European Journal of Pediatric Surgery Reports最新文献

Arterio-ureteral fistula (AUF) is a rare condition affecting nearby adult-only patients. Patients usually present with hematuria, often starting as intermittent hematuria which frequently increases, and may lead to hemorrhagic shock. Without rapid diagnosis and prompt treatment, AUF can be lethal. Risk factors for developing an AUF include a history of pelvic surgery mainly due to cancer, a history of pelvic radiation, a history of vascular surgery, or chronic indwelling ureteral stents (CIUS). Imaging to confirm diagnosis includes angiography, computed tomography (CT) scan, or retrograde pyelography, although AUF may be missed. Therefore, even if imaging is negative, the presence of hematuria of unexplained origin in combination with mentioned risk factors is highly suspicious for AUF and must be excluded. We report the case of a 16-year-old male patient who presented with a new onset of intermittent hematuria to our emergency room. The patient's history included previous pelvic surgery for resection of ganglioneuroma 6 years ago with bilateral replacement of the iliac artery and postoperative acute kidney failure with reconstruction of both ureters and CIUS. After the initial uneventful postoperative follow-up over 5 years, another Double J (DJ) catheter had to be placed into the right ureter due to hydronephrosis. Six weeks later, the patient presented with intermittent hematuria. Despite negative imaging, we performed immediate surgical exploration confirming the diagnosis of AUF. To the best of our knowledge, this is the first case of AUF under the age of 18 years reported in the literature. In conclusion, in patients with macrohematuria and a history of the abovementioned risk factors, AUF has to be kept in mind and must be reliably excluded.

Traumatic perineal injuries are rare but can result in significant morbidity, particularly when the anal sphincter is injured. The management of such injuries in the pediatric population is rarely noted in the literature. We aimed to describe reconstruction in such patients using lessons learned in reoperative anorectal malformation surgery. This is a single-institution retrospective case series of three pediatric patients who were referred to our institution with pelvic trauma who underwent anal sphincter reconstruction. Three patients aged 5 (female), 12 (male), and 13 (female) years were referred for reconstruction following pelvic trauma involving the anal sphincter, perineal body, and genitourinary system. All three underwent multidisciplinary evaluation with urology and gynecology (for females) and a subsequent repair with anal sphincter reconstruction utilizing a posterior sagittal approach. Two patients had ostomy reversal with appendicostomy for antegrade continence enemas and regained voluntary fecal control. The third patient is awaiting colostomy reversal but has regained volitional urinary control after urethral reconstruction. The experience gained from using the posterior sagittal anorectoplasty approach in reoperations for patients with anorectal malformations can be applied to cases of rectal trauma. Key aspects include mobilizing the rectum, repairing the sphincters, and placing them around the anus, and in females, reconstructing the perineal body. Pediatric pelvic trauma can cause devastating disruptions of physiology and are difficult to treat. Experience from reoperations for anorectal malformations can be applied to these cases, including the use of a multidisciplinary team and posterior sagittal approach.

Gastric teratomas are an extremely rare variety of teratomas in children. The aim of our series is to present the natural history and progression of the disease. Retrospective analysis of prospectively maintained data of all the gastric teratoma patients treated at our center was done from their electronic medical records. A total of four cases of gastric teratoma were found to have been treated, all of them being less than 1 year old with three-fourths being male. Typical imaging features of teratoma along with normal germ cell tumor markers helped in making a diagnosis. Surgery is the main form of treatment. In final histopathology, there was equal distribution of mature and immature teratomas. On long-term follow-up, there has been no incidence of recurrence. There needs to be reporting of more cases to verify its natural history.

Transverse testicular ectopy (TTE) is a rare anomaly in which both testicles descend through the same inguinal canal. Different variants of this anomaly exist, with the most common presenting as two separate spermatic cords and testicular vessel bundles. The management of this condition is challenging, as various factors have to be considered. We report on a 2-month-old preterm boy with TTE, admitted to the hospital due to an ipsilateral incarcerated inguinal hernia. Diagnostic workup included a physical examination revealing a large swelling in the right groin, ultrasound imaging that showed both testicles located in the right inguinal canal, and laboratory tests indicating a deficiency of anti-Mullerian hormone. All of these findings confirmed the diagnosis of TTE. Surgical treatment included diagnostic laparoscopy with herniorrhaphy, followed by inguinal revision with transseptal orchidopexy in a second procedure. The 12-month follow-up was uneventful. Though rare, TTE is an important differential diagnosis in case of an incarcerated hernia combined with (contralateral) empty scrotum. Pediatric surgeons must be aware of this entity. Meticulous diagnostic workup and careful surgical management are mandatory.

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.