Solitary Peutz-Jeghers Polyp in a Paediatric Patient

Hamartomatous polyps of Peutz-Jeghers are mostly found in patients affected by Peutz-Jeghers syndrome (PJS), but they can be rarely encountered in the general population. It is unclear whether a solitary Peutz-Jeghers polyp (PJP) is an incomplete form of PJS or a separate entity. We report a case of solitary PJP in a paediatric patient in whom the other features of PJS were absent. The patient underwent laparotomy due to small bowel intussusception secondary to an ileac polyp. Histological examination showed the characteristic features of PJP, but the patient did not fulfill the WHO criteria for PJS diagnosis (negative family history for PJS and absence of mucocutaneous pigmentation); moreover analysis of the STK11/LKB1 gene did not reveal any genomic abnormality. The clinical and investigative findings in our case suggest that the solitary PJP can be considered a different clinical entity from PJS

An unusual case of acute pancreatitis and gastric outlet obstruction associated with wandering spleen treated by laparoscopic splenopexy.

Wandering spleen (WS) is an uncommon condition, usually asymptomatic, often recognized as an incidental finding. When symptoms occur, they can vary, although acute abdominal pain is the most common presentation in the pediatric population. In some cases, WS can become a dangerous condition because of the risk of splenic ischemia from persistent pedicle torsion. We describe a case of WS in a 3-year-old boy presenting with vomiting, abdominal swelling, and acute pancreatitis; the diagnosis was obtained by ultrasound and computed tomography. Laparoscopic splenopexy was successfully performed through an extraperitoneal pocket and a Vicryl mesh. To the best of our knowledge, the combination of gastric outlet obstruction and acute pancreatitis has never been reported as presenting symptoms of WS

Preventing posterior sagittal anoplasty 'cripples' in areas with limited medical resources:a few modifications to surgical approach in anorectal malformations

Background: Anorectal malformations (ARM) are the most common neonatal emergencies in Sub-Saharan Africa countries. Late presentation, lack of pediatric facilities and trained paediatric surgeons influence the outcome of these patients. This study reports a 5-year of experience in the management of ARM at the Orotta Referral Hospital in Asmara (Eritrea) and proposes some modified surgical approaches to minimize the risk of complications and the length of hospital stay. Materials and Methods: We reviewed the records of 38 patients with ARM observed between September 2006 and April 2011. Since 2009 a modification of original posterior sagittal anorectoplasty (PSARP) was introduced, consisting in a long rectal stump (3 cms) closed and left at the perineal level, to be trimmed after two weeks. This avoided mucous spillage on the wound and prevented contamination. Post-operative course and outcome were evalued in the two group of patients divided according the type of surgical technique (Group A: Standard PSARP; Group B: Modified PSARP). Results: There were 21 boys and 17 girls aged 4 days to 9 years (median age 182 days). Of the 38 patients, 2 infants died before surgery and 3 refused preliminary colostomy. Previously confectioned colostomies often required revision or redoing due to severe prolapse or malposition. When possible, primary sigmoid colostomy was performed. There were 15 patients in Group A and 18 in Group B. Wound infection or disruption were recorded in 7 cases (46%) in Group A and in 2 (11%) in Group B. Late complications were related to anal stenosis, which required long term dilatations. Three cases needed a PSARP redo (2 in Group A, 1 Group B). Conclusions: We believe that our simple modification of original PSARP technique could be of help lowering post-operative complications rate and reducing hospital stay. Family compliance is mandatory for long-term surgical success. A relevant time must be spent in training to stoma care and post-operative anal dilatation

Plexiform schwannoma of esophagus in a child with neurofibromatosis type II.

Schwannoma is a benign neoplasia of the peripheral nerve sheath. Its localization in the gastrointestinal tract, and in particular in the esophagus, is very rare. According to the existing literature esophageal schwannoma has been reported so far only in adult patients. We report the case of an 11 year old patient with neurofibromatosis, type 2, who underwent surgical excision of a plexiform schwannoma of the esophagus