European Journal of Pediatric Surgery Reports最新文献

Background: Fibrous hamartoma of infancy (FHI) is a rare benign soft tissue tumor of early childhood, often misdiagnosed due to its clinical and/or radiological resemblance to vascular malformations or pediatric soft tissue neoplasms.

Case presentation: A 7-month-old male presented with a rapidly enlarging, firm, non-pulsatile subcutaneous mass involving the anterior aspect of almost the entire right arm. MRI suggested a low-flow vascular malformation; however, due to clinical concern for alternative pathology and the lesion's benign appearance, large size, superficial location, and resectability, complete excision was performed. Histopathology revealed the characteristic triphasic pattern confirming fibrous hamartoma of infancy. The patient recovered well with no recurrence at 3-month follow-up.

Conclusion: This case highlights the diagnostic pitfalls of FHI, which may closely mimic vascular anomalies on imaging, and underscores the importance of surgical excision for both definitive diagnosis and curative treatment. To our knowledge, this represents the first reported case of FHI from Palestine.

Waugh's syndrome, the rare coexistence of intussusception and intestinal malrotation, has rarely been reported in literature, with fewer than 100 cases described globally. Its diagnosis is challenging due to non-specific symptoms of both conditions and the frequent success of non-operative reduction of intussusception, which often results in underdiagnosis of malrotation. We present a case of a 6-month-old boy who developed recurrent vomiting, feeding intolerance, and failure to thrive. Ultrasound imaging revealed ileocolic intussusception and a mobile cecum. Laparoscopy confirmed malrotation, and the patient underwent manual reduction of intussusception, followed by Ladd procedure. The child had an uneventful postoperative recovery without complications. Early diagnosis of Waugh's syndrome requires a high index of suspicion, particularly in recurrent obstructive symptoms where malrotation may be present. Enhanced imaging techniques can facilitate prompt diagnosis and guide appropriate surgical intervention, preventing complications. Clinicians should maintain a high degree of suspicion for Waugh's syndrome to ensure timely and effective intervention.

Indocyanine green fluorescence (ICG-F)-guided surgery has, in recent years, optimized the precision and safety of surgical procedures. Although its applications are increasingly widespread, in most cases, the dye is administered intravenously. We present a case of inhaled indocyanine green use for the identification and resection of pulmonary metastatic nodules. A 12-year-old female patient with a history of sternal Ewing sarcoma with bone and pulmonary metastases was treated with chemotherapy, proton therapy, partial sternal and costal cartilage resection, and reconstruction with mesh and absorbable plates. She later developed pulmonary relapse, with two subpleural metastases identified in segments 6 and 9 of the right lung. After initiating chemotherapy, thoracoscopic surgery was planned to resect the nodules. Before surgery, nebulization of indocyanine green was performed via an endotracheal tube at 0.2 mg/kg using an inhalation chamber for 5 minutes at 6 liters per minute. During surgery, fluorescence was observed in the insufflated lung parenchyma, allowing for clear differentiation of metastatic nodules from peripheral fibrotic or inflammatory tissue and enabling a safe wedge resection of both lesions. The postoperative period was uneventful, and the patient is currently completing postoperative chemotherapy cycles.

Congenital hernia in the umbilical cord is a rare form of ventral abdominal wall defect, which, if not diagnosed, can lead to iatrogenic intestinal injuries due to improper umbilical clamping. We report a case of a newborn referred to our center for intestinal obstruction caused by a decapitated Meckel's diverticulum located within the umbilical cord. Only a few similar cases have been documented in the literature, including four fatalities. Our aim is to present this rare case to the surgical community to raise awareness about it as a potential differential diagnosis in neonatal obstruction cases and emphasize the importance of early treatment to reduce the risk of high morbidity and even mortality.

A posterior cloacal variant is a congenital malformation where a urogenital sinus terminates anterior to a normally placed anus. These are rare malformations with highly variable anatomy. We report on three cases of a novel phenotype of posterior cloaca encountered at our institutions between October 2021 and November 2023. Three newborn girls were referred with ambiguous external genitalia and an anorectal malformation. In all cases, a midline sac, which is likely fused labioscrotal folds, replacing the clitoris was noted anterior to the perineal orifices. Two of the three patients demised as a result of renal failure. The third patient underwent reconstruction and is well. This posterior cloacal phenotype appears to be frequently associated with severe renal insufficiency. In survivors of the neonatal period, a good cosmetic outcome is achievable. Functional outcomes remain to be assessed.

This case report describes the recurrence of an immature thyroid infiltrating teratoma in a female infant. Initially treated surgically for a cervical mass, the teratoma recurred, requiring further intervention. The case highlights the importance of multidisciplinary care and long-term follow-up in managing complex pediatric neoplasms.

[This corrects the article DOI: 10.1055/a-2692-6661.][This corrects the article DOI: 10.1055/a-2699-8104.].

Background: Kaposiform hemangioendothelioma (KHE) is a rare, aggressive vascular tumor frequently complicated by Kasabach-Merritt phenomenon (KMP), a life-threatening consumptive coagulopathy. Neonatal KHE-KMP management requires urgent intervention but is complicated by immunosuppression risks, especially in Bacillus Calmette-Guérin (BCG)-vaccinated infants.

Methods: A full-term male neonate with prenatal right upper limb thickening presented postnatally with a violaceous, firm mass. Laboratory findings confirmed KMP. Due to recent BCG vaccination, sirolimus was initially withheld. First-line therapies failed, prompting sirolimus initiation on day 3, supplemented by fibrinogen transfusions.

Results: Platelets normalized by day 13 (283 × 10 ⁹ /L) with marked tumor regression. Transient fever/diarrhea resolved with supportive care. At discharge (day 27), platelets stabilized (183 × 10 ⁹ /L). Three-month follow-up showed sustained platelet recovery (268-532 × 10 ⁹ /L), near-complete tumor resolution, and age-appropriate development. Prophylactic trimethoprim-sulfamethoxazole prevented infections.

Conclusion: Sirolimus is a critical salvage therapy for refractory neonatal KHE-KMP, even in BCG-vaccinated infants. Timely initiation reverses life-threatening coagulopathy and achieves favorable outcomes, necessitating multidisciplinary monitoring to balance immunosuppression risks.

A newborn of 32 + 6 weeks' gestational age with prenatal diagnosis of gastroschisis was born through elective caesarean section. Ultrasonography at 16 + 4 gestational weeks (GW) showed a gastroschisis with free bowel loops floating in amniotic fluid. From 27 + 4 GW onward, serial ultrasounds showed the disappearance of extra-abdominal intestine and progressive intra-abdominal intestinal loops dilation, raising suspicion for vanishing gastroschisis. Birth weight was 2,136 grams and the external appearance of the abdomen was normal. An exploratory laparotomy was performed, finding a dilated proximal jejunal loop with a type III intestinal atresia, microcolon, and no other remainder bowel in between. The total length of the small intestine was 21 cm. Serial transverse enteroplasties for intestinal lengthening (reaching 38 cm), along with lateroterminal jejunocolic anastomosis were performed. The patient was discharged after 5 months of hospitalization with home parenteral nutrition. At 2 years and 8 months of age, the child is thriving and off parenteral support. Vanishing gastroschisis is a rare and severe form of complex gastroschisis whose prenatal diagnosis is crucial for parental counseling, timely delivery, and early surgical intervention. Multidisciplinary approach is essential to manage intestinal failure and improve long-term outcomes in these patients.

Superior mesenteric artery (SMA) syndrome is a rare cause of proximal bowel obstruction in pediatric surgery. We present the management of a rare, idiopathic, and acute form of SMA syndrome in a teenage girl, successfully treated with a robotic approach. A 14.5-year-old girl with no prior medical history and a normal body mass index (BMI) for her age (18.4 kg/m ² ) was admitted to our department with acute proximal bowel obstruction syndrome. Initial imaging, including an abdominal X-ray, computed tomography scan, and upper gastrointestinal tract radiography, confirmed the diagnosis of SMA syndrome. Conservative management was initiated with nasogastric tube placement, postural adjustments, and optimal nutritional support. However, symptoms persisted, and surgery was performed 5 months after the initiation of conservative treatment. A robotic-assisted duodenojejunostomy, preserving the fourth portion of the duodenum, was performed without intraoperative complications. Postoperatively, the patient experienced immediate symptom relief and was discharged on postoperative day 4. The postoperative course and long-term follow-up (7 years) were uneventful. This case report describes an acute, idiopathic form of SMA syndrome successfully treated with robotic-assisted duodenojejunostomy in a teenage girl with a normal BMI. To our knowledge, this is only the second reported case of robotic surgery for SMA syndrome in the pediatric literature.