European Journal of Pediatric Surgery Reports最新文献

An inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor that occurs predominantly in children and young adults. Etiology remains unclear. But based on the frequent detection of chromosomic alterations, especially near the anaplastic lymphoma kinase (ALK) gene, IMT is now considered to be a true neoplasm. In addition, the possible aggressive behavior, and the ability to metastasize suggest at least an intermediate malignant potential. Surgery remains the treatment of choice, but the use of chemotherapy, nonsteroidal anti-inflammatory drugs, immunotherapy, and targeted therapy are reported. We describe a case of a pulmonary IMT in a 6-year-old boy with an incidental finding of a lesion in the right upper lobe. A video-assisted thoracoscopic right upper lobectomy with lymph node resection was performed. Microscopic examination confirmed the diagnosis of IMT with the nodule showing spindle cells in a background of plasma cells. ALK immunohistochemical expression was negative.

Patients with congenital diaphragmatic hernia (CDH) can present with other congenital anomalies, but an associated choledochal cyst (CC) has rarely been described. The simultaneous diagnosis of both anomalies complicates patient management. There is no consensus on the ideal timing for CC excision. Unrepaired CC is associated with risks of developing biliary sludge, choledocholithiasis, and cholangitis. After a CDH repair with mesh, secondary bacterial translocation caused by a delayed CC repair could lead to mesh superinfection. Conversely, early CC surgical management could cause mesh displacement and lead to CDH recurrence, requiring reintervention. We present the rare case of a CC occurring in a neonate with a prenatally diagnosed right CDH. One month after an uncomplicated CDH repair with mesh, while the patient was still hospitalized for pulmonary hypertension, she developed progressive cholestasis and acholic stools. Investigations revealed a nonpreviously suspected CC. Conservative treatment was attempted, but CC perforation with secondary biliary peritonitis occurred. Open CC excision with a Roux-en-Y hepaticojejunostomy was therefore performed on day of life (DOL) 41. Having suffered no short-term surgical complications, the patient was discharged on DOL 83 because of prolonged ventilatory support due to pulmonary hypertension. Now 12 months after surgery, she is doing well with normal liver function tests and imaging studies. In summary, CC should be considered in the differential diagnosis of progressive cholestasis in patients with CDH. Surgical repair of a symptomatic CC should not be delayed even in the presence of mesh given the risks of CC complications.

The conventional approach to managing a newborn with cloacal exstrophy typically includes separating the cecal plate from between the two hemibladders, tubularizing it to be included in the fecal stream, creating an end colostomy, and bringing the two bladder halves together. This study introduces an alternative approach wherein the cecal plate is retained in its original position and designated for future use as an autoaugment of the bladder. Four cases of cloacal exstrophy cases managed between November 2019 and February 2024 are described, with surgical approach and postoperative outcomes reported. Two patients who underwent traditional reconstruction experienced bacterial overgrowth attributed to stasis in the cecal plate, which manifested in increased ostomy output and feeding intolerance. Treatment in these two cases was to remove the cecum from the fecal stream and use it instead for a bladder augment. Learning from these cases, the third and fourth newborn's approach involved retaining the cecum in situ for autoaugmentation of the bladder and performing an ileal to hindgut anastomosis. No postoperative acidosis occurred in these patients. The alternative approach to the newborn management of cloacal exstrophy whereby the cecal plate is left in situ can decrease stasis and postoperative bacterial overgrowth. It allows for an autoaugmentation of the bladder and is technically easier than the traditional rescue of the cecal plate from within the two hemibladders.

Solid pseudopapillary neoplasm (SPN) is a rare low-grade malignant tumor of the pancreas that occurs predominantly in young females. This tumor is occasionally multicentric, posing a unique surgical conundrum for resection. We present a case of a 10-year-old female with a history of multicystic dysplastic left kidney and persistent urogenital sinus who was diagnosed with biopsy-proven multicentric SPN of the pancreatic head and tail and underwent middle-preserving pancreatectomy. The patient tolerated the surgery very well. Our case is one of the few reported cases of multicentric SPN in a pediatric patient, and the only case treated with middle-preserving pancreatectomy, which is a novel surgical option for protecting pediatric patients from total endocrine and exocrine pancreatic insufficiency. With the increase in the incidence of SPN, there is an increasing need for pancreas-preserving surgical options, particularly in pediatric patients.

Left ventricular diverticulum (LVD) is a rare malformation presenting in 0.05% of all congenital cardiac anomalies. It is associated with additional cardiac and extracardiac malformations. We report on a female neonate with prenatally diagnosed heterotaxia and dextrocardia who was born with a pulsating supraumbilical mass. Echocardiography revealed a diverticulum originating from the left ventricle, which was connected to the umbilicus. Magnetic resonance imaging confirmed an LVD without evidence of a diaphragmatic hernia on the day of life 9. The child underwent laparotomy/lower sternotomy, and the diverticulum and epigastric hernia were closed. The postoperative course was uneventful, and the girl was discharged on the 10th postoperative day. In a neonate with a pulsatile supraumbilical mass, the diagnosis of a congenital LVD should be taken into consideration. The treatment is straightforward and was successful in this single case.

A 13-year-old male patient with marfanoid features and pectus excavatum with Haller index 4 and correction index of 38% underwent the Nuss procedure with cryoanalgesia 9 days prior, which transpired uneventfully. Preoperative spirometry was normal, and echocardiogram showed light aortic valve dilation. A month later, during a routine outpatient checkup, he referred middle abdominal pain, denying respiratory symptoms nor thoracic pain. He presented bilateral apical and right basal hypophonesis. Chest X-ray revealed bilateral pneumothorax and right pleural effusion. Consequently, the patient was admitted to the emergency room, and a chest computed tomography was ordered, reporting right apical blebs. Bilateral thoracoscopy was performed, and apexes were checked for pulmonary blebs to rule out primary pneumothorax. In the right chest, a wedge resection of a distorted area on the apex and pleuroabrasion were done. Four air leaking eschars were found when performing lung expansion under water as leaking test, corresponding to cryoanalgesia intercostal eschars, and subsequently closed by primary suture. In the left chest, there were no blebs. However, another four pleural lesions with intact pleura in the left lower lobe were also found. Postoperative course was uneventful and chest drains were removed 48 hours after surgery. He remains asymptomatic 21 months after discharge. Cryoanalgesia in pectus excavatum is spreading due to the improvement in postoperative pain control. However, some complications may occur.

Concomitant presentation of jejunal atresia and Hirschsprung's disease is rare and places children at high risk for developing short bowel syndrome and parenteral nutrition dependence, which can affect the feasibility/timing of pull-through. A patient was born with jejunal atresia with a delayed diagnosis of Hirschsprung's disease. After several procedures and bowel resections, the patient was ultimately left with an end jejunostomy and long Hartman's pouch with short bowel syndrome, dependent on parenteral nutrition. The patient initially presented to our institution at age 2 with failure to thrive secondary to an obstructed/dilated jejunostomy and mild enterocolitis of their defunctionalized segment. The patient subsequently underwent completion of subtotal colectomy and revision of jejunostomy utilizing a serial transverse enteroplasty to manage the dilated bowel and gain length. The patient was able to wean off parenteral nutrition and achieve nutritional autonomy by age 5. Following this, the patient was able to undergo an ileoanal pull-through. After the pull-through, the patient was able to pass stool independently and suffered no major complications to date. Serial transverse enteroplasty can be successfully utilized in patients with a history of Hirschsprung's disease and jejunal atresia to achieve nutritional autonomy and ultimately reestablish gastrointestinal continuity with pull-through.

[This corrects the article DOI: 10.1055/a-2227-6389.].

A boy with congenital hydronephrosis underwent ultrasonography every month for follow-up. At 4 months of age, ultrasonography incidentally revealed congenital biliary dilatation (5-cm type Ia). We performed laparoscopic extrahepatic bile duct resection and hepaticojejunostomy. After dissecting the dilated common bile duct (CBD), we found that the arcading-like shaped right hepatic artery (RHA) coursed in front of the CBD. Additionally, a tiny duct was identified below the main hepatic duct. At first, we thought it was a lymphatic vessel and dissected it from the main hepatic duct. However, bile flow out was recognized after dissecting the tiny duct. Finally, we confirmed it as an aberrant bile duct from the caudate region. We anastomosed the bile duct from the caudate region and main hepatic duct in a double-barrel fashion and performed hepaticojejunostomy below the RHA. The postoperative course was uneventful. Ultrasonography showed no intrahepatic ductal dilatation including the caudate lobe.