Journal of Indian Association of Pediatric Surgeons最新文献

Background: Fetus-in-fetu (FIF) is an exceedingly rare condition, characterized by a fetal-like or fetiform mass with a calcified vertebral axis surrounded by other organs or limbs. This systematic review was conducted to comprehensively analyze the clinicopathological attributes, management strategies, and prognosis of FIF to consolidate existing knowledge on FIF.

Methodology: Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, a comprehensive search was performed across various electronic databases, using the keywords "fetus-in-fetu," "fetus-ex-fetus," "homunculus," or "fetiform teratoma" to look for published articles until December 2023. Data extraction and analysis were carried out for cases meeting the defined criteria for FIF diagnosis, that is "presence of both/either the vertebral column and/or long bones of extremities" and "absence of immature elements."

Results: A total of 249 case reports/case series comprising 241 single FIF cases and 33 multiple FIF cases were included for analysis. The majority of single FIF cases presented within the 1^st year of life, with a slight male predominance. Retroperitoneal location was most common, and imaging modalities played a crucial role in preoperative diagnosis. Complete surgical removal of the mass with detailed histopathological examination is the cornerstone of treatment for FIF, with favorable outcomes in the majority of cases. Both mature and immature teratoma can rarely be found in association with FIF.

Conclusion: This comprehensive systematic review enhances understanding of FIF, emphasizing the importance of accurate diagnosis by diligent histopathological examination, appropriate management, and vigilant postoperative monitoring for favorable outcomes.

We report the case of a 4-year-old child with a rare solitary neurofibroma-schwannoma hybrid tumor in the brachial plexus, not associated with known syndromes. Surgical management through a transclavicular approach enabled complete tumor excision with favorable postoperative outcomes. Histopathology and immunohistochemistry confirmed the tumor's hybrid nature.

Objective: To identify the genetic markers of spina bifida through a systematic survey of the exome in an Indian cohort.

Materials and methods: Three consecutive patients (P1: 1 year, male; P2: 2.8 years, male; and P3: 10 years, female) with spina bifida (lumbosacral meningomyelocele) underwent whole-exome sequencing (libraries: SureSelect Human All Exon V8; sequencing: 2 * 150 bp paired-end run, 100×) with NovaSeq 6000. Data analysis was performed using SMART-One™ (secondary analysis) and SMARTer™ (tertiary analysis) for automated quality check, alignment (GRCh38/hg38), variant calling, annotation (ClinVar, OMIM, avsnp150, 1000 Genomes v5b, ExAC v0.3, gnomAD v4.0, and esp6500vi2all v0.0.25), v0.0.25), interpretation. The pathogenic and likely pathogenic (ClinVar/ InterVar), non-synonymous, exonic markers (read depth ≥ 5) were matched with the Familial Neural Tube Defects (Version 1.10) panel (FNTD panel).

Results: Pathogenic variants overlapping with the FNTD panel were MTRR, CC2D2A, and ZIC2 in P1 and P2, TGIF1 in P1 only, and none in P3. Novel pathogenic/likely pathogenic variants common to all three patients were PRUNE1, PKD1, PDZD2, and DAB2 in the homozygous state as well as in the heterozygous state, PLK1 and NLGN2. The possible role of such markers in etiopathogenesis was explored through a literatur search.

Conclusions: The genetic landscape of the spina bifida in an Indian cohort is diverse compared to that reported from other parts of the world. A comprehensive catalog of single-nucleotide variants in the etiopathogenesis of the spina bifida on a background of the Familial Neural Tube Defects Panel has been generated.

Abdominal lipoblastoma is a rare tumor composed of embryonic fat. Mesenteric involvement is rarer, with only five cases reported in infants. We report a case of a 10-month-old infant with a lipomatous tumor arising from the ileal mesentery. Primary resection was done, and histopathology was suggestive of a lipoblastoma. The child is well in follow-up with no evidence of recurrence.

Aim: The aim is to study the demographics and management of unusual airway foreign bodies (UAFBs) in children.

Materials and methods: A retrospective observational study (2000-2020) of children with UAFBs, who underwent bronchoscopic removal. The demographics, types of foreign bodies, clinical and radiological features, management, and outcomes were collated and analyzed. Common foreign bodies, such as nuts, seeds, and vegetable aspirations, were excluded.

Results: Among 531 children who had bronchoscopic retrieval of airway foreign bodies, 74 (59 males/15 females) had unusual foreign bodies. These included pen caps, whistles, plastic objects, pins, coal piece, stones, clay, pencil, gold ornament, and glass bangle. Thirty-one children presented within 1 day of aspiration, 37 within 1 month and 6 till 6 months. Thirty-nine were below 5 years, and the rest were between 5 and 15 years. Cough, respiratory distress, fever, choking, and noisy breathing were common presentations. Others are stridor, whistling, cyanosis, loss, and change of voice. Chest X-ray showed ipsilateral hyperinflation (23), haziness (21), radio-opaque foreign body (17) and was normal in 14. The left main bronchus, followed by the right main bronchus and trachea, were the sites of impaction. Four children required additional procedures (tracheotomy and thoracotomy). There was one mortality in the study cohort.

Conclusion: Aspiration of unusual foreign bodies is not uncommon in children. Most aspirations are witnessed and predominant in boys. The left bronchus is the common site of impaction, and X-ray clinches the diagnosis. Presentation is delayed, and bronchoscopic retrieval, though challenging, has favorable outcome.

During face mask ventilation, invariably, some air enters the esophagus rather than the airways; this results in gastric insufflation while the esophagus is intact or loss of tidal volume in patients with a cervical esophagostomy (Cx-esophagostomy).

Objective: The objective of the study was to report the indigenous use of an electrocardiography (ECG) electrode in successfully occluding the Cx-esophagostomy to prevent inadvertent loss of tidal volume.

Materials and methods: Twenty-nine observations were recorded on 27 patients (mean age: 10.9 months; male:female = 2.6:1). The oral cavity, pharynx, and Cx-esophagostomy were cleared of saliva. Peristomal area was cleaned with dry gauze and dried with alcohol. Latex-free, 50 mm, ECG electrode was pasted symmetrically over the Cx-esophagostomy. The efficacy of the electrode was assessed using a five-point algorithm: (i) collapse of reservoir bag, (ii) chest expansion, (iii) ballooning of electrode, (iv) cotton-wisp test, and (v) change in compliance with manual compression of the electrode. Subgroup analysis was conducted for laterality and health of Cx-esophagostomy.

Results: Study cohort: Cx-esophagostomy was located on the left in 58.26% (n = 17/29) patients. The health of Cx-esophagostomies was graded from I to IV (n = 9, 16, 3, and 1, respectively). The collapse of the reservoir bag and chest rise indicated successful ventilation. Ballooning of the electrode with each breath was observed in 79% (23/29) patients. Partial separation of the electrode from the skin (exaggerated with each breath) was observed in 10.3% (3/29) patients (Group I: n = 1, Group III: n = 1, and Group IV: n = 1). This was related to poor local preparation or pooling of saliva (n = 1; Group I: resolved with procedural re-application of a fresh electrode) and underlying skin condition (n = 2; Groups III and IV: resolved only temporarily with re-application). The cotton-wisp test was positive in two additional patients (1 each in Groups II and III) and was related to the pooling of saliva. No change in compliance was observed with manual compression of the electrode. No difference in success was observed between left and right Cx-esophagostomies. Overall success with this technique was 82.8% with a potential for 100% in those with peristomal health graded I-III.

Conclusions: The ECG electrode is a technically simple, cost-effective, and widely available device, instrumental in addressing the problem of air leak through Cx-esophagostomy during face mask ventilation before intubation.

Introduction: Acute appendicitis is a common surgical emergency in children, often requiring imaging for confirmation due to diverse presentations. While computed tomography scan is favored in many centers for its sensitivity, it comes with radiation exposure and higher costs. Ultrasonography, being radiation-free and cost-effective, is gaining popularity, especially in pediatric cases. However, its reported accuracy varies in the literature. This study aims to evaluate the diagnostic accuracy of ultrasonography in pediatric appendicitis cases and to determine its precision in distinguishing between simple and complicated cases of appendicitis.

Materials and methods: A retrospective analysis was conducted on children with suspected appendicitis who presented to the department of pediatric surgery. All patients underwent ultrasonography followed by appendicectomy based on considered clinical decision. Ultrasonography findings were compared with intraoperative observations categorized as uncomplicated or complicated appendicitis.

Results: Among 152 patients, ultrasonography accurately diagnosed appendicitis in 94.6% of cases, with 5.38% having nonvisualized appendices. In our group, the sensitivity and specificity of ultrasound to detect appendicitis were 94.62% and 95.65%, respectively. The diagnostic accuracy of the test is 95.63%. However, in our study, the sensitivity of the ultrasonography to correctly identify if it is a simple or complicated appendicitis was only 54.9% but had a specificity of 98.7%. Thus, ultrasonography showed a much lower sensitivity (54.9%) in distinguishing between simple and complicated appendicitis.

Conclusion: Ultrasonography demonstrates high sensitivity and specificity in diagnosing pediatric appendicitis, making it a promising preoperative investigation. However, it may not accurately differentiate between simple and complicated cases. A comprehensive approach involving clinical and laboratory parameters alongside secondary imaging may be necessary for accurate diagnosis, especially in cases of perforated appendicitis.

Background: The occupational hazards of glutaraldehyde are well known; the possibility of harm to the patients has been highlighted in the form of isolated reports only.

Objective: To synthesize evidence for contact mucosal injury or injury due to intraperitoneal instillation of glutaraldehyde following its use during laparoendoscopy.

Materials and methods: The current review is Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) compliant. PubMed, PubMed Central, and Google Scholar were interrogated for animal and human studies upon the harmful effects of glutaraldehyde during laparoscopy and proctosigmoido-colonoscopy.

Results: Thirty-five studies substratified into animal experiments (n = 2), glutaraldehyde-induced colitis (G-iC) postendoscopy (n = 30), and laparoscopy (n = 3) were included. Rats suffered mucosal injury following colonic injection of glutaraldehyde which was time- and concentration-dependent quantum and developed bloody diarrhoea. Omental and renal injury was observed due to glutaraldehyde instillation during simulation of intra-peritoneal insufflation in rats; the serum leucocytes, CRP and creatinine were also elevated. G-iC following colonoscopy was related to contact mucosal injury due to failure (human or machinery) to rinse the chemical off the instrument surface or as a case of mistaken identity (glutaraldehyde was mistaken for saline or another reagent). The incubation period was <24 h in 90% of patients; clinical presentation was related to the initiation of intense inflammation. Intraperitoneal instillation during laparoscopy also leads to intense inflammation. One patient was asymptomatic, the second required laparotomy and peritoneal irrigation for 48 h, while the third case underwent multiple laparotomies for enteric cutaneous and enteroenteric fistulae, adhesions, and multiple intra-abdominal abscesses. Hydronephrosis consequent to retroperitoneal fibrosis has been reported.

Conclusions: The use of glutaraldehyde in the patient care facilities should be discontinued; when unavoidable, the standard processes of disinfection and rinsing must be stringently followed.