multiple yellowish pigments without a solid portion. A microscopic section showed membranous fibrous tissue with calcification and histiocytic infiltration () that looked like a vessel wall with atherosclerotic changes. The patient was discharged on the 6th post-operative day without any complications. The follow-up 2-dimensional echocardiography did not show any other abnormalities. |
A 15-year-old boy was referred to us from another center with complaints of lower abdominal pain, right lower limb swelling and pain, inability to walk, and rectal bleeding for 1 month. Two surgeries, 7 days and 5 days before presentation, were performed recently to remove K-wires inserted in the same hip one year back for slipped capital femoral epiphysis (SCFE). He sustained SCFE following a fall while practicing karate. The fixation was done with 2 K-wires which were buried under the skin during that surgery and the tips of the wires were not bent ().\nThe postoperative period was uneventful and the patient was able to perform regular activities 12 weeks postoperatively. He developed the abovementioned complaints one month prior to presentation for which he was taken to the center where first surgery was done. During this surgery, one of the K-wires broke and another could not be retrieved through the hip incision ().\nAfter surgery, the patient's condition deteriorated and he was brought to our center. At the time of presentation to us, the patient had high grade fever, tachycardia, weak pulse, unilateral right lower limb edema, and anxious look. On examination, a sutured wound in the right hip region and laparotomy wound were present. Rectal examination revealed fresh blood and bowel sounds were decreased. Movements of hip joint were restricted and extremely painful. Blood investigations revealed profound anemia, leukocytosis, and hypoalbuminemia. CT angiography revealed large pseudoaneurysm with a surrounding hematoma in the region of iliacus muscle, which was seen to be in direct communication with the caecum and large bowel loops as shown in Figures , , and .\nThere was active contrast extravasation into pseudoaneurysm and large bowel loops. Direct communication of a branch of the external iliac artery into pseudoaneurysm was seen. Hematoma in the right thigh was also noted. There was reduced flow in the rest of the arterial system supplying lower limb. No abnormalities of the venous system in the right lower limb were seen. On CT scan, a deformed right femoral head with postpinning defect, articular changes, and subluxation was visualized. After initial resuscitation, exploratory laparotomy was performed immediately with subsequent ligation of feeding vessel to the pseudoaneurysm and appendectomy (Figures and ).\nDays after surgery the patient was recovering well and his rectal bleeding stopped. However, his condition started deteriorating again with worsening of general condition, high grade fever, and other features of septicemia. Ultrasound abdomen revealed heterogeneous collections in the right medial thigh and abdomen, which suggested infection of the remaining hematomas. Exploratory laparotomy and exploration of medial thigh were done. Around 2 liters of pus was drained intraoperatively from both wounds and a drain was placed in situ. The condition of the patient improved gradually and the patient was discharged after 2 months on crutches. On follow-up after six months, the patient was walking comfortably without any aid and he has returned to his day to day activities. The surgical wounds have healed and scars appear healthy (). Radiograph at follow-up shows deformed femoral head on the right side (). True shortening of 2 cm of the right lower limb was found on follow-up, but no surgical intervention was done since the patient did not have any complaints. |
A 71-year-old woman visited an otorhinolaryngologist with 5-year history of nasal congestion. She was diagnosed as having a nasal polyp. Since IP was found at biopsy, she was referred to our hospital in order to undergo surgery. In the nasal cavity, the tumor that was suspected to be IP was observed in the middle and inferior meatus (). A CT scan of the paranasal sinus revealed a shadow that occupied the maxillary sinus and deviated to the ethmoidal sinus and a defect in the posterior wall and medial bone of the maxillary sinus (). Preoperative squamous cell carcinoma (SCC) antigen level was high at 11.0 ng/mL. We suspected a cancerous change of IP or a complication of cancer and performed another biopsy, concluding that the condition was IP. The lesion was examined by CT and MRI to locate the origin of IP. The CT did not reveal localized thickening of the bone, and the MRI did not show secondary changes in the maxillary sinus or a distinctive mass of serpentine cerebriform filamentous structure (). Findings revealed erosion or defects of the bone in the posterior and medial walls of the maxillary sinus, but the origin of IP could not be identified. It was believed that IP originated from a wide area. The patient refused to undergo lateral rhinotomy and was therefore informed that ESS and EMMM procedures would be used concomitantly and that a transantral approach (TA), Coldwell-Luc surgery, would be used if necessary. The patient gave her consent. The surgery was performed under general anesthesia. The uncinate process was removed and IP was found to be deviating into the nasal cavity and the normal mucosa in the anterior ethmoidal sinus. The frontal and maxillary sinuses were opened wide, while the posterior ethmoidal sinus was not opened. When observed at a 70-degree endoscope, the tumor in the maxillary sinus was seen to have adhered to the posterior, superior, and medial walls and the origin of IP was widely extensive, as anticipated preoperatively. The anterior and inferior lesions could not have been sufficiently resected if approached from the fontanelle of the maxillary sinus. Therefore, EMMM was selected (). Slightly posterior to the pyriform aperture, the mucosa was incised from the superior portion of the inferior turbinate towards the nasal floor, and the nasal mucosa was elevated from the lateral wall of the nasal cavity. The mucosa on the lateral side of the inferior meatus was detached from the medial side of the maxillary sinus. The inferior turbinate bone was observed endoscopically and the conchal crest was cut with a chisel, allowing for the inferior portion of the nasolacrimal duct to be observed and for the inferior turbinate bone to be freely moved medially. As the nasolacrimal duct could be clearly observed by endoscope, the tumor deviating to the inferior meatus and the lateral mucosa and the bony wall of the inferior meatus could be sufficiently resected. The lacrimal process of the inferior turbinate, the frontal process of the maxilla, and the inferior portion of the lacrimal bone were ground with a 2.5-mm Curved Diamond DCR Bur (Medtronic), which made superior portion of lacrimal duct visible. Maxillary sinus was widely opened from the inferior meatus side so that endoscope could be inserted from inferior meatus towards maxillary sinus. The anterior, inferior, and medial walls of the maxillary sinus could be observed endoscopically. Inverted papilloma had adhered to the anterior, medial, and inferior walls; that is, it adhered to the entire circumference of the maxillary sinus. It was considered that there were all IP-involved mucosae without normal mucosa inside the maxillary sinus. The posterior portion could be observed with a 0-degree endoscope and the anterior portion could be observed by 70-degree endoscope. Inverted papilloma could be resected with highly curved forceps. The pyriform aperture and the mucosa of the inferior turbinate were sutured and the surgery was completed. Since part of the tumor had eroded the bone of the posterior wall, destroyed the inferior meatus, and deviated to the nasal cavity, a complication of cancer was suspected and an intraoperative consultation was performed. No malignant finding was obtained. In this case, there was no thickening of the bony wall of posterior wall of the maxillary sinus. We did remove the mucosa only without thinning of the bony wall of the maxillary sinus. The patient was free from recurrence 3 months after the surgery (). The levels of SCC antigen had decreased to 2.9 ng/mL. Findings in the nasal cavity were similar to those after ESS (), and the patient did not complain of either an empty nose or dryness in the nose. The patient had mild numbness around the lips, but no symptom in the eyes, such as lacrimation. |
We present the case of a 56-year-old female with history of syncope due to third degree atrioventricular heart block presenting initially with onset of stroke symptoms six days after pacemaker placement and two days after hospital discharge. At 5 PM she developed abrupt onset of left facial droop along with left upper and lower extremity weakness. The patient was initially treated at an outlying hospital and received alteplase at 6:35 PM for treatment of acute ischemic stroke.\nA chest radiograph performed at the outlying hospital prior to alteplase administration demonstrated an enlarged cardiac silhouette when compared to prior radiographs showing only borderline cardiomegaly. Upon administration, the patient reported mild chest pain and was given nitroglycerine and morphine. Her chest pain resolved and she was transferred to our comprehensive stroke center for admission. The patient presented to our emergency department at 10:10 PM with a heart rate of 122 beats per minute (bpm) and a blood pressure of 109/41 millimeters of mercury (mmHg).\nAt 11:20 PM the patient went for a computed tomography angiogram (CTA) after an initial assessment by the emergency physician in consultation with the stroke-team attending physician. After CTA at 10:28 PM, she was documented to have a blood pressure of 49/25 mmHg and heart rate of 109 bpm. She was returned to the resuscitation bay for re-evaluation. Cardiac tamponade was suspected due to the extreme hypotension in the setting of thrombolytic administration after recent pacemaker placement.\nOn reassessment, the patient had become confused with a Glasgow Coma Scale of 14. The emergency physician performed a POCUS, which demonstrated a pericardial effusion with features of cardiac tamponade including diastolic collapse of the right ventricle (). At that point the diagnosis of cardiac tamponade was made. The patient was alert and responsive, so an intravenous bolus of normal saline was given while a stat surgical consult was obtained. The surgical team evaluated the patient at the bedside within minutes and was able to review the POCUS findings. As the patient was conscious, they elected to take her immediately to the operating room rather than perform a bedside pericardiocentesis.\nWhile in the operating room, approximately 400 milliliters of coagulated blood were evacuated from the pericardial sac with 150 milliliters of surgical bleeding. The operative report notes resolution of tachycardia following this intervention with heart rate trending down to a range of 80–90 bpm with concomitant improvement in blood pressure. She was discharged two days post-operatively with a pericardial catheter in place. Echocardiogram performed on day of discharge noted a small, residual pericardial effusion. |
A 48-year-old female was referred to the surgical oncology clinic for evaluation of a pancreatic mass. This was found incidentally on workup for an endometrial stromal sarcoma, for which she had undergone a total abdominal hysterectomy with bilateral salpingo-oophorectomy. The patient was asymptomatic.\nHer past medical history was significant for uterine sarcoma and for venous thromboembolism which led to a pulmonary embolus but was otherwise unremarkable. Her physical exam was unrevealing, as was her serum laboratory evaluation, with normoglycemia, normal hepatobiliary function, normal pancreatic enzymes, and no elevation in carbohydrate antigen 19-9, carbohydrate antigen 125, or carcinoembryonic antigen. CT and MRI imaging () revealed a mass at the neck of the pancreas, measuring 2.9 cm in its largest dimension, as well as the absence of the distal body and tail of the pancreas. The mass closely abutted the confluence of the portal vein and superior mesenteric vein, but there was no invasion. She underwent biopsy of this mass via endoscopic ultrasound which revealed features consistent with a well differentiated neuroendocrine tumor. The tumor was determined to be nonfunctioning given the absence of systemic symptoms and laboratory data to suggest hormone production.\nThe patient underwent resection of this mass via spleen preserving laparoscopic approach. Intraoperative images confirmed the absence of the distal body and tail of the pancreas (). Negative margins were achieved with this resection, and the pancreatic head and uncinate process were preserved, as were the splenic vein and artery. The pancreatic parenchyma was transected using a linear cutting stapler, with a closed staple height of 1.5 mm, and the remnant pancreatic neck was buttressed with an omental patch. A 19 Fr fluted Blake drain was placed at the resection margin at the time of surgery. Final pathology revealed a grade 1 well differentiated pancreatic neuroendocrine tumor (). Despite our intraoperative efforts to avoid it, the patient's postoperative course was significant for a pancreatic duct leak, which was well controlled by her drain, and she was discharged home on POD 4. Her drain was removed on POD 23. She had no evidence of diabetes or pancreatic insufficiency on follow-up evaluation. Her case was discussed at our multidisciplinary tumor board and no further treatment for this tumor was recommended. |
Mr. BH, 39 years old, right-handed, presented with a Bennett fracture of the first right metacarpal following a work accident. The fracture was initially treated in another institution by reduction and percutaneous pinning using Iselin’s technique. Three weeks later, the patient developed an inflammatory reaction around the entry point of the distal pin, at the second metacarpal. A purulent discharge led to early removal of the distal pin. The isolated organism was a methicillin-sensitive Staphylococcus aureus. Regular topical treatment was given, and antibiotic therapy was initiated for five weeks based on laboratory results. Bone healing of the Bennett fracture was obtained in the sixth week after the accident, and the proximal pin was removed.\nThree months later, the patient was referred to us by his doctor because of persistent pain at the second right metacarpal, accompanied by attacks of inflammation and intermittent purulent discharge. Standard hand X-ray demonstrated the presence of an area of osteolysis surrounded by osteocondensation at the second metacarpal, indicating a focus of osteomyelitis along the path of the distal pin (). An MRI was performed on the hand. It confirmed the diagnosis, revealing the extent of the focus of osteomyelitis, the path of the fistula, and the inflammatory extension into neighboring soft tissues ().\nSurgical treatment called for the induced membrane technique.\nThe first stage included a segmental resection of the area of osteomyelitis through a dorsal approach with excision of the reformed tissue while preserving the index finger extensor tendon.\nThe second metacarpal was stabilized with a mini external fixator, and a cement spacer was interposed ().\nAntibiotic therapy was initiated for six weeks based on sensitivity testing. The external fixator was then removed, and the hand temporarily immobilized with a splint until there was healing along the path of the inserts.\nThe second stage was performed in the eighth week. The cement spacer was removed and the biological chamber was filled with a spongy bone graft taken from the ipsilateral iliac crest. Internal fixation was performed with a mini plate while preserving the induced membrane. The postoperative period was uneventful. Bone healing was observed three months later () with complete disappearance of pain. The patient has regained normal use of the hand with a Quick DASH score of 11 and complete mobility (. We are currently at the 36-month follow-up. X-ray of the hand shows incorporation of the graft and evidence of corticalization (). |
A 21-year-old Ghanaian man presented initially with a 6-year history of progressively worsening pain and swelling in both knee and wrist joints, which moderately affected his activities of daily living. He reported episodes of fever and chills in the past, although these were absent at the time of presentation. He had polyuria, polydipsia, and nocturia but he did not have weight loss, headaches, or loss of vision.\nHe had profuse diaphoresis particularly of his face, hands, and feet but did not have any other symptoms of hyperthyroidism. He had noticed an increase in the size of his hands and feet and a change in his facial appearance which were his main concerns. He had been treated with analgesics in the past, which only transiently relieved his pain.\nThere was no past medical history of diabetes or sickle cell disease. He had been diagnosed as having chronic hepatitis B infection 4 years prior to seeing us but was not on any treatment. There is no family history of diabetes, sickle cell disease, or a presentation similar to his.\nHe is the second of three children of his parents who are both alive; no sibling has any stigmata of PDP. His father has three other children with another woman who have no stigmata of PDP. His mother has one surviving sibling with four children of whom none have stigmata of PDP. His other maternal cousins also do not have any stigmata of PDP.\nHis illness has taken a psychological toll on him because he has been unable to continue his education after secondary school although he excelled in his examinations and got a scholarship to the university. He is usually at home indoors, because of people’s comments about his appearance when he goes out. His older sister has had two suitors renege on their promise to marry her on meeting him for fear the disorder could be familial and be transmitted to their offspring.\nAn examination revealed a young man with coarse facial features, hyperhidrosis, cutis verticis gyrata (Fig. ), pectus excavatum, doughy palms, spade like hands and feet (Fig. ), digital clubbing (Fig. ), and pitting pedal edema at his ankles with profusely diaphoretic hands and feet. There was no cyanosis, jaundice, skin rash, or any stigmata of chronic liver disease. He had visual field defects in the superior temporal quadrant bilaterally on confrontation, mild wasting and weakness of proximal muscles, and an antalgic gait. His cardiorespiratory and abdominal examinations were normal.\nSignificant musculoskeletal findings included massive effusions of both knees with striae of the overlying skin (Fig. ) associated with limited range of movement. There was also evidence of bone expansion at his wrist joints without soft tissue swelling, tenderness, or warmth. There was reduced flexion and extension as well as crepitus at his wrist joints. He also had enlarged proximal interphalangeal (PIP) joints bilaterally with a good handgrip.\nLaboratory tests revealed mild normocytic normochromic anemia with hemoglobin of 10.9 g/dl. However, the following tests were within normal limits: erythrocyte sedimentation rate (ESR), fasting plasma glucose at 5.0 mmol/l, serum insulin-like growth factor 1 (IGF1), liver, renal, thyroid function tests, and serum corrected calcium of 2.32 mmol/L. His serum albumin level was 33 g/L (35–50 g/L). Preliminary investigations for a rheumatological condition such as rheumatoid factor, anticyclic citrullinated peptide (anti-CCP), antinuclear antibody (ANF), and creatinine kinase (CK) were all normal. A chest X-ray, echocardiogram, abdominal ultrasound, pelvic ultrasound, and magnetic resonance imaging (MRI) of his brain were also normal with no pituitary lesion seen; these findings ruled out rheumatological diseases, cardiopulmonary diseases, or acromegaly from a pituitary adenoma as a cause of the clinical features. He has financial constraints as the family is unwilling to support him because he refused to seek alternative treatment.\nArthrocentesis done under aseptic conditions yielded approximately 700 ml of straw colored, normal viscosity aspirate per knee joint. There was residual effusion after this therapeutic and diagnostic aspiration. Gram stain, culture, cytology, cell count, and analysis for crystals were normal with no bacterial growth.\nX-rays of his lower limbs showed periosteal thickening of the medial cortices of both femurs with sparing of the lateral cortices (Fig. ). There was uninterrupted thickening of the periosteum of both lateral and medial cortices of his tibia and fibula (Fig. ). Effusions of both knee joints were noted. The X-rays of his wrist joints, radius, and ulna showed diffuse bilateral symmetric periosteal thickening with marginal irregularities of both ulnae and medial aspects of both radial shafts (Fig. ). There was expansion of the ulna shafts with flaring of both distal radii and a suggestion of cortical thickening in the ulna aspects of the second to fourth digits of both hands (Fig. ).\nThe effusion accumulated rapidly within a week. He was initiated on prednisolone 40 mg daily, which was reduced by 10 mg per week over 6 weeks. He was also given diclofenac 75 mg twice a day and omeprazole 20 mg twice a day over a month. Physiotherapy was started with active and active-assisted exercises of both upper and lower limbs. A bone biopsy was considered but was not done; bone scans or genetic testing were not done either. Three weeks after admission, he was discharged on prednisolone 10 mg which he took until week 4 and was weaned off by week 6. His pain improved but did not resolve completely and he was walking unaided and performing tasks of daily living better than when he was admitted. He is currently on celecoxib 100–200 mg pro re nata (PRN; as needed) and physiotherapy.\nAt a review, 3 weeks after discharge, with his father, we noticed his father had clubbing of his fingers (Fig. ). There were no other symptoms and signs of pulmonary disease or hypertrophic osteoarthropathy in his father. Genetic testing was considered then but this was not available in our hospital and our patient’s father declined the offer to screen him for cardiopulmonary disease with X-rays. The time course of our patient’s illness is shown in Fig. . |
The first patient is a 58-year-old man who was a previous smoker who presented to an outside hospital with abdominal pain and was diagnosed with gallstone pancreatitis. However, despite having a laparoscopic cholecystectomy, he continued to have episodes of pancreatitis yearly for the next 3 years. Computed tomography (CT) scan demonstrated a pancreatic duct dilated throughout its course to a maximum size of 8 mm without a discrete pancreatic mass in the head of the pancreas. Endoscopic retrograde cholangiopancreatography confirmed a dilated pancreatic duct with a suspected filling defect of the common bile duct, after which the patient underwent sphincterotomy and stent placement.\nHe then presented to our institution for consultation. Further workup with endoscopic ultrasound (EUS) was performed, which also confirmed the dilated pancreatic duct, but noted irregular contour of the duct. In addition, free mucin was seen exiting the main papilla, and there was a papillary growth noted in the pancreatic duct in the head of the pancreas, all of which was consistent with a main duct IPMN (). Preoperative laboratory values, including CA19-9, were within normal limits. Of note, it was unclear if the dilatation of the pancreatic duct was secondary to obstruction from the mass in the pancreatic head or due to main duct IPMN involving the entirety of the duct.\nHe was scheduled for surgical resection after the stated workup. He was planned for a Whipple procedure (pancreaticoduodenectomy) with possible total pancreatectomy if intraoperatively the main duct was found to be involved by IPMN with high-grade dysplasia. Transection of the pancreatic neck identified cells of high-grade dysplasia free floating near the margin (although exclusive of the margin). In addition, upon probing the pancreatic duct in the tail of the pancreas, the probe did not slide smoothly, suggestive of additional growths in the duct. Due to his young age and high risk of developing pancreatic cancer, a completion pancreatectomy and splenectomy were performed. He was reconstructed in a Roux-en-Y manner. He recovered well from surgery without any postoperative complications.\nHis final pathology demonstrated a 1.4 cm moderately differentiated colloid carcinoma arising within main duct IPMN (intestinal type) with high-grade dysplasia in addition to multiple foci of pancreatic intraepithelial neoplasia. Staining of the tumor was strongly positive for MUC2 and weakly positive for MUC1 and MUC5. The tumor involved the peripancreatic soft tissue, but all margins were negative. 0 of 39 lymph nodes was involved with tumor. His final pathologic staging was pT3N0Mx. He was, therefore, referred for medical oncology consultation. He was recommended for adjuvant chemoradiation with gemcitabine before and after fluorouracil-based chemoradiation, which he is currently receiving and tolerating well. |
Patient n. 2 was a 60-year old female, diagnosed with suffering from POAG for ten years, under treatment with hypotensive drugs and regularly controlled IOP twice a year which appeared maintained within normal ranges. During a control visit in January 2016, the patient had reported irritating symptoms of eye discomfort, described as burning, itchiness, and feeling sand, mainly in her right eye (RE), over the last six months. The situation had been managed with the use of several types of tear substitutes, none of them successful in symptom relief. Symptoms were scored in the RE as OSDI of 74 out of 100 and a VAS score of pain of 85 mm out of 100. In the left eye (LE) the symptoms were defined by the patients as light and acceptable: OSDI score was 22 out of 100 and VAS 21 mm out of 100. Slit lamp evaluation had not shown epithelial damage, and there were no signs of inflammation, but only a reduced Tear Film Break-Up Time (TFBUT) of 4 seconds in RE and 8 seconds in LE had been recorded. A therapy with hyaluronic acid (HA) based tear substitute to be administered 4 times/day in both eyes was prescribed.\nIn March 2016, the patient was proposed to receive in her RE a treatment with topical CBS, as a compassionate unconventional therapy, with the aim to reduce the pain symptoms for which the previous therapeutic attempts had turned to be unsuccessful. The therapy for the contralateral LE was maintained with HA based tear substitutes.\nThe patient signed the specifically designed informed consent and started administration of the CBS eye drops in April 2016, with the posology of 0.4 ml (8 drops) in RE, each day for a total of two months. In the GF dosages determined for the two CBS lots were administered during the first and second month.\nOn September 2016, the patient reported a significant relief from subjective symptoms of discomfort; in RE the OSDI was 28 out of 100, with VAS: 32 mm out of 100. Also in LE a reduction was observed, with OSDI score determined as 16 out of 100 and VAS 15 mm out of 100. During this visit, also the IOP was measured, which resulted in the normal range in both eyes and the analyses of the visual field tests performed since 2006.\nIn the mean deviation (MD) values recorded over several visits performed from December 2006 through September 2016 were graphed. A significant progressive lowering in MD values was observed which was followed by an important amelioration in correspondence with the period of treatment with CBS, in the figure highlighted with the arrow. It has to be noted that the increase in MD values was recorded either in the treated right eye or in the untreated left eye. Moreover, the MD values recorded in September 2016, four months after the end of the CBS eye drop administration, showed in both eyes a further amelioration.\nThe improvement is also demonstrated by the analysis of the PSD shown in . A progressive worsening in PSD values had been observed over ten years, whereas a rapid change was recorded in correspondence with the CBS eye drop treatment, either in the treated right eye or in the untreated left eye.\nIn the central 30-2 visual field tests before ((a): right eye RE; (b): left eye LE), at the end (c, d) and after four months (e, f) from the end of the CBS eye drop treatment were shown. An amelioration of the defect was observed in all the four quadrants in both the treated RE and the untreated LE.\nTaking together these observations, a positive effect also in the left untreated eye could be recorded, which suggests a neural cross-talk mechanism between the eyes. |
A seventy-five-year-old female was referred by her general practitioner for management of a discharging sinus at the site of an open appendicectomy scar. The discharging sinus was associated with abdominal pain in the right iliac fossa. The patient reported that the pain commenced a month prior to presentation with the evolution of an erythematous area surrounding the scar that slowly evolved to a discharging sinus. Sixty years prior to presentation the patient had an open appendicectomy for management of clinical appendicitis. Unfortunately, due to the timing of presentation no record of that presentation or procedure existed for review. The patient reported no abdominal issues in her history after appendicectomy prior to this event.\nThe patient's past medical history included ischaemic heart disease for which she had cardiac stents and a cerebral artery aneurysm that had been clipped approximately 21 years prior to presentation. Regular colonoscopies were unremarkable and there was no documented or pathological history to suggest any inflammatory bowel disease. Her regular medications included aspirin, atorvastatin, and metoprolol, and she was known to be allergic to penicillin and cephalosporins.\nExamination of her abdomen revealed a retracted appendicectomy scar with surrounding erythema and a small sinus discharging faeco-purulent material. Her abdomen was soft and nondistended with tenderness to palpation in the right lower quadrant with associated guarding. Biochemical investigations were unremarkable. Computed tomography (CT) of her abdomen and pelvis was performed and suggested chronic tethering of the lateral wall of the ascending colon to the adjacent abdominal wall with herniation of part of the lateral wall of the ascending colon through a defect in the oblique muscles with fistulation from this hernia to the skin surface ().\nThe initial treatment goal was to aim for a “controlled fistula.” A controlled fistula refers to an enterocutaneous fistula without evidence of sepsis or localised infection []. The patient was treated with a course of intravenous clindamycin and metronidazole for five days and was discharged home with oral equivalent two-week course. The choice of antibiotics was based on empirical therapy for common gastrointestinal flora with the patient's allergies taken into consideration. At the time of discharge there were no features of abdominal wall cellulitis, and there was no reported or elicited abdominal pain. A controlled output from the sinus remained and was managed with simple absorptive dressings.\nThe patient was admitted for a definitive surgical procedure several weeks after her initial presentation. The aims of the procedure included refunctionalisation of the entire bowel, resection of the fistula and any associated bowel, and secure abdominal wall closure. The procedure commenced with en bloc resection of the appendicectomy scar with careful resection around the fistula tract while tracking its course into the intraperitoneal space. The fistula was found to be intimately related to the distal caecum that was adherent to the anterior abdominal wall. The specimen was liberated from the caecum using a linear stapler and was sent for histopathological analysis. The specimen consisted of a piece of fibrofatty tissue measuring 85 × 55 × 30 mm with an overlying ellipse of skin measuring 73 × 20 mm with an attached portion of intestine measuring 12 mm in length and 35 mm in diameter. On the skin an old scar measuring 55 mm was identified, and serial sections identified a sinus filled with faecolith measuring 3 mm in diameter. Sections of the specimen were examined microscopically that showed skin with underlying adipose tissue and a portion of a stump appendix showing mucosa with underlying submucosa and muscle layer. Surrounding tissue showed fibrosis, vascular proliferation, and foreign body type giant cell reaction. There was no evidence of malignancy. Overall the features favoured a stump appendix with a fistula tract.\nThe patient was admitted to a surgical ward for postoperative management. This was uncomplicated and she was discharged home five days after procedure. To date the patient has had no complications. |
Mr. BH, 39 years old, right-handed, presented with a Bennett fracture of the first right metacarpal following a work accident. The fracture was initially treated in another institution by reduction and percutaneous pinning using Iselin’s technique. Three weeks later, the patient developed an inflammatory reaction around the entry point of the distal pin, at the second metacarpal. A purulent discharge led to early removal of the distal pin. The isolated organism was a methicillin-sensitive Staphylococcus aureus. Regular topical treatment was given, and antibiotic therapy was initiated for five weeks based on laboratory results. Bone healing of the Bennett fracture was obtained in the sixth week after the accident, and the proximal pin was removed.\nThree months later, the patient was referred to us by his doctor because of persistent pain at the second right metacarpal, accompanied by attacks of inflammation and intermittent purulent discharge. Standard hand X-ray demonstrated the presence of an area of osteolysis surrounded by osteocondensation at the second metacarpal, indicating a focus of osteomyelitis along the path of the distal pin (). An MRI was performed on the hand. It confirmed the diagnosis, revealing the extent of the focus of osteomyelitis, the path of the fistula, and the inflammatory extension into neighboring soft tissues ().\nSurgical treatment called for the induced membrane technique.\nThe first stage included a segmental resection of the area of osteomyelitis through a dorsal approach with excision of the reformed tissue while preserving the index finger extensor tendon.\nThe second metacarpal was stabilized with a mini external fixator, and a cement spacer was interposed ().\nAntibiotic therapy was initiated for six weeks based on sensitivity testing. The external fixator was then removed, and the hand temporarily immobilized with a splint until there was healing along the path of the inserts.\nThe second stage was performed in the eighth week. The cement spacer was removed and the biological chamber was filled with a spongy bone graft taken from the ipsilateral iliac crest. Internal fixation was performed with a mini plate while preserving the induced membrane. The postoperative period was uneventful. Bone healing was observed three months later () with complete disappearance of pain. The patient has regained normal use of the hand with a Quick DASH score of 11 and complete mobility (. We are currently at the 36-month follow-up. X-ray of the hand shows incorporation of the graft and evidence of corticalization (). |
A 53-year-old right handed female was diagnosed with relapsing remitting MS in 2011 after clinical relapses in 2006, 2010, and 2011 and an MRI consistent with demyelination, none of which were treated with CR. She was started on interferon β-1a 30 μg IM weekly, a disease modifying therapy for MS, shortly after diagnosis. She had a sensory relapse in 2012 that resolved without CR treatment. Past medical history was significant for chronic calcific pancreatitis that was not autoimmune in nature; investigations by gastroenterology were negative and it was felt to be idiopathic in nature. There was no history of alcohol abuse or acute pancreatitis, despite a past history of a cholecystectomy. There was no known history of hypertension (HTN) and her blood pressure (BP) in the past was around 125/85 mmHg.\nIn June 2013 she presented with diplopia. A new intranuclear ophthalmoplegia (INO) was identified and she was diagnosed with a relapse. Treatment with high dose CR was offered and she was started on 1250 mg oral prednisone for 5 days with no taper. On day one of CR, she noted insomnia, dizziness, general malaise, and a headache described as dull and gradual in onset. Upon completion of the pulse CR treatment, her symptoms persisted and she therefore came to the Emergency Department. She was not seen by a physician as she felt that the wait was too long and left. Over the following week, her headache worsened and she re-presented to the Emergency Department, describing the headache as starting in the occipital region and migrating over time to be holocephalic. The pain was rated as 10/10 but continued to be dull and aching. No nausea or vomiting, visual changes, or new neurologic symptoms were present. Her BP was 199/110 mmHg and heart rate was 78 beats per minute and regular and general medical examination was within normal limits. The neurologic examination was consistent with the findings prior to the onset of the relapse; her INO and diplopia had resolved. Investigations in the Emergency Department included a normal electrocardiogram with normal sinus rhythm and no evidence of left ventricular hypertrophy, normal urinalysis including no protein or blood, and unremarkable pelvic/renal ultrasound including no renal calculi. An MRI of the head and C-spine was performed. In addition to T2 hyperintensities in the brain and the cervical cord consistent with MS, unchanged since the previous MRI six months earlier, there was also abnormal T2 signal in both thalami, the posterior occipital and posterior parietal white matter extending into the vertex. Mild local sulcal effacement associated with these new lesions was also noted. There was no diffusion restriction noted with these lesions. The findings described above were consistent with a diagnosis of PRES (). She was admitted to hospital; her BP was monitored and remained relatively unchanged for the next 48 hours despite treatment with hydrochlorothiazide and amlodipine. Her BP subsequently responded to labetalol and amlodipine and was stabilized at approximately 130/85 mmHg and her headache resolved. Investigations for other causes of PRES as well as secondary causes of HTN were performed. Laboratory investigations included serum sodium 135 mmol/L, serum potassium 3.0 mmol/L, serum creatinine 79 μmol/L, serum urea 4.1 mmol/L, serum calcium 2.21 mmol/L, and serum albumin level 43 g/L. Her body mass index was 24 kg/m2, she did not have signs of Cushing's syndrome or thyroid gland disease, there were no changes of hypertensive retinopathy, and kidney function was normal. Anti-nuclear antibody (ANA) was negative. A urinalysis was negative for protein and hematuria. She was not pregnant. Her medications at presentation included nortriptyline and interferon B-1a only, and she was on no over-the-counter medications that affect BP or are associated with PRES. A 24-hour urine collection for free cortisol and fractionated metanephrines level was done in hospital 19 days after her first dose of CR and showed increases in the cortisol (942 nmol/d) and normetanephrine (666 μmol/mol creatinine) levels but a normal metanephrine level (78 μmol/mol creatinine). Three subsequent 24-hour urine collections for fractionated metanephrines, fractionated catecholamines, and dopamine levels, including one collected 2 weeks after stopping nortriptyline, were normal, ruling out nortriptyline as a potential factor in her increased BP or PRES. A second 24-hour urine free cortisol 113 days after her first dose of CR was normal (285 nmol/d). Thus, no secondary causes of HTN or PRES were found.\nFollow-up MRI one month later demonstrated resolution of the signal change in the posterior cerebral hemispheres, consistent with resolution of PRES (). She was discharged on amlodipine 10 mg daily and labetalol 150 mg twice a day. She continued to be neurologically stable at her follow-up visit in the MS clinic one month after discharge with a blood pressure of 140/87 mmHg. |
A 73-year-old Chinese man was found a cervical mass for 1 month. He had a history of coronary heart disease for 2 years, but no relevant personal or family history of malignancy. Physical examination showed bilateral neck asymmetry, the trachea was deflected to the left, the carotid pulse was normal, approximately 6 × 5 cm solid masses were felt in the right lobe of the thyroid gland. The tumor mass border was unclear with smooth surface, and the tumor moved up and down with swallowing. The left lobe of the thyroid gland had no palpable mass and the neck had no palpable swollen lymph nodes. Accessory examination of neck color Doppler ultrasound at our hospital showed increase in size of the right lobe of the thyroid gland, which had abnormal shape. The upper right pole of the thyroid gland had an approximately 5.6 × 6.0 × 4.2 cm sized mass, with unclear margin, and multiple cystic nodules in the thyroid. The CT of thyroid showed increase in size of the right lobe of the thyroid gland, and an approximately 5.6 × 6.0 × 4.2 cm sized mass, the trachea was compressed and deflected to the left, the thyroid cartilage was compressed, deformed and reached the right subglottic region. The right thyroid lump had multiple calcifications, indicative of a tumor (Fig. ). Electronic laryngoscope examination showed ventricular bands thickening, the right ventricular bands compartment showed a rice-like projection, arytenoid region movement was poor (Fig. ). Thyroid function test was 5.07 mIU/L. The patient underwent subtotal thyroidectomy and excisional biopsy of neck mass under general anesthesia. Intraoperative findings indicated a hard mass in the thyroid gland area, the lateral border and the lower bound were closely related to the surrounding soft tissues, but there was an extremely close relationship between the medial border, the thyroid cartilage, and cricoid cartilage, with no gap between them. An ENT doctor was invited for intraoperative consultation, who suggested that some of the tumors should be removed and frozen. The rapid pathology of the tumor (thyroid right lateral lobe) indicated chondroma. The ENT doctor suggested laryngeal chondroma resection and tracheotomy under general anesthesia. Intraoperative findings indicated that the right lower half of the thyroid cartilage and the right half of the cricoid cartilage were chondroma, and complete resection of the tumor was performed. Postoperative pathology examination showed (right lobe of thyroid gland and larynx) chondroma (Fig. ). Given the advanced age of the patient, long surgical duration and poor cardiorespiratory function, the patient suffered sudden cardiac death after the operation. |
A 42-year-old female presented to us with a primary complaint of pain in the left side of her face for 3 yrs. The pain was spontaneous and oppressive in nature. She had a history of burning, a pricking type of dysesthesia (pins and needles feeling), intermittent in nature and radiated to the left temporal and orbital region. The unremitting nature of pain often made her feel anxious and agitated with lack of sleep. No trigger factors and aggravating or relieving factors were disclosed in the history. She narrated a history of uneventful extraction of a decayed upper third molar and a restoration of carious tooth citing as a possible source of pain by her dentist.\nHer medical history was unremarkable except the ingestion of a cocktail of medicines alternating from analgesics, antibiotics, steroids, and antidepressants prescribed by multiple physicians for the unremitting chronic pain she was experiencing. An array of investigations was performed ranging from MRI brain, OPG, and cephalograms that turned out to be inconclusive. Vascular decompression, central pontine dysfunction, skull base, and metastatic tumor were ruled out following the normal slices seen in MRI and CT. Routine chair side diagnostic tests were done to rule out odontogenic pain.\nOn clinical examination, a sharp localized pain in the hamular region was evident on palpation due to the elongated hamular process that had a knife-edge bony projection (). The overlying palatal mucosa had no change in color or texture. A local anesthetic (1 ml of 2% lidocaine) infiltration was injected with subsequent impermanent relief of symptoms in a localized area. Her oral examination was nonremarkable on the affected left side with deep dentinal caries with respect to 18 () and pulp stones with respect to 16 on the right side (). Blood investigations carry less significance except in the possible diagnosis of cranial arteritis and for autoimmune disorders such as Sjogren's syndrome.\nFollowing a failure of conservative remedies in the past, a prominent elongated hamular process (18.53 mm) noticed on a cone beam computed tomography: axial section (), 3-D reconstructed view (), and a positive diagnostic block [], we opted for a surgical shaving in pursuit of pain relief ().\nA longitudinal incision of the mucosa was planned along with dissection up to the pterygoid hamulus followed by resection of the hamulus from its base. The gross specimen measured 13 mm in length and its shape resembled an arrowhead (). |
A 55-year-old healthy woman was referred to our institution with a two-year history of progressive dysphagia to solids (). She reported a recent episode of solid food getting stuck in her throat, which prompted presentation to an outside endoscopist. The patient reported no alcohol use. She was a former smoker with a 15 pack-year history, but had quit over 20 years prior. The patient had a past medical history of gastroesophageal reflux disease, for which she was taking omeprazole, and hypothyroidism. She had no known history of any esophageal dysmotility disorder. There was a history of diabetes mellitus in her mother and son.\nPhysical exam and laboratory testing were unremarkable. Esophagography demonstrated a filling defect in the upper thoracic esophagus. Computed tomography (CT) demonstrated an 8 cm mass. Endoscopic ultrasound (EUS) demonstrated a pedunculated mass with a submucosal origin beginning at 20 cm from the incisors on the right side of the neck (). The lesion was felt to have the characteristic appearance of a FVP and the patient elected to proceed with resection.\nThe exploration began via a right cervical approach. The recurrent laryngeal nerve was identified and the cervical esophagus was mobilized. The mass was palpable on the posterior esophageal wall at the thoracic inlet. Upon a short myotomy, no stalk was identified and the mass could not be delivered to the neck. The cervical incision was closed and a right thoracotomy was performed. The mass was seen extending from the level of the azygos vein to the thoracic inlet. The esophageal muscular layer was intact. Following myotomy, the soft mass, which was densely adhered to the mucosa, was visualized and dissected from the underlying mucosa. It became evident that the mass maintained its attachment to a portion of the mucosa. Complete mobilization revealed the mass to be a lipoma at the tip of a large midesophageal diverticulum traveling in a submucosal plane. Repeat endoscopy demonstrated an ostium in the esophageal wall opening into a blind-ending pouch. The diverticulum was fully mobilized and resected using a stapler (). Mucosal closure was reinforced with overlying muscle and a pleural flap.\nThe patient was diagnosed with a large midesophageal diverticulum with a lead point lipoma. The patient’s postoperative course was uncomplicated. A postoperative esophagogram demonstrated no esophageal leak or obstruction. Pathology demonstrated a 7.5 cm diverticulum with a 4.5 cm lipoma without malignancy. At follow-up on the nineteenth postoperative day, the patient was tolerating a diet without dysphagia. |
A 75-year-old woman was admitted to our department with a 4-day history of anorexia, weakness, and persistent coughing. She had a clear medical history without cardiac, pulmonary, or any other problems. During the clinical examination a large mass was palpated in the lower abdomen. A simple chest X-ray () was conducted which revealed a large, solid lesion in the median lobe of the right lung that was consistent with lung cancer. The laboratory findings were normal. A biopsy from the right bronchus was obtained by bronchoscopy and showed no malignancy as also confirmed by the bronchial cytological result. The patient was further submitted to a colonoscopy where no abnormalities were obtained. An ultrasound of the upper and lower abdomen revealed a solid, promiscuous lesion of 16 × 14 cm in dimensions located into the small pelvis, surrounding the uterus and the ovaries. The CT scan of the thorax () showed a 4 cm multilobed mass behind and under the hilum of the right lung and multiple nodular lesions in both lungs. The abdominal CT scan () confirmed the presence of the known mass and revealed no lymphadenopathy. Consequently, the patient underwent exploratory laparotomy, during which a large, encapsulated mass was found to emerge deep from the small pelvis, extend up to the umbilicus region, and infiltrate the uterus, the adnexa, and the right pelvic wall, displacing upwards and to the left the rectum and the sigmoid colon. During the surgical manipulations both ureters were inadvertently injured and surgically repaired. Appendectomy was preformed, while a glandule located at the right pelvic wall was removed. Biopsies from the surgical specimen including the appendix and the glandule were sent. The postoperative period was uneventful. On the ninth postoperative day the patient complained about two small-sized painful tumorlike lesions located in the lower jaw (). Because of the progressive increase in their size, the lesions were removed and sent for biopsy. A few days later the results from the pathological examination of the surgical specimen were obtained and revealed an adenosarcoma of the right ovary, consisting of high-degree homologous and heterologous sarcomatous elements, expanding to the neighboring fibroid-fat tissue and too close (almost in contact) to the right ureter, part of which was incorporated into the specimen. Both the uterus and the fallopian tubes were free of malignancy. The biopsy from the glandule of the right pelvic wall showed extended infiltration from a sarcoma with morphological characters of a rhabdomyosarcoma. Finally, the appendix was free of malignancy. The patient was discharged on the 18th postoperative day. The glandules removed from the patient's mouth were found to harbor a malignant mesenchymatous neoplasm that represented a metastatic lesion from the known sarcoma. Within a month from her discharge, the patient was admitted to another hospital because of intense headache, vertigo, and vomiting. The brain CT scan showed metastases and 4 days later she passed away. |
A 77-year-old male was referred to our endoscopy unit for the evaluation of abdominal pain and a lesion that was observed in the hilum of the liver on cross-sectional imaging. On esophagogastroduodenoscopy (EGD), he was found to have a large submucosal lesion in the antrum of the stomach. The lesion was occupying the majority of the antrum and had an ulcer. Computerized tomography (CT) demonstrated a large, fat density, submucosal mass measuring 13 × 12 × 5.5 cm. A diagnosis of a large stomach lipoma was made. An EUS was performed that demonstrated a lesion at the level of the hepatic duct; a fine-needle aspiration (FNA) was performed and was proven to be a cholangiocarcinoma. The patient was not a surgical candidate and was started on palliative chemotherapy.\nFive months later, the patient presented with jaundice and was found to have biliary obstruction on imaging. An ERCP demonstrated a common hepatic duct stricture approximately 2.5 cm in length with dilatation of the proximal biliary tree (Klatskin tumor type II) []. An uncovered SEMS was inserted and his symptom resolved [].\nAfter 4 months, the patient presented with jaundice and elevated liver enzymes and was found to have stent dysfunction. The second ERCP demonstrated narrowing at the proximal end of the SEMS due to ingrowth. A 7 Fr, 12 cm long plastic biliary stent was inserted with good biliary drainage [].\nFour months after his second ERCP, he presented with repeated nausea and vomiting, and he was also noted to have jaundice and some itching. On imaging, the plastic stent had migrated distally and the biliary obstruction had recurred. An EGD demonstrated gastric outlet obstruction, with narrowing mainly at the junction of the first and second part of the duodenum that was passed with difficulty even after using a controlled radial expansion balloon. An uncovered 10 cm long metallic duodenal stent was inserted smoothly passing the stenotic area. An EUS-AG drainage procedure was planned for two reasons; (1) reaching the papilla was difficult using a duodenoscope due to the stenosis and (2) a percutaneous drainage procedure was not possible due to a large soft tissue mass in the right hypochondrial area that was thought to be a metastatic deposit.\nInitially, the biliary tree was punctured from the left lobe of the liver using a 19-guage FNA needle, and after confirming the correct position of the needle by aspirating bile and injecting contrast a cholangiogram was obtained []. A guidwire was advanced through the needle and through the prior SEMS into the duodenum. A straight catheter was then advanced and then the track was dilated using a 4-mm biliary balloon. The proximal end of the SEMS was also dilated using the same balloon. A 12 cm long uncovered SEMS was placed antegradely and co-axially into the initial SEMS and overlapping both the proximal and distal ends [Figures and ].\nThe patient improved in the form of nausea and vomiting, and the jaundice and his itching resolved. He resumed his palliative chemotherapy. |
A 35-year-old male presented to a regional urban hospital two days following an assault-related blunt traumatic injury. The evening before arrival at the emergency department, he noticed swelling around his chest and neck. It was worse the next morning, precipitating his presentation to hospital. On initial assessment, the patient had a Glasgow Coma Scale of 15, and vital signs were BP 125/66, HR 92, and SpO2 95% on oxygen at 5 litres per minute via nasal cannulas. At the time of presentation, the patient displayed moderate subcutaneous emphysema on physical examination and subcutaneous emphysema on chest X-ray ().\nComputed tomography of the chest, abdomen, and pelvis revealed a left-sided pneumothorax and subcutaneous emphysema (Figures and ). Significant laryngeal swelling was also noted (). The patient was found to have multiple rib fractures, a lacerated scalp, and a Grade 1 liver laceration. A chest tube was not inserted at this time, after consultation with a thoracic surgeon at the nearby Level 1 trauma hospital. Upon reviewing the CT, it was suggested that the relatively small amount of pneumothorax for the degree of subcutaneous emphysema indicated potential pleural adhesions. The view of the thoracic surgery service and trauma was that an incorrectly placed chest tube at the regional centre may have risked entering the lung parenchyma. The patient was transferred to a Level 1 trauma centre 4 and 1/2 hours after presentation arriving 30 min later.\nThe extent of the subcutaneous emphysema was such that the patient could not be placed in a cervical spine collar for transport to the referral facility. His cervical spine was instead immobilized with towel rolls. Vital signs remained stable in transit, and the patient arrived at the trauma centre awake, alert, and breathing spontaneously on supplemental oxygen. The patient was assessed by the trauma service and thoracic surgery.\nOver the next two hours, the patient's condition deteriorated. While the patient had been ordered to get admitted to the trauma nursing unit, the emergency room physician wisely held the patient in the high observation area of the emergency department. Seven hours after initial presentation to the regional hospital and two hours after arrival at the trauma centre, the patient demonstrated altered phonation in addition to yet greater swelling around the neck. In order to obtain a definitive airway in a controlled environment, the patient was taken to the operating room for intubation with surgical standby.\nIn the operating room, the patient's oxygen requirements increased, with desaturation on 10 litres per minute, now via facemask. The patient was also becoming increasingly agitated. An attempt was made at awake fiber-optic intubation, but the posterior oropharyngeal anatomy, glottis, and larynx could not be visualized. Given the increasing oxygen demands and the challenging airway, after considering all options, an awake tracheostomy was performed with a Shiley XLT extended-length tracheostomy appliance. A left thoracostomy tube was then placed. Bronchoscopy in the OR did not reveal proximal tracheobronchial injury.\nThe patient was transferred to the intensive care unit where he remained for 21 days. He had complications of ventilator-associated pneumonia and delirium due to substance withdrawal. A repeat bronchoscopy on day 18 was normal, and he was successfully weaned from the ventilator that day.\nSubcutaneous decompression was achieved with continued suction via the thoracostomy tube inserted in the operating room at the time of the tracheostomy. Considerable subcutaneous air was also seen escaping from the tracheostomy incision. The subcutaneous emphysema had resolved by day 14. He was transferred to the trauma ward on day 21 and decannulated on day 22. A normal CXR was performed on day 23 (), and he was discharged on day 28. |
A 21-year-old male presented to the head and neck oncology clinic with a history of recurrent lesion in left occipital region. He had a swelling in the left occipital region 1 year back. Although the swelling had been largely asymptomatic, patient sought medical opinion at a local hospital, due to its rapidly growing size and the presence of ulceration. The lesion was mobile with small area of ulceration in center. According to the CT scan findings, there was a soft tissue lesion 4.2 × 2.5 cm2 in subcutaneous tissue in posterior occipital region with no bony erosion or intracranial extension and a diagnosis of sebaceous cyst was made. Excision of the swelling was done but specimen was not sent for histopathological examination (HPE). Again, patient developed recurrence at the local site after 4 months. This was followed by excision of the swelling and this time the specimen was sent for HPE. The report came as sebaceous carcinoma and the patient was kept under follow up. After 3 months, patient again developed local recurrence and this time he sought medical advice at our oncology clinic. The swelling was 2 × 2 cm2 in size, subcutaneous, and mobile present at previous scar site in left occipital region. CT scan was suggestive of 2.1 × 2 × 1 cm2 lesion with subtle outer cortex erosion. Wide excision of the lesion was planned. Chest X-ray done for pre-anaesthetic check up was suggestive of nodular opacity in right middle lobe of lung. Following which, CT thorax was done. It was suggestive of multiple B/L lung opacities [Figures and ]. FNAC of the swelling at left occipital region and the bsiopsy of lung nodule [] both were sebaceous carcinoma. The patient underwent local excision with local rotation flap followed by systemic chemotherapy for lung metastasis. The patient received six cycles of 3 weekly paclitaxel (175 mg/m2) and carboplatin (Area under curve-5). Patient tolerated the chemotherapy well without any grade III or IV toxicities. Post three cycles of chemotherapy, the lung metastases had completely resolved on CT scan and patient was symptomatically better. CT scan done 6 months after completion of chemotherapy [Figures and ] revealed that patient was still in complete remission. The primary site as well was controlled and the patient was doing well. |
A 52-year-old right-handed woman with no prior history of neurological symptoms was referred to our hospital with chief complaints of aphasia and headaches and no sign of both extremities weakness. These symptoms had developed suddenly 1 week before she was admitted to the hospital. CT images revealed intracerebral haemorrhage (maximum 3.5 cm in diameter) in the left frontal lobe (Figures and ).\nCerebral angiography revealed a contralateral unruptured aneurysm in the M1 region (not shown). On the basis of the preoperative MRI findings (Figures and ), the patient was diagnosed with intracerebral haemorrhage (ICH) caused by cerebral cavernous malformation. We recommended surgical intervention since the lesion was symptomatic and we thought that there would be a future risk of rebleeding. A preoperative 3D fusion image was then constructed to simulate surgical procedures (Figures –). From the results of the preoperative simulation, we determined that a straight skin incision and small craniotomy behind the hairline would be enough to remove the hematoma (). Using the simulated 3D image, Broca's area (the inferior frontal gyrus) was identified to be caudal to the hematoma cavity (). This information led the surgical team to avoid contact with the caudal wall of the hematoma cavity so that Broca's area was not damaged.\nThe patient underwent craniotomy under general anaesthesia 1 week after the onset of haemorrhage. A straight skin incision was made behind the hairline, and a bone flap was removed to reveal the preoperatively targeted sulcus (). A cross-shaped opening was made in the dura () followed by a large opening in the arachnoid membrane over and inside the targeted sulcus (). Inside the sulcus, a gyrus flattened by the hematoma was observed as indicated in the preoperative simulation. A minimal corticotomy (4 mm) was then made on the gyrus to reach the hematoma (). Most of the hematoma was liquid and was easily removed by suction; however, detachment of the solid part from the surrounding brain was difficult. Haemostasis was accomplished after removing the solid portion of the lesion () that was pathologically identified as the cerebral cavernous malformation.\nA CT scan obtained after the surgery confirmed complete removal of the hematoma (Figures and ). The preoperative symptom of motor aphasia improved immediately after the operation. The patient was discharged from hospital after 1 month following speech rehabilitation. At the time of discharge, motor aphasia of the patient was almost alleviated. |
An 18-year-old female presented for orthopedic evaluation on referral from her pediatrician due to increasing pain in her right leg. The patient was a high school volleyball player who most recently participated in a tournament 3 weeks prior. Following this tournament, she was unable to participate in further competition due to increasing pain where ice and ibuprofen had not helped during this time period. On further questioning, she described having pain in both her right and left legs for more than a year but currently her right was more problematic.\nPhysical examination revealed that she was tender along the middle anterior border of her right lower leg. She remained motor, sensory, and vascular intact with a mild limp. Radiographs () of the right leg demonstrated changes consistent with chronic stress fractures along the anterior mid-diaphyseal tibia. Thickening of the cortex was noted with narrowing of the medullary canal and at least six linear cortical defects of varying sizes were seen.\nAn in-depth discussion of management options was discussed with the patient. On further investigation, it was discovered that the patient was on a scholarship track to a major Division I university. Since the patient had previously failed conservative management and the current severity of chronic stress fractures limiting her activities, prophylactic tibial nailing was determined to be the most viable option for the patient.\nProcedure performed was an antegrade remade statically locked intramedullary tibial nail. The patient was taken to the operative theater, placed supine, and draped in sterile fashion. A 4 cm incision was made over the proximal tibia with the tendon split in line with its’ fibers. An anterior guide wire was placed and confirmed with fluoroscopy in the lateral and anteroposterior views. A decompression hole was placed on the anteromedial aspect of the distal tibia. Following sequential reaming, a 10 mm by 375 mm tibial nail was placed. One distal locking screw was placed statically at this time with backslap technique performed. Following this, two proximal locking screws were placed. Radiographs confirmed placement of the nail and locking screws. She was placed into a CAM boot, made weight-bearing as tolerated with crutches. Post-operative radiographs were obtained demonstrating good placement of the intramedullary tibial nail.\nAt the 2 weeks post-operative visit, she had weaned herself to the use of one crutch. Her pain was well controlled and she had no acute complaints. Sutures were removed at this time. Her gross motor and sensory examination remained intact. She was to continue with physical therapy working on range of motion, strength, and gait training. Six weeks postoperatively, she had progressed to full weight-bearing without assistive devices. On gait examination, she maintained a mild antalgic limp to her operative leg, but her motor examination and sensory examination remained intact. Repeat radiographs () were obtained at this time demonstrating intact hardware. Prior cortical lines remained visible, more pronounced in the lateral radiograph. The patient was then lost to follow up secondary to moving out of the state.\nAt 2 years after her surgery, the patient presented for follow-up. She reported having returned to full unrestricted high-level athletic activities without pain. She was able to perform at a collegiate level in volleyball. On physical examination, there was no pain throughout the lower leg and full muscle strength. Radiographs were obtained at this time () demonstrating intact tibial intramedullary nail with resolution of all but one residual cortical defect as seen on the lateral radiograph. |
A 20-year-old Danish woman was referred with constant pain in the right proximal thigh that had progressed for 8 years. She had no other known medical conditions. Menarche was at age 13. There was no family history of sclerosing bone diseases. The limb pain was aggravated at night and was moderately relieved during physical activity and by cold baths. She was on daily analgesic medication with nonsteroidal anti-inflammatory drugs, paracetamol, and weak opioid agonists, and she was on sick leave from her university studies at the time of referral due to her symptoms. She was using shoe inserts because of leg length discrepancy.\nAt age 11 she had consulted a rheumatologist because of hip joint pain. Guided by ultrasound a hip joint puncture was performed due to fluid gathering; serology was negative. After the puncture, the joint pain ceased.\nSix months prior to referral to our clinic, she was involved in a traffic accident and hit by a car from the right at knee level. She was discharged from an emergency room (ER) with no suspected fracture and a radiological examination was not performed. She reported that there was significant worsening of the femoral pain after the traffic accident.\nA clinical examination revealed no skin lesions, café au lait spots, or redness; there was no increased temperature or swelling in her right thigh. Leg length was unequal but within normality.\nShe was instructed to fill out a diary with daily registration of pain on an analog scale from zero to 10, where increasing values were equivalent to more intensive pain. Orally administered analgesics were required when the pain exceeded a score of 6 or more, and the score 10 was reserved for symptoms that kept our patient from falling asleep and that did not respond adequately to orally administered analgesics.\nMagnetic resonance (MR) and positron emission tomography-computed tomography (PET-CT) scans revealed significant increased cortical thickness in the right femoral diaphysis that partially obliterated the medullary cavity (Fig. ). The surface of the sclerotic bone was uneven. A Tc99-bone scan showed a pathological increase in uptake in most of the right femoral shaft (Fig. ).\nBiochemical bone markers were normal, including normal alkaline phosphatase.\nScreening for the GNAS1 activating mutation R201 was negative in peripheral blood; as was mutation screening of the SQSTM1 gene, which has been reported to be mutated in Paget’s disease of bone.\nA bone biopsy was performed and showed thickened bone springs with preserved lamellar structure and slightly accentuated cement lines. There was no fibrosis, inflammation, or increased alcian positivity, which would be suggestive of active osteomyelitis. There were no signs of malignancy or neoplasia.\nA gynecological examination prior to referral had found a mild degree of polycystic ovaries (PCO) with normal androgen status. There were no endocrinopathies.\nDuring the clinical investigation, four tentative diagnoses were raised: atypical fibrous dysplasia, Paget’s disease of bone, chronic non-infectious osteomyelitis, and melorheostosis. The young age of our patient, normal alkaline phosphatase, and sparing of the metaphysis of the affected bone made Paget’s disease of bone highly unlikely.\nThe cortical thickening is atypical for the diagnosis of fibrous dysplasia. The absence of café au lait spots, the normal age at menarche, the absence of other endocrine disorders, and the negative GNAS1 mutation examination weighed against a diagnosis of fibrous dysplasia. The long bones are a common location for chronic non-infectious osteomyelitis and this can present in childhood; however, the radiological appearance and bone histology did not provide support for this differential diagnosis. With special emphasis on the uneven surface of the affected bone on X-ray (Fig. ), the diagnosis of melorheostosis, which was fully compatible with the relatively unremarkable histology, was found to be the most plausible diagnosis. In addition, the normal bone chemistry is in keeping with melorheostosis [] although in polyostotic cases alkaline phosphatase can be increased [].\nThe timeline for intervention and clinical outcome is presented in Fig. . Initially, zoledronic acid 5 mg was administered intravenously three times with 10-month and 12-month intervals; our patient had pain relief after the first administration, but a poor effect of the second and third injection. Using an analog pain scale she reported average pain of 9 prior to treatment, 6 after treatment with zoledronic acid, and 4 from 2 to 10 months after initiation of treatment with denosumab. Looking at the usage of pain medication, it went from daily dosage before treatment to 23% of days after treatment with zoledronic acid, and to no orally administered analgesics for 8 months following treatment with denosumab.\nIn October 2015, denosumab was chosen as the second line of treatment based on the hypothesis that the pain was caused by increased bone turnover. A dose of 60 mg was injected subcutaneously with some remission of pain that lasted for 6 weeks. At the same time as the pain relapsed, there was an increase in alkaline phosphatase, PINP, CTx, and osteocalcin in blood tests. Biochemistry before and during treatment is listed in Table . Based on these findings it was decided to shorten the interval between administrations to further strengthen control of a hypermetabolic state. The second dose of 60 mg was given after 8 weeks and this interval has been kept up to the present.\nBiochemistry after treatment with both zoledronic acid and denosumab showed suppression of bone-type basic phosphatase, osteocalcin, collagen I, and procollagen I, with a relative increase in parathyroid hormone, and plasma ionized calcium within the normal range. The injections with denosumab were well tolerated with no adverse effects reported. During the whole treatment period, the injections were administered from our out-patient clinic without cancellations.\nAfter the first three doses given 8 weeks apart, there was pain remission for 8 months; in this period our patient did not take any orally administered analgesics. At the latest follow-up in February 2018, she complained of a slight increase in pain and occasional use of paracetamol and nonsteroidal anti-inflammatory drugs.\nThe treatment with denosumab was well tolerated with no side effects observed.\nA Tc99-bone scan and computed tomography (CT) scan were performed in February 2017 and in February 2018, 5 and 17 months after the first injection with denosumab; the pathologically increased activity and cortical thickening were stationary. |
The patient was a 5-year-old boy presenting to the emergency department after sustaining a fall from 3 m height with a direct blow to the posterior aspect of the left elbow.\nAt presentation, he had severe left elbow and forearm pain with an S-shaped deformity of the left upper limb, along with ecchymosis of the medial side of the elbow and the anterior aspect of the forearm ().\nThe patient was complaining of severe pain with numbness in the territories of the ulnar nerve at the level of the wrist and hand without any signs of the median nerve or radial nerve neuropraxia or injury. There was no vascular compromise and the limb was warm well perfused.\nX-rays were done in the emergency department and showed a left supracondylar fracture, along with ipsilateral radial shaft fracture and ulnar shaft fracture (). The supracondylar fracture was flexion type, displaced with complete loss of anterior and posterior periosteal hinge, type III, according to Gartland classification. Both bone forearm fracture was A3 according to the AO classification, the radial shaft fracture was simple transverse, non-displaced, and located in the diaphyseal mid-third, while the ulnar shaft fracture was simple with slight shortening, and located at the junction of the diaphyseal mid-third and distal third. This fracture could not be classified according to Cuellar-Nieto classification because it does not involve any metaphyseal region of the radius and/or ulna.\nThe injured limb was immobilized in the emergency room without reduction. The pre-operative plan was to start with reduction and fixation of the supracondylar fracture to limit as possible any additive damage to the ulnar nerve from manipulation of the forearm when addressing the radius and ulna, and then reduce and fix the forearm fracture. Having a warm perfused limb, with ulnar nerve irritation in a setting of floating elbow, the surgical intervention was judged to be urgent but not emergent. That is why the patient was then taken to the operating room during the same day of the injury, where under general anesthesia, supine position, a closed reduction of the supracondylar fracture was tried once under the control of the image intensifier with no success. The reduction technique involved longitudinal traction applied to the forearm to reduce the humeral length while holding the olecranon; the elbow was then extended gradually to correct the angulation. After the failure of the closed maneuver, the decision for the open reduction was taken. After scrubbing and draping, a lateral incision centered over the fracture was made, which allowed direct access to the fracture that was reduced anatomically and fixed by 2 parallel percutaneous Kirschner wires inserted from distal to proximal (lateral to medial) and a 3rd one inserted from proximal to distal (lateral to medical), through the mentioned incision, crossing the other two wires under the control of the image intensifier. Care was taken to not cross the medial cortex distally to avoid any injury to the ulnar nerve. After confirming the stability of the construct, the forearm fractures were reduced closed and fixed by antegrade elastic nailing of the ulna and retrograde elastic nailing of the radius. All wires were embedded under the skin. Finally, a well-padded circumferential long arm cast was applied. Post-operative circumferential cast. Post-operative radiographs of the elbow () and forearm () are shown below. Post-operative, the patient was doing well, there were no signs of compartment syndrome, and ulnar nerve symptoms such as numbness and slight weakness of the 5th finger flexion were present. He was discharged after 2 days of monitoring without any signs of compartment syndrome.\nAt 3-week follow-up, the patient was improving in terms of ulnar nerve symptoms; follow-up radiographs showed increasing callus formation ().\nAt 2 months post-operative, the patient was readmitted for removal of cast and wires. Post-operative testing under general anesthesia showed a good range of motion of the elbow. Post-operative radiographs are shown in ().\nThe patient had complete recovery of the ulnar nerve symptoms after 4 months of the initial injury. The patient and family were completely satisfied by the results where there was no complication or residual signs or symptoms. |
A 23 years old Malay lady who was born with a solitary lesion over her right foot dorsum. Initially the size of the lesion was about the size of a pea and gradually increase in size and causing pain. At the age of 1 month old, parents claimed it was excised. Over the years, other cutaneous skin lesion erupted and size gradually increase. The biggest lesion was over the right arm and it was operated at the age of 2 in tertiary hospital by hand surgeon.\nPatient had her episodes of recurrent intestinal bleeding which resolves spontaneously since the age of 19 and requires frequent blood transfusions. Later, the patient was referred to surgeon for her chronic anemia with multiple cutaneous bluish lesion. Investigations that was done including OGDS, colonoscopy, capsule endoscopy and CT abdomen. Due to this indwelling problem, patient underwent laser treatment of the gastrointestinal lesion and she was symptom free for about one year. Sclerotherapy for lesions on each foot sole and right hand dorsum had to be done due to pain especially upon ambulation.\nUnfortunately a year later, patient had a massive gastrointestinal bleeding which require patient to underwent major surgery. Intraoperative findings were multiple naevi over small and large bowel with contact bleeding which were excised, large naevus at the mesentery at the proximal jejunum which was left in-situ, naevus over the appendix and appendicectomy was done and multiple naevi over the liver surface which was left in-situ. A total of 53 enterotomies and 9 colotomies was done for this patient. On the same setting of the surgery, over 112 cutaneous lesions were excised over suprapubic region and bilateral thighs. Post operatively she was recovered from any intestinal bleeding and does not require any blood transfusion. Clinically she was well and pink, laboratory investigations shows her hemoglobin level maintain at 10 gm/dL.\nA year later, patient complaining of bilateral cheek and intraoral lesions which has cause occasional pain and gum bleeding. Case was evaluated by plastic surgeon and multiple cutaneous lesions were noted over bilateral cheek, over the gums, lingual and sublingual, bilateral palms and soles, over the abdomen and back. The lesions were bluish to grey in color, soft and compressible in nature. MRI of head and neck as well as MRA of brain and cervical was done for her. Findings of the imaging were numerous venous malformation over pterygoid muscle, masseter muscle and infratemporal fossa, nasopharyngeal mucosal space, parapharyngeal space, bilateral parotid, submandibular space, sublingual space, soft tissue of the cheek, supraclavicular fossa, thyroid gland, scalp, superior and middle mediastinum. Intraoral lesions are over soft palate, intrinsic and extrinsic tongue muscles till floor of the mouth ().\nAt the age of 23, patient underwent sclerotherapy of bilateral cheek lesions. Sodium tetradecyl and Lipiodol was injected into both lesions by the help of our Interventionist Radiology. Post sclerotherapy two month later, size of the bilateral cheek lesions has reduced however the lesions was still present (, , , ). Patient underwent another session of sclerotherapy 3 month later and the lesions has shrunk further more. Patient is happy with the outcome and still under plastic surgery team follow up. |
A 70-year-old Sri Lankan Tamil male with a history of well-controlled type 2 diabetes mellitus and a goitre of 30 years presented with a painful enlargement of goitre on the left side for one month. He had progressively worsening difficulty in breathing with intermittent dysphagia for solids. He did not have any symptoms of local infiltration and was clinically euthyroid. Examination revealed a hard mass arising from the left thyroid lobe measuring 8 cm × 7 cm in size with gross tracheal deviation to the right side. In addition, there were two mobile lumps anterior to the mass in the subcutaneous tissue plane (). There was no retrosternal or retroclavicular extension on the left side. The right thyroid lobe was moderately enlarged and had multiple palpable nodules. There was no cervical lymphadenopathy. Ultrasound scan showed a large calcified left thyroid nodule and few superficial nodules. The outer surfaces of the nodules were delineated by an echogenic line suggestive of surface calcification. The internal echotexture of the nodules was not clearly appreciated due to artefacts from the surface calcifications. Right thyroid lobe showed only benign characteristics. Neck X-ray radiography showed a calcified left lobe with significant tracheal deviation to the opposite side (Figures and ). Thyroid stimulating hormone (TSH) and free thyroxine (T4) levels were within normal limits. Ultrasound-guided fine needle aspiration cytology (FNAC) showed scattered cyst macrophages, lymphocytes, and multinucleated giant cells in an eosinophilic background with scanty colloid. The features were compatible with a benign cyst (Thy 2).\nThe patient underwent a total thyroidectomy. Two confluent nodules were noted in the subcutaneous tissue plane extending through the deep fascia between the strap muscles to the calcified left lobe nodule. The deep fascia and strap muscles were thinned out and were adhered to the calcified left lobe (). Division of strap muscles on the left side was required to mobilize and deliver the left lobe containing the calcified nodule.\nMacroscopic assessment of the specimen consisted of the thyroid gland with the right lobe measuring 45 × 25 × 20 mm, the isthmus measuring 65 × 15 × 4 mm, and the enlarged left lobe measuring 80 × 75 × 55 mm. The outer surface of the gland was smooth. There were two confluent nodules over the anterior surface of the left lobe measuring 12 × 8 × 8 mm and 10 × 8 × 6 mm with a smooth outer surface.\nHistology of the thyroid revealed an encapsulated left lobe lesion composed of a thick fibrous wall with foci of calcification. A dense inflammatory reaction comprising lymphocytes, foamy histiocytes, and scattered multinuclear giant cells was present within the capsule. The lumen was filled with amorphous, eosinophilic material with cholesterol clefts. A thin rim of compressed thyroid tissue was noted outside the fibrous wall. Sections from the confluent nodules revealed similar histopathological features and showed encapsulated lesion surrounded by a thin fibrous capsule. They were filled with numerous foreign body type giant cells and foamy histiocytes admixed with amorphous eosinophilic material and cholesterol clefts. No thyroid or lymphoid tissue was seen. The right lobe and isthmus showed features of a colloid storing goitre. There was no evidence of malignancy in the entire specimen. Overall, features of the main calcified nodule of the left lobe and the two smaller confluent nodules were compatible with a colloid cyst with secondary changes including calcification and chronic inflammation. The patient had an uneventful postoperative recovery with the alleviation of the obstructive symptoms. He was discharged on the first postoperative day on thyroxine 100 mg daily and remained healthy without any obstructive symptoms during a three-month routine outpatient clinic review. |
An 18-year-old female patient reported with a complaint of swelling over the gums in the lower front tooth region that had developed over several months, which caused discomfort while eating. Detailed history revealed that the swelling was painless and initially small in size, which gradually increased to the present size. The patient also stopped brushing in the area due to excessive bleeding from that region while brushing. No relevant drug, dental, medical, and family history was present.\nAn extraoral examination showed no significant findings. Intraoral examination revealed a large sessile lobulated gingival overgrowth, appeared irregular in shape, measuring approximately 18 mm × 12 mm in dimensions, extending on buccal surfaces of 41, 42, 31, 32, and 33. It was reddish pink in color, having a smooth overlying surface with no ulcerations. The oral hygiene of the patient was poor, with large plaque deposits on the buccal surfaces of the involved teeth []. On palpating the lesion, it was firm, nontender, and bleeds on manipulation. Based on the above clinical findings, a provisional diagnosis of gingival pyogenic granuloma was considered. Teeth associated with it showed grade I mobility. Radiographic evaluation was also done to rule out any bony involvement, which showed no significant findings, except for the mild amount of bone loss associated with both central incisors. Complete blood count was found to be within the normal limits.\nAs the lesion was large and develops in an esthetic zone, it is difficult to choose an appropriate treatment modality. Surgical excisions of the lesion can apparently have relatively high chances of recurrence, and also there were chances of losing the anterior teeth; treatment with a laser was also inappropriate, as the lesion was thick. The patient has been informed about the various treatment options and their pros and cons. However, the patient was not willing to go for surgical treatment due to esthetic concern. Therefore, with patient consent, we chose sclerotherapy as a management approach in this case. The patient first underwent oral hygiene instructions and motivated to ensure optimal plaque control. At first, complete oral prophylaxis was done to remove the causative local factors, followed by local application of ornidazole (1.0%w/w) and chlorhexidine gluconate (0.25% w/w) gel [Ointment Ornigreat Gel; Mankind Pharma Ltd.] and metronidazole gel (1% w/w) (Ointment Metrohex Gel; Dr. Reddy's Lab. Ltd.) on an alternate basis twice a day []. Improvement in gingival health was noted during the reassessment []. After 2 weeks, the sclerotherapy procedure was planned. At first, surface local anesthesia was applied over the lesion. Then, 0.2–0.5 mL of 3% sodium tetradecyl sulfate (Setrol) [] was slowly injected into the base of the lesion by insulin syringe (0.3 mm × 8 mm size, 31G) until the solution gets leaked out from the surface of the lesion []. Patients were examined at weekly intervals up to 1 month to evaluate the response until the lesions reduced in size, and accordingly, the second, third, and fourth injections were planned and given at weekly intervals []. After 4 weeks of follow-up, the lesion was significantly regressed in size and extent [].\nThe patient was recalled every 3rd month for maintenance and to check for possible recurrence. This case was followed up for a period of 1 year, and there has been no recurrence so far []. |
A 33-year-old gentleman admitted to our hospital on 5 August 2011 presented with a 4-month history of pain in the left lower quadrant of the abdomen, which had worsened in the last 2 weeks before presentation.\nFour months ago, the patient began experiencing abdominal pain in the lower left quadrant without any apparent cause. The symptoms became increasingly aggravated during the last 2 weeks before presentation. The presenting symptoms included paroxysmal colic in the left lower quadrant of the abdomen, fever, nausea and vomiting. The patient also experienced fatigue and an 8 kg weight loss over the last 4 months. In a nearby local hospital, an abdominal plain film was obtained, which showed intestinal tympanites.\nFor further treatment, the patient came to our outpatient department and was admitted to the Department of Gastroenterology. A physical examination revealed a male patient with tenderness in the left lower abdominal region. There was no hepatomegaly or splenomegaly. Laboratory tests were performed, and the results were normal, including the levels of carcinoembryonic antigen and carbohydrate antigen 19–9. Computed tomography (CT) scans of the whole abdomen showed a significantly thickened intestinal wall located at the end of the jejunum and the proximal ileum, excessive ascites and a few enlarged lymph nodes in the abdomen (Figure ). Contrast-enhanced CT scans showed different degrees of enhancement in the hepatic arterial phase (Figure ).\nAn urgent general surgical consultation was required because of sudden severe aggravation of the abdominal pain. Abdominocentesis was performed, which showed a dark red fluid. The patient presented with peritonitis. Therefore, an emergency laparoscopic surgery was performed, which was later converted to a laparotomy following partial enterectomy. The operative findings included intra-abdominal bleeding of 1500 ml and an oedematous small intestine. Additionally, the greater omentum was found adherent to the small intestinal mesentery in the left upper abdomen with inflammation and blood oozing from the small intestine. During surgical exploration, dozens of irregular nodules of various sizes were found scattered on the surface of the liver. In addition, a mass of approximately 5.0 × 6.0 cm in size was found in the small intestine approximately 70 cm distal to the ligament of Treitz and was accompanied by an endoleak. During the operation, the tumor and the local mesentery of the small intestine were resected. Eight lymph nodes were examined, and two were found to be metastatic. On microscopic examination, vascular invasion of tumor tissue could be observed. The blood vessels of the tumor were abundant with tumor cells around them, and the tumor cells were arranged in a slit-shaped pattern (Figure ). The immunohistochemistry results showed that the tumor cells were positive for CD31 and vimentin (Figure ) and negative for CD34, actin, S-100, CD117 and CK56. Moreover, the Ki-67 proliferation index was less than 10% positive. On the basis of these findings, adenocarcinoma, intestinal tuberculosis, neuroendocrine tumor, malignant melanoma, Crohn’s disease, gastrointestinal stromal tumor (GIST) and lymphoma were excluded. Hence, the diagnosis of primary angiosarcoma of the small intestine with metastasis to the liver was confirmed.\nAfter surgical resection, the patient was managed with adjuvant chemotherapy and palliative care. Approximately 400 to 500 ml hemorrhagic fluid was drained during the postoperative period through the abdominal drains. Moreover, the volume of hemorrhagic fluid increased to 600 ml on the 6th postoperative day. Therefore, a cytologic examination was performed, which showed malignant cells, confirming the diagnosis of malignant ascites. The patient received frequent blood transfusions due to a progressive drop in hemoglobin. In the early morning hours of the 27th postoperative day, the patient experienced a sudden disturbance of consciousness along with a progressive decrease in blood pressure. Due to the above-mentioned circumstances, further treatment was refused by the patient’s relatives. The patient died on the morning of the 27th postoperative day. |
A 24-year-old man was brought by ambulance to our facility after having been involved in an unhelmeted motorcycle collision. He presented with sinus tachycardia up to 123 beats per minute, a Glasgow Coma Score of 15, and had an obvious deformity of the right lower extremity. His tachycardia improved with 2 L of crystalloid fluid bolus. He was stabilized in a cervical collar and a Sager traction splint on his right lower extremity and taken for CT scans of his head, cervical spine, chest, abdomen and pelvis as well as plain films of his extremities. According to our institutional protocol, he underwent CT angiogram of his neck given his skull base fracture. He was found to have a right kidney laceration with active extravasation, two large hepatic lacerations without active extravasation, a right midshaft femur fracture, bilateral distal radius fractures, a left occipital condyle fracture, a dissection of the left internal carotid artery, and pooling of IV contrast near the bifurcation of his inferior vena cava (IVC). This was concerning for a vena cava or iliac vein injury (Fig. ). Given his hemodynamic stability, we consulted the vascular and interventional radiology teams regarding management. We decided to proceed with visceral arteriogram to control bleeding from the right kidney as well as a venogram to further delineate the suspected venous injury (Fig. ).\nThe visceral arteriogram demonstrated extravasation from the right adrenal artery and superior pole of the kidney, both of which were embolized with microcoils. Post-embolization angiography demonstrated no further evidence of active extravasation. There was no injury to the aortic bifurcation. A venogram was then performed, which demonstrated a 2.8 cm pseudoaneurysm arising from the proximal portion of the left common iliac vein with involvement of the base of the IVC without active extravasation. There was no involvement of the right common iliac vein. The patient continued to be hemodynamically stable so the decision was made to observe him. After an initial hemoglobin drop from 14.3 to 6.9 g/dL within the first 48 h, he stabilized between 8 and 9 g/dL after two units of packed red blood cell transfusion and then remained hemodynamically stable throughout his stay. He was initiated on aspirin and therapeutic heparin for his carotid dissection on hospital Day 2 and underwent a cerebral angiogram and right internal carotid stent placement on hospital Day 14. A CT venogram on hospital Day 16 demonstrated a decrease in size of the pseudoaneurysm, and a stable prevertebral and pelvic retroperitoneal hematoma (Fig. ). These were managed conservatively as the patient’s hemoglobin and hemodynamics were stable. Given the concern for intraluminal thrombus extension from the pseudoaneurysm, the decision was made to continue therapeutic anticoagulation. He was bridged to warfarin on hospital Day 17 and discharged to a rehabilitation facility on hospital Day 20 on therapeutic anticoagulation and full dose aspirin. |
The patient is a 21-year-old female with no significant past medical history. Six months prior to her evaluation for surgery, the patient presented to her primary care physician with symptoms of severe, intermittent bilateral lower quadrant abdominal pain that would last for 1–2 weeks between episodes. She underwent a computed tomography scan of the abdomen and pelvis that showed moderate wall thickening of the terminal ileum and cecum, as well as a linear structure seen posterior to the cecum with a diameter of 1.2 cm, representing a dilated appendix. The patient subsequently underwent a colonoscopy that was notable for mild terminal ileal and cecal inflammation (Fig. ), with biopsies showing active ileitis and chronic active colitis with non-necrotizing epithelioid granulomas. The patient was prescribed a 2-month course of budesonide, which she completed initially without improvement in her symptoms. After completion of her budesonide course, the patient underwent blood testing notable for persistent evidence of inflammation including an elevated ESR and CRP of 25 and 9.7, respectively, but no other lab abnormalities, including a normal WBC count and negative tuberculosis testing.\nFour months after her original computed tomography scan, an MRI enterography demonstrated a normal-appearing terminal ileum and cecum, and an inflamed blind-ended tubular structure measuring up to 1.3 cm in diameter (Fig. ) with wall thickening and mural enhancement, arising from the cecum and consistent with acute uncomplicated appendicitis. Although at that time the patient was not experiencing abdominal pain, given the imaging findings, she was prescribed a 14-day course of ciprofloxacin and flagyl. A repeat MRI enterography following completion of the antibiotics again showed an inflamed appendix with slightly decreased wall thickening but with increased distention.\nGiven the worsening radiographic appearance of the appendix that raised concerns about impending perforation, along with the lack of a tissue diagnosis, a laparoscopic appendectomy was recommended. At the time of surgery, a severely inflamed appendix was visible in the pelvis, and was found to be densely adherent to the left pelvic sidewall immediately adjacent to the left ovary and fallopian tube (online suppl. video; for all online suppl. material, see ). The left ovary and fallopian tube were carefully dissected away from the appendix, the appendix was separated from the left pelvic sidewall, and an appendectomy was performed. The entire appendix appeared inflamed, but the base of the appendix and the adjacent cecum appeared normal. The patient's postoperative course was uncomplicated.\nThe resected specimen was examined grossly in pathology (Fig. ) and appeared inflamed but soft, without evidence of masses. The appendix had a maximal diameter of 1.9 cm with no masses or lesions identified. The luminal diameter measured up to 0.8 cm with an average wall thickness of 0.5 cm. Histologically (Fig. ), the appendix demonstrated acute appendicitis, marked mucosal chronic inflammation with crypt architectural distortion, transmural lymphoid aggregates, and epithelioid granulomas, consistent with Crohn's appendicitis.\nThe patient has done well postoperatively without recurrence of abdominal pain or loose stools, on no medication. |
A 32-year-old male with a history of insulin-dependent diabetes mellitus (IDDM) since 19 years of age presented with uncontrolled blood sugars and pedal oedema. He was detected to have diabetic nephropathy for the previous 2 years when he presented with frothing of urine. His glycosylated haemoglobin A1c was 11.8%, and a continuous subcutaneous insulin infusion with a pump had been prescribed as conventional insulin treatment failed to control his blood sugars. He had end organ involvement from diabetes manifesting both as nephropathy and proliferative retinopathy. He was also hypertensive and hypothyroid for which he was on treatment with a calcium channel blocker and beta blocker and 175 μg of tablet thyroxin per day. His renal functions showed a creatinine clearance of 20–30ml/min, (Class IV chronic kidney disease [CKD])[] and had not been initiated on haemodialysis. In view of his age, difficult blood sugar control, end organ involvement with diabetes and poor quality of life, a multidisciplinary team decision was to list him for a SPK transplant.\nA coronary angiogram was performed in our patient following an inconclusive stress test that revealed mild coronary artery disease. Multi systemic examinations including respiratory, central nervous system and liver were also performed to exclude contraindications for the transplant. After a detailed pre-anaesthesia evaluation, the patient was scheduled for surgery. The challenges anticipated were blood sugar control during the surgery, deterioration of native renal function perioperatively until the function of the new graft picked up, and preparedness of the team at the time of availability of the donor. When a suitable matched donor was identified following brain death, the patient was called in and investigations were performed according to protocols. The insulin pump was disconnected and insulin infusion started according to the protocol. The recipient was taken to theatre once the donor lymphocyte cross match was negative. He was started on infusion of prostaglandin E1 at 0.025 μg/kg/h as a vasodilator to optimise vascular flow and 5000 U unfractionated heparin subcutaneously for thromboprophylaxis. His investigations at the time of surgery were haemoglobin 9.9 g/dl, urea 88.8 mg/dl, creatinine 3.79 mg/dl, albumin 2.58 g/dl, sodium 143 mEq/L and potassium 4.2 mEq/L. Immunosuppression was induced with interleukin 2 receptor blocker basiliximab and protocols for maintenance planned with prednisolone, tacrolimus and mycophenolate mofetil.\nAnaesthesia was induced as using fentanyl, midazolam and propofol titrated to the loss of verbal response. Atracurium was used to facilitate endotracheal intubation and an infusion at 0.5 mg/kg/h was used during surgery. The radial artery was cannulated under local anaesthesia and the left internal jugular vein was cannulated with a 7.5 Fr triple lumen central venous catheter after intubation. A minimally invasive cardiac output monitor (Edwards EV-1000) was used to guide fluids and the use of inotropes.\nThe surgical procedure involved implantation of the donor pancreaticoduodenal graft in the right iliac fossa through a long vertical midline incision. Vascular inflow anastomosis was made between the graft splenic and superior mesenteric artery through a Y-shaped graft to the right common iliac artery. The outflow was through the graft portal vein to the infrarenal recipient vena cava. Graft duodenum was anastomosed to a Roux limb of jejunum after reperfusion to drain the exocrine secretion from the graft []. Unfractionated heparin at 5000 U intravenously was administered at the time of vascular anastomosis. The arterial blood sugars were monitored hourly and confirmed intermittently with laboratory values. Insulin infusions were given as per protocols and the target was to maintain blood sugars between 100 and 150 mg/dl.\nReperfusion of the pancreas is accompanied by a rapid fall in blood sugars needing reduction or cessation of insulin infusions. Our patient remained stable perioperatively, sugar levels normalised 2 h after reperfusion while insulin infusions were stopped immediately after reperfusion []. The renal implant was started after the pancreas, the graft being placed extraperitoneally in the left iliac fossa with vascular anastomosis to the external iliac artery and vein. Injections of 20% mannitol (100 ml), furosemide 80 mg and methylprednisolone 500 mg were administered at the time of renal graft implant. Renal reperfusion was completed 2 h following the pancreatic transplant.\nNoradrenaline at 0.08 μg/kg/min and vasopressin at 1.8 U/h were used as vasopressors guided by the measurements of systemic vascular resistance. During the surgery, the patient received 2.5 L crystalloids and 2 units of packed cells. Urine output from the native kidney was maintained during surgery (450 ml) and good perfusion of the graft kidney was observed. The patient was electively ventilated until correction of acidosis. He made an unremarkable recovery with normalisation of blood sugars and improvement of renal functions and was shifted from the Intensive Care Unit by the 6th post-operative day. He was discharged on the 15th post-operative day and has well controlled blood sugars and normal renal functions at 6 months follow-up. |
An 11-month-old male child was brought to the plastic surgery OPD of our institute by his parents with the chief complaints of increase in size of a swelling over upper lip with difficulty in oral intake for the last 1 week. On eliciting the history, it was revealed that the lesion was present since birth and had been gradually enlarging. The size of the lesion was measured to be 5.5×3.5×1.5 cm and was diagnosed as a case of hemangioma upper lip [Figures and ]. Previously, the patient did not have much difficulty in feeding but the difficulty rose since 1 week as the lesion had suddenly enlarged. He was being fed by the spoon from the angle of the mouth. Considering the age of the child, size of the lesion and most importantly the site of the hemangioma, it was planned to ablate and strangulate the vessels during the first stage so as to decrease the size of lesion before proceeding for surgical excision. Preanesthetic assessment revealed a very narrow mouth opening with difficulty in assessing the mallampatti grading [Figures and ]. All the investigations were within normal limits including the coagulogarm and weight of the child was found to be 8 kg.\nGeneral anesthesia was planned for the child with main stress upon airway management. The child was kept in a fasting state for 6 hours and was administered 2 mg of syrup midazolam 30 minutes before the surgical procedure. Airway trolley was made ready with all the pediatric airway equipment including the fibreoptic bronchoscope of 2.4 mm diameter. The child was taken to the operation theatre by one female staff who had been with him for the last half-an-hour and had developed a good rapport with the child. Pulse oximetry probe was attached to the great toe of right foot and a pediatric stethoscope was fixed on the precordium. Induction of anesthesia was achieved with sevoflurane in 6 L of oxygen administered through number 3 face mask attached to Jackson Rees circuit while covering the eyes with soft cotton pad to prevent injury to the eyes. A good intravenous access was secured with 22 G venflow and after establishing a good breathing pattern evident from the bag movements we administered 15 mg of ketamine. When we were sure that we can easily ventilate the patient, we administered 20 mg of succinylcholine while sevoflurane was kept at 2.5% during this stage. Gentle positive pressure ventilation was carried out for 1 minute. Laryngoscopy was done with miller blade size 2 from the left corner of the mouth and tongue was lifted in a diagonal manner and we were able to see the full view of glottis. During the whole exercise of laryngoscopy the fulcrum on the upper teeth was never made rather a forward and upward pressure was maintained without even touching the mass. An oral RAE endotracheal tube number 4.5 mm (internal diameter) was inserted from the right corner of the mouths while an assistant helped continuously in raising the mass with a soft cotton pad with gentle force so as to enable laryngoscopy and a good intraoral view. After checking the equal air entry bilaterally, we packed the oral cavity with a roll gauge to prevent any aspiration of secretions or possibly blood as the tube was without a protective cuff. The child was then paralyzed with 3 mg of injection atracurium and positive pressure ventilation was carried out throughout the procedure with Jackson Rees circuit with nitrous oxide and oxygen in the ratio of 60:40. Right from the beginning a team of plastic surgeon, a general surgeon and an otorhinolaryngeologist were scrubbed and ready with their equipment. Surgical procedure was initiated and peripheral surgical ablation of the feeding vessels was done with electric cauterization. Thereafter the intralesional injection of steroid was administered. The surgical procedure lasted for 15 minutes and at the end of surgery the child was reversed with 0.5 mg of injection neostigmine and 0.1 mg of glycopyrrolate. The dry intraoral roll gauge pack was removed and the child was extubated in a fully awake state after establishment of adequate and rhythmic respiratory efforts with good tidal volume and flexion of legs at the hip joint. He was given rectal suppository of 150 mg paracetamol for postoperative pain relief after induction of anesthesia. The child was kept in the recovery ward for 2 hours and later on shifted to the pediatric wing of the plastic surgery ward where he was kept for another 1 day and was discharged after an uneventful recovery period during the hospital stay. The parents were instructed for follow-up of the child after 15 days. |
A 14-year-old male patient visited our hospital with headache, nausea, vomiting, dysarthria and right hemiparesis grade I as the main symptoms. Dark brown colored large melanocytic nevus that covered about 1/2 of the trunk and various sized nevus ranging from several millimetre to several centimetre were observed throughout the whole body of the patient ().\nWhen the patient was 6 months old, in the dermatology department of our hospital, histologic examination of nevus sections showed nevus cell infiltration with pigmentation in the reticular dermis. No evidence of malignant transformation was found (). The patient suffered an onset of seizure when he was two years old and received treatment with anticonvulsant medication at the pediatric department of our hospital. Under NCM impression at the time, serial follow-ups for clinical symptoms were done for 12 years by the pediatrics department. During that time, there was no manifestation of abnormal neurological symptoms. The patient visited due to motor weakness. The computed tomography (CT) imaging scans showed intracerebral and intraventricular hemorrhage throughout left posterior frontal cortical area and left lateral ventricle. Under magnetic resonance image (MRI) gadolinum-enhanced T1 weighted image, partially high signal was detected in the cortical area of frontal lobe. Small inhomogenous high signal was also observed on the pons, temporal horn, and anterior portion of lateral ventricle through gadolinum-enhanced T1 weighted image. MRI scans also revealed a cyst in the posterior fossa and hypoplasia of the cerebellar vermis with dilatation of the entire ventricular system. Longitudinal diffuse enhancement lesion was observed along the posterior dura at the thoracic level in the whole spine MRI scans carried out for the purpose of screening but nodular lesion or focal mass lesion was not observed in other areas (). Although surgical treatment under NCM impression accompanies by DWC was recommended, we could not operate on him due to refusal by the guardian. After the conservative care during one month, the patient was discharged with improvement of neurological symptoms. However, the patient was hospitalized again with headache and right hemiparesis grade I after 4 months. Large mass, which is a marked enlarged state of left frontoparietal area, was observed under the MRI scans. The surgical treatment was performed. Tumor along with diffuse dark colored leptomeninges and with soft, friable, vascularized and poorly defined borders of margin was removed gross totally (). Histopathologic examination showed melanin-pigment laden neoplastic cells, necrotic change and pleomorphic malignant cell, which could be diagnosed the malignant melanoma (). No pathologic deposits of the skin area were seen in the positron emission tomography with 18-FDG (). It was possible to definitively diagnose NCM with DWC. After 2 month following first brain surgery, the patient was discharged with symptom improvement. Three months after discharge, the patient was hospitalized again with observation of increased intracranial pressure symptoms such as decreased mentality, headache and nausea. Re-grown large mass on the left frontoparietal area and new mass on left medial temporal area were observed under MRI scans (). The mass on the left frontoparietal area was removed gross totally once again through surgery with same pathological findings. On the 3rd week following the second surgical treatment, the patient complained of back pain, voiding difficulty, left side motor weakness grade III and respiratory difficulty. So spinal MRI scans was done, and mass lesion with high signal at the T2 weighted image and inhomogenous high signal at gadolinum-enhanced T1 weighted image along the C5-T3 area were observed, and bilateral laminectomy with partial removal of the mass was performed. The intradural mass showed a soft, friable, highly vascularized characteristics and infiltrated into the leptomenignes with extremely poorly defined border (). Although performing aggressive treatment, decreased of consciousness, respiratory difficulty and motor weakness continued in the patient, and progressive hydrocephalus was observed on CT scan. The guardian did not wish further treatment. The patient died on the 3rd week following the spine surgery. |
A 66-year-old woman presented at our hospital for the treatment of a dislocation 15 years after hemi-arthroplasty. She had undergone hemi-arthroplasty at another hospital using a porous coated anatomical stem (Stryker, MI) for a femoral neck fracture. The patient had poor posture due to compression fracture but was still able to walk until 4 months before presentation at our hospital. The patient had difficulty walking without experiencing trauma and was diagnosed with an anterior dislocation due to reduction of lumbar lordosis and severe posterior pelvic tilt. Because the dislocation could not be reduced at the other hospital, the patient was admitted to our hospital for treatment.\nPhysical findings at the time of her first visit to our hospital included height of 148 cm, weight of 38 kg, and body mass index of 17.4 kg/m2. There was no tenderness at the femoral triangle, her left leg was shortened in external rotation, she had limited range of motion (ROM; 70 ° of flexion, − 10 ° of extension and 10 ° of abduction) in her left hip joint, and she was unable to keep standing. An X-ray image of her hip in the supine position showed anterior dislocation of the hemi-arthroplasty with severe posterior pelvic tilt (Fig. ). An X-ray image of her lumbar spine showed a wedge-shaped compression fracture of the fifth lumbar vertebra due to osteoporosis and reduction of lumbar lordosis. The small bone fragment in the anterior part of the acetabulum indicated the occurrence of a dislocation fracture due to stress concentration on the anterior wall of the acetabulum.\nThe anterior pelvic plane (APP), defined as a plane through the bilateral anterior superior iliac spines to the pubic symphysis, was regarded as the anatomic plane of the pelvis in this patient. The angle between the APP and the coronal plane passing through the bilateral anterior superior iliac spines (ASIS) was defined as the sagittal pelvic tilt angle (APP tilting angle) in the supine or standing position. CT data and the Zed Hip system (Lexi, Japan) showed that the APP tilting angle in this patient at admission was 38 degrees posterior in the supine position.\nWe planned to transform hemi-arthroplasty into THA because anterior coverage with reduced cup anteversion was required for the prevention of anterior dislocation in this case. Due to the severe posterior tilting of the pelvis, cup positioning was planned preoperatively (Fig. ). Because there was no evidence suggesting the loosening of the femoral component, the stem was planned to be retained. CT showed that the anteversion angle of the stem on the femoral table top plane was 16 degrees. Based on a previously published method of calculation [, ], we planned radiographic cup positioning angle to be 40 degrees of inclination and 26 degrees of anteversion relative to the functional pelvic plane (FPP), which was defined as the coronal plane passing through the bilateral ASIS in the supine position. The anatomic cup positioning angle was planned to be 37.3 degrees of inclination and − 8.3 degrees of anteversion, whereas the radiographic cup positioning angle was planned to be 37 degrees of inclination and − 5 degrees of anteversion and the operative cup positioning angle was planned to be 36.8 degrees of inclination and − 6.3 degrees of anteversion, relative to the APP based on a previously published method []. Preoperative planning using the Stryker navigation system (Stryker, MI) [, ], a CT-based hip navigation system (Fig. ). We did not perform ROM simulation before surgery because the patient’s implant was already dislocated at the presence to our hospital and the CT data cannot to be used for the ROM simulation.\nThe operation was performed using a direct lateral approach. The stem was retained after confirmation of rigid stability during surgery. Exposure of the acetabulum revealed a bone defect in its anterior wall. A 46 mm cup was inserted at 37 degrees of inclination and − 5 degrees of anteversion relative to the APP, as determined radiographically using navigation. Because some patients experience progressive posterior changes in pelvic tilt after surgery, the dual mobility MDM X3 Mobile Bearing Hip System (Stryker, MI) was utilized to reduce the risk of re-dislocation. Intraoperative press fit on acetabular implant was adequate for initial stability.\nThe patient started standing and walking rehabilitation the day after the operation. Three months later, she was able to walk short distances without pain and had a Harris Hip score (HHS) of 78 points. Measurement of her APP tilting angle showed progressive posterior pelvic tilting after the operation (Fig. ), with CT 1 year later showing an APP tilting angle of 47 degrees in the supine position. Three years after the operation, EOS imaging (Paris, France) showed that her APP tilting angle was 60 degrees posterior in the standing position (Fig. ). The sagittal view of EOS imaging showed the angle between APP and femoral shaft was measured to be − 66 degrees (66 degrees extension), indicating hyperextension of the femur in the standing position. The maximum ROM of this patient was assessed by the Zed Hip system. Implant-to-implant impingement was observed at a flexion of 99 degrees and an extension of 68 degrees relative to the APP (Fig. ), indicating a high risk of posterior impingement in the standing position. Gait analysis was also performed 3 years after surgery using VICON612 (Vicon Motion Systems, Oxford, UK) and plug-in-gait marker placement []. The hip joint angle was defined as the angle between the APP and the femoral bone axis. The left hip joint was hyperextended relative to the right hip (Fig. ), although the patient was still able to walk for short distances without pain. At the final follow up of three years after operation, hip flexion, extension, inner rotation and external rotation angle and HHS were improved to 90 °, 0 °, 20 ° and 0 °, and was 74 points. |
In this article, we report the case of a 9-year-old female patient with no previous medical history and no significant family history of osteosarcoma or any other bone tumor, who presented in our clinic on January 2016 with a 1 year history of pain in her low back and left lower limb. She was diagnosed with osteosarcoma of the left tibia with a solitary metastasis in her L3 vertebrae on February 2016. She was subsequently treated with a left below knee amputation and L3 corpectomy with posterior spinal fusion and instrumentation from L1 to L5 with decompression laminectomy at L2-3, and L3-4 through a posterior and left thoracoabdominal approach in March 2016 ().\nShe was found to have an abnormal bone scan with a lesion at the L3 level 3 months after completing chemotherapy in November 2016. MRI scan and ultrasound guided fine-needle aspiration cytology (FNAC) of right paraspinal psoas tissue confirmed recurrent osteoblastic osteosarcoma on December 2016 (). On physical examination, she was able to ambulate with use of a below knee prosthesis and demonstrated no neurological deficits. The patient was started on second line drugs, including two cycles of ifosfamide/etoposide. Previous implants were well in place. Imaging was performed with radiographs, CT scan, bone scan, PET scan, and MRI scan with contrast enhancement to confirm only a single metastatic site (). For therapeutic strategy determination, the patient was introduced to our local tumor board. Preoperative workup was completed, and surgery was planned for a complex en bloc resection of L2, L3, and L4 with removal of deep spinal implants with anterior and posterior spinal fusion and instrumentation (). The option of nonoperative palliative care was offered to the patient and her family, but they elected to proceed with en bloc resection to maximize her chances of survival, in spite of high surgical risk and an overall poor prognosis. They were informed preoperatively that a complete resection would require sacrificing her nerve roots at L2, L3 and L4. A palliative decompression was not offered for the revision procedure as a treatment option as the patient was not complaining of pain or neurological symptoms, and it would not have improved her life expectancy. |
A 13-year-old boy was referred to clinic with a prominent dilatation on the right side of his neck that had been gradually enlarging since he sustained a displaced fracture of the middle third of his right clavicle 6 months previously. This was treated conservatively by the referring hospital []. The patient was uninhibited in daily activities, but complained of ongoing pain at the fracture site and was concerned about his cosmetic appearance. His past medical history consisted of a congenital atrial septal defect with partial anomalous pulmonary venous drainage, which was surgically corrected at the age of 18 months. On examination, there was a nonpulsatile dilation in the right anterior triangle of his neck overlying the clavicle which was tender on palpation. There was no other visible deformity and he had full range of motion of the ipsilateral shoulder.\nAn ultrasound and computed tomography (CT) scan were arranged, which revealed a hypertrophic angulated nonunion of the clavicle fracture. The surrounding callus formation was shown to be compressing the EJV, impeding its drainage, and causing the striking distension measuring 2 cm in diameter. The anatomy of the carotid and subclavian arteries appeared normal.\nFollowing multidisciplinary discussion, a decision was made to operatively reduce and fix the clavicle nonunion. Intraoperatively, gross callus formation was found at the fracture site. However, an arteriovenous fistula was also discovered in a vascular cavity in the callus between a branch of the subclavian artery and the EJV. The fistula was excised and adjacent veins ligated and closed by vascular surgeon before the clavicle fracture was reduced and fixed with a six-hole contoured plate without the use of bone graft.\nRadiographs taken 1 week postoperatively confirmed satisfactory position of the clavicle fixation. At 1-month follow-up, the EJV was less prominent and there was no residual pain around the shoulder. At 6-months follow-up, the patient was asymptomatic with full range of movement of the ipsilateral shoulder. Radiographs and CT scans showed union of the fracture site with good alignment of the clavicle []. A CT venogram revealed an enlarged right EJV with a stenosed lower end and tortuous collaterals to the right internal jugular vein and subclavian vein, but no residual arteriovenous fistula []. |
A 20-year-old Danish woman was referred with constant pain in the right proximal thigh that had progressed for 8 years. She had no other known medical conditions. Menarche was at age 13. There was no family history of sclerosing bone diseases. The limb pain was aggravated at night and was moderately relieved during physical activity and by cold baths. She was on daily analgesic medication with nonsteroidal anti-inflammatory drugs, paracetamol, and weak opioid agonists, and she was on sick leave from her university studies at the time of referral due to her symptoms. She was using shoe inserts because of leg length discrepancy.\nAt age 11 she had consulted a rheumatologist because of hip joint pain. Guided by ultrasound a hip joint puncture was performed due to fluid gathering; serology was negative. After the puncture, the joint pain ceased.\nSix months prior to referral to our clinic, she was involved in a traffic accident and hit by a car from the right at knee level. She was discharged from an emergency room (ER) with no suspected fracture and a radiological examination was not performed. She reported that there was significant worsening of the femoral pain after the traffic accident.\nA clinical examination revealed no skin lesions, café au lait spots, or redness; there was no increased temperature or swelling in her right thigh. Leg length was unequal but within normality.\nShe was instructed to fill out a diary with daily registration of pain on an analog scale from zero to 10, where increasing values were equivalent to more intensive pain. Orally administered analgesics were required when the pain exceeded a score of 6 or more, and the score 10 was reserved for symptoms that kept our patient from falling asleep and that did not respond adequately to orally administered analgesics.\nMagnetic resonance (MR) and positron emission tomography-computed tomography (PET-CT) scans revealed significant increased cortical thickness in the right femoral diaphysis that partially obliterated the medullary cavity (Fig. ). The surface of the sclerotic bone was uneven. A Tc99-bone scan showed a pathological increase in uptake in most of the right femoral shaft (Fig. ).\nBiochemical bone markers were normal, including normal alkaline phosphatase.\nScreening for the GNAS1 activating mutation R201 was negative in peripheral blood; as was mutation screening of the SQSTM1 gene, which has been reported to be mutated in Paget’s disease of bone.\nA bone biopsy was performed and showed thickened bone springs with preserved lamellar structure and slightly accentuated cement lines. There was no fibrosis, inflammation, or increased alcian positivity, which would be suggestive of active osteomyelitis. There were no signs of malignancy or neoplasia.\nA gynecological examination prior to referral had found a mild degree of polycystic ovaries (PCO) with normal androgen status. There were no endocrinopathies.\nDuring the clinical investigation, four tentative diagnoses were raised: atypical fibrous dysplasia, Paget’s disease of bone, chronic non-infectious osteomyelitis, and melorheostosis. The young age of our patient, normal alkaline phosphatase, and sparing of the metaphysis of the affected bone made Paget’s disease of bone highly unlikely.\nThe cortical thickening is atypical for the diagnosis of fibrous dysplasia. The absence of café au lait spots, the normal age at menarche, the absence of other endocrine disorders, and the negative GNAS1 mutation examination weighed against a diagnosis of fibrous dysplasia. The long bones are a common location for chronic non-infectious osteomyelitis and this can present in childhood; however, the radiological appearance and bone histology did not provide support for this differential diagnosis. With special emphasis on the uneven surface of the affected bone on X-ray (Fig. ), the diagnosis of melorheostosis, which was fully compatible with the relatively unremarkable histology, was found to be the most plausible diagnosis. In addition, the normal bone chemistry is in keeping with melorheostosis [] although in polyostotic cases alkaline phosphatase can be increased [].\nThe timeline for intervention and clinical outcome is presented in Fig. . Initially, zoledronic acid 5 mg was administered intravenously three times with 10-month and 12-month intervals; our patient had pain relief after the first administration, but a poor effect of the second and third injection. Using an analog pain scale she reported average pain of 9 prior to treatment, 6 after treatment with zoledronic acid, and 4 from 2 to 10 months after initiation of treatment with denosumab. Looking at the usage of pain medication, it went from daily dosage before treatment to 23% of days after treatment with zoledronic acid, and to no orally administered analgesics for 8 months following treatment with denosumab.\nIn October 2015, denosumab was chosen as the second line of treatment based on the hypothesis that the pain was caused by increased bone turnover. A dose of 60 mg was injected subcutaneously with some remission of pain that lasted for 6 weeks. At the same time as the pain relapsed, there was an increase in alkaline phosphatase, PINP, CTx, and osteocalcin in blood tests. Biochemistry before and during treatment is listed in Table . Based on these findings it was decided to shorten the interval between administrations to further strengthen control of a hypermetabolic state. The second dose of 60 mg was given after 8 weeks and this interval has been kept up to the present.\nBiochemistry after treatment with both zoledronic acid and denosumab showed suppression of bone-type basic phosphatase, osteocalcin, collagen I, and procollagen I, with a relative increase in parathyroid hormone, and plasma ionized calcium within the normal range. The injections with denosumab were well tolerated with no adverse effects reported. During the whole treatment period, the injections were administered from our out-patient clinic without cancellations.\nAfter the first three doses given 8 weeks apart, there was pain remission for 8 months; in this period our patient did not take any orally administered analgesics. At the latest follow-up in February 2018, she complained of a slight increase in pain and occasional use of paracetamol and nonsteroidal anti-inflammatory drugs.\nThe treatment with denosumab was well tolerated with no side effects observed.\nA Tc99-bone scan and computed tomography (CT) scan were performed in February 2017 and in February 2018, 5 and 17 months after the first injection with denosumab; the pathologically increased activity and cortical thickening were stationary. |
A 66 year old woman presented to hospital with acute onset of shortness of breath. She was diagnosed as having a non-ST elevation myocardial infarction complicated by congestive heart failure at a community hospital. The patient was stabilized with medical therapy, including parenteral nitroglycerin and non-invasive pressure ventilation. Subsequently, she was referred to our tertiary care centre for a coronary angiogram. Her past medical history included type 2 diabetes mellitus, hypertension, smoking, and peripheral vascular disease.\nCoronary angiogram revealed extensive coronary artery disease, including a 95% lesion of the left main coronary artery. There were no complications during the procedure. The left ventricular (LV) pressure was 90/45 mm Hg. While recovering from the angiogram, the patient became acutely dyspneic and tachycardic. Electrocardiogram revealed sinus tachycardia with no evidence of low voltages nor evidence of recurrent ischemia. Chest x-ray was consistent with pulmonary edema. Initial treatment included intravenous furosemide and nitroglycerin for acute congestive heart failure.\nAn urgent TTE was obtained to rule out complications related to ischemia or angiography. The left ventricle was mildly dilated with severe global systolic dysfunction and an ejection fraction of 20–25% (Figures and , see Additional files and ). There was mild mitral regurgitation with a pulmonary artery systolic pressure of 30 mm Hg. No mechanical complications due to myocardial ischemia were noted. A moderate sized pericardial effusion, measuring 25 mm in diameter was present, particularly along the RV free wall (Figure , see Additional file ). Echodense material attached to the RV was also observed, and was presumed to be thrombus (Figure , see Additional file ). The inferior vena cava was dilated and did not collapse with inspiration (Figure ). Right atrial systolic collapse and mild RV inversion was suggestive of cardiac tamponade physiology.\nThe patient was taken to the operating room for emergent coronary artery bypass grafting and management of the presumed hemodynamic significant pericardial effusion. While dissecting for visualization of the coronary arteries, a large amount of epicardial fat was noted covering the heart. There was no evidence of pericardial fluid or thrombus found during the surgery. Following successful revascularization and removal of the epicardial fat, the 'tamponade' physiology resolved. Post-operatively, the patient's admission was uneventful, with no further symptoms of chest pain or shortness of breath. She fully recovered and was discharged within four days. |
Patient is a 59-year-old lady who initially presented with bright red blood per rectum. She had a past history of Bartholin cyst removal years ago and subsequently has had rectovaginal fistula with multiple repairs. Worsening pain prompted more workup and imaging that showed the pelvic mass. In January 2017, MRI was done at Mayo Clinic which showed a polypoid mass in the lower rectum/anus with extension through the anterior wall of the rectum. It was associated with enlarged left external iliac lymph nodes and enhancing mass on the left side of the pelvis which extended into the sciatic notch. She had a CT-scan of the chest that revealed scattered bilateral sub centimeter pulmonary micro nodules which at the time were indeterminate. There was no prior dedicated prior chest imaging to compare the differences in some of these nodules.\nBiopsy came back as poorly differentiated squamous cell cancer. After a multidisciplinary tumor board discussion and consensus, definitive chemoradiation with fluorouracil and mitomycin was pursued in February 2017 with excellent clinical response with improvement of pain/bleeding.\nScans in May 2017 marked shrinkage in anorectal mass, left pelvic sidewall mass and metastatic external iliac lymph nodes. There was no evidence of metastatic disease in the abdomen. However, enlargement in the previously observed lung nodules was noted. This was now amenable to a biopsy that showed metastatic poorly differentiated squamous cell carcinoma that was consistent with the patient's history of anal primary. There was normal expression of MLH1, MSH2, MSH6, and PMS2; and no expression of PD-L1 (less than 1% of viable tumor cells exhibit membrane staining at any intensity). She was started on doublet chemotherapy with carboplatin and paclitaxel in July 2017. She had initial response followed by later progression noted in February 2018. She was switched to single agent nivolumab in February 2018 which she took till June 2018. Subsequent scans in June 2018 showed significant progression of disease (multiple >20 nodules, growth of existing lung metastases measuring on average more than 2 cm as well as a new liver lesion as measuring 1.6 cm). At the time, given the significant growth and lack of clinical trials but still excellent performance status of the patient, modified DCF (mDCF) was chosen given the data published by Kim et al in Lancet Oncology on 2 July 2018. We repeated the imaging after 2 months of treatment in September 2018, which showed a near-complete ongoing response (>20 lung lesions now indiscernible; liver mass now less than 1 cm). The CT scans done pre-DCF therapy and those done post-DCF therapy are shown in Figures and . The dramatic response may be secondary to prior exposure to immunotherapy, which is increasingly being reported across multiple tumor types where the sequence of immunotherapy followed by chemotherapy is yielding greater than historical responses. |
In this article, we report the case of a 9-year-old female patient with no previous medical history and no significant family history of osteosarcoma or any other bone tumor, who presented in our clinic on January 2016 with a 1 year history of pain in her low back and left lower limb. She was diagnosed with osteosarcoma of the left tibia with a solitary metastasis in her L3 vertebrae on February 2016. She was subsequently treated with a left below knee amputation and L3 corpectomy with posterior spinal fusion and instrumentation from L1 to L5 with decompression laminectomy at L2-3, and L3-4 through a posterior and left thoracoabdominal approach in March 2016 ().\nShe was found to have an abnormal bone scan with a lesion at the L3 level 3 months after completing chemotherapy in November 2016. MRI scan and ultrasound guided fine-needle aspiration cytology (FNAC) of right paraspinal psoas tissue confirmed recurrent osteoblastic osteosarcoma on December 2016 (). On physical examination, she was able to ambulate with use of a below knee prosthesis and demonstrated no neurological deficits. The patient was started on second line drugs, including two cycles of ifosfamide/etoposide. Previous implants were well in place. Imaging was performed with radiographs, CT scan, bone scan, PET scan, and MRI scan with contrast enhancement to confirm only a single metastatic site (). For therapeutic strategy determination, the patient was introduced to our local tumor board. Preoperative workup was completed, and surgery was planned for a complex en bloc resection of L2, L3, and L4 with removal of deep spinal implants with anterior and posterior spinal fusion and instrumentation (). The option of nonoperative palliative care was offered to the patient and her family, but they elected to proceed with en bloc resection to maximize her chances of survival, in spite of high surgical risk and an overall poor prognosis. They were informed preoperatively that a complete resection would require sacrificing her nerve roots at L2, L3 and L4. A palliative decompression was not offered for the revision procedure as a treatment option as the patient was not complaining of pain or neurological symptoms, and it would not have improved her life expectancy. |
This case is of a 55-year-old female who initially presented with a palpable left neck mass. The mass had been noted by the patient 8 years ago and had progressively grown in size. A computed tomography (CT) of the neck was obtained, which demonstrated a 3.5 × 2.3 × 4.6 cm lesion deep to the left sternocleidomastoid (SCM) muscle in addition to a smaller 1 × 1 × 1.8 cm left posterior neck-enhancing mass. A fine needle aspiration (FNA) had been performed 2 years prior at an outside hospital with pathology indicating a low-grade spindle cell proliferation. She was thus referred to our institution for further evaluation. At the time of initial presentation to our group, the patient had noted dysphagia, left ear ache and tinnitus, and neck pain localized to the two masses. A positron emission tomography (PET) CT was performed, which demonstrated increased fluoro-2-deoxy-\nd\n-glucose uptake in the high cervical mass and, to a lesser degree, in the mass located within the posterior triangle of the neck (\n). The outside tissue blocks were reviewed at our institution and felt to be consistent with a peripheral nerve sheath tumor compatible with schwannoma from both lesions. Because of the patient's ongoing symptoms and because malignancy could not be completely excluded based on the results of the prior FNA, the decision was made to proceed with surgical excision of both lesions. Prior to proceeding, a magnetic resonance neurogram was obtained (\n). Tractography demonstrated that the two lesions appeared to originate from the spinal accessory nerve and that apparent diffusion coefficient values were elevated in both masses, supporting the diagnosis of a less aggressive tumor (\n).\nThe patient was taken to the operating room for surgical resection. The patient was positioned with her head turned slightly to the right with the neck extended (\n). The smaller lesion was approached first through the posterior triangle of the neck. Stimulation mapping of the tumor was conducted. The tumor was found to be located on the distal spinal accessory (cranial nerve XI) nerve, with evidence of trapezius activation with nerve stimulation (\n). After identifying no overlying nerve fibers, the tumor was removed en bloc. A separate incision was made in the upper cervical region to approach the larger second mass located lateral and deep to the SCM muscle, which was reflected medially (\n). During dissection of the tumor away from the nerve, motor evoked potentials (MEPs) to the trapezius were lost. As the tumor was of significant size, view of the proximal aspect of the afferent nerve was initially obstructed. Distally, the tumor was mapped, and the fascicle of origin was identified, which appeared to activate the SCM. After significant debulking of the mass, the proximal fascicle of origin was identified but did not provide any muscle activation after stimulation. The tumor was therefore removed in its entirety. All parameters for brachial plexus monitoring remained stable.\nPathology for both lesions was consistent with schwannoma without malignant features. Next-generation sequencing analyzing the coding regions of 479 cancer genes as well as select introns of 47 genes using the UCSF 500 Cancer Gene Test revealed a small in-frame insertion at codon p.R177 of the Sox 10 gene. There were no identifiable alterations in NF1, NF2, LZTR1, SMARCB1, and TRAF7 genes. Despite the change in MEPs, the patient was noted to be full strength in all muscle groups in the left upper extremity including shoulder shrug and head turning immediately postoperatively. At follow-up, her neck pain and prior dysphagia had improved significantly. |
Our patient is a 60-year-old Caucasian woman with a medical history significant for SLE without organ involvement and a benign left breast mass status postexcision in 2006. She was in her usual state of health until screening diagnostic mammogram demonstrated an increased conspicuity of a bilobed nodule within the upper outer quadrant of the left breast. Ultrasound of the left breast demonstrated a 2.3 × 0.8 × 1.9 cm mixed echogenicity partially cystic and partially solid appearing mass 2:00, 9 cm from nipple correlating with bilobed mammographic finding. Given the benign appearance of the mass, short-term follow-up was recommended. The mass persisted on follow-up, and a biopsy was recommended. Pathology from the biopsy revealed a fibroepithelial lesion with features suggestive of phyllodes tumor. She was referred to general surgery and complete surgical excision was recommended. She underwent partial mastectomy, with final pathology demonstrating a 2.5 cm low-grade malignant phyllodes tumor with positive margins. She was taken back to the operating room several days later for re-excision of margins, with pathology indicating no residual tumor. At the time of surgery, the SLE was reasonably well controlled, although she had chronic lesions involving the scalp, torso, and upper extremities, all out of the future radiation treatment field, with limited response to systemic therapy.\nThe patient tolerated both operations well and presented to the radiation oncology department for discussion of adjuvant therapy. The role of adjuvant radiation therapy was discussed at length, and it was explained to her that given the rarity of the tumor, the role of adjuvant radiation therapy is not well defined. We discussed the potential local control benefit with adjuvant radiation therapy, although this was uncertain, and studies have suggested no overall survival benefit with adjuvant treatment. If she were to develop a local recurrence after resection alone, salvage treatment options of surgery and radiation therapy were discussed. Considering her history of chronic cutaneous lupus, she was advised that there was a higher risk of acute and chronic skin toxicity from radiation therapy. After a full discussion, observation was recommended given the SLE as well as the reasonable salvage options available, although she elected to proceed with adjuvant radiation therapy to reduce the risk for local recurrence.\nThe patient was treated in the prone position to a dose of 40 Gy in 15 fractions to the whole breast followed by a 10 Gy boost in 5 fractions. This fractionation and rationale for boost were based on our institutional standards for treatment of breast cancer. For the whole breast treatment, 2 standard oblique tangents and 6 MV photons were used. A field-in-field technique was used to improve dose homogeneity, with a hotspot of approximately 106%. Given the cavity was on the lateral right breast, a 10 Gy boost using a single en-face 16 MeV electron beam without bolus was used ().\nShe was treated concurrently with 200 mg of hydroxychloroquine 2 times daily for treatment of SLE during radiation, as directed by dermatology. The patient's skin was closely monitored throughout her treatment course by a multidisciplinary team of dermatology and radiation oncology and photos were taken periodically. The course of treatment was tolerated moderately well; she experienced acute toxicities of pain, edema, brisk erythema, blistering, and dry and moist desquamation, consistent with expected grade 3 radiation dermatitis within the treatment field. Unexpectedly, she developed a diffuse erythematous, painful rash across the anterior chest wall extending up to the central neck and chin with scattered bullae, which was outside of the treated site (). This required opioid analgesic for pain control, oral antibiotic for suspicion of skin infection, a slow steroid taper beginning at 60 mg daily, frequent Domeboro soaks, and application of topical agents such as Miaderm and silver sulfadiazine cream.\nAt 4 months after completion of treatment, the acute radiation dermatitis reaction within the treatment field had resolved; however, scattered erythematous plaques and scaling on the bilateral breast, anterior chest wall, and bilateral upper extremities persisted. After multidisciplinary discussion, this was felt to be secondary to lupus flare aggravated by radiation, although the etiology was uncertain. At last follow-up, she continued to recover well and was without mammographic findings concerning for malignancy. |
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