European Journal of Pediatric Surgery Reports最新文献

[This corrects the article DOI: 10.1055/s-0041-1723019.].

Cloacal exstrophy is a rare malformation that presents as a lower midline abdominal wall defect which affects the gastrointestinal and genitourinary systems. The components of cloacal exstrophy characteristically include omphalocele, exstrophy of perineal structures, and imperforate anus. Most of these patients also have renal anomalies such as pelvic kidney, fused kidneys, or solitary kidneys. This congenital condition can also be associated with spinal issues, such as spinal dysraphism. When combined with spinal defects, it is referred to as the omphalocele, exstrophy, imperforate anus, and spinal defects (OEIS) complex, and is one of the most challenging surgical conditions to manage. Here, we present a unique case of a low-birth-weight patient with OEIS and a liver containing giant omphalocele and the novel surgical technique used to manage her cloacal exstrophy whereby the cecal plate was not separated from the bladder halves, but rather left for an autoaugment, and the ileum was connected to the hindgut.

We report a 13-year-old girl who presented with a recurrent abdominal pain that started after her menarche. The abdominal palpation revealed tenderness over the left ovarian point. The laboratory study, ultrasonography, and abdominal X-ray were normal. The computed tomography and magnetic resonance imaging showed a double left renal vein with a retroaortic component, an increased left parauterine circulation, and ipsilateral ovarian vein engorgement. A diagnostic and therapeutic phlebography allowed a selective catheterization of a group of pelvic varicose veins draining to the left ovarian and to the internal iliac veins. There were no complications during the procedure and the symptoms disappeared 2 days later. Circumaortic left renal vein may cause hematuria, proteinuria, pelvic congestion syndrome, and massive hemorrhage during surgery. A conservative treatment is recommended for patients without gynecourological/renal symptoms or with mild hematuria. The endovascular treatment by gonadal venous embolization is safe and effective.

Congenital diaphragmatic herniae (CDH) with associated intrathoracic ectopic kidneys are rare congenital anomalies, with a reported incidence of only 0.25%. The authors report a case of a 24-day-old baby girl who was diagnosed with a left-sided CDH on a chest X-ray taken for pneumonia. Computed tomography scan showed CDH hernia, containing small and large bowel and whole left kidney with adrenal gland. Thoracoscopic reduction in the bowel, kidney, and adrenal gland into the abdomen and primary closure of the defect was achieved with no complications. During investigation of the child, it was discovered that her maternal aunt had also had a left-sided congenital diaphragmatic hernia containing the kidney, which was treated via open surgery after birth; she subsequently developed renal cell carcinoma and required radical nephrectomy of that kidney during her third decade.

Pediatric penile skin grafting is rarely performed. We present a case series of four pediatric patients receiving skin grafting due to the loss of penile skin. The four boys were followed up for 1 to 5 years. One full-thickness skin graft and three split-thickness skin grafts (STSGs) survived well with low Vancouver scar scale scores. One boy gradually developed lymphedema of the distal foreskin and underwent a second preputioplasty. He presented with normal erectile function and did not experience any pain. We propose thick STSGs as the most appropriate choice for pediatric penile skin reconstruction. Lymphedema of the foreskin is an important long-term complication of penile skin grafting.

Ruptured giant omphaloceles (GO) and gastroschisis with total liver herniation are rare cases of exceptionally large abdominal wall defects. Many of these children have lethal outcome. The surgical and postsurgical management are complex. We report on two cases treated with staged surgical repair using a wound retractor as a silo. With this technique, the liver and intestines could be reduced into the abdomen with secondary closure of the abdominal cavity within the first 1 to 2 weeks of life.

We present a case of a 6-month-old male infant with an anorectal malformation (ARM) who underwent colostomy as a newborn, and now presents for definitive repair. A colostogram is shown to identify the malformation and to help plan for the ideal surgical approach. The case is presented with a focus on surgical strategies for management of ARM in the male infant, with questions for the readers posed in a quiz format.

We present a case of a 14-year-old boy with chronic distension, poor growth, and chronic constipation. He undergoes anorectal manometry and rectal biopsy, confirming the diagnosis of Hirschsprung disease (HD). The case is presented with a key image and associated questions to prompt discussion on strategies for management and treatment of HD in late-diagnosed children.

Congenital perineal lesions are rare and can occur along with other birth defects such as anorectal malformations (ARMs) and urogenital anomalies. A colorectal hamartoma associated with a urogenital anomaly without ARM is extremely rare. We recently treated a newborn with posterior hypospadias and a solid perineal mass diagnosed as a colorectal hamartoma.

We report on a male preterm newborn with a large abdominal tumor found on prenatal ultrasound 2 weeks prior to delivery at 36 + 0 weeks of gestation. A postnatal abdominal plain film showed a mass with well-defined rim calcifications ("eggshell"), suggestive of a meconium pseudocyst. On the 4th day of life, the boy underwent exploratory laparotomy with resection of the cyst and end-to-back jejunojejunostomy. The postoperative course was uneventful. A meconium pseudocyst is the correlate of a sterile peritonitis caused by antenatal bowel perforation. It is an easily recognizable spot diagnosis any pediatrician and pediatric surgeon should be aware of.