Ravi Prakash Kanojia

Rectal ectasia is a condition characterized by significant dilatation of the rectum. It can be either primary or secondary to distal obstruction. The literature does not clarify the embryo-pathogenesis, diagnostic criteria, investigation protocols, or management strategies. A key aspect of diagnosis is to exclude medical causes of constipation and Hirschsprung's disease. Surgical management involves various interventions, but insufficient data support the literature. Here, we describe our experience with two children diagnosed with rectal ectasia. We discuss the presentation, diagnosis, surgical intervention, complications, and the rationale for selecting one procedure over another while reviewing the relevant literature.

Purpose Biliary atresia (BA) is a critical pediatric condition requiring timely intervention through Kasai portoenterostomy (KPE), and up to two-thirds of patients need liver transplantation (LT). The outcomes for BA patients still need improvement in low- and middle-income countries. This study aims to assess the socioeconomic and geographical profile of BA patients in India, focusing on their compliance with follow-up care, treatment-seeking behaviour, and acceptability of LT in addition, it provides recommendations to overcome identified challenges. Methods A retrospective observational study was conducted in a tertiary care centre in India, including 48 patients who underwent KPE between 2018 and 2022. SES was assessed using the modified Kuppuswamy scale, and geographical areas were classified as per the criteria of the Indian Census 2011. Compliance with follow-up, treatment-seeking behaviour, and LT acceptability were evaluated. Results The majority of patients belonged to rural areas and lower middle SES. Compliance with follow-up decreased over time, with financial and geographical barriers being significant challenges. Only one patient underwent LT due to prohibitive costs and limited accessibility. Conclusion Socioeconomic and geographical factors significantly impact the clinical outcomes of BA patients in India. Improving education, healthcare infrastructure, and financial support is crucial for enhancing patient compliance and access to necessary treatments.

Introduction: Wilms tumor (WT) forms 95% of pediatric renal malignancy. Five-year survival rates have dramatically improved and now approach 90 percent. We conducted this research with the specific research question of the recurrence rate, survival rate post treatment and post-surgical complications in WT. Methods: This is a retrospective 10-year study done between January 2010 and August 2022. The study included all patients with WT who underwent treatment in The Royal Hospital, Oman; and had a minimum of one year of follow up. Patients with incomplete hospital records were excluded. Data was retrieved from the hospitals electronic system with ICD code. The retrieved master data sheet was analyzed for patients’ demographics and outcome parameters. Results: Out of the 47 cases, 40 were confirmed cases of Wilms tumor with predominance of males (62.5%). The mean age at presentation was 2.85±1.79 years. Abdominal lump was the most common presentation; Out of the total patients, nine had distant metastasis (most commonly the lungs). There were five recurrences in WT patients and two deaths. There was one case of secondary malignancy (synovial sarcoma). The post-operative complication rate is 17.5%. Conclusion: 5-year Overall Survival was 92.3% (95% CI: 82.1% -102.5%) and 5-year Event (recurrence) Free Survival was 82.1% (95% CI: 68.0% - 96.3%). Post-surgical complications were seen in 17.5% of the patients. There was recurrence in 12.5% of patients. The study provides data for WT in Oman and can be used for reference comparison done in future analysis and in policy making resource distribution and planning of oncology services.

Diagnosing, managing and assigning gender for different causes of ambiguous genitalia in children can be challenging. This article will discuss about the types, diagnosis and treatment of disorders of sex development including congenital adrenal hyperplasia, 46 XY mixed gonadal dysgenesis and 46XY ovotesticular disorder of sex development. This article reports about 3 cases of disorders of sex development. The first case is about a three-year-old girl who was diagnosed with congenital adrenal hyperplasia, genital examination revealed clitoromegaly and a single urogenital sinus; she was managed medically and surgically. The second case is about a one-month-old child with 46XY karyotype, genital examination revealed penoscrotal hypospadias with right palpable and left impalpable gonads. Patient underwent diagnostic laparoscopy in which both female and male internal organs were found; based on these results a diagnosis of 46XY ovotesticular disorder of sex development was made. The third case is about a 3-month-old child with 46XY karyotype, genital examination revealed hypospadias with bilateral impalpable gonads. Diagnostic laparoscopy showed a uterus with a bilateral ovary-looking gonad; histopathology of the bilateral ovary-looking gonads was consistent with testicular tissue. Based on the above, a diagnosis of 46XY mixed gonadal dysgenesis was made. Disorders of sex development are classified into three main categories based on the karyotype, XX, XY and sex chromosome other than XX and XY. Laboratory investigations, karyotype, genetic analysis, imaging, surgery and tissue biopsy, all aid in diagnosing, deciding gender identity and managing different types of disorders of sex development.

Background: Acute exogenous lipoid pneumonia is an extremely rare pathology in children and occurs because of aspiration of oil or lipid-containing material. The presentation and radiological findings vary widely and the exact mechanism of injury to the lungs is not clear. Case Presentation: An 18-month-old girl presented with gradually increasing swelling and subcutaneous emphysema over the chest wall, neck, and face. The investigation revealed pneumomediastinum, pneumorachis, and soft tissue density in the left bronchus. The child underwent rigid bronchoscopy and retrieval of an organic foreign body. Conclusion: Acute exogenous lipoid pneumonia can occur in children because of aspiration of oil-secreting organic foreign body. The presentation can be atypical, clinical suspicion and early diagnosis with timely intervention are crucial for the appropriate management.

Autops Case Rep, vol.12, e2021399, 2022