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A 13-year-old girl presented with recurrent abdominal pain for the last 3 months that appeared after menarche. The pain negatively affected her quality of life including a decrease in her school attendance, forcing her to go to the emergency room on several occasions. The physical examination revealed tenderness over the left ovarian point with no signs of peritoneal irritation. The patient was evaluated by a multidisciplinary team made up of pediatric specialists in gastroenterology, surgery, nephrology, and radiology. Laboratory tests, including complete blood count, serum biochemistry, urinary sediment, biochemical test for renal function, celiac disease serology, fecal parasites, and calprotectin, were normal. The abdominal X-ray was also normal. The abdominal ultrasound did not reveal any abnormalities including both kidneys; however, intestinal gas prevented a complete visualization of the uterus and ovaries. Finally, the computed tomography with intravenous contrast (\n) and magnetic resonance imaging (\n) showed a double LRV with a retroaortic component, prominent left parauterine circulation and engorgement of the ipsilateral ovarian vein with a caliber of 8 mm and a grade II reflux. These findings were consistent with pelvic venous congestion (PVC) secondary to a circumaortic LRV with a posterior nutcracker phenomenon.\nA diagnostic and therapeutic phlebography was programmed. This procedure was performed through the right basilic vein and confirmed the existence of a left circumaortic renal vein and a group of dilated pelvic varicose veins draining into the left ovarian and internal iliac veins (\n). An embolization at a distal level of these vessels was accomplished using 8 to 10 mm Hydrocoils under a 0.035” guide (\n). The complete occlusion of the pelvic varices and the treated venous segment was confirmed with a final angiographic control. There were no perioperative complications. The patient reported moderate pelvic pain, which responded promptly to parenteral analgesia and ceased during the postoperative day 3, remaining asymptomatic after 18 months of follow-up. Urinary tests were requested at 1, 3, 6, and 12 months in which result was normal.
A tumorous mass at the right lateral side of the abdomen of a 4-year-old boy was detected in April 2011. He was diagnosed as having right kidney tumor and was subsequently hospitalized in the Department of Pediatrics of the Hirosaki University Hospital. After pediatric surgeons performed a total resection of his right kidney and a histopathologic examination, he was diagnosed as having clear-cell renal cell carcinoma. After surgery, he was treated with chemotherapy, and his general condition gradually improved. However, he then exhibited general malaise and was diagnosed as having secondary AML in May 2012. Although chemotherapy treatment for the AML was once again successful, subsequent tumor lysis syndrome, DIC, intracerebral hemorrhage, and secondary renal and heart failures led to severe deterioration of his general condition.\nAn ultrasound examination performed by his pediatricians detected bilateral intraocular protrusions, and he was referred to our ophthalmology clinic. We were unable to measure his visual acuity at the first examination, as he had been sedated for general treatment. Subsequent fundus examinations did show intraocular protrusions, and he was diagnosed with bilateral subretinal or choroidal hemorrhages. A few weeks later, bilateral dense vitreous hemorrhages occurred, his fundi became totally obscured, and he became blind. Electroretinograms (ERG) were extinguished bilaterally, and A/B-mode ultrasonographies revealed bilateral combined vitreous hemorrhage and hemorrhagic retinal detachment (fig. ).\nAfter intensive pediatric treatment, his general condition improved to the point where he was well enough to receive transplantation of umbilical cord blood stem cells in September 2012. Transplanted cord blood stem cells were successfully engrafted, and his general condition continued to improve. Because both his parents and the pediatricians wanted to treat his blindness with surgery, we agreed to perform vitrectomy associated with lens extraction and intraocular lens (IOL) implantation in his left eye. The left eye was chosen for the surgery as it still demonstrated a sluggish direct light reflex, while his right eye had completely lost the direct light reflex.\nAlthough his visual acuity showed no light perception bilaterally, preoperative ocular examinations revealed that a sluggish direct light reflex remained in his left eye and that bilateral dense massive vitreous hemorrhages could still be observed (fig. ). However, ultrasonographic examinations showed that the previously detected hemorrhagic retinal detachments (fig. ) were no longer observable.\nIn December 2012, the patient underwent 25-gauge pars plana vitrectomy combined with phacoemulsification and aspiration (PEA). We first prepared surgical ports for the 25-gauge vitrectomy and then uneventfully performed PEA before starting the vitrectomy procedure. After removal of the dense vitreous hemorrhage, we observed that the peripheral retinal color was mottled, similar to that which is seen after the complete absorption of a subretinal hemorrhage. Surprisingly, with the exception of numerous fine retinal folds, the retinal color in the posterior pole appeared almost normal, and the optic disc was not pale. Because we detected a putative perforating retinal hole in the mid-peripheral portion, laser photocoagulation was applied around the lesion. Next, an IOL was implanted in the bag, and a posterior capsulotomy was performed. We then closed all surgical incisions. No surgical complications occurred during or after the operation. Although the postoperative visual acuity remained at light perception for 3 months, it gradually started to improve 4 months postoperatively. The condition of the fundus appeared to be almost the same as that seen at the end of surgery (fig. ). An optical coherence tomography examination of the left eye indicated that while the shape of the central fovea was normal, the external limiting membrane was absent, and the inner segment ellipsoid line was extremely thin and not continuous under the fovea (fig. ). In September 2013, the corrected visual acuity in his left eye improved to 0.1.
A 49-year-old man, who had a left brachial plexus avulsion injury 10 years before, experienced severe neuropathic pain in his left upper limb immediately after the trauma. The patient complained of continuous burning, pressing, and tingling pain in the upper limb. From the beginning of the perception of the pain in his upper limb, he felt illusory perceptions of fingers touching his face although he did not perceive pain or any other sensory deficits in the face. He had been treated several times for the pain through left brachial plexus blockades and cervical epidural blockades, with no success. His neuropathic pain decreased slightly when taking pregabalin and with the application of cervical spinal cord stimulation (SCS), but it remained severe. He did not have any pain or trigger areas in the face getting caries of the teeth. He once underwent a dental treatment for his left mandibular molar tooth. When local anesthesia was applied around the left mandibular molar tooth (3 mL, 0.5% lidocaine), he felt the enlargement of that region, which was followed by an immediate disappearance of his neuropathic pain. At that time, the illusory finger sensations in the face disappeared. Approximately 2 hours after the dental treatment, the neuropathic pain returned and gradually increased to predental treatment levels. A nonsteroidal anti-inflammatory drug, loxoprofen, completely ameliorated the dental pain but was not effective against the neuropathic pain. Since then, the patient had 3 dental treatments, and local anesthesia around the left molar tooth consistently ameliorated his neuropathic pain. Analgesic effects consistently lasted for several hours following the administration of the local anesthesia. His neuropathic pain was able to be mildly controlled by a combination of pregabalin, SCS, and local anesthesia around the left molar tooth although the molar tooth had completely improved. The use of oral local anesthesia for breakthrough neuropathic pain had been especially effective.\nWe obtained the patient's consent to report his progress, in accordance with the Declaration of Helsinki.
A 3-year-old boy admitted to the University Children’s Hospital of Krakow following the first epileptic seizure. The patient complained of headaches and vomiting. Based on clinical presentation and medical history, intracranial hypertension was suspected. CT scan demonstrated bilateral fluid-filled spaces in the middle cranial fossa (the larger being situated on the left side), with hypotrophy of the adjacent cerebral structures. In addition, CT showed a minimal mass effect manifested as displacement of the longitudinal fissure of the brain to the right and compression and stenosis of the left lateral cerebral ventricle, as well as mild thinning of the squama of the temporal bone at the level of the malformation (Figs. and ).\nIn differential diagnosis, consideration was given to the open type of bilateral schizencephaly or bilateral arachnoid cysts of the lateral sulcus, with type III on the left and type II on the right side (Galassi classification).\nThe CT scan favored the arachnoid cyst (the mass effect, discrete thinning of the squama of the temporal bone), yet schizencephaly could not have been ruled out based on a single CT scan and medical history of the patient. The scan was performed as an emergency procedure in a patient with severe and growing in intensity symptoms of intracranial hypertension, what did not allow for extensive, time-consuming diagnostic management.\nA decision was made on a surgical intervention consisting in implantation of a cysto-peritoneal shunt on the left side. Following shunting, the patient developed complications consisting in small intracerebral and paracerebral hematomas (Figs. and ).\nIn subsequent follow-up scans, gradual resorption of hemorrhagic lesions was seen. The fluid-filled spaces were still present; albeit markedly smaller as compared to the initial scan, discrete asymmetry of the lateral ventricles persisted, but no displacement of the lateral fissure of the brain was detected. The patient improved clinically (Fig. ).\nFollowing implantation of a drain system and clinical improvement of the patient, a MRI scan of the head was performed, which ultimately confirmed that the fluid-filled spaces in the two middle cranial fossae corresponded to arachnoid cysts.
A 9-year-old male patient with severe intellectual impairment and limited mobility was referred to local cardiology services with a soft ejection systolic murmur. The electrocardiogram showed an incomplete right bundle branch block with QRS duration of 95 ms and right axis deviation. Chest X-ray results showed mild cardiomegaly. The patient was diagnosed with a large secASD with evidence of right ventricular volume overload based on a focused TTE. Foetal alcohol syndrome was determined as the most likely overriding aetiology for his cardiac abnormality and co-morbidities. The patient resided in the South Pacific islands and was referred to the regional overseas paediatric cardiac centre for transcatheter secASD closure.\nA full cardiac anatomy scan was completed using TTE at the cardiac centre. A diagnosis of secASD suitable for transcatheter device closure was confirmed. The patient had normal left ventricular dimensions and ejection fraction with no evidence of regional wall motion abnormalities. The right ventricle was severely dilated and had normal systolic function. Trivial mitral regurgitation was present. The patient had a severely dilated RCA origin from the right aortic sinus that measured 4.6 mm with a Boston Z-score of +4.65 ( and Video 1). There was no evidence of a left main coronary artery (LMCA) originating from the left aortic sinus ( and Video 2). On further interrogation using colour Doppler, only retrograde flow was noted in the region of the proximal LAD ( and Video 3). The LCX was not observed.\nThe coronary artery origins were further assessed using transoesophageal echocardiography (TOE) due to suspicion of a coronary artery abnormality. This was undertaken as part of the secASD imaging pre-intervention. An aortic angiogram was chosen as the next step if a coronary artery anomaly could not be ruled out. The TOE was unable to identify an LMCA and demonstrated abnormal coronary artery colour Doppler signal near the left atrial appendage ( and ).\nAn aortic root angiogram showed a single dilated RCA originating from the right aortic sinus, supplying the inferoseptal and inferior myocardium and retrogradely filling the LCX back to the expected position of the LMCA. There was no LMCA originating from the aorta ( and ).\nA selective RCA angiogram was subsequently performed to further define coronary supply and confirmed the above findings ( and ). There was adequate retrograde filling of the LCX as far as the LAD and LCX bifurcation. No LMCA was evident. The LAD was antegradely filled from the LCX and retrogradely by a large collateral artery from an RCA ventricular branch that extended to the cardiac apex. The middle third of the LAD was diffusely hypoplastic.\nA dobutamine stress echocardiogram (DSE) was immediately performed to determine the potential need for surgical coronary artery intervention. DSE analysis confirmed good left ventricular function with no evidence of regional wall motion abnormalities.\nThe transcatheter secASD closure was deferred pending cardio-surgical evaluation of the coronary artery findings. The overall appearance was judged to be a congenital coronary artery anomaly, but the diffusely hypoplastic LAD raised the question of an inflammatory, and thus acquired aetiology.\nIt was decided that the risks of surgical coronary artery intervention to bypass graft or re-anastomose the left coronary arteries to the aortic root outweighed the benefits. Contributing factors to this decision included no inducible ischaemia or wall motion abnormalities, adequate collateral and retrograde supply, distance from the aortic root to the reconstitution of the LAD and LCX, and the small calibre of both the LAD and obtuse marginal branch. An interval transcatheter secASD closure was successfully performed using a 24-mm Figulla Flex II ASD occluder (Occlutech GmbH, Jena, Germany), and the patient then returned to his country of origin. A follow-up stress echocardiogram will be performed in adolescence.
A 25 year-old primigravida patient was referred from a nearby health center to HEAL Africa Hospital, a multidisciplinary tertiary hospital for management of an acute abdomen at 33-weeks gestation.\nHer chief complaint was severe abdominal pain associated with each fetal movement for a period of 1 week prior to admission at the referring health center. Among other undocumented treatments, the patient had been managed with spasmolytics and hematinics but without relief of her pain. On August 30, 2019, when the patient’s condition worsened, the decision was made to transfer her for further care. She reported to the emergency unit of the hospital on the same date at 21h30.\nHer first and second trimesters had been uncomplicated. She had received antenatal care in a nearby clinic where she was treated with antihelmintics, iron and folic acid supplementation, as well as prophylactic anti-malarials, according to recommended standards. The patient had not had an obstetrical ultrasound. There was no history of any symptoms suggestive of sexually transmitted diseases such as vaginal discharge or genital ulcers.\nabout the duration of her cycle, she typically bled for 3 days per cycle and denied pain or passage of clots during menstruation. Dates of her last normal menstrual period were unknown.\nPast medical history was notable for malaria with two prior admissions to a nearby clinic. There was no history of chronic illnesses such as hypertension, diabetes mellitus, asthma or sickle cell disease, and the patient did not take any medications on an ongoing basis. She had never been tested for HIV.\nShe had never undergone myomectomy or any other surgical procedures and did not have a history of blood transfusion. There was no history of involvement in road traffic or other accidents.\nOn social history, the patient was the third born in a family of five children. Her parents and siblings were alive and healthy. There was no family history of chronic illnesses. The patient was a married housewife and did not smoke cigarettes or drink alcohol.\nIn summary, this was a healthy 25-year old primigravida who was admitted with a one-week history of severe abdominal pain associated with fetal movements at 33- weeks gestation in an otherwise uncomplicated pregnancy.\nOn physical examination, the patient was noted to be ill-appearing. Vitals signs were as follows: heart rate of 99 beats per minute, blood pressure of 120/69, respiratory rate was 22 breaths per minute, oxygen saturation of 98% on room air and temperature of 36.90 C.\nThe patient’s pulse was regular and of normal volume. Apex beat was noted in the 5th intercostal space with normal S1 and S2 on auscultation. Chest expansion was symmetrical and breath sounds were normal with bilateral good air entry.\nThe abdomen was symmetrical but tense without surgical scars. Striae gravidarum and a linea nigra were visible. There was marked tenderness on abdominal palpation, particularly in the peri-umbilical area and associated with each fetal movement. Palpation of the liver, spleen, and kidneys was limited due to the patient’s tenderness. Fundal height was not well delineated but was estimated at 28/40 weeks. There were no palpable contractions but marked abdominal tenderness was noted during fetal movement. Fetal parts were not easily palpable through the abdominal wall. Additionally, fetal presentation and fetal lie were not easily appreciated on physical exam. A regular fetal heart of 148 beats per minute was auscultated in the mesogastrium.\nExamination of the vulva and vagina were normal. The cervix was long, posterior, and not excitable. The os was closed. No abnormal discharge was noted.\nDiagnosis of an acute abdomen in the third trimester of pregnancy was made and acute peritonitis was suspected. Differential diagnosis included appendicular or other bowel perforation.\nThe patient was admitted to hospital. An emergency ultrasound showed a single viable pregnancy at 33-weeks gestation with a low-lying placenta and oligoamnios. Initial hemoglobin was 8.1 g/dl with a hematocrit of 22.6%.\nAfter intravenous access was obtained, intravenous fluids and initial pain management were started. The patient was counselled and consented for an emergency laparotomy.\nShe was taken to operating theatre and was given general anesthesia with endotracheal intubation. Both an obstetrician and a general surgeon scrubbed for the case. A sub-umbilical incision was made and then extended above the umbilicus. On entering the abdominal cavity, a huge reddish mass was identified. Fetal parts were visible through the membranes delineating the mass. There was minimal meconium stained amniotic fluid around the baby. Upon digital opening of the mass, a live 2000 g female baby in longitudinal lie with the head in the maternal pelvis was delivered [Fig. ]. APGAR scores were 8, 6, and 9 at 1, 5 and 10 min respectively. The baby was immediately taken to neonatology for thorough screening by the paediatrics team and was found to be healthy with no congenital abnormalities. Careful exploration of the abdomen revealed a placenta implanted on the greater omentum and on the small bowel mesentery [Fig. ]. There was no plane of cleavage and any manoeuver to remove the placenta was susceptible to bleeding. It was decided to leave the placenta in place. Membranes were stripped and the umbilical cord was cut near its placental insertion. The patient remained hemodynamically stable throughout the surgery and no complications were noted. However, she did receive one unit of whole blood transfusion.\nThe patient was admitted to the post-partum ward after she was fully awake and was started on parenteral antibiotics and analgesics for 3 days, after which she was transitioned to oral treatment. Patient was also started on hematinics. Weekly ultrasounds were planned as well as serial quantitative beta-HCG measurements to evaluate the status of the placenta.\nThe patient remained stable in the post-operative period. Her first post-partum ultrasound showed an intra-abdominal placenta in the hypogastric region extending to the left and right iliac fossas with evidence of vascularisation on Doppler. Partial placental detachment was noted with two pouches of encapsulated peri-placental hematoma having maximal diameters of 9.39 cm and 6.78 cm each. The anteverted, anteflexed and empty uterus was well visualized and the serum beta – HCG was > 1500 IU/ml.\nIn the second post-operative week, the patient did not have any major complaints. Ultrasound again demonstrated an intra-abdominal placenta in the hypogastrium, above the uterus, with an encapsulated peri-placental hematoma of 8.41 cm in the longest diameter. The uterus remained empty, anteverted, anteflexed. Serum beta–HCG returned at 653.9 IU/ml.\nThe patient continued to make a good recovery and the baby appeared to be healthy. The mother was discharged home on prophylactic antibiotics for 1 week with a planned follow up at the end of post-partum week 6.
A 36-year-old woman was hospitalized at a local hospital for pelvic mass found by physical examination in November 2017. Exploratory laparotomy was performed at that hospital immediately. Intraoperatively, a free mass at approximately 6 cm × 5 cm × 5 cm in size was observed at the bottom of the right pelvis and adjacent to the uterosacral ligament without any other abnormalities. Due to the examination of frozen sections showing germ cell tumor and consultation with relatives of this patient, pelvic mass resection, biopsy of bilateral ovaries and partial omentum resection were performed. Postoperative pathology reported yolk sac tumor of the pelvic mass, normal biopsy from ovaries and normal tissue of omentum. From December 2017 to June 2018, six courses of chemotherapy with BEP regimen were administrated for her. After five cycles of chemotherapy, serum tumor markers of the patient were all normal, but her alanine aminotransferase (ALT) was elevated nearly to 600 IU/L. She was treated with hepatoprotective therapy for 20 days before completing the sixth cycle chemotherapy. Unfortunately, the serum AFP increased again after sixth cycle of chemotherapy.\nDue to increase of serum AFP, the patient was admitted to our center in July 2018. Positron emission tomography–computed tomography (PET/CT) demonstrated a round-like mass at the right pelvis accompanied by increased [18F]-fluorodeoxyglucose (FDG) metabolism. CT scan also indicated a 2-cm mass at the right pelvis (\n). Considering relapse of the disease, the patient was hospitalized at our department for a second surgery. During the operation, a dumbbell-shaped, hard, and smooth mass at approximately 3 × 2 × 1.5 cm in size was found at the posterior lobe of right broad ligament and near the uterus. No abnormalities were observed at any other sites of the pelvic and abdominal cavity. After mass resection and right adnexectomy, frozen section was examined and demonstrated yolk sac tumor of broad ligament and normal right adnexa. In consideration of normal adnexa and consultation with her relatives, no other resection was conducted.\nPostoperatively, pathology report confirmed yolk sac tumor of broad ligament again and normal adnexa. Immunohistochemical staining yielded the following positive results: SALL4 +++, AFP ++, GPC-3 ++, AE1/AE3 +, Ki-67 positivity rate of approximately 70%, patchy positivity for CD117 and VIM+/−. Immunohistochemistry yielded negative results for CK7, CD30, PAX-8, and PLAP (\n). Considering diagnosis with yolk sac tumor, four courses of chemotherapy with BEP regimen were administrated for this patient from August 2018 to November 2018. After completing three cycles of chemotherapy, the serum AFP was 15.39 ng/ml. During the period of chemotherapy, adverse events were not serious which included decreased white blood cells and neutrophils. CT and magnetic resonance image (MRI) scan showed thickening of the area of the right adnexa not excluding relapse. She was discharged after four courses of chemotherapy and the follow-up failed later.
A 43 year-old woman was admitted to hospital with headache of two months' duration and left-sided pulsatile tinnitus that occurred suddenly one month ago and had become severe. She complained of a constant rhythmic sound that beat in synchrony with her heartbeat. The sound became louder in her ear, making it difficult to sleep. On admission, the patient did not have any otologic symptoms such as otorrhea, dizziness and hearing loss except ear-fullness in the affected ear. She had no previous history of surgery, head and neck trauma, or systemic diseases. Based on the results of a questionnaire in which the answers were measured on a 10-point visual analog scale (VAS), her intensity of tinnitus was 7, tinnitus-induced annoyance was 8 and the effects of the tinnitus on her life was 6, showing a high level of discomfort. The tinnitus handicap inventory (THI) was also high, as much as 76. The physical examination revealed no mass in the head and neck, and the otoscopy exhibited no abnormal characteristics of the tympanic membranes. No sound waves were detected by auscultation. The tinnitus sound became weaker when the patient turned her head to the left and when her neck was pressed with the hand. The result of the pure tone audiometric evaluation was 11 dB in the right ear and 18 dB in the left ear. The impedance audiometry produced a Type A curve for both ears. In the tinnitogram, the patient showed pulsatile tinnitus in the left ear. And the sound pressure was 24 dB over the frequency of 1000 Hz. The results of the auditory brainstem response test and distortion product otoacoustic emission test were normal.\nBased on the patient's condition and test results, pulsatile tinnitus was suspected, and CTA was performed as a screening test. In the CTA, a prominent vascular structure was observed along the left tentorial margin, and focal stenosis at the transverse-sigmoid junction was present. Distension of the left occipital artery was also observed (). The CTA findings suggested the possibility of a DAVF, and a 4-vessel CA was performed for the diagnosis.\nAngiography of the left internal carotid artery revealed no abnormal findings in the arterial phase. However, the left external carotid angiogram revealed an enlarged occipital artery. Also, the blood drained to the left transverse sinus and the meningeal vein passing through the transcranial route, extending from the left occipital artery, middle meningeal artery, posterior auricular artery, and superficial temporal artery. The diagnosis of DAVF, fed by the left external carotid artery (ECA), and draining into the left transverse sinus, was therefore made (). Although excessive drainage into the cerebral cortex or brainstem was not observed, transarterial embolization was performed due to the severity of the tinnitus symptoms and the high level of discomfort. First, a 6-Fr guiding catheter was placed through the left ECA to identify the enlarged occipital artery for catheterization. Embolizations with ethylene-vinyl alcohol copolymers (ONYX) were performed to embolize the four feeding arteries. Embolization of the middle meningeal artery was performed to embolize the fifth feeder, followed by transcranial arterial embolization in the proximal portion of the superficial temporal artery. A total of six feeding arteries were embolized. Post-embolization angiography revealed blood flow in fine arteries branching off from the posterior auricular artery and occipital artery. However, the feeders were too small and numerous to perform embolization on. During embolization, the pulsatile tinnitus disappeared, and no post-embolization complications were found. During the outpatient follow-up, the patient did not show tinnitus symptoms but claimed minor fullness in the ear. A THI questionnaire was conducted 3 months postoperatively, and the score for tinnitus-induced discomfort was 0. However, the VAS score for annoyance associated with ear-fullness on the affected side was 2.
A seventy one year old Caucasian female presented to the breast clinic with a painless growth on her right nipple of six weeks duration. She gave no history of itchiness, discharge or bleeding from the growth. She was otherwise asymptomatic regarding her breasts.\nShe had previously had no breast problems and had no family history of breast or ovarian carcinoma. She was nulliparous and a teetotaller.\nThe only medical history of note was severe osteoarthritis of her joints including her shoulder joints.\nClinical findings were threefold.\nShe had a polypoid hemispherical growth on her right nipple measuring 40 by 25 mm. This appeared extremely vascular with a necrotic slough covered surface (Figures &).\nShe was also found to have a clinically suspicious firm lump measuring 25 by 25 mm in the lower outer quadrant at 8 o'clock position of her right breast 5 cm from the nipple.\nThere were no palpable masses in her left breast nor palpable lymph nodes in her right axilla. In her left axilla was an enlarged firm lymph node 20 by 20 mm clinically equivocal.\nShe had bilateral mammography, ultrasound scan of the lower outer quadrant of her right breast and both axilla. Right breast mammography showed a 30 mm radiologically malignant lesion in the lower outer quadrant of her right breast. The left breast showed no suspicious features. Ultrasound scan of the right axilla was normal. Ultrasound scan of her left axilla showed an enlarged lymph node with poor corticomedullary differentiation; radiologically indeterminate.\nShe had a clinical trucut biopsy of the lump in her right breast and an ultrasound guided trucut biopsy of the indeterminate lymph node in her left axilla. Trucut biopsy of the lump in the right breast lower outer quadrant showed ductal carcinoma in situ. Trucut biopsy of the radiologically indeterminate lymph node in her left axilla showed reactive changes only.\nShe went on to have right mastectomy with axillary node sampling in the form of sentinel node biopsy.\nThe lump in in the lower outer quadrant of the breast was found to be predominantly ductal carcinoma in situ of intermediate and high grade of cribriform and apocrine type. Associated with the ductal carcinoma in situ were two small areas of invasive adenocarcinoma. These were grade 3 carcinomas with no lymphovascular invasion, negative oestrogen receptors and Her 2 status negative.\nThe proximal ducts approaching the nipple were unremarkable. The nipple was entirely replaced by the polypoid lesion. There was too much cytological atypia to consider this as a nipple adenoma. Purely on the basis of cytology and architecture, the appearances were suggestive of ductal carcinoma in situ. There was no evidence of an invasive lesion in this area. The typical features of Paget's disease of the nipple (intra epithelial tumour cells) were not seen. Further immunohistochemistry was performed on the nipple lesion. The tumour cells were negative for oestrogen receptors. Only an occasional smooth muscle cell was demonstrable within the lesion. The appearances therefore argued against a primary lesion of breast epithelium and suggested instead a skin adnexal tumour i.e. apocrine carcinoma.\nTwo lymph nodes were sampled which were free of tumour.
A 50-year-old man with gradually worsening anemia and suspected small bowel bleeding was referred to our hospital to undergo CE because esophagogastroduodenoscopy and total colonoscopy did not reveal the source of the gastrointestinal bleeding. His oral medications included several psychoactive drugs and iron preparations but not nonsteroidal anti-inflammatory drugs. He had no history of abdominal surgery. We did not perform patency examination before CE because we assessed low possibility of severe stenosis and inflammatory bowel disease based upon his clinical history and abdominal X-ray examination. CE (PillCam SB2 system, Given Imaging, Yokneam, Israel) demonstrated several ulcers at the small intestine, but the capsule did not reach the cecum during the recording time (stomach transit time: 220 minutes). These results were sent to his primary care doctor. Five months after the CE examination, he was referred again for the recurrence of anemia. Abdominal X-ray examination revealed that the capsule was retained at the pelvic cavity (). Follow-up abdominal X-ray examination after 7 days demonstrated that the capsule remained in exactly the same part but was rotatable (). Computed tomography (CT) showed that the capsule seemed to be floating in the small intestinal lumen with dilatation and fluid collection proximal to the capsule (). Additionally, the CT coronal view indicated findings suspicious for stenosis distal to the capsule (). The patient had had regular bowel movements and no abdominal complaints for the past 5 months. He could not confirm that the capsule was egested because he failed to monitor his stools. After proper informed consent was obtained, we retrieved the capsule by laparoscopy-assisted surgery. The patient declined endoscopic approach and treatments. Laparoscopic instruments were placed through 3 trocars. The CE was laparoscopically detected approximately 50 cm from the end of the ileum. After fluoroscopic confirmation, the part of the ileum with the capsule was moved outside the abdominal cavity, and we made the following observations around the area of retention: the fat-wrapping sign and a caliber change were observed distal to the capsule, with reddened serosa proximal to the capsule. The capsule could not pass through this stricture (). The small intestine was extensively evaluated, and no other abnormalities were found. We resected approximately 30 cm of ileum and performed a functional end-to-end anastomosis. An ulcer scar with stricture was macroscopically observed, and a wide ulcer was observed at the proximal side of the lesion (). On histopathological examination, the lesions were determined to be a nonspecific ulcer (Ul III) with fibrosis, formation of lymphoid follicles, and infiltration of monocytes and neutrophils to the subserosa, without evidence of malignancy, inflammatory bowel disease, or tuberculosis (). The patient's postoperative course was uneventful, and he was discharged on the postoperative day 8. There was no evidence of progression of anemia at a follow-up visit conducted 7 months after surgery.
A 29-year-old man from China had been suffering from bilateral ankle pain for approximately 3 years. He did not have trauma or a relevant past history. Symptoms had disappeared eventually but had later recurred approximately 1 year ago. He visited a nearby hospital for treatment, but the doctor had difficulty diagnosing and treating his condition. The patient visited our hospital for consultation and, subsequently, he was admitted.\nOn physical examination, the patient's foot and gait appeared normal. He experienced pain on initial walking or walking for prolonged periods. No swelling, redness, or local heat was detected on his both ankles, but dorsiflexion of his right ankle was limited. No instability was observed in both ankles, but the pain was noted during internal rotation of his right ankle.\nRadiograph of the ankle under weight-bearing revealed that the joint spaces had decreased in weight-bearing areas of both the ankles and that osteophytes had formed mostly in the anterior site of the joints. Moreover, cystic areas were observed in these weight-bearing areas of the tibia, but no pathological changes around the epiphyses plates were observed (). No deformities or narrow joint spaces were observed in radiographs of other joints such as fingers, wrists, knees, or hips. Magnetic resonance imaging (MRI) revealed many cystic lesions in both the tibia and talus (). Other inflammatory diseases such as rheumatoid arthritis or infection were ruled out through blood examination results. The patient's condition was diagnosed as KBD because of the area from which he originated, X-ray and MRI findings, and the exclusion of other inflammatory diseases.\nAlthough the patient received palliative treatments such as nonsteroidal anti-inflammatory drugs, steroid injection, and rehabilitation, his right ankle pain persisted. Therefore, we then provided an arthroscopic ankle arthrodesis. A medial portal was first made and peeled cartilage of both tibia and talus was observed when we looked into the joint by a scope; otherwise the color of the cartilage was normal (). The anterior cartilage was worn and eburnated. Then, a lateral portal was made, and remaining cartilage and anterior osteophytes were removed using a punch or shaver (). Dimples were made on the tibial and talar subchondral bone, and bleeding from these dimples was confirmed (). The remaining cartilage was removed and more of such dimples were created to completely fuse the tibia and talus (). After confirming that all the subchondral bone was removed, the ankle was fixed using three cannulated titanium screws (Stryker Orthopaedics, Mahwah, New Jersey). These screws were inserted from the medial condyle of the distal tibia to the lateral process of the talus, talar neck, and the back of the talus, respectively. The ankle was fixed in the neutral position. After the operation, the ankle was placed in a cast for 4 weeks. Two weeks after the operation, the patient was permitted to bear his weight. At one year after the operation, radiographs revealed adequate bone union (), and the patient was asymptomatic for pain at his right ankle.
Our patient is a 19-year-old girl, with a long surgical history of multiple complicated abdominal surgeries. She was diagnosed with Primary Hyperoxalosis for which she underwent same setting liver and kidney transplant. A few years afterward, she was diagnosed with intestinal type diffuse large B-cell lymphoma. After that, she had an ileocecal mass that obstructs the bowel lumen and required oncological resection with right hemicolectomy and anastomosis which was further complicated by anastomotic leakage, wound dehiscence and intra-abdominal sepsis thus requiring emergency exploratory laparotomy and Hartmann's procedure with end ileostomy. The patient then underwent laparotomy, Hartmann's revision and ileostomy site closure. For the purpose of graft preservation, the patient was on long-term immunosuppressants.\nAfter more than a year from the last surgery, she developed a central abdominal bulge at the site of her laparotomy scar; she had midline incisional hernia. Due to the long history of laparotomies and multiple surgical procedures, a multidisciplinary approach was necessary, and a surgical treatment plan was set.\nAn enhanced CT scan was performed and showed thinning of the anterior abdominal wall muscles with severe atrophy of the left rectus abdominis muscle with rectus diastasis of around 10 cm distance (Figure ).\nThe injection was performed by the interventional radiologist with the patient in supine position under ultrasound guidance (Figure ). Injection sites were marked at the anterior axillary line between the costal margin and anterior superior iliac spine according to the technique described by Smoot et al. The area was prepped and draped in a sterile technique which was followed by application of local anesthesia in the form of 1% lidocaine at the skin of injection sites. Under ultrasound guidance, BTA was injected at the three sites on either side of the abdomen. The patient received a total of 300 units of BTA diluted in 150 mL of 0.9% saline with a concentration of 2 units/mL. Each of the six injection sites received a volume of 25 mL. Each of the three injection sites on either side of abdomen were used to target the external oblique, internal oblique, and transversus abdominis muscles. After the procedure, the patient recovered smoothly and was discharged home the next day to return for surgery after 3 weeks.\nAbove the iliac crest, transversus abdominis at anterior axillary line. Mid abdomen, internal oblique at mid axillary line. Below costal margin, external oblique at anterior axillary line.\nPatient went for surgery 3 weeks after Botox injections. The procedure started by infiltrating normal saline for subcutaneous hydro-dissection followed by removing the old scar at the midline. De-epithelization was continued just beneath the skin to raise it above the adherent bowel underneath until reaching the normal fascia on both sides of the abdomen. Then, the abdominal flap was raised in the subscarpal plane above the fascia. Closure of the defect and plication of the recti were done followed by placement of sized on-lay fully resorbable monofilament mesh (Figure ).\nTo further relax the lateral abdominal muscles, a total of 200 units of BTA diluted in 8 mL of normal saline were infiltrated at the same previously injected sites (Figure ). Drains were placed bilaterally above the mesh. Subcutaneous tissue was approximated with interrupted sutures in two layers. Skin was closed with 4-0 Monocryl in subcuticular fashion (Figure ). Patient had no complications and was followed up for the next 18 months with no recurrence.
A 76 year old woman underwent mastectomy for a primary breast malignancy, shown histologically to be a low grade ductal adenocarcinoma (stage T1 N0). Three months after surgery, she complained of visual deterioration in her right eye. A lesion was identified on fundoscopy by the treating oncologists, and a presumptive diagnosis of choroidal metastasis from the breast malignancy was made without ophthalmic consultation. Palliative external beam radiotherapy (EBRT) (20 Gy total) was administered to the right orbit in five daily fractions. The patient was kept under regular review by her oncologist and remained stable with no enlargement of the lesion reported on serial magnetic resonance imaging.\nEighteen months after radiotherapy the patient was referred to our ophthalmic service due to failing vision in the right eye. Corrected visual acuity was 6/18 in the affected eye. Dilated examination using a slit lamp revealed an 11 × 10 mm elevated choroidal mass in the peripheral fundus, mainly yellow in color with some intrinsic pigmentation (Figure ) and with no associated sub-retinal fluid. B-scan ultrasonography showed a mushroom shaped lesion, choroidal excavation due to extension through Bruch's membrane and low internal reflectivity (Figure ). Fluorescein angiography demonstrated a "double circulation" (Figure ) with intrinsic vasculature seen within the tumour, and the larger normal retinal vessels seen more superficially. Examination of the left eye was unremarkable.\nA revised diagnosis was made of a primary choroidal melanoma, partially treated by radiotherapy, in the presence of a concomitant primary breast malignancy. Magnetic resonance imaging of the brain, chest radiographs and liver function tests demonstrated no evidence of metastatic disease. The patient declined further intervention initially and conservative management was initiated.\nThree years later, growth of the lesion was observed and the patient was referred to a regional ocular oncology service. Enucleation was performed, over four years after the initial observation of the ocular lesion, confirming the diagnosis of choroidal melanoma. To date, five years since initial detection of the lesion, the patient remains well with no evidence of metastatic melanoma.
A 50-year-old lady was referred to us as a case of duodenal cancer with gastric outlet obstruction. She presented with history of persistent vomiting for the last two months, which was bilious and increased in the severity over last few days. This vomiting was aggravated by oral intake and associated with abdominal pain. There is no distention nether change in the bowel habits. She does not have a significant past medical or surgical history.\nOn examination, she looked conscious, oriented, and vitally stable. Abdomen was soft, lax and not tender with normal bowel sounds. The initial blood work shows elevated white blood cells, low hemoglobin, and high platelets. Liver function test showed that alkaline phosphatase was 243 IU/L, Gamma-glutamyl transpeptidase was 296 IU/L, and amylase was 32 IU/L. Other lab analyses were within normal range.\nThe patient underwent upper gastrointestinal (GI) endoscopy which showed an ulcer in the gastric area and the duodenal area. Multiple biopsies taken from duodenum showed poorly differentiated adenocarcinoma. Computed tomography (CT) scan was carried out and showed mild wall thickening of the duodenum with narrow lumen about middle long segment of the 2nd part. Also a right axillary ill-defined mass was found with infiltration of the adjacent fat and no enlarged lymph nodes in the pelvis or the abdomen (). For that, a mammogram was done, which showed predominant fatty involvement, which was uncertain.\nShe had a Positron emission tomography scan as well, which showed two foci of increased activity involving the C-loop of the duodenum suggestive of duodenal carcinoma with hyperactive nodal disease noted in the right axillary region. Ultrasound guided biopsy was taken from right axillary lymph node and showed poorly differentiated carcinoma with positive estrogen receptor (ER). Immunohistochemistry study also done for the previous biopsies from the duodenum showed strong positivity for ER and positivity for human epidermal growth factor receptor 2 (HER2) (Figures and ). The overall pathological findings were consistent with poorly differentiated adenocarcinoma of primary breast origin, most likely invasive ductal carcinoma.\nThe patient underwent tumor resection and gastrojejunostomy anastomosis. After recovery from surgery, she was started on systemic chemotherapy Taxotere with Herceptin for 6 cycles. Afterwards, CT showed no residual of tumor and showed disappearing of auxiliary mass. So the patient was switched to Femara and Herceptin. During the treatment with Herceptin, she required stopping it few times and manage her cardiac ejection fraction EF decreased. Finally after 4 months of start of Herceptin, we required stopping it due to 20% drop in EF (35–40%) and symptomatic heart failure though patient was on antifailure treatment. She received a total of 25 doses of Herceptin. Three months later, she presented to emergency department with severe headache and CT showed brain metastases for which she received radiation therapy to brain ().\nSeven months after radiation therapy, she developed intestinal obstruction and she was admitted with diffuse peritoneal metastases and she had laparotomy as palliative surgery. Now she is on palliative chemotherapy with weekly Taxol and Herceptin as her EF is improved (50%).
A 51-year-old Caucasian male with no significant past medical history presented to the emergency room with a one-week history of right sided acute neck and arm pain associated with progressive swelling in the right upper extremity. He did not have such complaints in the past and had never seen a physician. He was smoking for 30 years (1 pack per year) but quit 5 years ago. His family history was significant only for coronary artery disease. Doppler ultrasound of the right upper extremity showed an occlusive thrombus in the right internal jugular vein; a nonocclusive thrombus in the right subclavian vein with minimal flow is within this vessel. The remaining veins including the axillary and brachial veins are patent. Hypercoagulable status work-up was unrevealing and meanwhile the patient was started on enoxaparin subcutaneously with a 1 mg/kg dose every 12 hours. Computed tomography (CT) scan of the chest and the neck with contrast showed a singular anterior mediastinal mass with persistent thrombosis of the right internal jugular, subclavian and innominate veins (Figures and ). There was questionable extension of the clot into the right sigmoid sinus (). CT brain and CT abdomen and pelvis were normal. Right video assisted thoracoscopic surgery was done and a biopsy of the anterior mediastinal mass was obtained with no complications. Biopsy showed mediastinal large B-cell lymphoma with sclerosis (Figures , , , and ). Bone marrow biopsy was obtained and excluded the involvement of bone marrow. Cytogenetics study for lymphoma staging showed an apparently normal 46, XY male complement. No apparent numerical or structural abnormalities were evident. Flow cytometry study showed that the bone marrow was not involved by B-cell lymphoma morphologically and immunophenotypically. Patient started chemotherapy sessions and was continued on enoxaparin subcutaneously. The PET scan was done, showing two large hypermetabolic nodal masses of the mediastinum representing the lymphoma. One component of the lower mass extends into the anterior mediastinum. The patient started chemotherapy with R-CHOP-regimen.
In May 2014, a 30-year-old Caucasian woman presented to our department (Fig. a). For the past 4 years, the patient had suffered from severe PPR (principally on the forehead, cheeks, and chin) with signs of rosacea fulminans, as well as severe background erythema in the central facial and forehead regions. She had previously been treated with metronidazole 7.5 mg/g cream and permethrin 50 mg/g cream, which had not provided a reduction in the number of inflammatory lesions. She had also been prescribed doxycycline modified-release 40 mg for 18 months, which had also proved unsuccessful in reducing the inflammation.\nAt presentation, the patient reported feelings of hopelessness, indicating a profound impact of the disease on her quality of life. Following diagnosis of PPR with severe background erythema, different treatment options were evaluated. In such a severe case, a specific strategy tailored to the patient was essential, particularly given the lack of success with prior regimens, which seemed to indicate that a more potent treatment regimen was needed in this patient to combat the inflammation upfront. Isotretinoin was initially considered; however, because the patient was not taking hormonal contraception at the time of her visit to our department, this therapy was contraindicated. The patient was offered a gynecology appointment to start hormonal contraception, so that isotretinoin could be an option in the future. The patient was initially prescribed an 8-week regimen of azithromycin 500 mg three times weekly (taken in the morning) in combination with prednisolone 30 mg once daily (reduced to 10 mg once daily after 1 week). The choice of this regimen was based on results from the literature showing that a rapid response can be obtained with a multimodal, tailored approach with a short course of macrolide antibacterials []. Furthermore, the use of corticosteroids over the short term is not strictly contraindicated in the treatment of rosacea and can be considered as an option to reduce inflammation in patients who present with signs of rosacea fulminans []. Metronidazole 7.5 mg/g cream twice daily was continued with this combination therapy.\nAfter 4 weeks of treatment, a reduction in the inflammatory lesion count was observed (Fig. b). A slight decrease in the severity of facial redness was also seen, especially on the forehead, most likely resulting from a reduction in lesional and perilesional erythema. The patient continued to take azithromycin 500 mg three times weekly and the prednisolone dose was reduced to 5 mg once daily. In addition, the patient continued to apply metronidazole 7.5 mg/g cream twice daily.\nDuring examination of the patient’s face 1 month later, only a small number of inflammatory lesions were present (Fig. c). The severity of erythema remained similar to that observed during the previous visit, indicating a reduction in the severity of the inflammation. Therapy with azithromycin 500 mg three times weekly and prednisolone 5 mg once daily was stopped; treatment with isotretinoin 10 mg once daily (taken with the main meal) was initiated, and application of metronidazole 7.5 mg/g cream twice daily was continued.\nWhen the patient returned to our department 4 months later, no inflammatory lesions were present; however, the erythema had worsened on the forehead and cheeks (Fig. d). The patient continued to apply metronidazole 7.5 mg/g cream twice daily, as well as isotretinoin 10 mg once daily for further improvement and as maintenance therapy to prevent relapse of inflammatory lesions. Treatment with brimonidine 3 mg/g gel, used as needed and applied in the morning, was initially started to the left side of her face in order to assess the impact of this agent on the background erythema (split-face assessment). Brimonidine, a highly selective α2-adrenergic receptor agonist [], is approved for the symptomatic treatment of facial erythema in adults with rosacea, in a formulation of brimonidine 3 mg/g gel (MIRVASO®; Galderma International, La Défense, France) []. This agent has previously been shown to be an effective option in the treatment of moderate to severe erythema, with a good safety profile [].\nIn December 2014, 2 months after commencing treatment with brimonidine 3 mg/g gel on the left side of her face and while continuing treatment with isotretinoin 10 mg once daily to maintain remission of inflammatory lesions, the patient returned to our department, showing mild erythema on the right side of the face, with background erythema cleared on the left side of the face (Fig. e). Of note, during the treatment period with brimonidine 3 mg/g gel, the patient did not experience any dermatological adverse events that required her to stop treatment temporarily.\nAt the most recent visit to our department in March 2015, the patient showed an increase in erythema severity on the right side of her face (not treated with brimonidine 3 mg/g gel) compared with the previous visit, reflecting the dynamic and changing nature of this symptom. The left side of the face, treated with brimonidine 3 mg/g gel on the morning of the visit to the clinic, remained clear of erythema (Fig. f). Isotretinoin treatment was stopped and the patient was advised to continue maintenance therapy with metronidazole 7.5 mg/g cream twice daily and brimonidine 3 mg/g gel.
A 56-year-old male presented to our clinic with bilateral giant inguinal hernias. His complaints were pain, bilateral inguinal mass and insufficient sexual activity. He was operated on four times before for right groin hernia. Concomitant diseases were arterial hypertension and diabetes mellitus. There were incision scars in the right groin region and bilateral giant inguinal hernias (). Laboratory findings were normal except for a slightly elevated blood glucose. Contrast-enhanced CT revealed that omentum, small bowel and colon were in the bilateral hernia sacs (). After evaluation of clinic signs and radiological findings, we decided to prepare the patient with PPP, after obtaining informed consent for the procedure. A peritoneal dialysis catheter was inserted into the abdominal cavity with a 3-cm lower midline incision under local anesthesia. Nitrous oxide was preferred for insufflation because of its inert structure. It was insufflated into the abdomen via a catheter. The first insufflation procedure was applied in the operating room and then at bed side (insufflator moved to the bed side). Daily insufflated gas volume, intraabdominal pressure and expansion of waist circumference at the level of umbilicus was recorded (). Insufflations went on for 18 days except day 11 because of a technical problem (). We determined that abdominal expansion did not increase any more after day 13 despite increased intraabdominal pressure. Waist circumference at the level of the umbilicus expanded and stayed at 11 cm at that time. During the procedures, we gave third-generation quinolones to the patient and an analgesic, when necessary.\nThe patient was operated on under general anesthesia on day 19. A Pfannensteil incision was preferred and there was sigmoid colon and 500 cc of fluid in the left hernia sac. Some small bowel segments, appendix vermiformis, cecum, and 200 cc of fluid was in the right hernia sac. We performed appendectomy for prophylaxis. The hernia contents were dissected easily from the hernia sac and the bilateral hernia contents were pushed into the abdomen easily. The peritoneum was closed with absorbable sutures. Stoppa technique was preferred and a 30×30 polypropylene mesh was placed and fixed to bilateral spina iliaca anterior superior, symphysis pubis, Cooper ligaments, and midline. Suction drains were placed on the mesh and in the scrotum bilaterally. An abdominal incision was closed layer by layer and a peritoneal dialysis catheter was taken out. The scrotum was elevated postoperatively. Mild shortness of respiration and abdominal distention was seen on postoperative day one. On postoperative day two, recovery was excellent and the postoperative course was uneventful (). Drains were removed on day 12 and the patient was discharged on postoperative day 14. A checkup after 11 months showed the hernia to be entirely cured.
A 40-year-old female patient presented at the Emergency Department of our institution with complaints of back pain for the last three days that started after moderate physical exertion. The patient referred no pain relief after taking anti-inflammatory drugs and denied respiratory symptoms such as chest pain, dyspnea, or cough. No weight loss, anorexia, or other symptoms were reported. There was no referral of previous surgeries or medications. A different, stronger anti-inflammatory drug was prescribed, but three days later she returned to the hospital, where a lumbar radiograph revealed the presence of a lytic lesion in the L4 vertebra. Due to the nonspecific appearance of the lesion, the patient underwent an MR of the lumbar spine. The exam revealed the presence of a heterogeneous, T1-hypointense mass in the body of L4, causing its partial destruction, and nerve root compression (). A CT-guided biopsy was performed in order to assess the etiology of this mass. The pathology report described the presence of tumor fragments of mesenchymal origin with smooth muscle differentiation that were diffusely positive for estrogen and progesterone receptors. No obvious nuclear atypia or mitotic figures were identified. Ki-67 proliferation index was less than 1%. The final report stated that the lesion was compatible with BML.\nSubsequently, a contrast-enhanced CT was performed to evaluate if other organs were affected: there were several soft tissue density round masses in the thorax, the largest being located in the left lung, measuring 44 mm (). There was a 12 cm mass in the left iliac crest that enhanced after intravenous contrast. This mass had a lytic component and exhibited an intrapelvic bulky element (). There was enlargement of the uterus due to the presence of several leiomyomas ().\nThe clinical conduct included vertebral subtotal tumor removal, laminectomy, and pedicle screw fixation on L3-L5 () to decompress the nerve roots and reduce the symptoms. Oophorectomy and hysterectomy were performed and confirmed the benignity of the leiomyomas. Outpatient treatment consisting of anastrozole, an aromatase inhibitor, was prescribed. At the 6-month follow-up CT examination, the nodular pulmonary and iliac crest masses kept the previous dimensions and no new lesions were observed. Long-term follow-up was recommended in this case.\nRegarding the low back pain, the patient referred a moderate improvement. Despite this, she needed to resort frequently to taking anti-inflammatory drugs after some physical exertion. The patient also reported a sensation of pressure in the pelvic region, especially in the lateral decubitus, most likely associated with the mass of the iliac bone.
A 39-year-old female was brought to our emergency room by a private vehicle after being shot. An evaluation revealed a wound at the lower left back and at the mons pubis. Exploratory laparotomy and resection of the sigmoid colon, left ovary, and fallopian tube with a colostomy was performed. Postoperative deep venous thrombosis prophylaxis in the form of enoxaparin, 30 mg every 12 hours, was given and then changed to heparin, 5,000 units subcutaneously every eight hours, due to worsening renal function. The patient did well until postoperative day 4 when she complained of increased abdominal pain out of proportion to the clinical findings. Her symptoms worsened the following day. A computed tomography (CT) scan of the abdomen and pelvis was obtained, the results of which were consistent with postoperative ileus and raising concerns for right colon ischemia.\nThe patient was managed conservatively, and bedside drainage of the superficial wound infection was done on postoperative day 6 with improvement in abdominal pain. On the following day, a repeat CT of the abdomen and pelvis raised more concerns for bowel ischemia; however, the patient refused reexploration (Figure ).\nA computed tomography angiogram (CTA) of the chest was obtained for worsening pulmonary symptoms on postoperative day 8 and was consistent with lower lobe pneumonia.\nFeculent drainage around the stoma was noticed on postoperative day 9, and an exploratory laparotomy was performed with resection of multiple small bowel ischemic loops, cholecystectomy, and right hemicolectomy. The rest of the small bowel appeared dusky and friable. After multiple visits to the operating room, she was left with only a few inches of the proximal jejunum. A CTA of the abdomen was done on postoperative day 13 from her initial surgery and was consistent with a superior mesenteric artery occlusion (Figure ).\nAdmission platelet count was 281, down to 82 on postoperative day 11 when she tested positive for heparin-induced antibodies. The pre-test probability for HIT score was high (2 for thrombocytopenia, 1 for timing, 2 for thrombosis, 2 for no other cause = 7). The heparin was stopped and argatroban was used. The patient did survive her injury and was referred to a small bowel transplant center.
A 20-year-old left-handed male college student incurred left brachial plexus injury in a motorcycle accident. He sought medical attention at our hospital two weeks after the injury. However, the rehabilitation did not begin until four months after the injury. MMT at the beginning of the rehabilitation on the affected side was as follows: deltoid, 2; biceps brachii, 2; triceps brachii, 4; wrist flexion muscles, 4; wrist extension muscles, 4; lumbricals, 4; and interosseous muscle, 4. His sensory function was only impaired in the region innervated by the axillary nerve. The assessment of ADL performance revealed that he was fully independent with the use of the unaffected limb. The preoperative rehabilitation involved ROM exercises and strengthening of the remaining muscles. Since surgical reconstruction of elbow flexion was planned, the focus was on strengthening of the muscle groups involved in wrist flexion and extension (). Recovery of the biceps brachii muscle (elbow flexion strength) stopped at MMT grade 2; thus, the modified SLM procedure was performed one year and five months after the injury. After immobilization with a cast for five weeks, the patient began training of the range of elbow flexion with active motions of the transferred muscle with the support of a sling. Simultaneously, the patient underwent active training of the wrist extensor muscles. Subsequently, he underwent active elbow flexion training, and we initiated manual resistance active elbow flexion training at four months postoperatively. At six months postoperatively, the patient achieved excellent elbow flexion strength (MMT 4), and the active range of elbow flexion increased from 0° preoperatively to 120° postoperatively (). The patient was able to lift a plastic bottle up to his mouth. At 12 months postoperatively, his elbow flexion strength remained at MMT 4 and the active range of elbow flexion increased to 145°. The preoperative MMT of the wrist extensor muscle group was 4, and it increased to 5 at 12 months postoperatively. Active ranges of elbow extension, forearm pronation/supination, and wrist flexion/extension at 12 months after surgery were −20°, full range, and full range, respectively. As with the patient in Case 1, the patient in this case was also able to carry a 5 kg object with both hands.
An 11-month-old male patient was referred to our department with the chief complaint of a “little ball” in the lower lip and that he had difficulty in sucking for more than 3 months. The baby was in good general health and no other symptoms were reported. Oral habits or a local trauma was not reported. The clinical examination revealed the presence of a soft tissue nodule on the lower lip mucosa () which was similar in color to the oral mucosa measuring approximately 5 cm at its widest diameter with a sessile base, flaccid consistency, clearly defined limits, and a smooth surface. Based on detailed history and clinical examination a provisional diagnosis of mucocele was made. After medical evaluation, and signed informed consent from the parents, an excisional biopsy was performed under local anesthesia. Due to the lack of baby's contribution, considering his little age, and as the procedure was simple, a decision was taken in favor of the physical containment (protective stabilization) with consent and aid of the parents: laying the baby on the chair, the mother laying over him holding the hands, and the assistant holding the baby's head. As the baby was crying continuously, it helped in keeping the mouth open. A local infiltrative anesthesia (2% lignocaine with epinephrine 1 : 80,000; one cartridge) was infiltrated around the lesion. Before infiltration, a topical anesthetic gel for 2 minutes was applied. The lip was then everted with digital pressure to increase the lesion's prominence. A thick silk thread was passed through the lesion at its largest diameter and a surgical knot was made followed by excisional biopsy using electrocautery (Figures and ), hence minimizing the chances of pain and postoperative bleeding. An analgesic was prescribed on the first postoperatory day to prevent any possible pain that could result in stress for the baby. The specimen was sent for histopathologic analysis which identified a large central mucous pooled area consisting of mucinophages, mucin containing cells, surrounded by compressed connective tissue wall, and forming granulation tissue () and confirmed the diagnosis as mucocele. After 2 hours, the patient recovered normal breastfeeding. The child reported uneventful recovery and an improved dietary habit one week postoperatively.\nThe baby was reexamined after 15 days and 6 and 12 months. No recurrence was observed after 12 months ().
Patient is a 68-year-old female who presented to the emergency room after a fall while walking her dog with complaints of headaches, nausea, and vomiting. Patient denied any loss of consciousness. Her past medical history consisted of hypertension and peripheral vascular disease. Patient smoked one pack of cigarette per day. On physical examination, she had no focal neurological deficits but was confused. Computed tomography (CT) scan showed a large left 1 cm convexity acute subdural hematoma (SDH) with 6 mm midline shift. Due to her disorientation and midline shift, the patient was taken back to the operating room (OR) for evacuation of the SDH. A reverse question mark incision was made with a standard trauma craniotomy. During the operation, the superficial temporal artery (STA) was mistakenly sacrificed. Patient tolerated the surgery well and continued to improve with her mental status postoperatively. She was discharged to rehab on postoperative day 7. The patient continued to smoke despite our recommendations.\nShe returned to the emergency room 19 days later complaining of fever, erythema, swelling underneath the wound, and purulent drainage on her pillow. The radiographic findings suggested that the infection was superficial. After being evaluated in the emergency department, she was taken to the OR for surgical lavage and debridement. During the operating, her bone flap was felt to be viable and free of infection, so it was not removed. Intraoperative cultures were found to grow methicillin-sensitive Staphylococcus aureus, and the patient was started on IV Nafcillin. Approximately 2 weeks later, purulent drainage returned from the incision site. Radiographic findings suggested that there was an intracranial component. She was taken to the OR for a wound washout. Given the degree of infection identified during the operation, as well as the intracranial infection noted in the subdural space, the decision was made to remove the bone flap.\nOver the next week, the wound started to break down. Subsequently, there was a wedge-shaped necrosis that developed on the flap []. Dura was visible in the area of wound breakdown. After consulting with plastic and oral maxillofacial surgery, the patient was taken back to the OR two weeks later for resection of necrotic scalp, dura repair and wound VAC placement. The dura was noted to be frail, shredded, and portions were missing [Figures –]. Because of the risk of neurological sequelae and cerebrospinal fluid (CSF) diversion with a VAC device placed on exposed brain tissue, water-tight closure of the dura was a necessity prior to placement of the VAC device. After achieving securable edges, Durepair® (Medtronic, Inc.) []. was sutured in to native dura with 4-0 Nurolon (Ethicon). Water-tight closure was confirmed with a Valsalva maneuver, and fibrin glue was not used. On gross inspection, the wound vac canister did not have any CSF contents. Negative pressure therapy was set to -50 mmHg []. The wound vac was changed approximately every 3 days until adequate granulation tissue was noted and the infection had subsided []. Intraoperative cultures during the wound vac changes grew Candida. Patient was started on diflucan.\nTwo weeks later, the patient was taken to the OR with plastic and oral maxillofacial surgery for a latissimus dorsi muscle flap reconstruction. The patient was monitored in the intensive care unit (ICU) with daily Dopplers to ensure the viability of the flap. The flap went on to necros as a result of an infection caused by Pseudomonas. The patient went to the OR for removal of her flap. The dura with the synthetic graft still had a water-tight closure. The wound VAC was replaced and changed approximately every 3 days. To ensure the next flap would not fail, the granulation tissue on the Durepair® (Medtronic, Inc.) was cultured. The intraoperative culture also grew Pseudomonas, but only on the synthetic dura. After a multidisciplinary discussion, we felt that a native substitute for the dura may decrease the risk of infection.\nThree weeks later, the patient was taken to the OR removal of dural substitute, harvest of fascia lata graft, and anterolateral thigh free flap. The fascia lata graft was harvested from the contralateral leg of the anterolateral thigh free flap. The fascial lata graft was secured in a water-tight fashion. The anterolateral thigh free flap was performed without any complications [].\nPatient returned to the ICU on the ventilator with continued sedation due to flap anastomosis fragility. The ventilator was weaned off on postoperative day 1. Patient was at her neurological baseline after extubation and transferred to the floor on postoperative day 5. Patient continued her antibiotic regimen of Nafcillin and Diflucan postoperatively. Patient followed up with neurosurgery, plastic surgery, and oral maxillofacial surgery. At the 6-month follow-up, the flap had healed without any signs or symptoms of infection. The patient has been followed for 14 months after surgery with the reconstruction intact [].
The 17-year-old female patient was admitted in order to undergo the removal of a cystic tumor of the upper mediastinum. The patient was diagnosed at the age of 10 while being examined after a chest trauma. Postero-anterior chest X-ray revealed a widened shadow on the left side of the upper mediastinum within the aortic arch.\nA computed tomography (CT) scan confirmed the presence of a cystic mass within the left upper mediastinum, situated laterally from the arch of the aorta and the pulmonary trunk (). The mass was defined as a well-circumscribed, thin-walled area filled with dense, homogeneous, hypodense liquid. There was no mediastinal adenopathy or communication between the cyst and the adjacent structures. At the same time, the CT scan revealed a parenchymal triangular area corresponding to persistent thymus.\nSince the diagnosis, the patient has remained under constant ambulatory control. In subsequent chest CT scans, the cyst remained stationary – no significant changes were observed in its size or characteristics. The dimensions of the cyst in magnetic resonance imaging (MRI) of the chest were comparable with those described after previous examinations (CC 5.2 cm × SD 4.1 cm × AP 4.2 cm). Magnetic resonance imaging also showed modeling of the left lung and aortic arch by the lesion; the image of persistent thymus was stationary. No other pathological changes in the mediastinum were found. Due to emerging symptoms in the form of hoarseness and dry cough, a decision was made to perform surgery. The resection was performed using a thoracoscopic procedure. After the induction of general anesthesia, the patient was intubated with a standard endotracheal tube. Intubation with a double-lumen endotracheal tube was abandoned, and the deflation of the left lung was obtained with pleural insufflation pressure of about 8 mm Hg. Three ports were placed: one 10 mm trocar in the anterior axillary line in the fourth intercostal space and two 5 mm trocars in the third intercostal space – the first in the midaxillary line and the second near the mammary line. Intraoperatively, the cyst was found to be overlying the root of the left lung, the left pulmonary artery, and the descending aorta; it was adjacent anteriorly to the left atrium and the ascending aorta (). A harmonic scalpel was used to separate the cyst from the surrounding tissues; special attention was paid to dissecting the anterior part of the cyst from the phrenic nerve and the posterolateral part from the vagus and recurrent laryngeal nerves ().\nThe cyst was dissected free from the trachea and the left pulmonary artery; it was then removed by sucking the mucous content through the aperture in the 10 mm trocar. The operative field was rinsed with an antibiotic solution, and a drain was placed. The surgery lasted 90 min. The chest drain was removed from the pleural cavity on the 1st postoperative day after a chest X-ray inspection confirmed the overall expansion of the left lung. To confirm the completeness of the surgery, a CT scan of the chest was performed on the 2nd postoperative day, revealing no embedded fluid reservoirs within the mediastinum. The patient was discharged on the 3rd postoperative day in good general condition.\nThe patient had no complications beyond short-term hoarseness. Histopathological examination confirmed the diagnosis of a bronchogenic cyst. During the follow-up period (3 years), there were no complications, the patient did not report any symptoms, and the imaging studies did not indicate any abnormalities.
The transplant recipient is a 58-year-old female who developed type I diabetes mellitus at 6 months old and used an insulin pump for blood sugar control. She developed end organ damage including renal failure but was not yet dialysis dependent. She also suffered from retinopathy and peripheral neuropathy. She did not have hypoglemic unawareness and had good blood sugar control with her insulin pump. The remainder of her medical history included hypertension, hyperlipidemia, and hypothyroidism. She had remote hysterectomy and appendectomy. She underwent a full evaluation for kidney and pancreas transplantation without significant findings. She was approved and listed for combined simultaneous kidney pancreas transplantation. At the time of transplantation her panel of reactive antigen (PRA) was 0% and there were no identifiable HLA specific antibodies. Hr BMI was 26 at the time of transplantation.\nThe donor was a 31-year-old male motorcyclist who collided with a concrete medium at highway speed. He was not wearing a helmet. The donor did not have significant past medical or surgical history, except for smoking cigarettes and occasional marijuana. There was no history of renal calculi or recurrent urinary tract infections. The donor family was not aware of his congenital renal anomaly and the patient did not have other congenital anomalies. A computed tomography (CT) scan of the abdomen done at the time of admission revealed an ectopic right kidney located in the pelvis and fused with the lower pole of the left kidney, compatible with crossed fused renal ectopia (). There were no other significant findings. The patient was hemodynamically stable with good urine output the time of procurement. The serum creatinine peaked at 1.8 and was 1.4 at the time of procurement. There was mild elevation of blood glucose requiring sliding- scale insulin.\nThe procurement of the kidneys, pancreas, and liver was completed in the standard fashion, flushing with Custodial HTK (Odyssey) solution. The kidneys were procured en bloc, with a complete cava and aorta. The left kidney had a single artery, and the right had three arteries. The upper and mid polar arteries of the right kidney originated from the aorta, while the lower pole artery originated from the right common iliac artery. The mid and lower pole arteries were smaller, accessory arteries. There were single renal veins bilaterally, which drained into the inferior vena cava. The left ureter crossed the midline and entered the bladder in the normal anatomic position.\nThe kidneys were transferred to our institution. The back table dissection and parenchymal transection was completed with the assistance of the urology service. Initially we had planned to split the kidneys and use each singly for transplant (). However, the right kidney was abnormal in appearance; in particular it was very thin. The mid and lower pole arteries were very small and were not connected to the aortic patch, making them difficult to reimplant. We elected not use the renal mass en bloc, due to the complex arterial anatomy, including the number, caliber, and configuration. At that time the right kidney was split from the left, carefully transecting the isthmus using sharp dissection. A few vessels and what appeared to be a small calyx were suture ligated with Vicryl suture. Next, the transection plane was closed using interrupted 0 chromic mattress sutures. Pledgets of fat were used to prevent any cut through of the sutures through the parenchyma (). Both the artery and vein were cleaned of surrounding neurolymphatic tissue.\nThe kidney and pancreas transplantation was then completed in the standard fashion. The kidney was anastamosed to the external iliac artery and vein on the left side. The kidney reperfused well, with minimal bleeding after reperfusion needing only bipolar coagulation for control. The pancreas graft was anastamosed to the right common iliac artery and vein and the donor duodenum to the recipient jejunum. Immunosupression included Campath induction, with Prograf, Cellcept, and a rapid steroid taper. The patient had immediate graft function and an unremarkable postoperative course. She was discharged on postoperative day seven. A protocol pancreatic biopsy at one year demonstrated Drachenberg grade III out V acute rejection. She was treated with thymoglobulin and Solumedrol and continued on steroids after discharge. She is now 2 years posttransplant with normal pancreatic (fasting blood sugar 86, c-peptide 2.1, hemoglobin A1C 5.4) and renal graft function (creatinine 1.0 and creatinine clearance 55).
A 65-year-old man was admitted to hospital with acute abdominal pain and hypovolemic shock. He was a cigarette smoker for more than 20 years, had arterial hypertension and a medical history of cholecystectomy and appendectomy. Abdominal CT imaging showed a huge retroperitoneal hematoma of 7 × 23 × 25 cm (Fig. ) and an aneurysm of the IMA about 2 cm from its origin resulting in concurrent occlusion of superior mesenteric and celiac arteries (Fig. ). The IMA also was very wound from its origin. The patient underwent midline laparotomy with a transperitoneal approach to the IMA by a multidisciplinary team of vascular and colorectal surgeons. Severe bowel adherences were observed which possibly helped to limit the massive hemorrhage limited to the retroperitoneum. The aneurysm was completely excised. The normal proximal and distal ends of the IMA were then mobilized. The artery was finally reconnected in an end-to-end manner to re-establish blood flow. Neither a graft nor a shunt was used, with only a short period of ischemia during the running suture. Heparin was rinsed only locally, due to the massive hemorrhage and the need for transfusion. Macroscopic examination of the aneurysm sac demonstrated typical appearances of atherosclerotic aneurismal tissue. There was no history of trauma or any clinical sign of sepsis that led us to suppose that another cause might be responsible for this lesion. The postoperative course was uneventful, and no symptoms of intestinal ischemia were noted.\nWorldwide, there have been only eleven reports of IMA aneurysms associated with tight stenosis of the SMA and celiac trunk [,]. Other publications have presented cases with abdominal angina and weight loss [] or even rupture []. Previous authors have conjectured that this situation may arise because of a ''jet disorder'' phenomenon. Concurrent occlusion of the superior mesenteric and celiac arteries leads to greatly increased and possibly turbulent blood flow in the IMA []. This produces localized areas with high arterial pressure that in an unadapted vessel can lead to aneurysm formation and subsequently thus to rupture, as was the case in our patient. The marginal artery of Drummond was also greatly increased in size (Fig. ) and supplied the entire gastrointestinal tract. Today, abdominal ultrasound, computed tomography, magnetic resonance imaging, and arteriography facilitate an early diagnosis. Although these patients are generally asymptomatic, it is generally acknowledged in literature, to treat VAA because of the risk of rupture or ischemia. Either surgical or endovascular therapeutic procedures can be performed in the treatment of this lesion and are well described in literature []. Percutaneous transcatheter coil embolization techniques are also used with increasing frequency in the treatment of VAA []. Nevertheless, a high incidence of aneurysmatic sac reperfusion and a relatively high morbidity are associated with this procedure. Other percutaneous techniques such as permanent liquid embolic material and the use of a covered stent have been proposed, but the results must yet be verified. In our case, because of the hypovolemic shock an emergency surgical approach was indicated. The IMA being very tortuous, thus implying a difficult endovascular approach, we decided to perform a direct anastomosis between both ends of the inferior mesenteric artery instead, which seemed more feasible in our case. In the same instance we could take advantage of this type of approach to also evacuate the symptomatic retroperitoneal hematoma mentioned above. The mesenteric circulation being not compromised, revascularisation of the SMA and celiac trunk was not undertaken.
A 43-year-old Caucasian woman presented in her 32nd week of gestation with a tumor of the parotid gland. She had noticed the swelling increasing over the past six months. She was otherwise asymptomatic, without any pain, mouth-opening difficulties or facial nerve dysfunction. Ultrasonography of her neck showed a parotid lesion 40 × 30 × 27.5 mm in size, with an heterogeneous appearance. Considering her advanced state of pregnancy, we advised conservative management in the form of watchful waiting and regular review until the birth.\nAt our patient's next review, only five weeks later, we noticed rapid growth of the lesion with expansion into the parapharyngeal space. There was still no facial nerve involvement, although our patient's mouth opening was slightly limited. Magnetic resonance imaging (MRI) of the neck showed that the tumor had increased to 70 × 60 × 60 mm, reaching the parapharyngeal space with marked obstruction of the oropharynx of about 50% (Figures , ). Further diagnostic tests such as fine-needle aspiration cytology (FNAC) were discussed. We were concerned about the risk of malignancy and increasing local complications due to the rapid growth. Considering the risk of spreading tumor cells and the unreliability of FNAC in identifying the malignant nature of parotid carcinoma [], we decided to perform surgical resection to prevent further complications and to obtain a secure diagnosis.\nAfter discussing this case with the gynecologists at our interdisciplinary tumor board meeting, it was decided to deliver the baby by caesarean section in the 38th week of gestation with epidural anesthesia using mepivacain and sufentanil. The surgical resection of the tumor was performed four days later. It was possible to completely remove the tumor via a standard cervicofacial incision without the need to resect the mandible. Although the tumor had stretched the facial nerve to double its usual length, there was no postoperative nerve dysfunction (House Brackmann grade I).\nOn histological analysis, the lesion was identified as a pleomorphic adenoma without evidence of malignancy (Figure ). Immunohistochemistry did not show any positive expression of oestrogen or progesterone receptors. Our patient remained well with no evidence of recurrence after a follow-up of one year.
A 70-year-old female was admitted to a local district general hospital following an out of hospital cardiorespiratory arrest. The patient had called an ambulance herself due to difficulty in breathing. On arrival, paramedics found a patient with acute dyspnoea and stridor who collapsed shortly afterwards with loss of cardiac output. Cardiopulmonary resuscitation (CPR) was commenced and there was return of spontaneous circulation after three minutes. The cardiac rhythm during these events was not recorded and no resuscitation drugs were administered. The patient was transferred to the emergency department where, due to continued poor respiratory effort, the patient's trachea was intubated after a rapid sequence induction and she was transferred to the critical care unit (CCU) for ongoing management.\nA presumptive diagnosis of cardiorespiratory arrest secondary to an exacerbation of COPD was initially made. A collateral history taken from relatives revealed that she had become progressively more breathless over the preceding three weeks, and despite a course of oral antibiotics and steroids there was little improvement. More detailed investigation of the patient's past medical history revealed that she was known to have a cystic swelling in the anterior cervical area. This had increased in size over the previous three weeks and was associated with progressive dysphagia and dyspnoea. The patient was awaiting outpatient otorhinolaryngology review.\nIn view of this history, computerised tomography (CT) imaging of the neck and thorax was performed. This revealed a seven-centimetre, well-defined, nonenhancing, rounded homogenous opacity at the thoracic inlet, consistent with a large midline thyroid cyst (). Significant consolidative change within the inferior lobe of the left lung was also noted (). Subsequent clinical examination revealed a large cystic swelling occupying the whole of the thyroid gland. Needle aspiration of the cyst yielded approximately 50 mL of frank blood.\nOn review of the clinical history and taking into account these new findings, it was felt that the patient had suffered acute airway obstruction secondary to haemorrhage into a preexisting thyroid cyst.\nThe patient was invasively ventilated for total of seven days before successful extubation to noninvasive ventilation. She received antibiotic therapy during this time for community-acquired pneumonia. In view of the progressive thyroid swelling causing dysphagia and airway compromise, it was felt to be highly likely that the patient had been suffering from chronic aspiration over the preceding weeks. This, in combination with acute airway obstruction, had caused a significant deterioration in the patient's condition leading to cardiorespiratory arrest.\nFollowing discharge from CCU, clinical examination revealed that the thyroid cyst had reaccumulated. Consequently, an elective subtotal thyroidectomy was performed and a large retrosternal cyst within a multinodular goitre was excised. Histopathological examination of the specimen identified a benign cyst within the isthmus of the thyroid gland. There was no evidence of malignant change.\nThe patient was subsequently discharged from hospital 6 days following the operation and reviewed in outpatient clinic three weeks later. She was commenced on levothyroxine therapy and has remained clinically and biochemically euthyroid, with no further enlargement of the remnant thyroid tissue.
A 48-year-old man with a history of alcohol abuse presented to the gastroenterology department for the first time in November 2006. He was suffering from severe epigastric pain and nausea for two days. Because the laboratory findings were suspicious for pancreatitis, the patient underwent computed tomography (CT) (Fig. ). The examination showed an edematous pancreas tail and fluid in the anterior prerenal space and in the anterior, posterior and lateroconal fascia. There were no signs of chronic pancreatitis or vascular complications. In the following months the patient had two similar episodes of acute tail pancreatitis.\nIn September 2009 he presented with vague epigastric pain of several weeks duration. Serum amylase and lipase levels were normal, however the cholestatic parameters were mildly elevated. Therefore, a magnetic resonance (MR) study was performed. The examination demonstrated moderate chronic pancreatitis of the tail with atrophy and irregular bording of the Wirsung’s duct. There were no other abnormalities. Further examinations also revealed a Helicobacter pylori gastritis.\nIn February 2012 the patient presented again to the gastroenterologist with epigastric and left upper quadrant pain. Laboratory findings were not suspect for acute pancreatitis. However, contrast-enhanced CT scan was performed in order to evaluate the known chronic pancreatitis and other causes of pain (Fig. ). This study surprisingly revealed a hypervascular lesion in the pancreatic tail consisting of a conglomeration of small hypervascular spots and blood vessels. Further examinations were performed in order to differentiate a vascular malformation from a hypervascular pancreatic tumor, especially an islet cell tumor or a hypervascular metastasis. Contrast-enhanced MR study showed a focal area of heterogeneous contrast enhancement without an apparent nodular component. The subsequent angiography showed two large feeding arteries in the early arterial phase, followed by a racemose vascular network, an early transient dense parenchyma stain in the early portal phase and early wash-out of the lesion in the portal phase (Fig. ). Based on these findings, a pancreatic arteriovenous malformation was suggested. The patient was treated with a resection of the pancreas tail and postoperative recovery was uneventful. Histopathologic examination revealed sequelae of chronic pancreatitis and numerous dilated blood vessels accompanied by blood clot formation and intimal hyperplasia (Fig. ). The histopathological findings were consisting with a pancreatic AVM.
An 83-year-old man underwent surgical excision of a malignant peripheral nerve sheath tumor (MPNST) at the right knee at another hospital and was then referred to our hospital for further chemotherapy. Three months after the start of chemotherapy, the patient complained of severe pelvic and low back pain. A PET CT was performed which showed multiple bony lesions with a variable (moderate to high) degree of metabolic activity raising suspicion that these were metastases. The lesions, however, had a very unusual appearance and evolution as seen on CT.\nInitially, the lesions presented as small rounded lytic lesions with a well-defined sclerotic margin (Figure ). They exhibited a rapid growth but the initial appearance of central lysis and surrounding sclerotic margin was preserved. Follow up PET CT scan was performed after seven weeks to evaluate the response to chemotherapy. It revealed a second concentric band (halo) around some of the lesions. This band (halo) had a ground-glass appearance and was surrounded by a second sclerotic rim. This rim was thinner and less sharply demarcated than the more central rim (Figure ). The multiple lesions were in different phases of evolution and as such had a different appearance at any given time.\nPresence of multiple lesions, a known primary tumor and moderate to high uptake of FDG tracer on PET scan (Figure ) suggested metastatic disease, despite the unusual appearance of the lesions.\nOther bony metastases were also evident on CT. Some were entirely sclerotic and a few had an ill-defined lytic appearance. Collapse of a vertebral body and fracture of the upper endplate of another vertebra due to metastases were also present.\nBone biopsy of a lesion with the above described doughnut appearance located in the left pubic bone was performed. A biopsy was obtained in the central lytic area and another biopsy in the peripheral halo (Figure ). Histological evaluation of both the samples showed infiltration of bone marrow by malignant spindle-shaped cells arranged neatly in bundles. Histological and immunohistochemical studies were compatible with metastases from a spindle cell tumor (in this case MPNST).
A 30-year-old female patient was admitted to our department with lower back pain and partial motor deficit in the lower extremities. X-rays showed an irregular lytic mass at S1-2 levels extending through the fourth lumbar vertebra. Coronal post contrast T2 and sagittal T2 MRI sequences revealed a heterogeneous hyperintense lesion with increased enhancement within the central canal from S1, destroying S1-2 and extending upward within the spinal canal to the L4 vertebra (). The diagnosis of subependymoma was confirmed with open incisional biopsy as needle biopsy was not diagnostic. The patient was treated with total sacrectomy via a combined anterior and posterior approach because of the involvement of the whole sacral bone through the sacroiliac joint and ligation of the internal iliac vessels through the anterior abdominal wall was the initial step in the surgery as the lesion was huge and destructive. The lesion extended into the spinal canal up to the level of L4 vertebra, and excision of the neural tumor was extended through the L3-5 level. S2-3 nerve roots were damaged partially as they were involved by the tumor tissue resulting in anal and urinary incontinence. Also, as the S1 nerve root was invaded by the tumor tissue, nerve root dissection was performed. Spinoiliac stabilization from the L3-L5 vertebra to the pelvis was performed with 4 rods, transpedicular screws, and 1 transverse connecting rod a in order to achieve stable fixation, and the sacral defect was reconstructed with 2 fresh frozen fibular allografts as demonstrated in lateral and AP X-rays taken in the eleventh month postoperatively, showing a slight bony union (). The transversely positioned fibular allografts secured the reconstruction mechanically ().\nThe patient was irradiated locally in order to avoid a possible recurrence. In the postoperative period, urinary and bowel incontinence resolved partially with medical and physical treatment. Also, deep wound infection was noted at the operation site, and it was treated with serial debridement and skin reconstruction. She had no evidence of recurrent disease and is ambulatory with the help of a walker during the 19 months after the operation because of residual motor deficit with slight low back pain.
A 73-year-old Caucasian woman was admitted for elective shoulder replacement for an arthritic shoulder. She was being treated for hypertension with beta-blockers and thiazide diuretics and her blood pressure was well controlled. She had a history of diverticulitis and varicose vein surgery with an uneventful outcome. Her body mass index (BMI) was 34 during pre-operative anesthetic evaluation. She had no family history of DVT/PE. Blood investigations including electrolytes, liver function tests and coagulation screening were normal. Results of her electrocardiogram (ECG) and chest X-ray were unremarkable. The comorbidities were well optimized and she was admitted a day before her operation.\nThe patient was operated under general anesthesia supplemented with an interscalene block for postoperative analgesia. An intermittent calf compression device was used for mechanical thromboprophylaxis intraoperatively. The operation was performed through an anterior deltopectoral approach. The patient had no intraoperative complications. The patient recovered satisfactorily from general anesthesia and was monitored in a high dependency care unit for postoperative optimization. The operated shoulder was supported in an arm sling support for comfort. The patient was prescribed postoperative opioid analgesics and antibiotic prophylaxis with three doses of cefuroxime. No chemical or mechanical thromboprophylaxis were used postoperatively.\nThe patient was already feeling well on the next postoperative day and her blood parameters were satisfactory. Her pain was well controlled and her arm continued to be immobilized by the sling. There was no excessive oozing from the surgical wound and there were no distal neurovascular deficits. The patient's calves were soft and nontender and she was ambulating well postoperatively. A systemic examination of the patient was unremarkable. However, while being monitored she suddenly became breathless, hypoxic and hypotensive and experienced intense sweating. She did not complain of chest pain and an ECG was negative for cardiac ischemic changes. Her PaO2 was 84% on room air. She was resuscitated with oxygen by mask and initiated on low molecular weight heparin in a therapeutic dose. Despite all resuscitative measures the patient could not be revived. A post-mortem examination was requested, which confirmed pulmonary embolism and left calf deep vein thrombosis.
A 76-year-old woman presented with a protruding scalp mass in the vertex after tripping a couple of days prior to her visit to our emergency department. She had undergone a left nephrectomy at another hospital following the diagnosis of RCC 18 years prior to admission. Magnetic resonance imaging (MRI) showed a large epidural well-enhancing hypervascular tumor in the vertex that compressed the superior sagittal sinus (SSS) downward, with parietal bone destruction. A homogenously well-enhanced tumor was also found along the vein of Galen, with another well-enhanced tumor in the 4th ventricle (). Brain computed tomography revealed calcification in the mass along the vein of Galen. Abdominal ultrasonography confirmed the left nephrectomy status and no RCC recurrence was evident in either kidneys. Whole body positron emission tomography study showed hypo-metabolism of three brain lesions compared to that of the cerebral cortex, indicating a low grade brain tumor, such as a meningioma. No other abnormal hypermetabolic lesions were found in the rest of the body. Tumor embolization for the vertex mass was performed two days before the surgery. The tumor was supplied by bilateral superficial temporal, occipital, and middle meningeal arteries, and these were successfully embolized using polyvinyl alcohol (Contour, Boston Scientific, USA). The patient underwent a right parietal craniectomy and resection of the vertex mass on Dec. 14, 2005, followed by cranioplasty with bone cement. The tumor was firm and fibrotic and was compressing the SSS. A final histopathological finding was compatible with a metastatic brain tumor from the RCC. The patient underwent fractionated stereotactic radiation therapy for the vertex tumor bed and 4th ventricular mass on Dec. 27, 2005. The tumor found along the vein of Galen was not included in the stereotactic radiosurgery because the MRI findings indicated that it was a meningioma.\nThe patient was admitted again on Jan. 31, 2008 for dizziness accompanied by gait disturbance. The brain MRI showed an increase in the size of the 4th ventricular mass with increased peritumoral edema (). No changes were noted in the sizes of the previously operated vertex mass or the mass along the vein of Galen. No other new intracranial lesions were found. The patient underwent a suboccipital craniotomy and tumor resection for the 4th ventricular mass, which was found to be highly vascular and tightly adhered to the choroid plexus. The lesion was completely removed, and no remaining tumor was observed on the postoperative MRI (). The histopathological diagnosis was also compatible with metastatic RCC, which was identical with the previous vertex tumor (). The patient recovered without any neurological deficits and received no further adjuvant therapy. A follow-up MRI was taken in our out-patient department after 16 months because she had stopped visiting the hospital after she was discharged. The MRI showed a new small enhancing mass in left cerebellum and recurrence of the vertex mass (). No changes were seen in the mass along the vein of Galen and 4th ventricular mass. The patient underwent gamma knife radiosurgery for the recurred vertex mass and the cerebellar mass in May, 2009. She survived for about two more years and died in June, 2011.
A 1-year-old boy was noted to have protrusion of his right eye and was diagnosed with the fetal type of orbital rhabdomyosarcoma in October 2008. The size of the mass was reduced by a peripheral blood stem cell transplantation, chemotherapy and radiation therapy with 50 gray units in the period from November 2008 to June 2009. He developed a cataract in the right eye in May 2011, and his visual acuity in that eye decreased to hand motion. He underwent phacoemulsification and aspiration, and intraocular lens implantation in the right eye in July 2011. There were no abnormal findings on fluorescein angiography, which was performed soon after the cataract surgery. His visual acuity after the cataract removal was 20/200 and, with amblyopia training, his visual acuity improved to 20/25. In May 2016, he underwent YAG-laser posterior capsulotomy and his visual acuity was maintained at 20/25.\nAt a regular examination in January 2017, his visual acuity was 20/60 in the right eye and 20/20 in the left eye. The intraocular pressure was 13 mm Hg in both eyes, as measured with noncontact tonometry. Slit-lamp microscopy showed superficial punctate keratopathy of the right eye. The left eye was clear. The bulbar conjunctiva was not injected in either eye. The anterior chamber of the right eye was deep and there were no signs of inflammation. The depth of the anterior chamber of the left eye was normal and the lens of the left eye was clear. Examination of the fundus by indirect ophthalmoscopy showed neither a retinal detachment nor retinal hemorrhages in either eye. Optical coherence tomography (OCT) showed macular edema in the right eye (Fig. ). There were no soft exudates or retinal hemorrhages (Fig. ). Fluorescein angiography showed a hyperfluorescent site on the right side of the fovea of the right eye, but there were no nonperfused areas (Fig. ). The macular edema was considered to be due to the prior radiation therapy.\nAs mentioned above, different types of treatment have been used for radiation maculopathy, including a sub-Tenon's injection of triamcinolone acetonide, intravitreal anti-VEGF agents and PDT using verteporfin.[ We explained these options to his parents, and they chose PDT because the anti-VEGF treatment would have required repeated intravitreal injections. However, PDT for this type of disorder had not been approved in Japan at that time. After receiving approval for PDT from our Ethics Committee, we performed low-emission energy PDT, in June 2017 with irradiation energy of 25 J/cm2 for 45 seconds. The patient's visual acuity improved after the treatment, and the macular edema decreased after the PDT (Fig. ). OCT showed that the foveal thickness decreased between 1 month and 3 months after the PDT but then gradually increased (Fig. ). However, in the long run the Ganglion Cell Complex is gradually decreasing after the PDT (Fig. ).\nAt a follow-up visit in July 2020, his visual acuity was 20/50 in the right eye and 20/20 in the left eye. OCT showed macular edema in the outer plexiform layer of the right eye, but the left eye was completely normal.
A 72 year old woman with medical history of well controlled hypertension and hypothyroidism underwent minimally invasive aortic valve replacement surgery for aortic stenosis. Postoperatively, she did well and was discharged with retained epicardial pacemaker leads, having received perioperative cefazolin. She did well for approximately two months but then developed redness, swelling and purulent discharge at the incision site in right inframammary area at the working port site. She had no fever, chills or systemic signs of infection, however, given clinical findings, she was taken to operating room for debridement. Purulent discharge was found superficially subcutaneously, and atrial and ventricular epicardial pacemaker wires were removed without complication. Blood and wound cultures were sent. One wire was covered by granulation tissue inside the chest wall and was unable to be removed, remaining in situ. The wound cultures were initially negative but did yield an acid fast bacillus, and she had been empirically started on amoxicillin-clavulanic acid and trimethoprim-sulfamethoxazole, completing only 10 of 21 days of therapy due to gastrointestinal side effects.\nIn post hospital discharge follow up visit two weeks later, she was noticed to have again purulent drainage and was seen by Infectious Diseases and re-admitted again for debridement and wound exploration. Mycobacterium abscessus group had been identified as the acid fast bacilli (AFB) in the initial culture. Again, the patient did not have systemic signs of infection or leukocytosis. Her wound was approximately 8.0 centimeters deep and contained purulent material again.\nSurgically, extensive incision and excisional debridement of the working port site tract, which was found to extend into the pleural space, and the remaining temporary pacing wire was removed. The final wound was 7.5 cm long and 3 cm wide and 8.5 cm deep extending into the pleural space. The entire tract was removed. Surgical pathology showed acute inflammation and granulation tissue without granulomas, and AFB stains were negative.\nBlood cultures were drawn, and following debridement, wound cultures were sent. Following that, her inflammatory markers improved with C-reactive protein decreasing from 15.8 mg/L to 1 mg/L (normal value< 8 mg/L), and erythrocyte sedimentation rate decreased from 58 to 12 mm/h (normal value below 20). The wound cultures again grew acid fast bacilli, characterized as Mycobacterium abscessus group by Matrix-assisted laser desorption/ionization time-of-flight (MALDI-TOF, Mayo Clinic Laboratory, Rochester, MN). The antimicrobial resistance profile showed sensitivity to only amikacin and resistance to imipenem, doxycycline, cefoxitin, linezolid, and trimethoprim sulfamethoxazole. Test for inducible clarithromycin resistance was positive and the organism was found to be sensitive to clofazimine (done at Microbiology Laboratory at National Jewish Hospital, Denver, CO, USA).\nDue to the medication cost, potential side effects and an upcoming family wedding, the patient opted against antimicrobial therapy. Over 6 weeks, her wound completely healed. Follow up CT imaging 2 months later revealed no evidence of persistent infection. 30 months later, she continued to do well without evidence of recurrence in the absence of specific antimicrobial therapy with normal inflammatory markers.
A 48-year-old man with a history of alcohol abuse presented to the gastroenterology department for the first time in November 2006. He was suffering from severe epigastric pain and nausea for two days. Because the laboratory findings were suspicious for pancreatitis, the patient underwent computed tomography (CT) (Fig. ). The examination showed an edematous pancreas tail and fluid in the anterior prerenal space and in the anterior, posterior and lateroconal fascia. There were no signs of chronic pancreatitis or vascular complications. In the following months the patient had two similar episodes of acute tail pancreatitis.\nIn September 2009 he presented with vague epigastric pain of several weeks duration. Serum amylase and lipase levels were normal, however the cholestatic parameters were mildly elevated. Therefore, a magnetic resonance (MR) study was performed. The examination demonstrated moderate chronic pancreatitis of the tail with atrophy and irregular bording of the Wirsung’s duct. There were no other abnormalities. Further examinations also revealed a Helicobacter pylori gastritis.\nIn February 2012 the patient presented again to the gastroenterologist with epigastric and left upper quadrant pain. Laboratory findings were not suspect for acute pancreatitis. However, contrast-enhanced CT scan was performed in order to evaluate the known chronic pancreatitis and other causes of pain (Fig. ). This study surprisingly revealed a hypervascular lesion in the pancreatic tail consisting of a conglomeration of small hypervascular spots and blood vessels. Further examinations were performed in order to differentiate a vascular malformation from a hypervascular pancreatic tumor, especially an islet cell tumor or a hypervascular metastasis. Contrast-enhanced MR study showed a focal area of heterogeneous contrast enhancement without an apparent nodular component. The subsequent angiography showed two large feeding arteries in the early arterial phase, followed by a racemose vascular network, an early transient dense parenchyma stain in the early portal phase and early wash-out of the lesion in the portal phase (Fig. ). Based on these findings, a pancreatic arteriovenous malformation was suggested. The patient was treated with a resection of the pancreas tail and postoperative recovery was uneventful. Histopathologic examination revealed sequelae of chronic pancreatitis and numerous dilated blood vessels accompanied by blood clot formation and intimal hyperplasia (Fig. ). The histopathological findings were consisting with a pancreatic AVM.
A 40-year-old woman was referred to the Department of Oral and Maxillofacial Surgery with the complaint of a painless swelling in the left cheek region with duration of 8 months [ and ]. The patient revealed that she had got her left upper molars extracted 2 months prior to her presentation to us. The swelling was initially small and had gradually increased to the present size. No neurological deficit was evident on subjective and objective examination. On extraoral examination, an ill-defined diffuse swelling of 6 × 5 cm size over the left cheek region was seen extending superiorly to the infraorbital region, and inferiorly 1 cm lateral to the angle of the mouth. The swelling extended medially till the ala of the nose and laterally to the preauricular region. It was firm to palpation and was not fixed to the underlying structures. There was no associated lymph node enlargement.\nOn intraoral examination, a diffuse swelling of 6 × 5 cm in size was noted on the left buccal mucosa, which extended superiorly until the maxillary tuberosity with obliteration of the buccal sulcus in relation to left maxillary molars. The lesion was firm in consistency with a smooth surface, and the overlying mucosa was normal. The lesion was evaluated with a panoramic radiograph and subsequently with a computed tomography (CT) scan. The panoramic radiograph showed destruction of the alveolar bone corresponding to the extracted molar region. The CT scan showed a hypodense lesion involving the infratemporal fossa [–]. It was interpreted as a benign mesenchymal tumor.\nAn incisional biopsy was performed and submitted for histopathological evaluation. The report revealed interlacing fascicles of spindle cells with dark staining wavy nuclei with less prominent amorphous eosinophilic zones representing verocay bodies and perivascular hyalinization. The cellular proliferations were intermixed with large foamy histiocytes along with areas of hemorrhage and cystic degeneration [–]. Although in certain fields there were nuclear hyperchromatism and mitosis, the overall histo-architecture was consistent with a diagnosis of ancient schwannoma.\nIn keeping the indolent nature and imaging findings, surgical excision of the lesion was planned through a transantral approach to the infratemporal fossa. A Weber-Fergusson incision was made, and the maxillary bone over the lesion was exposed till the pterygoid plates. The lesion was dissected from the buccal mucosa and traced to infratemporal fossa through transantral approach and was excised in toto. The immediate postoperative period was uneventful. The surgical specimen was submitted for histopathological evaluation, which confirmed the diagnosis of ancient schwannoma [–]. The patient is periodically monitored and is disease free at the last follow-up (2 years after surgery) [ and ].
A 72-year old woman experienced numbness below her left eye. She was examined with a CT scan of the paranasal sinuses revealing a tumor in the left maxillary sinus. Subsequently, exploratory surgery was carried out with tissue sampling of the mucosa in the floor of the sinus. The tumor was histologically classified as an adenoid cystic carcinoma. Immunohistochemical staining was positive for coloring with CD117 (C-kit). This supports the classification as an adenoid cystic carcinoma. Special coloring with Alcian also showed positive and strong coloring in the stroma between the cell layers. This further supports the diagnosis adenoid cystic carcinoma. The tumor did not show perineural invasion. A microscopical image of the tumor is presented in Figure . The patient was treated with radiation therapy intended to cure.\nHer medical history included hypercholesterolemia, macular degeneration, psoriasis and a long history of Sjögren’s syndrome. Because of dry mouth and severe caries due to Sjögren’s syndrome, the patient had had all her teeth removed and she had tooth implants in her lower jaw and dentures in her upper jaw. She had smoked 10 cigarettes a day for 45 years.\nPrior to the onset of her present disease, she had worked as an auxiliary nurse at a local hospital for 31 years. For 11 years, in the period 1967–1988, she had been working exclusively in the operating theatre at the general surgical ward. Her work tasks during this period had involved the handling of biopsies and she put the tissue samples in containers filled with formaldehyde. Containers of various sizes were employed: most surgeries involved preserving smaller tissue samples, but some surgeries involved preservation of larger organs, e.g. whole breasts. She had handled tissue samples several times per day throughout the 11 year period. Larger tissue samples were handled approximately a couple of times a week. After use, our patient disposed of the formaldehyde by pouring it into the sink in the operating theatre. Her work tasks also included preparing the surgical patients by cleaning the skin with a solution of iodine in ethanol and assisting as a non-sterile-assistant at various surgeries. In addition, her work involved daily disinfection of the operating theatre after use. The disinfection was performed by spraying the walls of the theatre, a procedure lasting 1 or 2 hours, which was performed three or four times a day throughout her 11 year period of work in the surgical ward. For the rest of her career, she had worked as an auxiliary nurse in the medical ward of the hospital, without any known exposure to formaldehyde.\nDisinfectants for use in hospitals may contain formaldehyde []. Although the patient did not remember what kind of disinfectant that was used at her work-place, it is possible that the patient was exposed to formaldehyde from disinfectant in addition to the formaldehyde used for the preservation of tissue samples. Throughout her 11 years of work in the operating theatre, she did not use adequate airway protection to prevent inhalation of formaldehyde. To our knowledge, measurements of formaldehyde in her work athmosphere were never performed. Both surgeons and nurses may have been exposed to formaldehyde in the operating theatres of the hospital. However, by directly handling the formaldehyde for tissue samples and for disinfection of the operating theatre, our patient was conceivably exposed to higher levels of formaldehyde vapors than her fellow workers. Her cancer was diagnosed 45 years after the first known exposure to formaldehyde vapors.
Elisabeth was nearly 60 years old and lived with her husband when she experienced a mild stroke. She worked as a manager at the time of stroke and the organization at the workplace was in the middle of a large change. Elisabeth was included in the ReWork-Stroke programme 8.5 months after her stroke. Due to her stroke, she had difficulties with reading, writing, fatigue, and ability to concentrate. These consequences after her stroke made it difficult for her to remain in a management position, which is why another person took this role. Elisabeth enjoyed being back at the workplace again and was looking forward to her return to work. She was supported by one of her colleagues and they had a close cooperation with the OT who came to the workplace regularly.\nThe WRI scores at baseline showed that some items concerning motivation for work were interfering with the return to work, i.e., expectation of job success, commitment to work and work-related goals, and they did not improve during the work trial. The logbooks revealed that Elisabeth was unsure about her possibilities to return to work. Her concerns about how to manage work tasks were discussed several times. Strategies on how to deal with the consequences of her stroke were suggested by the OT. The other work potential factors were mostly supportive or became supportive during the work trial. The AWP showed that Elisabeth had positive changes on all motor items between baseline and follow-up and reached competent performance on all except physical energy. However, on the process items, there was no change in three of the five items, i.e., knowledge, temporal organization and adaptation, and competent performances were not achieved.\nDifferent strategies and aids were proposed and discussed during the work trial and were also successively implemented and used by Elisabeth. Strategies were, for example, tape recording of meetings to substitute for memory problems, use of a speech dictation device instead of writing minutes, writing down of main points before calling someone, use of to-do lists to get structure and support for memory, and taking small breaks to stay more alert. Technical aids that facilitated her work were installed on her computer.\nDuring the work trial, ongoing discussions were conducted to find suitable work tasks that were manageable for her and that could be paid for in the long run. For example, she tried out working with bookkeeping as numbers were easier for her to work with than words. These tasks worked out well for her but were very tiring. She also tried some of her ordinary work tasks such as planning for and leading meetings.\nIt also became evident to co-workers during the work trial that Elisabeth had some difficulties to remember information. This was an interfering factor at work as she could, for example, forget to do work tasks that should have been done before meetings.\nDiscussions about her possibility to return to her former job duties despite her aphasia continued during the work trial.\nAt the follow-up nine months after the start of the work trial, Elisabeth was still in a work trial.\nGina was a 52-year-old administrator who was included in the ReWork-Stroke programme 19 months after stroke onset. On her inclusion, she still had some physical impairment, as well as aphasia and she also described an impact on cognitive functions such as concentration.\nHer WRI scores indicated a difficulty in working out abilities and limitations and this was one area that, according to the logbook, has been central during the work trial. Together with Gina and her employer, the OT discussed and planned for work tasks, working hours, and the social environment.\nAt the end of the work trial, the WRI showed both some positive and some negative changes. Most of the positive changes were for motivational factors, while negative change occurred for lifestyle factors that addressed daily routines. The logbook indicated that work demanded a lot of energy for Gina, which affected her life outside work.\nAWP showed a positive change in motor items with the exception of physical energy, which was below a level supporting return to work at the end of the work trial. This was, as underpinned in the notes of the logbook, an indication of the balancing between her strong wish to return to work and her energy to do so. During the OT’s recurrent visits to the workplace, subjects such as the pace of expanding working hours were often on the agenda. Gina wanted to hasten the process, but her boss and the OT were helping her to take it step-by-step by evaluating and discussing her achievements.\nThe other motor and process items showed unaltered competency or change to competent performance after the work trial.\nGina was working 50% at follow-up nine months after start of the work trial.\nHenry was 50 years old when he experienced a moderate stroke. He was married, had two children, and worked as a craftsman. After his stroke, he had both physical and cognitive difficulties. His balance was affected as well as his memory and vision. Due to his vision impairment, he was not allowed to drive which was a concern when discussing returning to work and the bus option was mentioned. The time from stroke onset to inclusion in ReWork-Stroke was seven months. Henry realized that his work tasks at his former workplace were too difficult due to the consequences of his stroke. He therefore used his contacts and found another workplace where he could start his work trial.\nIt was mainly the motivational factors, lifestyle factors and environmental factors in the WRI that supported his return to work. However, according to the WRI assessment Henry was unsure about his abilities and limitations after his stroke. This was also a focus during the work trial where his boss collaborated with Henry and the OT to find suitable work tasks for him. During the work trial, he could try out both his severity of work tasks and working hours.\nThe AWP scores showed a change to competent performance on three motor items. This was an important improvement, as Henry had physically demanding work. The AWP process items were all scored as incompetent performance after three months of the work trial. Two of the items, temporal organization and organization of space/objects, had changed from competent to incompetent. Strategies to handle the consequences after the stroke at work were discussed and suggested by the OT during the three months of the work trial. Henry was recommended to take short breaks and to only focus on one task at a time. His co-workers were supportive and assisted in reminding him about taking breaks and to adjust his working pace (work slower). Henry also needed to use different strategies and techniques to reduce the impact of impaired memory, such as to-do lists and the use of a calendar, both at work and in household chores at home.\nHenry tried to work full days and physically it worked out all right. He even had the energy to walk the dog after work, but his memory problems became worse. In cooperation with the OT and the workplace, the schedule was changed so that Henry could have a day of rest between two working days. The WRI item “pursues interest”, showed that he had enhanced his ability to seize opportunities to make his life stimulating and meaningful both in and outside work.\nAt the follow-up nine months after the start of the work trial, Henry was still on work trial.
A 67-year-old woman was admitted with recurrent chest tightness for four years, progressive dyspnea for six months, and nausea for two months. The past history included hypertension for over 30 years, and diabetes and hyperlipidemia for two years. A left mastectomy for breast cancer was performed 19 years ago, followed by local radiation therapy of three months. There was no evidence of recurrent disease.\nHer physical examination revealed the blood pressure of 105/80 mmHg and the heart rate of 118 beat/min in sinus rhythm. Moist rales were audible in both lungs. The heart edge enlarged towards the left. A grade IV pan-systolic murmur could be heard at the lower left sternal edge.\nComplete right bundle branch block was shown on her electrocardiogram. Her chest X-ray showed significant cardiomegaly with interstitial edemas in both lungs. Echocardiography () showed the hypokinesis in the middle to apex sections of the left ventricle and the paradoxical septal motion in the apex while the continuous interruption in the septum between the apex and middle sections with left to right shunting was observed, and left ventricular ejection fraction (LVEF) was 34%. The echocardiography performed three years ago demonstrated mild hypertrophy of the ventricular septum, normal myocardium motion and heart chambers, and LVEF from 58% to 65% while the recent echocardiographic results indicated the hypokinetic development of her left ventricular walls and LVEF from 43% to 48%. A coronary artery angiogram showed atherosclerosis in the mid-left anterior descending artery (LAD), and the luminal stenosis of 60% compared with 50% three years ago. There were multiple coronary fistulae in the LAD draining into the left ventricle and fistulae in the end of left circumflex (LCX) and the right coronary artery (RCA), compared with small left coronary fistulae in the LAD and none in the LCX and RCA three years ago. The angiogram also displayed the left ventricular septal perforation. The single photon emission computed tomography (SPECT) carried out two years ago illustrated an irregular radionuclide distribution in the anterior, inferior, posterior and septum segments of the myocardium with no evidence of ischemia after exercise.\nThe patient was initially diagnosed and treated in accordance with coronary artery disease. However, it was unsuccessful. Her symptoms worsened and progressive heart failure developed. Cardiac resynchronization therapy was performed to improve her progressive congestive heart failure, but the beneficial effect was not sustained. An intra-aortic balloon pump (IABP) was inserted, which improved her conditions significantly. However, she proceeded to worsen after the removal of IABP. The IABP was inserted again and the patient underwent surgical repair of her ventricular septal defect.\nAt the operation, her ventricular myocardium appeared dark grey and ventricular walls were thinner. A left apical aneurysm was present, but no mass was found in the chambers. A ventricular septal defect measuring 10 mm in diameter near the apex was identified. The aneurysm was removed and the defect was patched. Myocardial biopsy was taken from her ventricle myocardium and sent for histopathological analysis. The myocardium motion was not recovered and the patient died from refractory cardiac failure one week post operatively.\nThe histopathology report of the myocardial specimen confirmed the presence of malignant spindle cell neoplasm with prominent pleomorphism (). There were signs of mitotic activities with atypical features with coarse grain chromatin, thin cytoplasm and diffuse foliated necrosis. Immunohistochemical stains were positive for vimentin, smooth muscle actin, S-100 and caldesmon, and negative for pan-cytokeratin, myoglobin, CD34 and CD 99. These findings were consistent with the diagnosis of the CL.
Our patient is a 19-year-old girl, with a long surgical history of multiple complicated abdominal surgeries. She was diagnosed with Primary Hyperoxalosis for which she underwent same setting liver and kidney transplant. A few years afterward, she was diagnosed with intestinal type diffuse large B-cell lymphoma. After that, she had an ileocecal mass that obstructs the bowel lumen and required oncological resection with right hemicolectomy and anastomosis which was further complicated by anastomotic leakage, wound dehiscence and intra-abdominal sepsis thus requiring emergency exploratory laparotomy and Hartmann's procedure with end ileostomy. The patient then underwent laparotomy, Hartmann's revision and ileostomy site closure. For the purpose of graft preservation, the patient was on long-term immunosuppressants.\nAfter more than a year from the last surgery, she developed a central abdominal bulge at the site of her laparotomy scar; she had midline incisional hernia. Due to the long history of laparotomies and multiple surgical procedures, a multidisciplinary approach was necessary, and a surgical treatment plan was set.\nAn enhanced CT scan was performed and showed thinning of the anterior abdominal wall muscles with severe atrophy of the left rectus abdominis muscle with rectus diastasis of around 10 cm distance (Figure ).\nThe injection was performed by the interventional radiologist with the patient in supine position under ultrasound guidance (Figure ). Injection sites were marked at the anterior axillary line between the costal margin and anterior superior iliac spine according to the technique described by Smoot et al. The area was prepped and draped in a sterile technique which was followed by application of local anesthesia in the form of 1% lidocaine at the skin of injection sites. Under ultrasound guidance, BTA was injected at the three sites on either side of the abdomen. The patient received a total of 300 units of BTA diluted in 150 mL of 0.9% saline with a concentration of 2 units/mL. Each of the six injection sites received a volume of 25 mL. Each of the three injection sites on either side of abdomen were used to target the external oblique, internal oblique, and transversus abdominis muscles. After the procedure, the patient recovered smoothly and was discharged home the next day to return for surgery after 3 weeks.\nAbove the iliac crest, transversus abdominis at anterior axillary line. Mid abdomen, internal oblique at mid axillary line. Below costal margin, external oblique at anterior axillary line.\nPatient went for surgery 3 weeks after Botox injections. The procedure started by infiltrating normal saline for subcutaneous hydro-dissection followed by removing the old scar at the midline. De-epithelization was continued just beneath the skin to raise it above the adherent bowel underneath until reaching the normal fascia on both sides of the abdomen. Then, the abdominal flap was raised in the subscarpal plane above the fascia. Closure of the defect and plication of the recti were done followed by placement of sized on-lay fully resorbable monofilament mesh (Figure ).\nTo further relax the lateral abdominal muscles, a total of 200 units of BTA diluted in 8 mL of normal saline were infiltrated at the same previously injected sites (Figure ). Drains were placed bilaterally above the mesh. Subcutaneous tissue was approximated with interrupted sutures in two layers. Skin was closed with 4-0 Monocryl in subcuticular fashion (Figure ). Patient had no complications and was followed up for the next 18 months with no recurrence.
A 61-year-old man who had been diagnosed with hypertension and diabetes 15 years previously was referred to our outpatient clinic for an incidental finding on ultrasonography of a right renal mass. He had the nonspecific symptom of fatigue since 12 weeks previously. No palpable mass was detected on the physical examination. The results of routine laboratory examinations and urinalysis were within normal limits. A computed tomography (CT) scan revealed a 10-cm right renal mass with areas of necrosis and suspicious perirenal fat infiltration and invasion to the right adrenal gland. Some lymph nodes were enlarged in the aortocaval area. The tumor also infiltrated the right renal vein and the IVC as a typical advanced RCC (). A diagnosis of RCC with IVC thrombus (cT3bN1M0) was made and a radical nephrectomy with vena caval thrombectomy was planned.\nUnder general anesthesia, the patient was placed in the supine position with the right side elevated by 15 degrees. A midline abdominal incision was made and a Kocher maneuver was done to expose the right kidney. The liver was retracted to expose the right renal vein and IVC. The right kidney was mobilized and a venous cannulation was performed above and below the IVC mass, and arterial cannulation was performed on the left femoral artery. The incision on the IVC was made where the renal vein was ligated after cardiopulmonary bypass. Vena caval thrombectomy was done and reconstruction was accomplished by use of nonabsorbable sutures. En bloc resection of the right renal mass and regional lymphadenectomy were performed successfully. The pathological diagnosis was invasive urothelial carcinoma, of a high grade, with sarcomatoid differentiation, which invaded the renal cortex but was limited to the capsule (, ). The IVC thrombus was composed of urothelial carcinoma of a high grade; the lymph nodes were free of carcinoma (pT4N0M0). The patient was discharged on the fourth postoperative day, and adjuvant chemotherapy with the methotrexate, vinblastine, doxorubicin, and cisplatinum regimen is scheduled.
A 5-year-old female child reported to Oral and Maxillofacial Surgery department in AJ Institute of Dental Sciences, Mangalore, with the complaint of a painless swelling in the left side of the lower jaw since 1 month. The swelling was small in size and has gradually increased to the present size. The patient presented history of abscess with draining sinus secondary to decayed tooth in the left lower back tooth 2 months back. She underwent extraction of the involved tooth and incision and drainage of the abscess. Pus was sent for culture and sensitivity test that showed no growth. General examination concluded that the patient was moderately built and minor signs such as weight loss, fever, and cough were absent. Past medical and family history was not significant. When the patient reported, there was no discharge noticed with respect to the site.\nExtra-oral examination presented a distinct diffuse enlargement with imprecise borders of nearly 3 cm × 2 cm in the left submandibular region (). The superimposing skin was the same as the surrounding skin. On palpation, a single cervical lymph node was felt in the left submandibular region, which was enlarged, firm, non-fluctuant, incompressible and firm in consistency; although, there was negative indication of tenderness to the adjoining tissues. On intraoral examination, there was odontogenic involvement noticed. A clinical diagnosis of left submandibular TB lymphadenitis was pondered. Differential judgment of left submandibular sialadenitis left submandibular gland calcification was considered.\nThe routine blood investigations were done for the patient along with peripheral smear, blood culture and C-reactive protein test; however, there was no variation identified except that erythrocyte sedimentation rate was elevated (20 mm/h). Her chest X-ray gave a normal impression. Patient was advised for ultrasound of the neck that gave the impression as submandibular sialadenitis with no obvious collection and necrotic level IB lymph node. A computed tomography scan with intravenous contrast was also instructed for her, report of which presented with an enlarged lymph node. The patient was posted for excision of the lymph node under general anesthesia. When the excised specimen was histopathologically examined, it showed lymph node with thickened capsule, infiltrated by coalescent epithelioid histiocytic granuloma with areas of central caseous necrosis (). The report gave feeling as “TB lymphadenitis”\nPatient was referred to a physician who instructed a WHO endorsed anti-tubercular therapy: Isoniazid (INH, 100 mg/day), rifampicin (RIF, 300 mg/day), pyrazinamide (400 mg/day) for 2 months and INH (80 mg/day) and RIF (150 mg/day) for another 4 months. This anti-tubercular therapeutic regimen was administered for 4 months, and follow-up showed complete resolution of the lesion, thereby signifying a successful outcome.
In this peculiar case, a 59-year-old woman with dyspnea, severe mitral regurgitation, and atrial fibrillation was scheduled for elective mitral valve replacement (MVR). Coronary angiography showed mild atherosclerosis of coronary vessels without significant lesions. The right femoral artery was cannulated directly with a 19 French cannula (Medtronic DLP femoral) because right mini-thoracotomy access to the mitral valve was planned. After the opening of the chest, a bluish color of ascending aorta was noticed, and a type A AD was suspected, which was confirmed by transesophageal echocardiography. Management of AD with retrograde extension from entry tear in the descending aorta is controversial, especially when the false lumen of the ascending aorta is completely thrombosed in patients who are often clinically stable. In selected patients, a more conservative approach consisting of initial medical management with timely surgical repair gave excellent outcomes[.\nTherefore, the surgery was continued converting the procedure in median sternotomy, the right subclavian artery was directly cannulated, and antegrade extracorporeal circulation (ECC) was initiated with good flow and pressures in the cannula maintaining a deep hypothermia until 24°C. MVR with a mechanical Bicarbon mitral valve prosthesis (n. 29) was performed, and cardiopulmonary bypass (CPB) was terminated without any significant event. In early postoperative period, a conservative medical approach based on hemodynamic stability of the patient was planned. A computed tomography scan performed in the 7th, 14th, and 19th postoperative days revealed persisting type A acute AD with false lumen of ascending aorta without thrombosis. Based on this evidence, the surgical team changed the strategy towards a surgical approach. The patient successfully underwent surgery with a simple tube graft ascending aorta and hemiarch replacement and venous grafts to the right coronary artery because of suspected extension of dissection in right coronary sinus. The patient was discharged on the 15th postoperative day with a regular postoperative course; her last echocardiography findings before discharge showed moderate left ventricular dysfunction (ejection fraction: 40%).\nOne month later, she was urgently readmitted for onset of acute dyspnea, chest pain, and worsening of ejection fraction rated with echocardiography. Evaluation by cardiac catheterization revealed spontaneous dissection of left anterior descending coronary artery (LAD), occlusion of right coronary artery, and venous graft. The patient was successfully treated with coronary angioplasty and stents on LAD (), and a dual chamber implantable cardioverter defibrillator was implanted for primary prevention. She was discharged in good health, and the one-year follow-up was free of other important events.
In May 2008, 72 year old female was diagnosed of small cell lung cancer incidentally after she was hospitalized for anxiety and dizziness. She was hyponatremic and series of tests which included a CT scan of the chest which showed right hilar lymphadenopathy and mediastinal lymphadenopathy (Figure ). She then had a mediastinoscopy which was consistent with small cell lung cancer. PET/CT scan findings were consistent with CT scan. She was staged as limited stage small cell carcinoma of lung. She was started treatment with etopside and carboplatin. In July2008, after receiving two cycles of chemotherapy, she presented with new onset memory difficulties.\nFurther work-up included a MRI of brain with and without gadolinium as well as PET scan showed no evidence of metastatic disease to the brain. These symptoms were thought due to underlying anxiety or depression.\nFew days later she started with ataxia, vertigo and hearing loss. She was prescribed meclizine which provided no relief. She then went to emergency department. At that time she was found to orthostatic. She was rehydrated and discharged.\nAudiogram was done which revealed bilateral sensorineural hearing loss greater on the left side. The paraneoplastic workup was sent to rule out paraneoplastic syndrome. The anti-Hu antibody came positive. The diagnosis of anti-Hu syndrome was entertained. She was started on radiation therapy in addition to her chemotherapy for consolidation. She received 3960 cGy of radiation to the mediastinum in August 2008.\nThree weeks later, she began developing loss of smell sensation to the point that she was completely anosmic. Her unsteadiness reached to the point that she needed assistance for walking short distance. MRI of brain showed new area of edema and inflammation in the right medial temporal lobe as well as enhancement in the underlying meningi (Figure ). She was admitted for to the hospital for empiric IV acyclovir therapy for possible herpes encephalitis. The Lumber puncture was done which showed increased proteins (Total proteins 5.7 g/l, Beta globulin of 0.73 g/l) and lymphocytes. The CSF HSV/PCR was negative. Patient was discharged in the 6 th day from the hospital and received total 10 days of IV acyclovir as she improved on the treatment. Patient is now planned for further chemoradiation therapy.\nThe neurocognitive deficits in our patient were probably because of either anti-Hu syndrome or Herpes encephalitis.\nThe anti HU syndrome is a neurologic paraneoplastic syndrome. A specific anti body called anti HU is present in serum and CSF of these patients []. Patients with anti-Hu antibodies usually have SCLC (94%), multifocal neurological symptoms and a poor prognosis []. Anti-Hu encephalomyelitis is one of the most frequent paraneoplastic syndromes, classically presenting with diffuse neurological involvement []. Anti-Hu antibodies are present in about 50% of patients with limbic encephalitis and lung cancer and is characterized by marked reduction in short term memory and sub acute confusion. Many patients may also develop rapid pancerebellar dysfunction due to extensive purkinjee neuronal loss termed as sub acute cerebellar ataxia. The HU-Abs is reported to be present in about 23% of these patients. The Anti-Hu antibodies, in addition to SCLC are associated with prostate cancer and neuroblastoma [].\nAnti-HU antibodies are also associated with Encephalomyelitis, sensory neuropathy, and autonomic neuropathy. The limbic encephalitis and sub acute cerebellar degeneration are associated relatively often with cancer and are called "classical" PNS []. The treatment of PNS is control of the tumor. Antitumor treatment has established to stop paraneoplastic neurological deterioration. However, in debilitated patients treatment of underlying tumor is withheld because of the little chance of clinically relevant neurological improvement [].\nHerpes simplex encephalitis patients may have a prodrome of malaise, fever, headache, and nausea that is followed by acute or sub acute onset of an encephalopathy whose symptoms include lethargy, Confusion, and delirium. Patients may also have seizures, aphasia, and other focal neurological deficits. The herpes encephalitis is fatal in 7 to 14 days if not treated and mortality as high as 70% in untreated cases. Neurology consultation should be sought as HSV encephalitis is neurologic emergency and treatment should be started with acyclovir in suspicious cases or when diagnosis is established. [] In our case, the patient developed anosmia and that can be present in 65% of patients of HSV encephalitis []. However, PCR of spinal fluid for HSV was negative. PCR itself has a reported sensitivity of 98% and a specificity of 94% for the detection of HSV in the CSF [].\nFalse negative results may occur: []\n1. Occasionally in bloody specimens through inhibition of PCR\n2. When CSF has been obtained after starting Acyclovir.\n3. When lumbar puncture has been performed early in disease.\n4. When lumbar puncture was delayed approximately 10 days or more into the disease process\nSince a negative HSV PCR result does not entirely rule out the possibility of HSE, an intrathecal antibody response specific to HSV should also be sought [].\nAlthough we did not prove the agent microbiologically but the MRI changes and Clinical improvement, elevated proteins and lymphocytes in CSF points towards HSV encephalitis. Our next step is try to get diagnosis retrospectively by measuring serial serum and CSF titers although it has no role in acute diagnosis and treatment [].
A 91-year-old man presented to our hospital with a chief complaint of anal pain for 3 years. In another hospital, Candida infection was discovered in the anal skin of the patients, and ketoconazole was topically applied once a day. The patient was diagnosed with a node and erythema in the anal area. His medical history included anxiety disorder and chronic gastritis, and his medication history revealed that he had been on a gastric mucosa protectant for at least one year. His family medical history was not significant.\nFour years ago, the patient became aware of rashes in the anal area and received treatment at a nearby clinic. As there was no improvement, the patient was referred to the Department of Dermatology at our hospital. A biopsy of the node around the anus showed atypical cells developing as Paget's disease in the epidermis. Immunohistochemical analysis showed cytokeratin 7 (CK7) positivity, cytokeratin 20 (CK20) positivity, and gross cystic disease fluid protein 15 (GCDFP15) negativity. Tumor invasion with PS from the anus to the skin was suspected, and the patient was referred to our department for a close examination and surgical treatment.\nThe patient was 159 cm tall and weighed 47.8 kg, with a body mass index of 18.9 kg/m2. Erythema (8 cm × 6 cm) was accompanied by erosion around the anus. No mass was detected in rectal examination. There were no swollen lymph nodes on either side of the groin. Laboratory blood data on admission were not significant. Regarding contrast-enhanced abdominal CT findings, in the early phase, contrast enhancement was observed in the skin around the anus, but there was no contributory finding in the intestinal tract between the anal canal and anal opening (). Neither swelling of the inguinal lymph node nor distant metastasis was observed in the peritoneal cavity. Pelvic MRI did not reveal a clear mass or swollen lymph node in the anal area. Upper gastrointestinal endoscopy was noncontributory. Lower gastrointestinal endoscopy revealed no protruding lesion in the area between the anal canal and rectum. The mucosa of the anal canal was edematous and hemorrhagic (). Redness and swelling with white moss were observed in the anus (). Biopsy showed mammary/tubular adenocarcinoma and squamous cell carcinoma with atypical epithelium, with a tendency for keratinization in a sheet-like manner in the anal canal. In the anus, an atypical stratified squamous epithelium continuing to the outer mucosal epithelial layer grew to the funiculi in a sheet-like manner. Pagetoid cells were diffusely present in the epithelium, and an image of squamous cell carcinoma directly under the epithelium was obtained.\nTo cure the invasion of the skin by anal canal cancer with PS and squamous cell carcinoma of the skin, we performed laparoscopic abdominoperineal resection, lymphadenectomy, and skin carcinoma resection in the perineum. Neither lateral lymph node dissection nor inguinal lymph node dissection was performed. The skin around the anus was resected approximately 1.5 cm outside the erythema, the skin subcutaneous adipose tissue of the anus was removed, and the wound was closed without a skin flap.\nFor pathological examination, the resected specimens were used after formalin fixation. There was a 92 mm × 48 mm tumor accompanied by slightly protruding white moss in the entire circumference of the anus with a clear boundary between the tumor and the surrounding healthy epidermis ().\nThe histological findings revealed that an adenocarcinoma invaded the external anal sphincter and subcutaneous adipose tissue in the vicinity of the pectinate line of the anal canal (). Pagetoid spread of adenocarcinoma from the anal gland was observed in the epidermis. Pagetoid cells, which are large clear cells, grew in simplex and alveolar patterns in the epidermis. The open portion of the anal canal was slightly invaded up to the rectal mucosa (). The anal adenocarcinoma skin region partially continued to the hair follicles and was complicated by squamous cell carcinoma invading the dermis ().\nImmunohistochemical analysis of the resected tumor around the anus revealed that the adenocarcinoma was positive for CK7, Cytokeratin 19 (CK19), CK20, and CDX2 (Figures –), partially positive for MUC5A (), and negative for GCDFP15 (). Squamous cell carcinoma was positive for p63 () and CK5/6 () in all layers from the fundus to the surface layer.\nAlthough the patient developed ileus after surgery, he improved and was discharged on postoperative day 37. The patient did not require postoperative chemotherapy. He was an outpatient at our department and was being followed up. One year later, multiple liver metastases, lung metastases, and inguinal lymph node metastases were observed. He received radiation therapy (20 Gy/5 fractions) to relieve inguinal pain and pain associated with lower leg edema due to inguinal lymph node metastases. Unfortunately, the patient died 1 year and 6 months after surgery without local recurrence in the perianal area.
A 15-year-old boy presented with slowly progressive, bilateral weakness of the legs that had been present since beginning school. His pre- and perinatal history was unremarkable. Motor milestones were not delayed. He could not run and had experienced difficulty in climbing stairs since childhood. At the time of presentation he had difficulty in arising from a chair. A physical examination revealed diffuse muscle atrophy, especially in the lower extremities. An examination of muscle power disclosed a predominant proximal leg weakness with mild weakness in the bilateral upper extremities and distal legs. The deep tendon reflexes were preserved and there was no facial weakness or skeletal deformities. Serum creatine kinase was within normal limits (106 U/L). Needle electromyography disclosed active myopathic patterns in his limb muscles. Spine roentgenography indicated that scoliosis was not present. The findings of muscle CT and the pulmonary function test were normal.\nWith regard to family history (), the patient's mother had experienced developmental delay since infancy and sustained mild, nonprogressive proximal leg weakness. She had difficulty in climbing stairs and running. The mother reported that the proband's older sister also had mild, nonprogressive proximal leg weakness and developmental delay. His mother exhibited only mild limb muscle weakness in the proximal legs, without weakness in facial or upper-limb muscles. None of the family members had previously undergone surgery requiring general anesthesia. Muscle biopsy samples taken from the biceps brachii in the proband revealed many peripheral cores with unclear borders in the muscle fibers that were devoid of enzyme activities on nicotinamide dehydrogenase-tetrazolium reductase (NADH-TR) and cytochrome C oxidase (COX) staining. Almost all cores were singular within a muscle fiber, with two cores present in only a few fibers. In addition, all of the muscle fibers were type 1 fibers. Electron microscopy revealed misalignment of myofibrils in the core region compared with those within intact fibers ().\nSince screening the entire RYR1 coding region in CCD patients is impractical due to the size of the gene (>159 kb), mutation screening was first focused on the mutational hotspots. Since most of the dominant mutations have been identified in the C-terminal region, including exons 101, 102, and 103 of RYR1,,, these exons were first amplified through PCR. This genetic analysis revealed a novel heterozygous mutation [c.14590T>C (p.Tyr4864His)] in exon 101 (). This mutation was not detected in 100 control chromosomes by direct sequencing, and the predicted mutant amino acid tyrosine was highly conserved in the RYR1 protein between different species.
A 42-year-old male reported to a dental clinic immediately following a trauma due to domestic violence. Careful examination revealed no other injury except the fracture of the upper front teeth. The crown portion of the maxillary left central incisor had been fractured and had not been retained by the patient. Clinical examination revealed the presence of a horizontal fracture at the cervical level. The right central incisor had complicated vertical and oblique fractures, and the fragments were retained, attached to the soft tissues (). Exposed pulp was examined clinically and was confirmed radiographically (). Hence, root canal treatment was planned. The presence of normal occlusion was confirmed. The fractured fragment of the right central incisor was removed (). Normal root condition was assessed with preoperative radiographs.\nFollowing radiographic assessment, the following treatment plan was presented to the patient:\nEndodontic treatment and reattachment of the fractured tooth fragment of the left central incisor;\nA post and core treatment followed by full veneer restorations of the right central incisor.\nThe treatment plan was decided as follows: the completion of endodontic treatment of both central incisors, the reattachment of the fractured fragment of the left central incisor, and the preparation of a post space followed by the cementation of the post and the core build-up of the right central incisor. The fractured fragment from the left central incisor was placed in 5% sodium hypochlorite for two minutes to clear the pulp tissue remnants and was stored in normal saline before the commencement of the treatment.\nThe endodontic treatment was performed on both incisors. Following isolation using a rubber dam, an endodontic access opening was made under the magnification and illumination of a dental operating microscope (8x, Global Surgical Corporation, St. Louis MO, USA); using an endo-access bur (Dentsply Maillefer, USA), conventional access was prepared. Then patency was checked using 10 K-files (Kerr USA), and the working length was determined by using an electronic apex locator Root ZX mini (J. Morita MFG. Corporation, Kyoto, Japan) and a radiograph with an endodontic file inserted in the canal.. The root canals were cleaned and shaped by a Rotary Ni-Ti Protaper system (Dentsply Maillefer, USA) along with Glyde (Dentsply Maillefer, USA), using a crown down technique. The root canals were copiously irrigated with 5.2% sodium hypochlorite and 17% EDTA solution during the preparation. Following this, a final irrigation was done with a 2% chlorhexidine solution (V-concept, Vishal Dentocare, India). The root canals were dried using paper points. Before obturation, master points were seated to test their suitability to canals, and a radiograph was taken.\nBoth maxillary central incisors were obturated with the selected master gutta-percha cone (Protaper, Dentsply Maillefer) and sealer (AH-Plus, Dentsply, Maillefer, USA). The coronal gutta-percha cones were seared off using a heated instrument, and vertical compaction was done using heated pluggers at the individual canal orifices.\nReattachment of the fractured tooth fragment of the left central incisor was carried out using a dual-cure composite resin cement (Multi-Link Automix, Ivoclar Vivadent AG, Schaan/Liechtenstein), and the crown of the restored tooth was created using Luxacore (DMG Dental Avenue India) ().\nA post space was prepared in the radicular portion of the right central incisor using the glass fiber FRC Postec Plus system (FRC Postec Plus Ivoclar Vivadent AG, Schaan/Liechtenstein). An appropriately sized post was cemented using dual-cure composite resin cement (Multi-Link Automix, Ivoclar Vivadent AG, Schaan/Liechtenstein) () .The core build-up was subsequently done using Luxacore (DMG Dental Avenue India), a dual cured core build-up material.\nThe post and core were checked for the absence of mobility, and a radiograph was taken (). The restoration after final finishing was checked for occlusal harmony (). The complete procedure was carried out in a single visit, as the patient demanded immediate esthetics due to some social commitment later on the same day.\nAt a follow-up visit after five years and four months, both the incisors were firm, and the treatment outcome was found to be successful ().
A 68-year-old man presented to the emergency department of our hospital with a 6-day history of left hemiparesis and 1-day history of fever. The patient had a history of advanced non-small-cell lung cancer and was receiving 3-week cycles of pembrolizumab (200 mg) for approximately 4 months before admission. Initial cranial CT and MRI showed two lesions in the left frontal and right parietal lobes. Both lesions were <2 cm in size and exhibited homogeneous hyperintensity on diffusion-weighted MRI [-]. The primary consideration in this case was brain abscess. Due to the small size of the lesion, neurosurgical aspiration was planned in case the causative pathogen could not be identified by blood culture or treated by antibiotics.\nAfter blood cultures were obtained, empirical treatment with intravenous meropenem administration was initiated. Levetiracetam was started for seizure prophylaxis. Although the causative pathogen was not identified by blood culture, the lesion sizes reduced remarkably following antibiotic treatment, and left hemiparesis was improved. Therefore, neurosurgical aspiration was not performed. However, his left hemiparesis worsened 7 weeks after treatment initiation. Cranial MRI revealed that the mass lesion in the right frontal lobe was slightly enlarged and the surrounding vasogenic edema was exacerbated []. Blood cultures were taken again, and then neurosurgical aspiration was performed. Stereotactic needle aspiration of the lesion in the right frontal lobe yielded purulent discharge, leading to a confirmed diagnosis of brain abscesses for both lesions. S. intermedius growth was detected only in the purulent fluid culture and not in the blood cultures. There was a considerable reduction in the lesion sizes after surgical drainage and antibiotic treatment based on antibiotic-sensitivity test results. The origin of the brain abscesses could not be identified even after several examinations, including intraoral examination and panoramic radiography []. However, during treatment for lung cancer, the patient had undergone tooth extraction of the right mandibular second molar harboring apical periodontitis approximately 3 months before admission. Because S. intermedius, an indigenous oral bacterial species, was detected by the purulent fluid culture, oral infection or dental manipulation was considered as the possible cause of brain abscesses.\nAt 2 months after the surgery, the left hemiparesis had disappeared. Approximately 4 months after the surgery, the lesions had disappeared and the surrounding vasogenic edema had partially improved on the non-contrast MRI [ and ]. Cancer treatment had to be interrupted for several months to treat the brain abscesses, and the patient’s medical condition worsened rapidly due to cancer progression. Approximately 8 months after the surgery, pembrolizumab was administered for 2 months, followed by chemotherapy for 1 month due to a temporary improvement in his medical condition. However, he died 14 months after the surgery, unable to control the lung cancer that had grown rapidly when cancer treatment was interrupted.\nNone of the two patients had sinusitis, ear infection, or recent head trauma or surgery. Echocardiography was negative for cardiac diseases, including endocarditis and congenital heart defects. In addition, no infectious foci were found on the CT of the chest, abdomen, and pelvis.
The patient was a 25-year-old male who was admitted to our hospital (Henan Provincial Chest Hospital) in August 2019 with “a mass in the right lung, which had persisted for more than 10 years and had chest tightness as well as shortness of breath for more than 1 month.” In July 2009, the patient presented in a local hospital due to “fatigue” and chest Computed Tomography (CT) showed a shadow of multiple masses in the right lung, as shown in , but he was never treated. In March 2013, due to physical examination, the patient underwent a second chest CT () which revealed a larger shadow of masses in the right lung, compared to the one seen 4 years before. Additionally, CT-guided lung biopsy revealed alveolar epithelial cell hyperplasia and local tissue papillary hyperplasia, indicating possible diagnosis of PSP. The attending doctor then recommended a right pneumonectomy, but the patient refused to receive any further treatment. In July 2019, the patient experienced chest tightness and shortness of breath, accompanied by coughing and hemoptysis. Re-examination through chest CT (, ) revealed a large irregular mass in the right lung, with calcification and some low-density areas. Moreover, injection of the contrast medium showed uneven enhancement and multiple nodules of varying sizes surrounding the mass in the lung. The superior vena cava, some branches of the right pulmonary artery and the right pulmonary vein were also all invaded. Notably, the lesions were larger, compared to those observed in the chest CT performed in 2013. Furthermore, bronchoscopy revealed that the bronchial openings in the upper, middle, and lower lobes of the right lung were narrowed and fish-like tissues were seen at the opening of the dorsal section of the right lung, blocking the bronchial lumen. Thereafter, a biopsy of the diseased tissue was obtained and microscopic examination revealed a mass of atypical cells. Additionally, immunohistochemistry analysis suggested papillary adenoma and the patient was advised to have a larger tissue biopsy.\nAlthough the chest CT suggested that the patient most likely had a malignant tumor in the lung, a clear pathological diagnosis was not made even after several medical examinations. Subsequently, a CT-guided lung biopsy was performed, where the tumor tissue was punctured multiple times at different tumor sites and the pathological diagnosis was confirmed to be PSP (). It is noteworthy that these pathological findings were confirmed by different chief pathologists, in order to further verify the diagnosis.\nAdditionally, Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography (18F-FDG PET/CT) examination during hospitalization showed a malignant tumor in the right lung, with multiple metastases in the hilar and mediastinal lymph nodes, several bones, liver, and intra-abdominal lymph nodes ().\nColor Doppler echocardiography also revealed mild pulmonary hypertension (systolic pressure 43 mmHg, simplified Bernoulli equation) and pericardial effusion, about 10 mm from the top of the right atrium.\nLaboratory examination revealed that the level of Hemoglobin (HGB) was 84 g/L (120-160 g/L), the Red Blood Cell (RBC) count was 4.21 × 1012/L (4-5.5/L), Hematocrit (HCT) was 0.285 (0.36-0.5), white blood cell count was 8.6 × 109/L (4 ~ 10 × 109/L) and the percentage of neutrophils was 76.3% (50 ~ 70%). In addition, the concentration of glutamic pyruvic transaminase was (ALT) 41.2 U/L (0 ~ 40 U/L), that of glutamic oxaloacetic transaminase was (AST) 16.5 U/L (0 ~ 40 U/L), Total Protein (TP) concentration was 56.8 g/L (60.0 ~ 85.0 g/L), albumin concentration was (ALB) 21.6 g/L (35.0 ~ 55.0 g/L) and Globulin (GLOB) concentration was 35.2 g/L (20.0 ~ 35.0 g/L). Moreover, the patient had normal concentrations of tumor markers such as the Prostate Specific Antigen (PSA), Carcinoembryonic Antigen (CEA), Alpha-fetoprotein (AFP), cytokeratin 19 fragment, Neuron-specific Enolase (NSE), and Carbohydrate Antigen 125 (CA125).\nMoreover, whole-exome sequencing was performed on the patient's PSP tumor tissue and the matched normal sample from the peripheral blood was analyzed through the Switching Mechanism at the 5' end of RNA Transcripts (SMART) method. Notably, activation of the AKT1- p.E17K pathway was observed in 31 genes (AKT1, ALK, APC, ATM, BRAF, BRCA1, BRCA2, EGFR, ERBB2, FGFR1, FGFR2, FGFR3, HRAS, KIT, KRAS, MAP2K1, MAP2K2, MET, MTOR, NRAS, NTRK1, NTRK3, PDGFRA, PIK3CA, PTEN, RET, ROS1, SMAD4, TP53, TSC1, and TSC2).\nAfter hospitalization, physical examination showed that the patient was emaciated, had poor mental health, assumed a semi-recumbent position and had mild shortness of breath. The patient's temperature was 36.6°C, his pulse was 108 bpm, respiratory rate was 30 breaths per minute and blood pressure was 128/71 mmHg. In addition, a 15 cm surgical incision scar was seen on the right thorax and the chest cavity was symmetrical without deformity. Lung auscultation also revealed no respiratory sound on the right lung and the left lung was normal.\nNotably, the patient had undergone “right pulmonary bulla resection” surgery for the right pneumothorax, 19 years earlier and had no history of other diseases.\nFurthermore, the patient was diagnosed with: (1) Malignant PSP of the right lung with multiple metastases in the blood vessels, mediastinum, liver, bone, and celiac lymph nodes; (2) Hypoproteinemia; (3) Anemia; (4) Pulmonary hypertension; and (5) Pericardial effusion.\nAfter admission, the patient was placed on oxygen, administered with supplements and received supportive treatment. His symptoms improved slightly following this treatment. However, his physical condition was not suitable for surgical treatment and he chose to leave the hospital. Six months later, the patient died of breathing difficulties and cachexia.
The patient was a 60-year-old man who was admitted to our hospital for an emergency with severe abdominal pain. He did not take any regular medication. He had smoked a packet of cigarettes a day for 10 years and was a social drinker. He had no allergy and no family medical history. The enhanced contrast abdominal computed tomography (CT) showed a massive retroperitoneal hematoma around pancreas and a PDA aneurysm on pancreas head (A). A sagittal view of the CT angiography revealed extrinsic compression of the root of the CA by the MAL (B). His diagnosis was determined as MALS with a ruptured PDA aneurysm. His vital signs were within normal limits, therefore, we performed endovascular treatment for a ruptured PDA aneurysm at first. The angiography of the CA showed no antegrade blood flow to the proper hepatic artery (A), and that of the SMA revealed the retrograde flow via the pancreaticoduodenal arcade in addition to the replaced right hepatic artery (B). The ruptured PDA aneurysm was treated by transcatheter arterial coil embolization (C, D). 5 days after the treatment, he was discharged with no complication. Although he had no symptom caused by MALS after the treatment for a ruptured PDA aneurysm, there was always a risk of reformation of the PDA aneurysms. Therefore, we planned to perform elective laparoscopic treatment for MALS without ganglionectomy of the celiac plexus in the hybrid operation room to check blood flow of the CA intraoperatively.\nThe procedure was as follows: The patient was placed in the supine position with a 30 degree both legs opened. An open method was used to insert a 12-mm umbilical camera port, two 5-mm ports in the left upper abdominal, and a 10-mm and a 5-mm port in the right upper abdomen. After liver retraction, a Harmonic Scalpel®︎ (ETHICON, Tokyo, Japan) was used to divide the avascular region of the lesser omentum, and the left gastric artery (LGA) was identified on the suprapancreatic surface (A). While retracting the LGA, the anterior aspect of the aorta was divided adequately (B). The MAL was identified as a musculoaponeurotic band crossing over the anterior surface of the CA (C). The MAL was resected and the root of the CA was exposed sufficiently, and the celiac plexus was preserved as much as possible (D). Immediately after the MAL release without ganglionectomy of the celiac plexus, the angiography of the CA was performed to check blood flow. Unlike previous angiography, the antegrade blood flow to the proper hepatic artery was clearly depicted (A). The surgical operator had 18 years’ operative experience of general surgery and the duration of surgery was 183 min.\nHis postoperative course was uneventful and he was discharged on postoperative day 5. The follow-up CT performed 12 months after the surgery revealed no residual CA stenosis (B). He was satisfied with his current condition and had no complaint about our treatment.
A 49-year-old Caucasian female presented to the emergency department with abrupt-onset pain in the left flank, radiating to the left inguinal region. She had no concomitant diseases and no known history of trauma, anticoagulation, or bleeding diathesis. Physical examination revealed a mildly obese female with severe tenderness in the left flank and left upper quadrant. The patient was afebrile with normal blood pressure and without tachycardia. The patient's hemoglobin was 136 g/L, creatinine was 68 μmol/L, and the estimated glomerular filtration rate was 88 mL/min. Urine sediment was normal. Abdominal ultrasound examination revealed a large retroperitoneal mass around the left kidney with heterogeneous echogenicity of the lower pole of the left kidney. For further evaluation, computed tomography (CT) scan of the abdomen and pelvis with intravenous contrast was performed, revealing a large left perinephric hematoma that distended the renal fascia and displaced the peritoneal and retroperitoneal structures. The estimated size of the hematoma was 15 cm in the cephalocaudal, 4 cm in the anteroposterior, and 7 cm in the transverse dimensions. In addition, a hypodense mass without calcification was detected in the lower pole of the left kidney that was poorly marginated from the perinephric hematoma (). These findings were highly suggestive of the rupture of the angiomyolipoma of the left kidney with massive retroperitoneal hemorrhage, a condition known as WS. Because the patient was hemodynamically stable without significant changes in the hemoglobin level, conservative treatment was chosen, including the monitoring of vital signs (blood pressure and pulse), blood testing (hemoglobin and creatinine levels), and repeated ultrasound scans. She was initially administered intravenous fluids, after which she was placed on an oral diet and prescribed a broad-spectrum antibiotic. The patient remained stable throughout the hospital stay and was discharged ten days after admission. Follow-up examinations (including assessments of the hemoglobin and creatinine levels, ultrasound of the kidney, and CT scan) were performed 3 and 6 months after the initial presentation, revealing the complete morphological recovery of the left kidney with complete resolution of the perinephric hematoma (). After five years, a similar clinical scenario was observed, but on the right side (). The same diagnostic pathway with conservative management was performed with the complete resolution of the hematoma (). Now, 10 years later, the patient has normal renal function without retroperitoneal hematoma. On ultrasound, both kidneys appear normal with two AMLs (3 cm on the left and 4 cm on the right sides). Because the renal AMLs are bilateral and the patient has experienced episodes of bleeding on both sides, she is educated about the possibility of repeated bleeding. She has also been informed to report immediately in the case of sudden abdominal pain. At this time, our patient has refused any proposed surgical procedures (nephron-sparing surgery).
A 44-year-old female with a chronic perianal fistula presented to the clinic with a left lateral perianal mass associated with perianal bleeding, discharge and pain. The fistula tract was biopsied in 2017 and showed detached pieces of squamous epithelium and endocervical gland tissue in a background of acute inflammatory exudate. Subsequent magnetic resonance imaging (MRI) of the pelvis showed extensive subcutaneousinfiltrative changes with the fistula extending from the left inguinal crease to the anus.\nA computed tomography (CT) of pelvis with contrast in 2019 showed an 8 × 7.3 × 5.6 cm lobulated, hypodense structure within the subcutaneous tissues along the left gluteal cleft, inseparable from the left levator ani muscles and was reported to be of an unclear etiology. A MRI with and without contrast showed a 7.3 × 7.3 × 5.8 cm structure with peripheral rim enhancement within the subcutaneous tissues of the left gluteal cleft abutting the skin surface with intermediate T1 signal and heterogeneous but overall increased T2 signal. This mass was reported to be suspicious of an area of infected granulation tissue given the rim enhancement. Two fistulous tracts were visualized on MRI measuring 6 and 3 mm, respectively. Bilateral enlarged lymph nodes were identified as 1.7 cm in the short axis along with a mildly enlarged external iliac node measured as 1 cm in short axis. Colonoscopy was performed to rule out occult inflammatory bowel disease and the entire colon appeared normal.\nSurgery was scheduled to excise the suspected inflammatory tissue. An elliptical incision was performed around the perianal mass until healthy tissue was encountered. A small portion of the suspected inflammatory tissue was directly budding the medial portion of the anal sphincter and was left in place to maintain continence.\nMicroscopic examination of the excised mass revealed skin infiltrated by a neoplasm with extensive extracellular mucin pools. Within the mucin pools, there were clusters and strips of malignant intestinal type epithelium. These morphologic features were diagnostic of a moderately differentiated mucinous adenocarcinoma (). The immunohistochemical stains showed that tumor cells were positive for CK7, CK20 and CDX2 while negative for CK5/6 and PAX8, supporting an intestinal phenotype ().\nBilateral excisional inguinal lymph node biopsy was performed, and both were positive for mucinous adenocarcinoma. Staging CT of the chest, abdomen and pelvis showed two nonspecific adjacent 3 mm left lower lobe pulmonary nodules and two sub-centimeter hypodense lesions in the right lobe of the liver that were too small to accurately characterize. Metastatic disease could not be entirely excluded.
A 54-year-old male with history of anemia and rheumatoid arthritis had noted increased lower extremity edema, chronic cough, and shortness of breath for 3 months, at which point his rheumatologist treated him for pneumonia. With symptoms worsening, the patient visited a primary care physician, as his father had a history of coronary artery disease, colon cancer, and multiple basal cell skin cancers. The patient was then referred for a transthoracic echocardiogram (TTE), which revealed a large right atrial mass with reduced ejection fraction of 40% along with incidental lesions in the liver. A cardiac MRI and MRI of the abdomen/pelvis confirmed these results, revealing a lobulated right atrial mass measuring 5.4 cm × 5.3 cm with inferior vena cava (IVC) compression and adjacent multiple large liver lesions, the largest of which measured 6.6 cm × 7.0 cm × 7.3 cm. The cardiac mass appeared to extend through the right atrial wall and into the pericardium. The patient's abdominal MRI showed mass effect on the bile duct with central biliary duct dilatation as well as mass effect on the hepatic portal veins. Mass effect was also seen on the first portion of the duodenum causing gastric distention. After completion of both MRIs, subsequent ultrasound-guided liver biopsy returned the results of melanoma. Further workup revealed no cutaneous melanoma lesions. An eye examination was performed in order to exclude ocular melanoma. Based on the recent diagnosis of melanoma, the patient was scheduled to meet with his oncologist 4 weeks later in order to discuss treatment options. Three days prior to the patient's scheduled visit with his oncologist, the patient presented to the emergency department (ED) with worsening dyspnea and lower extremity edema. PET/CT imaging using F-18 fluorodeoxyglucose (FDG) was conducted for tumor anatomical localization. The scans displayed hypermetabolic masses within the right atrium and liver that are consistent with metastatic disease, as well as bilateral pleural effusions, likely secondary to resultant heart failure (). Preoperative coronary angiogram demonstrated perfusion to the mass by a dense network of neovasculature arising from the mid right coronary artery (). The cardiac mass was surgically removed, and the right atrium was reconstructed with a pericardial patch (). Pathologic analysis of the mass confirmed melanoma. After surgery, all cardiac chambers appeared normal in size and function with associated moderate tricuspid regurgitation. Beginning one month after surgery, the patient was treated for systemic therapy with four cycles of ipilimumab, 3 mg/kg every three weeks. He subsequently developed autoimmune nephritis and a significant nephrotic syndrome as well as anasarca but recovered with pulse steroid therapy within four weeks. He had an objective response to ipilimumab in the liver and no recurrence in the heart; however, he developed progression into the peritoneal cavity, for which he is now being treated with pembrolizumab, 3 mg/kg every three weeks.
A 12 year old boy presented with left ankle swelling and pain. He sustained left ankle sprain while playing at school. On examination, active ankle sprain dorsiflexion toe dorsiflexion and eversion were normal and there was no sensory impairment as well. Only left toe extension impairment was noted. The ultrasound was done to evaluate the soft tissue injury of the ankle. Initially, we focused on the ankle area for a sprain. When comparing the bilateral peroneus longus muscles, we found the loss of normal muscular echogenic appearance over the entire left side. Tracing this back to the origin of peroneus muscle, we found a hypoechoic cyst with a well demarcated margin neighboring the left peroneal nerve just posterior to the fibula head []. Lower limb nerve conduction and electromyography study for peroneal nerve were done. The left common peroneal nerve injury over the fibular head area was found (conduction velocity: 37 ms, normal value: More than 41 ms; peak amplitude: 1.7 mV, normal value: More than 2.0 mV in nerve conduction velocity (NCV) study; only increased polyphasic motor unit action potential was found in electromyography study). In order to more thoroughly evaluate the origin of the mass lesion and adjacent structure, a knee MRI was done. The MRI showed a 33 mm synovial cyst as originating from the proximal tibiofibular articulation with neither rupture nor regional infiltration []. A left PTFJ synovial cyst with extraneural compression over the common peroneal nerve was diagnosed.\nSurgery was performed and the synovial cyst was exposed in the PTFJ area. Compression of the common peroneal nerve by the synovial cyst at fibular head and neck was found during surgical exploration. The compressed nerve was thin by about 60% in diameter when compared to the noncompressed area. Total excision of the synovial cyst was performed and a synovial cyst with a fibrous wall was confirmed on histopathology. After 3 months of the surgery, the physical examination revealed normal muscle power of the peroneal muscle and normal gait pattern was obtained. He returned to sports postoperatively. One year later, the subsequent conduction velocity study of the common peroneal nerve revealed the parameters had returned to normal range (NCV: 46 ms; peak amplitude: 3.7 mV in NCV study; no polyphasic motor unit action potential was found in electromyography study). There was no recurrence of the cyst at 3 years of followup.
A 31-year-old woman was transferred at 24 weeks of gestation to Chungbuk National University Hospital from a local obstetric clinic because of suspicious acute aortic dissection (AAD). She had previously been diagnosed as pregnancy with Marfan’s syndrome; she then underwent a routine check-up. Her chest pain had developed 3 days prior, and she was examined by transthoracic echocardiography immediately. On arriving in Chungbuk National University Hospital, the mental status of the patient was clear and her vital signs were stable: heart rate of 120 beats/min, blood pressure of 125/76 mmHg, and respiratory rate of 22 breaths/min with 96% SpO2 in the room air. Urgent chest computed tomography revealed AAD (DeBakey type II) from the level of the Valsalva sinuses to the distal ascending aorta involving the right coronary orifice, without mediastinal hematoma or pleural effusion. The diameter of the ascending aorta was 52 mm at the sinotubular junction, and the annulus of the aortic valve was 33 mm (). On transthoracic echocardiography, the ejection fraction was 65% and moderate aortic regurgitation was observed. The fetal sonography was normal. Emergency operation with the fetus in the uterus was decided upon. Under general anesthesia, cardiopulmonary bypass was instituted by draining blood from the right atrium and perfusion into the femoral artery. The intraoperative finding was that the thin adventitia was bearing the vertically dissected aortic wall, involving the right coronary orifice (). A modified Bentall procedure was performed with fetal heart monitoring simultaneously. We made an effort to maintain the blood pressure at above 80 mmHg of systolic pressure during operation while keeping the body temperature over 32°C. We were able to keep the fetal heart beating well until the second cardiac arrest with moderate hypothermia for the bleeding control of the anastomotic site of the right coronary button. The bleeding was not controllable while maintaining the heart beat because of the repeated tearing of the dissected fragile button area through suturing, and the mother’s life was at risk. We controlled the bleeding site of the new right coronary orifice with an autologous pericardial patch and per formed bypass grafting (ascending aorta to right coronary artery with a saphenous vein graft). After this second cardiac arrest, we could not hear the fetal heart sound. The fetus was aborted and evacuated postoperatively 4 days later. After 6 days in the intensive care unit, the patient was transferred to the general ward and discharged on postoperative day 20 uneventfully.
A 52-year-old woman presented to the emergency department with 3 days of diffuse abdominal pain that started during a dance class. The pain had a sudden onset with radiation to her back and did not worsen with meals. She had mild associated nausea and bloating but no other symptoms including emesis, hematochezia, melena or diarrhea. Her medical history was notable for former smoking history (10 pack-years, quit 5 weeks prior to presentation), but she was otherwise healthy and was taking no medications. Her surgical history consisted of laparoscopic appendectomy and diagnostic laparoscopy for endometriosis, both of which occurred more than a decade prior to presentation. She was hemodynamically stable with systolic blood pressure ranging from 100 to 120 s mmHg, and her exam was notable for mild diffuse abdominal tenderness without evidence of peritonitis. Her complete blood count and basic metabolic panel were within normal limits, and a venous blood gas demonstrated no evidence of lactic acidosis. A computed tomography (CT) angiogram of the abdomen and pelvis demonstrated a 5–6 cm length proximal SMA dissection with high-grade stenosis of the true lumen as well as a short-segment right external iliac artery dissection with mild narrowing of the true lumen (). There was no radiographic evidence of bowel ischemia.\nShe was admitted and maintained on bowel rest and heparin infusion. Given persistent pain after 2 days of non-operative management, an abdominal arteriogram was performed through a left axillary artery cutdown. The dissection was identified 1.5 cm distal to the origin of the SMA (). Intravascular ultrasound was used to determine diameters and landing zones for stent placement (). The dissection was initially treated with a 6 mm × 40 mm self-expanding stent followed by post-dilation with 4 mm × 40 mm and 6 mm × 40 mm balloons. A 5 mm × 19 mm stent graft was deployed proximally with small overlap and ~3-mm extension into the aorta. Completion angiogram showed <10% residual stenosis (). As the right external iliac artery dissection was small and asymptomatic, no intervention was performed.\nPostoperatively, her abdominal pain resolved and she was discharged on postoperative Day 1 with aspirin 81 mg and clopidogrel 75 mg daily. A 4-week and 6-month follow-up abdominal duplex ultrasound demonstrated patent SMA stents without dissection or stenosis. Ankle-brachial indices at 6 months were normal bilaterally.
A 23-year-old man presented to the Emergency Department of our hospital with a 1-month history of pneumaturia, fecaluria, and mild abdominal pain on the right inferior abdominal quadrant. He referred that, one week earlier, he experienced an episode of macroematuria with no fever and regular bowel function. He was sent to Urological Department for a consultation. His clinical history was characterized only by appendectomy and a right hernioplasty that dated back to 3 years earlier with no complication. He had no history of chronic inflammatory disease of the bowels or mental disease. Examination revealed only a right lower abdominal pain during deep palpation. An ultrasound scan of the abdomen showed a hyperechoic rim on the right wall of the bladder, but it was not considered relevant. Cystoscopy showed two relatively small inflamed areas on the right anterolateral wall of bladder. Cystography, after a refilling of 500 mL, looked normal (). A CT of the pelvis revealed a threadlike element, probably a foreign body (length 6.5 cm and diameter 1–1.5 mm), angled in its caudal part. A tip of the foreign body was in the right anterolateral wall of bladder and the other one was in a pelvic loop of the sigmoid colon without any signs of abscess or fistula (). After CT the patient referred that he accidentally swallowed a toothpick two years earlier with no consequent complications and then he forgot about it.\nWe tried to remove the foreign body endoscopically. A second cystoscopy confirmed the presence of an inflamed wall, but the foreign body was not visible inside the bladder. Gastroenterologists tried to remove the toothpick by a colonoscopy but they did not find any evidence of the foreign body in the sigmoid lumen. Even after bladder distension with methylene blue and saline solution, the colonoscopy did not revealed signs of fistula or the tip of the toothpick. The failure of endoscopic procedures required a surgical treatment. The patient underwent laparoscopic segmental resection of the sigmoid colon and transanal Knight-Griffen anastomosis to remove the fistulous tract and the foreign body. The bladder was sewed up with an interrupted polysorb 2/0 suture. At the end of the surgical operation a vesical catheter was positioned and it was removed 10 days later after a negative cystography.
In August 2018 a 73-year-old woman came to our emergency department complaining about pain and inability to walk after an attempt to flex her left hip. She underwent an arthrodesis of the left hip around 40 years before because of a severe dysplasia of the hip. Although the leg discrepancy and the limited ROM before the injury, she was able to walk long distances.\nX-rays showed an intertrochanteric fracture of the arthrodesed hip () and CT scan was performed to better investigate the location and the morphology of the fracture (-). Based on the imaging, we planned to treat this fracture with a 4,5 LCP condylar plate of the contralateral side turned upside down.\nThe patient was operated supine on a fracture table with the leg in traction under spinal anaesthesia.\nA standard lateral approach was used to access the fracture. We exposed the atrophied gluteus maximus and then the gluteus medius and the fasciae latae to access the fracture. During the surgery we found a medial bone gap due to the comminution of the fracture, so we also used a synthetic cancellous bone block as a biological scaffold. Under a C-arm intensifier the fracture was reduced and fixed with the 4,5 condylar right femur plate as planned. Screws were inserted in a bicortical manner\nThe surgical time was 120 minutes.\nThe patient was allowed to freely mobilize in the bed and began physiotherapy the day after the surgery. She was discharged home after the 10th postoperative day going on her physiotherapy as an outpatient service. Outpatient physiotherapy was done either in a rehabilitation clinic and at patient’s home. For the first period no weight-bearing was allowed and therefore she faced only therapeutic exercise and transfer training.\nBreathing exercise, isometric strengthening, pool therapy, straight leg raises and continuous passive motion of the knee with the machine was performed twice a day for 3 days a week for 6 weeks. After that a partial weight-bearing was grant but avoiding leg position greater than 60° of flexion to limit stress on the implant until fusion was obtained.\nThe total weight-bearing was increased after 2 months when we observed a radiological sign of consolidation.\nGait training and instruction in activities of daily life were now possible.\nX rays and clinical follow-up were carried out at 1 – 2 – 6 (Fig. 6, 7, 8) and 18 months (Fig. 9).\nAt six months the fracture was completed healed, and the patient returned back to her preinjury level of function and autonomy.
Our patient, a 33-year-old primigravid, was introduced to our hospital with spontaneous pregnancy complicated by an ovarian cyst. At the initial examination at our hospital, we observed a single 9-cm cyst in Douglas' pouch that was subsequently diagnosed as an endometrial cyst. As there were no malignant findings, surgery was not performed during the pregnancy (Fig. ). There were no symptoms and no change in the size of the cyst during the pregnancy. To prevent a post-term pregnancy, induction of labor by oxytocin was started on the morning of the 41st week and 4th day of pregnancy. At our hospital, we performed epidural analgesia during labor only for patients with complications need analgesia during labor, such as cardiovascular or respiratory disease. Therefore, we do not perform epidural analgesia for this patient.\nThe oxytocin infusion was discontinued in the evening because no effective labor was achieved despite rupture of the membranes 5 h after labor induction. After the oxytocin infusion was stopped, the uterine contractions continued at 2-min intervals. The patient had difficulty walking on her own, but this was diagnosed to be due to the labor pain.\nThe following morning, delivery progressed to 9 cm of uterine cervix dilation. The patient's body temperature was 36.3°C, but blood sampling revealed a white blood cell count of 26,660/μL and C-reactive protein level of 14 mg/dL, indicating a high inflammatory response suspected to be due to an intrauterine infection for which cefmetazole sodium was administered at 1 g every 12 h. As the delivery arrested for 2 h after uterine dilation of 9 cm was achieved, the oxytocin infusion was restarted. The baby was delivered the same day. Because the patient complained of lower abdominal pain even after delivery, nonsteroidal anti-inflammatory drugs were administered. Thereafter, the patient could walk on her own but complained of intermittent nausea and had poor dietary and water intake, so fluid replacement was required from the second day after birth. Three days after birth, the nausea continued, abdominal pain was aggravated, and upper left abdominal tenderness and rebound pain were observed. Ultrasonography revealed ascites retention and reduction in the ovarian cyst (5.1 cm × 2.2 cm) (Fig. ), and rupture of the ovarian cyst was suspected. Abdominal computed tomography showed collapse of the ovarian cyst and retention of ascites in the upper abdomen corresponding to the ultrasound findings as well as dilation of the intestinal tract. Ileus due to the peritonitis associated with rupture of the endometrial cyst was diagnosed. Laparotomy revealed that the contents of the endometrial cyst had pooled in the peritoneal cavity (Fig. ), confirming its rupture (Fig. ). The contents of the cyst were chocolate-like fluid, and its amount was 125 mL. Hence, a unilateral cystectomy was performed. The postoperative prognosis was good, and the patient was discharged 5 days postoperative. The revised American Fertility Society classification was 30 points preoperatively, and the details were as follows; 20 points for left ovary's deep endometriosis larger than 3 cm, 2 points for its filmy adhesion was 1/3 to 2/3 enclosure, 4 points for its dense 1/3, 4 points for posterior partial cul-de-sac obliteration, and 0 point postoperatively. The histologic analysis of the isolated ovarian cyst showed phagocytosis of hemosiderin, and diagnosis of endometrial cyst was confirmed (Fig. ).
A 64-year-old immunocompetent Caucasian man with no history of cardiac disease presented with chest pain, dyspnea and edema of his lower limbs associated with a degeneration of his general state. On physical examination he had a temperature of 37°C, blood pressure of 100/74 mmHg, and heart rate of 30 bpm. His jugular venous pressure was high. The first and second heart sounds were normal without any audible murmurs, rubs or gallops. His chest was clear to auscultation. His hemogram, hepatic enzymes and inflammation markers were all normal. The patient was HIV-negative. His chest X-ray revealed cardiomegaly as well as bilateral pleural effusion. The standard 12-lead ECG indicated an atrioventricular block of the third-degree. It returned to normal spontaneously one hour later. Transthoracic echocardiography (TTE) (Figures and ), demonstrated not only a pericardial effusion of 23 mm by 35 mm with signs of tamponade but also the presence of a large mass at the level of the right ventricle. The mass had a wide base and was heterogeneous. It appeared lobulated with a tissular echo texture that measured 5.5 cm by 5 cm. It was also attached to the tricuspid valve creating a right ventricle inflow obstruction. The tumor spread over the right atrium. He underwent an urgent pericardial drainage which returned 600 cm3 of hemorrhagic liquid. Bacteriological and cytological analyses revealed large cells suggestive of a lymphoproliferative disorder. A computed tomography scan showed the presence of a right heart tumor on both sides of the tricuspid valve as well as peritoneal effusion. No other organ involvement was observed (Figure ). Coronary angiography accentuated an increase of a myocardial blush in favor of the highly vascular nature of the tumor (Figure ). This examination was performed because the patient was more than 40 years old. It was thought that emergency surgery might be necessary at any time because of size of his tumor.\nDue to the rapid impairment of his cardiac function and the life-threatening hemodynamic instability, an echocardiography was performed which showed an obstruction of the right ventricle inflow.\nHe underwent an emergency thoracotomy. The purpose of this surgery was not to remove the entire tumor. It was limited to freeing the tricuspid valve and the intra-right ventricle obstruction. Surgical resection of the mass was difficult and incomplete. The tumor had infiltrated his right atrium, the atrioventricular septum and the proximal side of the right ventricle. Surgical removal was laborious but without complications.\nThe tumor was submitted to the pathology laboratory as white and red soft fragments measuring 3 cm by 3 cm by 2 cm (Figures and ). Histological analysis revealed non-Hodgkin large B-cell lymphoma (CD45+ CD20+ CD3- BCl2+ CD20- CD10- BCl6-). The lymphoma was classified as IE, according to the Ann Arbor staging classification.\nChemotherapy with the R-CHOP (Rituximab, Cytoxan, Hydroxydaunorubicin (Adriamycin), Oncovin (Vincristine), Prednisone/Prednisolone) regimen began immediately after resection.\nAfter the first course of chemotherapy TTE demonstrated a reduction in the size of the mass (Figure ).
We report a case of a 68 years old woman presenting to the emergency department with macrohematuria and dysuria. She experienced fever several times, starting two months before medical examination. Fever was treated with NSAID. A history of recent pelvic pain was also reported. Her clinical status was good and no other symptoms or concurrent illnesses were present at the time of hospitalization.\nShe was admitted to Gynecologic service for further evaluation.\nThe patient was virgin and in post-menopausal status. Clinical history was unremarkable; negative either for previous medical or surgical procedures. Family history was positive for cancer with one male sibling deceased from a pancreatic malignancy; the remainder two siblings had no positive history for malignancies as well as parents and closest relatives. Social history was negative for use or addiction to drugs, alcohol and exposure to potential risk factors.\nA digital rectal examination had to be performed in place of the gynecologic bimanual examination. A gross, firm mass located in the pelvis was found at physical examination.\nPelvic US, abdominal CT scans, descending pielography, cystoscopy and chest RX were performed to both assess local disease and evaluate the presence of local and distant metastases.\nComputed tomography imaging confirmed the presence of a complex mass in the pelvis (13 × 14 cm) showing irregular contours and small areas of calcifications. Uterus and adnexa could not be clearly recognized, showing as being part of the mass. Iliac vessels and bladder were displaced. Imaging technique reports stated that no vessels or bladder invasion was present. A hypoplasia of the left kidney was also reported. No other metastasis was reported in the abdomino-pelvic cavity.\nPielography showed a delay in the opacification of the renal pelvis associated to a dilation of the right ureter that was compressed and displaced in its pelvic course till entering the bladder. No opacification of the contra lateral kidney and excretory ways could be obtained.\nCystoscopy showed, eventually, a bleeding lesion onto the bladder wall that raised suspicion of neoplastic invasion. The lesion appeared more likely as a loss of continuity of the urothelial mucosa; an ulcerating mass was visualized and eventually described on final report.\nChest RX and bone scan were negative for distant metastases.\nA multidisciplinary board, including a urologist, a radiologist, a radiation oncologist, an oncologist and a gynecologist, evaluated the collected data. It was eventually decided to surgically extirpate the tumor. A transurethral biopsy of the vesical wall lesion was not considered since major suspicion was addressing to uterine or adnexal neoplasms and the high stage was reasonably an indication to undergo definitive surgery.\nSurgery was eventually performed after pre-operative routine and evaluation.\nA longitudinal abdominal incision was preferred to access peritoneal cavity. A bulky mass was found in the pelvis. Uterus and adnexa appeared macroscopically free from neoplastic invasion. The mass showed a cleavage with recto-sigmoid tract, iliac vessels and pubic bone, whether uterus, adnexa, right ureter and left obturator nerve could not be separated from the tumor. A histological intra-operative examination revealed a malignant, spindled cell neoplasm. Surgery had to include a wide mass excision, in order to obtain free margins and control of local disease. Final treatment choice was a pelvic exenteration. This decision was made since local disease was advanced and it was set to obtain specimen's margins disease-free as a primary target.\nAn en-bloc resection of the mass, together with uterus, adnexa, bladder, distal part of right ureter and left obturator nerve was performed (anterior pelvic exenterantion).\nA cutaneous ureterostomy was placed in the site of ureteral dissection. The decision to perform this procedure in place of a standard urinary diversion, as a ileal conduit or Miami pouch, was made on the evidence of poor prognosis that appeared from surgical exploration and intra-operative histology that was addressing to a malignant stromal neoplasm. It seemed to be reasonable to lower intra- and post-operative morbidity whereas the potential overall survival of the patient appeared to be prospectively low.\nA final histological diagnosis of high-grade leiomyosarcoma, (Fig. ), (G3, FNCLCC 1986) pT2b pN0 pM0, AJCC (2002) Stage III, was made based on immunoreactivity to smooth muscle actin, score 2 (Fig. ). The specimen resulted negative to c-kit and EGFR. Proliferation index was 90%, evaluated thru MIB-1 (Ki-67) (Fig. ). Necrosis was inferior to 50% (score 2) and mitotic index higher than 20 mitosis per field/10 HPF (score 3). The tumor presented, macroscopically, as a bulky, brain-like, white-grayish mass. The tumor contained diverse necrotic and hemorrhagic areas, and invaded the vesical wall. The size of the mass was 14 × 11 × 6.5 cm. Histology confirmed that uterus and adnexa were free from neoplastic invasion. Surgical margins resulted negative at the final examination. LVI was not reported.\nPost-operative course was free from complications and the patient could be discharged from the hospital fourteen days after surgery.\nThe patient underwent chemotherapy one month after surgery, using doxorubicin as a single agent in an adjuvant setting. She died after the first cycle from a distant recurrence to the left lung.
A 22-year-old male, with a known case of temporal lobe epilepsy since childhood and mental retardation, was on sodium valproate and Levetiracetam for his epilepsy. He presented to the emergency room complaining of three episodes of generalized tonic clonic seizure within 15 minutes without returning to baseline consciousness between the seizures. The patient was sedated, intubated and admitted to the Intensive Care Unit (ICU) as a case of status epilepticus. Two days later, he developed a fever >38°c associated with increased tracheobronchial secretions. Relevant septic work-up was sent and it showed leukocyte count of 18.3 x 109/mL and culture of tracheobronchial secretions showed growth of Pseudomonas aeruginosa. A chest X-ray showed new infiltrates in right lower lobe consolidation () which could be sign of aspiration but risk of aspiration was low in this case because all measures to prevent aspiration were taken including a semi-recumbent position, surveillance of enteric feeding and use of promotility drugs. Diagnosis of VAP was made based on the development of pneumonia that occurred more than 48 hours after he was intubated and mechanically ventilated. He improved clinically so he was extubated. He stayed in ICU for 5 days and then was transferred back to a ward. He again became febrile and hypotensive on the second day of his stay on the ward. So, he was transferred back to ICU and antibiotics were modified to a combination of Piperacillin-Tazobactam and Vancomycin. He did not require mechanical ventilation this time and started to show improvement on second day of stay in ICU. He became afebrile and was transferred out of ICU to ward after four days’ stay in ICU. Upon transfer to a ward his vital signs were stable, he was afebrile and maintaining oxygen saturation on room air. He had returned back to his baseline condition prior to admission. After 5 days on the ward he gradually developed progressive bulbar weakness and ataxia. On examination, his vital signs were a pulse of 48 beats per minute, blood pressure of 96/63 mmHg and temperature of 36.9°c. On neurologic examination he had ophthalmoplegia, finger to nose dysmetria, dysdiadochokinesia, abnormal heel to shin test, generalized areflexia and difficulty in standing and needed full support to stand up. The rest of the neurologic examination was unchanged from his baseline examination. The patient underwent a lumbar puncture and a CSF analysis showed proteins 100mg/dl and a white cell count of 10. MRI brain and spine was also done which shows re-demonstration of atrophic left hippocampus with abnormal T2 and Flair hyperintense signals due to mesial temporal sclerosis ( and ). Levels of anti-ganglioside antibodies were: Anti-GD1b 198, Anti-GD1a 175, Anti-GM1 154, Anti-GM2 41 and Anti-GQ1b <30. Reference range for all these antibody is less than 30. Since the antibody tests were strongly positive electrophysiological studies were not performed which might have been compromised due to the patient being mentally challenged and thus unable to cooperate. A diagnosis of sero-negative MFS variant of GBS was made and the patient received 5 sessions of intravenous immunoglobulin (IVIG). After 3 days his hemodynamic status improved to give a blood pressure reading of 113/67mmHg, his heart rate was 68–75 beats per minutes but other neurologic symptoms did not improve. The patient was monitored closely until his hemodynamic status stabilized. Once hemodynamics were stable, the rehabilitation team was consulted. Written informed consent has been provided by the patient to have the case details and any accompanying images published, institutional approval is not required for case report publication.
A 63-year-old man came to our department because of recurring headache attacks, sometimes associated with ptosis and diplopia for 2 years. A typical headache attack would start with a mild pain restricted in the right supraorbital margin point and almost immediately after the onset of this pain the patient would suffer a distinctive head pain in the ipsilateral side. The head pain gradually reached its peak in minutes and was circumscribed within a line-shaped area of 5 mm to 10 mm in width. This line-shaped pain area linked the right supraorbital margin point with the ipsilateral posterior paretial region and was parallel to the sagittal midline of the head. The pain was described as moderate to severe distending, pressure-like and sometimes pulsating sensation with no radiation or moving, and the patient denied this pain epicranial but complain it intracranial. The headache was usually associated with nausea and phonophobia, often precipitated by physical activity and would last for 2 to 3 days and remitted spontaneously. In recent two years, the patient has had more line-shaped headaches without ophthalmoplegia, occurring approximately 15–20 times per year. He had also had three episodes of ptosis and diplopia in recent one year. These episodes of ophthalmoplegia all occurred at the day the line-shaped headache was remitting and resolved after approximate 7 to 10 days. He had a history of migraine without aura attacks in a frequency of 12–16 times per year for more than 20 years, which remitted 10 years ago. There was a family history of migraine affecting at least 5 individuals across four generations.\nOn examination during the episode of ptosis and diplopia, our patient was found to have a right third nerve palsy with pupil involved, which manifested as an incomplete ptosis and an inability to lower and adduct his right eye. Diplopia was found in all gaze directions, except for looking laterally to the right (Figure , taken 3 days after the onset of ophthalmoplegia). His right pupil was little larger than his left, but did react slowly to light and accommodation. Other nervous system examination, including trigeminal nerve examination, was normal.\nA brain MRI with contrast was performed 2 days after the onset of ophthalmoplegia, followed by a brain computed tomographic angiography (CTA) conducted 4 days after the onset of ophthalmoplegia, both of which were normal. Cerebrospinal fluid (CSF) analysis performed 3 days after the onset of ophthalmoplegia was normal, including cell counts, protein, glucose levels, and IgG index. Blood routine tests and other investigations including erythrocyte sedimentation rate, vasculitic autoimmune and venereal disease screens were all in normal range.\nThe patient accepted prophylactic treatment with sodium valproate (500 mg twice a day) together with flunarizine (5 mg twice a day) and he had neither the line-shaped headache nor ophthalmoplegia in the next eight-month follow-up.
A 35-year-old Saudi SCD male on hydroxyurea, with no other relevant medial or family history, presented to our university hospital outpatient clinic, complaining of left hip pain 18 months following cementless left total hip arthroplasty using the DePuy Corail Hip System. The patient presented complaining of left-sided thigh pain for one month that had started after performing a sudden motion of the hip, but had no traumatic event or history of infection. Upon examination, the patient displayed no signs of local infection, no tenderness, normal power, and intact distal neurovascular function. Moreover, his left hip range of motion was limited in extreme flexion and internal rotation due to pain. The Harris Hip Score (HHS), which is a 100-point questionnaire used to objectively assess the hip joint function according to four main domains (pain, function, deformity, and range of motion) [], was 33 points. X-rays and computed tomography showed broken distal femoral stem with no apparent cortical fracture, the proximal part of the femoral stem was loose while the distal fragment remained fixed inside the intramedullary canal (). Lab workup and inflammatory markers were taken and showed no signs of infection. The patient was diagnosed with aseptic loosening of the left femoral stem. He was subsequently scheduled for left-sided femoral stem revision.\nMultidisciplinary team was involved to manage the patient’s SCD condition and optimize the pre- and postoperative setup. It is important to maintain proper oxygenation and hydration, to prevent hypothermia, and to control the HgbS concentration. The patient underwent left-sided revision arthroplasty using a posterior approach to the hip along the previous surgical incision which was done by a senior orthopedic arthroplasty consultant. Soft tissue biopsy and fluid swabs were taken from the hip joint for frozen section, culture and sensitivity intraoperatively, and were all negative. The joint was then dislocated to address the femoral implant. The liner was intact and the proximal part of the femoral stem and head were removed easily. Afterward, we placed a Kirschner wire distal to the tip of the femoral stem to prevent the stem from migrating distally. Subsequently, a trephine reamer was adopted under guidance of the C-arm to disengage the distal femoral stem, whose size was chosen based on the diameter of the extracted proximal end. While using the trephine reamer, generous irrigation with saline was performed to avoid overheating and osteonecrosis of the bone. Finally, the distal fragment was caught inside the trephine reamer and was removed (). A 16 Wagner SL stem (Zimmer Biomet, Warsaw, IN, USA) was used during the revision procedure. Reduction and stability were confirmed, and since there was no cortical fracture, a prophylactic cerclage wire was not needed prior to stem insertion. A gross image of the extracted implant can be seen in ().\nThe operation went well with no intraoperative complications. Immediate post-operative X-ray is seen in . Five days after his procedure the patient was discharged and was meeting his physical therapy goals. The patient was instructed to fully weight bear and to avoid crossing his legs or flexing his hip or bending past 90 degrees in the early phase of his rehabilitation to prevent post surgical dislocations.\nThe patient was followed up with regularly without any complications, he had full range of motion and no signs of pain or deformity. His postoperative HHS was 76 points. The patient was able to go back to his normal day activities. However, it is important to mention that the patient required another revision procedure due to aseptic loosening at the distal tip of the implant diagnosed by bone scan. The patient was seen 2 years after his primary revision procedure at our university hospital’s outpatient clinic and was doing well, fully weight-bearing, and has full range of motion and no pain. X-rays showed well-fixed implants with no signs of loosening (). The patient returned to practicing his usual lifestyle with no apparent limitations in his activity, and expressed his happiness to regain full function and return to normal activity in a timely manner.
A 79-year-old male patient reported to the Department of Oral Pathology and Microbiology with a chief complaint of pain and swelling on the upper left buccal mucosa since 1-month. The swelling was insidious and gradually increased in size. The patient also reported reduced sensation on cheek mucosa of the left side.\nExtraorally, facial asymmetry was noticed with diffuse swelling on the left sided cheek region, approximately 3 cm × 2 cm in size, irregular in shape, vertically extending from inferior border of zygomatic arch up to 1–2 cm above corner of mouth and was extending from ala of nose up to tragus of the ear mediolaterally. The swelling was firm and nontender on palpation with normal appearing overlying skin. The swelling was not fixed to the underlying structures. On intraoral examination, the swelling was diffuse, soft and roughly oval in shape []. The color of the overlying mucosa was normal as that of the surrounding mucosa. The swelling was situated in maxillary buccal vestibule extending anteroposteriorly from 26 to 28 region and mediolaterally from buccal mucosa of left side obliterating the vestibule, crossing the alveolar ridge and extending onto palatal mucosa. The swelling was approximately 4 cm × 2 cm in size and was of smooth surface texture. On palpation, the swelling was tender and not fixed to underlying structures.\nRadiologically, on Waters view haziness was noticed in the left maxillary sinus region as compared to the right side, extending from superior border up to inferior border of sinus vertically and extending from medial to the lateral border of sinus mediolaterally [].\nAn incisional biopsy was advised from buccal mucosa of left side under local anesthesia after routine blood investigations, which were within the normal limits. Human immunodeficiency virus (HIV) Tridot screening test was also negative.\nOn histopathological examination, the hematoxylin and eosin stained tissue section showed diffuse, monotonous proliferation of round cells, in the form of sheets within the loose fibrillar connective tissue stroma []. These cells were having large nuclei with a small amount of cytoplasm resembling lymphoid cells. It also revealed the presence of cells with large nuclei showing prominent nucleoli arranged at the periphery, exhibiting condensation and vesiculation of chromatin with cellular and nuclear pleomorphism. These cells resembled centroblasts [] and immunoblastic cells []. The adjacent minor salivary gland tissue with mucous acini showed infiltration of these cells, disturbing the normal architecture.\nOverall histopathological features suggest the diagnosis of atypical lymphoproliferative lesion. For confirmation, further immunohistochemical evaluation was done.\nOn immunohistochemical analysis, CD45 (leukocyte common antigen) [] positivity distinguished malignant lymphomas from other non-lymphoid neoplasms, strong positivity for CD20 (B-cell marker) [] and weak expression of CD3 (T-cell) marker suggests the B-cell origin of the lesion and Ki-67 (proliferation marker) positivity indicates the proliferative potential of the lesion. Thus, the overall histopathological and immunohistochemical findings were suggestive of DLBCL.
A 62-year-old male underwent surgery for thoracic-cervical esophageal cancer and immediate reconstruction with the retrosternal gastric pull-up technique. The esophagus resection was performed at the level of the 6th cervical vertebra and a bilateral lower segmental neck dissection was performed (nos. 101 to 104) []. Therefore, the transverse cervical vessel was exposed in first surgery. Three days after surgery, the patient's neck exhibited swelling and redness, and the oral side of the pulled-up stomach became necrotic (). Thus, 4 days after the primary surgery, the patient underwent debridement of the necrotic tissue and the route of the gastric tube was changed from retrosternal to presternal. The resection of the necrotic gastric tube was 5 cm length, and debridement of necrotic tissue and washing with saline solution were performed. At the same time, immediate free jejunum transfer was performed as salvage surgery (). The jejunum was transected at the level of the second jejunal vessel, and the length of harvesting jejunum graft was 15 cm. After trimming of the jejunum graft, the jejunum graft, which was 10 cm length, was placed in the presternal space and joined to the distal end of the cervical esophagus and the proximal end of the residual gastric tube in an end to end anastomosis. The jejunal artery and vein were anastomosed to the right transverse cervical artery and the right internal jugular vein (). Because of the short span between first and second surgery, harvesting the free jejunum graft was performed without any difficulty. However, neck dissection during the first surgery caused some adhesion, which resulted in minor technical difficulties during microsurgery. In second time surgery, patient's vital signs were stable, and no fever or problems in respiratory or circulatory organs were observed. The C-reactive protein level was 22.6 mg/dL, but the white blood cell level was 8.7 × 10³/μL. The patient was able to start oral alimentation 10 days after salvage surgery, and the wound healed completely without complications (). Although he died from systemic metastasis from the primary cancer 6 months after the salvage surgery, the patient's quality of life after surgery, including his food intake, was very good.
The patient was a 24-year-old woman, 1 gravida and 0 para. She was diagnosed with a normal intrauterine pregnancy. There was no abnormality in her medical history and allergy history.\nAt 36 weeks and 2 days of gestation, she was diagnosed with a placental abruption and underwent emergency cesarean section immediately. A female baby weighing 2439 g was delivered with umbilical artery pH of 7.140. The amount of bleeding during surgery was approximately 658 ml including the amniotic fluid. Until the 2nd postoperative day, there were no abnormalities in her vital signs or urinary volume.\nBlood tests were performed on the 3rd postoperative day and revealed severe anemia with a hemoglobin (Hb) level of 3.9 g/dl. Performing an abdominal ultrasound examination and enhanced computed tomography (CT) of the lower abdomen, we found a hematoma with a diameter of 9 cm between the uterus and bladder (). A blood transfusion improved the anemia immediately. She did not complain of abdominal discomfort, and no signs of infection were observed. Thus, a conservative therapy was chosen. She was discharged on the 10th postoperative day and was followed as an outpatient.\nOn the 28th postoperative day, she came to our department with a complaint of abnormal vaginal bleeding. Blood tests did not reveal anemia (Hb 12.0 g/dl). Enhanced CT and magnetic resonance imaging (MRI) were performed, and there were no apparent findings of infection associated with the hematoma; however, we were concerned about a fistula at the site of the uterine incision. To remove the hematoma and check the incision site, we performed laparoscopic surgery.\nWe inserted a 12 mm port in the umbilical region and observed the abdominal cavity. The hematoma was located extraperitoneally inside the chorda umbilicalis (). Then, we performed resection of the hematoma laparoscopically. We cut the peritoneum inside the chorda umbilicalis and successfully reached the interior of the hematoma. The uterine incision site was located away from the hematoma; therefore, we thought that the fistula was not located between the incision and hematoma. We resected the hematoma and lavaged the space fully, followed by the placement of a drainage tube.\nThe patient had an uneventful postoperative course, and the drainage amount was small. We removed the drainage tube on the 4th postoperative day, and the patient was discharged on the same day.\nTwo days after her discharge, the patient returned to the emergency department of our hospital with a complaint of severe diarrhea, gastric discomfort, and a high fever.\nThe change in the patient's body temperature and treatment process is shown in .\nAt admission, the patient complained of a high fever, severe diarrhea, and intense abdominal pain. Enhanced CT was performed and revealed that a cyst with a diameter of 9 cm was located at the same site where the hematoma had existed (). The CT value inside the cyst was slightly elevated. Blood tests were performed, and the results showed a slight elevation of the WBC count and C-reactive protein (CRP) (10,800/µl and 1.6 mg/dl, resp.). We assumed that an infection occurred at the site of the hematoma, which resulted in the abscess. We administered antibiotics, namely, ABPC/SBT.\nThe antibiotics improved the diarrhea immediately; however, her body temperature was in the latter half of the 37°C range. Abdominal pain was improved to some degree. On the 4th postadmission day, we punctured the abscess transvaginally and absorbed dirty gray pus. We considered an anaerobic bacterial infection and changed the antibiotic from ABPC/SBT to TAZ/PIPC.\nOn the 7th postadmission day, her body temperature exceeded 38°C and her gastric discomfort tended to worsen. On the same day, bacterial culture examination of the pus revealed that E. tarda was detected. We performed the literature survey and found that abscess of E. tarda could be fatal. The patient's general condition tended to worsen. Although the antibiotics sensitivity test revealed that E. tarda was susceptible to almost all antibiotics, including ABPC/SBT and TAZ/PIPC, we thought that escalation of antibiotics was preferred. Consulting with the infection care team, we changed the antibiotics from TAZ/PIPC to MEPM.\nHer fever still remained above 38°C (), and her gastric discomfort tended to worsen each day. On the 9th postadmission day, we performed an enhanced CT scan and found an increase in abscess size (), which expanded into the peritoneal cavity, close to the ileocecal region. We decided that a drainage procedure was necessary.\nOn the 10th postadmission day, we performed a laparotomy to facilitate the drainage procedure. Approximately 200 ml of dark gray pus with a bad odor was absorbed. The abscess was mainly located in the obturator space surrounded by the bladder, uterus, ileum, and external iliac vein (). We resected the infected granulation tissue attached to the surface of the uterus, bladder, and pelvic wall and washed the area very carefully. The cranial side of the abscess expanded into the abdominal cavity and serosa of the ileum. The serosa of the ileum had become fragile. Although no apparent findings of perforation were observed, we resected the ileum to prevent the subsequent occurrence of perforation or diverticulum. Then, we placed a drainage tube and finished the procedure.\nE. tarda was detected from the drainage by bacterial culture examination. After the operation, her body temperature tended to decrease to below 37°C (). The WBC count and CRP value tended to decrease to 6300/µl and 0.61 mg/dl, respectively, on the 7th postoperative day. On the same day, we changed antibiotics from MEPM to ABPC/SBT. We removed the drainage tube on the 17th postoperative day. The antibiotic regimen was completed, and the patient was discharged on the 21st postoperative day.\nAn adhesion ileus occurred on the 30th postoperative day, but it improved using conservative therapy. Four months after the operation, we performed a pelvic MRI examination and found no abnormal findings in her uterus, bladder, and pelvic space ().\nThe patient is currently more than one and a half year post-op and has reported no complaints.
The patient was a 57-year-old male, with ESRD secondary to diabetes and hypertension, on hemodialysis for 20 months. He was evaluated to undergo living unrelated donor kidney transplant. The donor was a 54-year-old female with unremarkable past medical history. She was medically and surgically cleared after full assessment. Preoperative computed tomography (CT) angiography for the donor revealed a small complex cyst (Bosniak IIF), a short right renal vein (2 cm), and a right renal artery with an early bifurcation of an upper pole artery. Surgical approach deemed best by the living donor selection committee was to remove the right kidney with the complex cyst via laparoscopic approach.\nThe risks of surgery and potential complications were explained to both donor and recipient. Written informed consent was obtained prior to surgery from both patients.\nA standard hand-assisted laparoscopic living donor nephrectomy (LLDN) was performed to retrieve the right kidney and the right ovarian vein for reconstruction. The length of the donor renal vein was reduced by 10-15 mm due to the standard use of vascular stapler device to control the renal vein stump.\nStatus after procurement upon visualization in the back table is that the length of the donor renal vein was 3 mm. In order to elongate the vein, the donor ovarian vein was dissected and used for reconstruction. It was opened longitudinally, folded over, and anastomosed to the donor renal vein using end-to-end anastomosis with an 8-0 Prolene ().\nThe donor kidney had a renal artery with early bifurcation of an upper pole artery. After mobilization of the inferior vena cava as much as possible, the endovascular stapler was placed after the bifurcation from the renal artery and the length of the vessel was reduced by 10-15 mm. At the back table the donor kidney presented two individual arteries: the main renal artery and the short upper pole arterial branch. Consequently, the recipient IEA was used as an extension graft to perform an end-to-side anastomosis between the upper pole arterial branch and the main renal artery using 8-0 Prolene ().\nThe complex cyst was dissected all the way down into the calyces and substantial margin of healthy parenchyma was removed. Distal margin was sent to pathology to rule out malignancy. The calyces and the renal parenchyma were oversewed with 5-0 PDS and the edges of the defect with U-stitch of 4-0 PDS using pledget ().\nThe extended renal vein and the main renal artery were anastomosed end-to-side to the external iliac vein (EIV) and artery (EIA) respectively, using 6-0 Prolene ().\nWe performed an extravesical ureteroneocystostomy and a Jackson Pratt drain was placed. The warm ischemia time was 33 minutes. Postoperative course was uncomplicated and the patient has maintained adequate renal function with stable serum creatinine (1.32 mg/dl) at 5-month follow-up. The final pathology report and the intraoperative frozen section of the margin were negative for malignancy.
A 57-year-old female with widely metastatic high-grade serous adenocarcinoma of the ovary was referred to our tertiary palliative care clinic by her oncologist for management of severe cancer-related pain. Following her original diagnosis, she underwent a number of multimodal disease-directed therapies, including surgical resection and chemotherapy. She had significant ongoing mixed somatic and neuropathic pain in the left lower back as well as her pelvis, both sites of known metastatic disease. The back pain was noted to be a deep sharp pain without radiation to her extremities, located primarily around the region of her nephrostomy tube and into the abdomen. She would also have sharp lancinating neuropathic pain in her pelvis due to tumor burden with prolonged periods of sitting, walking, or activity.\nInitial treatment consisted of multimodal pharmacologic therapy including short-acting and long-acting opioids with nonopioid and adjuvant neuropathic agents. Despite these therapies, she had progressive pain and was ultimately increased to an opioid combination of morphine sulfate controlled-release (120 mg by mouth every 8 hours) and morphine sulfate immediate-release (30–45 mg by mouth every 3 hours as needed). Early changes yielded acceptable analgesia. Conversations and medication adjustments continued over the phone, given the distance between the patient's home and our institution. However, over several weeks' time, pain progressed despite further opioid increases. Given concern for escalating pain and poor response to several attempts at altering her opioid therapy, it was advised that she present for a consultation in the palliative medicine clinic. Fortunately, focused discussions with the patient and her family around the patient's goals of care were addressed with her palliative medicine team at every meeting. She was very clear that her top priority in her ongoing medical care was to achieve acceptable analgesia. During the interview in the clinic, the patient noted significant pain with generalized discomfort, a sense of restlessness, and new muscle fasciculations. Her husband accompanied her and noted intermittent confusion. Her physical exam featured delirium (Confusion Assessment Method positive) with diffuse myoclonus.\nOut of concern for opioid-induced neurotoxicity (OIN), the patient was admitted to the hospital for analgesic control and treatment of what was felt to be a toxic encephalopathy. Despite the known shared metabolic pathway (phase II metabolism) between morphine and hydromorphone, the patient's opioids were rotated from oral morphine to parenteral hydromorphone as the clinical picture continued to unfold. Further workup revealed an unremarkable head CT and EEG. Clinical evaluations and laboratory assessments suggested that, in the setting of her declining physical and renal function (creatinine 1.0 mg/dL with prior baseline 0.6 mg/dL and estimated glomerular filtration rate (eGFR) 55 ml/min/BSA with prior baseline >60 ml/min/BSA), the delirium could be the result of an accumulation of active neurotoxic morphine metabolites (morphine-3-glucuronide). In addition, she was dehydrated, constipated, and had not slept in several days. Unfortunately, her delirium persisted despite correction of metabolic derangements, hydration, and an aggressive bowel regimen.\nUrology was consulted, and a nuclear medicine dimercaptosuccinic acid (DMSA) scan revealed minimal function of the left kidney, despite the presence of a nephrostomy tube. Subsequent vascular radiological investigations revealed tumor-induced thrombosis with surmised infarction of her left kidney. It was concluded that the altered renal function due to infarction likely resulted in an inability to adequately excrete the morphine, and the accumulation of polar morphine metabolites resulted in OIN. As exemplified in this case, even with opioid rotation, centrally active metabolites of hydromorphone (hydromorphone-3-glucuronide) theoretically exist, given that it follows a similar phase II metabolic glucuronidation pathway as morphine, although the relative potency and effect of such hydromorphone metabolites are thought to be significantly less than morphine. The patient continued to have signs of OIN and was eventually transitioned to parenteral fentanyl, given that fentanyl is a unique medication that is largely hepatically metabolized into inactive metabolites. Despite common lore that fentanyl and methadone are agents that do no produce active metabolites and therefore do not elicit OIN, case reports of synthetic opioids eliciting OIN do exist in the literature [, ]. Unfortunately, despite aggressive supportive measures and conversion of parenteral hydromorphone to parenteral fentanyl without reduction for cross-tolerance, she continued to have persistent pain with minimal resolution of her systemic neurotoxicity.\nGiven the concern for poorly controlled cancer-associated pain, the palliative care and pain medicine teams collaborated from the initial visit with concerning symptoms of OIN. At our institution, the palliative clinic shares space within the pain clinic, and the collaboration required a simple conversation followed by same day pain consultation. Initially, the shared thought was moving directly to intrathecal (IT) TDD therapy, as this had been discussed over numerous palliative care visits as a potential option with her physicians and was in line with her goals to achieve enhanced pain control with limited side effects. However, given the underlying metastatic disease process and concurrent cancer treatments, she had a metabolic coagulopathy due to nutritional and micronutrient deficits that required correction with vitamin k to assist in the reversal of her international normalized ratio (INR), thus allowing for any type of procedural intervention.\nUltimately, given the persistent multifactorial delirium and rapidly changing situation, the pain medicine and palliative care teams convened a family conference with the husband, the patients' health-care power of attorney, to again address the current situation and make recommendations that seemed consistent with the patient's predetermined goals. Given the rapid progression of her symptoms despite aggressive attempts to correct potentially remediable factors, the shared medical decision-making discussion included moving forward with TDD, given that this therapy was thought to be the best chance for meeting the patients' goals for comfort and hope to have meaningful interaction with family by reducing the burden of systemic side effects from oral or parenteral opioid therapy. The patient's advanced directive was clear that she appointed her husband to make decisions on her behalf if she was ever in a situation where she was unable to provide consent. Informed consent was obtained through her appointed surrogate decision maker and designated health-care power of attorney.\nAfter correction of her metabolic coagulopathy yielding an INR <1.2, she proceeded with placement of an intrathecal TDD system (Medtronic SynchroMed™ II 40 mL pump and Ascenda catheter). The pump reservoir was placed in her right lower abdominal quadrant, and the catheter tip was placed at T10. Her TDD system was efficiently titrated to achieve acceptable levels of analgesia using a combination of opioid (hydromorphone 2 mg/mL) and local anesthetic (bupivacaine 10 mg/mL). The initial postoperative settings were 0.5 mg/day of hydromorphone in a simple continuous mode without bolus dosing (2.5 mg/day of bupivacaine). Within 24 hours of TDD placement, the patient experienced near complete resolution of her toxic encephalopathy and was able to engage in meaningful conversation with her family and health-care teams. There were no signs or symptoms of opioid withdrawal. In the early postoperative phase, she required additional dosing of oral hydromorphone, but was responsive to 2 mg orally every 3 hours as needed for breakthrough postsurgical pain totaling three to four doses per day. Given the robust response to TDD therapy, the patient was able to participate in goals of care discussions with her interdisciplinary palliative care team and elected to discharge closer to home under the provision of hospice services postoperative day (POD) two. Prior to leaving the hospital, the TDD rate was increased to 0.8 mg/day of hydromorphone, and she was provided with a personal therapy manager (PTM) allowing 0.1 mg/dose every six hours totaling four doses per day. The dose remained stable for seven days. As her oncologic disease continued to evolve, her TDD system was adjusted by her hospice provider to accommodate her daily use of oral opioid therapy. The pump was again adjusted on postoperative day nine, yielding a basal rate of 1.4 mg/day of hydromorphone with four PTM boluses of 0.15 mg/dose of hydromorphone every six hours. This dose remained for POD 9–13, and she passed away peacefully with acceptable analgesia at home with her family under the auspices of hospice two weeks after TDD implantation.
A 70-year-old gentleman presented for gastroenterology consultation after multiple episodes of severe abdominal pain that occurred over a period of six years. The patient had a past medical history of hypothyroidism and hypertension. He reported several episodes of nearly identical GI symptoms over this period of time. The episodes would begin with right upper quadrant abdominal pain that would be relatively sudden in onset and 3 out of 10 in severity. Over a matter of hours, the pain would crescendo to 10 out of 10 and would last three to four additional hours. During this period of time, the patient would pass very little flatus and would develop abdominal distention, severe diaphoresis, and nausea. The pain would settle in the right lower quadrant for several hours and then would almost completely resolve. The patient would then remain "sore" for a period of one to two additional days. On a few occasions, he had vomiting. He had two such episodes with vomiting prior to presenting for evaluation.\nBetween the episodes, the patient reported normal bowel habits. He had no history of diarrhea, weight loss, or significant nausea or vomiting. The patient had no history of anemia or GI bleeding. He had never undergone a colonoscopy in the past. The patient did not use aspirin or other non-steroidal anti-inflammatory medications on a regular basis.\nThe patient's physical exam was unremarkable. The patient was afebrile, vital signs were stable. The patient's abdomen was soft, non-tender and non-distended. There were no palpable masses in abdomen. There were no incisional scars on the abdomen.\nEvaluation included a CT scan of the abdomen and pelvis. The CT scan showed a benign appearing renal cyst and an area of abnormal ileum. An approximately 12 centimeter long portion of ileum demonstrated concentric thickening. The radiologist offered a presumptive diagnosis of Crohn's.\nLaboratory evaluation demonstrated a normal complete blood count and normal iron studies. The patient underwent colonoscopy to further evaluate the ileal thickening. He tolerated colonoscopy prep without difficulty. Colonoscopy demonstrated left colon diverticula. The terminal ileum was abnormal. The mucosa appeared atrophic and the luminal diameter was narrowed. There was also a mobile, pedunculated 3.5 centimeter frond-like mass. During the endoscopic evaluation, this lesion was seen to move proximally and distally over a span of at least 5 to 7 cm. It was difficult to biopsy given its movement. When the mass moved distally it filled the entire terminal ileal diameter (Figure ). The mass moved to the area of the IC valve but was not seen to enter the colon. Biopsies of the terminal ileum and the ileal mass showed scattered ulcers with crypt distortion and pyloric gland metaplasia suggestive of Crohn's disease. This did not explain the presence of a mass. Surgical management was recommended but the patient refused.\nThe patient had two additional episodes of abdominal pain in the months after the colonoscopy. After one of the episodes he underwent a small-bowel follow through which demonstrated 18 cm of abnormal small bowel in the distal ileum with effacement of the small bowel folds (Figure ). No mention of the ileal mass was made on the small bowel follow through.\nThe patient then agreed to surgical evaluation and underwent laparoscopic assisted right hemicolectomy. Exploratory laparotomy showed a thickened portion of ileum. Pathologic exam showed a 3 cm inflammatory fibroid polyp composed of an edematous stroma containing spindle shaped stromal cells, lymphoid nodules and eosinophils (Figure ). There was an adjacent "Crohn's like" inflammatory reaction similar to that seen in the initial biopsies. Granulomas were not identified (Figure ).\nThe patient has been symptom free for more than three years. He has had no further obstructive symptoms and he has no symptoms suggesting Crohn's disease.
A 25-year-old female student presented to us with a complaint of intermittent headache with tremor dysarthria and mild weakness on the right side of her body. The patient had no history of ptosis, diplopia, or decreased vision. There was no record of any systemic diseases and connective tissue disorders. Initially, she was managed symptomatically and evaluated.\nNeurological examination of the patient was not significant.\nComputed tomography of the brain and angiogram and magnetic resonance imaging were done followed by digital subtraction angiography which showed bilateral large cavernous segment aneurysms of the internal carotid artery (ICA) right more than left with fusiform dilatation of V2 segment of vertebral arteries and right ICA at CCA bifurcation []. Although endovascular management was at our disposal, we preferred microvascular management because of the following reasons:\nIn the present case, the aneurysm neck was too wide for single flow diverter to be considered as an option. Although multiple flow diverters can be used in tandem, they are found to be more unstable and failure can occur Our patient was young and flow diverter use would have left her on lifelong antiplatelets and anticoagulants With systemic vessel wall disease, complications such as vessel wall dissection along with other known complications such as thromboembolic phenomenon from the thrombus in giant aneurysm can happen even at experienced hands during endovascular procedure Due to our expertise in microvascular neurosurgery and patients and her relatives choice, microsurgical management in form of extracranial–intracranial (EC–IC) bypass was opted.\nThe patient underwent preoperative workup and optimized for planned surgery.\nHigh-flow EC–IC bypass with radial artery graft with insurance superficial temporal artery-middle cerebral artery (STA-MCA) bypass was planned. Intraoperatively, the right radial artery which was exposed for harvesting was found to have abnormal fusiform dilatation in its wall. Decision of harvesting the saphenous vein from the right lower limb instead of the radial artery as a graft was made. The rest of the procedure went uneventful with STA-MCA bypass then EC-IC high-flow bypass with saphenous vein graft and ligation of the right ICA at CCA bifurcation. Intraoperatively, indocyanine green and dual-image video angiography revealed functional bypass and no flow in right ICA. Due to suspicion of vessel wall abnormality encountered at the right radial artery and right ICA, wall biopsy of the right ICA, right saphenous vein, and right extracranial aneurysm (ECA) was sent for histopathological examination []. H and E staining, Azan staining, and elastic-Van Gieson staining were used. Biopsy from right ICA showed decreased density of elastic fibers in intima of the vessel. Disturbed angioarchitecture with drop out and tears and fragile vessel wall []. It was reported to be suggestive of Ehlers–Danlos syndrome (EDS). Postoperatively, the patient did well and had transient right hypoglossal nerve palsy due to handling during the isolation of cervical ICA and preparing tunnel for the saphenous vein graft. The 12th cranial nerve palsy improved during the patient hospital stay. The patient is planned for endovascular procedure on the left side.
A 34-year-old woman presented with complaints of umbilical hernia and had no secondary infection on clinical examination. The surgery was performed under conscious sedation and analgesia procedure which is routinely preferred by the anesthetists for minor surgeries. The patient experienced sudden, severe hemodynamic deterioration after undergoing this minor surgical procedure. Spontaneous breathing was maintained by continuous infusion of propofol at a rate of 100 μg/kg/min to achieve conscious sedation after administering fentanyl 100 μg intravenously. During surgery, the patient remained hemodynamically stable. Patient had nausea and vomiting in the early postoperative period, which were treated with 4 mg of intravenous ondansetron. A few minutes later, the patient experienced hemodynamic instability with sinus tachycardia and hypotension. Her heart rate was 122 regular beats/minute, and the blood pressure was 50/30 mmHg. The oxygen saturation had decreased to 80% as recorded by pulse oximetry, despite oxygen supply through face mask. She was immediately treated with intravenous dopamine and dobutamine as well as with ventilator support due to acute respiratory failure. The arterial gasometry showed oxygen pressure 40 mmHg and carbon dioxide pressure 49 mmHg; the electrocardiogram monitor showed sinus tachycardia. The signs of heart failure (HF) were found in the bedside digital X-ray of chest and emergency transthoracic echocardiography (TTE) revealed severe biventricular dysfunction with global hypokinesia. The LVEF was 42%. Coronary angiography showed no coronary lesions. An intra-aortic balloon pump was introduced for counterpulsation. The repeat TTE revealed LVEF 32% and a left ventricular extracorporeal membrane oxygenation (ECMO) device was inserted to assist the impaired clinical course of the patient. The LVEF gradually improved and on the 5th postoperative day the ventilatory support and circulatory perfusion assistance were removed. Troponin T and C reactive protein levels remained within normal limits. The other routine investigations like chemistry panel, complete blood cell count, and coagulation profile were within normal limits. The serology battery for myocarditis, blood cultures, urine cultures, and cytotoxic antibodies were found insignificant. An endomyocardial biopsy was not performed because of its low diagnostic yield. Three weeks later, a new TTE showed a non-dilated left ventricle, absence of segmental contractility alterations, and an LVEF in the normal range. She was reviewed for 6 months in out-patient department and was found asymptomatic and required no further treatment.
Patient B is a 34-year-old woman, a full sister of Patient A. After Patient A received the diagnosis of 16p11DS, a genetic assessment was suggested for Patient B too, as she shares some peculiar constitutional traits with her brother, such as obesity. The array CGH analysis confirmed the presence of the same microdeletion in the p11.2 region of chromosome 16, leading to the diagnosis of familial 16p11DS.\nAt the moment of clinical evaluation, Patient B was 151 cm tall and weighed 126 kg, with an equivalent BMI of 55.2, and she was nullipara. She was born from a eutocic delivery, and the speech and psychomotor development was described as normal. The patient referred to some difficulties in relationships with peers and ascribed to her own shyness and suspiciousness and some bullying issues in her middle and high school years. However, relational skills have always resulted in the norm, and the patient has never shown behavioral alterations attributable to a neurodevelopmental defect. Since primary school, her scholastic record was mediocre, and she left high school when she was 16 to start working initially as a kitchen assistant and then changing to several practical works. Nowadays, she works as a cashier in a supermarket, a job that she has been doing regularly for 8 months. Patient B has no history of psychiatric disorders, with the only exception of an episode of generalized anxiety with insomnia, which occurred in 2019 after her brother's physical aggression and hospitalization in the psychiatric ward. Such symptoms lasted about 1 month and were treated for 10 days with low dosages of oral delorazepam by one of the psychiatrists of our outpatient facility. After this acute episode, which fulfilled the diagnostic criteria for the adjustment disorder with anxiety, Patient B obtained a complete remission of symptoms, without the need for further neuropsychological therapies or follow-up.\nAs for her medical history, Patient B has been suffering from severe obesity since she was 10 years old, for which she has been followed by the nutrition services, with a hospitalization in the nutrition and metabolic ward in 2013, at the age of 25, and a following outpatient care programme lasting till 2017. During this period, Patient B has been able to lose 70 kg, reducing her BMI from 66 to 55, a value that she is currently maintaining. In 2013, she suffered from insulin resistance, from which she recovered with nutritional care. In the last years, Patient B developed a gonarthrosis, secondary to obesity, yet needing infiltrative treatment. The current drug therapy of Patient B consists of periodic cycles of injective folic acid and iron supplementation. With the exclusion of the above-mentioned brief consumption of delorazepam, Patient B was not prescribed any other psychiatric drug. Patient B has no lifetime history of substance abuse or addiction.\nFor a better consistency of the case presentation, Patient B has been assessed in our outpatient facilities with the same clinical interviews and neuropsychological tests as her brother.\nPatient B's SCID-CV did not highlight the presence of current or past major psychiatric disorders, with the exception of the adjustment disorder with anxiety from which she suffered for 1 month in 2019. Similar to Patient A, the SCID-PD dimensional scoring of Patient B showed sub-clinical avoidant and paranoid personality traits (8 and 5 out of 14, respectively), though not fulfilling the DSM-5 criteria for any full-blown personality disorder. On the other hand, the MMPI-2 did not confirm the same personality profile as SCID-PD; notably, MMPI showed an underreporting profile (L scale T score = 77) linked to a deliberate minimization of pathological features in order to give a more positive self-image. Anyway, the MMPI-2 revealed a personality profile featured by conformation to social rules and faith in others, excessive self-confidence, and overestimation of her capacity to fulfill the demands of the surrounding environment. No autistic traits were detected both at a dimensional evaluation with the MMPI and at a clinical psychiatric observation. As for the neuropsychological assessment, the WAIS-IV of Patient B showed an intellectual functioning at the lower level of the norm, with a total IQ score of 86. The attentive and executive functioning assessment was globally in line with the age and the schooling of Patient B. More specifically, the TMT did not reveal any deficit in visual attention and task switching, and the executive functional testing only showed a high score of 5 at the bizarreness index of the Cognitive Estimation Test (CET).
A 17-year-old boy came with a chief complaint of lump and pain on his left elbow. He previously fell from the stairs about 1 m in height with the elbow hitting the ground, but the pain is subsided in two days. One month later, the patient felt there was a lump with a size of a marbleon the left elbow, which getting bigger to a size of a tenis ball, and was accompanied by pain. General examination showed there was no abnormality but the patient complained of decrease 5 kg of total bodyweight. The local state of the left elbow shows fixed, solid mass on the posterior aspect of the elbow, with the size 14x12cm, 30 cm circumferentially (contralateral 27 cm) (). The range of motion of the left elbow joint could not be measured because of pain. On radiographs obtained at the first examination, an osteolytic lesion and periosteal reaction of the distal humerus was observed (). On magnetic resonance imaging (MRI), a low intensity was observed on T2-weighted imaging (). Computed tomography of the chest showed that there was no abnormality. The patient underwent histopathological and immunohistochemistry examination, with the result of conventional osteosarcoma (). The diagnosis of osteosarcoma was made.\nThe patient underwent neoadjuvant chemotherapy with cisplatin, ifosfamide, and adriamycin for 3 cycles. Limb salvage surgery by wide excision, cryosurgery followed by total elbow arthroplasty and ORIF with plate and screw. The patient was positioned right lateral decubitus position under general anaesthesia. The tumor was exposed and shaft humerus osteotomy within 5 cm above the tumor was performed. The radial artery, radial nerve, and ulnar nerve was identified and preserved. The bony segment was resected and prepared for autoclave processing. A thorough debridement of all the tumor and soft tissues from the resected bony segment was performed and the excised bony segment was frozen in pot containing liquid nitrogen for 20 min, thawed at room temperature for 15 min, thawed at room temperature for 15 min. The excised segment then thawed in distilled water for 10 min and reimplanted and fixed in place. Total elbow arthroplasty and ORIF with plate and screw was performed, the bone cement was also added. The implant was secured using advancement of extensor carpi ulnaris muscle and the wound was closed using primary suture.().\nPostoperatively we performed an X-Ray to evaluate the reconstruction. From the X-ray, the plate and screws are well-fixated (). One month postoperatively, the implant was well-fixated and the patient can slowly move his elbow without limitation.
A 55-year-old male patient reported with mild swelling and pain in the left zygomatic region. The history of present illness revealed that the patient had noticed the swelling in the last few weeks and it also had an associated intraoral ulcer. The patient was a known diabetic who was under medication. He had a habit of smoking cigarettes for the past 15 years. He smoked almost 15 cigarettes per day. The clinical examination revealed a firm swelling in the left zygomatic region measuring approximately 3 cm to 5 cm. It was mildly painful on palpation. The left maxillary region had significant paresthesia, nasal obstruction, and episodes of pain. A palpable left submandibular lymph node was present, which was also tender and fixed. The cervical lymph node on the left side was also palpable (Figure ). The intraoral examination revealed an ulceroproliferative growth measuring 4 cm to 6 cm in dimension.\nThe lesion was extending in relation to teeth 23, 24, and 25. The ulcer was covered with necrotic slough. Purulent discharge and bleeding were present in the lesion. The other teeth in the quadrant were missing (Figure ). A panoramic radiograph revealed extruded teeth 23, 24, 25.\nThere was increased radiopacity in the left maxillary sinus (Figure ). Water’s view showed the opacification of the entire left maxillary sinus.\nAn intraoral extension of this mass was also evident. The inferior, posterior, lateral, and medial walls of the left maxillary sinus appeared to be destroyed (Figure ). A computed tomography (CT) scan showed a lesion extending into the maxillary space and the nasal cavity. A heterodense soft tissue lesion showing heterogeneous contrast enhancement in the left maxillary sinus and hard palate with the destruction of the posterolateral wall, medial wall, and floor of the left maxillary antrum, extending into the adjacent retro maxillary space and medially extending into the left nasal cavity, obliterating all meati with the destruction of nasal turbinates. It was also inferiorly extending into the oral cavity. The CT was suggestive of carcinoma antrum. The CT was sufficient to understand the extensions of the lesions and the destruction of nasal turbinates. Further radiological investigations were, hence, not considered.\nConsidering the patient history and clinical features and the fact that patient was experiencing paresthesia, a biopsy was deemed mandatory (Figure ). On microscopic examination, the given hematoxylin and eosin (H&E)-stained soft tissue section showed dysplastic epithelial islands arranged in sheets and nests, invading fibrovascular stroma.\nAs shown in Figure , the dysplastic epithelial cells exhibited an increased nuclear-cytoplasmic ratio, individual cell keratinization, and increased mitotic figures.\nKeratin pearl formation was also evident, suggestive of well-differentiated squamous cell carcinoma. The patient was referred to the maxillofacial surgery department and briefed on surgical and chemotherapeutic treatment modalities. As the patient was from a poor socio-economic background and had also reported at an advanced stage, he declined treatment and was advised palliative management. The palliative treatment mainly concentrated on the pain relief and nutritional needs of the patient. The patient was treated with acetaminophen and, later, with stronger medications like opioids to manage pain. A feeding tube was inserted into the stomach through the throat since the patient was having extreme difficulty in swallowing. Sadly, the patient succumbed within a few weeks.
A 77-year-old woman with a sudden onset of left hemiparesis was admitted to our hospital. She had a previous history of hypertension and usage of aspirin and antihypertensive drugs and was suspected of acute stroke.\nAfter admission to the neurology ward and initiation of anticoagulant therapy, she progressed a severe neck pain irradiating in both shoulders, particularly to the left side, urinary incontinence, and quadriparesis. There was no previous history of neck trauma. The patient was conscious on examination and showed normal cranial nerve functions. She experienced pain in neck with a movement limitation. The neurological examination revealed quadriparesis which was more severe at the left side (left upper extremity force of M3 proximally and M2 distally). Muscle strength of the left lower extremities both in distal and proximal was M1. Muscle strength in the right side was M3 in the upper and lower extremities. Deep tendon reflexes decreased in the left extremities, without evidence of sustained clonus, and the Babinski sign was positive in the left foot. The patient was treated by enoxaparin and aspirin due to initial suspicion of stroke with hemiparesis. However, quadriparesis and intensified neck pain were seen on the 2nd day. Thereafter, X-ray images of her neck showed apparent degenerative changes. Magnetic resonance imaging (MRI) scans showed a lesion in the posterior epidural space of the neck parallel to C3-T1 area in T1 and T2 sequences with mixed signals, as well as spinal cord edema due to cord compression [Figures and ]. The expansion of the cervical epidural hematoma was likely due to anticoagulant usage by the patient.\nThere was a hyperdense area in epidural space in the CT scan []. The patient underwent an emergent cervical decompressive laminectomy wherein a dark brownish epidural hematoma compressing the spinal cord was found and resected. Following the surgery, the patient showed an improvement in the muscle forces of four limbs the day after surgery and gained complete muscle strength after physiotherapy.
Patient A, a 34-year-old single male, was recruited as a case of genetic high risk for schizophrenia 11 years ago, and underwent yearly follow-up evaluation. In his family pedigree, both of his parents had been diagnosed with schizophrenia, as well as one of his uncles. He had been suffering from bizarre psychotic behaviors and inappropriate rearing of the both parents from childhood. While he had not been quite interested in interpersonal relationship or academic achievement, he did not show any clinical symptoms at the time of baseline assessment. A diagnosis of schizoid personality disorder was given. Until 3 years of followed up, despite of stress from his parents' disease and his mother's death, he was free of psychiatric symptoms and at good functional status. He no more felt the need to participate in the follow-up program and he refused follow-up assessments afterwards. Thus, from 2008 till 2011, he had been lost to follow-up, although later retrospective assessment strongly suggested the presence of sustained distress during this period. In Jul 2012, he wanted psychiatric counseling for depressive mood, anger, and communication problem. However, his symptoms were very mild and he was still functioning well in his life. Although there were some psychiatric symptoms, he did not meet the criteria for ultra-high risk for psychosis. In Dec 2013, after 9 years of follow-up, attenuated psychotic symptoms such as idea of reference, suspiciousness occurred, the severity fulfilling the ultra-high risk for psychosis. He was diagnosed as both genetic risk and deterioration and attenuated psychotic symptoms syndrome of ultra-high risk for psychosis and also as depressive disorder not otherwise specified. We tried intensive cognitive behavioral therapy and supportive psychotherapy but his compliance was poor. In Jul 2014, he showed persecutory delusion with a high level of conviction and his transition to a psychotic disorder was suspected. Disorganized speech and negative symptoms were also observed. We assessed him as a case of transition to schizophrenia and atypical antipsychotic medication was prescribed. In Dec 2015, a11-year follow-up as a GHR individual and 2-year follow-up as a CHR individual and 1.5 year after onset of schizophrenia, his psychotic symptoms were quite alleviated but general functioning has not yet improved sufficiently.
A 55-year-old male patient reported with mild swelling and pain in the left zygomatic region. The history of present illness revealed that the patient had noticed the swelling in the last few weeks and it also had an associated intraoral ulcer. The patient was a known diabetic who was under medication. He had a habit of smoking cigarettes for the past 15 years. He smoked almost 15 cigarettes per day. The clinical examination revealed a firm swelling in the left zygomatic region measuring approximately 3 cm to 5 cm. It was mildly painful on palpation. The left maxillary region had significant paresthesia, nasal obstruction, and episodes of pain. A palpable left submandibular lymph node was present, which was also tender and fixed. The cervical lymph node on the left side was also palpable (Figure ). The intraoral examination revealed an ulceroproliferative growth measuring 4 cm to 6 cm in dimension.\nThe lesion was extending in relation to teeth 23, 24, and 25. The ulcer was covered with necrotic slough. Purulent discharge and bleeding were present in the lesion. The other teeth in the quadrant were missing (Figure ). A panoramic radiograph revealed extruded teeth 23, 24, 25.\nThere was increased radiopacity in the left maxillary sinus (Figure ). Water’s view showed the opacification of the entire left maxillary sinus.\nAn intraoral extension of this mass was also evident. The inferior, posterior, lateral, and medial walls of the left maxillary sinus appeared to be destroyed (Figure ). A computed tomography (CT) scan showed a lesion extending into the maxillary space and the nasal cavity. A heterodense soft tissue lesion showing heterogeneous contrast enhancement in the left maxillary sinus and hard palate with the destruction of the posterolateral wall, medial wall, and floor of the left maxillary antrum, extending into the adjacent retro maxillary space and medially extending into the left nasal cavity, obliterating all meati with the destruction of nasal turbinates. It was also inferiorly extending into the oral cavity. The CT was suggestive of carcinoma antrum. The CT was sufficient to understand the extensions of the lesions and the destruction of nasal turbinates. Further radiological investigations were, hence, not considered.\nConsidering the patient history and clinical features and the fact that patient was experiencing paresthesia, a biopsy was deemed mandatory (Figure ). On microscopic examination, the given hematoxylin and eosin (H&E)-stained soft tissue section showed dysplastic epithelial islands arranged in sheets and nests, invading fibrovascular stroma.\nAs shown in Figure , the dysplastic epithelial cells exhibited an increased nuclear-cytoplasmic ratio, individual cell keratinization, and increased mitotic figures.\nKeratin pearl formation was also evident, suggestive of well-differentiated squamous cell carcinoma. The patient was referred to the maxillofacial surgery department and briefed on surgical and chemotherapeutic treatment modalities. As the patient was from a poor socio-economic background and had also reported at an advanced stage, he declined treatment and was advised palliative management. The palliative treatment mainly concentrated on the pain relief and nutritional needs of the patient. The patient was treated with acetaminophen and, later, with stronger medications like opioids to manage pain. A feeding tube was inserted into the stomach through the throat since the patient was having extreme difficulty in swallowing. Sadly, the patient succumbed within a few weeks.
A 37-year-old male was first seen at the tertiary Headache Centre at the National Hospital for Neurology and Neurosurgery, UK, in September 2013 due to complex hemiplegic migraine. He was born to healthy non-consanguineous parents following an unremarkable pregnancy and perinatal course. His brother suffered from migraine, but otherwise there was no family history suggestive of hemiplegic migraine, neurological diseases or cerebellar syndromes. He failed to meet developmental milestones at 6 and 12 months of age and was subsequently diagnosed with athetoid cerebral palsy in a setting of hypotonia. During childhood, he exhibited signs of cerebellar ataxia, global development delay and mild intellectual disability. He experienced his first generalized tonic-clonic seizure at the age of 3 years following a mild head trauma at school, when a toddler hit his head with a toy car. This resulted in a coma and massive cerebral oedema requiring ICU admission and treatment with mannitol. During childhood he had several similar episodes of seizure-associated encephalopathy, for which no cause was found. None of these subsequent episodes resulted in cerebral oedema. He also experienced episodes of petit mal seizures. An EEG at the age of 13 years showed a sharp focus on the left temporal region. Notably, a muscle biopsy showed no evidence of mitochondrial disease.\nFrom the age of 7 years he started suffering from headaches. The episodes usually started with difficulty using the arm or leg followed by a facial drop on one side, blurred vision and slurred speech. The visual and speech symptoms lasted for approximately 90 min and were succeeded by a severe headache lasting from 2 days to a few weeks. The hemiparesis persisted during the headache, associated with photophobia and motion sensitivity. Initially he had 2–3 episodes per year, but this increased to 4 annually in his teenage years. All episodes were associated with mild head trauma or minor illness, usually viral infections.\nHe was at the time of referral taking Calcichew and alendronic acid for fracture of the femur and paracetamol for his headaches. Neurological examinations revealed slurred speech, slow tongue movements with brisk jaw jerk, hypotonia, pathologically brisk reflexes and an extensor plantar reflex on the right. There was no ataxia or nystagmus during examination at the headache centre.\nBecause of the hemiplegic attacks complicated by minor head trauma, and the episodes of encephalopathy during childhood, a severe CACNA1A mutation was suspected and a series of investigations including genetic testing was initiated. Neuropsychological testing revealed verbal and non-verbal scores in the impaired ranges, with a verbal IQ of 52 and a performance IQ of 55. MRI of the brain and cervical spine showed marked cerebellar hemispheric and vermian atrophy (Fig. ). The neurophysiological studies were unremarkable. Even though a CACNA1A mutation was suspected, whole genome sequencing was carried out on the patient and both parents, as a part of the 100,000 Genomes Project (Genomics England). The patient had not undergone genetic testing prior to the sequencing. Segregation analysis identified a de novo missense mutation in exon 25 of CACNA1A (c.4055G>A, p.R1352Q). This mutation was absent in his parents. His brother was not tested for the mutation. Figure indicates the location of the mutation in the protein. The mutation was located in a highly conserved region of the protein (online Supplementary Material; for all online suppl. material, see ) and SIFT [] and PolyPhen [] scores indicated a deleterious and damaging effect of the mutation.\nThe patient continues to have approximately one hemiplegic migraine attack yearly, often triggered by episodes such as falling or hitting his head. He also has episodes of hemiplegic migraine in the absence of minor head trauma, but has not had episodes of encephalopathy or cerebral oedema since childhood. He keeps himself fit and active, regularly going to the gym and golfing, and is currently functioning well in a part-time job and has attended college for adult education and life skill classes. He is advised to strictly avoid activities that involve any risk of trauma to the head and is using naproxen 500 mg with good effect on migraine aura and pain.
A 46-year-old female patient reported to our hospital and presented with a history of painless mass which had grown over a period of 6 months to the present size of 2 cm × 3 cm. She complained of mild obstruction of nose and thereby changes in her voice. On clinical examination, she had a well circumscribed sessile growth with irregular nodular surface arising from the posterior part of right maxilla []. The lesion extended anteriorly from the mesial aspect of right maxillary premolar region up to the junction of hard and soft palate posteriorly. Medially, the lesion was crossing the midline toward left side by around 2 mm and laterally it had involved the right maxillary alveolar ridge. The lesion was firm in consistency and the color was similar to that of surrounding normal mucosa. No secondary changes like ulceration were seen and no lymph nodes were palpable. On computerized topography examination, there was a well-circumscribed mass causing bony erosion and invasion of hard palate []. Based on these clinical and imaging features, it was provisionally diagnosed as malignant salivary gland tumor, most commonly mucoepidermoid carcinoma, and planned for right subtotal maxillectomy to avoid recurrence in future. The resected mass was well circumscribed and measuring about 2.5 cm × 1.5 cm []. The mucosal cut margin clearance was 1–2 cm, and the depth of clearance was 2.5 cm.\nOn histopathological examination, the multiple sections studied from the hematoxylin and eosin stained slides showed a well-circumscribed tumor mass composed of myoepithelial cells arranged in large lobules, sheets, and large nests. The cells are spindle and plasmacytoid shaped with a moderate amount of clear to pale eosinophilic cytoplasm. Nuclei were round to ovoid in shape and appeared relatively bland []. No ductal structures were evident and cellularity was more with minimal atypia, and without significant pleomorphism. Stromal hyalinization was noted and the overlying mucosa was intact and nonneoplastic. No mucous or intermediate cells were seen, ruling out the possibility of provisional radiological diagnosis, mucoepidermoid carcinoma. Thus, the final histological diagnosis based on these features was benign myoepithelioma.\nSince bony erosion and invasion are uncommon in benign lesions and for academic purpose, immunohistochemistry (IHC) was performed, which revealed a strong and diffuse positive reaction to S100 protein and smooth muscle actin. These IHC findings confirmed that the tumor cells were myoepithelial cells, consistent with the histopathological diagnosis of “benign myoepithelioma” of salivary gland origin.\nAfter the surgery, the patient had been under regular follow-up and no recurrence was observed in this 2 years period of follow-up. The surgical site also revealed the coincidental presence of nasal polyp in the patient during microscopic examination.
A 66-year-old man presented with intermittent dizziness for two years and aggravation for 10 d. The patient have been smoking for more than 30 years, with more than 20 cigarettes a day. He had drunk for more than 40 years and has stopped drinking now. The patient had a more than 10-year history of type 2 diabetes and severe diabetic eye disease with blindness in the left eye. The patient self-injected long-acting insulin subcutaneously, 12 units per day. The patient denied having other diseases. His father died of heart disease at the age of 72 years. His mother and children are healthy. First admission to hospital in March 2018: On physical examination, the patient’s blood pressure in the left upper limb was 120/70 mmHg, and blood pressure was not detected in the right upper limb. The patient was conscious and able to answer questions fluently, no abnormalities were detected on a nervous system examination, and the right brachial artery and radial artery pulsations were not detected. Second admission in October 2018: The pressures in the left and right upper limbs were 140/68 mm and 85/60 mmHg, respectively. The results of a nervous system examination were normal. The right radial artery pulsation could not be detected, but the left radial artery pulsation was accessible. The patient was conscious and could carry on a normal conversation but had slightly slurred speech. Imaging studies including a computed tomography (CT) scan of the head, computerized tomography angiography (CTA) of the vessels of the head and neck, and cerebral angiography. Upon the first admission to hospital in March 2018, CTA of the vessels of the head and neck showed severe stenosis at the beginning of the brachiocephalic artery. A CT scan of the head showed no obvious abnormalities. Cerebral angiography showed that the stenosis of brachiocephalic artery had almost occluded it (Figure ). Upon the second admission in October 2018, a CT scan of the head revealed multiple cerebral
A 63-year-old woman complained of a slightly swollen left leg and low lumbosacral pain (visual analog scale score: 4), and was admitted to our department. She had a history of diabetes mellitus and hyperlipidemia for more than 20 years, and these were treated by medications prescribed by her endocrinologist and cardiologist. She had an L3 OVCF, with a visual analog scale score of 8 2 months previously, and was successfully treated with PVP. She was discharged on postoperative day 2, with no cement-associated complications and a visual analog scale score of 2 (. Accordingly, a newly fractured vertebra was suspected at admission to our department. A physical examination showed a slightly swollen left leg with no other major complaints. The intensity of the low lumbosacral pain did not increase when she slightly twisted her spine. No major abnormalities of a regular laboratory test were observed.\nThe patient suddenly fainted when she was taking a shower on admission night 2. After general resuscitation of moving her back to the bed and resting with electrolyte fluid supplementation, she gradually became conscious and returned to a normal status. The next day, magnetic resonance imaging of the spine, computed tomography angiography, and ultrasonography were performed. No newly fractured spinal vertebra was detected. No major abnormality in the arteries or deep veins in both lower extremities was detected on ultrasound. A cement leakage that started from the L3 level and migrated to the L1 level, and was suspended in the inferior vena cava was detected (. Another cement leakage was detected in the right pulmonary artery (. After 4 days of general conservative therapy, the patient became agitated and her left leg started swelling (. Her condition became severe. General anti-coagulation therapy with 3000 units of low molecular weight heparin daily was empirically implemented. Relevant blood tests were performed again and showed the following: D-dimer concentration, 9.45 mg/L (normal range: 0–0.55 mg/L); hemoglobin concentration, 69 g/L (normal range: 115–150 g/L); red blood cell count, 2.43 × 10^12/L (normal range: 2.8–5.1 × 10^12/L), and hematocrit, 29.1% (normal range: 35%–45%). The patient later complained of slight left low abdominal pain, with no rebound, and an acute abdominal hemorrhage was suspected. Thereafter, enhanced computed tomographic scanning of the abdomen was performed. Thrombotic embolism in the inferior vena cava and bilateral iliac veins was detected, and a lower abdominal hematoma around the left iliopsoas muscle was also detected (.\nAfter consultation of our vascular interventionist, anti-coagulation therapy was temporarily stopped. Digital subtraction angiography was performed, which further confirmed our diagnosis. However, owing to the wide range of thrombotic embolism formation, no further interventional therapy, such as embolism extraction and filter insertion, was performed during the surgery. We then arranged for a multiple disciplinary team for assistance and also asked for help from prestigious vascular specialists in our province. Specialists empirically advised that a wide venous collateral network would eventually form. Therefore, conservative anti-coagulation therapy was recommended. The patient was then transferred to the Vascular Department for further anti-coagulation therapy, with 3 days of conservative therapy of blood transfusion and nutritional supplementation. The patient’s condition stabilized. A dissolved and stabilized left lower abdominal hematoma was detected on a computed tomography scan (. Therefore, anti-coagulation therapy with 5000 units of low molecular weight heparin daily was uneventfully administered. Fortunately, the patient’s condition became stable and the swelling in her left leg started to improve (. She was discharged home on day 21 with a prescription for 10 mg oral rivaroxaban daily. Long-term follow-up of this patient is on-going.\nThe reporting of this study conforms to the CARE guidelines.
A 56-year-old female patient with an unremarkable medical history visited our clinic with complaints of swelling, ecchymosis, and pain on the left side of her face. The dental history revealed that an endodontic retreatment therapy had been initiated on her maxillary left first premolar tooth 10 days previously, which was followed by a second session on the previous day. During the first session, the patient had felt light swelling, which resolved spontaneously a few hours later. At the second appointment, the treatment had to be stopped because of severe pain and hemorrhage from the root canal during irrigation of the root canal. No attempt had been made to restore the tooth other than placing a cotton pellet into the endodontic cavity. Another appointment had been scheduled by the general dentist to complete the treatment. The patient noticed swelling in her cheek several hours following the procedure, but she did not contact her doctor. However, her facial swelling worsened significantly over the next 24 hours, and the general dentist referred her to our clinic.\nApproximately 48 hours after the incident, extraoral examination revealed significant soft tissue swelling extending from the left infraorbital region to the mandibular border. Infraorbital ecchymosis and slight bruising near the nasolabial fold were observed (). Intraorally, there were no signs of mucosal ulceration or necrosis. The tooth being treated was tender to vertical and horizontal percussion. Panoramic radiography showed evidence of a previous root canal therapy with periapical radiolucency ().\nThe root canals were irrigated with saline solution, and the tooth was restored temporarily. To prevent the risk of infection, intramuscular clindamycin (600 mg twice a day) was administered for three days, and ibuprofen (400 mg) was prescribed for pain management, to be taken as required. Use of cold packs externally for the first day was replaced by warm compresses on the second day for treatment of the swelling. On the first recall, an increase in the ecchymosis was noticed; however, the swelling had decreased significantly (). Both swelling and ecchymosis kept progressively decreasing during the follow-up period, and the patient became asymptomatic on the 10th day after the procedure (). For the evaluation of the relationship between the tooth, alveolar bone, and the maxillary sinus, dental volumetric tomography (DVT) (Newtom 3G, QR s.r.l., Verona, Italy) was performed. DVT images revealed that the apex of the buccal root canal had perforated the maxillary cortical bone, creating a pathway for the solution into the soft tissues (Figures and ). After 10 days, the symptoms had completely resolved. At this time, a root canal dressing with Ca(OH)2 was placed in the canal. At the final appointment approximately 4 weeks after the procedure, the root canals were filled with AH Plus (Dentsply Maillefer) and gutta-percha ().
A 57-year-old woman with known hypothyroidism presented to her general practitioner with a few months’ history of orthopnoea and intermittent hoarseness. An ultrasound showed right-sided thyroid enlargement with an isthmus nodule extending to the left lobe. She developed progressive tightness in the neck and was seen in the rapid access ENT clinic. Flexible nasendoscopy examination was normal and the patient underwent urgent ultrasound-guided fine needle aspiration cytology (FNAC), which suggested possible lymphocytic thyroiditis. Subsequent core biopsy showed scanty lymphocytes and was non-diagnostic.\nNineteen days following ENT review, she presented to the ED with worsening shortness of breath. The patient was diagnosed with type 2 respiratory failure with acidosis and raised troponin. A CT pulmonary angiogram (CTPA) on admission demonstrated severe ground glass shadowing. She was therefore admitted under the medical team with a working diagnosis of atypical pneumonia. An ECG demonstrated a reduced ejection fraction of 32% with left ventricle apical hypokinesia and lateral wall akinesia. Cardiac MRI demonstrated an apical left ventricle hypertrophy suggestive of cardiomyopathy. As a result, a diagnosis of Takotsubo stress cardiomyopathy was made.\nDuring her inpatient stay, the patient suffered a cardiac arrest. Whilst spontaneous ventilation was achieved through cardiopulmonary resuscitation, worsening type 2 respiratory failure required intubation and mechanical ventilation in the ICU. The cause of arrest was attributed to acute respiratory distress syndrome secondary to acute pulmonary edema.\nIn light of her cardiorespiratory deterioration, the admission CTPA was re-reviewed where a large goiter with heterogeneous enlargement was noted with significant tracheal compression resulting in a minimal lumen size of 6 mm (Figure ).\nNo direct invasion of the trachea was seen on imaging. The radiological and clinical impression was that of a rapidly progressive and aggressive thyroid malignancy such as anaplastic carcinoma, rather than thyroiditis, as suggested on ultrasound. In view of the airway obstruction, the etiology of the ground glass appearance of the lungs was revised to pulmonary edema secondary to negative pressure respiration rather than an infective cause.\nOn day two of intubation, ENT was approached regarding surgical management of the airway, as the patient was deemed unsuitable for extubation due to the degree of tracheal compression. Therefore, a hemithyroidectomy of the larger right lobe of the thyroid was planned to decompress the trachea, gain a histological diagnosis and facilitate extubation.\nIntraoperatively, the thyroid lesion was more consistent with an infiltrative pathology involving the strap muscles. The thyroid was grossly enlarged, homogeneously firm with an appearance more suggestive of lymphoma rather than carcinoma. The trachea was identified distally, and the thyroid cartilage was exposed to gain control of the field. The infiltration extended laterally toward the carotid sheath, hence an attempt at a formal hemithyroidectomy was not feasible. On dissecting the thyroid tissue from the trachea, it was evident on the right lateral aspect that there was a clear invasion into the lumen of the trachea. This was inspected using a 0-degree and 30-degree Hopkins rod. It was subsequently decided that a wedge resection of the thyroid down to the trachea and the formation of a tracheostomy would be the safest option. A size 8-0 cuffed Shiley tracheostomy tube was placed.\nThe patient was stepped down to a ward-based level of care after 17 days in ICU. Histological diagnosis revealed large pleomorphic lymphoid blasts, admixed with small lymphocytes and histiocytes. Immunohistochemistry revealed the lymphoid cells to be positive with CD20, PAX5, and BCL-2, with an expression of CD10 and BCL6. In addition, the cells were negative with CD3, CD5, CD21, CD23, cyclin D1 and MUM 1. Ki 67 proliferation fraction was found to be between 60 and 70%. Stains for TTF-1 and CK8/18 were negative. Consequently, the features were compatible with a high-grade B-cell lymphoma and most likely, diffuse large B-cell lymphoma (DLBCL).\nUnder the care and close observation of the Haematology, Oncology, and Cardiology teams, the patient was commenced on six cycles of R-CHOP chemotherapy (four cycles at full dose, with two further cycles of rituximab). Her ejection fraction, troponin, and N-terminal brain natriuretic peptide (BNP) levels were closely monitored, as she was also given doxorubicin along her third cycle of chemotherapy. Marked improvement was noted on subsequent CT neck with contrast (Figure ).\nAn end-of-treatment positron emission tomography-computed tomography (PET-CT) scan revealed complete regression of her DLBCL.\nSince her discharge, the patient has been followed up closely by the Cardiology team and found to have restored ejection fraction on serial ECGs, as well as normal left ventricular size and function without any hypertrophy on follow-up cardiac MRI scan. The patient has also been decannulated successfully, following a brief period of observation in the hospital under the care of the ENT team.\nThe patient is currently being followed by the Hematology team at four-to-six monthly intervals and has been in remission for 18 months, without any palpable lymphadenopathy, organomegaly or masses, normalized weight, no systemic signs of infection, and normal blood test profiles (including thyroid function on maintenance dose levothyroxine).
A 57-year-old female with widely metastatic high-grade serous adenocarcinoma of the ovary was referred to our tertiary palliative care clinic by her oncologist for management of severe cancer-related pain. Following her original diagnosis, she underwent a number of multimodal disease-directed therapies, including surgical resection and chemotherapy. She had significant ongoing mixed somatic and neuropathic pain in the left lower back as well as her pelvis, both sites of known metastatic disease. The back pain was noted to be a deep sharp pain without radiation to her extremities, located primarily around the region of her nephrostomy tube and into the abdomen. She would also have sharp lancinating neuropathic pain in her pelvis due to tumor burden with prolonged periods of sitting, walking, or activity.\nInitial treatment consisted of multimodal pharmacologic therapy including short-acting and long-acting opioids with nonopioid and adjuvant neuropathic agents. Despite these therapies, she had progressive pain and was ultimately increased to an opioid combination of morphine sulfate controlled-release (120 mg by mouth every 8 hours) and morphine sulfate immediate-release (30–45 mg by mouth every 3 hours as needed). Early changes yielded acceptable analgesia. Conversations and medication adjustments continued over the phone, given the distance between the patient's home and our institution. However, over several weeks' time, pain progressed despite further opioid increases. Given concern for escalating pain and poor response to several attempts at altering her opioid therapy, it was advised that she present for a consultation in the palliative medicine clinic. Fortunately, focused discussions with the patient and her family around the patient's goals of care were addressed with her palliative medicine team at every meeting. She was very clear that her top priority in her ongoing medical care was to achieve acceptable analgesia. During the interview in the clinic, the patient noted significant pain with generalized discomfort, a sense of restlessness, and new muscle fasciculations. Her husband accompanied her and noted intermittent confusion. Her physical exam featured delirium (Confusion Assessment Method positive) with diffuse myoclonus.\nOut of concern for opioid-induced neurotoxicity (OIN), the patient was admitted to the hospital for analgesic control and treatment of what was felt to be a toxic encephalopathy. Despite the known shared metabolic pathway (phase II metabolism) between morphine and hydromorphone, the patient's opioids were rotated from oral morphine to parenteral hydromorphone as the clinical picture continued to unfold. Further workup revealed an unremarkable head CT and EEG. Clinical evaluations and laboratory assessments suggested that, in the setting of her declining physical and renal function (creatinine 1.0 mg/dL with prior baseline 0.6 mg/dL and estimated glomerular filtration rate (eGFR) 55 ml/min/BSA with prior baseline >60 ml/min/BSA), the delirium could be the result of an accumulation of active neurotoxic morphine metabolites (morphine-3-glucuronide). In addition, she was dehydrated, constipated, and had not slept in several days. Unfortunately, her delirium persisted despite correction of metabolic derangements, hydration, and an aggressive bowel regimen.\nUrology was consulted, and a nuclear medicine dimercaptosuccinic acid (DMSA) scan revealed minimal function of the left kidney, despite the presence of a nephrostomy tube. Subsequent vascular radiological investigations revealed tumor-induced thrombosis with surmised infarction of her left kidney. It was concluded that the altered renal function due to infarction likely resulted in an inability to adequately excrete the morphine, and the accumulation of polar morphine metabolites resulted in OIN. As exemplified in this case, even with opioid rotation, centrally active metabolites of hydromorphone (hydromorphone-3-glucuronide) theoretically exist, given that it follows a similar phase II metabolic glucuronidation pathway as morphine, although the relative potency and effect of such hydromorphone metabolites are thought to be significantly less than morphine. The patient continued to have signs of OIN and was eventually transitioned to parenteral fentanyl, given that fentanyl is a unique medication that is largely hepatically metabolized into inactive metabolites. Despite common lore that fentanyl and methadone are agents that do no produce active metabolites and therefore do not elicit OIN, case reports of synthetic opioids eliciting OIN do exist in the literature [, ]. Unfortunately, despite aggressive supportive measures and conversion of parenteral hydromorphone to parenteral fentanyl without reduction for cross-tolerance, she continued to have persistent pain with minimal resolution of her systemic neurotoxicity.\nGiven the concern for poorly controlled cancer-associated pain, the palliative care and pain medicine teams collaborated from the initial visit with concerning symptoms of OIN. At our institution, the palliative clinic shares space within the pain clinic, and the collaboration required a simple conversation followed by same day pain consultation. Initially, the shared thought was moving directly to intrathecal (IT) TDD therapy, as this had been discussed over numerous palliative care visits as a potential option with her physicians and was in line with her goals to achieve enhanced pain control with limited side effects. However, given the underlying metastatic disease process and concurrent cancer treatments, she had a metabolic coagulopathy due to nutritional and micronutrient deficits that required correction with vitamin k to assist in the reversal of her international normalized ratio (INR), thus allowing for any type of procedural intervention.\nUltimately, given the persistent multifactorial delirium and rapidly changing situation, the pain medicine and palliative care teams convened a family conference with the husband, the patients' health-care power of attorney, to again address the current situation and make recommendations that seemed consistent with the patient's predetermined goals. Given the rapid progression of her symptoms despite aggressive attempts to correct potentially remediable factors, the shared medical decision-making discussion included moving forward with TDD, given that this therapy was thought to be the best chance for meeting the patients' goals for comfort and hope to have meaningful interaction with family by reducing the burden of systemic side effects from oral or parenteral opioid therapy. The patient's advanced directive was clear that she appointed her husband to make decisions on her behalf if she was ever in a situation where she was unable to provide consent. Informed consent was obtained through her appointed surrogate decision maker and designated health-care power of attorney.\nAfter correction of her metabolic coagulopathy yielding an INR <1.2, she proceeded with placement of an intrathecal TDD system (Medtronic SynchroMed™ II 40 mL pump and Ascenda catheter). The pump reservoir was placed in her right lower abdominal quadrant, and the catheter tip was placed at T10. Her TDD system was efficiently titrated to achieve acceptable levels of analgesia using a combination of opioid (hydromorphone 2 mg/mL) and local anesthetic (bupivacaine 10 mg/mL). The initial postoperative settings were 0.5 mg/day of hydromorphone in a simple continuous mode without bolus dosing (2.5 mg/day of bupivacaine). Within 24 hours of TDD placement, the patient experienced near complete resolution of her toxic encephalopathy and was able to engage in meaningful conversation with her family and health-care teams. There were no signs or symptoms of opioid withdrawal. In the early postoperative phase, she required additional dosing of oral hydromorphone, but was responsive to 2 mg orally every 3 hours as needed for breakthrough postsurgical pain totaling three to four doses per day. Given the robust response to TDD therapy, the patient was able to participate in goals of care discussions with her interdisciplinary palliative care team and elected to discharge closer to home under the provision of hospice services postoperative day (POD) two. Prior to leaving the hospital, the TDD rate was increased to 0.8 mg/day of hydromorphone, and she was provided with a personal therapy manager (PTM) allowing 0.1 mg/dose every six hours totaling four doses per day. The dose remained stable for seven days. As her oncologic disease continued to evolve, her TDD system was adjusted by her hospice provider to accommodate her daily use of oral opioid therapy. The pump was again adjusted on postoperative day nine, yielding a basal rate of 1.4 mg/day of hydromorphone with four PTM boluses of 0.15 mg/dose of hydromorphone every six hours. This dose remained for POD 9–13, and she passed away peacefully with acceptable analgesia at home with her family under the auspices of hospice two weeks after TDD implantation.
A 31-year-old female presented with c/o progressive breathlessness since 6 months and recurrent episodes of cough with streaky hemoptysis since 2 months. She was initially evaluated elsewhere. Fiberoptic bronchoscopy (FOB) showed a tumor mass obstructing the left main bronchus (LMB). A biopsy was inconclusive, and, hence she was referred to our institute for further management. She has had an esophageal stent inserted for corrosive stricture of esophagus 3 years ago.\nChest examination revealed few crepitations in the left base. Complete blood count and liver and renal function were within normal limits. Pulmonary function test showed a restrictive pattern. A computed tomography (CT) scan of the thorax was done, which showed the esophageal stent eroding into the LMB []. There was no evidence of any major thoracic vessel invasion in the contrast CT scan. FOB was performed which showed the stent almost completely obstructing the LMB and mimicking a tumor mass []. Upper gastrointestinal endoscopy was performed in which only the upper part of the stent was visible, and the rest of the stent was embedded into the esophagus and covered with granulation tissue []. Since the stent could not be removed endoscopically, the decision for surgical removal was made after discussion with the thoracic and gastro intestinal surgeons.\nThe patient underwent thoracotomy with closure of the esophagobronchial fistula along with laparoscopic esophagectomy and gastric pull through. The esophagogastric anastomosis was done in the chest. Postoperative period was uneventful. Repeat bronchoscopy showed a patent LMB. Patient's symptoms improved, and she did not have any hemoptysis after the surgery. She was started on liquid diet 5 days after the surgery, following a gastrografin study which showed no leak.\nOn the 4th day after starting a liquid diet (postoperative day 9), she developed high grade fever along with cough and breathlessness. Repeat CT scan with oral contrast revealed a microleak at the anastomotic site with minimal mediastinal collection []. She was started on appropriate antibiotics based on the culture reports. The mediastinal collection drained through the thoracotomy incision. A temporary esophageal stent (covered megastent from NITI) was inserted. She improved with the above measures, and a repeat CT with contrast showed no leak. She was gradually started on a normal diet and was discharged with advice to review after 2 months for removal of the temporary stent.
We present a 28-kg, nine-year-old male who underwent successful placement of a Micra™ TPS via right internal jugular vein surgical cutdown as a first-line option. The patient presented after a witnessed syncopal episode that prompted his family to seek medical care. During evaluation, the patient experienced a second episode of syncope, which correlated with a sinus pause of 11 seconds on telemetry, prompting admission for further monitoring and testing. Telemetry demonstrated frequent three- to five-second asystolic pauses that were asymptomatic. Bedside orthostatic heart rate and blood pressure testing did not produce symptoms or reproduce sinus pauses. A complete neurologic and cardiac workup did not reveal any underlying abnormality or reversible etiology concerning the significant asystolic pauses and associated loss of consciousness. Given these results, the decision was made to proceed with pacemaker implantation.\nAs the patient was an active child participating in numerous sports, a leadless pacemaker was thus selected as the device of choice given its ability to allow our patient to continue sports participation in addition to the benefits of small size, reduced chance for certain complications, minimal cosmetic concerns, and the ability to be shut off in the future. With concern about the size of the delivery sheaths required to implant the Micra™ TPS, venous duplex scans of the lower extremities were obtained. Results demonstrated common femoral vein measurements of 7 mm bilaterally, which were deemed too small to accommodate the required 27-French (Fr) delivery sheath as, for a 27-Fr delivery system, the required vessel diameter size is, at minimum, 9 mm. Venous duplex imaging of the internal jugular veins was subsequently obtained, which demonstrated right internal jugular vein diameters of 7 mm, 12 mm, and 16 mm in the superior, middle, and inferior portions of the vessel, respectively (. Hence, the upper to middle portions of the vessel were considered borderline acceptable in terms of diameter and the lower portion was deemed more suitable for device insertion. The decision was made to proceed with the placement of a Micra™ TPS via surgical cutdown of the right internal jugular vein based upon vessel sizing as assessed through duplex studies.\nIn addition to duplex studies, thorough assessments of the cardiac structure and function were conducted prior to proceeding with Micra™ TPS placement. This constituted, first, a transthoracic echocardiogram that demonstrated normal segmental anatomy, ventricular function, and valvular function. Specifically, only trace tricuspid insufficiency was found. For better assessment of the cardiac chamber size, cardiac magnetic resonance imaging was performed, which demonstrated a right ventricular (RV) end-diastolic volume of 97 mL and RV end-diastolic volume indexed to 94 mL/m2. The volume of the Micra™ TPS is only 0.8 mL; thus, it was determined that the ventricular size was adequate for implantation as the Micra™ TPS would occupy less than 1% of the RV volume.\nThe implantation procedure was performed in conjunction with electrophysiology and vascular surgery. Cardiac imaging via transesophageal echocardiography (TEE) was used to visualize the tricuspid valve and assess for any negative impact on valve function. Patient positioning and operating room ergonomics were crucial to ensure ample space was available for all team members to work effectively. In addition, extra space was required at the head of the bed due to the length (105 cm) of the delivery cable. To achieve this, the following setup was strategized and implemented: anesthesiology at the superior right aspect of the patient, vascular surgery at the patient’s right, cardiac imaging with TEE at the patient’s left, C-arm for fluoroscope at the superior left aspect of the patient, and monitors at the foot of the bed, respectively. This allowed the electrophysiologists to work from the head of the bed with ample space to manipulate the delivery cable.\nAfter the patient was intubated and placed under general anesthesia, the neck was hyperextended and turned toward the left. After standard preparation and draping, a transverse incision was made approximately one fingerbreadth above the medial end of the right clavicle. The skin was incised, the platysma muscle was divided, and an avascular plane was developed between the two heads of the sternocleidomastoid muscle. Dissection continued to identify the internal jugular vein, and vessel loops were placed across the vessel. A micropuncture needle was inserted into the jugular vein in an antegrade fashion. Using Seldinger’s technique, a 0.014-in (0.36-mm) wire was placed, followed by a 4-Fr sheath. Next, an 8-Fr sheath was placed over a stiff 0.035-in (0.89-mm) wire. Over the stiff wire, the vessel entry site was serially dilated. At this time, the catheter was exchanged for a 16-Fr dilator, followed by the 27-Fr Micra™ delivery sheath, by the electrophysiology team. A heparin bolus of 3,000 units was administered.\nThe prepped Micra™ delivery system was flushed in the usual manner and advanced into the right atrium via the delivery sheath. The delivery catheter was inserted into the sheath, with the proximal handle rotated further counterclockwise to prevent the distal primary curve from facing toward the lateral wall of the right atrium. Due to space difficulties, as the delivery system passed through the tricuspid valve, the delivery sheath had to be pulled out of the cutdown site to allow for the delivery catheter to be positioned properly. Bleeding at the cutdown site was controlled by manual pressure via vascular surgery. After the distal delivery catheter passed into the RV, visible on both fluoroscopy and TEE in addition to producing premature ventricular complexes, the catheter handle was rotated further counterclockwise and the device cup was placed against the mid-RV septum. Contrast injection with a content mixture of one-third contrast and two-thirds saline revealed good positioning, with no impingement on tricuspid valve function as seen on TEE, and the device was purposefully implanted into the mid-RV septum under fluoroscopic guidance (.\nThe implant electrical measurements included sensing of 13.8 mV, impedance of 660 Ω, and capture threshold of 0.38 V at 0.24 ms. A tug test of the device was performed under high-resolution cinematography to confirm attachment of the Micra™ device, with all four tines secured to the endocardium and no loss of ventricular capture. The tether was cut and easily removed, leaving the Micra™ device implanted in the septum. The final settings were VVI at a low rate of 60 ppm (with hysteresis at 30 ppm). With this, the device was untethered and the Micra™ catheter was removed. The internal jugular vein defect was repaired with a running 5-0 Prolene suture (Johnston & Johnson, New Brunswick, NJ, USA) and the wound was irrigated with warm antibiotic solution. The incision was closed in multiple layers. Finally, the skin was closed with a reabsorbable 4-0 monocryl suture and Dermabond (Ethicon, Somerville, NJ, USA) was applied.\nThe following day, the patient underwent a repeat transthoracic echocardiogram to assess device placement, cardiac function, and valve function, which suggested appropriate positioning of the device as well as normal biventricular systolic function. The tricuspid valve was thoroughly imaged, revealing only trace to mild regurgitation, with normal excursion and coaptation of the valve leaflets. A chest X-ray was also obtained to further define positioning of the Micra™ TPS. The patient was discharged home after this testing was completed, which was the day after the procedure, without complications. He has been followed up with via regular outpatient visits and continues to do clinically well, with no apparent complications.
We present the case of a 63-year-old man who was admitted to our hospital with significant upper-body burns. The previous night he had been cooking when his clothing ignited. He did not call for help until the following morning.\nInitial assessment revealed approximately 60% total body surface area burn, most of which was full thickness involving his torso and upper extremities. In addition, there was some minor involvement of his lower extremities. The patient complained of pain and inability to pass urine. Limited history was obtained in the emergency department. The patient denied any allergies and was not taking any medications.\nIntubation for airway protection was performed early in the trauma room, and the patient was transferred to the burn unit where extensive fasciotomies to bilateral arms, hands, chest, and abdomen were performed at the bedside. Initial laboratory reports revealed acute renal failure with a creatinine level of 2.2, which marked the beginning of an undulating course. The next day, the patient was taken to the operating room for his first of several procedures. Fascial excision of both arms and left flank, with allograft, was performed. Skin biopsy was taken per protocol, with a view to obtaining cultured epidermal autograft.\nHis hospital course was complicated by pneumonia and polymicrobial sepsis. Organisms isolated from blood and wound-bed cultures included Pseudomonas, Aspergillus, vancomycin-resistant Enterococcus, and Klebsiella. Broad-spectrum antibiotic coverage was instituted.\nBy the sixth week of hospitalization, a decline in platelet function was noted from more than 150 × 10 to less than 15 × 10. This was paralleled by a generalized coagulopathy with a rise in international normalized ratio to more than 1.52. At this time, the patient had been taken to the operating room for fascial excision of the lower back with limited autografting. Although the procedure was uneventful, by the end of the operative procedure, bleeding was noted from the autograft donor sites. This was difficult to control, but sufficient hemostasis was achieved.\nUpon return to the burn unit, the patient became increasingly hemodynamically unstable, with serial hematocrit measurements revealing ongoing bleeding despite blood transfusions. After 12 units of packed red blood cells, 16 units of fresh frozen plasma, and 16 units of platelets, consideration was given to recombinant activated factor VII (rFVIIa). This was administered per pharmacy protocol, with subsequent stabilization of the patient and cessation of ongoing blood losses. A dose of 90 µg/kg was chosen on the basis of the use of the agent in other scenarios.\nWe present this case as a report of successful hemostasis in a patient with severe burn injury after the administration of rFVIIa.
A 65-year-old male marathon runner presented to a primary care physician with abrupt onset of posterior left knee pain that began two days before. There had been no injury associated with the pain. He denied systemic symptoms, including fever, chills, night sweats, or malaise. Past medical history included asymptomatic severe mitral valve regurgitation secondary to bileaflet mitral valve prolapse, for which he underwent robot-assisted minimally invasive mitral valve repair and prosthetic ring placement approximately 6 months prior to presentation. There was also a history of hypertension and hyperlipidemia. He lived in the central United States with his wife and was employed as an attorney. He consumed approximately two alcoholic drinks per night, was a never smoker, and had no history of intravenous drug use. There were no other significant exposures or travel documented.\nThe patient recovered from his mitral valve repair, had stopped anticoagulation after 6 weeks, and had returned to walking and/or running 3–5 miles per day. A transthoracic echocardiogram that was done approximately 2 months postoperatively demonstrated a normal functioning valve, with only trace regurgitation.\nHe was initially treated with symptomatic pain relief with hydrocodone-acetaminophen but had no improvement in the left popliteal fossa pain and was therefore referred to an orthopedic provider. A plain radiograph demonstrated Baker's cyst and he was treated with a cortisone injection. Due to persistence of symptoms, he was subsequently evaluated for possible deep venous thrombosis via venous ultrasound which indicated a left popliteal thrombus and aneurysm. As a result of this finding, he was admitted to an outside hospital for further evaluation.\nAt the outside facility, he underwent MRI of the left knee, CT angiogram with bilateral extremity runoff, and transthoracic echocardiogram. These studies revealed left popliteal aneurysm with occlusive eccentric thrombus and incomplete clot of a right renal artery branch. The following day, a transthoracic echocardiogram was done and showed a highly mobile mass attached to the anterior mitral valve leaflet, measuring approximately 1.5 cm.\nDue to the abnormal findings, he was transferred to our facility for further evaluation and management. On admission, the patient appeared well. Temperature was 36.8°C, blood pressure was 123/48, heart rate was 85 beats per minute, respiratory rate was 16 breaths per minute, and peripheral oxygen saturations were 98% on room air. A 1/6 holosystolic murmur heard best over the mitral valve was auscultated; the remainder of the physical examination was normal. There was no mention of onychomycosis. His laboratory evaluation demonstrated mild anemia (11.4 g/dL; reference 13.5–17.5 g/dL), elevated erythrocyte sedimentation rate (60 mm/1 hour; reference 0–22 mm/1 hour), and elevated C-reactive protein (75.9 mg/L; reference < 8.0 mg/L). The peripheral white blood cell count and differential, platelet count, INR, and prothrombin time were all normal.\nThe patient underwent transesophageal echocardiogram (TEE) which revealed two highly mobile masses attached to the mitral valve with the largest mass measuring approximately 1 cm by 3 cm. The patient underwent surgery the following day. Intraoperatively, the mitral valve had firm, white vegetations with destruction of both anterior and posterior leaflets. Samples were taken and sent immediately to pathology for review. The preliminary frozen pathology demonstrated fungal elements (Figures and ). He underwent aggressive debridement and mitral valve replacement with a porcine valve. The patient was started on intravenous liposomal amphotericin B 5 mg/kg/day intraoperatively and caspofungin 50 mg daily was added the next day for empiric treatment while waiting identification of the organism. Final pathology described active native valve endocarditis with fungal organisms involving the myxomatous mitral valve. The mitral valve tissue was cultured on inhibitory mold agar and ultimately grew a Scopulariopsis species with no other microbial growth, including negative bacterial and mycobacterial cultures. Fungal blood cultures were obtained but were negative for growth. Due to the severity of the disease and highly resistant nature of the organism, oral voriconazole dosed at 300 mg twice daily was also added to the antifungal regimen.\nThe day following mitral valve debridement and replacement, vascular medicine and vascular surgery were consulted regarding the suspected mycotic aneurysm and thrombus of the left popliteal artery. CT angiogram with lower extremity runoff was repeated and showed the left popliteal artery mycotic aneurysm and thrombus, as well as bilateral renal infarcts. Ten days after mitral valve surgery, he underwent resection of the popliteal artery and peroneal artery bypass. The pathologic examination revealed GMS-positive staining hyphae, and inhibitory mold agar cultures again grew a pure culture of Scopulariopsis species.\nThe Scopulariopsis species from the cultures was ultimately identified by combined phenotypic characterization and DNA sequencing as Scopulariopsis brevicaulis. Phenotypically, the organism was a tan powdery colony and microscopically demonstrated branching annellides with chaining, round conidia with a truncated base consistent with Scopulariopsis brevicaulis. From a sequencing standpoint, D2 ribosomal sequencing was utilized with the top 3 matches being a greater than 95% match (100%, 99.31%, and 95.45%) to Scopulariopsis species in the Mayo Fungal Library. Antifungal susceptibilities were completed utilizing broth dilution as per recommended guidelines from the Clinical and Laboratory Standards Institute and were consistent with those reported in the literature: amphotericin 2 µg/mL, caspofungin 1 µg/mL, micafungin 0.125 µg/mL, anidulafungin 4 µg/mL, posaconazole 2 µg/mL, voriconazole 8 µg/mL, itraconazole 2 µg/mL, and terbinafine 0.5 µg/mL [].\nLiposomal amphotericin was discontinued after 18 days due to acute renal injury, and caspofungin and oral voriconazole therapy were continued. He completed 8 weeks of combination therapy and was continued on oral voriconazole for chronic suppression. Voriconazole was chosen despite susceptibility data given reported clinical efficacy combined with lack of correlation between the susceptibility data and patient outcomes []. He unfortunately developed peripheral neuropathy, suspected by his local neurologist to be secondary to the voriconazole, and he was transitioned to delayed-release oral posaconazole dosed at 100 mg daily for chronic suppression [, ]. Posaconazole levels were followed and remained therapeutic with levels >1.0 mcg/mL. The inflammatory markers normalized at 6 months. Follow-up TEEs at 3 months and 7 months postoperatively did not show recurrence of endocarditis. Further, after cessation of the voriconazole he reported improvement, but not full resolution, of his neuropathy symptoms.
A 44-year-old man was assessed at our institution after emergency referral by his general practitioner with a one week history of left-sided abdominal and flank pain with pain spreading to the left thigh. There was a history of a fever-like illness and some diarrhoea. He was apyrexial at initial presentation however a fever developed later. His past history consisted only of mild asthma managed with metered dose steroid and bronchodilator inhalers and allergic rhinitis. There was tenderness on palpation in the left iliac fossa and left flank and straight leg raise on the left intensified the pain. Initial investigations showed a raised white count and a raised CRP of more than 100 mg/L but normal renal and hepatic function. He underwent chest and abdominal radiography which showed loss of the left psoas shadow but normal abdominal gas pattern and no pneumoperitoneum.\nAn initial diagnosis of acute diverticulitis with an associated inflammation or abscess within the left psoas was made. Intravenous access was established; intravenous fluids, analgesia and intravenous Co-Amoxiclav 1.2 g tds, and Gentamicin 5 mg/kg OD were administered.\nThe day after admission his pain had worsened particularly in the left thigh and increased weakness was noted in the left thigh. At this point a CT of the abdomen and pelvis was performed. This showed diffuse inflammation affecting the peritoneum of the left side of the abdomen, the pelvis, and the left psoas and retroperitoneum, but no collection was seen (). The following day the lower limb neurological symptoms worsened with numbness affecting the L1/L2 distribution, quadriceps weakness on the left, and similar weakness on the right side. An MRI of the thoracolumbar and sacral spine was performed and an opinion sought from the neurology service. The MRI did not reveal any significant abnormality of the spine or spinal nerve roots and confirmed the presence of inflammation affecting the left psoas, left sided retroperitoneum, and associated left sided abdominal and pelvic peritoneum. Repeat haematological investigations at this stage revealed an eosinophilia that peaked at 4.52 × 109/L (Normal range 0–0.5 × 109/L). The diffuse inflammation raised the possibility of inflammatory bowel disease and a colonoscopy was performed at this time; however, the colon was both macroscopically normal and random biopsies showed it to be microscopically normal.\nAt this stage ANA, p-ANCA, and c-ANCA, cryoglobulins, complement studies, hepatitis screen, and serum protein electrophoresis were all normal.\nOver the following 24 hours his abdominal symptoms and signs dramatically worsened and he became peritonitic; concern about the possibility of mesenteric ischaemia was raised and based on the clinical findings we proceeded to laparotomy. 4 litres of ascites were immediately identified at this point, which was thought to have arisen from a diffuse inflammatory peritoneal response and associated hypoalbuminaemia producing an ascitic transudate. The ascites were drained and a sample sent for cytological examination. Further examination of the abdominal cavity revealed diffuse inflammatory infiltrate particularly involving the appendix, small bowel, peritoneum, and omentum. An appendicectomy was performed along with sampling of the small bowel, peritoneum, and greater omentum.\nHe recovered slowly, but there was continued persistence of his neurological symptoms and pain. Cytology of the ascitic fluid showed numerous eosinophils, with histiocytes and reactive mesothelial cells whilst histology from the surgical samples which became available three days later showed tissue with diffuse serosal coverage by an extensive eosinophilic exudate which extended into the subserosa of the excised appendix with an associated subserosal focal eosinophilic vasculitis. H&E histological staining of tissue containing vessels with eosinophilic infiltrate and distortion is shown in . Within the omental tissue in addition to the eosinophilic infiltrate, occasional multinuclear giant cell were seen. No mycobacteria, parasites, or other infective organisms were seen.\nThese findings were strongly suggestive of Churg-Strauss and a specialist opinion was sought from the Rheumatology service at our institution. Pulsed intravenous methyl prednisolone 1 g tds was started and a plan made, once a full recovery from the surgery had occurred, to convert to oral prednisolone 50 mg per day (reducing dose regime) and cyclophosphamide 750 mg iv together with prophylactic Mesna 400 mg before and after the cyclophosphamide. This was initially planned at 2 weekly intervals and then monthly according to response with regular monitoring of the platelet count.\nHis recovery was unfortunately delayed by wound infection and then a wound dehiscence requiring resuturing under general anaesthesia. Therefore, although the steroid therapy was initiated immediately cyclophosphamide was delayed until 4 weeks after the abdominal resuturing. His quadriceps weakness slowly improved and a minor improvement in sensation in the L1 and L2 distributions were identified. He transferred to a rehabilitation facility 37 days after admission and 34 days after his laparotomy and was discharged home after 48 days. A reducing dose regime of oral prednisolone was prescribed on discharge, starting at 50 mg/day and reducing by 5 mg per week and plans for a reducing frequency cyclophosphamide infusion made. On review at 6 weeks his surgical wounds had healed, strength in his quadriceps had increased, and sensation in the L1 and L2 dermatomes had almost returned to normal.
A 12 years and 8 months old female presented with a double structural thoracic and lumbar scoliosis associated with Arnold-Chiari 1 malformation and an extensive cervicothoracic syringomyelia. At age 11 years and 11 months she developed acute weakness affecting both hands and feet with associated pins and needles down the arms and legs, as well as altered sensation affecting the right upper limb below the elbow while her bladder and bowel function was normal. The patient underwent an urgent cranio-cervical decompression including a posterior fossa craniotomy and C5/C6 laminectomy with syringostomy and placement of a syringopleural shunt to drain the syrinx []. She recovered well following surgery and there were no residual neurological signs or symptoms at the time of presentation in our clinic.\nOn clinical examination at age 12 years and 8 months, she had a progressive thoracic and lumbar scoliosis extending from T5 to T10 measuring 60° and T10 to L3 measuring 50° respectively. Neurological examination was normal. The patient had normal function including sport activities. She was skeletally immature and pre-menarche with Risser grade 1. An MRI of the whole spine demonstrated adequate decompression of the cranio-cervical junction and a small residual cervical syrinx which was considered to require no further treatment at the time by our neurosurgical colleagues.\nThe patient underwent posterior spinal arthrodesis extending from T4 to L3 at age 12 years and 10 months with the use of segmental pedicle screw and rod instrumentation and autologous iliac crest bone graft []. Spinal cord monitoring was performed during the procedure recording motor and sensory (cortical and cervical) evoked potentials which remained stable throughout. Postoperative neurological examination was normal. The patient had 3 doses of intravenous (IV) cefuroxime; one at induction to anaesthesia, and 2 after surgery at 8-hourly intervals.\nIn the postoperative period, the patient developed prolonged abdominal distension without pain which was considered to be due to paralytic ileus as the result of the major spinal operation, as well as the effect of narcotic analgesic agents delivered during and after surgery. There was no nausea or vomiting and the bowel sounds were preserved but reduced. She had no electrolyte abnormalities and her blood tests were normal other than an expected small reduction in haemoglobin. The spinal wound healed uneventfully and the patient mobilized out of bed on postoperative day 2.\nAt the end of the first week following surgery the abdominal distension began to increase and she started having generalized abdominal pain with associated tenderness in the right iliac fossa. She had no vomiting and no bowel movements. Repeat blood tests showed marginal leukocytosis coinciding with the onset of abdominal pain while the remaining parameters including electrolytes, kidney and liver function tests were within normal limits. She was initially managed conservatively maintaining fluid and electrolyte balance and placing a nasogastric tube for aspiration of gastric contents. Repeat ultrasounds and a CT scan of the abdomen did not demonstrate evidence of mechanical obstruction, congenital anomaly (such as a fibrous band or a malrotated bowel), or cecal/sigmoid volvulus []. During the whole postoperative period our patient was under regular review by our general surgical colleagues.\nDue to aggravation of the patient's abdominal distension and absence of clear signs of peritonitis pain a laparoscopy was decided on postoperative day 16 followed by an exploratory laparotomy. At that stage no definitive diagnosis was made. The laparotomy showed the cecum and ascending colon to be grossly inflamed with multiple ragged perforations, no dilatation and gross contamination of the abdomen. Multiple loops of small bowel were adherent to the anterior abdominal wall in the right iliac fossa. The remainder of the transverse, descending and sigmoid colon was found to be entirely normal. A limited right hemi-colectomy and ileocecal anastomosis was performed and the patient returned to the intensive care with nasogastric aspirations. She remained intubated for 7 days and was treated with meropenem and vancomycin for 2 weeks as the peritoneal cultures showed a mixed growth of Enterococcus and E. coli. This was changed to ciprofloxacin for an additional period of 3 weeks. She made gradual recovery with good bowel function, returned to feedings over a period of 2 weeks and was discharged on postoperative day 32.\nThe surgical specimen sent for histopathology included 3 cm of terminal ileum, 5.5 cm appendix and 12 cm of colon. A single large defect was identified at the cecum and multiple defects in the ascending colon above the ileocecal valve. The microscopic examination of the small bowel and appendix were normal. Sections from the colon and cecum showed ulcerations and perforation with organized fibrinous peritonitis. There were no features of Crohn's disease, ulcerative colitis or vascular abnormality. The surgical findings combined with the clinical picture and the pathology report confirmed the diagnosis of Ogilvie's syndrome.\nAt latest followup, 3.2 years after scoliosis surgery our patient was skeletally mature (Risser 5), had no abdominal complaints and was on a normal diet. Spinal radiographs showed maintenance of an excellent correction of her scoliosis and no signs of infection, instrumentation failure or nonunion. She had normal activities including sports and no complaints of her back.
A 66-year-old woman with severe AD complicated by oral dyskinesia was referred for dental treatment by her neurologist. She was unable to eat any solid food because of limited mobility of her maxillary teeth as a result of severe periodontitis. Because of her oral condition and subsequent lack of food, her body weight had decreased by ~5 kg within 2 months. Her husband was concerned for her health and hoped that her oral function would improve with the extraction of her diseased teeth and replacement with a removable denture. Written informed consent was obtained from the patient's husband for publication of this case report following the Ethical Guidelines of Kyushu University Hospital.\nHer medical history revealed that she developed severe AD in 2004. Her medical condition suddenly worsened in 2006, resulting in severe cognitive impairment. Since 2011, she has been medicated with NMDA inhibitors and cholinesterase inhibitors. She has continued to receive regular medical treatment.\nHer dental history revealed that she routinely visited a local dental office for oral care and maintenance until July 2012. Her family dentist recommended extraction of all her remaining maxillary teeth and asked her husband to consent to extractions under IVS in hospital because of the difficulty of conducting the dental procedure while she was awake. The patient's neurologist wrote a referral for her, and her husband brought her to our department, the Special Patient Oral Care Unit, Kyushu University Hospital, in October 2012. When she presented at our department, she exhibited typical features of AD, such as aphasia, gait apraxia, and disorientation. Her height and weight were 151 cm and 46 kg, respectively. Her serum albumin was 3.8 g/dL. She could not walk by herself and needed her husband's assistance. Her cognitive function and activities of daily living using the functional independence measure [] were 26 points (maximum possible score: 126, minimum possible score: 18). She frequently vocalized meaningless words and could not speak any meaningful words or understand any of our instructions. As she could not sit still, it was impossible to take X-ray images of her teeth. We were able to see and touch inside her mouth only when her husband held her face and opened her mouth gently in semi-Fowler's position. If we tried to insert any instrument into her mouth by ourselves, she brushed our hands away. With her husband's help, we were able to briefly assess her oral condition and established the necessity for multiple extractions of her remaining maxillary teeth and fabrication of a complete denture. We initially planned to take impression before extraction to maintain her original maxilla-mandibular horizontal relationship under IVS. However, this was not possible as the horizontal position had collapsed from severe lateral mobility of the metal bridge between 13 and 17. To avoid accidents and the need for restraint and to maintain the patient's dignity during dental treatment, we decided to use IVS for oral examination and dental management, with the approval of her husband. In addition, her medical doctor checked her general health and approved the use of IVS.\nOn the first day of her dental treatment, we planned to undertake a complete examination, tooth scaling, multiple tooth extractions, and an impression for her denture under IVS. The IVS procedure involved initial administration of 4 mg midazolam, and once the patient was drowsy, we added propofol for maintenance. Under IVS, we first took X-rays and checked the probing depth and mobility of the remaining teeth. We observed severe periodontitis of all maxillary teeth (12, 13, 15, 17, 21, 22, and 23) and moderate periodontitis of all mandibular teeth (). We scaled the teeth and subsequently extracted the maxillary teeth. All sockets were sutured using bioabsorbable thread. Using alginate in a custom impression tray, we took impressions of the maxillary arch and the opposing mandibular teeth for a complete maxillary denture. We finished the dental treatment after confirming that there was no bleeding from the sockets. The duration of the dental treatment was 57 min, and the duration of the anesthesia was 1 h and 20 min. In total, 49 mg of propofol was used. There were no perioperative complications.\nTo help stop bleeding and as an imitation trial of the complete denture, we made a temporary base plate that covered her maxilla with tray resin and a plaster model, which duplicated for her teeth impression for the denture until recovery from IVS. After recovery from IVS, we tried fitting the base plate and relined with tissue conditioner. Surprisingly, she easily accepted it. Following this success, we sought to fabricate a new complete denture, as requested by her husband.\nOne day after extraction, there was no bleeding inside the patient's mouth, and she was still wearing the base plate without any signs of refusal. We removed the temporary base plate, checked to ensure that there was indeed no bleeding, washed her sockets with saline, and then refitted the base plate to her maxilla. We provided her husband with instructions for care, which included removing and washing the base plate after every meal and before the patient went to sleep. This was done as training before use of the complete denture.\nOne week after the extractions, we attempted to take a bite using Willis's method in semi-Fowler's position, opening the patient's mouth with her husband's help. Maxilla-mandibular horizontal position was achieved by moving the patient's chin forward using our hands. After a trial fitting, we fabricated her initial denture. To avoid instability caused by lateral cuspal interference because of her oral dyskinesia, we used zero-degree artificial teeth (). On inserting the new complete maxillary denture, we found that the mucosal surface of the denture was ill-fitting, because the impression had been taken immediately after multiple tooth extractions. To improve the fit, we relined the denture using low-flow tissue conditioner, COE-COMFORT™ (GC, Tokyo, Japan) and held her jaw gently for a while in semi-Fowler's position because of her oral dyskinesia. When we tried fitting the new denture, she initially hesitated to wear it; however, she accepted it soon after it was relined, similar as to when she wore the base plate to stop bleeding. After occlusal adjustment of the denture using articulating paper to remove lateral cuspal interference when she was awake, we instructed her husband on how to manage the denture, including insertion, removal, and cleaning, and the patient went home wearing the denture.\nOne week later, she visited our department for a denture adjustment. Her husband told us that she had not complained or indicated that the denture caused any pain or discomfort and had not tried to remove the denture once inserted. Her husband reported that she was able to eat soft solid food such as grilled fish, and her appetite had increased. She visited our unit 2 days per week for a denture adjustment and relining over several weeks. Three months after the extractions, the sockets were almost completely healed and bone had recovered. We performed dynamic impressions for a week using the same tissue conditioner and temporarily kept her denture for relining, choosing an indirect procedure in the dental laboratory to avoid a poor result because of her oral dyskinesia and to avoid accidental swallowing of the reline materials. After relining and occlusal adjustment of the denture, she was again happy to wear the denture ().\nThe patient has since visited the department for oral hygiene management and denture adjustment, including partial direct rebasing around the sockets several times. Currently, she routinely receives oral examinations and hygiene management. She can eat almost all foods such as vegetables, meat, and fish, and her body weight has increased by 5 kg (now up to 51 kg) and her serum albumin level has also improved (4.3 mg/dL). From discussion with the patient's husband's and examining a visual analogue scale drawn by him, it is apparent that her appetite and food intake have greatly improved ().
A 69-year-old previously healthy male patient presented with a history of vomiting for five days' duration. The vomitus contained clear fluid initially, which later became brownish in colour. He had developed absolute constipation for three days. He had noticed gradual abdominal distension during this period. He had not undergone any abdominal surgeries in the past.\nOn examination, his abdomen was distended and there was mild tenderness in the lower abdomen. The examination of the respiratory system did not reveal any abnormalities. Biochemical investigations were unremarkable. With the clinical diagnosis of intestinal obstruction, he was referred for imaging studies.\nPlain X-ray abdomen was performed in the supine position and it revealed grossly distended small bowel loops with absent rectal gas (). There was no pneumoperitoneum.\nUltrasound scan of the abdomen revealed fluid filled aperistaltic bowel loops. Central abdomen was distended and presence of bowel gas hindered evaluating the deeper structures. Pancreas appeared normal. There was no free fluid in the abdomen. Liver, spleen, and both kidneys were normal on ultrasound. The superior mesenteric artery appeared normal at the origin and showed normal colour and spectral flow pattern. The portal vein was normal.\nCT abdomen showed grossly dilated jejunal and ileal loops. The appendix was identified separately. There were no fat strandings or fluid collections around the appendix. Distal ileal loops appeared collapsed. There was a soft tissue density band like structure extending from the collapsed ileal loops anteriorly ().\nHowever, its insertion site was not identified. There was no CT evidence of appendicitis. The cause for the small bowel obstruction was not identified on CT. However, as the patient had no previous surgeries, the possibility of a congenital band was highly suspected. The presence of a soft tissue density linear band led to the preoperative suspicion of a congenital band. Furthermore, Meckel's diverticulum was not considered on CT images.\nThere was a tubular retroperitoneal structure in the left side of the aorta which was extending up to the left renal vein. It was in continuity with the left external iliac vein and was identified as the left IVC. Right IVC was also noted. Left IVC was smaller in caliber than the right IVC ().\nLeft internal iliac vein was seen crossing the midline to drain into the right common iliac vein ().
Our patient is a 19-year-old girl, with a long surgical history of multiple complicated abdominal surgeries. She was diagnosed with Primary Hyperoxalosis for which she underwent same setting liver and kidney transplant. A few years afterward, she was diagnosed with intestinal type diffuse large B-cell lymphoma. After that, she had an ileocecal mass that obstructs the bowel lumen and required oncological resection with right hemicolectomy and anastomosis which was further complicated by anastomotic leakage, wound dehiscence and intra-abdominal sepsis thus requiring emergency exploratory laparotomy and Hartmann's procedure with end ileostomy. The patient then underwent laparotomy, Hartmann's revision and ileostomy site closure. For the purpose of graft preservation, the patient was on long-term immunosuppressants.\nAfter more than a year from the last surgery, she developed a central abdominal bulge at the site of her laparotomy scar; she had midline incisional hernia. Due to the long history of laparotomies and multiple surgical procedures, a multidisciplinary approach was necessary, and a surgical treatment plan was set.\nAn enhanced CT scan was performed and showed thinning of the anterior abdominal wall muscles with severe atrophy of the left rectus abdominis muscle with rectus diastasis of around 10 cm distance (Figure ).\nThe injection was performed by the interventional radiologist with the patient in supine position under ultrasound guidance (Figure ). Injection sites were marked at the anterior axillary line between the costal margin and anterior superior iliac spine according to the technique described by Smoot et al. The area was prepped and draped in a sterile technique which was followed by application of local anesthesia in the form of 1% lidocaine at the skin of injection sites. Under ultrasound guidance, BTA was injected at the three sites on either side of the abdomen. The patient received a total of 300 units of BTA diluted in 150 mL of 0.9% saline with a concentration of 2 units/mL. Each of the six injection sites received a volume of 25 mL. Each of the three injection sites on either side of abdomen were used to target the external oblique, internal oblique, and transversus abdominis muscles. After the procedure, the patient recovered smoothly and was discharged home the next day to return for surgery after 3 weeks.\nAbove the iliac crest, transversus abdominis at anterior axillary line. Mid abdomen, internal oblique at mid axillary line. Below costal margin, external oblique at anterior axillary line.\nPatient went for surgery 3 weeks after Botox injections. The procedure started by infiltrating normal saline for subcutaneous hydro-dissection followed by removing the old scar at the midline. De-epithelization was continued just beneath the skin to raise it above the adherent bowel underneath until reaching the normal fascia on both sides of the abdomen. Then, the abdominal flap was raised in the subscarpal plane above the fascia. Closure of the defect and plication of the recti were done followed by placement of sized on-lay fully resorbable monofilament mesh (Figure ).\nTo further relax the lateral abdominal muscles, a total of 200 units of BTA diluted in 8 mL of normal saline were infiltrated at the same previously injected sites (Figure ). Drains were placed bilaterally above the mesh. Subcutaneous tissue was approximated with interrupted sutures in two layers. Skin was closed with 4-0 Monocryl in subcuticular fashion (Figure ). Patient had no complications and was followed up for the next 18 months with no recurrence.
We present the case of a 47-year-old woman, with no underlying clinical conditions, who presented to the emergency department with a 2-day history of severe right upper quadrant abdominal pain radiating to the right shoulder. She described the pain as sharp and constant. Her pain was associated with recurrent episodes of vomiting. The patient reported taking paracetamol and an herbal product, which did not result in any improvement. She could not identify the ingredients of the herbal preparation, but she reported using it regularly for its analgesic properties. She does not drink alcohol and has never smoked. There is no history of liver diseases in the family.\nOn examination, her vital signs were within normal limits and the abdominal examination revealed a soft and lax abdomen with mild tenderness in the right upper quadrant. Two months prior to presentation, the patient experienced mild respiratory tract symptoms and she tested positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). She had negative results on two consecutive reverse transcription-polymerase chain reaction (RT-PCR) tests after 2 weeks of home isolation considering her mild symptoms with no derangements in laboratory parameters.\nAbdominal ultrasound demonstrated the liver with smooth capsular contour and normal parenchymal echogenicity. It had a normal hepatopetal blood flow in the portal vein with no evidence of biliary duct obstruction (Fig. ). Laboratory findings revealed markedly elevated hepatic enzymes and deranged coagulopathy profile. However, the viral serology, autoimmune, toxicology profiles were negative (Table ).\nIn light of the aforementioned clinical and laboratory findings, the patient was admitted to the intensive care unit for monitoring and continued supportive care. As per local institutional policy, the patient underwent RT-PCR for SARS-CoV-2, which revealed a positive result. However, the chest radiograph findings were normal. The patient developed rapid deterioration in the neurological status with a remarkable increase in the liver enzyme levels (Fig. ). The need for emergency liver transplantation was discussed. However, despite aggressive resuscitation, the patient suffered irreversible cardiac arrest and died. An autopsy examination was not performed.
A 23-year-old woman (gravida 3 para 2) with no significant medical history or family history was referred to our hospital at 30 weeks of gestation because of nausea, vomiting and general fatigue. These symptoms had first appeared at 28 weeks of gestation, and gait imbalance and difficulty with speech also occurred a few days before referral. On examination, her Glasgow Coma Scale score was E3V5M6, and she exhibited a tremor in her left upper limb. She had experienced repeated nausea and vomiting, which had led to 3 kg of body weight loss within one month. Her vital signs were normal, and laboratory data showed no significant changes. Because the symptoms had lasted for two weeks and were accompanied by a change in her level of consciousness, a neurological disorder was suspected. A brain MRI was performed to search for an intracranial lesion, and a mass was found on the left cerebellopontine angle nearly obstructing the fourth ventricle (A,B). Although the patient was prescribed steroids and osmotic diuretics, her symptoms caused by increased intracranial pressure remained unchanged. Obstetricians and neurosurgeons discussed her condition, which was considered to have a potential risk of obstructive hydrocephalus, and decided that the patient’s pregnancy should be terminated to proceed with treatment of the brain tumor. Cesarean section under general anesthesia was performed at 32 weeks of gestation, and the patient delivered a 1532-g female infant with an Apgar score of 4/7. One week after delivery, total removal of the tumor was conducted. A lateral suboccipital craniotomy was performed, with the dura incised along the sigmoid sinus. The tumor was detected after opening the lateral cerebellomedullary cistern. The floccular part of the fourth ventricle was assumed to be the tumor’s origin as the tumor adhered to that part. Total resection of the tumor was accomplished, which was confirmed by the postoperative brain MRI (C). She was discharged from the hospital 17 days after the operation with no complications. Pathologic examination revealed a papillary structure covered with a single layer of columnar epithelial cells along a narrow interstitium with abundant vessels, which concluded the pathological diagnosis was choroid plexus papilloma (D). No recurrence of the tumor had been seen for 18 months after the operation.
A 48-year-old man with a history of alcohol abuse presented to the gastroenterology department for the first time in November 2006. He was suffering from severe epigastric pain and nausea for two days. Because the laboratory findings were suspicious for pancreatitis, the patient underwent computed tomography (CT) (Fig. ). The examination showed an edematous pancreas tail and fluid in the anterior prerenal space and in the anterior, posterior and lateroconal fascia. There were no signs of chronic pancreatitis or vascular complications. In the following months the patient had two similar episodes of acute tail pancreatitis.\nIn September 2009 he presented with vague epigastric pain of several weeks duration. Serum amylase and lipase levels were normal, however the cholestatic parameters were mildly elevated. Therefore, a magnetic resonance (MR) study was performed. The examination demonstrated moderate chronic pancreatitis of the tail with atrophy and irregular bording of the Wirsung’s duct. There were no other abnormalities. Further examinations also revealed a Helicobacter pylori gastritis.\nIn February 2012 the patient presented again to the gastroenterologist with epigastric and left upper quadrant pain. Laboratory findings were not suspect for acute pancreatitis. However, contrast-enhanced CT scan was performed in order to evaluate the known chronic pancreatitis and other causes of pain (Fig. ). This study surprisingly revealed a hypervascular lesion in the pancreatic tail consisting of a conglomeration of small hypervascular spots and blood vessels. Further examinations were performed in order to differentiate a vascular malformation from a hypervascular pancreatic tumor, especially an islet cell tumor or a hypervascular metastasis. Contrast-enhanced MR study showed a focal area of heterogeneous contrast enhancement without an apparent nodular component. The subsequent angiography showed two large feeding arteries in the early arterial phase, followed by a racemose vascular network, an early transient dense parenchyma stain in the early portal phase and early wash-out of the lesion in the portal phase (Fig. ). Based on these findings, a pancreatic arteriovenous malformation was suggested. The patient was treated with a resection of the pancreas tail and postoperative recovery was uneventful. Histopathologic examination revealed sequelae of chronic pancreatitis and numerous dilated blood vessels accompanied by blood clot formation and intimal hyperplasia (Fig. ). The histopathological findings were consisting with a pancreatic AVM.
A 56-year-old man was referred to our institution for a case of facial palsy. He was treated with systemic methylprednisolone for 10 days, without improvement. On examination, he had a visual acuity of 1.0 in the right eye and hand motion in the left. Motility examination revealed limitation of abduction of the right eye. The patient had a frozen orbit with a limitation of all gazes in the left eye. Fundus examination revealed a pale optic disk, afferent pupillary defect, and an ischemic retina, indicating central retinal artery occlusion. Examination findings were also consistent with oculomotor, trochlear, and abducens nerve paresis. Mucormycosis was a result of steroid therapy, as the patient was diagnosed 2 weeks previously with an isolated cranial nerve VII and was started with steroids in a peripheral ophthalmology clinic. At his first presentation in Jordan University Hospital, he had a frozen orbit with cranial nerve III, IV, and VI palsies.\nThe patient was admitted, and the results of baseline investigations were normal, except for a high white blood cell count and hemoglobin A1C (HbA1c) of 10%. Systemic steroids were stopped, and the patient was started on insulin to correct his blood sugar. He was never diagnosed with diabetes mellitus before and denied any symptoms of diabetes mellitus. He was initially diagnosed with diabetes mellitus. He tested negative for ketone bodies and was treated in the ward. Results of other routine investigations, such as kidney function tests and complete blood count, were normal, except for high blood sugar and a high white blood cell count.\nThe differential diagnosis of his current symptoms was cavernous sinus thrombosis, a space-occupying lesion in the cavernous sinus or orbital cellulitis.\nComputed tomography of the brain and orbit was performed and revealed extensive sinus disease with left complicated ethmoidal and maxillary sinusitis extending into the left orbit with abscess formation. There was also extensive orbital cellulitis and evidence of left cavernous sinus thrombosis secondary to infection ().\nIt is possible that the patient had undiagnosed diabetes mellitus as indicated by a high HbA1c and was worsened by steroid therapy\nThe patient was started on systemic vancomycin and ceftriaxone, and a swab from the nasal cavity was sent for evaluation. Nasal swab revealed methicillin-resistant staphylococcus and positive fungal staining. The patient was started on systemic amphotericin B at a loading dose and continued with the therapeutic dose after 2 days of his presentation to our institution. Nasal endoscopy revealed diffuse white filaments and necrosis of the bone from which a biopsy was taken. Sinuses were drained, followed by ethmoidectomy and maxillectomy. A biopsy from the necrotic tissue was sent, which showed the presence of non-septate hyphae branching at right angles consistent with angio-invasive mucormycosis. However, workup to identify the exact causative organism of mucormycosis was not performed as it did not affect further management.\nThe patient was kept on daily irrigation of the sinuses along with administration of systemic amphotericin B. On follow-up magnetic resonance imaging, the patient had an intraorbital collection (). Subsequently, drainage of the orbital abscess, tarsorrhaphy, and excision of the necrotic conjunctiva were performed. The patient was kept on systemic amphotericin B and showed clinical improvement. He remained hospitalized until a repeat biopsy of the paranasal sinuses was negative for the fungi. He was discharged with residual paresis of all cranial nerves involved along with left eye ptosis and only hand motion in the left eye. He was followed up serially for 1 year and 4 months. Currently, the patient is afebrile with complete resolution of symptoms in the right eye and partial recovery of cranial nerve palsies. However, in the left eye, residual ptosis has remained and only hand motion is visible due to the previous central retinal artery occlusion related to his initial presentation.
An 18-year-old male suffered a valgus injury to the right knee due to a fall during a bigfoot race he took part in when he was 15 years old. He visited a different hospital because of right knee pain and effusion. However, no obvious ligament injury or fracture was noted on MRI and physical examination, and the patient was followed up at the hospital. At the age of 17, the patient gradually became aware of the valgus deformity of the right knee. At the age of 18, the valgus deformity had further progressed, and the patient visited our department due to continuous right knee pain. There were no comorbidities in particular and no past medical history of note. At the time of his initial visit, the patient was the manager of a high school baseball club. A physical examination revealed that the valgus deformity of the right knee was visibly obvious (). Joint effusion of the right knee and tenderness on the lateral condyle of the femur were noted. The range of motion was 0 (extension) to 140 (flexion) degrees denoting a slight flexion restriction. Lower limb length discrepancy (spinomalleolus distance (SMD)) was −3.0 cm in the right lower limb. X-ray images at the time of the initial visit to our department showed severe valgus deformity with a femoro-tibial angle (FTA) of 160 degrees and % mechanical axis (%MA) of 100% from the medial edge of the tibial plateau (). Valgus deformity of the right knee was pronounced with a mechanical lateral distal femoral angle (mLDFA) of 71 degrees in contrast to that of left mLDFA which was 87 degrees, whereas the medial proximal tibial angle (MPTA) was normal at 85 degrees (left: 85 degrees), suggesting that the femur side was at the center of valgus deformity. X-ray images taken at the time of injury (at age 15) showed no obvious fractures or growth plate fractures (). MRI at the time of injury (T2-enhanced) showed mild bone marrow edema in the lateral condyle of the femur and a high signal area suggestive of hemorrhage under the muscular layer (). X-ray images taken during the four years after the injury showed that the valgus deformity was gradually progressing each year (). A bony bridge on the lateral side of the growth plate was noted on MRI taken at the age of 16 (). Similar to the X-ray images, MRI performed at the age of 17 also showed a bony bridge on the lateral side of the growth plate. Based on these findings, the patient was diagnosed with a valgus deformity of the knee due to traumatic premature closure of the femoral growth plate. We decided to perform a biplane-cut medial closing wedge distal femoral varus osteotomy (DFO) using a locking plate and the central area of the deformity was the distal femur []. In preoperative planning, we planned to improve the mLDFA to 87 degrees and FTA to 178 degrees with a 16-degree correction (10 mm closing wedge) ().\nDuring preoperative arthroscopy, although the anterior cruciate ligament and the lateral compartment were normal, International Cartilage Research Society (ICRS) grade II to III cartilage damage was noted in the patellofemoral joint. The minimally invasive plate osteosynthesis (MIPO) technique was used for DFO. A 4–5 cm longitudinal incision was made at the lateral side of the femur just above the femoral epicondyle according to the biplanar technique. The TomoFix medial distal femur (MDF) anatomical plate (DePuy Synthes, Solothurn, Switzerland) was bent according to the individual's anatomy and positioned under the vastus medialis muscle for osteotomy fixation []. Postoperative X-ray images showed that the operation went almost as planned with an improvement of 86 degrees in mLDFA and 177 degrees in FTA. Regarding postoperative therapy, range of motion exercise was started one day after surgery, partial weight-bearing exercise was permitted three weeks postoperatively and full weight-bearing exercise four/five weeks postoperatively, and the patient started jogging four months postoperatively.\nAt six months follow-up, bony fusion was achived and %MA was 48.5% from the medial edge of the tibial plateau. Bony fusion is achieved and %MA is 48.5% from the medial edge of the tibial plateau. Valgus deformity has improved in appearance, whereas lower limb length discrepancy (SMD) improved to -0.5 cm in the left lower limb and the range of motion of the right knee also improved to 0 (extension) to 150 (flexion) degrees (). At the latest follow-up visit at 2 years after surgery, with respect to clinical scores, both the Knee injury and Osteoarthritis Outcome Score (KOOS) and International Knee Documentation Committee (IKDC) subjective score improved from 318 (before surgery) to 447 and 52 (before surgery) to 86, respectively. The KOOS showed that there was a significant improvement in symptoms, pain, sports, and quality of life.
A 63-year-old female patient was admitted to our hospital with intermittent epigastric abdominal pain for the past three months. Results of the physical examination on admission indicated no icteric sclera. The abdomen was soft with no palpable abdominal mass. The patient had experienced a weight loss of about five kg in the past two months and had no history of hepatitis B or C. She had been exposed to dogs and sheep and denied any history of exposure to infected cases from the epidemic area. After admission, CA19-9 was measured and was 796.20 U/mL. No serological examination for echinococcosis was performed because of the limited conditions of our hospital. Test results from pelvic ultrasound indicated menopausal uterus and uterine fibroids. Test results from gastroscopy showed chronic non atrophic gastritis. Colonoscopy revealed multiple polyps in the large intestine (basically removed); intestinal histopathology (cecum, biopsy) showed severe chronic inflammation of the mucosa and adenomatous hyperplasia of the individual glands. Contrast-enhanced computed tomography of the upper abdomen (Fig. A) revealed a more homogeneous thickening of the gastric wall in the antrum. Round unenhanced low-density foci with a diameter of 4.6 cm was seen in the S7 segment of the liver. Nodular calcifications were also observed. No significant dilatation was noted in the intrahepatic and extrahepatic bile ducts. The size and shape of the gallbladder were normal, the wall was not thick, and no significant abnormal density was observed in the cavity. The pancreas, spleen, and adrenal glands showed no significant abnormalities. Test results from computed tomography indicated liver cyst and intrahepatic calcifications. Contrast-enhanced magnetic resonance imaging of the liver and gallbladder (Fig. B, ) revealed a normal size and shape of the liver and proportion of each lobe, and the intrahepatic and extrahepatic bile ducts and flow vessels ran naturally. A long T1 and long T2 cystic signal with a diameter of about 4.7 cm was observed in the right lobe of the liver, with liquid level, short T1 high signal intensity in the lower layer, high signal intensity on diffusion-weighted imaging sequence, and enhancement of the cyst wall on the enhanced scan. Test results from magnetic resonance imaging showed that the space-occupying lesion of the right lobe of the liver had been considered to be more likely a hepatic hydatid cyst. Preoperatively, three-dimensional reconstruction demonstrated the location of the tumor and its relationship with the surrounding vessels (Fig. A).\nThe patient underwent laparoscopic S7 segmentectomy. Intraoperative findings showed that the tumor was located at the S7 segment of the liver and was about 5 × 4 cm in size and partially protruding from the surface of the liver, with an intact capsule and clear boundary with normal liver tissue. The tumor compressed the right hepatic vein and its tributaries and densely adhered to the right hepatic vein (Fig. B). On postoperative pathology, a mass was observed immediately adjacent to the liver capsule, with a volume of 5 × 5 × 4.5 cm. The section surface showed a brown turbid fluid, a smooth inner wall, and greyish red, greyish yellow, and soft section surfaces of other liver tissues. The pathological section showed a low-grade mucinous cystic neoplasm (volume 5 × 5 × 4.5 cm) in the S7 segment of the liver, with steatosis in the surrounding hepatic tissue area, chronic inflammatory cell infiltration in the portal area, and no tumor cell involvement in the margin of the liver resection.\nImmunohistochemistry demonstrated tumour cells CK7 (+), CK19 (+), and CEA (−); stromal cells ER (+), PR (+), α-inhibin (a small amount +), vimentin (+), desmin (+), and actin (+) (Fig. A–C). This study was approved by the Ethics Committee of Yantai Affiliated Hospital of Binzhou Medical University.