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A 35-year-old right-handed male presented to the emergency department at our hospital with a 24 history of a painful swollen right thumb with surrounding cellulitis. The patient worked at a metal fabrication factory, dealing with cutting, and welding of various metals from international suppliers. Other than a lumbar decompression a few years ago, he had no previous medical history of note. As a child, the patient had received a full immunization program but had no further boosters. There was no history of acute trauma, and he was systemically well. He was sent home with oral flucloxacillin and advised to return if symptoms worsened.\nA few hours later, he developed a high temperature with chills and returned to the emergency department where he was referred to the on-call orthopedic team. On further questioning, the episode had started with pain and swelling in the distal phalanx of the thumb the previous day. While at dinner, he experienced an episode lasting 30-60 s, when his thumb had become rigid, and he was unable to use cutlery, but this resolved spontaneously.\nOn examination, his right thumb and thenar eminence were grossly swollen, erythematous, and exquisitely tender to palpation. He held the thumb in flexion, with the interphalangeal and metacarpophalangeal joint movements very limited and painful. There was a small superficial collection dorsally on the radial aspect of the proximal phalanx base. Ascending lymphangitis was present extending into the axilla but without palpable lymph nodes. Furthermore, noted was a small, nearly completely healed puncture wound at the fingertip. He recalled that 2 weeks ago, he had been injured with a metal splinter that he was able to extract it using a needle; however, the wound became infected. It discharged pus for a few days, and then seemingly resolved of its own accord, so he did not seek help.\nThe patient was then admitted for intravenous antibiotics and strict elevation. Blood tests showed mild neutrophilia (8.8) and elevated C-reactive protein (114). Calcium, magnesium, liver function, and thyroid function tests were all within range. All investigations carried out on admission are summarized in .\nThe patient was taken to theater the next morning for a formal wound exploration and washout. At the time of tourniquet inflation, his right hand went into a carpopedal spasm, with full flexion of the metacarpophalangeal joints and wrist joint of the right hand with extension of the interphalangeal joints. His hand would remain in this position until an infusion of a muscle relaxant was given to allow surgery to proceed. At surgery, there was only a superficial collection with no tendon sheath involvement.\nThe unusual intra-operative events were discussed with a consultant microbiologist, who agreed that the signs would be consistent with a case of localized tetanus. Metronidazole was added to the antibiotic regimen, and a single infusion of human immunoglobulin (10,000 units) was given, as no tetanus immunoglobulin was available within the trust.\nA single dose of tetanus toxoid was also given as a booster, and the patient was then sent home after 48 h of monitoring. Wound swab taken intraoperatively did not grow any organisms. |
A 31 years old male with a past medical history of Hepatitis C, depression, and prior suicide attempts was brought to the emergency department (ED) by his family. Three days prior to admission he had ingested about 10 - 20 cc of antifreeze (ethylene glycol), 100 mg of Vistaril and 200 mg of Doxepinel in a suicide attempt. He had no contributory past surgical history or family history. He admitted to addiction to cocaine and heroin and was discharged from a detoxification facility 1 week prior to admission. He stated that he had no recollection of events that transpired between the ingestion of ethylene glycol and his presentation to the ED. He was found unresponsive at home and brought into the hospital and was awake and alert on presentation to the hospital.\nIn the ED the patient had a blood pressure of 103/59, heart rate of 92, respiratory rate of 18 and temperature of 100 degrees Fahrenheit. The patient was alert and oriented to self, location and time. The heart rate was regular with no murmurs, rubs or gallops, and the lungs were clear to auscultation, with no rales, rhonchi or wheezes. The abdomen was soft, non tender, not distended with no organomegaly, and bowel sounds were present in all four quadrants. The extremities had no edema, and had acceptable pulses were present. He was able to follow commands and did not have any short term memory defecit. Cranial nerves were intact, strength was 5/5 in all extremities, sensorium was intact to pinprick and light touch, and deep tendon reflex responses appropriate.\nThe patient was noted to have transaminitis which was attributed to Hepatitis C, and was also found to be in a high anion gap metabolic acidosis (17 mEq/L) with acute kidney injury (Creatinine 12.55 mg/dL), with total urine output of 5 mL during the first eight hours. Osmolar gap on presentation was within the normal range. He was admitted to the intensive care unit for treatment and observation and was started on intravenous normal saline, and oral pyridoxine and thiamine. An admission urinalysis had shown 3+ protein, 0 - 3 red blood cells, 6 - 12 white blood cells (per high power field), moderate bacteria, no casts, and no crystals. The fractional excretion of sodium was calculated to be approximately 20%. Renal sonogram revealed a right and left kidney of 13.4 and 14.2 cm respectively, with increased echotexture, and no hydronephrosis, masses or calculi. Serologic workup for causes of renal failure was found to be negative (C3, C4, antinuclear antibody, rheumatoid factor, anti-neutrophil cytoplasmic antibody and HIV). The cause of kidney injury was presumed to be due to oxalosis from the ethylene glycol ingestion. He continued to be anuric despite volume repletion and his electrolytes worsened, so he was started on hemodialysis. Renal core biopsy was obtained, and showed a significant amount of tubular injury caused by oxalate crystal deposition (Fig.1).\nFive days after admission the patient began complaining of tinnitus in his right ear as well as vertigo and acute vestibular neuritis was suspected. MRI of the brain along with a lumbar puncture was performed. He was found to have sterile meningitis. He was started on steroids and meclizine without significant improvement in symptoms.\nTwelve days after admission the patient began to complain of hearing loss in his right ear and developed left facial droop (distribution of V2 and V3) and right eye ptosis. His mental status began to worsen as he became increasingly lethargic, and he developed a fever and leukocytosis. He refused a repeat LP and empiric antibiotics were started. He began to have difficulty swallowing which was severe enough to require that all feeding be via a nasogastric tube. By this time, his hearing loss was profound, and it became difficult to communicate with him. Subsequently he began to have progressive shortness of breath and required intubation and mechanical ventilation. He failed to tolerate the removal of ventilatory support, and underwent tracheostomy and percutaneous endoscopic gastrostomy tube placement.\nThree weeks after intubation, the patient began to regain consciousness and had progressive improvement of his mental status. Pt was successfully weaned off of the ventilator at this point. He began to open his eyes, then began to answer questions appropriately and progressively improved until he returned to the baseline mental status that he displayed upon arrival to the ED. He was discharged to an inpatient rehabilitation center where his condition continued to improve and he was able to fully ambulate after several weeks. He continued to require hemodialysis, but began to produce urine. Repeat microanalysis of his urine failed to indicate active sediment and his renal function did not improve. The patient was finally discharged home with outpatient hemodialysis three times a week. Approximately 5 months later, his renal function began to improve and hemodialysis was dicontinued. A partial facial palsy remains, as well as a mild hearing deficit. |
A 15-year-old male with a history of Kawasaki disease diagnosed as a 4-month-old infant in Japan was referred to our institution when he was 13 years old. As per his medical records, he received a dose of IVIG after approximately 2 days of symptoms with no additional doses at any point. Subsequently, he developed giant coronary artery aneurysms of the right and left coronary systems. By echocardiography at that time, the left anterior descending (LAD) measured 7.3 mm in diameter, left circumflex (LCx) 5.5 mm, and right coronary artery (RCA) 10 mm. He was treated with aspirin, dipyridamole, and ticlopidine. In July 2003, coronary angiograms showed occlusion of both the LCx and RCA with recanalization in both coronary arteries.\nLater that year, after his family relocated to the United States, clopidogrel was substituted for ticlopidine and dipyridamole. In 2006, at the age of 6, after returning to Japan, repeat imaging suspected thrombus formation in the aneurysm in the LAD, and he was started on warfarin and aspirin. A scintigram at 12 years old with exercise loading showed no perfusion defects. A coronary computed tomography CT angiogram was performed in July 2013. Based on these findings, doctors in Japan recommended that he participate in low impact activities and no contact sports and with his everyday activities he reported no chest pain.\nHe was first seen in our institution at the age of 13 years, and his initial echocardiogram demonstrated a moderate-sized saccular aneurysm of the mid left anterior descending coronary artery, measuring up to 0.79 cm in diameter and 1.44 cm long. There was a small- to moderate-sized aneurysm of the right coronary artery, measuring 0.5 cm in diameter and 1.23 cm long (). There was no evidence of thrombus, his warfarin was switched back to clopidogrel, and he remained on aspirin. Annual follow-up demonstrated that the aneurysms were stable in size, but his most recent echo showed a new echogenicity along the saccular wall of the LAD aneurysm concerning for possible thrombus.\nGiven these findings, he was referred for low-dose coronary CT angiography (). The CT demonstrated a saccular aneurysm of the LAD with maximal diameter of 9 mm, containing mural thrombus and calcification, at the origin of the first diagonal branch (Figures and ). There was an additional small saccular aneurysm of the LCx with diameter of 6 mm and narrowing of the RCA ostium with an associated irregular area of hypoattenuation possibly representing thrombus, followed by a long saccular calcified aneurysm extending into the mid-vessel ().\nGiven the presence of coronary stenosis, he was referred for exercise treadmill stress test with SPECT myocardial perfusion imaging to evaluate the presence of inducible ischemia. His exercise capacity of 17 metabolic equivalents was excellent for age and gender. He had an appropriate hemodynamic response with no ST segment changes noted on electrocardiogram. At peak stress he was injected with 6.4 mCi of Tc99m sestamibi. Supine, prone, and gated SPECT images of the heart obtained after stress demonstrated normal myocardial perfusion with no evidence of infarction or ischemia, normal function and wall motion, and a left ventricular ejection fraction of 68% (). We performed our patient's SPECT with a radiation dose of under 2 mSv for the entire study.\nClinically, the patient has remained asymptomatic. He is at baseline a sedentary and overweight young man, with a BMI of 23 (86th percentile). According to the Japanese Kawasaki protocol, he would be allowed moderate exercise, but he was further limited to only light intensity sports. The clopidogrel was discontinued and he was restarted on warfarin given the presence of thrombus and size of the LAD aneurysm, and he was continued on aspirin as well. |
A 25-year-old male presented with a history of trivial fall from a ladder (5 Ft) and landing on the right heel. He complained of pain and swelling. On examination, he had swelling over the medial aspect of right heel (). The skin over the swelling was stretched and it was soft in consistency and tender. No relevant history and family history in view of bone cyst and malignancy. No evidence of any risk factors.\nRadiograph of right ankle revealed an eccentric, expansile, and cystic lesion of the calcaneum with thin septae traversing the cystic cavity giving it a blown out or soap bubble appearance. There was a cortical breach suggesting fracture (). In our case, we avoided computed tomography/magnetic resonance imaging (CT/MRI) due to the expense, and we proceeded with biopsy and histopathological examinations, which is more informative.\nThe patient underwent two staged procedure: First for biopsy followed by second procedure after confirmation by histopathological examination.\nUnder strict aseptic precautions, biopsy was done under anesthesia. The specimen was sent for histopathological examination and it was diagnosed by a pathologist as aneurysmal bone cyst.\nA differential diagnosis of aneurysmal bone cyst or giant cell tumor with pathological fracture was made. Under spinal anesthesia and strict aseptic precautions, the lesion was curetted and the cavity was filled with iliac bone grafts ( and ). The curetted specimen was sent again for histopathological examination, and the diagnosis was confirmed (). The wound was closed, dressed, and below knee posterior plaster slab was applied.\nPostoperatively, a nonweight bearing crutch walking was allowed for 6 weeks followed by partial weight bearing for further 4 weeks. Follow-up was done on 3rd and 6th month of post-operative ( and ). The patient returned to his activities at the end of 6 months, and we are still continuing the follow-up because the recurrence usually happens within 1 year. |
A 27-year-old right handed man refer to orthopedic uniclinic of Isfahan Al-zahra hospital on March 2011 with a 4 years history of gradual increase in size and deformity of the distal part of his left middle finger. Pain was a marked feature that disabled his life and was only relieved temporarily by analgesic and aggregated in nights. Twelve months earlier he had been treated for local infection, initially with a course of antibiotics. This treatment had been unsuccessful.\nOn examination, the finger was enlarged beyond the distal interphalangeal joint and clinically looked like an isolated clubbed digit. The pulp was big and there was increase in both longitudinal and transverse curvature of the nail-plate. The skin at the base of the nail-plate was swollen and shiny and there was a small amount of fluctuation. The nail-fold angle was obliterated. The patient did not report any previous trauma or infection; laboratory data, including laboratory tests for inflammation and rheumatic conditions, were normal, and CRP was negative. The middle finger was warm on palpation, and there were no evident superficial reticular veins. There was a slightly reduced range of motion in the distal interphalangeal joint compared to the contralateral hand. No satellite lymphadenopathy was observed.\nRadiographs revealed soft tissue edema on volar and dorsal side of finger. An oval shaped sclerotic mass in base of volar aspect of distal phalanx with tinny radiolucent border was seen []. In MRI, there was a 1.2 cm mass-like lesion in volar aspect of proximal half of the distal phalanx, being isosignal to muscles in T1W, and hyper in T2W images, causing saucerization of anterior surface of the bones. Nidus was not seen in plain radiography, but edema was obvious in MRI []. Unfortunately, we have no CT scan of finger in this patient.\nBased on clinical and radiographic findings, the presumptive diagnosis of osteoid osteoma was made (however other conditions, including infection and giant cell tumor were considered) and the patient underwent surgery. Through an approach on the volar side of the distal phalanx a hole was made in the area of osteosclerosis, thus revealing a small, less dense and relatively hyperemic area that flexor profundus tendon was attached to it. Then mass was removed en-block and Curettage was performed with a small curette and then tendon was attached to bone with tension suture, and specimen referred to pathology lab. Histological examination confirmed the diagnosis of osteoid osteoma, since the typical nidus was found in the histological specimen [].\nThe limb was immobilized with dorsal short splint for repair of tendon. In the first days following surgery, the pain with which the patient used to live with had already disappeared.\nThe patient, recently was seen 7 days after the operation, had no pain. Aspect and size of the finger and of the nail were similar to the contralateral one, having normalized a few months after surgery. |
A 35-year-old G4P2 attended for reproductive surgery consultation to discuss a variety of problems she began to experience subsequent to undergoing the Essure® procedure for permanent sterilization. HS was performed in October 2012 under general anesthesia; pre-procedure pelvic ultrasound was unremarkable. Before HS, the patient was in good general health and all of her pregnancies were established without medical assistance. Cervical cytology was routinely normal, the patient did not smoke and she never had a miscarriage. Soon after the Essure® devices had been placed, the patient reported gradually increasing pelvic pain, headache, dizziness, lower extremity paresthesia and fatigue. In the first six months following the Essure® procedure, the patient registered a 20 pound weight gain. The patient’s concerns persisted over a three-year interval, although her gynecologist was disinclined to attribute any of the problems to Essure®.\nThree months after HS, the patient presented for hysterosalpingogram as recommended. While the study did confirm bilateral tubal occlusion, it also revealed an abnormal (bifid) uterine cavity. 3-D ultrasound or other imaging was not performed to clarify or refine the diagnosis of the uterine anomaly and the patient received no further counseling.\nThe patient later requested surgical removal of the Essure® implants and the possibility of device migration required evaluation (). A repeat HSG was obtained in 2015. This study revealed minimal device migration and no contrast dye spill from either Fallopian tube, a deeply bifid uterine cavity was again the most conspicuous finding ().\nAt our center the patient was counseled about the atypical nature of her Essure® placement given the anatomical abnormality of the uterus. After obtaining informed consent, the patient underwent diagnostic hysteroscopy which verified an abnormal bifid uterus (). The ostia on the right was normal as was the entire right uterine segment. However, the Essure® device on the left partially extended into the endometrial compartment (). The exterior uterus was consistent with ESHRE Class IIb anomaly (uterine septum) (). There was no evidence of tissue perforation by either contraceptive device. Laparoscopic dissection of the left cornu and partial tubal resection bilaterally achieved complete removal of the Essure® implants. Removal of terminal markers was confirmed. Electrocautery was maintained at 40 W power throughout the case, blood loss was estimated to be less than 50 mL. The patient was discharged three hours after surgery and her postoperative convalescence has been uneventful. |
A 51-years-old Japanese man presented with a tumor in the right temple and underwent resection of the tumor at a medical college hospital in 1979. The histological diagnosis was hemangiopericytoma. Thereafter, he demonstrated local recurrence many times, underwent resection 5 times and embolization was performed 2 times. Despite these treatments, this tumor relapsed in the temple again and was treated by irradiation (total 61.8 Gy) in November 2002. However, the tumor appeared to persist and multiple lung metastases were detected. After 4 months, he consulted our hospital for their treatment. On the first medical examination at our hospital, we evaluated the skull base tumor with MRI (Figure ) and lung metastasis with chest-CT. Because lung metastasis remained stable, we performed Choline-PET and compared findings to those of FDG-PET. Choline accumulated in the tip of the right temporal lobe (SUV max 4.0) but FDG-PET could not detect in the tumor because it accumulated strongly throughout the whole brain without demonstrating the tumor (Figure ). In addition to PET study, angiography via the right external carotid artery demonstrated the lesion strongly (Figure ). Based on these examinations, we diagnosed this lesion as residual hemangiopericytoma. Then he underwent resection of the residual skull base tumor in February 2004. First, we incised the coronary line at the front of the head, and tried to approach this tumor via right temporal craniotomy without facial incision. We resected completely the tumor together with a part of dura because of dural invasion. The tumor showed diffuse invasion of tissue around the mass lesion. We reconstructed the skull base by galeal flap. In addition, we plugged the dead space after tumor resection with abdominal fat tissue (Figure ). There were no complications postoperatively. This tumor demonstrated abundant blood vessels and was composed of round or spindle-shaped cells, including dilated staghorn-shaped vessels (Figure ). Immunohistochemically, expressions of Vimentin and CD34(+) in cancer cells were positive on immunostaning, while these of S-100 protein and cytokeratin were negative(Figure ). This histological diagnosis was recurrent hemangiopericytoma. The patient demonstrated a favorable postoperative course and was discharged from our hospital on March 2004. To date, we have not detected any recurrence in the skullbase for 7 years postoperatively but lung metastases increased gradually. Currently, he can work as well as he could before onset of the disease. |
A 51 year-old man was admitted on the surgical ward in November 2003 for a right-sided abdominal pain which had been present for three days. Clinically, he was jaundiced with a high temperature. Blood tests showed white cell count (20.8 × 103/mm3), bilirubin (103 mg/dl) and ESR (75 mm/h). All other blood tests were normal. Ultrasound and CT scans of the abdomen revealed fluid collection/mass measuring 14 cm in the right hepato-renal space. The mass was separated from the liver, pancreas and the right kidney, but there was lack of definition of the right suprarenal gland. Because the patient was jaundiced, we thought that draining the cyst would relieve the pressure on the biliary system and resolve jaundice. About 150 ml brown-coloured fluid was aspirated from the mass under CT guidance and a sample was sent for histology. Cytological examination of the sample reported presence of amorphous material with occasional histiocytic cells. There was no evidence of malignancy. The patient was discharged in December 2003 after making good recovery.\nIn January 2004, the patient was readmitted for an abdominal pain and pyrexia. A repeat CT scan confirmed recollection of fluids at the same site (Figure ). He underwent a laparotomy for excision of the mass and right adrenalectomy in February 2004. The specimen was sent for histopathology.\nMacroscopical examination of the specimen reported an open cyst measuring 190 mm × 110 mm × 0.5 mm. The outer surface was pale-dark brown with an irregular defect measuring 1.8 cm × 1.5 cm in one area. Another area showed an adrenal tissue measuring 3.0 cm × 1.0 cm × 0.5 cm with yellow areas on the surface of the cyst wall. The inner area of the cyst wall was wrinkled with an exudate-like substance coating it in places.\nMicroscopically, the sections showed that the normal adrenal gland was adherent by fibrous tissues to the external wall of the cyst, but the cyst was not arising from the adrenal. The cyst wall consisted of a thick layer of fibrous tissues which showed focal calcifications and areas of acute and chronic inflammation. There was no epithelial lining present, but clusters of cholesterol crystals were adherent to the internal cyst wall. Granulation tissue also formed part of the lining of the cyst (Figures &). There was no atypia or malignancy. The overall appearances were those of an idiopathic benign retroperitoneal cyst. |
A 79-year-old woman presented with post-menopausal bleeding and pain in lower abdomen for 2 months. The obstetric history of the patient was P2L2 and the patient had attained menopause 30 years back. The age of menarche was 14 years. On examination, a firm mass was palpable in lower abdomen extending upto umbilicus. On vaginal examination, uterus size could not be made out and a large abdomino pelvic mass was palpable. Abdominal ultrasonography revealed a normal-sized uterus with endometrial thickness of 7 mm and a 20 × 10 cm solid mass in pelvis and lower abdomen. Left ovary was not seen separately and there was minimal ascites. Patient underwent endometrial aspiration and it was reported as endometrioid adenocarcinoma (Grade 1). Patient underwent staging laparotomy which revealed a 20 × 10 cm solid left ovarian tumor. The right ovary was normal and there was minimal ascites which was sent for cytology. On exploration, intestines, liver and biliary tract, pancreas, omentum, and fallopian tubes were without lesions and the uterus was of normal size. The patient underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy, infra-colic omentectomy, and pelvic lymphadenectomy and the specimen was submitted for histopathological examination.\nLeft ovary measured 21 × 14 × 10 cm and the cut section was homogenously fleshy with areas showing yellowish discoloration. Cut section of the uterus revealed a 4 × 3 cm exophytic fundal growth in the endometrial cavity infiltrating less than one-third of the myometrium. The right ovary, omentum, and bilateral fallopian tubes were grossly normal. On microscopic examination, left ovary showed features of a stromal tumor with minor sex cord elements []. The tumor comprised mainly of fibroma-thecoma component (more than 90%) with few aggregates of granulosa cells []. These granulosa cell aggregates were immunoreactive for inhibin [] and calretinin [].\nMultiple sections from the endometrial growth showed features of a well-differentiated endometrioid adenocarcinoma (Grade I). The omentum and lymph nodes were free of tumor.\nThe patient did not receive radiotherapy post-operatively and she is on regular follow-up. |
We report the case of a 69-year-old transgender woman. The patient underwent genital reconstruction surgery at the age of 25, by combined penile and scrotal skin inversion, and has been on hormone therapy ever since.\nOur patient was found having a left renal mass at the age of 65; therefore, she underwent left radical nephrectomy and retroperitoneal lymph node dissection. The pathology showed a left kidney renal cell carcinoma of clear cell type, grade 4. It invaded into the perinephric fat, and grossly extended into the renal vein. 0/4 lymph nodes were involved. The resection margins were negative for tumor. She developed an osseous metastasis in her left posterior rib 1 year after nephrectomy (at the age of 66), and received local radiation for the bone metastasis, as well as systemic targeted therapy by tyrosine kinase inhibitors.\nIn the fourth year after nephrectomy (at the age of 68), CT scans showed a new 1.5 × 1.5 cm nodule in the left lower lung of the patient. This lesion was presumed to be a new metastatic renal cell carcinoma. However, the nodule slowly grew to 3.3 cm in size during the next 12 months despite the systemic therapy for renal cell carcinoma.\nA transbronchial biopsy of the left lower lung lesion was performed, which showed a basaloid poorly differentiated carcinoma (Fig. ). Immunohistochemical stains showed that the tumor cells were diffusely positive for CK5/6 (Fig. ) and p40 (Fig. ), and negative for TTF1, PAX8, synaptophysin, and chromogranin. Ki67 stained approximately 80% of the tumor cell nuclei (Fig. ). These results supported a diagnosis of poorly differentiated SCC rather than a metastatic renal cell carcinoma or small cell carcinoma.\nSince the CT scan did not demonstrate abnormalities other than the lung lesion and the bone metastases, a PET-CT was performed. Consistent with the previous CT scan, PET-CT showed an FDG-avid density within the left lower lung (Fig. ), with multiple additional nodular densities within both lungs. Additionally, an irregular region of marked increasing FDG activity that measures approximately 6 cm in the craniocaudal dimension was present within the pelvis with multiple moderate- to high-grade FDG-avid lymph nodes present within the left external iliac region and left inguinal region (Fig. ). Due to marked reduced sensitivity on the associated non-contrast CT component of the PET-CT, the female transgender anatomy within the pelvis was not clearly identified. The case was reviewed at the multidisciplinary tumor board rounds. On discussion, the transgender status of the patient was noted, and the FDG avidity within the pelvis was localized to the neovagina. Retrospective review of the CT imaging identified subtle changes within the soft tissues of the neovagina region over time (Fig. ). An MRI was also performed in the interim and identified a tumor within the neovagina (Fig. ).\nGiven the above information, additional immunohistochemical stains were performed and showed that the tumor cells were strongly positive for p16 (Fig. ), suggesting a HPV-related SCC. In situ hybridization study further confirmed that the tumor cells are strongly positive for high-risk HPV RNA (Fig. ). The above study results were highly suggestive of a metastatic HPV-related SCC from the mucosa/skin of the oral cavity or anogenital region. A transvaginal biopsy was attempted but was not successful due to heavy bleeding. Nonetheless, a diagnosis of metastatic HPV-related SCC of the neovagina is established.\nDue to the terminal stage of the disease, the patient was provided palliative radiotherapy and unfortunately passed away 4 months after the diagnosis. |
An 82-year-old man with a history of hypertension was referred to our Department of Hepatobiliary Pancreatic Surgery with a persisting subxiphoid pain of 12 h duration. There was no positive sign, except for tenderness in the epigastric region. The results of the laboratory tests, including determination of blood glucose levels, were almost within the normal range. Computed tomography of the upper abdomen revealed an atrophic pancreas with a dilated pancreatic duct containing a single stone located at the pancreatic neck; no mass in the pancreatic gland head or biliary dilatation was detected (Figure ). The size of the stone was approximately 1.0 cm × 0.6 cm. Based on the clinical signs and test result, a diagnosis of pancreatic duct stone with CP was established.\nGiven the presence of only one stone in the pancreatic main duct, we elected to perform laparoscopic pancreatic duct incision and stone removal, followed by T-type tube drainage, under general anesthesia after obtaining informed consent from the patient.\nDuring the operation, the patient received prophylactic antibiotics intravenously, and intermittent pneumatic compression therapy and graduated compression stockings were applied to the lower limbs. After catheterization of patient, he was installed in a flat Lloyd-Davies position. The patient was tilted in a 30-degrees reverse Trendelenberg position during the operation by the use of two 5-mm ports, two 10-mm ports, and one 12 mm port (Figure ).\nBy an ultrasonically activated scalpel, the lesser sac was opened via the gastrocolic omentum. Then, mobilization of the gastric antrum was performed, followed by upward retraction of the stomach to expose the front surface of the pancreatic head to the gland body. The duct of the pancreas was located by ultrasound laparoscopy and opened (Figure ). An initial opening was made above the gland neck by the joint use of an electrocoagulation and an ultrasonically activated scalpel, which was then prolonged further downstream and upstream to perform fillet opening from the head to the body of the pancreas. Next, the pancreatic duct was explored, and one stone was removed. One Fr14 T-type tube was inserted into the pancreatic main duct, of which the incised section was sutured discontinuously with a 4-0 Prolene stitch. Further, the pancreatic proximal duct was unobstructed, and no stone was present, as verified by intraoperative X-ray radiography through a T-type tube with 30% iohexol solution (Figure ).\nAn uneventful operation was performed, and the laparoscopic procedures were concluded in 160 min with an approximate 50-mL intraoperative loss of blood. Neither intraoperative nor postoperative blood transfusion was necessary. Furthermore, the oral intake of fluids began on the first postoperative day (POD). The volume of pancreatic juice drained was about 150 mL per day. No complications occurred peri- or postoperatively. Eventually, the patient was discharged on day 11 postoperatively. The result of postoperative X-ray radiography through the T-type tube was identical to that obtained intraoperatively (Figure ). The T-type tube was withdrawn two months after the operation. At the 15-mo post-operative follow-up, neither the subxiphoid pain nor the pancreatic duct stone had recurred. |
We describe the case of a 38-year-old right-handed male nonsmoking computer technician who was known to have dilated cardiomyopathy. He noticed a soft lump around the right 4th metacarpophalangeal joint measuring 1.5 × 1.5 cm. The lump had been present for almost 1 year and had started to become painful, hard, and increasing in size in the last couple of months. The patient underwent magnetic resonance imaging (MRI), which showed evidence of tumor depicted on the dorsal subcutaneous aspect of the hand at the level of the 3rd and 4th metacarpal head digits measuring 2.4 × 2.1 cm in axial dimension and 3.7 cm in craniocaudal dimension, with volar extension to invade the muscles surrounding the 4th metacarpophalangeal joint, in close relation with the cortex of the 4th metacarpal bone and the proximal phalanx without definite invasion (Fig. ). It was reported that no signs of metastatic disease were found. A biopsy was done, which showed clear cell sarcoma, melanoma of the soft part, grade 2. The patient underwent incomplete excision outside our center, and the tumor was removed using a double incision (volar and dorsal approach).\nUpon his initial visit to our facility, the anterior and posterior incisions were completely healed and a drop in the ring finger was noted due to loss of extensor tendon, which was involved by the tumor as well. The specimen was reviewed and showed multiple peripheral and volar margins involved by the tumor. MRI of the right hand showed evidence of an ill-defined infiltrative soft tissue enhancement between the 3rd and 4th metacarpal bones extending through the metacarpophalangeal joint distally, but no definable well-defined nodules or masses. The enhancement was reaching the surrounding major tendons. Whole body positron emission tomography-computed tomography and brain MRI showed no evidence of metastatic disease. The multidisciplinary clinical decision was to proceed with 3rd and 4th ray amputation. It should be noted that considerable time was spent explaining the operation to the patient, with all possible complications including skin necrosis due to low vascularity, infection at the operation site, major hand dysfunction, and local tumor recurrence.\nUnder a bloodless field without exsanguination, we performed a V-shaped incision incorporating both the dorsal and the volar incisions that had been conducted previously and which had resulted in contamination of the field. A dorsal approach was utilized first, where we went through the layers (skin, subcutaneous tissue, extensor tendon, and periosteum) till the apex of our V-shaped incision proximally (Fig. ). We sent tissue samples for frozen sections from both proximal and distal aspects as well as both radial and ulnar areas to check our margins. A considerable amount of time was spent identifying any abnormal tissue around the site of dissection till we reached the base of the 3rd and 4th metacarpal bones and did our osteotomy there. We turned our attention to the V shape of the volar aspect and continued our dissection in layers while carefully inspecting any abnormal tissue and avoiding any violation of the residual tumor area (Fig. ). Carefully, we identified both common digital nerves and arteries, and we cut only branches supplying the 3rd and 4th digits while preserving the other digits' important neurovascular structures; again, tissue samples were sent for freezing, including the volar ligament plates. After that, the residual area was completely removed by en bloc excision (Fig. ) and sent for permanent pathology, which turned out to be completely excised with negative margins all around. We elected to close the wound by primarily using the aid of the volar ligament plates, then inserting a drain, and we ensured adequate hemostasis (Fig. ). The wound's edges came nicely together and sterile dressing was applied.\nThe patient was discharged from our facility 3 days after the procedure. His first follow-up visit was after 8 days, on which he showed a well-healed incision site. He was scheduled for regular appointments with physiotherapy. Three months after the operation, follow-up MRI was done; there were only ill-defined edematous soft tissue changes but no definable enhancing soft tissue masses. The most recent MRI showed no definable enhancing soft tissue mass at the surgical tumor bed that would account for local tumor recurrence. The patient was followed up for 2 years with no sign of local recurrence or metastasis. |
An 81-year-old man presented due to presyncope to the emergency department. The past medical history included blood hypertension and Whipple's operation for carcinoma of the ampulla of Vater that had been staged as pT2N1M0 two months earlier. He had not received adjuvant chemotherapy but was on prophylactic low-molecular-weight heparin treatment.\nThe clinical examination revealed ecchymosis and edema of the right shin and the right and left forearms (Fig. ). A digital rectal examination was negative for bleeding. The laboratory workup demonstrated normocytic normochromic anemia, slightly elevated lactate dehydrogenase, a prolonged aPTT with a normal prothrombin time, and fibrinogen with absence of schistocytes on a blood film. An ultrasound was performed due to enlargement of the right shin, which excluded thrombosis and confirmed the presence of hematoma. Differential diagnoses included an adverse reaction due to heparin use, antiphospholipid syndrome, and bleeding disorder due to the presence of an acquired inhibitor in the clotting cascade.\nHeparin was discontinued and mixing studies were performed. Mixing with normal plasma did not correct the aPTT and the lupus anticoagulant test was negative. The presence of an acquired inhibitor of factor VIII was then suspected. Unfortunately, it was not possible to perform a Bethesda assay at the hematology laboratory of our hospital at that time due to lack of reagent. An investigation with immunological studies excluded other autoimmune diseases. A computed tomography scan of the abdomen was performed, exhibiting two peritoneal nodules compatible with metastasis. Therefore, his clinical condition was indirectly attributed to tumor recurrence.\nUpon establishing a diagnosis of acquired hemophilia, immunosuppressive treatment was initiated (Fig. ). The patient received prednisone IV (1 mg/kg per day), cyclophosphamide (100 mg p.o.), and immunoglobulin G IV (400 mg/kg for 5 days). Due to the association of this condition with carcinoma of the ampulla of Vater, the patient also received a cycle of chemotherapy with gemcitabine (1,000 mg/m2 IV) combined with oxaliplatin (100 mg/m2 IV) on day 14 of hospitalization. The patient continued treatment with prednisone (1 mg/kg per day) combined with cyclophosphamide (100 mg per day p.o.) until signs of response were evident.\nThe patient had a prolonged hospitalization of 6 weeks. During his care, he complained of dysphagia, and laryngoscopy revealed a retropharyngeal hematoma (Fig. ). This complication was managed with temporary discontinuation of alimentation and initiation of parenteral nutrition. Furthermore, care was complicated by nosocomial fever, which was effectively managed with piperacillin/tazobactam and vancomycin, while the patient later exhibited chemotherapy-induced neutropenia, managed with filgrastim. The aPTT decreased 2-fold (Fig. ), while the levels of factor VIII remained undetectable during the entire course of hospitalization. The patient showed signs of clinical stability and was successfully discharged for further outpatient care. |
A 70-year-old white female presented to the emergency room with symptoms of a cerebrovascular accident. She had a history of multiple myeloma status post-autologous bone marrow transplant (BMT) with a conditioning regimen of high-dose melphalan 2 weeks prior to presentation. She woke up the morning of presentation and was found to be confused for a few minutes, followed by a gradual improvement in mental status. About an hour later, she started to experience a severe headache associated with blurry vision, and shortly thereafter she became disoriented again. Paramedics identified agitation, right-side neglect, left gaze deviation and right side weakness. On arrival in the emergency department, the patient's headache had resolved, but the patient was still agitated and disoriented. The patient's altered mental status (AMS) required that the history be obtained from the patient's husband. There was no history of recent infection, fever, weight loss or trauma. The review of systems was negative for photophobia, seizures or any other neurological issues. Pertinent past medical history was that of recent BMT with melphalan and poorly controlled hypertension. She had had thrombocytopenia since the time of BMT and chemotherapy. Her admission blood pressure was 221/114 with a mean arterial pressure (MAP) of 145 mmHg. Her admission NIH stroke scale score was 7, with problems in orientation, not following commands, not answering questions appropriately, left gaze preference, reduced blink on stimulus from the right and possible right-sided neglect. Her visual acuity was reduced to finger movements and light perception in both eyes. She was moving her extremities equally, bilaterally. Reflexes were brisk throughout with equivocal plantar response. The rest of the neurological exam was limited, as the patient was not following commands consistently. Our differential diagnosis at that time included cerebrovascular accident (CVA), PRES (due to elevated BP, recent chemotherapy and bone marrow transplant), seizures and complicated migraine. Since there was no motor deficit associated with the neglect and eye deviation, we were obligated to consider a broad differential diagnosis, including PRES. After the initial laboratory workup, we obtained a CT head and a CT angiogram of the head and neck with perfusion studies. The CTA of the head and neck failed to identify any major vessel cutoff or any acute hypo/hyper density, but the CTP demonstrated increased cerebral blood volume (CBV), cerebral blood flow (CBF) and reduced time to peak (TTP) in the posterior cerebral vascular distribution (see Figures and ). These imaging features were consistent with PRES, and we initiated intravenous anti-hypertensive medications. An MRI brain was obtained, which showed abnormal restriction in the parietal and occipital areas, confirming the diagnosis of PRES (see Figure ). Reduction of the patient's systolic BP from 220 to 180 was associated with slight improvement in her visual acuity and orientation within a couple of hours. Notable laboratory data revealed a platelet count of 11,000/μl and hemoglobin of 11 g/dl. All other laboratory tests were within the normal limits. There was no evidence of thrombotic thrombocytopenic purpura (one of the causes of PRES), and she was admitted with the diagnosis of PRES. At the time of admission, PRES was considered to be secondary to malignant hypertension complicated by recent chemotherapy and BMT. Over the subsequent 48 h, she returned to baseline with improvement of her blood pressure to normal range. |
A 29-year-old male patient weighing 57 kg who was diagnosed to have hemophilia type A since 13 years of age and was on intermittent blood and factor VIII (FVIII) transfusions presented with complaints of chest pain, breathlessness, and palpitations since 4 months which suddenly increased to Grade IV dyspnea since 15 days before admission.\nOn examination, he had elevated jugular venous distension, wide pulse pressure, epigastric pulsations, and a continuous murmur best heard over left sternal border. His electrocardiography (ECG) showed left ventricular hypertrophy with tall P-wave in lead II and a bifid P wave in lead V. Cardiac computed tomography angiography was performed wherein the aortic annulus was 25 mm, sinus - 37 mm, ascending aorta - 24 mm, and the noncoronary sinus showed a focal dilation (15 mm × 12 mm) protruding into the right atrium suggestive of RSOV of noncoronary sinus.\nLaboratory investigations revealed hemoglobin of 14 g/dl and platelet count 2 lakhs/cumm. His coagulation profile including prothrombin time and activated partial thromboplastin time was within normal limits except for FVIII level, for which FVIII assay was done, which was 1% of normal. His disseminated intravascular coagulation profile (platelet counts, fibrin degradation products, etc.) was repeated after injecting 3000 units of FVIII and his FVIII level increased to 100% of normal.\nA hematology consultation was sought, and the patient was planned for device closure of the RSOV under general anesthesia in catheterization laboratory by cardiologist, especially in view of Hemophilia type A to avoid risks associated with surgical repair of the RSOV. On of the cardiac surgery operation theater was kept on standby until the completion of the interventional procedure.\nPreoperative anesthetic evaluation was done. Oral premedication in the form of tablet diazepam 5 mg HS and tablet alprazolam 0.5 mg an hour before the procedure were given. Premedication in the form of intramuscular injections were avoided for fear of an intra-muscular hematoma. Three thousand units of FVIII (Eloctate®, antihemophilic factor, Fc Fusion protein, Biogen, USA) were given intravenous (IV) 1 day before the procedure and another 3000 units were given 30 min before the procedure on the day of intervention as advised by the hematologist.\nFour units of packed red blood cell, 4 units of platelet concentrate and 4 units of cryoprecipitate were arranged. Three vials of 3000 unit each were also arranged and the plan was to repeat 3000 units in the evening in case surgery was needed.\nThe patient was taken up for the procedure early morning as the first case. Monitoring included pulse oximetry, ECG, and noninvasive blood pressure. Intravenous cannulation was performed using 16 G cannula after local infiltration with insulin syringe. Injection midazolam 1 mg and injection fentanyl 100 μg were given intravenously. Anesthesia was induced with injection etomidate 20 mg and atracurium 40 mg IV was used as muscle relaxant. Trachea was intubated with 8.0 mm ID cuffed endotracheal tube and fixed at 22 cm. Anesthesia was maintained with oxygen, air, and isoflurane. End-tidal CO2 monitoring was attached. Transesophageal echocardiography (TEE) probe was inserted gently avoiding any trauma []. Injection paracetamol 1 g IV was administered 30 min before the completion of procedure for adequate analgesia.\nCatheters for loading the device were inserted through the femoral artery and the device was successfully deployed under fluoroscopic guidance []. The procedure lasted for about 2 h and was uneventful. An occluder device (Amplatzer, 2.5 mm) was successfully placed to occlude the ruptured aneurysm [ and ]. The patient was extubated in the cath lab table and was shifted to the ward in a stable condition. No intraprocedure blood or blood product transfusions were required. Postprocedure FVIII levels were maintained for at least 7 days. |
A 55-year-old Caucasian male, with a past medical history significant for tobacco abuse (41 pack-years), presented with shortness of breath accompanied by chest and back pain for two months. Blood workup showed a WBC count of 68,400 cells/µL, with an AEC of 27,360 cells/µL. A computed tomography (CT) pulmonary angiogram was performed, as he was hypoxic, and revealed a 3.6-cm speculated mass within the anterior right upper lobe, partially invading the anterior chest wall. It also revealed mediastinal and hilar adenopathy, an extensive osseous lesion (including compression fracture at T7), and a small pericardial effusion (Figure ). A CT of the abdomen and pelvis with contrast was performed and revealed a diffuse metastatic disease involving the liver, adrenal glands, spleen, and the bones. Magnetic resonance imaging (MRI) of the thoracic spine did not reveal spinal cord compression, but it did show the compression fracture at T7 and multilevel thoracic spondylosis. An MRI of the brain revealed a 5-mm lesion in the left occipital lobe, without edema or mass effect.\nThe hematology-oncology team was consulted for an evaluation of the metastatic disease and the eosinophilia. A core needle biopsy was obtained from a liver lesion and the result came back as poorly differentiated adenocarcinoma of the lung (cytokeratin 7, TTF1, and napsin-A were positive, while cytokeratin 2 and CDX2 were negative). Given his functional status, the decision was made to hold on systemic therapy and start on palliative radiation to the spine for pain control. The plan was to complete radiation sessions and then evaluate his functional status before starting systemic therapy.\nHe continued to have a high WBC count during the admission (Figure ). Therefore, a bone marrow biopsy was performed to rule out a hematologic malignancy and it revealed metastatic adenocarcinoma of the lung with no evidence of a myeloproliferative disorder. The flow cytometry from the bone marrow showed a CD5-positive clonal B-cell population, which was similar to the blood flow cytometry. Blood tests, including tests for Janus kinase 2 (JAK-2), calreticulin (CALR), MPL, BCR-ABL, and platelet-derived growth factor receptor (PDGFRA), were negative. The blood smear showed microcytic anemia with leukocytosis with absolute neutrophilia and eosinophilia. The serum immunoglobin E (IgE) was high at 377 IU/ml, and the tryptase level was low at 1.8 µg/L. Given these findings, his eosinophilia was related to a paraneoplastic process rather than a primary bone marrow disease.\nDuring the following days, the patient completed 13 sessions of radiation without improvement in his functional status, pain, or breathing. The case was discussed with the patient and his family; he decided that he would go with comfort measures, so he was discharged to the hospice facility. |
A 5-year-old Caucasian female presented to the outpatient clinic with her parents in 2001 complaining of being unable to use a pen with her right hand.\nAt first examination, the thumb was seen at adduction and flexion position, it cannot be extended, and after passive extension, it returns to original adduction and flexion position ().\nThere was a contracture and absence at the first web and the thumb was smaller than the opposite left one.\nThere was no extension in the interphalangeal joint due to the absence of EPL, also in the metacarpophalangeal joint due to the absence of EPB, whereas the CMC joint was stable.\nThe following movements: flexion, adduction and opposition were within normal.\nNeither stiffness nor instability in interphalangeal (IP) or metacarpophalangeal (MCP) joints was observed ().\nThe deformity was isolated as no bones were missing in her body and she was not suffering from any syndromes. Also, no similar cases were reported in her family history.\nWe ensured the diagnosis of congenital clasped thumb deformity with hypoplastic thumb. We did not face any difficulty with the differential diagnosis between this situation and neonatal trigger thumb because of the late presence of the patient at the age of 5 years. The late presence and the absence of EPL and EPB made the conservative treatment with splinting no more successful and the reconstructive surgery the only proper way to achieve good results.\nThe surgery was performed in one stage, it took 90 min under general anesthesia, a tourniquet was applied on the upper right arm and the first web was obtained by performing an expansion surgery with simple z-plasty. Then, a drill-hole was made in the base of the proximal phalanx of the thumb, where the extensor indicis proprius (EIP) tendon was passed through and sutured to itself, while the end of the flexor digitorum superficialis (FDS) of the ring finger was sewed to the end of the distal phalanx and fixed with a button, and the thumb was placed at full abduction and extension position and fixed with a K-wire (Kirschner wire) inserted from distal to proximal ().\nA supporting splint of the forearm and thumb was applied and then replaced with below elbow cast after removing the stitches two weeks after the surgery ().\nSix weeks later, the cast and the K-wire were removed. Six months later, she was able to hold a pen and write with good flexion and extension of the thumb (). The patient was examined in 2018 (). |
An 11-month-old boy was referred to a tertiary center for failure to thrive, poor muscle tone, short neck, kyphosis, and unusual spacing between teeth. He was diagnosed with infantile HPP after repeated low ALP activity test results and radiographic assessment of severe rickets-like skeletal changes and tongue-like lucencies projecting into the metaphyses. He was hospitalized multiple times for pneumonia likely related to musculoskeletal manifestation of HPP, which required treatment with intravenous antibiotics. At age 9 years, he developed persistent headaches; a magnetic resonance imaging (MRI) cranial scan confirmed craniosynostosis with Chiari malformation and cerebellar tonsillar herniation. He underwent craniovertebral decompression, with removal of the posterior arch of C1; a ventriculoperitoneal shunt was inserted to relieve intracranial pressure. He subsequently underwent 2 shunt revisions.\nThe patient experienced multiple fractures, starting at age 17 years, when he sustained bilateral femoral fractures when jumping off a wall; this required bilateral intramedullary rod insertion. At age 18 years, he sustained a right tibial fracture while jumping. Bone healing was delayed, but the fracture eventually healed satisfactorily. At age 20 years, he sustained bilateral femoral fractures when he rolled off his bed during a seizure and required rehabilitation for approximately 8 months.\nOver the first 18 years of life, the patient was hospitalized 8 times for a total of 43 days (Table ). Of these hospitalizations, 5 separate admissions required a stay of ≥5 days; the stay for insertion of a ventriculoperitoneal shunt was 14 days.\nOutpatient specialist visits represented a significant proportion of the healthcare resource utilization by this patient (Fig. ). Most of the outpatient specialist visits required consultation with providers in 12 specialties, including pediatric dentist (38 visits), pediatric endocrinologist (32 visits), neurosurgeon (27 visits), and general pediatrician (19 visits; Table ). Outpatient management consisted of diagnostic imaging procedures (Table ). The most frequent procedures were radiography of the limbs and spine, performed on 18 and 12 occasions, respectively; the patient also underwent 14 MRI cranial scans. Dental surgery and tooth extraction were performed as day case procedures for management of dental carries and malocclusion on 3 occasions. |
A 65-year-old man with severe chronic obstructive pulmonary disease (COPD) was admitted with a four-day history of chest pain and worsening shortness of breath. He explained the chest pain started suddenly when he tried to reach out for something on his computer table. It was located on the right anterior chest, sharp in nature, 7/10 in intensity, pleuritic, and worse with coughing and deep breathing. He had a past medical history of severe COPD with frequent exacerbations recently necessitating multiple antibiotics and steroid courses, coronary artery disease, gastroesophageal reflux disease (GERD), and hypertension. He had a 30-pack per year smoking history and quit about 10 years ago. He was a retired fireman living with his family.\nVitals signs in the emergency department (ED) were stable; he was breathing on ambient air. The physical examination demonstrated decreased breath sounds bilaterally without any wheezing or Ronchi. Moderate tenderness was present in the mid-axillary line in the fifth intercostal space, but no other abnormalities were noticed. Laboratory investigations were negative for any leukocytosis, troponin, or any other abnormalities. The electrocardiogram (EKG) showed a normal sinus rhythm. Computed tomography (CT) angiography was done to rule out pulmonary embolism (PE). The CT was negative for PE but showed mild peribronchial infiltrate in the right middle lobe and posterolateral lung herniation between the seventh and eighth ribs, with minimal subcutaneous emphysema along the right chest wall (Figure ).\nThe patient was admitted to the hospital and managed conservatively on broad-spectrum antibiotics, including vancomycin, levofloxacin, and 40 mg per day of prednisone. Two days later, his face swelled up suddenly with a change in the quality of his voice while he was eating dinner. An examination showed a swelling in the neck, diffuse crepitations on his body involving the face, all the way down to the buttocks. Repeat CT chest and neck showed extensive subcutaneous emphysema in the face, neck, chest, and mediastinum with a right-sided pneumothorax at the level of the previous lung herniation (Figures -).\nThe prevertebral and retropharyngeal air was demonstrated as compressing the oropharynx (Figure ). His oxygen requirement went up to 6 liters nasal cannula. A blowhole incision was made on the anterior chest wall, and he was observed in the medical intensive care unit (MICU). Surgery to close the defect was deferred due to his other comorbidities and the higher risk of post-operative complications. He improved gradually over the course of the next few days, completed the course of antibiotics with steroids, and was discharged to a rehabilitation center and did well post-discharge. |
Case
1: A 6-year-old boy who was previously well presented to us with a history of hoarseness of the voice over the past 1 month and breathing difficulties for the previous 2 weeks. There was no history of trauma, or any previous surgery of the head and neck region except for a tongue-tie release during the infantile period. There was no history of fever or loss of weight or appetite.
On examination, the patient’s vital signs were stable. Systemic examination did not reveal any abnormality. Flexible laryngoscopy showed a polypoidal mass approximately 1 × 1 cm in size in the free border and the undersurface of the right vocal cord extending down to the subglottis ().
The patient was evaluated with computed tomography (CT) of the neck, which revealed a polypoid nodule of size 1 × 1 cm in the right vocal cord projecting into the lumen with no evidence of extension into the anterior commissure or deep cartilage. An initial impression of papilloma was made. The patient underwent microlaryngeal gross total excision of the mass under general anesthesia. Histopathology of the excised mass showed polypoid fragments of the respiratory epithelium and metaplastic squamous epithelium. The subepithelium showed spindle-cell proliferation with storiform appearance and tiny microhemorrhages. There were dense infiltrates of lymphocytes, plasma cells, and eosinophils. The spindle cells did not show nuclear atypia or mitosis and possessed vesicular nuclei with conspicuous nucleoli ().
Immunohistochemistry was positive for vimentin and smooth muscle actin (SMA) (), negative for cytokeratin, desmin, anaplastic lymphoma kinase (ALK), S100 and cluster of differentiation 34 (CD34). These features were thus suggestive of a spindle-cell neoplasm favoring IMT.
The patient was followed up without any adjuvant therapy. After 4 months, he presented with repeat symptoms of hoarseness of voice. Laryngoscopy revealed a recurrent mass, 2 × 2 cm in size. He underwent a microlaryngeal excision using micro |
The patient was a 42-year-old Chinese woman who presented with a 10-year history of slowly growing masses in the maxillofacial region. In 1997, the patient found a mass in the back region of her right ear, and the diagnosis based on a biopsy of the mass was merely a benign tumor. Shortly after the excision, another mass emerged and gradually enlarged below the right earlobe region. The patient refused tumor excision due to fear of the surgery.\nSince the patient was referred to the Department of Head and Neck Oncology, West China Hospital of Stomatology, Sichuan University, in 2007, she had been feeling slight pain in the mass of the right parotid gland. Physical examination revealed an approximately 6 × 5-cm mass in the region of the right parotid gland. It was a multinodular lesion without mobility and tenderness, and positive to the position changing enlargement test. There was no evidence of sensory deficit or facial palsy. On panoramic radiography, there was a small-sized deformity in the right ramus. The computed tomography (CT) scan demonstrated multicentric soft tissue masses in the region of the right parotid gland, cheek, infratemporal fossa and parapharyngeal spaces; these masses enhanced peripherally with central heterogeneous irregular nonenhancement after administration of a contrast medium (fig. ).\nTo reduce blood loss during surgery, digital subtraction angiography (DSA) and superselective embolization were performed prior to the operation. DSA disclosed soft tissue in the right parotid gland region, with an abundant blood supply from branches of the right external carotid artery. With a guide wire, a 5F sheath was used to superselectively catheterize the subbranches of the right external carotid artery and embolize them with 550–750 um PVA particles. Immediate postinjection angiography showed an occlusion of the subbranches to the focus (fig. ).\nUnder general anesthesia, the mass in the right parotid gland region was completely excised without much blood loss. It was light yellow and about 6 × 5 × 5 cm. In attempting to remove other masses in the parapharyngeal space, the authors removed the right ramus and tried to pass through the lateral and medial pterygoid muscles. Unfortunately, there was a lot of blood loss from the pterygoid venous plexus and, at that time, the total blood loss had reached about 1,600 ml. The authors had to give up the removal of the other masses in the parapharyngeal space. The patient had an uneventful recovery, except for right facial palsy, and was discharged 7 days following the operation.\nThe lesion was identified as MPC. The pathological findings showed numerous gaping and branching, and thin-walled vessels concentrically surrounded by spindle ovoid myoid tumor cells with eosinophilic cytoplasm (fig. ). Hyalinization was present, but there was no necrosis, nuclear anaplasia, increased mitoses or infiltrative growth. There was positive immunoreactivity for SMA, MSA and desmin, but CD34 and CD99 staining were negative.\nThe patient had no more pain and was reviewed at 3- to 6-month intervals. There was no sign of recurrence in the right parotid gland region, and there was no obvious enlargement in the residual lesions 5 years postoperatively. She has had regular follow-up. |
We report the case of a 52-year-old man with synovial sarcoma of the right kidney. Laparoscopic nephroureterectomy was performed in January 2012, under the suspicion of renal cell carcinoma (fig. ). The postoperative course was complicated by pulmonary embolism. The patient was put on anticoagulant therapy with low-molecular-weight heparin.\nSurprisingly, the histologic findings were compatible with synovial sarcoma, verified by the detection of the typical t(X, 18) translocation and the SYT/SSX fusion gene. The initial assessment confirmed stage III disease: pT2b (maximum tumor size 8.2 cm), Nx M0 V1 (formation of a tumor thrombus in the right renal vein) G3.\nAccording to a meta-analysis published in 1997, adjuvant chemotherapy can prolong progression-free survival, and there is a trend towards prolonged overall survival []. Further prospective studies have evaluated this question, and in high-grade soft tissue sarcomas adjuvant chemotherapy can be considered []. Adjuvant chemotherapy was discussed with our patient and, bearing in mind the high risk of recurrence in this case (tumor size, histologic grading and angioinvasion), 4 cycles of adjuvant chemotherapy with doxorubicin and ifosfamide were applied []. At the end of the treatment visit, a CT scan was performed and no tumor could be detected, but there was still evidence of a pulmonary embolus in the left inferior lobe. Subsequently, the patient was kept on anticoagulant therapy with low-molecular-weight heparin.\nAt the first follow-up visit 4 months after the completion of adjuvant chemotherapy, there was still no evidence of local tumor recurrence or metastases; however, in the area of the left inferior lobe pulmonary artery, a persisting soft tissue formation of unknown etiology could be documented. This entity was deemed most likely to be scar tissue after thromboembolism with pulmonary infarction or residual thrombus.\nIn the further follow-up period 3 months later, a CT scan showed progression of this soft tissue formation with partial occlusion of the left inferior lobe pulmonary artery (fig. ); no uptake could be shown on PET/CT (fig. ). Subsequently, a bronchoscopy with endobronchial ultrasound-guided transbronchial needle aspiration was performed for tissue sampling of the endoluminal thrombus. Histology revealed a spindle cell proliferation, which was compatible with metastasis of the known synovial sarcoma. At this time, no other sites of active disease could be detected on CT. Following data favoring metastasectomy in isolated pulmonary metastasis in selected patients [], a lobectomy of the left lower lobe with subsequent resection of the lingula and bronchoplasty were performed (fig. ). Histology confirmed a synovial sarcoma metastasis (fig. ).\nWritten informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent form is available for review from the editor of this journal. |
A 73-year-old man was admitted in emergency to the general surgery department with a neck mass, sudden dyspnoea, stridor, dysphonia, and progressively worsening dysphagia. His medical history included a multinodular goiter ans right radical nephrectomy performed 8 years prior due to renal cell carcinoma. At annual follow-up, a CT of the thorax and abdomen was performed and the thyroid mass was also evaluated by ultrasonography and thyroid function tests. Five months earlier, the patient had undergone fine-needle aspiration consistent with multinodular goiter. Three days before admission the patient underwent a total-body CT scan that revealed a thyroid mass with substernal extension involving and obstructing the upper airways, right vocal cord and jugular vein and showed carotid artery compression and displacement, in addition to diffuse lymphadenopathy (Figure ).\nPhysical examination revealed a large, painful, diffuse, and predominantly right-sided thyroid tumour. Thyroid function tests were normal. A flexible laryngoscopy revealed right vocal cord palsy and left vocal cord paresis, with a nearly total reduction of the laryngeal lumen. Emergency endotracheal intubation was performed, followed by total thyroidectomy using loupe magnification [] with lymph node dissection. The surgery was completed by a tracheotomy, given the evident tracheomalacia. The thyroid gland was found to have been fully replaced by a soft yellow mass weighing 40 g and 8.5 × 5.5 × 4.5 cm large, with indistinct borders infiltrating peri-thyroid muscles and involving three lymph nodes. Histological examination revealed a carcinoma composed mainly of clear cells with scanty oxyphil cells. Neoplastic cells showed large pleomorphic nuclei and frequent mitoses. Lymphatic and vascular invasions were common findings. Immunohistochemistry revealed strong and diffuse expression of CD10 antigen (Figure ) was positive for Vimentin and negative for thyroid transcription factor-1 staining. Histology and immunohistochemistry were characteristic of metastatic clear renal cell carcinoma.\nThe patient had an uneventful postoperative course and was discharged after 10 days. Despite palliative chemotherapy, the disease progressed and the patient died 7 months later. |
An 87-year-old woman was referred to our hospital in May 2010 with severe left ocular pain since 4 days before. On initial examination, the left orbit was severely necrotized with several live larvae attached to the necrotic tissue. The upper and lower eyelids and the eyeball were completely destroyed. Periorbital tissues were swollen and erythematous ().\nAccording to her grandchild, the patient had not been able to walk or sit for several years, presumably due to back injury. She also had bladder and bowel incontinence. However, her mental status was intact. She had history of eyelid surgery due to a skin lesion 15 years ago which had been confirmed to be skin cancer. There were no medical records or documents however, and she had experienced no recurrences ever since.\nOrbital CT scan revealed disorganized globe and periocular tissues, with hemorrhagic patches in the left orbit filled with larvae. The orbital walls seemed intact and no larvae could be detected in the periorbital sinuses or cranium.\nOn the day of admission, the patient underwent exenteration of the left orbit (). All necrotic tissues and over 150 larvae were removed. A few larvae were collected and preserved in 70% ethanol. The larvae were identified as Chrysomya bezziana (Diptera: Calliphoridae) or old world screwworm fly. The most distinctive characteristics of this species include one pair of anterior spiracles with 4 to 6 digit projections at the ends, located on the first segment of the third instar of the larvae above the cephalopharyngeal skeleton (one pair of hooks at the base) (), and one pair of posterior spiracles at the bottom of the third instar of the larvae. Each is surrounded by a complete peritreme, without a distinct button. There are 3 slits inside each peritreme, which continue to its end (). is a picture of the complete third instar larva of Chrysomya bezziana, removed from the patient’s orbit.\nPathological findings of the exenterated tissue included unidentified and severely necrotic and hemorrhagic tissue.\nDuring the postoperative period, the patient remained under intensive supervision for wound infections and possibly missed larvae. Vaccination for Clostridium tetani was performed. As the wound began to heal, the patient’s general condition improved and she was discharged after two weeks. |
A 47-year-old man underwent percutaneous coronary intervention (PCI) secondary to unstable angina. The right common femoral arterial puncture site was closed with an Angio-Seal. There was no definite immediate complication, and the patient was discharged from the hospital.\nTwo days after the PCI, he presented pain and numbness of the right calf. Symptoms were progressively aggravated in the following week. He revisited the hospital eight days after the procedure. Upon physical examination, there was no evidence of hematoma or pseudoaneurysm at the puncture site. Ultrasonography showed presumed aggregated collagen with bright echogenic anchor of the Angio-Seal device in the right popliteal artery ().\nA left femoral artery puncture was made, and right lower extremity angiography was performed with a 5 Fr catheter. Total occlusion of the distal popliteal artery was observed in angiography (). Correlating with ultrasonography, a right popliteal artery occlusion due to the Angio-Seal that had broken away from its original location was diagnosed. The arteries distal to the occlusion were reconstituted by collateral vessels, showing reduced but persistent lumens.\nUnder local anesthesia, the right common femoral artery was exposed by a cardiothoracic surgeon. An 8 Fr long vascular sheath was inserted antegrade into the right superficial femoral artery. A 4 Fr Fogarty balloon catheter (Edwards Lifesciences, Irvine, CA, USA) was introduced into the vascular sheath, then inserted into the arterial lumen until the balloon was located distal to the embolus over an 0.018 wire (Terumo, Tokyo, Japan). The balloon of the Fogarty catheter was inflated and pulled back toward the femoral artery cutdown site (). While performing the procedure, we could recognize the location of the embolus in angiography, which was seen as a filling defect between the balloon. Contrast was made by injecting a small amount of contrast material through the sheath (). The embolus was successfully removed through the incision site of the femoral artery. It consisted of the Angio-Seal sponge with anchor, and had accumulation of acute thrombus proximally ().\nAfter a 12-month follow-up, the patient was pain free, and his post-interventional course was uneventful. |
A 68-year-old male presented with a slow-growing painless mass in his left preauricular area, detected 4 months ago. On physical examination, a non-tender lump measuring 3 cm in size was palpated in the left parotid region and partly fixed to underlying structures. Clinically, there was no evidence of facial nerve involvement nor regional lymphadenopathy. Laboratory tests showed the complete blood count and inflammation indexes within normal limits. CT revealed approximately 3 cm sized well-marginated heterogeneously attenuated soft tissue mass in the left parotid gland, involving both superficial and deep lobes, making the dumbbell shape. Contrast-enhanced images showed dense enhancement. However, on the delayed image, no definite contrast-washout nor gradual enhancement of the mass was noted (). Sonogram showed a heterogeneous hypoechoic mass in the left parotid gland (). And ultrasound-guided fine-needle aspiration cytology (FNAC) revealed organizing abscess, which is not corresponding with the characteristic of radiologically dense enhancement of the mass, suggesting unsatisfactory cytologic result. Despite the unsatisfactory cytologic result, the patient was advised to undergo left superficial parotidectomy for the possibility of malignancy, but he refused. After then, the size of the mass continued to grow. After 8 years, the patient presented with rapid swelling and tenderness of the left preauricular and submandibular areas. CT revealed the left parotid mass had grown up to approximately 7 cm, revealing internal non-enhancing low density portion and peripheral enhancing rim with perilesional fat infiltrations. The attenuation of its low density portion was measured as 47 HU. There was no definite enhancing nodular portion (). Sonogram showed large solid and cystic mass in both superficial and deep lobes of the left parotid gland (). The second ultrasound-guided FNAC was performed and the possibility of cystic metastasis, such as very well differentiated squamous cell carcinoma was suggested. Under general anesthesia, the patient underwent enucleation of the parotid tumor. Grossly, his left parotid gland was mostly replaced with cystic mass, measuring about 6 cm (). During the procedure, the cystic mass was ruptured and discharge of necrotic materials was noted. Histologic microscopy of the left parotid tumor revealed Warthin’s tumor with infiltration of stroma by malignant squamous cells (). The patient was finally diagnosed as squamous cell carcinoma arising from Warthin’s tumor of the left parotid gland. Positron emission tomography-CT (PET-CT) after surgery showed the absence of abnormal uptake, suggesting the left parotid gland was the only primary site. |
A 33-year-old male on buprenorphine-naloxone 4-1 mg for heroin dependence presented to an outside facility with an acute onset of right sided weakness and dysarthria approximately 4 hours after initial development of his symptoms. Social history was significant for daily cannabis use and 1 pack per day smoking history of more than 12 years. His last use of heroin was more than 3 years ago. He reported a 3-day history of fever, chills, night sweats, and malaise. Investigations showed leukocytosis (15.5) and a positive urine toxicology screen for tetrahydrocannabinol (THC). CT head showed no acute changes. However, CTA of the head and neck demonstrated a left middle cerebral artery (MCA) narrowing (distal left M1 and proximal M2) (). NIHSS at the time of presentation was 5. He was given normal saline followed by Lipitor 80 mg and aspirin 325 mg. He had worsening neurological deficits at the time of his transfer to a comprehensive stroke center (NIHSS 10). Physical exam showed a hemiplegic right upper and lower extremities with dysarthria. An MRI showed patchy diffusion restrictions in the left MCA territory suggestive of acute ischemic stroke as shown in . MRA showed partial resolution of left MCA vasospasm (). A perfusion scan was also performed and showed symmetrical mean transit and time to peak. Patient showed some improvement and he was admitted to neurocritical unit. The next morning, approximately 12 hours after his first CTA that showed M1 vasospasm, he underwent a diagnostic cerebral angiogram which was completely normal with patent and normal appearing left MCA. Lumbar puncture was performed to work up infectious and autoimmune causes of the stroke, which came back as negative. Transthoracic ECHO (TTE) and Transesophageal ECHO (TEE) were done to evaluate endocarditis, which were also unremarkable. Diagnosis of acute left MCA stroke secondary to vasospasm due to illicit drug use was established. His symptoms resolved over the course of next 48 hours and was discharged with a NIHSS of 0. For secondary prevention, he was discharged on aspirin. Lower dose of buprenorphine-naloxone, weaning off on cannabis use, and smoking cessation were recommended. A 3-week cardiac telemetry monitoring was ordered which came back negative. |
A 37-year-old woman presented at 28 + 5 weeks' gestation with persistent vomiting and reduced oral intake. She had a background of primary RFS, diagnosed at the age of 3 years old when she presented with rickets. She also had a long history of progressive renal insufficiency, persistent proteinuria, nephrocalcinosis, and recurrent nephrolithiasis. Additionally, she had a chronic right-sided hydronephrosis from a previous episode of nephrolithiasis and was under regular nephrology follow-up receiving oral solute replacement (see ).\nShe had two previous pregnancies, both monitored regularly due to her chronic kidney disease (CKD) and both delivered preterm due to placental abruption. Whilst her first pregnancy was uncomplicated until the sudden delivery at 37 weeks, the second pregnancy had been complicated by gestational diabetes, recurrent hypoglycaemia, and metabolic acidosis, which required occasional intravenous bicarbonate replacement. Following delivery at 30 weeks, the baby developed neonatal hypoglycaemia which prompted genetic testing, resulting in discovery of the HNF4A p.R63W genetic mutation in both mother and baby. Sadly, the child died at 6 months of age, possibly due to metabolic complications of the disease.\nDuring her third pregnancy, she was being seen regularly in both the consultant-led antenatal clinic and the nephrology clinic. At her booking appointment, she was normotensive with a body mass index of 27 kg/m2. Serum bicarbonate and creatinine were stable within her usual range at 21 mmol/l and 190 µmol/l, respectively, and urine protein/creatinine ratio was elevated but also stable for her at 223 mg/mmol. During early pregnancy, her bicarbonate levels had dropped as nausea prevented her from maintaining adequate oral replacement. Because of this, the decision had been made at 24 weeks to commence fortnightly bicarbonate infusions via the day case unit to manage her persistent metabolic acidosis. As in her second pregnancy, she had developed gestational diabetes () and therefore, was also under regular review in the joint diabetes antenatal clinic.\nDespite the infusions, her nausea progressed to persistent vomiting and she was unable to tolerate any oral solute therapy. On admission at 28 + 5 weeks, she was dehydrated with cool peripheries and dry mucous membranes. She was hypotensive at 89/50 mmHg, acidotic with low bicarbonate, and had borderline phosphate, potassium, and glucose levels (see ). Her urea and creatinine levels were at her baseline. There was concern that she may be developing HELLP syndrome as her alanine transferase (ALT) levels were elevated; however, her platelet count was greater than 100 × 109/l and haemolysis screen was negative. |
A 62-year-old woman came to the Department of Oral and Maxillofacial Surgery, University of Tsukuba Hospital, with a clinical diagnosis of a sublingual gland tumor. She had noticed swelling in the sublingual gland, with no pain, for 9 months. She had a history of hypertension, cerebral infarction, and hyperlipidemia. Her face was symmetrical and there was no trismus. The regional lymph nodes were normal, and the submandibular gland was hard and swollen. Examination of the oral cavity showed a hard, elastic mass measuring 28 × 13 mm on the left side of the floor of her mouth. The mucosal surface was normal, with no ulceration (). There was no observable saliva flow from the opening of Wharton's duct.\nMagnetic resonance (MR) images [short TI inversion recovery (STIR)] showed a well-defined tumor of the sublingual gland with a high-signal mass measuring 28 × 25 × 12 mm (). Fluorodeoxyglucose positron emission topography (FDG-PET) depicted a mass measuring 28 × 13 mm with a max standard uptake value (SUV) of 10.4 () and did not show any neck metastasis or distant metastasis.\nThe carcinoid was removed by primary excision performed extraorally under general anesthesia, with 10 mm surgical margins and submandibular dissection with the level I lymph node and submandibular gland. The intraoral resected defect was partially sutured and covered with polyglycolic acid (PGA) sheets and fibrin glue. The postoperative course was uneventful. One year later, there was no tumor recurrence or neck metastasis.\nHistological examination revealed that the resected tumor, which was 26 × 24 × 12 mm, was whitish in color and solid in consistency. The round cells were solid or arranged in cords, trabeculae, or nests, with hyalinized stroma with high vascularization. The tumor cells had eosinophilic cytoplasm. The nuclei were round and varied in size, and the rough chromatin and mitotic count was less than 1/10 HPF. There was no necrosis (). The tumor had partly infiltrated the sublingual gland and had no clear boundary. Immunohistochemical staining showed that the tumor was CD56 (+), chromogranin A (+), and synaptophysin (−) (). The Ki-67 index was 2.8%. A pathological diagnosis of typical carcinoid of the sublingual gland was made based on the morphological and immunological exam. The surgical margin was free, and no lymph node metastasis was found. |
A 25-year-old woman of African descent with NF1 presented to our clinic complaining of chronic pain, depression, and anxiety. She was diagnosed with NF1 at puberty but had reported symptoms since childhood. She was unaware of a family history of similar conditions. She has Lisch nodules on her iris bilaterally and 10 café-au-lait patches across her body. The patient also has numerous subcutaneous neurofibromas on her face (plainly visible and causing some facial disfigurement) and in several regions of her body. These caused her chronic, intermittent, sharp pain of varying intensity but averaging 6/10 on the 10-point pain scale. She has tried many OTC pain medications with limited benefits. She has had over 20 surgical resections and multiple laser treatments for the neurofibromas with limited success as they often recurred after treatment. She reports pain from both the treatment and the tumors themselves. The neurofibroma tumors have also caused her cosmetic issues. She reports being withdrawn, stressed, and anxious, especially in social situations when she feels that people are staring at her. She reports low energy and had lost interest in most activities. Furthermore, she reports that her condition has interfered with finding employment and leading a normal life. She states that her mood is low, and she has frequent crying episodes. Her general practitioner (GP) has prescribed multiple antidepressants that were not tolerated due to the side effects. Consistent with NF1 common symptoms, she reports having migraine headaches. She averages around 15 episodes per month varying in intensity between 5/10 and 10/10. She has reportedly used OTC pain medications for the migraines unsuccessfully.\nCBD oil has no established dosing guidelines or maximum doses, except in psychosis (800 mg) and seizure disorders (2,500 mg or 25 - 50 mg/kg) []. Dosing is highly individualized and relies to a great extent on titration. The general approach to initiation is to "start low and go slow." Many patients report benefits at low doses, starting with as little as 5 - 20 mg (0.25 ml - 1 ml) per day of oral oil preparations [].\nThe risks and benefits of using cannabis for her condition were discussed in detail. She was advised to start a three month trial of sublingual CBD oil at 4 mg (0.2 ml) twice per day (BID) and titrate upwards to 10 mg (0.5 ml) BID with a maximum increase up to 20 mg (1 ml) BID if no response. She was advised to try and hold it in contact with the buccal membrane for at least 90 seconds []. The recommended formulation had low amounts of tetrahydrocannabinol (THC) at less than 1 mg per 1 ml of oil. The low levels of THC reportedly assist CBD in reaching the receptors, having a synergistic benefit [].\nAt the three-month follow-up visit, the patient reported starting with 4 mg (0.2 ml) BID of cannabis oil with CBD to THC ratio concentrations of 20 mg/ml to 1 mg/ml (CBD: THC 20:1). This was gradually increased by the patient (self-titration) to 8 mg (0.4 ml) BID. During this period, the patient reported that her pain was significantly reduced (from an average of 6/10 down to 1/10). She reported being less emotional and feeling calmer. She said that her anxiety dropped from 9/10 down to 3 to 4/10 and that her mood had stabilized. Also, as an added benefit, she reported that her migraines had improved. She reported fewer episodes per month (five per month down from 15 per month before starting the CBD oil) and that she was able to decrease her dependence on OTC anti-inflammatory pills for pain relief. Overall, a significant improvement in her quality of life was evident by the change in her demeanor and a novel enthusiasm for seeking employment.\nCBD is generally better tolerated by patients when compared to THC. Some patients may experience side effects, usually mild and infrequent, including fatigue, diarrhea, increased/decreased appetite, and somnolence []. These may be due to the low levels of THC in the formulation []. Our patient did not report any side effects during the treatment period. |
A 60-year-old Caucasian female patient was diagnosed with Gitelman's syndrome when she first presented to our renal clinic in 2000. She had documented a history of persistent hypokalemia and hypomagnesemia since the last 8 years associated with intermittent fatigue and muscle weakness. Past medical history was significant for severe symptomatic hypomagnesemia after an episode of viral gastroenteritis in her early 20s although no detailed work up was done then. She has 3 children none of whom have congenital defects. She is a product of nonconsanguineous marriage. Her brother was diagnosed with “Bartter's syndrome” in adolescence although details of the circumstances leading to either his diagnosis nor genetic testing were available. Her only medications were potassium chloride 20 meq p.o. BID and magnesium oxide 400 mg p.o. BID. She was an occasional drinker, ex-smoker quit 20 years prior, and denied using recreational drugs. Biochemical panel supporting a diagnosis of GS is presented in . Patient refused genetic testing. Subsequent to our first clinic evaluation, oral potassium and magnesium supplementation was increased and her electrolyte abnormalities remained in normal range till the event of interest.\nThe patient was lost to follow-up in the renal clinic for 4 years till she returned back in July 2008. Interval history was notable for a brief period of hospitalization in January 2008 from lumbar vertebral compression fracture due to a fall. As per records, preoperative biochemistry panel showed a serum Na of 128 mEq/L (last recorded value was normal in 2007). During the hospital stay, serum sodium dipped further postoperatively to a nadir of 122 mEq/L while on saline infusions and nonsteroidal anti-inflammatory drugs (NSAIDs) and improved spontaneously to 130 mEq/L at the time of discharge. She had no symptoms of hyponatremia, and no renal opinion was sought in-patient. Notably, a patient admitted to be drinking large amounts of water (approximately 4–6 L/a day) to “keep the system clean” since the last 1 year or so. She denied any increasing thirst or compulsive drinking, changes in memory, mood or thought process, using psychoactive substances, antidepressants, opiods, or NSAIDs preoperatively.\nOn examination, supine pulse and blood pressure were 74/min and 94/58 while readings after 2 min of standing were 80/min and 88/58 with no orthostatic symptoms. In addition, physical examination showed no jugular venous distension, clinical euvolemia and normal chest, cardiac, neurological, and psychiatric evaluation. Repeat biochemical evaluation from our clinic, which are shown in , in the context of euvolemic hyponatremia and normal thyroid and adrenal function suggested a diagnosis of a syndrome of inappropriate antidiuretic hormone (SIADH) like state. No obvious cause of antidiuretic hormone (ADH) hypersecretion could be discerned in her clinical picture prior to the vertebral fracture. A whole body computed tomogrpahy scan was also done to rule out pulmonary or intracranial lesions as potential sources of ADH hypersecretion and was negative. She was started on fluid restriction to 1.5 L a day. Within 1-month, serum sodium went up to 134 and subsequently she became normonatremic []. |
A 15-year-old teenage boy initially presented in a district hospital, with a swelling at the right side of his neck, without any significant clinical symptoms. Although full details of the work-up performed at the time are not available, he was diagnosed with a branchial cleft cyst and was offered surveillance with follow-up imaging. Eight years later, he visited our unit. Physical examination revealed a painless palpable well-defined mass () within the right carotid triangle with positive Fontaine and Kocher I signs []. There was no palpable lymphadenopathy. An ultrasound scan was performed depicting a solid mass of mixed echogenicity in the right carotid triangle echogenicity in the left carotid triangle. This was suspected to be neurogenic in origin because of its location. A digital subtraction angiography (DSA) () followed, which revealed a 60x35mm protruding mass in the right carotid bifurcation, causing local compressive effects and posterior displacement of the vessels. The patient was admitted in our unit in order to be treated surgically. During the operation a large CBP was identified as seen in . The tumor was classified as Shamblin II and was completely excised. Histopathology study showed a 3 cm carotid body paraganglioma with characteristic “zellballen” growth pattern and cell nests surrounded by prominent fibrovascular stroma, with no evidence of malignancy (). The patient had an unremarkable recovery and was discharged home on the second POD.\nThe surgical technique adopted in both patients consisted of an oblique incision along the sternal head of the left sternocleidomastoid muscle under general anesthesia. Our strategy was to expose, dissect, and isolate the proximal common carotid artery using a vascular tape. The ansa cervicalis was also exposed early in the operation. The carotid bifurcation was exposed in a caudocranial approach. The proximal external and the proximal internal carotid arteries were isolated and controlled with vessel loops. Dissection was extended to the level of the digastor muscle in order to expose and control the distal internal carotid artery. During this process, the hypoglossal nerve was identified and preserved, by following the ansa cervicalis. The external carotid artery was cross-clamped temporarily. Finally, the tumor was removed with sharp dissection from the bifurcation with meticulous technique in order to avoid injury to the internal carotid artery and the cranial nerves. The reported plane of dissection reported as a white interface plane between the tumor and the vessels was not identified in the first patient as the tumor was severely adhering to the vessel wall and was typically found in the second patient. Following the removal of the CBP, the carotid sheath and platysma were approximated and the skin closed with a continuous subcuticular suture, after performing meticulous hemostasis (Figures and ).\nBoth patients had an annual postoperative follow-up with cervical ultrasound and carotid duplex ultrasonography with no evidence of local recurrence. Familial disease was excluded clinically, by screening the patient's first degree relatives with ultrasound imaging. |
A 25-year-old girl presented to our outpatient department with sudden and severe loss of vision in both the eyes since 3 days. She was admitted in the medicine department 15 days before with a history of fever, severe malaise, headache, abdominal pain, and vomiting of 5 days duration. She had also developed maculopapular rash over the trunk, limbs, and face on the sixth day of fever. She denied any history of visual disturbance during the course of her systemic illness. On investigation, the peripheral smear was negative for malarial parasite, and all other routine investigations were within normal limits except for thrombocytopenia with platelet counts of 9,000/μL at the time of admission. A diagnosis of DF was made after she was found positive for IgM, IgG, and dengue nonstructural protein 1 (NS-1) antigen. She was started on supportive therapy and platelet transfusions, following which there was an improvement in platelet count. She was recovered and discharged from the hospital after a week.\nOn ophthalmic evaluation, the vision in her both eyes was no perception of light. External ocular examination revealed bilaterally symmetrical normally placed eyelids. Ocular movements were full and free on both sides. Anterior segment evaluation of the right eye revealed total yellowish white opaque cornea mimicking corneal abscess, thinned out inferiorly near the limbus. Left eye showed ciliary flush, mild corneal edema, medium to large sized keratic precipitates, and 360° posterior synechiae with complicated cataract precluding fundus view. The intraocular pressure (IOP) was high in the left eye. Penetrating keratoplasty was planned in the right eye (RE) to preserve corneal integrity and for the removal of inflamed corneal tissue. Intraoperative findings revealed edematous opaque cornea [], inflamatory pupillary membrane [], and complicated cataract []. Corneal graft was sutured with twelve interrupted bites using 10-0 nylon []. The specimen was sent for histopathology and microbiological examination. Preoperative B-scan ultrasonography of both eyes showed vitreous hemorrhage, total retinal detachment, and choroidal effusions [Fig. and ]. The patient was started on medical therapy for raised IOP along with topical corticosteroids and lubricants in both the eyes. Histopathological examination of corneal tissue revealed stromal breakdown with myxoid change and neovascularization. Stroma also showed mixed inflammatory infiltrate comprising neutrophils and lymphocytes []. There was no evidence of neutrophilic abscess or micro-organism. Overlying epithelium was unremarkable. Microbiological examination was unremarkable. The vision remained no perception of light in both the eyes after about 6 months of follow-up [Fig. and ]. |
The patient is a 66 year-old woman who was diagnosed with a primary desmoplastic BCC arising on the left aspect of the chin in April 2008, which was resected with Mohs surgery. A localized recurrence in the same location was identified in June 2010, and a wide local excision revealed invasion into the left mandible and involvement of one out of 18 lymph nodes. The patient received adjuvant radiation and remained in remission until 2014, when enlarging lung nodules observed on surveillance chest imaging were biopsied and confirmed the presence of metastatic BCC. The patient subsequently received the Hedgehog pathway inhibitor (HHI) vismodegib from September 2014 until February 2015. She initially responded but discontinued because of progressive disease.\nIn August 2015 after continued slow progression, the patient enrolled on the phase 1 study of REGN2810 to a cohort receiving 10 mg/kg IV every 2 weeks, and received her first dose on August 3, 2015. Two lung metastases were followed as target lesions. The patient has not experienced any treatment-related adverse events of grade 1 or higher. Response assessments at the end of 8 weeks (3 % increase) and 16 weeks (10 % decrease) demonstrated stable disease by RECIST criteria. The response assessment at the end of 24 weeks demonstrated a reduction in target lesion measurements of 37 % (Fig. ), and this was confirmed at 32 weeks. Two sub-centimeter non-target lesions in the lung were stable (not shown). The patient completed the planned 48 weeks of protocol treatment with REGN2810 on June 20, 2016, and began post-treatment follow up with her medical oncologist. As of most recent radiology assessment on August 3, 2016, partial response has been maintained and the patient continues in post-treatment follow-up (12+ months).\nNext generation DNA sequencing (NGS) of an archived recurrent disease tumor sample was performed at Foundation Medicine, Inc (315 gene panel, FoundationOne™). This identified 14 genetic alterations (PTCH1 Y334*, ERBB4 E317K, KIT M882I, ATR R668W, GRM3 S154F, MYCN P44F, TP53 P177L, TP53 Q100*, TP53 S215N, FGF6 E119K, GRIN2A R1067W,INHBA R306C, PPP2R1A P179L, and TERT promoter -124C > T) that were predicted not to be present in germline. Immunohistochemistry (IHC) analysis of PD-L1 expression in this sample was negative (no staining) in tumor and infiltrating immune cells, using the rabbit anti-human clone SP142 (Spring Bioscience, Pleasanton, CA) and the BenchMark XT automated system (Ventana Medical Systems, Inc., Tucson, AZ). |
An 85-year-old man with a past medical history significant for gastroesophageal reflux disease presented to the emergency department with a chief complaint of chronic nausea and abdominal pain. The episodes of nausea and associated early satiety had progressed to the point that the patient had lost an estimated 25 lbs. over the preceding four months. The abdominal pain was located in the epigastric region, just inferior to the xiphoid process, and increased to 10/10 in severity with food intake. Esophagogastroduodenoscopy performed six weeks prior to admission showed no significant abnormalities; no biopsies were performed because the procedure was poorly tolerated. He had tested positive for Helicobacter (H.) pylori serum immunoglobulin G (IgG) four months prior and completed a course of lansoprazole, amoxicillin, and clarithromycin. Colonoscopy two years prior revealed no abnormalities. He reported no history of diabetes mellitus or Parkinson’s disease and no prior abdominal surgery, bowel obstruction, or ischemia. Physical examination was significant for a normal abdominal exam and no focal neurologic abnormalities, and laboratory testing revealed a normal hemoglobin A1c. Computed tomography (CT) angiography shortly after admission demonstrated no findings of mesenteric ischemia. His symptoms did not improve with ondansetron 4mg po TID. A gastric emptying study revealed moderate gastroparesis, with gastric retention at one, two, and four hours calculated at 95%, 74%, and 30.5%, respectively (upper limits of normal are 90%, 60%, and 10%).\nUpon further questioning, the patient stated that his nausea and abdominal pain had begun about a year before admission when he had gone on a cruise where he had eaten an excessive amount of shrimp, followed by acute onset of nausea, vomiting, diarrhea, and severe epigastric pain. When the symptoms persisted after his return from the cruise, he was evaluated at an outside hospital, where norovirus infection was identified. The patient was unable to recall where he had received care, and thus we were unable to confirm stool reverse transcription-polymerase chain reaction (RT-PCR) or serum antibody testing results. The patient cited this episode on the cruise as the onset of his nausea and abdominal pain and remarked that he had “not felt the same” since. |
A 64-year-old, 87 kg female presented for clipping of a 4 mm wide neck unruptured saccular anterior communicating artery aneurysm which was discovered incidentally during the evaluation of headaches and memory difficulties. Her past medical history was significant for remote breast carcinoma, hypertension, obstructive sleep apnea, and gastroesophageal reflux disease. She was a lifelong nonsmoker and notable preoperative medications included aspirin, furosemide, and propanolol. Preoperative imaging had no evidence of prior infarcts. After induction of anesthesia, a right internal jugular central venous catheter and radial arterial catheter were placed and maintenance of anesthesia was performed with a combination of 0.5 MAC sevoflurane, propofol 50 mcg/kg/min, and remifentanil 0.125 mcg/kg/min infusions. Mannitol 1 gram per kilogram for a total of 80 grams was administered. The neurosurgeon placed an 80 cm closed-tip, barium impregnated lumbar drainage catheter with a 0.7 mm inner diameter (Medtronic USA, REF 46419) at the L3-4 level prior to the start of surgery.\nThe surgery commenced and was progressing unremarkably. Prior to opening of the dura, 20 mL of CSF was drained over 15 minutes per neurosurgeon request. After dural opening and during dissection of the aneurysm, the surgeon requested the lumbar drain to be opened allowing further drainage of CSF. After approximately 15 minutes, the blood pressure sharply increased over the course of 1 to 2 minutes from a baseline systolic blood pressure of 130 mmHg to over 205 mmHg with an associated decrease in heart rate from 60 bpm to 50 which resolved over the course of minutes. This acute hypertension was treated with a number of interventions including 250 mg propofol and 1 mcg/kg remifentanil boluses aimed at treating light anesthesia as well as a bolus of 7.5 mg labetalol. At the time of the hypertensive event, the propofol, remifentanil, and Sevoflurane dosing had been stable and unchanged for over an hour. There was also no change in the level of surgical stimulation at this time as the dura had been incised and the neurosurgeons were using the operative microscope to expose the aneurysm. After the aneurysm was successfully clipped, the lumbar drain was closed and the surgery was completed without further episodes of hypertension. A total of 60 mL of CSF was drained via the lumbar drain during the case in addition to the losses from the surgical field.\nAt conclusion of the operative procedure, the patient remained comatose and unresponsive despite an hour in the operating room awaiting emergence from anesthesia. A postoperative head CT was obtained prior to transport to the intensive care unit which demonstrated mild cerebral edema and borderline inferior transtentorial herniation, but no significant hemorrhage or focal abnormalities. She was then transported to the Neurocritical Care Unit intubated and ventilated. Initial arterial blood gas analysis on arrival to the ICU did not reveal a cause to her delayed emergence (pH 7.35, pCO2 47 mmHg, pO2 323 mmHg, glucose 204 mg/dL, and sodium 137 mEq/L). She did not receive any benzodiazepines during the case, but did receive 50 mcg of fentanyl on induction and 1 gram of levetiracetam in addition to the propofol, remifentanil, and Sevoflurane maintenance. Initial neurological exam demonstrated midline and equal 4 mm pupils which were reactive to light bilaterally. She withdrew to painful stimuli in all four extremities. On postoperative day (POD) 1, her mental status continued to be depressed with a Glasgow Coma Scale of 7T (E2:V1T:M4). That day, an MRI was obtained which revealed bilateral thalamic infarctions on the diffusion weighted imaging which can be seen in . Supportive care was continued and over the course of several days, her mental status slowly improved to GCS of 10 (E4:V1:M5) on POD 5. This allowed for safe extubation that day. She continued to improve and was alert and oriented to person, place, time, and situation with some memory and attention difficulties by POD 16. A timeline of her in-hospital recovery can be found in . She was discharged home with referrals for home physical, occupational, and speech therapy on POD 19. She continued her gradual neurological improvement and by over a year postoperatively, Neurology records indicated she was living independently and her Montreal Cognitive Assessment score had recovered to a normal 27/30. |
An 80-year-old male was involved in a two-car collision as a driver of one of the vehicles. His seat belt was fastened. Physical examination revealed a blood pressure of 70/30 mm Hg, a heart rate of 83 bpm, oxygen saturation 70%, and decreased vesicular sounds. He was intubated, and he was treated with bilateral thoracotomy tube placement. Bloody thoracic effusion was confirmed by a left thoracocentesis. Approximately 450 ml of sanguineous effusion was drained by left thoracocentesis. He was immediately transported to Juntendo University Shizuoka Hospital. Thorough transportation, 200 ml of bloody thoracic effusion was drained subsequently. On arrival, hemoglobin was 10.2 g/dl. Chest radiograph revealed a normal mediastinum. His Focused Assessment with Sonography for Trauma (FAST) window was negative for fluid except left pleural effusion. Total body CT scan demonstrated left hemothorax with extravasation of contrast and slight pericardial effusion without extravasation of contrast (Fig. ). Left rib fractures, slight lung contusion in the left lower lobe, and clinically not significant left pneumothorax were also seen by the CT. Fifth and sixth rib fractures were not displaced. The patient was transferred to the intensive care unit, we used non-operative management in watchful waiting, because hemorrhage volume did not reach the criterion for operability and hemodynamic states were stable at first.\nWe took care of the patient in ICU for 3 h. We gave transfusion of red blood cells (12U) in order to maintain blood pressure of 100/60 mm Hg and a heart rate of 70 bpm and to maintain hemoglobin level of at least 10.0 g/dl for 3 h. This clinical condition meant hemodynamically unstable, we decided to perform thoracic surgical exploration in the operating room. We predicted that the sources of hemorrhage were the fifth and sixth intercostal arteries and lung contusion in the left lower lobe adjacent rib fractures. We performed left posterolateral thoracotomy, so that we could extend this incision. Thoracotomy revealed bloody effusion and clots, but revealed absence of intercostal artery injury. We identified large amount of fresh blood and clots below the left proximal pulmonary artery, and we identified pericardial lacerations which ran about 30 mm. Then, we extended the incision to anterolateral by about 10 cm. We observed this area carefully to confirm that fresh blood emerged from pericardial space. Then, we dissected pericardium of 50 mm in diameter in order to detect the source of hemorrhage. We detected that a single left appendage laceration ran about 10 mm. Left appendage bleeding could be controlled by astriction (Fig. ). We evacuated approximately 1500 ml of fresh blood and clots from the left thoracic cavity. Subsequently, we compressed bleeding point with Tissue Sealing sheet (TachoSil®, Tokyo, Japan) for 15 min. Exploration of heart and left pleural cavity did not show other injuries such as circumflex coronary artery injury and lung contusion and diaphragm injury. The patient’s postoperative course was uncomplicated. Two pleural drains were removed on postoperative day (POD) 7 and on POD 15, respectively. The patient was discharged on POD 49 without a sign of pleural effusion.\nCardiac rupture is an injury to the wall of the heart, usually associated with blunt trauma. The condition is rare, occurring in less than 2% of trauma patients in most reports, and the mortality remains high, which is generally attributed to failure to diagnose the condition. Quick diagnosis and repair of cardiac rupture are significant to the optimization of outcome [].\nCardiac rupture is classified into two categories depending on pericardial laceration. Thirty percent of cardiac ruptures are accompanied with pericardial lacerations [], which leads to hemothorax. Seventy percent of the cardiac ruptures are accompanied with cardiac tamponade. Usually, chest surgeons perform examinations of hemothorax at emergency department. Thus, chest surgeons must recognize the possibility of cardiac rupture with pericardial lacerations, which can produce a clinical picture of hemothorax.\nThis patient course provided one important clinical suggestion. Pericardial effusion is useful for building clinical reasoning. We can suspect cardiac rupture as the cause of hemothorax based on pericardial effusion, even if those are small in amount. Cardiac rupture patients who have pericardial tears present as hemothorax, because pericardial lacerations avoid gathering of intra-pericardial fluid [, ]. To the best of our knowledge, 11 previous alive cases of blunt cardiac rupture with concurrent hemothorax have been reported (Table ). Pericardial effusion on computed tomography (CT) was detected in six cases (54%). The other reports (46%) did not mention existence or non-existence of pericardial effusion. Pericardial effusion may be useful to predict cardiac rupture, even if there are small amount of pericardial effusion. In our case, pericardial effusions are little and there is no extravasation of contrast in the pericardial sac. Contrast collection in the pericardial space has unknown specificity of cardiac rupture in previous reports. Therefore, we must focus on pericardial effusions for rapid diagnosis and treatment when examining a hemothorax patient.\nWe should immediately examine sources of bleeding through thoracotomy if bleeding points are unclear on CT. Thoracotomy prove to be both diagnostic and theoretically therapeutic []. When the patient has massive bleeding unexplained by the estimated bleeding point, thoracotomy must be done. A question arises as to the option of incision. Once cardiac rupture is strongly suspected, incision is basically rapid, especially in cardiac arrest. Left anterolateral thoracotomy is speedy but it gives very poor access to any cardiac structure except the apex of the left ventricle []. In addition, the extension of this incision across the sternum into a clamshell (bilateral anterior thoracotomies) leads to four more bleeding vessels (internal mammary arteries and veins) to contend with []. However, in our case, we ought to have chosen the left anterolateral thoracotomy in supine position for an exploration of the left thorax, because the advantage of the left anterolateral thoracotomy is that the incision might be extended to the right if we needed to explore the right thorax.\nA query arises about the option of place at which we operate thoracotomy. For the rapidly decompensating or arresting patient, emergency thoracotomy, i.e., left anterolateral thoracotomy in an emergency room has proven to be life-saving []. Even if the patient is hemodynamically stable, initial drainage of more than 1000 mL or constant bleeding is at a rate more than 200 ml/h for four consecutive hours from an inserted thoracic drain [], then we must make thoracotomy prior to cardiac arrest. Meanwhile, “200 ml/h” is one of the indicators, because in our case drainage function from the chest tube was interfered by clot formation. Moreover, our patient might have a chance to go to the operating room earlier in order to stop the bleeding. |
A male patient aged 6 years and 8 months was referred to our pediatric dental clinic for hard-tissue removal around the crown of an impacted maxillary right first molar. The medical histories of the patient and his family were normal. Panoramic radiography and cone-beam computed tomography (CBCT) revealed that less than one-fourth of the root was formed, and it was shorter than that of the corresponding left maxillary molar; additionally, the crown was covered by a radiopaque lesion (Figure ). The differential diagnosis included odontoma, retention of the right maxillary first molar, and delayed root formation. The treatment plan involved the removal of the lesion to facilitate the eruption of the molar. During the first surgical procedure, which was performed under local anesthesia, the hard tissue covering the tooth was exposed to the oral cavity; however, the lesion could not be separated from the tooth. In due course, the tooth gradually emerged along with the lesion attached to it. A step lesion excavation was planned using an ultrasonic bone scalpel (Varios G95 and 970; Nakanishi Inc, Tochigi, Japan) and diamond bur. The procedure was painless and repeated five times over a period of 9 months from the age of 7 years and 3 months. At 7 years and 8 months of age, the occlusal surface of the molar located beneath the gingiva demonstrated a chalky-white appearance and was filled with carboxylate cement to prevent pulp infection. Instructions on the use of a tuft brush were given to the patient's parents (Figure ).\nAt 8 years and 2 months of age, CBCT revealed remnants of the lesion, which appeared to be attached to the pulp (Figure ). Therefore, the stepwise removal of the lesion was stopped, and an adhesive resin (Bondfill SB; Sun Medical Company, Ltd., Shiga, Japan) was applied to both the crown and the lesion as a protective measure (Figure ). At 9 years of age, the molar demonstrated occlusal contact with the opposing mandibular first molar, a positive reaction to the electrical pulp test, and normal tooth mobility.\nThe removed lesion fragments had enamel-like, glossy, and irregular surfaces. Histopathology conducted using hematoxylin and eosin staining revealed the presence of multiple holes accompanied by concentric cross-striation rings, while microcomputed tomography (microCT, SMX-100CT; Shimadzu Corporation, Kyoto, Japan) showed a highly calcified lesion with an X-ray impermeable area at the center (Figure ). Accordingly, the lesion was diagnosed as an enamel-like tissue-dominant complex odontoma that was partially attached to the first molar. |
A 43-year-old female was transferred to our hospital with asymptomatic non-ST-elevation myocardial infarction (). Cardiac troponin T was elevated (0.32 μg/L), and normal coronary angiography suggested embolic infarction (Figures –).\nSix weeks earlier the patient had been admitted to a stroke unit at an external hospital with hemiparesis of the right upper extremity, bilateral paraparesis, and dysphagia. A cerebral CT scan revealed acute posterior circulation territory ischemia. On the next day, the patient displayed pulselessness and cyanosis of lower extremities and elevated creatinine kinase (4,000 Units/L). An abdominal CT scan revealed embolism of the abdominal aorta, splenic and bilateral renal artery infarction, and multiple additional emboli in bilateral common and superficial femoral artery, left common iliac artery, and left popliteal artery. Emergency percutaneous embolectomy of aorta, bilateral iliac artery, right renal artery, and bilateral femoral artery was performed. Lysis catheters were placed in right and left popliteal arteries, and treatment with unfractionated heparin and urokinase was started and continued for one week. One day after initiation of local lysis, selective embolectomy of bilateral dorsal pedis and posterior tibial arteries was performed due to ongoing signs of lower extremity ischemia. Despite these efforts, the right lower leg had to be amputated one week later owing to irreversible ischemia. Ten days later, the patient developed right paraparesis due to acute media infarction confirmed by cranial CT scan. Three weeks prior to myocardial infarction, she was diagnosed with paralytic ileus, and ischemic segments of the small intestine were resected. Histological examination of embolic material removed from the right lower leg showed polygonal cells with eosinophilic cytoplasm and myxoid matrix, consistent with atrial myxoma. Transesophageal echocardiography (TEE) revealed an echogenic structure (1.0 × 0.5 × 0.5 cm) with a villous surface appearance in the left atrium attached to the fossa ovalis that was first diagnosed as atrial thrombus. At that time, the patient was deemed not suitable for open heart surgery due to a risk of intracerebral hemorrhage following media infarction, and she was scheduled for surgery 4 weeks later. Further retrospective anamnesis revealed that the patient was diagnosed with vasculitis 7 months earlier. Vasculitis was suspected owing to cachexia, night sweating, and elevated inflammatory markers including C-reactive protein (33.1–35 mg/L), leukocyte count (10.5–18.4 cells/nL), and erythrocyte sedimentation rate (69 mm/h). Furthermore, temporary weakness of the right arm and transient scotoma in the right eye suggested cerebral vasculitis, which was further supported by cranial magnetic resonance imaging revealing small lesions indicative of lacunar ischemia.\nFollowing coronary angiography in our institution, the diagnosis of left atrial myxoma was confirmed by TEE (Figures and ), and the patient was transferred to the department of cardiothoracic surgery. The myxoma was removed and atrial septum reconstruction was performed using a pericardial patch. During followup visits, the patient did not report any symptoms of embolism, and recurrence of left atrial myxoma was excluded by TEE two years after surgery. |
A 58 year old lady was referred by her General Practitioner (GP) after presenting with a 3 day history of moderate left sided pleuritic chest pain radiating to the left shoulder, low grade pyrexia and frontal headaches. She experienced no cough, hemoptysis, or shortness of breath but had recurrent episodes of upper respiratory tract infections in the past five months. There was no significant past medical history and she was a lifetime non smoker.\nClinical examination revealed left lower lobe crackles. Routine blood tests revealed a White Cell Count of 11.82 109/lt and a C-Reactive protein (CRP) of 164 mg/lt. The chest radiograph (CxR) demonstrated a raised left hemi diaphragm with volume loss in the left lower lobe (Fig ).\nDue to the equivocal findings on the CxR, she underwent a Computerized Tomography (CT) (Fig ,) of her thorax which revealed a large necrotic mass within the left lower lobe extending into the posterior mediastinum, surrounding the aorta and in close contact with the distal esophagus. It extended to the gastric fundus through extensive invasion of the left hemi-diaphragm. No mediastinal lymphadenopathy was reported.\nA CT guided biopsy was performed and the histology showed a spindle cell tumor. Immunohistochemistry revealed the tumor cells to be positive for CD 34, h-caldesmon (muscle marker) and focally positive for epithelial membrane antigen. The possibility of a 'solitary fibrous tumor' was raised as the clinical history indicated that this was primarily a chest tumor. She was subsequently referred to our team.\nAt thoracotomy the tumor was invading the left lower lobe but large part of it was descending in the posterior mediastinum and through the hiatus. The initial incision was extended to a left thoracolaparotomy and the mass was resected en block performing a left lower lobe lobectomy, a distal esophagectomy, partial gastrectomy and diaphragmatic resection with primary esophagogastric anastomosis and diaphragmatic reconstruction.\nOn histopathological examination the tumor was predominantly composed of spindle shaped cells, with oval nuclei and eosiniphilic cytoplasm. Focal myxoid change was present. There was some nuclear pleomorphism and in areas the mitotic count was more than 5/50 high power field (HPF). Although the tumor was predominantly within the lung parenchyma, it was seen to be arising from the esophageal wall, where it extended into the lamina propria and attenuated the overlying esophageal squamous epithelium (figure ).\nOn immunohistochemistry, the tumor cells revealed strong diffuse positivity for CD 34 and CD 117 (c-kit) (figure ). The cells were focally positive for the muscle markers including h-caldesmon, desmin, actin and myoglobin. Focal positivity was also seen with epithelial membrane antigen and cytokeratin (MNF 116). The tumor was negative for S 100 protein CD 99, Thyroid transcription factor (TTF 2) and calretinin.\nOn the basis of the above findings, the tumor was classified as a gastrointestinal stromal tumor arising from the esophageal wall and extending into the lung parenchyma. As the tumor measured more than 10 cm in size, with a mitotic count over 5/50 HPF extending into the lung parenchyma, it was categorized as a high risk GIST.\nFollowing post-operative recovery, the patient was referred to the oncology team for assessment regarding adjuvant therapy. |
A 42-year-old woman with a history of chronic migraine presented to the emergency department for a sudden thunderclap headache after swimming. She regularly used nonsteroidal anti-inflammatory medication and ergotamine associated with caffeine for chronic headache. At admission she described diffuse, explosive, and unusual headache with spontaneous resolution in 8 h. On initial examination, general and neurological clinical examination was normal. Arterial blood pressure was normal. The initial cerebral computed tomography (CT) scan without contrast was normal. The cerebrospinal fluid sampling obtained by lumbar puncture revealed presence of oxyhemoglobin with 290 red blood cells/mm3. The diagnosis of SAH was retained and a cerebral angiography scheduled for the next day. On day 1, she presented severe unusual headache with arterial hypertension followed by aphasia and a right hemiplegia. Shortly afterward, her consciousness worsened requiring intubation and intravenous sedation. A cerebral CT scan was immediately performed and revealed a left rolandic ICH (Fig. ). Cerebral angiography was performed showing a diffuse segmental narrowing of cerebral arteries without aneurism or arteriovenous malformation (Fig. ). The diagnosis of RCVS with hemorrhagic complication was retained and intravenous nimodipine was introduced. The ICH was removed by a neurosurgeon and an ICP monitoring probe was inserted in the left frontal lobe. Initial ICP reading was 15 mm Hg. Intravenous sedation was continued in the intensive care unit. However, norepinephrine was required to keep cerebral perfusion pressure (CPP) over 60 mm Hg. Immediate postoperative transcranial Doppler (TCD) was normal. A control cerebral CT scan was performed the next day showing a residual intracranial hematoma and perilesional edema. Forty-eight hours later, ICP increased to above 30 mm Hg despite optimization of medical treatment (head elevation, increased sedation, and osmotherapy). TCD readings for the right middle cerebral artery (MCA) were: pulsatility index at 1.6 and diastolic velocity at 23 cm/s, and for the left MCA: pulsatility index at 1.6 and diastolic velocity at 21 cm/s, probably corresponding to raised ICP. A new cerebral CT scan revealed similar findings. A left DC was performed facing refractory intracranial hypertension. Thereafter, ICP and TCD normalized and sedation was stopped in the following days. Mechanical ventilation was successfully weaned on day 7. The patient was discharged from the intensive care unit at day 14 with aphasia and right hemiplegia with progressive improvement from 3 months. She was independent at 6 months with a modified Rankin scale score of 2. Magnetic resonance imaging found no residual signs of RCVS at 3 months. |
A 48-year-old male presented with progressive enlargement of right testis of 3 weeks duration. There was associated dragging sensation in the right testicular region and in the lower abdomen. No associated urinary symptoms, fever or weight loss. No medical history of diabetes or hypertension and no family history of any solid or hematological cancers. Physical examination revealed a large right testicular swelling of 10 cm × 8 cm size, firm in consistency with mild pain on palpation, testicular sensation was preserved. There was no accompanying palpable inguinal adenopathy. Contra lateral testis was normal in size and unremarkable on physical examination. Systemic examination was unremarkable. Initial laboratory data revealed mild normocytic normochromic anemia with hemoglobin of 10.2 g/dL, platelet count of 2.9 lakhs/cu mm, lactate dehydrogenase of 369 U/L. Chest radiograph was normal. Patient underwent a computed tomography (CT) scan of the abdomen and pelvis, which revealed an enlarged right testis of uniform density with no retroperitoneal lymphadenopathy and right adrenal was mildly enlarged. FNAC of right testis showed provisional possibility of poorly differentiated carcinoma. Positron emission tomography/CT (PET/CT) was performed for initial assessment and staging, which revealed a markedly enlarged right testis showing intense metabolic activity with standardized uptake value (SUV) max of 35.5. Normal sized left testis also showed intense Fluoro deoxy glucose uptake with SUV max of 15.9. Hyper metabolic activity was also noted in loco regional small volume inguinal nodes bilaterally with SUV max of 15.5. There were additional areas of hyper metabolic activity noted in extra testicular extra nodal sites such as, contra lateral testis, adrenals, and small bowel loops []. Metabolically active area of a CT based hypo dense area encircling the superior vena cava right atrial junction []. Hyper metabolic nodule was noted in the subcutaneous planes of right thigh []. Patient underwent a right inguinal orchidectomy. Histopathology showed enlarged testis measuring 9.5 cm × 6 cm × 6 cm. Cut section was fleshy and grey white. Normal testicular tissue was not evident. Microscopy showed entrapped seminiferous tubules in sheets of monomorphic dyscohesive cells. These cells are large and show vesicular pleomorphic nuclei, clumped chromatin, small nucleoli, and a minimal amount of cytoplasm. Mitosis is increased. Surgical margin of the spermatic cord was free of tumor. Tunica was not infiltrated with tumor []. Immuno histo chemistry was carried out for further typing which showed diffuse membranous CD-20 positivity with increased Ki67 index of 85-90%. CD10 was negative. Findings were consistent with primary testicular diffuse large B cell lymphoma, Post surgery chemotherapy with Cyclophosphamide, Hydroxydaunorubicin, Oncovin (Vincristine), Prednisolone regimen was followed by an interim PET after four cycles of chemotherapy, which showed complete morphological and metabolic regression of all the lesions []. |
A 47-year-old man had started approximately 10 years ago as truck driver delivering fish and other seafood stored on crushed ice. He was in charge of the final quality check of the delivered seafood which included handling of single fish of various species and crustaceans. Plastic skirt and long-sleeved gloves were provided by the employer and were initially invariably used when handling raw seafood. But over the years with increased workload and pressure of time, upon arrival at the client in his rush he frequently did not take the time to change and therefore often times did not wear protecting clothes leading to increased direct contact with fish, crustaceans, the storage ice, and ice water.\nTwo years prior to presentation at our allergy clinic, the patient first developed itchiness, redness and swelling of the conjunctivae and eyelids one to two minutes after a single drop of ice water splashed into his eye. Although thereafter he tried to strictly avoid skin and mucosal contact, he developed several times within minutes after incidental direct contact to fish, crustaceans, or the storage ice pruritus, erythema and urticaria restricted to the contact sites on the hands and forearms. At one instance with rather intensive contact (carrying a full container by pressing it against the lower abdomen) he developed not only wheals at the contact sites but generalized urticaria. These symptoms subsided within two hours not requiring specific therapy.\nOne year later when consuming a zander/Sander filet which he had been offered by a client, he suffered from generalized urticaria with facial angioedema, nausea, vomiting and defecation. Emergency treatment with intravenously applied fluids, H1-antihistamines and corticosteroids lead to complete resolution of anaphylaxis symptoms within two hours. Previously, he had only sporadically consumed seafood, which he had tolerated until then without symptoms. After a second episode of generalized urticaria and angioedema after tasting a small amount of smoked eel/Anguilla and a shrimp/Penaeus salad he avoided all seafood.\nBecause of steadily progressive skin symptoms during seafood delivery the truck driver was transferred by the officials of his company to the washing unit of the fish delivery company where amongst others fish transport tanks are cleaned with pressured water. There on his first workday, when cleaning fish transport tanks he developed dyspnoea with exspiratory stridor. Immediately he stopped the cleaning procedure and left the room. Due to the severity of the asthma symptoms emergency treatment with inhalative and subcutaneously applied β-agonists was required.\nThis case illustrates the progression of food allergy symptoms depending on the site of allergen contact []. Presumably, percutaneous sensitization occurred by direct contact of skin and mucosa to fresh seafood stored on crushed ice because these were the initial sites of contact urticaria. With ongoing allergen exposure ingestion of fish and shrimps lead to anaphylaxis before inhalation of seafood-aerosols resulted in asthma symptoms.\nOn physical examination at our allergy clinic several clinical stigmata supporting atopy were observed: dry skin, keratosis pilaris, pityriasis alba, infrorbital skin fold and white dermographism. Routine laboratory parameters were within normal limits. Total serum immunoglobulin E (IgE) was highly elevated with 1.330 kU/L. Screening prick testing with inhalative allergens such as pollen, cat and dog dander, house dust mites and common food allergens including cow's milk, egg, finned fish, crustacean and hazel nut revealed IgE-mediated sensitizations against herring/Clupea (after 20 minutes wheal diameter 15 mm) and shrimp/Penaeus (after 20 minutes wheal diameter 22 mm including pseudopods). During the skin testing procedure the patient developed generalized pruritus and dyspnoea. These symptoms subsided after treatment with inhalative β-agonists, intravenously applied H1-antihistamines and glucocorticoids. Allergen-specific serum IgE against herring/Clupea (f205, CAP System, Pharmacia Diagnostics, Uppsala, Sweden), sardine/Sardina (f308), swordfish/Xiphias (f312) and shrimp/Penaeus (f24) was measured as 29.2, 6.6, 4.2, and 19.2 kU/L, respectively.\nThe most important heat and ingestion resistant fish allergens are parvalbumines, e.g. the 12 kDa muscle protein Gad c 1 []. Approximately 70 % of all patients with fish allergy develop symptoms to several fish species, the remaining 30 % react to only one fish species [,]. Tropomyosin (Pen a 1), another muscle protein, is the most important allergen of crustaceans []. Cross-reactivities within several crustacean species are common, therefore all crustaceans should be strictly avoided. While cross-reactivity of crustaceans with bony fish (Osteichthyes) is unlikely, cross-reactivity with mollusca (e.g. bivalvia), insecta (e.g. cockroach) and arachnida (e.g. house dust mites) are possible [].\nDue to typical clinical symptoms after exposure to seafood and positive test results diagnosis of IgE-mediated allergy to finned fish and crustaceans was established. The patient's allergy to crustaceans and finned fish was recognized as an occupational disease. Cause for sensitization was probably the ongoing skin contact to native fish and crustaceans for years. Sensitization was facilitated by irritative factors such as wet and cold working conditions (ice water) as well as his atopic background with consecutive disturbance of the physiologic skin barrier function. It has been shown that storage conditions may influence the skin irritancy of fish juice; fish kept on ice for several days enhances frequency and severity of symptoms such as itching, stinging, and erythema []. However, the most important risk factor for IgE sensitization against fish and/or crustacean proteins is atopy []. In a recent study of employees in the seafood processing industry skin symptoms were predominantly moderate and seldom interfered with working capacity []. However, in our case due to the severity of allergy symptoms, including generalized urticaria and asthma, there was a need for termination of the hazardous occupation, which in German jurisdiction is a crucial requirement for approving a condition as an occupational disease [,]. In case of accidental allergen contact leading to anaphylaxis the patient received emergency medication including an epinephrine-containing autoinjector and was instructed on the usage []. |
A 45-year-old man presented to our hospital in Belarus with the following history. He had been born in Belarus after an uncomplicated pregnancy. His family history was negative for congenital defects and the patient had no siblings. After birth, a systolic murmur was heard along the left sternal border leading to a referral to a pediatric cardiology centre in Ukraine where the diagnosis of tetralogy of Fallot was given based on physical examination, chest radiographs, and an electrocardiogram. Surgery was declined at that time. At no time in the patient's life were chromosomal studies undertaken. The patient had no children.\nAs a child, the patient had normal growth and mental development, but marked cyanosis, weakness, clubbing, and intolerance of moderate physical activity. The patient was referred to a medical institute in Moscow at age 18, after several episodes of syncope. A diagnosis of severe pulmonary stenosis with ventricular septal defect was considered at that time. A right Blalock-Taussig shunt was performed. The postoperative diagnosis was single ventricle type BIII with severe pulmonary artery stenosis and hypoplasia. After surgery, the patient was treated with digoxin, pentoxifylline, and spironolactone. The patient's condition improved significantly and he was able to walk several blocks without significant dyspnea.\nThe patient's condition remained stable for the next five years. He had shortness of breath with moderate exertion, but he was asymptomatic at rest. At 27 years of age, the patient reported an increase in dyspnea with minimal exertion. He was diagnosed with thrombosis of the Blalock-Taussig anastomosis and was treated with heparin for 4 weeks. He never returned to his improved, postoperative condition. He complained of palpitations, dull chest pain at rest, episodes of shortness of breath at rest, and abdominal pain. The patient had several documented episodes of ventricular tachycardia at age 35 years and was successfully treated with propafenone.\nAt 39 years of age, the patient presented to our hospital in Belarus. At the time of presentation, he complained of severe cyanosis, shortness of breath with minimal exertion, and chest pain. An electrocardiogram indicated sinus tachycardia of 120 beats per minute. The QRS axis was to the right (mean axis +130°). High QRS voltage suggestive of ventricular hypertrophy was noted. There were also ventricular extrasystoles in a trigeminal pattern and horizontal ST-segment depression in the inferior leads. A 24-hour cardiac monitor showed multiple episodes of non-sustained ventricular tachycardia with subjective feelings of palpitation and lightheadedness.\nThe patient was switched empirically from propafenone to mexiletine with better control of his ventricular tachycardia. His hematocrit was 58% to 64%. Transcutaneous oxygen saturation was 75% to 85% on room air. The patient could tolerate well most of his daily activities such as walking for two blocks, grocery shopping, and performing minor work at home. Over the next 6 years he had repeated hospitalizations for dyspnea, chest pain, and near syncope. He was treated with phlebotomies, saline and/or dextran infusions to improve viscosity, and medications including spironolactone, pentoxifylline, and aspirin.\nAt the time of the terminal hospitalization, physical examination was remarkable for a 3/6 systolic murmur heard at the upper left sternal border, edema of the right ankle and foot, and tachycardia of 140 beats per minute. The patient was noted to experience severe shortness of breath with even minimal exertion and was deeply cyanotic. The electrocardiogram is shown in Figure and demonstrated sinus rhythm with a mild tachycardia at a rate of 110 beats per minute, right axis deviation, and a non-specific intraventricular conduction delay with diffuse ST-T changes. Chest radiograph indicated a small pleural effusion on the right and increased pulmonary vascularity. The heart was moderately enlarged with prominence of the aorta. An echocardiogram was performed (Figure ). A single ventricle with unremarkable atrioventricular valves was seen. The end diastolic diameter of the ventricle was measured as 69 mm, the posterior wall thickness was 17 to 19 mm, and the left atrium measured 29 mm. The estimated ejection fraction was 45% to 50%. A vessel with a semilunar valve (the truncal valve) arising from the ventricle was seen. The cusps of the valve were hyperechogenic. Moderate regurgitation was noted. No pulmonary artery was seen. Doppler study of the lower extremities showed thrombosis in the veins of the right calf. Heparin therapy and intravenous fluids were initiated. The patient's condition deteriorated rapidly and several hours later he became comatose and died.\nAutopsy showed an enlarged heart that weighed 750 g, composed of two atria with an intact septum, and a single ventricle. The right atrium was enlarged to a diameter of 10 cm. The diameter of the left atrium was 3 cm. Both caval veins emptied normally into the right atrium. All four pulmonary veins entered the left atrium normally. Both atrioventricular valves had normal anatomy, free of vegetations. The single ventricle with left ventricular characteristics had a diameter of 12 cm with a wall thickness of 2 to 2.2 cm. A single artery, truncus arteriosus, arose from the ventricle and arched to the left. The truncal valve had three cusps that were moderately calcified. The coronary ostia and vessels were normal. The pulmonary artery trunk was located 3.5 cm from the origin of the truncus and divided to form left and right pulmonary arteries. At the hilum of the right lung the right pulmonary artery was surgically connected to the right subclavian artery. Red-gray masses were noted at the Blaylock-Taussig shunt anastomosis. Below the anastomosis the right pulmonary artery was almost completely occluded by a dark red adherent thrombus. The ligamentum arteriosum was a fibrous cord. Microscopically the lungs showed dilatation of the pulmonary arterioles and alveolar capillaries. Many bronchial and pulmonary arterioles contained recanalized thrombi or emboli.\nThe main anatomic diagnoses were: single ventricle with truncus arteriosus; status post Blalock-Taussig procedure; old thromboses of the established anastomosis; hypoplasia of the right pulmonary artery; congestive heart failure; recent thrombosis of the right pulmonary artery; and deep vein thrombophlebitis of the right calf. |
A 46-year-old Caucasian man was admitted to our department due to pain in his right proximal thigh. Medical history revealed no contributory family and past histories, and no neurological deficits were detected by physical examination.\nX-rays and MRI presented an expanding 10 cm lytic lesion with an inhomogeneous contrast medium enhancement occupying the right proximal femur and extended into the proximal shaft (). The lesion showed amorphous density resulting from a high-grade destruction of the cancellous bone. A perforation of the osseous cortex with an adjacent extraosseous mass was detected. Subsequently performed tumor staging including soft tissue scans of chest, abdomen, and pelvis gave no evidence of metastatic disease or evidence of another primary tumor.\nBiopsy specimen revealed a malignant mesenchymal fascicular spindle cell proliferation with smooth-muscle differentiation reflected by the expression of a-SMA (a-smooth-muscle-actin), desmin, and caldesmon by immunohistochemistry. The patient underwent limb salvage surgery followed by endoprosthetic replacement (). Wide surgical margins were achieved and after a 23-day inpatient stay, the patient was discharged from hospital with postoperative chemotherapy according to the EUROBOSS study protocol.\nThe explant consisted of a 25 cm long proximal femur () with a 10 cm tumor in largest diameter that mainly occupied the medullary cavity. An area of cortical perforation with an extraosseous soft tissue mass of 2 cm was seen. The tumor showed a grey-white cutting surface ().\nThe histological evaluation revealed a malignant fascicular spindle cell proliferation with an infiltrative growth pattern composed of interweaving fascicles. The tumor cells were spindle shaped, elongated, blunt ended, hyperchromatic, focally pleomorphic, and surrounded by a prominent eosinophilic cytoplasm (). With antibodies against a-SMA, desmin, and caldesmon the majority of the tumor cells showed a specific positive reaction. Immunohistochemistry using antibodies directed against Cytokeratin and S-100 were negative (). In the area of the previous biopsy site a spot of 0.2 mm of cartilaginous differentiation was found.\nAfter one year of followup, a successfully performed one-stage revision surgery became indispensable due to a late-onset infection of the primary prosthesis. For more than 9 months of followup after first surgery no evidence of metastatic disease or local recurrence was seen. Although two 2 mm lesions were detected in both lungs by soft tissue scans the lesions did not show any progression during the following 16 month of followup. |
A 48-year-old man was referred to our clinic due to intractable epistaxis on the left side. Although the patient had no remarkable medical history, he had been smoking 20 cigarettes per day for 30 years and had received no medical checkups or dental care for many years. On arrival, the source of bleeding was not visible due to continuous bleeding, and his blood pressure was significantly high (194/125 mmHg). Upon intraoral examination, marked accumulation of plaque and calculus was observed at the gingival margin of his maxillary left premolar and molar teeth (). Blood test revealed no significant abnormalities, including diabetes. The bleeding was stopped by nasal packing and nasopharyngeal balloon (hereafter referred to as anterior-posterior nasal packing: AP nasal packing). After hospitalization, a cardiologist started treatment for hypertension and his systolic blood pressure (SBP) decreased to 140–160 mmHg with 40 mg of nifedipine and 1.25 mg of bisoprolol fumarate, and then the pack was removed 5 days after packing. However, 2 days later, epistaxis relapsed, and he was referred to the interventional radiology division for angiography on the same day.\nAngiography of the external carotid artery revealed a well-developed left sphenopalatine artery (SPA) and retained contrast medium in the nose, but there was no extravasation. The left MA was selectively embolized using porous cellulose beads (PCBs; Asahi-Kasei, Tokyo, Japan) 230 and 400 μm in diameter. After embolization, the SPA and descending palatine artery (DPA) disappeared (). There was no bleeding after the procedure, although the patient reported slight pain in the left upper teeth, which increased gradually. On the day after removal of the AP nasal packing, the SBP was well controlled to around 120 mmHg by adding 80 mg of valsartan. He was discharged from the hospital 5 days after embolization because of no further epistaxis.\nNine days after embolization, he visited the dental clinic in our hospital because of increasing tooth pain. Marked gingival necrosis was observed on the palatal side of the left maxillary premolar and molar, and the alveolar bone was exposed due to the loss of gingiva (). The buccal side was intact and there was no tooth mobility. Panoramic and dental radiographic images detected a carious cavity, but no defects in the alveolar bone (Figures and ). He was hospitalized again and treated with necrotomy of the gingiva under local anesthesia preserving the alveolar bone, intravenous injection of ceftriaxone sodium (2 g/day), clindamycin (1200 mg/day), and prostaglandin E1 (120 µg/day) and hyperbaric oxygen therapy for 2 weeks. The severe pain was controlled well by oral tramadol hydrochloride/acetaminophen. While granulation occurred gradually around the wound, resorption of the alveolar bone occurred, and the palatal side of the first molar was fully exposed (). At 3 weeks after admission, the maxillary left first molar showed mobility, and the tooth was extracted. The extracted tooth had cavities and marked calculus accumulation on the root surface (Figures and ), indicating periodontitis that may have developed for several years. Two months after embolization, the wound was covered by granulation tissue and had epithelialized almost completely, with the exception of the deep socket on the palatal side (). |
A 6-year-old Ethiopian boy was admitted to the pediatric intensive care unit of the Black Lion Specialized Hospital with medulloblastoma and hydrocephalus. He was the first child in his family and came from low socioeconomic status. No history of birth defect, head injury, trauma, central nervous system infections, malignancy and related conditions, except that a year prior, he had been admitted in the private hospital intensive care unit, for more than a month; a ventriculoperitoneal shunt was placed for treating hydrocephalus but there was no recorded history of infections and complications. He presented in our hospital with high-grade global type of headache, poor appetite, nausea, vomiting, tiredness, decreased vision, and drowsiness. These are typical signs and symptoms for medulloblastoma. However, at admission he had no fever, cough, or chest pain. He was also conscious, with a Glasgow Coma Scale (GCS) of 15/15, with no motor deficit or sensory disorder. On admission, his blood pressure (BP) was 140/85 mmHg, with a regular heartbeat of 60 beats per minute, heart sounds were clearly auscultated with no murmur, there was no murmur of mitral insufficiency or ventricular defect, no signs of heart failure, and he was without edema of his lower limbs. His peripheral pulses were perceived symmetrically. The rest of the physical examination was also normal.\nOn admission, a brain computed tomography (CT) scan and brain magnetic resonance image (MRI) was performed and indicated the presence of medulloblastoma (a mass that was involved the posterior fossa, Fig. ). An immediate craniotomy procedure was performed for the removal of the mass. A biopsy was also performed and revealed no malignancy or spread of brain tumor. The radiologist and neurologist team suggested that no further treatment was needed, radiotherapy or chemotherapy. However, following the craniotomy procedure, the neurological findings progressively deteriorated. This showed he had developed obstructive hydrocephalus as he presented with gait disturbance, low attention, seizure, and uncontrolled body movement with an elevated intracranial pressure (ICP). To restore this CSF circulation within both lateral ventricles, a ventriculoperitoneal (VP) shunt was placed. However, his mental status could not be improved. Thus, on the 4th day, the VP shunt was replaced with external ventricular drainage (EVD) which was undertaken to control the elevated ICP because of hydrocephalus.\nOn the 6th of the surgery, he became comatose with a GCS score of 7/15 and hemiparesis. He needed nasogastric tube (NGT) feeding for nutrition. Meanwhile, he presented with high-grade fever (39.8 °C), severe headache, vomiting, lethargy, and deteriorating consciousness. The CSF examination revealed an elevated white blood cell (WBC) count of 21.0 (109) cells/L), reduced blood sugar (29 mg/dL), and elevated protein level (219 mg/dL).\nA blood culture specimen was drawn from his CSF. Awaiting the culture results, an empiric antibiotic therapy was initiated with an intravenous dose of vancomycin and piperacillin-tazobactam. However, he did not respond to this regimen. The second (a day after) CSF result showed that a glucose concentration of 32 mg/dL, a protein level of 260 mg/dL, and a WBC cell count of 20.0 (109) cells/L). The gram staining of the CSF had also shown no organisms. Seven days after surgery, blood culture revealed no growth.\nHowever, the clinical condition of the patient still did not improve. The vancomycin and piperacillin-tazobactam regimen was changed to cefepime and gentamycin for 7 days. Later, those regimens were also switched to intravenous ciprofloxacin and metronidazole. Despite taking these medications; his fever still persisted and a poor clinical response was observed. Thus, the CSF analysis was repeated on the 18th day of illness and revealed a glucose concentration of 21 mg/dL, a protein level of 200 mg/dL, and a total WBC cell count of 24.0 (109) cells/L). The CSF smear for Gram statin did not reveal any bacteria. However, the patient still had fever; low consciousness level, a low glucose level, and an elevated protein level were noted.\nOn the 25th day of admission, along with the ventriculoperitoneal shunt infection the MRI showed evidence for ventriculitis, including intraventricular debris in the ventricles on diffusion-weighted imaging and abnormal periventricular intensities on fluid-attenuated inversion recovery imaging (Fig. ). Examination of his CSF revealed a persistent elevation of his white blood cell count and reduction in glucose level. This was confirmed with one CSF culture along with a positive Gram stain, which was generated by A. baumannii. Although the shunt system was removed immediately and antibiotic treatment was initiated, the infection persisted despite intravenous administration of antibiotics including vancomycin and piperacillin-tazobactam, cefepime with gentamycin, followed by ciprofloxacin with metronidazole. The bacteria was resistant to all the antibiotics examined in the laboratory by disk diffusion susceptibility test, including β-lactamase inhibitor penicillins, carbapenems, cephalosporins, fluoroquinolones, and aminoglycosides. However, sensitivity to colistin, aztreonam and tigecycline was not tested because of it was unavailable in our hospital.\nLater, with the organism identified; the intravenous antibiotic therapy was continued with ampicillin-sulbactam 1.3 g every 6 h. For CSF isolates of A. baumannii, the minimum inhibitory concentration (MIC) of ampicillin-sulbactam was 4 μg/mL (details of each antibiotic MIC is stated in Additional file : Tables S1 and S2). Our patient’s fever begun to subside and he became afebrile on the 3rd day of the systemic therapy with ampicillin-sulbactam. The CSF culture was performed and was negative on the 10th day of this systemic therapy, and the CSF cell count had reduced to 15.0 (109) cells/L). On the 14th day of the therapy with ampicillin-sulbactam, the CSF culture was completely sterile, the CSF cell count had also dropped to 9.0 (109) cells/L), and the therapy was discontinued. The gradual improvement and clinical outcome of our patient was noted. Our patient’s level of consciousness rapidly improved. The overall condition of the child was improved oddly and progressively, he began to feed orally and became more collaborative. Three consecutive blood culture analyses were performed and revealed no microbial growth all over. Indicating that the infection had gradually subsided and had been cured.\nFurthermore, no complications or side effects were witnessed during the treatment. The clinical status of our patient was progressively and remarkably improved. Meantime, after a month the surgical team had diverted the EVD to the left side. Finally, our patient was discharged from the hospital in a stable and good condition. Our patient has been followed up for 2 months after being discharged from the pediatric ward as ambulatory. We found no evidence of recurrence of infection or any neurological defects or malfunction had occurred as of 6 months postoperatively (Fig. ). The patient is now able to walk without mobility aids, eat meals by himself, and live independently at home. |
In 2013, a 42-year-old female teacher with a body mass index of >30 was referred to the neurosurgical clinic by her neurologist, who was seeing her for symptoms related to IIH, to consider CSF diversion for medically refractive visual symptoms. The patient had bilateral papilledema and was complaining of frontal headache behind both eyes, sensitive to coughing, persistent nausea and vomiting, general lethargy, imbalance, poor concentration, and visual blurring. On examination, hemorrhage, hydrocephalus, space-occupying lesions, and venous sinus thrombosis were ruled out by imaging of the brain. Her neurological examination was normal with regard to limbs and cranial nerves. Assessment of the optic system showed normal color vision and visual acuity but infranasal field loss on the right and bilateral papilledema. A lumbar puncture (LP) was performed with an opening pressure was 33 mm H2O, and her symptoms improved significantly.\nIn her past medical history, the patient had been suffering from headaches since her first pregnancy in 2000 and since 2006 had been managed by a neurologist. In 2007, she had already been referred to a neurosurgeon since a left posterior frontal arachnoid cyst had been found on imaging of the brain. Although it was thought at the time that the cyst would be unlikely to be the cause of her headaches, a burr hole marsupialization was undertaken in 2008 which resulted in several months of good pain relief. Following on from this, the patient underwent a mini-craniotomy with further marsupialization of the cyst and obtained good symptom relief for further 6 months. Then, however, the symptoms reoccurred over the course of the next 2 years. With the cyst having been treated, the diagnosis of cluster headaches (responding well- to high-flow oxygen therapy) was made, and the patient attended a residential pain management programme in 2010 with good response.\nFurthermore, in 2012, the patient was involved in a camping gas canister explosion during which she was thrown 8 feet, landed on her back, and hit her head. Although she recovered well from her minor external injuries, her neurological problems in the way of headaches, nausea and persistent vomiting, and dizziness were exacerbated again. LPs repeatedly showed increased opening pressure. Finally, a diagnosis of IIH was made and acetazolamide commenced, but the patient developed poor concentration and a skin rash and acetazolamide had to be stopped. Even with topiramate and furosemide, the symptoms persisted and control was only possible with repeated LPs, all of which showed increased opening pressure. Eventually, the patient was referred back to neurosurgery for consideration of CSF diversion, where she presented with the signs and symptoms described above.\nFurther imaging was performed. Computed tomography (CT) venogram revealed a high-grade short-segmented stenosis at the right sinusoidal knee, a longer segmented stenosis of the distal left transverse sinus, and significant narrowing of the left sigmoid sinus close to the jugular foramen. Formal digital subtraction angiogram (DSA) and further superselective spinal angiography showed dorsal medullary early venous shunting in the form of a right-sided AVF at the level of C4 fed by the radiculomeningeal artery at C4/5 [].\nThe dorsal medullary vein drained into the right transverse sinus, likely causing venous hypertension []. Due to narrow vessel diameter, it was not possible to embolize this AVF endovascularly. The fistula was surgically divided in 2013 by means of a cervical C4/5 laminectomy and excision of communicating blood vessels. Initially, this resulted in some resolution of the symptoms with the improvement of the field defect and complete disappearance of the papilledema.\nPostoperatively, the patient's headache, nausea and vomiting improved considerably as did the visual fields.\nOn 2-year follow-up, the patient's neurological improvement is maintained. |
This is a story of a 12-year-old female from rural Nepal who developed rapidly progressive generalized rash along with mild fever and headache. She was diagnosed with seizure disorder 2 weeks prior to the current illness and was prescribed carbamazepine at the dose of 200 mg daily. On the 14th day of carbamazepine, she developed those rashes which were purpuric, some of which later turned to blisters, dusky colored, itchy to start with which later were tender, and covered almost 40% of body surface area covering trunk and face with involvement of oral mucosa and eye. It was associated with swelling of the face and mild fever.\nAs the whole country was under lockdown due to COVID-19 pandemic, it was impossible for the rural patient to visit the dermatologist. For this, she went to a nearby community health center, from where mobile teleconsultation was made with the help of a community health worker (health assistant).\nOn assisted physical examination with the help of the health worker, it was found that the patient was febrile with areas of blistering and peeling of the skin involving face, trunk, and extremities with crusting seen over lips and eyes (Figures and ). The Nikolsky sign was positive. The erythematous rash was covered almost all over the trunk with epidermal detachment of 40% body surface area.\nThe clinical impression of toxic epidermal necrolysis due to carbamazepine was made after taking detail history and serial clinical findings in video calls and photos sent in the mobile device.\nRegarding the management of the patient in rural setting, carbamazepine was stopped immediately. She was supplemented with a rapidly tapering dose of intravenous hydrocortisone along with fexofenadine, mupirocin ointment, and moisturizer. The erosions were smeared with mupirocin and paraffin gauges. Supportive treatment given included parenteral analgesics for pain management and intravenous fluids. The patient was advised for better nutrition for healing of the wound by giving adequate carbohydrate, high-protein diet, and vitamin supplements. She was switched over to oral prednisolone once the lesions started healing after five days. Later on, prednisolone was slowly tapered slowly over two weeks. The lesions healed with postinflammatory hyperpigmentation (Figures and ). |
A 67-year-old man complained of left shoulder pain for 2 weeks without any history of trauma. Bloody effusions were aspirated 5 times at a local orthopedic clinic during a 2-week period before the patient was referred to us. He had no previous history related to hemorrhagic factors. On examination, he had slightly limited range of motion, with active forward flexion to 160°, abduction to 120°, external rotation to 30°, and internal rotation to the fifth lumbar spine with the arm at the side. Passive range of motion was the same as active range of motion. Neer's impingement test and Hawkins test were positive. The greater tuberosity was seen irregular on radiography. MRI demonstrated a hematoma in the anterior portion of the subdeltoid bursa and bony erosion of the lateral acromion, similar to case 1 ().\nArthroscopy was performed to determine the cause of the hemorrhage. A hematoma was not observed in the glenohumeral joint. The intra-articular portion of the long head of the biceps tendon had completely disappeared. A bursal-side partial tear of the rotator cuff was noted. The size of the tear was 25 mm in the anteroposterior direction. Crater formation on the undersurface of the acromion and irregularity of the greater tuberosity were observed, which seemed to be kissing lesions (Figures and ). Active bleeding was seen at the exposed bone marrow of the acromion when the pressure of irrigation was lowered. The undersurface of the acromion was coagulated and the greater tuberosity was abraded to avoid impingement (). Acromioplasty was not performed in this case. The bursal-side partial tear was not repaired at that time ().\nOne month later, the patient underwent arthroscopic rotator cuff repair. The patient regained full range of motion 3 months after rotator cuff repair and returned to work as a taxi driver. At the final follow-up, 14 months after the first arthroscopy, he had no functional deficit. The UCLA score was improved from 14 points preoperatively to 35 points at the final follow-up. Hemarthrosis did not recur in the 14 months postoperatively. |
A 53-year-old right handed woman was referred to our hospital to treat progressive parkinsonism. The patient had experienced convulsions 3 years before her first visit to our hospital and was on medication (diphenylhydantoin 200 mg/day). She developed akinetic-rigid syndrome (dominant on the left side) and a shuffling gait several months after experiencing convulsions. The previous doctor prescribed L-dopa, which proved to have little effect on her condition. The patient had also demonstrated livedo reticularis of both forearms around the time she presented the akinetic-rigid syndrome. There was no personal history of venous or arterial thrombosis or miscarriage.\nOn admission, the patient was alert and cooperative, yet she appeared to be slightly depressed. Her speech was easily understandable, albeit slightly hypophonic. Upon examination, she had mild supranuclear gaze palsy showing slight difficulty in looking down. Her horizontal pursuit eye movements were slightly saccadic while the range of motion was normal. Motor power was intact, while exaggerated tendon reflexes were noted on the left side with positive jaw reflex. Babinski sign was negative on either side. Snout reflex was present, yet grasp and palmomental reflexes were negative. There was asymmetric upper limb akinesia and rigidity which was more severe on the left side than on the right. Also, dystonia of the left arm and leg was observed. The gait was slow and stiff with absent arm swing, yet her postural reflexes were relatively well preserved. The patient showed bilateral limb-kinetic apraxia which was prominent on the left side. She could barely use fingers in her right hand to show patterns such as victory sign; however, it was impossible to do so with her left hand. Miming the use of tools and habitual movements such as using a toothbrush, beckoning, and indication of getting away were also difficult for her especially with the left hand which was probably due to the limb-kinetic apraxia. Primary sensations were intact except for loss of joint position sense at her left elbow. There were no cortical sensory deficits such as agraphesthesia and astereognosis. The alien limb phenomenon, neglect syndrome, or myoclonus was not observed. Her mini mental status examination (MMSE) score was 20/30 demonstrating disorientation in time, dyscalculia, mild anterograde amnesia, and decreased digit span.\nLaboratory investigations revealed there were thrombocytopenia, slight increased serum creatinine with no proteinuria, elevated activated partial thromboplastin time (APTT), and positive anticardiolipin antibody with IgG titer 35 (positive > 9). Antinuclear antibody was present at a titer of 1 : 1280 (positive > 79), and anti-DNA antibody was also positive at a titer of 11.7 (positive > 6.0). Test result for SS-A and SS-B antibodies was negative. Complement levels were normal (). The serological abnormalities including positive anticardiolipin, antinuclear, and anti-DNA antibodies remained, as observed in subsequent tests performed after 3 months. Anticardiolipin antibody was detected in two different times; APS diagnosis was therefore performed. In addition, the present case fulfilled the American College of Rheumatology (ACR) criteria for diagnosing systemic lupus erythematosus (SLE) []; she manifested neurological symptoms (convulsions and dementia), and her laboratory data showed hematological involvement (thrombocytopenia), immunological disorder (positive anticardiolipin antibody) and positive antinuclear and anti-DNA antibodies. Brain MRI showed subcortical white matter ischemic changes, marked cortical atrophy that was prominent in the bilateral central areas, and several small infarcts in deep white matter of both hemispheres (). Single photon emission tomography using technetium-99m-L, L-ethyl cysteinate dimer (99mTc-ECD SPECT) showed decreased cerebral blood flow in the bilateral central areas, which extended to the frontal and parietal areas ().\nA diagnosis of CBS due to APS secondary to SLE was made, and aspirin treatment was initiated. Because the patient showed no active systemic disorder other than the neurological manifestations, no therapy was introduced due to SLE. During follow-up, diphenylhydantoin was replaced by zonisamide; however, anticardiolipin, antinuclear, and anti-DNA antibodies tested positive consistently suggesting that the SLE was not induced by diphenylhydantoin. Dabigatran etexilate (75 mg/day) was administered in addition to aspirin at a time; however, APTT prolonged over 3 times that of the contorls' and was discontinued. The patient presented left side dominant akinetic-rigid syndrome and limb-kinetic apraxia over the 8-year follow-up period, both of which changed little in terms of severity. She was ambulant until 61 years of age when she suffered from compression fracture of the 9th thoracic vertebra, which bound her to wheelchair. Repeated MRI scans performed during the follow-up period demonstrated slight progression of diffuse cortical atrophy and subcortical ischemic changes; however, no new infarction was noted. At the age of 62, she died from pneumonia at a local hospital. A postmortem study was not obtained. |
A 25-year-old female was diagnosed with focal segmental glomerulosclerosis when she was two years old. She was treated with steroids, but progressed to peritoneal dialysis (PD) at the age of three.\nSeven months later, when she was aged four, the patient was submitted to a first kidney transplantation with living related donor (mother), at another Transplantation Center. The immunosuppressive regimen consisted of cyclosporine, azathioprine, and prednisone. Due to low adherence to the immunosuppressive medications, she developed chronic rejection at the age of 16 - twelve years after transplantation - and returned to PD.\nAt the age of 20, the patient underwent a second kidney transplantation with living related donor (father). She developed biopsy-proven humoral rejection, and the kidney allograft had to be removed. The patient restarted PD. Three years and three episodes of peritonitis later, notwithstanding the fact that her peritoneal fluid effluent was cloudy and sandy, she refused to switch to hemodialysis (HD).\nAt the age of 24, the patient started losing weight associated with diffuse abdominal pain, nausea, and vomiting. She came to the Hospital Beneficência Portuguesa de São Paulo (BP) and was hospitalized with bowel obstruction. Laparoscopic biopsy of the peritoneum showed she had peritoneal sclerosis. She was suspected for EPS and prescribed therapy with prednisone 40 mg/day and tamoxifen 40 mg/day. She was switched from PD to HD.\nSix months later and aged 25, the patient was hospitalized again at BP for bowel obstruction caused by EPS. She was started on intravenous 25% sodium thiosulfate (80 mL diluted in 200 mL of 0.9% saline solution) three times a week administered in the last hour of the hemodialysis session. The patient had a good clinical response to this treatment, recovering the ability to defecate. She was discharged without gastrointestinal complaints. The patient went back to her hometown. However, the treatment was interrupted due to the unavailability of this medication in her hometown. The symptoms returned.\nFive months later, she was hospitalized again at BP with bowel obstruction. Computed tomography (CT) scans revealed diffuse thickening of the parietal and visceral peritoneum with extensive calcification involving the mesentery and bowel loops. She was also diagnosed with moderate pneumoperitoneum and ascites (). Video laparoscopy showed large amounts of purulent fluid and stenosis in the terminal ileum and cecum. The patient underwent open surgery, which found gross calcification in the intestinal cavity and altered anatomy of the small and large bowels ( e ), in addition to confirming the existence of bowel obstruction. The terminal ileum with stenosis was removed (), the ascending colon sutured, and a terminal ileostomy performed. The abdominal cavity was washed and drained. The anatomo-pathologic findings of the surgery specimen indicated the patient had fibrino-leukocytic chronic serositis coupled with fibrosis and hyalinization, associated with multiple sites with dystrophic calcification and an ileocecal valve with lipomatous hypertrophy.\nThe patient was kept on parenteral nutrition to improve nutrient uptake. She had a good clinical outcome her condition improved. The ileostomy was functional, and the patient was later switched to oral oral diet.\nThree months later, the patient had a hemorrhagic stroke and died. |
A 22-year-old primigravid female at 17 weeks’ gestation presented with chest pain refractory to sublingual nitroglycerin and an appropriate shock by her ICD. She had a history of anxiety, depression, and angiography-documented coronary vasospasm in multiple coronary territories. Prior cardiac arrest because of ventricular tachycardia that degenerated to ventricular fibrillation led to implantation of the ICD 5 years prior. Family history was negative for sudden cardiac death.\nThe patient was chronically maintained on verapamil 120 mg 3 times daily, isosorbide mononitrate 60 mg daily, and magnesium supplementation. She had a history of 7 ICD shocks, the last almost 4 years prior to this presentation. Starting at 9 weeks’ gestation of her pregnancy, she had had several admissions to outside facilities for angina requiring intravenous (IV) nitroglycerin administration, but those events were self-limited and no arrhythmia was noted.\nElectrocardiogram (ECG) on admission showed antero-septal ST segment elevation (). Fetal ultrasound showed twin pregnancy with only one surviving fetus on admission. Echocardiogram showed mildly depressed left ventricular function with septal hypokinesis. The patient was admitted to the intensive care unit, and IV nitroglycerin was started. ECG changes and the patient's chest pain resolved. The night after admission, however, she felt crushing chest pain and had 6 appropriate shocks by her ICD for ventricular fibrillation. She was intubated, sedated on propofol and fentanyl continuous IV infusions, and continued on IV nitroglycerin to 500 mcg/min and verapamil 120 mg 3 times daily.\nMultidisciplinary discussion involving electrophysiology, maternal-fetal medicine, cardiology, and interventional cardiology determined that given the patient's history, vasospastic angina was likely the cause of the arrhythmia, and alternative diagnoses such as spontaneous coronary artery dissection were less likely. Subsequent ECGs during her hospitalization showed ST segment elevation in different territories, further solidifying the diagnosis ( and ).\nDespite these initial measures, the patient continued to have angina with refractory ventricular tachycardia and ventricular fibrillation. In addition to high-dose intravenous nitroglycerin 500 mcg/min, dihydropyridine and nondihydropyridine calcium channel blockers (nifedipine 90 mg daily and verapamil 240 mg 3 times daily), and strict electrolyte control, the patient was treated with benzodiazepines (scheduled diazepam 7 mg 3 times daily with lorazepam as needed), a beta blocker (metoprolol tartrate 25 mg 3 times daily), and sedation with intubation to attempt to control her coronary vasospasm. Psychiatry was consulted to help with control of her anxiety. Despite these treatments, she continued to have refractory tachyarrhythmias, leading to multiple appropriate shocks by her ICD. After discussion with thoracic surgery and anesthesiology, chemical sympathectomy was performed with ropivacaine 4 mg/hr via a stellate ganglion catheter. This treatment initially improved the patient's angina; however, at 24-weeks’ gestation she again developed refractory ventricular tachycardia. In total, she had more than 20 shocks by her ICD by this point in her pregnancy.\nDiscussion with the patient, her family, maternal-fetal medicine, obstetrics, critical care, and cardiology concluded that the best option for the patient and the baby was to proceed with cesarean section and delivery of the baby, which occurred at 24 weeks, 6 days. With consent of the patient and family, permanent sterilization via bilateral tubal ligation was performed.\nAfter delivery, the patient was able to be rapidly weaned from all IV medications and the chemical sympathectomy catheter. She was discharged home on postoperative day 4 asymptomatic and on the following oral medications: alpraz-olam 1 mg every 6 hours as needed for anxiety, metoprolol tartrate 12.5 mg twice daily, isosorbide mononitrate 30 mg daily, magnesium oxide 400 mg daily, nitroglycerin 0.4 mg sublingual as needed for chest pain, oxycodone-acetaminophen 10/325 every 6 hours as needed for severe pain, sertraline 50 mg daily, and verapamil 80 mg 3 times daily. Her baby did not survive. The infant died in the neonatal intensive care unit more than a month later. |
A 44-year-old female presented with vaginal bleeding along with exertional chest discomfort. Vitals signs at the time of presentation were insignificant except for pulse 101 beats per minute with the normal electrocardiogram. However, she had no any past history of cardiovascular disease. Gynecological evaluations revealed cervical cancer (squamous cell carcinoma, stage IVB) which instigated further investigations and evaluations. Whole body positron-emitting tomography computed tomography (PET-CT) () revealed right ventricular mass possibly malignancy or tumor thrombus with enlarged lymph node in left inguinal area and retroperitoneum without other distant metastasis. For further evaluation of tachycardia and dyspnea occurred a week before visiting the hospital, transthoracic echocardiography () and pro B-type natriuretic peptide measurement were performed. Pro B-type natriuretic peptide was within the normal range but transthoracic echocardiography revealed right ventricular mass doubtful of malignant mass or tumor thrombus. Hence, she was referred to Department of cardio-thoracic surgery for further evaluation and management. She underwent total bypass surgery and removal of the mass. Histopathology revealed the mass to be a metastatic lesion from the cervical squamous carcinoma. Postoperatively, the patient received 175 mg/m2 paclitaxel (Genexol, Samyang, Seoul, Korea) and 50 mg/m2 of cisplatin for 12 cycles with 3 weeks apart. Until 9th cycle of chemotherapy, there was no relapse of disease which was confirmed by abdomen CT. She then underwent additional 3 cycles of chemotherapy as laboratory test and serum squamous cell carcinoma antigen level (5.2 ng/mL, normal <12.5 ng/mL) reported normal. A contrast enhanced CT was done after additional 3 cycles which unfortunately showed suspicious of newly metastatic lesion in the bladder. She tolerated the chemotherapy well till the 7th cycle only when she started complaining dyspnea on mild exertion. However, echocardiography was normal. Two months after completing 12th cycle, she started to suffer from the dyspnea again. CT scan of chest was performed, which confirmed the increased extent of focal filling defect (thromboembolism) in right pulmonary artery. Echocardiography showed newly developed severe pulmonary hypertension with right ventricular dysfunction. She was then treated with the intravenous heparin because of which she developed severe per vaginal bleeding. Unfortunately, she died of pulmonary embolism fourteen months and 4 weeks from the diagnosis of cardiac metastasis. |
We now present the case of a 75-year-old right hand dominant woman who fell down the stairs in her home and impacted her left hand in extension against a wall. She was found to have a left intra-articular distal radius fracture with greater than 25 degrees of dorsal tilt. She initially underwent closed reduction and splinting; however, subsequent imaging 1 week later revealed loss of reduction. Therefore, we took her to the operating room 2 weeks after the initial injury and performed open reduction and internal fixation with a second-generation, low-profile, fixed-angle, volar locking plate (Acumed, Hillsboro, Oregon). The pronator quadratus muscle was restored to its native position and provided coverage of the entire plate. The patient did well postoperatively and achieved union and full recovery of wrist and hand function.\nTwo and half a years later, the patient returned with a 2-month history of volar radial-sided wrist pain. She also complained of a rubbing sensation in this area with thumb motion. On examination, there was palpable crepitus with thumb interphalangeal joint flexion suggesting irritation of the flexor policis longus tendon. The patient was able to actively fire her FPL (Fig ). The patient was taken for surgical exploration, which revealed mature union of the fracture. The plate was found to be in good contact with bone with no prominent edges (Fig ). However, an obvious defect in the previously intact pronator quadratus was noted, where it appeared as if the plate had eroded through the muscle. The repair of the connective tissue at the edges of the muscle to the surrounding periosteum/brachioradialis radially and joint capsule distally appeared intact. There was clear demarcation between the intact more proximal muscle fibers and the more distal ruptured area of the muscle (Fig ). Irritation of the flexor pollicis longus tendon was identified in this location. There was a partial thickness laceration comprising approximately 20% of the tendon substance (Fig ). This was repaired using 5-0 polypropylene epitendinous sutures. The plate was removed and the remaining intact pronator quadratus was again laid back in its native position.\nAt final follow-up examination postoperatively, the patient reported complete resolution of her previous volar radial-sided wrist pain. On examination, she had good motion of her thumb interphalangeal joint with no palpable crepitus. |
The patient was a 12 year old boy, only child of consanguineous parents, reported to the department of pedodontics and preventive dentistry with the complaint of small teeth. Parents noted small teeth ever since the eruption of permanent teeth. No abnormalities were reported, however, in their extended family.\nPhysical growth was within normal limits. The patient was of normal in stature, appearance, height, and weight for his age. Upon examination of the limbs, hands, skin, hair, nails and eyes were all appeared normal. No abnormality was noted in neck, back, muscles, cranium and joints as well. Intellectual and scholastic performance was also normal. His medical history was unremarkable; no other abnormalities were noted in the history apart from the difficult delivery. The child was examined and found to be free of any gross abnormalities.\nHis blood profile was normal. Serum calcium, phosphorous and alkaline phosphatase levels were also normal. Endocrinological investigation was carried out to rule in or out the possibility of hormonal disorder, and the results were within normal limits.\nThe intraoral soft tissues were healthy, but the teeth were abnormal in size and shape (Figure and ). Diagnostic casts were obtained to aid in diagnosis (Figure ). Patient was in permanent dentition, teeth present were small in size. The patient had normal occlusion with excessive spacing between the teeth. Fully erupted mandibular mesiodens was present between the central incisors.\nThe anterior teeth lacked normal size in all dimensions. Most of the anterior teeth were "peg-shaped" without the typical variation in mesiodistal and labiolingual dimensions. Almost all the maxillary anterior teeth did not have lingual pits whereas mandibular central and lateral incisors had prominent pits on the lingual surfaces. The posterior teeth were also small and exhibited a short occlusogingival dimension. Overall, the dentition was smaller than that of the average adult (Table and ). Orthopantomogram or the Intra oral periapical radigraph could not be taken because the patient was not able to afford.\nThe simultaneous presence of microdontia and supernumery teeth is been reported in the Cleidocranial dysplasia, Craniometadiaphyseal dysplasia, Dermoodontodysplasia, Hypodontia and nail dysgenesis, Orofaciodigital syndrome type 3 and Tricho-rhino-phalangeal syndrome type 1. However in this case, except for the dental abnormality in the form of generalized microdontia and the presence of fully erupted mandibular mesiodens between the central incisors were found and no other clinical features observed, therefore all the syndrome associated with the simultaneous presence of microdontia and supernumery teeth were ruled out along with Taurodontism, microdontia, and dens invaginatus as well as Distal symphalangism, hypoplastic carpal bones, microdontia, dental pulp stones, narrowed zygomatic arch (Table ).\nA diagnosis of non-syndromic occurance of true generalized microdontia with mandibular mesiodens was made as no systemic condition was observed. The fully erupted mandibular mesiodens was extracted under local anesthesia in order to correct midline and to facilitate the orthodontic treatment. |
A 67-year-old man was admitted to the intensive care unit (ICU) due to a second exacerbation of COPD in two weeks. He came from a first aid unit, had already been through orotracheal intubation, and was receiving mechanical ventilation and antibiotics. The patient was a heavy smoker (120 pack-years of tobacco exposure) and started to claim dyspnea to great efforts over the previous few months; he had also lost 8 kg in less than a month. There was no other known comorbid condition in his clinical history. After some days of stabilization in the ICU, the patient was referred to the general internal medicine unit, where another exacerbation took place, along with fever and productive cough, likewise controlled with bronchodilators and antibiotics.\nOn physical examination, multiple seborrheic keratoses on the back of the hands (), elbows, and trunk were observed. They were asymptomatic and have appeared in the previous four months. These lesions alerted to the possibility of a paraneoplastic manifestation, hypothesis also considered by the dermatologists who followed the case.\nThe search for an occult neoplasia was then initiated. The investigation comprehended thorax CT-scan, upper gastrointestinal endoscopy, colonoscopy, and blood analysis, which pointed no malignancy. Colonoscopy showed four sessile polyps, one in the ascending colon (measuring 10 mm), histopathologically defined as a tubulovillous adenoma with high-grade dysplasia, and three in the transverse colon (the biggest measuring 9 mm), corresponding to tubular adenomas with high-grade dysplasia. Ultimately, abdomen CT-scan revealed one irregular-shaped vegetation on the bladder floor, measuring 22 × 18 × 16 mm, with no apparent adipose tissue invasion (). The patient denied previous hematuria or any other urinary symptom.\nDue to an incomplete stabilization of the pulmonary condition, the patient was discharged and returned after a month for cystoscopy and transurethral resection of the bladder tumor. Histopathological examination of the removed tissue confirmed a noninvasive low grade papillary urothelial carcinoma. Control cystoscopy did not show any residual mass. Adjuvant therapy with intravesical BCG administration was then commenced.\nNine months after the start of the treatment, the patient still presented residual lesions of seborrheic keratoses, which are then pruriginous (). |
K, 11-year-old, Hindu male child, studying in Class 5, belonging to a middle socioeconomic status, resident of Delhi, living in a joint family, presented in the Child and Adolescent Psychiatry OPD, Department of Psychiatry, PGIMER, Dr. RML Hospital, New Delhi, with chief complaints of episodic “rage attacks” from last 1 year. He was referred by the school authority for detailed psychiatric evaluation.\nThe patient was apparently asymptomatic 1 year back and was well adjusted to his personal and social life when he showed a “rage attack” lasting for not more than 5 min. The episode was sudden in onset without any precipitating or aggravating factor. During the episodes, he suddenly became very violent and started hitting others and breaking things which were within his reach. He would shout and use abusive languages. The violent behavior was unprovoked and nondirectional. There were no apparent reasons for such behavior as reported by family members. During such episodes, the patient would not respond to any verbal command and would keep on moving around in an abnormal way. It would be difficult to control him and lot of force was needed to control him. He would calm down by himself after around 5 min. He also did not have any memory for any of the episodes. When asked, he would say that he never did any such thing and could not give a reason for such behavior. Following the episodes, he would feel exhausted and would complain about numbness in his limbs. He would prefer to lie down and take rest for 15–20 min following the episodes. In the beginning, the episodes used to occur at a frequency of 2–3/months. As the illness progressed, the number of episodes increased to 2–3/week in last 3 months. Because of such episodes of sudden rage in school, he was suspended from school 1 month back. During the interepisodic period, the patient was totally asymptomatic.\nPast history, family history, premorbid temperament, and personal history was noncontributory, and there were no other symptoms suggestive of generalized tonic-clonic seizure. Detailed child psychiatry assessment and neurological examinations did not reveal any abnormality. Detailed routine blood and urine investigations including thyroid and other endocrine functions were within normal limits. Patient's old treatment records showed that patient was earlier diagnosed as psychosis NOS, bipolar disorder, and dissociative disorder by three different clinicians. The patient was also prescribed olanzapine 10 mg for 2 months, but there was no response. The patient also underwent counseling sessions by psychologists, but the episodes of “rage attacks” increased over a period. No significant psychopathology could be elicited in his mental status examination. On the basis of history, mental status examination, neurological examination, psychological evaluation, and appropriate laboratory tests, a differential diagnosis of intermittent explosive disorder, dissociative disorder, and partial seizures was kept. MRI brain was within normal limits and EEG showed spike and slow wave discharges in bilateral frontotemporal leads. On the basis of EEG changes, our diagnosis was revised to complex partial seizure. The patient was started on sodium valproate, and dose was gradually increased to 600 mg/day over a period of next 4 weeks as per her body weight. There was a significant reduction in his “rage attacks.” He is currently well adjusted in his personal and social life. |
A 27-year-old Indian woman presented to our hospital with a very similar history and findings to that of case report 1, although her swellings were not as large. She presented with progressively increasing vulval swellings over a period of four years. She had a past history of fever, night sweats, weight and appetite loss, and vaginal discharge. Her menses were normal. Seven years prior to presentation, she also had generalized lymph node tuberculosis with discharging cervical and inguinal sinuses, for which she had received a full course of anti-tubercular therapy. Her tuberculosis was completely cured by the anti-tubercular therapy and she did not show any evidence of a recurrence.\nHer general physical examination was normal and there was no lower limb edema. She had a 15 × 7 cm labial swelling on the left side and a 9 × 5 cm labial swelling on the right side (Figure ). The skin overlying the swellings was thickened. She had the puckered scars of healed sinuses without any palpable lymph node in the inguinal and cervical regions (Figures and ). The rest of the physical examination, including her vaginal wall, chest and abdomen, was normal.\nAll of our routine investigations, including hemoglobin, total and differential cell counts, blood urea nitrogen, Mantoux test, night blood smear, chest X-ray, ultrasound of her abdomen and pelvis, and pap smear were normal.\nShe was taken up for surgery and a wide local excision with primary closure was performed. Both of her labia majora were given a natural soft and bulging appearance. There was slight oozing in the post-operative period, but healing occurred with primary intention (i.e.the incisions that were closed with sutures healed normally). Her post-operative period was uneventful. A follow-up period of six years revealed a satisfactory recovery and, unlike case report 1, she experienced minimal discharge from the wound site and her recovery was uneventful.\nA histopathological examination of the specimen in both cases showed changes of lymphedema. The features were suggestive of non-specific inflammation. There was, however, no clear evidence of tuberculosis in the specimens (in the form of granulomas and/or acid fast bacilli), malignancy, filariasis or donovanosis(Figure ). |
We report the case of a 43-year-old woman patient with refractory epilepsy due to pain related to dysmenorrhea and anamnestic history of ovarian cancer at the age of 27 yrs. The patient came in Emergency Repart of our Hospital for a epileptic crisis and, in emergency, she was treated with Verapamil 10 mg iv, immediately after with verapamil hydrochloride 20 mg in 250 ml of saline solution iv as maintenance dose. Unfortunally only after verapamil hydrochloride 20 mg in 250 ml of saline solution iv we had the possibility to evaluate the eeg (). At the time of the first visit in Internal Medicine Department, the patient had reactivation of pain and consequently seizures for two days, with a rate of three seizures every 24 h, the so-called breaking-throught crisis. The patient was admitted in Internal Medicine Department with this oral therapy: l lamotrigine, clonazepam, levomepromazina maleato and verapamil hydrochloride 20 mg in 250 ml of saline solution iv. Early treatment modification with fans and the antiepileptic drugs gave a transient improvement of symptoms, but became ineffective in the following epileptic crisis. For this reason we administred a loading dose of lacosamide 100 mg cpr every 12 h and the treatment with verapamil has been slowly decreased until the total suspension. After 48 hours of treatment with new anti-antiepileptic lacosamide in loading dose and anti inflammatory drugs we noted a reduction in pain intensity (mean verbal numeric scale (vns) reduced from 10 to 5 and the total absence of seizures despite the total suspension of verapamil. Only add-on treatment with Lacosamide was effective to stabilize the seizures during pain due to dysmenorrhea. The patient was discharged from hospital after four days with this therapy: lamotrigina 100 mg h 8 AM, 2 PM, 8 PM, nozinan 25 mg 1 cpr at 8 PM, rivotril 15 ggtt at 8 AM, rivotril 25 ggtt at 8 PM and lacosamide 50 mg 1 cpr at 8 AM and 1 cpr at 8 PM as maintenance dose. At the time of ambulatory after fifteen days, during the visit, the patient was in stable condition and did not refer seizures or pain. We suppose that pain during dysmenorrhea could be linked to uterin fibroma or other gynecological diseases, for this reason the patient is under control for gynecological conseling because of her anamnestic history of ovarian cancer. Actually we are waiting the report of gynecological counseling. |
A 56-year-old, developmentally-delayed, male was struck by a motor vehicle. Emergency medical personnel escorted him to a local hospital. There he was found to have bilateral hemopneumothoraces. Bilateral chest tubes were placed. The medical team also intubated and pharmaceutically paralyzed the patient in preparation for transfer to our level-1 regional trauma center. During transfer, hemodynamic instability required aggressive resuscitation involving blood transfusion. Upon arrival at our institution, it was discovered that the patient had multiple injuries. He suffered a mechanically unstable pelvic fracture, multiple rib fractures, and a wrist injury involving a scaphoid fracture. Additionally, radiographic data from the outside hospital demonstrated concern for an intimal flap tear in the descending aorta. Resuscitation efforts in our SICU established hemodynamic instability. Therefore, his aortic injury was managed non-operatively with observation and cardiovascular monitoring. At that point, there was no concern for spinal injury as the total spinal CT scan did not demonstrate bony injury. Unfortunately, retrospective review would show that the upper thoracic spinal segment was only analyzed with axial images. There were no sagittal or coronal reformations. Nonetheless, the patient underwent uneventful pelvic fracture and wrist fracture fixation. During his SICU course, he developed pulmonary complications including pneumonia. This problem further caused bacteremia evidenced by positive blood cultures. Antibiotic treatment was started. A surveillance CT scan was obtained five days after admission to evaluate for changes to the aortic intimal flap. This scan, utilizing its sagittal and coronal reconstructions, demonstrated a T3-T4 subluxation indicative of a DISH hyper-extension-type fracture (Figure ). The spine team was, therefore, consulted. The nature of this unstable injury mandated surgical intervention. A posterior spinal instrumentation and fusion approach was selected. At the time of posterior spinal surgical exposure, the patient was found to have left T3-T4 facet joint infection. This was associated with hematoma surrounding a T4 transverse process fracture and a left T4 costo-transverse fracture-subluxation. Intra-operative cultures were obtained. Cultures at the time of surgery grew Haemophilus influenzae (HI) and Methicillin-sensitive Staphylococcus aureus (MSSA). These cultures were consistent with the blood cultures obtained from his episodes of bacteremia related to the lung infection. Intra-operative decision was made to proceed with fixation and fusion secondary to the need for immediate stability of the DISH fracture (Figure ). Otherwise, the surgery proceeded without complication. Post-operatively, the infectious disease specialist recommended six weeks of culture-directed antibiotics. Three weeks following the surgery, the patient was discharged to a rehabilitation facility. At that stage, he had no wound problems, symptoms or signs of infection, or spinal implant complications. The most recent follow-up imaging demonstrates well fixed spinal implants and no concern for superficial or deep peri-incisional infection. The patient also remains neurologically intact. |
A 57 year old woman was admitted to our hospital on July 18, 2017 because of tiredness and fever. The patient had a body temperature of 39 degrees without other discomfort. Physical examination indicated no positive symptoms. MRI of the epigastrium showed that there was an elliptical hyperintense signal below the pancreas on the T1WI (Figure ) and slight hyperintensity on the fat-suppression T2WI (Figure ). The internal signal was heterogeneous and a clearly bounded tumor 3.4 cm × 2.8 cm in size was observed. Marked enhancement on the arterial phase was inhomogeneous (Figure ). In addition, the venous phase and delayed phase continued to intensify (Figure -F). On account of the hypervascular mass located under the pancreas, it was highly possible that the mass was a nerve incretory tumor.\nLaboratory examinations showed that the HIV antibody was negative and the white blood cell count was 14.6 × 109/L. Tumor markers and other laboratory test results were within the normal range. The preoperative diagnosis was an retroperitoneal neuroendocrine tumor. Laparoscopic surgery was performed to remove the retroperitoneal tumor. No ascitic fluid or obvious conglutination were found. When the gastrocolic ligament was incised, the lesion was located behind and below the transverse mesocolon. Intraoperative ultrasonography indicated that the tumor had an abundant blood supply, and its right side was close to the inferior mesenteric artery and vein (Figure ). The flexible tumor was 3.0 cm × 3.0 cm in size with a clear border (Figure ). The tumor was completely removed by blunt and sharp separation for further analysis (Figure ). Rapid histopathological examination revealed that the mass was possibly retroperitoneal CD together with a follicular dendritic cell tumor, which was confirmed by subsequent postoperative pathology (Figure -F). Immunohistochemical staining showed that a portion of cells were positive for CD21 and negative for CK, a small number of cells were positive for S-100 and slightly positive for CD1a and CD23, and about five percent of cells were positive for Ki67. The patient recovered well after surgery without chemotherapy, and no relapse was found during the follow-up period. |
A 10-year-old boy presented to the pediatric emergency with bouts of massive hematemesis, passage of fresh blood per rectum, and melena for 2 h. There was a previous history of massive hematemesis 2 weeks back, which resolved spontaneously after 2 units of blood transfusion. He had a history of anorexia and weight loss over the last 1 month. There was no history of trauma, foreign body, or corrosive ingestion. On examination, the child was restless and irritable. He had marked pallor, a low volume pulse, and a pulse rate of 140 per minute and blood pressure of 100/60 mmHg. Systemic examination was unremarkable. The child was resuscitated with fluids and packed red blood cells (PRBCs). However, massive hematemesis and melena continued resulting in progressive worsening of hemodynamic status over the next few hours. Resuscitation was stepped up with transfusion of multiple PRBC units to make up for the ongoing blood loss, and the child was intubated for prevention of aspiration and put on support. The preliminary reports revealed hemoglobin of 2.5 g/dl, platelet count of 200,000/mm3, and a normal prothrombin time. Upper GI endoscopy showed active esophageal bleeding and a pale esophageal mucosa with an ulcerated lesion of around 1 cm in diameter with overturned margins and a clot over it, in mid-esophagus at 25 cm from the incisors. Rest of the esophagus was unremarkable. Hemostatic procedures in the form of sclerotherapy around the lesion were tried, but re-bleeding occurred during the procedure. Child was shifted to pediatric intensive care unit, where resuscitative measures were continued and ionotropes were started. Imaging with contrast enhanced computed tomography (CT) scan thorax showed multifocal parenchymal consolidation and marked soft tissue thickening in mediastinal planes with esophageal mural and periesophageal thickening. The intravenously administered contrast was seen filling the esophageal lumen with a linear track extending from medial aortic border to the esophagus []. Based on clinical, endoscopic, and imaging findings, a diagnosis of AEF was made and the child was taken up for emergency thoracotomy. On exploration by left thoracotomy, mediastinum was studded with multiple necrotic nodes and inflammatory soft tissue. Mid esophagus and the aorta were encased in dense inflammatory tissue preventing separation of the esophagus from the aorta. Therefore, the aorta was transected between clamps to approach the AEF [], which was then dissected off the posterior wall of the aorta. Damaged segment of the aorta was resected and continuity restored using Dacron graft within 20 minutes without cardiopulmonary bypass. A partial esophagectomy of the densely inflamed esophagus, closure of distal esophageal stump, and cervical esophagostomy was performed. A gastrostomy and feeding jejunostomy were also placed. In the post-operative period, the inotropes were gradually tapered and stopped, and the child was weaned of the ventilator over the next few days. The histopathological examination of lymph nodes, aortic wall, and esophagus showed presence of necrotizing epithelioid cell granulomas compatible with tuberculosis []. The Mantoux test done in postoperative period was strongly positive (18-mm induration). There was global neurological impairment with poor recovery secondary to neurological injury as a result of massive preoperative blood loss and hypoxemia. However, the child could be discharged home on anti-tubercular drugs and enteral nutrition through the feeding jejunostomy, pending an esophageal replacement. |
A 6-year-old boy was evaluated for a 2-year history of unexplained cyanosis and dyspnea on exertion. Physical examination on admission revealed cyanosis and digital clubbing with a resting oxygen saturation of 78% on room air, mild hepatomegaly, and jaundice were also noted. Lung sounds were clear and cardiac examination showed normal heart sounds without any murmurs. Blood test results showed a hemoglobin level of 160 g/L and hematocrit of 48%. Liver function test results demonstrated mild jaundice with a total bilirubin of 41 μmol/L. Serum ammonia was elevated to 49 μmol/L (normal range 9–33 μmol/L) but no evidence of hepatic encephalopathy was found. The coagulation profile was slightly abnormal with a prothrombin time of 16.7 s, an international normalized ratio (INR) of 1.43, and activated partial thromboplastin time (APTT) of 45 s. His chest X-ray showed increased pulmonary vascular patterns. ECG was unremarkable and the echocardiography examination revealed a mild left-sided volume overload (a left ventricular end-diastolic dimension of 43 mm). A contrast echocardiography with agitated saline showed the appearance of microbubbles in the left atrium after four cardiac cycles, which suggested pulmonary arteriovenous fistula. A contrast-enhanced computed tomography (CT) scan of the abdomen revealed no sign of portal vein and demonstrated an abnormal communication between the splanchnic vein and systemic venous circulation in which intestinal and splenic venous blood bypassed the liver completely and drained directly into the inferior vena cava (IVC). Cardiac catheterization was performed for further examination of the unexplained cyanosis. A superior mesenteric arteriography, obtained in the venous phase, confirmed an end-to-side porto-caval shunt with no intrahepatic portal vein, superior mesenteric vein joined the splenic vein and then the confertus splanchnic vein bypassed the liver and drained into the IVC (). Right cardiac catheterization showed a normal pulmonary artery pressure of 15 mmHg and selective lung angiography showed diffuse small reticular end-on vessel pattern on both lungs and immediate imaging of the left atrium, suggestive of pulmonary arteriovenous fistula (PAVF) (). Based on these findings, the patient was diagnosed with hepatopulmonary syndrome secondary to type I Abernethy malformation. |
The patient was a 53 year old Caucasian woman, without any previous major medical problems, who was recently diagnosed with synchronous bilateral breast cancers and who underwent a right modified radical mastectomy and a left total mastectomy and left axillary sentinel lymph node biopsy for a pT2, pN1, estrogen receptor positive, progesterone receptor positive, HER-2/neu negative invasive lobular carcinoma of the right breast and a pT1b, pN0, estrogen receptor positive, progesterone receptor positive, HER-2/neu negative invasive ductal carcinoma of the left breast, respectively. The patient was subsequently recommended for placement of a subcutaneous implanted port for administration of postoperative adjuvant systemic chemotherapy.\nTherefore, the patient was taken to the operating room by the surgeon for subcutaneous port placement. At the request of the patient, this procedure was done under general anesthesia. The left side was selected, as it represented the side of her earlier-stage breast cancer. A left cephalic vein cutdown approach was undertaken in the left lateral infraclavicular region, by the methodology as previously described by Povoski []. Upon creating a transverse venotomy in the anterior wall of the left cephalic vein and passing a 9.6 French single lumen silicone catheter centrally, it was noted on real-time intraoperative fluoroscopy of the thoracic region that the 9.6 French single lumen silicone catheter eventually advanced downward in a craniocaudal fashion along the left paramediastinal border. As a result of this finding, intraoperative venography (Figure ) was undertaken by the surgeon in a non-digital subtraction fashion through the 9.6 French single lumen silicone catheter and at several distances from the entry point into the left cephalic vein, as the 9.6 French single lumen silicone catheter was sequentially advanced from approximately the 8 cm mark to the 15 cm mark. A total of approximately 50 milliliters of iohexol injectable contrast (300 mg/mL) was utilized during intraoperative venography. With the tip of the 9.6 French single lumen silicone catheter first positioned in the region of the mid-portion of the left subclavian vein, but at a point at which some resistant to further advancement of the 9.6 French single lumen silicone catheter was noted, intraoperative venography performed through the 9.6 French single lumen silicone catheter (Figure ) revealed a small (3 to 4 mm) venous branch off of the left subclavian vein that was first directed horizontally for approximately 3 to 4 cm and then was re-directed cephalad in a rightward direction across the upper thorax/lower neck region. Just before the transition from the horizontal to cephalad portion of this small (3 to 4 mm) venous branch off of the left subclavian vein, a tiny (1 to 2 mm) venous tributary was seen to originate off of the small (3 to 4 mm) venous branch. This tiny (1 to 2 mm) venous tributary was noted to meander in a generalized horizontal fashion across the midline of the upper thorax region and into the contralateral right hemi-thorax region. Subsequently, after repositioning of the 9.6 French single lumen silicone catheter and overcoming the previous resistence to catheter advancement, and with the tip of the 9.6 French single lumen silicone catheter now positioned more centrally (but still horizontally) in the region of the left subclavian vein (Figure ), and then with further catheter advancement with the tip of the 9.6 French single lumen silicone catheter then positioned even more centrally in a craniocaudal fashion in the upper left paramediastinal border region (Figure ), intraoperative venography revealed the presence of a relatively large diameter craniocaudally-oriented venous structure located to the left side of the midline in the medial left hemi-thorax region in a location adjacent to the cardiomediastinal silhouette and which appeared to eventually drain into the cardiac silhouette. There was absence of visualization of an identifiable left innominate vein on intraoperative venography. This relatively large diameter craniocaudally-oriented venous structure coursing downwards on the left side of the midline in the medial left hemi-thorax region was intraoperatively suspected by the surgeon to represent a PLSVC.\nSame-day consultation with the interventional radiologist revealed a similar opinion. However, based upon the intrinsic limitations of the non-digital subtraction intraoperative venography procedure performed, an accurate assessment of the point of insertion of the PLSVC into the venous return of the heart and the anatomy of the contralateral right-sided central venous system could not be adequately determined. Therefore, subsequent standard digital subtraction venography was recommended by the interventional radiologist.\nThe subcutaneous port placement procedure was uneventfully completed by the surgeon by placing the tip of the 9.6 French single lumen silicone catheter to approximately the 15 cm mark within the recognized PLSVC and attaching the 9.6 French single lumen silicone catheter to an implantable port (Titanium Bard PowerPort, C. R. Bard, Inc., Salt Lake City, UT) and closing the port insertion surgical skin incision site that was located in the left lateral infraclavicular region.\nA subsequent posterioranterior and lateral chest x-ray (Figure ) was performed and demonstrated the implanted left-sided subcutaneous port and the attached 9.6 French single lumen silicone catheter and its course along the medial left hemi-thorax region in a location adjacent to the cardiomediastinal silhouette, consistent with PLSVC.\nIn the subsequent weeks after left-sided subcutaneous port placement, digital subtraction venography of the left-sided central venous system (by way of the left-sided subcutaneous port) (Figure ) and digital subtraction venography of the right upper extremity veins and right-sided central venous system (by way of a peripheral vein in the dorsum of the right hand) (Figure ) were both performed by the interventional radiologist within the interventional radiology suite.\nAt approximately two weeks after left-sided subcutaneous port placement, the patient underwent digital subtraction venography of the left-sided central venous system by way of the left-sided subcutaneous port (Figure ), in order to fully characterize the central venous drainage pathway of the PLSVC (i.e., the point of confluence of the PLSVC with the venous return of the heart). The left-sided subcutaneous port reservoir was accessed in a sterile fashion using an 18-gauge Huber needle. Power injections were performed at 5 mL/second of iodixanol injectable contrast (320 mg/mL), with maximum injection pressure set at 300 PSI. Digital subtraction imaging was performed at 6 frames/second during the power injection. Digital subtraction venography confirmed that the point of confluence of the PLSVC with the venous return of the heart was at the right coronary sinus and into an atrial structure within the cardiac silhouette (Figure ). Delayed digital subtraction images demonstrated that the atrial structure then drained into the right ventricle and subsequently into the pulmonary arteries (Figure ), confirming that the atrial chamber receiving the venous return from the PLSVC was indeed the right atrium. Further later delayed digital subtraction images demonstrated the pulmonary venous return and the filling of the left side of the heart and subsequent aortic outflow (Figure ). There was no evidence of early arterial filling. There was no evidence of right to left shunting on the early images, nor was there evidence of left to right shunting on the delayed images.\nApproximately four weeks later, the patient underwent digital subtraction venography of the right upper extremity veins and the right-sided central venous system by way of a peripheral vein in the dorsum of the right hand (Figure ), in order to fully characterize the right-sided peripheral and central venous anatomy. A vein in the dorsum of the right hand was accessed in a sterile fashion using an 18-gauge angiocatheter. Power injections were performed at 3 mL/second of iodixanol injectable contrast (320 mg/mL), with maximum injection pressure set at 600 PSI. Digital subtraction imaging was performed at 3 frames/second during the power injection. Digital subtraction venography demonstrated normal venous anatomy within the right forearm and right upper arm regions. The more central right-sided veins, including the right axillary vein and right subclavian vein were also normal in appearance. Incidentally, there was partial fenestration of a portion of the right subclavian vein, a commonly encountered venous entity, which is usually of no clinical significance. Her right superior vena cava (SVC) was somewhat smaller in caliber than is usually seen in someone without a co-existing PLSVC. However, her right SVC was approximately of the same size as her PLSVC that was seen on her prior venography imaging. The right SVC venous return to the heart was into the right atrium, and without venographic evidence of right-to-left shunting or left-to-right shunting. The venous flow from the right atrium was identical to that seen during the previous digital subtraction venogram of the left-sided central venous system performed by way of the left-sided subcutaneous port.\nThereafter, the patient was allowed to use her left-sided subcutaneous port for continued administration of postoperative adjuvant systemic chemotherapy, blood draws, and all necessary subsequent contrast-based imaging. The patient had no detectable problems during the utilization of her left-sided subcutaneous port and had no resultant complications. The patient's left-sided subcutaneous port was eventually removed after she completed her postoperative adjuvant systemic chemotherapy, some seven months after its original placement. |
An 88-year-old man presented with a 6 months history of progressive loss of extension at the proximal interphalangeal joints (PIPs) of the right little, ring and middle fingers. It had gradually progressed to a fixed flexion deformity at the PIP joints and an extension deformity at the distal interphalangeal (DIP) joints of these three fingers. There was no past history of trauma to the neck or hands. There were no associated urological complaints or bone pains.\nGeneral examination of the patient was unremarkable. On examination of the right hand, there was wasting of small muscles and thenar eminence of the hand. The medial three fingers were flexed at the PIP joints and extended at the DIP joints. Passive movements were not possible at these joints due to fixed deformity caused by the long-standing paralysis []. There was loss of sensation in C8 and T1 dermatomes. Rest of the upper limb was normal. Examination of the right cervical region revealed a 5 cm nontender hard, fixed swelling occupying the right posterior triangle of the neck. Lymph nodes at other sites in neck and elsewhere were not palpable.\nDigital rectal examination (DRE) revealed a hard, enlarged prostate gland. His routine biochemical and hematological workup (blood urea, creatinine, electrolytes, complete blood counts and liver function test) was within normal limits. His serum prostate-specific antigen (PSA) was 184 ng/ml. Ultrasound of the kidney, ureter, and bladder region revealed bilaterally normal kidneys and bladder and the prostate weighted 60 cc with an intravesical protrusion of 5 mm. A transrectal ultrasonography-guided 12-core biopsy of the prostate was performed which showed adenocarcinoma of the prostate with Gleason score of 7 (4 + 3) occupying >50% in nine of the twelve cores with perineural invasion (PNI). Computed tomography (CT) of the neck showed a 6 cm soft-tissue mass occupying the paravertebral region on the right side of the neck with extension into the foramina of C6, C7, and T1 vertebra along with osteoblastic metastases []. Biopsy from this mass revealed adenocarcinomatous deposits. There was no evidence of lesions in the cervical spinal cord. Contrast CT abdomen showed extraprostatic disease with multiple pelvic and extensive retroperitoneal lymph node enlargement. In view of the symptomatic bony and visceral metastases and old age, the patient underwent complete androgen blockade (CAB) with bilateral orchidectomy and bicalutamide. On follow-up, the patient was asymptomatic, but the neurological manifestation did not resolve. He developed castration-resistant carcinoma prostate at 1 year and was started on enzalutamide. However, the patient succumbed to the disease within 2 years of diagnosis. |
A 55-year-old Japanese woman was referred to our hospital because of hepatic tumors detected by abdominal ultrasonography during a screening examination. Her medical and family history was unremarkable; her occupational and residential history showed no apparent exposure to asbestos. She received no medical drugs, and neither smoked tobacco nor drank alcohol. On admission, her general status was unremarkable; her temperature was 36.2 °C and her blood pressure was 110/70 mmHg with a regular heart rate of 80/minute. A physical examination revealed no abnormal findings; neurological examinations were unremarkable. All laboratory data, including levels of the serum tumor markers carcinoembryonic antigen, cancer antigen 19-9, alpha fetoprotein, protein induced by vitamin K absence or antagonist-II, and cancer antigen 125 were within normal ranges (Table ). An abdominal enhanced CT scan revealed four hypervascular, round hepatic tumors. One tumor, which was 32 mm in diameter, was in contact with the lateral liver segment and the hepatic hilus; the other three tumors were in contact with the diaphragm and measured 7 mm in segment 4, 17 mm in segment 7, and 15 mm in segment 8 (Fig. a–d). There was no pleural effusion or ascites, and no other nodular lesions in her chest or abdomen were observed. Similar to the CT findings, magnetic resonance imaging (MRI) revealed a tumor of lower intensity than the liver on T1-weighted images, and with higher intensity than the liver on T2-weighted and diffusion-weighted images (Fig. a–c). Upper and lower gastrointestinal endoscopic examination findings were normal. We hypothesized that the tumor arose from her liver, and diagnosed our patient as having multiple hepatic hemangiomas, at first. However, imaging findings were not typical of hepatic tumor: all tumors were on the edge of the liver and the tumor margins were clear. Moreover, the tumor in segment 7 appeared continuous with the diaphragm with similar high-density contrast medium (Fig. d). We then performed CT angiography to obtain further details.\nCT findings during arterial portography showed enhancement in none of the tumors (Fig. a and b), but CT during hepatic arteriography revealed strong enhancement in the tumor in the lateral segment and that this tumor was fed by the left gastric artery. In contrast, the remaining three tumors had no enhancement and were fed by the right inferior phrenic artery (Fig. c and d). Imaging findings indicated that the liver was not the origin of these tumors. Moreover, all tumors exhibited increased uptake of 18F-fluorodeoxyglucose, with a standardized uptake value of 7.8 with positron emission tomography (PET)-CT (Fig. ). We considered these tumors to be peritoneal malignant tumors, preoperatively, and performed surgical resection. Laparotomy revealed that the tumor in the lateral segment was clearly in contact with the hepatoduodenal ligament, and that the other three tumors were on the diaphragm (Fig. a–c). We performed tumorectomy with left liver lobectomy (Fig. ) and partial diaphragmatic resection. Histopathological and immunohistochemical examinations of the resected specimens were positive for CD34, CD31, D2-40, and calretinin, which confirmed epithelioid mesothelioma (Fig. a–h). Our patient was not given any medication except a general drip infusion and prophylactic antibiotics; she was discharged 10 days after surgery without complications. CT and PET-CT revealed no recurrence 20 months after surgery without adjuvant therapy. |
A 24-year-old, ASA II, G1P0 full-term parturient presented to the Labor and Delivery for vaginal delivery. Her past medical history was significant for gestational diabetes and ACM-1.\nA diagnosis of ACM-1 was made 12 years ago when a brain MRI was performed for right-sided numbness developing following a motor vehicle collision. The patient had been entirely asymptomatic since then, and had been seen by an outside neurologist frequently for the past 10 years. She was assured by her neurologist 2 months prior that vaginal delivery and labor epidural placement would be safe, however she had not been seen by an anesthesiologist in the anesthesia preop clinic before.\nIn the 39th week of pregnancy, she presented to our hospital for rupture of membranes and uterine contractions; anesthesia was consulted for an epidural placement. During the anesthesia evaluation, it was noted that she had an exaggerated patellar reflex and a questionable left-sided Babinski; subsequently, MRI images were requested from an outside hospital. A thorough review of the brain MRI demonstrated an advanced form of ACM-1 with 1.7 cm transtonsillar herniation and a large syrinx extending down from C1 to C5. Concerned with the progression of the transtonsillar herniation during labor, an urgent neurosurgery consult was requested. Following a discussion with the patient, family, primary OB team, and the neurosurgeon, a plan for cesarean section was made per neurosurgical recommendations, including avoidance of neuraxial anesthesia and use of careful laryngeal manipulation and endotracheal intubation.\nA bolus dose of terbutaline was given to stop active contractions and the patient was brought to the operation room. The patient was placed in a left lateral uterine displacement position, and standard ASA monitors were applied. Concern regarding further progression of the herniation due to increased ICP during head and neck movement and laryngoscopy, led to an awake fiberoptic intubation was performed under generous topical anesthesia consisting of 6 ml of 1% lidocaine spray. A multimodal general anesthesia was used with the mixture of 1 MAC of desflurane, oxygen, and air and controlled ventilation. Mild hyperventilation was provided to ensure an end-tidal CO2 of 30 mmHg to prevent a possible increase in ICP. A healthy infant male was delivered with Apgar scores at 1 and 5 minutes of 9/9. After umbilical cord clamping, an infusion of mannitol 0.5 mg/kg and dexamethasone 10 mg was given.\nThe remainder of the case was without incident. Post-extubation neurological assessment was grossly normal. The patient had no complaints of headache or pain in the post-anesthesia care unit and she was advised to follow-up with the neurosurgeon later. |
A 44-year-old Japanese woman with a 6-year history of insulin-dependent diabetes mellitus and an 11-year history of central diabetes insipidus presented with a pain in the genital area worsening over 2 weeks, general fatigue, and loss of appetite. Two months earlier, patient underwent a urinary catheter insertion as a management for urinary frequency, but it was removed during the previous hospital stay, a month before her recent hospitalization, for possible urinary infection. She has had frequent hospital admissions (6 times/year) and was hospitalized 3 months before her recent admission because of edema of the pelvic area and lower limbs. The patient developed diabetes mellitus after undergoing total pancreatectomy for nesidioblastosis, a surgical procedure which involved the removal of patient’s pancreas including the spleen and gallbladder. Her sister was also diagnosed with idiopathic central diabetes insipidus; thus, a family etiology was suspected. The patient had a surgery for suspected tongue cancer 2 years ago and was also suspected of non-alcoholic steatohepatitis with episodes of hepatic encephalopathy. Although she was on multiple medications including subcutaneous insulin injections and desmopressin tablets, her glycemic and hydration status were poorly controlled.\nFour days prior to the present admission, she visited a gynecologist for her inguinal pain. No uterine tenderness or exudate was observed, and she was prescribed gentamicin and lidocaine ointments for possible local infection. She developed edema in the pelvic area with loss of appetite, and her home doctor consulted the university department 1 day before the present admission.\nUpon admission, the patient appeared weak but was alert and had low-grade fever (37.4 °C) under a regular use of acetaminophen (1500 mg/day) and diclofenac (75 mg/day). Her blood pressure was not significantly different from previous measurements (88/42 mmHg) but a sinus tachycardia (heart rate 125/min) was noted. She complained of continuous abdominal pain and tenderness in all four quadrants. No abdominal guarding or rigidity was observed, but she had severe edema in the pelvic and bilateral femoral areas without necrotic skin discoloration. Laboratory investigations revealed a white cell count of 16,310/μL with neutrophilia (90.8%), elevated C-reactive protein of 22.18 mg/dL, and no serum sodium or potassium abnormalities. Serum aspartate aminotransferase and alanine aminotransferase were elevated at 466 U/L and 148 U/L, respectively. The patient’s international normalized ratio was high (2.26), but disseminated intravascular coagulation score did not meet the criteria. The patient’s HbA1c level was 8.8%, and blood sugar at admission was 316 mg/dL. She had low serum albumin concentration (1.7 g/dL), elevated serum ammonia concentration (154 μg/dL), and elevated lactate level (10.3 mmol/L). No ketonuria was noted, but significant pyuria was observed.\nThe abdominal ultrasound was unrevealing; thus, an intravenous treatment with ceftriaxone (1 g every 8 h) was initiated empirically after obtaining the blood and urine culture samples. A CT scan performed the following morning revealed the presence of air in the soft tissue of the inguinal and pelvic areas, such as pectineal and psoas major muscles (Fig. ). Immediate infectious and surgical consultations were made, and the antibiotics were changed to meropenem (1 g every 8 h), vancomycin (1 g every 12 h), and clindamycin (600 mg every 8 h). Gram-positive cocci and gram-positive rods were found in the initial blood cultures. In the evening of hospital day 2, a surgical debridement of the extraperitoneal pelvic tissue with colonostomy was performed, and the CT image after the operation suggested a complete resection of the affected tissue. However, hypernatremia (a serum Na concentration of 160 to 170 mEq/L) ensued as the use of nasal desmopressin could not effectively control the patient’s central diabetes insipidus after the operation.\nNo bacteria could be cultured from the debridement tissues (Table ). The blood culture isolates were finally identified as Streptococcus constellatus using superoxide dismutase A sequencing and C. ramosum by 16S ribosomal DNA sequencing []. The minimum inhibitory concentrations (MICs) of various antibiotics were tested using Etest for C. ramosum [] and the broth microdilution method [] except imipenem and meropenem (Etest) for S. constellatus (Table ). These results were interpreted using the Clinical & Laboratory Standards Institute M11-A8 document [] for C. ramosum and M100-S24 document for S. constellatus. Both strains were susceptible to penicillin, meropenem, and clindamycin. Despite the continued use of susceptible antibiotics and intensive care, disseminated intravascular coagulation and pancytopenia developed, and the patient died on hospital day 8. |
A 43-year-old male patient with a previous history of arterial hypertension and cholecystectomy for gallstone disease, presented in our clinic in 1986 with renal insufficiency secondary to mesangial glomerulonephritis and without any evidence of a renal mass as analyzed by sonography. In 1992, a renal mass on the left side, compatible with renal adenocarcinoma, was detected during a surveillance annual abdominal sonography and was later confirmed by CT scan. The diameter of the lesion was 5 × 5 cm. The same year, the patient underwent left side radical nephrectomy with histopathological diagnosis of clear cell adenocarcinoma pT2pN0M0 (primary tumor localized in the kidney parenchyma without metastases in the regional lymph nodes and without distant metastases according to "tumour-node-metastasis" (TNM) classification. Mesangial glomerulonephritis in the remaining right kidney progressed to end-stage renal failure. In 2000, the patient had peritoneal dialysis and, because thoracoabdominal CT examination revealed no signs of local or distant renal tumor recurrence, the patient was recommended for renal transplantation.\nIn October 2000, he received a renal transplant from cadaver and started immunosuppressive medication. The regimen included: Prednisolone, Tacrolimus (calcineurin inhibitor) and Mycophenolate. The immunosupression was not augmented since there were no signs of rejection.\nIn December of the same year, the patient presented with spontaneous pneumothorax on the left side and underwent pleural punction and aspiration. CT scan showed disseminated pulmonary nodules (Figure ) with mediastinal adenopathy. Abdominal CT scan showed a functional transplanted right kidney without malignant lesions. There were no signs of tumor recurrence in the kidneys. Bone scintigraphy was negative for metastases. Histological analysis of biopsies of pleural parietal and pulmonary nodules from the left lung identified them as metastases from clear cell renal carcinoma. DNA microsatellite analyses using AmpFlSTR Profiler Plus PCR Amplification Kit (PE Applied Biosystems, Madrid, Spain) (Figure ) demonstrated that the metastatic lesions did not originate from the donor kidney but from the the primary renal tumor removed with the diseased kidney 8 years earlier. Importantly, these metastatic nodules appeared two months after initiation of immunosuppressive treatment. It was decided to remove the transplant and stop the immunosuppressive medication. The patient was commenced on hemodialysis. In February 2001, immunotherapy with IL-2 and IFN-alpha was initiated, which was well tolerated by the patient. In August 2001, chest CT demonstrated enlargement and progression of the pulmonary metastases. The patient presented with asthenia, anorexia, anemia and bone pain, and he was referred to the Pain Care Unit. In October 2001, one year after commencement of immunosuppressive medication, the patient died. |
A 24 year old G2P0 woman presented to our fetal center at 22 weeks and 4 days with a monochorionic diamniotic twin pregnancy where twin A was found to have a small oral mass (2 × 1.3 cm) with cystic and solid components in twin A and a large solid, vascular and pedunculated mass (9 × 7 cm) in twin B. Fetal ultrasound showed normal Doppler studies in both twins with no signs of hydrops and no evidence of twin-twin transfusion syndrome initially. Fetal MRI demonstrated no evidence of airway obstruction in Twin A, however MRI confirmed a significant intraoral component in Twin B with substantial lateral deviation of the airway in the oropharynx, normal trachoesophageal complex, and fluid present in the stomach. At 24 weeks and 4 days, the fetuses developed Stage I TTTS. Fetoscopic-guided laser photocoagulation of placental anastomoses and amnioreduction (1L) was performed with survival of both fetuses. The patient was discharged home post-operative day 3.\nDue to the anticipated complexity of patient delivery and that this was a unique case to our institution, we conducted a multidisciplinary, table-top simulation at 27 weeks gestation. This included orientation to simulation processes such as confidentiality, mutual respect, suspension of disbelief, and debriefing principles. The simulation facilitators were trained in debriefing with good judgment and PEARLS facilitation (, ). In brief, the scenarios which were discussed included (1) scheduled EXIT procedure at 32 weeks, (2) urgent EXIT procedure before 32 weeks and (3) emergent caesarian section. For each scenario and each phase of the EXIT procedure, detailed allocation of personnel and their responsibilities were clearly defined. Contingency plans for fetal airway management were delineated and necessary facilities and equipment were identified.\nA repeat fetal MRI was performed at 28 weeks, demonstrating substantial growth of oral lesions in both twins (see ). Twin A demonstrated obliteration of the fluid space between the tongue and palate with involvement of the entire floor of the mouth. The oral lesion of twin B had substantial increased with a predominantly solid components and significant vascularity. The pedunculated lesion occupied the oral cavity and floor of the mouth, extending along a pedicle to large 400 cc external component (). Both fetuses demonstrated growth restriction with greater growth restriction in twin B.\nAt 28 weeks and 6 days, twin B was noted to have intermittent, severe decelerations with increase in painful maternal contractions refractory to magnesium sulfate. The decision to perform the EXIT procedure urgently was made. In the operating room, ultrasound demonstrated both twins in a cephalic position with Twin A more anterior and inferior. Twin A was partially delivered, exposing head and bilateral upper extremities. A direct laryngoscopy was performed while on uteroplacental circulation, demonstrating a large symmetric lesion involving the floor of the mouth. Fetal airway was established with a 2.5 uncuffed endotracheal tube via Seldinger technique over a rigid bronchoscope. Twin A was subsequently delivered and taken to its designated operating room where tube placement was confirmed, tube sutured at the gums and transition to neonatology team for further evaluation and resuscitation.\nTwin B was subsequently partially delivered and noted to have a double nuchal cord. The pedunculated portion of the epignathic mass was bleeding and necrotic. This external portion was debulked using an endo-GIA stapler at the pedicle with good hemostasis. A direct laryngoscopy was attempted on placental circulation, but consistent with prenatal imaging, there was substantial involvement and distortion of the oral pharynx with inability to identify the airway. The nuchal cord was unable to be reduced with persistent fetal bradycardia therefore a blind nasal intubation was performed with clamping and division of the cord with transfer to the designated operating room for an emergency tracheostomy. A tracheotomy was performed and a 2.5 uncuffed endotracheal tube was passed into the tracheotomy. A red rubber was then passed in a retrograde fashion through the tracheotomy and out the mouth. An endotracheal tube was affixed to the red rubber and pulled antegrade via the oropharynx. The tracheotomy was repaired and the patient was subsequently transferred to the NICU. |
A 26-year-old Thai woman presented with a foreign body in her left orbit that had been retained for 8 months. Eight months previously, she had sustained a gunshot injury to her left eye. There was only a small wound on her left eyelid (Fig. ); she had normal eye movement, a normal visual field, and no pain. A plain radiograph was performed at that time; it detected a round-shaped metallic foreign body located medially in her left eye globe. She was then diagnosed as having a retained foreign body in her left orbit and she was advised to have clinical observation. However, 8 months later, she developed pain in her left eye without any sinonasal symptoms. After discussion about the risk of surgery and retention of an orbital foreign body, an ophthalmologist referred her to our department for the minimally invasive procedure option of removal of the foreign body using an endoscopic transnasal approach. On examination, the movements of her left orbit were not restricted and there was normal visual acuity (20/20) with no proptosis or chemosis. A computed tomography (CT) scan of her left orbit revealed a round-shaped metallic foreign body in the medial intraconal space, and lateral attachment of posterior ethmoid sinus, measuring 6 mm (Fig. ). A transnasal endoscopic approach, with navigator assistance (Brainlab), was used to remove the bullet. Uncinectomy and anterior-posterior ethmoidectomy were performed. The location of the intraconal metallic foreign body was confirmed with a navigator system, then part of the lamina papyracea was removed and the periorbita incision was done. The defect was enlarged and the fibrotically encapsulated bullet was found lying in the orbital fat (Fig. ). The fibrotic capsule was dissected, and the bullet was delivered through her left nostril with probes and curetted (Fig. ). No intraorbital bleeding or damage to any soft ocular structures was noted. A relative afferent pupillary defect was found in her left eye during removal of the foreign body, but it resolved and no postoperative complications were observed. |
The patient is a 12-year-old female with OI type III with a history of severe scoliosis later treated with posterior spinal fusion and extremity fractures treated surgically. At the age of 11, she underwent a posterior fossa Chiari decompression for a Chiari malformation with syringomyelia. On routine imaging the next year, the syrinx recurred concurrent with progressive basilar impression without marked signs of myelopathy. The patient's primary complaint was hiccups, though her mother noticed occasional difficulty with manual dexterity. While they noted no ataxia, the patient had not ambulated in a year.\nDuring the period of diagnosis, magnetic resonance images of the cervical spine taken 6 months apart showed an increase in the size of the cervical syringohydromyelia but unchanged basilar impression and hydrocephalus []. The basilar impression was documented by magnetic resonance imaging and computed tomography. McRae's measure was 9.5 mm, Chamberlain's was 22 mm, and McGregor's was 24 mm.\nThe surgical plan was for indirect decompression with cervical traction in preparation for occipital cervical fusion and definitive treatment of the syrinx. A full ring carbon fiber halo was applied with 12 fixation pins at 2-inch pounds each. Traction was applied at approximately 2 pounds and gradually increased to 19 pounds over the course of 1 week, with careful monitoring of neurological symptoms and pain. She had some evidence of hypoglossal nerve traction. The weight was decreased and her symptoms resolved. The halo was applied for 2 weeks; the patient returned to the operating room at this point for posterior decompression and occipitocervical fusion. At the same time, placement of a fourth ventricular catheter to alleviate the recurrent syringomyelia was performed.\nAt the start of the operation, while prone, gentle traction was applied to simulate traction and indirectly decompress her basilar impression with maintained baseline neuromonitoring. The previous posterior midline exposure was used to expose her occipital bone, foramen magnum, C1, and C2. A revision suboccipital craniectomy and decompression with the placement of a fourth ventricular-to-cervical subarachnoid catheter was performed. Preoperatively-studied vertebral anatomy could accommodate a C1–C2 transarticular screw only on the right, and a C2 isthmic pedicle screw was placed on the left with both connecting to an occipital plate with four-screw fixation [Figure –]. Bone morphogenetic protein-2 sponges and local autograft were applied to the area for fusion due to the very little calvarial periosteum. A custom halo vest was applied for 2 months after surgery and replaced with a cervical collar for 3 more months. Postoperatively, McRae's measure was 4 mm, Chamberlain's measure was 20 mm, and McGregor's measure was 22.5 mm and symptoms fully resolved. At 5-year follow-up, imaging shows stable occipital bone-C2 fusion without a change in craniocervical bony relationships [Figures and ]. However, the cervical syrinx recurred without clear symptoms due to an occluded fourth ventricular catheter revealed at the yearly examination; the patient underwent re-exploration of Chiari decompression with the revision of the fourth ventricular subarachnoid catheter and duraplasty, resolving her symptoms []. At the last outpatient visit (6 months after the second surgery), the patient was communicative. She complained about a tractable migraine without aura and status migrainosus. |
A Syrian 7-year old girl was referred to us for suspected immunodeficiency of unknown etiology in a clinical context of multiple congenital abnormalities and developmental delay. She belonged to a family of Palestinian ethnicity, characterized by a high degree of consanguinity in two consecutive generations; in particular, the parents were first cousins. An 8-year old sister and a 3-year old brother were healthy; three first cousins, also born from a consanguineous marriage, died of recurrent, severe infections before age 2, lacking any diagnosis in Syria (Fig. ).\nThe girl was born by normal vaginal delivery, after a spontaneous and uneventful pregnancy; she was full term and small for gestational age (birth body weight (BW) 2.5 kg, <3rd percentile at the 39th week of gestation). She required early admission to NICU, on day 2, for respiratory distress secondary to infective pneumonia and feeding problems with poor suckling. Thereafter, she experienced recurrent infections: lower respiratory infections with wheezing approximately every 3–4 weeks, suppurative otitis media, sinusitis, and oral candidiasis. At least three bacterial pneumonia episodes have been radiologically documented from 2013 to 2014, during the permanence of the child in Qatar, and, as a result of the recurrence of the respiratory infections, the chest MRI performed in February 2014, showed left lower lobe collapse and consolidation and chronic mild pleural effusions. No anatomical abnormalities, potentially predisposing recurrent lower respiratory tract infections were detected. During the previously mentioned time lapse, chronic sinusitis and recurrent suppurative otitis media were diagnosed, and Streptococcus pneumoniae and Haemophilus influenzae were isolated in several ear swabs. The brain MRI in February 2014 detected bilateral otomastoiditis and pansinusitis. Besides the infectious complications that required broad spectrum antibiotic treatment, the patient was presenting feeding difficulties (poor oral food intake and failure to thrive), requiring nutritional support with enteral feeding through a nasal gastric tube.\nStarting from the age of 4, the child developed 4 episodes of urosepsis, secondary to severe vesicoureteral reflux (grade IV-V) and leading to left kidney hypoplasia and scarring, as documented in the urethrocystography in November 2013 and abdomen MRI in April 2014. She also presented with primary nocturnal enuresis but not incontinence.\nPhysical examination showed generalized hyposomia (BW and stature <3rd percentile for age) and microcephaly (head circumference < <3rd percentile for age). Accordingly, the radiological bone age was significantly delayed (3 ½ years at age of 6). The facial features included triangular face, small and slightly down-slanting eyes, hypotelorism, prominent nose with wide nasal pyramid, low-set and large ears and wide mouth. Moreover, she presented with sparse, thin hairs and nail dystrophy (Fig. ).\nThe skeletal survey revealed the presence of eleven pairs of ribs and bilateral finger/toe clubbing.\nThe patient had mild developmental delay with learning difficulties, underdeveloped language skills, and cognitive impairment, partly due to lack of schooling for the frequent hospitalizations. There was no history of developmental regression. Brain MRI showed normal parenchyma and ventricular system.\nAs for the lab tests, serial full blood counts from 2013 to 2014 pointed out a condition of persistent neutropenia (ranging from 500/mm3 to 1000/mm3), with a physiologic neutrophil response to acute infections (up to 2000/mm3), anemia (Hb ranging from 9 to 10 g/dl) and mild thrombocytopenia (PLT count between 90,000 and 130,000/mm3). Pancytopenia was revealed for the first time at age of 3, due to the appearance of bruises and petechial rash (PLT 50,000/mm3, Hb 7 g/dl, leucopenia not otherwise specified).\nThe level of immunoglobulins (Ig) and Ig subclasses surprisingly revealed only mild hypo-IgG2 and -IgM (IgG 1050 mg/dl (n.v. 633–1280), IgA 353 mg/dl (n.v. 33–202), IgM 22 mg/dl (n.v. 48–207), IgG1 954 mg/dl (n.v. 377–1130), IgG2 43.3 mg/dl (n.v. 68–388), IgG3 80.1 mg/dl (n.v. 15–89), IgG4 1.2 mg/dl (n.v. 1.2-169). Nevertheless, the flow cytometry count of T- and B-lymphocytes and NK cells, showed a severe B and T helper lymphopenia, as detailed in Table . The patient had an incomplete but severe block in precursor B cell differentiation, resulting in extremely low levels of blood B cells, whereas the less pronounced reduction of T cells was mainly caused by an increase in the CD8+ T cells, probably related to chronic infections. These findings were confirmed in a second test, after recovery from acute infections; no systemic steroids were being administered at that time.\nNormal karyotype, 46,XX was detected along with normal plasma amino acids screen and oxidative burst in both monocytes and neutrophils in response to stimulants, such as phorbol myristate acetate.\nIn early 2015, the patient left Qatar back to Syria, where she died of fatal bilateral pneumonia, and her immunological work-up remained incomplete, i.e. lymphocyte T-mitogen stimulation and the response to vaccinations could not be tested. |
A 27-year-old Caucasian woman was admitted to the Emergency Department of our Institution because of bloody diarrhoea – up to 10 bowel movements per day – during the last month, 3 weeks after quitting smoking. Physical examination showed no abnormalities but confirmed haematochezia on digital rectal examination. Colonoscopy showed continuous severe colonic inflammation with small ulcers from the anus to the descendent colon, classified as grade 3 in Mayo endoscopic sub-score and 3 points in Ulcerative Colitis Endoscopic Index of Severity (UCEIS); complete examination was not performed because of the risk of perforation. Empirical antibiotic treatment with ciprofloxacin and metronidazole, as well as oral and rectal mesalamine were started and partial symptomatic improvement was achieved. Venous thrombosis prophylaxis with subcutaneous enoxaparin, 40 mg per day, was started. At admittance, haemoglobin, white cell count, platelets, fibrinogen and C reactive protein (CRP) were within the reference range. Stool cultures were negative. Cytomegalovirus (CMV) infection was also ruled out in colonic biopsies (polymerase chain reaction – PCR – and, later, immunohistochemistry). As bloody diarrhoea persisted 48 h later, and histopathological examination of colonic biopsies showed crypt distortion, a mixed inflammatory infiltrate of the lamina propria and crypt abscesses suggesting the diagnosis of UC, intravenous methylprednisolone (1 mg per kg of weight, daily) was started. After 3 days of corticosteroids the patient achieved partial clinical response (6 bowel movements per day, Edinburgh index 2 points, CRP within the normal range); nevertheless, 2 weeks later infliximab therapy (5 mg/kg of weight) was started due to sustained clinical activity, with 10 bloody bowel movements per day and a progressive increase of CRP levels, up to 10 mg/dL. Three days after the first dose of infliximab, the patient presented a massive lower bleeding with haemodynamic instability and severe anaemia; CT scan showed active arterial haemorrhage from ascendant colon; a subsequent arteriography demonstrated active arterial bleeding from a colic branch of the superior mesenteric artery; selective transcatheter embolization with platinum microcoils (MicroNester©, Cook Medical) was performed with immediate technical success; nevertheless, the patient persisted with rectal bleeding 2 days after embolization, requiring laparoscopic subtotal colectomy and ileostomy. Pathological evaluation of the colon confirmed the diagnosis of UC. Eight days after surgery the patient was discharged. |
In this case, we noted a 14-month-old girl who presented with a preliminary diagnosis of hepatosplenomegaly. Upon review of her medical history, there were no pathological findings other than that a hepatosplenomegaly was present at the physical examination. Subsequently at the laboratory examination, hepatic enzymes, alpha-fetoprotein, alpha-1 antitrypsin, liver autoantibodies and coagulation parameters were normal, while the thrombocytopenia was observed at complete blood count (82×109/L). The portal vein Doppler ultrasonography revealed an incidence of hepatosplenomegaly and AHL, which is in the same echogenicity of the liver and approximately 5.5 cm in size extending to the right upper pole of the kidney, as noted with separate portal and hepatic veins. The main portal vein diameter was measured at 15 mm (normal lower limit <13 mm), and the flow rate is 30 cm/second (normal range 13–23 cm/second) []. Upon review of the magnetic resonance imaging of the liver, it was revealed that the AHL is 56×55 mm size extending inferiorly from the segment 5–6 level, with a separate portal and hepatic veins, and was noted to be compressing the right upper pole of the kidney on T2-weighted coronal image (). The endoscopic examination revealed grade 2 esophagial varices with three strands, and a notation that the varices were more evident when the patient lay on the left side during the endoscopy. The observed red spots were not visible during the review (). The patient was started on a program of propranolol (1 µg/kg/day) and followed with an endoscopic examination with a six months interval. During the follow-up, the patient underwent endoscopy and the grade of varices were noted as stable upon follow up. The noted thrombocyte levels were 104×109/L. The surgical treatment was planned in the case of a progression of varices grade or with the incidence of patient variceal bleeding.\nThe researchers have obtained a written consent form from the family in this case study. |
A 68-year-old Japanese man with a history of distal partial gastrectomy for gastric cancer 10 years earlier was admitted for surgical treatment of intrathoracic esophageal cancer (T3, N2, M0, stage III). He underwent subtotal esophagectomy with three-field lymph node dissection and removal of the remnant stomach with the abdominal lymph nodes. The alimentary continuity was reconstructed with a pedicled jejunal limb through the antethoracic route. When we separated the diaphragm from the esophagus and removed xiphoid surgically to prevent a pedicled jejunal limb injury, the pericardium was opened. Anastomosis of the esophagus and jejunum was carried out instrumentally with a circular stapler. A postoperative enteral contrast examination showed smooth passage and no deformity of the reconstructed jejunum. The patient was discharged about 4 weeks postoperatively. About 6 months after the esophagectomy, the patient was readmitted to our hospital because of abdominal discomfort and vomiting. His vital signs were stable and unremarkable. He was thin, with a body mass index of 18.6 kg/m2. Physical examination revealed that the pedicled jejunum through the antethoracic space was markedly dilated and the abdomen was soft and flat (Fig. ). Laboratory data showed only leukocytosis with no other remarkable findings. A chest roentgenogram revealed an increased cardiothoracic ratio of 70%, and an enteral contrast study showed a bird’s beak deformity. The swallowed barium remained static in the reconstructed jejunum. Computed tomography of the thoracoabdominal region showed the reconstructed jejunum within the pericardium anterior to the heart (Fig. ). We diagnosed the patient with IPDH after esophagectomy and performed an emergency laparotomy. Upon opening the abdominal cavity, we found that the reconstructed jejunum had herniated into the pericardium through an approximately 4-cm-diameter diaphragmatic defect without a hernia sac (Fig. ). The herniation was easily reduced, and the congestion of the incarcerated jejunum resolved. There was no evidence of bowel ischemia. Primary closure of the diaphragmatic defect was accomplished using vertical mattress sutures with 1–0 silk (Fig. ). Because the diaphragm adhered to the pericardium around the diaphragmatic defect, we closed these together. Moreover, to reinforce the closure of the diaphragmatic defect, we used a graft harvested from the rectus abdominis posterior sheath. Interrupted sutures with 3–0 nylon were placed to fix the 8 × 6 cm graft of the rectus abdominis posterior sheath to the diaphragmatic defect, preventing recurrence of the hernia (Fig. ). Postoperatively, an upper gastrointestinal study confirmed free flow of contrast medium from the cervical esophagus into the intra-abdominal jejunum through the pedicled jejunum. The postoperative course was uneventful, and the patient was discharged on the seventh postoperative day. |
A 19-month-old male in his usual state of good heath presented with a three-month history of progressive non-bloody, non-bilious emesis. The mother denied history of fever, diarrhea, or abdominal pain. His growth parameters plotted above the 75th percentile for height and weight, and there was no previous history of gastroesophageal reflux symptoms. He was referred to our institution where an upper GI series (UGI) showed 13 ingested foreign bodies connected end-to-end and looped from the stomach through the pylorus into the duodenum (). His mother reported that he had received a box of plastic colored magnets as a gift several months earlier.\nHe was brought to the operating room where emergent endoscopy revealed that the most proximal, red-colored neodymium magnet in the stomach was magnetized to the anterior wall of the stomach (). Attempts to grasp the magnet with forceps were unsuccessful. The gastric foreign body had tightly adhered to the most distal, yellow-colored neodymium magnet located in the small intestine adjacent to the stomach. The forceps could not separate the magnets due to the strong magnetic attraction. A loop snare was inserted between the magnets and the proximal, red-colored magnet was tightly grasped. Significant force was applied to separate the proximal magnet from the wall of the stomach; however, this only led to the emergence of the distal, yellow- colored magnet from the small intestine through the gastric lumen (). This raised the possibility of intestinal perforation. Pediatric surgery was consulted intraoperatively. After much discussion, the decision was made to continue attempts at endoscopic removal prior to considering surgical exploration, as the patient remained clinically stable with a benign abdominal examination.\nThe proximal magnet was once again tightly grasped with the loop snare and drawn closely to the tip of the scope for better control (). In one smooth motion, the endoscope was torqued in a clockwise fashion and quickly withdrawn with some force. This separated the proximal gastric magnet from the distal small bowel magnet. The magnets were withdrawn through the mouth, magnetized to one another in linear fashion. Repeat endoscopic inspection of the area revealed a gastroduodenal fistula (), but no evidence of perforation.\nThe patient was admitted to the hospital for observation. He was given nothing by mouth and started on prophylactic intravenous antibiotics while pediatric surgery remained consulted. Follow-up 3-view abdominal films showed no evidence of perforation. He did not develop signs or symptoms of peritonitis. The patient did well during his hospitalization. His diet was gradually advanced as tolerated, and he was discharged home after three days of observation. The patient did well clinically and remained asymptomatic at his outpatient clinic visit two weeks later. Follow-up UGI six months later showed no evidence of a persistent gastroduodenal fistula.\nInformed patient consent was obtained for publication of the case details. |
A 62-year-old female of Polish origin presented with burning pain and tingling dysesthesias in both hands and feet as well as progressive numbness in the distal upper and lower extremities that she had first experienced 5 years prior. Over the course of disease, she had developed a severe gait disorder, a bladder dysfunction and alternating episodes of constipation and diarrhoea, the latter resulting in considerable weight loss. Her body mass index was reduced to 18 kg/m2. The family history was negative for similar symptoms or neurologic disorders. Previously, the patient was treated with intravenous immunoglobulins assuming an underlying inflammatory neuropathy, as NCS results fulfilled the electrophysiological EFNS/PNS (European Federation of Neurological Societies/Peripheral Nerve Society) criteria for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The treatment had no impact on disease progression, and even an interfering somatoform disorder was suggested.\nPhysical examination revealed slight bilateral paresis of plantar flexion (MRC 4/5), a prominent atrophy of both quadricep muscles, most pronounced on the right side, and distal symmetrical hypoesthesia for touch, thermal stimuli, and vibration in the lower extremities, but pain sensation was not impaired. Tendon reflexes in both legs were decreased, and the patient presented with a mild afferent gait ataxia. The upper extremities showed no weakness and only a slight thermal hypoesthesia in both hands and dysesthesias.\nThorough NCS revealed a severe sensorimotor polyneuropathy with features of both axonal damage and demyelination. EMG recordings did not show pathological spontaneous activity but a high degree of polyphasic potentials and a neurogenic recruitment pattern of motor unit potentials. Using the Ewing test, a reduction in heart rate variability indicating autonomic neuropathy was detected. As outlined above, routine laboratory testing and investigation of cerebrospinal fluid yielded no decisive results. A rectal biopsy was negative for amyloid deposits.\nMolecular genetic analysis revealed a pathogenic mutation in exon 3 of the TTR gene, p.Glu81Lys (Glu61Lys), which was previously only reported in Japanese patients []. One of the patient’s sons tested positive for the abovementioned amyloidogenic mutation, and the other son refused genetic testing. Finally, with a delay of more than 5 years from symptom onset, treatment with tafamidis was initiated and continued for approximately 4 years. Recently, the treatment was switched to patisiran on both the patient’s wish and the physicians recommendation based on individual indications and contra-indications. Meanwhile a growing cardiomyopathy was detected. (s. Figure ). |
A 36-year-old female was scheduled for elective cesarean section with spinal anesthesia due to her history of cesarean section. Preoperative examinations did not show notable health problems. The infant had an Apgar score of eight points at 1 min and nine points at 5 min. Surgery was completed in about 60 min; however, persistent bleeding was observed from the vagina. Uterine hemorrhage was observed by transvaginal examination, and an attempt of hemostasis did not succeed. After blood loss exceeded 1500 ml, laparotomy was performed for hemostasis. Anesthesia was changed to general anesthesia, which was induced by 5 mg of midazolam, and tracheal intubation was performed after the administration of 50 mg of rocuronium. While preparing the transfusion, plasma expander was rapidly administered; however, systolic blood pressure dropped to approximately 50–60 mmHg, hemoglobin to 4.2 g/dL, and hematocrit to 13.4%. The blood loss exceeded 4000 ml; therefore; treatment was changed to total hysterectomy. On rapid transfusion of 1120 ml of packed red blood cell in approximately 15 min by syringe, systolic blood pressure increased to approximately 80–90 mmHg. Arterial blood gas analysis performed on the completion of the rapid red cell transfusion revealed that serum potassium level had increased to 8.3 mEq/L. We immediately performed the intravenous administration of calcium gluconate 2250 mg, insulin 10 units, glucose 40 g, and furosemide 40 mg. Her cardiac rhythm remained stable. The hemorrhage continued, and therefore, subsequent administration of packed red blood cells was performed using a potassium absorption filter (KPF-4 Kawasumi Laboratories, Tokyo, Japan). Thereafter the hyperkalemia gradually improved. Ultimately, there was 11497 ml of blood loss, with the administration of 2520 ml of packed red blood cell transfusion, 1920 ml of fresh frozen plasma, and 200 ml of platelet concentrate. Even though the administration of packed red blood cells was continued with the KPF, serum potassium levels were not increased again, and final potassium level was 4.3 mEq/L. Systemic management was continued in the Intensive Care Unit following the surgery, and on the day 15 postoperatively, the patient was able to walk independently and discharged without complications. Her uterus was diagnosed with placenta increta pathologically at a later date. |
A 9-year-old Chinese girl was referred to our hospital. The patient's chief complaint was the pain of the posterior part of left mandibular and found a nodule with pus discharge 1 month before her visit. The patient was in a normal growth status without short height and developmental malformation. The parents of the patient were informed of the objectives of this research, and written informed consent was obtained prior to conducting the study. The study was approved by the Ethical Committee of the School of Stomatology, Fourth Military Medical University.\nPhysical examination revealed a nodule with pus discharge and swelling under the left of the chin. Gentle pressure on the surrounding tissue elicited thick purulent drainage from the central punctum (Fig. A). Intraoral examination (Fig. B–D) revealed the red and swollen alveolar mucosa in the primary left mandibular second molar (#75) region was obvious and the tooth was only partially erupted. What was more serious was that all teeth in the mixed dentition exhibited abnormal shape except the disorderly aligned bilateral permanent incisors both in maxillary and mandibular. The crowns of the primary canines and the primary maxillary first molars (#54 and 64) were enlarged, bulbous, and malformed with 2 or 3 cusps, which looked like either fusion or germination. Furthermore, the tooth #64 had level III mobility with no positive signs under percussion or palpation. The primary maxillary second molar (#55 and 65) and the primary right mandibular first molar (#84) were similarly enlarged with developmental grooves existed dividing the crowns into different sizes of lobules. In addition, there were some small tooth-like structures around the #84. The first permanent molars lacked clearly recognized cusps, resembling the tied end of a sausage. The teeth #75 and 85 were only partially erupted. According to her guardians, the patient's incisors had started to erupt in her 4-year-old age and had never been replaced, which indicated that the patient's primary incisors were congenitally absent. Her parents also confirmed that the eruption of her primary dentition was delayed compared with normal children.\nThe panoramic radiograph (Fig. E) revealed tooth-like radiopaque masses surrounded by a large radiolucent zone with a well-defined margin in the molar area of left mandibular. Radiopaque masses in the bilateral primary mandibular molar region appeared to be 4 odontoma and the germs of bilateral permanent mandibular premolars were absent. Radiologic and clinical findings were mostly consistent with a diagnosis of pericoronitis causing cutaneous sinus tracts in the left mandibular premolar region. Besides, radiographic analysis of the abnormal molars showed taurodontism changes with larger pulp chambers, some of which were duplicated, and the root length was short compared with crown height; some were taurodont in configuration. The spiral CT images (Fig. A) of both jaws were reconstructed and the craniofacial skeletons were normal.\nAccording to the otolaryngologic examination, the patient had distinct bilateral sensorineural hearing loss of acuity to frequencies above 1000 Hz. Her threshold was normal at low frequencies but obviously drop up to 75 dB at higher frequencies (Fig. F). The otolaryngologic examination confirmed her auditory structures, temporal bone, and ethmoid air cells were normal. Based on the typical dental phenotype and bilateral sensorineural, high-frequency hearing loss, the patient was clinically diagnosed as the otodental syndrome. The patient's parents were healthy and had no similar clinical signs, and neither radiographic examinations nor laboratory assays revealed any defects.\nTooth #64 and the tooth-like radiopaque mass in the #75 region were removed during surgery. Tooth #64 fused with abnormal morphology of 3 crowns and roots (Figs. B and C). During surgery, 2 teeth were found in the left mandibular region; one was globodontia with an enlarged crown, large pulp chamber, short roots, and pulp stones (Fig. D and E). The other was a tooth-like structure surrounded by a fibrous capsule like granulation tissues in the chronic inflammation condition (Fig. D and F).\nWe further used micro-computed tomography (micro-CT) to detect the inner structure of extracted #64; the findings showed the fusion of dentin and enamel defect in the grooves of cusps (Fig. A). The major root canal system was completely fused with 2 supernumerary components, and other components were incomplete, sharing part of the root canals with them (Fig. B). Cross-sectional images showed that the thickness of enamel was obviously increased (Fig. C), and there existed pulp stones in the root canals (Fig. D). In the fused regions, part of enamel invaginated into the dentin and irregular calcification could be observed (Fig. D).\nThe histologic staining of tooth #64 showed that the dentin was fused with 3 tooth-like structures and several pulp stones were existed. Moreover, we found that the necrotic tissue existed in a pulp cavity which was independent of the major root canal system. In addition, the morphology of odontoblast was in a high columnar shape with massive vacuolated changes (Fig. A–C). To our knowledge, this report was the first case of abnormal dentine–pulp complex of globodontia.\nMicroscopically, the tumor showed irregularly arranged dentin-like hard tissues with odontogenic epithelium, enamel matrix (Fig. D and E). At the periphery area, a dental follicle originated capsule could be found. The diagnosis of a complex odontoma was confirmed.\nOne month after surgery, there was a sign of healing of the extra oral lesion and in the follow-up appointment in the 3 month, the patient was found to be asymptomatic and even the evidence of the extra-oral sinus had disappeared (Fig. A). Intraoral examination (Fig. B–D) revealed the red and swollen fast wane in the #75 region and the tooth was partially erupted in the #74 region. The panoramic radiograph (Fig. E) showed that the tooth-like radiopaque masses were totally removed.\nTo further explore the causative gene, the genomic DNA was obtained from all members of the family and healthy individuals. However, DNA sequencing and analysis of deaf genes gap junction protein beta 2 (GJB2), gap junction protein beta 3 (GJB3), solute carrier family 26 member 4(PDS, or SLC26A4) had not revealed any mutation or single nucleotide polymorphism (SNP). Moreover, 3 exons of FGF3 were PCR amplified with the use of primer pairs, as previously reported.[ The exon3 of causative gene FGF3 could be amplified in the unaffected family members and unrelated control individual but not in the affected patient. Furthermore, we amplified and screened the other exons of FGF3 gene of the patient and found no mutations in coding regions by DNA sequencing analyses. The results of our limited genetic examinations indicated that the microdeletion of FGF3 Gene at chromosome 11q13.3 could exist. Further genetic studies should be performed to assess the abnormal variant of FGF3 gene. |
An 82-year-old Australian female was referred by her general practitioner to a general surgeon for biopsy +/- excision of a clinically up to 15 mm lesion of the mid-nose, which had been rapidly enlarging in size over the previous two to three weeks. The lesion was subsequently managed surgically by wide local excision and closed with an advancement flap. The histopathology report described an ulcerated tumor composed of undifferentiated small cells invading into the superficial subcutis. The cells stained strongly positive for cytokeratin-20 (CK-20), chromogranin, synaptophysin, and CAM 5.2; and stained negative for both thyroid transcription factor-1 (TTF-1) and CK-7. The immunohistochemical profile was therefore consistent with diagnosis of MCC []. In terms of the surgical margins, the peripheral margins were clear by at least 3 mm and the deep margin was seen to be clear by approximately 1.5 mm. There was neither lymphovascular space invasion nor perineural invasion seen.\nThe case was subsequently discussed at the hospital’s multidisciplinary (MDT) specialities meeting, with the consensus recommendation for discussion of postoperative radiotherapy under radiation oncology. Review of staging CT imaging three weeks postexcision showed features concerning for local recurrence just adjacent to the surgical scar site on the left side of the nasal bridge, or left nasal region of the maxilla. On review in the radiation oncology clinic, the suspicious region had a palpable nodule underneath it with firm and hard consistency, felt to be just less than 5 mm in diameter. Fluorodeoxyglucose-18 positron emission tomography (F-18 FDG PET) staging scan was subsequently organized in addition to a biopsy from this suspicious nodule.\nThe PET scan confirmed the clinical findings of loco-regional recurrence postsurgery, with a moderately glucose avid >10 mm (in short axis) subcutaneous nodule over the left anterior maxilla adjacent to the nose (as described clinically), an avid >10 mm (in short axis) enlarged left Level IB node just anterior to the left submandibular gland, and an equivocal <10 mm (in short axis) right Level IB node adjacent to the submandibular gland.\nFine needle aspiration (FNA) of the left cheek lesion showed specimen demonstrating paranuclear dot positivity for CK-20 and positivity for synaptophysin and chromogranin, and CAM5.2; TTF-1 and CK7 immunohistochemistry were again negative, consistent with MCC [].\nThe case was then further discussed at a large metropolitan centre Head and Neck MDT. By that time, the recurrent nodule on the left nasal region of the maxilla had grown to up to 3 cm with overlying erythema, and on examination there was palpable Level II cervical nodal regions bilaterally (Figure ). The MCC was therefore now consistent with T1N2M0, Stage III disease []. The consensus recommendation from the MDT panel was for definitive intent radiotherapy to the nose, to the bilateral neck, and to consider the bilateral cheeks. The patient’s ECOG status was 1.\nThe patient was treated to a total dose fractionation of 60 Gray in 30 fractions, 2 Gray per fraction, daily for six weeks, with 6 megavoltage (MV) photons, using volumetric modulated arc therapy (VMAT). A simultaneous integrated boost (SIB) technique was employed, in which the left nasal region of the maxilla and bilateral Level IB cervical nodes received 60 Gray in 30 fractions, and a total dose of 50 Gray was administered to the bilateral facial nodes, resected nasal disease (with a margin), bilateral parotids, and intervening lymphatics. A 1 cm custom tissue equivalent material (commonly referred to as bolus) was applied over the left nasal region. A mouthbite was used, and the nose was packed with gauze. A gross tumour volume (GTV) representing the recurrent nodule on the left nasal region of the maxilla was outlined; there were further expansions with a clinical tumour volume (CTV) expansion of up to approximately 5 mm and a planning target volume (PTV) expansion of up to 10 mm for the structures treated to 50 and 60 Gray as described above (Figures -).\nThe patient was also referred for a discussion of concurrent carboplatin chemotherapy under medical oncology []. Carboplatin was poorly tolerated, leading to a hospital admission, and chemotherapy was subsequently withdrawn after a single cycle. At that point, there was already noted in the clinic to be a marked reduction in the size of the lesion with radiotherapy. By the completion of radiotherapy, there was essentially complete remission clinically of the lesions of both the left nasal region of the maxilla and the bilateral Level II cervical nodes.\nBoth parotids were included in the treatment field. The mean dose to the bilateral parotid glands was between 53 and 54 Gray. Approximately 23% of the total volume of the mandible received a dose of 50 Gray, with no ‘hot spots’ (as defined by ICRU50 as a volume outside the PTV receiving dose greater than 100% of the specified PTV dose) in the mandible. Acute toxicities [defined by Common Terminology Criteria for Adverse Events (CTCAE) Version 4 as occurring within 90 days of treatment] reported by the patient included Grade I xerostomia with reduced saliva without significant dietary alteration, Grade I dermatitis with mild erythema in the treatment field, Grade I fatigue, and Grade I facial oedema with localized edema. Late toxicities, defined by CTCAE as occurring more than three months after the completion of radiotherapy, included the persisting or consequential Grade I xerostomia; and Grade I facial edema with localized edema. The relatively mild reported xerostomia by the patient both in the acute and late stages was anomalous given that there was no sparing of either parotid gland from the target volumes. However, approximately 18 months postcompletion of radiotherapy, a lower right premolar tooth was extracted by the patient’s local dentist. The cause was thought by the treating dentist to be in the context of xerostomia and dose received to the mandible.\nThe PET to assess treatment response three months postcompletion of radiotherapy demonstrated a complete metabolic response. All subsequent imaging have demonstrated complete response with no evidence of recurrence or distant disease. This has correlated with findings on clinical examination on three-six monthly reviews (Figure ). The patient remains well and has 6-12 monthly interval CT re-staging. |
The patient was a 34-year-old gravid 1 para 0 female at approximately 10 weeks gestational age by last menstrual period who presented to a community emergency department (ED) with complaints of right lower quadrant abdominal pain. The patient reported that the pain woke her up from sleep at 3 a.m. The patient arrived to the ED approximately three hours after the onset of pain. The patient described the pain as a “constant pinching” localized only in the right lower quadrant and it was unrelieved by acetaminophen. The patient denied nausea or vomiting. She also denied any vaginal bleeding or discharge.\nA physical exam revealed a temperature of 36.8 Celsius, pulse of 82 beats per minute (BPM), respiratory rate of 18 breaths per minute, and blood pressure of 125/67 mmHg. At the time of the initial examination the patient appeared comfortable and in no acute distress. Her abdominal exam revealed a soft abdomen with normal bowel sounds. No masses, distention or tenderness were detected. Pelvic exam was conducted and was noted to have no vaginal discharge or bleeding, and no pelvic masses were appreciated by the provider. However, it was noted that body habitus limited the exam.\nA formalized pelvic ultrasound (US) showed a single live intrauterine pregnancy with a fetal heart rate of 163 BPM. Large right ovarian cysts were noted and there was arterial flow noted centrally in both the left and right ovaries. The right ovary measured 7.7 cm × 4.8 cm × 5.9 cm, whereas the left ovary was only 1.9 cm × 1.4 cm × 1.6 cm.\nThe case was discussed with the on-call obstetrician who stated that it was most likely a corpus luteal cyst and the pain should resolve on its own. Since the pain had completely, resolved the patient was discharged home with scheduled follow-up in two days with obstetrics and gynecology (OB/GYN).\nThe patient had an unplanned return to the ED nine hours from time of discharge with recurrence of her abdominal pain. The patient reported that the pain was located in the same location and felt similar, although now it was much more intense and was not resolving. She was also very nauseated and actively vomiting.\nReexamination revealed a temperature of 36.1 degrees Celsius, pulse rate of 64 BPM, respiratory rate of 18 breaths per minute and a blood pressure of 133/78 mmHg. At the time of reevaluation the patient appeared in acute distress, doubled over and moaning in pain. The patient was intermittently vomiting what appeared to be gastric contents. However, her abdominal exam again showed no peritoneal signs, no focal tenderness and no masses. The patient’s abdominal examination did not match her level of distress. OB was again consulted and requested an additional formal US.\nThis time, repeat US again showed an enlarged right ovary with multiple cysts. At the time of the repeat US the right ovary measured 8.23 cm × 8.41 cm × 5 cm, whereas the left ovary was 2.22 cm × 2.94 cm × 3.22 cm. Using color Doppler, blood flow was not demonstrated in the right ovary, whereas left ovary demonstrated adequate blood flow.\nOB evaluated this patient and took her to the operating room. The patient had an exploratory laparoscopy performed, which revealed that the right infundibulopelvic (IP) ligament was twisted times three. A laparoscopic needle and syringe were used to drain two simple ovarian cysts. The right ovary was manually detorsed and healthy viable ovarian tissue returned. Postoperatively the patient was started on intravaginal progesterone 200mg for the following four weeks. At term the patient had a spontaneous vaginal delivery of a healthy girl. |
In June 1986, a 28-year old man sustained hemopneumothorax due to 3rd-6th costal fractures and brachial plexus injury in the left side during a motor cycle accident. He was referred to our clinic 2 months after initial trauma due to there has been no spontaneous recovery of the upper limb function. Upon physical examination, a positive Claude Bernard-Horner's sign was found. The function of the shoulder, elbow, wrist and hand was completely lost. Plain chest X-ray film also showed elevation of the left diaphragm. Electromyogram detected no SEP from C5-T1 nerve roots and NAP could be recorded. The diagnosis was preganglionic injury of the C5-T1 nerve roots, ie., total root avulsion injury of the brachial plexus. The EMG exam also suggested concomitant complete palsy of the accessory nerve and phrenic nerve. Based on our observation of over 1000 cases of brachial plexus injuries, no patient suffered functional loss from single C7 root injury, therefore we postulated that C7from the healthy limb may be sacrificed and used as a donor nerve to reconstruct the injured plexus. During surgical exploration of the affected plexus, C5-T1 nerve roots were found avulsed. Due to extensive scarring in the neck/shoulder region, the accessory nerve and motor branches of the cervical plexus could not be used. The phrenic nerve was found to be buried in the scarry tissue on the surface of the fibrosed anterior scalenus muscle and a neurolysis was performed.Upon electrical stimulation a strong contraction of the diaphragm could be induced which indicated the viability of phrenic nerve. It was then divided with its proximal stump transferred directly to the anterior division of the upper trunk (fascicles destined to the musculocutaneous nerve). Since there were no further donor nerves to be used in the injured side, the contralateral C7 nerve was decided to be used and therefore exposed and divided. In the injured side, the ulnar nerve was freed from the wrist level to upper arm and then transposed and tunnelled subcutaneously to the incision in the healthy side to be sutured with the contralateral C7 nerve. The ulnar vessels were dissected along with the ulnar nerve and the anastomosis was performed: ulnar artery with the transverse cervical artery and ulnar vein with a tributary of the external jugular vein.\nOn first postoperative day, sensory deficits were found in the index finger and middle finger and motor function of the healthy upper limb was normal. The muscle strength of latissimus dorsi, triceps brachii, extensor digitorum communis, flexor carpi ulnaris and extensor carpi radialis was all above M4. The grip strength was 36 kg (40 kg before operation) and the pinch strength was 4 kg (same as before operation). The patient did not manifest any signs of respiratory disorder. The paraesthesia in the healthy hand disappeared 2 weeks later and the grip strength returned to 40 kg and all joint function of the healthy side was normal.\nIn October 1987, 14 months after the first stage, percussion along the ulnar nerve from the healthy side toward the injured side showed Tinel's sign positive at the midpoint of the upper arm indicating the regeneration reached the site, which corresponded with the regenerating rate of approximately 1 mm per day, (38 cm over 420 days). The muscle strength of the biceps recovered to M3. Therefore the regenerated ulnar nerve was divided in the upper arm and transferred to the median nerve. In February 1989, 30 months after first stage, the muscle strength of the flexor carpi radialis recovered to M3, the flexor digitorum superficialis of 2nd - 5th fingers was M2, and sensation recovered to S2 in the radial 3 fingers. The muscle strength of biceps was M4 and the patient could freely flex the elbow without initiated by respiration. In August 1990, 4 years after first stage, the muscle strength of flexor carpi radialis, palmaris longus and flexor digitorum superficialis was M4, flexor digitorum profundus and flexor pollicis longus was M3 and sensation in the radial 3 fingers recovered to S3.\nTherefore we conclude the indications for contralateral C7 transfer as follows:\n1, No available neurotizers in the affected side;\n2, Used as one of the neurotizers in multiple neurotization in total root avulsion injury;\n3, When one of the multiple neurotizations fails for any type of avulsion injury |
In March 2021, a 68-year-old male patient was admitted to The Fourth Hospital of Hebei Medical University, Shijiazhuang, Hebei Province, China as an outpatient with ‘type 2 diabetes mellitus’ due to 10 years of elevated blood glucose and numbness and coldness in both lower extremities with intermittent claudication for more than 1 month. Physical examination showed no abnormalities. The patient was admitted with a lower extremity vascular ultrasound examination. Examination of the ultrasound findings suggested the possibility that the patient had multiple plaque formations due to atherosclerosis. In addition, routine blood tests were performed on the patient. The patient had an increased white blood cell count with a value of 11.63 × 109/l. The patient had an elevated neutrophil count with a value of 9.136 × 109/l. The patient had an enhanced neutrophil percentage with a value of 78.60%. The rest of the routine blood tests showed normal results.\nDuring admission, the patient developed an unexplained fever and his symptoms continued to worsen. Due to the patient's symptoms mentioned above, routine blood tests were performed again. The results of the examination showed that the patient had a white blood cell count of 17.86 × 109/l, a neutrophil count of 14.32 × 109/l and a neutrophil percentage of 80.20%. All routine blood test values were within normal limits except for the white blood cell count, neutrophil count and neutrophil percentage, which were higher than the previous test values. According to the patient's severe and persistently worsening fever symptoms, the patient was considered to have a serious infection, but the patient's foci of infection were limited, and it was recommended to change the antibiotic anti-infection treatment. Cefoperazone sodium sulbactam was administered (2.25 g cefoperazone sodium sulbactam dissolved in 250 ml of saline, administered intravenously three times a day for 3 days), but the patient's febrile symptoms were not effectively relieved and the treatment was not effective. The patient then underwent an abdominal CT examination. Based on this examination, the patient was diagnosed with a cystic solid lesion in the right costophrenic angle (). To further clarify the cause of the fever, CT-guided puncture of the right costophrenic angle abscess was performed in stages; and 90 ml of purulent fluid and 100 ml of bloody fluid were withdrawn (), while negative pressure continuous drainage was applied. To clarify the nature of the lesion, puncture fluid was sent for cytoculture examination. The bacterial culture results showed Salmonella Dublin infection (). Following the CT-guided puncture of the right costophrenic angle abscess, the patient developed hypoproteinaemia, so they were administered 5g of hydrolyzed protein orally once daily for 1 day to improve their nutritional status. Then they were administered an additional 30 g of hydrolyzed protein orally once daily for 1 day. Following abscess drainage, administration of 2 g ceftriaxone sodium dissolved in 100 ml saline injected intravenously once a day for 2 days and hydrolyzed protein supplementation, the patient's body temperature normalized at 2 days. At the same time, the patient's routine blood tests were reviewed, which showed that the values had returned to normal. A repeat CT scan of the upper abdomen showed changes in the right costophrenic angle after abscess drainage and the right costophrenic angle abscess disappeared (). Based on this examination, the patient’s life was significantly better than before treatment. A balloon dilation of the arteries in the lower extremities was performed 3 days later to relieve the patient's discomfort in both lower extremities.\nEthics committee approval was not required because of the nature of this study (case report). The reporting of this study conformed to CARE guidelines.\n The patient provided written informed consent for publication of this case report. |
A 63-year-old male with type 2 diabetes mellitus of 30 years duration, complicated by obstructive sleep apnea, hyperlipidemia, bilateral proliferative retinopathy, obesity, and prostate cancer presented to the emergency department with complaints of shortness of breath, weight gain of 20 lbs, and increased abdominal girth occurring over the course of two weeks.\nThe patient has a history of poorly controlled diabetes mellitus with hemoglobin A1c (HbA1c) greater than 9% with the use of insulin pump therapy for over 10 years. He suffers from bilateral diabetic retinopathy which is being followed by an ophthalmologist but does not have any history of peripheral neuropathy, angina, intermittent claudication, or transient ischemic attacks (TIAs). He also does not have any history of diabetic ketoacidosis.\nHe had visited his endocrinologist five months prior to the current presentation for a routine follow-up visit. He was using the Medtronic MiniMed 530G pump with U-200 lispro insulin with continuous glucose monitoring. Shortly after that visit, his insulin pump was upgraded to the Medtronic MiniMed 670G pump with Guardian 3 sensor without any changes in the pump settings or insulin formulation. The patient was instructed in the use of the “Auto Mode” feature and began using that feature more than 70% of the time.\nAbout 10 weeks later, due to formulary issues, the insulin type was changed to U-100 aspart and the pump settings were adjusted to reflect the change in concentration. After three weeks, the patient had another follow-up visit at which point his HbA1c was 8.3% showing remarkable improvement. He did not have any complaints of edema or shortness of breath at that time and his weight was at his baseline. He did complain of the need for frequent pump reservoir refills due to the high doses of daily insulin requirements and asked that his insulin be changed to a more concentrated formula. After a discussion of the potential risks of doing so and at his request he was switched to U-500 regular insulin and pump adjustments were again made accordingly.\nAbout two weeks after the initiation of U-500 regular insulin, the patient started noticing an increase in his weight and the development of bilateral lower extremity edema. Within two weeks increase in abdominal girth and shortness of breath ensued for which he eventually presented to the emergency department.\nLaboratory studies including comprehensive metabolic panel (albumin: 4.3 g/dl), troponin, pro-B-type natriuretic peptide (proBNP), thyroid stimulating hormone (TSH), and urinalysis as well as electrocardiogram were all unremarkable. He had mild microalbuminuria with urine microalbumin 32.2 mg/L and urine microalbumin/creatinine 18.7 µg/mg creatinine. Imaging studies including chest X-ray, CT angiography (CTA) scan thorax, and CT scan abdomen/pelvis did not show any pulmonary embolism, pleural effusion, or free pelvic fluid. Echocardiogram was done and did not reveal any findings suggestive of systolic or diastolic heart failure. The patient was discharged from the ED and advised to follow up with his primary care provider (PCP).\nDuring the follow-up visit with his PCP, he was diagnosed with insulin edema based on the exclusion of other causes of acute generalized edema. He was started on a 10-day course of spironolactone 50 mg/daily, advised on salt and fluid restriction, and asked to monitor his weight daily. He was also referred to his ophthalmologist for evaluation of the development of macular edema.\nA follow-up visit on week 21 post-pump change showed a decrease of 10 lbs in body weight with significant improvement in the edema and shortness of breath. The ophthalmology visit had ruled out macular edema (Table ). |
A 55-year-old diabetic hypertensive woman presented to the emergency department with acute retrosternal chest pain. Physical examination showed a heart rate of 110b.p.m., a blood pressure of 90/60 mmHg, and was otherwise unremarkable. Electrocardiography displayed non-specific ST-T wave changes, and cardiac troponin T was positive (2 ng/L). Upon admission, the haemodynamics of the patient continued to deteriorate, with a falling blood pressure of 75/50 mmHg and signs of poor peripheral perfusion. With a working diagnosis of non-ST-elevation myocardial infarction, emergency echocardiography was performed showing preserved left ventricular function and no pericardial effusion. The right ventricle could not be visualized due to acoustic window limitations. The suprasternal window (Figure ) was used to view the aortic arch, considering aortic dissection in the differential diagnosis. Unexpectedly, however, a mobile thrombus inside the right pulmonary artery was seen, establishing the diagnosis of pulmonary embolism. The patient received thrombolytic therapy, accelerated streptokinase regimen,with significant improvement in the haemodynamic status. Follow-up echocardiography revealed resolution of the thrombus. Further workup for hypercoagulable states is recommended.\nPulmonary embolism is sometimes a challenging diagnosis, with fatalities attributed to missed diagnosis rather than failure of therapy. Transthoracic echocardiography signs depend on the indirect detection of acute right ventricle pressure overload. Direct visualization of right-sided thrombi is possible in only <4% of cases.\nHigh-risk pulmonary embolism in this case was diagnosed by direct thrombus visualization in an unusual echocardiographic view in a patient with limited acoustic windows and when computed tomography pulmonary angiography was not immediately available.\nAlthough the detection of thrombi with echocardiography is uncommon in pulmonary embolism, their recognition can have significant impact on patient management. This case demonstrates the role of bedside echocardiography in acute cardiovascular care, with particular stress on the suprasternal view that can unusually show a right pulmonary artery thrombus.\nConsent: The author/s confirm that written consent for submission and publication of this case report including image(s) and associated text has been obtained from the patient in line with COPE guidance.\nConflict of interest: none declared. |
A 29-year-old man with a history of depression and heroin dependence, but HIV-negative and otherwise immunocompetent, was found unresponsive with agonal respirations. He was pulseless and cyanotic; cardiopulmonary resuscitation was initiated and the patient was intubated on scene. He was determined to be in ventricular fibrillation and defibrillated several times. Due to poor peripheral vascular access, an IO device was inserted in the left proximal tibia and used to administer naloxone, epinephrine, and amiodarone.\nAt the local emergency department (ED), advanced cardiac life support continued for ventricular fibrillation alternating with pulseless electrical activity arrest. The patient’s core body temperature registered 28.3°C and aggressive rewarming was undertaken. The IO device was used to administer fluids and medications until it infiltrated and central venous access could be established. After almost 30 minutes, return of spontaneous circulation was achieved. The IO device was removed within an hour of arrival at the local ED.\nThe patient was subsequently transferred to our tertiary-care hospital ED and admitted to the intensive care unit. He had a complicated hospital course punctuated by volume overload, rhabdomyolysis, and acute kidney injury requiring mechanical ventilation and continuous veno-venous hemodialysis. He also developed a 9×8 cm necrotic wound over the left medial shin at the site of the previous IO device, managed conservatively with topical wound care. He was discharged after two weeks without residual neurological or functional deficits.\nSix weeks later, the patient returned to our ED with increased pain and malodorous serosanguinous drainage from a non-healing 7×5 cm wound involving his left medial shin. Plain radiography demonstrated underlying demineralization of the anterior tibial cortex (). Operative debridement confirmed necrotic bone and periosteum. Tissue cultures grew Escherichia coli, methicillin-sensitive Staphylococcus aureus (MSSA), Bacteroides fragilis, and other mixed microorganisms, and histopathology was consistent with acute osteomyelitis. Following serial debridement, the patient underwent left medial gastrocnemius muscle flap and split-thickness skin graft coverage of the wound. He was treated with a four-week course of intravenous (IV) ceftriaxone and oral metronidazole followed by two weeks of oral amoxicillin-clavulanic acid. He returned not long after with soft tissue infection involving the muscle flap and received an additional six weeks of intravenous ertapenem due to persistent tibial osteomyelitis.\nSeveral months later, a sinus tract draining purulent material surfaced at the site of his muscle flap. Magnetic resonance imaging demonstrated extensive osteomyelitis of the left proximal tibia with centrally necrotic bone, left knee septic arthritis, and myositis involving the muscle flap (). The patient subsequently underwent multiple operative incision and debridements of the left tibia with canal reaming and placement of an intramedullary antibiotic drug delivery device. Bone cultures grew MSSA once more. He was treated with six weeks of IV ampicillin-sulbactam and transitioned to chronic antimicrobial suppressive therapy with oral amoxicillin-clavulanic acid. The patient has remained on oral antimicrobials for the past two years with no additional infectious complications involving the left proximal tibia. |
A 34-year-old man presented with a 3-month history of paresthesia of his left foot. On examination, he had impairment of dorsal column sensation in both feet, with intactness of other modalities of sensation, and preserved motor and bladder functions. He had no other comorbidities in the form of diabetes or coagulation disorders. He had not undergone any previous surgical procedures. He was on a short course of gabapentin prior to the diagnosis. A magnetic resonance imaging (MRI) scan revealed an intradural extramedullary lesion arising from the T8 root on the left side compressing the spinal cord.\nHe underwent surgery for excision of the tumor. Under general anesthesia, a subspinous laminectomy was performed from T7 to T9, and the tumor was approached intradurally on the left side.\nIn subspinous laminectomy, the interspinous ligament on the distal limit of the laminectomy is cut and the spinous processes of the desired vertebrae are cut using angled bone-cutting forceps and turned proximally by preserving the interspinous ligament on the proximal limit of the laminectomy. On completion of the procedure, the spinous processes are sutured back.\nA radicular vessel was seen dorsal to the tumor which was preserved. Intracapsular debulking was performed and the tumor was excised following resection of the dorsal nerve root from which the capsule was seen to arise. The tumor was yellowish-grey and bilobed and was sent for histopathologic examination. The dura was closed in the standard fashion and gel foam and a dural patch were placed epidurally. A subfascial gravity drain was placed. No suction was applied, as is the standard procedure following durotomy. Adequate hemostasis was achieved. The patient recovered from anesthesia with normal neurology. He was progressing well for the first 2 days following surgery. He was kept on log rolling (i.e. simultaneous turning of shoulder, back and pelvis with assistance) and assisted with movements of the limbs.\nOn the first postoperative day (POD), the drain collected 150 ml which was predominantly altered blood. During the second POD, the drain collected 200 ml which contained cerebrospinal fluid (CSF) mixed with altered blood. On the third POD, drain was 150 ml of clear CSF, indicating that hemostasis was adequate in the immediate postoperative period.\nAfter 72 hours, our patient experienced stabbing pain in his back with radiation to the legs following repeated unassisted voluntary twisting of the body, despite being advised of the need for strict log rolling. A fresh streak of blood was observed in the drain by the attending clinician when the pain was experienced. Gradually, the patient complained of progressive numbness of both his lower limbs with deterioration in motor power to grade 3 with sensory impairment from T10 downwards. The time interval from the onset of pain to neurological deterioration was 2 hours. His motor power deteriorated further to grade 0, with complete anesthesia below T10, within a further short period of about 1 hour. By this time, the drain showed the collection of more fresh blood. This alerted us to the possibility of a bleed producing a neurological deficit. The patient was started on methylprednisolone as per the NASCIS 3 protocol []. His coagulation profile was checked and was found to be normal.\nEmergency spinal imaging was advised. Although MRI is the investigation of choice, it could not be carried out due to technical faults in the machinery. Hence, an emergency computed tomography (CT) scan with reconstruction was performed which showed a large extradural hematoma (Figure ) at the level of the surgical laminectomy. The patient underwent emergency re-exploration 3 hours following the onset of neurological deficit. There was a large hematoma (4.5 cm × 7 cm × 5 cm and approximately 150 ml) on the right side, extending into the epidural space and compressing the cord (Figure ). The blood clot had pushed away the sutured spinous process superiorly and to the left. The hematoma was evacuated. It was bright red in color suggesting a fresh arterial source. While removing the clot, active arterial spurting from vessels in the right paraspinal muscles was observed at two sites and these were promptly coagulated. No active bleeding from the epidural space was seen. The dura was reopened, but there was no blood collection inside. Neural tissues were found to be normal. Dural closure was performed. Cord pulsation was felt. Spinous processes were removed and paraspinal muscle closure was performed over an epidural drain. A second drain was used in the supramuscular plane. The skin was approximated using subcuticular sutures.\nImmediately postoperatively, the patient regained motor power and sensations. He had grade 4 power of both lower limbs and mild paresthesia. He showed good motor and sensory recovery during the following week. Methylprednisolone was continued for 24 hours. He was mobilized cautiously with particular instruction not to strain. He had an uneventful recovery. |
Sixty-three-year-old woman presented with multicentric left breast cancer requiring mastectomy. She is 69 inches tall with a weight of 215 pounds and a corresponding BMI of 31.8 kg/m2. She has a history of inferior pedicle breast reduction surgery performed 20 years ago (Fig. 1). Despite her previous reduction, she has a very large breast volume and footprint that will be difficult to replicate on the reconstructed left side. She desires nipple preservation and wants to keep her native right breast. We proceed with a left nipple-sparing mastectomy through her previous vertical limb and immediate prepectoral reconstruction using a full-height variable-projection tissue expander (width = 16 cm, height = 16.5 cm, projection = 6.8 cm, and volume = 850 ml) with anterior coverage using an acellular dermal matrix. Twelve weeks later, we exchange her tissue expander for the largest anatomical implant available, a full-height extra-projection 775 ml implant (width = 15.5 cm, height = 16 cm, and projection = 7.1 cm). She also undergoes a contralateral reduction of 300 g to achieve better symmetry. The final result is shown in Figure . Despite using the largest and tallest implant available and reducing the right breast by an additional 300 g, the entire upper pole of the left breast was depleted with significant size asymmetry between the breasts. We discussed multiple sessions of lipofilling to fill this defect but felt that an autologous flap would be more definitive. The LICAP flap was chosen to reconstruct the upper pole of the left breast. An intraoperative photograph is shown in Figure , where an extended flap is dissected based off the known perforators that arise anterior to the latissimus muscle at the level of the inframammary fold as previously described. This flap is rotated on its pivot point and used to reconstruct the upper pole of the breast by suturing it to the underlying pectoralis muscle. The final result 6 months after surgery is shown in Figure , where we have reconstructed the upper pole of her breast with the LICAP flap and have acceptable symmetry between the 2 sides. The patient is discharged the same day, and drains are removed on postoperative day 4. |
Male, 61 years old, complained of pain and swelling in his genital area (). Local examination revealed found swelling, erythematous, and a small patch of necrotic skin on the scrotum. The patient has diabetes and presented with a very odiferous wound due to infection of the penoscrotal area. We debrided the wound, and his metabolic status was controlled with three sessions of HBOT before closing the defect with STSG, followed by three HBOT sessions in the 7 days post skin graft (). Pus culture results indicated the presence of staphylococcal bacteria, and Meropenem was prescribed for antibiotic therapy according to the sensitivity test.\nFournier’s gangrene is necrotizing fasciitis arising from infection caused by aerobic and anaerobic microorganisms that affect the perineal area and can spread to the abdomen. The risk factors include diabetes mellitus, the use of steroid drugs, alcoholism, and disorders related to the immune system. The initial treatment for Fournier’s gangrene is debridement to prevent the infection from getting worse, followed by the administration of antibiotics against specific microorganisms. However, the low blood supply to the infected area can substantially slow the wound healing process. In this case, HBOT can help to accelerate wound healing by increasing oxygen levels and encouraging fibroblasts proliferation and tissue regeneration. Many cases of using HBOT as adjunctive therapy for Fournier’s gangrene have been described.,\nIn the case of Fournier’s gangrene, HBOT can be particularly beneficial; namely, the high oxygen concentration helps repair hypoxic tissues and cells, inhibits the growth of microorganisms, and accelerates wound healing. By enhancing the generation of reactive oxygen species (ROS), HBOT serves as a bactericide and/or bacteriostatic agent against anaerobic bacteria and restores the bactericidal activity of leukocytes in hypoxic wounds. In complex cases, such as those with diabetes mellitus comorbidity, the combination of adjunctive therapies such as VAC and HBOT has yielded satisfactory results in infection management., |
A 16-year-old female presented with a complaint of involuntary leakage of urine since birth. The patient reported being able to void normally apart from the involuntary leak. The previous leak of a few drops had increased in amount over the past 2 months, thus prompting medical attention. She could manage with 2 to 3 pads during the day, with 10 to 12 daytime voids in amounts ranging from approximately 90 to 240 mL. Her nighttime incontinence was severe; she consistently woke up wetting her bed every night.\nOn examination, the external urethral meatus was normal as was the external genitalia, and the vaginal introitus was moist. Renal parameters and the results of urine analysis were normal. Ultrasonography revealed right kidney hydroureteronephrosis (HDUN) changes and an atrophic left kidney. The intravenous urogram showed left kidney calcification with nonvisualisation of the same. The right kidney was hydronephrotic with a dilated ureter and dilated vagina wrongly interpreted as a normal urinary bladder (). A diethylenetriaminepentaacetic acid renal scan showed a nonfunctioning left kidney, delayed excretion phase of the right kidney, and a glomerular filtration rate of 65.71 mL/min. On cystovaginoscopy, the external urethral meatus and urethra appeared normal, with a small urinary bladder with approximately 20-mL capacity and an absent trigone. Urine could be seen coming out through the vaginal introitus. The vagina was highly capacious but ureteric orifices could not be identified. Computed tomography revealed calcification in the left kidney and left ureter, a nonexcreting left kidney, HDUN changes on the right side with delayed excretion, and a small urinary bladder separate from the capacious vagina (). The accumulating contrast was well delineated.\nOn exploration, the urinary bladder was represented by a tubular structure engulfed by the capacious vagina. The right ureter was moderately dilated, and the left ureter was minimally dilated and calcified. Both ureters terminated on the anterolateral wall of the vagina (). Ileocystoplasty with right ureteral reimplantation was done. The bowel contributed 90% of the reconstructed bladder wall, because the native bladder was atrophic. Left-sided nephroureterectomy was done. The histopathological examination revealed tuberculosis, for which antitubercular therapy was given. There was no evidence of tuberculosis in the excised urinary bladder specimen. The postoperative period was uneventful. The patient voided well with straining and is fully continent. Clean intermittent self-catheterization fourth hourly was advised for the initial few months, which the patient was later weaned from because she maintained good voiding. |
This patient is a 14-year-old male, who felt a popping sensation and significant right knee pain while jumping and colliding with another player during a basketball game the previous day. Following the injury, he was evaluated in an outside emergency department, where anterior, posterior, and lateral radiographs obtained in the emergency department demonstrated a tibial fracture consisting of two primary components (). He was placed in a cast and sought a second opinion regarding findings and management.\nUpon presentation to the clinic the following day, he reported mild pain (3/10) and noted no normal function of his leg. A physical exam was performed but was limited due to pain. Following the review of radiographic imaging, an MRI was performed, which demonstrated a type IIIB tibial tubercle avulsion fracture and complete tear of the patellar tendon from its distal attachment site, as well as a hematoma at the fracture site (). After discussing the findings with the family, the patient was scheduled to undergo open reduction internal fixation of a type IIIB fracture and repair of the patellar tendon three days following the initial injury.\nAn 8-centimeter anterior incision was made at the superior aspect of the tibial tubercle and extended distally. At the patellar tendon insertion site on the tibia, the tendon was noted to be completely avulsed from the bone cortex distally, while proximally, the tendon remained attached to the displaced tubercle. The tendon remained attached to the inferior pole of the patella. The anterior tibial plateau fragment was anatomically reduced using two fully threaded noncannulated screws (Arthrex, Naples, FL), while the tibial tubercle fragment was reduced via bicortical fixation with a 50 mm fully threaded 3.5 mm cortical screw (Arthrex, Naples, FL).\nThe distal patellar tendon was completely avulsed through two-thirds of its length. To restore the native footprint of the patellar tendon, a 4.5 mm PEEK (polyetheretherketone) corkscrew anchor (Arthrex, Naples, FL) was placed slightly lateral to the anatomic insertion site to avoid a stress riser on the anterior tibial cortex. The anatomic repair of the patellar tendon was completed with two mattress sutures and tied.\nIn addition to the avulsion of the patellar tendon and periosteum, it was noted that fascial tissue with tibialis anterior muscle belly avulsed through the injury site causing subacute extensive bleeding within the anterolateral compartments (). This scenario raised concern for impending compartment syndrome, and an anterolateral compartment release was planned.\nThree 3-centimeter incisions were made along the anterolateral aspect of the leg. The first was located 3 centimeters distal to the neck of the fibula, the second was located 10 centimeters above the distal fibula tip, and the third was located at the midpoint between the two. Under endoscopic visualization, the intramuscular septum was identified and Metzenbaum scissors were used to cut through the fascial compartment beginning in the anterior compartment and extending proximally then distally to the midtibia (). The fascial incision was extended posteriorly into the peroneal compartment and then was extended proximally and distally to the midtibia. These steps were repeated for the midpoint and distal incision sites. Distally, the course of the superficial perineal nerve was identified and the nerve itself was protected during the distal release of the anterior compartment. It was believed that the impending compartment syndrome occurred due to damage to the surrounding bony and muscular tissue. A medium Hemovac drain was placed along the length of the lateral compartment, exiting in the posterolateral proximal leg. The patient was placed in a hinged knee brace which was locked in extension. He was discharged home later that day.\nOn postoperative day number two, the patient's Hemovac drain was removed by a family member. The patient was seen 1 week postoperatively and noted moderate pain (6/10) and 0% normal function. On physical examination, incisional sites were clean, dry, and intact and a small fracture blister was noted on the posterior aspect of the knee—which was cleaned and redressed. Radiographic imaging revealed well-positioned screws, no evidence of new fractures or foreign bodies, and early evidence of callus formation. Two and a half weeks after surgery, the patient presented to the clinic for evaluation. He reported that he had no pain (0/10) and had 5% of his normal function at this time. On physical examination, he noted no tenderness to palpation of the knee joint, and he had 40 degrees of knee flexion. Anterior-posterior and lateral X-rays were taken which showed evidence of callus formation in the bone (). At this time, it was recommended that the patient begin gentle active range of motion exercises with extension and light flexion. He was also encouraged to become full weight-bearing with the brace until its removal two months postoperatively.\nFive months postoperatively, the patient reported no pain (0/10) and possessed 95% of his normal function at this time. On physical examination, he was nontender to palpation along the joint line. There was no laxity with varus or valgus stress. He demonstrated 5/5 quadriceps strength with no evidence of an extensor lag. He had an active range of motion from 0 to 130 degrees of flexion, and there was no lag with straight leg raise. Repeat anterior-posterior and lateral X-rays demonstrated a well-reduced tibial tubercle fracture as well as well-positioned and nondisplaced hardware (). |
A 24-year-old Caucasian man presented with a several month history of muscle pain, fatigue and insidious onset of pitting edema to his lower extremities. His symptoms progressed to include bilateral arm swelling, muscle pain to the thenar eminence of both hands, and paresthesias to his hands. He did not have a rash, joint pain, Raynaud phenomenon, oral ulcers, fever, hardening of the skin or weight loss, and he denied any cardiac, respiratory, genitourinary, or gastrointestinal symptoms. Prior to symptom onset, he had traveled to South America and reported possible ingestion of undercooked meat as well as swimming in a river and lake. He had frequent visits to the Northeastern United States and had recently spent an extended period of time outdoors in Rhode Island. Medical, surgical, and family histories were unremarkable, and he was not taking any medications. He had a history of mild alcohol intake, no history of smoking, and some marijuana use. On examination, his vital signs revealed a blood pressure of 104/57 mm Hg, pulse of 56 bpm, and normal temperature. He had no significant findings on head, neck, cardiovascular, respiratory, or abdominal exam. He had no cervical, axillary, or inguinal lymphadenopathy. He had significant pitting edema on his feet and legs extending up to his knees as well as non-pitting edema on the dorsum of both hands. Although there was edema, the skin was soft without any significant hardening and was without any overlying erythema. There were no signs of skin dimpling or grooves. His neurological examination revealed normal strength.\nHis initial laboratory work was significant for a mild eosinophilia of 700 with a normal white blood cell count, hemoglobin, and platelets. He had normal calcium, creatinine, and electrolyte levels. His alanine aminotransferase was slightly elevated and total protein slightly low, but he had normal albumin and bilirubin. His thyroid-stimulating hormone was slightly increased, but his free T4 and total T3 were normal. His urinalysis was normal. He had a normal level of creatine phosphokinase, sedimentation rate, and C-reactive protein. Further workup revealed a positive anti-nuclear antibody with a titer of 1:160 with a speckled pattern. His extractable nuclear antigen panel was negative, including Scl-70. His ANCA, myeloperoxidase and proteinase-3 serum studies were also negative. His ACE level and complement levels were normal. Infectious workup revealed negative stool studies for culture, ova, and parasites. Antibodies for HIV, CMV, and Trichinella were negative. Serum for histoplasmosis, cryptosporidium, coccidiomycosis, and interferon gamma release assay were negative. A blood smear for parasites was negative. An ELISA IgG/IgM test for Lyme disease was positive with subsequent testing with Western blot strongly positive for IgG (eight out of ten bands positive) and also positive for IgM (two out of three bands positive).\nThe patient had extensive imaging done with a normal CT scan of his neck, chest, abdomen, and pelvis. An echocardiogram was also normal. Due to a previous negative workup along with persistent pain and swelling, an MRI of his right lower extremity was performed (see Figure ). Imaging demonstrated extensive circumferential edema with enhancement of the superficial soft tissues, superficial fascia, and, to a lesser extent, deep fascia of the lower leg. Taking into account the mild peripheral eosinophilia and the imaging findings, a diagnosis of eosinophilic fasciitis was considered. To complete the workup, a biopsy of the fascia, muscle, and adipose tissue of the left calf was taken (see Figure ). The biopsy did not include the dermis. Surprisingly, there was no evidence of eosinophilic fasciitis. Instead, the specimen illustrated a striking granulomatous fasciitis and vasculitis. The fascia showed exuberant granulomatous inflammation (Figure A) with an inflammatory infiltrate that was made up predominately of histiocytes and CD3-positive T cells with very rare eosinophils (Figure B). The granulomatous inflammation centered primarily on small- to medium-sized blood vessels and was non-necrotizing. While the vessels did not display overt fibrinoid necrosis, they did appear damaged with loss of endothelial cells confirmed with CD31 immunostaining. The inflammatory infiltrate was seen extending into adipose tissue and particularly around blood vessels within the fat. The adjacent skeletal muscle also showed perivascular inflammation and vasculitis in both the perimysial and endomysial compartments. There was no endomysial fibrosis, fatty infiltration, or inflammation surrounding muscle fibers. AFB (acid-fast bacteria) and Wade-Fite stain were negative for mycobacterial organisms. GMS (Grocott-Gomori's methenamine silver) stain was negative for fungal organisms. Due to the positive Western blot for Lyme, a Warthin-Starry silver nitrate stain was performed to evaluate for spirochetes; however, no definitive organisms were seen. A Borrelia PCR analysis of the tissue was performed as well, but no DNA was detected.\nThe patient was given a diagnosis of granulomatous fasciitis along with a diagnosis of Lyme disease. He was first treated with doxycycline for 42 days straight due to initial Lyme serology being positive. The calf biopsy revealing fasciitis was not performed until the patient was 3 weeks into the doxycycline course. Once fasciitis was diagnosed, he was started on a prednisone taper starting at 1 mg/kg/day for a week with taper by 10 mg every 2 weeks. After 2 months of treatment with prednisone, the patient had near resolution of symptoms. A repeat MRI was performed 82 days after the initial MRI with the previously seen changes consistent with fasciitis nearly completely resolved with only a thin sliver of edema over the superficial fascia. On re-evaluation of the patient, he reported skin changes to his left upper arm at 20 mg of prednisone per day and skin changes to his left forearm at 7.5 mg per day. Examination of the upper arm revealed an atrophic patch with some overlying erythema and examination of the forearm showed indurated, bound-down, tense skin with a slight groove and minimal overlying hyperpigmentation. The rest of the dermatological examination was within normal limits. Skin biopsy revealed marked septal thickening with sclerosis, sparse lymphoplasmacytic infiltrate along the dermal subcutaneous junction, and swollen, homogenized collagen fibers with diminished spaces between the fibers (see Figure ). The clinical examination along with pathology revealing dermal and subcutaneous sclerosis was consistent with a diagnosis morphea profunda. Lyme serology was repeated, but Western blot for IgG was negative. A scleroderma antibody panel was negative as well. He was started on methotrexate and a higher dose of prednisone. |
A 34-year-old male presented with painless gross hematuria. A screening urine cytology was suggestive of urothelial cancer. Magnetic resonance imaging of the urinary bladder showed a giant pedunculated tumor (67 mm × 37 mm × 58 mm) on the anterior wall, with multiple daughter tumors up to 15 mm in size [], but all appeared to be non-muscle invasive. A cystoscopy confirmed extensive papillary tumors occupying almost the entire bladder cavity, except for a part of the right lateral wall. The prostatic urethra was also involved by the tumor. A contrast-enhanced computed tomography revealed normal upper tracts and absence of distant metastasis. The clinical tumor stage was ≤T1N0M0. TUR was performed with a curative intent using the standard mono-polar TUR system (Olympus Corporation; Tokyo, Japan). After resection of a number of daughter tumors, sequential resection was performed for the main tumor. The tumor was highly vascular and hemostatic coagulation was required after resection of every chip. As the resection continued closer to the tumor base, maintaining hemostasis became harder because of active bleeding and the papillary shape which covered the bleeding sites and prevented accurate pin point coagulation. All attempts of coagulation with the resection loop and the ball electrode were ineffective because the bleeding point could not be localised as the vision was impaired. Thus, we decided to insufflate the bladder with CO2 to achieve a clear cystoscopic view []. The CO2 intravesical pressure was maintained at 12–15 mmHg by the laparoscopic CO2 insufflator (high flow insufflation unit UHI-4, Olympus Corporation; Tokyo, Japan), and the bleeding point was localised. Tumor resection was then continued with conventional pure cut mode with intermittent smoke evacuation to ensure visibility. Clots and resected tissues were removed intermittently by flushing with saline.\nThe conventional coagulation mode often caused tissue carbonization, which was liable to dislodge from the coagulated surface, resulting in inadequate hemostasis. Instead, the soft coagulation mode provided a better hemostasis. These steps were repeated numerous times, and the exophytic portion of the tumor was finally resected. The operative time was 6 h, but there were no signs of hyponatremia or hypercapnia. Blood transfusion was not required and the hemoglobin fall was from 13.8 mg/dl to 12.9 mg/dl during the surgery. The postoperative course was uneventful and he was discharged on the 4th postoperative day. Although we attempted bladder preservation based on the preoperative imaging, he finally underwent radical cystectomy as the histopathology showed muscle invasive urothelial cancer (high grade, pT2). |
Colorectal Cancer History
A 30-year-old male presented to the oncology clinic at a tertiary healthcare center in Jeddah complaining of painless rectal bleeding, which was progressive in nature. It became persistent with every bowel movement for a duration of ten weeks with no other associated symptoms. Upon further questioning, the patient revealed that he is a smoker for more than 10 years with no family history of malignancies. Regarding laboratory investigations, all initial parameters were within normal limits except for slight anemia and a mildly elevated carcinoembryonic antigen (CEA) of 7 ng/ml. Subsequently, the patient had a biopsy done in a different hospital setting, which showed invasive adenocarcinoma of the rectum. The patient then returned to the oncology clinic and a Computed Tomography Scan as well as a Magnetic Resonance Imaging of the pelvis was done, which showed rectal cancer with no evidence of distant metastasis (T3N1M0). Consequently, neoadjuvant therapy was started in the form of 25 cycles of radiotherapy along with chemotherapy in the form of Xeloda (Capecitabine) tablets 825 mg/m2, equal to 1500 mg in the morning and 1000mg in the evening.
The patient then underwent abdominoperineal resection surgery for tumor removal as no peritoneal metastasis evidence was found, with removal of 20 lymph nodes which were histopathologically negative for malignancy. Subsequently, he completed four cycles of six planned cycles of XELOX (Oxaliplatin and Capecitabine), which the patient refused to continue due to neuropathy, over a period of four months and was lost to follow up.
Therapy Related Myeloid Leukemia
The patient presented to the emergency department six months after he was lost to follow, complaining of abdominal pain, distention, and bruising spots for few days. The abdominal pain was moderate in intensity and the ecchymosis was found in multiple areas throughout his body. Regarding his lab investigation, pancytopenia was seen with hemoglobin 8.9 g/dL, white blood cell |
A 17-year-old boy with history of fall from height of approximately 15 meters presented to our institution after receiving primary care at another hospital. At presentation, he was conscious, hemodynamically stable maintaining oxygen saturation at 98% on room air with no visible signs of respiratory distress. His Glasgow coma scale (GCS) was 15, and was able to move all four limbs. The patient had sustained open fracture both bone left leg along with fracture right ankle.\nChest radiograph showed no intrathoracic injury with normal lung parenchyma. Computed Tomography showed burst fracture of fifth lumbar vertebra with canal compromise () and ruled out any injury to head, cervical spine, thorax and abdomen. After primary care, patient was admitted to the orthopedic ward for spine stabilization surgery and surgery for lower limb fracture.\nOn day four post-admission, an emergency consultation call was sent to our intensive care unit (ICU) in view of patient’s deteriorating status. When seen, he was grossly pale and febrile at 101 F, pulse rate of 140 per minute, systolic blood pressure of 80 mmHg and respiratory rate of 32 per minute maintaining oxygen saturation around 90% on oxygen face-mask. Patient’s GCS was 15. Chest auscultation revealed bilateral diffuse coarse crepitation and he was immediately transferred to the ICU. Initial arterial blood gas (ABG) showed partial pressure oxygen (pO2) of 49mm Hg on oxygen by face mask. Patient was intubated, sedated, paralyzed and put on mechanical ventilation with initial settings of volume assist control and high positive end expiratory pressure (PEEP). Central venous catheter was secured in right internal jugular vein under ultrasound guidance. An arterial line was secured in right radial artery for invasive blood pressure and arterial blood gas analysis. Chest radiograph showed bilateral fluffy opacities (). Preliminary blood investigations were mostly unremarkable except for hemoglobin of 6.8 mg/dl and raised ESR of 44. Fundus exam specific for FES was normal and there was no petechial rash on general examination. Urine for fat globules was positive. Over the next few hours, patient’s hypoxemia worsened requiring higher fraction inhaled oxygen (FiO2) of up to 0.8 and PEEP of 16 cm water. A decision was made to turn the patient into prone position after discussion with the orthopedic surgery team in view of unstable lumbar spine fracture. Patient’s family were informed about the specific risks and benefits of prone positioning, particularly in a patient with pre-existing unstable lumbar spine fracture and a written consent for the same was obtained. Positioning was done with the help of 5 trained ICU staff, using logrolling technique for turning the patient lateral followed by a 6th member placing the spinal board under the patient. After securing the patient on the spinal board, he was then shifted to one edge of the bed while the head gel support, chest and pelvic roll were placed in position. Patient was then shifted to prone position and spinal board was removed. Patient’s hands were abducted and placed next to the head and all the pressure points were padded using pillows and cotton rolls.\nOver the next 16 hours, we kept our patient sedated and paralyzed in prone position during which he received targeted fluid therapy and remained hemodynamically stable. Wake up test and pupil examination were done at regular intervals. Serial ABG’s showed dramatic improvement with Fio2 requirement decreasing to 0.4 and PEEP of 8 following which patient was repositioned supine. Two units packed red cells were transfused while the patient was prone. Chest radiograph showed resolving lung infiltrates and we decided to electively mechanically ventilate our patient for next 24 hours in supine position. Weaning was started next day and patient was extubated a day after and transferred back to ward 24 hours later.\nThe patient later on went on to have multiple surgical procedures for injuries to spine and bilateral lower limbs. He was then followed for 8 weeks in the out-patient department following discharge during which he recovered well without any neurovascular deficit. |
A 62-year-old female patient who is heavy smoker presented with a burning sensation and discomfort in her left breast that has been recurring over a month prior to admission to the hospital. No fever, chills, or any other symptoms were described. She reported a past medical history of hypertension and a surgical history of hemorrhoidectomy, dilation and curettage surgery, colonoscopy, and gastroscopy.\nPhysical examination revealed a palpable left breast mass (measuring approximately 3 × 3 cm) in the upper quadrant with no overlying skin changes. The right breast exam was normal. No palpable locoregional lymphadenopathy (axilla and supraclavicular lymph nodes) was noticed. Routine blood tests (complete blood count with differential, electrolytes, prothrombin time, partial prothrombin time, and international normalized ratio), chest X-ray, and electrocardiogram (ECG) were all normal.\nMagnetic resonance imaging (MRI) of the left breast showed an ill-defined deep retroareolar spiculate lesion extending over 3 × 1.5 cm revealing early enhancement peak with associated architectural distortion. There were no axillary lymph nodes or abnormal bone signal intensity. No cutaneous thickening or retraction was seen. Findings were suggestive of BIRADS type IV lesion ().\nAn excisional biopsy was performed and revealed breast tissue with extensive lymphocytic infiltrate intermixed with neoplastic epithelial cells (). Immunohistochemistry results were positive for CK AE1/E3 antibody in the neoplastic epithelial cells with no expression of estrogen or progesterone receptors, and HER2/neu was not overexpressed (). The lymphocytes in the background stained positive for both CD3 and CD20 (Figures and ).\nThe patient underwent a left modified radical mastectomy. Eleven lymph nodes were dissected and free of tumor. The mastectomy specimen showed a 3.5 × 3 × 3 cm cavity at the site of the previous excisional biopsy. On histological examination, apocrine metaplasia was identified but no residual tumor was detected. To note, apocrine metaplasia is a very common incidental benign finding that is considered part of or associated with fibrocystic changes, and hence, does not affect prognosis and management []. Accordingly, no adjuvant hormonal therapy, chemotherapy, or radiotherapy was given to the patient.\nNo evidence of recurrence was noted on a 2-year follow-up. |
Case 2: A 45 year old female presented to the Endodontics Department of Stomatology Hospital of Tianjin Medical University with the complaint of a toothache during mastication in the maxillary right posterior region for more than 1 wk. Both second maxillary molars were missing and the patient denied tooth extraction or orthodontic history. However, she had undergone root canal treatment in the right posterior tooth several years previously. Clinical examination revealed a metal crown and pain on vertical percussion in tooth 16. A buccally periodontal pocket approximately 6 mm was detected in tooth 16, which suggested an unhealthy periodontal condition. The patient’s medical history was noncontributory. A preoperative intraoral periapical radiograph revealed a maxillary first molar with an unsatisfactory root canal filling, with two roots and an obvious low-density image around the periapical area that indicated severe inflammation (Figure ). From the clinical and radiographic findings, a diagnosis of apical periodontitis was made, and re-root canal treatment was planned and accepted. Written informed consent was obtained from the patient. Access to the pulp cavity and root canal was achieved through the crown using the DOM. The former core and gutta-percha were removed, and only two separate root canals were detected (Figure and C). In order to ensure the efficacy of treatment, CBCT was suggested to determine the number of root canals.\nBased on the direct images as well as those reconstructed from CBCT, it was surprising to find that both of the patient’s maxillary first molars only had two roots and root canals, buccal and palatal, as seen in case 1 (Figure -E). The re-root canal treatment was completed and confirmed by CBCT and DOM as in patient 1. Her pain was relieved after the first treatment, as shown by the patient’s subjective sensation. The root canals were filled with gutta-percha (Figure -E) and the tooth was finally restored using composite resin.\nIt is significant that in both of these cases, the right maxillary first molars were symmetrical to the left maxillary first molars in their anatomical details. This has seldom been reported before and illustrates the condition of two root canals and two roots in both maxillary sides in these two patients. |
A 38-year old, previously healthy man presented on his first admission with a 5-day history of dizziness, slurring of speech and disturbance of gait and balance. There was a family history of multiple sclerosis (paternal grandfather and a paternal cousin) but not of any other cerebellar disease. The patient smoked 30 cigarettes per day for the last 25 years. Neurological examination revealed dysarthria, trunk and gait ataxia and exaggerated tendon reflexes of the upper and lower limbs. Spontaneous nystagmus was absent and pursuit eye movements were smooth. Routine hematology was normal but the cerebrospinal fluid (CSF) showed elevated cell count (407 lymphocytes/μl) and protein (1 g/l) while glucose and lactate were normal. CSF cytology provided no evidence of malignant cells but oligoclonal IgG bands were detected in the CSF. HSV- and VZV-PCRs were negative. Cerebellitis caused by an unknown infectious agent was suspected and a polyvalent intravenous therapy with aciclovir, ceftriaxone and ampicilline was initiated which halted disease progression. Cerebral MRI was unremarkable. During the next months he underwent two neurorehabilitation treatments without relevant efficacy on the residual symptoms, but 10 months after disease onset he rapidly deteriorated.\nOn admission this time he reported severe gait and trunk unsteadiness, difficulties in writing, massive slurring of speech and problems of swallowing. On physical examination he showed a substantial weight loss of 15 kg over the last 12 months but no enlargement of lymph nodes. Neurological examination revealed a cerebellar syndrome with an exaggerated horizontal nystagmus during lateral gaze and a scanning dysarthria. Fixation suppression of the vestibulo-ocular reflex was abolished. There was no muscle weakness or sensory loss and his limb reflexes were normal but plantar responses were bilaterally extensor. He showed severe dysmetria of all four limbs and trunk and gait ataxia. Walking without aid was impossible.\nRoutine biochemistry and hematology were again normal. Cranial MRI now showed marked cerebellar atrophy. The CSF contained 18 leukocytes/μl, 0.85 g/l protein, oligoclonal IgG bands, and normal glucose and lactate concentrations. CSF cytology was again normal. Infectiological screening of the CSF (HSV-1 and -2, VZV, CMV, EBV, HIV, treponema pallidum, brucella, borrelia) was negative. Further biochemical examination showed normal vitamins B12 and E and very-long-chain-fatty acids. Anti-gliadin-antibodies were negative. Squamous cell carcinoma-antigen (SCC) was mildly elevated (2.7 U/l, reference < 1.5) while NSE, PSA, CYFRA 21–1, CEA and electrophoresis were normal. Classical onconeural antibodies (anti-Hu, -Tr, -Yo, -Ma, -Ta, amphiphysin, CV2/CRMP5) were not detected via immunofluorescence test (IFT). MRI of the entire spinal axis did not show any metastases and search for a pulmonary or abdominal neoplasm in CT-scans of the chest and abdomen was also unremarkable. A whole-body PET/CT-scan revealed a mild hypermetabolism of the left thyroid lobe and the right tonsil and neighboring tongue-base (Figure a,b). Sonological and histological examination of the thyroid did not detect any malignancy. Inspection of the tonsils revealed no signs of a neoplasm except for an asymmetry with a larger right tonsil. Histological examination after tonsillectomy revealed a lymphoepithelial carcinoma of the lower tonsil and tongue base with low-grade differentiation (Figure c,d). The mitosis marker Ki67 was present in 40% of tumor cells. Immunohistochemical workup revealed numerous CD3+ T- and CD20+ B-lymphocytes with positivity for bcl2. Staining was negative for CD23, CD30, CD34, CD138, and the macrophage marker KiM1P. Additional resection of the neighboring tongue ground including neck dissection detected no further neoplastic tissue. Accordingly, the interdisciplinary tumor board did not recommend radio- or chemotherapy. In the course of the next eighteen months the patient underwent further intensive neurorehabilitation treatment leading to a stable cerebellar dysfunction with only slight progression of dysarthria which was treated every three months with steroid pulses (i.v. methylprednisolone 1 g/day for 5 consecutive days). |
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