Challenges in the management of high-altitude illnesses and emergencies in Bhutan and Nepal.

Many popular tourist attractions and trekking routes in Bhutan and Nepal are situated between 3000 and 6000 m in elevation. High-altitude emergencies are becoming more common and medical providers must be aware of the practical and medical issues in managing these disorders. We reflect on the challenges in providing high-altitude emergency medical services in Bhutan and Nepal.

Rheumatic heart disease in school-attending Nepalese children: A descriptive analysis of the national heart screening database.

OBJECTIVE: Rheumatic Heart Disease (RHD) remains a significant public health problem with high morbidity and mortality in children and young adults from lower-middle income countries like Nepal. However, a nation-wide database of the disease is lacking for designing effective future prevention and control programmes and strategies. The aim of our study is to estimate the prevalence of RHD in school-attending Nepalese children. METHODS: We performed a cross-sectional descriptive analysis of a nationally representative database of Nepal Heart Foundation (NHF) national RHD screening programme which included school-attending Nepalese children between five and sixteen years of age. The screening was conducted between May 2015 and March 2020 in 236 schools, representing all seven provinces, across all three ecological zones of Nepal. Transthoracic two-dimensional echocardiography was performed in all eligible children with more than grade one murmur on cardiac auscultation. We estimated the prevalence of RHD among school-attending children as the number of RHD cases per 1000 school-attending children with a 95% confidence interval. RESULTS: The database included a total of 107,340 children who were screened clinically, of whom 10,600 (9·9%) underwent transthoracic two-dimensional echocardiography. The overall prevalence of RHD was 2.22 cases per 1000 school-attending children (95% CI:1·94 - 2·50). The highest prevalence was observed among children living in the southern Terai ecological zone (2·89 per 1000, 95% CI (2·32-3·46)) of Nepal. Among the provinces, Karnali had the highest prevalence of RHD (3·45 per 1000, 95% CI (2·42-4·48)). Among the districts screened, Kalikot had the highest RHD prevalence (5.47 per 1000, 95% CI (3.02-7.92)). CONCLUSION: Primordial, primary and secondary prevention programmes should pay special attention to southern Terai zone, particularly the under-privileged children from remote districts.

Large posterior mediastinal ganglioneuroma with intradural cervical spine extension: A rare case report and review of literature.

Introduction: Ganglioneuroma (GN) is a rare benign tumor of ganglion cell origin and can occur anywhere along the sympathetic chain. These tumors can grow to a significant size without any symptoms unless they exert a mass effect on the region they grow and start showing symptoms. Spinal extensions are rare and they may produce neurological symptoms warranting further investigation. Case presentation: We described a case of posterior mediastinal ganglioneuroma in a 4-year-old boy with cervical extension who presented with quadriparesis. The radiological scan revealed large ganglioneuroma having an intradural extramedullary extension with a large posterior mediastinal component compressing and displacing the surrounding structures. He underwent consecutive surgeries for complete excision of the tumor following which he regained his power in his upper and lower limbs over the period of 2 months. Conclusion: Para spinal mass with consistent radiological features suggests ganglioneuroma but confirmation should be done with biopsy. Complete excision of the tumor is the treatment of choice with close follow-up for clinical improvement and recurrence.

A rare case of Erdheim-chester disease reported from Nepal.

Introduction: Erdheim-Chester Disease (ECD) is a rare non-Langerhans cell histiocytosis with a propensity to involve multiple organs. Case presentation: We report a case of a patient in mid-60s with occipital headache and ataxia. Following the radiological and immunohistochemical investigations and genomic studies, a diagnosis of ECD was made with two intracerebral lesions. Brain lesions were resected and the patient was discharged with the medication Vemurafenib. After 3 years of diagnosis and 13 years of initial presentation, patient passed away. Discussion: ECD frequently presents with Diabetes Insipidus as initial presentation, long bone osteosclerosis as the most common presentation, and has multi-system predisposition. ECD can be differentiated from Langerhans Cell Histiocytosis (LCH) with immunohistochemistry images of the biopsy specimens. Further, with genomic analysis of ECD, the neoplastic nature has been highlighted and targeted therapies like Vemurafenib and Cobimetinib are shown to be effective. Conclusion: Good clinical judgement and supporting investigations can aid in diagnosing rare entities like ECD even in resource-limited settings.

Clinical spectrum and management of dystonia in patients with Japanese encephalitis: A systematic review.

BACKGROUND: Japanese encephalitis (JE) is a potentially fatal viral infection with a wide range of manifestations and can also present with a variety of movement disorders (MD) including dystonia. Dystonic features in JE are uncommon. Here, we have tried to summarize the clinical features and management of dystonia among JE patients with a comprehensive literature search. METHODS: Various databases, including PubMed, Embase, and Google Scholar, were searched against the predefined criteria using suitable keywords combination and boolean operations. Relevant information from observational and case studies was extracted according to the author, dystonic features, radiological changes in the brain scans, treatment options, and outcome wherever provided. RESULT: We identified 19 studies with a total of 1547 JE patients, the diagnosis of which was confirmed by IgM detection in serum and/or cerebrospinal fluid in the majority of the patients (88.62%). 234 (15.13%) of JE patients had dystonia with several types of focal dystonia being present in 131 (55.98%) either alone or in combination. Neuroimaging showed predominant involvement of thalami, basal ganglia, and brainstem. Oral medications including anticholinergics, GABA agonists, and benzodiazepines followed by botulinum toxin were the most common treatment modalities. CONCLUSION: Dystonia can be a disabling consequence of JE, and various available medical therapies can significantly improve the quality of life. Owing to insufficient studies on the assessment of dystonia associated with JE, longitudinal studies with a larger number of patients are warranted to further clarify the clinical course, treatment, and outcome of dystonia.

Living with a giant inguinoscrotal hernia for 35 years-a case report.

In this modern era, giant inguinoscrotal hernias are very rare to experience in a medical career. We discuss a case of a 65-year-old man with a history of an inguinoscrotal hernia with progressive growth for the past 35 years. On examination, he had a 20 cm × 15 cm non-reducible swelling with multiple ulcers over the skin surface extending to the mid-thigh with otherwise no other bladder and bowel complications. These large hernias pose a different set of surgical problems. Open surgery was performed, hernial sac opened, contents reverted and left orchidectomy were done with scrotal reconstruction. The defect was closed with Vicryl 1-0 over the muscle layer and the skin was stapled. Daily wound care was provided. Besides, this case also compels us to explore possible reasons for the occurrence of such potentially dangerous surgical problems in low-to-middle income countries (LMIC).

A rare case of Creutzfeldt-Jakob disease reported from Nepal.

Creutzfeldt-Jakob disease, though rare, should be considered in the clinical picture of rapidly progressive dementia and absence of verbal response as evident in our case despite the absence of typical radiological picture.

Penile strangulation with a plastic bottle neck: Intervened by an atypical instrument: A case report.

INTRODUCTION AND IMPORTANCE: Strangulation of penis, a surgical emergency, is often encountered in patients with psychiatric disorders and patients attempting to increase sexual arousal. Materials ranging from metallic to non-metallic like plastic bottles are used by the patients. The important factors to be considered for the successful treatment include the availability of instruments and the surgical skills of the doctor. CASE PRESENTATION: A 45 year-old man with a comorbidity of severe depression presented to Emergency with a two months long history of penile strangulation with a plastic bottle neck. The gross appearance of the penis showed edema and proliferative growths. He was intervened with a cable wire cutter as standard instrument failed to do so. The patient was discharged on the same day of intervention. However, he was lost to subsequent follow up. DISCUSSION: Penile strangulation which is common in people with mental disorders should be considered as a surgical emergency as it can present with devastating complications. No specific methods and tools have been recommended for the removal of those objects. The shame felt by patient is the root cause for late surgical consultation and are prone to develop complications. Simple instruments can be used for the intervention provided good surgical skills are demonstrated. CONCLUSION: Common in psychiatric patients who deny medical attention due to shame, penile strangulation should be intervened quickly and simple instruments not routinely used in surgical practice can be effectively used to remove the offending objects.