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A 28-year-old female from another prefecture came to our department for psychiatric diagnosis and treatment. Because her obstetrician informed her that her husband's sperm and her egg were old given their age, she worried about the health of her fetus during pregnancy. After reading a newspaper article on Down syndrome, she began to have thoughts about the term Down syndrome. When she thought of it, she subsequently and repeatedly thought of the word healthy until she canceled the term Down syndrome. She was diagnosed with OCD in a psychiatric hospital at the age of 26, but she refused to be treated because she believed that she could cure the OCD with her own efforts. When she was 27 years old, the words unhappy, death, die, and kill came to her mind at the same time as the face of an acquaintance. She tried to counteract these obsessive-compulsive thoughts by repeatedly thinking of the words different and not die. After four months, she began to think of the words rape, crematorium, neck hanging, unhappy, death, die, and kill and then images of a shelf of gods, a shrine, a Buddhist altar, the sun, and the sky. She tried to avoid looking at these things. She visualized the faces of her parents, siblings, and relatives appearing in her images at the same time as the images of these words. She tried to erase the words and images by repeating the word different. Because her obsessive-compulsive symptoms did not improve, she revisited the psychiatric hospital and was diagnosed with OCD again. Treatment with aripiprazole and fluvoxamine was initiated. She had received psychoanalytic counseling from a psychiatrist for 6 months, but she did not experience relief. She discontinued the psychoanalytic counseling and drug treatment after consultation with her doctor. Next, she visited our department with the hope of improving her obsessive-compulsive symptoms. She was diagnosed with OCD according to the Diagnostic and Statistical Manual for Mental Disorders (fifth edition) on the basis of her recurrent thoughts and images and her attempts to suppress them. We excluded current Axis I diagnoses such as major depressive disorder using the Mini International Neuropsychiatry Interview (MINI). She was prescribed escitalopram (20 mg for 6 months) and CBT. She was asked to look at the images of a shelf of gods, a shrine, a Buddhist altar, the sun, the sky, and the faces of her parents, siblings, and relatives three times a day. After 6 months of these treatments, her obsessive-compulsive symptoms improved to the extent that they did not bother the patient. After 6 months, she could begin the qualification tests and do housework such as cooking, washing, and cleaning. Our treatment was successful, and the patient showed clinically significant changes in her obsessive-compulsive symptoms. The first treatment was probably not adequate, and after initiating an adequate treatment the patient fully responded. The Yale-Brown Obsessive Compulsive Scale score reduced from 31 points to 10 points within 6 months of treatment. Changes in [oxy-Hb] were measured as an index of changes in cerebral blood volume and in [deoxy-Hb] using a 47-channel NIRS machine (Hitachi ETG-4000; Hitachi Medical Systems, Tokyo, Japan). The correspondence between NIRS channels and measurement points on the cerebral cortex was confirmed by comparison with the results of a multiparticipant study of anatomical craniocerebral correlation []. We used verbal fluency task in the phonemic variant. NIRS results showed increased oxygenated hemoglobin levels in the frontal and temporal lobes after 6 months (). |
A 51-year-old black male with a past medical history of hypertension and cardiomyopathy (ejection fraction 15%) was brought to the emergency room with complaints of severe stabbing, abdominal pain located in the epigastric area radiating to the right upper quadrant. The pain was precipitated after intravenous cocaine injection. The pain was associated with nausea, nonbloody vomiting, and diarrhea with nonbloody, liquid stools. Pertinent positive finding on physical exam was moderate abdominal tenderness in the epigastric area and right upper quadrant on deep palpation. Lung exam revealed bilateral pulmonary congestion and tachycardia on heart examination. On initial vitals, blood pressure was 188/95 and heart rate was 104. No significant changes in the baseline laboratory studies were noted except urine drug screen was positive for cocaine. CT scan of the abdomen showed extensive portal venous gas, mesenteric venous gas throughout the abdomen []. There was gas also seen in right inguinal region extending into the right hemiscrotum, with wall thickening of the ascending colon. The electrocardiogram showed no ST/T changes. The patient was given a nitroglycerin patch for the elevated blood pressure. Empiric intravenous antibiotics and analgesics were administered. Before the patient was taken to the operating room for the diagnostic laparoscopy to find out the cause of portal vein air, CT scan of the abdomen was repeated and it was done 5 h after the first CT abdomen. The repeat CT scan revealed near complete resolution of the portal venous gas and mesenteric venous gas []. Next day abdominal X-rays were done, which were also negative for extra luminal air. Septic workup including blood culture, urine culture, stool for clostridium difficile, HIV, hepatitis A, B, C was negative. Hepatobiliary iminodiacetic acid scan was also negative for acute cholecystitis and showed a patent biliary tree. After 4 days in the hospital, the patient's clinical condition improved and he was subsequently discharged home. |
A 37-year-old Hispanic male with a past medical history significant for testicular cancer and nephrolithiasis and unremarkable past ocular history was examined in the hospital by the emergency department provider for right eye pain. The previous night, the patient was handling some coral he had bought online and cut his hand on the rock. Additionally, upon putting the coral in his aquarium, a plume of dust was released into the air. Within a couple of hours, he noticed severe right eye (OD) pain. At no point during his care did the patient have any complaints or abnormal findings on his left eye (OS). It is possible that he may have inadvertently touched the affected eye (OD) with his fingers but did not recall this when he gave his history.\nThe patient presented to the emergency room the morning after exposure where he was examined by an emergency room provider, given erythromycin ointment and discharged home with outpatient ophthalmology follow-up scheduled. Over the course of that day, his previously healthy dog got sick and died, and his cat developed respiratory symptoms. The patient’s 17-year-old daughter and her boyfriend also developed a cough. The patient developed worsening eye pain and respiratory symptoms and re-presented to the ED that same night where he was evaluated by the ED provider, prescribed topical ciprofloxacin, and sent home again. The patient awoke the next morning (2 days after exposure) with eye pain and discharge, difficulty breathing, epigastric abdominal pain, streaks up his forearm, and diffuse pruritis. He re-presented a third time to the ED, was admitted to the hospital, and was later transferred to the ICU for increasing shortness of breath and a likely diagnosis of palytoxin exposure.\nChest and hand X-rays showed bibasilar infiltrates but no obvious signs of infection and a head CT (obtained over concerns for preseptal cellulitis) showed only preseptal tissue swelling. White blood count and C-reactive protein were elevated (18.3 and 200, respectively, on admission). Broad-spectrum antibiotics including meropenem 1000 mg three times a day, linezolid 600 mg two times a day, and vancomycin 15mg/kg every eight hours were all started IV by the ICU team due to his symptoms and respiratory distress, and ophthalmology was consulted. Exam revealed visual acuity in the right eye was counting fingers, and he had 1+ edema of the upper and lower lids, chemosis, 2+ conjunctival injection, diffuse superior and peripheral punctate keratitis with a 360° edematous cornea at the limbus. The pupil was miotic, presumed to be secondary to pain medication. The patient was diagnosed with presumed toxic palytoxin keratitis.\nThe patient was started on Tobradex (Alcon Laboratories, Inc) four times a day, cyclopentolate 2% two times per day, and artificial tears every two hours while awake. Fourteen hours later, his vital signs stabilized and his breathing had improved. Mucus was noted in the inferior fornix. A peripheral inflammatory ring and 1+ folds in Descemet’s membrane were also observed. His WBC had improved (7.3) and broad-spectrum antibiotics were discontinued. He was discharged home (now day four after exposure) on his ophthalmic drops as well as ampicillin and amoxicillin.\nThe following day (five days after initial exposure), he presented to the clinic for initial evaluation with the author (Hollingshead Eye Center, P.C., Boise, ID) for follow-up where the patient complained of discharge and worsening severe pain in his right eye. His left eye was asymptomatic. He complained of feeling ‘sick to his stomach’ and had a headache. On examination, his best-corrected visual acuity was to hand motions OD and 20/25 OS. Slit-lamp examination showed upper lid edema and 2+ diffuse conjunctival injection without limbal blanching. Corneal scarring, opacification, and edema were present. Fluorescein (BioGlo Fluorescein Sodium I.P. 1 mg, Omni Lens Pvt. Ltd.) staining revealed 95% loss of epithelial cells with trace epithelium remaining at the corneal limbus from 10 to 1:30 clock hours. Folds in Descemet’s membrane were present and an irregular stroma was observed. Visualization of the anterior structures was difficult due to 2+ corneal edema, consistent with a Grade III injury in the Hughes classification scheme (as modified by Thoft).\nAfter instilling proparacaine (Proparacaine Hydrochloride Ophthalmic Solution, USP 0.5%, Alcon Laboratories, Inc), the eye was rinsed with a full bottle of Accu-WASH Eye Irrigating Solution (Accutome) as it had not been previously irrigated. Following the rinse, the patient was again numbed with proparacaine, and a Prokera (BioTissue, Inc) amniotic membrane was placed. Topical prednisolone 1% (brand unknown) and moxifloxacin 0.5% (brand unknown) were prescribed four times a day and topical cyclopentolate 1% (brand unknown) three times a day. A shield at night with shatter-proof glasses and preservative-free artificial tears, one drop hourly while awake, were recommended.\nTwo days later (seven days after initial exposure), the patient was seen again with a complaint that his amniotic membrane graft had fallen out. The examination was unchanged with the exception that he’d now developed thinning along the inferior limbus. The membrane was replaced, and topical doxycycline 100mg twice a day with oral vitamin C 1000 mg was added to his treatment regimen. The amniotic membrane fell out again one day later and the decision was made to leave it out since the patient would not tolerate it. On examination four days later (eleven days after exposure), he complained of photophobia and a continued headache. On examination, the opacification had worsened and worsening inferior limbal thinning (now estimated to be 50% thinned) was noted. The frequency of prednisolone drops was increased to every two hours.\nOn follow-up one week later, there was no subjective improvement and the eye was still in severe pain. However, there was some improvement, with the limbal epithelial edge healing (epithelium present from 10:00 to 2:00) but still with an 80% epithelial defect. The surface layers of the cornea were gelatinous and a bandage contact lens was inserted to help facilitate healing. The inferior thinning remained stable at 50%. Over the course of several more exams in the following two weeks, the patient’s pain had decreased significantly, but the photophobia remained. Additional healing was noted at the limbal edge but with no improvement of the thinning along the inferior limbus. The cornea continued to have a gelatinous appearance, with the cornea appearing to shift and move with even gentle pressure such as with the patient blinking.\nOn day 29 after initial exposure to palytoxin, the patient presented emergently to the clinic with excessive pain and a right-sided headache. He stated that he put some pressure on his eye and eyebrow and it, “gushed water everywhere.” Examination showed 2+ Meibomian gland dysfunction OD and possible corneal perforation. Intraocular pressure was unmeasurable. The iris was adherent to the area of presumed perforation; Seidel-sign was negative, and the cornea was so opaque and edematous that the anterior chamber was difficult to visualize. Emergent penetrating keratoplasty was scheduled at the hospital’s earliest availability. Brimonidine 0.1% twice a day was prescribed to minimize aqueous production in the hopes of keeping the iris plugging the presumed corneal perforation until surgery could be done.\nOn examination the following day, just prior to surgery, 10:00–2:00 epithelialization was stable. Severe thinning along the limbus was noted in the 4:30–7:30 position with a bulging overhanging ledge of swollen white cornea. This thinning was presumed to be the site of perforation. Bullae was present in the inferior cornea. Seidel sign was negative along the entire cornea and limbus.\nSurgery consisted of anterior and posterior synechialysis, temporary tarsorrhaphy, and corneal transplant with an 11 mm corneal graft into an 11 mm bed – the largest trephines available at the time of surgery. Following uneventful surgery, a successful clear, large corneal scleral transplant with intact sutures was noted. Postoperatively, suture removal was completed over the course of 17 months. Mild non-progressive peripheral anterior synechia was noted in the areas where the graft extended into the sclera, mild posterior synechia, and a 2+ anterior and posterior subcapsular cataract. Fourteen months postoperatively mild graft rejection was noted and the patient’s prednisolone was increased from once daily to every 2 hours for two weeks, then tapered back to once daily. Eighteen months after penetrating keratoplasty the final sutures were removed, the graft remained clear, and the patient’s best spectacle-corrected visual acuity was 20/200, pinhole 20/70 (). At final follow-up, twenty months following PTX exposure, scleral lens fitting allowed for 20/15 best-corrected visual acuity. |
A 78-year-old female presented at our hospital with discomfort in the inner portion of the lower lip since several months. The discomfort gradually increased, and the patient visited our hospital in June 2012 for a detailed examination. Her medical history included hypertension, and she was taking an antihypertensive drug for the same. She had a medium build and was short in stature. An extraoral examination revealed several elastic and hard lymph nodes on both sides of the neck. In addition, granular areas were observed roughly at the center of the vermillion. Intraoral examination revealed a superficial granular mass measuring 26 mm × 22 mm and extending from the inner cutaneous portion of the lower lip, which was connected to the vermillion [Figure and ]. No induration was felt on palpation. On the basis of these findings, we diagnosed lower lip cancer with metastasis to the lymph nodes on both sides of the neck.\nHistopathological examination of a biopsy specimen from the primary lesion revealed well-differentiated squamous cell carcinoma []. Moreover, 2-[fluorine-18] fluoro-2-deoxy-d-glucose (FDG) positron emission tomography/computed tomography showed increased FDG uptake by the primary lesion [Figure and ]. Several metastatic lymph nodes were also confirmed on both sides of the neck. A final diagnosis of squamous cell carcinoma (T2N2cM0) of the lower lip was made, and treatment was accordingly planned. First, excision surgery was performed. However, extracapsular invasion was a concern with lymph nodes showing rim enhancement; therefore, we explained the possibility of additional surgery involving the primary tumor and both sides of the neck.\nThe surgery involved left total neck dissection, right conservative neck dissection, and primary tumor resection involving all layers, with 1 cm safety margins. This resulted in the full-thickness loss of the oral mucosa at the center of the lip including the vermilion and the vermilion border. Considering that a unilateral Abbe flap would be inadequate, we decided to perform reconstruction with a bilateral Abbe flap. Using a #15 scalpel, we placed a sharp incision. Then, taking care to avoid arterial injury in the upper lip, we used forceps and ophthalmic scissors to obtain sufficient mobility for inferior reversal [Figure and ]. Similarly, a flap was prepared on the contralateral side [], and the bilateral flaps were placed over the defect in the lower lip []. We confirmed adequate mouth opening to ensure satisfactory postoperative function. However, immediate suturing would result in an insufficient external nose width; therefore, we placed an incision at the ala of the nose and made every effort to maintain the shape of the ala. The final sutures were placed with trimming at some parts of the flaps for the achievement of a proper fit []. To prevent wound dehiscence, the vermilion on both sides was sutured to restrict opening. During the postoperative healing period, a nasoenteric feeding tube was used, and the patient did not experience any other difficulties. Two weeks later, the upper and lower lips were separated under local anesthesia [Figure and ]. Despite the presence of a residual hematoma, the patient quickly recovered the ability for oral ingestion []. Because of extracapsular invasion in the lymph nodes, irradiation was performed to lower the risk of relapse. Once the patient could tolerate the intake of porridge, a total dose of 66 Gy was delivered to both sides of the neck and the primary tumor site. After the mucositis had subsided, the patient was discharged when she could again tolerate the intake of porridge. The patient remained healthy without relapse for 5 years after surgery. Over time, the scar became inconspicuous, and although her mouth opening was somewhat restricted, she could open her mouth wide enough to wear dentures [Figure –]. |
A 16-year-old female was referred to clinic with a 6-month history of gradual decline in visual acuity and metamorphopsia. There was no relevant medical or previous ocular history. Visual acuity at presentation in the right eye was 6/6 and 6/18 in the left eye. Examination of the fundus revealed a well-defined orange lesion at the macula with overlying pigmentary changes ().\nSpectral domain OCT (TopCon 3D 1000) revealed presence of SRF, a dome shaped RPE detachment with evidence of choroidal enlargement and a central retinal thickness of 297 microns (). FFA revealed RPE mottling, during the vein phase, a gradually increasing hyperfluorescence of a well demarcated lesion, and late persisting hyperfluorescence with no evidence of pinpoint leakage or late phase leakage. This increasing hyperfluorescence appears to be occurring within the entire tumor with possibly some pooling under the subretinal space. (). ICG reveals also a well-defined mass and no evidence of hot spots, staining, or leakage of the inner choroid (). Finally, B-scan provided the information to confirm the clinical diagnosis. An elevated choroidal mass of high reflectivity and acoustic shadowing was noted (pseudooptic disc) ().\nThe patient was referred to a specialist ocular oncologist and following assessment it was decided that active intervention was not currently appropriate and regular followup was the preferred management plan.\nThe patient was seen in clinic for followup 7 months later. Patient reported that visual acuity remained stable but metamorphopsia was still present. On clinical examination visual acuity remained stable at 6/18. Fundoscopic examination revealed that the osteoma had grown superiorly and temporally (). OCT showed that SRF was still present, with an increase in central retinal thickness to 340 microns (). A hyperreflective spot posterior to the SRF was noted and corresponded to atrophy. It was decided that management would remain conservative with followup in a further 6 months.\nThe patient was again seen for followup a further 6 months later. The patient reported visual acuity and metamorphopsia remained stable. Visual acuity was again recorded at 6/18. The osteoma continued to grow in a superotemporal direction (). OCT revealed that SRF was still present but central retinal thickness decreased to 251 microns (). Although anti-VEGF therapy could be a reasonable option at this point, the conservative approach of close followup was selected, mostly because of the lack of progression in symptoms and the stability of central retinal thickness. |
A 43-year-old lady with no previous medical illness presented to the emergency department with complaints of perioral numbness, altered tongue sensation, speech difficulty, and facial droop as noted by her husband since that morning. She was able to mobilize well with no peripheral limb weakness. She had been complaining of upper back pain for the previous 3 days and was seen by a general practitioner and given symptomatic medications with not much relief. She had mild headache and vomiting on the day before her presentation. She denied any fever, headache, neck ache, visual disturbances, giddiness, or tinnitus. There was no history of trauma, travel in the last few years, or joining any adventure expeditions. She denied any history of smoking, alcohol intake, blood transfusion, or sexual promiscuity.\nExamination revealed right facial nerve (lower motor neuron) palsy with mild slurring of speech. Examination of the other cranial nerves was normal. Power in all limbs was normal, deep tendon reflexes were present, and her sensory examination was unremarkable. There were no cerebellar signs.\nComputed tomography of the brain was done which did not reveal any obvious abnormality. She was referred to the ophthalmology department and no exposure keratopathy was identified. The patient was presumptively diagnosed to have right Bell's palsy and discharged with acyclovir, prednisone, analgesia, eye patch, and moisturizing eye drops. She was given an outpatient otolaryngology followup.\nShe represented to the emergency department after 3 days with progressive bilateral weakness of the face and drooling. She also had some blurred vision then but denied any weakness of limbs. Examination revealed bilateral lower motor neuron facial nerve palsy. No other neurological sign was elicited during this presentation. Her hematological and biochemical blood tests, including liver function tests and thyroid levels, were normal.\nAs bilateral facial palsy is suggestive of a possible underlying etiology, the patient was admitted under the care of neurology department. Chest X-ray and spinal X-rays were reported to be normal. Patient declined lumbar puncture and CSF examination. Serological tests for various agents, including thyroid peroxidase antibodies, antinuclear antibody, anti neutrophil cytoplasmic antibody, syphillis antibody, lyme (Borrelia) IgM, and Epstein-Barr virus capsid Ag IgM antibody tests, were all negative. Lyme (Borrelia) IgG and Epstein-Barr virus capsid Ag IgG antibody tests were positive. Magnetic resonance imaging (MRI) of the brain was reported to have bilateral asymmetrical enhancement of the facial nerves, right more than left, at the apex of their intracanalicular portion, as well as their labyrinthine, tympanic, and mastoid segments, which is may be due to atypical Bell's Palsy.\nThere was no progression of her symptoms during her admission. She was discharged in a stable condition after a 3-day stay in the hospital and she completed the course of acyclovir and prednisone. The patient has been asymptomatic since then and no recurrence has been noted during her followup visits at the National Neurology Institute. |
A 68-year-old woman presented with a one-month history of masses in the breast and chest wall. The patient recalled having dysphagia for dry food, xerophthalmia, xerostomia, and weight loss for 2 years; however, she did not undergo any test to search the cause of these symptoms. Physical examination revealed two masses in the chest wall and right breast, respectively, which showed poor toughness and mobility. On ultrasound examination, a heterogeneous hypoechoic mass (2.2 cm × 1.2 cm) with irregular shape and margin, angular periphery and incomplete capsule was revealed in the right breast about 5 cm from the nipple at the 10 o’clock position. The mass showed an aspect ratio less than 1.0 and posterior acoustic enhancement. No obvious blood flow was detected by color Doppler imaging (). It was classified as category 4 (suspicion for malignancy) according to the Breast Imaging Reporting and Data System for ultrasonography. The left breast and both axillary areas had no abnormality. In addition, another heterogeneous hypoechoic mass (5.8 cm × 2.2 cm) with striped hyperechoic features and unclear demarcation from surrounding muscle was found in the left anterior chest wall (). Striped blood flow with a high resistance index of 0.78 was revealed by color Doppler imaging (). Subsequent CT examination showed a slightly high-density mass with regular shape and margin in the right breast (). Likewise, a soft-tissue density mass was seen in the chest wall around the sternal stalk, and there was no distinct demarcation between the mass and the pectoralis major muscle (). All the masses showed homogeneous enhancement on enhanced CT images ( and ). In addition, no significant abnormality was found in the skull, pelvis and hip bones by X-ray findings.\nUltrasound-guided biopsy of breast and chest wall masses was done, and the hematoxylin and eosin (HE) staining of the sections showed lymphocyte proliferation (). Immunohistochemical analysis showed that the neoplastic cells were positive for CD138 (), IgD () and restriction for λ light chain, but negative for CD56 () and CD117 (). The diagnosis based on the pathological examination was plasma cell tumors. However, full blood count, liver and kidney functions were all in normal ranges. And then bone marrow biopsy revealed that the immature plasma cells accounted for 56% (). Meanwhile, flow cytometry of bone marrow aspirate with CD138/CD38 gating strategy showed positive expression for CD38, CD138, CD10, CD20, CD28, CD33, and λ. Serum protein electrophoresis and immunofixation were subsequently requested, showing monoclonal (M) protein (9.70%) with type IgD λ paraprotein (7.240 g/L). In addition, urinary Bence Jones protein was positive for λ free light chain, which showed kappa (κ) and λ at a concentration of 0.038 g/L and 2.910 g/L, respectively, the κ/λ ratio decreased significantly. According to the clinicopathological and laboratory findings, MM (IgD λ, stage III A) was diagnosed. Furthermore, we performed some tests to reveal the cause of xerostomia and dry eyes. Antinuclear antibodies were positive at 1/320, and the count of antibodies against Ro/SSA and La/SSB in serum was more than 200 U/mL. The presence of these antibodies along with clinical symptoms established the diagnosis of SS.\nBased on the above findings, the patient was diagnosed as extramedullary MM complicated with primary SS, and then she was treated with bortezomib, thalidomide and dexamethasone. The clinical symptoms were improved after three courses of therapy, xerostomia and xerophthalmia alleviated, and the breast and chest wall masses almost disappeared simultaneously on repeated CT examination ( and ). The patient’s condition was stable, and we will continue to follow her outcome. |
A 48-year-old man first visited an otolaryngology clinic in May 2005 because of exertional dyspnea. His symptoms persisted and deteriorated over several months, and he was found to have a tracheal tumor. At this point, he was transferred to a university hospital. The patient did not have hypertension, diabetes, tuberculosis, viral hepatitis, or HIV infection. Several years earlier, he had undergone an operation for appendicitis. He did not have any known drug allergies. His family did not have a history of respiratory cancer. The patient reported that he had smoked approximately 4 cigarettes a day since he was 23 years old and had drunk 50 mL of Korean distilled spirits per day since he was 20 years old. He had no dyspepsia or resting dyspnea. Visual inspection of the larynx did not reveal any remarkable findings. The Epstein-Barr virus (EBV) antigen/antibody status was not available from the medical records.\nComputed tomography (CT) of the patient’s neck, conducted on May, 2005, revealed a 3.5-cm polypoid soft-tissue tumor at the inner side of the right posterior wall of the subglottic region, extending to the outer side. The tumor blocked the inner cavity of the trachea. Laser microsurgery was conducted for partial excision of the tumor and frozen section biopsy. The final diagnosis on the basis of the frozen biopsy was tracheal ACC, with no extra-tracheal metastasis. Adjuvant radiation therapy was administered twice during next year. A follow-up medical examination was performed 5 years later, during which, chest radiography revealed multiple metastatic nodules in both lung fields. The patient subsequently received palliative chemotherapy.\nThe patient was discharged from the army after 3 years of service (1977–1980) and worked in rice farming (1981–1994). In 1995, he entered tire manufacturing, and was assigned to the curing process, where he worked for 10 years, until 2005. The curing process involved the use of 72 vulcanizers (installed with 144 molds). His main duty was preheating and changing molds and bladders. Groups of 3 workers were required to perform the mold-changing job. The workers moved the molds and bladders by using a forklift after the molds had been used several times or if the size of the tire being made needed to be changed (Figures and ). Molds were changed at a frequency of 3 to 5 times a day, and each change required approximately 1 to 2 hours. Preheating the mold required a high temperature of 180°C to 190°C for 4 to 6 hours. The patient was occasionally asked to clean the molds using an airbrush and apply anti-rust agents afterwards.\nAvailable environmental measurement data provided by the company included information from 1997 to 2005, but information for 1999 was missing. The risk factor measured by the company was total dust until 2002. The company then changed to measuring rubber fumes (Table ). During this period, the total dust and rubber fumes were measured to range from 0.03 to 0.91 mg/m3 and 0.03 to 0.57 mg/m3, respectively. The exposure level to total dust was less than 10% of the Occupational Exposure Limits (OEL) determined by the Korean Ministry of Employment and Labor []. The exposure level to rubber fumes was close to 95% of the British OEL (0.60 mg/m3) []. Other potential respiratory carcinogens that the patient might have been exposed to included N-nitrosamines and polycyclic aromatic hydrocarbons (PAHs) []. N-nitrosamines had not been measured until 2000 and had always been less than the detection limit. PAHs were measured to range from 1.4 to 22.6 μg/m3, which was less than 10% of the Korean OEL (200 μg/m3) []. Temperatures from 1998 to 2001 exceeded the limits for medium-grade labor intensity (75% of working, 25% of rest, 28°C) [].\nThere was a possibility of exposure to PAHs vaporized from anti-rust agents applied to molds due to the high temperature. In addition, cleaning the molds with an airbrush might have generated dust from pipe insulation materials. We analyzed a sample of those materials for the presence of asbestos and PAHs. The insulation material was found to contain glass fiber. Major components of the anti-rust agents were found to be mineral oil (CAS NO. 64742-55-8, 90%) and barium sulfate (CAS No. 25619-56-1, 7%); however, when these chemicals were exposed to temperatures similar to those used during the mold preheating process (180‒195°C ), PAHs were not detected, as assayed by the gas chromatography–mass spectrometry (GC-MSD) analytical method. |
A 64-year-old male with a past medical history of metastatic RCC to the thyroid and the lung presented to the emergency department in February 2020 with a 5-month history of nonbloody diarrhea (2-3 bowel movements daily), decreased appetite, 25-lb weight loss, and memory loss. The patient had left nephrectomy and left adrenalectomy in 2004, total thyroidectomy with neck dissection, and parathyroid re-implantation in 2017. Pathology of the latter was clear cell carcinoma with a small focus of follicular variant papillary thyroid carcinoma measuring 0.4 cm, negative lymph nodes. No metastatic lesions were documented in the left adrenal gland pathology. Furthermore, he had radiation therapy to the lung in 2017 followed by chemotherapy with pazopanib since March 2017. The starting dose was 800 mg orally daily and then it was decreased to 400 mg daily afterward.\nThere was no prior history of steroids, opiate, appetite stimulants like Megace, or new medications use since 2017. There was no history of trauma to the abdomen nor any history of autoimmune disease (personal or in the family). Interestingly, the patient had a prior history of type 2 diabetes diagnosed at age 54, requiring more than 50 units of insulin as a total daily dose. However, there was a significant improvement in his blood glucose levels in the past 6 months; hence, insulin was discontinued. Vital signs on admission showed a blood pressure of 115/55 mm Hg, a heart rate of 71 beats per minute, a temperature of 36.5 °C, and a respiratory rate of 18 breaths per minute with an oxygen saturation of 98% on room air. Orthostatic vital signs were positive. Physical examination revealed diffuse skin hyperpigmentation with no significant abnormalities on the abdominal examination. Initial laboratory tests revealed hyponatremia, hyperkalemia, elevated creatinine, and normal blood glucose ().\nOriginally, hypovolemic hyponatremia was suspected; hence, volume resuscitation with normal saline 0.9% was started. Workup for other causes of hyponatremia including endocrine causes such as thyroid function tests and morning cortisol were ordered. The morning cortisol was 5 µg/dL, thyroid stimulating hormone level was 0.23 uIU/mL with normal free thyroxine and total triiodothyronine levels. The patient was on a stable dose of levothyroxine replacement therapy 125 µg for the past year. Adrenocorticotropic hormone (ACTH) stimulation testing was subsequently ordered. Abnormal test results were noticed with baseline cortisol level of 4.9 µg/dL and 60 minutes cortisol level of 6.1 µg/dL, thus confirming the diagnosis of adrenal insufficiency. Notably, albumin was normal at 4.1 g/dL, with ACTH level at baseline at 309 pg/mL and suppressed dehydroepiandrosterone sulfate level. Other workups for pituitary hormones did not show significant abnormalities except for low insulin-like growth factor 1 level (). Notably, magnetic resonance imaging of the head performed 5 days prior to admission did not show any intracranial abnormalities, specifically no pituitary lesions. A recent computed tomography scan of the abdomen showed normal right adrenal and status post left adrenalectomy. Additionally, 21-α hydroxylase antibodies were negative.\nThe patient was treated empirically with high-dose steroids intravenously every 8 hours. Two days later, repeat ACTH level continued to be elevated at 324 pg/mL, confirming the diagnosis of PAI. The steroid dose was subsequently tapered to maintenance physiological daily dose of oral hydrocortisone (10 mg in the morning/5 mg in the afternoon orally). The patient demonstrated significant symptomatic improvement including mental status and appetite with a reduction in nausea, vomiting, and malaise as well as normalization of electrolyte levels. He was provided with education regarding adrenal insufficiency and a follow-up with the endocrine division upon discharge. |
A 73-year-old man was lost consciousness and was transferred to our hospital. The patient had a history of testicular cancer treated with chemotherapy 2 years earlier and was in remission. He was barely able to communicate, and the complaint was unknown. Furthermore, he was in shock with cold extremities. His blood pressure was unmeasurably low, and neither the radial nor the common carotid artery was palpable. However, his limbs were still moving. His Glasgow Coma Scale result was E1V2M4 and his mental status was agitated state. Peripheral intravenous infusion was initiated and transthoracic echocardiography revealed pericardial effusion and almost no systole. Pericardial fluid was hyperechoic, which could signify hematoma formation. Pericardiocentesis was duly considered, but if a hematoma was present, it could have led to insufficient drainage. We decided to initiate ECMO in the emergency ward based on a judgment of obstructive shock or cardiogenic shock. Seven minutes after arrival, cardiopulmonary resuscitation (CPR) was started because of CPA (his breathing and limbs stopped moving), before beginning ECMO. We also considered emergency left thoracotomy, but ECMO was prioritized. We inserted an ECMO catheter under ultrasound guidance. ECMO circulation was started 17 minutes after arrival and 10 minutes after CPA. Twelve-lead electrocardiography (ECG) revealed a QS pattern and ST elevation in leads V1-V5 (Figure ). We did not drain the pericardial fluid, but ECMO flow was stabilized using rapid infusion. The patient was immediately cooled with ECMO and reached a body temperature of 34°C 23 minutes after CPA. He was then transferred for computed tomography (CT) for diagnosis. Contrast-enhanced CT showed no acute aortic dissection (AAD), but the anterior wall myocardium showed poor contrast; hence, we diagnosed LVFWR. According to his family, he had never complained of chest pain. We assumed that the myocardial infarction had occurred 12 to 24 hours before his arrival. No leakage of contrast agent was apparent (Figure ). Blood tests revealed a troponin-I concentration of 13.4 ng/mL (Table ); additional blood test results are shown in the Table . Emergency coronary angiography (CAG) was performed to evaluate coronary artery lesions and to determine surgical procedures. In the CAG images, the #7 branch of the left anterior descending coronary artery (LAD) was completely occluded, and the #4 branch of the right coronary artery provided collateral circulation to the LAD (Figure ). There was no other significant stenosis, and no rupture point could be found on left ventricular angiography. An intra-aortic balloon pump (IABP) was inserted under fluoroscopy, and the patient was immediately transferred to the operating room for surgical hemostasis.\nUnder general anesthesia, a skin incision was made from the sternal notch to the xiphoid process, and the pericardium was exposed by a median sternotomy. Leakage of bloody pericardial fluid was observed when the pericardium was incised. The pericardial fluid was aspirated to remove the blood clots in the pericardium, and oozing blood was observed near the cardiac apex (Figure ). The myocardium around the bleeding point had a poor color tone, but no myocardial necrosis was observed in other areas. A total of five pieces of TachoSil® (Takeda Pharmaceutical Company Limited) were attached, focusing on the bleeding site, and hemostasis was confirmed (Figure ). We did not perform a coronary artery bypass graft (CABG) for the affected LAD because we believed that it would promote intramyocardial bleeding, which could lead to the development of blowout rupture and perforation of the interventricular septum. A towel was inserted into the pericardium to cover the bleeding area. Next, substernal and intrapericardial drains were inserted, and a 20-cc syringe was modified and used as a sternal bridge to prevent bony union. Four pieces of gauze were also inserted into the subcutaneous space. The skin was not sutured, but instead was covered with an Esmarch tourniquet and sutured, and a sterile drape was applied. Negative pressure was applied to the drain, and the chest was temporarily closed with a vacuum pack. Surgery was performed consistently with only ECMO support and without cardiopulmonary bypass. The intraoperative blood pressure averaged 100/60 mm Hg, and the pulse was approximately 60-90 beats/minute. The lowest blood pressure recorded was 80/45 mm Hg. The total blood transfusion volume included 280 mL red blood cells and 600 mL platelets; fresh frozen plasma was not used.\nConsidering the possibility of recurrent cardiac tamponade, the patient was admitted to the intensive care unit (ICU) with temporary chest closure. There was marked hemorrhagic drainage from the substernal and pericardial drains, and blood products were supplemented to stabilize the hemodynamics. We continued to use 34℃ TTM after surgery. If hemostasis could not be obtained, we would switch to 36℃ TTM in consideration of the effect on coagulation ability. We maintained his body temperature at 34℃ for 24 hours, and then rewarmed to 36℃ over the next 48 hours. From the start of rewarming, cardiac function and wall movement, other than the anterior wall, gradually improved. The ECMO flow was weaned to 1 L/minute as the cardiac function improved. Under these conditions, the blood pressure was maintained at 130/80 mm Hg and the pulse rate at 80/minute, and ECMO was subsequently withdrawn after the clamp test. After which, ECMO was discontinued, but the consciousness disorder persisted. On the sixth day of hospitalization, the patient was able to communicate. It was judged that the left ventricular assisting effect of the IABP had diminished, and we removed the device. On the same day, the amount of urine started to increase and diuresis began. Because edema of the mediastinal tissue and myocardium was controlled, we closed the chest incision on the 9th day of hospitalization. The patient was extubated on the 14th day of hospitalization, and on the 15th day of hospitalization, he was transferred to the general ward without neurological deficits. He is currently receiving rehabilitation.\nAll subjects enrolled in this research have given their informed consent, which has been approved by the institutional committee on human and/or animal research, and this protocol has been found acceptable by them. |
A 48-year-old Hispanic male presented to our institution after being diagnosed with PAM six years prior via surgical lung biopsy during a video-assisted thoracoscopic surgery (VATS) procedure. He became oxygen-dependent a year after. After three years, the patient underwent a lung transplant evaluation but did not receive one due to a history of alcohol abuse, lack of social support, and poor esophageal motility. More recently, the patient was hospitalized multiple times for respiratory failure requiring high flow oxygen and steroids. At presentation, the patient was on 10 L oxygen and still experiencing dyspnea at rest. He also endorsed periodic sharp chest pain with exertion, peripheral edema, and fatigue. Medical history was notable for pulmonary artery hypertension treated with sildenafil, ambrisentan, and Lasix. He also had a history of insulin-dependent diabetes, chronic pancreatitis, gastroesophageal reflux disease (GERD), hypertension, and alcohol abuse. Family history was notable for two sisters, out of a total of eight siblings, who have also been diagnosed with PAM with a presumed genetic origin, though so far, the patient refuses to get tested, and further information is unavailable. These siblings have been controlled with medical treatment and had not had lung transplants.\nFollowing the presentation to our institution, the patient was evaluated and referred to the lung transplant program. Pulmonary function testing demonstrated a normal FEV1/FVC ratio with decreased FEV1, FVC, and TLC. The patient was unable to remove his oxygen supply long enough to perform DLCO. Chest X-ray demonstrated the classic “sandstorm” appearance of severe bilateral interstitial hyperdensities with a basilar predominance (). A chest CT scan showed chronic interstitial lung disease with diffuse ground-glass opacities, peripheral cystic changes, and numerous areas of calcification ().\nThe patient underwent a bilateral lung transplant. A successfully treated pneumonia complicated his immediate postoperative course, and he went home three weeks after surgery. Gross examination of the explanted lungs demonstrated anthracotic pleural surfaces with diffuse fibrous adhesions. Upon sectioning, the parenchyma showed diffuse fibrosis and bilateral diffuse calcifications. Histologic analysis revealed numerous bilateral intra-alveolar laminated eosinophilic bodies in association with marked interstitial fibrosis. Multiple foci of osseous metaplasia and chronic inflammation with germinal center formation were identified (Figures and ). These changes were more prominent at the bases, although involvement was diffuse and bilateral.\nMultiple readmissions complicated the patient's posthospitalization course. Nine days after his initial discharge, he presented with fever and cough. Workup showed pleural effusions and evidence of sternal dehiscence. Bronchoscopy showed anastomotic necrosis, and biopsy showed evidence of minimal acute rejection (grade 1A). The patient underwent sternal hardware replacement. One week following discharge, he presented with hypoxemia during pulmonary rehabilitation therapy and was readmitted. A chest CT was suspicious for pneumonia. Repeat biopsies showed patchy perivascular chronic inflammation with no acute rejection (grade 0). However, C4d immunostaining showed strong linear staining of several small interstitial vessels, and the patient tested positive for donor-specific antibodies with increasing mean fluorescence intensity (MFI) for HLA-DQ7. The patient was given IVIG and underwent five rounds of plasmapheresis. Repeat biopsies following plasmapheresis were negative for evidence of rejection. FEV1 and FVC have both improved from pretransplant levels. DQ7 MFI has decreased significantly, but the patient remains on maintenance intravenous immunoglobulin (IVIG).\nDue to PAM's family history and known familial nature of this disease, genetic testing was recommended but has not been completed at the time of this publication. |
A 40-year-old man was involved in a rollover motor vehicular accident and sustained polytrauma. His injuries included a left scapular comminuted fracture, multiple rib fractures, pulmonary contusions, a left hemothorax, pelvic bone fractures, and a proximal descending aortic. His initial Glasgow Coma Scale was 15. An initial head and cervical spine CT was negative for intracranial or spinal pathology. Three days after the accident, he was transferred from an outside hospital for a stent graft procedure for the aortic injury. A few days after stenting, he developed a pulmonary embolism which led to anticoagulation. He was awake and moving all four extremities to command at this time. Two weeks after the accident, he became acutely quadriplegic. A head CT demonstrated a hyperdense lesion at the anterior foramen magnum compressing the brainstem. A cervical spine MRI scan showed an acute intra- and extradural hematoma in the region of the foramen magnum causing a mass effect on the medulla (). The ligament between the tip of the dens and the base of the skull was edematous and discontinuous, consistent with a rupture of this ligament. In a preoperative CT of the head and cervical spine (), the basion-dens interval was 15.41mm. The Power's ratio was 1.054 without a fracture. There was vertical displacement of the occiput and atlas. He was diagnosed with Type II AOD. It was thought that the AOD resulted in the epi- and subdural hematomas, brain stem compression and quadriparesis. He was taken to the operating room emergently for decompression and stabilization using the same technique of direct internal occipito-atlanto-axial fixation with placement of occipital, C1 lateral mass, and C2 pedicle screws as described in the previous case with additional lateral mass instrumentation at C3.\nThis patient was then placed in a halo vest for only 2 months. Thirteen months after surgery, he was painless and is now neurologically intact. Lateral cervical spine radiography showed atlanto-occipital (AO) fusion with good bony growth (). |
A 39-year-old male patient presented at our emergency room after a traffic accident and the brain computed tomography (CT) scan indicated an incidental arachnoid cyst on posterior fossa. At that time, the patient complained only of facial abrasions and the left knee pain, but not of headache or visual disturbance. In eight months, however, he revisited our Department of Ophthalmology to check the decreased visual acuity, visual field defect, and headache. On the ophthalmologic examination, bilateral papilledema (), decreased visual acuity, and visual field defect () were found. After the transfer to the Department of Neurosurgery, we rechecked brain CT scans (). The diameter of the patient's arachnoid cyst did not change compared to the previous CT scans; however, the slightly enlarged third ventricle was compressing the optic chiasm and the suprasellar area. Since visual complications were expected, we recommended an emergency operation. However, the patient refused the operation for personal reasons. One week later, he came back to the hospital with nearly complete visual loss. His right eye was completely blind, whereas his left eye recognized only light ().\nOn that day, he underwent an emergency operation. A conventional suboccipital craniectomy was performed on the patient lying on the operating table in the prone position. After the dural incision, the arachnoid cyst was exposed and cystic fluid was expelled. Arachnoid membrane was carefully dissected and the arachnoid cyst was fenestrated to cisterna magna for connecting with the cerebrospinal fluid (CSF) flows. Arachnoid membrane which covering the foramen of magendie was also fenestrated and the CSF flows was running properly (). Excluding the brain tumor, the collected membrane was sent to the pathologic laboratory. In high power field microscopy under the hematoxylin and eosin stain, the cystic membrane was composed by membranous fibrocollagenous tissues without tumor cells. Finally, the patient was diagnosed as arachnoid cyst ().\nOne week afterwards, we checked the brain CT scans and the removal of the cyst and the decreased ventricle size were confirmed. On the ophthalmologic examination, the papilledema and visual field were slightly improved; however, the patient's visual acuity did not improve. Upon the tenth day after the operation, the patient was discharged and, during the next one month, he was subject to the steroid therapy.\nIn five months after the operation, papilledema (), headache, and radiological images () quickly returned to the normal ranges. Nevertheless, visual acuity and visual field improved slowly and slightly (). The blind right eye improved to recognize the light and the blurred left eye improved to acknowledge the silhouette of an object; thus, it did not fully recovered to the prior, normal condition. |
A 41-year-old male patient, weighing 72.2 kg and measuring 168 cm tall, visited the hospital with his main complaint being a fracture of the right calcaneus and an L4 radius compression fracture that he had received after falling from a 7 m bridge. On the initial visit, the patient underwent catagmatic surgery for the open fracture. Slow wound recovery was observed because of a considerable volume of exudate and a hemorrhagic tendency. The coagulation-hemostasis test showed that the prothrombin time (PT) was 14.0 seconds, which was within the normal range (11.5 to 15 seconds), and the activated partial thromboplastin time (aPTT) was 180 seconds, which was longer than the normal range (28 to 45 seconds). The coagulation factor test performed in the Department of Hematology and Medical Oncology showed that the activity of coagulation factor IX in the patient's blood was as low as 27%; therefore, the patient was diagnosed with hemophilia B. The wound management was performed by continuing to use a coagulation-hemostasis test, regular injections of vitamin K to enhance coagulation factor synthesis, proper wound disinfection, and antibiotics. The patient was preoperatively injected with recombinant coagulation factor IX (Wyeth Inc., Seoul, Korea; 40 IU/kg IV) one day before the operation. One hour before the operation, an injection with the same amount of recombinant coagulation factor IX was administered. Botropase (Hemocoagulase 1N1HU), a hemostatic, tranexamic acid, and vitamin K were also intraoperatively injected. Because one IU of coagulation factor IX per 1 kg of body weight can increase the coagulation factor IX activity by 1%, the required quantity of the coagulation factor IX was calculated as follows: Factor IX dose (IU)=Desired factor activity (%)×weight (kg). Because of the wide dissection area, the dose given to the patient was calculated according to the medical guideline that states that the coagulation factor activity is increased by 50% to 80% before a major operation such as abdominal surgery, brain surgery, or orthopedic surgery [].\nFollowing general anesthesia, the patient was moved into a prone position. An open wound measuring 5×4 cm, a part of the calcaneus, and the Kirschner wire used on the initial visit to the hospital, were exposed. The expected incision line was drawn including the margin of the wound. After performing the debridement, the island flap was drawn at the lower-third of the leg to be fitted to the area from which the soft tissue had been removed (6×5 cm). The vascular pedicle included 2-3 cm wide fasciae, the sural nerve, and the accompanying sural artery. The small saphenous vein was included to make the venous return easier. The length of the vascular pedicle was calculated by adding 1-2 cm to the length between the proximal boundary of the deleted region and the pivot of the flap. It was verified that the vascular pedicle was not compressed. No vasogenic hemorrhage at the arteriole or venule was observed during the operation.\nThree vials of BeneFix (1 vial: 1,000 µ/10 mL), a hemostatic, and vitamin K were injected together intraoperatively, on the day of the surgery, and continuously injected on the day after the surgery. Two days after the operation, the BeneFix (1 vial) bid was maintained. While the patient remained under follow-up care, the bloody exudate and hematoma had increased. Hemostasis and hematoma removal were performed on postoperative day (POD) 5 and again on POD 7. On POD 7 the dose of BeneFix was increased to 4 vials and the packed red blood cells of 3 pt were administered. The bloody exudate was continuously observed even after the second hematoma removal, but the quantity had decreased and the hematoma was no longer observed (). The BeneFix dose was maintained at 2 vials qd for one week, the catamnesis was observed, and a hemostatic and vitamin K were continuously injected. The recombinant coagulation factor IX was injected on the 17th day after the second hematoma removal operation. The follow-up coagulation-hemostasis test showed that the normal range level had been maintained (). Thereafter, hematoma, hemorrhage, and other infections and complications were not found. Ongoing hematologic tests showed that the results were in the normal range and the wound recovered without any specific complications. |
A 27-year-old male presented to the Oral and Maxillofacial Surgical Unit of Saraswati Dhanwantari Dental College and Hospital, Parbhani, in October 2017 with a single large swelling in the submental region. The patient had a history of hematoma due to unknown impact 5 years back, and a painless tiny mass below 1 cm was observed under the chin with gradual progression during 1 year. On examination, the swelling was approximately 4 cm × 3 cm × 2 cm, freely movable without any fixation to the underlying or overlying structures and was nontender and doughy in consistency with no lymphadenopathy on palpation. Intraorally, there were no relevant dental findings, and the tongue movements were normal [].\nAspiration cytology revealed 0.5 ml-thick semifluid material, which was fixed with hematoxylin and eosin, PAP, and Giemsa stains. Microscopic examination revealed areas of blood elements, clusters, and singly dispersed benign enucleated squamous cells. These features were suggesting a diagnosis of dermoid cyst. Ultrasonography revealed a well-defined thin-walled homogeneous hypoechoic lesion measuring 25 mm in the submental region. Vascular lesions were excluded on color Doppler study. There was no evidence of any communication with the thyroid gland, and features were suggestive of a benign cystic lesion. Correlating the above findings, a provisional diagnosis of dermoid cyst was established.\nThe patient was informed about the surgical procedure of cyst enucleation under general anesthesia and the chances of postoperative scar in the submental area. Thereafter, the procedure was carried out with a written consent of the patient.\nEnucleation of the cyst was performed under general anesthesia using transcutaneous approach with a skin incision over the submental region followed by dissection thoroughly over the subcutaneous plane and platysma muscle and protecting the anatomical structures. The capsule of the cyst was completely removed with external traction and blunt dissection, and surgical wound was closed layer by layer with absorbable and nonabsorbable sutures [Figure and ].\nPostoperative histopathological examination of the excised specimen showed a fibrocollagenous cyst wall with prominent granular layer along with scattered chronic inflammation, lumen of cyst filled with flakes of lamellated keratin, and occasional giant cells noted within the stroma, supporting a diagnosis of dermoid cyst [].\nPostoperative recovery was uneventful, and the patient was discharged on the 2nd day with oral antibiotics prescribed for 5 days with frequent review. Postoperative 6th month review showed no signs of recurrence. |
An 18-year-old female presented with a painful slowly expanding swelling of left maxilla that had started approximately 3 months earlier []. She also complained of restricted mouth opening, nasal discharge, and lacrimation on the left side. On extra-oral examination there was an obvious swelling on the left side of the cheek and mild proptosis of the left eye. Intra-oral examination showed an expansive mass in the upper left premolar and molar region with sulcus obliteration. The overlying mucosa was slightly bluish purple in color and firm in consistency. There was also expansion of the palate. Clinically Grade II mobility of teeth 25, 26, and 27 were noted.\nComputed tomography scan revealed a soft tissue mass completely obliterating the left maxillary sinus. The mass extended inferiorly into the body of the maxilla up to the alveolus, medially it obliterated the nasal cavity. Superiorly, it extended up to the floor of the orbit and posteriorly, it reached the pterygoid plates. Root resorption of teeth 25, 26, and 27 was evident indicating the aggressiveness of the lesion [Figures and ].\nAn incisional biopsy was performed under local anesthesia. Tissue was removed from intraosseouss lesion located in the alveolar ridge with a surgical curette and hemostasis was achieved and primary closure done. The specimen was submitted to the Oral and Maxillofacial pathology department. The histopathplogical examination showed diffused distribution of giant cells and confirmed an aggressive GCG [].\nThe patient was an young unmarried female and as aesthetics was a major concern, an intra-oral approach was planned as opposed to the conventional extra-oral approach. Since the lesion was quite extensive involving the whole of maxillary antrum extending upto left ethmoidal sinus, orbital floor and pterygoid plates, it was decided to perform an Unilateral (left) Le Fort I osteotomy to gain complete access to the entire lesion. An intra-oral vestibular incision was made extending from the midline to the left second molar region. The underlying bone was exposed and Le Fort I cuts were performed, followed by mid-palatine split and down fracture of the left side maxilla []. This provided excellent visibility and access to the entire lesion.The lesion was excised with the help of surgical curettes and the entire bony margins were visualized for any remnants []. All the mobile teeth on that side were extracted. Hemostasis was achieved and then the ostetomised maxilla was reduced and fixed with the help of a 4-hole ‘L’ plate and screws made of Titanium []. Patient was checked at regular intervals with radiographs [] and CT scans []. Patient had no recurrence of the lesion during a 2-year follow-up period. |
A 74-year-old woman was referred to us for refractory heart failure and perioperative management of a left femoral neck fracture. Her past history was remarkable for mitral and aortic valve replacement (Hall–Kaster mechanical valves) for severe rheumatic mitral stenosis and aortic regurgitation at the age of 49 years. She developed RHF because of TR and had isolated TVR with a 27-mm CE bovine pericardial valve at the age of 64 years (15 years after the first surgery). She had been stable for 10 years; however, she developed refractory leg oedema at 74 years of age. She was treated medically in another hospital. Three months before the referral, she suffered a left femoral neck fracture in a fall; nevertheless, surgical treatment was declined in another hospital because of high operative risks. However, she strongly desired surgical treatment and was, therefore, referred to us. The initial evaluation revealed an emaciated patient with severe leg oedema and elevated JVP up to her jaw level in the seated position. Pan-diastolic rumble was audible at the 4th left sternal border. Laboratory data revealed severe hypoalbuminaemia of 2.8 g/dL. Evaluation revealed protein-losing gastroenteropathy because of high venous pressure. Echocardiography revealed severe bioprosthetic TS with TVG as high as 20 mmHg (Figure and B). Because of poor nutrition, redo TVR was deemed to be too risky. Therefore, the patient was treated with the less invasive balloon tricuspid valvuloplasty before the hip replacement surgery, and we expected improvements in her haemodynamic and nutritional condition (Figure ). The right atrial pressure decreased from 18 to 13 mmHg, and her general condition improved (Figure and E). After the balloon valvuloplasty, she underwent successful left hip replacement surgery without complication. However, she developed refractory RHF because of recurrent worsening of TS 8 months later. After the second balloon valvuloplasty (requested by the cardiac surgeons as a bridge, Figure ), redo TVR with a SJM tissue valve was finally performed. Examination of the explanted valve revealed the following findings (Figure ). X-ray examination showed moderate calcification along the free margin of the leaflets. Heavy pannus (host tissue overgrowth) was observed in both atrial and ventricular aspects. All the commissures appeared to have been fused by the pannus, and the fused commissures were partially split by the balloon valvuloplasty. |
A 9-year-old female leucoderma patient presented to the stomatology department of a public hospital in Rio de Janeiro, Brazil. She complained of small nodules in the left parotid region that had developed over the course of 2 years. Her main complaint was of recurring periods of worsened symptoms characterized by the exacerbation and remission of gland volume that was possibly triggered by occasional otolaryngologic infections or unrelated to these infections. These symptoms suggest juvenile recurrent parotitis. Facial panoramic radiography revealed the presence of multiple circular radiopaque masses in the left parotid region (Fig. ). The ultrasound revealed increased volume of the left parotid, with imprecise borders, heterogeneous echotexture with hypoechoic and hyperechoic areas within it. These features were suggestive of an inflammatory process associated with calcifications in the parenchyma of the gland. CT scan revealed a dense mass in the left parotid; it was heterogeneous and included calcifications in its center (Fig. ). Because of the association between the patient’s clinical history, her clinical presentation, and the imaging findings, the possible origin of the calcified materials was questioned. There was evidence of sialoliths or dystrophic calcification associated with recurrent inflammation/infection. Sialoliths are typically symptomatic because of their association with secondary bacterial infections, which are generally treated with systemic antibiotic therapy. Spontaneous remission of bacterial sialadenitis associated with sialoliths is not expected. In addition, sialoliths generally observed as oval-shaped calcified masses or fusiforms on imaging. Because of the pediatric nature of this case, the clinical conduct selected to treat this patient was clinical follow-up and the use of imaging and functional assessments of the gland affected every 6 months or when any signs and/or symptoms appeared. After 48 months, the patient is asymptomatic, without periods of exacerbation of the condition. Recent ultrasound (Fig. ) demonstrates an improvement in the inflammatory aspect of the gland. Clinical and imaging follow-up will be maintained. |
A 65-year-old gentleman presented to the out-patient department with three-month history of continuous, dull aching pain in the left iliac region, radiating to the left thigh. He did not have constitutional symptoms like fever, weight loss, and loss of appetite. Initial examination revealed a 3 × 4 cm tender swelling with indistinct margins localized over the lateral aspect of the left iliac crest. There was increase in temperature and erythema. General examination was unremarkable except for pallor. There was no localized or generalized lymphadenopathy. Blood investigations revealed normal haemogram, ESR of 20 mm/hour and C-reactive protein of 34 mg/dl. Plain radiographs revealed an osteolytic lesion with indistinct margins, cortical breach, moth eaten appearance, and narrow transitional zone (). Bone scan showed isolated uptake in the left ilium. In view of age and location, differential diagnosis included primary bone tumor and indolent infection. A needle biopsy was performed. The histopathological examination showed trabeculae of cortical, cancellous, and scanty necrotic bone with intervening inflammatory granulation tissue densely infiltrated by lymphocytes and histiocytes consistent with nonspecific chronic osteomyelitis (). No tumor markers or special strains were done. In spite of the biopsy suggesting chronic osteomyelitis, the cultures were negative for mycobacterium, fungal elements, and bacterial elements. Further, there was no conclusive clinicoradiological correlation. Hence, it was suggested that the patient underwent an open biopsy proceed excision of the iliac lesion. However, the patient decided to defer any surgical procedure. Hence, he was started on a course of empirical antibiotics and advised to review regularly.\nAt his follow-up visit, the patient appeared pale and had significant loss of weight. The swelling had increased to size of 10 × 10 cm, with extent up to the left inguinal region. The left lower limb was edematous. There was no localized lymphadenopathy. Hip movements were painfully restricted. Plain radiographs of pelvis showed a permeative lesion with significant periosteal reaction over the left iliac crest (). The chest radiograph, computed tomography of chest, and ultrasound abdomen were negative for any lesion. An open biopsy was performed revealing cores of scanty partially crushed tumor composed of sheets of medium-sized round cells with round to oval nuclei, small nucleoli, scant to moderate amounts of cytoplasm, and areas of necrosis. Immunohistochemistry revealed tumor cells that were positive for CD20 and Bcl-6 and negative for Tdt, Bcl-2, MUM-1, CD5, CD23, and CD3. A diagnosis of high grade B cell Non-Hodgkin's lymphoma was made (Figures and ). Medical oncology opinion was obtained and the tumor was staged as Ann Arbor Ia–Extra nodal, with risk stratification being low-intermediate. The patient was then planned for a combined treatment modality with chemotherapy and radiotherapy. |
During the routine upper limb dissection for undergraduate medical students, the authors spotted an unusual constellation of upper limb arterial, muscular and nervous variation in a 65-year-old female of Indian origin. In the present case, SBUA was noticed bilaterally. On the left side, SBUA was originating from the third part of axillary artery proximal to the origin of anterior and posterior circumflex humeral arteries; on the right side, SBUA was originating from brachial artery just below the origin of profunda brachii artery and above the level of insertion of coracobrachialis (, ). In the arm bilaterally, the SBUA was running medial to the normal brachial artery but in a more superficial plane. In the cubital fossa, SBUA was superficial to the pronator teres muscle and continued as the superficial ulnar artery on both sides. In the forearm, SBUA continued its medial course superficial to the flexor muscles. In the distal part of the forearm, SBUA passed deep to the tendon of palmaris longus and palmaris profundus and then was medial to the palmaris longus tendon in the wrist. Its contribution to the superficial palmar arch was similar to that of the normal brachial artery bilaterally.\nOn the right side, palmaris longus also had a rare variation of a fleshy belly instead of a long tendon (, ). Authors noticed Palmaris profundus originating from the posterior aspect of the epimysium of the palmaris longus and a few of its fibers arising from the common flexor origin. As the name implies, palmaris profundus belly was deep to palmaris longus, and the tendon of palmaris profundus passed deep to flexor retinaculum and was then inserted into the under surface of the palmar aponeurosis (). In the carpal tunnel, it was accompanied by the median nerve. Palmaris profundus received nerve supply from the anterior interosseous nerve. Palmaris longus in the left side had the characteristic long tendon. In the left side, a few fibers of musculocutaneous nerve joined the median nerve below the level of the insertion of the coracobrachialis. Bilaterally, the brachial artery divided into radial artery and common interosseous branch at the level of the neck of the radius. Further course of radial and common interosseous arteries was normal. |
The patient was a 73-year-old man who was referred to our hospital from the local hospital for the evaluation of dizziness, weakness of the lower extremities, and blindness of the left eye. The symptoms were first noticed 20 days before admission, after a walk. His blood pressure at that time was 180/100 mm Hg (≤140/90 mm Hg). He had been diagnosed with hypertension and coronary heart disease one month before the episode, with the highest blood pressure recorded of 200/110 mm Hg; however, he did not receive any treatment. He was a habitual smoker, smoking approximately 900 cigarettes a year. With regard to family history, he reported that his father had died of some form of brain disease.\nThe findings on physical examination at the time of admission to our hospital were as follows: body temperature 36.5°C (36.3-37.2°C); pulse rate, 60 beats/min (60-100 beats/min); respiratory rate 19 cycles/min (16-24 cycles/min), and blood pressure 200/100 mm Hg (≤140/90 mm Hg). He was conscious, with normal verbal fluency, but cognitive decline. Examination of the eyes revealed symmetry of the eyelid fissures and normal eye movements on both sides, without nystagmus. The diameter of the left input was 4.0 mm and of the right pupil was 3.0 mm, with a slow response and high sensitivity to light. The visual acuity by left eye was limited to hand movement only. Muscle strength of the upper limbs was of grade 5 and that of the lower limbs was 4+, with normal muscle tone and tendon reflexes (++) in both the upper and lower limbs. The Babinski sign was positive on the left side and negative on the right side. When the Romberg test was performed, he had imbalance on opening or closing the eyes. No abnormalities were detected on examination of the chest and abdomen, and no edema was noted in the lower extremity. He had arrhythmia and premature beat, but no pathological murmurs were detected on auscultation of the valve area. Vascular sonography of the neck revealed a bilateral increase in the intima-media thickness of the carotid artery and bilateral plaque formation in the carotid arteries. Echocardiography revealed: aortic regurgitation, reduction in the magnitude of the motion of the left ventricular anterior wall, left ventricular diastolic dysfunction, and arrhythmias. No abnormalities were detected on transcranial Doppler ultrasonography. Transesophageal echocardiography showed patchy, hypoechoic areas in the aortic arch. The Holter exam showed sinus rhythm, with junctional escape beat and wandering heart rhythm. The average heart rate was 61 beats/min (range, 46-87 beats/min), with continuous ST-segment depression and T-wave flat changes. The serum levels of brain natriuretic peptide level was 147.5 pg/mL and homocysteine was 23.43 µmol/L. The results of other routine blood, urine, and feces tests were normal. The findings of cranial MRI, MRA, and fundus photography are shown in . |
A 34-year-old man with KTS and no surgical history was referred to our hospital with
severe knee arthropathy that had been worsening over the preceding months. He
mentioned stiffness of the left knee and inability to perform flexion-extension
movements, to the point of being unable to walk on his own, and rated the pain as 10
out of 10 with poor response to analgesics.
His physical examination was remarkable for the significantly larger diameter of the
left leg, with extensive palpable varicose veins and changes to the skin consistent
with venous stasis. The position of his knee joint was fixed, with movement
restricted from 75 degrees in extension to 90 degrees in flexion (). Discrepancies in leg length and
diameter were difficult to measure due to flexion contracture. Neurologic findings
were normal and while the dorsalis pedis and posterior tibial arteries were difficult
to palpate, the extremity was well perfused.
A Doppler ultrasound examination (DUS) showed absence of venous obstruction and
normal arterial flow. CT Angiography of the leg evidenced important varicose
development of the superficial venous system, increased soft tissue component, and
bone hypertrophy with marked thickening and cortical irregularity in the fibula and
the distal part of the femur. Magnetic resonance imaging (MRI) showed intraarticular
varicose vessels as well as intramuscular location in the biceps femoris and
semimembranosus and involvement of sciatic nerves, with no alterations of joint
structures ().
The case was presented to a multidisciplinary committee including vascular and
orthopedic surgeons. Based on the benign prognosis and the high morbidity of surgical
treatment, conservative management was chosen as the first option. The patient was
enrolled on a physiotherapy program, which consisted of attending a rehabilitation
clinic, twice a week initially and then twice a month after the ninth month of
treatment, where he performed strengthening and functional exercises to improve range
of motion and joint stability |
A man who was 42-years-old in 1997, was admitted with a chief complaint of abdominal pain, and was later diagnosed with colon cancer. He was treated with sigmoid colon cancer radical surgery on 23 June 1997. Postoperative histopathological analysis revealed sigmoid colon cancer graded as Dukes C1 (Fig a). The patient did not receive any chemotherapy or radiotherapy after the surgery.\nIn October 2003, the patient again presented with abdominal pain and colonscopy revealed colon cancer. He underwent radial ascending colon carcinoma sugery in 08 October 2003. Histopathological analysis demonstrated moderately and poorly differentiated adenocarcinoma of the ascending colon invading to the outer membrane (Fig b). Following surgery, the patient was then treated with oral Carmofur 150 mg t.i.d.\nIn April 2009, the patient was admitted to the First Affiliated Hospital of Zhejiang University School of Medicine with gross hematuria, which was later diagnosed as bladder cancer. The patient underwent right pelviolithotomy combined with excision of the remaining right ureter and the bladder cuff. Postoperative histopathological analysis revealed (bladder, right ureter and right renal pelvis) multiple invasive stage II urothelial papillary carcinoma (Fig c). The patient was treated with infusion chemotherapy (Doxorubicin) following surgery.\nIn July 2010, the patient again complained of gross hematuria and was subsequently admitted to People's Hospital of Jinyun Town. He received a partial bladder resection and postoperative pathology subsequently indicated invasive high-level papillary urothelial carcinoma of the right wall of the bladder (Fig d).\nIn September 2011, a mass (2 × 2 cm) which was stiff and fixed in the left neck was discovered. With no treatment, biopsy of the mass was taken one month later (23 October 2011) at the Cancer Hospital of Fudan University. Pathological study showed metastatic carcinoma with necrosis (Fig d). Chest CT scan showed a mass (5.9 × 5.0 cm) in the right upper lobe, which was suspicious of a malignant tumor (Fig a–c). Radiation therapy was commenced after 27 December 2012. On 25 November 2013, the patient died of multiple organ dysfunctional syndrome (MODS). |
A 59 year old male presented with a 2 week history of progressively worsening exertional dyspnea (NYHA class II), bilateral lower extremity edema and abdominal distension. He denied any fever, chills, chest pain, cough, orthopnea, paroxysmal nocturnal dyspnea, palpitations, nausea, vomiting, abdominal pain, syncope or weight loss. He presented to the Emergency Room after his cardiologist found a right atrial mass on echocardiogram during an office visit. His past medical history was significant for treated colorectal cancer, hyperlipidemia and gout.\nThe patient was diagnosed with colonic cancer 5.5 years earlier for which he underwent surgical resection with right hemicolectomy. The patient did not receive any adjuvant chemotherapy or radiation. He continued to follow up with his primary oncologist with surveillance CT scans and had completed his 5 year surveillance.\nFamily history was significant for colon cancer in his uncle and breast cancer in two of his sisters. He denied any tobacco use or alcohol intake.\nThe physical examination was remarkable for decreased lung sounds at the lung bases, moderate ascites and bilateral 1+ pitting edema up to the knees. EKG and cardiac enzymes were unremarkable. The patient underwent a CT angiogram of the chest which revealed a 4.0 cm low attenuating right atrial mass. The lung parenchyma had some ground-glass opacities bilaterally which were thought to be related to motion artefact or less likely suggestive of pulmonary edema. Deep venous thrombosis was ruled out with a negative lower extremity Doppler. The following day, he underwent paracentesis which showed a Serum Ascitis Albumin Gap of 1.4 and 726 nucleated cells (41% lymphocytes, 54% macrophages and 5% neutrophils). Cytologic examination was negative for malignancy, with findings notable for mesothelial cells and macrophages. Cardiac MRI showed a 8.3 cm × 6.5 cm right atrial mass extending from the suprarenal IVC into the hepatic veins and occupying the majority of the right atrium. The mass was noted to be isointense to myocardium on T1 weighted imaging, hyperintense to myocardium on T2 weighted imaging; with positive uptake of Gadolinium contrast in the peripheral segments of the tumor on first pass perfusion imaging, and had heterogeneous uptake on Late Gadolinium Enhancement imaging. A small circumferential pericardial effusion was also seen. Other laboratory studies were majorly unremarkable; with a normal CBC, CMP, PSA and serum CEA levels. The major differentials at this point were atrial myxoma, sarcoma or metastatic disease. It was believed that the shortness of breath was liked related to the increased right atrial pressures.\nCardiothoracic surgery performed an echocardiographic and fluoroscopic guided biopsy of the right atrial mass. The biopsy showed metastatic moderately differentiated colonic adenocarcinoma (Fig. ). Mismatch repair protein expression analysis by immunohistochemistry showed no loss of MLH1, MSH2, MSH6 or PMS2 expression. No RAS, HRAS, NRAS or BRAF mutation was detected on next generation sequencing. MRI of the brain was negative for metastasis. However, MRI bone scan showed that the patient had a low T1 signal intensity in the left distal clavicle that was suspicious for metastatic disease. PET/ CT, was performed to evaluate if the disease was oligometastatic, confirmed metastatic disease in the left distal clavicle with a pathological fracture. The right atrial mass was also noted on PET/CT with abnormal uptake (SUV of 11). Treatment was initiated with plans for 4 cycles of FOLFIRINOX/ bevacizumab. Radiation therapy to the distal clavicle was deferred at this point pending response to chemotherapy.\nColonoscopy was performed during the hospitalization to rule out a second primary colorectal carcinoma. It showed a patent end to side ileo-colonic anastomosis with no endoscopic evidence of colon cancer was noted in the entire colon.\nOn his clinic visit to receive his second cycle of chemotherapy, he was noted to be doing much better with his symptoms of dyspnea and lower extremity edema being significantly better. However, he noted that the left clavicle was a little more painful, so oral opioids were added. At this point, radiation to the clavicle was planned. Repeat echocardiogram performed a month later showed a decrease in the size of the right atrial mass from 4 cm to 3 cm. On his most recent clinic visit, he continues to be asymptomatic and tolerated cycle 7 of chemotherapy with bevacizumab. A cardiac MRI is pending for him with plans to attempt surgical removal by the cardiothoracic surgery team if the tumor has regressed to the supra-diaphragmatic area. |
A 51-year-old woman presented with fatigue, nausea, and vomiting for three days. She was diagnosed with metastatic melanoma to pelvic nodes in July 2014 and was treated with ipilimumab (anti-CTLA-4) in combination with nivolumab (PD-1 inhibitor) between September 2014 and April 2015, receiving a total of 4 cycles of combination therapy and 9 cycles of nivolumab monotherapy with an initial mixed response followed by slow progression of disease. In April 2015 she was enrolled on a study of radiation (to pelvic mass) in combination with pembrolizumab (PD-1 inhibitor), receiving a total of 9 cycles of pembrolizumab with no toxicities. In December 2016 she was found to have new brain metastases and in January 2017 pembrolizumab was added. The day following her second dose of pembrolizumab, she developed fatigue and nausea and began having intermittent vomiting and diarrhea. Abdominal CT scan demonstrated diffuse colitis. Infectious studies including C. difficile antigen, stool culture, viral PCR, and ova and parasites exam were all negative. She was started on methylprednisolone 2m/kg/day. Over four days of hospitalization, her abdominal pain worsened and she developed melena, which progressed to bright red blood per rectum. She was given infliximab at 10mg/kg. Repeat imaging performed 48 hours later due to severe abdominal distension showed large amounts of free air with gaseous distention of large and small bowel loops, consistent with perforation in the context of colitis with ileus. She was taken to the operating room for emergent bowel resection and a perforation site was identified at the transverse colon. The resected transverse colon serosa was congested and dusky with site of perforation identified (). The colonic mucosa revealed diffusely edematous folds as well as confluent areas of yellowish exudate and multifocal ulcers (). Histologic sections confirmed the presence of transmural necroinflammation and multifocal ulceration (Figures and ). The findings were of a fulminant colitis with multifocal ulceration and perforation. No evidence of metastatic melanoma to the bowel was identified. |
A 24-year-old male reported to the outpatient department with complaint of swelling of the anterior lower jaw region for 9 months. He had a history of traumatic injury and extraction of teeth from the same region around a month before the onset of swelling [].\nThe personal and family history was not relatable to the present condition.\nExtraoral examination revealed a single large asymptomatic firm swelling approximately measuring 4 cm × 4 cm in the mandibular midline. The overlying skin showed scar of the previous trauma. Intraorally, the swelling was soft to firm, nontender and nonfluctuant of approximate size 4 cm × 3 cm, extending from 34 to 43 region with obliteration of labial vestibule [Figure and ]. The mucosal surface was normal in color without signs of any drainage. Anterior mandibular teeth 41, 31, 32 and 33 were missing due to previous trauma while 42 showed grade II mobility.\nOrthopantomogram (OPG) showed well-defined unilocular radiolucency in the mandibular anterior region crossing the midline and root resorption of 42 along with a tooth-like radiopaque mass within the lesion []. Cone-beam computed tomography (CBCT) showed a round unilocular lesion with complete destruction of labial bony plate and irregular resorption front towards lingual side []. Non uniform resorption of bone and a tooth-like calcification was evident in the 3D reconstruction image of CBCT [].\nThe routine blood examinations showed no alterations.\nThe thick yellow fluid discharge at the time of incision biopsy on H&E-stained smear showed population of large oval to round cells with vesicular as well as hyperchromatic nuclei within a background of red blood cells.\nMicroscopically, unencapsulated sheets of proliferating odontogenic epithelial cells were seen with a dual cellular pattern. Few cells were round to ovoid with eosinophilic cytoplasm and hyperchromatic nuclei and the other composed of basaloid cells with pale cytoplasm and large vesicular hyperchromatic nuclei [Figure -]. Areas of calcifications were seen close to few tumor islands and within the ghost cell clusters []. The tumor cells showed extensive nuclear and cellular pleomorphism, cellular atypia and increased mitotic figures (>6/HPF) [Figure -]. Features of ghost cell keratiniation were evident at many focuses as large round pale eosinophilic malignant epithelial cells which lack nuclear features [ and ]. Multinucleated giant cells were evident at places were the ghost cell interacted with overlying connective tissue stroma []. The possibility of any odontogenic cyst, COC, ameloblastomas and calcifying epithelial odontogenic tumor (CEOT) were ruled out narrowing down the differential diagnosis to GCOC and DGCT. The presences of dentinoid in such calcifications were ruled out using Van Gieson's staining []. Subsequent immunohistochemical examination using Ki67 (>60%) [] showed a high malignant potential of tumor while higher p53 expression, [] both favored a malignant ghost cell lesion the GCOC over the benign DGCT. Correlating the clinical, radiological, histopathological and IHC expressions the final diagnosis was GCOC. |
A 57-year-old man with known Marfan syndrome was admitted to the emergency department with sudden onset of right arm and leg weakness and slurred speech 50 min before presentation. His past medical history included a metallic aortic valve replacement 12 years ago, which was infected with MRSA and so this was replaced with tissue valve a month later. Other history included mild congestive heart failure (New York Heart Association class II) and a transient ischemic attack 2 years ago. Echocardiography (ECHO) 1 year prior to this admission had shown an ejection fraction of 41% but stable prosthetic valves. He was fit and independent in activities of daily living. His medications on admission were frusemide, bisoprolol, amlodipine, losartan, spironolactone, and fluoxetine. He was no longer taking aspirin and dipyridamole at the time of admission.\nOn examination, he had a partial left anterior circulation syndrome with right hemiparesis (Medical Research Council grade power of 3/5 in the arm and 4/5 in the leg), a right upper motor neuron facial palsy, expressive dysphasia, and right sided visual neglect. His National Institute of Health Stroke Scale (NIHSS) score was 12. His blood pressure was low at 100/80 mm Hg. Apart from the thoracotomy scar, and the tall features of Marfan syndrome his physical examination was normal. Routine blood tests, electrocardiogram and chest X-ray were normal. A computed tomogram (CT) of the head, performed 2 h after symptom onset, did not show evidence of infarction or haemorrhage (Figure ). Intravenous thrombolysis with alteplase at a dose of 0.9 mg/kg bodyweight was started 2 hours and 30 min after the onset of symptoms. There were no immediate complications, and he remained haemodynamically stable. Twenty hours later he became drowsy. His NIHSS score worsened to 18 with more pronounced right hemiparesis. A repeat CT head scan (Figure ) showed an established left middle cerebral artery territory infarct with haemorrhagic transformation within the infarcted area. There was also swelling of the ipsilateral left cerebral hemisphere with mild midline shift towards right. Over the next few days, the patient gradually improved with continued physiotherapy. At 9 days, he was able to transfer independently from bed to chair. His NIHSS score was 7 with word finding problems, partial right hemianopia, right visual neglect and a mild right hemiparesis. A Repeat CT head scan at 2 weeks showed an established infarct and complete resolution of haemorrhagic transformation (Figure ). At this stage he was commenced on aspirin. He was discharged home after 3 weeks when he was mobilizing independently, though he had mild right upper limb weakness, dysphasia and cognitive impairment. At three months he was able to communicate and physically independent, but he was unable to return to his usual hobbies and work because his memory had deteriorated, he could not coordinate his right hand, and had word finding problems. He underwent further investigations to discover the aetiology of the stroke. Carotid Doppler did not show any evidence of significant carotid stenosis. ECHO cardiography showed a normal aortic root and a normally functioning prosthetic aortic valve though the ejection fraction was low at 41 %. There was no evidence of aortic dissection. |
A 50-year-old Caucasian female, with no previous smoking history or pancreatic cancer family history, presented to our clinic for surgical evaluation of a biopsy-proven PDA noted at the junction of the body and tail of the pancreas. The patient had previously undergone a classic pancreaticoduodenectomy (Whipple) procedure with concurrent hemicolectomy 6 years prior (in 2011) for a T3N0M0 adenocarcinoma of the pancreatic head that had invaded the mesentery of the proximal transverse colon. After the 2011 surgery, pathology revealed a poorly differentiated PDA along with a PanIN grade 2. The final pathology showed negative surgical margins, positive perineural spread, and 0/33 specimen lymph node involvement. From June 2012 to October 2012, the patient underwent and completed adjuvant chemotherapy with gemcitabine, capecitabine, and radiation at an outside hospital. She was carefully followed by her medical oncology team with serial CA 19-9 monitoring and abdominal MRIs on an ongoing basis. Due to her young age, in 2011, she underwent genetic screening and no germline mutations were identified. Since the time of the primary resection, the patient had been high functioning and healthy, with the exception of some problems of early satiety and recurrent cholangitis. These sequelae were attributed to close proximity of the gastrojejunostomy and hepaticojejunostomy, with possible reflux of intestinal contents up the afferent limb, all partially managed by diet changes.\nIn 2017, a biannual screening MRI with intravenous contrast showed a new pancreatic lesion measuring 2.3 × 2.2 cm in the tail of the pancreas (). Esophagogastroduodenoscopy and endoscopic ultrasound-guided biopsy identified it as a poorly differentiated adenocarcinoma. From 2011 to 2017, she had had close followup with serial CA 19-9, and a measurement of this marker after identification of the lesion on MRI showed an elevation, which was confirmed on repeat testing (61 and 55 U/mL; normal <37 U/mL). This was the first instance of two consecutive CA 19-9 measurements outside of the normal range since resection of the primary cancer 6 years prior. When the patient presented to our institution a few months later, the CA 19-9 had returned to normal at 32 U/mL (), and there was a moderate increase in CEA (16.9 ng/mL; normal <3 ng/mL). The patient received a second MRI of the abdomen and pelvis with contrast to identify distant disease, which showed the lesion to be confined to the pancreas. Along with the MRI of the abdomen, a CT of the chest was preformed, which showed no gross metastatic lesions. A completion pancreatectomy was scheduled with revision and lengthening of the jejunal limb proximal to the gastrojejunostomy to resect the tumor and treat her episodes of early satiety and recurrent cholangitis.\nIntraoperatively, the patient's three Whipple anastomoses were noted to be grossly intact and there was no evidence of metastatic disease. First, the stomach was divided approximately two centimeters proximal to the prior gastrojejunostomy. The jejunostomy was closed, and gastrocolic and gastrosplenic ligaments were divided. The splenic artery was ligated, and the splenocolic ligament was divided. The spleen and pancreas were mobilized out of the retroperitoneum. The jejunum was divided between the pancreaticojejunostomy (PJ) and hepaticojejunostomy. The proximal jejunum, prior PJ, remaining pancreas, and spleen were removed. The tumor was noted to be grossly confined to the pancreas. The distal end of the stomach was delivered through the mesocolon and a retrocolic gastrojejunostomy was undertaken 60 cm downstream from the hepaticojejunostomy. The anatomy before and after this operation is shown in .\nThe patient tolerated the procedure well and had an uncomplicated hospital course. The patient was closely followed postoperatively and did well. She completed two cycles of adjuvant chemotherapy with gemcitabine and capecitabine from January 2018 to April 2018, but did have some neutropenia at the end of her adjuvant therapy requiring pegfilgrastim. Her intermittent fevers, early satiety, and abdominal pain resolved after the surgery. She became an obligate insulin-dependent diabetic after the completion pancreatectomy procedure and now requires exogenous pancreatic enzymes to support her nutrient absorption.\nThe specimen was found to be consistent with a poorly differentiated invasive adenocarcinoma. Resection margins were negative, and 2 of 17 lymph nodes were positive for metastatic cancer. We sent representative slides of the patient's 2017 tumor and 2011 tumor to Perthera (McLean, VA) for next-generation sequencing (NGS) and histological analysis, which tested for mutations in a total of 315 genes and stained for various predictive biomarkers (). Both lesions showed the same mutations in KRAS (G12R), CDKN2A (splice site 151-1 G to A), and TP53 (Y220C). In addition, her 2011 tumor had a mutation in ACVR1B (S4) that was not present in the 2017 tumor, and the 2017 tumor had amplifications of MYC and mutant KRAS that were not present in the 2011 tumor (). Upon histological analysis, staining for MLH1, MSH2, MSH6, PMS2, pAKT, and HER2 was similar in both samples, but in the 2017 tumor, there was 60% increased staining for RRM1 and 20% increased staining for ERCC1, which changed the classification from low to high staining for ERCC1. |
A 21-year-old female patient (162 cm, 48.4 kg) visited the emergency room with abdominal pain after eating a heavy meal five hours previously. She had been treated at a local psychiatric clinic for anorexia nervosa and bulimia nervosa, and she had a relatively low body mass index of 18.44 kg/m2.\nWhile visiting the emergency room, her abdomen looked markedly distended. She suffered from nausea and retching but was unable to vomit. Her vital signs upon arrival at the emergency room were as follows: Blood pressure 128/83 mmHg, pulse 78 beats/min, respiratory rate 18 breaths/min, and a body temperature of 36.8℃. Chest AP and simple abdomen flat upright view revealed that the stomach was full of food material with air-fluid level. Computed tomography showed a massive gastric overdistension with pneumoperitoneum (). A Foley catheter was inserted into the bladder cavity, and the amount of urine there was nearly zero (). Levin tube insertion revealed that the material in her stomach was incompletely digested and that drainage was not effective. The initial complete blood cell count was within normal ranges, including a hemoglobin level of 12 g/dl. Blood chemistry had no unusual characteristics except doubly elevated levels of amylase and lipase.\nWhile going through the various examinations, the emergency specialist noticed that her both lower extremities were turn to be cyanosis. The pulsation of both lower extremities could not be checked. Furthermore, her heart rate was changed to a tachycardic rate of 135-143 beats/min two hours after it was first checked. Therefore, the surgeon decided to perform an emergency exploratory laparotomy in the operating room.\nAs the patient entered the operating room, she displayed a slightly drowsy mental disorder with fast and shallow respiration. Her approximate breathing rate was more than 30 breaths/min.\nWe applied invasive arterial pressure monitoring with a 20 G angiocatheter on her right radial artery and inserted a 7 French central line catheter on her left internal jugular vein subsequently, prior to induction. Just as before the induction, her blood pressure dropped to 80/48 mmHg, her heart rate was 110 beats/min, and her mental status turned to a stupor. We tried to oxygenate the patient with a mask fitting and insufflations of 100% oxygen for several minutes. Anesthetic induction was conducted with midazolam 3 mg and then rocuronium 50 mg IV bolus, and 1 min after endotracheal intubation was performed without prior positive pressure ventilation with the facial mask. Just after intubation, the patient's vital signs were tolerable, with a blood pressure of 91/42 mmHg and a heart rate of 116 beats/min. For anesthetic maintenance, sevoflurane inhalation with remifentanil continuous infusion was started. A few minutes after induction, the first arterial blood sample for blood gas analysis was drawn, and the results indicated both mixed metabolic acidosis and respiratory alkalosis (). Hyperkalemia was also detected at 7.4 mmol/L, so the anesthesiologist ordered that a nurse get calcium, sodium bicarbonate, and insulin. At that time, the anesthesiologists noticed that the electrocardiogram readings seemed abnormal. Meanwhile, just after the abdominal cavity was opened and the gastric contents were sucked out through a small gastric incision, her blood pressure abruptly dropped to 36/22 mmHg (), and the electrocardiogram showed bizarre and wide QRS. Immediate treatment was conducted, which included the rapid infusion of 800 ml of crystalloid and 400 ml of colloid fluid, the start of a continuous infusion of norepinephrine at 0.3 µg/kg/min, the bolus administration of 300 mg of calcium, and 20 mEq of sodium bicarbonate. However, her tremendously decreased blood pressure could not be restored and her cardiac rhythm became asystolic. It only took 12 minutes from the initiation of the exploratory laparotomy to asystole. We started cardiac compressions immediately, and during the cardiac compression for resuscitation, five 1 mg doses of epinephrine bolus injection were given at a rate of one every few minutes. A bolus of 20 IU of vasopressin was also injected after the administration of the fourth dose of epinephrine. Insulin 10 IU was also administered, and both 300 mg of calcium and 300 mEq of sodium bicarbonate were injected approximately every 5 min, and so a total of 1,200 mg of calcium chloride and 1,200 mEq of sodium bicarbonate were administered to correct the hyperkalemia. Even though the procedure made for less than 50 ml of blood loss, four units of packed RBC were transfused for the decreasing tendency of hemoglobin down to 4.9 g/dl. Additional fluids as well as furosemide 40 mg IV bolus were given to correct the anuria. Consequently, as much as a total of 1,200 ml of crystalloid and 600 ml of colloid, which were loaded with 960 ml packed RBC were given during the cardiac resuscitation. The results of the arterial blood gas analysis throughout the anesthesia and resuscitation are presented in , and the change of potassium is presented in . All efforts for resuscitation including cardiac compressions were maintained for 29 minutes. Despite these exertion, the asystole state persisted, her blood pressure was not checked and no cardiac rhythm was restored, so the surgeon in charge pronounced her dead. The operating note illustrated that not only the stomach but also the small bowel and colon showed color change, suggesting necrosis. There was no description of the other solid abdominal organs. |
A 32-year-old man was admitted to our hospital with appetite loss. He had a history of traumatic transverse cervical spinal cord injury at the C5 level due to suicide attempt at the age of 18. As a result of cervical spinal cord injury, he was paralyzed in the lower body. Contrast-enhanced computed tomography (CT) revealed a late-onset traumatic diaphragmatic hernia with strangulated ileum (Fig. ). The small intestine, transverse colon, and omentum were displaced into the left thoracic cavity, and some portions of these organs showed a decrease in blood flow. Left lung collapse and a compressed right lung with mediastinal shift were evident. The patient underwent emergency surgery. After replacing the incarcerated organs to their original positions, scattered areas of necrosis were identified in the small intestine, transverse colon, and omentum (Fig. ). By using interrupted sutures with non-absorbable 1–0 monofilament, the diaphragmatic orifice was closed. Wedge resection with primary closure was performed for the colonic necrosis in two places. Partial resection, 45 cm long, with end-to-end anastomosis was performed for the small intestine. The necrotic omentum was removed (Fig. ). In addition, a gastrostomy tube was placed since delayed initiation of oral intake was expected. The patient developed severe septic shock postoperatively. Treatment-resistant critical hypotension with non-compensatory tachycardia developed, likely due to parasympathetic nervous system damage related to the cervical spinal cord injury. On postoperative days (POD) 3 and 6, cardiac arrest occurred. Fortunately, he was rescued by cardiopulmonary resuscitation with administration of large doses of vasopressin and catecholamine. However, peripheral vasoconstriction, increased intra-abdominal pressure, and ischemia of the gastrointestinal tract developed, which resulted in colonic anastomotic leakage with diffuse peritonitis, abdominal wound dehiscence, and collapse of gastrostomy on POD 6 (Fig. ). The patient was unable to undergo surgical repair because of his poor general condition with continuing severe septic and neurogenic shock. Therefore, he underwent AVS through the open abdominal wound and it was the first procedure at the intensive care unit. The procedure of AVS was as follows: 1. the open wound and peritoneal cavity were rinsed with normal saline and necrotic and/or contaminated tissues were debrided (Fig. ); 2. wound dressing materials (DUOACTIVE® ConvaTec, New Jersey, USA) for protecting healthy skin around the open wound were patched along the abdominal wound in piecemeal fashion so as to adjust dressing materials to the complicated shape of the wound (Fig. ); 3. two drainage tubes with multiple side holes, up to 30 cm from the tip, were placed in the abdominal cavity through the open abdomen and the enteric contents were suctioned through the drainage tubes using a Continuous Suction Unit MERA Sacuum (Senko Medical Instrument Manufacturing CO, Tokyo, Japan) set to 50–75 mmHg continuous negative pressure; and 4) the entire wound was filled with saline-moistened gauzes and covered with polyurethane drape (Fig. ). The colonic anastomotic leakage showed gradual healing over the course of 2 months, followed by contraction and closure of wound dehiscence (Fig. ). Because the gastric fistula remained, a gastrostomy balloon catheter was placed through the gastric fistula. The patient resumed oral intake on POD 112 and left the hospital on POD 190 with the gastrostomy balloon catheter and without incisional hernia. |
A 22-year-old Caucasian man with a medical history of epilepsy, nephrolithiasis, and nutcracker syndrome presented with abdominal and perineal pain. The patient’s family history was negative for nutcracker syndrome and pelvic congestion syndrome. The pain was ongoing for approximately 3 years before the initial consultation, and a left renal vein transposition was undertaken 1 year before initial consultation, without any improvement in his symptoms (Fig. ). The pain was located in the left flank as well as variably in the left lower quadrant and left lower back. The pain consistently radiated to the groin and perineum and variably radiated to the left posterior thigh. The patient described the pain as sharp, shooting, and piercing. His pain was constant, with a baseline intensity of 6/10 on the visual analogue scale. His pain improved with medications, including gabapentin and hydrocodone-acetaminophen, restricting movement, and lying flat; it worsened with movement and sitting upright or standing. Associated signs included a varicocele, which had been repaired 6 years before initial consultation, and associated symptoms included infrequent nausea and vomiting. The patient’s vital signs were within normal limits. His physical examination revealed tenderness to palpation over the left flank without rebound tenderness or guarding. His laboratory test values were normal, except for microscopic hematuria on urinalysis. Due to continued flank pain at 6 months postoperatively, a repeat renal angiogram/venogram was obtained, which showed that although the left renal vein was successfully transposed, the left renal vein remained compressed with continued mild reflux into the left adrenal and left lumbar veins. Because our patient’s pain variably radiated into his posterior thigh, MRI of the lumbar spine was conducted, which showed mild bilateral L4 and L5 foraminal stenosis and mild disc bulge with mild mass effect on the left L5 nerve root. Even so, the results of straight leg raise and reverse straight leg raise tests were negative. In addition, the results of electromyography of the sciatic nerve and its branches were normal.\nThe patient underwent a diagnostic celiac plexus block with 70% improvement in his symptoms for 6 days, indicating a high likelihood of visceral origin of his pain. A celiac plexus neurolytic block was subsequently performed but relieved only 60% of his pain for 2 months. He was administered an injection of 5 ml of 0.25% bupivacaine followed by 15 ml of 98% dehydrated alcohol using the central technique. Although his flank pain was almost entirely relieved, his perineal and testicular pain, which was far more bothersome, persisted. As such, a ganglion impar block was undertaken, which significantly improved his pain (80% for 3 months). The block included 15 ml of 0.25% bupivacaine with 15 mg of dexamethasone. As a result, the patient underwent serial ganglion impar blocks over the course of 5 years, which significantly improved his symptoms (70–100%, lasting for 3–4 months). Some residual mild left flank pain persisted at times. Ganglion impar neurolysis was proposed; however, the patient declined for two reasons. Primarily, he was fearful of the possibility of perineal and testicular dysesthesias and hyperesthesias. Secondarily, because pain relief from neurolysis generally lasts no more than 6 months, he did not think the benefits outweighed the risks, considering that he was already benefiting from long-lasting pain relief from his current intervention. Although ganglion impar blocks were relieving his symptoms well, the patient wanted to explore more permanent options after approximately 2 years of consultation. For this reason, an intrathecal morphine trial was undertaken, but he was dissatisfied because of excessive pruritus and constipation and only a 60% reduction in pain. Later, a dorsal root ganglion trial was proposed; however, the patient had hesitation concerning permanent implantation of a device at that time. No adverse or unanticipated events have occurred as a result of his ganglion impar blocks. |
A 69-year-old female was referred to our clinic for an incidental finding of a large Morgagni hernia found on a recent CT chest scan for lung cancer screening. Patient reported occasional shortness of breath after prolonged ambulation but denied chest pain. She did have remote history of acid reflux symptoms but nothing recently. She denied issues with prematurity or issues with development as an infant, chest trauma, or MVA history. She did complain of occasional right shoulder pain but attributed this to arthritis. Denied history of heart attack, stroke, DVT, or PE. She had a 30-pack-year smoking history but quit a year prior. She was up-to-date on her colonoscopy, current within the past year. She denied hematochezia and melena, bowel habit changes or major body weight changes as well as any current abdominal pain. On examination her vitals were within normal parameters. Heart and lungs were unremarkable. Abdominal examination was soft with normal bowel sounds and nontender. Remainder of examination was unremarkable. Laboratory values included a normal CBC and BMP. A CT chest scan had demonstrated a large retroxyphoid hernia of Morgagni involving several loops of small bowel and transverse colon located in the right inferior hemithorax (Figs and ). No evidence of acute incarceration or strangulation were noted. A detailed discussion was undertaken with the patient regarding her hernia and she was consented for a laparoscopic repair with mesh.\nPatient underwent a laparoscopic approach in lithotomy positioning with the primary surgeon working between the legs. Three working ports were used, a 12 mm port at the umbilicus and two 5 mm ports; one in the LUQ and one in the RUQ. Upon initial laparoscopy multiple loops of small bowel were progressively reduced out of the hernia sac which also included the ascending colon and part of the transverse colon (Figs and ). All the small bowel and the colon appeared viable. The redundant parietal peritoneal hernia sac was excised out of the right inferior hemithorax utilizing a LigaSure (Covidien) (Fig. ). The falciform ligament was also taken down all the way to the diaphragm. The defect in the diaphragm measured to be approximately 9 cm by 4 cm. A section of Pariatex composite mesh was then trimmed to 2 cm in width by 9 cm in length. Three stay sutures of 0 Ethibond were placed laterally and in the middle of the mesh. This was placed into the peritoneal cavity after soaking it in vancomycin with local anesthetic. The sutures were then percutaneously brought through the diaphragm edge that was unattached to the anterior abdominal wall and then subsequently through the anterior abdominal wall. These were then tied thereby re-approximating the unattached edge of the diaphragm to the anterior abdominal wall near the xiphoid (Fig. ). Additional 0 Ethibond sutures were placed in between these initial ones percutaneously with a suture passer.\nAdditionally, another Pariatex composite mesh was then trimmed to 12 cm in width by 9 cm, soaked in vancomycin with local anesthetic and then placed into the abdominal cavity. It was positioned over the area of the repair and fixed into place with absorbable tacks around its caudad edge and centrally. Along the cephalad edge it was fixed with a running V-lock absorbable suture to the diaphragm. Fibrin glue was placed along this same edge (Fig. ). The ports were removed and incisions were closed.\nPatient’s postoperative course progressed well. She was monitored overnight and discharged the following day. She was seen for follow-up in 2 weeks out of surgery and did quite well. She was tolerating a regular diet and having bowel movements. A month after surgery another CT scan was obtained which demonstrated a postoperative seroma in the right inferior hemithorax (Fig. ). Currently, the patient is to be seen in a 6-month follow-up to have another CT scan at that time. |
A 63-year-old North American Caucasian man was admitted to our hospital after falling down four steps and sustaining head trauma. At presentation, he stated he had a headache, but denied visual changes, numbness or weakness. He was neurologically intact; notably, pronator drift was absent. His medical history was significant for NPH for which a programmable Medtronic Strata® ventriculoperitoneal (VP) shunt was placed three years prior to this event.\nA computed tomography (CT) examination showed the ventricular catheter and an acute right posterior convexity subdural hematoma (Figure ). The SDH overlying the right convexity measured 3cm in the greatest transverse diameter, causing mass effect on the ipsilateral brain parenchyma and posterior horn of the lateral ventricle.\nOur patient was admitted to the intensive care unit for close neurological monitoring. Our patient was neurologically intact, and, thus, conservative management was favored over operative treatment. However, given the propensity for expansion of acute subdural hematomas in the presence of low-pressure ventriculoperitoneal shunts and given the moderate size of our patient’s subdural hematoma, simple observation was thought to be high risk. In addition, our patient reported dramatic improvement of his NPH symptoms after shunting, and, thus, simple shunt closure via ligature was not optimal. The authors chose to treat our patient’s acute subdural hematoma by utilizing a technique that has been used in the treatment of subdural hygromas and chronic subdural hematomas. This technique, wherein the programmable valve setting is changed to reduce CSF drainage, allowed conservative management and observation of our patient without operative intervention.\nThe programmable valve was adjusted transcutaneously from 1.0 to the maximum setting of 2.5, thereby reducing CSF drainage. A repeat head CT obtained the following day revealed no significant change in the size of the subdural hematoma. He remained clinically unchanged and neurologically intact. He was then discharged from the hospital with a plan for close follow-up. Six days later, our patient was admitted with worsening symptoms of NPH including gait ataxia and urinary incontinence. CT examination showed complete resolution of the acute SDH and dilated ventricles consistent with our patient’s known history of NPH (Figure ). The valve setting was reduced from 2.5 to 0.5 in order to promote greater CSF drainage. This alleviated the NPH symptoms. Our patient remains well one year after sustaining the traumatic subdural hematoma (Figure ). |
A 48-year-old woman was referred to our hospital because of upper abdominal pain. Her medical and family histories were unremarkable, and her abdomen was soft, with no palpable mass. Laboratory investigation revealed slight leukocytosis (white blood cell count 10 280/μl), but other data, including the serum concentrations of tumor markers, immunoglobulin (Ig) G and IgG4, and pancreatic hormones (gastrin, insulin, glucagon, somatostatin, and pancreatic peptide) were all within their normal ranges.\nAbdominal-enhanced computed tomography (CT) revealed a low-density pancreatic body tumor of 25 mm in diameter, which was close to the gastroduodenal artery (GDA), and was accompanied by atrophy of the distal pancreatic parenchyma and dilation of the upstream MPD (). Moreover, CT imaging of the portal phase revealed a filling defect within the splenic vein where it was in contact with the tumor (). On magnetic resonance imaging, the tumor appeared as an area of signal hyperintensity on T2-weighted and diffusion-weighted images (). On positron emission tomography-CT scan, the tumor exhibited greater uptake of 18F-fluorodeoxyglucose (). Although a definitive preoperative diagnosis could not be made, we suspected pancreatic ductal adenocarcinoma, and distal pancreatectomy with lymph node dissection was scheduled, without neoadjuvant chemotherapy, after obtaining consent from the patient. The pancreas was divided along the left edge of the GDA after mobilization of the artery; however, pathological examination of a frozen section of the pancreas stump was positive for PNET, and therefore a total pancreatectomy was performed (). The duration of surgery was 610 min, the intraoperative blood loss was 195 mL, and blood transfusion was not performed.\nThe gross appearance of the resected specimen was of a white, solid, nodular tumor measuring 25 × 15 mm, which was located in the pancreatic body, with spread into the MPD and protrusion into the splenic vein (). Microscopically, the tumor largely comprised fibrovascular stroma, with small nests and cords of uniform cells arranged in a rosette-like pattern (). Immunohistochemistry showed that the Ki-67 labeling index was 5%, and there was positive staining for chromogranin A and synaptophysin. Therefore, a diagnosis of non-functioning PNET G2 was made and one of the 32 lymph nodes examined was tumor positive. The patient was discharged 30 days after surgery, having experienced no complications, and no tumor recurrence occurred in the 13 months following, in the absence of adjuvant therapy. |
A 25-year-old right-handed man presented to the emergency department with a headache, right-sided weakness, confusion, and aphasia which began the previous night (day 0). On day 0, the patient awoke late in the afternoon with a headache and some confusion according to his family. The patient then went back to sleep until the following morning. Upon awakening, the patient exhibited right-sided weakness and difficulty speaking in addition to the headache and confusion. His mother also reported the patient was nauseous and had episodes of vomiting throughout the night. The patient had a questionable history of migraine with prolonged aura. Prior to the onset of the headache, the patient was in his usual state of health. Unique to this experience, which prompted his parents to bring him to the emergency room, was the persistence of headaches, hemiplegia, and difficulty speaking. A level II stroke alert prompted a CT of his head and Computed Tomography Angiography (CTA) of his head and neck; both were negative for any signs of a stroke, stenosis, or occlusion. The consulting neurologist diagnosed the patient with atypical migraine since the symptoms were similar to those previously experienced by the patient in prior attacks. The patient was instructed to return for possible MRI of the brain if symptoms persisted and was referred to an outpatient neurology visit for follow-up. The patient was discharged with a diagnosis of atypical migraine and with instructions for follow-up or to return to the emergency department if symptoms worsened. The patient returned the following day (day 2) due to the persistence of symptoms. Thus, the patient was admitted for observation with right-sided weakness, aphasia, and fever. At 5:00 PM on day two, the patient recorded a temperature of 100.9℉. During the next several days, the patient’s temperature ranged from 98.3℉ − 102.6℉. The patient was febrile to a temperature max of 102.6℉ recorded on day 4 at 4:00 AM. The patient’s fever subsequently broke by at 6:00 AM of day 4. Of interest, the patient reported two female cousins with similar symptoms.\nThe neurological findings and fever prompted empiric treatment for meningitis. A lumbar puncture (LP) was ordered to assess for infectious etiologies. The patient was unable to be interviewed due to aphasia. History obtained from the patient’s parents revealed the patient had been working outside at a car shop and taking several stimulant weight loss supplements in the days immediately prior to admission. The clinical exam showed an alert, dysarthric patient with global aphasia and weakness in the right upper and lower extremities. The first MRI was obtained approximately two days after symptom onset. T2-fluid-attenuated inversion recovery (FLAIR) showed no signs of acute intracranial abnormality but did show scattered hyperintensities in the white matter of frontal lobes bilaterally (Fig. ).\nOn the day following his admission (day 3), the results of the patient’s LP showed clear, colorless fluid which was negative for xanthochromia. Glucose and protein were within normal limits while white blood cells and red blood cells showed 2 cells/mm3 and 1 cells/mm3 respectively. Polymerase Chain Reaction (PCR) was negative for enterovirus, herpes-simplex virus (HSV) 1 and 2, and cryptococcal antigens. The culture of the cerebrospinal fluid (CSF) was ultimately negative for bacterial species, but his fever persisted. He underwent seizure-like activity which prompted treatment with 1 mg Intravenous (IV) Lorazepam and subsequent resolution of the episode. A loading dose of 1000 mg of IV Levetiracetam followed by 500 mg twice a day as well as further workup was ordered. Another MRI was obtained the day following his seizure-like activity (day 4), which showed diffuse diffusion-weighted imaging (DWI) and T2/FLAIR hyperintensities throughout the left cerebral hemisphere cortices. Electroencephalogram (EEG) was ordered and returned abnormal results indicative of global central nervous system (CNS) dysfunction and focal dysfunction of the left hemisphere. Encephalitis was suspected, but the exact etiology was still unknown.\nEmpiric treatment for bacterial meningitis was discontinued on day 5 following negative LP and advice from infectious disease consultations. With a tick-borne molecular panel still pending, empiric treatment with doxycycline was advised. HIV, fungal, bacterial, and viral agents all returned negative. On day 7, the patient experiences a generalized tonic-clonic (GTC) seizure which resulted in the decision to start long-term EEG and repeat MRI. The repeat MRI showed similar findings to the previous MRI - T2/FLAIR hyperintensity of the left cerebral cortex without enhancement and associated sulcal effacement. At this time, the differential was still broad including encephalitis of viral, neoplastic, autoimmune, or idiopathic origins. A steroid regimen was employed.\nFigure highlights the MRI findings throughout the course of the patient’s hospital stay as well as the resolution and follow-up visits. Day 11 was notable for more prominent hypointensity on Apparent Diffusion Coefficient (ADC) in the left cerebral hemisphere cortices and temporal lobe. MRI on day 15 revealed even more prominent hypointensity on ADC. Continuous EEG recording was started on day 12 and continued for 9 days. The findings from EEG were largely non-specific but included left-hemispheric slowing with mild diffuse slowing. By the end of the EEG recordings, the left hemispheric attenuation had largely resolved.\nTo rule out germ cell tumor, CT of the chest, abdomen and pelvis was obtained on day 16 and showed no evidence of neoplasm. Despite the lack of success identifying a causal agent, the patient showed signs of clinical improvement with the persistence of the aphasia. Due to the negative LPs, the negative pan CT, and non-specific EEG findings, the differential now included limbic encephalitis, autoimmune, prion disease, Transient Headache and Neurologic Deficits With Cerebrospinal Fluid Lymphocytosis (HaNDL syndrome) and last, but not least, the Rasmussen syndrome. LP, anti-Hu/Ma, anti-Ta, anti-NR1, anti-AQP4, anti-CASPR2, anti-LGI1, anti-Yo, oligoclonal bands, ANA, anti-double stranded DNA, complement C3 and C4, SSA-SSB antibody, RNP-SM antibody, encephalitis antibody evaluation (ANNA-1/anti-Hu, ANNA-2/anti-Ri, ANNA-3, PCA-1/anti-Yo, PCA-2, PCA-TR/anti-Tr, AGNA/anti-SOX1, Amphiphysin Ab, anti-CRMP5/anti-CV2, anti-GAD65, anti-MA2/Ta anti-myelin, AQP4/Ab/NMO-IgG), an epilepsy autoimmune evaluation (AChR Ganglionic Neuronal Ab, AMPA-R Ab, Amphiphysin Ab, AGNA-1, ANNA-1/anti-Hu, ANNA-2/anti-Ri, ANNA-3, CASPR2-IgG, CRMP-5-IgG, DPPX Ab, GABA-B-R Ab, anti-GAD65, anti-GFAP, LGI1-IgG CBA, mGluR1 Ab, NMDA-R Ab, N-Type Calcium Channel Ab, P/Q-Type Calcium Channel Ab, PCA-2, PCA-Tr), a neurology antibody order (ANNA-1/anti-Hu, ANNA-2/anti-Ri, PCA-1/anti-Yo, PCA-TR/anti-Tr, AGNA/anti-SOX1, anti-Ma2/Ta, anti-Zic4, anti-CRMP5/anti-CV2, amphiphysin Ab, anti-GAD65) and protein 14-3-3 were ordered as part of the follow-up. The decision was made to start the patient on a course of IVIG while additional investigations were still pending. The patient showed improvement after starting the IVIG and was discharged on multiple medications including lamotrigine, levetiracetam, magnesium chloride, riboflavin, valproate sodium and instructions with close follow-ups. At the outpatient follow-up with neurology, the patient showed significant improvements in language, showed no stroke-like symptoms, and denied any seizure-like activity. The rest of the laboratory investigations were unremarkable except genetic testing. The analysis of four genes (ATP1A2, CACNA1A, SCN1A, PRRT2), was positive for a heterozygous, AD mutation in ATP1A2 (c.2936 C > T p. P979L). The patient had a recurrence of seizure which resulted in another short hospital stay. |
A 48-year-old Caucasian female was found unresponsive at the scene of a house fire with burns to the face [<1% total body surface area (BSA)] and inhalation injury. She was intubated via endotracheal tube at an outside facility and subsequently transferred to our burn center for definitive care. The initial bronchoscopy showed diffuse carbonaceous sputum with pale friable mucosa extending throughout the airways distally. The patient was intubated for airway protection, which required adequate sedation and neuromuscular blockade. Due to the inability of the patient to provide a detailed medical history, the patient’s sister and psychiatrist supplied the relevant information, which included a diagnosis of severe depression with psychotic features. On admission, the patient’s list of home medications included paliperidone palmitate 234 mg once monthly as an intramuscular injection, paliperidone 6 mg by mouth daily, citalopram 40 mg by mouth daily, trazodone 150 mg by mouth at bedtime, risperidone 2 mg by mouth twice daily, and clonazepam 1 mg by mouth twice daily. The patient had no known drug allergies or other relevant medical history. She was a chronic smoker and substance abuser, but on admission the urine drug screen was negative.\nUpon admission to the burn ICU, the patient had a mild leukocytosis (white blood cell count 15.8 k/mm3) but was afebrile, normotensive, and demonstrated adequate urine output (>0.5 mL/kg/hr). She had evidence of acute respiratory distress syndrome (ARDS), with a ratio of partial pressure arterial oxygen and fraction of inspired oxygen of less than 200, and was started on pressure control ventilation. Due to the severity of the inhalation injury and the need for prolonged mechanical ventilation with aggressive pulmonary hygiene, a tracheostomy was performed on the second hospital day (HD). Post-operatively, the patient became febrile with an associated tachycardia. By HD 5, the patient expressed thick yellow secretions during suctioning and on physical examination diffuse rhonchi were appreciated, worse in the right lung than the left, corresponding with a worsening right-sided, patchy infiltrate seen on chest X-ray. A culture from the broncheo-alvelolar lavage (BAL) on HD 6 revealed gram-positive cocci in clusters (Table ). Blood cultures obtained on HD 4 also grew Gram-positive cocci in clusters, and the patient was started on empiric vancomycin and cefepime on HD 6 for presumed hospital-acquired pneumonia with associated bacteremia. However, despite an aggressive vancomycin dosing schedule [1750 mg intravenous (IV) every 6 h] and adequate, supratherapeutic, vancomycin trough concentrations of 26 mg/L, the patient’s clinical status did not improve. She continued to be febrile and tachycardic, requiring increased ventilatory support and oxygenation requirements. Repeat urine and blood cultures obtained on HD 5 were negative for the Gram-positive pathogen, which was at this time identified as MRSA. The repeat bronchoscopy on HD 10 demonstrated mild improvement in secretions, but the Gram-stain from the BAL subsequently yielded growth in culture of >110,000 colony forming units/mL gram-positive cocci in clusters, later identified as MRSA with a vancomycin MIC of 2 mg/L by automated susceptibility testing. Due to the patient’s deteriorating clinical status on HD 10 while on high-dose vancomycin therapy, the decision was made to modify the antibiotic regimen. At the Detroit Medical Center, in accordance with suggested treatment pathways, MRSA bacteremia and/or pneumonia and a vancomycin MIC of ≥2 mg/L constitute a reason to change therapy to a suitable alternative. However, with consideration of the patient’s medication history, and current medication regimen of antipsychotics and a selective serotonin reuptake inhibitor (SSRI), linezolid was not employed due to the possibility of drug–drug interactions with the psychiatric medications. After consultation with the infectious diseases (ID) pharmacist and the ID medical consult service, a decision was made to initiate ceftaroline fosamil (Teflaro) for the treatment of this patient’s MRSA pneumonia.\nCeftaroline fosamil (Teflaro) is a new advanced generation cephalosporin approved by the FDA for use in acute bacterial skin and skin structure infection (ABSSSI) including MRSA and CABP caused by susceptible bacteria. Ceftaroline binds to the penicillin-binding proteins including PBP 2a associated with methicillin resistance in S. aureus and prevents the cross-linkage of peptidoglycan in bacterial cell walls [–]. Ceftaroline fosamil, a prodrug, is converted to the active form of ceftaroline via serum phosphatases and undergoes minimal cytochrome P450 oxidation. Approximately 88% of a dose of ceftaroline is excreted renally, with only 6% of the drug recovered in the feces []. The prescribing information indicates the approved dose of ceftaroline fosamil administered intravenously is 600 mg every 12 h for ABSSSI and CABP []. However, due to the increased volume of distribution (V\nd), renal clearance, and urine output in burn injury patients, the patient’s regimen was empirically modified to 600 mg IV every 8 h, and administered as a 2-h infusion to ensure that adequate drug concentrations be maintained throughout the dosing interval. The clinical MRSA isolate tested had a ceftaroline MIC of 0.5 mg/L by Etest® (bioMérieux SA). This antimicrobial regimen was continued for a total of 14 days. The patient became afebrile 48 h after initiation of ceftaroline and remained afebrile for the length of the hospital stay. Rapid clinical improvement was seen after initiation of ceftaroline; the patient was subsequently weaned from the ventilator on HD 22 and decannulated 2 days later. However, because of her psychiatric illness and auditory/visual hallucinations, hospital discharge was delayed to allow for psychiatric placement.\nDue to the relative lack of clinical data supporting the use of ceftaroline in burn patients, serum concentrations of ceftaroline were obtained for PK characterization and potential dosage adjustment. Serum drug levels were obtained on day five of ceftaroline therapy. Concentrations were obtained 30 min post-infusion, 2 h later, and a final concentration drawn 30 min prior to the next dose (Table ).\nThe ceftaroline bioassay was performed according to previously published methods []. Briefly, quarter-inch disks were placed on agar plates (antibiotic medium number 11) pre-swabbed with Bacillus subtilis ATCC 6633. The disks were spotted with 10 μL of ceftaroline standards (2.5, 10, and 40 mg/L) or plasma samples. Each standard and sample was tested in duplicate. Plates were incubated for 18–24 h at 37 °C, after which the zone sizes were measured using a protocol reader (Protocol; Microbiology International, Frederick, MD, USA). Ceftaroline half-life, C\nmax (peak), and C\nmin (trough) mg/L were determined from concentration-versus-time plots assuming a one-compartment model (Table ). Patient-specific concentrations and time above MIC (T > MIC) were calculated utilizing first-order elimination concepts (Table ). The elimination half-life, peak and trough concentrations, and area under the curve (AUC) were calculated using PK Analyst Software (version 1.10; MicroMath Scientific Software, Salt Lake City, UT, USA). |
A 4-year-old boy on PD due to end-stage kidney disease resulting from bilateral hypoplastic kidneys, was admitted to our hospital with the complaints of fever, abdominal pain, and cloudy peritoneal effluent on PD, 1 day after biting and accidentally opening the PD catheter while in the bath. He was planned to receive living related KT from his father.\nHis vital sign was the following: temperature of 37.0 °C, blood pressure of 96/48 mmHg, pulse of 144 beats per minute. On physical examination, he appeared fine and his extremities were not cold. There was no abdominal tenderness or other signs of peritoneal irritation. The catheter exit site was clear. Laboratory examination at admission showed the following: white blood cell count, 8180/μL; C-reactive protein, 3.17 mg/dL; and dialysate cell count, 775 /μL with 74% polymorphonuclear leukocytes. Although the peritoneal fluid culture was negative, he was clinically diagnosed with bacterial peritonitis and started on intraperitoneal vancomycin and ceftazidime. His condition improved promptly after admission, and his white blood cell count in the PD fluid returned to normal on day 7 after hospitalization. He was discharged after completing the 2-week antibiotic therapy.\nHe was readmitted due to fever 37 days after the discharge. His vital signs were as follows: temperature, 38.3 °C; blood pressure, 102/56 mmHg; and pulse, 112 beats/min. He had no abdominal pain, and the catheter exit site was clear. Abdominal computed tomography showed no evidence of encapsulating peritoneal sclerosis or abscess. However, due to a dialysate cell count of 1160/μL accompanied with persistent fever, the patient was started on intraperitoneal vancomycin and ceftazidime on day 5 after readmission, with consideration of relapse of bacterial peritonitis.\nAlthough the bacterial and viral cultures of peritoneal fluid and blood culture were negative, the mycobacterial culture of peritoneal fluid became positive on day 9 after readmission. Interferon gamma release assay for tuberculosis and tuberculin test were negative, and atypical mycobacteria were suspected; therefore, the antibiotics were changed to clarithromycin, ciprofloxacin, and meropenem, considering rapid growing mycobacteria. The PD catheter was removed on day 19 after readmission, and hemodialysis was initiated using a cuffed 18-gauge catheter inserted in the internal jugular vein. Finally, M avium was identified on day 30, and we diagnosed him with MAC peritonitis that occurred on PD. The antibiotic therapy was changed to clarithromycin (5 mg/kg/d), ethambutol (20 mg/kg alternate-day treatment), and rifampicin (10 mg/kg/d) (Fig. ). The patient's fever gradually improved, finally resolving in 1 month.\nThe 1-year anti-mycobacterial therapy was completed without any adverse events. We decided the plan of >6-month observation period after the treatment completion to confirm no recurrence of MAC peritonitis before proceeding to KT. He eventually received ABO-compatible living-donor KT from his father 8 months after the completion of anti-mycobacterial therapy. His post-transplant immunosuppressive regimen comprised methylprednisolone (mPSL), tacrolimus (Tac), and mycophenolate mofetil (MMF) (Fig. ). His renal function was stable with a creatinine-estimated glomerular rate of 56.0 mL/min/m2 and no recurrence of MAC peritonitis at last follow-up 2 years after the KT. |
A previously healthy 10-year-old girl presented with a large swelling arising from the right little finger of 6 months duration. The swelling was initially small and painless to start with and was associated with fever and chills. She was seen at a local hospital, where a radiograph was taken which revealed a cystic and lytic lesion of the little finger proximal phalanx []. It was diagnosed as osteomyelitis, and she was treated with analgesics and antibiotics. As the swelling and pain progressed, she was referred to this institute. The swelling was 6 × 6 cm, tender, pulsatile and firm in consistency. The fresh radiograph showed a large soft tissue mass with some calcification within and complete destruction of the phalanx [Figure and ]. Magnetic resonance imaging (MRI) scan of the hand revealed an ill defined expansible lesion in the proximal phalanx with marrow signal changes that were T1 hypointense and T2/Short tau inversion recovery hyperintense with juxtacortical soft tissue mass []. Fine needle aspiration cytology (FNAC) of the lesion was performed and reported as having features of “small blue round cell tumor.” Computed tomography (CT) of the chest, abdomen, and pelvis was normal. Axillary lymph nodes were enlarged, and FNAC had features suggestive of reactive lymphadenitis. The patient underwent wide excision with fifth carpometacarpal joint disarticulation. Histopathology of excised tissue showed small round cells with high nuclear-cytoplasmic ratio and coarse chromatin with occasional pseudorosettes, suggestive of Ewing's sarcoma. The cells were periodic acid Schiff positive and immunohistochemistry was positive for CD99 [Figure and ].\nThe patient was discharged and advised to review after 3 weeks for chemotherapy. However, the patient was non-compliant and returned about 4 months after the surgery with pain and swelling of the dorsum of the hand with dilated veins []. There was a history of fever. Blood investigations revealed hemoglobin of 9 g% and erythrocyte sedimentation rate (ESR) 42 mm at 1 h. Radiograph revealed cystic lesion in the third metacarpal diaphysis with sclerosis and minimal periosteal reaction []. MRI scan showed marrow signal changes involving second, third and fourth metacarpal bones with cortical erosion and breakdown of third metacarpal bone []. CT scan of the chest revealed bilateral multiple metastatic deposits. Tc 99 bone scan did not reveal any other site of metastasis. The patient underwent a transradial amputation about 5 cm. proximal to the wrist joint. Histopathology and immunohistochemistry of the excised specimen confirmed it as Ewing's sarcoma. Postoperatively, chemotherapy was started using cyclophosphamide, vincristine, actinomycin, and doxorubicin. The patient is surviving at 24 months of follow-up. The chest metastasis has partially resolved, and there is no local recurrence or evidence of metastasis to other sites. |
A 49-year-old man with a history of acromegaly was admitted to our hospital with the concern of recurrent shortness of breath and dyspnea on exertion during the previous 2 years, and he had experienced an episode of presyncope 2 weeks prior without any further evaluation. He was a chef in a local restaurant for almost 30 years. He had no family history of any diseases and no past history of hypertension, diabetes mellitus, sleep apnea, or sudden cardiac death. He did not smoke or consume alcohol. The patient provided a history of stereotactic radiosurgeries twice in a decade or so and adherence to treatment with a somatostatin analog (octreotide given 40 mg once per month through intramuscular injection) at the time of diagnosis 20 years before. The patient was overweight and moderately nourished. He was 1.85 m (73 inches) tall, weighed 134 kg, and had a body mass index of 39 kg/m2. His blood pressure was 110/60 mmHg, and his heart rate was 92 beats/min with sinus rhythm. He had distinct skeletal features that included prominent superciliary arches and nose bridge, enlargement of the tongue and lip, and large hands and feet. Cardiac auscultation revealed irregular premature beats and pathological third heart sound, and a systolic murmur was discovered over the apex and aortic area. Bilateral extensive borders of cardiac dullness were noted. His physiological reflexes were present without any pathology. An electrocardiogram demonstrated sinus rhythm with wide (160 ms) QRS duration of left bundle branch block (LBBB) (Fig. ). The patient’s condition was classified as New York Heart Association (NYHA) stage III–IV.\nOn admission, magnetic resonance imaging showed pituitary macroadenoma. Given the symptoms described, we arranged blood testing of myocardial injury markers showing an elevated brain natriuretic peptide level of 740 pg/ml indicating cardiac failure (Table ). Hormone laboratory tests performed subsequently demonstrated excessive secretion of GH and IGF-1, twofold greater than the reference normal upper limit, which was consistent with pituitary macroadenoma (Table ). Other routine analyses of liver and renal function were roughly normal.\nA Holter monitor was ordered for underlying arrhythmias to explain the patient’s dyspnea, chest discomfort, and presyncope. It demonstrated sinus rhythm with an average heart rate of 68 beats/min, frequent ventricular premature beats, and nonsustained ventricular tachycardia (up to 2200 ms) (Fig. ).\nA chest x-ray showed a cardiothoracic ratio (CTR) of 78%. Echocardiography showed diffuse impairment of left ventricular (LV) systolic motion, reaching an LVEF of 16%. We noted hypertrophy of the ventricular septum at 18 mm, ventricular dilation, with LV diameter of 72 mm. The right ventricle and atrium and the left atrium were also dilated with moderate mitral regurgitation and mild tricuspid regurgitation. There was no associated systolic anterior motion (SAM) of the mitral valve. Dyssynchrony of the biventricular systolic motion was apparent.\nGiven an exertional component to the symptoms together with echo presentations in order to better exclude ischemic cardiomyopathy, coronary angiography was performed, which showed normal coronary arteries without stenosis, and left ventriculography applied simultaneously revealed an EF of 20% with diffuse LV hypokinesis.\nGiven the patient’s previous medical history of acromegaly, the absence of obstructive coronary artery imaging findings or segmental dyskinesia, family history of hypertrophic cardiomyopathy (HCM), symmetric hypertrophy, as well as absence of SAM of the mitral valve, acromegaly-induced cardiomyopathy was confirmed, which was absolutely opposed to coronary heart disease (CHD) and HCM.\nThese results indicated that it was probably not a case of hereditary cardiomyopathy; therefore, we diagnosed the patient as having secondary dilated cardiomyopathy due to acromegaly, even taking it a step further progressing to congestive heart failure secondary to acromegaly-induced dilated cardiomyopathy.\nChronic excess of GH and IGF-I secretion affects cardiac morphology and performance [], so etiological treatment for acromegaly-induced cardiomyopathy is crucial to suppressing GH secretion or blocking GH action for the sake of reversing acromegaly-induced cardiomyopathy. The mainstay of treatment acknowledged globally is surgical resection of the pituitary adenoma [], which was unfortunately considered high-risk given our patient’s cardiac condition (NYHA stage III–IV). Although stereotactic radiosurgery combined with somatostatin analogs and GH antagonists administrated previously were effective in suppressing hormones, they could not help his cardiac function. Therefore, we carefully administered diuretics, vasodilators, angiotensin-converting enzyme inhibitor (ACEI), β-blockers, and spironolactone for management of heart failure following the current guidelines []; in the meantime, octreotide (200 μg/day) was administered for the control of GH excess. After good compliance of pharmacotherapy and a regular medical examination regimen for nearly half a year, the serum GH and IGF-1 concentrations decreased from 32.50 ng/ml to 1.98 ng/ml and 627.00 ng/ml to 229.10 ng/ml, respectively, but the patient was hospitalized again because of uncontrollable cardiac failure. Accompanied by the normalization of GH and IGF-1 levels, the patient’s cardiac function did not seem to take a favorable turn upon readmission. Though echocardiography showed a recovered EF value from 16% to 28%, a significant ventricular mechanical dyssynchrony was detected as formerly. Electrophysiological study was performed using a nonaggressive stimulation protocol, which revealed a nonsustained ventricular monomorphic tachycardia []. In the presence of overt ventricular dyssynchrony, complete LBBB, LVEF< 35%, inducible ventricular tachycardia, and symptomatic heart failure despite guideline-directed medical therapy, surgical indication was rarely assessed by neurosurgeons, and stereotactic radiosurgery together with pharmacotherapy produced infinitesimal effects. Therefore, we boldly recommended cardiac resynchronization therapy with defibrillator (CRT-D) implantation based on device implantation official guidelines [, ]. The patient underwent CRT insertion finally and was discharged to home 5 days later, pharmacotherapy continued as usual (Fig. ).\nTelephone follow-up was arranged, and the patient claimed symptom improvement following the device insertion 1 month later and was basically back to normal life. We required that he return for follow-up at 1 month, 3 months, and 6 months after the interventional therapy. The patient has been followed in our outpatient clinic for nearly half a year now. During his last visit, echocardiography identified improved LVEF of 54%, and a chest x-ray showed reduced CTR of 60%. The patient was in NYHA functional class II (Fig. ). |
A 62-year-old woman with a past medical history of obstructive sleep apnea, hypertension and no toxic habits, presented with an 18-month history of progressive pain, swelling and enlarging mass on the dorsum of her dominant right wrist. Patient reported that the mass progressively changed from a soft to a hard consistency during this time, producing a restriction on the range of motion (ROM) at the hand. Two years before our initial visit, she had a non-displaced triquetrum fracture after falling from standing height at her home ().\nAt evaluation, a non-obese (body mass index = 24) patient with a palpable, soft and non-tender mass on the dorsum area of her right wrist was noted. The right hand had no neurological or vascular compromise. She had swelling and pain in dorsal wrist, with limited ROM of fingers at active and passive extension. Laboratories studies were negative for rheumatologic disease. Radiographs revealed a partially calcified mass in the dorsal aspect of the wrist concerning for a possible osseous or cartilaginous lesion (). A magnetic resonance image (MRI) of the right wrist showed an extensor tenosynovitis and a heterogenous lesion (measuring 2.6 × 1.3 × 2.5 cm) at the level of the first carpal row; suggestive for focal pigmented villonodular synovitis (PVNS), soft tissue chondrosarcoma or TC ().\nAfter MRI results, an initial open intralesional biopsy was performed due to the assessment of an indeterminate soft tissue lesion with high probability of malignancy []. The pathology report from initial biopsy revealed a TC lesion. Upon diagnostic confirmation, patient was oriented on the prognosis and scheduled for an open mass excision. At surgery, patient was blocked with a supraclavicular anesthesia. A dorsal surgical incision was done at the previous initial biopsy site, along with a wide dissection of extensor tendons and extensor tenosynovectomy. The extensor retinaculum was surgically opened and a wrist joint arthrotomy was performed. At further exploration, a 2.2 × 1.3 cm soft tissue tannish trabeculated rubbery mass was excised from the carpus with an extensive curettage ( and ). The joint capsule was irrigated, and extensor retinaculum and adjacent tissues were rearranged for wound closure. Patient tolerated procedure without major complications. The pathology specimen was sent to National Institute of Health which revealed similar findings to initial biopsy, confirming a well-differentiated lesion consistent with TC.\nAt 1-week postop visit, patient had significant improvement of associated pain symptoms, wounds healed appropriately, and an adequate hand ROM returned. After 2 years of follow-up, patient continues to do well, and no recurrence or metastasis have been observed (). |
63 year old male with history of active heroin abuse, non-compliance, hypertension, obesity, CHF, hypercholesterolemia, multiple prior intraabdominal surgeries, including appendectomy, cholecystectomy, several ventral hernia repairs, long history of large ventral hernia, presented with worsening abdominal pain and distention for 2 weeks. Two days prior to admission, he noticed that his ventral hernia on right side of the abdomen became more swollen and painful. In the ER he became obtunded, developed severe respiratory distress and required endotracheal intubation. Subsequent CXR showed free intraabdominal air. Plain abdominal radiograph showed markedly distended cecum pointing to the left upper quadrant and free air (). At this point surgical consultation was obtained. Vitals: T 99, BP 174/90, pulse 115, respirations 12. Clinical exam showed distended abdomen and very large tender hernia occupying all right side of the abdomen and extending to the left of midline with redness and warm skin above it. Patient had multiple surgical scars and another 2 smaller hernias in lower abdomen. Abnormal laboratory results: WBC 15,000, creatinine 4.5. Subsequent CT of the abdomen and pelvis without contrast showed tremendous amount of free air and markedly distended cecum, partially contained in the ventral hernia and crossing midline to the left, suspicious for cecal volvulus (). Patient was immediately taken to the OR, where diagnosis of cecal volvulus was confirmed. Cecum, terminal ileum, and ascending colon were twisted in axial plane, with concomitant clockwise torsion and grossly perforated hepatic flexure, significant fecal spillage and large abscess cavity along the transverse mesocolon. Size of the hernia was approximately 20 × 30 cm. Hernia contained part of the cecum. Right colon had significant adhesions from prior surgeries, including those inside the hernia sac. Patient had loss of the domain. There was another lower abdominal wall hernia containing old infected mesh and communicating with major right sided abdominal wall hernia defect. The volvulus was reduced in counterclockwise direction. Extensive lysis of adhesions, right hemicolectomy, abdominal washout, explantation of infected mesh was performed. Because of extreme condition of the patient, damage control procedure was chosen and abdomen was temporally closed with negative pressure (VAC) dressing. Patient remained intubated in ICU. After 48 h of resuscitation patient improved: pressors were discontinued and he had good urine output. Patient was taken back the OR. Reopening of recent laparotomy, ileocolostomy anastomosis and ventral hernia repair with biological mesh was performed. Repair of abdominal wall defect was challenging because of the size of the hernia. Despite meticulous dissection, we had 10 × 12 cm defect in the middle, which was not possible to approximate. This area was bridged with one piece of biological mesh. Another bigger piece of mesh (same type) was placed above the bridged area to have 7–8 cm overlapping from the edges of the hernia. Mesh was secured to the fascia with unabsorbable sutures and titanium tackers. Patient had successful recovery. He was extubated 5 days later, started on regular diet. All drains were removed before his discharge to rehabilitation center. A week after his discharge, patient was readmitted with pneumonia, and CT of the abdomen was performed in the ER. CT showed no bowel obstruction and no hernia (). Patient was discharged to home in stable condition and was followed in the clinic without evidence of recurrence during 4 months after surgery. |
A previously healthy 57-year-old woman, with no significant past medical history, presented to the surgical department of our hospital for definite management of a primary pancreatic leiomyosarcoma, after being treated with adjuvant chemotherapy.\nOne year before her last admission, she was initially admitted to our emergency department due to abdominal pain, fatigue, and weight loss. She was totally healthy prior to these symptoms. She then underwent magnetic resonance imaging (MRI) that was indicative of a pancreatic head lesion along with possible metastatic liver lesions, superior mesenteric vein occlusion, and portal vein infiltration (Fig. a, b). The decision was to undergo an endoscopic ultrasound (EUS) biopsy in order to determine the exact nature of the lesion. EUS report was indicative of pancreatic leiomyosarcoma.\nMultidisciplinary team’s decision was to use gemcitabine- and docetaxel-based chemotherapy as up-front treatment to assess tumor response. Follow-up CT scan and magnetic resonance imaging (MRI) after the completion of chemotherapy regimen showed downsizing of the pancreatic mass, as well as downsizing of suspicious for malignancy segment III liver lesion (Fig. c, d).\nBased on the response to chemotherapy, tumor characteristics, and physical status of the patient, multidisciplinary team’s decision was to proceed to surgical exploration. Due to local expansion of the pancreatic tumor, its relation with the superior mesenteric and portal vein, and the underlying SMV thrombosis, excision of the pancreatic tumor was not feasible. Intraoperatively, a small piece of tumor was excised in order to be sent for histopathology. Surgeon’s decision was to ablate the tumor with irreversible electroporation (Fig. ). Metastatic liver lesions were identified with the use of intraoperative ultrasound. Segment III liver lesion was resected, while smaller lesions of the right lobe were ablated using microwave ablation.\nThe patient had an uneventful postoperative recovery and complete resolution of her symptoms. Histopathological examination of pancreatic lesion as well as segment III liver lesion revealed sarcomatous tissue of high cellularity with fascicular pattern, increased mitotic activity, and diffuse cytoplasmic immune reactivity for SMA, desmin and h-Caldesmon, and chromagen DAB (Figs. and ). Surprisingly enough, pathological report of a smaller liver lesion was indicative of angiomyolipoma staining positive for HMB45 and Melan-A. The lesion was a benign hamartomatous, circumscribed but unencapsulated hepatic mass composed mainly by mature lipocytes and limited mesenchymal component (smooth muscle cells), showing no marked atypia and thick-walled vasculature. Myoid component was positive for ΗΜΒ-45 and Melan-A. Based on the histopathological report, tumor board decided that the patient should be treated with adjuvant therapy for leiomyosarcoma after surgery. A regimen with anthracycline and olaratumab was used for 3 months. Follow-up imaging in 6 and 12 months showed no progression of the disease (Fig. a–d). |
A 23-year-old female patient was referred to our institution with a huge mass on the posterior scalp near the midline. The patient's parents gave the history that the mass on the posterior scalp was present since birth which gradually increased to the present size.\nThe swelling was painless and was gradually enlarging. On examination, there was a well-demarcated solitary pear-shaped sessile swelling in the occipital region near the midline measuring 7 cm × 6 cm in its greatest dimension []. On physical examination, no other swelling was noted elsewhere in the body.\nThe skin overlying the swelling was normal in color and texture but slightly stretched. The absence of hair on the surface of the swelling was noticed. There was no local rise in temperature associated with the swelling. The swelling was nontender, soft and putty in consistency, and mobile over the underlying structures. The surface of the swelling was lobular with smooth bumps. The swelling was fluctuant, compressible, and nonpulsatile. On radiographic examination, a soft tissue shadow of the cyst was seen at the occipital region without any bony erosion.\nOn the basis of the clinical examination, a provisional clinical diagnosis of lipoma was made.\nFine-needle aspiration cytologic (FNAC) was carried out to evaluate the nature of the contents of the cyst. Contents of the lesion were aspirated using a wide bore needle and was sent for microscopic examination. The section revealed the presence of numerous cells with empty cytoplasm and peripherally placed nucleus resembling adipocytes. Few keratin strands were also seen in the section. The FNAC diagnosis was given as lipoma.\nSurgical excision of the cyst was then planned under general anesthesia. The specimen was sent for histopathological examination [].\nSoft tissue was received in 10% formalin. The tissue was soft, putty, and fluctuant inconsistency. On cutting the mass, muddy paste got extravasated [], which resembled soggy keratin. The histological section revealed cyst wall and a cystic lumen with overlying epidermis []. The cyst wall showed signs of rupture and disintegration. The overlying cystic epithelium is disintegrated due to inflammation and was not evident in the section.\nKeratin flecks were seen with squamoid cells []. Spillage of keratin in the surrounding connective tissue provoked giant cell reaction, and many cholesterol clefts are also evident throughout the section that is usually referred to as “keratin granuloma” [].\nThe histopathological diagnosis of “epidermoid cyst” was given. |
Raymond was a 40-year-old male referred to a specialist service for adults with psychosis and complex mental health needs in one of the National Health Service Trusts in East Midlands, UK, for an individual psychotherapeutic input. Raymond was diagnosed with paranoid schizophrenia and a comorbid anxiety disorder (not otherwise specified). A number of ongoing symptoms were reported, including derogatory and threatening auditory hallucinations, paranoid delusions, high anxiety levels, and social avoidance and withdrawal. Past and ongoing interventions consisted of pharmacotherapy with a maintenance dose of antipsychotics and tranquilisers, social inclusion activities facilitated by a community based team, and recurrent crisis oriented admissions to acute mental health wards.\nSince early adolescence, Raymond regularly used excessive amounts of alcohol and cannabinoids, which initially seemed to be his way of conforming to peer pressure in the deprived area where he lived. He received strict upbringing from his father; thus spending hours in pubs appeared to function as an avoidance of exposure to distressing stimuli at home. With time, Raymond became dependent on the use of illicit substances. He was trained as a builder and enjoyed his work. Yet, after the onset of psychosis Raymond gave up his trade. His first episode of hearing voices occurred at the age of 30 and involved his first admission to an acute mental health ward, where he underwent an alcohol detoxification. Raymond has managed to remain abstinent from alcohol since and yet continued to use cannabinoids on a regular basis. After a few years of remission, the second episode of psychosis occurred and was followed by another inpatient admission. Subsequently, Raymond remained abstinent from cannabis as well. However, auditory hallucinations persisted on a daily basis. Additionally, Raymond developed a range of paranoid appraisals of voices and delusional beliefs about other people's vicious intentions towards him, which precipitated social withdrawal and triggered high anxiety levels. At the time of referral, Raymond lived isolated on his own in a house, where he had installed surveillance cameras and barricaded his bedroom at nights. He was unemployed and in receipt of social benefits.\nAs in generic cognitive-behavioural models, assessment in CBTp aims to evolve into a case formulation; hence a range of cognitive interview methods were employed. Furthermore, to formally assess the person's symptomatic presentation and evaluate the intervention outcomes, a standardised psychiatric measure, the Brief Symptom Inventory (BSI), was administered with the client. The BSI is a 53-item self-report inventory, which has been designed to reflect the symptom patterns among mental health in- and outpatients. Each BSI item is rated on a five-point scale (0–4) reflecting a person's distress from “not at all” to “extremely.” The BSI is a measure of the current symptom status and is scored on the following subscales: somatisation, obsessive-compulsive, interpersonal sensitivity, depression, anxiety, hostility, phobic anxiety, psychoticism, and paranoid ideation []. The BSI was shown to demonstrate sufficient psychometric properties. Normative samples for BSI included 1002 adult psychiatric outpatients, 974 adult nonpatients, 423 adult psychiatric inpatients, and 2408 adolescent nonpatients; internal consistency was established using Cronbach's alpha coefficients for all nine dimensions, which ranged from 0.71 to 0.85, while test-retest reliability coefficients were estimated between 0.68 and 0.91 []. Internal structure and construct validity were found to be adequate: orthogonal varimax loadings determined from principal components analysis ranged from 0.35 to 0.71 []. Convergent and discriminant validity was examined through comparison with the Minnesota Multiphasic Personality Inventory; correlation coefficients scoped from 0.31 to 0.72 []. The BSI has also been standardised and normalised on the British population [, ].\nFor the purpose of Raymond's assessment, the BSI subscales of paranoid ideation (PAR) and anxiety (ANX) were administered. Raymond's preintervention PAR score was 2.20 and his ANX score was 2.50. Both scores were elevated by more than one standard deviation above the UK outpatient mean and indicated heightened levels of difficulties in both symptom areas.\nAs depicted in , a cognitive model of psychosis with comorbid emotional distress and safety behaviour developed by Jones [] was employed to formulate Raymond's symptomatic experiences and to illustrate his difficulties in a diagrammatic form.\nDuring the assessment, Raymond identified a number of triggers. He noticed that he would hear voices while he was bored at home, kept silent, or had nothing to occupy his mind with. Furthermore, the voices would become particularly active in the late evening, upon nightfall. The voices felt like they were coming from inside of his head and were screaming derogatory and threatening comments. Raymond misinterpreted the voices as hearing someone else's thoughts and was becoming increasingly delusional in his beliefs about other people, including his neighbours and random pedestrians. In consequence, he experienced elevated emotional, cognitive, and bodily symptoms of anxiety and employed a range of safety behaviours. These, in turn, contributed to his hypervigilance and preoccupation with the voices and prevented disconfirmation of his paranoid beliefs.\nBased on the recommendations made in the clinical guideline for schizophrenia, 16 sessions of individual CBTp were contracted. The following intervention goals were agreed on: enhancement of strategies to cope with voices, paranoid/delusional beliefs and anxiety, and reestablishment of autonomy at nights. The intervention plan was informed by a CBTp manual [] and incorporated the following treatment modules: psychoeducation, cognitive restructuring of delusional appraisals of voices, behavioural training (graded exposure), cognitive therapy for secondary symptoms (comorbid anxiety), and self-management planning (relapse prevention).\nBased on the formulation diagram, the cognitive model of psychosis was thoroughly discussed. Raymond was educated about the significance of cognitive mediation in psychosis, where particular appraisals of voices predict individual distress and coping behaviour []. In Raymond's case, persecutory beliefs triggered high anxiety levels, avoidance, and escape-type reactions. The crucial role of safety behaviour in maintaining anxiety and preventing the disconfirmation of delusions [] was also discussed.\nFurthermore, the concept of “punishment paranoia” [] as a cognitive representation of fundamental concerns in one's life was brought to Raymond's attention. Also, relevant outcomes of cognitive neuroscience research in auditory hallucinations were discussed, particularly, the evidence for subvocalisation that accompanies the experience of hearing voices, suggesting that auditory hallucinations might be misattributions of internal mental events [].\nIn order to compassionately challenge and restructure the paranoid appraisals of auditory hallucinations, this module of the treatment began with evidential analysis of the content of delusional beliefs []. Guided discovery technique was regularly used with good effects. Raymond found no evidence to support his persecutory beliefs about voices. Contrary to his appraisals, Raymond realised that he had remained safe and had not been attacked or even threatened by anyone for numerous years.\nOnce the initial doubt in delusional explanations was instigated, cognitive restructuring proceeded to the reattribution of beliefs about voices []. In this stage, Raymond recognised the presence of recurrent negative internal dialogues he conducted with himself in his thoughts, which frequently precipitated the experiences of auditory hallucinations. The voices, in turn, seemed to represent his essential worries about his life, for example, “I've wasted my life.” Subsequently, Raymond made a pragmatic use of psychoeducational discussions on the subjects of misattribution of internal mental events and punishment paranoia. He developed an understanding of functional associations between voices, persecutory cognitions, and his own concerns and expressed his disappointment with how he had conducted his life since adolescence, hence incorporating the new knowledge he had gained so far in therapy.\nSubsequently, Raymond was encouraged to practice identification of internal dialogues on a daily basis. Such dialogues occurred mainly in the moments of boredom. To restructure such unhelpful and dysfunctional cognitive experiences, Raymond begun implementing a range of modified self-statements [], which were initially agreed on with the therapist, for example, “I do not need to be punished for anything, as I have never hurt anyone. I have already improved my life, quit drinking and drugs, and deserve to be happier.” From this point in therapy, Raymond regularly practiced reframing the persecutory appraisals of his experiences, identifying anxiety-inducing cognitions and replacing them with evidence and modified self-statements.\nFollowing completion of the cognitive restructuring module, Raymond reported a noticeable reduction in his experiences of anxiety. Subsequently, he voiced a growing readiness to relax a range of his safety behaviours he employed predominantly in the night time. Hence, behavioural experiments were used to address the unhelpful behaviour maintaining the cycle of paranoid appraisals of voices and comorbid emotional distress []. The overarching assumption tested was “If I do not remain isolated and vigilant, activate the security system and barricade the bedroom, then I'll be assaulted.” Initially, testing such assumption appeared too challenging for Raymond; thus behavioural experiments were employed as a series of graded exposure tasks []. Consequently, the assumption was reframed stepwise and tested gradually, that is, through removal of doorstop, unlocking bedroom door, substituting watching security cameras in the evening with watching movies, and eventually removing barricade on subsequent nights and then every night. Raymond's engagement with social situations, such as casual walks, grocery shopping, and family visits, was encouraged via graded exposure tasks, as well.\nBehavioural experiments utilised through graded exposure affected further improvements in Raymond's psychosocial functioning. Tested assumptions were disconfirmed and replaced with “These are just my habits that are so difficult to break.” Raymond's safety behaviours relaxed considerably, and some were eventually abandoned.\nFollowing completion of cognitive and behavioural interventions for the symptoms of psychosis, Raymond experienced a considerable reduction in his psychological distress and reported low anxiety levels. Therefore, it was no longer necessary for the symptoms of comorbid anxiety to be addressed in a separate module of the intervention.\nThe self-management planning focused initially on recognising early warning signs for antecedents of derogatory voices and addressing them accordingly. Organising activities in the evening was already addressed in earlier therapy stages. Hence, prevention of boredom became the focus towards the end of therapy. A family meeting was organised with the community-based mental health team and plans were made for gradual reinstatement of Raymond's interaction with his relatives and acquaintances. Furthermore, during the course of intervention, Raymond reflected on his life and realised that, despite his intellectual capacities and learning potential, he never felt confident enough to undertake further education. Since his abilities to cope with voices and persecutory beliefs increased considerably and anxiety levels reduced, Raymond decided to explore evening courses provided in the local college and pursue further qualifications. Finally, a CBTp self-help guide [] was introduced to support the client's continuous recovery.\nSubjectively, Raymond reported numerous substantial improvements in his psychological functioning at the end of therapy. Some of his pre- and postintervention comments, evidencing subjective importance of the therapy outcomes, are quoted in .\nNot only did the client's strategies to cope with voices, paranoia, and anxiety improved considerably, but also, as illustrated by the pre- and postintervention behaviour frequency samples (), the second therapeutic goal, that is, reestablishment of autonomy at nights, was achieved as well.\nFurthermore, as shown in , the posttreatment administration of BSI confirmed substantial reduction in both paranoid ideation and anxiety levels, as the client's scores dropped below the UK outpatient means. |
A 10 year old white girl with severe (class 3: BMI ≥140% of the 95th percentile for age and sex) obesity and otherwise normal development presented to the Pediatric Weight Management Clinic with her mother. The mother reported that the patient had been at the 75th percentile for height and weight for most of the patient's life but she experienced a “20 to 30 pound” weight gain over the past year. The mother further explained that this recent weight gain coincided with treatment of seasonal allergies with montelukast and she wondered if this may have been the cause of the weight increase. The patient had no prior weight loss attempts.\nThe patient was born full term, weighing 3.18 kg. The mother's pregnancy was uncomplicated, as was the patient's newborn course. Aside from seasonal allergies, the patient was healthy. She had no history of hospitalizations, surgeries, or mental health concerns. She was not taking any medications.\nThe patient was eating regularly-spaced meals consisting primarily of highly processed foods and simple carbohydrates (e.g., pastries for breakfast, potatoes with cheese for dinner). The family was eating fast food three times per week on average. The patient endorsed having a big appetite and feeling hungry all the time. She was eating while watching TV and when bored. She denied binge eating, loss of control eating, emotional eating, sneaking/hiding food, or eating during the night. Her physical activity was limited to gym class at school three times per week.\nThe patient was living with her mother and her mother's partner. The patient's parents divorced when she was very young and the mother's partner had been living with them since the patient was a toddler. The patient saw her biological father rarely. She had no siblings. She was attending fourth grade and enjoyed reading and writing. The mother and her partner worked full-time and the patient was cared for by a baby sitter after school a few times per week. They had no food insecurity. The family history was notable for obesity in both biological parents and type 2 diabetes in the maternal grandmother.\nThe patient's review of systems was negative. She reached menarche several months prior to presentation. On physical examination, her weight was 70.31 kg (155 lbs.), height was 142 cm (4'8”), and BMI was 34 kg/m2 (145% of the 95th percentile). Her blood pressure was 105/65 mmHg and pulse was 74 beats per minute. Her physical examination was normal. The results of her fasting labs were: total cholesterol 176 mg/dL (normal: < 170 mg/dL), HDL-c 49 mg/dL (>45 mg/dL), LDL-c 96 mg/dL (< 110 mg/dL), triglycerides 157 mg/dL (< 90 mg/dL), ALT 27 (< 50 U/L), AST 29 (< 50 U/L), glucose 98 mg/dL (70-99 mg/dL), and HbA1c 5.5% (0-5.6%). Her Pediatric Symptom Checklist (routinely obtained in the Pediatric Weight Management Clinic) score was 8 (> 28 is considered abnormal).\nThe patient and family were started on a program of lifestyle modification therapy and responded particularly well with decreasing fast food consumption and liquid calories. Further, the patient started bringing her lunch to school instead of eating the school fare and was able to keep a food log almost daily. The patient's physical activity, however, continued to be limited. Over the course of 5 months, the patient's BMI decreased 5 units (15%), from 34 kg/m2 to 29 kg/m2 (145% of the 95th percentile to 125% of the 95th percentile).\nAt the end of the 5 month period, coinciding with the end of the school year and beginning of summer vacation, the patient's sleep/wake cycle became irregular. Because she did not like the hot weather, she chose to stay inside all day. Her mother left prepared meals for the patient to encourage healthy eating while mom was at work. Despite this, the patient's BMI began to trend upward from 29 kg/m2 to 31 kg/m2 over the summer months. Upon school resuming in the fall, the patient's sleep/wake cycle normalized and eating behaviors and patterns improved, returning to those of the previous school year. The patient's BMI stabilized for a few months but then increased further. The patient expressed frustration because she believed that she was eating well, which was indeed reflected in her daily food logs. She continued to attend monthly visits with the Pediatric Weight Management Clinic dietician, psychologist, and pediatrician with specialized training in obesity medicine. Yet, the patient's BMI continued to increase such that by 2 years after her initial appointment, the patient's BMI returned to baseline (135% of the 95th percentile) (see Figure ).\nSuspecting that metabolic adaptation was causing the patient's weight rebound, adjunct pharmacotherapy was recommended. Orlistat was considered but not started because of concern about gastrointestinal side effects and lack of insurance coverage. Metformin may have been another reasonable option but the patient's fasting glucose and HbA1c were in the normal range and she did not have acanthosis nigricans on physical examination which would have suggested insulin resistance. She was ultimately started on topiramate 75 mg daily in addition to ongoing LSMT. She and her mother were cautioned that although topiramate is not FDA-approved for the indication of obesity (in children or adults), multiple studies have demonstrated clinically-meaningful weight loss efficacy in adults. Additionally, it was explained that the side effect profile in children is well established stemming from its use for epilepsy treatment.\nAfter 4 months of treatment with topiramate, the patient's BMI trajectory plateaued yet was not decreasing as was desired. Recognizing that the combination of topiramate and phentermine is the most effective weight loss medication currently available for adult obesity, phentermine 15 mg daily was added to the topiramate 75 mg daily. The patient and mother were informed that phentermine is FDA-approved only for individuals older than 16 years and for “short-term use.” With combination treatment for ~22 months, the patient experienced good BMI reduction, from 34.1 to 25.7 kg/m2. Her blood pressure and heart rate were monitored regularly and though her blood pressure did not increase, her heart rate increased slightly from 60 to 70 s, in line with the mechanisms of action of phentermine (stimulant-like effects). Later, the patient reported that she was experiencing some “memory” issues but noted no change in her academic performance. Although it seemed unusual for this type of symptom to emerge 10 months after starting topiramate, the topiramate dose was decreased from 75 to 50 mg daily and the memory issues resolved. Written informed consent was obtained from the parent of the patient for the publication of this case report. |
The patient, a 16-year-old man, presented to our orthopedic outpatient clinic with right knee pain and occasional locking after one basketball game 3 mo ago.\nThe pain appeared first after one basketball game, was followed by locking, and progressively worsened over 3 mo. During this period, the patient received pain medication and physical therapy, which failed to relieve his symptoms. The patient presented with right knee pain and occasional locking and underwent an MRI examination at the outpatient clinic.\nThe patient had tenderness and swelling in the right knee, which was worse when the limb was moved with flexion or extension. The neurovascular status was normal.\nMRI (3.0 T) was used to assess the lesions of the right knee. T2-weighted imaging revealed localized cartilage and corresponding subchondral bone defects in the lower right femur with BMLs below and in front of the femoral condyle. The joint cavity and suprapatellar capsule had a large amount of fluid. The loose body was formed from partially low-signal subchondral bone and medium- to high-signal cartilaginous material (Figure ).\nThe patient was diagnosed with OCD based on symptoms, physical examination, and imaging and was scheduled for subchondral bone implantation combined with intra-articular injection of PBSCs. The patient's right knee was braced and braked with the knee-aiding system preoperatively. The loose body was not removed prior to injection due to the patient's lack of surgical intent.\nThe patient was subcutaneously injected with recombinant human granulocyte stimulating factor (150 μg each time, twice a day for 5 d). When the patient's white blood cell count was > 25 × 109, 50 mL of peripheral blood stem cell suspension was collected with a COM. TEC blood cell separator (Fresenius HemoCare Company). Forty milliliters of stem cell suspension and an equal volume of normal saline were mixed and diluted 1:1, and the diluted stem cell suspension was packed in 16 split-core tubes in equal volume. Five milliliters of human peripheral blood lymphocyte separation solution was added into each split-core tube and centrifuged at 2000 r/min for 20 min at room temperature (eccentric radius: 7.5 cm). The sucked stem cell layer was transferred to another centrifuge tube, and centrifuged at room temperature at 1800 r/min for 20 min (centrifugal radius: 7.5 cm). The supernatant was discarded to obtain relatively pure stem cells. The CD34+ cell count in the purified solution of peripheral blood stem cells was 3 × 107/L.\nBased on the MRI results, the location of the lesion area was marked with fine metal wires on the surface of the knee in both the frontal and lateral positions. With routine disinfection and anesthesia of the surgical area, a bone marrow needle was percutaneously punctured to the subchondral bone site under C-arm fluoroscopy, as shown by MRI via the lateral knee approach. When the needle made contact with the subchondral bone, the needle was rotated to the left and right, slowly drilling into the bone. After the puncture needle was fixed in the subchondral bone, the needle core was removed, and the syringe was attached to inject PBSCs into the subchondral bone through the needle channel. Removing the needle, pressure was applied to the puncture point to stop bleeding for 5 min. The operation is illustrated in Figures and .\nThe patient's right knee was braced with the knee-aiding system to prevent further damage due to knee instability for 1 mo. During this period, the patient was advised to complete 20-30 min of a range of motion knee flexion and extension exercises once a day. Then, the patient was allowed to partly bear weight and gradually transitioned to weight-bearing as tolerated. After 2 mo, the patient was allowed to conduct mild physical activity within tolerable limits and gradually increased activity levels. |
Informed patient was consent was obtained for this patient's treatment.
We present a case of a 55-year-old male with no significant past medical history, who presented to emergency department with a recent onset of gait imbalance, as well as subjective numbness and hypoesthesia of the bilateral lower extremities. He had sustained multiple falls during the previous two weeks prior to admission. Shortly after admission, he developed urinary retention. An MRI of the thoracic spine revealed an approximately 2.9 x 3.8 x 3.7 cm extradural midline mass centered in the posterior elements of T5 (Figures -).
The mass extended into the spinal canal with severe thoracic cord compression. There was increased signal within the spinal cord from the lower T4 level to the upper T6 level consistent with edema. The enhancement pattern was fairly homogeneous, with extradural enhancement extending superiorly to the T3-4 level and inferiorly to the T6-T7 level. No extension into the neural foramina was noted. The subsequent radiographic and metastatic workup was negative for a primary malignancy or any other metastatic lesions. Over the course of the first twelve hours of admission, the patient’s neurological exam continued to decline with a deterioration of his sensory symptoms. He was then given 10 mg of intravenous dexamethasone and surgical planning was undertaken.
Hospitalization: The patient was taken to the OR under general anesthesia. He was positioned prone on the Jackson table. Under fluoroscopy, we identified the level of the T5 spinous process, counting proximally from the sacrum. The incision was made over the spinous processes from T4-T6. The fascia was incised on both sides of the spinous processes. We performed a subperiosteal muscle dissection on both sides simultaneously. The tumor was readily visible anteriorly to the spinous process of T5, which was partly involved in the tumor. We dissected around the tumor with good visualization of bone lateral to the |
A 43-year-old female patient, presenting unilateral cleft lip and palate (UCLP), sought care at the Hospital for Rehabilitation of Craniofacial Anomalies (HRAC-USP). Her complaints were difficulties in functioning, poor esthetics, and poor prostheses (two complete dentures), since the maxillomandibular relationship was distorted ( and ). Her psychological state was also a concern, and the patient’s expectations were high.\nSix implants were placed in the maxilla and four in the mandible ( and ). Maxillary and mandibular protocols were planned and mandibular full arch prosthesis was concluded before the orthognathic surgery, to guide the occlusion.\nThe first prosthesis was fabricated without consideration for the correct maxillomandibular relationship. Conversely, an adequate relationship between the prosthesis with soft tissue and lip support was achieved. This new prosthesis was fabricated in only acrylic resin, without metal framework, and its primary function was anchored to the orthognathic surgery.\nSince the maxilla is presented in two segments, the maxillary prosthesis was sectioned to facilitate the orthognathic surgery planning, and leveling the maxilla (). The newly achieved position was registered () and duplicated, and two casts were obtained and mounted in a semi-adjustable articulator () to aid in the planned surgery ().\nDuring surgery, the maxilla and the mandible were positioned correctly. A guide was used to direct this new position, and a new maxillomandibular relationship was established. Note that the implants inserted before the surgery were important in assisting the surgeon to obtain and maintain the suitable maxillomandibular position.\nAfter 3 months, as expected, the maxillomandibular relationship relapsed (). A new maxillary complete prosthesis, with a favorable maxillomandibular relationship and metal framework, was carried out to correct the occlusion ().\nThe case was concluded after orthognathic surgery and the patient was satisfied with the cosmetic and functional outcome and was reintegrated into society. Two years later we can see the stability of the case report (, ). |
A 50-year-old male house painter with longstanding shoulder pain and glenohumeral arthritis refractory to conservative management underwent a TSA with press-fit humeral stem (Global Unite, Depuy, Warsaw, IN, USA) and uncemented glenoid component (Anchor Peg Glenoid, Depuy, Warsaw, IN, USA) in his nondominant extremity. His preoperative imaging revealed a biconcave glenoid with 20° of glenoid retroversion and posterior subluxation of the humeral head []. Intraoperatively, the anterior glenoid was reamed preferentially in an attempt to correct the posterior retroversion of the biconcave glenoid prior to placement of the glenoid component. Postoperative imaging revealed a well-fixed glenoid component with persistent 20° of retroversion []. The humeral component was placed according the native humeral version. Intra-operative examination following component implantation revealed a stable glenohumeral joint and no additional corrections or modifications were performed. Postoperatively, the patient had a sense of instability coupled with pain and weakness. While he did not experience frank dislocation episodes, the pain and dysfunction prevented him from returning to work. His examination revealed posterior apprehension, but normal strength of both the deltoid and rotator cuff musculature. He did not improve despite 2 months of dedicated physical therapy. Given the clinical history, imaging and exam findings, the patient was diagnosed with posterior instability as a result of glenoid retroversion.\nPotential options for treatment in this situation included revision of the glenoid component with bone grafting or placement of a posterior bone block with retention of the glenoid component. Given the amount of morbidity involved in a revision surgery, a less invasive and alternative method of treatment was chosen. An arthroscopic posterior bone block procedure was performed using an iliac crest graft.\nFour months following his index arthroplasty surgery, the patient returned for revision surgery. He was placed in the beach chair position. A 25 mm × 10 mm × 10 mm tri-cortical bone graft was harvested and prepared. Shoulder arthroscopy was then performed using standard portals. Verification of glenoid fixation was confirmed, and a horizontal slit in the posterior rotator cuff muscles was performed. In addition, the bone on the posterior glenoid was prepared to a flat surface with an arthroscopic burr []. Utilizing a custom double cannula instrument (DePuy-Mitek, Raynham, MA, USA), the graft was advanced through the widened posterior portal []. Graft was then precisely placed on the posterior glenoid with the surface of the bone block placed parallel and flush with the surface of the glenoid prosthesis []. Fixation of the bone block was performed with two parallel, cannulated, titanium, 3.5 mm screws. Given the existing Anchor Peg Glenoid component (Dupuy, Raynham, MA, USA) relies heavily on the central peg, care was taken to ensure that both screws lie below the equator of the glenoid prosthesis []. This was critical as damage to the central peg risks compromising the fixation of the glenoid component. By placing the 10 mm wide graft flush with the size 48 mm glenoid, the anterior to posterior articular surface area was effectively increased by 37%.\nAt 14-month postoperatively from the bone block procedure, the patient regained near full motion and strength. He has no sense of pain or instability with resisted forward flexion with his arm in an adducted and internally rotated position []. The patient's subjective shoulder value is 80, with a 2 out of 10 pain score. Imaging obtained at 14-month revealed osseous integration of the bone block to the posterior glenoid, no evidence of glenoid prosthesis loosening and an intact central peg []. |
Our patient was a 64-year-old male with a past medical history remarkable for multiple urinary tract infections, urosepsis, and transverse myelitis diagnosed one year prior to presentation, as well as deep vein thrombosis treated with anticoagulation. He presented with a decreased level of consciousness of one day's duration and personality changes (low anger threshold and mood changes) for three weeks. The patient had never had these episodes before. He had a blood pressure of 130/70 mmHg, heart rate of 89 beats per minute, temperature of 36.7 °C, respiratory rate of 18 breaths per minute, and oxygen saturation of 97% on room air. Of note, the patient had an altered baseline cognitive function and required assistance for performing his daily activities.\nOn examination, he was comatose with a Glasgow coma scale score of 6. There was significant cogwheel rigidity of the left upper extremity with a near-normal tone on the right side. Bilateral atrophy of the lower extremities, more pronounced on the left, was noted with bilateral foot drop and positive Babinski sign, bilaterally. Blood cell counts, hepatic enzymes, ferritin, blood urea nitrogen (BUN), urine drug screen, and creatinine levels were all within normal limits. Urine and blood cultures were drawn and sent but returned negative. CT scan of the head was performed and demonstrated an intracranial hemorrhage measuring 2 x 1.2 cm, with 6.5 mm extension craniocaudally, in the right temporal lobe. Figure demonstrates the large area of associated edema.\nThese findings were confirmed by an MRI of the brain, which also revealed an additional area of hemorrhage in the right frontal lobe, with a large amount of surrounding edema but no midline shift, and smaller hemorrhages in the left hemisphere suggesting vasculitis (Figures -).\nLumbar puncture was performed and repeated three weeks later. Their results are summarized in Table .\nInitial vasculitis workup was negative for antinuclear antibody (ANA), complement component 3 (C3), and antineutrophil cytoplasmic antibodies: P-ANCA, C-ANCA. Syphilis, hepatitis B and C, West Nile virus antibody [immunoglobulin G (IgG) and immunoglobulin M (IgM)], herpes simplex virus (HSV) polymerase chain reaction (PCR), HIV 1 and 2, and hypogammaglobulinemia with serum immunofixation levels were also negative.\nIn view of CT and MRI findings suggestive of vasculitis, and a vague initial presentation, a brain biopsy was performed for further workup. The biopsy revealed angiocentric granulomatous inflammation with focal vessel disruption and associated parenchymal hemorrhage, consistent with a diagnosis of granulomatous vasculitis (Figures -, -).\nThe anticoagulation was discontinued and an inferior vena cava filter (IVC) filter was placed. The patient received levetiracetam 1,000 mg/day, multiple high doses of steroids, and six cycles of cyclophosphamide, each administered one month apart. The dosage of cyclophosphamide was 870 mg/m2 per cycle in the first three cycles followed by 945 mg/m2 for the subsequent cycles.\nThe patient was followed up every four to six weeks during the first two years, and subsequently every four months until now. After induction, he has remained in remission without any maintenance therapy to date (eight years post-presentation). |
A 60-year-old man with a past history of RCC (clear cell type, G2, T1b N0 M0 Stage I) treated by a right nephrectomy in June 2015 was required to have a follow-up examination at 6-month intervals after surgery, without the use of an anticancer agent. In January 2018, a routine gastrointestinal endoscopy found an ulcerative lesion of approximately 10 mm diameter in the greater curvature of the gastric body (Fig. ). An endoscopic ultrasonography (EUS) of this lesion showed the first three sonographic layers were blurred, which suggested submucosal invasion. An endoscopic biopsy of the lesion exhibited clear cytoplasm with prominent nucleoli, which was histologically compatible with metastasis to the stomach of the patient’s known RCC. On the other hand, computed tomography (CT) incidentally detected a well contrast-enhancing round-shaped mass in the fundus of the gallbladder (Fig. ). Additional ultrasonography revealed a sessile polypoid lesion, and gallbladder stone and wall thickening were not observed. Although these findings were lacking conclusive evidence of diagnosis whether the gallbladder tumor was primary or metastatic, the circumstantial evidence potentially pointed to the tumor as a metastasis from the patient’s known RCC. 18F-Fluoro-deoxyglucose positron emission tomography combined with CT (FDG-PET/CT) was performed as a preoperative workup to detect other possible remote metastasis. However, specific FDG uptake was not shown, even in the gastric and gallbladder tumors. The blood examination was unremarkable.\nIn February 2018, a gastric wedge resection via laparoscopic and endoscopic cooperative surgery (LECS) technique was applied to the gastric tumor, and laparoscopic cholecystectomy to the gallbladder tumor was simultaneously performed (Fig. ). The operation lasted 190 min with little intraoperative blood loss. Intraoperative pathologic diagnosis was not performed in this case. The hospitalization period after surgery was not eventful, and the patient was discharged on postoperative day 7. Histological examination confirmed that the tumors of the stomach and gallbladder were both metastatic RCC. Immunohistochemical staining was strongly positive for CAM 5.2 and vimentin, supporting the diagnosis. Macro- and microscopic findings are shown in Fig. . Thereafter, the patient required examination every 3 months without the use of anticancer agents and has survived without relapse to 12 months after the surgery. |
The first case of intracranial AVM along with a spinal AVM involves a 12-year-old girl who presented to us with a 3-month history of headache and progressively worsening spastic paraparesis with reflex spasms of both lower limbs. There was no history of lower back pain and no bladder or bowel disturbances. Examination showed power of 3/5 in the right lower limb and 4/5 in the left lower limb, with normal bulk but increased muscle tone. She also had brisk reflexes in both lower limbs. Cognitive functions, speech, and upper limb reflexes were normal, along with intact sensations and cerebellar functions. She had been in a good state of health in the past and her family history was negative for any hereditary vascular disorders or AVMs. Magnetic Resonance Imaging (MRI) of dorsolumbar spine showed epidural flow voids. Spinal angiogram showed an AVM in mid-dorsal region at level T8–T9 with three feeders comprising left 8th and right 9th and 10th intercostal arteries []. Brain imaging studies were done to investigate any intracranial pathology responsible for her persistent headache. Her brain MRI and Magnetic Resonance Angiography (MRA) revealed a small AVM in the left hippocampus supplied by left posterior cerebral artery and with deep venous drainage [Figure and ]. There was no hemorrhage from the lesions, and ischemia due to spinal AVM seemed to be the probable cause of her paraparesis.\nSpinal AVM embolization was carried out by polyvinyl acetate (PVA) and histoacryl particles under general anesthesia and complete embolization was achieved []. No treatment was offered for her cerebral AVM and her headache was managed conservatively with analgesics. The hospital course was smooth and she was discharged after a total of 5 days of hospital stay.\nOn follow-up after 1 year, the patient was doing well with no significant symptoms. She was able to work independently and did not require further treatment. |
A 62-year-old man complained of spontaneous onset left shoulder pain and stiffness for around 2 weeks. X-ray showed an expansile and lytic lesion over the proximal humerus []. The cortical bone was so thin that it could not be well delineated. Magnetic resonance imaging (MRI) showed a T2 hyperintense expansile mass. The humeral head was deformed, but the lesion remained intraosseous. There was no extraosseous soft tissue component. Open biopsy of the lesion confirmed GCT [].\nTreatment options of extended curettage versus wide local excision were discussed with the patient. He opted for joint preservation procedures. The patient also requested to try denosumab as a neoadjuvant treatment after we presented to him a few recent reports which showed that denosumab could induce ossification in GCT. Informed consent was obtained, and the patient received 6 doses of denosumab (120 mg subcutaneous injection on day 0, day 6, day 13, day 29, day 57, and day 85, respectively). The dosage and interval were similar to the protocol used by Thomas et al.[] Clinically, the left shoulder pain decreased although the motion was still limited. He was put on calcium and multivitamin supplement, and regular blood tests showed normal renal function and calcium level. The patient did not experience any side effect. Regular X-ray showed no progression of the lesion. In addition, the lesion became more sclerotic. There was progressive bone deposition in the tumor, and the cortex became more clearly delineated [Figure –]. A reassessment MRI showed no interval change in size or signal of the lesion.\nThe patient received operation on day 118 (around 4 months) after starting denosumab. An extended curettage was performed through a deltopectoral approach. We opened a cortical window at the metaphyseal area. The tumor tissue inside was found to be extremely hard and was significantly different from the soft texture of the usual GCT. Normally for GCT, we could easily scoop out the tumor tissue by curette. But it was not possible in this case. Rongeur and scalpel were needed to remove most of the tumor tissue until the subchondral and metaphyseal bone was reached. But the difference in texture between the tumor tissue and normal bone was not distinct. High-speed burr was used to lightly debride the surrounding bony cortex. Hydrogen peroxide (H2O2) and water (H2O) were used for irrigation. Cement and three intra-medullary titanium elastic rods were used to fill up the cavity and also as prophylaxis of fracture. Postoperative X-ray showed no residual lytic lesion. Histological examination of the tumor tissue revealed mainly fibro-osseous tissue. There were interconnected broad trabeculae of bone embedding a small number of stromal cells []. No giant cells were present in the sample.\nThe patient received two more doses of denosumab (120 mg) after the operation at 1 and 2 months respectively. Upon follow-up at 2 years postoperation, the patient reported complete resolution of bone pain. The shoulder could flex up to 70° and abduct up to 90°. The patient was satisfied with the functional outcome. There was no recurrence on X-ray [] and MRI. |
A 58-year-old woman with a history of Chiari-type 1 malformation initially presented in 1993 with complaints of headache, dizziness, and blurry vision in her right eye (OD). On initial neuro-ophthalmological examination, she was found to have a best-corrected visual acuity of 20/15 in both eyes (OU). Despite preserved central acuity, she was found to have a dense relative afferent pupillary defect (RAPD), decreased color vision, and significant inferior arcuate scotoma in the OD. No abnormalities were noted in her left eye (OS). MRI and high-resolution CT imaging demonstrated enhancement of the right optic nerve within the orbit consistent with an ONSM. Given the preserved central visual acuity, the patient elected for close observation. On subsequent visits, the patient was found to have a progressive decline in visual acuity (20/50 OD) with significant visual field loss resulting in vision in the superior nasal quadrant only. Secondary to progressive vision loss, the patient elected to proceed with radiotherapy (RT1). She was treated with a single lateral conformal field to the right retro-orbital region to a dose of 4005 cGy given in 15 fractions (267 cGy per fraction) over three weeks. The treatment fields included a portion of the posterior retina to encompass the optic nerve enlargement extending up to the globe. A 5◦ gantry angle was used to avoid irradiating the contralateral optic nerve. The patient tolerated the treatment well. Following completion of RT1, the patient reported progressive improvement in vision and was found to have 20/15 vision OD with a full visual field and resolved RAPD. Surveillance MRI following radiotherapy showed a decline in the size of ONSM.\nNineteen years after the first treatment, the patient reported increasing visual shadows and a subjective decrease in vision in the OD. An MRI revealed a new enhancement around the right optic nerve consistent with type 1 ONSM recurrence contained within the orbit. With preserved central acuity, the patient elected again for close observation. The patient was closely followed for seven years following the initial recurrence of the tumor over which time, the peripheral field slowly but progressively declined to near-complete restriction and her RAPD returned. Visual acuity declined to 20/50 (OD). MRI at that time revealed diffuse T2 hyperintensity and contrast enhancement throughout the intraorbital segment of the right optic nerve-sheath complex (Figure ). This was felt to represent an infield recurrence, although the initial treatment fields were not available for review. The patient's case was discussed in a multidisciplinary tumor board, and it was decided to proceed with re-irradiation prior to considering more invasive procedures.\nBecause of the excellent response to the initial course of therapy, it was decided to proceed with a similar dose fractionation regimen for the second course of radiation treatment (RT2). Treatment planning CT scan was performed using a helical scanner with 0.125mm slice thickness and fused to the diagnostic MRI sequences. The gross tumor volume (GTV) was defined as the area of abnormality seen on the diagnostic MRI and encompassed all of the intra-orbital and most of the intra-canalicular portion of the optic nerve. An additional clinical target volume (CTV) margin for sub-clinical disease spread was not added. A planning target volume (PTV) expansion of 3-5mm was used to account for uncertainties in patient setup. Optimization structures for the PTV and the optic chiasm were created to limit the radiation dose to the pre-chiasmatic nerve fibers, so as to not affect the vision in the contralateral eye. Other organs at risk (OARs) included the bilateral orbits and the contralateral optic nerve. A treatment plan utilizing two partial arcs (from 210 degrees to 10 degrees in the clockwise and counterclockwise direction) and intensity-modulated radiotherapy treatments (IMRT) was used (Figure ). The patient was re-irradiated to a dose of 4000 cGy given in 16 fractions. Doses received by the PTV and OARs are shown in Table . A thermoplastic mask was utilized to assist with patient positioning and the patient was instructed to look straight ahead prior to the treatment planning scan as well as prior to each treatment. Similarly, orthogonal pair kV imaging was used prior to each treatment to confirm the patient setup.\nFollowing RT2, the patient again noted progressive improvement in visual function and reported that she was no longer colliding with walls. Her visual acuity remarkably improved to 20/15 OU with an expansion in the superonasal and temporal visual fields to 71% of normal function (Figure ). Following RT2, the patient experienced dry eye, transient dyschromatopsia, ocular neuromyotonia, and discomfort behind the eye. At the most recent follow-up, one year after the second radiotherapy course, the patient demonstrated lasting treatment effect with 71% of her normal visual field function and no evidence of radiation optic neuropathy/retinopathy. Retro-orbital discomfort was successfully treated with carbamazepine, which has similarly reduced the frequency of diplopia from ocular neuromyotonia. Her dry eye was well managed with ophthalmic drops. |
An 8-year-old boy came to surgical emergency with complaints of generalised abdominal pain, abdominal distension and obstipation for last 1 day. Pain suddenly started in the right upper abdomen, which later on became diffuse involving whole of the abdomen. There was no history suggestive of any other pre-existing illnesses or haemolytic disease. Physical examination revealed diffusely tender and distended abdomen. Guarding and rebound tenderness were present in all the quadrant of the abdomen. Laboratory investigations were essentially within normal limit except raised total leukocyte count. Chest and abdominal X-rays were also unremarkable. Based on the history and clinical profile of the patient, diagnosis of duodenal perforation peritonitis was rendered, and following this, urgent exploratory laparotomy was scheduled. Abdominal exploration was done through midline incision, and around 700 ml of biliopurulent fluid was aspirated. Intraoperatively, 1 cm × 1 cm perforation at the neck of the gallbladder was noticed, and this region was surrounded by the pus flakes []. The gallbladder wall appeared thickened and it had no stones. Cholecystectomy was done after ligating the cystic duct and the artery []. Rest of the abdominal viscera was found normal. The abdomen was closed after adequate lavage and placement of the right subhepatic drain. Resected specimen was sent for histopathological examination.\nHistopathological examination showed focally denuded lining epithelium with marked edema and congestion just below the epithelium. Muscle fibres appeared to be separated due to inflammatory infiltrate, oedema and congestion. There was dense acute on chronic inflammatory infiltrate extending from mucosa to serosa suggestive of acute on chronic cholecystitis []. In view of incidentally detected GBP, we further investigated this patient for associated enteric fever, dengue fever and haemolytic disease, and none of these illnesses could be established in this patient. Post-operative period was uneventful and he was discharged on the 4th day. During a follow-up of 2 months, he remains asymptomatic. |
A 59-year-old Hispanic male with a past medical history of diabetes, hyperlipidemia, chronic obstructive pulmonary disease (COPD), and remote smoking history (quit 29 years ago) was admitted to the hospital by his pulmonologist for recurrent bilateral pneumonia over the past 10 months. The patient's initial symptom was a cough associated with lying supine, eating, or drinking. He also reported that his oxygen saturation would decrease to the 80s while sleeping and unintentional weight loss of 40 pounds over the past 10 months. On further questioning, the patient reported having progressive dysphagia from solids to liquid over the past 10 months.\nHe reported being admitted to a different hospital approximately three-weeks prior, where he was diagnosed with persistent pneumonia and treated with antibiotics and steroids. The patient's hospital course was complicated secondary to pleural effusion, which required a thoracentesis. Pleural fluid analysis was negative for malignancy. CT scan of the chest during his previous hospital stay revealed extensive bilateral infiltrates and a 3 cm density in the left upper lobe. He was discharged and to follow up as outpatient with his pulmonologist.\nOn this admission, his initial vital signs were temperature 98.2 F, pulse 84 beats per minute, respiratory rate 15 breaths per minute, blood pressure 111/69 mmHg, and oxygen saturation 93% on room air. Physical examination was significant for rhonchi and lymphadenopathy. Chest X-ray and CT of the chest showed pneumonic infiltrates and pleural effusion (Figure and Figure , respectively), which required drainage by thoracentesis.\nAs the patient had dysphagia and weight loss, a barium swallow was done, which was remarkable for slow emptying and severe backflow in the esophagus followed by coughing associated with dysphagia. The patient had undergone an esophagogastroduodenoscopy (EGD) due to findings on barium study. EGD revealed a gastric fundi mass approaching the gastroesophageal junction. Biopsies taken from the gastric-mass were positive for gastric adenocarcinoma. A repeat thoracentesis was performed. Pleural fluid cytology was positive for gastric adenocarcinoma.\nA follow-up CT of the abdomen and pelvis and bone scan revealed metastatic disease (Figure and Figure , respectively). Furthermore, the patient required bilateral indwelling pleural catheters secondary to having persistent pleural effusions and a percutaneous endoscopic gastrostomy tube for nutritional support. Oncology had determined that the patient was not a candidate for chemotherapy. Ultimately, the patient was transferred to an inpatient hospice unit as he had a short life expectancy due to having severe metastatic gastric adenocarcinoma. |
The patient is a 33-year-old healthy right-hand-dominant male who worked as an electrician and presented to our clinic after sustaining a closed left clavicle fracture in a motocross (motorcycle) race accident. The acute fracture was at the lateral third of the clavicle shaft and there was an established fracture nonunion at the medial shaft (type 1B1) [] (). At the time of this recent injury he was wearing a Leatt brace, which is designed to decelerate the head and protect the cervical spine during a crash (). There was no neck or chest wall injury, loss of consciousness, or other significant injuries. He did not seek medical attention until appearing in our clinic one week later.\nThe patient reported having a history of “several left clavicle fractures”, the first occurring in high school in a motocross accident. He received little medical attention and only one radiograph was taken. Three years later in another motocross accident he stated that he “fractured the same clavicle in the same place as before”. In view of the persistent pain with crepitus it is likely that the fracture never healed. With respect to the biomechanics of the chronic medial fracture, a direct blow from striking the handle bars of the motorcycle initially caused this fracture. By contrast the lateral fracture was from indirect trauma, with the fracture occurring at the outer edge of the Leatt brace.\nNo surgeries had been performed on the clavicle. However, over the two years prior to being seen in our clinic the medial clavicle nonunion had become increasingly painful with overhead activities, which reflected the greater demands of his work as an electrician. In the setting of the acute lateral-shaft fracture, the patient requested surgery to address both the nonunion and the acute fracture.\nBoth fractures were treated with open reduction with internal fixation (ORIF) using a 12-hole nonlocking reconstruction plate and thirteen 3.5 mm screws. In accordance with the preoperative plan, the medial scapula nonunion was treated with realignment, autogenous bone graft, and ORIF; the latter included spanning the plate onto the sternum in order to provide rigid fixation for the medial reconstruction (). This was done because it was deemed necessary to place four screws, purchasing eight cortices, medial to the medial fracture (two in the medial clavicle and two in the sternum). The rationale for doing this was (1) the reported difficulty in obtaining enough screw purchase into the small medial clavicle fragment [, –] and (2) our initial impression that our patient might be noncompliant with lifting and shoulder motion restrictions, which could lead to nonunion if rigid fixation was not employed. Also, the only other reports that we could locate at that time that described sternoclavicular plating (for fracture or sternoclavicular dislocation) used a hook plate [, ], which is designed for a lateral clavicle fracture []. We were concerned a hook plate would not provide sufficient rigidity for our patient.\nThe surgery was done using a beach chair position and an X-ray C-arm intensifier to ensure accurate screw placement. Although the surgery seemed uneventful, the patient had shortness of breath in the recovery room and a pneumothorax was diagnosed with an anterior-posterior (AP) radiograph (). The pneumothorax was not detected intraoperatively with the C-arm X-ray intensifier because only the upper lung could be seen clearly, the remainder being obscured by the operation table. A chest tube was placed in the recovery room. The chest tube was removed without complications two days later.\nThree months later, the medial one-third of the plate was cut and it was removed along with the four medial screws as planned (). Preoperative radiographs showed that the medial fracture was healing well. The remainder of the plate and the remaining screws had not been removed at final follow-up 3.5 years later. The patient reported complete satisfaction with his final result, which included no pain or dysfunction and had a final DASH (disability of arm, shoulder, and hand) score of 8 (0 = best, 100 = worst) []. He had also returned to full work capacity by five months after the index surgery. |
A 42-year-old Caucasian man had a 21-year history of a traumatic spinal cord injury and complete T6 paraplegia due to a motorcycle accident in 1989, which was initially treated non-operatively. He subsequently developed a syrinx from C3 to T5 associated with post-traumatic deformity and underwent posterior instrumentation with hooks, rods and posterolateral fusion from T3 to L3 in 1994 (Figure ). In 2005 he sustained bilateral distal femoral shaft fractures after falling from his wheelchair and underwent bilateral retrograde femoral intramedullary nail fixation which achieved successful union of the fractures. He presented with a 6-month history of lethargy, increasing lower torso and lower limb spasms associated with severe sweating, hypertension and recurrent headaches. He noticed a change in his posture, with kyphotic deformity in his lumbar spine and was no longer able to sit upright in his wheelchair. He also developed symptoms of urinary retention whereas previously he could initiate urinary voiding with bladder tap.\nPlain X-rays showed severe bony reabsorption and destruction distal to the previous fusion coupled with new bone formation around the destroyed L4 vertebra (Figure ). A computed tomography (CT) scan showed extensive endplate and osseous destruction of his L3 and L4 vertebral bodies, with gas vacuum phenomenon at this level. A magnetic resonance imaging scan confirmed the bony and soft tissue destruction at L3 and L4, as well as showing abnormal paravertebral soft tissue and a complex paraspinal 7cm diameter mass within his psoas muscle with associated multifocal and multilocular fluid collections, areas of dystrophic ossification and nonspecific soft tissue inflammatory changes.\nA staged combined surgical approach was undertaken in order to obtain circumferential arthrodesis. The first surgery involved a posterior approach and extension of the previous fusion from L3 to S1 with pedicle screws and rods (EXPEDIUM® Spine System, DePuy Spine, Raynham, MA, USA; Figure ). These were connected to the previous spinal instrumentation using rod connectors and cross-links. The second surgery was performed after 1 week and involved a direct lateral trans-psoas retroperitoneal approach to L4, where extensive destruction with bony debris was observed. L4 corpectomy and anterior reconstruction using an expandable titanium cage stabilized with vertebral body staples and screws in L3 and L5 held with a 6.25mm diameter rod (LEGACY™ Spinal System, Medtronic Sofamor Danek, Memphis, TN, USA) was performed (Figure ). Histopathology of the corpectomy specimen showed necrotic tissue associated with nonspecific chronic inflammation, fibrosis and granulation tissue with no evidence of infection or malignancy (Figure ). Culture results were negative for bacteria, acid-fast bacilli, Brucella, and fungi.\nThe patient was mobilized postoperatively but after 10 days unfortunately experienced recurrence of the clicking when sitting forward, associated with recurrence of intermittent sweats. Repeat X-rays showed disengagement and subluxation of the anterior cage, suggesting persisting instability of the Charcot spine at the operated level (Figure ). He thus subsequently underwent a third surgery which was performed posteriorly, revealing severe loosening of the L5 and S1 screws bilaterally and involving revision and extension of the posterior instrumentation to his ilium with screws and cobalt-chrome rods (Expedium). Morcellized femoral head allograft and recombinant human bone morphogenetic protein-2 (INFUSE®, Medtronic Sofamor Danek, Memphis, TN, USA) were added to the decorticated bone. He was then immobilized in bed for 6 weeks. After this time, a repeat CT scan of his spine showed posterolateral spinal fusion consolidation of the bone graft and fusion mass from T4 to his ilium (Figure ), he was allowed to mobilize and did not have recurrence of his symptoms.\nAt 10 weeks after his final spinal surgery and 4 weeks after recommencing mobilization, he heard a sudden crack in his right hip region when bending forward in his wheelchair. This was associated with deformity and swelling around the hip region and an immediately floppy leg. X-rays showed a comminuted intertrochanteric right femoral neck fracture, between the previous spinopelvic fixation and the retrograde femoral nail (Figure ). The spinopelvic instrumentation was unchanged. He underwent further surgery involving removal of the previous retrograde femoral nail, reduction of the intertrochanteric femoral neck fracture and fixation with an antegrade femoral intramedullary device (Long Gamma® Locking Nail, Stryker Orthopaedics, Mahwah, NJ, USA). He made an uneventful recovery and was able to mobilize as tolerated. Of interest, radiographs taken at 6 weeks postoperatively showed a stress response of the bone on the contralateral (left) side, with periosteal bone formation in the subtrochanteric region (Figure ). |
A 63-year-old cachectic female with a medical history of multiple myeloma and advanced amyloidosis was admitted to our institution with complaints of left lateral thigh pain and nausea. At the time of presentation, her myeloma and associated vertebral fractures were under active treatment with cyclophosphamide and bortezomib. Ibuprofen had been prescribed 10 days prior to admission for musculoskeletal pain. The patient’s nausea was associated with increasing abdominal distention over approximately 3 months. Computerized axial tomography performed on admission (Fig. ) demonstrated a dilated stomach containing fecalized material. The patient reported passing gas and stool. She was placed on a general diet and was managed without nasogastric decompression as per her medical team. She continued to receive ibuprofen and was treated with pulse dose steroids, cyclophosphamide and bortezomib until hospital day 5 when the patient complained of sudden, severe abdominal pain.\nAn abdominal plain film revealed substantial pneumoperitoneum and general surgery was consulted with concern for perforated viscus. Examination at that time revealed a tympanic, diffusely tender abdomen without overt signs of peritonitis. The patient’s amyloidosis had resulted in severe macroglossia with multiple episodes of epistaxis and glossal hemorrhage. Nasogastric decompression was deliberately deferred given her friable macroglossia and expected endotracheal intubation. She was taken to the operating theater for exploratory laparotomy which preceded in standard fashion after rapid sequence induction.\nElectrocautery was used to dissect through the subcutaneous tissues. When a deliberate incision was made into the peritoneum a loud clap was heard which was followed by a dull roar and 3–4 s of blue flame erupting from the peritoneal cavity. In this interval, the attending physician attempted to extinguish the fire with a moist towel causing a superficial burn to one of his fingers. When the flame had self-extinguished, the patient’s wound was inspected and remarkably found to be free of injury or burn. The abdomen was inspected thoroughly and was also without any evidence of burn injury. An ulcer found on the greater curvature was biopsied and Graham patched. The pylorus was hypertrophic, but widely patent with good movement of gastric contents into the duodenum.\nPost-operatively the patient did well without apparent complication. An upper gastrointestinal series was performed on post-operative day 5 (Fig. ) which was negative for leak but notable for very limited transit of contrast into the duodenum 2 h after administration. Her pathology returned negative for H. Pylori, negative for malignancy, but positive for abundant amyloid deposits. Ultimately, she was discharged home to hospice care given her overall decline. |
A 67-year-old male patient was admitted to the gastroenterology department due to abdominal pain over 3 months. He was hospitalized with a diagnosis of gastricism and a proton-pump inhibitor was started, but abdominal pain persisted. Physical examination showed mild epigastric tenderness. A complete blood count on admission were as follows: white blood count 9.76 × 109/L, the C-reactive protein level 138.31 mg/L, red blood count 3.79 × 1012/L, hemoglobin 120 g/L, platelets 118 × 109/L, liver function tests, kidney function tests and pancreatic enzyme levels were within normal limits. Abdominal computed tomography (CT) was scheduled revealing a linear, hyperdense, foreign body along the stomach wall and pancreatic neck (Fig. a), and bone condition CT clearly shows the position and shape of the fish bone in the abdominal cavity (Fig. b). The patient was questioned about her past medical history. He remembered that he had abdominal pain after eating fish and something else 3 months ago. After urgent consultation, we made a final diagnosis of localized inflammation caused by a fish bone penetrating the posterior wall of the gastric antrum and migrating into the neck of the pancreas. Upper gastrointestinal endoscopy was performed; however, in addition to the chronic atrophic gastritis and distal gastric ulcer, no foreign body was found. Later, the patient was transferred to department of hepatobiliary and pancreatic oncology and underwent laparoscopic surgery. The patient was placed in a supine position. The operator stood on the right side of the patient, the assistant on the left side, and the scopist between the patient’s legs. Five trocars were placed: one above the navel for the laparoscopy (10 mm), two in the upper right abdominal quadrant (12 mm, 5 mm), and one in the upper left abdominal quadrant (10 mm, 5 mm). Fibrous structures were observed between the small curvature of the stomach and pancreas neck, and after the adhesions were dissected, a fish bone was identified and removed laparoscopically (Fig. a). The foreign body was identified as a 3.2-cm-long fish bone (Fig. b). Bleeding was controlled by pressure with a hemostatic gauze, and no suture repair was performed, because the penetrated wall was small and no leak was observed in both stomach and pancreas. Surgical intervention was completed after placing a drain in the operation area. The operation time is 2 h, and the bleeding during the operation is about 100 ml. Postoperative antibiotherapy was started, with proton-pump inhibitor treatment continuing for three more days. Clear fluid was drained, finally the drain pipe was removed on the third day after surgery. The patient was discharged from the hospital on the fifth day after surgery without any postoperative complications. And CT reexamination had not found obvious abnormality 2 months after the surgery. |
A 26-day-old-male baby, born at 35 weeks gestational age with 2 kg birth weight was referred for ROP screening. During the early neonatal period, there was a history of multiple systemic problems like shock, seizures, anemia, and respiratory distress syndrome requiring ventilator support for four days. The child was diagnosed to have stage 3 zone 2 posterior ROP with flat neovascularization, multiple vascular loops with intraretinal shunts in zone 2 posterior avascular retina, and plus disease in both eyes. In view of anticipated rapid progression, laser photocoagulation by Indirect ophthalmoscope laser delivery (LIO) was planned. Detailed history regarding COVID symptoms and travel from containment zone, elicited from parents, was negative. COVID-19 status of the parents and child was unknown. After obtaining informed consent from parents, the baby was taken up for LIO under topical anesthesia.\nLaser treatment in a crying baby can be a significant aerosol-generating procedure.[] To avoid this, an ACB was used. ACB has six sides, named A-F, described in detail later. The baby was dressed up in OR (operating room) dress and covered with a small blanket, adequate to prevent hypothermia. Trained OR assistants handled the baby under the supervision of the anesthetist and the surgeon. The baby was placed inside the ACB and removed via the open side B after lifting the plastic sheet. For easy access by the surgeon, the baby was placed over two pillows covered with blankets inside the ACB. ECG chest leads, and pulse oximeter probe were connected and, heart rate and oxygen saturation were monitored by an anesthetist throughout the laser procedure. The suction machine attached to a suction tube passed through Side A aperture was switched on. The baby was held by an assistant through side B, while the surgeon did laser by passing the hands through the holes in sides C, D, and E []. The laser was performed using double frequency ND: YAG (532 nm)––Oculight TX (IRIDEX, Mountain view, California, USA), with the power of 120 mW, exposure, and Interval of 100 mS each (1800 burns/eye), under topical anesthesia (0.5% Proparacaine). The laser burns of optimal intensity were obtained in the appropriate areas []. There was one episode of bradycardia (heart rate lower than 100/minute), the procedure was paused, treated by applying gentle physical stimulus over the baby's sole, and then laser resumed. The child was observed two hours after the laser and was doing well. On follow up, the child was seen to have good regression of the disease, so the treatment was considered adequate and advised to review after a week. |
A 56-year-old male patient presented to our outpatient department with chief complaints of pain and restriction of movement of right shoulder for the last six weeks. He had a history of multiple shoulder dislocation. The first dislocation occurred following an event of trauma 10 years back which was reduced in a nearby hospital. He had suffered from three more dislocations since then, each reduced in a hospital. The last episode of dislocation occurred one month back, following a fall onto the right shoulder. This time the patient went to a bonesetter, who attempted to reduce the shoulder but failed. The patient neglected the dislocation and has not sought any further treatment before reporting to us.\nOn physical examination, there was a loss of deltoid contour. There was no sensory deficit. Duga’s test was positive. The range of motion of the right shoulder was grossly restricted and painful. His abduction was possible up to 30 degrees, and rotational movements were limited. The constant score in the preoperative period was 37. X-ray imaging showed anterior glenohumeral dislocation, while the MRI was suggestive of a Bankart lesion. CT scan showed a glenoid bone loss of 7% with a non-engaging HSL involving 21% of humeral head (Figure ). We planned for open reduction and Bankart repair, as per the treatment protocol described earlier.\nThe patient was positioned supine. Under general anesthesia, after proper draping and painting of the limb, a deltopectoral approach was used to open the joint. The clavipectoral fascia was incised, and the long head of the biceps tendon was traced proximally to identify the rotator interval. We performed lesser tubercle osteotomy and reflected the subscapularis medially to expose the joint capsule. The anteroinferior labrum was found to be torn. After capsulotomy, the joint was reduced, and labral repair was done using two double-loaded metallic suture anchors.\nWe started pendulum exercise on the first postoperative day and advised the patient to use an arm sling for four weeks. The patient was allowed to do a progressive range of motion exercise with gradual increments. However, we restricted external rotation in abducted position and internal rotation up to six weeks to protect the Bankart repair and the lesser trochanter osteotomy. The patient had achieved a forward flexion of 110 degrees and external rotation of 30-degrees in a neutral position at four weeks, after which we started light theraband exercise. Following six weeks, we initiated the restricted rotational movements and allowed forward flexion up to 130°. At three months, we allowed abduction and external rotation as tolerated without pain and started the theraband exercises against resistance. Finally, we permitted overhead head activities after four months of the surgery. At the end of the one-year, he has maintained the range of forward flexion of 130°. However, he had an abduction of 120° and external rotation of 30°. His constant score was 78 at the final follow-up. |
A 22-year-old female professional water-polo player presented to our Hand Department complaining of persistent pain on the dorsoulnar aspect of the right wrist (dominant hand) during the last 4 months, aggravated by sport activity, and not responding to medical treatment or rest. The patient did not report any acute trauma at that region and noted that her symptoms worsened while shooting. At that time a painful snapping sensation over the dorsoulnar aspect of the wrist followed the forceful forearm rotation. Physical examination revealed swelling, edema and tenderness over that area. A “clunk” and palpable dislocation of the ECU tendon along the ulna head with active or passive supination and ulnar deviation of the wrist was observed. Hand and finger function was normal, and neurovascular examination showed no abnormality.\nThe patient underwent radiological investigation with plain radiographs of the wrist, that were negative for any bone pathology ( and ), and dynamic Ultrasound (U/S) at rest (with the wrist in 0° of flexion-extension) and during pronation and supination with comparison to the contralateral ECU tendon. During supination, the unstable ECU tendon slided abruptly over the ulnar wall of the distal ulnar groove and dislocated volarly, while during pronation the tendon relocated into the groove ( and ).\nDiagnosis of recurrent ECU tendon dislocation was made on the basis of clinical history, physical examination and imaging findings. As the patient’s symptoms had not improved after a 4-month period of conservative management and in conjunction with a highly demanding sport, surgical intervention was opted.\nSurgical reconstruction of ECU tendon sheath was carried out under axillary block anesthesia using a high humerus tourniquet. A straight dorsomedial incision was made along the distal ulna starting 5 cm proximal to the wrist and ending 1 cm proximal to the joint (). The extensor retinaculum, found to be intact, was opened longitudinally over the ulnar aspect. Dislocation of the ECU tendon in ulnar-palmar direction was confirmed with the forearm in supination and the wrist in palmar flexion. The fibro-osseous sheath was found to be torn along its entire length from the ulnar retaining wall (). The distal ulnar groove was deep enough to retain the tendon in its normal position and it was not reconstructed. The ECU tendon was elevated, reduced into the groove and 4 mini bone suture anchors were placed along the groove’s ulnar margin (). The sutures were then passed through the ulnar border of the ECU sheath in horizontal mattress fashion and were tied, securing tendon sheath to bone. A piece of extensor retinaculum was used as a graft, to strengthen the reconstructed sheath (). Splinting of the humerus-forearm with the elbow flexed to 90° and the wrist in approximately 30° of extension, radial deviation, and pronation was applied for 6 weeks. A progressive rehabilitation program was prescribed after cast removal, including active and passive wrist flexion, extension, forearm supination and pronation, and progressive strengthening with gradual return to sport activities. The patient was allowed to fully participate in her team’s schedule 4 months postoperatively. Follow-up evaluation 12 months postoperatively revealed no recurrent dislocation of the ECU tendon and normal painless range of motion of the affected wrist.\nAll procedures concerning the management of the patient were in accordance with the Helsinki Declaration of 1975, as revised in 1983. |
A 67-year-old female presenting with dizziness was diagnosed with high-degree atrioventricular block (Figure 2A) and referred for pacemaker implantation in August 2020. Her medical history showed hypertension and previous stent implantation for the treatment of left anterior descending artery stenosis with long-term clopidogrel medication. The intracardiac electrogram showed infra-Hisian block, and the His bundle capture threshold was 5 V/0.5 ms. Then, we performed left bundle branch pacing. The lead was advanced into the LBB area located below the septal tricuspid valve. The procedural steps for delivering LBBP and detailed criteria to confirm LBB capture have been described previously, the key point of which is to screw deep enough into the septum (, , ). At the site A, we failed to capture the LBB () even when we tried to screw deeper where we recorded a smaller LBB potential. Then the lead was moved inferiorly and posteriorly to the site B, LBBP was finally successfully achieved with a larger LBB potential and pacing threshold of 0.5 V/0.5 ms (). One hour after the operation, the patient complained of chest pain, and her blood pressure decreased from 131/72 to 96/57 mmHg. The ST-segment depression was recorded during intrinsic rhythm by programming the device to a lower pacing rate () and pacing parameters remained stable. The bedside echocardiogram revealed an interventricular septal hematoma measuring 8 mm in width without pericardial effusion (). Immediate coronary angiography demonstrated contrast agent overflow and retention in the interventricular septum (IVS) (). The symptoms were relieved quickly, and her blood pressure increased with a normalized ST segment half an hour later (). An echocardiogram was taken again 2 h later, and hematoma progression was not observed. Six hours after implantation, the troponin I level increased from 0.004 to 32.589 μg/L. The next day, computed tomography angiography (CTA) showed a hematoma in the basal segment of the IVS (). After 1 month, echocardiography, CTA confirmed the hematoma had resolved (, ). No contrast agent overflow was found in the interventricular septum () at 1-month follow-up. At 1, 3, and 6 months after implantation, the pacing parameters and LVEF remained stable. |
A 28-year-old male presented to the emergency department with complaints of severe right testicular pain and swelling for the past 11 h. He stated the pain began acutely around 3 AM that morning with associated nausea. The previous day, he noted mild soreness in the right testicle which he attributed to having hit more than 100 golf balls at the driving range. He denied trauma to the area and reported being in a monogamous sexual relationship. His past medical history was significant only for mild intermittent asthma, and he noted allergies to cefaclor and certain types of alcohol. He denied any fevers, chills, or vomiting.\nThe patient was afebrile with a heart rate of 114 beats/min and blood pressure of 134/91 mmHg. Physical examination revealed a swollen, tender, and somewhat high-riding right testicle with a hydrocele. Laboratory testing was significant only for a white blood cell count of 13.6 × 109 cells/L. A urological surgery consultation was obtained for concerns of testicular torsion. A testicular ultrasound revealed normal testicular blood flow, normal bilateral epididymi, a large simple right hydrocele, a small left varicocele, and a possible right spermatic cord hematoma. Due to concerns that the patient's original complaints were secondary to testicular torsion that had spontaneously resolved, he was taken to the operating room for scrotal exploration with possible orchiopexy and orchiectomy.\nGeneral anesthesia was induced with propofol, fentanyl, midazolam, lidocaine, and succinylcholine, and endotracheal intubation was successful on the first attempt. Following induction and initiation of positive pressure ventilation, the patient's blood pressure progressively decreased from a preinduction mean arterial pressure (MAP) of 100 mmHg to a nadir of 40mmHg over a period of 30 min despite administration of 600 mL of normal saline, 400 mcg of phenylephrine in divided doses, and 500 mL of albumin. The patient's MAP increased to 60 mmHg only after temporary discontinuation of volatile anesthetic, serial administration of 10–50 mcg boluses of epinephrine and 2-8 unit boluses of vasopressin, and continued albumin administration. Hydrocortisone was administered in addition to epinephrine out of concern for a possible anaphylactic reaction.\nDuring this period of hemodynamic instability, scrotal exploration revealed a normal right testicle with normal blood flow. A right-sided noncommunicating hydrocele with cord edema and varicosities was noted. As there was no significant pathology, the scrotal incision was closed, and there was minimal blood loss associated with the procedure.\nAs the surgical drapes were removed, it was noted that the patient was plethoric and that ST-elevation was developing in electrocardiogram leads II and V5. A TEE probe was placed for further evaluation of the patient's hypotension. TEE examination immediately revealed a 22 mm pericardial effusion with diastolic compression of the right atrium and right ventricle []. There was a mild, global decrease in systolic function with an estimated left ventricular ejection fraction (LVEF) of 45%, dilated inferior vena cava and hepatic veins, and spontaneous echo contrast in the IVC []. A diagnosis of pericardial tamponade was made, and the on-call cardiac surgeon was requested for the emergent consultation. A right internal jugular central venous catheter was placed and revealed a central venous pressure of 28 mmHg. An arterial blood gas sent at this time showed a lactate of 4.6 mmol/L. Upon arrival of the surgeon, a subxiphoid pericardial window was performed with drainage of approximately 500 mL of serous fluid and immediate stabilization of the patient's hemodynamics without the continued need for vasopressors or inotropes.\nOn postoperative day 1, the patient was successfully extubated. He remembered a globus sensation that he had experienced the day prior to the presentation, but no other symptoms that may have raised suspicion for an intrathoracic abnormality. A follow-up transthoracic echocardiogram demonstrated normalized biventricular function with an LVEF of 60–65% and no residual pericardial effusion. Bacterial cultures of pericardial fluid and pericardial tissue were negative as well as those for acid-fast bacilli, fungus, and anaerobic bacteria. Viral myopericarditis was made as the diagnosis of exclusion. On postoperative day 3, the patient was discharged with orders to follow-up with his primary care physician, a urologist, and a cardiologist in the next 2–3 weeks. |
A 38-year-old Chinese man visited the Department of Periodontics (West China Hospital of Stomatology, Sichuan University, Sichuan, China) with gingival enlargement, loose teeth, and tooth loss. The chief complaint of the patient was generalized gingival enlargement beginning 2 years ago, sometimes with pus. The teeth gradually loosened several months ago, and the left maxillary first molar was lost following severe pain. A local doctor diagnosed him as periodontitis, and then he was referred to the Department of Periodontics for periodontal therapy.\nMedical history: The patient was a smoker for 20 years, averaging 20+ cigarettes per day. There was no relevant family history and no special disease history, no history of allergies, and the patient was not on any drugs. The extraoral examination showed facial symmetry and no obvious abnormalities in the lymph nodes of the head and neck. Intraoral examination showed that the patient has extremely poor oral hygiene, with a mass of dental plaque and debris covering almost all the teeth. The teeth had subgingival and supragingival calculus from the incisors to the molars. Gingivae were generally enlarged and red, and the buccal gingivae of the left mandible and maxilla were even enlarged toward the occlusal surface. The involved gingivae were edematous and tender, the gingival margin was inflamed, and there was no ulceration or significant necrosis. General findings were bleeding on probing, deep periodontal pockets, and attachment loss. Tooth mobility varied from Io-IIIo, and was IIIo in the left maxillary first premolar and second premolar (Fig. A). Mucosal texture and color were basically regular. There were no specific findings in the salivary glands, and salivary gland ducts could open normally. Panoramic radiography showed generalized alveolar bone loss (ABL), especially alveolar bone resorption at the left maxillary first and second premolars, as well as the left and right posterior teeth from alveolar crest to the apical area, but the maxillary sinus was intact without damage (Fig. B). No significant abnormalities were found in further clinical examinations, bone marrow examination, and tests for infectious diseases such as acquired immunodeficiency syndrome (AIDS), syphilis, hepatitis B and C, and mycobacterium tuberculosis. Serological examination showed that C-reactive protein was high at 14 mg/L (normal range: 0–10 mg/L), the serum IgG was 23.70 g/L (normal range: 8.00–15.50 g/L), and IgG4 concentration was 2.800 g/L (normal range: 0.035–1.500 g/L). Then biopsy of gingivae was carried out under anesthesia from the left maxillary first and second molar.\nHistological analysis of hematoxylin and eosin staining (H&E) demonstrated ulceration, a large infiltration of lymphoplasmacytic cells, a storiform pattern of fibrosis, and mitotic figures (Fig. C-a, b); the preliminary diagnosis was plasma cell granuloma. Immunohistochemical (IHC) staining results showed that scattered infiltration of 10 IgG4-positive plasma cells in the high-power field (HPF) (Fig. C-c, d). Other IHC markers, CD38, Ki-67/mum, kappa, lambda, EBER, and Pan Cytokeratin (PCK) were positive (Additional file : Figure S1, data not shown). Position emission tomography-computed tomography (PET-CT) also excluded the possibility of cancer, and there were no obvious changes in other organs. According to clinical manifestations and serological and histological features, the lesion was regarded as an inflammatory condition. After 2 months, the patient was diagnosed as IgG4-related periodontitis by the Department of Rheumatology and Immunology (West China Hospital of Sichuan University) on the basis of the diagnostic criteria for IgG4-RD, although there were only 10 IgG4-positive plasma cells in the HPF. Using a new classification of periodontitis from a 2017 workshop [–], he was then diagnosed as periodontitis (Stage III, generalized, Grade C) and classified as manifestation of systemic diseases by Department of Periodontics.\nThe treatment plan for this patient was oral corticosteroids and periodontal therapy guided by the Department of Rheumatology and Immunology (West China Hospital of Sichuan University) and the Department of Periodontics. The rheumatologist recommended oral corticosteroids therapy. The initial dose of prednisolone was 0.6 mg/kg for 2 weeks (40 mg/day), which was then tapered by 5 mg every week for 6 weeks to determine a maintenance dose (5 mg/day). At the same time, the periodontist carried out periodontal therapy, including oral hygiene instruction, supragingival cleaning, subgingival scaling, and root planning (Fig. A). Three months later after non-surgical therapy, the periodontist re-evaluated the periodontal condition of the patient, then suggested he should have supportive periodontal therapy (SPT).\nAfter 6 months of multidisciplinary comprehensive treatment, an approach using corticosteroids with periodontal non-surgical therapy, gingival edema disappeared, the color of the gingivae turned pink, gingivae recessed, periodontal pockets became shallow, and gingival enlargement significantly decreased (Fig. B). In serological testing, IgG4 concentration was 0.584 g/L, which was in the normal range. After this, the rheumatologist recommended a supportive dose of 5 mg/day and revisit every 3 months, while the periodontist suggested that patient should have SPT every 3 months and quit smoking (timeline in Additional file : Figure S2, data not shown). |
A 35-year-old woman in the sixth month of pregnancy, presented to the emergency department post fall down straddle injury onto a sharp object (rock), during her work as a farmer. Her injuries involved the genitalia. There was profuse vaginal bleeding with an inability to pass urine. Examination revealed severe laceration of the anterior vaginal wall with complete urethral avulsion (). Abdominal ultrasound findings showed that the urinary bladder was full and tender, with no intra-abdominal collection. The fetus was alive in the 26th week, with a cephalic presentation. Full informed consent was obtained for the possibility of preterm termination of pregnancy, urinary diversion, urethral stricture or fistula, incontinence, vaginal infection, or stenosis. Emergency surgery under general anesthesia, in the dorsal lithotomy position with hemostasis was carried out. A small catheter was passed from the anteriorly displaced meatus for 2 cm in the intact distal urethra, to the level of the avulsed urethra. Thereafter, the catheter was manipulated back into the bladder, until urine passed through the catheter from the urinary bladder. Primary urethral repair was performed trans-vaginally with end-to-end urethral anastomosis over stenting silicone catheter by interrupting 5/0 vicryl. The muscularis over the urethra was closed in a second layer, and the vaginal mucosa was then closed as a third layer by 2/0 vicryl; with an attempt being made to offset the suture line from the muscular closure (Figures ). The catheter was removed 4 weeks postoperatively, and the repair was intact. She was fully continent and voided satisfactorily with a good stream. At full term, a lower cesarean section was carried out and yielded a well baby of 3 kg. She was followed up every 2 months for 2 years to monitor any lower urinary tract symptoms and sexual dysfunction. Two years post surgery, she is continent with a good stream of urine with a normal sexual life (). |
A 50-year-old Caucasian female, with no previous smoking history or pancreatic cancer family history, presented to our clinic for surgical evaluation of a biopsy-proven PDA noted at the junction of the body and tail of the pancreas. The patient had previously undergone a classic pancreaticoduodenectomy (Whipple) procedure with concurrent hemicolectomy 6 years prior (in 2011) for a T3N0M0 adenocarcinoma of the pancreatic head that had invaded the mesentery of the proximal transverse colon. After the 2011 surgery, pathology revealed a poorly differentiated PDA along with a PanIN grade 2. The final pathology showed negative surgical margins, positive perineural spread, and 0/33 specimen lymph node involvement. From June 2012 to October 2012, the patient underwent and completed adjuvant chemotherapy with gemcitabine, capecitabine, and radiation at an outside hospital. She was carefully followed by her medical oncology team with serial CA 19-9 monitoring and abdominal MRIs on an ongoing basis. Due to her young age, in 2011, she underwent genetic screening and no germline mutations were identified. Since the time of the primary resection, the patient had been high functioning and healthy, with the exception of some problems of early satiety and recurrent cholangitis. These sequelae were attributed to close proximity of the gastrojejunostomy and hepaticojejunostomy, with possible reflux of intestinal contents up the afferent limb, all partially managed by diet changes.\nIn 2017, a biannual screening MRI with intravenous contrast showed a new pancreatic lesion measuring 2.3 × 2.2 cm in the tail of the pancreas (). Esophagogastroduodenoscopy and endoscopic ultrasound-guided biopsy identified it as a poorly differentiated adenocarcinoma. From 2011 to 2017, she had had close followup with serial CA 19-9, and a measurement of this marker after identification of the lesion on MRI showed an elevation, which was confirmed on repeat testing (61 and 55 U/mL; normal <37 U/mL). This was the first instance of two consecutive CA 19-9 measurements outside of the normal range since resection of the primary cancer 6 years prior. When the patient presented to our institution a few months later, the CA 19-9 had returned to normal at 32 U/mL (), and there was a moderate increase in CEA (16.9 ng/mL; normal <3 ng/mL). The patient received a second MRI of the abdomen and pelvis with contrast to identify distant disease, which showed the lesion to be confined to the pancreas. Along with the MRI of the abdomen, a CT of the chest was preformed, which showed no gross metastatic lesions. A completion pancreatectomy was scheduled with revision and lengthening of the jejunal limb proximal to the gastrojejunostomy to resect the tumor and treat her episodes of early satiety and recurrent cholangitis.\nIntraoperatively, the patient's three Whipple anastomoses were noted to be grossly intact and there was no evidence of metastatic disease. First, the stomach was divided approximately two centimeters proximal to the prior gastrojejunostomy. The jejunostomy was closed, and gastrocolic and gastrosplenic ligaments were divided. The splenic artery was ligated, and the splenocolic ligament was divided. The spleen and pancreas were mobilized out of the retroperitoneum. The jejunum was divided between the pancreaticojejunostomy (PJ) and hepaticojejunostomy. The proximal jejunum, prior PJ, remaining pancreas, and spleen were removed. The tumor was noted to be grossly confined to the pancreas. The distal end of the stomach was delivered through the mesocolon and a retrocolic gastrojejunostomy was undertaken 60 cm downstream from the hepaticojejunostomy. The anatomy before and after this operation is shown in .\nThe patient tolerated the procedure well and had an uncomplicated hospital course. The patient was closely followed postoperatively and did well. She completed two cycles of adjuvant chemotherapy with gemcitabine and capecitabine from January 2018 to April 2018, but did have some neutropenia at the end of her adjuvant therapy requiring pegfilgrastim. Her intermittent fevers, early satiety, and abdominal pain resolved after the surgery. She became an obligate insulin-dependent diabetic after the completion pancreatectomy procedure and now requires exogenous pancreatic enzymes to support her nutrient absorption.\nThe specimen was found to be consistent with a poorly differentiated invasive adenocarcinoma. Resection margins were negative, and 2 of 17 lymph nodes were positive for metastatic cancer. We sent representative slides of the patient's 2017 tumor and 2011 tumor to Perthera (McLean, VA) for next-generation sequencing (NGS) and histological analysis, which tested for mutations in a total of 315 genes and stained for various predictive biomarkers (). Both lesions showed the same mutations in KRAS (G12R), CDKN2A (splice site 151-1 G to A), and TP53 (Y220C). In addition, her 2011 tumor had a mutation in ACVR1B (S4) that was not present in the 2017 tumor, and the 2017 tumor had amplifications of MYC and mutant KRAS that were not present in the 2011 tumor (). Upon histological analysis, staining for MLH1, MSH2, MSH6, PMS2, pAKT, and HER2 was similar in both samples, but in the 2017 tumor, there was 60% increased staining for RRM1 and 20% increased staining for ERCC1, which changed the classification from low to high staining for ERCC1. |
A lump found in the left kidney during physical examination of a 51-year-old woman was diagnosed as kidney cancer, and the woman underwent a radical nephrectomy of the left kidney via laparoscope surgery on 29 November 2016. She had no history of kidney disease nor any family history of cancer. Pathology revealed a nuclear grade 2 clear cell carcinoma (TNM-staging: pT2 pN0 G2 M0) with favorable prognostic features according to MSKCC criteria, and thus the patient underwent a regular comprehensive review after surgery. Right middle femur metastasis occurred in January 2018, and positron emission tomography-CT (PET-CT) showed multiple lung metastases on 5 February 2018. At this time, she experienced low fever, poor appetite, and fatigue. Physical examination of lungs showed clear breath sounds, no weakening and no rales. She began therapy with sunitinib, and zoledronic acid, 4 mg every 4 weeks. During sunitinib treatment, a grade 2 hand-foot skin reaction occurred. After a 2-month treatment, a CT showed that the lung metastasis had increased in size, which was evaluated as progressive disease according to RECIST 1.1.\nAfter providing informed consent, the patient received 2 mg/kg nivolumab combined with approximately 5 × 109 CIK cells every 3 weeks starting in April 2018. After two cycles of therapy, the patient experienced increased appetite, but a CT scan performed 2 months after initiation of this therapy revealed enlarged and new pulmonary metastases. We continued to apply nivolumab and CIK cells combination therapy. After 6 months of therapy, a CT showed that the lesions in the lungs had begun to decrease (). Subsequent CT scans showed progressive disease reduction until November 2019.\nIn December 2018, the patient experienced intermittent headaches. No fever, nausea or vomiting was present, and no change in blood pressure, appetite and physical status occurred. Enhanced head magnetic resonance imaging (MRI) showed two nodules on the right parietal lobe that were considered metastases. After multidisciplinary consultation, local radiotherapy for head lesions was recommended, but the patient refused. Oral painkillers were used to control headaches and treatment with nivolumab and CIK cells continued. After an additional 5 months (May 2019), symptoms disappeared, and another enhanced MRI found the brain lesions had disappeared ().\nDuring treatment with nivolumab and CIK cells, no adverse events occurred except for a fever of 38.8 °C at the beginning of therapy. This was managed with physical cooling treatment; no corticosteroids were given.\nAt a follow-up in November 2019, CT scans showed right hilar lymph nodes that were significantly enlarged and low fever, fatigue, and poor appetite were present. Treatment with axitinib was started, achieving partial remission (PR). As of the last follow-up on 6 May 2020, the disease continues to stabilize. The timeline of the patient’s disease development, treatment and outcome is shown in . |
A 49-year-old man was referred to the Oral Medicine Department of the Mashhad Faculty with an intra-oral mass in the left alveolar area. The patient had extracted his first mandibular molar tooth two months ago due to mobility. Afterwards, he noticed a mass in this area. Two other teeth were also extracted during this time as a result of progressive mobility. At the same time, the size of the lesion was increasing.\nIntra-oral examination revealed expansion in the mandibular bone and a painless exophytic lesion with a verrucous and granular surface on the left side of the mandibular ridge. The anterior part of this lesion was purple ().\nThe inferior parts were bony hard in consistency; but the alveolar area was firm. The right lateral incisor and left canine tooth both had luxation grade 3. The clinical diagnosis was oral SCC or verrucous carcinoma. Panoramic radiography showed a well-defined radiolucency with sclerotic borders between the left first molar and the right first premolar tooth ().The Border was not intact in some areas ().\nIncisional biopsy was performed under local anesthesia. Histopathological examination of the peripheral lesion revealed malignant neoplastic proliferation of squamous epithelium with up-growth and down-growth that created a cauliflower appearance. The elongated rete ridges appear to push into the underlying connective tissue. The connective tissue in this region was fibrous and inflamed. The histopathological appearance indicates verrucous carcinoma ().\nIn the histopathological evaluation of the central lesion, neoplastic proliferation of the odontogenic epithelium was observed to contain islands of columnar cells with polarized nuclei, which were similar to ameloblasts in the periphery and stellate reticulum-like cells in the center with acanthomatous changes and cystic degeneration. Fibrotic stroma was observed between the neoplastic parts. The histopathological appearance indicates plexiform ameloblastoma ().\nThe patient was referred to the Otolaryngology Department for curative surgery. A midline labiotomy incision with an extension to the left side of the neck was done. The mass was totally exposed by a releasing incision in the gingivo-buccal sulcus. A rim of the inferior border of the left mandible and chin was preserved and the cyst was completely removed with the tumor above it. The final diagnosis was verrucous carcinoma in addition to mural ameloblastoma. Reconstructive surgery was performed with an iliac crest bone graft to repair defects of the jaws. After two years, the recurrence of the lesion was not observed. |
The 70-year-old male patient was admitted to the Department of Neurology with isolated left upper extremity weakness and clumsiness. He had wrist drop and was unable to grip with the fingers. He complained of no sensory loss or numbness. His past medical history included long-term oral anticoagulation due to recurrent lower extremity deep vein thrombosis, glaucoma, and a non-significant aortic valve stenosis, with vascular risk factors including a 5-year history of treated hypertension, hypercholesterolemia, and a history of non-significant stenosis of the left anterior descendent coronary artery. On admission, no abnormality could be detected by physical examination, apart from the neurological signs, which included a wrist drop on the left side with decreased grip strength (Fig. ). Pronation and wrist dorsiflection were lost, as well as the ability to form a ring with the thumb and the index. The reflexes of the left upper extremity were slightly brisk, with not pathological reflexes present and no sensory deficits. Laboratory parameters were without abnormal findings except for a slightly elevated fasting blood glucose level. Cranial CT revealed contrast-enhancing, irregularly shaped lesions with diameters of 7, 10, and 9 mm (in the temporal, parietal, and frontal lobes, respectively), surrounded by perifocal edema. Carotid duplex ultrasonography did not demonstrate signs of focal atherosclerotic plaques, circulatory disturbance or stenosis on either side. In accordance with the findings of the CT scan, the cranial MRI confirmed the tumor in the right precentral gyrus, corresponding to the ‘hand knob’, accompanied by further similar lesions posteriorly in the parietal and the temporal lobes, and in the left frontal lobe (Fig. ). Electromyography and nerve conduction studies did not support a peripheral origin underlying the paresis. The neuropsychological examination revealed a deficit in the Luria three-step test as a single alteration, which performance could, however, be remarkably improved by verbal clues. Chest X-ray performed as part of the search for primary tumor revealed no abnormality. Non-contrast and contrast-enhanced chest CT detected an irregularly shaped mass in segment 10 of the left lung, with inhomogeneous enhancement of the contrast agent and multiple associated lymph node conglomerates, suggesting lung cancer as the primary tumor. Abdominal ultrasonography did not detect malignancy. The patient received palliative steroid therapy, with no improvement in the paresis during the observation period. Based on the recommendation of the tumor board, the patient was transferred to the Department of Pulmonology for bronchoscopic tissue sampling. The verification of the diagnosis by this means, however, was unsuccessful. The diagnostic process had to be suspended due to an acute bleeding duodenal ulcer, and the patient received blood transfusion and local hemostatic treatment in the Department of Surgery. In association with the antibiotic treatment, the patient developed pseudomembranous colitis caused by Clostridium difficile. In addition to the persisting colitis, fever occurred accompanied by hemoptysis and coughing. The subsequent chest X-ray did not confirm pneumonia. Despite the applied antibiotic and oxygen therapy, respiratory failure developed, and the patient passed away 2 months after the onset of the neurological symptom. The autopsy and the histopathological analysis identified an adenocarcinoma (mucinous tubular adenocarcinoma with solid anaplastic parts) both in the brain and the lung; (Fig. ). |
Our patient, a 36 year-old white man, presented to the emergency department following 12 h of right flank pain radiating to the groin, associated with intractable nausea, that had not responded to hydration and ibuprofen. While generally in good health, except for a history of recurrent nephrolithiasis, he was approximately 3 weeks post-operative from a deviated nasal septum repair. He reported being a current smoker with no personal or family history of pulmonary problems, including: asthma, COPD, cystic fibrosis or alpha-1 anti-trypsinase deficiency. His nasal septal surgery and post-operative course had been uneventful, allowing him to return to his construction job 2 weeks after his surgery. He described no pulmonary complaints, however, on review of systems, he described an audible “rhythmic clicking” sound when lying on his left side. His wife confirmed that the sound first appeared soon after his surgery. Initially, she could hear it up to three feet away, most pronounced when lying in bed. Gradually the sound became quieter and infrequent during the subsequent week.\nPhysical examination revealed head, neck, chest, cardiac and specifically respiratory exam within normal limits. Attempts to reproduce the sound by palpation or positioning in the left lateral decubitus position were unsuccessful. The abdominal exam identified only right flank and costovertebral tenderness.\nAn abdominal and pelvic CT revealed a 5.3 mm left distal ureteral stone with mild hydronephrosis. Proximal abdominal CT scan slices appeared suspicious for a small pneumothorax later confirmed by chest CT which delineated a tiny left anterior basilar pneumothorax, free of mass, nodule, or infiltrate. CT showed no evidence of hilar adenopathy nor bullae (Fig. ).\nOur patient was admitted to the hospital, placed on high flow O2 therapy as well as aggressive fluid hydration and tamsulosin 0.4 mg daily. He was discharged on hospital day 2 after a CXR confirmed resolution of the pneumothorax with recommendations to follow up with his primary care physician and urologist. |
Our patient is a 19-year-old G4P0212 who presented at 33 weeks and 5 days of estimated gestational age with preterm contractions every 5–10 minutes, vaginal spotting, and pink vaginal discharge. Her pregnancy had been complicated by antiphospholipid syndrome and cervical incompetence. A McDonald cerclage was placed at 13 weeks of gestation. She denied loss of fluids upon admission and a sterile vaginal exam showed 1 cm of cervical dilation with an intact cerclage. Her past obstetrical history was noted for two prior preterm deliveries secondary to cervical incompetence. Pertinent past medical history includes asthma, depression, two prior cervical cerclages, two cesarean deliveries (one of which was a classical incision), and an appendectomy. With a concern of threatened preterm labor, the patient was admitted and started on intravenous magnesium sulfate and intramuscular antenatal corticosteroids for fetal lung maturity. She was also given vancomycin for group B-streptococcal prophylaxis as she was allergic to penicillin. Uterine contractility increased in frequency thereafter and the patient then developed leakage of fluids and was ruled in for preterm premature rupture of membranes. Expectant management with latency antibiotics was followed. By the second day after admission the patient continued to leak clear vaginal fluid but progressed to have more frequent uterine contractions. A thick, foul-smelling yellow discharge emanated from the external cervical os and she developed uterine tenderness. At this point, the patient was stable and fetal heart tones were reassuring but the decision to remove the cerclage was made in light of impending chorioamnionitis with the newly diagnosed uterine tenderness. Accordingly, magnesium sulfate was discontinued, intravenous gentamicin was added, and the cerclage was removed. A sterile speculum exam revealed a visually 3 cm dilated cervix prompting delivery via repeat cesarean section. Precesarean white blood cell count was 25400 cells/mm3 with a 90% left-shift. She underwent an uneventful cesarean delivery of a female infant weighing 2092 grams with Apgar scores of 7 and 8 at one and five minutes, respectively. Umbilical cord arterial and venous blood pHs were 7.19 and 7.26. Arterial blood gases were as follows: Pco2 = 72 mmHg, Po2 = 45 mmHg, HCO3− = 23 mmol/L, and base excess = 1. The neonate was started on prophylactic broad-spectrum antibiotics and had an uneventful course. The neonate's complete blood count at the time of birth revealed the following parameters: white cell count of 15700 cells/mm3, hemoglobin of 20.3 g/dL, hematocrit of 61.5%, and platelet count of 308000/mm3, and the blood cultures were negative for any bacterial growth. Placental bacterial cultures from both fetal and maternal surfaces were collected. The maternal interface of the placenta cultured methicillin resistant staphylococcus aureus (MRSA) and on pathologic evaluation revealed moderate chorionitis ().\nOn the first postoperative day, the patient complained of uncontrollable moderate-to-severe abdominal and pelvic pain that worsened with deep inspiration. She progressed to develop marked tachycardia of 160 beats/min, tachypnea with 25 breaths/min, worsening chest pain, and shortness of breath. On physical exam her abdomen was distended and soft and foul-smelling vaginal discharge was noted. A computed tomography (CT) angiogram was performed and ruled out pulmonary embolus as a cause of her symptoms. Her symptoms worsened dramatically with further abdominal distension and an increase in her vaginal discharge. She developed a temperature of 102°F, chills, sustained tachycardia, tachypnea, and a white blood cell count of 2500 cells/mm3 with a 90% left-shift. Sepsis was a major concern at this point and blood cultures were obtained. Repeat CT imaging with intravenous contrast revealed an enlarged postpartum uterus, free air in the abdomen, copious fluid in the pelvis, and diffuse mesenteric edema indicative of peritoneal inflammation. She underwent an exploratory laparotomy. The patient's abdomen was noted to be full of purulent discharge and the uterus was necrotic with overlying fibrinous exudates. An estimated 700 mL of purulent discharge was evacuated and sent for cultures followed by a hysterectomy as the uterus was necrotic and was presumed to be the focus of infection. The procedure was uncomplicated. The entire small bowel was run and noted to be normal. The surgery was concluded with copious irrigation. She was then transferred to the intensive care unit and started on intravenous vancomycin and meropenem. Her pathology report noted diffuse polymorphonuclear infiltrates, necrotic myometrial tissue, and purulence on high power microscopy (). Both blood and purulent discharge cultures grew Fusobacterium necrophorum and the antibiotics were continued for an additional 48 hours. By hospital day 14, the patient was in a stable recovery but had complained of nausea and occasional vomiting and difficulty in breathing. She had subsequent bilateral pleural effusions, left lung pneumothorax, left lung consolidation (), acute urinary retention, renal failure, multiple abdominal and pelvic abscesses, and persistent nausea and vomiting. The patient had a nasogastric tube placed and underwent multiple drainage procedures for the pelvic and abdominal abscesses and had a chest tube placed for her left pulmonary pneumothorax and consolidation that subsequently tested positive for FN. At this point, vancomycin and meropenem were restarted. Repeat CT imaging of the chest, abdomen, and pelvis showed stable fluid collections thereafter and clinical improvement followed. The patient's last febrile episode was on hospital day 25. Renal function, nausea, and vomiting improved slowly and her bowel function returned to normal thereafter. The patient was eventually discharged from the hospital on day 34 from admission and was scheduled for a 14-day course of intravenous ertapenem. Her recovery was otherwise uneventful thereafter. The neonate remained in the intensive care unit for 19 days and was then discharged in a stable condition. |
A 74-year-old Asian woman complained of right knee pain without any cause. Because the pain persisted for several months, she went to a nearby hospital where she was referred to our institute for a second opinion and treatment. She had thyroid cancer and received tumor resection 5 years prior to the knee symptom without local recurrence. Radiographs demonstrated a 4×4.5 cm osteolytic lesion in her medial femoral condyle (Fig. ). On magnetic resonance imaging (MRI), the lesion was depicted as low intensity on T1-weighted image (T1WI), low-intermediate intensity on T2-weighted image (T2WI), and highly enhanced after gadolinium contrast administration. There was no penetration of the tumor through the cortex and no soft tissue mass was present (Fig. –). An open biopsy was performed and, histologically, the tumor showed clusters of epithelial cells. These epithelial cells were oval or spindle-shaped with squamous differentiation, and surrounded with fibrous stroma. Tumor cell nuclei were relatively uniform, did not show pronounced atypia, and had a low rate of mitosis. Reactive bone formation, similar to fibro-osseous lesion, was seen around the epithelial cells (Fig. ). On histological examination, adamantinoma was initially considered for the diagnosis. However, it was inconclusive due to its unusual localization. Considering the patient’s age, metastatic squamous cell carcinoma was possible although the primary lesion was not evident. Moreover, it was difficult to exclude metastasis of the thyroid cancer she had 5 years ago because papillary carcinoma of the thyroid gland sometimes undergoes squamous metaplasia thereby resembling squamous cell carcinoma. Because there was no other known lesion after further screening, we performed a wide resection of the tumor and implanted a tumor endoprosthesis to reconstruct the defect (Fig. –). The resected specimen showed the same histological feature as the biopsy sample; therefore, the final diagnosis remained inconclusive. She was discharged without any complication. Five years later during a routine follow-up examination, an abnormal shadow occupying the upper lobe of her right lung was detected (Fig. ). She subsequently underwent lobectomy for the lesion. A histopathological section showed proliferation of spindle-shaped and squamoid epithelial cells with mild nuclear atypia, surrounded by fibrous stroma, which was strikingly similar to the findings of the femoral lesion resected 5 years ago (Fig. ). Of interest, the epithelial cells spread through the alveolar wall without destruction of the alveolar structure. This feature is quite different from that of squamous cell carcinoma, either primary or metastatic, which usually shows destructive growth (Fig. , ). The bone and lung specimen were both negative for thyroid transcription factor-1 (TTF-1) and thyroglobulin immunostain, which precluded the diagnosis of thyroid papillary carcinoma. These findings led to a conclusion that the lung mass is a metastasis from the distal femoral lesion, and the tumor which occurred in the medial femoral condyle was finally diagnosed as adamantinoma.\nAt the latest follow-up, positron emission tomography-computed tomography (PET-CT) showed a recurrent tumor in the upper lobe of her right lung and further metastasis in her lumbar spine (Fig. ). |
Three years ago, A 56-year-old Asian man presented to our clinic with debilitating dysphagia involving solids for several months. The patient had a heavy smoking history (3 packs/day, 30 years), but no alcohol drinking history. He had uncontrolled diabetes mellitus over 10 years, and underwent coronary artery bypass graft surgery 8 years previously.\nThe patient had previously visited a local clinic with heartburn and dysphagia, where he underwent esophagogastroduodenoscopy (EGD) without a biopsy. EGD revealed esophageal stricture and mucosal abrasion. When initially referred to our clinic, provisional diagnosis included gastroesophageal reflux disease, thus, proton pump inhibitor therapy began at first visit. We planned further evaluations like computed tomography (CT), barium swallow, esophageal manometry, and endoscopic biopsy, but he constantly refused admission or further evaluations and was lost to follow-up for 18 months because of his poor economic status.\nAs time went by, his dysphagia became aggravated and was associated with weight loss. He revisited the clinic with a body weight loss of 7 kg (47 kg at visit; usual weight 54 kg) and dysphagia involving both solids and liquids; he could barely swallow a liquid diet and had difficulty even in swallowing pills.\nPhysical examination revealed no superficial lymph nodes. A blood test showed that complete blood cell counts, creatinine, and liver function tests were normal. EGD showed severe esophageal stricture at 32 cm from incisors and mucosal erosions with exudate (Fig. ). Biopsies were performed at erosions (only 2 pieces of tissue). Biopsy results showed necroinflammatory exudate. Barium swallow (Fig. ) showed stricture in the mid-to-distal esophagus and delayed contrast passage. CT scan (Fig. ) demonstrated an esophageal lesion, length about 70 mm, in the lower esophagus with marked luminal narrowing and wall thickening, without enlarged lymph nodes. An abdominopelvic CT scan of the pancreas was performed and showed normal.\nPrevious proton pump inhibitor therapy was not effective and the lesion was long. Findings of body weight loss of 7 kg and wall thickening of the esophagus might suggest malignancy, but no lymph node enlargement was revealed on CT scan. There was a previous CT scan a year previously, because of coronary artery bypass graft history. In the previous scan, there was no esophageal wall thickening or enlarged lymph nodes. To evaluate esophageal motility disorders, esophageal manometry was planned but the stricture was too narrow for a catheter to pass.\nAs the disease showed benign features (benign biopsy, no lymph node enlargement), we suggested esophageal dilatation rather than surgical resection. But the patient wanted immediate resolution of symptoms and refused the step-wise procedure. Thus, the surgical measure Ivor-Lewis esophagectomy was performed. His postoperative course was uneventful, and he was discharged on postoperative day 10. The oral intake of soft diet was tolerable at the time of discharge.\nUpon gross examination, the resected specimens showed marked wall thickening and stricture with mucosal erosion in the lower esophagus (Fig. ). Histologically (Fig. ), scattered lymphoid follicles and prominent muscular hypertrophy were observed in the submucosal layer. Dense lymphocyte and plasma cell infiltration was observed in the submucosal layer and the proper muscle layer. Immunohistochemistry (Fig. ) revealed IgG4+ plasma cells at a level of >50/HPF and a ratio of >90′ IgG4+/IgG+ plasma cells. Postoperative serum level of IgG was 1,450 mg/dL (normal range: 700–1,600) and IgG4 was 171.0 mg/dL. Based on the comprehensive diagnostic criteria for IgG4-RD [], He was diagnosed with an IgG4-RD, definitive, with esophageal involvement.\nFollowing achievement of remission by surgical method, the patient received maintenance prednisolone therapy (30 mg/day), which was slowly tapered. His dysphagia improved greatly and he returned to his usual weight (54 kg). After prednisolone therapy, follow-up serum IgG4 level dropped to 48.9 mg/dL and prednisolone therapy was tapered. Serum IgG4 level sustained a similar level (52.1 mg/dL after 6 months; 49.0 mg/dL after 11 months). |
In a work-up of a 40-year-old female with a history of low back pain, a routine MRI of the lumbar spine identified an incidental lesion. The axial T\n1 weighted image showed a 21-mm round, homogeneous lesion of low signal intensity interforaminally in the left S2 segment of the sacrum. The axial short tau inversion-recovery image showed a well-defined lesion with an inhomogeneous signal intensity throughout the lesion with high peripheral rim intensity. The axial T\n1 image with fat saturation after contrast media injection showed moderate enhancement throughout the lesion and in the peripheral rim (). A radiograph of the pelvis was performed to further characterize the lesion. On clinical examination, the sacral area was not painful to palpation. Owing to the atypical appearance of the lesion and the non-specific nature of the MRI signal pattern, a bone scan was performed that showed a solitary lesion on the left side of the sacrum with increased metabolic activity. For further characterization, and in the search of a potential primary tumour, positron emission tomography (PET)-CT was performed, which showed a well-defined sclerotic lesion with mild fludeoxyglucose (FDG) avidity (average standardized value 2.5) in the S2 segment and no other abnormalities (). Owing to the metabolic activity of the lesion, the patient was referred to the orthopedic oncology department for image-guided biopsy of the lesion. The biopsy specimen consisted of a few small fragments of bone marrow, some skeletal muscle, fibroadipose tissue and blood clots. Infiltration of the otherwise normal bone marrow with scattered small groups of big foamy cells was identified (). The foamy cells had vacuolated cytoplasm and small centrally located nuclei. The cells were negative for cytokeratin AE1/AE3, CD68, barchyury, Melan A, HMB 45, desmin and smooth muscle actin but positive for S100 protein (). The cells contained multiple lipid droplets and numerous large mitochondria; the existence of the latter was exhibited with antimitochondrial marker (). A pathological diagnosis of hibernoma was made in correlation with the imaging findings.\nOwing to the clinical assessment in correlation with the history of bilateral lumbago, worse after physical activity, the patient was diagnosed with chronic lower back pain. At 1-year follow-up, the patient was being managed with analgesics and physical therapy. |
An 11-year-old girl presented to the emergency department with concerns of severe abdominal pain and two episodes of fresh blood in stool in one day. The pain was in the umbilical region, episodic, sharp in nature, non-radiating, and aggravated after having meals. She also had nausea and gave a history of constipation for the past five days.\nShe had been experiencing similar episodes of moderate abdominal pain for the past two years. However, the episodes of pain had increased in severity and frequency over the past month, occurring almost every two to three days. She had been sent home twice from school in her last semester due to her symptoms and had a history of multiple hospital visits. Her sleep was often interrupted by the pain at night. She also had concerns of occasional bloating and diarrhea but had no history of weight loss or fever. She resided in a suburban town with four other family members in a well-ventilated house and belonged to the middle socioeconomic class. Groundwater was their main source of drinking water, and there was no significant travel history. None of her family members or school fellows were reported to have any similar symptoms. Moreover, there was no family history of celiac disease, inflammatory bowel disease, or acid peptic disease. She reached menarche at 11 years and had regular menstrual cycles. On examination, the abdomen was soft, non-distended, with deep tenderness in the umbilical region. Perianal examination showed an anal fissure in the six o’clock position.\nShe was initially suspected to have appendicitis or ovarian torsion and was admitted under the care of the pediatric surgery department. Laboratory findings showed: hemoglobin 9 g/dL, mean corpuscular volume (MCV) 81.1 fL, white blood cell count 6,250/mm3, and platelet count 158,000/mm3. Outside stool routine examination was normal. Pancreatic function tests were also in range, and urinary tract infection was ruled out. An abdominal ultrasound was done, which showed a bulky right ovary containing a cyst measuring 40 x 35 mm with echoes and septations. It was likely to be a hemorrhagic cyst. Further evaluation of the cyst - consisting of levels of alpha-fetoprotein, beta-human chorionic gonadotropin (b-HCG), lactate dehydrogenase (LDH), and CA-125 - was normal. A gynecology consult was also done, and she was discharged on a trial of oral contraceptives, antacids, and pain medication. For her anal fissure, she was given topical lidocaine gel for local application.\nThe patient followed up after one week with the persistence of abdominal pain. Results of repeat blood investigations were in the reference range, except for an erythrocyte sedimentation rate (ESR) of 54 mm/hour. To rule out inflammatory bowel disease, the parents were advised by a pediatric gastroenterologist to give their daughter’s stool sample for fecal calprotectin and to follow up with test results. Gynecology follow-up was also done, and since the trial of oral contraceptives had not been successful in relieving her pain, the case was referred to a pediatric surgeon who decided on an elective date for laparoscopic ovarian cystectomy.\nTwo days later, the patient presented again to the emergency department with severe central abdominal pain. On request of the parents, a laparoscopic right ovarian cystectomy and appendectomy was performed by the pediatric surgeon in order to achieve resolution of symptoms. The pediatric gastroenterologist was taken on board before discharge who advised an out-patient follow-up with stool for fecal calprotectin and magnetic resonance enterography (MRE) to rule out inflammatory bowel disease.\nThe patient followed up after a week, without resolution of abdominal pain even after undergoing surgery. The MRE report showed no evidence of bowel stricture, wall thickening, fold thickening, abnormal enhancement, or mass lesion. Repeat routine stool examination was normal. However, the level of fecal calprotectin was raised (251 µg/g). Accordingly, endoscopy and colonoscopy were advised. Duodenal biopsy revealed vegetative growth of G. lamblia with no evidence of celiac disease. (Figure and Figure ).\nColon biopsy was negative for inflammatory bowel disease. The patient was treated for giardiasis with oral tinidazole and metronidazole. Her symptoms resolved thereafter, and she has been asymptomatic ever since. |
In January 2021, a 2-year-old boy of white Caucasian origin presented to his local ophthalmologist for acute unilateral sixth nerve palsy and was subsequently transferred to our pediatric emergency department for further evaluation. The patient, generally being fit and well, had developed a sudden dysfunction in lateral movement of his left eye, resulting in a continuous abduction deficit with consecutive fixated turn of the head to the left side. His medical history was unremarkable for trauma, headache, vomiting or fever. He had not received any vaccinations within the last few weeks. Apart from a mild gait instability, there were no concomitant symptoms or other focal neurological deficits on clinical examination. The patient did not suffer from any chronic diseases and did not take any regular medication; his vaccination status was complete according to national recommendations.\nThree weeks prior to onset of symptoms the patient had experienced a respiratory tract infection resulting in an increased respiratory rate, dry cough, intermittent fever and loss of appetite, lasting for 2 weeks. Symptomatic treatment was initiated by his local pediatrician, who attributed the patient's symptoms to a common cold rather than COVID-19. Thus, no oropharyngeal swab for SARS-CoV-2 or other viruses was obtained. At the same time, the patient's father and his uncle developed cough, dyspnea, sore throat and muscle aches; the uncle tested positive for SARS-CoV-2 on PCR from oropharyngeal swab (). The child's uncle does not live in the same household but had been in close contact to the patient 4 days prior to his positive test for several hours due to an indoor-birthday party. The patient's relatives were unvaccinated as at that time the COVID-19 vaccines were still unavailable for the general public.\nOn admission, laboratory inflammatory markers including C-reactive protein were negative. Full blood count showed mild thrombocytopenia (186 109/) but was unremarkable otherwise. Cranial contrast-enhanced magnetic resonance imaging (MRI) showed an hypoplastic left abducens nerve and atrophy of the corresponding left lateral rectus muscle compared to the contralateral side (). There were no signs suggesting any inflammatory intracranial process or elevated intracranial pressure, no papilledema. A lumbar puncture was performed. The cerebrospinal fluid (CSF) opening pressure was 24 cmH2O corresponding to the upper limit of normal range () thus diagnostic lumbar puncture was followed by therapeutic drainage of 8 ml CSF. Routine CSF laboratory parameters yielded a normal result; no oligoclonal bands were detected on CSF/serum. Multiplex-PCR (Filmarray, BioFire, Biomerieux Lyon, France) from CSF was negative for cytomegalovirus (CMV), enterovirus, herpes simplex viruses 1 and 2, human herpesvirus 6, human parechovirus, varicella zoster virus, Cryptococcus neoformans and gattii, E. coli K1, Haemophilus influenzae, Listeria monocytogenes, Neisseria meningitides as well as Streptococcus agalactiae and pneumoniae. An additional multiplex-PCR performed on an oropharyngeal swab sample yielded a negative result for adenovirus, coronaviruses 229E, HKU1, NL63 and OC43, human metapneumovirus, human rhino-/ enterovirus, influenza virus A and B, Middle East Respiratory Syndrome Coronavirus (MERS-CoV), SARS-CoV-2, parainfluenza virus 1–4, respiratory syncytial virus, Bordetella pertussis, Bordetella parapertusssis, Chlamydophila pneumonia and Mycoplasma pneumoniae. Testing for Borrelia burgdorferi showed no antibodies in neither serum nor CSF. An EEG was unremarkable. Repeated ophthalmologic examinations revealed incomitant squint angles due to left-sided sixth nerve palsy and a significant abduction deficit of the left eye, consistent with the diagnosis of left abducens nerve palsy. An underlying retraction syndrome was considered unlikely due to the sudden onset of symptoms and absent globe retraction. Optic nerve examination was unremarkable.\nReal-time reverse transcriptase PCR (rRT-PCR) test for SARS-CoV-2 (oropharyngeal swab sample) was negative on admission, while serology turned out to be positive for SARS-CoV-2 anti-spike IgG (Euroimmune, Germany). Of particular note in this context, SARS-CoV-2 specific IgG was also detected in CSF. Pathogen-specific antibody index as an indicator for potential intrathecal antibody production was negative, suggesting involvement of central nervous system being secondary to systemic infection rather than direct viral infection (). An rRT-PCR for SARS-CoV-2 from CSF was negative.\nGiven the boy's history of recent respiratory tract infection, COVID-19 very likely in his father and proven in his uncle, and detection of SARS-CoV-2-IgG antibodies in the patient's serum and CSF, post-infectious abducens nerve palsy appeared to be the most likely diagnosis. During inpatient stay, symptoms already showed spontaneous mild improvement without therapeutic measures. Following discharge, the boy was regularly seen for ophthalmologic follow-ups. Three months following onset of abducens nerve palsy, the family noticed a distinct improvement in eye movement and the child eventually made a full recovery 2 weeks later. |
An 8-year-old male Ugandan child presented with a 1-year history of a progressive abdominal swelling and flank pains. His mother reported they have been in and out of hospitals with the child due to frequent complains of abdominal discomfort. He was treated with antipyretics and unspecified antibiotics for 6 months prior to their referral to our facility for further investigation and management. There was no history of constipation, hematuria, jaundice, urinary retention, or bleeding diathesis. He was the third born in a family of 5 siblings. There was no history of similar condition in the family. There was no family history of malignancy. His father is a businessman, and the mother is a housewife. His childhood was uneventful.\nOn clinical examination the child was stable and active (Eastern Cooperative Oncology Group Functional Status 3). His vitals were within the normal limits. Conjunctivae was pink and Chest was essentially normal on physical examination. The abdomen was distended and had mild generalized tenderness. There was a palpable mass, approximately 7 finger breaths in the right upper quadrant, which was firm, non-tender, and not ballotable and did not cross the midline. The mass was dull to percussion and active bowel sounds appreciated. There were no enlarged peripheral lymph nodes.\nComplete blood count showed mild anemia with a hemoglobin level of 9.5 g/dl, while the other indices were normal. Renal function test and liver function tests were all normal.\nUltrasound scan of the abdomen showed a heterogeneous huge mass in the right lobe of the liver, involving segment V, VI, VII and VIII measuring about 11.8 cm by 9.71 cm. The liver was enlarged in size measuring 17.88 cm at mid clavicular line. There was a moderate flow in color Doppler in the mass. Besides there were multiple hypoechoic solid lesions on both kidneys of varying sizes, the largest on the left measured 3.02 cm by 2.55 cm. The inferior venacava showed normal caliber and flow. The origins of the right and left renal veins were visualized but the mid and distal portions were compressed by the masses. There were no enlarged lymph nodes noted in the abdomen (Fig. A–C).\nContrast enhanced chest-abdomen-pelvis computed tomography (CT) scan showed huge ill-defined, homogeneously enhancing (43–70 HU) hypo-dense solid mass in V, VI, VII and VIII segments of the liver, measuring 16.39 cm by 11.21 cm by 10.2 cm. There was an ill-defined enhancing (30–63 HU) hypo dense solid mass in the right kidney measured 4.89 cm by 4.96 cm by 3.9 cm. There was an ill-defined enhancing (35–62 HU) hypo dense solid mass in the left kidney measured 4.88 cm by 5.92 cm by 4.5 cm with areas of central necrosis. The right renal vein was infiltrated. The right renal artery however was normal. The left renal artery and vein were normal. The ureters and urinary bladder were normal. The portal vein, intrahepatic ducts and common bile ducts were normal. The abdominal aorta and inferior vena cava were normal. There were no enlarged abdominal lymph nodes. There was no ascites. The visualized bones were normal with no focal bone lesion. The radiologic chest anatomy was normal (Fig. A–D). Histological examination of renal biopsy specimen was favorable for chemotherapeutic regimens.\nIn consultation with pediatric oncology tumor board, nephron-sparing surgery was deemed in appropriate given the poor long-term survival even in patients without distant metastasis. Our patient was commenced on combination chemotherapy (adriamycin and vincristine) with a palliative intent. The dose of vincristine was 1.5 mg/m2 body surface area administered as intravenous bolus injection and that of Adriamycin was 50 mg/m2 BSA infused over 4–6 h were given at 21-day interval. After three cycles of chemotherapy, the abdominal distention had reduced, and the child gained some weight. He remained well until first 6-months from the initiation of his chemotherapy until we unfortunately lost him to follow up. |
A 73-year-old female was referred to our Trust by her general practitioner with a 5-month history of a painless vaginal mass, which extruded from the introitus on straining, but was otherwise asymptomatic. This was originally thought to be a vaginal prolapse; however, examination revealed a soft, well-defined pink mass occupying the upper vagina and an MRI of the pelvis was requested for further characterization.\nMRI was performed using a 3.0 T system utilizing axial T1 weighted fast spin echo; small field of view axial, coronal and sagittal T2 weighted fast spin echo; and T1 weighted fat-saturated sagittal images before and after gadolinium contrast administration, obtained in the arterial and portal venous phases. Diffusion-weighted imaging was also acquired. The images demonstrated a 47 × 40 × 44 mm well-circumscribed, oval mass in the upper vagina. On the T1 weighted images, the signal intensity of the abnormality was intermediate, similar to that of the skeletal muscle (). However, on T2 imaging, there were discrete zones within the lesion; the anteroinferior aspect was of high T2 signal with no enhancement, whereas the posterosuperior aspect was of low T2 signal with avid enhancement (–). There was no restricted diffusion. The posterior wall of the retroverted uterus was demonstrated to abut the superior surface of the lesion and the vaginal lumen was deviated anteriorly. Normal vaginal wall was seen to extend around the lesion’s anterior and posteroinferior surfaces. The lesion appeared to be arising within the left posterolateral vaginal wall and there were areas of loss of definition of the outer margin of the vagina. There was no involvement of the rectum, urethra or bladder; however, there were hazy low T1 and T2 signal changes in the left paravaginal fat.\nThrough a MDT discussion, it was agreed that owing to the suspicious imaging features of enhancement and tissue inhomogeneity, a staging portal venous phase CT scan was required to look for evidence of metastatic spread. Again, the vaginal lesion demonstrated fluid and soft tissue attenuation areas with regions of enhancement (). Significantly, there was no evidence of distant spread or lymph node enlargement. After further MDT discussion, the mass was still thought to be suspicious for malignancy and the patient underwent surgery.\nThe uterus, ovaries, cervix and upper vagina were removed en bloc and macroscopic examination revealed a well-circumscribed 45 mm polypoid mass arising from the paracervical upper vaginal tissue. The cut surface of the lesion was fleshy grey and white in colour, and was mainly solid in nature.\nMicroscopic examination () revealed an unencapsulated lesion with a spindle cell morphology arranged occasionally in fascicles. Beneath the surface epithelium, there was a grenz zone. The spindle cells were set within finely collagenized stroma and were bland in nature, with no conspicuous mitoses identified. Areas of oedema and myxoid change were also present, with no evidence of haemorrhage or necrosis.\nImmunohistochemistry demonstrated that the lesional cells expressed desmin, vimentin, oestrogen and progesterone receptors. The Ki67 proliferation index was low. Immunohistochemistry for MNF116, alpha smooth muscle antigen, smooth muscle myosin, h-caldesmon, S100 and CD34 were negative in the lesional cells.\nThe morphological and immunohistochemical profile was considered consistent with a superficial cervicovaginal myofibroblastoma, which is also known as superficial myofibroblastoma of the lower female genital tract. |
A 23-year-old Chinese female (social gender) presented to our hospital to receive treatment for primary amenorrhea. Physical examination showed normal breast development, an immature vulva, development of the vulva in infancy, no pubic hair, a blind end in the deep part of the vagina, no penile or testicular tissue, and no hypospadias. With the consent of the patient, we performed computed tomography (CT); this revealed huge cystic lesions in the pelvic cavity in the right lower abdomen. Many nodules were present in the inner wall of the cyst and a cord shadow was evident on the left edge of the mass. We were unable to visualize the uterus or bilateral fallopian tubes. Ultrasonography revealed a huge mass in the left adnexal region; we were unable to detect a normal uterine sonogram in the anatomical area of the uterus. The area close to the right adnexal region presented with a 3.0 × 1.3 cm2 striped weak echo area by ultrasound; we considered this to be the ‘primordial uterus’.\nFollowing surgical resection, we examined the gross morphology of the specimens. The mass was cystic, and the structure of the mass was papillary. Microscopic observations revealed a multi-stage branching structure and a large number of irregular papillations (Fig. a). These papillations branched gradually from largest to smallest and were covered with a cubic columnar epithelium and a ciliated, eosinophilic, cytoplasm (Fig. b). Abnormal streak ovarian tissue was evident around the mass. A large number of fibrous tissues were also evident in the specimen, although there were no mature follicles. Immunohistochemical staining showed that the tumor cells expressed high levels of estrogen receptor (ER), progesterone receptor (PR), WT1, PAX8, CK7 and EMA; P53 was expressed but wild type and P16 was only expressed at low levels (Fig. ). The morphological characteristics evident on hematoxylin and eosin staining, along with the immunohistochemical results, were indicative of a serous borderline tumor of the ovary.\nThe tissue that we considered as the “primordial uterus” by ultrasound was located in an area close to the right adnexal region. Anatomical analysis revealed that this tissue was atrophic, cord-like, and smooth; it was also surrounded by a capsule and was devoid of ligaments and fallopian tubes. We were surprised by the lack of a uterine cavity, endometrium, or muscle wall, although the yellowish, spongy, soft tissue appeared to be denatured and atrophic tubular tissue. These tubules were composed of multiple layers of cells that exhibited cytoplasm that stained in either a rich or light manner with hematoxylin and eosin. We believe that these structures were Sertoli cells in seminiferous tubules (Fig. c). We also observed eosinophils around these seminiferous tubules; we believe that these structures were Leydig cells. Consequently, we believe that this structure was actually an immature testis (Fig. d). Based on microscopic observations of the tissue, we considered that this was not a primordial uterus; rather, this was a case of cryptorchidism. Further examination of the sex chromosomes revealed that the patient was 46, XY (Fig. ); her disorder was therefore diagnosed definitively as 46, XY DSD. The patient had testes, which were able to secrete Mullerian tube inhibitor, thus inhibiting the development of the uterus and fallopian tubes. The patient had also undergone normal breast development and had a female appearance, although her vagina had a blind end and the external genitalia appeared female without pubic hair. The development of secondary sexual characteristics was abnormal; there was no uterus, no fallopian tubes, and no hypospadias. Although sex hormone tests revealed high levels of testosterone, there was no obvious development of male external genitalia. Therefore, the patient was diagnosed with complete androgen insensitivity syndrome complicated by a serous borderline tumor of the ovary. After 6-months of follow-up, the patient was in good condition. |
A 60-year-old woman presented to a local hospital after she began to experience right upper quadrant pain, nausea, and vomiting. Abdominal ultrasonography showed marked dilatation of the intrahepatic bile duct (IBD) in the left lobe of the liver and common bile duct with intraductal stones.\nShe was diagnosed with cholangitis and then referred to our hospital in May 2011 for further investigation and treatment. She underwent laparoscopic left lobectomy for a left IBD stone and choledocholithotomy with t-tube drainage for a common bile duct stone. After one year, follow-up computed tomography (CT) showed no definite abnormalities, but laboratory data showed elevated serum levels of cancer antigen 19-9. Thus, CT scan was performed at three-month interval and a mass was observed at the atrophied right posterior segment of the liver. In addition, a palpable mass was found at the t-tube site and histologic diagnosis of the lesion by excisional biopsy was interpreted as adenocarcinoma (stage IV).\nShe was referred to our department for palliative chemo- therapy and received treatment with gemcitabine and cisplatin. She neither consumed any alcohol nor smoked and she reported no family history of illness. After eight cycles of gemcitabine and cisplatin (days 1 and 8: cisplatin 25 mg/m2 followed by gemcitabine 1,000 mg/m2; repeated every three weeks), the follow-up CT showed progressive disease, showing an interval increase in the size of the metastatic lesion in the abdomen, thus, the regimen was changed to cisplatin 60 mg/m2 on day 1 and 5-fluorouracil 1,000 mg/m2 on days 1-5.\nHowever, two months after the first infusions of cisplatin and 5-fluorouracil, the patient developed gait disturbances on day 6 of the third infusion. She could not maintain a standing position with her eyes open, and a tandem gait was impossible because of severe positional imbalance. Brain magnetic resonance imaging (MRI) with T2-weighted images (-) and diffusion-weighted images (-) showed high signal intensity in the extrapontine region, including the corpus callosum, midbrain, internal capsule and bilateral cerebellar peduncles. Laboratory results showed mild neutropenia, normocytic normochromic anemia (hemoglobin 10.8 g/dL), and normal C-reactive protein at 0.08 mg/dL. Her plasma sodium and potassium concentrations were 140 mmol/L and 3.5 mmol/L, respectively. Blood urea nitrogen and creatinine levels were normal. However, the potassium level had decreased to 2.6 mmol/L on day 8. She had been given potassium chloride 600 mg orally until day 3 (days 8-10 of infusion) when selfambulation was possible. Her potassium concentration was altered to 3.2 mmol/L and 3.0 mmol/L on days 9 and 10.\nShe discontinued cisplatin and only oral doxifluridine (5-fluorouracil) was administered as a salvage therapy. Two months after discharge, prominent clinical improvements were noted and the results of MRI with diffusion-weighted imaging correlated well with clinical outcome (). |
We presented a 43-year-old man, with no previous clinical related disease, which was operated on the back (L5 laminectomy) due to a workplace accident seven years ago. The persistent low back pain led the surgical team to reoperate him two years later, making a spinal arthrodesis L5-S1. After being pain free for six months, he experienced severe pain in the lumbar region again and in left lower extremity (visual analogic scale [VAS], 8). He was re-evaluated by the orthopedic surgeon finding no cause to justify the symptoms after either clinical or radiologic explorations ( and ). Physical rehabilitation program failed to improve patient's condition and therefore, he was sent to the Chronic Pain Unit. The type of pain in this case was described as a mixture of nociceptive and neuropathic pain, but the latter was the main factor detected by the DN4 test (7/10). As a first choice, two caudal steroids injections with 80 mg triamcinolone were carried performed in a three-month period. Pain intensity improved for only a short time, shorter than three weeks after each injection, and unfortunately, the last one produced a local infection that was treated with antibiotics. Due to the potential technical complications, the patient refused caudal epidurolysis. Pulsed radiofrequency was performed on the left L5 dorsal ganglion with also short-term pain relief of shorter than three months. The ganglion was located with the X-ray beam in both posterior-anterior and lateral views and by sensory stimulation (frequency, 50 Hz; pulse width, 1 msec; and voltage, 0.4-0.6 V). Lesion parameters were programmed for preventing temperature exceeding over 42℃, i.e. pulse at 45 V for two cycles of 120 seconds. Although SCS was proposed, the patient failed the psychologic exam due to generalized anxiety disorder and major depression. He was afraid of repeating interventional techniques and selected oral opioid prescription as the best option for himself. TENS was prescribed for low back pain. Medical treatment was based on dexketoprofen (25 mg, TDS), pregabalin (150 mg/d), amitriptyline (75 mg/d), duloxetine (60 mg/d), quetiapine (100 mg/d), and tramadol (400 mg/d) without desirable pain relieve (VAS > 6). Higher dose of pregabalin and amitriptyline were not tolerated well due to severe adverse effects such as dizziness, drowsiness, dry mouth, and edema. Tramadol was changed into oxycodone but increasing the dose was not tolerated well due to constipation, which was treated with the macrogol (polyethylene glycol) laxative. OXN was initiated as an opioid rotation strategy and the equianalgesic opioid dosing for switching from tramadol to oxycodone (oral-oral) was calculated based on McPherson ML, opioid conversion calculation (120 mg to 20 mg). Good pain control (VAS < 4) was obtained with increasing doses until 40/20 + 20/10 mg each eight hours. With 90 mg naloxone per day, no significant adverse effects were observed during the next twelve months. Immediate release oxycodone (20 mg) was also indicated for breakthrough pain. The Lattinen Index also improved from 17 to 10 points. |
A 54-year-old male patient with hypertension diagnosed one year before sought medical attention due to facial plethora and distal paresthesia that had progressed over the previous eight months. During the physical examination, he presented a full-moon face, facial plethora, and a nodule at the cervical area.\nThe patient had been referred from another institution in which thyroid ultrasound and color Doppler were performed. These tests showed a hypoechoic nodule in the left thyroid lobe measuring 6.7 × 3.2 × 3.5 cm, with punctate calcifications and a predominantly central vascular pattern. A fine-needle aspiration biopsy (FNAB) of the cervical nodule showed abundant pleomorphic cells with round and hyperchromatic nuclei, gross chromatin, and occasional evident nucleoli. These cells ranged in shape from plasmacytoid to fusiform, had ample cytoplasm and sporadic long extensions, and were either loose or gathered in irregular groups, with molding and overlap. Bizarre, multinucleated cells with atypical mitoses were also observed. A pinkish, glassy material was observed on the background, along with some leukocytes and erythrocytes.\nThe neck computed tomography (CT) showed a tumoral mass invading the posterior wall of the trachea, with absence of a cleavage plane between the mass and the esophagus, invasion of prevertebral muscles, and contact with thoracic vertebral bodies. The chest CT showed a large heterogeneous solid lesion, with the epicenter in the left superior mediastinum, absence of a cleavage plane between the lesion and the left lobe of the thyroid, and the largest anteroposterior diameter of 5.0 cm. The lesion extended from the glottic area to the lower portion of the trachea, invading esophagus, posterior wall of the trachea, and left jugular. Enlarged lymph nodes in the mediastinum and cervical groups were also observed.\nDuring hospitalization, the patient showed a persistent increase in blood glucose levels that reached 300 mg/dL, hypokalemia refractory to intravenous potassium, and increased urinary free cortisol levels. The plasma cortisol levels after overnight suppression with 1 mg of dexamethasone were 26.8 µg/dL, reinforcing the suspicion of CS, which was confirmed by plasma ACTH measurement of 109 pg/mL. The levels of serum calcitonin and urinary 5-hydroxyindoleacetic acid were also determined to screen for MTC and associated carcinoid syndrome. To exclude the diagnosis of pheochromocytoma, the levels of urinary catecholamines, metanephrines, and normetanephrines were also measured. Table shows the main laboratory tests performed during hospitalization.\nDue to the occurrence of hypercortisolism, ketoconazole 200 mg twice a day was prescribed, but three days later had to be suspended due to an increase in transaminases levels thrice above the reference value. Insulin therapy was started and potassium chloride replaced (oral and intravenous). Since the nodule cytological examination obtained by FNAB could not offer a conclusive diagnosis, an open biopsy of the lesion was performed. The carotid artery showed extensive invasion by the tumor and was lacerated during the procedure, requiring rapid intervention. The histopathological examination of the biopsy material indicated a neoplasm, which was confirmed as an MTC by immunohistochemistry.\nAfter discussing the case with a multidisciplinary team, it was recommended radiotherapy and chemotherapy with doxorubicin 38 mg/week for five weeks, and cisplatin 40mg/m² to a total dose of 76 mg every 21 days. During control echocardiography, the patient presented ejection fraction of 51%, and doxorubicin was suspended. He progressed with gradual clinical improvement and the tumor volume reduced. However, due to severe peripheral neuropathy, cisplatin was suspended and replaced by sunitinib 50 mg/day associated with prednisone 20mg/day. Sunitinib induced substantial toxicity, with an increase in serum glutamate-pyruvate transaminase (SGPT) and gamma-glutamyl transferase (GGT) twice above the reference value, requiring dose reduction to 25 mg/day. The abdominal CT performed six months after starting sunitinib therapy detected hepatic metastases, the largest one with 9.3 × 6.3 cm. Afterwards, the multidisciplinary team decided to suspend the medication and maintain clinical support. The patient died 23 days later. |
The third patient was a 45-year-old female with metastatic small-cell lung cancer who had undergone primary concurrent chemoradiotherapy for then-limited-stage disease, but experienced subsequent metastatic recurrence. Thereafter, she underwent salvage cisplatin/irinotecan with partial response; however, following this, she had rapid progression including bulky retroperitoneal adenopathy, with resultant back and flank pain. Following enrollment in the current study, two haploidentical related donors with identical blood types were identified. After the first three infusions (alternating donors), her back pain resolved completely and quality of life significantly improved, and she was thus able to resume her usual activities. Of note, following the third infusion she developed large DTH-like lesions at her IL-2 site injections (), and thereafter her dose of IL-2 was reduced by protocol design due to adverse effects (confluent erythematous rash, grade 2 muscle aches, decreased appetite, and decreased quality of life). Interestingly, the pain related to her cancer resolved after her first donor infusions, returned following second donor infusion, then resolved again following the third infusion (first donor). Thereafter, the first donor became her primary donor for the remainder of the study. After 10 cycles she was restaged and showed no progression in the retroperitoneal nodes, but progression in her adrenal gland for a mixed response. Her quality of life changes on study, associated with her donor infusions, are depicted in . Thereafter, she progressed rapidly in the retroperitoneal nodes, with associated recurrence of back pain. Further salvage chemotherapy was not effective until she received an irradiated blood transfusion from her first donor and her back pain resolved. Subsequently, she developed metastatic disease to the brain, and elected to proceed with hospice, 14 months after starting protocol therapy. She did not demonstrate any signs or symptoms of GVHD at any time during or after her immune transplant.\nA summary of the study patients' response and survival is given in . |
A 67-year-old male with a past medical history of congestive heart failure, non-ischemic dilated cardiomyopathy, atrial fibrillation, and pacer-dependent heart block, presented to the University of Miami emergency department with refractory complex partial seizures. Due to the fact that the patient was pacemaker-dependent, he was unable to undergo MRI. Therefore, a contrasted CT scan of the brain was performed, which demonstrated a heterogeneously enhancing left frontal mass just anterior to the left motor cortex thought to be either a primary central nervous system tumor or a metastatic lesion (Figure ).\nA full metastatic workup was conducted with no primary lesion detected. Given the location of the lesion, further imaging was thought to be necessary for two reasons: 1) to further elucidate the differential diagnosis of the lesion and 2) to help safely guide resection or biopsy of a lesion in close proximity to eloquent brain tissue. As a result, cardiothoracic surgery was consulted for the possible replacement of the patient’s current pacemaker with an MRI-compatible alternative. The patient subsequently underwent the removal of their pacemaker and replacement with a Surescan Medtronic DDD Pacemaker RVDR01 (Minneapolis, MN, US). Following this procedure, an MRI was performed and demonstrated a multi-lobulated, relatively uniformly enhancing left frontal lesion (Figure ). Due to the lack of a definitive diagnosis, the decision was made to perform an awake craniotomy with MRI-assisted stereotactic guidance and intraoperative electrocorticoraphy for a definitive diagnosis. The lesion was able to be resected with the initial pathology suggestive of Mycobacterium tuberculosae brain abscess. The patient was placed on anti-tuberculosis medication and intravenous (IV) antibiotics and discharged home. Approximately one year later, he returned to the hospital after suffering a partial seizure with subsequent right upper extremity weakness. He was found to have a recurrence of his left frontal lesion and was again taken to the operating room for surgical resection. Repeat imaging at one year did not demonstrate any further recurrence of his previously seen lesion. |
An 11- year-old boy with a complaint of swelling in left side of the upper jaw was referred to our clinic.\nSystemic signs and symptoms, past medical history and hematologic tests were within normal limits. The radiographies from chest and skull were unremarkable and no cutaneous abnormality was revealed. In panoramic radiograph, two radiolucencies with corticated border were revealed around the unerupted mandibular left canine and the unerupted maxillary left second molar area. Maxillary second molar was displaced ().\nRegarding the radiographic examination and presence of unerupted teeth and their location, the initial differential diagnosis was dentigerous cyst and the second was KCOT. Other odontogenic cysts and tumors such as adenmatoid odontogenic tumor were considered as other differential diagnoses.\nEnucleation of the cystic lesions was performed under local anesthesia and tissue samples were obtained for histopathologic examination. The surgical specimens were sheet- like with cystic appearance. After processing, the tissue samples were sectioned and stained with hematoxylin and eosin (H&E).\nThe histopathologic examination revealed that the cystic lining of mandibular lesion was corrugated parakeratinized epithelium with uniform thickness of 5-6 peg formation. The cyst wall was composed of a non-inflammatory fibrous connective tissue. All these features established the diagnosis of KCOT for the mandibular lesion (). But the maxillary lesion showed an inflammatory odontogenic cyst appearance with inflammatory cells infiltration in fibro-vascular connective tissue wall. The epithelial lining showed varying degrees of hyperplasia and rete ridge formation ().\nWith definitive diagnosis of KCOT in mandibular lesion and absence of any evidence of NBCCS in clinical examinations, multiple sections were cut from the maxillary lesion. These sections were obtained because of the probability of multiple non- syndromic KCOTs.\nH & E slides were evaluated carefully and at last a small epithelial lining with characteristic features of KCOT was detected ().\nAccording to these entire features which were correlated with the clinical and radiographic findings, the diagnosis of KCOT was established for both cystic lesions. |
A 31-year-old morbidly obese female initially presented to an outside hospital in February of 2014 with pulmonary embolism (PE) and iliofemoral deep venous thrombosis (DVT), which was presumably caused by her body habitus and oral contraceptive use. She subsequently underwent placement of a Cook Celect rIVCF (Bloomington, IN, USA) followed by pharmaco-mechanical thrombolysis of the DVT. Post-lysis, an attempt to extract the filter was made, but it could not be retrieved due to severe tilt. Although the patient complained of new-onset, severe abdominal pain shortly after the attempt at retrieval, it was felt that the filter could not be retrieved due to malposition. The subsequent plan was to anticoagulate the patient with Coumadin and leave the filter in place permanently. She was then transitioned to rivaroxiban (Xarelto) and hematologic workup was negative for inherited thrombophilia.\nThe patient continued to complain of severe, disabling abdominal pain, and 2 months later, she underwent computed tomography angiography (CTa) which demonstrated severe malpositioning of the device, with orientation at a near-transverse angle and the filter hook protruding beyond the wall of the IVC just caudal and anterior to the right renal vein ( and ). Struts of the filter appeared to be outside the IVC and appeared to be abutting the aorta as well. Given these radiographic findings and persistent abdominal pain requiring narcotics, another retrieval attempt was recommended—likely via an open approach, which the patient declined.\nThe patient subsequently presented to our institution for a second opinion 4 months after filter placement with persistent pain and was subsequently taken to the angiography suite for a second endovascular retrieval attempt. Venous access was obtained through the right internal jugular and right common femoral veins, and attempts were made to reposition and retrieve the filter. An attempt was made to balloon the filter off the cava wall with very aggressive traction on the filter with a glidewire looped around the apex of the filter. Unfortunately, the filter could not be disengaged from the wall of the vena cava despite multiple attempts.\nGiven the persistent abdominal and back discomfort, the patient still wished to have this device removed. However, given two failed endovascular attempts, a recommendation was made for laparoscopic or open filter retrieval. Three days after the most recent failed attempt at endovascular retrieval, she was taken for a planned laparoscopic or open retrieval. Unfortunately, venogram demonstrated extensive thrombus in the IVC cephalad and caudad to the filter, and filter retrieval was abandoned. Instead, the patient underwent initiation of catheter-directed thrombolysis with placement of the lytic catheter across the thrombosed segment. She remained intubated due to issues with poor respiratory mechanics in the context of her obesity and the need to lie flat for ongoing lysis via a groin sheath. The patient had an otherwise uncomplicated course of lytics, with complete resolution of the IVC thrombus after 24 h, and she was transitioned from heparin to Coumadin.\nAfter a 3-month course of anticoagulation, venogram was again performed which showed no residual thrombus in the IVC (), and the patient decided on another attempt at laparoscopic IVC filter retrieval with an open approach if it could not be extracted laparoscopically. During the procedure, the right common femoral vein was accessed with a micropuncture kit and ultrasound guidance by the vascular surgery team, and a 14 French (Fr) sheath was placed so that an occlusion balloon could be rapidly deployed if caval perforation occurred. The general surgery service then achieved peritoneal access via Veress needle (Covidien, Dublin, Ireland) in the left upper quadrant, and the abdomen was insufflated to an intra-abdominal pressure of 17 mmHg due to the excess weight of the abdominal wall, which led to initial difficulty achieving adequate insufflation at a pressure of 15 mmHg; 5-mm laparoscopic ports (Covidien, Dublin, Ireland) were placed in the left upper quadrant, right lower quadrant, left lower quadrant, epigastric, and periumbilical areas. An additional 12-mm port (Covidien) was placed in the right upper quadrant to facilitate filter retrieval, for a total of six laparoscopic ports. Visualization was achieved with a conventional-length 5-mm 30° laparoscope mounted on a Storz high-definition camera (Karl Storz GmbH & Co. KG, Tuttlingen, Germany). We should note that our camera port was rotated throughout the procedure as necessary for adequate visualization. For proper exposure of the IVC, the right colon and duodenum were mobilized laparoscopically. There did not appear to be any free fluid within the peritoneal cavity upon thorough initial laparoscopic inspection. Upon Kocherization, the IVCF tip was seen to be cleanly protruding from the wall of the cava, with a lack of both leakage around the filter tip and local peritoneal reaction. No defect was noted on the duodenum and minimal dissection around the filter hook was required for adequate visualization.\nWe subsequently used a gooseneck snare passed through the 12-mm port to grasp the top of the filter, and a 12 Fr sheath was passed down over the snare to collapse the filter. The filter subsequently easily disengaged from the wall of the IVC and was pulled up and out of the abdomen along with the 12 Fr sheath. Prior to removal of the filter, a Coda balloon (Cook, Bloomington, IN, USA) had been positioned at and level of the filter, and concomitant with filter extraction was inflated to balloon tamponade any bleeding from the IVC (). There was minimal bleeding and after 3 min of balloon inflation and completion venography demonstrated no extravasation (). The general surgery team laparoscopically ran the bowel from the ligament of Treitz to the terminal ileum, and there was no evidence of serosal tear or other injury. Wounds were closed primarily, and the patient was extubated at the end of the procedure and subsequently had an uncomplicated hospital course with discharge on postoperative day 1 and no systemic anticoagulation.\nShe was seen in clinic 2 weeks following her procedure and was noted to have subjective improvement in her abdominal and back pain. The authors would like to note that our institutional review board (DUMC) policy does not require prior ethics approval in the reporting of individual cases, and that written informed consent was obtained relative to the individual whose disease process is herein discussed. |
A 13-year-old girl presented with a progressive painless abdominal distention for almost two months. She had not experienced any menstruation cycles before. Physical examination revealed shifting dullness on a very distended abdomen. Abdominopelvic ultrasonography showed increased amounts of ascites and a cystic lesion with few septations in the pelvic cavity that measured 70 mm × 64 mm in greatest diameter and seemed to have mostly arisen from the left adnexa. Right ovary was also enlarged with a cystic lesion of 36 mm in maximum diameter. Massive amounts of ascites in the abdominopelvic cavity were present. Abdominopelvic CT scan demonstrated a cystic mass lesion arising from the left ovary with multilocular thick-walled components and a large cystic component extending to the right side of the pelvic cavity. There was also large amounts of ascites (). The levels of β-hCG, AFP, CEA, and CA 19–9, CA-125 were measured preoperatively which were all within the normal range. Tumor resection with left salpingoophorectomy and bilateral omentectomy was performed through which about 10 liters of transudate ascites fluid was evacuated from the abdomen. Cytology of ascitic fluid was negative for malignancy.\nGrossly, there was a tumor on the surface of the ovary without any obvious capsule associated with the attached oviduct. Tumor was a large predominantly cystic multilocular lesion measuring 9 × 5 × 3.5 cm filled with clear yellow fluid and scattered papillary projections. Histopathology revealed ovarian tissue partially replaced by a neoplasm of complex growth pattern with nodules of ovoid and polygonal pale eosinophilic to clear cells as well as multiple irregular follicles with cystic changes in some of them and proliferations of granulosa-like cells in the ovarian stroma associated with pseudopapillary projections compatible with Juvenile granulosa cell tumor (). Immunohistochemical analysis of tumor was positive for calretinin (), inhibin (), and CD99. Chest CT-scan and bone survey were unremarkable. Based on the FIGO staging system for ovarian tumors, the patient was classified as stage IC []. In our patient, serum inhibin level was not assessed before surgery, because the diagnosis of GCT was not suspected. However, there was an increased level of inhibin measured immediately after the surgery. Based on the results of the follow-up imaging postoperatively which showed evidence of residual tumor in the pelvic cavity and serum inhibin level, the patient was scheduled to receive 4 cycles of adjuvant chemotherapy with carboplatin, bleomycin, etoposide, and vinblastine. Follow-up imaging studies after commencing chemotherapy came as normal. The patient who had not experienced menstruation before developed a normal pattern of menarche shortly after the surgery. She was in complete remission without any residual tumor in an 18-month follow-up period. |
Eleven years ago, when our patient was a 33-year-old unmarried nulligravida, she developed CCC of the ovary for the first time. At that time, her menstrual cycle was regular and 30 days long, with dysmenorrhea, manifesting as lower abdominal and lumbar pain, persisting for 5–7 days. There was no family or medical history of note. She had visited a local clinic with the chief complaint of lumbar pain, where a right ovarian tumor 8 cm in size that included solid internal components, was identified, and she was referred to the University of Tsukuba Hospital. Transvaginal ultrasound had revealed a right adnexal mass with solid components, 68 mm × 53 mm in size, and an intramural uterine fibroid 23 mm × 27 mm, with no hypertrophy of the endometrium, which measured 2.1 mm. Blood counts and blood biochemistry test results revealed no abnormalities. Her serum CA19-9 level was 45.9 U/ml, CA125 was 22 U/ml, and CEA level was 1.3 ng/ml. Abdominal computed tomography (CT) and magnetic resonance imaging (MRI) revealed an 80-mm unilocular cystic mass in the right ovary with a papillary protrusion of longest diameter 45 mm. The left ovary was not enlarged, and there was no obvious peritoneal dissemination or enlarged lymph nodes. Surgery, comprising right adnexectomy, left ovarian biopsy, partial omentectomy and uterine fibroid removal, was performed. The right ovary was enlarged to 8 cm in size, and it was removed without intraoperative rupture and with its capsule intact. The left ovary was not enlarged, but was seen to have a small endometrial cyst, which was resected. The absence of peritoneal dissemination and enlarged lymph nodes was confirmed during surgery. The pathological diagnosis was CCC localized to the right ovary and endometriosis was observed in the left ovary, with ascites cytology Class III. The cytology was composed of clusters that include atypical cells having somewhat nuclear enlargement, and it makes a diagnosis difficult to distinguish mesothelial cells from malignant cells. The patient expressed a strong desire to preserve her fertility. Hence, pelvic and para-aortic lymph node dissections were performed via staged laparotomy, which revealed no metastases in any of the 91 pelvic or para-aortic lymph nodes that were removed, leading to a diagnosis of Stage IA CCC. Four courses of postoperative combination paclitaxel (175 mg/m2) and carboplatin (AUC6) chemotherapy (TC therapy) were administered to complete the initial treatment.\nNine years after the initial therapy, MRI during regular 6-monthly monitoring revealed the appearance of a 95-mm polycystic mass with a mural nodule of longest diameter 53 mm in the left adnexal region. Those findings were never seen at the previous MRI. At this time, her serum CA19-9 level was 12.9 U/ml, CA125 was 18.3 U/ml, and CEA level was 0.5 ng/ml, all of which were within normal limits. Disease recurrence in the contralateral ovary was diagnosed, and total abdominal hysterectomy, left adnexectomy and omental biopsy were performed. At the time of surgery, the left ovary was enlarged to 95 mm in size, and it was removed intact without intraoperative rupture of its capsule. The internal lumen of the tumor contained several milky-white mural nodules extending around 15 mm into the cavity (Fig. ). The histological diagnosis was CCC, but in addition to the possibility of recurrence, it was also considered that the tumor might have developed de novo, and the fact that clear cell adenofibroma (CCAF) was also present (Fig. ) suggested that this might have provided the genesis for its development. In addition, there were no endometriotic lesions in the non-solid cyst wall, and it only consists of fibrous membrane. The tumor was localized to the left ovary, and since ascites cytology was negative, it was diagnosed as Stage IA disease. The treatment was completed with four courses of postoperative TC therapy.\nTwo years after the completion of treatment, the patient is continuing outpatient monitoring with no sign of recurrence to date. |
An 81-year-old woman was presented in the emergency department (ED) after falling on her left hip. She complained from severe pain in the left hip and inability to bear weight on her left leg. Clinical examination showed a shortened left leg in external rotation; all movements as well as axial pressure were painful.\nRadiographs of the left hip, anterior-posterior and axial, showed a pertrochanteric multifragmentary fracture of the proximal femur (AO Classification 31-A2) ().\nThe next day the patient was operated on where the fracture was fixed with a third-generation short Gamma nail (Stryker, Kalamzoo, USA), using a set screw to control rotation of the lag screw. The procedure and postoperative course were uneventful, with the Gamma nail in good position as could be confirmed on the postoperative X-ray (Figures and ). The patient was able to mobilize and was allowed full weight bearing of the left leg the next day. After 11 days the patient was discharged to a rehabilitation center.\nAfter six weeks we saw the patient in the outpatient clinic for a scheduled follow-up. At that point there were no complaints and she was mobilizing with a walker. The X-ray after 6 weeks () showed an identical position of the Gamma nail and consolidation of the fracture.\nUnfortunately, three months after the operation the patient presented herself again in the ED with acute pain in her left hip for 2 days and an inability to walk. There was no preceding trauma and there were no signs of an infection. The X-ray in the ED showed medial migration of the lag screw ().\nIt was decided to remove the Gamma nail and replace it by a cemented total hip arthroplasty (Exeter Stem, Rimfit cup, Stryker) during the same session. Intraoperatively, the fracture appeared consolidated and there were no signs of infection.\nPatient was allowed to bear full weight one day after surgery. The X-ray showed a good position of the total hip arthroplasty (). All the cultures that were taken during the removal of the Gamma nail showed no growth of bacteria.\nDespite admission into the coronary care unit (CCU) for 1 day after the procedure for continuous monitoring of the cardiac rhythm, the rehabilitation was uneventful. After 15 days, the patient returned to the rehabilitation center for further recovery. |
An 86-year-old female with a history of metastatic ovarian cancer presented to the ED with painful bilateral lower extremity edema and a left lateral leg ulceration. Her metastatic ovarian cancer had been diagnosed by malignant pleural effusion five months earlier, and she had completed neoadjuvant chemotherapy with carboplatin and Taxol approximately one week prior to this presentation. She was admitted to the hospital and started on cefazolin for left lower extremity cellulitis on hospital day one.\nOn admission, plain films and ultrasound did not reveal any evidence of osteomyelitis, fracture, DVT, or abscess to the left lower extremity. On exam, she had 3+ pitting edema below the knee bilaterally as well as chronic venous stasis changes. The patient also had a venous ulcer (approximately 2 cm in diameter) on the anterolateral aspect of the distal third of her left lower leg. At the time of admission, this venous ulcer had some serous weeping but no purulent drainage or fluctuance on examination. Her initial Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) score was 4, suggesting a low risk for necrotizing fasciitis; however, on hospital day 3, her CRP began to uptrend and she became febrile. At this point, her antibiotics were switched from cefazolin to vancomycin to cover MRSA.\nOn hospital day five, the patient was noted to have a new erythematous area over the anterior left knee, inferior to the patella (). Ultrasound revealed a small fluid collection superficial to the patellar tendon in the infrapatellar region measuring 3.3 × 2.5 × 0.4 cm (). The infrapatellar bursa was aspirated and sent for culture. The patient was started on piperacillin-tazobactam, given the patient's immunocompromised status and subsequent risk for atypical and gram-negative organisms.\nAn MRI was performed on hospital day seven (this was delayed due to the patient's pacemaker) but did not reveal any evidence of osteomyelitis. The patient was clinically improved after starting piperacillin-tazobactam, and vancomycin was discontinued on hospital day seven. On hospital day eight, aspirate cultures returned with Pseudomonas aeruginosa; she was stable for discharge at that time and was sent out with a ten-day course of levofloxacin (culture was pan-sensitive) and close follow-up with infectious disease. |
A 37 years-old unmarried premenopausal female presented in the emergency of our hospital with complaints of bleeding from ulcerative lesion in the left breast for a couple of hours. The patient had history of ulcer in the left breast for past 2–3 weeks which developed over the underlying huge breast lump. The lump was present for past 5 months but rapidly grew over the last 3 months to current size. There was no history of coagulopathy, trauma, chronic liver disease or history of similar illness in first degree relatives. The patient attained menarche at the age of 15 years. She is a non-smoker and denies history of contraceptive use or alcohol intake.\nOn examination in our emergency there was severe pallor with tachycardia. However the blood pressure was maintained. There was an oozing ulceration in upper outer quadrant of the left breast with underlying huge lump measuring 15 × 15 cm occupying the upper outer, lower outer and upper inner quadrants with deformed shape of the breast (a). The skin surrounding the ulcer was erythematous and edematous. Axilla did not show lymphadenopathy.\nBefore the development of breast lump, the patient had itchy lesion at the same site. The patient unfortunately attributed all these events to that itchy lesion and sought help late. There was no history of headache, shortness of breath, abdominal distension or bone pain.\nOn investigating the patient, she was severely anemic with hemoglobin of 5 gm% with normal total and differential counts. Coagulation parameters and liver function tests were normal. Chest X-ray showed no evidence of metastasis other than dense breast shadow on left side due to huge breast lump(a).\nMammogram revealed homogeneous radiodense lobulated mass occupying whole of left breast with subcutaneous thickening on the background of dense breast (b). Ultrasound (USG) of abdomen and pelvis did not reveal any abnormal findings. Trucut biopsy from the breast mass showed necrotic areas with fibrocollagenous tissue with proliferation of oval to elongated spindle cells showing mild degree of atypia.\nAfter discussion about the possibility of metaplastic versus malignant phyllodes tumor, the patient underwent modified radical mastectomy (MRM). Histopathological examination showed maximum tumor size of 14 cm with increased stromal cellularity, loss of stromal-epithelial balance and frequent mitoses more than 45 per 10 high power fields () typical of malignant phyllodes. All the resected margins, nipple and areola were free of tumor. Lymphovascular and perineural invasions were not identified. Seventeen axillary nodes retrieved were free of tumor.\nPostoperatively patient gained weight and was doing well. Three months after surgery, the patient started developing progressively increasing headache, nausea and vomiting for which she got admitted in our center. There was no local recurrence. However on further investigations, she had a huge cystic lesion with mural nodule in her brain (b,c,d). Contrast enhanced computed tomography (CECT) of chest, abdomen and pelvis showed lesions in lower lobe of left lung (a) of size 3.9 × 3.6 cm of +40 Hounsfield unit (HU) in posterobasal segment and right adrenal gland () of 5.8 × 5.1 cm of +30HU with significant enhancement in post-contrast images. Functional evaluation for the adrenal mass with urinary metanephrines and serum cortisol was negative. Patient refused biopsy from right adrenal gland and lung, but while preparing for burrhole biopsy from brain, patient succumbed to the disease. The patient underwent serial ultrasound of abdomen and pelvis during hospital stay which showed increase in size of 4 cm of adrenal mass in 15 days (tumor velocity). |
A 95-year-old man was admitted to the geriatrics department with a complaint of blood in the sputum for 20 days. Nasopharyngoscopy and gastric ultrasound did not find the evidence of bleeding from the upper airway and stomach. Combined with chest computed tomography (CT) results, hemoptysis was highly suspected due to bronchiectasis with infection. After conservative treatment, the hemoptysis gradually resolved. However, progressive dyspnea occurred in course of the disease. On 10th October 2016, ETI was performed due to asphyxia and respiration arrest. Spontaneous breath was rapidly restored with bag-mask ventilation and sufficient bloody sputum suction (100 ml). He was then transferred to the intensive care unit for further treatment.\nMechanical ventilation was removed 28 days after ETI, and oxygen was administered through the endotracheal tube (ETT). Due to neurological insult, his airway clearance ability was still poor, and the standard for removal of the ETT cannot be achieved. However, his family members refused to perform tracheostomy as special considerations.\nDuring the following day 240 and day 329 after ETI, three dimensional (3D) CT reconstruction of the airway was performed and laryngeal damages, such as tracheal esophageal fistula and significant airway stenosis around the balloon were not found (Fig. ). On day 459 after ETI, ETT exchanged was performed. In gross observation, the ETT was changed to yellow in color. Meanwhile, the balloon was stiff and inelasticity (Fig. ). Although on day 547 after initial ETI, 3D CT of the airway indicated that there was a possible fistula between the anterior wall of the upper esophagus and the posterior wall of the trachea (Fig. ). However, the gastroscopy did not reveal the fistula on the esophagus and the chest CT follow-up also did not indicate mediastinal infection after enteral nutrition through a gastric tube.\nOn day 662 after initial ETI, ETT exchanged was performed again due to the leakage of the balloon. Laryngoscope showed that a large amount of granulation tissue surrounded the glottis, and the glottis was barely visible. Finally, on day 839, he received tracheostomy due to acute sputum occultation within ETT and abrupt oxygen desaturation.\nVentilator-associated pneumonia was reported on day 11. Acinetobacter baumannii, Pseudomonas aeruginosa, and Klebsiella pneumonia were identified in the following sputum culture. On day 58 after initial ETT, pressure ulcer occurred on the lip and recovered subsequently. |
A 21-year-old female with no comorbidities and negative family history for any autoimmune disease started experiencing low-grade undocumented fever for 3 months in August 2019 which was continuous, non-radiating, and shooting in nature with no aggravating or relieving factors. Radiological studies were unremarkable.\nOver the course of a few months, the patient began to experience arm and limb claudication. This lasted 30 days and was relieved on its own, only to reoccur 10 months later. During this period, the patient also suffered a 5-kilogram weight loss, headache, dizziness, and nausea. She visited a local general practitioner, who offered her symptomatic treatment.\nIn October of 2020, the patient noticed that her radial pulses were weak and she was admitted. On examination, her brachial and radial pulses were weak, impalpable bilaterally and her BP was non-recordable manually. Ophthalmic examination revealed bilateral narrowing of arterioles in both eyes. However, no sign of arteritis was observed in the retina. Investigations indicated that the patient had hypochromic microcytic anaemia with haemoglobin level of 8. Furthermore, a CRP of 40, an ESR of 50, and a low HDL cholesterol of 30 were observed. CT angiogram showed narrowing in right ICA and left CCA, a segment of the right subclavian artery was narrowed distal to its origin from brachiocephalic artery, short segment narrowing in the celiac trunk, and narrowing at origin of SMA as can be appreciated in Figure .\nAlong with this, left superficial and deep femoral arteries were markedly attenuated with collateral formation. The rest of the investigations including ANA and anti-ENA screens were negative. The patient was started on methylprednisolone 500 mg IV for 3 days, tablet prednisone 25mg BD, tablet aspirin 75 mg OD, tablet methotrexate 20g weekly, capsule gabapentin 75 mg BD, tablet folic acid 5 mg OD, and tablet atorvastatin 20 mg HS. The patient responded well to the treatment given, her pulses were palpable again and her symptoms improved. Inflammatory markers came down to normal range and steroids were gradually tapered off over a period of 1 month. Currently patient remains asymptomatic. |
A 61-year-old woman presented with two supernumerary nipples located along the milk line on each side of the upper abdomen. During a few months before referral, the patient had noticed a firm palpable mass in close relations to the supernumerary nipple on the right side (). She had no other symptoms. Bilateral mammogram and ultrasound revealed normal breast parenchyma. Ultrasound of the supernumerary nipple on the right side confirmed a small mass in relation to this nipple, presenting as a hypoechoic, well-defined area, measuring approximately 10 mm in diameter ().\nUpon clinical examination, the supernumerary nipple on the right side presented with inversion and a palpable firm mass underneath this nipple. Ultrasound-guided needle core biopsy (16G) was performed (). The pathology report described elongated epithelial islands composed of cubic cells, with a small centrally located lumen with focally identifiable cuticles. The epithelial structures were surrounded by a dense fibrous stroma. The microscopic analysis indicated the possibility of SyT and the lesion was categorized as a B3-lesion (a lesion with uncertain malignant potential) ().\nFollowing a Multidisciplinary Team Conference, it was decided to recommend resection with a 5 mm rim of normal tissue, which was subsequently performed ().\nMacroscopically the tumour measured 7 x 5 x 5 mm and was described as a firm and grey-white tumour, in close relations to the supernumerary nipple. Microscopically the tumour measured 13 mm in diameter and was localized in the deep part of dermis and underlying subcutaneous tissue with no relation to the epidermis. The tumour was composed of solid trabecular and glandular imitating formations with focal cysts. The epithelium consisted of cells with slightly irregular nuclei and indistinct nucleoli, surrounded by an eosinophilic cytoplasm. The glandular structures were lined by cuboidal cells. The tumour was in close association with the lactiferous ducts and smooth muscle of the nipple (). Foreign body giant cell reactions, due to ruptured cysts, were identified.\nImmunohistochemical analysis showed positive reaction for CK5, CK14, and P63 () in association with the epithelium presenting as solid cords, whereas the glandular luminal cells showed positive reaction for CK7 (). Thus, the immunohistochemical analysis demonstrated the complex nature of this lesion. The diagnosis was SyT based on the pathology report.\nA re-excision was performed due to insufficient rim of normal tissue in the cranial direction in order to minimize the risk of recurrence. |
A 67-year-old male with a past medical history of congestive heart failure, non-ischemic dilated cardiomyopathy, atrial fibrillation, and pacer-dependent heart block, presented to the University of Miami emergency department with refractory complex partial seizures. Due to the fact that the patient was pacemaker-dependent, he was unable to undergo MRI. Therefore, a contrasted CT scan of the brain was performed, which demonstrated a heterogeneously enhancing left frontal mass just anterior to the left motor cortex thought to be either a primary central nervous system tumor or a metastatic lesion (Figure ).\nA full metastatic workup was conducted with no primary lesion detected. Given the location of the lesion, further imaging was thought to be necessary for two reasons: 1) to further elucidate the differential diagnosis of the lesion and 2) to help safely guide resection or biopsy of a lesion in close proximity to eloquent brain tissue. As a result, cardiothoracic surgery was consulted for the possible replacement of the patient’s current pacemaker with an MRI-compatible alternative. The patient subsequently underwent the removal of their pacemaker and replacement with a Surescan Medtronic DDD Pacemaker RVDR01 (Minneapolis, MN, US). Following this procedure, an MRI was performed and demonstrated a multi-lobulated, relatively uniformly enhancing left frontal lesion (Figure ). Due to the lack of a definitive diagnosis, the decision was made to perform an awake craniotomy with MRI-assisted stereotactic guidance and intraoperative electrocorticoraphy for a definitive diagnosis. The lesion was able to be resected with the initial pathology suggestive of Mycobacterium tuberculosae brain abscess. The patient was placed on anti-tuberculosis medication and intravenous (IV) antibiotics and discharged home. Approximately one year later, he returned to the hospital after suffering a partial seizure with subsequent right upper extremity weakness. He was found to have a recurrence of his left frontal lesion and was again taken to the operating room for surgical resection. Repeat imaging at one year did not demonstrate any further recurrence of his previously seen lesion. |
The proband was a 35-year-old male from Hebei province in the north of China. He is of Han ethnicity and was born to consanguineous parents. His family pedigree is shown in Fig. . The proband was normally delivered after a full-term pregnancy, and birth weight and length were within normal ranges. The initial signs and symptoms appeared when he was 6 years old. Deformity of interphalangeal joints initially appeared in the fingers. Hips, knees, and wrists were then gradually involved. Diagnosis of JRA was considered by local hospitals, and glucocorticoids were prescribed without any efficacy. As he grew up, his symptoms deteriorated. He had to walk with crutches at 16 years of age because of arthritis of the lower extremities. At 26 years of age, he first experienced progressive pain with numbness radiating down his entire left leg and right thigh. At 34, he started to have mild difficulty in urination. Thereafter, his leg pain progressed and he became immobile. Treatment with tramadol, physical therapy, and spine injection were tried but were not effective. He had a younger brother with a similar clinical presentation but who also had mild neurological impairment (Fig. ).\nThe proband’s height and weight were 162 cm and 72.5 kg when he was admitted to our hospital. His visual analogue scale (VAS) score was 9. He did not have behavioral difficulties and was not retarded in his intellectual development. Physical examination showed multiple malformations of the major limb joints, especially of the knees and hands (Fig. ). Amyotrophy of both lower limbs was obvious. Cervical and lumbar movements were limited with compensatory kyphosis. The muscular strength of all four limbs was normal. Dysesthesia was found in the posterolateral left calf, dorsolateral left foot, and perineal area. Bilateral knee-jerk reflexes and ankle reflexes were hypo-induced. The erythrocyte sedimentation rate (13 mm/h) and C-reactive protein level (2 mg/L) were both within the normal range. Tests for rheumatoid factors were negative.\nSpinal x-rays showed flat and osteoporotic vertebral bodies. Pedicles were short, and end plates were irregular. Bone bridges were seen at many levels. Kyphosis was detected in both the cervical and upper thoracic spine. Magnetic resonance imaging showed multilevel Schmorl nodes. Multilevel disc herniation and hypertrophic ligamentum flavum caused lumbar canal stenosis from L2 to S1 (Fig. ). |
A 45-years-old male engineer with no history of trauma, presented to us with progressive upper back pain for two months that eventually became severe and disabling. The pain was non mechanical and was present even at rest and night. He denied any constitutional symptoms. He had no significant medical history except that he was a smoker for over 20 years.\nOn neurological examination, patient indicated altered sensation from T8 dermatome and below; however, upper and lower limb motor power was normal. He had an unsteady gait and exaggerated deep tendon reflexes in both lower limbs.\nInitial X-rays of the thoracic spine were unremarkable, but MRI demonstrated abnormal marrow replacement and enhancement of the entire T6 vertebra including its posterior elements and right 6th posterior rib. MRI axial cuts at T6 level revealed right postero-lateral epidural extension of the lesion causing severe spinal canal, right lateral recess and right exit foraminal stenosis (, ). In addition, an irregular right lung nodule was noted. CT evaluation confirmed the vertebral involvement () and the presence of a lobulated right lung nodule with emphysematous changes in bilateral upper lobes. Further radiological assessment revealed no other lesion elsewhere.\nDuring the course of the work up, he developed bilateral lower limb weakness and was unable to stand. A diagnosis of thoracic myelopathy due to cord compression was made and immediate surgical management in the form of T6 decompression laminectomy along with T3-T8 posterior instrumentation was performed (). Surgery was uneventful; patient had good recovery and was ambulating independently.\nTissue biopsy from T6 right pedicle revealed presence of metastatic tumour cells with moderate amount of eosinophilic cytoplasm in the marrow spaces suggestive of a metastatic carcinoma with neuro-endocrine features (). Tumour cells were positive for cytokeratin AE1/3, carcinoembrionic antigen (CEA), chromogranin and synaptophysin. Proliferation marker Ki67 was found to be 40%.\nEarly post-operative MRI showed adequate decompression of the spinal canal at T6 level and the patient had interval resolution of symptoms; but there was abnormal fluid collection from the surgical site extending up to the subcutaneous layer, likely to be seroma, which was conservatively managed. Chemotherapy with Carboplatin and Etoposide was initiated after satisfactory wound healing three weeks after surgery.\nOne month after the index surgery, patient developed significant motor deficit in the lower limbs (Right L2-L5 = 2/5; Right S1 = 4/5; Left L2-S1 = 4/5) and had bowel and bladder incontinence. An urgent MRI was done which revealed recurrence of the lesion causing near total obliteration of the spinal canal ().\nA second surgery at this stage was considered high risk and the decision was made to initiate concurrent site specific T4-T7 radiotherapy (30 Gy in 10 fractions) along with chemotherapy. Interestingly at the end of radiotherapy and four courses of chemotherapy (4 months post-op), there was significant clinical improvement of neurology and complete resolution of the soft tissue enhancement surrounding the spinal cord was evident in the MRI (). With appropriate physiotherapy and rehabilitation, patient gradually regained full power in both lower limbs by 6 months. He continues to be under oncology follow up and is ambulant with support. |
A 28-year-old female patient reported to our institution with a complaint of a depression appearing on the right lower facial region. It had started as a small depression two years earlier, with no accompanying pain or discomfort, which progressed to the present state of asymmetry and abnormal appearance. Her medical records showed no history of facial trauma or dental infections. On examination, the facial asymmetry was found to be due to deficiency of the fat layer in the right parasymphyseal region, extending inferiorly to the lower border of the mandible []. The regional skin was extremely thin with pigmentary changes []. The intraoral examination revealed healthy oral mucosa and a complete set of dentition. Localized idiopathic subcutaneous atrophy of the face was considered as a clinical diagnosis and the relevant diagnostic workup was started.\nAn en-face photograph and profile views of the patient were taken and analyzed together, to assess the degree of asymmetry [Figures and ].\nUltrasonography using, 7-10 MHZ frequency linear probe (Volvuson 730 pro expert GE machine) revealed the normal superficial skin layer bilaterally. The thickness of subcutaneous plane was 0.22 cm on the right side and 0.28 cm on the left side. Altered echogenicity was noted on the right masseter muscle. On the affected side, the muscle thickness was 0.83 cm, whereas, on the normal side, it was 1.26 cm [Figures and ]\nThe serial axial and coronal CT sections of the craniofacial bones obtained with multiplanar and volume-rendered reconstructions confirmed the asymmetry of the subcutaneous soft tissues, which were atrophied on the right cheek region. The underlying muscles and bones appeared normal [Figures and ]\nThe patient was advised biopsy, but she refused. Clinical investigations showed normal values for complete blood count, blood glucose, serum cholesterol, and triglycerides. Plasma C3 and C4 levels were within normal limit. The Antinuclear Antibody (ANA) test was negative.\nBased on the clinical and imaging, we concluded the final diagnosis as localized, idiopathic involutional grade 1 lipoatrophy of the face. The patient was treated in the Plastic and Reconstructive Surgery Department. Autologous fat harvested from the abdomen was injected to the subcutaneous plane to rebuild the sunken area [Figures and ] |
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