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A 46-year-old man was referred to our clinic due to chief complaints of chin deviation to the right side. There were right posterior cross bite and dental midline deviation to the right side by 5 mm (Fig. ). In panorama radiograph, bony exophytic proliferation of the condyle was observed (Fig. ). With additional imaging of CT and three-phase bone scan, tentative diagnosis of osteochondroma or osteoma on the left mandibular condyle was made.\nReconstruction and differentiation of tumor mass and surrounding structures were done using Mimics (Materialize NV, Leuven, Belgium). In a mandible only view, tumor mass could be observed at all directions. Resection line of tumor mass was identified (Fig. ). We could anticipate that zygomatic arch obstructed access to the resection line and removal of the resected mass; thus, we plan zygomatic arch osteotomy. The tumor mass was about 20 × 20 × 15 mm; the size of zygomatic arch osteotomy was enough to be 15 mm for drawing out the resected mass.\nUnder general anesthesia, a preauricular incision was made. Dissection and bleeding control was done until zygomatic arch and temporomandibular joint capsule appeared. Two osteotomy lines were made. A posterior one was made at the foremost point of articular eminence while an anterior one was made at 15 mm in front of the posterior line. Zygomatic arch osteotomies were completed, and the fragment was displaced downward with the masseter muscle still attached. The joint capsule was incised, and the lateral pterygoid muscle was dissected to expose the tumor mass. Tumor mass was resected with saw. The surface of condyle was smoothed with round bur. Tumor mass was sent to the Department of Pathology. The zygomatic arch fragment was repositioned and fixed with a four-hole straight shape miniplate (Fig. ). Finally, normal occlusion and proper midline relationship were checked (Fig. ).\nAfter the operation, intermaxillary fixation was done for 2 weeks with rubber rings. A month later, normal occlusion with proper dental midline was maintained (Fig. ).\nCT was taken immediately after the operation. It confirmed that the tumor mass was removed (Fig. ).\nPathologic diagnosis of the tumor mass was the osteochondroma (Fig. ).\nThere are many theories about the etiology and pathogenesis of cartilage-capped and exophytic bony growths. They can occur in bones formed by endochondral ossification. They can also develop from displacement of the lateral portion of the growth plate which then proliferates in a direction diagonal to the long axis of the bone and away from the nearby joint []. In our patient, exophytic projection was extruded from the lateral portion of the condyle head with a direction diagonal to the long axis of the condyle. The peripheral part of the physis has been considered as a hernia from the growth plate []. This hernia may be idiopathic or traumatic. Regardless of the cause, the result is an abnormal extension of metaplastic cartilage responding to factors that cause exostosis growth by stimulating the growth plate. Development of these tumors in the mandibular condyle tends to support the theory that epiphyseal cartilage is idly located on the bone surface. It has been suggested that stress in the tough insertion region where local accumulation of cartilage dislocation is present can induce the formation of these tumors []. This could explain the fact that in the mandible, these lesions often arise at the coronoid process attached to temporalis and anteromedial condylar region attached to lateral pterygoid muscle insertion. Some authors also believe that trauma might play a role in the formation of these lesions [].\nHistopathological findings are crucial to the diagnosis of osteochondroma. Histologic examinations include a cartilaginous cap similar to that seen in a normal cartilage, endochondral ossification, cartilaginous islands in the subcortical bone, and a marrow space contiguous with the underlying bone (Fig. ). It has been reported that the cartilaginous cap might be 10 mm or greater in thickness in the axial skeleton []. However, it tends to be thinner in the maxillofacial region. It might be absent in long-standing cases [].\nWhen considering treatment, the growing state and the type of osteochondroma are crucial. Growth status can be judged by repeated occlusal evaluation or bone scintigraphy. If an active growth is noticed in a child and the asymmetry is large, subtotal condylectomy is usually performed. However, in adult patient with the same symptoms, both side condylectomy and orthognathic surgery should be included. After such condylectomy, lateral open bite on the contralateral side might occur unless some kind of reconstruction is performed. A costochondral graft or a total stock joint prosthesis may be used. However, it has disadvantages such as exploration of the second surgical site, donor site morbidity, and bone resorption. A total joint prosthesis has disadvantages of high cost, material wear and potential failure, and restricted use in the growing patient [, ]. An alternative method might be vertical osteotomy of the ramus and advancing it superiorly to form a new condyle underneath the disc as described previously []. Locally derived bone graft attached to the medial pterygoid muscle has been utilized []. Some authors have proposed conservative condylectomy with less complications []. This protocol is applicable to osteochondromas involving the head of the condyle without extension of tumor into the neck. In patients with osteochondroma, the condylar head usually enlarges fairly and the neck of the condyle is significantly thickened []. Such neck thickening makes it possible to reproduce the remaining condylar stump for functioning as a “new” condylar head. The articular disc is then repositioned onto the “new” condyle and stabilized [].\nIf the condition is inactive and there is no TMJ symptom, the reason for surgical intervention can be cosmetic or associated with dysfunction of mastication. In this situation, treatment can be chosen depending on osteochondroma classification. In type 2 with globular expansion, condylectomy should be performed as described above. In type 1 with protruding expansion, just local excision is sufficient. Some excellent outcomes have been reported for type 1 []. In this study, the patient’s age was 37 years old. There was a mild bone activity on the condyle head in the three-phase bone scan. Exophytic bony protrusion area was approximately 2 × 2-cm sized, heading for anteromedial of the condyle. It was type 1 osteochondroma. Based on these facts, we decided to do local excision.\nAfter 3D reconstruction of the CT images, the tumor mass was growing to anteromedial side of the mandibular condyle; thus, it might be difficult to approach to the mass through only preauricular incision due to the obstruction of zygomatic arch (Fig. ). To allow the access of cutting instruments to the anteromedial side of the mandibular condyle and removal of the excised tumor without extension of incision or dissection, we planned zygomatic arch osteotomy over the tumor mass. During the surgery, zygomatic arch osteotomy provided easy access to anteromedial portion of the condyle without additional incision or wide dissection, and tumor mass was drawn out with only 4 cm of preauricular incision with zygomatic arch osteotomy. Though minimally invasive procedure in this study cannot be applied in all osteochondroma cases, it can be one of the simple treatments for not-growing type 1 osteochondromas.\nThe malignant potential and risk of recurrence are the major drawbacks of conservative procedure. The recurrence rate for solitary osteochondromas in long bone is approximately 2% []. Of all the condylar osteochondromas reported, three have shown recurrence [, , ]. For the three cases, excision and condyloplasty rather than condylectomy were done as the initial procedure []. Because of a short follow-up period, we cannot evaluate recurrence for this case. However, based on mild bone activity in three-phase bone scan and other cases in the literature, it is expected that there will be no recurrence. |
A 70-year-old man presented to our hospital with complaint of right thigh pain. A computed tomography (CT) scan revealed a hematoma in the right thigh. We recommended hospitalization, but the patient wanted outpatient treatment with tranexamic acid because he had received the same treatment earlier for this condition. He received hemostatic infusion for three consecutive days in the outpatient clinic, and hemoglobin level of 13.2 g/dl from one month ago dropped to 11.3 g/dl on the day of injury and to 7.9 g/dl two days after the injury. However, there was no further progression of anemia and the pain decreased. Therefore, the infusion was stopped on the fourth day.\nWhen we looked at the past medical records, he had presented to our hospital with the principal complaint of persistent subcutaneous hemorrhage at the age of 54 years. He had an abnormal prothrombin time (PT) % of 21.0% and activated partial thromboplastin time (aPTT) of 99.4 seconds. Consequently, he was referred to two university hospitals but no specific cause could be found. The doctor at the hospital had said, “factor VIII 4.7% and factor IX 0.6% suggest a broad spectrum of coagulation factor abnormalities. Although he had positive lupus anticoagulant (LAC), the diagnosis of antiphospholipid antibody syndrome could not be made. We cannot make a diagnosis, but FFP improves the coagulation activity to some extent, so use it in the event of bleeding.” Subsequently, there were numerous recurrent bleeding events, with major bleeding occurring every two years, such as in the ilium, pelvic cavity, subdural, iliopsoas, brachial muscles, and mediastinum (). However, many of these bleeds healed spontaneously within a few days with conservative treatment. For this reason, the patient was given the provisional diagnosis of “circulating anticoagulant factor disorder” at the outpatient clinic of our hospital. Considering the age of the patient, the possibility of additional serious bleeding events was high, and therefore, we decided to examine the patient again.\nHe had never undergone a gastroscopy or surgery until the age of 54. He had no history of chronic diseases, such as allergic diseases and collagen diseases. His only regular medication was tranexamic acid. There was no subcutaneous hemorrhaging on physical examination. Blood tests showed a prolonged PT-INR of 4.5, along with aPTT of 99.6 seconds (). As reported 16 years ago, coagulation activities of factor V, VII, VIII, IX, X, XI, XII, and XIII were all reduced (1–26.5%). In addition, lupus anticoagulant (diluted Russell snake venom time method) and cardiolipin antibodies were positive, as were anti-factor VIII and IX antibodies by the Bethesda method.\nBoth PT-INR and aPTT were prolonged, which led us to suspect diseases such as disseminated intravascular coagulation (DIC), liver failure, antiphospholipid antibody syndrome, vitamin K deficiency (including due to warfarin), and drugs (heparin, argatroban, and DOAC). However, platelet count, Fib, and proteins induced by vitamin K antagonism (PIVKA) test were within the normal range, and the disease course did not fit these diagnoses.\nTherefore, we suspected a deficiency of coagulation factor II, V, and X which are common to the coagulation pathways evaluated by PT and aPTT. We first performed the hepaplastin test (HPT) and thrombotest (TT) to investigate whether the coagulation tests changed with factor V (FV) supplementation (). The TT value was 3.7%, while the TT value calculated from PT-INR was 21% []. We determined that the patient was FV deficient because of the difference in TT induced by a high-dose of bovine FV supplementation.\nNext, we performed a cross-mixing test to determine whether FV deficiency was congenital or acquired. The results showed an immediate type of inhibitory pattern (). Incubation at 37°C for 2 hours is required for anti-factor VIII antibody because of its weak inhibitory effect, whereas anti-FV antibody has a strong binding power and does not require incubation. This case also showed an immediate inhibitory response, suggesting the presence of anti-FV antibodies.\nSince anti-FV antibody measurement is a laboratory-level test, we asked other institutions to measure them. As expected, both an immunoblot assay and enzyme-linked immunosorbent assay (ELISA) using purified human plasma FV confirmed the presence of anti-FV autoantibodies [] and he was diagnosed with autoimmune FV deficiency. The plan is to treat him with immunosuppressive drugs in the future. |
A 62-year-old previously well male presented to the emergency department with sudden onset right lower quadrant abdominal pain. A computed tomography (CT) of the abdomen demonstrated an inflamed appendix with enlarged locoregional lymph nodes along with a 4-cm lesion in the right adrenal gland with central necrosis. Incidentally, there was also found to be a 64 mm × 53 mm × 51 mm mass in the right lower lobe of the lung with several satellite lesions in the right lower and middle lobes (Fig. ). The patient was admitted and commenced on intravenous amoxicillin and clavulanic acid.\nThe patient is a lifelong non-smoker and had no respiratory symptoms at presentation. He was fit and physically active and had no family history of malignancy. Other significant medical history included erosive osteoarthritis, obstructive sleep apnoea, diverticular disease, and a previous squamous cell skin cancer excised from his right thigh with clear margins. He had undergone a colonoscopy four years prior which had revealed diverticulosis with no lesions suspicious for malignancy.\nOn day 2 of his admission, he underwent a laparoscopic appendicectomy which revealed a very thick and erythematous appendix adhered to the mesocolon. Once recovered on day 5 of admission, the patient underwent an endobronchial ultrasound-guided biopsy of his subcarinal lymph nodes which revealed adenocarcinoma. The specimen stained positive for cytokeratin 7 (CK-7), thyroid transcription factor 1 (TTF-1), and napsin A which suggested a primary lung cancer. The adenocarcinoma was diffusely positive for epidermal growth factor receptor (EGFR) exon 19 mutation and negative for both anaplastic lymphoma kinase (ALK) and c-ros oncogene 1 (ROS-1) mutations (Fig. ).\nThe anatomical pathology from the appendicectomy then revealed an adenocarcinoma with an identical immunohistochemical profile with lymphovascular invasion and involvement of the mesoappendix. A subsequent fluorodeoxyglucose (FDG) positron emission tomography (PET) scan demonstrated diffuse metastatic disease with bone, pancreas, and brain metastasis, confirmed on cerebral magnetic resonance imaging (MRI).\nThe patient recovered well from his appendicectomy and after confirmation of his EGFR mutation was commenced on osimertinib, a tyrosine kinase inhibitor.\nUsing the search (“Lung Neoplasms”(Mesh)) AND “Appendicitis”(Mesh)), MEDLINE revealed 32 results, of which 21 were excluded due to either not being related to lung cancer, not being related to appendicitis, or not being original research. This revealed 12 case reports for inclusion, of which two were found via review of included report bibliographies, with two publications unable to be included as the full article could not be viewed. All case reports were of metastatic lung cancer causing appendicitis.\nOf the 12 cases included, the mean age was 63.7 years with 11 of 12 cases being male. In only three cases did the patients present with an appendicitis which subsequently led to the diagnosis of metastatic lung cancer. Of the eight patients with a pre-existing diagnosis of metastatic lung cancer, the mean time from diagnosis was just less than eight months, with six of these receiving chemotherapy at the time of presentation. Out of 12 cases, 10 presented with a perforated appendix.\nOf these 12 cases, half were small cell and half were non-small cell carcinomas [, , , , , , , , , , , ]. |
The patient, a 22-year-old female, with no significant previous medical history, presented to her family doctor with complaints of pelvic discomfort, dysuria, and dyspareunia, for the last 3 months. At the time of consultation, she also notes a whitish odorless vaginal discharge. She denied any episodes of fever, low back pain, menstrual disorders, vulvar symptoms, or changes in urine. The gynecological examination showed sparse, whitish airy leukorrhea, with no visible lesions on the cervix. The pelvic bimanual examination caused pain with the mobilization of the cervix and adnexal regions. She was empirically treated with metronidazole vaginal suppository id for 7 days and azithromycin 1 g single dose. A pelvic ultrasound (bladder and gynecologic), urinalysis, and urine culture were requested.\nThe gynecologic ultrasound showed only a small amount of fluid in the endocervical canal with a thickness of 1.2 mm, and ovaries of normal morphology and dimensions, with some bilateral cystic structures of probable functional nature. The bladder ultrasound described a little vaguely nodular thickening area in the anterior wall, of relatively regular contour measuring 9 mm × 4 mm. The urinalysis and urine culture were both unremarkable. Considering these results and the clinical improvement observed, it was decided to perform a new pelvic ultrasound in 2 months. Meanwhile, the patient went to the emergency department with complaints of dysuria, pollakiuria, and urinary urgency. These symptoms were interpreted as cystitis, and a single dose of fosfomycin was empirically prescribed.\nThe new bladder ultrasound, performed 2 months after the first one, showed a hypoechoic nodule on the anterior wall, measuring 16 mm × 11 mm × 6 mm, with regular contours, well-defined limits and homogeneous texture, compatible with benign lesion.\nThe patient was referred to the urology consultation. About 2 months after this referral and 1 month before the urology consultation, the patient went again to the emergency service with complaints of dysuria and pollakiuria, with a urine dipstick analysis compatible with cystitis and having been prescribed norfloxacin.\nFollowing the urology consultation, the patient underwent cystoscopy, which revealed a lesion protruding into the bladder lumen. A transurethral resection of the bladder was scheduled and performed in 2 months, and it went with no complications. The anatomopathological analysis established the diagnosis of bladder leiomyoma [Figures and ]. The patient was evaluated after a month and considering she was asymptomatic; she was discharged from the urology consultation.\nAbout 2 weeks after the hospital discharge, the patient went to her family doctor with complaints of dysuria and suprapubic and low back pain, which persisted since the date of discharge, with progressive worsening. She was apyretic and had a negative renal Murphy's punch sign (or Pasternacki's sign). A urine culture was ordered before empirical antibiotic therapy with fosfomycin, but the patient failed to do it. To this date, the patient remains asymptomatic. |
A 54-year-old Italian, Caucasian woman with a history of autoimmune thyroiditis was seen at the surgical unit for pain, redness and swelling of the left breast which had developed over the preceding 72 hours. The patient had been on 10 mg prednisone for 1 month secondary to autoimmune hemolytic anemia. On physical examination, she was afebrile. Erythema and edema of both areolar and peri-areolar areas of the left breast were present with dimensions of about 9 ×6 cm and with homolateral tender lymph node enlargement. Acute bacterial mastitis was the initial diagnosis and cefotaxime 2 g/day was prescribed for the first 2 weeks. At this point, due to persistence of symptoms, breast ultrasound, mammography, fine needle aspiration, and tru-cut needle biopsies were performed. Histopathology results were consistent with a chronic inflammatory reaction. Subsequently, a draining sinus developed and a pus sample was sterile.\nOne week later, another swab grew both Pseudomonas luteola and Staphylococcus epidermidis prompting us to perform a second needle aspirate. Cefotaxime 1 g TID and oral ciprofloxacin 500 mg BID were prescribed for a total of 3 weeks. During this period, mild improvement was seen but as the fistula closed her symptoms relapsed so it was decided to perform surgical excision of the abscess. At surgery, galactophora ducts were found to be filled with purulent material. Also a mass of 9.5 × 7 × 5 cm, with cavitation and purulent material, was excised. Once again, histopathology reports showed a chronic inflammatory reaction with giant cells. About 1 week after surgery, the inflammation reappeared and a third needle aspirate was performed. This time, M. abscessus was identified and confirmed in two subsequent cultures [,]. Acid-fast bacilli (AFB) were also seen in the pus after Zhiel-Neelsen staining (Figure ). At this point, the patient was transferred to the infectious diseases service.\nOn admission, the patient had an area of inflammation of about 6 × 7 × 4 cm in the external quadrants of her left breast. A few days later, a fistula formed and microbiological tests confirmed the presence of M. abscessus.\nThe isolated strains were identified by the reverse hybridization method (INNO-LiPA, Belgium) and confirmed by genetic sequencing [,]. Antimycobacterial susceptibility tests were performed in microdilution broth (MHB, BioMérieux, France) []. M. abscessus results were as follows: susceptible: clarithromycin (MIC 0.125 mg/L) and amikacin (MIC 2 mg/L); intermediate susceptible: cefoxitin (MIC 16 mg/L) and imipenem (MIC 8 mg/L); resistant: ciprofloxacin (MIC 8 mg/L), doxycycline (MIC 8 mg/L), co-trimoxazole (MIC >64 mg/L) and linezolid (MIC 32 mg/L). Abnormal laboratory test results are listed in Table . HIV serology was negative, CD4+ lymphocytes were normal and blood cultures were negative for AFB. Therapy consisting of clarithromycin 500 mg BID, imipenem 1 g BID and amikacin 1 g/day was started. The latter was discontinued after 2 weeks due to possible vestibular side effects. Prednisone was also discontinued without recurrence of hemolysis. A chest CT scan confirmed left breast soft tissue and glandular involvement (Figure ). Thirty-two days after admission, the inflammation was considerably reduced and the patient was discharged on clarithromycin 500 mg twice a day. Only 1 week later, the patient had to be readmitted because of recurrence. An ultrasound scan showed a hyperechogenic area with hypoechogenicity and aspiration was performed. This time, AFBs were not identified in the pus and the cultures were negative. Imipenem was restarted with clarithromycin for a further 3 weeks. Thereafter, clarithromycin alone was prescribed for another 10 weeks. Eighteen months after treatment discontinuation, the patient remains disease-free. |
A 70-year-old female patient was transferred to our institute emergency room for acute progressive quadriparesis. The past initial cervical magnetic resonance imagings (MRI) which were checked 2 months ago showed just multiple degenerative changes without any compressive spinal cord lesion. She has been treated with cervical epidural steroid injection for neck pain in local clinic about 5 hours before hospital arrival. After cervical injection, suddenly neurological symptoms occurred and became worse progressively. At the initial neurological examination in our hospital, her mentation was awake and alert. Her pupils were both 2 mm sized and normally reacted to the brightness of light. However, she was unable to move bilateral lower extremities voluntarily at all in response to painful stimuli. Motor grade of upper extremities were 2/5 on right side and 3/5 on left side. Her all senses (pain, temperature, light touch, vibration) was decreased beneath C4-5 level. Cervical MR images showed a mottled mixed signal intensity of nodular lesions in right side of intradural and extramedullary space at level from C3 to C7 and a high intensity of spinal cord with bulging contour of spinal cord that means a spinal cord injury (). We planned to perform emergency operation for decompression and hematoma removal. We performed cervical laminectomy on C3-6 and evacuated hematoma via intradural approach (). After emergent operation, she needed intensive care to cure bradycardia, hypotension and respiratory function for 3 weeks. Unfortunately she began a late course of motor recuperation. 3 months later, postoperative MR images showed all subdural hematoma was removed and spinal cord compressive lesion was not detected any more (). Her neurological symptoms improved to motor grade 3 of lower extremities and ambulating with a wheeled walker. A physical examination showed motor strength in right upper extremities of 3/5 and left upper extremities of 4-5. Currently, she was transferred to the rehabilitation department. |
A 67-year-old female presented with a low back pain and left sciatica. Although the patient had experienced occasional mild low back pain for several years, her low back pain markedly worsened 2 months before her first visit to our service. She also complained of newly developed left sciatica during this period, resulting in intermittent claudication. She had not noticed her urinary symptoms specifically, until she was asked at the examination, but she stated that those symptoms had started several months before her first visit. She denied any history of trauma, infectious diseases, or surgeries related to her spine. She had a history of several abdominal surgeries: cholecystectomy for gallstone and Hartmann’s procedure for rectal cancer 10 years ago and Miles’ surgery for anal cancer 9 years ago. She also had a mesh repair surgery for ventral hernia 5 years ago. All abdominal procedures were performed under general anesthesia with epidural anesthesia in her thoracolumbar spine. Radiographs of her lumbar spine showed some degenerative changes including decreased disc height and mild scoliotic changes. Magnetic resonance imaging (MRI) of her lumbar spine demonstrated an intradural extramedullary spinal cord tumor at the T12–L1 level (2.1 cm × 1.2 cm), and her spinal cord was markedly shifted anteriorly by the enlarged tumor (Fig. ). Computed tomography (CT) of her thoracolumbar spine did not show any calcification in the spinal canal (Fig. ). Because she had a history of contrast dye anaphylactic shock, a myelography was not performed. She also denied taking an MRI with contrast.\nSpinal dysraphism or skin abnormalities were not observed in her lumbar/sacral region. On her neurological examination, she showed full strength and an unremarkable sensory deficit in bilateral upper extremities. The patient showed motor weakness 4/5 in the left lower extremity (iliopsoas, hamstring, quadriceps, foot dorsiflexion, and plantar flexion). Decreased sensation in the left L1 to L3 (6/10) and the left L4 to S1 (8/10) distribution was observed. The right lower extremity demonstrated full strength, and her sensation was intact in the right lower extremity. Although sphincter tone was not diminished and perianal sensation was intact, she was found to have urinary symptoms, such as urinary frequency and a feeling of residual urine. Reflexes were normal in the upper extremities bilaterally; however, hyperreflexia was observed in the patellar reflex bilaterally. Babinski sign was negative bilaterally. Because her symptoms deteriorated, a surgical treatment was performed.\nA laminectomy from T12 to L1 was performed, and the local dura mater was incised in the midline until the tumor was exposed (Fig. ). The tumor excision was performed using microscopy. There was a mild adhesion between the tumor and the arachnoid membrane. Lumbar spinal nerve roots were not involved. Because there was some adherence between the tumor’s thin capsule and the conus medullaris, the capsule ruptured during resection. The spilled tumor contents were removed as well as the tumor itself (Fig. ). The thin capsule attached to the conus medullaris was also removed carefully. After the spinal canal was flushed by large amount of water, the dura mater and arachnoid membrane were sutured tightly. The surgical time was 151 min, and the estimated blood loss was 48 ml. Motor evoked potentials were used for neurological monitoring, and there was no alarm during the procedure. Histological examination of the specimens demonstrated that the cyst walls lined with stratified squamous keratinizing epithelium surrounded by the outer layer of collagenous tissue with the absence of skin adnexa. Abundant keratin material was also observed (Fig. ). A diagnosis of epidermoid cysts was confirmed. She could ambulate immediately postoperatively, and her left sciatica and leg weakness significantly improved 3 months after the surgery. Her MRI showed complete resection of the tumor, and there was no recurrence at 2-year follow-up (Fig. ). |
An 81-year-old lady presented for further evaluation of progressive dysphagia with associated weight loss. The patient reported a 4-month history of unintentional weight loss of 5 kg. She is a life-long nonsmoker with no past medical history other than hypertension and hypercholesterolaemia, for which she takes a statin and an amlodipine/valsartan combination. Bloods were all within normal limits except for a mildly elevated creatinine at 105 umol/l (Hb 13.5 g/dl, MCV 87.9 fl). Physical exam was noncontributory. In particular, there was no evidence of secondary metastatic disease.\nAn oesophagogastroduodenoscopy (OGD) was carried out which was significant for two distinct oesophageal tumours. The first was found at 30–35 cms with an appearance of bulging mucosa (). A second tight stricture was seen at 40–46 cm abutting the oesophagogastric junction with extensive mass lesion extending into the fundus of the stomach (). The intervening oesophagus from 35 to 40 cm was normal in appearance as was the remainder of the OGD with the scope passing without difficulty to the second part of the duodenum. There was no endoscopic evidence of Barrett's oesophagus.\nMultiple biopsies were taken and a staging CT of thorax, abdomen, and pelvis was performed. This confirmed the two separate tumours along with celiac adenopathy suggesting locoregional spread.\nPET/CT showed that diffuse metabolic activity to a maximum of 14.2 SUV was evident at both tumour sites and there was associated gastric wall thickening compatible with extensive local spread of the distal mass. Large metabolically active lymph nodes at the level of the celiac axis measuring up to 15 mm were evident with a maximum SUV of 4.5 ().\nAnalysis from the biopsies confirmed the presence of two histologically distinct masses. Specimen A from the mid-oesophagus showed high grade carcinoma with some features suggesting neuroendocrine differentiation (). Overlying squamous mucosa shows moderate to severe dysplasia. Immunohistochemistry showed positive staining for CK 5/6, P63, and CAM 5.2 confirming a diagnosis of SCC (). No histological evidence of Barrett's oesophagus was seen.\nSpecimen B from the lower oesophagus showed invasive moderately differentiated mucinous adenocarcinoma which was HER 2 negative ().\nProgressive dysphagia warranted a repeat OGD with the insertion of an oesophageal stent. demonstrates the lower oesophageal mass prior to stenting. A 12 cm partially covered Wallflex stent was placed across the lower malignancy (). The second stricture was not stented as it was felt that covering both strictures with one stent would offer a suboptimal result.\nHer case was subsequently discussed at our hospital MDT. It was decided that radiation therapy was unsuitable in this case. A radiation field of at least 20 cms would be required to encompass both proximal and distal tumours along with the positive lymph nodes. Safe delivery of full dose radiation to this extent in combination with chemotherapy would not be feasible.\nChemotherapy was commenced as per the standard CROSS regime of weekly paclitaxel and carboplatin as it was felt that these agents would treat both cancer types. This was administered for twelve weeks after which time a restaging CT scan was performed. This CT showed a slight increase in size of one of the previously noted celiac lymph nodes. As a result, the chemotherapy regime was switched to bolus fluorouracil (5 FU) and folinic acid. This was continued for three cycles. This treatment yielded a good outcome with the patient surviving for 15 months from initiation of treatment. |
A 57-year-old male patient was admitted to our center because of a cystic mass in the right upper lung field that was discovered incidentally by chest X-ray during a routine health checkup. He was a current smoker with more than 30 pack years of cigarette exposure. He had been diagnosed with chronic obstructive pulmonary disease (COPD) 20 years previously and had been using an inhaled corticosteroid and bronchodilator. The patient has been working at the convenience store, and had no history of trauma or use of any anticoagulants. He had no chest pain, fever, cough, or sputum. There were no other episodes of hemoptysis. His physical examination showed decreased lung sounds in both lung fields without wheezes or crackles. There was no palpable lymph node enlargement. Routine laboratory studies including a platelet count and coagulation profiles yielded normal results, except a slightly increased C-reactive protein (CRP) level (7.13 mg/dL). Pulmonary function tests showed a severe obstructive pattern, the forced expiratory volume in 1 second (FEV1) was 1.38 L (36% of predicted volume); the forced vital capacity (FVC) was 4.08 L (85% of predicted capacity); and the ratio of FEV1 to FVC was 34%. The diffusing capacity was 15.7 mL/mm Hg/min (61% of predictive capacity). Several sputum analyses were negative for malignancy, bacterial infection, and tuberculosis. There was no evidence of human immunodeficiency virus infection. Chest X-ray showed a clearly defined homogeneous round opacity without calcification in the right lower lung field (). Computed tomography (CT) scanning of the chest revealed a huge round cystic mass across the upper and lower lobes of the right lung, which measured 9×7.9 cm at its maximum diameter ().\nSingle port video-assisted thoracoscopic surgery approach using 5-mm scope through a 3.0 cm skin incision was performed under general anesthesia and one lung intubation. After dissection of the pleural adhesions present, a thick yellowish cystic mass with a well-defined margin of 9×8×8 cm in size was found along the major fissure between the right upper and lower lobes (). Fine needle aspiration collected a dark brownish colored viscous fluid. The mass was resected completely. Pathological examination revealed a large amount of dense fibrous tissue with blood clots, compatible with pulmonary hematoma (), with no evidence of malignant cell infiltration. After postoperative care, the patient was discharged without any complications. A follow-up chest X-ray after 2 months showed no recurrence, and there was no evidence of recurrence 1 year after the follow-up period. |
This case presents a 50 year old divorced woman who was off work due to depression in response to her mother's cancer diagnosis and ultimate passing. The client had been off work for approximately 2 years prior to her referral for CWH. She had been off work once before (for 6 months) 1 year prior to the current leave, again due to her emotional struggles related to her mother's diagnosis. The client was being followed by her family physician and her psychiatrist, each on a monthly basis. She had also seen a psychologist bi-weekly for 18 months. The client was prescribed Bupropion and vitamins.\nThe client was referred for CWH at ERGO-Wise, an occupational therapy private practice in Ottawa, Canada that specializes in workplace mental health and RTW preparation. The client had graduated from a University level nursing program and had worked in the healthcare field for over 30 years, including 20+ years in her pre-disability adjudicator job at a government agency. She had been divorced for 20 years after having been in an abusive marriage. She noted difficulty with authority and conflict as a result of this relationship. The client lived on her own in close proximity to her one daughter. Regarding engagement in meaningful activity, the client reported reading, knitting, and going for daily walks.\nConsistent with CWH protocol, the client had received medical clearance to return to work (at the time of the referral) due to a reduction in her depression symptoms and an improvement in her mood state. Regarding RTW barriers, the client reported: (i) decreased cognitive abilities (e.g., reduced concentration); (ii) fatigue and decreased stamina; and (iii) the need to update her computer skills. The client also reported that she lacked assertiveness and felt threatened by authority and/or conflict situations. This manifested in heightened anxiety with interpersonal relationships for fear of potential discord. summarizes the intervention timeline.\nFollowing referral to CWH and with the client's signed consent to proceed with an intake interview and to release information to the referring person, the treating occupational therapist conducted a semi-structured interview to obtain background information about the client's work history, disability trajectory, and occupational performance issues. The interview helped the therapist understand the client's occupational performance challenges and RTW obstacles while it gave the client the opportunity to better understand the CWH intervention and the therapist's role in the RTW process. Through this intake process, the therapist made a positive determination about the client's suitability for CWH.\nThe CWH intervention was provided in a simulated work environment located in an office building conveniently situated for car or public transportation access. The office suite resembled a typical work environment with several workstations each equipped with a computer, keyboard/mouse, height adjustable monitor, and fully adjustable chair. The intervention was 4 weeks in duration with the following structure: Week One: 2 ×2 h; Week Two: 2 ×3 h; Week Three: 3 ×3 h; Week Four: 3 ×4 h; totaling 31 h.\nThe CWH intervention was provided by a registered occupational therapist aptly qualified for this role given that the intervention's underlying principles are cornerstones of the occupational therapy profession. These include assessment of occupational functioning, work demands analysis, and functional gap mitigation (, ). Furthermore, the intervention's use of work as a treatment modality is a well-grounded occupational therapy strategy (). The occupational therapist's skills in clinical observation and clinical reasoning (), together with expertise in mental health () and knowledge of fatigue/pain management strategies (e.g., pacing, ergonomics) () well-position the occupational therapist for this key role in RTW preparation.\nOn the first day of CWH, the client completed three self-report questionnaires that address constructs that can relate to RTW outcomes (). The questionnaires were the Work Ability Index (WAI) (), the Multi-Dimensional Assessment of Fatigue (MAF) (), and the Beck Depression Inventory II (BDI-II) (). These three questionnaires were also administered on the last day of the CWH program serving as pre- and post- self-report measures of change following participation in CWH.\nThe WAI is utilized for the self-assessment of work ability and the evaluation of the effects of intervention programs on work ability (). The MAF measures four dimensions of fatigue (e.g., severity, distress, timing, degree of interference in activities of daily living) (). The BDI-II is a widely used screening measure of depressive symptoms ().\nThe client's first work task was to complete a job description which re-connected her to her pre-disability work. This task required concentration and memory and although these cognitive abilities had atrophied while being on disability, the client noted that it felt good to use them again, even if not at full capacity. The job description helped the occupational therapist gain a better understanding of the client's job demands and work tasks. Through job analysis, the therapist determined the cognitive abilities the client required to meet her job demands setting the stage for designing work simulations that modeled the client's job tasks and/or the cognitive abilities needed to perform her job. Work simulations are the cornerstone of the CWH intervention. The occupational therapist ensured they were relevant and meaningful to the client and to her work. Such customization of work tasks renders the work more “real,” even if simulated.\nIn order to address the client's concern regarding outdated computer skills, the occupational therapist incorporated work tasks that required the use of Microsoft Office (e.g., Word, Excel) as well as Internet searches to assist the client in becoming more current in technology and various applications she would need once back at work. Meaningful and relevant work simulations included having the client research new policies/procedures related to her workplace, new programs, and new projects that were undertaken at work during her absence. This provided the client a sense of what happened while she had been away to help her feel less “lost” once back at work. The client also created a program proposal and budget that helped re-familiarize her with Excel and its functions. Through these work tasks, the client rebuilt her cognitive abilities (e.g., concentration, attention to detail) and regained self-confidence. Work stamina also increased through graded work tasks and progressive work hours.\nAnother component of the client's CWH program was coping skill development utilizing professional development videos, role plays and coaching by the occupational therapist. Effective communication strategies, such as assertiveness skills, were targeted for the purpose of interpersonal relationships and enhanced social skills with a view toward helping the client better cope at work thus potentially serving in a preventative role in future sick leave (). The occupational therapist also equipped the client with strategies to help with her heightened anxiety such as positive self-talk, cognitive reframing, and pacing. Discussions with the therapist rounded out the client's program so that it was a blend of work tasks and skill development.\nConsistent with occupational therapy practice and its promotion of health and well-being through occupation, the client's engagement in meaningful activity (outside of work) was monitored through self-reports and discussions with the therapist. This augmented the focus on work functioning by including a more holistic and integrated approach to treatment and RTW preparation.\nThe client reported generalized neck pain which was associated with time at the computer. The occupational therapist paid attention to the client's workstation ergonomics and ensured that her setup included ideal equipment heights for proper positioning. In addition, the therapist provided education on pacing strategies for pain/fatigue management and education on ergonomic principles to ensure that body biomechanics were addressed with a view toward minimizing pain/discomfort and potential impact on functioning.\nIn-session metrics included a self-report 10-point fatigue scale to quantify the client's fatigue. Overall fatigue levels were moderate and although there was an increase of one to two levels from start to end of a session (e.g., 4.0–6.0; 5.0–6.0), levels remained in the moderate range (see ). This pattern remained stable across the 4 weeks even with progression in hours and task complexity. Such a profile suggests that the client was tolerating CWH hours/tasks. Indeed, her work stamina increased from 2-h to 4-h sessions and her weekly work hours increased from 4-h to 12-h with consistent work productivity.\nAssessment of work ability was derived from clinical observations, session tools/metrics, and the City of Toronto's Behavioral/Cognitive Functional Abilities Evaluation (). The latter includes ratings of 16 behavioral and cognitive abilities (e.g., attention to detail, multitasking) according to clearly-defined criteria. Ratings of five executive skills were added to evaluate goal-directed behavior, problem solving, and emotional regulation.\nFor example, “ability to self-supervise” is evaluated along four criteria of function: (1) Cannot self-supervise, requires constant work supervision; (2) Requires frequent supervision; (3) Can tolerate infrequent supervision; and (4) Able to carry out work tasks in a self-supervised manner. The client in this report was given a rating of three with the following comment: “The client was able to work independently with minimal supervision once tasks were determined. Within each task, she was able to self-supervise and to progress from beginning to end without ongoing supervision.”\nFindings from the client's CWH program revealed no limitations in cognitive abilities that would preclude a return to competitive employment; however, several issues needed to be addressed through accommodations in order to maximize RTW success:\nReduced work pace pointed to the need for limited multitasking and (tight) deadlines.\nPotential for fatigue required the incorporation of pacing strategies at work including regular breaks, a walk outdoors and healthy eating (e.g., snacks, lunch).\nReported neck pain/discomfort needed to be addressed through a worksite ergonomic assessment to identify/rectify ergonomic risks (e.g., equipment heights).\nPotential for heightened anxiety (especially with anticipated negative situations and/or confrontation) required the client to use the assertiveness and stress management strategies learned at CWH.\nNotwithstanding these issues, the client demonstrated work ability as evidenced in her adoption of a work routine and display of work skills. Through engagement in work simulations, she demonstrated task engagement, sustained concentration, the ability to work in a sequential manner to achieve task goals, and the mental flexibility to respond to task requirements. She was able to work effectively on both structured and non-structured tasks. She demonstrated the ability to learn and to apply learnings to concrete work tasks. Such markers of work performance are consistent with competitive employment and were suggestive of RTW potential.\nIn order to build on the gains the client made during CWH, the occupational therapist recommended the client follow a gradual RTW (GRTW) schedule upon returning to work with its inherent progressive increase in hours and progressive increase in job duties. The therapist recommended that multitasking and deadlines be avoided during the GRTW and that they be phased in gradually thereafter. Close monitoring was also recommended to ensure the client was coping with the work transition and was able to increase work hours and job demands. Access to job accommodations upon RTW is more typical among employees with high socioeconomic status (SES) and educational level () thus positioning this client in a likely advantageous position for RTW success.\nIntervention outcome is also noted through the client's pre- and post- questionnaire scores which indicated that her perceived work ability increased, fatigue decreased, and depression symptoms decreased (see ). These quantitative findings aligned with the clinical observations of the client's occupational functioning during CWH.\nThe increased perceived work ability from 3 to 7 (on a 10-point scale) reflected the client's recognition of the gains she made in cognitive abilities through experiential task mastery which contributed to her sense of efficacy and work ability. Reduced fatigue levels (from 31 to 24) reflected a decrease in the client's fatigue in her personal life (outside of CWH) consistent with her report of increased activity re-engagement. This outcome is noteworthy given that low activity is often a symptom of a depression and has been found to be predictive of repeated sick leave in a population of depressed patients (). Depression severity decreased from a score of 32 (“severe” depression) to 20 (“borderline” clinical depression). This aligned with the client's overall positive outlook to RTW by the end of her CWH program.\nThree months after program completion, the client participated in a semi-structured phone interview with the occupational therapist. This follow-up interview provided the client the opportunity to reflect on her experience of the intervention and to consider that experience in relation to her own RTW trajectory. The interview protocol was two-fold. First, it consisted of directed questions regarding the client's employment status to determine if she had returned to work or if she had remained on disability. Indeed this is considered to be the primary measure of RTW success (). If working, the client was asked to rate her sense of well-being at work along three pre-defined categories: (1) working/many issues (struggling); (2) working/some issues (coping); and (3) working/few or no issues (doing well) (). The second aspect of the protocol consisted of open-ended directed questions regarding the client's perceptions of and experiences with the CWH intervention given the time lag since participation in the intervention. This included inquiry into how the CWH intervention helped her prepare for RTW, what elements of the CWH intervention she found helpful, and if there were any aspects of the intervention she did not find useful or would have liked done differently.\nThe client reported that she was at work “doing well.” She noted that when she returned to work (after CWH), she adhered to an 8 week GRTW schedule which started at 8 h/week with a progressive increase in hours from week to week. At follow up, she reported to be working full time hours assuming a full workload with good stamina and concentration. She shared that the CWH intervention had been helpful and indeed prepared her to return to her pre-disability job. The client noted that the CWH experience had given her increased self-confidence and that the coping skills she had learned were indeed helpful once she was back at work. She also reported that she was seeing her psychiatrist every 3 months and no longer saw her psychologist. |
A 30-year-old female Caucasian was referred by her urologist because of persistent asymptomatic microhematuria and a suspicious finding on CT scan. The patient was naive to medication except oral contraception. Family history for malignancies in the upper and lower urinary tract, as well as for stone disease, was negative.\nShe was normotensive without pathologic findings in the physical examination. Laboratory findings were within normal range. Urinalysis demonstrated blood in the urine without proteinuria or signs of infection. A pregnancy test was negative.\nRenal- and bladder ultrasonography as well as the cystoscopy were without pathological findings.\nCT urography revealed a 5 mm filling defect in the left caudal calix (), so she was referred to our department to undergo retrograde pyelography and ureterorenoscopy to rule out upper urinary tract tumor.\nThe patient underwent cystoscopy with left-sided retrograde pyelography after sampling a urine cytology from the left renal pelvis. The urine cytology revealed papillary formations with nuclear atypia without malignancies (PAP III). The retrograde pyelogram showed the previously described contrast-filling defect in the caudal calix of the left kidney (). Because of a stenosis of the distal ureter, we placed a 7F Double-J stent and performed fURS 2 weeks later. The fURS was performed with a 9.9F video ureteroscope (Olympus, Tokio, Japan) and showed pathologic findings neither within the ureter nor within the renal pelvis. However, in two calices of lower pole, nodular calcified papillary structures were found (), which were both biopsied endoscopically and a 7F Double-J stent was placed again.\nA second urine cytology from the left renal pelvis was taken during fURS, with similar results as obtained at the initial intervention. The histology analysis (including hematoxylin and eosin staining, Berliner Blau and CK20) of the biopsies showed a reactive urothelial hyperplasia without any malignancies.\nAfter thorough communication with the patient about the benign cause of the AMH, we removed the Double-J stent without a subsequent surgery (e.g., ablation with a holmium-YAG laser). After 3 months, the patient was still asymptomatic and no changes were noted on CT scan with respect to contrast enhancement or the size of the lesion. |
A 14-month-old boy was referred to the Department of Pediatric Dentistry at Osaka University Dental Hospital with the chief complaint of early loss of primary teeth. Intraoral examinations revealed that the primary mandibular bilateral incisors and the primary mandibular left lateral incisor were missing. We previously described a patient with HPP who was diagnosed on the basis of early exfoliation of primary teeth []. The present patient exhibited a low level of serum alkaline phosphatase (192 IU/I); radiological examinations of the lower extremities and hands did not show metaphyseal irregularities indicative of rickets. Tissue-nonspecific alkaline phosphatase gene sequencing demonstrated a heterozygous c.550C > T (p.Arg184Trp) mutation. The patient’s condition was classified as odonto-type HPP, based on the results of these examinations.\nThe patient’s primary mandibular right lateral incisor spontaneously exfoliated when he was 2 years and 11 months of age; the primary maxillary left incisor also exfoliated when he was 4 years and 1 month of age. Partial dentures were applied for space maintenance; there were no problems regarding subsequent replacement with the corresponding permanent teeth (A). However, the primary mandibular right first molar was appeared to be submerged when the patient was 8 years and 3 months of age; the severity of submergence was greater when the patient was 9 years of age (B,C). The primary mandibular right first molar was about 1.5 mm submerged from the primary mandibular right second molar, and the periodontal ligament cavity was partially unclear (A,B). The primary mandibular right first molar was considered to be ankylosed, although the corresponding tooth in the opposite quadrant did not show similar findings. Thus, extraction of the affected tooth was performed (C). Histopathological analysis of the tooth showed disturbed cementum formation and acellular cementum, with wide and wavy ivory tubules and aplasia (D). The patient’s postoperative course was good, and no particular complications were observed. The mandibular right mandibular first premolar showed a tendency to erupt (D). |
A 51-year-old male patient had been suffering from a massive lymphedema of the lower extremities also including the scrotum for almost 20 years. Despite consequent lymph drainage up to four times per week, the patient showed progressive symptoms with suddenly occurring diarrhoea, a massive impairment of physical capabilities and a severe reduction in the quality of life. The patient's weight was consistent with 110 kg at a height of 184 cm [body mass index (BMI): 32.5 kg/m²]. Pulmonary, cardiac and hepatological diagnostics showed no underlying disease. Gastroscopy and colonoscopy gave no pathological findings. The only exceptional result was a protein deficiency with a chronic lowered total serum protein (47 g/l; normal range 64–84 g/l). Gel serum electrophoresis showed a general protein deficiency.\nSince conservative treatment with lymph drainage showed no improvement of the patient's symptoms, further diagnostic steps including a push–pull enteroscopy revealed the PIL of the jejunum to an extent of about 50 cm (). Subsequent capsule endoscopy confirmed the limited involvement of the proximal to mid jejunum.\nDue to the extensive impairment of the patient and the therapy-refractory course, the indication to exploratory laparoscopy with segmental intestinal resection was given.\nDuring the laparoscopic operation, an extensive part of the small intestine appeared macroscopically clearly altered (). Thus, the laparoscopic-assisted resection of the conspicuous intestine (95 cm) with end-to-side anastomosis was performed.\nHistopathological analysis confirmed the PIL with dilated submucosal and subserosal lymphatic vessels (Fig. a and b).\nThe patient was discharged on the seventh postoperative day. During an outpatient visit after 2 weeks, a slight improvement of the total serum protein to 52 g/l was obtained.\nAn outpatient follow-up after 6 months showed a reduction in the patient's weight from 110 to 105 kg (BMI: 31 kg/m²), and the frequency of lymph drainage had been reduced to twice per week. Physical capabilities improved, and diarrhoea has not occurred since 4 weeks after the operation. Total serum protein level was at the lower end of the normal range (63 g/l) with an otherwise normal serum electrophoresis. |
A 24 years old primigravida who had previous normal antenatal (ANC) visit along with USG at 17 weeks showed single live fetus at 16 weeks of gestation (WOG) without any gross fetal anomalies at Darchula district hospital, presented with the chief complaints of pain abdomen and per vaginal leaking for 1 day at 31 weeks of gestation in the same hospital and was referred initially to nearby tertiary care Seti Zonal Hospital. Further, she was referred to our centre (Patan Academy of Health Sciences). Repeat USG was done at 31 weeks which revealed severe oligohydramnios with amniotic fluid index (AFI) 1.5cm and intrauterine growth retardation (IUGR) with the expected fetal weight of 1365 grams only. Emergency lower segment caesarean section was performed with prior administration of 2 doses of dexamethasone (12mg) and prophylactic antibiotics to the mother. A female baby was born with a birth weight of 1.2 kilograms and was immediately transferred to the neonatal intensive care unit (NICU) due to respiratory distress. There was no gross congenital anomaly (no murmur) noted except the club foot. She was managed conservatively with intravenous fluids, antibiotics and non-invasive ventilation (Continuous positive airway pressure (CPAP) at 6 centimetre water) with an initial diagnosis of respiratory distress syndrome with a pre-term baby with very low birth weight. Further, NG tube insertion was tried but failed to pass beyond 10 cm for feeding purpose. Therefore, a red rubber catheter was inserted and a chest X-ray ()\nwas performed which demonstrated EA with TEF. Eventually, surgical correction was planned with all the pre-operative evaluation done to rule out other associated anomalies and complications. Right thoracotomy with ligation of the fistula with end to end anastomosis was performed without any perioperative complications ().\nAdditionally, a prophylactic chest tube was kept in situ and the NG tube was fixed. Furthermore, post-operatively continuous monitoring of vitals, haemoglobin, electrolytes, nasogastric and chest tube care, antibiotics coverage (Piperacillin-tazobactam + Metronidazole), and maintaining neutral neck position were done. Repeat chest X-ray on the first post-operative day (POD) showed atelectasis of the left lung and chest tube in situ on right (). NG feeding was started on the second post-operative day and endotracheal tube extubated on day three. Initially kept on RAM cannula and then on continuous positive airway pressure (CPAP). There was no collection in the chest drain. The upper gastrointestinal study was carried out on the 7th POD which was inconclusive with right-sided pneumonia. Antibiotics upgraded to Meropenem and Colistin. Fluoroscopic evaluations on 9th POD demonstrated patent oesophageal lumen with dye in the stomach without tracheal and mediastineal spillage and normal expansion of the bilateral lungs field (). Therefore, oral feeding (as tolerated) started on the same day followed by oro-gastric feeding; however, sucking reflex was absent. The chest tube was removed on the 10th POD. Complete breastfeeding commenced on 21st day and transferred to the children ward and subsequently discharged at weight 1900gm. The patient came on emergency after 2 weeks with complaints of choking, chest X-ray done which revealed oesophageal stricture which is one of the early post-surgical complications (). The oesophageal stricture was endoscopically managed with balloon dilatation. She is under regular follow-up and since has been uneventful. Her current weight is 10 kg at 18 months of age. |
A 44-year-old woman was diagnosed with iron deficiency anemia but showed no abnormalities on gastrointestinal tract endoscopy 5 years prior to the current presentation. A blood test for health screening showed anemia with hemoglobin 7.6 g/dL, and uterine fibroids were suspected on abdominal ultrasonography. She was diagnosed as having an intra-abdominal tumor on magnetic resonance imaging (MRI) for detailed examination and was referred to our hospital.\nThe abdomen was flat and soft, with an elastic mass of poor mobility which was the size of an infant's head was palpable below the umbilicus to above the pubis. There were no blood test abnormalities; CEA, CA19-9, SCC, and the interleukin 2 receptor level were within normal limits. Abdominal MRI revealed a homogeneous and well-demarcated 74 × 98 × 122 mm mass near the cranial end of the uterus, with a low signal intensity on T1-weighted image, and mostly low signal intensity and partially high intensity on T2-weighted image (Figures and ). Abdominal-enhanced computed tomography (CT) showed a well-demarcated and contrast-enhanced oval mass with a smooth margin in the pelvis. The tumor was supplied by the superior mesenteric artery, and the surrounding lymph nodes were enlarged (Figures and ). Upper gastrointestinal tract endoscopy showed an easily bleeding tumor with an ulcer in the third part of the duodenum, involving half the circumference (). The biopsy results were inflammatory exudates and granulation tissues. FDG-PET/CT showed heterogeneous uptake inside the tumor with SUVmax 6.3 (Figures –). A slight 18F-FDG uptake was observed in the enlarged lymph nodes with SUVmax 2.6. Along with the facts that there were no malignant cells detected by endoscopic biopsy and the CT image showing enlarged surrounding lymph nodes, the patient was suspected of nonepithelial malignancy of the duodenum such as gastrointestinal stromal tumor (GIST) and malignant lymphoma. As we could not make a definite diagnosis preoperatively, she underwent surgery for an accurate diagnosis and treatment.\nSurgery was performed under general anesthesia; laparotomy was performed via midline incision from 5 cm above the umbilicus to above the pubis. There was a 12 cm tumor centered on the third part of the duodenum, extending to the fourth part. The tumor extended into the pelvis and pulled the duodenum and the ligament of Treitz caudally (). There was no infiltration of the tumor into the pancreas or the small intestine. The duodenum was transected at the third part of the duodenum on the oral side. On the anal side of the tumor, the jejunum immediately after the ligament of Treitz was transected, and the tumor was resected. The enlarged lymph nodes sampling were also performed. In reconstruction, end-to-end duodenojejunostomy was performed (). She had an uneventful postoperative course and was discharged 9 days after surgery.\nPathological findings showed a solid tumor growing from the muscle layer of the duodenum through the serous membrane. The duodenal mucosa was invaginated at the site of the tumor, forming a deep ulcer (). The tumor showed uniform growth of long and spindle-shaped cells in an intricate manner, and the number of nuclear divisions was 1 or less per 50 visual fields (). There were no evidence of calcification, hemorrhage, or degeneration within the tumor. On immunostaining, α-smooth muscle actin was positive, while S-100, c-kit, and CD34 were negative (Figures –). The MIB-1 index was low at 3% maximum, and there were no neoplastic lesions in the enlarged lymph nodes. Based on these findings, a leiomyoma was diagnosed. Since then, no relapse has occurred for two years and six months. |
A 38-year-old woman (G6P3023) at 24 weeks gestation presented with vaginal bleeding. She reported that 1 week before she awoke in a “puddle of fluid.” She denied gross hematuria. She had a history of 3 Caesarean sections. Fetal ultrasound showed complete placenta previa with placental vessels invading the bladder confirming PP (). She was admitted for expectant management. Maternal fetal medicine, anesthesia, neonatal intensive care, and urology were all consulted.\nMagnesium sulfate, antibiotics, and steroids were administered prophylactically. On hospital day #2, the patient had an increased oxygen requirement and tachycardia. A computed tomographic scan of the chest revealed extensive bilateral pulmonary emboli. She underwent inferior vena cava filter placement, was transferred to the surgical intensive care unit, and continuous heparin infusion was initiated.\nOn hospital day #6, the patient went into labor and was taken to the operating room for a multidisciplinary procedure. She underwent exploratory laparotomy and repeat Caesarean section through a fundal uterine incision by the obstetrics team. A viable female neonate was delivered with Apgar scores of 9 and 9. A total abdominal hysterectomy and lysis of adhesions were then performed by the gynecologic oncology service. The anterior uterine wall was then recognized to be affixed to the bladder. Dissection of the anterior uterine wall from the posterior bladder was accompanied by large posterior cystotomy. On routine inspection, decreased efflux was noted from the right ureteral orifice, and the right ureter was markedly dilated.\nAt this point, intraoperative urology consultation was requested. The right ureter was secured, and a suture was identified that appeared to be constricting it. This was released with immediate return of urine from the ureteral orifice. A double-J ureteral stent was placed, and cystorrhaphy was performed. No leak was identified on bladder irrigation, and an omental flap was placed between the bladder and the vaginal cuff. A Jackson-Pratt drain and a Foley catheter were placed. In total, the patient required resuscitation with 37 units of various blood products.\nThe patient's postoperative course was complicated by intermittent fevers and multiple blood transfusions. A voiding cystourethrogram (VCUG) was performed on postoperative day (POD) #14, which demonstrated a small leak from the posterior bladder wall. Foley catheter was maintained, and a repeat VCUG was performed on POD #21 showing persistent leak. She was discharged home with a Foley catheter in place.\nAt her follow-up visit on POD #39, a VCUG revealed resolution of the leak, and the Foley catheter was removed. The patient's ureteral stent was removed 11 weeks postoperatively. |
A 37-year-old male was seen in the outpatient clinic because of weight loss, fatigue, and abdominal discomfort. Current height was 175 cm, weight 67 kg, with an unintended weight loss of 6 kg over the last few months. There had been symptoms of fatigue, which the patient attributed to increased workload. The abdominal discomfort included distention without pain or nausea. There were normal and unchanged stools and no icterus.\nThe patient had no other symptoms at presentation and showed a good performance status. At age 8, he had been diagnosed with right-sided WT with metastases to lymph nodes and lungs, and with thrombosis of the inferior vena cava and right renal vein. He was treated with intended curative right-sided nephrectomy and thrombosis evacuation, in combination with adjuvant radiation and chemotherapy. He received chemotherapy pre- and postoperatively with dactinomycin, actinomycin D, and vincristine, and 20 cycles with 30 Gy of radiation treatment to the right side of the abdomen for lymph node metastases. There was a cover over a large portion of the right lobe of the liver.\nThe patient had mildly elevated liver enzymes during the pre- and postoperative chemotherapy, with a maximum ASAT at 224 U/L (10–40), an elevated alkaline phosphatase level between 350 and 650 U/L (80–250), with persistent normal γ-glutamyltransferase.\nThe liver enzymes normalized after the end of treatment. There was no evidence of hepatomegaly, jaundice, ascites or liver pain during the 10 years of follow-up after radiation and chemotherapy. Follow-up included periodically clinical evaluation, blood tests, whole-body CT scan, ultrasound of the abdomen, and a plain thoracic X-ray. Blood tests were normal apart from a moderate thrombocytopenia at 90–125 × 109/L (150–450). Radiographically, there were venous collaterals at the level of the previous inferior vena cava thrombosis. In addition, ultrasound showed slowly progressive splenomegaly to 16 cm developing over the years, which was not further investigated. Five years after treatment, an abnormal ultrasound pattern was observed in the liver parenchyma without signs of cirrhosis and normal flow in the portal and liver veins; no further action was taken. At the age of 18, after 10 years of follow-up, the patient was discharged in a clinically stable condition. Hereafter, the patient had had no contact with the health care system until the current admission.\nOn admission, aged 37, the patient's physical presentation was normal, apart from the findings of cutaneous abdominal varices and an enlarged spleen. Laboratory examinations showed elevated ALAT at 115 U/L (10–70), alkaline phosphatase at 199 U/L (35–105), low albumin at 35 g/L (36–48), normal bilirubin at 22 μmol/L (5–25), and thrombocytopenia at 75 × 109/L (145–350). Other results, including a full blood count and screening for metabolic, viral, and autoimmune liver disease, were all normal. A CT scan of the thorax/abdomen showed no sign of malignancy, but hypoplasia of liver segments 2 and 3 was seen, a reduced perfusion in the liver segments 6 and 8 as well as splenomegaly at 18 cm. In addition, several enlarged lymph nodes (1–3 cm) were observed in the retroperitoneum and around the celiac trunk and radiation sequelae in the right colon (Fig. ). A lymph node biopsy showed reactive changes without evidence of malignancy and bone marrow biopsy was normal. A colonoscopy revealed minor inflammation, histologically compatible with radiation sequelae.\nTwo months after initial presentation, the patient developed ascites and new imaging with CT and ultrasound showed otherwise unchanged conditions. A gastroscopy revealed grade 2 esophageal varices.\nA needle biopsy of the liver was performed. This revealed an enlargement of most portal tracts, with varying mild to moderate fibrosis. There was some degree of periportal fibrosis, but only occasional short septa with no evidence of bridging fibrosis or of cirrhosis. Several of the fibrotic portal tracts showed evidence of obliterative portal venopathy, normal portal veins being either lost (with evidence of replacement by many smaller vascular structures), or showing sclerosis with fibrous thickening of the portal vein wall (Fig. ). In other portal areas, portal veins were dilated with herniation into the surrounding liver parenchyma (so-called periportal shunt vessels; Fig. ).\nA combined liver vein and spleen pulp pressure measurement was performed as previously described [] and showed a normal pressure gradient across the liver (5 mm Hg), but a high gradient from the spleen to the free hepatic vein (29 mm Hg). This confirmed the expected presinusoidal hepatic origin of the patient's NCPH.\nThe patient was treated with loop diuretic medicine and an aldosterone antagonist for ascites and propranolol was administered to reduce the portal pressure gradient. As a result, the patient became asymptomatic without ascites and is able to work full time. |
We report the case of a 58-year-old man (50 kg body weight) with a right-sided MRSA PPE. He was readmitted to the intensive care unit critically ill with severe dyspnoea, fever (maximum 42 °C), pancytopenia and coagulopathy. Fourteen weeks earlier he underwent extended right pneumonectomy with partial resection of the left atrium, resection of the pericardium and Vicryl mesh coverage. Three days before readmission the second cycle of adjuvant chemotherapy (doxorubicin and ifosfamide) was applied. The R0 lung resection had been performed in April 2013 for a central pulmonary high grade sarcoma (NOS) measuring 15.3 × 13.3 × 10.0 cm and involving the right middle and lower lobe. The bronchial stump had been covered with a pericardial fat pat.\nAfter readmission the patient was stabilized with intravenous (IV) antibiotics (meropenem 500 mg td, and vancomycin 500 mg bd) and thoracoscopic empyema debridement with the introduction of two drains, followed by daily irrigation with 1.5 L of normal saline. MRSA was isolated in the pus as well as in the nose and bronchial system. The calculated antibiotics were deescalated to IV vancomycin given 1 g bd. After 3 weeks MRSA still persisted in the irrigation fluid, and thus 1 g of vancomycin was instilled into the cavity after each irrigation. Seven days later MRSA still persisted in the fluid and thus the treatment strategy was changed (). Open re-debridement with extensive washing with hydrogen peroxide and gauze packing with vancomycin-solution was performed three times every third day, combined with a latissimus muscle myoplasty () to cover the pericardial mesh and bronchial stump region as well as to reduce the residual space. The muscle was attached to the mediastinum with several polydioxanone sutures and fixed with another layer of antibiotic gauze. Wound closure with removal of the gauze and instillation of another 2 g of vancomycin took place 9 days after the first open debridement. The final microbiology was negative. The treatment was accompanied by superficial measures to eradicate MRSA from the skin, throat and nose.\nIV vancomycin and intensive irrigation with normal saline did not eradicate MRSA, but allowed the patient to recover from severe septicemia. Filling the cavity with vancomycin solution was not effective either. The irrigation fluid remained cloudy and positive for MRSA (). The open debridement demonstrated a thick peel formation with fibrin and pus attached all over the cavity. Two further debridements were necessary to gain adequate clearance before muscle coverage was possible (). The patient tolerated all operative procedures and recovered, gained weight and was fully mobilized. He was discharged 17 days after the latissimus myoplasty. The pleural fluid aspirate 3 weeks and also 3 months later as well as the nose swabs and sputum were negative for MRSA, and the patient was free of inflammatory signs. Computed tomography of the thorax demonstrated no abscess formation and no signs of tumor recurrence at 3 months (). |
An 18-month-old female previously had a tracheostomy placed in infancy to treat an airway obstruction secondary to RS. The recommendation was to first correct the cleft palate, followed by mandibular distraction in order to decannulate the child.\nThe cleft palate was closed at 19 months of age. A 2-flap palatoplasty with levator retropositioning was performed. The patient’s postoperative course was uneventful. Two months following cleft palate closure, the patient was re-evaluated for mandibular distraction. A preoperative computed tomography (CT) scan demonstrated an incidental cystic lesion of the left mandibular ramus measuring 3.9 cm in length and 2.2 cm in the transverse dimension with a floating molar tooth. The mass was unilocular and distorted both cortices of the mandible. It appeared to be emanating from the second molar on the left side and extended to the level of the condyle. In order to obtain a definitive diagnosis, the decision was made to enucleate the mass.\nAt the age of 25 months, the patient underwent enucleation of the mandibular cyst. A subperiosteal exposure was performed. Upon making the osteotomy, copious amounts of yellow-colored, almost purulent fluid were expressed. The mass had obliterated both cortices of the bone. The floating teeth and surrounding bone were removed and curetted. The periosteum was intact and the only portion of viable bone was along the posterior border of the ramus. No bone grafting was performed (, ). The intraoral incision healed without any complications and observation was recommended.\nSurgical pathology identified the mass as a dentigerous cyst. Multiple sections revealed a cyst composed of dense fibrous tissue lined by squamous epithelium. Ameloblastic epithelium was not identified, and there was no evidence of carcinoma. Culture of the cyst aspirate showed the growth of many beta-hemolytic group F streptococci and possible anaerobic species. Also present were 2 molar-like tooth structures, which measured 1.1 cm ×1.0 cm×0.6 cm and 1.1 cm×0.9 cm×1.2 cm, with the crown aspect of 1 of them projecting into the lumen of the cyst.\nAt 3 and a half years of age, the patient continued to demonstrate an inability to tolerate capping of the tracheostomy. An approximately 16-mm overjet was present centrally. The tongue remained posteriorly displaced. A CT scan showed a regenerated mandibular ramus that could accommodate a distraction (, ).\nAt that time, the patient underwent successful bilateral mandibular osteotomy and distraction. Approximately 2.5 months later, the internal distractors were removed. Two months later, she was decannulated. A follow-up CT scan performed 2 years postoperatively demonstrated bone stock after distraction (). |
In 2012, a 13-year-old boy was brought into the emergency department (ED) of our institute about 6 h after being injured by a winnowing fan blade. He complained of headache and one episode of vomiting. After the injury, he was unconscious for about 10 min following which he regained consciousness. He did not have seizure or auro-nasal bleed. In the ED, he was conscious and had a tender closed wound with an obvious palpable defect in the skull topography; a depression of 1.5 cm bone fragment pushed to a depth of approximately 1 cm from the skull silhouette. Noncontrast computed tomography (NCCT) scan of the brain and skull showed a depressed fracture in the left frontal region with underlying hypodense area showing edema [Figure and ]. No underlying hemorrhagic contusion, extra- or sub-dural bleed was, however, visible.\nSurgery was offered to the patient and his family; however, the family was reluctant and denied any surgical intervention. Despite multiple counseling sessions by many health-care consultants and neurosurgeons, the family left the hospital against medical advice 2 days after admission. We tried to follow-up with the family 1-month after the admission, but they said that the patient was asymptomatic and did not need any medical attention. Subsequent follow-up attempts failed because of unresponsive family members.\nIn 2014, the patient was readmitted at our institute with complaints of two episodes of seizures in the previous 12 h. There was no history of injury or any neurological symptoms in the intervening 2 years. A repeat NCCT scan of the brain and skull showed no defect in the bony topography of the skull []. There were, however, bifrontal hypodense areas, more on the left side, suggestive of gliosis []. No neurosurgical intervention was required. The patient was discharged 2 days later, with advice of two antiepileptic drugs. He returned to the ED with another episode of seizure, 42 days after the previous admission. The patient has been symptom-free for more than 6 months since the adjusted dose of antiepileptic drugs. |
A 61-year-old Caucasian male presented at our Department of Urology (Ferrara, Italy) with an history of dysuria, urinary frequency associated with a single episode of hematospermia. For about two weeks prior to his admission he had been taking antibiotics prescribed by his general practitioner for a presumed diagnosis of prostatitis. He was put on ciprofloxacin 500 mg orally twice a day for two weeks. Despite taking medications his symptoms persisted. His past history was remarkable for kidney stones and hypertension. On physical examination, oral temperature was 36.0°C (96.8°F), blood pressure 140/90 mmHg, heart rate 85 beats/min and pulse oximetry 98% on room air. Rectal examination revealed a regular prostate in size and consistency but with a not particularly hard fluctuant extra prostatic mass of approximately 1 cm in diameter on the left-posterior base of the gland. Full blood count, serum urea, electrolytes, liver function test, serum amylase and serum prostate specific antigen levels (PSA: 3.4 ng/mL) were within normal limits. There was no growth in his urine culture; his urine cell count determined by flow cytometry revealed: white blood cells 7 u/L, red blood cells 124 u/L, normal epithelial cells. Reviewing his medical history showed that 1 year before he underwent TRUS prostate biopsy because of his high PSA (5.8 ng/mL) detected on routine check-up. TRUS findings consisted in benign prostatic hyperplasia and the histopathological features of 14 biopsy specimens showed benign prostate tissue with elements of chronic inflammation. Transabdominal ultrasonography showed a simple right renal cyst of 3 cm in diameter. TRUS was used for the diagnosis () and confirmed the origin of the lesion from the left SV without infiltration of rectum, bladder or prostate. With TRUS we observed a left SV increased volume that contained inhomogeneous mass vascularized on color-Doppler sonography. The mass had irregular borders and the size was estimated by the formula: D1=1.1 cm × D2=1.4 cm × D3=0.8 cm (D1, the transverse; D2, the anteroposterior; D3, cephalocaudal dimension of SV mass). Transrectal ultrasonography-guided biopsy of the mass lesion in left SV was performed. The pathological analysis of the biopsy specimen revealed an adenocarcinoma exhibiting papillary and tubulopapillary structures characterized by polygonal cells with pleomorphic nuclei, clear cytoplasm and extracellular mucin deposition (). The cells were uniformly strongly immunoreactive for cytokeratin-7 (CK-7) and carcinoembryonic antigen (CEA). There was no immunoreactivity for PSA, prostate-specific acid phosphatase (PSAP) and CK-20. Additionally, these tumors have been reported to be also positive for CA-125. Therefore a combination of positive staining for CK-7, CEA and CA-125; with negative staining for CK-20, PSA and PSAP is the pattern of immunohistochemical findings noted for this rare tumor. The computed tomography (CT) of the abdomen-pelvis and chest X-ray was negative for metastases. A month later the patient underwent a Radical Prostatectomy and pelvic lymph nodes dissection with laparoscopic technique. The final pathology report confirmed the result of the SV biopsy. Patient did not receive other therapy in a neoadjuvant or adjuvant setting. The patient more than three years after RP has a good performance status without any symptom, and with negative imaging for metastases in the follow-up. |
A 70-year-old woman underwent mitral valve surgery for mitral stenosis with heart failure. The mitral valve replacement with bioprosthesis under cardiopulmonary bypass was uneventful. At 4 hours after arrival to the intensive care unit, she was withdrawn from the ventilator and had stable circulatory and respiratory conditions.\nOn postoperative day (POD) 1, wheezing was heard on the left lung field, and chest X-ray demonstrated opacity on the left lung (Figure ). She did not complain of dyspnea, and her oxygenation was kept normal; we placed her on noninvasive positive pressure ventilation to expand the left lung. On POD 2, computed tomography scan revealed collapse of the left lung and marked exudate in the left main bronchus (Figure ). Therefore, she was intubated and was placed on high positive pressure mechanical ventilation.\nThe first bronchoscopy on POD 3 showed secretions. Treatment with beta-agonist bronchodilator and inhaled mucolytic was continued for several days, but the left lung condition did not improve. On the second bronchoscopy on POD 8, there was a rubbery and mucinous bronchial cast, which was extracted (Figure ). On histologic examination (Figure ), the cast mainly comprised mucous material and few histiocytes; there was no eosinophils and neutrophils, which indicated acellular plastic bronchitis. Based on these findings, she was diagnosed as type 2 plastic bronchitis localized to the left lung. Cultures taken from the cast were negative. We started steroid therapy and attempted sputum drainage by active postural changes for the next 2 weeks. Several casts were drained recurrently, but the air entry to the left lung did not improve and bacterial infection developed in the damaged left lung. We started her on culture-guided intravenous antibiotic therapy, but the infection spread to the right lung rapidly, causing critical hypoxia despite the maximum mechanical ventilator support. She died the following day. Postmortem examination was declined by the family. |
A 53-year-old woman presented with a partial motor seizure, with postictal transient right-sided hemiparesis. Brain MRI showed a non-enhancing deep-seated lesion in the left cingulate gyrus, extending to the mid-/posterior corpus callosum inferiorly and to the paracentral gyrus superiorly. Preoperative fMRI of hand clenching, finger tapping, and toe wiggling tasks showed BOLD activations in the anatomically expected M1 location and more anteriorly in the superior frontal gyrus. DTI tractography using fMRI BOLD activation areas as seed ROIs was used to generate the left CST and the left FAT. A 3D rendering of preoperative T1 post-contrast MRI reconstruction highlighted two prominent cortical veins entering the superior sagittal sinus in the planned craniotomy. Taken together, preoperative imaging suggested a narrow window for a safe operative corridor to the tumor.\nA left frontal craniotomy with transcranial and direct cortical motor evoked potentials as well as subcortical motor mapping was planned. The patient was positioned supine with head neutral and 30 neck flexion. Via a linear incision a craniotomy was performed to expose the superior sagittal sinus and coronal suture. iUS-1 confirmed adequate exposure of the tumor (). A second iUS (iUS-2) was performed after the dura mater was opened to validate the continued accuracy of neuronavigation; there was a potential for a brain shift in the anteroposterior direction due to cerebrospinal fluid (CSF) drainage but none was observed. We attempted an interhemispheric dissection, which was limited by the bridging veins merging with the sinus along its length, prompting pursuit of a transcortical approach instead. The cortical entry point was defined between the two hand and foot activation areas connected by the corticospinal tract and the frontal aslant tract, using a combination of motor mapping and navigating the iUS-2 volume with the preoperative MRI (). The anterior-most and superficial part of the tumor was well differentiated from the surrounding brain, but the posterior and deeper portion was only partially distinguishable. Surgical resection was pursued posteriorly until subcortical stimulation of motor tract responses reached a threshold of 2.5 mA. A third iUS sweep (iUS-3) indicated some residual tumor in the anterior-most part of the cavity, cloaked by a sheath of arachnoid in the cingulate sulcus (). iMRI at this point confirmed residual tumor in both the anterior and posterior aspects of the surgical cavity (). Additional microscopic resection of the anterior residual tumor was achieved. In the early postoperative period, the patient exhibited right-sided hemiparesis, most prominent on lower extremity with preserved tone, with near-complete recovery by postoperative Day 10 barring minor gait apraxia, and a full recovery after 3 weeks consistent with a supplementary motor syndrome. Pathology was compatible with glioblastoma multiforme |
In 2009, a 30-year-old woman, native of Karachi (Sindhi ethnicity), presented at our clinic with a 3-year history of discharging sinus of her right hip and inability to ambulate. She had a history of right proximal femur fracture secondary to a trivial trauma 11 years earlier (Figure ). She initially remained under treatment of bone setters for 3 months and then she underwent fixation (in 1998) with dynamic hip screw (Figure ) in a tertiary hospital without a biopsy being done although history and X-rays were suggestive of pathologic fracture. She remained symptomatic postoperatively and gradually the implant cut through the femoral head (Figures and ). In 2007 the implant was removed (Figure ) in another tertiary hospital and tissue was sent for histopathology which showed hydatid disease. Postoperatively she developed discharging sinus for which she underwent multiple debridements along with prolonged courses of albendazole but her condition did not improve.\nIn 2009, she was offered a hemipelvectomy after which she visited our hospital. On examination she was unable to bear weight on her right leg. Her right leg was shortened by 4cm; the girth of her right thigh was increased by 8cm. There were multiple discharging sinuses over her right thigh and right hip with seropurulent discharge. There were multiple scars over the lateral aspect of her right proximal thigh with limited, painful range of motion of her right hip. Distal neurovascular status was intact. X-rays were performed that showed grossly extensive involvement of her proximal femur and acetabulum (Figures A, B and C). Magnetic resonance imaging (MRI) of her pelvis was done which showed erosions and altered signals in right proximal femur, acetabuli, ischium pubic rami and right ilium (Figures , and ). There was atrophy of her hip muscles, scarring and lymphatics obstruction. Her uterus, ovaries and opposite hip were unremarkable. An ultrasound of her abdomen was unremarkable. Options of hemipelvectomy versus a limb salvage form of modified internal hemipelvectomy and wide margin resection were explained to her. The second option had a risk of recurrence and infection but she opted for the same.\nIn March 2009, an internal hemipelvectomy with wide margin resection of soft tissue and proximal femur was done (Figures A and B). Perioperatively frozen sections were sent from resected margins that were reported as disease free (no cyst wall, no scolices). Postoperatively she remained on skeletal traction and received albendazole therapy for more than 3 months. As her wound and infection parameters gradually improved she underwent reconstruction with free vascularized fibular graft along with locking compression plate (LCP) in August 2009 (Figures A and B). Postoperatively a hip spica was applied. Follow-up imaging was done to assess union (Figures A and B; Figures A, B and C). Gradually her ambulation progressed and she was able to ambulate without support after a long time.\nShe remained ambulant ‘as tolerated’ until February 2013 when she had a fall at home and sustained right patella fracture for which tension band wiring was done (Figures A to E). Her postoperative recovery remains smooth and now, in August 2014, 60 months (5 years) postoperative from her reconstructive surgery she is ambulant without any support. At present she is infection and disease free. |
A 19-year-old female was evaluated at the Department of Adult Psychiatry Outpatient Clinic with complaints of lack of enjoyment of pleasurable activities, sleep disturbances, suicidal thoughts, and self-induced injury at times of high stress via superficial cutting behaviors on her forearms. These symptoms were in association with ongoing family conflict with her mother and her sister with whom she lived.\nAt the psychiatric evaluation, she expressed that her self-esteem was strongly affected by her turbulent family relationships, and she seemed extremely unhappy. She reported superficial cutting behaviors at least 2 times a week, usually in an impulsive fashion. Laboratory evaluations including hemogram, liver function tests, total protein, vitamin B12, folic acid, T3, T4, and TSH were within normal limits. Baseline psychiatric evaluation with the Beck Depression Inventory (BDI-II) revealed scores of 21 (moderate depression) []. According to clinical evaluation as well as DSM-V criteria, the patient was diagnosed with major depressive disorder and borderline personality disorder, and she was started on citalopram 20 mg by mouth per day []. Cognitive behavioral therapy focusing on negative cognitions was also initiated. Partial response to treatment was observed at the 12th week with reduction of BDI-II score to 12 (mild mood disturbance).\nWhile she was under follow-up at the Department of Adult Psychiatry Outpatient Clinic, she admitted to a prior ingestion of a portion of a small bottle (less than 30 milliliters per her description of the product packaging) of a commercially available chemical skin exfoliator containing TCAA. This consumption was impulsive per her description and occurred after an argument with her mother. While such products available over the counter can typically range from 8% to 30% TCAA ingredient which determines strength, she did not recall what percentage of TCAA was in the product []. She had purchased the TCAA peel to brighten and even out her skin tone by reducing the appearance of hyperpigmentation (acne marks and freckles). She believed the product to also contain antidepressant TCA, since the product was marketed per her report as a “TCA peel,” which is often done in the industry. She knew that TCA antidepressants can be extremely damaging or even fatal in overdose which was her intent. TCA effects in overdose are well documented in the literature []. Her symptoms after ingestion were burning pain on her lips and in her mouth, which frightened her somewhat, preventing her from consuming more of the product. She also complained at the time of mild abdominal discomfort followed by an episode of vomiting. Fortunately no other symptoms were reported and all of the symptoms resolved within a few hours. She was asymptomatic at the time of reporting this event to the outpatient clinic. |
A 72-year-old female with an unremarkable medical history presented to the Department of Otolaryngology-Head and Neck Surgery at Athens Medical Center (AMC), with a history of an eight-month-long right swelling in the region of the supraclavicular fossa. No other clinical symptoms were mentioned. Clinical examination revealed a firm painless mass. Examination with rigid endoscopes was unremarkable. The patient underwent neck ultrasonography, which displayed a multinodular thyroid of 0.1-0.3 cm nodules and a well-defined mass of 4.5 x 2.5 x 2 cm situated in the lower pole of the right thyroid lobe. CT showed a vascular mass in the right supraclavicular region and in close proximity to the lower pole of the right lobe of the thyroid. Furthermore, three-dimensional CT angiography demonstrated a vascularized mass mainly supplied by the subclavian artery, the common carotid, and the innominate (Figures , ).\nThe results of two attempts of FNA on the mass were inconclusive while the third attempt suggested the presence of an ectopic thyroid tumor. FNA on the thyroid was also conducted, which revealed atypical follicular colloid cells of the Bethesda 3 category. Vanyllylmandelic acid was not examined. Thyroid hormone levels were within the normal range. Taking these findings together, we presumed it was an ectopic thyroid tumor in close proximity to the lower pole of the right lobe. The surgical plan consisted of a right hemithyroidectomy together with full excision of the presumed ectopic thyroid tumor. Preoperative embolization was not carried out. Surgery was successfully executed, even though aggressive intraoperative bleeding occurred during the dissection of the mass. Due to the proximity of the tumor to major vessels, a vascular surgeon was present during the operation. Postoperative recovery was uneventful with no complications. After pathological examination and verification of histological results, which provided a surprise finding, epinephrine and norepinephrine were examined and were found within normal ranges. A PET-CT was performed two months after the surgery and revealed the absence of residual tumor. Genetic testing revealed no relationship with any SDH mutations. |
A 55-year-old woman was referred to the Peking Union Medical College Hospital in September 2015 for rectorrhagia and intermittent abdominal pain lasting 6 months. The patient had a past medical history of ovarian endometriosis and had undergone excision of bilateral ovarian chocolate cysts in 1988 when she was 30 years old. Histological examination showed benign bilateral endometriosis of the ovaries. The patient was not treated with any hormonal therapy following hysterectomy. She has a familial history of endometriosis comprising her mother, one sister and one aunt.\nPhysical examination of the patient was unremarkable, except for the tenderness of the left lower quadrant of the abdomen.\nSerum CA125, CEA, and CA199 were within a normal range. A colonoscopy was performed (Fig. ), which revealed an ulcerated fleshy neoplasm that was 15 cm from the anal margin and blocked 50% of the passage of the endoscope. It was possible to pass the endoscope beyond the lesion. The mass appeared to bleed relatively easily and had a diameter of 2 × 3 cm. The surface of the lesion was irregular, and the margin was unclear. The surrounding colonic mucosa was rough. The results of the scan also revealed a 0.8 cm sessile polyp in the ascending colon. Multiple endoscopic biopsies were taken. Pelvic ultrasound showed multiple uterine leiomyomas, which presented as multiple heterogeneous internal echoes. A computed tomography scan revealed eccentric thickening of the wall of the recto-sigmoidal junction (Fig. ). A whole body FDG-positron emission tomography (PET) was requested (Fig. ). F18-FDG PET/CT imaging showed local thickening and narrowing of the recto-sigmoid colon wall and hypermetabolic lesions. FDG-PET/CT also found enlarged pelvic lymph nodes with pathologic FDG-uptake. Since the pathology obtained from the colonoscopy showed evidence of a metastatic adenocarcinoma, and the lesion was confined to the pelvic cavity, laparoscopic surgery was performed on October 20, 2015. During the procedure, a 3.5-cm ulcerated mass was identified above the peritoneal reflection with multiple adhesions to the uterine wall. The surgeons examined the pelvic cavity carefully and found apparent adhesions in the pelvic cavity due to the previous surgical procedures. The adhesions present between the tumour and the uterus were severed. There were no obvious tumours on either of the ovaries. A rectal anterior resection with lymphadenectomy was performed. The lymph nodes around the common, internal, and external iliac arteries, the middle rectal artery root, and the obturator space were dissected. Hysterectomy and bilateral salpingo-oophorectomy were performed. There was no residual macroscopic tumour present at the completion of the surgery.\nThe endoscopic biopsy specimens were fixed in 10% buffered formalin and embedded in paraffin. Four μm sections were stained with standard haematoxylin and eosin (H&E). Immunohistochemical stains were performed using the following antibodies: CK7, CDX-2, CA-125, Villin (Dako); CK20 (OriGene Technologies); PAX-8, WT-1(XiYa Biology); P16, ER, PR (VENTANA); P53 (Maixin Biotech. Co., Ltd.). The H&E slides and immunohistochemical stains were reviewed by two pathologists. The excised bowel was sent for histological analysis. The pathologic specimen consisted of an ulcerated mass with grey, solid, friable cut surface. The mass measured 3.5 × 2 × 1.2 cm and was attached to the wall of the colon. The mass involved the muscularis propria macroscopically. Microscopic sections revealed a high-grade serous carcinoma extending through all of the layers of the intestine (Fig. ). Tumour cells appeared as irregular tubular structures, moderately differentiated, with no sign of atypical adenomatous hyperplasia. The section margins were free of tumour, but the circumferential resection margin (CRM) was positive. Eight out of 30 lymph nodes that were collected were positive. The uterus and the ovaries were free of cancer. According to the preliminary pathological diagnosis, the TNM staging was determined to be pT4bN2bM0 (IIIC) based on the AJCC Staging System. The final histology was confirmed after discussion between several pathologists who diagnosed the patient with pronounced focal atypical endometriosis in the intestinal wall and the right ovary (Fig. ). The immunohistochemical profile showed CK7 positivity and CK20 negativity, as well as negative CDX2 (Fig. ). The case was positive for CA125, PAX8, P16 and mildly positive for oestrogen receptors. The Ki67 index was 30%. Based on the immunohistochemical characteristics of the primary intestinal adenocarcinoma, it was determined that the lesion was an endometrial carcinoma deriving from colorectal endometriosis.\nThe patient received adjuvant chemotherapy consisting of 175 mg/m2 Taxol and AUC5 carboplatin for eight cycles. The patient experienced reversible chemotherapy-induced myelosuppression and gastrointestinal reactions during chemotherapy. The timeline of the process was shown in Table .\nAt 23-months, the patient had a follow-up appointment and reported difficult defecation at the 22nd month. The CT scan, PET-CT and colonoscopy showed local recurrence in the lower rectum, which was confirmed by pathological diagnosis. The patient has received Taxol and carboplatin chemotherapy again and will receive another surgery after two cycles of chemotherapy. |
An 86-year-old man was referred to our hospital for the treatment of EGC. A tumor was located on the anterior wall of the antrum. Endoscopically estimated tumor size was 1.2 cm (). Significant perigastric lymphadenopathy and distant metastasis were not found on abdominal computed tomography (CT). ESD was successfully performed in en bloc fashion, and all resection margins were tumor-free (). Resected specimen size was 4.2 cm. Total procedure time was 25 minutes. No free air was observed on chest radiography performed immediately after ESD (). Pathological analysis of the ESD specimen indicated that the lesion was a 0.8 cm-sized moderately differentiated adenocarcinoma confined within the lamina propria layer. No lymphovascular invasion was detected. Twelve hours after the ESD procedure, the patient complained of severe abdominal pain. Because he did not have fever and a follow-up chest radiography at that time revealed no free air (), the patient was conservatively managed with a proton pump inhibitor and pethidine. Twenty-three hours after ESD, his pain aggravated, and a follow-up chest radiography showed free air (). Emergency upper endoscopy revealed a 1 cm-sized gastric wall defect in the ESD-induced ulcer base (), and the perforation site was then closed with three clips (). Because clipping seemed successful, the patient was conservatively managed with antibiotics, a proton pump inhibitor, and parenteral nutrition. With this treatment, his abdominal pain improved, and defecation occurred 3 and 4 days after clipping. Five days after clipping, the abdominal pain again exacerbated. Abdominal CT showed a large pneumoperitoneum complicated with ascites, suggestive of panperitonitis, and the patient agreed to undergo emergency explorative laparotomy. However, the laparoscopic approach was not feasible in this patient due to massive complicated fluids and inflammatory tissues in the peritoneal space. During open surgery, a 3 cm-sized laceration was found at the previous ESD site, and primary suturing of the laceration was performed without gastrectomy (). The patient was discharged 14 days after surgery without any further complication. |
A 78-year-old man referred to our hospital had a history of hypertension, Type 2 diabetes, hyperuricemia, and chronic subdural hematoma. The patient visited a former physician due to transient ischemic attack causing paralysis on the left side. Cervical magnetic resonance angiography revealed severe stenosis of the right internal carotid artery. The patient did not experience impaired consciousness or neurologic deficits, and the coagulation/fibrinolytic testing results were not remarkable. A carotid echography revealed that the peak systolic velocity of the right internal carotid artery was 276 cm/s. The plaque was mobile with low echogenicity.\nA cervical three-dimensional computed tomography angiography examination showed stenosis of the right carotid artery from C4/5 to C5/6. The stenosis rate was 71% based on the method used in the North American Symptomatic CEA Trial [].\nCervical magnetic resonance imaging (MRI) revealed an unstable plaque with a major axis of 20 mm showing T1 (black blood) high intensity from the right common carotid artery to the proximal internal carotid artery [].\nThe patient had severe symptomatic stenosis of the right internal carotid artery, which was an indication for revascularization. We planned to perform CEA because the plaque was vulnerable, and the patient did not present with high-risk factors for the procedure. Clopidogrel 75 mg/day was initiated at the time of onset but was discontinued 5 days before surgery.\nWe performed CEA in a hybrid operating room. The Furui’s double-balloon shunt system (Muranaka Medical Instruments, Osaka, Japan) was used, and balloons in both the common carotid artery side and the internal carotid artery side were injected with 0.5 mL of air. Then, we removed the plaque and sutured the carotid artery. An intraoperative digital subtraction angiography (DSA) revealed extravascular pooling of contrast media in the distal carotid artery of the cervical region, indicating vessel injury. The internal carotid artery, distal to the injury site, presented with severe stenosis due to hematoma compression []. Since the injury site was distal to the operative field, vessel repair through direct surgery was challenging to perform. Hemostasis was achieved in the operative field. Therefore, we urgently performed CAS to repair the injured vessel and to facilitate dilation of iatrogenic internal carotid artery stenosis. We explained to the family that CAS for hemorrhagic lesions is an off-label treatment, and they provided informed consent for the procedure. Aspirin 200 mg and clopidogrel 300 mg were administered through the nasogastric tube before the procedure.\nSince we performed CEA in the hybrid operating room, immediately after the end of CEA, we also performed DSA with the transfemoral technique on the same bed. A right common carotid angiography revealed extravasation of the right internal carotid artery at the injury site, and contrast medium pooled in the extravascular space. The internal carotid artery, distal to the injury site, had severe stenosis and blood flow delay in the right middle cerebral artery. The right anterior cerebral artery could not be visualized [].\nThe Carotid WALLSTENT (Boston Scientific, Natick, MA, USA) measuring 8 × 29 mm was placed to cover the distal stenosis and the injury site. Although stenosis improved, blood flow delay from the injury site to the hematoma cavity remained. Hence, the Carotid WALLSTENT, measuring 10 × 31 mm, was placed and overlapped. Then, leakage of contrast medium from the injury site decreased []. We did not superimpose the third stent considering the high risk of thrombosis. On the right common carotid angiography after CAS, blood flow delay in the right middle cerebral artery improved, and the right anterior cerebral artery could be visualized [].\nPostoperatively, the patient continually received conservative management, including strict blood pressure control, under general anesthesia with intubation. Then, aspirin 100 mg and clopidogrel 75 mg were continuously administered. On DSA, extravascular pooling was further reduced at the end of CAS on postoperative day (POD) 1 []. Cervical computed tomography scan images were obtained on POD 2 (without contrast), 3 (with contrast), and 6 (without contrast). However, there was no increased blood mass [-].\nThe patient was extubated on POD 6. He presented with hoarseness and right miosis due to Horner’s syndrome, that is, lower cranial nerve symptoms. However, both spontaneously disappeared overtime. Therefore, hematoma decompression was considered effective. A head MRI on POD 13 revealed no new cerebral infarction or intracranial hemorrhage. On DSA, the extravascular pooling of contrast media in the injury site disappeared on POD 14 []. He was discharged to home without sequela on POD 21. |
A 64-year-old African-American male presented with an elevated PSA of 9.3 ng/mL and no previous history of prostate biopsy. He had a systematic TRUS-guided extended sextant biopsy with two of 12 cores demonstrating prostate cancer, one with GS 4+3 and a second with GS 3+4, both in the left apical region. He had no baseline urinary or bowel problems, but did have erectile dysfunction adequately managed with sildenafil taken as needed for sexual performance. His AUA urinary symptom score was 3 and SHIM score was 14 without use of PDE5 inhibitors. Using the web-based Memorial Sloan Kettering Cancer Center nomogram, his risk of nodal involvement was estimated to be 7% []. He was in good overall health and his age-adjusted life expectancy was estimated to be 19.4 additional years using the Social Security Administration life tables. After discussion of all treatment options with the multidisciplinary team, he elected to pursue definitive treatment with prostate SBRT.\nDiagnostic multi-parametric prostate MRI and review by the multidisciplinary prostate imaging conference previously demonstrated a 1.7 cm T2-weighted hypointense lesion with corresponding restricted diffusion in the anterior apical transition zone left of midline that was considered high suspicion based on imaging parameters but associated well to the systematic biopsy cores positive for prostate cancer (Figure ). The whole prostate volume and the area of MRI cancer suspicion were segmented using post image processing software. Following the MRI, three gold fiducial markers were placed in the urology office via a TRUS-guided approach. The radiation therapy planning CT scan was scheduled two weeks after fiducial placement. No urinary catheter, rectal balloon, or rectal spacer was used.\nAfter the simulation CT scan was completed, axial T2-weighted and post contrast T1-weighted MRI were fused with the CT images using radiation therapy planning software. Target volumes of the prostate and the high-risk lesion were then generated on the CT based on MRI segmented regions of suspicion. The radiation oncologist and urologist met to review the image fusion and target volumes prior to proceeding with final treatment planning. In this case, the highest suspicion lesion was located in the left anterior apical prostate as visible on T2-weighted MRI. Hence the prostate surrounding the high-risk lesion was included in the high-risk target volume for planned SIB (Figure ). For transition zone and central gland lesions, limiting dose to the urethra presents the greatest challenge to radiation therapy planning. However, most prostate SBRT protocols recommend achieving normal tissue dose limits over complete coverage of the high-risk target volume. In this case, adequate coverage was possible while limiting urethral dose to an acceptable range.\nThe high-risk volume received 40 Gy SIB in five fractions while the entire prostate received 36.25 Gy. He was placed on tamsulosin during radiation therapy after developing dysuria, diminished urinary stream, and frequency corresponding to an increased AUA symptom score of 14. These irritative urinary symptoms were his only radiation-associated side effects. He did not develop any significant lower gastrointestinal symptoms. Six months after his treatment, he continued to have irritative urinary symptoms and remained on tamsulosin. |
A 54-year-old female complained of a slowly growing mass in her thigh over a 34 year period. She has a two year history of hemodialysis due to chronic renal failure. A physical examination demonstrated a small melon sized mass on the anterior side of the distal thigh. The mass was not tender but rubbery hard and mobile.\nThe plain radiographs revealed a huge soft tissue mass with irregular calcification in the distal thigh. On MRI, the tumor was 17 × 11 × 9 cm in size located under the quadriceps muscle. The tumor matrix was partially calcified and relatively well defined with irregular low signal intensity in the T1 weighted image and heterogeneous low and high signal in the T2 weighted image. There was no abnormal signal of the underlying bony cortex and bone marrow ().\nGrossly, the excised tumor was a well circumscribed, lobulated round and firm mass. The cut surface was yellowish-white and fibrous to myxoid without any areas of hemorrhage or necrosis.\nThe optical microscopy examination demonstrated a mass with sharp demarcation, a nodular growth pattern and intervening hypocellular collagenous stroma (). The tumor showed a biphasic pattern with fibrous and myxoid areas. The cells were small, bland, regular, fibroblastic spindle cells with minimal nuclear pleomorphism, low to moderate cellularity, and swirling, whorled growth (). The background matrix ranged from fibromyxoid to densely fibrous ().\nImmunohistochemically, the tumor cells were diffusely and strongly positive for vimentin, but were negative for cytokeratin, smooth muscle actin, S-100 protein and neuron specific enolase. The tumor was identical immunohistochemically to a low grade fibromyxoid sarcoma with strong positivity to vimentin ().\nFive years after the excision, the patient is alive with no evidence of local recurrence or distant metastasis. The ranges of knee and hip joint motion were normal and the strength of the quadriceps muscle was good (). |
A 16-year-old male child was referred to us who had progressed from low grade astrocytoma to gliosarcoma over a long period of 15 years. The child was diagnosed as a case of pilocytic astrocytoma at 6 months of age (in September 2000). The presenting signs and symptoms were progressive increase in head size for 3 weeks with tense and bulging fontanelle, refusal to take feed for 2 days and loss of consciousness for 1 day. CECT scan of head revealed a large cystic space occupying lesion of size 13.7 × 6.7 cm replacing most of the left cerebral hemisphere with an enhancing soft tissue component in its wall suggestive of pilocytic astrocytoma. The infant underwent tapping of tumor cyst followed by left parietal craniotomy and excision of tumor. The postoperative histopathology report showed features suggestive of pilocytic astrocytoma. The child was not planned for any adjuvant radiation and was kept on regular follow-up.\nPatient was referred to our center when he developed presenting complaints of headache on and off with multiple episodes of vomiting and disturbance of gait for 1 month at the age of 16 years. On examination, his Glasgow coma score (GCS) was E4M5V6 (15/15) and power was grade 4/5 in right side upper and lower limbs and 5/5 with normal tone and reflexes on the left side. On further investigation, contrast-enhanced magnetic resonance imaging (CEMRI) of brain showed a well-defined extra-axial collection in left fronto-temporo-parieto-occipital region with nodular thickening of the meninges on the medial aspect of collection displaying intense enhancement on post-contrast images. A patchy ill-defined non-enhancing edema was noted in left temporo-parietal region which was further continuous in the posterior fossa extending into left CP angle compressing and displacing the midbrain and pons towards right. The significant mass effect on the left lateral ventricle with midline shift towards right was seen. CECT scan of head was also done which revealed ill-defined multilobulated, cystic solid lesion measuring approximately 10 × 6 × 6.5 cm, with significant enhancement on post-contrast study, involving left occipito-parietal region with soft tissue contents of lesion being hyperdense and cystic contents hypodense. A large hypodense concavo-convex collection was also seen in left fronto-parieto-occipito-temporal region causing significant mass effect in form of displacement of midline structures towards right side by 21 mm causing compression of ventricular system resulting in hydrocephalus without any evidence of hemorrhage. Atrophy of left cerebral hemisphere was seen. Thus, the features on CECT suggested pilocytic astrocytoma with cyst rupture resulting in left subdural collection with obstructive hydrocephalus.\nThe patient underwent left fronto-temporo-parietal craniotomy with cyst decompression and excision of the tumor. Postoperative histopathology report showed features of high grade malignant tumor with variable morphology in the lateral and medial aspects. On the lateral aspect, the tumor cells showed mainly papillary and perivascular arrangement with cells being round to ovoid in shape with moderate amount of eosinophilic cytoplasm and markedly pleomorphic round nuclei. Brisk mitotic activity and necrosis was also noted. On IHC, the tumor cells showed diffuse positivity with GFAP and were negative for epithelial membrane antigen. On the medial aspect, the tumor cells were predominantly oval to spindle in shape, arranged in interlacing fascicles and bundles, showing marked nuclear atypia and brisk mitoses. Tumor giant cells were interspersed. Occasional papillary configuration was also noted. On IHC, the tumor cells in sarcomatous areas showed diffuse positivity for vimentin, while the glial areas are highlighted by GFAP. CSF cytology showed no presence of malignant cells. Chest X-ray and ultrasound of abdomen were done to assess for any distant metastases, which were reported as normal studies. The child received adjuvant concurrent chemoradiotherapy followed by adjuvant chemotherapy and is being followed up since 4 months. |
A 69-year-old Caucasian male presented to his outpatient gastroenterology office with complaints of copious rectal discharge and for discussion of treatment options. His medical history was significant for trauma leading to a T7 spinal cord injury resulting in a thoracic arteriovenous malformation requiring surgical intervention. His postoperative course was complicated by paraplegia leading to him being constricted to a wheelchair. Twenty-five years after his initial surgery, the patient developed a left ischial ulcer and a sacral decubitus ulcer with concomitant methicillin-sensitive Staphylococcus aureus (MSSA) bacteremia, which was treated surgically with multiple debridements and prolonged courses of broad-spectrum antibiotics. During this time, the patient also underwent sigmoid end-colostomy placement due to chronic constipation. After another three years without complications, he presented with a new ischial wound and fevers, with wound cultures positive for MSSA, Streptococcus agalactiae, and Morganella morganii. He was treated with incision and drainage along with another eight-week course of ceftriaxone and metronidazole. In the following months, he began to have new onset of copious, purulent rectal discharge, leading to poor healing and further debridement procedures for his wounds.\nWhen inquiring further, the patient previously reported minor mucus drainage from the anus, occurring about once every three weeks. However, in the months leading to his presentation, his purulent fluid drainage increased in both volume and odor, with up to estimated 1 L daily of purulent rectal discharge. He was referred to a colorectal surgeon due to above-mentioned complaints. On presentation, his physical examination was notable for paraplegia, flexion contracture of bilateral knees, and a well-pouched colostomy in the left lower quadrant of the abdomen. No parastomal hernia was noted. His rectal examination was notable for a normal-appearing anus with some minor residual hemorrhoidal skin tags and obvious atrophy of both buttocks. Digital rectal exam (DRE) demonstrated very low rectal tone without sensation and a large amount of thin, yellow purulent drainage from the rectum. A right ischial ulcer was present, measuring 5 cm in diameter, tracking medially and superiorly with easily palpated underlying bone.\nAn anoscopy was performed revealing a large amount of purulence from the rectal wall in the right posterior location. Due to this, there was a concern for a rectal abscess with fistula and a magnetic resonance imaging (MRI) of the pelvis without contrast was obtained which revealed a soft tissue ulceration in the right ischial region extending from the skin to the ischial tuberosity and no evidence of focal fluid collection to suggest drainable abscess or other fluid collection (Figures , ). The patient underwent further evaluation with an anorectal examination under anesthesia and a flexible sigmoidoscopy (FS). DRE produced a large amount of purulent drainage, FS confirmed the same purulent fluid to the top of the rectal stump, and the fluid was sent for gram stain and culture. No connection to the sigmoid colon was found, thus confirming that the colostomy was in fact an end colostomy. The rectal mucosa appeared atrophied and with some diversion proctitis, but no evidence of granulation tissue or pseudomembranes, and no clear source for the purulence was discovered. The healed abscess cavity of the right ischial decubitus ulcer was aspirated and did not show evidence of purulent fluid. After a thorough examination as noted above and an X-ray barium enema, it was determined that there were no clear fistulous connections/fistula, and the working diagnosis included infectious versus inflammatory proctitis. His fluid drainage was positive for extended-spectrum beta lactamase (ESBL) Citrobacter freundii and Proteus mirabilis requiring a four-week course of ertapenem.\nAt this point, it was suspected that intestinal dysbiosis may be contributing to the patient’s current symptoms and a decision was made to treat him with autologous FMT. This involved daily enemas with a mixture of approximately 50 cc of feces from his ostomy with 50 cc of distilled water and retained for 20 minutes or as long as possible. The patient noted immediate improvement within two days when the rectal discharge had stopped completely. After approximately two weeks, patient began to have reoccurrence of his rectal discharge and the results of the enemas proved not to be permanent. The patient and his colorectal surgeon ultimately decided to move forward with a rectal mucosectomy, removing the mucosa of the rectum. The patient had slow wound healing following this procedure but decreased rectal discharge. |
A four-year-old boy presented with complaints of recurrent epistaxis and a bony white mass in the right nostril, in November 2020. The child’s mother initially noticed a small intranasal mass in March 2020, which she said has gradually increased to the current noticeable size. She did not visit a hospital earlier because of the coronavirus disease-19 (COVID-19) pandemic. However, the epistaxis episodes became frequent in the last few days. The child’s mother gave a history of loss of a maxillary central incisor following a dental trauma in November 2019, due to a fall. The mother was not able to find the tooth and was considered lost. A few weeks later, she felt a small and hard bump beneath the right nasolabial skin, which gradually kept drifting nasally, until she noticed a white mass in the right nasal cavity. On endoscopic nasal examination, a bony-hard, white mass was seen erupting from the floor of the right nostril and medial to the anterior edge of the inferior turbinate. A dome of granulation tissue was seen surrounding the base of the mass (Figure ).\nOral examination showed a missing right upper central incisor tooth (Figure ).\nThe CT scan showed a radio-opaque mass located in the floor of the right nasal cavity between the inferior turbinate and the nasal septum. The mass was surrounded posteriorly by soft tissue, consistent with the clinical finding of granulation tissue. With the bone window setting of a 500-HU window length (WL) and 1500-HU window width (WW), the lesion was noted to have a homogeneous high attenuation equivalent to that of the teeth and its root was buried in the mucosa of the nasal floor but was not surrounded by any bony socket. A slit-like cavity in sagittal cut and a focal central radiolucency in coronal cut, both correlating to the pulp cavity was seen (Figure ). These findings confirmed a diagnosis of the nasal tooth.\nThe patient underwent endoscopic removal of the nasal tooth. Gross examination of the excised tooth showed a root/crown ratio of ∼2, and the mesiodistal diameter of the crown exceeded its cervico-incisal length (Figure ). Both these findings, along with an absence of upper central incisor on clinical examination, confirmed the nasal tooth which is consistent with a deciduous maxillary central incisor. |
A 51-year-old Caucasian man of Spanish nationality was admitted to our department for diarrhoea. The symptoms began 11 years ago and were intermittent and mostly nocturnal. He worsened 3 months before admission with exacerbation of number of deposition, abdominal pain and weight loss. He was on PPI therapy on demand and at high doses for pyrosis. He consumed no toxics. His medical history was irrelevant and his physical examination was unremarkable except for the presence of a mass in his left upper quadrant of abdomen. No palpable lymph nodes and no splenomegaly were found.\nA complete blood count revealed mild leukocytosis (leukocytes 16.000, neutrophils 83%). Coagulation study and biochemistry tests including liver, kidney and thyroid gland function, iron metabolism and tumour markers presented no remarkable alterations. The reactive C protein was of 42.7 mg/dl (normal: 0-5 mg/dl). Gastrin level was of 114 ng/ml (normal: 13-115 ng/ml). Secretin induced test at 15 min was of 322 ng/ml. VIP and 5-HIAA levels were normal. The stool exam was negative for bacterial and parasites.\nAbdominal ultrasound revealed a liver mass in the 2nd and 3rd segment of a 10 x 8 cm diameter and small bowel distension. A helical computed tomography scan and a magnetic resonance imaging confirmed the liver mass with contrast uptake (). The fine-needle aspiration of the liver mass was informed as metastasis of an adenocarcinoma.\nUpper endoscopy was performed and showed grade B (Los Angeles classification) esophagitis and gastritis as well as hypertrophy of the gastric folds and multiple duodenum ulcers with unspecific biopsy and H. Pylori negative. Barium intestinal study showed the same findings, without any small bowel lesions.\nThe gammagraphy with 111 In-DTPA octreotide showed pathologic uptake in the upper abdomen, especially at the jejunum wall, with high expression of serotonin receptors. A PET study was performed which showed high abnormal uptake in the liver mass as well as a diffuse uptake into the axils, small bowel wall and spleen. The study was completed by endoscopic capsule examination which did not reveal alterations at that level and with a complete colonoscopy without relevant findings. The echocardiography was normal.\nA left hepatectomy was performed and the pathologic diagnosis pointed out to a primary neuroendocrine tumour (carcinoid tumour) with poor mitotic activity and important necrosis. Inmunohistochemistry, on paraffin-embedded formalin fixed sections of the tumour, showed positive staining for synaptophysin, chromogranine, enolase, CK7 () and negative staining for CEA (). A moderate staining was detected for gastrin ().\nDuring the follow up, the patient referred total disappearance of the initial clinical symptoms. Upper endoscopy revealed no duodenum ulcers and his serum gastrin levels decreased till 19 pg/ml two months after surgery.\nThe patient remained free of symptoms two years after surgery. |
A 12-year-old boy presented to the outpatient department of our hospital presenting with complaint of swelling in the right shoulder region which was insidious in onset with gradual increase in size and deep aching pain since past 6 months. Patient had no history of trauma, any constitutional or systemic complaints. On local examination, there was diffuse swelling involving the right shoulder region with slight increase in local temperature and tenderness of proximal humerus. The movement around the shoulder was painful and there was no associated neurovascular deficit in the upper limb. Patient was subjected to radiograph of the proximal shoulder. All the hematological and routine biochemical parameters were normal except for mild increase in erythrocyte sedimentation rate (ESR-32 mm/1st h). Plain radiograph revealed a large expansile osteolytic lesion with characteristic blown out “soap bubble appearance” (, ) involving the proximal humerus and abutting the growth plate.\nRadiologically it was classified as Enneking Stage 3 [] cyst which is locally aggressive and expanding with significant cortical destruction and Capanna type 2 [] lesion involving the entire bony segment (proximal metaphyseodiaphyseal region) with marked expansion and cortical thinning. Usually, clinical and radiologic features may be sufficient for a presumptive diagnosis of ABC, but establishing the histological diagnosis is essential before committing to definitive treatment. Hence, percutaneous needle biopsy of the lesion under fluoroscopic guidance was undertaken. The histology was likened to a “blood-filled sponge” composed of blood-filled anastomosing cystic cavernomatous spaces separated by wall composed of fibroblasts, myofibroblasts, and osteoclast such as giant cells, osteoid, and woven bone [, , ] confirmed the diagnosis of ABC (, , ). Due to the unusually large bony involvement, osteolytic and expansile characteristic of the lesion intervention is preferred over mere observation. Although resection/excision or curettage with bone grafting is commonly undertaken, concerns were for issues of subsequent bony reconstruction given the size of defect with possibility of need of an implant for stabilization, likelihood of damage to growth plate and functional compromise of the shoulder. Hence, a decision to treat the patient with liquid absolute alcohol based sclerotherapy was planned. This being a harmless and easily available material in most hospital settings, while other agents which have been tried for sclerotherapy may not be easily available. Sclerotherapy was done under general anesthesia with fluoroscopic guidance to ensure correct positioning of 18 G needle into the lesion. We proceeded to injecting 96% ethanol in the dosage of 1 ml/kg. Higher doses of alcohol in children can lead to alcohol poisoning with severe symptoms []. Moreover, precaution was taken to stop drug administration when increased resistance to injection was observed to prevent extravasation into especially draining veins and surrounding soft tissues and other drug related complications. After 10 min, the alcohol was subsequently flushed with saline. Following the procedure, the patient was given nonsteroidal anti-inflammatory drugs (Tab. Ibuprofen 400 mg twice a day) for 3 days with a 5 days course of broad spectrum antibiotics (Tab. Cefuroxime 250 mg twice a day), to minimize pain and local inflammatory reaction that may follow the injection. Patient was hospitalized for 48 h especially to monitor injection associated complications such as local inflammatory reaction, severe pain, and dizziness after injection. The radiological follow-up was performed after 3 months and based on the radiological response by observing reossification of the lesion, the therapy was repeated at 3 and 6 months follow-up. Patient showed almost complete reconstitution of lesion and bone remodeling with good shoulder function and intact growth plate (, ). In search of literature, we have not found such a large sized aggressive ABC of proximal humerus at an early age of 12 years, managed successfully with a commonly available sclerotherapy agent, that is, absolute alcohol []. |
Patient 2 is a 31-year-old British woman with a diagnosis of TRS. She first presented to psychiatric services in 2002 (age 17) with flattened affect, inappropriate laughter, social withdrawal and functional decline. She was diagnosed with schizophrenia and over the next 10 years she was treated with various antipsychotics up to maximal British National Formulary recommended doses, including risperidone up to 8 mg, aripiprazole up to 30 mg and amisulpiride up to 800 mg, to which she demonstrated a limited response.\nHer medical history at this time included poorly controlled type I diabetes mellitus. Her blood sugar fluctuated from hypoglycaemia to hyperglycaemia and she was repeatedly admitted to medical wards for diabetic ketoacidosis.\nBetween 2009 and 2012 she presented with persistent positive psychotic symptoms: responding to auditory hallucinations, prominent thought disorder and inability to manage basic aspects of her personal care. This led to a psychiatric admission in June 2015 and clozapine was commenced in July 2015. There was no significant improvement in her mental state on clozapine, and over the next 6 months various augmentation strategies were attempted without success. These included amisulpride up to 800 mg daily, aripiprazole up to 20 mg daily and lurasidone 74 mg daily – all provided no significant benefit.\nShe was admitted to the National Psychosis Unit in October 2016 having been an inpatient for 16 months with persistent psychotic symptoms. Psychotropic medications on admission included clozapine 350 mg and lurasidone 74 mg daily (she had been treated with this for over 6 months). She was also prescribed insulin and pioglitazone to manage her diabetes. On admission she scored 110 on the PANSS total score and was unable to attend sufficiently to complete the MMSE. MRI of the brain was normal. Blood results at admission were within normal limits except for a raised HbA1c 9.5% (4.1–6.0), random glucose 22.4 mmol/L (3.0–6.0), ALP 209 IU/L (30–130), cholesterol 5.4 mmol/L (1.0–5.0) and lipid ratio 4.5. Her clozapine level on admission was 0.30 mg/L.\nIn early November 2016 her clozapine dose was increased to 375 mg daily, with a subsequent serum level of 0.66 mg/L. Her diabetes remained extremely difficult to control and she had a medical admission for diabetic ketoacidosis. By mid-December 2016 the increased clozapine dose had conferred no additional clinical improvement. Given the difficulty controlling her diabetes and the lack of any clear benefit from clozapine treatment for over a year, clozapine was stopped and instead lurasidone was increased to 111 mg. At the end of December 2016 vortioxetine 10 mg was commenced as a trial adjunct to enhance her cognition and attention. Within a few weeks of initiating vortioxetine, there was a remarkable improvement in all domains. She was more engaging, appeared brighter in mood, less distressed by psychotic symptoms and was able to attend to her self-care and acknowledge the change since admission, saying that her thinking felt clear and that voices were bothering her less. By January 2017 she scored 60 on the PANSS total score and 27/30 on MMSE. By February 2017 her diabetic control had improved: pioglitazone had been stopped, she required lower doses of insulin and her HbA1c had come down from 9.5% to 7.9%. By April 2017, she had maintained her improvement on lurasidone 111mg and vortioxetine 10 mg and was awaiting discharge. |
A one-year-old Omani female child presented to the emergency room with fever and severe respiratory distress. She was the sixth child of a second-degree consanguineous marriage and was found to have dysmorphic features at birth. Chromosomal analysis showed trisomy 21. Her cardiac echocardiogram was normal. Routine thyroid screens were normal. The baby had no respiratory problems in the initial newborn period. There was history of feeding difficulty since birth in the form of wet cough after feeding and the child had recurrent vomiting of unaltered food. The problems with feeding were accentuated when the semisolid feeds were commenced and she had multiple hospital admissions for vomiting and was documented to have had aspiration pneumonia on each of these admissions. She was treated symptomatically and discharged. She was presumed to have gastroesophageal reflux and no further evaluation was performed. She has been immunized to date and has global developmental delay with severe failure to thrive.\nOn examination, she was in severe respiratory distress with supra-sternal, intercostal, and subcostal recessions. She had noisy breathing with copious secretions and drooling. She was anicteric, acyanotic, but had moderate pallor. There was no clubbing, edema, or generalized lymphadenopathy.\nThe child was tachypneic with a respiratory rate of 60 breaths/min. Oxygen saturation was 78% in room air and 98% with 3 L oxygen/min. She was tachycardic with a heart rate of 120 beats/min. She was febrile with a temperature of 39 °C. Her blood pressure was 92/69 mm Hg.\nThe child had a weight of 5 kg, head circumference of 42 cm and length of 68 cm. Her developmental evaluation showed a developmental age of six months as opposed to a chronological age of one year for gross motor, speech and fine motor functions. Her weight was well below the first percentile for her age. She had good personal social interaction with gestures.\nComplete physical examination showed typical morphological features of trisomy 21. Neurological examination showed decreased tone and normal deep tendon reflexes. All of her pulses were well felt. Heart sounds were normal. There were no murmurs and no features suggestive of cardiac failure. She had copious oral secretions and wet cough and was in respiratory distress. Breath sounds were equal bilaterally with coarse crepitations and rhonchi. Abdomen was soft and non-tender without organomegaly.\nThe child was treated with intravenous antibiotics and antiviral medications along with oxygen driven nebulisations and intravenous fluids. She was also given proton pump inhibitors intravenously. Her blood investigations showed raised C-reactive protein (CRP) with neutrophilic leukocytosis and the blood cultures were negative. The chest X-ray showed extensive bronchopneumonia (). A repeat cardiac echo and a thyroid screen were done which was normal. She improved symptomatically with therapy, but began having emesis again when oral feeds were resumed with cereals.\nA gastroenterology consultation was taken in view of the recurrent vomiting and aspiration pneumonia with failure to thrive. A barium swallow () was done, which showed an obstruction at the lower end of the esophagus with proximal dilatation and pooling of barium.\nShe was fed exclusively via an orogastric tube for a few days to prevent further episodes of aspiration and to help her lungs clear up in readiness for anesthesia. Her pneumonia improved and she was taken up for an endoscopic evaluation under general anesthesia. On endoscopy (), an esophageal web of thickness less than 5 mm with a pinpoint orifice was noted in the lower esophagus just above the gastroesophageal junction, and was dilated using a controlled radial expansion balloon from 8 to 12 mm. There was only mucosal and sub mucosal involvement with no involvement of the muscular portion of the esophagus. There were no features of esophagitis or endoscopic findings suggestive of achalasia.\nInitially, she was kept nil per oral and then fed liquids via the nasogastric tube, and then semisolids orally. Post procedure, she had some feeding issues with intermittent vomiting, but she improved and at the time of discharge, she was on full oral feeds with no vomiting or further signs of aspiration. She was discharged with a weight of 6.35 kg (an increase of 1.35 kg), length of 68 cm, and a head circumference of 42 cm. At eight months following the endoscopy, she continues to be well and is thriving. |
A 34-year-old woman with CD (gravida 1, para 1) who had been diagnosed with pregnancy-induced hypertension (PIH) and marginal placenta previa at 20 weeks of pregnancy was referred to our center because of fetal growth restriction (FGR). She exhibited intermittent genital bleeding after admission, and her pregnancy was interrupted due to massive bleeding at 26 weeks 6 days of gestation. The history of the mother such as PIH, thrombocytopenia, low antithrombin III, and FGR suggested a minor form of HELLP syndrome; however, her liver enzyme levels (aspartate aminotransferase [AST]: 34 IU/L, alanine aminotransferase [ALT]: 20 IU/L) did not meet the criteria. A female infant was born via emergent cesarean section (birth weight: 666 g [−1.7 SD]; Apgar scores of 3 and 5 at 1 and 5 minutes, respIectively).\nThe mother had developed CD at 25 years of age; however, she had not been diagnosed with vitamin K deficiency before pregnancy. The disease deteriorated during her first pregnancy (28 years of age) with the symptoms of intractable diarrhea and coagulopathy (prothrombin time: 57%) since 33 weeks of gestation. Based on the findings of reduced prothrombin time, she was diagnosed with vitamin K deficiency and received intravenous vitamin K administration on the day before delivery. Her first child, a male infant, was delivered at 33 weeks of gestation 6 days at regional hospital by emergent cesarean section and found to have third-grade IVH at birth; this proceeded to posthemorrhagic hydrocephalus. At 29 years of age, the mother underwent surgical repair of an anal fistula and required an elemental diet and parenteral nutrition with daily prednisolone (40 mg/d). At 31 years of age, infliximab and oral mesalazine were administered for treatment of an ileum-skin fistula. She achieved remission and required no steroid treatment thereafter. Her coagulation status at delivery was normal ().\nThe infant was tracheally intubated soon after birth, received an artificial pulmonary surfactant for respiratory distress syndrome, and was mechanically ventilated on synchronized intermittent mandatory ventilation mode. She was hypotensive on the neonatal intensive care unit (NICU) admission and required inotropes and volume expanders including albumin, packed red blood cells, fresh frozen plasma (FFP), and hydrocortisone via umbilical venous catheters to maintain her blood pressure. She also received a dose of indomethacin at 15 hours, and patent ductus arteriosus was closed at 36 hours after birth. Initial blood tests revealed leukocytopenia and massive coagulopathy (). Initial brain ultrasound () and echocardiography revealed no abnormalities. Her coagulopathy was recovered on day 2 () after intravenous administration of vitamin K2 (menatetrenone, 1 mg) at the admission to NICU and FFP. She required 2 additional doses of surfactant on days 2 and 4 of life for respiratory distress. Her cardiorespiratory condition was maintained under mechanical ventilation and inotrope management in the postacute phase; however, enteral nutrition could not be increased (<20 mL/kg/d) because of feeding intolerance. In addition, following brain ultrasound performed at 8, 27, 36, and 46 hours and 4 days after birth revealed no abnormality. On day 8 of life, pulmonary hemorrhage occurred unexpectedly, and ultrasound examination revealed grade 2 IVH ( and ) and relapse of the coagulopathy without signs of disseminated intravascular coagulation (). The coagulopathy gradually improved after multiple intravenous administrations of vitamin K2 (1 mg on days 8 and 9 and 2 mg on day 14), packed red blood cells (days 9 and 18), and FFP (days 8-12, 15, and 18); the IVH did not progress thereafter. She has never received fat-soluble vitamins with parenteral nutrition. Tests for lupus anticoagulant (day 10) and protein induced by vitamin K absence or antagonist II (PIVKA-II, day 14) were negative. She had no signs of infections throughout the course. The coagulopathy did not relapse, and the absorption of hemorrhage and the formation of subependymal cysts were confirmed by brain ultrasound on day 34 (). Brain magnetic resonance imaging performed on day 115 did not reveal any abnormal findings except right obsolete hemorrhage. She was discharged on day 121 of life. She recently turned 7 years of age with no episodes of bleeding tendency or developmental delay (deviation quotient assessed by Wechsler Preschool and Primary Scale of Intelligence at 6 years of age: 90). |
31-year-old male presented with dyspnoea on exertion NYHA class II, orthopnoea, and lower limb oedema for three weeks. These symptoms were preceded by flu-like symptoms. He was also noted to be hypertensive by his GP a week prior to admission and was started on Ramipril. He has no other significant past medical history apart from a high Body Mass Index of 38. He consumed about 40 units of alcohol per week and he has a positive family history of ischaemic heart disease (father and two grandfathers had myocardial infarction at the age of 50). Examination revealed a blood pressure of 160/70 mmHg, heart rate of 110 beats per minute, oxygen saturation of 97% on room air, raised jugular venous pressure, bibasal crepitation, lower limb oedema, and no cardiac murmurs. Radiofemoral delay was not checked during initial assessment but was detected following the result of the CT scan. Initial investigations included an electrocardiography which showed sinus tachycardia with left ventricular hypertrophy. High sensitivity troponin-T was elevated at 55 ng/L (upper limit 14 ng/L), with no rise on serial testing. Chest X-ray revealed marked cardiomegaly with a cardiothoracic ratio of 0.68 and pulmonary congestion but there was no rib notching. TTE confirmed a severely dilated left ventricle (left ventricular internal dimension-diastole of 6.9 cm) with severe global systolic dysfunction, moderate concentric left ventricular hypertrophy, bicuspid aortic valve without significant gradient, and an estimated ejection fraction of 25–30%. Two weeks following admission he complained of sudden onset severe left arm pain and CT left upper limb confirmed embolic occlusion of the left brachial artery at the level of the distal third of the humerus and incidental coarctation of the aorta at the isthmus (). The coarctation measures approximately 0.5–0.6 cm, compared with 2.0 cm in the distal arch and 2.7 cm in the proximal descending aorta. Follow-up TTE suggested the presence of coarctation of the aorta on a suprasternal view which was not performed at the time of his first TTE (). The patient was then treated with intravenous diuretics along with other usual heart failure measures. The patient's heart failure responded very well to treatment. He lost 20 kilograms with diuretics and his blood pressure was adequately controlled. He also had successful embolectomy of his left brachial artery and was put on intravenous heparin and then had oral anticoagulation with warfarin. He is on regular follow-up in the cardiology clinic and is awaiting surgical intervention for his aortic coarctation. |
A 14-month-old, 8-kg, routinely vaccinated male was transferred to a tertiary care pediatric medical center with concerns for respiratory distress. He had a 1-week history of fever, decreased neck mobility, and decreased oral intake. The patient was diagnosed with streptococcal pharyngitis 9 days prior, and he was being treated with a course of amoxicillin. Due to worsening symptoms, his parents brought him to the nearest hospital where a lateral neck film showed a large amount of retropharyngeal thickening and airway displacement (). His white blood cell count was elevated at 33,000/mm3. He was transported by helicopter to our institution for further management. Physical examination revealed an alert child with a muffled voice. Vitals sign consisted of a respiratory rate of 25 breaths/minute, heart rate of 161 beats/minute, and temperature of 37.3°C. He was maintaining oxygen saturations of 100% on room air. Oropharyngeal examination showed significant bulging of the posterior pharynx. Further imaging was delayed until the airway was secured.\nThe necessary airway equipment as well as surgical instruments for tracheotomy was set up prior to the patient entering the operating room. In an attempt to keep the child calm and to avoid any unnecessary manipulation of the airway, he was transported to the operating room in his mother’s arms. Anesthesia was induced by slow, controlled inhalation of sevoflurane in nitrous oxide and oxygen with the goal of maintaining spontaneous ventilation. After ensuring an adequate depth of anesthesia and properly positioning the patient, the pediatric otolaryngology team proceeded with direct laryngoscopy. The epiglottis could be visualized, but the posterior larynx was compressed anteriorly in such a way that the vocal cords could not even be seen (). Attempts at placing an endotracheal tube with a stylet as well as the rigid ventilating bronchoscope through the larynx were unsuccessful. Air exchange ceased, and bag-valve-mask ventilation was not possible. There was significant upper airway obstruction from mass effect alone (never a visible lumen), and this was not believed to be due to laryngospasm. The oxygen saturation began to decline rapidly, and the decision was made to proceed with emergent tracheotomy tube placement. A vertical skin incision followed by vertical tracheotomy was performed with a scalpel, and the airway was established within 15 seconds. Oxygen saturations quickly normalized, hemostasis was achieved, and the tracheostomy tube was secured. The posterior pharyngeal bulge was very tense, and an incision and drainage was performed with the drainage of approximately 100 mL of purulent fluid. Immediately following the drainage, a grade I view of the larynx was obtained (). The patient was transferred to the pediatric intensive care unit in stable condition. Postoperative computed tomography scan showed there was no mediastinal component that needed further intervention. Cultures of the abscess grew beta-hemolytic streptococcus group A. Serial bedside laryngoscopy was done to confirm that there was no re-accumulation of the abscess and improvement of pharyngeal edema before initiating tracheostomy decannulation process. The tracheostomy tube was removed on postoperative day 6, and 2 weeks later the stoma was fully healed. |
A 14-and-a-half-year-old boy from Fayoum Governorate in rural Egypt complained of an initially painless right foot swelling for the past six years. Multiple sinus tracts developed with an initial purulent discharge and an eventual extensive black granular discharge. He was previously subjected to multiple bony debridement procedures in other institutions to which the he did not respond. Based on the clinical/radiologic suspicion and results of culture and sensitivity from discharging sinuses that revealed Gram positive Staphylococcus aureus, the patient was diagnosed with nonspecific bacterial calcaneal osteomyelitis. The patient was treated with multiple courses of oral and intravenous antibiotics over the following six years, to which he did not respond. The patient walked with painful limp that deteriorated over the past years till he finally used crutches permanently and became a household ambulatory. The patient was nonimmunosuppressed and had no systemic manifestations or other skeletal complaints. Blood and serum chemistry were also unremarkable.\nBy examination, there was diffuse right foot oedema and tenderness especially over the hind foot. Two discharging sinuses were observed in relation to the hind foot. There was painful passive motion of the subtalar and ankle joints.\nX-ray findings of the foot and ankle revealed massive bony destruction of the calcaneus consistent with chronic osteomyelitis. MRI of the right ankle and foot was performed to characterize and evaluate the extent of the disease. Technique of examination included sagittal T1-, STIR, and T2-weighted images, axial T1- and T2-weighted images, and coronal T2-weighted images. The salient abnormality was the markedly altered marrow signals seen almost totally replacing the marrow texture of the calcaneus and to a lesser extent involving the opposing posteromedial aspect of the talus eliciting abnormal low T1, high T2, and iso-to-high STIR signals with evident cortical breaching and associated large soft tissue component eliciting low T1 and high T2 and STIR signals (Figures , , , and ).\nIn march 2015, we performed total calcanectomy through a heel splitting approach. Under general anesthesia, a high tourniquet was employed in the prone position. Anatomic pathology of the excised calcaneus is depicted in Figures , , , , and . The histopathological features were consistent with eumycetoma (). The patient was put on oral Itraconazole therapy for 8 weeks. Oral clindamycin therapy was used to treat secondary bacterial infection. Significant clinical improvement was observed at regular followup intervals. The discharging sinuses and the surgical wound demonstrated sound healing. |
A 57-year-old Japanese woman presented with epigastric tenderness. Gastroendoscopy showed a growing lesion (size, 16 mm × 11 mm) in the greater curvature of the upper gastric body, and the mucosa adjacent to the lesion showed atrophic gastritis. Histopathological examination of a biopsy sample revealed NEC. Helicobactor pylori were not detected by Giemsa staining. In addition, serum levels of chromogranin A and gastrin were not measured. The patient subsequently underwent distal gastrectomy. One year after surgery, liver metastasis was found in abdominal computed tomography examination, and chemotherapy was performed.\nIn low-magnification images, tumor cells were distributed in two lesions. One lesion was constructed by tumor cell proliferation within the submucosal layer, and another lesion was found in the muscle layer; these two lesions were discontinuous (Figure ).\nMicroscopic examination revealed that the tumor tissue was composed of three subtypes of tumor components as follows. The first component was composed of cuboidal epithelial cells with uniform, oval nuclei showing fine nuclear chromatin and arranged in a trabecular growth pattern with focal rosettes. Tumor cells had very few mitotic figures [< 2 per 10 high-powered fields (HPF)] and no necrosis. This component corresponded to the NET G2 component (Figure ). The second component consisted of diffuse proliferation of tumor cells with small nuclei showing irregular, dense nuclear chromatin and high mitotic counts, corresponding to the NET G3 component (Figure ). The third component was composed of diffuse proliferation of tumor cells with large, irregular, coarse nuclei, high mitotic counts, and geographic necrosis, similar to the large cell NEC component (Figure ). Although the transition between the NET G2 component and the NET G3 component was confirmed, the submucosal lesion and intramuscular lesion were separated by the muscle layer (Figure ). Marked lymph vessel and venous invasion were observed in the submucosal layer, as determined by D2-40 immunohistochemical staining and EVG staining (Figure , inset).\nIn addition, the mucosa adjacent to the tumor showed atrophic gastritis with intestinal metaplasia, but not type A gastritis. No endocrine cell micronests were found in the surrounding mucosa.\nImmunohistochemical examination was performed using an auto-immunostaining system (Dako EnVision System, Denmark) for neuroendocrine differentiation, cell proliferation activity, p53 overexpression, and mucin phenotype (Muc2, Muc5AC, Muc6, CD10). The positive rate of Ki-67 was calculated using an APERIO virtual slide system (AT2; Leica Biosystems, United States).\nAll tumor components were immunopositive for chromogranin A, synaptophysin, and CD56, and decreased immunoreactivity was found in the NEC component. Differences in Ki-67-positive rates were found among NET G2, NET G3, and NEC components (NET G2, 6.5%; NET G3, 99.5%; NEC, 88.1%). Overexpression of p53 protein was not found in any component. With regard to the mucin phenotype, no mucin markers were expressed in any tumor component (Table ).\nDNA from NET G2, NET G3, and NEC components was extracted separately. PCR-allelic imbalance (AI) analyses were performed using a thermal cycler (GeneAmp PCR System 9600; Perkin-Elmer, CA, United States) according to previously reported procedures[]. AIs on chromosomes 1p, 3p, 4p, 5q, 8p, 9p, 13q, 17p, 18p, and 22q were examined using 22 highly pleomorphic microsatellite markers (D1S228, D1S548, D3S2402, D3S1234, D4S2639, D4S1601, D5S107, D5S346, D5S299, D5S82, D8S201, D8S513, D8S532, D9S171, D9S1118, D13S162, TP53, D18S487, D18S34, D22S274, D22S1140, and D22S1168).\nIn addition, PCR-MSI analysis was performed as described previously[]. Five different loci were assessed for MSI, including all those recommended by the Bethesda panel for colon cancer (BAT25, BAT26, D5S346, D2S123, and D17S250)[].\nDNA methylation at the six specific promoters originally described by Yagi and colleagues was quantified[]. Methylation of three markers (RUNX3, MINT31, and LOX) was analyzed, and samples with at least two methylated markers were defined as highly methylated epigenotype (HME) tumors. The remaining tumors were also screened for methylation at three other markers (NEUROG1, ELMO1, and THBD) and were defined as intermediate methylation epigenotype (IME) tumors if they had at least two methylated markers out of the three markers proposed as a second panel. Tumors not classified as HME or IME were designated as low methylation epigenotype (LME).\nIn the present case, DNA methylation status was LME, and MSI was not found in all tumor components. AIs at 1p, 3p, 8q, TP53, 18q and 22q were commonly found in all tumor components. Although AI of 4p was found in NET G3 and NEC components, it was not observed in the NET G2 component. In addition, AIs at 5q and 9p were found in the NEC component only (negative for NET G2 and G3 components; Figure ). |
A 79-year-old man proceeded in the emergency department with skin cut 2 x 1.5 cm in the dorsal side of the right hand from a sharp wooden segment during logging. After surgical lavage and exploration for soft-tissue damages or subcutaneous foreign bodies (under local anesthesia), it was found that both the extensor digitorum tendons and the neurovascular structures of the dorsal hand were intact. No foreign body was identified. Thus, a skin closure with polypropylene non-absorbable sutures was performed. Antitetanic serum injection at once and antibiotics (cefuroxime 500 mg) for 7 days was given to the patient.\nTwo weeks postoperatively, following sutures removal, there was no sign of local pain, skin irritation, edema, or inflammation. However, 2 months post-traumatically, the patient returned to the hospital with mild pain and local swelling over the traumatized area. No neurovascular or functional deficits were diagnosed. Laboratory tests did not reveal any sign of infection, while the X-ray control was negative for bone lesions or the existence of foreign bodies. The patient was treated with non-steroidal anti-inflammatory drugs for 5 days. One week later, the patient was almost free of symptoms.\nTen months after the initial injury, the patient visited again the orthopedic department displaying limitation in the range of motion (RoM) of the right ring finger and persistent deteriorating pain, both in extension and flexion. The clinical examination demonstrated a palpable mass at the route of the common extensor tendon of the fourth finger. An ultrasonographic (U/S) evaluation revealed the presence of a well-defined hypoechoic cystic mass within the tendon, surrounding an echogenic lesion 0.6 x 0.2 cm, presumably intratendinous foreign body.\nA surgical investigation was executed, initially with a longitudinal skin incision just above the U/S finding. No sign of any tendon inflammation was observed. Taking care not to critically damage the tendon, a longitudinal incision on the dorsal surface of the tendon was carried out. An intratendinous cystic lesion was revealed (, ).\nThe thickness of the cyst wall was measured around 0.2 cm. Afterward, a cyst incision revealed the existence of a small wooden piece, which was removed along with the cystic layer ().\nThe dimensions of the foreign body were identical with those of the pre-operative sonographic measurement. As the last step, the tendon was carefully repaired, and trauma was closed in layers. The post-operative histopathological examination illustrated the presence of a cyst filled with keratinous material and lined with stratified non-keratinized squamous epithelium diagnosed as an epidermoid cyst.\nThe accelerated rehabilitation protocol included passive RoM of the involved finger for 10 days followed by progressive active mobilization for another 2 weeks. Three weeks postoperatively, the patient was free of symptoms without any functional deficits, managing to fully restore the normal RoM, and return to proinjury daily activities. |
A 55-year-old male had continuously visited the Department of Oral and Maxillofacial Surgery at Yonsei University College of Dentistry for MRONJ which had primarily developed after zoledronic acid and capecitabine administration for previous bone metastasis of medullary thyroid cancer. Pain, bone exposure, and pus discharge in the right mandible and left maxilla first occurred 5 years after discontinuing bisphosphonate and capecitabine administration. At that time, the patient was going through chemotherapy with another antineoplastic drug, everolimus. Clinical, radiological features of both jaws, and pathological evaluation of the mandible confirmed the diagnosis as MRONJ.\nSymptoms of the left maxilla started as chronic apical abscess with tooth mobility in the left premolars. Primary bone exposure with osteonecrosis at the left maxilla was observed after extraction of these teeth in a local clinic. The remaining sockets showed delayed bone healing with pus discharge and incomplete soft tissue covering.\nRadiologic examination by CT () revealed that there was an irregularly mixed radiolucent/radiopaque area in the left maxilla with discontinuity of the cortical barrier adjacent to the left buccal vestibule, palate, nasal cavity, and maxillary sinus. Mucosal thickening was seen in the left maxillary sinus and nasal cavity. These radiologic features persisted in the additional CT views taken 2 years later (data not shown).\nAfter a year of conservative treatment and another year and a half without periodic follow-up, the patient re-visited the hospital for recurrent bone exposure in the left maxilla with pus discharge. An ill-defined radiolucent/radiopaque lesion with definite alveolar bone loss was seen at the left maxilla on panoramic views (). The exposed sequestra were removed and sent for pathological evaluation.\nOn pathological examinations, the tissue specimen revealed mostly several necrotic bone fragments with surrounding nonspecific bacterial colonies and necrotic soft tissue. The bone fragments had mature calcified matrix and irregular margins without any vital osteoblastic or osteoclastic rimming. The lacunae were empty of osteocytes, indicating bone necrosis. Pathological features were consistent with MRONJ, and there was no evidence of metastatic medullary thyroid cancer. Interestingly, between the bone fragments, there was a worm-shaped organism with several false annuli (pseudosegmentation) along the body and features of a hook or claw-like structure at the broader cephalic-end (). Focal areas of necrosis were noticed within the parasite. Due to these features, the organism was diagnosed as a pentastome by further evaluation at the Department of Internal Medicine and the Department of Environmental Medical Biology.\nBy further oral history taking, the patient disclosed that he had been occupied for about 10 years in foreign trade business since his thirties, with local food exposure in the Middle East, Southeast Asia, and Africa. Short subsequent visits every year continued in several countries. There were no specific symptoms at that time. The patient did not have any specific signs or symptoms of other visceral pentastomiasis. The patient had performed periodic whole blood laboratory tests for thyroid cancer treatment, and the results were evaluated. Right before parasite discovery at the Department of Oral and Maxillofacial Surgery, the laboratory results showed eosinophilia (eosinophil count: 507.6/μl). Laboratory antibody screening for other types of parasite infections revealed positive for toxocariasis IgG, and parasiticidal treatments for both pentastomiasis and toxocariasis (albendazole 800 mg/day ×5 days) were prescribed []. Since then, periodic follow-up along with supportive care for thyroid cancer is being done. |
A 60-year-old female presented with a 2 month history of headache and dizziness. Her past medical history was unremarkable. Her family history was not significant and she had no stigmata of VHL disease. Pituitary function was normal. There was no neurologic deficit.\nBrain magnetic resonance imaging (MRI) demonstrated an isointense suprasellar mass on the T1-weighted images. On the T2-weighted images, the mass was heterogenous in signal intensity. The mass exhibited marked homogenous contrast enhancement with intravenous administration of gadolinium and it appeared that the tumor was originated from the pituitary stalk. The mass was measured to be 10 to 12 mm with multiple signal voids inside, and no other intracranial lesion was identified ().\nOperative resection of the mass in the suprasellar region was performed with a right frontotemporal craniotomy. In the surgical fields, an obviously hyperemic and friable round mass with a thin capsule located mainly in the ventrocaudal portion of the pituitary stalk was identified. It was adherent to the paper thin leafs of the pituitary stalk. Adhesion to the diaphragma sellae was not observed. During the operative resection, brisk bleeding due to the vascularity of the lesion was controlled with a bipolar coagulator and hemoclip. The mass that contained the lump of blood vessels was gross totally extirpated ().\nHistological findings showed a vascularized mass composed of numerous thin-walled vascular channels lined endothelial cells and numerous ovoid, vacuolated stromal cells in a hematoxyline and eosin stain. This is consistent with the typical well-known morphology of HBLs. Mitotic figures were rare. Immunohistochemical staining was performed with antibody for CD 34. Endothelial cells showed a positive reaction for CD 34 ().\nPostoperatively, the patient experienced considerable improvement of her symptoms and the postoperative period was uneventful except for occurrence of transient diabetes insipidus which was successfully managed with fluid control and hormonal replacement therapy. During the follow up period, a manifestation of the VHL complex was investigated and screening for other HBLs was performed, but there were no remarkable findings. Cranial MR images, obtained 6 months after surgery, demonstrated an intact pituitary stalk and no residual tumor (). Up to the present, 1 year after surgery, she lives well without neurological deficits or pituitary dysfunction. |
A 74-year-old Caucasian Portuguese woman was referred to our Oral Medicine and Surgery Department by her general practitioner because of a painless swelling of the mandible. The patient reported that the nodular lesion was present for 8 years without pain and notable changes in size. Anamnesis disclosed presence of osteoporosis, hypercholesterolemia, arterial hypertension, and peripheral venous insufficiency.\nAt oral examination an enlargement of the mandible in buccal left premolar region extending from the canine to the first molar () was evident. The overlaying mucosa was of normal colour and texture. This nodule of 2 × 2 cm was firm and painless and had a nontender bony-hard consistency with no fluctuation on manual palpation. Both premolars were vital, and the second one had a partial crown fracture. Both were covered by the lesion. Root remnants of first and second molars were present. The patient had other radicular fragments, dental caries, and periapical periodontitis. No associated cervical lymphadenopathy was detected.\nPanoramic radiography showed a radiolucent lesion with a multilocular appearance between the mandibular left canine and second premolar (). The upper margin of the lesion was well-defined, contrary to the ill-defined lower margin. Root resorption was absent. CT scan revealed a multilocular lesion containing small calcified masses (). This lesion, sized 2.2 × 1.5 × 2.1 cm, involved and displaced 34 and 35 teeth, with expansion and thinning of the buccal and lingual cortical plates partially eroded. The inferior margin of the lesion showed a proximity relation with mandibular canal and mental foramen.\nDifferential diagnosis included odontogenic myxoma, central giant cell granuloma, osseous dysplasia, desmoplastic fibroma, calcifying epithelial odontogenic tumour, keratocystic odontogenic tumour, ameloblastic fibroma, and ameloblastoma.\nExtraction of 37, 36, 35, and 34 was performed and a bioptic specimen of the lesion was obtained under local anaesthesia (2% lidocaine with 1 : 100,000 epinephrine). Histopathological evaluation showed a lesion composed of a fibroblastic/collagenous tissue with many strands or nests of odontogenic epithelial cells without cytological atypia. Focally prominent hyaline calcified foci, resembling osteoid and dentinoid material, were also present (). Such features favoured the diagnosis of a central odontogenic fibroma. After one month, the patient underwent the excision of the lesion under local anaesthesia using Er:YAG (2940 nm) laser for bone osteotomy (). We used a Deka Smart 2940 Plus laser, with articulated arm, with an angulated mirror hand piece, 1 mm of spot, on a short-pulse mode, 15 Hz, 300 mJ, 4.5 W output power, power density of 573.25 W/cm2, and fluence of 38.22 J/cm2. During the excision, we observed that the lesion was almost encapsulated except at its inferior margin with a more ill-defined border. Because of this, additionally to the enucleation and curettage of the lesion, a peripheral osteotomy was performed until clear bone tissue was obtained to ensure complete elimination of the tumour. There were no postoperative complications such as pain, paresthesia, and swelling one week after surgery. Histopathologic examination of the whole specimen confirmed the diagnosis of central odontogenic fibroma, epithelium-rich type (complex or WHO type). No recurrence was seen at the follow-up visits () during a period of 4 years. |
A 17-year-old-female patient presented to Otorhinolaryngology Outpatient Department with a history of swelling over the anterior aspect of neck with a gradual increase in size for last 6 months. There was no history of pain, dysphagia, or hoarseness of voice. There was no history of cold or hot intolerance, tremor, palpitation, diarrhea, constipation, any menstrual irregularities, or any radiation exposure. On examination, a single, well-defined, painless, soft to firm swelling was seen in the midline of neck, measuring about 2 cm × 1.5 cm. The swelling moved with both swallowings as well as the movement of tongue. The overlying skin was normal, and there was no palpable cervical lymphnodes. The routine laboratory investigations including complete blood count, liver function test, and kidney function test was within normal range. The thyroid function test was within normal limit. The ultrasonography (USG) of neck revealed a midline complex cystic mass (2 cm × 1.5 cm) above the thyroid isthmus with multiple internal septations and a central large nodule with heterogeneous echogenicity and coarse calcification []. Both lobes of thyroid were normal in size and echo pattern with smooth margins. The isthmus was also normal, and there was no evidence of necrosis or hemorrhage. Thus, a diagnosis of infected TGDC was made. Following this diagnosis, Sistrunk operation was done which consist of resection of the cyst along with a central portion of the hyoid bone and dissection of the thyroglossal tract up to the base of the tongue. The resected specimen sent for histopathological examination. On gross examination, a globular soft tissue mass measuring 2 cm × 1.5 cm was received along with 1.5 cm elongated fibrous tissue attached to globular mass and a piece of bone. On cut section, a unilocular cyst (1 cm × 0.8 cm) filled with brownish material was seen. Microscopic examination showed a cyst lined by cuboidal epithelial cells with foci of papillary projections with broad fibrovascular core []. The papillae were lined by cuboidal epithelial cells, some of which show nuclear clearing and grooves along with the presence of psammoma bodies in the fibrovascular core []. The fibro-collagenous wall of the cyst contains few colloid-filled follicles, blood vessels, and areas of hemorrhage. Thus, a diagnosis of papillary carcinoma arising in a TGDC was rendered histopathologically.\nThe patient is in regular follow-up and remains asymptomatic for last 1 year. There were no signs of any recurrence. Repeated USG of neck showed normal thyroid scan. |
A 4-month-old female infant was seen with clinical features of persistent hypoglycemia. She was born at 40-week gestation by lower segment cesarean section and weighed 3.9 kg. Her mother did not have diabetes mellitus and she was not on any medications. There was hypoglycemia necessitating dextrose infusions from the second to eleventh day of life. We do not have calculations of the glucose delivery rate in mg/kg/min administered to the baby in that nursing home at that time. The baby was discharged after blood glucose ‘normalized.’ There were 3 episodes of generalized tonic-clonic seizures at 33 days of age necessitating admission to the local medical college hospital, where the baby received intravenous dextrose, hydrocortisone and an unsuccessful trial of Nifedipine. The baby did not have any features of macrosomia. The parents had been adding one sugar cube to each top-up feed of formula following a breast feed but the blood glucose still remained low.\nFrom a neurodevelopmental point of view, her ability to support her head had not fully developed at 4 months. However, she did have social smile and was fixing and following. There was no family history of either diabetes mellitus or CHI.\nInvestigations at the age of 4 months when she was assessed in the pediatric endocrinology clinic were demonstrated in .\nGallium-68 DOTANOC PET/CT scan (Discovery VCT PET scanner, GE Healthcare, Chicago, IL, USA) demonstrated diffuse increased uptake of 68Ga DOTANOC in the body of the pancreas (). A screening echocardiogram prior to commencing diazoxide therapy was negative for structural disease. She was also prescribed hydrochlorothiazide in line with current recommendations to prevent fluid overload in patients on diazoxide. Early weaning was recommended as she was 4 months old.\nShe was commenced on diazoxide therapy with transient improvement in blood glucose levels on an initial total daily dose of 5 mg/kg/day. After 2 days, the dosage of diazoxide was increased to 15 mg/kg/day with some improvement in the blood glucose levels (60–100 mg/dL). Her parents reported that she became more alert and interactive. Genetic tests performed on the infant and her parent's samples at Genetics Laboratory, Royal Devon & Exeter Hospital, United Kingdom confirmed that she had inherited heterozygous ABCC8 mutation from her father which is usually suggestive of focal disease. Sequence analysis identified a previously reported heterozygous paternally inherited missense ABCC8 mutation (p.R1421C [c.4261C>T]) []. There have been no published reports of CHI in India caused by this mutation.\nIn view of the suboptimal response to diazoxide therapy, she was referred to the paediatric surgeons who performed a laparoscopic subtotal pancreatectomy with preservation of the head of the pancreas. The whole excised specimen () was sent for histopathology. The histopathology was suggestive of diffuse hyperplastic pancreatic islet cells suggestive of diffuse CHI ().\nPostoperatively, the infant was started on enteral feeds within 12 hours of surgery. There was initial hyperglycemia which gradually resolved over 48 hours. Apart from 2 episodes of hypoglycemia in the postoperative period, she made an uneventful recovery and was discharged on the 5th postoperative day. The family was counselled to look for features of hypoglycemia, monitor blood glucose at home and to continue breast feeding with a top up feed with formula milk if the blood glucose dropped below 60 mg/dL.\nOne week following the surgery, she was subjected to a controlled fast. Three hours after the fast commenced, the blood glucose dipped to 58 mg/dL. This suggested that there is still some residual hyperinsulinism. Currently, the baby is breast fed and has been fully weaned. Blood glucose levels are between 70–110 mg/dL on frequent feeds and the baby has not been recommenced on diazoxide. Further pancreatic surgery might be warranted if the hypoglycemia recurs, but a balance has to be struck between preventing hypoglycemia and rendering the infant diabetic with a more radical resection of the pancreas.\nInstitutional Review Board approval for this case was not required as this case report described the treatment of a single patient. Parent's written consent has been obtained for this publication. |
A 37-year-old female, otherwise healthy patient, presented to observation with a 3-month history of a mass in the upper right side of the abdomen, which has been gradually increasing in size, and no other associated symptoms. Past medical history was remarkable for atrial septal defect closure at the age of ten, but no previous abdominal surgeries or trauma. Patient did not smoke, drink alcohol or took any medication. During the physical examination, inspection showed a tattoo on the right side of the umbilicus (Fig. A); palpation at the same level revealed a painless and firm mass of 5 × 4 cm2 approximately, with not easily definable edges. Ultrasonography showed a 4 cm2 mass with no clear margins. Abdominal magnetic resonance (MRI) was subsequently performed describing a 17 × 19 × 50 mm3 mass confined into the rectus abdominis (Fig. A and B). Needle biopsy samples were taken, and pathology report confirmed the diagnosis of a desmoid tumour.\nThe surgery for the mass removal was performed under general anaesthesia and the incision was placed in the upper edge of the tattoo, just above the tumour localization (Fig. ). A segment of 7 × 5 × 3 cm3 was resected from the rectus abdominis. Abdominal wall was subsequently reconstructed with primary fascial closure reinforced with underlay polypropylene mesh. Local bupivacaine infusion catheter was placed for pain control []. Abdominal suture was carried out in three layers abdominal fascia using Vicryl 3-0 for abdominal fascia and dermis, and skin with single stiches prolene 4-0 (Fig. B). Wound healing was uneventful and the specimen confirmed a desmoid tumour, without mitotic or necrotic features, radically excised. At three months follow-up the scar was difficult to notice because of the tattoo. At 2-year follow-up, the patient was satisfied with his tattoo scar, while no tumour recurrence revealed by MRI, either abdominal hernia nor bulging could be detectable (Fig. B). |
A 26-year-old Caucasian man with no history of disease was admitted to Gazi University Emergency Department after he had a syncopal episode in his home. The patient was in his usual good state of health until he suddenly collapsed while standing and lost consciousness for approximately five minutes. He recovered spontaneously but was extremely weak and dyspneic. He was also diaphoretic and tachypneic, but denied any associated chest pain or palpitations. No tonic-clonic activity was witnessed, and he experienced no incontinence.\nThe patient was a computer programmer and he had been working 18 hours a day without rest periods for a month. On admission, physical examination revealed a diaphoretic and dyspneic patient without focal neurologic findings. His heart rate was regular but tachycardic at 128 beats/minute, his blood pressure was 126/72 mmHg without orthostatic changes, and his respiratory rate was 32 breaths/minute. The room air oxygen saturation was 90%, and arterial blood gas analysis in room air revealed hypoxemia (PO2 = 58 mmHg) with an elevated alveolo-arterial oxygen gradient (A-a O2 gradient). Examination of his head and neck was normal. The results of chest wall examination revealed reduced breath sounds bilaterally at the lung bases. The findings of heart and abdominal examinations were unremarkable, but on examination of his legs, deep venous thrombosis (DVT) was noted in his left leg, with a positive Homans' sign in the left leg and the left calf measured 3 cm more than the right one.\nLevels of serum electrolytes, glucose, blood urea and creatinine, and complete blood counts were normal. Results of a computed tomographic scan of his head were negative for bleeding, aneurysm or an embolic event. Chest X-ray was clear. An electrocardiogram showed a regular rhythm consistent with sinus tachycardia; there were Q and T waves in lead III and an S wave in lead I. A ventilation-perfusion scan demonstrated an unmatched segmental perfusion defect, indicating a high probability of the presence of a pulmonary thromboembolism (PTE) (Figures and ). A transthoracic echocardiogram revealed normal left ventricle function without a patent foramen ovale, an atrial septal defect or a ventricular septal defect, but with mild pulmonary hypertension (42 mmHg). A Doppler scan of the legs revealed an acute DVT in the patient's left leg, in the popliteal vein. Thrombolytic treatment was not given - the patient received standard anticoagulation treatment with unfractionated heparin and an oral anticoagulant. Before treatment, a blood sample was taken to examine the thrombophilia panel. After a 12-day course of hospital treatment, he was discharged on oral warfarin therapy. The patient's long-term follow-up was performed by the Department of Pulmonary Disease, and we learned that the patient was well for four months after that episode without any evidence of recurrent syncope or pulmonary embolism. |
A 31-year-old white female presented in infancy with nephrocalcinosis, hyperoxaluria and kidney failure. She carried a presumed diagnosis of PH but genetic testing was not done. She underwent two kidney transplants, the first from her father at 15 months of age, with subsequent failure due to severe cytomegalovirus infection. She restarted dialysis and had a second transplant from her mother at 23 months of age. She did well until she became pregnant at the age of 31 years and self-discontinued her immunosuppression, leading to the rapid onset of end-stage kidney disease (ESKD) requiring hemodialysis (HD) thrice weekly. Shortly after dialysis initiation, she presented with acute lower extremity deep vein thrombosis (DVT) and a negative workup for clotting disorders and was treated with warfarin. A few months later she developed nonhealing wounds on both lower extremities that were felt to be calciphylaxis exacerbated by warfarin, a known risk factor for calciphylaxis []. Due to the severe peripheral vascular disease and the worsening ulcerated lesions, the patient underwent a right below the knee amputation and left transmetatarsal amputation.\nShe was then evaluated for a kidney transplant and genetic testing was done, revealing type I PH with two AGXT gene mutations: c.33dupC and Gly170Arg type I (courtesy of the Rare Kidney Stone Consortium). The histology collected from tissue at the time of amputation was retrieved and revealed heavy deposition of calcium oxalate crystals in the medial layer of arteries (), demonstrating the etiologies of the vascular occlusions and amputations were likely due to widespread oxalate deposition from acute rejection/kidney failure in a setting of PH-1. The patient was listed for a combined kidney–liver transplant and underwent HD six times per week to optimize oxalate removal. Pyridoxine was added to her treatment regimen since it has been found that patients with PH-1 with the Gly170Arg and Phe152Ile gene mutations are highly responsive to pyridoxine supplementation, which is a cofactor to the AGXT enzyme, leading to a significant reduction of urinary oxalate levels [, ]. The liver–kidney transplant was done and she is doing well 5 years posttransplant. |
A nine-year-old entire male greyhound presented with head trauma resulting from a collision with a park bench. The dog had no previous significant clinical history. On examination, cardiovascular parameters were stable overt distress in the animal not apparent. The dog was ambulatory with normal gait and posture devoid of proprioceptive deficits.\nThorough head inspection revealed subcutaneous emphysema between the eyes and a superficial cut to the right dorso-orbital region. Mild right unilateral epistaxis was noted. The dog resented palpation of the right frontal bone and a communication with the sinonasal cavity was inferred by the presence of a flail segment movement of the bone synchronous with respiration. Cranial nerve examination demonstrated bilateral delayed pupillary light reflex (PLR) and normal pupil size, the remainder of the neurological examination was within normal limits.\nA right-sided frontal bone depression fracture was suspected founded on clinical findings. Radiographs and Computer Tomography (CT) imaging with a three-dimensional reconstruction of the skull were performed under general anaesthesia (see Fig. -). Radiographs of the cervical spine were unremarkable.\nCT imaging revealed a comminuted, depressed fracture of the frontal bone that extended from the level of the maxillary recesses up to the caudal aspect of the frontal sinuses at the level of the dorsal aspect of the right maxillary, nasal and frontal bones. Surgical repair of the defect was warranted to reestablish sinus architecture and mechanical stability []. Further, fracture comminution is associated with soft tissue contracture leading to cavitation with connective tissue scarring and sequestrum formation [], fracture repair addresses soft tissue injury and may minimize long-term risks of complication []. Surgery was carried out three days after admission.\nA standard dorsal approach to the frontal bone was taken (Fig. -). A malleable highly porous Ti mesh (0.2 mm thickness with 1.4 mm by 0.6 mm elongated pores) was contoured to the patient’s skull (Fig. -).\nThe Ti mesh was firmly seated against the skull and a 1.6 mm drill bit, with a 6 mm stop, was used to drill pilot holes through the mesh into the skull. A 2.1 mm diameter resorbable poly-L, D-lactic acid (PLDLA) thermoplastic pin was placed into the pilot hole and the ultrasonic trode applied to the proximal end of the pin (Fig. -). The ultrasonic device was activated melting the outer surface of the pin and allowing it to be advanced into the pilot hole. Further pilot holes were drilled around the periphery of the Ti mesh at 20-25 mm intervals and thermoplastic pins inserted until the Ti mesh was adequately secured.\nThe surgical site was flushed with copious volumes of sterile saline and a sheet of gentamycin-impregnated collagen (Collatamp®, EusaPharma) was overlaid on the implant to prevent infection and assist in achieving a pneumatic seal. Prophylactic intravenous antibiotic cover was provided by clavulanated amoxicillin (20 mg/kg; Augmentin, GSK) given 30 min pre-operatively and every 90 min thereafter for surgery duration. Recovery from anaesthesia was uneventful. Post-operative analgesia was provided with intravenous methadone (0.2 mg/kg q4hours; Comfortan, Dechra). Postoperative radiographs and CT images showed satisfactory positioning of the Ti mesh and adequate coverage of the calvarial defect (Fig. -).\nThe dog was discharged two days after surgery with a 10 day course of oral carprofen (2 mg/kg q24hours; Rimadyl, Zoetis) and oral clavulanic acid-amoxicillin (20 mg/kg q12h; Synulox, Zoetis).\nThe dog re-presented two weeks after discharge, with hyperthermia and swelling around the surgical site. Subcutaneous oedema was present in the absence of emphysema. Sampling for culture and sensitivity was prevented by the absence of nasal and surgical site discharge, exposure of the wound to obtain a swab sample was deemed inappropriate. Antibiotic therapy effective against common nasal pathogens [] was introduced with oral cefalexin (20 mg/kg q 12 hours; Therios, Ceva) for ten days. Clinical signs had resolved at 1 week post treatment.\nAt the six weeks recheck, the physical examination was within normal limits. No pain was elicited upon palpation of the surgical site and no indications of infection recurrence was found. Radiographs and CT of the skull revealed a slight uplift of the mesh at its most rostral aspect from the frontal bone. Mesh uplift was not a clinical concern at this stage. Radiographs and CT scan at 6 months revealed soft tissue swelling between the rostral mesh portion and the skull (see Fig. -). Decision was made to trim and re-contour the uplifted mesh. The procedure and recovery were uneventful and no further complications were experienced.\nAt 2 years assessment, radiographs and CT scan confirmed adequate contouring and positioning of the Ti mesh. Radiographs revealed a bone-dense opacity between the rostral part of the mesh and the skull potentially associated with mesh micromotion stimulating periosteal reaction (Fig. -). The degree of new bone formation around the mesh periphery was difficult to ascertain radiographically, however, clinical examination was unremarkable and cosmetic result was satisfactory. The owners did not raise any further concerns regarding the patients ability to participate in normal lifestyle and activity. |
An 18-year old male patient was referred to our emergency clinic with injuries sustained in a paragliding accident. During takeoff from the slope with a parachute, and after achieving an altitude of 10-15 meters, the patient stated that he had dropped on his feet due to loosening of the security ties.\nPhysical examination revealed limitation of movement, ecchymosis, and edema over both feet and ankles. Pain and tenderness were especially localised anteriorly on the right ankle and dorsally over the calcaneocuboid joint on the right foot, and anteromedially on the left foot and around the left medial malleolus. There was no open wound and neurovascular status was intact in both feet. Antero-posterior (AP) and lateral (LAT) radiographs of the foot and ankle revealed coronal shear fracture of the body of the talus, an anterior process fracture of the calcaneus extending to the calcaneocuboid joint and a nondisplaced navicular body fracture of the right foot () and a displaced fracture of the navicular body accompanied with posteromedial process fracture of the talus on the left foot ().\nTo determine the exact localisation of the fragments and the degree of fracture displacement more accurately, a computerised tomography (CT) scan was performed. This revealed the coronal shear fracture of the talar body and the navicular fracture displaced 3mm and 1mm respectively, while the anterior process fracture of the calcaneus was minimally displaced on the right foot (). On the left foot, the posteromedial process fracture of the talus was displaced approximately 3mm, and revealed fragmentation, and that the navicular fracture consisted of three fragments and was dorsally displaced ().\nThe patient was operated on eight hours from the time of the trauma. Under general anaesthesia and pneumatic tourniquet, an anteromedial incision was made for the talar body fracture at the right side. Neurovascular structures were identified and protected, and an oblique osteotomy was performed on the medial malleolus. The talar body fracture was visualised. The vertical fracture line in the coronal plane was close to the posterior and displaced causing stepping-off on the talar dome but had no fragmentation. Following curettage and irrigation of the fracture site, it was anatomically reduced, and fixation was performed with two 4.5mm self-tapping screws from anterior to posterior to obtain compression. The medial malleolus osteotomy was fixed with one 4.5mm self-tapping screw and figure-eight wire looping. Fractures of the anterior process of the calcaneus and navicular body were managed non-surgically. The foot and leg were immobilised in plaster cast postoperatively ().\nAn anteromedial incision was also used for the left foot. Following the identification and retraction of the neurovascular structures, an oblique medial malleolus osteotomy was carried out. Posteromedial process of the talus was explored. After anatomic reduction, a small lateral incision was made and a 4.5mm self-tapping screw was placed through the lateral aspect for fixation of the large posteromedial fragment. Extending the incision towards the navicular bone, the fracture line and talonavicular joint were explored. The small bone fragments at the fracture site were removed. After anatomic reduction, fixation was achieved utilising a 4.5mm self-tapping screw from the dorsal to the plantar aspect. The talonavicular joint was stabilised with a Kirschner wire from anterior to posterior, and it was left extruding on the skin. Medial malleolus osteotomy was fixed with a 4.5mm self-tapping screw. A short leg cast was applied postoperatively ().\nParenteral cefazolin sodium (3x1 gr/day) was administered for three days postoperatively. After six weeks of immobilisation, plaster casts were removed and the Kirschner wire stabilising the talonavicular joint was pulled out. Radiographic evaluation following a rehabilitation period of six weeks showed consolidation and union of all fractures without any loss in reduction in both feet, and full weight bearing was encouraged. At 36 months follow-up there was no evidence of avascular necrosis. All fractures had completely healed. There were minimal arthritic changes at the right subtalar and calcaneocuboid and left talonavicular joints. There was no complaint of pain and the range of motion of both feet and ankles were near full. The patient regained his full activity level at the 5th month postoperatively. Maryland scores of the right and left feet were 85 (good) and 90 (excellent), respectively (). |
A 4-year-old boy without significant medical history was admitted for limited neck motion for 3 wk. The physical examination showed an irreducible torticollis with analgesic attitude of cervical spine. The active and passive mobilization of the neck was painful and no motor or sensory deficit was detected. The general condition of the patient was good, the clinical examination did not show a tumoral syndrome and the neurological examination as well as skin examination and laboratory tests were normal.\nThe magnetic resonance imaging (MRI) of cerebro-spinal cord uncovered an infiltrative mass involving the dens of C2 which is hypointense on T1 sequence and hyperintense on T2 sequence, extending to the surrounding soft tissues leading to an increase in C1-C2 space, without compression of the spinal cervical cord. Complementary CT showed fragmented dens with important C1-C2 dislocation (Figure ).\nThe odontoïd and mass biopsy was performed by endoscopic guidance. Histological features were consistent with inflammatory EG. The positivity of the immunostain by the antibody anti Ps100 and the antibody anti CD1a confirms the diagnosis of LCH (Figure ).\nInitial treatment was started prednisolone 40 mg/m2 per day orally, with weekly reduction starting from week 4 and intravenous Vinblastine 6 mg/m2 per week for six weeks. An external immobilization by a cervical collar was maintained during the entire period of chemotherapy.\nThe evolution was marked by a decrease in pain secondary to the active mobilization of the neck with a persistent passive analgesic position. The control radiologic MRI showed a displaced horizontal fracture of the dens responsible for a posterior wall recoil reducing cervical occipital hinge without intramedullary signal abnormality. The infiltrative process had regressed in size (Figure ).\nA posterior cervical arthrodesis was performed and the spine was stabilized with a metal lacing associated with tricortical iliac crest graft interposed between the posterior arch of C1 and C2. No neuro-vascular complications have been detected.\nThe patient is still under treatment consisting of prednisolone 40 mg/m2 per day orally for five days in a week every four weeks and IV Vinblastine 6 mg/m2 bolus every four weeks for 12 mo. Repeated CT scans revealed at 5 mo a consolidation of C2 fracture with moderate stenosis of the occipital hinge (Figure ) |
A 54 year-old Caucasian male without significant comorbidities was diagnosed with IgG kappa multiple myeloma in 2005. Initial treatment consisted of doxorubicin, vincristine, and dexamethasone followed by an autologous stem cell transplant (SCT) with melphalan 200 mg/m2 conditioning. He remained in remission for 2.5 years, at which time he relapsed and was treated with a series of doublet regimens followed by a second autologous SCT in 2011, with melphalan 200 mg/m2 conditioning. He relapsed 4 months after the second transplant and was treated with carfilzomib but quickly progressed. The patient eventually achieved a very good partial response with bendamustine and dexamethasone and underwent reduced intensity conditioning with fludarabine 30 mg/m2 on days − 6 to − 2 and melphalan 50 mg/m2 on days − 3 to − 2, followed by a 9/10 matched unrelated allogeneic SCT in November 2012. GVHD prophylaxis consisted of sirolimus and tacrolimus starting day − 3 as well as methotrexate on days + 1, 3, 6, and 11. On day + 27 post-transplant the patient developed acute kidney injury (creatinine of 2.6 mg/dL from a baseline of 0.7) that was attributed to calcineurin inhibitor toxicity. The patient was switched to mycophenolate mofetil and corticosteroids for GVHD prophylaxis, with normalization of kidney function. By day + 130 the patient was felt to be in at least very good partial remission based on negative serum protein immunofixation and 99.8% peripheral blood donor chimerism.\nOn day + 132, the patient returned to the hospital with diarrhea with scant blood. He underwent colonoscopy with biopsy. Histologic analysis demonstrated findings consistent with CMV colitis and GVHD: crypt apoptotic bodies, ulcerations, and CMV inclusions were noted. He was started on ganciclovir, and prednisone was increased from 60 mg daily to 60 mg twice daily. He was discharged 2 weeks later, at which time the platelet count had decreased from 93,000/μL on admission (normal 150,000–450,000/μL) to 29,000/μL. The thrombocytopenia was attributed to a combination of antiviral medication and CMV infection.\nHe returned to the hospital 1 week later (day + 146) with recurrence of profuse diarrhea with small amounts of blood and associated abdominal cramping. Diarrhea was attributed to worsening GVHD. He was restarted on tacrolimus but continued to have maroon-colored stool output. A colonoscopy was repeated and was notable grossly for pancolitis with scattered ulcerations, ileocecal valve ulceration, mild ileitis, anorectal junction ulcers, and internal hemorrhoids that were not bleeding. The pathology report again suggested CMV infection and GVHD (increased crypt apoptotic bodies, focal erosions, and a positive CMV immunostain). During that admission, a diagnosis of TMA was considered due to a persistently low hemoglobin of approximately 9 g/dL (normal 13–17 g/dL) and thrombocytopenia that persisted in 30,000–50,000/μL range. A peripheral smear at that time showed 2–4 schistocytes/HPF in the setting of a haptoglobin of 22 mg/dL (normal 36–195 mg/dL) and a lactate dehydrogenase (LDH) of 731 U/L (normal < 260 U/L). Overall, the patient met 6 out of 7 of the TA-TMA criteria proposed by Cho et al. His tacrolimus was again discontinued, and he was maintained on a combination of sirolimus (goal level 5–7), mycophenolate mofetil, and steroids for GVHD treatment. At the time of discharge, his hemoglobin was 8.7 g/dL and platelets were 20,000/μL.\nOn day + 156 the patient returned to the hospital with multiple episodes of bright red blood per rectum. He had diffuse patchy ecchymoses on physical exam. The platelet count was 17,000/μL. A colonoscopy was repeated, and per visual inspection the GVHD was felt to be improved. Hemorrhoidal bleeding was suspected as the cause of the bleeding, and colorectal surgery was consulted. He underwent sclerotherapy for hemorrhoids prior to discharge.\nOn day + 188 TMA was again considered in the setting of an LDH of 1147 U/L. Sirolimus was discontinued. A peripheral blood smear, however, showed no schistocytes, and haptoglobin returned within low-normal range at 43 mg/dL. TMA was therefore felt to be unlikely. A CT scan of the abdomen and pelvis, performed for another indication, revealed an unexpected finding of pneumatosis intestinalis involving the ascending and transverse colon. This finding was attributed to GVHD and CMV and, as the patient was felt to be relatively asymptomatic, no specific intervention was performed. Sirolimus was restarted given the concerning imaging findings and lack of strong evidence for TMA.\nThe patient was re-admitted on day + 211, this time with melenic stool. A tagged red blood cell scan identified the ascending colon as a source of bleeding. An angiogram with attempted embolization was unsuccessful due to inability to identify a bleeding vessel. Hemolysis labs were repeated given persistent cytopenias, including a hemoglobin of 7.1 g/dL and a platelet count of 37,000/μL. Haptoglobin was undetectable, and LDH was elevated at 1254 U/L. A repeat peripheral smear demonstrated a normocytic, normochromic anemia without increase in schistocytes. Sirolimus was again discontinued. The patient remained anemic and intermittently refractory to red blood cell transfusions, prompting two additional attempts at angiogram/embolization, neither of which successfully identified a bleeding vessel. A bone marrow biopsy was repeated and revealed a hypocellular marrow without evidence of myeloma relapse.\nOn day + 217 the patient was transferred to the medical ICU for high volume bloody stool output, a hemoglobin of 6.3, and lightheadedness. The INR was 1.0 with a PTT of 22. A wireless capsule endoscopy and a repeat tagged red blood cell scan were both unsuccessful at identifying a source of bleed. A colonoscopy with biopsy was repeated; 2 visible vessels were identified and clipped. Histologic analysis did not show evidence of persistent GVHD or CMV colitis. An EGD with biopsy revealed no abnormalities on histologic analysis. He was managed supportively with transfusions as needed but then began to develop confusion, agitation, and increasing anger. Psychiatry was consulted for delirium. LDH was 767 U/L, and the haptoglobin was low at < 35 mg/dL. Platelets were 47,000/μL, and the INR was 1.1. A peripheral smear was repeated and did not show schistocytes. An ADAMTS13 level was ordered given recurrent suspicion for TMA, and therapeutic plasma exchange (TPE) was initiated empirically pending that result. The patient’s confusion was noted to improve following TPE, and he completed a total of 5 exchanges. LDH improved to 372 U/L. ADAMTS13 level ultimately returned at 60% (normal > 66%). Shortly thereafter, the patient developed copious hematochezia associated with a drop in hemoglobin from 8.9 to 6.9 g/dL. He was taken urgently to the operating room for visceral angiogram, which did not identify a bleeding vessel. An ileocolectomy was performed, but the patient suffered a cardiac arrest intra-operatively and expired shortly thereafter.\nA post-mortem analysis of the resection specimen revealed TMA involving numerous arteries and arterioles in the ileal and colonic submucosa as well as few thrombosed arterioles in the muscularis propria and deep lamina propria of the mucosa (Figs. and ). Rare thrombosed arterioles were identified in the appendix. The TMA in many of the vessels was active, characterized by endothelial cell swelling, endothelial cell detachment with intimal expansion by pale plasma protein material (“mucoid intimal edema”), and accumulation of fibrin and red cell fragments within expanded intimal zones and vascular lumina. A smaller number of arteries and arterioles showed features of persistent or chronic microangiopathic changes, such as concentric layers of new basement membrane material alternating with intimal edema well as focal presence of foam cells within artery intimal zones. All venous structures were patent without thrombosis. In areas of more severe TMA, there were foci of perivascular hemorrhage and deep ulceration extending into the superficial muscularis propria. Non-ulcerated mucosa showed architectural distortion and crypt regenerative features, consistent with previous injury. Additionally, there were areas of crypt dropout with replacement of mucosa by healing granulation tissue and an overlying layer of regenerative epithelium. Scattered CMV inclusions were visible within endothelial cells of the granulation tissue. However, there was no evidence of CMV infection by light microscopy or immunohistochemistry within endothelium of vessels affected by TMA. There was no significant increase in crypt apoptosis, arguing against GVHD.\nA retrospective review of the patient’s previous colonic biopsies was performed. In addition to the initially reported findings, biopsies from days 146 and 217 showed subtle features of TMA (Fig. ); these included rare thrombosed arterioles in the deep lamina propria and superficial submucosa surrounded by perivascular hemorrhage as well as few arterioles with subendothelial expansion by pale material accompanied by underlying layers of new basement membrane material. |
A 68-year-old female, right hand dominant, slipped on ice and fell on her left outstretched hand. There were no open wound, no neurovascular deficit and no other significant injuries. The skin was not at risk. Standard x-rays of the shoulder girdle showed a minimally displaced lateral fracture and a second displaced medial fracture of the left clavicle. A conservative treatment was proposed with regard to the patient’s age and her low functional demand. A week later the assessment was completed by a computed tomography (CT) scan which underlined a displaced medial fracture with shortening of the clavicle bone (). This statement drove us to propose a final fixation of the clavicle by means of two locking plates. The hardware used consisted of two 3.5/2.7 locking compression (LC) anatomical plates with a lateral extension (DePuy Synthes®, United States). A left sided plate was used for the lateral fracture and placed cranially while a right sided plate was used for the medial fracture and placed anteriorly. The patient was operated in beach chair position under general anesthesia. Antibiotic prophylaxis was given at the induction. A longitudinal anterior incision was used. The reduction of the medial side of the intermediate segment of the clavicle was particularly difficult due to the small medial fragment still attached to the sternoclavicular joint, and the rupture of the costoclavicular ligament and the lever arm produced by the coracoclavicular ligaments still attached to the lateral side of the intermediate fragment. Fixation of the lateral fracture facilitated the reduction and fixation of the medial fracture. Three 2.7 mm locking screws were placed in the medial fragment and stabilized the sternoclavicular joint by means of a transosseous non-resorbable suture of #2 FiberWire (Arthrex®, United States) running through two holes of the extension of the right sided plate placed anteriorly and through two tunnels of 2 mm of diameter drilled in the manubrium. Early passive and active mobilization of the left shoulder was begun from the second postoperative day. A sling was used for six weeks as antalgic support and the abduction of the left arm was limited to 90° during this period because of the poor quality of the clavicle bone and the relative stability of the fixation of the medial fracture. After six weeks the patient was practically pain free and presented a limitation of the range of shoulder motion. After three months the patient was able to completely move her left shoulder. After six months bony consolidation of the clavicle was achieved (). After the last control (at 12th months) the Disabilities of the Arm, Shoulder and hand (DASH) score was 5.0 and there was no significant hardware irritation. |
In May 2013, the patient, a 63-year-old male retired truck driver presented with hoarseness of voice. He is an ex-smoker with a 30-pack year history and history of heavy alcohol use. Other medical history included depression, gastro-oesophageal reflux disease (GORD), hypothyroidism and chronic obstructive pulmonary disease (COPD). Investigations demonstrated a left-sided glottic tumour and the patient proceeded with vertical hemilaryngectomy. Histopathology of the glottic tumour revealed a T3N1M0 SCC with micrometastatic involvement in 1 of the 25 resected lymph nodes. At the time of diagnosis, a positron emission tomography (PET) scan demonstrated no metastatic involvement. Following surgery, the patient attended annual reviews by his ear, nose and throat surgeon and did not demonstrate evidence of recurrence.\nHe re-presented 42 months later with a 1-week history of right lower quadrant pain and no other associated symptoms. Initial abdominal ultrasound demonstrated no abnormalities and he progressed to contrast enhanced computed tomography (CT) of the abdomen and pelvis. CT demonstrated a focal abnormality involving the pelvic-ureteric junction characterised by mural thickening and hyper-enhancement of the right renal hilum suggestive of a neoplastic process. The lower pole of the ipsilateral kidney also demonstrated a 3 cm cyst that appeared to be benign. The contralateral kidney and liver were unremarkable on CT.\nHe was referred to a urology team on the suspicion of urothelial carcinoma. The urology team performed right-sided cystoscopy and retrograde pyelogram with lavage cytology which demonstrated a normal collecting system and normal urothelial cells which made the differential diagnosis of urothelial malignancy unlikely. The differential diagnosis included renal cell carcinoma (RCC). He had no haematuria; urine microscopy, culture and sensitivity were normal, and there were no other urological symptoms. The opinion of the interventional radiologist was that a percutaneous biopsy of the mass was too risky given its proximity to major vasculature structures. The patient underwent a positron emission tomography–magnetic resonance imaging (PET-MRI) scan that demonstrated a right-sided renal hilar mass encasing the renal vessels and showed evidence of invasion into the inferior vena cava (IVC) with possible invasion of the right crus of the diaphragm. No metastatic lesions were detected at other sites ().\nLaboratory tests revealed renal insufficiency with a serum creatinine of 130 µmol/L and an estimated glomerular filtration rate of 49 mL/min/1.73 m2 and a urinary albumin to creatinine ratio of 0.9 mg/mmol. The unusual finding of an infiltrative renal hilar mass on CT that did not demonstrate the usual characteristics of RCC on imaging and was not amenable to biopsy was brought to a multidisciplinary team (MDT) meeting for discussion. The MDT came to the consensus that the renal mass should be managed surgically, as if RCC. The risks and benefits of surgical management were discussed with the patient, and he underwent radical nephrectomy with caval thrombectomy and IVC replacement. The patient recovered from surgery.\nMacroscopic features showed a 45 mm cream-coloured tumour in the mid- to upper pole with no haemorrhage or necrosis (). The tumour showed invasion through the IVC wall, extending into the psoas muscle. Histopathology demonstrated a moderately differentiated SCC with associated fibrosis (). The tumour also showed extensive lymphovascular invasion and peri-neural invasion involving the vascular margin. Two of the five resected hilar lymph nodes demonstrated involvement, whereas the one resected pre-aortic lymph node did not demonstrate involvement. The patient was referred to a radiation oncologist to discuss the possibility of adjuvant radiotherapy. |
A 50-year-old man presented with a swelling in the left axilla of 2 months duration. Initially, it was a small swelling that had increased progressively and reached a size of 7 cm × 5 cm. The patient was febrile on and off for last 2 months. He had no significant past or medical history. There was no regional lymphadenopathy; general physical and systemic examination was within normal limits. Local examination of left axilla revealed a large, firm, nontender pedunculated swelling of size 7 cm × 5 cm. Skin overlying the swelling was inflamed and ulcerated. With a clinico-radiological diagnosis of soft tissue sarcoma, the patient was referred for FNAC.\nHighly cellular smears showed cohesive cell clusters of malignant cells composed of two cell population; polyhedral cells with dense basophilic cytoplasm along with glycogen containing pale/clear cells with a clear cytoplasm. The nuclei were cytologically pleomorphic, hyperchromatic, round to oval with coarse chromatin and prominent nucleoli. The nuclei were eccentrically located within a moderate amount of basophilic cytoplasm, with a relatively well-defined cell borders imparting a plasmacytoid appearance. There were many bi-and multinucleated cells [Figures and ]. Few cells were showing squamous differentiation. At places acinar pattern and tubule formation along with numerous mitotic figures were seen. Cytological diagnosis of malignant eccrine acrospiroma was offered. Subsequently, surgical excision with adequate margins was done and sent for histopathological examination.\nGross examination revealed an ulcerated skin covered well-circumscribed solid mass measuring 5 cm × 4 cm × 3 cm []. Cut section was grey-brown with areas of hemorrhage and necrosis. Microscopic examination showed a skin covered tissue revealing sharply demarcated lobules of tumor cells located in the dermis and extending into the subcutaneous tissue. There was no connection between the epidermis and the tumor. Most of the lobules were solid and some contained cystic spaces filled with homogenous, eosinophilic material and lined with a thick coat of tumor cells. Interstitial hyaline collagen changes were present around lobules. The cell population was made up of two cells types; predominantly clear cell type and fusiform cell type with eosinophilic cytoplasm []. The tumor cells were highly pleomorphic with irregular nuclear membrane, coarsely clumped nuclear chromatin, prominent nucleoli, and clear cytoplasm. Numerous mitotic figures and atypical mitotic figures were appreciated []. Tumor cells were seen invading into the surrounding stroma. Angioinvasion was visible along with focal areas of necrosis. No basaloid, sebaceous, or trichilemmal differentiation were noted. Periodic acid-schiff (PAS) positive diastase-resistant material was demonstrated in clear cytoplasm. Hence, a diagnosis of malignant eccrine acrospiroma was made. |
A 53-year-old female patient presented to our emergency room with subarachnoid hemorrhage (SAH) and intracerebral hemorrhage (ICH) after undergoing burr hole trephination and catheter drainage at a local hospital. She did not exhibit any neurologic deficits and only complained of severe headache. Upon reviewing her transfer request, we discovered that she had experienced progressive headache, transient motor weakness, and mild dysarthria for a month. Preoperative neuroimaging assessments with magnetic resonance (MRI) and angiography (MRA) revealed no abnormalities other than a subdural hygroma with arachnoid cyst (). The local hospital was unable to find any other cause for the mild dysarthria and transient motor weakness; therefore, they determined that the subdural hygroma was the cause of the patient's symptoms. She underwent evacuation of the subdural hygroma through burr hole trephination. A subdual drainage catheter (12 Fr round catheter) was placed in the subdural space, but only 130 cc of cerebrospinal fluid (CSF) was drained over a 24-hour period and the headache persisted postoperatively and became more severe. An emergency brain computed tomography (CT) scan revealed a newly developed ICH and SAH in the contralateral hemisphere and SAH in the ipsilateral hemisphere (). Thereafter, the local hospital transferred the patient to our emergency room for evaluation and treatment of the remote hemorrhage.\nNo other causes of remote hemorrhage in the contralateral hemisphere (e.g., vascular abnormalities) were identified upon reviewing the findings of the previous brain MRA examination (). According to the local hospital's medical records, she was not on any anticoagulation and antiplatelet medications and her coagulation panel showed findings within the normal limits (prothrombin time [PT] time 10.0 seconds, PT international normalized ratio [INR] 0.93 and activated partial thromboplastin time [aPTT] 21.8 seconds). During the operation, the patient's condition was stable and blood pressure (BP) was within the normal limits. Therefore, the remote hemorrhage was suspected to be related to CSF overdrainage through the subdural drain after the burr hole trephination. We closed the subdural catheter and the patient's headache gradually subsided. Follow-up brain CT scans were performed 5 and 14 days after the catheter closure. SAH and ICH had resolved and the subdural space was refilled with CSF. One month later, the patient returned to a normal daily life. |
A 12-year-old mentally subnormal boy from rural background was brought by his family with a lump in the left subareolar region which was first noticed 6 years back as a small pea sized nodule and grew painlessly to the size of presentation. The patient had no other complaints and his relatives denied any history of similar complaints in the family. The family members reported delayed milestones for the patient who performed poorly for his age and had poor speech and mental development. On examination the patient showed hypertelorism, low set ears, and left sided undescended testis. The breast examination showed a 6 × 6 cm size lump underlying the areola which was firm to hard in consistency and fixed to the overlying thinned skin. The lump was mobile over the underlying muscle. The patient had no axillary lymphadenopathy. On evaluation, otorhinolaryngoscopic evaluation of the patient was within normal limits. Core needle biopsy of the patient showed fibrocollagenous tissue with mild epithelial hyperplasia and was suggestive of gynaecomastia. Ultrasonography of the axillae and the abdomen was normal. Ultrasonography of the scrotum localised the left testis to the left inguinal region.\nIn view of clinical suspicion the patient was advised a wide local excision of the lump and axillary sampling which on histopathological examination revealed a 5 × 4 cm firm grey lesion harbouring secretory carcinoma with foci of stromal fibrosis and apocrine change with pathologically negative 8 lymph nodes. The margins were negative for presence of the tumor. The tumor was negative for estrogen and progesterone receptors and Her2neu. On immunohistochemistry the tumor stained positive for S-100 stain ().\nIn view of the subnormal mentation of the patient and the lack of definitive supportive data in the disease subset, his relatives refused adjuvant therapy. The patient subsequently is on periodic surveillance and is healthy after 6 months of follow-up. |
A 37 year old Caucasian woman presented to our referral centre with invasive melanoma. For one year she had noticed a posterior thigh raised pigmented lesion, which rapidly grew over three months. It measured 2 cm and bled spontaneously. She had no constitutional symptoms such as fever, malaise, or weight loss, or significant medical history. Histopathology of the initial shave biopsy demonstrated invasive melanoma with Breslow thickness greater than 4.9 mm (positive deep margin), Clark level IV, ulceration, and a mitotic rate of 28 mitoses per square millimeter.\nPhysical examination revealed a 2.5 cm healing biopsy site on the left thigh with residual pigmentation. Further examination was unremarkable, with no satellite lesions, in-transit disease, palpable lymphadenopathy or abdominal organomegaly. No additional suspicious skin lesions were identified.\nDue to the aggressive pathological features of the primary lesion and difficulty with clinical nodal assessment due to the patient’s body habitus, she was staged with FDG PET-CT. Intense hypermetabolic activity was seen in the ipsilateral left superficial inguinal lymph node basin, the largest of which measured 19 x 14 mm, and two or three adjacent tiny lymph nodes. Additionally, a single 9 mm deep inguinal (external iliac) lymph node demonstrated intense hypermetabolic activity (Figure A and B). Moderate hypermetabolic activity was seen on the posterior left thigh corresponding to the residual neoplasm and biopsy site. There was some physiological FDG uptake but no further suspicious areas identified. Ultrasound-guided fine-needle aspiration of the largest superficial left inguinal lymph node demonstrated metastatic melanoma.\nThe patient underwent a wide local excision of the primary tumor and left superficial and deep inguinal node dissection.\nPathology identified eleven lymph nodes in the superficial and deep lymphadenectomy specimen. One of the superficial left inguinal lymph nodes contained metastatic melanoma, confirmed by HMB-45 and melan-A positivity. However, five deep inguinal lymph nodes (2 large and 3 smaller nodes) were partially involved by a florid atypical lymphohistiocytic proliferation with a brisk mitotic rate and abundant necrosis. The specimen was referred for Hematopathology consultation due to a concern of high-grade lymphoma. The involved lymph nodes showed partial architectural effacement by a mixed proliferation of small lymphocytes, large immunoblast-like lymphocytes, and histiocytes, present in a predominantly paracortical distribution. Mitotic rate in these areas was very brisk (up to 10 mitoses per high power field). Many of the histiocytes had eccentric crescent-shaped nuclei and contained abundant intracytoplasmic karyorrhectic debris (Figure A). Neutrophils and eosinophils were noticeably absent. In some areas, the cellular proliferation was entirely replaced by large zones of necrosis. Fibrin thrombi were identified within numerous blood vessels in and around the necrotic areas. By immunohistochemistry, most of the lymphocytes were CD8 positive cytotoxic T-cells. The abundant histiocytes were highlighted by CD68 (Figure B) and lysozyme, and also showed strong expression of myeloperoxidase (Figure C). Ki-67 staining confirmed a high proliferative index of approximately 80% (Figure D). The uninvolved areas of the lymph node showed reactive follicular hyperplasia and fine septal fibrosis, but were otherwise normal. These morphologic and immunophenotypic features are classic for histiocytic necrotizing lymphadenitis, also known as KFD. This proliferation was morphologically and immunophenotypically distinct from the patient’s concurrent metastatic melanoma, which consisted of sheets of large, pleomorphic epithelioid cells with expression of HMB-45 and melan-A. |
A 15-year-old girl child from Chitradurga district in the state of Karnataka-India had presented to the emergency ward with a history of generalized seizures. Mother noticed that after pouring hot water on the head, while giving the bath, the child suddenly became floppy and developed generalized seizures with the loss of consciousness. She has had similar episodes from the age of 5 years. Each episode is preceded by pouring hot water over head while taking a bath and splashing water on her body never caused seizures. She had been started on several anticonvulsants by several doctors, but they had been discontinued as they had been ineffective in preventing seizures. The details of previous treatment were unfortunately unavailable. We were also unsure about her compliance with previous treatment. Her birth was uneventful, and the development was normal. There was no family history of similar seizures. On examination, the child was afebrile, conscious, and there were no neurological deficits. Routine blood tests, including complete blood count, serum electrolytes, serum calcium, blood sugar, and magnetic resonance imaging (MRI) brain, as well as electroencephalography (EEG) were within normal limits. The seizure was provoked in the hospital under controlled settings, and the child developed generalized tonic-clonic seizures within 30 s of pouring water on her head which was heated to 42°C. The seizure was controlled with intravenous midazolam. Treatment with anti-epileptic drugs (AEDs) was planned to prevent HWE in this child. The main reason to try this treatment was the advanced age of the child and enables her to be more independent. Phenytoin (PHT) and carbamezepine were two options, but both require to be taken daily. Hence intermittent prophylaxis with clobazam (CLB) to be taken 2 h prior to having a head bath was considered. The next day, the child was given 5 mg of CLB and seizure provocation was attempted after 2 h. EEG could not be recorded during the attempted seizure provocation. The child did not have a seizure after pretreatment with CLB. The child was discharged with instructions to take CLB 2 h before having a bath. They were also advised to reduce the temperature of the water while bathing. In the follow-up visits, the parents reported that the child did not have any seizure recurrences. |
A 47-year-old male admitted to our department with pain and swelling on his right calf. The patient was diagnosed with RA 5 years ago. He had no history of knee trauma. On physical examination, tenderness of wrists and elbows, swelling on the left wrist and contracture of right elbow were found. McMurray test was negative bilaterally. There was no swelling in the left knee. Right knee flexion was limited and Ballotman test was positive. There was bilaterally knee joint tenderness with palpation.\nHe had painful swelling and redness on his right calf (Fig. ). There was a persistent itching on the skin of the right calf. Homans test was positive on the right. Plain radiographs showed mild degenerative changes at knee joints. Erythrocyte sedimentation rate was 22 mm/hour, C-reactive protein was 24.7 mg/L, WBC count was 14350, rheumatoid factor was positive. Complete blood count except for WBC and biochemical laboratory tests were within normal limits.\nThese findings on the calf were similar to deep vein thrombosis. We used ultrasonography for differential diagnosis of deep vein thrombosis. Ultrasonography revealed massive fluid collection within the fascial compartments and gastrocnemius muscle layers (Fig. ). There were two septas between fluid collections. Color Doppler ultrasonography showed normal flow pattern in popliteal veins. According to these findings, the patient was diagnosed as Pseudothrombophlebitis syndrome due to rupture of Baker’s cyst. 280 cc inflammatory fluid was aspirated from three different points under the guidance of ultrasonography. After the intervention, the swelling on the right calf decreased. The patient was relieved and elastic bandage was applicated to his calf. After 1 week follow-up, the swelling on his calf repeated and another intervention was performed. Approximately 100 cc inflammatory fluid was aspirated and 1 cc steroid (betamethasone) was injected into right knee joint. After 1 month follow-up, we found that the pain and redness on his calf was clearly decreased. The patient’s pain visual analog scale (VAS) value was decreased from 8 to 2. We observed that the patient continued to improve at follow-up visit 3 months later; VAS value was 0 and there was no swelling or redness on his calf. |
A 47-year-old African American female with past medical history of hypertension was transferred from an outside hospital after presenting with left sided upper and lower extremity weakness., MRI brain was performed which demonstrated multiple ring-enhancing lesions on post contrast T1 images, the largest within the right basal ganglia and the pons. There was also a smaller lesion in the left medial occipital lobe (refer . The findings were concerning for possible demyelinating disease such as tumefactive multiple sclerosis, metastases or infection. A CT chest, abdomen, and pelvis revealed uterine masses likely representing uterine fibroids but otherwise no evidence of malignancy. Subsequently MRI of the pelvis was performed for further work up of the uterine masses which showed the large heterogeneous uterus was most likely due to degenerative fibroids and no suspicious uterine lesion was seen. Lumbar puncture was not performed due to concern for increased ICP. TTE was performed to rule out infective endocarditis and showed no evidence of valvular vegetations. Autoimmune and infectious serum panel were all negative.\nA brain biopsy was performed and final pathology showed necrotizing granulomas with associated calcifications consistent with NS. Biopsy stains for acid-fast and fungal (methenamine silver and PAS) organisms, with appropriate positive controls, were performed and were negative. A Congo red stain, with an appropriate positive control, was also performed and was negative. The patient was started on methylprednisolone 1 g daily for a total of five days. At the time of discharge the patient was switched to prednisone 60 mg daily for a total of 30 days and advised to follow up in the neurology clinic in four weeks after discharge. The patient reported an improvement in the left sided weakness on his follow-up visit. In addition to prednisone 60 mg daily, the patient was prescribed omeprazole 20 mg daily and advised to continue with home medications (refer ). |
Patient is a 24-year-old female student who aspired to work for animals. She was referred to a psychiatric clinic by her friend. She complained of mood swings and difficulty maintaining a routine. She had loss of appetite, increased need for sleep, and lack of motivation. She reported that she was missing her office and eventually quit her job. Her work was exhausting, and on weekends she slept for 12-14 hours to catch up. During college years, she also struggled with attending classes, and maintaining relationships was stressful. She felt that her personal space was taken over, and therefore she broke up with her boyfriend. During assessment she reported narrow interest in animals; she described different animals in a pedantic manner. She has been interested in animals since early childhood. She had no friends in childhood and interacted mainly with her older brothers’ friends. She struggled with sustaining friendships and did not interact with people much. She shared that she felt different from others and in a large group often struggled to interact with others. During assessment she reported that she imitates others’ accent, expressions, and way of talking to fit in during social interactions. She often ends up upsetting others as she comes across as too honest. She enjoyed spending time with animals more than meeting friends and prefers detailed instructions to complete a task. She had an aversion for tight jeans, wool sweaters, and wet socks. She consulted a psychologist once in the past and had no other relevant medical history. She scored 29.5 on CARS2-HF, signifying mild to moderate symptoms of autism spectrum disorder. She was receptive to the diagnosis of ASD and during psychoeducation sessions asked many questions pertinent to the presentation and diagnosis. She was recommended weekly psychotherapy sessions. During her one-month tele-psychiatry follow-up, she reported to have made clinical improvement with ongoing therapy and reading self-help books. |
A 38-year-old woman from Elazig, Turkey with atrial septal defect was admitted for exertional dyspnea and fatigue. She had a general tendency and swelling both of the lower limbs at term (38-40 weeks’ gestation). Echocardiography had documented atrial septal defect () and she had three children in alive. She had an intrauterine death at 28 weeks’ gestation.\nOn physical examination, she presented in both lower limbs swelling. A venous color flow Doppler ultrasonography of the both lower-extremity were requested, showing bilateral venous insufficiency, but there were not peripheral venous thrombosis. On auscultation, fixed split of second heart sound and pansystolic murmur on the left side of sternal border were heard. ECG was NSR. A chest X-ray showed mild heart enlargement. Echocardiography documented enlargement of the right ventricle and both atria with atrial septal defect (approximately 33 × 38 mm) and normal function of both ventricles (). Right-sided pulmonary veins were not identified. Multidetector Computerized Tomography (CT) angiography confirmed atrial septal defect and abnormal return of right-sided pulmonary veins into the right atrium () and showed inferior vena cava on the right side of spine and showed total interruption of IVC below the hepatic veins (). Multidetector CT angiography provided full description of a complex anomaly documenting right inferior vena cava with azygous continuation, draining into the superior vena cava (, ) and hepatic veins were draining directly into the right atrium (). Ultrasound revealed polysplenia (), 0.5 cm nodule in the left thyroid lobe and 1 cm cystic mass in the right over.\nA standard median sternotomy was performed. The pericardial cavity was opened and the heart was exposed. SVC was dissected out to the bridging vein, exposing the azygos vein and anomalous pulmonary veins. Cardiopulmonary bypass was established with cannulation to the ascending aorta and SVC was cannulated by L-shaped catheter, because of absence the IVC. The IVC cannula was not cannulated. After cross-clamping of the ascending aorta, cardiac arrest was obtained using St. Thomas solution through the root cannula under moderate hypothermia. A tourniquet was applied to the SVC and the anomaly was approached by a longitudinal right atriotomy parallel to the atrioventricular sulcus. We saw atrial septal defect and abnormal return of right-sided pulmonary veins into the right atrium (). We performed intraatrial rerouting and to drain the anomalous pulmonary veins into the left atrial cavity, using a patch made of a polytetrafluoroethylene (PTFE) in a patient with 5-0 polypropylene (). The suture line of the patch was placed away from the sinus node and the pulmonary veins. After the operation sinus rhythm was recovered. There were no complications with follow-up of one year. |
A 61-year-old woman with no past history of cardiopulmonary disease was diagnosed with chronic myeloid leukemia (CML) and initially started on Imatinib therapy. Four years later, a bone marrow biopsy showed progression of CML to precursor B-cell acute lymphoblastic leukemia (ALL) and the patient was switched to therapy with Dasatinib at a dose of 140 mg daily due to disease progression on Imatinib. At this time, the patient also received two cycles of chemotherapy with Cyclophosphamide, Vincristine, Adriamycin, and Dexamethasone. Following this, a repeat bone marrow aspirate showed complete hematologic, cytogenetic, and molecular remission and the patient was continued on Dasatinib therapy. However, 26 months later, the patient developed a persistent dry cough and severe dyspnea on exertion. Initial laboratory testing showed a white blood cell count of 3700, with 46% neutrophils and 45% lymphocytes. Blood cultures were negative for infection, and the patient did not produce adequate sputum for culture. A chest X-ray showed bilateral pleural effusions with cardiomegaly, suggestive of right ventricular failure (). A CT scan was performed, which noted an enlarged central pulmonary artery and bilateral pleural effusions; no other abnormalities concerning the lung parenchyma, pulmonary veins, or interstitium were reported. No pulmonary embolism was seen. An echocardiogram confirmed the presence of a dilated and hypokinetic right ventricle and a dilated right atrium ( and ). Other potential causes of pulmonary hypertension (PH) were excluded by a negative V/Q scan and a review of past records showing normal echocardiographic testing one year prior. A right heart catheterization (RHC) was performed with a mean pulmonary artery pressure (mPAP) of 54 mmHg, pulmonary capillary wedge pressure (PCWP) of 11 mmHg, and pulmonary vascular resistance (PVR) of 18.22 Woods Units, yielding a diagnosis of pulmonary arterial hypertension (PAH). The right atrial (RA) pressure was 19 mmHg, and the cardiac output (CO) by the Fick method was 2.36 L/min (Cardiac index of 1.46 L/min/m2). Vasoreactivity testing was not performed. In clinic follow-up one month after diagnosis, a D-dimer test was done and found to be elevated at 1.73 mg/L (reference = 0–0.5 mg/L); however, a polymerase chain reaction (PCR) test for the BCR-ABL gene was < 0.001% at that time, consistent with her levels while in remission. A complete autoantibody screen was also done and found to be negative (antinuclear antibody, anti-double strand DNA, anti-Smith, ANCA, ribonucleoprotein, anti-histone, SCL-70, chromatin, centromere, SSA/SSB). Her BNP was noted to be 560 pg/mL (reference = 0–100 pg/mL), her 6-minute walk test (6MWT) was 250 m using 2 L oxygen via nasal cannula with a nadir pulse oximetry saturation of 96%, and her functional class was WHO-FC III. Dasatinib was suspected to be the precipitating factor for the patient’s new PAH given the temporal association with the patient’s symptoms and exclusion of other etiologies, and was promptly replaced with Nilotinib (a related second-generation tyrosine kinase inhibitor with a lower rate of drug-induced PAH compared with Dasatinib) to continue therapy for CML. Given the severity of the patient’s symptoms and RHC data, the patient was also started on combination therapy with a phosphodiesterase-5 inhibitor, Tadalafil, at 20 mg daily and an endothelin receptor antagonist, Ambrisentan, at 5 mg daily. The patient was also started on furosemide at 10 mg daily and was not started on systemic anticoagulation. The Tadalafil was up-titrated over a period of four weeks to 40 mg daily, followed by an up-titration of Ambrisentan to 10 mg daily over the following four weeks. The patient experienced marked symptomatic improvement, and repeat chest X-ray () and echocardiogram four months after beginning therapy showed complete resolution of the PH with a normal right ventricular size and function ( and ). A repeat RHC performed at the same time showed full resolution of PAH, with mPAP of 18 mmHg, PCWP of 9 mmHg, and PVR of 1.96 Woods Units. The RA pressure was 5 mmHg and the CO by the Fick method was 4.59 L/min (cardiac index of 2.51 L/min/m2). Her BNP was measured in clinic at this time and had decreased to 18 pg/mL, her 6MWT had improved to 425 m on room air with a nadir pulse oximetry saturation of 96%, and her functional class was WHO-FC I. Given the marked improvement both symptomatically and hemodynamically, Ambrisentan and furosemide were discontinued and Tadalafil was weaned sequentially over the following four months. Despite the cessation of her pulmonary vasodilator therapy, the patient did not have a return of her dyspnea or exercise intolerance. She continues to remain symptom-free after five months without targeted PH therapy. |
A 63-year-old woman presented to the emergency room with lower abdominal pain associated with nausea and vomiting for 3 days. She described the pain as dull, involving the right lower quadrant, non-radiating and constant. She also reported multiple episodes of non-bloody and nonbilious vomiting. Her prior medical problems included osteoarthritis requiring right-sided total knee replacement 5 years prior to this episode. She had been taking ibuprofen 400 mg for knee pain on as needed basis for the past 8 years. She had no family history of inflammatory bowel disease, colon cancer or any other GI-related disorder. She denied smoking, using alcohol and she reported no illicit drug use. She did not have any known drug allergies.\nOn initial examination in the emergency department, she was afebrile, hemodynamically stable and was not in any distress. Physical examination, including the abdominal examination, was unremarkable. Her laboratory investigations did not reveal any leukocytosis, but CRP was elevated to 55 mg/L. Computed tomography (CT) of the abdomen with intravenous contrast (Fig. ) showed focal mucosal thickening in the cecum which was highly suspicious for colonic malignancy. Subsequently, the patient underwent a colonoscopy which showed a frond-like/villous, infiltrative, ulcerated, large partially obstructing mass in the ascending colon in close proximity to the cecum (about 4 cm from the cecum) (Fig. ) and a large, ulcerated mass in the cecum. Biopsy samples were taken from both masses and pathology showed fragments of colonic mucosa with focal minimal hyperplastic changes, ulceration, mild acute and chronic inflammation and no malignancy. She was evaluated by both surgery and oncology and gastroenterology recommended to repeat colonoscopy with biopsy, but she refused and proceeded with right hemicolectomy, due to a suspicion of colonic malignancy in view of a colonic mass. Examination of the specimen after surgery showed an ascending colon mass measuring 3.2 × 2.5 cm and a cecum mass measuring 2 cm × 1.5 cm. Histopathology showed fibrinosuppurative exudate, granulation tissue and fibrosis with reactive changes. There was no evidence of malignant inflammatory bowel disease, ischemic colitis, granuloma, viral inclusion body or parasite. In view of isolated colonic ulcers in the setting of NSAID use, NSAID-induced colopathy was suggested. The patient was instructed to avoid NSAIDs. |
This is a report on a 32-year-old man with a history of two previous melanomas with concurrent plaque-type psoriasis. His history dates to June 2009, when he developed his first melanoma on the right occiput, Clark's level IV, tumor thickness 1.53 mm, nonulcerated, mitotic index 1/mm2. He was treated with wide local excision and sentinel lymph node biopsy in June 2009, the latter being negative for nodal metastasis. In November 2009 he presented with a 1-cm blue to black nodule on the right posterior neck, which on complete lymph node biopsy demonstrated nodal metastasis, prompting a complete lymph node dissection in December 2009, during which time 46 lymph nodes were removed, all of which were negative for any additional metastasis. He developed a second primary melanoma on the right upper chest in September 2012, Clark's level IV, tumor thickness 0.9 mm, nonulcerated, mitotic index 3/mm2 that was similarly treated with wide local excision and sentinel lymph node biopsy in November 2012; this too was negative for nodal metastasis.\nThe patient has a longstanding history of concurrent psoriasis since childhood. He has a strong family history of psoriasis in his sister, father, and maternal grandmother. Despite being of moderate severity, the psoriasis has been largely managed topically with various topical steroids and calcipotriol with betamethasone dipropionate. He had, at one point, a course of acitretin which was ineffective. He never received methotrexate, cyclosporine, or any of the biologics. After being disease-free from his melanoma for more than 3 years, the patient requested a more aggressive therapy for his psoriasis. The safest drug in the context of stable stage IIIB melanoma was thought to be apremilast which he started in August 2015 (30 mg twice a day after initial dose escalation as per drug protocol). At the time of initiation of therapy, he had numerous 2–5-cm plaques on the elbows, knees, thighs, and trunk (PASI 13.1). The patient had a dramatic response to his treatment, and by November 2015 he had just a few scaly pink papules <1 cm on the arms, lower limbs, and torso. Within 4 months of starting therapy, the patient had noted a “new” blue 5-mm papule on the left shoulder, which clinically and on dermoscopy was thought to represent a blue nevus. He returned for follow-up in March 2016 with two new 1–2-mm blue-black papules near the site of his first melanoma on the scalp. Subsequent biopsy confirmed recurrence of his melanoma in the form of satellitosis; molecular testing on this specimen detected the BRAF V600E mutation. He went on to develop multiple additional papules, primarily on the trunk and upper limbs (7 in total), which on biopsy were in keeping with cutaneous metastasis (stage IVa) (Fig. ). Screening for systemic progression was negative. The patient discontinued apremilast as there was concern that his tumor had recurred because of the drug. He was placed on anti-PD1 therapy with pembrolizumab, which had to be discontinued due to severe liver toxicity and markedly elevated creatine phosphokinase. He is currently being treated with dabrafenib and trametinib. As the patient was started on systemic therapy promptly after being diagnosed with metastatic disease, we cannot comment whether the discontinuation of apremilast resulted in any regression of his cutaneous metastases. |
A 65-year-old male with diabetes mellitus was referred to our hospital for the management of urethral stricture disease. He had a history of voiding lower urinary tract symptoms for 6 months and subsequently developed periurethral abscess, went into acute urinary retention, and developed Fournier's gangrene. He was treated at that time with suprapubic cystostomy and debridement of the penile, scrotal, and some parts of perineal gangrenous skin and skin grafting of these areas. He presented to us 6 months later for urethral reconstruction. Local examination at presentation revealed meatal stenosis with balanitis xerotica obliterans changes and absent prepucial skin. The whole of the penile and scrotal skin had been replaced by adherent grafted skin. His testes were pulled up and lying at the superficial inguinal region due to scars and contractures []. Antegrade and retrograde urethrogram revealed extensive areas of narrowing in the penile and bulbar urethral regions []. Because of the complex urethral stricture, he was given the options of two-staged urethroplasty or a permanent perineal urethrostomy, seeing his age and comorbidities. The patient refused for the two-staged procedure and underwent a perineal urethrostomy fashioned with the help of an inverted “U-shaped” perineal skin flap and bilateral KDPIF. The procedure was performed under anesthesia with the patient placed in lithotomy position. Bulbar urethra was exposed with an inverted U-shaped perineal flap. The keystone flaps were designed with perpendicular incisions made at either angle of the lateral defects meeting the curvilinear incision marked parallel to the defects at a distance approximately twice the size of defects. Flaps were subsequently mobilized with the underlying subcutaneous tissue over the fascia and sutured into the original defect. The donor area was closed primarily over drains [ and ]. The patient recovered uneventfully, and he voided well when the catheter was removed on the 7th postoperative day. Uroflowmetry showed a smooth bell-shaped curve with wave of 12 ml/s. He was satisfied with the outcome of his treatment. |
A 42-year-old female presented with Hunt and Hess grade IV subarachnoid hemorrhage (SAH), which improved to grade III after placement of a ventriculostomy. Computed tomography (CT) and CT angiography revealed a diffuse SAH and ruptured basilar tip aneurysm []. Digital subtraction angiography (DSA) confirmed the diagnosis of a basilar bifurcation aneurysm with a wide neck and shallow dome []. Due to its unfavorable neck/dome ratio, endovascular obliteration of this aneurysm was thought to be high risk. The basilar bifurcation was noted to be quite high in relation to the dorsum sella []. A right cranio-orbital approach was used for the clipping of the aneurysm in this patient. After wide opening of the Sylvian fissure and arachnoid cisterns, the PComA, and the P1 and P2 segments of the PCA were isolated. Due to the high riding basilar bifurcation, dissection of the basilar bifurcation was restricted by the PComA, which was tethering the PCA. Because the PComA was not a fetal type, the decision was made to divide the PComA. However, before dividing it in its perforator-free segment, we performed ICG-VA with a temporary clip on the perforator-free segment, and demonstrated that both perforators were filling from both the PCA and the internal carotid artery (ICA). The PComA was divided between the proximal and distal perforators. Another ICG-VA was performed and this showed that the perforators were still filling. The aneurysm was then clipped (see video, , which demonstrates the surgery, 3 min 18 s, and 71.8 MB). Postoperatively the patient's clinical status improved and she was extubated on the 7th postoperative day with intact speech and was able to follow verbal commands. A postoperative angiogram showed the total obliteration of the aneurysm []. Two days after the removal of the external ventricular drainage, the patient suffered a rapid decline because of right intraventricular hemorrhage. Emergency evacuation of the hematoma was performed. She eventually made a good recovery and was discharged to a rehabilitation center. On postoperative 3rd month follow-up she was neurologically intact. |
The patient was a female aged 48 years old, who had a lumbar mass on her right lower back for 4 years, and was admitted to hospital more than a year after noticing the enlargement of the mass. The mass on the right lower back was unintentionally discovered 4 years earlier and was approximately 2 × 2 cm in size. The patient did not experience any discomfort such as chills, fever, or local pain. The mass began to enlarge 1 year earlier without discomfort. The past surgical history included total hysterectomy in 2013. On examination, a cystic mass of about 5 × 5 cm was observed in the right lower back, with clear borders, smooth surface, hard on palpation, no pressure pain, and no mobility. Laboratory examinations showed decreased hemoglobin levels of 102 g/L (normal value: 130–175 g/L). Liver function, renal function, AFP, CEA, and CA199 were normal. Color Doppler ultrasonography showed mixed echoes on the right lower back. Plain CT scans showed irregular low-density cystic masses on the right retroperitoneum and on the soft muscles of the back. The size was about 10.1 × 8.5 cm. The borders were unclear and the density was irregular. The solid region of enhanced scans showed uneven strengthening, with the mural wall showing nodular strengthening (Figs. and ). Plain MRI scans showed mixed-signal cystic solid masses in the right retroperitoneum and lower back, with necrotic bleeding, and cystic changes in the masses. T1WI showed equal or slightly lower signals, with T2WI showing high signals. The cyst wall, partition, and mural nodules were significantly strengthened, and the right kidney and liver moved forward under pressure. The boundaries with subcutaneous and surrounding muscles were unclear (Figs. –).\nBiopsy pathology prompted the diagnosis of a solid-pseudopapillary tumor, and surgical treatment was provided. Intraoperatively, a giant cystic solid tumor was observed behind the right retroperitoneum, located extremely behind the right kidney below the liver. The tumor pushed the right kidney forward and medially, and the medial side was adjacent to the inferior vena cava, the right adrenal gland, and the right diaphragm. The dorsal base of the tumor invaded the lower back muscles. The tumor, right adrenal gland, part of the diaphragm and lower back muscles were removed. The tumor had a positive resected margin, and the tumor invaded the fascia. The size of the specimen was about 10 × 8 × 5 cm. Histopathology showed tumor cells arranged in a papillary shape under a light microscopy. The cells were round or oval, the nuclei were round, and the chromatin was coarse-grained. The nucleoli were obvious and mitotic cells were abundant (Fig. ). Immunohistochemistry findings: Vimentin(+), S-100(+), CD68(+), CD163(+), CD1a(−), CD21(−), β-catenin(cytoplasm+), ER(−), PR(weak+), Fli(−), CD99(−), Catenin-b(−), CD10(−), CK(AE1/AE3)( −), cyclin D1(−), Galectin-3(−), EMA (weak +), Ki-67 (+, about 10%), the cell atypia was obvious. Electron microscopy was not performed due to limited conditions. IDCS was diagnosed based on cell morphology and immunohistochemical examination (Figs. –). There was no tumor involvement in the right adrenal gland. The patient received adjuvant radiotherapy for 2 months after the surgery. The radiotherapy dose was DT 50GY/25F. After radiotherapy, the patient's white blood cells were reduced to 2.13 × 109/L. No obvious tumor recurrence or metastatic signs were observed in the abdomen during 4 months of follow-up. Chest CT showed multiple metastases in both lungs 12 months after radiotherapy. He was given doxorubicin + ifosfamide combined chemotherapy and is still under follow-up. |
A 38-year-old woman with a 3-year history of cholecystolithiasis was examined at a local hospital and referred to our hospital for endoscopic gallstone removal without gallbladder excision. When she was hospitalized at our hospital, the acute right upper abdominal pain had persisted for two hours.\nOn physical examination, no signs of jaundice were seen in the skin or sclera. The patient's abdomen was soft, with no sign of lumps, with tenderness other than rebound tenderness in the gallbladder area. Murphy's sign was positive. Ultrasonography revealed several movable masses of 7 × 10 mm and 7 × 9 mm in size, which were strongly echogenic, with acoustic shadowing in the body of the gallbladder; an immovable strongly echogenic mass of 7 × 10 mm with acoustic shadowing in the neck of gallbladder and cystic duct; and poor sound penetration and a dense, low-light spot in the dark space of the bile. There was no thickening of the gallbladder wall. The gallbladder emptying index was 30%. No obvious abnormalities were seen in the liver, spleen or pancreas.\nThe patient was diagnosed as having cholecystolithiasis complicating acute cholecystitis and incomplete biliary-tract obstruction. The patient had been given anti-inflammatory treatment after the examination for 2 weeks and the symptoms of acute cholecystitis had disappeared before the operation was schedule, and the patient was strongly in favor of preserving the gallbladder.\nConsequently, after approval by the medical ethics committee and provision of informed consent by the patient, the gallbladder was laparoscopically isolated and transected at the bottom (< 6 mm) under general anesthesia. First, the bile was drained with a sterile ventricular drainage tube to a sterile injector, and transferred to sterile tubes. Next, the gallbladder was explored with a three-channeled cholecystoscope (CHiAO; Chinese national patent number: ZL200810026985.X HAWK, China []), and the stones were collected with a stone extractor. We found only mild congestion in the gallbladder mucosa, which indicated only slight inflammation of gallbladder. Using endoscopy, we found many small, semitransparent stones (< 1 mm) adhered to the gallbladder wall (Figure ). These stones were removed with an endoscopic attachment (CHiAO absorbing box; Chinese national patent number: ZL 201110167069.X) designed to remove sludge-like gallstones combined with seven types of manipulation (pushing, squeezing, pressuring, tearing, bracing, flushing, and sucking) [], while several large stones (> 5 mm) were removed with a stone extractor (Figure ). The small stones were yellowish and semitransparent, and the large stones were polyhedron or globular in shape, and had a radial, layered arrangement in profile (Figure ). The bile was yellowish, opaque, turbid, and very viscous. After centrifugation at 1,450 g for 10 min, the bile supernatant was transferred to a clean tube, and about 0.5 mL of sediment was kept. The bile sediment was smeared onto labeled slides and viewed under a system microscope (BX51; Olympus, Tokyo, Japan). Colorless hexagonal plate crystals with high refractivity and limpid edges were seen, which were judged to be cystine crystals based on their morphology. Meanwhile, colorless, transparent crystals (in the shape of rectangles with missing corners or squares with missing corners, or glass flakes) were seen, which were judged to be cholesterol crystals from their morphology (Figure ).\nThe gallbladder stones were analyzed with a Fourier transform infrared spectrometer (TENSOR27; Bruker Optics GmBH, Ettlingen, Germany) in the frequency range of 400 to 4,000 per cm at 4 per cm resolution. The results indicated that the small stones were cystine stones and that the large stones were cholesterol stones; (Figure ).\nNext, some of the small stones and a piece of a large stone were fixed onto the sample table and dried at 60°C overnight, then sputter-coated with gold (ETD-2000, Beijing Elaborate Technology Development Ltd., China) and observed under a scanning electron microscope (EVO LS10; Carl Zeiss, Cambridge, England). The samples were photographed and analyzed with an energy spectrometer (X-Max; Oxford Instruments plc, Oxford, UK). Under scanning electron microscopy, the small stones were found to be composed of hexagonal cystine crystals (30-270 μm), some with prominences on their edges. The energy spectrum indicated that the elemental composition was carbon, oxygen, nitrogen, and sulfur, along with gold from the coating. The large stones were composed of lamellar cholesterol crystals with a thickness of about 1 μm. The energy spectrum indicated that the elemental composition was carbon and oxygen, with gold from the coating (Figure ). |
This 65-year-old woman had exhibited progressive gait disturbance over a one year period. For 15 years, her son had been in a vegetative state due to head trauma, and she had taken care of him. That had been a source of enormous stress for her. She was diagnosed with depression and had been taking antidepressants for five years. Last year, she felt that her legs dragged and were slow. These symptoms progressed slowly, despite intermittent fluctuation. When she presented in our clinic, she exhibited depressive mood; however, she denied any autonomic symptoms, including postural hypotension, urinary frequency, or constipation.\nOn neurological examination, she exhibited a mild masked face. Although the speed and amplitude of foot tapping were within normal limits, the amplitude of finger tapping decreased, additionally, speed slowed bilaterally. The motor power of her four extremities showed no weakness and there were no abnormalities on sensory examination. Deep tendon reflex was also normal. During walking, both of her feet dragged and were slowed with a bilateral decrease in arm swing. Sometimes, she hesitated to move exhibiting a glue feature while turning.\nThe findings of laboratory studies were unremarkable. A magnetic resonance imaging scan of her brain showed no abnormal findings. Interestingly, the patient reported that she could relieve her symptoms at will. When she concentrated hard on her gait or when she touched her hand to the posterior of her neck, her dragged and slow gait improved dramatically. Also, her abnormal gait improved when the examiner touched his hand to her neck (see ). Based on these curious conditions, we gave her placebo injections along with suggestion. However, there was no symptomatic change following this procedure. Subsequently, she was taken for an Fluoropropyl carbomethoxy-3b-(4-iodophenyltropane) ([18F]-FPCIT) positron emission tomography (PET) scan for evaluation of basal ganglia lesions. Results revealed a reduction in putaminal uptake bilaterally as compared to normal controls (). She was then diagnosed with PD, levodopa treatment was initiated. After two months of levodopa treatment, her bradykinesia on both fingers had improved; however, her abnormal gait had not improved markedly. Informed, written consent was obtained after the patient had been given a complete description of the study using a . |
A 36-year-old smoking woman (16 pack years) with a past medical history of three pregnancies, appendectomy and cholecystectomy, referred to her physician with symptoms remembering her previous pregnancies. She was under birth control pill but presented with a 7-week amenorrhea and a β-hCG level of 850 UI. Her body mass index was of 18 and the physical examination revelaed breast enlargement. A pelvic ultrasound exploration showed a uterine vacuity with a left lateral uterine heterogeneous mass. Even with no bleeding and no pain, ectopic pregnancy was suspected. She was referred to a first gynecologic center where she underwent surgical treatment. Laparoscopic exploration detected a 4 cm left ovarian cyst, a voluminous hematoma of the left broad ligament of uterus nearby the left suspensory ligament, and voluminous left pelvic varicose veins. The hematoma was spreading along the left salpinx. To rule out ectopic pregnancy and as the hematoma was extensive, left adnexectomy followed by uterine curettage were performed. Despite surgical treatment, β-hCG increased from 850 to 900 UI, therefore medical treatment by methotrexate was prescribed.\nPathologic examination found no signs of ectopic pregnancy or malignancy.\nDespite methotrexate treatment, β-hCG still increased to 1,335 UI two weeks later. She was referred to our center from this moment. An extragonadal germ cell tumor was suspected and a computed tomography (CT) scan was performed. It showed a voluminous anterior mediastinal tumor with no radiological compressive signs and no other extension site. αFP and angiotensin- converting enzyme were negative. The review of the chest X-ray showed an evident anterior mediastinal tumor ().\nAfter multidisciplinary agreement, biopsy through the mini-invasive right anterior video assisted thoracotomy was performed for neo-adjuvant chemotherapy adaptation to histological proof. Pathologic examination concluded a NSGCT with choriocarcinoma characteristics and reported positivity of β-hCG and placental alkaline phosphatase.\nFirst line chemotherapy was based on etoposide, ifosfamide and cisplatin (VIP). In the meantime, we completed explorations with bone scintigraphy and cerebral scan. These detected no secondary localizations. Serum tumor markers were unchanged. Compliance to the four cycles of VIP was good under cover of antiemetic treatment. However, evaluation after four cycles of chemotherapy showed progression with appearance of liver metastasis in spite of decrease in β-hCG level (830 UI) and mediastinal mass volume to 30% of its previous volume. A second line salvage chemotherapy based on taxol and carboplatin was delivered. The tumor continued to aggressively metastasize throughout brain and bone. The patient died 16 months after her initial presentation as ectopic pregnancy (12 months after applying first line chemotherapy). |
A 67-year-old white man with a history of hypercholesterolemia, morbid obesity, insulin dependent diabetes, and prostate cancer presented in 2007 with a T2N0M0 squamous carcinoma of the left glottic larynx. Treatment was primary chemoradiotherapy. A local recurrence developed in 2009, which was treated with a left type III, modified radical neck dissection, left tracheoesophageal groove dissection, hemithyroidectomy, laryngectomy, and tracheoesophageal puncture. The postoperative course was complicated by a pharyngocutaneous fistula, which resolved with conservative management. In 2011, a regional recurrence of squamous cell carcinoma developed in the right paratracheal groove with overlying skin involvement. A right radical neck dissection, parastomal skin resection, and partial pharyngectomy was performed. Immediate reconstruction consisted of a right pectoralis major myocutaneous flap to close the hypopharynx defect with split-thickness skin grafting externally. The postoperative course was complicated by a pharyngocutaneous fistula at the superior margin of the permanent tracheostoma (). Reirradiation was performed perioperatively. Following 8 months of conservative treatment, the patient requested closure of the persistent fistula.\nBased on previous reports demonstrating recovery of radiated tissue with autologous fat grafting, fat transfer was performed in the current patient to improve tissue quality in preparation for flap transfer or as a primary means of achieving closure. Using hand-assisted liposuction techniques, autologous fat was harvested from the patient's abdomen and purified using Telfa rolling. Approximately 8 mL of purified fat were injected into the area immediately surrounding the pharyngocutaneous fistula using both blunt cannulas and 18 gauge needles (). The tissues were markedly fibrotic; however, use of the sharp needle was helpful in fat transfer. After 3 months of conservative measures (ongoing gastrostomy tube feeds and local wound care), the fistula size reduced by approximately 70% (). The quality of the tissues surrounding the fistula improved becoming softer and less fibrotic. Encouraged by these results, a second fat grafting procedure was performed. The edges of the wound were debrided to remove the epithelialized tract and approximately 6 cc of purified lipoaspirate was injected in the tissues surrounding the defect using blunt and sharp needles. One month following the second procedure, the fistula closed spontaneously. It has remained closed 1 year later with the patient tolerating a regular diet (). |
A 39-year-old female patient was admitted to hospital with epigastric pain which was lasting for two months. She had no remarkable history except for her mother having DM. Moreover, a physical examination revealed epigastric tenderness on palpation, and laboratory examinations revealed no important findings. Endoscopy revealed edematous and fragile papilla of Vater, and a polyp was seen on the 2nd part of duodenum. A biopsy yielded a hyperplastic polyp, and a biopsy from the papilla was performed due to the imaging findings on endoscopy with suspicion of an ampulla tumor; however, biopsy specimens yielded no malignant cell.\nUltrasonography (US) of the patient showed a diffusely enlarged hypoechoic pancreas with punctate hyperechogenities representing calcifications (). There was no altered vascularization of pancreatic parenchyma on Doppler US. Computed tomography (CT) revealed a huge pancreas, which was enlarged diffusely with multiple calcifications and punctate hyperdensities. The pancreatic duct and peripancreatic fat tissue were normal. There was no inflammation sign (). CT of the thorax did not yield an important feature in the mediastinum and lung parenchyma. On magnetic resonance imaging (MRI), a diffusely enlarged pancreas was seen as hypointense on both T1- and T2-weighted images (). There were hyperintense cystic nodules in pancreas parenchyma on T2-weighted images (). After gadolinium administration, enhancement of the pancreas began in the early phase, and became more prominent at delayed images. Punctate nodular unenhanced areas in pancreas parenchyma were seen on delayed images. Since the patient's history revealed no sign for chronic pancreatitis and amylase levels were normal, a trucut biopsy with an 18 G needle (Bard Biopsy Systems, Tempe, AZ) was performed. According to the histopathological results, the diagnosis was islet cell primary amyloidosis of the pancreas. Only islet cells in the islands of Langerhans were stained red with congo red ink. Exocrine cells of the pancreas were spared (). Fibrosis across the islet cell islands accompanied amyloidosis. The diagnosis of primary amyloidosis was established due to a color change of amyloid deposits after permanganate administration, which is not seen in secondary amyloid deposits. An oral glucose tolerance test revealed impaired glucose tolerance with increased glucose levels (160 mg/dL) during the 2nd hour after oral glucose administration, which was attributed to impaired glucose tolerance. |
A 42-year-old female presented with involuntary paroxysmal spasms of the left masticatory muscle, of 6 months duration. The spasms were mild in the early stage; however, they increased gradually in duration and frequency. The spasms were frequently painful and sometimes awoke her from sleep. The lower half of the face on the left side hypertrophied over time. She had no specific medical diseases.\nOn admission, her masseter muscle on the left was markedly hypertrophied. However, neither FHA nor a skin lesion was noticed. The spasms usually continued for about 10 seconds, but sometimes lasted for up to a few minutes. Neurologic examinations, including masticatory muscle function and jaw jerk, were normal. Hematological, biochemical, and serologic tests, including antinuclear antibody, rheumatoid factor, anti-DNA antibody, and C-reactive protein, were normal. The blink reflex was normal on an electrophysiological study. Masseter reflexes elicited by tapping her chin and recorded by surface electrodes from the masseter muscle showed delayed latencies and decreased amplitudes on the left side during the spasm episodes (). Moreover, the masseteric silent period was attenuated on the affected side (). Surface electromyography demonstrated irregular bursts of motor unit potentials at 50 to 200 Hz during the period of involuntary spasm or at random intervals (). Magnetic resonance imaging of the head showed pronounced hypertrophy of the left masseter muscle (). A muscle biopsy done at the central region of the hypertrophied muscle revealed nonspecific results.\nThe frequency of the spasm and pain improved slightly after treatment with phenytoin. Several local injections of 20–50 units of botulinum toxin in the left masseter muscle led to marked improvement of her symptoms. After 3 months, the hypertrophy of the masseter muscle was substantially decreased (). The benefits of the botulinum toxin injections persisted for 4 months, and subsequent injections were performed with equal success. The follow-up electrophysiological profiles were also improved. |
A 62-year-old woman was referred to the Department of Surgical Sciences, University of Insubria, Varese, for left flank pain. Physical examination was unremarkable. Complete blood cell count, electrolytes, eosinophil count, serum biochemistry and urinalysis were within normal limits. Plain radiographs of the abdomen showed a small round opacity, about 3-cm in diameter, in the left upper region of the abdomen, with evidence of calcifications. Chest X-ray was normal. The patient underwent ultrasound examination (US) that showed a calcified heterogeneous cystic mass between the superior left kidney pole and spleen in the retroperitoneal area. CT scanning demonstrated the presence of a solitary cystic mass with a calcified wall within the left adrenal gland with no enhancement after iv contrast media injection. No other intra-abdominal or intra-thoracic masses were found, either in the liver, peritoneum or lung. MRI T2-weighted imaging showed a hypointensive lesion with a calcified wall, and a peripheral hyperintensive nodule (Figure , ). CT and MRI findings suggested adrenal echinococcosis. The specific serology for immunoglobulin anti-E. granulosus resulted positive 1:61 (n.v. < 50). Urinary catecholamines and metanephrine levels were within normal limits. The patient confirmed that she had had animal contact. The albendazole preoperative therapy resulted in the disappareance of pain. The diagnosis was confirmed by surgery. The cyst was removed by a transabdominal laparoscopic approach. The exploration of the rest of the peritoneal cavity did not reveal any other lesions. The area around the cyst was carefully packed with gauze soaked in 20% hypertonic saline solution. The cyst was not drained nor aspirated during the procedure. An appropriate dissection plane between the cyst and the adrenal gland could not be found as it was hard and adhesive, so both the cyst and the left adrenal gland were endoscopically removed. Pathological examination of the specimen confirmed that it was a hydatid cyst of the adrenal gland. Macroscopic pathologic examination showed a circumscribed unilocular cystic lesion 3-cm in diameter with a thick fibrous wall of 2-mm. It weighed about 57 grams. On palpation there were stone hard areas of calcification within the cyst wall. Examination of the contents of the cyst showed the typical clear fluid of a hydatid cyst with sand pasty material and calcified bodies. Moreover necrotic material with protoscolices was present (Figure ). No protein was detected and the glucose level was 72 mg/dl. The patient's postoperative course was uneventful. The patient was discharged on the third postoperative day. Therapy with albendazole was administrated for the 4 weeks following surgery (30 mg/kg/day orally). Six months later, the patient is well and in good condition with no further symptoms. |
Ms AB, a 31 year old lady known to be suffering from personality disorder complained of troublesome voices off and on for the last few years. More recently she felt that these voices were telling her to self-harm. These were presumed to be dissociative in nature and no antipsychotics had been prescribed consistently. We added quetiapine 50 mg at night with a plan to increase the dose gradually. However, an increase to 100 mg caused drowsiness and the patient felt unable to increase the dose further. She also complained of muscle spasms which improved on procyclidine. Ten days after the increase to 100 mg, the client complained of a feeling of heaviness in her breasts and later of galactorrhoea. A serum prolactin estimation (17 days on 100 mg) showed raised levels (603 mu/l; N = 102–496). A retrospective medicine chart review showed only two extra doses of 50 mg having been used in the last week before the estimation.\nI investigated a number of other potential reasons for elevation of prolactin and consequent galactorrhoea. Thyroid stimulating hormone (TSH) level was within normal limits. A brain scan was not performed, as the elevation was less than two times the upper limit of normal, which made it unlikely to be a pituitary tumour. Other medications that the client was on included divalproex sodium 1250 mg, gabapentin 900 mg and venlafaxine 150 mg. These were on similar doses before the quetiapine was started and after the quetiapine was stopped. At the time of the second prolactin estimation, these were still continuing with no symptoms and the prolactin levels were normal.\nThe quetiapine dose was reduced to 50 mg and then stopped. The galactorrhoea subsided in three days and the heaviness in breasts remitted in seven days.\nA repeat serum prolactin estimation two months after stopping the quetiapine showed normal levels (359 mu/l; N = 102–496). No other antipsychotic was tried. |
A 29-year-old Moroccan man without medical history, had a five pack-years smoking history and stopped smoking seven years ago. He presented with a four-month history of chest pain and minimal hemoptysis. The physical exam was normal. A chest X-ray (Figure ) showed a large left well- rounded pulmonary opacity.\nA chest computed tomography (CT) scan showed a tumoral lesion at the left upper lobe measuring 12.5 × 8 cm. This process was well limited, heterogeneous containing calcifications and areas of necrosis extended into the chest wall without bone lysis or lymph nodes (Figure A) staged T3N0.\nBone scintigraphy, cerebral and abdominal CT scan showed no evidence of distant metastasis. Trans-thoracic biopsy was performed, histopathological study of the biopsy specimen showed a numerous glands showing an endometrioid morphology, occasional squamoid morules and relatively clear cytoplasm, the Ki-67 was 1% (low proliferation index) and based on these histopathological findings, the diagnosis of fetal adenocarcinoma was concluded (Figure ), then the patient received neoadjuvant CMT based on etoposide at dose of 120 mg/m2 day one to three and cisplatin at 100 mg/m2 on day one every three weeks. Evaluation after three cycles showed disease stabilization according to Response Evaluation Criteria In Solid Tumors (RECIST) (as immunohistochemistry was unavailable).\nThirty days after the last cycle of CMT the patient underwent a left upper lobectomy with lymphadenectomy (Figure ). The microscopic examination of the specimen found a tumor characterized by two component lesions, the first composed with well-differentiated elements of glandular proliferation rarely isolated or grouped in clusters (polyadenoid structures), which were sometimes separated by undifferentiated beaches of cells with blastomatous differentiation showing moderate cytonuclear atypia and a high mitotic activity. The tumor resection was complete with negative lymph node. The postoperative course was without anomalies.\nThe patient underwent a chest CT scan assessment every 3 months during the first year and then every 6 months during the second year. At 2 years of follow-up, there is no evidence of disease recurrence (Figure B). |
A 9-month-old male infant presented with urinary retention. The parents also gave history of constipation and passing of small amount of urine per void since the last three months. Abdominal examination revealed distended bladder. Child was catheterized and enema was given for rectal evacuation. Following bladder decompression a smooth, firm lump of approximately 7 cm X 5 cm was palpable in the hypogastrium. Rectal examination revealed a firm lump which was palpable anteriorly. Ultrasound showed an anechoic cystic lesion posterior to the urinary bladder with septations within it. CECT abdomen revealed a 7 cm X 3 cm X 2.5 cm well defined elongated fluid attenuating hypodense cyst in the recto-vesical pouch. There were no calcifications within the mass. The mass was causing compression on the bladder anteriorly and the rectum and sigmoid colon posteriorly. The urinary bladder showed diffuse wall thickening.\nThe child was taken up for cystoscopy and laparoscopic cyst excision. Cystoscopy did not show any communication of the cyst with the posterior urethra. The bladder was found to be trabeculated. Laparoscopy showed hypertrophied bladder and thick walled cystic swelling posterior to bladder and anterior to rectum compressing both.[Fig. 1 and 2] The cyst was densely adhered to rectum and bladder. Adhesiolysis was difficult laparoscopically and hence open deroofing of the cyst was done. Both vas deferens were identified and saved. Majority of the cyst was excised and the remaining cyst cavity mucosa was cauterized. A drain was placed in rectovesical space. Postoperatively, the child improved and was discharged. The fluid cytology revealed few inflammatory cells in a proteinous background. Histopathological analysis of the excised wall showed largely ulcerated areas with focal areas showing intact mucosa formed by cuboidal epithelium consistent with the diagnosis of Mullerian duct cyst. The child presented for follow up in outpatient department after three months and was doing well without any symptoms. |
I report the case of a 32-year-old Ethiopian male patient from Addis Ababa who presented with lower abdominal pain of three months’ duration.\nThe abdominal pain started after our patient inserted metallic and wooden materials via a self-inflicted stab wound from a rusted knife into his left lower abdomen, 3 months prior to his presentation to our hospital. He stated that he did this to commit suicide. He did not seek any medical attention at that time because he had no family around and lived on the street. He claimed that he did not take any medication for the pain or the wound.\nOur patient was taken to a clinic run by Missionaries of Charity in Addis Ababa by police after they saw the wound. He was then referred to our hospital for better management. Three months earlier, he had visited a psychiatric hospital for his psychiatric problems. He had not received any treatment because he had been asked to bring a relative with him to make sure he took his medications properly. He claimed that he occasionally used Khat (Khat is the fresh leaves and twigs of the shrub Catha edulis, which has a stimulating and euphoric effect when chewed or brewed as tea). He was single and had completed 12th grade in school.\nOn examination, he was not sick looking, was indifferent to his surroundings, and had stable vital signs. The pertinent finding was of a clean, healing granulating wound in his left lower abdomen measuring 2×2 cm with skin loss. There was tenderness on deep palpation in his lower abdomen but no rebound tenderness (Fig. ).\nRadiographic investigations revealed two radio-opaque shadows on the plain film of his abdomen, estimated to be 12–15 cm long (Fig. ). We diagnosed intra-abdominal foreign bodies and opted for exploratory surgery.\nA laparotomy revealed that the materials were lodged in his sigmoid colon. There were three wooden sticks of 8–10 cm in length in addition to two sharp pieces of saw (Fig. ). The area including the perforation in the colon was completely sealed by omentum. The materials were extracted via an enterotomy on the anti-mesenteric side of his colon and the site was closed primarily. The area was lavaged with a liter of saline and his abdomen closed. Our patient had a psychiatric consultation and was started on appropriate medications. He was discharged a week later with no major complications.\nOur patient was seen on two occasions after discharge and his surgical wound and stab wound healed completely. He started regular follow-up with the psychiatric unit of our hospital and showed marked improvement. |
A 52-year-old female experienced right knee joint pain when going up and down stairs one month prior to her presentation to a clinic. Examination by a local doctor and magnetic resonance imaging (MRI) resulted in a diagnosis of lateral meniscus injury. She was treated conservatively for 2 months, but the pain was not alleviated, and her left knee joint also became painful so she was referred to our hospital immediately. Examination at our department showed a restriction in knee joint range of motion (ROM), with right knee extension −15° and flexion 125° and left knee extension −10° and flexion 135°. Tenderness was present in the lateral femorotibial joint spaces of both knees, and clicking was palpated during flexion. The McMurray test also revealed lateral pain and clicking in both knees. There were no indirect signs of discoid lateral meniscus such as lateral joint space widening, squaring of the lateral femoral condyle, high fibular head, or hypoplasia of the lateral tibial plateau in plain radiographs (). Full-length frontal plain radiographs of the legs showed hypoplasia of the femoral lateral condyle and varus deformity of the articular surface of the proximal tibia. The femorotibial angle was 176° on the right side and 177° on the left side. The mechanical lateral distal femoral angle was 83° on the right side and 80° on the left side. The medial proximal tibial angle was 80° on the right side and 77° on the left side. Both Mikulicz lines were slightly displaced inwardly (). T2-weighted MRI showed that the medial menisci in both knees were incomplete discoid menisci, and there was high intensity in the posterior section. T2-weighted MRI of the lateral menisci in both knees showed complete discoid menisci and a horizontal high intensity area (). She underwent surgery immediately. Arthroscopy revealed that the lateral menisci of both knees were complete discoid menisci. Based on these findings, the patient underwent partial meniscectomy, leaving margins of about 6 mm. There was a horizontal tear from the anterior to the posterior section. The medial menisci were incomplete discoid menisci, but there were no findings of abnormal mobility or injury; therefore, and the medial menisci were observed without treatment (). At six months postoperatively, her pain and ROM restrictions disappeared. MRI showed no obvious changes in the medial menisci. One year and seven months postoperatively, she experienced no recurrence of pain or hydrarthrosis, and plain X-rays showed lateral femorotibial joint space narrowing (). |
A 39-year-old man presented with dizziness and gait disturbance of 2-years duration. The onset and progression were gradual. Over the past year, dysarthria and liquid dysphagia developed and progressed. He had been a problem alcohol drinker for the past 7 years. Over the past 3 years, he had become impatient and was easily angered. One year earlier, he was admitted to a local psychiatric hospital because of abnormal behavior, such as stealing alcohol from a store. He was told that the results of brain magnetic resonance imaging (MRI) performed during that admission were abnormal, but this was not followed up. He continued to drink after discharge. In his family history, his elder brother had alopecia and a similar neurological condition, and died at the age of 46 years.\nThe physical examination revealed near-total alopecia. On neurological examination, he was alert and oriented. Gaze-evoked nystagmus was observed in both directions and was worse when looking to the right. He also had dysarthria and dysphagia. His motor strength was normal, as were the results of the sensory examination. The deep tendon reflexes were decreased slightly in both the lower and upper limbs. There was no Babinski’s sign or ankle clonus. He had marked dysmetria in both upper limbs that was worse on the right. He was barely able to walk, with a broad-based gait.\nA review of the previous brain MRI results showed mild brainstem and cerebellar atrophy, with a subtly increased signal intensity in the right middle cerebellar peduncle on fluid attenuated inversion-recovery (FLAIR) imaging. Cerebral cortical atrophy was also present. The MRI was repeated and showed worsening of the brainstem and cerebellar atrophy. The FLAIR images showed symmetric high-intensity signals in the posterior limbs of the internal capsule, right middle cerebellar peduncle, and brachia of the inferior colliculus where it joins the medial geniculate body ().\nComplete blood counts, erythrocyte sedimentation rate (ESR), urinalysis, plasma electrolytes, and kidney, liver, and thyroid function tests were normal. Vitamin B12 was somewhat low at 196 pg/mL (normal range, 200–1,000 pg/mL), and the folate level was low at 1.3 ng/mL (normal range, 3–15 ng/mL). An intramuscular vitamin B12 injection and oral folate did not improve his condition. A Venereal Disease Research Lab oratory (VDRL) test for syphilis was negative. An electrocardiogram and a chest X-ray were normal. Gene tests for spinocerebellar ataxia types 1–3, 6, 7, and 17; dentatorubropallidoluysian atrophy; fragile X syndrome; and Friedreich’s ataxia were all negative. The plasma levels of pyruvate, lactic acid, homocysteine, and tumor markers were all normal. The adrenocorticotropic hormone (ACTH) level at 8 a.m. was 90 pg/mL (normal range, 0–60 pg/mL) and the cortisol level was normal at 10.2 μg/dL (normal range, 5–25 μg/dL). An adrenocorticotropic hormone (ACTH) stimulation test resulted in a defective rise in the cortisol level. A very long-chain fatty acid assay showed a C22 : 0 of 2.565 μM/L (normal, <1.310 μM/L), C24 : 0/C22 : 0 of 1.785 (normal, <1.390), and C26 : 0/C22 : 0 of 0.114 (normal, <0.023). |
A 67 year old female was initially presented to the Emergency Department with a complaint of epigastric abdominal pain. She described a two weeks history of that pain. Her medical history was unremarkable. On examination, she was in discomfort, with a heart rate of 90/min, blood pressure 110/60 mmHg and respiratory rate 18/min. On palpation, epigastric tenderness was present and a pulsatile mass was detected in this region. The laboratory analysis yielded hemoglobin (Hb): 11.2 g/dl (12-16), white blood cells: 14600/mm(3) (4500-11000) with neutrophils 78%. Findings on her electrocardiography and chest radiography were interpreted as normal. The patient was admitted to the Intensive Care Unit (ICU). An urgent computed tomography scan was performed which showed an infra-renal abdominal aortic aneurysm with a transverse diameter of 7 cm () starting 3 cm distal to renal arteries and ending 1 cm above common iliac arteries. CT findings include early detection of contrast material in the inferior vena cava, suggestive of an aortocaval fistula (). The ACF was concealed by a huge mural thrombus.\nOver the next 12 hours, the jugular venous pressure (JVP) elevated gradually, the blood pressure decreased and the patient became oliguric. Cardiac assessment did not show any pathologic change, so the patient was prepared for an emergent laparotomy. At laparotomy, a large retroperitoneal hematoma was detected and the aorta was exposed and clamped proximally from the lesser sac under the diaphragm and distally at the common iliac arteries. Having opened the aneurysm sac, the aortocaval fistula was identified. The venous bleeding into the aneurysm sac was controlled by direct pressure. The fistula was over sewn from within the aneurysm sac with 3-0 prolene. Then the aneurysm was replaced with a 20 mm diameter tubular graft. The patient was returned to the ICU after completion of the operation. She recovered very soon and was discharged in seven days. |
A 28-year-old woman presented with a 1-week history of headache, nausea, and vomiting. The patient had a medical record of acute lymphoblastic leukemia at 3 years of age. She had received a chemotherapy regimen that comprised vincristine, methotrexate, L-asparaginase, cytosine arabinoside, and 6-mercaptopurine, as well as whole-brain irradiation at a total dose of 18 Gy. The combined treatment brought the patient into clinical remission, and the patient was followed up until she was 18 years old with no relapse of the disease. Thereafter, she was lost from oncologic follow up. Six months before her visit to our hospital, she consulted another doctor about a mild head injury. Computed tomography (CT) of the brain showed no traumatic findings but incidentally revealed a small lesion in the left frontal convexity (Figure ). Six months later, she suffered from headache, nausea, and vomiting. A further CT examination revealed rapid enlargement of the lesion, and she was referred to our institution. Magnetic resonance imaging (MRI) of the brain revealed two other small-mass lesions in the right middle and frontal fossae (Figure ). Surgical resection of the left frontal-mass lesion was performed. During surgery, the tumor was found to adhere to the dura mater of the left frontal convexity and the wall of the superior sagittal sinus. The tumor was extensively removed, and the dural attachment was cauterized (Simpson grade 2). Histopathological examination revealed frequent mitoses, hypercellularity, and focal necrosis in the tumor specimens. Immunohistochemical analysis revealed an MIB-1 labeling index of approximately 20%. The pathological diagnosis was atypical meningioma.\nPostoperatively, the patient presented with neither neurological deficits nor radiological evidence of recurrence at the surgical site. Six months after surgery, a follow-up brain MRI revealed enlargement of the right middle fossa lesion. Thus, the two lesions on the right side were resected. Histopathological examination identified both tumors as meningothelial meningioma with MIB-1 labeling indices of approximately 10% and 15%, respectively. The postoperative clinical course was uneventful, and the patient developed no neurological deficits. Nine months after the first surgery, a follow-up MRI revealed a tumor recurrence in the left parasagittal region. Twelve months after the first surgery, a follow-up MRI identified a recurrence of the left frontal convexity meningioma in the frontal superior sagittal sinus and the frontal convexity dura matter. During the third operation, the tumor was found to have invaded into the frontal superior sagittal sinus, where it had attached to the bridging vein. The tumor was resected extensively and the dural attachment was cauterized. After surgery, the patient developed no neurological deficits. |
A 43-year-old Caucasian female with a history of nonischemic dilated cardiomyopathy with left ventricular ejection fraction (LVEF) of 10–15% status following Thoratec Heart-Mate II left ventricular assist device (LVAD) (implanted 2 years prior as a Bridge to Transplant) was transferred to our tertiary care facility for management of unresolving pseudomonas driveline infection. The patient secondary to persistent pseudomonas bacteremia despite adequate treatment with intravenous antibiotics underwent LVAD removal with reimplantation with another VAD. The patient also underwent an AICD lead extraction with generator change secondary to questionable vegetation on the defibrillator lead on transesophageal echocardiogram. The patient did well following that and remained home for 4 months while awaiting a cardiac transplant. Her past history was significant for hypertension, dyslipidemia, recurrent pulmonary embolism, history of herpes zoster infection with postherpetic neuralgia, and intracerebral hemorrhage.\nFour months later the patient was electively admitted for transplant evaluation. Her panel reactive antibody (PRA) levels were found to be low at 4% as measured by flow cytometry using HLA class I Luminex-coated beads. The patient (CMV positive) finally underwent a CMV negative, Epstein-Barr virus (EBV) positive orthotopic heart transplant without the need for desensitization. The patient's immediate postoperative course was complicated by multiple failed attempts at extubation secondary to fluid overload that required tracheostomy and acute kidney injury requiring temporary hemodialysis (with complete eventual recovery of renal function). The patient after 4 weeks, on routine surveillance endomyocardial biopsy (EMB), was found to have ISHLT grade 2R acute cellular rejection which was successfully treated with intravenous pulsed steroids and mycophenolate mofetil. The patient was eventually discharged home 2 weeks later and was followed as an outpatient. Three months subsequent to transplant the patient started to develop signs and symptoms of upper respiratory tract infections manifesting as unremitting cough. The patient admitted was found to have viral infection with positive serologies for entero, rhino, and coronaviruses, and the EMB was negative for rejection. The patient was managed conservatively without any antiviral treatment except prophylactic ganciclovir for CMV prophylaxis and discharged home. The patient did present again with similar respiratory symptoms a month later at which time it was decided to treat the patient with a course of oseltamivir (Tamiflu) for a clinical suspicion of influenza. The patient was discharged only to be readmitted 2 months later (6 months after transplant) for symptoms of exertional dyspnea, nausea, and abdominal pain. The patient was found to have low cardiac index (1.59 L/min/m2) and elevated right sided pressures on right heart catheterization while the EMB remained negative for cellular or humoral rejection. An echocardiogram at the time revealed a mildly depressed left ventricular ejection fraction at 40% with mild right ventricular dysfunction. The patient's panel reactive antibodies were undetectable. lists the trends in the available viral titres and other laboratory data (glucose and lipids). The patient was treated with intravenous methylprednisolone and plasmapheresis to treat for possible graft dysfunction. The next day the patient had a sudden cardiorespiratory arrest and died despite prolonged attempts at resuscitation.\nA postmortem analysis revealed microscopic changes of concentric intimal proliferation and inflammation resulting in near-total luminal occlusion in the epicardial and the intramyocardial coronary vessels, suggestive of graft vasculopathy (Figures –). There was no evidence of rejection seen. |
This case represents an 84-year-old man with a known history of left anterior descending (LAD) coronary artery lesion with a mass noted on the tricuspid valve. He had previously undergone percutaneous coronary intervention with 2 stents placed in the LAD coronary artery, 6 years earlier. Subsequently, he was being closely followed by his cardiologist for the coronary artery lesion, when the right atrial mass was found on transthoracic echocardiography. This mass was observed for a 6-month period. A repeat echocardiogram revealed a rapid expansion of the mass. Specifically, the mass grew by 1 cm in that 6-month period. The mass was found to be fixated on the tricuspid valve. Also at this time, the patient had some complaints of dyspnea on exertion, which prompted a cardiac workup. The results of the workup eventually showed a lesion within the previously placed stent. The combination of in-stent thrombosis of the coronary stent with the rapid progression of the intracardiac mass incited the decision for surgical intervention. The patient was taken to the operating room for a single vessel coronary artery bypass grafting and excision of the intracardiac mass.\nThe operation was performed under normothermic cardiopulmonary bypass using ascending aortic and bicaval cannulation (Video). The right atrium was opened and a solid, well-encapsulated 1-by-2-cm mass was found to be attached to the atrial side of the tricuspid valve (Fig ). It was subsequently shaved off and sent to the laboratory for histological evaluation (Fig ). The right atrium was then closed, followed by the single coronary artery bypass grafting utilizing the left internal mammary artery to the LAD. After completion of the procedure, no regurgitant flow was demonstrated at the tricuspid valve. The patient was discharged 5 days after the operation, following a postoperative pneumothorax, which resolved spontaneously. The final pathology on the right atrial mass confirmed the diagnosis of PFE.[] |
The menopausal 56 year old female patient (parity, 2-0-0-2) without specific medical history and history of hormone replacement visited the outpatient clinic for vaginal bleeding on August 2004. She was diagnosed with a cervical myoma 2 years ago at a local clinic. Since there was no change in the size of the myoma and bleeding, regular follow-up was conducted at the local clinic. Papanicolaou test (PAP) smears has remained normal for the recent 2 years. On speculum exam, polyps were not observed but an approximately 9 cm sized mass occupying the vagina was observed. Since it was difficult to find the cervical os due to the mass, the endometrium was difficult to evaluate properly. Endometrial thickness on ultrasound was approximately 3 mm and no specific findings were observed. Because postmenopausal vaginal bleeding continued for approximately 3 months, and high vascularity of the irregular contoured-mass was observed on Doppler ultrasound, total abdominal hysterectomy and bilateral salpingo-oophorectomy was performed due to the possibility of malignancy on August 27, 2004. After incising the tumor along the middle border of the anterior uterine wall, a huge well circumscribed solid mass was found fully filling the whole lower uterus. The mass was slightly friable with bleeding and necrosis. An ill-defined scattered necrotic solid mass was observed along the whole myometrial layer. According to the pathology report (), it was consistent with an uterine carcinosarcoma (with heterologous components) with negative resection margin. Extensive lymphatic and vascular invasion were observed on the tumor. Metastasis to both ovaries was additionally diagnosed by the pathology () despite the absence of no gross lesions at the time of surgery. On immunohistochemical staining, cytokeratin was positive while vimentin, CK20, CD10, epithelial membrane antigen was negative.\nTo identify the metastatic loci, abdominopelvic magetic resonance imaging (MRI), positron emission tomographycomputed tomography (PET-CT) and tumor markers (CA-125 and CA 19-9) were conducted 4 weeks after the operation. On MRI, multiple metastatic lymph nodes were observed in the pelvic cavity. Two metastatic nodules on the right lobe of the liver were observed. Furthermore, additional peritoneal seeding nodules were observed between the right lobe of the liver and upper pole of the right kidney, and a soft-tissue mass was observed on the vaginal stump. On PET (), abnormal fluorodeoxyglucose (FDG) uptake increase was confirmed on the aortocaval, mesenteric, bilateral iliac chain, left obturator lymph node, hepatic nodules, and peritoneal seeding nodules. The CA-125 was 50.0 U/mL. With the impression of an uterine carcinosarcoma stage IVB, we started palliative chemotherapy for 6 cycles from September 18, 2004 to January first, 2005; 5,000 mg/m2 of ifosfamide (with mesna) and 50 mg/m2 of cisplatin. No adjuvant radiotherapy was conducted. During the 6 cycles of chemotherapy, no serious side effects were found and the Eastern Cooperative Oncology Group (ECOG) performance scale was 0-1. The tumor marker (CA-125) normalized after 1 cycle of chemotherapy. Four weeks after completion of chemotherapy, MRI and PET-CT scans were followed-up. The lesions mentioned above were almost diminished over 80% (partial response by Response Evaluation Criteria In Solid Tumors [RECIST] criteria). Thereafter, the progress was observed every 3 months for the first year and every 6 months thereafter. No tumor recurrence, distant metastasis and meaningful lymph node enlargement has been reported on the image studies. Recent PAP smears as well as the tumor marker are all normal as of present (June, 2010). Her general condition is very tolerable with excellent ECOG performance scale (0-1). |
A 44-year-old man came to the emergency department in our hospital with suboccipital headache and paresthesia of the left arm. Examining his history, he was generally healthy and had no distinguishable medical history besides several near syncopes. However, he had not visited a hospital when he started to feel a near-syncope.\nTwo days before admission, the patient started to experience dizziness and syncope while he stood up in the pub. After recovery of consciousness, he felt a suboccipital headache and paresthesia of the left arm, so he visited the emergency department of another institute. At that time, since his symptoms had vanished, the hospital discharged him without complete medical checkup such as a computed tomography (CT) or magnetic resonance imaging (MRI). However, when he returned home, the headache and paresthesia reappeared, causing the patient to visit our hospital for accurate medical evaluation. The medical evaluation results are as follows: physical and neurological exam were normal except for paresthesia of the left arm. The CT scan revealed a 6.6×5.4 cm sized large cystic lesion of the left CPA with no abnormal enhancement by contrast medium (). The MRI demonstrated a cystic lesion, similar to an arachnoid cyst, with shift of the cerebellar hemisphere, pons and medulla to the right side ().\nAfter admission, general evaluation followed to focus on the cardiogenic syncope. During the routine check-up within the emergency room, the electrocardiogram showed a sinoatrial node block. Consultation with a cardiologist followed to evaluate the sinoatrial node block and syncope. Furthermore, an echocardiogram, 24 hours Holter monitoring and flecainide provocation test was performed to rule out the possibility of Brugada syndrome. Despite these efforts, the result of cardiac evaluation did not show any abnormality which could cause the syncope.\nWe assumed that EC resulted in the syncope and therefore decided to intervene surgically. A left suboccipital retrosigmoid craniotomy was conducted to approach to the cyst, and which was followed by fenestration into the subarachnoid space. Partial cystic wall removal was also conducted for pathological diagnosis. The pathological examination with immunohistochesmistry study showed features of a typical ependymal cyst. The lining epithelium of the cyst consisted of simple cuboidal epithelial cells with no prominent cilia (). The epithelial cells showed diffuse positivity for glial fibrillary acidic protein () and dot-like positivity for epithelial membrance antigen (). Postoperatively, the hospital course was favorable, with the exception of nausea and vomiting which persisted for several days and which resolved later. He was discharged at 14 days after surgery. At the last follow-up at 4 months after surgery, he was neurologically normal and free from near syncope. |
A 34-year-old female patient visited the Department of Oral Medicine and Radiology, with the chief complaint of swelling on the left side of the face that had persisted for 6 months []. Two years prior to this, the patient had pain in the left body of the mandible, which was dull, continuous and radiating to the temple region. Patient gave no history of trauma to the region and no evidence of pathology was noted during the clinical examination and radiological investigations. She was advised regular follow-up, however, patient did not adhere to this instruction.\nThe current lesion presented as a well-defined swelling over the left lower border of the body of the mandible, 1 cm in front of the angle, 1×1 cm in size, oval in shape, bony hard, tender, and attached to the lower border of mandible, with no local rise in temperature. The overlying skin was normal in color and was not attached to underlying structure. Intraoral examination revealed lingually tilted tooth number 35 and periodontal pocket in relation to teeth 37 and 38 with no significant finding with respect to the swelling [].\nOn the basis of history and clinical examination, provisional diagnosis of osteoma of left body of mandible was given. Differential diagnosis of exostosis and osteoblastoma was considered. The patient was subjected to routine hematological and radiographical examination. The orthopantamograph [] view revealed a well-defined radiopaque mass, round in shape, measuring 1×1 cm over the left inferior border of the mandible.\nCT view revealed a well-defined radio-dense area attached to the left medial aspect of lower border of mandible \n [Figures and ]. On the basis of radiographical examination, diagnosis of osteoma of left body of mandible was given. An excisional biopsy of the lesion and subsequent histopathological analysis revealed well-differentiated mature bone with proliferation of cancellous bone, thus confirming the lesion as osteoma. |
A 45-year-old Asian male with a history of nasopharyngeal carcinoma (NPC) was referred to our clinic in the spring of 2016 after he was found to have NPC recurrence. His past medical history was insignificant except for a four pack-year smoking history (2006–2010). He had no personal or family history of allergies, asthma, chronic obstructive pulmonary disease (COPD), or atopy. The patient was diagnosed via fine needle aspiration of a cervical lymph node in 2013 and underwent definitive chemoradiation therapy (CRT) from late 2013 to early 2014 (cisplatin and conventional radiation therapy (XRT) with a total dose of 56 Gy over 28 fractions). However, 9 months after completion of CRT, a positron emission tomography (PET) scan was suspicious for recurrence. Nasal biopsy was performed, but was found to be negative. Roughly 19 months after completion of his CRT, the patient’s surveillance PET scan was again suggestive of recurrence. In January 2016, the patient underwent an endoscopic left maxillary antrostomy, left total ethmoidectomy, and sphenoidotomy with biopsies at another institution. Pathology confirmed recurrence of poorly differentiated NPC. At that time, the patient’s disease was determined to be endoscopically unresectable so he was referred to our institution for consideration for open resection. Upon referral, a magnetic resonance imaging (MRI) was obtained, which demonstrated extensive left skull base recurrence of the primary NPC with cavernous sinus, Meckel’s cave, and perineural involvement along V3 to the left mandible (). There was also involvement of the orbital apex and temporal lobe dura. Symptomatically, the patient reported numbness along the left side of his face and decreased taste along the left hemi-tongue. He denied any difficulty swallowing, vision loss, hearing loss, or weakness. After presenting the patient at our tumor board, it was decided that the patient’s volume of disease was unresectable, but his excellent performance status and minimal symptoms made him an appropriate candidate for our phase I IA cetuximab clinical trial with concurrent re-irradiation.\nIn April 2016, the patient was enrolled and underwent the first dose of his two scheduled IA cetuximab doses of 100 mg/m2 in a Phase I dose-escalation clinical trial (NCT02438995). The morning of the infusion the patient’s vitals were stable (T: 99, heart rate (HR): 78 bpm, blood pressure (BP): 119/65, respiratory rate (RR): 14, SaO2: 98%) and consistent with patient’s baseline vitals. As per the study protocol, the patient was prophylactically treated with 50 mg of diphenhydramine 1 hour prior to the start of the IA cetuximab infusion. After groin puncture and demonstrating a safe intravascular positioning of the microcatheter for IA infusion, a total of 81.5 mL of cetuximab was infused over 12 min ( and ). After infusion of the first 50 mL, the patient developed a cough with concurrent elevation of his HR up to 110 bpm. He was treated with 100 mg intravenous hydrocortisone sodium succinate and his symptoms resolved, allowing for the remainder of the infusion to be given to completion without adverse effects. The patient did not experience any severe vascular pain or vascular spasms as a result of the procedure. The catheter and femoral sheath were removed and hemostasis was achieved with a perclose device. Subsequently, while still in the cath lab, the patient’s HR and RR suddenly increased to 110 bpm and over 20, respectively. His BP dropped to 50/30, his oxygen saturation dropped to 90, and he experienced bronchospasms. The anesthesiologist treated the patient with 20 mg of epinephrine, 180 mcg of phenylephrine, and 4 mg of Zofran, along with positive ventilation and a total of 900cc of Lactated Ringers. After intervention, the patient’s vitals stabilized (HR: 91 bpm, BP: 111/66, RR: 16, SaO2: 99), and he was transferred to the holding area for observation and recovery. The patient never demonstrated signs of flushing, rash, fever, chills, and dyspnea and his vitals remained continuously stable for the following 24 h of observation. The patient was then discharged home and reported feeling well with no complaints during a follow-up phone call 3 days post infusion.\nPer protocol, the patient was removed from the IA cetuximab trial due to severe IR, so he never received the second scheduled dose. However, he proceeded to receive intensity-modulated radiation therapy (IMRT) to his left nasopharynx, skull base, cavernous sinus, and Meckel’s cave. He received a total of 7000 cGy over 35 fractions with some minor overlapping of his prior radiation site; 16 months out from completion of his one dose of cetuximab and re-irradiation, the patient’s surveillance imaging still shows no evidence of disease. |
A 40 year old male presented with a chief complaint of pain in the right dominant wrist. Two months prior to presentation, he sustained injury to the right wrist while protecting himself with extended right upper extremity upwards from heavy object falling from a high place. Initially, he did not seek any treatment despite immediate severe pain. However, symptoms of the injured wrist, including swelling and pain increased over time. Examination of the right wrist revealed generalized swelling, diffuse tenderness, limited range of motion, at wrist and an abnormal bony prominence in the palmar aspect of the scaphoid. The function of the fingers was unimpaired. There was no sign of neurovascular injury. Radiographs of wrist showed an isolated palmar dislocation of the scaphoid and there were no abnormal findings in other carpal bones []. These findings were confirmed with computed tomography [].\nInitially, closed reduction was attempted by longitudinal traction and ulnar deviation undermanual application of direct pressure over the dislocated scaphoid under general anesthesia, but it was unsuccessful. We determined further attempts would be futile considering that 2 months had already passed from the date of trauma. Thus, open reduction was performed through a dorsal approach. After capsulotomy, the isolated palmar scaphoid dislocation was observed, and a complete tear of the scapholunate ligament was noted []. Although we could not identify the integrity of the volar side ligaments, we assumed that they were disrupted. Reduction was interrupted due to fibrotic soft tissue interposed in the scapholunate interval. After debridement and retraction, the dislocated scaphoid was reduced []. The torn scapholunate ligament was sutured using a Mitek anchor (DepuyMitek, Inc. Raynham, MA, USA) and the reduced scaphoid was stabilized using two Kirschner wires (one scapholunate wire and one scaphocapitate wire). In addition, Blatt's dorsal capsulodesis was performed using a Mitek anchor to prevent rotary subluxation of the distal pole of the scaphoid []. After wound closure, an above the elbow thumb spica splint was applied for 2 weeks. An above the elbow thumb spica cast was worn in the same position for the subsequent 3 weeks, which was followed by application of a below the elbow thumb spica cast for an additional 2 weeks. In the 7th postoperative week, Kirschner wires were removed and rehabilitation was started.\nThe patient showed satisfactory wrist function on subsequent followup. He was doing regular physiotherapy. He was able to return to his work as a manual laborer and had no complaints of residual pain. At 6 years followup, his radiographs of wrist showed a normal carpal alignment with a scapholunate gap of 3 mm and no evidence of avascular necrosis (AVN) of the scaphoid []. The grip strength measured using a dynamometer was 21 Kg in the right wrist (20 Kg in the left wrist). The range of motion in the right wrist was 60° dorsiflexion, 55° palmar flexion, 25° ulnar deviation and 15° radial deviation (70°, 70°, 30° and 15°, respectively, in the left wrist). |
A 29-year-old Hispanic male presented with a week of blurriness in his left eye that progressed to complete vision loss. He was HIV positive for 6 years with a latest CD4 count of 100 and a viral load of 617 copies/mL on HAART therapy. He noted fevers and chills, but no other symptoms. On examination, the left pupil was fixed with no response to light. A reverse relative afferent pupillary defect was present in the right eye. The left optic nerve was edematous with blurring of margins. A CT scan showed enhancement of the retrobulbar optic nerve of the left eye with an adjacent, discrete soft tissue enhancement bordering the posterior globe (Figure 1 ). Two weeks later, an MRI showed diffuse thickening and contrast enhancement involving the retrobulbar portion of the left optic nerve associated with a surrounding soft tissue lesion within the intraconal orbital fat, and the lesion seemed to be extending more posteriorly than the previous CT image (Figure 2 ). The mass abutted the posterior aspect of the sclera. The lesion demonstrated low T1 and intermediate T2 signal intensities and heterogeneous contrast enhancement. There was also nodular contrast enhancement of the left optic nerve disc. The size of the lesion measured 17.4 mm x 15 mm x 10.6 mm. Cerebrospinal fluid studies were unremarkable. An anterior orbitotomy with exploration and biopsy of the left orbital mass was performed.\nHistology of the mass showed tissue diffusely infiltrated with large, dysplastic lymphocytes with vesicular nuclei (Figure 3A ). Immunohistochemical stains showed that the lymphoid cells were positive for CD10 and CD20 (Figure 3B and 3C ) and were negative for CD3 and keratin AE1/AE3. Ki-67 demonstrated proliferative activity in greater than 90% of tumor cells (Figure 3D ). Additional immunohistochemical stains showed that the tumor was positive for BCL-2 and BCL-6. The patient was diagnosed with DLBCL (germinal center type) and was referred to oncology. He underwent staging and there was no other evidence of the disease in any other system in the body. He was started on R-CHOP chemotherapy. He was given one round of R-CHOP before he was unfortunately lost to follow-up; one round of R-CHOP administration did not improve his visual acuity. |
A 68-year-old man was referred to our hospital with a deteriorated general condition due to chronic hepatitis C genotype 1b. In this advanced stage of disease, treatment with pegylated interferon, ribavirin and a direct acting antiviral (DAA) was not indicated. Progressive cirrhosis necessitated a liver transplant 2 years later. The pre-transplant ophtalmological examination was performed 6 months before the actual liver transplantation and was completely normal. Following the transplantation, treatment with triple immunosuppression with tacrolimus, mycophenolatic mofetil and prednisolone was initiated. Tacrolimus was replaced by cyclosporine because of confusion. Three months later, the liver function had normalized with therapeutic blood levels of cyclosporine around 75 µg/l, the viral load at that moment was 130,499 IU/ml. Four months after the liver transplantation, the patient developed an acute visual loss in both eyes. Best-corrected visual acuity was 0.6 in the right eye and 0.12 in the left eye. There was a relative afferent pupillary defect in the right eye. On fundoscopy, bilateral optic disc oedema with haemorrhages at the disc margin was seen (Figure 1 ). Treatment with cyclosporine A was interrupted and the dose of prednisolone was increased to 24 mg in combination with mycoplenolatic mofetil. One month later his vision was at 0.4 in the right eye and 0.25 in the left eye. The papillary oedema gradually subsided and the optic nerves became pale. Goldmann perimetry showed normal peripheral limits and central visual field testing showed a superior nasal scotoma in the right eye and an inferior nasal scotoma in the left eye. Brain MRI (magnetic resonance imaging) was within normal limits. A blood sample could not detect any infectious causes of an optic neuropathy, but an HCV viral load was >5 million IU/ml was measured. Patient was closely monitored, but after one month there was no visual improvement and optic pallor was progressive bilaterally; the dosage of prednisolone was increased to 2 x 32 mg. Six months after transplantation liver function tests deteriorated and the patient developed a histologically proven fibrotic cholestatic hepatitis. Antiviral therapy with peginterferon alpha 2a and ribavirin was given for one month. During that month BCVA slightly decreased and visual fields deteriorated. After this month, seven months after the liver transplant, therapy was switched to ribavirin and sofosbuvir. Afterwards visual acuity stabilized and Goldmann perimetry showed a scotoma inferior and nasal in the right eye and a narrowing of the peripheral field in the left eye. Central visual field examination showed an increase of the inferior nasal scotoma in both eyes. Ten months after the transplant, therapy was switched to daclatasvir and sofosbuvir because of the availability of DAA (direct acting antiviral) in Belgium. Prednisolone was progressively tapered and cyclosporine was reintroduced. The viral load dropped to 373 IU/ml. Over the following months, vision improved slightly to 0.7 in the right eye and to 0.5 in the left eye. The peripheral and central visual fields improved. The optic nerves had become atrophic (Figure 2 ). Liver function test normalized and the viral load became undetectable. Three months after the start of daclatasvir and sofosbuvir the patient was a sustained responder and the liver test was completely normal. |
A 48-year-old, previously healthy female patient visited the emergency department with initial presentation of intermittent abdominal pain and anal bleeding. The patient explained that the symptom had first begun 4 to 5 years ago. Under the clinical impression of hemorrhoids, the patient underwent a hemorrhoidectomy. The patient had no particular family history, changing bowel habits or mucoid diarrhea. An abdominal computed tomography (CT) was conducted 2 weeks later as a part of the evaluation process, which revealed a colocolic intussusception from the sigmoid colon to the distal rectum. In addition, prominent mucosal folds were observed without any ischemic change or pericolic abnormality. Following the CT scan, a barium enema revealed a large movable and compressible submucosal mass in the distal portion of the sigmoid colon (). Multiple similar yet smaller lesions were also present near the main mass in the sigmoid colon. The intussusception spontaneously regressed without any complications. Colonoscopy revealed several large polypoid masses in the rectosigmoid colon and multiple biopsies were obtained. The biopsied specimens were diagnosed as chronic nonspecific inflammation without any definite submucosal masslike lesions. Due to the presence of such large lesions, the patient underwent a laparoscopic low anterior resection in which a 21.5-cm segment of colon was resected.\nThe surgical specimen revealed multiple, large, tree-like branching polypoid masses, with elongated or pedunculated stalks or a wide broad base, measuring up to 14.0×5.0×4.5 cm in dimensions of the largest polypoid mass (). Upon microscopic examination of the lesions, most of the polyps had long, elongated, and loose or fibrotic submucosal elongated stalks, while some were sessile. There was neither prominent cytologic atypia nor active inflammation in the mucosa. There was also proliferation of mature fat tissue, lymphoid follicles, multiple dilated blood vessels, and hypertrophied smooth muscle of the muscularis mucosae (). Immunohistochemical staining with α-smooth muscle actin revealed hypertrophied smooth muscle of the muscularis mucosae (). However, there was no tree like proliferation of the muscularis mucosae as in Peutz-Jeghers polyps. Staining with other antibodies, such as anaplastic lymphoma kinase, c-Kit, β-catenin, CD34, and S-100 protein, showed negative findings in all. |
A 15-year-old boy without any medical history was referred to our hospital for osteomyelitis of the sternum and a subcutaneous abscess extending over half of the anterior chest wall. The patient's chest had been elbowed by an opponent during a basketball game 19 days earlier. He stated that there were no abrasions or lacerations at the time of injury, but 10 days later, he developed chest wall swelling () and fever. He consulted a local orthopedist and underwent chest computed tomography (CT), leading to provisional diagnosis. The chest mass was punctured, and the pus was drained. Subsequently, the patient was sent to our hospital. Chest CT revealed a sternal fracture and extensive fluid collection in the chest wall (). Increased density of the region behind the sternum was also observed, but we concluded that inflammation had not affected the anterior mediastinum. Because the patient did not appear severely ill, we performed pus drainage from a minimal incision and subcutaneously applied a 19 Fr silicone drain connected to a closed drainage system (BLAKE drain and J-VAC Reservoir; Johnson and Johnson Medical, Tokyo) under general anesthesia. The drained material yielded a Staphylococcus aureus isolate on microbiological culture; this culture was sensitive to all antibiotics tested. The patient was treated by intravenous administration of sulbacillin for 2 weeks, followed by oral administration of tosufloxacin for 10 days. The drain was removed on postoperative day 14. The patient was discharged on postoperative day 26, and antibiotic therapy was discontinued. While infectious granulation was observed at the drain tube-penetrating site for a few months after discharge, complete epithelialization of the site was confirmed 4 months after the surgery. Follow-up CT performed 4 months after surgery showed disappearance of fluid collection and ossification of the fracture site (). The patient did not show any symptoms and signs of recurrence 6 months after the surgery. |
A 55-year-old man with the end-stage renal disease was assessed for decreased appetite, dysphagia, and nausea during the hemodialysis. He was on a routine hemodialysis program for 4 h, three times a week, for 7 years. In his history, he had essential hypertension for 15 years. The etiology of end-stage renal disease may be hypertension or systemic amyloidosis in our patient, but kidney biopsy had never been performed. He had been diagnosed with RA 20 years ago and had been taking leflunomide 20 mg/day regularly. His other medication was darbepoetin alfa 40 mcg/week, acetylsalicylic acid 100 mg/day, calcium acetate 700 mg three times a day, and lansoprazole 30 mg/day. On physical examination, the thyroid was diffusely enlarged (Grade 4) with multiple nodules. He had signs of long-standing RA in the form of joint deformities and rheumatoid nodules.\nLaboratory findings of the patient can be seen in . Abdominal ultrasonography (USG) was performed, and it revealed normal findings. In addition, the fecal occult blood test was negative, and the patient did not have weight loss, so intraabdominal malignancy was not considered.\nOn thyroid USG, the parenchyma was homogeneous. Both of the thyroid lobes were enlarged. A large number of heterogeneous, hypoechoic solid and mixed nodules with cystic and calcified degeneration were found on both the lobes and isthmus, the largest one being on the right lobe (50 × 28 × 24 mm). Aspiration biopsy of the largest nodule showed a benign characteristic. As the patient had a large number of nodules, total thyroidectomy was performed.\nAccording to macroscopic pathological examination, the right lobe was 12 × 7.5 × 7 cm, left lobe was 9.6 × 6 × 5 cm, and whole surface was encapsulated. In the right lobe section, a partially cystic nodule 4 × 3 cm in size separated by marked and regular borders from surrounding tissue was detected. One cystic nodule 4 × 4 cm in size in the left lobe of the section was observed. The surrounding thyroid tissue was hardened and had a light brown and homogeneous appearance. Microscopic examination revealed colloid-containing, enlarged and elongated thyroid follicles. Extracellular, acellular and homogeneous eosinophilic amorphous proteinaceous material (amyloid) accumulation was observed diffusely between or around the follicles and around the blood vessels. Mature fat attracted attention among the follicles and amyloid []. In the sections stained with Crystal Violet, between thyroid follicles and blood vessels, diffusely metachromatic staining extracellular amyloid accumulations were observed [].\nIn the sections stained with Congo red, the accumulation of amyloid showed apple-green birefringence under polarized light. Immunohistochemical examination for CD3, CD20 and CD138 were performed to exclude plasma cell dyscrasias and hematolymphoid neoplasms that could be avoided even though no significant mass was observed in the case. Due to these examinations, a very small number of CD138-stained plasma cells were detected but CD3, CD20-stained lymphocytic cells were not detected.\nImmunohistochemical examination with thyroglobulin, TTF-1, CEA, synaptophysin, chromogranin, and CD56 was performed to show that the follicles were lined with thyroid cells in order to differentiate medullary carcinoma. Cells that lined the follicles were stained with thyroglobulin and TTF-1 while staining with CEA, synaptophysin, chromogranin, and CD56 was not detected. There was no evidence of medullary carcinoma. In light of all these clinical, macroscopic, histopathologic, and histochemical and immunohistochemical findings, the case was reported as amyloid goiter.\nComplaints of dysphagia, nausea, and decreased appetite of the patient improved after the surgery. The patient is at the postoperative 12thmonth of follow-up and on 100 mcg levothyroxine replacement therapy, leflunomide 20 mg/day, and undergoing regular hemodialysis in our center. |
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