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A 52-year-old male patient without any relevant medical history visited the hospital with a symptom of recent lower abdominal pain. The patient had been smoking for 35 years and had a past history of heavy alcohol consumption. He received a lipoma surgery for a palpable mass pressure in the anterior neck portion 3 years ago. For the first time in his life, the patient received colonoscopy, which showed multiple polyps of different size and shape in the terminal ileum, the colon and the rectum (), especially hundreds in the rectum. Several biopsies were taken throughout the entire large bowel including the terminal ileum, and they were histopathologically confirmed as inflammatory and hyperplastic polyps. Physical examination revealed that the head circumference of the patient was 630 mm (for reference, the average and 99 percentile occipitofrontal circumferences of Korean males between 50 and 59 years old are 568 and 601 mm, respectively), suggesting macrocephaly. Also black pigmented cutaneous papules could be seen on the lips and multiple soft humps were found in the posterior neck, arm, and hip. The neck computed tomography (CT) revealed dense fat masses in the posterior neck as well as in both the submandibular and supraclavicular areas (). The upper GI endoscopy revealed multiple mucosal papillomas at the gingiva, oral cavity, soft palate, pharynx, and epiglottic areas (). There were also elevated flat lesions of whitish color in the esophagus, and biopsies showed they were squamous acanthosis. The multiple round gastric and duodenal polyps of various sizes were turned out to be hyperplasia ().\nConsidering all symptoms and the results of the examinations, CD was the most likely clinical diagnosis. Imaging studies of the brain, thyroid, chest, and abdomen as well as PTEN gene mutation test were performed. At thyroid ultrasonography (USG), an oval shaped isoechoic nodule of 1.2 cm in diameter was found in the isthmus. The fine needle aspiration (FNA) was consistent with nodular hyperplasia. The abdominal CT scan presented hemangiomas approximately 2 and 2.7 cm in size in both lobes of the liver, and a gallbladder stone of 7 mm in size. The brain magnetic resonance imaging (MRI) and the chest CT did not show any mass or abnormal finding. The laboratory test results were also within the normal ranges.\nThe examination of the genomic DNA showed a known mutation, c741dupA, in exon 7, which is registered in the Human Gene Mutation Database. It was predicted to lead the frameshift mutation that results in the formation of the premature stop codon (p. Pro248ThrfsX5) for the PTEN protein translocation.\nThe study was extended to the patient's daughters in order to carry out the adequate screening. The eldest daughter, who was 28-year-old at the moment, received a total thyroidectomy with left modified radical neck dissection and high dose 131I therapy (150 mCi) about a year ago. At that time, thyroid USG showed that there were three thyroid nodules and abnormally enlarged multiple lymph nodes in the left upper and mid jugulodigastric chain. FNA revealed papillary carcinoma with lymph node metastases. Biopsy of the surgical specimen showed multifocal intrathyroidal papillary carcinoma and Hashimoto's thyroiditis with the right perithyroidal and the left level IV and V regions lymph nodes metastases (). Breast USG showed multiple cysts of well-defined smooth borders which were less than 1 cm in diameter and a septated cystic mass which was 2.8 cm in diameter. Core needle biopsy of the 2.8 cm sized cystic mass at the outer area of the lower right quadrant revealed intraductal papilloma. She also had the head circumference of 610 mm in diameter, indicating macrocephaly (The average and 99 percentile occipitofrontal circumferences of Korean females aged between 25 and 29 years old are 552 and 583 mm, respectively). The black pigmented lip, the multiple papillomas of the gum and the soft tissue mass in the size of 9×6×5 cm on the right side of the posterior axillary area, were also found, and the excisional biopsy of the soft tissue mass revealed lipoma. GI tract examination, which was the first time in her life, showed multiple acanthosis lesions in the esophagus, numerous polyps in the second and third regions of duodenum, one fundic gland polyp, and the presence of a lot of inflammatory and hyperplastic polyps in the terminal ileum and the rectum.\nIn case of the second daughter who was 25-year-old at the moment, there were no suspicious results on the physical examination, GI tract endoscopy, USG of breast and thyroid, and PTEN mutation test was negative.
A 68-year-old woman presented with dizziness and headache. She had no neurological deficits, such as paralysis or sensory disturbances. Magnetic resonance imaging (MRI) revealed a 9 mm, unruptured, thrombosed aneurysm anterior to the brainstem []. Since the patient was asymptomatic, she was followed up on an outpatient basis. However, MRI at age 70 revealed increased aneurysm size (14 mm) and brainstem mass effect [ and ]. Three-dimensional digital subtraction angiography revealed a right proximal AICA aneurysm []. The AICA supplied the cerebellar hemisphere and did not anastomose with the posterior inferior cerebellar artery. Due to increased risk of rupture and mass effect, we decided to perform surgical intervention. Endovascular treatment was excluded because the aneurysm was of fusiform type. Parent artery occlusion posed a risk of cerebral infarction. Posterior transpetrosal approach was needed to perform complete trapping of the aneurysm. However, the approach could be too invasive to perform for asymptomatic patients. We decided to perform distal clipping of the aneurysm to combine its blind end with the occipital artery (OA)-AICA bypass to avoid cerebral infarction. Transcondylar fossa approach was selected because it is minimally invasive and makes reaching the distal end of the aneurysm and AICA easier. We did not approach the anterior brainstem. The distal AICA arose from the aneurysm body []; therefore, we clipped the AICA just distal to the aneurysm after the OA-AICA bypass [-]. We aimed to make the aneurysm thrombosed. Postoperative MRI demonstrated sporadic, asymptomatic cerebellar infarctions []. Indocyanine green video angiography showing the adequate flow of the bypass []. However, the bypass patency was not clear on MRI after the treatment. We considered the possibility that the bypass was not depicted due to an artifact or that the bypass was thrombosed due to lack of demand. The patient was discharged with a modified Rankin scale score of 1. Six months postoperatively, the aneurysm growth slowed down; however, its size was 15 mm, and the brainstem mass effect persisted [F igures 4a and b]. Therefore, we considered secondary treatments, such as parent artery occlusion with endovascular technique. However, the aneurysm size decreased without additive treatments. Twelve months postoperatively, the aneurysm size was 12 mm, and the brainstem mass effect disappeared [ and ]. Thirty-three months after surgery, the aneurysm had shrunk, and the patsient had no neurological deficits [ and ].
Patient: 57-year-old female Chief complaint: Mental change and spasm Current medical history: Above 57-year-old female patient didn't have an unusual medical history in the past and visited a neurosurgical clinic in another hospital a month before visiting the hospital because she couldn't open both of her hands. With a consideration of Parkinson's disease, she was prescribed with levodopa and then the course was going to be observed in an outpatient clinic. However, the symptom became worse after she started taking levodopa and then she visited the outpatient clinic of the hospital again in a week. The course was going to be examined after stopping to take levodopa but the symptom seemed to get worse gradually even if she stopped taking levodopa. After 5 days, the patient visited an emergency room in the hospital and brain magnetic resonance (MR), brain computed tomography (CT), and C-spine MR were performed; since there was no special opinion with an exception of spinal stenosis, she was discharged from the hospital again. But, the symptom was still lasted; she had been found as tumbling and lying down on the floor a week before visiting the hospital. Since the patient still had the symptoms, she wanted to visit the other hospital. However, she had spasm and a mental change in a car while driving to the other hospital to visit. Then she visited an emergency room in our hospital; she showed low serum calcium, 5.3 mg/dL (reference: 8.2-10.2), in the blood test at that time so she was hospitalized in division of endocrinology and metabolism, department of internal medicine. Medical history in the past: The patient graduated an elementary school which was not well-educated. From the medical history in the past, no unusual medical history was found in the patient with an exception of taking antihypertensives due to the hypertension that was diagnosed 2 years ago. Family history: There was no unusual disease or abnormality in the family history
In January 2015, a 52-year old woman with obesity and hypertension was admitted to the cardiac intensive care unit of a hospital in Northern Italy for acute dyspnea and chest pain. Computed tomography angiography revealed filling defects affecting the right and left branches of the pulmonary artery suggestive of pulmonary embolism (PE). She was initially treated with intravenous heparin sodium and bridged to warfarin as a long-term anticoagulant. Before the hospital discharge she was prescribed a vitamin K free diet reported in Table . Although the general recommendation is to maintain a stable and consistent intake of Vitamin K, in the clinical practice patients are suggested to avoid food high in vitamin K and they are given general dietetic guidelines.\nDuring her first month as an outpatient it was very difficult to get her INR into the therapeutic range. Her family doctor recognized that poor nutrition is an important factor in establishing therapeutic levels and she was referred to our Nutritional Unit outpatient service (NU) for a nutritional assessment and specific medical nutrition therapy. Figure shows the timeline of present illness, intervention and follow-up.\nShe reported a long-term history of obesity and hypertension. She denied allergies and cigarette smoking and reported a sedentary lifestyle with no physical activity. The patient was nulliparous and had been on oral contraceptive therapy since the age of 25. The therapy was discontinued by her physicians after her PE. At the time of the examination she was on nebivolol, alprazolam and warfarin adjusted for INR, as well as vitamin B12 and folate supplementation (400 μg). Her family history was negative for thrombosis and cardiovascular diseases.\nThe patient reported that she began struggling with obesity during her childhood. She began adulthood at a weight of 90 kg (BMI 35.0 kg/m2). Maximum and minimum weight reported by the patient were 117.5 kg (BMI 45.9 kg/m2) and 69 kg (BMI 27.0 kg/m2) respectively at 51 and 26 years of age. Soon after reaching her peak weight she started a hypocaloric diet as suggested by her family doctor, Table .\nAnthropometrical, bioelectrical and clinical findings at physical examination are showed in Table with biochemical analyses. The patient’s BMI was greater than 34.99 Kg/m2, class II obesity according to WHO classification []. Fat mass was estimated by bioelectrical impedance analysis (BIA) using a BIA 101 Akern s.r.l. (Italy). The exam, performed under standardized conditions [] revealed an important increase in fat mass although a normal state of hydration. The waist-to-hip ratio indicates an android fat distribution. Evidence of increased visceral fat depot is shown by waist circumference measurement.\nResting energy expenditure (REE) has been estimated by means of Mifflin predictive equations [], which is more likely than the other known equations to estimate REE to within 10 % [].\nBefore the PE, the patient was following the diet reported in Table , which was prescribed by her family doctor. Total energy and nutrient intakes were analyzed by trained dietitians using dedicated software developed by our Nutrition Unit incorporating “Food Composition Database for Epidemiological Studies in Italy” by Gnagnarella P, Salvini S, Parpinel M. (Version 1.2015 Website ).\nThe diet followed by the patient before the PE was a low-calorie diet that did not satisfy the dietary recommendations for protein and micronutrient intake (LARN 2014) [].\nThe nutrient composition of the diet prescribed to the patient after the PE upon hospital discharge is reported in Table .\nVitamin K free diets typically are lacking in vegetables, with a consequent insufficient dietary fiber and folate intake. Protein intake prescribed to the patient was far lower than any standard recommendation (less than 0.33 g/ kg of body weight) while the total fat and in particular the saturated ones were higher than the standard recommendations (LARN 2014 and ESC 2012 []).\nThe patient’s laboratory values at the time of her first examination in our NU are shown in Table . She had high levels of homocysteine and low prealbumin. She also was vitamin D deficient as can be seen in patients with obesity [].\nBoth folate and vitamin B12 concentrations levels were within the accepted normal ranges, likely due to her supplementation and not her dietary intake.\nAfter the nutritional assessment, a balanced, low-calorie diet was prescribed, with particular attention to sodium and soluble carbohydrates intake. The protein intake prescribed was equivalent to 0.8 g/Kg of actual body weight. The nutritional composition of the diet prescribed at our NU is shown in Table .\nIn order to avoid a sharp change in vitamin K intake the starting vitamin K intake prescribed was low (57.88 μg per day). Gradually her vitamin K intake was increased to 150 μg per day. Folate supplementation was prescribed (400 μg per day) as well as vitamin D supplementation (300,000 IU every 3 months for 9 months) [].\nThe benefit of having her seen in the NU was that our staff was able to take the appropriate amount of time to counsel the patient and to ensure she understood the dietary guidelines and risk of complications in order to transform the acquired knowledge into behavior change and to achieve greater adherence.\nThe patient’s INR values were tested on a regular base. As shown in Table , the INR values were therapeutic and remained constant immediately following initiation of the prescribed diet.
A 72-year-old man visited us with a chief complaint of intermittent intractable pain occurring in the right chest. On past history, the patient sustained a fracture of the left femur. At department of orthopedic surgery of our medical institution, the patient was hospitalized to take the surgery. At the time of admission, due to the presentation of varicella zoster and its resulting pain extensivly occurring along the right 6-8th dermatomes, the patient received drug treatments at department of dermatology. During the hospitalization treatments, the symptoms were improved. Two months following the discharge, the patient complained of severe pain occurring in the corresponding dermatomes. Then, the patient visited department of dermatology and was referred to a pain clinic. On physical examination, the patient complained of allodynia, hyperesthesia and hyperalgesia where there was a concurrent presence of the scar tissue formed in the right 6th, 7th and 8th dermatomes. Besides, the patient also complained of sleep disturbance. At the time of admission, VAS was 8/10 points. On routine lab test, there were no notable findings that impede the treatments for diabetes mellitus, blood coaguation disorder and hemostasis abnormality. For the treatment, a needle was placed in the thoracic 8th and 9th multifidus muscle and thoracic 7th and 8th, 8th and 9th and 9th and 10th tissue around the vertebral facet. Four weeks following the treatment, VAS was decreased from 8 points at the time of admission to 2 points. An examination was performed two weeks later, and this showed that VAS was maintained at 2 points and this led to a closure of the treatment.\nIn the above patients, the treatment period and frequency were four weeks and a total of six times. Based on the characteristics of patients, the age, sex, disease duration, the pattern of pain and the affected dermatomes showed the different characteristics (). Following the treatment, VAS improvement rate (VAS improvement rate (%)= VAS prior to treatment - VAS during evaluation × 100 ) was almost consistent ().
A 53-year-old man came to the Otorhinolaryngology department as an outpatient in September 2011 for the chief complaint of clicking tinnitus on the left. The patient had a past medical history of arrhythmia and heart valve disease but there was no history of trauma or surgery which could have led to tinnitus. Since July 2008, he had received rehabilitation treatment due to cerebral infarction in the middle cerebral artery zone. The clicking tinnitus started suddenly 3 weeks before he visited the hospital, mostly during mastication and when opening and closing his mouth. During the 3 weeks, the clicking tinnitus happened without strength change and without other otologic symptoms such as otalgia and otorrhea. In the otoscopic examination, the tympanic membrane was normal but there was slight protrusion of the soft tissue, originating from the anterior wall of the left EAC. This protrusion was observed when the mouth was closed and if it was open, the protrusion retracted (). The clicking sound that was heard in the left ear of the patient was also heard by the observer when he put a stethoscope on the EAC of the patient when the mouth was opened and closed.\nThe patient did not feel uncomfortable in his daily life from the clicking tinnitus, and the Tinnitus Handicap Inventory (THI) score was 18. According to the pure tone audiometry, the air conduction average threshold value (4 division method) was 23 dB on the right and 22 dB on the left while the average threshold value from bone conduction audiometry was 21 dB and 20 dB respectively. On the impedance test, both sides were A type. We conducted a tinnitogram but the patient complained of just clicking and we failed to find the exact strength and frequency.\nAccording to his otologic history, he had clicking at the time of mastication. Pure tone audiometry also failed to detect sensorineural or conductive hearing loss while the otoscopy found an anterior wall protrusion at the left EAC, and so TMJ herniation was suspected. Therefore, we conducted TMJ X-ray and temporal computed tomography. From the TMJ X-ray, we could see a left temporomandibular joint herniation at the time of mastication (), and the temporal bone computed tomography detected a 6 mm bony defect in the left EAC anterior wall and subsequent soft tissue herniation (). At the right EAC, there was no bony defect or other unusual findings. The left clicking tinnitus complained by the patient was revealed to occur from TMJ tissue herniation into the EAC at the time of mastication. Therefore, we considered surgical treatment for the left EAC anterior wall defect but the patient refused surgery because he felt uncomfortable with surgical treatment as he was receiving rehabilitation for cerebral infarction, and furthermore, he did not suffer much inconvenience from the clicking tinnitus. Thus, the authors treated him with an anxiolytic and muscle relaxant for 2 weeks, after which we could not see any change in loudness of the tinnitus (according to the patient) and emotional reaction. Now, the authors are observing the progress of the clicking tinnitus.
A 64-year-old male referred to our hospital 4 weeks after having a laparoscopic cholecystectomy complicated by a common bile duct injury. He had undergone a difficult laparoscopic cholecystectomy with intraoperative bile duct injury suspected at the end of the procedure at a peripheral hospital. The surgeon inserted a drain in the surgical bed at the time of the surgery. Postoperatively, the patient was started on piperacillin and tazobactam, and underwent an endoscopic retrograde cholangiopancreatography (ERCP), which confirmed the presence of a common bile duct injury. A biliary stent was placed. The patient continued to have daily bile drainage ranging from 100 to 350 ml per day.\nUpon presentation to our center, the patient was febrile (38.9°C) and complained of a left upper limb pain and swelling. The surgical drain was draining a mixture of bile and blood. His WBC count was 14 × 109/l, total bilirubin 35 µmol/l and the direct bilirubin 21 µmol/l. A duplex ultrasound of the upper limb revealed a left brachial vein thrombosis. Hematology was consulted, and a diagnosis of acute upper limb venous thrombosis was established. Owing to the presence of blood in the drain, he was kept on the maximum prophylactic dose of unfractionated heparin. A CT angiography of the abdomen showed two collections, one at the surgical bed near the drain, and the other was subcapsular below the left lateral lobe of the liver, as well as an aneurysm of the replaced right hepatic artery with an active bleeding blush (Fig. ). The patient was immediately referred for an angiography, which confirmed the CT scan findings (Fig. ). An arterial stent was inserted at the location of the aneurysm (Fig. ), and a pigtail drain was inserted to drain any residual collection. An ERCP was also performed, which revealed a Strasberg Class D injury, and a plastic biliary stent was inserted. After the angio-stent insertion and stabilization of the patient, heparin infusion was started. Five days later he developed hematemesis and melena with a significant drop in his Hb to 2 g/l, and his total bilirubin became 183 μmol/l of which 91 μmol/l is direct. A gastroscopy was performed and showed hemobilia (bleeding from the ampulla of Vater). Subsequent angiography demonstrated a leak of contrast just above the arterial stent; hence, a further stent was placed to cover that area of the aneurysm. Similar symptoms reoccurred a week later, and a new angiography showed a new aneurysm from the left proper hepatic artery. A percutaneous thrombin injection of the aneurysm was performed as the bleeding branch was unreached via direct angiography and was filling in retrograde perfusion. During recovery a chest spiral CT was performed, which revealed the diagnosis of a bilateral segmental pulmonary embolism. Heparin infusion with low targets of partial thromboplastin time of 50–60 was started.\nOn Day 7, the patient bled again from the same aneurysm of the left hepatic artery. A repeated angiography revealed the bleeding with a reduced flow in the stented, replaced right hepatic artery (Fig. ). The active bleeding was stopped using gel-foam embolization of the two branches of the left hepatic artery (Fig. ) with a decision to embolize the whole left hepatic artery if bleeding did not stop while holding the heparin infusion. The patient's liver function was preserved, and the bleeding stopped despite anticoagulation.
A 50-year-old male was brought to the emergency department after he jumped from a 5-meter bridge in an attempted suicide and fell on the hard concrete below. Upon admission, the patient was agitated, disoriented, and in intense respiratory distress. Examination revealted that the patient’s airway was clear, but there was a bilateral absence of breath sounds and hyperresonance on percussion. The patient was hemodynamically stable. He was intubated due to respiratory failure. Bilateral chest tubes were inserted based on a high clinical suspicion of pneumothorax. Thereafter, the patient developed a large subcutaneous emphysema, despite the fact that the chest tubes were functioning with his severe air leakage. Past medical history was unremarkable with no previous formal depression diagnosis. A social history check showed daily marijuana and tobacco use.\nThe patient was sent for a head, neck, thorax, abdomen, and pelvis CT scan. The scan revealed giant bullous emphysema on the superior lobes bilaterally, right pneumothorax with a collapsed lung, along with multiple rib fractures, and lung emphysema (, ). A hip dislocation was detected, and closed reduction was performed.\nThe patient was sent to the ICU, where he improved clinically after conservative treatment with continuous negative pressure suction using a 20 cm water column. He was extubated after 48 h, with persistence of the air leakage on both chest tubes.\nOn the day following extubation, he developed hypoxia associated with disorientation and agitation and had to be intubated again. Hypoxia was postulated from pulmonary contusion and ventilator-associated pneumonia worsening his already baseline compromised lung. He developed sepsis, and subsequently acute kidney injury with the need for dialysis.\nDuring the course of 5 days, the patient presented hypoxia and a severe mixed metabolic and respiratory acidosis, despite the use vancomycin and piperacillin/tazobactam. The treatment with bilateral chest tubes associated with continuous negative pressure aspiration did not correct the air leakage, which caused an important lost of tidal volumes on the ventilator. Changes in ventilator parameters (increases in PEEP, tidal volumes, etc.) did not improve his oxygenation or decrease his pCO2. In an effort to expand his lungs and improve his ventilatory function, we decided to perform a bilateral bullectomy.\nAs the patient had poor surgical status, only a right bullectomy was performed 8 days after the trauma. The right side was chosen over the left, because it showed more compression and a larger residual, healthier parenchyma. After surgery, the right side fistula was resolved (). Four days after surgery, the patient developed a right-sided empyema, right lung incarceration, and blood clots in the chest tube (). In order to resolve these issues, videothoracoscopy and decortication of the right lung were performed, which resulted in the resolution of these problems () and improvement in ventilatory parameters with conservative treatment for the left lung. Notwithstanding antibiotic treatment and successful surgical interventions, the patient died on the 25th day of hospitalization due to infectious complications.
A 25-year-old male, from the farming community, presented with a lump in the abdomen since 3 years, which was gradually increasing in size but with a rapid increase in size since 4 months. The patient was married with two children and there was no history of erectile dysfunction. Clinical examination showed fullness of abdomen in umbilical, right iliac, and left lumbar regions, both the testes were absent in the scrotum. An ultrasound examination revealed a large mixed echogenic mass measuring 15 × 10 cm located in the right side of the pelvis. The clinical diagnosis was a tumor in the undescended abdominal testis. Serum levels of human chorionic gonadotropin and alpha-fetoprotein were normal.\nPer-operatively there was a large mass in the right side of the pelvis adherent to the pelvic organs along with a rudimentary uterus and one side gonad. The specimens were sent to the department of Pathology.\nGross examination showed a rudimentary uterus of size 6 × 2 × 1 cm with one side gonad of 2.5 × 1.5 cm, which was gray brown and soft to cut, along with two tubular structures. The other side mass was 17 × 13 × 5 cm, cut section of which revealed a tumor that was predominantly solid with dark brown and gray white areas [].\nMicroscopic examination of the uterus showed well-developed endocervical tissue with thinned out ectocervix, endometrial glands in primitive stroma, and normal myometrial tissue. The gonad with two tubular structures showed histologic picture of a prepubertal testis with no spermatogenesis, a fallopian tube, and epididymis.\nThe tumor was composed of groups of cells with clear cytoplasm, centrally placed hyperchromatic nucleus, and prominent nucleoli. These groups of cells were separated by thin fibrous septae with lymphocytic infiltrates and also large areas of necrosis. Focal areas showed syncytiotrophoblast type of giant cells. The histologic picture was that of a classical seminoma []. Internal iliac lymph nodes and omentum showed secondary deposits.
A 55-year-old woman presented with several months of diplopia and complete ophthalmoplegia of the right eye. MRI revealed a 6.9 cm x 6.6 cm x 6.9 cm destructive lesion involving the sphenoid bone and extending into the sphenoid sinus, cavernous sinus, suprasellar cistern, and posterior fossa most consistent with a chondrosarcoma (Figure ).\nThe patient was taken to the operating room for an endoscopic endonasal resection of this lesion and provided informed consent for use of her case for academic and research purposes. A lumbar drain was placed preoperatively and kept clamped throughout the procedure. An expanded endoscopic approach was performed with wide maxillary antrostomy and ethmoidectomy. A nasal septal flap was harvested and rotated into the maxillary sinus. The sphenoid sinus was opened and tumor was mobilized away from the sinus and dissected from the sella. Tumor was followed inferior and dissected off the clivus and superiorly off the suprasellar arachnoid. After tumor mobilization and debulking, the dura of the clivus was noted to be incompetent and opened to allow for mobilization of tumor from the arachnoid of the posterior fossa. Attention was then turned to debulking tumor from the lateral gutter of the sella, cavernous sinus, and middle fossa with the use of angled endoscopes and instruments. At the conclusion of the resection, persistent noncatastrophic arterial bleeding was noted near the right ICA, behind which the most lateral dissection had been performed in an attempt to resect the middle fossa component. No clear point of extravasation was identified, however, the ICA was not fully exposed. Although the bleeding could be controlled with direct focal packing of hemostatic material, there was no way to provide durable hemostasis and thus the area was packed and the patient was taken for an emergent angiogram intending to come back to the operating room for completion of the closure.\nThe angiogram showed a small pseudoaneurysm along a branch arising from the ICA likely proximal to the intradural segment although the exact branch was difficult to identify as the course of the ICA had been significantly altered by the presence of the tumor (Figure ,B). Given good collateral flow through the anterior communicating artery and posterior communicating artery (PCom), a decision was made to proceed with carotid sacrifice with coil embolization of the right ICA at the ophthalmic segment (Figure ). Subsequent angiographic runs confirmed filling of the ophthalmic branch through ethmoidal branches of the external carotid artery.\nThe patient was transferred back to the operating room where the nasal packing and hemostatic material was removed with gentle irrigation. The sella was filled with an abdominal fat graft and the wound closed with the previously harvested nasal septal flap and the nose packed with NasoPore (Stryker, Kalamazoo, MI, USA) and silastic splints. The patient was transferred to the intensive care unit and maintained on vasopressors for five days to ensure adequate perfusion. Postoperative MRI showed near total resection with a small residual along the most anterior and lateral aspect of the middle fossa tumor component. Final pathology was chondrosarcoma, WHO grade II. She was discharged home in good condition and subsequently underwent fractionated radiotherapy with intensity-modulated radiation therapy (IMRT) for a total of 66 Gy in 33 fractions. She also underwent strabismus surgery to help accommodate for her ophthalmoplegia and on her last clinic visit two years after surgery was doing well with stable tumor on imaging and resolved cranial nerve deficits (Figure ).
An 89-year-old Japanese woman with a 1-month history of stoma bleeding was referred to our hospital. She had undergone a transverse loop colostomy 35 years previously for an iatrogenic colon perforation that occurred during left ureteral lithotomy. Upon physical examination, the patient was found to have a hard nodule at the colostomy site, measuring 3 cm (Figure ). A biopsy of the nodule suggested adenocarcinoma. Laboratory data revealed anemia and renal dysfunction. Serum carcinoembryonic antigen and carbohydrate antigen 19-9 levels were not elevated. Staging computed tomography showed a 3-cm tumor adjacent to the stoma with no invasion into the abdominal muscle. There was no evidence of metastatic disease. Colonoscopy via the stoma revealed no other lesions in the colon or rectum. On the basis of our findings, the preoperative diagnosis of the stoma tumor was transverse colon cancer (T2, N0, M0, stage I). We concluded that colectomy and repositioning of the colostomy site were appropriate for this patient. Although we thought stoma closure was possible, the patient and her family were opposed to this strategy because they were concerned about anal function.\nUnder general anesthesia, a laparotomy was performed via a peristomal incision with 5-mm skin margins, and the tumor was covered using a surgical glove to avoid tumor seeding. Adhesion around the stoma was not strong, and there was no evidence of peritoneal dissemination. Only pericolic lymph nodes were dissected. The colon was separated from the tumor by 5-cm margins, and the specimen was removed en bloc (Figure ). An end colostomy was constructed to a new site on the right side of the abdomen. The deficit in the abdominal wall was repaired, and the skin was closed via a purse-string suture. The operative time was 102 min, and blood loss was 52 mL. Macroscopic examination showed complete excision of the tumor with clear margins (Figure ).\nThe patient was discharged 8 days after surgery. Her postoperative course was uneventful, and she recovered without any complications. One year and five months after surgery, there is no evidence of recurrence.\nMicroscopic examination of the tumor showed moderately differentiated adenocarcinoma mixed with well-differentiated adenocarcinoma (Figure ). The tumor extended into the proper muscle layer and directly invaded the skin. There was no lymph node metastasis. The final diagnosis of the stoma tumor was transverse colon cancer (T2, N0, M0, stage I).\nThe study protocol was approved by the ethics committee of Showa University Northern Yokohama Hospital. The study was conducted according to the Declaration of Helsinki.
A 58-year-old man was referred to our institution for a suspicious mass within the neck of the pancreas with a dilated pancreatic duct and evidence of chronic pancreatitis identified by abdominal computed tomography (CT). An endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreatography (ERCP) identified adult diagnosed pancreatic divisum with a 2.5 cm mass within the neck of the pancreas and pancreatic duct stricture. A fine needle aspiration (FNA) of this area showed only chronic inflammation and a CA 19-9 level returned within normal limits. Review at our institutional multidisciplinary conference led to recommendations for surgical resection, and ten months after his initial presenting symptoms, the patient underwent a pylorus-preserving pancreaticoduodenectomy. The final pathology revealed a specimen with chronic pancreatitis and one small focus of invasive carcinoma with perineural invasion. No tumor was identified in twenty-one lymph nodes. All surgical margins were negative for tumor. The patient made an uneventful recovery and afterward elected to forego any further adjuvant treatments.\nThe patient did well while undergoing routine surveillance until approximately 15 months later when he began to experience mild to moderate abdominal discomfort and nausea. A repeat CT scan of the abdomen revealed a mass at the site of the previous surgical resection with narrowing of the PV confluence and extensive lymphadenopathy at the root of the mesentery. An ultrasound-guided biopsy of this area confirmed recurrent well-differentiated adenocarcinoma, and the patient began concurrent chemoradiotherapy with continuous infusion 5-fluorouracil and external beam radiation therapy (EBRT) for a total dose of 54 Gy.\nOn follow-up the patient developed upper gastrointestinal (GI) bleeding, requiring multiple blood transfusions, and on upper endoscopy was found to be due to varices of the proximal gastric wall, which appeared to extended through the anastamosis into the efferent jejunal limb. At the time of endoscopy there was no evidence of active bleeding, and so we elected to proceed with medical management for the patient to include β-blockers and intravenous octreotide.\nDuring the same hospitalization the patient again developed variceal bleeding. We found that the recurrent pancreatic tumor had caused a high-grade neoplastic obstruction of the SV at the PV confluence and as a consequence resulted in mesenteric hypertension and gastric and small bowel varices that were refractory to our medical management (Figures , , and ). Because of this treatment dilemma we sought the expertise of our interventional radiology department who confirmed the SV obstruction and dilated varices by transhepatic portography (). They were then able to successfully treat the patient by transhepatic endovascular stenting of the SV obstruction with a 10 mm × 42 mm wall stent followed by an 8 mm balloon dilation. Repeat splenic venography demonstrated the PV confluence to be patent with good flow across the stent along with dramatic decompression of the collateral gastric and small bowel varices ().\nImmediately after the procedure the patient had no additional episodes of melena or hematemesis, and no further blood transfusions were required. Seven days following the procedure the patient was discharged home from the hospital tolerating an advancing diet. He died 4 months later from natural progression of the malignancy. During this time, he did not experience any additional episodes of GI bleeding and remained relatively comfortable.
A 57-year-old male patient with lung carcinoma was referred for 99m Tc MDP skeletal scintigraphy for detection of skeletal metastases. Three hour after injection of radiotracer, the whole-body bone scan was performed which revealed a photopenic defect in vertex of skull in anterior projection []. In view of history of lung malignancy, it was initially diagnosed as lytic lesion representing skeletal metastases. However, on careful evaluation of whole-body image in posterior projection, no such defect was noted []. On further evaluation, we observed increased tracer uptake in the edge of static chest and pelvis images in anterior projections [Figure and ]. However, again spot views of same regions in posterior projections were within normal limits [Figure and ]. These discrepancies caught our attention, and we suspected these to be artifactual. Suspecting nonuniformity, we moved the patient toward the central field of view of the detector to eliminate the artifact from images and noticed no such defect in the skull [Figure and ]. We immediately performed the intrinsic uniformity test with 99m Tc point source for the anterior detector, which showed the increased value with a similar pattern of artifact as appeared in patient images toward useful field of view (UFOV) []. Surprisingly, the artifact did not appear in the daily quality control test performed in the morning. We informed the service engineer about the incident. The engineer found malfunctioning of two PMTs toward Y axis in the anterior head of the gamma camera. On the next day, service engineer opened anterior head of gamma camera and replaced the malfunctioning PMTs located in the border. After the service engineers rebuilt the head, tuning was performed to ensure that PMTs voltage gain was within limits. After completion of tuning, intrinsic uniformity test was performed to ensure all the values are within accepted limits []. After correcting the mistake, another skeletal scintigraphy was performed which was within normal limit and show no such defects in the edge of spot views at anterior projections [].
Eight years ago, a 31-year-old male patient presented with symptoms of dull back pain, insidious in onset, felt in the lower lumbar and gluteal region, accompanied by morning stiffness which initially improved with activity. Within a few months, the problems became persistent and bilateral. With the passage of time, he experienced neck pain and stiffness with restricted mobility. He was diagnosed with having AS based on available criteria. Then, after 8 years, at the age of 39 years, he experienced weakness of all four limbs which started gradually and was progressive for the past 1 year. It started with the right upper limb followed by involvement of the right lower limb. Both left upper and lower limb were also involved sequentially within a few months with progressive wasting and atrophy of muscles incapacitating him to perform his daily works. There was also a history of slurring of speech and occasional choking along with flickering movement of the muscle. Bladder and bowel functions were normal. The patient is nondiabetic but hypertensive. There was no history of chronic obstructive airway disease, tuberculosis, or trauma. There was no history of fever, abdominal pain, convulsion, skin changes, diminished vision, ptosis, redness of the eye, or any episode of bloody diarrhea. He denied any substance or illicit drugs abuse. Family history was insignificant.\nOn clinical examination, general survey was unremarkable. Higher function and cranial nerve examinations were normal. The neck was stiff with restricted movements in all directions. Other signs of meningeal irritations were absent. The cranium was normal, but the spine was curved with concavity anteriorly. In motor system, there was gross atrophy of muscles of the shoulder and pelvic girdles and distal limb muscles without any sensory loss. There was gross atrophy of the tongue, and fasciculation was also seen. The tone was increased. Power was 3/5 in proximal group of muscles in all four limbs with severe weakness in distal muscles with bilateral claw hands []. Deep tendon reflexes in both the upper and lower limb were exaggerated bilaterally. Plantar reflex was extensor bilaterally. Glabellar tap and pout reflexes were present. Cerebellar signs were absent.\nMovements were restricted in all directions in cervical, dorsal, and lumbar spines. Modified Schober test was positive. Small joints of hands were swollen without any signs of inflammation. There was clawing of fingers and toes. No extraarticular manifestation was present.\nInvestigations of routine blood were not remarkable apart from high erythrocyte sedimentation rate of 72 mm. Liver function and lipid profiles were normal. C-reactive protein was positive. Rheumatoid factor and anti-cyclic citrullinated peptide both were negative. Urine and stool analysis were normal. Both antithyroglobulin and anti-microsomal antibodies along with thyroid profiles were in normal range. Analysis of human leukocyte antigen-B27 (HLA-B27) was negative. Electrocardiography showed normal sinus rhythm. Echocardiogram was normal. Electromyography and nerve conduction velocity study of all four limbs revealed chronic diffuse alternating hemiplegia of childhood disease with secondary motor axonopathy. Dual-energy X-ray absorptiometry scan of hip and left forearm showed osteopenia (T score is 1.3 and 1.2, respectively) and lumbosacral spine was normal (T score 0.3).\nChest X-ray showed increased reticular markings. X-ray of the cervical and dorsolumbar spine revealed gross ligamentous calcification of both anterior and intraspinal ligaments along with the loss of cervical and lumbar lordosis []. Shoulder and bilateral knee joint X-rays both were normal. Hip X-ray showed left hip arthritis. Juxtaarticular osteopenia was seen in X-ray hand and wrist. X-ray of SI joint revealed mild sclerosis bilaterally. Magnetic resonance imaging of the spine revealed ankylosis of lower thoracolumbar vertebra with calcified intervertebral discs. Discs were fused in all direction with height reduction. No evidence of cord compression and significant compromise of neural foramina were present. Based on the clinical presentations, examinations, and radiological features, this patient was diagnosed with a case of seronegative spondyloarthropathy, i.e., AS and amyotrophic lateral sclerosis.\nHe was put on methotrexate 10 mg once a week with folic acid and calcium supplementation. He was already on levothyroxine 50 μg once a day plus antihypertensive in the form of amlodipine and losartan combination. At his last turn up in outpatient, bath ankylosing spondylitis disease activity index score remains the same.
A 20-year-old unmarried girl was admitted with primary complaints of lower back pain, progressive proximal muscle weakness of lower limbs for the past 4 ½ years and bony pain in both thighs and in pelvic region for the past 4 years. Four years ago she had one episode of sudden onset flaccid weakness of all four limbs associated with neck muscle weakness. At that time, there was no history of seizures, change in sensorium, and bladder or bowel involvement. She recovered completely within 24 hours after treatment with intravenous fluids. Her symptoms, however, progressively worsened and for the past 2 years, she has started having difficulty in walking. She denied any history of weakness or bony pain in the upper limbs. She was treated with oral calcium and injectable vitamin D without any relief of her symptoms. In January 2010, she developed spontaneous pain in her right thigh without any history of trauma and was diagnosed to have a fracture shaft of right femur and was managed conservatively. In the 1st week of July 2010, she developed another fracture of the left femur diaphysis after trivial trauma. At this juncture she consulted an orthopedic surgeon, who suspected MBD and referred her to the Endocrinology department for further investigations. She gave a history of grittiness and foreign body sensation in both eyes and also history of dryness of mouth for the past 2-3 years. She had achieved menarche at age of 15 years and gave a history of regular menstrual periods. There was no family history of a similar illness. On examination she was found to be short statured. Her supine length was 140 cm (<5th centile on K. N. Agarwal growth chart), against target height of 157 cm (50th centile on K. N. Agarwal growth chart). Muscle power in the upper limbs was normal, while proximal muscle power in lower limbs was 4/5, with normal distal power. Sensory examination was normal. Investigations are summarized in . Her bone scan and radiograph pelvis are shown in Figures and .\nA provisional diagnosis of MBD secondary to distal RTA was made. There was also evidence of proximal tubular involvement as suggested by generalized aminoaciduria and albuminuria. Further investigations were carried out to find out the etiology of RTA. The significant findings on working up for etiology were a positive rheumatoid factor and ANA. Schirmer's test was 5 mm in right eye and 13 mm in left eye. Both anti Ro and anti La antibodies were strongly positive. Sialogram parotid gland demonstrated bilateral symmetrical diffuse punctate sialeactasis, suggestive of Sjögren's syndrome [].
The patient is a 64-year-old African American man who presented with back pain in the fall 2012. Diagnostic work up for the back pain included a computed tomography scan, which showed a 7 cm incidental mass on the right kidney. He then underwent right nephrectomy and pathology studies of the mass were consistent with a 5.3 cm RCC Furham grade 3, identified grossly as a golden yellow tumor with a hemorrhagic surface showing several white and gray areas that occupied the inferior pole. The excision margins were found to be negative and it was concluded that the tumor was confined to the kidney with no involvement of the vein, the renal sinus and the local lymphovascular structures. Later in August 2014, the patient noted a left tonsillar mass with cervical lymphadenopathy and underwent bilateral tonsillectomy after an inconclusive biopsy. The surgical pathology report concluded that the mass was a 1.0×0.4×0.4 cm carcinoma metastasis, with similar histologic features to the previous RCC. The right tonsil was normal. The excision margins were again free of carcinoma. Subsequent repeat positron emission tomography scans through spring 2015 demonstrated constantly progressing and increased osteoblastic activity with associated active soft tissue in multiple sites including left clavicle (medial third), left sixth rib, left T012 hemivertebra, right acetabulum and ischium, and right mid femur. These foci of activity were compatible with malignant and metastatic etiology. In the summer of 2015, the patient presented with bleeding lesions of the scalp grossly visible as three heterogeneously ulcerated masses, the largest of which was on the forehead measuring 4.4×3.3 cm ().\nPathology and immunochemical analyses revealed atypical cells, positive for CAM 5.2 and negative for CK7 and CD68 consistent with metastasis from a primary RCC. Late in 2015, the patient presented with fever and altered mental status due to a bacterial infection. An incidental new pulmonary mass was found in the left lower lobe, presumably another metastatic mass of his previous RCC.\nDuring 2015, the patient was treated with everolimus. Everolimus is a mammalian target of rapamycin (mTOR) inhibitor which had previously been shown to prolong progression-free survival relative to placebo in patients with metastatic renal cell carcinoma whose disease had progressed while on vascular endothelial growth factor-targeted therapy with (with tyrosine kinase inhibitors such as sunitinib or soratinib). However, the patient decided to discontinue treatment late in 2015 due to relentless clinical progression of his disease as evidenced by the appearance of bleeding metastasis on the scalp and worsening symptoms such as chest pain and left leg pain. The patient declined pursuing further chemotherapy or radiation therapy and opted for hospice care.
A 47-year-old male with a history of ischemic cardiomyopathy received an ICD for spontaneous, hemodynamically unstable sustained Ventricular Flutter (VF). The ICD was a Medtronic Protecta VR with a quadripolar DF-4 single coil lead (model 6935M). The maximum shock energy output for this generator is 35 Joules (J). Intraoperative induced ventricular tachycardia (VT) at cycle length (CL) of 300 msec was terminated by Anti Tachycardia Pacing (ATP). A subsequent induced VF was terminated by a 15 J shock on the first attempt.\nA month later, while exercising and after skipping few doses of his heart failure medications, the patient sustained five episodes of shocks (). Arrhythmia interrogation revealed sustained VT at a CL of 230-240 msec, which was terminated with a 24.8 J shock (). However, immediately after the initial episode, VT had recurred at a CL similar to first episode. A 24.8 J shock was delivered which resulted in a stable VT at a CL of 230 msec and what appeared to be a slightly different morphology. A 35 J shock was delivered (), which resulted in a stable VT at a CL of 250 msec that was morphologically different from previous episodes. Another shock at 35 J was then delivered, with another change in the VT morphology and stabilization of cycle length at 280 msec, which was in the VT therapy zone. In an attempt to terminate the tachycardia, ATP was deployed 3 times resulting in acceleration of the VT to a CL of 230 msec (). Another shock was delivered at 35 J, which resulted in successful termination of VT after 8 beats with variable CL (). Bursts of polymorphic VT were noted after termination of the VT. Thus, 2 shocks at maximum output did not terminate the VT, and only the third shock at 35 J resulted in an "unclean" termination of the arrhythmia. Mexiletine 200 mg twice daily was started and patient was referred to our institution for further management.\nAfter careful interrogation, and failure to identify any reversible causes of the increase in defibrillation thresholds (complete metabolic profile, cardiac enzymes), Mexiletine was discontinued due to concerns that it could further increase the defibrillation threshold. [] It was felt that there was not a sufficient margin of safety in the defibrillation threshold, and implantation of subcutaneous shocking lead was planned.\nSince adding another lead is not a possibility with the DF-4 header, we arrange with a manufacturer to get a pre-production adaptor/splitter () with bifurcated DF-1 and DF-4 inputs into the ICD's DF-4 header. A single-coil DF-1 subcutaneous defibrillation electrode (Medtronic 6996SQ) was tunneled from the left precordial area to the left axilla (). The new DF-1 lead and the existing DF-4 lead were both connected to the bifurcated adaptor, which, in turn, was connected to the DF-4 port of the original ICD generator.\nIntraoperative VF was successfully induced with shock on T. The first shock, at 15 J, successfully terminated the VF and sinus rhythm emerged. The measured shocking impedance was 53 ohms. A second arrhythmia induction resulted in VT at a CL of 280 msec. Five attempts of ATP were deployed with eventual degeneration of the VT into ventricular fibrillation. A 7.9 J shock failed to defibrillate, but a 15 J shock successfully terminated the ventricular fibrillation. A third induction resulted in VT at cycle length of 290 msec, which was terminated with an 8 J shock. ATP was removed from the therapies because of its ineffectiveness and pro-arrhythmic effects. Repeat interrogation a month later revealed no changes in the defibrillation lead impedance, pacing lead impedance, or pacing thresholds.
An 83-year-old female with bilateral primary TKA performed 17 years prior presented to the clinic. The patient was referred with worsening left knee pain, reported gait instability, and swelling for three months duration. Until this point, she had been completely asymptomatic. She was initially seen and treated by an orthopaedic surgeon from an outside facility with physical therapy, followed by a left knee arthrocentesis to rule out infection. The aspirate demonstrated proteinaceous fluid with few benign inflammatory and epithelial cells and cultures were found to be negative. Due to the increasing pain, gait instability, and discomfort, coupled with lack of relief by the current measures, she was referred to the orthopaedic surgery clinic at our institution for further evaluation.\nAt her initial visit, the patient reported steadily increasing, sharp pain localized to her left knee joint with associated swelling that worsened with ambulation and prolonged standing and lacked improvement with conservative management. Her day-to-day activities were becoming restricted secondary to the pain and she reported occasional falls due to the perceived instability of her knee joint. Physical exam revealed a mild antalgic gait and tenderness over her proximal tibia. An in-house X-ray was notable for an increase in size and number of osteochondral bodies in the left suprapatellar recess with a left joint effusion and “lysis and subsidence of the tibial component and decreased thickness, suggestive of loosening and wear” (Figures -). The patient then underwent a bilateral knee bone scan which confirmed the tentative diagnosis of implant loosening with polyethylene wear and instability. The patient was counseled on her treatment options, including surgical and non-surgical management, and elected to undergo revision surgery of her left knee arthroplasty.\nA classical anterior approach to the knee was made through the patient’s previous scar. A medial parapatellar arthrotomy was performed, after which the knee joint was exposed, revealing extensive osteophytes around the patella (Figure ). The osteophytes were removed and a medial release was performed, allowing for removal of the previous components (Figure ). The femoral component was found to have bone ingrowth, which had encased the patella (Figure ). Visualization of the bone-cement interface intra-operatively proved difficult. Upon gross visual inspection, it appeared that local long-term reaction at bone-cement interface had engulfed the cement and resulted in direct ingrowth of bone to implant. Bone-implant interface tissue was taken for histology examination. Microscopically, the sections examined showed papillary synovial proliferation which is consistent with the patient’s history of long-standing osteoarthritis (Figure ). Multiple foreign body giant cells, which are formed by fused macrophages, are seen in response to polarizable foreign material (Figure ). Orthopaedic implants can cause chronic inflammation and giant cell foreign body response as seen in this case (Figure ).\nAfter component removal, joint preparation was done in standard fashion by membranous tissue removal and minimal freshening of previous bone cuts. Then revision of knee components was performed as per standard technique.\nPostoperative X-rays confirmed excellent placement of a left knee arthroplasty (Figures -). The patient experienced no postoperative complications and was discharged from the hospital on postoperative day 2. She then followed up in the clinic two weeks postoperatively. The patient stated her pain was well controlled and had been working well with physical therapy. X-rays performed at this time reported that the left knee arthroplasty was in expected position with no evidence of hardware failure or loosening (Figures -). She reported that she was pleased with her new prosthesis.
We present the case of a 26-year-old male who is referred to the digestive consultation by two episodes of spontaneous paraesophageal abscess in an interval of 2 years.\nIt is a patient with no pathological history of interest that is presented in the Emergency Service for dysphagia for solids of 3 days of evolution that at the same time was suffering stabbing chest pain and fever of up to 38.8 °C in the last 24 h. In the last year the patient had already been in the Emergency Room (ER) twice for chest pain with non-altered complementary tests. The patient denies having any traumatic history or onset of symptomatology after food impaction. The physical examination shows no abnormality on a hemodynamically stable patient. It is performed a blood test showed a C reactive protein (CRP) 190 mg/L (Normal values 0–5 mg/L), and white blood cells 12,000/μL (Normal values 4000–10,000). For that reason it is decided to perform thoracic-abdominal computed tomography (CT), where a collection of 8 × 4 × 5 cm is displayed in the third inferior–posterior of the esophagus compatible with hematoma vs mediastinal abscess (Fig. ).\nThe surgery service is contacted and it is decided to choose the conservative treatment with broad-spectrum antibiotics and absolute diet. During the admission, a echocardiogram with normal results was performed, an esophagogram that does not present alterations and a gastroscopy, where a linear ulcer of 5 mm in distal third of esophagus with biopsy that shows granulation tissue was found.\nThe patient is discharged 7 days after, with the normalization of his analytical and clinical parameters, and showing a correct oral tolerance for later control in consultations.\nAn outpatient USE is requested 3 weeks later, after being discharged, where no paraesophageal collection is displayed. Gastroscopy was repeated where the esophageal ulcer is not visualized and biopsies are taken from the distal and proximal esophagus. In those biopsies, it is noticed an eosinophilic inflammatory infiltration of 40 eosinophils per field.\nThe patient does not attend any control, so no treatment is started.\nOne year later the patient returns to the emergency department with chest pain and dysphagia with same characteristics, and elevation of CRP and white blood cells. Again, a toraco-abdominal CT is performed, objectivizing mediastinal collection in the same location as 1 year before, with a size of 7 × 4 × 4 cm, compatible with abscess, which is retreated in a conservative manner with broad spectrum antibiotics. After 10 days, a CT control confirms resolution of the collection.\nAmbulatory gastroscopy is performed with biopsy-taking by objectivizing an eosinophilic inflammatory infiltrate compatible with eosinophilic esophagitis.\nThe patient denies dysphagia, chest pain, heartburn or any other clinic between episodes of mediastinal abscess.\nIt starts treatment with proton pump inhibitor in double doses during 8 weeks, persisting the eosinophilic inflammatory infiltrate in the biopsies. It is agreed a diet with the patient where two foods will be removed (milk and wheat), obtaining histological remission, and identifying the milk as the cause of the inflammation.\nAfter 2 years of follow-up, the patient maintains milk and derivatives restriction, and has not shown again any episodes of mediastinal abscess.
A 68-year-old Caucasian man was referred to our out-patients clinic following the incidental discovery of a 6 × 8 cm distended iliac vessel whilst having an ultrasound for surveillance of liver cirrhosis. His clinical history revealed a progressive history of right flank pain, worsening right leg claudication and a persistently cold sensation in his right foot. He also complained of progressive breathlessness on exertion, clinically suggestive of deteriorating CCF. There was no history of trauma or previous surgery. Ankle-brachial pressure measurements revealed a reduced index of 0.76 on the right, and a normal index of 1.35 on the left with corresponding toe pressures of 55 mmHg and 143 mmHg respectively. A contrast enhanced computed tomography (CT) scan showed an isolated right common iliac artery (CIA) to right common iliac vein AVF (Figure ). His aorta was normal in caliber, measuring 19 mm at the aortic bifurcation. The aneurysmal segment began immediately distal to the aortic bifurcation at the origin of the right CIA, with no normal segment of CIA evident. The aneurysm measured 9.4 cm in maximal diameter and extended to within 17 mm of the right iliac bifurcation. It was impossible to differentiate the arterial wall from the venous wall in the aneurysmal segment on either ultrasound or CT scans. His inferior vena cava (IVC) was grossly distended with a uniform diameter of 36 mm in its full course.\nPre-operative discussions focused on finding an endovascular strategy to solve the problem. As no normal caliber proximal right CIA existed, an isolated iliac covered stent was impossible as no proximal sealing zone existed. This required sealing a proximal stent in his aorta. Bifurcated endografts have been used to seal ilio-iliac fistulae previously []. The aortic bifurcation diameter was 19 mm in this case and although some devices have reported success negotiating smaller aortic bifurcations [], we felt an aorto-bi-iliac graft would be in danger of being compressed at the bifurcation with a high risk of occlusion. As a result, a hybrid approach with deployment of an aorto-uni-iliac (AUI) device followed by a femoro-femoral bypass was planned. Our patient was operated upon in our endovascular suite (Siemens) under spinal anesthesia. Both common femoral arteries (CFA) were controlled and cannulated with 6Fr sheaths (Johnston & Johnston). His aorta was cannulated with a 0.035 wire Bentson wire (Cook Medical, Bloomington, IN, USA) via each CFA with the assistance of an angled catheter (Kumpe, Cook Medical). A diagnostic flush pigtail catheter was placed in his aorta via the contralateral limb. A stiff 0.035 Amplatz wire (Amplatz Super Stiff, Boston Scientific) was exchanged as access to the ipsilateral limb. Following an angiogram to confirm renal anatomy, an aorto-uni-iliac device (Zenith Renu, Cook Medical) was deployed from immediately below his renal arteries to his mid right external iliac artery, thus occluding his right internal iliac artery. A 14 × 10 mm Amplatzer occlusion device (AGA Medical Corp, MN, USA) was deployed via the contralateral limb to his proximal left CIA. A completion angiogram confirmed exclusion of the ilio-iliac AVF. A right to left femoro-femoral bypass with 8 mm polytetrafluoroethylene (PTFE) was performed to re-establish flow to his left leg as well as providing left internal iliac perfusion.\nOn the first postoperative day, the mass was no longer pulsatile and the machinery-like murmur in the right iliac fossa was absent. A CT scan on postoperative day two confirmed a patent AUI endovascular graft and patent femoro-femoral bypass, as well as confirming exclusion of contrast from the right iliocaval system (Figure ). His right foot no longer felt cool and the postoperative ankle brachial index (ABI) confirmed an index of 1.05 on the right and 0.95 on the left, with corresponding toe pressures of 119 mmHg and 117 mmHg respectively. He was discharged after seven days without complication. At a clinic one month later, his claudication had resolved completely and he no longer had symptoms suggestive of CCF. A duplex ultrasound confirmed exclusion of arterial flow from the iliocaval vessels.
A 30-year-old woman working as a clerical staff, requiring daily commuting by crowded trains, was suffering from left knee pain for last 6 years. Her difficulty in walking had worsened since last 6 months and did not respond to nonsurgical management. She was bedridden for 2 months before she presented to us. She also had a history of intermittent rectal bleeding.\nOn examination, there were cutaneous and soft tissue hemangiomas alongwith varicose veins throughout the affected left lower extremity from hip to toes, mainly on the gluteal region, posterior thigh, popliteal fossa, calf and foot [Figure -]. There were no visible salmon patches or portwine stains. Left lower extremity was not significantly enlarged compared to right lower extremity. There were no lesions over the anterior aspect of left knee. There was a 10° valgus alignment of the knee []. She had a flexion deformity of 20° with further flexion up to 40°. The movements were very painful in the available range of motion (ROM). The knee society pain score was 29 and her functional score was 0. There was no true leg length discrepancy. Dorsalis pedis and posterior tibial pulsations were palpable. The distal neurological status was normal. The right knee had full range of movements with no pain or deformity. There were no vascular lesions in right lower extremity. Bilateral hip examination was normal.\nRadiographs [] showed severe degenerative tricompartmental arthritis in the left knee joint with bone loss from the lateral femoral condyle and the lateral tibial plateau. There was subchondral sclerosis and osteophyte formation. Phleboliths in the vascular malformations were noticed on the X-rays.\nMagnetic resonance imaging and magnetic resonance venogram [] of the left lower limb with pelvis showed vascular malformation; mostly multifocal hemangiomas involving superficial and deep soft tissues of the entire left lower limb, pelvic region, rectum and left femur. There were no communications between the superficial and the deep lesions. Dual phase angiogram did not show any feeder artery or draining vein. As there were no feeder vessels, preoperative embolization was not done. Her coagulation profile was normal (bleeding time -5 s, clotting time -3 min, prothrombin time-13 s, international normalization ratio-1.1, activated partial thromboplastin time-28 s, Factor VIII-110% and Factor IX-100%). She did not have any systemic involvement. In the absence of limb hypertrophy, we could not label it as classical Klippel–Trenaunay syndrome, but it can be called as a variant of Klippel–Trenaunay syndrome.\nSurgery was done under spinal anesthesia with tourniquet and exsanguination of limb using Eshmarch bandage. Anterior midline incision with medial parapatellar approach was used. Computer-assisted navigated surgery was done due to intra medullary involvement of the lesions []. Ci navigation system (DePuy, Brainlab, Germany) was used. Two thin threaded pins with bicortical holds were used to fix the clamps carrying the tracker ball arrays. The pin hold was good and remained well till the end of the surgery as the bone quality was good on both femoral as well as tibial sides. The synovium was grey-black with an orange tinge of the remaining articular cartilage. Femoral condyles were squared. Femoral bone cuts also revealed vascular malformations in the bone which were curetted out [].\nCemented TKA using posterior cruciate substituting femoral prosthesis (PFC Sigma, Depuy, Johnson and Johnson) with all-poly tibial component [] was done. Bone wax was used to control bony bleeding from metaphyseal lesions at the femoral cuts. Complete hemostasis was achieved after release of tourniquet. Closure was done under re-inflated tourniquet as there was generalized ooze but no active bleeding. Total tourniquet time was 80 min. Intravenous (I.V) tranexamic acid was used (900 mg I.V. infusion, first dose 30 min before tourniquet inflation, second dose 3 h later, and additional third dose 6 h after second dose).\nPostoperatively, her hemoglobin dropped from 12.5 g% to 8.6 g% and she was transfused three units of packed cell volume. Intra-operative blood loss was 300 ml. Postoperative drain was kept for 48 h. The total collection in drain was 650 ml. Prophylactically oral rivoroxaban 10 mg (Xeralto - Bayer Zydus Pharma) was given 2 weeks postsurgery, because such patients have increased risk of thromboembolism. Subsequently, she was kept on oral aspirin 75 mg daily for a period of 4 weeks. Patient was kept on intermittent calf pumps for 3 days postsurgery. She was given DVT above knee stocking and encouraged to do active ankle-foot exercises. Aggressive mobilization was used under supervision of a physiotherapist. Continuous passive motion and full weight bearing walking with a walker were started from next day of surgery. She was walking with walker support and had 0–90° ROM by 7th day, when she was discharged. Her wound healed well, and sutures were removed on the 15th day postsurgery.\nAt 1-year followup, her knee society pain score improved to 97 and functional score improved to 100. She was walking normally without pain and had joined her job. She was also able to commute by crowded public trains without any support.\nHistopathological examination of the removed bone and synovial tissue showed vascular proliferation with lymphoplasmacytic exudates. Conglomerate of vessels with thick walls and red blood cells in slit like lumina with hemosiderophages were seen. These findings were consistent with the histological examination of hemangiomas of Klippel–Trenaunay Syndrome.
A 68-year-old woman presented at our hospital with right upper quadrant pain. She had been diagnosed as having asymptomatic cholecystolithiasis 4 years previously. A diagnostic abdominal computed tomography (CT) scan had revealed a 1.9 cm radiopaque stone (), and thereafter, the patient was monitored by imaging alone once a year. The patient had shown neither symptoms nor changes on imaging until 6 months before the present admission, when CT revealed that the gallbladder stone was compressing the common hepatic duct, although the patient remained asymptomatic (). Subsequently, even though no symptoms were evident and liver function remained normal, abdominal CT conducted 2 months before the present admission showed progressive compression of the common hepatic duct by the gallbladder stone ().\nOn admission, the patient's laboratory findings suggested abnormal liver function with elevated levels of alanine aminotransferase (320 U/L), aspartate aminotransferase (275 U/L), and total bilirubin (0.6 mg/dL). Abdominal CT showed that the gallbladder stone was obstructing the common bile duct with dilatation of the intrahepatic duct (). Based on these findings, we diagnosed the patient as having Mirizzi syndrome and cholangitis. ERCP revealed a round filling defect of the bile duct and inflow of contrast medium along the impacted gallstone directly (). Subsequently, the naïve cystic duct was also identified; it separated from the filling defect (). These findings allowed preoperative diagnosis of cholecystobiliary fistula, and accordingly, the patient was categorized as having Mirizzi syndrome type III, based on the Csendes classification []. Liver function and the inflammation status improved rapidly after placement of a plastic biliary stent, and surgery was performed one month after ERCP. A subcostal incision revealed that the gallbladder had consolidated and hardened; subtotal cholecystectomy was performed, leaving a flap of the gallbladder wall to repair the bile duct. Upon opening of the gallbladder, a large gallstone was removed, revealing a cholecystobiliary fistula involving up to two-thirds of the circumference of the common bile duct (). The fistula was repaired using the cuff of the gallbladder for closure and distal placement of a T tube (). The postoperative course was uneventful, and the patient was discharged on day 17 after surgery.
A 30 year old woman became unwell after swimming with crocodiles while suspended under water in a protective cage in the Australian Northern Territory. She was vertiginous after coming out of the water and required admission to a tertiary hospital emergency department and was discharged with a diagnosis of vestibular neuronitis.\nShe continued to experience symptoms of vertigo, difficulty focusing, dizziness and a feeling of falling to the right for over 4 months and saw various healthcare providers with no cause found. She had a plain computerized tomography of the head without contrast that was reported as normal. Eventually she was referred to a neuro-ophthalmologist for the complaint of "difficulty focusing" and automated perimetry showed a small incomplete congruous homonymous quadrantanopia (Figure ). Magnetic resonance imaging (MRI) of the brain revealed a left occipital infarct. There were multiple areas of bilateral posterior circulation infarcts involving the cerebellum, and right thalamus (Figures ). Echocardiography, carotid ultrasound and hypercoagulable screens were negative. A computerized tomography (CT) angiogram showed a right vertebral dissection with both thinning and a double lumen of the vertebral artery (Figure ). The patient was placed on anticoagulant therapy with no further neurological symptoms.\nVertebral artery dissection (VAD) is a potentially fatal condition where disruption of the vessel wall results in thromboembolism and subsequent ischemic stroke. It may occur following neck injury resulting in neck extension, flexion or rotation [-]. Symptoms include neck or head pain, partial Horner's syndrome and those of ischemic stroke in its involved territory []. The presentation may vary from case to case. The patient likely sustained the VAD following minor neck torsion injury during swimming, followed by a shower of emboli causing multiple posterior circulation infarcts. The congruous nature of the quadrantanopia pointed to an occipital lobe infarct.\nThis case demonstrates the importance of ancillary imaging in making the diagnosis. CT angiography provides accurate imaging of the vessel lumen and dissection length. It can identify VAD in both large and medium vessel dissections in the neck []. T1-weighted MRI is an alternative but may not detect initial stages of intramural haemorrhage seen in early VAD[]. It is for this reason that, recent studies suggest that CTA is the preferred imaging modality to identify cervical dissections, especially for VAD [].\nManagement of VAD to prevent further embolic stroke is done on a case by case basis. There is no data to support the therapeutic superiority of anticoagulants over antiplatelet agents[]. In this case the patient had no contraindications to anticoagulants including intracranial arterial dissection, large stroke, enlarging intramural haematoma and/or high risk of bleeding. The presence of multiple cerebral emboli with no contra-indication favoured the use of anticoagulants in this case.
A sixty year old man presented with diffuse, dull aching pain in left lower abdomen for 3 years and left gluteal region for 2 years. There was severe radiating pain in whole left lower limb more during walking, that made him unable to walk for 1year.On examination there was a left gluteal mass of about 15×20cm, ill defined margins, hard in consistency, tender, seemed to be fixed to the underlying bone and overlying muscle. Cough impulse was negative but very painful to the mass and to the whole left lower limb. Bulk and power of all groups of muscle in left lower limb were normal. His sensory function and deep tendon reflexes were normal but left hip joint movement was restricted and very painful .Left sided straight leg rising test was positive (100). In the left iliac fossa there was a tender, hard, ill defined, intra abdominal mass which did not move with respiration and in knee- elbow position it became impalpable. Patient could not walk but could stand with much difficulty. Preoperative FNAC from gluteal mass reported as schwannoma. Plain X-ray showed a wide gap in left iliac bone. MRI revealed left sided retroperitoneal tumour extending to the left gluteal region through the ileum (Figure-1&). Patient underwent operation under general anesthesia. An S-shaped incision starting from tip of 12th rib curving over the middle of iliac crest extended over the gluteal region and ended just above the greater trochanter (). With the help of iliac osteotomy the dumble shaped tumour (passing through the ileum) was removed. The retroperitoneal portion was well capsulated () but gluteal portion invaded into the muscle irregularly, inferiorly it was on close proximity of the left acetabulum but did not break the articular cartilage. The sciatic nerve was moderately compressed by the mass and there was also some adhesion with the nerve which was released under the microscope. There was no attachment with any other nerves. Gap in the ileum was strengthened with a prolene mesh. The wound closed in layers after leaving a retroperitoneal drain. Histopathology of operative specimen reported as myospherulosis ().\nRetrogradely, patient had no history of intramuscular injection and other medication in the last thirty years. Three months after the operation, pain disappeared and patient could walk without support but there was some limping on left side. At six month after operation patient could walk normally. Post operative MRI six months after operation showed no residual or recurrent tumor ().
A 20-year old man was referred to the maxillofacial department following a single alleged punch to the left side of the face. He complained of swelling and parasthesia of his left cheek, left lateral nose and upper left gingivae.\nOn examination he had gross swelling of the left side of the face, but there was no bony deformity or bony tenderness. A fracture of the left lateral wall of the maxillary sinus was diagnosed on the basis of the occipito-mental (OM) radiographs () and he was treated conservatively with oral antibiotics. One week after his injury, a small haematoma was noted on the left cheek. He was advised to massage the haematoma to decrease its size and to return to the outpatient clinic in one week. Two days later he presented with increased oedema. On examination he was found to have a 3-cm×3-cm fluctuant erythematous swelling of the external left cheek. He underwent incision and drainage of the suspected infected haematoma under general anaesthesia. More than 50 ml of purulent fluid was drained and the patient was discharged two days later.\nAn orthopantomograph (OPG) was taken to rule out dental abscess. The OPG () showed radiolucency in the left side of the maxilla extending from the upper right central incisor to the upper left first molar, which had not been clear on the previous occipito-mental views.\nA computed tomography (CT) scan showed an extremely large lobulated lesion with a relatively well defined border extending from the upper right lateral incisor to the upper left first molar (). The lateral wall of the antrum was perforated. Due to the massive size of the lesion and its multi-lobulated nature on the CT scan, the diagnosis of an odontogenic keratocyst was suspected, but ameloblastoma could not be ruled out.\nFollowing the OPG and CT results, a biopsy was performed at two sites under local anaesthesia. Histopathology reports indicated this was a cystic cavity lined with stratified squamous epithelium showing some hyperplasia. The wall of the cyst consisted of dense fibrous connective tissue with abundant chronic inflammatory cells, few mast cells, and eosinophils. The condition was diagnosed as a radicular cyst.\nThe cyst was enucleated by a Caldwell-Luc approach and was found to extend through the entire maxilla on the left side into the antrum, and distally to the second molar tooth. The postoperative course was uneventful. The patient required long-term follow-up to check for recurrence. He was referred to his GDP for thorough investigation and treatment of non-vital teeth.
A 4-year-old girl presented with repeated episodes of chest infection. Sequential chest radiographs showed an area of nonresolving consolidation in the left lower lobe. She was referred to the radiology department for CT angiography based on the clinical suspicion of pulmonary sequestration. The patient underwent multidetector CT on 64-slice state-of-the-art Siemens scanner. The examination was performed at 150 mA and 120 kV. Plain scan of the chest was performed from the level of thoracic inlet to just below the inferior pole of the kidney. Non-ionic iodinated contrast 50 mL was given at the dose of 2 mL/kg of body weight for the angiographic study. Using the bolus tracking method, the angiogram was done by an automatic trigger system with a density of 100 HU in the ascending aorta. CT scan was acquired during quiet respiration without sedation. Images were processed with standard soft tissue settings (300 window width [WW], 40 window length [WL]) and lung settings (1200 WW, -600 WL). Axial CT images showed a solid heterogenously enhancing mass located in the left lower lobe, above the diaphragm. On angiography, three anomalous vessels arising from the descending aorta were seen supplying the enhancing mass lesion on the arterial phase []. Multiple veins were seen draining the mass into the left inferior pulmonary vein and eventually into the left atrium on the venous phase []. Evaluation of the origin as well as the entire course was difficult with axial images alone. Hence further evaluation was done by processing the images and obtaining 3D volume rendered and maximum intensity projection images in different anatomic planes using the Wizard workstation []. Based on the radiologic findings, the pediatric surgeon did a thoracotomy and resected the sequestered lower lobe lung segment. Our findings were confirmed intraoperatively. Postoperative chest radiograph revealed resolution of left lower zone consolidation. Along with sequestration, another anomaly (ectopic right kidney in the pelvis) was picked up during the course of the study.
A 58-year-old female presented to the emergency department with intermittent, crampy right-sided abdominal pain, nausea, and vomiting, which began approximately 18 hours previously. Her past medical history was significant for hypertension and her surgical history included a thyroidectomy for treatment of thyroid cancer and a Caesarean section. A contrast enhanced CT abdomen and pelvis was obtained, demonstrating multiple fluid-filled, dilated small bowel loops in the right abdomen, which were predominantly anterolateral to the ascending colon and cecum (). In addition, two transition points were identified in the right lower quadrant, with one transition point at the distal ileum just proximal to the cecum and a second transition point in the proximal ileum. The two transition points were in close proximity to each other, indicative of closed loop obstruction. Decreased wall enhancement of the dilated small bowel loops was concerning for ischemia. Mucosal hyperenhancement of the ileum at the proximal transition point was felt to relate to ischemia or decompressed state (). Given the patient's symptoms and findings of closed loop obstruction on CT, the patient was taken to operating room. In the operating room, an internal hernia with closed loop obstruction was confirmed and resulted from herniation of small bowel through an adhesion of a transverse colon epiploic appendage to the ascending colon mesentery. The herniated small bowel was nonviable and a total of 60 cm of small bowel was resected (). Retrospectively, kinking of the ascending and transverse colon could be seen on the initial abdominal CT and was felt to correspond with the site of adhesion ().\nAfter resection, the patient's small bowel was left in discontinuity and an abdominal wound-vac was placed. The following day, the patient returned to the operating room, at which time the terminal ileum was also found to be nonviable. An ileocecectomy with enterocolonic anastomosis was performed.\nThe patient had a complicated postoperative course, but was ultimately discharged approximately two weeks after the initial surgery.
A 28-year-old man with severe aortic regurgitation underwent aortic valve replacement with a mechanical prosthetic valve about 10 years ago. At the time of this operation the ascending aorta was slightly dilated and measured as 3.4 cm. An ascending aortic aneurysm was suspected with chest X-ray in routine follow-up of the patient who had not been controlled until that time (). Echocardiography and chest-enhanced computed tomography revealed a giant ascending aortic aneurysm about 16 cm in diameter with intact aortic arch (). Prosthetic valve function and other cardiac structures were assessed as normal with two-dimensional and color Doppler examination. An elective operation was planned for the aortic aneurysm. The operation was performed under cardiopulmonary bypass, established by cannulation of the right femoral artery and right atrium via the right femoral vein. Cardiopulmonary bypass was started before sternotomy to decompress the aneurysm. Chest was opened with a median resternotomy. A giant ascending aortic aneurysm was occupying most of the space in the pericardial cavity, with the heart lying posteriorly (). The aortic arch was not found to be involved. After careful dissection of the aneurysm we were able to cross clamp the aorta proximally to the brachiocephalic trunk. After cross-clamping, the aorta was opened and cardioplegic solution was infused into each coronary artery. The previously implanted valve prosthesis was intact and assessment of valve functions was normal. Aneurysm of the ascending aorta was resected leaving the functional old mechanical prosthesis in place and we implanted a 34-mm Hemashield woven graft (Meadox Medicals Inc, Oakland, NJ, USA), associated with the left and right coronary artery button implantation. Distal anastomosis of the aortic graft was performed under aortic cross clamp (Figures and ). Aortic clamping time was 117 minutes. Weaning from cardiopulmonary bypass and the postoperative course was uneventful. The patient was discharged without complication 10 days after surgery. Marfan syndrome was clinically excluded. Histological findings of the aortic aneurysm wall showed cystic medial necrosis ().
A 41-year-old man was brought to our ED complaining of confusion. One week earlier he had started to experience severe nausea and vomiting. These symptoms were followed by diplopia, dysarthria and also dysphasia. One day later he had experienced gait disturbances and progressive ataxia accompanied with confusion, apathy and disorientation. He had no history of alcoholism, drug abuse or previous surgery but had a history of untreated Crohn disease. Just before the arrival in our ED, he was hospitalized in another center for about one week but all investigations had failed to reveal the diagnosis. When he was admitted to our ED, he was confused and disoriented to time and place. He was dysarthric and replied with inappropriate answers. Cranial nerve examination showed bilateral horizontal nystagmus in lateral gaze, left abducens nerve palsy and upward gaze palsy. Pupils had normal size and normal light reaction. Gag reflex was absent. In motor examination, tone and force of muscles were normal, DTRs were +2 in all limbs. Plantar reflexes were upwards bilaterally. Sensory examination was normal. Bilaterally, finger to nose exam was abnormal and dysmetria was seen. After reviewing all previously performed investigations, MRI was performed which was consistent with the diagnosis of WE ( and ). Treatment with thiamine led to partial resolution of his upward gaze palsy and nystagmus on the first day. At the end of the third day of treatment, except for gate ataxia, all other symptoms were fully corrected and he was totally conscious. After the fifth day his gait became normal and after one week he was discharged in good general condition.\nIt should be mentioned that after recovery he denied any alcohol consumption but mentioned occasional use of Clonazepam. All other clinical settings related to WE described in previous reports were thoroughly ruled out, including malnutrition, history of gastrointestinal surgical procedures, unbalanced nutrition including staple diet of polished rice, chronic diarrhea, cancer, systemic diseases such as renal disease, hyperthyroidism, and chronic infectious febrile diseases, magnesium depletion, and use of drugs known for causing WE.
A 63 year old white female with past medical history significant for coronary artery disease with stent placement presented to the hospital with diffuse cervical, inguinal, and axillary lymphadenopathy that had been present for almost two years. The patient reported that her lymph nodes were progressively increasing in size and causing her to have dysphagia to solids and shortness of breath when lying flat. She also endorsed looser fitting clothes indicating weight loss and drenching night sweats.\nThe patient said that she first developed lymphadenopathy on the right side of her neck two years ago while working in a chicken farm with exposure to ammonia. She saw a physician for lymphadenopathy at that time but was told that her swollen lymph nodes were likely reactive from ammonia. The patient quit her job but the lymphadenopathy persisted.\nExtensive work-up for the patient's lymphadenopathy was done with the highest suspicion for malignancy versus infectious etiology as the cause of the patient's presentation. Computed tomography of the chest showed diffuse cervical, mediastinal, and axillary lymphadenopathy with areas of necrosis and scattered groundglass opacities in the upper and lower lobes with multiple bilateral pulmonary nodules. Computed tomography of the abdomen and pelvis showed extensive abdominal, retroperitoneal, and pelvic lymphadenopathy, splenomegaly, and scattered sclerotic foci throughout the vertebral bodies and pelvis.\nInfectious work-up including HIV, syphilis, histoplasma, tuberculosis, toxoplasma, blastomyces, brucella, bartonella, coccidioides, and HTLV I and II proved to be negative. However EBV viral capsid antibody, early antigen IgG, and nuclear antigen antibody were all found to be elevated.\nNext, a fine needle aspiration and core biopsy were performed on the patient's left axillary node. Surprisingly the results proved to be negative for malignancy and the patient was diagnosed with granulomatous lymphadenitis with eosinophilic infiltrate. However, due to the patient's extensive lymphadenopathy an excisional lymph node biopsy of the left inguinal area was performed. The biopsy results showed T-cell lymphoma with Ebstein-Barr virus highlighting rare small-sized cells on immunoperoxidase studies. Polymerase chain reaction of the sample showed T-cell receptor beta and gamma gene rearrangement.
An 18-year-old Chinese male accompanied by his parents sought a consultation at the National Dental Centre Singapore. Their chief concern was an unerupted upper right central incisor (#11) resulting in an unaesthetic anterior space. The patient had no relevant medical history and was otherwise healthy. He did not recall any prior history of trauma to his front tooth. Intra-oral examination showed that he was disease free but he also presented with a submerged lower left deciduous second molar.\nThe patient had a Class I malocclusion on a mild skeletal III base. He had upper moderate spacing due to an impacted and dilacerated upper right central incisor. The space at the upper right central incisor was reduced at 5.5 mm []. The analysis of the lateral cephalometric radiograph confirmed a mild skeletal Class III relationship [].\nThe panoramic radiograph demonstrated an impacted maxillary right central incisor and a submerged lower left deciduous second molar with a missing lower left second premolar successor []. The angle between the root and the crown of the central incisor was found to be dilacerated on the lateral cephalometric radiograph at approximately 90°. The crown of the tooth was displaced palatally with the root displaced labially and out of the alveolar housing as seen on the cone beam computed tomography (CBCT) []. The root length measured as the perpendicular distance from the cementoenamel junction to the apex of the root using the Simplant Pro 17.1 software is 8.2 mm [].\nRedistribute the space in the maxillary anterior region Restore the normal appearance of the maxillary anterior teeth Maintain the space at the lower left second premolar site for a future implant replacement.\nSeveral treatment options were explored; the following are the two possible treatment alternatives:\nExcision of the impacted central incisor, orthodontic space opening and restoration with a bridge or an implant later when growth has ceased Surgical exposure, orthodontic space opening and traction of the impacted dilacerated central incisor into proper position.\nWith regards to option 2, the patient and parents were informed of the possibility of a failed traction of the dilacerated central incisor due to ankylosis, severe mobility due to lack of alveolar support as the tooth was out of the alveolar housing, external root resorption and the loss of tooth vitality.\nAfter informing all possible treatment alternatives, the patient chose to try to save the tooth and bring it into proper position. They were informed that should the tooth be ankylosed and not amenable to orthodontic traction, the excision of #11 and replacement with a bridge or an implant at a later stage was still a viable option.\nPhase I of the treatment was to create sufficient space for the traction of the impacted incisor. The submerged deciduous lower second molar was found to be ankylosed and had to be surgically excised. The removal of the deciduous tooth was performed to allow continued vertical development of the alveolus. This occurs from bone apposition that occurs on top of the interdental septum of adjacent non-ankylosed permanent teeth via forces from both the periodontal and gingival fibres.[]\nOnce adequate space was achieved, the patient was referred to the oral surgeon for an open exposure of the impacted incisor. An open window exposure of the labial surface of the crown on the palatal side was performed. A button was bonded to the labial surface of the crown a week after the exposure. Light orthodontic traction using the powerchain from #11 button to an upper 0.018 inch stainless steel archwire was initiated. Once more of the #11 crown was visible, a bracket was bonded and further traction was performed using a piggyback 0.014 inch nickel-titanium archwire on a 0.018 inch stainless steel base archwire. Rectangular archwires with palatal root torque were placed to move #11 root palatally []. Tooth vitality tests were performed every 3 months and the tooth #11 responded normally.\nThe impacted and dilacerated right maxillary central incisor was successfully positioned into proper alignment through a single stage crown exposure and orthodontic traction. As the crown of the tooth was moved buccally, there was insufficient buccal cortical bone which resulted in a transient buccal recession as seen in . Palatal root torque was applied gradually to position the tooth root back into the alveolus which improved the gingival contour and reduced the amount of buccal recession on the tooth. The tooth also had prominent mammelons [] and was extruded gradually to allow for enameloplasty of the incisal edges to improve the aesthetics and further helped to reduce the amount of buccal recession. The tooth had acceptable gingival contour with sufficient attached gingiva, hence no further soft tissue grafting procedures were indicated []. The crown of the tooth was positioned in its ideal position with respect to the contralateral central incisor [], while the root of the dilacerated central incisor was positioned palatally []. The tooth remained vital, responding normally to pulp tests. On the periapical radiograph [], the incisor reveals a shortened root due to the 90° crown-root angulation. On the periapical radiograph as well as the orthopantogram there is a radiolucent area at the apical portion of the root, this is consistent with the lack of cortical bone on the buccal aspect of the tooth as the root is positioned palatally. The lack of buccal cortical bone on the apical aspect can be seen as a buccal defect on the intra-oral photographs in . The post-treatment CBCT [] shows that there was mild root resorption of the dilacerated central incisor. The post-treatment root length measured as the perpendicular distance from the cementoenamel junction to the root apex using the Simplant Pro 17.1 software is 8.12 mm []. Clinically, the tooth has normal mobility as compared to the adjacent incisors. The tooth has been asymptomatic and pulp testing showed a vital pulp at the 1-year follow-up.
A 33-year-old Chinese female presented with concerns about the appearance of her anterior teeth and was referred by a private dental practitioner to the Prince Philip Dental Hospital (PPDH) for aesthetic management of her upper worn anterior teeth. History revealed that she had endodontic treatment on her two upper central incisors because of pain. She also had some tenderness on her lower right molar when chewing. She reported gastric regurgitation after eating but denied self-induced purging at that time.\nThis patient presented with competent lips, average lip line, normal TMJ's, and class III incisor relation () on a skeletal class 3 base. The patient had good oral hygiene and a healthy periodontal condition. Tooth 13 was missing and 12 had drifted to the 13 position (). There was severe erosive tooth surface loss on the palatal and incisal surfaces of teeth 11, 21, and 22 with exposure of dentine over the whole palatal surface. The crown height of the two upper central incisors was reduced to 5 mm. This gave rise to a reduced visible crown height and a reverse smile line (). The gingival level of the upper central incisors was level with the lateral incisors and below that of the canine teeth. A crack line was detected at 46 distoocclusal area () with no periapical radiolucency (), and a diagnosis of crack tooth syndrome was suspected. Teeth 12, 22, 23, and 46 were judged vital with the use of electric pulp testing.\nThis patient was advised to seek medical consultation and management concerning her gastric regurgitation. She received conservative medical counseling for approximately one year, and her gastrointestinal disturbance improved. To prevent further symptoms and crack propagation, an orthodontic band was cemented on 46 after which no pain during chewing was reported. Upper and lower impressions were recorded using irreversible hydrocolloid (Aroma Fine Plus, GC Corporation, Tokyo, Japan). A facebow record was taken, and the casts were hand articulated and mounted in maximum intercuspal position. After analysis of the study models, it was decided to perform localized “intrusion” of the anterior teeth using a lower supraoccluding appliance and to build up the correct contour of upper incisors to facilitate the restorative rehabilitation. It was determined that an increase in the OVD by 1.5 mm would provide sufficient space for restoration. To control the anticipated increased OVD for the composite build-up, a silicone putty (Exafine Putty, GC Corporation, Tokyo, Japan) jaw record was performed on the posterior teeth of the articulated study models as a reference jig.\nThe supraoccluding appliance was waxed up on the lingual surface of the lower incisors with a bite platform designed to load the opposing incisor axially. Incisal hooks were included to give the appliance resistance form. This was then cast in cobalt-chromium (CoCr) (Remanium® GM 800+, DENTAURUM GmbH & Co., Ispringen, Germany) (). The appliance was then cemented on lower incisors with glass ionomer cement (Ketac Cem, ESPE-Premier Sales Corp., Norristown, PA, USA). At the same visit, composite resin (Aeliteflo™, Bisco Inc., Schaumburg, IL, U.S.A) was directly added to restore the incisal edges of 11 and 21 and facilitate occlusion on the opposing bite platform at the increased OVD of 1.5 mm (Figures and ) using a silicone putty jaw record as a jig.\nThe patient was informed of possible transitory problems with this supraoccluding technique, including thermal sensitivity; difficulty in eating, speaking, or sleeping; and temporomandibular joint (TMJ) pain []. This patient was reviewed at one week and then at monthly intervals. She reported only some reduced chewing efficiency at the first review and no other symptoms. In order to determine whether tooth movement had occurred, an interocclusal record using Protemp (Protemp II, ESPE, Seefeld, Germany) was taken and it was then inserted at the next review appointment. If tooth movement had occurred, there would be interocclusal separation of the upper anterior teeth with the bite platform of the appliance.\nAfter 2 months, the posterior teeth were found to be in occlusion (). At this time, further “intrusion” of the upper incisors was judged to be necessary if the incisal overjet was to be changed from reverse to positive. Therefore, further composite build-up was performed on the incisal edges of 11, 21, and 22 to again create supraoccluding restorations on the lower appliance (). The OVD was raised by another 1.5 mm which was measured between the midlabial gingival margins of 21 and 32. Complete reestablishment of all occlusal contacts was achieved after another seven months (). Therefore, at this time, the lower supraoccluding appliance was removed by cutting off the incisal hooks and tapping off the prosthesis.\nThe 46 remained symptomless, and a full gold crown restoration was provided for cuspal protection. To allow tooth preparation of sufficient resistance form for the future crowns, it was decided to cement glass fibre posts on 11 and 21 with composite core build-up to restore the palatal tooth surface loss. Teeth 12 to 23 were prepared and provisional restorations made according to the diagnostic wax-up of the study casts which were taken at the new OVD and after complete reestablishment of occlusal contacts (). With the temporary restorations in the mouth, the aesthetic outcome as well as patient's expectations were assessed (). It was determined that lengthening the 13 to 23 incisally by 1 mm would improve the crown proportions, and crown lengthening was proposed to the patient. The soft tissue requirement was guided by a stent showing the expected final crown contour and margins. Surgical crown lengthening was performed () with alveolar bone being removed by approximately 1.5 mm limited to the midlabial of 12, 11, and 21. Tooth preparation margins were refined, and new temporary restorations using Protemp were made chairside using an index of the diagnostic wax-up of the expected final restorations ().\nThe intrusion of the incisors now permitted the incisal edges to be positioned with a positive overjet and overbite. A period of six months was allowed for the healing and stabilization of the soft tissues, and the patient was satisfied with the aesthetics with diagnostic restorations following review after 6 months. The form and contour of these diagnostic restorations were recorded and copied in the final restorations. Definitive restorations were provided in Empress II (Ivoclar Vivadent, Amherst, NY, USA) and cemented with an adhesive resin cement (Calibra, Dentsply, Konstanz, Germany) (Figures , , and ).\nThe patient achieved good oral hygiene and maintained periodontal health during the follow-up period (Figures , , , , , and ). No radiographic periapical change of the crowned teeth or resorption of the roots was observed at 5 years follow-up ().
A 65-year-old woman presented with abdominal pain and hypophagia for 2 weeks. She had a medical history of alcoholic pancreatitis. A computed tomography (CT) scan of her abdomen and pelvis revealed thickening of the transverse colon wall. Colonoscopy showed an ulcerated tumor in the transverse colon, and histological analysis indicated moderately differentiated adenocarcinoma. Serum carcinoembryonic antigen (CEA) levels were normal, and no sites of distant metastasis were reported on preoperative examination. She underwent a laparoscopic extended right hemicolectomy using the non-touch isolation technique. Histopathological examination of the specimen revealed a moderately differentiated adenocarcinoma invading the subserosal layer with low venous invasion (v1) and no lymphatic invasion (ly0). The surgical margins were negative. A total of 92 lymph nodes were removed, of which none showed metastases. The tumor was diagnosed as stage IIA (T3, N0, M0) according to the International Union Against Cancer tumor, node, and metastasis (TNM) classification (7th edition) []. The patient had an uneventful recovery and was on regular follow-up every 3 months without adjuvant chemotherapy.\nFifteen months after her colectomy, a CT scan demonstrated a tumor in the antero-lateral rectal wall that was 20 mm in diameter, and a right ovarian tumor that was 25 mm in diameter (Fig. ). The ovarian tumor was a cystic mass with a solid component on magnetic resonance imaging (Fig. ). Colonoscopy revealed a superficial elevated lesion in the middle rectum that was shown by histological examination to be moderately differentiated adenocarcinoma (Fig. ). Serum CEA levels were normal. These findings were indicative of rectal cancer with ovarian metastasis, or double primary cancer of the rectum and ovary. A diagnostic and therapeutic laparoscopy detected no disseminated peritoneal metastases or liver metastases. Intraoperative cytological examination of the peritoneal lavage was negative for carcinoma. An operative rapid pathological diagnosis of the resected right ovarian tumor indicated metastasis of the colorectal cancer. Thus, we performed laparoscopic low anterior resection preserving the left colic artery with partial resection of the vagina, and bilateral adnexectomy.\nThe resected rectal tumor measured 20 × 18 mm (Fig. ). Histological examination demonstrated a moderately differentiated adenocarcinoma that had invaded the vagina and formed a metastasis in one of 32 lymph nodes. High venous invasion (v3) and low lymphatic invasion (ly1) were observed. The adenocarcinoma was mainly present in the submucosa and muscularis propria with a small range of invasion to the vagina (Fig. ), while the carcinoma-involved region of the mucosal layer had mucosal colonization representing the spread of metastatic tumor cells along the basement membrane of preexisting crypts and/or villi (Fig. ) []. There was no adenomatous precursor at the edge of the tumor, and surgical margins were negative. The right ovarian tumor was moderately differentiated adenocarcinoma that was positive for cytokeratin (CK) 20 and negative for CK7 immunohistochemical staining, indicating metastasis of colorectal cancer. The rectal and ovarian tumors shared high similarities with transverse colon cancer in architectural and cytological atypia (Fig. ). Both adenocarcinomas of the transverse colon and rectum were negative for p53 immunohistochemical staining and RAS wild type in genetic assessment (Fig. ). These findings support a diagnosis of rectal and ovarian metastases from primary transverse colon cancer.\nThe patient recovered well after surgery, and adjuvant chemotherapy was decided after a multidisciplinary meeting. She was treated with eight cycles of oxaliplatin and capecitabine, and neither relapse nor metastasis has been observed 18 months after surgery.\nThe incidence of colorectal metastasis from primary colorectal cancer is rare, and distinguishing primary from metastatic colorectal cancer can be challenging. To our knowledge, only three reports in English and six in Japanese have described suspected cases of colorectal metastasis of colorectal cancer [–]. We reviewed a total of eight patients, including seven out of the abovementioned nine patients with detailed clinical information as well as our own (Table ). The patients were three males and five females, with a median age of 66.5 years (range 52–88 years). Ascending colon cancer was the most common primary tumor (n = 3), followed by tumors of the sigmoid (n = 2), transverse colon (n = 2), and cecum (n = 1). Metastatic colorectal lesions were located in the rectum in all cases. In terms of other metastatic sites, seven patients had other metastases (lung, liver, spleen, abdominal wall, axilla, and ovary) and one had no other metastases.\nA previous study of 278,208 malignancies in a nationwide Japanese pathologic autopsy database from 1990 to 2003 identified 18,252 case with metastatic colorectal cancer. Of these, 1302 (7.1%) were from primary colorectal cancer []. The database does not include information about the suspected metastatic pathway (hematogenous, lymphogenous, direct invasion, or dissemination), but most metastatic tumors in the database are attributed to direct invasion or disseminated metastasis, reflecting late-stage disease. However, the number of reported clinical cases might not represent the actual incidence of colorectal metastasis from colorectal cancer. One possible reason for the rarity of reported colorectal metastasis cases is that most cases occur as part of systemic advanced disease, for which surgical resection will not be performed []. The other reason is that diagnosing metastasis is difficult [, ]. Although gastrointestinal metastatic carcinoma usually represents a submucosal tumor, differential diagnosis (as primary disease or metastasis) becomes difficult if the tumor invades the mucosal layer. Estrella et al. argued that metastatic carcinomas involving the mucosal surface frequently mimic second primaries, so histologic features cannot reliably distinguish metastatic from primary carcinoma []. Additionally, primary colorectal cancers resembling submucosal tumors have been reported in previous studies [, ]. This explains why colorectal metastases may be misdiagnosed and treated as primaries.\nDistinguishing primary from metastatic colorectal cancer can be challenging, but a comprehensive evaluation of histological features, clinical history, and tumor distribution enables making the correct diagnosis and implementing the optimal treatments []. In this case, we suspected a metachronous metastasis for the following reasons. First, histological findings demonstrated that the adenocarcinoma was mainly present in the submucosa and muscularis propria with mucosal colonization, which is a supportive finding for metastatic carcinoma, as this indicates a low possibility of primary rectal cancer and implantation of the transverse colon cancer. Second, the rectal and ovarian tumors were very similar to the transverse colon cancer in terms of architectural and cytological atypia, further suggesting metastasis of primary transverse colon cancer. Third, there was no adenomatous precursor at the tumor edge, indicating metastatic cancer. Fourth, both adenocarcinomas of the transverse colon and rectum were negative for p53 in immunohistochemical staining and RAS wild type in genetic assessment, indicating metastatic rectal cancer from primary transverse colon cancer.\nIn reviewed cases, the exact mechanism of colorectal metastasis from primary colorectal cancer has not been fully elucidated; thus, the possibility of hematogenous or lymphogenous spread remains. We suspected hematogenous or lymphogenous metastatic pathways to the rectum from the primary transverse colon cancer because the carcinoma was mainly located in submucosa and muscularis propria; however, there was no additional evidence than tumor localization. In this case, the rectal tumor penetrated into a small range of the subserosal layer with invasion to the vagina; additionally, there was a metastatic ovarian carcinoma. Peritoneal metastatic invasion primarily toward the deep layer of the rectal wall cannot be ruled out. The metastatic colorectal lesions were located in the rectum in all reviewed cases, and this suggests that peritoneal spread to Douglas’s pouch or the rectovesical pouch is a possible pathway for colorectal metastasis from primary colorectal cancer.\nConsidering that the majority of reported cases had multiple metastases, the existence of other metastatic lesions might be a risk factor for colorectal metastasis. Therefore, more attention should be paid to colorectal lesions when other metastatic sites have been identified. Colorectal metastases usually represent late-stage disease and have poor prognoses; however, prolonged survival after surgery and complementary therapy can be achieved in some patients []. In this case, neither relapse nor metastasis has been observed 18 months after surgery. The follow-up periods varied too widely to evaluate patient prognosis in reviewed cases. Therefore, additional studies are needed to better understand this rare metastasis and to determine the optimal therapeutic strategies.
A 38-year-old female patient came with a chief complaint of difficulty standing up from squatting position since 2 years ago. The patient also suffered heaviness and numbness from her hips that radiated to both of her knees and ankles. The symptoms worsened steadily in the past 4 months with both lower limbs getting weaker. Laboratory results came out normal, with no signs of infection or positive tumor markers. Radiological examination showed no apparent abnormalities as well. An MRI was obtained, and a tumor mass in the intradural region level of T10–T12 was found (). At that time, the patient was offered surgery, but she chose to undergo treatment with a bone setter. Around two months later, the patient returned to hospital with profound weakness on her lower extremity. Her physical examination revealed paresis from her thigh on both lower extremities grade 1-2/5 power in left and right lower limbs, respectively. Increased patellar reflexes were found on both limbs. Another MRI was performed and showed that the mass had grown to lumbar vertebrae L2, accompanied with worsening of the neurological statuses and impaired sensibility, as well as defecating and urinating problems (). From the history, spinal manipulation procedure was performed by a bone setter, although no specific techniques were available for review.\nA surgical procedure was proposed for exploration and decompression to the patient. The operation started by opening the lamina on T10–T12 levels, followed with laminectomy and hemostatic procedure to stop the bleeding, until the dura was exposed (). A dense mass from T10 to T12 was palpable from the dura layer. After we exposed the lamina, we observed that the dura was tense from touch, and a solid mass underneath the dura was palpable from T10 to L2. Intradural tumor excision was performed by a sharp 3 mm incision in the midline of the dura and then continued with blunt dissection, opening the dura layer to caudal L2 and to cranial T10. Dura was then parted with stay thread until the vessel-rich tumor mass was exposed. The tumor mass was excised, and the surgical field was contaminated by blood from hematomas from tumor vessels (). Bleeding was found to originate from the anterior part and posterior part of the cord, no bleeding source from the cerebrospinal fluid nor the subarachnoid space. The tumor mass was successfully evacuated with fragments of hematomas and necrotic tissues. The dura layer was then closed with a continuous suture. The tumor mass was fixed and transported for histopathology examination ().\nAt the time of discharge, the patient did not regain the function on her lower extremities (1-2 out of 5 on neurological motor examination). After six months of follow-up, some improvement on her lower extremity function was noted. Motor strength was returned to 3-4 out of 5, and the patient was able to ambulate using a walker. No improvement of her bowel and bladder symptoms was noted.
A 22-year-old right hand dominant male presented in the outpatient department with complaints of shortening and inability to extend middle finger of the left hand. On examination, there was an extension lag at the middle finger, poor grip strength, and reduced height of middle ray (). The patient had a history of incidental trauma to the left hand for which he was treated conservatively. Over the 6 months after the trauma, the patient started developing the symptoms in the form of gradual shortening and reduced handgrip strength and presented to our hospital. On investigation, blood parameters including calcium levels and parathormone levels were normal. The X-ray showed pathological fracture of third metacarpal with osteolytic lesion involving base of second metacarpal and carpal bones (). Magnetic resonance imaging showed almost complete osteolysis of the 3rd metacarpal with only the base visible and fracture of the 4th metacarpal with thinning and resorption of the bone and surrounding soft tissue. It also showed pathological fracture involving capitate with patchy areas of osteolysis (). Serial X-rays taken at 4 weeks, 8 weeks, 12 weeks, 18 weeks, and 6 months revealed that there was progressive concentric reduction of metacarpal shaft, first involving third then fourth metacarpal giving appearance of a sucked candy (). At this point in time, we kept in mind the differential diagnosis of primary bone tumor like aggressive giant cell tumor or aneurysmal bone cyst or metastasis from any other primary tumor. Positron emission tomography scan was done to rule out any tumor, however, no evidence of tumor was found. Parathyroid hormone levels were within normal limit. Biopsy suggested myxoid areas, proliferating vessels interposed with skeletal muscle fragments with no evidence of malignancy. Based on the clinical-radiological and histopathological presentation, we made a diagnosis of vanishing bone disease. Since there was increased proliferation of blood vessels, we planned to reconstruct vanished metacarpals with non-vascularized fibula as there already was a hypervascular bed. Dorsal S-shaped incision starting from the metacarpal head to radiocarpal joints was taken. Intraoperatively, it was found that there was a thin shell of cartilage from third metacarpal head along with intact collateral and intermetacarpal ligaments. Non-vascularized fibular autologous graft was harvested from the ipsilateral limb and fixed with transverse k-wires to adjacent metacarpal (). Intraoperatively, osteolytic lesion involving carpal bones was curated and bone graft substitute was used to fill cavity and some graft was put alongside of fibular graft. Postoperatively, hand and wrist were immobilized with plaster slab in functional position. The patient was started on 1000 mg elemental calcium in the form of calcium carbonate tablets. Vitamin D, 60,000 IU, once weekly for 12 weeks was also administered. Bisphosphonates were given in the form alendronate 70 mg once weekly for 8 weeks. K-wires were removed at end of 8 weeks and gradual physiotherapy was started to improve hand function. At the end of 2 years, graft was completely incorporated () and the patient gained functional recovery () with active flexion at metacarpophalangeal joints of the 3rd and 4th finger from 0 to 70° and normal range at proximal interphalangeal (PIP) joint (0–100°) and DIP (0–80°) joints. The patient has 60% grip strength in affected hand as compared to normal hand (using dynamometer) with no evidence of recurrence (additional file 1: Movie 1).
Male patient, 71 years old, presented to the emergency department complaining of episodes of intermittent diarrhea without any fever, vomiting, or abdominal pain. The symptoms had appeared, for the first time, two months prior to the patient's presentation. During the clinical examination, there were no palpable masses of the abdomen and an abdominal distention could not be clearly diagnosed, due to patient's obesity. The patient complained also about loss of appetite during the last two months.\nRegarding the standard serum investigations, we observed anemia (HCT 30%), without any other abnormal values of biochemical tests. There was an ultrasound requested, if there was a significant amount of intraperitoneal ascites discovered. Tumor markers were also measured in serum, where Ca 19-9 and CEA serum levels were higher than normal values (Ca 19-9 = 204.4 IU/mL and CEA = 83.5 ng/mL). The computed tomography (CT) control of the abdomen showed that the peritoneal cavity included a significant amount of ascites, and a cystic mass (8 cm in diameter), with small calcifications within its wall, was found in the right iliac fossa, possibly a mucocele of the appendix. Moreover, no enlarged lymphnodes of the abdomen were observed. Colonoscopy and gastroscopy findings were negative for pathology. A small amount of ascitic collection was drawn percutaneously for cytologic examination. The result was a small number of observed lymphocytes in the setting of large amount of mucous matrix. There were no malignant cells observed.\nThe patient underwent an exploratory laparotomy for definite diagnosis. The abdomen was full of gelatinous ascitic liquid and diffuse implantations of the peritoneum. Extended adhesions of the small intestine to the abdominal wall due to neoplastic implantation were found as well. A large mucocele was identified arising from the appendix. The disseminated picture of the disease indicated a cytoreductive surgery procedure. Biopsies were taken from the abdominal wall and the mesenterium. They revealed elements of peritoneal mucinous neoplasm of high malignant grade and microscopic lesions of low differentiated character. The histopathology and further immunoassays (CK7 negative and CK20 positive) confirmed the diagnosis of a disseminated PMCA of appendiceal origin. The patient underwent 4 cycles of systematic chemotherapy (5-fluorouracil based) as well, which was interrupted due to secondary toxic effects and progression of the disease in the abdomen.
A 65-year-old woman presented with abdominal pain and hypophagia for 2 weeks. She had a medical history of alcoholic pancreatitis. A computed tomography (CT) scan of her abdomen and pelvis revealed thickening of the transverse colon wall. Colonoscopy showed an ulcerated tumor in the transverse colon, and histological analysis indicated moderately differentiated adenocarcinoma. Serum carcinoembryonic antigen (CEA) levels were normal, and no sites of distant metastasis were reported on preoperative examination. She underwent a laparoscopic extended right hemicolectomy using the non-touch isolation technique. Histopathological examination of the specimen revealed a moderately differentiated adenocarcinoma invading the subserosal layer with low venous invasion (v1) and no lymphatic invasion (ly0). The surgical margins were negative. A total of 92 lymph nodes were removed, of which none showed metastases. The tumor was diagnosed as stage IIA (T3, N0, M0) according to the International Union Against Cancer tumor, node, and metastasis (TNM) classification (7th edition) []. The patient had an uneventful recovery and was on regular follow-up every 3 months without adjuvant chemotherapy.\nFifteen months after her colectomy, a CT scan demonstrated a tumor in the antero-lateral rectal wall that was 20 mm in diameter, and a right ovarian tumor that was 25 mm in diameter (Fig. ). The ovarian tumor was a cystic mass with a solid component on magnetic resonance imaging (Fig. ). Colonoscopy revealed a superficial elevated lesion in the middle rectum that was shown by histological examination to be moderately differentiated adenocarcinoma (Fig. ). Serum CEA levels were normal. These findings were indicative of rectal cancer with ovarian metastasis, or double primary cancer of the rectum and ovary. A diagnostic and therapeutic laparoscopy detected no disseminated peritoneal metastases or liver metastases. Intraoperative cytological examination of the peritoneal lavage was negative for carcinoma. An operative rapid pathological diagnosis of the resected right ovarian tumor indicated metastasis of the colorectal cancer. Thus, we performed laparoscopic low anterior resection preserving the left colic artery with partial resection of the vagina, and bilateral adnexectomy.\nThe resected rectal tumor measured 20 × 18 mm (Fig. ). Histological examination demonstrated a moderately differentiated adenocarcinoma that had invaded the vagina and formed a metastasis in one of 32 lymph nodes. High venous invasion (v3) and low lymphatic invasion (ly1) were observed. The adenocarcinoma was mainly present in the submucosa and muscularis propria with a small range of invasion to the vagina (Fig. ), while the carcinoma-involved region of the mucosal layer had mucosal colonization representing the spread of metastatic tumor cells along the basement membrane of preexisting crypts and/or villi (Fig. ) []. There was no adenomatous precursor at the edge of the tumor, and surgical margins were negative. The right ovarian tumor was moderately differentiated adenocarcinoma that was positive for cytokeratin (CK) 20 and negative for CK7 immunohistochemical staining, indicating metastasis of colorectal cancer. The rectal and ovarian tumors shared high similarities with transverse colon cancer in architectural and cytological atypia (Fig. ). Both adenocarcinomas of the transverse colon and rectum were negative for p53 immunohistochemical staining and RAS wild type in genetic assessment (Fig. ). These findings support a diagnosis of rectal and ovarian metastases from primary transverse colon cancer.\nThe patient recovered well after surgery, and adjuvant chemotherapy was decided after a multidisciplinary meeting. She was treated with eight cycles of oxaliplatin and capecitabine, and neither relapse nor metastasis has been observed 18 months after surgery.\nThe incidence of colorectal metastasis from primary colorectal cancer is rare, and distinguishing primary from metastatic colorectal cancer can be challenging. To our knowledge, only three reports in English and six in Japanese have described suspected cases of colorectal metastasis of colorectal cancer [–]. We reviewed a total of eight patients, including seven out of the abovementioned nine patients with detailed clinical information as well as our own (Table ). The patients were three males and five females, with a median age of 66.5 years (range 52–88 years). Ascending colon cancer was the most common primary tumor (n = 3), followed by tumors of the sigmoid (n = 2), transverse colon (n = 2), and cecum (n = 1). Metastatic colorectal lesions were located in the rectum in all cases. In terms of other metastatic sites, seven patients had other metastases (lung, liver, spleen, abdominal wall, axilla, and ovary) and one had no other metastases.\nA previous study of 278,208 malignancies in a nationwide Japanese pathologic autopsy database from 1990 to 2003 identified 18,252 case with metastatic colorectal cancer. Of these, 1302 (7.1%) were from primary colorectal cancer []. The database does not include information about the suspected metastatic pathway (hematogenous, lymphogenous, direct invasion, or dissemination), but most metastatic tumors in the database are attributed to direct invasion or disseminated metastasis, reflecting late-stage disease. However, the number of reported clinical cases might not represent the actual incidence of colorectal metastasis from colorectal cancer. One possible reason for the rarity of reported colorectal metastasis cases is that most cases occur as part of systemic advanced disease, for which surgical resection will not be performed []. The other reason is that diagnosing metastasis is difficult [, ]. Although gastrointestinal metastatic carcinoma usually represents a submucosal tumor, differential diagnosis (as primary disease or metastasis) becomes difficult if the tumor invades the mucosal layer. Estrella et al. argued that metastatic carcinomas involving the mucosal surface frequently mimic second primaries, so histologic features cannot reliably distinguish metastatic from primary carcinoma []. Additionally, primary colorectal cancers resembling submucosal tumors have been reported in previous studies [, ]. This explains why colorectal metastases may be misdiagnosed and treated as primaries.\nDistinguishing primary from metastatic colorectal cancer can be challenging, but a comprehensive evaluation of histological features, clinical history, and tumor distribution enables making the correct diagnosis and implementing the optimal treatments []. In this case, we suspected a metachronous metastasis for the following reasons. First, histological findings demonstrated that the adenocarcinoma was mainly present in the submucosa and muscularis propria with mucosal colonization, which is a supportive finding for metastatic carcinoma, as this indicates a low possibility of primary rectal cancer and implantation of the transverse colon cancer. Second, the rectal and ovarian tumors were very similar to the transverse colon cancer in terms of architectural and cytological atypia, further suggesting metastasis of primary transverse colon cancer. Third, there was no adenomatous precursor at the tumor edge, indicating metastatic cancer. Fourth, both adenocarcinomas of the transverse colon and rectum were negative for p53 in immunohistochemical staining and RAS wild type in genetic assessment, indicating metastatic rectal cancer from primary transverse colon cancer.\nIn reviewed cases, the exact mechanism of colorectal metastasis from primary colorectal cancer has not been fully elucidated; thus, the possibility of hematogenous or lymphogenous spread remains. We suspected hematogenous or lymphogenous metastatic pathways to the rectum from the primary transverse colon cancer because the carcinoma was mainly located in submucosa and muscularis propria; however, there was no additional evidence than tumor localization. In this case, the rectal tumor penetrated into a small range of the subserosal layer with invasion to the vagina; additionally, there was a metastatic ovarian carcinoma. Peritoneal metastatic invasion primarily toward the deep layer of the rectal wall cannot be ruled out. The metastatic colorectal lesions were located in the rectum in all reviewed cases, and this suggests that peritoneal spread to Douglas’s pouch or the rectovesical pouch is a possible pathway for colorectal metastasis from primary colorectal cancer.\nConsidering that the majority of reported cases had multiple metastases, the existence of other metastatic lesions might be a risk factor for colorectal metastasis. Therefore, more attention should be paid to colorectal lesions when other metastatic sites have been identified. Colorectal metastases usually represent late-stage disease and have poor prognoses; however, prolonged survival after surgery and complementary therapy can be achieved in some patients []. In this case, neither relapse nor metastasis has been observed 18 months after surgery. The follow-up periods varied too widely to evaluate patient prognosis in reviewed cases. Therefore, additional studies are needed to better understand this rare metastasis and to determine the optimal therapeutic strategies.
A 21-year-old African American male with no past medical history was in his usual state of health until January 2014, when he developed unusual periorbital swelling and a rash. He was evaluated at an outpatient ophthalmology clinic and was started on 60 mg prednisone daily. His periorbital swelling improved with this therapy, but as the prednisone was tapered he began experiencing symmetric bilateral proximal muscle weakness and soreness of the upper and lower extremities. This was accompanied by a hyperpigmented rash on the trunk, extensor surface of the arms, and upper thighs with associated soft tissue swelling. Two weeks after the completion of the prednisone taper, he began experiencing dysphagia to solids more than to liquids and suffered several choking episodes. This forced a change in his diet to thick liquids; as a result, by the end of the month he had lost 60 pounds. Given his worsening symptoms, he presented to our outpatient clinic in July 2014. Blood work for autoantibodies including ANA and dsDNA was negative. He was found to have an elevated total CK (1274 IU/L). ESR and CRP were normal. An upper endoscopy did not reveal any structural abnormalities.\nIn August 2014 during a follow-up visit at our clinic he was found to be very weak, barely able to climb stairs or stand up from a seated position. On examination, hyperpigmented pruritic frank macules were noted on the extensor surface of his arms, trunk, and upper thighs in multiple phases of healing (). His hands and lips were swollen. Hand grip strength was reduced bilaterally. He had 3/5 strength in the left upper extremity and 4/5 on the right upper extremity. He was not able to raise his arm above his head; muscle bulk was decreased in the upper arm compared to the lower extremity. Hip flexors were 3/5 bilaterally, but quadriceps and hamstrings were 4/5 (). In this setting he was immediately transferred to a tertiary care facility.\nInitial lab work there revealed elevated enzymes with AST of 75 U/L, creatinine kinase level of 1017 IU/L, myoglobin level of 233 ng/mL, LDH level of 386 U/L, ferritin 3151 ng/mL and ESR 33 mm/hr, uric acid 6.8 mg/dL, and being HIV negative (). Based on the clinical presentation and history, inflammatory myositis was suspected and rheumatology and dermatology were consulted. Blood works including an ANA, anti-Jo-1, anti-Ro, anti-La, anti-RNP/Smith, serum myoglobin, aldolase, CMV/EBV titers, and anti-histone antibodies were all negative.\nHe underwent CT scan and MRI of his chest, abdomen, and pelvis. Edema found in the bowel wall on CT () prompted a colonoscopy with biopsy but results were nonspecific. MRI revealed intramuscular and subcutaneous edema (), but no occult malignancy. Skin biopsies were obtained from his right extensor arm and anterior chest which revealed a vacuolar interface dermatitis with dermal mucin deposition. A muscle biopsy of his left biceps demonstrated perifascicular atrophy and inflammation consistent with dermatomyositis. For his dysphagia, he was evaluated by a speech-language pathologist and underwent a cine esophagram which showed severe swallowing weakness with aspiration; a PEG tube was subsequently placed. He then began therapy with IVIG and pulse-dose pulse steroids with an eventual taper to 60 mg prednisone. Following IVIG treatment his hemoglobin dropped to 6.9 mg/dL with an increased total bilirubin and LDH suggestive of intravascular hemolysis. Fecal occult blood test was negative and upper-GI bleed was not suspected leading to a glucose-6-phosphate dehydrogenase (G6PD) assay that confirmed very low activity of the enzyme. Thus he was diagnosed with G6PD deficiency and appropriate medication adjustments were made. Hemolysis was thought to be the result of stress from hospitalization. After 1 unit PRBC transfusion his hemoglobin remained stable for the course of the admission and he did not require any more transfusions. He demonstrated clinical improvement over the ensuing days, at which time his antibody testing for NXP-2 antibodies came back positive. NXP-2 antibodies were detected by the Oklahoma Myositis Research Foundation Panel.
This 15-year-old male had experienced symptoms for 3 weeks prior to admission, which were initially limited to persistent bilateral frontal headaches. Two weeks before presentation, he began to complain of peripheral vision loss in his left eye, which at one point became bilateral then returned again to just left-sided visual field deficits. His symptoms again worsened in the 4 days leading up to admission, when he began to complain of increased headache severity, along with new ataxia, left-sided hemiparesthesias, and worsening vision loss. His speech became rambling and incoherent, and he was generally sleepy. On the day he was admitted, all of his symptoms were at their peak severity.\nUpon admission, the patient was sleepy but alert and oriented. His eye examination revealed decreased peripheral vision bilaterally and a lack of convergence. He reported left-sided facial numbness, and he demonstrated difficulty with finger-nose pointing and heel-to-shin on his left side. Strength was decreased in the left upper and lower extremities. Magnetic resonance imaging (MRI) revealed multiple areas of ischemic infarction throughout the posterior circulation of the brain. The lesions were seen in the left lobe of the cerebellum, the right and left occipital lobes, and thalamus bilaterally. They appeared to be of various ages, which suggested a stepwise and stuttering course of infarction. Selected MRI images are presented in . The MRI also revealed an abnormal bony process off the left occipital condyle that appeared to be in contact with a small left vertebral artery. It was felt that the bony spicule was likely traumatizing the left vertebral artery resulting in emboli showering to areas of the brain in the posterior circulation and leading to infarctions. The MRI findings prompted magnetic resonance vascular studies with 3-dimensional reconstruction. These images showing the left vertebral artery and the occipital bony spicule are presented as . Echocardiogram and coagulation studies were performed to rule out alternative causes of strokes, and these tests were all negative. The patient was immediately started on aspirin and placed in a cervical collar due to suspicion of vertebral artery dissection and distal embolization.\nOnce it was decided that the bony spicule was most likely causing vertebral artery dissection and distal embolization, the decision was made to schedule diagnostic transfemoral angiography under general anesthesia with potential endovascular treatment to confirm the adequacy of collateral circulation. Preoperative angiography showing a nondominant left vertebral artery and a dominant right vertebral artery is pictured in , along with postoperative angiography demonstrating sufficient collateral circulation. An alternative option could have been to attempt a conservative trial of anticoagulation and a cervical collar, but it was felt that the risk of further strokes outweighed the risk of the procedure. The patient underwent the endovascular occlusion of the left vertebral artery and tolerated the procedure without complication. The patient had no further strokes after his procedure. He returned to clinic 6 months after his procedure, and his only lingering deficit was homonymous hemianopia and mild blurring of his vision in his far right visual field.
A 74-year-old homeless male with a history of chronic alcoholic pancreatitis developed progressive epigastric abdominal pain and nausea that prompted his presentation to the emergency department at a community hospital. Laboratory studies showed mild transaminitis and hyperbilirubinemia (total bilirubin of 4.2 mg/dL) on initial evaluation. Computed tomography (CT) of the abdomen and pelvis revealed obstruction of the pancreatic and common bile ducts by what was felt to be a pancreatic head mass vs pseudoaneurysm of the GDA. Further evaluation with magnetic resonance cholangiopancreatography showed a 6-cm dilation of the proximal GDA consistent with the diagnosis of visceral artery pseudoaneurysm. Endoscopic retrograde cholangiopancreatography was performed with sphincterotomy and biliary stent placement. Pancreatic stent placement was also attempted but was unsuccessful because of significant external compression from the pseudoaneurysm that prevented cannulation of the major duct.\nFollowing the patient's transfer to our facility for a higher level of care, interventional radiology was consulted for visceral angiography and possible coil embolization of the pseudoaneurysm. The angiographic anatomy () was deemed unsuitable for a variety of endovascular interventions. CT angiography was obtained to facilitate thorough operative planning for definitive repair ().\nAfter review of all available imaging, the patient was taken to the operating room. The abdomen was entered via an upper midline laparotomy. A Bookwalter self-retaining retractor (Symmetry Surgical, Inc.) was placed to provide wide exposure. The hepatic flexure and transverse colon were mobilized and retracted inferiorly away from the head of the pancreas. The pulsatile GDA pseudoaneurysm was easily palpable at this point. The gastrohepatic ligament was opened, and the common hepatic artery proximal to the GDA was mobilized. The proximal right and left hepatic arteries were mobilized and controlled with vessel loops given the lack of a proper hepatic artery between the GDA origin and the bifurcation. The pylorus and first portion of the duodenum were then mobilized away from the head of the pancreas. The liver was noted to have good arterial inflow from the celiac trunk, both on palpation and intraoperative ultrasound with temporary occlusion of the GDA. Clamps were applied proximally and distally following heparinization, and the GDA was divided at its origin. At this point, the surgical team believed that primary closure of the arteriotomy created by division of the GDA would result in potentially hemodynamically significant stenosis and compromise hepatic artery inflow. Bovine pericardial patch angioplasty was performed, and the proximal and distal clamps were released without evidence of compromised inflow. Examination of the pseudoaneurysm revealed some continued faint pulsatility. The duodenum was further mobilized, and the anterior inferior pancreaticoduodenal artery distal to the pseudoaneurysm was identified. The artery was clamped with cessation of all pulsatility and subsequently ligated. The pseudoaneurysm was then opened, a large amount of thrombus was evacuated, and an aneurysmorrhaphy was performed. A Blake drain was placed, and the abdomen was closed in the typical fashion to complete the case.\nThe abdominal drain was removed on postoperative day 7. The patient's postoperative course was uncomplicated aside from extensive social work issues because of his transient social situation and difficulty with posthospitalization placement.
A healthy 15-year-old girl was referred following a five day history of a unilateral red left eye. The eye was becoming progressively more inflammed, with epiphora, photophobia and blurred visual acuity.\nOn examination, the visual acuity in the affected eye was 6/6 compared with 6/5 in the other eye. The conjunctiva was inflamed with a follicular reaction including the corneal margins superiorly. There were enlarged pre auricular nodes. A diagnosis of viral conjunctivitis was made and viral plus bacterial swabs were taken.\nThe patient was commenced on fucithalmic to prevent secondary infection and told to re attend if she deteriorated. Five days later, she returned feeling that the eye had become more tender with increased swelling of the lids plus tenderness over the maxillary sinus.\nOn examination, there was marked oedema of the upper and lower lids accompanied by erythema. The tarsal conjunctiva revealed follicles. Extra ocular movements were full but uncomfortable on elevation. Pupil, colour vision examination and direct visualisation revealed a healthy disc. She was apyrexial and systemically otherwise well. A secondary diagnosis of bacterial pre septal cellulitis was made and the treatment was changed to oral ciprofloxacin 750 mg twice daily for one week, plus two hourly topical exocin drops.\nTwo days later the patient was reviewed. She now complained of a large amount of purulent discharge. The pre septal swelling was still marked and examination of the conjunctiva again revealed large numbers of follicles and large papillae.\nAll bacteriology and viral swabs had been negative and the patient was re swabbed for chlamydia despite denying any genitourinary symptoms. Giemsa staining of conjunctival scrapings revealed cytoplasmic inclusion bodies and the patient received a one gram single dose of Azithromycin. She was asked to attend the local genitourinary clinic and to alert any current and previous sexual partners to their need to do likewise. Her symptoms completely resolved following administration of the Azithromycin and there were no further complications. Unfortunately, the patient declined to have photographs taken.
A 68-year-old man presented for evaluation of a new asymptomatic bump on his left arm. His previous skin examination had been six months earlier and the lesion had not been present. He previously had three basal cell carcinomas (on the left temple, left side of his upper lip, and left mid back excised 32 years, four years and two years earlier, respectively) and one squamous cell carcinoma (on his right upper back that was excised seven years ago). He also had actinic keratoses that were treated with liquid nitrogen cryotherapy.\nHis past medical history was significant for severe acne vulgaris as an adolescent, hypercholesterolemia, hypertension, and prostate cancer that was diagnosed one year ago. He is currently with no evidence of malignant disease after treatment which included a robotic-assisted laparoscopic prostatectomy (with negative margins for tumor) and a bilateral pelvic lymph node dissection (with none of eight nodes positive for cancer). His current oral daily medications included amlodipine 10 mg and simvastatin 20 mg.\nCutaneous examination showed a six by six millimeter flesh-colored nodule on the extensor aspect of his left arm near the elbow (Figure ). A shave biopsy of the superficial portion of the nodule was performed. The site was treated topically with mupirocin two percent ointment, three times daily, until it had healed.\nMicroscopic examination of the hematoxylin and eosin-stained tissue specimen showed strands and nodular aggregates of atypical basaloid tumor cells in the dermis reaching the deep margin of biopsy; the overlying epidermis was thin with effacement of the rete ridges and sparse overlying orthokeratosis (Figure ). Deposition of amorphous material filled the dermal stroma between the tumor aggregates (Figure ); the amount of amyloid present was abundant (+++). The lateral aspect of the specimen showed solar elastosis in the upper dermis above the tumor and below the epidermis (Figure ).\nCorrelation of the clinical presentation and pathological findings established the diagnosis of a nodular basal cell carcinoma with extensive amyloid deposition and adjacent solar elastosis. The residual tumor was excised and a side-to-side layered closure was used to resolve the surgical wound. The surgical site was healed without recurrence at a follow-up examination nine months later.
A 56-year-old male presented with a chief complaint of “my lower teeth are crocked.” His dental history included multiple restorations, fixed prosthetic, and root canal treatments.\nClinical examination revealed complete lip competency with small chin and no mentalis muscle strain. His profile was convex and on smiling, he displayed almost 80% of his maxillary incisors with no gingival display. Molars and canines were in Class I relationship. The overbite was 60% and overjet was 4 mm, with the maxillary and mandibular midline coincident to one another and to the face. Oral hygiene was fair although there was localized mild gingival recession. The maxillary arch was well aligned, with mild anterior crowding. Bolton analysis indicated a maxillary dental excess of 1 mm. There was 6 mm of crowding in the mandibular anterior region.\nThe panoramic radiograph showed a full permanent adult dentition with multiple restoration and root canal treatments. There was mild generalized bone loss and root morphology was within normal limits (). Cephalometric findings included a well-positioned maxilla and mild retrognathic mandible, resulting in mild Class II skeletal pattern. The maxillary and mandibular incisors were proclined and protruded.\nThe primary objective of the treatment was to address the patient's chief complaint, that is, to resolve the mandibular crowding and improve the overjet while avoiding more proclination of the mandibular incisors.\nSeveral treatment alternatives were presented to the patient. The first was to extract all first premolars in both arches to alleviate the crowding and to achieve proper inclination and position of the anterior teeth. The problem with this option was that it was too aggressive.\nThe second option was to alleviate the maxillary and mandibular crowding by interproximal reduction using the air rotor stripping technique [, ]. However, the mandibular anterior teeth were not suitable for interproximal reduction due to their small size and shape. Furthermore, air rotor stripping of the posterior segment was not a suitable choice because of the Class I occlusion and presence of posterior crowns.\nThe third option was to extract a mandibular incisor to relieve the crowding. This plan would maintain the mandibular incisors in their current position and inclination while maintaining the Class I molar and canine relationship. A diagnostic wax set-up was made to confirm this treatment option. The major drawbacks to this option were that a mandibular incisor would have to be extracted, the mandibular midline would be lost, and the overjet could be increased.\nThe patient was presented with all options and he agreed to the mandibular incisor extraction using the Invisalign appliance. The mandibular left central incisor was selected in this case because it was the most malaligned and lingually positioned and thus contributed most to the crowding, and also the attached gingiva was the least healthy of all the mandibular incisors.\nAfter the mandibular left central incisor was extracted (), maxillary and mandibular polyvinyl siloxane impressions were taken and sent to Invisalign to fabricate the aligners. A proprietary Align Technology software ClinCheck system (Align Technology, Santa Clara, CA, USA) generated the patient's tooth set-up and stages of tooth movements in three dimensions and was reviewed by the orthodontist on a computer.\nBefore the delivery of the first aligner, vertical composite attachments were bonded to the mandibular teeth to prevent tipping during space closure and a button attachment was bonded on the maxillary right central (). The patient was seen every four weeks for delivery of new aligners and to monitor treatment progress and aligner fit. The patient was instructed to change to the next set of aligners every 2 weeks. Twelve aligners were required in the maxillary arch and 20 for the mandibular. Interproximal reduction was required in the maxillary lateral and central teeth with a total of 0.5 mm of reduction on each side.\nTotal treatment time was 10 months. The patient was then given Essex-type maxillary and mandibular retainers to be worn at night.\nThe Class I molar and canine relationships were maintained. The mandibular extraction space was completely closed and the maxillary incisors were well aligned. Even though interproximal reduction was performed between the maxillary incisors, the overjet was not changed and was acceptable to the patient. The gingival recession in the mandibular right central incisor region did not change during treatment and both arches showed good alignment ().\nThe posttreatment panoramic X-ray revealed a relatively well-aligned upper and lower incisor roots ().
A 26-year-old housewife and resident of Karachi, Pakistan with a known history of polycystic ovarian syndrome was admitted to the emergency department with complaints of abdominal and chest pain, loose stool, vomiting, shortness of breath and reduced urine output. She developed abdominal pain 5 days prior to the admission. The pain was generalized, and nonradiating in nature. Her pain scored 8/10 on the pain scale. It was also associated with nausea, vomiting and abdominal distension. She also had had loose watery nonbloody stools with a frequency of 4–5 episodes per day in the previous 4 days. This was accompanied by progressive shortness of breath, reduction in urine output and peripheral edema. Her past medical history included primary infertility from polycystic ovarian syndrome, which had been diagnosed 8 years back. She was started on intravenous GnRH agonist for induction of pregnancy, and she had received 18 intravenous injections in the past 20 days before admission. On top of this, she had also received 11 injections of follicle-stimulating hormone. Keeping in mind the drug history, gastrointestinal symptoms and anasarca, a diagnosis of OHSS was made. At the time of presentation to the emergency department, she was drowsy with a regular pulse of 120 beats/min and had a blood pressure of 70/50 mm Hg. She was afebrile, but was tachypneic with a respiratory rate of 32/min. Her oxygen saturation was 85% on room air. She was pale, with ankle and sacral edema. Cardiovascular examination revealed sinus tachycardia and normal first and second heart sound. Respiratory examination showed bilateral basal rales and decreased air entry at the bases. Her abdomen was tender with evidence of tense ascites. Neurological examination showed a decreased mental state with no evidence of focal neurological deficit. Our patient underwent various laboratory tests shown in table and table . In view of the hemodynamic instability, she was managed in a special care unit with intensive monitoring. She required ventilatory support through BIPAP because of increasing respiratory compromise and type I respiratory failure. She was started empirically on intravenous piperacillin/tazobactam (Tazocin) 2.25 g every 6 h because of raised white blood cell counts, C-reactive protein and raised procalcitonin. Her ascites was drained through a pigtail catheter as it was felt that this was impeding her respiratory effort. She was also supported by renal replacement therapy (hemodialysis) primarily for fluid removal in the first dialysis. However, after the first dialysis, she had a persistently low central venous pressure (6–8 mm Hg) and hypotension, and no fluid was removed during the subsequent 3 sessions. After draining of ascites, her tachypnea improved. She was cautiously given boluses of saline along with albumin intermittently to keep the central venous pressure between 10 and 12 mm Hg.\nHer ascitic fluid analysis showed glucose of 100 mg/dl, protein 3.8 g/l, and her total leukocyte count was 1,397/μl. Her differential leukocyte count was 80% neutrophils and 20% lymphocytes. Her blood and urine cultures were negative but the ascitic culture grew S. maltophilia which was sensitive to piperacillin-tazobactam. The patient started producing urine on day 5. Her supportive management was continued. She did not need any further dialysis.\nOur patient made a gradual recovery through medical supportive measures and was able to come off dialysis and BIPAP. Her repeat procalcitonin and white blood cell counts showed a decreasing trend. Her care was deescalated to a general ward after 4 days and she was discharged home after 9 days. She was followed in clinic after 1 week. A total of 14-day course of antibiotics was completed. She completely regained her kidney function and her follow-up creatinine was 1.1 mg/dl.
A 26-year-old male patient presented with the chief complaint of generalized pus discharge from gums which he had been experiencing intermittently for the past 2 years (Figures and ). Pus discharge was associated with bad breath and usually subsided spontaneously after a few weeks. There was no associated complaint other than a generalized mild hypersensitivity to cold and sweet food. He had a history of extraction of lower left posterior tooth due to caries exposure and extraction of lower front tooth due to mobility about 1 year before. There was no history of any other dental treatment.\nThe patient was systemically healthy, and medical history did not reveal any relevant findings. Family history revealed that the patient's mother had similar complaints of mobility, pus discharge, and spontaneous exfoliation of some teeth following which she consulted a dentist and underwent total extraction by the age of 40. The patient was a nonsmoker, and there was no history of use of any other forms of tobacco.\nExtraoral examination revealed bilateral submandibular lymph node enlargement, which was firm, mobile, and nontender.\nAll teeth were present except for 46, 26, and 41. Tooth 46 was extracted due to caries and 41 was extracted due to mobility. Tooth 26 was grossly decayed with just root stump present. The oral hygiene status of the patient was fair with moderate deposits of calculus and plaque.\nIntraoral examination revealed a normal color of gingiva except in the labial aspect of 31, 32, and 33 where the marginal gingiva was slightly reddish. Gingival margins were rounded, and exudation was present in relation to labial aspects of mandibular anterior teeth and maxillary central incisors. There was generalized bleeding on probing and recession in relation to most of the teeth, especially more in maxillary central incisors and mandibular anterior teeth.\nThere was grade I mobility of 15 and 22 and grade II mobility of 11, 12, 21, 31, 32, 33 and 42. Proximal contacts were lost between maxillary and mandibular anterior teeth with pathologic migration of 11, 21, 31, 32, and 42 and extrusion of 31. Grade II furcation involvement was present with molars and maxillary first premolars. A full mouth periodontal examination revealed generalized deep periodontal pockets and severe generalized clinical attachment loss ().\nSevere periodontal destruction was evident with more than 10 mm of clinical attachment loss at multiple sites especially in the incisor and canine regions.\nOPG and IOPA X-rays revealed a generalized distribution of periodontal bone loss especially severe in the incisor and canine regions with the molars and premolars affected to a lesser degree (). There was predominantly vertical bone loss in the canine and incisor regions. Routine blood investigations were within normal limits.\nA diagnosis of generalized aggressive periodontitis was made according to the established criteria (American Academy of Periodontology, 1999).
An 82-year-old male with squamous cell carcinoma of the left posterior mandibular gingiva underwent a partial mandibulectomy (#35 tooth to the left mandibular angle) combined with left supraomohyoid neck dissection. Reconstruction of the composite tissue defects of the mandible was conducted with a radial forearm free flap (RFFF) for the soft tissue and a customized 3D-printed titanium block at other general hospital in Korea. The RFFF failed due to venous thrombosis, and the titanium block set in the mandibular defect was exposed out of the left submandibular area as well as the oral cavity, resulting in a wide orofacial fistula. Soft tissue reconstruction was performed using a pectoralis major myocutaneous flap to close the fistula and cover the exposed titanium block. However, the soft tissue coverage failed again and the chronic fistula was followed by severe scar contraction. When the patient was referred to the department of oral and maxillofacial surgery in Asan Medical Center 6 months after the first surgery, there was a severe infection around the exposed titanium block (Fig. ). The presence of residual tumor was confirmed in MRI images taken after visiting our clinic. Therefore, we removed the titanium block and residual failed RFFF and performed further resection from the mesial surface of the #42 tooth to the ascending ramus of the left mandible. For the wide defect of the composite tissue defect, left side mandibular reconstruction was done with a FFF. The contour of the fibula was designed on a virtual surgery simulation and a 3D surgical template was printed (Aview(R) Modeler; Coreline Soft, Seoul, Republic of Korea). The harvested fibula was osteotomized into two parts to align with the planned contour of the reconstructed mandible. The contoured FFF was fixed by semi-rigid fixation with mini plates and mono cortical screws. After the fixation of the fibular bone, vessel anastomosis was performed, and flap perfusion was confirmed. The peroneal artery and one vena comitans were anastomosed with the facial artery and vein on the contralateral side (Fig. ).\nSevere atrophy of the soft tissue at the surgical site due to scar contraction and breakage of normal occlusion, which was established intraoperatively, gradually appeared over 1 year following FFF reconstruction. Facial asymmetry developed as the midline mandible dentition severely deviated toward the side that was replaced with FFF. Therefore, an orthognathic surgical correction method was planned to solve the malocclusion and facial asymmetry caused by the scar contraction. After taking an alginate impression of the remaining dentition, a stone model was fabricated and an occlusion wafer with ideal occlusion was made. First, an arch bar was applied to the remaining teeth for intermaxillary fixation. In the mandible on the right side, the mandibular movement was obtained through SSRO. However, the virtual simulation surgery indicated that SSRO would not be sufficient to achieve normal occlusion, which was confirmed intraoperatively. Therefore, we also performed a sliding osteotomy at the anterior junction between the FFF and the #42 tooth on the mesial side. Through this, we confirmed that normal occlusion could be established, and the occlusal wafer was set between the maxilla and the mandible. After intermaxillary fixation, the segmented bones were fixed with mini plates and screws. Elastic guidance was applied to maintain the reestablished normal occlusion for 4 weeks (Fig. ). Afterward, three fixtures of the dental implant were installed onto the FFF 3 months after surgery for stable occlusion and facial contour. The stable occlusion was maintained both at rest and during mastication after prosthodontic treatment.
A 50-year-old woman (height 155 cm, weight 60 kg) first presented to our hospital with left hip pain, limited range of motion, and limping. Trendelenburg gait was observed. She did not have a history of treatment for DDH. She developed hip joint pain and underwent right DTT at the age of 20 years. Her hip pain had improved after the surgery; however, she started to feel pain again at 2 years after DDT. She underwent right-sided THA at another hospital at the age of 47 years.\nRadiographs showed severe osteoarthritis of her left hip joint and the left greater trochanter, which had moved to the level of the lesser trochanter. The past THA on the right hip joint was visible as well (). The LLD was 55 mm.\nThe right THA was performed at the age of 47, and the left THA was performed at the age of 50. The THA procedures comprised conventional cementless THA in the same way as in Case 1 (anesthesia, surgical approach, implants, and rehabilitation).\nIntraoperatively, the surgeon had to pay close attention to identify the gluteus medius and minimus muscles and prevent damage to the same muscle due to hypertension during the reduction after implant placement, similar to the circumstances of case 1. The operation time was 60 min, and the total blood loss was 488 g (intra and postoperative blood loss). The leg lengthening was 27 mm, and 28 mm of the LLD persisted.\nAs in Case 1, the pain improved remarkably; however, the patient presented with LLD due to an abduction contraction following extension of the leg length. The abduction contraction improved within two months under physical therapy. No other complications, such as dislocation, neurovascular abnormality, or infection, were observed. Final radiographs at 11 years after left THA did not show subsidence of the stem or any type of loosening around the cup or stem (). The preoperative JOA hip score was 39 points, which improved to 71 points at 11 years after surgery. Trendelenburg gait had improved.
A 66-year-old female with a history of mucinous adenocarcinoma of the cervix presented to the neurosurgery outpatient clinic for evaluation of a sellar mass found during workup of adrenal insufficiency and hypothyroidism. The patient did not have unusual headaches or vision problems. Three months prior to discovery of the sellar mass, she was diagnosed with stage IIb mucinous adenocarcinoma of the cervix and was treated with chemotherapy. At the time of neurosurgery clinic presentation, she was neurologically intact, including full visual fields. Laboratory work-up demonstrated pituitary insufficiency with central hypothyroidism.\nThe initial magnetic resonance imaging (MRI) revealed a 1.8 × 1.1 cm contrast-enhancing mass within the sella, with extension to the suprasellar cistern and optic chiasm abutment. Preoperative imaging obtained the following month in preparation for surgery demonstrated that the mass had grown to 2.2 × 1.5 cm ().\nThe patient underwent an endoscopic endonasal approach for resection of the intradural sellar mass. Intraoperative findings demonstrated a very firm, infiltrative, vascular mass with dense adherence to surrounding structures, including the dura, medical cavernous walls, and diaphragma. Intraoperative frozen section pathology was consistent with metastatic carcinoma. The tumor was debulked until normal appearing pituitary tissue was identified and the margin of tumor adherence to the diaphragma was reached. Postoperatively, the patient did well without new hormonal deficiencies or vision problems. A subtotal resection (>80%) was achieved (). Gross histology and immunohistochemical staining ultimately confirmed the diagnosis of metastatic mucinous adenocarcinoma of the cervix (Figures and ).\nThe patient's immediate postoperative course was unremarkable. Given the diagnosis and intraoperative/postoperative findings of subtotal resection, adjuvant chemoradiation therapy was encouraged but the patient refused additional treatment. She was discharged home two days after surgery. She developed decreased left eye visual acuity and ptosis one week after surgery. A CT of the head at this time did not show any intracranial hemorrhage and a repeat MRI showed new enhancement suggestive of tumor recurrence within the sellar and suprasellar regions. The patient was started on steroids but declined any further treatment, including repeat surgery. Her ophthalmic symptoms ultimately progressed to a complete left cranial nerve III palsy four weeks after surgery. Follow-up MR imaging at five weeks after surgery revealed significant progression of the tumor to 2.9 × 2.4 cm with significant suprasellar extension (). Though a computed tomography scan of the chest, abdomen, and pelvis at this time demonstrated no new neoplastic burden, a radiotracer bone scan demonstrated likely new metastatic lesions in the skull, bilateral humeri, bilateral acetabula, bilateral femurs, and the lumbosacral vertebrae. After further discussion with her gynecologic oncologist and radiation oncology, the patient again refused pursuing any further treatment, including palliative radiation or systemic therapies and elected to pursue home hospice. The patient died approximately two months after surgery.
A 62-year-old woman was presented to the First Affiliated Hospital of Wenzhou Medical University, in November 2018 for palpable masses in the right neck for a week. Five years ago, in addition to the right hemithyroidectomy, she was treated in a local hospital for thyroid nodules with routine ipsilateral stage VI lymph node dissection. The subsequent histopathologic analysis revealed papillary thyroid cancer follicular variant (FVPTC). After the operation, the patient took a daily dose of levothyroxine tablets (100 μg).\nIn November 2018, the patient discovered multiple palpable masses in the right neck near the scar from the first operation and came to our hospital for further examination. Physical examination revealed several palpable nodules with a maximal size of about 1*1 cm located within the scar site of the previous surgery (). Neck ultrasonography revealed the presence of three hypoechoic nodules with a maximum size of 9*7*9 mm subcutaneously, at the right anterior neck and the right supraclavicular, characterized by irregular edges, irregular internal echoes, and intramodular blood flow (). We performed a fine-needle aspiration biopsy of one of the nodules to explain the histopathology of the nodules. The histologic findings suggested thyroid cancer infiltration or metastasis, and we recommended further surgical treatment, which she accepted. A preoperative enhanced computed tomography scan showed increased subcutaneous soft tissue mass in the right neck and multiple enlarged lymph nodes on both sides of the neck (). However, the thyroglobulin was normal, and the thyroid autoantibody tests were negative.\nFollowing the completion of the preoperative examination and removal of the contraindications, right lateral (levels III/IV) neck dissection with wide excision of subcutaneous tumor tissue in addition to residual thyroidectomy was performed under general anesthesia. During the operation, we completely removed the superficial layer and nodules of the subcutaneous cervical fascia, the thyroid cartilage, the superior margin of the clavicle, the bilateral layer of the sternocleidomastoid muscle, and the anterior cervical fascia (). Three nodules with a maximum size of 8*8 mm were visible in the right subcutaneous and subclavian epithelium of the anterior neck, with a relatively hard texture (). Histopathology revealed the presence of thyroid tissue in the latissimus muscle, while the subcutaneous nodules were considered as infiltration or metastasis of FVPTC. Microscopic examination revealed atypical irregular follicular cells with ovoid nuclei, nuclear overlap, and nuclear grooves without papillary structures, as well as surrounding fibrous tissue reaction (). However, no metastatic lymph node was observed. The surgical operations were successfully performed, and the patient recovered well. After surgery, the patient received radioactive iodine therapy and was regularly monitored. She continued with the daily levothyroxine tablets (200 μg) dose and her condition is currently stable, with no signs of disease progression.
A 63-year-old Caucasian man was admitted to our hospital for further workup of a solitary pulmonary lesion. A simultaneous diagnosis of stage I renal cell carcinoma of the left kidney and adenocarcinoma of the rectum had been made in March 2005 which was treated by left nephrectomy, total mesorectal resection, and adjuvant radiochemotherapy according to the AIO protocol. Postoperative tumor staging and grading of the rectal carcinoma was pT3, pN0 (0/15), G2, R0, and M0. Regular followup until July 2010 was without pathological findings and the carcinoembryonic antigen (CEA) and CA19-9 serum concentrations were normal. A computed-tomography-guided core biopsy of the pulmonary lesion was performed; histological and immunohistochemical analysis showed an adenocarcinoma consistent with metastatic colon cancer. On further workup, an abdominal ultrasound showed a 25 mm polypoid lesion within the gallbladder (). Contrast enhanced ultrasound was performed for further characterization. Imaging in low mechanical index (MI) technique after intravenous injection of 5 mL sulphur hexafluoride microbubbles (SonoVue) showed an intense homogenous contrast signal within the gallbladder mass. Contrast enhancement was detected after 20 seconds and preceded the appearance of contrast in the adjacent liver tissue by approximately 20 seconds. The contrast signal decreased over time but no complete washout phenomenon was observed (see and Supplementary Video Sequence in Supplementary Material available online at ). The diagnostic and therapeutic options were discussed by the multidisciplinary tumor board and a decision was made to resect the pulmonary metastasis and to perform a cholecystectomy. While the pulmonary tumor was confirmed to represent metastatic colon cancer, an intramucosal metastasis of a clear cell renal cell carcinoma was found in the gallbladder (). After successful complete resection of both metastatic lesions, the patient remained without recurrent disease for more than two years. In November 2012, a computed tomography (CT) scan showed hepatic metastases which were considered metastatic renal cell carcinoma because of the radiologic appearance. The patient was therefore treated with pazopanib. Followup in February 2013 showed marked tumor regression.
A healthy 22-year-old Caucasian male referred to our department suffering from pain and limited range of motion (ROM) of the right ring finger. The patient sustained in August 2015 a penetrating injury on the palm of the right hand during his sport activity. He did not refer to any emergency department or general pratictioner, the wound was small and healed naturally. One month later pain started and limitation of ROM of the right ring finger gradually worsened. Because of his clinical disorders, a trigger finger was diagnosed by another orthopedic who performed a steroid injection. The treatment was ineffective, then he referred to our department in November 2015. The clinical examination showed a small scar on the palm, located proximally to the fourth metacarpophalangeal joint and the ROM of the proximal interphalangeal (PIP) joint of the ring finger was limited to 40 degrees of extension and complete (100 degrees) in flexion, both in passive and active motion. The feeling of numbness just under the scar, the first steroid injection that was not effective and the young age of the patient, led us to perform an ultrasound examination of the hand tendons (), which showed a suspect tear of the flexor digitorum superficialis tendon of the ring finger, proximally to the pulley A1 that appeared thickened. The surgical exploration of the tendon was the treatment of choice, which was performed 2 months and 10 days from the injury.\nThe incision was made at the pulley A1 and extended Brunner-like proximally in direction of the scar. A partial longitudinal flap-shaped tear was discovered, large approximately half of the entire tendon diameter, of the radial half of the flexor digitorum superficialis tendon that hooked at the entrance of the A1 pulley (). The flexor digitorum profundus tendon was intact. Because of the size of the flap-shaped tear, it was decided not to remove it, but it was sutured to the tendon in anatomic position with simple stitches (nylon 4-0) and the pulley A1 release completed the procedure to avoid risk of post-operative re-triggering. The examination during surgery showed the restoration of ring finger ROM (0–100 degrees) and the disappearance of the trigger, then the exploration of the distal pulleys (A2 and A3) was not necessary. Also, a capsulotomy of the PIP was not necessary because the limitation of ROM was not due to a capsular contracture. The patient was recommended to frequently move the fingers even with the bandage, to prevent the formation of adhesions. The cutaneous sutures were removed after 15 days. There was no need for physiotherapy as the ROM has been completely restored almost immediately. After 3 months of follow-up, no evidence of relapse was recorded and the ROM was 0–100 degrees.
A 14-year-old male had an extensive flame burn in the right side of neck, face, and scalp, anterior chest, and right upper limb at the age of 2 years.\nNo past medical or surgical history. No known drug history, no family history of any genetic disorder, patient and both parents are non-smokers.\nHe received multiple skin grafting procedures and an amputation of the right hand. The burn to his scalp was treated with a burr hole to the outer cortex to allow granulation tissue to cover the scalp, and then the wound was skin grafted.\nThe patient chronically continued to have unstable skin over the scalp and kept developing recurrent ulceration. On 29 March 2012, the patient was admitted throw the clinic due to his chronic condition of recurrent open wounds for many years.\nA plan was set for the removal of the unstable ulcer and scarred skin of the scalp and coverage with an omental free flap and split thickness skin graft ().\nUnder general anesthesia with endotracheal intubation, the patient was in supine position, with draping of the head circumflex, face, abdomen, and left thigh down to the knee.\nThe General Surgery team was consulted for laparoscopically harvesting the omental flap and was performed by Dr I.Anwar based on the left gastroepiploic artery. The right superficial temporal artery was identified to be the recipient vessel ().\nThe ENT team did a right neck exploration to explore the neck vessels as a backup in case the right superficial temporal artery was not suitable.\nThe free omental flap based on the left gastroepiploic artery was anastomosed to the right superficial temporal artery. The micro procedure of omental free flap was performed by DR F.Hashem, Plastic surgeon.\nThe patient was given heparin 2000 U intraoperatively. The flap was viable with bleeding with a positive Doppler signal.\nAfter establishing flap revascularization, all the excising unstable scarred and ulcerated skin covering the scalp was resected, and the underlying irregular skull was smoothed down using a flat burr. The omental flap then covered the scalp, and a split-thickness skin graft from the thigh was placed over the omental flap ().\nThe patient was monitored postoperatively in intensive care unit. Post-operative care for microsurgery and wound care for skin graft over omental flap.\nThe hospital course was uneventful. The patient was kept on IV anticoagulants (dextran and heparin) for 5 days.\nThe patient tolerated the procedure well. No wound complications were reported during his two years follow-up (). Re-Exploration nor revision of the surgery were needed
A 71-year-old male patient was transferred to our department due to soft tissue defect in the left lower leg and infected Achilles tendinitis. The patient underwent incision and drainage of both lower legs with necrotizing fasciitis, at another hospital two months ago. Continuous wound care was performed; however, the left leg open Achilles tendinitis and soft tissue defects were not resolved.\nPhysical examination revealed a 12 × 5 cm wound with exposed Achilles tendon over the posteromedial aspect of lower one-third of the leg (Fig. ). His wound culture grew methicillin-resistant Staphylococcus aureus (MRSA).\nWe performed an operation with the patient placed in the supine position. All infection associated with necrotic Achilles tendon in the proximal muscle tissue was excised (Fig. ). After debridement, the patient had a 16 cm tendon defect from the muscle with the ankle joint in neutral position. He had 2 cm of the distal tendon attached to the calcaneus. We extended the skin incision to the outside of the zone of injury in the anterior aspect of the ankle, dissected anterior tibial artery and vena comitantes to perform vascular anastomosis out of injury zone. We made a template with surgical glove, which included the vascularized fascia lata for the reconstruction of Achilles tendon (Fig. ). We used the already manufactured template on the ipsilateral thigh, centering the flap over the perforator and drew the flap larger than the recipient site (Fig. ). A 14 × 7 cm ALF flap with a large piece of fascia lata (bilaterally, approximately 2 cm extra fascia is taken) was harvested (Fig. ). The donor defect was closed primarily over a silicon drain. For the reconstruction of tendon, the fascia lata was repaired first using multiple figure-eight sutures and modified Becker method[ with 4–0 prolene sutures at the separated end-to-end of the Achilles tendon. An end-to-end microvascular anastomosis was performed between the anterior tibial vessels and the flap pedicle vessels using 9–0 sutures microscopically after inserting the flap into the defect (Fig. ).\nPostoperatively, the ankle and leg were wrapped in a bulky dressing and immobilized with an above-knee splint and the flap was monitored intensively for 7 days. Anticoagulation therapy with prostaglandin E1 (10 μg/day) and heparin (5000 units/day) were administered for 1 week and aspirin 100 mg once a day for 4 weeks after the surgery. The flap survived completely without complications. Passive and active exercise of the ankle joint was started at 6 weeks after surgery. Subsequently, the patient underwent a graduated rehabilitation program, from a non-weight bearing exercise to partial-weight bearing exercise. Twelve weeks after the surgery, the patient was permitted full-weight bearing with gait training.\nAt 12 months of follow-up, the patient was able to resume full daily activities, felt a little discomfort at the donor site after more than 2 h of hiking, but was able to walk without pain and without the need for support, also was able to squat, showed an ankle range of motion of 15° dorsiflexion and 45° plantar flexion, and the American Orthopaedic Foot and Ankle Society (AOFAS) score was 94 (Fig. ).
A 45-year-old male patient, known to have white matter leukodystrophy and generalized spasticity of unknown etiology which started at the age of 14, was progressive and kept bedridden for the past 7 years for which a Baclofen pump was inserted. Also, he had mental disability. The patient was diagnosed with locally advanced low rectal cancer with distant metastasis to multiple organs including perineal and inguinal skin, lung, external iliac, colon, and inguinal lymph nodes metastasis. In 2018, about 1 month prior to referral to King Faisal Specialist Hospital and Research Center, the patient was following with Neurology regarding his condition, where he was found to have lower GI bleeding and surgery was involved. He underwent investigation for lower GI bleeding including colonoscopy, and he was found to have rectal mass and could not pass the scope above it as well as the skin lesion which was biopsied. The case was discussed in the multidisciplinary tumor board and planned for diversion loop colostomy as well as rectal biopsies and inguinal area skin biopsy followed by palliative radiation therapy to the pelvis. Computed Tomography (CT) scan of the abdomen and pelvis (, , ) demonstrated a circumferential enhancing wall thickening involving the whole rectum with ill-defined hypodense area seen 7–9 o’clock with possible involvement of the anal canal associated with diffuse edema and fat stranding of the mesorectum. There were multiple necrotic lymph nodes noted in the mesorectum and bilateral internal iliac region, the largest one in the right internal ilium measuring 2 cm. There were multiple necrotic lymph nodes seen on the right external iliac (measuring 1.6 cm) and bilateral inguinal area, the largest one on the left side measuring 3.4 × 3.3 cm. There were bilateral symmetrical hilar necrotic lymph nodes measuring on the right side 2.5 × 2 cm and on the left side 3.2 × 1.6 cm. At the perivascular space, they measured 1 cm, being at least T3 N2.\nMagnetic Resonance Imaging (MRI) for local staging was contraindicated as the patient was with an implanted pump. CT Chest () demonstrated multiple bilateral tiny pulmonary nodules, the largest one measuring 4 mm in the right upper lobe. Upon examination, the patient was bedridden with poor functional status. Glasgow coma score (GCS) was 15/15, having generalized spasticity. Perineal examination revealed multiple exophytic masses in the scrotal skin, inguinal folds, and perineum and gluteal folds, which were firm to hard in consistency with a few being ulcerated especially over scrotum with little oozing of the serosanguinous fluid. There was no mechanical obstruction of orifices (anal or urethral). The patient was in the general surgical ward to continue postoperative care and management. Histopathological exam of rectal biopsies revealed moderately differentiated rectal adenocarcinoma, while the skin of the right inguinal area showed metastatic cutaneous rectal adenocarcinoma (a, b, c). Unfortunately, later, the patient developed respiratory failure secondary to aspiration pneumonia which ended by cardiopulmonary arrest and death.
A 67-year-old man from India, who was recently diagnosed with latent tuberculosis, presented to an outside hospital and was admitted for six days with generalized fatigue and hypotension. On presentation, he reported a 2-week history of generalized fatigue with systolic blood pressures in the 60s, as recorded at home. He also reported a 30-pound weight loss which was attributed to diuretic use and dietary changes in the setting of congestive heart failure. With these complaints, his losartan (angiotensin receptor blocker) dose was reduced, which was ineffective in alleviating his symptoms. His history was notable for an LTBI diagnosed three months prior to presentation, for which he was being treated with INH (300 mg daily) and pyridoxine. He had been treated for 11 weeks by the time he presented, and was noted to tolerate the therapy well for at least four weeks without changes in dietary or sleeping habits, per documentation by his primary care provider. His medical history was otherwise notable for atrial fibrillation, beta thalassemia, and tachycardia-induced cardiomyopathy with reduced ejection fraction. He had no known history of liver disease or diabetes. His other medications included apixiban, metoprolol, furosemide, losartan, and hydroxyzine (for insomnia). He had no prior history of heavy alcohol consumption or recreational drug use, and he worked in the electronics and computer industry. There was no family history of cirrhosis or other liver disease.\nHis initial workup was notable for elevated liver function tests, as shown in . A computed tomography scan of the abdomen demonstrated no intrahepatic biliary dilation and no apparent fatty change. An abdominal ultrasound revealed a simple cyst and heterogenous echotexture, with mild subcapsular nodularity. Based on his workup, his liver dysfunction was postulated to be drug-induced liver injury from INH or apixaban. Both medications were discontinued. He was discharged for outpatient management. However, follow-up laboratory testing indicated an increasing bilirubin, which resulted in readmission to the hospital. A liver biopsy was considered but not performed, as his bilirubin levels began to downtrend.\nOne week later, the patient experienced a syncopal episode while having a bowel movement, and was admitted to a different hospital. He presented with altered mental status and generalized pruritis. Laboratory data revealed worsening liver function () and acute kidney injury, for which he was treated with albumin, midodrine, and octreotide. His pruritis improved with the introduction of cholestyramine.\nAutoimmune serologies, including antinuclear antibody, antimitchondrial antibody, and anti-smooth muscle antibody, were negative. Viral hepatitis serologies were negative. HIV infection was ruled out. The patient’s acetaminophen level, urine toxicology screen, and serum ferritin level were within normal limits. A transjugular liver biopsy was performed and demonstrated cholestatic hepatitis, thought to be drug- or toxin- related. His mental status initially improved with lactulose, but worsened thereafter, prompting a transfer to our hospital for consideration of liver transplantation for acute liver failure, about six weeks after initial presentation.\nPhysical examination at time of transfer was remarkable for jaundice, icteric sclera, and altered mental status. He was somnolent but arousable to noxious stimuli, oriented only to self, intermittently following some commands, and had notable asterixis. He had an irregular rhythm and a murmur on cardiac examination, vesicular breath sounds, and a benign abdominal examination. He was noted to have Grade III hepatic encephalopathy in the setting of acute liver failure, and was admitted to the intensive care unit before being listed for liver transplantation as Status 1A. On day four in the hospital, he underwent orthotopic liver transplant surgery, and was extubated on post-operative day one. He was transferred to the floor on post-operative day three, but subsequently suffered a sudden aspiration event resulting in a pulseless electrical activity and fatal cardiopulmonary arrest.
A 57-year-old lady reported to our institution with an endometrial biopsy showing well-differentiated adenocarcinoma. Slide review confirmed the diagnosis. Magnetic resonance imaging (MRI) of the pelvis showed a 3 × 2 cm lesion in the endometrial cavity with superficial myometrial invasion without any enlarged pelvic or paraaortic nodes. She underwent robotic staging surgery for carcinoma endometrium in February 2015 using the da Vinci Xi robotic platform. As there is facility for fluorescence imaging in the robotic platform, it was decided to use ICG for sentinel mapping in her. Consent for the procedure was obtained from the patient and also from the hospital’s ethical committee.\nConcentration of ICG used was 0.5 mg/mL, a 4 mL of this solution was injected intracervically at 3 and 9 o’clock positions, both submucosally and deep into the cervical stroma. The injection was done after port placement but before the docking of the robotic platform. After complete inspection of peritoneal cavity and collecting pelvic washings, the fluorescent imaging was switched on. The time taken from the cervical injection till this point was 20 minutes. Under fluorescence imaging, the site of intracervical injection was well lit up but no lymphatics or lymph nodes were identified in the pelvic nodal areas. We then looked into the paraaortic area with fluorescence imaging and saw a lit up lymphatic channel crossing the right common iliac and lighting up of a right periaortic node (). Bilateral pelvic lymphadenectomy and removal of the lighted up paraaortic nodes were done. Complete paraaortic nodal dissection was not done as it is our practice to do paraaortic lymphadenectomy for high-risk histologies.\nHistopathology report showed 4.5 × 4 × 2.5 cm grade 2 endometrioid carcinoma infiltrating to outer one-half of the myometrium with lymphovascular emboli. Two out of three paraaortic nodes removed were positive for tumour metastasis. Three out of 11 left iliac nodes were also found to be positive, and the ten right iliac nodes removed were found to be negative. The case was discussed by the multidisciplinary tumour board, and it was decided to give her adjuvant radiotherapy with chemotherapy in view of node positivity.
A one and-a-half year old boy was presented to the emergency department with a four day history of coughing, repeated vomiting, irritability and refusal of food. His symptoms began after returning from a picnic. The boy had no prior history of breathing difficulties or cyanosis. A medical examination revealed that the boy had a low grade fever and a few crepitations in the left lower chest. A chest radiograph () revealed a rounded, coin-like foreign body at the cervicothoracic junction. An endoscopy performed under general anaesthesia revealed a foreign body in the upper esophagus at 15 cm from the incisors. The button battery was partially embedded in the esophageal wall, which was edematous and ulcerated with charred tissues around the foreign body. During manipulation, the patient's blood oxygen saturation level dropped, which suggested that signs of pneumothorax were present and this prompted the insertion of bilateral chest tubes. As a result, the patient's oxygen saturation level improved and the bilateral air entry was confirmed by a chest radiograph. Following a difficult manipulation, a lithium button battery was removed from the patient. The battery shell remained intact without signs of leakage. Regardless, the patient was kept on nasogastric tube feeds and broad spectrum antibiotic coverage. Ten days after the procedure, a gastrograffin esophagogram was performed, which did not reveal any evidence of a leak. Consequently, the patient was started on oral feeds and was discharged two days later, however was readmitted almost six weeks later, with complaints of neck pain, stiffness, restricted neck movement and intermittent fever since the previous two weeks. The MR imaging () showed evidence of spondylodiscitis involving the T1-2 disc with enhancing endplates and extension of the enhancing granulation tissues into the prevertebral region. In addition, narrowing of the tracheal lumen was also noted at this level. Also, there was diffuse enhancement in the prevertebral soft tissues around the esophagus, trachea and mediastinal planes, which suggested mediastinitis (). A gastrograffin esophagogram had not revealed any obvious leakage; however, the patient did improve with antibiotics and was afebrile, free of pain and had improved neck movement at the time of discharge.
A 17-year-old female senior high school student was referred to our cornea clinic with a 4-day history of pain, tearing, and vision loss in the left eye. She had used OK lenses for vision correction for 1 year. She often touched the lenses directly with her wet hands after washing her hands with tap water. She had received an antiviral treatment for 3 days at the previous clinic, but the symptoms worsened before referral to our cornea clinic. Upon presentation, her visual acuity was counting fingers at 15 cm. Slit-lamp examination revealed stromal edema () and a corneal ulcer measuring 2 × 2 mm with fluorescent staining (). Confocal laser microscopy revealed structures highly resembling amoebic cysts within the ulcer (). She was treated with 0.02% chlorhexidine eye drops hourly for 7 days. The corneal edema subsided () and the fluorescein staining was weakly positive (). The 0.02% chlorhexidine eye drops were used every 2 hours for the following 4 weeks and then reduced to four times a day for another 4 weeks. At the 2-month visit, her corrected vision was 20/20 with slight corneal opacity (). The eye drops were discontinued when the absence of amoebic cysts was confirmed by confocal laser microscopy. She developed no recurrence during the 7-month follow-up.\nIn total, 289 articles were identified using the index words from the three databases (PubMed, n = 175; Embase, n = 110; Cochrane Library, n = 4). After removing 127 duplicate articles and 3 articles with no authors by Endnote software or manually, 159 articles were screened by reading their titles and abstracts. When summarizing these individual cases, we collected the complete patient information, OK lens wearing status, diagnostic examination findings, and treatment plan. Based on our strict inclusion and exclusion criteria, we identified 13 relevant case reports for data analysis after removal of review articles, general serial reports, and irrelevant reports. These 13 case reports involved 24 eyes of 20 patients using OK lenses (). Among all reported cases, the patients’ ages ranged from 9 to 41 years and the highest incidence of AK occurred in the 10- to 19-year age range. The mean age at presentation was 19.4 ± 8.2 years, and female preponderance was identified (male:female ratio of 1.0:2.3). The most common risk factor for AK in OK lens wearers was rinsing the lenses or cases with tap water. Practices such as inappropriate lens care procedures, patient noncompliance with the practitioner’s instructions, and persistent lens use despite discomfort emerged as potential risk factors for AK in OK lens wearers. The performance of microbiological culture as the diagnostic method was reported in 83% of the cases. Among them, one patient had bilateral symptoms but was culture-positive on only one side. Confocal microscopy was used for confirmation in three eyes. The median duration from onset of symptoms to diagnosis was 20 days. Among all 20 patients, 42% had poor outcomes as defined by best-corrected visual acuity of less than 20/40 or requirement of surgery.
We report the case of a 38-year-old pregnant lady with a diagnosis of idiopathic aortitis associated with aortic root aneurysm and severe aortic valve regurgitation.\nThe patient had a history of cardiac murmur, having had an echocardiogram performed two years before the episode showing a mild mitral regurgitation with a normal aortic valve and aorta. She was asymptomatic until the 4th month of pregnancy when she began with tachycardia and exertional dyspnea which rapidly evolved to rest dyspnea; she was admitted to the hospital, and an echocardiographic study was performed, showing a very severe aortic regurgitation and a 5.5 cm aneurysm of the root and ascending aorta; the left ventricle was markedly dilated with a borderline ventricular fraction. No computed tomography scan was done to avoid potential damage to the fetus.\nShe was transferred to our unit for urgent surgery for a planned valve-preserving surgery.\nShe was operated on under general anesthesia and median sternotomy. The aortic arch and the right atrium were cannulated in a standard fashion, and cardiopulmonary bypass (CPB) was established under normothermia. CPB flows were kept high maintaining a mean arterial pressure above 70 mmHg, and the use of vasoconstrictors was avoided [, ].\nThe aorta was clamped and opened showing a marked thickening of its wall. The root was dissected, and the coronary ostial buttons trimmed; the tissues around the root had heavy adherences indicating an inflammatory process. Geometric height of the leaflets was measured with a ruler; it was 15 for the noncoronary leaflet, 14 for the right, and 14 for the left. A valve-sparing remodeling procedure was done using a dacron straight graft of 24 mm. The technique of the remodeling procedure followed the recommendations of the Homburg group []. The post procedure intraoperative echocardiogram showed a residual mild regurgitation of the valve, which was considered acceptable. The jet was central, in its origin and direction, and the effective height of the repaired cusps was 8 mm.\nThe fetal status was assessed before surgery and after the completion of the procedure by transabdominal echography, showing an alive fetus.\nThe postoperative course of the patient was uneventful. She was discharged on metoprolol and iron. A transthoracic echocardiogram was performed before discharge showing a mild regurgitation of the aortic valve with a marked unloading of the left ventricle.\nShe continued her pregnancy, and a healthy girl was born with a vaginal delivery.\nShe was investigated thoroughly, and all known causes of aortitis or genetic disorders were discarded. No other signs or symptoms of the disease were found. She was diagnosed with idiopathic aortitis at that moment. Anyhow, she was started with immunosuppressant treatment with corticoids.\nShe underwent clinical and serial echocardiographic follow-up. The 6-month echo showed a progression of the regurgitation which was moderate at that time, and in the 1-year echo, it became severe. As she continued to be asymptomatic, a narrower follow-up was decided, but 3 months later, the left ventricle had dilated markedly, and redo surgery was indicated.\nRedo surgery was performed through a resternotomy with central cannulation. The Dacron graft was opened through a transverse incision, the aortic valve was measured and resected, and a 23 mm mechanical prosthesis was inserted in a supra-annular position with pledgeted sutures. The leaflets were markedly retracted at that time, with a geometric height of less than 4 mm. The Dacron graft was then sutured, and the patient was weaned from CPB and transferred to the Intensive Care Unit for an uneventful recovery.\nTwo years later, a new-onset supra-aortic vessel narrowing has been detected, and a diagnosis of Takayasu disease has been made.
A 65-year old male patient with atrial fibrillation conducted anterogradely through an AP, was hospitalized for catheter ablation. He presented recurring episodes of palpitations lasting minutes to hours every 2 to 3 weeks. The physical examination revealed a grade II apical systolic murmur and was otherwise normal, without any sign of left or right heart failure. The twelve-lead ECG showed sinus rhythm with a heart rate of 70 beats per minute, normal PR interval(160ms) with no delta wave. Echocardiography revealed a non-dilated left ventricle with a normal ejection fraction of 58%. Neither the 24-hour Holter ECG, nor the exercise stress test revealed presence of AP – the PR interval was normal and no delta wave was detected. Most probably AP has become latent due to chronic amiodarone treatment of 200 mg/d for several months.\nThe patient underwent an electrophysiological study under local anesthesia. During hospitalization, the ECG records in both atrial fibrillation and sinus rhythm revealed no AP (Figure A, B,). A catheter was placed in each of the: coronary sinus, right ventricle, high right atrium, and at the His bundle region. Programmed stimulation of the atria and ventricles was performed. A normal His-ventricle (HV) interval of 48 ms was documented without any sign of antegrade or retrograde conduction through an AP. After atropine injection repeated atrial and ventricular stimulation could not induce any reentrant tachycardia and the HV remained normal with antegrade and retrograde conduction through the nodal pathway. Atrial fibrillation was induced by aggressive atrial stimulation of 150 ms but it only resulted in narrow QRS and no conduction through an AP (Fig. ). As a result, we decided to inject adenosine boluses of 12, 18, and 36 mg followed by 10 cc of saline flush, with 3 to 5 beats conducted through a left lateral AP (Fig. ). Mapping could not be performed during the transient effect of adenosine. Since 5 mg of betablocker and 1 vial of Digoxin were injected but without any sign of AP, the AP was further mapped under continuous adenosine infusion of 1.5 mg/kg adenosine over 5 minutes. Under adenosine infusion the AP became apparent (Fig. ) and the mapping of the lateral mitral ring together with catheter ablation using the transseptal approach were possible. Only antegrade conduction through the AP was observed, without retrograde conduction. After 3 minutes of mapping, a good spot was found in the left lateral wall and catheter ablation was performed (Fig. ) with subsequent complete AV block due to adenosine (Fig. ). After the infusion was stopped the normal sinus rhythm resumed. Upon repeating the bolus of adenosine with complete AV block no sign of AP was detected (Fig. ). Programmed atrial and ventricular stimulation was performed without conduction through an AP.\nIn the follow-up, a catheter ablation of atrial fibrillation was also performed as the patient continued to present atrial fibrillation (AF) with narrow QRS.
A 31-year-old Brazilian woman presented to our Surgery Department of Oral and Maxillofacial Surgery for evaluation of cystic lesions in her teeth 13 and 15 and a swelling on her right maxilla (Fig. ). In her anamnesis, she reported that she had already had endodontic treatment for her teeth 13 and 15 without regression of the lesion. Her teeth 14 and 24 had been previously removed for orthodontic treatment. A radiographic and computed tomography (CT) examination showed a radiolucent lesion, diffuse, of approximately 20 mm, involving the teeth 13 and 15. The initial diagnosis was inflammatory periapical cyst (Fig. ).\nShe underwent preoperative laboratory tests and an excisional biopsy of the lesion was undertaken under general anesthesia. The choice of the use general anesthesia was due to the size of the lesion and possibility of enucleation without removal of the teeth involved. The collected material was sent for pathological examination. The result was compatible with CGCL (Figs. and ), which differed from our diagnostic hypothesis. After the definitive diagnosis, she was advised to carry out regular monitoring because of the risk of recurrence.\nShe self-monitored for a period of 10 months; she then presented again with a complaint of swelling on the site, but without the presence of pain. A new CT scan was requested and indicated recurrence of the lesion, which now involved the region’s incisor, canine, and premolar (Fig. ). New laboratory tests were carried out: alkaline phosphatase, calcium, phosphorus, and parathyroid hormone (PTH). We ruled out the possibility of the lesion being compatible with Brown tumor of hyperparathyroidism. A possible loss of the four dental elements involved was not ruled out.\nWe proposed new treatment with intralesional corticosteroid injections to our patient in an attempt to reduce the lesion for a future enucleation. She was given a drug application schedule: nine applications in the affected regions with intervals of 15 days between applications. Infiltrations were composed of: Triancil® (triamcinolone hexacetonide) + Xylestesin® (lidocaine hydrochloride; Fig. ). On average, 1.5 ml of the solution per session was applied at different points. After 5 months, there was a reduction in the lesion. New imaging tests showed that her tooth 16 had external root reabsorption and we decided to carry out an extraction of this tooth during the enucleation of the remaining lesion. We also found the formation of a septum in the damaged area, indicating bone formation (Figs. and ).\nThe enucleation of the remaining lesion was performed under general anesthesia. During surgery we removed tooth 16 as planned. Tooth 15 had no support because the buccal bone plate had been compromised, so we decided to extract it during surgery (Fig. ). A radiograph was performed 7 days postoperatively (Fig. ).\nAt 6 months postoperatively, she is on a quarterly follow-up, without recurrence of the lesion (Figs. and ). A bone reconstruction of the region is planned for 1 year postoperatively.
A 25-year-old man presented to emergency department with involuntary sustained upward deviation of eyes for one day. According to the patient, he had been using marijuana almost once in a week for the last 5 years but this time he wanted to try a different drug. One day ago, he smoked angel dust with tobacco and also snorted it a little. This was the first time he was using PCP and as per the patient he used very small quantity. After that he felt dizzy and slept whole night. When patient woke up in the morning, he had both of his eyes involuntary deviated in upward direction. His girlfriend immediately brought him to ED for further evaluation. The patient denied any fever, headache, light headedness, slurred speech, weakness, diplopia, and auditory or visual hallucinations. Patient did not have any major medical illness other than marijuana use. He denied any family history of seizures, stroke, or cancer. Patient was not on any medication and also denied any accidental use of antiemetics or antipsychotics recently.\nHe was hemodynamically stable at the time of his admission. On neurological examination, he was well oriented in time, place, and space. Pupils were equal and reactive to light. Sustained conjugate upward deviation of eyes was noted. The patient was able to bring his eyes back to normal position with forceful effort but eyes used to deviate back to upward position within few seconds. Visual field, visual acuity, and ocular movements testing could not be done due to his upward deviation of eyes. Intraocular pressure was normal and fundoscopy showed normal retina and fundus. Cranial nerve functions were intact, power was 5/5 in all extremities, and there was no sensory loss. The rest of the physical examination was unremarkable. All laboratory data including complete blood count, serum electrolytes, and renal and liver function tests were within normal limits. Urine drug screen was done which came back positive for phencyclidine (PCP). Based on the onset of oculogyric crisis after taking PCP and positive urine drug screen, the diagnosis of PCP induced oculogyric crisis was made. Patient was seen by a neurologist for the evaluation of oculogyric crisis who recommended against any neurological imaging as the case was typical of dystonia and the patient did not have any headache, neck stiffness, seizure, or focal neurological deficits suggestive of intracranial pathology. Decision was made to give IV Benadryl and check response first.\nPatient was given 50 mg of diphenhydramine (Benadryl) intravenously once. After 30 minutes, patient's eyes reverted back to normal position. Repeat neurological examination showed equal and reactive pupil. Eye movements were normal in all directions with normal visual acuity and no visual field defect. There was no motor or sensory deficit and his gait was normal. Patient was discharged on 25 mg of Benadryl TID for the next 2 days and also counselled about cessation of illicit drugs. Patient did not have any acute dystonia on follow-up.
A 27-year-old previously healthy man presented with one-month history of left thigh pain which was worse at night. There was deep tenderness at his upper left thigh but no swelling or skin changes. Computed tomography (CT) revealed a small calcified nidus surrounded by a radiolucent rim within the thickened anterior cortex in the shaft of the left femur, with periosteal reaction (). The patient underwent CT-guided percutaneous RFA with a presumptive diagnosis of osteoid osteoma. He was symptom-free after the procedure. The histopathological examination found scanty fibrous tissue and bone with infiltration by inflammatory cells. However, a culture of the specimen was not done.\nThree weeks later, he developed progressive left thigh pain with erythema and induration over the quadriceps despite anti-inflammatory medications and physiotherapy. Radiographs demonstrated periosteal reaction over the site of previous RFA (), which raised the clinical suspicion of osteomyelitis. MRI of his left thigh demonstrated thickened anterior cortex with a cloaca and inflammatory changes in the adjacent marrow and skeletal muscles. There were several well-circumscribed, enhancing mass-like lesions beneath the deep muscles adjacent to the site of previous RFA (). A diagnosis of post-RFA osteomyelitis was made, with a differential diagnosis of intramuscular soft tissue tumour.\nHe underwent an open biopsy and surgical irrigation. Pockets of blood-stained soft tissue were discovered along the femoral shaft. These corresponded to the intramuscular masses seen on MRI and were proven to be granulation tissue on histopathological examination. There was inflammation of the adjacent muscles but no abscess collection. His symptoms improved following the surgical irrigation and oral Cloxacillin therapy. Over the subsequent months, he developed several episodes of recurrent left thigh swelling with purulent sinus discharge and was treated with multiple courses of oral antibiotics. His symptoms eventually resolved after a second surgical irrigation and an extended course of Cefoperazone.\nFollow-up MRI performed four months after the second surgery demonstrated a small area of low T1signal, high T2 and high STIR signal within the marrow cavity, which was enhanced post-gadolinium, and was consistent with residual marrow oedema (). There was complete disappearance of the mass lesions and deep muscle enhancement which were demonstrated in the earlier MRI. The patient has remained well eight months after the second surgery.
This case report describes a 54-year-old male who underwent a laparoscopic sleeve resection procedure for morbid obesity. Prior to the procedure, the patient had a weight of 115.9 kilograms and a body mass index (BMI) of 49.77 and had failed both diet and medical weight management. His other medical history included hypertension, dyslipidemia, and sigmoid diverticulosis. His surgical history comprised of a laparoscopic cholecystectomy and an open reduction internal fixation of fracture on his ankle. He has a family history of diabetes mellitus type 2 in both parents. The patient admitted to drinking alcohol weekly but denied tobacco and recreational drug use. Physical examination did not reveal lymphadenopathy or hepatosplenomegaly. Preoperative evaluation of the peripheral blood was within normal limits with no evidence of a hematological malignancy or lymphoproliferative process. In addition, the patient did not have any positive findings on review of systems or any symptoms. Preoperative gastroduodenoscopy did not reveal any lesions; however, two antral biopsies were taken which revealed mild chronic inactive gastritis. A “sleeve gastrectomy specimen” was received in the pathology department. On gross examination, the gastric mucosa was described as tan-pink, smooth, and glistening with a focal area of hemorrhage (). There were no masses, no nodularities, no ulceration, or abnormal rugal folds. Representative sections of the hemorrhagic region were taken, along with several routine random areas of normal-appearing mucosa. Other than the focal area of hemorrhage, there was little evidence to indicate an underlying pathological process or malignancy, as small hemorrhagic areas are common findings in surgical specimens. On histological examination, sections taken from the hemorrhagic areas showed a diffuse atypical lymphoid infiltrate extending into the lamina propria and submucosa, with nodular pattern and focal germinal centers (). Proliferation of plasma cells was also evident. In addition, there was an associated architectural distortion with gastric glandular atrophy (). Immunohistochemical stains with the appropriate controls were obtained revealing diffuse sheets of lymphoma cells that were positive for CD20, CD43, and BCL-2. They were negative for CD3, CD5, CD23, CD138, BCL-6, and Cyclin D1. The plasma cells were monoclonal with kappa restriction. The proliferation index by Ki-67 was low, approximately 1–5%, except for the reactive germinal centers. Helicobacter (H.) pylori stain on three of the blocks was negative. With these histological findings, the patient was diagnosed with a MALToma. Positron emission tomography (PET) scan was performed which revealed a lesion in the sigmoid colon. Subsequently, the patient underwent another round of biopsies which showed mild chronic inactive gastritis within the stomach and benign lymphoid aggregates in the colon. The patient was placed on omeprazole, clairthromycin, and amoxicillin. And, six-month follow-up established endoscopic and histologic remission.
A 65-year-old man referred to the emergency department for evaluation of the lower extremity swelling associated with pain for four days. Physical examination demonstrated an overweight man (BMI 29.6 kg/m2) with extensive pitting edema of the left lower limb from the groin to the knee joint with calf tenderness. Color Doppler ultrasound revealed an extensive DVT involving common iliac, external iliac and common femoral vein as well as superficial femoral down to popliteal vein. He was treated by bed rest, elevation with bandaging of left leg, 6000 IU of low molecular weight heparin subcutaneously twice a day and further evaluation was performed to find the underlying etiology. Investigations including hematological, immunological, biochemical, lipid profile, protein S and protein C were normal. Abdominal and pelvic ultrasound (US) showed incidental finding of severe left hydroureteronephrosis with almost lost of cortical thickness, for that abdominal and pelvic computed tomography (CT) scan revealed marked left-sided hydroureteronephrosis and an impacting stone measuring (18 × 10 × 10 mm) at the level of L5/S1 () with signs of DVT affecting left iliac and femoral vein below the above mentioned region (). Next day percutaneous nephrostomy was performed to decompress the hydronephrotic kidney. He was kept as an inpatient for one week under observation then after discharged home on oral anticoagulation in the form of rivaroxaban 20 mg daily. Six weeks later, color Doppler US showed complete recanalization of the superficial femoral, popliteal as well as the proximal segment of deep veins of the leg but common iliac, external iliac and common femoral veins and proximal superficial femoral vein were still partially thrombosed. Under spinal anesthesia, left ureterorenoscopy showed an impacted stone at the level of iliac vessel pulsation causing edema and external compression of the iliac vessels. Through pneumatic lithotripsy, the stone was fragmented and JJ stent inserted (). Next day the patient discharged home and continued on taking his antithrombotic treatment (rivaroxaban 20 mg). The JJ stent was removed 3 weeks later. Three months after that, Doppler US showed complete recanalization of iliac vessels.
A 74-year-old healthy Korean woman presented with a history of intermittent epigastric pain. Her husband and son were known to have developed gastric cancer. The patient underwent endoscopic examination of the stomach in December 2005, which demonstrated a depressed mucosal lesion in the anterior wall of the distal gastric body (). Histological examination revealed signet ring cell carcinoma. Abdominal computed tomography (CT) excluded gastric wall thickening, lymphadenopathy and distant metastasis. The diagnosis was type IIb early gastric cancer for which surgery was recommended, but she declined for an unspecified reason.\nThe patient took antacids and digestive medicines irregularly, and underwent a repeat endoscopy in September 2006, which showed no interval changes. However, at the third endoscopy in October 2009, a satellite lesion was detected in the greater curvature of the distal gastric body adjacent to the previously confirmed malignant lesion. Histological examination of the satellite lesion confirmed signet ring cell carcinoma of the stomach, identical to the primary lesion. A CT scan showed no interval changes and the patient continued to decline surgery. In March, 2010, while no interval changes were shown on repeat endoscopy, she finally agreed to undergo surgery.\nThe pre-operative physical examination was unremarkable. The levels of carcinoembryonic antigen and carbohydrate antigen 19-9 were normal. All routine laboratory test results were within normal limits.\nShe underwent open curative distal subtotal gastrectomy, stapled Billroth I gastroduodenostomy and D2 extended lymphadenectomy in May 2010. Two type IIb early gastric cancer lesions were located in the anterior wall and greater curvature of the distal gastric body without associated metastatic lesions in the resected lymph nodes ().\nPostoperative histological examination confirmed invasion of signet ring cell carcinoma into the gastric submucosal layer (). The primary lesion measured 3.0×4.5 cm on the anterior wall of the distal gastric body, and the satellite lesion measured 2.0× 2.5 cm on the greater curvature of the distal gastric body. The two lesions were pathologically not connected. There was no evidence of metastasis in the 40 lymph nodes resected. The final diagnosis was stage Ia signet ring cell carcinoma of the stomach, based on the seventh edition of Union for International Cancer Control classification.
A 65-year-old man referred to the emergency department for evaluation of the lower extremity swelling associated with pain for four days. Physical examination demonstrated an overweight man (BMI 29.6 kg/m2) with extensive pitting edema of the left lower limb from the groin to the knee joint with calf tenderness. Color Doppler ultrasound revealed an extensive DVT involving common iliac, external iliac and common femoral vein as well as superficial femoral down to popliteal vein. He was treated by bed rest, elevation with bandaging of left leg, 6000 IU of low molecular weight heparin subcutaneously twice a day and further evaluation was performed to find the underlying etiology. Investigations including hematological, immunological, biochemical, lipid profile, protein S and protein C were normal. Abdominal and pelvic ultrasound (US) showed incidental finding of severe left hydroureteronephrosis with almost lost of cortical thickness, for that abdominal and pelvic computed tomography (CT) scan revealed marked left-sided hydroureteronephrosis and an impacting stone measuring (18 × 10 × 10 mm) at the level of L5/S1 () with signs of DVT affecting left iliac and femoral vein below the above mentioned region (). Next day percutaneous nephrostomy was performed to decompress the hydronephrotic kidney. He was kept as an inpatient for one week under observation then after discharged home on oral anticoagulation in the form of rivaroxaban 20 mg daily. Six weeks later, color Doppler US showed complete recanalization of the superficial femoral, popliteal as well as the proximal segment of deep veins of the leg but common iliac, external iliac and common femoral veins and proximal superficial femoral vein were still partially thrombosed. Under spinal anesthesia, left ureterorenoscopy showed an impacted stone at the level of iliac vessel pulsation causing edema and external compression of the iliac vessels. Through pneumatic lithotripsy, the stone was fragmented and JJ stent inserted (). Next day the patient discharged home and continued on taking his antithrombotic treatment (rivaroxaban 20 mg). The JJ stent was removed 3 weeks later. Three months after that, Doppler US showed complete recanalization of iliac vessels.
The propositus is a 3-year-old right-hand dominant girl who presented to a multidisciplinary neurodevelopment clinic with a question of developmental delay and hypotonia. Early spotting in the third week complicated the pregnancy however, no subsequent difficulties occurred. She was born at 41 weeks of gestation by vaginal vertex delivery. Apgar scores were 8 at 1 min and 9 at 5 min. The birth weight was 3935 grams. She had a stable neonatal course and was breastfed for the first 7 months of life. She presented with early emesis but with no history of reflux or aspiration. She did have a history of chronic respiratory congestion since early infancy and was subsequently diagnosed with asthma; which was treated with Flovent and Ventolin. She had a history of recurrent middle-ear infections requiring myringotomy tubes. Serial neuro-opthalmologic assessments were performed for mild intermittent exotropia, which had been improving. An MRI of her brain at 1 year of age showed normal brain structures with no evidence of agenesis of the corpus callosum.\nEarly in her second year of life, her parents were concerned when her walking milestone was delayed. She walked at the age of 23 months, but continued to have difficulties with balance and coordination and was quite a cautious child. Her first words were around the age of 8 months. Slow progress was made and by her assessment at 31 months of age she had approximately 30 single words and was not combining words into phrases. She had difficulties with pronunciation and remained unintelligible to unfamiliar people. In her fine motor skills, she was found to be severely delayed in both manipulation and skills required for self-care. She was not toilet trained. A decrease in pain sensation was also reported. She was very social, had a happy temperament and enjoyed a variety of play activities although at a younger developmental level than her chronological age. There was no regression and no signs of autism.\nA formal assessment of her motor development using the Peabody Developmental Motor Scale (PDMS-2) at the age of 31 months indicated that she had a severe delay in fine and gross motor skills. On the Fine Motor Scales Subset, she scored less than the first percentile with overall skills equivalent to those of a 17-month-old in visual motor tasks and that of an 11-month-old in hand grasping skills. In the Gross Motor Scales Subset, she was at the second percentile and this gave her an age equivalent of 16 to 17 months. Her speech and language development was assessed using the Preschool Language Scales 4 (PLS-4) and was found to be at the first percentile for both auditory comprehension and expressed communications. Her overall cognitive abilities were assessed using the Bailey Scales of Infant and Toddler Development 3rd Edition Cognitive Scales, where she was found to be in the extremely low range at approximately the 17-month-old level, suggesting that she would learn at a slow rate and would require repeated learning opportunities to acquire new concepts.\nHer family history revealed that both parents are healthy, nonconsanguinous and have completed university degrees. The only other pregnancy was that of a 4-year-old sister with normal developmental. A paternal uncle died at 6 years of age with a history of motor developmental disability and one of the father’s cousins was diagnosed with autism.\nOn physical examination, the patient was very interactive with good eye contact. Her weight was 12.8 kg (25%), height was 93 cm (50%), and head circumference was 47.5 cm (30%), with a prominent forehead. Bilateral supranumerary nipples were present on general examination and was otherwise normal. Her skin examination revealed a small midline hemangioma over the upper back. Generally decreased tone was revealed from her neurological examination and her deep tendon reflexes were 1+ in the upper and lower extremities. She could walk independently but fell quite often during the examination. She had a wide-based gait on assessment.
We are reporting a 57-year-old Arabic female who noticed a lesion of the right side of the scalp in July 2019, it was not tender and measured approximately 1.5 cm. She underwent a partial excision at a local hospital with a preliminary diagnosis of a benign lesion. The final pathology was a malignant lesion. No official pathology report was available for review. She refused further management. The lesion progressed again rapidly within one month; she presented herself to another private hospital where more radical surgery was carried out with left selective neck dissection, this was done on October 16, 2019. The pathology report was spindle cell squamous cell carcinoma, all margins were negative and all the lymph nodes were negative. She was referred to King Fahad Medical City, Riyadh, Saudi Arabia, in November 2019 for further management. She was a fit lady, not a smoker or drinker, however, was a known diabetic on oral hypoglycemic drugs for 10 years with fair glycemic control. She was complaining of mild pain at the site of the lesion with no other associated symptoms. On examination, there were two small nodules at the margins of the previous surgical scar. The superior nodule measure 1 cm x 1.5 cm and the other was 1.5 cm x 2 cm at the inferior surgical margin (Figure ).\nShe underwent full reevaluation with magnetic resonance imaging (MRI) of the head and neck and computer tomography (CT) of the chest, abdomen, and pelvis. The MRI showed that there was evidence of residual/recurrent soft tissue tumor at the superior and inferior aspects of the surgical bed. The first lesion at the high left parietal area measured 1.2 cm x 2 cm x 2.1 cm, it demonstrated features of internal hemorrhage. The other lesion noted at the left occipital region measured about 1.2 cm x 2.3 cm x 2.5 cm. Both lesions demonstrate post-contrast enhancement. There was no evidence of underlying bony involvement and no intracranial extension was seen. A few small lymph nodes were noted at the posterior neck on the left side (Figures , ). The CT chest showed multiple lung metastases (Figure ).\nThe histopathology review has shown malignant spindle cell neoplasm, poorly differentiated/sarcomatoid squamous cell carcinoma with perineural invasion but without lymphovascular invasion (Figure ). Immunohistochemistry staining showed positive staining of vimentin, desmin, and cluster of differentiation (CD) 10. The cytokeratin staining, cytokeratin (CK)-Pan, CK5/6, P63, and CK7 were focally positive. S100, human melanoma black (HMB)-45, CD117, paired-box gene 8 (PAX-8), estrogen receptors (ER), progesterone receptors (PR), CK20, gross cystic disease fluid protein 15 (GCFP-15), CD34, CD1a, myoblast determination protein 1 (MyoD1), and myogenin were all negative. All of the above were supporting the diagnosis.\nShe was discussed in the tumor board and the decision was to give palliative radiotherapy to the local lesion and to start palliative systemic chemotherapy. She was fit with Eastern Cooperative Oncology Group (ECOG) performance status of one. After a full discussion of the possible benefits and side effects, it was decided to start her on pembrolizumab, cisplatin, and 5-fluorouracil protocol since the main histopathological component was squamous cell carcinoma. Programmed death-ligand 1 (PD-L1) testing was sent abroad since the local laboratories were still not accredited at that time. She received three cycles with good tolerance but the reevaluation CT scan showed progression of the lung metastases. At that time the PD-L1 testing came back as negative. We substituted pembrolizumab with cetuximab. Further three cycles were given but after the third cycle, she started to have shortness of breath. Chest x-ray and CT scan showed further progression with right pleural effusion. She was admitted for pleural drainage and pleurodeses. After stabilization, she was given single-agent doxorubicin. After three cycles of doxorubicin, she had symptomatic improvement with the partial radiological response, but she refused further treatment because she felt more fatigued during chemotherapy. She was kept under follow-up with palliative, symptomatic care at her regional hospital. She progressed further and died after three more months.
A 75-year-old man presented to our institution with a 40-year history of dyspeptic symptoms. Physical examination was unremarkable. Contrast-enhanced computed tomography (CECT) of the chest, abdomen, and pelvis revealed a 4 × 3-cm well-circumscribed mass localized in the head of the pancreas. This tumor showed peripheral enhancement in the early arterial phase and homogenous enhancement in the late arterial phase. The pancreatic duct was not dilated, surrounding vessels were free, and there was no evidence of any nodal or distant metastasis. Core needle biopsy of the mass revealed the presence of spindle cells without any atypia. Two years before presenting to our institution, the patient presented to another center for the same complaint, where he underwent CECT of the abdomen, which revealed a 4 × 3-cm mass in the head of the pancreas. Based on the presence of a pancreatic head mass of constant size for two years and core needle biopsy showing spindle cells, the patient was diagnosed with primary pancreatic leiomyoma and was advised to be under follow-up. He was given symptomatic treatment for dyspepsia, to which he responded well. During yearly follow-up examinations for the next eight years, which included imaging studies, the mass size did not change and the patient remained asymptomatic. The patient was then lost to follow-up.\nThirteen years after the initial diagnosis of the pancreatic mass, the patient presented with a three-month history of loss of appetite, significant weight loss, and a lump in the right upper abdomen. Physical examination revealed a 10 × 12-cm hard, nodular mass in the right hypochondrium, which did not move with respiration. Hemogram and liver function tests were normal. Abdominal ultrasound examination three months before the latest admission revealed a 13 × 10-cm solid mass in the pancreatic head. Abdominal CECT at admission revealed a 15 × 13 × 10-cm mass with heterogenous enhancement in late phase involving the pancreatic head with main portal vein encasement and dilated pancreatic duct and common bile duct (Figure ).\nImmunohistochemical evaluation revealed that the biopsy samples collected by ultrasound-guided fine-needle aspiration were positive for smooth muscle actin and desmin but negative for c-Kit, DOG-1, CD34, and S-100. The Ki-67 index was 20%. These results led to the diagnosis of primary pancreatic leiomyosarcoma (Figure ). The patient could not tolerate chemotherapy including doxorubicin and ifosfamide and received best supportive care. The patient died 11 months after the leiomyosarcoma diagnosis.
A 55-year-old Caucasian female presented to the surgical office with a chief complaint of chronic epigastric abdominal pain for seven months duration. The patient was referred to the office after an emergency department visit for her chronic abdominal pain. The workup showed normal laboratory values with a nonspecific, lobulated soft tissue lesion located within the mesentery of the upper abdomen measuring up to 3.2 cm on computed tomography with intravenous and oral contrast. The patient noted her pain had developed and had been persistent since she underwent laparoscopic appendectomy for acute appendicitis roughly one year prior. She described her pain as sharp in nature with intermittent flares. The patient reported that eating exacerbated her symptoms. She endorsed anorexia as well as weight loss. Physical examination at that time was considered unremarkable. The patient’s medical history included gastroesophageal reflux disease, gastritis, depression, and a seizure disorder. The patient’s surgical history included a cholecystectomy for cholelithiasis, hemorrhoidectomy, tonsillectomy, and an appendectomy. Family and social history were non-contributory.\nBased on the initial radiographic results and the location of the mesenteric lesion, the patient was referred for endoscopic evaluation and possible biopsy. They were then started on a trial of proton pump inhibitors. The patient had multiple visits to her primary care provider and was eventually transitioned to sucralfate as her symptoms were unresolved. The patient underwent esophagogastroduodenoscopy that revealed LA Grade B gastroesophageal reflux disease, a 2 cm hiatal hernia, and diffuse gastritis. Repeat computed tomography of the abdomen and pelvis with intravenous and oral contrast was performed revealing a lobulated lesion at the level of the kidneys within the anterior mesenteric fat, concerning for an enlarged lymph node or a neoplastic mass as demonstrated by the arrow in Figure . The mass was deemed inaccessible to endoscopic biopsy due to interference of surrounding structures and risks associated.\nThe patient returned to the clinic for a follow-up examination. After an informed conversation regarding the possible etiologies of the mass and medical and surgical options, the decision was made to further characterize the soft tissue mass in preparation for surgery. Magnetic resonance enterography was performed which redemonstrated the previously reported mid-abdominal mesenteric mass located posterior-inferior from the gastric antrum, with etiology remaining unclear. In addition, there was a continued concern for an enlarged lymph node versus an underlying neoplastic process as seen in Figures , . Interventional radiology was consulted for further recommendations regarding potential image-guided biopsy of the mesenteric lesion. Due to the inability to safely perform a biopsy of the mass, surgical intervention was again considered. A discussion was held with the patient highlighting the persistent history of abdominal pain in the setting of an unknown mass. Pre-intervention informed consent centered on surgical approach, risks, and benefits of surgery. In reflection of the size, location of the mass, and maximization of surgical yield, the decision was made to attempt a diagnostic laparoscopy with possible wide local excision, possible laparotomy.\nThe patient was consented for and underwent diagnostic laparoscopic exploration. Upon entering the abdomen, the omentum, mesentery, and peritoneum were without signs of gross disease. Takedown of the gastrocolic ligament and omentum was performed and upon entering the lesser sac, there was a firm, mobile mass located inferior to the greater curvature of the stomach. Careful dissection was attempted via laparoscopic approach, but due to the dense nature of the mesentery and inability to properly characterize the location of the mass about the surrounding structures, the decision was made to convert to an open procedure. The mass was found to lay within the mesentery and was free of attachments to the underlying vasculature and pancreas. Careful sharp excision was performed to circumferentially inscribe the mass as demonstrated in Figure . After meticulous dissection of the mass, it was safely removed in one piece as demonstrated in Figure . The specimen was then sent to the department of pathology to further characterize the lesion. Due to the lack of gross neoplastic or desmoplastic reaction surrounding the mass, the decision was made to close primarily without further dissection.\nThe postoperative period was uneventful and the patient remained stable throughout its entirety. The patient was discharged without complication on postoperative day two. The patient was seen in the office for follow-up at one week and at one-month post-operatively in which she endorsed the slow resolution of her previous symptoms. The authors can not say with certainty that her symptoms improved secondary to surgical intervention.\nHistological evaluation of the surgical specimens revealed adipose tissue with a nodular collection of inflammatory cells composed predominantly of histiocytes with interspersed lymphocytes. The histiocytes contained foamy cytoplasm and surrounding areas of confluent fat necrosis with associated cholesterol clefts. Granular basophilic particles representing dystrophic calcification were dispersed throughout the areas of fat necrosis. Examination by polarized light microscopy did not show foreign material. Special stains for infectious organisms were negative. Histological examination of the surgical specimen is seen in Figures , , . The pathological evaluation concluded the lesion in question was mesenteric fat necrosis without any evidence of atypia or malignancy.
We report a case of a 34-year-old male presented to emergency department with acute onset of irritability and aggressive behavior. He was admitted to the psychiatric ward and was observed to have fluctuations in his symptoms. The patient reported that he ingested some glasses and plastic particles when he went mad which caused abdominal pain. Surgical opinion and abdominal ultrasound revealed no evidence of foreign body or any other abnormal finding. He reported a past episode of psychiatric symptoms in 1994 which lasted for 3 weeks and required hospital admission, but the patient was unable to recall details of his past symptoms. During his current hospitalization his mood was observed to be labile and he described his symptoms as if it was “a dream state or a movie.” The mental state examination revealed auditory hallucinations and paranoid delusions. He scored 19 on the mini-mental state exam (MMSE). His medical records over the past 2 years indicated visits to the dermatology, ophthalmology, and dentistry clinics. He was diagnosed with chronic gingivitis, pulpitis, and caries into pulp in dentistry clinic. In 2010, the patient presented to ophthalmology clinic with a pigmented lesion in the left lower eyelid which was present since childhood. One year prior to visiting ophthalmology clinic, the patient noticed having additional lesion adjacent to it. No itching over the lesion was present and the patient denied excessive tearing or trauma. He was noticed to have ulceration and occasionally oozing from the lesion. Excision biopsy of the lesions were performed which revealed basal cell carcinoma. His visits to dermatology clinics indicated previous presentations with pitted keratosis involving hands, callosities, and seborrheic dermatitis. On examination, there were numerous palmar pits, red-coloured and measuring about 1-2 mm in diameter (). Brain CT scan revealed extensive calcification along falx cerbri and around the cerebellar vermis and few tiny calcifications in the cerebellum bilaterally and in the high parietal regions at the vortex (Figures and ). He had low (20 ng/L) vitamin D level (normal range between 75 and 250 ng/L). Parathyroid hormone level was high at 114.3 ng/L (normal range is 12–88 ng/L). He had high calcium level (2.65 mmol/L), normal phosphorus (0.96 mmol/L), and normal magnesium level. His Chest X-ray was normal, while an X-ray report of cervical spine, done in 2010, revealed loss of cervical lordosis and sclerosis of the endplates with anterior calcification at the level of C5-C6. There was also a mild kyphosis at the level of C4-C5. Posterior facets were reported to have shown sign of degeneration.
A 52-year-old man was admitted to the head and neck surgery department for a left cervical adenopathy. No primary cancer site had been identified from endoscopic and imaging examinations. After three sessions of chemotherapy, both the scanner (figure ) and MRI (figure ) revealed an adenopathy that measured slightly more than 60 mm and exhibited invasion into the common and internal carotid artery. Angiographic examinations demonstrated a complete circle of Willis. We decided to carry out en bloc resection of the carotid artery and reconstruction with superficial femoral artery transplantation (figures , , , , and ). Clamping of the carotid artery lasted 25 minutes. Electroencephalogram (EEG) recordings during the operation did not elicit any cerebral pain. Post-operative care was uneventful, and the patient did not show any sign of neurological deficiency. The pathology analysis of the tumor confirmed that the carotid adventitia was invaded (figures and ). The patient benefited from cervical irradiation with a total administered dose of 70 grays. After three years of follow-up, the patient survived without any cancer recurrence, which was confirmed by 18 FDG-PET/CT (figures and ).\nEn bloc resection of the carotid artery allows for better regional control of the cancer compared to other procedures, such as radio chemotherapy or carotid artery dissection only. [] Nevertheless, the global survival rates at 5 years are identical for carotid artery dissection and en bloc resection. []\nWhen massive cancer invasion of the carotid artery is present (for instance, when the tumor invades the vessel over the adventitia), carotid artery dissection cannot be performed. Thus, in such cases, en bloc resection of the artery is necessary for proper surgical management of the cancer. Several imaging signs are useful for predicting a massive invasion of cancer into the carotid artery. These include deformation of the artery (which becomes oval), encasement of more than 180 degrees, and segmental obliteration of the fat between the adenopathy and the carotid artery. [,]\nThe main risk associated with an en bloc resection of the carotid artery is cerebral stroke. If the carotid artery is removed but not reconstructed, the risk for developing a stroke is almost 30%. However, proper reconstruction of the carotid artery reduces this risk to less than 3%.[]\nArteriography, angio-TDM, or MRI allows one to verify the permeability of the Circle of Willis, and therefore minimizes neurological risk when performing arterial clamping. Arteriography is the gold standard and has the advantage of allowing one to carry out a clamping test.\nOperative surveillance was conducted with EEG. However, similar to the use of evoked potentials, the efficiency of EEG in predicting neurological risk has not been fully demonstrated. Nevertheless, several operative assessments should be performed in order to prevent a stroke: maintenance of a stable median arterial pressure greater than 10 mmHg, achievement of a very short clamping time (less than 30 minutes if possible), and initiation of anti-coagulation (injection of 100 UI/kg of heparin) one minute before clamping. HHHeparin injection causes a more difficult tumor removal, and for this reason carotid reconstruction was performed last in our operative protocol.\nThe superficial femoral artery was used for the transplantation. The femoral artery has the advantage of having a comparable caliber to the common carotid artery, permitting good congruence in an arterial anastomosis. As a living tissue, the femoral artery is also resistant to infection following postoperative radiotherapy. In contrast, the absence of stenosis at the arterial level should be controlled by at least a Doppler echo. Moreover, transplantation of the femoral artery necessitates reconstruction of the section removed, which was replaced with Dacron prosthesis.\nCertain authors use Dacron or ePTFE (expanded polytetrafluoretylene) prosthetics to carry out the carotid anastomosis. []
A 68-year old male patient visited his family doctor complained for atypical upper abdominal pain. After obtaining informed consent from the patient, we began with a typical clinical examination. This examination was based on medical history and any probable symptom and signs that were presented by the patient. No typical clinical finding was observed so the physician proposed further investigation with the ultrasound (US) of the upper abdomen. The US performed revealed a mass in the retroperitoneal space, possibly pertaining to the left adrenal gland. This mass was described with clear boundaries, dotted calcification and mild central tumor vasculature. This aspect made the differential diagnosis more complicated, spanning from adrenal adenoma to pheochromocytoma. The size of the tumor was approximately 5cm as measured by US. For this reason imaging procedure included CT scan for further investigation of this mass. CT scan confirmed the presence of the mass and described a tumor in the left adrenal gland, with mild inhomogeneous solid characteristics and an oval size of approximately 42x38x32mm (Fig.). The tumor had also low electron levels on the Hounsfiled scale (about 15 ΗU) and the CT showed a small dot calcification over the tumor. The washout percentage of the tumor was measured at a minute and 15 minutes later after intravenous administration of a radiocontrast agent. The washout percentage did not help the differential diagnosis of this adrenal incindentaloma because there observed further intake of the radiocontrast agent in the late CT images of the tumor (about 40-45 ΗU 15 minute later). Retroperitoneal lymph node involvement or other metastases was not detected.\nHowever the imaging criteria weren’t sufficient for the investigation of the AI. Naturally the hormonal activity of the tumor had to be investigated. The patient was referred to the department of endocrinology of the University hospital of AHEPA Thessaloniki to complete the examinations required. These included a 24hr urinary free cortisol and 24hr urinary metaneprine levels as well as catecholamines levels. Plasma aldosterone and renine levels were not required because the patient did not present with medical history of hypertention. Both biochemical examinations showed that hormone levels were within normal parameters.\nVMA was 2.67mgr/24h (2-7mg/24 hours) and cortisol was 28.5μgr/24h (3.5-45µg/24h). In conclusion and taking into account the above measured parameters the AI seemed to be a non-secretive adrenal tumor. Only the increased size of the tumor (42mm>40mm) was an inclusion criteria for the surgical excision of this AI. The non diagnostic washout percentage gave us a little evidence about the malignancy of the tumor. The patient underwent to laparoscopic excision of the tumor for both therapeutic and diagnostic purposes. The surgical specimen had an oval shape and its size was in its longest axis was 4,8cm. The tumor was solid, with no hemorrhage and necrotic signs with its cut surface showing a white color as described in literature (Fig.,Fig.). The histological examination showed many Schwann cells inside fat tissue as well as gangliocytes. Immunohistochemically Schwann cells were at S100 protein positive [S100(+)] and gangliocytes NSE (+) (Fig.,Fig.,Fig., Fig.). The postoperative period was free of complications. Six months later a scheduled follow up examination was performed, with a new CT scan. There was no evidence of recurrence of the tumor in the retroperitoneum.
A 72 year old lady presented to us as an emergency with abdominal pain, intermittent vomiting and worsening constipation of a few days duration. She also reported a significant weight loss over past few months. Her relevant past history included rheumatoid arthritis and pT1 N0 M0 carcinoma of the right breast, 3 years ago, for which she underwent wide local excision and axillary node sampling followed by adjuvant radiotherapy. She was also on Arimidex as hormonal therapy. Her general examination was unremarkable and the abdominal examination revealed a distended abdomen with a suggestion of fullness in the right iliac fossa.\nA computed tomography (CT) scan of the abdomen showed a caecal mass causing intestinal obstruction (figure ). The patient underwent an emergency right hemicolectomy and made a satisfactory postoperative recovery.\nAt 13 months follow up she had no signs of recurrence of tumour. CT Scan of her chest and abdomen did not show any visceral metastasis. A Magnetic Resonance Imaging Scan and Bone Scan with intravenous MBq Tc 99m-HDP with imaging at 3 hours ruled out bony metastasis. Carcinoembryonic Antigen (CEA) and Cancer Antigen 15-3 (CA153) levels done 6 monthly in the follow up period were within normal limits.\nThe histology of the wide local excision and axillary sampling specimen had revealed a grade 1 infiltrating ductal carcinoma (Figure and ) with no lymphovascular invasion. The tumour was 11 mm in maximum diameter and the closest radial margin was 6 mm inferiorly. None of the thirteen lymph nodes recovered showed any evidence of metastasis. It was positive for both Estrogen and Progesterone receptors. Expression of HER 2 protein was negative. There was only focal ductal carcinoma in situ (DCIS) seen within the tumour.\nOn histopathological examination of the right hemicolectomy specimen, an ulcerated tumour was identified in the caecum. Multiple sections from the caecum showed an adenocarcinoma with tumour cells in nests and groups with focal cribriform pattern. The tumour extended into the mucosa, muscle and the subserosa. No transformation to malignant epithelium was identified in multiple sections (figure and ). Proximal and distal resection margins were tumour free. Immunohistochemistry showed positive staining with Cytokeratin (CK) 7 (figure ) and CEA (figure ) whereas staining with CK20 (figure ), CDX2 (figure ) and Estrogen receptor(ER) were negative. Progesterone receptor (PR) showed equivocal nuclear staining. Eight out of eleven mesenteric nodes showed tumour deposits. A histological diagnosis of metastatic breast carcinoma was made in light of the histological pattern of the tumour, previous history of breast cancer, positive immunostaining with CK7 and CEA and negative with CK20 and CDX2.
A 14-year-old male had an extensive flame burn in the right side of neck, face, and scalp, anterior chest, and right upper limb at the age of 2 years.\nNo past medical or surgical history. No known drug history, no family history of any genetic disorder, patient and both parents are non-smokers.\nHe received multiple skin grafting procedures and an amputation of the right hand. The burn to his scalp was treated with a burr hole to the outer cortex to allow granulation tissue to cover the scalp, and then the wound was skin grafted.\nThe patient chronically continued to have unstable skin over the scalp and kept developing recurrent ulceration. On 29 March 2012, the patient was admitted throw the clinic due to his chronic condition of recurrent open wounds for many years.\nA plan was set for the removal of the unstable ulcer and scarred skin of the scalp and coverage with an omental free flap and split thickness skin graft ().\nUnder general anesthesia with endotracheal intubation, the patient was in supine position, with draping of the head circumflex, face, abdomen, and left thigh down to the knee.\nThe General Surgery team was consulted for laparoscopically harvesting the omental flap and was performed by Dr I.Anwar based on the left gastroepiploic artery. The right superficial temporal artery was identified to be the recipient vessel ().\nThe ENT team did a right neck exploration to explore the neck vessels as a backup in case the right superficial temporal artery was not suitable.\nThe free omental flap based on the left gastroepiploic artery was anastomosed to the right superficial temporal artery. The micro procedure of omental free flap was performed by DR F.Hashem, Plastic surgeon.\nThe patient was given heparin 2000 U intraoperatively. The flap was viable with bleeding with a positive Doppler signal.\nAfter establishing flap revascularization, all the excising unstable scarred and ulcerated skin covering the scalp was resected, and the underlying irregular skull was smoothed down using a flat burr. The omental flap then covered the scalp, and a split-thickness skin graft from the thigh was placed over the omental flap ().\nThe patient was monitored postoperatively in intensive care unit. Post-operative care for microsurgery and wound care for skin graft over omental flap.\nThe hospital course was uneventful. The patient was kept on IV anticoagulants (dextran and heparin) for 5 days.\nThe patient tolerated the procedure well. No wound complications were reported during his two years follow-up (). Re-Exploration nor revision of the surgery were needed
An 83-year-old female with bilateral primary TKA performed 17 years prior presented to the clinic. The patient was referred with worsening left knee pain, reported gait instability, and swelling for three months duration. Until this point, she had been completely asymptomatic. She was initially seen and treated by an orthopaedic surgeon from an outside facility with physical therapy, followed by a left knee arthrocentesis to rule out infection. The aspirate demonstrated proteinaceous fluid with few benign inflammatory and epithelial cells and cultures were found to be negative. Due to the increasing pain, gait instability, and discomfort, coupled with lack of relief by the current measures, she was referred to the orthopaedic surgery clinic at our institution for further evaluation.\nAt her initial visit, the patient reported steadily increasing, sharp pain localized to her left knee joint with associated swelling that worsened with ambulation and prolonged standing and lacked improvement with conservative management. Her day-to-day activities were becoming restricted secondary to the pain and she reported occasional falls due to the perceived instability of her knee joint. Physical exam revealed a mild antalgic gait and tenderness over her proximal tibia. An in-house X-ray was notable for an increase in size and number of osteochondral bodies in the left suprapatellar recess with a left joint effusion and “lysis and subsidence of the tibial component and decreased thickness, suggestive of loosening and wear” (Figures -). The patient then underwent a bilateral knee bone scan which confirmed the tentative diagnosis of implant loosening with polyethylene wear and instability. The patient was counseled on her treatment options, including surgical and non-surgical management, and elected to undergo revision surgery of her left knee arthroplasty.\nA classical anterior approach to the knee was made through the patient’s previous scar. A medial parapatellar arthrotomy was performed, after which the knee joint was exposed, revealing extensive osteophytes around the patella (Figure ). The osteophytes were removed and a medial release was performed, allowing for removal of the previous components (Figure ). The femoral component was found to have bone ingrowth, which had encased the patella (Figure ). Visualization of the bone-cement interface intra-operatively proved difficult. Upon gross visual inspection, it appeared that local long-term reaction at bone-cement interface had engulfed the cement and resulted in direct ingrowth of bone to implant. Bone-implant interface tissue was taken for histology examination. Microscopically, the sections examined showed papillary synovial proliferation which is consistent with the patient’s history of long-standing osteoarthritis (Figure ). Multiple foreign body giant cells, which are formed by fused macrophages, are seen in response to polarizable foreign material (Figure ). Orthopaedic implants can cause chronic inflammation and giant cell foreign body response as seen in this case (Figure ).\nAfter component removal, joint preparation was done in standard fashion by membranous tissue removal and minimal freshening of previous bone cuts. Then revision of knee components was performed as per standard technique.\nPostoperative X-rays confirmed excellent placement of a left knee arthroplasty (Figures -). The patient experienced no postoperative complications and was discharged from the hospital on postoperative day 2. She then followed up in the clinic two weeks postoperatively. The patient stated her pain was well controlled and had been working well with physical therapy. X-rays performed at this time reported that the left knee arthroplasty was in expected position with no evidence of hardware failure or loosening (Figures -). She reported that she was pleased with her new prosthesis.
A 32-year-old male reported with a chief complaint of unesthetic appearance of the face due to depression in the lip region on the left side of the face. Extra orally, two scars and a prominent depression in lower lip region were noticeable on the left side of the face. On intraoral examination, mandibular anterior and premolar teeth were missing and a bony defect (12 mm × 19 mm) with flabby tissue at the base was present at the respective region []. The complete case history of the patient was taken, which revealed that the patient had undergone treatment for comminuted mandibular fracture in symphseal-parasymphyseal region on the left side of face 3 years back. This segment of the mandible along with five teeth (mandibular anteriors and premolars) was removed during open reduction of the fracture followed by placement of titanium reconstruction plate. The intraoral closure of the defect was done by placing the temporal flap over the defect. The relative positions of the two mandibular segments and the reconstruction plate could be clearly appreciated on orthopantogram []. The tissues in the defect region were 12 mm below the cervical margins of the adjacent teeth, attributing it as a Siebert's Class III defect []. The various treatment modalities were thoroughly explained to the patient. Considering the reluctance for the surgical procedure and economic status of the patient a modification of Andrews bridge was chosen as the treatment option. The modification planned was to replace the costlier prefabricated bar and sleeve attachment of conventional Andrews system with two small disc shaped magnets. The advantages and disadvantages were clearly explained to the patient and informed consent was taken. The step by step procedure is as follows:\nDiagnostic impressions were made and casts were prepared The selected abutment teeth (41, 36) were prepared for metal ceramic restorations. The gingival retraction was done using gingival retraction cord (Ultrapak) and putty wash impression (Express STD Putty 3M, ESPE, USA and Express Light body STD Quick Wash, 3M ESPE, USA) was made. Provisional crowns (Protemp – II, 3M ESPE, USA) were cemented on both the prepared teeth The maxillary and mandibular casts were mounted, and the wax pattern was fabricated in Blue Inlay Wax (Bego, USA) comprising of a rectangular bar connecting copings on both the prepared teeth. The pattern was casted in Nickel chrome alloy (Wiron 99, Bego, USA) The cast metal framework was tried in patient and checked for any impingement of the basal tissues The framework was again tried in after application of Porcelain (Ceramco-3, Dentsply, USA). Afterwards, the framework was reseated on the cast and a self-polymerizing clear acrylic (DPI, India) flange was fabricated from the bar in the framework till the soft tissues on the base of the defect. The tissue surface of the flange simulated the sanitary pontic design []. A magnet (NdFeB magnet, Ni-plated–disc shaped magnet 2 mm × 1.5 mm in size, Techtone Electronics, Mumbai, India) was placed in buccolingual and mesiodistal center of the lingual surface of this flange [] The framework was cemented in vivo with temporary cement (Rely-X Temp, 3M ESPE, USA). An impression of the mandibular arch after cementation of the framework was made using irreversible hydrocolloid (Algitex, DPI, India). The cast was poured (Kalstone, Kalhabhai, India) and undercuts were blocked. The cast thus obtained was used for the fabrication of removable component of the prosthesis Mounting of the casts was followed by teeth arrangement and esthetic try in Acrylization of prosthesis was done with heat cure acrylic resin (DPI, India) The undercuts in the prosthesis were removed before insertion. A counter magnet was placed on intaglio surface of lingual flange of the removable part of the prosthesis corresponding to the magnet on fixed part of the prosthesis []. The framework was cemented permanently, and the removable component was placed [] The technique of insertion and removal of the prosthesis was taught to the patient. Oral hygiene Instructions and recall schedule were explained to the patient.
A healthy thirteen-year-old male, who is a competitive basketball and baseball player, sustained a closed, completely displaced fracture involving the medial clavicle after falling off an all-terrain vehicle. He was initially seen in the emergency department, placed in a sling, and referred to the orthopedic service for definitive management. The patient is right-hand dominant and does not smoke. He has no prior history of injury to the right clavicle. At the time of his orthopedic consultation, his pain was described as moderate and sharp localized to the medial end of the right clavicle. The patient reported no numbness or tingling in his arm.\nPhysical examination revealed a healthy-appearing, pleasant male responding appropriately and in no apparent distress. The pertinent findings on examination included no signs of cervical radiculopathy, no pain or winging of the scapula, intact skin, but significant tenting and swelling over the medial right clavicle, and obvious asymmetry of the right clavicle compared to the left clavicle consistent with a displaced fracture. The end of the clavicle lateral to the fracture site was displaced anterior to the medial component. The remainder of the physical examination revealed no additional injury to the upper extremity. Motor function and sensation were intact throughout the right upper extremity. The brachial and radial pulses were normal and symmetric to the left upper extremity.\nInitial radiographs of the right clavicle revealed a fracture involving its medial end without disruption of the sternoclavicular joint (Figures and ). Subsequent CT scan revealed a completely displaced fracture of the right medial clavicle. The fracture site was lateral to the medial physis of the clavicle and without disruption of the sternoclavicular joint. The CT scan allowed for a more definitive characterization of the fracture pattern. Given the rarity with which medial clavicle fractures not involving the physis occur in the pediatric population, a CT scan was desired to provide this information and to help with surgical planning.\nNonoperative and operative treatment options were discussed with the patient and his parents. The factors relevant to pursuing operative treatment included complete displacement at the site of the fracture, desire to obtain anatomic alignment of the fracture to promote healing, potential impact of the displaced clavicle fracture on shoulder function relative to daily activities and overhead sports in basketball and baseball, and clinical outcome allowing the patient to return to activity without pain and restricted function. The operative treatment agreed upon was reduction of the fracture and placement of an elastic intramedullary clavicle nail. Informed consent was signed by the parents and the patient.\nAt the time of the surgery, a trial of closed reduction revealed an unstable fracture. Subsequently, an incision was made centered over the fracture site. Exposure of the fracture site confirmed complete displacement with the lateral end of the clavicle anterior to the medial end. A drill hole using a 2.7 mm drill was made approximately 1 cm lateral to the medial end of the clavicle. The fracture was reduced and then a 1.5 mm elastic intramedullary titanium nail was passed through the drill hole in the metaphyseal area of the clavicle. The nail was advanced into the intramedullary canal, across the fracture site, and into the lateral end of the clavicle. Placement of the intramedullary nail was confirmed under fluoroscopy (Figures and ). The reduction and fixation were stable as the right upper extremity was brought through a full range of motion. An end cap was placed on the medial end of the clavicle nail to prevent migration. After wound closure and placement of a sterile dressing, the right upper extremity was placed in a shoulder immobilizer. The patient recovered from anesthesia without complications. Preoperative and postoperative examinations of motor function, sensation, and pulses were equivalent.\nPostoperatively, the patient was immobilized for 6 weeks until healing was evident on radiographs. He then started active-assisted shoulder stretching exercises at home. Although earlier mobilization could have been considered for this patient, we thought it prudent given his age and nature of the fracture to protect him during the immediate postoperative period for 6 weeks. Given the desire to return the patient to his basketball season and knowing the stress he would put on it during other sports requiring overhead activity of his operative arm, we wanted to ensure complete healing and avoid potential mechanical complications relating to the nail during the early postoperative period. At 8 weeks after surgery, the patient started using his right upper extremity for simple activities of daily living and strengthening exercises. At 10 weeks after surgery, he returned to basketball without any pain or functional limitations. Radiographic images in orthogonal planes confirmed complete healing of the fracture (Figures and ). Approximately 18 weeks after his initial surgery, the patient underwent an uncomplicated procedure to remove the intramedullary nail. Two weeks after intramedullary nail removal, the patient returned to activities without complications.
A 65-year-old lady from Syrian descent was referred to our institution in September 2014 for painless rapidly enlarging mass of the right cheek, which she noted only for 2 months prior to presentation. Relevant history included an early stage right breast invasive ductal carcinoma diagnosed 6 years earlier and treated by a lumpectomy with ipsilateral axillary lymph nodes dissection, followed by 6 cycles of adjuvant chemotherapy with CMF (cyclophosphamide, methotrexate, and 5-fluorouracil), focal radiotherapy to the chest wall and right axilla, and subsequent hormonal manipulation with letrozole for 5 years.\nThe patient was disease-free for the following 6 years until a growing right cheek mass led her to seek medical advice.\nPhysical exam revealed a palpable firm, nontender, nonmobile right inferior parotid mass of 2 cm at the level of the angle of the mandible. The right corner of the mouth dropped with asymmetry that increased with facial expression, thus indicating right mandibular branch palsy. The rest of the exam did not reveal any palpable cervical, supraclavicular, or axillary lymph nodes on both sides. Fibroscopy was done and was normal.\nContrast enhanced MRI scan of the neck revealed the presence of a 2 cm ill-defined mass of the right parotid gland at the inferior border, hypointense on T1 and mild hyperintense on T2 with moderate enhancement after gadolinium injection. No cervical lymphadenopathy was detected. Fine needle aspiration biopsy (FNAB) of the parotid mass revealed the presence of malignant cells of breast origin. Staging FDG-PET CT scan was obtained showing accumulation of 18-FDG in the right parotid and ruled out other distant or locoregional metastases ().\nA total parotidectomy was subsequently done with preservation of all facial nerve branches. However, the dissection of the mandibular branch was done with probable remnant of tumor cells on the nerve. The retroauricular vein was sacrificed because of direct invasion by the tumor. The anatomic pathology report confirmed the secondary nature of the tumor (of breast origin) invading the parotid parenchyma with perineural and perivascular invasion (). Although immunocytochemistry was not done on the FNA sample and was not needed for diagnosis, immunostaining with anti-estrogen receptor (ER) antibodies was performed on the final surgical pathology specimen and further supported the diagnosis as the tumoral cells were ER positive (). GATA3 immunostaining, a specific marker for breast cancer, was also done confirming the breast origin of the tumor ().\nThe patient tolerated well the procedure and was referred for adjuvant radiotherapy.
In November 2009, a 48-year-old Philippine man without relevant comorbidities was admitted to an Asian hospital to undergo surgical resection of a high-risk ileal 10 cm GIST.\nAfter surgery, he moved to Italy and came to our institute for a second opinion. Tumor tissues for the molecular analysis to sequence c-KIT were not available, so he was treated with a 1-year adjuvant treatment with imatinib at a daily dose of 400 mg daily, according to 2009 international guidelines.\nOn October 2012, a follow-up abdominal computed tomography (CT) scan detected a 45×32 mm unique local relapse, and treatment with 400 mg a day imatinib was administered again. A tumor biopsy was not performed due to the patient’s refusal.\nA CT scan performed after 6 weeks from imatinib onset showed that the lesion increased in size, with no areas of reduced contrast enhancement. Imatinib dosage was then increased to 800 mg a day, but a subsequent CT scan performed after 6 weeks showed no signals of treatment response. The lesion was unique at CT scan and was amenable to radical surgery; on February 27, 2013, the patient underwent surgical disease excision. A large implant of recurrent GIST was visible on the peritoneal surface of the abdominal wall, 8.5 cm in longitudinal diameter. It was apparently increased with respect to the previous CT scan, despite the fact that the patient had not interrupted imatinib administration. An enlarged epiploic appendix of the sigmoid colon was removed for histology, and a peritoneal washing was performed for cytology. The recurrent lesion was eventually radically removed, together with the adherent omentum, taking care not to open the lining capsule surrounding it.\nMacroscopically, the tumor was roundish and with a hard consistency; the maximum diameter was 8.5 cm. The cut surface was grayish and dishomogeneous for the presence of hemorrhagic areas. Histologically, the tumor was composed of bland spindle cells ( and ). There were no areas of tumor necrosis. Many dilated and thrombosed vessels resembling similar findings seen in neurogenic tumors were intermingled within the tumor cells. Immunocytochemical stains revealed strong cytoplasmic expression of CD117, DOG1, and CD34 (). No expression was detected for desmin and S100 protein. c-KIT (exons 9, 11, 13, and 17) and PDGFRα (exons 12, 14, and 18) mutational analyses were performed by bidirectional Sanger sequencing, using BigDye Terminator chemistry, on a 3500 Dx Genetic Analyzer. The test results showed a single mutation in exon 17 of the c-KIT gene (pN822K; ), confirmed in two independent amplifications, while the PDGFRα mutational status was wild type. Based on the evidence of prior response to imatinib (no tumor shrinkage at instrumental evaluation and no pathological response at the histological report) and the evidence of this rare mutation, treatment with imatinib was not restarted and we decided to begin a clinical–instrumental follow-up every 3–4 months.\nAt the time of this report, 18 months after surgical resection of the relapsed disease, the patient is still in complete remission.
A 10-year-old African American girl was admitted to our hospital with chief complaints of fever and abnormal movements.\nThe patient is a previously healthy, a 10-year-old girl who two weeks prior to admission started having episodes of headache on and off controlled with Tylenol. Headache was generalized, not associated with nausea or vomiting. Three days prior to admission, the patient had low grade fevers, and mom noticed some abnormal movements of the extremities which progressed over the next 2 days to the point where she cannot eat or dress on her own. She also had difficulty in walking and holding on to objects. H/o lip smacking and slurred speech are present for the past 3 days. H/o rash in the lower extremities which looked like hives is present for the past 2 days.\nH/o decreased appetite with loss of weight is present. The day prior to admission the patient had fevers with temperature up to 101 F, and with progression of involuntary movements the patient was taken to her PCP who started her on Acyclovir. With the symptoms getting worser, the patient was taken to an emergency center that evening before being transferred to our hospital for further care and management.\nNo H/o any prior hospitalization. Past H/o sore throat about a month ago which lasted for 2 days. At that time the child did not receive any medical attention, and sore throat went away on its own.\nThe child was adopted, and not much is known about the birth and family history. As per adoptive parents biological mom might have been worked up for some autoimmune diseases, the specificity of which is unknown.\nOn admission, the patient had a temperature of 99.5 F, weight of 55.5 kg (lost 10 pounds over the past 2 to 3 weeks), blood pressure of 99/77, heart rate of 129, and respiratory rate of 20.\nOn Neurological exam, the patient was awake, alert and cooperative. Involuntary lip smacking with tongue protrusion and slurred speech were present. Pronounced choreoathetoid movements of the upper and lower extremities were present. Milkmaid grip was positive. With arms outstretched above the head noticeable chorea with pronation of forearm was present.\nOn Motor exam Tone and reflexes were equal and normal in all 4 limbs. Strength was decreased in the lower extremities and choreiform movements were present.\nSensation was intact. She had trouble with walking without support.\nShe had a diffuse macular rash involving the lower extremities which is erythematous and confluent in some areas. The rest of her physical exam including her thyroid, cardiovascular, respiratory, and abdominal exam was normal.\nLab values from the outlying ER include a CBC which showed anemia with a Hb of 10.8 g/dL, hematocrit of 32.5%, MCV of 76.5, and thrombocytopenia with platelet count of 89000/mm3. CT scan and a spinal tap were done which were essentially normal. CSF studies showed no pleocytosis with normal protein and glucose. A gram stain of the CSF was negative. Bacterial culture and HSV PCR were pending at the time of admission.\nThe patient was worked up for the differential of choreiform movements. High on the list of our suspicion was sydenham's chorea secondary to rheumatic fever. ASO titre was elevated at 215 IU/ML (<150), ESR was elevated at 55 mm/hr (0–10), and urine analysis was positive for proteins at 25 mg/dL (neg), ketones at 50 mg/dL, and small blood. The patient was initially started on penicillin.\nAn echocardiogram of the heart was done () which showed 9 mm × 8 mm echogenic, nonmobile area on the posterior mitral valve leaflet suspicious for thrombus versus vegetation with normal function, and anatomy of the valves.\nMRI of the brain was done which was normal except for nonspecific small white matter hyperintensities in the left frontal lobe.\nWith the patient's history of fever, and possible thrombus versus vegetation on echocardiogram, thought of infective endocarditis was entertained. Blood cultures were sent, and the patient started on Lovenox and ceftriaxone pending culture results. For the possible thrombus, coagulation workups were done including a prothrombin time which was normal. Test for Cardiolipin IgG was negative at 8.3 GPL (Neg < 10.0 GPL) and Borderline positive for Cardiolipin IgM at 14.5 GPL (Borderline 10.0–14.9 GPL).\nNext on the list of our differential was systemic lupus erythematosus. With Biologic mom's possible workup for autoimmune disease and the fact that chorea even though is a rare complication for SLE can be a presenting feature especially in pediatric SLE, a blood analysis for ANA was sent. Other labs that were done to rule out other causes of chorea including urine and serum toxicology screen, thyroid panel, and lyme titres which came back negative.\nOn day 2 of the hospital course ANA titres came back positive at 1 : 1280 with a homogenous pattern. Further testing for SLE revealed positive results for anti-DS DNA at 473 IU/ML (Neg < 100), anti-Smith antibodies at 177AU/mL (Neg < 100), Sjogren's anti-SS-A at 937 AU/mL (Neg < 100), Sjogren's anti-SS-B at 118 (Neg < 100), and antihistone abs at 330 AU/mL (Neg < 100), and complement levels for C3 was low at 55 mg/dL (86–184). CPK levels were also abnormally elevated.\nBlood cultures and HSV PCR from the outlying hospital came back negative. Ceftriaxone and acyclovir were stopped and patient was started on IV steroids for SLE.\nThe patient's chorea dramatically improved after starting steroids. She received Solu-Medrol at 500 mg daily for 3 days and slowly tapered over to oral steroids over a course of 2 weeks. The patient was discharged home on oral steroids and Lovenox with follow-up appointments scheduled with rheumatology and cardiology.
A 35-year-old African man presented with an 8-year history of left upper limb pain. The pain was so severe that he was unable to sleep at night. He complained of almost incessant pain and paresthesia from the left side of his neck, and along his entire left upper limb. Touching him anywhere on his limb and neck would elicit a burning pain that radiated along his entire upper extremity. There was no antecedent history of trauma, and he did not have any features of neurofibromatosis type 1.\nOn further probing, he reported that the pain had initially started from the tip of his left middle fingertip. Over time, the pain had grown worse, and extended to involve the entire limb.\nThe pain was aggravated by cold weather, lifting of heavy weights with his left hand and direct pressure or trauma to his left middle finger. It was relieved by rubber band tourniquet (self-applied) and by rubbing either side of his finger on a rough cloth or other rough surface. During the 8-year period, he was managed for multiple different diagnoses including neurogenic pain, neuroma, gout and thoracic outlet syndrome. He underwent multiple diagnostic tests including computed tomography (CT) scan and magnetic resonance imaging (MRI) imaging of his spine and brachial plexus. He had a total of 12 stellate ganglion blocks administered over the 8 years without a resolution of symptoms.\nOn examination, he had neck tenderness along the brachial plexus roots, and along his entire left upper limb. His left middle finger was visibly thickened and dry, with exquisite tenderness over the entire pulp, precluding further examination.\nA diagnosis of chronic regional pain syndrome (CRPS), most likely secondary to a glomus tumor, was made. Previous MRI images were inconclusive, and we did not see the need to get new ones. We proposed an exploration of his left middle fingertip, and informed him that the finding and excision of a glomus tumor would be curative.\nHe consented to surgery, which was performed under a regional block and tourniquet, under loupe magnification. A 10×6 mm tumor was found adjacent to bone and excised (Fig. ).\nHis postoperative recovery was uneventful; he reported uninterrupted sleep for the first time in 8 years. So happy was he with his outcome, and so concerned for others that might be in pain like he had been, that he approached a local newspaper where he narrated his experiences []. The histopathology was reported as a glomus tumor (Figs. , , and ).
The patient was a 66 year-old male who was admitted to our hospital with extensive cellulitis and fasciitis of his left chest wall and axillary region. He mentioned trauma to his left arm 2 months ago which had caused a swelling and tenderness in his left arm. In another hospital he had undergone several debridements of soft tissue necrosis and broad spectrum antibiotic therapy. The patient had been diagnosed having necrotizing fasciitis at the previous hospital and the “finger probe test” was positive which identifies the disease.\nHe had a history of uncontrolled diabetes mellitus and coronary artery disease (,,). The patient was transferred to the ICU and after evaluating the wound the decision to amputate the extremity was made and amputation was done the day after admission. In order to control his infection after taking multiple cultures from his wound, blood and urine, high-dose clindamycin, vancomycin and 24 million units of crystal penicillin was started. The patient was admitted on an emergency basis and before operation the lab tests were: Hb: 8 mg/dl, WBC 25000, BUN 10, creatinine 1.1 mg/dl; the cultures grew a mixed bacteria, consisting of methicillin resistant Staphylococcus aureus, Streptococci and Pseudomonas aureoginosa resistant to all the antibiotics. The blood and urine cultures were negative since the patient was receiving high doses of antibiotics. During the first postoperative day the patient developed a low hemoglobin and a 2.5 mg/dl creatinine, thus the antibiotic regimen was adjusted accordingly. The patient also had a post operative blood sugar of 331 mg/dl.\nThe patient became lethargic and because of his respiratory distress he was intubated and mechanical ventilation was started. The blood test revealed a white cell count of 40,000/dl and hemoglobin of 9 g/dl. His ABG also showed compensated metabolic acidosis. On the second postoperative day the patient’s metabolic acidosis progressed and because of increasing creatinine levels acute tubular necrosis was diagnosed. He received 2 units of packed cell and Lasix. On the 3rd postoperative day he developed a low blood pressure which was unresponsive to dopamine drip. On the same day the patient had bradycardia and asystole and underwent CPR. The CPR was unsuccessful and the patient passed away.
A 50-year-old male patient, a known case of COPD, presented with the features of BPF on the right side for 1 month. The patient was a chronic smoker and did not give any history suggestive of pulmonary Koch's or any other associated disease apart from COPD. The patient had sudden-onset breathlessness and chest pain 1 month before, which was diagnosed as spontaneous pneumothorax, and an intercostal drain was inserted but even after 1 month of all conservative measures, the lung remained collapsed [] and there was a large air leak in the intercostal drain; negative suction on the intercostal drain was also not effective. Bronchoscopic closure of the BPF was also attempted but was not successful. CT chest revealed collapsed and entrapped lung [Figure and ], with surgical emphysema of the subcutaneous tissues due to rupture of the emphysematous bullae on the right side along with presence of emphysematous bullae on the left upper lobe. Surgical repair was planned as with all the conservative measures, the lung remained collapsed and the air leak persisted. The patient was emaciated and weak and was pre-operatively optimized with a high-protein diet, multivitamins, bronchodilators, incentive spirometry and antibiotics for another week before surgery. General anesthesia was given using double-lumen endotracheal tube and supplemented with thoracic epidural analgesia and invasive arterial pressure and central venous pressure monitoring were also performed. The right posterolateral approach was chosen, intercostal muscle flap was harvested and part of the fifth rib was resected. The lung was found completely entrapped in a fibrous peel [] and decortication of the thickened visceral peel was performed from the entire right lung [] and inferior pulmonary ligament was also ligated and divided. The site of the BPF was localized in the segmental bronchus to apical segment of the right upper lobe and closure of the fistula with polypropylene 4’0’ sutures was performed and reinforced with intercostal pedicled muscle flap []. There were multiple small unruptured bullae present near the site of the BPF, and they were also closed with polypropylene 4’0’ sutures. The repair site was tested for air leak after pouring saline in the thoracic cavity and after ensuring no major air leak, two intercostal drains were inserted and standard thoracotomy closure was carried out. The patient was extubated in the operating room and deep breathing exercises were started from the first post-operative day to keep the lungs expanded. Chest roentgenogram on the second post-operative day [] revealed partial re-expansion of the lung along with presence of residual space in the upper zone. With continued chest physiotherapy and respiratory exercises, there was complete re-expansion of the lung with obliteration of the remaining space in the upper zone []. The patient recovered well and was discharged after 10 days. Histopathological examination of the resected visceral peel revealed non-specific inflammation without any evidence of granuloma formation or dysplasia and cultures were sterile.
A 5-year-old boy sustained a pedestrian traffic accident on March 18, 1977, which resulted in the loss of the right radial shaft, severe soft tissue injuries to the extensor muscle belly, avulsion of the entire forearm skin with some skin loss, and elbow dislocation (). He was able to move his fingers. Wound debridement and reduction of the elbow were carried out on the day of admission, which was followed by skin graft. The wound was completely covered with the grafted skin, and infection was also controlled by the end of April. The radiograms taken on May 25, 1977 showed the preserved distal physis of the radius. Transposition of the distal end of the osteotomized proximal ulna shaft to the remaining distal metaphysis of the radius was performed on June 8, 1977 ().\nOn the radiogram of the right forearm, taken 4 weeks after the reconstructive surgery, new bone formation in the ulna periosteal tube was shown, and complete bone reformation of the ulnar ray was seen 3 months after surgery. Hypertrophy of the ulna was seen 6 months later. At the time, the boy demonstrated almost normal right hand function with a stable wrist (). Then, the boy was lost to follow up for a while. On January 5, 1979, the boy revisited the outpatient clinic complaining instability of the right wrist with degradation of right hand function. The radiograms taken on the day disclosed pseudoarthrosis at the metaphysis of the radial ray of the bifurcated single bone forearm (). A periosteal corticocancellous graft harvested from his right tibia was grafted to the nonunion site on January 10, 1979 (). Radiograms of the right forearm, taken 6 months after bone graft disclosed solid union. Follow-up was continued until June 22, 1992 and at the final follow-up, normal wrist and hand function was restored. The forearm lengths of the normal left and the reconstructed right sides were 27.5 cm and 17.5 cm, respectively, at the age of 20 years (). However, there was no growth discrepancy between the radial and ulnar rays of the reconstructed one bone forearm, and no wrist deformity.\nInformed consent was obtained from the patient for publication of this case report.
A 39-year-old woman was scheduled to undergo split thickness skin grafts as part of a reconstructive program following extensive burns. She was burned in a house fire 2 years previously, when she suffered 93% total body surface area burns, with a burn index of 85. Her history was significant for having undergone 39 reconstructive operations over 2 years. Her neck was reconstructed using a graft from the latissimus dorsi, and both femoral veins were occluded due to multiple accesses and indwelling catheters. A subclavian venous catheter had been inserted once before under ultrasound guidance. Physical exam showed her weight was 49 kg, and her height was 155 cm. Most of her body was covered by hard contracted skin. There were no peripheral veins evident on inspection.\nTo obtain venous access for this operation, we searched for veins using a linear probe ultrasound device (6–15 MHz, SonoSite Edge, FUJIFILM SonoSite, Inc., Washington, USA) on the chest and arm. This revealed accessible veins, including the subclavian and axillary veins on the anterior chest, and the brachial vein of the right arm. An indwelling catheter in the right brachial vein was considered as the first choice to avoid possible complications of using the subclavian or axillary veins such as pneumothorax or hemothorax. The right brachial vein measured 3 mm by ultrasound imaging.\nThe right arm had restricted motion range due to extensive contractures. She was positioned in the right semilateral position to gain easy access to the medial side of the arm by supination and abduction. A pillow was placed behind her back to maintain this position. The skin of the arm was prepped with 1% chlorhexidine alcohol solution and covered with a sterile drape. The ultrasound probe was covered with a sterile plastic probe cover. The indwelling venous catheter was placed using sterile barrier precautions. Local anesthetic (5 mL of 1% lidocaine) was injected, and a 20G catheter-over-the needle (48 mm) was inserted. Ultrasound-guided venous catheterization was performed. To access the brachial vein, we used the short-axis out-of-plane approach to avoid mechanical complications of unanticipated artery or peripheral nerve injuries, and then used the long-axis in-plane approach to penetrate the anterior vein wall and cannulate the vein. After inserting the cannula, a guide wire was placed through the cannula using the modified Seldinger technique. We placed a single lumen polyurethane catheter (3 Fr., SMAC Plus, Nippon Covidien, Japan), a commercially available central venous catheter (there is no commercially available midline catheter in Japan). Successful insertion of the catheter was confirmed with ultrasound imaging (Fig. ). The catheter entered the vein 6 cm from the skin entry site (Fig. ). After placing the catheter, we checked venous flow using color Doppler imaging (Fig. ).\nSurgery was performed the following day with no adverse events. A split-thickness skin graft was harvested from the head, and grafted to the neck. The skin graft became infected several days later and the midline catheter was used for administration of antibiotics. A second skin graft was performed again postadmission day 16 and the catheter removed on postadmission day 24. She was discharged without complications.
An 11-year-old female child with hypoplastic left heart syndrome underwent staged palliation culminating with an extracardiac fenestrated Fontan procedure at 4 years of age. Before the Fontan procedure, she maintained oxygen saturations between 75% and 77%, whereas after the Fontan procedure, she maintained saturations between 92% and 94%. She did well for the subsequent 7 years, but by 12 years of age, there was evidence of Fontan dysfunction. At 12 years of age, a cardiac catheterization was performed which showed evidence of suboptimal Fontan pathophysiology with elevated Fontan pressures of 18 mmHg, hepatic vein wedge pressure was 20 mmHg, and right ventricular end-diastolic pressure was elevated at 16 mmHg.\nAn echocardiogram at the same time demonstrated moderate-to-severe tricuspid valve regurgitation and moderately decreased right ventricular systolic function.\nShe underwent an abdominal magnetic resonance imaging (MRI) as a routine screening for Fontan-associated liver disease. MRI was significant for evidence of hepatomegaly, liver congestion, and presence of hypervascular nodules. There was an incidental finding of a 3.7 cm complex, heterogeneous mass that appeared to be arising exophytically from the head/uncinate process of pancreas with the duodenum draped anteriorly over the mass [].\nThere was a coincidental history of chronic abdominal discomfort, poor appetite, and poor weight gain over the preceding year. In light of the MRI findings, there was concern that the mass was contributing to some of her symptoms. The main differential diagnosis based on the MRI findings was pseudopapillary tumor of the pancreas. Secretory tumors such as pheochromocytoma were considered less likely due to location and absence of typical signs and symptoms such as hypertension and tachycardia. Diagnostic certainty was necessary to establish heart transplant candidacy. The anatomic location of the mass in continuity or in proximity to the uncinate process of the pancreas warranted planning for potential Whipple procedure. After extensive discussions among the different specialties and the family, a decision was made to proceed with the surgery.\nIn the operating room, the mass was immediately visible on the right side on abdominal exploration. Neovascularization was seen around the mass and it was found to be moderately adherent to the inferior vena cava. After severing all vascular ties, the mass was found to be discrete from the duodenum and pancreas. It was lifted up from its retroperitoneal location after a generous Kocher maneuver, removed from the body, and sent to pathology. A liver biopsy was also obtained at the same time and sent for pathology.\nOn gross examination, the mass was pink-red to pink-tan rubbery in consistency. Frozen section was suggestive of paraganglioma. Hematoxylin and Eosin stains showed well-defined nests of cuboidal cell with abundant cytoplasm, separated by highly vascularized fibrous septa []. Immunostains exhibited synaptophysin positive, chromogranin A positive, and S100 positive for sustentacular cells []. These findings were consistent with diagnosis of paraganglioma.\nLiver biopsy histopathology showed moderate-to-severe fibrosis with sinusoidal dilatation []; the Masson's trichrome stain confirmed the degree of fibrosis []. Findings were consistent with Fontan-associated liver disease.\nThe patient recovered very well from the procedure and was extubated shortly after arrival at the intensive care unit. Her hemodynamics were stable throughout the procedure as well as recovery. Over the subsequent 2 months, she continued to demonstrate cardiac dysfunction and developed exertional dyspnea. On follow-up, imaging (positron emission scan) as well as endocrine assessment in the form of metanephrine levels was unremarkable.\nIn the setting of failing Fontan physiology as well as the presence of associated liver disease, the patient was, therefore, evaluated and listed for heart transplantation. In certain patients with Fontan-associated liver disease, including those with significant fibrosis but without ascites, with low (<2) varices, ascites, splenomegaly, and thrombocytopenia scores and low modified end-stage liver disease score, we have opted for single-organ transplantation (heart) rather than dual heart–liver transplantation. She underwent heart transplantation without complications and now 6 months out she is thriving.
A 40-year-old female patient presented to our emergency department after a simple fall. She reported pain upon palpation of the lower lumbar spine and radiating pain to her left buttock and lower leg. The patient reported a history of low back pain and left sciatica, with exacerbation of the symptoms after the fall. Clinically, no neurologic deficit was present. The straight leg raising test was positive on the left side. Simple radiological control of the lumbar spine revealed a sagittal cleft of the body of L4 vertebra on the AP view () and a wedge-shaped deformity of the same vertebra on the lateral radiograph (). Additional hematologic evaluation that included total leukocyte count, erythrocyte sedimentation rate, serum calcium, serum alkaline phosphatase, and serum protein electrophoresis was performed, in order to rule out a pathologic fracture or infection. All values were within normal limits. Computed tomography (CT) imaging of the lumbar spine showed a large, symmetrical cleft-like defect involving the entire vertebral body of L4 with no evidence of canal compression (). The spinal arch of L4 was intact and no paravertebral soft tissue swelling was evident (). A posterolateral prolapse of the L5-S1 intervertebral disc was evident, which correlated with the patient's symptoms. The diagnosis of L4 butterfly vertebra was then established. A thorough clinical examination of the patient, the patient's medical history, and her family's medical history did not reveal any information associated with the incidental finding of the butterfly vertebra. Subsequently, a simple radiological evaluation of the entire spine was performed, in addition to ultrasonography of the heart, urogenital, and hepatobiliary systems, so as to exclude associated congenital abnormalities. A magnetic resonance imaging (MRI) of the lumbar spine reconfirmed the sagittal defect of the L4 vertebral body, occupied by intervertebral disc tissue that included nucleus pulposus material (). The intervertebral discs L3-L4 and L4-L5 communicated with a bar of disc material which prolapsed in the cleft of the L4 body. Incidentally, a hemangioma was found to be present in the body of L4 vertebra ().\nThe patient was treated for her low back pain with analgesics and physiotherapy. The benign nature of the butterfly vertebra was explained to her and she was informed that no further treatment was required. Treatment of the L5-S1 disc prolapse was deferred for a later stage.
Patient SA was a 72-year-old, right-handed woman who first reported noticing her symptoms 3 years previously when she had a fall. At that time, it was observed that her speech had a telegraphic quality. She developed progressive difficulty speaking and writing, swallowing, and controlling her right hand. She began to use her right arm less frequently. Although she could voluntarily move it if necessary, there was a lack of spontaneous use. Soon, she began to experience difficulty chopping vegetables using the right hand. She encountered problems with her right hand grip, but at that time had no difficulty letting objects go. Prior to testing, she noted that her walking had slowed. She began to experience difficulties standing from a seated position. There was no family history of neurodegenerative disease.\nOn examination, she had a profound expressive aphasia and impaired articulation. However, she was able to comprehend 3-stage commands well. Visual fields were full to confrontation. There was no evidence of visual or tactile extinction. Eye movements were full, but she was slow to initiate saccades, particularly towards the left compared to the right and there was some evidence of gaze impersistence. Such oculomotor deficits are not uncommon in CBS patients. There was no facial weakness and palatal movements were normal. There was no pout reflex.\nThere was rigidity of the right arm and poor fine finger movements, but good strength throughout. The right hand showed evidence of mild alien hand behaviour, with involuntary grasping of any object that was brought close to it. The patient was adamant that she was not willing the hand to do this, and she could not stop this behaviour even when she made an effort to do so. There was no evidence of alien hand behaviour in the left hand.\nExamination did not reveal any dystonia or limb apraxia, above and beyond the problems associated with fine control of the right hand movements. There was no amorphosynthesis in the left hand. When she walked, there was reduced arm swing, more prominently on the right than on the left, but she had a good stride length and postural reflexes were intact. There was no evidence of some of the other behaviours which are common in AHS: no levitation of either arm, no mirror movements, and no intermanual conflict between the hands. Overall, the clinical presentation was considered to be consistent with CBS.\nMagnetic resonance imaging (MRI; ) demonstrated cortical atrophy, slightly more prominent over parietal than frontal regions and in the left hemisphere compared to the right. In addition, there was reduction in volume of the caudate head bilaterally. These findings would be consistent with the clinical diagnosis of CBS. Selected images in demonstrate loss of volume of the left medial frontal and parietal cortex with a pathologically widened cingulate sulcus (white arrowhead); loss of cortical volume adjacent to a widened intraparietal sulcus particularly involving the superior parietal lobe, most prominently on the left (yellow arrowhead); widened sulci over superior parietal and frontal regions, including the left central sulcus (red arrowhead); and reduction in caudate head volume bilaterally (left side marked with green arrowhead).\nSA completed the two different experiments on two different days, approximately 4 weeks apart. The affordance task was performed first. This study was approved by the local human subjects ethics committee and the patient gave written informed consent prior to testing.
A 59-year-old man with past medical history of hyperlipidemia presented to the ED following two episodes of unwitnessed syncope. The patient reported a sudden onset of nausea and vomiting prior to these episodes. He described a room-spinning sensation when he awoke associated with ringing in the ear. He regained consciousness within one to two minutes. While attempting to stand, he again lost consciousness. In the ED, the patient denied any symptoms.\nIn the ED, his physical examination was significant for symptomatic orthostatic hypotension. Vitals included blood pressure of 88/54 mmHg and a heart rate of 85 beats/minute. The patient was afebrile. The patient's initial laboratory workup results are presented in Table . His electrocardiogram showed normal sinus rhythm. His chest X-ray findings were unremarkable. A CT scan of the patient's head showed an apparent hyperdensity in the left intracranial internal carotid artery just proximal to the bifurcation, representing an artifact or the presence of an embolus. It also showed an apparent sellar mass with calcifications and suprasellar extension. A CT scan of the abdomen showed cholelithiasis and was negative for any acute pathology.\nThe patient was admitted for further evaluation of syncope. Sepsis was considered given his leukocytosis and hypotension. A CT scan of the head showing a questionable embolus was followed by a neurological evaluation to test for a possible stroke. The patient was admitted to a telemetry unit to rule out syncope due to cardiac arrhythmias. Intravenous fluids were started to treat his hypotension.\nOn the patient's second day of admission, the intensive care unit team was consulted for persistent hypotension. Intravenous hydration was recommended along with a follow-up workup for sepsis and pulmonary embolism. A morning cortisol level was also ordered to rule out adrenal insufficiency. The patient was not started on antibiotics at that time as he remained afebrile, the leukocyte count normalized, and the blood culture showed no bacterial growth. A CT angiogram of his chest was significant for a small filling defect in a right upper lobe segmental pulmonary artery, compatible with pulmonary embolism. The patient was not started on therapeutic anticoagulation as a follow-up ventilation-perfusion scan showed low probability for pulmonary embolism.\nThe patient underwent a cardiology evaluation for syncope and suspected underlying arrhythmia. Telemetry monitoring was negative for significant cardiac arrhythmias. Echocardiography showed an ejection fraction of 55%, normal diastolic function, and normal valves. The results of his neurological exam were benign, as the patient had no focal weakness and reflexes were normal. MRI of the brain with contrast was negative for a stroke but was significant for an enhancing mass filling and expanding the sella turcica, measuring 1.5 cm caudally, 1.2 cm anteroposteriorly, and 1.5 cm transversely. It encroached on and elevated the optic chiasm (Figures , ).\nOn the patient's third day after admission, his morning cortisol level was inappropriately low (1.6 µg/dL; reference range: 6.0 to 18.4 µg/dL). The endocrinology team was consulted over concerns for secondary adrenal insufficiency due to the sellar mass. A cosyntropin stimulation test showed maximum cortisol elevation to 10.1 µg/dl. Table present the patient's hormone level results. He was diagnosed with central hypothyroidism, then panhypopituitarism due to the pituitary macroadenoma. The patient was started on hydrocortisone, 10-mg tablet in the morning and 5-mg tablet in the evening, along with levothyroxine, 50-mcg tablet once daily.\nThe patient was discharged with recommendations for outpatient follow-up with the endocrinology team for continued monitoring of his pituitary hormone level deficiencies and sellar mass. Outpatient ophthalmology follow-up was also recommended for visual field assessment, as the mass was causing elevation of the optic chiasm. Finally, his care team recommended outpatient neurosurgery follow-up evaluation for possible surgical resection of the mass. The outpatient follow-up assessments were ultimately delayed due to the novel coronavirus disease (COVID-19) pandemic.
A 20-year-old male patient, with an alleged history of accidental fall from a height of about 20 feet, presented to the emergency department on May 30, 2018. Initially, he was taken to a local hospital where computed tomography of the head was done which revealed an extra-axial hyperdense collection of hemorrhagic attenuation with few air pockets within and along the right parietal and anterior temporal convexity with mass effect in the form of compression of right lateral ventricle and minimal midline shift to left [Figures and ] along with fracture of right parietal bone extending up to squamous and mastoid parts of right temporal bone []. Hemorrhage was noted along the falx. The initial scan was done at a primary center where no neurosurgeon was available, and hence, he was shifted to our tertiary care center 7 h after the initial scan. On general examination, the patient was in a state of unconsciousness, with stable vital signs. Right otorrhagia was noted which can be attributed to the fracture described above. On neurological examination, Glasgow Coma Scale score was E1V2M3, and anisocoria was noted with right pupil size of 5 mm and left pupil size of 2 mm. Both pupils were sluggishly reacting to light. The patient was intubated and a computed tomography of the brain was repeated which, to our astonishment, revealed no epidural hematoma but showed hemorrhage along the falx, and the same fracture details described above and an increased epicranial soft-tissue thickening were also noted [Figures and ]. Another scan was taken after 4 h, and no change in the status was noted. After 2 days of admission and intensive neurological care, the patient succumbed to sudden cardiopulmonary arrest due to ventricular arrhythmias.\nThe initial intention was not to go for a conservative management. However, the first scan was done at a rural center where no neurosurgeon was available and hence referred to our hospital which is a tertiary care center. By the time the patient reached us, it was almost 6–7 h since the first scan was taken. Hence, an emergency repeat scan was done to assess the current status and take up for surgery, which, to our astonishment, revealed a resolution of the epidural hematoma. Moreover, this changed our plan of management from surgical to conservative.
A 43-year-old man developed unremitting pain in the lower back and the right leg, which especially increased while walking. Subsequently, the patient was admitted to our hospital. The patient received bed rest for more than one month. Magnetic resonance (MR) and emission computed tomography (ECT) images demonstrated the presence of L3, S2 and S3 vertebral lesions that were metastatic tumors. Computed tomography (CT) images demonstrated the presence of metastatic tumors that involved the right acetabulum and the femoral head () that were associated with lung cancer (as determined by surgical pathology). Based on these clinical findings, the patient was diagnosed with multiple metastatic bone tumors. The patient received radiation therapy and experienced pain relief for one month. However, the patient experienced recurrent pain and the pain had seriously affected sleep and the daily activities of the patient. The pain was insensitive to drug administration. Furthermore, side effects of opiate use such as effects on the gastrointestinal tract, had become apparent. Due to the short life expectancy of the patient, a surgical procedure was not performed. Consequently, a percutaneous procedure was considered due to the potential ability to provide prompt pain relief and functional improvement. The patient was informed about the possible benefits and risks of the treatment. Written informed consent was obtained from the patient and his family.\nFollowing detailed analyses of the lesions and adjacent structures, we developed a modified procedure for the treatment of the case. First, PVP was performed on the L3 lesion, and subsequently backache was alleviated. Second, nine days later, POP was performed on the right acetabulum and femoral head. Prior to the performing the procedure, it was essential to review the radiology films of the patient to avoid causing injury to important vessels and nerves. We administered local anesthesia to the patient and placed the patient in the prone position on the uniplane digital subtraction angiography (DSA, AXIOM Artis dTA, Siemens, Erlangen, Germany) operating table. A fluoroscope draped with a sterile sleeve was positioned to allow an anteroposterior (AP) view. A small dermatotomy incision was made with a scalpel blade. Thereafter, a bone puncture needle (13 G, Cook, Bloomington, IN) was used; the needle penetrated the neck of the upper portion of a femur lever attached to the femur flank along the femur neck axis (). We adjusted the cephalopod and left-right angle according to positions depicted on sagittal and axial images until the needle attained the lesion, as seen for all fluoroscopy projections. After removal of the needle, commercially available polymethyl methacrylate (PMMA) (Simplex P, Stryker Howmedica Osteonics, East Rutherford, NJ) was carefully injected into the lesion under continuous fluoroscopic monitoring via lateral and AP projections in order to ensure adequate lesion filling and to avoid PMMA leakage. When bone cement appeared at the neck of the femur cerebral cortex edge, injection was ceased. A total of 5 ml PMMA was injected into the right femur head. Post-procedural fluoroscopic observations showed optimal filling of the lesion with no evidence of PMMA extravasation (). Subsequently, we used a bone puncture needle to penetrate the right acetabulum. For successful penetration, 6 ml of PMMA was injected into the acetabulum. Immediate post-procedural X-ray photographs demonstrated good distribution of cement in the lesion and there was no evidence of PMMA extravasation (). Blood pressure, an electrocardiogram and pulse oximetry waveforms of the patient were monitored and were observed to be steady during the procedure.\nThe patient experienced considerable pain relief and functional enhancement, and the patient was able to walk with the aid of crutches on the following day. The patient was evaluated prior to and following the procedure for reduction of pain intensity using a numerical rating scale (NRS) with scores ranging from 0 (no pain) to 10 (worst pain imaginable) (). The NRS score decreased from 8 to 2 within 48 hours following POP. CT scans acquired three months following the procedure revealed a stable femur head with no further tumor growth and an unchanged location of the instilled cement (). The patient has continued to be pain-free without medication and has experienced no limitations in activity.
A 23-year-old woman with no known risk factors for breast and/or ovary cancer who had a fibroadenoma excision under local anesthesia in Jan 2019 was presented to our hospital because of left breast mass. The mass was first noticed in March 2019 and was increasing in size dramatically, growing from the breast towards the armpit over the next two months (see in the Appendix A section for timeline). The fast-growing mass was deep, hard and minimally mobile. It was associated with skin redness and engorgement of the breast. There was no nipple discharge. There were no other masses noticed in the right breast or any other site (). There were no systemic symptoms like fever, general weakness or change in body weight. By May 2019 the mass started to feel painful and the patient was unable to sleep without taking NSAIDs. Hence, the patient visited the surgery clinic. Upon physical exam, the young lady looked well, with stable vital signs. Her right breast showed longitudinal scar lateral to areola related to the previous excision of fibroadenoma. There were no palpable masses on the right side. The left breast was markedly enlarged, especially in the outer quadrant, with a small patch of skin discoloration (necrosis) measuring around 1 cm in the upper inner quadrant above the nipple. Many dilated superficial veins were seen, with no nipple discharge. On palpation of left breast, two masses were felt: one parenchymal measuring almost 10 × 8 cm, and the other deep between breast tissue and axilla (muscular) with ill-defined inner borders. Both masses were hard and immobile, and they were fixed to underlying structures. No palpable axillary lymph nodes were found.\nThe rest of her physical exam results were within normal limits. Her blood and laboratory results of complete blood count, kidney function tests, and liver function tests were all within normal limits.\nBreast ultrasound and breast MRI were performed on the 11th of May 2019 and showed normal right breast and multiple left breast lesions. Lesions' appearance was suggestive of bilobed/multifocal intra-parenchymal phyllodes tumor (). A well-circumscribed mass located between the anterior chest wall and pectoralis major muscle was seen (). The impression of a multifocal intra-parenchymal and sub-pectoral phyllodes tumor was concluded by MRI imaging (). Histopathological study of left breast mass Tru-Cut biopsy performed on the 27th of May 2019 showed phyllodes tumor of borderline category: it featured an adequate sample of tissue with fibro-adenomatoid changes showing prominent stromal cellularity, occasional mitoses, and no apparent stromal atypia. A pan CT scan revealed no radiological evidence of distant metastatic disease.\nThe following surgery was performed on the 3rd of June 2019: left breast nipple-sparing mastectomy with the excision of a retro-pectoral mass () and immediate breast reconstruction using a submuscular Silicone Implant (305 cc Mentor™ anatomical implant) & Ti-Loop™ Mesh.\nMastectomy specimen showed a 16 × 14 × 10 cm mass, comprised of two tumors, (). First one is 8 × 7 × 6 cm, and the second one is 12.5 × 8 × 7.5 cm. Microscopic examination showed hypercellular stroma with atypia, the mitotic rate is high and about more than >10 mitotic figure per 10 high per field with atypical mitosis, with marked stromal overgrowth with focal osteoid matrix identified. The densely packed anaplastic stromal cells are infiltrating into adjacent borders, representing a malignant phyllodes tumor, that is incompletely excised. The 1st tumor is reaching the anterior and lateral margins (subareolar/cutaneous margin) 0.6 cm superior margin, posterior and medial margins are free (more than 1 cm away). The second tumor is also reaching the anterior margin (subareolar/cutaneous margin), Posterior margin (chest wall) is 0.1 cm away, lateral and medial margins are free (more than 1 cm away). Both, Heterologous elements (osteoid) and stromal overgrowth are identified. Histopathology for retro-pectoral mass showed an 8 × 7 × 6 cm malignant phyllodes tumor that is excised with 0.1 cm superior margin and 0.2 cm for both lateral and medial margins.\nOne-month post-operation and after full patient recovery (), the patient was sent to a radiotherapy unit at another medical center for assessment and consultation. The patient was planned for adjuvant radiotherapy to the chest wall: 2.67 Gy over 33 sessions starting on the 16th of September and finishing by the 3rd of November 2019.\nThe patient is now scheduled for 6-month-interval follow-up for local recurrence and distant metastatic workup.
A 35-year-old G4P2012 presented to labor and delivery at full term in early labor with spontaneous rupture of membranes. She had undergone living donor kidney transplant 8 years prior for ESRD suspected secondary to IgA nephropathy versus post-streptococcal glomerulonephritis. She was maintained on Prograf and prednisone throughout her pregnancy with a stable baseline serum creatinine 1.2 mg/dL. Obstetrical history was significant for two prior uncomplicated Cesarean deliveries, both prior to her kidney transplantation. It was unclear from her records whether she had undergone counseling regarding pregnancy post-transplant. She declined a of trial of labor after Cesarean section despite counseling on the risk of injury to the transplanted kidney given its location in the operative field, which was confirmed on transabdominal ultrasound prior to the case.\nThe patient was taken to the operating room for and indicated repeat Cesarean section. Routine dissection was performed down to the level of the fascia through a Pfannenstiel incision and the fascia was carefully transected horizontally in the usual fashion. When the superior fascial edge was lifted for traction to allow for dissection from the underlying rectus muscle, brisk pulsatile bleeding was noted. The transplanted kidney was noted to be densely adherent to the overlying anterior abdominal wall, and avulsion of a portion of the transplanted kidney was confirmed (Fig. ). Direct pressure was applied to the organ while the Trauma Surgery team was consulted. The avulsion was repaired using 3–0 pledgetted Prolene sutures in interrupted horizontal mattress fashion and using Evarrest compound. At this time, the decision was made to extend the skin incision in the vertical midline for improved access to the uterus given the concern for further shearing forces to the exposed transplanted kidney in the process of delivering the baby. The rest of the case was uneventful. No blood product transfusion was necessary. After the transplanted kidney was repaired, a viable male infant with Apgars of 8 and 9 weighing 2460 g was delivered.\nPostoperative serum creatinine levels remained stable between 1.1–1 .3mg/dL and the patient maintained adequate urine output. Renal ultrasound immediately postpartum and 1 month postpartum demonstrated normal arterial and venous flow with no evidence of ischemia or perinephric hematoma.
An 11-year-old girl was referred to our hospital in August 2010 with a lesion in the right cheek area which was progressively enlarging. The patient complained that her nose and mouth corner were crooked and that her face was swelling. In clinical examination, the patient had a slight asymmetry in the right midface as a result of buccal and palatal cortical expansion from the right maxillary canine to the molar region, resulting in depression of the nasal alar and mouth corner (Fig. ).\nA panoramic radiograph showed an increased bone density on the right maxilla and zygoma and obliteration of the right maxillary sinus. Computed tomography (CT) revealed a 4.5 × 4 × 4.5 cm, expansile ground-glass opacity lesion involving the right maxillary sinus, right maxillary alveolar process, zygoma, and hard palate. Bone scan revealed an irregularly shaped hot uptake in the right maxilla, and no abnormally increased uptake was observed at any other sites (Fig. ). The physical examination did not show any other lesions, and the patient had no history of pain, trauma, loosening of teeth, or trismus. Based on the typical radiologic findings, the patient was diagnosed with FD, and no additional biopsy was performed. The patient had regular follow-up every 6 months to monitor the lesion’s progress. At the 1-year follow-up, the development of tooth germ within the lesion was normal, and slight expansion of the lesion to the bucco-lingual side was observed. Because we thought the patient was still growing and increasing in height, we decided to conduct an ongoing progress observation.\nAbout 3 years later in December 2012, there were no significant changes of the FD lesion, but the distance from the mouth corner to the inner canthus was about 2 cm longer on the right side than on the left. Periodic observation was continued, and in August 2017, corrective surgery was planned because the maturation of the lesion was confirmed to be complete and there were no changes in the size of the lesion. At that time, the distance from the mouth corner to the inner canthus was 2.5 cm longer on the right side than on the left, and the distance from the occlusal plane to the outer canthus was 5 cm longer on the right side than on the left. Bone contouring surgery, the primary treatment for facial asymmetry and fibrotic bone lesions, was planned (Fig. ).\nThe patient wanted to improve asymmetrical facial appearance through the surgery. Therefore, we aimed not only to remove the FD lesion, but also to make the patient’s facial as symmetrical as possible. For this, direct visualization and surgical approach to the infraorbital rim and lateral area of zygoma were required, but the surgical approach through buccogingival incision had limited access to these areas. On the other hand, the midfacial degloving approach was expected to help reestablishing symmetric facial contour by allowing direct comparison of the lesion with the normal side. Moreover, this approach provides esthetically acceptable outcomes, leaving no scars and no functional disability. Therefore, we decided to perform the operation through the midfacial degloving approach.\nWith the patient under orotracheal anesthesia, the lesion was removed by the midfacial degloving surgical procedure. Local anesthesia with 2% lidocaine with epinephrine (1:100,000) was infiltrated into the maxillary vestibular mucosa and into the nose. The procedure is performed with a maxillary vestibular incision and three intranasal incisions to expose the entire midface skeleton that include (1) bilateral intercartilaginous, (2) complete transfixion, and (3) bilateral piriform aperture incisions (Fig. ).\nA buccogingival incision was made in the maxillary vestibule approximately 5 mm superior to the mucogingival junction and extended from the second molar to the contralateral second molar. Periosteal elevators were used to elevate the tissues in the subperiosteal plane fist over the anterior maxilla and then extending widely to encompass posterior tissues behind the zygomaticomaxillary buttress. The infraorbital neurovascular bundle was identified superiorly and dissected. Subperiosteal dissection along the piriform aperture stripped the attachments of the nasal labial muscularture to allow its complete release from the midface skeleton. The mucoperiosteal flap was elevated up to the piriform aperture.\nThe intercartilaginous incision divided the junction between the upper and lower lateral cartilages (Fig. b). An incision was made along the inferior border of the upper lateral cartilage, beginning at the lateral end and extending medially curved into the membranous septum anterior to meet transfixion incision (Fig. a). Laterally, the incision was sufficient that it extended to the piriform aperture. The lower lateral cartilage was eventually displaced superiorly during the degloving procedure, whereas the upper lateral cartilage remained attached to the midface skeleton. The transfixion incision was used to separate the membrane septum/columella from the cartilaginous septum. An incision was made along the caudal border of the septal cartilage from the medial end of the intercartilaginous incision toward the anterior spine (Fig. a). The intranasal incision was made by a full-thickness incision down through the periosteum of the piriform margin and the nasal floor.\nDissection through the intercarilaginous incision allowed access to the nasal dorsum and bones (Fig. b). Sharp subperichondrial dissection with a scalpel or a blunt dissection with scissors freed the soft tissues above the upper lateral cartilage as in a standard open rhinoplasty. The dissection should be within the subperichondrium plane to prevent injury to the overlying musculature and blood vessels of the nose. Elevation extended laterally to the nasomaxillary sutures and superiorly to the glabella. Retraction of the freed soft tissues allowed sharp incision to be made with a scalpel or with sharp periosteal elevators through the periosteum at the inferior edge of the nasal bones. Elevation of the soft tissue laterally to the piriform aperture was also performed so that the maxillary vestibular dissection was easily connected to this pocket.\nAfter the connection of the nasal and oral incisions, the midface was degloved. The midface skin was separated from the maxilla and the nasal pyramid. The upper lip and the intact nasal columella, nasal tip, and alar cartilages were then retracted by two Penrose drains introduced through the nostrils over the nose to the level of the inferior orbital rim. This approach provided visualization of the medial maxillary wall, pterygoid junction, nasofrontal suture, infraorbital rim, and laterally to the temporal process of the zygoma (Fig. d). Under direct visualization, the overgrowing bone lesion was then excised using osteotomes and saws. The right maxilla was drilled further at the orbital rim and laterally till zygomatic complex. The contour of the midface was reestablished using burr to give a cosmetically normal looking midfacial skeletal contour while protecting the infraorbital nerve (Fig. ). For the removed lesion, a biopsy was performed for the accurate diagnosis and histologically confirmed as FD. The soft tissues were then carefully redraped and the nasal tip brought back into position. The intranasal incisions were closed using 4-0 resorbable sutures (vicryl), and the transfixion sutures were precisely performed to determine the final position of the nasal tip and prevent vestibular stenosis. The cinch suture of alar base was used to prevent postoperative alar base widening. The intraoral incisions were closed using a 3-0 black silk. Nasal packing into the maxillary dead space with Vaseline gauze was done for 3 days in order to minimize the postoperative bleedings.\nThe patient’s postoperative course was generally uneventful. There was moderate nasal crusting for 3–4 days. Mild swelling with periorbital ecchymosis disappeared after 2 weeks, and transient paresthesia around the infraorbital nerve spontaneously resolved after 3 month. No postoperative complications such as epistaxis, vestibular stenosis, or esthetic problems of the nose were seen. Clinical and radiographic examinations obtained 4 months after surgery showed the anatomical structures were in a stable state without recurrence of FD (Fig. ). The esthetic result was satisfactory for the patient, and occlusal state was also well maintained (Fig. ). Therefore, no additional orthodontic treatment or orthognathic surgery was performed.
An 80-year-old man was admitted to our hospital for the treatment of a rectal tumor found incidentally by rectal examination. The tumor, about 3 cm in diameter, was located on the right side of the lower rectum 3 cm above the anal verge. The pathological analysis of the biopsy sample revealed that the tumor was a moderately differentiated adenocarcinoma. Abdominal computed tomography (CT) and magnetic resonance imaging (MRI) indicated that the rectal cancer invaded into the muscularis propria without distant metastases and that lateral pelvic lymph node (LPLN) was not enlarged with a maximum long-axis diameter <3 mm. The most important problem was that the patient had a huge benign prostatic hypertrophy, the size of which was 85 × 80 × 70 mm (Fig. a–c). To achieve complete TME with negative CRM, a hybrid transabdominal-transanal approach for ISR was conducted.\nFirst, vascular division and mobilization of the left colon were performed laparoscopically. The transabdominal approach was continued until the anterior dissection of the rectum became difficult due to a huge prostatic hypertrophy. Next, the circumferential rectal incision and subsequent intersphincteric dissection were performed under direct vision to enable attachment of a single port device (GelPoint Mini; Applied Medical). After closure of the anal orifice, the GelPoint Mini was placed to start the transanal approach. Posterior side of the rectum was first dissected until the transanal approach was connected to the dissection layer made by the transabdominal approach. The dissection procedure was extended to the lateral side. Bilateral pelvic splanchnic nerves were identified at the 5 and 7 o’clock positions. At the anterior side, the proper dissection layer cannot be easily identified because of the perineal body and the enlarged prostate. Once the dissection plane between the rectum and the prostate could be identified, it was relatively easy to continue along the same plane. The assistance provided by the laparoscopic approach was useful to determine the appropriate dissection line in the transanal approach.
A 15-year-old female patient was referred to us for evaluation of uveitis treated in other clinics before. Child reported first symptoms 5 years ago, which consisted of blurry vision in right eye first then left eye and redness of eyes. She had oral aphtae and oral ulcers as well, that improved over time with treatment. She was treated earlier for right sided knee inflammation. She has been diagnosed with uveitis and for 5 years treated with on and off topical, peribulbar and systemic corticosteroids with periods of improvement and periods of worsening of symptoms. She developed cataract in both eyes and was planned for surgery two years ago, but parents refused surgical intervention. Two years prior to referral to our clinic she had laser fotocoagulation in both eyes due to retinal vasculitis and was started on Methotrexat 15 mg weekly. Despite the administration of local and systemic corticosteroids and MTX, inflammation persisted; therefore, the patient was referred to our clinic for second opinion.\nThe best corrected visual acuity (BCVA) was 0.9 in RE and 0.6 in LE, and the IOPs of the right and left eye were 17 and 18 mmHg, respectively. Anterior chamber cell grade of 0.5+ in the both eyes. She had posterior synechiae in both eyes and posterior cortical cataract in both eyes (). The vitreous cell grade was 3+ in the right eye and 2+ in the left eye. On ultrasound she had inflammatory reaction in vitreus, more prominent in right eye (). Posterior segment was hazy due to vitreal changes and cataract, but laser burns could be seen as well as signs of vasculitis and macular changes. We performed fluorescein angiography and SD OCT on patient to reveal intraretinal cysts on both eyes and perifoveolar oedema right eye ( and ).\nNon-ocular manifestations oral ulcers were improved with previous treatment. Skin or genital lesions were not observed. She was refered to pediatric rheumatologist for complete evaluation. All laboratory workup was within normal limits. Chest X ray and abdominal ultrasound were normal. Interferon-gamma release assay and Quanti Feron Tb gold test for tuberculosis infection and Treponema pallidum antibody hemagglutination test for syphilis were negative; Hepatitis HBs and anti HBc were negative as well as HCV Ag; angiotensin-converting enzyme, antinuclear antibody, and anti-citrullinated protein antibody levels were within the normal range. MRI of brain and cervical spine were normal, as well as EEG. Neurological examination was normal. She tested positive for HLA-B51. There was no family history of autoimmune diseases. Following treatment, initiated in the previous clinic, was continued: 0.1% dexamethasone eye drops (four times/day), tropicamide/phenylephrine eye drops (once/day), Methotrexat (MTX) 15 mg once a week followed by folic acid. The patient was diagnosed with the incomplete type of Behçet’s disease on the basis of the presence of a typical ocular symptoms and recurrent oral ulcers, retinal vasculitis recurred in both eyes in spite of treatment with MTX, therefore, initiation of adalimumab was proposed. Subcutaneous adalimumab injection was then introduced. The patient was 15-year-old and weighed 57 kg when adalimumab was initiated, thus we administered 40 mg every 2 weeks. After 2 months BCVA improved in both eyes, to 1.0 RE and 0.7 LE. The anterior chamber in both eyes improved to 0 , within 2 months after beginning adalimumab administration. Posterior synechiae and cataract did not change. Oral ulcers and problems with knee did not repeat after adalimumab therapy. The inflammation subsided. After three months she was taken off local medication. On the first follow up SD OCT showed significant improvement () as well as after 18 months.\nAfter 12 months with adalimumab we decided to lower MTX dosage to 10 mg once per week and she remained symptoms free with this lower dose. The follow-up period after introduction of adalimumab was 18 months (at the time this report was written) and the patient did not experience any side effects. Her ocular and oral symptoms of BD were well controlled.
A 49-year-old male patient came to our hospital with a tumorous growth located in the frontal region of his head, which developed over approximately 3 years prior to presentation. He had undergone an operation in another hospital about 2 years earlier but the lump had been growing rapidly over the preceding 6 months. Pathologic analysis revealed malignant schwannoma. On physical examination, we encountered two firm, noncompressible, nontender, nonpulsatile masses that measured approximately 6 × 5 cm and 8 × 8 cm in the frontal region with head CT scan reveal bone discontinuity due to bony destruction [].\nThere were no clinical signs suggesting neurofibromatosis and the family history was negative. The patient underwent microsurgical resection via a frontal craniotomy followed by plastic and reconstructive in a single surgery. The involved bone and a 2-cm margin of healthy tissue were excised together with the tumor mass. The involved bone was brittle and soft, so it rongeured until thick and healthy bone was encountered. There was no infiltration of the underlying dura mater and the lesion could be completely excised en bloc. Cranioplasty was required to close the resulting 4 × 4 cm calvarial defect. After excising the mass, the scalp defect measured approximately 15 × 12 cm and the reconstruction was completed using a myocutaneous flap with a muscle cuff along with the vascular pedicle [Figure and ]. The graft was taken from anterolateral thigh and femoral circumflexa artery – superficial temporal artery anastomosis was performed. The wound healed well and no surgical complications arose. Conventional radiotherapy of 50 gray was performed 2 months after the surgery. The final pathological diagnosis of MPNST was made based on examination of the surgical specimen. Light microscopy revealed that the tumor cells were monomorphic spindle-shaped cells with frequent mitotic figures []. Immunohistochemistry revealed positive immunoreactivity with S-100 and ki-67 protein [Figure and ]. During 1 year follow-up and 3 months interval MRI, no recurrence was seen.
A 65-year-old, both maxillary and mandibular partially edentulous, male was referred to the Department of Prosthetic Dentistry, Istanbul University, Faculty of Dentistry. The patient's chief complaints were reduced function and unaesthetic appearance because of missing teeth and the lack of retention and stability with his previous RPD.\nThe patient's medical history revealed that he had radiation therapy on the right sight of mandibular buccal shelf region at the head and neck area. At the extraoral examination, a scar tissue at the right buccal shelf area was observed due to the surgical tumor resection that was operated 25 years ago. In addition, loss of fibroelasticity of the right peripheral soft tissue and perioral region was discovered due to the radiotherapy while the left side indicated no abnormality []. When a dental anamnesis was taken, the patient has reported that three maxillary teeth with severe mobility were extracted before application to the Department of Prosthetic Dentistry Clinic. The patient had no existing dentures for both jaws. He had difficulty in usage of his previous dentures due to lack of retention. Intraoral examination revealed that the patient had two maxillary central and lateral teeth with moderate mobility and five mandibular teeth, right lateral to left canine with no mobility []. Reduced vestibular sulcus depth and a fibrous scar tissue were examined at the right buccal region. Radiographic examination using the existing panoramic X-ray showed that right maxillary canine and maxillary central incisor teeth were previously extracted. In addition, mandibular right first premolar with an existing periapical lesion was also decided to be extracted [].\nThree treatment options were presented to the patient.\nMaxillary and mandibular conventional clasp-retained RPDs were rejected because of the patient's previous complaint about lack of retention and stability with his previous dentures Maxillary and mandibular implant-supported fixed prostheses were rejected due to the financial limitations of the patient Maxillary complete denture and mandibular implant-supported RPDs were chosen by the patient as an optimal treatment with the advantage of the increased retention and stability provided by the implants for the mandibular RPD. The cons and pros for the extraction of maxillary left central and lateral teeth were explained in details to the patient in terms of biomechanics and esthetics. The patient preferred the extraction of the remaining teeth, and the future fabrication of a maxillary complete denture was planned. Written informed consent before surgical and prosthetic treatment was obtained from the patient.\nUnder local anesthesia, mucoperiosteal flaps were elevated and two implants (4.1 mm diameter, 10 mm length; Straumann AG, Waldenburg, Switzerland) were placed in posterior region of the mandible, both on the right and left sides []. Primary closure was obtained in both operation sites. Prophylactic antibiotics and nonsteroidal anti-inflammatory drugs were prescribed. Sutures were removed on the 7th postoperative day. At the end of 3 months, the osseointegration of the implants was checked on the panoramic radiograph []. The healing of the mandibular distal edentulous sites seemed to be normal and gingival formers were placed []. At this stage, two maxillary teeth were also extracted before the initiation of the prosthodontic therapy.\nPreliminary impressions were made using an alginate impression material (Italgin Chromatic Alginate, BMS Dental, Capannoli, Italy) and individual impression trays for both maxilla and mandible were fabricated using autopolymerizing acrylic resin. After border molding on the maxillary tray, the final impression was made using a zinc oxide eugenol material (SS White, C/O Prima Dental Group, Gloucester, England). For the mandible, the locator abutments (H 3 mm, coated Ti alloy, Straumann AG, Basel, Switzerland) were torqued to the implants with a 25 N/cm []. For the mandibular impression, impression copings were attached to the locator abutments. Cingulum rest seats were prepared on the mesial site of the mandibular right canine and between the mandibular first and second lateral incisors. After that, the final impression of the mandible was made using an addition silicone impression material (Dentasil A, DENTAC, Senden, Germany). Locator analogs were attached to the impression copings [] and the impression was poured [].\nRPD framework was designed on the mandibular cast with T-bar clasps on the terminal abutment teeth and a lingual plate as a major connector. The cingulum rests were fabricated on the rest seats []. The framework at the implant abutment region was designed circular around the abutment, and the distance between the abutment and the framework was approximately 2 mm so as to support the denture base acrylic resin []. The RPD was cast using a chromium-cobalt casting alloy (DFS, Ländenstrabe, Riedenburg, Germany).\nArtificial teeth (NT Optima, Toros Dental, Antalya, Turkey) setup was completed and tried in the mouth. The maxillary complete denture and mandibular RPDs were delivered to the patient Locator abutment matrix and black processing nylon insert were connected to the mandibular RPD using autopolymerizing acrylic resin (Self-cure acrylic, IMICRYL, Istanbul, Turkey). After polymerization, the denture was removed and the pink locator attachments were fitted []. After delivery, the patient was recalled weekly for 4 weeks []. The patient was satisfied with the function of his dentures as well as the improved esthetics [].