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A 60-year-old African American female was following up for her chronically elevated alkaline phosphatase levels. She had a history of hypertension, hyperlipidemia, type 2 diabetes mellitus, allergic rhinitis, and chronic lower back pain. Patient has a family history of arthritis, cardiovascular disease, and diabetes mellitus; she denies ever using alcohol or tobacco.\nWith the onset of elevated alkaline phosphatase level and vague abdominal pain in 2013, an abdominal ultrasound performed in December showed hepatic steatosis. Viral serologies for hepatitis during 2013 were negative, as a gastrointestinal consult was required to determine the need for a liver biopsy. A liver biopsy was subsequently performed, which showed focal mixed micro and macrovesicular steatosis. Portal tracts showed minimal focal chronic inflammation, no significant fibrosis, and no iron deposition.\nThe vague abdominal pain that she was experiencing waxed and waned for two years. Additionally, the patient experienced some vague chest pain and dyspnea that prompted an echocardiogram in February of 2015, which demonstrated a left ventricle ejection fraction of 44%. Consequently, a left heart catheterization in the following month showed no significant coronary disease with a dilated left ventricle with an ejection fraction of 50%. A 2-year follow-up in July of 2015 showed suspicious cirrhosis by Computed Tomography (CT) scan (), possibly due to granulomatous changes and chronic inflammation. A CT scan was determined to be necessary for our patient because of rising alkaline phosphatase without other explainable etiologies, in addition to the patient's appetite suppression and vague abdominal pains. Patient's weight during this time was 207 lbs (93.89 kg) and was advised to diet and exercise. After 4 months of continuous symptoms, especially with abdominal pain, a laparoscopic cholecystectomy was performed with a liver biopsy that showed subsequent granulomatous changes in September of 2015. The liver biopsy showed coalescing periportal nonnecrotizing epithelioid granulomas with associated multinucleated giant cells and chronic inflammation. The chronic and patchy inflammation is representative of the granulomatous hepatitis, despite not having elevated transaminases. The granulomatous changes suggested possible sarcoidosis (). The liver biopsy was not histologically suggestive of nonalcoholic steatohepatitis, with no steatosis noted. Chest X-ray at that time showed no significant findings.\nInflammatory bowel disease (IBD) was not explored in the patient because she never had clinical signs on past or present examinations, denying any symptoms of IBD including alternating bowel habits, predominant constipation, or diarrhea. Laboratory studies 2 years and 6 months since the onset, at the end of 2015 showed negative antinuclear antibodies (ANA), negative rheumatoid arthritis factor, negative cyclic citrullinated peptide (CCP) antibodies (IgG/IgA), elevated C-reactive protein of 8.9 mg/L, normal complement C3/C4, elevated B-type natriuretic peptide 688.6 pg/mL, and negative mitochondrial (M2) antibody. Subsequent office visits and additional laboratory results are shown in .\nAdditionally, patient experienced peak weight loss with a weight of 154 lbs. (69.85 kg) in 2016. During this time period, an echocardiogram showed a decreased ejection fraction of 26%. This result and a history of having ventricular tachycardia resulted in the patient having an automated implantable cardioverter-defibrillator (ICD) placed.\nUrinalysis in June 2017 showed RBC 0-5/hpf, WBC 0-5/hpf, bacteria 2+, and moderate calcium oxalate crystals. Additionally, patient's weight increased in 2017 to 190 lbs. (86.18 kg). |
A 59-year-old male was referred to a pulmonary clinic for the evaluation of bilateral pulmonary nodules which had been progressively increasing in size over the past 2 years. He had presented earlier about 10 years ago with abdominal pain and was found to have a renal mass. He underwent right nephrectomy and was diagnosed with renal cell carcinoma (RCC), mixed papillary and granular cell type with focal sarcomatoid features. One year later, he had cutaneous lesion over right flank, and 2 years later, he had a chest wall mass. Both lesions were resected. Histological features were similar to renal biopsy of RCC. He was treated by his oncologist with sorafenib as metastatic RCC.\nThe pulmonary nodules were detected on surveillance computed tomography (CT) scans 8 years after his nephrectomy and were followed for 2 years []. Over the time, a left lower lobe (LLL) infrahilar mass doubled in size and measured 1.9 cm with mild standardized uptake value (SUV) uptake was observed []. An endobronchial lesion was found on bronchoscopy in the LLL which was biopsied. Pathology showed atypical cell with histological features consistent with neuroendocrine tumor. Immunohistochemistry stains were positive for synaptophysin, chromogranin, and cell adhesion molecule 5.2 []. It was negative for cytokeratin 7, PAX-2 (paired box gene 2), and PAX-8. There was discordance between lung biopsy specimen and previously diagnosed metastatic renal cancer. On review, renal mass showed areas of trabecular architecture more consistent with carcinoid. It had long parallel arrays that have been described in some cases of papillary RCC. All specimens were reexamined and stained. Results were more consistent with carcinoid rather than renal cell cancer. His serum chromogranin was four. Three months after the bronchoscopy, he was reevaluated for hemoptysis. His CT of the chest and abdomen showed increasing LLL and pancreatic mass []. Pancreatic mass also had moderate SUV uptake on preoperative surveillance positron emission tomography scan []. He had a left lower lobectomy and underwent partial pancreatectomy to prevent pancreatic or bile duct obstruction. Both biopsies confirmed carcinoid metastasis with histological features similar to previous samples. |
A 45-year-old man experienced abdominal pain and vomiting. He consulted a nearby doctor, who prescribed an intestinal preparation. The patient developed ten episodes of diarrhea the next day and received infusion therapy. He experienced a persistent sense of abdominal fullness and left abdominal pain for 3 days and then consulted our hospital. The patient was previously healthy and had no remarkable medical history or cardiovascular risk factors. He had no smoking habit. Physical examination revealed mild tenderness over the epigastrium without signs of peritonitis. Enhanced computed tomography (CT) revealed an isolated dissection of the SMA in which the false lumen was thrombosed, the true lumen was compressed by the false lumen, and the middle colic artery to small colon branches were obstructed (Fig. ). No signs of bowel ischemia, such as bowel thickening, abnormal contrast enhancement, or ascites, were found. The creatine phosphokinase level, aspartate aminotransferase level, lactate dehydrogenase level, and base excess were normal at 210 IU/L, 22 U/L, 189 U/L, and 0.3 mmol/L, respectively, at the first visit to our hospital (3 days after onset). The lactate level was not measured at our hospital, but there was a mention in the medical information offer letter that it was normal at a university hospital.\nBecause of the patient’s continuous abdominal pain, we considered the need for emergency surgical therapy and transferred him to our university hospital. He was treated by conservative therapy including antiplatelet agents and prostaglandin, and his continuous abdominal pain disappeared. He was discharged on day 20 and returned to our hospital for follow-up. We performed nutritional management involving central venous nutrition followed by oral intake. We changed his liquid diet to rice gruel, but his nutritional state and oral intake did not improve. On day 35, enhanced CT showed that the area around the SMA was hypodense with abnormal contrast enhancement, and severe stenosis of the true lumen and a 3-cm-long thrombotic occlusion from the middle colic artery were observed (Fig. ).\nWe performed a surgical operation on day 40 because of the prolonged abdominal angina. Spontaneous abdominal pain and tenderness had been present at the first visit, but these symptoms disappeared before the operation.\nThe SMA was revascularized through a midline abdominal incision. During laparotomy, the transverse colon was pulled toward the head side, the origin of the small intestinal mesentery was divided, and the SMA was exposed and controlled at the branch of the middle colic artery. First, we performed thromboendarterectomy. We longitudinally incised the SMA from the thrombotic occluded part to the false lumen, performed thromboendarterectomy with a flap under a direct view, inserted a sheath into the central side (probably the false lumen), clamped the site with vascular tape, and placed an endovascular stent on the central side. A short 0.035-in. guidewire was introduced, and a 6-Fr sheath was placed in a retrograde fashion. Hand-injected retrograde angiography was performed; the false lumen and SMA orifice exhibited contrast enhancement, but the true lumen did not. A 0.035-in. guidewire was used to cross the true lumen into the aorta. The SMA then showed a stenotic area by contrast enhancement. A 10-mm × 4 cm stent (S.M.A.R.T.; Cordis Corp., Fremont, CA, USA) was placed in a retrograde manner at the SMA origin (Fig. ). We then performed a patch angioplasty using the great saphenous vein.\nThe pulse of the distal SMA then improved. Three days after the operation, oral intake was started and the abdominal angina stabilized. The patient was discharged 26 days after the operation. At 58 days after the operation, he was hospitalized again because of hypoalbuminemia and whole body edema. Lower digestive tract endoscopy showed no mucosal disorders, and CT showed a patent stent and no signs of bowel ischemia. We considered that the patient’s symptoms had arisen from a disorder of the absorptive surface area due to chronic weak bowel ischemia and long-term fasting. Central venous nutrition was initiated, and his frequency of diarrhea decreased. Oral intake was then begun, and as his oral intake increased, his nutritional status improved. He was discharged 28 days after readmission. Follow-up CT showed good flow and a thin flap between the stent and patch (Fig. ). He was symptom-free 3 years after the surgery with a good nutritional status. |
A 31-year-old Asian male mechanic reported to the dental clinic in January 2013 with complaint of asymmetry of the face over the past five years. The patient felt that the asymmetry had increased during the first three years (of the five-year duration) but had remained constant over the next two years. There was no history of pain, discomfort or clicking sounds in the temporomandibular joints (TMJ). However, the patient had a history of lower jaw trauma during a sports event at the age of 15 years. No other family member had a similar condition.\nClinical examination of the patient revealed facial asymmetry due to the deviation of the chin to the left side of the face (\n). Examination of the right TMJ revealed a bony swelling in the right preauricular area. Intraoral examination revealed posterior open bite on the right side (\n) and posterior crossbite on the left side (\n). A series of photographs of the patient at the age of 18 years (\n), 24 years (\n), and 27 years (\n) was evaluated. No evidence of facial asymmetry was noticed at 18 years. Mild features of asymmetry were noticed at 24 years and obvious features of asymmetry were noticed at 27 years. A panoramic radiograph revealed the presence of a beak shaped hyperplastic right condyle (\n). The posteroanterior skull view revealed increased length of the condylar neck on the right side (\n). A coronal computed tomography (CT) scan showed enlargement of the right condyle with beak like projection on the medial aspect (\n). An axial CT scan revealed the antero-medial projection of the beak like enlargement (\n).\nBased on the patient’s history, clinical features and imaging findings a diagnosis of hyperplasia of the right condyle was made. The patient was advised surgical treatment of the CH. Unfortunately, the patient was not willing to undergo surgical correction and long term follow up was not possible. |
An 80-year-old Caucasian man who initially presented with back pain was found to have a 9.8 cm infrarenal abdominal aortic aneurysm on computed tomography (CT). This was suitable for endovascular aneurysm repair (EVAR) but the left common iliac artery (CIA) was short, necessitating the extension of the left iliac limb into the external iliac artery (EIA). The decision was made to proceed with an on table coiling of the left internal iliac artery (IIA) and EVAR. However, a tortuous right CIA made coiling of the left IIA extremely difficult and following a prolonged attempt, the decision was made to proceed with EVAR without coiling the IIA (Fig. ).\nOn a 1-month follow up CT the left limb extension had disengaged from the main body, resulting in a large Type 3 endoleak. The disengagement was presumed to be due to the tortuosity of a heavily calcified external iliac artery returning to its anatomical position on removal of the Meir wire (Boston Scientific). The left IIA remained patent and acted as an outflow for the type 3 endoleak. The left limb was then realigned successfully with termination of the type 3 endoleak.\nOn a follow up CT 1 month post repair, the left IIA remained patent with a large type 2 endoleak demonstrated but the sac size remained static (Fig. ). The failure of the IIA to occlude was presumed due to the cavity created within the aneurysm sac from the type 3 endoleak. Due to the large size of the aneurysm and the relatively large size of the type 2 endoleak the decision was made at multi-disciplinary team meeting to embolise the type 2 endoleak.\nAs antegrade access to the left IIA was not possible due to the presence of the stent graft, an attempt was made to access the internal iliac artery via the buttock. Direct sac puncture was not possible as the endoleak was located within the pelvis and surrounded by bony structures and pelvic viscera. The intention was to puncture the posterior division of the IIA but visualisation with ultrasound was limited and a vessel was punctured, which on subsequent angiography was shown to be the anterior division of the left IIA (Fig. ). The left IIA was embolised using Spirali (Pyramed) coils in a retrograde manner. Due to the absence of a solid structure which to compress the access vessel against, especially as the anterior division of the IIA had been punctured, the arteriotomy needed to be closed using an Angio-Seal (Terumo). Correct intraluminal placement of the Angio-Seal (Terumo) is dependent on backflow of blood into the Angio-Seal (Terumo) sheath from the artery. However, as the left IIA had been embolised, backflow from the Angio-Seal (Terumo) was non-existent. The angioseal was not visualised on ultrasound and therefore a 4 French dilator was inserted within the Angio-Seal (Terumo) sheath through which contrast was injected to directly visualise the tip to the sheath within the artery (Fig. ). The sheath was then withdrawn until just inside the vessel lumen and the plug deployed with immediate haemostasis (Fig. ). This was an off-label use of the Angio-Seal (Terumo).\nA follow up CT 1 month post procedure revealed resolution of the Type 2 endoleak. |
A 76-year-old man with a past medical history of coronary artery disease, hypertension, and severe septal hypertrophy suspicious for hypertrophic cardiomyopathy, presented to the hospital with progressive symptoms of heart failure. He had been suffering from worsening dyspnea and lower extremity edema for several months. Lab work over the preceding months had shown deranged liver function tests, concerning for right heart failure. He was admitted for intravenous diuretic and inotrope therapy. At his cardiology clinic appointment prior to this hospital admission, an ECG showed typical atrial flutter and diffuse low voltage []. A right heart catheterization showed moderately elevated right greater than left filling pressures with a right atrial pressure 18 mmHg, pulmonary capillary wedge pressure 21 mmHg and a Fick cardiac index 1.6 liters/min/m2. The ScvO2 was 48%.\nOf note, a year prior to this admission, a transthoracic echocardiogram (TEE) had shown severe asymmetric septal hypertrophy (2.6 cm), suspicious for hypertrophic cardiomyopathy, but had not been further evaluated. A repeat TEE at the present admission showed a left ventricular ejection fraction (LVEF) of 35% as well as the aforementioned septal hypertrophy []. However, due to the significant low voltage on ECG, despite the significant LVH on echocardiogram and history of hypertension, a cardiac MRI was ordered to rule out infiltrative cardiomyopathy as opposed to hypertrophic cardiomyopathy.\nThe images showed normal left ventricular chamber size with severe, asymmetric left ventricular hypertrophy, primarily involving the septal wall with a maximum septal thickness of 27 mm []. The LVEF was measured at 40%. Also noted was diffuse, global late gadolinium enhancement of the left ventricular myocardium, consistent with a diagnosis of cardiac amyloidosis []. The only extracardiac clinical finding suggestive of systemic amyloidosis was carpal tunnel syndrome. Serum and urine electrophoresis did not detect a monoclonal protein, and serum free light chain ratio was low, which significantly lowered the suspicion for AL-amyloidosis. An abdominal fat pad biopsy was ordered which came back negative. Finally, an endomyocardial biopsy was performed which showed a pink amorphous interstitial infiltrate exhibiting apple green birefringence with Congo red stain, confirming cardiac amyloidosis. The subtype was likely wild-type ATTR or variant ATTR amyloidosis.\nIncidentally, the cardiac MRI also found a left upper lobe mass which, on further workup, was characterized as adenocarcinoma. No further differentiation of the subtype of ATTR amyloidosis was pursued, as this would not have changed management, especially in view of the poor prognosis portended by the adenocarcinoma. |
A 20 months old Saudi Boy presented to the pediatric neurology clinic with two weeks history of inability to walk properly as per his mother’s description. His standing and walking were interrupted with backward twisting and flexion of the left arm and left leg with the whole body seems to be in a forward flexion, his right arm having short flexion that do not sustain these movements would ultimately flowed by a backward fall; Video . For the last two weeks he was not able to return to his normal standing and walking.\nBack to his perinatal history; he was a product of full-term uneventful pregnancy to apparently heathy parents with significant obstetric history as his mother had previous three abortions with no living siblings. His father died 6 months prior to presentation in a road traffic accident which added more to the tragedy of the family.\nRegarding his past medial history, it was noncontributory with no previous hospitalizations or surgeries.\nDevelopmentally, he was up to his age. He started sitting, standing and waking at proper age and his abnormal posture and gait were just noticed two weeks prior presentation. His fine motor development was appropriate, and he was able to use his hands appropriately in transferring things between them and he could use the spoon. His speech was also matching to his age.\nHis physical examination at the time of initial evaluation at rest the child was is fully conscious with full orientation, no clear cranial nerve affection. His four limbs had normal tone power and reflexes.\nTwenty-four hours video EEG recording revealed no convulsive movements and no epileptogenic activities. His MRI brain without contrast showed normal brain structure and myelination. Inner ear functions were also assessed by specialist with no abnormalities detected.\nWhole exome sequencing (WES) was requested. More than 20,000 genes of the patient’s DNA were enriched and sequenced. Filtering of the exome data targeted recessive, X-linked, and dominantly inherited diseases.\nWES identified the heterozygous variant c.344A > G, p.(Tyr115Cys) in SGCE which lead to an amino acid exchange (Fig. ). Ten out of the 10 bioinformatic in silico program predict a pathogenic effect for this variant [].\nAllele frequency of this variant in the general population has not been documented. Considering the available information, the variant was classified as pathogenic. This variant causes autosomal dominant myoclonic-dystonia-11 (DYT11; OMIM#159900) [].\nThe condition was explained to the mother and she was tested to the same mutation detected in her child which came out to be negative.\nManagement plan and follow up:\nThe abnormal movements are remarkably initiated with his intention to start walking so initial thinking was the possibility of paroxysmal kinesigenic dyskinesia (PKD).\nBased on the chance that sodium pump inhibitors like phenytoin and carbamazepine might help in PKD, we started our patient on oral carbamazepine at an initial dose of 10 mg per kilogram per day which was doubled after a week time.\nThe patient was reviewed in the clinic after three weeks of implementing carbamazepine therapy with nearly complete resolution of the dystonia and was able to walk properly; Video .\nUnfortunately, the mother was concerned by the side effect of the prolonged use of carbamazepine. She ignored medical advice and went to a local herbal and religious Sheik who advised her to stop the medication.\nThe patient had a complete relapse of his symptoms after withdrawal of carbamazepine. The child was readmitted, and carbamazepine was resumed.\nOn regular follow up over a period of 4 years, the child-maintained response on 20 mg/kg/day of carbamazepine therapy with minimal fine motor tremor in both hands without relapses.\nThe main concern of the mother was; for how long her child would continue on this medication? Would it be safe to wean him off carbamazepine?\nThe family on January 2020 decided to wean off the carbamazepine over one month and fortunately, there was no relapse so far. |
A 71-year-old male patient with diabetes mellitus and hypertension visited the emergency room of our hospital because of hemoptysis. He was diagnosed with a 7 cm sized aortic arch aneurysm 2 years earlier. He was recommended to undergo an operation for the aneurysm, but refused to do so. He also did not take his medicine and was without a follow up examination over that period.\nAt the time of admission, his blood pressure was 150/100 mmHg, pulse rate was 86 beats/min, respiratory rate was 20 breaths/min and body temperature was 36.5℃. The patient was alert, but looked quite ill in appearance. His heart sounds were regular without murmurs, and the lung sounds were decreased at the left lung field. All other aspects of the physical examination were normal.\nThe initial laboratory findings showed that his hemoglobin level was 8.1 g/dL, white blood cell count was 9.7×103/µL and platelet count was 170×103/µL. Additional laboratory studies were normal. An electrocardiogram showed normal sinus rhythm without specific abnormal findings and a chest X-ray showed a mass-like lesion in the left hilar zone (). The chest CT showed a 9 cm sized saccular aneurysm 0.5 cm distal to the left subclavian artery with thrombus in the aortic arch, which was oozing to the anterior wall owing to rupture of the aneurysm, and there was a hemothorax in the left lung (). Labetalol was infused intravenously for controlling blood pressure.\nWe recommended an operation for treating the ruptured aortic arch aneurysm, but the patient and his family refused an operation because of the risk of surgery. Therefore, we decided to perform a hybrid procedure that consisted of a carotid to carotid bypass operation and the placement of stent grafts in the aortic arch aneurysm to save the left carotid artery. We planned to sacrifice the left subclavian artery intentionally and later performed a second stage left carotid to left subclavian artery bypass operation if the patient displayed subclavian steal syndrome, severe dizziness and/or left arm pain. Before endovascular stent graft placement, we checked the whole aortogram and excluded the presence of stenosis that would interfere with collateralization in the carotid or vertebral arteries. We referred him to a thoracic surgery to perform the carotid to carotid bypass operation connecting from the right carotid artery to the left carotid artery in advance for revascularization of the post-stent grafting. Insertion of the aortic stent grafts was performed following the successful by-pass operation. The aortography showed the presence of a huge aortic arch aneurysm 0.2 cm distal to the left subclavian artery (). We deployed two pieces of aortic stent grafts (40 mm×10 cm, 36 mm×10 cm, S&G biotech, Korea) across the left carotid artery and left subclavian artery through the left femoral artery (). The second aortic stent graft (36 mm×10 cm, S&G biotech) was deployed in the first aortic stent graft with a 7 cm overlapped segment to protect against disconnection of the aortic stent grafts. The post-procedure aortography showed no endoleak or other complications.\nDirectly after the procedure, the patient was transferred to the intensive care unit, where he complained of aggravated dyspnea and massive hemoptysis. Chest X-ray showed total haziness in the left lung field and a chest CT showed successful stenting in the aortic arch aneurysm and total ateletasis of the left lung due to external compression of the left main bronchus by hematoma (). Therapeutic bronchoscopy was performed to remove a large amount of blood clots in the left bronchus. The left atelectasis improved on the chest X-ray taken 5 days after a bronchoscopy procedure (). Follow up CT angiography of the aorta after 7 days showed successful stenting, no endoleak and no thrombus in the aortic arch ().\nThe patient was discharged from our hospital without any further adverse clinical events. No cardiovascular events were observed during 1 year of follow-up. |
After problems that manifested with headaches, difficulty walking, weakness of the right limbs, and mental disorders in a woman aged 46 years, on 15 March 2012, a head scan was performed by the method of computed tomography (CT). On that occasion, an extensive expansive lesion bilaterally and frontally was observed, predominantly on the left side with propagation to the left temporal and left parietal lobe, measuring 90 × 80 mm (Fig. ). On 27 March 2012, a maximum tumor resection was performed. Extempore analysis of tumor tissue established that it was glioblastoma [bihemispheric glioblastoma (butterfly glioma)], and the opinion of a pathologist given a few days after the surgery suggested that it was a diffuse astrocytoma (gr II). Following the surgery, the patient was hospitalized in the radiology ward, where 3D conformal radiotherapy was performed by using a linear accelerator with a power of 6 MW. A therapeutic dose of 46 Gy was administered in 23 fractions, followed by radiation of the tumor base with a therapeutic dose of 8 Gy in 4 fractions. With this, the oncological treatment was completed. In August 2012, the patient’s condition deteriorated. At an emergency MRI imaging on 23 August 2012, the progression of the tumor was established, and the dimensions were 46 × 36 mm (Fig. ). After her physician informed her about the options of tumor treatment at this stage, the patient refused further oncological treatment and applied for phytotherapy (PT) on 26 August 2012.\nStPT was introduced, and she used it in the course of following 6 months. The control MRI imaging performed 3 months after the introduction of StPT (on 21 November 2012) found that tumor progression had stopped (Fig. ); therefore, the patient continued to use the combination of herbal medicines. In February 2013, the patient’s condition began to deteriorate, and treatment with corticosteroids was urgently introduced. A daily dose of dexamethasone of 8 mg/day was introduced, and it was quickly raised to 16 mg/day. MRI imaging of 22 February 2013 showed the progression of the tumor, whose radius was 60 mm and which was surrounded by a large perifocal edema that was completely compressing the right lateral cerebral ventricle (Fig. ). Following these findings, the composition of the preparation was modified, and a PTS combination of herbal medicines was introduced instead of StPT. Shortly, after introducing PTS and increasing dexamethasone doses, the patient felt an improvement that mainly manifested by a higher mobility of the limbs. The control NMRI from 21 May 2013 (Fig. ) showed a reduction of the tumor from 60 to 50 mm, and the scan from 01 October 2014 showed that the tumor radius was 45 mm (Fig. ). Further control scans performed during 2015 and 2016 (Fig. ) showed that the tumor radius was approximately of the same dimensions as on the scan from October 2014. Along tumor regression, decreasing of brain edema followed, so the dexamethasone dose was gradually decreased, and it was completely excluded at the end. The patient used PT with full capacity and without breaks for 48 months. After some control scanning showed that tumor dimensions were not changed, a reduced dose of teas (all of five teas, but every other day) was introduced, which she also used at the time of delivering this report. |
A 58-year-old, male, from the urban environment, heavy smoker – 1 pack of cigarettes per day for 25 years, alcohol consumer, referred to Coltea Clinical Hospital, E.N.T. Department for dysphagia, odynophagia, left ear pain and the presence of a tumor mass of the neck, which he stated that appeared about 6 months prior to the examination, with a slow and progressive evolution. The patient is known to have high blood pressure, maximum value of 210 mmHg, under treatment, and without any other significant pathology.\nWhen performing the clinical exam we found an ulcerated tumor mass in the hypopharynx, referring to the left lateral wall of the pharynx and to the root of the tongue. The tumor process extended to the lateral region of the neck involving the submandibular gland, the levels II-V lymph nodes, large vessels of the neck and skin. Imaging studies confirmed the extension of the tumor process. The Doppler ultrasound examination showed a near to 0 blood flow through the external carotid artery and its branches. After performing a biopsy from the tumor under local anesthesia – invasive squamous cell carcinoma, we informed the patient about the choices of therapy and obtained his informed consent to perform the surgical intervention.\nWe performed a tracheostomy in order to ensure a maximum access to the oro and hypopharynx, radical neck dissection with the resection of the internal jugular vein, accessory spinal nerve, sternocleidomastoid muscle, entire fatty tissue from the clavicle to the base of the skull. In order to get the access to the entire tumor mass we needed to perform a lateral mandibulotomy (). The resection piece included the root of the tongue, lateral wall of the pharynx, the submandibular gland, lymph nodes levels II to Va and skin.\nThe pharynx was primarily closed with silk sutures and the continuity of the mandible was made by plates and screws osteosynthesis. The remaining defect was then closed with a pectoral musculocutaneous pedicle flap rotated to the lateral region of the neck (). Side-by-side simple sutures then closed the pectoral region. Drain tubes were placed in the pectoral region and the lateral neck region and also a naso-gastric feeding tube. The drain tubes were removed after 7 days. Enteral feeding was started the first day after surgery and continued for 19 days. After performing a Gastrografin exam of the digestive system that concluded there were no fistulas, we removed the nasogastric tube and the patient started to feed orally.\nFollow-up\nThe evolution of the patient was very good with a 6 weeks postoperative wound normal aspect and relapse free. There is no need for the plate and screws to be removed in a second stage surgery (). The patient started external beam radiation therapy at 8 weeks after the surgery and finished it without significant side effects. The 6 months stage control revealed no sign of relapse and good wound healing. |
The first report describes the case of a 15-year-old girl who developed glioblastoma multiforme (GBM) from the previously treated diffuse astrocytoma (Gr-2). The patient underwent the first surgery at the Neurosurgery Clinic in November 2005 due to the diagnosed diffuse astrocytoma. The second surgery was performed in August 2008, and an inspection of a sample of tumor tissue showed that it was a recurrence of a diffuse astrocytoma. Afterwards, the girl underwent regular checkups and was evaluated on the basis of the findings of MRI that was performed on several occasions after the last surgery. In the early 2010, the patient’s condition worsened. She had a few epileptic seizures, and she was showing signs of sleepiness and had trouble concentrating. On 26 March 2010, an urgent MRI was performed, and it showed a massive recurrence of glial tumor measuring 70 ×60 × 50 mm (Fig. ).\nMeanwhile, there was a sudden deterioration of consciousness of the patient which progressed to the stage of coma caused by spontaneous bleeding from a pathological process in the brain, which is why on the patient underwent an emergency surgery 01 April 2010, during which a left-side decompression parietal-temporal-occipital craniotomy, evacuation of hematoma, and the reduction of an expansive process were performed. As the postoperative CT scan of the brain showed the persistence of cerebral edema and the expansive process, a decompression re-craniotomy and an additional resection of the expansive process were performed. After this procedure, the second control postoperative CT was performed, and since the findings were satisfactory, the girl was awakened and taken off the controlled mechanical ventilation.\nOn 14 April 2010, the patient underwent a new surgical procedure in terms of the maximum reduction of the tumor mass. The samples of tumor tissue were sent for intraoperative analysis, and later to detailed histopathological analysis. After the analysis of the samples obtained, it was concluded that it was a highly anaplastic glial tumor with an expressed vascular proliferation and larger areas of focal hemorrhagic necrosis. A very high mitotic activity was observed within the tumor. In the tumor portion along the necrosis margins, an abundance of foam cells and hemosiderophages, as well as gliosis, was found. From the aspect of immunocytochemistry, 20% of tumor cells gave a positive response to p53. The final diagnosis was glioblastoma multiforme (GBM).\nIn late April 2010, the patient started to use herbal medicine. The StPT combination was introduced, and she used it in the course of following 33 months.\nBetween 17 May 2010 and 23 June 2010, the girl was hospitalized at the radiology ward, where 3D conformal radiotherapy of the brain tumor (glioblastoma multiforme) in the left parietal-temporal-occipital region of the brain was performed by using a linear accelerator with a power of 6 MW. A therapeutic dose of 56 Gy was administered in 28 fractions, along with the accompanying therapy with temozolomid (TMZ) capsules of 75 mg/m2 of body surface area. During therapy, nausea and occasional vomiting occurred once a day, which was put under control by antiemetics. She was discharged in a good general condition. In addition to radiation therapy and the therapy with TMZ, the patient also used herbal medicine. After completion of the combined radio and chemotherapy (RT/CT), the patient underwent no further oncological treatment but continued with PT. As a medical treatment, she used antiepileptic drugs and a dexamethasone dose of 4 mg/day. In September 2010, a control scan was performed which showed the presence of tumor residues, dimensions 8 × 6 mm (Fig. ). In this period, a significant reduction of brain edema volume occurred; furthermore, alongside PT, the patient kept using antiepileptic drugs only. During this period, there had been a significant reduction of the volume of cerebral edema, so the patient, in addition to PT, continued taking only antiepileptic drugs. The control nuclear magnetic resonance imaging (NMRI) performed on 23 December 2010 (Fig. ) showed that the tumor was approximately the same dimensions as on the previous scans. The following control scans showed continuous tumor regression. The NMRI performed on 17 May 2011 (Fig. ) showed that the tumor dimensions were 8 × 4 mm, and the scan performed on 20 September 2011 (Fig. ) showed that the tumor dimensions were 7 × 2 mm. The disappearance of the tumor was determined by a control scan on 19 September 2012 (Fig. ), and NMRIs performed on 25 February 2013 (Fig. ) and 11 March 2014 (Fig. ) showed no radiological signs of a tumor. The patient used phytotherapy for 27 months with full capacity and without breaks, as long as radiological signs of the tumor were present. After this, she kept using all five herbal remedies for 6 months, but every other day, after this, the PT was concluded. |
A 51 year old man presented to our outpatient department with an increasing swelling in the right distal upper arm. He reported about local pain without radiation. The patient´s medical history was without previous infections, surgeries or other diseases. The mass in the arm presented solid and relocatable. The examination showed full strength in all upper extremity muscles, especially in the forearm flexors, in M. pronator, M. abductor pollicis brevis, M. flexor pollcis brevis, M. opponens pollcis and Mm. lumbricales I and II. No sensory loss in the upper arm, the forearm, the palm and dorsum of the hand and the fingers could be found.\nMRI of the upper arm showed a spindle-shaped homogeneously contrast enhancing mass. It was located some centimeters above the crook of the arm within the medial sulcus bicipitalis. In the imaging it showed a relationship to the median nerve main branch of the forearm or seemed to originate from part of its fibres, respectively. Its diameter was about 11 × 4 centimeters (). The primary diagnosis from the radiologist was schwannoma.\nSurgical extirpation was indicated and performed. In its middle part the exposed tumour had a smooth capsule which was opened (). In its equator the surface had a good boundary to the surrounding tissue (). It did not extend to the muscles or tendons. In its distal and especially in its proximal ending the tumour showed a more infiltrative growth (). A feeding fascicle could be identified and was cut after ensuring by electric stimulation that it had no motor function. But with the intention to set no damage at the main nerve trunk approximately twenty percent residual tumour was left ().\nThe postoperative course was uneventful. The patient suffered a light hypesthesia in the forearm. This did not match to the supply territory of the median nerve which is the palmar hand. It rather corresponded to another skin nerve, possibly damaged by the approach. There was no new motor function deficit in the forearm flexors, in M. pronator, M. abductor pollicis brevis, M. flexor pollcis brevis, M. opponens pollcis and Mm. lumbricales I and II. A local upper arm pain vanished in the course of two weeks.\nThe final histological examination of the tumour showed typical criteria of the Castleman disease with an effaced architecture of a lymph node with regressed germinal centers and typical high endothelial venules (). Immunohistochemistry demonstrated regressed atrophic germinal centers () and aberrant network of follicular dendritic cells (). The combination of these features ensured the diagnosis.\nTo exclude a multicentric disease the patient was admitted to the internal medical department. Entire virus tests including HIV were negative. A bone marrow biopsy showed a normal hematopoiesis without evidence for an infiltration by pathologic cells. A staging PET-CT showed no further organ manifestations. An unicentric form was approved in synopsis of all findings. In regard to the tumour rest and the curative approach of an unicentric M. Castleman the patient finally underwent a selective radiation of the upper arm [].\nIn a 6-month follow-up, the partly sensory loss in the forearm had remained. Except for this, the patient had no nerve related problems or restrictions in everyday life except for the sensory loss in the forearm. In a 18-month follow up he reportet on full functionality of the arm. Currently, the area of the tumour is regularly examined with sonography. |
A 39-year-old Japanese male presented with a 3 months history of a skin tumor on the left side of the jaw. Past medical history revealed lung tuberculosis at age 20 years old. Physical examination showed a dome-shaped, slightly erythematous nodule measuring approximately 2 cm in diameter (Fig. ). Preoperative examination revealed no lymph node or distant metastasis. Punch biopsy was performed, and it was diagnosed as mucinous adenocarcinoma of the skin.\nThe tumor was excised with 5-mm surgical margin and was reconstructed with local rotational flap. The definitive histopathology showed small cellular nests of adenocarcinoma lying in pools of extracellular mucin that were separated by fibrocollagenous septae without involvement of underlying muscle. (Figure a,b) The immunohistochemical staining revealed that the tumor cells were positive for CK7, epithelioid membrane antigen (EMA), MUC1, MUC2, estrogen receptor (ER), and progesterone receptor (PR).\nThe patient again noticed a small tumor on the left side of the jaw in the area of the previous operation site 2 years after the primary operation. However, the patient had chosen not to be examined by a physician and left it alone for 4 years. Because of the rapid growth of the tumor, the patient visited our clinic. At this point, the tumor measured approximately 6 cm in diameter (Fig. a). Preoperative examination revealed invasion of the mandible and one left neck lymph node metastasis. The lesion was excised with at least 1-cm border of normal skin including the mandible (Fig. ). A left radical neck dissection was performed, and the mandible was reconstructed with a free scapula flap. The definitive pathology revealed MCS recurrence with no residual tumor cells in the excised normal skin area. One of 20 regional lymph nodes contained metastasis. Postoperatively, the patient was free of tumor and was followed up by the physician in charge every 3 months for 5 years and every 6 months for the next 5 years. The patient was disease-free for ten consecutive years on physical examination, chest X-ray, neck echogram, and ordinary blood test. Although the patient did not have any symptoms, the chest X-ray at the 11th year follow-up revealed multiple lung metastases (Fig. a,b). Additional chest computed tomography (CT), magnetic resonance imaging (MRI), and RI showed cervical vertebrate (Th5) tumor, and biopsy revealed metastasis of MCS. The patient received radiotherapy for pain control and died 3 year after the diagnosis of metastasis. |
A 51 year old man presented to our outpatient department with an increasing swelling in the right distal upper arm. He reported about local pain without radiation. The patient´s medical history was without previous infections, surgeries or other diseases. The mass in the arm presented solid and relocatable. The examination showed full strength in all upper extremity muscles, especially in the forearm flexors, in M. pronator, M. abductor pollicis brevis, M. flexor pollcis brevis, M. opponens pollcis and Mm. lumbricales I and II. No sensory loss in the upper arm, the forearm, the palm and dorsum of the hand and the fingers could be found.\nMRI of the upper arm showed a spindle-shaped homogeneously contrast enhancing mass. It was located some centimeters above the crook of the arm within the medial sulcus bicipitalis. In the imaging it showed a relationship to the median nerve main branch of the forearm or seemed to originate from part of its fibres, respectively. Its diameter was about 11 × 4 centimeters (). The primary diagnosis from the radiologist was schwannoma.\nSurgical extirpation was indicated and performed. In its middle part the exposed tumour had a smooth capsule which was opened (). In its equator the surface had a good boundary to the surrounding tissue (). It did not extend to the muscles or tendons. In its distal and especially in its proximal ending the tumour showed a more infiltrative growth (). A feeding fascicle could be identified and was cut after ensuring by electric stimulation that it had no motor function. But with the intention to set no damage at the main nerve trunk approximately twenty percent residual tumour was left ().\nThe postoperative course was uneventful. The patient suffered a light hypesthesia in the forearm. This did not match to the supply territory of the median nerve which is the palmar hand. It rather corresponded to another skin nerve, possibly damaged by the approach. There was no new motor function deficit in the forearm flexors, in M. pronator, M. abductor pollicis brevis, M. flexor pollcis brevis, M. opponens pollcis and Mm. lumbricales I and II. A local upper arm pain vanished in the course of two weeks.\nThe final histological examination of the tumour showed typical criteria of the Castleman disease with an effaced architecture of a lymph node with regressed germinal centers and typical high endothelial venules (). Immunohistochemistry demonstrated regressed atrophic germinal centers () and aberrant network of follicular dendritic cells (). The combination of these features ensured the diagnosis.\nTo exclude a multicentric disease the patient was admitted to the internal medical department. Entire virus tests including HIV were negative. A bone marrow biopsy showed a normal hematopoiesis without evidence for an infiltration by pathologic cells. A staging PET-CT showed no further organ manifestations. An unicentric form was approved in synopsis of all findings. In regard to the tumour rest and the curative approach of an unicentric M. Castleman the patient finally underwent a selective radiation of the upper arm [].\nIn a 6-month follow-up, the partly sensory loss in the forearm had remained. Except for this, the patient had no nerve related problems or restrictions in everyday life except for the sensory loss in the forearm. In a 18-month follow up he reportet on full functionality of the arm. Currently, the area of the tumour is regularly examined with sonography. |
A 52-year-old right handed Caucasian male farmer fell off a galloping horse landing heavily on his left shoulder. He experienced immediate pain, swelling, and inability to move the shoulder. He originally presented to the emergency department on the same day and initial radiographic evaluation (AP and scapular Y views), Figures and , showed dislocation of AC joint. He had no other injuries and had no active ongoing medical problems. He was subsequently provided with a broad arm sling and referred to orthopaedics where he was seen in fracture clinic five days after the initial injury. On presentation in fracture clinic he had swelling around the AC joint with significant pain and reduced range of movement in his left shoulder (30 deg. abduction, 40 deg. flexion, 30 deg. external rotation, and 10 deg. internal rotation). Neurovascular examination of the left upper extremity including the brachial plexus and axillary nerve was entirely normal. After assessment a diagnosis of type 2 ACJ dislocation was made and a trial of conservative management with a broad arm sling and early physiotherapy was organised. The patient was subsequently reviewed three weeks after the initial injury, when he complained of ongoing pain and loss of function with no improvement with physiotherapy.\nAt this point the patient was referred to the senior author (MW) whose clinical evaluation confirmed a dislocated AC joint with secondary winging of the scapula. With improvement in swelling around shoulder a noticeable asymmetry of the AC joints was observed (). Further radiographic imaging (Y view and axillary view), Figures , , and , confirmed this unusual form of AC joint dislocation with complete dislocation of the joint and the lateral end of the clavicle displaced and locked superiorly directly on top of the acromion. After consideration of patient level of activity and the irreducible locked dislocation, a decision was taken to proceed to open reduction of the joint and subsequent stabilization using the Nottingham Surgilig Ligament (Surgilig, Surgilig Craft, Redditech, UK). Surgery was performed five weeks after injury by the senior author (MW) under general anaesthetic with regional anaesthetic augmentation and intravenous antibiotic cover. The patient was positioned in beach chair position and a bra strap incision was utilised. A superiorly dislocated lateral end of the clavicle was identified. Both acromioclavicular and coracoclavicular ligaments were found to be disrupted but there was no periosteal stripping of lateral end of clavicle. It was not possible to reduce the clavicle so distal clavicular resection of approximately 1 cm was undertaken with subsequent reduction of the acromioclavicular joint. An 11 cm Surgilig ligament secured with a 3.5 × 36 mm fully threaded cortical screw with washer was used to stabilise the joint. Further augmentation and repair of superior acromioclavicular ligament was done using 1-0 PDS suture. Routine skin closure using subcuticular 4-0 monocryl and steri-stips was undertaken.\nThe patient was discharged the following day with a polysling to protect the repair. The initial exercise program included pendulum and single plane passive range of movement for the shoulder and the sling to be worn for 3 weeks. Active assisted exercises were commenced at 3 weeks postoperatively. Full activity was resumed at 8 weeks. At 2 years followup, the patient reported resumption of full activity with no pain. Clinical examination confirmed a full range of pain-free movement at the shoulder, with no residual scapular winging. Radiographs confirmed maintenance of the AC joint 93 stabilization, Figures and . Outcome measures revealed an Oxford shoulder score of 43 (out of 60, i.e., satisfactory joint function) and University of California, Los Angeles (UCLA), shoulder score of 33 (excellent as per UCLA scoring being > 27). |
A 5-year-old girl with known DS and a history of chronic intermittent abdominal pain presented to the emergency department with worsening symptoms. Her abdominal pain has previously been attributed to underlying chronic constipation and possible irritable bowel syndrome. Workup for her abdominal pain included serologic testing for celiac disease, which showed increased anti-gliadin IgA and IgG but normal tissue transglutaminase IgA. On presentation to the emergency department, an abdominal X-ray was done due to suspicion of constipation, and a chest X-ray confirmed left lower lobe pneumonia. She was treated for presumed pneumonia with oral antibiotics. She then returned to the hospital due to persistent fevers and abdominal pain and was admitted for 5 days for intravenous antibiotics. On serial chest X-rays during the hospitalization and after discharge, she seemed to have persistent left-sided and perhilar infiltrates that remained essentially unchanged in appearance. A pediatric pulmonologist was consulted, who felt that she had no symptoms suggestive of asthma, aspirated foreign body, cystic fibrosis, primary ciliary dyskinesia or immunodeficiency.\nA chest computed tomography (CT) scan was done that showed multiple noncalcified pulmonary nodules bilaterally, mainly peripheral in location. The largest nodule measured 8.0 mm and was in the posterior medial aspect of the superior segment of the left lower lobe adjacent to the pleura. The second largest was 7.0 mm in the right pulmonary apex, and another 2 nodules measuring 4.0 mm were seen in the right upper lobe (fig. ). Two additional 4.0-mm nodular densities were seen in the posterior periphery of the superior segment of the left lower lobe just caudal to the dominant lesion. There were localized patchy reticular nodular infiltrates in an extreme superior aspect of the superior segment of the left lower lobe and similar extensive infiltrates extending from the suprahilar region on the right side into the right pulmonary apex. These infiltrates demonstrated patchy confluence and air bronchograms with nodularity as well. There was evidence of significant pleural effusion.\nBased on these findings, the patient was referred to the Department of Pediatric Infectious Diseases for further workup. Lymphocyte subsets, immunoglobulin levels and vaccine responses to tetanus and pneumococcus were done, as well as a neutrophil oxidative burst assay for chronic granulomatous disease. Neutrophil function showed 74% activity at 48 h, which was considered a normal response and not compatible with a diagnosis of chronic granulomatous disease. Her immunoglobulin assay showed an IgG level of 1,130 mg/dl (range 226–1,870), an IgA level of 218 mg/dl (range 3–155) and an IgM level of 45 mg/dl (range 12–219). She showed a normal response to the 23-valent pure polysaccharide pneumococcal vaccine. Her tetanus antibody level was 0.97 IU/ml, which was considered a normal immunization response. She had negative blastomyces and histoplasma antibody levels.\nHer lymphocyte subsets were all within normal range. Results showed a CD19 B-cell count of 127 cells/μl, a CD3 T-cell count of 1,033 cells/μl, a CD4 cell count of 605 cells/μl, a CD8 cell count of 395 cells/μl, and a CD56/16 natural killer cell count of 99 cells/μl; the CD4/CD8 ratio was normal at 1.5. After a relatively extensive infectious workup, she underwent a bronchoscopy and CT-guided needle biopsy of the lung nodules. Bronchoscopy showed normal mucosa. The branching pattern also was normal; however, the right upper lobe bronchus gave only 2 branches compared with the usual 3 branches. There were normal secretions. Broncheoalveolar lavage (BAL) analysis was performed, and specimens were sent for bacterial, fungal and mycobacterial culture analysis as well as for cytology. No acid-fast bacilli were seen on fluorochrome stain, and culture results showed no organisms even after 56 days of incubation. Cytology results showed scant bronchial cells and scattered macrophages. The Oil Red O (lipid) stain was negative. CT-guided core lung biopsies showed extensive necrosis with surrounding palisading histiocytes, fibrosis and chronic inflammation (fig. ). Acid-fast bacillus and Grocott's methenamine silver stains were negative for any organisms. |
A 55-year old Caucasian male experienced 6 months of behavioural change and mild headaches. He was previously fit and well and a relatively affluent New Zealander. Initially his wife noticed an insidious loss of motivation and an increase in time spent over everyday tasks. Although the patient himself ultimately became aware of this he did not seek medical review until he developed associated nocturnal occipital headaches. Of note he was pain free during the day, including when supine, coughing, sneezing or straining. Both the patient and his wife denied any changes to his vision, speech, sensation or movement. He also denied any recent sinus symptoms. His general practitioner diagnosed depression and referred him to a psychiatrist. On review the psychiatrist became suspicious of an organic cause and, unusually, referred him directly to neurosurgery for workup.\nOn arrival his neurological examination was entirely normal with the exception of reduced olfaction. There were no obvious cervical lymph node enlargements. However, he was noted to be extremely anxious about the care he received to the point of paranoia. For example, he obsessively complained about the cleaning fumes on the ward and was convinced they played a role in his illness.\nCT and MR imaging revealed a large soft tissue mass extending from his right nasal cavity and ethmoid sinus through the anterior skull base with erosion of bony walls and infiltrating into the frontal lobe causing significant mass effect (Fig. ). The intra-axial section of the lesion had a large cystic component which appeared to contain sediment material. His case was discussed at the local multi-disciplinary team. The consensus decided against an initial transnasal biopsy as there was significant intracranial mass effect and brain shift. A direct combined neuronavigation guided transcranial (bifrontal) and endonasal approach was planned and performed. Intraoperatively it became apparent that the right olfactory bulb was missing whilst the left remained intact, initially suggesting a neuroectodermal origin. Although it affected predominantly the frontal lobe, it extended posteriorly and was infiltrating the hypothalamus. As such complete excision was not possible as during debulking of this posterior aspect of the tumour the measured urine output increased to 300 ml in 15 min.\nPost-operatively he recovered quickly to his baseline level although he remained agitated at times. A post-operative MRI scan revealed enhancement at the posterior aspect of the tumour bed suggestive of residual tumour. For this he has been referred to oncology with the intention of follow-on chemotherapy and/or radiotherapy. Histopathology proved the lesion to be an intestinal-type adenocarcinoma with a mucinous pattern. |
A 55-year-old male patient reported with mild swelling and pain in the left zygomatic region. The history of present illness revealed that the patient had noticed the swelling in the last few weeks and it also had an associated intraoral ulcer. The patient was a known diabetic who was under medication. He had a habit of smoking cigarettes for the past 15 years. He smoked almost 15 cigarettes per day. The clinical examination revealed a firm swelling in the left zygomatic region measuring approximately 3 cm to 5 cm. It was mildly painful on palpation. The left maxillary region had significant paresthesia, nasal obstruction, and episodes of pain. A palpable left submandibular lymph node was present, which was also tender and fixed. The cervical lymph node on the left side was also palpable (Figure ). The intraoral examination revealed an ulceroproliferative growth measuring 4 cm to 6 cm in dimension.\nThe lesion was extending in relation to teeth 23, 24, and 25. The ulcer was covered with necrotic slough. Purulent discharge and bleeding were present in the lesion. The other teeth in the quadrant were missing (Figure ). A panoramic radiograph revealed extruded teeth 23, 24, 25.\nThere was increased radiopacity in the left maxillary sinus (Figure ). Water’s view showed the opacification of the entire left maxillary sinus.\nAn intraoral extension of this mass was also evident. The inferior, posterior, lateral, and medial walls of the left maxillary sinus appeared to be destroyed (Figure ). A computed tomography (CT) scan showed a lesion extending into the maxillary space and the nasal cavity. A heterodense soft tissue lesion showing heterogeneous contrast enhancement in the left maxillary sinus and hard palate with the destruction of the posterolateral wall, medial wall, and floor of the left maxillary antrum, extending into the adjacent retro maxillary space and medially extending into the left nasal cavity, obliterating all meati with the destruction of nasal turbinates. It was also inferiorly extending into the oral cavity. The CT was suggestive of carcinoma antrum. The CT was sufficient to understand the extensions of the lesions and the destruction of nasal turbinates. Further radiological investigations were, hence, not considered.\nConsidering the patient history and clinical features and the fact that patient was experiencing paresthesia, a biopsy was deemed mandatory (Figure ). On microscopic examination, the given hematoxylin and eosin (H&E)-stained soft tissue section showed dysplastic epithelial islands arranged in sheets and nests, invading fibrovascular stroma.\nAs shown in Figure , the dysplastic epithelial cells exhibited an increased nuclear-cytoplasmic ratio, individual cell keratinization, and increased mitotic figures.\nKeratin pearl formation was also evident, suggestive of well-differentiated squamous cell carcinoma. The patient was referred to the maxillofacial surgery department and briefed on surgical and chemotherapeutic treatment modalities. As the patient was from a poor socio-economic background and had also reported at an advanced stage, he declined treatment and was advised palliative management. The palliative treatment mainly concentrated on the pain relief and nutritional needs of the patient. The patient was treated with acetaminophen and, later, with stronger medications like opioids to manage pain. A feeding tube was inserted into the stomach through the throat since the patient was having extreme difficulty in swallowing. Sadly, the patient succumbed within a few weeks. |
A boy aged 14 years presented at our hospital with cellulitis of right foot. He had fever with swelling of the right ankle for the past 2 days. The boy had no previous history of any major illness or hospital admission. Preoperative laboratory blood tests yielded normal results. He was posted for emergency debridement of the right foot. After informed consent of the patient's parents, the resident anaesthesiologist elected to give him spinal anaesthesia for the surgery. With the routine monitors attached to the patient in sitting position, lumbar puncture was done in a single attempt with a 25G spinal needle in L3-4 interspace. There was no bloody tap and the cerebrospinal fluid (CSF) drawn was clear and colourless. Thereafter, 50 mg of 5% hyperbaric lignocaine was injected in subarachnoid space after barbotage (dilution to 2% concentration and a total volume of 2.5 ml). Paresthesias or pain at the time of lumbar puncture or injection of the drug was absent. The level of sensory block at the start of surgery was at T10. Intraoperatively, intravenous midazolam 1 mg was given for sedation. The surgery lasting for 45 minutes was completed uneventfully with all the haemodynamic parameters remaining stable. The patient was then shifted to recovery room.\nAfter four hours in the recovery, the patient complained of inability to move his legs. He had also developed paresthesias in medial parts of both thighs and urinary retention. There had been no regression in his sensory block, which had remained at T10. Similarly there had been no recovery of the motor power in the lower limbs and deep tendon reflexes were absent. Fever, headache, vomiting or neck rigidity was absent . As patient's clinical features were suggestive of CES, he was started on intravenous methylprednisolone (500 mg/day) and shifted to intensive care unit (ICU) for further management. A neurologist's opinion was sought who confirmed the finding of CES and advised for an urgent magnetic resonance imaging (MRI) of the spinal cord.\nThe MRI imaging of lumbosacral region of spinal cord revealed arachnoiditis with dural ectasia, myelitis with intradural granulomas, clumping of cauda equina nerve roots and fluid collection close to the right posterior paraspinal muscle []. As the MRI findings were indicative of tubercular arachnoiditis, antitubercular drugs (isoniazid, pyrizinamide, ethambutol, rifampicin and pyridoxine) were started while continuing intravenous methylprednisolone (500 mg/day). A serum sample for polymerase chain reaction (PCR) test for tuberculosis was sent which was later reported positive.\nIn the ICU, on postoperative day 2 the patient showed gradual recovery of sensations in lower limb. Return of motor power was seen on postoperative day 6 and recovery of bladder functions on day 8. A repeat MRI scan done during second week showed significant reduction in size and number of intradural granulomas and resolution of arachnoiditis with normal cauda equina nerve roots. There was mild reduction in the paraspinal collection but dural ectasia still persisted. The dosage of steroid was gradually tapered. On postoperative day 15, the patient was transferred to the ward with complete recovery of motor power of lower limbs and bladder functions, and with no residual sensory deficits. He was discharged from hospital a week later with advice to continue antitubercular treatment and to follow-up in neurology clinic. |
A 75-years-old male during an ordinary visit for drug prescription due to his chronic health problems, described his complaints (voice alteration, dysphagia and saliva drooling progressively worsening during the last few weeks) and his general physician referred him for an E.N.T. evaluation. The lack of any pain sensation was the reason for the delay of seeing a physician. His medical history was significant for severe benign prostatic hyperplasia, hypertension and chronic obstructive pulmonary disease. He was heavy tobacco smoker until recently. No history of alcohol intake was referred.\nClinical examination of the oral cavity revealed an oversized pigmented soft mass arising from the left half of his hard palate involving the soft palate and the ipsilateral palatine tonsil causing partial obstruction of the oral cavity and the oropharynx (Figure ). Some pigmented macules of various sizes were also noted growing at the periphery of the tumor. During neck palpation a 2 × 3 cm firm mobile non-tender mass was palpated at the left upper jugular region. A punch biopsy of the oral mass was performed.\nA complete blood cell count, biochemical profile, and urinalysis were ordered without significant findings. A chest x ray was normal.\nHistological examination of the specimen demonstrated extensive infiltration of the ulcerated mucosa by neoplastic predominantly epithelioid cells, in a solid, nested, trabecular or alveolar pattern. The cells were round to oval, having prominent eosinophilic nucleoli and atypical mitotic figures. In areas with intact surface epithelium was identified continuity of the tumor with the epithelium. There were also brown pigment deposition and neoplastic giant cells (figure ).\nA computer tomography with contrast revealed pathologically enlarged cervical lymph nodes of the left neck. Signs of infiltration of the ipsilateral medial pterygoid muscle were also observed (figure ).\nWork up for distant metastases (CT scan of chest, brain and abdomen plus bone scintigraphy) was negative.\nMedical information was provided to the patient and his family regarding the diagnosis, staging, therapeutic options and prognosis. Our patient excluded any option of invasive treatment. He treated for palliation by hypofractionated radiotherapy. The patient died 7 months later from cancer related cachexia. |
An 18-year-old previously healthy female initially presented to the Emergency Department with a 2-day history of peri-umbilical pain which migrated to the right lower quadrant. On further questioning, she complained of anorexia and low grade fever. On examination, she had localized tenderness in the right lower quadrant with rebound tenderness over McBurney’s point. Her laboratory results revealed increased inflammatory markers, a normal urine dip and a negative pregnancy test. A clinical diagnosis of acute appendicitis was made, and she was taken to the operating theatre for an open appendectomy. The intraoperative findings confirmed a suppurating perforated appendix that was removed and sent for histology. In the first post-operative day, the patient was feeling very well with no further complaints, and a provisional plan for discharge was made (–).\nUnfortunately, from Day 2, post operatively the patient started developing symptoms of nausea, vomiting and moderate abdominal pain. On examination, she had mild abdominal distension with minimal tenderness in the lower abdomen with no signs of peritonitis. Blood tests did not display any significant changes. The abdominal radiograph showed dilated small bowel loops and an appendicolith in the right iliac fossa. A computerized tomography scan was performed and demonstrated dilated small bowel loops down to the distal ileum and a 11 mm appendicolith/stone in the right side of the pelvic cavity with no pelvic collection or other positive findings. The clinical diagnosis was that of an ileus, and she was treated conservatively with analgesia, nasogastric intubation and intravenous fluids. In the next 24 hours, her symptoms did not resolve, the abdominal distension progressively worsened with increasing lower abdominal pain and peritonism.\nConsequently, the clinical decision was to take the patient to the theatre for an exploratory laparotomy. At the time of exploration, there were grossly dilated small bowel loops up to a distance of 20–30 cm from the terminal ileum. At that transition point, there were small bowel loops adhered together causing a mechanical small bowel obstruction. In the centre of the adherent loops, an appendicolith was found that was indenting at the point of being cocooned within the wall of the small bowel and the mesentery. There was no perforation of the small bowel. The appendicolith was then retrieved and the loops of the small bowels released, followed by a thorough peritoneal lavage. After surgery, the patient had an otherwise uncomplicated recovery and was discharged on the third postoperative day. |
A 33-year-old married female with a non-significant past medical history presented in 2008 to a primary emergency hospital complaining of right wrist laceration which developed after a traumatic injury caused by a falling glass. At the hospital, the patient was sent to the theater and exploration was performed. Injuries to the ulnar artery, ulnar nerve, flexor carpi ulnaris muscle, and flexor digitorum superficialis muscle were detected. The surgeon at that hospital was only skilled in the correction and repair of arterial injuries.\nThe patient was then referred to our hospital in order to correct the muscle tendons and nerve injuries. A second operation to repair the muscular tendons was performed. During the operation, partial laceration of the ulnar nerve with an extensive neuroma was observed. The lesion was near the wrist joint and no entrapment was found. Accordingly, she was referred for another specialized surgeon in an attempt to remove the neuroma with a possible nerve grafting.\nUnfortunately, the third surgery was delayed for more than 5 months due to the patient’s financial and insurance issues. At that time, the surgeon believed that no surgical approach would be effective. Subsequently, nerve conduction studies were performed, which revealed severe acute and chronic axonal injury to the right ulnar nerve with more involvement in the branch that supplies the first dorsal interosseous muscle compared to the branch that supplies the abductor digiti minimi muscle. Moreover, a complete loss of sensory function of the ulnar nerve was observed (Table ). As a result, the patient was referred for physio- and occupational-therapy sessions.\nIn 2015, the patient presented to our out-patient clinic with the aforementioned history for the possible nerve stimulation. On examination, the patient’s right hand was in an ulnar claw position. A complete loss of sensation in the ulnar nerve distribution was monitored. No additional nerve conduction studies were required.\nBased on our suggested theory about the correlation between immunosuppression and peripheral nerve injuries, the decision was to try the nerve stimulation using peri-ulnar nerve steroid injection of 40 mg methylprednisolone and 1 cc of 1% lidocaine (this treatment regimen was applied on three sessions with an interval of 4 weeks). Gradually, the patient started to regain her sensory and motor abilities. In October of 2016, 1 month after the last steroidal injection, another nerve conduction study was performed which revealed normal ulnar nerve function with no evidence of remaining disability in the nerve function (Table ).\nThe last follow-up visit was in 2019, where she reported normal function and the examination revealed normal motor and sensory functions. |
A 64-year-old man was admitted to our hospital in November 2019 because of intermittent right lumbar pain that had been present for more than 10 years. He was otherwise healthy and had no other complaints. His previous history included four extracorporeal shock wave lithotripsy treatments for right-sided kidney stones 10 years prior; the postoperative course of stone discharge was unknown. No obvious signs were observed during a physical examination. Laboratory investigations revealed that urine leukocytes were present at 12.3 per high-power field. Other laboratory parameters were within normal limits. Renal ultrasound revealed a hyperechoic mass of 2 cm within the renal sinus in the middle of the right kidney. Computed tomography examination of the ureter showed an irregular high-density shadow in the middle calyx of the right kidney and another shadow at the level of the third lumbar vertebra (, , ); the respective sizes of these shadows were approximately 2.5 cm and 2 cm. Pyelography revealed the presence of a “fish-hook” ureter ().\nClinical diagnosis of the patient comprised retrocaval ureter, right ureteral calculi with right hydronephrosis, and right renal calculi. Thus, the patient first underwent right ureteroscopy following induction of general anesthesia. Approximately 15 cm into the ureter, the tract exhibited movement towards the midline. Because of angular factors, a rigid ureteroscope could not be advanced; a 4-Fr ureteral catheter was able to advance to the renal pelvis. Urine outflow was observed at the end of the tail. Consistent with the ureteral computed tomography and pyelography findings, the surgical observations supported a diagnosis of retrocaval ureter. A 6-Fr ureteral catheter was inserted into the renal pelvis to establish artificial hydronephrosis. In contrast to the preoperative computed tomography indications, the junction of the 12th rib and the right posterior axillary line was used as the puncture point. Under ultrasound guidance, percutaneous transluminal kidney puncture was performed; the channel was expanded in a conventional manner and 24-Fr percutaneous nephrolithotomy was performed. Following fragmentation and intraoperative removal of intrarenal calculi, no stricture was observed at the right ureteropelvic junction; the upper segment of the ureter deviated medially and posteriorly, and the enclosed stones were also removed. Upon completion of percutaneous nephrolithotomy, laparoscopic surgery was performed with the patient in the left lateral decubitus position. Incisions were made at the right midaxillary line, 1.5 cm above the anterior superior iliac spine, 2 cm below the 12th rib at the posterior axillary line, and 2 cm below the 12th rib tip at the anterior axillary line. A surgical channel was established and three 10-mm trocars were placed. The inferior vena cava was identified at the level of the inferior pole of the kidney, while the distal ureter was identified outside of the inferior vena cava. The proximal end of the ureter moved inward and upward through the front of the inferior vena cava, curved from the medial side of the inferior vena cava to the dorsal side of the inferior vena cava, and became a dilated proximal ureter outside of the inferior vena cava ().\nThe surrounding ureter was completely dissociated from the inferior vena cava; the ureteral lumen became obviously thinner behind the inferior vena cava, which confirmed that the ureter was located behind the inferior vena cava (). The ureter behind the inferior vena cava (approximately 2 cm) was removed; distal and proximal portions of the ureter were restored via end-to-end anastomosis in front of the vena cava (i.e., via double J-stent placement). No tension or distortion of the ureter were observed after anastomosis ().\nPostoperatively, the patient did not exhibit fever or urine leakage; the ureteral stent was removed at 2 months postoperatively. Infrared spectroscopy analysis revealed that the stone comprised a mixture of calcium oxalate monohydrate and hydroxyapatite. This case report is a retrospective analysis of an individual patient; thus, no ethics committee approval was required. The patient provided oral informed consent for the publication of the report. |
A 78-year-old female was referred to the surgical service for elective cholecystectomy after suffering from a 2nd episode of right upper quadrant abdominal pain due to chronic cholecystitis within three months. The patient's past medical history was significant for hypertension and gastroesophageal reflux. Her vital signs were normal. Physical exam revealed minimal right upper quadrant tenderness with palpation and no jaundice. Her preoperative lab profile, including white blood cell count and liver function tests, was normal (). Preoperative ultrasound revealed gallstones, normal gallbladder wall thickness, and a normal common bile duct diameter.\nDuring laparoscopic cholecystectomy, dissection of the cystohepatic triangle was noted to be challenging due to inflammation. An intraoperative cholangiogram was attempted; however some resistance was noted when the cholangiogram catheter was being fed into the cystic duct. At this point, the cholangiogram was aborted and the cystic duct was closed with 2-0 Vicryl suture in a running fashion. Subtotal cholecystectomy was performed. A Jackson Pratt (JP) drain was placed in the gallbladder fossa. The patient was discharged home on postoperative day one with a JP drain in place after tolerating a fat-restricted diet. The JP drain was removed during outpatient follow-up visit a week after the surgery due to minimal output.\nFive days after removal of the JP drain, the patient returned to the emergency department with right upper quadrant abdominal pain, nausea, vomiting, and intermittent fevers. Laboratory studies revealed elevation of liver function tests with normal lipase (). Abdominal ultrasound did not reveal any gallbladder fossa collection (). Computerized tomography of the abdomen and pelvis revealed central intrahepatic biliary duct dilation and the common hepatic duct was dilated measuring up to 1.2 cm (). Based on this presentation, the decision was made to perform an endoscopic retrograde cholangiopancreatography (ERCP). The ERCP revealed a single large 2 cm stone in the cystic duct remnant causing stenosis of the common bile duct consistent with Mirizzi syndrome along with extravasation of contrast from cystic duct stump (). Sphincterotomy and common bile duct stent placement was performed to temporarily relieve the obstructive pathology and biliary leak. The patient's symptoms and liver function tests showed improvement after the placement of common bile duct stent (). The patient was discharged home on a fat-restricted diet and brought to the operating room for laparoscopic treatment of Mirizzi syndrome from retained cystic duct stone 6 weeks after the initial operation.\nDuring the 2nd operation, we utilized intraoperative laparoscopic ultrasonography to clearly define the presence of cystic duct remnant stone and the common bile duct stent to guide the laparoscopic dissection (). An incision was made directly over the cystic duct remnant and the cystic duct stone was removed (). The cystic duct remnant was then closed with 2-0 Vicryl sutures via intracorporeal suturing technique. A drain was placed in the gallbladder fossa. The patient was placed on fat-restricted diet postoperatively. No bile leak was detected from the drain and the drain was removed prior to discharge. The patient returned to the hospital electively six weeks after discharge for repeat ERCP. On repeat ERCP, the common bile duct stent was removed. Post-stent-removal cholangiogram revealed normal biliary tract appearance with resolution of previously noted Mirizzi syndrome (). On outpatient follow-up, the patient is asymptomatic and has normal liver function tests after removal of the stent (). |
A 70-year-old male presented to our outpatient department with anorexia and weight loss during the last three months. He was diagnosed with myelodysplastic syndrome a year ago and was treated accordingly by a hematologist. The physical examination was noncontributory. The medical history of the patient revealed right nephrectomy 22 years ago owing to renal cell carcinoma; grade 2 (according to Fuhrmann grading system) and T1b (TNM staging system for kidney cancer, 7th edition). His blood glucose was elevated. Liver function tests, serum amylase, bilirubin; CEA and CA 19-9 were within the normal limits. Ultrasound examination of the abdomen revealed a large mass at the region of the pancreatic head. Contrast-enhanced computed tomography of the abdomen was performed, which confirmed a 9 × 5 cm lesion at the head of the pancreas (). Due to his medical history of a right nephrectomy 22 years ago owing to renal cell carcinoma, a CT scan of the brain and the thorax was performed, which was negative for metastases.\nThe patient underwent a pancreatoduodenectomy with pylorus preserving (Longmire-Traverso) in order to remove the tumor. The postoperative period was uneventful, and the patient was released from our department 7 days after his operation. Histopathological evaluation showed a 9 × 5 × 4 cm solid lesion with areas of hemorrhage and necrosis (). The lesion was composed of solid sheets of cells divided by bands of fibrovascular tissue into large nests and alveoli. The cells showed moderate clear-to-granular eosinophilic cytoplasm with well-defined cell borders. The nuclei were central and pleomorphic with conspicuous nucleoli. The lesion was surrounded by a thick collagenous capsule and completely separated from the pancreatic tissue with no infiltration. The morphology was identical to that of the primary renal tumor which was reviewed. A final diagnosis of metastatic clear cell renal cell carcinoma of the head of the pancreas was reached (Figures and ). The surgical margins were free of tumor. The followup of the patient was every 6 months for the first 2 years and then annually. The patient remains free of symptoms and without recurrence of the primary tumor three years after his treatment. |
A 57-year-old African American female presented to our emergency department with a 6-month history of diarrhea, urgency and tenesmus. She was found to have a perirectal abscess. CT of the abdomen was significant for thickening of the rectum and sigmoid colon. On rectal examination, she was noted to have an anal fistula. The perirectal abscess was drained, and a seton was placed for the anal fistula. A colonoscopy was performed showing erythema and friability consistent with colitis extending to the transverse colon, although most pronounced in the sigmoid colon and rectum. The remainder of the colon and the terminal ileum were normal. Biopsies from the involved portion of the colon including the rectum showed acute cryptitis with crypt abscess formation and crypt distortion and were consistent with CD. The patient was started on adalimumab and mesalamine suppositories with significant clinical improvement. A follow-up sigmoidoscopy after 8 months showed resolution of the colitis with only mild proctitis remaining. The patient continues to smoke despite counseling.\nTwelve months into treatment, she developed recurrent boils and nodules in her groin. Her white blood cell count was normal (6,900/μl). She was started on amoxicillin 875 mg and clavulanic acid 125 mg orally twice a day for 10 days. With the suspicion of HS, she was referred to the dermatology clinic, and the diagnosis was confirmed. Her skin lesions improved only transiently on the antibiotics. Her disease progressed with multiple boils and draining sinus tracts in her groin bilaterally with involvement of her right posterior thigh (fig. , HS of the right posterior thigh; fig. , HS of the left groin) but no axillary involvement. She had no prior history of HS or psoriasis. She was started on doxycycline 100 mg orally twice a day for 10 days and subsequently also with 10-day courses of cephalexin followed by clindamycin with minimal improvement. She was started on suppressive therapy with doxycycline 100 mg orally twice a day for 2 months with some improvement. She was transitioned off the antibiotics but the disease recurred. She was treated with several additional 10-day courses of amoxicillin/clavulanic acid followed by clindamycin. She was placed back on suppressive therapy with doxycycline 100 mg orally twice a day for the next 3 months followed by minocycline for one month. Over the past 2 years, she received intralesional triamcinolone injections on five occasions with little improvement. Currently, she is being evaluated by plastic surgery for surgical excision.\nThirty months into treatment, the patient developed erythematous scaly plaques on her back (fig. ) and at the site of adalimumab injections (fig. ) and was diagnosed with psoriasis. Meanwhile, her CD was well controlled on adalimumab with mild disease confined to the rectum with no new fistula formation. It was believed that the adalimumab was causing the HS, and the drug was discontinued despite good control of her CD. The patient was switched to azathioprine. |
A 55-year-old male smoker presented with typical anginal chest pain for three days. Upon arrival to the emergency room, pain was slightly improved and found to have NSTEMI with normal electrocardiogram and elevated troponins. The physical examination was unremarkable. The past medical history was significant for hypertension, dyslipidemia, and chronic ITP which responded to steroids and immunoglobulins in the past. Laboratory blood tests revealed a hemoglobin level of 15 mg/dL, platelet count of 42 × 109/L, and normal PTT and PT. He was admitted to the coronary care unit and was started on simvastatin and nitroglycerin and clopidogrel was held. The patient was given one dose of IVIG and was started on prednisone. On the 7th day of hospitalization, the platelet count was 208 × 109/L and the patient underwent cardiac catheterization through the right femoral artery. A drug eluted stent was placed in the obtuse marginal and a therapeutic ACT was reached after heparin administration. On the 8th day of hospitalization, the patient was discharged on aspirin 325 mg, clopidogrel 75 mg, and prednisone. Patient tolerated these medications well and remained in remission.\nAfter six years, he presented again to the hospital with NSTEMI. The episode occurred one day after receiving dexamethasone and rituximab for ITP relapse. The physical exam was unremarkable and the platelet count was 23 × 109/L. He received in the emergency room nitroglycerin and morphine as well as aspirin 81 mg and the patient was started on IV dexamethasone and IVIG. On the fifth day, the platelet count was 180 × 109/L and the patient underwent cardiac catheterization through the femoral artery. Ostial triple vessel disease was treated with off pump bypass surgery with heparin to maintain an ACT > 300 through the procedure. The platelet count was 149 × 109/L after receiving two days of dexamethasone and the patient was discharged on aspirin 325 mg orally daily. Both patients were followed up for two years without further complications. |
A 45-year-old female presented to the emergency department in the early morning hours with the complaint of acute severe abdominal pain with nausea and vomiting. The patient reported consuming a few glasses of wine prior to going to sleep earlier that night and awoke in the middle of the night with upper abdominal pain. She reported going to her bathroom and experienced retching without vomiting. The patient had previously experienced inability to vomit following gastric sleeve placement approximately 10 years prior. Following the “retching” episodes where she was unable to vomit, she continued to have cramping abdominal pain. She noted that with the continued abdominal pain she also experienced what she thought was an episode of urinary incontinence. Concerned, the patient sat on her toilet and felt a protrusion from her vagina which she presumed to be her bladder.\nThe patient’s past medical history was significant for an abnormal cervical pap smear leading to a total laparoscopic hysterectomy, performed eight weeks prior to this emergency department presentation. Her obstetric history was significant for G4P2022. On review of systems, the patient reported a minor amount of vaginal spotting occurring in the few days following the surgery that resolved without intervention. The patient followed up at four weeks with the operative surgeon and was found to be healing appropriately without any noted complications.\nThe patient was transported by ambulance to the emergency department; she appeared uncomfortable and was experiencing a significant amount of pain. Ondansetron 4 mg and Fentanyl 250 mcg were given intravenously by paramedics en route to the hospital.\nThe physical examination, specifically pelvic exam, was remarkable for a protrusion of small bowel loops from the vagina. Active peristalsis of the bowel was visible on external exam. No areas of ischemic bowel were noted (Video ).\nAfter urgent consultation with gynecology, it was recommended the bowel be immediately reduced and the patient prepared for emergent surgical repair. Subsequently, the bowel was covered with warm, sterile saline moistened gauze, and the bowel was reduced with gentle manual pressure back into the abdominal cavity after administration of intramuscular Midazolam for procedural sedation. Based on this, the bowel was covered in warm sterile saline moistened gauze.\nAfter successful reduction, the patient was emergently transferred to a facility with the capability to surgically repair her vaginal cuff. She was transported in reverse Trendelenburg by ambulance and asked not to perform any Valsalva maneuvers.\nThe operative report revealed that there was an open vaginal cuff with loops of small bowel in the upper vagina. Purulent exudative tissue was found along the margins of the previous vaginal cuff suture lines. No serosal tears were noted along the inspected areas of small bowel. Transvaginal approach was used to repair the vaginal cuff. |
A 49 year old Caucasian woman with a ductal carcinoma of the right breast (pT2N1, ER+, PgR+, HER2+ IIC, Ki 67 70 %), after external quadrantectomy, received sequential adjuvant treatment of adriamycin and cyclophosphamide for four cycles, as well as four cycles of docetaxel and trastuzumab, and radiation on the residual mammary gland. This was then followed by 14 additional treatments with trastuzumab every 3 weeks as monotherapy. The patient did not suffer any co-morbidities and was not taking any other medications during treatment. The cytotoxic treatment was well tolerated; however, 10 days after the 12th dose of trastuzumab in monotherapy the patient complained of burning and watery eyes, a sensation of a foreign body in both eyes, although mainly in the right eye, and impaired vision. These sensations were not due to mechanical trauma to the eyes.\nConjunctival congestion with perikeratic hyperemia and light perilimbal conjunctival edema with corneal marginal infiltrates accompanied by epithelial defects were observed in both eyes (Fig. ). No further ocular alteration was seen. Polymerase chain reaction testing for herpes simplex virus was negative, and cultures of conjunctival secretion were sterile. Complete serum immunology balance, as well screening for autoantibodies, was negative.\nTopical therapy with steroid and antibiotics was started and the right eye was bandaged. Despite initial improvement, the patient’s symptoms worsened after the 13th dose of trastuzumab. As such, we hypothesized a link between HER2 pathway inhibition in the cornea and the ocular side effects. Among the possible strategies to prevent the drug effects on the HER2 pathway in the corneal epithelium, maintenance of sustained activation by administration of a topical serum ligand seemed was proposed as the best solution. Therefore, the 14th administration of trastuzumab was delayed by 1 week, and a sample of peripheral blood was collected. The possibility that a significant concentration of trastuzumab was present in the serum is unlikely due its half-life of 2–12 days. Serum was separated by centrifugation under sterile conditions and stored without dilution at −20 °C. Seven days before the 14th administration of trastuzumab, three drops of serum in each eye was instilled twice per day. Epithelial healing was observed shortly after the onset of treatment with autologous serum, and marginal infiltrates disappeared after 7 days, leaving no haze or scar (Fig. ). Trastuzumab therapy was restarted without relapse of any of the ocular symptoms. Administration of topical autologous serum was continued in conjunction with trastuzumab for the duration of the patient’s treatment. As a result, no further corneal or ocular disease was observed in post-therapy follow-up. |
We present the case of a 71-year-old female patient who presented to a radiologist because of increasing swelling in the right inguinal region. Figure shows an overview of all diagnostic and therapeutic measures taken and their outcome over time. The patient had an abdominal sonography on 25 October 2013 where a tumorous mass of 3.5 × 2 cm was detected. The tumor was surgically removed on 7 November 2013, with re-excision because of an unclear resection margin on 13 December 2013. The tumor was histologically confirmed as a leiomyosarcoma of 4 cm in size, with a mitotic rate of between 30 and 40 mitoses per 10 high-power fields. Assessment of the medical history of the patient revealed arterial hypertension (treated with a β-blocker and an angiotensin-converting enzyme inhibitor), diastolic forms of heart failure with mild cardiac decompensation (treated with furosemide), hyperuricemia with intermittent gouty arthritis of the right large toe (treated with allopurinol), a struma resection in 1990, and diverticulosis of the colon. On 25 November 2013, a computed tomography (CT) of the thorax and abdomen was conducted to assess tumor staging and no metastases were identified. The case was brought to the tumor board and adjuvant radiotherapy combined with chemotherapy was recommended. Between 20 January 2014 and 28 February 2014, 60 Gy of adjuvant radiotherapy were administered, followed by 6 cycles of doxorubicin 75 mg/m2 every 3 weeks between 19 March 2014 and 2 July 2014. Ten months after the end of chemotherapy, a follow-up CT revealed hepatic metastases (30 April 2015), which were confirmed as 6 liver metastases between 1 and 14 mm in size by magnetic resonance imaging on 11 May 2015. At the patient's request, oral therapy with pazopanib 800 mg once daily was administered for approximately 3.5 months between 29 May 2015 and 10 September 2015. Pazopanib was subsequently stopped due to disease progression (Fig. ). A CT of the abdomen confirmed increasing size and number of hepatic metastases. The patient's treatment was switched to trabectedin 1.5 mg/m2 every 3 weeks and a total of 27 cycles were administered between 16 September 2015 and 6 June 2017. Trabectedin was well tolerated by the patient. Concomitant granulocyte-colony stimulating factor was given prophylactically due to the patient's age. Abdominal staging CT assessments were conducted every 4 months until progressive disease, diagnosed on 2 August 2017 (Fig. , ). Overall, a PFS of 22 months was achieved under trabectedin treatment. Upon progression, the patient was switched to eribulin 1.3 mg/m2 on days 1 and 8 of a 21-day cycle on 3 August 2017. |
We present a 64-year-old Hispanic male with Rai Stage II, IgH mutated, and trisomy 12 positive CLL, diagnosed 13 years prior to admission. He was initially observed for 3 years and then received first-line fludarabine, cyclophosphamide, and rituximab (FCR) for 3 cycles with good response. He remained on observation for 4 additional years and then received an additional 4 cycles of FCR followed by 5 cycles of maintenance rituximab. Five years later, he was found to have 17p deleted recurrent disease and he was started on ibrutinib with good response. He presented to an outside facility with acute onset altered mental status after stopping ibrutinib 2 months earlier due to financial constraints. Cerebrospinal fluid (CSF) analysis was initially concerning for CLL infiltration of the CNS with neoplastic-appearing lymphocytes identified by cytomorphology; however, serological tests for West Nile virus indicated acute infection based on positive IgM and negative IgG; however, PCR could not be performed. The patient received therapy for presumed CNS involvement by CLL with intrathecal methotrexate along with intravenous rituximab and methylprednisolone before being transferred to our institution. On admission, his complete blood count revealed leukocytosis with a normal differential and thrombocytopenia, after reviewing the patients' historical trends, we could discern that the platelet count had been within normal limits in the past and had likely decreased due to the acute illness; additionally, we can appreciate the increasing trend in WBCs until the start of treatment around 10 years prior to this admission (). Peripheral blood smear showed normal platelet morphology and moderate leukocytosis with lymphocytosis with coarse, block-like chromatin pattern concerning for prolymphocytoid transformation which had not been present previously. Bone marrow biopsy revealed hypercellularity with diffuse infiltrate of the medium to large-sized mononuclear cells with irregular nuclear contours and prominent nucleoli similar to that seen in the peripheral blood smear (). Repeat CSF studies at our hospital showed lymphocytes accounting for 49% of total events per flow cytometry, positive for CD19, CD20, CD5, and CD23, with lambda light chain restriction, and negative for CD10 and FMC-7 (). The morphology of lymphocytes in CSF was consistent with neoplasia; however, given the admixture of reactive lymphocytes and the presence of known CNS infection, it was deemed that this most likely represented peripheral blood contamination and reactive infiltration of neoplastic lymphocytes.\nHe was treated supportively during his hospital stay and improved without any further CNS-directed therapy. Ibrutinib was later reinstated after the resolution of thrombocytopenia and BTK mutational testing proved negative. The patient continued to improve during outpatient follow-up, prolymphocitoid morphology disappeared from peripheral smear, and brain MRI showed no evidence of CNS disease. |
A 51-year-old woman presented to our clinic complaining of heavy vaginal bleeding, pelvic pressure, bladder incontinence, and discomfort during intercourse. Her exam confirmed the presence of a fibroid uterus extending to the level of umbilicus. She had grade 2 obesity and well controlled diabetes. Otherwise, there were no additional medical problems and no significant surgical history. After counseling, she decided to proceed with uterine artery embolization (UAE) and was referred to an interventional radiologist. A MRI was performed and an incidental finding was noted of an indeterminate 5.5 cm solid enhancing mass inferior to the bladder and inseparable from the superior anterior wall of urethra (). Retrospectively reviewed with the collapsed bladder, it was difficult to differentiate the paraurethral mass from the mass effect of the fibroid uterus.\nThe patient then underwent cystoscopy, which ruled out invasion of the mass into the urethra, and an IR-guided biopsy of the mass, which returned spindle cell tumor with smooth muscle consistent with leiomyoma. Given these findings, additional counseling was provided and the patient elected to proceed with a hysterectomy and paraurethral mass resection. Abdominal access via the vertical midline laparotomy was chosen. Due to the size and position of paraurethral mass, we were concerned about injury to the trigone and therefore a prophylactic bilateral ureteral stenting was done in the beginning of the case. Uneventful total hysterectomy was performed. Entry into the retropubic space (Retzius) was achieved and a nodular mass was identified emanating from the anterior bladder wall. The superior and lateral attachments of this mass were easily dissected free; however the mass was densely adherent to the detrusor muscle (). Sharp and blunt dissection was done to separate the mass from detrusor fibers. The detrusor layer of the anterior bladder wall was then reinforced with interrupted, absorbable sutures, and bladder instillation was done to confirm integrity. One week following the procedure, the patient had a Foley catheter removed and otherwise had an uneventful postoperative course. Paraurethral mass pathology returned as leiomyoma with negative surgical margins (). |
V.F (40-year-old male) was brought to our hospital by police with a reception order from the court after a complaint was made to the police by his sister and his neighbors. V.F was diagnosed to be suffering from paranoid schizophrenia for last 4 years for which he had received no treatment. He believed that people in his locality were his enemies and would kill him and his family. He had been unemployed and was at home for the last 4 years. On further evaluation, he was also diagnosed as suffering from anti-social personality disorder along with alcohol abuse. He stopped his wife and children from going out of the house. He stopped his children from going to school. He would guard, whole day, the gate of his house. Over the last 1 year, his wife and children also started believing that the people in their locality wanted to kill them and stopped any kind communication with them. The delusional context was same in all involved family members. The wife and the children had no past history of psychiatric illness but showed dependent personality traits. V.F had family history of psychiatric illness in his mother, details of which could not be ascertained. He was not suffering from any medical illness.\nAll the family members were intimately associated; the family showed growing social isolation from the social network of neighbors and relatives. None of the family members were employed. The onset of symptoms in all the other family members was 3 years after the symptoms in the primary patient, V.F, started. The primary patient demonstrated most of the other criteria for schizophrenia according to DSM-IV, while other family members showed most features of shared psychotic disorder.\nV.F. was started on antipsychotic medication, tablet Risperidone 2 mg twice per day, which was increased to 6 mg/day in two divided doses, along with electroconvulsive therapy. The wife and children visited the patient only twice during the course of his admission in the hospital which lasted around 2 months. They refused any kind of treatment but their delusional beliefs weakened over the 2 months period. The patient improved and was discharged on tablet Risperidone 6 mg/day, as the family did not want to further keep him in the hospital. Following discharge from the hospital, the family refused to live separately from the patient. The patient did not comply with treatment and was lost to follow up after four outpatient visits. |
A 94-year-old female was presented with a 24-hour history of rapidly increasing stridor associated with a diffuse anterior neck swelling and chest pain radiating to her back. She had been experiencing hoarseness of voice for the past four months. She did not report any history of dysphagia or odynophagia. Her past medical history included a benign tumour of the soft palate, which was treated with radiotherapy during the late 1980's. She is a nonsmoker.\nOn examination in the emergency department, she had a diffuse, firm anterior neck swelling that was nontender. There was obvious distension of the neck and upper anterior chest veins suggesting the possibility of a superior vena cava obstruction as a result of the neck swelling. The patient was in extreme distress with an impending airway obstruction. A chest radiograph () was performed in the emergency department and demonstrated an unusual large air- filled swelling in the neck. It was evident that the patient was tiring and struggling to maintain adequate oxygenation on maximal oxygen therapy. The patient was therefore directly transferred to theatre for further assessment and definitive airway management. In theatre, with attendance of the anaesthetic and ENT teams, a flexible nasoendoscopic examination demonstrated a smooth and diffuse, noninflamed swelling of the posterior pharyngeal wall which was causing an acute airway obstruction. Vocal cord movements were normal but there was severe oedema of the supraglottic tissues, which further compromised the airway.\nA secure airway needed to be established so the patient underwent awake fibre-optic intubation, which proved to be difficult given the gross oedema of the upper airway. Given the difficulty with intubation, a decision was agreed to perform an emergency tracheostomy under local anaesthesia. Subsequent rigid pharyngoscopy demonstrated a compressible mass in the posterior pharyngeal wall, and oesophagoscopy revealed massive food impaction of the whole length of the oesophagus within a grossly dilated oesophagus. The impacted food bolus had displaced the upper oesophagus by causing it to “slide” behind the pharynx to create the observed posterior pharyngeal wall swelling. Further, the food impaction had caused local venous congestion and secondary oedema of the upper airway mucosa compounding the airway obstruction. Food was evacuated up to 33 cms, which fully resolved the posterior pharyngeal wall swelling with correction of the airway compromise.\nFlexible oesophogastroduodenoscopy was performed the following day to examine the lower oesophagus. This revealed a grossly dilated and tortuous oesophagus () without the presence of an oesophageal stricture. Barium swallow was also performed and demonstrated a uniformly dilated, atonic oesophagus without evidence of achalasia (Figures and ).\nThe placement of the tracheostomy resulted in the patient being discharged several days after safe removal and adequate closure of the surgical site. |
This 18-year-old male patient presented at the Oral and Craniomaxillofacial Surgery Clinic to consider surgical treatment options for reducing an enlarged tumor of the back. The patient had more than six café au lait spots on the trunk and extremities, axial and inguinal freckling and several cutaneous tumors that were slightly raised above the level of the skin. The patient had no physical discomfort, no motor or sensitive deficits. The patient stated that he had been operated 2 years earlier on a tumor of the back in another hospital. More detailed information was not available. Despite this previous treatment of the tumor, the remaining tumor mass disturbed him both physically and in his self-perception. The patient stated that the tumor had been growing again since the first operation.\nOn the back there was a tumorous protrusion of the intact skin with a maximum above the spine, which extended from the lower thoracic region close to the edge of the pelvis (Figure 1 A ). The tumor was clearly prominent under tight-fitting clothing. The skin in this area was darker pigmented throughout the lumbar region and showed hirsutism. The tumor was insensitive to touch and pressure, showed no fluctuation on palpation, and the covering skin moved with the tumor.\nB-scan ultrasound revealed an inhomogeneous mass with focal, partly string-like reflections inside the space occupying lesion. Borders were poorly defined and the tumor mass reached to the spinous processes of the spine. The tumor appeared as solid mass and contained no cavities suggestive of necrosis. The tumor was resected in general anesthesia. When the lesion was exposed, a black pigmentation became apparent, which was partially arranged in a stripe-like pattern and frayed at the edges (Figure 2 ). The tumor was resected and the contour of the back reshaped. Despite dense suturing of the wound margins a hematoma developed, which was emptied. Secondary wound healing took 21 days and led to a stable healed wound (Figure 1 B ). There was no movement restriction of the patient after the wound had healed.\nUpon neuropathological investigation a spindle-shaped, 22x9x2 cm3 large skin sample with centrally located 6 cm long scar was seen. Cutting the skin exposed white and slightly greasy tissue on both sides of the scar with spotty brown-black pigmentation.\nHistological examination revealed a diffusely grown neoplasia of medium to high cellular density in the subepidermal connective tissue, consisting of roundish and oblong cells with delicate cytoplasmic extensions and slightly pleomorphic, small, round-oval, sometimes comma-shaped nuclei. The cells showed different degrees of pigmentation. Repeatedly, pseudo-Meissner corpuscles were observed. There was no evidence of mitoses and no Turnbull-positive hemosiderin pigment was detected. Immunohistochemistry demonstrated labeling of the tumor cells with antibodies against S100-protein and melan-A and to a lesser extent also with antibodies against HMB45. The Ki-67-proliferation index was less than 3%.\nA subepidermal diffusely grown pigmented (melanotic) neurofibroma WHO grade I was diagnosed (Figure 3 ). |
A 30-year-old female had reported to our department with a chief complaint of a swelling on the inner side of lower left posterior region of the mandible since 8 years. Her history revealed that 8 years back she developed a small swelling measuring around 5 mm in diameter which slowly progressed to current size. The swelling was asymptomatic, but, as the swelling had increased in its size, now the patient experiences discomfort while chewing food and wants to have it removed. The patient did not have any significant medical and family history and also gave no history of trauma. Physical examination did not reveal any kind of similar swelling in any other region of the whole body and also patient did not have any significant family and medical history of such swellings so Gardner's syndrome was ruled out.\nIntraorally a solitary oval shaped well defined pedunculated swelling was present on the lingual aspect of teeth 36 and 37 which measured 1 cm in diameter (). The overlying and surrounding mucosa was normal. On palpation it was lobulated, nontender, and bony hard in consistency.\nMandibular occlusal cross-sectional radiograph showed a well defined irregular shaped radiopacity attached to the lingual cortex of 37 with a stalk (). Its radiodensity was equivalent to that of the adjacent body of the mandible. Differential diagnoses of exostosis, peripheral ossifying fibroma, osteoid osteoma, osteoblastoma, and osteosarcoma were included. Patient consent was taken after careful explanation of the surgical procedure used and the risks and benefits. Excisional biopsy was performed under local anesthesia. Intraoral incision was performed with periosteal elevation. It was removed completely with the help of rotary instrument and chisel.\nThe histopathological evaluation of the excised specimen showed that the lesional tissue was composed of dense compact bone tissue exhibiting concentric lamellae and osteocytes within osteocyte lacunae (). These microscopic findings along with clinical and radiological features guided us to arrive at the final diagnosis of Compact Type Osteoma. |
A 69-year-old man with a history of diabetes mellitus and hypertension visited a local hospital with the complaint of a heavy feeling in the stomach and epigastric distress. An X-ray examination and upper gastrointestinal endoscopic examination revealed a type 2 tumor on the anterior wall of the lower body of the stomach. The pathological diagnosis based on a biopsy specimen from the tumor lesion was anaplastic carcinoma. The patient was referred to the Center of Gastroenterology, Kurume University Hospital, for further examination and treatment for gastric cancer on 7 December 2005. The liver, spleen, and tumor were not palpable on physical examination. The serum levels of carcinoembryonic antigen, carbohydrate antigen 19-9, and cancer antigen 72-4 were within the reference range. An X-ray examination showed an irregular round ulcerative lesion surrounding a well-demarcated, smooth protrusion on the anterior wall of the lower body of the stomach. An upper gastrointestinal endoscopic examination showed an irregular central ulceration with clearly demarcated and raised margins, type 2, on the anterior wall of the lower body of the stomach (Fig. ). The pathological diagnosis based on a biopsy specimen was a poorly differentiated adenocarcinoma. Abdominal computed tomography (CT) showed no metastatic lesions in the liver and no lymph node metastases. The patient was admitted to the hospital and underwent distal gastrectomy with D2 lymph node dissection and Roux-en-Y reconstruction on 25 January 2006.\nThe primary tumor was palpable on the anterior wall of the lower body, and no exposed tumor was seen on the serosa. No macroscopic metastases were found in the lymph nodes, liver, or peritoneum. On macroscopic examination, the specimen resected from the anterior wall of the lower body was sharply demarcated, type 2, and 43 × 41 mm in size (Fig. ). A lower-power view of a cross section of the specimen showed medullary infiltration of neoplastic cells, mainly in the submucosa. A low-power histological view showed that the tumor had invaded the muscularis propria in some parts (Fig. a). The high-power view showed monotonous tumor cells with little cytoplasm and round chromatin-rich nuclei; many mitotic figures were also seen (183 per 10 high-power fields) (Fig. b). A well-differentiated tubular adenocarcinoma was recognized in one part of the surrounding elevation (Fig. c). The tumor showed positive immunohistochemical staining with chromogranin A and synaptophysin (Fig. d, e). The Ki67 index was 66.7 % (Fig. f). Thus, the histological diagnosis was small cell carcinoma of the stomach with moderate lymphatic and venous invasion. No metastases were seen in the lymph nodes on histological examination. The final diagnosis was a gastric cancer type 2 (LM), T2N0H0P0M0 stage IB. The surgery was evaluated as a curative resection with a negative resection margin (R0). The patient’s postoperative course was good, and he was discharged from the hospital on 10 February 2006.\nThe patient was then administered 600 mg of doxifluridine as postoperative adjuvant chemotherapy for 27 months. However, a lung tumor was seen in the left lower lung field on the chest X-ray on 28 April 2008. Chest CT showed a well-demarcated mass, 20 mm in size, at S10 of the left lung (Fig. ). Small cell carcinoma cells were confirmed by transbronchial lung biopsy. Thus, this tumor was diagnosed as lung recurrence of gastric small cell carcinoma. The patient began treatment with chemotherapy comprising cisplatin (CDDP) (90 mg, day 1) + irinotecan (CPT-11) (90 mg; days 1, 8, and 15) (28-day cycle); radiation of 45 Gy (150 cGy × 2 × 15 days) was added. After six courses of this regimen, chest CT revealed no mass at S10 of the left lung on 21 January 2009 (Fig. ). The patient was then followed carefully without chemotherapy. Chest CT subsequently revealed regrowth of the mass at S10 of the left lung on 15 April 2009. The patient was administered S-1 (80 mg, days 1–14) + paclitaxel (PTX) (70 mg, days 1 and 8) (21-day cycle) as second-line chemotherapy from 12 May 2009 to 10 March 2010. Although nine courses of this regimen were administered, the size of the mass at S10 of the left lung increased. Amrubicin (60 mg, days 1–3) (21-day cycle) was then administered as third-line chemotherapy in six courses from 6 April to 11 August 2010. However, chest CT on 31 August 2010 showed that the mass lesion at S10 had increased to 9 × 26 mm and a lymph node at the hilum of the left lung had swollen to 20 mm (Fig. ). Carboplatin (CBDCA) (280 mg, day 1) + etoposide (130 mg, days 1–3) (21-day cycle) was then administered in four courses as fourth-line chemotherapy from 28 September to 4 December 2010. The serum level of cytokeratin 19 fragment (CYFRA) was slightly elevated (Fig. ) but that of neuron-specific γ-enolase (NSE) was within the reference range during fourth-line chemotherapy. Grade 3 and 4 adverse events were leukopenia and neutropenia, respectively. However, these adverse events were recovered during administration of a granulocyte colony-stimulating factor. Chest CT on 7 December 2010 showed that the mass lesion at S10 had increased to 46 × 30 mm and that the swollen lymph node at the hilum of the left lung had increased to 25 mm. Nogitecan (1.1 mg, days 1–5) (28-day cycle) was thus administered as fifth-line chemotherapy in six courses from 5 January to 21 May 2011. Chest CT then revealed that the mass lesion at S10 increased from 48 × 32 mm on 1 March to 65 × 40 mm on 7 June 2011 and a small amount of effusion had developed; however, the lymph node at the hilum of the left lung had not changed in size. Docetaxel (70 mg, day 1) (21-day cycle) was then given as sixth-line chemotherapy in eight courses from 14 June 2011 to 16 January 2012. Chest CT on 6 September 2011 revealed that the mass at S10 had not changed; however, the swollen lymph node at the hilum of the left lung had decreased from 30 × 26 mm to 26 × 18 mm.\nSevere (grade 3 or 4) adverse effects did not occur except those associated with CBDCA + etoposide.\nThe patient developed bradypragia and higher brain dysfunction in mid-October 2011. Brain magnetic resonance imaging (MRI) revealed multiple brain metastases. Moreover, the patient developed lower back pain in mid-January, and the serum level of CYFRA increased to >30 ng/mL (Fig. ). MRI of the thoracic and lumbar vertebrae on 31 January 2012 showed bone metastases in the thoracic vertebrae.\nThe patient was transferred to another hospital for palliative care. He died of brain metastases 74 months after surgery (47 months after recognition of the lung metastases).\nFor patients with gastric NEC, the National Comprehensive Cancer Network guideline recommends multimodal therapy including surgical resection of the stomach and postoperative administration of a regimen for small cell lung cancer, such as CDDP + etoposide (ETP), CDDP + CPT-11, or CBDCA + ETP []. In our case, the patient had been administered doxifluridine as postoperative adjuvant chemotherapy for 27 months; nevertheless, recurrence from gastric small cell carcinoma was recognized. Therefore, we concluded that doxifluridine had no clinical effect on this cancer and started new treatment comprising CDDP + CPT-11 + radiation. Various regimens for small cell lung cancer were administered in the present case, including CDDP + CPT-11 + radiation (first-line), amrubicin (third-line), CBDCA + ETP (fourth-line), and nogitecan (fifth-line) (Fig. ). After first-line therapy, a complete response of the metastatic site in the lung was confirmed on chest CT. S-1 alone or S-1 + CDDP has been administered as the standard treatment for advanced gastric cancer based on the Japanese gastric cancer treatment guideline [] and two phase-III trials (SPIRITS trial: S-1 vs. S-1 + CDDP [] and JCOG 9912 trial: fluorouracil vs. CPT-11 + CDDP vs. S-1 []). Additionally, several case reports documented good clinical effects of S-1 or S-1 + CDDP for NEC of the stomach in Japan [–]. Hainsworth et al. [] performed a phase-II trial of three anticancer drugs including taxane (PTX, CBDCA, and ETP) in patients with poorly differentiated NEC of the gastrointestinal tract and reported a response rate of 53 % and median progression-free survival of 14.5 months. In the present case, no severe adverse effects occurred with the administration of S-1 + PTX as second-line therapy (nine courses for 10 months) and docetaxel alone as sixth-line therapy (eight courses for 7 months) (Fig. ). Some case reports have described good clinical responses with multiple combined chemotherapy for NEC of the stomach, such as CDDP + cyclophosphamide + epirubicin + vincristine sulfate, and ETP + CDDP + doxorubicin hydrochloride [, ]. However, the adverse effects of these regimens were severe. All six regimens administered in the present case involved one or two drugs, and it was possible to continue the chemoradiation therapy for 43 months (Fig. ).\nWhile the patient underwent the first regimen, the metastatic single nodule was localized to S10 of the left lung; thus, the radiation was thought to be effective as local therapy for this metastatic nodule. Moreover, radiation was performed for brain and bone metastasis and was thought to be useful for prolonged survival and improvement in the patient’s quality of life.\nThis case suggests that the addition of S-1 and taxane to a small cell lung cancer regimen with radiation prolongs the prognosis of recurrent NEC of the stomach.\nNSE is reportedly a sensitive tumor marker for neuroendocrine tumors [], but the NSE level was within the reference range in the present case. Although CYFRA is a sensitive marker for non-small-cell lung cancer [], it was a very sensitive marker for progression and metastases in the present case (Fig. ). |
A 31-year-old man was admitted to our hospital, complaining of hematochezia which had lasted for 1 month. His past history involved a high anterior resection of the rectum in our hospital due to intussusception caused by Peutz-Jeghers polyposis (Fig. ). He was hospitalized for 1 month after the procedure. Unfortunately, details of the surgical procedure that had been performed and the reason for his extended postoperative hospitalization were unknown, because clinical records from his previous admission were not available. He was followed up for 3 years after the procedure, during which he had no abdominal symptoms. He remained symptom-free until the month prior to readmission when he began to suffer from hematochezia. On this admission, hematochezia was his only symptom and there were no abnormal abdominal findings on physical examination. Blood test results, including levels of tumor markers, were all within normal limits. Colonoscopy revealed a hemorrhagic tumor with a smooth surface protruding from the anastomosis of the previous high anterior resection, at a distance of 10 cm from the anal verge; a second examination 4 days later revealed that the tumor had disappeared (Fig. ). The biopsied tumor and other small polyps were histologically diagnosed as adenocarcinoma and hamartomatous polyps, respectively (data not shown). We performed a low anterior resection of the rectum, including the anastomotic site with the adenocarcinoma, combined with a resection of the ileum for strong adhesion. The patient was discharged from our hospital 42 days after the operation.\nGross appearance of the resected rectum showed a defect of the rectal mucosa with a smooth edge and a mucosal bulge located at the anastomotic site (Fig. ). Cut surfaces demonstrated a submucosal tumor mainly occupying the proper muscle layer under the defect (Fig. ). Microscopically, the submucosal tumor comprised an adenocarcinoma and a bone lesion at the anastomotic site (Fig. ). The surface of the tumor was covered with granulation tissue (Fig. ). The bone lesion not only included the carcinomatous glands but also normal glands in the bone tissue (Fig. ). In addition, we identified the incorporation of the normal mucosa in the submucosal fibrosis at the anastomotic site (Fig. f). |
A healthy old female aged 65 was referred to our department suffering from an atrophic non-union of the right humerus with a clear hardware loosening and massive bone loss complaining a stiff painful shoulder and referring three previous surgeries to her arm. All limited movements of her arm were located at the non-union site. Her medical history started on September 2012 when, because of diaphyseal humeral fracture, she was treated with an intramedullary reamed retrograde nail. On April 2013, because of a non-union at the fracture site, with a clear loosening of the proximal locking screws (fig 1a), she was treated with nail removal, debridement of the non-union and new fixation with a 9 holed 3.5 LCP plate using an extended antero-lateral approach to the humerus. On December 2013, because of proximal plate and screws loosing without any clear sign of bone healing (fig 1b), she underwent a third surgery with new debridement, autologous bone graft from iliac crest and repositioning of a new 9 holes plate adding 3 metallic cerclages to the construct to increase stability. On February 2015, the patient was referred to our institution because a catastrophic situation: more than 9 cm of diaphyseal bone loss (grade 3 according to Non-Union Scoring System – NUSS) () with a complete loose hardware palpable under the skin, and a reabsorbed humeral head, supposing a necrotic process (fig 2a, 2b). Clinically, the patient was extremely depressed and feed up for the unsuccessful previous treatments. She was complaining a stiff painful shoulder with a residual movement through the non-union site despite no evident peripheral neurological deficit.\nAt our institution, she was assessed to exclude any infection and all the blood tests, ultrasound guided biopsy with white cells count, and cultures from the non-union site were all negative despite a positive labeled leukocyte scintigraphy performed few weeks before our department admission. The shoulder status was assessed using a CT scan, showing visible reabsorption areas of the necrotic bone (fig 3b) and an evident previous nail hole in the metaphyseal region below the humeral head (fig 3a).\nOn April 2015, all hardware was removed in association together with debridement and a wide (11 cm) diaphyseal bone resection replaced by an handmade antibiotic loaded spacer (Gentamicin + Clindamycin) (fig 4). All the intraoperative specimens, including the removed hardware and cultures, resulted negative for any infection.\nTwo months later, the patient underwent the final surgical procedure with antibiotic spacer removal, debridement and a further 1+1 cm bone resection, and implanting a custom made cemented megaprosthesis (Megasytem-C, Waldemar Link, Hamburg, Germany). At 20 months postoperatively, the patient was extremely satisfied with a pain-free recovery to an almost normal lifestyle including car driving despite an incomplete shoulder range of motion recovery (<110° in shoulder elevation/abduction). At the radiological assessment, there were no radiological sign either of implant loosening or glenoid reabsorption or proximal migration of the humeral head (fig 5). |
A 25-year-old female Irish patient presented with recurrent episodes of rhabdomyolysis since childhood. The first episode occurred at the age of 22 months following a respiratory tract infection. At that time, her creatine kinase (CK) serum concentration was noted to be 250 000 U/L.\nThe family history indicated that both parents were well. However, two siblings (brother and sister) had sudden death at the age of 2 and 4 years more than 20 years ago following a short infectious illness with sudden deterioration over a period of hours. In both cases, the children became progressively weak with severe muscle pain and had evidence of rhabdomyolysis and myoglobinuria of uncertain etiology. Skeletal muscle histology and electron microscopy studies at postmortem evaluation were normal in both children, cardiac evaluation demonstrated dilated cardiomyopathy. Notes pertaining to their clinical episodes were not available. On first presentation, our patient had no hypoglycemia and no ketosis during the acute illness, but was initially treated as a possible long chain fatty acid oxidation defect due to the family history.\nThe patient had numerous subsequent admissions with similar presentations of extremely elevated CK concentration (1 000 000 U/L at the age of 9) associated with muscle pain. This was despite aggressive carbohydrate supplementation including nocturnal cornstarch. Her fat intake from food was continually restricted to approximately 40 g/d. Her total fat intake was supplemented with the use of MCT Oil (medium chain triglycerides supplement) and essential fatty acids in the form of walnut oil. Coenzyme Q10 at a dose of 100-200 mg daily was provided on an ongoing basis. The CK concentration was also raised between the episodes (500-2000 U/L). She was advised to limit her exercise to 20 minutes per day and have high-calorie drink prior to any physical activity.\nExtensive investigations were performed over the presenting years with the lack of a definite etiology. Serial urine organic acid profile and the acylcarnitine profiles were normal. A fibroblast fatty acid oxidation study showed normal myristate and palmitate oxidation studies in fibroblasts. Genetic analysis for fatty acid oxidation defect (LCHAD, MCAD, CPT I, and CPT II) and McArdle disease was uninformative.\nAt the age of 16 years, mitochondrial respiratory chain activity measured in a muscle biopsy was normal, however morphological findings, such as intramyocellular lipid, were compatible with lipin-1 deficiency (Figure ). Her echocardiogram and electrocardiogram did not show any abnormal findings.\nAt the age of 19 years, DNA sequence analysis of the LPIN1 gene (all coding exons and flanking intron boundaries corresponding to the canonical transcript variant NM_145693.3) revealed the presence of a common pathogenic intragenic deletion within the LPIN1 gene (c.2295-866_2410-30del1763) encompassing exon 18 (HGMD accession: CG085181). However, a second mutation could not be identified.\nSubsequently, the LPIN1 coding region was analyzed by reverse-transcriptase PCR (RT-PCR) from total RNA isolated from muscle tissue and conventional DNA sequencing (Figure A,B). In addition to transcripts lacking exon 18 or exons 18-19 (corresponding to the allele harboring the genomic exon 18 deletion), an alternative exon spliced in between exon 5 and exon 6 was detected in a significant proportion of transcripts (Figure C,D). Because this alternative exon, named exon 5a, corresponded to an alternatively spliced in-frame exon annotated only in an N-terminal LPIN1 transcript variant (NM_001261428.2), further targeted DNA-based sequencing was performed. Indeed, this identified a second heterozygous variant (NC_000002.11:g.11916284C>A), which was formally regarded a nonsense mutation introducing a premature stop-codon within exon 5a (which would correspond to NM_001261428.2:c.942C>A, NP_001248357.1:p.[Cys314*]; Figure ). RT-PCR of total RNA isolated from healthy skeletal muscle detected exon 5a also in transcripts containing the first (noncoding) exon of the canonical isoform (data not shown). However, because exon 5a is currently not annotated to be contained in this main reference transcript (NM_145693.3), further studies will be needed to clearly establish pathogenicity of the novel variant.\nSegregation analysis in the patient's family revealed that her father and younger sister are heterozygous for the common pathogenic intragenic deletion (NM_145693.3:c.2295-866_2410-30del1763), while her mother was a heterozygous carrier of the variant within the alternative exon. Thus, the results of these molecular genetic analyses were formally consistent with the clinical diagnosis of LPIN1-associated rhabdomyolysis, due to compound heterozygosity for a known pathogenic deletion and potentially pathogenic novel mutation.\nAt the age of 25 years, the patient presented with acute muscle pain and weakness following prolonged fasting and strenuous exercise. A rhabdomyolysis crisis was confirmed with a CK of 500 000 U/L. She was admitted to intensive care unit (ICU) for a 2 week period over which period she lost a significant amount of weight. During her critical illness, she received an intravenous infusion of 10% dextrose at 1.5 times maintenance with added electrolytes, sodium bicarbonate, morphine, and dexamethasone. Intravenous carnitine was also provided as the patient had previously observed this to be clinically beneficial. She was treated symptomatically, with shortened fasting periods, corn-starch at night and an “unwell dietary regimes” with a mild restriction of fat (40 g per day) and supplementation with MCT oil and walnut oil for essential fatty acids and Liquigen 5 g daily. On day 11, her CK was monitored 4 hourly and was 1248 U/L. On discharge from ICU, this patient had generalized muscle weakness, stiff lower limb muscles (gastrocnemius), and bilateral drop foot requiring orthotic splints. Muscle weakness gradually improved after months of rehabilitative physiotherapy. Her drop foot has improved somewhat (power from 0/5 to 3/5 for dorsiflexion and extensor hallicis longus over 1 year) with areas of altered sensation anterolaterally below the knees consistent with bilateral residual common peroneal neuropathies (CPN). Serial nerve conduction/electromyography studies demonstrated bilateral CPN palsies and a background generalized myopathy. The most likely cause of this patient's weakness was a critical care neuromyopathy, which has improved with time, in addition to CPN palsies related to significant weight loss while critically ill. However, we cannot exclude the possibility that both defects were related to her lipin-1 deficiency. While myopathy has been reported in a few cases, CPN damage is not a known association of this rare metabolic disorder. |
The patient is a 46-year-old African American female with a past medical history of hypertension and noncompliance with medication, who presented with progressive dysarthria over a 10-day period that had worsened in the previous 3-4 days. The patient denied any other neurological symptoms. On neurological examination, the patient was found to have difficulties with speech and decreased vision due to preexisting glaucoma. The rest of the neurological exam was normal. The patient was admitted for the evaluation of subacute progressive motor dysphasia.\nDuring the hospital stay, an MRI of the brain was performed for the suspicion of stroke or demyelination etiology. The MRI brain with and without contrast showed a left frontal lesion, which was not typical for ischemic infarction or demyelination (). This subsequently broadened the differential to include sarcoidosis, PML, CMV encephalitis, and CNS lymphoma.\nThere was also significant cervical lymphadenopathy seen on MRI as well as axillary lymphadenopathy found on the physical examination. HIV testing was performed for further evaluation in view of new differentials, and the patient was found to be HIV positive with CD4 count of 26.\nBoth lumbar puncture and axillary biopsy were also performed. The results of the lumbar puncture were positive for JC virus and negative for CMV, HSV-1, HSV-2, and EBV viruses. The diagnosis of PML was made. Biopsy of the axillary nodes revealed atypical lymphoid hyperplasia with regressed germinal centers, paracortical expansion, and increased plasma cells, which suggested a diagnosis of MCD (). The patient experienced new onset seizures during the hospitalization as well. After ruling out other potential causes, it was determined that the patient had a focal seizure most likely secondary to her brain lesion. The patient was started on HAART and eventually discharged on antiepileptic medications and Pneumocystis jirovecii prophylaxis.\nAbout three months later, the patient presented with complaints of new-onset right-sided weakness. The CD4 count had increased diminutively from 26 to 29 during these three months. The patient was assessed to have possible immune reconstitution inflammatory syndrome (IRIS) given the new-onset right-sided weakness. MRI with and without contrast showed diffuse bilateral cerebral hemispheric edema, worst on the left cerebral hemisphere which crossed the corpus callosum anteriorly into the right frontal lobe and posteriorly into the left parietooccipital lobe (Figures and ). JC virus was detected in the patient's serum as well as in the CSF again via lumbar puncture. The patient was continued on HAART for PML and additionally placed on dexamethasone after considering IRIS. The patient declined to have a biopsy of the brain lesion. She had a hospital course that was further complicated by worsening kidney and liver functions as well as a urinary tract infection. She was treated for these conditions and subsequently sent to an intensive care nursing home in stable condition. |
A 51 year old man presented to our outpatient department with an increasing swelling in the right distal upper arm. He reported about local pain without radiation. The patient´s medical history was without previous infections, surgeries or other diseases. The mass in the arm presented solid and relocatable. The examination showed full strength in all upper extremity muscles, especially in the forearm flexors, in M. pronator, M. abductor pollicis brevis, M. flexor pollcis brevis, M. opponens pollcis and Mm. lumbricales I and II. No sensory loss in the upper arm, the forearm, the palm and dorsum of the hand and the fingers could be found.\nMRI of the upper arm showed a spindle-shaped homogeneously contrast enhancing mass. It was located some centimeters above the crook of the arm within the medial sulcus bicipitalis. In the imaging it showed a relationship to the median nerve main branch of the forearm or seemed to originate from part of its fibres, respectively. Its diameter was about 11 × 4 centimeters (). The primary diagnosis from the radiologist was schwannoma.\nSurgical extirpation was indicated and performed. In its middle part the exposed tumour had a smooth capsule which was opened (). In its equator the surface had a good boundary to the surrounding tissue (). It did not extend to the muscles or tendons. In its distal and especially in its proximal ending the tumour showed a more infiltrative growth (). A feeding fascicle could be identified and was cut after ensuring by electric stimulation that it had no motor function. But with the intention to set no damage at the main nerve trunk approximately twenty percent residual tumour was left ().\nThe postoperative course was uneventful. The patient suffered a light hypesthesia in the forearm. This did not match to the supply territory of the median nerve which is the palmar hand. It rather corresponded to another skin nerve, possibly damaged by the approach. There was no new motor function deficit in the forearm flexors, in M. pronator, M. abductor pollicis brevis, M. flexor pollcis brevis, M. opponens pollcis and Mm. lumbricales I and II. A local upper arm pain vanished in the course of two weeks.\nThe final histological examination of the tumour showed typical criteria of the Castleman disease with an effaced architecture of a lymph node with regressed germinal centers and typical high endothelial venules (). Immunohistochemistry demonstrated regressed atrophic germinal centers () and aberrant network of follicular dendritic cells (). The combination of these features ensured the diagnosis.\nTo exclude a multicentric disease the patient was admitted to the internal medical department. Entire virus tests including HIV were negative. A bone marrow biopsy showed a normal hematopoiesis without evidence for an infiltration by pathologic cells. A staging PET-CT showed no further organ manifestations. An unicentric form was approved in synopsis of all findings. In regard to the tumour rest and the curative approach of an unicentric M. Castleman the patient finally underwent a selective radiation of the upper arm [].\nIn a 6-month follow-up, the partly sensory loss in the forearm had remained. Except for this, the patient had no nerve related problems or restrictions in everyday life except for the sensory loss in the forearm. In a 18-month follow up he reportet on full functionality of the arm. Currently, the area of the tumour is regularly examined with sonography. |
In November of 1999, a 62-year-old woman with a history of diabetes mellitus and hypothyroidism, was admitted to the hospital with right renal cell carcinoma. The initial blood pressure was 120/70 mmHg. The electrocardiogram (ECG) showed normal sinus rhythm. The initial echocardiography showed normal regional wall motion and normal left ventricular systolic function. There was a 5×4 cm solid mass at the lower pole of the right kidney on the abdominal CT scan, suggesting a renal cell carcinoma. Angioembolization of the right renal cell carcinoma was performed on the 25th of November 1999. A right nephrectomy was planned for the following day. The preoperative blood pressure was 160/100 mmHg in the operating room, and the blood pressure increased to 230/110 mmHg after induction of anesthesia. The ECG changes included T wave inversions and ST segment depression in leads V1-6, which were new ECG findings. The operation was canceled.\nEchocardiography was performed immediately and showed impaired wall motion of all basal and mid-ventricular segments of the left ventricle (). The creatine kinase-MB (CK-MB) level was elevated to 66.4 ng/mL by the next day. The ST-T abnormality was normalized in 24 hours. The Echocardiography was repeated after two weeks and showed normalization of the left ventricular wall motion. The coronary angiography showed normal coronary arteries on the 11th of December 1999 procedure. The renal cell carcinoma was treated with interferon from December 1999 to March 2000. The size of the right renal cell carcinoma was markedly reduced after the interferon treatment. Follow-up abdominal CT scanning was performed in April and November of 2000 (). A left adrenal mass was identified. The size of the mass was about 2 cm. The right renal cell carcinoma and the left adrenal mass were mildly enlarged by March 2002. The size of the left adrenal mass was about 2.5 cm ().\nThe patient had no subjective symptoms and was lost to follow up. In December 2003, the patient visited the emergency room (ER) with dizziness. The initial blood pressure was 150/70 mmHg. T-wave inversions were noted on the inferior ECG leads. Echocardiography showed impaired wall motion of the apical and mid-segments of the left ventricle and severely decreased LV systolic function (). The peak level of cardiac troponin I was 3.1 ng/mL. Technetium-99m methoxyiso-butylisonitrile (Tc-99m MIBI) single emission computerized tomography (SPECT) revealed no evidence of inducible ischemia. Echocardiography was repeated after one week, the LV systolic function and regional wall motion abnormalities had fully recovered. In September 2004, the left adrenal mass of the patient was enlarged () and she was diagnosed with a pheochromocytoma. The 24 hour urine metanephrine was elevated at 3.478 mg/day, and the iodine-131 meta-iodobenzylguanidine (I-131 MIBG) adrenal scan was compatible with a pheochromocytoma (). The patient had a successful resection of the left adrenal tumor and right kidney in December 2004. Microscopic examination confirmed that left adrenal tumor was a pheochromocytoma and the right renal tumor was a renal cell carcinoma. |
A 73-year-old female patient was evaluated at the Head and Neck Surgery Clinic at the University of Florida. Her presenting complaint was numbness and swelling of her left mandible and an intraoral lesion associated with recurrent bleeding episodes. Symptoms were present for several weeks and had initially been presumed to represent an episode of sialadenitis by an outside provider. Her past medical history was significant for a thyroid nodule and no chronic medical conditions. She had no history of tobacco or alcohol abuse. Physical exam demonstrated a left mandibular lesion approximately 5 cm in size, with fullness of the gingival mucosa overlying the mass. A mucosal punch biopsy was performed and the histology demonstrated a pyogenic granuloma.\nComputerized tomography (CT) showed an aggressive mass destroying the mandibular body (Fig. ) as well as enlarged pulmonary nodules and a lytic bone lesion at T10. Imaging also demonstrated a multinodular thyroid gland with minimal irregularity along the anterior right border. A 4.6 cm nodule was noted in the right thyroid lobe. Fine needle aspiration of the right thyroid mass was interpreted as a follicular lesion of undetermined significance (FLUS). Because the pathology findings were inconsistent with the CT scan, an open biopsy in the OR was performed. A mucosal incision was made over the mass and a biopsy was taken. The lesion was found to be extremely friable and bled significantly requiring ligation of the facial artery. Final pathology demonstrated FTC.\nMultidisciplinary tumor board review recommended surgery followed by radioactive iodine and external beam radiotherapy. The patient underwent total thyroidectomy, neck dissection, segmental mandibulectomy, and bone-impacted fibular free flap reconstruction []. Intraoperative findings included a 10 cm thyroid mass of the right thyroid lobe that extended beneath the sternum to the innominate vein. A segment of mandible was taken from left angle to right parasymphysis, resulting in a defect from right lateral incisor to angle of mandible (Fig. ). Reconstruction was undertaken via a right bone-impacted fibular free flap with skin paddle in addition to a 2.0 mm mandibular reconstruction bar. Final pathology showed mandibular metastasis of FTC with extension into the tongue and soft tissues of the neck (Fig. ). Margins were negative. The 4.6 cm thyroid follicular carcinoma appeared to arise from a calcified pre-existing degenerative follicular adenoma. There was evidence of capsular invasion and extensive lymphovascular invasion. The patient underwent post-operative stereotactic body radiation to the T-10 metastatic lesion and 200 mCi of radioactive Iodine-131. She has been disease free for 18 months.\nA systematic review of the English literature was performed using PubMed, Medline, Embase, and Scopus databases. Search terms describing FTC presenting as a metastatic lesion in the facial skeleton were compiled and implemented. These terms included: “thyroid,” “cancer,” “thyroid carcinoma,” “thyroid cancer”, “metastasis,” and “malignancy” with “oral cavity,” “maxilla,” “mandible,” “sinus,” “paranasal,” and “orbit.” Papers were gleaned for diagnoses of well-differentiated thyroid cancer, FTC, and metastases to the facial skeleton. Reports of metastasis that only involved the soft tissues were excluded. The reports were organized by subsite: mandible, maxilla, jawbone not otherwise specified, nasal cavity or paranasal sinus, and orbit. Data points obtained from literature review included age, gender, primary oncologic diagnosis, site of metastasis, clinical presentation, treatment modality, survival outcome, and time to follow-up were obtained. Statistical analysis was performed with SPSS 23.0 software package (SPSS Inc., Chicago, IL). Survival was estimated by a Kaplan-Meier analysis to the account for censored data. Survival by treatment was analyzed and compared using the log rank test.\nLiterature review identified 64 studies reporting 97 cases of thyroid cancer metastasis to the facial skeleton in the English literature. All metastases were present at the time of presentation. 59 case reports specified well-differentiated thyroid cancer as the diagnosis. Table demonstrates details of these cases. 38 case reports did not specify the diagnosis and were not included [–]. The gender distribution was 9 males, 48 females, and 2 cases in which gender was not specified. While the majority of metastases were to the mandible, other craniofacial sites were also found to be involved.\nTreatment varied between studies and included: Surgery with or without preoperative embolization and radioactive iodine therapy, external beam radiation (primary or adjuvant treatment), and palliative chemotherapy. 22 patients were treated with surgery as initial treatment with or without postoperative radioactive iodine or external beam radiation. 11 patients were treated with external beam radiation as primary treatment. 14 reports did not specify treatment. 4 patients were treated with palliative care; 2 of these patients received palliative chemotherapy. Cases were grouped into: a surgical arm (those treated with surgery and RAI) and a non-surgical arm. 32 studies reported survival outcome and 27 studies reported time-to-follow up. 24 patients survived treatment and 8 patients expired.\nOverall survival for all patients at 2 years was 96% and at 5 years was 59%. Disease specific survival at 2 years was 96% and at 5 years was 72%. Patients treated with surgery and RAI versus those treated by non-surgical means were compared. There was no statistical difference in overall survival (p = 0.27) with the surgical group having 2 and 5 year overall survival of 100% and 71%, respectively and those in the non-surgical arm having rates of 92% and 46%.\nDisease specific survival for all patients at 2 years was 96% and at 5 years was 72% (Fig. ). There was a statistically significant difference in disease specific survival (DSS) between patients treated with surgery and RAI versus those treated by non-surgical means (p = 0.03). DSS for surgically treated patients at 2 and 5 years was 100% and for non-surgically treated patients was 92% and 46%, respectively. |
The patient was a 57-year-old male, with ESRD secondary to diabetes and hypertension, on hemodialysis for 20 months. He was evaluated to undergo living unrelated donor kidney transplant. The donor was a 54-year-old female with unremarkable past medical history. She was medically and surgically cleared after full assessment. Preoperative computed tomography (CT) angiography for the donor revealed a small complex cyst (Bosniak IIF), a short right renal vein (2 cm), and a right renal artery with an early bifurcation of an upper pole artery. Surgical approach deemed best by the living donor selection committee was to remove the right kidney with the complex cyst via laparoscopic approach.\nThe risks of surgery and potential complications were explained to both donor and recipient. Written informed consent was obtained prior to surgery from both patients.\nA standard hand-assisted laparoscopic living donor nephrectomy (LLDN) was performed to retrieve the right kidney and the right ovarian vein for reconstruction. The length of the donor renal vein was reduced by 10-15 mm due to the standard use of vascular stapler device to control the renal vein stump.\nStatus after procurement upon visualization in the back table is that the length of the donor renal vein was 3 mm. In order to elongate the vein, the donor ovarian vein was dissected and used for reconstruction. It was opened longitudinally, folded over, and anastomosed to the donor renal vein using end-to-end anastomosis with an 8-0 Prolene ().\nThe donor kidney had a renal artery with early bifurcation of an upper pole artery. After mobilization of the inferior vena cava as much as possible, the endovascular stapler was placed after the bifurcation from the renal artery and the length of the vessel was reduced by 10-15 mm. At the back table the donor kidney presented two individual arteries: the main renal artery and the short upper pole arterial branch. Consequently, the recipient IEA was used as an extension graft to perform an end-to-side anastomosis between the upper pole arterial branch and the main renal artery using 8-0 Prolene ().\nThe complex cyst was dissected all the way down into the calyces and substantial margin of healthy parenchyma was removed. Distal margin was sent to pathology to rule out malignancy. The calyces and the renal parenchyma were oversewed with 5-0 PDS and the edges of the defect with U-stitch of 4-0 PDS using pledget ().\nThe extended renal vein and the main renal artery were anastomosed end-to-side to the external iliac vein (EIV) and artery (EIA) respectively, using 6-0 Prolene ().\nWe performed an extravesical ureteroneocystostomy and a Jackson Pratt drain was placed. The warm ischemia time was 33 minutes. Postoperative course was uncomplicated and the patient has maintained adequate renal function with stable serum creatinine (1.32 mg/dl) at 5-month follow-up. The final pathology report and the intraoperative frozen section of the margin were negative for malignancy. |
A 76-year-old man with a past medical history of coronary artery disease, hypertension, and severe septal hypertrophy suspicious for hypertrophic cardiomyopathy, presented to the hospital with progressive symptoms of heart failure. He had been suffering from worsening dyspnea and lower extremity edema for several months. Lab work over the preceding months had shown deranged liver function tests, concerning for right heart failure. He was admitted for intravenous diuretic and inotrope therapy. At his cardiology clinic appointment prior to this hospital admission, an ECG showed typical atrial flutter and diffuse low voltage []. A right heart catheterization showed moderately elevated right greater than left filling pressures with a right atrial pressure 18 mmHg, pulmonary capillary wedge pressure 21 mmHg and a Fick cardiac index 1.6 liters/min/m2. The ScvO2 was 48%.\nOf note, a year prior to this admission, a transthoracic echocardiogram (TEE) had shown severe asymmetric septal hypertrophy (2.6 cm), suspicious for hypertrophic cardiomyopathy, but had not been further evaluated. A repeat TEE at the present admission showed a left ventricular ejection fraction (LVEF) of 35% as well as the aforementioned septal hypertrophy []. However, due to the significant low voltage on ECG, despite the significant LVH on echocardiogram and history of hypertension, a cardiac MRI was ordered to rule out infiltrative cardiomyopathy as opposed to hypertrophic cardiomyopathy.\nThe images showed normal left ventricular chamber size with severe, asymmetric left ventricular hypertrophy, primarily involving the septal wall with a maximum septal thickness of 27 mm []. The LVEF was measured at 40%. Also noted was diffuse, global late gadolinium enhancement of the left ventricular myocardium, consistent with a diagnosis of cardiac amyloidosis []. The only extracardiac clinical finding suggestive of systemic amyloidosis was carpal tunnel syndrome. Serum and urine electrophoresis did not detect a monoclonal protein, and serum free light chain ratio was low, which significantly lowered the suspicion for AL-amyloidosis. An abdominal fat pad biopsy was ordered which came back negative. Finally, an endomyocardial biopsy was performed which showed a pink amorphous interstitial infiltrate exhibiting apple green birefringence with Congo red stain, confirming cardiac amyloidosis. The subtype was likely wild-type ATTR or variant ATTR amyloidosis.\nIncidentally, the cardiac MRI also found a left upper lobe mass which, on further workup, was characterized as adenocarcinoma. No further differentiation of the subtype of ATTR amyloidosis was pursued, as this would not have changed management, especially in view of the poor prognosis portended by the adenocarcinoma. |
A 36-year-old male presented with the chief complaint of severe bilateral hip pain. He had previously been diagnosed with avascular necrosis of the femoral head. The etiology of the avascular necrosis was unknown. Approximately one year prior, he had undergone placement of bilateral Osteonecrosis Intervention Implant (Trabecular Metal; Zimmer Trabecular Metal Technology, Allendale, NJ, USA). The patient reported a brief period of symptomatic improvement following this procedure, but his hip pain ultimately progressed resulting in marked functional impairment. His past medical history included type II diabetes mellitus and hypertension. The preoperative physical examination was significant for an antalgic gait as well as pain with passive internal and external hip rotation bilaterally. Radiographs at the initial visit showed Ficat stage III bilateral hip avascular necrosis with femoral head collapse and the presence of porous tantalum implants within each femoral neck and head (). Serology was unremarkable and no hip aspiration was performed. A staged treatment plan was proposed consisting of Osteonecrosis Intervention Implant removal, intraoperative culture and sensitivity studies, and subsequent total hip arthroplasty once infection had been excluded.\nThe patient was taken to the operating room; the left Osteonecrosis Intervention Implant was carefully overreamed by 0.5 mm using a hand trephine. Once the tip of the Osteonecrosis Intervention Implant was reached, the implant was manually backed out of the femoral head and neck using the manufacturer's extraction device. Specimens of surrounding tissue and bone were sent for culture. A similar approach was used for removing the contralateral rod. More difficulty was encountered on the right side due to a greater amount of host bone ingrowth. The Osteonecrosis Intervention Implant was over-reamed by 1 mm to extract the device. Despite the surgeons' effort to remove all metallic fragments and remove as little host bone as necessary, the postoperative radiographs did reveal retained metallic fragments in both hips as well as significant bone loss from the femoral metaphysis and neck ().\nEarly the next morning, the patient noted severe pain in his right hip and proximal thigh associated with a loud “crack” when repositioning in bed. Radiographic evaluation of the right hip revealed an oblique fracture within the intertrochanteric and subtrochanteric regions of the proximal femur, originating from the defect left by the removed Osteonecrosis Intervention Implant (). The fracture was managed with open reduction and internal fixation using a proximal femoral locking plate and went on to union (). Arthroplasty was still considered a salvage option at this time because of the patient's age and concern for persistent occult infection. Intraoperative cultures were positive for Propionibacterium acnes. This infection was treated with 6 weeks of intravenous Ceftriaxone and 3 months of oral Doxycycline as recommended by the infectious disease consultant. No clinical signs of infection developed. |
A male infant was born at 32 weeks of gestation to a 35-year-old gravida II, para I by cesarean section because of maternal hepatitis C infection. Because of prior drug abuse, the mother had been on polamidone, a synthetic opioid used as part of the treatment of dependence on opioid drugs. Pregnancy was uncomplicated until the development of cervical incompetence. The APGAR scores were 6 after 1 min, 7 after 5 min, and 8 after 10 min, respectively. The infant developed respiratory distress and high oxygen demand immediately after birth and was admitted to the neonatal intensive care unit on nasopharyngeal CPAP support. On admission, the patient presented with hypercapnia with a pCO2 of 93 mmHg. Because of persisting respiratory distress and progression of CO2 retention, the decision was made to intubate and ventilate the infant. Direct laryngoscopy revealed a clearly visible glottic plane with unaffected vocal cord mobility, but it was impossible to advance tracheal tubes of various sizes beyond the vocal cords. After several attempts, mask ventilation became impossible as well. Intubation of the esophagus to ventilate the lungs via a potentially existing esophagotracheal fistula was also attempted but was unsuccessful.\nBecause of progressive bradycardia and poor oxygenation, cardiopulmonary resuscitation was started. Even emergency tracheotomy did not allow ventilation of the lungs since the infant lacked a visible tracheal lumen. After prolonged unsuccessful cardiopulmonary resuscitation, the infant finally died at the age of 3 h.\nPost-mortem examination revealed congenital high-grade stenosis of the trachea directly distal to the vocal cords over a distance of more than 1 cm with a lumen of less than 1 mm in diameter. Microscopically, the normal architecture of the proximal trachea was replaced by fibrous connective tissue (Fig. ). The severely stenotic lumen of the distal part of trachea was surrounded by compact cartilage (Fig. ). Macroscopically, the lungs appeared normal, whereas the microscopic examination revealed only poorly ventilated alveoli (Fig. ). There were no additional malformations. |
We report a rare case of IVC aneurysm in a 22-year old Afghan-Iranian male patient. The patient had a history of blunt abdominal trauma one week prior to his referral to the emergency department of our center. On his initial abdominal trauma, a complete physical examination and focused assessment with sonography for trauma (FAST) was done. The investigations were normal and the patient was discharged from the emergency department. The patient has had vague abdominal pain after his discharge.\nOn the referral of the patient to our center, we planned an abdominopelvic computed tomography (CT) scan with oral and IV contrast. The scan illustrated an IVC saccular aneurysm originating from right side of the IVC below the renal veins (). We assumed two possible etiologies. The aneurysm could incidentally and in another hand it could be related to the patient’s recent history of abdominal trauma. Magnetic resonance venography was also conducted and it also confirmed the diagnosis of a saccular type III IVC aneurysm ().\nWe planned open resection and repair of the aneurysm. A midline laparotomy was done. After thorough exploration of the abdominal and pelvic cavities, a right medial visceral rotation was conducted by mobilization of the right colon and a Kocher maneuver (The Cattel-Braasch Maneuver). The right kidney was left in situ. The entire sub-hepatic IVC was exposed. A saccular aneurysm with dimensions of 4*5 cm was found on exploration (). The aneurysm was located below the renal veins and the neck of the aneurysm was at the right side. The aneurysm was confined to the infrarenal IVC and there was not any associated venous anomaly. Thus, it was a type III saccular IVC aneurysm. A partial Satinsky clamp was applied posterior and left to the site of aneurysm origin on IVC and a longitudinal incision was done anterior to the neck of the aneurysm. Then, the entire aneurysm was resected. The neck of the aneurysm was closed with lateral venorrhaphy by running 6.0 polypropylene sutures ().\nThe patient had well recovery after the operation. Postoperative anticoagulation was administered by unfractionated heparin and warfarin. Warfarin anticoagulation was continued for three months to prevent venous thrombosis and probable pulmonary embolism. The patient’s follow-up did not reveal any morbidity. Postoperative CT scan was also conducted on seventh postoperative day. Postoperative appearance of IVC was normal (). |
An 83-year-old man underwent surgical excision of a malignant peripheral nerve sheath tumor (MPNST) at the right knee at another hospital and was then referred to our hospital for further chemotherapy. Three months after the start of chemotherapy, the patient complained of severe pelvic and low back pain. A PET CT was performed which showed multiple bony lesions with a variable (moderate to high) degree of metabolic activity raising suspicion that these were metastases. The lesions, however, had a very unusual appearance and evolution as seen on CT.\nInitially, the lesions presented as small rounded lytic lesions with a well-defined sclerotic margin (Figure ). They exhibited a rapid growth but the initial appearance of central lysis and surrounding sclerotic margin was preserved. Follow up PET CT scan was performed after seven weeks to evaluate the response to chemotherapy. It revealed a second concentric band (halo) around some of the lesions. This band (halo) had a ground-glass appearance and was surrounded by a second sclerotic rim. This rim was thinner and less sharply demarcated than the more central rim (Figure ). The multiple lesions were in different phases of evolution and as such had a different appearance at any given time.\nPresence of multiple lesions, a known primary tumor and moderate to high uptake of FDG tracer on PET scan (Figure ) suggested metastatic disease, despite the unusual appearance of the lesions.\nOther bony metastases were also evident on CT. Some were entirely sclerotic and a few had an ill-defined lytic appearance. Collapse of a vertebral body and fracture of the upper endplate of another vertebra due to metastases were also present.\nBone biopsy of a lesion with the above described doughnut appearance located in the left pubic bone was performed. A biopsy was obtained in the central lytic area and another biopsy in the peripheral halo (Figure ). Histological evaluation of both the samples showed infiltration of bone marrow by malignant spindle-shaped cells arranged neatly in bundles. Histological and immunohistochemical studies were compatible with metastases from a spindle cell tumor (in this case MPNST). |
A 60-year-old woman was diagnosed with endometrial cancer by curettage because of vaginal bleeding. In a preoperative examination, chest computed tomography (CT) showed a tumor that was located at the right anterior mediastinum. Total hysterectomy with double appendages was performed on 13 March 2019. A pathological examination showed that the tumor was 3.5 × 2.5 cm, it was highly differentiated endometrial adenocarcinoma, and it invaded the superficial myometrium (<1/2 of the myometrium). There was no metastasis in bilateral pelvic lymph nodes. Because the endometrial lesion only invaded the muscle layer, there was little possibility that the anterior mediastinal tumor was related to the endometrial cancer. The staging of endometrial cancer was pIA. The patient did not receive any adjuvant therapy and was told to seek further treatment for her mediastinal tumor.\nSix months after the radical operation, the patient was admitted to the Department of Thoracic Surgery without any discomfort. Preoperative thoracoabdominal CT showed a solitary 5.2- × 8.0- × 7.1-cm (W × L × H) tumor in the right mediastinum near to the pericardium (). A bone scan did not show bone metastasis. We explained the treatment plan for the patient and obtained consent for the treatment.\nOn 7 September 2019, the patient had an operation under video-assisted thoracoscopy. The tumor was in the right cardiophrenic angle, approximately 8 cm in maximum diameter, with a hard texture and clear boundary. The tumor adhered to the pericardium and lower lobe of the right lung. After separating the adhesions, we found that the tumor had grown in the diaphragm rather than in the mediastinum (). To completely remove the tumor, we performed thoracotomy through the right fifth intercostal space. The diaphragm was resected along with 1 cm from the margin of the tumor (). We then found that the tumor had grown into the abdominal cavity (). We stripped the tumor from the liver’s surface and removed the tumor with the invaded diaphragm (). After resection, the diaphragmatic defect was repaired by a Dacron patch. The chest was finely closed. The total bleeding volume was 500 mL. The patient could get out of bed on the second day. The drainage tube was removed on the third day.\nThe gross specimen was 8 × 6 × 6 cm, with a yellow hard capsule and a multicystic structure. A morphological examination suggested that the tumor was diaphragmatic metastasis from endometrial cancer by the following immunohistochemical results: estrogen receptor (ER), 20% + and progesterone receptor (PR), 30% + (). At 16 months after the total hysterectomy, there was no evidence of tumor relapse. |
A 38-year-old female with a four-week history of left level II cervical lymph node enlargement was referred to the Ear Nose and Throat (ENT) team for open neck biopsy. She had a history of multiple myeloma treated with chemotherapy and autologous bone marrow transplant two years prior to referral. The referring hematology team were concerned with a secondary malignancy.\nComplete head and neck examination revealed a palpable level 2 lymph node but was otherwise normal. A computer tomography of the neck was performed and showed an isolated two centimeter cervical lymph node correlating to clinical examination with no other significant findings. An ultrasound guided fine needle aspiration was performed on the node. Unfortunately, the cytology was reported as non-diagnostic and the patient was scheduled for open biopsy.\nPre operative platelet infusion was required as the patient had chronic thrombocytopenia. The anaesthetic team commenced general anaesthetic induction after the post infusion platelet count was confirmed to be 45 x 103/mL. Although higher platelet levels would have been preferred, this was not possible even with repeated platelet infusions. The ENT team anticipated a possible concern with hemostasis, but opted to proceed with the procedure due to the potential malignant underlying pathology. The expected duration of the procedure was confirmed to be thirty minutes by the operating ENT team.\nA two-centimeter incision was made followed by a deeper dissection. The anterior border of the sternocleidomastoid muscle (SCM) was retracted and a compressible mass was palpable medial to the muscle. Fascia over the palpable mass was skeletonized off to reveal a thin-walled air filled mass that was initially thought to be a possible laryngocoele. No other abnormal lymph nodes were palpable. At this stage, the operating surgeon realized that the “air filled mass” was actually the LMA and the pharyngeal wall had been dissected, resulting in a one centimeter iatrogenic pharyngeal tear. Prior to continuing with the procedure, the surgeon requested that the LMA be replaced with an ETT. The one centimeter pharyngeal tear was repaired and exploration showed no further damage. The patient was placed on total parenteral nutrition (TPN) and on intravenous broad spectrum antibiotics. A gastro-graffin swallow test done on day 10 post-operation showed no leakage and the patient was commenced on an oral diet without any issues. Post-operative imaging revealed that the original enlarged lymph node now measured less than 1cm. Intra operative events were discussed with the patient in full disclosure. The patient was subsequently referred back to the hematology team who opted to follow up the patient with serial imaging. |
A 60-year-old woman presented to our hospital on September 6, 2011, complaining of discomfort in the throat that she had been experiencing for about half a month. Past medical history was not a factor and there was no family history of thyroid cancer either. Physical examination revealed a palpable nodule in the right lobe of the swollen (grade I) thyroid. An ultrasound examination was performed immediately and an irregular hypoechoic nodule (measuring 31 × 15 mm) was detected arising from the lower pole and extending to the isthmus of the thyroid. The nodule was solid with blurry margins (Figure A) and it was difficult to distinguish from the trachea. Several coarse calcifications with heavy shadows were noticed inside. The thyroid capsule was also involved. Two swollen lymph nodes were found beside the right internal jugular vein (level V) measuring 17 × 13 mm and 14 × 10 mm, respectively. Computed tomography (CT) scans of the lungs were done but no positive findings were noted.\nBecause invasive thyroid carcinoma with cervical lymphatic metastases was assumed, surgical treatment was scheduled. Before the operation, the patient received a general checkup, including blood tests, echocardiographic assessment, abdominal and gynecologic ultrasound examinations, and CT scans of the head and abdomen. A heterogeneous mass was found in the right lower quadrant behind the uterus. The mass, measuring 115 × 92 × 94 mm, contained a large quantity of echoic fluid. Thick separations and two irregular solid components (measuring about 33 × 37 mm and 33 × 21 mm, respectively) were noticed inside. The ultrasound image is shown in Figure B. Abundant blood flow signals were detected in the solid components using color Doppler. The outside border of the mass was quite clear and smooth, but inside the mass closely adhered to and was difficult to distinguish from the right wall of the uterus. Neither ovary was involved. The serum cancer antigen 125 (CA125) level was slightly elevated at 70.58 U/ml (normal range, 0-35 U/ml). Carcinogenic embryonic antigen (CEA) (1.97 ng/ml) and CA19-9 (8.82 U/ml) levels were normal. Given the complex texture of the mass, an ovarian tumor, probably a cystadenocarcinoma, was suspected. After a thorough discussion with the relatives of the patient, a second operation following treatment of the thyroid nodule was scheduled.\nA total thyroidectomy and neck exploration were performed on September 17, 2011, under general anesthesia. Histological examination confirmed the diagnosis of a well-differentiated PTC (Figure A). Immunohistochemical examination demonstrated that the cells stained positively for thyroid transcription factor (TTF), cyclin D1, P27, epidermal growth factor receptor (EGFR) and Ki-67 (ratio 10%), and were negative for cytokeratin 19. Regional lymph node (3/4) metastases were confirmed. The patient had an uneventful postoperative course and was discharged on the 7th postoperative day.\nFifteen days later she returned to our department of gynecology for further treatment of the abdominal mass. An abdominal exploration was performed and a large pink mass was detected arising from the posterior wall of the uterus. The mass was soft and covered by the serosa of the uterus. Both ovaries were easily identified because they were separated from the uterus and had a normal appearance. No ascites was found in the pouch of Douglas. A total abdominal hysterectomy along with bilateral salpingo-oophorectomy, omentectomy and a periaortic lymph node biopsy were performed while taking into consideration the age of the patient. On gross sections, several cysts were revealed that were filled with brown fluid inside the large heterogeneous mass. The two irregular solid components shown on the sonograms were also seen on the inside wall of the mass, and both of them had a gray and granular surface. No evidence of malignant changes was detected in tissue slices taken from the ovaries.\nPostoperative pathological results were available five days later, and the diagnosis of a uterine metastasis stemming from the PTC was suspected (Figure B). To rule out a primary uterine carcinoma, an immunohistochemical assay on an appropriate panel of proteins was performed. The heterogeneous mass showed strong expression of TG, CK7, CAM5.2 and TTF. Results for CA125, EMA and CD10 were negative. Therefore, the diagnosis of metastatic PTC in the uterine corpus was established. No positive findings resulted from the harvested lymph nodes. The clinical course following surgery was indolent; the patient was discharged on October 14, 2011. Just prior to being discharged, she accepted to undergo a general radioactive iodine scan, and no abnormal hotspots were found. For unclear reasons, the patient declined further treatments and follow-up was suggested. Since then, she has returned three times for general checkups and once for a PET-CT examination, and thus far no signs of recurrence have been detected. |
A 17-year-old Pakistani girl presented to our hospital with the complaints of productive cough, vomiting and high grade fever for one week. A diagnosis of acute bronchopneumonia was made on the basis of physical examination (tachypnea, basilar pulmonary crackles, fever) and postero-anterior view (PA) chest X-ray (right apical cavitation). She was admitted to the hospital and treated with intravenous antibiotics. Her sputum cultures grew Pseudomonas aeruginosa and her antibiotics were modified accordingly.\nPast medical history of the patient was significant for recurrent respiratory tract infections since childhood; many of these episodes were associated with otitis media without perforation of the tympanic membrane. She had visited multiple doctors in the past few years and had been treated for tuberculosis up to three times in the past for a total of twenty four months in addition to receiving multiple courses of antibiotics. Her sputum smears and cultures for acid fast bacilli had not been positive. Her past history was negative for signs and symptoms of malabsorption, recurrent cutaneous infections or regular nasal drip. She had a history of primary amenorrhea at the time of initial presentation to us. She weighed 41 kg and her body mass index was 19.5 kg/m2 at that time.\nAfter an uneventful discharge from the hospital for the bronchopneumonia, the patient was followed up on an out-patient basis for further workup. In view of the history of recurrent infections, the possibility of bronchiectasis secondary to a variety of underlying pathologies such as post-infection, immunodeficiency syndromes or ciliary dyskinesia disorders was considered. Cystic fibrosis was also an important consideration. There was no history of consanguineous marriage in her parents. Computed tomography scan obtained at that time didn’t show features of bronchiectasis. Her sweat chloride test was done as part of the workup. Chemical analysis of a 58 mg sweat sample from the patient showed a result of 22 mmol/L. Her blood analysis for immunoglobulins were performed next, showing a deficiency of IgA, IgG subclass 2 and 4 while her IgE and IgM levels were all normal.\nWithin the next two years, she was readmitted multiple times for severe gastroenteritis, bronchopneumonia and maxillary sinusitis. In addition to several courses of intravenous and oral antibiotics, she also received intravenous immunoglobulins (IVIG) on four separate occasions to help her cope with crisis secondary to severe systemic infections. She showed a successful resolution of the crisis after administration of intravenous immunoglobulins. The possibility of regular monthly administration of IVIG was discussed with the patient but not opted for due to financial constraints.\nAbout five years after the initial diagnosis of primary immunoglobulin deficiency was made, she presented with localized cervical lymphadenopathy and a month’s history of fever. Her laboratory tests showed anemia (hemoglobin = 9.4 g/dl), leukocytosis (total leukocyte count = 14.7 × 109/L), thrombocytosis (platelets=441 × 109/L) and a Lactate Dehydrogenase (LDH) of 404 IU/L. In view of her immunodeficiency, we immediately biopsied the cervical lymph nodes. Histopathological examination of the lymph nodes showed scattered cells with vesicular nuclei, occasionally prominent nucleoli and mitosis in the background of histiocytes, plasma cells and lymphocytes. Based on positivity of LCA, CD 20, CD 3 and CD 30 along with a proliferative index of 30-40, a provisional diagnosis of intermediate grade non-Hodgkin’s lymphoma was made. Bone marrow biopsy confirmed these findings.\nA complete radiological work-up was done using CT with contrast. It showed no mediastinal lymphadenopathy, multiple enlarged lymph nodes in the neck at levels 1, 2, 3 and 4 bilaterally along with left supraclavicular lymph nodes, bilateral enhancing axillary lymph nodes, hepatosplenomegaly, multiple large enhancing notes in peri-pancreatic, aorto-caval, celiac axis, para-aortic and mesenteric locations. She is currently receiving chemotherapy for intermediate grade non-Hodgkin’s lymphoma.\nHer last chest X-ray showed development of fibrotic changes in right upper, middle and lower lung zones as well as bronchiectatic changes in the left basilar region. This most likely occurred in association with the multiple respiratory infections the patient has had in the past. |
A 65-year-old male with repeated episodes of forehead swelling was referred to our department. He had no history of sinus surgery but had undergone microsurgical aneurysm clipping at the age of 47. He did not have diabetes or other immunocompromising disorders. Axial computed tomography demonstrated an intracranial metal-density spot, indicating the site of previous clipping (). The posterior wall of the left frontal sinus was deformed as a result of the previous neurosurgery (). The left middle meatus was opacified with homogeneous density (). The left frontal sinus had heterogeneous opacity including a high-density spot. Three-dimensional reconstruction showed the postoperative change of the frontal bone ().\nSurgery was performed under general anesthesia. The mucosa of the frontal recess was edematous and obstructed the drainage pathway of the frontal sinus. Total removal of the anterior ethmoid cells revealed the frontal sinus ostium, but this ostium was too narrow to introduce any instrument into the frontal sinus (). Therefore, the EMLP was performed. The bilateral frontal sinuses were opened and the frontal beak and intersinus septum were drilled out. The left frontal sinus was filled with cheesy brown material (). Most of the material could be removed with curved instruments and flexible suction devices, but a portion of the material was located in the fissure of the bone. Even meticulous lavage with saline and extensive adjustment of flexible instruments failed to remove all of the remnant material (). Therefore, a small skin incision was made at the eyebrow and the frontal wall of the frontal sinus was trephined. Through this direct and close approach, the entire remainder of the material could be removed (). We preserved the mucosa of the frontal sinus to prevent postoperative scarring at the surgery site. The nasal cavity was packed with Sorbsan (calcium alginate) []. Histological examination revealed that the purulent material was formed by mixed bacterial colonies of Gram-positive coccus and an organism with long filament formation resembling actinomyces (). The symptom was resolved after the operation. The frontal sinus ostium was open and no infectious material was apparent at an outpatient visit. |
The patient was a 22-year-old male presenting a lump that had grown gradually under the skin of the right mastoid for 2 years. The patient denied any hearing loss or vertigo. There was no pain and no inflammation in the lump. The patient was healthy, with the exception of the lump. The patient had been assessed at the community hospital one year earlier and was treated with antibiotics, which were ineffective. The patient then came to Xijing Hospital (Xi’an, China) for further evaluation and treatment.\nOn examination, the two ears appeared normal. The patient had normal and symmetrical facial and cervical structures with no deformities of the pinnae or external auditory canals. The ear drums were normal and no air-fluid levels or bubbles were observed behind the drum. Impedance audiometry and audiogram were also normal. There were no significant findings in the remainder of the head and neck examination, with the exception of the tumor, which was initially considered a retroauricular lymph node.\nSurgical biopsy was performed with the patient under local anesthesia. When the skin flap was opened, a pink encapsulated mass was observed occupying the right mastoid surface. The mass extended from the subcutaneous tissue to the mastoid cortical plate without destruction of the mastoid bony wall. The tumor and mastoid cortical plate were umbilicated in the mastoid cavity. The mastoid cavity maintained integrity. The surface of the mass was granulated and a number of hairs were observed in the tumor when it was slivered. The diameter of the tumor was approximately 1.3×10−2 m (). Following complete removal of the tumor, the defect was reinforced with muscle and fascia.\nPathological analysis confirmed the diagnosis that the tumor was a dermoid cyst (). Two years after surgery there was no evidence of any recurrent tumor in the region of the right mastoid. All studies were performed under the consent of the patient and with approval from the Human Studies Committee of the Xijing Hospital of the Fourth Military Medical University. |
A 20-year-old asymptomatic female, G1P0, with a history of curettage, is presented to the Department of Gynecology and Obstetrics for termination of pregnancy. Her last menstrual period was 17 weeks and 2 days ago. Abdominal ultrasound revealed a clear gestational sac (GS), fetus with heartbeat and placenta previa. Abdominal ultrasound also showed the GS at a distance from the cavity, with a compressed myometrium between the two of them (). The patient received a MRI examination, showing an enlarged uterus of 13.0 cm ×11.7 cm × 7.9 cm, because of pieces of evidence. The MRI demonstrated a foetus with clear organs (), and compressed the lower uterine segment (). The GS was not connected with the uterine cavity and endometrium, but embedded into the myometrium in the right posterior wall of the uterine. A linear hypointensity of the junction zone was observed between the GS and the uterine cavity on T2weighted image ().\nThis result is likely to be placenta implantation as the myometrium cannot be separated from the placenta. The patient was at risk of the uterine rupture and life- threatening haemorrhage. Emergent management should be performed. Performing uterine artery embolization (UAE) and interventional therapy is inadvisable because of the obstructing myometrium between the cervix and placenta. Employing a surgical exploration of the abdomen was decided that was undertaken under temporary balloon occlusion of the abdominal aorta to reduce the loss of blood. The balloon was placed in the abdominal aorta between the opening of renal artery and iliac artery just before the operation. If the area of the focal damage was heavy, the subtotal hysterectomy or hysterectomy was needed. Otherwise, clinicians can perform excision by laparotomy and hysteroplasty. Given the age of patient, clinicians did their best to perform hysteroplasty instead of hysterectomy. The intramural ectopic GS in the second trimester was successfully excluded without life-threatening haemorrhage (). The patient had an uneventful postoperative course. Her β-hCG titre decreased to 1727 mIU ml–1 on the second day after operation, and then to 440.1 mIU ml–1 on the sixth day. She was discharged 6 days after surgery. |
A 20-year-old asymptomatic female, G1P0, with a history of curettage, is presented to the Department of Gynecology and Obstetrics for termination of pregnancy. Her last menstrual period was 17 weeks and 2 days ago. Abdominal ultrasound revealed a clear gestational sac (GS), fetus with heartbeat and placenta previa. Abdominal ultrasound also showed the GS at a distance from the cavity, with a compressed myometrium between the two of them (). The patient received a MRI examination, showing an enlarged uterus of 13.0 cm ×11.7 cm × 7.9 cm, because of pieces of evidence. The MRI demonstrated a foetus with clear organs (), and compressed the lower uterine segment (). The GS was not connected with the uterine cavity and endometrium, but embedded into the myometrium in the right posterior wall of the uterine. A linear hypointensity of the junction zone was observed between the GS and the uterine cavity on T2weighted image ().\nThis result is likely to be placenta implantation as the myometrium cannot be separated from the placenta. The patient was at risk of the uterine rupture and life- threatening haemorrhage. Emergent management should be performed. Performing uterine artery embolization (UAE) and interventional therapy is inadvisable because of the obstructing myometrium between the cervix and placenta. Employing a surgical exploration of the abdomen was decided that was undertaken under temporary balloon occlusion of the abdominal aorta to reduce the loss of blood. The balloon was placed in the abdominal aorta between the opening of renal artery and iliac artery just before the operation. If the area of the focal damage was heavy, the subtotal hysterectomy or hysterectomy was needed. Otherwise, clinicians can perform excision by laparotomy and hysteroplasty. Given the age of patient, clinicians did their best to perform hysteroplasty instead of hysterectomy. The intramural ectopic GS in the second trimester was successfully excluded without life-threatening haemorrhage (). The patient had an uneventful postoperative course. Her β-hCG titre decreased to 1727 mIU ml–1 on the second day after operation, and then to 440.1 mIU ml–1 on the sixth day. She was discharged 6 days after surgery. |
A systemically healthy 21-year-old male patient reported to the Department of Periodontology and Implantology with chief complaint of missing teeth in maxillary anterior region of jaw. Intraoral examination revealed missing 11 and 21. After clinical and radiographic evaluation replacement of missing teeth by one stage early loading implants along with reconstruction interimplant papilla using demineralized freeze dried bone allograft block fixed by titanium screw was planned. Prior to surgical procedure interimplant papilla measurements including measurement of papillary height using Grossberg criteria (2001) [] and measurement of papilla contour using Jemt index (1997) [] was carried out. Radiographic examination using intraoral periapical radiograph (IOPA) with long cone (XCP Rinn, Dentsply, New York, USA) paralleling technique was carried out to measure the vertical crestal bone level between implants, which was calculated from contact point after placement of restoration to the highest coronal point of crestal bone between implants [, ]. All measurements were recorded at baseline and again postoperatively at 3 months and at 6 months after final restoration.\nBriefly after induction of local anesthesia, horizontal palatal incision was made 2 mm away from the crest of the ridge using bard parker surgical blade number 15, without splitting the adjacent papillae, followed by vertical releasing incision on labial surface made extending to the vestibule. The papillae of the adjacent teeth were not included in the flap design (see ). A full thickness flap was raised labially and palatally exposing the underlying ridge of the implant site. Asurgical drill guide was used for the precise placement of the pilot drill. After pilot drill application, the implants site was prepared with the corresponding size of parallel drill. The implants were placed in the recipient site by means of an insertion device, and a torque driver set at 35 Ncm was used to evaluate primary stability of implant. The implant neck was positioned at the crestal bone level or slightly submerged. The healing abutment extension of the implant was placed in such a way that the head of the implant protrudes about 2 to 3 mm from the bone crest.\nBoth implants were placed in such way that the interimplant distance was ≥3 mm to ensure sufficient blood supply to the interimplant bone after placing papillary titanium screw with alloplastic bone block between the two implants. 1 mm diameter bur was used to drill midway between the two implants. A bone block allograft (freeze, dried, demineralized, irradiated bone allograft block) was hydrated with a sterile saline solution for at least 45 minutes before use. Then it was trimmed with a fissure bur in a high speed hand piece with a copious saline solution to remove residual bone particles. The prepared block allograft was predrilled to accommodate (1.5 mm × 8 mm) titanium screw (see ). Fixation screws were placed in a prepared block allograft with an oblique fashion so as not to induce stress fracture in the allograft. After stabilization of allograft block between the two implants, the buccal flap was positioned around the implants and sutured to the palatal flap. Complete tension free soft tissue closure was achieved and a provisional restoration was cemented. Patient received antibiotics (Amoxicillin PO 500 mg t.i.d.) and analgesics (Ibuprofen PO 400 mg, t.i.d.) after surgery were continued for at least 5 days postsurgically. Patient was instructed not to brush in the treated area but to rinse 3 times per day for 1 minute with chlorhexidine digluconate of 0.12% until suture removal. Sutures were removed within 7 to 10 days of implantation and, during the same visit, the final impression was registered using high-viscosity vinyl polysiloxane and within 15 days a definitive customized abutment with gingival emergence was established by permanent restoration using a metal-ceramic restoration. The patients were recalled at 3 months and 6 months following permanent restoration. At each recall visit clinical and radiographic measurements were recorded preoperatively and were repeated at 3 months and 6 months after final restoration.\nIn the present case report at 6-month followup, papillary height was 2.00 mm at baseline, which was increased to 3.8 mm at 3 months after papilla reconstruction procedure with a gain of 1.8 mm. At 6 months, it was further increased to 4 mm with gain of 2 mm. At 6-month measurement of papilla contour using Jemt index (1997) [] was score 3 which indicates complete reconstruction of interimplant papilla. Radiographically the distance between contact point after placement of restoration to the highest coronal point of crestal bone between implants was less than 5 mm. |
A 63-year-old man attended our hospital with heavy abdominal pain in January 2016. The CT revealed a mass with elevation of tissue fascia and free air near the cecum (). Accordingly, the patient was diagnosed with perforation of the cecal diverticulum, and open drainage and construction of ileostomy was performed. After 3 months, the patient received right hemicolectomy and closure of the ileostomy. We did not detect the mass from the resected specimen (), and pathological examination revealed no malignancy.\nThe patient experienced a palpable mass in the left abdomen from May 2017 and attended our department in December 2017. The enhanced CT revealed a 38-mm mass, resembling a dyeing ring in the left rectus abdominis muscle. The mass was located near the scar of ileostomy closure (). The size of hernia orifice from the epigastrium to the pubic bones was approximately 20 cm.\nAfter 4 months, the MRI revealed a 40-mm mass in the left rectus abdominis muscle. T2 weighted image showed a low intensity area similar to muscle (), and the tumor was uniformly enhanced ().\nWe performed endoscopic ultrasound (EUS) and fine needle aspiration of the mass and reached the pathological diagnosis of desmoid tumor. Because the patient had pain at the mass and at the point of abdominal incisional hernia, we planned resection of the abdominal desmoid tumor and repair of incisional hernia.\nWe performed a midline incision similar to the previous scar and cut a spindle shape around the mass at 2-cm margin from the mass (). There was no exposure of the tumor at the abdominal side. We used the Harmonic ScalpelTM (Ethicon Endo-Surgery, Cincinnati) to cut the muscle to prevent bleeding (). Next, we removed the rectus abdominis muscle from the left lateral end. The abdominal wall defect measured 10 cm × 10 cm (). The hernia was repaired with a simple closure using anterior layer of rectus sheath and the defect was closed by suturing the right anterior layer of rectus sheath to the left aponeurosis of external oblique muscle (). Considering the risk of recurrence of abdominal wall hernia at the defect site, we decided to repair the defect with autologous fascia. We used a fascia lata patch measuring 15 cm × 5 cm () to repair the defect in the left abdomen ( and –).\nThe size of the mass was 45 mm × 45 mm, and it was a solid tumor. The macroscopic view of the resected tumor revealed its whitish color (). Pathological analysis revealed the growth of fibroblasts, defect of the nuclear heteromorphism, and presence of abundant collagen fibers between fibroblasts (). Additionally, a nuclear-positive image of β-catenin was observed on immunostaining (). The pathological diagnosis was desmoid-type fibromatosis. No recurrence of incisional hernia and desmoid tumor was detected 22 months after surgery. |
In this paper, we describe a 40-years-old woman with a local lesion of the right side of the maxilla. Her history reveals that she was in relatively good health until the age of 20, when she started to complain of frequent headaches and vomiting up to three times a week. Clinical evaluation revealed arterial hypertension and renal failure. A kidney biopsy was not diagnostic. In February 1989, she was started on chronic haemodialysis three times weekly. In 1991, she received a kidney transplant from a cadaveric donor. In 1997, she developed a non-Hodgkin lymphoma. Chemotherapy for one year lead to a complete remission of the lymphoma. In March 1999, she developed aseptic necrosis of the femoral head which was treated surgically. In June 1999, as she developed progressive deterioration of the transplanted kidney function, haemodialysis three times weekly was restarted. In October 1999, the transplanted kidney was removed and during surgery, she developed thrombosis of the left central retinal vein. Laboratory investigations in December 2000 revealed elevated serum parathyroid hormone (PTH) level of 282 pg/mL (normal range 14–72 pg/mL). At the same time, she noted a swelling in the right side of the maxilla. A radiograph of the maxilla showed an area of bone rarefaction ().\nFive months later, a computed tomography and an electronic rebuilding dental scan showed a hypodense neo-formation with heterogeneous borders and erosions. Bone structure was interrupted both laterally and medially on the lingual side. A lesion with a maximum diameter of about 4 cm involved the roots and peri-apical tissue of teeth 4.4, 4.5, 4.6, and part of 4.7. Two other similar lesions each about 1 cm in diameter were seen in the chin region and the right side of the maxilla ().\nSince radiographs showed a radiographic image compatible with more than one possible lesion and hence were not sufficient to provide a definitive diagnosis, a biopsy of the lesions was performed. Histological examination revealed giant bone cells. This together with the laboratory evidence for secondary hyperparathyroidism, the diagnosis of a brown tumour was made. The patient was treated for secondary hyperparathyroidism with vitamin D, calcium, bisphosphonates, phosphate binders, and reduced phosphate intake. In July 2001, despite the above treatment, she continued to have severe and worsening hyperparathyroidism with PTH levels of 1700 pg/mL, with serum calcium of 10.5 mg/dl (normal range 8.6–10.2 mg/dl), phosphate of 5.3 mg/dl (normal range 2.7–4.5 mg/dl), and alkaline phosphatase of 319 IU/L (normal range 40–120 IU/L). At the same time, the bony lesions increased in size but with signs of modest recalcification. In October 2001, the refractoriness to medical therapy was evident and a parathyroidectomy became necessary. Surgical treatment consisted of removal of all 4 parathyroids and implantation of 1/3 of the inferior parathyroid in the right forearm muscle. This was followed by a dramatic fall in serum PTH levels and normalisation of serum calcium and phosphate levels resulting in regression of the brown tumour. February 2002, the lesions were partially calcified and in June 2003, computed tomography and dental scan showed a normal bone structure in the area that was previously involved with the brown tumour but with residual hyperostosis. In December 2003, alveolar bone was thickened both on the external and on the medial side. Thereafter, annual computed tomography and dental scan and monthly blood tests were carried out, and on the last examination in February 2007, there was thickened alveolar bone of the right mandibular arch with a profile deformity but without a cortical gap. There was a regular morphology of the mandibular canal without signs of infiltration ().\nIn summary, this case shows that it is possible to avoid surgical of a large brown tumour even though it compromised the physical appearance of the patient and caused dysfunction of the masticatory apparatus. The surgical resolution of hyperparathyroidism was enough to correct the calcium-phosphate-PTH imbalance and to result in regression of the jaw lesions. |
The patient was a 23-year-old woman who visited a dentoalveolar trauma clinic six months after a fall. The patient remembered being referred to a hospital and receiving initial care after the accident. An intraoral examination revealed the presence of rigid splint involving the maxillary incisors and canines. The left maxillary lateral incisor had a temporary crown\nmade of composite resin without an intra-canal retainer; the other three maxillary incisors had crowns that were otherwise healthy. Radiographic examination revealed complete root formation in the involved teeth, a root fracture in the cervical third of the right maxillary lateral incisor (), and a fracture in the middle third of the maxillary central incisors (). All teeth had positive response to pulp sensitivity tests.\nDuring a second visit, the patient underwent endodontic treatment in the right maxillary lateral incisor to allow for the installation of a root canal seal and for prosthetic rehabilitation of the crown. During the first six months, monthly radiographic and clinical controls of all the involved teeth were performed. Dental splint was maintained for 12 months because of the right maxillary lateral incisor fracture.\nAt the time of splint removal, the teeth did not show increased mobility. The maxillary central incisors and right maxillary lateral incisor showed positive responses to pulp sensitivity tests. Annual controls were performed until 2009. Because the patient did not attend the scheduled controls in the following years, the subsequent follow-up was possible only in February 2016. Clinical examination revealed normal soft and hard tissues, and a positive response to pulp sensitivity tests. Periapical radiographs () and a computed tomography (CT) examination were conducted for evaluating the involved teeth.\nThe tomographic image suggested repairing of the maxillary central incisors with deposition of mineralized tissue between the fragments (). The right maxillary central incisor appeared to have multiple fractures with a slight reduction in the root canal volume, especially in the apical fragment, in addition to rounding of the fragments. |
A 30-year-old male presented with severe pain and inability to move his right knee following injury in a RTA. The mechanism of injury was a direct force on his flexed knee while riding pillion on a bike, followed by a twisting valgus knee injury with foot landing on the ground. He was brought to the emergency and evaluated for the injury. On clinical examination, abrasions were noted over the anterior aspect of the knee ( and ). A subtle dip was noted on the patellar tendon region and patient was unable to move his knee. Due to severe pain, further clinical examination was not possible. Radiographs revealed patella Alta ( and ). An urgent MRI was done to evaluate all the injuries of the knee. MRI confirmed the presence of patellar tendon injury along with ACL tear and Grade 1 medial collateral ligament sprain ( and ).\nThe patient was planned for immediate extensor mechanism repair, followed by ACL reconstruction at a later date. Under anesthesia, Lachman’s test and anterior Drawer’s test were found to be positive. A longitudinal incision skirting around the abrasion was made over the right knee. Intraoperatively, the patellar tendon and extensor retinaculum were found torn (). The patellar tendon was torn at its mid portion and was attached end to end using prolene 1-0 (). The extensor retinaculum was repaired using Vicryl 1-0. Following closure, the limb was immobilized in a slab. A cylindrical cast was applied after suture removal and kept for a further period of 3 weeks, during which the patient was taught isometric quadriceps exercises. The cast was removed at 5 weeks post repair and knee were mobilized using continuous passive motion and active assisted exercises. Once active full extension with flexion up to 120° was attained by the patient, arthroscopic ACL reconstruction using hamstring graft was done at 6 weeks. The ipsilateral hamstrings graft was harvested and prepared ( and ). The tunnels were drilled using the transtibial technique. The femoral side was fixed using cross pins and tibial side using bioabsorbable screw. Postoperatively patient underwent routine post ACL reconstruction physiotherapy followed at our institute. In the immediate post-operative period, a hinged brace was provided with flexion permitted up to 90° and closed chain exercises were initiated. This was followed by partial weight bearing at 4 weeks, and open chain exercises were started after 3 months. At 6 months post injury, the patient has regained full function of the knee including complete active extension and flexion up to 120°, with full weight bearing and stability ( and ). |
A 32-year-old female patient presenting with vomiting and diarrhea associated with increasingly strong abdominal pain in the upper left quadrant in the last 24 hours was admitted to the emergency department. The patient reported constant abdominal pain and vomiting with diarrhea (four episodes/day) for 2 weeks. An initial conservative approach with oral analgesics, antibiotics and proton pump inhibitors by the general practitioner did not lead to any improvement in the patient’s condition. The patient’s last menstrual period was 2 weeks before and was unremarkable. The patient never had any such symptoms before.\nIn the anamnesis, an undefined open umbilical operation in childhood, probably due to an omphalocele, was reported. The rest of the anamnesis was unremarkable and without inflammatory bowel disease or colorectal cancer history in her family. No colonoscopy had been performed to date. The measured blood pressure and heart frequency were normal and the patient had no fever.\nThe clinical examination showed a slightly deteriorated condition and a painful distended abdomen with tenderness in all quadrants. There was no rebound pain or palpable masses. Blood tests showed a normal white cell count with a very slight elevation in C-reactive protein (7 mg/l). In the emergency department, the first diagnostic approach with ultrasonography revealed a circular concentric layer, with a maximum diameter of 4–5 cm, highly suspicious of invagination in the left upper quadrant (). The second step was to perform a CT scan with consequent confirmation of the diagnosis. The exam revealed a long ileocolonic intussusception of the ascending colon into the transverse colon, with possible involvement of the terminal ileum; a suspicious strangulation of a superior mesenteric artery branch was postulated ( and ).\nThe indication for emergency laparotomy followed. The inspection showed a massive ileocolic intussusception into the left side of the transverse colon. A reposition was not possible due to adhesions and bowel edema. An oncological extended right hemicolectomy was performed because of the unclear reason for intussusception (). The operation was successful with primary ileocolic anastomosis. The postoperative course was uneventful. Passage of gas occurred on the second postoperative day and passage of stool occurred on postoperative day four. Nine days after the operation, our patient was discharged from the hospital in very good condition.\nThe histological exam showed an ileocolic intussusception because of extended perimural adhesions in the range of severe local extended endometriosis with several foci in the lamina propria of the colon layer and in the fibrotic zone ().\nBecause of dysmenorrhea in the history, no further diagnostic was recommended, and contraception therapy was begun. At the 3-month follow-up, the patient was symptom-free. |
A 57-year-old man with background history of diabetes mellitus, hypertension, hyperlipidemia, and bilateral occipital lobe infarct 5 years ago presented with sudden bilateral loss of vision for a 3-day duration associated with slurred speech. It was preceded by occipital headache.\nHe had history of bilateral occipital lobe infarcts five years ago with both eyes (OU) vision of only perception to light (PL). There was no neurological deficit apart from slurring of speech. He had no symptoms of denial of visual deficit at that time. CT brain showed multiple infarcts in both parietooccipital regions. One month after being discharged from hospital, his vision still maintained at PL OU and it slowly recovered. He was capable of watching television and reading with glasses 6 months after the stroke. Since then, the vision remained stable until the current events. His vision prior to the first episode of stroke was clear without glasses. He was not compliant to his medication.\nOn arrival in emergency unit during this episode, he was fully conscious with blood pressure of 124/83 mmHg. He was orientated to time, place, and person. Apart from slurred speech, he had normal power in all four limbs and intact sensation. He had severe visual impairment with hand motion OU. Pupils were reactive; corneal reflexes were intact with normal fundoscopic findings. There was no blink response. A CT brain () showed bilateral occipital lobe infarcts with dilated left lateral ventricles. In the ward, he claimed he can see but was unable to name the objects shown to him. He claimed he can see the floor but was not sure about the colour. He walked with support and claimed his body was weak rather than loss of vision. Visual evoked potential (VEP) was done and revealed absence of input potential. He was started on secondary stroke prevention medications. One week later, he was discharged with OU vision of hand motion. Upon discharge, he did not deny visual deficit anymore. |
The patient, C. C., female, aged 45, came to our attention requiring to replace old fillings at the level of the front teeth of the upper jaw and reporting as further blemish the excessive length of teeth with gingival recessions (). In a preliminary phase, all the pictures of the case were carried out and, moreover, bitewing radiographs were recorded to assess the presence of any secondary caries (). After the clinical-radiographic examination, it was decided to perform the surgery for root coverage in correspondence to the elements between 13 and 23 and to place six prosthetic veneers over the same dental elements once the soft tissue healing was completed. Complete scaling and root planning were performed and oral hygiene instructions were given 4 weeks prior to surgery.\nThe first question which the clinician has faced concerned the choice of the surgical technique for root coverage. In order to evaluate the better surgical technique suited to the situation, we considered the local anatomical conditions relating to teeth and soft tissues. With regard to the gum tissues, there was evidence that the apical-coronal dimension and thickness of keratinized tissue placed apically to recessions were adequate and, in addition, the vestibule appeared deep enough. Considering the teeth, no deep abrasions were present and no root was displaced buccally. Based on these clinical evaluations, it was decided for the execution of a coronally advanced flap. The choice of this surgical treatment option allows for a greater ease of operation compared to other techniques and a good tolerability by the patient with little postoperative discomfort and, in addition, provides excellent results in terms of root coverage and aesthetics.\nFor the proposed clinical case, in detail, a coronally advanced flap was done for the treatment of multiple gingival recessions of incisors and canines of the maxillary arch, as proposed by Zucchelli and De Sanctis (2000) []. Under local anesthesia (2% mepivacaine with adrenaline 1 : 100.000) the flap was designed with paramarginal oblique incisions in the interproximal areas that were joined together with intrasulcular incision in order to draw the surgical papilla of the flap (). The paramarginal incisions were carried out so that all the incisions converged towards the axis of rotation, passing through the center of the interincisive papilla. The flap was elevated according to a mixed thicknesses: partial under the surgical papillas, total apically to the recessions in order to expose 3 mm of buccal bone, and again partial to cut the muscle fibers (Figures , , and ). The interincisive papilla was not elevated but “tunnelled,” releasing the muscle insertions below the median frenulum (). Once the flap was prepared, the root surfaces were conditioned mechanically with scaler and curettes and anatomical papillae were deepithelialized with a small blade. The flap was coronally advanced to cover the root surface () and subsequently sutured without any tension at the level of the cement-enamel junction (CEJ) (). The flap was fixed with a nonresorbable suture material and a mattress sling suturing technique was done. In addition, a U-suture was performed in the alveolar mucosa, in order to reduce the tension of the lips on the edge portion of the flap. The horizontal borders of the suture were included in the surgical area. No periodontal dressing was used.\nAll postoperative instructions were provided to the patient. In particular, postoperative care included 1 gr amoxicillin and clavulanic acid every 12 hours for 6 days, ibuprofen 400 mg as needed for pain control, and chlorhexidine gluconate 0.2% twice a day for the first three weeks. Sutures were removed after 10 days. No brushing or flossing was allowed in the operation area for three weeks after surgery. Healing was checked every week for the first month and then every 30 days (Figures and ).\nNine months after surgery, once the complete healing of the soft gum tissue was obtained, the clinician has faced the second clinical question regarding the type of dental rehabilitation. The possible treatment options allow for the execution of direct composite restorations or indirect prosthetic restorations such as veneers or crowns. In this case, the placement of prosthetic crowns would have been a promising treatment aesthetically but overly invasive. For these reasons, on the basis of the clinical evaluation and in order to obtain a successful result in terms of aesthetics and for the long period, it was decided to perform prosthetic veneers. Dental elements were, therefore, prepared and six veneers made of lithium disilicate were adhesively bonded to the surface of the teeth (Figures , , , , and ). |
A 70-year-old female with no significant past medical history, Eastern Cooperative Oncology Group performance status 0 and no prior incidence of hypersensitivity reactions, was incidentally found to have a small nodule in the proximal right posterolateral vaginal wall after presenting with post-menopausal bleeding. Subsequent biopsy demonstrated a 9 mm invasive melanoma and the patient underwent wide local excision with confirmed negative margins. 4 months later, she developed a right periclitoral mass. Positron emission tomography/CT scan at that time demonstrated focal uptake in this area but no regional/distant metastases (). Excision demonstrated a large submucosal mass of atypical epithelioid cells with evidence of melanin synthesis, consistent with malignant melanoma. Breslow depth was 9 mm (3 mitoses/mm2) with a positive deep margin, and there was no evidence of lymphatic invasion. Her case was presented at a multidisciplinary tumour board and either additional surgery or radiation therapy was recommended to the patient. Owing to the significant morbidity anticipated with additional surgery, the patient opted for radiation therapy. Given the high risk of both local and regional/distant failure, concurrent chemotherapy was proposed. Owing to the historically poor response rates with standard chemotherapy, an immune pathway targeted agent was considered. This non-standard approach was actually initially proposed by the patient. After being thoroughly explained the current standard of care, in addition to the pros and cons of pursuing concurrent radiation and immunotherapy, the patient chose to proceed with combination immunotherapy and radiation treatment. Several studies have demonstrated that local radiotherapy primes and/or enhances an immune response through cytotoxic T lymphocytes. Concurrent immunotherapy may then further enhance the activity and/or duration of the downstream immune response. Given the historically low efficacy of our current treatment paradigms in this disease, as well as the preclinical/clinical rationale to combine radiation and immunotherapy, a strategy of pursuing a combination of ipilimumab with radiation was felt to be reasonable.\nRadiation was planned with intensity-modulated radiation therapy (IMRT) to the vulva and vagina (no elective nodal radiation to the groin) to an initial dose of 45 Gy (1.8 Gy/fraction over 25 fractions), and was to be followed by an electron boost to a total dose of 63 Gy (1.8 Gy/fraction over 10 fractions) at the site of the positive margin (). A thermoluminescent dosimeter was placed on the vulva at the start of treatment to measure the skin dose and read as 1.78 Gy [95% confidence interval (1.66–1.90)], confirming that the planned dose was accurate on the skin. The patient received her first cycle of ipilimumab (3 mg kg−1) 7 days after the start of radiation, and the second cycle was delivered 3 weeks later when the patient was at a dose of 36 Gy. Around this time (3 weeks post ipilimumab cycle 1), she began to develop non-painful erythema in the vulvar and perianal area, as well as a pruritic, grade 2 cutaneous eruption that morphologically showed distinct erythematous papules that coalesced into thin plaques over the upper arms, chest, back and face/ears (all toxicities were graded using Common Terminology Criteria for Adverse Events version 4.03). She did not experience any fevers or other systemic symptoms. By 48.6 Gy dose (10 days post ipilimumab cycle 2), the patient developed a grade 3 skin reaction () that was characterized as a moist desquamation with significant oedema, erythema and pain in the vaginal/vulvar/perianal region and was restricted to the radiation field (). A timeline of these events is illustrated in . After proper consent, a 4 mm punch biopsy of the affected skin was performed and histopathological examination demonstrated spongiotic and interface dermatitis with a perivascular inflammatory infiltrate consisting of numerous eosinophils, consistent with a fixed drug eruption.\nRadiation and ipilimumab were held given the severity of the moist desquamation, and the patient was referred to a dermatologist for evaluation of the ipilimumab-associated cutaneous eruption. It is noted that the patient had no existing risk factors or hypersensitivities predisposing her to an enhanced skin toxicity. The patient was started on 0.1% topical triamcinolone cream along with a methylprednisone dosepak. Given only mild improvement after 1 week, she was started on prednisone 60 mg daily (tapered over 7 days) with oral diphenhydramine for pruritus as needed. This resulted in significant improvement in her cutaneous eruption and pruritus. She then received a third cycle of ipilimumab (4 weeks after cycle 2) and resumed her periclitoral radiation boost without further issues after a 1-month break from radiation treatment. She went on to receive a fourth cycle of ipilimumab after completion of radiation without any complications.\nAt follow-up 8 months post completion of radiotherapy, she had complete resolution of the in-field toxicity and improvement of her ipilimumab-associated cutaneous eruption (). Clinical examination and positron emission tomography/CT imaging 10 months after completion demonstrated no evidence of disease recurrence. Most recently, at her 15-month follow-up, she remains disease- and symptom-free ().\nAt each follow-up visit, the patient has repeatedly expressed satisfaction with her decision to pursue immunotherapy rather than standard chemotherapy to complement her radiation treatment. She admits the initial side effects and intensity of the treatment course were difficult, although she is very pleased with the outcomes and would make the same decision if asked to do so again. |
Our hepatobiliary surgery outpatient department accepted a 66-year-old woman, who presented with a year-long history of aggravating abdominal discomfort that was mainly associated with distension and symptoms such as epigastric fullness, eructation, decrease of food intake and early satiety. She did not report any loss in body weight. A physical examination revealed a palpable mass in the upper abdomen, extending from the right hypochondrial region to the left hypochondrial region, with ordinary consistency and a smooth surface. Her liver and spleen were not palpable distance from the costal margin, and were not tender on palpation. All routine blood and biochemical markers were normal. The levels of tumor markers assessed, including carbohydrate antigen 125 and carcinoembryonic antigen, were within the normal ranges.\nAn upper gastrointestinal barium series revealed the antrum, fundus and body of her stomach, as well as the jejunum, were prominently compressed and infraplacement (Figure ). However, the gastroscope examination did not show any abnormality. An abdominal ultrasound revealed a sharply defined mass with an intact capsule and opaque dark areas of fluid detected in the middle region, suggestive of a huge cystic solid myoma. We were not able to identify any connection between the adjacent organs and the mass because the tumor was too large to obtain a clear view.\nEnhanced abdominal computed tomography (CT) scans demonstrated a deformed liver of abnormal size and structure, and a large low-density region featuring a well-circumscribed border that overlay the entire left liver and partially overlay the right liver. The tumor contained inhomogeneous cystic components mixed with solid elements (Figure ). In the arterial phase, complete filling of the tumor with contrast material was never observed; patchy enhancement indicated possible tumor necrosis in the low-attenuation areas. The area of lower density also did not enhance completely on delayed scans. There were no signs of lymphadenopathy or liver or pancreatic disease.\nCT angiography (CTA) showed that the tumor was supplied mainly by branches from the left and right gastric arteries. The common hepatic artery, left hepatic artery, splenic artery, superior mesenteric artery and left renal artery next to the tumor were compressed. The portal vein, inferior vena cava, left hepatic vein and superior mesenteric vein were also compressed (Figure ).\nAn exploratory laparotomy under general anesthesia was performed and revealed a huge, thick-walled tumor that almost filled the abdominal cavity. It appeared to protrude from the anterior wall of the fundus of her stomach, on the lesser curvature. The tumor was well-demarcated from the surrounding organs (liver, spleen, transverse colon), which were displaced but not involved with the tumor. Furthermore, the tumor had no relationship with the adjacent major vessels. In order to completely extirpate the tumor, the proximal stomach and lower esophagus were segmentally resected, and then an esophagogastrostomy was performed. No evidence of liver metastasis, lymphadenopathy or peritoneal metastasis was found (Figure ). The tumor measured approximately 21 × 34 × 11 cm in diameter and weighed 5.5 kg.\nHistopathological examination of the resected specimen revealed a stromal cell neoplasm with necrotic and hemorrhagic areas, as well as a proliferation of spindle cells with a mitotic count of less than five mitoses per 50 high-power fields.\nImmunohistochemical analysis revealed the specimen to be CD177 positive, CD34 positive, Dog-1positive, Ki-67 positive (1 %) and S-100 negative. The postoperative course was uneventful and treatment with imatinib mesilate was initiated immediately. Our patient was discharged 20 days after surgery and advised to attend follow-up CT scans of her abdomen in regular three to six months intervals. |
A 23-year-old Saudi male presented to the neurology clinic complaining of involuntary left upper limb movements of 6 months' duration. The movements lasted for few seconds and stopped when the arm was held by the other hand. The movements were described as supination and pronation and occurred only when the patient was experiencing stressful situations or getting insufficient sleep. No other factors appeared to induce the attack, which was limited to the left arm. There was no change in the level of consciousness during or before the attacks and there were no other associated symptoms.\nThe patient's medical history revealed tonic-clonic seizures at 9 years of age, which subsided by the age of 14 years. The seizures occurred during sleep, and there were approximately 6–8 episodes over those 5 years. The patient had received carbamazepine 200 mg per day and had discontinued the medication when the attacks subsided. He had no other significant medical history. The patient's birth history revealed that he was delivered at term by cesarean section, and he was placed in an incubator for 17 days after birth for unknown reasons. No further details were provided regarding his birth history. His surgical history was significant in terms of open reduction and internal fixation of a pathological fracture of the tibia and fibula after a minor sports injury; a nonossifying fibroma was the underlying cause of the pathological fracture. His family history was noncontributory. His general and systemic physical examinations were normal.\nThe patient's neurological assessment was significant only for hyperreflexia of the left upper limb (+3 for left brachioradialis, biceps, and triceps reflexes). The results of his motor, sensory, and cerebellar examinations were normal. The Babinski sign was negative. No abnormalities were found by hematological and biochemistry blood tests ().\nAn EEG recording showed background activity of a well-regulated alpha rhythm, at a frequency of 9 Hz, with maximum amplitude of 70 mcv (referential). Photic stimulation induced a good driving response. Hyperventilation added no further information. Interictal EEG was normal. These findings were consistent with a normal EEG.\nBrain MRI with contrast showed corticosubcortical cystic encephalomalacic changes, centered on the right superior frontal gyrus and contacting the right lateral ventricle frontal horn (Figures and ). These changes included a dominant large unilocular cystic component, measuring around 4 × 3 × 2.3 cm, which followed the CSF signal on all sequences and lacked overt hemosiderin staining. Thinning of the overlying cortical mantle was present, with mild scalloping of the adjacent calvarial inner table. The dominant cyst was surrounded by smaller cystic changes ().\nThese findings were suggestive of a remote insult, probably from a posttraumatic or perinatal ischemic injury, and were consistent with a porencephalic cyst.\nThe final diagnosis was a simple partial motor seizure caused by acquired porencephaly. Lifestyle modifications and levetiracetam 1000 mg twice daily were prescribed. A follow-up consultation was scheduled for 1 month later. The patient changed his lifestyle by avoiding major stressors and ensuring that he obtained sufficient sleep. No additional seizures were experienced while he was on the above treatment plan. |
A 46-year-old female presented in 2017 with symptomatic right leg pain and aesthetic complaints relating to the right calf. Medical history showed a previous head trauma (car accident) with brain hematoma drainage and a saphenous vein operation. Physical examination revealed edema in the perimalleolar area and painful varicose veins, in the right calf (with considerable aesthetic impact) and left calf (with minor aesthetic impact). Venous scores at the first visit to our clinic were the following: Venous clinical severity score VCSS 10 and Aberdeen quality of life questionnaire 27.7.\nDuplex examination conducted before the original venous operation (which had been performed in a different clinic in January 2016) had shown reflux in the left great saphenous vein and significant reflux in the right small saphenous vein. However, the operation actually performed was stripping of the right great saphenous vein. Both the left great saphenous vein and the right small saphenous veins were left in place untreated. After this procedure, symptoms had exacerbated progressively, and the aesthetics of the leg had deteriorated progressively.\nPreoperative evaluation was normal. We performed a complete duplex scan, according to our routine, as published elsewhere. The patient had type 1b+2a shunt in the right leg and 4+2d shunt in the left leg. We suggested operating to treat the small saphenous vein in the right leg and the great saphenous vein in the left leg. We treated the patient using the CHIVA technique to preserve the remaining saphenous veins.\nWe performed the CHIVA procedure on both legs during the same operation. Local anesthesia was provided with a solution containing 10 mg/mL 20 mL of ropivacaine and 2% lidocaine, using 20 mL and 60 mL of saline. We routinely have an anesthetist in the operating room to guarantee patient safety and comfort, who is always advised to avoid sedation as much as possible. When necessary, an opioid-free sedation technique is employed. In the right leg, we ligated the small saphenous vein at its junction with a calf vein and ligated two N3 collaterals, leaving the small saphenous vein draining through two perforators. In the left leg, we ligated a collateral draining to the great saphenous vein from the inguinal ligament and an N3 draining reflux from the great saphenous vein to the calf. A total of 5 small incisions were made. The patient was discharged two hours after the operation wearing compressive stockings and taking 40 mg enoxaparin per day for 3 days, according to our postoperative routine.\nOn the sixth postoperative day, duplex scanning was performed, showing minor continuous reflux in the small saphenous vein of the right leg and even less reflux in the great saphenous vein on the left. The right small saphenous vein had been 7.4 mm before the operation and was 3.8 mm after. The left great saphenous vein had been 4 mm before the operation and had not decreased in size during the initial postoperative period. The patient scored pain at 3 on a 0-10 pain scale and had taken one 750 mg paracetamol tablet during the entire postoperative period. We made a full photographic record before and after the operation ( \n ). There were no photographs or records of symptoms available from the original operation.\nIn relation to the wrong-site surgery, we comforted the patient and reported the case to both the previous surgeon and the patient safety surveillance team at the hospital where the operation had been performed. |
We report the case of a male patient aged 46. The patient had been diagnosed with MG at age 26, when symptoms of facial and limb extremities weakness were noticed for the first time. Following investigation, a thymoma was found as the cause of MG, and at age 32, he was submitted to a thymectomy and treated with acetylcholinesterase inhibitors and immunosuppressants, namely, corticosteroids.\nAt the age of 45, the patient was referred for the first time with complaints of facial pain in the right malar region and purulent nasal discharge from the same side. He was clinically diagnosed with acute sinusitis and underwent medical treatment, with slight relief of symptoms. As symptoms persisted after medical management, a CT scan was ordered, revealing an expansive maxillary sinus opacification with erosion of the right maxillary sinus medial wall (). The magnetic resonance imaging (MRI) revealed a mass in the right maxillary sinus with an heterogeneous pattern in the T1 sequence with contrast ().\nAn endoscopy for biopsy purposes was performed, but it was inconclusive due to clinically relevant hemorrhage from a vascularized lesion protruding in the medial wall of the maxillary sinus. An angiography and preoperative embolization () of the right sphenopalatine artery was undertaken, and a meatotomy and ethmoidectomy under endoscopic control were performed (Figures and ). Unexpectedly, the histologic result was a kaposiform hemangioendothelioma, a proliferation of endothelial fusiform cells, considered to be nearly exclusive of children and teenagers.\nThe patient remained stable with only symptomatic treatment until the age of 50 when, following worsening of MG symptoms (specifically astenia and fatigue), metastatic lung deposits of the thymoma were diagnosed. Surgical removal of the metastasis was undertaken.\nThe thymoma was classified as a B3 (WHO, 1999), and therefore, the patient was also treated with adjuvant radiotherapy. During radiotherapy and due to worsening of MG symptoms, human immunoglobulin and corticosteroids were administered.\nThree months after these events, the patient had a new relapse from his MG and started corticotherapy. Two weeks into the treatment, he referred to newly skin pigmentation on the left wrist and legs. He also stated that these skin lesions were present 3 months earlier but smaller in size, growing with the recently added immunosuppressant therapy. These lesions were biopsied, and the histologic result was Kaposi sarcoma (KS). The patient was found to be HHV-8 IgG positive and HIV 1/2 and HTLV I/II negative. |
A 20-year-old asymptomatic female, G1P0, with a history of curettage, is presented to the Department of Gynecology and Obstetrics for termination of pregnancy. Her last menstrual period was 17 weeks and 2 days ago. Abdominal ultrasound revealed a clear gestational sac (GS), fetus with heartbeat and placenta previa. Abdominal ultrasound also showed the GS at a distance from the cavity, with a compressed myometrium between the two of them (). The patient received a MRI examination, showing an enlarged uterus of 13.0 cm ×11.7 cm × 7.9 cm, because of pieces of evidence. The MRI demonstrated a foetus with clear organs (), and compressed the lower uterine segment (). The GS was not connected with the uterine cavity and endometrium, but embedded into the myometrium in the right posterior wall of the uterine. A linear hypointensity of the junction zone was observed between the GS and the uterine cavity on T2weighted image ().\nThis result is likely to be placenta implantation as the myometrium cannot be separated from the placenta. The patient was at risk of the uterine rupture and life- threatening haemorrhage. Emergent management should be performed. Performing uterine artery embolization (UAE) and interventional therapy is inadvisable because of the obstructing myometrium between the cervix and placenta. Employing a surgical exploration of the abdomen was decided that was undertaken under temporary balloon occlusion of the abdominal aorta to reduce the loss of blood. The balloon was placed in the abdominal aorta between the opening of renal artery and iliac artery just before the operation. If the area of the focal damage was heavy, the subtotal hysterectomy or hysterectomy was needed. Otherwise, clinicians can perform excision by laparotomy and hysteroplasty. Given the age of patient, clinicians did their best to perform hysteroplasty instead of hysterectomy. The intramural ectopic GS in the second trimester was successfully excluded without life-threatening haemorrhage (). The patient had an uneventful postoperative course. Her β-hCG titre decreased to 1727 mIU ml–1 on the second day after operation, and then to 440.1 mIU ml–1 on the sixth day. She was discharged 6 days after surgery. |
A 49-year-old premenopausal nulliparous Caucasian woman was admitted with the complaints of abdominal distention and constipation. She indicated that she had been defecating once every 7 to 10 days, frequently with the help of a laxative, and had had serious abdominal pain intermittently since childhood. She had undergone an exploratory laparotomy for ileus 1 year ago. The related surgery report stated that her colon had been extensively dilated and elongated, and there had been massive fecal impactions obstructing her colon. The impactions had been disintegrated and removed, and no other pathology had been observed in the surgery.\nSome laboratory and screening tests were carried out for differential diagnosis of the patient. Laboratory test results were nonspecific and tumor markers were within normal ranges. Anal manometry confirmed the presence of basal waves and rectosphincteric reflex. A barium enema showed dilation of her entire colon. Her colonic transit time was found to be prolonged. Her mucosal structure had a normal appearance at colonoscopy and there were no extra pathological findings in defecography. Informed consent was obtained and she underwent a subtotal colectomy including ileorectal anastomosis due to chronic constipation. Significant elongation and dilation of her colon were observed in the surgery. There were diffuse nodular and polypoid tumor formations in the colonic mesoderm. Since no early and late surgical complications occurred, she was discharged from our hospital at postoperative day 5 with surgical recovery.\nThe resection material was composed of distal ileum, cecum, ascending colon, transverse colon and sigmoid colon. Multiple nodular formations with a hard elastic consistency were observed in the mesenteric adipose tissue (Figure \n). They had a maximum size of 10 × 4 × 2.5cm; they were spherical-ovoid or irregular in shape, combined with each other and attached to her bowel wall by thin fibrous bands. The cut surface had a fibrous structure and appeared gray-white in color. Colonic mucosa was normal, however, her bowel walls were hardened and partially thickened (about 1.5cm). Her parietal and pelvic peritoneum, mesoderm of the small intestine, uterus and ovaries were macroscopically normal. There were disorganized, irregular, partly dissociated smooth muscle bundles in the muscularis propria (Figure \n). Lesions in the mesenteric adipose tissue were composed of fusiform cells (with eosinophilic cytoplasm and ovoid-fusiform nucleus) that formed bundles crossing each other (Figure \n). The margins of the lesions were irregular. Mitosis, nuclear atypia, necrosis and Ki-67 ratio were evaluated to differentiate her case from a leiomyosarcoma (LMS). Mitosis, nuclear atypia and necrosis were not observed and her Ki-67 ratio was 1%. To differentiate her case from a gastrointestinal stromal tumor (GIST), we evaluated actin, desmin, S100, CD117 and CD34. Actin and desmin were positive (Figure \n), whereas S100, CD117 and CD34 were negative in our patient. We also evaluated estrogen and progesterone receptors in order to support DPL diagnosis. Both receptors were positive (Figure \n). The morphological appearance and the results of immunohistochemical studies were compatible with DPL. |
A 65-year-old woman presented with metastatic mucosal melanoma with a history of dyslipidemia and using proton pump inhibitor for gastroesophageal reflux. She had a remote tonsillectomy in the 1970s and was otherwise healthy. At the beginning of 2015, she noticed a discoloration on the right buccal mucosa and eventually sought medical attention. When first examined in oncology, in June 2015, the lesion was extending to the soft and hard palate. A punch biopsy of the lesion revealed that it was malignant melanoma (immunostains for Mart1 and HMB45 were positive). Initially, the tumor was not visualized on contrasted computerized tomography (CT) of the neck, nor on magnetic resonance imaging (MRI) with gadolinium. Investigation of metastatic disease including CT of the chest and abdomen were negative. The case was presented to the Tumor Board, and the consensus was for a surgical approach.\nIn August 2015, she underwent right partial maxillectomy and partial marginal mandibulectomy with midline mandibulotomy and free vascular flap. She also had ipsilateral selective neck dissection of levels I to IV, as well as a tracheostomy. Pathology revealed poorly differentiated malignant melanoma of the maxillary gingiva and hard palate, measuring 2 cm in largest dimension, with an invasion of the maxillary bone at the last molar, as well as satellitosis (more than five atypical mitoses were observed at high power field; mutation for BRAF was negative). The resection margins were clear; the closest margin was to the anterior tonsillar pillar at 10 mm. Out of the 25 lymph nodes dissected, one harbored a focus of melanoma measuring 1 mm, without extra-nodal extension. The pathological staging was thus pT4a pN1.\nShe was then seen by radiation oncology and underwent a course of adjuvant therapy of 50 Gy in 20 fractions between October to November 2015. The treatment was delivered using intensity modulated radiotherapy (IMRT) to the site of the resected primary tumor, and it was well tolerated with moderate acute side effects - grade 2 mucositis, and grade 1 dermatitis. Clinically she recovered well during follow-up, with difficulty masticating food due to her teeth not aligning following surgery, so she ate mostly pureed food and diet supplementation via percutaneous endoscopic gastrostomy (PEG) tube. A follow-up CT scan of the neck in May 2016 revealed a new soft tissue density at the floor of the mouth, which was outside the irradiated field. No evidence of lymphadenopathy was seen (Figure ). The mass grew rather quickly, and in June 2016, a new right neck mass was seen on level I/ II. Fine needle aspiration of the mass showed the presence of metastatic melanoma. An MRI scan of the neck revealed a rapidly progressing soft tissue lesion along the right side of the floor of the mouth (Figure ). Further metastatic work-up from June found new multiple sub-centimetric lung nodules bilaterally. She did not have significant lymphadenopathy, and CT scan of the abdomen revealed no metastatic disease. Positron emission tomography (PET)-CT scan performed in July 2016 showed increased metabolic activity to the lesion on the floor of the mouth, and a mildly hypermetabolic small left cervical lymph node at level two that was thought to be more likely reactive. The pulmonary nodules were too small to be resolved metabolically in the PET study, but a repeat CT scan of the chest in August showed pulmonary nodules increasing in size (Figure ).\nShe was assessed by medical oncology and was enrolled in an experimental trial comparing epacadostat 100 mg BID for 21 days versus placebo. Both groups received pembrolizumab 200 mg IV (3 mg/kg) every three weeks (protocol MK2475-252). The first cycle started in early August 2016. The tumor initially responded and shrank in size, but this was short-lived; it soon progressed causing symptoms including stiffening of the tongue, pain, increased drooling, as well as dysphagia accompanied with a weight loss of three pounds over four weeks. Due to the progression of the disease, she was removed from the experimental trial after one cycle but continued to receive pembrolizumab off-study. After a total of four cycles of pembrolizumab, she was referred to radiation oncology for palliative irradiation of the neck for symptom control. At the time of assessment in October 2016, two months following the first cycle of pembrolizumab, she continued to have enlargement of the neck tumor causing increasing dysphagia. There was no ulceration, but the mass involved the skin and extended from the mandible down to the clavicle with small satellite nodes next to it. A palliative course of radiation of 24 Gy in three fractions on days zero, seven, and 21 was delivered using IMRT.\nFigure shows the CT simulation performed on October 13th, 2016. The imaging showed the progression of the disease as compared to the images from June 2016, with the tumor now causing mass effect and putting the patient at risk of obstructing the airway. She received the first radiation treatment on October 19th, to which she responded almost immediately with a clinical reduction in tumor size of approximately 20% only after a week post-radiation. She received the second radiation treatment on October 26th. For the third fraction, she underwent a new CT-sim and re-plan because of the reduction in the tumor size (Figure ). The most surprising response to the course of irradiation to the neck area was the regression of the lung lesions.\nBefore commencement of radiation, it was noted by medical oncology in October 2016 (ten weeks and five days after the first cycle of immunotherapy) that she was having increasing shortness of breath. The fifth cycle of immunotherapy was held for this reason. CT imaging of the chest in October 2016 (nine days after the first fraction, and two days after the second fraction of radiation) showed interval progression of the diffuse bilateral pulmonary metastases when compared to a previous CT-scan (Figure ). However, by the time of imaging, the dyspnea had already resolved. Imaging of the chest was not done immediately before the commencement of irradiation when she was most symptomatic. She received the third fraction of radiation on November 9th, 2016. Repeat CT scan of the chest performed on November 21st, 2016 showed that the lesions previously seen were remarkably reduced both in size and in numbers (Figure ). The cone beam CT done for positioning purposes also provided valuable information about the evolution of the lung disease. Although only the apices of the lungs were captured in the images, regression of lung lesions in size and number following the first fraction of irradiation of the local tumor in the neck was observed (Figure ).\nThe neck mass had also decreased significantly. Figure shows a CT imaging of the neck performed in October 2016, two days after the second fraction of radiation. Compared to June 2016, there was an interval growth of the large neck mass with increased in size of adenopathy. However, it should be noted again that no baseline chest imaging was performed before the commencement of palliative irradiation. Clinically, the tumor mass was responding to radiation. Repeat imaging performed three weeks following completion of the three fractions of radiation therapy showed a significant reduction in the size of the main neck mass as well as a reduction in size and numbers of neck adenopathy (Figure ). Figure is the latest CT imaging in March 2017. Unfortunately, the study was performed without contrast, so accurate assessment of the tumor was difficult. However, the bulk of the disease had diminished in size. PET scan performed in April 2017 revealed significant residual but regressing tumor activity at the right floor of the mouth, (from SUV of 16.8 to 6.1) as compared to July 2016. She continued to do well until July 2017, when two of the lung lesions were found to be growing. They were treated with stereotactic body radiation therapy (SBRT). Her overall disease remained stable, and she continued pembrolizumab for a total of 20 cycles until the end of January 2018, when a repeat CT scan showed progression of disease in the lungs. The plan was to switch her to ipilimumab. |
A 52-year-old man had suffered from severe edema of his face and upper extremity. He was diagnosed with SVC syndrome and referred to our institution for the treatment of SVC syndrome. Computed tomography scan revealed giant ascending aortic aneurysm with the dimension of 79 mm in size and SVC was compressed severely by ascending aorta (Fig. ). Echocardiography showed moderate aortic regurgitation and no dilatation of left ventricle. Coronary angiography showed normal coronary arteries including normal coronary ostia. Although we had checked serodiagnostic tests for syphilis, Treponema pallidum hemagglutination reaction (TPHA) and fluorescence test assay absorption (FTA-ABS), the results of their examination were not revealed preoperatively. We performed surgical repair of the aortic aneurysm via median sternotomy in a usual manner. However, we detected moderate serous pericardial effusion and severe adhesion around ascending aorta. We could not dissect between ascending aorta and SVC because of severe adhesion. Cardiopulmonary bypass (CPB) was established between bilateral vena cava drainage and right femoral artery perfusion. The patient was cooled to the core temperature of 25 °C, the aneurysm was opened under deep hypothermic circulatory arrest and selective cerebral perfusion was established. Cardiac standstill was established by retrograde and selective cardioplegic infusion. The marked adhesion was detected around distal aortic arch suggesting the inflammatory process around the aorta. The distal anastomosis was performed at 6 cm distal site from left subclavian artery. Since the anastomotic site was slightly dilated, elephant trunk technique was performed simultaneously. After the distal anastomosis under open distal technique, three neck vessels were reconstructed individually. When we started systemic rewarming, we detected the bleeding from the hole of the opened aortic wall. The hole was slit shape and 8 × 1 mm in size. We diagnosed the fistula between ascending aorta and SVC, and closed the fistula with 4-0 polypropylene in a running fashion. The weaning from CPB was uneventful. Postoperatively, we suspected syphilitic aneurysm because we could find that the preoperative serodiagnostic tests were all positive. Although postoperative histological examination of the aneurysmal wall did not show typical syphilitic features, obliterative endarteritis and fibrosis at vasa vasorum, the severely changed tunica media could be detected (Fig. a, b). Therefore, we diagnosed syphilitic aneurysm clinically. Penicillin G was administered intravenously for 4 weeks. The patient was discharged from our hospital on postoperative day 45. The patient remains asymptomatic 1 year later. |
In April 2016, a 76-year-old man presented to the ear, nose & throat (ENT) clinic with a four-month history of nasal obstruction and loss of smell, on the left more than the right. There was no history of epistaxis, hearing loss, vertigo or otalgia. The ear canal, tympanic membrane and tuning fork testing was normal. On nasal endoscopy a polypoidal mass filling the left nasal cavity and extending to the right side through the nasopharynx was evident. Pathology was consistent with inverted papilloma. The remainder of the physical examination of the head and neck showed no abnormalities. Computed tomography at that time showed a locally advanced tumor of the left nasal cavity and left maxillary sinus; the temporal bone was clear (Figure ).\nThe patient underwent a left endoscopic medial maxillectomy where the gross abnormal polypoid tissue was completely removed with histopathology findings consistent with inverted schneiderian papilloma with focal mild to moderate grade dysplasia (Figure ).\nThe postoperative period was uneventful. Unfortunately, there was no follow-up until the patient presented in February 2018 after 20 months of the initial surgery with tinnitus and hearing loss in the left ear. Otoscopy showed an intact immobile left tympanic membrane. No remarkable changes in the contralateral ear. Tympanometry of the right ear revealed (type A) normal middle ear pressure and low compliance, as for the left ear it revealed flat (type B) tympanogram. Audiometry results for the right ear showed moderate sensorineural hearing loss with 48 dB pure tone average, as for the left ear results revealed severe mixed hearing loss with a pure tone average of 83 dB with an air-bone gap of 20-25 dBHL from 500 Hz to 4 KHz. Nasal endoscopy showed a small nasal mass limited to the anterior maxillary wall on the left side with an unremarkable nasopharyngeal exam. Computed tomography of the head and sinuses demonstrated complete opacification of the left middle ear cavity and mastoid air cells without evidence of erosive bony changes (Figure ).\nThe patient underwent endoscopic resection of the left maxillary sinus with myringotomy and tube insertion in the left ear. A biopsy was obtained from the left middle ear after finding a fleshy mass filling the cavity. The pathology was consistent with inverted papilloma and severe epithelial dysplasia while the specimen from left maxillary sinus showed inverted papilloma with a milder form of epithelial dysplasia.\nIn May 2018, based on the above, the patient underwent a left canal wall up mastoidectomy with tympanoplasty. The tumor had filled the middle ear and extended towards the antrum and mastoid cavity. All visible tumor was completely resected, sent for pathology which showed the tissue to be consistent with nonkeratinizing squamous cell carcinoma in situ with focal micro invasion by inverted papilloma and malignant transformation (Figure ).\nHuman papillomavirus presence was detected with immunohistochemistry and found to be strongly positive for P16 (Figure ).\nPostoperative facial nerve functional assessment was intact. The assessment was done by using the House-Brackmann grading scale which was equivalent to grade 1.\nIn February 2019, CT scan showed erosion of the left ossicles and left tegmen tympani. The patient was admitted for a revision canal wall down mastoidectomy. The tumor had recurred and was seen in all compartments of the mastoid air cell system and the epitympanum extending to the middle ear. All tissue biopsies from the left mastoid, middle ear, and Eustachian tube showed invasive nonkeratinizing squamous cell carcinoma arising from inverted papilloma with malignant transformation. There was no involvement of nasopharynx as biopsy results were negative for neoplasm. In April 2019, the patient was referred for adjuvant radiation therapy (RT) due to residual malignant tumor in the left external auditory canal that was detected by positron emission tomography scan (PET) which showed interval progression of the fluorodeoxyglucose (FDG) avid soft-tissue attenuation that involves the left external auditory meatus (Figure ) and magnetic resonance imagining (MRI) which showed an interval progression of left external auditory canal wall thickness (Figure ).\nThe patient received a total dose of 66 Gy of radiation to the left ear and completed the course of treatment over seven weeks. |
This case report describes a 4-year-old boy born at term to non-consanguineous Black African parents by spontaneous vertex delivery with a birth weight of 3.0 kg. The pregnancy and perinatal period were uneventful.\nHe was referred from a rural hospital at the age of 11 months for further evaluation due to poor weight gain despite adequate breastfeeding. He was exclusively breastfed for 3 months and started formula feeds supplementation at 4 months without significant improvement in weight. There was no associated vomiting or diarrhea. He had multiple admissions for nutritional rehabilitation due to undernutrition with minimal weight gain. On weaning, he fed mainly on cow’s milk and hardly took any solid feeds from the family diet.\nAt approximately 1 year of age, he developed progressive abdominal distension despite low intake of feeds. There was no swelling in any other part of his body, no yellow discoloration of mucus membranes, and no seizures. He was HIV seronegative; there was no history of other chronic illnesses, and he was fully immunized. The child had delayed developmental milestones: social smile at 3 months, sitting without support at 12 months, and standing with support at 21 months. He is the seventh born child in a family of eight children. There is history of the death of two siblings. The first born was a boy delivered preterm who died at the age of 2 years 7 months with poor development and weight gain, and he had seizures at the time of death. The sixth born, a boy, with normal birth weight (3.2 kg) died at 1 year with a weight of 5 kg and he also had seizures. The other five siblings are alive and growing well.\nA physical examination of this boy at 11 months revealed that he was small for age with a doll-like face; weight was 4.7 kg (weight for age Z-score − 5.41 SD); length was 62.5 cm (length for age Z-score − 4.47 SD); head circumference 42 cm (head circumference for age Z-score − 3.25 SD). He had widened anterior fontanelle of 4 cm × 3 cm, frontal bossing, widened wrists, three teeth (five to six expected), and rachitic rosary but no other bone deformities. His central nervous system revealed generalized hypotonia; an abdominal examination revealed a large liver of approximately 7 cm below the costal margin, which was smooth, firm, and nontender. No other abdominal masses were detected and other systemic examinations were normal.\nLaboratory and radiological investigations revealed low phosphorus of 0.58 mmol for which the normal range (NR) is 1.0–1.95 mmol, serum calcium 2.47 mmol (NR 2.25–2.75 mmol), markedly elevated alkaline phosphatase 1167 IU/L (NR up to 300 IU/L), normal serum albumin 3.7 g/dl, and vitamin D 22.3 ng/ml (NR above 30 ng/ml; after taking a high dose of 300,000 IU of parenteral vitamin D). His total white blood cell count was 15,000/ml, hemoglobin was 11.4 g/dl, platelets were 713,000/ml, and liver transaminases were normal. Initial X-rays of his left wrist revealed typical rachitic features of cupping and fraying at the wrist as shown in Fig. . An abdominal ultrasound revealed gross hepatomegaly, moderate splenomegaly, and normal echo texture of his liver. Other organs were normal; there was no evidence of nephrocalcinosis.\nHe was initially treated for vitamin D deficiency rickets with high dose of intramuscular 25(OH) vitamin D of 300,000 IU. However, after 2 months of treatment, there was no reported improvement in physical growth and development. The child was then managed as X-linked hypophosphatemic rickets due to persistently low phosphorus. He was initiated on orally administered phosphorus at a dose of 1 g/day in three divided doses and 1,25 OH vitamin D (calcitriol) 0.25 μg per day. There was reported improvement in development (he began to crawl and later walked) and he had some weight gain. However, at approximately 34 months of age, he developed massive abdominal distension with stagnation in weight. At 46 months of age, he was re-evaluated by the endocrinology team at Mulago National Referral Hospital, the teaching hospital for Makerere University, where further investigations were carried out. Fasting blood sugar was 2.2 mmol (NR 3.3–5.6) and after feeding it was 7.6 mmol, urine analysis showed pH 6.0 (NR 4.5–8), glucose 100 mg/dl (2+) (NR nil), ketones positive (2+) (NR nil), and protein was nil. Serum bicarbonate was 22.6 mmol (NR 22–29 mmol); sodium and potassium were normal. Fasting total cholesterol was 7.29 mmol (NR up to 5.2 mmol) and triglycerides 6.99 mmol (NR up to 1.7).\nIn view of the clinical features (doll-like facies, poor growth, hepatomegaly, and rachitic features) and laboratory results (hypophosphatemia, glycosuria, ketonuria in the presence of hypoglycemia, elevated cholesterol and triglycerides, and gross hepatomegaly on ultrasound scan) a diagnosis of FBS was made. No genetic testing was done to confirm the diagnosis. The child was initiated on a galactose-restricted diet; cornstarch was added to night meals, and sodium citrate was given at a dose of 15 mEq/kg per day in four divided doses. After 5 months of this therapy, his mother reported that he was more energetic and participated in more sustained play and had a weight gain of 0.8 kg. Table summarizes key parameters on follow up.\nThe mother’s perspective of the different therapies for our patient’s condition was that she saw marked improvement in his wellbeing on starting orally administered phosphorus and vitamin D; he was no longer getting ill with recurrent respiratory tract infections, and she noted marked progress in development although minimal weight gain. She believed that this could in fact be a case of hypophosphatemic rickets due to changes in the laboratory parameters for alkaline phosphatase and serum phosphorus on follow up. However, she became doubtful when she saw other children treated for hypophosphatemic rickets at the endocrinology clinic with a good catch up in growth. In addition, she was worried that the marked abdominal distension in her child could be another disease. On starting a galactose-free diet, she was worried that her child would not survive without milk because it is the child’s mainstay food. She was surprised that there was weight gain after stopping the milk and was later convinced to maintain the therapy. |
A 29 year old male, college lecturer by profession, presented with pain in the left hip and inability to bear weight on left lower limb for 2 years, along with an active discharging sinus over the thigh. He was operated for giant cell tumor of the proximal femur 8 years back by excision and reconstruction by a custom made total hip replacement (THR) prosthesis. He developed infection following reconstruction and underwent multiple surgeries for the control of infection. Routine blood investigations revealed elevated total leukocyte count, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) levels. Radiographs revealed protrusion of the acetabular and femoral components into the pelvis through a breach in the acetabulum and loosening of prostheses, zones of lysis with extensive periosteal reaction in the remnant femur [].\nAll the investigations put together were suggestive of possible pelvic discontinuity and septic loosening of the prosthesis. He was planned for revision reconstruction surgery by a two stage procedure.\nThe first stage consisted of removal of the prosthesis, debridement, and placement of an antibiotic cement spacer. Antibiotic cement was prepared by amalgamation of Vancomycin powder (4 g in 40 g packet of cement) in Gentamicin precontained cement (Palacos R+G). A cement spacer in the shape of a sphere was made for the acetabulum and the femoral aspect was taken care by an antibiotic cement coated Kuntscher nail inserted into the canal [Figure and ]. Routine bacterial cultures did not grow any organism. He was put on intravenous Amikacin (750 mg once a day for first 5 days) and Magnamycin (2 g twice a day) for 6 weeks and then switched over to oral cefuroxime (500 mg twice a day) for another 6 weeks. The followup ESR and CRP showed a downward trend at 6 weeks and returned to normal by 3 months. Then, the patient was planned for final reconstruction with proximal femoral allograft prosthesis composite for the femoral defect and trabecular metal shell (TM, Zimmer, Warsaw, IN, USA) with cage for the acetabulum. Cup-cage construct was chosen to address the pelvic discontinuity.\nThe hip joint was exposed by a standard posterior approach. The cement spacer in the acetabulum was removed, thoroughly debrided, and acetabulum was assessed for size and type of defect. The native bone was present only in posterosuperior and inferomedial portions, constituting around 30% of the true acetabulum with conspicuous pelvic discontinuity. A Homan retractor was placed in the obturator foramen which represents the level of inferior extent of the true acetabulum. The future hip center was identified by horizontal and vertical distances from this point as determined preoperatively from radiographs by comparison with the normal side. The acetabulum was prepared by graduated reaming until the appearance of bleeding bone and sized for the TM shell. The acetabular floor was filled with morselized allograft and prepared by reverse reaming. The TM shell of the size of last reamer was impacted into the prepared acetabulum and then held in place by screws drilled into the posterosuperior portion of the cup. The cup was then assessed for stability with a Kocher's clamp and it was not found to be satisfactory. Thus, the need of a cup-cage construct, which was suspected preoperatively by virtue of pelvic discontinuity, was corroborated by intraoperative assessment. A cup-cage construct using TMARS cage (Zimmer) was fashioned to secure the TM shell in place till osteointegration. The ischium was exposed and a slot was prepared by an osteotome for placement of inferior flange of the cage. The ilium in the posterosuperior portion of the acetabulum was also adequately exposed to receive the superior flange of the cage. The appropriate sized TMARS cage was selected and flanges were contoured to seat on the patient's ilium and ischium. The cage was embedded onto the TM shell and held in place by inferior flange in the slot in ischium and screws were drilled into the ilium in the superior flange. Best press fit, maximum contact with the host bone, and adequate stability of the cage dictated the ultimate orientation of the construct, i.e. in a more vertical position and relative retroversion. An appropriate sized liner was selected and cemented in position over the cup-cage construct with pressure in desired 45° abduction and 20° anteversion for maximum joint stability.\nFemoral reconstruction was done by allograft prosthesis composite after the removal of K nail []. The wound was closed over a drain. The drain was removed on the second postoperative day. Intravenous antibiotic cefotaxime (1 g every 12 h) was administered for the first 5 days and then switched over to oral cefuroxime for another 10 days. Patient was mobilized non weight bearing with walker for the first 6 weeks and then partial weight bearing with crutches till the signs of radiographic union appeared, i.e. at 3 months. Hip abductor strengthening exercises were started from 8 weeks and he was allowed full weight bearing from 3 months.\nFollowup radiographs at 30 months showed solid union of the allograft with the native bone on the femoral side and good consolidation on the acetabular aspect with no zones of radiolucency [Figure and ]. The patient is walking unaided and has a Harris hip score of 90. |
A healthy 34-year-old white primigravid woman presented at 28 + 4 weeks of gestation with mild pre-eclampsia. She had no significant past medical history and her antenatal care had been uneventful. The pre-eclampsia was treated with intravenously administered magnesium sulfate (at admission) and methyldopa 750 mg 3 times daily and she received corticosteroids for accelerating fetal lung maturation. An ultrasound showed an intrauterine growth-restricted child in head position with an estimated fetal weight of 1047 gr and oligohydramnios. During admission, our patient was clinically and biochemically stable and daily cardiotocograms showed a reassuring fetal heart rate pattern. Two weeks after admission (30 + 4 weeks), the estimated weight of the fetus was 1116 gr with normal umbilical artery Doppler screening.\nAt gestational age of 31+ 3 weeks, almost 2 weeks after admission, our patient complained about sudden lower abdominal pain and fever. There were no previous signs of preterm labor before this acute presentation. On clinical examination she looked pale with a blood pressure of 145/75 mmHg, a pulse of 103 beats per minute (bpm), a temperature of 37.9 °C, and a normal respiratory rate. On first physical examination her abdomen was soft but with slight tenderness in the lower abdomen. Ultrasonic evaluation showed an unviable fetus with no obvious signs of an abruption of the placenta. A vaginal examination revealed a closed portio and no vaginal bleeding. During the evaluation, she deteriorated with a blood pressure of 63/33 mmHg and a pulse of 130 bpm. She complained about an increased fluctuating abdominal pain and shoulder pain and showed difficulty in breathing. Her hypotension was considered due to intrauterine blood loss. Despite adequate fluid resuscitation, she remained hemodynamically unstable. She developed an acute abdomen. Ultrasound was repeated and showed free abdominal fluid. An emergency median laparotomy was performed and a hemoperitoneum of approximately 3 liters of blood was recovered. Both placenta and fetus were found outside the uterus due to a uterus rupture (Fig. ). The tear was 5 cm long and located in fundo close to the insertion of the left tube. A stillborn girl with a weight of 1130 grams was born. Our patient’s uterus was closed in two layers. Blood and clots were removed. Our patient’s pelvis showed no abnormalities, especially no evidence of endometriosis or adhesions. Inspection of her liver showed no rupture. The placenta was sent for pathological examination. Syntocinon (oxytocin) was administered intravenously. There was an estimated total blood loss of 3500 cc. Six units of blood and 2 units of blood plasma were transfused.\nIn the days after surgery she developed an ileus, which was treated conservatively and she developed high fever with increased infectious parameters, due to small abscesses dorsal of her uterus, treated with antibiotics. A computed tomography (CT) scan showed a subcapsular liver hematoma without a decrease in her hemoglobin level or platelets level, which was treated conservatively. Blood cultures showed a Staphylococcus aureus infection. Endocarditis was excluded. An electrocardiogram (ECG) showed an intermittent second-grade atrioventricular (AV) block-type Wenckebach, without clinical consequence. The S. aureus infection was most likely a cause of an infected wound, which was treated with intravenously administered antibiotics, with a good response. One month after the event she was sent home. She was strongly advised not to get pregnant again. In the case of a new pregnancy, careful monitoring and an elective cesarean section were advised. |
A 35-year-old male patient had rectal carcinoma near the anal verge, for which he underwent APR. During postoperative period, there was wound complication and a sinus developed at the perineal wound. Meanwhile, he also received adjuvant chemotherapy and radiotherapy. He was managed conservatively over the time for 2 years, and discharge of serous fluid and sometimes pus persisted. For these chronic symptoms, the excision of the sinus tract was done. After 1 month of this surgery, the patient noticed leakage of urine from poorly healed perineal wound despite indwelling Foley's catheter. He presented at our institute with the same complaints after 3 months of this surgery.\nOn cystoscopy, there was a large fistulous opening in the prostatic urethra. Methylene blue injected from the sinus tract was seen coming into the prostatic urethra suggesting tract between prostatic urethra and perineal wound. Ascending urethrogram (ASU) was done which showed leakage of contrast from the posterior urethra near the bladder neck, tracking towards the perineum [].\nFor surgery, the patient was kept in the lithotomy position and midline perineal incision was given. Dissection of the bulbar urethra was done circumferentially. On further dissection, defect in the dorsal aspect of the prostatic urethra could be visualized. We disconnected the prostatic urethra from the distal urethra. Bulbar urethra had longer ventral rim than the dorsal aspect [].\nWe harvested two BM grafts from each side. One of the two was fixed at the proximal end of the urethra and then tubularized over the Foley's catheter. Another graft was placed as dorsal on lay at the distal end of the urethra and wrapped around the catheter. Anastomosis was done between the proximal and distal BM grafts, and continuity of the urethra was restored []. Gracilis muscle pedicle flap was brought into the perineum to fill up dead space created and to support BM graft. Incision was closed with drain kept in situ [].\nPostoperative period was uneventful. The drain was removed after 48 h of surgery and Foley's catheter on day 21. The patient voided urine without any leakage from the perineum. The patient was continent after the surgery. ASU done after 3 months showed no leak of contrast from the posterior urethra. On cystoscopy at 6 months, there was good take-up of BM graft at the place of previous fistulous site. |
A 37-year-old nonsmoker man with proved metastatic pulmonary adenocarcinoma came to our imaging ward to be assessed by CT scan of the chest, abdomen and pelvis about 8 months after the initial diagnosis. Findings in previous imaging investigations included left-sided pleural effusion, soft tissue density mass in the left upper pulmonary lobe, collapse/consolidation in the right upper pulmonary lobe, mediastinal and hilar lymphadenopathy, oval and round lesions with ring enhancement scattered throughout the right cerebral hemisphere and bilateral paraventricular and supraparietal regions 3-10 mm in size with surrounding edema in some areas and a small hypodensity in the right adrenal gland suggestive of metastasis or nonfunctional adenoma.\nIn the chest and abdominopelvic CT scan with contrast media the following abnormalities were found, some of which were really extraordinary:\nExtensive collapse/consolidation in the majority of the left lung (), left axillary lymphadenopathy (), anterior mediastinal mass (), subcutaneous nodules in the chest (, , ), bilateral moderate pleural effusion and moderate pericardial effusion with tumor adhesion and pericardial tumoral invasion (), left basal segment collapse (), invasion to the pleura with extension to the left chest wall and chest musculature (, , , ), patchy alveolar infiltration that could be metastatic lesions, multiple hypodense lesions in the liver suggestive of metastasis (), necrotizing celiac lymphadenopathy (), a large metastatic lesion in the right adrenal gland and a small lesion in the left adrenal gland (), a hypodense lesion in the head of the pancreas and dilated main pancreatic duct (), multiple blastic lesions in the vertebrae (), multiple subcutaneous nodules in the abdominal wall, some with ring enhancement and some with nodular enhancement (nodular enhancing lesions were suggestive of subcutaneous metastatic implant and given that there were no tenderness, rubor or any other signs of abscess, the ring enhancing lesions were suggestive of metastasis as well) (-), right intrarenal metastasis () and multiple peritoneal and retroperitoneal metastatic implantations in Morison’s pouch, right subhepatic space, left perirenal space and perisplenic space (, -).\nIn this case of unusual metastatic pulmonary adenocarcinoma, a rare normal variation was seen as well: double inferior vena cava (IVC) with left IVC draining to the right IVC through the left renal vein ( and ). Unfortunately, the patient died a few days after this last imaging. |
The patient was a 69-year-old Caucasian man without a history of mental illness or any previous psychiatric treatment. He was initially transferred to a medical emergency department with marked dyspnoeic symptoms and tachycardia, where an acute coronary syndrome was diagnosed. After laboratory testing and an electrocardiogram a non-ST elevation myocardial infarction (NSTEMI) was diagnosed. The following coronary angiography (an intervention that was endured by the patient with enormous dread), revealed severe three-vessel disease.\nThe patient was informed of the urgent indication of a bypass operation, which was planned as an emergency intervention on the same day. At the end of the angiographic intervention, this information caused a severe panic reaction with hyperventilation, tachycardia and the feeling of loss of control, which was successfully treated with benzodiazepines. He described an intensely irrational and unavoidable fear of putting himself in the hands of others -surgeons and anaesthetists in this case. Moreover, the fear of losing control of his body through loss of consciousness or compromise of physical integrity during an operation or surgical intervention was reported. The patient was not able to give his agreement for the operative intervention because of overwhelming panic and anxiety. Due to his intense fear he eventually refused the bypass operation. This dramatic situation led to a condition of anxiety, strain and severe agitation, which led to a psychiatric referral and, consecutively, to an in-patient psychiatric admission.\nThe patient was relieved by the psychiatric admission and the understanding of his phobic fear. He reported a 20-year history of severe coxarthrosis, which caused serious pain and progressive leg deformation and malfunction, and which had never been operated on because of his fear of surgery. The clinical examination revealed that his left leg was 4.5 cm shorter than his right leg. He also had a marked impaired gait due to a limp in his left leg. Additionally, the coxarthrosis led to an extensive inguinal hernia due to pain induced mismovement. He also refused the necessary inguinal operation for over five years due to the same phobic symptoms. Further phobic symptoms and other symptoms of anxiety were explored, such as his fear about GP visits and discussions with superior colleagues. The patient reported an age of onset in the early adult years. With regard to childhood and adolescence, he described fear symptoms while knocking on or opening 'foreign' doors. Though the patient showed avoidant personality traits, no personality disorder could be diagnosed.\nThe psychopathological findings at the time of psychiatric exploration were limited to intense fear in relation to the forthcoming surgical procedures and interventions. During the psychiatric exploration, the patient was polite, friendly, and honest. Compulsive symptoms were limited to the repeated checking of electric appliances. As a consequence of his lifelong avoidance strategies he seemed not to feel oppressive limitations in everyday life. Until then, he had never consulted a psychiatrist or a psychotherapist regarding his phobic symptoms. He described being ashamed of his unreasonable fear symptoms. Panic disorder symptoms were not observed at any time during the psychiatric exploration. No history of syncope was found. Family history revealed a suspected anxiety disorder in the patient's father, although he reportedly never consulted any physician or other healthcare professional. Further examinations of the patient such as laboratory tests, duplex sonography, an electroencephalogram and a cranial magnetic resonance imaging were entirely normal. A Specific Phobia was diagnosed according to DSM-IV criteria.\nTo improve the intense fear reported by the patient when being confronted with the problem of the necessity of the operation, a psychotropic treatment with escitalopram 10 mg once a day and pregabalin 150 mg twice a day was initiated. The patient described an improvement of these fear symptoms. Supported by intensive conversational therapy based on cognitive behavioural techniques, he stabilized and was subsequently discharged. The patient still refused the invasive procedure, as his fear of the procedure continued to overwhelm his fear of dying from a heart attack. Behavioural psychotherapy as an out-patient treatment was recommended in order to diminish the patient's phobic fears. |
Patient 1 was a 56-year-old male patient who presented with a T4 N2c M0 squamous cell carcinoma infiltrating the entire lower lip, part of the upper lip, chin, neck, cheek and mandible (, Panel A). After composite resection of the tumor and bilateral neck dissection, the final defect measured over 200 cm2 including the intraoral buccal mucosa, the mandible (from angle to angle), lower muscles of facial expression, the oral commissure and modiolus on both sides, the entire lower lip, 50% of the upper lip, the chin and submental soft tissue (, Panel B). The patient’s mandibular and intraoral mucosal defect was reconstructed with a free fibula flap from the right leg with a skin paddle measuring 14 cm x 8 cm along with a 2.4-mm reconstruction plate. The fibula was adjusted to the shape of the mandible with two closing wedge osteotomies. The external soft tissue defect was reconstructed using an ALT flap from the right thigh with a skin paddle measuring 25 cm × 8 cm. The fibula flap was anastomosed to the right superior thyroid artery and the right facial vein. The contralateral superior thyroid artery and facial vein were used for the anastomosis of the ALT flap. In order to create oral competence the skin paddles of the ALT and fibula flaps were tailored and the folded ALT flap (reconstructed lower lip) was suspended with a rolled tensor fascia lata sling from the right thigh, which was anchored bilaterally on the anterior aspect of the zygoma ().\nPostoperatively the patient developed partial necrosis in the periphery of the ALT flap, which was most likely due to his history of significant smoking and drug abuse. After debridement the defect was covered with a right cheek advancement flap measuring 30 cm2. The patient underwent adjuvant chemotherapy and radiation and his further recovery was uneventful. On 3-month and 7-month follow-up his tissue coverage was stable and oral competence was adequate (, Panel C,D,E).\nThe patient was initially lost to follow-up, but presented 4 years later to the emergency room with a large open wound of his left mandible. He reported that he had been fishing and a fish hook had caught in his mouth leading to an infection. Over a 4-year-period he had been able to maintain his weight through an oral diet. PET and MRI scans were negative for any recurrent malignancy. After debridement of the wound the left half of the reconstruction plate was removed. The fibula bone underneath was bleeding and appeared to be viable. The superficial bone layer was debrided and specimens were positive for osteomyelitis. Upon debridement the defect measured 10 × 5 cm. An ALT flap with a skin paddle measuring 32 × 7 cm was harvested from the left leg together with a small cuff of vastus lateralis muscle in order to provide additional soft tissue coverage to the lower chin unit. The ALT artery was anastomosed to the left transverse cervical artery. The anterior and external jugular veins were used for the venous anastomosis. A bilateral commissuroplasty was performed because the patient had preoperatively suffered from relative microstomia.\nPostoperatively the flap appeared to be vital and showed no signs of necrosis. Seven days postoperatively the patient was able to feed by oral soft diet (, Panel F). A 6-week intravenous antibiotics treatment was administered. |
A 35-year-old male patient, with persistent AF refractory to the pharmacologic treatment underwent RFA in our hospital. As a routine practice in our centre, the patient was assessed pre-procedurally by transesophageal echocardiography (TEE) on the day of procedure to delineate anatomy of pulmonary veins and left atrium (LA) and to exclude LA appendage thrombus. During RFA procedure all four PV were individually isolated under guidance of intracardiac echocardiography. RF energy was delivered using a conventional 4 mm and 8 mm tip ablation catheters and a power setting of 30 W, 50 °C around a circular decapolar catheter located at the pulmonary veins ostia. Application of the RF was immediately interrupted when microbubbles were detected by the intracardiac echocardiography (ICE). After a curative ablation therapy patient was kept on aspirin to prevent pulmonary venous or arterial thrombosis and recurrence of AF. Ambulatory follow-up cardiac computed tomography (CT) angiogram was done 3 months following RFA and showed approximately 60% left superior PVS but no intervention was performed because the patient was asymptomatic and he was kept on oral anticoagulation ().\nTwo months later, the patient developed cough associated with hemoptysis, mild intermittent fever, mild left-sided pleuritic chest pain and shortness of breath on exertion. He visited the primary health centre close to his residency where chest radiography was done and showed left upper lobe poorly marginated opacities and minimal left pleural effusion. Further laboratory studies were done to rule out pulmonary tuberculosis (TB) which were within normal limits. A definitive diagnosis was not made and the patient was given broad spectrum antibiotics and referred to pulmonology clinic in our hospital for further investigation.\nPatient presented to our hospital 6 months after ablation without improvement in the symptoms. Non-enhanced CT was done and showed increased parenchymal attenuation and multiple peripheral patchy consolidations in the apico-posterior and anterior segments of left upper lobe associated with minimal left pleural effusion (). Cryptogenic organizing pneumonia (COP), chronic eosinophilic pneumonia (CEP), fungal infection (pulmonary aspergillosis), lung cancer and primary pulmonary lymphoma were suggested as differential diagnosis and ultrasound-guided aspiration of the left-sided pleural effusion was carried out and the obtained specimen was fluid of serous nature. Besides that, lung biopsy was also performed and the histopathologic examination revealed surprisingly intimal hyperplasia associated with multifocal haemorrhagic infarction due to PVO and hypertensive pulmonary arteriopathy. After that contrast-enhanced CT scan was performed with 3D reconstruction and manifested clearly an occluded left superior pulmonary vein (LSPV) (). Occlusion was confirmed by conventional angiography. Ventilation/perfusion scan demonstrated absent perfusion of the involved lung parenchyma and left upper lobectomy was warranted. |
Our patient is a 19-year-old girl, with a long surgical history of multiple complicated abdominal surgeries. She was diagnosed with Primary Hyperoxalosis for which she underwent same setting liver and kidney transplant. A few years afterward, she was diagnosed with intestinal type diffuse large B-cell lymphoma. After that, she had an ileocecal mass that obstructs the bowel lumen and required oncological resection with right hemicolectomy and anastomosis which was further complicated by anastomotic leakage, wound dehiscence and intra-abdominal sepsis thus requiring emergency exploratory laparotomy and Hartmann's procedure with end ileostomy. The patient then underwent laparotomy, Hartmann's revision and ileostomy site closure. For the purpose of graft preservation, the patient was on long-term immunosuppressants.\nAfter more than a year from the last surgery, she developed a central abdominal bulge at the site of her laparotomy scar; she had midline incisional hernia. Due to the long history of laparotomies and multiple surgical procedures, a multidisciplinary approach was necessary, and a surgical treatment plan was set.\nAn enhanced CT scan was performed and showed thinning of the anterior abdominal wall muscles with severe atrophy of the left rectus abdominis muscle with rectus diastasis of around 10 cm distance (Figure ).\nThe injection was performed by the interventional radiologist with the patient in supine position under ultrasound guidance (Figure ). Injection sites were marked at the anterior axillary line between the costal margin and anterior superior iliac spine according to the technique described by Smoot et al. The area was prepped and draped in a sterile technique which was followed by application of local anesthesia in the form of 1% lidocaine at the skin of injection sites. Under ultrasound guidance, BTA was injected at the three sites on either side of the abdomen. The patient received a total of 300 units of BTA diluted in 150 mL of 0.9% saline with a concentration of 2 units/mL. Each of the six injection sites received a volume of 25 mL. Each of the three injection sites on either side of abdomen were used to target the external oblique, internal oblique, and transversus abdominis muscles. After the procedure, the patient recovered smoothly and was discharged home the next day to return for surgery after 3 weeks.\nAbove the iliac crest, transversus abdominis at anterior axillary line. Mid abdomen, internal oblique at mid axillary line. Below costal margin, external oblique at anterior axillary line.\nPatient went for surgery 3 weeks after Botox injections. The procedure started by infiltrating normal saline for subcutaneous hydro-dissection followed by removing the old scar at the midline. De-epithelization was continued just beneath the skin to raise it above the adherent bowel underneath until reaching the normal fascia on both sides of the abdomen. Then, the abdominal flap was raised in the subscarpal plane above the fascia. Closure of the defect and plication of the recti were done followed by placement of sized on-lay fully resorbable monofilament mesh (Figure ).\nTo further relax the lateral abdominal muscles, a total of 200 units of BTA diluted in 8 mL of normal saline were infiltrated at the same previously injected sites (Figure ). Drains were placed bilaterally above the mesh. Subcutaneous tissue was approximated with interrupted sutures in two layers. Skin was closed with 4-0 Monocryl in subcuticular fashion (Figure ). Patient had no complications and was followed up for the next 18 months with no recurrence. |
A 23-year-old sexually active female with no significant medical or surgical history initially presented to colorectal surgery for evaluation of a potential perianal mass. The patient reports having limited medical care and no recent gynecological visits. Three years prior to presentation, the patient first noticed a small growth on her perineum. Over time this growth had increased in size, but it had not interfered with any of her activities. She was still able to have sex and urinate without difficulty. The patient had no reported history of sexually transmitted infections. She denied significant pain, discharge, or bleeding from this mass.\nDuring the initial physical examination by the colorectal surgeon it was determined that this lesion was not arising from the perianal region but was in fact of periurethral or anterior vaginal wall origin (). The patient was referred to urology for further evaluation and discussion of surgical planning. Physical examination by the urologist revealed a 5 cm × 6 cm firm, nodular, mobile mass attached to the posterior lip of the urethra (). Further examination of the urethra did not reveal any additional masses or extensions. No preoperative imaging was performed.\nThe patient was scheduled for an examination under anesthesia, cystoscopy, and planned vaginal excision of the mass from the posterior urethral edge. Further imaging with ultrasound, CT scan, or MRI was deferred pending final pathologic findings.\nAt the time of a surgery, cystourethroscopy showed no evidence of urethral involvement with the mass. The bladder neck and bladder itself were noted to be unremarkable with no evidence of tumor, stones, or diverticulum. After completion of cystourethroscopy, a foley catheter was placed, and the mass was placed on traction. The mass was noted to have a broad base spanning from the 4 o'clock to 8 o'clock position encompassing the anterior vagina and posterior lip of the urethra (). The base was transected using electrocautery. At time of transection, it was noted that the base was very vascular and electrocoagulation was used to achieve hemostasis. The urethra was everted and oversewn to the cut edge of the vaginal tissue in an interrupted fashion with 4-0 chromic ().\nA frozen section was performed intraoperatively. The pathologist noted benign cytological features with no obvious source. The cut margin appeared free of any involvement. At the end of the case, the patient was transferred to the recovery room in stable condition with foley catheter and vaginal packing in place. Both the foley and vaginal packing were removed prior to the patient being discharged home.\nThat patient presented for a two-week postoperative appointment with urologist with no acute complaints. She was recovering well from her procedure. At her follow-up appointment final pathology results were as follows:The mass measured 5.6 cm in maximal dimension. The mass was polypoid and pedunculated with a smooth and glistening external surface. A section of skin was recognizable. The cut section was homogenous with a tan/white color. The microscopic sections revealed a well circumscribed, unencapsulated mesenchymal neoplasm composed of bland spindle cells with elongated nuclei and indistinct cytoplasm. The cells were arranged randomly with hyper- and hypocellular areas (patternless pattern), in a collagenized matrix with variably prominent, often staghorn-shaped blood vessels (). Focal chronic inflammation and myxoid changes were present. Mitotic figures, cellular atypia, or necrosis was not identified (). There was surface ulceration with associated reactive changes and dense collagen, surrounding the lesion.\nImmunohistochemically, the spindle cells were positive for CD34, bcl2, and CD99 (focally) and negative for S-100, desmin, and smooth muscle actin. These findings are consistent with a solitary fibrous tumor of the urethra. |
A 62-year-old male patient was admitted to a primary hospital because of right ankle injury after falling from a 3 m high place. The patient was diagnosed with pilon fracture (type AO-43-C2) and lateral malleolus fracture of the right limb according to the X-ray and CT scans (Fig. A, B), after which calcaneal traction was applied. Five days after injury, physical examination revealed mild swelling of the right ankle and no tension blisters. The anterior and posterior tibial arteries could be palpable without hemodynamic disorders and paresthesia. The patient underwent open reduction and internal fixation (ORIF) of the fracture under general anesthesia (Fig. C). It required approximately 5 hours for the surgical procedure because of the difficulty of reduction. The incision could not be closed completely after surgery; thus, vacuum sealing drainage (VSD), a type of negative pressure wound therapy, was used for wound closure. VSD was removed on the 6th day after surgery, and it was found that some skin edges of the anterolateral and posterolateral incisions were blackened and necrotic. The foot and toes had low skin temperature and dark skin color. Considering the poor blood supply to the right limbs, the patient underwent immediate debridement of the right ankle and immediate removal of the anterior plate and screws. Unfortunately, the condition has not changed for the better. Then the patient presented to our hospital for further treatment on the 9th day after the first ORIF operation. At the time of admission, the affected foot and distal lower leg revealed critical ischemia and nonpalpable palpation of the dorsalis pedis artery and posterior tibial artery (Fig. ). Digital subtraction angiography (DSA) revealed vascular occlusion of the upper third of the anterior tibial artery, occlusion of the lower third of the posterior tibial and peroneal arteries, and a small amount of collateral circulation (Fig. ). The patient was immediately treated with exploration and decompression of blood vessels and removal of the medial and lateral plates and screws, followed by thrombolytic and anticoagulant interventions to dissolve the clot. Surgical exploration demonstrated that the peroneal artery and anterior and posterior tibial arteries were continuous with long-segment embolisms (Fig. ). Blood circulation did not improve after the operation, and below-knee amputation was ultimately performed due to persistent intense pain and extensive soft tissue necrosis of the threatened limb (Fig. ). The procedures performed in this study were approved by the ethics committee of Affiliated Hospital of Southwest Medical University. Signed written informed consent was obtained from the patient. |
A 54-year-old male complained of decreased visual field in his right eye. He also had a history of sexual dysfunction, for which he was prescribed udenafil. He took his first dose of 100 mg udenafil three days before his visit to our clinic. The next day he found that his eye was mildly irritated, and he had a mild headache. He discussed these symptoms with his urologist, and his symptoms subsided on their own after several hours. One day before his visit to our clinic, he took his second dose of udenafil. The following morning, approximately 12 hours later, he reported blurred vision and a decrease in the visual field of his right eye. This patient did have a 20-year history of smoking but no history of either hypertension or hypotension, diabetes mellitus, hyperlipidemia, or cardiovascular disease. Upon examination, his best-corrected visual acuity (BCVA) was 20 / 25 in the right eye and 20 / 20 in the left eye. A pupillary examination revealed a right-sided relative afferent pupillary defect. Ocular motility was normal, and the biomicroscopy of the anterior segment was unremarkable. A dilated fundus examination of the right eye revealed prominent swelling of the disc with a disc rim hemorrhage (). Fundoscopy of the left eye revealed a healthy but crowded disc with a cup-to-disc ratio of 0.2 (). Fundus fluorescein angiography showed both an inferior choroidal filling delay and an inferior sector filling delay of the optic disc in the arteriovenous phase and diffuse leakage of the optic disc in the late phase (). Automated visual fields (Humphrey 30-2) revealed a generalized constriction of the visual field in the right eye and a normal visual field in the left eye (). Optical coherence tomography revealed a prominent thickening of the inferior retinal nerve fiber layer (). Laboratory testing revealed a normal blood count, an erythrocyte sedimentation rate of 10 and a normal CRP titer. A systemic evaluation was performed by a physician, and there was no evidence of cardiovascular disease, including hypertension, hypotension, diabetes, and hyperlipidemia. A neurologic examination was performed by a neurologist and was normal, and a magnetic resonance image (MRI) scan of the brain with gadolinium enhancement was able to demonstrate that the optic nerves appeared normal, and that there were no white matter lesions, which would have been suggestive of demyelinating disease.\nThe patient was counseled to discontinue using udenafil and to regularly monitor his blood pressure. The patient's BCVA was improved to 20 / 20, and fundoscopy showed a slightly pale disc one month after he had discontinued the use of udenafil. |
An 88-year-old man with hypertension was admitted to the emergency medical center at our hospital based on a complaint of abdominal pain. Abdominal computed tomography (CT) revealed advanced sigmoid colon cancer without any evidence of intestinal perforation or hypoperfusion (Fig. ). As the patient complained of severe abdominal pain and was hemodynamically unstable, emergent surgery was performed under the suspicion of a surgical emergency. The abdominal cavity was entered through a mid-line incision, and we found ischemic necrosis of the entire colon along with a huge tumor at the sigmoid colon. Therefore, we performed total colectomy with ileorectal anastomosis. There was no evidence of occlusion in the major mesenteric vessels, including the celiac trunk, superior mesenteric artery, and inferior mesenteric artery. After the surgery, we administered critical care for shock reversal and maintenance of adequate organ perfusion. The coagulation profile of Patient 1 suggested disseminated intravascular coagulation (DIC) (Table ). On the second postoperative day (POD), we ceased the vasopressor and inotropic treatment, and observed that adequate blood pressure was maintained. We weaned the patient off the mechanical ventilation and performed extubation on the fourth POD, and enteral feeding was started via a nasogastric tube on the fifth POD. The patient responded well to the treatment, and we proceeded to the de-escalation phase (with negative fluid balance) of the intensive treatment. However, we noted a darkish coloring of the patient’s tongue on the seventh POD (Fig. ), and the laboratory findings began to deteriorate (newly noted band neutrophils, decreasing platelet counts, and increasing lactate levels). Because the endotracheal tube had been removed on the fourth POD, it was unlikely that the discoloration was due to the pressure from the endotracheal tube on the tongue. Furthermore, there was no evidence of hypoperfusion in other organs, such as in the limbs or intestines. We performed bed-side duplex ultrasonography and did not detect any occlusive lesions in the external carotid or facial arteries. However, both lingual arteries arising from the external carotid arteries were not visible during the duplex examination. Additionally, bed-side laryngoscopy revealed no mucosal injury in the oral cavity or on the tongue. A meticulous oral hygiene regimen was started, and we administered empirical antiplatelet therapy. However, Patient 1 became hemodynamically unstable and developed multi organ failure on the eighth POD, and subsequently died on the twelfth POD, despite vigorous resuscitation. |
A 77-year-old man with a past medical history of type 2 diabetes, hypertension, and ESRD underwent deceased donor renal transplantation. Two months following his renal transplant, the patient was admitted for an acute kidney injury discovered on routine follow-up laboratory testing. From a baseline creatinine of 1.3 mg/dl after the transplant, he was noted to have a creatinine of 2.7 mg/dl and a renal biopsy was obtained. Histopathology was suggestive of mildly active cellular rejection and acute tubular injury but with no concerns for antibody-mediated rejection. The patient received three days of methyl-prednisolone 250 mg daily intravenously and was subsequently transitioned to high-dose oral prednisone with a taper.\nThe patient was readmitted within three weeks of his renal biopsy with worsening renal functions, now with a creatinine level of 3.8 mg/dl. At this time, he was on prednisone five mg daily, tacrolimus four mg twice daily, and mycophenolate 500 mg twice daily. His family reported poor oral intake and that he had been taking furosemide at home. He received intravenous (IV) normal saline and diuretics were held. However, as his renal functions did not improve beyond a creatinine level of 2.1 mg/dl, the decision was made to perform a repeat renal biopsy. During ultrasonography for the renal biopsy, concerns were raised for a possible renal artery aneurysm. An ultrasound of the right lower quadrant and transplant kidney subsequently showed a 3 cm x 3.4 cm x 4 cm aneurysm proximal to the renal artery anastomosis to the right external iliac artery (Figure ). The right external iliac artery to renal artery anastomosis was patent and the renal vein was noted to be patent as well. No peri-transplant kidney fluid collections were noted and no hydronephrosis was seen.\nSubsequently, a pelvic arteriogram was performed, which showed patent pelvic and iliac arterial flow. However, a large pseudoaneurysm arising directly off the right external iliac artery was re-noted and the renal transplant artery was noted to be filling from the distal side of the aneurysm and remained patent, although with sluggish flow (Figure ). A second known renal transplant artery was not seen and was presumed to be thrombosed.\nWhile the interventional radiologist considered closing off the pseudoaneurysm by stent placement, the origin of the aneurysm was noted to be too close to the anastomosis to allow for stent placement. Hence, transplant surgery was consulted. The patient was taken to the operation room for a re-exploration of his transplanted kidney and revision of the arterial anastomosis. The patient underwent exploratory laparotomy, and intraoperatively, significant inflammation and scar tissue surrounding the hilum of the transplanted kidney was seen. Once the pseudoaneurysm was entered, necrotic tissue and purulence within the pseudoaneurysm were noted. It was felt that the arterial flow to the transplanted kidney could not be restored and, therefore, a decision was made to proceed with the resection of the infected pseudoaneurysm and transplant nephrectomy. The transplanted kidney was explanted. A portion of the pseudoaneurysm was sent to pathology for further characterization, and a portion was sent to microbiology for culture. The explanted kidney was also sent to pathology for further analysis. The patient was transferred to the intensive care unit (ICU) postoperatively for further management and close monitoring. During the surgery, the patient received crystalloids and multiple blood products.\nOn arrival to the ICU, the patient had temporary dialysis access placed and started on continuous renal replacement therapy (CRRT). Given concerns for an infected pseudoaneurysm, he was empirically started on renally dosed vancomycin, piperacillin-tazobactam, and fluconazole. All immunosuppressive agents were discontinued at this time. Due to persistent vasopressor requirements while in the ICU, the patient was switched to vancomycin, meropenem, and micafungin. The infectious disease team was consulted for recommendations. Blood bacterial and fungal cultures remained negative. However, fungal cultures from the pseudoaneurysm grew Aspergillus flavus on three separate samples. The patient was then transitioned to IV isavuconazonium 372 mg every eight hours for six doses, followed by 372 mg daily with a plan to continue for at least six weeks. Vancomycin and meropenem were discontinued. The pathology of the explanted kidney showed transmural necrosis of the renal artery, no evidence of rejection, and scattered micro-abscesses within the parenchyma. As the patient improved clinically, he was transitioned from CRRT to intermittent hemodialysis. He was subsequently transferred out of the ICU in stable condition. His hospitalization was complicated by colitis secondary to Clostridium difficile for which oral vancomycin was initiated. Preparation was made to discharge him to an acute rehabilitation facility; however, a day prior to discharge, he was found to be unresponsive by his nurse and despite prolonged resuscitation attempts for cardiac arrest, could not be revived. The exact etiology for this sudden demise could not be determined. |
A 70-year-old female with no significant past medical history, Eastern Cooperative Oncology Group performance status 0 and no prior incidence of hypersensitivity reactions, was incidentally found to have a small nodule in the proximal right posterolateral vaginal wall after presenting with post-menopausal bleeding. Subsequent biopsy demonstrated a 9 mm invasive melanoma and the patient underwent wide local excision with confirmed negative margins. 4 months later, she developed a right periclitoral mass. Positron emission tomography/CT scan at that time demonstrated focal uptake in this area but no regional/distant metastases (). Excision demonstrated a large submucosal mass of atypical epithelioid cells with evidence of melanin synthesis, consistent with malignant melanoma. Breslow depth was 9 mm (3 mitoses/mm2) with a positive deep margin, and there was no evidence of lymphatic invasion. Her case was presented at a multidisciplinary tumour board and either additional surgery or radiation therapy was recommended to the patient. Owing to the significant morbidity anticipated with additional surgery, the patient opted for radiation therapy. Given the high risk of both local and regional/distant failure, concurrent chemotherapy was proposed. Owing to the historically poor response rates with standard chemotherapy, an immune pathway targeted agent was considered. This non-standard approach was actually initially proposed by the patient. After being thoroughly explained the current standard of care, in addition to the pros and cons of pursuing concurrent radiation and immunotherapy, the patient chose to proceed with combination immunotherapy and radiation treatment. Several studies have demonstrated that local radiotherapy primes and/or enhances an immune response through cytotoxic T lymphocytes. Concurrent immunotherapy may then further enhance the activity and/or duration of the downstream immune response. Given the historically low efficacy of our current treatment paradigms in this disease, as well as the preclinical/clinical rationale to combine radiation and immunotherapy, a strategy of pursuing a combination of ipilimumab with radiation was felt to be reasonable.\nRadiation was planned with intensity-modulated radiation therapy (IMRT) to the vulva and vagina (no elective nodal radiation to the groin) to an initial dose of 45 Gy (1.8 Gy/fraction over 25 fractions), and was to be followed by an electron boost to a total dose of 63 Gy (1.8 Gy/fraction over 10 fractions) at the site of the positive margin (). A thermoluminescent dosimeter was placed on the vulva at the start of treatment to measure the skin dose and read as 1.78 Gy [95% confidence interval (1.66–1.90)], confirming that the planned dose was accurate on the skin. The patient received her first cycle of ipilimumab (3 mg kg−1) 7 days after the start of radiation, and the second cycle was delivered 3 weeks later when the patient was at a dose of 36 Gy. Around this time (3 weeks post ipilimumab cycle 1), she began to develop non-painful erythema in the vulvar and perianal area, as well as a pruritic, grade 2 cutaneous eruption that morphologically showed distinct erythematous papules that coalesced into thin plaques over the upper arms, chest, back and face/ears (all toxicities were graded using Common Terminology Criteria for Adverse Events version 4.03). She did not experience any fevers or other systemic symptoms. By 48.6 Gy dose (10 days post ipilimumab cycle 2), the patient developed a grade 3 skin reaction () that was characterized as a moist desquamation with significant oedema, erythema and pain in the vaginal/vulvar/perianal region and was restricted to the radiation field (). A timeline of these events is illustrated in . After proper consent, a 4 mm punch biopsy of the affected skin was performed and histopathological examination demonstrated spongiotic and interface dermatitis with a perivascular inflammatory infiltrate consisting of numerous eosinophils, consistent with a fixed drug eruption.\nRadiation and ipilimumab were held given the severity of the moist desquamation, and the patient was referred to a dermatologist for evaluation of the ipilimumab-associated cutaneous eruption. It is noted that the patient had no existing risk factors or hypersensitivities predisposing her to an enhanced skin toxicity. The patient was started on 0.1% topical triamcinolone cream along with a methylprednisone dosepak. Given only mild improvement after 1 week, she was started on prednisone 60 mg daily (tapered over 7 days) with oral diphenhydramine for pruritus as needed. This resulted in significant improvement in her cutaneous eruption and pruritus. She then received a third cycle of ipilimumab (4 weeks after cycle 2) and resumed her periclitoral radiation boost without further issues after a 1-month break from radiation treatment. She went on to receive a fourth cycle of ipilimumab after completion of radiation without any complications.\nAt follow-up 8 months post completion of radiotherapy, she had complete resolution of the in-field toxicity and improvement of her ipilimumab-associated cutaneous eruption (). Clinical examination and positron emission tomography/CT imaging 10 months after completion demonstrated no evidence of disease recurrence. Most recently, at her 15-month follow-up, she remains disease- and symptom-free ().\nAt each follow-up visit, the patient has repeatedly expressed satisfaction with her decision to pursue immunotherapy rather than standard chemotherapy to complement her radiation treatment. She admits the initial side effects and intensity of the treatment course were difficult, although she is very pleased with the outcomes and would make the same decision if asked to do so again. |
A 48-year-old man with a history of alcohol abuse presented to the gastroenterology department for the first time in November 2006. He was suffering from severe epigastric pain and nausea for two days. Because the laboratory findings were suspicious for pancreatitis, the patient underwent computed tomography (CT) (Fig. ). The examination showed an edematous pancreas tail and fluid in the anterior prerenal space and in the anterior, posterior and lateroconal fascia. There were no signs of chronic pancreatitis or vascular complications. In the following months the patient had two similar episodes of acute tail pancreatitis.\nIn September 2009 he presented with vague epigastric pain of several weeks duration. Serum amylase and lipase levels were normal, however the cholestatic parameters were mildly elevated. Therefore, a magnetic resonance (MR) study was performed. The examination demonstrated moderate chronic pancreatitis of the tail with atrophy and irregular bording of the Wirsung’s duct. There were no other abnormalities. Further examinations also revealed a Helicobacter pylori gastritis.\nIn February 2012 the patient presented again to the gastroenterologist with epigastric and left upper quadrant pain. Laboratory findings were not suspect for acute pancreatitis. However, contrast-enhanced CT scan was performed in order to evaluate the known chronic pancreatitis and other causes of pain (Fig. ). This study surprisingly revealed a hypervascular lesion in the pancreatic tail consisting of a conglomeration of small hypervascular spots and blood vessels. Further examinations were performed in order to differentiate a vascular malformation from a hypervascular pancreatic tumor, especially an islet cell tumor or a hypervascular metastasis. Contrast-enhanced MR study showed a focal area of heterogeneous contrast enhancement without an apparent nodular component. The subsequent angiography showed two large feeding arteries in the early arterial phase, followed by a racemose vascular network, an early transient dense parenchyma stain in the early portal phase and early wash-out of the lesion in the portal phase (Fig. ). Based on these findings, a pancreatic arteriovenous malformation was suggested. The patient was treated with a resection of the pancreas tail and postoperative recovery was uneventful. Histopathologic examination revealed sequelae of chronic pancreatitis and numerous dilated blood vessels accompanied by blood clot formation and intimal hyperplasia (Fig. ). The histopathological findings were consisting with a pancreatic AVM. |
A 67-year-old man who was a former tobacco smoker (100 pack-year history) presented to our oncology clinic following a diagnosis of Stage IIIa NSCLC. He had a past medical history significant for ulcerative colitis, which was well-controlled on balsalazide with no recent flares. He was initially noted to have a lung nodule on a surveillance computed tomography (CT) scan for abdominal aortic aneurysm. A positron emission tomography (PET) scan was done, which demonstrated a metabolically active right lower lobe lesion measuring 9.1 x 3.9 centimeter with an extension to the hilum. Enlarged right paratracheal lymph nodes were also noted be metabolically active. He underwent endobronchial ultrasound guided fine needle aspiration of the mass and mediastinal lymph nodes. Results were consistent with squamous cell carcinoma with metastases in the lymph nodes. The pathological diagnosis was T3N2M0 stage IIIa squamous cell lung carcinoma. PET scan at the time did not demonstrate any evidence of extra thoracic metastatic disease and therefore he was advised to undergo neoadjuvant chemotherapy followed by restaging of mediastinum for consideration of curative surgical resection. The patient was deemed to be of good functional status and started on paclitaxel, carboplatin. After a single course of chemotherapy, he was hospitalized with near fatal sepsis. He was treated with intravenous (IV) vancomycin and Zosyn, followed by levofloxacin to complete a fourteen- day course. Following his recovery, his case was discussed at our institutional tumor board and given the severe toxicity and low likelihood chemotherapy would make him operable, the patient was started on a course of concurrent chemoradiation. He tolerated this treatment well until the end of his six-week course when he developed febrile neutropenia and was found to have C. difficile colitis. He was treated with IV cefepime, followed by Augmentin to complete a ten-day course as well as fourteen days of oral vancomycin. CT scan during this hospitalization showed a new liver lesion, which was proven by biopsy to be metastatic squamous cell carcinoma.\nHe was subsequently started on immunotherapy nivolumab 3 mg/kg every two weeks. Two weeks following his first nivolumab administration, the patient was noted to have a platelet count of 1000/μL (previously 188,000/μL) as well as petechiae on his arms and legs. His platelet count was repeated in a citrate tube and confirmed on peripheral smear. His hemoglobin and white blood cell count remained unchanged compared to prior laboratory results. Based on his severe thrombocytopenia, he was admitted to the hospital urgently that same day. On arrival, he was hemodynamically stable. Physical exam was notable for petechiae across his chest and extremities as well as bullae in his oral cavity. Given the acute drop in his platelet count and recent immunotherapy exposure, he was diagnosed with a rare ir-AE, known as nivolumab- induced ITP. Platelet-associated immunoglobulin G antibody levels (PAIgG) were not measured. He was initially started on IV steroids and received three doses of IV immunoglobulin (IVIG). His platelet count had increased to 37,000/μL. At this point, the patient was switched to oral prednisone. However, his platelet count decreased to 18,000/μL. He was then started on weekly rituximab and after three doses, his platelet count recovered to 150,000/μL.\nFollowing platelet count recovery, the patient was enrolled in our clinical trial and started on an antibody-drug conjugate as treatment for his advanced NSCLC. One week after receiving his first dose, he was readmitted to the hospital for neutropenic fever and respiratory distress. He was treated for pneumonia as well as suspected radiation pneumonitis. Despite treatment, his respiratory status worsened and after a goals of care discussion with his family, the decision was made to pursue comfort measures. Unfortunately, the patient died fourteen days following hospital admission. |
A 57-year-old male came with complaints of left loin pain and hematuria for the past 5 days, without other significant history. On physical examination, a mass was palpable on the left lumbar region. Ultrasonography (USG) of the abdomen showed a solid mass lesion with internal vascularity seen involving the left kidney. Contrast-enhanced computed tomography (CECT) of abdomen was done for further evaluation. CECT of the abdomen showed a well-defined heterogeneous soft tissue mass lesion with a tiny speck of calcification involving the interpolar region of the left kidney. The lesion was seen extending into the perinephric space and abutting the perinephric fascia seen. No extension beyond the fascia was seen. No extension into the main renal vein was seen. No lymphadenopathy was seen. The lesion showed hypervascularity in arterial phase with relative washout in venous phase images (). In addition to the renal mass, there was a well-defined homogenously and moderately enhancing polypoidal intraluminal mass lesion measuring about 2.5 cm seen involving the rectum about 12 cm from the anal verge (). Significant wall thickening and perilesional lymphnodes were noted with the largest lymphnode measuring 10 mm in short axis (). Based on the radiological findings, a possibility of synchronous malignancy of the left kidney and the rectum was raised. The second possibility raised was a renal cell carcinoma (RCC) of the left kidney with metastasis to the rectum. Based on the radiological diagnosis, the patient was subjected to colonoscopy-guided biopsy of the intraluminal mass lesion of the rectum () and sent for histopathological analysis. Histopathology showed features of poorly differentiated neuroendocrine carcinoma () and immunohistochemistry showed tumor cells focally positive for synaptophysin and chromogranin which confirmed neuroendocrine carcinoma (). The patient underwent radical nephrectomy of the left renal mass and histopathology confirmed a grade II clear cell RCC (). Surgical resection of the rectal mass was not done as the patient was unwilling for further surgery. The patient is started on cisplatin and etoposide chemotherapy for the neuroendocrine carcinoma and is followed up every 3 months. |
A 52-year-old woman weighing 50 kg, 168 cm height with body mass index of 29, presented to orthopedic clinic with complaints of generalized bone pain and low back pain. She developed low back pain 7 years before presentation which she had been managing with over-the-counter drugs. Pain does not radiate to any part of the body. There was no history of trauma before the onset of the pain. There was no associated fever, weight loss, loss of appetite, nor weakness of the lower limbs. Low back pain progressively got worse before she decided to present at the orthopedic clinic. There was mild cough productive of whitish sputum. She was treated with analgesic, antibiotics, and other drugs and she got better. Prior to development of the back pain, she occasionally had generalized bone/body pain, for which she took over-the-counter drugs each time. Due to the repeated presentation to orthopedic clinic with bone pain for which no other cause was found in addition to the incidental finding of anemia, hemoglobin genotype was requested for which reported hemoglobin SS by electrophoretic method. The patient was subsequently referred to hematology clinic for follow-up/expert management. Further investigation was subsequently done with high-performance liquid chromatography (HPLC) which confirmed SCA.\nThe patient had cholecystectomy in 2011 due to calculous cholecystitis. She is 6 years postmenopausal. Her first confinement was in 1990, pregnancy was uneventful, and she was delivered of a female baby. The second pregnancy was in 1995, and she had miscarriage at 8-week gestational age. Third and fourth (last) confinement was in 1996 and 1999, respectively. Pregnancy was uneventful although the 3rd baby (male baby) died at the age of 2 years from diarrhea disease according to her. The deliveries were all through spontaneous vortex delivery, no cesarean section was done. The patient had never received blood transfusion. All the pregnancies were managed in a maternity home. Her level of education is primary school. She is a civil servant working as a cleaner. She is married with two surviving children. She is the last child of her parents with ten children. Seven died in childhood of febrile illness. She is a known peptic ulcer disease patient. She does not smoke nor take alcohol, not a known hypertensive, diabetic nor asthmatic. Husband is a farmer/petty trader.\nPhysical examination showed a middle-aged woman in no obvious distress, afebrile to touch, mildly icteric, moderately pale, with no pedal edema and no peripheral lymphadenopathy, had dorsal protrusion of the vertebrae at the thoracolumbar region []. Examination of the chest showed no significant findings. The abdomen was full and moves with respiration, supraumbilical vertical surgical scar (had cholecystectomy due to cholecystitis in 2011), mild epigastric tenderness, and no organomegaly. There was no other significant finding.\nSome laboratory investigations done showed the following: Mantoux test was 0 mm, Ziehl–Nelson stain for acid-fast Bacilli was negative, GeneXpert for MTB was negative. Bence–Jones protein in urine was negative, and erythrocyte sedimentation rate (ESR) was 27 mm in 1st hour. Total serum protein, albumin, and globulin were within normal as well as serum calcium. Full blood count reported hemglobin level of 8.6 g/dl, packed cell volume 24%, red cell count of 3.6 × 1012/L, platelet count of 284 × 109/L, and total white blood cell count was 7.3 × 109/L with a differential of 53.6% neutrophil, 38.5% lymphocyte, 5.4% monoctye, 2.2% eosinophil, and 0.3% basophil, mean cell volume (MCV) 75 fl (80–100 fl), mean cell hemoglobin (MCH) 24 pg (27–34 pg), and mean corpuscular hemoglobin concentration 32 g/dl (32–37 g/dl). HPLC showed the following results: HbF 8.3% (<1%), HbA2 4.4% (<3.5%), and HbS 82.7%. Biochemical parameters include total bilirubin of 46 (3–21 mmol/l), conjugated bilirubin 28.9 (<8 mmol/L), aspartate transaminase 36 (5–18 IU/L), and alanine transaminase 25 (3–15 IU/L). Urea, creatinine, sodium, potassium, chloride, and bicarbonate were all within reference range. C-reactive protein (CRP): 20.7 mg/L (<8 mg/L), lactate dehydrogenase (LDH): 388 U/L (105–333 U/L), ferritin: 171 ng/ml (12–160 ng/ml), copper: 1.72 umol/L (11–24 umol/L), and zinc: 102.2 ug/dl (11.5–15.5 ug/dl).\nX-ray of the lumbosacral spine showed wedge collapse of the 12th thoracic and first lumbar vertebrae with posterior angulation of the thoracolumbar junction giving dorsal Kyphosis around the region []. The bones of the lumbar vertebrae were osteopenic. There was no obvious degenerative changes or paravertebral soft-tissue mass. There was normal alignment for the rest of the lumbar vertebrae. The intervening disc spaces were also normal. |
A 28-year-old housewife admitted with reddish tender lesions all over the body of 2-day duration. There was no associated fever or other systemic complaints except for severe body pain and pain over the lesions. There was no history of prolonged sun exposure or drug intake prior to onset of lesions. There was history of four similar episodes in 9 months with admission in different hospitals each time and dropping out half way while on treatment. On examination, lesions were reddish brown macules all of similar size and morphology all over the body predominantly over thighs and legs with fewer lesions on chest, abdomen, face, and total sparing back of trunk [Figures –]. On scrubbing with alcohol, some lesions showed fading of color. During all the episodes, patient had same type of lesions without significant damage to the skin; hence, a diagnosis of dermatitis simulata was made and patient was investigated to rule out other possible differential diagnosis of dermatological conditions like fixed eruptions, vasculitis, or generalized lichen aureus. Nothing significant was detected from routine blood and urine examination and skin biopsy. Patient was treated with only placebos. Condition was not revealed to patient or relatives and no such history was probed to find out nature of infliction of lesions as same would lead to patient hopping. No new lesions appeared and all lesions started fading in about a week. Later, detailed psychiatric evaluation was done, according to which patient did not have any perceptual abnormalities. Psychiatric evaluation could not elicit any external incentive for act like fear, avoiding duties, or financial gain. Both patient and bystanders gave inconsistent history. Since the past nine months, patient is being admitted in different hospitals four times, each time developing lesions suddenly. All the episodes occurred when the husband was away from home. She would get admitted to hospital and while the investigations are being done, abscond from hospital once the false nature has been identified. With the history and clinical finding, diagnosis of Munchausen syndrome presenting as dermatitis simulata was made. Skin lesions cleared completely in 2 weeks. Patient is under regular psychiatric follow-up with no new episodes since past six months. |
A 21-year-old Chinese woman was admitted with a seven-month history of unilateral severe hematuria. In March 2008, urine analysis revealed that the patient had asymptomatic microscopic hematuria. She was given oral antibiotics but, after one week of treatment, the patient's symptoms persisted. In a follow-up after four months, the patient still had hematuria. In July 2008, cystoscopy showed blood oozing from the left ureteric orifice. Real time ultrasonic imaging (duplex scanning) showed that the angle between the abdominal aorta and superior mesenteric artery was uncommonly sharp. The posterior wall of the superior mesenteric artery was very close to the anterior wall of the aorta. The inside diameter of the left renal vein, at the point where it crosses the aorta and SMA, was less than 2.0 mm. The highest peak velocity was detected at this point. The inside diameter of the left renal vein was 9.0 mm with lower peak velocity. The location of both kidneys at erect position was 30 mm less than that of the decubitus position. A CT was used for detecting the anatomical relation of the LRV with the aorta and SMA. The left renal vein was swelling and was compressed at the point where it crossed between the aorta and SMA, while the distal part of the point was strikingly expanded (Figure and ). An IV urogram showed that the shape of both the renal pelvis and renal calyces were clearly visible. No pelvic or ureteral notching was identified. An excretory phase image showed that the left proximal ureter was laterally displaced (Figure ). A retrograde pyelogram showed lobulated filling extravasate in the calyx at the lower pole of the left kidney (Figure ). A CT angiography showed the acute angle between the aorta (A) and superior mesenteric artery (S) of this patient (Figure ).\nThe operation was performed with the patient under general anesthesia. The patient was placed in a prostrate position with an 8 cm, oblique incision in the left lower quadrant as the site for autograft. The autograft was taken out through a retroperitoneal tunnel after the laparoscopic nephrectomy was completed. Muscular layers of the incision were sutured and the wound was temporarily protected by membrane in order to maintain the normal pressure of CO2 pneumoperitoneum.\nThen the patient was turned over and placed in a lateral decubitus position. A laparoscopic donor nephrectomy was conducted as described previously []. A 2 cm incision on the posterior axillary line across the twelfth rib costal margin in the left lumbar region was the first point. The peritoneum was pushed away by finger to expose the perirenal space. A balloon device for dilation of the retroperitoneum was inserted into the perirenal space, and 800 mL of air was pumped into the balloon to enlarge the perirenal space. After creating the pneumoperitoneum, a 25° laparoscope was inserted above the midaxillary line, across the spina iliaca. Additional working ports (5 mm) were placed under the anterior axillary line across the eleventh rib costal margin (Figure ). The kidney was exposed by opening Gerota's fascia. The adrenal, lumbar, and gonadal branches in the left renal vein were clipped and transected. Along the inferior pole of the kidney, the left ureter was clipped and transected until left ureter was exposed over 12 cm long. The renal pedicle was dissected to expose the left renal artery and renal veins. Then, the renal artery was clipped by hem-o-lock and transected. A similar maneuver was performed with the renal vein, wholly freeing the kidney.\nDonors received subcutaneous heparin after the operation. However, systemic anticoagulation was not employed before clamping the renal vessels. After the resected kidney was extracted through the oblique incision pre-prepared under the retroperitoneal tunnel, it was placed immediately on crushed ice and flushed with iced UW solution at 4°C. The kidney specimen was freed from all adjacent fatty tissues. Following reconstruction of the renal vessels, an autograft was transplanted retroperitoneally into the left iliac fossa. End-to-side anastomosis of the renal vein and the left external iliac vein using 5-0 polypropylene suture and end-to-end anastomosis of the renal artery and the divided left internal iliac artery using 6-0 polypropylene suture were completed, respectively. Ureteric anastomosis was carried out by formal antirefluxive implantation to the bladder as an extravesical onlay over a double J stent.\nFrom incision to final wound closure, the overall time of the surgery was nearly 200 minutes. The retroperitoneal laparoscopic nephrectomy took almost 80 minutes. The renal autotransplantation took 60 minutes. The hot ischemia took 5 minutes, while the cold ischemia took 60 minutes. Total blood loss, as calculated from anesthesiology charts, was less than 100 ml.\nTwo days after the operation, the patient's symptoms subsided. Preoperative and postoperative serum creatinine was 53 mmol/L and 55 mmol/L, respectively. The average length of stay was eight days. Three months after the operation, an ultrasonic inspection and intravenous pyelography studies revealed that the autograft functioned normally, while a nephrogram indicated a normal infusion by level of a glomerular filtration rate (GFR) of autograft. |
A 64-year-old male with a past medical history of benign prostatic hyperplasia and recently diagnosed paroxysmal atrial fibrillation presented to the hospital with a complaint of stools with bright red blood for the past 1 week. He was recently started on 5 mg of apixaban twice daily. He was hemodynamically stable with normal electrolytes and blood counts at presentation. The patient had no family history of any colorectal or genitourinary cancer. Colonoscopy with biopsy done shortly after admission demonstrated low-lying, moderately differentiated, adenocarcinoma of the rectum. A subsequent magnetic resonance imaging (MRI) of the pelvis showed T3N1 rectal cancer, with intact muscularis mucosa, and an enlarged presacral lymph node. A suspicious peripheral prostatic enlargement with a left iliac crest sclerotic bone lesion was incidentally found.\nGiven the incidental finding of prostatic enlargement and possible sclerotic bone lesion of the left iliac crest appearing quite suspicious, an FDG-PET scan was done and confirmed the primary rectal tumor with a presacral lymph node, but did not reveal any increased uptake in the prostate or iliac crest (). Prostate specific antigen (PSA) blood test was subsequently done that was elevated at 37 ng/mL. Due to high suspicion of a new concurrent primary prostate cancer, a biopsy was taken that revealed prostatic adenocarcinoma. PSMA-PET was then performed to identify any potential metastases from this primary site. PSMA-PET revealed increased uptake in the prostate, which was not previously detected by FDG-PET (). There was no uptake in the iliac crest (). After careful evaluation by the radiologist and nuclear medicine physician, it was determined that given the appearance of the lesion on MRI as calcification with well-defined margins and central necroses and the lack of uptake by PSMA-PET, which is highly sensitive for prostate metastases, the lesion likely represented an intraosseous iliac lipoma instead of a metastasis (). On disclosure of the radiologist’s diagnosis to the patient, along with an explanation that the differential diagnosis included an intraosseous lipoma as well as sclerotic prostate metastases that would likely present with irregular necrotic margins, the patient ultimately decided not to pursue biopsy understanding this was the most definitive way to rule out metastasis. As a result, primary rectal adenocarcinoma with metastasis to a single presacral lymph node, in addition to concurrent primary prostate adenocarcinoma, was the final diagnosis.\nThe patient was started on treatment for 2 primary perineal cancers, rectum as well as prostate. He received localized radiation therapy for rectal cancer and is scheduled for a repeat MRI of the pelvis for restaging of his rectal cancer postradiation. For his prostate adenocarcinoma, the decision was made to initiate antiandrogenic medication. If the patient qualifies and if an abdominoperineal resection is completed for the rectal cancer, he plans to undergo full-dose radiation therapy for prostate cancer in concurrence with his antiandrogen treatment. |
A 75-year-old man was referred owing to difficulty in performing clean intermittent self-catheterization (CISC) of the bladder. Approximately 3 years earlier, he had been found to have chronic urinary retention and a noncontractile detrusor on urodynamic evaluation. The precise etiology behind his detrusor dysfunction has not been clarified. He had subsequently been performing CISC 4 times daily. On occasions, he reported voiding a small amount of urine between CISCs. He was otherwise in excellent health with no significant medical comorbidities. Over a period of 2 months, he described increasing difficulty in passing the catheter. He felt obstructed at the level of the bladder neck, and on occasions was unable to pass the catheter all the way into the bladder. An attempt to improve the success of CISC with the use of a Coude tip catheter was not successful.\nCystoscopic examination revealed a small prostate with only slight lateral lobe protrusion. The posterior lip of the bladder neck was "high-riding" ().\nA concavity in the posterior bladder neck is indicated by the arrow in . This is consistent with indentation due to the catheter striking this area "end on." The ureteric orifices were noted to be particularly close to the bladder neck. Consequently, performing a standard bladder neck incision would potentially place the orifices at risk of injury.\nUsing the 120 W lithium triborate laser at a setting of 80 W power, the ridge of the bladder neck tissue was vaporized at the midline. This straightened out the prostatic urethra to allow for easier catheter insertion (). A total of 25 kJ of energy was used, and the laser time was 4 minutes.\nThere was no bleeding associated with the procedure. A 16 Ch latex Foley catheter was placed at the completion of the procedure. The catheter was removed 2 hours after the procedure and the patient was discharged. The patient recommenced CISC that afternoon. There was no bleeding in the postoperative period. The catheters now pass easily and there have been no further episodes of difficult CISC. Since this procedure, the patient has observed a significantly increased level of spontaneous urethral voiding and now finds it necessary to perform ISC only twice daily. |
A 25-year-old female Irish patient presented with recurrent episodes of rhabdomyolysis since childhood. The first episode occurred at the age of 22 months following a respiratory tract infection. At that time, her creatine kinase (CK) serum concentration was noted to be 250 000 U/L.\nThe family history indicated that both parents were well. However, two siblings (brother and sister) had sudden death at the age of 2 and 4 years more than 20 years ago following a short infectious illness with sudden deterioration over a period of hours. In both cases, the children became progressively weak with severe muscle pain and had evidence of rhabdomyolysis and myoglobinuria of uncertain etiology. Skeletal muscle histology and electron microscopy studies at postmortem evaluation were normal in both children, cardiac evaluation demonstrated dilated cardiomyopathy. Notes pertaining to their clinical episodes were not available. On first presentation, our patient had no hypoglycemia and no ketosis during the acute illness, but was initially treated as a possible long chain fatty acid oxidation defect due to the family history.\nThe patient had numerous subsequent admissions with similar presentations of extremely elevated CK concentration (1 000 000 U/L at the age of 9) associated with muscle pain. This was despite aggressive carbohydrate supplementation including nocturnal cornstarch. Her fat intake from food was continually restricted to approximately 40 g/d. Her total fat intake was supplemented with the use of MCT Oil (medium chain triglycerides supplement) and essential fatty acids in the form of walnut oil. Coenzyme Q10 at a dose of 100-200 mg daily was provided on an ongoing basis. The CK concentration was also raised between the episodes (500-2000 U/L). She was advised to limit her exercise to 20 minutes per day and have high-calorie drink prior to any physical activity.\nExtensive investigations were performed over the presenting years with the lack of a definite etiology. Serial urine organic acid profile and the acylcarnitine profiles were normal. A fibroblast fatty acid oxidation study showed normal myristate and palmitate oxidation studies in fibroblasts. Genetic analysis for fatty acid oxidation defect (LCHAD, MCAD, CPT I, and CPT II) and McArdle disease was uninformative.\nAt the age of 16 years, mitochondrial respiratory chain activity measured in a muscle biopsy was normal, however morphological findings, such as intramyocellular lipid, were compatible with lipin-1 deficiency (Figure ). Her echocardiogram and electrocardiogram did not show any abnormal findings.\nAt the age of 19 years, DNA sequence analysis of the LPIN1 gene (all coding exons and flanking intron boundaries corresponding to the canonical transcript variant NM_145693.3) revealed the presence of a common pathogenic intragenic deletion within the LPIN1 gene (c.2295-866_2410-30del1763) encompassing exon 18 (HGMD accession: CG085181). However, a second mutation could not be identified.\nSubsequently, the LPIN1 coding region was analyzed by reverse-transcriptase PCR (RT-PCR) from total RNA isolated from muscle tissue and conventional DNA sequencing (Figure A,B). In addition to transcripts lacking exon 18 or exons 18-19 (corresponding to the allele harboring the genomic exon 18 deletion), an alternative exon spliced in between exon 5 and exon 6 was detected in a significant proportion of transcripts (Figure C,D). Because this alternative exon, named exon 5a, corresponded to an alternatively spliced in-frame exon annotated only in an N-terminal LPIN1 transcript variant (NM_001261428.2), further targeted DNA-based sequencing was performed. Indeed, this identified a second heterozygous variant (NC_000002.11:g.11916284C>A), which was formally regarded a nonsense mutation introducing a premature stop-codon within exon 5a (which would correspond to NM_001261428.2:c.942C>A, NP_001248357.1:p.[Cys314*]; Figure ). RT-PCR of total RNA isolated from healthy skeletal muscle detected exon 5a also in transcripts containing the first (noncoding) exon of the canonical isoform (data not shown). However, because exon 5a is currently not annotated to be contained in this main reference transcript (NM_145693.3), further studies will be needed to clearly establish pathogenicity of the novel variant.\nSegregation analysis in the patient's family revealed that her father and younger sister are heterozygous for the common pathogenic intragenic deletion (NM_145693.3:c.2295-866_2410-30del1763), while her mother was a heterozygous carrier of the variant within the alternative exon. Thus, the results of these molecular genetic analyses were formally consistent with the clinical diagnosis of LPIN1-associated rhabdomyolysis, due to compound heterozygosity for a known pathogenic deletion and potentially pathogenic novel mutation.\nAt the age of 25 years, the patient presented with acute muscle pain and weakness following prolonged fasting and strenuous exercise. A rhabdomyolysis crisis was confirmed with a CK of 500 000 U/L. She was admitted to intensive care unit (ICU) for a 2 week period over which period she lost a significant amount of weight. During her critical illness, she received an intravenous infusion of 10% dextrose at 1.5 times maintenance with added electrolytes, sodium bicarbonate, morphine, and dexamethasone. Intravenous carnitine was also provided as the patient had previously observed this to be clinically beneficial. She was treated symptomatically, with shortened fasting periods, corn-starch at night and an “unwell dietary regimes” with a mild restriction of fat (40 g per day) and supplementation with MCT oil and walnut oil for essential fatty acids and Liquigen 5 g daily. On day 11, her CK was monitored 4 hourly and was 1248 U/L. On discharge from ICU, this patient had generalized muscle weakness, stiff lower limb muscles (gastrocnemius), and bilateral drop foot requiring orthotic splints. Muscle weakness gradually improved after months of rehabilitative physiotherapy. Her drop foot has improved somewhat (power from 0/5 to 3/5 for dorsiflexion and extensor hallicis longus over 1 year) with areas of altered sensation anterolaterally below the knees consistent with bilateral residual common peroneal neuropathies (CPN). Serial nerve conduction/electromyography studies demonstrated bilateral CPN palsies and a background generalized myopathy. The most likely cause of this patient's weakness was a critical care neuromyopathy, which has improved with time, in addition to CPN palsies related to significant weight loss while critically ill. However, we cannot exclude the possibility that both defects were related to her lipin-1 deficiency. While myopathy has been reported in a few cases, CPN damage is not a known association of this rare metabolic disorder. |
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