Malignant hyperthermia in a paediatric patient; managed successfully without dantrolene: a case report.

Introduction: Malignant hyperthermia (MH) is a potentially life-threatening pharmacogenetic syndrome triggered by volatile anaesthetics, succinylcholine, and stress such as vigorous exercise. Case presentation: The authors describe a case of an 8-year-old male who presented with classical symptoms of MH after induction of general anaesthesia and symptomatic treatment was done successfully due to the unavailability of Dantrolene. Discussion: Definitive diagnosis of MH can be done based on a contracture test in fresh muscle biopsy in the presence of halothane and caffeine. In the absence of muscle biopsy and genetic testing, diagnosis for MH can be done based on MH scoring. Conclusion: Anesthesiologists should be made aware that proper symptomatic management can also save the life of a patient. Also, strong advocacy should be done to ensure the availability of Dantrolene and further strengthen lab facilities to confirm diagnosis to facilitate diagnosis and management in the future.

Histopathological Insight of a Case of Adenoid Ameloblastoma: A Rare Odontogenic Tumor.

Adenoid ameloblastoma with dentinoid had been perceived as a rare odontogenic tumor with bridging histopathological features between ameloblastoma and adenomatoid odontogenic tumor. Due to the mixture of histopathological features of two separate and well-recognized entities, adenoid ameloblastoma was also regarded as a hybrid lesion. The diversity in the histopathological presentation among the cases has disaccorded the nature, behaviour, and prognosis of this pathology. Despite the literature acknowledging the histopathological diversity, categorizing all these variations into one and addressing them as a single entity was lagging till the 5th edition of the odontogenic tumor classification by the WHO was forwarded. With the establishment of the new terminology of adenoid ameloblastoma and the enlistment of its diagnostic criteria, the scientific literature has advocated updating, contributing, and redefining the various aspects of this pathology. Here, we present a case of a 34-year-old male who presented with a chief complaint of swelling in the lower front region of his jaw in the past one month. The swelling was associated with pain that was sudden in onset with a progressive increase in size. The swelling was also associated with discharge that resembled pus. A panoramic radiograph showed a mixed radiopaque and radiolucent area, extending from the distal aspect of 32 up to the distal aspect of 43. The entire cystic lining along with the growth was excised and sent for histopathological examination. Correlating clinically, the histopathological features are suggestive of adenoid ameloblastoma. Scientific literature has stood as a boon to evidence-based practice. The diagnosis for the present case report is truly an outcome of the literature-based update which helped the diagnosis of the case as a separate entity rather than as a hybrid pathology. The goal was to enhance the understanding of the lesions in terms of their clinical characteristics and diverse histopathological morphology.

Evaluation of variations of optic nerve course in relation to posterior paranasal sinuses in MDCT in a tertiary care center of Nepal: a retrospective cross-section study.

Background: The proximity of optic nerves to the posterior paranasal sinuses (PNS) is a critical consideration in preventing optic nerve injuries during functional endoscopic sinus surgery. Methods: A retrospective cross-sectional study was conducted on 367 patients aged 13 years and above. Four radiologists, each with 2-6 years of experience, evaluated computed tomography scans of the PNS and the head of these patients. The optic nerves were classified into four types based on DeLano's classification, and their respective prevalence rates were determined. Additionally, the prevalence of optic nerve dehiscence and pneumatization of the anterior clinoid process was assessed. Results: A total of 734 optic nerves were evaluated and categorized into four groups as per DeLano's classification. The most common type was classified as type 1 optic nerve, representing 65.4% of all optic nerves. Types 2, 3, and 4 optic nerves accounted for 16.9, 8.6, and 9.1%, respectively. The prevalence rates of type 1, type 2, type 3, and type 4 optic nerves were 76.6, 24.5, 12.3, and 14.4%, respectively. Optic nerve dehiscence was observed in 14.3% of cases, with a prevalence of 21.2% in the study. Type 3 optic nerves were most commonly associated with dehiscence, with 49.2% of them showing dehiscence in their course through the ethmoid or sphenoid sinus. The prevalence of pneumatization of the anterior clinoid process in the study population was 28.3%, with type 3 optic nerves being the most frequently associated. Conclusion: An understanding of the relationship between optic nerves and posterior PNS, as well as awareness of optic nerve dehiscence, is essential in preventing injuries during sinus surgeries. Type 1 optic nerve predominates among Nepalese patients visiting our hospital.

Retroperitoneal Castleman's disease in a young Nepalese girl: A rare cause of childhood abdominal mass.

Introduction: Castleman's disease (CD), or benign angio-follicular lymph node hyperplasia, is an uncommon condition in childhood. When a child presents with a huge retroperitoneal mass and inconclusive findings on clinico-radiological evaluation or tissue sampling, management becomes exceedingly difficult. CD herein becomes an uncommon presentation of an uncommon diagnosis. Case presentation: A six-year-old girl with no past medical problems presented to the office with a slowly progressive, painless mass over the right lumbar region for a year. Abdominal ultrasound showed a well-defined oval mass in the right periumbilical region, further evaluation of which with a computed tomography scan suggested lymphoma. A preoperative core-cut biopsy could not confirm the findings and suggested a neoplastic lesion, probably an inflammatory myofibroblastic tumour or small round cell tumour. She underwent an exploratory laparotomy with in-toto excision of the mass. Intraoperatively, a solid retroperitoneal tumour measuring 8×8×6 cm was found. Histopathology and immunohistochemistry confirmed a unicentric CD of the hyaline-vascular type. At two years of follow-up, she remained asymptomatic and disease-free. Conclusion: While CD in children is rare, retroperitoneal localization of the same can further add to the diagnostic conundrum. However, if carefully considered, an en-bloc surgical resection offers complete treatment.

Quantifying the potential epidemiological impact of a 2-year active case finding for tuberculosis in rural Nepal: a model-based analysis.

OBJECTIVES: Active case finding (ACF) is an important tuberculosis (TB) intervention in high-burden settings. However, empirical evidence garnered from field data has been equivocal about the long-term community-level impact, and more data at a finer geographic scale and data-informed methods to quantify their impact are necessary. METHODS: Using village development committee (VDC)-level data on TB notification and demography between 2016 and 2017 in four southern districts of Nepal, where ACF activities were implemented as a part of the IMPACT-TB study between 2017 and 2019, we developed VDC-level transmission models of TB and ACF. Using these models and ACF yield data collected in the study, we estimated the potential epidemiological impact of IMPACT-TB ACF and compared its efficiency across VDCs in each district. RESULTS: Cases were found in the majority of VDCs during IMPACT-TB ACF, but the number of cases detected within VDCs correlated weakly with historic case notification rates. We projected that this ACF intervention would reduce the TB incidence rate by 14% (12-16) in Chitwan, 8.6% (7.3-9.7) in Dhanusha, 8.3% (7.3-9.2) in Mahottari and 3% (2.5-3.2) in Makwanpur. Over the next 10 years, we projected that this intervention would avert 987 (746-1282), 422 (304-571), 598 (450-782) and 197 (172-240) cases in Chitwan, Dhanusha, Mahottari and Makwanpur, respectively. There was substantial variation in the efficiency of ACF across VDCs: there was up to twofold difference in the number of cases averted in the 10 years per case detected. CONCLUSION: ACF data confirm that TB is widely prevalent, including in VDCs with relatively low reporting rates. Although ACF is a highly efficient component of TB control, its impact can vary substantially at local levels and must be combined with other interventions to alter TB epidemiology significantly.

Von Hippel Lindau Disease with central nervous system and multiple visceral manifestations: A case report.

Von Hippel-Lindau (VHL) disease is an autosomal-dominant syndrome caused by mutations in the VHL gene, located on the short arm of chromosome 3. Patients with VHL are likely to manifest with a spectrum of multiple benign and malignant tumors involving various organ systems. We present a case of a 28-year-old female without a remarkable family history who presented with complaints of hematuria and abdominal discomfort. Initial laboratory investigations confirmed hematuria. Subsequent abdominal computed tomography scan revealed heterogeneous enhancing solid mass in bilateral kidneys, avidly enhancing mass in the right adrenal gland, bilateral simple renal cortical cysts, and a pancreatic cyst. With a provisional diagnosis of VHL disease, an MRI of the brain and spine was performed, which showed the presence of a cerebellar hemangioblastoma. Her catecholamine and vanillylmandelic acid levels were in the normal range not in line with pheochromocytoma. The patient then underwent bilateral partial renal nephrectomy and right adrenalectomy. Histopathologic examination reported clear renal cell carcinoma and pheochromocytoma of the right adrenal gland mass. Molecular genetic testing confirmed the presence of VHL disease.

Infantile choledochal cyst presenting with an epigastric bilioma: An iceberg phenomenon.

INTRODUCTION: Choledochal cysts (CC) are rare congenital biliary dilatations that are capable of presenting with a gamut of clinical findings. Perforation, a rather rare presentation, can account for 1.8-7% of cases. In an infant with non-specific abdominal complaints and a vaguely palpable upper abdominal mass, a perforated CC may be overlooked, leading to a delayed or misdiagnosis and a detrimental outcome. CASE PRESENTATION: We describe a 10-month-old girl who presented to the office with upper abdominal pain and associated fullness for two weeks. An abdominal ultrasonogram revealed perihepatic collections and an evident dilatation of the common bile duct. The bile aspirated from the collections prompted pigtail drainage as an emergency. Magnetic resonance cholangiopancreatography later revealed a spontaneously perforated CC with residual bilioma. Elective resection after six weeks, followed by bilio-enteric reconstruction, resulted in an uneventful recovery. CLINICAL DISCUSSION: Biliary peritonitis and localized biliomas are rare forms among the wide range of findings that can accompany CC. Satisfactory outcomes can be achieved with a two-stage procedure that entails biliary drainage with sepsis control, followed by resection of the CC and bilio-enteric reconstruction a few weeks later. CONCLUSION: It is important to be aware of the spectrum of findings that CC may present. We discussed our experience successfully treating a perforated type IVa CC.

A cystic non-Wilms renal tumor in a Nepalese boy: A rare case of clear cell sarcoma.

INTRODUCTION: Clear cell sarcoma of the kidney (CCSK) is an uncommon childhood malignancy known for its aggressive behavior and tendency to recur and metastasize to the brain and bones. CASE PRESENTATION: We report a four-year-old boy evaluated for non-specific abdominal pain with an incidental radiological finding of a right lower pole cystic renal mass initially thought to be cystic Wilms' tumor. A pretherapy core biopsy of the mass suggested a clear cell tumor. An open transperitoneal radical nephroureterectomy with aortocaval lymph node sampling was done. Based on histopathological findings and immunohistochemical analysis, CCSK was diagnosed. Appropriate chemotherapy and radiotherapy were instituted postoperatively. At six years follow-up, he was tumor-free and doing well. CLINICAL DISCUSSION: The workup for CCSK includes diagnostic and metastatic imaging, histopathology, and immunohistochemistry analysis. Diagnostic segregation of CCSK and Wilms' tumor is imperative to institute optimal oncological management and improve overall treatment outcomes. CONCLUSION: Considering the age of presentation and clinico-radiological appearance, CCSK can be mistaken for Wilms' tumor, which is much more common in occurrence despite the distinguishable histopathological features, treatment modalities, and prognosis.

Histopathological Characterization of a Series of Oral Basaloid Squamous Cell Carcinoma.

Basaloid squamous cell carcinoma (BSCC) is a rare, distinctive, and aggressive variant of squamous cell carcinoma (SCC) primarily seen in the upper aerodigestive tract with epiglottis, soft palate, and base of the tongue being site of high preference in head and neck region. It differs from conventional SCC histologically and immunologically, is most frequently found in males in their sixth and seventh decades, and is frequently linked to alcohol and tobacco use. High stage disease with distant metastases, a high recurrence rate, and a dismal prognosis is how BSCC typically manifests. In the present article, we report four cases of BSCC.

Transanal minimally invasive surgery - A single-center experience.

Background: Transanal minimally invasive surgery (TAMIS) was described in the literature 10 years ago. This procedure requires laparoscopic technical skills. It has been well accepted widely worldwide. TAMIS has been applied to multiple procedures, including excision for rectal polyps and cancer, with acceptable outcomes. The study aimed to assess the outcomes of TAMIS in a large district general hospital. Methodology: A retrospective study on prospectively collected data on 52 consecutive patients of TAMIS performed in a single unit was conducted between May 2014 and February 2020. Data were collected on patient demographics, clinical diagnosis, peri-operative findings, pathological findings, adequacy of excision and complications. Patients were followed up as per the trust and national post-polypectomy guidelines. Results: Among the 52 patients, TAMIS procedures were completed in 50 patients, of which 31 were female. The procedure was successful in 96.5% but had to abandon in two cases. There was no conversion to another procedure. Pre-operative indications were rectal polyps and one case was an emergency TAMIS in a patient who was bleeding following incomplete colonoscopic polypectomy. The final histology reported that the majority were benign polyps (46), and only 11 cases were malignant. The median distance of the lesion from the anal verge was 6 cm (3-10 cm). The median operative time was 55 min (8-175 min). A total of 45 (77.5%) lesions were completely excised and had negative microscopic margins. Most patients (64%) were discharged home the same day. No complications were observed at a median follow-up of 20 months (6-48 months). There was no mortality. Conclusions: Our data suggest that TAMIS can be safely performed in a district general hospital for both benign and early rectal cancer. TAMIS was also able to control post-polypectomy bleeding and completion of rectal polypectomy. In selected cases, day-case TAMIS is safe and feasible.

Blind loop mucocele of a side-to-end colorectal anastomosis as a rare cause of large bowel obstruction.

Large bowel obstruction (LBO) after colorectal surgery draws wide differentials. To our knowledge, LBO due to blind colonic limb mucocele of a side-to-end colorectal anastomosis has not yet been described. We report a man in his late 50s presenting with pain, abdominal distension and constipation. He had extensive surgical history; notably, a side-to-end colorectal anastomosis was fashioned following Hartmann-type colostomy reversal. CT and MRI suggested a mucus-filled short blind colonic segment compressing the anastomotic site and causing LBO. Flexible sigmoidoscopy under general anaesthesia showed external rectal compression and lumen narrowing. Transrectal needle aspiration of the blind segment yielded 145 mL of mucoid fluid. The patient's symptoms improved and he was discharged with outpatient Gastrografin enema and flexible sigmoidoscopy which confirmed successful blind segment emptying. This case highlights that blind colonic loop mucoceles in colorectal anastomosis can rarely cause obstruction, and endoscopic management is feasible when accurate diagnosis is confirmed on imaging.

The stone beneath my skin: Pilomatricoma in a young Nepalese girl.

Introduction: Pilomatricoma is a rare and benign tumor affecting children and adolescents. It originates from the matrix cells of hair follicles, the usual sites being head-neck and upper extremities. Due to its rarity, it is often misdiagnosed delaying definitive treatment. We report a case of pilomatricoma over the left gluteal region in a young Nepalese girl that was initially thought to be a calcified granuloma. Case presentation: A six-year-old girl presented with a painful swelling over the left buttock for one year that was gradually increasing in size. On examination, a solitary, well-circumscribed, tender swelling with hard consistency and a bumpy irregular surface measuring 3 × 2 cm was noted over the subcutaneous plane of the left gluteal region. Surgical excision of the mass was done which demonstrated features of pilomatricoma on histopathological examination (HPE). She recovered and remained disease-free at one year follow-up. Conclusion: This case highlights one of the handful presentations of pilomatricoma involving the buttock. Pilomatricoma is rarely considered a differential diagnosis of benign masses, the diagnosis of which is ascertained mostly after an HPE of the excised specimen. Surgical excision with clear margins is not only diagnostic but therapeutic in most situations.

Occurrence of osmotic demyelination syndrome in diabetes mellitus: A case report and literature review of various etiologies for osmotic demyelination syndrome.

Osmotic demyelination syndrome is a rare condition reported mainly in the case of rapid correction of hyponatremia, but it can occur even in the case of complicated diabetes mellitus either during rapid correction of hyperglycemia or anytime during the complicated diabetes mellitus. We report a case of complicated diabetes mellitus developing osmotic demyelination syndrome. The patient had presented with altered sensorium and seizure, which was initially diagnosed as hyperglycemia, but during his treatment, the magnetic resonance imaging of brain revealed central pontine myelinolysis. Our search on the causes of osmotic demyelination syndrome other than rapid correction of hyponatremia has revealed several other causes like autoimmune liver disease, Sjogren's syndrome and non-Hodgkin's lymphoma in addition to diabetes mellitus.

Guillain-Barre Syndrome following lower segment caesarean section under spinal anesthesia: A case report.

Symptoms of Guillain-Barre Syndrome (GBS) may be mistaken for typical puerperal changes, delaying diagnosis. Surgery and anesthesia may be triggers for GBS with an overall increase in pro-inflammatory cytokines in the postpartum period. We report a unique case of GBS in the postpartum period who made a good recovery with supportive measures.

Breast abscess in two Nepalese newborns: An unusual series.

Background: Breast abscess in newborns is an exceedingly rare pyogenic inflammation that usually starts as mastitis neonatorum. Although mastitis can respond to antibiotic therapy in the initial stages, once advanced into a purulent collection, decompression may be indicated either in the form of needle aspiration or even surgical drainage. We present two newborns with breast abscesses managed surgically with successful outcomes. Case presentation: Case 1: A 13-day-old boy presented with swelling and redness over the left breast for a day. Local examination revealed a warm, indurated swelling with redness and fluctuation. Needle aspiration confirmed pus. Intravenous (IV) antibiotics were started right away followed by pus drainage, the culture of which yielded Methicillin-Sensitive Staphylococcus aureus (MSSA). Regular wound care subsequently resulted in complete recovery.Case 2: A 15-day-old boy presented with swelling, redness and milky discharge from the right breast for two days. The swelling was erythematous, tender and indurated but not cystic or fluctuant. Ultrasonogram was suggestive of an abscess. Once again, IV antibiotics were administered followed by drainage. Pus yielded MSSA. Following regular wound dressings, satisfactory healing was achieved. Conclusion: Neonatal mastitis and breast abscess are uncommon. Early recognition followed by appropriate antibiotic therapy and drainage of the abscess is the mainstay of treatment.

Efficacy of Curcumin in Combination with Intralesional Dexamethasone with Hyaluronidase in the Treatment of Oral Submucous Fibrosis: A Randomized Controlled Trial.

OBJECTIVE: The objective of this study was to determine the efficacy of curcumin in combination with  intralesional dexamethasone with hyaluronidase in the treatment of oral submucous fibrosis (OSF). METHODS: This randomized, double blind, parallel design, clinical trial was conducted at B.P. Koirala Institute of Health Sciences, Nepal. Thirty-four patients with clinically diagnosed OSF were randomized into two groups (17 participants in each) with baseline treatment of intralesional dexamethasone with hyaluronidase for 6 weeks for the both. Curcumin (2gm/day) was provided to Group A (Test) and Group B (Control) received placebo. Interincisal mouth opening, tongue protrusion, cheek flexibility and visual analogue scale (VAS) scoring of burning sensation of oral mucosa was recorded at baseline, 6, 8 and 12 weeks follow-up and independent t-test was used to compare the improvements in two groups. RESULTS: On comparing the 6 weeks and baseline values, in Group A and B the mean difference in mouth opening was 8.82±1.33 mm and 5.53±1.17 mm respectively (p<0.001), in cheek flexibility was 2.94±1.02 mm and 1.94±1.24 mm respectively (p=0.02) and in tongue protrusion was 6.23±1.48 and 3.65±1.37 mm respectively (p<0.001). The findings were consistent in the 8 weeks follow-up. In 12 weeks follow-up, on comparing with the baseline values, in Group A and B, the mean difference in mouth opening was 8.71±1.16 mm and 5.35±1.22 mm respectively (<0.001), ), in cheek flexibility was 2.81±1.01 mm and 1.76±1.35 mm respectively (p=0.02) and in tongue protrusion was 6.06±1.48 and 3.35±1.50 mm respectively (p<0.001). Both the arms showed 100% improvement in burning sensation in 6, 8 and 12 weeks follow-up. CONCLUSION: Curcumin in combination with intralesional dexamethasone with hyaluronidase is efficacious in the treatment of OSF.

Rare case of pernicious anaemia from a university hospital of Nepal: A case report.

Introduction: Pernicious Anaemia is a rare autoimmune disorder prevalent among 0.1% of the general population and is characterised by decreased cobalamin absorption. This condition is overlooked because of its rarity, insidious onset of non-specific symptoms and clinically asymptomatic state. Elevated serum intrinsic factor antibody level along with reduced Vitamin B12 level confirms the diagnosis. Case presentation: Pallor and abdominal tenderness was present. Haematological investigations showed elevated platelet count, elevated Mean Cell Volume reduced haemoglobin level(11.4 g/dl), reduced Vitamin B12 and high serum intrinsic factor antibody level. Serum parietal cell antibody was positive. The patient responded well to parenteral Vitamin B12. Discussion: In Pernicious anaemia, serum intrinsic factor antibody and parietal cell antibody are high which are responsible for reduced Vitamin B12 absorption. Studies have also shown positive correlation between H pylori and Pernicious Anaemia. Neurological symptoms are less common but may present as paraesthesia, changes in gait or spasticity due to peripheral neuropathy. It is also associated with autoimmune diseases. Untreated pernicious anaemia can lead to neurological and gastrointestinal complications. Conclusion: Pernicious Anaemia is an overlooked condition because of its insidious onset of non-specific symptoms, clinically asymptomatic state, rarity and therefore timely diagnosis of Pernicious Anaemia still remains a challenge.

Thyrotoxic periodic paralysis in a patient with Graves' disease: A case report.

Introduction and importance: Thyrotoxic periodic paralysis (TPP) is a rare and often misdiagnosed, hypokalemic periodic paralysis with features of mainly recurrent acute limb weakness with good treatment outcome if diagnosed early. Case presentation: We here report a case of a 25-year-old male with a history of recurrent bilateral upper and lower limbs weakness resolved by potassium infusion later found to have Thyrotoxicosis (Graves' disease). MRI scans of the brain had no abnormal finding while thyroid scintigraphy showed diffuse toxic goiter. Clinical discussion: Graves' disease shares a majority of TPP while, other causes like toxic adenoma, thyroiditis, toxic multinodular goiter, amiodarone induced thyrotoxicosis, levothyroxine intoxication and thyrotropin (TSH) producing pituitary adenoma are also associated with TPP. The management of thyrotoxicosis by medical therapy, surgery or radioactive iodine therapy is the mainstay of treatment of TPP patients. For the treatment of acute attacks, potassium administration is necessary keeping in mind the problem of hyperkalemia because of excess doses of potassium as it shifts to extracellular space. Conclusion: TPP should be considered as a differential in the cases of limb weakness and the secondary causes especially Thyrotoxicosis and precipitating factors should be identified.

Cronkhite-Canada Syndrome: A Case Report.

Cronkhite-Canada Syndrome is a rare disease characterised by diffuse gastrointestinal polyposis, abdominal pain, diarrhoea, cutaneous and mucosal hyperpigmentation, alopecia, and onychodystrophy. Here we report a case of a 40-year-old female with Cronkhite-Canada Syndrome, who presented with the complaints of diffuse abdominal pain, blood mixed stools, and diarrhoea associated with tenesmus. She had nausea and reduced appetite and lost 10 kgs in 3 months. She had hair fall (alopecia), atrophic changes of nails (onychodystrophy), and hyperpigmentation of the skin in fingers, tongues, and lips. Histopathological biopsy of the gastric and colonic biopsy revealed polypoid edematous mucosa and the colonic biopsies showed scattered dilated glands with inflammatory exudate and mucin. She got Entamoeba histolytica and COVID-19. She received respective antibiotics and protein diets that helped relieve the symptoms. After 4 weeks of steroids, her symptoms improved drastically. Corticosteroids, treating co-infection along with nutritional counselling can be helpful to relieve the symptoms. Keywords: alopecia; case reports; cronkhite-canada syndrome; hyperpigmentation.