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A 71-year-old Irish Caucasian female presented with sub-acute bilateral lower limb pitting edema and dyspnea. Her background history included obesity (BMI 45.5), atrial fibrillation, obstructive sleep apnea, subclinical hyperthyroidism, eosinophilic colitis, and diverticular disease. She was an ex-smoker with a 20 pack-year history. Furthermore, she was under surveillance for a 3 cm ovarian cyst with the local gynecology services.\nThe patient had normal cardiac and respiratory examinations. Her transthoracic echocardiogram showed an ejection fraction of more than 50% with normal left and right ventricles and valves. Her chest radiograph was also unremarkable. An abdominal ultrasound was performed to assess for a pelvic mass that could impede pelvic venous return leading to peripheral edema. Ultrasound revealed an 8 cm hyperechoic mass lesion at the superior pole of the right kidney as well as a 2 cm hypoechoic liver lesion (Figure ). Subsequent computed tomography (CT) the of abdomen and pelvis with contrast confirmed an 8 cm smoothly defined heterogeneous right adrenal mass (Hounsfield Unit 57.75) and a number of low attenuation lesions throughout the liver (Figure ).\nThe patient exhibited potential features of cortisol excess including weight gain of 11 kg over two months and depression. She also had a newly diagnosed type 2 diabetes mellitus prior to admission with glycated hemoglobin (HbA1c) of 50 mmol/mol. The hormonal evaluation was consistent with hypercortisolism with failure to suppress on overnight dexamethasone suppression test and elevated 24-hour urinary free cortisol (Table ). There was no clinical or laboratory evidence of pheochromocytoma, hyperaldosteronism, or increased sex hormone production.\nThe patient was transferred to a tertiary referral center for surgical evaluation. Magnetic resonance imaging (MRI) and repeat CT thorax, abdomen, and pelvis demonstrated a large necrotic adrenal mass consistent with a primary adrenocortical carcinoma with direct invasion into the intrahepatic inferior vena cava. Multiple lung nodules concerning pulmonary metastasis were also observed. A diagnosis of adrenocortical carcinoma with pulmonary and hepatic metastasis was made. Due to metastatic disease, she was deemed unsuitable for surgery and treated pharmacologically with mitotane and metyrapone to manage symptoms caused by excess cortisol production by the tumor. She subsequently progressed to palliative care and died three months after diagnosis. |
A 38-year-old primigravida at 32 weeks and 5 days of gestation presented to labor and delivery with heavy vaginal bleeding for three hours, cramping, and leaking of fluid. Obstetric history was significant for being a late registrant from a different country, advanced maternal age, and marginal cord insertion. On speculum examination, a 6 x 3 cm nonfriable polyp in the endocervical canal with a small amount of blood was noted in the vaginal vault. She was discharged after complete evaluation for rupture of membranes and other causes for antepartum hemorrhage. A dedicated sonogram was scheduled to evaluate the origin of the polyp. She presented to the ultrasound unit at 37 2/7 weeks actively bleeding with 50 mL of bright red blood in the vagina and was experiencing intermittent bleeding twice weekly since 35 weeks of gestation. On sonogram, a 3,105 g fetus in cephalic presentation with a posterior fundal placenta and a polyp measuring 6.63 x 1.32 x 1.68 cm, with a stalk in the cervical canal, was noted (Figure , ).\nShe was transferred to labor and delivery and was counseled by maternal-fetal medicine about options to proceed with either an elective cesarean section followed by polypectomy or polypectomy and expectant management of spontaneous labor. The patient opted for the latter option. Polypectomy was performed in the operating room as described below under spinal anesthesia. Two grams of cefoxitin was given as surgical prophylaxis in anticipation of proceeding with cesarean delivery in case of prolonged bleeding after polypectomy. The polyp was friable and removed in two pieces. She was discharged home the next day. The estimated blood loss was 20 mL. Pathology showed two 2.3 x 2.0 x 0.2 cm endocervical polyps with acute and chronic inflammation, as well as microglandular hyperplasia. The patient had an uneventful prenatal course following polypectomy. She was admitted for spontaneous rupture of membranes at 39 6/7 weeks and was induced with misoprostol, Cook balloon, and oxytocin and had an uncomplicated normal spontaneous vaginal delivery with an estimated blood loss of 300 mL.\nProcedure for polyp removal\nPatients were counseled about a lack of definitive recommendations in the literature regarding the management of polyps during pregnancy, and written consent was obtained after informing patients about the possible risks of bleeding, infection, preterm labor, premature rupture of membranes, chorioamnionitis, and miscarriage. All three patients had a dedicated ultrasound prior to polypectomy and were determined to have endocervical polyps with the origin in the cervical canal. Patients were kept in dorsal lithotomy position, and the perineum was prepped and draped in a normal sterile fashion. A bivalve speculum was placed in the vagina. The exact location of the polyp and stalk was then identified. The polyp was grasped with DeBakey’s forceps, and a 0-Vicryl Endoloop was used to strangulate the stalk closer to the base. The polyp was detached from its stalk using electrosurgery, and the base of the stalk was cauterized to ensure hemostasis. The fetal heart rate was assessed after the procedure by either ultrasound or performing a nonstress test. |
A female in her early 70s was referred to our hospital for endoscopic treatment of a 15-mm laterally spreading tumor adjacent to the appendiceal orifice, which was detected incidentally during a screening colonoscopy. An ER of the lesion had been attempted at another hospital without success and only biopsy had been performed after that. Her comorbidities included hypertension, dyslipidemia, and osteoporosis. Her medical history was negative for previous abdominal surgery, allergies, smoking, and alcohol consumption.\nMagnifying endoscopy with narrow-band imaging and chromoendoscopy suggested the tumor was a simple adenoma. After obtaining written informed consent, we performed ER of the tumor. We began injected glycerol into the submucosal layer and incised the mucosa bordering the appendix side of the tumor using a DualKnife® (Olympus Corp., Tokyo, Japan). Subsequently, the submucosal layer was carefully dissected using the same device. However, due to the scarring and fibrosis resulting from the previous attempt at resection, the submucosal layer did not lift adequately, and the submucosal dissection was extremely difficult. Consequently, an intraoperative micro perforation occurred but was tightly closed by clips. When the tumor was almost resected by ESD, we completely removed it using a snare. The size of the lesion was 15 × 15 mm (Fig. ). After the ESD, there was neither postoperative bleeding nor inflammatory reaction suggesting peritonitis. The patient was discharged 5 days after ESD as scheduled. Pathological examination revealed that the tumor was a high-grade tubular adenoma, and we diagnosed a curative resection of the lesion.\nThe patient initially did well after she was discharged. However, 10 months later she experienced sudden severe abdominal pain and a low-grade fever before a meal. She visited her family physician immediately. Physical examination disclosed localized rebound tenderness at McBurney's point at that time. The laboratory analysis revealed an inflammatory reaction with a leukocyte count of 14,700/μL (79.1% neutrophils) and a C-reactive protein level of 4.5 mg/dL. Therefore, she was suspected of having acute appendicitis requiring emergency surgery. She was transferred to a general hospital near the clinic.\nAbdominal computed tomography showed appendiceal wall thickening and swelling, an intraluminal fecal calculus, and fluffing of the surrounding fat tissue. The patient was diagnosed with acute appendicitis and underwent emergency surgery. Because the surgeon suspected that her appendicitis was associated with residual tumor, the patient underwent a laparoscopic cecectomy. Intraoperatively, the appendix was firmly adherent to the terminal ileum and retroperitoneum, and the root of the appendix was intussuscepted into the cecal lumen.\nMacroscopic examination of the resected specimen revealed a perforation in the central portion of the resected appendix and a thick scar obstructing the appendiceal orifice that did not allow the passage of a probe (Fig. ). Microscopic examination revealed no residual tumor and identified a broad band of fibrotic tissue adjacent to the appendiceal orifice consistent with a scar (Fig. ). The lesion was pathologically diagnosed as gangrenous appendicitis. The scar was attributed to the previous ESD and was the only cause of appendicitis identified.\nThe patient was discharged 4 days after surgery. Two months postoperatively, she underwent a 1-year ESD follow-up colonoscopy. This examination showed a postoperative scar, and there were no other findings. |
A 28-year-old man (height 158 cm, 45 kg) presented to the preanesthetic assessment clinic before an elective right total hip replacement. He had ankylosing spondylitis that severely restricted the range of motion in his cervical, thoracic and lumbar spine. There was no cardiopulmonary involvement, and his hematological and biochemical parameters were within normal limits. Radiological studies done 1 month previously, revealed solid ankylosis of the facet joints between all lumbar vertebrae [].\nAirway was anticipated to be difficult because of the lack of any extension of the cervical spine, fixed cervical flexion resulting in chin on chest deformity leads to difficulty with forward vision and severe cervical kyphosis. Awake intubation with lighted stylet was planned.\nThe patient was evaluated and signed the informed consent to undergo total hip arthroplasty (THA) under posterior lumbar plexus continuous plexus nerve block (CPNB). Prolonged analgesia and potential risks of using both catheters were discussed with the patient and family members.\nOn the day of surgery, the patient was brought to the block room, where intravenous access was secured, and standard monitors were applied. After administration of 100 μg of fentanyl and 0.25 mg of atropine blockade of the bilateral superior laryngeal nerve with 2 mL 2% lidocaine each side and translaryngeal block with 1.5 mL 6% lidocaine (1.5 mL of 2% lidocaine + 1.5 mL 10% lidocaine) was performed. Immediately, after was performed awake tracheal intubation with the aid of a lighted stylet. Induction of anesthesia with propofol, sevoflurane and controlled ventilation.\nThe patient was placed in the right lateral decubitus being conducted continuous blockade of the lumbosacral plexus (psoas compartment and parasacral) with Tuohy continuous nerve block set, with the help of the neurostimulator. The stimulating current delivered was 0.5 mA, with a stimulation of 0.3 ms at 2 Hz. Quadriceps contraction and plantar flexion of the foot were detected in all patients. Injected 40 mL of 2% lidocaine solution with epinephrine plus bupivacaine 0.5% (50:50) in the lumbar plexus, and 20 mL of the same solution in parasacral. The contiplex catheter was advanced to a depth of 5 cm past the needle tip in both plexus. Approximately, 5 mL of contrast (iohexol with 300 mg/mL Ominipaque®) were injected in both catheters to study the dispersion of a local anesthetic [].\nDuring the procedure, 1500 mL of crystalloids and 500 mL of Voluven® (6% hydroxyethyl starch 130/0.4 in 0.9% saline) were administered. The urinary catheter was not used during the procedure. The sensory and motor blocks were adequate, and the patient remained hemodynamically stable intraoperatively. The surgery lasted 2:15 h.\nAt the end of the procedure, the patient was placed in the supine position. He recovered from the general anesthesia and was extubated. Before his discharge, a disposable elastomeric pump (Easypump®, B. Braun, Germany) containing 400 mL of 0.1% bupivacaine was connected to the both catheters. The pump was programmed for infusion at a rate of 10 mL/h, patient was transferred to ward.\nDuring the 40 postoperative hours, boluses were not necessary. Both catheters were removed without intercurrences. After catheter removal, pain was controlled with oral ketoprofen and dypirone. |
A 41-year-old female patient presented with posterior neck pain at our outpatient clinic. The patient had a tingling sensation from the right axilla to the fingertips. She had no special trauma history or other underlying diseases. On physical examination, the spurling test was positive. There was tenderness to the right trapezius muscle with a pain score of 7-points on the numeric rating scale (NRS). The symptom improved after a paravertebral block at the T2 level. She revisited the outpatient clinic 15 months later, with the same symptoms and again received the paravertebral block at the T2 level. Two weeks later, she still had a pain score of 4 to 6-points on NRS. Findings in the cervical spine magnetic resonance imaging (MRI) showed broad central disc protrusion at the C5-6 and C6-7 levels. Two weeks later, cervical Racz neuroplasty was done, and on the next day, after infusion of hypertonic saline through the Racz catheter for 30 minutes, the catheter was removed with no side effects. Two months later, the patient visited the outpatient department with a new symptom. She complained of a tingling sensation on her right arm, without pain in the neck or arm, which subsided after receiving physical therapy and medications. The patient consequently refused further intervention or radiologic examination, and continued to receive physical therapy for neck and arm in the department of rehabilitation medicine.\nThe patient visited the outpatient clinic every two months, due to numbness of the right arm and a pain score of 3 to 8-points on NRS. She was prescribed 225 mg of pregabalin, and received a paravertebral block on the T2 level. She was maintained on physical therapy. Twenty-eight months after the Racz neuroplasty, the patient still had pain in the neck and right arm, and the intensity and duration of the pain was progressive. She received a paravetebral block on T2 and a right brachial plexus block, when the pain score reached 7-point on NRS. However, the pain did not subside; we therefore chose to do a cervical epidural block as the next line of treatment.\nDuring the procedure, we found a foreign body in the cervical epidural space by fluoroscopy (). It resembled a Racz catheter fragment inserted 30 months previously. C-spine computed tomography images were obtained to determine the exact location of the proximal and distal tip of the catheter and whether it was knotted or looped. We confirmed that the tip of the catheter was at the right C6-7 foraminal zone (). We decided that surgical removal was necessary because of the presence of neurologic symptoms. The patient consented, and we consulted a neurosurgeon for surgical removal of the catheter fragment. Two weeks later, it was removed under fluoroscopy in the operating room. After a linear skin incision on the T1-2 level, the paraspinal muscle and interspinous ligament were dissected. The distal sheared tip of the catheter located at a 2 cm depth from skin, was removed. There was no knotting or looping in the removed catheter (). Findings during the operation were not suggestive of any fibrosis or granuloma tissue formation. The patient's symptom subsided immediately after surgery. During the 9 months post-surgical follow-up, the patient had no neurologic symptoms, nor pain in the neck and arm. |
A 34-year-old Polish Caucasian male was admitted to the Dermatology Clinic of the Poznan University of Medical Sciences due to the presence of an erythematous, exfoliating, clearly distinct lesion located on the index finger of the right hand, spreading onto surrounding areas (Figs. , ).\nThe first signs of the disease had appeared in August 2012, after a contact with raw fish—the patient is a cook. Due to a suspicion of erysipeloid, the patient underwent an antibiotic therapy (intravenous clindamycin, 2 × 600 mg daily, for the period of 5 days) coupled with steroids administered topically.\nA series of diagnostic examinations were carried out during his hospitalization. A swab for bacteriological, skin biopsy for histopathological and a sample for mycological examinations were acquired. The bacterial culture was found to be positive for Enterococcus faecalis. The direct mycological examination was negative. Other examinations, such as the mycological culture and histopathology, were awaited for during the stay of the patient at the clinic. Due to the lack of significant improvement, the patient was discharged, while any further procedures were performed on an outpatient basis at the Dermatology Counseling Service of the clinic.\nThe results of the histopathological examination indicated a widened, swollen epidermis with a thickened stratum granulosum and a thick stratum corneum, in which there were numerous, round, fungal spores of 2.5 μm in diameter (Figs. , ). On the other hand, the result of the mycological culture was negative. Due to discrepancies in the results, a second sample for mycological examination was acquired during a follow-up appointment. This time the results of the direct mycological examination were positive.\nItraconazole (daily dose 100 mg) was administered orally. Additionally, an imidazole derivative was administered topically. The patient claimed an improvement in his dermatological condition after 2 weeks.\nThe mycological cultures were maintained for 20 days at 25 °C on two kinds of media: Sabo Sabouraud dextrose agar with chloramphenicol and Sabo Sabouraud dextrose agar with chloramphenicol and actidione. A fungal colony with a reddish brown color and a brownish reverse, strongly furrowed, suede-like surface and a raised center was observed (Fig. ). A microscopic examination revealed numerous cigar-shaped and thin-walled septate macroconidia. Pyriform microconidia were located on short branches along hyphae (Fig. ). A urease test, carried out on Christiansen’s medium, and dermatophyte test medium examination were both positive. Since the strain matched the original description, it was identified as T. rubrum var. raubitschekii [].\nThe following 3 weeks brought a further improvement in the dermatological status (Fig. ). It was decided to continue the itraconazole treatment while the topical drug was switched to a pyridinone derivative. The patient received itraconazole for a period of 2 months, yet due to low tolerance to the topical drug, the pyridinone derivative was no longer administered. Afterward, the patient showed no signs of the infection.\nIn order to reassess the diagnosis, we analyzed a ribosomal RNA genomic fragment [] and the urease gene of this isolate as described by Hiruma et al. []. Because the isolate ceased to grow on any new media, we isolated its DNA from a plate deposited for long-term storage at our clinic. One hundred and twenty milligrams of the colony was acquired from the deposited agar plate and ground in liquid nitrogen. DNA was isolated with the use of the column-based GeneMATRIX Plant & Fungi DNA Purification Kit (EURx, Poland) that includes both RNase A and proteinase K.\nFirstly, we analyzed a fragment containing partial 18S rRNA gene, ITS1, 5.8S rRNA gene, ITS2 and partial 28S rRNA gene with the use of common ITS1 and ITS4 primers for PCR and sequencing. PCR was performed with the following ingredients: 25 pmol of ITS1 and ITS4 primers each [], 0.5 µl of FastStart Taq DNA Polymerase (2.5 U, Roche), 10 µl of 5 × GC-rich solution, 5 µl of 10 × PCR buffer with 20 mM MgCl2, 1 µl of dNTPs (10 mM each), 5 ng of DNA sample, all in a volume of 50 µl. PCR was performed with the following program: pre-denaturation—4 min, 95 °C; amplification—35 × [denaturation: 1 min, 95 °C; annealing: 1 min, 55 °C; elongation: 1 min, 72 °C]; cooling: 1 min, 40 °C. Gel electrophoresis and paired-end sequencing revealed a 692-bp-long fragment, presenting a 100 % coverage with multiple GenBank T. rubrum entries and with our own T. rubrum reference strain. This result was expected as T. rubrum and T. rubrum var. raubitschekii were found to be identical in this genomic region [].\nSecondly, urease gene fragment was amplified by PCR with the following ingredients: 25 pmol of TrURE1S and TrURE1R primers each [], 0.5 µl of FastStart Taq DNA Polymerase (2.5 U, Roche), 10 µl of 5 × GC-rich solution, 5 µl of 10 × PCR buffer with 20 mM MgCl2, 1 µl of dNTPs (10 mM each), 0.5 µl of DMSO, 5 ng of DNA sample, all in a volume of 50 µl. Touchdown PCR was performed with the following program: pre-denaturation—4 min, 95 °C; amplification—35 × [denaturation: 1 min, 95 °C; annealing 1 min, 69–63 °C, step size 0.5 °C, step delay 0; elongation: 1 min, 72 °C]; cooling—1 min, 40 °C. Gel electrophoresis revealed that the product was approximately 330 bp long, suggesting a urease-positive T. rubrum rather than T. rubrum var. raubitschekii (Fig. ). According to Hiruma et al. [], the genomic sequence of a raubitschekii urease amplicon should also include a 68-bp intron and a 9-bp-long coding insertion (DDBJ AB719058, CBS 100084). The PCR product was sequenced from both ends. This analysis revealed a sequence of 329 bp (including primers) presenting a 100 % coverage with XM_003233873 (T. rubrum CBS 118892 urease (TERG_05790) mRNA) and with the corresponding genomic sequence (NW_003456423.1, Gene ID: 10376003). Both the 68-bp intron and the 9-bp insertion, specific to the CBS 100084 raubitschekii strain, were missing. Based on these results, it is more likely that the isolate was, in fact, a urease-positive T. rubrum. |
At the time of the study, Mr Pi (72 years old) was 4 years post-stroke and living with his adult daughter (Meijie) and wife (see ). He was usually in his wheelchair and could stand but was not able to walk. He was dependent on his family for all aspects of his life, from toileting and transfers to meal preparation, although he could eat on his own. His diabetic medication was also managed by his caregivers. His scope of activities was therefore limited and not high in complexity. He also did not like to talk much after his stroke. At first glance, it would seem that he was a silently compliant person, living peacefully at home, taken care of by his family. However, it was clear that he had cognitive deficits. This was reflected in his cognitive screening, where he only managed 10 out of the maximum 30 points [via the Montreal Cognitive Assessment () was administered to him as part of the larger study]. He demonstrated challenging behavioural issues, such as fluctuating moods (resulting in temper tantrums), and occasionally intentionally urinated on the walls of his bedroom, which stressed his family. His behaviour was construed as “naughty” but harmless. Meijie felt that, compared to his previous state where he was completely bedbound and on a feeding tube (for the first month post-stroke), his current condition was a lot better and that he had “stabilised” because he could sit up and could eat well.\nMeijie and Mrs Pi monitored Mr Pi's health in the local government health clinic within the village. Still, they had never discussed any of his behavioural issues with the healthcare provider as they did not think that the issues were related to the stroke. Mr Pi's consultations with his healthcare providers were generally short (regular checking of vital signs, blood pressure and blood tests reviews), and not conducive for in-depth discussions regarding Mr Pi's management at home. Especially for chronic stroke survivors like Mr Pi, it was assumed that the carer could manage and that, if they had any issues, caregivers themselves would bring them up with the doctor. On the other hand, Meijie had no expectations that anyone in the health clinic would be able to help her, nor was she able to explicitly verbalise what aspect she wanted help with. Even when explicitly asked if there she desired any help in the care of her father, Meijie responded, “Can't think of anything. [I] just take care of him like this.”\nMeijie's perception of Mr Pi's health was influenced by his previous (poor health) state, and her expectation of a “healthier” body was based on this lower baseline. Because he had put on weight, could sit up on his own, and communicate his likes and dislikes to a certain degree, he was considered “okay.” His current state of abilities and behavioural issues were normalised as part of who he was. His family had adjusted to his current state without any expectation of further improvements. This normalisation is similar to findings from India, where the high tolerance of impairments and low expectations for older people was borne out in the low reporting rates or health-seeking for memory issues by their family members (). Despite regular follow-ups as part of his health management plan, Mr Pi's behavioural and cognitive issues were not detected by his health providers, because his caregivers did not feel that these issues were significant. There was also a lack of monitoring of his cognitive status. Mr Pi was never administered any formal assessments for his cognitive status throughout his care. |
During anatomic study of the abdominal cavity, additional renal branches from the aortic axis were revealed in a 97-year-old male Caucasian cadaveric donor. Continued dissection outlined notable anatomic variations in the arterial, venous, and ureteric patterns. No urologic or cardiovascular issues were reported by the donor or family at the time of enrolment in the Deeded Body Program. For this type of study, Institutional Review Board approval is not required for research conducted with cadaveric specimens.\nBoth kidneys were retroperitoneal and similar in size with measurements of 12.3 cm (l) and 12.7 cm (r) in the craniocaudal direction. Despite the fact that the lengths were similar, there were marked differences in the relative positioning of the superior and inferior poles. The superior pole of the right kidney was situated more superiorly. The inferior pole of the left kidney was positioned near the superior border of the left common iliac artery. Although each kidney occupied an extended volume, neither kidney had a pelvic component. Hilar structures entered/exited the organs anteriorly, not with the typical medially projected hilum ().\nA total of five major arteries (3l and 2r) emerged from the aortic and common iliac axes (Figures and ). The superior left renal artery originated from the abdominal aorta and supplied the superior pole. This artery (5.2 mm diameter) branched into two vessels of similar caliber to supply the upper third of the organ. Supply to the left gonad originated from the more inferior of these two branches (Figures and ). The middle left renal artery (6.1 mm diameter) originated from the iliac junction, just anterior to the median sacral artery (Figures and ). The inferior left renal artery (5.4 mm diameter) originated from the common iliac artery, and coursed posteriorly to the kidney before entering the hilum anteriorly (from the lateral aspect of the organ), to supply the inferior pole (Figures , , and ). On the contralateral side, the vasculature to the right kidney consisted of only two renal arteries, both originating from the abdominal aorta. The superior renal artery (6.2 mm diameter) branched laterally from the aorta (at the same level as the left superior renal) and supplied the superior pole. As with the contralateral side, this artery also branched into two vessels of similar caliber, but supplied the superior half of the organ (Figures and ). In contrast to the contralateral side, the right gonadal artery branched directly from the aorta at its generally observed position, just inferior to the (superior) renal artery. Arterial supply to the inferior pole (6.5 mm diameter) originated on the lateral aorta, at a level inferior to the inferior mesenteric artery, and branched into two arteries (Figures , , and ).\nMultiple contributing veins, of different caliber, coalesced into four major renal veins (2l and 2r) that returned blood from the kidneys to the IVC (). The relative positioning of the superior renal veins followed the conventional pattern, entering the IVC through single vessels at a level just inferior to the superior mesenteric artery as it emerged from the aorta. On the right side, three major veins merged into a short segment (3 mm in length) to drain the superior 2/3 of the organ. The inferior 1/3 of the organ was drained through two primary contributors that merged into a single vein that ultimately drained into the IVC at the level of the previously described right inferior renal artery. A visible and substantive anastomotic connection was evident between the superior and inferior venous pathways (). On the left side, the superior renal vein received contributions from the suprarenal gland and the superior half of the kidney. Venous return from the left gonad ultimately merged into the most inferior branch of the three primary contributors to the superior renal vein (Figures and ). The inferior renal vein drained directly into the anterior aspect of the IVC at the junction of the common iliac veins, posterior to the bifurcation of the aorta. The most inferior of the three primary contributors to the inferior renal vein emerged from the posterior lateral aspect of kidney and spanned the hilum to ultimately converge into the inferior renal vein. The middle contributor emerged from the parenchyma, but was unremarkable. As on the contralateral side, a visible and substantive venous anastomotic connection was evident between the superior and inferior aspects of the kidney ().\nUnilateral duplication of the kidney with concomitant ureters was evident for the right side (). Aligned with the arterial pattern of this organ, the superior ureter demonstrated a discreet collecting system with well-formed minor and major calyces draining into a defined renal pelvis. The majority of the superior renal pelvis was positioned posterior to the vasculature (Figures and ). At the inferior portion of the kidney, the hilum was more anteriorly directed with the calyces and renal pelvis more evident given their anterior position relative to the vasculature. Both ureters continued inferiorly and independently to enter the bladder (). Histological investigation of the ureterovesical junctions was not completed; however a distinct orifice near the bladder trigone was evident for each ureter. On the contralateral side, the single ureter emerged from a widened and anteriorly projected hilum (). Major calyces from the peripheral parenchyma were evident and these calyces contributed to an elongated renal pelvis. The remaining course of the left ureter was unremarkable. |
A 14-year-old male with continuous left low back pain, suspected of malignancy, referred to our hospital. He had been aware of left low back pain for the last 3 months. A clinical examination revealed spontaneous pain of his left low back and the paresthesia of left S1, 2 lesion. Plain radiograph of the pelvic bone showed osteolytic changes of the left iliac bone (). CT and MRI demonstrated that a 7.2 × 9.5 × 3.0 cm tumor arising from the left iliac bone extended into the left side of sacral bone (Figures and ). The large mass showed low intensity on T1-weighted images and high intensity on T2-weighted images and was enhanced by contrast agent (). A contrast-enhanced CT suggested the existence of the tumor thrombus of IVC (). Open biopsy of the tumor was performed. Histopathologically, the tumor cells were small and round shaped and have abundant glycogen (). Immunostaining analysis showed MIC2 positive and NSE positive. The histopathological diagnosis was Ewing sarcoma. Before treatment, insertion of permanent IVC filter was performed to prevent fatal pulmonary embolism []. We performed irradiation (total 31.2 Gy) and total 4 cycles of chemotherapy (combination of VCR, Act-D, IFM, and ADR) because it was considered to be difficult to resect the mass surgically. After irradiation and a 1 cycle of chemotherapy, the tumor volume was reduced successfully and radiologically evaluated as partial response (PR) by both CT and MRI (Figures and ). Moreover, the tumor thrombus of IVC has completely vanished (). In addition, we performed 3 cycles of chemotherapy. 4 years after the treatment, coin lesion of the left upper lung appeared (). Suspected of lung metastasis, segmental excision of the left upper lung was performed. The histopathological diagnosis was equally Ewing sarcoma. 14 years after the surgery, the patient has been remained free of any evidence of recurrence and tumor thrombus (Figures –). |
A 75-year-old gentleman first presented to primary care with a three month history of left-sided headaches and diplopia. He also complained of altered taste sensation and paraesthesia in the left maxillary region. There was no history of epistaxis. The patient attended his general practitioner on multiple occasions and was given sinusitis treatment until a CT scan was finally performed due to persistence of symptoms, upon which the patient was then referred to the ENT service.\nHe has a background history of fast atrial flutter (on bisoprolol), hypertension, polymyalgia rheumatica, benign prostatic hyperplasia, and previously excised papillary squamous cell carcinoma in the left thigh. He quit smoking 40 years prior to presentation. Otherwise, he had lived independently with WHO performance status of 1 (Karnofsky status 90).\nThe patient underwent complete head and neck examination including flexible nasoendoscopy. There was no discreet neck lymphadenopathy. Nasoendoscopy revealed a mass down to the left inferior turbinate obscuring the left nasal cavity. He complained of diplopia on the left side on the lateral gaze with proptosis of approximately 3 mm compared to the contralateral side. Vision was 6/30 bilaterally with glasses. There was no relative afferent pupillary defect or papilloedema.\nInitial CT scan revealed an aggressive lesion in left ethmoidal and frontal sinuses, invading the left orbit and anterior cranial fossa. CT neck and thorax showed no cervical or chest lymphadenopathy.\nSubsequent MRI imaging also showed evidence of bone erosion with breaching of the dura in the vicinity of the left orbitofrontal cortex although there was no signal change in the brain to suggest brain invasion (). There was destruction of left lamina papyracea. An incidental right anterior cranial fossa meningioma, distant from sinonasal lesion was also identified. This pathologic finding did not have any significant clinical relevance with the primary disease.\nA staged whole body positron-emission tomography (PET) scan showed no other distant lesions but confirmed lesion progression through the frontal sinus.\nOur initial differential diagnoses were squamous cell carcinoma, carcinosarcoma, lymphoma, teratocarcinosarcoma, olfactory neuroblastoma, small cell carcinoma and alveolar rhabdomyosarcoma.\nAfter the diagnostic workup, the patient underwent endoscopic examination of the nose and biopsy of the lesion under general anaesthesia. Extensive disease was noted at the ipsilateral maxillary antrum. Histology revealed an extensive necrotic biphasic epithelioid, spindled malignant neoplasm in keeping with carcinosarcoma. Immunohistochemistry afterwards showed no loss of DNA mismatch repair (MMR) protein expression.\nAfter a successful biopsy and radiological investigative workup, the patient was discharged home. Unfortunately in the community, his preexisting comorbidities worsened, and he developed poorly controlled fast atrial flutter and urosepsis with E. Coli bacteraemia. He was readmitted for antimicrobial and supportive treatment. The patient eventually recovered and was discharged.\nDiscussion was undertaken at the multidisciplinary meeting and with international experts in the field. The consensus is that although the disease can potentially be resected with major surgery such as craniofacial resection, due to its location and size, surgical treatment would have significant risk of bleeding, cerebrospinal fluid (CSF) leak, and meningitis. After detailed consultation and discussion about the possible treatment options including the risks of such procedures with the patient and his family, he was not keen to proceed and opted for palliative radiotherapy and symptomatic management.\nThe radiotherapy dose delivered was 20 Gy in five days, then a two-week break and finally another 20 Gy in five days. One year after diagnosis, patient tolerated the radiotherapy and continued to lead an independent life. |
A 26-year-old female without significant past medical history presented to the Emergency Department complaining of persistent nausea and vomiting, as well as multiple pre-syncopal episodes. Her family also noted that she had been suffering increasing memory loss over the previous few weeks, often not recognizing members of her own family. There was no reported history of substance use. In the emergency department, the patient’s mental status rapidly worsened. She developed expressive aphasia with nonsensical speech, followed by decreased consciousness. She was admitted to the intensive care unit for close neurologic monitoring and further workup. Given concern for viral encephalitis, acyclovir was started empirically.\nVital signs were within normal limits. Neurological exam was significant for expressive aphasia, inability to follow commands, and orientation to self only. Babinski response was present bilaterally. There was intermittent ophthalmoplegia and hyperreflexia was found in both lower and upper extremities. Laboratory studies and urine toxicology were unrevealing of any profound metabolic or toxic disturbance.\nImaging including CT and MRI/MRA of the brain revealed no intracranial or neurovascular lesions ruling out vascular or mass lesions as causes of her encephalopathy. Lumbar puncture and CSF analysis revealed a lymphocytic pleocytosis, but no organisms on gram stain. EEG was significant for mild encephalopathy with no focal lateralizing or epileptiform discharges. HIV, herpes, and syphilis screens were also negative. Acyclovir was discontinued. At this juncture, the possibility of an autoimmune phenomenon was entertained as all objective data were unrevealing.\nIn order to assess for paraneoplastic syndrome, tumor markers, and CSF autoimmune antibody serologies were sent, and the patient underwent CT scan of the chest, abdomen, and pelvis. The latter revealed an 8 cm × 4 cm cystic lesion, suspected to be a teratoma, within the right ovary ().\nShe was started on intravenous immunoglobulin (IVIG) for presumed anti-NMDA receptor encephalitis and experienced minimal improvement in neurologic status by day 2 of therapy. She then underwent successful laparoscopic resection of the ovarian mass. Pathology confirmed a poorly differentiated teratoma (). The patient’s mental status drastically improved by postoperative day 1, and completely normalized by postoperative day 2. She completed a 5-day course of IVIG and was discharged home. Although CSF and serum assays for a wide array of autoimmune antibodies, including anti-N-methyl-d-aspartate receptor (NMDAR), anti-amphiphysin, and anti-voltage-gated potassium channel-complex (VGKC), were negative, given the characteristic neuropsychiatric dysfunction and rapid resolution of symptoms in a young lady with an underlying teratoma, anti-NMDAR encephalitis was the final diagnosis. |
On 12 March 2020 as the first cases of COVID-19 were being reported in Michigan, but prior to general availability of SARS-CoV-2 testing, a 42-year-old male patient developed a mild acute illness characterized by congestion, sore throat, subjective fevers, dry cough and chills along with body aches and fatigue. The patient has been in his usual state of health before developing the symptoms. His only medical history includes multiple episodes of kidney stones. Symptoms improved with acetaminophen and ibuprofen and completely resolved after 5 days.\nEleven days after resolution, on 28 March, the patient developed new symptoms of vertigo, nausea, and vomiting exacerbated by any movement of his head and body along with “pressure” like sensation in the occipital area of his head and mild photophobia. His symptoms acutely worsened throughout that day, resulting in the complete inability of the patient to sit up or to perform any activities of daily living because of severe vertigo and intractable vomiting. Emergency medical services (EMS) were called and the patient was taken to the Emergency Department (ED) for intractable vomiting and dry heaving. The patient denied any speech impairment, limb weakness, or hearing loss. He has never experienced similar symptoms before.\nUpon presentation to the ED, his blood pressure was 130/79 mm Hg, heart rate was 93 beats per minute, respirations were 23 per minute, and oxygen saturation was 100% on room air. He looked to be in severe distress from nausea, dry heaving, non-bilious vomiting, and severe vertigo. Neurologically, his cognition was intact, and the cranial nerve exam was normal. Finger to nose movement and rapid alternating movements of the extremities were intact as well. There was no fluid behind his tympanic membranes, and no oropharyngeal exudates. A leftward beating horizontal nystagmus was noted. Gait could not be assessed as the patient was unable to even lift his head from the pillow. The rest of his neurologic exam was noted as normal. Because of the lack of auditory complaints, the patient did not have a hearing assessment.\nClinical laboratory results obtained in the ED were significant for leukocytosis with lymphopenia of 0.8 bil/L (). Cardiac markers and chemistries were unremarkable. Head computed tomography (CT) and brain CT angiography ruled out possible central vertigo etiologies. As the patient had severe vertigo upon presentation, he could not tolerate any vestibular testing.\nThe patient was treated with IV antiemetics (phenergan 25 mg), IV diazepam (2 mg), and IV fluids with significant improvement in his symptoms, and he was discharged home from the ED within 2 h. Nasopharyngeal SARS-CoV-2 reverse transcription polymerase chain reaction (RT-PCR) was obtained and yielded a negative result. Three days later, due to persistent vertigo, the patient was evaluated by a neurologist. His exam revealed persistent leftward nystagmus and a positive Romberg sign with a tendency toward the right. The patient was diagnosed with right vestibular neuritis. He was prescribed a course of oral prednisone (50 mg daily for 5 days) and meclizine 25 mg every 8 h as needed, with a gradual improvement of his symptoms over the next 2 weeks, resulting in minimal residual vertigo. Two weeks after his ED visit, the patient had testing that detected immunoglobulin G (IgG) specific for SARS-CoV-2. Repeat blood count at the same time demonstrated normalized values. |
A 32-year-old female patient reported with a chief complaint of a swelling on the palate present for 3 years. Her history revealed that the swelling had gradually increased in size since its onset. Clinical examination revealed an ovoid swelling of approximately 4 cm × 3 cm in the left posterior palatal region []. The mass was firm and mucosa over the mass was smooth and erythematous. The swelling was not associated with pain, discharge, or paresthesia. Left submandibular lymph nodes were tender and palpable. No remarkable medical history was revealed except for hypertension. Orthopantomograph showed no evidence of any palatal bone pathology [].\nThus, a provisional diagnosis after clinical and radiographic examination of a minor salivary gland tumor was made. All laboratory investigations were carried out and were found to be within normal limits before the surgical procedure. After taking an informed consent from the patient, surgical removal was done under local anesthesia without complications. A full-thickness mucoperiosteal flap was elevated, and a well-encapsulated mass was noted which easily separated out from the underlying periosteum. It was possible to remove the lesion in-toto and submitted for histopathological examination.\nGrossly, the specimen consisted of a mass of grayish-white tissue. The surface was rough and had a gritty texture when cut []. The tissue was then processed, and the sections were stained with hematoxylin and eosin (H and E) stain and immunohistochemistry using CK19 marker was also performed.\nH and E-stained sections revealed a solid, well-circumscribed lesion composed of a prominent collagenous and hyalinized fibrous connective tissue stroma supporting a proliferation of odontogenic epithelium. Within the stroma, multiple microcystic spaces lined by epithelium exhibiting the typical histologic features of an odontogenic keratocyst (OKC) were present []. The centers of some follicles were packed with layers of desquamated parakeratin []. The cells of the basal layer of the follicular structures were columnar and exhibited hyperchromatism and palisading with reversed polarity and subnuclear vacuolation, producing inductive effect in surrounding stroma [Figures and ] In addition, some ameloblastic follicles, anastomosing and plexiform ribbons and interconnecting cords of odontogenic epithelium were also seen.\nAt the periphery of the tumor, there were collapsed cystic areas containing desquamated parakeratin, pacinian-like stacks of lamellated parakeratin []. Due to rupture of some of these follicles, extruded parakeratin was seen in connective tissue stroma producing a foreign body giant cell reaction [].\nFrom histopathological features, confirmed diagnosis of keratoameloblastoma was given. Immunohistochemistry analysis revealed that many peripheral and central follicular cells showed affinity for CK19 which confirms its odontogenic origin thus supporting the proposed histopathological diagnosis [].\nOn clinical and radiographical correlation, the lesion was finalized to be a peripheral variant of keratoameloblastoma. |
An 18-year-old male patient was admitted to our dermatology outpatient clinic with complaints of swelling, pain, redness in the right half of his lip. The patient who had widespread pustular acne on his face was questioned about his history of medication use, such as isoretinoic acid, that could cause infection by mucosal integrity loss. The patient stated that he did not use any medication. The patient, in whom empirical antibiotherapy and antiviral treatment (amoxicillin, valasiclovir) was initiated by the dermatology doctor, was admitted to the Ear-Nose-Throat outpatient clinic three days after the increase in lip swelling and the onset of systemic complaints such as fever and weakness.\nPhysical examination revealed extremely edematous erythematous lips, more prominent in the right half of the lower lip, and multiple palpable lymphadenopathies (LAPs) in the right cervical chain (). In the evaluation of the oral and mucosal structures, there were no findings other than erythema and swelling on the lips. Initially, laboratory tests were requested to investigate infective causes and the patient was referred for ultrasonography. In ultrasonographic examination, diffuse thickening, more prominent in the right half of the lip, and marked increase in vascularity, as well as a heterogeneous non-locular collection with a dense content compatible with abscess was observed (). Multiple LAPs with thickened cortex were detected in the right cervical chain at levels 1-2-3-4. Linear effusions consistent with edema were observed in the right half of the face and around the lips under the skin. Allergic causes were not expected since the patient’s complaints had onset only three days ago, but immunological tests were also requested. However, when findings in favor of an abscess were described in ultrasonographic examination, the tests were canceled to focus on infection. Culture antibiogram was not taken since empirical antibiotherapy had already been started and the patient was using antibiotics for three days. In laboratory tests, leukocytosis (15.8x10 µL) was detected in neutrophil dominance (80%) with erythrocyte sedimentation rate (ESR: 41 mm/h) and high CRP (7.5 mg/dL). While HSV 1, HSV 2, VZV IgG antibodies were positive, IgM antibodies were negative. The patient stated that he had been picking and peeling his dried lips for a long time. The condition of the patient—who had no known chronic disease—was then evaluated as a primary lip abscess due to exfoliating his lips. Drainage was performed with a syringe, and about 10 cc of pus was evacuated and intravenous antibiotic treatment (sultamicillin), oral antibiotics (amoxicillin) and antiviral therapy (valasiclovir) were continued for two days. Complaints of the patient, who also had spontaneous drainage during follow-up, regressed almost completely within three days and the lip returned to its normal state (). The patient provided his signed informed consent for publishing his data and images. |
A 16-year-old G1P0010 female with recurrent vaginal bleeding presented to the ED with an episode of acute heavy vaginal bleeding. The patient reported soaking through multiple sets of clothes and passing golf ball-sized clots. The patient’s vital signs on presentation showed a blood pressure of 116/70 mmHg, pulse of 100 beats per minute, respiratory rate of 18 breaths per minute, and temperature of 37°C. The patient denied any episodes of lightheadedness, dizziness, or syncope. Pertinent laboratory values on presentation are illustrated in Table .\nPhysical examination revealed mild tenderness along the midline and both the right and left lower quadrants. On pelvic examination, there was active bleeding from the cervical os, which was closed. The patient experienced a significant drop in hemoglobin from her previous ED visit four days prior when her hemoglobin was 10.2 g/dL. This was her third visit to the ED in one month with similar complaints. The patient had a suction dilation and curettage (D&C) for voluntary termination of pregnancy two months prior and a repeat suction D&C one month prior for retained products of conception. Since discharge, the patient had reported continued mild-to-heavy vaginal bleeding with dark and bright red blood. At her last ED visit, she was admitted for evaluation. At that time, even though uterine AVM was discussed, repeat evaluation of the imaging was determined to be low risk and she was discharged home.\nTransvaginal ultrasonography (TVUS) was obtained which revealed a slightly thickened endometrium with increased vascularity and a hypoechoic, hypervascular focus in the endometrium. This led to a concern for retained products of conception or an AVM secondary to prior uterine surgeries (Figure ).\nMRA was obtained and revealed abnormal vascularity suggestive of an AVM within the endometrial cavity at the junction between the body and the fundus of the uterus. Furthermore, a pelvic MRI was obtained which demonstrated an enhancing 8 × 4 mm polypoid structure within the endometrial cavity (Figure ). These findings were suggestive of an AVM or post-traumatic changes from the patient’s prior uterine surgeries. IR was consulted for a diagnostic angiogram to confirm the suspicion of a uterine AVM. Prior to the patient’s diagnostic angiogram, she experienced an episode of vaginal bleeding with the passage of a medium-sized clot and her hemoglobin dropped to 5.9 g/dL. She was transfused with two units of packed red blood cells prior to the procedure. Transfemoral angiography was performed in the IR suite where pelvic angiography demonstrated an abnormal blush in the pelvis that corresponded with the location of the AVM (Figure ).\nSelective angiography of the left uterine artery confirmed an abnormal hypervascular nidus in the uterus which then gave rise to an early draining vein which drained into the right internal iliac vein (Figure ).\nUsing the coaxial catheter technique, a microcatheter was advanced into the arcuate branch of the uterine artery supplying the AVM. Initial embolization was performed with a small volume of 900-1,200 µ Embosphere particles (Merit Medical, South Jordan, UT); however, given the brisk flow through the shunt, it was clear this would not be an optimal strategy. Therefore, n-butyl cyanoacrylate liquid embolic was also not used. The branch was embolized with detachable microcoils. Follow-up angiography confirmed occlusion with no further supply to the AVM via the left uterine artery.\nAngiography of the right internal iliac artery was performed, which showed persistent flow to the AVM from the right uterine artery. Selecting the right uterine artery was extremely difficult, and distal selection was not possible. Hence, embolization of the right uterine artery was performed with gel foam. The final pelvic angiography showed good perfusion of the uterine body and fundus as well as occlusion of the AVM (Figure ).\nThe patient was re-evaluated the next morning after her procedure. She denied any vaginal bleeding or pain and did not report any post-procedure complications. Her hemoglobin was found to be stable at 9.3 g/dL upon discharge from the hospital. |
A 39-year-old white woman presented to the Department of Otorhinolaryngology-Head and Neck surgery, Medical University of Innsbruck, with an 18-month history of nasal obstruction. One year ago, an ambulatory ear, nose and throat examination and tissue biopsy had revealed a chondroma of the left nasal cavity. Surgical resection at that time was not possible because of gravidity. The patient was now presenting to our clinic because of a worsening of symptoms during the last few weeks.\nHer medical history included Maffucci syndrome, diagnosed in early childhood. Relapsing hemangiomas, especially in the metacarpophalangeal joints of the right hand and chondromas in the left lower extremity had necessitated prior surgical interventions (Figure ).\nThe initial endoscopic evaluation revealed a large mass in the left nasal cavity with no signs of inflammation or destruction of surrounding tissue.\nComputed tomography confirmed the clinical findings and showed a tumorous mass of diameter 3.7 × 3.2 × 2.6 cm affecting the nasal cavity, the posterior naris, the base of the sphenoid sinus and the lateral wall of the maxillary sinus (Figure ). Magnetic resonance imaging of her skull revealed an infiltration of the nasal septum and the base of the sphenoid sinus (Figure ).\nA complete surgical resection of the tumor was decided. The tumor was resected via a transnasal endoscopic approach with the help of computer-assisted navigation. During surgery, the smooth and lobulated tumor was found to have infiltrated the nasal septum and the anterior wall of the sphenoid sinus. Consequently the posterior part of the nasal septum and the anterior wall of the sphenoid sinus had to be removed, resulting in a permanent connection of both nasal cavities. The tumor was affecting the left maxillary sinus but without infiltration of the surrounding bone, so it could easily be removed. All surrounding bony structures were smoothed with a sinus drill device. Frozen sections were taken from the margins and confirmed complete tumor resection.\nPossible surgical complications could be divided into intracranial, orbital and local complications. Local complications include anosmia, bleeding and septal abscess. Intracranial complications include rhinoliquorrhoea leading to meningitis, encephalitis or intracranial abscesses. Possible orbital complications include double vision resulting from motility dysfunction, loss of vision, and orbital infections or intraorbital hematoma. Postoperatively, none of these complications was noted.\nA histological workup revealed a mesenchymal tumor with a few enlarged chondrocytes with irregular and hypochromatic nuclei within a hyaline cartilaginous matrix. The cell density was increased but mitotic figures were rare and had a bland histological appearance so that the tumor was classified as a conventional low-grade chrondrosarcoma (Figure ).\nBecause of the complete surgical resection of the chondrosarcoma, no additional radiotherapy was necessary. The interdisciplinary head and neck tumor board decided on a clinical follow-up. Because of the recurrent hemangiomas, therapy with propranolol (Inderal; Astra Zeneca Inc., Vienna, Austria) was initiated.\nChondrosarcomas are the third most common type of primary bone tumors (after osteosarcoma and myeloma) []. Only 10% are found in the head and neck region []. According to our histological findings the majority of chondrosarcomas of the skull base are conventional chondrosarcomas with good or moderate differentiation. Other rare types that present at the skull base are poor differentiated mesenchymal chondrosarcomas or extraskeletal myxoid chondrosarcoma [].\nThe mean age at diagnosis varies between 30 and 60 years []. Presenting symptoms are nasal mass and obstruction and depend on the involvement of adjacent structures []. The main affected sites are the sphenoid and maxillary sinus []. Overall survival after 5 years is estimated at 80.7% [], depending on size, localization and grade []. The metastasis rate is higher in primary and dedifferentiated chondrosarcomas than in secondary ones [].\nSurgical resection is the most effective therapy. The resection margins should be determined in relation to the grade of differentiation of the tumor. While wide bloc excision is recommended for high-grade chondrosarcomas, local curettage is sufficient for low-grade tumors. Because of the low percentage of dividing cells, poor vascularization and the production of extracellular matrix, chondrosarcomas are relatively radio- and chemotherapy resistant. Radiotherapy is feasible after incomplete resection or whenever resection is not possible, to achieve local control [].\nPatients with Maffucci syndrome have a 25% to 30% risk of developing chondrosarcomas and are usually younger than those with primary chondrosarcoma []. The chondrosarcomas usually present at the upper and lower extremities. An involvement of the head and neck region in Maffucci syndrome is exceedingly rare []. Enchondromas of the axial skeleton have a higher risk of malignant transformation than those found in the smaller bones of the extremities. Enchondromas in patients with Maffucci syndrome have a more locally aggressive growth pattern, so that the differential diagnosis towards a low-grade chondrosarcoma is even more difficult than usual [].\nRandom, periodically performed X-ray examinations give little additional information on malignant transformation and are considered useless. Changes in the clinical symptoms should be an indication for surgical intervention rather than radiological findings []. The clinical symptoms our patient presented with determined the immediate surgical intervention in this case. Additional therapy was not necessary because of the low grade of the lesion, for which regional curettage is sufficient []. |
A 80-year-old female with hypertension, diabetes mellitus, was referred to our hospital for fever, metrorrhagia and rest pain in right leg. Medical history reported uterine cancer and gallbladder cancer treated many years before with hysterectomy and cholecystectomy, . Five years before, a right superficial femoral artery stenting was performed for rest pain. No coronary artery disease. At admission, her physical examination revealed atrial fibrillation, blood pressure of 150/70 mmHg, fever (37.5 °C), respiratory rate of 20 breaths/min and oxygen saturation of 95%, abdomen's soft and non-tender with inflammation in genital region caused by Candida albicans infection with Vescicovaginal Fistula complicated by urinary tract infection. Vascular examination showed lack of distal pulses with cyanosis of right foot. Duplex ultrasound (DUS) showed narrowing residual lumen and monophasic wave on both iliac-femoral axes with occlusion of stent in SFA.\nThe subsequent CT-angiography showed any significative alteration in the thoracic and abdominal aorta, but in iliac-femoral axes were confirmed an important atherosclerotic disease with significative stenosis on both common and occlusion of right external iliac artery; furthermore, right superficial femoral artery showed an occlusion of stent with its proximal tract involving the origin of right deep femoral artery (DFA), determining an obstacle to direct flow (). After a gynaecological and urological consult that declared as not urgent the problems previously highlighted, the reduced blood flow in the right leg and foot was identified as the main cause of right leg clinical conditions. A simultaneous multilevel hybrid treatment was proposed to address simultaneously the MPAD. After spinal anaesthesia, the hybrid approach consisted of a first step with common femoral endoarterectomy and dacron patch angioplasty prior section of stent involving the origin of DFA. The iliac axis stenosis was also addressed by placing a 7 × 70 mm Silene (InSitu Technologies, USA - Minnesota) in the common iliac artery with extension in external iliac artery with 7 × 150 mm Viabahn Stent graft (WL Gore and Associates, Flagstaff, AZ, USA). After intervention, a direct blood flow was registered in the right DFA. On second post-operative day, the patient was asymptomatic for pain with normalization of cyanosis. The patient was discharged after a week with double antiplatelet therapy and advanced inguinal medication to avoid infectious complications.\nAfter two months of the index procedure, the patient was readmitted due to inguinal wound dehiscence with a purulent secretion through a fistulous channel and signs of sepsis. The patient had missed every check and urological problems were progressed with urine output on the inguinal wound, despite the presence of bladder catheter.\nThe control CT showed an occlusion of iliac stent with gas bubbles inside the stent graft and peri-iliac soft tissue attenuation that suggested a graft infection (, ). Moreover, there was a suspect of disconnection between patch and arterial wall with dislocation outside of arterial wall in final tract of viabahn stent graft (). Antibiotic treatment was initiated with intravenous meropenem and vancomycin.\nLaboratory data were as follows: White blood cell (WBC) 37,000/μL (neutrophils: 95%), C-reactive protein (CPR) 131.5 mg/dl, pro calcitonin 11.6 μg/dl, Hemoglobin (Hb) 8.9 g/dl, Haematocrit (Hct) 27.3%.\nThe patient was transferred to the operating room, under general anaesthesia, long midline laparotomy was performed. After peeling off the intraperitoneal adhesion, right common iliac artery was controlled (). In abdomen was present ascites. The stent-graft was completely removed with a traction after a surgical access on common iliac artery and common femoral artery where patch was partially disconnected from arterial wall (, ). A procedure of revascalarization was not taken into account for high risk of reinfection and the lack of arterial wall suitable for a vascular anastomosis. Intravenous meropenem and vancomycin were continued for 30 days after surgery, followed by a further 15 days of oral levofloxacin. The cultures from the expulsion site and the explanted stent-graft demonstrated Staphylococcus aureus sensitive to the meropenem; thus, the antibiotic regime was continued throughout hospitalization.\nAt 24 h from index operation, an ischemic deterioration of right leg was present and a major amputation was needed.\nAfter amputation Clinical and laboratory findings were suggestive for sepsis regression: White blood cell (WBC) 20,000/μL (neutrophils: 81%), C-reactive protein (CPR) 108.5 mg/dl, procalcitonin 8 μg/dl, Hemoglobin (Hb) 8.5 g/dl, Haematocrit (Hct) 26%.\nThe patient was discharged home after 3 weeks with three per day amoxicillin/clavulanic acid 875/125 mg and daily wound dressing. After two weeks, the surgical wound was closed. |
A 48-year-old man with end stage renal disease (ESRD), on regular thrice-a-week hemodialysis for 4 years, noticed that his AVF for dialysis vascular access had stopped working and reported to the hospital.\nHe was on regular treatment for hypertension for 17 years before he was detected as having deranged renal function and shrunken kidneys in 2009. The etiology of his kidney disease was unknown, he was not a diabetic. He reached ESRD in May 2010 and was initiated on hemodialysis. He had no history of coronary artery disease, cerebrovascular accidents, peripheral vascular disease, or any other thrombotic events. He did not smoke. The side to side radio-cephalic AVF on the right side was created in 2010, following primary failure of left radio-cephalic AVF. He received hemodialysis thrice a week for 4 h each; the AVF was cannulated by the step ladder technique with the proximal needle being placed more than 5 cm from the AVF anastomosis site. The AVF supported a blood flow of 300-350 ml/min consistently over the past 3 years. He was taking clopidogrel 75 mg/day for the past 3 years.\nOne year back, on a day when he was due for hemodialysis, he noticed the absence of thrill over the AVF and he reported to the surgeon who created the AVF, in another city. The AVF apparently started working without any specific intervention at that point of time. No further evaluation was undertaken to assess the AVF.\nHe had undergone hemodialysis uneventfully, the day before he presented to us. The dialysis session got over by 10:00 PM and the fistula bands were removed by 1:00 AM. He did not notice any abnormality with the AVF the next day morning, when he woke up from sleep. At about 4:00 PM in the evening, he noticed that the AVF appeared “collapsed” and on palpation found the thrill to be absent. He reported to our hospital at 6:30 PM. He denied any history of trauma or prolonged pressure over the AVF, he had mild pain, and noticed a small swelling over the AVF anastomosis site. On examination, there was no bruit over the AVF. Ultrasonography Doppler revealed a thrombus at the anastomotic site with no blood flow. The rest of the AVF was free of thrombus.\nAfter discussing the available and feasible treatment options with the patient, thrombolysis of the blocked AVF was attempted. Written informed consent was taken after explaining the procedure and the possible benefits and risks involved.\nAn 18G intravenous cannula was placed in the AVF, with the tip of the cannula reaching the thrombus at the anastomotic site []. Flushing was done with 20 ml sterile normal saline to ensure that there was no counter-puncture and resultant extravasation of the thrombolytic agent. Tissue plasminogen activator (tPA - Injection Actilyse, Boehringer Ingelheim India) was reconstituted to a concentration of 0.5 mg/ml. A tourniquet was placed proximal to the IV cannula to prevent the injected tPA from reaching the systemic circulation. A volume of 4 ml (2 mg) of the reconstituted tPA was injected through the IV cannula into the AVF segment, close to the thrombus. Manual manipulation of the anastomotic site where the thrombus was lodged was carried out, during and following injection of tPA, to soften the thrombus. Vascular thrill appeared at the anastomotic site within a couple of minutes. Since the thrill remained feeble, another injection of 4 ml (2 mg) tPA was administered, along with manual manipulation, 5 min after the first injection. A prominent thrill reappeared promptly, and the AVF venous segment demonstrated evidence of good blood flow. The tourniquet was removed after 30 min. The patient was advised limb exercise with a soft ball, and he was started on infusion of unfractionated heparin and tablet aspirin.\nAn angiogram of the right upper limb (radial artery and the AVF) was performed the next day morning by the cardiologist and showed excellent flow across the AVF anastomotic site with no evidence of stenosis at the anastomotic site or in the venous system, with small thrombus at AVF []. He underwent hemodialysis the same day afternoon, with the AVF supporting blood flow >300 ml/min. He was treated with anticoagulation with heparin for 5 days and discharged with advice to continue aspirin and clopidogrel. |
A 51-year-old Asian man with alcoholic liver cirrhosis presented to our emergency department initially with upper gastrointestinal bleeding. Esophagogastroduodenoscopy revealed a mass lesion over the upper middle segment of his esophagus []. An esophageal biopsy specimen revealed esophageal squamous cell carcinoma, poorly differentiated type (cT2N2M1; PD-L1:10%). Contrast-enhanced computed tomography of the chest revealed mediastinal lymph node metastases and lung metastasis. The patient also had hematuria, and cystoscopy revealed a tumor over the posterior wall of the bladder; biopsy results were consistent with the esophageal biopsy results. Skeletal scintigraphy showed the presence of multiple bone metastases. Due to the patient's underlying condition of decompensated liver cirrhosis, he was treated initially with palliative radiation therapy to the esophageal cancer primary site only.\nOne month later, he presented with blurred vision in his left eye. Ophthalmologic examination revealed visual acuity of 20/320 in the left eye and 20/25 in the right eye. Intraocular pressure was 10 mmHg in the left eye and 8 mmHg in the right eye. Fundus examination of the left eye revealed a submacular large yellowish-white mass. Ultrasonography of the left eye demonstrated a huge choroidal mass with moderate-to-high internal reflectivity at the superior temporal area []. Optical coherence tomography (OCT) examination of the left eye showed a large elevated choroidal mass with shallow subretinal fluid []. Fluorescein angiography (FA) displayed hyperfluorescence in the late venous phase with dye staining in the left eye [].\nOur impression was that the esophageal squamous cell carcinoma metastasized the choroid. Due to multiple metastases, we administered two cycles of chemotherapy with cisplatin and 5-fluorouracil as adjuvant chemotherapy. However, his disease still progressed and his vision did not improve. A fundus examination of the left eye revealed an increase in size of the choroidal mass with inferior retinal detachment []. The patient then received external beam irradiation with 60 Gy over 30 fractions to the left eye for visual restoration. After 6 weeks of treatment, his visual acuity improved from 20/320 to 20/100 in the left eye. Fundus examination revealed marked regression of the tumor when compared with the images taken before initiating treatment [].\nAlthough the choroidal metastatic tumor showed signs of regression, his disease progressed, and metastasis to his whole body including the brain, lung, liver, skin, lymph nodes, bladder, bone, and peritoneum was revealed. He is now being treated with nivolumab and is still alive since his initial diagnosis 13 months ago. |
A 76-year-old woman presented at our outpatient clinic with symptomatic OA of the right knee. At admission her height was measured to be 139.8 cm and her weight was measured to be 46.5 kg. Her past medical history included NF, congenital pseudarthrosis, and combined deformities of the tibia and fibula that resulted in the shortening of the right lower limb. At presentation, her examination revealed a limited active range of motion at the right knee joint (from 0 to 90°), a valgus deformity of 14° using the femoro-tibial angle measurement, severe pain, and a progressively worsening antalgic gait due to the preexisting limb shortening. These deformities, including pseudoarthrosis, are consistent with skeletal deformities previously described in patients diagnosed with NF. She was diagnosed with Grade 4 OA as per the Kellgren-Lawrence (KL) classification based on the results of radiographic imaging and the physical examination (). Her walking distance was limited due to pain. Passive range of motion studies revealed significant hyperextension (−10°) consistent with an increased risk for dislocation. The physical exam also revealed peroneal nerve palsy and café au-lait spots across her entire body, the latter finding indicative of advanced NF. She had previously undergone a bone transplant at the fibula with a graft taken from the tibia approximately 50 years prior; as such, there was only a limited amount of viable bone remaining to support the successful implantation of a prosthesis. As there was not sufficient viable bone stock available to support the ready-made intramedullary stem used in total knee arthroplasty (TKA; ), our initial plan was to perform a bicompartmental knee arthroplasty (BKA). Furthermore, as part of the preoperative procedure at our hospital, we conducted a bone density test. This evaluation revealed a femoral bone density of 0.525 g/cm3, which affirmed our decision to avoid a TKA, as the quality of the available bone stock would unlikely be sufficient to support adequate fixation of the larger implants required in this procedure. Magnetic Resonance Imaging (MRI) was performed to provide maximum visualization of the lesion, given the discrepancies in the anatomical structures and also to evaluate the soft tissue condition (). Although a bicompartmental knee implant was templated, the cartilage on the medial side of the joint was found to be intact on the coronal MRI scan, thereby presenting a contraindication to this procedure (). Additional radiographic studies revealed that the patient had both femoral and tibial insufficiency and a significant reduction in the bone stock available for the positioning of an implant that required a tibial stem. As this patient also exhibited problems with osteogenesis due to NF, all procedures were planned with the goal of limiting the degree of tissue damage. With this in mind, we considered unicompartmental knee arthroplasty (UKA) as a minimally invasive procedure that might be used for the successful amelioration of her OA symptoms. A lateral UKA to fix the valgus deformity was discussed with the patient as a means to provide adequate relief from the pain resulting from the bone-on-bone contact at the lateral side as well as to address the problem of hyperextension. The patient agreed to this course of action. Preoperative planning focused on addressing the issues associated with bone-on-bone contact as well as repair of the valgus deformity with the goal of promoting stability at the knee. The range of motion was verified preoperatively, and femoral and tibial contact points were mapped across the full range of motion to determine the degree of the anterior slope. Unfortunately, the dimensions of the anterior slope precluded the use of an implant of the size that would be desirable given the patient’s tibial dimensions. We were also aware of the fact that the risks of dislocation are increased in NF patients due to characteristic joint hypermobility and problems with soft tissue in areas such as the skin, menisci, and musculature.\nDuring surgery, the surrounding tissue was debrided, and a large fragment of debris was removed from the joint space. Visual inspection of the knee confirmed the diagnosis of isolated lateral OA with intact medial articulation (). A femoral unicompartmental knee system (Physica ZUK; size D) implant and a 29 mm × 50 mm tibial implant (Zimmer®) were inserted. Perioperative radiographs revealed that, although the trial implant was slightly larger than ideal, synchronous soft tissue balance of the knee was confirmed (). Unfortunately, as customized implants were not available, space remained between the femoral implant and the femoral condyle.\nPrior to final fixation, the articulation site was copiously irrigated to facilitate the penetration of cement into the femur and tibia. The final implant was then inserted into small holes that were drilled into the tibia and femur that also facilitated cement adhesion. The cement was molded to fill the gap observed during the trial implantation ().\nPostoperative imaging and physical examination revealed that the anterior tibial slope was repaired and that the valgus deformity was reduced to 5° from the 14° determined preoperatively (). The patient was capable of ambulation within 24 hours after completion of the procedure. However, the patient developed a varus thrust and continued to require epidural analgesia for 48 hours after the procedure. The rehabilitation plan included assisted range of motion and partial weight-bearing walking exercises. The patient exhibited a myopathic gait; this was deemed acceptable given her preexisting musculoskeletal condition. One week after the procedure, the patient continued to ambulate with the assistance of two walking poles and a knee brace. She reported that soreness developed after walking for approximately 20 meters; this was attributed to preoperative muscle stiffness (). The active range of motion extended from 0 to 110°; this represented a significant improvement over her preoperative range of 0 to 90°. Ankle movement was restricted due to preexisting peroneal nerve palsy and mild pain was present after the surgery. The most substantial functional gains from the lateral UKA procedure were the improvements in the knee alignment and the reduction in the valgus deformity. Both of these corrections contributed to significant improvements in patient ambulation.\nTwo weeks after the procedure, the patient experienced a fall that resulted in a stress fracture of the tibial plateau. The lesion was diagnosed as a proximal oblique fracture of the tibia. This was most likely due to a mismatch between the implant and patient-specific mechano-physiology which may have potentially resulted in sagittal overcutting. The tibial UKA component was not displaced (). Three days post-fracture, a surgical procedure involving a locking plate was planned. A distal tibia anterolateral plate (A.L.P.S®) with nine holes was rotated upside down to achieve successful fixation of the fracture. Eight locking cortical screws were inserted; postoperative imaging documented adequate fixation and support (). Given the high risk for dislocations, continuous passive motion therapy was contraindicated. Within five days after the second procedure, the patient reported only minimal pain and was able to undergo successful rehabilitation to improve her range of motion. We note that her participation in the standard rehabilitation program was interrupted due to the ongoing impact of the COVID-19 pandemic. This was unfortunate, as adequate postoperative rehabilitation can be critical for recovery. The patient was provided with a fixed knee brace to stabilize her leg during the first two months after the insertion of the locking plate. As the COVID-19 pandemic was then under adequate control in the region, the patient was able to follow a rehabilitation plan that included both passive and active progressive range of motion exercises, partial weight-bearing walking regimens, and measures designed to manage pain and swelling. The patient was put on a partial weight-bearing walking regimen (30% to start, gradually increased to 100% body weight every week) beginning at one week postoperatively. At the two-month postoperative checkup, the patient was able to walk with a full weight load without assistance (). Four months after the procedure, the patient reported significant improvements with respect to pain and active range of motion. The patient returned to her activities of daily life and did not need a knee brace or supporter (). Radiographic images revealed callus formation at the fracture site, indicative of new bone formation. We planned to follow this patient on a more long-term basis to manage pain and swelling and to maintain the improved range of motion and weight-bearing capability. However, the overall impact of COVID-19 limited the extent to which we could follow this patient after the five-month check-up. Nonetheless, the patient planned to continue with the prescribed rehabilitation plan. The patient presented for a 7-month postoperative checkup and reported some residual pain that she managed effectively with the pain medication regimen that was prescribed. She reported that she was able to walk on most days and carry out day-to-day activities without difficulty. On certain days, she noted some instability at her knee joint which is likely attributable to the postoperative fracture. Further radiographic studies revealed appropriate fracture healing, and her physical exam documented appropriate knee alignment.\nAlthough the patient was capable of ambulation prior to the UKA procedure, she relied on external support and experienced substantial pain due to osteoarthritis and poor knee alignment. The contraindications to a conventional TKA outweighed the possible benefits that might be achieved in this case. A UKA seemed to be a more appropriate solution toward resolving the pain resulting from bone-on-bone contact and the valgus deformity. The UKA procedure is less invasive, maintains the kinematics at the knee, preserves more bone, and facilitates easier revision surgery if this becomes necessary.\nThe decision to perform a UKA was controversial due to the unique complexities associated with this patient. However, these therapeutic modalities resulted in a significant improvement in the patient’s quality of life. This outcome was evaluated quantitatively by the Knee injury and Osteoarthritis Outcome Score (KOOS) which is included in the (). An increase of 8–10 points on the KOOS total score has been clearly established as a clinically significant improvement. Our patient exhibited a 23.2-point increase in KOOS in response to treatment. This finding indicates that the patient experienced significant benefit from the complete treatment plan. Specifically, the patient reported improvements in areas including but not limited to, a reduction in pain, reduced stiffness, improved mobility, and a better quality of life. |
A 55-year-old Caucasian female presented with productive cough, dyspnea, chest pain and weight loss. The patient's medical history was significant for hypertension, hypercholesterolemia, dyslipidemia, chronic renal failure, gastroesophageal reflux disease and chronic anemia. She was a 30 pack-year smoker and her mother was diagnosed with lung cancer. Her physical examination was unremarkable. An initial chest X-ray showed a large subcarinal mass. On computed tomography (CT), a 6.2 × 5 cm tumor was detected in the subcarinal area (Figs and ). A bronchoscopy with transbronchial biopsy was performed, and the resulting pathological analysis was suspicious for malignant cells; however, no specific tumor type was identified. A positron emission tomography scan showed a large hypermetabolic subcarinal lesion with a standard uptake value of 14.3, consistent with malignancy. The differential diagnosis at the time included an infected bronchogenic cyst, esophageal duplication cyst or a malignant subcarinal lymph node. Thoracoscopic surgical resection was planned for diagnostic and therapeutic purposes.\nPrior to surgical resection, esophagoscopy and bronchoscopy documented the absence of any communication of this mass to the esophagus and airway. The resection was completed thoracoscopically, with a five-port approach. One port was made at the mid-axillary line of the eighth intercostal space for the camera, in addition to two anterior and two posterior ports. The dissection of the tumor began at the level of the inferior pulmonary ligament and posterior mediastinum, all the way to the azygous vein. The esophagus was found completely adherent to the tumor, necessitating an esophageal myotomy for en bloc resection. The esophageal mucosa was not violated at any point throughout the procedure. The anterior dissection proceeded at the level of the posterior atrial wall, the inferior and superior pulmonary veins, the main trunk of the pulmonary artery, and the membranous areas of the right and left main stem bronchi. Complete gross resection was observed at the conclusion of the operation. At the end of the procedure, intraoperative endoscopy was performed to evaluate the esophageal wall. Air was insufflated into the esophagus to confirm the absence of any leaking from the mucosa.\nThe specimen consisted of an encapsulated red-brown tissue weighing 57 g, and measuring 5.5 × 4.5 × 5 cm. The mass was largely necrotic and surrounded by fibrous tissue. Some sheets of viable tumor cells were present, and these contained pleomorphic nuclei, vesicular chromatin, and prominent nucleoli. The tumor cells were strongly positive for CAM 5.2, but negative for CK7, CK5/6, CK19, Calretinin, TTF1, CEA, AFP, HCG, PLAP, CD5 and CD56. This excluded any cells of pulmonary, mesothelial or germ cell origin. Thus, thymic carcinoma was determined to be the most fitting diagnosis based on the morphology and staining. The surgical margins were clear of malignant cells.\nThe patient was referred for adjuvant radiation. At the most recent follow-up visit 9 months after surgery, the patient was well with no evidence of recurrence on CT scan. |
A 55 -year-old Syrian gentleman presented with complaints of low back pain of six months duration which gradually increased in severity over weeks. He denied trauma at any point in time. His past history included diabetes mellitus, hypertension, coronary artery disease, and benign prostatic hypertrophy, and he was on regular medication. The pain was more on movement like walking, bending forward, and getting up from a chair; however, he could walk with pain. There was no radiation of pain, motor or sensory complaints in lower limbs, bowel or bladder complaints. He denied fever, joint pain or swelling, neck pain, respiratory or gastrointestinal symptoms. He took medical consultation a few times before for the above complaint and was prescribed analgesics but had only partial relief. A lumbar spine X-ray, which was done six months prior, was reported to be normal. He reported a weight loss of 15 kilograms over the previous four months, which he claims to be intentional.\nThe patient also complained of intermittent pain over the right testis over the last six months. Testicular ultrasound done six months prior reported right-sided epididymitis, and he was prescribed oral antibiotics for two weeks. However, he did not get complete relief, and the pain persisted. He denied contact with animals but gave history of drinking raw camel milk two years prior to the current admission.\nPhysical examination revealed tenderness over the lower lumbar spines. There was no spinal deformity or local swelling over the back, and there was no neurological deficit. Scrotal examination showed tenderness over the right testis with swelling and tenderness over the right epididymis. The patient was afebrile during his hospital stay. Other system examination was unremarkable.\nLaboratory investigations were normal except for mild microcytic hypochromic anemia (Hb 12.7 g/dl) and mildly elevated CRP of 9.1 mg/L (normal range: 0-5) (Table ).\nLumbar spine X-ray was repeated, which showed small lytic foci involving the superior half of the L4 vertebral body with an ill-defined cortex along anterosuperior aspect and mild reduction of L3-L4 disc space (Figure ).\nMRI spine showed L3/4 prevertebral soft tissue edema/fluid with focal erosion along the left anterior superior margin of L4 superior endplate. Spondylo degenerative changes of the lumbar spine with multiple disc lesions were also found (Figure ).\nTesticular ultrasound showed right-sided epididymo-orchitis with funiculitis. Abdominal ultrasound revealed hepatosplenomegaly (spleen 16.9 cm, liver 19.3 cm).\nBased on the chronic nature of the symptoms and multisystem involvement, an infective cause of tuberculosis or brucellosis was considered. The quantiferon test for tuberculosis was negative. Blood culture grew Brucella melitensis. Brucella IgG antibody assay was positive. A probable diagnosis of brucellosis with spondylitis and right-sided epididymo-orchitis was made. Aspiration from the spinal lesion was deferred as there was no significant fluid collection.\nThe patient was treated with streptomycin for two weeks and oral rifampicin and doxycycline for 12 weeks. After two weeks of treatment in the hospital, he showed good clinical improvement, with the severity of back pain decreasing significantly and complete resolution of testicular pain. Repeat blood culture was sterile. He was discharged home after two weeks, and on follow-up in the outpatient clinic after six weeks, he was doing fine. |
An 11-month-old boy born with multiple congenital malformations has never been discharged home. He spent his first three months in neonatal intensive care unit, where he has gone through some interventions, such as Blalock-Taussig procedure for complex cyanotic heart disease, tracheostomy for congenital tracheal stenosis, and gastrostomy to lower bronchoaspiration risk. Since his birth, he required ventilator support and, despite many attempts, he was never weaned. He underwent chest tomography and bronchoscopy, which also diagnosed the left main bronchus stenosis. Besides the heart and airways double lesion, he has other malformations: grade V unilateral vesicoureteral reflux and anal imperforation. He is on antimicrobial prophylaxis and he has a colostomy.\nDaily procedures such as tracheostomy aspirations cause much suffering, which is manifested by intense sweating, severe cyanosis, and sometimes the need for urgent cannula replacement. Several arterial punctures were performed for blood analysis and vein punctures for peripheral or central venous accesses. In addition, he had to be treated twice for bronchial hyperreactivity with intravenous beta-2 agonist and ventilation under muscle paralysis. Another life-threatening event was a hypovolemic shock secondary to severe bleeding following an airway dilatation procedure. There were two more episodes of dilation by bronchoscopy and a cardiac catheterization, totalizing six anesthetic procedures.\nMeanwhile, his ability to interact socially leaves the health care team amazed. Despite an expected developmental delay due to prolonged hospitalization, he is achieving milestones: clapping hands and sending kisses.\nWe do not know how much discussion was held regarding the limits of treatment before the performance of each procedure, such as tracheostomy and the first cardiac surgery. Currently, the problematic issue is whether to submit him to definitive cardiac procedure or initiate palliative care. The heart surgery involves technical difficulties for total correction, and it probably will not make ventilator weaning easier. On the other hand, palliative care holds many possibilities and they have never been discussed. Withdrawing life support was never an issue.\nThe decisions became more difficult in this case because they rely totally on the medical staff. The family has poor emotional bond with the child. Despite the possibility of aid provided by local social service, for transport fees, for example, parents rarely visit him or make phone calls to enquire about the child. |
A 38-year-old male presented to emergency department with complaints of rapidly worsening shortness of breath since 2 days. Further enquiry on history revealed that patient had a painless swelling over the anterior aspect of neck since last 15 years that had rapidly increased in size over the preceding 2 months. The patient also complained of pain and heaviness over the right side of chest. The pain was poorly localized and was nonpleuritic. There was no history of cough, hemoptysis, dysphagia, and fever. There was no other significant past, personal, or occupational history. At the time of presentation, the patient appeared anxious, drowsy, diaphoretic and in severe respiratory distress with visible use of accessory muscles of respiration. A loud stridor was audible. Respiratory rate was 36/min with paradoxical respiratory movements, blood pressure 162/98 mmHg, heart rate 142/min, and oxygen saturation while breathing room air was 76%. On local examination, a large multilobulated, firm swelling was visible and palpable over the midline neck with fullness extending till the sternal notch []. Swelling was immobile and nontender. Two days before presentation, the patient had been evaluated at another center and investigated. Computed tomography (CT) scan of the neck and thorax was performed which revealed [Figure and ] a large heterogeneous mass with areas of extensive central necrosis in relation to the right lobe of thyroid with right paratracheal extension causing >90% long tracheal luminal and significant right main bronchus compression. Fine-needle aspiration cytology (FNAC) from the neck swelling had been performed following which the breathlessness worsened and the patient was referred to our facility. FNAC revealed poorly differentiated anaplastic carcinoma of thyroid.\nIn view of impending respiratory arrest at the time of presentation, endotracheal (ET) intubation was attempted in the emergency room. A 7 mm size ET tube was negotiated with difficulty and the patient was initiated on mechanical ventilation. However, ventilating the patient was very difficult and peak airway pressures were very high. In view of difficulty in ventilation (high FiO2 of 1.0, peak airway pressure of 45–50 cm of H2O, high-level EtCO2 of 55–60 mmHg and persistent hypoxemia), pulmonary medicine referral was sought. Urgent rigid bronchoscopy was planned for securing the airway and airway restoration via airway stent insertion. The patient was shifted to the interventional pulmonology suite on mechanical ventilation. Hemodynamic monitoring was initiated. Dexmedetomidine infusion (0.75 mcg/kg over 10 min followed 0.5 mcg/kg/h) was administered while the rigid bronchoscopy operator was ready with the ventilating tracheobronchoscope. Flexible bronchoscopy through the ET tube was considered but as the patient was in impending respiratory arrest, a direct rigid bronchoscopy was considered the preferable option thereby minimizing any delay and risk of worsening hypoxemia during the flexible bronchoscopy. After administration of intravenous fentanyl (75 mcg) and hydrocortisone (100 mg), ET tube was removed and quick rigid bronchoscopic intubation was performed using the conventional technique without the utilization of a laryngoscope using a size 11 ventilating tracheobronchoscope (Karl Storz, Germany). Ventilation was done through the side ventilation port of the ventilating bronchoscope using 100% oxygen. Jet ventilation was not utilized. During the initial rigid bronchoscope insertion, the patient was breathing spontaneously. Ventilation was performed by the anesthetist using the conventional anesthesia workstation using bag. As soon as the scope was maneuvered beyond the vocal cords, near complete tracheal obstruction due to mucosal infiltration and extrinsic compression was visible starting 2 cm below the vocal cords. The rigid bronchoscope was gently and swiftly negotiated and carinal visualization was obtained. However, right main bronchus was seen to be extrinsically compressed. After carinal visualization, airway pressures reduced, oxygenation improved (SpO2 98%), and CO2 levels reduced. As airway had been temporarily secured, muscle relaxant (intravenous atracurium (30 mg) was administered and oxygenation was continued for 3 min. A thorough suctioning of airway secretions was performed using a therapeutic video bronchoscope (Olympus 1T-180 videobronchoscope, 6.0 mm diameter and 3.0 mm working channel, Olympus Medical Systems Corporation, Tokyo, Japan.) inserted through the rigid bronchoscope. The size 11 scope was removed and subsequent intubation was performed with size 14 rigid tracheobronchoscope. A tracheobronchial covered self-expanding Y metallic stent (dimensions: Tracheal limb - 18 mm × 60 mm, Right bronchial limb - 14 mm × 15 mm, left bronchial limb - 14 mm × 25 mm, Ottomed, Mitra and Co., India) was deployed at the level of lower trachea near carina. Following Y-stent insertion, both the left main and the right main bronchus distal to the stent were patent. As the tracheal limb length was insufficient to completely cover the upper tracheal involvement, a second tracheal (18 mm × 60 mm) covered self-expanding metallic stent was deployed co-axially into the tracheal limb of the Y-stent to cover and expand the upper trachea. Proximal end of tracheal stent was positioned 2 cm below the vocal cord []. The distal end of tracheal stent was overlapped (2 cm) on the proximal end of tracheobronchial Y-stent. Thus, luminal patency was maintained in both the trachea and bilateral main bronchi. No balloon dilatation was required in either of the stents to open them up. Subglottic area was patent.\nPoststent deployment, the patient was ventilated with supraglottic airway device (I-gel) and dexmedetomidine infusion was stopped. Airway pressures came down to 20–24 cm of H2O and EtCO2 became normal in the range of 34–38 mmHg. After the procedure, the patient was maintaining oxygen saturation with FiO2 of 0.3. The patient was shifted to respiratory intensive care unit for observation and I-gel was removed after 4 h when the patient was fully alert, responding to verbal commands with adequate respiratory efforts. Postprocedure X-ray chest showed the correct positioning of both the tracheal and tracheobronchial Y-stent [Figure and ]. The patient was discharged 2 days later and advised for further follow-up in the oncology services. |
A 42-year-old woman with no significant medical history visited her previous doctor complaining of heavy periods and was diagnosed with submucosal fibroids by transvaginal ultrasonography. Magnetic resonance imaging (MRI) at our hospital (A, B, and C) revealed a low signal for the left ovarian tumor on T2-weighted images, consistent with a fibroma or Brenner's tumor. However, a high signal on T1-weighted images and a low signal on fat-suppressed T1-weighted images were suggestive of a main, fatty component. We therefore considered a mature teratoma during differential diagnosis. Mesenteric lipomas are rare and consequently were excluded from the differential diagnosis.\nThe patient was referred to our hospital for surgery. Transvaginal ultrasound showed a 27.1 mm × 34.8 mm tumor (). After preoperative administration of two doses of gonadotropin-releasing hormone agonist, total laparoscopic hysterectomy, left ovarian tumor resection, and right oophorectomy were planned. Four months passed between initial diagnosis and surgery at our hospital.\nThe intraoperative images are shown in . Although a left ovarian tumor was diagnosed preoperatively, the intraoperative findings showed that the tumor-derived from the sigmoid colon mesentery adhered to the uterus's posterior wall and the left ovary. The tumor was removed by detaching it from the surrounding connective tissue. The tumor was covered with a white membrane and contained degenerated adipose tissue. The surgical plan was modified to include total laparoscopic hysterectomy, bilateral oophorectomy, and mesenteric tumor resection. The operation time was 129 minutes with 20 mL blood loss.The 4-cm tumor weighed 20 gm. The pathological findings are shown in A and B. These findings include fatty tissue with degeneration and necrosis with fibrosis and macrophage reaction, consistent with a lipoma.\nSince the sigmoidal mesenteric tumor was composed of adipose tissue, we diagnosed the patient with a sigmoidal mesenteric lipoma. She was discharged from the hospital without any postoperative complications and is currently being treated as an outpatient at our hospital. |
A 74-year-old woman was admitted to the emergency department of our hospital after a closed injury of her left femur due to a fall. Plain X-rays showed a spiral shaft fracture of the proximal third of the femur. The radiographs of the pelvis and the patient's history as well revealed that she was also suffering from bilateral congenital hip disease (Figures and ). The form of dysplasia of the left hip was type B2-low dislocation according to Hartofilakidis classification or type III according to Crowe classification [–]. The right hip lesion was more severe, classified as type C2-high dislocation or type IV according to Hartofilakidis and Crowe classifications, respectively [–]. There was a leg length discrepancy of 3 cm, with the injured leg being the longer one. Before the fracture, the left hip was almost immovable, ankylosed in fixed external rotation. The right hip was free of pain with functional range of movement. The patient had never been treated for the CHD, and she was walking with the use of a cane. She was also able to do the usual activities of daily living without significant difficulty. According to her medical history, she was a smoker and was receiving medication for arterial hypertension and hypothyroidism.\nTaking into consideration the “personality” of the fracture, the patient's needs, and the surgical options, we performed an open reduction and internal fixation (ORIF) using the NCB periprosthetic femur polyaxial locking plate system (Zimmer Biomet, Indiana, USA). The operation was performed two days after the fracture, under spinal anaesthesia. The patient was placed in the right lateral decubitus position, and the middle and proximal thirds of the femur were accessed through a lateral approach. The subtrochanteric area of the femur was exposed with anterior retraction of vastus lateralis after an L-shaped detachment of its origin. The fracture was anatomically reduced, and two lag screws were placed initially. Then, the NCB periprosthetic femur plate was placed as a neutralization plate (). Image intensifier was used for the confirmation of the optimal position of the plate. The patient received the standard scheme of perioperative chemoprophylaxis with teicoplanin as well as pre- and postoperative thromboprophylaxis with the use of bemiparin sodium. Intraoperatively, she was transfused with two units of concentrated red blood cells.\nThe patient was mobilized on the first postoperative day (POD), and she stood upright using crutches, without weight bearing on the fourth POD. The postoperative period was uneventful, and she was discharged on the 6th POD. Because of the leg length discrepancy, she was protected from weight bearing for six weeks when she was reviewed in our outpatient clinic. At that time, the patient was free of pain, and the subsequent X-ray images showed signs of fracture healing (Figures and ). Following that, she was encouraged to start walking with partial weight bearing. The patient is being followed up for ten months now, and she has fully returned to her previous walking status and activities of daily living. |
A 37-year-old woman presented with a 3-month history of exertional dyspnea, cough and one recent episode of hemoptysis. At the time of admission, she showed no hypertension or systemic symptoms. A chest radiograph showed a left hilar mass with a lobulated margin (). The axial and coronal CT scans were obtained with using 16-channel multidetector CT after IV administration of contrast media. A mildly enhancing left hilar mass with an endobronchial protrusion and an extension along the left lingular segmental bronchus was noted (). The lung setting image showed localized emphysema distal to the endobronchial lesion ().\nBronchoscopy revealed a mutilobular, hypervasacular mass obstructing the lumen of the left lingular segmental bronchus (). A careful biopsy was taken from the peripheral portion because the tumor tended to bleed. This tumor was believed to be either an endobronchial bronchogenic carcinoma or another type of hypervascular tumor. The pathologic diagnosis was chronic inflammation with granulation tissue, which was not in accordance with the radiologic findings.\nWe subsequently performed a left upper sleeve lobectomy with dissection of the mediastinal lymph nodes. The patient suffered no hypertensive crisis during or after surgery. The gross examination showed an endobronchially growing solid mass along the bronchial lumen, the so-called toothpaste figure, and this mass measured 7×3 cm in dimension. The remaining lung parenchyma showed no remarkable change (). The mass showed a yellowish brown granular appearance with infiltration into the bronchial wall. The microscopic examination revealed that the tumor consisted of nests of epitheloid cells in an organoid or alveolar pattern, and this was surrounded by a delicate, richly vascular reticulin network, producing the classic 'zellballen' or basket pattern (). The tumor cells showed marked nuclear pleomorphism and up to one or two mitotic figures/10 high power fields. There were associated microscopic necrotic foci. Immunohistochemical staining for chromogranin, which is a marker for neuroendocrine tumors, was strongly positive. There was no metastasis observed in the dissected lymph nodes.\nAfter surgery, a CT evaluation of the neck and abdomen demonstrated no abnormal findings. The biochemical study revealed the following: the urine epinephrine level was 2.3 µg/day (0-20 for the normal range), and the norepinephrine level was 37.3 µg/day (15-80 for the normal range). The final diagnosis was a primary pulmonary paraganglioma with malignant potential. |
A systemically healthy male patient, 17 years old, with a prominent large swelling at the left mandibular ramus area leading to facial asymmetry which had developed over the previous 4 years, had referred to the Istanbul University, Faculty of Dentistry, Department of Oral and Maxillofacial Surgery. The swelling was not painful and no orofacial infection signs were reported. The patient had a history of head trauma due to falling off from a tree at the age of 6.\nOn extraoral examination, a hard, subcutaneous, multinodular, and painless mass of the complete left mandibular ramus was present. Deviation of the mandible to the right during mouth-opening and the protrusion are detected. During intraoral examination, there were no signs of pathology at the left mandible or the mandibular ramus area. With the radiological examination, the panoramic radiograph showed a large lobulated radiodense mass in the left mandibular ramus area and it also appeared to have a retromandibular partition (). These findings were suggestive of a calcified odontogenic tumor affecting the jaw bone requiring a segmental or block resection. For further evaluation of the character and extent of the mass, Cone Beam Computed Tomography (CBCT) was performed. The CBCT scan showed a diffuse enlargement measuring 4.5 × 3.5 × 3 cm in the entire left mandibular ramus extending anteriorly to the mastoid process of the parietal bone (). Because of the actual dysphagia, the facial asymmetry, and the progression of the lesion, it was decided to perform an operative correction to the left mandible. Under general anesthesia with nasoendotracheal intubation, the mandible was approached extraorally. 6 ml of 1/100000 adrenaline containing articaine HCl was injected to control the local hemorrhage. Using a fissure bur and a chisel, the lobular part of the lateral and medial side of the ascending ramus were resected and the mandible was reshaped (Figures and ). Besides this, healing was uneventful. Postoperative follow-up in the 6th month with dental volumetric tomography scans showed no evidence of pathology with an acceptable mandibular contour. In the next 5 years follow-up, there was no recurrence.\nThe patient also had class III skeletal malocclusion. After cephalometric analysis and malocclusion of the skeletal class III of the patient were detected, orthodontic treatment started. Following the initial treatment, the patient's wisdom teeth were extracted. The patient was made ready for orthognathic surgery.\n7 years after the osteoma surgery, orthognathic surgery was performed. The bone at the left mandibular ramus area was fully regenerated and healthy. By performing two separate surgical operations instead of one operation, a possible bad split was avoided. Patient's age became proper for the surgery and the patient went through orthodontic treatment before the surgery. Two separate surgeries were comfortable for the patient. The only disadvantage of the separate surgeries was expensiveness of the treatment process.\nMaxilla Le Fort I osteotomy was performed with 3 mm anterior and 4 mm right, and mandibular bilateral sagittal split osteotomy was performed and 1 mm back and 2 mm left was taken (Figures –). At the end of the first year following the operation, the patient's existing tooth deficiencies were rehabilitated by applying a dental implant (). |
Our patient is a 76-year-old woman with a past medical history of complete infranodal heart block diagnosed in the year 2000 with an electrophysiology study. She underwent the implantation of a dual-chamber pacemaker, and in 2003 she presented with sudden cardiac arrest (in the setting of preserved left ventricular (LV) systolic function) that was attributed to long QT syndrome. She was upgraded to a dual-chamber implantable cardioverter-defibrillator (ICD) at that time. She did well for 12 years, until she presented in late September 2015 with RV lead fracture and total failure to capture. She was lightheaded with activity, and her electrocardiogram (ECG) demonstrated marked bradycardia and complete heart block (. She had a right bundle branch block (RBBB) pattern escape rhythm, and was monitored for 48 hours on telemetry prior to revising her lead as she was hemo-dynamically stable. Also, she had been developing progressive HF symptoms over the past three years, along with a cardiomyopathy. She was diagnosed with right breast cancer in 2012 and was treated with doxorubicin. An echocardiogram in early 2015 demonstrated that her LV ejection fraction had decreased to 35%. It was unclear if her cardiomyopathy may have been related to chemotherapy, or was due to pacing-induced cardiomyopathy.\nGiven this patient’s complete heart block and worsening HF symptoms in the setting of a wide QRS RVP morphology, it was elected to upgrade her pacing system to a biventricular (BiV) ICD (. During the proce-coil was in the middle cardiac vein. Additionally, she had dure, a temporary pacing wire was initially placed from suboptimal coronary venous branches and LV pacing the right femoral vein. It was discovered that her old RV requiring very high outputs (4.5 V at 1 ms), resulting in left phrenic nerve capture. Her options included surgical placement of an epicardial LV lead or attempts at HBP. However, it was unclear whether HBP would help recruit conduction in a patient who had had long-standing infranodal block with no atrioventricular (AV) conduction for the past 15 years. |
The patient was a 29-year-old G1P0 Chinese female with the AB, Rh positive blood type. She had no significant medical history and symptoms. At 22 weeks’ gestational age (GA), the fetal ultrasound showed that the echo of the intestinal canal in the right lower abdomen of the fetal was slightly enhanced, and no obvious abnormality was found in amniocentesis result. At 23+2 weeks’ GA, the fetal ultrasound indicated fetal growth restriction (FGR). Thus, symptomatic support treatment, such as fluid replacement and improvement of placental microcirculation was applied during the next week. The fetal ultrasound at 32 weeks’ GA showed FGR still exist, while there was a 9 × 7 × 9 mm mixed echo area in fetal brain at the junction of the anterior horn and the body of the left lateral ventricle, which might be a hemorrhagic focus. However, the fetal middle cerebral artery peak systolic velocity (MCA-PSV) increased to 1.69 MoM. Due to the MCA-PSV value was higher than 1.5 MoM, which is a recognized predictor of severe fetal anemia,[ we decided to carry out a fetal umbilical cord puncture in order to identify the etiology. The blood type of the fetus was AB, Rh positive. Both the direct antiglobulin test and irregular blood group antibody screening were negative. Since the hemoglobin level of fetal cord blood test was normal and the hemolysis test was negative, fetal anemia was excluded. However, the fetal cord blood test revealed a severe fetal thrombocytopenia (platelet count, 23 × 109/L). Antibodies of HPAs and human leukocyte antigens between mother and fetus were positive, and thus the diagnosis of FAIT was confirmed. The intravenous immunoglobulin (IVIG) therapy was first recommended but the patient refused owing to financial consideration. Then she was treated with dexamethasone acetate tablets (Xianju Company, China) 0.75 mg twice a day until delivery. At 33+3 weeks’ GA, the fetal ultrasound showed that the hemorrhagic focus area increased to 13 x 9 x 12 mm and the MCA-PSV increased to 1.90 MoM. Multi-disciplinary team advised that with the increase of gestational weeks, fetal platelets would be continuous destructed, which might lead to progressive thrombocytopenia and increase the risk of fetal ICH. So, cesarean section was performed at 34+2 weeks’ GA. There were no bleeding spots or ecchymosis on the newborn's skin. The Apgar scores of the newborn was 9 at 1 minute (skin color deducted 1 point), 10 at 5 minutes and 10 at 10 minutes, respectively. The platelet count of the newborn was 70 × 109 /L, and progressively decreased to 12 × 109 /L until the third day after birth. The neonatal cerebral ultrasound on day 5 and day 12 showed that the area of hemorrhage was in the process of absorption. The platelet count of the newborn increased gradually to 59 × 109 /L after one-month postnatal anti-platelet antibody treatment including methylprednisolone sodium succinate (Pfizer Manufacturing Belgium NV) 1.6 mg/kg for 3 days, IVIG (Hualan Biological Engineering Inc, China) 1 g/kg twice and prednisone acetate tablets (Xianju Company, China) 2 mg/kg every 12 hours (gradually reduced to 0.75 mg per day till 1 month). During the postnatal one-year follow-up, the neonate showed normal developmental milestones and had no abnormal signs of neurological symptoms. |
A 63-year-old male with multiple medical problems was referred to the general surgery service for surgical evaluation for high-grade gastric outlet obstruction seen on CT scan. The patient presented with intolerance to oral intake, three days of worsening nausea and nonbloody/nonbilious vomiting, throat fullness, and a feeling of a “brick” in his stomach. He also complained of a ten-pound weight loss over the previous three months. He reported his last bowel movement to be four days prior to presentation and flatus one day prior to presentation.\nThe patient's past medical history was significant for gastroesophageal reflux disease (GERD), insulin-dependent diabetes mellitus, hyperlipidemia, nonischemic cardiomyopathy (ejection fraction of 20%), and coronary artery disease status-post STEMI and stent placement (five years prior to presentation). The patient also had two recent admissions to the medicine service within the previous three months for progressively worsening atypical chest pain (most recently 1 month prior to presentation). Due to the patient's significant cardiac history, he was evaluated and ruled-out for acute coronary syndrome during both admissions and discharged with a diagnosis of GERD on a high dose H2-blocker and proton-pump-inhibitor.\nHe was also referred to the gastroenterology service for further management of his GERD, and it was felt at this time that he may benefit from reflux surgery. However, given the patient's cardiac history, it was decided to continue with maximal medical therapy and to consider reflux surgery if his symptoms did not improve in three-month time. The patient's history was also notable for an episode of gallstone pancreatitis (four years prior to presentation). Of note, during this episode, he underwent an ERCP with needle knife sphincterotomy due to inability to cannulate the common bile duct (CBD) with a sphincterotome. A cholangiogram at that time showed a dilated CBD and intrahepatic ducts with a filling defect within the CBD, with balloon sweeps showing debris but no definitive stones.\nPhysical exam revealed dry mucous membranes, moderate abdominal distention, epigastric fullness, and hypoactive bowel sounds. Although there was abdominal discomfort, frank tenderness was absent. The patient's laboratory values were also unremarkable, with normal complete blood count, basic metabolic panel, liver function tests (except for an albumin of 3.1), and amylase and lipase. Chest X-ray () showed enlargement of the gastric silhouette and abdominal X-ray () showed a dilated and partially fluid filled stomach. A CT of the abdomen and pelvis with oral and IV contrast was then ordered to further evaluate the patient's gastric distension. The study revealed marked gastric distension with large amount of intraluminal gastric contents/bezoar (Figures and ) and a small bowel feces sign in the region of the pylorus and first portion of the proximal duodenum with surrounding inflammatory changes and mucosal wall thickening. There was no passage of oral contrast beyond the stomach. Additionally, a partially calcified 1.6 cm stone within the CBD () with associated 1.6 cm CBD dilation and minimal intrahepatic biliary ductal dilation was also visualized.\nAs a result of these findings, the patient was taken to the operating room for an esophagogastroduodenoscopy (EGD) for aspiration of the gastric contents and biopsies of any potentially obstructing masses. Due to the significant amount of gastric fluid and bezoar encountered, however, visualization proved difficult resulting in a prolonged and tedious aspiration of gastric contents. Eventually, after aspiration of approximately five liters of fluid, a pinpoint pylorus and a stiff, nonpliable, and nondistensible gastric antrum was visualized. The significantly narrowed pylorus could not be entered despite attempts with a pediatric endoscope and balloon dilation. As such the scope was removed after several biopsies of the pylorus and antrum were taken due to concern for carcinoma as the underlying etiology for the patient's gastric outlet obstruction given no obvious findings of ulcers and the stiffened pylorus and antrum. A nasogastric tube was placed for continued gastric decompression.\nThe biopsy results returned negative for dysplasia, malignancy, or Helicobacter pylori. Due to the uncertain etiology of the patient's condition as well as the preoperative finding of choledocholithiasis on abdominal CT, the patient was taken to the operating room after five days of nasogastric decompression for a diagnostic laparoscopy and possible exploratory laparotomy. The diagnostic laparoscopy revealed no evidence of metastases and the procedure was converted to an exploratory laparotomy. Deeper dissection again revealed no evidence of malignancy but, however, did reveal a markedly atrophic gallbladder and a fistula between the body and the first portion and the duodenum. Once the fistula was taken down, numerous gallstones were expressed from within the duodenum at the cholecystoduodenal fistula site. Following removal of the gallstones a cholangiogram was performed and a filling defect was noted in the distal CBD. A balloon Fogarty catheter was used to express a large common bile duct stone and a repeat cholangiogram no longer revealed a filling defect. A T-tube was inserted and a retrogastric-retrocolic gastrojejunostomy was then performed to bypass the patient's high-grade gastric outlet obstruction.\nThe patient's postoperative hospital course was complicated by episodes of fever, hypotension, and slow return to bowel function. He was discharged home on postoperative day thirteen after successfully tolerating an oral diet. On subsequent clinical follow-up visits, the patient was recovering well from his surgery and had regained some of his weight. |
A 63-year old male who presented on the 18th of September 2012 with reduced left eye vision to 6/36 as a result of a 6-month period of retina detachment. Upon fundus examination an inferior macular involving chronic retinal detachment was observed (Fig. a). The patient was scheduled for a left eye vitrectomy and had an uneventful surgery on the 21st of September 2012. On the 1st post operative day his visual acuity was noted to be counting fingers with a reattached retina. At the next visit 1-week post surgery, he complained of pain in the left eye and vision remained limited to counting fingers but improved marginally to 6/60 with pinhole. At a subsequent visit he complained of reduced vision and foreign body sensation. His vision was reduced to hand motion, and he developed hyperemia and keratic precipitates. Posterior segment examination revealed opaque silicone oil. A faint retinal view could be seen. At his next visit few days later, there was severe conjunctival hyperemia, increasing number of anterior chamber cells and hypopyon. Due to this presentation and a heightened index of suspicion for SORE, the patient was scheduled for immediate silicone oil removal. On the 5th of October 2011, the silicone oil was removed. The procedure involved extraction of the silicone oil, multiple irrigation of the vitreous cavity with vancomycin and ceftazidime (in a concentration of 1 mg/0.1 ml and 2 mg/0.1 ml respectively) into a fluid filled eye. After this irrigation of the vitreal cavity, multiple air fluid exchange was performed. Then, 0.1 ml each of vancomycin, ceftazidime and dexamethasone was injected into the vitreous cavity. Intravitreal 10% C3F8 was used as tamponade after which the three sclerostomy sites were securely closed using 8–0 vicryl sutures.\nMicrobiology study: silicone oil and fluid mixture yielded heavy growth of gram-negative bacilli, which was isolated and identified as P. aeruginosa. It was sensitive to ceftazidime and co-trimoxazole, but resistant to amikacin, ciprofloxacin, levofloxacin, and gentamicin.\nThe patient was commenced on oral co-trimoxazole, frequent topical moxifloxacin and dexamethasone.\nThe intraocular inflammation settled completely with this treatment.\nAn uneventful left eye cataract surgery (phacoemulsification with implantation of posterior chamber intraocular lens) was performed on the 18th of December 2012. Post operatively, final vision settled to 6/9 with correction of − 0.50DS/− 3.00DC × 80. The patient’s eye remained quiet and retina attached with no PVR formation or macular edema (Fig. b, c). His fellow eye remains satisfactory (Fig. d).\nA summary of the clinical presentation of SORE is seen in Table . |
Case 2. A 67-year-old man was diagnosed with an ONB (Kadish grade A-B) in 1989. He underwent lateral rhinotomy followed by irradiation and regularly completed follow-up visits at another hospital. At the end of August 2012, or 24 years after the initial tumor, the patient was referred to us for nosebleeds resulting from a mass in his right nasal cavity (). The tumor was biopsied under local anesthesia, and a diagnosis of recurrent ONB was made.\nA coronal CT image showed the mass occupying the right anterior ethmoid sinus without a middle nasal turbinate. A contrast-enhanced axial CT scan showed patchy heterogeneous enhancement within the mass. A T1-weighted axial MRI showed a low-intensity area (), whereas a T2-weighted coronal image showed a slightly high-intensity area (). A contrast enhanced T1-weighted coronal image showed some enhancement within the lesion (). There was no evidence of the tumor having invaded the dura or intracranial space.\nAn operation by EEA was performed. The tumor relapsed 24 years after the first-line treatment (open surgery plus radiation) which made radiotherapy impossible; therefore, surgery was performed with a dural biopsy and duraplasty using fascia lata plus a pedicled nasal septal flap. First, biopsies were performed at 4 sites of about 15 mm surrounding the surgical stump; the safety margin was confirmed with intraoperative rapid diagnosis. The tumor with the mucosa around the base of the tumor was abraded from the mucosa at the base of the nose. The right anterior ethmoidal artery was clipped and cut to control blood flow from the mucosa at the cribriform plate to the dura mater.\nThe mucosa at the cribriform plate was ablated, and the dura at the tumor base was resected (). After confirming that the dura mater was intact by using an intraoperative rapid diagnostic test, one piece of fascia lata was spread inside the dura mater in an underlay (), while another piece was implanted between the dura mater and the bones at the base of the skull. Surgery was completed after a pedicled nasal septal flap was created to cover the dura mater (). Hematoxylin-eosin stains showed a nest-like tumor mass under the mucosa. The tumor cells showed monotonous growth. Based on slight anisokaryosis and the presence of mitotic figures, the tumor was diagnosed as an ONB, Hyams's stage II (). MR imaging () and local findings () 24 months after surgery showed no evidence of tumor recurrence. |
A 76-year-old immunocompetent male called our office and complained about mild fatigue accompanied by a mild diffuse pain in the right side of his face. The patient was advised to come to the office, which he did 3 days later when a characteristic maculopapular rash, respecting the midline, had been established on the right side of his face, in the distribution of the ophthalmic branch of the trigeminal nerve. The patient was thoroughly examined and had only signs of blepharoconjunctivitis with eyelid swelling, presence of vesicular rash in both the upper and lower eyelid, and mild hyperemia of the conjunctiva, without fluorescein or rose Bengal staining. Hutchinson sign was absent, and no other pathological findings of the cornea or signs of intraocular involvement were noticed.\nThirteen months earlier, he had undergone a trabeculectomy procedure on his right eye for advanced glaucoma with inadequate IOP control despite being on the maximum topical medical treatment and acetazolamide 250 mg, three times a day. Based on the advanced stage of the disease with only central island of visual field remaining, a glaucoma filtering surgery was decided. Preoperative IOP was between 23 and 25 mm Hg.\nA trabeculectomy was performed with our standard technique with a square flap and two 10-0 nylon sutures at each corner. MMC 0.2 mg/mL was applied for 2.5 min intraoperatively and watertight closure was achieved. At 12 months postoperatively, the patient had an IOP between 8 and 10 mm Hg on multiple visits with no antiglaucoma medications used, and hence, the trabeculectomy was considered successful. Patient's last follow-up visit was 10 days prior to the herpes zoster infection when the patient was examined and photos were taken (Fig. ). The bleb was diffuse with mild vascularity, no signs of encapsulation and the Seidel test was negative. The bleb was staged according to the IBAGS (Indiana Bleb Appearance Grading Scale) as H2\E1\V2\S0 [], IOP was measured with Goldmann applanation tonometry at 9 mm Hg, and surgery was documented still successful.\nOn current examination, the intraocular pressure was slightly more elevated than usual at 15 mm Hg with only a mild hyperemia noted over the trabeculectomy bleb. The patient was diagnosed with a primary HZO infection and was started on valacyclovir 1,000 mg three times per day, prednisolone acetate 1% three times a day, and topical lubrication. On the third day, the bleb was noted to be flattered more hyperemic, and the IOP was not controlled, measured at 25 mm Hg. The cornea was not involved, and the anterior chamber had no inflammation. At that point, the patient was placed on topical glaucoma medication with b-blocker and carbonic anhydrase inhibitors, and previous treatment was continued. One week after the initial presentation, even though the cutaneous symptoms had improved, the bleb was completely flat and a very high IOP was established besides topical treatment with antiglaucoma drops at 35 mm Hg (Fig. ). At that point, a decision not to try needling with 5-FU injection was made because of the significant scarring and flattening of the bleb.\nSix months after the infection, the patient had a failed bleb and required both topical maximal therapy with antiglaucoma medication and carbonic anhydrase inhibitors per os to control the IOP in his right eye. Secondary to the elevated IOP the patient's visual field deteriorated and the remaining central island constricted significantly. |
A 39-year-old right master-handed gentleman presented approximately 20 hours after sustaining a crush injury to his left non-master hand in a log splitter at home. His past medical history was significant for hypertension.\nThe patient initially was seen at a neighboring institution for evaluation and treatment. The physical exam on transfer revealed bruising of the distal volar forearm just proximal to the wrist without skin laceration. Radial and ulnar pulses were lacking distal to the area of injury. Clinically, the hand was cool, slightly blue, and lacked digital pulp turgor. Plain X-ray revealed pisiform fracture and scapholunate dissociation. The patient underwent emergency angiogram, which revealed abrupt cutoff of radial and ulnar artery flow into the left hand at the level of the distal forearm just proximal to the wrist junction. There was no evidence of extravasation of dye (). Radiologic interpretation was felt to be consistent with ulnar and radial artery spasm. The patient was not felt to be a candidate for tissue plasminogen activator (tPA), given the nature of his injuries. Since the patient showed no improvement on IV Heparin and the hand remained mottled and cool, emergent surgical exploration was recommended and accepted.\nFasciotomies were performed on the volar forearm, the thenar eminence, and the inter-metacarpal regions in conjunction with a carpal tunnel release. Additionally, a Guyon’s canal release also was performed. The median and ulnar nerves were both intact. The ulnar artery was severely bruised and thrombosed over a length of 8 cm, extending from the ulnar aspect of the superficial palmar arch to 2 cm proximal to the volar wrist crease. At that level in the distal forearm, a total avulsion injury of the ulnar artery was evident with the proximal and distal components of the ulnar artery separated by 5 cm (). The intervening gap between the ends of the ulnar artery contained torn and stretched adventitial tissue.\nExploration of the radial volar forearm showed a discrete 0.75 cm area of thrombus within the radial artery about 1.5 cm proximal to the wrist crease. The remainder of the architecture of the radial artery was intact. A radial artery thrombectomy was performed.\nVascular reconstruction of the ulnar artery was performed using an 8 cm interposition saphenous vein graft from the forearm to the superficial palmar arch. The patient was maintained on aspirin post-operatively, and strong Doppler signals were maintained from both the radial and ulnar arteries.\nA post-operative MRI revealed scapholunate ligament disruption and rotary subluxation of the scaphoid (). The lateral view of the wrist demonstrated dorsal tilt of the lunate consistent with a DISI deformity. In addition, a fracture fragment was seen distal to the ulnar styloid process, likely representing an avulsion off the pisiform.\nAt 12 months, the patient underwent a proximal row carpectomy as well as an open procedure using a K-wire to pin a dislocated capitate of the wrist. The patient currently has normal 2-point discrimination in all digits and the ability to pinch with 5 pounds of strength.\nThe patient has some residual intrinsic muscle fibrosis and decreased thenar muscle mass. The thumb-index space is tight, secondary to partial adductor muscle fibrosis, and the patient has difficulty with prehensile activities secondary to this fibrosis. He also has difficulty extending the thumb out of the plane of the palm and thumb abduction secondary to partial fibrosis of thenar muscles; however, he can oppose his thumb pulp to all fingertips. In addition, the patient has returned to work. |
A 33-year-old pregnant woman (G2P1A1) on the 15th week of gestation was admitted to our Emergency Department with a 3-week history of headache and unsteadiness followed by rapid worsening within 5 days characterized by projectile vomiting, confusion, and psychomotor agitation. A brain magnetic resonance imaging (MRI) scan showed tetraventricular hydrocephalus secondary to obstruction of the cerebrospinal fluid through the foramina of Luschka and Magendie caused by a homogenously enhancing cortical-subcortical lesion localized on the right cerebellar hemisphere, responsible for perilesional edema and characterized by evidence of dural infiltration. The latter, initially mistaken for dural tail, oriented toward the suspicion of posterior fossa meningioma (see ).\nGiven the critical clinical and radiological scenario, surgical excision was expedited. The patient successfully underwent a suboccipital craniectomy in a sitting position, with the insertion of external ventricular drain (EVD) through the right Keen's point and gross-total removal of the lesion, which resembled an aggressive glioma rather than a meningioma. The general anesthesia was carefully tuned to avoid any impact on the fetus' wellbeing; the postoperative course was uneventful, the EVD was removed within 1 week, and the patient experienced a full recovery with unremarkable neurological status at the time of discharge from the hospital. The histology and the immunohistochemistry analysis surprisingly gave a final diagnosis of GS (see ); this was confirmed following a second opinion sought from a center of excellence for neuropathology. The consensus from a multidisciplinary team involving gynecologists, neurosurgeons, and oncologists was to have the best interest meeting with the patient and family to decide how to handle the challenges of the adjuvant treatment. Given the patient's decision not to interrupt her pregnancy, no chemotherapy or radiotherapy could be carried out despite the aggressive histology would have warranted them. Following the birth of a healthy baby girl, the patient was transferred to the Radiotherapy Unit to start her first cycle of temozolomide along with conventional radiotherapy. Unfortunately, the almost immediate onset of generalized seizures represented the first flag of disease progression, which led to a sudden and irreversible clinical decline followed by the patient's death within few weeks. She was survived by an inconsolable husband and never managed to see her baby safely discharged from the neonatal intensive care unit. |
A 25-year-old male was admitted for elective aortic and pulmonary valve replacement. On admission, he was found to have a C-reactive protein (CRP) of 102 mg/l with normal white cell count. His past medical history was significant for congenital heart disease with a true bicuspid aortic valve and ascending aortopathy. He was asymptomatic as a child and remained under surveillance for gradual worsening of his aortic stenosis until undergoing balloon dilatation aged 9, followed by a Ross procedure aged 15 for severe stenosis. He developed progressive aortic root dilation and underwent a redo operation with a Bentall procedure using a 29-mm Perimount prosthesis aged 20. He remained asymptomatic over the following years, until recent echocardiographic surveillance showed development of severe aortic stenosis and mixed disease of the right ventricle–pulmonary artery (RV–PA) homograft, prompting admission for elective surgical intervention.\nOn admission the patient reported to feeling well, aside from recent flu-like illness which had resolved a week prior. At that time, he had been feeling feverish with myalgia and a non-productive cough. He denied any localising symptoms and his observations were all within normal limits. His physical examination was normal, barring the expected ejection systolic and early diastolic murmurs, with no peripheral stigmata of endocarditis. On the day of admission, he underwent a computerised tomography (CT) scan of the aorta in preparation for surgery, which included views of the chest and abdomen. Review of this scan revealed no focus of infection, or para-aortic collections. His urine dipstick and respiratory viral PCR screen were both negative.\nWhile awaiting surgery, he began to clinically deteriorate with tachycardia and fevers. Repeated transthoracic echocardiography (TTE) showed no obvious independently mobile structures on the valves, however due to the extent of degenerative changes of the valve it was difficult to fully exclude vegetations (). The patient’s CRP continued to rise over the next 2 days and his blood cultures came back positive for A. aphrophilus.\nHe was started on Ceftriaxone and Gentamycin, however by Day 5 his CRP had climbed to 317 mg/l with a white cell count of 12.8 × 109 g/l and he remained pyrexial. Most significantly, his ECGs showed progressive prolongation of PR interval () from a baseline of 158 to 292 ms. The daily progressive PR interval prolongation was highly suspicious of aortic root abscess formation and triggered urgent further imaging. Transoesophageal echocardiography (TOE) showed a large echo-dense structure around the aortic root extending posteriorly towards the left atrium. Several small cavities were seen in the mass with blood flow and communication between the cavity and the aortic root (, –). There was also localised extension of the abscess border into the left atrium with potential risk of rupture. Repeat CT was carried out which showed an increase in low attenuation material around the aortic root with two new regions of internal contrast extravasation. This was in keeping with aortic root abscess at the level of the aortic prosthesis with extension into the roof of the left atrium ().\nHe was taken urgently to theatre for debridement and redo-Bentall operation with a mechanical aortic valve and replacement of the RV to PA conduit. Explanted tissue did not grow anything at culture, but 16S rDNA PCR screen was positive and seq1 PCR detected A. aphrophilus. He had an uncomplicated recovery post-operatively and was treated with a further 2 weeks of intravenous antibiotics with appropriate normalisation of his CRP. Antibiotic treatment was changed to oral Ciprofloxacin and completed a total of course of 6 weeks. He remained well since stopping antibiotics with no further fevers and normalCRP. |
The patient was a 37-year-old married Japanese woman. She was referred to the Department of Psychiatry at the Toho University Omori Medical Center, Tokyo, to receive care for a severe postpartum depressed mood and intense suicidal ideations. The participants provided written informed consent prior to enrollment in this case report.\nShe had no previously documented psychiatric history and no documented family history of psychiatric or perinatal illness. She had been brought up in an urban environment since childhood. She had a good relationship with her parents and did not experience any abuse or maltreatment. Her character was honest, diligent, and orderly, and she had an especially strong sense of responsibility. After graduating from university, she worked as an assistant curator in a museum. At the age of 34 years, she met and married her husband, who was an engineer. They were not eager for her to become pregnant. Although she and her husband moved to the countryside because of his work, she decided to continue her job because, despite a 3-hour commute, she found her work to be very fulfilling.\nAt the age of 36 years, she became pregnant. She quit her job to become a housewife and became bored with her daily life; she also felt that living in the countryside was inconvenient. Her husband was busy with work and left all the pregnancy preparations to her, which caused her to feel frustrated. She started feeling very anxious about her primiparity and child-care, and she moved to her parents' house to receive their support. During her 39th week of pregnancy, she had a forceps delivery because of a birth canal infection. The delivered boy was 3150 grams and had no deformities of any kind. However, she could not hold her child immediately after childbirth because she was receiving treatment for her infection. She appeared to lack affection towards her baby because she could not remember how she felt when she eventually held her baby for the first time.\nIt was difficult for her to control her baby when he cried, and she felt fatigued and anxious. He was very demanding for his mother's breast milk, and she continued to feed him because of a feeling of responsibility. A month after the delivery, depressive symptoms (depressed mood, abnormal fear, and insomnia) appeared and gradually worsened. She felt a strong sense of distress while she was with her baby and was confused as to how to care for him. She had difficulty asking for help because she felt that she should do everything on her own. She began to regret having given birth because it had led to her present circumstances. Her husband was still unable to provide her with either emotional or physical support, but her mother continued to help her care for her baby. The patient's lack of affection worsened and was accompanied by other depressive symptoms, such as poor concentration, indecisiveness, and reduced energy. The patient began to worsen daily and began to have difficulty taking care of both herself and her baby.\nThree months after the birth, she visited a psychiatric outpatient clinic and was diagnosed as having postpartum depression and bonding disorder. Her Temperament and Character Inventory (TCI) scores were as follows: Novelty Seeking (NS): −1.78; Harm Avoidance (HA): 2.52; Reward Dependence (RD): −2.09; Persistence (PS): 1.78; Self-directedness (SD): −2.1; Cooperativeness (CO): −1.35; and Self-transcendence (ST): −0.54. Her temperament was judged as logical and obsessive-compulsive, and her character was judged as melancholic and schizoid.\nTreatment with an antidepressant (sertraline, 25 mg/day) and psychotherapy was started, but her depressive symptoms worsened. Because of strong nausea, the treatment with sertraline was discontinued and treatment with mirtazapine (15 mg/day) and olanzapine (initial dose, 2.5 mg/day) was started. Finally, she was admitted to the Department of Psychiatry at the Toho University Omori Medical Center.\nOn admission, her Hamilton Rating Scale for Depression (HRSD) score was 35. She was characterized as having a depressive mood most of the day, markedly diminished interest and pleasure, insomnia, psychomotor agitation, loss of energy, feelings of worthlessness, indecisiveness, suicidal ideation, and a suspicious attitude. We diagnosed her as having peripartum-onset major depression, and we gradually increased the dosage of mirtazapine up to 45 mg daily and that of olanzapine up to 10 mg daily, with poor results. Therefore, nortriptyline was prescribed along with these drugs. After the dosage of nortriptyline was increased to 100 mg, her depressed mood and indecisiveness began to improve at around day 45 of her hospitalization. She was allowed to stay overnight at her house, with her child, on day 53. However, her depressed mood and anxiety were suddenly exacerbated when she returned to the hospital. Hence, we started a series of electroconvulsive therapy (ECT) treatments beginning on day 72 and her depressive state improved somewhat.\nShe made comments such as “Even though I should have been happy to have been with my child, it did not go so well emotionally” and “I'm convinced that I'm incurable.” Her score on the Mother to Infant Bonding Scale (MIBS), which is a 10-item self-reported instrument, was 12, indicating a severe bonding disorder []. We focused on the bonding disorder and provided Kangaroo Care during family sessions. We started providing Kangaroo Care with her baby for two hours in a private room of the ward while her husband was present. During the Kangaroo Care session, we facilitated skin-to-skin contact and educated her regarding parenting behavior in cooperation with the ward nurses. Two sessions were provided, and similar follow-up care was subsequently provided by midwives. She learned to recognize her baby's gestures, facial expressions, and emotions. She was transfixed and embarrassed when the baby was crying, and she barely smiled at the baby during the first session. To allow the patient to gain self-confidence, we first had the patient hold her baby while he was in a good mood. We repeatedly showed her how to cope with the baby's discomfort in a concrete manner and told her that her baby was very cute to improve her capacity to verbalize her emotions. Her husband was encouraged to send her photos and movies of her child taken at home every day based on the policy of imaginary exposure. After this intervention, her awareness of her child's feelings and her ability to provide flexible care for her baby improved.\nThe patient began to experience tender feelings towards her child and her anxiety decreased (HRSD = 7). She tried staying at home overnight, and her depression and bonding problem did not worsen. On day 114, she was discharged from the hospital. She was taking maintenance doses of 100 mg of nortriptyline, 30 mg of mirtazapine, and 5 mg of olanzapine per day at the time of her discharge. We continued outpatient treatment and supported her in caring for her child. In addition, we liaised with midwives, public health nurses, and home visit nurses regarding her care and ideal environment after discharge. At 9 months after her discharge, her MIBS score was 5 (). A tendency towards an improvement in her bonding disorder was seen, and her depression remained in remission (HRSD = 6). |
A four-year-old Japanese girl with no remarkable medical history was referred to our orthopedic clinic for treatment of 2 cm of LLD. She had a two-year history of progressive LM in a wide range of the posteromedial aspect of the right thigh and the medial aspect of the right lower leg. At the first presentation, skin lesions exhibited hyperpigmentation, induration, and xerosis. The range of motion of the right knee was full extension to 80° of flexion. Radiographs of the right lower extremity revealed dysplastic/atrophic femur and tibia. LLD increased with time and reached nearly 10 cm at seven years of age (). As she and her parents refused to undergo epiphysiodesis of the unaffected side of the lower extremity, we performed simultaneous lengthening of the right femur and tibia using a unilateral external fixator (EBI/Zimmer Biomet Carbon Rail Deformity System; Warsaw, Indiana, USA). She had taken low-dose prednisolone every day or every second day prior to the first lengthening procedure. The dosage regimen had been dependent on the disease activity based on clinical and thermographic assessment. Tibial osteotomy was performed with the Gigli saw, whereas femoral osteotomy was done with a multiple drilling technique. No postoperative immobilization was used, and full-weight bearing was encouraged from the second postoperative day. After 14 days of the waiting period, distraction of the femur and tibia was commenced at a rate of 1 mm and 0.5 mm per day, respectively. Femur was lengthened at the same rate throughout the distraction period, whereas the distraction speed of the tibia was gradually decreased after the lengthening callus showed thin and sparse on radiographs. Distraction of the tibia was occasionally interrupted until the callus width and continuity were reestablished. As a result, the lengthening period/amount of lengthening of the femur and tibia were 90 days/83 mm and 163 days/37 mm, respectively, and an overall leg length was 7 mm longer in the affected limb at the end of the lengthening period (). During the neutralizing period, an accordion technique and daily low-intensity pulsed ultrasound (LIPUS) exposure were applied to the tibia to stimulate callus maturation. She received LIPUS treatment using a sonic accelerated fracture healing system (SAFHS; Teijin Pharma Ltd., Tokyo, Japan) once a day for 20 minutes without interruption. After 84 days and 194 days of the neutralizing period in the femur and tibia, respectively, the device was loosened to allow dynamization of the lengthened callus so that it could fully mature. The dynamization period reached 49 days in the femur and 58 days in the tibia to obtain matured callus exhibiting fusiform/cylindrical shape and similar density to that of the adjacent cortical bone on radiographs. Before pin removal, we dislodged the fixator frame with the fixation pins leaving in situ for a while to monitor the development of regenerate bone fracture or bending. The monitoring period was 47 days for the tibia and only one day for the femur, because the femoral pins had already been loosened. A healing index (HI) was 29 days/cm and 129 days/cm in the femur and tibia, respectively. Regenerate fracture of the femur, however, occurred due to minor trauma three days after the pin removal (). Since parental consent for open reduction and internal fixation was not obtained, she was treated conservatively with skin traction, resulting in malunion associated with a marked anterolateral bowing.\nAfter the first lengthening procedure, LLD gradually increased again and reached 11 cm at eleven years of age (), when the flexion angle of the right knee decreased to 30 degrees. The second simultaneous lengthening of the femur and tibia was performed through percutaneous osteotomy using a multiple drilling technique. In the femur, acute correction of the bowing was done at the osteotomy site with the use of a fixator. The angulation was corrected up to 25 degrees using a proximal rotational clamp, followed by mechanical realignment of the bone axis using a distal translational clamp. After correction of the angular deformity, the osteotomy site was compressed (). Distraction by 1 mm and 0.5 mm per day was initiated at 14 days postoperatively in the femur and tibia, respectively. During the lengthening period, the rate of distraction was adjusted appropriately in order not to deteriorate the continuity of the callus on radiographs. Since the callus was poorly consolidated in the femur (), a modified “chipping and lengthening technique” was performed to enhance bone regeneration at nine months postoperatively () []. Briefly, both ends of the osteotomy site and the callus were drilled with a 3.0 mm Kirschner wire in advance and then broken into smaller pieces with an osteotome. Subsequently, the comminuted bones were compressed until a radiolucent area was no longer recognized. Hard callus that obliterated the medullary cavity at the ends of the osteotomy site was removed with a sharp spoon. Two weeks after the chipping surgery, the distraction was resumed at a rate of 0.5 mm per day. The lengthening period/amount of the femur and tibia were 435 days/55 mm and 209 days/29 mm, respectively, and an overall leg length was 31 mm shorter in the affected limb at the end of the lengthening period. Symptomatic pin tract infection occasionally occurred during the treatment period and was resolved with oral antibiotics without any sequelae. The HI of the femur and tibia was 182 days/cm and 222 days/cm, respectively. Currently, two or three years have passed since the final removal of the femoral or tibial pins, respectively, and 38 mm of LLD is left with acceptable lower limb alignment (). The range of motion of the right knee is 20° of flexion and 0° of extension, but she can walk independently without a brace or a crutch. She and her parents are satisfied with the outcome despite the long treatment period. |
A 28-year-old man was admitted to the emergency room with complaints of a quadriparesis attack that had occurred 3 hours prior to the visit. There was no facial or respiratory muscle weakness or autonomic symptoms. The patient reported mild muscle pain after exercising the day before the visit and consumption of a lot of bread for dinner. Paralysis of the upper and lower limbs appeared bilaterally and started in distal areas such as hands and feet and progressed to the proximal area. The patient reported no paresthesia or hypoesthesia.\nHe had no respiratory or swallowing difficulties, he denied facial muscle weakness, and conversation was possible. He did not report any medications or herbal supplements, and there was no history of thyroid disease. The patient reported no underlying disease and no history of arrhythmia, chest pain, or dyspnea. In addition, the patient mentioned that he had experienced intermittent episodes of weakness in the lower extremities after exercising since his early twenties, although the symptoms were temporary and improved with time. There was no similar episode or specific disease history in the patient's family ().\nOn physical examination, the patient's blood pressure was 134/78 and the heart rate was 84 beats/min. His body mass index was 19.96, indicating normal weight. No jugular venous distension, goiter, or lymphadenopathy was observed. On auscultation, a regular heartbeat was noted, and there was no murmur. There were no specific findings on chest and abdominal examinations. There were no deformities or edema of the extremities, and the distal pulses were present and bilaterally equal.\nNeurological examination revealed motor grade 1 and flaccid paralysis of all extremities. There were no specific findings in the sensory test. His blood sugar test indicated hypoglycemia or hyperglycemia.\nInitial electrocardiogram(ECG) revealed a heart rate of 78, a flat T wave, and QT prolongation (QT/QTc 542/621 ms) (). The results of the blood test conducted at the visit were available after 1 hour and 30 minutes and showed potassium at 1.50mmol/L (3.5-5.0mmol/L), while arterial blood gas analysis indicated metabolic acidosis. Although intravenous potassium infusion was started immediately, his blood pressure temporarily decreased to 80/50 and his ECG showed bradycardia, ST depression, and a U wave (). The patient complained of chest discomfort and dyspnea. At that time, the result for cardiac enzyme was in the normal range, and the serum potassium level was 1.35mmol/L (3.5-5.0mmol/L). The potassium injection rate was increased accordingly, and oxygen was supplied. After 20 minutes, his blood pressure reached 110/60 and the pulse rate recovered to 67/min, but chest discomfort continued. Intravenous potassium infusion was continued, and the patient was admitted to the intensive care unit for closer observation. In the follow-up ECG after 12 hours, he had a normal sinus rhythm, and the QRS prolongation resolved (). The muscle paralysis gradually improved starting from 4 h after his initial hospital visit. In follow-up ECG, a normal sinus rhythm was noted. After 24 h, the muscle weakness completely resolved, and the serum potassium level increased to 3.4mmol/L.\nA test was performed to evaluate the cause of hypokalemia. The patient's urine sodium and potassium as well as serum aldosterone and renin levels were measured to eliminate adrenal involvement, and the values were normal. Thyroid function test results were in the normal range. After normalization of potassium level, his potassium remained in the normal range without infusion, and he recovered without any neurological deficits. There were no specific findings in the subsequent blood tests.\nA long exercise test was performed to differentiate between inherited neuromuscular disease, but no findings were indicative of hypoPP. However, the patient had previously experienced an episode of mild muscle weakness, and he reported at this visit that his symptoms had occurred after a break from exercising and with a high carbohydrate diet; therefore, hypoPP associated with genetic disease was suspected, and next-generation sequencing (NGS) with a related gene panel for neuromuscular genetic disease was performed. An Arg669His mutation of SCN4A was noted in the NGS results, and the condition was diagnosed as hypoPP ().\nThe patient was educated about aggravation factors that could help prevent symptoms, and potassium tablets were provided for use when muscle weakness occurred. The patient is undergoing regular follow-up and has been in a stable state without any paralysis attacks for more than 6 months. |
A Folliculotropic Mycosis Fungoides was diagnosed in a 58-year-old male patient in 1997 and treated with local chlormethine between 1998 and 2006.\nIn 2006, MF progressed toward a tumoral form with infiltrating plaques and nodules all over his body, the most important being an exophytic one arising from the nasal region. No Sezary cell was noted in the blood smear. A biopsy of cutaneous tumor was performed and the pathologist confirmed a localization of tumoral nontransformed MF.\nBetween 2006 and 2014 the patient received several systemic treatment lines including methotrexate, PUVA therapy, pegylated liposomal doxorubicin, polychemotherapy, histone deacetylase inhibitors, and anti-CCR4 monoclonal antibody. All these drugs were without long-lasting effect and tumoral lesions progressed including the tumoral lesion of the nose (Figures and ).\nThe extent, progression, and resistance of his skin lesions gave a palliative intent to his treatment project. The patient reported that the aspect of his nose refrained him from interacting with people, which led him progressively to get socially isolated. He reported difficulties in interacting with his family members especially with his young grandchildren. Histology from the nasal lesion was obtained and showed classical Mycosis Fungoides of granulomatous type without transformation.\nHe was then referred to our radiotherapy unit in August 2014. We opted for a conventional radiotherapy with 12 MeV electrons and 6 MV and 18 MV photons. The patient received 36 Gy in 18 fractions (2 Gy per fraction, 5 fractions per week).\nLesions disappeared completely within a few weeks (Figures and ). The patient presented acute grade I radiodermatitis (NCI CTCAE Version 4.03) which resolved spontaneously. No clinical relapse had been noted 3 years after the treatment.\nAs the physical appearance of the irradiated nose got better the patient reported a psychological relief. The recovery of the normal aspect of his nose helped him resume some of his social activities, use public transportation, and better interact with friends and family members. |
A 9-year-old boy accompanied by his father and paternal uncle presented to pediatrics outpatient department of Pt. B. D. Sharma PGIMS Rohtak with complaints of fit-like episodes for last 3 years and hematemesis for 1 year. The general physical examination of the child was absolutely normal. Routine investigations complete hemogram, bleeding and clotting time, prothrombin time, partial thromboplastin time, INR were done and found to be within normal range. Earlier treatment records showed that the child was already taking anti-epileptic treatment, but still there were fit-like episodes. The child was admitted in pediatrics ward. Upper GI endoscopy, fiberoptic laryngoscopy, and bronchoscopy revealed no abnormality. CT scan head and EEG brain were also normal. The previous records showed multiple admissions of the child in the past for similar complaints. The child had undergone many invasive investigations earlier from different hospitals. Father and paternal uncle used to stay with the child and showed over concern about the child's illness and repeatedly requested for more investigations. The child had 2-3 fit-like episodes in the ward in front of treating team, which were not true seizures. The child also had repeated episodes of hematemesis in the ward, and each time, father collected the sample in the bottle to show to the treating doctors. The sample collected did not clot for 2-3 days, which aroused suspicion, and was sent for microscopic examination. It was found that there were no RBCs in the sample. The sample contained only salivary secretions mixed with some reddish-brown chemical.\nThe child was referred to psychiatry department and admitted there. The child was separated from father and paternal uncle, and mother was asked to stay with the child. There was no hematemesis in next 1 week. The child remained comfortable with his mother, and during repeated interviews, child reported to the treating team that his father and paternal uncle used to give him betadine solution just before the act of emesis. The father and paternal uncle were confronted, but they were reluctant to agree their role in deception. They displayed anger and disbelief and insisted that their son should be immediately discharged and refused to contact the social services. Mother and child were incorporated in the treatment part. The social services decided to put the child under mother's custody.\nMother informed that the reason for this fabrication may be her dysfunctional family life. Father and paternal uncle were unemployed and used to stay idle at home. On multiple times, they were asked to do some work for earning, but they never tried. She used to do work in the field as well as at home. There were frequent fights with them due to poor financial condition at home. |
A 34-year-old Caucasian female presented to the emergency department with complaints of right hemiparesis, numbness, dysphagia, and ataxia. These complaints began as numbness over her right scapular area two weeks prior, and the numbness later progressed to include the right leg. Additionally, she reported five days of waking up during the night with severe headaches. Although she reported a history of migraines, which were usually accompanied by an aura, she stated that these new headaches were different in nature. She also had no known allergies and no previous surgeries. Her mother had a history of breast cancer, and her father had a history of ischemic heart disease. A review of systems was otherwise negative. After workup in the emergency department and consultation by neurology, she was referred to neurosurgery for management of a high-grade brainstem tumor.\nExamination\nOn initial presentation, the patient was alert and oriented to person, place, and time with a Glasgow Coma Scale (GCS) score of 15. Her pupils were equal, round, and reactive to light. Cranial nerves II-XII were grossly intact. Motor testing revealed that strength was 5/5 in both upper and lower extremities. Pronator drift was noted in the right upper extremity. Dysmetria was noted in the right upper extremity during finger-to-nose testing, and discrimination of fine touch was subjectively diminished on the entire right side from the zygomatic process to the foot. Patellar reflexes were 3+ bilaterally. Over the course of her initial admission, the patient demonstrated a progressive decline, to include mild left facial droop, dysarthria, and a worsening dysphagia. A percutaneous endoscopic gastrostomy (PEG) tube was placed because the patient was unable to swallow without effort. These findings continued until her initial discharge. Upon readmission, the patient displayed similar findings. Left lower quadrant abdominal pain radiating to the shoulder and acute numbness of the left chest and shoulder were also noted. These findings progressed and worsened until the patient was intubated after deteriorating to a GCS score of 10.\nImaging\nMagnetic resonance imaging (MRI) of the brain with and without contrast revealed a heterogeneous T1 hypointense (Figure ) and T2 hyperintense (Figure ) signal abnormality involving the pontine base and extending into the medulla. There was mildly increased relative cerebral blood flow and blood volume within the enhancing portion of the abnormality, suggesting neovascularity. The pontine component of the lesion also showed no abnormal enhancement or significant hyperperfusion. Mild mass effect on the fourth ventricle was noted. There was no peritumoral edema or significant mass effect. The remainder of the brain revealed no abnormal enhancement. No abnormal leptomeningeal enhancement was observed. A magnetic resonance imaging (MRI) scan of the spinal column with and without contrast also demonstrated no evidence for metastasis to the cervical, thoracic, or lumbar spine, with normal caliber and signal intensity of the spinal cord. A computed tomography (CT) scan of the chest with (Figure ) and without (Figure ) contrast enhancement revealed scattered small ground glass and nodular opacities bilaterally. These were nonspecific, with differentials including inflammatory and infectious etiologies, although metastatic foci remained difficult to completely exclude in the context of malignancy. A CT of the abdomen and pelvis with contrast revealed no intra-abdominal mass or evidence of metastatic disease.\nDifferential diagnosis\n- High-grade glioma\n- Primary central nervous system (CNS) lymphoma\n- High-grade medulloblastoma\nManagement\nThe patient was initially started on dexamethasone 4 mg orally every six hours until imaging was reviewed. Dexamethasone was discontinued due to a concern for possible lymphoma, but shortly thereafter the patient began to complain of worsening ataxia, dysarthria, and dysphagia. Dexamethasone was subsequently resumed.\nOperation\nThe patient was transported to the operating room on Day 5 of her hospitalization to undergo a closed stereotactic needle biopsy. Once the patient was anesthesized, the head was secured and the fiducials on the scalp were registered by cameras into the computerized stealth CT neuronavigation system in the operating room. A minimal amount of hair was shaved from the scalp and a small incision was marked out. This area was then meticulously cleaned and draped in a sterile fashion. An opening in the skull about the size of a quarter was made (burr hole), exposing the dura which was then opened. A stereotactic biopsy needle was then introduced with intraoperative use of the neuronavigation system in order to guide the needle to the target using a contralateral approach (Figure ). Biopsy samples were successfully obtained for pathologic examination. After the incision was closed, a clean and dry dressing was applied. The patient was then extubated in the operating room and transported to the post-anesthesia care unit in stable condition.\nPostoperative course\nPostoperatively, a nasogastric tube was placed to provide nutrition, due to worsening dysphagia. On Day 10 of her hospitalization, a PEG tube was placed. The patient was scheduled for chemotherapy and craniospinal radiation therapy, and was discharged on Day 12. Four days later, the patient returned due to worsening symptoms, and dexamethasone was increased to 10 mg orally every six hours. The patient was started on temozolomide IV (intravenous) on Day 10 of readmission, but she developed respiratory acidosis/hypercapnia with a CO2 of 115 and was started on a bilevel positive airway pressure (BIPAP), two days later. As a result, a rapid response team was called to transfer the patient to the intensive care unit (ICU). The temozolomide was converted to oral dosing via a PEG tube. The patient’s family was consulted regarding the patient's status and their options. The patient chose to update her status to do not intubate. The patient was extubated prior to her discharge to a hospice care facility, where she passed away four days later.\nPathologic findings\nThe brain biopsy was originally interpreted as a classic medulloblastoma (WHO Grade 4). The tumor was composed of sheets of small blue cells with rare poorly formed rosettes (Figure -). An immunohistochemical stain for glial fibrillary acidic protein (GFAP) and synaptophysin revealed moderate astrocytosis (Figure ) and little synapse loss (Figure ), respectively. Reticulin staining was also negative. Staining for p53 was positive, and Ki-67 index was about 50%. The sample was then sent to St. Jude’s Children’s Research Hospital in Nashville, TN, for further molecular analysis. Although their differential diagnosis also included medulloblastoma, the diagnosis was amended at St. Jude’s Children’s Research Hospital to small cell glioblastoma (WHO Grade 4). Their analysis indicated only focal immunoreactivity for GFAP and weak immunoreactivity for synaptophysin. However, immunoreactivity for Olig-2 and p53 was present in a majority of the tumor cells. Interphase fluorescence in situ hybridization (iFISH) analysis revealed platelet-derived growth factor receptor A (PDGFRA) amplification. No amplification of MYC, NMYC, or epidermal growth factor receptor (EGFR) was observed. |
A 35-year-old female presented with a painless, progressive diminution of vision in right eye since 3 years. She also complained of forward protrusion of right eyeball for past 2 years. General physical examination was unremarkable. On ocular examination best corrected visual acuity in the right eye was perception of light absent and in the left eye was 20/20. Exophthalmometry showed 3 mm proptosis in the right eye. The size of proptosis did not vary with posture or with valsalva maneuver. The restricted ocular movements in all gazes in the right eye were suggestive of right-sided III, IV and VI cranial nerve palsy. The ocular movements in the left eye were normal. There was diminished corneal sensation in the right eye. The rest of the cranial nerve examinations were normal. Slit lamp examinations of the eyes showed dilated pupil in the right side which was not reacting to light. The fundus examination revealed secondary optic atrophy in the right eye and normal left eye. Hematological tests were within normal limits. MRI images of brain and orbit [Figure and ] demonstrated a large well-marginated extra-axial middle cranial fossa mass with myxomatous regions at the right anterior temporal region extending into right intraconal space of orbit through optic foramen and superior orbital fissure to produce displacement of optic nerve and eyeball to cause proptosis. The mass was hypointense on T1, hyperintense with hypointense septae on T2 and marked enhancement of the mass with diffuse irregular hypointensity on hyperintense background on gadolinium-enhanced sequences were suggestive of trigeminal schwannoma. There was encasement of the right internal carotid artery. The mass extended into right suprasellar area, interpeduncular fossa, perimesencephalic and cerebropontine angle cistern to cause contralateral shifting of the brain stem. CT of the brain revealed destruction of lesser wing and body of sphenoid bone on the right side with enlargement and erosion of ipsilateral superior orbital fissure, foramen ovale and foramen rotundum by the large hypodense well-marginated densely enhancing mass. The tumor entered the orbit through the superior orbital fissure which was extraconal. To become intraconal in the orbit, the tumor had eroded the lesser wing of sphenoid between the superior orbital fissure and the optic foramen thereby entering the annulus of Zinn. On the basis of the MRI and CT features and the location of the mass, a suggestive diagnosis of schwannoma of ophthalmic division of right trigeminal nerve was made. The patient was taken up for an excision biopsy under general anesthesia. A right-sided pterional craniotomy with orbitozygomatic extension was done. The mass was found to be extradural in position covered on the surface by the outer layer of the dura. This was dissected off and piece meal excision of the tumor mass was done including the intraorbital portion due to attachments to adjacent structures. The distal portions of right-sided III, IV and VI cranial nerves from the tumor could hardly be recognized intraoperatively. The right eyeball was enucleated by the ophthalmologist and the tumor masses were sent for histopathological examination.\nThe postoperative period was uneventful except mild numbness in the right half of the forehead. On histopathological examination, a biphasic pattern of tumor cells were seen with areas of closely packed spindle cells having fusiform nuclei and eosinophilic cytoplasm (Antoni A). In some places cells were arranged in palisades referred to as Verocay bodies. There was no evidence of malignant transformation. All these features confirmed the diagnosis of benign schwannoma []. After about 6 month follow up there were no signs of recurrence of the tumor. |
A 58-year-old female patient with high blood pressure on amlodipine 10 mg (1cp/d), presented to our department of maxillofacial surgery in February 2021 for a left maxillary mass that had been evolving for 6 months. The history of the disease goes back to 6 months before his admission with the appearance of a left maxillary tumefaction progressively increasing in volume responsible for homolateral exophthalmos and partial nasal obstruction without any other associated thoracic or abdominal-pelvic discomforts, all evolving in a context of apyrexia and weight loss quantified at 8 Kg in 6 months.\nThe clinical examination revealed a hard, painless and deep fixed left maxillary mass filling the upper oral vestibule, with no skin or mucosal signs, partial left nasal obstruction and left exophthalmos with no decrease in visual acuity or oculo-motricity disorders. Examination of the lymph nodes did not reveal any palpable cervical or axillary adenopathy. The thoracic and abdominal examination did not find any palpable mass or inflammatory skin signs on the surface.\nFacial Magnetic resonance imaging revealed an expansive process with a polylobed contour centred on the left maxillary sinus in T1 isosignal and T2 hypersignal, heterogeneously enhancing after injection of contrast, measuring 32×20×38 mm with extension towards the corresponding jugal soft tissues, the left nasal cavity and the left orbital cavity reaching the contact of the inferior right muscle [].\nA biopsy of the mass was performed under local anesthesia via the left superior vestibular approach and the anatomopathological result came back in favour of a monophasic synovial sarcoma with positivity of the Epithelial Membrane Antigen (EMA) on immunohistochemistry [].\nA thoracic-abdominal-pelvic CT scan was performed to evaluate the extent of the disease, which revealed a tissue mass in the right anterior chest wall measuring 58×43×35 mm with extension to the adjacent lung []. A surgical biopsy under general anesthesia of the thoracic mass by anterior transcutaneous approach was performed and the anatomopathological result was similar to the left maxillary mass. Given the similar anatomopathological findings of the two maxillary and chest wall masses and the impossibility of identifying the primary location, the diagnosis of synchronous double location of synovial sarcoma was retained.\nA multidisciplinary consultation was held to discuss the case and the decision to treat with chemotherapy was indicated. The patient was referred to oncology for curative chemotherapy and then followed as an outpatient at a rate of twice a month for the first three months and then once a month for one year. |
A 31-year-old male—previously healthy—was brought to the emergency department by ambulance after he lost his consciousness and fell down during running on a treadmill with no witnesses to the event. He was complaining of bilateral hip pain and inability to bear weight. The patient denied any incontinence but reported tongue biting. The patient had a history of drug abuse for body building purposes for the past 3 years. He reported taking anabolic steroids, growth hormone, thyroxine, and creatinine with no professional supervision and no compliance for dose limits.\nUpon physical examination, the patient was confused, with tender bilateral hips and externally rotated lower limbs, with no neurovascular compromise. Laboratory investigations revealed pan-pituitary axis insufficiency and mild vitamin D deficiency (). Radiological investigations showed bilateral neck of femur fractures. Both of which were graded as type IV according to Garden's classification (). A computed tomography (CT) scan of the pelvis confirmed the diagnosis with more comminution seen in the left side (). A CT scan of the head was done and was unremarkable. Endocrinologists were consulted, and the advice was to keep the patient on corticosteroids and wean him off after the surgical intervention.\nThe patient was stabilized and cleared for surgical intervention. He was operated on the same day of admission. A fracture table was used to facilitate closed reduction. After sound reduction fixation was achieved by 6.5 mm cannulated cancellous screws on one side and a sliding hip screw with an antirotation screw on the other side. The senior author's decision to fix the left side with a sliding hip screw was explained by more comminution and higher risk of construct failure compared to the right side. Immediate postoperative images showed acceptable reduction and fixation (). Later during the admission, the neurology team was consulted and an Electroencephalogram (EEG) showed Frontal Intermittent Rhythmic Delta Activity (FIRDA), and Magnetic Resonance Image (MRI) of the brain was unremarkable. The patient was diagnosed as a case of Generalized Tonic Clonic Seizure (GTCS) and was started on levetiracetam.\nThe patient was discharged on a wheel chair at the beginning and gradually converted to partial- and then full-weight bearing within four months. He had a total of six months of regular follow-up postoperatively with strict physical therapy and rehabilitation plan. In the last follow-up 18 months postinjury, a plain radiograph showed complete fracture union on both sides with no signs of avascular necrosis (). His gait was normal, and he could return back to his normal daily activity and noncontact sports.\nA follow-up EEG and video monitoring after stopping the hormones and supplements for 6 months were unremarkable, the final diagnosis was a single episode of GCTS due to an overdose of anabolic hormones, and the epilepsy medication was stopped by the neurologist. |
A 37-year-old woman, without medical/surgical history, had a radiopaque subdermal contraceptive implant (Nexplanon, Merck & Co., Whitehouse Station, NJ, USA) inserted in the left upper limb for contraception at a local clinic in 2014. The patients experienced irregular vaginal bleeding for two years after subdermal contraceptive implant and decision was made to remove the device. The subdermal contraceptive implant was not located by digital palpation or by ultrasound scanning of the insertion site. X-ray evaluation of both humerus anterior and posterior was conducted and radiopaque foreign body was not detected. In addition, the long bone upper extremity standing anterior posterior lateral X-ray was performed, and it showed that about 4 cm length rod-like material is seen at the left lower thoracic area (). Under the impression of foreign body at lower thoracic area, A non-contrast-chest computed tomography (CT) was performed, looking for a possible migration. It revealed that the foreign body was in left lower lobe of the lung (probably in subsegmental pulmonary artery branch in left posterior basal segment) (). The patient did not experience symptoms including chest discomfort or dyspnea while having the subdermal contraceptive implant. After consultations with cardiology and chest thoracic surgery department, the foreign body removal through intervention was considered initially at the division of cardiology, department of internal medicine. However, the foreign body was present in the lung parenchyme, and the decision was made to remove it through surgery at the department of chest thoracic surgery. The patient was admitted to cardiothoracic department. Under general anesthesia, the patient was positioned in the right decubitus position during a surgery and a 6-cm minithoracotomy has performed at left intercostal space. Left lower lobe of lung was retracted using a long clamp and the site of foreign body was confirmed manually. Bovie cauterization was done to expose the foreign body and enucleation was performed immediately afterward. A 24 Fr-standard chest tube was inserted and the surgery was completed (). The chest tube was removed on postoperative day 2. The patient was discharged from the hospital without complication. And further contraception plan was discussed at the outpatient clinic of the department of obstetrics and gynecology. |
A 65-year-old male presented to the emergency department after the acute onset of paraplegia associated with severe interscapular pain and hypertensive crisis. The patient had previously undergone open surgical repair of an infrarenal abdominal aortic aneurysm with aorto-aortic reconstruction sutured proximally approximately 15 mm below the lowest (right) renal artery and distally to the aortic bifurcation. Computed tomography angiography (CTA) showed the presence of an acute Stanford Type B aortic dissection originating just below the ostium of the left subclavian artery (LSA) and extending downward to the level of the renal arteries. Another radiological finding was a right HGA aneurysm measuring approximately 40 mm in maximal transverse diameter. The main aortic visceral branches (celiac trunk, superior mesenteric artery, renal arteries) were all patent and all took off from the true lumen. The abdominal aorta was almost completely occluded for a short segment just below the level of the renal arteries. This finding could be explained, in our opinion, by reflection and overturning of the dissection flap over the suture line of the preexisting surgical graft. The two iliac arteries were otherwise reperfused through the pelvic collateral network (\nand\n).\nThe patient was immediately transferred to the operating theater and underwent endovascular repair of the descending thoracic and abdominal aorta. After bilateral surgical cutdown of the common femoral arteries (CFAs), we gained access to the true lumen above the renal arteries using combined angiographic and transesophageal echocardiographic control. At first, we covered the proximal entry tear with placement of a thoracic endograft (Valiant, MEDTRONIC) without any oversizing. Proximal and distal landing zones were achieved respectively in Ishimaru's zone 2 of the aortic arch and approximately 10 cm above the ostium of the celiac trunk. We then proceeded with placement of a bifurcated abdominal endograft (Endurant II, MEDTRONIC) with 15% oversizing. Proximal landing zone was achieved just below the right renal artery while distal landing zone was reached, bilaterally, above the bifurcation of the common iliac artery (CIA). At the end of the procedure, no residual filling of the false lumen was noted. Owing to the patient's unstable clinical condition, we decided not to perform any adjunctive procedure (i.e., restoration of blood flow to the LSA or exclusion of the right CIA aneurysm) at this time and the patient was transferred to the intensive care unit.\nFollowing the operation, the patient's condition improved and he fully recovered from paraplegia. He was discharged home 4 weeks after the index procedure. CTA performed at this time demonstrated correct placement of the endografts with adequate exclusion of the false lumen, patency of all aortic side branches, and absence of any detectable endoleak. At this time, the patient was also screened for the most common genetic aortopathies but the tests were negative.\nWe scheduled elective exclusion of the right HGA aneurysm 4 months after the index event. Open repair of the iliac aneurysm with HGA preservation was ruled out because of the patient's preference for endovascular treatment. As the patient's anatomy was unsuitable for HGA preservation, we planned for its exclusion. We assessed the likelihood of PSCI through dynamic angiography associated with neuromonitoring. In the operating room, the patient was positioned supine and general endotracheal anesthesia was administered. The right HGA was thereafter occluded for 10 minutes (\n), with simultaneous recording of motor-evoked potentials (MEPs) and somatosensory-evoked potentials (SSEPs) bilaterally on the legs. Systemic heparinization was not administered during the balloon occlusion test. Our neurophysiologist interpreted the examination as negative for any signs of ischemia of the spinal cord, and thus we proceeded with coil embolization of the right HGA and subsequent positioning of a straight iliac endograft (Endurant II, MEDTRONIC) whose distal landing zone was achieved in the external iliac artery, covering the orifice of the HGA. The postoperative course was uneventful and the patient was discharged home 4 days after the operation.\nCTA follow-up at 1, 6, 12 and 24 months showed satisfactory placement of all endografts without any signs of endoleak or endograft failure. There was effective remodeling of the descending thoracic aorta, with volume reduction of the false lumen. The patient was free from new-onset neurologic and/or aortic complications at the longest available clinical follow-up. |
A 71-year-old male with a medical history of type 2 diabetes and prostatic hyperplasia initially presented with weight loss and exertional dyspnoea in August 2020. A CT scan on 28 August 2020 revealed marked mediastinal lymphadenopathy (a) and multiple lung nodules as well as a tumour of the right kidney. A cryobiopsy of an intramural bronchial right lung lesion was reported as a metastasis of grade 2 clear cell renal cell carcinoma (RCC). The patient started to develop signs and symptoms of heart failure confirmed by high levels of N-terminal pro-hormone B-type natriuretic peptide and low left ventricular ejection fraction of 25% at the beginning of October 2020 and was admitted for treatment to an internal medicine department on 10 November 2020. On 21 November 2020, he unexpectedly developed abdominal discomfort and diarrhoea. A PCR test for SARS-CoV-2 was positive. Aside from supportive measures, no specific treatment for COVID-19 was administered. As the cardiac failure had improved by this time, he was discharged after the end of the prescribed quarantine period on 1 December 2020. Right nephron-sparing nephrectomy was planned and carried out on 14 January 2021 confirming the histology of clear cell RCC. The patient was then referred for systemic treatment of mRCC.\nAt his first oncology appointment he was well, with an Eastern Cooperative Oncology Group (ECOG) performance status of 1. He had a urinary catheter for obstruction due to prostatic hyperplasia. His medication at this time included metformin, spironolactone, furosemide, and metoprolol. Laboratory results were unremarkable except for renal impairment with a glomerular filtration rate (GFR) of 30 mL/min and a moderately increased neutrophil count (8800/µL). A CT scan was ordered to ascertain the extent of the disease prior to planned targeted therapy and was carried out on 15 February 2021; surprisingly, this showed marked regression of all tumour lesions (b). Consequently, no treatment was initiated and a follow-up CT scan on 18 May 2021 confirmed ongoing substantial partial regression of metastatic lesions (c). |
A 68-year-old male with a past medical history of a neuroendocrine tumor (NET) of the left femur presented with progressive dyspnea, orthopnea, and lower extremity edema. Three years ago, the patient was found to have a mass on the left femur. Biopsy revealed poorly differentiated neuroendocrine carcinoma of unknown primary. He had undergone surgical resection of the left femoral tumor and above-knee amputation with adjuvant chemotherapy (cisplatin and etoposide) and radiation therapy. Routine surveillance imaging showed no evidence of malignancy. Chest computed tomographic (CT) and magnetic resonance imaging of the abdomen/pelvis with contrast were performed at three-month intervals for the first year followed by six-month intervals. The patient was in clinical remission for the last two years.\nOn physical exam, his blood pressure was 119/76 mmHg, heart rate was 104 beats per minute, respiratory rate was 22 breaths per minute, and jugular venous pressure was elevated. Grade III/VI systolic ejection murmur was present at the left sternal border and rales at the lung bases. Chest X-ray revealed cardiomegaly and bilateral pleural effusions. A transthoracic echocardiogram revealed a large mass measuring 8.10 X 6.54 cm within the right ventricle causing right ventricular outflow obstruction, and the left ventricular ejection fraction was 60-65% (Figure ). Cardiac magnetic resonance imaging confirmed the mass extending from the right ventricular free wall with compression of the left ventricle and dilated right atrium (Figure ). A positron emission tomographic/computed tomographic scan showed increased standardized uptake value activity of 9.3 in the right ventricular mass (Figure ). Cardiac biopsy of the right ventricular mass was consistent with metastatic neuroendocrine tumor (Figure ). The tumor cells were negative for synaptophysin and chromogranin A but positive for CDX2, a marker for neuroendocrine tumor of unknown primary []. In view of the tumor that caused impairment in the right ventricular filling and congestive heart failure (CHF), the patient received chemotherapy (doxorubicin and cyclophosphamide) with improvement in the right ventricular failure. |
A 70-yr-old woman, gravida 8 para 3, was referred to a tertiary medical center from a local hospital for vaginal bleeding of 3 weeks' duration. She had undergone a punch biopsy at the local hospital, which resulted in a preliminary diagnosis of adenoid cystic carcinoma or carcinoid tumor. Her medical history included diabetes mellitus (DM) for 10 yr which had been controlled by medication; in addition, her mother died of uterine cervical cancer. Physical examination showed uterine cervical erosion. The biopsy specimen taken at the local hospital showed pathologic evidence of a high grade malignant epithelial tumor, with features unusual for a cervical tumor. She therefore underwent a loop electrosurgical excision procedure (LEEP) cone biopsy, which revealed a basaloid squamous cell carcinoma. A colonoscopy, intravenous pyelogram, and cystoscopy showed no evidence of metastatic disease. Magnetic resonance imaging showed a 2 cm sized cancerous mass confined to the cervix, with no evidence of invasion of the vagina or fornix and no evidence of pelvic lymphadenopathy (). The tumor was classified as clinical stage Ib1. A radical hysterectomy was performed, along with bilateral salpingo-oophorectomy, pelvic lymph node dissection, and paraaortic lymph node sampling. The pathologic diagnosis was basaloid squamous cell carcinoma. The depth of invasion was 5/7 mm full thickness of the cervical wall. There was no evidence of tumor in sections taken from 26 lymph nodes. The resection margin of the vaginal cuff was clear. No adjuvant treatment was administered, and the patient was discharged. In the 12 months since discharge, she has shown no evidence of recurrent or metastatic disease. Follow-up is ongoing at Asan Medical Center.\nA well-defined, fungating firm mass (1.5×1.0×0.6 cm) was present in the posterior wall of the cervix and invaded 5 mm into the cervical wall (full thickness, 7 mm). The parametria and vaginal cuff showed no tumor invasion. The cut surface of the mass was gray and granular. The tumor cells were immunopositive for p63 and immunonegative for S-100 protein. The cells appeared basaloid with small hyperchromatic nuclei, distinct nucleoli and scanty cytoplasm. There was also peripheral palisading, supporting the above diagnosis (). |
A 59-year-old woman presented for evaluation of scalp alopecia. Her past medical history was significant for PT1cN1mi estrogen receptor (ER)+, progesterone receptor (PR)+, human epidermal growth factor receptor (HER)2+ g3 invasive ductal carcinoma of the right breast diagnosed 15 months earlier. She had been treated with bilateral lumpectomy with right-sided sentinel lymph node biopsy and started chemotherapy nine months earlier; she received pertuzumab, docetaxel, carboplatin, and trastuzumab every three weeks for six cycles and was maintained on trastuzumab 6 mg/kg every three weeks for one year. Three weeks after completing taxane chemotherapy, she began treatment with anastrozole 1 mg daily (which was switched to tamoxifen 20 mg daily due to joint pain). She was also treated with radiation therapy and is currently on neratinib 240 mg daily; neratinib is a tyrosine kinase inhibitor anticancer drug used to prevent recurrence in patients with early-stage HER2+ breast cancer who have finished at least one year of post-surgery trastuzumab therapy.\nShe noted hair loss beginning after her first course of systemic chemotherapy. It became more extensive throughout the remainder of her treatment. She had not experienced any regrowth of scalp hair since the completion of chemotherapy nor during her current hormonal therapy.\nCutaneous examination revealed alopecia of the scalp. The clinical presentation was most consistent with female pattern alopecia with diffuse and nearly complete hair loss on the central and vertex region with retention of hair on the occipital scalp. There was partial, diffuse hair loss – to a lesser degree – on the parietal scalp bilaterally (Figure ). There was also loss of hair on the eyebrows, axillae, pubic region, and upper lip. However, these areas had already slowly started to show regrowth.\nBiopsies from the right and left sides of her parietal scalp, in areas of alopecia with some preservation of follicles, were performed for horizontal and vertical sectioning. Both showed similar pathologic changes of a non-scarring alopecia. The predominant feature noted was extensive miniaturization of the hair follicles; this change was most suggestive of androgenetic alopecia. However, other findings – present to a lesser extent – included pigment casts in hair follicles, increased catagen to telogen ratio, and empty fibrous tracks; these changes may be observed in alopecia areata.\nCorrelation of the patient’s history, clinical presentation, and pathologic findings supported a diagnosis of antineoplastic (chemotherapy and hormonal) treatment-associated alopecia. Specifically, her features were consistent with those previously reported in patients with breast cancer after taxane chemotherapy and adjuvant hormonal therapy who developed permanent alopecia []. Treatment was initiated with minoxidil 5% foam to be topically applied to the scalp twice daily.\nThe patient returned for follow up four months later. She was pleased with the clinical outcome and had noticed increased scalp hair growth; however, she commented that she always used minoxidil once daily and occasionally twice daily. In addition, hair growth on the eyebrows, axillae, and pubic area continued to demonstrate clinical improvement. She decided to continue treating her scalp in a similar manner.\nHer subsequent follow-up visit, six months later (after ten months of topical minoxidil therapy), showed additional hair regrowth. Specifically, the central and vertex area of her scalp had thickening of her hair; in addition, there was new hair growth on the parietal regions bilaterally (Figure ). She continues to use 5% minoxidil foam once daily. |
A 53-year old man visited the emergency room with suddenly developed mental deepening. On past history, he had an uncontrolled hypertension with left hemiparesis due to head injury of traffic accident 20 years ago. On neurological examination, he showed semi-comatose mental status. Glasgow Coma Scale was 5. He could not open his eyes. He was withdrawal to pain. He was in intubated state. The pupil size was 8 mm on right eye and 3 mm on left. The light reflex was absent on both pupils. The corneal reflex was present. Brain CT showed 8.6-cm-sized solid and cystic mass on right temporal lobe that was associated with hemorrhage (). Solid lesion showed a strong enhancement after an administration of contrast media (). The mass effect was severe with 1.8 cm-midline shift from right side to left. The provisional diagnosis was high grade glioma associated with acute hemorrhage. Because of his poor neurological status and severe mass effect, emergency craniotomy and tumor removal were performed. Intraoperatively, the mass was an intraparenchymal lesion without dural attachment (). The lesion had a cyst with yellowish fluid and myxoid change. It was acute hematoma and firm solid lesion. The solid hemorrhagic lesion contained many vasculatures that were relatively well-circumscribed from normal white mater. The mass was totally resected. Postoperative MRI showed no enhancing lesion on right temporal lobe. Postoperative 3 weeks later, he was recovered to confused mental status with dysarthria.\nPathologically, tumors showed a patternless arrangement. Both the hypocellular area and the hypercellular area appeared. The hyalinized band divided the boundary between hypocellular and hypercellular areas. It was surrounded by thin walled staghorn-like vasculatures (). Other portions consisted of tissues arranged very tightly which meant solid portion, which had more cellularity and much more dense structures. In random arrangement, stromal surrounding cells were much less while mitosis and necrosis were more common. Mitosis exceeded 70 in 10 high power fields (). Immunohistochemistry showed STAT6 expression restricted in the nucleus (). Anaplastic solitary fibrous tumor/hemangiopericytoma with high mitotic index was diagnosed. Postoperative radiotherapy of 5,000 cGy was done. Postoperative 4 months later, the patient was recovered to alert mental state. Brain MRI showed no recurred lesion (). |
We present a case of a 50-year-old Caucasian male who presented to the emergency department with complaints of lower abdominal pain, fever and sweating. On examination, the patient had tenderness to palpation in the right iliac fossa, with significant rebound tenderness and guarding. Body temperature was recorded at 38.5°C.\nThe patient's past medical history was significant for an incident of similar pain six months prior to presentation. He was diagnosed with sigmoid diverticular disease confirmed by computed tomography (CT) scan and managed conservatively. A subsequent colonoscopy confirmed the diagnosis, and did not reveal any other colonic pathology.\nFurther investigations revealed a raised white blood cell count of 16,000 per microliter. CT scan of the abdomen and pelvis disclosed evidence of extensive free gas under the right dome of the diaphragm confirming suspicion of a perforation. Fluid-filled prominent loops of small bowel were noted. However, none of them were dilated to suggest obstruction. Mild bowel thickening was also noted around the cecum. Pneumatosis coli suggestive of ischemic bowel, extending from the cecum to the proximal ascending colon was seen. A blind ending structure with calcifications was also seen (Figure ). Other significant findings on the scan included a liver cyst, consistent with the patient’s previous CT scan, and consolidation at the base of the right lung.\nSubsequently, the patient underwent an emergency laparotomy and a diffuse four-quadrant peritonitis was seen. A giant perforated MD, 80 cm proximal of the ileocecal valve was identified as the cause of the peritonitis. The giant MD measured approximately 10 cm in length and 2.5 cm in width (Figure ). A small perforation at the tip of the MD was observed. No other intraabdominal pathology was identified, in particular, there was no evidence of colonic ishchaemia. Resection of the segment of small bowel bearing the MD was performed with a side-to-side stapled anastomosis.\nPathologic examination showed a T-shaped length of bowel, 6 x 5 x 3 cm in maximal dimension, with a 3 x 5 x 6 cm portion of mesentery attached. The presumed diverticulum was 5 cm long with a maximal inner circumference of 5 cm. The perforation measured 0.2 cm and the lumen of the specimen was stained green. No heterotropic tissue was identified.\nPost-operative recovery was uneventful and the patient was discharged on oral antibiotics. |
A 26 year-old man was identified to donate marrow for his brother. His height was 178 cm and his weight was 79.2 kg (Body mass index 25.0). He had no bleeding history or other medical problem. Bone marrow harvesting was performed under spinal anesthesia []. The patient was put in the prone position, and the bony landmarks of the posterior iliac crest and sacroiliac joint were palpated for the identification of a proper puncture site (Fig. ). Aspiration trocar and needle were pushed through the skin and subcutaneous tissue to the posterior iliac crest, and the cortical bone was punctured. Bone marrow aspiration was performed after positioning the needle tip within the cortical wall of the posterior crest []. There was no repositioing of the needle. The total surgery time was 62 min. A total of 900 cc of bone marrow(450 cc per site) was collected which yielded 1.46 × 108 CD34-positive cells from the two puncture sites shown in Fig. . No special problems occurred during the procedure. The donor was hospitalized one more day after bone marrow harvesting to check complications and to control the pain. There was no evidence of hematoma on the puncture sites. While in hospital, he suffered mild pelvic pain which had responded to an oral non-steroidal anti-inflammatory drug (NSAID).\nTwo days after the bone marrow harvesting, a pain of tingling and stabbing nature appeared on his left posterior thigh and calf. Pain score was noted at Visual Analogue Scale (VAS) 7 points on resting and aggravated with motion. Allodynia was present. Sensory of all dermatome was intact, and no muscle weakness was present. However, there was gait disturbance due to pain.\nWe conducted a pelvic magnetic resonance image (MRI), nerve conduction study (NCS), and electromyography (EMG) for evaluation. T1 and T2 weighted images of the pelvis magnetic resonance image (MRI) showed patchy edematous change with enhancement in the sacrum, retrosacral muscles, and subcutaneous layer, and the left S2 neural foramen (Fig. a, b). Imaging studies indicated that the left S2 nerve root was injured by mechanical damage when the puncture needle was inserted and that the nerve irritation and inflammation were the cause of the patient’s symptoms [, ].\nAfter 1 month since the pain developed, nerve conduction study (NCS) and electromyography (EMG) were performed. Nerve conduction study (NCS) revealed normal velocity and amplitude of the common peroneal nerve, tibial nerve, sural nerve, and superficial peroneal nerve. Hoffmann reflex, pudendal evoked potential were within normal limits. Electromyography (EMG) showed abnormal spontaneous activities, which are denervation potentials, in the S2-innervated intrinsic foot muscles and the S1-S2 nerve root innervated muscles such as the soleus, gastrocnemius, and lumbar paraspinalis muscle (Table ) [–]. The amplitude of the abnormal spontaneous activities were about 100 μV, indicating that the development of muscle membrane instability following neural injury occurred within 1 month [] (Fig. ). Electrodiagnostic results along with the patient’s clinical presentation and MRI findings led us to a diagnosis of left S2 radiculopathy.\nThe patient took pregabalin 75 mg two times per day to control the pain, and after 3 months of medication, the patient’s pain improved from VAS 7 to 5 []. A follow up nerve conduction study (NCS), electromyography (EMG) and pelvic magnetic resonance image (MRI) were performed 3 months after onset. Consistently, nerve conduction study (NCS) and Hoffmann reflex were within normal limits, and abnormal spontaneous activities were observed in S2 nerve root innervated muscles. In the pelvic magnetic resonance image (MRI), little residual enhancement was still present along the left S2 nerve root (Fig. c, d).\nAt 6 month follow up, visual analogue scale (VAS) further improved to VAS 3, and electromyography (EMG) showed motor unit action potentials (MUAPs) of re-innervation pattern instead of abnormal spontaneous activities, indicating recovery state (Table , Fig. ). The patient took pregabalin for a total of 8 months. After that, the patient stopped medication. One year later, the patient’s pain was reduced to a level that was not inconvenient, and we did not prescribe any further medication. Additional nerve conduction study (NCS), electromyography (EMG), and magnetic resonance image (MRI) were not performed. |
An 86-year-old multiparous Japanese woman with an unremarkable medical history was transferred to our hospital after a traffic accident (Table ). She was hit by a car while walking at a crosswalk. On presentation to our emergency department, she complained of pain in her buttock. Her Glasgow Coma Scale score was 15/15. Her blood pressure was 100/53 mmHg, heart rate was 93 beats/minute, respiratory rate was 15 breaths/minute, and oxygen saturation was 100% while breathing 2 L/minute of oxygen. A whole-body computed tomography scan revealed fractures of her left pubic bone and sacrum and a hematoma with contrast extravasation in front of the sacrum (Fig. ). Her blood pressure then suddenly dropped to 67/38 mmHg secondary to hemorrhagic shock. Rapid resuscitation with fluids and blood was performed. We attempted to perform transcatheter arterial embolization (TAE). Based on angiographic findings (Fig. ), bilateral internal iliac artery embolization was performed with gelatin sponge particles. She received 560 ml of packed red cells, 480 ml of fresh frozen plasma, and 200 ml of platelets, and she became hemodynamically stable.\nAfter admission to our intensive care unit, she developed shaking chills and a high fever. She was hemodynamically stable the following day. However, reddish urine was observed. Her serum lactate dehydrogenase level was extremely high, and fragmented red blood cells were present on peripheral blood smears. On the third day of hospitalization, despite fluid challenges and the use of diuretics, she became anuric and thus underwent hemodialysis. However, she developed severe delirium and was intubated under sedation. She was not diagnosed as having TTP at this point because her platelet count was not reduced despite the worsening of her hemolysis. Her prothrombin time and activated partial thromboplastin time were normal, and her fibrin degradation products were returning toward the normal concentration within 3 days of admission; therefore, disseminated intravascular coagulation (DIC) was excluded.\nOn the fifth day of hospitalization, her platelet count, measured by a different hemocytometer, was very low. Her fragmented red blood cell concentration measured by visual judgment based on the International Council for Standardization in Haematology (ICSH) reference method was 28.8% (Fig. ). We finally confirmed the diagnosis of TTP based on the classic pentad of TTP and began plasma exchange.\nAfter retrospectively checking her platelet count, we found that it had decreased to 55 × 109/L on the second day of hospitalization (Fig. ). The presence of many fragmented red cells is often associated with a spurious increase in the platelet count because the fragmented red cells are erroneously measured as platelets by automated blood cell counters.\nPlasma exchange was continued for 5 consecutive days. Her clinical course dramatically improved in just a few days, and her platelet count increased. She was weaned from hemodialysis on the 15th day of hospitalization. She recovered fully and was discharged from our hospital on the 31st day of hospitalization. The ADAMTS13 activity measured by an enzyme immunoassay on the third hospital day was not reduced (65%), but a direct Coombs test was negative and the complement factor level was normal. |
A 62-year-old man, with no significant past medical history, was diagnosed with non-invasive bladder cancer in 2016. The patient received six rounds of intravesical BCG treatments and an additional BCG treatment with Interferon-G following the BCG treatments. Subsequently, two follow-up cystoscopic examinations for routine surveillance did not reveal any evidence of cancer recurrence. About five to six months following the negative cystoscopic exams, the patient presented to our hospital emergency department with symptoms of new onset of severe axial back pain along with thoracic radicular symptoms, with radiation of pain to the sternum. Additionally, on examination, the patient had signs of early myelopathy, with numbness about an inch below the nipple line a month prior to presentation at our clinic with exaggerated lower extremity reflexes and increased tone, pathological reflexes. The patient was extensively worked up for alternate causes of chest pain, and the cardiac and upper gastrointestinal exams were negative. Imaging of the thoracic spine was completed because of the symptomatic and clinical localization to the mid-thoracic dermato-myotomes. MRI images (Figure A - D) revealed a contrast-enhancing mass lesion with paraspinal extension, erosion of the adjacent bone and disc space, and ventral lateral epidural extension causing spinal cord compression.\nCT of the thoracic spine (Figure A & B) revealed erosive changes within the vertebral bodies and adjacent endplates along with disc height loss.\nBased on the imaging, a diagnostic biopsy was performed. The patient underwent a CT-guided bone biopsy with interventional radiology (IR). Preliminary reports were negative for malignancy, and cultures were negative. In order to differentiate between metastatic tumor and infection, we decided to obtain tissue from the paraspinal abscess via a transthoracic approach. Along with cardiothoracic surgery (CTS), the patient underwent video-assisted thoracoscopic surgery (VATS) for aspiration and biopsy of the pre/paravertebral soft tissue abscess and several biopsies of the lesion adjacent to T5 were taken. The clinical appearance was characteristic of a caseous necrotic purulent yellow material. The patient had symptomatic progression of his myelopathy, and we decided to decompress the dural tube and cord, stabilize the patient to relieve the symptoms, and treat him accordingly. While his cultures were awaiting, he underwent a posterolateral T4-T5 decompression with a laminectomy, along with evacuation of a granulomatous mass. This was followed by a T3-T6 posterior thoracic fusion. Intraoperative findings revealed a thick firm, vascular yellow grey tissue mass with necrotic cheesy material surrounding the thecal sac and exiting nerve roots. It was sent for pathology and appropriate cultures. Although several samples were obtained from both these procedures, only one sample was AFB positive.\nBased on the intraoperative findings, AFB staining, and pathology of granuloma in consult with infectious disease, he was started on anti-mycobacterial treatment with isoniazid (INH), rifampin, and ethambutol. Subsequently, the patient completed one year of anti-mycobacterial treatment with healing and fusion along with the resolution of his myelopathic symptoms. His follow-up contrasted MRI of the thoracic spine at 18 months post evacuation of spinal mass with instrumented fusion showed resolution of prior T4-T5 vertebral osteomyelitis and stable hardware.\nAt clinical follow up at 18 months post-surgical intervention, the patient was not experiencing back pain, radicular symptoms, numbness, or weakness |
A 30-year-old woman (G3 P1 + 1) booked for antenatal care at seven weeks of gestation. She had had an uncomplicated pregnancy with spontaneous vaginal delivery two and a half years previously, with epidural analgesia, as well as a prior miscarriage.\nA year after her first delivery, she presented to the emergency department with severe back pain, worse on the right side. An X-ray of her spine showed instability of the third to fifth lumbar vertebrae, with widening of the disc spaces. She was referred to the orthopedic surgeons and had progressive worsening of her back pain and new-onset weakness in her right leg over the course of one year. Magnetic resonance imaging (MRI) of her lumbar spine revealed multi-level lumbar disc prolapse involving the third lumbar vertebra (L3) to the first sacral vertebra (S1), worse at L3/L4 (see , ). Spinal surgery was discussed, but the patient declined at the time and was managed conservatively. She was also diagnosed with type 1 Arnold-Chiari malformation () six months prior to her index pregnancy, following complaints of neck pain radiating to both arms, with tingling, numbness and shooting pains, and was under the care of neurosurgeons. Her last MRI scan was a month before pregnancy and did not show any significant progression in her disease.\nHer main symptoms at booking were occipital headaches radiating to both arms, severe low back pain radiating to both legs, worse on the right, and weakness in both legs. She did not have any bowel or bladder dysfunction. She was managed mainly with paracetamol. She continued with her physical therapy remotely due to the COVID-19 pandemic and could not access hydrotherapy, which had previously been helpful.\nHer pregnancy was otherwise uncomplicated, with regular follow-up in the consultant antenatal clinic. She had anesthetic consultations at 30 weeks of gestation and again at 36 weeks to plan her anesthetic management. She also had both neurosurgery and orthopedic consultations at 14 and 30 weeks of gestation.\nBoth her neurosurgeon and orthopedic surgeon recommended delivery by cesarean section (CS) under regional anesthesia at a multidisciplinary discussion between all consulting physicians, due to the potential risks of aggravating her disc prolapse by an attempt at vaginal delivery. This was also the preferred option of the patient. General anesthesia (GA) presented potential dangers, as head extensions and raised intracranial pressures could aggravate her neurological symptoms. She had a CS planned for 38 weeks of gestation, but she presented the night before her scheduled CS with spontaneous rupture of membranes and mild contractions. She had an uncomplicated emergency CS under spinal anesthesia done by the head of obstetric anesthesia, using ultrasound guidance.\nHer immediate post-operative period was uncomplicated; there were no neurological symptoms nor worsening of her existing symptoms. She was discharged home on the second day post-delivery. No new developments were reported in follow-up calls after two weeks and one month. |
A previously healthy 40-year-old woman was referred to us by an ophthalmologist who noticed anisocoria with a small right pupil and a dilated left pupil. She had no ocular symptoms and saw the ophthalmologist because of conjunctivitis. She gave a history of flu-like illness that lasted about three months; that acute illness occurred five months before the eye examination and resolved eventually without any treatment. No diagnosis was made at that time. She did not complain of difficulties with her vision and could read without difficulty. She denied fever, fatigue, dizzy spells, tachycardia, syncope, recurrent vomiting, dry mouth, rhinitis, dry eyes, constipation, or bladder symptoms. She did not complain of lack of sweating (anhidrosis) or hyperhidrosis and tolerated well changes of temperature. Her general physical examination was unremarkable. Blood pressure was 120/80 mmHg in supine and standing position, and her pulse was 65 beats per minute in supine and standing position. Neurological examination showed generalized absence of muscle stretch reflexes that could not be elicited with the Jendrassik maneuver. Several independent neurologists confirmed the absence of reflexes in the lower and upper extremities. Plantar reflexes were flexor. Romberg sign was absent; there was no weakness and no sensory ataxia. Careful sensory examination for touch, pin prick, and cold was normal. Position sense of the toes and fingers was normal. She could perceive well the vibration of a tuning fork (256 Hz) distally and proximally in both upper and lower extremities. Eye movements were normal. Her right pupil was 1.5 mm and had an oval shape, with no response to light (); the left pupil measured about 6 mm and responded very poorly to light. The response to light of the left pupil was segmentary with a slight response of the right superior segment. Both responded poorly to accommodation although the left pupil remained slightly more miotic after an accommodative effort. The pupils contracted briskly with pilocarpine dilution of 0.125%. Brain and cervical cord MRI was normal. A five-minute ECG strip in DII appeared to have an almost fixed heart rate. The lack of significant tachycardia during standing and the ECG findings prompted the physicians to perform autonomic cardiovascular studies.\nA normal sympathetic skin response (SSR) was obtained in the palms and the soles with electric stimulation of the median nerve. The latency at the palm was 1.34 seconds, and that at the soles was 1.89 seconds. The SSR could also be obtained with deep inspiration and with a cough. Four extremities, sensory and motor conduction velocities, and F responses were performed and found to be normal (Viking, Viasys, 2008).\nWe studied the HRV and the SBPV by capturing the RR interval and the systolic, diastolic, and mean blood pressure using Finometer® PRO (Finapres Medical Systems BV, Netherlands) under the following conditions: (1) supine at rest during a period of five minutes; (2) standing up, for five minutes, after the BP and the HR have become stabilized; and (3) during rhythmic breathing at 0.1 Hz (6 cycles per minute) during a period of five minutes. The signals were obtained with 200 Hz sampling rate with an analogic/digital resolution of 16 bits. We analyzed the five-minute time series for the RR intervals and the blood pressure time series with the BeatScope® software. The interbeat intervals time series were analyzed in milliseconds (RR intervals) and also in beats per minute (HR); the blood pressure time series were analyzed in mmHg. We performed the time domain analysis measuring (1) mean HR and SBP, (2) standard deviation of HR and SBP, (3) range of HR and SBP, (4) maximum, (5) minimum, and (6) ratio of maximum/minimum HR in the three conditions. We studied the frequency domain using the Fast Fourier Transform (MATLAB®, 1999) and obtained the following data: (1) absolute power of HF (high frequency, 0.15–0.4 Hz), (2) absolute power of LF (low frequency, 0.04–0.15 Hz), (3) sympathovagal balance (SVB = LF/HF), (4) tachograms, and (5) total spectral analysis of the HR and SBP. We also obtained the histograms and the Poincaré plots of the HR in the three conditions. These studies were performed in the morning after a light breakfast. The patients did not use anticholinergics, adrenergics, beta-blockers, coffee, or tobacco before the studies.\nThe HRV was ostensibly decreased in the time domain in the supine and standing position and also during rhythmic breathing (Figures , , and ). The SD was markedly decreased, as were also the maximum/minimum ratio, the range, and the variation coefficient (VC) (). The analysis of the HRV in the frequency domain, in the three conditions, showed a decrease in the absolute power of LF and HF. The increase in SBV was due to the decrease of both the LF and the HF, although the HF was most severely decreased ().\nIn , the normal values of the SBPV are depicted in the time domain together with the patient's values. The patient's values are within normal limits which suggest normal innervation of the blood vessels. In , the values in the frequency domain in normalized units are shown in the three conditions; the patient's values are within normal limits. |
A 62-year-old female patient with a medical history of endometrial adenocarcinoma, whose primary tumor resection 5 years earlier, experienced tumor recurrence with an abdominal mass measuring 12 × 15 × 10 cm, along with metastatic lesions to the vertebral body, complicated by multiple segmental compression fracture. She presented to us with severe pain, refractory to conventional oral medical pain management, including oxycodone 120 mg q12 hours, gabapentin 900 mg tid and flurbiprofen axetil 50 mg bid. There was no prior history of epilepsy or other neurological diseases. Subsequently, intrathecal drug delivery system was implanted with the tip of the intrathecal catheter placed at the T7 level at 1 hospital in Zhejiang Province. The patient was given an external PCA pump contained 1 mg/ml morphine in normal saline, with the infusion rate set at 1 ml bolus injection each time, with a 2 hours lockout interval. The total intrathecal dose of morphine was about 3 mg per day at first, and then gradually increased with titration to pain control. Unfortunately, her pain persisted even when the morphine dosage was increased to 12 mg/d by 1 month later. She was then offered adjustment by adding ropivacaine 1.25 mg/ml to the 1 mg/ml morphine solution, as well as adding continuous infusion at 0.2 mg/h in addition to the bolus injection at 1 ml each time, with a 1-hour lockout interval. Her severe pain persisted even at a continuous infusion rate increased up to 1.0 ml/h, along with a 1.5 ml bolus injection with a 1-hour lockout interval over the next 2 weeks. Spinal myoclonus occurred at the time the total dose of morphine reached 45 mg and ropivacaine reached 22.5 mg per day. Frequent symmetric spastic contractions in the lower limb muscles began. The duration of spasms was 6 to 8 seconds on average and occurred at about every 30 seconds. Uncontrollable twitching and jerking of the muscles exacerbated her pain and caused further distress. The patient's heart rate, respiration rate, oxygen saturation, and muscle strength remained stable. Laboratory findings included a complete blood count, metabolic panel, hepatic and renal function, and electrocardiogram were recorded and showed normal findings. Midazolam 10 mg total and sodium valproate 0.4 mg/40 ml was given immediately through slow intravenous injection without improvement. Subsequently, her intrathecal infusion dosage was reduced to half and her spasm subsequently resolved. Additional intravenous medication through a morphine PCA was then added to assist in control of her pain, along with her intrathecal morphine infusion set to below 30 mg per day and ropivacaine to ≤15 mg per day, until the patient died from multiple organ failure 3 weeks later. |
Patient 7 is a 37-year-old female who presented to the W-TBI clinic in July 2017 after suffering a motor vehicle accident in which they did not lose consciousness. At her initial appointment, the patient reported having constant headaches since the accident, with shooting, throbbing pains in her head, photosensitivity, difficulty driving, fatigue, poor memory, and trouble reading. She reported that her headaches were more severe during the night but improved after standing upright. Her ROS was positive for fatigue, nausea, easy bruising, debilitating headaches, neck and shoulder pain, hypermobility (Beighton score 5/9), low blood pressure and dizziness when standing, and cognitive impairment. Her past medical history was significant for melanoma, breast cancer, and heavy menstrual periods. The patient was diagnosed with postconcussion syndrome with potential elevated ICP. The patient was prescribed home orthoptic exercises and recommended to sleep in the reverse Trendelenburg position for a few nights to assess for the possibility of increased ICP, since she awoke every morning with headaches and a sense of pressure in the head. Prior to re-evaluation, the patient reported that her headaches improved in the morning after sleeping in the reverse Trendelenburg position, and so an MRI with contrast was ordered to explore findings that might be consistent with elevated ICP; the MRI showed some decrease in ventricle size, possibly consistent with intracranial hypertension, but was otherwise unremarkable. The patient was offered a trial of acetazolamide in August 2017 and she reported improvement initially with this medication but then symptoms of headache and blurred vision returned. The patient returned to the W-TBI clinic in October 2017 and noted that she had worsening nausea when trying to read or use the computer and could not complete home orthoptic exercises without vomiting. Her review of systems was positive for fatigue, frequent nausea, headaches, easy bruising, hypermobility, low blood pressure and dizziness when standing up, and cognitive impairment. The patient was diagnosed with possible cervicogenic headaches and possible occipital neuralgia, convergence insufficiency, intracranial hypertension, and post-concussion migraines. She was offered brock string exercises and also referred to a pain management specialist for her neck pain. Prior to her return in January 2018, the patient received occipital nerve blocks by pain management which was unsuccessful. The patient reported inability to tolerate Brock string exercise for more than a minute at a time, with minimal improvement in reading or memory and other cognitive tasks; ROS was also unchanged. Because of the patient’s minimal improvement, history of hypermobility), and easy bruising, the patient was suspected to have a hypermobility disorder such as Ehlers Danlos. She was referred to a geneticist who confirmed the diagnosis of EDS hypermobile type III in September 2018. At a follow up in the W-TBI clinic, the patient demonstrated unimproved convergence, smooth pursuits, and stereoacuity, suggesting she had reached maximal medical improvement. |
A five-year-old typically developing female was referred by her primary care provider for evaluation of a rash reported to be sensitive to sunlight and had been reoccurring for the last 4 years. Cutaneous exam revealed fine scale on her trunk and extremities as well as small pink flat papules on lower left leg. She was diagnosed with ichthyosis vulgaris and provided with a treatment plan. She was subsequently referred to gastroenterology for decreased appetite. Two weeks later the child presented to the Emergency Department for right knee pain. Her mother reported her daughter had experienced bilateral lower extremity pain and had been limping. During the evaluation she was able to walk for the physician without reporting pain and no tenderness was reported with palpation. The mother reported her daughter was bitten by a tick about 1 year ago and that no workup or treatment was conducted. Radiographic scans of the right knee and leg showed no abnormalities. Laboratory testing for Lyme disease was conducted and found to be negative. She was discharged with the diagnosis of tenosynovitis. The child presented again to the Emergency Department the following week for left knee and ankle pain. She reported pain in her left calcaneus when asked to bear weight. Migratory arthralgia was noted and further laboratory testing, including a vitamin C level, was ordered. Her vitamin C level was found to be < 5 umol/L (reference range: 23 to 114 umol/L). Her prealbumin level was also low, 14 mg/dl (reference range: 17–36 mg/dl) as was both her ferritin 3.9 ng/ml (reference range: 6.2–137.0 ng/ml) and her iron saturation 15% (reference range: 20–55%). Both vitamin A and vitamin D were found to be within the normal reference range. With the diagnosis of the vitamin C deficiency, the child was started on ascorbic acid and referred to the feeding program.\nThe participant was the youngest of six children and raised by her biological parents in a middle-class household in a small town. She was born full term by caesarian section with a birth weight of 3.88 kg. There were no prenatal or postnatal complications. No delays in development were noted by the primary care provider or parents. She scooted at 6 months and walked before 1 year. No problems were noted with her gait until several weeks prior to the initial visit to the Emergency Department for knee pain.\nAt 1 year of age, she transitioned from infant formula to milk and cereal snacks. She never accepted baby food. For 4 years after transitioning off infant formula her diet consisted of cereal snacks, one type of cracker, and, inconsistently, chocolate pudding, vanilla ice cream, chocolate, and banana. Except for the occasional banana, she never ate fruit, vegetables, or meats. She drank skim milk, water, and, rarely, soda. She refused to taste new foods or drinks. Additionally, at the time of her diagnosis with vitamin C deficiency, her body mass index was at the 1st percentile. Based upon her inadequate growth and extremely limited diet, she was admitted to an intensive day treatment feeding program. During the course of intensive treatment she learned to eat 29 foods from all food groups through the use of an intervention involving gradual repeated exposure to novel foods []. In 6 months after intensive treatment, her weight had increased by four kilograms, her height increased by three centimeters, and her body mass index reached the 61st percentile. At 1 year after completion of intensive treatment, her height had increased by 7.6 cm and her weight had increased by seven kilograms Her body mass index reached the 85th percentile (see Fig. ). Across the span of the 1 year after intensive treatment she continued to be monitored as an outpatient by a feeding therapist who continued to address the child’s diet variety and helped the family maintain the gains made during intensive treatment. At all outpatient appointments, a meal was conducted allowing the therapist to verify the child’s consumption of a variety of foods.\nHow unusual was this case?\nTo determine how this case compared to the existing literature, articles describing cases of scurvy secondary to diet limitations published from 2000 to 2018 were reviewed. PubMed and Google Scholar were searched using the search terms, “scurvy” and “ascorbic acid deficiency”. The reference section and citation listing of each article identified from these searches were then examined to identify additional articles. Sixty-one articles describing either a case study or a case series reported on 77 children diagnosed with scurvy as a result of diet insufficiencies (see Additional file for an alphabetical listing of all studies). Selected demographics from these studies are shown in Table . While the child in the current study was only slightly younger than median age as the children in the existing literature, she differed from most of these children who were predominantly males with special needs, most often, autism spectrum disorder. Of the 77 reported cases, only six were females without special needs.\nWhile all 77 children reported in these studies had limited diets, it is not clear that all could be described as selective eaters or to what degree their diets were the result of refusal to eat a variety of foods. Six of the children exhibited oral motor dysfunction which limited the range of foods they could eat [, ]. While some, or possibly all, of these six children might have obtained sufficient nutrition by consuming lower texture foods, oral supplement, or multi-vitamins, it is probable their parents did not know how to modify their diets to match their oral motor limitations. There were also three children dependent upon tube feeds who either received an insufficient amount of enteral formula [] or received homemade tube feeds deficient in vitamin C []. There were other children whose diets were not adjusted to meet their specific health needs, for example, one child receiving a ketogenic diet was not receiving any vitamin supplementation [] and another child received only a limited diet due to multiple food allergies []. For various reasons, the parents of 18 children, (five children with typical development, 14 children with special needs other than autism) limited their diets to the point these children developed vitamin C deficiency. Of the nine children with typical development with parent-limited diets, all but two were less than 2 years of age.\nFifty-nine of the children described in the existing literature could be described as selective eaters whose scurvy resulted from their limited intake. Of these children, 41% had autism spectrum disorder, 31% had intellectual disabilities, and 86% were male. Four of these 59 children were females with typical development like the girl in the current study. The child in the current case study had a diet similar to the diets of these 59 children identified as selective eaters in the existing literature on vitamin C deficiency. None of these children reportedly consumed vegetables or fruits, most consumed only starches and dairy products, with a few eating a limited number of proteins. The child in our case study was anemic, as were 42% of the children in the existing literature, an expected finding given the role of vitamin C in iron absorption. While the child in our case study did not exhibit signs or symptoms indicative of additional nutrient deficiencies, other deficiencies were considered likely so she was placed on a multivitamin within a day of the initial diagnosis of vitamin C deficiency. Her pediatrician conducted further testing and found her vitamin A and vitamin D levels were within the normal range. Of the 59 children described in the existing literature, 22% were identified with an additional nutrient deficiency beyond anemia (e.g. vitamin A, vitamin D). Given the diets reported for these 59 children, it is possible, if not probable, that more of these children had other nutrient deficiencies, but further deficiencies were either not reported or not identified.\nThe girl described in our case study had a body mass index at the 1st percentile prior to her feeding treatment. Underweight was also a common problem among the 59 children in the existing literature, with 32% being described as underweight. It is not surprising so many of these children were underweight. Many of children had anemia, which decreases appetite and eating was no doubt uncomfortable or even painful for many of these children, 71% of whom exhibited gingival symptoms. |
A 41-year-old man presented to our Neurology Department with pain at the level of the right shoulder and right interscapular–vertebral region, with onset for about 6 months. In the last 30 days, the pain exacerbated and radiated in the anteromedial part of the arm and clavicular area. The patient also reported pain in the joints of the distal part of upper and lower limbs, especially in the small joints, which afterward became swollen.\nThe patient presented with a medical history of arterial hypertension for the last 10 years, treated with beta-blockers and sartans, and he was a heavy smoker (in the last period he used only electronic cigarettes).\nThe neurologic examination was normal, except for slightly diminished deep tendon reflexes of the lower limbs. The patient reported pain that did not correspond to any radicular or nerve territory, which was exacerbated by pressure on the distal third of radius and ulna, but was not related to active or passive movements of the cervical spine. The general examination showed clubbing of the fingers and toes (not mentioned initially by the patient, but present for about 1 year) with an increase of the shoe size of more than 1.5 sizes, enlargement of the large joints, swollen extremities with a tubular appearance (Figs. and ), a right lateral cervical mobile, painless adenopathy of 1 cm diameter, and pachydermia with thickening of the skin of the scalp, forehead, and fingers, with cranial skin folds.\nCervical magnetic resonance imaging performed in another clinic before the admission to our department showed the presence of disc protrusions from C2 to C7, with concomitant C2, C4, and C5 root compressions. The electrophysiological study revealed an active denervation in the territory of the right C5, C6, and C7 roots.\nThe radiographic examinations of the upper and lower limbs depicted symmetric osseous abnormalities, typical for periostosis, and linear halving of the diaphysis with an increase in the bone circumference. There were no fractures or cortical destruction (Figs. and ). Transthoracic echocardiography and electrocardiography were normal.\nThe computed tomography (CT) of the thorax, abdomen, and pelvis revealed the presence of a 23/19 mm (transverse) and 20 mm (cranial–caudal) mass lesion, respectively, having speculated margins, contrast enhancement, and areas of necrosis at the level of the dorsal segment of the right upper pulmonary lobe. The mass presented several extensions to the pleura and determined pleural invasion (Figs. and ). In addition, multiple mediastinal adenopathy were noticed, and some of them presented central necrosis. No oncologic abnormalities were found at the examination of the abdomen and pelvis. Also, the cerebral CT scan did not show any pathological findings.\nLaboratory findings were normal, except for the presence of inflammatory syndrome, slight anemia, and high values of vascular endothelial growth factor (VEGF) (1291 pg/mL, with normal values less than 100 pg/mL).\nThe patient was admitted to the Pneumology Clinic, where biopsy from the lateral cervical adenopathy was performed (tumor excision was not an option, due to the presence of multiple adenopathy). The anatomopathological examination of multiple fragments from the lymph node revealed multiple neoplastic infiltrates, suggestive of adenocarcinoma metastasis.\nPositron emission tomography with 2-deoxy-2-[fluorine-18]fluoro-D-glucose confirmed the presence of the pulmonary malignant tumor with multiple active adenopathy.\nBased on the clinical examination that revealed signs and symptoms suggestive of Pierre Marie–Bamberger syndrome, the X-ray examination that showed abnormalities compatible with the same pathology mentioned earlier, and on the positron emission tomography and histologic findings, the diagnosis of pulmonary adenocarcinoma with lymph nodes metastases and paraneoplastic hypertrophic osteoarthropathy was established.\nThe patient received treatment with nonsteroidal antiinflammatory drugs and opiate analgesics, which relieved the pain without any adverse event; he was then referred to the Oncology Department for further treatment of the primary pathology. The patient was treated with different types of chemotherapeutics, immunotherapy, and gamma-knife radiotherapy for cerebral metastases. Unfortunately, the disease progressed despite all of these therapeutic measures and the patient died 9 months later. |
A 47-year-old man (an employee of the hotel sector from Bogotá) with a past medical history of polycystic kidney disease presented to the emergency room with complaints of one week of erythema and purulent drainage of the anterior chest wall associated with a fever of up to 38.5 °C. He received cephalexin during a three-day course without any improvement.\nUpon physical examination, he was in no acute distress, and his skin exam was remarkable for an erythematous left periareolar nodule with swelling, warm, and purulent discharge (), along with signs of systemic inflammatory response without clinical findings of sepsis organic dysfunction (). The patient started on therapy with intravenous clindamycin. However, he deteriorated due to the worsening of chronic kidney disease with the further requirement of renal replacement therapy.\nThe patient was taken to surgery, where a 15-cm abscess with skin necrosis over the area of renitence was found. The cytochemical results of the abscess showed abundant leukocytes and the isolation of Chromobacterium violaceum (). Antibiograms were not made, and blood cultures were negative. The patient continued intravenous clindamycin with no improvement at the third day of admission, so doxycycline was started at a 100 mg BID dosage with partial response. At day five of admission, he was taken to another surgical procedure, and negative pressure therapy was placed over his wound. This therapy allows one to draw fluid from an infected site using a vacuum system, which is especially useful for deep, complicated, and nonhealing wounds. The antibiotic was again changed to tigecycline at a 50 mg BID dosage for ten days.\nAfter five days of tigecycline, he underwent surgery again, negative pressure therapy was withdrawn, and the wound was covered with a fasciocutaneous flap. He was discharged after completing 10 days of antibiotic treatment, with no signs of systemic inflammatory response and with adequate closure of the skin wound (). |
An asymptomatic 72-year-old woman with a history of hysterectomy without ovariectomy underwent a pelvic ultra-sound that revealed a cystic formation on the left ovary. Pelvic magnetic resonance (MR) confirmed the diagnosis of a left ovarian cyst and revealed a solid expansive mass in the presacral area that was ~6 cm in diameter, had a lobulated morphology, was strictly adherent to the sacral periosteum, was mainly adipose but exhibited an extremely non-homogeneous signal intensity (Fig. ). The MR framework did not exclude the diagnosis of a malignant neoplasm such as a liposarcoma. The patient also underwent a contrast-enhanced CT scan that revealed the presence of an elongated formation of mainly adipose density (but with multiple areas of structural non-homogeneity) that was adherent to the presacral fascia (without bone infiltration) and exhibited a modest enhancement; the presence of multiple areas of structural non-homogeneity and modest enhancement precluded the exclusion of a malignant liposarcoma (Fig. ). The risk that it was a malignant neoplasm contraindicated the execution of a CT-guided biopsy.\nConsidering the need to remove both the ovaries and the presacral mass, we opted for a laparoscopic approach. Upon laparoscopic exploration of abdominal cavity, the cystic formation of the left ovary was confirmed, and a bilateral ovariectomy was performed due to the age of patient. After mobilization of the sigmoid and intra-peritoneal rectum, the pelvic space and the presacral area were exposed to gain access to the lesion that was observed on the MRI and CT. The mass was multilobulated and well-encapsulated. The mass appeared to be easily dissociable from the mesorectal fascia, but it was tenaciously attached to the presacral fascia and strongly suspicious for neoplastic infiltration. An en bloc removal of the neoplasm with clear margins was performed, and the portion of the presacral fascia that was tenaciously adherent to the mass was included in the exeresis. After the removal of the neoplasm, the site of the exeresis appeared to exhibit significant bleeding; therefore, we performed hemostasis by placing hemostatic material (oxidized and regenerated cellulose topical hemostatic dressings).\nPathological examination revealed mature adipose tissue mixed with hematopoietic tissue, which included erythrocytes, myelocytes and megakaryocytes (Fig. ). There was no evidence of malignancy. These findings were consistent with a presacral myelolipoma. |
An 82-year-old woman was electively admitted for left TKR. Her medical history included acute myocardial infarction 10 years prior, as well as hypertension, left total hip replacement and a stable, 4.5 cm ascending aorta aneurysm.\nShe was taking the following medications: telmisartan 40 mg once a day, aspirin 100 mg once a day, metoprolol 50 mg two times per day, rosuvastatin 10 mg once a day, rabeprazole 20 mg and lercanidipine 10 mg. Her preoperative anaesthesia assessment included an ECG which showed a right bundle branch block, as well as a chest X-ray and transthoracic echocardiogram, which showed borderline left atrial diameters, normal left ventricular size and systolic function, and an estimated ejection fraction >50%. There were no overt regional wall motion abnormalities. Mild degenerative changes of the aortic and mitral valves were observed, along with mild aortic valve insufficiency. The ascending aorta was dilated (approximately 5 cm). There were no respiratory symptoms, and spirometry did not show significant abnormality.\nThe patient underwent TKR. On the first day after the operation, she developed dense right arm weakness and left homonymous hemianopia. There were no other obvious focal neurological deficits. CT and brain MRI showed innumerable T2 FLAIR hyperintensities, demonstrating high diffusion-weighted imaging signals that were consistent with multifocal acute infarcts. These were predominantly cortically/subcortically distributed in both the right and left cerebral hemispheres within the frontal, parietal and occipital regions; additionally, there was a small focus within the left cerebellar hemisphere, favouring a central embolic cause. The patient was managed according to an acute stroke unit pathway, moved to a bed with telemetry monitoring and clopidogrel was added to her medications.\nFollowing surgery, the patient complained of increasing shortness of breath and was noted to be hypoxic on room air, up to 86%. A physical examination did not reveal any other significant findings. Her blood tests remained within normal ranges. CT pulmonary angiogram did not show pulmonary embolism; however, it showed left lower lobe atelectasis and evidence of right ventricular strain. The contrast was seen refluxing into the hepatic veins, which was consistent with right atrial dysfunction. During admission, progressive dyspnoea at rest prompted several emergency calls and subsequent admission to a high-dependency bed. Extensive investigations were undertaken to determine possible causes of her symptoms, including sepsis, pulmonary embolism, pulmonary hypertension and heart failure. Blood tests remained unchanged, apart from a transient rise in serum troponin, which was treated as NON-ST elevation myocardial infarction for medical management only. CT of the pulmonary artery and transthoracic echocardiography were repeated, which showed normal right ventricular size with moderate systolic dysfunction, moderate right ventricular hypertrophy and significant pulmonary hypertension. No intracardiac thrombus was visualised. Two weeks postsurgery the patient achieved marginal improvement, requiring 2 L of oxygen via nasal prong; therefore, she was transferred to the rehabilitation ward.\nInterestingly, in the rehabilitation ward, emergency calls were activated repeatedly, daily in the morning for persistent hypoxia; her symptoms and oxygen requirement worsened when she sat in a chair, compared with lying flat in bed; moreover, standing caused a significant drop in oxygen saturation, which prevented her participation in the physiotherapy programme (). |
A. P., a 61-year-old woman, was taken to the emergency ward of the Tehran Heart Center, Iran, by her husband and son. On admission, she was suffering from dyspnea, and there was a sewing needle penetrating her chest. During the physical examination, the heart and lungs were normal. Emergency fluoroscopy, echocardiography and computed tomography (CT) scan were performed. The chest CT scan revealed that the needle had penetrated into the heart, and echocardiography demonstrated that the needle had ruptured the pericardium, but it had not entered the left ventricle. The patient underwent surgery 4 hours after admission, and the needle was removed successfully.\nAn interview with her family disclosed a history of major depressive disorder and pharmacological treatment. During the first 3 sessions of psychiatric counseling after the surgical operation, the patient denied a suicide attempt. She claimed that an accidental fall was the cause of her injury. On the 4th session, however, she admitted that the problem was not accidental, stating that she had attempted unplanned impulsive suicide by swallowing needles twice before, once 10 years and once a year previous to this. In both cases, she had been hospitalized for some days and discharged without any complications.\nThe patient had been suffering from major depressive disorder since the sudden death of her son, 10 years ago. During the previous 5 years, she had received different pharmacological treatments, namely clonazepam, alprazolam, venlafaxine, and Depakine, which were not followed through. No history of psychotherapy or electroconvulsive therapy was reported.\nThe patient reported having experienced auditory hallucinations during different periods of taking low-dose benzodiazepine pills. According to her family, during these periods, the patient had experienced disorientation, disturbed consciousness, distraction, and auditory hallucinations. Through these accounts we were guided toward the delirious picture of those episodes in her past. She also self-medicated with benzodiazepine pills in those periods reported by the family, as she was not receiving adequate attention from them.\nThe family members described her as a demanding and irritable person who needs a great deal of attention, and is also prone to impulsive behavior when under stress or not receiving adequate attention.\nAfter thorough examination, venlafaxine was prescribed as antidepressant. The patient should have remained hospitalized until the completion of the treatment, but the patient as well as her family members consented to leave the hospital after 5 days despite the advice of the medical team. Efforts to convince her to adhere to the course of treatment failed due to her refusal to do so. |
The patient is a woman in her early fifties. She was diagnosed with MS after a sensory attack in the left shoulder in 2008 and a sensorimotor attack in the right leg in 2013. She was treated with interferon beta 1a from April 2013, and with fingolimod from September 2013 after a motor attack in the left leg from which she recovered partially. Treatment was changed again to natalizumab in January 2014 when macula edema was suspected. She remained clinically and radiologically stable until natalizumab was terminated in the beginning of June 2015, after she tested positive for John Cunningham virus. Alemtuzumab was started at the end of July 2015. During the first cycle (12 mg for 5 days) she had transient sinus bradycardia down to 30 beats per minute but no other adverse events.\nThe patient remained clinically stable with an expanded disability status scale (EDSS) score at 2.5 until the second cycle (12 mg alemtuzumab preceded by 1000 mg methylprednisolone, 12 mg cetrizine and 1000 mg paracetamol for three consecutive days) in July 2016. Except for transient bradycardia there were no immediate adverse reactions, but some hours after the last infusion of alemtuzumab she became sick with nausea and fever up to 40 °C. At admission to hospital she was awake and did not have neck stiffness or other focal signs except abdominal discomfort and mild headache. She was febrile (39.5 C) and clinically dehydrated but normotensive. C-reactive protein was 180, lymphocytes were below the detection limit but the number of granulocytes was normal. As she did not have new neurological symptoms, neither detailed neurological examination, brain imaging nor lumbar puncture were performed. Four out of four blood cultures were positive for L. monocytogenes (confirmed by 16S RNA sequencing) which was susceptible to trimethoprim-sulphamethoxazole, ampicillin, erythromycin, meropenem and penicillin. She recovered rapidly and completely upon treatment with ampicillin and trimethoprim-sulphamethoxazole. |
A 32-year-old-woman was referred to our hospital with 39.4 weeks of gestation for evaluation by the pediatric surgery service of a female fetus with a large mixed echogenic mass located in the oropharyngeal region. The mass was initially discovered in the 36th gestational week with a 2-dimensional (2D) sonogram. The medical history revealed that the parents were healthy, non-consanguineous, and did not have a family history of congenital anomalies.\nThe pediatric evaluation included 4-dimensional echography that revealed the presence of a tumor that occupied the entire oral cavity and protruded from the mouth, as well as the presence of polyhydramnios.\nDue to the nature of the tumor, its anatomical location, and dimensions, the pediatric surgery service together with the gynecology and obstetrics service planned a cesarean section and an ex-utero intrapartum treatment (EXIT) procedure as a first option.\nThe large dimension of the tumor () made it necessary to secure the airway at birth by tracheostomy. A female infant of 2,740 g was born with a height of 47 cm, an Apgar score of 7/9, and no apparent motor or sensory alteration. On examination, mild hypertelorism was identified, as well as an incomplete right cleft lip. The tissue distension produced by the tumor caused a pseudomacrostoma of the right side. The tumor practically occupied the oral cavity and protruded from the mouth; its aspect was rough and irregular. With a tongue depressor, it was seen that the mass occupied the hard palate, also finding a fissure in the soft palate on the right side. The pediatric surgery service decided to only resect the extraoral tissue for study.\nOnce the newborn was stabilized after extracorporeal collection, she was hospitalized in the pediatric intensive care unit. Based on the previously mentioned characteristics and the great heterogeneity that these types of lesion represent, a consultation from the plastic surgery service was requested for integral management at a second surgical time. A pathology study reported a partially resected mature teratoma derived from the three germ layers.\nDuring her stay in intensive care, a computed tomography (CT) scan was performed which reported that the residual tumor had irregular borders and dimensions of 0.9 × 2.2 × 3 cm, predominantly occupying the hard palate, in addition to great heterogeneity in the density of its tissues. The evaluation was complemented with magnetic resonance imaging (MRI) because of the finding of polyhydramnios. In the intentional search for synchronous alterations of the central nervous system, a nasoethmoidal meningocele and the absence of the cribriform plate were identified; this conditioned the hypertelorism. The morphology and density of the brain parenchyma were conserved with adequate differentiation of the grey and white matter, grey nuclei, and normal middle-line structures.\nAfter the assessment of the newborn with residual tumor () by our plastic surgery service, we opted to plan her surgical treatment using virtual image reconstruction (3D CT) to more precisely establish the extension, depth, and relationship of the tumor with surrounding tissues (). Based on this large 3D perspective, we were able to establish exactly the limits of the tumor, and therefore the free millimeter margin to be resected.\nAs a complement, we manufactured a 3D stereolithographic model of the facial skeleton to scale, which allowed the team members to become more familiar with the surgical act. 3D visualization of the tumor margins was essential, as well as the residual defect that would have to be reconstructed ().\nOn day 12 after birth, under general anesthesia, a bloc resection of the tumor was performed. This left a palatal soft-tissue defect of 0.7 × 0.9 mm and a small bone defect of 0.2 × 0.3 mm due to tumor compression on the hard palate; it was possible to cover the defect by a contralateral rotation of a gingival periosteum flap. The great soft-tissue distention caused by the tumor during its growth deformed the anatomy of the cleft of the lip; due to these characteristics, we only perform lip adhesion (). The definitive treatment of the cleft lip and pseudomacrostoma was deferred for a second procedure.\nThe postoperative period was uneventful; the patient was discharged 10 days after the surgical procedure in agreement with the pediatric surgery service, and subsequent clinical evaluations continued in the outpatient clinic. The following 6 postoperative months passed with adequate healthy-child control. The most predominant aspect was that there was no evidence of tumor recurrence. The gradual regression of tissue distention showed a slight improvement in the appearance of the pseudomacrostoma (). Based on this assessment, it was decided to perform the closure of the cleft of the soft palate. This was carried out without complications; in addition to this, a small touch-up of the primary lip adhesion was also performed, which consisted of an aesthetic refinement of the subunits of the central labial area. |
A 40-year-old woman, P 2 living 2, presented in the Department of Gynaecology with the complaint of progressively increasing distension in abdomen for last 1 year. It was associated with pain lower abdomen for last 6 months. Surprisingly, there were no complaints regarding change of menstrual pattern, urinary or bowel habits, anorexia or fever.\nOn examination, a huge abdominal mass occupying whole of the hypogastrium, both iliac fossa, umbilicus, and left hypochondrium, was noted. The mass was 10 × 8 inches in size, smooth surfaced with regular margin, soft to firm in consistency, nontender, side to side mobile, lower limit not reachable, and engaged in the pelvis more toward the left side. There were no ascites clinically. On per vaginal examination, the same mass was felt obliterating all the fornices; uterus was easily palpable of normal size but dextro deviated.\nUltrasonography revealed a huge complex mass arising from the pelvis and extending into the abdomen occupying almost whole of the abdomen with well-defined margins; complex internal echotexture, predominantly echogenic with multiple poorly hypoechoic areas along with two cystic areas, each measuring around 4 cm × 5 cm in size. Doppler sonography showed minimal blood flow to the tumor. Both the ovaries could not be visualized. A normal size uterus was seen pushed to the right side with normal central endometrium. Free intraperitoneal fluid or lymphadenopathy was absent. CA 125 was 5.2 IU. Computed tomography scan was not done due to financial reasons.\nClinically provisional diagnosis of the benign ovarian tumor was made based on Doppler and biomarker CA 125 levels. After preanesthetic work up, the patient was taken up for laparotomy. Per operatively soft mass of size 12 × 10 inches with intact external capsule was seen, growing into the leaves of left broad ligament and extending above the umbilicus. Both ovaries, right tube and uterus was normal but left fallopian tube was stretched over the mass. On opening the broad ligament leaf, it was found to be arising from posterior surface of upper part of cervix. Left ureter was catheterized to avoid injury. Mass was shelled out followed by total hysterectomy with left-sided salpingo-oophorectomy []. There were no pressure changes in ureter or bowel. Omentum and rest of the abdominal organs were normal. Cut section showed few solid areas along with multiple cystic areas, one containing black tarry material suggestive of red degeneration. Postoperative period was uneventful.\nHistopathology of the mass revealed benign spindle cell tumor (leiomyoma uteri) with red, cystic, myxoid and hyaline degenerations and dystrophic calcification []. The definitive diagnosis came out to be cervical fibroid with marked degenerative changes. |
A 46-year-old paraplegic woman presented to the outpatient urology department, complaining of recurrent febrile urinary tract infections and severe urinary incontinence for 2 years. She suffered from persistent malodorous urine and skin breakdowns from constant urine leakage. She typically used long-term urethral catheters, which had resulted in dilatation and pressure necrosis of the urethra with subsequent severe incontinence.\nThe vaginal examination revealed destruction of the urethra and a 10 cm opening permitting the urinary bladder wall to prolapse into the vagina (Figs. , ). Three fingers could be easily inserted into the urethra, and the bladder wall could practically be observed from the outside. A diffuse scar tissue area had replaced bladder trigone, and ureteral orifices were visible through the bladder opening. An intravenous urography was performed to check the anatomy of the upper urinary tract, which was within normal limits. The computed tomography (CT) scan did not assist in the diagnosis because of the artifacts caused by the bilateral total hip replacements.\nThe patient underwent a combined transvaginal urethral closure with anterior colporrhaphy and a Mitrofanoff procedure to ensure a continent stoma for future CISC. The patient was placed initially in the dorsal lithotomy position, and a retractor was placed in the vagina. The labia were retracted laterally with stay sutures. The damaged bladder wall was circumcised, and an anterior vaginal flap was raised. Special care was taken to entirely free the lateral part of the bladder base and the attachments of the pubic bone. There was extensive destruction of pubourethral ligaments, and that allowed a complete mobilization of the bladder wall from the symphysis pubis. The opening was closed with a 2/0 polyglactin (Vicryl™) suture. A second layer, using the same suture, was applied to invaginate further the bladder opening to ensure an excellent postoperative result. An anterior colporrhaphy was accomplished to push anteriorly further the bladder (third layer). Finally, the anterior vaginal flap was advanced to cover the area where the previous urethra and bladder neck were present, forming the last (fourth) layer of closure.\nA laparotomy followed immediately after that, and an appendicectomy was done, maintaining its blood supply. The tip of the appendix was transected, and a catheter was introduced to ensure patency. A Mitrofanoff procedure was performed, and the appendix brought out through umbilicus. A small cystostomy was made, and the bladder mucosa was prepared to accomplish an appendico-vesical anastomosis. Recovery from this procedure took six weeks. Initially, the Mitrofanoff channel was not used for draining urine. During this time, a suprapubic catheter was inserted to drain urine. The suprapubic catheter was removed once CISC through the Mitrofanoff channel started. The suprapubic catheter opening closed easily.\nDuring the initial postoperative period, the patient received oxybutynin for three months. The patient is compliant with CISC and remains continent twelve years after surgery. She has a normal renal function with no upper tract dilatation on ultrasonography. The woman underwent evaluation recently, and she is still dry with no urinary fistula reported (Fig. ).\nThe patient reported complications concerning recurrent urinary tract infections and urine leakage when delays the emptying. She performs four CISC per day and undergoes annual evaluation with an ultrasound examination to ensure that any stones that may have formed are removed. |
In 2011, a 42-year-old female was diagnosed with a large mesenteric DT with a CTNNB1 T41A somatic mutation (). The mesenteric tumor was intimately related to the superior mesenteric vessels, and due to its size and location, was not considered surgically resectable without major morbidity (sacrificing most of her small bowel and/or considering a small bowel transplantation). The patient was treated with imatinib with no response and then received 2 subsequent lines of therapy (tamoxifen and doxorubicin) without any evidence of an objective response to either agent. Approximately 14 months after her initial diagnosis, after failure of these therapies, the patient was enrolled into a phase 1 trial of AL101 at a dose of 8.4 mg once weekly (QW) on a 4-week cycle. AL101 was reasonably well tolerated; most adverse events were grade 1/2 and included diarrhea and fatigue. The diarrhea fluctuated but remained at a grade 1 or 2 level and was managed with antidiarrheals, when required. On cycle 21 day 22, the patient developed grade 3 diarrhea; therefore, because the diarrhea was intermittent before this but longstanding, the dose of AL101 was reduced to 6 mg QW for cycle 22. Grade 3 diarrhea was short-lived and resolved with intervention and dose modification. During cycle 26, the dose of AL101 was further reduced to 4 mg QW due to ongoing fatigue, lymphopenia, albuminemia, and intermittent episodes of diarrhea. The patient received AL101 during the clinical trial for approximately 4.6 years (60 cycles). At the close of the trial, the patient was transferred to a compassionate use program during which the patient received two additional doses. The patient achieved a PR per Response Evaluation Criteria in Solid Tumors (RECIST) version 1.1 at cycle 14, after approximately 1 year of treatment with AL101, and this response was maintained for more than 3.6 years while participating in the phase 1 trial ( and ). The maximal response was a 41% decrease in the longest diameter tumor (a decrease from 178 mm to 105 mm). The patient elected to discontinue AL101 treatment after 4.6 years, with follow-up continuing. This decision was a personal choice made in consultation with the treating team. The decision was based, in part, on a plateau in disease response, burdensome ongoing intravenous therapy for which travel was required, and mild toxicities, but troublesome considering the good disease control. The prospect of restarting drug/rechallenging remained open to the patient. Hence, the decision to initially hold and then cease treatment was made. Off treatment, this patient has maintained a PR for an additional 4 years, with an overall duration of response of 8.6+ years. The patient remains on active surveillance. |
The patient is a 57-year-old male with an extensive smoking history who underwent right upper lobe lobectomy in May 2012 for a clinical stage I adenocarcinoma of the lung. He was found to have microscopic ipsilateral mediastinal adenopathy. He received adjuvant chemotherapy with pemetrexed and cisplatin followed by radiation therapy for his pathologic stage IIIA (pT2aN2M0) adenocarcinoma of the lung. A positron emission tomography (PET) scan in February 2013 did not show any evidence of malignancy.\nOne year after completion of adjuvant chemotherapy, in October 2013, the patient developed headaches. Magnetic resonance imaging (MRI) of the brain was consistent with four intracranial metastases. PET/CT scan revealed several subcentimeter metastatic pulmonary nodules. EGFR/ALK/ROS1 testing at that time did not reveal any targetable mutations. He underwent whole brain radiation therapy.\nIn the next two years, the patient had progression of disease (POD) in the lung through several lines of chemotherapy. He also developed CNS progression with three new lesions in December 2014, for which he underwent stereotactic radiation therapy (SRS). Six months later, the patient developed two more intracranial lesions for which he again received SRS. New intracranial subcentimeter metastatic disease was identified in September 2015 which was not amenable to further radiation. Immunotherapy with the checkpoint inhibitor nivolumab was initiated in November 2015 ( and ).\nEosinophil counts dating back to 1998 had always been within normal limits except for a brief period of mild increased eosinophilia after adjuvant chemotherapy in 2012 which spontaneously resolved. Four weeks after initiation of nivolumab, his absolute eosinophil count was noted to be elevated at 2.86 × 109/L; all other hematopoietic cell lines remained unaffected. He had denied any travel within the previous five years and denied any exposure to any known allergens, new products, or new medications. He was asymptomatic, and on physical examination, there was no evidence of skin rash or splenomegaly.\nOn a follow-up visit in April 2016, after eight cycles of nivolumab, the peripheral blood smear revealed markedly increased eosinophils, but no other significant findings.\nFurther work-up of the eosinophilia was performed and was unrevealing. Multiple stool samples were obtained, and testing for culture, ova, and parasites remained negative on three separate occasions as was testing for Clostridium difficile toxin. Serum IgG for Strongyloides and serum QuantiFERON test were negative. Liver function tests, thyroid function tests, cortisol, and B12 levels were also normal. Echocardiogram, cardiac enzymes, and EKG did not reveal any abnormalities. Evaluation with bone marrow biopsy or molecular/cytogenetic testing was not pursued as it was felt that there was a clear temporal association of eosinophilia with nivolumab administration. MRI of the brain in February 2016 revealed continued mild progression of metastatic lesions and a new lesion in the left temporal lobe. The PET/CT at that time was consistent with systemic progression in the bone and lung. Given the degree of eosinophilia and evidence of progression, the decision was made to hold further nivolumab therapy in March of 2016.\nAs the eosinophilia (3.5 × 109/L) was significant and persisted despite discontinuation of nivolumab, the patient was started on prednisone 10 mg daily in May 2016. Repeat MRI of the brain revealed an increase in the size of known intracranial metastases within the infratentorial and supratentorial regions. There was also a new area of edema and gyral-based enhancement with associated mass effect within the superior posterior left temporal lobe; this pattern of enhancement was considered atypical for metastatic disease. The differential included radiation related necrosis, encephalitis/cerebritis, or postinflammatory process.\nGiven the concern for possible immune-mediated encephalitis/cerebritis, the patient was started on dexamethasone 4 mg twice a day at that time which was later increased to 4 mg three times a day, with a resolution of eosinophilia in two weeks. Two weeks later, in June 2016, the patient started developing thrombocytopenia. Evaluation of the peripheral smear confirmed thrombocytopenia with myelocytes, metamyelocytes, few nucleated red cells, and teardrop cells which was felt to be consistent with a possible myelophthisic process from marrow infiltration of the tumor. A repeat MRI of the brain two weeks later revealed a decrease in edema, the mixed response of lesions with some increasing and some decreasing in size, and the greater confluence of the left temporal lobe lesion which however remained stable in size.\nThe next day, the patient presented to the emergency room with worsening shortness of breath. The computerized tomography angiogram of the chest was consistent with marked progression of the disease. Despite treatment with broad-spectrum antibiotics and aggressive treatment in the intensive care unit, the patient expired one week after his admission with the progression of the disease. An autopsy not performed as per the family's request. |
A 29-year-old male was referred to our clinic with an 8-year history of multiple painless scrotal nodules that had progressively increased in size and number. The few 0.5–1 cm subcutaneous papules on both hemi-scrotal tissues were sometimes itchy. The patient denied a history of scrotal trauma or ingestion of any medication before the onset of a papule. There was no urethral discharge, penile ulceration, or inguinal swelling. The patient noticed some of the papules disappeared after discharge of a whitish or chalk-like substance from them. He had no previous history of sexually transmitted infection. As the number and size of the nodules were increasing, he became uncomfortable, embarrassed, and did not want others to see the lesion. He reported that he was not sexually active and had avoided intimate relationships because of his scrotal lesions. There was no history of any chronic illness or medication use. Physical examination showed multiple palpable but painless dark brown intradermal nodules on the scrotum. The calcified nodules involved both hemi-scrotum, but were preponderantly on the left. No other skin lesion was noticed. The phallus was circumcised and the testes were palpable and grossly normal. Laboratory evaluation revealed normal serum phosphorus and a slightly lower calcium level. The patient underwent excision of the lesions near the median raphe under local anesthesia using a single elliptical incision. Other individual nodules on both hemi-scrotums were excised individually and closed separately to preserve some uninvolved skin and to allow for adequate scrotal skin closure. The defects were closed using 3-0 prolene suture interrupted technique (). There were no postoperative complications to date, and the patient had an excellent scrotal cosmetic outcome.\nHistology sections revealed calcium deposition, and basophilic globules of varying sizes and shapes within the reticular dermis. There was an occasional scalloped appearance, with some of the smaller fragments surrounded by multinucleated giant cells and a sparse lymphocytic infiltrate. In between the globules, there were dense collagenous stroma and areas of fibrosis that were seen, and a fibrous capsule surrounded the entire lesion. The histopathologic diagnosis was “ISC”, and no sign of epithelial lining was found in the nodules (–). |
A 27-year-old female with a history of FAP (with a documented APC germline mutation) underwent a planned prophylactic total proctocolectomy and ileoanal pouch approximately 5.5 years ago. This surgery incidentally revealed the presence of multiple DTs (). A right iliac fossa mass was palpable on clinical follow-up 7 months after surgery and computed tomography imaging indicated the likely presence of a solitary 7-cm pelvic mass, which provided evidence for recurrent DT. On multidisciplinary review, biopsy of this tumor was thought to be high risk due to its location, and the diagnosis of recurrent DT was therefore made clinically and radiologically. Tamoxifen and 2 subsequent lines of chemotherapy (dacarbazine/doxorubicin, methotrexate/vinblastine) were administered without significant symptomatic or radiological response. Approximately 4.2 years ago (1 year and 4 months after diagnosis), AL101 therapy was therefore commenced on a compassionate access basis at a dose of 4 mg QW; however, the dose was reduced to 2.4 mg QW due to grade 2 elevated liver function tests. AL101 was further reduced to 2.4 mg every 2 weeks due to grade 3 diarrhea that resulted in dehydration, dizziness, and hospital admission. Grade 3 diarrhea had a sudden onset, occurring within 1-2 weeks of treatment initiation; it was short-lived and resolved with dose modification and without significant medical intervention. AL101 was subsequently reduced to 2.4 mg every 3 weeks because of fatigue and nausea, as well as the burden of coming into the clinic for regular injections. Imaging at cycle 21 day 13, approximately 1.5 years after AL101 initiation, revealed a PR (the longest diameter of tumor decreased from 191 mm to 76 mm, representing a 60% decrease) that has been maintained over the past 2.6 years ( and ). The patient has continued treatment and the PR is ongoing. The patient reported improved quality of life, maintained throughout the duration of the treatment, with minimal side effects and short infusion time. |
The liver graft was from a young male patient, 20 years old, who weighed 57 kg, was 1.78 meters tall, and had a body mass index (BMI) of 18 kg/m2. He was admitted to a general hospital with a subarachnoid hemorrhage and intracranial hematoma on the left side, and he suffered brain death. He had a previous pathological history of SCA and was treated for his anemia with several blood cell therapies and a splenectomy when he was 16 years old. He was receiving ceftriaxone, meropenem, and vancomycin when he was submitted to donor surgery, five days after the neurosurgery. He was under low doses of vasopressors: norepinephrine (0.18 mcg/kg/min) and vasopressin (0.02 mcg/kg/min). The best suitable recipient was chosen by balancing the risk of a hematological disease or thrombotic risk factors associated with the recipient remaining on the waiting list and either dying or dropping out of the list. All laboratory analysis and liver function of the donor were normal. After all analysis and arguments discussed with the transplant team (surgeons, hepatologists, and infectologists), as risk of using SCA graft, probabilities of developing disease, and a few case reports in the literature, on the other hand, the benefits of being an excellent hepatic graft option, the recipient and her family were informed of all risks and probabilities, and a unanimous informed consent decision was made to receive the donor liver and follow with the transplant. The donor surgery was fine and was not associated with any complications. Both of the deceased patient's kidneys and liver were donated to three different recipients in different centers.\nThe liver recipient was a 37-year-old woman, and her blood type was the same as that of the donor. She weighed 54 kg and was 1.65 meters tall. Her BMI was 19.8 kg/m2. She was diagnosed with hepatitis B virus (HBV) cirrhosis and hepatocellular carcinoma (HCC) according to the Milan criteria (2 tumors each with diameter ≤ 3 cm, without extrahepatic and major vessel involvement). Model for end-stage liver disease (MELD) score was 18 and Child-Pugh-Turcotte (CPT) classification was B7. The serum alpha-fetoprotein (AFP) value was increasing recently to the transplant (>200 ng/ml). In the waiting list for liver transplant, the patient performed 3 transarterial chemoembolization (TACE) sessions, initially with completely treated areas but afterwards showing partial treatment (progression). The liver allograft weighed 1.495 kg. The cold ischemia time was 8 hours and 30 minutes, and the warm ischemia time was 38 minutes. The transplantation was performed on September 27th, 2016. The patient received four units of red blood cells, three units of platelets, and eight units of plasma during the surgery. The liver biopsy protocol included preclamping and prerevascularization biopsy with mild siderosis and steatosis, hepatocyte ballooning, rare canalicular cholestasis, and moderate sinusoidal congestion with predominance of drepanocytes ().\nThe postrevascularization biopsy revealed mild ischemia reperfusion injury (grade 2), including apoptosis of hepatocytes and a minor neutrophilic sinusoidal infiltrate. Kupffer cell erythrophagocytosis and scarce sickle cells were still seen in a biopsy performed on the following day (), along with some ischemic areas. The immunosuppression protocol included corticosteroids every day after the anesthetic induction for 6 months. In terms of the glucocorticoid withdrawal regimen, basiliximab 20 mg was administered on the anesthetic induction and on the fourth postoperative day; mycophenolate mofetil and tacrolimus were administered from the 4th day on (due to the institutional protocol and literature we initiated the onset of tacrolimus later due to acute renal failure and using protocol with corticosteroids and basiliximab). The tacrolimus dose (0.10-0.15 mg/kg/day administered twice a day) was adjusted based on the liver blood tests and the blood level 6-10 ng/ml was maintained during this first year.\nThe LT recipient also received antibiotics based on the medical profile of the liver donor: vancomycin and meropenem for seven days plus Amicacina for two days. Furthermore, she received hepatitis B immunoglobulin (HBIG) for seven days and then monthly for a year. She still receives entecavir (ETV) daily for preventing hepatitis B recurrence. As part of the LT protocol, she was submitted to a Doppler ultrasound of the vascular graft anastomosis investigation on the first postoperative day, and no abnormalities were noted. The follow-up of the vascular imaging examination will depend on clinical and laboratory data. Despite her good postoperative evolution, she developed acute kidney failure and she required hemodialysis for three weeks. The cause of acute renal failure and hemodialysis was due to liver transplantation procedure and the baseline renal function somewhat altered. Her recovery of renal function was complete, and she did not require renal replacement therapy anymore. She was discharged from hospital on the 37th postoperative day. Postoperative chemotherapy was not necessary due to good evolution and postoperative oncologic control image (CT) with absence of recurrence in the transplanted liver and decrease in the serum tumor marker (AFP <10 ng/ml). A long-term follow-up of 18 months revealed a good evolution, with normal liver blood tests.\nThis study was approved by the Institutional Review Board and accomplished the entire requisite for studies in humans according to the guidelines of the 1975 Declaration of Helsinki. |
A 36-year-old male was referred to our outpatient clinic for discomfort in the left mandibular second molar under a crown bridge restoration; he experienced slight discomfort while chewing. There was no history of trauma, hospitalization, or medical, endocrine, or systemic disease. Hematological investigations, including a complete blood count and measurement of the calcium, phosphorus, and alkaline phosphatase levels, revealed values within the normal range.\nA clinical examination showed no tenderness upon palpation of the labial tissues over the periapical region of any tooth. There was no tenderness to percussion; however, the patient felt discomfort while chewing with the left mandibular second molar. There was no evidence of adenopathy, paresthesia, or motor nerve deficiency in the head and neck area. A clinical examination revealed moderate oral hygiene and healthy gingival tissues. The mobility of the teeth was within the normal range. An intraoral examination also revealed that the patient was caries-free and had four amalgam restorations and a fixed bridge to replace lost mandibular premolars and molars.\nAll teeth except the bridge teeth were pulp-tested using a refrigerant spray and electrical pulp test, and all responded within normal limits. Periodontal probing was consistently 3 mm or less, and no bleeding on probing was detected.\nInitially, a panoramic radiograph was taken. The radiograph showed evidence of resorption in several teeth, including the right maxillary central incisor, first and second premolars, left maxillary central and lateral incisors, second premolar, and first molar, and the unerupted left maxillary canine (). A decision was made to perform CBCT with 3D reconstruction to obtain a more precise location and definition of the pathological features of the resorption sites. CBCT was performed in all three dimensions - axial, sagittal, and cross-sectional - with a slice thickness of 1 mm by using a Promax CBCT scanner (Planmeca, Helsinki, Finland) together with 3D rendered images. The axial () and panoramic reconstructed images () of the left maxillary first molar clearly showed multiple resorptive areas. The cross-sectional images showed internal resorption of the teeth, including the right maxillary central incisor, first and second premolars, left central and lateral incisors, canine, and right mandibular second premolar (). Further, external resorption was found in the teeth, including the left maxillary second premolar, first molar, mandibular second molar, and right mandibular first molar (). The 3D rendered images were not sufficiently clear to detect the nature or extent of the external or internal resorption of the teeth (). Based on the diagnosis of multiple internal resorption from the panoramic and CBCT images, we decided to perform dental examinations of the patient's family members to assess multiple resorption because of the possibility of a hereditary influence. However, no clinical or radiographic examination could be performed because it was not possible to contact any of the family members. CBCT demonstrated that the lesions were generally larger than was evident from the periapical or panoramic films. No treatment was performed because the patient refused treatment. |
A female patient aged 64 years old presented with chest pain and progressive dyspnea (New York Heart Association Class II). The patient had Ross operation 40 years ago for rheumatic aortic valve disease. Twenty years after the Ross procedure, the patient had aortic valve replacement with a tissue valve and open mitral commissurotomy for concomitant mitral valve stenosis. The patient had concomitant coronary artery disease and stenting of the left anterior descending and the circumflex coronary arteries 2 years prior to the latest presentation. The patient had atrial fibrillation, and the echocardiography showed mild global hypokinesia of the left ventricle, severe mitral and tricuspid regurgitation, severe mitral stenosis with the valve area of 1.1 cm2, calcific aortic tissue valve with moderate aortic stenosis, and severe aortic regurgitation. The ejection fraction was 45%, and pulmonary artery systolic pressure was 50 mmHg. The chest computed tomography scan showed calcific ascending aorta (Fig. ), and the carotid Doppler showed 50% bilateral stenosis. After a multidisciplinary discussion, we decided to implant a transcatheter aortic valve and perform open surgery to replace the mitral and repair the tricuspid valve. The aortic valve was replaced with Evolut R 29 mm (Medtronic Inc., Minneapolis, Minnesota, USA). After 3 months, the patient had mitral valve replacement through a median sternotomy. There was a 1 cm distance between the Evolut R valve and the brachiocephalic artery, and the aorta was heavily calcific, making the aortic cross-clamp not feasible. We decided to perform beating mitral valve replacement under hypothermia (30 °C) with no aortic cross-clamp nor cardioplegic arrest. The aorta was cannulated just below the brachiocephalic artery, and both superior and inferior vena cava were directly cannulated. Transeptal approach was used, and the mitral valve was replaced with Mosaic tissue valve 25 mm (Medtronic Inc., Minneapolis, Minnesota, USA), and the tricuspid valve was repaired with a Tri-Ad semi-rigid ring 26 mm (Medtronic Inc., Minneapolis, Minnesota, USA). The insertion of an aortic root vent was not feasible because of the hostile aorta. The patient had right ventricular dysfunction with difficult weaning from cardiopulmonary bypass, and she required extracorporeal membrane oxygenation (ECMO) support, intra-aortic balloon pump (IABP), and high inotropic support, and the course was complicated by pleural effusion requiring drain insertion (Fig. ). The ventricular function recovered after 1 day of ECMO support, and the patient was weaned from mechanical support gradually. Pre-discharge echocardiography showed 9 mmHg pressure gradient on the aortic valve with a mild paravalvular leak, functioning prosthetic mitral valve, and trivial tricuspid regurgitation. The hospital stay was 33 days. |
A 75-year-old man visited our hospital because of exacerbation of lower extremity edema that he had noticed 3 months before admission. He had received annual medical checks and had no specific medical history. His body weight had increased by 10 kg over the past 5 months, and his systemic edema was remarkable. Urinary protein was 9.9 g/day, no microscopic hematuria was detected, and his serum creatinine was 1.15 mg/dL on admission. The serum protein fractions showed an M peak at a high level and serum IgM was 1657 mg/dL. Protein electrophoresis showed IgM κ type M proteinemia. Although his monoclonal IgM was increased, bone marrow biopsy showed normal findings, with no infiltration of abnormal lymphocytes, in plasma cells, or chromosomal abnormalities, suggesting that this was a case of IgM monoclonal gammopathy rather than primary macroglobulinemia.\nBecause the patient did not agree to our proposal initially, a renal biopsy was performed 3 months after his first admission to elucidate the cause of his nephrotic syndrome. Thus, the etiology of his nephrosis was initially unknown due to the lack of renal biopsy. Considering his background, the possibility of membranous nephropathy was high; consequently, we administered 30 mg of oral prednisolone first. Three months after his first admission, the patient agreed to receive a renal biopsy.\nUnder light microscopy, the biopsy sample largely consisted of renal cortex and contained 34 glomeruli. Marked enlargement of the mesangial region and strong periodic acid-Schiff (PAS)-positive deposits were observed on the glomerular basement membrane and mesangial matrix (Fig. a). Periodic acid-methenamine-silver (PAM) staining lead to strong silver staining of the spicula in the glomerular basement membrane and mesangial matrix area (Fig. b), but only weak DFS staining and scarce positive blue-green birefringence under polarized light microscope was observed (Fig. c). The immunofluorescence analysis showed that IgM and κ chains were clearly deposited on the glomerulus, and mild deposits were found in the mesangial region. No significant deposition of other globulins was observed; we considered this to be a case of monoclonal deposition of IgM-κ chain (Fig. ).\nElectron microscopy showed deposition of fibrillar materials in the subepithelial and subepithelial regions of the mesangial and glomerular basement membranes. Deposition of fibrillar substances approximately 20 nm in diameter was also observed in the subendothelial and subepithelial layers of the mesangial and glomerular basement membrane by electron microscopy (Fig. ). Based on the results of DFS staining and electron microscopy, the patient was diagnosed with FGN. This diagnosis was confirmed 6 months after his first admission because renal biopsy could not be performed at first admission. Further, it took approximately 2 months to receive the electron microscopy result. Since urinary protein did not improve after administration of oral prednisolone only, 150 mg of mizoribine was added as a drug for nephrosis. Temporary urinary protein decreased to approximately 4 g/gCr, but a significant increase in serum creatinine subsequently occurred. A daily dose of 100 mg of oral cyclophosphamide was started on the 180th day to halt disease progression of FGN and suppress the production of monoclonal IgM, resulting in a reduction of the increase in serum creatinine and a decrease in urinary protein. One month after initiation, the daily dose of cyclophosphamide was decreased to 50 mg and continued for 2 weeks. Soon after, the patient decided to cease administration of oral cyclophosphamide and oral prednisolone; the cumulative dose of cyclophosphamide was approximately 3.7 g. Although cyclophosphamide might have had a positive effect on renal prognosis, the progression of renal dysfunction could not be halted, and hemodialysis was initiated on the 230th day. The patient had melena caused by intestinal bleeding, the etiology of which was amyloidosis, but a diagnosis of AHL was not confirmed in the intestinal tissue. There was no symptom of nerve conduction from amyloidosis. The melena was not observed at the time of hemodialysis initiation, but occurred 2 years after hemodialysis initiation. In addition, the patient contracted liver cirrhosis and was troubled by ascites. Due to the severe intestinal bleeding and liver cirrhosis, his blood pressure decreased and finally he could not receive hemodialysis. The patient died from multiple organ failure at another hospital approximately 2 years after hemodialysis initiation. A cardiac echocardiogram performed 1 month before his death did not show evidence of cardiac amyloidosis, such as shaggy heart and cardiac wall thickening. Therefore, multiple organ failure was presumed to be mainly caused by the liver dysfunction.\nBased on the several new insights into FGN recently reported, we decided to reassess the renal biopsy. Even though DFS staining was weak positive, Congo red staining was positive, leading us to believe that this was a case of congophilic FGN. To confirm the diagnosis, the renal tissue was evaluated by LMD/MS and the Scaffold database, and a minimum of 4 mass spectra as described previously []. Notably, the presence of massive monoclonal heavy chain components (constant region), light chain components (constant region), serum amyloid P components, and apolipoprotein E (Apo E) components indicated that this was a case of heavy-and-light-chain amyloidosis (Fig. ). No DNAJB9 component was detected. Although a blue-green birefringence under polarized light microscope in DFS staining was scarce (Fig. c), that in Congo red staining was positive (Fig. d). |
An 18-year-old woman with T2N0M0 laryngeal cancer was admitted in October 2008 to receive radical radiotherapy. The patient reported an increasing hoarseness lasting for five months. She negated alcohol or tobacco use, laryngeal papillomatosis, and prior neck radiotherapy. Laryngoscopic examination revealed a lesion involving the left true vocal cord, left laryngeal pocket, and posterior part of the ventricle false cord. Biopsy of the lesion revealed moderately differentiated squamous cell carcinoma of the larynx (). CT demonstrated thickening of 8 mm of the left false vocal cord, shallowing the left laryngeal ventricle, and infiltration extending to the upper part of the vocal cord and small (up to 5 mm in the greatest diameter) unspecific lymph nodes of the neck. Staging work-up, including chest CT and ultrasound examination of the neck and abdomen, was negative for metastases. The patient was referred to radical radiotherapy with four lateral 6 MEV photon beams (5.5 cm × 7.5 cm) to the total dose of 66 Gy in 33 fractions. She started treatment two weeks after the diagnosis. Another laryngeal examination, performed on the first day of radiotherapy, showed previously absent infiltration of the anterior commissure and subglottis. Owing to these findings, after delivering 6 Gy, the lower radiation fields were extended by 0.5 cm. Additional CT, performed for radiotherapy modification revealed previously absent, enlarged, left-sided cervical lymph nodes of the second group, outside the irradiated region. An ultrasound of this region showed enlarged lymph nodes with a central hyperechoic area. The biggest lymph node was 2.5 cm in diameter and was localised at the left mandibular angle. Radiotherapy was resumed after a three-day pause. The patient refused fine-needle aspiration biopsy of the neck lymph node. PET-CT performed without interrupting radiotherapy revealed metabolically active lesions in the larynx and in the left cervical lymph nodes at the level of the third cervical vertebra (SUV of 3.8 and 2.4, respectively). After 19 fractions the enlarged cervical lymph nodes were removed. The surgery caused an additional four-day pause in radiotherapy. The tissue material contained two lymph nodes (17 and 12 mm in diameter), with macroscopic features of typical reactive inflammatory response. Histopathology examination showed HV type of CD (). During six-year follow-up, she has been free of cancer recurrence and CD disease symptoms. |
A 60-year-old female with osteoarthritis of the right knee underwent Mako/Stryker robotic-assisted total knee arthroplasty. The patient's past medical history consisted of hypertension. No medical conditions predisposing the patient to a fracture were noted. A midvastus approach was used, and trackers were placed with unicortically inserted pins in the tibial shaft and femoral shaft for the robotic tracking system. The operation was performed without complication, and the tracking pins were removed manually at the conclusion of the case. Postoperative radiology reports noted proper positioning of prosthesis with no acute fracture.\nPhysical therapy was begun on postoperative day 1 with partial weight bearing with a front wheel walker with PT assistance and was able to walk the entire hospital corridor two times. Patient was discharged on postoperative day 2 with outpatient physical therapy arrangements. Patient had a satisfactory postoperative course.\nThe patient had an uncomplicated recovery until 6 weeks postoperatively when she was at work and sustained a ground level fall due to acute pain of the right thigh and her leg “giving out.” Patient was unable to bear weight on the extremity. She presented to the emergency department where she was found to have a complete, oblique fracture through the midshaft of the right femur (). On radiography, the femoral tracking pin site is clearly visible at the location of the fracture in the distal segment (). The patient was taken to the OR and underwent intramedullary nailing of the right femur with retention of right knee prosthesis hardware (). CT scan of right lower extremity confirmed evidence of a fracture through the prior surgery pin tracks. Following intramedullary nail placement, the patient had a normal postoperative course. Final films at 6 months showed a healed fracture with stable orthopedic hardware (). At this time, the patient was pain free, ambulating without issue, and elected to proceed with an as needed follow-up. |
A 70-year-old man who complained of nausea, vomiting, and weight loss was referred to our hospital for examination and treatment. He had a history of hyperthyroidism, diabetes, hypertension, osteoporosis, infectious uveitis, and retinal detachment. Computed tomography revealed thickening of the gastric wall from the gastric body to the prepyloric region, as well as retention of food residues (Fig. ). Esophagogastroduodenoscopy revealed friable and irregular mucosa, and a depressed lesion extending from the gastric antrum to the lower body (Fig. ). An esophagogastroduodenoscopy biopsy revealed poorly differentiated adenocarcinoma (por) (Fig. ). Surgery was performed for preoperative diagnosis of undifferentiated advanced gastric cancer. Total gastrectomy was initially planned but then was abandoned during the operation in favor of gastrojejunostomy because of a strong adhesion to the head of the pancreas. Systemic chemotherapy with the SOX regimen (S-1, 120 mg/day on days 1–14; oxaliplatin, 170 mg on day 1) was initiated as treatment because the patient tested HER2-negative in immunohistochemical staining.\nAfter cycle 1 of oxaliplatin infusion, the patient realized that his right eye had visual field impairment, which he described as darkening of the right half of his visual field and loss of vision lasting about 1 min and occurring about 7 times a day. The daily frequency of this occurrence gradually decreased, and his visual field impairment improved in 1 week. No visual field impairment occurred in the left eye. Since the same symptoms recurred from the second to the fifth cycle of treatment, we consulted with an ophthalmologist. An ophthalmologic examination revealed no obvious damage to the retinal and optic nerves. The patient showed no abnormal findings in the brain or around the orbit on magnetic resonance imaging (MRI) (Fig. , b), no stenosis or aneurysm in the internal carotid artery or ophthalmic artery on 3D-MRI (Fig. ), and no plaque or stenotic lesion in the bilateral carotid arteries on carotid artery ultrasound. Thus, the ophthalmologist arrived at the diagnosis of amaurosis fugax, but without mentioning its cause or recommending any specific treatment. His clinical course led us to suspect oxaliplatin-associated amaurosis fugax and to discontinue oxaliplatin and switch to S-1 monotherapy from cycle 6. Subsequently, the patient's amaurosis fugax improved.\nAfter an improvement shown after cycle 6 in gastric wall thickening on computed tomography, total gastrectomy was performed with D2 lymph node dissection. The gastric lesion in the patient was diagnosed as tubular adenocarcinoma, well-differentiated type (tub1 > tub2), 45 × 13 mm, ypT3 (SS), ly0, v1, N0, M0, grade 1b, stage IIA. The patient underwent adjuvant chemotherapy with S-1 and has shown no recurrence for 6 months. In addition, no visual field impairment occurred since. |
A 53-year-old Sri Lankan male with a background history of diabetes and hypertension for 14 years presented with left side (non dominant) isolated hand swelling for a 7-month duration. Its progressive enlargement was associated with pain and restriction of movements. There were no other small or large joint symptoms. He did not have episodes of fever, and he maintained good physical well-being in terms of appetite and weight. He did not give any past history of chronic productive cough, pulmonary tuberculosis, or any contact history.\nOn examination, there was a swelling near the wrist joint and carpal region both volar and dorsal aspects (). The area was not warm, and mild tenderness was elicited. Flexion extension and circumduction movements were reduced. Distal neurovascular examination was unremarkable. His ESR was 98 mm/hr with full blood count and other biochemical investigations within the normal range. Initial digital X-ray of the hand showed destructive type lytic lesions involving mainly the carpal bones and bases of the 2nd to 5th metacarpals with sparing of the radiocarpal and distal radioulnar joints (). His chest X-ray was normal. He underwent a magnetic resonance (MR) scan of the hand which showed multiple destructive lesions in the carpal bones, surrounding focal fluid collections with narrowing of the intercarpal and carpometacarpal joints (). Flexor muscle tendons were intact. Upon initial assessment with basic investigations and imaging, a conclusive diagnosis was not achieved. A decision was made to go ahead with a synovial biopsy, and an intraoperative caseous material was noted. After the new finding, other investigations in relation to caseous necrosis were carried out. His Mantoux test was positive with 12 mm of induration. Serological assessment for melioidosis was negative. Histology sample showed multiple Langhans type of giant cell associated with caseating granulomas, and the Xpert MTB/RIF test was positive. He was started on antituberculosis treatment with hand physiotherapy and occupational therapy. He was improved in terms of pain and swelling with antituberculosis treatment without any significant side effects of the treatment, and his culture was also positive for Mycobacterium tuberculosis. |
A 65-year-old Caucasian male presented with an elevated PSA of 8.98 ng/mL and a history of TRUS-guided extended sextant biopsy negative for prostate cancer three years prior to presentation. At the time of his prior prostate biopsy, his PSA was 7.25 ng/mL. He had mild baseline lower urinary tract symptoms with an associated AUA urinary symptom score of 14. He denied any erectile dysfunction and had a SHIM score of 25. MP-MRI was performed, and review by the multidisciplinary prostate imaging conference revealed patchy diffuse abnormal signal that is often seen in patients with prior biopsy history and/or inflammation. Despite this diffuse irregularity in signal in the right posterolateral peripheral zone, there was a focal area of well-defined hypointensity with corresponding diffusion restriction suspicious for harboring prostate cancer. There was notable central gland hyperplastic nodules with regional areas of low T2 signal intensity in the left anterior transition zone that was low suspicion for representing malignancy more likely representing benign prostatic hyperplasia nodularity. With these findings, he underwent MRI/TRUS fusion-targeted biopsy that demonstrated GS 4+3 adenocarcinoma in 80% of the specimen cores sampled from the MRI-targeted lesion in the posterior peripheral zone. After discussion of all treatment options with the multidisciplinary team, he elected to proceed with prostate SBRT.\nThe patient then had TRUS-guided fiducial markers placed followed by the radiation therapy planning CT scan two weeks after fiducial marker insertion. CT and MRI fusion was performed to allow for CT generation of the target volumes of the prostate gland and the high-risk intraprostatic lesion which was used for MRI-targeted biopsy proven to represent the intermediate-risk prostate cancer. The radiation oncologist and urologist met to review this image fusion between treatment simulation CT and MP-MRI as well as target volumes for SBRT and SIB. In this case, the highest suspicion lesion was located in the posterior peripheral zone, well visualized on T2-weighted MRI around which the SIB region was drawn. Dose limitations in the region of the rectum were considered posteriorly, and the ipsilateral neurovascular bundle exposures were considered but not considered dose limiting (Figure ).\nThe SIB volume in the posterior peripheral zone received 40 Gy in five fractions while the entire prostate gland was administered 36.25 Gy over the same time course. The patient was being managed with tamsulosin prior to starting radiation therapy due to his history of lower urinary tract symptoms. He developed moderate dysuria during the final two fractions of radiation therapy that was managed by increasing his tamsulosin to twice daily dosing. One week after radiation was completed, he developed a self-limited diarrhea lasting three days. By his one-month follow-up visit, his urinary and gastrointestinal symptoms had returned to baseline. |
We present here a case of an 81-year-old female patient who was admitted to the hospital owing to progressive abdominal enlargement for more than 1 year, accompanied by secondary dyspnea for 1 week. Her clinical manifestations included intermittent postmenopausal bleeding, which occurred three times 6 months previously, lasting for 3 to 5 days each time, and abdominal pain. Moreover, severe dyspnea occurred only in the supine position without a pillow. However, her medical history did not show smoking or respiratory diseases, and only mild hypertension. A physical examination showed an enlarged uterus that was similar in size to that at term pregnancy, with an irregular surface and a substantial abdominal mass that originated from the pelvic cavity (). Additionally, her body mass index was 18.67 kg/m2 and score of World Health Organization Performance Status was 3.\nA blood test and liver function tests showed mild anemia (102 g/L) and hypoalbuminemia (24 g/L) after admission. Tumor markers were in the normal reference range, including cancer antigen (CA) 199, carcinoembryonic antigen, alpha fetoprotein, CA153, and neuron-specific enolase, and only CA125 was slightly elevated to 55 U/L. Chest computed tomography showed that both lungs were slightly thickened without nodules with enlarged lymph nodes. Magnetic resonance imaging of the abdomen and pelvis suggested uterine leiomyosarcoma (). The patient was initially diagnosed with leiomyosarcoma stage I.\nUnder multidisciplinary cooperation and complete correction of hypoalbuminemia and anemia, the patient underwent exploratory laparotomy, total hysterectomy, and bilateral salpingooophorectomy and omentectomy. Intraoperatively, the peritoneum showed signs of further tumor spread. However, in a palpation examination of the liver and parietal peritoneum of the pelvis, no metastases were found. She recovered well postoperatively and was discharged home on day 8. Further chemotherapy or pelvic radiation was recommended, but not accepted. Follow-up physical examination and magnetic resonance imaging 4 months after surgery showed no abnormalities or signs of tumor recurrence.\nFinally, the patient was diagnosed with PMRSu by histopathology combined with immunohistochemistry and stage III C according to the International Federation of Gynecology and Obstetrics 2009. Macroscopically, the surface of the uterus was smooth and irregular, and weighed 5200 g, and its size was approximately 26.0 cm in maximum diameter (). Furthermore, in the uterus, the tumor tissue showed diffuse infiltrative growth, with a hard texture and a small amount of necrosis and liquefaction ().\nHistology of formalin-fixed, paraffin-embedded, hematoxylin–eosin-stained tumor sections showed sheets of large atypical cells and polygonal PMRSu with abundant acidophilic cytoplasms (). The tumor cells invaded adipocytes of the omentum and showed fat infiltration ().\nImmunohistochemical reactions for epithelial membrane antigen (CD34), Melan A (HMB45), α-smooth muscle actin, calmodulin (caldesmon), and S-100 were negative (). In contrast, demsin was overexpressed in the cytoplasm of myogenic tumor cells, including pleomorphic rhabdomyoblasts and normal vascular smooth muscle cells (). MyoD1 and myogenin, which are specific markers of striated muscle cells, showed moderate nuclear staining in morphologically-appearing rhabdomyoblasts (), and Ki-67 positivity was approximately 20% (). The final diagnosis was PRMSu with metastasis to the great omentum. The pathological results were confirmed by Zhejiang Province Pathology Association.\nWe performed a systematic review of the literature that focused on different treatment strategies and prognosis. We found 28 cases of PRMSu described in the English language literature between 1982 and 2020. Recorded variables, including age, size of the uterus, vaginal bleeding, extrauterine spread, treatment methods, and follow-up are shown in . The Kaplan–Meier method was used to analyze and compare the overall mean survival rate and different mean survival rates under different treatment strategies. IBM SPSS statistical software version 19.0 (IBM SPSS, Armonk, NY, USA).\nThis systematic review of the literature showed that the mean age (± standard deviation) of patients was 67.50 ± 9.83 years and age ranged from 35 to 87 years. The mean size of the uterus was 12.00 ± 6.28 cm (range: 4.5–26 cm), and 16 of the 28 patients presented with extrauterine spread at the time of diagnosis. The overall mean survival was 16.95 ± 4.12 months (95% confidence interval [CI] = 8.87–25.03). The mean survival rate of patients without extrauterine spread at the time of diagnosis was 15.00 ± 2.72 months (95% CI = 9.68–20.33), whereas that with extrauterine spread was 16.24 ± 5.45 months (95% CI = 5.55–26.92). Patients with adjuvant chemotherapy showed the best outcome (95% CI = 8.35–41.15). Patients with only surgical treatment showed the shortest survival at the time of diagnosis (95% CI = 0.10–12.54) (). |
The 45-year-old female complained of painless swelling in the upper right posterior region of the jaw since 1-year []. An intraoral clinical examination revealed a single diffuse swelling on right retro molar region extending toward ramus and right posterior part of the hard palate and soft palate up to right lateral pharyngeal wall []. Three months back diffuse extraoral swelling appeared, which gradually increased and measured approximately 4 cm × 4 cm []. Two bilateral submandibular lymphnodes were palpable of size approximately 0.5 cm × 0.5 cm, roughly oval, firm, mobile and nontender on palpation. Orthopantanogram revealed a single well defined multicystic irregular corticated lesion involving lower right 6, angle and coronoid process of ramus of mandible of size approximately 4 cm × 5 cm, resulting in the destruction of coronoid process of the mandible []. Computed Tomography Scan revealed an evidence of expansile lytic lesion with multiple thin bony separations suggestive of a destructive lesion [Figures , , ].\nBased on the clinicoradiological examination, provisional diagnosis of salivary gland malignancy, odontogenic tumor or the connective tissue malignancy was hypothesized. After an incisional biopsy patient was subjected to surgery with her consent. During the surgery a large cystic lesion was observed, contains large cystic spaces full of mucoid material. Hemimandibulectomy of the right side was performed, and the patient was kept under long-term follow-up []. After 6 and 12 months follow-up no abnormality was detected.\nOn gross examination, resected specimen retrieved from the right side of the mandible involving half of the posterior 2/3rd of the body of the mandible to the right condyle. The cut surface showed heterogeneity with some well demarcated cystic area filled with mucin and firm areas at places. Microscopically, examined hematoxyline and eosin stained tissue section was showing areas of sheets of epidermoid cells admixed with some cystic structures [Figures and ]. Small pools and droplets of mucicarmine, periodic-acid Schiff 1, Alcian blue positive [Figures –] mucin were identified throughout the section.\nThe tumor nests cells displayed clear to eosinophillic cytoplasm, well-defined cytoplasmic membranes, inconspicuous intercellular bridges, and only minimal nuclear pleomorphism, with prominent nucleoli and few abnormal mitotic figures [Figures and ]. At places, some mucous cells as well as intermediate cells were visible within the sheets of epidermoid cells which were highlighted by the mucin specific stains [Figures –]. The nests were surrounded by extensive hyalinized stromal sclerosis devoid of lymphocytic infiltration [Figures and ]. More than 60% stroma of the examined tissue section was hyalinized. No evidence of perineural invasion or necrosis. Thus on the consolidation of these histological features the reported case was classified as a Sclerosing variant of Intermediate grade Mucoepidermoid carcinoma. |
A 70-year-old man presented to the emergency department at our institution, with angina-like chest pain, palpitations, and sweating.\nOne year prior to his current admission, he underwent endocrine evaluation following an incidental adrenal finding on imaging measuring 25 × 34 mm2. A 24-hour urine collection for catecholamines revealed a urine epinephrine level of 150 μg (normal limit: <27 μg/day). A 1-mg overnight dexamethasone suppression test was abnormal, with serum cortisol level of 188 nmol/l (normal limit < 50 nmol/l). He then underwent an ambulatory high dose dexamethasone suppression test. That day he was referred to the emergency department due to angina-like chest pain, palpitations, and sweating. He underwent cardiac catheterization, which demonstrated no significant pathology in the coronary arteries, and was discharged the next day. Past medical history was significant for hypertension, type II diabetes mellitus, and dyslipidemia.\nA year later, on current admission, the patient's electrocardiogram showed sinus rhythm, new T wave inversion in lateral and posterior leads, with no conduction abnormalities. Bedside echocardiography demonstrated hypokinesis in the distribution of the left anterior descending coronary artery. The patient underwent urgent catheterization which once again demonstrated no pathology in the coronary arteries. A subsequent echocardiogram demonstrated normal ventricular function with moderate mitral and tricuspid regurgitation.\nDuring his stay in the coronary care unit, the patient experienced several episodes of hypertension and tachycardia, refractory to treatment with calcium channel blockers, beta and alpha adrenergic blockade, angiotensin receptor blockers, and furosemide. As part of resistant hypertension evaluation, abdominal computed tomography was performed and a 36 × 34 × 22 mm3 right adrenal mass was identified. The adrenal mass had a density of 39 Hounsfield units (HU) prior to intravenous contrast injection, increasing to 74 HU following contrast injection. Notably, the patient experienced a severe event of hypertension, palpitations, and chest pain following administration of 100 mg hydrocortisone as preparation for contrast agent infusion as he had received a diagnosis of sensitivity to intravenous contrast material. Plasma metanephrine was found to be 1200 pg/ml (normal < 90 pg/ml) with a normal normetanephrine level of 163 pg/ml (normal < 196 pg/ml). Plasma cortisol level at 8 am, after overnight 1-mg dexamethasone suppression, was 2770 nmol/l, while level at 8 am without dexamethasone suppression was 3292 nmol/l (normal range 100–690 nmol/l). ACTH level was 174 pmol/l (normal range 1.9–10.2 pmol/l). Renin and aldosterone levels were within normal limits.\nThe patient's case was presented at a multidisciplinary team meeting and surgery was advised. Following 2 weeks of alpha blockade, uneventful laparoscopic right adrenalectomy was performed, with stable blood pressure throughout the procedure. The postoperative course was uncomplicated and the patient was discharged on day two.\nPathology revealed a 36 mm pheochromocytoma of the adrenal gland with a scaled score (PASS) of 7 (vascular invasion, predominantly diffuse growth, high cellularity, and spindling of cells). Diffuse adrenal cortical hyperplasia was noted (). Immunostaining was positive for ACTH and synaptophysin and negative for chromogranin, inhibin, calretinin, and S-100 protein. shows the pathology of adrenal tumor, pheochromocytoma, and diffuse growth pattern. depicts positive immunostraining for ACTH. depicts the tumor's vascular invasion, and the adrenal gland's hyperplastic cortex.\nDuring 23 months of follow-up the patient had no cardiac events, his blood pressure decreased to 126/79, and he was able to decrease his antihypertensive medications. A CT scan performed 7 months following surgery revealed normal postoperative changes with no evidence of recurrence. |
A 24-year-old married Arab woman had been admitted to a local health center 2 months prior to referral to our urology department. She had been hospitalized there four times in 1 year for acute pyelonephritis. The fourth episode raised the suspicion for an underlying problem and justified her referral to our urology department after management of the acute pyelonephritis.\nOn admission, she complained of ascending left-sided flank pain during micturition but did not have dysuria or hematuria. She also had a history of frequent urinary tract infections (UTIs) as a young adult.\nShe was perfectly asymptomatic on the right side. A physical examination was normal. Her temperature was 37.4 °C, her blood pressure was 128/84 mmHg, and her pulse rate was regular at 76 beats per minute. Laboratory tests were normal; in particular, a urine examination showed no leukocyturia or bacteriuria.\nShe underwent an abdominal ultrasound which showed an asymmetric size of the kidneys and a bilateral chronic pyelonephritis aspect. Her right kidney measured 10 cm while the left measured 12 cm.\nA voiding cystourethrography (VCUG) was performed and showed grade IV VUR on the left side and grade I VUR on the right (Figs. and ).\nAn abdominal and pelvic computed tomography (CT) scan detected a left completely duplicated collecting system with hydroureteronephrosis and poor opacification of the upper pole moiety. In addition, the parenchyma of the upper pole moiety was atrophied with secretory and excretory delay. In association with VCUG findings, it appeared that the refluxing ureter was the one that drains the upper pole moiety and inserts lower into the bladder. On the right, a duplex collecting system was detected with hypotonic calyces, pelvis, and ureter of the upper pole moiety. An atrophic parenchyma and poor opacification of the upper pole moiety was also detected (Figs. and ). Renal scintigraphy was not available.\nWe carried out a left heminephrectomy because of the poor functioning of the upper pole moiety based on imaging findings associated with recurrent UTIs (Fig. ). On the right side she underwent dextranomer/hyaluronic acid (Deflux®) injections. Dextranomer/hyaluronic acid (Deflux®) was injected submucosally below the ureteral orifice at the 6 o’clock position to create a prominent bulge and raise the distal ureter and ureteral orifice.\nA year after the surgery she has no complaints. The symptoms are completely resolved. Biological and radiological follow-up is unremarkable. A timeline of the case is presented in Figure . |
A 21-year-old healthy nulliparous female presented to a regional hospital in Australia in spontaneous labour at 39 weeks and 4 days of gestation. Her pregnancy was otherwise uncomplicated and she had no medical and surgical history of note.\nThe patient presented to the hospital with a cervical dilation of 4 cm. Her first stage of labour lasted 1 hr and 45 minutes and her second stage lasted 1 hr and 16 minutes, respectively. Analgesic treatment in the first stage included nitrogen oxide as required and 10 mg of intramuscular morphine. Analgesic used in the second stage was only nitrous oxide as required. An episiotomy was performed at delivery. She delivered a live born infant weighing 3690 g with APGAR scores of 9 and 10. The 3rd stage lasted 15 minutes with delivery of an intact placenta.\nAfter delivery, she further requested nitrous oxide and had 10 mls of 1.0% lignocaine infiltrated into the perineum for an episiotomy repair.\nApproximately 2 hours postpartum, the patient started complaining of sudden onset dyspnea and lower chest tightness. Her GCS was 15 at this time and her vital signs were oxygen saturation >95% on room air with a respiratory rate of 18 breaths per minute, pulse of 85 beats per minute, BP 130/78, and a temperature 37.2°C.\nPhysical examination at the time revealed swelling in the neck and jaw line and palpable crepitus in the anterior chest wall, neck, and jaw, consistent with subcutaneous emphysema.\nInitial management included observation and pulse oximetry, as she was clinically stable and able to have a conversation without increase in chest pain or decrease in oxygen saturation.\nSubsequently a chest X-ray (CXR) was obtained which demonstrated pneumomediastinum with air tracking superiorly into the pericardiac spaces (). Following telephone consultation with a cardiothoracic team at our major tertiary referral center, a computed tomography (CT) contrast study was ordered as recommended. CT contrast reconfirmed pneumomediastinum and subcutaneous emphysema and ruled out esophageal injury (). It also demonstrated extension of air in the cervical region ().\nThe findings were once again discussed with the cardiothoracic team who advised expectant management due to the stability of the patient's clinical state.\nHer postnatal course was uncomplicated thereafter and she only requested routine oral postpartum analgesia such as Panadol and Ibuprofen. Subcutaneous crepitus receded and became minimal by postpartum day 2 for the remainder of her hospital stay. She remained clinically stable and all observations were within normal limits. She was observed for 48 hours postpartum and was discharged on day 2 with the extended midwifery service. A repeat CXR done 2 weeks postpartum was normal and showed resolution of all findings (). |
A 21-year-old male reported with a diffuse swelling and pain in the lower right posterior region of jaw for 4 months. Pain was dull and intermittent with increase in difficulty during eating and speech. The patient gave a history of extraction 1 month before for the same complaint, but there was no relief. After 2 months, growth appeared intraorally. Medical history was non-contributory.\nExtraoral examination revealed a diffuse swelling on the right side of mandible, extending anteroposteriorly from 1 cm behind angle of mouth to angle of mandible. Superoinferiorly, 2 cm below line drawn from angle of mouth to tragus of ear to 4 cm below lower border of mandible roughly measuring around 5 cm × 4 cm in size. Skin over swelling appeared normal on inspection and firm and tender on palpation. Right submandibular lymph node was palpable, enlarged, firm to hard, and fixed (). Intraoral examination revealed a soft tissue growth along the right side of mandibular arch. It extended from first premolar to retromolar region causing obliteration of buccal and lingual vestibule. Overlying mucosa was red. Surface appeared smooth with areas of depression at places ().\nRadiographic evaluation included panoramic radiograph, occlusal radiograph, and cone beam CT. Panoramic radiograph revealed an ill-defined mixed radiolucent and radiopaque lesion along the right side of body of mandible denoting irregular areas of osteolysis (). The cross-sectional occlusal radiograph of the right side of body of mandible showed bicortical expansion and presence of radial spicules which spread outside the lingual side of jaw bone giving sunray appearance (). Aspiration was done which gave yellowish viscous blood tinged fluid. Based on clinical and radiological findings, the provisional diagnosis of malignancy of right side of the mandible, hemangioma, and osteosarcoma was given.\nThe incisional biopsy revealed osteoid tissue with irregular trabeculae with osteoblasts which were large round to oval, epithelioid in appearance with hyperchromatic nucleus and prominent nucleoli and occasional atypical mitoses. Intertrabecular areas showed solid cellular stroma. As the biopsy was incisional, tumor border cannot be traced. Hence, the diagnosis was given as aggressive osteoblastoma/Osteoblastoma like osteosarcoma(). Lesion was excised. Grossly, there was extensive soft tissue destruction with an associated soft tissue mass ().\nThe histopathology revealed abundance of osteoid and tumor bone without osteoblastic rimming suggestive of osteoblastic osteosarcoma (). Other areas showed fibrous spindle cell areas where osteoid or tumor bone was scarce i.e. (fibroblastic/fibrohistiocytic type ). At focal places, vessels were numerous, dilated and proliferating giving a haemangiopericytomatous appearance () (Staghorn type appearance). |
A Japanese woman in her 50s underwent surgical resection for a tumor arising in the left retroperitoneum. Preoperative computed tomography (CT) and positron emission tomography (PET)-CT as well as intraoperative findings showed no distant metastasis, no obvious peritoneal dissemination, and no invasion of the uterus or ovaries (Supplementary Information). Histologically, the tumor was identified as a leiomyosarcoma of 12 cm × 11 cmin size. She was treated with postoperative chemotherapy with 6 cycles of doxorubicin, followed by chemotherapy with pazopanib for 8 months. During the observational follow-up period, imaging studies indicated peritoneal recurrence. While chemotherapy with pazopanib was promptly restarted, the tumor mass eventually grew to an obvious tumor in the peritoneum. She underwent debulking surgery twice for peritoneal tumors, but the tumors recurred. She received chemotherapy with gemcitabine and docetaxel, which was discontinued due to the onset of interstitial pneumonia. Cancer genome profiling was conducted to search for genome-matched treatment strategies in daily clinical practice in cancer precision medicine. She was referred to us for genetic counseling and interpretation of the RAD51D splicing variant, which was originally detected in the tumor genome profiling analysis. She had no family history of RAD51-related cancers or other types of malignancies, including sarcoma (Fig. ).\nIn histological assessments, the tumor showed fascicular proliferation of spindle cells with eosinophilic cytoplasm. The nuclei showed moderate atypia, and some tumor cells had prominent nucleoli. Occasional mitotic figures were also observed. Tumor necrosis was not observed (Fig. a). In immunohistochemical studies performed at Mita Hospital adjunctive to the International University of Health and Welfare, Japan, the tumor cells were shown to be weakly positive for α-smooth muscle actin and desmin. These findings are consistent with a diagnosis of leiomyosarcoma, equivalent to histological grade 1 according to the French Federation of Cancer Centers Sarcoma Group (FNCLCC) grading system for sarcoma [].\nThrough genomic profiling analysis of the designated 324 genes of the patient’s tumor samples by using the FoundationOne CDx® (Foundation Medicine Inc., MA, USA) oncology panel, two pathogenic variants in RAD51D (splicing variant) and TP53 (missense variant) and intermediate amplifications of four genes were detected (Table ). On the basis of the recommendations by the expert panel, which is a tumor board that provides molecular and clinical interpretations and suggestions for the results of oncology panel testing, the patient received genetic counseling and subsequently underwent germline analysis for RAD51D c.904-2A > T [NM_002878]. The same splicing variant of RAD51D was detected in the analysis of DNA extracted from lymphocytes by direct DNA sequencing using capillary gel electrophoresis and fluorescence detection (Sanger sequencing technique and application), which was performed in a Clinical Laboratory Improvement Amendments (CLIA)-certified laboratory. After extensive genetic counseling based on the results obtained from germline testing, her two daughters, shown as IV-1 and IV-2, also underwent predictive genetic testing, and both were found to be positive for the variant (Fig. ).\nTo evaluate the potential effects of the spicing variant due to a single-nucleotide alteration at the 3′-end of intron 9, the schematic structure and location of which are shown in Fig. b, we used four computational prediction tools, Max Entropy Scan (MES), NetGene2, Splice Site Prediction by Neural Network (NNSplice), and Alternative Splice Site Predictor (ASSP) to analyze the potential effects of RAD51D c.904-2A > T [NM_002878]. All four tools predicted that the RAD51D variant attenuated normal splicing and yielded a shorter form of the RAD51D protein (Table and ). Furthermore, cBROCA analysis, an experimental analysis for RNA as shown by Casadei et al. [], also predicted that this alteration would weaken the native splice acceptor site and create or strengthen alternative novel splice acceptor sites, resulting in shorter forms of the protein products of RAD51D. Taken together, these computational analyses suggest that a single-nucleotide alteration in intron 9 of RAD51D c.904-2A > T [NM_002878] attenuates normal splicing of RAD51D mRNA, resulting in the production of a shorter form of RAD51D protein (a representative truncated product is shown in Fig. c and d). Although there are no specific consensus domains in exon 10, which is truncated by attenuated splicing, the aggressively malignant phenotypes observed in this case would be evidence for RAD51D c.904-2A > T [NM_002878] as a novel loss-of-function variant involved in the formation and/or progression of malignant tumors.\nAccording to the ClinVar database (Variation ID: 472631) (, accessed in August 2021), the clinical significance of RAD51D c.904-2A > T [NM_002878] is likely pathogenic or uncertain, meaning that its clinical significance has not yet been determined and is a subject of debate in interpretation. Allele frequencies of RAD51D c.904-2A > T (NM_002878) in the general population were 6.60 × 10− 4 (ToMMo 8.3KJPN) and 4.13 × 10− 4 (HGVD ver2.3, dbSNP rsID: 1403784434) in Japanese people and 3.98 × 10− 6 in the global population, suggesting that the splicing variant RAD51D c.904-2A > T [NM_002878] was extremely rare in the global population as well as in Japan, which may be potentially indicate that it is a pathogenic variant (Table ). |
A 12-year-old Caucasian girl presented to our clinic with a history of worsening the left thigh pain for the last 3 months. She was previously healthy, with no history of trauma, and neither she nor her mother could recall any triggering factor for the onset of the pain. There was a decrease in the appetite but no weight loss or fever. At the time of initial evaluation, she was experiencing pain throughout the day and night. She quantified her pain as 8 out of 10 in the visual analog scale (VAS). She had stopped all physical activities completely and was walking with the aid of crutches. The thigh was slightly swollen and was very tender to light touch, with quadriceps atrophy, but no increase in local temperature. The active and passive ranges of motion of the hip and the knee were globally decreased, due to severe pain. The rest of the clinical examination was normal, in particular, no foci of infection were apparent elsewhere in the body, and no skin conditions were detected. She had no pets, no history of travel, and her familiar history was unremarkable. Her initial blood work revealed only a slight increase in the erythrocyte sedimentation rate (24 mm/h).\nThe initial radiographs of the left thigh showed a proximal third diaphyseal lesion of the left femur with aggressive appearance (). It was a centro-medullary, permeative lesion with ill-defined margins and a wide zone of transition. There was cortical thickening with areas of scalloping and cortical erosion, and circumferential onion skin appearance denoting moderately aggressive periosteal reaction. No Codman’s triangle or sunburst appearance, indicating faster and more aggressive periostitis, were visible. MRI showed a 12-cm medullary lesion with hyposignal in T1-weighted and hypersignal in fat-suppressed T2-weighted images, with medullary enhancement after contrast injection. There were areas of cortical disruption, extensive periosteal reaction and perilesional soft tissue edema, but no soft tissue invasion ( and ).\nNeedle biopsy was performed 2 weeks after the initial presentation for microbiological and pathological evaluation, and no evidence was found for infection or tumor (). Serology for Bartonella, Brucella, Salmonella, Tularemia, Coxiella and Mycobacterium tuberculosis were negative. The patient was kept under observation by the tumor team. She was taking acetaminophen for pain relief and the clinical picture remained unaltered for the next month. A second biopsy was obtained through a mini-open approach again, showing no evidence of tumor or infection. At this point, the diagnosis of CRMO was considered, and a scintigram was obtained, which showed unifocal heterogeneous uptake at her left femoral diaphysis, with no other suspicious hot spots. She was then started on 250 mg naproxen t.i.d. with 20 mg omeprazole, 10 mg prednisone daily and a single dose of 30 mg pamidronate. Her VAS score showed initial improvement, from 8 to 3, with pain affecting her only on weight-bearing and no pain at night.\nShe was maintained on this regimen for 6 months. During this period, she also experienced isolated episodes of pain on her right thigh and on her left ankle, with no abnormal findings on the radiographs. After an 18-month follow-up, she demonstrated progressive improvement of her left thigh pain that occasionally recurred with less and less frequency. Although she was walking without crutches, she was still restricted from physical activity. |
A 45-year-old female reported to our institution with the chief complaint of ulcers in the mouth and eruptions on the face for 3 days. History revealed the presence of pricky type pain 4–5 days ago. Then, she had noticed vesicles, which appeared 3 days ago on the right side of the face and in the oral cavity. Subsequently, the vesicles ruptured to form ulcers which were very painful. Extraoral vesicles were intact. All the vesicles and ulcers were limited to the face and oral cavity of the right side only until the midline.\nMedical history and dental history were not contributory except for the fact that the patient had undergone extraction of teeth 6–7 years ago. On examination, the right submandibular lymph nodes were palpable, tender and mobile. Examination of face revealed multiple vesicles extending from the right preauricular area to the right corner of the mouth. Encrustation was seen on the right side of the lip, but was not crossing the midline [].\nIntraorally, multiple shallow ulcerations with erythematous irregular borders and tissue tags were seen on the buccal mucosa, tongue and labial mucosa unilaterally on the right side. These ulcers were painful causing difficulty in eating and mouth opening. There were no other skin lesions accompanying the orofacial lesions. After careful clinical examination, a provisional diagnosis of HZI was made [Figures and ].\nClinical differential diagnosis included herpes simplex infection (HSV). HSV infection appears in a similar fashion and if mild and localized to one side may be mistaken for HZI; cultures helps to differentiate between the two.\nCytosmear prepared from the labial mucosa revealed epithelial cells. Epithelial cells were arranged in clusters, and few isolated cells were seen. These epithelial cells were showing intranuclear eosinophilic inclusions with margination of chromatin resembling Cowdry A type inclusion []. Multinucleated cells [], perinuclear halo [] and nuclear fragmentations [] were also seen.\nCytological features were suggestive of the herpes infection. Hence, correlating the clinical feature with cytological features, a final diagnosis of HZI was concluded. |
A 69-year-old man with 1-year history of a retractable mass in the right groin arrived at our emergency department with a 5-day history of progressively aggravated right inguinal pain associated with the appearance of an incarcerated right femoral hernia. He denied abdominal pain and distension, nausea and vomiting, fever, and any other signs of bowel obstruction. He had no other relevant medical history. His vital signs were normal, and his body mass index was 18.0 kg/m2. Physical examination revealed a non-reducible tender lump in the right groin below the level of the right inguinal ligament. The lump was approximately 6 × 4 cm in size, warm, and erythematous. Routine blood examination revealed a white cell count of 8.62 × 109/L and neutrophilic granulocyte percentage of 75.7%. A computed tomography scan revealed a quasi-circular hypodense lesion of approximately 6.0 × 4.0 × 4.0 cm located medial to the right femoral vessels and connected to the abdominal intestines (–). There were no signs of intestinal obstruction.\nThe patient was preoperatively diagnosed with a right incarcerated inguinal hernia and taken to the operating room for surgery under general anesthesia after providing written consent for treatment. Preoperative prophylactic antibiotics were administered. An oblique incision was made above the inguinal ligament in the right groin, and an anterior approach was performed. Dissection in layers revealed an incarcerated right femoral hernia under the inguinal ligament, and the hernia could not be reduced from below. The peritoneum was dissected through Hesselbach’s triangle, revealing a non-reducible appendix in the femoral canal without signs of peritonitis. The tip of the appendix was purulent and swollen. After yellow-white purulent fluid was extracted by fine needle puncture from the hernia sac, the tip of the appendix was still unable to be reduced because the femoral ring was narrow and the tip of the appendix was purulent and swollen. We cut off the inguinal ligament and then raised the hernia sac and appendix ( and ). Open appendicectomy was performed, and the right femoral hernia sac was resected. We closed the peritoneum with absorbable suture and repaired the hernia by suturing the conjoint tendon to Cooper’s ligament with PDS-II suture. The inguinal ligament was then repaired using PDS-II suture. Finally, a negative-pressure drainage tube was placed in the inguinal canal.\nPostoperatively, the patient received intravenous antibiotics for 5 days and stayed in bed for 7 days. The negative-pressure drainage tube was removed on postoperative day 4. The histopathologic examination confirmed purulent appendicitis. The patient recovered uneventfully and was discharged on postoperative day 8 in good general condition.\nThe reporting of this study conforms to the CARE guidelines. |
A 52-year-old premenopausal female presented at the emergency department with acute right-lower abdominal pain for one-month duration that was aggravated in the four days before admission. Examination of the abdomen revealed right iliac fossa tenderness, associated with rebound and guarding. The clinical findings were consistent with acute appendicitis. One year earlier, she had a full colonoscopy that showed a single colonic polyp, diagnosed microscopically as tubular adenoma. Four years ago, she had antral gastric biopsy which revealed mild chronic antral gastritis.\nDuring her admission, an ultrasound of the abdomen and pelvis was performed and showed retrocecal appendix with early signs of inflammation (edematous wall) and a 9 × 4.6 cm uterus with a small hypoechoic fundal interstitial fibroid. A 2.5 × 2.9 cm right adnexal solid mass was also seen, with no follicles within and good vascularity. It showed intimate relation to both the right lateral uterine wall and the right ovary. A differential diagnosis of either a subserosal fibroid or a complicated ovarian cyst was suggested.\nHowever, she was urgently taken to the operating room for emergent laparoscopic appendectomy. On exploring the abdominal cavity, minimal fluid was found in the peritoneum. The right ovary was noted to have a 5 × 3 cm hard mass, separated from the uterus, not adherent to or infiltrating the surroundings (). The left ovary was within normal size, showing 2 small cystic lesions. The appendix showed congestion and hyperemia. Appendectomy was completed by the surgical team and then right salpingo-oophorectomy was done by an OBGYN team. Both specimens were sent for pathological examination.\nGrossly, a 5 × 2.5 × 2 cm right ovary with attached 5 × 1 × 0.7 cm fallopian tube was received. The cut surface of the right ovary was solid and white with whorled appearance. Microscopically, it showed a normal ovarian tissue with spindle cell tumor. The tumor cells were arranged in interlacing bundles with eosinophilic cytoplasm and cigar-shaped nuclei; no significant mitosis or pleomorphism was seen (Figures and ). The tumor cells showed diffuse strong positive for desmin reaction and negative reaction for S100, inhibin, and CD68 markers, confirming the diagnosis of leiomyoma of the ovary ().\nAdditionally, a 6.5 × 1.5 × 1 cm specimen of lusterless appendix was received for histopathological examination. Microscopically, the appendix showed a tumor in the distal portion, measuring 8 mm in maximum dimension (). The tumor cells were polygonal with eosinophilic granular cytoplasm and central, round to oval, and moderately pleomorphic nuclei having a salt-and-pepper chromatin pattern. The tumor cells were arranged in nests with focal acinar patterns separated by thin fibrocollagenous stroma. They showed focal goblet cell differentiation with positive for mucin stain and amounted to 5–10% of the total tumor volume. The tumor was present in the submucosa and the muscularis propria extending to the subserosa. There was focal vascular invasion. The tumor cells showed strong positivity for synaptophysin and chromogranin (), confirming the diagnosis of goblet cell carcinoid tumor. Interestingly, estrogen receptor marking was performed in both tumors and showed strong positive reaction in the cells of each. |
A 42-year-old male patient presented with headache, right eyelid swelling and right eye redness that he had suffered with for one month. The patient had a history of a car accident with head injury five months ago. On physical examination, right eye exophthalmos and chemosis was observed, and bruit was auscultated on the right eye ball.\nA CT imaging study of the brain implied the existence of a right carotid-cavernous fistula; the scan revealed a prominent right superior ophthalmic vein ().\nThe patient was taken to an angiography suite for endovascular treatment of the suspected fistula. An initial angiogram of the right internal carotid artery (ICA) verified the rapid opacification of the cavernous sinus with drainage into the superior ophthalmic vein, the inferior petrosal sinus and the pterygoid plexus. The origin of the fistula was difficult to visualize despite the acquisition of multiple projection angiograms. With performing an ipsilateral compression study of the ICA, the fistula was suspected to have originated from the proximal portion of the cavernous ICA (). To verify the exact location of the fistula, an Exelsior 18 microcatheter (Boston Scientific, Fremont, CA) and a Transend microguide-wire (Boston Scientific, Fremont, CA) were used for the superselective angiogram and embolization. However, it was difficult to advance the mirocatheter to the cavernous sinus through the small sized fistula, and there was remarkable resistance to moving the microguide-wire back and forth through the fistula. After several trial of catheterization and removal of the microcatheter, an angiogram was done. We found sudden occlusion of the fistula by a blood clot that was protruding into the lumen of the ICA (). The patient's symptoms were immediately alleviated after angiography. We stopped the interventional procedure and conducted two serial follow-up angiograms at one day and one week after the interventional procedure to evaluate the precarious protruding blood clot and the state of the occluded fistula. Progressive resolution of the thrombus was shown on the serial angiogram (). A small residual fistulous channel was noted near the occluded fistula, but it showed stasis of the contrast dye and there was no connection to the other venous channels. Three weeks after therapy, the patient's symptoms had completely resolved. |
A 64-year-old man, who had no past history of major disease, presented with neck mass, hoarseness, and easy choking. Fine needle aspiration of bilateral thyroid glands was performed and cytology showed plenty of single or cohesive tumor cells, and anaplastic carcinoma was suspected. Image studies revealed several nodular lesions with strong heterogeneous enhancement within bilateral thyroid glands. The patient received a radical thyroidectomy. On gross examination, the left thyroid gland was 7.3 × 4 × 3.5 cm in size with one major well-defined nodule, and the right thyroid gland was 5.5 × 4 × 3 cm in size with several nodules. The major well-defined nodule in the left thyroid gland measured 4.5 × 3.5 × 3 cm in size, which appeared tan and partially white in color. It was solid and soft to mildly firm in consistency. Histological exam of thyroid glands revealed follicular adenoma with thick to thin capsule in left side and adenomatoid nodules in right side. Within follicular adenoma and adenomatoid nodules were multifocal areas showing an abrupt transition to a morphologically distinct neoplasm comprised of cells with hyperchromatic nuclei and scanty cytoplasm arranged in large sheets and broad ribbons (Figures and ). The immunohistochemical stains of those neoplastic cells with hyperchromatic nuclei were positive for synaptophysin, chromogranin-A, TTF-1, and cytokeratin, while being negative for calcitonin and thyroglobulin (Figures and ). The surrounding nonneoplastic gland was not involved by the carcinoma. The features suggest metastatic small cell carcinoma of lung origin. Postoperative computed tomography revealed a 4.7 cm tumor mass in the left upper lobe of lung with mediastinal lymphadenopathy. A mediastinum lymph node biopsy was performed. There were many metastatic neoplastic cells present in the lymph node which cytomorphologically and immunohistochemically was identical to metastatic small cell carcinoma in thyroid glands. Small cell carcinoma of lung metastasizing to follicular adenoma and adenomatoid nodules of thyroid glands was diagnosed. |
A thirty-two-year-old female patient presented to the HOSMAT hospital with a complaint of bilateral neck pain which was exacerbated when the head was turned to either side and during swallowing since 4 years. She had been reviewed by many consultants over the past few years for the same without getting any relief. She was referred to us by the Ear, Nose, and Throat Team. There were no other clinical symptoms like headache, temporomandibular pain, or ear pain. There was no history of previous trauma or any surgical procedure. On palpation, the styloid process could be felt quite easily intraorally and extraorally. A course of oral amitriptyline was offered to the patient in an attempt to treat the condition conservatively, but this did not help the patient get relief from pain. Therefore, surgical excision via Risdon's incision () was planned for the bilaterally elongated styloid processes. The patient was offered surgical excision through an intraoral approach, but declined the same when advised of the need for elective tonsillectomy bilaterally. When the styloid process was evaluated radiographically, on the right side it was found to be a Type II variation and on the left side a Type I variation. This was confirmed during the intraoperative phase (Figures and ). Investigations performed included computed tomography scan with 3 dimensional reconstruction (Figures and ).\nThe resection of the elongated styloid process was done with general anesthesia with oral intubation. After Risdon's incision, a subplatysmal flap was elevated with care to preserve the marginal mandibular branch of the facial nerve. The posterior border to the sternocleidomatoid muscle and the posterior belly of the digastric muscle was identified carefully, and the dissection was done in the stylopharnygeal recess. The tip of the elongated styloid process was easily felt in this gap. The entire length was exposed with judicious use of monopolar and bipolar diathermy. The tissues underneath the styloid process were protected using retractors, and the dissection was carefully extended cranially to enable a lengthier resection. A 701 bur was used to cut the elongated styloid process (Figures and ). Plastic closure with drains in situ was employed (). When the patient was reviewed after 24 hours, she was totally symptom-free. Now at one year after surgery, she continues to be symptom-free and is free of any medication. |
Patient 6 is a 49-year-old woman who presented to the W-TBI clinic in April 2017 after being injured in a motor vehicle accident in February 2017. The patient could not recall whether she had lost consciousness following the accident. In the days following the accident, the patient started noticing nausea and headaches with reading on the computer, increased somnolence, and worsening discomfort with motion, sound, or lights. The patient had an MRI and CT following the accident which were both read as normal. At her initial appointment, the patient noted that she had enjoyed reading before the accident, but now could use a computer for only five minutes intervals prior to becoming nauseous and experiencing headache. Her past medical history was notable for breast cancer, migraines, rectal bleeding, gastric polyps, and dyspepsia. Her ROS was positive for fatigue, IBS, more headaches since the accident, increased urinary frequency, burning pain in neck and shoulders, sound sensitivity, worse bruxism since the injury, poor balance, insomnia, and cognitive impairments. The patient was diagnosed with convergence insufficiency and postconcussion syndrome and prescribed home-based orthoptic therapy. In June 2017, the patient was re-evaluated and demonstrated little improvement in her symptoms or upon examination. She did report some improvement in her fatigue, sleeping and cognition. Continued orthoptic training was recommended and that patient returned in October 2017 reporting further improvement in reading, driving, and computer use. However she stated she was not yet at her pre-morbid functioning. It was noted that the patient was hypermobile at that visit (Beighton score 4/9). In addition, she reported that the putative diagnosis of occipital neuralgia offered at previous visits was confirmed by pain management specialists. She demonstrated improved convergence insufficiency consistent with her reduced visual symptoms. The patient was referred for a genetics evaluation and in July 2018, a clinical diagnosis of Ehlers-Danlos, hypermobility type III was made. In May 2019, the patient returned to the W-TBI clinic reporting that her symptoms had “plateaued”. Her ROS was significant for fatigue, headaches, occipital neuralgia, and impaired cognition and appeared to have reached maximal medical improvement. |
A 50-year-old Hispanic man with known history of obesity and hypertension presented with a 3-month history of abrupt episodes of weakness and altered mental status. He referred severe psychomotor symptoms as well as anxiety, aggressiveness, agitation, weakness, diaphoresis, and decreased visual acuity occurring at any time of the day and improved by eating. Upon further questioning, our patient, who worked as a truck driver and whose symptoms had reflected poorly in his workplace, also expressed overall discomfort with life and wishes of death due to the intensity of his symptoms. He had no history of tobacco smoking or excessive alcohol consumption. Medications taken prior to admission included candesartan 16 mg and hydrochlorothiazide (HCTZ) 12.5 mg once a day for hypertension.\nDuring the following weeks, the symptoms increased in severity and frequency, with continued unremitting anxiety and weakness accompanied by loss of consciousness and extreme burning sensation in his entire body. After this acute episode, he was evaluated by a primary care physician in a local hospital and hospitalized for 4 days, where he continued to present recurrent episodes of irritability and aggression to self and others. He was discharged and referred for psychologic evaluation by mental health services at another institution. No specific diagnoses were given.\nUpon release from mental evaluation, he was evaluated by a third physician, who performed a 3-hour postprandial blood glucose test which revealed a blood glucose level of 58 mg/dL. Suspecting an insulinoma, our patient was admitted to a third-level hospital, and upon arrival his vital signs were: afebrile (37 °C), heart rate of 93 beats per minute, blood pressure of 161/84 mmHg, respiratory rate of 19 breaths per minute, and oxygen saturation of 98% on room air. A physical examination revealed an oriented, well-nourished, and hydrated patient, without any stigmata on skin, a normocephalic and atraumatic head, and no masses or lesions. His eyes and ear, nose, and throat examinations were all within normal limits. His lungs were clear to auscultation without any added sounds, and heart sounds had a regular rate and rhythm without any murmurs, rubs, or gallops. An abdominal examination revealed a soft and nontender abdomen without any masses or organomegaly. Extremities showed no deformities, edema, skin discoloration, swelling, or tenderness. A neurological examination was within normal limits and showed an alert patient oriented to person, time, and place with fluent speech and comprehension. Cranial nerves II–XII were intact, with steady gait, with grossly intact sensation in all extremities. The reflexes were symmetric and 2+ at the biceps, triceps, knees, and ankles and had full strength in all extremities. Initial laboratory studies revealed a hemoglobin level of 15.7 g/dL (normal range, 13.7–17.5 g/dL), white blood cell count of 9.51 K/uL (normal range, 4.23–9.07), and calcium levels of 9.60 mg/dL (normal range, 8.42–10.22).\nHe was placed on the standard 72-hour fast to induce a hypoglycemic episode and became symptomatic after 20 hours. Further laboratory testing revealed blood glucose of 29 mg/dL (normal range, 100–125 mg/dL), increased insulin at 426 mIU/L (normal range, 2.6–24.9 mIU/L), and C-peptide levels of 12.83 ng/mL (normal range, 0.5–2.0 ng/mL). Sulfonylurea levels, abdominal ultrasound, and contrast magnetic resonance imaging (MRI) were also performed without findings. Prolactin, parathyroid hormone (PTH), albumin, and calcium levels, as well as MRI of his brain were normal, ruling out multiple endocrine neoplasia type 1 (MEN-1).\nPrior to imaging, liver and renal function tests were performed, revealing alanine aminotransferase (ALT) levels of 27 U/L (normal range, 21–72), aspartate aminotransferase (AST) of 24 U/L (normal rang, 17–59), and creatinine levels of 0.89 mg/dL (normal range, 0.66–1.25). Urine analysis revealed no abnormal findings. After negative initial imaging, a triple phased computed tomography (CT) scan was performed, with an early arterial phase revealing a 1.8 × 1.6 cm hypervascular mass located at the tail of the pancreas (Figs. , ). Both kidneys had normal size and morphology, and slight hepatomegaly was shown as well as round nodules less than 10 mm in size in segments 3, 7, and 8.\nAt the completion of imaging studies, our patient was presented to the surgical department and scheduled for distal pancreatectomy in addition to a splenectomy due to the tumor’s close proximity to his spleen.\nDuring the course of surgery an incidental appendectomy was also performed. A 4.5 × 3.5 × 1.5 cm specimen (Fig. ) was collected during surgery and sent for pathologic analysis; the pathologic analysis reported the diagnosis of insulinoma, an encapsulated mass within the specimen that measured 1.5 × 1.5 × 1.0 cm and is consistent with the findings on CT scan.\nUpon microscopic evaluation, histologic analysis revealed pancreatic tissue organized into islets within a vascularized stroma interspersed by hyaline tissue (Fig. ). Surgical margins were clear of invasion and no metastasis was found in liver or abdominal lymph nodes.\nOur patient had no complications during the immediate postoperative period, in which he was tested for glucose (190 mg/dL, normal range < 126 mg/dL), lipase (207 U/L, normal range < 160 U/L), and calcium (8.5 mg/dL, normal range, 8.5–10.2 mg/dL), and administered omeprazole (40 mg intravenously once per day), insulin glargine (100 UI subcutaneous injection once per day), atenolol (100 mg orally once per day), HCTZ (25 mg orally once per day), metoclopramide (10 mg intravenously every 8 hours), nalbuphine (10 mg intravenously every 8 hours), and ceftriaxone (1 g intravenously every 12 hours). During the third postoperative day (POD), elevated respiratory rate, decreased oxygen saturation, and chest auscultation of diminished breaths sounds with rhonchi, pointed toward a diagnosis of left lobar pneumonia with pleural effusion. However, he was discharged on the fifth POD and prescribed ambulatory management and follow-up by endocrinology, pulmonology, and internal medicine units. On the ninth POD, he returned to our hospital with acute pain and serohematic secretions at the surgical site and was managed with drainage of the seroma and intravenously administered ketorolac (30 mg intravenously twice a day). A vaccination scheme was established on the 19th POD follow-up visit and started on the 18th POD with Prevnar-13® (pneumococcal 13-valent conjugate vaccine, diphtheria CRM197 protein), followed by Influenza (34th POD), and diphtheria and tetanus (DT) booster (53rd POD). On POD 21, he returned to our emergency room with acute pain, 10/10 intensity of both left quadrants. Laboratory results returned “within normal limits” and he was discharged on ambulatory management. He remains asymptomatic at 6-month follow-up and is currently being treated with ciprofibrate (100 mg orally once per day), amlodipine (10 mg orally once per day), enalapril (20 mg orally once per day), and aspirin (81 mg orally once per day) |
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