Journal of Indian Association of Pediatric Surgeons最新文献

Lipofibromatosis is a rare and benign soft-tissue tumor predominantly affecting children. It commonly presents as a slow-growing, painless mass, often misdiagnosed due to its rarity and variable presentation. We report the unusual case of an 8-month-old male with a congenital upper thoracic mass initially suspected to be a lipomyelomeningocele. Clinical examination and ultrasound supported this diagnosis, but magnetic resonance imaging findings suggested a soft-tissue tumor. The child underwent excisional biopsy, and intraoperative findings revealed a highly vascular, well-defined mass without spinal cord involvement. Histopathological analysis confirmed lipofibromatosis. The postoperative course was uneventful, and no recurrence was observed after 1 year of follow-up. This case highlights the diagnostic challenges associated with lipofibromatosis and its potential for misdiagnosis, and the importance of histopathology in establishing a definitive diagnosis. Early complete surgical excision remains the preferred treatment to prevent recurrence.

Introduction: Urethral hair is a rare but significant issue in cases of severe hypospadias, typically after using hair-bearing skin grafts. This can cause early and late complications, including urinary obstruction, infections, and stone formation. Prevention is the key, favoring nonhair-bearing grafts. If hair-bearing skin is used, hair removal techniques are employed. Managing complications may require surgical interventions such as endoscopic hair removal or repeat urethroplasty. Without available literature, this systematic review, elaborating and comparing various management techniques for urethral hair, attempts to generate evidence for managing such pertinent clinical scenario.

Materials and methods: PubMed, SCOPUS, Embase, and Web of Science databases were explored, and relevant studies were selected. Data were extracted to study the bibliographic information of each article, the number of patients in each article, the age of patients, primary diagnosis, the method used to manage the urethral hair, the number of times the procedure was repeated for satisfactory results, and total follow-up duration before reporting the results.

Results: A systematic review identified 80 articles, 16 eligible for full-text review, including 14 case reports and two original articles, covering 41 male patients treated from 1977 to 2023, with substantial agreement on study quality assessment among the observers. Most cases (n = 37/41) involved hypospadias follow-ups, with laser-based hair removal being the primary treatment (n = 32/41 patients). Various lasers, such as neodymium-doped yttrium aluminum garnet and carbon dioxide, were used, and a median of one procedure was needed.

Conclusion: Managing urethral hair posthypospadias repair requires laser treatments and continuous follow-up to prevent complications and ensure success.

Umbilical hernia is very commonly encountered in infants. Obstruction in an umbilical hernia, though rare, has been reported in literature. We report a rare case of a 2-month-old boy who had an obstructed cecum in the umbilical hernia with localized perforation and omphalitis.

Hair tourniquet syndrome (HTS) may commonly involve a genital structure causing strangulation and ischemic changes. The current standard of care for penile HTS leading to amputation is microsurgical reimplantation with approximation of the urethra, corporal bodies, and dorsal vein and artery anastomosis. In our case of delayed presentation of HTS of the glans penis, we did a nonmicrosurgical reimplantation which has shown satisfactory results.

Background: Appendicitis is one of the most common surgical emergencies in children. Despite the availability of a plethora of advanced diagnostic imaging, diagnosis of acute appendicitis in young children remains a challenge.

Materials and methods: This prospective observational study compared the surgeon's diagnosis with the intraoperative findings in the 78 children who underwent a laparoscopic appendectomy at a tertiary center over a duration of 18 months. After the clinical evaluation of the child, the surgeon was asked to fill out a pro forma. The delineation of complicated from uncomplicated appendicitis by a surgeon's clinical assessment was taken into consideration for the purpose of this study.

Results: Symptoms such as loose stools and vomiting were statistically significant in predicting the severity of appendicitis (P = 0.04 and P = 0.01). A value of total leukocyte count above 11,425 was found to be predictive of complicated appendicitis (sensitivity - 70.6% and specificity - 51.2% and area under the receiver operating characteristic curve [AUROC] - 0.67) with differential leukocyte count (neutrophils) above 74.4% (sensitivity - 80.6% and specificity - 62.5%). Similarly, on comparing the uncomplicated and complicated groups, the high-sensitivity C-reactive protein value was found to be significant (P = 0.001, sensitivity - 80%, specificity - 76.5%, and cutoff >71.20 mg/dl). Appendicitis Inflammatory Response score above 7 indicated complicated appendicitis (sensitivity - 73.5%, specificity - 61.4%, AUROC - 0.744, P = 0.002). Ultrasound findings help identify complicated appendicitis cases (P = 0.0004, sensitivity: 59%, specificity: 60%, and AUROC: 0.67).

Conclusion: Surgeon prediction was compared with intraoperative findings which were statistically significant with P = 0.0004, a sensitivity of 70.59%, and a specificity of 90.91% (accuracy - 82.05%, positive predictive value - 81.24%, and negative predictive value - 84.72%). Hence, a surgeon can play a crucial role in predicting the severity of the disease and can reduce the morbidity and mortality rates associated with complicated appendicitis.

Introduction: Infantile hemangiomas affect 4%-10% of children under 1 year, while venous malformations (VMs) occur in 1-5/10,000 births. Oral hemangiomas (OHs) often ulcerate and lead to complications, whereas VMs can cause significant morbidity through bleeding, painful expansion, or obstruction of vital structures. Early and effective treatment is crucial, and intralesional diode laser therapy has been proposed as a promising intervention.

Patients and methods: A prospective interventional cohort was conducted on 40 patients: 29 patients with OH and 11 patients with oral VMs at the pediatric surgery department of Cairo University Children Hospital. A 980-nm diode laser (Lasotronix-Smart M^®) was used for intralesional coagulation and excision. Outcomes, including lesion size, color improvement, and patient-reported satisfaction, were assessed at 1- and 6-month posttreatment.

Results: The median pretreatment lesion size of 16.5 mm significantly decreased to 13.2 mm (P < 0.01). Color improvement was observed as normal in 25% of cases, near normal in 52.5%, slight improvement in 17.5%, and no change in 5%. Fifty percent of patients reported their condition as "much better." Complications included edema in 87.5%, ulceration in 30%, bleeding in 7.5%, infection in 7.5%, and recurrence in 2.5%.

Conclusions: Intralesional diode laser therapy is a safe and effective modality for managing OHs and VMs in children, providing significant cosmetic and functional benefits with a low recurrence rate.

Rubber band syndrome (RBS) is a rare but potentially serious condition resulting from applying constricting objects, such as rubber bands, around a digit or extremity. This syndrome presents a diagnostic challenge due to its variable clinical presentation, subtle onset, and overlap with other conditions such as cellulitis, traumatic injury, and paronychia. The diagnostic process is based on a proper history, physical examination, and imaging techniques such as ultrasound and magnetic resonance imaging. Surgical intervention may be required in necrosis, infection, or compartment syndrome cases. This case report describes the management of a child with RBS by surgical exploration and highlights the clinical presentation, diagnostic challenges, and management strategies. Preventive measures, including parental education, child supervision, and safe storage of rubber bands, are also crucial in reducing the incidence of this condition.

Aim: The contemporary surgical management of congenital pouch colon (CPC) includes either excision or tapering of the pouch. The aim was to analyze the results of a new technique of pouch tapering by excising the lateral outpouching like a diverticulum and pull-through of the remaining tapered colon.

Methods: In this retrospective study, patients with type 1 and 2 CPC who underwent a three-stage operation consisting of (a) sitting a stoma just proximal to the pouch preserving the ileocecal valve wherever possible, (b) pouch tapering with pull-through, and (c) stoma closure was analyzed. The terminal fistula was separated from the bladder in males and its lowest termination in females. Pouch tapering was done eliminating the lateral outpouching such as a diverticulum of the pouch and tubularization of the remaining colon. The tapered colon was pulled through the sphincter complex and anoplasty was completed. The postoperative results were analyzed for fecal continence, nutritional status, and any re-dilatation.

Results: The study included eight patients with five males and five females over the period of the last 16 years. In three female patients, the residual lateral outpouching like a diverticulum with its vascular pedicle was retained with a small external stoma and two patients later underwent bladder augmentation using the same for management of urinary incontinence. During follow-up visits up to 4 years, the bowel continence was fair with minimum peri-anal excoriation and no re-dilatation with satisfactory nutritional status.

Conclusions: The outcome of this New tapering technique (NTT) for type 1 and 2 CPC by eliminating the lateral outpouching like a diverticulum and retaining the remaining tubular terminal bowel was satisfactory in terms of continence and nutrition. The excluded portion of the pouch is a potential source for bladder augmentation.

Duodenal perforation (DP) is a very rare entity in neonates. Although there can be several causes of retroperitoneal mass in a neonate, DP presenting as a retroperitoneal mass has not been described in the literature. We present a case of DP masquerading as a retroperitoneal mass in a 28-day-old neonate.