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A 69-year-old Japanese woman was referred to our department in August 2015, complaining of swelling in the right preauricular region. She had shown a mass lesion of the right lung on a chest x-ray and was referred to the university hospital. Fluorodeoxyglucose-positron emission tomography (FDG-PET) examination showed accumulation indicative of a pulmonary lesion and a temporomandibular joint lesion. The temporomandibular joint lesion had been enlarging but was otherwise asymptomatic. Her medical history included treatment for pulmonary tuberculosis at the age of 13 and surgery to remove breast cancer at the age of 53.\nOn examination, a tender mass in the right preauricular region was palpable. The chin of the mandible was deviated to the right side during mouth opening (mandibular maximum mouth opening, 41 mm). Facial nerve function and mandibular nerve were intact. There was no indication of cervical lymphadenopathy.\nA panoramic radiographic examination showed resorption of the right mandibular condyle to the ramus (Fig. ). Computed tomography (CT) showed destruction of the right mandibular condyle and a large mass lesion with enhanced margin in the masticator space; a cystic lesion was present inside the tumor mass. Three-dimensional CT was useful to understand the bone resorption findings of the mandibular condyle. There was no finding of metastatic cervical lymph node (Fig. ). T1-weighted magnetic resonance imaging showed an enhanced mass lesion in the right masticator space. Because some portions of the mass lesion showed high intensity in T2-weighted images, cystic lesions were suspected to exhibit changes of blood flow or retention of high-protein liquid. Tumor development was not observed in the articular disk of the temporomandibular joint (Fig. ). FDG-PET revealed abnormal FDG uptake in the right submandibular condyle and masticator space.\nA malignant tumor was suspected after analysis by various modalities. Therefore, we performed an incisional biopsy via preauricular incision. Histopathologically, the tumor was largely composed of proliferative, atypical, spindle-shaped cells. Some tumor cells showed increasing mitotic change and extreme atypia (Fig. ). The histopathological findings of biopsy suggested spindle-cell sarcoma. Tumor resection was performed with the patient under general anesthesia via a combined preauricular and transmandibular approach to the masticator space and infratemporal fossa.\nAfter preauricular temporomandibular incision, the superficial temporal fascia and temporal fascia were elevated. The facial nerve (temporal and zygomatic branches) was protected by the fascia (Fig. a).\nThe facial nerve trunk was identified, and its branch was traced according to the conventional method.\nA midline lip-splitting incision was connected to the submandibular incision and accessed the anterior mandibular ramus. The lip-split incision traversed to the periosteum of the mandible; the periosteum dissector was used to elevate the soft tissues of the mandible, and the lateral mandible was exposed. The branch of the facial nerve was traced continuously with the skin flap, and the masseter muscle and deep parotid gland were dissected on the tumor side (Fig. c). The mandible was resected to within ≥ 20 mm from the primary tumor on the anterior of the mandibular ramus. The masseter muscle was divided at the lower edge of the zygomatic arch; further, the temporal muscle was divided horizontally at the height of the zygomatic arch to reach the side of the temporal bone. Excision was performed on the temporal bone to the base of the pterygoid process, and an osteotomy was performed continuously from the maxilla to the pterygoid process. Osteotomy was performed via ultrasonic scalpel. Then, deep excision was performed on the skull base until the foramen ovale was reached. The mandibular branch (VIII) of the trigeminal nerve was resected at the foramen ovale, and bone wax was used to fill the foramen ovale when hemostasis was achieved. The tumor was excised in bulk with the surrounding tissue (Fig. d). Especially, the glenoid fossa was close to the primary tumor site; however, it was excised including the joint disc, and the glenoid and infratemporal fossa were excised, including the periosteum of the skull base. For that reason, the margin was considered complete, and we did not perform the histopathological evaluation intraoperatively.\nThe surgical defect was reconstructed with a free vascularized fibula with skin paddle (Fig. e). The occlusion was performed with intermaxillary wire fixation for 1 week postoperatively. There were no abnormal findings during the postoperative course with complete healing. Right-sided facial nerve dysfunction appeared immediately after surgery.\nThe resected specimen exhibited nearly identical histological findings as observed in the biopsy. Because it involved a periosteal reaction corresponding partially to Codman’s triangle, the tumor was thought to have derived from bone (Fig. ). Immunohistochemical analysis showed positive staining for vimentin, MIB-1 index (40–80%), desmin, α-smooth muscle actin, Bcl-2, neuron-specific enolase, and S-100; it showed negative staining for AE1/AE3, caldesmon, and CD34. Thus, the final diagnosis was osteosarcoma (fibroblastic). The resection margin was negative for tumor. After excision of the mandibular tumor, excision of lung cancer was performed under thoracoscopy by a respiratory surgeon in our hospital. Although we recommended adjuvant treatment after surgery, the patient refused this treatment.\nThere has been no evidence of local recurrence or distant metastasis through 30 months of follow-up. The chin of the mandible deviates to the right side during opening (maximum mouth opening, 40 mm). Centric occlusion has not changed. Facial nerve dysfunction gradually improved and became mild according to House-Brackmann Scale evaluation (Figs. and ). |
A 30-year-old female diagnosed elsewhere with choroidal melanoma with retinal detachment, presented to our emergency department with complaints of seeing black spot in front of the right eye accompanied with intermittent flashes for 20 days. On examination, the best-corrected visual acuity (BCVA) was 6/9 in the right eye and 6/5 in the left eye. Intraocular pressure measured with Goldmann applanation tonometry was 11 and 15 mmHg in the right and left eye, respectively. Anterior segment examination was unremarkable. Fundus examination of the left eye was within normal limits. Fundus examination of the right eye revealed a large endophytic yellowish white, well-demarcated mass, with surface vascularity, located in the temporal quadrant extending up to the macula along with the presence of multiple subretinal yellowish infiltrates inferiorly []. There was overlying exudative retinal detachment involving the inferior portion of the retina reaching the macula. A provisional diagnosis of adult onset retinoblastoma with subretinal seeding and exudative retinal detachment was made. The patient underwent ocular ultrasound examination of the right eye which revealed a dome-shaped mass with homogenous internal structure, high surface reflectivity, and variable low to medium internal reflective echoes, with a basal circumference of 11.1 mm and height of 5.8 mm and the presence of an exudative detachment reaching up to the macula. Magnetic resonance imaging (MRI) of brain also revealed a retinoblastoma in the right eye with no evidence of extraocular or optic nerve invasion. The patient subsequently underwent six cycles of chemotherapy (carboplatin, vincristine, and etoposide) combined with transpupillary thermotherapy over a period of 6 months with regular monthly follow-up. After the third cycle, the tumor mass started showing excellent regression []. As some residual activity was present at the end of six cycles, she underwent two more cycles of chemotherapy and by the end of 8 months, the tumor had regressed completely leaving a flat scar []. The lesion was quiescent for a period of about 10 months. On follow-up, 10 months after the last treatment, BCVA was 6/9 in the right eye and fundus examination revealed a regressed tumor with the presence of multiple small new tumor foci in the periphery [] which were treated with laser using indirect ophthalmoscopy and transconjunctival cryotherapy. She also received three more cycles of chemotherapy. She was followed-up for a period of 2 years and in the event of new tumor foci [], she was given focal therapy (either transpupillary thermotherapy or cryotherapy) subsequently resulting in tumor regression []. After 2 years, at a follow-up visit, the BCVA had decreased to 6/60 in the right eye and fundus examination revealed significant media haze due to massive vitreous seeding [] and the presence of complicated cataract along with a substantial increase in the tumor size []. She was given the options of enucleation or lens sparing external beam radiotherapy. Patient was not willing to undergo enucleation initially. As the tumor showed further progression over the next 2-3 months, the patient was advised to undergo enucleation with ball implant with the aim of preventing further tumor spread and she agreed. Histopathologic examination of the enucleated globe revealed a moderately differentiated retinoblastoma with choroidal invasion of more than 3 mm and presence of tumor cells in the anterior fibers of the sclera. There was no evidence of optic nerve invasion. In view of choroidal invasion, she was advised 6 cycles of adjuvant chemotherapy; however, patient was not keen to undergo treatment and underwent just one cycle of chemotherapy and then discontinued treatment. On her last follow-up visit, 6 months later, the socket was healthy and the left eye remains normal. |
A 63-year-old female with a history of prior lacunar stroke, hypertension, chronic back pain, and hypothyroidism presented to a community emergency department one hour after the acute onset of severe bilateral lower extremity pain which progressed, within minutes, to bilateral lower extremity generalized weakness and then flaccid paralysis. In addition, within an hour of symptom onset, she developed dull, diffuse abdominal pain with radiation to the bilateral flanks, lasting one hour with subsequent resolution.\nAt presentation, she was found to have a blood pressure of 131/88 mmHg, tachycardia with a heart rate of 110 beats per minute, oral temperature of 96.8 degrees, F and SpO2 of 97% on room air. The patient presented with 0/5 strength and decreased sensation to light touch and areflexia of the bilateral lower extremities. Noncontrast computed tomography (CT) of the head showed a remote lacunar infarct. CT angiography of the abdomen and pelvis showed no evidence of pulmonary embolus, abdominal aortic aneurysm, or aortic dissection. Surface electrocardiogram showed sinus tachycardia without ischemia.\nThe patient was transferred to a tertiary care center for advanced imaging and care, where a T2-weighted magnetic resonance image (MRI) showed a nonspecific central intrinsic signal hyperintensity in the conus region extending to the level of T8, concerning for acute spinal cord ischemia (Figures and ). Transthoracic echocardiogram, performed to determine if there was a cardiac source of emboli, showed atypical dyskinesia with left ventricular apical ballooning and an ejection fraction of 35%, a characteristic of neurogenic stunned myocardium (Figures and ). Cardiology was consulted, and a diagnosis of takotsubo cardiomyopathy was made, with no further recommendation for heparinization or catheterization. Pertinent laboratory results are shown in .\nThe patient was admitted to the neurology inpatient service for supportive care including physical and occupational therapy. During admission, the patient was evaluated for stroke risk factors, and statin therapy was subsequently initiated for hyperlipidemia. No other specific underlying etiology of the spinal cord ischemia was found. Cerebrospinal fluid studies were performed to rule out other etiologies for paralysis and were unremarkable. The patient was started on low-dose beta-blocker and ACE-inhibitor therapy per cardiology recommendations, and the patient was discharged to a rehabilitation facility with the recommendation to have a transthoracic echocardiogram six months after discharge. The patient was subsequently lost to followup. |
A 37-year-old female patient presented with a 5 months history of constant pulsatile frontal headache. The headaches were only somewhat responsive to paracetamol but she had to take narcotics to get to sleep. Recently, she had noticed that she was unable to distinguish smells while cooking and the food had a different taste for her.\nNeurological exam revealed anosmia and bilateral papilledema more severe on the left side. Visual acuity was 20/20 and 20/40 in the right and left eyes, respectively. The remainder of the physical exam and the laboratory tests were all within normal range.\nAxial brain CT scan was performed and demonstrated an extra axial hyperdense interhemispheric space-occupying lesion within the anterior cranial fossa. Surrounding vasogenic edema was evident. Brain magnetic resonance imaging (MRI) with and without contrast revealed the mass that was isointense relative to the gray matter on T1 and T2-weighted images. The tumor showed a homogenous contrast uptake with gadolinium injection suggesting anolfactory meningioma [].\nThe patient was operated in the supine position and the head was placed in the neutral position. A bifrontal craniotomy was performed over the superior sagittal sinus (SSS). The dura was opened in a linear fashion on both sides of SSS and it was ligated at its anterior third. Gross total resection of the firm tumor was made, the infiltrated dura was resected and the tumor's adhesions to the anterior skull base were coagulated (Simpson grade II).\nThe patient awoke with sudden bilateral blindness. Her vision was no light perception (NLP) in both eyes. Extraocular movement and IOP were normal. Fundoscopy revealed no apparent change. The anesthesiologist records documented no intra-operative hypotension. Early axial brain CT scan excluded the possibility of developing mass effect and 24 h brain MRI showed no residual tumor. One main concern was the possibility of inadvertent surgical trauma to the optic nerves or chiasm. Review of the operative video excluded this possibility, as well as any external pressure on the eyes during the surgery. Visual evoked potential (VEP) responses were completely abolished in both eyes.\nSerial therapeutic lumbar punctures (LP) were done and high dose methylprednisolone were given for three days with the hope to resolve possible optic nerve entrapment, which was unsuccessful. After six weeks post-procedure, she gained some light perception in her right eye. Follow-up fudoscopy revealed bilateral optic disc atrophy after two months. During 52 months follow up, her visual status showed no improvement. Fundoscopy revealed bilateral optic atrophy [].\nInformed consent was sought and granted from the patient's family to report this case. |
A 26-year-old man was referred to our eye hospital in July 2017 with a bulbar conjunctival lesion (8 mm × 6 mm) of the right eye. Upon examination, a large pigmented lesion with numerous small cysts was present on the superior bulbar conjunctiva (). The initial clinical findings showed a tumor, which appeared to be smooth, soft, and mobile.\nFor this case, an AMT using the inlay technique was applied, and the surgery is shown in . The conjunctival tumor was resected using a wide excisional biopsy with a tumor safety distance of 2.0 mm. Then, the resection was performed using the “no-touch” technique. Later, the conjunctival defect was reconstructed using an AM, which was placed with the stromal side facing down toward the defect. It was secured to the surrounding conjunctiva by five interrupted 9-0 Vicryl sutures.\nA topical corticosteroid (dexamethasone), antibiotic eye drops (0.5% moxifloxacin hydrochloride ophthalmic solution), and artificial tears were used postoperatively four times a day over a 2-week period. Then, the corticosteroid eye drops were reduced by one drop each week, while the artificial tears were still applied four times a day for 8 weeks.\nshows the histopathological slides of the nevus stained with hematoxylin and eosin (×20 and ×50 magnification), on which numerous nests of nevus cells containing abundant melanin pigment could be seen under the conjunctival epithelium. shows this eye’s course of healing over time, in which a reduction in the inflammation and healing of the conjunctival defect could be seen. The conjunctival epithelium grew over the AM graft; therefore, the AM became partly integrated into the host tissue. In addition, the conjunctival inflammation decreased quickly. Six months after the resection and AMT, a slit-lamp examination revealed stable findings.\nWritten informed consent of the patient was obtained to publish this case report. In addition, this case report was approved by the institutional review board of the University of Tübingen and adhered to the tenets of the Declaration of Helsinki. |
Patient A.B, 47 year old woman with 10 year history of gradual painless proptosis of the right eye. Examination showed right visual acuity of 6/12, non axial proptosis, superotemporal firm, non tender orbital mass, mild ophthalmoplegia, and choroidal folds on fundoscopy. The right optic disc was normal. Regional lymph nodes were not palpably enlarged. The left eye was normal at presentation. Cranial computerized tomography scan showed a circumscribed superolateral orbital mass, with no intracranial extension. An assessment of a lacrimal gland tumour was made.\nThe two patients had lateral orbitotomy in conjunction with the maxillofacial surgeons. At surgery, a C shaped skin incision was made just lateral to the lateral orbital rim, Hemostasis was secured. Subcutaneous tissue and temporalis muscle were dissected to gain access to the lateral orbital rim. Periosteal elevator was used to expose the fronto zygomatic suture, and with the aid of a drill, two bore holes were drilled on either sides of the suture to facilitate the removal of a bone window by a rotating electric saw (). The periorbital was then incised to expose the orbital contents. The lateral rectus and orbital fat were retracted to expose the retroocular space. In the first case, a firm fusiform retroocular tumor, measuring 30 by 20 by 20 mm was excised and sent for histopathological examination (). In the second case, a firm encapsulated mass was removed completely from the lacrimal fossa measuring 35 by 33 by 32 mm. It was also sent for histopathologic examination (). The periorbital was closed and the bone fragment secured with surgical wire. The periosteum, temporalis muscle, subcutaneous tissue and skin were closed in layers. The proptosis regressed promptly ( and ). Hemostasis was secured by firm bandaging. Intravenous antibiotics and oral steroid were administered for infection prophylaxis and postoperative edema respectively.\nAll the instruments used were provided by the maxillofacial surgery department.\nThe sections of specimen from the first patient were in keeping with pilocytic astrocytoma grade I. In the second patient, the sections showed a malignant epithelial neoplasm consistent with adenocystic carcinoma of the lacrimal gland. Differential diagnosis of proptosis in an adult include other causes of lacrimal swelling such as dacryoadenitis, benign mixed lacrimal tumors, secondaries from breast in women and prostate in men. Investigations and examination did confirm a malignant neoplasm.\nPatients had postoperative external beam radiotherapy. |
A 27-year-old morbidly obese African American, gravida 1, para 0 female presented during the first trimester of her pregnancy with swelling in the right jaw and loosening of teeth. The patient also complained of localized pain but denied fever, weight loss, and other constitutional symptoms. On examination, there was a tender swelling of the right mandible. A biopsy was performed that showed mixed inflammation with numerous foamy histiocytes suggestive of Rosai-Dorfman disease. A CT scan was subsequently performed to delineate the extent of the disease to rule out nodal disease and other possible areas of involvement. The CT scan demonstrated a lytic lesion involving much of the body of the right side of the mandible, extending into the mentum of the mandible and crossing the midline into the anterior aspect of the left body of the mandible (). The lesion showed avid radiotracer uptake on scintigraphy (). Needle core biopsies were performed of the suspicious lymph nodes, which on histopathologic evaluation showed reactive hyperplasia. The patient subsequently underwent a segmental mandibulectomy.\nThe gross specimen received in pathology consisted of a 14.0 cm segment of the mandible with teeth number 19-27 and 31-32 (). Teeth 17-18 and 28-30 had been removed previously. The mandible was markedly asymmetric with an enlarged and bulging right lateral portion. Sectioning revealed a gray, soft, focally cystic mass involving two-thirds of the resected segment of the bone. Histologic evaluation showed a locally destructive lesion composed of numerous foamy histiocytes, admixed with lymphocytes, neutrophils, and scattered eosinophils and plasma cells infiltrating into the peri-mandibular soft tissue ().\nSome of the histiocytes also demonstrated emperipolesis (). Immunohistochemical stains showed the histiocytes to be positive for CD68 and S100 while negative for CD1a (). The morphology and immunohistochemical profile were consistent with Rosai-Dorfman disease. The concomitantly removed submandibular gland and suspicious lymph nodes showed no evidence of involvement by the disease. The patient underwent reconstruction with osteomycocutaneous fibular flap. |
A 7-year-old boy whose features included microtia, external-ear canal stenosis, congenital cholesteatoma in the middle ear and mastoid with postauricular abscess and automastoidectomy and a first branchial cleft fistula opening in the middle ear through the zygomatic root was referred to our clinic ().\nDischarge from the periauricular sinus with wide tract had been occurring since 3−4 years previously. Discharge ended upon antibiotic treatment, but was reported again after cessation of treatment. In a clinical examination, we observed severe auditory canal stenosis and 55-db conductive hearing loss in pure tone audiogram. These results were consistent with tuning tests. Facial nerve function was normal. Imaging studies such as computed tomography (CT) showed the extent of the bony erosion in the mastoid air cells (automastoidectomy) with cortical fistula to the skin. The middle ear and mastoid were filled with the appearance of soft tissue ().\nThe first branchial fistula and its tract to the zygomatic root were shown by fistulography ().\nAn exploration of the mastoid through a postauricular approach, with the fistula site in the incision, was planned. One large mastoid cavity was found to be full of cholesteatoma, extending from the anterior wall of the middle ear where the branchial fistula was opening and completely obliterating the external auditory canal, middle ear and mastoid cells with ossicular chain erosion. Canal wall down mastoidectomy with canaloplasty and wide meatoplasty was performed. The Eustachian tube was also cleaned completely. The branchial cleft fistula from the anterior auricle to the zygomatic root was excised through parotidectomy and facial nerve dissection ().\nThe tract of the first branchial fistula crossed the superior branch of the facial nerve. There was a hole in the zygomatic root in which the epithelium was extended to the middle ear. The tract was excised completely and confirmed pathologically as squamous epithelium. The site of the operation was closed. Facial nerve function was normal in the post-operative period. No evidence of recurrence was observed during a 6-month follow-up period. |
A 65-year-old male presented with a three-week history of progressively worsening abdominal distension. He denied any abdominal pain but stated that he noticed an increased frequency of bowel movements. His past medical history was unremarkable. On examination, he was afebrile and had a hugely distended abdomen with an immobile, nontender mass occupying all four quadrants of the abdomen. Computed tomographic (CT) scan revealed a large, heterogeneous lobulated mass occupying most of the abdomen (Figure ). The peripheral component appeared lipomatous and the margins of this component were difficult to estimate accurately. There was also a heterogeneous nonlipomatous component that contained areas of lesser density, as well as a central stellate region of calcifications. The preoperative differential diagnosis included a retroperitoneal sarcoma (especially dedifferentiated liposarcoma), desmoid tumor, undifferentiated carcinoma, carcinoid or sclerosing mesenteritis; lymphoma was also considered.\nWhile neoadjuvant chemotherapy and radiation comprise a frequent approach for retroperitoneal sarcomas at our institution, the extent of the tumor made this approach unfeasible. Resection was therefore planned unless an intraoperative biopsy revealed lymphoma. Resection was accomplished through a T-type incision (Figure ), and entailed removal of the right kidney, terminal ileum, ascending colon, sigmoid colon and the left spermatic cord structures, all of which were intimately attached to the mass. Encasement of the external iliac artery and vein was also encountered near the end of the procedure. This was not fully appreciated preoperatively, as that component of the tumor was so much less conspicuous on CT than the rest of the tumor, given its fatty consistency (Figure ). The mass was split in half to facilitate dissection from the iliac vessels. An anastomosis was constructed from descending colon to rectum. A transverse colon mucous fistula and an ileostomy were brought out, as the ileum was dusky at the end of the procedure.\nThe tumor was submitted for histological examination in two parts measuring, 30.0 × 10.0 × 6.5 cm and 32.0 × 20.0 × 10.0 cm, weighing 19.8 kg in total (Figure ). On gross examination the mass was variegated, with fleshy and solid cystic degeneration containing areas of osseous consistency. Microscopic examination revealed a juxtaposition of well differentiated liposarcoma and a spindle cell sarcoma with heterologous chondrosarcomatous elements consistent with a dedifferentiated liposarcoma (Figure ).\nThe patient was discharged from hospital on the seventh postoperative day. He was followed in the surgical oncology outpatient clinic monthly. Four months after resection the patient had a follow-up CT scan, which demonstrated an intra-abdominal recurrence consisting almost completely of a calcified, nonlipomatous tumor (Figure ). The patient died one month later. |
A seven-year-old girl presented to our specialized pediatric dentistry office referred from her dentist for a persistent firm swelling localized at the upper right maxillary region.\nMedical and physical examinations revealed a healthy girl with no extra-oral findings except the facial asymmetry. Intra-orally, a painless tumefaction of hard consistency obliterating the vestibule of the right maxilla and extending from the tooth # 53 to the distal of the tooth # 16 was detected ().\nThe overlying mucosa was normal in color and texture. On palpation, no regional lymphadenopathy was noticed.\nThe panoramic radiograph, done previously by her dentist, showed a well-defined radiolucent lesion in the right maxilla and absence of the tooth # 54 which was extracted as an attempt of treatment for her condition ().\nIn order to assess the bone and to evaluate the extension of the lesion, a cone-beam computed tomography (CBCT) radiography was requested.\nThe CBCT radiograph showed a well circumscribed mixed image, starting distal to the tooth #53 till the mesial of the # 55, and from the crestal edge till the germ of the permanent canine with a diffuse border containing fine radio-opaque trabeculae with a buccal, palatal, and crestal expansion of the bone; a disruption of the buccal bone was also noticed. The image of the right sinus was normal ().\nAn incisional biopsy was performed and sent for histopathologic assessment.\nMicroscopic examination showed a proliferation of fusiform cells with regular nuclei arranged in bundles and storiform pattern associated with multinucleated giant cells. Within these irregular strands, mineralized pieces in the form of trabeculae were found. The stratified squamous epithelium surface showed moderate acanthosis and hyperkeratosis ().\nThe histological diagnosis of trabeculae juvenile ossifying fibroma was made.\nThe tumor measuring approximately 1.8 x 2.5 cm was surgically excised under general anesthesia via intra-oral approach. During the procedure, the teeth # 53 and # 55 were removed ().\nPost-operative recovery was without complications, and the patient was discharged from the hospital 48 hours following the surgery.\nHistopathological results of the excisional biopsy were consistent with that of the incisional one.\nThe patient was followed for two years, once every six months. She remained recurrence-free clinically and radiologically (). |
A 36-year-old sub-Saharan African woman presented to the Accident and Emergency department with a 7-hour history of vomiting and central abdominal pain radiating to the back. There were no respiratory, cardiovascular or urinary symptoms, and past medical history was unremarkable. The blood results included an amylase of 3070 U/litre and an abdominal ultrasound showed multiple tiny gallstones confined to a thin-walled gallbladder with normal pancreas, liver, kidneys and spleen. A diagnosis of gallstone-induced pancreatitis was made and laparoscopic cholecystectomy was performed 5 days later, once her symptoms had settled. An on-table cholangiogram demonstrated a filling defect at the distal end of the CBD with no duodenal filling. Laparoscopic CBD exploration was undertaken and two stones were removed from the distal CBD using a Dormia basket through the choledochoscope. A Latex 12-Fr T-tube was inserted into the CBD at the end of the procedure. The patient made an uneventful recovery postoperatively and was discharged with the T-tube spigotted and left in situ.\nA T-tube cholangiogram 3 weeks later excluded any bile duct obstruction or leakage and the T-tube was therefore removed without difficulty. However, the patient soon started vomiting and complained of increasingly severe right upper quadrant abdominal pain. Following overnight observation, ultrasonography for suspected bile leak was inconclusive. Biliary peritonitis was clinically suspected and an emergency diagnostic laparoscopy was performed. This revealed that the fistula had become disconnected at the point of contact with the anterior abdominal wall (Fig. ). Bile was clearly visible draining from the fistula opening (arrowed). The whole length of the fistula was inspected and no other leak was found; the proximal junction with the CBD was intact. The distal fistula was recannulated with a 10 Fr Latex drain (Fig. ) and bile was observed to be draining freely from it. Following an uneventful recovery, cholangiography of the cannulated tract with the Latex drain in-situ was repeated after 5 weeks and no dye was able to pass down it. The tract was therefore presumed to have closed. The Latex drain was removed 24 hours later with good recovery to date and no further complications. |
The patient was a 41-year-old female who was diagnosed with sigmoid adenocarcinoma in January 2018. The patient was considered potentially eligible for surgery. Surgical intervention revealed stage T4N2M1 tumor, which involved all layers of the colorectum, showed peritoneal dissemination, and metastasized to the ovaries. Complete cytoreduction was achieved by sigmoid resection, extended lymph node dissection, hysterectomy with adnexectomy, and parietal subtotal peritoneumectomy. The molecular analysis revealed that the tumor contained KRAS G12D mutation. The anatomic involvement of the ovaries suggested that the patient may have a concurrent ovarian cancer. However, the pathological report and the KRAS mutation analysis confirmed the gastrointestinal origin and the identical KRAS status for all tumor lumps. Therefore, the diagnosis of multiple primary carcinoma (i.e., the combination of CRC and ovarian cancer) was considered highly unlikely and the woman was managed as a CRC patient. The FOLFOX adjuvant therapy was given for 6 cycles. Starting from June 2018, the patient went on to observation. The disease relapse was revealed in November 2018: there was a metastatic spread in the lungs and peritoneum accompanied by ascites. The patient received XELOX plus bevacizumab from November 2018 to May 2019, then capecitabine plus bevacizumab from July 2019 to January 2020. This resulted in disease stabilization lasting until March 2020, when computed tomography revealed progression of lung metastases, involvement of the lung lymph nodes and bilateral pleuritis. The performance status deteriorated to ECOG 2.\nThe reintroduction of chemotherapy, for example, the use of irinotecan-containing schemes, did not look feasible due to apparent chemoresistance of the tumor and poor condition of the patient. The option of a compassionate experimental therapy was discussed with this woman, and she provided an informed consent to receive binimetinib 30 mg twice per day, hydroxychloroquine 400 mg twice per day, and bevacizumab 7.5 mg/m2 every 3 weeks. The treatment started in the beginning of April and led to the relief from dyspnea within the first days of the therapy. Twelve days of the treatment resulted in a decline of the serum level of the СA 19-9 marker from 118 to 69 U/mL and of CEA protein from 430 to 251 U/mL, while there was a rise of the CA-125 marker from 164 to 340 U/mL. The performance status improved to ECOG 1. The control computed tomography was carried out 6 weeks after the treatment start and revealed evidence for a 17% reduction in the size of lung metastases (stable disease by RECIST 1.1, shown in Fig. ). |
A 57-year-old female who underwent SPK (1998) and developed eventual loss of function of both renal and pancreas grafts secondary to chronic rejection. She underwent a live donor renal transplantation (2015) placed in the left iliac fossa via midline laparotomy and transperitoneal approach. Her failed pancreatic graft remained in situ, on the right side. Renal function remained normal until 1 week prior to her acute presentation.\nShe presented to her local hospital due to sudden onset of severe abdominal pain with associated nausea, vomiting and diarrhoea. She was tachycardic though normotensive on presentation and found to have generalized severe abdominal pain. CT imaging revealed an SBO with an evidence of ischaemia as well as an obstructed ureter and renal collecting system within the allograft (). She underwent laparotomy, division of band adhesion in the left pelvis and 1.2 m of small bowel resected due to ischaemic necrosis with an end-to-end hand sewn anastomosis.\nPost-operatively, she was noted to be anuric and the quaternary referral centre with transplantation services was contacted for transfer.\nAfter transfer, the patient was noted to have ongoing severe abdominal pain in the lower abdomen and ongoing anuria. She was haemodynamically stable, afebrile and presentation was consistent with acute graft injury secondary to ischaemic bowel.\nOn arrival, her haemoglobin was 120 g/L with white cell count 14.7 × 109/L, and CRP 157 mg/L, creatinine 348 umol/L (baseline 80), eGFR 10 mL/min, Urea 10.1 mmol/L and potassium 5.2 mmol/L.\nRepeat CT imaging with IV contrast at the referring hospital prior to transfer revealed large volume free fluid intraperitoneally with ongoing dilatation of the renal collecting system which was contrast filled. The distal ureter was not seen entering the bladder. The free fluid in the pelvis also demonstrated increased density raising concern for haematoma or possibly intravenous contrast ( and ).\nThe patient underwent a repeat laparotomy 48 hours after initial operation. Findings during the case included large volume of intraperitoneal urine with a complete transection of the transplant ureter (). The transplant ureter had been mistaken for a band adhesion, and the small bowel had clearly herniated beneath ureter, causing obstruction to the kidney and infarction of the strangulated small bowel. The ureter was reimplanted onto the bladder with the formation of a Boari flap, stented, without tension, having first excised the end of the diathermied ureter to ensure viable and well-vascularized tissue (–).\nThe patient recovered well post-operatively with immediate function of the renal graft and rapid normalization of the creatinine within 24 hours. She was discharged home post-operative Day 5 with an IDC in situ and returned for a CT cystogram and removal of stent. Unfortunately, her recovery was further complicated by stricture of the reimplanted transplant ureter. The ureteric obstruction was decompressed with a nephrostomy tube. A nephrostogram, antegrade stent insertion and removal of nephrostomy were subsequently performed. Her renal function normalized and her IDC removed. She was discharged home with urological follow-up. |
A 10-year-old female with a one-year history of a progressively enlarging right parotid mass was referred for pediatric otolaryngology evaluation. The patient had no known risk factors for head and neck malignancies. There was no family history of thyroid or other malignancies and no history of childhood radiation. Examination revealed a 2 cm right parotid mass with associated cervical lymphadenopathy. Workup for autoimmune disease revealed CBC, ESR, ANA, and immunoglobulin tests to be within normal limits with no immunophenotypic evidence of ALPS. CT demonstrated enlarged right intraparotid and cervical lymph nodes. Ultrasound-guided biopsy of the parotid mass revealed a reactive lymphoid process. Excisional biopsy of the multiple cervical lymph nodes revealed florid follicular lymphoid hyperplasia with few follicles demonstrating infiltration of the germinal center by small mature mantle zone lymphocytes consistent with a pathologic diagnosis of PTGC. Due to the potential association with NLPHL, PET/CT was performed, and increased uptake was noted in the right parotid, bilateral jugulodigastric chains, right supraclavicular region, right axilla, peripancreatic region, and bilateral inguinal regions (). Additional excisional biopsy of a particularly high FDG uptake supraclavicular lymph node revealed PTGC without any evidence of malignancy (). The patient was followed up clinically and the lymphadenopathy remained stable for two years. Incidentally, the patient was evaluated by her primary care physician for thyroid abnormalities after developing unexpected weight gain and fatigue. Laboratory workup was negative for hypothyroidism. Thyroid ultrasound demonstrated an isolated left thyroid nodule and subsequent FNA was suggestive of papillary thyroid carcinoma. Total thyroidectomy with central neck dissection was performed. Despite the presence of persistent bilateral jugulodigastric lymphadenopathy, the lateral necks were not addressed surgically. Pathology confirmed papillary thyroid carcinoma (), and two of four lymph nodes demonstrated metastatic disease without extranodal invasion. Pathologic TNM stage was T1a, N1a, and M0 (Stage 1). No lymph nodes had evidence of PTGC. Postoperative radioactive iodine scan showed minimal uptake in the thyroid bed and no uptake in the lateral necks. The patient received adjunct radioiodine (RAI) ablation therapy. The patient remains well without evidence of recurrent disease 3 years after treatment. |
A 24-year-old gentleman with no past medical history had been experiencing debilitating chest pains for 2 years. He described these as varying in intensity, worsened by exercise, and relieved partially by analgesia. They were not associated with palpitations or syncope.\nUnfortunately, due to his symptoms he had to give up his studies and minimize his physical activity. He had trialled multiple analgesics (daily paracetamol and tramadol) with partial relief of symptoms.\nHe underwent serial investigations at his local cardiology unit, including a cardiac CT which identified an absence of pericardium on the left side of the heart. He was referred to the Adult Congenital Heart Disease (ACHD) team at The Royal Brompton Hospital.\nWhen first reviewed in the ACHD clinic he was not cyanosed, in sinus rhythm with no evidence of cardiovascular decompensation. His apex beat however was grossly displaced to the left and more so on lying supine. His first heart sound was normal, second split, there was also a soft one-sixth systolic heart murmur. The rest of his examination was unremarkable.\nHis electrocardiogram showed him to be in sinus rhythm at 78 b.p.m., with normal conduction times but a poor R wave progression and an intermittently changing axis.\nA chest radiography (Figure ) which showed situs solitus, levocardia, with a left aortic arch, no displacement of the heart to the left, and a protrusion at the level of the left atrial appendage at the left upper quadrant of the heart.\nHis echocardiogram showed a structurally normal heart with maintained ventricular function. Stress echocardiography showed mild right ventricular dilatation at rest with increase in right ventricular volume directly after exercise. A cardiac CT (Figure ) revealed a partial absence of pericardium on the left side with a small layer of pericardium on the upper right heart border. The heart was grossly shifted leftwards and posteriorly, highly suggestive of enhanced heart mobility. Similarly, no pericardium could be seen on cardiac magnetic resonance imaging (CMR). There was lung tissue in between the base of the heart and diaphragm (Figure ), the heart was displaced to the left of the chest and the RV appeared dilated (Figure ).\nThe patient’s symptoms were thought to be due to his absent pericardium and related to cardiac mobility. We were struck by the discrepancy of the erect Chest X-ray (CXR) and supine CMR with regards to cardiac position, mobility, and the severity of disabling symptoms. We thus discussed the patient at our ACHD Multidisciplinary Team meeting and offered him surgery, in an attempt to immobilize his heart, hoping to improve his symptoms and quality of life.\nThe heart was exposed via a left postero-lateral thoracotomy. There was complete pericardial agenesis on the left side and on the diaphragmatic surface. Several 0.4 mm thickness Gore-tex® patches were sutured together to create a large sheet and this was sutured down with widely spaced interrupted 3-0 Prolene® sutures to the posterior mediastinum, starting at the level of the left pulmonary artery, posterior to the left atrial appendage and along the left hilum and inferior pulmonary ligament, to the diaphragm and down. This was then brought forward to the anterior chest wall and then fixed anteriorly along the anterior chest wall with the superior end free (see Figure ). A left pleural drain was inserted and a paravertebral catheter for analgesia, followed by routine multilayered chest closure.\nThe post-operative course was uneventful apart from a small left apical pneumothorax with a small pleural effusion. Both resolved spontaneously within days.\nEchocardiography on Day 5 showed that the RV was of normal size with no evidence of pericardial effusion nor compression of cardiac chambers. The patient was discharged 8 days after his procedure on simple analgesia, mobilizing well with improved symptoms.\nThree months from surgery the patient had come off analgesia completely, with resolution of his debilitating chest pains. He was resuming his University studies and restoring normality in his life. |
A 38-year-old woman with a medical history of hypertension, chronic obstructive pulmonary disease, osteoarthritis of knees, chronic smoker, and cocaine abuser presented to the emergency department with complaints of painful body rash which appeared within some hours after smoking cocaine. The rash first appeared on her right arm, followed by rash on the right thigh, left thigh, and left arm. This rash was associated with a burning pain, ten out of ten in intensity without radiation and mostly located on her right arm and right thigh. There was no skin peeling or purulent discharge noted. She denied having any oral or genital mucosal ulcer. She denied any physical injury, fall, pruritus, gastrointestinal symptoms, genitourinary symptoms, fever, chills, chest pain, dyspnea, headache, blurring of vision, dizziness or urinary symptoms. She denied any other systemic complaint. Her home medications include amlodipine, albuterol inhaler as needed, and acetaminophen as needed. She has never been prescribed any anticoagulant including warfarin.\nAlthough she has been smoking cocaine for more than 20 years, the first episode of skin rash post cocaine use did not occur until 3 years ago. At that time, the rash also occurred on bilateral legs and she was admitted to a local hospital. She underwent skin biopsy and it revealed vasculitis with fibrin-platelet thrombi consistent with levamisole-induced vasculitis. She was counseled to stop using cocaine and was treated with intravenous steroid followed by tapered dose of prednisone. The next episode of similar rash lesions of bilateral legs developed in 2014 after smoking cocaine and she again responded to steroids treatment. Six days prior admission to our hospital, she used a larger amount of cocaine than usual (200 dollar-worth). She denied any allergy to cold weather, food, or medications.\nInitial vital signs included a temperature of 98°F, pulse rate 98 beats/min, respiratory rate 18 breaths/min, blood pressure 105/72 mmHg, and oxygen saturation 97% on room air.\nPhysical examination revealed tender, retiform, violaceous necrotic purpuric lesions with overlying bullae and healed old scars on both lower extremities on posterior aspect of the right thigh and right arm [Figures and ]. There was no pus or discharge. Pulmonary examination as well as cardiovascular, central nervous system, abdominal examinations were within normal limits.\nInitial laboratory tests showed white blood cells 4.1 × 109/L, normal differentials, erythrocyte sedimentation rate 52 mm/h, C-reactive protein 22.1 mg/L, hemoglobin 12 g/dL, hematocrit 35.3%, platelets 363,000/μL, blood urea nitrogen 22 mg/dL, creatinine 0.8 mg/dL, glomerular filtration rate 85.32 ml/min, and total creatine kinase 51 IU/L. Comprehensive metabolic panel, kidney function test, liver function tests, and coagulation profile were within normal limits. Urinalysis revealed high specific gravity with proteinuria (100 mg/dL) and 5–15 red blood cells per high power field. Urine toxicology test was positive for cocaine. Levamisole levels were not done. Serological tests for human immunodeficiency virus, hepatitis A, B, C virus, and rapid plasma regain were negative. Immunologic tests revealed positive antinuclear antibody with nucleolar pattern, high atypical perinuclear antineutrophil cytoplasmic antibody (atypical P-ANCA), 1:320 titer (normal: <1:20), antimyeloperoxidase (MPO) antibody 20.4 U/mL (normal: 0–9 U/mL) with negative P-ANCA. Cytoplasmic ANCAs (C-ANCA) and anti-proteinase 3 (PR3) antibody were negative. Rheumatoid factor, JO-1 antibody, Sjögren's antibody, Sjögren's syndrome type B antibody, antinuclear antibody, antidouble-strand deoxyribonucleic acid, anti-Smith antibody, centromere B antibody, complement C3, C4, total complement (CH50) levels, and chest X-ray were within normal limits. Testing for cryoglobulins, lupus anticoagulant, anticardiolipin antibody, antiphospholipid antibody, and antihuman neutrophil elastase (HNE) antibodies was not done. Serology tests results done during episodes of cocaine-induced vasculitis within 3 years showed high titers of P-ANCA [].\nIn 2013 and 2014, vasculitic panels showed a positive P-ANCA with high anti-MPO antibody. However, in 2016, atypical P-ANCA pattern with high anti-MPO antibody was found.\nA punch skin biopsy of 3 mm in diameter and 2 mm in length was performed from her right upper leg lesion. Low magnification (×10) hematoxylin and eosin (H and E) stain showed superficial and deep vasculitis and perivasculitis []. In higher magnification (×40), the H and E section revealed leukocytoclastic vasculitis with fibrinoid necrosis and a mixed inflammatory cell infiltrate composed of neutrophils, eosinophils, and lymphocytes. There was marked leukocytoclasis (karyorrhexis and nuclear dust). Focal area showed marked red cell extravasation and vascular thrombosis [].\nThe patient was counseled to stop using cocaine and was treated with methylprednisolone 125 mg intravenously on the admission day. Oral prednisone 60 mg daily was started on day 1 and continued for 3 days. The improvement from skin lesions was seen after 72 h from admission [Figures and ]. Tenderness over skin lesions was resolved. Prednisone 40 mg per os was given on day 4 and she was discharged with oral prednisone 20 mg daily for 1 week, followed by 10 mg daily for another week. She was recommended to follow-up with rheumatology clinic; however, she did not follow-up. |
A 38-year-old woman with a 3-month history of an anterior cervical mass located in the suprasternal region of her neck was referred to our hospital. Physical examination revealed that the 3-cm movable neck mass was firm and slightly tender on palpation and had a distinct margin from surrounding tissues. An ultrasound examination showed that the well-defined oval mass was 31 × 23 × 17 mm in size and exhibited heterogeneity. Furthermore, a non-enhanced computed tomography (CT) scan of the neck revealed that the distinct neck mass in the subcutaneous tissue had a mixture of soft tissue and fatty components (Fig. ). Based on these findings, the cervical tumor was clinically diagnosed to be an unusual lipoma with degeneration; however, we could not exclude the possibility of it being a malignant tumor such as liposarcoma. Thus, fine-needle aspiration cytology of the tumor was performed, but no diagnosis of malignant cells was obtained. Further examinations were conducted to address concerns related to her menstrual pain, and abdominal magnetic resonance imaging revealed bilateral ovarian cysts in the lower abdomen. Under general anesthesia, the patient underwent neck mass extirpation and bilateral ovarian cystectomy; pathological examination of the cysts resulted in a diagnosis of ovarian endometriotic cysts. During the surgery, the cervical mass was well demarcated and did not adhere to the surrounding tissues. The postoperative course was uneventful. The gross pathology report showed that the neck mass measured 3.0 × 2.5 × 2.0 cm. The cut surface of the specimen was heterogeneous, solid, whitish, and yellowish (Fig. ). Microscopically, the tumor was composed of spindle cells, epithelial nests, and mature adipose tissue (Fig. a, b). Immunohistochemical examination revealed that both spindle cells and epithelial nests were positive for cytokeratin (CK) AE1/AE3 (Fig. ). As these histopathological findings were consistent with the features of EHT, a diagnosis of EHT was confirmed. Over a follow-up period of 30 months, this patient exhibited no evidence of recurrence. |
A 48 year-old female never-smoker initially noticed some shortness of breath and increased work of breathing in the autumn of 2013. She was evaluated at her nearby Emergency Department in early November of 2013 and treated for an upper respiratory infection and asthma exacerbation with azithromycin and a short course of prednisone. Her symptoms persisted until January of 2014 when computed tomography (CT) of the chest was unremarkable. At this time she also had associated hemoptysis and stridor. Upon evaluation by pulmonology, her symptoms were felt to be more consistent with vocal cord dysfunction as opposed to lung pathology. On January 29, 2014, she presented with acute respiratory distress and otolaryngology was consulted. A flexible laryngoscopy demonstrated a friable appearing erythematous mass inferior to the level of the true vocal cords at the posterior commissure. CT of the neck showed a mass in the left cricoid cartilage with invasion into the surrounding strap musculature (). Tracheostomy was performed simultaneously with biopsy of the mass; pathology revealed high grade osteosarcoma. Staging positron emission tomography with CT (PET/CT) showed the cricoid mass to be hypermetabolic with suspicious left level IIA and V PET avid lymph nodes (). She was evaluated for neoadjuvant chemotherapy and completed 3 cycles of cisplatin (100 mg/m2 on day 1) and doxorubicin (25 mg/m2 on days 1-3) with dose reduction of cisplatin to 80 mg/m2 on the third cycle, which finished on April 12, 2014. On April 30, 2014, a post-chemotherapy PET/CT showed interval decrease in size and FDG uptake in the left-sided cricoid mass (). The previously seen suspicious lymph nodes were no longer hypermetabolic. On May 27, 2014, she was taken to the operating room for a total laryngectomy and left thyroid lobectomy. Intraoperative frozen sections reported negative margins, however, final surgical pathology revealed residual disease at the deep tracheal margin. There was no tumor necrosis present. As demonstrated on CT, the tumor appeared to arise from the cricoid cartilage and extended beneath the subglottic mucosa focally into the skeletal muscle. On June 2, 2014, she was taken back to the operating room for re-resection of the tracheal margin, the tissue revealed no evidence of sarcoma.\nHer case was discussed at the multidisciplinary head and neck tumor board. Due to the initial positive margin, high grade pathology and poor response to chemotherapy, post-operative radiation was recommended. On August 20, 2014, she finished a course of post-operative radiation with 6000 cGy delivered in 30 fractions to the surgical bed using volumetric modulated arc therapy (VMAT). Her subsequent follow-up has consisted of clinical visits with appropriate labs and imaging of the neck every 3 months. On September 9, 2016 her most recent CT scan showed stable postsurgical changes of the neck without evidence of recurrent or residual neoplastic disease. Her CT scans of the chest have also remained unremarkable.\nThe first case of laryngeal osteosarcoma was described by Jackson & Jackson in 1942. It has proved to be an exceedingly rare diagnosis with only 33 cases reported to date. It predominantly affects males with only 3 of the 33 reported cases in females (9%). Due to the low incidence, risk factors are extrapolated from other sarcomas of the head and neck region, as well as more common presentations of osteosarcomas. Kassir et al. proposed that skeletal Paget’s disease, fibrous dysplasia, retinoblastoma, and previous radiation were possible risk factors. There have been 3 cases (9%) of laryngeal osteosarcomas that developed after radiation to the neck for previous nasopharyngeal cancer, squamous cell carcinoma of the supraglottic larynx, and carcinoma in-situ of the true vocal cords. Other suggested factors include laryngeal framework ossification and dedifferentiated chondrosarcomas. Though smoking and alcohol are well established factors in carcinosarcoma/sarcomatoid carcinomas of the larynx, the reporting of these two factors are incomplete among case reports in osteosarcoma thus any association is currently inconclusive. Patients tend to present after the 6th decade of life, with a median age at diagnosis of 61 years and a reported age range of 38-84 years. Of the 33 published cases to date, 29 were at least 50 years old with age not provided in a single reported case.\nAs would be expected for malignancies originating in the larynx, dyspnea and hoarseness are common presentations. Of the 33 reported cases, 15 (45%) reported dyspnea at presentation and 20 (61%) reported hoarseness with an additional 5 (15%) reporting general dysphonia not specified as hoarse voice. Other reported presenting symptoms include dysphagia in 5 (15%), odynophagia in 4 (12%), stridor in 3 (9%), painless mass in 2 (6%), hemoptysis in 2 (6%), acute airway obstruction in 2 (6%), cough in 1 (3%), otalgia in 1 (3%), and globus sensation in 1 (3%) ().\nOn physical exam, all of the patients with reported laryngoscopies (direct or indirect) had abnormal findings. The majority of the tumors arose from the true vocal cords (13 patients, 39%). The anterior commissure and thyroid cartilage were the next most common sites with 4 cases (12%) each. Two cases (6%) reported tumor arising from the post cricoid space and two from the cricoid. The remaining 8 (24%) were not otherwise specified (). CT scans showed infiltrating masses often with calcifications.\nOnce tissue is obtained, the histologic diagnosis continues to be elusive. Sampling errors can increase the false negative rate. Further, fixation, antibody characterization and antigen retrieval can also misconstrue false positive or false negative immunohistochemical findings. Pathologists must also sufficiently identify malignant osteoid production and exclude epithelial differentiation to preclude the diagnosis of carcinosarcomas. Additionally, multiple histologic subtypes have been reported with osteoblastic osteosarcoma being the most common but with fibroblastic, chondroid, telengectatic, small cell and well-differentiated variants also reported. Due to location, presentation, and appearance both on imaging and histologically, the differential for this diagnosis may include spindle cell sarcoma, carcinosarcoma, metastases from a primary sarcoma, and other malignant tumors with osseous metaplasia.\nThe exceedingly low incidence of the tumor precludes strong evidence-based decision making. Therapies have been extrapolated both from principles of laryngeal cancer as well as osteosarcoma such that surgery maintains its principle role for curative treatment. Thirty of the 33 patients reported underwent some form of surgery comprising of 24 total laryngectomies, 4 partial laryngectomies and 2 local excisions (Supplementary Table S1). Three patients underwent primary radiation therapy in lieu of surgical intervention despite the fact that osteosarcomas are generally thought to be radioresistant. Additionally, 7 patients had postoperative radiation and 6 had chemotherapy incorporated into their primary treatment. In total, ten patients received salvage therapy with 2 receiving local excision for skin-only recurrences, 5 receiving salvage radiation therapy, and 4 receiving chemotherapy after recurrence.\nRecurrence free and overall survivals appear to be mixed in patients diagnosed with osteosarcoma of the larynx ( and Supplementary Table S1). Of the 33 published cases, 12 patients (36%) had not succumbed to their disease at the time of last follow-up which ranged from 4 to 96 months following diagnosis. Further, durable disease-free survival after initial treatment was achieved in 9 reported patients (28%) with follow-up again ranging from 4 to 96 months. Of note, a single patient died from unrelated causes at 12 months following diagnosis and 2 additional patients did not have any reported post-treatment follow-up. Importantly, all patients who remained recurrence free and/or alive at the time of last follow-up underwent surgical resection as the primary mode of therapy with 9 undergoing a total laryngectomy, 2 undergoing partial laryngectomy, and 1 receiving local excision only. Of the patients who recurred, the majority (13 patients, 39%) failed distantly with local recurrence in 4 (12%) and regional recurrence in 7 (21%). Regional failure consisted of recurrence in the soft-tissues of the neck either with or without associated cervical lymph node involvement. Two reported cases were noted to have recurrence without specification regarding type or location. Notably, recurrence both locoregionally and distantly tend to occur early, with the majority of patients failing within 12 months of initial diagnosis with median times to recurrence being 5 months and 12 months, respectively (Figure 3a-b). Of the four patients who recurred locally, all were initially treated with surgical resection with 2 receiving post-op radiation therapy and one receiving chemotherapy as part of their initial treatments. Upon local recurrence, the two patients who received prior radiation therapy were treated with salvage surgery with or without chemotherapy and remained without disease recurrence at 60 months while the remaining 2 patients were treated with salvage radiation with or without further resection and survived 14 and 3 months, respectively. Regional recurrence in the neck was rarely seen without concurrent uncontrolled local or distant failure as well, with information regarding salvage therapy being generally sparse. Almost invariably, patients with regional failure ultimately died from their disease though 2 of the 7 reported cases did not provide follow-up information following disease recurrence.\nDistant failure appears most commonly in the lungs (present in 9 cases reported), followed by bone (2 cases) and mediastinum (1 case). In 2 cases, location of distant failure was not reported.\nPatient outcomes based on treatment were notably heterogeneous as well (Table 3). Of the 22 patients who received surgery alone as initial treatment for their disease, 3 (13.6%) failed locally, 6 (27.3%) failed regionally, and 10 (45.5%) failed distantly while 8 (36.4%) remained disease-free at the time of last follow-up. As would be expected, total laryngectomy was associated with less local failure when compared to smaller surgeries with 1 patient (5.6%) failing locally following total laryngectomy only while 2 of the reported 4 patients who underwent local excision or subtotal laryngectomy alone suffered local relapse. Of the 6 patients treated with a combination of surgical resection and radiation therapy, 4 (66.7%) remained disease-free at the time of last follow-up with a single patient recurring locally and another recurring distantly. Only one reported patient was treated initially with a combination of surgical resection and chemotherapy and was noted to suffer early distant failure at 3 months postresection, precluding further assessment of durable local response with this combination. Interestingly, upfront trimodality therapy did not appear to further improve patient outcomes in the three reported cases, with one patient failing locally, one failing distantly, and the currently reported patient remaining without recurrence at 25 months following initial diagnosis. |
A 36-year-old woman presented with epigastric pain and anemia. Gastrointestinal endoscopy revealed an elevated mucosal lesion with linear ulceration in the anterior wall of the gastric antrum, located very close to the pylorus (Fig. a). A definitive diagnosis could not be obtained, even with an incisional biopsy. She was referred to our hospital for further investigation and treatment.\nThe patient’s medical history and family history were noncontributory. Laboratory evaluation, including tumor marker levels, showed results within the normal range except for the presence of anemia. Endoscopic ultrasonography showed a heterogeneous echoic tumor in the submucosal layer not involving the muscularis propria (Fig. ). Computed tomography (CT) showed a nodular soft tissue mass in the gastric antrum, with no apparent metastatic lesions. There was no significant uptake of 18F-fluorodeoxyglucose into the tumor on a positron emission tomography/CT scan (Fig. , ).\nThe results were inconclusive for excluding malignant potential of the tumor. Additionally, resection with ESD technique was difficult to perform, given the high risk of perforation during the procedure. Under the presumption that the tumor was a GIST, schwannoma, or other types of submucosal tumors, we sought to achieve a definitive diagnosis by radical resection for total biopsy with minimal margins. Among the various surgical procedures, we chose LECS, with more than usual caution on the prevention of gastric juice leakage into the intraperitoneal cavity (Fig. , ). LECS was performed as described by Hiki et al. []. Briefly, it is executed in three main parts. First, the endoscopist marks the smallest negative margin around the tumor using the ESD technique. Second, the submucosal incision is extended toward the serosa passing through the muscularis propria, resulting in a small artificial perforation. Finally, a surgeon resects the tumor laparoscopically with the guidance of the endoscope and surgical marks. The defect in the gastric wall is then closed either with the continuous hand-sewn technique or using linear staples. We chose the continuous hand-sewn technique, because a portion of the suture line was on the pylorus.\nThe resected specimen appeared as a gray white solid tumor, measuring 25 × 22 × 20 mm (Fig. , ), with negative margins. The tumor was located in the lamina propria mucosae and protruded into the muscularis propria, showing no necrosis or lymphovascular invasion. Histopathological examination revealed partial dense collagenous matrices and networks of fine capillary-caliber blood vessels, showing infiltration of lymphocytes, plasma cells, and mast cells. The tumor demonstrated lobular or fused nodular growth of spindle cells without atypical cytology, with abundant alcian blue-positive myxoid extracellular matrix (Fig. , ).\nAfter the confirmation of the diagnosis and the decision that additional treatments were unnecessary, the patient was discharged on postoperative day 8 with no complications, such as delayed gastric emptying or outlet obstruction. She has been followed in the outpatient clinic and shown no signs of recurrence or metastasis. No distortion of the stomach was evident on endoscopy (Fig. ). |
The patient was a 3-year 7-month-old female referred by her speech-language pathologist, presenting with oromyofascial dysfunction characterized by speech sound production errors, difficulty swallowing, open mouth breathing, and noisy breathing during sleep. With respect to sleep, there were reports of difficulties going to sleep, waking up two to three times per night to drink water, getting up to go to the bathroom, open mouth breathing while asleep (), snoring during sleep, and sweating more than usual during sleep. She experienced wheezing that was associated with asthma, which was treated with an Albuterol sulfate inhaler. There were reports of difficulty with effective chewing. In addition, the patient would eat around 50% of her meals, before refusing the rest. She experienced chronic cough and recurrent upper respiratory tract infections.\nPhysical examination () of the patient showed her to be well developed, well nourished, and to appear the stated age. The patient was alert, oriented, able to communicate, and respond appropriately to questions. During the nasal examination, the nose had no external deformity. The nasal septum was straight, and the inferior turbinates were grade 2 bilaterally. There were class 3 dental occlusion and a class 3 facial-skeletal relationship characteristic of anterior-posterior maxillary deficiency. Oropharyngeal examination showed grade 3 modified Mallampati tongue position and grade 2 tonsils. The patient was found to have a restrictive class 2 upper labial frenulum with tethering of the upper lip () and restrictive grade 4 lingual frenulum [] ().\nBased on the patient history and the physical examination, the assessment revealed that the patient had functional and structural mouth breathing due to open mouth posture and low tongue posture in the setting of restrictive labial and lingual frenulum. The risks and benefits of lingual and labial frenuloplasty to facilitate lip closure and proper tongue resting posture were discussed with the parents and included, but were not limited to pain, inflammation, bleeding, scarring, need for revision surgery, and failure for significant improvement. An informed consent document was signed by the parents.\nThe maxillary labial frenuloplasty was performed under general anesthesia. Local anesthesia was achieved by applying 1 cc of 0.25% Marcaine with 1 : 200000 epinephrine to the maxillary labial frenulum via a 27-gauge needle. Pressure was applied lateral to the frenulum to locate the point of maximum tension. The maxillary labial frenulum was incised at the base of attachment with the use of sharp scissors. A V-to-Y lip closure was performed with a 4-0 chromic suture applied in a simple interrupted fashion ().\nThe lingual frenuloplasty procedure was then performed. A 2-0 silk suture was applied to the tip of the tongue as a retraction stitch. Local anesthesia was achieved by applying 1 cc of 0.25% Marcaine with 1 : 200000 epinephrine to the lingual frenulum via a 27-gauge needle. The tongue was retracted in the anteroposterior direction extending to the roof of the mouth and maxillary central incisors. Tension was applied to the floor of the mouth to protect the floor of mouth salivary glands, as well as Wharton's duct. A hemostat was used to clamp the restrictive lingual frenulum 5 mm above the attachments of the sublingual gland duct. The fibrous band was gently excised with the use of iris scissors. The underlying myofascial fibers of the genioglossus muscle were dissected further, with blunt cotton tips and sharp iris scissors being used to release the muscle from the overlying mucosa. The dissection was continued until there was adequate improvement to the tongue range of motion such that the tongue could extend up towards the maxillary central incisors in maximal mouth opening position. Simple interrupted sutures were used to close the diamond-shaped defect into a vertical line, as a means to lengthen the ventral tongue, with 4-0 chromic suture applied in a simple interrupted fashion. In total, the tongue was released from a grade 4 restricted range of motion to a grade 1 range of motion (). All wounds were hemostatic at the completion of the procedure. The patient was gently awoken from anesthesia and taken to recovery in stable condition.\nThe patient returned to the clinic four days after the procedure. The wound sites were healing appropriately, and there were no postoperative complications observed. The patient's mother reported that within the first day of returning home, the patient's issues with chewing had improved significantly, and she was more interested in eating different foods. In addition, her appetite appeared to have increased, and the patient would complete her entire meal before asking for more food (as compared to having only eaten around 50% before treatment). By the fourth day after surgery, the patient exhibited closed-mouth nasal breathing while asleep (). There were no longer any observed events of snoring and/or noisy breathing. The mother reported that the patient had remained compliant with myofunctional and speech therapy.\nThe patient returned for a 2-month postoperative visit. During the examination at this visit, no scar tissue was observed, and the wound sites had closed. Grade 1 tongue range of motion was observed ().\nThe patient's family wrote in a letter to the clinic approximately six months after the procedure, mentioning that the patient was doing very well with no complications. In addition, the patient had been reported to have completely stopped mouth breathing and snoring while asleep. However, the patient was reported to still have occasional episodes of cold and cough, as well as one episode of asthma exacerbation. Finally, the patient was reported by her family to have made progress with myofunctional and speech therapy, but the goals of eliminating the tongue thrust, achieving proper resting posture, and improving speech sound production errors were not met due to early discontinuation of treatment. |
A 57-year-old Caucasian woman was originally diagnosed with invasive ductal carcinoma in her right breast in February 2011 (ER-positive, PR-positive and HER2-negative). She was also diagnosed as carrying a mutation of BRCA 2. The patient underwent bilateral mastectomy and right-sided sentinel lymph node biopsy with 1 of 6 lymph nodes being found to be positive for metastasis. Unfortunately, she declined adjuvant chemotherapy and radiation recommended by her treating physicians, but only received adjuvant hormonal therapy with letrozole. In February 2012, she presented with renal failure and severe bony pain, and was found to have hypercalcemia with extensive osseous metastasis. CT-guided biopsy of one of the pelvic lesions revealed metastatic adenocarcinoma, consistent with her ER-positive primary breast cancer. The patient received aggressive hydration and denosumab for hypercalcemia. She also received radiation to her sacrum and bilateral sacroiliac joints to palliate her pain. The patient was enrolled in a clinical trial, receiving hormonal therapy with the combination of tamoxifen and metformin. Her monthly denosumab injection for bone metastasis was also continued. Unfortunately, her disease continued to progress, and she was found to have extensive lymphadenopathy involving the cervical, mediastinal and pelvic area. In January 2013, the patient was transferred to our center.\nAs breast cancer in BRCA mutation carriers has been previously shown to respond to platinum-based chemotherapy [], treatment with single-agent carboplatin (AUC 5) was initiated. Three days after starting carboplatin, the patient developed a severe headache and projectile vomiting. An MRI of her brain revealed a large, dura-based, contrast-enhancing extra-axial mass, approximately 3.3 × 4.3 cm in size, causing severe vasogenic edema in the right frontotemporal region, resulting in a significant midline shift. She was seen by a neurosurgeon, and an emergent decompressive craniotomy was performed. The pathology of the resected mass was consistent with metastatic breast cancer. Postoperative brain MRI showed marked improvement, but unfortunately it also demonstrated some small dura-based masses over the left cerebral hemispheres. The patient recovered quickly and subsequently received whole-brain radiation therapy to treat dural metastases. Despite the delay of systemic therapy for almost 2 months due to craniotomy and radiation, her extra-cranial disease responded well radiographically to the first dose of carboplatin, and thus this agent was continued until June 2013.\nAt this time, PET-CT showed a progression of the disease in the cervical, mediastinal and retroperitoneal lymph nodes. Carboplatin was discontinued and PLD was initiated as a second-line chemotherapy. For the first cycle, a total of 85 mg (40 mg/m2) of liposomal doxorubicin in 250 ml of D5W (5% glucose solution) was prescribed with the same premedications used for the prior carboplatin. PLD was infused at a rate of 1 mg/min for the first 20 min. As no infusion-related adverse effects were observed, the rate was increased to 1.6 mg/min in order to complete the infusion over 1 hour according to our standard protocol. Twenty minutes later (approximately 50 mg PLD in total was given), the patient was noted to develop confusion. She had some tangential thoughts, started to make nonsensical comments to the people around her and began to tell stories from the past. The infusion was held and she was observed for 30 min, but her symptoms did not improve. An on-call physician was contacted to assess her. She was found to be alert and oriented and also able to answer questions appropriately. There were no neurological deficits. As the patient had an MRI earlier that day, no further imaging studies were performed. The infusion was discontinued due to the mental status change, and the patient was observed closely. Her symptoms persisted for 18 h and then resolved spontaneously. The patient's acute transient encephalopathy was deemed secondary to PLD. When she returned for the second cycle of chemotherapy, she was not rechallenged with PLD; instead, she was being administered conventional doxorubicin. This agent was tolerated well without any mental status change for another 5 months. Unfortunately, the patient developed a disease progression, requiring further palliative therapy with an eventual transfer to a hospice service, where she died 2 months later. |
A 74-year-old woman with no medical history presented to the emergency department with postprandial unrelenting chest pain radiating from the right breast to the right upper abdominal quadrant and accompanied by nausea. Physical examination was significant for a tender mass palpated in the right upper and lower quadrants. Right upper quadrant ultrasound displayed choledocholithiasis with common bile duct dilation at 1.1 cm, unchanged from 1 year earlier, and sludge in the gallbladder without cholecystitis. Vitals were within normal limits with the exception of raised blood pressure of 149/67 mm Hg, likely secondary to pain. Endoscopic ultrasonography (EUS) examination confirmed the choledocholithiasis, sludge, common bile duct dilation, and thickened gallbladder wall. Incidentally, it also revealed a type B dissection of the thoracic aorta and aortic arch (Figure ).\nAn aortic dissection (AD) occurs secondary to injury to the intima and media layers of the aorta. Location dictates categorization, with type A AD occurring in the ascending aorta, while type B AD occurring in the descending portion. Traditionally, computed tomographies with contrast dye, transesophageal echocardiogram, and/or magnetic resonance images are commonly used to diagnose AD. However, when patients present asymptomatically or atypically or present with a predominant gastrointestinal complaint, a comprehensive EUS examination that includes cardiac structures could provide an alternative method of early detection and treatment of cardiac diseases. The endoscope is introduced into the esophagus similarly to a transthoracic echocardiogram and scans from 120° to 180° and can be flexed anteriorly for detailed imaging. Its limitations are a poor visualization of right-sided cardiac structures, no retroflexion or lateral flexion options, detection of low flow rates rather than the high-velocity cardiac flow rates, and have less than half the frame rate of a transesophageal echocardiogram. However, it allows for a thorough and precise visualization of the aortic valve, the mitral valve, ascending and descending aorta, pericardium, left atrial appendage, and interatrial septum because of these structures' proximity to the esophagus. In this manner, we hope to widen the utility of an EUS to capture critical findings that would be otherwise missed. |
A 4-year-old boy presented to the emergency department with complaints of abdominal distension and pain for five days. He had a history of bilious vomiting with worms in the vomitus for past four days. There was a history of constipation for four days. The boy did not have fever or trauma to the abdomen. He was from a rural area in India, with frequent day to day contact with cattle. His parents were farmers by occupation and were illiterates. He weighed 14 kg and never had a previous history of passing worms in stools. He had a history of pica. There was no history of loss of appetite until recently for past one week. He was managed conservatively in another hospital for three days and referred to us for further management. On examination, the child was febrile, pulse rate 120 per minute, and blood pressure: 90/60 mm Hg. The abdomen was distended with few visible dilated bowel loops. On palpation, there was guarding and rigidity in the centre of the abdomen; no organomegaly. Bowel sounds were sluggish. Digital rectal examination revealed an empty rectum.\nBlood investigations were as follows: Haemoglobin 10 g/dl, total counts 20,000/mm3; differential count: Neutrophils 76%, lymphocytes 16%, eosinophils 10%. Renal function tests were normal, Widal test result was negative and blood culture was reported as “no growth”. Chest X-ray erect showed no free air under the diaphragm. Abdominal X-ray revealed dilated gas filled small bowel loops. In view of abdominal signs and hypotension, written informed consent was obtained from the child’s parents and the boy underwent emergency laparotomy after adequate resuscitation.\nOn laparotomy, the entire small bowel was dilated and filled with worms. Distal ileal volvulus () was present with a single 1 cm sized perforation in the antimesenteric border of the ileum and a worm was seen protruding out through the perforation (). The rest of the bowel was healthy. As the ileum containing the bolus of worms was thinned out, an enterotomy was performed to extract the worms. After removing all the worms (). through the enterotomy, resection of the ileum with ileostomy was done. Postoperatively, the patient was started on the anti-helminthic drug mebendazole and had an uneventful recovery. Follow-up stool examination revealed no Ascaris eggs or worms. The ileostomy was closed at a later date. |
A 57-year-old man had abnormal X-ray findings during routine medical checkup and an enhanced computed tomography (CT) showed right aortic arch, Kommerell diverticulum (KD), and aberrant left subclavian artery (ALSA). The maximum diameter of KD was 38 mm, and the aneurysm of aorta was 63 mm (). CT revealed a 50% stenosis of the orifice of ALSA. Cerebral magnetic resonance imaging angiography showed hypoplasty of left vertebral artery. Operation was indicated because the diameter was critical, despite the fact that the patient had no symptoms due to KD.\nAfter consideration of using 3D-CT imaging with the ribs (), it was decided to go for a posterolateral thoracotomy approach through a fourth inters costal incision.\nAll the aneurysm repairs were performed using hypothermic cardiopulmonary bypass and an interval of hypothermic circulatory arrest. Cardiopulmonary bypass was established with descending aortic cannulation and bicaval drainage. The left heart was vented through the right upper pulmonary vein. The right phrenic, vagus, and recurrent laryngeal nerves were identified and protected. After establishing circulatory arrest, the aorta was incised and transected proximally just distal to the orifice of the right subclavian artery. Descending aorta with KD was replaced using one branched graft for the reconstruction of ALSA. However ALSA was not able to be reconstructed anatomically and ligated because ALSA was too deep and difficult to make anastomosis. Rewarming was initiated after completion of the proximal graft to aorta anastomosis. Distal graft to aorta anastomosis was performed under descending aortic clamping and blood supplying from proximal anastomosed graft and descending aorta.\nAfter operation, there were no symptoms of an ischemia of the left arm. The systolic blood pressure of the right arm and the left arm was 110 mmHg and 70 mmHg, respectively. Postoperative CT revealed enhanced ALSA clearly without delay, which was supplied with blood from many branches through the narrow and invisible collateral arteries (). |
A 36-year-old female patient without a past medical history was referred to a multidisciplinary pain clinic at our center for severe intractable pain in her right arm, wrist, and hand. Progressive pain developed in our case one month after orthopedic surgery on her forearm. She suffered a consistent severe stabbing and shooting pain, hyperalgesia, and allodynia with cramping and numbness in her right upper limb which interfered with her sleep. On physical examination, severe intractable pain, allodynia, hyperalgesia, skin temperature and color asymmetry, edema, and trophic changes in the nails of the right upper limb were identified. Full flexion, extension, and abduction at the right shoulder, elbow, and wrist were restricted. Neurological examination was normal. Magnetic resonance imaging, X-ray, laboratory blood tests, and work-up identified no pathology, thus supporting the diagnosis of CRPS. Thrombosis was ruled out through a color Doppler ultrasound. A nerve conduction study showed no significant difference between the right and left upper limbs. The patient's pain score was 10 out of 10 on the numerical rating scale (NRS), where 0 is the total absence of pain and 10 is the worst pain imaginable. Her quality of life had clearly deteriorated. CRPS type 1 was diagnosed according to the International Association for the Study of Pain (IASP) criteria. The patient took tramadol HCL 100 mg, celecoxib 200 mg, pregabalin 75 mg, and amitriptyline 25 mg, but they had no effect. She received a stellate ganglion block (SGB) with 2 ml bupivacaine HCL 0.5% and 2 ml of lidocaine 2% and reported a significant decrease in pain (NRS improved from 10 to 3) for 6 days after block. The patient tolerated this procedure well, but her pain returned after one week. As before, the pain was severe and constant and interfered with her sleep. The patient then underwent pulsed radiofrequency (PRF) of stellate ganglion one month later with RF current for 20 milliseconds at 2 Hertz and maximum electrode temperature below 42°C for a total duration of 120 seconds, but her pain again returned after one week and was severe, constant, and interfered with sleep. The patient underwent a brachial plexus block through the axillary block approach, but it was ineffective. Six months later, she received authorization for a Bier block, which she underwent with 20 ml lidocaine 0.5% and 8 mg dexamethasone. This procedure was well tolerated, and for six months after the procedure, the patient's pain decreased more than 80% with her pain score reduced to 3 on the NRS and a near-normal performance status for daily activities. |
An elderly female patient, aged 73-year-old, was admitted to our hospital for unexplained acute abdominal pain and bloating after the occurrence of vomiting. Upon admission, the patient was observed to have an abdominal bulge and total abdominal tenderness accompanied by rebound pain and muscle tension. The patient had a medical history of total colectomy in combination with an ileal pouch-anal anastomosis (IPAA) for the treatment of functional chronic constipation or chronic idiopathic constipation (CIC) 2 years before hospital admission.\nGeneral physical examinations were as follows: heart rate: 120 bpm; blood pressure: 85/50 mmHg; Laboratory tests revealed a white blood cell (WBC) count of 17 × 109 / L, and lactic acid (LA) level of 6.3 mmol / L; Abdominal enhanced computed tomography (CT) showed dilation, as well as gas and liquid accumulation in the distal portion of the small intestine, ventral to the distorted stomach. It was observed that a segment of the small intestine was prolapsed via the defected lesser omentum, passing the retrogastric lesser curvature to enter the ventral part of the stomach. CT images in the abdomen also revealed a hypodensity in some fragments of the small intestine, liquid accumulation around the liver and spleen, as well as pelvic effusion (Fig. ).\nBased upon the abdominal CT findings, the patient was diagnosed with having a lesser omental hernia complicated with intestinal obstruction, acute diffuse peritonitis, and septic shock.\nThe patient was immediately treated with an emergency laparotomy. During the procedure, ascites, of approximately 1500 ml liquid with blood, were observed. Notably, a fragment of the small intestine protruded from the lesser retrogastric curvature of the stomach into a defected lesser omentum (Fig. a) and appeared necrotic and black. The defected omentum was approximately 3 × 4 cm in size (Fig. b), with the necrotic fragment of the small intestine measuring approximately 200-cm in length. The necrotic portion of the small intestine was resected, anastomosis was performed, and the defected lesser omentum was closed. The patient was subsequently transferred to the intensive care unit (ICU). The tracheal intubation was removed 2 days after the operation, and the patient was transferred to a local hospital for further treatment.\nThe risks and benefits of the surgical procedures were explained to the patient, and written informed consent was obtained. |
A 25-year-old man was admitted with trichoblastoma in the nuchal area with frequent recurrences since birth. The mass gradually increased to walnut-size, and the first excision surgery was performed in a local medical center (LMC) 3 years ago. The mass recurred at the same location and the patient underwent a second excision surgery at the LMC and was diagnosed with trichoblastoma after biopsy. After excision, the mass recurred with an increased size, and a third excision was performed in the LMC. However, when the mass recurred at the same site for the fourth time, the patient was transferred to our department (). The patient’s history, family history, and results of the laboratory study were unremarkable. A palpable lymph node was found in both, the internal jugular and popliteal cervical areas, and ultrasonography-guided aspiration was performed, but no metastasis was observed. The pathological diagnosis after the third excision surgery in the LMC was adnexal tumor with hair follicle differentiation with nonmalignant potential with involvement of the base. Immunohistochemistry results were positive for cytokeratin (+), p53 (−), and Ki-67 (1+). For accurate diagnosis, we requested for the specimen to be examined again in our pathology department. The specimen was identified as a skin adnexal carcinoma, of hair follicle origin and lowgrade malignancy (). The preoperative neck magnetic resonance image (MRI) revealed lobulated soft tissue lesions involving superficial fascia and infiltrating into both proximal trapezius muscles (). Positron emission tomography-computed tomography (PET-CT) revealed three hypermetabolic mass lesions about 4 cm in size on the subcutaneous layer of the posterior neck, and no distant metastases was observed. In the plastic surgery department, wide excision including a 1.5-cm safe margin and reconstruction with a free anterolateral thigh flap were performed. After extensive excision, histological examination revealed skin adnexal carcinoma, originating from the hair follicles, and low granular malignancy, consistent with trichoblastic carcinoma, with clear resection margin (). Even though partial flap necrosis had occurred, it was treated with debridement and split thickness skin graft. Neck MRI performed at 6 months postoperatively and PET-CT at 1 year postoperatively showed no remnant mass, recurrence, or distant metastasis. There was no palpable mass 5 years postoperatively, and there was no recurrence on follow-up of 5-year neck CT and PET-CT (). |
A 60-year-old lady was referred to our center with a complaint of progressively increasing breathlessness and cough off and on since 2 months. She had no past history of respiratory complaints. Her general examination revealed a right sided breast lump, which was firm and non-mobile.\nHer treatment history revealed that she had noticed a breast lump about 10 months back, the size of which was about 2 × 2 cm. She had waited 2 months before seeking proper specialist medical care. Histopathology and immunohistochemistry examination was done by the referring center which revealed the lump to be hormone receptor negative Her-2/ neu positive ductal carcinoma in situ with node negative status. She was prescribed on neoadjuvant chemotherapy in the form of cyclophosphamide, methotrexate, and 5-flourouracil for six cycles by the previous center.\nShe had taken oral cyclophosphamide 150 mg daily for 14 days with injection methotrexate (40 mg/m2) and injection 5-flourouracil (600 mg/m2) on days 1 and 8, repeated every 28 days. She had completed six cycles by the time she presented to our center. She had been administered a total of 12.6 g cyclophosphamide, 720 mg methotrexate, and 11 gm of 5-florouracil.\nThe patient stated that she developed nail pigmentation which started proximally and spread distally over the duration of chemotherapy. Pigmentation involved all the nails of the hand and toes except the second and third toes of the right foot [Figures and ]. The pigmentation pattern was uniform in all the nails of the hands and toes except first toe of both feet where it was in form of transverse bands at presentation. The pigmentation varied from black to brown and was deeper and extensive in the finger nails compared to the toe nails. The patient had noticed progressively increasing pigmentation from the third cycle of chemotherapy onward and did not regress after chemotherapy had been stopped. There was no associated skin or mucous membrane pigmentation. The patient did not suffer significant systemic toxicity.\nA chest x-ray of the patient revealed a right-sided pleural effusion. The pleural fluid was hemorrhagic on gross examination and cytochemical analysis revealed a lymphocyte predominant nature with adenosine deaminase level of 18U/L. On microscocopic examination of the pleural fluid sediment, malignant cells with a high nucleo-cytoplasmic ratio were found. Because of the advanced nature of the disease and the exhaustive past treatment, the patient refused any further workup. She was subsequently lost to follow-up. |
A four-year-old female child with acyanotic congenital heart disease, that is, moderate ostium secundum ASD and branch pulmonary artery stenosis, since three months of age presented with classical features of facial dysmorphism such as frontal bossing, triangular facies, deep-set eyes, a pointed chin, and mild joint laxity (Figure ). Delayed development was noted with mental development more delayed than motor development along with poor speech. Ocular examination revealed posterior embryotoxon and mild pigmentary retinopathy in the right eye. Chest X-ray showed abnormal segmentation in the T5 vertebrae (butterfly presentation) (Figures , ) and past history was positive for recurrent infections and negative for neonatal cholestasis and maternal abnormalities. The above findings were highly suggestive of ALGS.\nA confirmatory cardiac CT scan was performed which revealed a moderate ASD of ostium secundum type along with hypoplastic main right and left pulmonary arteries, with a McGoon ratio of 1.65 and Nakata index of 169.36 mm2/m2 (Figure ). The common hepatic artery was seen arising from the proximal portion of the superior mesenteric artery, and a small vertical midline cleft in the body of the T5 vertebra, mainly in the posterior portion, was noted; these findings appeared to be congenital variants.\nFurther detailed evaluation and a preoperative color Doppler revealed severe bilateral branch pulmonary artery stenosis with moderate ostium secundum ASD and bidirectional shunting. Right ventricular pressure was significantly elevated with hypertrophy and dilatation of the right atrium and the right ventricle with normal biventricular function. Ultrasonography of the abdomen and pelvis reported no significant findings.\nThe patient was planned for bilateral branch pulmonary artery plasty with glutaraldehyde treated pericardial patch and direct closure of the atrial septal defect leaving a patent foramen ovale (PFO). The patient tested negative for severe acute respiratory syndrome coronavirus 2. She was operated on uneventfully and shifted to the Cardiac Surgical Intensive Care Unit on mechanical ventilation along with adrenaline and milrinone support. The patient was extubated on the third day after the surgery after 51 hours of Intensive Care Unit turnaround time. A postoperative echocardiogram color Doppler was performed one week after the surgery which revealed a severe obstruction in the left pulmonary artery flow with a peak gradient of 100 mmHg with diastolic tailing in the left pulmonary artery flow. In addition, it showed good flow in the proximal right pulmonary artery as well. Moreover, a small ostium secundum ASD with shunting from the right to left and severe right ventricle dysfunction with tricuspid annular plane systolic excursion of 5 mm were reported. Gradually, she was initiated on a liquid diet and started accepting full feeds. She was discharged in a stable condition with a healthy wound. She was also advised to follow proper wound care and hygiene and was scheduled for a follow-up. |
A 29-year-old man, a former semiprofessional handball player, had a traumatic rupture of the proximal side of the patellar tendon of the left knee in 2016 during a match. No associated disease was reported. Primary surgery was performed in another health facility through a median approach using 2 anchors for tendon repair protected by an additional ipsilateral semitendinosus graft (patellar and tibial tunnels). The patient came to our health facility following severe functional deficits after an iterative rupture without having experienced any new trauma 13 months after the initial surgery. The iterative rupture of the knee extensor mechanism was also an iatrogenic fracture of the transverse patellar tunnel (). Clinically, walking was not possible, there was a lack of active extension and hemarthrosis with pain. There were no scar problems, no signs of deep or superficial infection, and no cutaneous wound. A huge gap was clinically observed between the patella and the patellar tendon. Considering the patient's age, his preinjury sports level, and lack of active extension, the decision was made to perform a revision procedure. An artificial ligament (LARS®) and two adjustable loops, free ends of the PULLUP® BTB (SBM SAS, France), were used to enhance the patellar tendon repair.\nA preoperative lateral standard X-ray was taken of the contralateral knee at 30° of flexion to measure the Caton-Deschamps index and patellar height (). The patient was placed in a supine position under general anesthesia with a tourniquet at the proximal part of the thigh. The previous median approach was used. The patellar fracture and the site of the previous rupture were cleaned to remove fibrous tissue and hematoma. Previous anchors were left in place.\nThe first step was to place the ligament advanced reinforcement system (LARS® polyethylene terephthalate fibers 6 mm ref. L030307 ACFAR 32 CK). A new transverse tunnel was drilled in the tibia, distally to the tibial tunnel of the initial surgery, with a 5.5 mm drill, and the LARS® was inserted in the tibial tunnel. The artificial ligament was then passed through the lateral retinaculum and above the patella at the junction with the quadriceps tendon in a Pulvertaft manner and through the medial retinaculum to return to its origin (). Two longitudinal tunnels were drilled in the patella using a 2.4 mm drill. The loops of a PULLUP® BTB (the plate was removed from the device) were first passed into the patellar tendon and then into the patella through the two longitudinal tunnels using a shuttle relay. Next, the free ends were pulled down in the opposite patellar tunnels. At the proximal side of the reconstruction, the two free ends of the PULLUP® BTB were inserted into each braid to close the system (Figures and ). The distal and medial ends of the LARS® were tightened with a clamp in order to restore normal patellar height and secured with 2 staples. Then, the 2 PULLUP® BTB loops were tightened (). The previous tendon rupture was closed and reinforced with separate X-knots using absorbable sutures. The skin was closed. The knee was placed in an articulated brace with compressive ice therapy for 24 hours, and a postoperative X-ray was taken ().\nFor postoperative care, weight bearing was not allowed for 6 weeks and mobilization of the knee was immediately started between 0 and 45° for 3 weeks then from 0 to 90° from 3 to 6 weeks. No complication was reported during the postoperative period. At 3 months, the patient was pain free and could walk without the aid of crutches. He was able to resume handball practice at 6 months after a control MRI. At one-year follow-up, he was able to play handball with complete knee extension strength (compared with the contralateral side) and was able to return to a semiprofessional level. The range of motion of the knee was 0-0-130°. The MRI at 1 year showed complete healing of the patellar tendon and the bone (). |
The patient described herein was a 29-year-old woman who underwent gluteal augmentation with solid silicone implants on the border of Colombia and Venezuela in January 2016. Information from this procedure was unavailable. One week after surgery, the patient presented with a soft tissue infection with wound dehiscence and gluteal discharge; she underwent gluteal revision surgery with lavage, extraction and replacement of the gluteal implants, and antibiotic management. For 1-month, prolonged serous secretions continued and the patient received ongoing antibiotic therapy. Subsequently, her postoperative course apparently improved, and she remained asymptomatic for 8 months.\nThe patient presented for a consultation for a 3-day clinical course of progressive-onset pain in the right gluteus associated with changes in skin color and edema. There was no history of gluteal trauma (). In addition, the patient described rapidonset asymmetry of the buttocks. A physical examination confirmed that the left gluteus was flaccid and smaller than the contralateral side, while the right gluteus was larger, painful, and showed acute striae.\nNuclear magnetic resonance imaging was ordered, and showed displacement of the left gluteal implant into the right gluteal pocket, with the implants partially covered by the gluteal muscle ().\nA surgical intervention was proposed for removal of the implants and delayed reconstruction after the conditions of the soft tissue improved; however, the patient did not agree to implant removal and wished for the implants to be repositioned. Therefore, it was decided to perform reconstructive surgery, after warning the patient of the risks. The operation was started by approaching the old incision in the gluteal cleft, where there was an absence of adhesion between the skin and the pre-sacral area. We then found two undamaged 350-mL gluteal implants (Polytech) with rough surfaces. One was located above the other in the right gluteal pocket (). The left gluteal pocket had an extensive constriction, while the right pocket was enlarged. In addition, there was a large tunnel that communicated between the two pockets, and was covered by thickened capsular tissue ().\nSubsequently, capsulotomy and enlargement of the left gluteal pocket in the lateral, superior, and inferior quadrants was done, followed by plication with Vicryl 0/0 sutures of the right pocket at the upper lateral quadrant. The implants and pockets were washed with abundant irrigation of iodine solution, and the implants were repositioned in the previously formed individualized pockets, making a closure in the following four planes along the middle line of the sacrum; (1) Plane 1: formed by carving capsular tissue flaps from the roof of the pocket’s dome, as well as from the pre-sacral portion, and sutured with separate points using Vicryl 2/0; (2) Plane 2: formed by carving fatty fascial flaps from the buttocks, and sutured to the pre-sacral fascia with separate points using Vicryl 2/0; (3) Plane 3: formed by suturing the dermal plane to the deep plane with Vicryl 3/0; (4) Plane 4: skin closure on the gluteal cleft with mattress points made using Prolene 3/0.\nA drain was left in each gluteal pocket for 15 days with careful recommendations for postoperative care on an outpatient basis. The patient experienced a good postoperative course with no associated complications (), and at a 5-month follow-up examination, we observed adequate positioning of the implants without malrotation, as well as a well-defined gluteal cleft with a high-quality scar (). |
A 29-year-old woman, being artificially inseminated with twins, was hospitalized to get a Cesarean section. A Cesarean section was scheduled for her on the next day, but sudden pain and progression of the childbirth delivery forced her to the childbirth delivery room early. As she had undergone a vaginal delivery 3 years ago, vaginal delivery was induced but failed. Therefore, a decision was made to do a Cesarean section.\nAfter entering the operating room, the surgeons made the parturient woman take the lithotomy position to try a vaginal delivery again, but it was unsuccessful. Spinal anesthesia was administered to the patient since she had spent only 5 hours fasting. The surgeons put the patient in the right lateral decubitus position and disinfected the puncture part between the L3-L4 spinous processes with betadine solution. Then, the surgeons inserted 26 G Quinke, a spinal needle, into the puncture part by the midline approach method to verify whether the cerebrospinalfluid (CSF) would smoothly release. After seeing a smooth flow, the surgeons injected 10 mg of hyperbaric 0.5% bupivacaine and 20 µg of fentanyl. During the insertion of the needle and injection of the drug, the mother didn't complain of any pains or paresthesia on her back and lower limbs. Following the verification of the sensory blockade up to T6 thoracic vertebra 10 minutes after the drug injection, the surgeons began the operation for Cesarean section.\nAfter the delivery, a Hartmann solution containing 20 units of oxytocin was injected; however, the vaginal contraction was still insufficient. Therefore, the surgeons injected 0.2mg of methylergometrine into her triceps brachialis muscle and 500 ml of normal saline containing 1 mg of sulprostone into her body at the rate of 100 ml per hour. Thirty minutes after the delivery, when her blood pressure declined to 70/40 mmHg, 8mg of ephedrine was injected, and her blood pressure returned to the normal level and stayed at 110/60 mmHg afterward. For the rest of the operation time, there was nothing unusual. Following the operation, the mother was brought to the postanesthetic recovery unit to be observed for 1 hour. After the doctors found that her sensory blockade was maintained up to T12 thoracic vertebra and there was no abnormality, they decided to move the patient to her ward. Two days after the operation, the anesthesiology department was asked for a joint treatment because the patient complained of numbness and paresthesia on her left leg. Although the mother said that she had paresthesia on her left femoral region, the doctors couldn't find any dyskinesia, decline in strength, lumbago, pooling of urine and feces, or paresthesia on the perineal region.\nFurthermore, knee and ankle jerk reflexes were proven normal. The authors scanned her lumbar vertebrae with magnetic resonance imaging (MRI) to verify epidural hematoma or abscess, but nothing unusual was discovered. The authors also conducted a blood test to determine infection and inflammatory condition, and obtained the result that her white blood cell (WBC) count was 6,400/µl and erythrocyte sedimentation rate (ESR) was 13 mm/hr, both of which are normal. In addition, she didn't suffer any fever. Subsequently, the authors decided to remain on the watch without additional treatment, considering that, according to neurological and laboratory tests, the patient had little possibility of nerve injury from spinal anesthesia, epidural hematoma, and abscess. However, after the patient continuously complained of the symptom for days, needle electromyography and nerve conduction studies were conducted on her truncus and lower limbs to identify any lesions in the nerve root and peripheral nerve.\nAs a result, the sensory nerve conduction velocity (SNCV) test discovered a left lateral cutaneous nerve injury while the needle electromyography study didn't find anything unusual (). In the following pelvic compression test, the patient said that her symptom was reduced when her left pelvis was compressed. Considering her symptom, which was limited to left lateral cutaneous nerve, and the result of the pelvic compression test, the authors diagnosed her as meralgia paresthetica and prescribed nonsteroidal anti-inflammatory drugs for conservative treatment. She was allowed discharge and was traced as an outpatient for a month. According to the result of the observance after discharge, the numbness on her left femoral region disappeared without special treatment, and complete blood cell count (CBC) and ESR were also within normal levels. |
A 55-year-old incarcerated male presented to the emergency room with a two-week history of left-sided scrotal pain and swelling.\nThis patient had a history of prostate cancer and high-grade urothelial bladder cancer. His prostate cancer was diagnosed 15 years prior to the current presentation, managed with radiation, and had since remained stable. The patient's high-grade, high-risk bladder cancer was diagnosed two years prior to current presentation. At the time, the patient initially presented with hematuria. A CT urogram performed at the time revealed a nonspecific bladder mass. Biopsy of the mass confirmed urothelial carcinoma. The patient's therapy course included a transurethral resection of the bladder tumor (TURBT), intravesical mitomycin, interferon alfa-2b, and intravesical BCG therapy. He received a total of seven intravesical injections of 50 mg live BCG per injection (350 mg cumulative dose) over the course of 18 months.\nThe patient's other significant medical history included rheumatoid arthritis, for which the patient was on weekly methotrexate and daily tofacitinib treatment. Both medications were discontinued on admission.\nThe patient developed scrotal pain two weeks prior to admission. He initially presented to the emergency department with this pain. At that time, he was diagnosed with acute bacterial epididymitis and was prescribed a course of ciprofloxacin. He had no improvement in his symptoms despite this treatment. Over the subsequent days, he reported experiencing intermittent chills and night sweats. He denied any penile discharge or any history of a sexually transmitted disease.\nEvaluation of the testicle with ultrasound revealed multiple diffuse nodular areas of hypoechogenicity (Figure , ), as well as marked hypervascularity involving all of the left-sided structures (Figure , ). Routine blood and urine cultures were negative, and mycobacterial blood and urine cultures, as well as bladder biopsy, were pending as of this writing.\nAntituberculous therapy was started based on the following considerations regarding the patient's presentation. The lack of observed response to ciprofloxacin therapy decreased the likelihood of uncomplicated bacterial orchitis. We considered this patient's history of bladder cancer involving multiple BCG treatments and the increased likelihood of BCG orchitis. Possibly, his immunocompromised state secondary to the malignancy, in combination with mitomycin chemotherapy and immunomodulatory rheumatoid arthritis treatment, could have increased his susceptibility to a mycobacterial infection. Finally, as ultrasound findings were characteristic of BCG orchitis, the patient was promptly initiated on a combination therapy with levofloxacin, rifampin, isoniazid, and ethambutol. The patient showed marked improvement in pain control and testicular swelling over the following weeks. |
A 67-year-old man presented to our department with an 8-month-history of involuntary twisting and rolling movement of abdominal muscles. The patient reported having abnormal head turning movement for the past 20 years. The head movement was involuntary and disappeared during sleep. For the past 4 years he noticed abnormal twisting movement of the right shoulder and elbow. The head and right upper limb movements were not troublesome nor painful and thus he did not seek medical attention. Subsequently and for the past 8 months he started experiencing abnormal involuntary twisting and rolling movements of abdominal muscles. There was no pain or discomfort accompanying the movements. The movements were intermittent occurring throughout the day but disappeared during sleep. He reported having difficulty breathing and falling asleep due to the movements. He had no family history of movement disorders and had not been prescribed psychotropic or antidopaminergic medications. He denied having any surgery or trauma. Careful psychiatric assessment showed no evidence of depression, anxiety, and no history of neuroleptics use.\nNeurological examination revealed normal higher mental functions, cranial nerves, as well as motor and sensory functions. Blepharospasm and laterocollis to right side were noticed. Occasionally, periodic dystonic posturing was observed in the right upper limb involving the shoulder and elbow.\nIn the supine position, involuntary writhing movements of the abdominal wall muscles were seen in a wave fashion that rolled across the abdomen due to asynchronous contractions of the rectus abdominus muscle (Additional file 1: video, segment 1). The movements did not show any variation in intensity or frequency in different positions nor during inspiration and expiration.\nAll lab tests including copper, ceruloplasmin, thyroid hormones, liver and renal functions, and blood smear were unremarkable. Cranial and spinal magnetic resonance imaging (MRI) were normal. Fluoroscopic screening did not show any diaphragmatic flutter.\nNeedle electromyographic (EMG) recordings of the orbicularis oculi as well as neck, trunk, and abdominal muscles showed abnormal motor unit potential discharges, some of them tonic, varying over a wide range of frequency from 3–5 to 30–40 sec. In addition, sudden discharges from groups of motor unit potentials was seen lasting from as brief as 10–20 msec to over 3–5 sec. The discharges were arrhythmical and asynchronous in separate muscles. They were also repetitive but highly unpredictable (Fig. ).\nA diagnosis of multisegmetal dystonia with new-onset belly dancer’s dyskinesia was established. The patient was initially started on benztropine and clonazepam and the doses were built-up gradually. Follow up 2 months later did not show any improvement. Since the abdominal dyskinesia was the only bothersome condition for the patient and due to drug failure, he was selected for bilateral rectus abdominus injection with BTX under ultrasound guidance. The procedure was performed using an aseptic technique under ultrasound guidance to decrease the risk of complications such as penetration of the abdominal cavity, peritonitis, or perforation of abdominal viscera []. A total of 300 units of BTX (Botox©, Allergan, Irvine, CA) was injected in the rectus abdominus muscles bilaterally, in which 50 units/site in a total of 6 sites were administered.\nA follow up 2 weeks after the injection, tremendous improvement was reported by the patient. He was able to perform his of daily living activities. On his 1 month follow up, the patient reported complete disappearance of the abnormal abdominal movements with no side effects. His breathing difficulty and sleep pattern improved.\nThe effects of the injections lasted for 6 months, after which the patient received another set of injections of the same dose utilizing the same technique. The BTX effects in the second and third sessions lasted for about 8 months each. The patient was doing very well on his last follow up visit with no reoccurrence of the movements or adverse reactions to the injection. |
Patient 10 (Tables and ), a 76-year-old female, was referred to the department of neurology in July 2012 because of progressing cognitive decline over the last 12 months, loss of weight, nausea, gait disturbance and tremor. She was seen on May 2011 for the first time by a neurologist with a 3-month history of dull holocephalic headache who ordered a cranial magnetic resonance imaging (MRI) and diagnosed a tension-type headache and a depressive disorder. Treatment with an antidepressant (duloxetine) was started. The patient experienced no improvement and a second examination by another neurologist was undertaken 2 months later. Again no focal neurological signs could be detected. Due to the weight loss, an occult neoplasm was suspected but not detected during an extensive inpatient investigation at a medical department during February 2012; however, the MRI showed bilateral white matter lesions (WML) and an old lacunar lesion located at the left striatum, the latter was not seen in the previous MRI from May 2011. Since the patient also suffered from mild hypertension, vascular encephalopathy was thought to be the cause of the progressive cognitive decline. Extensive neurocognitive testing was carried out in a rehabilitation centre in May 2012 and disclosed a severe decline of attention, memory and executive functions corresponding to subcortical dementia (Fig. ). When the patient was seen for a further diagnostic work-up at the SMZ-Ost-Donauspital in July 2012, the weight was 47 kg and a weight loss of 20 kg was reported over the past year. The gait was insecure with postural instability and with a tendency to fall when turning around. Frontal signs were positive, the voice was quiet, the tonus was mildly elevated and showed a slight hesitancy (“Gegenhalten”), tendon reflexes were brisk, paresis and pyramidal signs missing. There were no signs of ataxia, but a mild bradykinesia. Action tremor was more distinct than a mild resting tremor. Again, neurocognitive testing and gait disturbances were consistent with subcortical dementia (Figs. and ). Regarding the mild signs of parkinsonism, dementia with Lewy bodies (DLB) was also suspected but excluded by a dopamine transporter (DAT) scan. Fluorodeoxyglucose positron emission tomography (FDG-PET) demonstrated hypometabolism in the left striatum and in the left frontotemporal cortex (Fig. ). Cerebrospinal fluid (CSF) showed signs of a chronic lymphocytic inflammation. The CSF markers for dementia, total tau protein and phosphor-tau were within the normal range, while beta-amyloid 1-42 and the Innotest-amyloid-tau index (IATI) were found to be below the reference values (beta-amyloid 1-42: 290 pg/ml, reference value > 500 pg/ml; IATI 0.6, reference values > 1). Finally, LNB was diagnosed when further CSF examinations disclosed a highly elevated Bb-specific-AI indicating local intrathecal Bb-specific antibody synthesis (Table ). The patient was treated with 2 g ceftriaxone daily for 3 weeks.\nNeurological symptoms and impaired cognitive functions, although persistent for a year, recovered rapidly within a few weeks (Figs. , and ) and so did the pathological CSF findings (Table ). A follow-up FDG-PET examination showed the left frontotemporal hypometabolism in remission, while this was not the case for the cystic lacunar lesion in the left striatum. A new and clinically silent small right thalamic lesion was detected that was not present in the pretreatment MRI (Fig. ). The Consortium to Establish a Registry for Alzheimer’s Disease (CERAD) test battery at the last follow-up in April 2014 scored within the age-dependent normal range with the exception of verbal learning and semantic verbal fluency (Fig. ). In a telephone call in February 2018 at the age of 82 years, the patient reported no gait problems or cognitive impairment and had just returned from a trip to Cuba. |
A 64-year-old male patient was referred to the emergency department of our hospital, with a four-day history of spontaneous abdominal pain radiating on the posterior aspect of the left thigh. He reported no trauma history, but during the past few hours, pain had severely deteriorated. In regards to his past medical history, the patient was on treatment for hypertension and atrial fibrillation. In particular, he was on an angiotensin II receptor blocker and on warfarin as an oral anticoagulant.\nThe patient was afebrile, and his vital signs were within normal values (blood pressure 115/78 mmHg, heart rate 82 beats/min, SpO2 99%). Physical examination revealed diffuse abdominal pain, radiating on the posterior aspect of the left thigh. Peripheral vascular examination was within normal values on both lower limbs, with palpable pulses and capillary refill time of less than two seconds. Neurological condition, on the other hand, was impaired on the left lower limb, and objective deficiencies were obvious. Examination revealed reduced strength of the left lower limb. The patient was unable to straight leg raise, to flex the hip or the knee, and had developed hypoesthesia on the anterior aspect of the thigh. The patella reflex was absent on the affected side.\nLaboratory data revealed prolonged prothrombin time with an international normalized ratio (INR) level of 4.5, and a haematocrit and haemoglobin decrease of 33.1% and 10.7 g/l, respectively. The rest of the full laboratory analysis was within normal values. Imaging studies included abdominal ultrasound scan (US), lumbar spine, and pelvis CT scans. Lumbar spine pathology was excluded, and a large retroperitoneal haematoma was unveiled within and around the left iliopsoas muscle (35 cm x 9 cm x 6 cm) (Figure ).\nThe patient was admitted to the hospital, and warfarin anticoagulation regimen was intermitted. Prolonged INR was reversed within 48 hours with the aid of vitamin K administration. A new CT scan was performed on day 3 post-admission. No change in the size of the haematoma was revealed (Figure ). At that point, there was a consultation with the radiology department whether the haematoma could be percutaneously drained; however, the CT scan revealed a clotted formation that would not allow for successful drainage.\nThe patient was managed conservatively. Physical therapy had been initiated by day 1 post-admission. The patient was discharged after six days of hospitalization and after pain management had been established. Nerve conduction studies were performed three weeks later and revealed left femoral nerve palsy. A new CT scan was performed two months post-discharge and unveiled absorption and a significantly reduced size of the haematoma (Figure ). The patient demonstrated gradual improvement and recovered after eight months of physiotherapy and rehabilitation. This included almost complete motor and complete sensory function restoration of the left femoral nerve. |
A 73-year-old male, height 172 cm and weight 75 kg, was referred to our institution after having been seen 3 months previously due to worsening lower back pain which radiated to the left buttock and thigh as well as claudication that had lasted three months. His lower back pain had been treated with caudal blocks for the last 3 years. A lumbar spine radiograph revealed degenerative scoliosis and narrowing of multiple intervertebral disc spaces. A magnetic resonance imaging (MRI) scan demonstrated marked lumbar 3-4 (L3-4) intervertebral disc extrusion, lumbar spinal stenosis, and an L3 compression fracture. He was treated with one L3-4 epidural block, medial branch blocks, and received nonsteroidal anti-inflammatory drugs. His radicular pain and claudication improved, but he complained of low back pain consistently. Therefore, a transforaminal epidural block and percutaneous epidural neuroplasty were performed. After the treatments, all symptoms improved, and only mild lower back pain which increased with walking remained. However, 2 weeks later, he was hospitalized for left leg paralysis and excruciating lower back pain that did not diminish when laying down. MRI was repeated, which identified aggravated L3-4 disc extrusion and a dilatated abdominal aortic aneurysm of nearly 6.0 cm (). Careful review of the previous MR images identified a curvilinear atherosclerotic (calcified) lateral wall of an abdominal aortic aneurysm and fusiform aneurysmal dilation of the abdominal aorta anterior to the vertebral body (). The patient was referred to the neurosurgery department of our hospital for further examination and treatment for the disc extrusion and abdominal aortic aneurysm.\nHe presented to our hospital with intractable lower back pain and pitting edema of both lower extremities. His blood pressure was 110-130/79-90 mmHg and his pulse rate was 70-80 min. Abdominal contrast enhanced computed tomography (CT) confirmed a focal lower abdominal aortic aneurysm with crescent-form mural thrombus formation, without evidence of rupture (). A sensory conduction study and motor conduction study revealed left lumbar radiculopathy mainly of the L4, L5 nerve roots, as well as hypoesthesia on the right L4 to L5 dermatomes. Muscle strength was fair plus in left L5 innervations on manual muscle testing. The range of motion and reflexes were normal. In all extremities, the arteries were readily palpable and no pulsating mass was detected on abdominal palpation. There were no other abnormal findings on the physical examination. Bloodwork showed total cholesterol 191 mg/dl, triglycerides 137 mg/dl, LDL cholesterol 137 mg/dl, HDL cholesterol 30 mg/Dl and HDL ratio 6.3. The surgeons elected to perform decompressive hemi-laminectomy and microdiscectomy on L3-L4 and transforaminal lumbar interbody fusion on L3-L4. After neurosurgery, the patient's neurologic symptoms improved except for the lower back pain. He was transferred to the thoracic surgery department and underwent endovascular aneurysm repair and aortic stent graft interposition. After surgery, his lower back pain diminished. He underwent physiotherapy for 1 month and was then discharged. |
A 26-year-old pregnant (22 weeks of gestation) female with amphetamine and marijuana use was presented to our Burn Center following a home explosion. This resulted in 60% TBSA second- and third-degree burn wounds to all extremities circumferentially, bilateral shoulders, neck, and abdomen. Most of the patient’s burns were second degree. The patient had a Glasgow Coma Scale of 15 upon arrival to the trauma bay. The patient was hypotensive and tachycardic and was intubated immediately to protect her airway during burn resuscitation. On admission the patient received an MRSA nasal swab that was negative; however, a subsequent MRSA nasal swab on hospital day 3 was positive, as shown in .\nOn physical exam, she was edematous from resuscitation and continued to exhibit normal fetal heart tones. On hospital day 4, the patient required increasing fraction of inspired oxygen and positive end-expiratory pressure support. The patient was then transitioned to volumetric diffusive respiratory (VDR). Paralytics were required for ventilator asynchrony despite sedation and pain medication as she developed acute respiratory distress syndrome (ARDS). On hospital day 5, the patient showed increasing levels of both lactate and white blood cells. The endotracheal aspiration culture grew MRSA, Enterobacter, and Escherichia coli (E. coli). Additionally, it was noted that the patient was experiencing greater than two loose bowel movements requiring a rectal tube to protect the burn wounds from contamination. The patient was then started on vancomycin and piperacillin/clavulanate intravenously and eventually transitioned to a conventional ventilator.\nThe patient’s hospital course was complicated with an ischemic right foot on day 8. The etiology is likely due to the combination of shock, femoral arterial line, as well as preexisting vascular injury due to multiple surgeries in the past for clubfoot. Amputation of the foot was performed when vascular repair was not possible. During this period, the patient’s lung injury worsened and again required a VDR (). She continued to experience rising lactate levels with increasing diarrhea volume. On hospital day 12, blood cultures showed MRSA bacteremia. Bronchoscopy was performed and showed tracheitis with loose secretions. The mucosa distal to the carina was normal.\nDue to the increasing white blood cells and bands, the team was concerned that the patient may have developed chorioamnionitis and endometritis. Therefore, she was administered gentamicin and clindamycin per obstetrics recommendations. She continued to experience increased stool content and worsening lactate, leading the team to suspect enterocolitis. She also experienced worsening watery diarrhea. At this point, the team suspected pseudomembranous colitis, and metronidazole was added via enteral route. After administering the metronidazole, the patient showed little or no improvement, and stool cultures tested negative for C. difficile toxins A and B. The patient was unstable for sigmoidoscopy. Decubitus positioning resulted in severe hypoxia. Ultimately, a stool culture showed MRSA growth, and treatment was converted to enteral vancomycin. Watery diarrhea began to subside within 48 h of administering enteral vancomycin. At this point, the patient’s clinical course was focused on treating her ARDS. She continued to experience worsening hypoxia, requiring VDR and paralysis. Linezolid replaced intravenous vancomycin for better lung penetration. Enteral vancomycin was continued for 10 days, representation of her clinical diarrheal course is shown in .\nOn hospital day 26, it was found that the patient had experienced a rupture of the maternal membrane, Fern positive, which required immediate induction of labor. The fetus was delivered with no spontaneous movements, no spontaneous breathing, and no tone. Rescue efforts were not performed by family request due to the poor prognosis. Following the delivery of the fetus, the patient’s condition began to improve. By hospital day 40 the patient showed reduced oxygenation requirements, reduced ventilator support, and improved chest X-ray as shown in . She subsequently required skin grafting and was discharged to the acute ventilator rehabilitation facility after 72 days with near complete resolution of lung opacities shown in . The computed tomography (CT) on hospital day 20 demonstrated diffuse bilateral infiltrates, pleural thickening and subpleural cystic changes () at that time. |
An 81-year-old man with medical history significant for atrial fibrillation (on rivaroxaban), chronic kidney disease, hypertension, hyperlipidemia, glaucoma, gout and melanoma status post excision presented with cholecystitis that was managed with a cholecystostomy drain at another institution and symptoms resolved. Four months later he represents with several days of nausea, bilious vomiting, and abdominal pain. At presentation, he had normal bowel function with a last bowel movement approximately two days prior to admission. The patient was a former smoker and non-drug user without alcohol history. He had no significant family history recorded. On physical exam, he was afebrile, and his vital signs were notable for irregular heart rhythm, consistent with atrial fibrillation with rapid ventricular response (heart rate in the 130 s), and he demonstrated moderate right upper quadrant tenderness; his cholecystostomy tube remained in place with dark, thick bilious drainage and flushed easily. Laboratory work on admission showed white blood cell 10.9 K/cu mm. Liver enzymes were normal with aspartate amino transaminase of 15 U/L, alanine amino transaminase of 9 U/L, alkaline phosphatase of 78 U/L, and total bilirubin of 1.2 mg/dL. CT scan demonstrated pneumobilia, massive gastric distension, and an obstructing 5.8 cm calcified intraluminal mass in the first and second portion of the duodenum ().\nThe patient was placed on broad spectrum antibiotics to control early a potential development of cholangitis and a nasogastric tube was placed for gastric decompression. Heart rate control was managed with betablockers and calcium channel blockers. In preparation for surgery, his anticoagulation was held, and total parenteral nutrition was initiated.\nThe patient underwent exploration via a right subcostal incision. The location of the impacted stone was readily identified in the first and second portions of the duodenum (D1 and D2, respectively) which were severely inflamed up to and including the pylorus, with the gallbladder adherent to the anterolateral surface of D1 and D2. Several maneuvers including partial Kocherization, were utilized in attempt to mobilize the stone for extraction via gastrotomy; however due to the large size, location of the cholecysto-duodenal fistula, degree of impaction of the stone, and surrounding inflammation, this was not feasible. Cholecystectomy and cholecysto-duodenal fistula takedown were then performed, where it was noted that the stone was substantially (2–3x the diameter) of the fistula. This required extension of the defect over the anterolateral surface of D1 through the pylorus and a 6.2 × 4.2 × 3.5 cm stone was successfully removed (, ). The edges of the fistula were freshened and reconstruction was accomplished with Roux-en-Y duodenojejunostomy and gastrojejunostomy tube placement.\nThe patient’s intraoperative course was additionally remarkable for hemodynamic instability, atrial fibrillation with rapid ventricular response and sepsis during manipulation of the liver and duodenum. Management required anti-arrhythimics, electrical cardioversion, multiple vasopressors, blood product transfusions and continuous intraoperative transoesophageal echocardiography. Blood loss was modest. He was taken to the intensive care unit post-operatively and he rapidly recovered. Vasoactive medications were weaned, and he was extubated on post-operative day 1. On post-operative day 6, he had an upper gastrointestinal (GI) contrast study without evidence of leak; jejunal tube feeds were initiated and tolerated well. TPN was discontinued. He had post-obstructive gastric emptying dysfunction and required decompression through the gastric port of his gastrojejunostomy tube for several days prior to successfully transitioning to a regular diet. He was discharged to a rehabilitation center on post-operative day 21. His gastrostomy tube was removed at a follow-up clinic visit. |
A 20-years old male sprinter presented to our department complaining of sudden pain in both lower limbs along with painful calf cramps and feet numbness after intense training. Symptoms were apparent for more than three months even after running less than 100 meters and it severely affected his overall sports performance.\nPhysical examination revealed no motor deficit in both legs. Skin and temperature sensation determined with a swab or pinching and an alcohol-soaked gauze, respectively, were normal. Peripheral pulses were palpable in the posterior tibial and dorsalis pedis arteries; the ankle-branchial index (ABI) was 1.0 in both legs (normal values: 0.9-1.2). The above parameters remain unchanged after the treadmill walking test. However, while running on the treadmill, the skin sensation below the knees was reduced. The pinch test of plantar and dorsal feet was also abnormal. Deep palpation of the popliteal fossa was extremely painful and the pain radiated toward the feet but the Tinel’s sign in popliteal fossa was negative. The ABI values remained stable in both legs. Magnetic resonance imaging (MRI) of both knees did not reveal any abnormal findings. Electromyography (EMG) test showed normal function of all nerves of the lower extremities. Similarly, digital subtraction angiography (DSA) revealed normal popliteal artery flow bilaterally. According to the symptoms and the laboratory findings, sciatic nerve entrapment to the popliteal fossa was suspected and after a thorough discussion with the patient, consent was obtained for surgical exploration of the sciatic nerve.\nUnder general anaesthesia, a bilateral posterior approach of popliteal fossa using a lazy S-shaped skin incision was utilized. In both legs, the sciatic nerve was found to be entrapped proximally from its division to the tibial and common peroneal nerves from the terminal branch of the SSV into the PV (Figure ). The SSV had previously divided into its terminal popliteal branch that ran obliquely and it crossed above the sciatic nerve and Giacomini vein which is a thigh extension of SSV. The terminal section of SSV was subsequently ligated and resected to relieve the pressure on the sciatic nerve (Figure ). Postoperatively, partial weight-bearing with crutches for two weeks was suggested. After that time, gradual mobilization and return to sports was recommended. At three months from surgery, the patient was pain free and was able to run without facing intermittent claudication. At the latest follow-up one year after surgery, he was able to run long distances as well as sprint and train without any restriction. |
Twenty-one year-old woman was reffered to our department at November 2014 from a medical center where she was followed for 2 years with diagnosis of immune thrombocytopenia and ıron deficiency anemia. During that follow-up period she did not experience any bleeding and had moderate thrombocytopenia required not any treatment with antihemoragic drugs. However an oral iron supplementation on an occasional basis was given for anemia. Therefore she was referred to our clinic for further diagnosis and treatment. Her medical history showed that, she had been recovering while she was receiving oral iron supplementation; however her hemoglobin (Hb) levels were decreasing soon after cessation of treatment. Her thrombocytopenia (ranged from 45.000 to 55.000/mm3) persisted and there were no clinical findings consistent with blood loss that can explain a possible iron deficiency anemia. Also according to her anamnesis she reported complaints of chronic weakness, intermittent spontaneous ecchymoses, and an intermittent abdominal pain of unknown etiology. All necessary evaluations including abdominal ultrasound, and also assessments regarding thrombosis and FMF were performed however no clinical evidence was found for her abdominal pain.\nHer thrombocytopenia (ranged from 45.000 to 55.000/mm3) persisted and there were no clinical findings consistent blood loss that can explain a possible iron deficiency anemia. On physical examination, she was anemic (pale appearance of skin and mucous membranes), her spleen was slightly palpable (1 cm from 12th rib), and there were a few small (diameter <1 cm) ecchymosis at her lower extremity. During admission a series of laboratory tests and a bone marrow examination have been performed. Initial laboratory test showed a thrombocytopenia with a mild anemia and increased serum LDH levels (). Urine analysis showed hemosiderin cylinders. Bone marrow examination (both aspiration and biopsy) revealed just a mild erythroid hyperplasia (normal cellularity: 60%). Hypersegmentation in megakaryocytes and and slight dysplastic changes (multiple separated nucleus) were also observed. Cytogenetics was performed and result was 46,XX. Presence of iron deficiency was observed via bone marrow biopsy performed using iron staining. Therefore we performed fluoresceinlabeled proaerolysin (FLAER) flow cytometric assay with the pro-diagnosis of non-immune mediated hemolytic anemia. Flow cytometric evaluations (with FLAER and/or CD16, CD24, CD66b) showed a PNH clone of 15% for erythrocytes, 64% for monocytes, and 60% for granulocytes. According to clinical, laboratory, and cytogenetic result the patient was diagnosed with PNH and eculizumab therapy was started. After initiation of eculizumab therapy, abdominal pain attacks disappeared, both hemoglobulin and LDH levels normalized, and platelet values increased slightly as expected. Also no adverse reaction was seen due to eculizumab therapy. |
A 13-year-old Caucasian female with no significant past medical history presents to the emergency department with significant chest pain after being impaled by a metal post. She states that she was climbing a fence when she slipped and fell directly on top of the post. Emergency medical services detached the post from the fence. On arrival, she was hemodynamically stable with a Glasgow Coma Scale (GCS) of 15. The patient endorsed severe pleuritic chest pain and lightheadedness but had equal breath sounds bilaterally and was saturating well on room air.\nTrauma workup was initiated to determine the extent of internal injuries. The chest X-ray demonstrated a large metallic foreign body projecting into the right hemithorax with no mediastinal widening or pneumothorax (Figure ). Further characterization with CT chest showed the metallic object entering superolaterally through the anterior-inferior mediastinum and right hemithorax terminating near the right heart border and hilum (Figure ). The aorta was normal in contour and caliber. The cardiovascular surgery team was consulted for the evaluation and management of the injury.\nDue to the penetrating injury and concern for damage to vital structures such as the ventricles or right hilum, a median sternotomy was emergently performed in order to remove the foreign body from the thoracic cavity. On surgical exploration, there was penetrating trauma to the mediastinum just superficial to the pericardium and hilum. Miraculously, the foreign body did not pierce the ventricles or the lungs due to a continuous piece of metal crossbar that ran perpendicularly, preventing further entry. The metallic rod measured approximately 45 cm in length. Right thoracostomy and mediastinal tubes were placed in order to drain serosanguinous fluid and postoperative air (Figure ). The patient was closely monitored in the surgical intensive care unit (ICU). On postoperative day 2, chest tubes were removed, and she was transferred to the floor. Her sternal discomfort was well-controlled with multimodal pain medications. The following day, the patient was asymptomatic and back to her baseline and safely discharged home with close follow-up with cardiovascular surgery as an outpatient. |
A 45-year-old Caucasian man was admitted to the Accident and Emergency Department of our hospital after presenting with a two-week history of progressive walk impairment associated with paresthesias around his mouth and the fingers on his right hand. The most remarkable features of his medical history were the presence of type 2 diabetes (recently diagnosed), dislipemia, high blood pressure and morbid obesity (body mass index: 41.3).\nA medical examination showed a lower limb paresis (grade 3/5), which was more prominent in dorsal flexion of both feet, an absence of reflexes and a loss of superficial sensitivity in his left limb. The presence of enlarged lymph nodes in the cervical area was also evident; they were variable in size (some of them of more than 1cm) and mobile. Furthermore, a large scalp mass was identified in the parietal-occipital area; it was stuck to the skull, had an irregular shape, a tough texture, and palpation was not painful. Apart from these findings, the presence of a bilateral gynecomastia and a hyperpigmentation of the skin, mostly of the areoles, were noteworthy.\nAn electromyography (EMG) test was performed to better determine the neurological symptoms. The EMG showed a motor-dominant polyneuropathy with demyelinating features in his lower limbs. The administration of steroids only partially improved his neurological symptoms.\nA cranial computed tomography (CT) scan was performed and showed a mass in the scalp with intense underlying bone erosion (Figure ). The mass was in contact with the posterior part of the sagittal sinus, but did not invade it. There were no signs of brain damage or infiltration and the tumor presented intense vascularization, mainly from vessels of the external carotid artery (ECA). A magnetic resonance imaging (MRI) scan could not be performed because of our patient’s high body weight. A whole-body CT scan was carried out and revealed adenopathies in the cervical area, mediastinum and retroperitoneum. Splenomegaly was also identified.\nHis laboratory test results showed a significant increase of immunoglobulin G (IgG) with a clear peak in the proteinogram, increase of lambda and kappa light chains in serum and high levels of vascular endothelial growth factor (VEGF) (Table ). No Bence Jones proteinuria was identified. A bone marrow biopsy showed reactive changes and intense plasmocytosis and pathological analysis of one of the cervical lymph nodes showed reactive changes with peripheral follicular proliferation, hyalinization of the vessels and the presence of plasma cells (Figure d). These findings were compatible with Castleman’s disease. Serological tests for human herpes virus-8 (HHV-8) and human immunodeficiency virus (HIV) infection were performed, with negative results, because of the association between Castleman’s disease and these viral infections [,]. Castleman’s disease occurs in about 11 to 30% of patients with POEMS syndrome [].\nThe decision was taken to surgically remove the parietal-occipital mass, with a previous embolization of the main arteries feeding the tumor in order to avoid excessive bleeding during surgery. The patient was operated on under general anesthesia. We performed a circular craniectomy around the tumor and a complete excision of the lesion by dissecting its adherences with the dura mater, which did not seem to be damaged (Figure a and 2b). A titanium mesh was placed in the osseous defect.\nPathological analyses concluded that the tumor was a well-differentiated plasmocytoma with expression of CD38, IgG and lambda light chain (Figure c). A bone marrow biopsy showed reactive changes with intense plamocytosis.\nSubsequently, according to the definitive pathological results, dexamethasone pulse therapy was initiated (three pulses per month of 20mg/day for four days) and the patient also received radiotherapy to the skull to complete the treatment, with an accumulated dose of 50Gy in the surgical field.\nAs mentioned above, his neurological status improved slightly with the initial prescription of steroids, but neurological deficits recovered progressively after surgery. The rest of the POEMS features also improved after surgery and even more so when steroids and radiotherapy were initiated (two weeks after surgery).\nApart from his clinical status, his response to treatment was evaluated with positron emission tomography (PET), which was performed two months after the last steroid pulse. It showed a complete resolution of adenopathies in the different areas and no abnormal activity was identified in the skull. Moreover, vascular endothelial growth factor (VEGF) and lambda chain serum levels normalized progressively. During the 18-month follow-up, there have not been any clinical or laboratory abnormalities to indicate a recurrence of the disease. No complications related to the surgical procedure were reported (Figure ). |
A 64-year-old man was referred to the otorhinolaryngology outpatient clinic of the Karadeniz Technical University Medical School with a 2-month history of hoarseness. He had not been smoking for 5 years but had smoked for 10 years previously. He denied excessive alcohol consumption. A laryngeal examination revealed thickening in the left true vocal fold. There was fixation of the left vocal cord and approximately a 3 cm tumor arising from the left true vocal fold invaded the left arytenoid and occupied the ventricle. A biopsy was taken from the lesion with suspension microlaryngoscopy under general anesthesia. A histopathological diagnosis of pleomorphic rhabdomyosarcoma was made based on the detection of fused cells of variable dimensions with eosinophilic cytoplasm, which were irregular and hyperchromic (see ). They also had large or multiple nuclei. The neoplastic cells expressed desmin (see ), actin, and myogenin (see ) in immunohistochemical testing, confirming the diagnosis of rhabdomyosarcoma. The immunostainings with p63, p40, EMA, and cytokeratin-7 were all negative excluding the diagnosis of sarcomatoid carcinoma. Computerized tomography (CT) examinations of the abdomen and thorax revealed no metastases, and no lymphadenopathy was detected in the neck. The tumor was clinically staged as T3N0M0 (stage III) glottic cancer. The patient underwent total laryngectomy (see ) and left radical neck dissection with an uneventful postoperative recovery. A histological examination of the total laryngectomy specimen confirmed the diagnosis of pleomorphic rhabdomyosarcoma and excised lymph nodes were reported to have reactive hyperplasia. Because the surgical margins were in close proximity to the neoplastic cells (<1 cm), 3-dimensional conformal radiotherapy was performed with 6 MV photons in an Elekta Synergy Platform linear accelerator. Grade III-IV mucositis did not develop, as the patient received Ethyol and glutamine during the therapy. The patient decided not to receive chemotherapy after the evaluation of the tumor size, tumor grade, and lymph node involvement. The patient has continued monthly follow-up visits and has remained disease-free for 2 years after the histological diagnosis. |
A 13-year-old boy referred to the Department of Pedodontics with a chief complaint of irregular teeth. The patient had been referred by an orthodontist who had placed orthodontic brackets on selected incisors as well as primary maxillary right canine. Radiographic examination had been ignored prior to treatment, leading to erroneous placement of a bracket on the primary canine.\nAn extra-oral examination showed no swelling or lymphadenopathy. The intra-oral examination showed a complete set of permanent teeth in the left maxillary area up to the second molars but on the right side, the primary canine was firmly retained. However, its permanent counterpart was not visible intra-orally. There was neither a canine bulge nor a cortical expansion of the maxilla. The mandibular arch had a full complement of permanent teeth up to the first molars except the mandibular second primary molars which were near exfoliation.\nAn orthopantomograph revealed a supernumerary tooth-like structure between the roots of permanent maxillary right lateral incisor and primary maxillary right canine. It was enclosed in a unilocular cystic space, which had caused divergence of the roots of these teeth (). In addition, there was an overlying impacted, mesially rotated permanent right canine. Radiologically, the lesion resembled a compound odontoma or a dentigerous cyst.\nThe over-retained primary canine was extracted. The embedded tooth-like structure was surgically enucleated along with its cyst-like lining (). It had a conical crown and a short root with a closed apex. It was then sent for histopathological examination. The patient is currently undergoing comprehensive fixed mechanotherapy to facilitate eruption of impacted maxillary right canine.\nHistopathological examination showed strands of polyhedral epithelial cells with pleomorphic nuclei and calcification in the form of Liesegang rings (). The diagnosis confirmed calcifying epithelial odontogenic tumor. On Congo red staining, the tissue specimens showed mainly blood and fibrin with few calcified areas and scant epithelial rests. The Congo red staining was negative for amyloid. |
A 34-year-old gravida 4, para 2 female presented to the Perinatology Centre for management of presumed heterotopic angular pregnancy located in the left cornu of the uterus. The patient had undergone ovarian stimulation. Her gestational age was 13w + 3d based on early ultrasound. A Non-Invasive Prenatal Test showed no pathology. The patient’s prior pregnancies included two full-term normal spontaneous vaginal deliveries and one ectopic pregnancy. The patient had a history of laparoscopic ovarian cystectomy, appendectomy, cholecystectomy, laparoscopic treatment of ovarian apoplexy and laparoscopic treatment of ectopic pregnancy in the left fallopian tube.\nOn admission, the patient’s vitals were stable. The patient reported episodic abdominal pain on the left side. Transabdominal ultrasound imaging revealed a hypoechogenic 3.05 × 3.08 cm size mass in the left cornu of the uterus, filled with fluid (without viable embryo), which, by evaluation of the blood flow, could have been related to the uterus. .\nAn urgent Magnetic Resonance Imaging (MRI) was performed. A 36 × 20 × 36 mm size cystic mass with T2-hyperintense wall in the left cornual region was observed. An MRI scan also showed one more fetus inside the uterine cavity with placenta located on the left lateral wall. The diagnosis of a heterotopic angular pregnancy in the left cornu of the uterus was established. The patient was hospitalized for further observation.\nDuring hospitalization, blood and urine tests’ results were within the normal range. A multidisciplinary team (MDT) decided to keep monitoring the growth of both pregnancies by ultrasound while maternal vitals were stable and noted that further management would be determined according to the clinical situation. On the third day of hospitalization the patient started to feel more severe pain in the hypogastric region, radiating to the back and the left groin. The MDT made a decision to perform a diagnostic laparoscopy. Laparoscopy confirmed heterotopic angular pregnancy in the left enlarged, swollen cornu of the uterus. The left ovary and fallopian tube were not damaged and there were no signs of uterine rupture. and .\nThe postoperative period was uneventful. After the surgery the MDT decided not to perform any further surgical interventions and to keep monitoring maternal status as well as ultrasonographic findings. The patient‘s general condition improved; the pain subsided.\nIn 6 days, ultrasonography was repeatedly performed. The hypoechogenic mass on the left cornual region of uterus was observed; however, its size remained unchanged. Another fetal ultrasound was scheduled in a week and the patient was discharged for further outpatient care. The similar ultrasonographic view was observed during the following ultrasound scans at the 15th and 20th wk.\nOn the 22nd week of gestation, the woman was admitted to tertial level hospital complaining of pain in hypogastric and left iliac regions of the abdomen, provoked by physical exercise. Ultrasonography was performed, the remaining unchanged mass in the left cornu of the uterus was observed as well as an intraamniotic septum in the lower segment of the uterus, as shown in and . The fetal growth of the intrauterine pregnancy was unaffected and matched its gestational age. Conservative treatment was chosen, the pain resolved and the patient was discharged in 2 days.\nAt the 27th, 30th, 35th and 37th weeks of gestation, the ultrasonography was performed to monitor any possible changes of the mass in the left uterine cornu—the mass was compressed and its size did not differ significantly.\nAt the 41st week of gestation, the patient was admitted to the Obstetrics department due to the spontaneous rupture of membranes—transparent amniotic fluid was observed. A healthy female neonate (weight 2850 g, height 50 cm) with Apgar scores of 9 at 1 min and 10 at 5 min was born via spontaneous vaginal delivery in occiput posterior position. No Oxytocin was used during the labor. On the first day after delivery, transabdominal ultrasound was performed. A compressed mass of 3.3 × 1.4 cm remained visible in the left cornu of the uterus. Moreover, an intensified blood flow was observed on the left corner of the uterus in comparison with the right uterine corner. The postpartum and postnatal periods were uneventful, and the patient was discharged together with her newborn 2 days later.\nAfter the discharge, follow-up visits were arranged in an outpatient clinic. The further postpartum period was uneventful, and the patient did not have any specific complaints. The β human chorionic gonadotropin (β-hCG) blood tests were performed on the 1st, 7th, 14th and 30th days after delivery—in 30 days it decreased drastically from 3602 international units per liter (IU/L) to 1.78 IU/L accordingly.\nThe follow-up transvaginal ultrasonography was performed after one month. The remaining mass sized 1.67 × 0.56 cm with visually more intensive blood flow was observed. . |
A 46-year old nonsmoker male presented in December of 2007 with right supraclavicular lymphadenopathy. An excisional biopsy of the lymph node was performed. Histopathology () showed the morphologic features of SCLC including small to medium size cells, high nuclear/cytoplasmic ratio, salt and pepper chromatin with inconspicuous nucleoli, nuclear molding, and high mitotic activity. Immunostaining showed that the tumor cells expressed synaptophysin and chromogranin and discontinuous cytokeratin markers. TTF-1 was also positive. Imaging was performed with PET/CT showing a 5 cm right hilar mass and right paratracheal lymphadenopathy and no disease elsewhere including a negative brain MRI.\nThus, he was deemed to have limited stage disease and was treated accordingly with cisplatin and etoposide and concurrent radiation therapy (). He achieved a complete response after 6 cycles of chemotherapy and subsequently underwent prophylactic cranial irradiation. He was monitored clinically and by imaging every 3 months. In May 2009, the disease relapsed with left supraclavicular lymphadenopathy and was confirmed by excisional biopsy. He underwent radiation therapy with concurrent cisplatin and etoposide for 2 cycles followed by 4 cycles of oral topotecan. He had complete response again that lasted for one year. In September 2010, he had a relapse presenting with mediastinal lymphadenopathy. After another 5 cycles of cisplatin and etoposide, he had near complete response, and he was monitored clinically. After progression in September 2011 with increased hypermetabolic activity in the right hilum and paratracheal lymph nodes, he was started on carboplatin and etoposide. He had good response after 4 cycles and was switched to oral etoposide. He again progressed with increased metabolic activity in the right hilar and paratracheal region.\nAt this time, genomic profiling of his prior tumor biopsy was undertaken. This showed that his tumor harbored a novel JAZF1-MYCL1 gene fusion but lacked alterations in TP53 and RB1. This was performed in a CLIA-certified, CAP-accredited commercial laboratory. The technique used was next-generation sequencing with hybridization-captured, adaptor ligation-based libraries to high, uniform coverage (>500×) for all coding exons for 236 cancer-related genes plus 46 introns from 19 genes frequently rearranged in cancer []. All classes of genomic alterations (GA) were identified including base substitutions, insertions/deletions, copy number alterations, and rearrangements. Although mutations in TP53 and RB1 are observed in vast majority of SCLC cases, a small fraction of these tumors can be wild type for TP53 and RB1 []. There were no mutations in EGFR, BRAF, and MET or rearrangements in ALK or ROS1 that are seen in other types of lung cancer. He was then enrolled in a clinical trial with Aurora A kinase inhibitor (MLN8237/Alisertib-50 mg BID for 7 days of 21 days cycle), in April 2012 [, ]. He experienced an objective response after 4 cycles and near complete response after 10 cycles of therapy (). He remained on this drug for 23 cycles (18 months). In September 2013, he developed progression with aortocaval lymphadenopathy. Over the next 18 months, he was treated with several chemotherapeutic agents with disease progression as his best response (carboplatin plus etoposide, topotecan, everolimus, temozolamide, docetaxel, and sunitinib). His disease progressed to involve several organs including the brain, spinal cord, liver, pancreas, adrenals, bone, and pleural, pericardial, and peritoneal spaces. During this time, he underwent several palliative procedures including spinal decompression surgery, multiple instances of stereotactic brain radiation, and ureteral and biliary stents to relieve obstruction.\nHe was then started on nivolumab (3 mg/kg every 2 weeks) in August 2015 based on preliminary results from a phase I/II study []. He had a dramatic response. Initially, he had recurrent pleural effusions requiring 6 thoracenteses in the first few weeks as well as pericardial effusion with cardiac tamponade requiring pericardial window []. He had evidence of partial response at 8 weeks of therapy and near complete response at 16 weeks of therapy in December 2015 (). He developed local recurrence in right adrenal gland in May 2016, which was treated by right adrenal gland resection. Unfortunately, the disease progressed again in April 2018 with transient response to platinum doublet. This was followed by a rapid progression, and he succumbed to disease in October 2018. |
A 34-year-old Para 1 + 1 (1 alive) woman presented at the Gynaecological Outpatient Clinic of a public tertiary hospital in Lagos, Nigeria with 8 years history of recurrent vaginal mass and 5 years history of vaginal pain. The mass was initially small like a bean seed but gradually increased in size until it became palpable at the introitus. Vaginal pain was persistent, aching in nature, with a severity of 6 on the numerical pain scale and no aggravating or relieving factors. No history of vaginal instrumentation, abnormal vaginal bleeding or discharge, weight loss, respiratory, urinary and bowel symptoms. She had no history of pelvic irradiation, insertion of vaginal implant, personal or family history of uterine fibroids or malignancies. She had two surgical resections of the tumour in the past, but histology was not carried out due to financial constraint.\nHer general, breast and abdominal examination findings were essentially normal but vaginal examination revealed a polypoid mass in the lower vagina. A diagnosis of recurrent vaginal polyp with suspected malignancy was made and she was counselled for examination under anaesthesia (EUA), wide local excision (WLE) and histological evaluation of the tumour.\nInvestigations revealed normal haematological, renal and liver function profiles. Hepatitis B, C and human immunodeficiency virus screenings were negative; Pap smear was negative for intraepithelial lesion or malignancy. Serum cancer-antigen 125, carcino-embryonic-antigen, chest X-ray and abdomino-pelvic computerized tomography scan were normal.\nEUA revealed normal vulva and polypoid mass ~6 cm × 4 cm × 2 cm arising from the lower third of the right lateral vaginal wall. The mass was irregular, firm, encapsulated and attached to the muscular wall. The cervix, uterus, adnexae and rectum were normal. There were no enlarged inguinal lymph nodes. A WLE with a surgical margin of 10 mm was done and histology revealed nodular multi-lobulated solid firm masses with greyish white cut surfaces on macroscopy. Microscopic analysis showed a highly cellular encapsulated lesion, composed of interlacing fascicles of pleomorphic spindle shaped cells with marked nuclear pleomorphism, hyperchromasia, bizarre multinucleated giant cells with abnormal mitosis >10 mitoses/10 HPF. The tumour cells show moderately strong positivity for ki-67, alpha-smooth muscle actin, and desmin but was negative for s-100 (–) and the surgical margins were positive for malignant cells. A diagnosis of poorly differentiated VLMS was made with International Federation of Gynecology and Obstetrics clinical stage 1B disease. She had sequential chemo-radiation therapy with four courses of docetaxel and gemcitabine chemotherapy followed by external beam (45 Gy) and intra-cavitary (25 Gy) radiation. She responded well to the therapy and she is currently being followed up in the clinic. |
In November 2004, a 54-year-old woman was referred to the breast cancer center of our hospital due to microcalcification in the left breast, which was incidentally detected by mammography during routine breast cancer screening. Ultrasonography showed a hypoechoic lesion with an irregular margin in the left breast (). A vacuum-assisted breast biopsy of that lesion revealed a microinvasive ductal carcinoma. She underwent subcutaneous mastectomy with axillar lymph node dissection and immediate implant insertion. Pathology results revealed a T1miN0M0 invasive ductal carcinoma (). Immunohistochemistry results indicated estrogen receptor negative, progesterone receptor positive (50%), borderline cerbB2 (2+/3), and 5% Ki67 positivity (). She completed endocrine therapy with anastrozole medication for 5 years. She was regularly followed up at the breast cancer center and underwent appropriate tests including mammography and breast ultrasonography every 6 to 12 months.\nThe patient had histories of gastric cancer and Sertoli-Leydig cell tumor of the right ovary. Fifteen years ago (1989), she was diagnosed with advanced gastric cancer at the age of 39 years. She underwent radical subtotal gastrectomy at another hospital and then received adjuvant chemotherapy for 6 months. Ten months ago (2004 January), a 4-cm right ovarian mass was incidentally detected by computed tomography (). Serum carcinoembryonic antigen, carbohydrate antigen 125, and carbohydrate antigen 19-9 levels were within the normal limit. She underwent bilateral salphingo-oophorectomy. Pathology results revealed a Sertoli-Leydig cell tumor with moderate differentiation (). She had a history of receiving total abdominal hysterectomy due to a uterine leiomyoma 2 years ago. Twelve years ago, she was diagnosed with pulmonary tuberculosis, which was cured under medication for 1 year. She reported having no other past medical histories and any family history of malignancies. She regularly visited a family medicine clinic for routine health check-ups that included tests such as gastroscopy. She also regularly visited a gynecology clinic for routine gynecological examinations.\nIn August 2010, a sub-centimeter-sized nodule was incidentally detected in the left thyroid. Malignant cells of the suspected papillary carcinoma were diagnosed by fine-needle aspiration cytology. She underwent total thyroidectomy (). Pathology results showed T1aN0M0 papillary thyroid cancer (). She was regularly followed up for thyroid cancer with a 6-month interval, receiving appropriate tests that included neck ultrasonography. In August 2016, several hypoechoic masses with an extent of 2.8 cm were incidentally detected in the right breast by routine follow-up ultrasonography (). A core needle biopsy revealed a high-grade ductal carcinoma in situ. She received a mastectomy with a sentinel axillar lymph node biopsy. Final pathology results revealed a T1miN0M0 invasive ductal carcinoma (). Immunohistochemistry revealed estrogen receptor negative, progesterone receptor negative, borderline cerbB2 (2+/3), and 30% Ki67 positivity (). She did not receive any adjuvant therapy for her right breast cancer. Mutation tests on BRCA1 and BRCA2 showed no clinically significant mutations, although unclassified heterozygous variants (851T>G [c.623T>G], p.Val208Gly) were detected for BRCA2. She was regularly followed up at the breast cancer center with a 3-month interval.\nIn February 2017, multiple liver masses were incidentally detected by computed tomography (). Further evaluation revealed an additional 2-cm rectal mass by computed tomography and colon fibroscopy (). The serum carcinoembryonic antigen level was within the normal limit. Biopsies for liver and rectal masses led to the diagnosis of grade 2 neuroendocrine tumors for both the liver and rectal masses (). She was diagnosed with a primary neuroendocrine tumor of the rectum with multiple metastases to the liver. She was 66 years old at that time. She refused further evaluation and treatment at this hospital. She decided to visit another hospital, and further follow-up was lost. She died in August 2017. The types of organs involved in this case are summarized in .\nThis case report was approved by the Institutional Review Boards of Seoul Metropolitan Government Seoul National University Boramae Medical Center (20-2019-2) with a waiver of informed consent. |
A 59-year-old male was referred to the Department of Orthopedic Surgery, Osaka City University Hospital (Osaka, Japan)in February 2008. The patient presented with a seven-month history of gradually increasing pain in the right leg. The patient had previously visited Fuchu Hospital (Izumi, Japan) due to an abnormal shadow on the right proximal femur and was subsequently referred to our hospital. A plain film revealed an osteolytic lesion with an ill-defined margin in the right proximal femur (), suggesting a malignant bone tumor. Pelvic CT also revealed a mass in the right ilium, extending into the gluteal muscle (). Lung plain film () and CT () confirmed multiple masses in bilateral lung fields. Screening of the abdominal CT to detect the primary cancer revealed an occupying mass in the left kidney (). Other metastases involving the pancreas and abdominal lymph nodes were also revealed. Subsequent to consultation with the Department of Urology and clinical staging, the patient was diagnosed with advanced-stage RCC (cT3aN3M1; stage IV). Resection of the primary RCC and palliative surgery with a γ-nail for an impending fracture of the right proximal femur were performed simultaneously, which revealed hemorrhagic brown tissue. The histology of a surgical specimen revealed that the tumor was composed of cells with clear cytoplasm and alveolar structural patterns. The pathological diagnosis of the surgical specimen of the curettage material was consistent with renal clear cell carcinoma.\nAt two weeks post-surgery, radiotherapy (36 Gy/12 fractions) was administered to the tumor in the right proximal femur for three weeks and subcutaneous injection of interferon-α (5×106IU) was started (5 times per week, for 9 months). The patient then received 200 mg oral sorafenib combined with interferon-α every day for two weeks, subsequently the dosage of sorafenib was increased to 400 mg. No major adverse effects were experienced, but a dry skin rash developed on the face and trunk, and the patient experienced mild diarrhea. Subsequently, the dose of sorafenib was reduced to 200 mg for two weeks. Four weeks later, the dose was increased to 400 mg. Treatment with sorafenib was continued for eight months and the dose (400–600 mg) was determined according to the adverse effects experienced by the patient.\nAt eight months post-surgery, a plain film () showed no apparent progression in the right femur, and pelvic CT demonstrated regression of the mass in the right ilium (). Plain film and CT of the lungs also revealed favorable responses (). The abdominal lymph node swelling was also reduced. The patient remained alive with the disease at the time of follow-up at 36 months post-surgery. |
In 2005, a 59-year-old male patient who suffered from occipital headache underwent complete surgical excision of a solitary cerebellar mass. Occipital transtentorial approach was done, and pathological diagnosis was confirmed to be hemangioblastoma (). He had no family history of VHL disease and no clinical stigmata to suggest the presence of VHL disease. VHL gene mutation was not detected in his peripheral blood sample. His symptom resolved after surgery, and the magnetic resonance image (MRI) of the brain showed no remnant lesion postoperatively. The patient was followed up with MRI, and no evidence of residual or recurrent lesion was observed until 2010.\nIn 2010, MRI of the brain showed a newly developed nodular mass at right medial edge of tentorium cerebelli suggesting dissemination of HB (). And in 2012, similar mass was observed at the posterior falx on the MRI (). There was no evidence of recurrence at the resected area and workup for VHL disease was also negative. He had no clinical symptom. He received cyberknife radiosurgery (total dose of 1,800 cGy/3 fx each) for each lesion in 2010 and 2012. After radiosurgery, the size of each lesion was reduced temporarily, but soon after the lesion at tentorium grew larger and other masses have newly developed around the pons and midbrain ().\nWhole spine MRI revealed negative finding until 2012, but a intradural extramedullary mass was noted at T6-7 level in 2012 without any symptom (). In 2014, during follow-up of the thoracic lesion, he developed severe back pain at T9 dermatome, which correlated with the lesion. Spine MRI was done and it showed enlargement of the previously noted tumor mass at T6-7 level. Workup for VHL disease was done again, and the peripheral blood was analyzed for the presence of VHL gene mutations by direct sequencing method using ABI 3,730 sequencer. However, VHL gene mutation was not detected and there was no evidence of VHL disease. The patient underwent surgery for thoracic mass, and the tumor was completely removed (). The pathologic diagnosis of the tumor was HB, which is same as the cerebellar lesion resected previously (). The Ki-67 index was 10%. After surgery, his symptom was improved and until this case being reported, his Karnofsky Performance Scale score was 90 and remained symptom-free. |
A 39-year-old male patient, a known case of non-ischemic dilated cardiomyopathy (Left Ventricular Ejection Fraction 30%) who was in NYHA functional class II on drug therapy (Frusemide 20 mg, Aldactone 50 mg, Ramipril 5 mg and Carvedilol 6.25 mg twice daily) presented with history of recurrent palpitation and pre-syncope of 24-hour duration. His ECG revealed polymorphic ventricular tachycardia (PVT) []. On further enquiry, it was found that the patient is taking amiodarone 600 mg daily for last 3 months. Amiodarone was prescribed to him for one episode of sustained monomorphic ventricular tachycardia (VT) requiring cardioversion and he was continuing with the loading dosage of amiodarone by mistake for 3 months. There was no history of any other drug intake. Serum electrolytes were within normal limits. As there was association with no other offending agent other than high dosage of amiodarone, we kept the possibility of amiodarone induced PVT. The patient was treated with intravenous magnesium (2 g) along with intravenous lidocaine (1 mg/kg IV bolus) with out any effect. Intravenous Atropine 1.2 mg was also given to accelerate the sinus rate but failed to suppress the arrhythmia. Two more boluses of lidocaine at five minute intervals along with infusion at a rate of 1 mg/min were administered but the patient continued to have PVT. As the arrhythmia failed to respond to intravenous magnesium, atropine and lidocaine, we thought to try phenytoin to suppress the arrhythmia (a class IB antiarrhythmic drug that suppresses early after depolarization (EAD)) responsible for initiation of PVT secondary to amiodarone overdose).[] Phenytoin was administered in a dosage of 100 mg slowly every 5 minutes. After administration of 300 mg of phenytoin, significant reduction in episodes of PVT was noted and sustained sinus rhythm with corrected QTc of 490 milliseconds was restored within 30 minutes []. The patient was administered intravenous phenytoin 300 mg/day for 2 more days followed by oral phenytoin 300 mg/day. He was discharged after 1 week on oral phenytoin. Predischarge holter monitoring showed normal sinus rhythm with infrequent monomorphic VPCs and no evidence of nonsustained or sustained VT. As the patient had developed amiodarone-induced PVT due to overdose, we also assessed the patient for extra cardiac toxic effects of amiodarone. The patient did not have any history of dry nonproductive cough and lung function test was normal. His liver function test was normal but thyroid function test showed evidence of hypothyroidism (TSH>10 m IU/ml with decrease in T3 and T4 levels). The patient, however, did not have any symptoms of hypothyroidism which can be subtle or even absent.[] Ophthalmological examination revealed evidence of corneal deposit but no visual impairment. In view of previous history of ventricular tachycardia requiring cardioversion with LV dysfunction, patient was advised Implantable Cardioverter-Defibrillator, but he refused due to non-affordability. He is at present asymptomatic and on medical follow-up with diuretic (Frusemide 20 mg+Aldactone 50 mg), ACE inhibitor (Ramipril 5 mg), β-blocker (carvedilol 50 mg/day), L-thyroxine 25 μg and oral phenytoin (300 mg/day) for last 2 months. |
A 23-year-old male presented with a persistent moderate headache and blurred vision on the right side following the sudden onset of a severe headache 2 weeks previously. Computed tomography (CT) revealed a 4 cm-diameter hyperdense intracerebral hematoma in the left occipital lobe and a hypodense, thin chronic subdural hemorrhage along the left cerebral convexity (). Magnetic resonance imaging (MRI) showed no enhanced lesions besides the hemorrhage (), and cerebral angiography revealed no abnormal findings. Left occipital craniotomy and hematoma removal were performed for exploration. Intraoperative findings revealed the presence of mixed hematomas (liquid and dense hematomas) and small-sized abnormal vessels. Biopsy specimens of both hematomas and vessels were obtained. A histopathological examination revealed no tumor cells besides the hematomas and abnormal vessels. The patient underwent annual follow-up MRI evaluations, and no new lesions were detected for over 4 years ().\nFive years later, a MRI revealed that a 7.5×4×5 cm-sized mass in the same location as the previous hematoma. The mass was visualized as mixed intensity on T1- and T2-weighted MR images and as a region of strong enhancement after intravenous gadolinium injection. There was no calcification and little cerebral edema was observed surrounding the mass (). Based on these findings, a brain tumor with intratumoral hemorrhage was suspected. The patient underwent a left occipital craniotomy and gross total resection of the tumor. Intraoperative findings confirmed that the tumor showed no continuity with the ventricular system (). Histopathological examination revealed clear cells with a sheet-like, papillary, or columnar to tubular arrangement. In addition, perivascular pseudorosettes were observed in the tumor. Immunohistochemical staining was focally positive for glial fibrillary acidic protein (GFAP) and epithelial membrane antigen, suggestive an ependymoma. Moreover, the tumor cells had diffuse nuclear pleomorphism, high cellularity, hemorrhage, necrosis, and a relatively high Ki-67 index (approximately 10%) (). Taken together, these factors confirmed a diagnosis of anaplastic ependymoma. Whole-spine MRI was performed for cerebrospinal fluid (CSF) dissemination workup, and there was no evidence of dissemination. Adjuvant local field radiotherapy (5,940 cGy in 180 cGy daily fractions) was administered to the left occipital lobe.\nFour years later, the patient was re-admitted to the hospital because of tumor recurrence at the cervicomedullary junction and dissemination at the intradural space of the eighth to ninth thoracic levels, without recurrence at the primary site (). He underwent a midline suboccipital craniotomy and hemilaminectomy at T8-9. Histopathological examination confirmed recurrence and dissemination of the tumor. Adjuvant spine local radiotherapy (4,500 cGy in 180 cGy daily fractions) was then administered. |
A 65-year-old gentleman, with a known case of chronic liver disease, presented with complaints of diffuse abdominal distension for two days associated with non-radiating, intermittent, colicky peri-umbilical pain. He had multiple episodes of bilious vomiting. He had not passed stool for one week and flatus for three days. There were no similar events in the past. He was not a known tobacco or alcohol abuser. He underwent no previous surgery.\nOn examination, he was found to be dehydrated with tachycardia. His abdomen was grossly distended and tender with diffuse involuntary guarding and rigidity. There was no mass palpable in the abdomen, no free fluid, and the hernial orifices were free. The bowel sound was absent. Digital rectal examination revealed collapsed and empty rectum with normal stool staining. His other systemic examination findings were clinically unremarkable.\nHis blood investigations revealed elevated blood urea nitrogen of 118 mg/dl and creatinine level of 3.6 mg/dl, suggestive of acute on chronic kidney disease, a normal hemogram, and a serum amylase of 207 IU/L. The serum amylase was at the upper limit of normal, and this was anticipated as such mild elevation is common in acute abdominal emergencies. A plain radiograph of the abdomen revealed multiple dilated small bowel loops with air-fluid levels, and ultrasonography (USG) of the abdomen revealed dilated aperistaltic small bowel loops (Figure ).\nThus, the diagnosis of acute intestinal obstruction was considered. A contrast-enhanced computed tomography (CECT) could not be done owing to a long waiting list for the procedure and also due to underlying renal failure in the patient. He was taken for emergency laparotomy as X-ray showed a classical picture of bowel obstruction and also for fear of poor outcomes in intestinal obstruction while waiting for CECT. As the patient was symptomatic for one week and with adhesive intestinal obstruction ruled out, as there were no prior abdominal surgeries in the past, conservative management was not considered. Intra-operatively, diffuse saponification of omental fat, inflamed lesser sac with extensive saponification, and calcification suggestive of acute pancreatitis were noted. The small and large bowel loops were grossly distended but viable with no obvious transition point. The dilated colonic loops were decompressed with a flatus tube. On further investigations to identify the cause of acute pancreatitis, he had hypercalcemia of 14 mg/dl. Thus, serum parathormone (PTH) levels were tested and found to be more than 2,000 pg/ml. USG of the neck was suggestive of a right inferior parathyroid adenoma. It was then confirmed by technetium (Tc-99m) sestamibi single-photon emission computed tomography (SPECT), which showed a focus of increased tracer uptake in a soft tissue nodule measuring 2.5 × 2.1 cm in the right para-tracheal region and posterior to the inferior pole of the right lobe of the thyroid. Delayed images showed persistent tracer retention in the focal uptake suggestive of an adenoma in the right inferior parathyroid gland (Figures , ). Thus, he was diagnosed to have primary hyperparathyroidism (PHPT)-induced hypercalcemia causing acute pancreatitis.\nMeanwhile, he underwent one session of hemodialysis along with anti-hypercalcemic measures, including intravenous fluids with forced calciuresis with diuretics to normalize his serum calcium levels. After proper stabilization and thorough pre-operative work-up, he underwent excision of the right inferior parathyroid adenoma under general anesthesia. The frozen section of the specimen was consistent with adenoma without any evidence of malignancy. His post-operative PTH level was 135.6 pg/ml, and calcium was 8.7 mg/dl. His post-operative period was uneventful, and he was thus discharged in a stable condition. |
In December 2006, a 31-year-old woman was referred to a neurologist because of consciousness disorder and fainting. Her main problems were obesity, snoring and waking up with a feeling of suffocation in the middle of sleep. The intraoral examination showed a large soft palate (). The soft palate was scored as class III according to the Mallampati classification (visualization of the soft palate and the base of the uvula) []. The electroencephalogram (EEG) showed focal dysrhythmia during hyperventilation with scattered sharp waves (). The patient was depressed and had sleep disorders such as sleep apnea and myoclonus, especially at the onset of sleep. She had experienced several occurrences of complete loss of consciousness during swimming and at work. The patient was on anticonvulsants and antidepressants (at first, she had been prescribed with Lamotrigine for 5 months, but later she was given 500mg Sodium valproate per day).\nOne of the best treatments for snoring during sleep is UUUP. The success rate of this type of surgery is reported to be between 16% and 83% [].\nWe chose a minimally invasive surgical procedure for the present case since the patient had a proper facial profile and a large soft palate (class III according to the Mallampati classification) [].\nIn May 2007, after analyzing the lateral cephalogram, we evaluated the craniofacial and pharyngeal airway morphology before the surgery. Under general anesthesia, 1cm of the soft palatal mucosa, from the right tonsil to the left tonsil, was removed. The patient’s tonsils were also removed during the surgery, and the anterior and posterior tonsillar pillars were sutured together ().\nThe symptoms were significantly decreased after the recovery. The patient no longer had sleep apnea, and antidepressants and antiepileptic drugs were discontinued. After the surgery, sharp waves were detected on the EEG at the level of the trachea (), but the patient was clinically asymptomatic. The 10-year follow-up showed no symptoms of sleep apnea or seizure. The patient did not lose any weight during the follow-up period. |
We present the case of a six-year-old boy who was brought to the emergency department by his mother after an episode of convulsion. She reported that he had jerky repetitive movements of all extremities that lasted around two minutes with spontaneous termination. He was not responding during the episode. The mother reported that his skin color turned blue. The mother reported that the movement was associated with urinary incontinence and frothy secretions from the mouth. There were no warning signs before the event. The mother reported that the child did not complain of any unpleasant sensation before the spell. The event occurred suddenly while the child was playing with his friend. He was not deprived of sleep before the event. The child appeared tired after the spell and he fell asleep. The child did not have a febrile illness. The mother reported no history of similar episodes.\nRegarding the medical history, the child was known to have asthma and eczema. His conditions were well-controlled. There was no history of previous surgeries. The perinatal history was uneventful. He was full-term with a birth weight of 3.8 kg. He was up-to-date with the vaccination schedule. He was not born of a consanguineous marriage. No family history of epilepsy was present. The social history was non-contributory.\nUpon examination, the child appeared alert and conscious. No dysmorphic features were evident. He was not pale, jaundiced, or cyanosed. His vital signs included a temperature of 36.5℃, a heart rate of 90 bpm, blood pressure of 80/58 mmHg, and respiratory rate of 23 bpm. His oxygen saturation was 99% on room air. The child had normal speech and comprehension appropriate for his age. His social and behavioral skills were normal. Upper and lower limb examination revealed normal tone and power. His gait was normal. The cardiorespiratory examination was unremarkable. Initial laboratory investigation revealed a hemoglobin of 14.2 g/dL, leukocytes count of 7,000 cells//µL, and platelet count of 390,000/µL. The random blood sugar was within the normal limits. Other biochemical findings, including electrolytes, hepatic, and renal profiles, were within the normal limits (Table ).\nConsidering the aforementioned findings of unexplained first-time seizure with normal laboratory markers, the child underwent magnetic resonance imaging for the brain to rule out any structural abnormalities. The scan demonstrated a large well-defined extra-axial cystic lesion occupying most of the left hemisphere that is connected to the ventricular system. The lesion had no grey-matter lining and it strictly followed the cerebrospinal fluid in all sequences (Figure ). Such finding represented the diagnosis of a giant left porencephalic cyst. The patient was referred to the pediatric neurology team. The child was given anticonvulsant therapy (valproic acid) to prevent further seizure episodes. The patient was followed at our institution for six months. He did not develop any further seizures while he was on anticonvulsant therapy. |
An 8 year old male was brought to the hospital with the history of difficulty in breathing since morning and an episode of convulsions followed by unconsciousness while on his way to the hospital. On examination he had increased surface temperature (37.8°C), tachypnoea with paradoxical respiration, tachycardia (124/min), hypertension (210/140 mmHg), Glasgow Coma Scale (GCS) of 3/15 and arterial oxygen saturation (SpO2) of 93% on 4 liters of oxygen. He was immediately intubated with a provisional diagnosis of hypertensive encephalopathy. All investigations being inconclusive to establish a diagnosis, the history was reviewed. It was revealed that at night the child was sleeping on the floor in open air. He suddenly woke up at 3.30 a.m. and complained of severe abdominal pain before going to sleep again. Two hours later he again woke up and complained of difficulty in breathing, swallowing and speaking. He also developed double vision. On his way to the hospital he had an episode of convulsions followed by unconsciousness. Considering the clinical history, physical findings (bulbar and motor paralysis) and inconclusive investigations, an Elapid snake bite was suspected. However, the absence of fang marks or localized swelling and the unusual presentation (hypertension, convulsions and unconsciousness) led to a mistaken diagnosis that stopped us from administering anti snake venom (ASV). Meanwhile the child was managed with ventilator support, Nitroglycerine infusion, intravenous Phenytoin and broad spectrum antibiotics. However, persistent motor paralysis with no improvement in GCS for more than 20 h with no identifiable cause led us to consider an ASV trial. Accordingly, 10 vials of polyvalent anti-venom were administered following which there was a dramatic improvement in both GCS and motor power. However, as he still had abdominal respiration another 10 vials of ASV were administered. Despite the full course of ASV the respiratory muscles appeared weak as tidal volumes generated were low requiring high pressure support mechanical ventilation. A single dose of neostigmine 0.8 mg and atropine 0.1 mg intravenously was given without much improvement. Thereafter the patient was gradually weaned and extubated after 8 days of mechanical ventilation and was discharged from the ICU on the ninth day without any residual morbidity. |
The patient was a 29-year-old woman who had undergone debridement and drainage for cervical lymph node tuberculosis 9 years prior to the current presentation. She had been examined 7 years prior to the current presentation via abdominal computed tomography (CT) at another hospital due to abdominal pain. That CT examination revealed a mass in the pancreas body and enlarged lymph nodes in the abdominal cavity. Understandably, at that time it was suspected that she had pancreatic cancer with lymphatic metastasis. Apart from the CT report however, the specific clinical examination index of the patient compiled at the other hospital was unavailable. Two years after the symptoms had been relieved via treatment with traditional Chinese medicine, the patient began experiencing repeated vomiting and melena that had persisted for the subsequent 5 five years; up to the time of the current presentation. At our hospital she stated that her psychological status, appetite, and sleep were normal, and she exhibited ochrodermia but no fever, jaundice, petechiae, or ecchymoses. On physical examination her general condition was good, and there was no abdominal tenderness, abdominal muscle tension, rebound pain, abdominal mass, or hepatomegaly. There was also no swelling of the cervical, supraclavicular, axillary, or inguinal lymph nodes.\nTo clarify the cause of the patient’s condition, biochemical blood analysis and routine blood examinations were performed. Adenosine deaminase and liver function were within normal ranges (Table ), but erythrocyte, platelet, and leukocyte counts were reduced (Table ). Alpha fetal protein, tumor associated antigen 125, and tumor associated antigen 199 results were normal. Tests for human immunodeficiency virus, hepatitis B virus, hepatitis C virus, and M. tuberculosis infection were negative, but an interferon gamma release assay was positive.\nTo further aid in the diagnosis, imaging examinations including abdominal non-contrast and contrast-enhanced CT, CT angiography (CTA), CT venography, and portal-phase three-dimensional vascular reconstruction were performed. Both non-contrast and contrast-enhanced CT indicated non-obvious enhancement in both the arterial and venous phases, and a heterogeneous, non-vascular, low-density mass was depicted in the lower margin of the pancreas body with poorly defined edges and dimensions of approximately 3.1 × 2.0 cm (Fig. a). An intumescent spleen and multiple nodular dense shadows around the pancreas, hepatic hilar region, and mesentery were also visible on CT (Fig. a). CTA, CT venography, and portal-phase three-dimensional vascular reconstruction depicted an enlarged splenic vein, narrowed initial section of the splenic vein, and tortuous gastric veins (Fig. b and d). Gastroscopy detected varicose veins under the gastric fundus mucosa (Fig. c), but the esophageal mucosa was smooth. Chest CT was conducted to investigate a potential history of tuberculosis, and it depicted a cable-like increased density of flaky shadows in the posterior segment of the upper lobe tip of the left lung. Based on the above results the patient was diagnosed with LSPH. Due to the nature of the mass in the pancreas however, it was unclear whether it was tuberculosis, a tumor, or another lesion. In the present case the mass was located in the lower margin of the pancreas body, and peripancreatic blood vessels were abundant, so it is difficult and dangerous to operate aspiration. According to our Multiple Disciplinary Team, endoscopic ultrasound-guided fine-needle aspiration biopsy (EUS-FNAB) was not applicative.\nDue to repeated hematemesis symptoms, after a blood transfusion and the improvement of anemia the patient underwent a splenectomy and perigastric fundus vascular dissection, and a lesion excision was performed for biopsy. Intraoperatively an enlarged spleen with dimensions of approximately 34 × 25 × 15 cm was observed. After dissociating the peritoneal adipose tissue it was evident that the left gastric vein, right gastric vein, left gastroepiploic vein, and right gastroepiploic vein were extensively tortuous and dilated (Fig. a). As the dissection deepened, swelling of the lymph nodes at the greater curvature, hepatoduodenal ligament, and lower margin of the pancreas were apparent (Fig. b). Intraoperative ultrasound suggested that the mass occupying the lower margin of the pancreas was an abscess. Because it exhibited a caseous necrosis profile the sample was submitted for biopsy, and a necrotizing granulomatous lymphadenitis compatible with tuberculosis was observed (Fig. c and d). After obtaining these results microbiological analysis was performed, and Ziehl–Neelsen staining was suspiciously positive. Therefore, the diagnosis of LSPH caused by lymph node tuberculosis was confirmed. Based on the newly generated clinical evidence it was concluded that the patient’s diagnosis 7 years prior should have been peripancreatic lymph node tuberculosis rather than pancreatic cancer. |
A 31-year-old male patient visited Severance Hospital for a scalp and skull defect and uncontrolled wound infection (). Two years earlier, the patient had a decompressive craniectomy for traumatic subarachnoid hemorrhage and intracranial hemorrhage due to a motorcycle accident. Subsequently, drainage and insertion of an external ventricular drain for a subdural abscess were performed. Cranioplasty with cryopreserved autologous bone graft was performed 6 months after the trauma. The patient continuously scratched the surgical site with his hand, which resulted in a subsequent scalp wound and infection. The infected bone graft was removed, and the soft tissue defect was repaired with local flap advancement. However, surgical site infection and scalp defect recurred, and it was not improved with continuous wound care, debridement, and antibiotic treatment for 3 months. The patient was admitted to our hospital for infection control and reconstruction. At the time of the admission, his level of consciousness was alert, but he was quadriplegic and showed a low score (19) on the Mini-Mental State Examination. Previously grafted artificial dura was exposed, and Klebsiella pneumonia, methicillin-resistant Staphylococcus aureus (MRSA), and Acinetobacter baumannii were identified in wound cultures.\nSince resolution of the infection was a priority, removal of the exposed artificial dura, extensive debridement including infected bone, massive irrigation, duroplasty with Biodesign dural graft (Cook Medical, Bloomington, IN, USA), and defect coverage with LD myocutaneous free flap and split-thickness skin graft were performed (). Considering the high risk of infection, cranioplasty was planned to be performed at least 1 year after confirming resolution of the infection. The size of the LD flap and its skin paddle was 17×19 cm2 and 7×19 cm2, respectively. The facial artery and vein on the right cheek were prepared as donor vessels, and anastomosis was performed.\nNine days after the surgery, the skin graft became unstable, and necrosis of the flap was observed. MRSA and A. baumannii were reported from pus-like fluid accumulated under the flap. A detachment of the LD flap, debridement, duroplasty, and 17×19 cm2 sized anterolateral thigh (ALT) myocutaneous free flap transfer were performed, and the ALT flap was taken successfully. Vessel anastomosis was performed proximal to the same recipient vessels.\nThe reconstructed area initially had a convex shape due to a sufficient volume of the ALT flap. Six months after the second microsurgical surgery, flap sinking on the cranial defect area was observed (). However, since there was no evidence of neurologic deterioration, we decided to perform the cranial reconstruction following the originally planned schedule.\nThe cranioplasty was done with a patient-specific PEEK implant (Depuy Synthes; Oberdorf, Switzerland) at 1 year and 3 months form the second microsurgical reconstruction. The implant was designed with less curvature than of the contralateral side because we worried that the pedicle vessel would be damaged if the flap was stretched out too much. Furthermore, the implant was designed to match the irregular thickness of the reconstructed scalp flap observed in the planning computed tomography (CT) scan to achieve a smooth and concave head shape considering (). During surgery, previously grafted ALT flap was successfully separated from the dura with careful protection of the flap pedicle. Pedicle identification and flap monitoring using Doppler were performed periodically. After the skull defect was fully exposed, bone margin debridement, dura tenting suture, and implant fixation with titanium hardware were performed (). Finally, the flap was sutured to the scalp flap in its original position with minimal tension. The depressed external contour was improved after the cranioplasty and the postoperative CT scan identified sufficient brain re-expansion and normalization of ventricle sizes (). There was no evidence of complications such as infection, wound dehiscence, or fluid collection over the 1-year follow-up period (). |
A four-year-old girl presented to the emergency service with painful left hip and fever. There was no previous relevant medical history. There were no other local or systemic symptoms, except for a cervical adenopathy. On physical examination, she walked with a limp, and movements of the left hip were painful (mainly external rotation), but not restricted. Blood exam revealed anemia (Hb 8.7 gr/dL), normal WBC, ESB of 123 mm, and reactive C protein of 149.7 mg/L. An initial X-ray to the pelvis revealed no changes. An ultrasound of the left hip was performed revealing small infusion and synovitis. Guided puncture was then performed being macroscopically compatible with reactive arthritis, and general and bacteriological tests were demanded. Because of the unusual characteristics of the pain, a CT scan to the abdomen and pelvis was performed revealing a left adrenal mass and retroperitoneal adenopathies in the celiac trunk and superior mesenteric artery ().\nDespite the painful complaints of the patient, no bone or articular involvement was found in the CT scan. No further alterations were reported in the thoracic CT scan or in peripheral blood smears. Bacteriological examination of the hip effusion was negative. MRI was also performed. The direct myelogram was compatible with infiltration from neuroblastoma. Bone marrow biopsy and cervical adenopathy specimens were collected to perform histological diagnosis. Skeletal scintigraphy demonstrated numerous points of osteoblastic activity compatible with metastatic activity, and the 12 iodine-123 metaiodobenzylguanidine scintigraphy concluded the following: “Abdominal mass with low expression of noradrenergic transporters. Diffuse bone metastasization with high expression of noradrenergic transporters. No other soft tissue involvement was detected.” In the histological report of the cervical adenopathy, the diagnosis of neuroblastoma NOS was performed. Immunohistochemistry revealed extensive expression for synaptophysin and CD56 (NCAM) and absence of expression of myogenin. ().\nBone marrow biopsy revealed extensive metastatic involvement. The patient started chemotherapy two weeks after admission, with 8 cycles of rapid COJEC protocol. After six months of follow-up, the primary tumor was still without criteria for resection, despite a decrease in the metastatic involvement. Given the chemotherapy-related renal toxicity, it was decided to proceed with irinotecan in combination with temozolomide (TEMIRI). After thirteen months of follow-up, no significant regression of the primary tumor occurred, so surgery was contraindicated and the patient was proposed for stem cell treatment. |
A 65-year-old woman with gallbladder cancer was referred to our hospital for surgery. The laboratory examination revealed obstructive jaundice and cholangitis. Computed tomography (CT) showed gallbladder cancer involving the hepatic hilum, including the portal bifurcation. Preoperative cholangitis developed several times, and the endoscopic biliary stent was exchanged three times. The indocyanine green clearance was 0.113. The estimated volume of the future liver remnant was 507 mL and 31%. Percutaneous transhepatic portal embolization of the right portal vein was performed. Two months later, right hepatectomy, extrahepatic duct resection, and portal vein resection were performed (Fig. ). The left hepatic artery (LHA) was carefully exfoliated to the threshold of the hepatic parenchyma, but the bifurcation of the LHA to the segment 2 artery (A2) plus the segment 3 artery (A3) and segment 4 artery (A4) was not dissected; the tissue surrounding the LHA was difficult to dissect due to the inflammation wrought by preoperative cholangitis. Histological examination of the tumor showed moderately differentiated adenocarcinoma (pathological T4bN1M0, stage IV according to the Union for International Cancer Control classification of malignant tumors, 7th edition []).\nPostoperative blood examination showed slight elevation of liver enzymes and total bilirubin, and the patient had an uneventful postoperative course without liver failure or bile leakage. Screening CT on postoperative day (POD) 6 revealed a pseudoaneurysm of the LHA with a diameter of 6 mm (Fig. ). Angiography showed that the sac-like pseudoaneurysm was located on the bifurcation of the LHA to A2 plus A3 and A4 (Fig. ). Stent placement in the LHA or selective embolization of the pseudoaneurysm was considered for treatment. However, it seemed difficult to place the arterial stent because the LHA was too narrow and the pseudoaneurysm was located very close to the arterial bifurcation. We also hesitated to perform selective embolization because of the higher risk of migration of embolus material to the LHA. Therefore, we carefully followed up the pseudoaneurysm. However, CT on POD 15 showed enlargement of the pseudoaneurysm to a diameter of 10 mm; therefore, we decided to embolize the pseudoaneurysm to prevent rupture (Fig. ). As a cautionary measure, portal vein arterialization (arterioportal shunting) was planned to maintain the oxygen level of the remnant liver, even if the LHA was occluded by migration of the embolus material. The arterioportal shunt was constructed by anastomosing the ileocecal artery and vein under general anesthesia on POD 15 (Fig. ). Next day (POD 16), embolization of the pseudoaneurysm was successfully performed by selective injection of liquid thrombin without occlusion of the LHA (Fig. a). The postoperative blood data were within normal limits. Refractory ascites (3 L/day) developed thereafter, and portal hypertension was suspected as the major cause of the uncontrollable ascites. Twenty-one days later (POD 37), re-angiography confirmed complete embolization of the pseudoaneurysm and the patency of the LHA (Fig. b). Coil embolization of the arterioportal shunt was then successfully performed. The ascites was rapidly resolved and the patient was discharged on POD 45. |
A 68 year old gentleman was referred to the cardiology clinic due to an episode of chest pain which occurred whilst travelling from the UK to Australia via Singapore. He developed chest tightness after take-off in the UK which persisted continuously for the entire duration of the flight until it landed in Singapore on transit. When the flight touched down on ground, he noticed that the tightness had completely disappeared. It recurred after take-off and once again, persisted throughout the journey from Singapore to Australia. The pain was cardiac in nature. He has never smoked. A chest x-ray and a CT pulmonary angiogram done previously had not revealed any lung parenchymal disease. His cholesterol levels were 5.3 with raised low density lipoprotein (LDL) of 3.5. There was no significant past medical history except for benign prostatic hypertrophy for which he took Finasteride and Tamsulosin. Physical examination was unremarkable.\nHis resting electrocardiogram (ECG) revealed a normal sinus rhythm with normal cardiac intervals. His clinical examination was normal. Blood pressure in clinic was raised at 165/112 mmHg, however, a subsequent 24 h blood pressure monitor only revealed a mildly raised mean nocturnal blood pressure of 137/87 mmHg. Initially a CT coronary angiogram was organised due to the unusual presentation. This revealed significant stenoses in the proximal to mid left anterior descending artery (LAD) and the proximal right coronary artery (RCA) with a 50% stenosis in the circumflex and obtuse marginal artery. An invasive coronary angiogram confirmed critical proximal to mid RCA stenosis (Fig. ) and a long segment of proximal LAD stenosis (Fig. ).\nFractional flow reserve (FFR) measurement in the RCA was strongly positive (Fig. ) and the lesion was subsequently treated with a drug eluting stent. FFR measurement in the LAD was also physiologically positive and in turn this also treated with a single drug eluting stent. Following treatment he flew again with no recurrence of symptoms. |
We present a 57 year old gentleman with CKD 5 who had an autogenous brachiocephalic fistula 4 months prior to presentation to us. During their last surgical clinical visit, the fistula was noted to be poorly maturing and then referred to interventional radiology for fistulogram and possible endovascular intervention to assist with fistula maturation.\nThe patient had a fistulogram which demonstrated a high grade juxta-anastomotic stenosis which was successfully balloon dilated. After a 6 week follow up clinic visit the fistula was still immature and a duplex scan, a second fistulogram with possible intervention were requested.\nFistulogram was performed via an antegrade approach from an access just proximal to the swing point. There was an “apparent” stenosis (Fig. ) which was angioplastied then followed by severe spasm (Fig. ). which was perceived by the operator to be recalcitrant stenosis. In the light of this perceived recalcitrant stenosis, a decision to stent the area was taken. After measuring the vessel diameter based on the immediate post-plasty images a 6 mm diameter × 5 cm length Viabahn stent (Gore & Associates, Flagstaff, AZ) was selected and deployed in the standard fashion.\nFollowing stent deployment, the stent migrated and stopped at the confluence of the cephalic vein and the subclavian vein (Fig. ). At the time the operator thought the stent was stable and unlikely to cause harm to the patient in this position. However, after reviewing the images with colleagues including vascular surgeons a decision was made to attempt to retract the stent into the arm which would be easier for the surgeon to retrieve the stent surgically, if required. The patient was subsequently brought back 24 h later to the interventional radiology suite.\nInitial fluoroscopic image of the left shoulder region demonstrated the stent was absent from the final position documented the previous day indicating the stent had migrated further (Fig. ). Fluoroscopic scanning of the chest identified the stent to overlie the left lower lobe (Fig. ).\nSubsequent Pulmonary angiogram confirmed the stent to lie within a segmental pulmonary artery of the left lower lobe (Fig. ).\nAfter discussion of the options, risks and benefits with the patient and a multidisciplinary team, a decision to attempt stent retrieval was made versus leaving the stent in situ.\nAfter appropriate informed written consent, the right groin was prepped and in the standard fashion. Right common femoral vein access was then upsized to accept a 16 F sheath (Cook, Bloomington. IN USA). Main pulmonary access was then performed with an APC pulmonary catheter (Cook, Bloomington. IN USA). The APC catheter was then removed over a Storq wire (Cook, Bloomington. IN USA) wire and subsequently a 12 F 70 cm braided sheath was advanced into the main pulmonary artery and then left lower lobe pulmonary artery. Pulmonary angiograms performed identified the optimal projection to identify the vessel to access. After accessing the appropriate vessel the 12F sheath was advanced just to the origin of the branch above the stent. Subsequently a 15 mm Amplatz Gooseneck snare (ev3, Plymouth MN, USA) was manipulated until the stent was lassoed at about half way along the stent. Given the flexibility and potential collapsibility of the Viabahn stent it was over-sheathed carefully collapsing and gently retracting the captured stent to minimize potential vessel injury (Fig. , Additional file ). Once the stent had been totally ensheathed, the 12F sheath was retracted through the outer 16F sheath coaxially. The stent was retrieved intact (Fig. ).\nThe procedure was performed under moderate sedation using Fentanyl and midazolam with continuous monitoring of the patient’s vitals by a dedicated nurse. Throughout the procedure the patient remained hemodynamically stable with normal respiratory function. Post procedure the patient was observed for 6 h post procedure before being discharged home in a stable condition.\nThe patient has so far been followed up for 3.5 years and has not developed any adverse pulmonary or cardiac condition. Interval CTPA done at an outside facility showed normal pulmonary vasculature with no evidence of pulmonary vessel injury. |
A 28-year-old woman complained of pain and tumefaction in the upper portion of her right arm. She did not have fever, or trauma. Physical examination, showed a raised mass in the proximal portion of the right arm; with no clinical signs of neurovascular damage. There was no local erythema or skin lesions, and no palpable lymphadenopathy. There were pain and limitation of abduction, internal and external rotation of the right limb.\nThe remainder of the physical examination was normal. Radiographs revealed a diaphyseal pathologic fracture involving the right humerus with a periosteal reaction. Magnetic resonance imaging (MRI) of the right arm (Figure ) showed an eccentric mass in the diaphysis of the humerus, accompanied by an overlying periosteal reaction. The mass extended to a height of 11 cm, and into surrounding soft tissues. The shoulder and elbow appeared normal. There was no skip metastasis.\nA biopsy was performed. Microscopic examination showed fascicles of spindle cells with areas of collagen fibers, with an elevated mitotic index (Figure ). The microscopic aspect suggests the diagnosis of high grade FS of bone.\nA technetium-99 m labeled methylene diphosphonate radionucl ide (Tc 99 m/HMDP) bone scan revealed an area of increased uptake in the right proximal humerus, without other foci of abnormal isotope uptake that corresponded in location to the abnormalities visualized on MRI. Computed tomographic (CT) scanning of the thorax performed revealed no abnormalities. Laboratory test results were normal, left ventricular fraction ejection (FEV) was normal and equal to 69%.\nSurgical resection is the standard treatment of FS of bone. Early studies support the use of amputation []. At the Memorial Sloan-Kettering Cancer Center (MSKCC) more than 85% patients with histologically verified primary fibrosarcoma of bone, were treated by major amputation between 1918 and 1973. Nevertheless low-grade periosteal FS were treated by local wide excision rather than amputation, with encouraging results []. Also many studies have demonstrated a comparable rate of disease control and survival with amputation and Limb-salvage procedures, as long as wide resection margins are achieved, in the treatment of sarcoma of the extremities [,]. Furthermore conservative surgery improves the quality of life of patients with best functional results [,]. All this data encourage us to believe that a limb saving surgery should be seriously considered in the management of FS of bone.\nIn our case limb salvage surgery was not possible at the time of first presentation, and consequently neo-adjuvant chemotherapy was considered in order to ovoid amputation, and to achieve a wide surgical excision.\nThe role of chemotherapy in FS of bone is unknown. Up to now, no large chemotherapy studies of FS of bone are published; and only few case reports are reported. There is no recommendation regarding the optimal drug regimens, and the protocols used are formulated at the discretion of the medical oncologist, and were most commonly based on Adriamycin and cisplatin.\nAPI regimen is an active combination in the treatment of osteosarcoma (French Sarcoma Group FGS) [] with 37-47% of good pathologic response but there is no data concerning efficacy of this protocol in FS of bone.\nBased on this data, we use the API combination (adriamycin 60 mg/m2 and cisplatin 100 mg/m2 on day 1 and ifosfamid 1.8 g/m2/d during 5 days with Uromi thexan (Mesna®) 1800 mg/m2/d during 5 days) as neo-adjuvant chemotherapy. G-CSF (filgastrim) was administrated from day 7 to day 14 of each cycle.\nHematologic and non hematologic tolerance to chemotherapy was evaluated after each cycle, and we showed two episodes of neutropenia (grade III and I) and 1 episode of inter-costal Zona after the second course of chemotherapy, successfully managed with Valaciclovir.\nOur case showed excellent clinical and radiological partial response (Figure ) after 3 courses of chemotherapy.\nA conservative surgery was performed after 3 cycles; The patient received limb-salvage procedures with wide local resection of the tumor, reconstruction with humeral centromedullary nailing, and replacement of the excised segment of bone by cemented spacer.\nInterestingly, histological study of the specimen showed pathologic complete response of the tumor (Figure ), suggesting an important antitumor activity of API combination in FS of bone.\nThree post-operative courses of API were programmed; however, only 2 cycles of chemotherapy were administrated, and the treatment was discontinued because of serious adverse event (medullar aplasia that was successfully managed). |
An 84-year-old woman (5′6″; 144 lb) was referred to a cardiologist by her primary care practitioner for evaluation of exertional dyspnea and chest pain upon walking a few feet. Her medical history included hypertension, hypothyroidism, and osteoarthritis. Her medications included nebivolol, levothyroxine, aspirin, and citalopram. Cardiolite stress test revealed no ischemia, and a 2-dimensional echocardiogram demonstrated normal left ventricular systolic function with no wall motion abnormalities, no significant valvular heart disease, and no pericardial effusion. An electrocardiogram showed normal sinus rhythm, and no acute changes were noted.\nBased on her history of continued symptoms, the patient underwent 2 cardiac catheterizations. The first catheterization revealed normal right-sided heart pressures and 80% stenosis of the left circumflex artery and was followed by percutaneous coronary intervention. The second catheterization occurred 2 months after the first and revealed a patent left circumflex artery stent, but 70% stenosis of the right coronary artery, for which the patient again received percutaneous coronary intervention.\nThe patient continued to experience exertional dyspnea and chest pain, and she subsequently developed dysphagia to solid foods and episodic dizziness. Consultations with a pulmonologist, gastroenterologist, and otolaryngologist revealed no underlying cause for these symptoms apart from evidence of esophageal dysmotility. Her dysphagia persisted despite treatment with a proton pump inhibitor and a histamine H2 receptor blocker. A cardiac event monitor showed no evidence of significant bradycardia or tachycardia, although the patient reported 1 episode of low heart rate (35 beats per min).\nShe was subsequently referred to an electrophysiologist for evaluation of presumed bradycardia. Orthostatic evaluation found a supine blood pressure of 150/80 mmHg with a heart rate of 70 beats per min. Upon standing for 3 min, the patient’s blood pressure dropped to 110/74 mmHg with a heart rate of 76 beats per min. The magnitude of the drop in blood pressure met the diagnostic criteria for orthostatic hypotension (ie, a drop of 20 mmHg systolic or 10 mmHg diastolic), and the lack of an adequate compensatory heart rate increase upon standing was consistent with a neurogenic cause [, , ]. Tilt-table testing results were also consistent with a diagnosis of neurogenic orthostatic hypotension. The relationship between heart rate and blood pressure upon 60° head-up tilt in a normal individual versus an orthostatic response in a patient with neurogenic orthostatic hypotension is depicted in Fig. [].\nInitially, nonpharmacologic treatments were successful. These included high fluid and increased salt intake; small, frequent, low carbohydrate meals; the use of waist-high compression stockings; aerobic exercises targeted to the lower body; and elevation of the head of the bed. However, episodes of lightheadedness, chest pain, and dyspnea upon standing became more frequent over time. The patient was prescribed droxidopa (200 mg; 3 times daily) for treatment of her symptomatic neurogenic orthostatic hypotension. Droxidopa treatment significantly improved her symptoms, with the patient reporting resolution of her chest pain and significant improvement of dyspnea and dizziness. Six months later, the patient reported gait instability, and upon leaving the exam room, a shuffling gait and unequal arm swings were observed. She was referred to a neurologist and was diagnosed with Parkinson disease, for which she was prescribed carbidopa/levodopa. The patient continued to be followed through the collaborative care of both electrophysiology and neurology. |
The patient was a 65-year-old woman who presented with a 1-month history of bloody stool. A digital colonoscopy with biopsies revealed adenocarcinoma in the sigmoid colon. The patient elected to undergo primary laparoscopic colon resection, and the procedure was reported to be uneventful. However, on the 6th postoperative day, the patient noticed a large amount of yellow fluid coming out of a left side abdominal drain. The fluid appeared to be urine. An abdominal ultrasonography showed a collection of fluid in the patient's pelvis. A contrasted computed tomography (CT) scan showed contrast extravasation in the pelvis and around the descending colon (). The patient had decreased serum protein and albumin; however, complete blood count, creatinine, liver functions, and urine analysis were normal. On the 8th postoperative day, the patient was taken to the operating room for a ureteroscopy. The ureteroscopy revealed that the left ureter was completely severed about 4 to 5 cm from the ureteral orifice (, transected distal end of the ureter). The bowels could be seen through the ureteroscope (, intraabdominal cavity with bowels seen through the distal end of the transected ureter). No other obvious injury was identified. With patience, persistence, and some difficulty, the severed upper end of the ureter was identified and entered (, the proximal end of the transected ureter). We estimated that there was a 3- to 4-cm gap between the two ends of the ureter. Two 0.035″ guidewires were first passed, followed by the placement of two 4.5F Double-J ureteral stents. After placing the Double-J stents, the abdominal drainage quickly subsided. An abdominal ultrasonography 6 days after tube placement showed complete resolution of the abdominal fluid collection. The abdominal drain was removed and the patient was discharged. At the 3-month follow-up, a repeat CT scan showed no hydronephrosis, no abdominal fluid collection, and no contrast extravasation. The patient, however, had an asymptomatic urinary tract infection from Klebsiella pneumoniae, which was treated and resolved. A follow-up ureteroscopy over a guidewire showed excellent healing and realignment of the disrupted ureter. The only obvious sign of the transacted ureter was that the mucosa was paler than normal. Because of this unconventional treatment, we decided to continue stenting the ureter with two fresh 4.5F Double-J stents for an additional 4 weeks. Stents were removed 4 months after the injury. Follow-up contrast-enhanced CT scans taken 8 and 14 months after the initial endoscopic treatment showed mild but unchanged residual dilation of the renal pelvis and ureter with good drainage. There were no other abnormalities. |
The patient was a 21-year-old Caucasian female with a past medical history of uncomplicated laparoscopic appendectomy (1 month prior to the time of presentation), major depressive disorder, asthma, iron deficiency anemia, pelvic inflammatory disease secondary to sexually transmitted Chlamydia trachomatis infection, and SLE. She presented to the emergency department from an outside hospital complaining of severe right upper quadrant abdominal (RUQ) pain for one day that was non-radiating. She had been hospitalized for two weeks prior to the time of this presentation with a de novo diagnosis of SLE with the debut of GI symptoms after running out of her original prescription for steroids and hydroxychloroquine two days prior. Overall, her abdominal pain was accompanied by fever, nausea, vomiting, diarrhea, headaches, diplopia, generalized muscle weakness, and arthralgias. She denied vaginal bleeding or discharge. Physical examination was most significant for diffuse tenderness to palpation of the abdomen especially in the RUQ, a negative Murphy's sign, and well-healing surgical incision sites. Vital signs remained within normal limits. An erythematous facial rash with a butterfly pattern was noted as well as tenderness to palpation of the metacarpophalangeal and interphalangeal joints of both hands. The differential diagnosis at this point included a severe lupus flare secondary to medication shortage, acute cholecystitis, acute pancreatitis, Celiac disease, or enterotoxigenic Escherichia coli (ETEC or traveler's diarrhea). Blood work including complete blood counts and metabolic profiles were negative for any acute processes during her admission except for an elevated lipase level three times above normal. A urine pregnancy test was also negative. Stool tests for white blood cells, gram staining, ova and parasites, and Clostridium difficile were also negative. A CT scan of the abdomen and pelvis with contrast showed moderate wall thickening of the duodenum and proximal jejunum and moderate adjacent-associated inflammatory stranding most compatible with infectious/inflammatory enteritis (Figure ; obtained with consent from the hospital radiology department). Thus, we could safely rule out Fitz-Hugh-Curtis syndrome as well despite the patient's history of pelvic inflammatory disease.\nThe case was determined to be non-surgical based on the lack of acute abdomen on exam and negative imaging findings. Gastroenterology planned for upper endoscopy (esophagogastroduodenoscopy, EGD). Rheumatology ordered autoimmunity standard tests and started the patient on pulse-dosed intravenous (IV) methylprednisolone (which was transitioned to oral prednisone after three days), oral hydroxychloroquine, and low-dose lisinopril once daily for scleroderma renal crisis (SRC) prophylaxis. EGD showed edematous mucosa in the duodenum and jejunum without active bleeding, gastropathy, or ulceration (Figure ; obtained with consent from the hospital endoscopy department). Biopsies obtained from the EGD were non-specific (Figure , obtained with consent from the hospital pathology lab). The autoimmunity standard tests including anti-nuclear antibody (1:2560 titer), double-stranded DNA (1:80 titer), C3 (45 g/L), C4 (4 g/L), and anti-Smith (175 AU/mL) were strongly positive. Antiphospholipid antibodies were negative, as were anti-transglutaminase (TTG) antibodies for Celiac disease. This confirmed the diagnosis of a severe flare of lupus; more specifically, lupus enteritis secondary to medication shortage was determined to be the principal cause of the patient's abdominal pain. The patient continued to have intermittent bouts of moderate to severe periumbilical abdominal pains and non-bloody diarrhea that were treated with supportive care. Her symptoms responded very well to treatment by day four, and she had no further complications during her hospital stay. |
A 40-year-old woman with a past medical history of familial hypercholesterolemia presented to the emergency room complaining of pain localized to the left second middle and distal phalanges, left third distal phalanx, and right third distal phalanx following a thermal burn from exposure to LN2. The patient works in research and accidentally dropped a pair of rubber gloves into a container with LN2. She instinctively reached into the container to retrieve her gloves and her fingers contacted the LN2 for approximately 30 s. She immediately noticed pain of 8/10 severity in her left index and middle fingers as well as her right middle fingertip. Upon examination, her left index and middle fingers (Figures -) and her right middle fingertip showed clear blisters. The regions were well perfused. She denied any other symptoms. Bilateral X-rays of the hands and X-rays of the affected fingers showed no acute bony abnormalities. The emergency room team contacted poison control who recommended infusion with NAC. The emergency room began a 16-h IV infusion of NAC 200 mg/mL (20%) 4500 mg in 5% dextrose solution and consulted both orthopedic surgery and vascular surgery, who did not recommend surgical intervention. They chose to admit the patient overnight for observation.\nThe patient was discharged the next morning after receiving one dose of NAC. At this time, her fingers were swollen and erythematous. Orthopedic surgery recommended a 10-day course of 100 mg oral doxycycline two times per day and expedient follow-up in clinic.\nAt follow-up four days following discharge, she continued to report pain and throbbing in the affected fingers. On examination, her fingers remained swollen and blistered. Her motor and sensory exam was normal with the exception of loss of sensation to the left index finger distal phalanx in the area of her blister. Capillary refill remained brisk for less than three seconds. The left index finger blisters were lanced with a 15-blade scalpel in clinic, and the patient was given instructions to apply mupirocin ointment two times daily and to follow-up in clinic in one week for another skin check. She continued to return every one to two weeks for regular skin checks. Subsequent exams showed decreased discoloration and swelling as well as improved sensation and range of motion.\nThe patient was contacted at six months post-injury for final follow-up. She stated that she felt great and had completely normal range of motion. She also stated she had no functional limitations and was able to complete activities such as typing or playing the piano without any trouble. Bilateral DASH scores were obtained, resulting in a score of 0/100 for the right hand and a score of 3.3/100 for the left hand. Images of the patient’s left hand at six months of follow-up are shown in Figures -. |
Case 1 was that of a 71-year-old woman who had a history of rheumatoid arthritis and was taking methotrexate, bucillamine, and salazosulfapyridine. She was diagnosed with dry eye by a local physician and prescribed rebamipide ophthalmic suspension to be applied four times a day, which she did for ~4 years until March 2018. In early April 2018, the patient experienced pain and pruritus around her left eye and in mid-April 2018 visited a general practitioner, who prescribed anti-allergic eye drops. Five days later, she experienced severe pain and swelling around her left eye and visited Tsukazaki Hospital for treatment.\nThe patient's visual acuity on the first visit was 20/20 for the right eye; the visual acuity of the left could not be measured because the eyelid was difficult to open. The intraocular pressures were 8 mmHg for the right eye and 13 mmHg for the left eye. Redness and swelling were observed around the nasal side of her left lower eyelid (). Plain computed tomography performed on the same day revealed a high-density mass in the lacrimal sac, ocular cellulitis, and ocular deformation due to an orbital abscess (). On the same day, an incision was made on the lower orbital margin and the intraorbital abscess was drained. Klebsiella pneumoniae, Streptococcus anginosus, Enterococcus faecalis, and Peptoniphilus asaccharolyticus were isolated from the abscess culture. Daily infusion of ceftriaxone sodium hydrate 1 g was initiated. The next day, her left visual acuity was 20/200. Slight Descemet membrane folds were found in the cornea of the left eye; no inflammation was noted in the eye and the fundus was normal. Computed tomography showed recovery of the left eyeball morphology, but the lacrimal sac concretion was still present.\nTwo days after her first visit, the vision in her left eye improved to 20/20; the swelling around the eye reduced as well. Three weeks after her first visit, endonasal dacryocystorhinostomy (DCR) was performed on the left side and an 11 × 8 mm lacrimal sac concretion was removed. After the surgery, no symptoms of infection were observed and the postoperative course was uneventful. Light and fluorescence microscopy of the lacrimal sac concretion revealed unstained amorphous crystal-like structures, a high bacterial count, and yeast-like fungi. Scanning electron microscopy with energy dispersive X-ray spectroscopy (SEM-EDX) revealed calcium phosphate crystals with a sharp edge (). Infrared spectrophotometry revealed that the spectra of the lacrimal sac concretion demonstrated specific infrared absorption bands near 3,280, 1,644, 1,602, 1,540, and 760 cm−1, which overlapped with the characteristic peaks of rebamipide (). High performance liquid chromatography (HPLC) also revealed that the lacrimal sac concretion had a rebamipide content of 33.3%. |
The patient was male, 56 years old. He was diagnosed as primary liver carcinoma complicated with a 20 years history of HBV-related liver cirrhosis, 3 years of hypertension, and 2 years of type II diabetes. He suffered with 3 times of transcatheter hepatic arterial chemoembolization, 1 time of percutaneous transhepatic sonographically guided radiofrequency ablation of liver cancer, and 1 time of laparoscopic microwave coagulation in Couinaud segment I due to uncontrolled liver carcinoma. The AFP was 36.13 μg/L. HBV DNA was lower that 100 IU/mL. Finally, he had successful LT from a donation after cardiac death donor. The preoperative model for end-stage liver disease score was 7. The operative course was uneventful.\nThe patient received tacrolimus-based immunosuppression with the serum level of 8 to 12 μg/L. Basiliximab (20 mg, Simulect) was used intravenously on day 0, 4 postoperative respectively as an immune induction therapy. Entecavir and hepatitis B immunoglobulin were used regularly to prevent hepatitis B recurrence. As the vascular anastomosis was satisfying, initial anticoagulation was not in regular usage postoperation.\nOn day 8 postoperation, ALT and AST, the indicator of graft function, were getting almostly normal. He suffered with hypoproteinemia that needed 2 to 3 doses of albumin to keep ALB at normal range. But the patient started to complain of edema in both lower limbs and scrotum. Draining fluid from abdominal cavity was getting more than before. Both collateral arteries and venous of lower extremity were subjected to ultrasonic examination by Doppler method, which showed as patent blood flow without embolism. However, Doppler ultrasound of the liver graft presented as thrombosis in the right portal vein and nubilous posthepatic inferior vena cava (IVC) on day 11 after transplantation. Contrast-enhanced ultrasound was further adopted to confirm the thrombosis with a size of 4.5 cm × 1.5 cm in the right portal vein. The liver graft was getting larger and the oblique diameter of right lobe of liver was 18.5 cm. The peak velocity right hepatic artery was 172.5 cm/s, resistent index (RI) 0.54, while the peak velocity left hepatic artery was 62.4 cm/s, RI 0.66.\nUrgent angiography under local anesthesia was performed to identify the blood flow of IVC. Transcutaneous transfemoral catheterization portography revealed no obstruction of IVC and hepatic veins trunks. At the same time, we were trying to get rid of thrombosis in the right hepatic portal vein. We then decided to treat with indwelling catheter thrombolysis as the first therapeutic option. Since there was no dilated bile duct in the graft, it was difficult to guide therapeutic percutaneous puncture catheter to portal vein through interventional method. Therefore, we chose the ultrasound-guided transcutaneous transhepatic puncture to place a hydrophilic 0.035-inch guidewire (Terumo, Tokyo, Japan) to the portal trunk. Then, a 5-Fr sheath introducer (Medikit) was placed through the guidewire with tip close to the superior mesenteric vein under direct vision of the interventional therapy. Contrast agent was injected from the sheath introducer to the portal vein, and a lateral view of the DSA confirmed complete occlusion of the right portal trunk with development of varicose veins in the portasystemic collateral vessels (Fig. ).\nInfusion catheter (4-Fr Fountain Infusion System; Merit Medical OEM) was introduced into the portal trunk through percutaneous transhepatic tract (Fig. ). Thrombolytic therapy was initiated with continuous infusion of urokinase (10000 U/kg/day) and low molecular weight heparin (4000 U/day). Closed observation was maintained regarding bleeding at the site of puncture and the entire body, especially gingival hemorrhage.\nA Doppler sonography performed 3 times per week after initiation of thrombolytic therapy demonstrated gradually reduced embolism of right portal trunk. Contrast-enhanced ultrasound was used to recheck the state of thrombosis in the right portal vein after 3 days of thrombolysis therapy. The thrombosis shrinked at a size of 0.7 cm × 0.6 cm. Although the oblique diameter of right lobe of liver was the same size, the peak velocity right hepatic artery was getting lower as 79.7 cm/s. Two weeks later, ultrasound examination showed that the oblique diameter of right lobe of liver was 16.6 cm. There was no thrombosis in PV and patent blood flow of hepatic artery with a peak velocity of 56 cm/s, RI 0.65.\nA follow-up DSA with contrast injection from the infusion catheter 2 time per week after initiation of thrombolysis procedure revealed gradually patent portal vein trunk (Fig. ). The catheter was removed after 2 weeks of thrombolytic therapy, and vascular blockage was proceeded for hemostasis.\nAfter treatment with above-described interventional radiology methods, the patient discharged after recovery with anticoagulation therapy with bayaspirin (100 mg/day) for a long-term follow-up. Until this paper submission, the patient has been followed with normal graft function and patent portal vein for 22 months. Informed consent was obtained from the patient prior to treatment. |
A 50-year-old male patient presented with a 2-year history of left-sided typical HFS. Painless irregular clonic contraction of the facial muscles began initially in the orbicularis oculi muscle of the lower lid. It gradually spread to other muscles innervated by the facial nerve on the left side of the face, including platysma. The paroxysm was induced or aggravated by emotional tension, stress, and voluntary and reflexive movements of the face. He had significant difficulty in his work and social life despite 2 times of botulinum toxin injection. Medical treatment with carbamazepine (up to 600 mg) and baclofen (30 mg) was not effective. He was referred for surgical treatment. His medical history was unremarkable. His physical and neurologic examinations were normal, including hearing. No tinnitus or discernible noise heard in his left ear was found. Only typical nature of clonic hemifacial spasm was evident. Abnormal synkinesis between the orbicularis oculi and orbicularis oris muscles was found by the electromyographic examination of the blink reflex. Despite typical HFS, there was no discernible vascular structure in the REZ of left facial nerve (). However, a meatal loop of AICA abutting to the cisternal portion of the facial nerve was found.\nUnder the impression of HFS caused by neurovascular compression of distal facial nerve, standard microsurgical procedure was performed as described previously [, , ]. In addition to intraoperative monitoring of BAEPs, LSR, which is an abnormal muscle response demonstrated by EMG recordings from mimic muscles that are innervated by a different branch of the facial nerve [], was also monitored throughout the operation. The entire course of the facial nerve and offending arteries were exposed under microscopic vision. Upon exposure of the REZ of the facial nerve, there was no offending vessel in the REZ as expected (). The distal, cisternal segment of the facial nerve was found to be bent by a meatal loop of the AICA (). A small piece of Teflon felt was interposed between the facial nerve and the meatal loop of the AICA with extreme care not to stretch the internal auditory artery and the distal facial nerve (). After interposition of Teflon felt, LSR immediately disappeared and BEAP was stable also (). The closure of the dura and wound was performed in routine manner. The HFS resolved completely following the surgery. The postoperative course was uneventful with no signs of facial weakness or hearing impairment by pure-tone audiometry. No recurrence of HFS or neurologic sequelae was evident at a 12-month follow-up. |
A 9-year-old boy had an increase in abdominal volume associated with nonspecific abdominal pain and fever for 2 months, evolving without improvement of symptoms, so he was admitted for investigation presenting a swelling in the left foot. After 3 weeks the patient presented a painless swelling of frontal region, without other signs of inflammation or cervical lymph node enlargement. Painful swelling also appeared in the left ankle. There was no epidemiological history of TB. TST with PPD Rt23 was 11 mm and the HIV-test was negative. Chest X-ray was normal; chest CT revealed the presence of 1.1 cm lymph nodes in the pre-tracheal region, 0.8-1.1 cm near the right anterior costophrenic sinus and other smaller lymph nodes in anterior mediastinum and infracarinal region. In addition, there was a small right pleural effusion without lesions in pulmonary parenchyma. Ultrasonography and abdominal CT performed one week before revealed the presence of a moderate ascite, enlarged liver (regular contour and homogeneous density), small lymph nodes along the right common iliac vessels and 1.9 x 1.2 cm nodular image next to the left diaphragmatic dome (probably a lymph node). The cranial CT showed a frontal lytic lesion in the midline measuring 2 cm and a thickness of 0.7 cm; the internal bone layer was intact and there was a rupture of the external plate extending beyond the extra-cranial layer (). The extracranial segment had a soft tissue density of 1.6 x 0.7 cm, with normal cerebral parenchyma, ventricular system and cisterns. The skull biopsy showed a chronic inflammatory process with granuloma and multinucleated giant cells; Ziehl Neelsen, Groccot and PAS staining did not show microorganisms. A left ankle synovium biopsy was also performed. The Xpert MTB/RIF test on synovium material for Micobacterium tuberculosis complex was positive. The patient underwent an immunological investigation but it was not possible to show an altered functional assay for the IFN-g/IL12 axis, with no response to either cytokine.\nThe patient received RHZ regimen to treat TB for 9 months with complete frontal mass regression. He maintained the small joint movement restriction with limited left ankle flexion. He improved his weight significantly and completed treatment after 9 months. The frontal mass disappeared, maintaining the small joint restriction with residual limitation of left ankle flexion. There was an important weight gain. |
A 45-year-old man sustained a posterior dislocation of his left shoulder following a convulsive seizure while he was playing soccer. He did not have a history of any previous injury or symptoms regarding his shoulder. X-rays and CT showed the joint fixed in dorsal dislocation with the presence of a reversed Hill-Sachs defect affecting 30% of the articular surface (). Behind the background of the literature, the decision for a surgical approach was made [].\nThe patient was placed in beach chair position with an image intensifier perpendicular to the patient's axis from the contralateral side to allow arthroscopy and radiographs simultaneously. Under general anaesthesia, a closed reduction of the glenohumeral joint was performed and standard arthroscopic portals were placed. At the anteromedial humeral head the reversed Hill-Sachs lesion was visualized (); the findings were in accordance with the preoperative X-rays and CT scans (). No more injuries were found in the anterior shoulder region with an intact tendon of the subscapularis, the biceps brachii tendon, and an intact labrum. Through a lateral approach a k-wire was placed into the central defect of the Hill-Sachs lesion (); it can be helpful to guide the k-wire by a tibial guide used in cruciate ligament surgery (Arthrex, USA). Guided by the k-wire a cannulated sizer (8 mm diameter, BioMatrix CRD instruments, Arthrex) was inserted for reduction of the fracture under arthroscopic visualization (). For internal fixation the subcortical defect was filled with an injectable bone substitute (Cerament) to prevent secondary dislocation. After hardening of the bone substitute the reconstruction of the humeral head was documented by arthroscopy (). Wounds were closed and the portals were covered with sterile dressings.\nX-ray and CT scan of the shoulder two days after the index procedure showed an intact glenohumeral articulation with a restored humeral head and a subcortical defect filled up with Cerament (Figures and ).\nThe shoulder was braced in neutral rotation for 2 weeks postoperatively and easy functional physiotherapy was started.\nX-ray at follow-up 6 month after the index procedure documents the bony remodeling of the bone substitute (). At that time the patient was pain-free (VAS 0) and satisfied with the outcome (Constant score: 78, Rand-36 score: 84, Rowe score: 81) with a good ROM with 90° abduction and 110° elevation (Figures and ). |
A 6-month-old baby girl, a product of a monozygous twin pregnancy and vaginal delivery, was referred to the plastic surgery department with a large right-sided craniofacial mass. The mass was noted immediately at birth and had slowly grown. The mass did not cause respiratory obstruction and did not obscure the visual field. Postnatally, the baby was diagnosed with a cardiac ventricular septal defect and a patent ductus arteriosus, for which she underwent an uncomplicated open banding procedure at the age of 2 months. The family reported normal development of the child and no concerns regarding milestone achievements compared to her fully healthy twin sister.\nOn clinical examination, she had a large right-sided irregular “elephant trunk-like” lesion measuring 10×12 cm that extended from the right medial canthal area to the right side of her oral commissure. The cephalic component of the mass was soft and compressible, but the caudal segment had a bony block, as shown in . The mass completely obstructed and distorted the right nasal airway. The child was able to open her eyes fully. She did not have any intraoral lesions. Intranasal examination showed a bony bar stemming from the inferior turbinate and obstructing the right nasal airway passage. She had a mild hypertelorism. The rest of the head and neck examination was normal. A detailed pediatric ophthalmological assessment showed normal visual acuity and fields, normal pupillary responses, and normal intraocular findings.\nComputed tomography (CT) and magnetic resonance imaging (MRI) showed an isodense mass centered in the floor of the anterior cranial fossa measuring 30×30×26 mm, with herniation of the mass through a right-sided bony defect in the roof of the ethmoidal sinus. The mass then extended to the atretic right-side nasal cavity. There was also a tubular structure in the right atretic nasal airway, with a bony solid block stemming from the right maxillary process into the paracentral proboscis. Right-sided choanal atresia was found, with obliteration of the right-sided nasopharyngeal opening by the bony block. The right-sided maxillary sinus was noted to be hypoplastic. shows a coronal view of the mass.\nThe child underwent resection of the lesion, performed by craniofacial surgery and neurosurgery teams. The cranial component of the tumor was approached and exposed through bicoronal bifrontal craniotomy. After opening the dura, the anterior third of the superior sagittal sinus was ligated. This allowed adequate retraction of the anterobasal part of the frontal lobes and exposure of the lesion. The tumor was found to be soft, cheese-like, and vascular.\nThereafter, the extradural part of the residual lesion was pushed through the right ethmoidal bony defect into the right nasal cavity. The bulk of the facial component (proboscis lateralis) was removed via an upper medial canthal incision extending to the lateral nasal and nasolabial skin crease line. This provided adequate exposure for the upper nasal component of the tubular tumor, as shown in . The bony block stemming from the maxilla was trimmed and a right nasal tunnel was created.\nFinally, the cranial bones were fixed and bicoronal flaps were closed in layers. Two cranial drains were inserted. The facial incisions were closed after achieving adequate hemostasis. The operation lasted 14 hours and the estimated blood loss was 1,000 mL. The child was transfused with eight units of red blood cells, four units of platelets, and four units of fresh frozen plasma. The child tolerated the operation relatively well, with one episode of hemodynamic instability.\nPostoperatively, the child was monitored in a pediatric intensive care unit. Her postoperative recovery was complicated by a persistent leak of cerebrospinal fluid (CSF), episodes of respiratory distress, and central diabetes insipidus (CDI). The CSF leak resolved with acetazolamide and conservative management. She was extubated successfully on day 8 postoperatively and discharged home on day 14. On discharge, she was still suffering from CDI, and was managed by the pediatric endocrinology team.\nHistopathological examination of the resected specimens showed fibrofatty tissue with bundles of smooth and skeletal muscle sheets and fibroblastic cells. The overall appearance of the resected tumor was consistent with a cystic craniofacial teratoma.\nThe child was followed up in the plastic surgery clinic at 6 months postoperatively. Head and facial CT scans showed a small residual intracranial tumor. Clinically, her nasal obstruction had resolved and the right nasal airway remained patent. The child is scheduled for further facial procedures to correct the right-sided nasal deformity at the age of 2 years. Furthermore, she will require a procedure to correct hypertelorism at the age of 8 to 10 years. |
A 55-year-old woman presented three days after a sudden onset of right-sided chest pain, pleuritic and positional in nature, associated with an acute onset of shortness of breath. She had gone to her primary care physician, who performed a chest X-ray and urged her to come to the hospital. Upon presentation at the emergency department, her oxygen saturation was above 95% on room air, and she was not in any respiratory distress, but her exam was significant for decreased breath sound on the right. A chest X-ray confirmed a large right-sided pneumothorax with small pleural effusion. A chest tube was inserted on the right side for the resolution of the pneumothorax, and subsequent computed tomography (CT) scan of the chest revealed bilateral diffuse bullous disease of the lung with multiple cysts (Figure -). The patient underwent video-assisted thoracoscopic surgery for right thoracoscopic wedge resection of a lung bleb and talc pleurodesis. Gross examination of the specimen revealed several dilated air-like spaces ranging from 0.2 cm to 0.4 cm in size. The hospital course was complicated by postsurgical pneumonia, but she recovered fully and was discharged to home with only minimal symptoms of dyspnea on exertion. Upon further investigations, she was found to have multiple small lesions of angiomyolipoma on the right kidney with diffuse retroperitoneal lymphadenopathy. One of the lymph nodes was biopsied, and pathology revealed predominantly spindle cells positive for HHF35 and smooth muscle actin, consistent with the diagnosis of leiomyoma. At the eight-month follow-up at the pulmonology clinic, her pulmonary function test (PFT) showed normal vital capacity and forced expiratory volume in one second (FEV1), but moderately reduced diffusion capacity, which may also be related to LAM. At her 12-month and 24-month follow-up visits, her PFT results showed improvements in peak flow and diffusion capacity, and the patient continues to report no symptoms other than minimal dyspnea on exertion. |
A 35-year-old male presented with a 6-month history of the appearance of multiple discrete fluid-filled lesions over erythematous skin, of size around 0.1–0.2 cm, which gradually increased to a size of around 1–3 cm. The lesions first appeared over the trunk and gradually over other parts of the body. The lesions used to rupture after 5–6 days leaving behind raw erosions, which were nonspreading in nature. The erosions used to get crusted in the next 4–5 days which fall off leaving behind light-colored skin. There was no history of any drug intake before the appearance of the lesion. On cutaneous examination, few tense bullae of size around 0.5 cm were found to be present in the perineal region []. There were multiple healed hypopigmented lesions with perifollicular hyperpigmentation, with some lesions showing crusting over them. A peculiar finding of the distribution of lesions was that the lesions covered the whole body but spared a band-like area in the hip region both in anterior and posterior aspects []. Oral mucosa was not involved. Remaining systemic examinations were within normal limit. A provisional diagnosis of bullous pemphigoid with the reverse Koebner phenomenon was made with the probable cause of sparing being the tightness of clothing in that particular area. Suspecting the same biopsy was sent for histopathology and direct immunofluorescence from both the lesion and the spared area. The histopathology of the lesion showed subepidermal split with a mixed inflammatory infiltrate, which was composed of predominantly eosinophils, neutrophils, and lymphocytes [], but the histopathology of the spared skin showed no abnormalities []. However, the direct immunofluorescence from the perilesional area showed linear deposits of immunoglobulin G and C3 in the dermoepidermal junction [], but the direct immunofluorescence from the spared area showed no deposits. Indirect immunofluorescence and salt splitting could not be done because of resource constraints. Rest of the laboratory investigations were within normal limits. As the diagnosis of bullous pemphigoid was confirmed, the patient was started with oral prednisolone and oral dapsone. |
A 69-year-old Caucasian woman presented with a 2 months history of foreign-body sensation in the throat. She had a negative history for tobacco and alcohol use. The patient underwent regular gynecological examinations and cytological cervical cancer screenings until the age of 64, and no HPV-associated genital lesions or cytological alterations were found. No anogenital tumor had become symptomatically apparent since then and extending also into the follow-up period after treatment of the index throat condition. The patient got infected with hepatitis B virus (HBV) by blood transfusion at the age of 26, and had been treated for lichen ruber planus in the oral cavity at the age of 34. The ENT (Ear Nose and Throat) physical examination revealed an enlarged left tonsil without ulceration. On palpation, the left tonsil was found to be firm to hard in consistency. She did not have any palpable cervical lymphadenopathy. A computed tomography (CT) scan with contrast of the neck confirmed a left tonsillar lesion of 20 × 23 × 26 mm in dimension, extending up to the soft palate, without crossing midline, and down to the glossotonsillar sulcus, and revealed two homogeneously enhancing lymph nodes, with maximum transverse diameters of 13 mm, in the left submandibular triangle. Baseline investigations including complete and differential blood cell count, serum electrolytes, liver and renal function tests, electrocardiogram, and X-ray chest, were well within normal limits. The patient underwent wedge biopsy of the left tonsillar mass. Pathologic review revealed the presence of a poorly differentiated SCC with basaloid features.\nThe patient underwent left side mandibulotomy, extended radical tonsillectomy and ipsilateral selective neck dissection (levels I-II-III-IV). The surgical defect was reconstructed with a microvascular ulnar forearm flap. Final pathology confirmed an infiltrating, poorly-differentiated SCC of the left tonsil, with tumor measuring 28 mm in greatest dimension and a thickness of 14 mm, abundant comedo-type necrosis and the presence of both vascular and perineural invasion. Histopathological examination of the neck dissection tissue showed an enlarged lymph node with intracapsular metastasis at level II (pT2N1). To minimize the risk of locoregional recurrence the treatment was completed with adjuvant external beam radiation therapy (EBRT), which started 7 weeks after surgery. Radiation treatment targeted the tonsillar bed and the left cervical region. A dose of 60 Gy was delivered with conventional fractionation (2 Gy/fraction, once daily, five times weekly). The HPV molecular data were not available at the time of clinical diagnosis and tumor treatment. After 1 year of follow-up, the patient is well with no evidence of recurrent cancer.\nAnalyses were performed in fresh-frozen tumor biopsy, formalin-fixed paraffin-embedded (FFPE) tumor tissue and serum. All samples were collected at the time of initial tumor diagnosis before surgery and radiotherapy.\nGenomic DNA was extracted from sections of fresh-frozen and FFPE tissues following previously described procedures [,,]. More than 80% of the cells in hematoxylin eosin-stained adjacent sections were neoplastic.\nHPV genotyping was performed by BSGP5+/6+ −PCR/MPG [-], an assay capable of amplifying about 150 base pairs (bp) of the L1 gene of 51 HPV mucosal types, followed by hybridization to type-specific probes on fluorescent suspension array beads. It includes detection of the human β-globin gene as DNA quality control as well as internal PCR and hybridization controls. Sample DNA was also analyzed by MY09/11-PCR amplification followed by both Restriction Fragment Length Polymorphism (RFLP) analysis and direct sequencing [].\nGenomic DNA extracted from both fresh-frozen and FFPE tumor tissues contained HPV58 as single mucosal HPV infection, as shown by both mucosal HPV genotyping methods.\nTotal RNA was obtained from FFPE tissue sections using the Pure-Link FFPE Total RNA Isolation Kit (Invitrogen, Carlsbad, CA) following the manufacturer’s protocol. Viral RNA expression was analyzed by E6*I reverse transcription (RT)-PCR []. Briefly, the HPV type-specific assay generates 70 bp long cDNA amplicons across the E6*I splice site that are subsequently detected by hybridization to type-specific and splice site-specific probes on fluorescent suspension array beads. A duplex assay was performed for RNA of HPV16 and the cellular housekeeping gene ubiquitin C (UbC), and a singleplex assay for HPV58. The sample was positive for the HPV58 E6*I and UbC transcripts and negative for the HPV16 E6*I transcript.\nThe HPV antibody status was analyzed by a glutathione S-transferase (GST) capture immunosorbent assay using as antigens full-length HPV proteins bacterially expressed as GST fusion proteins [,] in combination with fluorescent suspension array beads as previously described [,,]. Antibodies to the E6 and E7 oncoproteins of high-risk HPV types 16, 18, 31, 33, 45, 52 and 58 and low-risk types 6 and 11 were quantified. Positivity was high for E6 and E7 of HPV58. Weaker signals were observed for E7 of the closely related HPV types 31 and 33 and are interpreted as cross-reactivity with the HPV58 E7 protein (Figure ).\nThe expression level of the cellular proteins p16INK4a and pRb, markers for HPV transformation, were evaluated by immunohistochemistry (IHC). The monoclonal antibodies CINtec (V-kit, MTM laboratories, Heidelberg, Germany) and NCL-RB (Novocastra, Newcastle, UK), were used for p16INK4a and pRb, respectively. Well characterized sections from CxCa and a healthy mucosa were used in each staining batch as reference for scoring of the protein expression levels for both markers. IHC was evaluated independently by two investigators. The tumor cells of the sections showed strong nuclear and diffuse cytoplasmic staining of p16INK4a (>95%) and low staining of pRb (<25%), while proliferating basal and parabasal cells of the tumor-adjacent mucosa showed low p16INK4a (<5%) and high pRb (>25%) expression (Figure ).\nTP53 mutation status was determined in genomic DNA isolated from the fresh-frozen biopsy. Exons 4–10 were amplified using specific primer pairs for each exon as described in the IARC protocol []. Amplification products were sequenced using the ABI PRISM® 3730XL Genetic Analyzer (Applied Biosystems, Foster City, CA). No mutations or polymorphisms were observed. |
A 20-year-old competitive male soccer player, who presented with a two-year history of recurrent bilateral groin pain and stiffness, was admitted to our institution. Bilateral examination showed pain in hip flexion, adduction, and internal rotation (anterior impingement test); abduction and external rotation were normal. The patient had bilateral hip flexion up to 100° and internal rotation of 0°. Plain radiographs of the pelvis showed a well maintained joint space and a combined type FAI image. The alpha angles were 78° for the right side and 76° for the left side; the lateral centre edge (LCE) angle was 45° on the right and 47° on the left. Both hips showed a large superior rim fracture ().\nA magnetic resonance arthrogram showed FAI and an anterosuperior bilateral labral tear (). A bilateral hip test with intra-articular lidocaine was positive (showing an intra-articular and labral pain). The patient was then treated with an arthroscopic procedure of each hip, with a three-month gap period in between them both. The out-in hip arthroscopy approach described by Sampson [] was used in both surgical events. A labral tear associated with a large superior fracture of the bone rim was found; the cartilage at the junction with the labrum was intact in both hips. Pincer resection was performed with power instruments, which included about 30% of the fractured segment. This was performed under X-ray supervision and the remaining bone fragment was secured with an arthroscopic assisted 3.0 mm cannulated screw () in both sides. Finally, the labral fixation was secured with 3 translabral suture anchors and an arthroscopic cam resection of the femoral neck was performed. The final examination of the hip motion under arthroscopic vision probed no impingement at all.\nThe patient was restricted to foot flat weight bearing and two crutches for 6 weeks and progressed back into soccer at 4 months postoperatively. Physiotherapy was indicated starting the 5th day after surgery. At two-year follow-up, he had no pain during soccer or other athletic activities, the hip impingement signs were negative, and the range of motion of his hips had improved to 120° of flexion and 30° of internal rotation. His modified Harris Hip Score showed a variation from 81.3 to 100 points on the right and from 87.1 to 96.1 points on the left, and his visual analogue pain score decreased from 8 to 1.\nRadiographs at follow-up taken at six months showed that the rim fracture healed. The LCE angle had decreased from 45° to 32° postoperatively in both hips and the hip joint space was still well maintained (). |
A 6-year-old girl presented to Tongji Hospital affiliated to Tongji Medical College, Huazhong University of Science and Technology, with 1-week history of gradual painless proptosis of the right eye. Ophthalmologic examination revealed 20/20 visual acuity in both eyes, no conjunctival congestion, no afferent pupillary defect, and a normal fundus. The ocular protrusion was measured by Hertel exophthalmometry. Results showed 18 mm for the right eye, 13 mm for the left eye, and the interorbital distance was 90 mm. A well-defined, nonmobile, nontender soft mass 4 cm × 3 cm in size was palpated in the temporal portion of the right orbit. The mobility of the right eye was limited in upgaze and lateral gaze. An orbital computed tomographic (CT) scan showed a uniform density soft-tissue mass in the right lateral orbital wall area extending into the orbit, the intracranial, the temporal fossa, and the adjacent soft tissues. There was no clear boundary between the mass and lateral rectus, and the neighboring orbital wall was destroyed (). Contrast-enhanced CT combined with CT angiography showed a nonuniform enhancement soft mass 4 cm × 5.5 cm × 6.5 cm in size invading the right sphenoid and temporal bone. The mass displaced the right middle cerebral artery a little bit, and the boundary with the branch of right anterior cerebral artery was not very clear (). Magnetic resonance imaging (MRI) of the orbit showed a mass with mixed long T1 and long T2 signals in the right retrobulbar region outside the muscle cone. The right lateral orbital wall was destroyed, the lateral rectus was compressed, and the right temporal lobe was pushed backward with a clear boundary noted (). No abnormalities were detected on systemic examinations, including blood and urine tests, abdominal ultrasound, and chest CT.\nInformed consent was obtained from her parents. The plan was to remove the mass with the help of neurosurgeons followed by the chemotherapy. Intraoperatively, a soft tumor invading the right squama temporalis, the sphenoid ridge, and the lateral orbital wall was noted. It was located mainly in the lateral epidural and periorbital fascia with 4 cm × 3 cm × 3 cm in size. The boundary was clear and the blood supply was rich. No metastatic lesion was found. So the tumor was completely removed successfully. Tissue specimens obtained demonstrated monophasic SS with spindle-shaped mesenchymal cells (). Immunohistochemical stains were positive for vimentin, CD99, calponin, and Bcl-2 and negative for α-smooth muscle actin (α-SMA), muscle-specific actin (MSA), CD34, S-100, myeloperoxidase (MPO), epithelial membrane antigen (EMA), Hector Battifora mesothelial epitope-1, phosphoenolpyruvate carboxykinase (PCK), and cytokeratin 7 (CK7) (). After the right lateral orbital and right temporal tumor resection, the patient began postoperative chemotherapy with CVADIC (cyclophosphamide [CTX] 500 mg/m2 intravenously; d1, vincristine [VCR] 1.5 mg/m2 intravenously; d1-d5, adriamycin [ADM] 50 mg/m2 intravenously; d1, dacarbazine [DTIC] 200 mg/m2 intravenously; d1-d5, Q21d). She received 5 courses of chemotherapy in the 1-year follow-up, and no sign of tumor relapse or metastasis was detected. |
In August, 2008, a 36 year-old woman with no prior history of liver disease presented to her primary care physician complaining of abdominal pain of five days duration. The pain was constant, sharp, localized to the right upper quadrant, non-radiating, and associated with nausea but no vomiting. Review of systems was positive for fatigue. Initial laboratory testing revealed only a mildly elevated alanine aminotransferase (ALT) of 42 IU/L. A right upper quadrant ultrasound demonstrated hepatomegaly and steatosis. Two weeks later, the patient presented to an outside hospital with worsening right upper quadrant pain, low-grade fevers, nausea and vomiting. An MRI of the abdomen revealed a two cm enhancing lesions of the right hepatic lobe. Fine needle biopsy of the lesion demonstrated a nonspecific, mixed inflammatory cellular infiltrate and steatohepatitis. There was no evidence of malignancy. Fungal and mycobacterial cultures were negative. The patient was discharged without a specific diagnosis, and no treatment was instituted.\nTwo months later, the patient presented to Loyola University Medical Center with worsening right upper quadrant pain and fever. An abdominal CT scan revealed a 4.8 × 4.7 cm mass lesion involving the right hepatic lobe that was suspicious for malignancy (Figure ). The patient underwent a partial right hepatectomy with excision of the mass. Histopathological studies of the resection specimen showed florid necrotizing granulomatous inflammation with pseudotumor formation (Figure , ). Immuno-histochemical stains for mycobacteria, fungal organisms, and cytomegalovirus were negative. The patient's presenting symptoms resolved, and she was discharged home after an uneventful postoperative recovery.\nIn April of 2009, the right upper quadrant pain and low-grade fevers recurred. The patient presented to an outside hospital where an extensive evaluation was initiated (Table ). Serologies for acute viral hepatitis were negative. Qualitative antibodies for toxoplasmosis, human immunodeficiency virus, and Entamoeba histolytica were negative. An EIA for Borrelia burgdorferi antibodies was negative. IFA assays for Bartonella henselae and Bartonella quintana were negative. Testing for urinary histoplasmosis and blastomycosis antigens and cryptococcus serum antigens were negative. Skin testing for tuberculosis and quantiferon gold were negative. A random liver biopsy showed mild macro- and micro-vesicular steatosis and non-specific chronic inflammation. Repeat special stains, fungal and mycobacterial cultures were negative. The patient's symptoms improved, and she was discharged home on analgesics.\nIn July of 2009, the patient once again presented to an outside hospital with right upper quadrant pain, nausea, and fevers of up to 102.4°F. An abdominal CT revealed several low attenuation lesions involving both segments of the liver, with the largest one measuring 4.1 × 2.9 cm (Figure ). The patient was transferred to Loyola University Medical Center for further evaluation. A detailed travel and exposure history to ticks, rodents or other vectors of unusual infectious etiologies was unrevealing. On physical examination, the patient was afebrile. There was no lymphadenopathy or hepato-splenomegaly. Her right upper quadrant was tender to palpation without rebound tenderness or guarding. Laboratory analysis revealed an aspartate aminotransferase (AST) of 93 IU/L UNITS, alanine aminotransferase (ALT) of 74 IU/L, alkaline phosphatase of 193 IU/L, and total bilirubin of 0.6 mg/dL. The hemoglobin was 13.4 gm/dL, and the WBC count was 4.9 K/UL with a normal differential. Bacterial blood cultures, urinalysis, stool cultures, and stool tests for Clostridium difficile were negative. A chest radiograph showed no infiltrates or lymphadenopathy. The antinuclear antibody was 1:40, and anti-mitochondrial antibody testing was negative.\nOn the third day of her hospitalization, the patient developed a temperature of 101.1°F. A comprehensive investigation for fever of unknown origin was initiated (see Table ). Antibody titers for Bartonella quintana, and Brucella sp. were negative. An RPR was negative. Repeat HIV antibody testing was negative. A peripheral smear for malaria was negative. A transesophageal echocardiogram did not reveal any valvular vegetations. The patient refused a lumbar puncture. A percutaneous liver biopsy was performed and revealed extensive granulomatous hepatitis with occasional fibrin rings, with a background of mixed, micro- and macro-vesicular steatosis. Based on the presence of fibrin rings, the possibility of Q fever was entertained. However, ELISA testing for Coxiella burnetti antibodies was negative. Repeat special stains and cultures for AFB, fungal organisms, and cytomegalovirus were negative. Steiner silver stains for spirochetes and bacteria - including Bartonella sp.- were negative. The patient was empirically treated with a seven-day course of piperacillin and tazobactam. She remained afebrile for the remainder of her hospitalization. Based on the diagnosis of granulomatous hepatitis and the unrevealing workup for infectious organisms, the patient was started on empiric prednisone. Her liver enzymes, which had already been down-trending at the start of prednisone treatment, normalized over the next several weeks. Her symptoms resolved, and she was discharged home on a tapering dose of prednisone.\nBased on the striking features of her hepatic granulomas, the possibility of hepatic Bartonella infection was raised, despite the negative Bartonella antibody titers. A sample of formalin-fixed liver tissue from the initial liver resection was sent to the University of Arkansas for PCR testing. Using previously validated assay conditions, a 153 bp fragment of the B. henselae 16S rRNA gene was amplified, and confirmed by Southern blot hybridization [,]. Upon further questioning, the patient reported that she had intermittently come in contact with a cat while visiting her mother's house, although she did not recall any cat scratches or bites.\nBased on the presumptive diagnosis of hepatic bartonellosis, the patient was started on azithromycin 250 mg daily, and her prednisone was discontinued. Two weeks later, the patient developed diarrhea and abdominal cramping that were attributed to her antibiotic. Azithromycin was discontinued, and the patient was started on a nine-week course of clarithromycin at a dose of 500 mg twice daily. Her symptoms completely resolved.\nIn May of 2010, the patient presented to our emergency room with recurrent right upper quadrant pain. On examination, she was afebrile and her vital signs were stable. There was no jaundice. Right upper quadrant tenderness was present in the area of the excisional scar. There was no palpable hepato-splenomegaly. No skin rashes were present. AST and ALT levels were elevated to 109 IU/L and 75 IU/L, respectively. The total bilirubin, alkaline phosphatase, and INR were normal. A liver-protocol CT revealed a 2.4 cm, low-density lesion at the previous surgical site, which was thought to be nonspecific. However, given her recurrent symptoms, she was empirically treated with a six-week course of ciprofloxacin 500 mg twice daily.\nAt a subsequent clinic visit in September of 2010, she reported improvement in her abdominal pain. However, the AST and ALT activities had risen to 139 IU/L and 195 IU/L, respectively. A transjugular liver biopsy demonstrated small, scattered, non-caseating granulomas with a background of micro- and macrovesicular steatosis. A sample from the biopsy was sent to the University of Washington for PCR analysis. Using conventional PCR conditions and primers targeting the ribC gene (5'-GATATCGGTTGTGTTGAAGA-3', 5'-AATAAAAGGTATAAAACGCT-3') [], a 393 bp PCR product specific for B. henselae was amplified from the biopsy material. In order to determine whether the patient was bacteremic, venous blood samples were sent to the Intracellular Pathogens Research Laboratory (IPRL), Center for Comparative Medicine and Translational Research, College of Veterinary Medicine, North Carolina State University. Using a previous described diagnostic platform [,], conventional PCR targeting the 16S-23S intergenic spacer (ITS) region was performed on DNA extracted from blood, serum, and Bartonella alpha Proteobacteria growth medium (BAPGM)enrichment blood cultures. No amplification products were obtained from pre-enrichment blood and serum samples obtained on three sequential days. In contrast, a target band was obtained from one of three post-enrichment blood cultures. DNA sequencing of the amplicon was diagnostic for the ITS region of the SA2 strain of B. henselae, thereby establishing B. henselae bacteremia. The patient's serum tested negative for antibodies against B. henselae, B. koehlerae, or Bartonella vinsonii subsp. berkhoffii genotypes I, II or III antigens []. Subsequently, using 16S-23S ITS primers, B. henselae DNA (SA2 strain by DNA sequencing) was amplified and sequenced from paraffin blocks containing material from the initial liver resection (January 2009) and from the follow-up biopsy obtained in September of 2010 (Figure ). These data demonstrated the presence of the SA2 B. henselae strain in the patient's liver throughout her course. Immunohistochemical staining for B. henselae using a recently described B. henselae monoclonal antibody assay [] showed rare coccobacilli with positive staining and appropriate morphology for B. henselae, however, clusters of organisms were not visualized (data not shown). |
We present a 31-years old male with a right ear hearing loss of 3 months duration. He did not experience earache or discharge before the hearing loss. He; however, experienced visual acuity and dizziness. General physical examination did not yield much. Cranial nerve examinations were unremarkable. We did not observe any cerebellar signs and symptoms. We did not observe any discharges from the ear and otoscopic examination did not reveal same. Rinne and Weber tests demonstrated sensorineural hearing loss. Fundoscopic examination did not yield much. Routine laboratory investigations were within normal rangers.\nCT revealed a cystic-solid mass at right CPA measuring about 5.3 × 4.3 cm with slightly dense regions. We noticed right auditory canal expansion (Fig. A). Enhanced CT demonstrated partial heterogeneous enhancement. We noticed thick and enlarged blood vessels all-round the lesion most of which drained into an enlarged great cerebral vein. Computed tomography angiography (CTA) also showed (Fig. B and C) a right CPA mass encasing the right vertebral artery (VA). The distal lumen of right VA was narrow due to compressive effect of the lesion. The right anterior cerebral artery was also narrow. The bilateral internal carotid arteries were normal. MRI also revealed a cystic-solid right CPA lesion (Fig. A–C) measuring about 5 × 4.0 × 3.5 cm. The solid part of the lesion demonstrated mixed signals intensities on T1 and T2-weighted images. We observed heterogeneous enhanced images on enhancement MRI. The auditory canal was slightly enlarged. The mass was compressing the cerebellum as well as the fourth ventricle. We did not observe hydrocephalus. Our working diagnosis was an acoustic neuroma with cystic changes.\nWe totally resected the lesion via the retro-sigmoid approach. Intraoperatively, we saw a cystic-solid tumor which was red in color and composed of vascular tissue. We saw a multitude of thick feeding arteries and veins all around the solid portion. The solid part was tough in consistency with clear boundaries. It was bleeding profusely on touch. The solid portion was adhered to the trigeminal nerve, acoustic nerve, facial nerve as well as the brain stem. The tumor originated from the cerebellar pial vessels and took an intra-axial route. We first and foremost identified the feeding arteries and electro-coagulated them to minimize intraoperative bleeding therefore blood loss was minimal (<200 mls). We then carefully resected of the tumor by separating it from the above vital structures. We utilized electromyographic (EMG) and auditory brainstem responses (ABRs) to monitor the cranial nerves above. All cranial nerves functions were intact after the operation.\nOn histochemical staining, we observed intracytoplasmic glycogen within the foamy cytoplasm (Fig. A and B). Immunohistochemistry studies revealed that, the tumor cells were D2–40 (+), carbonic anhydrase (CAIX) (+ ), neuron-specific enolase (NSE) (±), Inhibin- α (+),brahma related gene-1 (BRG1) (+), Ki67 (+, 20%–30%), glial fibrillary acidic proteins (GFAP) (−), S-100 (−), CK (−), epithelial membrane antigen (−), P63 (−), CD34 (−), signal transducer and activator of transcription 6 (−), desmin (−), smooth muscle actin (−), thyroid transcription factor 1 (−), chromogranin A (−), synaptophysin (−). These finding are consistent with the diagnosis of HMB. Postoperative MRI demonstrated total resection of the tumor (Fig. A–C). Two years follow-up revealed no recurrence of the lesion and the patient is well. His right sided hearing loss was restored after the operation. |
We report a case of a 73 year old man presenting with a non healing wound on his right forearm for 4 weeks. The wound started from an insect bite and progressively enlarged with increasing pruritus and burning sensation. Clinically an ill-defined ulcer was seen on his right forearm with surrounding erythema and erosion. There was a yellow crust overlying the center of the ulcer and the periphery was scaly (Figure ). Further inquiry revealed history of self treatment with a yellow solution to clean his wound for 3 weeks. He could not recall using the solution in the past and did not seek any medical advice prior to the current visit. Patient was provisionally diagnosed to have allergic contact dermatitis secondary to acriflavine. Acriflavine usage was stopped and the ulcer was treated with daily non-occlusive saline dressing. The lesion improved a week later looking noticeably dry, less inflamed and smaller in diameter (Figure ). At the end of two weeks, there was complete resolution of the ulcer, leaving a residual post-inflammatory hyperpigmentation. A patch test using Standard European series (Chemotechnique) containing 28 different allergens and acriflavine was done seven months later. The result showed a positive reaction (2+) to acriflavine while the rest were negative.\nAcriflavine or commonly known as Flavin, is an acridine derivative of proflavin used as a topical antiseptic agent. It is a yellow or orange colour solution which stains the skin and may cause irritation, inflammation or blister upon contact []. It is commonly used as skin disinfectant for minor wounds, burns, infected skin and is effective against both gram positive and negative bacteria []. Acriflavine was initially used during the First World War as a treatment for African trypanosomiasis (sleeping sickness). In addition to its disinfecting property, it has also been shown to inhibit cancer progression in animals []. Although used in dilution (0.1%), it has been documented to produce potential skin irritation and is still widely used for wound dressing in both hospitals and outpatient clinics.\nContact dermatitis is an eczematous symptom occurring as a result of skin exposure to an irritant or sensitizing agent. It is generally categorized as allergic contact dermatitis and irritant contact dermatitis. Allergic contact dermatitis is an immune mediated inflammatory reaction while irritant contact dermatitis is a non-allergic inflammatory reaction causing direct cell damage resulting in skin dryness, redness or even burns []. Common antibacterials and antiseptics that cause an allergic reaction are Neomycin (40.6%), Soframycin (15.1%), Dettol (10.9%), Savlon (8.3%) and Acriflavine (5.2%) [,]. Besides contact dermatitis, acriflavine has also been shown to cause perioral and mucosal odema [].\nIndividuals of all age groups and ethnicity have potential risk for developing contact dermatitis ranging from diaper dermatitis in infants to hair dye and fragrance dermatitis in the elderly []. Various studies have shown increasing prevalence of contact dermatitis to topical medicaments with advancing age [,]. This is probably due to the fact the elderly have been exposed to multiple allergens during their life time compared to younger individuals. Atopic individuals were previously thought to be more susceptible to contact dermatitis. However, a definite relationship between atopic dermatitis and contact dermatitis is yet to be established as current evidence show controversial results [].\nTypical features of dermatitits medicamentosa are non healing wound, worsening or enlarging wound with presence of surrounding dermatitis. Staining of the wound provides a clue to identify the irritant; yellow (acriflavine) or dark brown (potassium permanganate). The reaction is usually gradual and not an acute event. Hence patients are usually unaware of the underlying cause for the reaction which further delays wound healing. Withholding the irritant and appropriate topical management speeds up recovery.\nPatch test is the gold standard for detection and confirmation of contact dermatitis. This is a simple yet cost effective test that facilitates early and accurate detection of underlying cause for suspected contact dermatitis []. It also serves as an important tool in patient education to avoid further exposure to the known allergens. Although the test is simple to perform, adequate experience and training is required for accurate interpretation of the results. Patch test is best avoided during an acute reaction and performed about six months after an initial contact to prevent further aggravation of the existing dermatitis []. |
The 39-year old, 68 kg and 165 cm patient, mother of a 20 year old son, was referred to our institution during the 33rd week of pregnancy. One year before, she had experienced multiple transitory ischemic attacks, she was in NYHA (New York Heart Association) class IV and IVNC was diagnosed based on echocardiography. Besides warfarin, which during pregnancy was switched to low molecular weight heparin, pharmacological therapy with an angiotensin converting enzyme inhibitor had been established. Although severe ventricular arrhythmias had been diagnosed, the patient refused implantation of an internal defibrillator. The transthoracic echocardiography (TTE) at admission showed the typical picture of IVNC: a massively dilated left ventricle (end-diastolic diameter of 80 mm - normal value <50mm), a mitral valve regurgitation grade I, a severely impaired left ventricular ejection fraction (LVEF) of 25% (normal value>55%) and a semi-systemic systolic pulmonary artery pressure (PAP) of approximately 60 mmHg. Due to the inherent risk of cardiac decompensation in the late pregnancy, the patient was admitted to our heart centre one week later and elective caesarean section scheduled for the 35th week of gestation. Due to the risk of sudden life-threatening decompensation during surgery, it was planned to perform the operation under general anaesthesia and “stand by” of veno-arterial extracorporeal membrane oxygenation (ECMO) for extracorporeal life support. On the day before surgery, TTE revealed further decrease of the LVEF to 20%, increase of the mitral valve regurgitation to grade II and increase of the systolic PAP to 2/3rds of the systemic value of approximately 80 mmHg.\nThe patient received 150 mg of ranitidine 12 and 2 hours before the operation. After having arrived in the anaesthesia induction room in nearly sitting position, 30 ml of sodium citrate (0.3 molar) was administered orally. Standard monitoring with electrocardiogram and pulsoxymetry was established. In addition, paddle leads for external defibrillation were placed. Thereafter, an arterial line was placed into the left radial artery; a 4 lumen central venous catheter and a 9 gauge introducer were placed into the right jugular internal vein in local anaesthesia. A pulmonary artery (PA) catheter with atrial and ventricular pacing option (Baxter International, Illinois, USA) was inserted into the introducer and placed into the PA.\nAt this time the heart rate was 110/min with multiple polytopic ventricular extra-systolic (VES) beats. In the nearly sitting position, the arterial blood pressure was 110/70/90 mmHg and the PAP 75/30/50 mmHg with a pulmonary capillary wedge pressure of 30 mmHg. Pharmacologic therapy with a continuous infusion of dobutamine 3 µg/kg/min and milrinone 0.25/kg/min was started. Additionally, two 6 French introducer catheters were placed under local anaesthesia into the right femoral artery and right femoral vein. These catheters were inserted to facilitate quick percutaneous access to the vessels for emergency installation of ECMO for extracorporeal life support in case of acute cardiac decompensation.\nAfter these preparations, the patient was transferred into the operating room and preparations for surgery were performed.\nAfter pre-oxygenation, anaesthesia was induced with 5 mg of rocoronium, 15 mg of etomidate, 35 mg of S ketamine and 100 mg of succinylcholine and a rapid sequence intubation was performed. Three minutes later, a healthy 2800 g child was delivered by classic longitudinal caesarean section (APGAR values of 9-10-10). Thereafter, anaesthesia was continued with a bolus of 30 mcg of sufentanil and 35 mg of rocoronium and a continuous infusion of remifentanil (2 µg/kg/min) and propofol (0.1 mg/kg/min) without using Target Controlled Infusion (TCI). Additionally, 1 g of paracetamol and 1 g of novamin-sulfate and 4 mg of ondansetron were infused. At the end of the procedure residual muscle relaxation was antagonized with 300 mg of sugammadex.\nDuring the entire period of anesthesia, hemodynamics remained completely stable, the PAP decreased to 1/3rd systemic values of 50/30/20 mmHg while, however, polytopic VES were still observed. The completely awake and orientated patient was extubated on the surgical room. The patient was transferred to the intensive care unit (ICU) and she remained here for 5 days. Therapy with milrinone and dobutamine was continued during ICU stay. The patient was discharged uneventfully from hospital 14 days after surgery. |
A 54-year-old female was brought to the hospital by EMT (emergency medical team) with suspicion of opiate overdose. EMT was called by a family member after the patient was found to be less responsive and difficult to arouse. The patient received 4 mg of naloxone subcutaneously in field by the EMT which improved patient's responsiveness but pushed her into severe opiate withdrawal. In the emergency department, the patient was found to be awake, alert, and anxious. She was not oriented but was able to follow simple commands. The patient was also diaphoretic, tachypneic with respiratory rate in 30 and tachycardic with heart rate in 150 s. Lungs were clear on auscultation with good bilateral air entry. Cardiovascualr exam was unremarkable with regular rhythm good pulses present in all four extremities. Abdominal exam was also unremarkable. Neuromuscular exam showed tremulousness and muscle fasciculation in different muscle groups of the face, upper limb, and lower extremities, exacerbated deep tendon reflex and normal motor strength with severe pain on movement of left hip. The patient had a past medical history of AIDS, hepatitis C, and bipolar disorder but as per her home medication list she was not on any treatment for them. At home, the patient was on 150 mg of methadone for maintenance and oral morphine sustained release and immediate release for pain control for her recent hip fracture. The first ABG done at the time of patient's ED presentation was on a nonrebreather mask; it showed pH 7.694, PCO2 19.6 mmHg, PO2 224 mmHg, O2 saturation of 100%, and lactate of 2.97 and her initial EKG showed bigeminy with the rest of the details obscured by artifacts as seen in . In the next 30 minutes patient's respiratory rate had decreased to 18–25 breaths per minute at which time a repeat ABG was done on room air that showed pH of 7.641, PCO2 20.3 mmHg, PO2 of 86 mmHg, and O2 saturation of 98% with lactate of 2.48. While being on telemetry, the patient had sinus tachycardia with PVCs and runs of bigeminy. The patient was subsequently deemed stable and taken for CT scan of heat and chest for further evaluation, while in CT room the patient went into generalized tetany and then into cardiac arrest with initial rhythm of ventricular tachycardia that degenerated to torsade de pointes and ventricular fibrillation. Code was run for 25 minutes, during which the patient was intubated and received 2 doses of epinephrine and 4 gm of magnesium and was shocked 3 times. She had a return of spontaneous circulation after the third shock. The patient was initially started on hypothermia protocol; however, it was abandoned as patient showed movement of extremities. The labs obtained prior to patient's cardiac arrest showed Na+ 138, K+ 3.4, Mg+ 2.0, Ca2+ 8.9, HCO3 18, and anion gap of 16; however, this sample was hemolyzed. Repeat labs obtained 30 minutes after intubation showed K+ 3.5, Ca2+ 7.6, iCa2+ 3.92, Mg+ 2.6, and HCO3 16 with anion gap of 17. EKG obtained after cardiac arrest showed sinus tachycardia. Urine toxicology obtained for the patient was positive only for methadone and opiates. Patient's serum albumin level was 2.9. The patient was extubated on day 2 and was discharged from hospital on day 8 of hospitalization. |
A 74-year-old man was diagnosed with early gastric cancer via endoscopy at a regional hospital. He was then transferred to our Hospital for tertiary management. Endoscopic ultrasonography was performed to determine the depth of the tumor. This showed that the lesion was confined to the mucosa (). Computed tomography then revealed the presence of a suspected GIST, in the form of a 2×2 cm mass at the hilum of the spleen ().\nThe two management options were either a total gastrectomy or a subtotal gastrectomy with local resection of the hilar mass. In view of the small size of the suspected GIST and the advanced age of the patient, the latter approach was selected to minimize risk and maximize quality of life. The patient experienced no complications, and was discharged home 10 days postoperatively.\nA pneumoperitoneum was created using a carbon dioxide (CO2) pressure of up to 12 mmHg. Five trocars were inserted, including one for the telescope. The patient was then placed in the supine position and the head was elevated. After confirming the absence of systemic disease, the great omentum and lesser omentum were divided using electrocautery and ultrasonic shears (LCS; Ethicon Endo-Surgery, Cincinnati, OH, USA). A 2×2 cm mass was observed at the hilum of the spleen. Intraoperative endoscopy revealed that there was no mass in the stomach, and it was therefore concluded that the mass had extruded into the peritoneal cavity only. Careful inspection confirmed that it would be possible to preserve two or more of the short gastric arteries below the GIST (). Wedge resection of the GIST was then performed using an Endo-GIA 60 device (). The left gastroepiploic vessels and the right gastroepiploic artery and vein were then ligated and divided. The duodenum was divided using an EndoGIA 60 device, and the lymphatic tissues of stations 8, 9, 7, and 11 were dissected. After confirming the adequacy of the lymphatic dissection, a 5 cm right-subcostal minilaparotomy incision was made. The anvil of the circular stapler was inserted into the duodenum, and the minilaparotomy incision was used for the insertion of a purse-string suture. An intracorporeal Billroth I stapled anastomosis was created using a hand access device (Gel Port; Applied Medical, Rancho Santa Margarita, CA, USA).() Surgery was concluded after confirming that there was no bleeding in the operative field or tension at the anastomosis site.\nOn visual inspection, the adenocarcinoma appeared grossly similar to early gastric cancer type IIc. The tumor was 3.2×2.2 cm in size, and the proximal and distal resection margins were 11.3 cm and 1 cm, respectively. The GIST was 2.2 cm in size, and a clear resection margin was achieved.\nHistopathological examination revealed that the adenocarcinoma was moderately differentiated and confined to the mucosa (T1aN0M0, stage 1A). Its type according to the Lauren classification was intestinal. No metastasis was detected in any of the 12 retrieved lymph nodes. The GIST was benign, and consisted of spindle cells with no atypia. Immunohistochemical analyses revealed that the GIST was positive for C-kit and CD34, and negative for smooth muscle actin. |
A 74-year-old woman was admitted to the hospital for fever and general weakness. On the computed tomography scan, there was large abscess cavity showing partially septated cystic lesion with peripheral inflammatory hyperemia in the right hepatic lobe. Incidentally, a lobulated, 5 × 3.2 cm, heterogeneous pancreatic mass containing several intratumoral calcification and cystic change on the tail of the pancreas and a 6-cm, well-defined mass with heterogeneous contrast enhancement in the left lobe of the liver were detected (). Two months after conservative treatment for hepatic abscess, the abscess was completely resolved. However, the mass in the left lobe of the liver remained unchanged and then ultrasound-guided liver biopsy was performed. The tumor consisted of papillary neoplasm showing monomorphic polyhedral cells with hyalinized fibrovascular stalks, which was best classified as metastatic SPT of the pancreas. Immunohistochemistry was positive for vimentin, antitrypsin, and neuron specific enolase. On the presumption of an SPT of the pancreas with hepatic metastasis, the patient underwent radical antegrade modular pancreatosplenectomy with hepatic resection. On operation field, there were no seeding nodules or other metastatic lesions in the abdominal cavity. Encapsulated masses, 6 × 5 cm and 5 × 4.5 cm in size, were found in the segment of the left liver and in the tail of the pancreas, respectively. All surgical margins were clear. Microscopically, the growth pattern of the pancreas tumor was heterogeneous, with a combination of solid and pseudocystic structures in varying proportions. The tumor was composed of monomorphic polyhedral cells with hyalinized fibrovascular cores, thereby leading to the characteristic pseudopapillary appearance (). Mitotic activity was low. However, perineural invasion and infiltration into the peripancreatic fat tissue could be seen. Twenty-five lymph nodes examined were free of tumor. Less than 5% of tumor cells were positive for Ki-67. The Microscopic findings of the hepatic tumor were similar to the findings of the pancreatic tumor. A final diagnosis of SPT of the pancreas with metastasis to the liver was made. Currently the patient is alive without recurrent disease (10 months after operation). |
A 52-year-old married woman was suffering from recurrent depressive disorder as described in the International Classification of Diseases (ICD)-10. There is significant family history of mood disorder as the patient's mother had been hospitalized thrice for depression which affected her during her childhood and teens. The patient is the eldest of three sisters. Her younger sister was suffering from dysthymia and her youngest sister who has a learning disability had undergone treatment for depression.\nHer first depressive episode occurred at age 40. She had previously been well and in particular did not have dysthymia or any abnormal personality traits. She was treated with dosulepin (previously called dothiepin) and she recovered with a dose of 150 mg daily, but developed sweating as a side effect. She had migraine and was treated with methysergide. A neurologist thought her migraine might respond to amitriptyline, but she complained of a racing heart while taking it. She did not persist with subsequent treatment with venlafaxine XL 75 mg nocte. Minor side effects explain the diversity of antidepressant drugs she has been prescribed.\nAfter a period of being well, she was referred back to psychiatric services at age 49. Her general practitioner wrote in her referral letter that he had started her again on dosulepin 150 mg nocte six weeks ago. By the time she saw the psychiatrist in October 2001 she was well. She did not persist with dosulepin and in August 2003 had morbid thoughts and suicidal ideas. She was started on mirtazapine and the dose was increased from 15 to 45 mg nocte. By March 2004, she was well, but had stopped taking antidepressant drugs. She was referred again in October 2004 when her general practitioner had started her on lofepramine 140 mg nocte. By December 2004, she was again well, but once more stopped taking the antidepressant.\nShe was referred again in July 2005 when she reported no point in living. She was started on escitalopram 10 mg nocte. While when previously started on the antidepressant drug treatment she experienced transient suicidal thoughts occasionally, this time her thoughts were considerably more intense, intrusive, and compelling. The thoughts were continuous and persistent. She kept thinking “I can’t go on. I’d be better off dead”. Her impression was that her suicidal thoughts were qualitatively different from any she had previously experienced. She was slightly agitated. Six days after starting on escitalopram she took a large overdose; this was the first time in her life that she had harmed herself. She reported no preceding adverse events or changes. Her husband had continued to be supportive as had been the case in the past. Subsequently, her depression was successfully treated with trazodone, after which she did not experience suicidal thoughts. |
A 31-year-old female, gravida 2, para 2 (G2 P2), referred to the gynecologic clinic with a main complaint of secondary infertility during the last twelve months. Written informed consent was obtained from the patient for publication of this case report and any accompanying images. The patient had two previous C-sections. Her menstrual cycles were ovulatory. Spermogram was unremarkable and hormonal assay did not show any abnormality. HSG was performed and revealed a migrated copper IUD with its long tail out of the uterine cavity (Fig. ). Plain abdominopelvic radiography also indicated a rotated T-shaped IUD in the pelvis (Fig. ). A transvaginal ultrasound was also performed by a skilled radiologist who reported a copper IUD in the bladder lumen with a small portion of it within the bladder musculature. The patient had taken multiple courses of antibiotics for urinary tract infection (UTI), but her symptoms never disappeared. The patient also underwent cystoscopy for recurrent infection last year, but no pathological finding was detected. Eventually, the patient said that her symptoms were related to the possible adhesions following two previous operations. The patient had a history of IUD insertion following her last C-section about eight years ago. After three years, however, the patient decided to remove it due to recurrent vaginal secretions. Three years later, her IUD was expelled, and another copper IUD was inserted. The patient removed the other copper IUD for becoming pregnant about two years ago. The patient was very confident of its removal, but her recent HSG, interestingly, demonstrated a rotated copper IUD in the pelvic cavity. Physical examination was essentially normal. A baseline complete blood count, urea, and creatinine levels were normal. The patient was candidate for a hysteroscopic cystoscopy. Hysteroscopy was normal except for a small fibrotic defect at the lower segment of the uterus. During cystoscopy, the long tail of the copper IUD was found on the posterolateral border of the bladder far from ureteral offices (Fig. ). The two short arms adhered to each other were embedded in the mucosal and muscular layer (Fig. ). No calculus was observed within the bladder cavity. Using glycine as the media, a mono-polar loop entered the bladder. A gentle and brief cautery was applied on the mucosa where the shadow of a short arm of the device was observed. The copper IUD was safely removed through the urethra using a special grasper. There was no major defect in the place of the copper IUD in the bladder. The bleeding points were effectively cauterized (Fig. ). The patient was discharged the next day with an indwelling catheter. After catheter removal, the patient did not complain of any urinary symptoms. |
A 37 year old man was referred to a district hospital with pectus excavatum and progressive shortness of breath. Native computed tomography revealed an excessively deformed chest (Figure ) and symptoms were thought to be related to the anatomical situation. After presentation of the patient in the thoracic surgery unit, he was scheduled for an operative correction. A Ravitch-procedure had been performed in July 2009. The patient's pectus excavatum was addressed using two metal bars. They were placed in a parallel fashion with the ends supported by the lateral thorax at the level of the third and fourth rib and fixed with ripclamps. The middle portion of the chest was straightened by the bar running underneath it. The patient showed an uneventful post-op course and was discharged on day 9 postoperatively.\nAlthough the anatomical shape was almost normalized after the operative intervention, fatigue, shortness of breath and palpitation were still persistent. A broken and dislocated lower metal bar with concomitant instability (Figure ) led to a reevaluation in May 2010. After realization of a follow up computed tomography surprisingly and as a co-finding, a severely dilated ascending aorta >7 cm was found, located in direct neighborhood to the metal bars (Figure ). The following cardiological investigations (echocardiography, MRI) revealed a tricuspid aortic valve with a severe aortic regurgitation due to an annuloaortic ectasia (73 × 74 mm root), a massively dilated left ventricle (LVEDD 85 mm) without hypertrophy and a slightly reduced ejection fraction of 45%. The mitral valve showed a normal morphology with mild regurgitation but with normal annular, valvular and subvalvular conditions.\nThe patient was transferred to the Hospital Clinic for surgical correction of the cardiovascular pathology. After midline sternotomy the two titan bars were identified. The lower dislocated and broken bar was removed completely, the upper bar was cut and 3 cm were removed. The pericardium was totally intact after the Ravich-procedure. After opening of the pericardium the huge annuloaortic aneurysm became visible. After heparinization and installation of the extracorporal circulation with aortic cannulation of the arch and venous cannulation using a two-stage cannula placed into the right atrium cardiopulmonary bypass was started. The ascending aorta was distally crossclamped directly underneath the brachiocephalic trunc. After inspection of the aortic valve and almost complete resection of the ascending aorta, a valve sparing aortic root replacement (David procedure) using a straight 30 mm Hemashield prosthesis with lateral insertion of the coronary ostia was performed. The echocardiography showed a perfect valve function with low gradients. After weaning from bypass and decannulation protamin was substituted. The sternum was closed using the Robiscek wire reinforcement technique. Apart from a short period of atrial flutter and a spontaneously resolved paralytic ileus the patient's postoperative course was uneventful and he was discharged at day 10 postoperatively. |
A 68-year-old woman who did not exhibit any symptoms, including those of myasthenia gravis or other autoimmune diseases, was found to have an abnormal shadow on her chest roentgenogram at medical check-up () and was referred to our hospital. A chest computed tomography (CT) scan showed a 4.5-cm-diameter tumor with a homogeneous inner component in the anterior mediastinum (). The tumor was adjacent to the superior vena cava and the ascending aorta. Blood tests showed all tumor markers within the normal range. Thymoma was suspected, and surgical excision was planned. The patient underwent total thymectomy via a median sternotomy. Grossly, the tumor was widely and firmly attached to the pericardium and right lung and was thought to have invaded the pericardium and lung. Therefore, pericardial resection and right upper lung partial resection were performed. The tumor measured 6 cm × 4.5 cm × 3.5 cm and involved the pericardium and left lung. The cut surface was grayish with lobular formation. Intraoperative histologic examination of a frozen specimen led to a diagnosis of thymoma. No serious complications were observed during the postoperative course, and the patient was discharged on the 17th postoperative day. Postoperative microscopic examination showed lobular and alveolar structural growth patterns that were arranged in epidermoid features with prominent nuclear palisading around perivascular spaces, and lacking nuclear atypia and scant lymphocytes, and had invaded the pericardium and S3 segment of the right lung, and these findings were consistent with a Masaoka stage III type B3 thymoma, and stage IIIa according to the 8th edition of the International Association for the Study of Lung Cancer and the International Thymic Malignancy Interest Group TNM staging system (). However, rosette-like arrangements were observed in part of the tumor (); therefore, immunohistochemical examinations were performed. The tumor cells were diffusely positive for synaptophysin () and chromogranin A (), but negative for CD5 and c-Kit. From the aforementioned findings, although there was rosette formation and positive immunohistochemical findings, it was diagnosed as a Masaoka stage III type B3 thymoma with a neuroendocrine differentiation rather than a neuroendocrine tumor due to cellular morphology such as prominent nuclear palisading around perivascular spaces and lacking nuclear atypia.\nThe patient received 40 Gy radiotherapy for adjuvant therapy and had no recurrences for 3 years postoperatively. Unfortunately, pleural dissemination and multiple lung metastases were found on follow-up CT 3 years and 6 months after thymectomy. She was treated with three courses of chemotherapy, based on a platinum doublet using carboplatin and etoposide, and subsequently with six courses of adriamycin, cisplatin, and cyclophosphamide, and these therapies achieved a partial response. The disease was stable for about 2 years without any treatment and progressed thereafter. Chemotherapy was restarted with four courses of amrubicin and subsequently with four courses of nab-paclitaxel, but the number and size of multiple pulmonary metastases increased, and a pleural effusion appeared. The patient died of the disease 8 years and 3 months after thymectomy. |
A 48-year-old man was admitted to our hospital complaining of fever, chills, sweating, and dyspnea. He has a history of admission to our hospital for evaluation and management of suggested deep neck infection with small amount of left pleural effusion, which was improved with antibiotic and symptomatic therapy. He had smoked 1 pack per day for 25 years, and taken herbal medication for 1 month before admission, which ameliorated his symptoms a little. He had no underlying illness such as hypertension, diabetes, or autoimmune diseases. On physical examination, he showed generalized edematous appearance with venous engorgement of neck and upper chest. The lung sound was diminished on both side of lung during auscultation of chest.\nRoutine laboratory test including liver function test and renal function test showed normal range of results. Autoimmune markers such as fluorescent antinuclear antibody and anti-neutrophil cytoplasmic antibody also showed negative result. Chest X-ray showed massive pleural effusion in both pleural cavity without parenchymal abnormality (). Neck computed tomography (CT) and chest CT showed thrombophlebitis at left innominate vein, subclavian vein and left internal jugular vein and enlarged lymph node at left neck and axillar area with both pleural effusion and ascites and diffuse soft tissue edema (, ). Two-dimentional echocardiography showed spongiform appearance with prominent trabeculation of left ventricle (LV) with preserved LV systolic function, and no significant valvular dysfunction.\nPleural fluid analysis showed lymphocyte predominant exudates nature. But that did not suggest any specific disease. Pleural biopsy through video-assisted thoracoscopic surgery showed chronic inflammation with lymphoplasmacytic infiltration and fibrosis. Plasma cells were positive for IgG and IgG4 in immunohistochemical stain, and the ratio of IgG4 to IgG-positive plasma cells is 0.24 (). Lymph node biopsy of neck on previous admission for evaluation and management of suggested deep neck infection also showed same result for IgG and IgG4 stain (). Serum IgG4 level was 248.0 mg/dL (normal range, 6.1-121.4 mg/dL). Laboratory test for common plasma cell disorder showed negative results.\nWe could diagnose with IgG4-related disease. We started systemic glucocorticoid therapy (prednisolone 0.6 mg/kg) with anticoagulation (subcutaneous injection of enoxaparin 1 mg/kg twice a day). He showed symptomatic improvement after treatment. And the chest X-ray and chest CT which was taken after 2 months of therapy showed almost completely disappeared pleural effusion and ascites, and decreased soft tissue edema (). |
A 43-year-old female, whose height was 160 cm and weight 46 kg [body mass index (BMI) 18.0], was admitted due to 2 months of repeated fever and space-occupying lesions in the liver on May 15, 2020 (day-1). The highest body temperature appeared at night but returned to normal in the morning, with dry cough, shortness of breath, and dull pain in the liver area. The physical examination of the whole body showed no obvious positive signs, there were no abdominal tenderness and percussion pain, and no enlarged superficial lymph nodes were palpated. So, the patient still could live and work properly. She lived in Chongqing city, whose economic situation is medium as a furniture sales staff. She visited Guangzhou province 6 months ago on business for 2 days. She denied any contact with livestock and eating wild vegetables, raw fish, shrimp, and so on, but drank tap water every day. The local hospital prescribed empirical antimicrobial therapy with azithromycin and Chinese herbal medicines; however, the treatment failed to relieve the symptoms of the patient. Thus, the patient was transferred to our hospital for further evaluation. An outline of the episodes is described in . Chest CT scan showed no infection in the lungs, while mass-like shadows in the liver were observed on the abdominal CT scan. Routine blood parameters revealed elevated white blood cell and eosinophil count ().\nEnhanced MRI of the upper abdomen showed the increased liver volume, uneven liver parenchyma signal, liver interstitial edema, and accumulated fluid in the abdominal cavity on hospital day 2 (). Laboratory tests showed elevated IgE (333 ng/mL), white blood cell, and eosinophil count on hospital day 5. Microbial tests, including blood cultures, stool examination, and stool precipitation, did not detect any pathogens. Eggs were not detected by direct fecal smear and fecal washing precipitation. Due to excessive pharyngeal reflex during gastroduodenal tube placement, two attempts failed to obtain the bile of the patient. The diagnosis of tuberculosis was excluded for the negative results of the chest CT scan, tuberculin skin test, tuberculosis IgG and IgM antibody screening, and T-Spot examination. Meanwhile, ovarian tumors, liver tumors, gastrointestinal tumors, and hematological tumors were also excluded temporarily as the negative results of abdominal enhanced CT and tumor marker detection such as AFP, CA-50, CEA, TAP, CA-125, CA-199, CA-242, and CA-724 and so on. The serum parasite IgG antibody, including schistosome, paragonimiasis, cysticercosis, trichinella spiralis, liver fluke, sparganosis, and hydatid IgG antibody detected by ELISA were negative on hospital day 12. Piperacillin-sulbactam was used as an antibacterial treatment for 10 days from hospital days 4–13.\nHowever, the body temperature of the patient fluctuated several times, and the eosinophil count was not significantly reduced (). So, an enhanced CT of the lower abdomen was performed on hospital day 13, which showed multiple shadows of abnormal density in the liver and a small amount of effusion, suggesting the possibility of infectious and neoplastic lesions (). Because of the high fever, biapenem was adjusted to be used to cover drug-resistant bacteria for more than 2 weeks from hospital day-14 to day-32.\nTo further confirm the diagnosis, an ultrasound-guided percutaneous liver biopsy was conducted on hospital day-14. The hepatic histopathology reported mild inflammation in the portal area and eosinophil infiltration in the inflammatory necrotic area, with no evidence of malignancy, liver abscess, and parasitic infection on hospital day 20 ().\nAs treatment with antibiotics was ineffective (long-term fever, significantly elevated eosinophils count, intrahepatic lesions), the enhanced MRI of the upper abdominal was re-examined on hospital day 33, which indicated no absorption of intrahepatic lesions (). Meanwhile, combined with Chongqing epidemiological studies of Clonorchis sinensis, parasite infection was suspected and praziquantel was used as treatment. The body temperature decreased, and the patient was discharged on day 36.\nDuring the period of hospital days 33 and 67, the patient still had repeated fever with a maximum temperature of 39°C (). An enhanced MRI of the upper abdominal showed that the liver shadow had less absorption (). So, albendazole was adjusted as the treatment on the follow-up day. Albendazole was orally taken 0.8 g once a day for half a month. As the body temperature of the patient decreased, the dose of albendazole was decreased to 0.6 g for half a month after drug withdraw. On the follow-up day, the dose of albendazole was not adjusted and the patient was prescribed to take albendazole according to taking half a month and stopping for half a month. Firstly, the peak temperature of the body decreased significantly. Secondly, the eosinophil count decreased and ultimately returned to normal. However, there was still a high fever up to 40°C (), occasionally. Meanwhile, the enhanced MRI of the upper abdominal reexamined on follow-up day 137 showed that there was no significant absorption of the lesions compared with the previous (). So far, after a total of 106 days of empirical anti-parasitic treatment, the body temperature and eosinophil count decreased significantly, but the imaging manifestations did not improve significantly.\nTo identify the pathogen, liver tissue collected on hospital day 14 and peripheral blood on follow-up day 137 were sent for mNGS with the written consent of the patient. The total reads of the liver tissue and blood were 6.93 and 30,379 Mb, respectively. Meanwhile, the reads mapped to microbes in the liver tissue and blood were 934,425, and 29,009. The number of species-specific reads aligning to the Fasciola hepatica genome in the liver biopsy and blood was 3 and 51, respectively (). Finally, the disease was diagnosed with fascioliasis hepatica with triclabendazole as the first-line drug (). However, for the sake of the unavailability of triclabendazole under the epidemic of COVID-19 in China and other countries, and the partial effectiveness of albendazole on the fascioliasis hepatica which significantly decreased the body temperature and eosinophil count of the patient, albendazole was used with a prolonged treatment time following the previous way.\nEventually, after receiving treatment of albendazole for more than 7 months, the body temperature and eosinophil count of the patient normalized with no obvious clinical manifestations. So, albendazole was discontinued on day-300. After more than 3 months after drug withdrawal on follow-up day 148, the enhanced MRI of the upper abdominal showed that the intrahepatic low-density shadow was smaller (), and the blood mNGS was negative (), suggesting the albendazole treatment was effective. |
A 25-year-old female presented to the hospital with a 6-month history of exertional dyspnea and unproductive cough. She denied hemoptysis, chest pain, or orthopnea. Her past medical history included a painless mandibular mass removed through a conservative surgical procedure, in another hospital facility, 5 years before. This relapsed 2 years later and required a re-excision at the same facility. She was told that the excised tissue was reported as ameloblastoma, but did not retain a copy of the histological report. A month before presenting at our facility, she had a mandibular reconstruction of the second excision’s surgical site.\nOn physical examination, she was mildly tachypneic with the respiratory rate of 22 breaths/min. There was a dull percussion on the entire left hemithorax where no breath sounds were audible. She had a swelling on the right lower jaw measuring 3 cm in diameter with an overlying surgical scar. The remaining physical examination was unremarkable.\nThe chest x-ray showed a homogenous opacity on the left hemithorax with ipsilateral tracheal deviation and mediastinal shift. A computerized tomography scan showed a left posteromedial soft tissue mass with the upper pulmonary lobe atelectasis. A working diagnosis of a mediastinal tumor — probably of thymic origin — was considered, and the patient was subjected to a left thoracotomy. Intraoperatively, the mass was occupying the space between the descending thoracic aorta and the pericardium with partial left lung collapse. Efforts to remove the tumor provoked profuse and uncontrollable bleeding; the patient developed hemorrhagic shock followed by cardiopulmonary arrest, resulting in death.\nPermission for a limited autopsy of the thorax to obtain biopsy samples of the tumor was obtained from the patient’s relatives. The autopsy showed an oval to globular mass in the mediastinum firmly adhered to the pericardium and the mediastinal surface of the left lung ( and ).\nThe tumor was firm in consistency, measured 12 × 9 × 8 cm, and weighed 140 g. At the cut surface, the mass showed a greyish and whitish lobulated appearance with areas of anthracotic pigment deposits, and a greenish fluid was draining from its surface. There were multiple nodules on the pleural surface of the left lung. Cut sections of the lung showed consolidations, with extension of the tumor into the lumen of the left bronchus.\nHistology of the tumor showed nodular aggregates of stellate and spindle-shaped cells arranged in a reticular appearance, which were bounded by low columnar cells arranged in a palisade (). These cells have a coarse chromatin nuclear pattern and moderate amphophilic cytoplasm ( and ). There were very few mitotic figures in the tumor cells ( and ).\nThere was infiltration of the trachea by the tumor cells (). Focal nests of tumor cells within the lung were also seen (). A diagnosis of metastatic ameloblastoma was made. |
We present a case of a 28-year-old G1P0 female at 39 weeks coming for a primary cesarean delivery due to breech presentation. The past medical history was significant for extensive substance use disorder. On initial pre-operative consultation, it was discovered that the patient had oscillated between extensive drug use and rehabilitation cycles for a dozen years, experimenting with multiple illicit substances, causing her family and husband a lot of anger and strife. She underwent extensive rehabilitation and behavioral therapy through the years and was able to stabilize on maintenance dose buprenorphone + naloxone combination lozenge (Suboxone®) 8 mg/2 mg daily. She had been on this stable dose for 2 years prior to discovering that she was pregnant and was seen in consultation 4 weeks prior to her scheduled cesarean delivery.\nThe patient’s major concern was fear of receiving opioids and relapsing back into the addiction spiral. To appropriately plan, a large multidisciplinary team comprised of her addiction specialist, the in-house pain management physician, obstetrical anesthesiologist, the patient’s obstetrician, and labor and delivery charge nurses was assembled. An exhaustive discussion was held that covered all narcotic, non-narcotic, regional and alternative options for the perioperative period. All options, possibilities and acceptable expectations were discussed at length. The patient’s family was a major support structure and with her permission, they too were involved in the final planning. After two pre-operative visits, a plan was agreed upon and the cesarean delivery was scheduled.\nThe patient was the first case of the day and she took her daily Suboxone® maintenance dose. Her extended family and husband were present and provided immense support prior to her being transferred into the operating room (OR).\nIn the OR, standard American Society of Anesthesiologists (ASA) monitors were applied to the patient. She was properly positioned and an epidural was placed at the T8 level via loss of resistance to air technique. The catheter was threaded and a test dose was administered, which was negative for intravascular or intrathecal placement. This was followed by a spinal block with a 27-g pencil-point needle at the L4-L5 level. The 1.6 mL of 0.75% hyperbaric bupivacaine (12 mg) with 200 µg epinephrine was administered. After confirming a bilateral T5 sensory spinal level, the cesarean section was uneventfully performed and a healthy baby was delivered.\nAfter closure, the anesthesia team performed bilateral ultrasound-guided transverse abdominal plane blocks with a total of 15 mL 0.2% ropivacaine per side. The patient was then transferred to the post-anesthesia care unit and an infusion of 0.0625% of bupivacaine at 6 mL/h was started in the epidural. She was also given ketorolac 15 mg and acetaminophen 1,000 mg IV every 6 h for the next 24 h.\nThe patient was seen the following morning for her post-operative check with her epidural having been running for 18 h. She had been out of bed to her chair, starting in the prior evening with minimal discomfort. Her IV ketorolac and IV acetaminophen were switched to oral ibuprofen 400 mg and acetaminophen 650 mg every 6 h in addition to her daily maintenance Suboxone®. After expressing a desire to be free from the epidural, the infusion was paused, and then removed 4 h later, after experiencing minimal pain.\nThe remainder of the patient’s stay was unremarkable. She went home on post-operative day 2, having received no narcotics besides her maintenance Suboxone® dose throughout her entire perioperative stay. Her pain scores were minimal and she was very enthusiastic and satisfied with her care. She expressed immense gratitude and on serial follow-ups we were advised that the patient was doing great at home with no pain, not having taken any medication at all besides her daily maintenance Suboxone®. |
We report the case of a female 9-month-old infant who was presented to the emergency department because of the absence of micturition for 3 days. The pregnancy and delivery history was normal. Clinical examination showed no congenital abnormalities. The infant's body weight was 7.5 kg. Neither the bladder nor the kidneys were palpable clinically. The infant was quiet in bed. A Foley urethral catheter was inserted and revealed no urine in the bladder. Serum creatinine was 6.7 mg/dl, serum K was elevated to 6 mEq/dl, Na was 132 mEq/dl, and hematocrit was 32%. Abdominal ultrasound showed mild hydronephrosis of the left kidney (). The right kidney was atrophic with poor corticomedullary differentiation and stones (). Kidney, ureter, and bladder showed four radiopaque shadows opposite the left sacroiliac joint and one in the renal area on the right side. Moreover, another radiopaque shadow was found at the level of L3 on the right side (). A percutaneous nephrostomy tube was inserted into the dilated left pelvicaliceal system for drainage for several days. After the intervention, the serum creatinine level improved to 1.6 mg/dl and K returned to a normal level on the third day. Exploration of the left middle third of the ureter was performed with the removal of four stones and a double (DJ) stent was fixed. Creatinine returned to 0.5 mg/dl after 2 weeks. The stent was removed after 2 weeks. Chemical analysis of the stones revealed pure cystine crystals. Right kidney function will be evaluated by [DMSA] isotopes. A right nephrectomy may be considered because of the stones and infection. Metabolic workup for cystinuria was performed via a positive nitroprusside test. The diagnosis was confirmed by stone analysis by use of chemical methods. Three months later, the results of a renal ultrasound and renal function tests with urine analysis were repeated and were shown to be normal.\nA follow-up protocol was given to the parents to prevent the recurrence of stones. It consisted of urine analysis every 2 months and serial ultrasonography for early detection of stone formation every 3 months. Plain abdominal radiography was not necessary. A detailed feeding history of the patient was reevaluated during each visit by the pediatrician. The parents were advised to ensure constant, ample fluid intake during both daytime and nighttime. Potassium citrate (1-1.5 mEq/kg per day) or a mixture of potassium citrate and sodium citrate (Polycitra) was administered with monitoring of urinary pH. Penicillamine was avoided because of its serious side effects, such as hypertension, heart failure, renal failure, and electrolyte imbalance. |
A 38-year-old male with a past medical history of right inguinal hernia, presented to the emergency room with a 10-day history of right flank pain. His pain came in waves and fluctuated in intensity throughout the day, in the last 24 h prior to admittance his pain became more intense and was followed by recurrent episodes of severe vomiting.\nOn clinical examination, a dehydrated and tachycardic patient was encountered. He had pain in his right flank that radiated to his lower abdomen and groin. Complete blood count (CBC) and urinalysis appeared normal, however, an abdominal computed tomography (CT) detected bilateral nephrolithiasis, multiple 5 mm, and 6 mm lesions were identified on both the right and left kidney. Also, a 7 mm hyperdense shadow in the middle of the right ureter that caused proximal hydronephrosis was detected as well (Fig. A and B).\nUrologist consultation was required and he underwent ureteroscopic lithotripsy without complications, the ureteral stone was removed and a right double J-stent was placed. Afterward, ESWL was decided for the right nephrolithiasis. A total of 3000 shocks were delivered at a maximum power of 15 kV and a rate of 80 shocks per minute. After the therapy was completed, the patient was discharged without any apparent complication. Nonetheless, 12 h after ESWL, severe pain appeared in his right flank, thus he presented to the emergency room. On clinical examination, a dehydrated, tachycardic and hypotensive patient was encountered. Pain was discovered on his right flank without tenderness. A contrast-enhanced abdominal CT revealed a 9 × 6 × 4 cm3 subcapsular liver hematoma, with an estimated volume of 300 ml that involved almost the entire right liver, without an active contrast material extravasation (Fig. A–C).\nAfter adequate resuscitation and analgesia, his pressure and vital signs were stabilized, laboratory exams did not reveal leukocytosis or anemia however his hemoglobin (Hb) level decreased 4 points from 18 to 14 g/dl. He was admitted for close surveillance and under continuous monitoring. During hospitalization, the patient remained stable without tachycardia or episodes of hypotension, his urinary output was normal and his pain disappeared. However, 48 h after admittance, a new CBC revealed a Hb of 10 g/dl, a new CT was performed and the liver hematoma had grown ~50% (Fig. A and B), yet as the patient remained stable, non-operative management of his liver hematoma was decided.\nBlood transfusion was not required and after 7 days of conservative therapy, as the patient was completely asymptomatic and his hemoglobin remained stable, he was discharged. On follow-up controls patient is doing well, without any pain, his last Hb was 11 g/dl and a remarkable reduction of the hematoma was seen on a new CT scan. |
A 70-year-old Japanese man with melena that began five days before admission was admitted to our hospital. He complained of abdominal discomfort and epigastralgia. Five years before, he had undergone a right upper lobectomy for lung cancer (T1b N0 M0 Stage IA). Physical examination revealed no abnormal findings associated with the abdomen except a surgical scar on the right side of his chest. Evaluation of laboratory data on admission revealed that his hemoglobin level was 7.0 g/dL and hematocrit value was 23.1%. Upper gastrointestinal endoscopy revealed a distinctly protruding lesion (diameter: 5 cm) at the lesser curvature of the middle third of the gastric body (Figure ). The surface of the tumor bled easily on contact with the endoscope. Microscopic examination of hematoxylin and eosin (H&E)-stained biopsy specimens of the lesion revealed sheets of spindle cells. Immunohistochemical studies showed that the tumor cells were positive for S-100 and negative for c-kit, CD34, and smooth muscle actin. The MIB-1 index was 48.5%. Based on these findings, the tumor was diagnosed as a gastric malignant schwannoma. Abdominal computed tomography (CT) revealed a thickened posterior wall of the gastric body (Figure ). There was no evidence of lymph node swelling surrounding the stomach or metastatic liver tumors.\nHe underwent a distal gastrectomy with regional lymph node dissection. Macroscopically, the elevated lesion was approximately 6 × 5 cm in diameter and located at the lesser curvature of the gastric body (Figure ). The covering mucosa was ulcerated. The tumor was located mainly in the proper muscle layer. There was no lymph node involvement and the surgical margin was negative for tumor cells. Microscopic examination of the resected and H&E-stained specimens showed a spindle cell neoplasm arranged in a palisade manner that was consistent with a schwannoma (Figure ). Mitosis was scattered with 10 mitoses per 50 high-power fields. Immunohistochemistry revealed that the tumor cells were positive for S-100 protein and negative for c-kit and smooth muscle actin (Figure ). These histopathological and immunohistochemical findings are consistent with a gastric malignant schwannoma.\nHis postoperative course was uneventful and he was discharged from our hospital on day 12 after surgery. However, abdominal CT performed three months after surgery revealed multiple liver metastases and ascites (Figure ).. He died five months after surgery without undergoing any additional treatment. |
A 59-year-old female initially presented to the neurosurgery clinic with complaints of insidious onset of right leg radicular pain. She has no history of numbness, tingling, weakness, or bowel or bladder incontinency. She has never experienced any similar pain episodes. Physical examination had significant right side straight leg test positive at 45 degrees and left lower extremity at 70 degrees. Plain lumbosacral X-ray was inconclusive. The patient's lumbar MRI reported right L4-L5 lateral disc herniation with extrusions and the patient had partial response to conservative management.\nShe underwent a minimally invasive hemilaminotomy of right side L4-L5 and removal of the extruded disc particles. The patient had an uneventful postoperative course. She had immediate pain relief after surgery and was able to ambulate on postoperative day one. She was discharged from the hospital the following day after surgery.\nThe patient reported a new pain experience on the left buttock that started insidiously two weeks after discharge. The pain was constant burning at the left pelvic region, with sharp shooting pain in the left leg. The patient reported pain radiating in multiple trajectories but on clinical assessment had no specific dermatomal distribution. Pain was not aggravated by coughing or sneezing but is more during a sitting position.\nOn a new physical examination, a healed surgical scar of posterior approach was seen over the midline lower lumbar area. There was no sign of infection at the surgical scar. The patient walked with an antalgic and waddling gait. There was a significant Tinel sign on the left gluteal region over sciatic nerve. Straight leg rising test was negative in supine, sitting, and prone position. Detailed physical examination included spine mobility with great range of motion with no pain, manual muscle testing with normal muscle strength, and deep tendon reflexes were normal. Piriformis stretch test was significantly positive and could aggravate the patient's pain.\nThe patient had an MRI of lumbosacral, which showed postoperative changes with no disc herniations at the lower lumbar spine.\nThroughout the patient's multiple pain clinic visits, we began a multimodality treatment, which included antiepileptic and antidepressant antalgic medication management, muscle relaxant, short acting narcotic medication, and physical therapy.\nWe decided to perform a diagnostic piriformis muscle block, when the combination of muscle relaxants, antidepressants, and antiepileptic medications did not provide significant relief.\nThe patient had a piriformis muscle injection under ultrasound guidance where 5 cc of 1% lidocaine solution was injected in the targeted muscle. The patient demonstrated immediate pain relief.\nRepeated piriformis muscle injection was able to provide short course pain relief. We decided to do a pelvic MRI to visualize suspected pathologies. An MRI of the pelvis, without contrast, including coronal T1, STIR, axial T1, T2 fat-sat, and STIR sequences, was done. MRI imaging revealed left side sciatic neuritis (see ).\nWith the diagnosis of sciatic neuritis at the left pelvic region, we continued on our multidisciplinary aggressive pain management with a reasonable time frame. The patient was very motivated and followed all steps of the recommendations with significant efforts. She did not respond to the treatment plan after four months of continued aggressive management. We decided to proceed to neuromodulation modality by placing a temporary percutaneous thoracic spinal cord stimulator (SCS). The patient reported a complete response with almost 100% pain relief during one week of the SCS trial. We were able to decrease the patient's medications dramatically during the trial period. The patient then underwent a permanent implant of SCS. On a one-year followup, the patient had remained pain-free without using medication. |
An 11-year-old Turkish boy consulted to the Gazi University Faculty of Dentistry Department of Pediatric dentistry clinic (Ankara, Turkey) with sensitivity to cold and sweets. No spontaneous pain was reported by the patient. The medical history was noncontributory. In the clinical examination gross occlusodistal caries was observed on his mandibular right second premolar (number 45) without signs of extraoral or intraoral swelling or sinus tract formation (). The lower second premolar tested negative to percussion and palpation tests and the mobility was within the normal limits. Pulp vitality tests using electric pulp test showed a normal positive response without lingering sensation. Additionally, the adjacent teeth responded positive and within the normal limits to the electric pulp test. Radiographic examination revealed a close relation between caries and the pulp horns and undeveloped root with a wide-open apex and no evidence of periradicular pathology ().\nAs a result of clinical and radiographic assessments, the pulpal status of the lower right second premolar was determined as vital with reversible pulpitis due to caries. The treatment plan included the removal of the carious lesion and clinical evaluation of the pulp exposure. Vital pulp therapy including partial pulpotomy with calcium hydroxide was planned and an informed consent was obtained from patient's father.\nAfter local anesthesia, the tooth was cleaned with pumice and isolated with a rubber dam. Caries removal was performed with a sterile high-speed 801-016 ML diamond round bur with copious irrigation which was continued with steel round bur and exposure of pulp horns with moderate bleeding was observed. The amputation of the 2-3 mm damaged pulp was executed. The cavity was rinsed with saline and a sterile cotton pellet moistened with saline was used to apply moderate pressure to the exposed pulp for 5 min. After the homeostasis was achieved, calcium hydroxide was gently placed on the exposed pulp. A glass ionomer cement (Kavitan Pro, Spofa Dental, Czech Republic) was used as base material and the tooth was restored with amalgam ().\nThe patient was scheduled for 1 month follow-up in order to assess root developmentvitality and examine any signs or symptoms. The parents were informed to call the dental clinic if the patient reported any pain or discomfort.\nAt the 1-month follow-up, the clinical examination showed an intact restoration and absence of any abnormal signs or symptoms. The patient reported no pain or discomfort. The tooth tested positive to the electrical pulp test and radiographic examination showed continued development of root and maturation of the root ().\nAt 3 and 6 months, no abnormal findings were observed, and the clinical examination revealed no changes from the previous visit. The pulp continued to respond positive to the electrical pulp test. Root maturation was observed as a significant progression when compared with the preoperative radiographs (Figures and ).\nThe patient was reexamined 12 months after treatment began, and reported as asymptomatic. The pulp tests repeated and the tooth responded normally to the tests. A periapical radiographical assessment showed continued root development and apical closure (). Root maturation (maturogenesis) appeared normal with no evidence of internal root resorption or pulp calcification. |
A four-year-old girl presented to the emergency service with painful left hip and fever. There was no previous relevant medical history. There were no other local or systemic symptoms, except for a cervical adenopathy. On physical examination, she walked with a limp, and movements of the left hip were painful (mainly external rotation), but not restricted. Blood exam revealed anemia (Hb 8.7 gr/dL), normal WBC, ESB of 123 mm, and reactive C protein of 149.7 mg/L. An initial X-ray to the pelvis revealed no changes. An ultrasound of the left hip was performed revealing small infusion and synovitis. Guided puncture was then performed being macroscopically compatible with reactive arthritis, and general and bacteriological tests were demanded. Because of the unusual characteristics of the pain, a CT scan to the abdomen and pelvis was performed revealing a left adrenal mass and retroperitoneal adenopathies in the celiac trunk and superior mesenteric artery ().\nDespite the painful complaints of the patient, no bone or articular involvement was found in the CT scan. No further alterations were reported in the thoracic CT scan or in peripheral blood smears. Bacteriological examination of the hip effusion was negative. MRI was also performed. The direct myelogram was compatible with infiltration from neuroblastoma. Bone marrow biopsy and cervical adenopathy specimens were collected to perform histological diagnosis. Skeletal scintigraphy demonstrated numerous points of osteoblastic activity compatible with metastatic activity, and the 12 iodine-123 metaiodobenzylguanidine scintigraphy concluded the following: “Abdominal mass with low expression of noradrenergic transporters. Diffuse bone metastasization with high expression of noradrenergic transporters. No other soft tissue involvement was detected.” In the histological report of the cervical adenopathy, the diagnosis of neuroblastoma NOS was performed. Immunohistochemistry revealed extensive expression for synaptophysin and CD56 (NCAM) and absence of expression of myogenin. ().\nBone marrow biopsy revealed extensive metastatic involvement. The patient started chemotherapy two weeks after admission, with 8 cycles of rapid COJEC protocol. After six months of follow-up, the primary tumor was still without criteria for resection, despite a decrease in the metastatic involvement. Given the chemotherapy-related renal toxicity, it was decided to proceed with irinotecan in combination with temozolomide (TEMIRI). After thirteen months of follow-up, no significant regression of the primary tumor occurred, so surgery was contraindicated and the patient was proposed for stem cell treatment. |
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