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A 7-year-old boy was referred to our clinic for evaluation of cardiac murmur. He had speech disorder, hemifacialmicrosomia, bilateral epibulbardermolipoma, bilateral preauricular tags and triangular face. His general appearance was compatible with GS []. His left arm and femoral pulses were very weak and a thrill was palpable in the suprasternal notch. A grade 3/6 ejection systolic murmur was heard at the upper right and left sternal borders with wide radiation to the back. Blood pressure was not elevated in either arm. Electrocardiogram was normal, but chest X-ray showed a concavity in the upper left border of cardiac silhouette.\nThe patient was admitted and elective cardiac catheterization was performed. The left-sided ascending aorta, was accessed through the right axillary artery and the right-sided descending aorta was entered through the left femoral artery. Complete right heart study was performed through a right femoral access and was normal. Left ventricular pressure was 150 mmHg and pressure of descending aorta was 85/63 mmHg. Ascending and descending aortograms in antero-posterior and lateral views demonstrated right tortuous circumflex aortic arch with hypoplasia of the distal segment and long-segment coarctation without any significant collateral arteries [ and ]. There was a 70 mm Hg pressure gradient (PG) between left ventricle and ascending aorta and 20 mm Hg PG between the distal aortic arch and the post-coarctation segment of descending aorta.\nCardiac CT angiography and cardiac magnetic resonance angiography showed a vascular ring [] produced by the retro-esophageal segment of the right aortic arch and an aberrant hypoplastic left subclavian artery arising from the area of coarctation. Vertebral artery Doppler ultrasound study excluded subclavian steal syndrome. Patient was discussed on the Congenital Cardiovascular Interventional Study Consortium in September 2013. He was referred to surgeon for reconstruction of hypoplastic aortic arch and repair of coarctation. However, the family opted to not follow the recommendation\nA simple embryologic model of paired aortic arches, including third, fourth, and sixth aortic arches, was made to explain the embryologic basis of the right circumflex aortic arch [].\nUsing this model and comparing the aorta of the patient with it, the embryologic basis of the development of a right aortic circumflex aortic arch was revisited. |
Case #1 is a 3 years old term-born girl referred for persistent cough and dyspnea and intermittently required oxygen supplementation started one month before presentation, and chest CT performed 3 months after onset showed diffuse ground glass opacities and subpleural parenchymal micronodules. She is the second daughter of non-consanguineous healthy parents. Her sister died in the first year with respiratory insufficiency of unidentified etiology. The child was in good general condition. Chest auscultation was unremarkable. Humoral and cellular immunity testing, sweat tests and genetic analysis for cystic fibrosis,, cardiac evaluation, abdominal ultrasound all resulted normal. CT scan confirmed widespread patchy ground glass opacities and subpleural micronodules, with interstitial thickening. Bronchoscopy showed normal airways; bronchoalveolar Lavage (BAL) showed normal cytology and lipid index, negative bacterial culture, and negative respiratory viral immunofluorescence and PCR tests. SFTPC gene sequencing detected the I73T mutation. Parents, who were both asymptomatic, were not mutation carriers. The genetic findings are difficult to interpret, as parental testing suggests a de novo mutation, but would imply that the sibling died of an unrelated cause. The child was started on hydroxychloroquine 5 mg/kg/day per oral (HCQ). Her cough and dyspnea improved after one week and she was discharged home on room air. Three months after she was still completely asymptomatic, and a repeat chest CT was improved, showing disappearance of the ground glass abnormalities but persistence of micronodules. After six months we observed a complete recovery of the CT findings. At 3 years of age the child is still on treatment, with no respiratory symptoms.\nCase #2 is a 4 years old male born at term, asymptomatic with normal growth and development until he presented at 6 months of age with a respiratory syncytial virus (RSV) bronchiolitis, which evolved in acute respiratory distress syndrome (ARDS) requiring surfactant administration and mechanical ventilation for one week. Bronchoscopy was normal; lung CT showed ground-glass attenuations suggestive of ILD. Immunological screening showed a mild IgA and IgG deficiency. Sweat test and cystic fibrosis genetic test were normal. The infant was discharged home after one month, on oxygen and oral methylprednisolone. He was referred to our center at 12 months,of age. The infant was in poor nutritional status, with a weight <10th percentile. Clinically, he had intercostal retractions, diffuse crackles on auscultation and hypoxemia on room air. Infection workup was negative. Bronchoscopy showed no airway anomalies but lipid-laden alveolar macrophages were seen on BAL fluid examination, indicating chronic tracheal aspirations. CT scan showed widespread ground glass opacities and multiple subpleural and intraparenchymal cystic areas predominantly in the upper lobes. The infant continued oxygen and steroids at higher dosage, but a repeat chest CT at 18 months showed more extensive anomalies. An open lung biopsy was then performed, and showed thickened interstitium with inflammatory cells and fibrosis, alveolar type II cell hyperplasia, and cellular and amorphous material in alveolar lumen, a picture consistent with desquamative interstitial pneumonia (DIP), suggestive of genetic surfactant disorders. SFTPC gene sequencing revealed a mono-allelic I73T mutation. Parents declined genetic testing. The infant was discharged with monthly methylprednisolone pulse therapy plus oxygen. Despite an initial improvement, the repeat chest CT after 10 pulses showed progression of ILD. At that stage, HCQ was added to methylprednisolone. After 6 months we observed an improvement in SaO2/CO2 nocturnal monitoring, allowing weaning then discontinuation of oxygen therapy and switching to hydrocortisone as a lower potency steroid regimen. At the age of 36 months, since chest CT was stable compared to the previous one, HCQ was replaced by azithromycin given its better safety profile. At 4 years of age, chest CT was unchanged and the infant was stable off oxygen and steroids.\nCase #3, male, born at term with no neonatal issues. Family history was unremarkable. The infant had several episodes of lower respiratory tract infections starting at 2 months of age. At one year of age, he was hospitalized in the pediatric intensive care unit for acute respiratory failure, intubated and ventilated. Cystic fibrosis and immunodeficiency were excluded. Chest radiography showed diffuse opacities of both lung fields and interstitial thickening. Lung CT showed multiple ground-glass consolidations, and thickening of the bronchial vessels. He underwent a lung biopsy, which showed DIP. Immunohistochemistry showed marked proSP-C accumulation in hyperplastic type 2 cells and in alveolar macrophages. Electron microscopy documented abnormal type II pneumocytes with numerous large cytoplasmic vesicles (endosomes) filled with unstructured phospholipids, consistent with SP-C deficiency [, ]. Molecular analysis identified an I73T mutation, inherited from mother who is an asymptomatic carrier. HCQ was added to steroids after the report of the biopsy but the patient died after 18 days of intractable respiratory failure.\nILD in infants have a wide range of etiologies including genetic abnormalities of the surfactant system, immunological defects, environmental exposures and idiopathic entities such as neuroendocrine cell hyperplasia of infancy and pulmonary interstitial glycogenosis []. These three cases illustrate the variability of clinical severity associated with the I73T mutation: one asymptomatic at 3 years, one with chronic lung disease at 4, and one who died at 1. Similar phenotypic variability was described in a single Mauritanian family affected with I73T, where 5 affected subjects had very different onset, symptoms, radiological presentation and outcomes []. The underlying mechanisms of this variable penetrance are not fully understood.\nTrans-heterozygosity has been reported as a molecular mechanism explaining this expression variability. In a recently published report, three infants with SP-C deficiency due to an I73T mutation and unusually early respiratory symptoms carried an additional mutation in the ATP binding cassette protein A3 gene (ABCA3), a phospholipid carrier essential for lamellar body formation and surfactant intracellular assembly. ABCA3 deficiency, which typically leads to lethal neonatal-onset respiratory distress syndrome, is transmitted in autosomal recessive mode and heterozygous carriers are unaffected []. However, this report suggests that mono-allelic mutations in different genes, which would be asymptomatic if isolated, may inflict multiple hits may have cumulative effects on the surfactant synthesis pathway and modify the disease course. We screened the 3 patients in this report for mutations in the SFTPB, ABCA3 and NKX2.1 genes, all associated with lung disease in human, and none were found.\nThere are several lines of evidence that respiratory infection, particularly RSV, can trigger severe lung injury and catastrophic respiratory failure in SP-C mutation carriers. Genetically modified animal overexpressing a common human mutant pro-SP-C (Δexon4, a BRICHOS domain mutation) show an increased rate of alveolar cell death []. SP-C deficient mice exposed to RSV have a stronger inflammatory response and more severe interstitial lung disease than controls, and restoration of SP-C expression by transgene therapy has a rescue effect in these animals, which could pave the way to future gene therapy in human [, ]. SP-C mutations result in loss of TLR3 modulation, IL-8 induction and paracrine modulation of the CCR2 and CXCR1 chemokine receptors on leukocytes leading to uncontrolled inflammation and fibroblast proliferation. In a recent clinical study by our group, 4 out of 7 cases were revealed by an unusually severe/protracted bronchiolitis episode, and similar observation was reported by others [, ]. In this report, cases #2 and 3 confirm this concept.\nThere have been no controlled trials of any treatment in children ILD and the management is based upon uncontrolled studies, case series, case reports and unsystematic observations. Steroids, hydroxychloroquine and azithromycin are commonly used in SP-C-related ILD. Azithromycin has anti-amyloid properties, and hydroxychloroquine affects intracellular proSP-C processing; hence these drugs may possibly have selective effects on specific mutations, although there are currently no clinical data available to support this concept. Lung transplantation is an option for children with end-stage ILD, but its use is limited by donor availability and poor outcomes [, , ].\nThe broader availability of genetic testing has made early, noninvasive diagnosis accessible to clinicians. CT findings were consistent with ILD in the three patients, consistently with recent literature []. With proper technique, chest CT imaging patterns may sufficiently suggest a diagnosis so as to avoid the need for surgical lung biopsy. Chest CT is also useful in monitoring the effects of treatment, as there is a strong correlation between clinical improvement and CT findings trend: as previously described, even in our patients 1 and 2, clinical improvement and weaning-off oxygen were associated with a reduction of ground glass opacities.\nWhen lung biopsy is needed, chest CT is important in guiding the selection of biopsy sites. Surgical lung biopsy using video-assisted thoracoscopy (VATS) is recommended for infants with clinical urgency to identify the specific form of ILD or in whom other diagnostic evaluations have not yielded a specific diagnosis. Lung biopsy specimens should be handled according to established protocols, with sections available for histopathology, immunohistochemistry, microbiologic culture, electron microscopy, immunofluorescence or other special studies, in consultation with a pediatric pathologist experienced in childhood ILD []. |
A 21-years-old Caucasian woman presented to a private dental clinic with a chief complaint of asymptomatic swelling in the gingiva observed four years prior. A gradual increase in size and no history of previous treatment were also reported during the anamnesis. The patient signed the informed consent, which represents the ethical approval of the faculty committee. Her medical and socio-economic histories were not contributory. The extra-oral evaluation did not reveal changes. The intraoral examination revealed a sessile nodule with a color similar to that of the mucosa and a focal erythematous area with a fibro-elastic consistency measuring 1.5 cm in the largest diameter extending from the inferior right lateral incisor to the inferior right first premolar. The lesion involved the vestibular and lingual gingiva, causing displacement of the inferior right canine (Fig. ).\nPanoramic reconstruction and parasagittal slices of the Cone Beam Computed Tomography (CBCT) showed a slightly superficial hypodense area between the inferior right lateral incisor and inferior right canine with reabsorption of the alveolar crest (Fig. ). Based on the clinical and immunological aspects, the main diagnosis hypotheses included peripheral ossifying fibroma, peripheral giant cell lesion, and ancient pyogenic granuloma. The peripheral odontogenic tumors were also included as a differential diagnosis. An excisional biopsy was performed and a clear separation was noted between the lesion and mandible bone during the trans-surgical approach. The histopathological analysis revealed a well-circumscribed proliferation comprising numerous islands and strands of epithelial polyhedral cells with well-defined borders and marked round nucleus in the connective tissue under the mucosal epithelium. Numerous nests, cords, and small islands of polyhedral cells with clear and vacuolated abundant cytoplasm were observed interspersed with the amorphous eosinophilic deposits (Fig. ). Immunohistochemistry was performed, which yielded positive results for CK-19 in the epithelial cells, except for the clear cells. Congo red staining showed the presence of amyloid-like deposits with apple-green birefringence under polarized light (Fig. ). A final diagnosis of a peripheral CEOT rich in clear cells was reached. No complications were observed in the postoperative appointment and a follow-up schedule was established. The patient has had no recurrence after 22 months (Fig. ). |
Our patient is a 54-year-old Caucasian male with a history of cardiac and pulmonary sarcoidosis, hypertension, premature ventricular contractions (PVCs), and obesity who presented with acute onset right hemianopsia, memory recall difficulty, and alexia without agraphia. He was in his normal state of health and doing yard work when the symptoms began.\nIn the past year, incidental PVCs were found on 12-lead electrocardiogram (ECG) during a preoperative evaluation for dental work. Holter monitoring revealed a 12% PVC burden over 24 hours, indicating an indeterminate degree of ventricular dysfunction. Cardiac evaluation of the PVCs included transthoracic echocardiogram which revealed hypokinesis of the left inferior ventricular wall with an ejection fraction of 35%. Cardiac catheterization for investigation of structural blockages of coronary vessels yielded no significant CAD.\nThese findings were suspicious for an infiltrative process. This hypothesis was supported by cardiac MRI showing sarcoid infiltrates on T2-weighted images and by discovery of noncaseating granulomas on pulmonary node biopsy. He was diagnosed with cardiac sarcoidosis three months after initial presentation. During this time, he showed no clinical symptoms of systemic sarcoidosis or heart failure. An implantable cardioverter defibrillator was placed for primary prevention of arrhythmias secondary to cardiac sarcoid. He was doing well for one year until he presented with stroke symptoms.\nOur patient endorsed decreased vision on the right and described the words on his lawn mower being visible but not readable. He also acknowledged trouble with recalling names and specific events.\nUpon examination, our patient demonstrated normal speech and language. He was asked to write a simple sentence and performed the task without difficulty. When asked to read the sentence, he was unable to do so, representing alexia without agraphia. He showed right homonymous hemianopia. The remainder of the physical exam was normal.\nAt admission, initial computed tomography (CT) scan was negative. Subsequent brain magnetic resonance imaging (MRI) and magnetic resonance angiogram (MRA) confirmed a left posterior cerebral artery (PCA) infarction (). MRA of the neck was unremarkable. The stroke was suspected to be cardioembolic in origin due the PVC burden and reduced ejection fraction of 35% promoting possible thrombus formation. Transesophageal echocardiogram revealed no thrombus or patent foramen ovale (PFO) and supported the previous finding of hypokinesis of left inferolateral ventricular wall (). Hypercoagulable workup was nonrevealing. There was no family history of sarcoidosis or early age stroke.\nBy the time of discharge, our patient's visual symptoms returned to baseline and he was given high dose aspirin and atorvastatin for secondary stroke prophylaxis. Cellcept and prednisone were prescribed for management of sarcoidosis. A LINQ device was placed for continuous ECG monitoring. Lisinopril and metoprolol were maintained for pressure and rhythm control. |
A 63-year-old Chinese female was admitted to our clinic with progressive exertional dyspnea, and upper limb and facial edema of 60 days duration. There were no clinical signs of myasthenia gravis. An enhanced chest computed tomography (CT) scan revealed a 77 mm × 67 mm irregular anterior mediastinal mass with heterogeneous enhancement compressing the mediastinal great vessels, invading the LBCV and growing into the right atrium, associated with intraluminal filling defects. Multiple collateral vessels were seen in the right anterior chest wall (Fig ). Fine needle aspiration (FNA) was carried out and revealed a diagnosis of thymoma.\nThe patient underwent a radical excision of the mediastinal mass, the invaded parts of the mediastinal pleura and the pericardium, double partial upper lobes, SVC, LBCV and RBCV, with reconstructions of LBCV and RBCV to the right atrium with grafts in August 2011. Intraoperatively, the mass was located in the superior anterior mediastinum and encased the LBCV. It invaded into the right atrium through the LBCV, RBCV and SVC and formed tumor thrombi. Pathologically, the tumor was described as a huge grayish yellow tough mass of 12 cm × 8.5 cm × 7 cm in size (Fig ). Microscopically, the tumor consisted of neoplastic epithelial cells and non-neoplastic lymphocytes. Most areas were type B2 thymoma, epithelial cells were arranged in sheets or cords, and some lymphocytes could be seen between tumor cells (Fig ). The tumour had a highly vascular appearance. Some areas were type B1 thymoma (lymphocyte-rich thymoma), and a few neoplastic epithelial cells could be seen against a background of lymphocytes. Immunohistochemical staining showed that CK19 was positive for epithelial cells. CD99, TdT, CD5, CD3 and Ki67 were positive for lymphocytes. Histopathological diagnosis indicated a mixed pattern of type B1 and type B2 thymoma, predominantly type B2 thymoma (WHO classification). The tumor stage was IVA due to the pleural and pericardial dissemination (Masaoka stage). The patient was discharged from hospital on postoperative Day 18 and without local recurrence or distant metastasis after 50 months of follow-up. |
A 40-year-old female patient presented to our Orthopedic department with complaints of pain, infection, and foul-smelling discharge from the left shoulder region along with limitations in left shoulder joint movements (Figure ).\nShe was facing social stigma and boycott due to her ailment. A detailed past medical history from the patient revealed a history of multiple surgeries in the left shoulder region from different hospitals. Around 20 years ago (2000), the patient had experienced pain in the left shoulder following trivial trauma. On radiological evaluation, it had been diagnosed as a pathological fracture with a well-defined lytic lesion in the proximal humerus (Figure ).\nThe lesion had been treated with curettage and iliac crest grafting at that time. The histological examination of curettage tissue had confirmed the diagnosis of GCT (Figure ).\nAfter treatment, the patient had been almost asymptomatic for three years when, in 2003, she had again developed gradual onset of pain in the same region, which had been diagnosed as a case of recurrent GCT on radiological assessment (Figure ).\nThe patient had then undergone extended curettage followed by fibular strut graft and bone substitutes filling in the bone void (Figure ).\nPostoperatively, the patient had developed an infection; she had been discharged and managed with conservative treatment (antibiotics and dressing). However, five years later (2008), the tumour had recurred again (Figure ).\nExtensive curettage and cementation of the cavity were performed (Figure ).\nHowever, postoperative infection occurred this time as well, which progressed to a persistently draining sinus. Due to repetitive infections, the patient had lost hope and enthusiasm and had not approached any hospital for the next 10 years when she presented herself to our OPD in 2018 with a complaint of discharging wound and skin defect in the left shoulder region.\nA non-healing wound of approximately 5 x 5 cm with underlying exposed bone cement and purulent discharge was present in the left deltoid region on clinical examination (Figure ). Scar marks of previous surgeries were also present. Radiologic, hematologic, and microbiology investigations were performed. The radiograph showed a cavity filled with bone cement in the proximal humerus with a radiolucent area abutting its lower end (Figure ).\nInflammatory markers such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were also raised. Methicillin-sensitive Staphylococcus aureus (MSSA) and Streptococcus pyogenes grew in wound culture. The patient was started on intravenous clindamycin (300 mg qid) and cefuroxime (500 mg bid) for two weeks; however, the infection did not resolve. She was labeled as a case of infected recurrent GCT. A two-stage treatment was scheduled with a plan to eradicate the infection and excision of the lesion in the first stage, followed by the reconstruction of the defect with a customized mega prosthesis in the second stage.\nFirst-stage operation\nOn the basis of preoperative radiological examination, wide local excision was planned including 1 cm of healthy bone margin. Under general anaesthesia, the patient underwent en-bloc excision (15 cms) of the proximal humerus and cement including a 5-mm healthy skin margin around the wound through the previously healed scar of the deltopectoral approach (Figure ).\nExtensive debridement of nonviable infected tissue was done, including scared deltoid and rotator cuff. The wound (bone void) was thoroughly irrigated with 5% povidone-iodine and hydrogen peroxide solution and temporarily filled with an antibiotic spacer. The antibiotic spacer was made on the OT table with bone cement that contained vancomycin (4 g/40 mg), gentamicin (80 mg in 2-ml solution), and Steinmann pin (4.5) as the core (Figure ).\nMultiple Provisional holes were created in its proximal part for temporary fixation of muscle to maintaining the length of muscle and capsule cover. The wound was closed in a layered manner over a negative suction drain to prevent postoperative collection formation and removed after 48 hours. Antibiotic spacer eluted antibiotics in the local tissue environment, which helped to control the infection. Postoperatively, the patient did not report any local or systemic complications. There was no wound dehiscence or discharge, and the suture line had healed completely. Her ESR and CRP also reduced to baseline levels. The patient was instructed to report after three months for the second-stage surgery.\nSecond-stage operation\nThe proximal humerus region was exposed through the previous deltopectoral incision. An induced membrane was found around the spacer; however, there was no granulation tissue around it. Antibiotic cement spacer was removed, and the cavity was thoroughly cleaned with betadine and hydrogen peroxide aseptic solution. Distal humerus shaft was prepared with sequential reaming up to 7 mm in size. The bony defect was measured, and a modular proximal humerus reconstruction implant of appropriate size was selected (Restor humerus IM stem of 7-mm diameter and 80-mm length, Restor resection piece 75 mm, Restor humeral head; Modular Resection Prosthesis, Syncera, Smith & Nephew, London, UK). The implant's stem was fixed with PMMA gentamicin cement, and the rest of the implant components were assembled in appropriate rotation. The shoulder joint was reduced and covered with the fibrous capsule. Skin and subcutaneous tissues were closed in a layered manner over a drain. The drain was removed two days after surgery. No postoperative complications were present in the patient. Hand and elbow physiotherapy was started on the second postoperative day. However, the shoulder was kept immobilized with a brace for three weeks before physiotherapy.\nPatient follow-up after 2.5 years (2020)\nAfter 2.5 years of treatment at our institution, the patient is currently asymptomatic, and the suture line is healthy. She does not have any clinical signs of infection and has a full range of motion at hand and elbow. The shoulder joint is stable; however, there is no active movement. The radiograph below shows the humerus stem well-seated without any cement-bone interface radiolucency or lysis of the bone (Figure ).\nInflammatory markers such as ESR and CRP are within the normal range. She is able to perform household activities and personal care without any assistance. Figure shows the clinical photograph of the patient’s stable shoulder. |
A 59-year-old man, diagnosed with bipolar disorder I for many years, presented with an episode of bipolar depression with symptoms such as anhedonia, depressed mood, reduced energy, psychomotor retardation, demotivation, anxiety, and decreased social activity, which had been gradually worsening for two weeks. Significant impairment in his usual functioning was noted, given that the patient lost the interest and ability to work in the projects that excited him the most, for example, he abandoned the writing of a new book (months before, when stable, he managed to translate a book he had already published). He also stopped taking care of his diet, with less and less elaborate meals. These symptoms were interpreted as an episode of bipolar depression. At that time, he was being treated at the day hospital of the psychiatric department and was medicated with semisodium valproate 1500 mg id, olanzapine 10 mg 2id, aripiprazole 10 mg id, and lorazepam 2.5 mg 3id.\nAbout six months before this episode, the patient was hospitalized for a period of six weeks following a suicide attempt through self-induced deep cuts in the upper limbs, with severe bleeding that motivated hospitalization in the intensive care unit. This event occurred in the context of severe depression with psychotic characteristics (with delusional ideas of guilt and ruin). In that time, medication was adjusted with the introduction of fluoxetine 20 mg id, olanzapine 10 mg id, and diazepam 5 mg 2id (in addition to semisodium valproate and lorazepam, which he was already taking). Initially, a clinical improvement was noticed, but, soon after discharge, he had a subsequent manic switch, with elevated mood, increased energy, psychomotor agitation, verborrea, disinhibition, involvement in new projects, and regular cocaine consumption. Thus, the medication was progressively readjusted and cessation of cocaine consumption was promoted, with progressive clinical improvement, until he turned to the depressive mood described at the beginning of this case report.\nIn addition to this recent psychiatric history, the patient had already been hospitalized six times in psychiatry wards due to decompensation of his bipolar disorder. In some of these hospitalizations, he also presented with mental and behavioral disorders induced by psychoactive substance use, which normally takes place after the installation of elevated mood and disinhibition. In fact, he has a history of drug abuse, mainly cocaine but also cannabinoids, heroin, and alcohol. His medical history also includes hypertension, treated with lisinopril 10 mg id and amlodipine 5 mg id.\nFacing the current depressive episode, lab tests were carried out and no abnormalities were found in blood cell count, renal function, electrolytes, liver function, folic acid, B12 vitamin, total cholesterol, and glucose. Valproate levels were 61.7 mg/L and urine drug screening was negative. Regarding the treatment, it must be taken into account that, in previous depressive episodes, the patient responded poorly to quetiapine and reported serious adverse effects of lithium, such as muscle twitch and trembling, pointing out his low tolerability to the drug. Given this background, the decision was to start lamotrigine in titration up to 25 mg id, in addition to the semisodium valproate he was already taking. We also decided to suspend aripiprazole.\nAbout three to four weeks after initiating lamotrigine, the patient presented with an expansive mood, sudden involvement in new projects (intention of publishing new books), increased energy, easy social interactions, disinhibition, fast speech, and insomnia. At that time, the dose of lamotrigine was 25 mg id, and the taking of the medication was supervised by the nurses in the day hospital. The patient himself early recognized that his mood was unwell, explaining his clinical condition with the introduction of lamotrigine: "lamotrigine is like methamphetamine, I am no longer depressed." After the onset of this manic episode, the patient resumed cocaine use and, at this point, we must highlight that the consumption only began after the exacerbation of manic symptoms, given that the previous drug screenings, regularly performed, were consistently negative. Therefore, lamotrigine was gradually discontinued, aripiprazole 10 mg was reintroduced, sodium valproate dose was increased to 2 g id, and flurazepam 30mg id was started. At the same time, cocaine use was discouraged again, and the patient stopped using it. About two weeks after the discontinuation of lamotrigine, a gradual clinical stabilization was observed, with a return to euthymic mood and normal speech debt, stable sleep and appetite, and clear absence of psychotic activity or suicidal ideation, overlapping his previous functioning. |
An 18-year-old male patient, who was diagnosed with uncontrolled hypertension four years ago, was referred to our pain center by a cardiologist. Four antihypertensive drugs were used (nifedipine 30 mg, chlorthalidone 25 mg, doxazosin 4 mg, and minoxidil 5 mg). In addition, irbesartan 150 mg, spironolactone 25 mg, verapamil 240 mg, and carvedilol 25 mg were administered. However, his hypertension could not be controlled; his systolic BP was >170–180 mmHg, occasionally >200 mmHg. The cardiologist at our institution evaluated the causes of hypertension to exclude secondary hypertension, and concluded that he had essential hypertension. The cardiologist considered that the main factor in the resistance to controlling hypertension was obesity (weight: 106 kg, height: 177 cm, body mass index (BMI): 33.8). However, the weight loss strategy failed. Two years ago, when the patient was 16 years old, the cardiologist tried a new approach, the renal denervation procedure. However, there was no significant improvement in his blood pressure. The patient was referred to our pain center for a celiac plexus block (CPB) six months ago and we performed a prognostic CPB with local anesthetics (1% lidocaine 10 cc per side). We performed a fluoroscopic-guided CPB using the bilateral paravertebral posterior approach. The needle was advanced alongside the vertebral body to avoid the transverse process, visceral organs, and vascular structures. After diffusion of contrast material was confirmed, local anesthetics were injected. One h after the procedure, the systolic and diastolic BP dropped to ~150 mmHg and ~90 mmHg, respectively. The BP was maintained at a similar level for four days. It was checked hourly for six hours and four times a day for the next four days. Then the systolic BP increased to 170 mmHg. This result confirmed the short-term effect of the CPB procedure in terms of a decrease in BP. We planned CPB with botulinum toxin at the next follow-up and performed CPB with Botox® (botulinum toxin 50 IU, two vials containing a total of 100 IU, Allergan Inc., Irvine, CA, USA) in the patient (). The patient’s systolic BP decreased to ~150 mmHg within 30 min.\nDuring the one-month observation period after CPB using Botox, the patient’s systolic and diastolic BP were controlled at ~150/90 mmHg using the medications mentioned above, with the exception of on one occasion (BP was 170/100 mmHg). Three months after the first injection, we performed a second CPB using the same dose of botulinum toxin (). The patient’s systolic BP declined to ~150 mmHg and has been controlled at an appropriate level for four months. |
A 50-year-old female with a history of myelofibrosis status post allogeneic peripheral blood stem cell transplantation complicated by graft-versus-host disease and hemolytic anemia secondary to major ABO incompatibility on prednisone, tacrolimus, and rituximab presented to our emergency department with complaints of shortness of breath and abdominal distension. Upon evaluation, the patient was found to be tachypneic, tachycardic, and requiring supplemental oxygen via nasal cannula. Abdominal examination was notable for mild left lower quadrant tenderness with no peritoneal signs. Laboratory evidence demonstrated pancytopenia (WBC 3.0 × 109 cells/L, Hgb 6.2 g/dL, Plts < 2 × 109 cells/L), elevated lactic acid, mildly elevated transaminases, and acute hyperbilirubinemia. The patient was transfused platelets and packed red blood cells in addition to crystalloid fluid. Blood cultures were obtained followed by the initiation of broad-spectrum antibiotics with ceftazidime and vancomycin. A computed tomography (CT) scan of the chest, abdomen and pelvis with intravenous contrast was obtained. The CT scan of the chest was notable for bilateral peripheral and basilar predominant ground glass and consolidative pulmonary opacities most concerning for atypical or viral pneumonia. The CT of the abdomen and pelvis () demonstrated an enlarged, 20 cm spleen with multiple wedge-shaped regions of hypoattenuation consistent with acute splenic infarcts. A dominant wedge-shaped area in the posterior superior spleen containing a significant amount of intrasplenic and subcapsular air was noted with continuation of the air tracking into the splenic vein and the portal venous system. These findings were most consistent with splenic necrosis. The patient was admitted to the medical intensive care unit for further management of septic shock.\nShortly upon arrival to the medical intensive care unit, the patient required intubation for increased work of breathing in the setting of septic shock. Infectious disease, acute care surgery, interventional radiology, hematology, and the bone marrow transplant services were all consulted to assist with management. Within less than 12 hours, the patient's blood cultures were positive for gram variable rods in all bottles. The patient's antibiotics were broadened to meropenem, vancomycin, and doxycycline. Given her underlying comorbidities, micafungin was also initiated out of concern for possible fungal infection. Acute care surgery and interventional radiology deemed the patient unsuitable for any immediate invasive intervention secondary to the patient's profound and refractory thrombocytopenia (platelet count < 2 × 109 cells/L) and anemia (hemoglobin 5.8 g/dL) which did not improve despite multiple transfusions.\nOver the next 24 hours, in hopes of preventing ongoing hemolytic anemia, the patient underwent her first out of five exchange transfusions which was performed without complication. At 48 hours after admission, the patient's initial blood cultures speciated to Clostridium perfringens. The respiratory viral panel, SARS-CoV-2 PCR, and the BIOFIRE® FILMARRAY® Pneumonia plus Panel were all negative. Vancomycin and doxycycline were discontinued while micafungin and meropenem were continued given high concern for additional enteric organism involvement.\nNinety-six hours after admission, the patient remained pancytopenic. Colony-stimulating growth factor was given in an attempt to improve her leukopenia. A repeat CT scan of the abdomen and pelvis demonstrated decreased splenic gas with resolution of portal venous and splenic vein gas. No discrete splenic abscess was identified. A transthoracic echocardiogram was negative for valvular vegetations. The second set of blood cultures obtained 48 hours after admission was no growth to date. The patient was hemodynamically stable without the need for vasopressors and was on room air after a self-extubation event.\nDespite such medical treatment, the patient's third set of collected blood cultures for the hospitalization returned positive for Clostridium perfringens on hospital day six. Given high concern for lack of source control, a third CT abdomen and pelvis was performed which more clearly demonstrated a large splenic abscess. Given improvement in the patient's leukopenia, hemodynamics, and now available HLA matched platelets, interventional radiology and surgery were reengaged for possible intervention. On hospital day eight, the patient underwent successful percutaneous splenic drain placement. Intraoperative cultures collected from the spleen grew Clostridium perfringens.\nThe percutaneous splenic drain was removed on hospital day 23 after CT imaging demonstrated improvement, but not complete resolution of the splenic abscess. The patient's antibiotics were narrowed to ertapenem with an anticipated six-week course. The patient's remaining hospital course was complicated by acute encephalopathy, persistent pancytopenia requiring stem cell boost, and stercoral colitis. Ultimately, the patient was discharged to a rehabilitation facility on hospital day 40.\nTwenty-seven days after discharge from the hospital and eleven days after the completion of her six-week course of ertapenem, the patient returned to the hospital febrile, tachycardic, and with a CT abdomen and pelvis demonstrating reaccumulation of splenic air and fluid. Blood cultures were again positive for Clostridium perfringens. Despite the risks given the patient's pancytopenia, the patient underwent preoperative splenic embolization with interventional radiology followed by a total splenectomy the following day by acute care surgery. The patient spent several days in the surgical intensive care unit but was ultimately discharged from the hospital on postoperative day 11. Pathology from the spleen demonstrated a 12.5 × 8.5 × 7.5 cm abscess cavity. Microbiology performed on the spleen grew Clostridium perfringens. Blood cultures collected after splenectomy remained negative for Clostridium perfringens suggesting appropriate source control. |
A 78-year-old Korean woman visited our hospital complaining of hoarseness, which had developed 1 month previously. Our patient had an unremarkable medical history except she was taking antihypertensive medication. Flexible laryngoscopy detected a mass approximately 1 cm in diameter on the posterior wall of her subglottic region that blocked approximately 80 % of her trachea (Fig. ). Neck computed tomography, without limiting her vocal cord movement, was performed owing to the suspected malignant tumor in her subglottic region. A well-localized 1 × 0.8 cm polypoid mass was detected in the posterior wall of her subglottic region (Fig. ).\nOur patient underwent a bronchoscopic biopsy using a bronchus endoscope. Examination of the specimen showed the presence of epithelial and myoepithelial cells. Consequently, a tracheostomy and laryngeal microsurgery were performed under general anesthesia for a final biopsy and complete excision, with consideration given to the signs and symptoms of tracheal obstruction.\nA laryngeal microscopic examination revealed the round 1 × 0.8 cm protruding mass at the bottom of the interarytenoid space. Above this, the 0.8 × 0.8 cm mass protruded like a spire. Complete excision of the masses was possible using a carbon dioxide laser because there was no adhesion between the masses and the surrounding larynx (Fig. ).\nThe gross findings of a histopathological examination were of ash-colored masses and a microscopic examination showed a tube-shaped structure composed of two layers. The inner layer was an oval or round monolayer of cells and the outer layer had polygonal cells with transparent cytoplasm (Fig. ). Because the transparent myoepithelial cells in the outer layer showed weak-positive immunohistochemical staining for smooth muscle actin (SMA) and p63, and the epithelial cells were strongly positive for low molecular cytokeratin, epithelial-myoepithelial carcinoma was diagnosed (Fig. ).\nThere were no complications, and our patient was discharged from hospital 10 days after surgery. Although additional radiation treatment was planned to prevent local recurrence, our patient refused this treatment. At the time of writing, her condition is being monitored by our outpatient clinic. There were no unusual findings 1 year after surgery. |
A 23-year-old Caucasian man with no medical history was referred to our emergency department by his primary care physician because of swelling and erythema of the little finger of his left hand for ten days prior to presentation. He had been prescribed the antibiotic Augmentin® (amoxicillin-clavulanic acid), and the primary care physician had already incised the finger releasing clear liquid (no pus). Despite this therapy, his complaints persisted. He had no fever or itching, and had never had these complaints before (Figures and ).\nWe confirmed the erythema and swelling of the distal phalanx of his left little finger, with vesicles with a yellow translucent colour. From these vesicles, a clear fluid spontaneously discharged. There was no pus, bony tenderness or pain over his flexor tendons. The motion of his finger was unlimited, and he had no fever (Figures and ).\nOn the basis of the clinical appearance we considered herpetic whitlow with superinfection. When asked, he confirmed he had also observed vesicles on his genitals. He denied ever having sexual intercourse or contact with infected individuals. A polymerase chain reaction on herpes simplex virus type 1 was positive on both the material from his genital vesicles and on material from his finger. We referred him to a dermatologist for further treatment. Because of spontaneous subsidence of his complaints, Fucidine® (fusidic acid) cream was chosen as primary treatment. In other cases, in which subsidence does not occur spontaneously, antiviral agents such as acyclovir or valacyclovir may also be used.\nThe herpes simplex virus causes two types of infections: primary and recurrent. Usually, a break in the skin barrier (for example a wound) allows the virus to enter the tissue and establish an infection. Appearing several days after a person's first exposure, the sores of a primary infection last approximately 1 to 3 weeks. They heal completely, rarely leaving scars. Nevertheless, after the primary infection, the virus remains in the body, hibernating in nerve cells. Certain triggers can cause the hibernating (latent) virus to become active and travel back to the skin. Recurrent infections tend to be milder than primary infections, and generally occur in the same location as the primary infection [,]. |
A 45-year-old male was referred to our center for changes in his facial appearance with ocular asymmetry started 3 months before. No previous records of trauma or surgical procedures were reported in paranasal sinuses. The patient never complained of symptoms of acute or chronic rhinosinusitis. The clinical and ophthalmological examination revealed a 3 mm left enophthalmous and hypoglobus with neither visual deficits nor diplopia.\nThe patient underwent a computed tomography (CT) of paranasal sinuses which showed opacification and hypoplasia of left maxillary sinus, lateralization of the uncinate process, and depression of homolateral orbital floor. A bilateral concha bullosa of the middle turbinate was also present (,:-). Moreover, left SSS was diagnosed, and the patient underwent functional endoscopic sinus surgery. Antrostomy was performed with retrograde uncinectomy together with the aspiration of mucoid secretion in the left maxillary sinus followed by the removal of the lateral lamella of the concha bullosa of the middle turbinate on both sides. The patient showed the regression of the aesthetic defect and improvement of the left enophthalmos and hypoglobus six months after surgery. A post-operation CT scan was performed 10 months after surgery and allowed to find a complete re-ventilation of the left maxillary sinus with normalization of the maxillary sinus walls which appeared thin and demineralized before the intervention. On the right side instead, an initial and mild lateralization of the uncinate process could be identified with a still well-ventilated maxillary sinus (,: -).\nAfter 24 months post-surgery, the patient underwent a CT scan for other reasons, and a complete right maxillary sinus opacification was observed with lateral displacement of the uncinate process and a thinning of the maxillary sinus bone walls, especially the posterior and superior ones. A lowering of the orbital floor was also present, compared to the contralateral side; therefore, diagnosis of right SSS was made (,:-).\nRight endoscopic uncinectomy and middle meatotomy were performed, and marked implosion of the sinus walls was found with the presence of dense mucoid secretions. A CT scan performed after 5 years showed well-ventilated maxillary sinuses, bone reposition in the orbital floors and maxillary sinus walls, partial re-expansion of the sinuses, and repositioning of the eyeballs at the same level (,;-). |
Our patient is a 54-year-old Caucasian male with a history of cardiac and pulmonary sarcoidosis, hypertension, premature ventricular contractions (PVCs), and obesity who presented with acute onset right hemianopsia, memory recall difficulty, and alexia without agraphia. He was in his normal state of health and doing yard work when the symptoms began.\nIn the past year, incidental PVCs were found on 12-lead electrocardiogram (ECG) during a preoperative evaluation for dental work. Holter monitoring revealed a 12% PVC burden over 24 hours, indicating an indeterminate degree of ventricular dysfunction. Cardiac evaluation of the PVCs included transthoracic echocardiogram which revealed hypokinesis of the left inferior ventricular wall with an ejection fraction of 35%. Cardiac catheterization for investigation of structural blockages of coronary vessels yielded no significant CAD.\nThese findings were suspicious for an infiltrative process. This hypothesis was supported by cardiac MRI showing sarcoid infiltrates on T2-weighted images and by discovery of noncaseating granulomas on pulmonary node biopsy. He was diagnosed with cardiac sarcoidosis three months after initial presentation. During this time, he showed no clinical symptoms of systemic sarcoidosis or heart failure. An implantable cardioverter defibrillator was placed for primary prevention of arrhythmias secondary to cardiac sarcoid. He was doing well for one year until he presented with stroke symptoms.\nOur patient endorsed decreased vision on the right and described the words on his lawn mower being visible but not readable. He also acknowledged trouble with recalling names and specific events.\nUpon examination, our patient demonstrated normal speech and language. He was asked to write a simple sentence and performed the task without difficulty. When asked to read the sentence, he was unable to do so, representing alexia without agraphia. He showed right homonymous hemianopia. The remainder of the physical exam was normal.\nAt admission, initial computed tomography (CT) scan was negative. Subsequent brain magnetic resonance imaging (MRI) and magnetic resonance angiogram (MRA) confirmed a left posterior cerebral artery (PCA) infarction (). MRA of the neck was unremarkable. The stroke was suspected to be cardioembolic in origin due the PVC burden and reduced ejection fraction of 35% promoting possible thrombus formation. Transesophageal echocardiogram revealed no thrombus or patent foramen ovale (PFO) and supported the previous finding of hypokinesis of left inferolateral ventricular wall (). Hypercoagulable workup was nonrevealing. There was no family history of sarcoidosis or early age stroke.\nBy the time of discharge, our patient's visual symptoms returned to baseline and he was given high dose aspirin and atorvastatin for secondary stroke prophylaxis. Cellcept and prednisone were prescribed for management of sarcoidosis. A LINQ device was placed for continuous ECG monitoring. Lisinopril and metoprolol were maintained for pressure and rhythm control. |
A 54-year-old woman presented with sudden-onset loss of consciousness followed by repeated generalized seizures. During ambulance transport, she was drowsy but able to protect her airway. On admission to the National Brain Aneurysm Center, she suffered another seizure. An intravenous anticonvulsant load was administered, and she was emergently intubated. Admission CT-scan revealed a hyperdense 2 × 3 cm2 lesion involving the temporoparietal junction with an associated 7 mm high attenuation nidus at its anterior margin. This lesion was surrounded by vasogenic edema []. MRA demonstrated the presence of a 2 mm presumed mycotic aneurysm arising from a distal M3 branch of the right MCA. Cerebral angiogram confirmed the small, partially thrombosed aneurysm arising from a large posterior division M3 segment. The filling portion of the aneurysm measured 2 × 2.2 × 1.5 mm3 []. The involved arterial segment continued beyond the aneurysm to supply a significant portion of the nondominant posterior frontal and anterior parietal regions.\nA thorough medical evaluation disclosed no evidence of fever, and the white blood cell count and tagged white blood cell exam were unremarkable. Her erythrocyte sedimentation rate was within the normal limit, but her C-reactive protein was elevated. Cardiology consult, chest, and abdominal CT-scan were all noncontributory. It was felt that this lesion most likely represented a mycotic aneurysm, and after combined consultations with the neurovascular and infectious disease services, a decision was made to proceed with treatment of the aneurysm. Careful study and evaluation of the aneurysm outflow revealed important supply to the nondominant motor cortex. Thus, simple sacrifice of the involved arterial segment seemed ill-advised. In addition, it was unclear how straightforward it would be to locate the aneurysm within the sylvian fissure or whether the involved segment could be primarily repaired once identified. Due to the poor caliber of the posterior division STA, a traditional extracranial-intracranial (EC–IC) bypass was not feasible. Therefore, we decided to perform an IC–IC short jump graft bypass in preparation for endovascular sacrifice of the parent vessel related to the aneurysmal segment.\nAt surgery, the anterior division of the STA was exposed and dissected over an adequate length, and a generous craniotomy was performed. Intraoperative angiography was utilized to localize a cortical artery arising from the involved segment as well as a nearby cortical artery arising from a distinct, uninvolved MCA branch. A segment of the STA was harvested, and then 10-0 suture was utilized to anastomose this short segment to both the involved and normal cortical arteries, both as end-to-side grafts []. This created a short jump graft allowing for subsequent sacrifice of the diseased artery. Intraoperative angiogram after completion of the bypass demonstrated patency of the jump graft [].\nFollowing surgery, the patient was taken directly to the biplane-suite for coil embolization of the parent right MCA-M3 posterior division at the level of the aneurysm []. Postoperatively, the patient remained neurologically stable, and the remainder of her hospital course was unexceptional. She was discharged home in good condition. |
A 45-year-old male patient came to the department with the chief complaint of loose teeth in upper and lower front jaw regions since 1 year with swollen and bleeding gums. Patient first noted bead like nodular growth over the gums which progressively enlarged to the present size covering almost entire teeth interfering with further cleaning of teeth.\nThe patient was hypertensive since 1.5 years and was under medication Coronol-AM (atenolol, 50 mg + amlodipine, 5 mg) once daily. He denied the history of any adverse habits.\nThe patient was moderately built and nourished with no signs of anaemia and jaundice and noncyanosed. His vital signs were within the normal range.\nIntraoral examination revealed generalized enlargement of attached gingival extending up to marginal and interdental gingiva. Surface of the gingiva appears lobulated with loss of scalloping (). Poor oral hygiene status of patient was assessed by the presence of local irritating factors which surrounded the teeth.\nBased on drug history and clinical examination of the patient provisional diagnosis of combined gingival enlargement was made. Complete hemogram of the patient was done, but all the parameters were within the normal range. Orthopantomogram was taken which revealed generalized bone loss ().\nAfter this, incisional biopsy was done. Histopathological report revealed few areas of hyperplastic orthokeratinized and parakeratinised stratified squamous epithelium and connective tissue exhibiting mixture of dense and loose fibrous component. Inflammatory cell infiltrate with PMLs and dilated blood capillaries with few areas of calcifications were also evident.\nCorrelating history, clinical examination, and investigations, final diagnosis of combined gingival enlargement (amlodipine induced and inflammatory) was made. Patient was referred to periodontics department for further treatment. In the preliminary phase, extraction of teeth (11, 21, 22, 23, 31, 32, and 34) with hopeless prognosis was recommended. Planned sessions of scaling and root planning with drug change with the patient's physician consent were performed. Patient was put on tablet Normadate 100 mg twice daily and was evaluated after the period of 1.5 months. There was drastic change in the clinical picture of gingiva with complete loss of inflammatory component (). |
The second case refers to a 48-year-old woman, a busy manager with a history of depression and sleep disturbance. She has had three terminations of pregnancy and one delivery by cesarean section. She smokes approximately ten cigarettes per day and has high cholesterol serum levels. She takes several medications: a selective serotonin reuptake inhibitor (escitalopram), two benzodiazepines (delorazepam and clonazepam), and a statin. She reports a four-year history of urinary symptoms: daily UUI episodes, mild stress urinary incontinence (SUI), and two episodes of nocturia per night. She wears pads every day. The urology consultation revealed some degree of pelvic pain, especially during vaginal examination. The urine dipstick was negative and there was no PVR. No specific causes of the symptoms such as urine tract infection were identified. The patient also complained of mild dyspareunia and occasional constipation. The urine culture turned out to be sterile, with no blood in urine, and the pelvic ultrasound scan and urine cytology were also negative. The cystoscopy, which was performed as a result of the presence of storage symptoms and to rule out a bladder tumor in this current smoker, was normal.\nIn OAB patients, it is of utmost importance to consider all comorbidities. Anxiety and depression may play a role, feeding a vicious circle. Moreover, medications to treat neurological or psychiatric disorders can influence OAB and be responsible for side effects [, ]. Gastrointestinal disorders are frequently associated with OAB, such as constipation in this case, but patients rarely raise the topic. An overlap exists between irritable bowel syndrome and OAB [].\nThe patient was prescribed a β3 agonist, pelvic floor muscle training (PFMT) and bladder retraining. Four months later, she noticed some degree of improvement, but had stopped the treatment as she felt that she had no time for PFMT. She was not compliant with the bladder drill either, and soon stopped the β3 agonist because she did not sense any real improvement. She also felt that she did not have the time to complete a bladder diary. She was prescribed fesoterodine 8 mg for three months. In parallel, her general practitioner asked for vaginal and urethral culture swabs, which were negative. After three months, her urinary urgency improved, but she said that the few remaining episodes of urgency were “killing her life” and that she did not want to be on pills for her whole life. Therefore, she refused to continue the treatment and requested an “easy fix”. Her reaction highlights the need for careful consideration of the consequences of incontinence in terms of QoL. A publication from Vaughan et al. [] reported that OAB and incontinence synergize to reduce QoL, especially in the domains of sleep, elimination, usual activities, discomfort, distress, vitality, and sexual activity.\nConsistent efficacy on urgency symptoms with a significant decrease in UUI and urgency episodes has been reported with fesoterodine at doses of 4 and 8 mg compared to placebo () [, , , ]; however, some patients may react differently. Patient satisfaction is an important driver of treatment success []. Patient expectations should be considered carefully in the context of OAB management. The achievement of patients' goals was measured in the Study Assessing FlexIble-dose fesoterodiNe in Adults (SAFINA study) [], a 12-week multicenter open label study with 331 OAB adults, using the Self-Assessment Goal Achievement (SAGA) questionnaire. Fesoterodine treatment resulted in 81.3% of patients declaring that their goals were “somewhat achieved/achieved” or that the result “exceeded/greatly exceeded their expectation”.\nOur case patient had very specific expectations; she refused to have an implant (neuromodulation), saying “I'm not going to be an android!” She accepted botox injections, and so a first set of injections was performed under local anesthesia. She found the injections “a little painful” and “a big annoyance”, but at the one-month follow-up visit after botox injection she reported no more UUI episodes and an improvement in frequency and the number of urgency episodes, as well as in QoL. Even though she stated that she did not like the idea of being a patient for the rest of her life, she accepted subsequent injections.\nThe clinical points that can be learned from this case are as follows:All OAB cases are different, and a thorough evaluation is mandatory to adequately address each case. It is important to assess other aspects, such as functional and psychological disorders that may influence symptoms, and to consider nonneurogenic OAB as a multifactorial disease. The major goal of initial therapy is to meet the patient's expectations regarding the reason for their visit, to improve their satisfaction, and their QoL. Due to fesoterodine's characteristics and flexible dosage, improvement of symptoms and achievement of the patients' goal are usually high with this medication. When patients have specific requirements, all options should be discussed and the patient's agreement obtained. A customized approach is a crucial factor for treatment success. OAB management should be personalized; beware of a simplistic application of a standardized treatment algorithm. |
In early March 2020, an 85-year-old male presented to clinic for follow-up after being diagnosed with congestive heart failure in the emergency department one month prior. His presenting symptoms at that time were shortness of breath on exertion as well as orthopnea. Electrocardiogram demonstrated sinus rhythm with first degree AV block as well as a left bundle branch block (LBBB) that had been documented previously in 2017. The chest x-ray showed mild pulmonary edema as interpreted by the emergency room physician. BNP levels are not available in the region where this patient resides. Medications started in the emergency department included furosemide 20 mg daily as well as ramipril 2.5 mg daily. The patient stated he felt much better and only experienced mild dyspnea with prolonged exertion. Physical exam was unremarkable and vital signs were within normal limits. The patient has a history of benign prostatic hyperplasia and gastroesophageal reflux for which he takes dutasteride 0.5 mg daily, tamsulosin 0.8 mg daily, and rabeprazole 20 mg daily. There is no other past medical history, specifically no diabetes, hypertension, dyslipidemia, kidney disease, sleep apnea or anemia. He quit smoking over 50 years ago and his body mass index is normal. Laboratory investigations immediately prior to starting furosemide and ramipril showed a serum creatinine of 105 µmol/L, sodium of 139 mmol/L, potassium of 4.9 mmol/L, hemoglobin of 140 g/L, and platelets of 212 x 109/L. An echocardiogram had been arranged but would not be completed for several months, possibly longer due to the pandemic restrictions in place. The patient wondered whether he needed to continue the ACE inhibitor and questioned if there were any other medications that would be helpful for his condition.\nAs the patient had no other medical co-morbities, determining whether this was HFpEF or HFrEF had important implications regarding whether the patient should remain on an ACE inhibitor or if a beta-blocker and MRA would be of benefit. While awaiting the results of the echocardiogram, ML was implemented in the hopes of determining this distinction at the point of care.\nThe algorithm returned a diagnosis of HFrEF. This result was not entirely unanticipated as a lean male without hypertension or atrial fibrillation is not a classical HFpEF patient. A LBBB is also associated with a decreased ejection fraction, often in the absence of obvious cardiovascular disease. While a certain degree of confidence could be placed on this result, it was decided further treatment would await the echocardiogram. He remained stable on these medications with no further exacerbations of his HF. The echocardiogram completed 3 months later demonstrated decreased systolic function with an ejection fraction of 26%. He was subsequently started on metoprolol as well as spironolactone and is currently awaiting cardiac catheterization. |
A 63-year-old male initially presented for orthopedic evaluation after experiencing one month of left lateral hip and thigh pain. His past medical history includes JAK2+ myelofibrosis, which was diagnosed three years prior and treated with splenectomy. He also underwent a bone marrow transplant one year before his current presentation and was placed on chronic immunosuppression. Notably, this patient was very physically active and ran a five-kilometer race one month prior to his presentation. On physical exam, his pain was reproducible when bearing weight on his left lower extremity. He was found to be anemic with a hemoglobin of 8.9 g/dL (two months prior 13.0 g/dL) and also in a state of thrombocytosis with a platelet count of 544 K/uL. Full length femur radiographs were obtained and demonstrated a permeative, lytic lesion of the proximal femur that involved the medullary canal and lateral cortex (Figure ). Advanced imaging provided further evidence of the lesion’s involvement into the surrounding soft tissue (Figure ).\nAn open biopsy of the femoral lesion was performed. Histologic sections of the biopsy tissue showed bone marrow fibrosis with increased megakaryocytes with some displaying hyperchromatic nuclei. Bone trabeculae showed osteosclerotic change and remodeling (Figure ). Immunohistochemistry stain for CD61 highlights megakaryocytes (Figure ) and reticulin stain demonstrates extensive marrow fibrosis (Figure ). Thus, the final diagnosis of myelofibrosis was concluded.\nThe patient underwent left femur intramedullary nail fixation followed by palliative radiation therapy, 25 Gy in 10 fractions. Two months after fixation of the left femur, he experienced new right hip and thigh pain. This new pain was not reproducible with weight-bearing. Full length right femur radiographs were obtained that showed lateral cortical thickening of the proximal femur with mottled bone demineralization. Advanced imaging showed identical findings to his myelofibrotic lesion on the contralateral side. The decision was made to not prophylactically fix his right femur because there was no cortical destruction seen on imaging and he did not have any mechanical pain. This lesion was managed with radiation therapy, 25 Gy in 10 fractions.\nDuring radiation treatment for his right proximal femur, he developed progressively worsening right heel pain. Over several months, it became swollen and allodynic. Radiographs demonstrated a subtle, postero-superior calcaneal lucency (Figure ). Advanced imaging provided further demonstration of a permeative lesion on the right, posterior calcaneus with cortical destruction and a medial soft-tissue mass (Figure ). An ultrasound-guided biopsy was performed, which confirmed a myelofibrotic lesion. He completed radiation therapy, 20 Gy in 10 fractions, with symptomatic improvement.\nEight months from his original presentation, he developed right shoulder pain. Radiographs were obtained and an acromio-clavicular lesion was noted (Figure ). Due to the progressive multi-focal bony lesions and short interval between presentations, ruxolitinib was initiated for systemic treatment. His shoulder pain improved with one month of physical therapy and medical management.\nTwo months later he was admitted to the hospital after a low energy fall and sustained a right intertrochanteric femur fracture. Prior to fixation, the patient began to clinically decline and was intubated secondary to severe acidosis. He continued to decline, requiring dialysis for kidney failure and vasopressors for hypotension. The decision was made to withdraw care and transition to palliative care. Just 10 months after his initial presentation, the patient passed away. A timeline of the patient's clinical course is displayed in Figure . |
The patient was an 18-year-old woman with mild epigastralgia who was admitted to a nearby hospital. An abdominal CT scan showed a 60-mm mass confined to the upper pole of the spleen; therefore, she was referred to our department for surgical treatment (Fig. a, b). The tumor showed higher intensity on T1-weighted MRI and lower intensity on T2 imaging than the normal spleen. In dynamic MRI, the contrast was gradually enhanced from the edge toward the inside, with a spoke-wheel pattern, without malignant findings (Fig. c). From such findings, the tumor was suspected to be splenic lymphangioma. It was a benign tumor, but we decided to resect it because of her symptoms. We performed laparoscopic partial splenectomy on the patient with the plan to divide the upper pole branch of the splenic artery and remove only the upper spleen (Fig. d). The patient underwent surgery under general anesthesia in the lithotomy position by a co-axial approach using four ports and a liver retractor (Fig. ). After peeling the tissue around the pancreatic tail and spleen hilum, the upper pole branch of the splenic vessels was clamped (Fig. a). The ischemic side of the tumor was confirmed, and the upper pole branch of the splenic vessels was divided as planned (Fig. b). Using a bipolar electrocoagulation hemostasis device and a vessel sealing system, the spleen was dissected at the ischemic parenchyma approximately 1 cm from the demarcation line. Because the ischemic-side spleen was dissected, there was less bleeding (Fig. c). The excised spleen was retrieved from the umbilical wound and extended several centimeters using a retrieval bag without crushing the specimen. The operation time was 217 min, and bleeding was minimal. The patient was discharged after 8 days without complications. The patient’s symptoms disappeared with no recurrence at 1 year postoperatively. Blood tests and CT scans confirmed no problems with the remaining spleen (Fig. d). The specimen showed a well-defined mass of 60 × 48 mm (Fig. a). Histologically, vascular-like structures were growing, and in the lumen, lightly acidic serous substances and histiocytic cells were stored (Fig. b). Immunohistochemical staining yielded D2-40 weakly positive (Fig. c), CD31-positive, CD34-negative, and CD8-negative results (data not shown); hence, a cavernous type lymphangioma was diagnosed. Ethics committee approval was unnecessary for our case report, and the patient gave informed consent; anonymity was preserved. |
A 21-year-old female patient presented to the Department of Conservative Dentistry and Endodontics with chief complaint of continuous pain in the right mandibular posterior region. There was no dental history of trauma or any hereditary conditions. Medical history was non-contributory.\nOn clinical oral examination, the right mandibular second molar had deep occlusal caries exhibited abnormal crown morphology with a paramolar on the mesiobuccal aspect of the crown []. Distinct developmental occlusogingival grooves between the paramolar and its normal counterpart were noticed. However, the probing depth was within the normal limits, that is, between 1 and 3 mm. Despite the presence of the grooves, there was no discernible separation between the two teeth. The fused teeth showed increased buccolingual width with a wide distinct crown. The right mandibular first molar also showed the deep carious lesion adjacent to fused tooth. Other oral examinations revealed absence of both right and left mandibular third molars and had no history of extraction.\nOn electric pulp testing, right mandibular second molar showed delayed response compared with adjacent teeth and was tender on percussion. Preoperative radiographs taken at different angulations revealed union between the right mandibular second molar and the paramolar []. Based on clinical and radiographic examinations, a diagnosis of irreversible pulpitis with acute apical periodontitis was made.\nLocal anesthesia was administered and endodontic access cavity preparation was done under rubber dam isolation []. The prongs of the rubber dam retainer had to be trimmed to accommodate the unusual anatomy of the tooth. The pulp chamber of second molar was large with visible dentinal map, whereas the pulp chamber of paramolar was small with a round orifice. The second molar had three separate mesiobuccal (MB), mesiolingual (ML), and distal (D) canals. The paramolar had a single canal located mesiobuccally to the MB canal of the second molar. Working lengths were measured with electronic apex locator (DentaPort ZX, J Morita, USA) and confirmed by radiograph []. However, by exploring the canal of paramolar and MB canal of second molar by using two separate K-files combined with an apex locator provided evidence of communication between the two pulp canal systems. Cleaning and shaping of the root canal system was completed using ProTaper rotary system (Dentsply Maillefer, Switzerland). Canals were copiously irrigated with sodium hypochlorite (2.5%), followed by normal saline. The canals were dried with sterile paper points, calcium hydroxide was placed in the root canal, and the access cavity was temporized with Cavitemp (Ammdent, India). The patient was recalled after 1 week for obturation.\nAfter a week, the tooth was asymptomatic, and the root canal was obturated using single cone technique with AH Plus (Dentsply Maillefer, Switzerland) as a sealer []. Both the access cavities and mandibular first molar were restored permanently with a universal composite resin restorative material (Z250; 3M, ESPE, Germany) [].\nInformed consent from the patient was obtained, and a CBCT of the right mandibular region was performed. The involved tooth was focused and CBCT gave a three-dimensional view of fused teeth [ and ], as well as all the root canals in transverse, axial, and sagittal sections. The CBCT images revealed an example of fusion before root formation between MB root and paramolar []. There was connection between the paramolar and MB canal of second molar in the middle third region of root and exhibited a Type II canal configuration (according to Vertucci's configuration) with a thin lip of dentin separating both the canals []. After 1-year follow-up, there were no clinical symptoms, and the recall radiograph appeared normal []. |
A 69-year-old female with a history of type 2 diabetes mellitus, atrial fibrillation on apixaban, and chronic kidney disease was referred to our department for a large right groin hematoma with extensive ecchymosis complicated by hypotension after a percutaneous coronary intervention with drug-eluting stent (DES) (Figure ). An urgent angiogram showed active bleeding from a needle injury to a branch of the SFA which was successfully treated with a 7 x 22 mm atrium balloon-expandable (BE) covered stent to the SFA, after attempts to cannulate the branch failed. She was transferred to the Coronary Care Unit thereafter.\nIn light of the DES, she was started on dual antiplatelet therapy and a one-week course of co-amoxiclav, with daily iodine-soaked gauze dressing to the groin puncture site. Her recovery was otherwise uneventful during this admission and she was transferred to a community hospital for rehabilitation thereafter.\nShe was readmitted to us five weeks later after the previous puncture wound started to ooze blood. A focused duplex ultrasound scan of the femoral artery excluded a recurrent pseudoaneurysm but sized the hematoma at 15.21 x 8.37 x 4.83 cm.\nShe was started on antibiotics and assessed by plastic surgery who felt that the overlying skin was non-viable requiring surgical debridement and evacuation of the hematoma. However, she adamantly refused surgery, so we attempted to manage the fluid discharge with a stoma bag over the sinus. The drainage was constant and fairly voluminous (200 ml/day) and the position of the sinus close to the groin crease was suboptimal for sealing, both factors in combination causing the stoma adhesive to be ineffective with persistent leakage around the stoma, which in turn caused her great distress. We then placed a 13 cm PrevenaTM Peel & PlaceTM system over the sinus which managed to seal the leakage effectively and drained the fluid from the Prevena dressing in a closed fashion into an InfoVACTM machine.\nAt one week, the skin remained unhealthy-looking but the rate of drainage had decreased to about 50 ml/day (Figure ). Prevena was reapplied for fluid management and attached to an ActiVAC machine and the patient was discharged.\nOn inspection a week later, the hematoma was smaller at 9 x 4 cm, and no longer fluctuant (Figure ). As fluid drainage had ceased, the Prevena was discontinued.\nTwo weeks later, which was four weeks from the time of first application, the previously-deemed nonviable skin now appeared healthy although somewhat scarred (Figure ). She was given an open appointment. |
A 67-year-old man with a history of hepatitis C virus (HCV) and hepatocellular carcinoma (HCC) underwent orthotopic liver transplantation in 2006. He presented with decompensated liver disease marked by ascites and hepato-renal syndrome leading to renal failure. He was on hemodialysis prior to transplant and had a Model for End-Stage Liver Disease (MELD) score of 35 at time of transplant. His donor was a 49-year-old man with no history of malignancy. Explant pathology revealed established cirrhosis with a 2.5 cm moderately differentiated HCC with evidence of microvascular invasion. After transplantation, he maintained stable liver function on an immunosuppressive regimen of tacrolimus and mycophenolate mofetil. He underwent two post-transplant liver biopsies, which revealed HCV recurrence: Grade 1, Stage 1 in 2008 and Grade 1, Stage 2 in 2010. His liver enzymes remained in the normal range at this time. He underwent HCC surveillance at 6 monthly intervals for the first 2 years after transplant and yearly thereafter for the next 3 years.\nIn 2007, a small pigmented skin lesion above his left eyebrow grew larger and became ulcerated. A biopsy in September 2009 revealed an ulcerated melanoma at least 0.7 mm in Breslow thickness. Wide local excision with sentinel lymph node biopsy performed in November 2009 demonstrated residual ulcerated melanoma, 2.51 mm thick, Clark Level IV, with a mitotic index of 11/mm2 and no lymphovascular or perineural invasion. Two sentinel nodes were negative for disease. Initial clinical staging by American Joint Committee on Cancer (AJCC) 7th edition was T3bN0, Stage IIB. In 2010, HCC surveillance imaging revealed a 5 cm right adrenal mass that was subsequently resected. The pathology revealed metastatic HCC. At this point his immunosuppression was switched from tacrolimus to rapamycin 3 mg daily and he was continued on mycophenolate mofetil 500 mg twice daily.\nThe patient underwent active surveillance with clinical exams and imaging studies until October 2013, when he noticed a swollen mass in the left parotid region. A needle biopsy revealed melanoma wild-type for BRAF and cKIT. A positron emission tomography/computerized tomography (PET/CT) scan performed in November 2013 demonstrated prominent focal hypermetabolic activity in bilateral lung nodules, bony foci and a parotid lesion, consistent with M1c disease. Magnetic resonance imaging (MRI) of the brain showed no evidence of metastases. A repeat scan in January 2014 showed increasing nodal and bony disease and new hepatic metastases in the allograft. At that point, he was initiated on therapy with paclitaxel. His rapamycin was reduced from 3 mg to 1 mg daily and mycophenolate mofetil was discontinued, given prior clinical reports documenting tumor regression with reduction of immunosuppression [,]. He completed 5 cycles of chemotherapy as well as 14 of 20 planned fractions of palliative radiotherapy to the hip before experiencing multifocal disease progression in April 2014, with increasing disease burden in the lungs, mediastinal lymph nodes, liver and spleen.\nAfter a multidisciplinary team-based discussion including both medical oncology and transplant medicine, the decision was made to begin therapy on ipilimumab while maintaining rapamycin at 1 mg daily. Given the increased risk of graft rejection, the treatment plan included weekly monitoring of liver function tests. The patient received his four induction doses of ipilimumab 3 mg/kg between April 2014 and July 2014. The patient experienced a mild non-pruritic rash on his torso after the second infusion, which lasted one week and resolved with topical steroids. The aspartate aminotransferase (AST), alanine aminotransferase (ALT) and alkaline phosphatase were mildly elevated at baseline (Common Terminology Criteria for Adverse Events [CTCAE] v4.0 Grade 1) and remained stable throughout the course of treatment. Total bilirubin was within normal limits at baseline and remained normal throughout the course of treatment. The fourth and final dose of ipilimumab was administered at week 10, on 7/1/2014. On week 12, a Grade 2 transaminitis and alkaline phosphatase elevation developed, with no associated hyperbilirubinemia. The patient was managed conservatively with frequent laboratory tests. AST and ALT levels peaked at a Grade 3 on week 16 with stabilization or improvement the following week without intervention (Figure ). Because the patient remained asymptomatic and compliant with weekly laboratory testing, it was decided to maintain him on close monitoring and defer initiation of corticosteroids. Alkaline phosphatase levels peaked at week 17, but remained Grade 1 throughout the course of treatment. At last monitoring in February 2015, this value had returned to within normal limits. Total bilirubin has remained within normal limits since the induction of treatment. By week 20, all lab values resolved to Grade 1, and conservative management was continued. The patient declined a recommended liver biopsy. The patient remains stable 10 months following induction of treatment.\nRepeat CT scans conducted in July 2014, after his fourth infusion of ipilimumab, showed dramatic tumor regression in the lungs (Figure a) as well as the liver (Figure b). Clinically, the patient continues to feel well and is undergoing continued close monitoring for both his liver function and disease status. |
A 54-year-old male patient presented with a two-day history of severe left-sided, lower back pain which disappeared with the appearance of left-sided lower limb pain felt deep inside the whole lower limb and described by the patient as intense internal pressure (VAS 8/10). On examination, power was 5/5 with no sensory deficit and normal reflexes. The patient received NSAID painkillers and active bed rest was advised. However, on the patient's insistence, a lumbar spine X-ray and MRI were done which showed mild IVD protrusion. The patient was reassured and sent home. A week later the patient presented to the ER with an 18-hour history of heaviness and difficulty in raising the left foot when walking, with numbness along the lateral part of the leg and dorsum of the foot. The pain was moderate (VAS 5/10). No sphincter-related symptoms were observed. According to the patient, the decreased pain caused the delay in presentation, against the instructions on first evaluation. On examination of the foot dorsiflexion was 2/5 (movement on gravity alleviation) with decreased sensation along the left L5 dermatome. There were normal reflexes and sphincters. The patient underwent immediate, new lumbar MRI which showed a large disc sequester with disc migration. His status was fully explained; he was admitted and underwent microdiscectomy L4/5.\nThe initial back pain lasted for 2 days, mostly caused by stretching and pressure on the weakened annulus fibrosis. This type of pain disappears or decreases once the annulus opens and nucleus pulposus leaks to the spinal or root canal. Initial left lower limb pain is a typical description of painful radiculopathy where mass pressure and inflammatory irritation of the nerve root and dorsal root ganglion cause neuropathic pain. This is different from nociceptive and referred pain types. Dorsal root ganglion hosts the cell bodies of sensory nerves with bidirectional connections (to the periphery and spinal cord). Injury to the nerve root motor fibers causes weakness.\nThe majority of patients with lumbar disc and radicular pain improve with conservative treatment. Surgical indications are acute or progressive motor weakness, sphincter dysfunction, intractable pain not responding to analgesia, and pain affecting patient daily life, not responding to 6 weeks of conservative treatment in the absence of acute surgical indications. In all cases, there should be a correlation between the clinical picture and MRI findings. In the current case, the patient has left L5 nerve root symptoms and signs (dorsiflexion weakness, normal reflexes, and dermatologic radiculopathy). The expected IVD prolapse occurred on either the left posterior-lateral L4/5 or left extreme lateral L5/S1 disc. Although there is a debate about dermatologic sensory distribution in root-originating symptoms and variations in clinical presentation (i.e., the L4/5 disc causing S1 root symptoms via compressing the root in higher position, groin pain with lower lumbar discs due to paravertebral sympathetic ganglion pathway entering at L1 or L2 nerve), we presented the commonly encountered scenarios. |
A 50-year-old male underwent emergency treatment for acute psychosis (delusions and hallucinations) in a psychiatric hospital and received haloperidol. The patient experienced the following side effects in the post hospital phase: acute dystonia, parkinsonism, dysarthria, and akathisia. The medication therapy was changed to a cariprazine-clozapine combination and was then continued with only cariprazine. A dose of 3 mg of cariprazine in monotherapy achieved stable improvement and full patient functionality for a period of at least 1 year.\nA family history uncovered mental health problems in a sister, which was likely depression. The patient was born in a difficult labor, and presented fetal macrosomia. At an early age, the patient experienced difficulty pronouncing words and had attended speech therapy. He had average grades in school and was a loner. He continued his education at the university and attained a doctoral degree. For the past 20 years he has worked at a public institution at a senior level position.\nThe patient divorced 15 years ago and has two children. He currently lives with his father and sister and has had a girlfriend for several years with whom he shares common interests in astrology and the occultism.\nThe patient had rarely been ill during his lifetime and indicated only a gastric ulcer as a problem. Approximately 5 years ago, he suffered a concussion, but did not incur permanent damage. He does not consume alcohol or other addictive substances.\nAn overview of events, medications, evaluation, and associated comments about first hospital treatment episode is found in . The patient was initially admitted to an acute psychiatric inpatient unit at the instigation of the family as he had rapidly—within a period of 1 week—developed acute psychosis, psychomotor agitation, and thoughts of being cursed.\nUsing the International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD 10) (), a diagnosis of paranoid schizophrenia (F20.0) was made. Organic causes such as drug-induced psychosis, delirium, and metabolic disturbances were excluded. Differentiation from acute schizophrenia-like psychotic disorder (F23.2) was made based on detailed information from the patient and relatives concerning the duration of the psychosis. At the inpatient unit, the patient received haloperidol up to 15 mg/day (which was initially given intramuscularly in a dose 7.5 mg/day and then perorally 15 mg/day), 6 mg/day of trihexyphenidyl, and 5 mg of olanzapine in the evenings. The patient remained hospitalized for 31 days. Throughout this period, his acute psychotic symptoms lessened, although they were not eliminated. Some delusions remained, and the patient was suspicious.\nThe patient underwent a psychodiagnostic examination, and it was noted that his thinking was distinctly peculiar, atypical, and characterized by making judgments on the basis of assumptions understandable to himself but difficult for others to understand. The personality profile reflected fatigue, an apathetic state, a low energy level, and difficulty in motivating himself with purposeful actions. Interpersonal relations showed a tendency toward social introversion with avoidance and distancing behavior, a limited ability to express feelings and experiences, sensitivity to other people's attitudes toward him, cautiousness, and slight suspiciousness.\nThe patient was discharged from the hospital with recommendations to take 15 mg/day of haloperidol, 6 mg/day of trihexyphenidyl, and 5 mg of olanzapine in the evenings. The patient had planned to return to work. However, his condition deteriorated within ~2 weeks following hospital discharge. An overview of events, medications, evaluations, and comments about second hospitalization and outpatient treatment is available in . The patient began to exhibit side effects from the neuroleptics including parkinsonism, akathisia, dysarthria, and acute dystonia. This was partially the result of the patient reducing the dosage of trihexyphenidyl. In addition, without full understanding about the role of medications, he had little fluid intake due to a fear of sweating. He sought help from the outpatient service because of the pronounced neuroleptic side effects. His treatment was subsequently adjusted, and he was rehospitalized.\nHospital treatment consisted of intravenously administered diazepam to alleviate the side effects, stopping the administration of haloperidol and olanzapine and, instead, introducing 6 mg/day of cariprazine with 37.5 mg of clozapine in the evenings, and 6 mg/day of trihexyphenidyl. Clozapine was added to avoid psychosis as result of the rapid changing of medicines from first generation antipsychotics to cariprazine. Given a treatment history that included an acute psychotic episode, possible future monotherapy appeared to be unlikely. A more likely option was a combination of cariprazine and clozapine. However, the patient received complex therapy during his hospital stay, including psychological counseling, drama, music, and visual art therapy (), as well as ergotherapy sessions. The medication side effects resolved, and the patient regained confidence in therapy. Therefore, 6 mg/day of cariprazine was recommended following hospital discharge.\nAfter discharge, the patient returned to work. He currently sees a psychiatrist on a regular basis, and the dose of cariprazine has been gradually reduced to 3 mg/day in monotherapy. To date, his condition is stable. He has been fully functional for a year, with no positive or negative symptoms such as a loss of drive. However, a diminished capacity to express feelings are mildly pronounced. No additional psychological and social therapies have been needed. The patient is positive about his future treatment course; although, no final decision has been made concerning future medication use. While the patient is interested in quitting medication, there is the risk of future psychotic episodes or, in the case of a worsening mental health status, he might avoid treatment based on his negative experience. |
A 58-year-old male with no prior medical problems, who was leading an active lifestyle, presented to our emergency department with progressively worsening shortness of breath and abdominal distension for one month. His breathlessness had gradually worsened to the point where he felt winded even upon getting out of bed. He also reported intermittent abdominal discomfort associated with a decrease in appetite for a similar duration. Initially, the patient sought medical attention from his primary care physician and was started on treatment for community-acquired pneumonia without any significant improvement. He denied any fever, chills, night sweats, or weight loss. Review of systems was unremarkable including bowel habits, which were regular.\nThe patient had been a nonsmoker his entire life but had secondhand exposure to smoking from his wife. His alcohol consumption was limited only to social occasions and he denied any illicit drug use. He had no known allergies, nor did he take any daily medications. Family history was not significant for any malignancies. No recent travel was reported and by occupation, he was a tailor.\nOn examination, the patient was found lying comfortably in bed. Vital signs were significant for a pulse rate of 106 beats per minute, respiratory rate of 17 breaths per minute, and oxygen saturation of 98% on 2 liters of oxygen via nasal cannula. He was normotensive and afebrile. No cyanosis, clubbing, rash, or lymphadenopathy was noted. Respiratory examination revealed decreased air entry and dullness on percussion on the right side of the chest. His abdomen was distended, non-tender with a palpable liver and spleen, and he was noted to have shifting dullness. The rest of the examination was within normal limits. Laboratory data are summarized in Table .\nUpon admission, a chest x-ray showed a near-complete opacity of the right hemithorax secondary to a large pleural effusion (Figure ). A bedside thoracocentesis was performed and 600 milliliters of whitish milky pleural fluid was drained (Table ). The pleural fluid analysis was consistent with chylothorax. A computed tomography (CT) scan of the abdomen and pelvis with contrast revealed a large soft tissue mass in the region of the pancreatic head with extensive diffuse lymphadenopathy and minimal perihepatic ascites (Figure ). Further review of imaging revealed soft tissue deposits in the anterior abdomen and pelvis with peritoneal enhancement concerning for peritoneal carcinomatosis. The initial impression was pancreatic adenocarcinoma with peritoneal seeding. An oncology consultation was requested and a biopsy of the peritoneum was advised due to ease of access. An ultrasound-guided biopsy of the peritoneal lesion with analysis of the peritoneal fluid (Table ) and a CT-guided placement of a right chest pleural drainage catheter was performed by interventional radiology. Analysis of the peritoneal pathology specimen revealed a mature B-cell neoplasm reported as a grade 2 follicular lymphoma (Figure ), and immunophenotype stains revealed CD20+, CD79A+, CD10+, BCL-2+, BCL-6+, CD5-, CYCLIN D1-, CD3-, CD43-, AE1/AE3-. Cytology from the previously obtained pleural fluid also demonstrated lymphomatous infiltration. Due to persistent right pleural effusion, embolization of the thoracic duct was attempted without success. The patient was eventually transferred to a tertiary center under thoracic surgery service for further management of the high output chylothorax. He eventually was enrolled in the lymphoma program of the tertiary care center and started on chemotherapy. |
A 23-year-old woman was admitted because of a 20-day history of gross hematuria. She had no lower urinary tract symptoms or systemic symptoms such as fever, joint pain, or rash. She had no relevant medical history. Her laboratory test results showed no abnormalities. Serum and urine protein electrophoresis revealed no monoclonal band. Ureteral computed tomography showed thickening of the left anterior and posterior walls of the bladder (). Cystoscopic examination revealed multiple lesions and obvious vessel engorgement on the anterior and posterior walls of the bladder. The base was wide and bulging, showing a crater-like appearance. The largest diameter of the base was about 3.0 cm (, area B), and the bilateral ureteral orifices were not involved. Tissue biopsy demonstrated urothelial tissue hyperplasia and interstitial degeneration with edema. Transurethral resection of an isolated lesion ( and , area A) was performed to achieve a diagnosis. The other lesions were not treated.\nPathologic examination showed amyloid depositions in the subcutaneous interstitium of the urothelium, and Congo red staining was positive (). After consideration, the patient decided to undergo transurethral resection of the bladder lesions 1 month later. During the operation, the intravesical lesions were found to have significantly improved. Only a few small bleeding foci and yellow mucosal bulges were observed at the untreated sites (, area B). A healing scar was seen in the biopsy site (, area A). Therefore, the patient did not undergo a further operation and was planned to return for follow-up in 2 months. Repeat cystoscopy 3 months after the resection biopsy showed that the lesions had nearly disappeared (). No blood vessel dilation or bleeding foci were seen. Nine months later, no lesion recurrence or new lesions were found (). The patient did not take any medication during this period. At the time of this writing, she was still undergoing follow-up and had no hematuria. |
A 25y/o South East Asian male medical student presented in our outpatient department in January 2018 with left-sided cervical lymphadenopathy. The patient reported small bulges along the left side of his neck for one month. Associated symptoms included one month of low-grade fever and fatigue. There was no history of night sweats or reported weight loss. A course of antibiotics two weeks earlier did not improve his symptoms. On presentation, the patient was hemodynamically stable with a temperature of 100.1°C, heart rate of 98 beats/min, respiratory rate was 18 breaths/min and blood pressure was 115/80 mm/hg. On physical examination there was diffuse left cervical and supraclavicular lymphadenopathy. Lymph nodes were rubbery, soft and mobile. There were no changes in hands, eyes or ears. His nose and throat examination were normal. On auscultation of the chest, breath sounds were normal bilaterally and normal heart sounds where present. The abdominal examination was also normal. Initial lab investigations included complete blood count with total and differential leukocyte count, metabolic profile, erythrocyte sedimentation rate (ESR) and lactate dehydrogenase (LDH). This was to rule out any possibility of lymphadenitis, or neoplastic disorder. On laboratory examination there was an increase in lymphocytes percentage (40%) but no leukocytosis and an increase in inflammatory markers including ESR and LDH (\n).\nA provisional diagnosis of tuberculous lymphadenitis was made based on his occupation. Further investigations were ordered to determine the size and extent of the lymphadenopathy. These included ultrasonography of the neck and abdomen, to visualize any hidden lymphadenopathy that might have been missed during the initial physical examination; chest x-ray, to rule out any active tuberculosis; and interferon-gamma release assay.\nOn ultrasonography (\n), the patient showed enlarged multiple discrete left cervical and supraclavicular lymph nodes measuring up to 16×10mm. The rest of the ultrasound report did not show any abnormalities. Chest x-ray was normal and interferon-gamma release assay (IGRA) was not conclusive due to possible delayed transport affecting lymphocyte viability, per the pathology lab. PPD was not not performed as IGRA was done. Anti-tuberculosis therapy was deferred till a formal diagnosis.\nTo ensure a definitive diagnosis, surgery with lymph node excision and biopsy was performed. An excisional lymph node biopsy from the anterior cervical chain was performed and on histopathological analysis it showed necrotizing lymphadenitis with partial alteration of structure by clusters of histiocytic and interspersed nuclear debris. In preserved areas, lymphoid follicles with pale staining germinal centers were also seen. No evidence of tuberculous granulomas or malignancy was found. Unfortunately, microphotographs could not be procured as testing was done in a third-party laboratory. Stains for acid fast bacteria were also negative. The culture did not show any growth. No further infectious work up was performed.\nIt was decided that no antibiotics should be given to the patient at this time and watchful waiting was advised. For fever, 500mg paracetamol twice daily was prescribed for one week only. The patient was followed up twice a month in our outpatient clinic to monitor any spread of the lymphadenopathy. The disease course was uneventful. The patient was not given any further medication and watchful waiting was continued. Within two months the lymphadenopathy decreased dramatically, and the patient reported no fever. It completely disappeared in four months. |
A 36-year-old Caucasian woman was evaluated with chief complaint of gluteal pain radiating to her leg. Her medical history was remarkable with gunshot injury to the affected leg with multiple pellets dispersed into her pelvis and proximal part of the thigh, as shown in Figs. and . She had gunshot injury 20 years ago. She was previously diagnosed as having lumbar disc herniation at L4–5 level. She underwent a previous discectomy outside our institution 2 years ago. A radiological examination revealed the presence of recurrent disc herniation, as well as multiple shotgun bullets in her pelvis and thigh. One of those bullets was deep into the sciatic nerve inside her quadratus femoris muscle.\nElectromyography (EMG) showed the presence of chronic sciatic nerve injury. Since it was clinically impossible to distinguish lumbar disc herniation from the sciatic injury, we decided to proceed with removal of the foreign object and neurolysis of the sciatic nerve followed by L4–5 discectomy and fusion. We decided to perform those procedures in two different settings. The first surgery included access to the sciatic nerve in the upper portion of her thigh and exposing the nerve fibrotic bands around the nerve. The dissection proceeded deep into the nerve within a muscle, where a bullet was found and removed. The distance from the bullets to the nerve was approximately 2 cm. Muscle tissue around the bullets was excised for analysis. For comparison, another specimen was obtained from the gluteal muscle, superficially away from the nerve and all the bullets. Two weeks later, she underwent scheduled L4–5 discectomy and fusion. Her postoperative course was uneventful. On follow-up examination at 6 months, she was essentially symptom free.\nA scanning acoustic microscope (AMS-50SI) developed by Honda Electronics (Toyohashi, Japan), whose schematic setup is shown in Fig. , was used in AI mode. It has a transducer with quartz lens, a pulser/receiver, an oscilloscope, a computer, and a display monitor. An 80 MHz transducer is installed within the microscope, which generates the signals and collects the reflected acoustic waves. Water is the coupling medium between the quartz lens and the substrate. For two-dimensional scans, an X-Y stage, controlled by a computer, is used. An oscilloscope analyzes the reflected signals from both the reference and target material after being collected by the transducer. As a result, acoustic intensity and impedance maps of the region of interest with 300 × 300 sampling points are obtained.\nThe principle of SAM in AI mode is demonstrated in Fig. . Distilled water is widely used as reference. The signal reflected from the target is\nwhere, S0 is the generated signal by the 80 MHz transducer, Ztarget is tissue’s AI and Zsub is the polystyrene substrate’s AI (2.37 MRayl). The tissue’s AI is calculated by combining the reflected signals from the tissue and the reference. The signal reflected from the reference iswhere Zref is the AI of water (1.50 MRayl). Then, the target’s AI is written aswith a constant signal S0 [] generated by the transducer.\nElectron microscopy-based imaging and chemical analysis studies were performed in a JEOL JIB-4601 focused ion beam scanning electron microscope (FIB-SEM) multi-beam platform coupled with an Oxford X-MaxN EDS system, as shown in Fig. .\nBlood samples were collected in test tubes containing ethylenediamine-tetraacetic acid (EDTA) and no anticoagulant on the day of the first surgery (foreign object removal) prior to the procedure. Then, 2 ml of 20% trichloroacetic acid (TCA) was supplemented into the blood samples to release the red blood cells (RBC) and other ingredients. The supernatant part was received from blood with TCA by centrifugation at 4000 revolutions per minute (rpm) for 20 minutes for the analysis of Pb and cadmium (Cd) within total blood. Coagulation of blood samples enabled serum trace element analysis: chromium (Cr), Fe, Cu, magnesium (Mg), manganese (Mn), selenium (Se), and Zn. The serum specimen was prepared using Hettich Universal centrifuge by centrifugation at 3000 rpm for 15 minutes, separating from cells immediately after and storing at − 20 °C until the analysis [].\nAfter weighing the left sciatic nerve tissue samples, they were digested with 2 ml of 65% nitric acid (HNO3) at 180 °C in the incubator for 1 hour. Then, 2 ml of 65% perchloric acid (HClO4) was added into the cooled mixture. Then, the mixture was digested at 200 °C in the incubator until the volume was halved. Digested materials were vortexed and diluted in water to a total volume of 10 ml. Concentrations were given in micrograms per gram (μg/g) wet tissue weight [].\nAll glassware were maintained at 10% (volume/volume; v/v) HNO3 before use, cleaned with deionized water, and dried in an incubator at 100 °C overnight. Pb, Cd, Cu, Cr, Fe, Mn, Se, and Zn elements were detected by inductively coupled plasma optical emission spectrophotometer (ICP-OES 6000, Thermo, Cambridge, United Kingdom). Measurements for each element were done three times and averaged. The ICP-OES was operated with argon carrier flow rate of 0.5 L/minute, plasma gas flow rate of 15 L/minute, sample flow and elusion rate of 1.51 L/minute, and peristaltic pump speed of 100 rpm, selecting the suitable wavelength for Pb, Cd, Cr, Cu, Fe, Mn, Se, and Zn, which were 220.353 nm, 228.802 nm, 267.716 nm, 324.75 nm, 285.213 nm, 357.610 nm, 196.090 nm, and 206.200 nm, respectively. Transport lines were obtained using 1.25 mm internal diameter polytetrafluoroethylene tubing. Element levels were indicated in micrograms per deciliter for serum (μg/dl) and μg/g for wet tissue. The standard concentrations for standard graph calibration were arranged from standard stock solutions of 1000 μg/ml for each analyzed element [].\nThe tissue samples were investigated by using AI mode of SAM. Figure shows the AI map of the tissue obtained away from the gunshot. The map was constructed by collecting the reflections of acoustic signals, generated by the transducer within SAM, from surfaces of the reference (water) and the tissue sample on the polystyrene substrate. At specific locations within the sample, the AI was calculated to be higher than 2 MRayl, indicating accumulation of elements with different elastic properties. Figure shows the AI map of the tissue obtained close to the gunshot. As can be seen in this image, almost everywhere had an AI of greater than 2 MRayl.\nSEM images of the tissue far away from the gunshot were obtained at magnifications of 5000 × and 500 ×, as shown in Figs. and , respectively. Similarly, SEM images for the tissue close to the gunshot were obtained at magnifications of 5000 × and 500 ×, as shown in Figs. and , respectively. The images were acquired at 5 keV energy for both tissue samples.\nThe SEM images show that the tissue far away from the gunshot keeps its original structure, whereas the tissue close to the gunshot seems to be deformed and torn up. These results demonstrate the degree of damage the impact of gunshot causes on soft biological tissues.\nTable represents the EDS measurements in SEM, carried out for determining the elemental distribution differences in the deformed tissues. The measurements show the percentages of the residue elements detected on tissues far away from the gunshot and close to the gunshot. According to the results, among all residue elements, Pb, Cr, Fe, and Mn are found to be higher in weight content in the region close to the gunshot, when compared to distant region. Cd and Cu levels do not differ much; however, Zn level is lower in the tissue close to the gunshot.\nWe determined Pb, Cr, Cd, Cu, Fe, Mn, Zn, and Se levels in both tissue samples. Pb, Cr, Fe, Se, and Mn levels were higher in the tissue close to the gunshot, conversely, Zn level was lower in this sample (Table ). Blood Pb and blood Cd, and serum Cr, Cu, Fe, Mn, Se, and Zn levels of our patient were also analyzed and the results are shown in Table , however, we did not observe significant differences when compared to reference values. |
Patient is a 34-year-old trans man who is married to a cis-gender female. He previously underwent top surgery and later desired removal of his reproductive organs with preservation of his vaginal canal for two main reasons. Firstly, his dysphoria was unrelated to urinating in the standing position and more related to the absence of a phallus and his desire to use his phalloplasty for intercourse. In addition, he preferred to avoid the untoward sequelae associated with a vaginectomy and a urethral lengthening procedure []. Furthermore, the patient and his female spouse wanted to keep his vaginal canal for sexual intimacy. The patient had two letters from his mental health teams recommending him for surgery, both noting the patient’s desire to maintain his vaginal canal.\nEven though this procedure would lead to decreased urinary complications, it is more challenging to perform since the patient desired a phalloplasty with scrotoplasty and future testicular implant placement in an anatomic region occupied by his native introitus and vaginal canal (). After careful consideration of the advantages and disadvantages of different flaps, the patient chose a musculocutaneous latissimus dorsi flap for his phalloplasty []. A disadvantage of this flap versus others is a lower sensory recovery because only one nerve, the lateral branch of the thoracodorsal nerve, is supplying it. The recipient artery chosen was the descending branch of the lateral femoral circumflex, the recipient vein was the greater saphenous vein and nerve anastomosis was a split lateral thoracodorsal nerve with one end anastomosed to the ilioinguinal nerve on the left (end to end with 9-0 nylon suture epineural sutures) and the clitoral nerve on the right (end to side) with epineural sutures using 9-0 nylon suture and an operative microscope. Cadaver nerve grafts from Axogen Inc., Alachua, FL, were used as interposition nerve grafts in both nerve anastomoses. Advantages include a more concealed donor site and a very sizeable tissue construction, although this means a skin graft on the donor site might be necessary, readily removed with serial excisions or tissue expansion if desired. A scrotoplasty was performed using the labia majora soft tissues, later used for testicular implants. This was created with bilateral V-Y random pattern labia majora flaps advanced inferiorly to just superior to the donor defect within the inferior labia majora. These are sutured in the midline and cephalad to the clitoral hood region. Careful attention was paid to maintain the vaginal canal appearance and capacity while constructing the phallus, especially during the microvascular portion of the procedure. The phallus was constructed, and the clitoral hood, clitoris and vaginal canal were not injured or modified (). Hegar dilators were useful for identification of the vaginal canal during the dissection to avoid injury to the vaginal canal and maintain adequate aperture (). Six months later after flap transfer, the patient had an insertion of bilateral testicular prosthesis above clitoral hood prior to penile implant placement (). Serial excision of skin grafted donor site was followed by a left latissimus dorsi myocutaneous flap (). Successful insertion of penile prosthesis was performed at 8 months when the patient exhibited tactile sensation to two-thirds down his penile shaft from the base of his phallus. (). |
A 52-year-old male with a history of Stage IIIb melanoma initially underwent a wide local excision with a sentinel lymph node biopsy for a 2.1 mm in-depth melanotic lesion of his left upper arm melanoma. The sentinel lymph node biopsy at that time was negative for malignancy. Two years later he returned with an in-transit metastasis proximal to the original lesion. He underwent re-excision and axillary lymph dissection at that time. Following the removal of this lesion he was started on ipilimumab at 6 mg/kg. He had completed two injections spaced 1 month apart before he returning to clinic with complaints of diarrhea and significant abdominal pain. At that time a CT scan (Fig. ) and colonoscopy with biopsy were performed, both were consistent with active colitis. Adjuvant therapy was suspended and the patient was started on prednisone at standard dosage. The patient’s symptoms improved over the course of the next week. However, 2 weeks following initiation of immunosuppresion he presented acutely to the emergency department with hypotension, peritonitis and free air beneath the diaphragm. He was taken to the operating room for exploratory laparotomy. Following entrance to the abdomen via a midline incision extensive inflammation was noted from the terminal ileum to the mid-transverse colon. Perforation of the cecum was the salient finding (Fig. ). A right extended hemicolectomy was performed with end ileosotomy. Pathology was consistent with ipilimumab-induced perforation colitis (Fig. ). The patient tolerated the procedure and was cared for in the surgical intensive care unit overnight before being released to the general surgery floor in the morning. His postoperative course was complicated by continued symptomatic enteritis for the next 2 weeks; however by Week 3 repeat colonoscopy revealed evidence of regeneration and healing of the colonic mucosa. He was discharged home and will likely be scheduled for ileostomy takedown in 6–8 weeks following cessation of immunosuppression. |
A 71-year-old Caucasian woman was referred to our department in September 2013 because of the development of anaesthesia of the lower right lip and chin. This symptom was preceded by a sudden diffuse pain in the right mandible which lasted a few hours one month before the development of the neurological impairment. A dental extraction in the anterior region of the mandible was performed by dental practitioner due to severe periodontal disease after the onset of the first sudden mandibular pain. History taking of the patient revealed that she suffered from hypertension, chronic obstructive pulmonary disease, rheumatoid arthritis (RA), and an IgM monoclonal gammopathy of undetermined significance (IgM-MGUS). At the first clinical evaluation she was taking methotrexate (MTX) (10 mg once a week), folic acid (5 mg/daily), prednisone (7.5 mg/daily), ibuprofen (80 mg/daily), and calcium.\nThe intraoral clinical examination was unremarkable, but an ulcer of 1 cm wide could be seen in the lower lip. A painful hard swelling was evident on palpation in the lower right vestibular fornix in the premolar area and in homolateral submandibular space. Lymphadenopathies were detected in the right later cervical, supraclavicular, and subaxillary groups and in the left axillary group. The patient suffered from chronic periodontitis which during years determined tooth loss and prosthetic therapy with dental implants and partial removable dentures. There were no direct dental or other local causes which could explain the onset of anaesthesia of right inferior alveolar nerve.\nA dental panoramic tomography (DPT), a maxillofacial CT scan, and an ultrasound (U/S) of the neck were performed to assess the morphology and the limits of the lesion. The DPT revealed a radiopaque area diffused from 4.5 to 4.8 with some microlacunae on the alveolar ridge and lower mandibular cortex. The CT scan confirmed the microlacunar reabsorptions due to bony structural rearrangement of all the mandibular cortices peripheral to the radiopaque lesion diffused from 4.5 to 4.8. The centre of the lesion was characterized by hypodensity of the spongiosa. The mandibular canal was detectable only in the distal sections (). In the U/S, a 2 cm wide hypoechogenic mass was clearly visible. This mass was very close to the right corpus of the mandible with well-defined margins. The cortical bone peripheral to this finding revealed signs of cortical erosion. Three swollen lymph nodes were detectable close to the mass in the submandibular space. These glands showed a metastatic pattern. Other reactive lymph nodes were detectable bilaterally in the later cervical groups.\nThe patient was then referred to the maxillofacial surgery department in order to proceed with a biopsy of the mass and obtain a histopathology characterization of the lesion. Fragments of tissue were collected from the mandibular periosteum, medullary and cortical mandibular bone, and inferior alveolar nerve. The histopathological examination of the material obtained with the biopsies demonstrated a diffuse proliferation of large lymphoid cells with quite abundant basophil cytoplasm and pale perinuclear ring, oval nuclei with dispersed chromatin, and one or more nucleoli (, hematoxylin-eosin 400x). Immunohistochemical reactions were performed and the neoplastic cells were diffusely positive for CD20 and BCL2 and weakly positive for BCL6. The proliferation index, evaluated with Ki67, MIB1 clone, was high: about 70%.\nAfter the diagnosis of DLBCL the patient underwent a PET with 18-FDG and a total body CT-scan with contrast dye for proper staging of the neoplasm. The PET examination revealed an increased uptake in right mandible and in lymph nodes of the supradiaphragmatic, mediastina, aortic, and subcarinal groups. Other lymphadenopathies were detected by the CT-scan bilaterally in the submandibular, digastric groups and in the right subclavicular group.\nThe final diagnosis revealed a DLBCL IVA with bone marrow involvement (BM+) and a monoclonal IgM component (CM IgM-MGUS) in association with RA.\nThe patient was then referred to the department of haematology to initiate proper treatment. |
Our patient is a 38-year-old woman with a past medical history most significant for a Roux-en-Y gastric bypass surgery in March 2017, complicated by a marginal ulcer at the anastomosis site with subsequent perforation. The patient could not tolerate oral nutrition and became severely malnourished, and a PICC line was placed for TPN in December 2017. Chronic TPN use led to recurrent candidemia with blood cultures growing Candida albicans resistant to fluconazole and voriconazole. Over several months, her PICC line was replaced thrice, and she was ultimately treated with micafungin. The patient had multiple admissions over the past few months for workup of altered mental status and unsteady gait. Her other past medical history included systemic lupus erythematosus treated with mycophenolate mofetil and hydroxychloroquine, left portal vein branch thrombosis on enoxaparin therapy, type 2 diabetes mellitus not on insulin, fibromyalgia, depression, and anxiety. She presented to our hospital in July 2018 with complaints of tremors, impaired mobility, confusion, gait instability, falls, and lethargy.\nOn admission, she was noted to be confused, with horizontal nystagmus and dilated pupils as well as bradypnea. Dilated fundus examination was indicative of candida retinitis. She was lethargic but arousable. Her speech was difficult to understand, and the history of presenting illness was provided primarily by the patient's mother who relayed to the team that she had been having tremors, episodes of confusion, and numerous falls for the past several months. Her mother also noticed that she had increased irritability and emotional lability during this time. She was initially admitted to the intensive care unit for airway management due to her acute encephalopathy. Due to a lack of meningeal signs, a lumbar puncture was not performed.\nThe infectious disease service was consulted, and she was started on liposomal amphotericin B and flucytosine for a total of 6 weeks. MRI brain showed worsening T1 hyperintensities from June 2018 to July 2018 in the bilateral globus pallidi, as evidenced by the arrows in Figures and , which were thought to be secondary to manganese toxicity from chronic TPN use. Furthermore, a repeat MRI brain was performed in August 2018, one month after discontinuation of TPN, which showed improvement of the T1 hyperintensities in the bilateral globus pallidi, as evidenced by the white arrows in , approaching the intensity level noted in June 2018. Other supporting findings included T1 hyperintensities in the substantia nigra, as evidenced by the arrows in , and decreased signal in the bilateral globus pallidi on T2-weighted images, as evidenced by the arrows in . With antifungal treatment and cessation of TPN, her mentation and neurological symptoms improved within 3-4 days of cessation of TPN.\nGeneral surgery was consulted for gastrostomy tube placement in her remnant stomach, as the patient was unable to consume more than 500 calories by mouth per day due to complaints of nausea and vomiting. Although our patient presented to the hospital seven months after initiation of TPN therapy, her Parkinsonian symptoms had begun to manifest after three months on TPN. |
A 40 kg, 15-year-old boy presented with a history of episodic headache, vomiting, and difficulty in walking of 4-years duration. There was no history of trauma or medical comorbidities. His neurological examination revealed bilateral upward gaze palsy with alternate convergent squint. The patient had positive cerebellar signs in the left side. Computed tomographic (CT) scan of head showed a posterior third ventricular mass involving adjacent left thalamus causing proximal hydrocephalus of lateral ventricles and anterior third ventricle []. A midline suboccipital craniotomy and excision of the tumor in sitting position was planned. The routine investigations were within normal limits. He was premedicated with intramuscular glycopyrrolate 0.2 mg, one hour before induction of anaesthesia. In the operating room, routine monitors like ECG, pulse oximetry, and noninvasive BP were connected. Anesthesia was induced with intravenous propofol 100 mg and fentanyl 100 mg, followed by rocuronium 40 mg to facilitate tracheal intubation. Anesthesia was maintained with isoflurane, 66% N2O in O2, and intermittent boluses of fentanyl and rocuronium. Arterial BP, central venous pressure (CVP), and nasopharyngeal temperature were monitored, continuously, during the intraoperative period. End-tidal CO2 and CVP were maintained at 35 ± 2 mmHg and 8 ± 2 mmHg, respectively. The surgery lasted for 5 hrs. Blood loss of 350 ml was recorded. Fluids were adequately replaced and the patient remained hemodynamically stable throughout the procedure. At the end of surgery, residual neuromuscular blockade was reversed but the trachea was not extubated since the patient remained drowsy. He was transferred to intensive care unit but drowsiness remained even an hour later. Gradually the patient became unconscious (able only to localize pain). An urgent CT scan of head was done which revealed extensive pneumoventricle and a small residual tumor in left lateral ventricle []. Under local anesthesia, right frontal precoronal twist drill burr-hole was made and around 50 ml of air was tapped under water seal. The patient became conscious but drowsiness persisted. A repeat CT scan 12 hrs later revealed decreased extent of ventricular air. Ventricular tapping was repeated and approximately 30 ml of air was aspirated. The patient was mechanically ventilated for yet another day and trachea was extubated on third postoperative day (POD). The patient made a gradual recovery and was discharged from the hospital on eighth POD. |
A 33-year-old married male, from urban Manipur, of middle socioeconomic status, diploma holder in pharmacology and unemployed (previously working as a pharmacist), was admitted in our tertiary psychiatric facility. He presented with insidious onset and continuous progressive illness of 8-year duration, characterized by heroin use in a dependence pattern, associated with harmful use of tobacco (cigarette smoking). The first exposure to heroin was 8 years back as an experiment with friends. After a month of daily use of injectable heroin, the patient started buying heroin for himself spending up to Rs 500 per day (which was 90% of his average daily income). He started using heroin alone, up to 0.5 gram per day, which was more than twice the amount of initial use, to get the desired effects of relaxation and euphoria. Over the next 2 years, he started to develop withdrawal symptoms after 6-9 hours if he did not take heroin. These symptoms included body ache, back pain, runny nose, stomach cramps, feeling restless, with lack of appetite and sleep, and constipation. The patient had been married during this time but was unable to abstain from heroin consumption despite his spouse's multiple pleas. He was therefore admitted to a deaddiction facility where he stayed for 1.5 months. However, he relapsed within a week postdischarge due to craving. In the last 8 years, there were four episodes of overdose requiring hospitalization, and several of his friends expired due to heroin overdose during this time. However, he was unable to refrain from the same, with a maximum period of abstinence being 1.5 months and three previous relapses. Thus, the patient was admitted at our center and started on opioid substitution therapy after obtaining informed consent. At the time of admission, multiple injection marks with thrombosed veins were noted on his upper limbs, and he was in a preparation stage of motivation.\nThe patient was started on 2 mg BPN built over 1 week to 18 mg along with nicotine patch 21 mg daily. From the third day of his admission, the patient was referred to the yoga facility in the institute. A 60-minute validated yoga program for ODS was included as an adjunct in his routine []. The program included practices of physical postures, sun salutations, relaxation techniques, regulated fast and slow yogic breathing practices, mantra chants, and yoga-based lifestyle counselling. One to one supervised yoga was taught for 5 days per week for the first 2 weeks, followed by tele-yoga practice of the same module (3 times per week) plus home practice postdischarge (with recorded videos) up to the 3rd month. After that, he continued practicing yoga on his own. An online booster session was offered to the patient every month after the 3rd month. The patient was followed up on a monthly basis over phone and was motivated to continue practicing yoga.\nTo evaluate how well the patient learnt yoga, the yoga performance assessment scale (YPA) [] was applied by the yoga therapist on each follow-up assessment. Plasma beta-endorphin levels were also assessed at baseline, after 2 weeks, and at the end of 9 months. The patient was followed up for the next 9 months with assessments of several clinical parameters including Buprenorphine dosage. Plasma beta-endorphin levels were also assessed at the baseline and after 2 weeks of supervised practice. Clinical assessments were performed by a psychiatrist. Standard procedures were followed for assessing endorphin levels []. Written informed consent was obtained from the patient. |
A 26-year-old Thai woman presented with a foreign body in her left orbit that had been retained for 8 months. Eight months previously, she had sustained a gunshot injury to her left eye. There was only a small wound on her left eyelid (Fig. ); she had normal eye movement, a normal visual field, and no pain. A plain radiograph was performed at that time; it detected a round-shaped metallic foreign body located medially in her left eye globe. She was then diagnosed as having a retained foreign body in her left orbit and she was advised to have clinical observation. However, 8 months later, she developed pain in her left eye without any sinonasal symptoms. After discussion about the risk of surgery and retention of an orbital foreign body, an ophthalmologist referred her to our department for the minimally invasive procedure option of removal of the foreign body using an endoscopic transnasal approach. On examination, the movements of her left orbit were not restricted and there was normal visual acuity (20/20) with no proptosis or chemosis. A computed tomography (CT) scan of her left orbit revealed a round-shaped metallic foreign body in the medial intraconal space, and lateral attachment of posterior ethmoid sinus, measuring 6 mm (Fig. ). A transnasal endoscopic approach, with navigator assistance (Brainlab), was used to remove the bullet. Uncinectomy and anterior-posterior ethmoidectomy were performed. The location of the intraconal metallic foreign body was confirmed with a navigator system, then part of the lamina papyracea was removed and the periorbita incision was done. The defect was enlarged and the fibrotically encapsulated bullet was found lying in the orbital fat (Fig. ). The fibrotic capsule was dissected, and the bullet was delivered through her left nostril with probes and curetted (Fig. ). No intraorbital bleeding or damage to any soft ocular structures was noted. A relative afferent pupillary defect was found in her left eye during removal of the foreign body, but it resolved and no postoperative complications were observed. |
A 62 year-old male had displayed bradykinesia and tremor of his right limbs for one year, during which he was able to perform limited fine movements such as dressing himself, lacing up his shoes and brushing his teeth. His tremors were aggravated by nervousness and relieved when asleep. He had had a history of hypertension and took a daily dose of 5 mg amlodipine. The patient had no history of any other chronic illnesses and was not on any other type of medication. Neither the electrocardiogram nor the Holter monitor showed any abnormalities. His baseline recumbent-upright blood pressure (BP) and heart rate (HR) were normal prior to treatment with piribedil, as shown in Table . He was diagnosed with PD based on the Movement Disorder Society clinical diagnostic criteria []. Initially, he received dopamine replacement therapy of 50 mg piribedil per day. Although there was no significant improvement in symptoms neither did he feel any discomfort. Therefore, starting the first dose change of piribedil, he added extra 50 mg to his dose. About two hours later after the first change in dose, the patient experienced symptoms of dizziness and sweating; he collapsed half an hour later. Whilst in a sitting position, the patient’s BP and HR were measured immediately. The BP reading was 85/48 mmHg and HR was 45 beats/min. His symptoms continued for the duration of the day with sitting BP fluctuating between 80–95 mmHg to 45–68 mmHg. Because his head computerized tomography examination found no abnormalities, the patient received 500 mL of 0.9% sodium chloride solution, after which his symptoms improved. Due to adverse drug reactions (ADRs), the patient was started on a second dose change of piribedil, i.e. an extra dose of 25 mg piribedil was to be taken in the afternoon in addition to the existing 50 mg taken in the morning. After two and a half hours, the patient experienced dizziness, sweating, nausea and vomiting, with a BP reading of 70/45 mmHg and a HR of 47 beats/min when sitting. His BP recovered to 105/65 mmHg and HR to 60 beats/min after elevating his lower limbs and resting for 20 mins. As a result of these incidences, the patient was switched to pramipexole to replace piribedil. After taking pramipexole 0.125 mg or 0.25 mg three times a day (tid), the symptoms of hypotension and bradycardia disappeared but a reduced amplitude of his right arm swing was still observed. Finally, after the pramipexole dose was increased to 0.375 mg tid, the patient showed marked improvements in motor symptoms. The changes in BP and HR are shown in Fig. . |
A 32-year-old healthy female reported with the chief complaint of a growth in relation to upper front teeth. It had been present for the last 1 year. Initially, the lesion appeared as a small swelling on the gums, and slowly grew to its present size. There was no history of pain but patient reported difficulties in speech and bleeding on brushing. She was very much concerned about her aesthetics due to unpleasant appearance of gums. She also reported history of cleft lip repair at the age of 10 years.\nExtra-oral clinical examination revealed no evidence of lymphadenopathy or other pathosis. There was a scar present on the upper lip due to cleft lip repair. Intraoral examination revealed poor oral hygiene and an erythematous growth present on the labial and palatal aspect of maxillary central incisors. The lesion appeared exophytic and nodular. The surface was irregular on palatal aspect and smooth on labial aspect. It was pinkish red in color, sessile and firm in consistency [Figures and ].\nIntraoral periapical radiograph was obtained of teeth #11 and #21. It revealed crestal bone resorption between central incisors and between right central and lateral incisors []. Complete blood counts were obtained and found to be within normal limits. The differential diagnosis consisted of irritation fibroma, pyogenic granuloma and peripheral giant cell granuloma.\nAn excisional biopsy of the lesion was planned, using a diode laser, under local anesthesia, after obtaining informed consent from the patient. Protective eyewear was used by the operator, assistant and patient. Full mouth scaling and root planing was done. The area surrounding the lesion was infiltrated with local anesthetic (Lignocaine 2% with adrenaline 1 : 80,000). The lesion was removed using diode laser (Picasso, AMD LASERS®, Indianapolis, USA) with 810 nm wavelength in contact mode and pulse of 30 ms duration and 30 ms interval with initiated tip at 2.0 Watts power []. Excised lesion was put in 10% formalin and sent for biopsy []. Adjacent root surfaces of central and lateral incisors were planed using Gracey curette #1-2. The operative field was irrigated with sterile normal saline [Figures and ]. Patient was advised to avoid hot and spicy foods for 3 days. No medications were prescribed.\nOn Hematoxylin & Eosin staining, hyper parakeratinized epithelium was observed with cellular stroma composed of plump fibroblasts, loosely arranged collagen fibres and varying sized blood capillaries engorged with erythrocytes. Small basophilic areas of calcifications were present in the centre of specimen suggestive of POF [Figures –].\nPatient was recalled after 1 week for post-operative evaluation. She did not report any discomfort at the operated site. No scar was observed, and healing was satisfactory. No tenderness was observed on palpation. After 1 year of follow up, there has been no complication or recurrence of the lesion [Figures and ]. |
A 7-year-old boy was evaluated for a two month history of atypical movements presenting in the evening, and lasting an hour after eating. These stereotypical movements with the head and chest bending forward and to the left side, accompanied by a grimace were associated with sensation of breath locking without cyanosis. Pain and dysphagia were absent. The neurological examination was normal. The possibility of Sandifer syndrome posturing occurring with gastroesophageal reflux disease (GERD) was considered but neither pain nor back hyperextension were associated with the atypical movements. PPI did not improve his symptoms. Various prokinetic agents (metoclopramide, motilium, cisapride and erythomycin) were also inefficient.\nUpper endoscopy and esophageal biopsy did not reveal eosinophilic esophagitis or other abnormalities. ENT exam was normal. A severe gastroparesis was demonstrated on milkscan study. Two 24 hour esophageal pH probe studies pointed out severe GER. High resolution manometric evaluation of the oesophagus revealed normal sphincter pressures and relaxations with no dysmotility of the esophageal body. Electroencephalography and polysomnography were normal. Because of the unexplained dyspnea associated with this abnormal posture, a head MRI was performed and revealed a CM I: cerebellar tonsils extending to 12 mm, with syringomyelia (D4-D5) (Figure ).\nFor a long period of time, the child abnormal movements were only considered to be tics and the CM I was considered a fortuitous finding. Since the child remained symptomatic despite medical treatment, it was eventually decided to proceed with surgery. The operative procedure was done one year after the onset of his symptoms. He underwent posterior fossa decompression with upper cervical laminectomy and expansion duroplasty. No postoperative complication occurred. Postoperative MRI confirmed adequate decompression. His atypical posture and dyspnea completely resolved in the week after surgery. More than two years after surgery, the child remains asymptomatic. The patient and parents have refused any further invasive testing (such as a control esophageal pH probe study) as the patient was symptom free. |
A 66-year-old African American female with a past medical history of refractory immunoglobulin G (IgG) lambda MM, essential hypertension, and chronic kidney disease presented to the emergency department with five days of right upper quadrant pain.\nHer MM was diagnosed one year prior when she presented with altered mental status, uremia, hypercalcemia, hypoalbuminemia, and paraproteinemia. A skeletal survey at that time revealed multiple thoracic spinal lytic lesions and an eroding soft tissue mass at the level of T10. Further evaluation revealed a very high IgG level, elevated M protein band, and a kappa/lambda ratio <0.01 (normal 0.26-1.65). A biopsy from the soft tissue mass revealed a plasmacytoma. Radiation therapy was initiated for 10 days. She received three cycles of bortezomib and dexamethasone followed by two cycles of bortezomib, dexamethasone, and lenalidomide. Her disease progressed, and a subsequent bone marrow biopsy revealed hypercellular bone marrow with 70% atypical plasma cells. The patient subsequently received seven cycles of carfilzomib, lenalidomide, and dexamethasone. She was not a candidate for bone marrow transplantation given the high plasma cell burden.\nOn her current presentation, the pain was sudden in onset, intermittent, worse with eating, and without radiation. The pain was associated with nausea and anorexia, but she was without any change in bowel habits. She denied any previous similar episodes. Upon physical exam, the patient was in distress but remained alert and oriented. Her vital signs were all stable. She exhibited right upper quadrant abdominal tenderness without rebound or guarding. Her initial labs are presented in Table . The patient was admitted to the hospital for further evaluation of her abnormal labs and supportive treatment.\nThe patient was started on intravenous hydration and was made nil per os. An abdominal ultrasound revealed a distended GB with sludge (Figure ). The GB wall was thickened up to 9.5 mm, and the sonographic Murphy sign was positive. The common bile duct and common hepatic duct measured 6.3 mm and 3 mm, respectively. The liver measured 18.3 cm and exhibited normal echogenicity. There were no intraparenchymal masses or fluid collections. The portal and hepatic veins were patent.\nThe patient was diagnosed with acute cholecystitis. Intravenous piperacillin-tazobactam 3.37 g every eight hours was initiated, and the patient was referred for open cholecystectomy given her overall condition and lactic acidosis. Intra-operatively, the GB was thickened and firm but not overly distended or perforated. The GB was dissected from the liver edge, and a liver biopsy was performed successfully.\nThe pathology report from the cholecystectomy revealed chronic cholecystitis with involvement of the GB submucosa and serosa by abnormal plasma cells with lambda light chain restriction (Figure ). Subsequently, Congo red stain of the GB sections revealed apple-green birefringence throughout the submucosal areas consistent with amyloid deposits. The liver biopsy exhibited abnormal plasma cells in periportal locations with lambda light chain restriction as well; Congo red stain was not done on the liver sample.\nThe patient declined any further treatment for MM and decided to proceed with hospice care. She was discharged home with comfort measures. |
An 80-year-old male presented with a mass in the right breast of 10 years’ duration with rapid progression in growth since the previous 3 months.\nMammography showed a well-defined mass in the right breast with ipsilateral axillary lymphadenopathy.\nUltrasonography of the abdomen and computerized tomography of thorax showed no abnormalities.\nFine-needle aspiration cytology showed cellular smears with dispersed single cells and loose sheets. Tumor cells showed moderate cytoplasm and indistinct cell borders. Many nuclei showed moderate anisocytosis, irregular nuclear contours, prominent nucleoli and sprinkled chromatin [Figure and ].\nConsidering these features, a cytological diagnosis of NE carcinoma of the breast was arrived at. Confirmation of diagnosis by histopathological examination and immunohistochemical study was suggested.\nThe patient underwent right radical mastectomy with axillary lymph node dissection. Gross examination revealed a tumor measuring 10 cm × 8 cm × 8 cm, with a solid, grey-white appearance and a focal cystic area []. Six lymph nodes were identified. Extensive tissue sampling was done from the breast tumor and the lymph nodes. Histopathological sections studied showed a malignant tumor with cells in solid nests, trabecular and organoid patterns, separated by delicate fibro-vascular connective tissue stroma. The tumor cells contained moderate amounts of eosinophilic cytoplasm and showed nuclear pleomorphism. The nuclei were round to oval with occasional grooving and irregular outlines. Many nuclei contained large prominent nucleoli and sprinkled chromatin []. Atypical mitoses ranging from 6 to 10/10 hpf were seen. A tiny focus showed a mucin pool with floating small nests of tumor cells. A focal area in the tumor also showed features of invasive papillary carcinoma []. Four out of the six resected lymph nodes showed metastatic deposits from the primary breast tumor.\nImmunohistochemical staining revealed cytoplasmic positivity for chromogranin which confirmed the NE nature of the tumor []. To exclude a non-mammary primary site, the chest, abdomen and pelvis were thoroughly examined for abnormalities, but none was detected.\nFinal diagnosis of primary pure solid NE carcinoma of the breast with a minor component of papillary carcinoma and metastasis to ipsilateral axillary lymph node was arrived at, following which the patient received adjuvant chemotherapy. |
A 60-year-old male patient was referred to the center with velopharyngeal inadequacy. The anatomic deficiency was due to surgical resection. The same was performed to treat squamous cell carcinoma. Surgical resection was done after the clinical findings and biopsy (histopathological findings) confirmed oral squamous cell carcinoma. The patient reported 4 months after surgical resection with the chief complaint of difficulty in swallowing and unclear speech. Following surgical resection the patient had also undergone radiation therapy (total of 44 Gy dose), which was completed 4 weeks before the patient had reported to the center. Extra oral inspection and palpation revealed no relevant findings. Intraoral findings revealed an anatomic deficiency of a portion of the hard palate and soft palate []. A triangle shaped defect extending from the midline (apex of triangle) and covering the junction of hard and soft palate on the left side was seen. The defect included the entire portion of the soft palate on the left side and 1 cm (from the midline) on the right side. The massive extent of the defect caused difficulty in swallowing [] and during speech (speech intelligibility score of 6 and hypernasality score of 5) [].[] Full complement of the dentition was present. Mild decrease in saliva content was noted.\nFor the decreased saliva content, the patient was advised to use a saliva substitute spray (Mouth Kote Dry mouth spray, Parnell Pharmaceuticals, Inc., USA). The patient was advised to use it by spraying in the mouth 3–5 times a day, swirl for 8–10 s each time and then spit out.\nFor the anatomic deficiency of hard palate, an immediate treatment plan was to provide continuity of the velopharyngeal mechanism by artificial prosthesis (speech aid appliance) in heat polymerized polymethyl methacrylate.\nPrimary impressions (in irreversible hydrocolloid) were made, and primary model was obtained in Type III gypsum. On the model, a heat polymerized tray, extending to cover the entire recorded area of the defect was formed. This tray was used to record the movement of the posterior pharyngeal wall (blue arrow) and a lateral pharyngeal wall (red arrow). For the same, low fusing compound was adapted to the posterior-most extent of the tray, and the patient was asked to perform side to side movement, bend head in front and back direction following a circular path. Following this the patient was asked to swallow. This helped to record the anterior and posterior tonsillar pillars, tori tubari, Passavant's ridge, and the anterior tubercle of the atlas. Shiny areas (that indicated the lack of tissue contact) were remolded.\nOnce satisfactory molding was ensured (by speech and swallowing), about 2 mm of the surface of the molded impression was reduced and tissue conditioner was applied all over the surface of the remaining impression. All the molding movements were repeated, and the molded tray was left in the patient's mouth for 1 day [Figure and ]. The following day the tray was removed and the impression recorded was processed in heat polymerized resin. Prior to polymerization of speech aid prosthesis, an index of the inferior (tongue) surface was made in polyvinyl siloxane (PVS) in putty consistency. The same was used to form the “lid” after hollowing the speech aid bulb.\nAfter the completion of polymerization of the speech aid prosthesis, hollowing of the speech aid bulb was done, and the inferior (tongue) surface was formed in self-cure resin as a separate lid. Self-polymerizing resin was adapted in dough stage over the previously formed PVS index as a flat sheet of 2 mm. The region of resin in contact with the index helped to duplicate the tongue impression during swallowing. The resin lid formed was removed from the PVS index and seated on the hollowed out speech aid bulb. This was done as the size of the speech bulb was large. If the entire bulb had been fabricated in heat polymerized resin, there was a chance of increasing the weight of the prosthesis.[]\nThe final speech aid prosthesis was inserted, and pressure spots were observed using a pressure indicating paste. The tongue surface was made a concave, and the superior surface was made convex and smooth to deflect the secretions into the oral cavity [Figure –].\nSpeech therapy (two sessions per week, each session lasting for 2 h) was performed by a speech pathologist for 1 month. To correct hypernasality tactile feedback was taught to the patient. Improvement in the speech was noted after 1 month. Improvement of swallowing was also evident during the follow-up. The speech intelligibility score and hypernasality score attained after the prosthesis and speech therapy was 1 each.[]\nA follow-up of 9 months revealed improvement in speech [] and a better control in swallowing with no nasal regurgitation []. An overall improvement in quality of life was evident with the patient showing a healthier state both physically (attributed to improved nutritional intake) and mentally (improved confidence). Continuous use of saliva substitute also showed a more lubricated oral cavity, facilitating easier deglutition. |
On 4 February 2018, a 39-year-old female without any underlying diseases was admitted to the hospital due to fever and dyspnea lasting 2 days and 5 h, respectively. This patient experienced the influenza-like symptoms 2 days prior to hospitalization, and requested an ambulance due to the gradually exacerbating dyspnea 5 h prior to admission. The results of the laboratory tests are shown in . Multiple invasive development in the lung tissue was demonstrated by computed tomography and X-ray examination (shown in ). After admission, endotracheal intubation was immediately applied as the rescue approach, and antimicrobial treatment (i.e., moxifloxacin) combined with antiviral therapy (i.e., oseltamivir) were administered. The detection of the influenza B virus in the tracheal secretion was performed through reverse transcription-PCR. Moreover, bronchial secretion and a blood sample were collected immediately for pathogen detection using both NGS at BGI-Shenzhen and methods of bacterial culture commonly used in the clinical microbiological laboratory. After 4 h, the influenza B virus was identified in the tracheal secretion through reverse transcription-PCR. Subsequently, the patient received an injection with dexamethasone and ECMO was used as life support 8 h after admission. Unfortunately, the patient expired 11 h after admission. The details of the treatment process are shown in . Twenty hours after admission, NGS at BGI-Shenzhen identified S. aureus in both the tracheal secretion and blood sample of this patient. Moreover, NGS identified 2,037 sequence reads (8.40934%) uniquely corresponding to the S. aureus genome; the identified S. aureus was a positive carrier of the PVL genes () (the virulence factors of S. aureus identified via NGS are shown in ). The PVL genes were also amplified through PCR using the blood sample of this patient as previously described (). In contrast, the commonly used bacterial culture methods were negative for the blood sample and tracheal secretion. Collectively, these results supported that the influenza virus B infection in this patient was complicated with fulminant pneumonia and sepsis caused by S. aureus infection. |
A 71-year-old male presents to the emergency department with symptomatic bradycardia and syncope. To note, he had altered mental status prior to arrival and was unresponsive upon arrival. The patient had experienced a syncopal episode in the parking lot of a restaurant after having dinner. His daughter caught him before he fell so he did not sustain a head injury. His past medical history includes atrial fibrillation, coronary artery disease, congestive heart failure, chronic kidney disease stage III, and chronic systolic heart failure (left ventricular ejection fraction 30%-35%). The patient had been prescribed to wear a LifeVest wearable cardioverter defibrillator but was described by family as non-compliant. As per emergency medical services (EMS), the patient’s heart rate was at 26 bpm and he was hypotensive. Administered atropine elicited no response. A transcutaneous pacer was placed, raising his heart rate up to 70 bpm, but showed intermittent capture with an amplitude up to 175.\nIn the resuscitation bay, the patient was hemodynamically unstable with a heart rate as low as 20 bpm. He showed signs of anasarca with facial swelling, 3+ pitting edema in the extremities, and a firm edematous abdomen. He was hypotensive with a systolic blood pressure of 50 mmHg and was significantly short of breath. He remained unresponsive and was intubated for airway protection. A one-liter normal saline bolus was given with minimal effect on the blood pressure. As the transcutaneous pacer continued to capture poorly, the patient began to further decompensate. The possible use of a transvenous pacer was under consideration for stabilizing the patient’s heart rate; however, isoproterenol was administered instead. The isoproterenol improved both his heart rate and blood pressure, and the transcutaneous pacer was turned off. However, it became evident that the patient was hypothermic with a body temperature of 30.6 °C. Lab results indicated significant hypothyroidism with a high TSH of 52.2 uIU/mL [normal 0.358-3.742] and low free T4 of 0.64 ng/dL [normal 0.76-1.46]. The combination of signs and symptoms strongly pointed to the case of MC.\nThe patient’s family confirmed that there was no family history of hypothyroidism, but reported that the patient had a 14-year history of taking 200 mg amiodarone daily for atrial fibrillation. The family also recounted a hospital admission in February 2018 for upper extremity cellulitis. The patient’s lab work noted a depressed thyroid panel with an elevated TSH of 15 uIU/mL and low free T4 of 0.64 ng/dL. Previous TSH for patient earlier that year was within normal limits. His previous hospital visits suggest cellulitis as the precipitating factor for the amiodarone-induced MC.\nOnce the MC diagnosis was confirmed, 75 mg IV-levothyroxine was administered with 50 mg IV-solucortef. The patient was then transferred to the ICU. Following clinical course, the patient’s heart rate and blood pressure rapidly stabilized. Levothyroxine was titrated down, and within several hours of admission, his temperature normalized at 36.3 °C. The patient was extubated soon after stabilizing. Over a clinical course of three days, the patient’s TSH normalized to 6.06 uIU/mL at which he was then transferred to a tertiary care medical center to manage his case further due to the complexity of his issues. |
A 57-year-old man with cough and minimal whitish expectoration was diagnosed with adenocarcinoma after a biopsy was conducted on the upper lobe mass of his left lung on March 4, 2016. Mutational analysis revealed that he lacked EGFR mutations or ALK gene rearrangements. CT scans showed that this patient had developed multiple metastases, including mediastinal and hilar lymph nodes, and also to the right intrapulmonary lymph nodes. He received 6 cycles of Pem-Cis chemotherapy, followed by thoracic radiation. Review of the CT scan at completion of sequential chemoradiotherapy treatment resulted in a significant shrinkage in the primary tumor in his left lung, while simultaneously resulting in a slight increase in metastatic lymph nodes (Fig. ). In February 6, 2017, CT scans showed that the tumor progressed rapidly. He was then scheduled to receive gemcitabine plus nedaplatin (Gem-Ndp) chemotherapy. However, the patient had to discontinue the planned 2nd cycle of this regimen as he developed moderate pneumonia. After systemic antibiotic treatment, the patient improved symptomatically and became increasingly energetic. However, CT scans on March 29 revealed that his tumors had progressed further. Owing to his poor physical condition after Gem-Ndp treatment, the chemotherapy regimen was changed to Pem-Cis. However, CT scans on May 11 failed to detect any shrinkage in his tumor. In addition, lymph node metastases increased post-treatment. The patient subsequently started standard of care pembrolizumab treatment at 2 mg/kg intravenously every 3 weeks for 6 cycles. During hospitalization, the general condition of the patient was good, with signs of fatigue only present at the first 2 days after each pembrolizumab infusion. The treatment failed to result in an anti-tumor response. The patient then received 2 cycles of initially used chemotherapy regimen Pem-Cis. Interestingly, we detected a significant shrinkage in the enlarged mediastinal and hilar lymph node metastases, with the primary site in the left lung exhibiting no further progression.\nRecent studies have suggested that clinical responses are associated with several potential biomarkers, including PD-1, PD-L1, and CD3.[ To investigate the clinical association of these factors with the efficacy of PD-1 blockade, we performed immunohistochemistry analysis of PD-1, PD-L1, and CD3 in specimens obtained from this patient. The results showed that the tumors from exhibited CD3+ T cell infiltration, but no PD-1 or PD-L1 expression (Fig. ). |
P, a seven-year-old male child, was brought by his parents with chief complaints of repetitive meaningless body movements, hand flapping, poor language/communication development, not managing his activities of daily living, not having peer group/family interactions. Based on the history, clinical interview and assessments, he was diagnosed as having childhood autism.\nThe baseline assessments were done using various rating scales including childhood autism rating scale (CARS), vineland social maturity scale (VSMS) and visual analogue scale (VAS) as per clinician observation and parental reports. On VSMS, he obtained a score of 59. On CARS, his score was 35 indicating mild autism. It was revealed that he was partially dependent for all his basic activities of daily living, had marked language and communication deficits. He could understand one command instructions. The target behaviors identified during this stage included repeatedly moving his head, flapping his hands along with training in self help and communication skills.\nIntervention process was carried out in thirty-two sessions over a period of three months using PECS along with traditional behavioral techniques such as DRA, reprimand and task direction. The intervention program was so planned that it targeted the specific areas of improvement in his communication, self help skills and decrease behavioral problems.\nPECS was carried over the six phases: In Phase 1 called “physical exchange phase,” the therapist worked as a communiation partner and based on reinforcer sampling, made a picture of the reinforcer which was placed under a clear container, so the child could see it, but not get it. When the child looked interested in the item, the therapist gave the child the picture card. Then the child was prompted to hand the picture card back to the therapist who after receiving the card, verbalized the request aloud ("You want biscuit! You can have it!"). At this point, the requested item was given to the child. In Phase 2 (expanding spontaniety phase), therapist moved slightly away from the child so that the child had to move toward the therapist to place the picture card in his hand. During Phase 3 (discrimination training) the child was given more than one picture card and he had to choose a desired object, and then gave that card to the therapist. Initially, he had a hard time distinguishing between the two pictures; however, eventually he could do that without any difficulty. In the next phase Of “sentence structure”, the child was given a card with the phrase “I want ____” on it. This card now had to be used with the picture card showing what was desired. This was done so that the child would learn communication using complete sentences. Even though the child could not yet read, he gradually learnt to recognize the words as sight words on the cards. In Phase 5 the child was directly asked “What do you want?” and the child had to hand a picture card to enable the child know how to communicate his desires. After the five phases, PECS was generalized to more than one therapist, and he was also taught how to communicate his experiences outside the therapy room.\nSince on analysis it was found that most of his stereotyped behaviors were relatively independent of social consequences, techniques such as DRA, reprimand and task direction were also used. Using DRA, he was reinforced contingent upon the performance of a desirable behavior mentioned in his activity schedule. When a pre set number of alternative behaviors were met, he was given an activity reward. In the initial phase, a fixed reinforcement schedule was followed which was later changed to variable interval schedule. The edible reinforcer was given intermittently, and replaced with verbal praise. Throughout the sessions, all stereotyped responses received an immediate verbal reprimand and task direction. It involved calling his name and directing his attention back to the task at hand. |
A 73-year-old female was hospitalized for aortic valve replacement to fix severe aortic regurgitation and stenosis. She had been subject to follow-up after being diagnosed with hypertension, aortic regurgitation, and stenosis five years earlier. During the follow-up, surgery was decided due to worsening symptoms and echocardiographic diagnosis. She was diagnosed with early gastric cancer in the antrum and was scheduled to undergo gastrectomy after recovery from heart surgery. Standing 144 cm tall and weighing 50 kg, the patient showed vital signs within normal ranges except for signs of anemia, with a hemoglobin level of 8.5 mg/dl and hematocrit at 25.4%\nPrior to anesthetic induction, she was administered 2 mg of midazolam by intravenous injection for sedation. Preoxygenation was performed through nasal cannula at 3 L/min. The right radial artery was secured, and invasive arterial pressure monitoring was begun. Prior to induction, the patient showed stable vital signs: an arterial blood pressure of 138/70 mmHg, a pulse of 66 beats/min, a breathing rate of 18 breaths/min, and 100% oxygen saturation of the peripheral vein. After administering 3 mg of midazolam additionally, 2% propofol was infused using a target-controlled infusion pump (TCI pump) with a target plasma concentration of 3.0 µg/ml. At the same time, induction was performed by administering 500 µg of fentanyl diluted to 5 µg/ml in the burette infusion set by drip infusion. After allowing muscle relaxation with 10 mg of vecuronium and checking for stabilization of the patient's vital signs, intubation was performed followed by mechanical ventilation with O2-medical air (fraction of inspired oxygen at 0.5).\nIn order to expand the internal jugular vein for central venous catheter insertion, the patient had her right arm fixed to the trunk in the Trendelenburg position. With a small pillow placed underneath the right shoulder and head turned to the left, the position facilitated access to the right internal jugular vein. A venipuncture site was sterilized for an aseptic procedure. Left-handed palpation was performed on the internal carotid artery, lying between and beneath two bodies of the right sternocleidomastoid muscle and located 3 cm above the superior border of the clavicle. An 18-gauge central venous needle was inserted towards the ipsilateral nipple at a point 1.5 cm medial to the external jugular vein. A 5 ml syringe connected to the inserted needle was used for aspiration, and the color and pressure of the blood confirmed that it was venous blood. After removal of the syringe, a J-tip guide wire was inserted with its end pointing toward the heart. The guide wire was inserted smoothly up to 20 cm. After removal of the needle and the incision on the skin, a 9-Fr introducer sheath (Advanced Venous Access HF®, Edwards Lifesciences, Irvine, USA) accompanying dilator for the catheter was pushed in along the guide wire. There was a slight feeling of something getting stuck in the middle, which is normally experienced during successful central venous catheter placement. The insertion was made without much resistance, and the aspiration of blood from all three lumens of the catheter was successful. An intravenous catheter (PreSep™-central venous oximetry catheter set, Edwards Lifesciences, Irvine, USA) with each of its lumens filled with saline solution was inserted up to 20 cm through the introducer. Then, it was pulled back 3 cm to check again for blood aspiration. No noticeable resistance, arrhythmia, or changes in vital signs were observed during the insertion of the intravenous catheter. Monitoring of central venous pressure or central vein oxygen saturation was yet to be performed, and there were no signs of abnormal placement of the central venous catheter or other complications related to the procedure. However, a portable X-ray performed immediately after the procedure revealed that the end of the catheter was inserted to the ipsilateral subclavian vein ( and ). The authors first considered removing the inserted catheter entirely and using the left internal jugular vein but finally decided to correct the placement under image guidance with the catheter inserted after taking various circumstances into account.\nIn order to correct the placement of the catheter, the inserted intravenous catheter was removed. The J-tip guide wire was carefully inserted, once again, about 15 cm through the catheter introducer. The end of the catheter was pulled back to the internal jugular vein () and temporarily positioned. While a continuous X-ray was performed using a mobile-type diagnostic X-ray apparatus (C-arm fluoroscope), the end of the J-tip guide wire was pulled back to the superior clavicle. Then, the J-tip was rotated 180° towards inside and carefully inserted to the superior vena cava (). This was followed by a successful attempt to position the end of the catheter near the junction of the right atrium along the corrected guide wire ( and ). The entire process from catheter insertion to correction was executed aseptically under single venipuncture. The time spent correcting the malpositioning was less than 10 minutes.\nNo abnormal signs such as edema or hematoma were found on the patient's right shoulder and arm until the surgical procedure came to an end and she left the room. The patient had her central venous catheter removed one week later and was discharged from the hospital after receiving subtotal gastrectomy in the surgical department one month later. |
A 60-year-old man with a history of asthma, benign prosthetic hypertrophy, and hyperlipidemia presented 1.5 years after an uncomplicated primary right TKA done by an outside surgeon. He had been complaining of 4 months of increased pain in his right knee. An aspiration had been attempted, yielding 1 mL of sanguinous fluid which had not been sent for analysis. The patient continued to have swelling and increased pain in the knee, and an MRI was obtained demonstrating “pseudotumor” (Figure , A–C). He was then referred to our orthopaedic oncology office for further evaluation and management.\nAfter review of initial radiographs (Figure , A and B) and CT (Figure , A–C), the patient underwent an open biopsy of his right tibial lesion adjacent to the tibial baseplate one week after presentation to the office. Pathology from his initial biopsy was consistent with GCTB. One week following his open biopsy, the patient underwent a complex reconstruction of his proximal tibia as well as patellar tendon (Figure , A and B).\nIntraoperatively, complete destruction of the medial cortex of the tibia was noted, with the area infiltrated extensively by tumor. After the initial anterior exposure through the previous TKA incision, the area was extensively curettaged. A high-speed burr and argon beam coagulator was then used to complete the resection at the edges of the cavity. Following the removal of the mass, we noted that the tibial baseplate was mechanically stable even after the extended curettage. An intraoperative determination was made to preserve the primary arthroplasty components and to reinforce the tibia with cement and Steinmann pin fixation. Steinmann pins were fired distally into the tibia, which allowed buttressing of the tibial baseplate proximally. The entire excisional cavity was then packed with polymethyl methacrylate (PMMA) cement. Intraoperative examination demonstrated that the construct had excellent stability and strength afterward.\nFollowing reconstruction of the proximal tibia, attention was turned toward the patellar tendon. We noted that the destructive process had eroded much of the patellar tendon and reconstruction was required. Marlex mesh was used in the technique described by Browne and Hanssen. The mesh was layered into a construct with approximate width as the patellar tendon and then weaved into the remnant of the native patellar tendon into normal tendon tissue. #5 Ethibond suture was used to reinforce the closure and attachment of the Marlex mesh to the tendon, avoiding the placement of mesh adjacent to skin.\nBefore discharge, the patient was placed in a long leg bivalved cast. Three weeks postoperatively, the patient was transitioned into a hinged knee brace, which is locked in extension while upright. The patient was then instructed to allow for bed dangles with the knee. At 6 weeks post-op, the patient began physical therapy for gentle range of motion of the knee, still with brace locked in extension while ambulating. At 7 weeks, the patient was placed on Keflex for 1 week after he noticed a small amount of discharge from his distal incision site after a scab was removed, with resolution of symptoms. Three months post-op, the patient was allowed to weight bear as tolerated on his extremity. At this time, he was started on a trial of denusumab (Amgen Manufacturing Limited) adjuvant chemotherapy under the medial guidance of his oncologist. The patient developed a rash after two doses and was changed to zoledronic acid (Zometa; Novartis Pharmaceuticals Corporation) for a total of 6 months of diphosphonate therapy. He completed the course without further incident. Radiographs taken at 16 months demonstrated maintained alignment without evidence of component subsidence or implant failure (Figure , A and B). At a 20-month follow-up, the patient was weight bearing on the extremity without assistance, using a cane only for long distances. |
A 48-year-old Caucasian female with a 10-year history of MF was referred to our bone marrow transplantation clinic for evaluation of biopsy proven large cell transformation of her MF. On presentation her only symptom was itching at sites of MF plaque-like lesions which involved her trunk and extremities in addition to tumor formation on her neck as well as left lower abdomen, right shoulder, right upper back, and right mid back. At time of referral, she was on topical treatment with UVB therapy. Her positron emission tomography (PET) scan showed multifocal lymphadenopathy in the abdomen and chest in addition to multiple nodular lesions in both lungs suspicious for lymphoma. A skin biopsy done earlier showed large cell transformation with CD4 positive T-cells which show aberrant loss of CD3 and CD7. The density and distribution of T-cells was consistent with tumor stage MF. Based on the above findings the patient was diagnosed with stage 4 MF with large cell transformation.\nSystemic therapy was initiated with cyclophosphamide, adriamycin, vincristine, and prednisone (CHOP) regimen and due to limited response to first cycle of CHOP, etoposide was added to her second chemotherapy cycle. With continued limited response her chemotherapy regimen was switched to ifosfamide, carboplatin, and etoposide (ICE) as salvage therapy. Repeat PET showed improvement of abdominal and lung lesions following which she received an ablative preparative regime of fludarabine, total body irradiation (1320 cGy), and Cytoxan and sUCB (single unit umbilical cord blood) transplant. Mycophenolate was stopped at day +30 and cyclosporine was tapered following day +100. Posttransplant course was complicated by development of acute skin and gastrointestinal graft-versus-host disease (GvHD), which responded to steroids. Her day +60 peripheral blood chimerism analysis revealed 100% donor cells. Posttransplant day +100 PET scan showed complete remission of her lymphoma.\nFive months after transplant she experienced recurrence of several erythematous plaque-like lesions involving her flanks, back, left neck, proximal inner right thigh, and left posterior thigh. She was off posttransplant immunosuppression at this time but was on steroids for treatment of her GvHD. A repeat biopsy of the lesion showed a mix of tumor and plaque stage MF. T-cell immunophenotype was CD4 positive and CD3/7/8 negative with scant CD30 positivity which was consistent with her pretransplant skin biopsy phenotype. Repeat chimerism analysis on bone marrow continued to show 100% donor cells. Steroids were slowly tapered off and she was started on escalating doses of interferon therapy and also bexarotene without systemic chemotherapy. Skin lesion's chimerism analysis showed 70% donor cells consistent with trafficking of donor cells to skin lesions.\nShe is currently 2 years after transplant and continues to have CTCL skin lesions. Her latest skin biopsy performed as a part of annual posttransplant evaluation continued to show MF; however, her bone marrow biopsy then showed 100% donor chimerism without any evidence of lymphoma confirmed either by morphology or by flow cytometry.\nLastly, to further investigate our case we performed T-cell gene rearrangement study on pre- and posttransplant skin specimens according to national guidelines. The study proved that lymphoma cells from posttransplant skin lesions were the same clones from pretransplant skin lesions based on the size and the region of peaks as shown in . |
A 39-year-old male with skin burns on his right arm and right hemiabdomen has been referred from the emergency room. Injuries were caused by contact with high voltage (220 kV) electrical current via fishing rod he held in his right hand. Lab tests upon arrival showed high values of serum bilirubin and low values of iron, while ECG and other lab results had normal values. Initial liver ultrasound, done one hour after the electric shock, was normal (GE, USA). However, due to the unstable condition and severe burns on the patient's right hemiabdomen, the first ultrasound was limited in time, quality, and subjectivity of the radiologist. This would explain why there was no substantial tissue destruction visualized with the first ultrasound, as expected in the liver injury caused by Joule heating. A day after, a follow-up abdominal ultrasound showed an extensive liver damage, which presented on the ultrasound as a hyperechogenic, homogenous, nonlinearly shaped focal lesion, situated in segments VIII and V, sized 7 cm × 8 cm (). In hepatorenal recess a small collection of fluid was observed (). Doppler imaging in the described area did not show signs of vascularization centrally, with only slight signs of vascularization around periphery (Figures and ). US was followed with CT of the abdomen, done on multilayered Siemens apparatus (Erlangen, Germany) in three phases: noncontrast phase, arterial phase, and portal venous phase. On noncontrast CT phase lesion was poorly visualized, shaped as a flower with a lower density than liver parenchyma (11–50 HJ) with larger dimensions, 11 cm × 14 cm (). In arterial phase liver lesion was better visualized. Central parts of the lesion after the contrast has been administered remained hypodense (23 HJ), while margins of the lesion were intensively hyperdense, up to 116 HJ (). In portal venous phase, peripheral part of the liver lesion became washed out, except in the central hypodense parts. This liver lesion was marked as focal coagulant necrosis (). |
Patient B was a 74-year-old woman with a history of breast cancer that had been treated with curative intent 10 years earlier. She was referred with shortness of breath. A CT scan showed multiple lung nodules. In addition, a PET-CT scan showed 4 PET-positive lesions in the right lower lobe, as well as 2 nodules in the right middle lobe and an enlarged PET-positive lymph node in station 7, which were suspicious for metastatic breast cancer lesions. Mammography of the right breast showed no local recurrence of the tumor. EUS-FNA of station 7 was performed using a 19G needle. Directly after the procedure, the patient experienced pain and was admitted for observation. A chest X-ray showed subcutaneous emphysema on the left side of the neck. A non-contrast CT scan showed pneumatosis in the mediastinum, and pleural effusion and atelectasis on both sides (). The laboratory results demonstrated a CRP of 600 mg/L and a WBC count of 22×109/L. The patient was placed on a fasting regimen and intravenous antibiotics were started. The patient experienced clinical deterioration and was admitted to the intensive care unit (ICU) 2 days after the EUS-FNA. She was septic, with a blood pressure of 90/50 mm Hg, a regular heart rate of 110 beats/min, a temperature of 37.7°C, and a respiratory rate of 21 breaths/min. Upper gastrointestinal endoscopy revealed a laceration of the esophagus at 27 cm from the incisor teeth. Consequently an 8-cm colon stent was placed endoscopically. The next day, a chest tube was placed in the left pleural space, draining 400 mL of turbid fluid immediately. Then, u-VATS debridement of a right-sided thoracic empyema was performed and 2 chest tubes were placed in the posterior mediastinum. Postoperatively the patient returned to the ICU. On the following days, the drains kept producing excessive turbid fluid. On day 4 after surgery, upper gastrointestinal endoscopy was repeated; the stent appeared to have been displaced and had to be replaced slightly more proximally. The chest tube in the left pleural space could be removed 6 days after surgery. The next day, another chest CT scan was performed due to an increase in production in the upper chest tube, and demonstrated a small area of fluid collection around the stent and pleural fluid around the lower lobes of both lungs, with atelectasis on the left side. The decision was made to debride the left pleural cavity in combination with opening and draining the posterior mediastinum via u-VATS. Two 28F chest tubes were placed. Because of the size of the esophageal defect and the continuing mediastinal and thoracic leakage, it was decided to laparoscopically place a gastrostomy for gastric decompression and a feeding jejunostomy. Thereafter, a fast recovery and normalization of inflammatory markers was observed. At 14 days after the last surgical procedure, a CT scan with contrast showed no leakage. The gastrostomy could be removed, and the patient was admitted to the rehabilitation center. The esophageal stent was removed 2 months after initial placement. Upper gastrointestinal endoscopy did not show a remaining esophageal defect and the patient returned to a normal diet. The pathology report of station 7 revealed metastatic breast cancer. The patient is currently receiving treatment for metastatic disease. |
A 31-year-old woman was admitted to our hospital with a complaint of sluggishness, memory decline, and weakness in the right limbs for >20 days. Three months prior, she had experienced sudden numbness in the right limbs and was diagnosed with acute infarction in the left thalamus. Since then, she had received oral daily treatment of 100 mg aspirin plus 75 mg clopidogrel as antithrombotic therapy. She had no medical history of hypertension, diabetes, heart disease, smoking, or alcohol use. After hospitalization, neurological physical examination showed slight speechlessness, decline in recent memory and calculation, tongue deviation to the right when extended, and slight numbness in her right limbs. Routine laboratory examination showed no significant abnormalities, while cranial magnetic resonance imaging (MRI) showed a new infarct in the anterior portion of the left thalamus, with old infarcts in the left thalamus and right cerebellar hemisphere (). Head computed-tomography angiography (CTA) and digital subtraction angiography (DSA) showed a localized, small protuberance at the V3–V4 junction area of the left VA ( and ), which was thought to be a dissection on high-resolution (HR) MRI (). Carotid ultrasonography and CTA both showed that the left VA was normal when the neck was in the neutral position (), but was occluded when the neck was rotated to the right (). Atlanto-occipital fusion and basilar invagination were also observed on CTA (), and MRI showed the left atlas lateral mass was small with dysplasia (). The patient was given anticoagulant therapy (150 mg twice daily) with dabigatran and a neck brace to prevent excessive neck activity. At the 3-month follow-up, the left VA was still occluded during neck rotation, although the patient had not had stroke recurrence. She then underwent atlantoaxial fusion (), and at 6 months after surgery repeated CTA showed disappearance of the small protuberance at the V3–V4 junction of the left VA (). |
A 47-year-old Han woman was admitted with weakness in the right lower limb and numbness in the left lower limb for more than 20 days. More than 20 days earlier, the patient presented with weakness in the right lower limb without cause. Half a day later, numbness was observed in the left lower limb accompanied by a cold sensation. No defecation disorders were observed. Lumbar MRI at an external hospital indicated protrusion of the intervertebral disk in the S3-4 and S4-5 regions. The patient did not improve after lumbar disease was treated and had aggravated weakness in the right lower limb and walking difficulties. No abnormalities were observed in the past medical history. Physical examination: Vital signs were stable. Cranial nerves were normal. Grade 4 muscle power was found in the right lower limb, while grade 5 muscle power was revealed in the left lower limb. Normal muscle tone was found in all four limbs. The knee reflex was exaggerated in her right lower limb. Pain was reduced below the left sternal angle. Proprioception and vibration sensation in the right lower limb were diminished. The right Babinski sign was positive. The results of a routine blood test, hepatorenal function examination, electrolyte examination and antinuclear antibody spectrum were normal. Serum herpes, CMV and EBV DNA levels were negative. The CSF cell count was 1 × 106/L, and the protein level was 328 mg/L. CSF smear and acid-fast staining were negative. CSF culture, OB and AQP4 antibody were negative. Brain MRI was normal. Cervical MRI indicated hyperintense and enhanced signals in the C7-T2 region (Fig. a–d). Myelitis was considered. The patient was administered methylprednisolone (1000 mg for 5 days) and then prednisone (55 mg, orally, once a day, reduced by 10 g per week). After methylprednisolone pulse therapy was given, the symptoms were significantly improved. Fifteen days later, cervical MRI was rechecked, indicating that the lesions were significantly reduced (Fig. e–h). The patient recovered and was discharged. |
A 59-year-old man with complaints of epigastric discomfort for 5 months underwent upper endoscopy in a local hospital in July 2018; the gastroscopy report described a “foreign body in the mucosa of the esophagus”. At that time, a nematode body was removed under endoscopy, and parasitic infection was considered. The removed worm body was discarded without further detection. The patient was then referred to our hospital after approximately two weeks in August 2018, and gastroscopy was recommended again.\nThe endoscopy showed a white movable worm-like object adhering to the esophageal mucosa at approximately 35–38 cm from the incisors, which was more clearly depicted by narrow-band imaging (Fig. a, b). Observing the peristalsis of the worm, we were able to distinguish its head and tail, and the worm was carefully removed in one piece using biopsy forceps to grab the head and placed in formalin (Fig. c, d, Additional file ). Parasitologists at our hospital confirmed that the object was a mature female G. pulchrum individual, which may have been living in the body for at least 1 year.\nNo other worms were found in the patient’s oral cavity, esophagus, stomach or duodenum. Physical examination and clinical examination revealed no abnormalities, and no parasite eggs were observed in multiple stool tests. This patient had no high-risk factors for esophageal cancer, such as smoking, flushing after drinking, or tumor history. He was a farmer in Hebei Province, China, and he had not traveled abroad. His living environment was relatively poor, and he also admitted to drinking unboiled water and eating uncooked vegetables that he grew with human feces fertilization. No animal was kept in his house. We suppose that the route of transmission was accidental ingestion of water or food contaminated by intermediate insect hosts.\nIn addition to the worm, this patient also had a large area of early esophageal cancer around the esophagus, which was 19–25 cm away from the incisors (Fig. e). Magnifying gastroscopy and ultrasound endoscopy indicated that the lesion was limited to the M2 layer (Fig. f), and no lymph node metastasis was found on enhanced computed tomography (CT). The patient underwent endoscopic submucosal dissection (Figs. g and ). The pathological report revealed M2-infiltrated early esophageal squamous cell carcinoma of the esophagus, with focal infiltration of the muscularis mucosa but no complete infiltration. As no tumor tissue was present at the resection margin, curative resection was achieved. The squamous cell carcinoma lesion did not contain any parasites.\nForty-eight hours after the operation, the patient began taking prednisone acetate 30 mg Qd to prevent stenosis and albendazole for 7 days as antiparasitic treatment. The patient developed esophageal stenosis after surgery, though it steadily improved after multiple dilations. There was no parasite recurrence after repeated gastroscopy, and the patient’s symptoms disappeared. |
A 68-year-old diabetic man, who had been on dialysis for 3 years, presented with a five week history of severe back pain that was unresponsive to bed rest, analgesics, and bracing. Lateral extension and lateral flexion radiographs revealed a T12 vertebral body fracture with an interbody vacuum cleft showing mobility. The cleft had been present right after an injury-falling on his buttocks - and gradually increased in size on sequential plain films ().\nThe patient was placed in the prone position under general anesthesia. Under C-arm confirmation, through a very small longitudinal skin stab incision on the bilateral pedicle, using a cannulated drilling system and a dilation technique, 1 mL syringes (JMS Co., Ltd., Tokyo, Japan) were set into both sides of the pedicle. The vertebral body was irrigated by saline, which was injected into one of the pedicles. A return stream was allowed to exit on the contralateral pedicle (). It was also irrigated from the opposite side of the pedicle. We routinely try to remove sequestered hematomas by saline irrigation so as to increase the potential space for CPC and prevent increased intra-vertebral body pressure, which can potentially cause pulmonary embolism and extravasation of CPC. Additionally, the presence of a hematoma and a low powder/liquid ratio in the CPC decreases the final compressive strength of the CPC []. These conditions may cause failure of CPC-assisted vertebroplasty.\nA pediatric uromatic balloon, size 8 Fr (Medicon Co., Ltd., Osaka, Japan) was then introduced into the cavity and inflated using contrast medium (). Approximately 3 ml of the contrast medium was necessary. The balloon inflation further enlarged the pre-existing fracture cavity and made it more apparent. Obstructing one side of the pedicle with a finger made it impossible to inject saline from the other side, and the cavity was considered to be closed except for the bilateral pedicle route.\nBefore starting to knead the CPC, the pediatric uromatic balloon was once again introduced into the created cavity and inflated to achieve hemostasis inside the vertebral body. CPC (Biopex R, Mitsubishi Materials Co., Ltd., Tokyo, Japan) injections were done using a small hand-piece cement-gun after 1-3 minutes of kneading at room temperature. We injected 6 ml of CPC and the surplus CPC flowed out of both syringes. The patient was able to begin ambulation on day four postoperatively using a brace. shows a postoperative radiograph and a computed tomography (CT) scan. Postoperative correction loss continued until 6 weeks postoperatively. Bone union was obtained at 8 weeks postoperatively. shows a 1 year postoperative radiograph and CT scan. |
A 39 year-old woman, known case of neurofibromatosis from childhood, was admitted to the emergency department because of one-week history of intractable bloody diarrhea. She had suffered from chronic intermittent bloody diarrhea for eight years. Within these years, she received 5-aminosalisylate drugs irregularly with only slight improvements in her complaints. In our first visit, her major complaint was bloody diarrhea and colicky abdominal pain. Symptoms such as nausea, vomiting, anorexia, fever, or fecal incontinence were not mentioned. She had more than eight episodes of diarrhea each day and also nocturnal episodes, interfering with her sleep, for a week prior to the visit. Her stool was mixed with bright red blood.\nShe remembered no important childhood or adulthood illness except a systolic hypertension diagnosed eight months ago and controlled by metoprolol 25 mg/day. She was not a smoker and did not mention alcohol consumption. She had a history of three admissions for resection of schwannoma of peripheral nerves in lower extremities. One of her daughters had skin lesions compatible with neurofibromatosis. There was no positive family history for Inflammatory Bowel Disease (IBD).\nThe patient was a middle-aged woman, ill appearing and cachectic. Her vital signs were stable. Physical findings on examination of heart and lung were normal. Abdomen was soft and on superficial palpation, a plenty of freely movable, soft, and non-tender subcutaneous and intradermal nodules of varying sizes were detected. She had left lower quadrant tenderness. On skin examination, there were widely distributed freckles and hyper pigmented macula and patches with sharp border of variable sizes (). The hyper pigmented brown patches were mostly seen on abdominal and lumbosacral areas. Extraintestinal manifestations related to IBD were not detected.\nAfter admission, the patient was ordered to take nothing by mouth and parenteral fluid administration was started for her. Laboratory data were as the followings: WBC 5600 with 20% lymphocytes, Hb 12.8 g/dl, MCV 79, Platelet count 456000, BUN 24 mg/dl, Cr 0.8 mg/dl, Na 138 meq/l, K 4 meq/l, Ca 9 mg/dl, P 3.9 mg/dl, Albumin 3.1 g/dl, AST 13 U/l, ALT 8 U/l, and ESR 24 mm/1st h. Stool exam showed many WBC and RBC without any evidence of parasites and stool culture was negative for infectious colitis (shigella, salmonella, yersinea, and campylobacter). A plain abdominal x-ray was taken and there was no abnormal finding. The patient's chest x-ray was also normal.\nShe received hydrocortisone 100 mg Q8h and ceftriaxone 1 gr BD intravenously, and subsequently lactase free and fiber free diet with sulfasalazine was started on the second day. She was closely monitored at emergency department. After negative stool culture result, ceftriaxone was discontinued. On the third day, symptoms were subsided and the patient was transmitted to the ward and received colon preparation. On the fifth day she underwent total colonoscopy and ileal intubation. In colonoscopy, avascularity, erythemathous and edematous mucosa, and ulceration with pseudopolyps up to 30 cm from anal verge were seen (). Terminal ileum was normal. Multiple biopsies were obtained from rectum and sigmoid mucosa. In microscopic examination the lamina propria was infiltrated with lymphocytes and plasma cells. The crypts showed goblet cell depletion, cryptitis, and crypt abcesses () and inflammatory pseudopolyp was seen in other sections ().\nOne week after admission, she discharged from hospital while on prednisolone 50 mg and sulfasalazine 4 gr, daily. Corticosteroid was tapered and discontinued about 1.5 month later and during about 2 years follow up the patient was symptom free on sulfasalazine. Since the patient was cachectic, abdominopelvic Computer Tomography (CT) scan was done for her. Bilateral masses were identified within psoas muscles, which were sharply marginated and homogenous, with water attenuation. Also, a few subcutaneous neurofibromas were present on right flank (). More Caudal CT scan showed bilateral presacral and nerve root foraminal Neurofibroma, mildly expanding the sacral foramina, as well as pelvic masses of neurofibromatosis displacing the rectum and uterus anteriorly. Incidentally, a large right ovarian cyst was also detected (). |
A 57-year-old man presented with a chief complaint of right facial swelling after traveling to South Korea. Physical exam demonstrated a palpable right parotid mass. The patient had no other symptoms and denied dysphagia, pain, swelling, or difficulty opening their jaw.\nCT scan of the neck with contrast demonstrated a 2.1 × 1.0 × 2.0 cm ovoid low-density rim-enhancing lesion in the superficial lobe of the right parotid gland ().\nFNA of the right parotid and right lymph node demonstrated benign cyst contents from the parotid, from the right lymph node, and a polymorphous lymphoid population consistent with lymph node sampling. There was no cytologic evidence of malignancy. However, a single degenerated structure suspicious for the neck, scolex, and partial strobila of the cestode was identified (). The slides were sent to the Centers of Disease Control (CDC), and it was confirmed to be a cestode. Further classification of the cestode was not possible due to the degenerated nature of the organism. Given these findings, the patient was prescribed two doses of praziquantel 600 mg over a 10-day period for a total of 10 mg/kg and advised to follow-up in 3 months.\nThe patient returned and noted some residual right facial swelling. The mass was nontender, and the patient denied dysphagia or pain with chewing. Of note, the patient had not traveled and had no further infectious exposures. An ultrasound was performed and revealed a residual 1.9 × 0.9 × 0.8 cm hypoechoic lesion within the right parotid gland (). A repeat FNA was performed and yielded another 2 cc of clear fluid with only secretory debris without evidence of a cestode. At this time, further workup was offered including potential surgical excision, but the patient wished to defer this due to continued travel plans.\nThe patient returned 6 months following the initial diagnosis, and his swelling and symptoms were much improved. At the time of publication, he continues to do well without any further treatment. |
We present the case of a 56-year-old female with a past medical history pertinent for depression, cirrhosis, chronic pancreatitis, tobacco abuse, and alcohol abuse. She presented to the emergency department (ED) for shortness of breath, general malaise, and dyspnea on exertion after a mechanical fall while ambulating and subsequent motor vehicle collision earlier in the day. The patient also endorsed several months of progressive loss of appetite and increasing nausea with vomiting over several days prior to evaluation. Alcohol use was significant for consuming about six beers plus wine or liquor daily. The examination findings were significant for profound abdominal distention and palpation of a firm mass in the left upper quadrant. CT of the abdomen and pelvis revealed a “large developing fluid collection in the left upper quadrant of the abdomen estimated up to 22 cm maximal dimension with regional mass effect.” With a history of chronic pancreatitis and atrophy of the pancreas with calcifications present on the imaging obtained, a pancreatic pseudocyst was the most likely differential (Figures -).\nInterventional gastroenterology was appropriately consulted with recommendations to correct electrolyte derangements, notable for hyponatremia to 125, prior to endoscopic intervention. Following medical optimization for the presenting condition, the patient underwent a successful endoscopic ultrasound-guided pancreatic pseudocyst cystogastrostomy. Procedural endosonography revealed a 21 × 12 cm in maximal cross-sectional diameter large, anechoic lesion consistent with suspected pseudocyst in the peripancreatic region. Intervention by the consulted specialists resulted in a cystogastrostomy with stent placement and subsequent drainage of 4.5 L of dark, debris-laden fluid with additional drainage into the gastric lumen (Figures -). The stent was exchanged for double pigtail catheters connecting the pseudocyst to the stomach prior to the patient’s discharge (Figure ).\nThe patient was seen once again approximately three months after her initial discharge. Imaging at that time showed that there was no evidence of re-accumulation in the pseudocyst (Figures -). |
A 73-year-old man presented to the emergency department with right leg swelling and an acute kidney injury. CT scans revealed a 6.2 mm unresectable right pelvic mass compressing the external iliac vein and ureters, a right acetabular metastasis, and external iliac vein thrombosis. Subsequent bladder biopsy led to the diagnosis of locally advanced transitional cell carcinoma. Malignant cells were positive for CK7, GATA3, and p40 with patchy positive staining for CK20. The patient had a background of benign prostatic hypertrophy, stage three chronic kidney disease, and melanoma of the right shin which had been excised several months prior. During a three-month period, he was treated with three cycles of gemcitabine on days one and eight in a 21-day cycle. Due to the lack of tumour response and multiple side effects, this was ceased, and one month later, he was commenced on Pembrolizumab. He received three 200 mg doses over a nine-week period. Three days prior to the final dose, he developed acute on chronic lumbar back pain with no inciting trauma. Over the following week, he developed an asymmetrical grade 3 paraesthesia of the upper and lower limbs, though retained brisk reflexes []. He also developed hypoesthesia and hypoalgesia in a banded distribution extending from the T10 to L1 dermatomes. Pembrolizumab was discontinued, and neurooncological investigations commenced. A Naranjo score of 7 was calculated, indicating a probable adverse drug reaction.\nBrain and spine magnetic resonance imaging (MRI) demonstrated no evidence of haemorrhagic, ischaemic, or metastatic events and no cord compression. An inversion sequence could not be administered due to significant renal impairment. Fat saturation sequencing demonstrated no evidence of radiculopathy. Nerve conduction studies demonstrated bilaterally reduced lower limb motor units with retained sensory responses and reduced F-waves in the upper limbs. F-waves compare the conduction in the proximal half of the nerve pathway to the distal and can be used to distinguish a root lesion from a distal neuropathy []. Nerve conduction findings assisted in the exclusion of axonal loss and demyelinating syndromes such as acute inflammatory demyelinating polyneuropathy. Cerebrospinal fluid (CSF) was clear with an elevated protein level (2100 mg/L, normal 150-500 mg/L) and raised white blood cell count (35 × 10 [6]/L). There were no malignant cells or organisms, and the glucose level was normal (3.2 mmol/L). Multiple kappa and lambda IgG bands were present in trace amounts. Additional laboratory tests for anti-acetylcholine receptor antibodies, anti-ganglioside antibodies, and an infectious screen were negative. Respiratory function tests were also performed and normal.\nTreatment included a course of intravenous immunoglobulin (IVIG) commenced at 30 g then 25 g daily for five days. The patient had been receiving 2 mg daily of dexamethasone for treatment of cancer symptoms, and this was left unchanged. Mild improvement in symptoms occurred one week following IVIG, and the patient was then transferred to a Rehabilitation Unit. After five weeks of multidisciplinary team input, he was discharged able to walk with a single point stick. A restaging CT scan was performed which demonstrated progression of acetabular cortical bone destruction adjacent to the malignancy compared to four months earlier, but no new metastatic disease or lymphadenopathy. No further immune treatment was offered for the primary malignancy. |
A 57-year-old man with cough and minimal whitish expectoration was diagnosed with adenocarcinoma after a biopsy was conducted on the upper lobe mass of his left lung on March 4, 2016. Mutational analysis revealed that he lacked EGFR mutations or ALK gene rearrangements. CT scans showed that this patient had developed multiple metastases, including mediastinal and hilar lymph nodes, and also to the right intrapulmonary lymph nodes. He received 6 cycles of Pem-Cis chemotherapy, followed by thoracic radiation. Review of the CT scan at completion of sequential chemoradiotherapy treatment resulted in a significant shrinkage in the primary tumor in his left lung, while simultaneously resulting in a slight increase in metastatic lymph nodes (Fig. ). In February 6, 2017, CT scans showed that the tumor progressed rapidly. He was then scheduled to receive gemcitabine plus nedaplatin (Gem-Ndp) chemotherapy. However, the patient had to discontinue the planned 2nd cycle of this regimen as he developed moderate pneumonia. After systemic antibiotic treatment, the patient improved symptomatically and became increasingly energetic. However, CT scans on March 29 revealed that his tumors had progressed further. Owing to his poor physical condition after Gem-Ndp treatment, the chemotherapy regimen was changed to Pem-Cis. However, CT scans on May 11 failed to detect any shrinkage in his tumor. In addition, lymph node metastases increased post-treatment. The patient subsequently started standard of care pembrolizumab treatment at 2 mg/kg intravenously every 3 weeks for 6 cycles. During hospitalization, the general condition of the patient was good, with signs of fatigue only present at the first 2 days after each pembrolizumab infusion. The treatment failed to result in an anti-tumor response. The patient then received 2 cycles of initially used chemotherapy regimen Pem-Cis. Interestingly, we detected a significant shrinkage in the enlarged mediastinal and hilar lymph node metastases, with the primary site in the left lung exhibiting no further progression.\nRecent studies have suggested that clinical responses are associated with several potential biomarkers, including PD-1, PD-L1, and CD3.[ To investigate the clinical association of these factors with the efficacy of PD-1 blockade, we performed immunohistochemistry analysis of PD-1, PD-L1, and CD3 in specimens obtained from this patient. The results showed that the tumors from exhibited CD3+ T cell infiltration, but no PD-1 or PD-L1 expression (Fig. ). |
A 68-year-old woman consulted because of a left vulvar lesion. As a pathological antecedent of interest, she had presented multifocal motor neuropathy in the left upper limb that was treated with rituximab.\nDuring the gynaecological examination, a 2.5-cm irregular pigmented lesion was observed in the upper third of the left lower lip (Fig. ) with a raised and rough acetowhite lesion inside of it (Fig. ). There were no palpable inguinal nodes.\nA vulvar biopsy was performed using a punch under local anaesthesia. The biopsy demonstrated a superficial spreading malignant melanoma. The Breslow index was 0.72 mm and Clark's level IV. The PET-CT examination did not show any metastatic lesion. Surgery consisted of vulvectomy with selective sentinel node biopsy technique. Surgery was carried out 5 days later under general anaesthesia with previous technetium 99m (Tc99m) injection. The resection limits were defined, as shown in Figure . Intraoperatively, permanent bladder catheterization was performed, and methylene blue was injected at the level of the lesion. A bilateral inguinal incision was made, but sentinel lymph nodes could not be identified because of the absence of Tc 99m or methylene blue, so inguinal lymphadenectomy was performed. Wide excision of the melanocytic lesion was performed, including the clitoral hood, the left lower vulvar lip, and a part of the left greater vulvar lip. The clitoris was subsequently exteriorized at the level of the upper pole. The resection of the mass and repair of the incision are shown in Figure . Subsequently, the left vulvar lip defect was covered with a left gluteal flap (Fig. ). The histopathologic study confirmed the diagnosis of vulvar melanoma of superficial extension with a Breslow of 0.89 mm and Clark level IV with ulceration (Fig. ). The resection margins were reported to be free of lesions. Six left inguinal lymph nodes and 5 right inguinal lymph nodes were obtained, all of which were negative for melanoma infiltration.\nThe postoperative evolution was satisfactory. No adjuvant therapy was indicated. |
We report the case of a 42-year-old woman who presented in our clinic due to recent diagnosis of early left breast cancer. The computer tomography performed revealed no other organs compromised or distant metastatic extension. The patient did not have relevant medical history of interest, except for a Darier's disease known for many years.\nThe patient underwent a lumpectomy with axillary lymph node dissection. The pathological diagnosis was moderately differentiated invasive ductal carcinoma (IDC) of the right breast of 2.3 cm in size with metastasis detected in 1/18 lymph nodes removed (pT2 pN1 M0). Immunohistochemical study of the tumor cells showed positive staining for both estrogen receptors (ER) and progesterone receptors (PR) in 95% and 95%, respectively, and showed positive membrane staining of HER-2 marker. With this diagnosis, the patient received postoperative adjuvant chemotherapy consisting of 6 cycles of FEC followed by maintaining treatment with trastuzumab for 1 year and adjuvant hormone therapy with tamoxifen 20 mg daily during the following 5 years. Then, the patient was referred to our radiation oncology department where she received adjuvant radiotherapy (50 Gy) on left-breast residual tissue.\nShe was primarily diagnosed of DD in 1991 with pruritic eruption on the trunk. There was no family history of a similar skin summer. A biopsy specimen revealed acantholytic dyskeratotic cells in the upper epidermis, suprabasal clefts with focal hyperkeratosis, and parakeratosis which were consistent with the clinical diagnosis of Darier's disease. Various therapeutic options had been applied: lotions containing cortisone, estrogens, or testosterone and several homeopathic options. Due to progression disease, the patient was treated with oral retinoids (acitretin: initial 10 mg) plus cyclosporine, obtaining an initial good response with disappearance of the skin lesions. After nine months using systemic retinoid and cyclosporine, the papules have reappeared in almost the whole body. This systemic therapy is still ongoing, but the patient occasionally required the topical corticosteroids during acute flares.\nBefore the radiotherapy, the patient showed hyperkeratotic, brownish papules extending in a linear pattern from the neck to the abdomen, especially on both breasts and inframammary area (). During the radiation, she developed grade 1 to 2 dermatitis in the irradiated area ().\nOne month after the end of radiotherapy, the patient showed a good remission of the skin lesions in the irradiated zone (). At a followup of 6 months, the patient has no skin lesions in the irradiated zone (). |
A 55-year-old Japanese woman was referred to our hospital because of hepatic tumors detected by abdominal ultrasonography during a screening examination. Her medical and family history was unremarkable; her occupational and residential history showed no apparent exposure to asbestos. She received no medical drugs, and neither smoked tobacco nor drank alcohol. On admission, her general status was unremarkable; her temperature was 36.2 °C and her blood pressure was 110/70 mmHg with a regular heart rate of 80/minute. A physical examination revealed no abnormal findings; neurological examinations were unremarkable. All laboratory data, including levels of the serum tumor markers carcinoembryonic antigen, cancer antigen 19-9, alpha fetoprotein, protein induced by vitamin K absence or antagonist-II, and cancer antigen 125 were within normal ranges (Table ). An abdominal enhanced CT scan revealed four hypervascular, round hepatic tumors. One tumor, which was 32 mm in diameter, was in contact with the lateral liver segment and the hepatic hilus; the other three tumors were in contact with the diaphragm and measured 7 mm in segment 4, 17 mm in segment 7, and 15 mm in segment 8 (Fig. a–d). There was no pleural effusion or ascites, and no other nodular lesions in her chest or abdomen were observed. Similar to the CT findings, magnetic resonance imaging (MRI) revealed a tumor of lower intensity than the liver on T1-weighted images, and with higher intensity than the liver on T2-weighted and diffusion-weighted images (Fig. a–c). Upper and lower gastrointestinal endoscopic examination findings were normal. We hypothesized that the tumor arose from her liver, and diagnosed our patient as having multiple hepatic hemangiomas, at first. However, imaging findings were not typical of hepatic tumor: all tumors were on the edge of the liver and the tumor margins were clear. Moreover, the tumor in segment 7 appeared continuous with the diaphragm with similar high-density contrast medium (Fig. d). We then performed CT angiography to obtain further details.\nCT findings during arterial portography showed enhancement in none of the tumors (Fig. a and b), but CT during hepatic arteriography revealed strong enhancement in the tumor in the lateral segment and that this tumor was fed by the left gastric artery. In contrast, the remaining three tumors had no enhancement and were fed by the right inferior phrenic artery (Fig. c and d). Imaging findings indicated that the liver was not the origin of these tumors. Moreover, all tumors exhibited increased uptake of 18F-fluorodeoxyglucose, with a standardized uptake value of 7.8 with positron emission tomography (PET)-CT (Fig. ). We considered these tumors to be peritoneal malignant tumors, preoperatively, and performed surgical resection. Laparotomy revealed that the tumor in the lateral segment was clearly in contact with the hepatoduodenal ligament, and that the other three tumors were on the diaphragm (Fig. a–c). We performed tumorectomy with left liver lobectomy (Fig. ) and partial diaphragmatic resection. Histopathological and immunohistochemical examinations of the resected specimens were positive for CD34, CD31, D2-40, and calretinin, which confirmed epithelioid mesothelioma (Fig. a–h). Our patient was not given any medication except a general drip infusion and prophylactic antibiotics; she was discharged 10 days after surgery without complications. CT and PET-CT revealed no recurrence 20 months after surgery without adjuvant therapy. |
Patient no. 1
A 24-year-old man with a history of posterior fossa tumor requiring VP shunting presented with worsening headaches, confusion and weight loss over several months. He had a history of vague pain along the shunt site, chest and abdomen as well as swallowing difficulties over several weeks. The patient had a history of multiple shunt revisions, the most recent of which approximately 10 months prior to our evaluation when he had undergone a proximal shunt revision for an obstructed ventricular catheter.
Patient Course
A CT of the head and abdomen revealed ventricles stable in size and a pseudocyst around his distal catheter (Figure ).
He was immediately taken to the operating room for complete shunt externalization and placement of an external ventricular drain (EVD). He was started on broad-spectrum antibiotics (vancomycin, cefepime and metronidazole) and underwent percutaneous drainage of the pseudocyst in his abdomen. Cultures from both the percutaneous drainage and CSF were positive for acid-fast bacilli (AFB) and presumed NTM. His antibiotics were switched to azithromycin, amikacin and cefoxitin by postadmission day 4. He continued to decompensate requiring multiple EVD replacements due to loculated ventriculitis. AFB was identified as Mycobacterium abscesses. Intrathecal (IT) amikacin was started on postadmission day 9. This was delayed due to the inability to clamp the EVD and safely administer IT amikacin. During the course of treatment, he had significant QTc interval prolongation which necessitated holding IV azithromycin and starting linezolid with readministration of azithromycin via nasogastric tube to minimize the risk of QTc prolongation by avoiding high peaks. He remained critically ill on a ventilator and required 12 EVD replacements in attempts to clear his loculated hydrocephalus. A brain MRI with contrast showed severe ventriculitis and cerebral inflammation (Figure ).
On postadmission day 30, he was transitioned to comfort care. He passed away on postadmission day 36.
Patient no. 2
A 71- |
A 73-year-old male patient presented to the surgical ward with a general malaise and right-sided abdominal pain that had presented for 2 days. His past medical history included left nephrectomy due to nephrolithiasis at the age of 48, CKD of unknown etiology, and right hemicolectomy with adjuvant chemotherapy due to colonic adenocarcinoma 5 years prior to the current hospitalization. The physical examination at the admission to our hospital revealed no signs of peritonitis. Goldflam sign was absent. The abdominal ultrasound revealed no pathology. A noncontrast abdominal CT scan was performed, and a suspicion of either inflammatory infiltration or surgical scarring in the right middle abdomen was indicated by the radiologists. Laboratory results showed signs of AKI, with plasma creatinine 390.6 μmol/L on day 1 rising quickly to 647.6 μmol/L on the second day of hospitalization, metabolic acidosis, hyponatremia (sodium 133.7 mmol/L on day 2), and hyperkalemia (potassium 5.49 mmol/L on day 1 and 5.77 mmol/L on day 2). CRP started to increase on day 2, reaching 52.4 mg/L on the following day. The empirical antibiotic therapy consisting of ciprofloxacin and metronidazole was administered after both blood and urine samples were taken for bacteria culture test.\nShortly after being admitted to our ward, the patient developed anuria, despite intravenous infusions of fluids and loop diuretics initiated at the hospital emergency department and continued at our ward. The kidney function impairment reflected by the raising plasma creatinine levels in subsequent days was worsening rapidly. Considering deteriorating patient's general condition and the laboratory findings, an acute daily hemodialysis treatment was started, and a decision to transfer the patient to the nephrology ward in our hospital was made.\nIn the nephrology ward, the pharmacotherapy was maintained with no significant modifications, and the patient's condition temporarily improved. Over the course of 3 days, serum creatinine decreased from 827.5 μmol/L to 403.5 μmol, and diuresis increased to up to 4 L per day. Serum electrolytes were within the normal range. Hemodialysis was deemed no longer necessary.\nAfter 7 days of hospitalization, the patient complained of a sudden exacerbation of the abdominal pain. The pain was located in the right middle abdomen and radiated to the right iliac fossa and was accompanied by a positive Goldflam sign. At the same time, urine output started to decrease rapidly, and macroscopic hematuria had been observed before the patient became anuric for the second time. Serum creatinine increased quickly to 801.6 μmol/L. The changes of serum creatinine and urine output are presented in Figure . Both abdominal ultrasonography and plain abdominal X-ray showed no abnormalities aside from a slight pelvicalyceal system dilation. A second contrast-enhanced CT scan was performed, this time with urography phase, revealing the extravasation of the contrast medium in the proximity of the right renal pelvis (Fig. ) and alongside the right ureter (Fig. ), suggesting the rupture of the renal pelvis.\nThe patient had 1 acute hemodialysis session performed and then was transported to the urology ward. A cystoscopy was performed, during which a double-J ureteral stent was placed into the right ureter. After the procedure symptoms subsided, diuresis reached 5 L per day and plasma creatinine decreased to 296.2 μmol/L. The patient was discharged in a good general condition, with frequent checkups at a local nephrology clinic recommended. |
A 47-year-old right-handed Japanese man was admitted to Keio University Hospital with acute onset of sensory aphasia.\nFourteen days before his admission the patient began to have difficulties in operating computers and document processing software. The symptoms worsened over the next 10 days, without headache, fever, or seizure. Three days before his admission, at evening, he suddenly became incoherent and agitated in association with impaired auditory comprehension. He underwent a brain MRI at another hospital; he was suspected of having a herpes simplex encephalitis (HSE), and then he was referred and admitted to the department of neurology at our hospital for further evaluation and treatment. Prior to admission he had no hypoglycemic episodes, preceding viral infection, recent medication changes, or other precipitating events leading to seizure.\nHe had a past medical history of type-2 diabetes since the age of 41 years, for which he had been treated with acarbose, but he had no other history including cardiomyopathy, atrial fibrillation, migraine, sensorineural hearing loss, seizures, or psychiatric illness. His growth and development were normal. His mother suffered from type-1 diabetes and deafness, which had begun in her 40s, and suddenly died of unknown cause at the age of 68 years. He had no habit of smoking, drinking, or the use of illicit drugs.\nOn admission (day 1), the temperature was 36.6 °C, the blood pressure 110/65 mmHg, the pulse 84 beat per minute, and the oxygen saturation 97% while he was breathing ambient air. The height was 154 cm, and the weight was 42 kg; the body mass index was 17.7, but physical examination was otherwise unremarkable. On neurologic examination the patient was awake but agitated and uncooperative. He was able to speak fluently but he had paraphasia and preservation; naming, repetition, and auditory and reading comprehension were severely impaired, implying sensory predominant aphasia. Motor and sensory examination was grossly intact. The neck was supple.\nAll results of the blood tests on admission were unremarkable, including serum CK level (71 U/L), except an elevated level of HbA1c (7.3%). Cerebrospinal fluid (CSF) examination revealed 1 white blood cell/μL, and a protein level of 46 mg/dl. Oligoclonal bands were negative. A polymerase chain reaction testing was negative for herpes simplex virus. CSF levels of pyruvic and lactic acid were not examined. A brain MRI obtained on admission showed increased diffusion-weighted imaging (DWI)/fluid-attenuated inversion recovery (FLAIR) signal in the left anterolateral temporal lobe mainly affecting the cerebral cortex with underlying subcortical edema (Fig. , arrows). Apparent diffusion coefficient (ADC) maps showed mild reduction in ADC values in the left anterior temporal cortex (Fig. , arrowheads) suggesting cytotoxic edema, but increase in ADC values in the underlying subcortical white matter suggested vasogenic edema. A MRA did not show vasospasm or vascular occlusion. An electroencephalogram (EEG) recorded on day 4 showed sporadic sharp waves at the left fronto-parietal region.\nAfter admission the patient was empirically treated with intravenous administration of acyclovir and oral levetiracetam (1000 mg/day) for possible HSE. He underwent a whole-body CT and a random skin biopsy but the results were both unremarkable. For possible autoimmune encephalitis, he was also treated with intravenous high-dose methylprednisolone (IVMP: 1000 mg/day, 3 days) on day 10; however, the follow-up brain MRIs obtained on day 7 and day 14 showed a continuous spread of the lesion posteriorly along the cerebral cortex. The follow-up EEG recorded on day 27 showed focal periodic epileptiform discharges (FPEDs) at the left temporal region. The dosage of levetiracetam was increased to 3000 mg/day. However, the brain MRI obtained on day 28 showed a newly appearing lesion in the right anterior temporal lobe (Fig. ). Because multifocal cortico-subcortical MRI lesions are characteristic features of anti-γ-aminobutyric acid A receptors (GABAaR) encephalitis [], the patient was also treated with a total of 5 cycles of plasma exchange (PLEX) between day 33 and day 42, without obvious beneficial effects.\nIn the evening on day 43 the patient began to complain of feeling dizzy. Two days later dysarthria and cerebellar ataxia became clear. The brain MRI obtained on day 45 showed extensive increased DWI/FLAIR signals in the cerebellum mainly along the folia, without apparent reduction in ADC values (Fig. b). A few days later, the patient became mute and somnolent. An EEG revealed FPEDs mainly at the left frontal region. On day 48 CSF lactate and pyruvate levels were 10.2 mmol/L and 0.33 mmol/L, respectively; CSF lactate/pyruvate (L/P) ratios were markedly elevated (30.9). The follow-up brain MRI obtained on day 52 showed expansion of cerebellar lesions, which predominantly affected the folia with edema. Tonsil and nodule were involved, but the deep cerebellar nucleus or cerebellar peduncle were not involved (Fig. d).\nBased on possible maternal inheritance of diabetes, short stature, elevated CSF L/P ratios, cerebral SLLs, and the lack of evidence of cardiogenic embolism, the patient underwent a genomic analysis, which demonstrated a m.3243A > G mutation with 17% heteroplasmy in leukocytes, confirming the diagnosis of MELAS.\nAfter the treatment with taurine (12,000 mg/day), L-arginine (12,000 mg/day), vitamin B1 (100 mg/day), and carnitine (3000 mg/day), the patient became able to follow simple commands, and he was transferred to a rehabilitation center on day 146. These drugs were well tolerated and there were no adverse events. The brain MRI obtained 4 months after admission showed diffuse brain atrophy including the cerebellum. The modified Rankin Scale at the last follow-up (9 months after admission) was scored 4, with residual cognitive deficits. Nerve conduction study, needle electromyography, and audiometry were not performed during the course of the disease because no consent was obtained for these studies. |
A 24 year old female reported to the Department of Orthodontics and Dentofacial Orthopaedics for the management of malaligned teeth in upper and lower jaw. She had normal intelligence and was tall. Family history revealed that her father had similar Class III malocclusion, but no other symptoms were observed. A detailed medical and dental history was obtained from the patient and from her parents.\nAfter a normal pregnancy, she was born with a birth weight of 2500 g. She was having episodes of cyanosis while crying i.e. a bluish tint of skin, lips and finger nails; and was diagnosed to have ventricular septal defect (VSD) by echo-cardiography. A VSD is a defect in the septum between the heart's two lower chambers, the ventricles. This defect allows oxygen-rich blood from left ventricle to mix with oxygen-poor blood from right ventricle. The problem persisted until the defect was repaired. VSD was repaired with open-heart surgery at the age of two years.\nPatient had also reported an history of umbilical hernia at birth. A hernia at birth will push the belly button out. It showed more when she used to cry because the pressure from crying makes the hernia bulge out more. To handle the situation, the parents used to apply pressure by adhesive tapes over the belly button as prescribed by the paediatrician. The umbilical hernia shrunk and closed by itself in around 22 months of age.\nThe patient had congenital cataract and was operated for both eyes at four and at six years of age by an ophthalmologist []. There was no family history of cataract, birth defects, genetic disease or cardiac anomalies. She also had syndactly of second and third toes. The fingers were normal. [Figure and ].\nParents mentioned delayed eruption of the primary dentition, initiated at the age of 2 year, 6 months. Severe dental and skeletal abnormalities were found on detailed clinical examination.\nExtra-oral examination presented a long and narrow face with concave profile. The eyebrows were laterally curved and thick []. Intraoral examination revealed a Class III malocclusion with a negative over jet and deep overbite. Dental and chronological age of the patient was not coinciding as multiple retained deciduous teeth, namely maxillary right lateral incisor, first and second molars; left lateral incisor and first molar along with root stumps of both right and left canine were retained. In the mandibular arch, right central incisor, lateral incisor, canine and left canine and second molar teeth were retained. Both the maxillary right premolars and mandibular left second premolar were absent on clinical examination [].\nSevere dental and skeletal abnormalities were found on detailed radiographic examination. Lateral cephalometric radiograph showed skeletal Class III and high angle pattern [] Posterio-anterior radiograph revealed a skeletal midline shift to the right []. The panoramic radiograph showed permanent teeth with extremely long roots and open apices. The roots of maxillary canines were in relation with the inferior border of the orbits and the lower canine roots almost reached the lower border of the mandible. Permanent maxillary right first premolar was missing while second premolar was impacted with distal aggregation. The maxillary left central incisor had dilacerated root; all four third molars were congenitally missing []. All the main findings were recognized in this patient, therefore the case was diagnosed as OFCD syndrome. An integrated orthodontic, endodontic and prosthodontic treatment was planned. |
A 15-year-old teenage boy initially presented in a district hospital, with a swelling at the right side of his neck, without any significant clinical symptoms. Although full details of the work-up performed at the time are not available, he was diagnosed with a branchial cleft cyst and was offered surveillance with follow-up imaging. Eight years later, he visited our unit. Physical examination revealed a painless palpable well-defined mass () within the right carotid triangle with positive Fontaine and Kocher I signs []. There was no palpable lymphadenopathy. An ultrasound scan was performed depicting a solid mass of mixed echogenicity in the right carotid triangle echogenicity in the left carotid triangle. This was suspected to be neurogenic in origin because of its location. A digital subtraction angiography (DSA) () followed, which revealed a 60x35mm protruding mass in the right carotid bifurcation, causing local compressive effects and posterior displacement of the vessels. The patient was admitted in our unit in order to be treated surgically. During the operation a large CBP was identified as seen in . The tumor was classified as Shamblin II and was completely excised. Histopathology study showed a 3 cm carotid body paraganglioma with characteristic “zellballen” growth pattern and cell nests surrounded by prominent fibrovascular stroma, with no evidence of malignancy (). The patient had an unremarkable recovery and was discharged home on the second POD.\nThe surgical technique adopted in both patients consisted of an oblique incision along the sternal head of the left sternocleidomastoid muscle under general anesthesia. Our strategy was to expose, dissect, and isolate the proximal common carotid artery using a vascular tape. The ansa cervicalis was also exposed early in the operation. The carotid bifurcation was exposed in a caudocranial approach. The proximal external and the proximal internal carotid arteries were isolated and controlled with vessel loops. Dissection was extended to the level of the digastor muscle in order to expose and control the distal internal carotid artery. During this process, the hypoglossal nerve was identified and preserved, by following the ansa cervicalis. The external carotid artery was cross-clamped temporarily. Finally, the tumor was removed with sharp dissection from the bifurcation with meticulous technique in order to avoid injury to the internal carotid artery and the cranial nerves. The reported plane of dissection reported as a white interface plane between the tumor and the vessels was not identified in the first patient as the tumor was severely adhering to the vessel wall and was typically found in the second patient. Following the removal of the CBP, the carotid sheath and platysma were approximated and the skin closed with a continuous subcuticular suture, after performing meticulous hemostasis (Figures and ).\nBoth patients had an annual postoperative follow-up with cervical ultrasound and carotid duplex ultrasonography with no evidence of local recurrence. Familial disease was excluded clinically, by screening the patient's first degree relatives with ultrasound imaging. |
A 66-year-old African American female with a past medical history of refractory immunoglobulin G (IgG) lambda MM, essential hypertension, and chronic kidney disease presented to the emergency department with five days of right upper quadrant pain.\nHer MM was diagnosed one year prior when she presented with altered mental status, uremia, hypercalcemia, hypoalbuminemia, and paraproteinemia. A skeletal survey at that time revealed multiple thoracic spinal lytic lesions and an eroding soft tissue mass at the level of T10. Further evaluation revealed a very high IgG level, elevated M protein band, and a kappa/lambda ratio <0.01 (normal 0.26-1.65). A biopsy from the soft tissue mass revealed a plasmacytoma. Radiation therapy was initiated for 10 days. She received three cycles of bortezomib and dexamethasone followed by two cycles of bortezomib, dexamethasone, and lenalidomide. Her disease progressed, and a subsequent bone marrow biopsy revealed hypercellular bone marrow with 70% atypical plasma cells. The patient subsequently received seven cycles of carfilzomib, lenalidomide, and dexamethasone. She was not a candidate for bone marrow transplantation given the high plasma cell burden.\nOn her current presentation, the pain was sudden in onset, intermittent, worse with eating, and without radiation. The pain was associated with nausea and anorexia, but she was without any change in bowel habits. She denied any previous similar episodes. Upon physical exam, the patient was in distress but remained alert and oriented. Her vital signs were all stable. She exhibited right upper quadrant abdominal tenderness without rebound or guarding. Her initial labs are presented in Table . The patient was admitted to the hospital for further evaluation of her abnormal labs and supportive treatment.\nThe patient was started on intravenous hydration and was made nil per os. An abdominal ultrasound revealed a distended GB with sludge (Figure ). The GB wall was thickened up to 9.5 mm, and the sonographic Murphy sign was positive. The common bile duct and common hepatic duct measured 6.3 mm and 3 mm, respectively. The liver measured 18.3 cm and exhibited normal echogenicity. There were no intraparenchymal masses or fluid collections. The portal and hepatic veins were patent.\nThe patient was diagnosed with acute cholecystitis. Intravenous piperacillin-tazobactam 3.37 g every eight hours was initiated, and the patient was referred for open cholecystectomy given her overall condition and lactic acidosis. Intra-operatively, the GB was thickened and firm but not overly distended or perforated. The GB was dissected from the liver edge, and a liver biopsy was performed successfully.\nThe pathology report from the cholecystectomy revealed chronic cholecystitis with involvement of the GB submucosa and serosa by abnormal plasma cells with lambda light chain restriction (Figure ). Subsequently, Congo red stain of the GB sections revealed apple-green birefringence throughout the submucosal areas consistent with amyloid deposits. The liver biopsy exhibited abnormal plasma cells in periportal locations with lambda light chain restriction as well; Congo red stain was not done on the liver sample.\nThe patient declined any further treatment for MM and decided to proceed with hospice care. She was discharged home with comfort measures. |
A 19-year-old male presented in the emergency department with one episode of melena per day, for one week. It was associated with vomiting, shortness of breath and palpitations. His hemoglobin level on initial complete blood count was 5.80 g/dL, signifying severe anemia according to WHO guidelines []. His lab parameters on admission are presented in Table .\nImmediately packed red blood cells (RBCs) were requested from the blood bank. On forward typing his blood group was labeled as O positive and his serum showed strongly positive indirect Coomb’s test with a negative direct Coomb’s. On extended 11 cell panel antibody testing, his serum demonstrated pan-agglutination which matched with monoclonal panel cells having anti-Kell, anti-Lub, and anti-Kpb antibodies. On cross match with four O negative and four O positive packed RBCs, +4 incompatibility was seen with all. Meanwhile a detailed history of the patient revealed two distinct episodes of epistaxis in childhood and a family history of his paternal grandmother having an increased bleeding tendency. In view of his past history of fresh frozen plasma infusions, it was interpreted that the patient may have multiple alloantibodies in blood leading to gross incompatibility. Considering the urgency of the situation, one unit of the least incompatible (O negative) packed RBCs was issued after washing with normal saline thrice, to the emergency department. Transfusion was started under strict monitoring by the emergency department physicians. After slow transfusion of around 10 ml blood, the patient started shivering and his temperature spiked to 101°F with tachycardia and hypotension. The transfusion was stopped immediately and the patient was given intravenous antihistamine and hydrocortisone. Meanwhile, he was transferred to the intensive care unit (ICU) where he received intranasal desmopressin and intravenous factor VIII.\nTransfusion reaction workup revealed a grade 4+ pan agglutination in his serum. During repeat blood grouping, forward typing did not demonstrate any reaction to anti-A and anti-B antisera, like a normal O blood group. However, on reverse typing, his serum showed strong agglutination with group O pooled control cells. His post saline wash incompatibility with O negative red cell concentrate showed minor difference from grade +4 agglutination (pre-wash) to grade +3 clumping (post-wash). A fresh RBCs sample from the patient showed negative direct Coomb’s test, while fresh serum sample remained positive for indirect Coomb’s test. This workup strongly raised the suspicion of Bombay phenotype and his red cells were tested with anti-H lectin, which showed no agglutination. This confirmed his blood group as Bombay phenotype. The reactions observed with Bombay phenotype compared to other blood groups, on forward and reverse typing, are illustrated in Table .\nImmediately, voluntary donor pools were contacted in blood banks throughout the country. Overnight, a donor with Bombay negative blood group was arranged from Karachi. The packed RBCs were airlifted to Islamabad maintaining the cold chain. After crossmatching with recipient’s blood showed no reaction, the donor blood was transfused to the patient. Meanwhile, a distant relative of the patient from a nearby city, with Bombay positive blood group, consented to donate blood at our blood bank. Two days later, another unit of packed RBCs was transfused to the patient. His hemoglobin after two transfusions rose up to 7.40 g/dL. As his melena settled down on supportive therapy, an endoscopy was performed that suggested an underlying hiatal hernia. After surgical consultation, the patient was advised to reduce weight and discharged from the hospital, with a scheduled follow-up visit.\nIn view of the patient’s past medical history and family history, during the follow-up visit, a von Willebrand factor antigen, von Willebrand factor functional activity and factor VIII levels were ordered. His von Willebrand factor antigen level was <2.0%, von Willebrand factor functional activity was <4.0% and factor VIII level was 18.5%, consistent with type 3 von Willebrand disease. The patient and his family were counselled accordingly and referred to the hematology clinic. |
A 29-year-old man was referred from another urologic clinic for severe perineal pain with a weak urine stream and dysuria. When he visited Kyungpook National University Medical Center, he reported a history of acute urinary retention and had a Foley catheter in place for about 1 month. A digital rectal examination (DRE) showed a relatively enlarged prostate with tenderness on pressure. An abdominal computed tomography (CT) scan revealed an 8×7×4.5-cm-sized moderately enlarged prostate. The estimated volume of the prostate by transrectal ultrasound (TRUS) and the prostate-specific antigen (PSA) level were 80 mL and 8.0 ng/mL, respectively. Considering the relatively young age of the patient, acute bacterial prostatitis was suspected, but the patient's clinical symptoms did not exactly line up with acute bacterial prostatitis. To relieve the patient's symptoms and drain the urine, we performed a suprapubic cystostomy and started the patient on intravenous antibiotics, but his symptoms were not relieved. Repeated TRUS and DRE suggested a prostate cold abscess, but prostate malignancy could not be ruled out. Thus, we performed prostate magnetic resonance imaging (MRI).\nThe prostate MRI showed a huge prostatic mass that obstructed the bladder neck (). According to the results of the MRI, we performed transurethral resection of the prostate (TURP) to relieve the bladder outlet obstruction and to confirm the pathologic nature of the mass. The pathology report from TURP indicated high-grade sarcoma with extensive necrosis. We performed chest CT and a whole-body bone scan to evaluate the extensiveness of the disease to determine the feasibility of surgery. No metastatic lesions were found on the chest CT or whole-body bone scan. In the prostate MRI, the whole prostate was shown to be involved by the malignant lesion and rectal wall invasion was suspected. Considering the particularly young age of the patient and the lack of evidence of bladder invasion, we decided to perform RALP and extended pelvic lymph node dissection, with an expectation of sparing the bladder. This decision was made with the informed consent of the patient and the patient's legal guardian. During the operation, rectal wall invasion of sarcoma was noted. Accordingly, the anterior wall of the rectum was resected and repaired. Gross examination of the radical prostatectomy specimen showed a 12×8.0×7.0-cm-sized relatively well-demarcated mass containing hemorrhage. The final histopathologic analysis revealed PSS with invasion to both seminal vesicles. The rectal mucosa and prostatic resection margins were positive for tumor, but none of the lymph nodes had tumor present (). After 1 month of follow-up, the PSS had recurred and metastasized to the lung and liver. The patient's symptoms and the recurred mass in the pelvis became aggravated at 45 days after the operation. The patient was transferred to another hospital for hospice care. |
A 38-year-old Iranian man, residing in a rural area, was admitted to our surgery unit with a history of back pain, chest pain, paraplegia and urinary incontinence within the last 45 days. The patient had a history of hydatid cyst(s) in the liver, lungs, and chest wall. The first surgery occurred 26 years previously when he was 12 years old and underwent thoracotomy for two hydatid cysts in the left lung. Subsequently, in April 1999, the patient was diagnosed with a hydatid cyst in his left lung and underwent surgery again. Nine years later, after confirmation of a hydatid cyst in his left lung, he underwent thoracotomy. In May 2011, computerized tomography (CT) revealed multiple cysts located behind the left lung and the fourth rib (R4), leading to the diagnosis of paraspinal hydatidosis and a second thoracotomy. In May 2014, his imaging results demonstrated the presence of four hydatid cysts in the left hemithorax. Total cystectomy was performed for one cyst under the latissimus dorsi, and two cysts behind the third rib (R3) and one cyst behind his left clavicle were drained. In the same year, one hydatid cyst was detected in his liver, and hepatic resection was performed. In March 2018, imaging results demonstrated the presence of multiple cystic lesions under R3, R3 and the fifth rib (R5). Thoracotomy was once again performed, and the cystic lesions and the necks of R3, R4, and R5 were removed. After surgery, albendazole therapy (400 mg/kg) was initiated and continued.\nIn December 2019 the patient was referred to our surgery unit with progressive weakness. Spinal magnetic resonance imaging (MRI) showed multiple spinal epidural cystic lesions at the level of the third to fourth thoracic vertebrae (T3–T4) (Figs. , ) and that the pedicles on both sides of T4, some parts of the lamina, and the vertebral body were destroyed. The patient underwent surgical resection with the costotransverse approach, and multiple epidural cystic lesions at the T3–T4 level were completely removed. Multiple extradural cystic lesions were carefully excised to avoid intraoperative rupture of the cysts. Intraoperatively, irrigation with hypertonic saline (as scolicidal agents) and cotton pads soaked with hypertonic saline were used. Since the T3 and T4 pedicles had been destroyed, posterior fusion with pedicular screw was performed. The diagnosis of hydatid cyst was confirmed by pathological examination, following which treatment with 400 mg/kg albendazole was started, with the recommendation that the treatment continue for 6 months. Within 2 weeks after surgery, his lower extremity forces dramatically returned and he was full force after 4 weeks. |
A 72-year-old Japanese woman was referred to our department She complained of a purulent discharge from a left submandibular fistula and severe pain with hypoesthesia of the left submental region. The patient’s masticatory function was significantly impaired, and she had trouble sleeping because of pain. A panoramic radiograph taken at the first visit showed a fracture of the left inferior mandibular border with minimal deviation. Computed tomography (CT) showed extensive, morphologically irregular sequestrum formation (80 × 35 × 20 mm) in the left mandibular body.\nFollowing initial diagnosis of diabetic nephropathy 13 years earlier, the patient’s disease had already advanced to the point where she needed insulin therapy. Eight years before the referral, diabetic nephropathy progressed further, acute congestive heart failure developed, and emergency dialysis was simultaneously introduced. For the last 8 years, the patient had been undergoing HD every Monday, Wednesday, and Friday morning at 9:00 am for 4 hours owing to kidney failure caused by advanced diabetic nephropathy. Thus, 5 µg of darbepoetin alfa was administered weekly immediately after dialysis to improve renal anemia and maintain hemoglobin level at 10–12 g/dL. To control mineral metabolism and elevated parathyroid hormone (PTH) levels associated with dialysis, the patient was medically managed with active vitamin D3 and sedimented calcium tablets. Then, 7 years before the referral, she underwent a two-branch percutaneous coronary intervention for exertional angina pectoris, and 5 years before the referral, the patient underwent extraction of all lower left molars for periodontitis at a dental clinic, after which the mucosa was completely covered and healed. Due to renal osteodystrophy-induced osteoporosis, she had been receiving monthly antiresorptive intravenous therapy with 1 mg ibandronate sodium hydrate for the last 3 years. Two years before the referral, the patient developed fractures of the second, third, and fourth metatarsals on the left side, which revealed that she had severe chronic kidney disease–mineral and bone disorder (CKD-MBD) [] with a young adult mean (YAM) value of 45%. Six months before the referral, the patient began experiencing pain in the left mandibular body and paralysis of the mental region, and she visited her primary care dentist for diagnosis and treatment. CT scan revealed normal findings in the left mandibular body, with no tooth or foreign substances as a source of infection, and no jawbone exposure or bone resorption. Although the cause could not be identified, continued observation revealed bone exposure, and the patient’s condition deteriorated. She was then referred to a public municipal hospital where stage III MRONJ was diagnosed, based on the AAOMS guidelines []. She was treated in the hospital with regular flushing and antibiotic therapy; however, the local infection gradually worsened, and the patient was referred to our department.\nPreoperative blood sampling showed elevated levels of C-reactive protein (CRP), suggesting a systemic as well as a local inflammatory response (Table ). The abscess was drained, and daily intravenous antibiotic treatment of 1 g ceftriaxone sodium hydrate commenced. Additionally, ibandronate sodium hydrate administration was discontinued after consulting with the doctor responsible for the patient’s dialysis given that the YAM value had recovered to 51%. A panoramic radiograph revealed a displaced pathologic fracture on the left side of the atrophic mandibular body, corresponding to stage III MRONJ (Fig. ). Although the acute inflammation disappeared with anti-inflammatory treatment, the cutaneous fistula worsened, and the deviation of the pathologic fracture of the mandible increased. Figure shows the clinical course from the onset of diabetic nephropathy leading up to the surgery for sequestrectomy. Consequently, the patient underwent decortication and fistula closure under general anesthesia 2 months after the referral. Immediately before the surgery, the anticoagulant used for dialysis was changed from heparin to nafamostat mesylate to reduce the risk of intraoperative bleeding. Because she also had cardiac disease, she was managed in the intensive care unit during the perioperative period. Intravenous injection of 1 g cefazolin sodium was administered at least 30 minutes prior to the surgery to prevent surgical-site infection. Oral hygiene practices were performed daily by an oral surgeon (KY) during the hospitalization, and cefcapene pivoxil hydrochloride hydrate was orally administered at a dose of 100 mg once daily for 6 weeks after the surgery. The antibiotic dose was reduced to one-third of the usual dose because the patient was under dialysis management. Antibiotics were administered for a relatively long period of 6 weeks until complete mucosal closure, and the route of wound infection was completely eliminated. After confirming that swallowing function was intact using the water swallowing test, the nasogastric tube was removed on the second postoperative day, and a soft dialysis diet was initiated. The sequestrectomy and fistula closure were successful, and no abnormal bleeding or postoperative infection was observed. Intraoperatively, the periosteum was left intact, and the sequestrum was dissected from the cortical surface (subperiosteal resection). The general perioperative condition of the patient was well maintained under HD management, and there were no cardiac events.\nHistopathology revealed osteonecrosis and the presence of Staphylococcus species and Gram-negative bacilli that were identified as facultative anaerobes, and there was no evidence of tumor disease. During the first 3 months after surgery, the wound was regularly cleaned, and a soft diet was followed to avoid force loading.\nSurprisingly, the 6-month follow-up CT scan and panoramic radiograph showed spontaneous bone regeneration. The fracture was replaced by new bone, bone healing in the periosteum was observed, and the patient was able to eat with a denture. Furthermore, the Semmes–Weinstein monofilament (SAKAI Medical Co., Ltd., Tokyo, Japan) examination performed 6 months after surgery revealed that the patient could identify the lowest target force (0.008 g) as a sensation and that thigmesthesia had recovered to normal, with no difference between the left and right sides of the submental region.\nBlood sampling after the surgery showed CRP within normal limits and an improvement in hematologic and biochemical values. The patient’s oral condition remained good, with no findings of infection to date (Fig. , 3 years after the referral; Additional file : Fig. S1 shows the most recent X-ray imaging findings of spontaneous bone regeneration 6 years after the referral).\nA literature search was conducted in PubMed according to the following method reported by Matsuda et al.: [] (spontaneous [Title]) AND (regeneration [MeSH Terms]) AND (mandible [MeSH Terms]) AND (“2003” to “Present” [Data-Publication]).\nEleven papers were identified by our literature search [–], including 26 patients (15 males and 11 females; mean age 23.2 ± 15.7 years). Ameloblastoma was the most commonly treated disease (12 cases), followed by ossifying fibroma (6 cases). The oldest patient, aged 70 years, was a case of MRONJ []. Immediate reconstruction was reported as performed in seven cases, most of which were segmental mandibulectomy with preservation of the periosteum to achieve spontaneous bone recovery. |
A 10-year-old boy presented with complaints of progressively increasing weakness of all four extremities for one year. Weakness was more on the left side then the right. Patient had a spastic circumducting gait on the left side. Complaints of painful and restricted neck movements both on the vertical and horizontal axis were present since one year. Child had wasting of left hand and forearm muscles and occasional symptoms of L‘Hermitte’s phenomenon. There were no complaints of sphincter disturbances. The child was having breathing difficulty for the last 20 days. There was a history of trivial trauma three months prior to the onset of complaints. The child fell from a running bullock cart but had no symptoms at the time of fall.\nOn examination the child was tachypneic with the entire accessory muscles in action. There was wasting of trapezius, sternocleidomastoid, suboccipital group of muscles on the left side along with left forearm and hand muscles wasting. Spastic quadriparesis with patellar and ankle clonus was present. Posterior column sensation was impaired and pain and temperature was lost from C2 to T4 dermatome on the left side. Radiogram of the CV junction did not reveal any abnormality on the neutral or flexion and extension views. High-resolution thin-section computed tomography of the CV junction revealed a fusion defect of atlas posterior arch in the center with a faint bony bar in the canal [Figures and ]. Magnetic resonance imaging showed a posteriorly compressing atlas stenosis with a syringomyelia extending from medulla to C5 vertebra level []. Surgical decompression was done by a posterior approach. Part of occiput up to C3 spinous process was exposed. On dissection we found that there was no fusion defect of C1 arch, rather the posterior arch was incurved inward towards the canal in an odd fashion, which was severely compressing the cord. The incurved part was soft and cartilaginous. The posterior arch was excised in the midline along with the compressing incurved element and immediately the pinched cord was released of compression and started pulsating wonderfully. Dura mater was not opened.\nA per-operative flexion – extension radiograph of the CV junction – was taken to look for any instability which was not there and therefore no instrumentation was attempted.\nPost-operatively the child did well. There was immediate improvement in his respiratory embarrassment and gradually the spasticity came down. Sutures were removed on the seventh post-operative day and the child was discharged with advice of physiotherapy. The child has improved completely on one-year follow-up. |
The proband was a 35-year-old male from Hebei province in the north of China. He is of Han ethnicity and was born to consanguineous parents. His family pedigree is shown in Fig. . The proband was normally delivered after a full-term pregnancy, and birth weight and length were within normal ranges. The initial signs and symptoms appeared when he was 6 years old. Deformity of interphalangeal joints initially appeared in the fingers. Hips, knees, and wrists were then gradually involved. Diagnosis of JRA was considered by local hospitals, and glucocorticoids were prescribed without any efficacy. As he grew up, his symptoms deteriorated. He had to walk with crutches at 16 years of age because of arthritis of the lower extremities. At 26 years of age, he first experienced progressive pain with numbness radiating down his entire left leg and right thigh. At 34, he started to have mild difficulty in urination. Thereafter, his leg pain progressed and he became immobile. Treatment with tramadol, physical therapy, and spine injection were tried but were not effective. He had a younger brother with a similar clinical presentation but who also had mild neurological impairment (Fig. ).\nThe proband’s height and weight were 162 cm and 72.5 kg when he was admitted to our hospital. His visual analogue scale (VAS) score was 9. He did not have behavioral difficulties and was not retarded in his intellectual development. Physical examination showed multiple malformations of the major limb joints, especially of the knees and hands (Fig. ). Amyotrophy of both lower limbs was obvious. Cervical and lumbar movements were limited with compensatory kyphosis. The muscular strength of all four limbs was normal. Dysesthesia was found in the posterolateral left calf, dorsolateral left foot, and perineal area. Bilateral knee-jerk reflexes and ankle reflexes were hypo-induced. The erythrocyte sedimentation rate (13 mm/h) and C-reactive protein level (2 mg/L) were both within the normal range. Tests for rheumatoid factors were negative.\nSpinal x-rays showed flat and osteoporotic vertebral bodies. Pedicles were short, and end plates were irregular. Bone bridges were seen at many levels. Kyphosis was detected in both the cervical and upper thoracic spine. Magnetic resonance imaging showed multilevel Schmorl nodes. Multilevel disc herniation and hypertrophic ligamentum flavum caused lumbar canal stenosis from L2 to S1 (Fig. ). |
A 20-year-old pregnant woman (gravida 1, para 0 at approximately 13 weeks and three days) presented to her obstetrician’s office with watery vaginal discharge mixed with vaginal bleeding. She had no pertinent past medical or surgical history. An abdominal ultrasound revealed a live twin DCDA spontaneous intrauterine gestation and a significantly low amniotic fluid volume involving fetus A but an acceptable amount of amniotic fluid in Fetus B, with heart rates of 163 beats per minute (bpm) and 168 bpm, respectively (Figure ). The patient was advised to be on pelvic rest and told to go to the emergency department (ED) if there was a worsening or recurrence of her vaginal bleeding. The following week, the patient presented to the ED with similar watery, blood-tinged vaginal fluid and abdominal discomfort. Abdominal ultrasound findings were unchanged from the previous study, and her care team diagnosed her with PV-PPROM. The patient was extensively counseled and chose to manage expectantly with weekly outpatient follow-up visits pending signs of infection or maternal compromise. The patient’s pregnancy was monitored via serial abdominal ultrasounds during office visits that demonstrated live twin pregnancies with continued anhydramnios of Twin A. At 23 weeks' gestational age, she experienced increased leaking of clear fluid, and she was admitted to the hospital for continuous monitoring with daily nonstress tests (NST), ultrasounds every four weeks, and a regimen of antibiotics, betamethasone, and magnesium. Latency antibiotics regimen at admission included azithromycin 1 gram once orally, ampicillin 2 gram intravenous every six hours for two days, followed by an additional five days of amoxicillin 500 milligrams three times daily. During this time, the patient experienced no abdominal discomfort, odorous discharge, or other signs of infection. Abdominal ultrasounds revealed similar findings as before with a finding of live twin pregnancies. While serial ultrasonographic examinations showed an initial delay in the growth of Twin A, it approached normal growth by 27 weeks and showed no obvious signs of pulmonary hypoplasia. A series of NST results were reactive.\nMoreover, there was evidence of Twin A producing amniotic fluid as it was able to maintain a single maximal vertical pocket (MVP) around 2.0 cm despite vaginal leakage of fluids, which was reassuring. The patient was given labetalol for treatment of sinus tachycardia. On day 113 post-PPROM, at 30 weeks, zero days (30w0d), the patient reported concerns of uterine contractions every four minutes with a pain rated as 5/10 on the analog pain scale. Intravenous betamethasone and magnesium sulfate were given, but no additional tocolytics were given. Preterm labor occurred at 30w1d, and a primary low transverse Cesarean section was performed on the 114th day after PPROM. Twin A was a male baby, weighing 1520 g, with an appearance, pulse, grimace, activity, respiration (APGAR) score of seven and eight. Twin B was also a male baby, weighing 1650 g, and had an APGAR score of five and eight. Pathologic examination of the placenta showed no signs of chorioamnionitis. The postoperative course was complicated by endometritis, for which the patient was treated with antibiotics. The patient was discharged after three days. Twin A was admitted to the neonatal intensive care unit (NICU) after showing signs of severe distress and retractions with poor air exchange indicative of pulmonary hypoplasia and was electively intubated. After five days, he was transitioned to nasal continuous positive airway pressure (NCPAP) for two weeks and then transitioned to a high-flow nasal cannula along with furosemide twice daily for four days, then once daily for two months. He was also given spironolactone, chlorothiazide, and budesonide inhaler daily during his hospital course. He also received two periods of 10-day hydrocortisone taper, one month apart. Twin A was discharged after three months and 15 days, showing marked improvement. Twin A was improving and stable on 0.5 L supplemental oxygen, chlorothiazide, and spironolactone. He also received palivizumab.\nTwin B was also admitted to the NICU for prematurity and respiratory distress treated with rescue surfactant and NCPAP, which was discontinued after two days. Then he was transitioned to breast milk and room air with occasional episodes of hypoxia, needing 1 L of nasal cannula oxygen. Twin B was discharged after one month and was doing well without any complications. |
An 11-month-old boy was referred to a tertiary center for failure to thrive, poor muscle tone, short neck, kyphosis, and unusual spacing between teeth. He was diagnosed with infantile HPP after repeated low ALP activity test results and radiographic assessment of severe rickets-like skeletal changes and tongue-like lucencies projecting into the metaphyses. He was hospitalized multiple times for pneumonia likely related to musculoskeletal manifestation of HPP, which required treatment with intravenous antibiotics. At age 9 years, he developed persistent headaches; a magnetic resonance imaging (MRI) cranial scan confirmed craniosynostosis with Chiari malformation and cerebellar tonsillar herniation. He underwent craniovertebral decompression, with removal of the posterior arch of C1; a ventriculoperitoneal shunt was inserted to relieve intracranial pressure. He subsequently underwent 2 shunt revisions.\nThe patient experienced multiple fractures, starting at age 17 years, when he sustained bilateral femoral fractures when jumping off a wall; this required bilateral intramedullary rod insertion. At age 18 years, he sustained a right tibial fracture while jumping. Bone healing was delayed, but the fracture eventually healed satisfactorily. At age 20 years, he sustained bilateral femoral fractures when he rolled off his bed during a seizure and required rehabilitation for approximately 8 months.\nOver the first 18 years of life, the patient was hospitalized 8 times for a total of 43 days (Table ). Of these hospitalizations, 5 separate admissions required a stay of ≥5 days; the stay for insertion of a ventriculoperitoneal shunt was 14 days.\nOutpatient specialist visits represented a significant proportion of the healthcare resource utilization by this patient (Fig. ). Most of the outpatient specialist visits required consultation with providers in 12 specialties, including pediatric dentist (38 visits), pediatric endocrinologist (32 visits), neurosurgeon (27 visits), and general pediatrician (19 visits; Table ). Outpatient management consisted of diagnostic imaging procedures (Table ). The most frequent procedures were radiography of the limbs and spine, performed on 18 and 12 occasions, respectively; the patient also underwent 14 MRI cranial scans. Dental surgery and tooth extraction were performed as day case procedures for management of dental carries and malocclusion on 3 occasions. |
A 36-year-old Japanese man was referred for medical consultation because of behavioral problems at work, suspected to be caused by his communication difficulties. His history was negative for injury or illness from birth through childhood, and he had no previous diagnoses of any psychiatric disorders, including developmental disorder. His relevant family history was unknown. He successfully graduated secondary school, with no obvious impairments in literacy, and was working as a school janitor at the time of referral. No personality disorders were observed. However, his communication skills were weak, which was considered to be the main cause of his social difficulties at work. However, his life-long language disorder was primarily masked because he was seldom in social situations. He reported occasional alcohol intake and tobacco smoking, but neither to excess.\nHis workplace stress had recently increased, which was suspected to have likewise increased his communication difficulties. Specifically, he was increasingly unable to follow instructions. After an initial examination by a neurologist revealed no organic causes for his symptoms, he was referred to a psychiatrist at the authors’ clinic. On intake assessment, it was noted that he muttered to himself and communicated poorly with others. Although aphasia was suspected, no diagnosis was given at that time.\nAt follow-up 2 years later, when our patient was 38 years of age, his Mini-Mental State Examination (MMSE) score was 28/30, which was within normal limits, and his cognitive subscale score of the Alzheimer’s Disease Assessment Scale–Japanese version (ADAS-cog) was 11.6/70, which was borderline between cognitive normal and mild cognitive impairment [, ]. A second psychiatrist took over his case. This psychiatrist conducted examinations regularly for the next 3.5 years before reducing his visits to once per year. He continued this annual schedule from age 41 to 44 years. Again, no diagnosis was given at that time.\nAt age 44 years, our patient again encountered workplace difficulties under a new boss. Our patient’s wife reported that, according to his boss, he could no longer manage working and the boss found him smoking in a nonsmoking area on one occasion. A third psychiatrist was consulted and asked our patient to explain the episode, which he answered with a superficial statement and noted that he considered it to be “somebody else’s problem.” The attending psychiatrist at the consultation also noted that our patient had a limited vocabulary, spoke in incomplete sentences, and often provided responses that were difficult to understand; our patient also seemed to have few social activities. Further questioning confirmed that these communication and social problems had been present since childhood, consistent with his slightly poor school record and limited occupational abilities. According to his wife, this disorder was present when they first met over 20 years earlier. The attending psychiatrist suspected the diagnosis of language disorder based on the Diagnostic and Statistical Manual of Mental Disorders (DSM), Fifth Edition (DSM-V) criteria, and that the developmental disorder had been masked since infancy because our patient was not active in settings that required social communication.\nThe psychiatrist developed a strategy to determine whether these symptoms were psychogenic or organic in origin. Laboratory results showed no remarkable findings and were negative for syphilis, human immunodeficiency virus, and human T-cell lymphotropic virus type 1 and type 2. Concentrations of thyroid-stimulating hormone, free triiodothyronine, free thyroxine, antinuclear antibody, vitamin B1, vitamin B2, vitamin B12, and folic acid were all normal. Otorhinolaryngology testing confirmed normal hearing, and neurologic testing found no evidence of Parkinsonism. In addition, neither dysarthria nor dysphemia were observed.\nTherefore, additional neurocognitive, psychological, and neuroimaging tests were performed to identify neurodevelopmental/communication disorders, including the following: MMSE, ADAS-cog, Frontal Assessment Battery (FAB), Wechsler Adult Intelligence Scale–Third Edition (WAIS-III), Wechsler Memory Scale–Revised (WMS-R), Japanese Standard Language Test of Aphasia and Japanese Raven’s Coloured Progressive Matrices (RCPM) [, ], Parent-Interview ASD Rating Scale–Text Revision (PARS-TR) obtained with the help of his wife, and Autism-Spectrum Quotient (AQ). Total scores were as follows: MMSE, 27/30, indicating the upper limit of mild cognitive impairment; ADAS-cog, 17.3/70, suggesting Alzheimer disease (AD); and FAB, 13/18, which is within normal limits [, , ]. Subset scores were as follows: MMSE delayed recall score, 2/3, suggesting slight impairment of recent memory; ADAS-cog subtests, 8/10 for word recall, 3/5 for both expressive language and language comprehension, and 2/5 for commands []; and FAB verbal fluency, 0/3. Collectively, these results indicated difficulty in language usage and memory. Although the ADAS-cog results suggested the possibility of AD, there was no evidence of dementia according to the MMSE.\nTable shows results of the other neurocognitive tests. At baseline age of 44 years, there was a discrepancy between verbal and visual memory indices on the WMS-R. Results of logical memory I and II subscales were at the third and first percentiles for his age, respectively, and were much lower than those for visual reproduction I and II. No significant discrepancies were observed among group indices of the WAIS-III at baseline, and all the group indices were under 100. Alternatively, his RCPM score for nonverbal neurocognitive performance was much better than average for his age []. His AQ was 31, but he did not meet DSM-V criteria for autism-spectrum disorder (ASD) []; furthermore, the PARS-TR results also provided no evidence of ASD. In addition, the Tokyo version of the Childhood Autism Rating Scale was administered []; however, reliable data could not be obtained because of poor communication between patient and examiner. Other baseline tests and imaging studies were performed to exclude specific organic disorders and are discussed in the context of the 2-year follow-up results for comparison.\nAt follow-up 2 years later, when our patient was 46 years of age, delayed recall index and visual reproduction II percentiles of the WMS-R had decreased from baseline, and delayed recall impairment was apparent in both verbal and visual domains (Table ). The results of other neurocognitive tests were not remarkably different from those at baseline (Table ).\nTo exclude specific organic disorders, such as AD, dementia with Lewy bodies (DLB), frontotemporal dementia (FTD), and other types of dementia, most of the following neuroimaging tests were performed when our patient was age 44 years (baseline) and again at 46 years: magnetic resonance (MR) imaging, (123I) iodoamphetamine single-photon emission computed tomography (123I-IMP-SPECT), 18F-2-fluoro-2-deoxy-D-glucose (FDG)-positron emission tomography (PET), 11C-Pittsburgh compound B (PiB)-PET amyloid imaging, 18F-THK5351 PET tau imaging, and 123I-metaiodobenzylguanidine (MIBG) myocardial scintigraphy. No abnormalities were observed on MR images at either baseline assessment or 2-year follow-up; the images were almost completely normal and did not show small vessel disease. Only age-related slight atrophies were observed at either baseline assessment or 2-year follow-up. Similarly, FDG-PET imaging at baseline showed no evidence of neurodegenerative disease; however, baseline glucose uptake in the cerebrum was reduced compared to that in the cerebellum. Although FDG-PET was not conducted again after 2 years, 123I-IMP-SPECT revealed global hypoperfusion at both baseline and 2 years later (Fig. ). Moreover, posterior cingulate perfusion had decreased further at 2 years after baseline as demonstrated by statistical voxel-based analysis using three-dimensional stereotactic surface projection software. No pathologic amyloid-β deposition on PiB-PET imaging was shown at either time []. Slight tau accumulation was noted in the bilateral medial temporal lobes on 18F-THK5351 PET imaging at both times, but this finding was not considered pathologic. Myocardial scintigraphy, which was performed only at baseline, showed normal MIBG uptake at both early and delayed images (heart/mediastinum ratios of 2.72 and 3.09, respectively).\nNo genetic testing for young-onset dementia was performed because the results of PiB-PET imaging and 18F-THK5351 PET imaging excluded AD, and because several other considerable types of dementia derived from neurodegenerative diseases, including DLB, Parkinson disease (PD), FTD, idiopathic normal pressure hydrocephalus (iNPH), corticobasal degeneration (CBD), and progressive supranuclear palsy (PSP), were also excluded based on clinical features and the results of MR images, 123I-IMP-SPECT, and MIBG scintigraphy.\nCollectively, these investigations suggest that progressive global cerebral dysfunction underlies our patient’s communication disorder. This finding is also supported by our patient’s wife who had noted his communication difficulties since they first met 20 years earlier. On the basis of this history and recent test results, language disorder according to DSM-V and progression of both visual and verbal memory impairments were diagnosed (Table ) []. |
This was a case report of 45-year-old female patient who presented with a complaint of a painful enlarging swelling, 9 cm × 8 cm in size, of 2 years duration in the left maxillary region. On palpation, there was significant buccal and lingual cortical expansion [].\nOrthopantomograph revealed a mixed radiolucent and radiopaque lesion extending from 21 region to the region of left maxillary tuberosity, superiorly it was extending up to the left infraorbital margin with haziness observed in the left maxillary sinus; the floor of the left orbit was intact. Computed tomography (CT) scan in axial view revealed a hyperdense mass involving the entire left maxillary sinus and breaching all the walls of the sinus [].\nBased on the clinical, radiological and the CT scan features, a provisional diagnosis was given as benign odontogenic tumor probably ameloblastoma. The incisional biopsy was reported as desmoplastic ameloblastoma based on which the patient underwent surgical excision of the mass under general anesthesia.\nMacroscopically, the resected mass was grayish-white in color; measured 6 × 5.5 × 4.5 cm, was oval, had a lobulated surface and was firm in consistency. The specimen was cut in the middle and the features were examined. It showed a large cystic area in the center with sprouting mass having a pedicular attachment at one area. The outer area appeared to be solid in nature [].\nMicroscopically, the lesion gave the picture of desmoplastic ameloblastoma in the form of odontogenic epithelium seen as follicles as well as strands simulating cord-like structures in a fibrous stroma. Peripheral columnar ameloblast-like cells were inconspicuous about the epithelial islands; however, in the areas where follicles showed expansion, well-formed peripheral columnar cells with reversed polarity were appreciated. The stromal component was densely collagenized giving a hyalinized picture [].\nAn interesting finding was the presence of abundant eosinophilic, homogenous extracellular material juxtaepithelially. This material was interpreted as dentinoid [].\nIn the peripheral areas of the lesion, plump fibroblasts as well as pleomorphic cells invading the bony trabeculae, were noticed []. These dysplastic cells were negative for cytokeratin 19 and positive for vimentin [], thus reflecting their mesenchymal nature. However, the center of the lesion exhibited odontogenic epithelial strands and follicles which were positive for cytokeratin 19 [] and negative for vimentin [].\nAnother atypical finding was the presence of polygonal cells with abundant granular eosinophilic cytoplasm and peripherally placed nucleus []. These cells were positive for CD68 [] and vimentin [] and were negative for cytokeratin [], thus confirming the stromal nature of the granular cells. |
This was a case of a 48-year-old female patient who presented to the out-patient department (OPD) with the complaints of severe headache, dizziness and palpitation. She was a known case of essential hypertension and on drug treatment for hypertension. She was taking amlodipine (2.5 mg once daily) and metformin (250 mg twice daily) for 6.5 years. She has some difficulty with taste identification and stopped her drug amlodipine 25 days ago before presenting to OPD. On questioning the reason made for stopping the drug amlodipine alone and not metformin was logical for her, adding that her husband was diabetic and on metformin therapy but without any change in taste alteration. Hence, she herself stopped the other drug (amlodipine) she was taking. She also added that there was some improvement in loss of taste sensation. However, the actual complaint which brought her to the OPD was symptoms of hypertension which was due to the stoppage of the antihypertensive drug. On examination, she was found to be apparently normal. Her oral cavity was also examined for any pathology and was found normal. Clinical examination pertaining to central nervous system (including cranial nerve examination) and peripheral nervous system was also within normal. Vitals - Pulse rate - 98/min; blood pressure - 160/100 mm Hg; respiratory rate - 16/min and no abnormal deviation in auscultation findings. Since her blood pressure was high, she was again prescribed the same medication (amlodipine) with dosage increased to 5 mg once daily. At 8 days later, she presented with the similar complaint but with controlled blood pressure proving that amlodipine can cause dysguesia. Electrocardiography was within the normal limits except for changes pertaining to chronic blood pressure. The investigation also excludes any mineral/vitamin (zinc) deficiency []. Hence, she was asked to stop the drug and was changed on other medication which does not cause dysguesia namely cardio-selective β-blocker bisoprolol (2.5 mg). She was followed for next 15 days and 30 days for blood pressure and her status of taste sensation. It was found blood pressure was 110/70 mm Hg taken as average and she feels comfortable with moderate improvement in taste sensation. |
The patient was an 89-year-old woman who visited a neighborhood hospital 4 h after developing abdominal pain and vomiting of sudden onset. She was referred to our hospital 2 h later with the diagnosis of intestinal obstruction. She had a history of undergoing cesarean sections. On arrival at our hospital, her vital signs were stable; examination revealed that she was 145 cm tall and weighed 36.9 kg (calculated body mass index [BMI], 17.5). She had severe tenderness in the lower abdomen, but no signs of peritoneal irritation. Blood tests showed an elevated white blood cell count, although the serum C-reactive protein (CRP) was normal. Blood gas analysis showed mild acidosis, with a pH of 7.383, and a base excess (BE) of − 3.7. Contrast-enhanced computed tomography (CT) revealed a small bowel forming a closed loop, with poor contrast effect, and dilatation of the oral side of the small bowel (Fig. a, b). Ascites was also identified (Fig. c). The patient was diagnosed as having strangulated bowel obstruction, and emergency surgery was performed.\nAt laparotomy, bloody ascites was observed. Two segments of the ileum were tied together forming a knot, and both segments were necrotic due to impaired blood flow (Fig. ). There was a band formation between a nearby segment of the small bowel and the abdominal wall, probably attributable in part to the previous cesarean sections, but there was no evidence of intestinal obstruction. Although it was necessary to release the strangulated small bowel, we did not immediately release the knot, but first proceeded with ligation of the mesenteric vessels draining the strangulated small bowel, to prevent dissemination of toxic substances from the necrotic bowel to the systemic circulation. Ligation of the mesenteric vessels was followed by resection of a 100-cm segment of the knotted necrotic ileum, 10 cm from the ileal end. Hand-sewn anastomosis was performed with the Albert-Lembert suture. The volume of blood loss was 282 ml, and the operation time was 1 h 41 min.\nThe resected specimen showed the two intestinal segments wrapped together forming a knot, as indicated by the intraoperative diagnosis, and the strangulation was released by untying the knot (Fig. ). There were no abnormalities on the mucosal surface other than signs of necrosis. Histopathological examination of the resected ileum showed that the resected small bowel was remarkably devoid of crypt epithelium, and there was severe congestion and hemorrhage extending from the intrinsic mucosal layer to the submucosa, which was considered to represent an ischemic change.\nThe postoperative course was uneventful, and the patient was discharged on the 13th postoperative day. |
A 32-year-old woman presented to the emergency department (ED) with complaint of resolved left-sided facial droop and a lingering paresthesia of her left upper extremity. Her medical history was relevant for recurrent otitis media infections; she was otherwise healthy and worked full time. She was not a frequent air traveller, nor did she have a history of scuba diving. Her symptoms began approximately six hours prior to arrival to the ED while she was aboard a flight across the country. She was not coughing, sneezing, or deliberately attempting a Valsalva maneuver when her symptoms started, but as the plane took off she experienced sudden, severe left ear pain and felt left-sided facial as well as left upper extremity numbness. She also felt that her face was “drooping” and when she checked her reflection, she noticed that she had droop on the entire left side of her face: she could not lift her eyebrow, could not smile or frown, was unable to close her eye and was drooling out of the left side of her mouth. She was given a warm compress for her ear by airplane staff and the symptoms resolved within approximately 30 minutes, although her ear pain remained.\nNeither her droop nor her numbness was present by the time the plane landed. Her only lingering complaint was that of a “strange sensation” she could not describe in her left upper extremity. She specifically denied sensations of numbness, weakness or paresthesias after the event. Strength and sensation were fully intact. She presented to the ED with these complaints. On exam in the ED, her initial vital signs were within normal limits, and her neurologic exam was completely normal. Her National Institutes of Health Stroke Scale was zero. Her tympanic membranes were intact bilaterally, with subtle bulging of the left concerning for otitis media without signs of rupture.\nRoutine laboratory data was unremarkable; however, computed tomography (CT) was notable for small foci of air in the subdural space scattered along the left aspect of the outside of the superior sagittal sinus (), as well as a focus along the left cerebellar tentorium. There was no midline shift or mass effect. Also noted was pneumatization of the squamosal portion of both temporal bones, and both petrous apices. A CT internal auditory canals was performed (). Neurology, neurosurgery, and otolaryngology were consulted for management.\nHer transient facial droop was attributed to an air pocket near the facial nerve that would have expanded with cabin pressure change, but had since been reabsorbed and therefore was not captured on imaging at the time of the patient’s presentation in the ED. A presumed defect in the dura was discussed by both neurosurgery and otolaryngology, although this defect was not identified on imaging. Otolaryngology recommended placement of a myringotomy tube after discharge from the ED. No acute surgical interventions were indicated as per neurosurgery.\nThe patient followed up with otolaryngology the following day and had uncomplicated placement of a myringotomy tube with aspiration of a thick mucoid effusion. She reported immediate resolution of her ear pain following placement. She was scheduled to fly back to her hometown and follow up with her local otolaryngology physician, as well as obtain repeat head imaging to confirm resolution of the PNC. |
A 59-year-old female with left TKA was referred to our clinic complaining of left knee pain and instability. She had an extensive orthopedic history as a result of significant bilateral tricompartmental arthritis unresponsive to conservative management, including a reported history of 10 surgeries on her right knee related to TKA. She had undergone a left TKA 6 years earlier using a PCL-sparing prosthesis (Zimmer Natural-Knee gender specific high flexion knee system, Zimmer Inc., Warsaw, IN, USA). Following this procedure, she had recurrent sharp lateral joint pain during activity, requiring arthroscopic lysis of adhesions after 1 year. At that time, she did not experience any issues with instability. Twenty months postoperatively, she was noted to have 120 degrees of flexion with good varus-valgus stability but continued to have pain. She underwent revision TKA 22 months after the index procedure and intraoperatively was found to have lateral impingement due to scar tissue along with loose tibial and patellar components. At this setting, she underwent debridement of scar tissue with synovectomy and revision of femoral and tibial components. This revision lasted 34 months, before atraumatic dislocation of the polyethylene insert after standing from a seated position prompted another revision. In this procedure, she was revised to a condylar femoral constrained prosthesis, the NexGen Legacy Constrained Condylar Knee (LCCK), Zimmer (Zimmer Inc., Warsaw, IN, USA). This prosthesis is designed to limit varus/valgus movement to 1.25 degrees and internal/external rotation to 2 degrees. After 10 months with this condylar constrained prosthesis, she again experienced dislocation of the polyethylene insert. At that time, she underwent another revision replacing her 19 mm polyethylene insert with a 22 mm insert due to lateral collateral ligament (LCL) laxity that was documented intraoperatively. This thicker liner was observed intraoperatively to stabilize the joint without altering the knee's mechanics and range of motion. Despite this change, she experienced another dislocation of her polyethylene liner only 5 months after her revision. She underwent her fourth revision, and after the knee could not be reduced with a trial of a 25 mm liner, a 22 mm liner was again used. Following this revision, she continued to experience instability with minimal activity and was referred to our clinic for evaluation for PLC instability and possible ligament reconstruction.\nOn exam, she was noted to have significant LCL laxity, especially in flexion, and increased posterolateral rotational instability (positive dial test), consistent with PLC instability. The patient was scheduled for PLC reconstruction to restore stability to her knee. This reconstruction was accomplished by a modified version of the fibular-based technique described by Larsen et al. [] (Figures and ). A tibialis anterior allograft was used instead of the described semitendinosus graft, and no figure eight loop was incorporated into the reconstruction. The allograft was passed through a single 7 mm fibular tunnel created in a slightly oblique anterolateral to posteromedial direction (Figures and ). Then, a single tunnel of 9 mm diameter and 30 mm depth was created at the femoral site and both ends of the graft were attached at the same femoral site with a Milagro Advance (Depuy Synthes Mytek, Raynham, MA, USA) interference screw (Figures and ). Two separate Beath pins were drilled lateral to medial to the femoral socket to create two independent tunnels. Then, the sutures placed at each end of the graft were passed through these lateral-to-medial tunnels to create aperture fixation in the medial side (Figures and ). The graft was tensioned into place with the knee in 30 degrees of flexion, slight valgus stress, and internal rotation of the foot. Intraoperatively, there was no observable varus or posterolateral instability following placement of the graft.\nAt 1-year follow-up, the patient had no pain or subjective instability. She had pain in the anterior thigh and limited knee flexion for several months, which significantly improved with deep tissue massage into the distal quadriceps area. The range of motion was 5° loss of extension to 120° of flexion. The varus stress test revealed no lateral opening and the dial test was negative. The patient had a score of 54.8 for the Physical Component Summary and 59.8 for the Mental Component Summary of the SF-12. The patient had a Knee Score of 83 and a Function Score of 100 in the Knee Society Score. On a 0-to-10 satisfaction scale where 10 is the maximal satisfaction, the patient rated her surgical treatment as 10. |
An 80-year-old male patient who was found to have stomach wall irregularities during routine medical check-up was referred to Keio University Hospital. Endoscopic examination detected a 20-mm protruding lesion in the greater curvature at the middle third of the stomach (Fig. ). The tumor was almost covered by normal mucosa, and the top of the lesion was partly depressed. Narrow-band imaging (NBI) showed the vascular surface pattern to be irregular. Further, EUS showed a thick, low echoic lesion in the second layer of the gastric wall, and the nodular part of the lesion had infiltrated into the submucosa (Fig. ). On the basis of the above findings, we suspected the tumor to be gastric cancer mimicking SMT, similar to linitis plastica of the stomach with SM invasion or a lymphoproliferative disorder such as a malignant lymphoma. However, the endoscopic mucosal, boring, and mucosal-incision-assisted biopsies revealed only a small number of atypical epithelial cells and no malignant cells. Additionally, CT and fluorodeoxyglucose positron emission tomography (PET) showed no lymph node metastasis. We diagnosed the tumor to be a gastric submucosal tumor with a high suspicion of malignancy based on the qualitative diagnosis. Therefore, an excisional biopsy was planned. Using endoscopic diagnosis as the reference, ESD was not selected because a conventional radical gastrectomy would have been highly invasive. Instead, we planned a partial gastrectomy using LECS for tumor resection.\nTo avoid peritoneal dissemination, we chose the modified CLEAN-NET procedure []. Since the EUS revealed that the tumor was unlikely to spread laterally, we planned to excise the raised part only. The cutting line was decided to be 4 mm outside of the expected border.\nThe technical details of this surgery involve (1) detecting the lesion and performing an endoscopic submucosal injection, (2) performing seromuscular dissection around the tumor under laparoscopic view, (3) cutting the mucosal layer using a mechanical stapler (Fig. ), (4) and suturing the seromuscular layer.\nThe procedure duration was 51 min, blood loss was 0 mL, and there is no perforation of the gastric wall. The patient’s postoperative course was uneventful. Histopathological analysis of the specimen identified the tumor to be a moderately differentiated adenocarcinoma, pT4a(SE), Ly1a, V0, negative lateral margins (Fig. ). On the basis of these results, the tumor was classified as pT4a cN0M0 cStage IIB (according to TNM classification, 8th ed.), and an additional radical gastrectomy was planned.\nA robotic-assisted distal gastrectomy with D2 lymphadenectomy was performed 6 weeks after the first procedure. The duration of the procedure was 338 min, and the blood loss was 50 mL. Adhesions from the previous procedure were not severe, and the postoperative course was uneventful. At the time of the final pathological diagnosis, there was no metastasis in the regional lymph nodes and no residual carcinoma in the resected stomach. The patient refused adjuvant chemotherapy of S-1 because of his old age and underwent follow-up examinations every 6 months. |
A 60-year-old Sri Lankan woman with longstanding hypothyroidism, diabetes mellitus, hypertension, hyperlipidaemia and bronchial asthma presented to a general medical ward with a recent history of a large, spontaneous, painless bruise over her right thigh. Medication included low dose aspirin 75mg daily. There was no family history of bleeding disorders and she was haemodynamically stable. An ultrasound scan excluded coexisting deep soft tissue haematomas and a full blood count demonstrated a white blood cell count of 11.2×109/L with normal differentials, haemoglobin level of 12.3g/dL and a platelet count of 258×109/L. Coagulation screening revealed an aPTT of 66.4 seconds with normal bleeding, prothrombin and thrombin time, results that were confirmed over repeated assays. The results of her blood films, urea, electrolytes, creatinine and liver function tests were all normal.\nFurther investigation in our haematology unit demonstrated the presence of a time-dependent inhibitor of coagulation via prolonged aPTT and a mixing study that did not correct with the addition of normal plasma and incubation for 2 hours (aPTT was 52 seconds when the mixing test was performed, with a ratio of her plasma to normal plasma of 50:50). A mixing study of incubated and fresh mixed plasma did not demonstrate a temperature-dependent inhibitor of coagulation (aPTT was 27 seconds with a ratio of her plasma to normal plasma of 50:50). Clotting factor VIII assay and inhibitor titres were not possible due to a lack of facilities. An indirect assay of deficient factor was carried out by adding factor VIII or IX deficient plasma to her plasma. The aPTT was corrected by adding factor IX deficient plasma, but not by factor VIII deficient plasma, thus suggesting factor VIII deficiency. Plasma fibrinogen was 260mg/dL (150 to 250) and platelet aggregation studies were compatible with the expected aspirin-induced changes. This therefore suggested a diagnosis of acquired haemophilia A. Investigation for associated conditions revealed positive antinuclear antibody (ANA) and antithyroid peroxidase (anti-TPO; microsomal) antibody titre of over 1/80 and 1000IU/L respectively. Her thyroid-stimulating hormone (TSH) level was 4mU/L (normal range 0.3 to 4.2mU/L) during the present admission. A previous hyperthyroid state with TSH of <0.01mU/L and free thyroxine (T4) of 2.87ng/dL had led to the present hypothyroidism with a corrective thyroxine replacement therapy of 100μg daily. The present state of hypothyroidism with a high titre of anti-TPO antibody was suggestive of autoimmune thyroiditis. Anti-double-stranded DNA was negative. Tests for lupus anticoagulant and anti-cardiolipin antibody were negative. These tests were carried out due to the isolated prolonged aPTT and the positivity of ANA respectively.\nAcquired haemophilia A in association with autoimmune thyroiditis was therefore diagnosed. It was promptly treated with a combination of oral prednisolone 60mg daily and alendronate, calcium and vitamin D therapy for bone protection.\nFour weeks later she presented with painful swelling of her right calf muscle. There were no features of compartment syndrome and the diagnosis of deep muscle haematoma was confirmed by ultrasound examination. The aPTT was 96 seconds on admission. Acute limb-threatening bleeding was successfully managed with recombinant activated factor VII (NovoSeven®). Cyclophosphamide 100mg daily was added to the immunosuppressive regime to induce remission. Eight weeks later, aPTT returned to within the normal range and there were no further haemorrhagic manifestations. |
The present case report is about a 28-year-old female patient who presented for the evaluation of a swelling on the front region of her face. Clinically, the swelling was painless with associated nasal discharge and deviation of the nasal septum. She had a bony hard, non-tender swelling of approximately 3 × 2 inches, extending superoinferiorly from the infra-orbital ridge of the right side to the level of the right corner of the mouth and anteroposteriorly from the right corner of the mouth to the anterior border of the ramus []. The borders of the swelling were diffuse and the skin overlying the swelling was normal in color. Intra-oral examination revealed a non-tender, bony hard swelling extending from the maxillary right central incisor to the right second premolar, thereby obliterating the buccal vestibule []. The associated teeth were displaced and showed Grade-I mobility.\nThe computed tomography (CT) scan revealed a solitary, homogenous hypodense lesion of the right side maxilla encroaching onto right maxillary antrum as well as the right side of the nasal cavity. The lesion crossed the midline medially, extended superiorly to right infra-orbital margin and inferiorly to the right side palate []. CT 3-D reconstruction of paranasal sinus region revealed cortical destruction of the right side maxilla encroaching onto right maxillary antrum as well as the right side of the nasal cavity and also showed displacement of teeth and root resorption []. Orthopantomograph revealed a unilocular radiolucency involving the right maxilla with tooth displacement and root resorption [].\nThe lesion was surgically removed. On gross examination, the specimen was a white gelatinous mass. Histopathological examination of the biopsy specimen revealed haphazardly arranged stellate, spindle shaped and round cells in an abundant, loose myxoid stroma that contained only a few collagen fibrils [Figures and ]. The loose, myxomatous tissue was seen filling the marrow spaces between the bony trabeculae []. |
A 20-year-old male was referred to the Department of Conservative Dentistry and Endodontics because of swelling in his left mandibular region. His medical status was noncontributory. According to the patient's clinical records, he reported a history of pain in the lower left side 1 month back. He consulted a general dentist, where caries excavation in the left mandibular first molar (tooth 36) was done followed by temporary restoration.\nOn extraoral examination, there was a bone expansion on the left area of the mandible. On intraoral examination, there was a hard swelling of the buccal vestibule cortex in the concerning region covered with normal mucosa. Tooth 36 had occlusal caries, which was slightly tender to percussion with probing and exhibited normal mobility. The electronic pulp test was negative for tooth 36. A panoramic radiograph showed a well-circumscribed radiolucent lesion located around the apex of the distal root of tooth 36 [] just above the mandibular canal. The lesion was approximately 10 mm in maximum diameter. After evaluating all the data, a root canal treatment of the left first molar was planned. At the same appointment, the root canal treatment was initiated on tooth 36. A rubber dam was applied and the access cavity was prepared. A hemorrhagic, purulent exudate was found from the distal canal of tooth 36. The working length was estimated using an apex locator. The distal canal was instrumented with size 15–40 K-files and mesial canals with size 15–30 K-files using a step-back technique. During the instrumentation, the canal was irrigated copiously with 3% sodium hypochlorite solution using a 27-gauge endodontic needle after each instrument. Drainage was performed until the discharge through the canal ceased. The canals were dried with sterile paper points and then dressed with calcium hydroxide. A sterile cotton pellet was inserted into the access cavity before sealing it with a temporary filling material. The calcium hydroxide dressing was changed every 1 week for three times. After 3 weeks, the discharge from the canals did not cease completely. The treatment procedure was changed. The canals were irrigated and the smear layer was removed with 17% EDTA followed by 3% sodium hypochlorite. The canals were dried, and a triple antibiotic paste consisting of ciprofloxacin, metronidazole, and minocycline (100 mg of each drug in 0.5-ml total volume) was placed with the help of a lentulo spiral. The compounding of the antibiotic paste was standardized for all three cases. The paste was changed every month for a period of 3 months until the teeth displayed no symptoms. On examination, the teeth showed no pain on percussion, soft tissues were found healthy, and the canals were dry. The canals were irrigated with 3% sodium hypochlorite followed by normal saline and obturated with gutta-percha and AH plus by using a lateral compaction technique. The restoration was accomplished with silver amalgam. After 12 months [] and 16 months [], the radiographs showed complete bony healing with well-defined trabeculae. |
A 7-year-old boy was admitted to the hospital with a guillotine-type pyramidal amputation of the distal one-third of the glans penis distal to the corona of the glans penis (). The prepuce was also excised completely. The circumcision had been done in an outpatient clinic by a person who was neither a urologist nor a surgeon and who had inadvertently caught the glans in the clamp, which resulted in glans penis amputation. The glans penis had been stored in sterile saline solution until reaching our hospital after 1 hour, and the reconstruction was performed within 2 hours of the amputation.\nThe cut surfaces were cleaned with sterile saline and swabbed with povidone-iodine. The urethra was stented with an 8 Fr urethral catheter from the external urethral meatus through the proximal urethra. It was hard to see the proximal part of the intact urethral mucosa to dissect and make anastomotic sutures in the glans penis. Therefore, we just approximated the separated parts of the urethral mucosal surfaces over a urethral catheter. Then the amputated part of the glans penis and the proximal glans penis were anastomosed with intermittent 4/0 Vicryl. The proximal penile skin and mucosa of the glans penis were approximated with intermittent 4/0 Vicryl to complete the circumcision (). No tourniquet was applied to the radix penis during the operation for bleeding control. The glans was pale pink in the first day after the operation. In our opinion, this was an early sign of blood perfusion of the glans penis. Care was taken postoperatively to immobilize the anastomosis. We did not need to place a cystostomy catheter.\nOn the second postoperative day, edema and some eggplant discoloration were seen at the distal part of the anastomosis. Because of this insufficient oxygenation of tissue, HBO therapy was started immediately and was continued until the 20th postoperative day. We gave cefuroxime axetil 500 mg tb during the postoperative period for 10 days. On the 7th postoperative day, partial necrosis developed over the glans penis near the frenulum (). On the 12th day the necrotic crust was debrided superficially and on the 14th day the urethral stent was removed. The glans gained vitality progressively and had healed completely by week 4. The patient voided with a good stream (). No urethral fistula or stricture developed at the anastomotic site. After 2 months, there was no meatal stenosis, voiding was normal, and the cosmetic and functional results were still excellent. |
A 28-year-old man presented to the emergency department of our hospital complaining of sudden painful loss of vision in the left eye. He gave a history of hit by a sharp metallic object on the left eye while hammering an iron plate. His left eyelids were mildly swollen, but the orbital rim was intact with no crepitation. Visual acuity in the left eye was limited to the perception of hand motion, while the visual acuity in the right eye was 20/20, and the left intraocular pressure (IOP) was not measurable. Slitlamp examination revealed a full thickness scleral laceration of 4.0 mm length with prolapsed uveal tissue, a shallow anterior chamber, and a traumatic cataract. The details of the posterior segment could not be visualized. Computed tomography (CT) demonstrated an intraorbital foreign body with intensity of iron that had passed through the left eyeball and was located in the intraorbital space close to the optic nerve (Fig. ). The right eye was normal.\nThis study was conducted at the Shandong University Qilu Hospital and the procedures used were approved by the Ethics Committee of the Shandong University Qilu Hospital. The procedures conformed to the tenets of the Declaration of Helsinki.\nThe primary repair of the scleral perforation with abscission of the prolapsed and necrosed uveal tissue was done on the emergency basis. The conjunctiva was incised along the limbus cornea, the sclera was exposed, and the scleral laceration was confirmed and sutured. We actually attempted to maneuver the foreign body behind the eyeball with the use of a magnet, but this was not successful. We therefore severed medial rectus muscle. An iron foreign body was found and was removed in a single piece (Fig. ). From outside of the eye, the exit laceration could not be confirmed. The operation was completed without a scleral suture of the exit laceration. Postoperatively, the intravenous antibiotics were administered, topical antibiotics and steroids with cycloplegics. Visual acuity in the left eye was perception of hand motion, the wound was healthy with intact sutures, the anterior chamber was formed, and the lens was cataractous with no view of the retina. The IOP in the left eye was 13 mm Hg. Removal of the foreign body was confirmed by a postoperative CT scan.\nTwelve days later, the patient underwent pars plana lensectomy and 3 ports (23-gauge) pars plana vitrectomy. We observed a scleral exit laceration near the optic disc, but did not see any objects. The full-thickness posterior pole defect was closed. With endolaser photocoagulation and silicone oil injection, the patient had his retina reattached. On follow-up after 4 weeks, the visual acuity was always limited to hand motion perception, scleral sutures were intact. The anterior chamber was formed. The IOP was 15 mm Hg. At 3 months after the operation, visual acuity in the left eye was the perception of hand motion and the left IOP was 15 mm Hg (noncontact tonometer). There were no postoperative complications (including retinal detachment, proliferative vitreoretinopathy, infection, sympathetic ophthalmia, and hemorrhage). |
A 36 year old Caucasian woman with a 4 year history of infertility presented at 9 weeks gestation for antenatal care following a successful single embryo transfer after intra-cytoplasmic sperm injection (ICSI). First Trimester screening at 12 weeks gestation demonstrated a trisomy 21 risk of 1:2760 and a trisomy 18 risk of 1:4060. However, detailed ultrasonic examination of the fetus revealed a hypoechoic area on the anterior abdominal wall which was thought possibly to be fluid distending the urethra or an extra-abdominal mass such as an omphalocoele. At 16 weeks gestation a repeat ultrasound examination identified an umbilical cyst, of dimensions 1.7 cm x 1.6 cm x 1.8 cm with vessels coursing around it with no gut present within and a normal anterior abdominal and bladder wall. The uncertainty of the type of cyst and possible reduction in the fetal dimensions required trisomy 18 to be excluded and an amniocentesis revealed a normal XX karyotype. The umbilical cyst increased progressively with advancing gestation, increasing in dimension, to 4.9 x 4.5 x 4.7 cm at 35 weeks gestation (See\n).\nOn the ultrasound, the cord developed homogeneous echogenicity, which was thought to be due to increased Wharton’s jelly, seen more commonly with pseudocysts of the cord; or due to oedema which was a more sinister sign of possible cord compression/cord accident. In view of the latter, after a course of preoperative maternal cortico-steroids to enhance fetal lung maturity, the patient underwent an elective caesarean section under a combined epidural/spinal anaesthetic block, with delivery of a healthy female infant. Unfortunately, during the process of the delivery the cord cyst ruptured making accurate diagnosis impossible. However the cord was thickened with Wharton’s jelly and was elongated with an umbilical stump with overlying skin up to 2 cm in length. The cord was divided in the standard fashion and a normal placenta was removed.\nOn the 5\nth day post-delivery, the cord clamp fell off and each time the baby cried a stream of urine jetted out from the umbilicus (see\n). This phenomenon finally ceased 4 weeks after delivery, following successful treatment of a urinary tract infection. A patent urachus was confirmed on the ultrasound. The very prominent umbilical stump created the appearance of a ‘pseudophallus’ due to bulging from increased intra-abdominal and bladder pressure after each normal micturition.\nA successful laparoscopic excision of the urachus was performed 11 weeks after delivery. At surgery, the entire dome of the bladder was seen to be in continuation with the umbilicus rather than the more commonly seen urachal tract. The ureteral orifices in the bladder were not affected by the closure, whilst a micturating cysto-urethrogram performed post-operatively failed to show any evidence of uretero-vesical reflux. The baby was discharged home 36 hours after surgery. The baby had no further urinary tract infections post-operatively and follow-up blood pressure, growth and development were normal at 18 months of age. |
This patient is a 14-year-old male, who felt a popping sensation and significant right knee pain while jumping and colliding with another player during a basketball game the previous day. Following the injury, he was evaluated in an outside emergency department, where anterior, posterior, and lateral radiographs obtained in the emergency department demonstrated a tibial fracture consisting of two primary components (). He was placed in a cast and sought a second opinion regarding findings and management.\nUpon presentation to the clinic the following day, he reported mild pain (3/10) and noted no normal function of his leg. A physical exam was performed but was limited due to pain. Following the review of radiographic imaging, an MRI was performed, which demonstrated a type IIIB tibial tubercle avulsion fracture and complete tear of the patellar tendon from its distal attachment site, as well as a hematoma at the fracture site (). After discussing the findings with the family, the patient was scheduled to undergo open reduction internal fixation of a type IIIB fracture and repair of the patellar tendon three days following the initial injury.\nAn 8-centimeter anterior incision was made at the superior aspect of the tibial tubercle and extended distally. At the patellar tendon insertion site on the tibia, the tendon was noted to be completely avulsed from the bone cortex distally, while proximally, the tendon remained attached to the displaced tubercle. The tendon remained attached to the inferior pole of the patella. The anterior tibial plateau fragment was anatomically reduced using two fully threaded noncannulated screws (Arthrex, Naples, FL), while the tibial tubercle fragment was reduced via bicortical fixation with a 50 mm fully threaded 3.5 mm cortical screw (Arthrex, Naples, FL).\nThe distal patellar tendon was completely avulsed through two-thirds of its length. To restore the native footprint of the patellar tendon, a 4.5 mm PEEK (polyetheretherketone) corkscrew anchor (Arthrex, Naples, FL) was placed slightly lateral to the anatomic insertion site to avoid a stress riser on the anterior tibial cortex. The anatomic repair of the patellar tendon was completed with two mattress sutures and tied.\nIn addition to the avulsion of the patellar tendon and periosteum, it was noted that fascial tissue with tibialis anterior muscle belly avulsed through the injury site causing subacute extensive bleeding within the anterolateral compartments (). This scenario raised concern for impending compartment syndrome, and an anterolateral compartment release was planned.\nThree 3-centimeter incisions were made along the anterolateral aspect of the leg. The first was located 3 centimeters distal to the neck of the fibula, the second was located 10 centimeters above the distal fibula tip, and the third was located at the midpoint between the two. Under endoscopic visualization, the intramuscular septum was identified and Metzenbaum scissors were used to cut through the fascial compartment beginning in the anterior compartment and extending proximally then distally to the midtibia (). The fascial incision was extended posteriorly into the peroneal compartment and then was extended proximally and distally to the midtibia. These steps were repeated for the midpoint and distal incision sites. Distally, the course of the superficial perineal nerve was identified and the nerve itself was protected during the distal release of the anterior compartment. It was believed that the impending compartment syndrome occurred due to damage to the surrounding bony and muscular tissue. A medium Hemovac drain was placed along the length of the lateral compartment, exiting in the posterolateral proximal leg. The patient was placed in a hinged knee brace which was locked in extension. He was discharged home later that day.\nOn postoperative day number two, the patient's Hemovac drain was removed by a family member. The patient was seen 1 week postoperatively and noted moderate pain (6/10) and 0% normal function. On physical examination, incisional sites were clean, dry, and intact and a small fracture blister was noted on the posterior aspect of the knee—which was cleaned and redressed. Radiographic imaging revealed well-positioned screws, no evidence of new fractures or foreign bodies, and early evidence of callus formation. Two and a half weeks after surgery, the patient presented to the clinic for evaluation. He reported that he had no pain (0/10) and had 5% of his normal function at this time. On physical examination, he noted no tenderness to palpation of the knee joint, and he had 40 degrees of knee flexion. Anterior-posterior and lateral X-rays were taken which showed evidence of callus formation in the bone (). At this time, it was recommended that the patient begin gentle active range of motion exercises with extension and light flexion. He was also encouraged to become full weight-bearing with the brace until its removal two months postoperatively.\nFive months postoperatively, the patient reported no pain (0/10) and possessed 95% of his normal function at this time. On physical examination, he was nontender to palpation along the joint line. There was no laxity with varus or valgus stress. He demonstrated 5/5 quadriceps strength with no evidence of an extensor lag. He had an active range of motion from 0 to 130 degrees of flexion, and there was no lag with straight leg raise. Repeat anterior-posterior and lateral X-rays demonstrated a well-reduced tibial tubercle fracture as well as well-positioned and nondisplaced hardware (). |
A 42-year-old woman came to Seoul National University Dental Hospital complaining of intermittent stinging pain in her right cheek. Previously, she had visited a local dental clinic due to intermittent pain in the past 6 months. The vitality test of upper-right teeth had been performed. Even though the teeth had been vital, she had received endodontic treatment on the upper-right second premolar due to worsening pain. Then, she was referred to the Department of Oral and Maxillofacial Radiology at our hospital when she complained of new sharpening pain on the right side of her face after two or three days.\nThere were no clinically abnormal findings in physical and oral examinations. The patient only had a history of orthognathic surgery on both the maxilla and the mandible performed about 21 years prior to this visit. Panoramic and Waters' radiographs revealed increased haziness and dome-shaped homogeneous radiopacities in the lower regions of both maxillary sinuses (). Endodontic retreatment was performed on the upper-right second premolar because it was thought that radiologically, the origins of the pain were not in the maxillary sinuses but in the teeth, resulting in only a temporary reduction of pain that later intensified.\nComputed tomography (CT) was performed for finding other origins of the pain, and low-attenuation lesions were detected in both maxillary sinuses. The lesions measured approximately 36mm×27mm×20 mm in the right maxillary sinus, and 30mm×21mm×25 mm in the left maxillary sinus. The margins were undulated, while corticated discontinuously. The lesions extended slightly below the floor of both nasal sinuses. No internal calcifications were present. Postoperative signs including antral wall thickening, sclerotic change, and loss of continuity of the cortical antral wall lining, were not observed on the CT images. Furthermore, there were no characteristic features of distinct expansion or bone destruction of soft tissue (). The lesions were eventually diagnosed as retention pseudocysts formed between septa.\nThe origins of the pain seemed to be not odontogenic lesions but the low-attenuation lesions in both maxillary sinuses. In order to resolve the patient's severe pain, enucleation of the cysts with the removal of the plates and screws on both maxillary sinuses was performed, and biopsy specimens were obtained for histopathological examination.\nPhotomicrographs showed that inflammatory cells had infiltrated around the cystic cavity (). The lesions had true cystic structures lined by ciliated, pseudostratified columnar epithelium (). These findings of the microscopic examination were consistent with the postoperative maxillary cysts. Finally the lesions were diagnosed with them in both maxillary sinuses.\nAt 21 months of follow-up, the lesions healed uneventfully and there was no evidence of local recurrence. |
A 43-year-old male sustained an extensive penetrating injury to his left frontotemporal and preauricular region—from a broken grass-cutting blade—with an incompressible pulsating lesion (). He was immediately transferred to a trauma center. His vital signs showed systolic blood pressure of 60–70 millimeters of mercury and his pulse rate was 140 beats per minute and oxygen saturation was 80%. The physical examination suggested an active arterial bleed from the maxillary artery, anterior to the mandibular neck. A few minutes after the patient arrived, his consciousness was deteriorating to E1V1M5 and an endotracheal tube was inserted. An immediate transfusion of group O red blood cell was initiated.\nThe trauma plus protocol (The protocol trauma team activation for severe trauma patient of institute) was activated and the patient was immediately transferred to the operating room. A longitudinal skin incision along the anterior margin of the left sternocleidomastoid muscle was performed. The posterior belly of the digastric muscle was cut to reveal the carotid bifurcation clearly. The left common facial vein was divided. The left common carotid artery, external and internal carotid arteries were exposed and encircled by vascular slings. Transient left ECA occlusion by application of a Rumel tourniquet was performed (). A massive artery bleedwas controlled. The duration between the activation of the trauma protocol and the occlusion of the ECA was 8 minutes. The penetrating tract at the preauricular region was tamponaded by balloon (). Subsequently, the patient was transferred to computed tomographic angiography (CTA) of the brain and neck to evaluate the adjacent structure and intracranial vascular injury. The study revealed a metallic foreign body with multiple left side skull fractures with multiple comminuted fractures involving the zygomatic process of the left temporal bone, lateral wall of left orbit, left orbit apex, left superior orbital fissure and left optic canal, squamous part of left temporal bone, wall of sphenoid and ethmoid sinuses, and the greater and lesser wing of the sphenoid bone (). There was acute subarachnoid hemorrhage along the left Sylvian fissure and an acute subdural hemorrhage along the frontotemporal convexity with brain swelling (). There was no evidence of intracranial arterial injury (). The patient was returned to the operating room and the foreign body was carefully removed. A completed transection of the maxillary artery was identified and ligated, then the ECA was released and revealed no active bleeding site. The total duration of the transient ECA occlusion was 100 minutes. The foreign body fragments were removed. The dura was repaired and a subgaleal drain was placed. The patient had received 6 units of packed red cells, 6 units of fresh frozen plasma and 4 units of single donor platelets. Ongoing resuscitation continued in the trauma intensive care unit (ICU). The preauricular area was re-explored over the next 2 days. The left mandible condyle fixation with plate and screw was performed and the complete transection of the facial nerve was re-anastomosed to the great auricular nerve. The duration of stay was 10 days. The patient had good levels of conscious with a GCS of E4V5M6 and motor power grade V. The patient was paralyzed on the left side of the face from the facial nerve injury and suffered permanent visual loss in the left eye due to extensive optic nerve damage. |
A 24-year-old Caucasian man presented with a several month history of muscle pain, fatigue and insidious onset of pitting edema to his lower extremities. His symptoms progressed to include bilateral arm swelling, muscle pain to the thenar eminence of both hands, and paresthesias to his hands. He did not have a rash, joint pain, Raynaud phenomenon, oral ulcers, fever, hardening of the skin or weight loss, and he denied any cardiac, respiratory, genitourinary, or gastrointestinal symptoms. Prior to symptom onset, he had traveled to South America and reported possible ingestion of undercooked meat as well as swimming in a river and lake. He had frequent visits to the Northeastern United States and had recently spent an extended period of time outdoors in Rhode Island. Medical, surgical, and family histories were unremarkable, and he was not taking any medications. He had a history of mild alcohol intake, no history of smoking, and some marijuana use. On examination, his vital signs revealed a blood pressure of 104/57 mm Hg, pulse of 56 bpm, and normal temperature. He had no significant findings on head, neck, cardiovascular, respiratory, or abdominal exam. He had no cervical, axillary, or inguinal lymphadenopathy. He had significant pitting edema on his feet and legs extending up to his knees as well as non-pitting edema on the dorsum of both hands. Although there was edema, the skin was soft without any significant hardening and was without any overlying erythema. There were no signs of skin dimpling or grooves. His neurological examination revealed normal strength.\nHis initial laboratory work was significant for a mild eosinophilia of 700 with a normal white blood cell count, hemoglobin, and platelets. He had normal calcium, creatinine, and electrolyte levels. His alanine aminotransferase was slightly elevated and total protein slightly low, but he had normal albumin and bilirubin. His thyroid-stimulating hormone was slightly increased, but his free T4 and total T3 were normal. His urinalysis was normal. He had a normal level of creatine phosphokinase, sedimentation rate, and C-reactive protein. Further workup revealed a positive anti-nuclear antibody with a titer of 1:160 with a speckled pattern. His extractable nuclear antigen panel was negative, including Scl-70. His ANCA, myeloperoxidase and proteinase-3 serum studies were also negative. His ACE level and complement levels were normal. Infectious workup revealed negative stool studies for culture, ova, and parasites. Antibodies for HIV, CMV, and Trichinella were negative. Serum for histoplasmosis, cryptosporidium, coccidiomycosis, and interferon gamma release assay were negative. A blood smear for parasites was negative. An ELISA IgG/IgM test for Lyme disease was positive with subsequent testing with Western blot strongly positive for IgG (eight out of ten bands positive) and also positive for IgM (two out of three bands positive).\nThe patient had extensive imaging done with a normal CT scan of his neck, chest, abdomen, and pelvis. An echocardiogram was also normal. Due to a previous negative workup along with persistent pain and swelling, an MRI of his right lower extremity was performed (see Figure ). Imaging demonstrated extensive circumferential edema with enhancement of the superficial soft tissues, superficial fascia, and, to a lesser extent, deep fascia of the lower leg. Taking into account the mild peripheral eosinophilia and the imaging findings, a diagnosis of eosinophilic fasciitis was considered. To complete the workup, a biopsy of the fascia, muscle, and adipose tissue of the left calf was taken (see Figure ). The biopsy did not include the dermis. Surprisingly, there was no evidence of eosinophilic fasciitis. Instead, the specimen illustrated a striking granulomatous fasciitis and vasculitis. The fascia showed exuberant granulomatous inflammation (Figure A) with an inflammatory infiltrate that was made up predominately of histiocytes and CD3-positive T cells with very rare eosinophils (Figure B). The granulomatous inflammation centered primarily on small- to medium-sized blood vessels and was non-necrotizing. While the vessels did not display overt fibrinoid necrosis, they did appear damaged with loss of endothelial cells confirmed with CD31 immunostaining. The inflammatory infiltrate was seen extending into adipose tissue and particularly around blood vessels within the fat. The adjacent skeletal muscle also showed perivascular inflammation and vasculitis in both the perimysial and endomysial compartments. There was no endomysial fibrosis, fatty infiltration, or inflammation surrounding muscle fibers. AFB (acid-fast bacteria) and Wade-Fite stain were negative for mycobacterial organisms. GMS (Grocott-Gomori's methenamine silver) stain was negative for fungal organisms. Due to the positive Western blot for Lyme, a Warthin-Starry silver nitrate stain was performed to evaluate for spirochetes; however, no definitive organisms were seen. A Borrelia PCR analysis of the tissue was performed as well, but no DNA was detected.\nThe patient was given a diagnosis of granulomatous fasciitis along with a diagnosis of Lyme disease. He was first treated with doxycycline for 42 days straight due to initial Lyme serology being positive. The calf biopsy revealing fasciitis was not performed until the patient was 3 weeks into the doxycycline course. Once fasciitis was diagnosed, he was started on a prednisone taper starting at 1 mg/kg/day for a week with taper by 10 mg every 2 weeks. After 2 months of treatment with prednisone, the patient had near resolution of symptoms. A repeat MRI was performed 82 days after the initial MRI with the previously seen changes consistent with fasciitis nearly completely resolved with only a thin sliver of edema over the superficial fascia. On re-evaluation of the patient, he reported skin changes to his left upper arm at 20 mg of prednisone per day and skin changes to his left forearm at 7.5 mg per day. Examination of the upper arm revealed an atrophic patch with some overlying erythema and examination of the forearm showed indurated, bound-down, tense skin with a slight groove and minimal overlying hyperpigmentation. The rest of the dermatological examination was within normal limits. Skin biopsy revealed marked septal thickening with sclerosis, sparse lymphoplasmacytic infiltrate along the dermal subcutaneous junction, and swollen, homogenized collagen fibers with diminished spaces between the fibers (see Figure ). The clinical examination along with pathology revealing dermal and subcutaneous sclerosis was consistent with a diagnosis morphea profunda. Lyme serology was repeated, but Western blot for IgG was negative. A scleroderma antibody panel was negative as well. He was started on methotrexate and a higher dose of prednisone. |
A 60-year-old woman presented to the spine clinic for a complaint of progressive severe pain in her lower back of approximately 2.5 years’ duration. She previously underwent an L4-S1 TLIF for mobile spondylolisthesis and radiculopathy 3 years earlier at an outside institution. The patient reported significant improvement in her preoperative symptoms at that time, to the point where she was participating in physical therapy with minimal pain. At 6 months after the index surgery, she fell and reported hearing a distinct “pop” in her back. Since the fall, she remained bedridden with shooting, “shock-like” pain down her left leg associated with a progressively worsening left-sided foot drop (muscle strength 2/5) but with intact genitourinary function and perineal sensation. She was seen in our clinic 2.5 years after the index surgery for the progression of these symptoms. Radiological workup with radiographs and magnetic resonance imaging of the lumbar spine were notable for retropulsion of the L5-S1 interbody cage into the spinal canal, otherwise excellent positioning of the L4-5 interbody, and pedicle screws with posterolateral bridging bone [Figure and ]. These findings were stable over the previous 2.5 years based on radiographic studies obtained after her fall. The patient's original surgeon suggested revision surgery with the high risk of cerebrospinal fluid (CSF) leak and neurological deficit and the patient deferred a surgical option at that time. However, because of the her progression of her symptoms, she sought a second surgical opinion.\nConsidering the prolonged interval between the index surgery and the time of presentation to our clinic and the exact central position of the retropulsed interbody [], we were concerned that significant scar tissue and osteophytic ridge formation would hinder visualization of the graft and place the surrounding neural tissue at risk due to the retraction that would be necessary if the interbody were accessed via the original surgical corridor. In addition, the patient had adjacent segment degeneration and stenosis at the L3-4 segmental level, which explained her progression of symptoms. Our recommendation was a more direct transdural approach that would represent virgin territory, obviating scar tissue while providing adequate visualization with minimal retraction of neurological tissue during retrieval of the interbody. An anterior approach via anterior lumbar interbody fusions was also considered, but a posterior-only approach was recommended because of the presence of additional pathology at L3-4 that required treatment. After risks, benefits, and alternatives to intradural and transdural approaches were explained, the patient agreed to proceed with transdural removal of the retropulsed L5-S1 interbody, along with an L3-4 laminectomy and extension of her fixation from L3 to S1.\nThe prior surgical incision was opened, and surgical dissection was carried down to the existing instrumentation at L4-5 and L5-S1. After the locking caps on all six screwswere removed, the titanium rods were removed, and each screw was inspected for pseudarthrosis. Bilateral L4 and L5 screws were notably loose and promptly replaced with screws of larger diameter. An L3-4 laminectomy was performed, and L3 pedicle screws were placed. After this, we turned our attention toward the L5-S1 interspace, where the surgical corridor for the original TLIF was unidentifiable secondary to several large osteophytic ridges and extensive scar tissue. Residual lamina at this interspace was identified and removed easily with punch Kerrison rongeurs (Integra LifeSciences, Plainsboro, NJ) until normal midline dura was identified. The surgical microscope was brought in for the transdural portion of the operation. The thecal sac was opened with a number 11 blade in a linear fashion [], and after the egress of CSF, the dura was tacked to the muscle margins. With the retraction of intradural sacral roots [], the herniated interbody was easily visible and palpable through the ventral dura; it was compressing the left-sided exiting sacral rootlets. A Penfield number 2 was used to gently retract the nerve rootlets to either side, and a number 11 blade was once again used to open the ventral dura in a linear fashion directly over the herniated interbody [Figure and ]. Once open, the ventral dura was retracted to either side of the herniated interbody, and a graft inserter was threaded into the interbody []. Using repetitive rocking motions medially/laterally to disrupt any bridging osteophytes attached to the interbody, wecarefully shimmied the cage out through the ventral dura, traversing the thecal sac, and finally out through the opening in the dorsal dura []. The intradural nerve rootlets were inspected for any damage. The dura was closed in sequential fashion, ventrally then dorsally, using 5-0 Prolene sutures (Ethicon Endo-Surgery, Inc., Blue Ash, OH) and buttressed with DuraSeal (Covidien, Mansfield, MA) sealant []. Segmental fixation was completed with titanium rods locked in place from L3 to S1, along with autograft/allograft bone placed posterolaterally []. A lumbar drain was not placed, and the patient was kept flat for 24 h. A few hours postoperatively, the patient noted immediate improvement in her pain but had no improvement in her foot drop. She was up and mobilizing on postoperative day (POD) 2 with the physical therapy team, off all narcotics on POD 3, and discharged to rehabilitation on POD 4. At her 12-month follow-up visit, she was self-ambulatory, performing her normal activities of daily living, and had a complete resolution of pain and some improvement in foot drop (to muscle strength 3/5). |
A 42-year-old male, nonsmoker, with medical condition significant for hypertension presented to the emergency department after a fall followed by two episodes of seizures. On presentation physical examination was notable for altered level of consciousness and mild symmetrical decrease in power of 4/5 in all four limbs. Laboratory workup including complete blood count, electrolytes, coagulation panel, lipid profile, urine, and serum drug screen was unremarkable. CT scan head revealed a 1.5 cm left temporoparietal lobe intraparenchymal hemorrhage with surrounding edema as shown in (). As part of the diagnostic workup, an ECG was also performed on admission which was normal. The patient was admitted to the neurointensive care unit (NICU) for further management. A computerized tomography angiogram was performed, which showed early draining veins at the site of the lesion, suspicious for an underlying vascular malformation. Subsequently a cerebral angiogram was performed which confirmed the presence of an AVM underlying the hemorrhage (). A partial embolization of the AVM was performed, and the patient was boarded for surgical resection ().\nOn day 3 of admission, the patient complained of sudden-onset chest pain. He described it as left sided, retrosternal, sharp, nonradiating pain, worsened when lying down on left side, lasted 2-3 minutes and then resolved spontaneously. It did not recur however prompted an ECG which showed sinus rhythm with nonspecific ST segment elevation in leads V3-V6 (). Cardiology was consulted who deemed the ECG changes as J point elevation suggestive of benign early repolarization and not a true acute coronary event. A high sensitivity cardiac troponin assay done immediately and repeated two times at 6 hours and 12 hours from the onset of symptoms remained negative (<0.017 ng/ml; normal value <0.057 ng/ml). A transthoracic echocardiogram (TTE) performed later that day revealed no regional wall motion abnormalities or left ventricular dysfunction. The next day, patient was taken for craniotomy and surgical resection of the AVM (). The surgery was uneventful. A follow-up ECG on the postoperative day 1 revealed pronounced ST elevation with new T wave inversions (in leads V2-V6) highly suggestive of acute STEMI (). The patient was completely asymptomatic with no chest pain or other cardiac symptoms. Serial estimation of high sensitivity cardiac troponin was again negative (<0.017 ng/ml) and a repeat TTE was unremarkable. Given these findings and the absence of the symptoms, no intervention was done and he was monitored in the NICU.\nThe patient did not have any further untoward event(s) and continued to do well postoperatively with normalization of his ECG changes over the next 48 hours (). He improved neurologically and was transferred out of the ICU on day 7. He was subsequently discharged on day 12 with home health physical therapy, neurosurgery, and cardiology follow-up appointments. An exercise stress test was eventually performed 3 months' after discharge which did not reveal any evidence of coronary artery disease. |
A 59-day-old, 5.3 kg, male patient presented for general anesthesia for undergoing craniotomy and vertical parasagittal right hemispherotomy. He was normally delivered at 39 weeks of gestation with a birth weight of 3,180 g without any complications or abnormal findings. One week after birth, his seizures started with repetitive clustered tonic spasms for 30 min, at a frequency of 10 clusters a day. He was admitted at an outside hospital, and vigabatrin 250 mg and phenobarbital 20 mg were administered. Yet his seizures were refractory to anticonvulsants, so he was transferred to our hospital at the age of 30 days.\nThere were no abnormal physical findings and laboratory investigations. The electrocardiogram and chest X-ray were also normal. After admission, he had generalized tonic seizure with right-ward eyeball deviation, blinking and nystagmus at a frequency of 1-3 a day. Flexor spasms in clusters were also observed for 10 minutes, at a frequency of 7 clusters a day. His magnetic resonance imaging (MRI) showed cortical dysplasia at the right temporal and parietal lobes (). On the positron emission tomography (PET) study, abnormal high uptake for that age was discovered at the right cerebral hemisphere. His video EEG for 2 days demonstrated excessively asynchronous and discontinuous rhythms, the so called suppression-burst patterns, and this was consistent with OS during both the awake and asleep states (). He was diagnosed with OS and right hemispherotomy was planned for treatment.\nHe was transported to the operating room and the immediate vital signs revealed a blood pressure, pulse rate and oxygen saturation of 83/42 mmHg, 152 beats/min and 100%, respectively. Before induction, 0.02 mg of glycopyrrolate was injected intravenously. The patient was preoxygenated and general anesthesia was induced with thiopental sodium 25 mg and atracurium 3 mg. Mask ventilation was begun with 100% oxygen and there was no difficulty in ventilation. After confirming full relaxation, the patient's trachea was intubated with a 3.5-mm ID endotracheal tube and volume controlled ventilation was provided with a tidal volume of 45 ml. The respiratory rate was adjusted to maintain the end-tidal carbon dioxide (EtCO2) at 35-40 mmHg. Anesthesia was maintained with sevoflurane 2.5 vol%, FiO2 0.5 with air and 2 L/min of inspiratory fresh gas flow. A 24-G catheter was placed in the left radial artery for continuous BP monitoring and arterial sampling, and 20-G catheter was inserted into the right femoral vein.\nDuring surgery, the blood pressure, pulse rate and oxygen saturation were stable at 62-74/38-51 mmHg, 127-151 beats/min and 99-100%, respectively. The intra-operative arterial blood gas analysis was in the normal range, and the pH, partial pressure of arterial oxygen (PaO2) and partial pressure of arterial carbon dioxide (PaCO2) was 7.43, 173.2 mmHg and 33.1 mmHg, respectively. On completion of surgery, spontaneous respiration was fully restored by manual ventilation. Atropine 0.05 mg and neostigmine 0.1 mg were administrated to reverse the residual muscle relaxation. The endotracheal tube was removed after confirming that the patient was awake and the muscle function had been fully restored. The operation was uneventful. The patient was transported to the post-anesthetic care unit (PACU). The operation time was 6 h 45 min, and the duration of anesthesia was 7 h 55 min. The total amount of fluids administered was 400 ml of 1 : 4 dextrose solution and 500 ml of normal saline, and 200 ml of packed RBCs were also transfused. The total blood loss was 170 ml and the urine output was checked to be 280 ml. The preoperative level of hemoglobin was 9.0 g/dl, and the postoperative level was 10.5 g/dl.\nIn the PACU, the patient showed stable vital signs and a regular breathing pattern. His mentality was alert and there was no newly developed neurologic deficit. There were no complications, including hematoma or loss of distal pulsation on the sites of the radial arterial and femoral venous cannulation. He stayed for 50 min in the PACU and then he was moved to the neurologic intensive care unit. He had no seizure in the first 7 days after surgery and then he was discharged. During the 2 months after surgery, he had no seizure and he has been followed up at our hospital. |
The patient was a 74-year-old woman who was initially examined in neurosurgery for visual disturbance of the left eye. As computed tomography (CT) revealed a shadow in the sphenoid sinus, she was examined in the Department of Otolaryngology of Tokyo Medical University Hachioji Medical Center, revealing a polyp in the middle nasal meatus. Although a soft tissue shadow was observed in the left sphenoid sinus, no damage to the surrounding bone tissue was evident (). Preoperative visual acuity was 0.03. No signs of cranial nerve paresis were observed. On the same day, the left sphenoid sinus was opened under general anesthesia using an endoscope.\nMucosal hypertrophy was found, and the procedure was completed following removal of the mucosal lesion. Pathology specimens of the removed mucosal lesion were found to represent sinus mucosa with no neoplastic changes. As postoperative visual acuity only improved to 0.6, postoperative steroid therapy was initiated but did not improve vision. One month after surgery, visual disturbance of the right eye, headache, and ophthalmalgia appeared. Visual acuity was negative for light sense, and paresis of cranial nerve III was observed. CT showed soft tissue shadows in both sphenoid sinuses and the left posterior ethmoid sinus, accompanying skull base damage (). Using an endoscope, biopsy of the right sphenoid sinus was conducted under general anesthesia. Pathological examination revealed medium-sized, circular atypical cells manifesting amorphous medullary proliferation. Nuclei were deeply stained and frequently eccentrically located, cytoplasm was lightly eosinophilic, and cells with small amounts of brown pigment were evident in parts. Immunohistochemically, the tumor cells were positive for HMB-45 and melan A antibodies, and a very small number were positive for cytokeratin (MNF-116). On the basis of these findings, malignant melanoma was diagnosed (). Multiple metastases, to the spine and ischium, were observed on positron emission tomography-CT. As the patient did not desire active treatment such as chemotherapy, only palliative care was provided, and she died 3 months later. |
A 67-year-old man complained of left shoulder pain for 2 weeks without any history of trauma. Bloody effusions were aspirated 5 times at a local orthopedic clinic during a 2-week period before the patient was referred to us. He had no previous history related to hemorrhagic factors. On examination, he had slightly limited range of motion, with active forward flexion to 160°, abduction to 120°, external rotation to 30°, and internal rotation to the fifth lumbar spine with the arm at the side. Passive range of motion was the same as active range of motion. Neer's impingement test and Hawkins test were positive. The greater tuberosity was seen irregular on radiography. MRI demonstrated a hematoma in the anterior portion of the subdeltoid bursa and bony erosion of the lateral acromion, similar to case 1 ().\nArthroscopy was performed to determine the cause of the hemorrhage. A hematoma was not observed in the glenohumeral joint. The intra-articular portion of the long head of the biceps tendon had completely disappeared. A bursal-side partial tear of the rotator cuff was noted. The size of the tear was 25 mm in the anteroposterior direction. Crater formation on the undersurface of the acromion and irregularity of the greater tuberosity were observed, which seemed to be kissing lesions (Figures and ). Active bleeding was seen at the exposed bone marrow of the acromion when the pressure of irrigation was lowered. The undersurface of the acromion was coagulated and the greater tuberosity was abraded to avoid impingement (). Acromioplasty was not performed in this case. The bursal-side partial tear was not repaired at that time ().\nOne month later, the patient underwent arthroscopic rotator cuff repair. The patient regained full range of motion 3 months after rotator cuff repair and returned to work as a taxi driver. At the final follow-up, 14 months after the first arthroscopy, he had no functional deficit. The UCLA score was improved from 14 points preoperatively to 35 points at the final follow-up. Hemarthrosis did not recur in the 14 months postoperatively. |
A 39-year-old Yamani woman was referred to us for right-cheek DFSP. The lesion began as a small nodule 2 years previously and increased in size over time. Recently, the patient experienced itching over her face, particularly around the lesion, with no associated pain or trismus. The patient had a history of facial trauma 20 years back, but no history of radiation treatment and no significant medical history.\nPhysical examination revealed a nodular mass of 4 5 cm over the right cheek, surrounded by skin discoloration, and texture changes reaching up to the nasolabial fold anteriorly, lower eyelid superiorly and the level of the lower lip inferiorly. The nodule was hard and not fungating. Head-and-neck examinations including neck lymph nodes and cranial nerve assessments were unremarkable. All laboratory results were within normal ranges.\nComputed tomography (CT) revealed an exophytic skin lesion on the right cheek reaching the buccal space with maximum transverse, anteroposterior and craniocaudal dimensions of 31, 28, 30 mm, respectively. No bone invasion was noted. Magnetic resonance imaging (MRI) indicated that the mass extended to the anterior surface of the masseter muscle, with parotid duct medialization due to mass effect (). The referred histopathology slide of the incisional biopsy was reviewed, and a diagnosis of DFSP was confirmed.\nThe case was discussed with a multidisciplinary team, and treatment options were discussed with the patient. WLE with negative margins was planned as we do not have access to MMS. Unfortunately, intraoperatively, the zygomatic branch of the facial nerve was found to be involved and had to be excised. An intraoperative frozen section confirmed negative margins, and the resulting defect was reconstructed using a harvested left antro-lateral thigh (ALT) flap. An anastomosis to the facial artery was created () using the vein coupler for venous anastomosis. The donor site was closed primarily.\nHistopathological examination confirmed a DFSP of 3 2.5 2.5 cm in size with close margins of less than 2 cm and reaching the deep-inked margin. There was no evidence of perineural or lymphovascular invasion.\nThe flap completely healed by postoperative day 14 with good face contouring (). All branches of the facial nerve were intact except the zygomatic branch, which was excised during the surgery, with the loss of nasolabial fold at the site of surgical incision without any impact on the eye. The patient underwent adjuvant radiotherapy for the right cheek lesion (60 Gy in 30 fractions) and followed for 6 months. There was no effect on visual filed, acuity and eye movement. Furthermore, no recurrence or residual disease was detected by the head CT scan. As the patient has a higher risk of local recurrence, she will be followed every 3 months for physical examination and every 6 months for radiological evaluation for the first 2 years and then every 6 months for the next 3 years, followed by annually for life. |
The present case is about a 21-year-old female patient reported to the Department of Periodontics with the chief complaint of a swelling on her hard palate.\nThe lesion had grown rapidly to the present size over duration of 2 weeks. The growth was asymptomatic except for the uneasiness which the patient mentioned. The patient's medical history was non-contributory and she did not give any history of intake of any medications.\nClinical examination revealed a solitary, exophytic, pedunculated, spherical-shaped, reddish pink swelling with distinct borders and irregular surface []. Adjacent palatal mucosa was normal. Swelling was located in the anterior part of the hard palate just posterior to the central incisors measuring 1.5 cm × 1.5 cm in size. On palpation, the swelling was non-tender, soft to firm in consistency, and blanched on pressure.\nOcclusal and intra oral periapical radiograph showed no loss of bone in relation to the lesion [Figures and ]. Based on the clinical signs and symptoms, a provisional diagnosis of pyogenic granuloma was established and capillary hemangioma was kept as differential diagnosis. The treatment was planned under local anesthesia with all necessary emergency equipment's at hand under the guidance of a trained anesthetist, keeping in mind the differential diagnosis of capillary hemangioma.\nThe lesion was excised in toto using electrosurgery. Periodontal dressing was placed on the operated area, and the patient was given post-operative instructions. After 1 week, the dressing was removed. The healing was uneventful [].\nThe excised tissue was fixed in 10% neutral buffered formalin and was sent for routine hematoxylin and eosin (H and E) staining. The histopathological section of the specimen stained with H and E showed a polypoidal structure lined by stratified squamous epithelium with focal areas of ulceration. There were underlying numerous vascular channels lined by endothelial cells admixed with varying number of inflammatory cells. Focal areas of hemorrhage were also evident [Figures and ]. The connective tissue revealed numerous blood capillaries of varying caliber with plump and proliferating endothelial cells. The endothelial cells depicted varying degrees of atypia. The overall features were suggestive of capillary hemangioma. |
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