European Journal of Pediatric Surgery Reports最新文献

Small bowel volvulus is a rare but important cause of abdominal pain and small bowel obstruction in children and adults. In the neonate, small bowel volvulus is a well-known complication of malrotation. Segmental small bowel volvulus is a lesser-known condition, which occurs in children and adults alike and can rapidly progress to bowel ischemia. Primary segmental small bowel volvulus occurs in the absence of rotational anomalies or other intraabdominal lesions and is rare in Europe and North America. Clinical presentation can be misleading, causing a delay in diagnosis and treatment, in which case the resection of necrotic bowel may become necessary. We report on a 14-year-old girl who presented with severe colicky abdominal pain but showed no other signs of peritoneal irritation or bowel obstruction. An emergency magnetic resonance imaging was highly suspicious for small bowel volvulus. Emergency laparotomy revealed a 115 cm segment of strangulated distal ileum with no underlying pathology. We performed a detorsion of the affected bowel segment. Despite the initial markedly ischemic appearance of the affected bowel segment, the patient achieved full recovery without resection of bowel becoming necessary.

Currarino syndrome (CS) is a rare condition that presents with any combination of a sacral defect, a presacral mass, and an anorectal malformation. This collection, referred to as Currarino's triad, may not necessarily present as all three abnormalities in the diagnosis of the syndrome. Anal canal duplication (ACD) is an even rarer occurrence. We present a case that lies on the CS spectrum with an associated ACD and discuss a complex surgical challenge that necessitated a customized management plan, devised through a multidisciplinary approach.

Acute appendicitis is common in children and adolescents. Recently, conservative antibiotic treatment is regarded to be a safe approach to treat uncomplicated appendicitis. It is already established as initial treatment in cases of perforated appendicitis with perityphlitic abscess, commonly followed by interval appendectomy. We report on a 13-year-old boy with uncomplicated appendicitis and a 17-year-old girl with complicated, perforated appendicitis and perityphlitic abscess in whom initially successful antibiotic treatment led to a delay in detection of a carcinoid tumor (neuroendocrine tumor, NET) of the appendix. NET of the appendix, with an incidence of 0.03 to 0.8% in the pediatric population undergoing appendectomy for acute appendicitis, are usually incidental findings after appendectomy with no secure method for detection prior to surgery. Raising concern about this rare but severe disease, we recommend information of patients and their parents about the potential risk of belated diagnosis before opting for conservative their treatment of acute appendicitis. Furthermore, patients successfully treated conservatively require a close follow-up by ultrasound. In presence of any conspicuous finding, especially on imaging, appendectomy should be considered.

We report on an 11-year-old girl with cystic fibrosis who presented with thoracic pain and an extensive subcutaneous emphysema and subsequently developed progressive respiratory distress. The chest computed tomography revealed a huge pneumomediastinum. Due to the development of severe respiratory failure, urgent needle thoracocentesis was necessary that resulted in only temporary improvement. Therefore, under general anesthesia two mediastinal drains were introduced. Using active suction, the size of the pneumomediastinum decreased gradually and the drains were removed after 3 weeks. Here, we describe an extremely rare situation, when acute surgical intervention was necessary in a child with spontaneous pneumomediastinum.

The most common congenital lung malformations are congenital pulmonary airway malformations and pulmonary sequestrations. Many surgeons advocate resection to prevent complications of infection, malignancy, and pneumothorax. The standard of care is lobectomy, but segmentectomy and embolization alone have been reported. These methods avoid the complications of lobectomy but are not widely practiced due to concerns about incomplete resection or involution of the lesion. We present a novel approach to the treatment of a pulmonary sequestration in a 7-month-old male using preoperative embolization followed by a sublobar pulmonary resection. The embolization clearly demarcated the affected lung intraoperatively, thereby facilitating complete removal of the lesion with a segmental lung resection rather than complete lobectomy.

We present a case and discuss the management of a posterior cloacal variant not as yet described in the literature. A 5-week-old infant presented to our institution with a posterior cloacal variant and transposition of the clitoris and labia. After initial radiological investigations, staged operative intervention was performed over a 1-year period. This included an initial laparotomy (with drainage of hydrocolpos and formation of a colostomy), a left ureteric reimplantation and a posterior sagittal anorectoplasty due to a rectoperineal fistula. The child is under continued long-term follow-up by our specialist pediatric surgical team.

The case of a toddler with long-channel cloaca, mild chronic kidney disease (CKD) due to renal dysplasia, and early onset of ulcerative colitis (UC) is herein reported. The patient underwent definitive repair of cloaca, that included vaginal elongation with colon, at 5 months of age and was admitted for episodes of vaginal bleeding at 22 months of age. A vaginoscopy revealed a severe inflammation of the colonic neovagina. As rectal bleeding was also noticed, she underwent a colonscopy that showed the same macroscopic inflammatory picture. Neovaginal and colonic biopsies confirmed UC. The mother turned out to be affected by UC since adolescence. The patient is now on oral therapy with mesalazine and topical steroid and mesalazine in the neovagina. The association between cloaca and inflammatory bowel disease (IBD) is anecdotal, but the family history of IBD should be considered when planning the surgical reconstruction of patients with cloaca. In this patient, the occurrence of UC may require a new neovagina in the future and the concomitance of CKD may complicate the overall management due to the potential nephrotoxicity of drugs used for UC therapy.

Anorectal malformation with rectovestibular fistula associated with vaginal agenesis is rare. We report on a child in whom this combination was diagnosed at the age of 1 year. After creation of a divided descending colostomy, we chose to leave the rectum-rectovestibular fistula to function as a neovagina, while the sigmoid colon was relocated via modified posterior sagittal anorectoplasty. The colostomy was closed 6 months after the main surgery. After a follow-up of 3 years, the patient is continent for stool and urine. The introitus of the neovagina appears wide and easy to explore. We conclude that our surgical approach may be a good option for these children.

Pyloric web is a rare cause of gastric outlet obstruction. Classical pyloric web can be diagnosed by obtaining a patient history, physical examination, and plain abdominal X-ray, whereas a perforated web leads to incomplete intestinal obstruction. Delayed diagnosis is rare, and the definite diagnosis is made by upper endoscopy. In this report, we report a case of a girl in whom a pyloric web was diagnosed at the age of 13 months.

Kasai portoenterostomy (KPE) is currently the first-line treatment for biliary atresia. Many pediatric surgeons have reported that the dissection of the fibrous remnant at the porta hepatis is one of the most important components of this procedure. Furthermore, laparoscopic portoenterostomy is being increasingly used to treat biliary atresia. An advantage of laparoscopic surgery is that surgeons can more easily identify microbiliary ducts, owing to the magnification. We report the case of a 61-day-old girl on whom we performed an exploratory laparotomy and diagnosed type III biliary atresia using intraoperative cholangiography. For the first time, we performed an open KPE using an 8K ultra-high-definition television system. This allowed us to clearly view the porta hepatis and to successfully perform the portoenterostomy.