Journal of Indian Association of Pediatric Surgeons最新文献

Esophageal stenosis is an uncommon clinical condition defined as a fixed narrowing of the esophagus. It can be congenital or acquired. The incidence of congenital esophageal stenosis (CES) is 1 in 25,000-50,000 live births. Most of these patients present in early infancy and many of them require surgical intervention. We report a very interesting case of a 4-month-old child with esophageal stenosis involving two different segments of the esophagus who was successfully treated surgically. This is one of the rarest variants of CES which involves two different segments of the esophagus.

Neuroblastoma (NB) tumor rupture, typically intra-abdominal, is a rare and life-threatening condition with a poor prognosis, often associated with large aggressive tumors. Intrathoracic ruptures, rarely reported, may result from thoracic NB or malignant invasion. We present the first case of spontaneous intrathoracic rupture in a 2-year-old boy with abdominal NB.

Prolonged mechanical ventilation following cardiopulmonary and neurological events oftentimes necessitates a tracheostomy and tracheal granulation is one of its most common late complications. The literature recommends that large granulation be managed through surgical excision or endoluminal techniques. A 6-year-old boy presented with high-grade tracheal stenosis secondary to endotracheal cuff-related injury and prolonged tracheostomy. We present an unconventional yet successful method of management that included multiple sittings of endoscopic rigid bronchoscopic dilation and endoluminal cryotherapy.

Primitive myxoid mesenchymal tumor of infancy (PMMTI) is a low to intermediate-grade, poorly differentiated myofibroblastic tumor and is characterized by its tendency to recur locally. It commonly occurs in the 1^st year of life and is predominantly seen in the axial skeleton and rarely in the retroperitoneum. We report one such case of PMMTI, which is the second case reported in English literature.

We present a rare case of a 17-month-old child diagnosed with MS-stage neuroblastoma and associated chronic diarrhea due to elevated vasoactive intestinal peptide (VIP) levels. The unusual occurrence of a VIP-secreting tumor in a patient with MS neuroblastoma is a rare combination, not previously reported in the literature. The patient underwent exploratory laparotomy and excision of the tumor, leading to a significant decline in VIP levels and resolution of symptoms.

Background: Technical and immunological advancements have improved patient and graft survival rates in pediatric kidney transplantation (PKT). However, the landscape of PKT in India remains underexplored. We conducted a questionnaire-based survey among Indian pediatric surgeons to delineate contemporary trends in PKT.

Methods: The online survey comprised 10 questions assessing pediatric surgeons' involvement in PKT, existing challenges, and educational needs.

Results: Of 610 surveyed pediatric surgeons, 120 responded (19.67% response rate). Majority of the respondents were affiliated with teaching programs, either in the public sector (61.18%) or in corporate setups (25.21%). While 65.83% of respondents managed pediatric patients with chronic kidney disease, only 10% had PKT programs at their institutes. Most of the respondents (67.5%) believed that pediatric surgeons should perform PKTs. Despite this, only 15.83% were part of the renal transplantation team at their institute and only 2.5% routinely performed PKTs. Most of the respondents (63.33%) advocated for mandatory KT exposure during postdoctoral training.

Conclusion: Despite enthusiasm among pediatric surgeons, actual involvement remains low. Dedicated PKT programs are necessary to address these challenges along with improved training by integrating transplantation sciences into the pediatric surgical curriculum. The study underscores the need for pediatric surgeons to specialize in transplantation and advocates for policy changes to address the prevailing challenges.

Background: Intranodal hemangiomas are rare benign vascular tumors of the lymph nodes, often misdiagnosed as malignant lymphadenopathies due to their clinical and radiological features. This case report and systematic review aim to elucidate the epidemiology, clinical profile, and therapeutic interventions for intranodal hemangioma, enhancing diagnostic accuracy and management.

Methods: A systematic review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Searches were conducted in PubMed, Scopus, Web of Science, and Google Scholar up to March 15, 2024, using keywords like "intranodal hemangioma," "primary intranodal hemangioma," "nodal hemangioma," "vascular tumor," and "lymph node hemangioma." Inclusion criteria included studies on clinical presentation, diagnostic strategies, and therapeutic interventions for intranodal hemangioma in patients of all ages. Excluded were reviews, opinion pieces, non-English studies, cases of hemangioendothelioma, sclerosing hemangioma, and animal studies. Key aspects analyzed included demographics, clinical presentation, management strategies, and histopathological findings.

Results: Our case involves a 3-year-old female with a submandibular mass, initially diagnosed with hemangioma. After no response to beta-blocker therapy and conflicting imaging results, surgical excision was performed. Histopathology confirmed intranodal hemangioma. From 233 records, 19 publications (36 patients, including our case) met the inclusion criteria. The review shows common clinical presentations as painless, slowly enlarging lymph nodes, primarily in the cervical and axillary regions. Diagnostic challenges often require imaging and biopsy to differentiate from malignancies.

Conclusion: Recognizing intranodal hemangiomas, particularly in childhood, is crucial to prevent misdiagnosis and inappropriate treatment. The rarity of these tumors in childhood and their clinical presentation similar to malignancies in older patients make diagnosis challenging without widespread awareness. Surgical excision remains the mainstay of both diagnosis and treatment, often leading to favorable outcomes.

Background: A discrepancy between the true and measured value of stretched penile length (SPL) may be a result of errors that can either be systematic or random. Hence, it becomes important to focus on the quality of measurements to prevent any iatrogenic harm to the patients.

Objective: The objective of this study was to assess the magnitude of intra- and interobserver variations in the measurement of SPL with the SPLINT technique.

Materials and methods: SPL was measured prospectively in a cohort of 449 boys aged 0-14 years including 68 infants (substratified into Group I: >4 years, Group II: 4-8 years, and Group III: >8 years) with the SPLINT technique by expert (E: E1 and E2) and trainee (T: T1 and T2) surgeons after completing a three-tiered training module. Intra- and interobserver variability was assessed through descriptive statistics, intraclass correlation (ICC), relative technical error of measurement (rTEM), and reliability or R (%).

Results: Intraobserver variability: the mean difference between the two readings (E1 and E2) is 0.08 cm (95% confidence interval [CI]: 0.073-0.087), ICC was 0.998 (95% CI: 0.997-0.998), and intraobserver variability ≤0.1 cm in 85% of the participants (n = 370 of 433). The rTEM and reliability (%) were 1.82% and 98.1% (Group I), 1.65% and 98.9% (Group II), and 1.09% and 99.7% (Group III), respectively. The intraobserver variability was observed to be inversely proportional to the age of the participants (correlation coefficient = -0.56). Interobserver variability was calculated separately for expert versus trainee and trainee versus trainee (T-vs-T) measurements. For expert versus expert, ICC, rTEM, and reliability (%) were 0.984, 2.4%, and 96.8% (Group 1), 0.992, 2.07%, and 98.3% (Group 2), and 0.997, 1.38%, and 99.05% (Group 3), respectively. A similar pattern of variability was observed for T-vs-T measurements. The reliability (%) of the SPL by experts is consistently more than that of trainees across all age groups; however, the difference ameliorates with the age of participant.

Conclusions: The study has validated the SPLINT technique by demonstrating a high level of intra- and interobserver reliability. The adequacy of the training modules for SPL measurements described in this study has also been established. Evidence that the SPL can be used as an objective marker of penile dimensions is herewith furnished.

Morel-Lavallée lesion, also called closed degloving injury, is a rare clinical entity in routine pediatric surgical practice. We present a case of an 11-year-old girl presenting with a left hip lesion, treated with compression bandage, and the clinical outcome was favorable.