Experience with retroperitoneoscopy in pediatric surgical oncology.

BACKGROUND: Retroperitoneoscopy (RS) has been successfully introduced in adult oncology for diagnostic procedures, staging, and surgical treatment. Its value for children has rarely been reported. This report describes the authors' experience using RS in the diagnosis and staging of cancer for children and adolescents. METHODS: All RS procedures performed at the authors' institution between 2004 and 2010 were reviewed. The authors' operative technique entails a 10- to 12-mm flank incision followed by finger and balloon dissection of the retroperitoneal areolar tissue, with carbon dioxide (CO(2)) insufflation used to push the peritoneal lining medially. One to two additional working ports are placed above the iliac rim and below the costal margin. In cases of peritoneal tear with leakage of CO(2) and progressive retroperitoneal impingement, a Veress needle is placed in the umbilicus for pressure release. RESULTS: This review included 16 patients with a median age of 16.4 years (range, 4.4-29.8 years) who underwent RS for lymph node sampling (9 cases), diagnostic biopsy (6 cases), or resection of a metastatic nodule (1 case). Four complications were encountered (3 conversions to open surgery and 1 self-limited gross hematuria). The mean operative time was 123.3 ± 33.5 min. The patients required 1.1 ± 0.8 days of intravenous analgesia on the average. The mean hospital stay was 1.7 ± 0.6 days. CONCLUSIONS: The authors believe that RS is a safe surgical technique for access to the retroperitoneum in pediatric patients. In cases of a peritoneal tear, placement of a Veress needle in the umbilicus effectively prevents conversion to open surgery. Retroperitoneoscopy should be considered for children who need biopsies, lymph node dissections, or resections of primary tumors in the retroperitoneum.

Age-related changes in the neuromuscular development of the internal anal sphincter.

PURPOSE: The internal anal sphincter (IAS) plays an important role in the pathophysiology of constipation and incontinence. We hypothesized that functional bowel obstruction in premature infants is because of a poorly developed IAS. We investigated the neuromuscular development of IAS in fetal, newborn, and adolescent pigs. METHODS: Paraffin sections of IAS from 5 different age groups, E60, E90, 1 day, 4, and 12 weeks old, were stained with protein gene product 9.5 (PGP9.5), *-smooth muscle actin (*-SMA), caldesmon (CALD), calponin (CALP), and desmin (DES) antibodies. Quantification of results was performed by grading the density of immunostaining. RESULTS: The PGP9.5-positive ganglion cells were observed in the myenteric and submucosal region of the entire length of the IAS at E60. An increase in ganglion cell size and density was observed with increasing age. There were striking differences in the density of PGP9.5, alpha-SMA, DES, CALD, and CALP immunoreactive fibers between prenatal and postnatal period with gradient increase in the number of fibers from after birth to 12 weeks of age. CONCLUSION: This study shows for the first time that there are age-related differences in the distribution of neurons and smooth muscle cell components in the IAS. The decreased expression of contractile and cytoskeleton proteins in smooth muscle cells together with decreased expression of neurons in the IAS in the perinatal period may lead to motility dysfunction causing functional intestinal obstruction seen in premature infants.

Experience with aortic grafting during excision of large abdominal neuroblastomas in children.

BACKGROUND: Total or near total resection of high-risk, stage 4 abdominal neuroblastoma has been correlated with improved local control and overall survival but may be complicated by vascular injury. We describe our experience in the management of significant aortic injuries during this procedure. METHODS: With the institutional review board waiver, medical records of children who had major abdominal aortic reconstruction during neuroblastoma resection from 1996 to 2006 were retrospectively reviewed. RESULTS: There were 5 children with aortic grafting: 3 girls and 2 boys. Mean age at surgery was 7.2 years (range, 16 months to 17 years). Two children were operated on for recurrent retroperitoneal disease. Tumor encasement of the aorta was seen in all children. In 3 children, the injury occurred during dissection of paraaortic and interaortocaval lymph nodes below the level of the renal arteries. In the remaining 2 children, injury occurred early during mobilization of the tumor. Three polytetrafluoroethylene tube grafts and 1 on-lay patch graft were used to repair the 4 distal aortic injuries. One 4-year-old female with aortic and renal arterial injuries was managed with an aortic Dacron tube graft and a polytetrafluoroethylene tube graft for the renal artery. The mean period of follow-up is 28 months after aortic graft (range, 3 months to 10 years). Total colonic ischaemia, transient acute tubular necrosis, and duodenal perforation were seen in one child, who needed subtotal colectomy and ileostomy. Another child with an omental patch over the graft had a transient duodenal obstruction, which was managed conservatively. There were no other complications, and 4 of the 5 children are disease-free to date. One child at 10 years after his distal aortic tube graft remained asymptomatic with normal distal blood flow on magnetic resonance angiogram and with normal growth. CONCLUSION: The neuroblastoma surgeon should be prepared to perform aortic and vascular reconstruction. Aortic encasement, preoperative radiation therapy, and reoperative surgery were observed in these patients and may be risk factors.

Orchidopexy and its impact on fertility.

We critically analysed the factors known to influence the fertility potential after orchidopexy in children. A comprehensive literature review of all publications in the English language listed in Medline using the words cryptorchidism, undescended testis, orchidopexy, fertility, semen analysis and surgery from 1970 to 2005. In unilateral ectopic, canalicular and emergent testes, as long as the surgery is carried out in early childhood, fertility outcome is good (>90%). The majority of the bilateral abdominal testes are infertile. While unilateral abdominal testes and unilateral absent or vanishing testes have favourable fertility potential, quantification has proven difficult. Despite multiple studies, fertility in crypt orchid tests is still an uncertain issue. Hormonal treatment in conjunction with early surgical correction has not been fully explored. We recommend orchidopexy soon after 6-7 months of age, corrected for term, to maximise the future fertility potential.

Atresia of the ileocecal junction with agenesis of the ileocecal valve and vermiform appendix: report of a case.

Intestinal atresia involving the ileocecal region is a very rare intestinal malformation, and the presence or absence of the ileocecal valve influences its surgical management. We report the case of a male newborn with a provisional diagnosis of distal ileal atresia, in whom laparotomy revealed that the entire ileocecal region was atretic with an absent ileocecal valve and appendix vermiformis. We resected the dilated terminal ileum together with the atretic segment and performed an ileocolic anastomosis between the terminal ileum and the transverse microcolon without valve reconstruction. When last seen, 8 months after the operation, the baby was developing normally. Ileocolic anastomosis without valve replacement appears to be sufficient if an ileocecal valve is completely absent and only a short segment of the terminal ileum is lost.

Enteric nervous system and developmental abnormalities in childhood.

ENS consists of a complex network of neurons, organised in several plexuses, which interact by means of numerous neurotransmitters. It is capable of modulating the intestinal motility, exocrine and endocrine secretions, microcirculation and immune and inflammatory responses within the gastrointestinal tract, independent of the central nervous system. Though the embryological development of various plexuses are completed by mid-way of gestation, the maturation of neurons and nerve plexuses appear to continue well after birth. Therefore, any histological or functional abnormalities related to the gastrointestinal function must be investigated with the ongoing maturational processes in mind.

Postnatal development of the mucosal plexus in the porcine small and large intestine.

Knowledge regarding the foetal and postnatal development of the enteric nervous system is crucial for the understanding of congenital disorders. While lot of information exists regarding the myenteric and submucosal plexuses, the development of the mucosal plexus has not been previously studied. The mucosal innervation seems to play an important role in the local reflex activity of the gut. In this study, we examined the development of enteric mucosal innervation in the pig at various ages of life. Small and large bowel paraffin-embedded specimens were stained with PGP 9.5 and neurofilament protein in three piglets from six age groups (60 and 90 days gestation, newborn, 4 and 12 weeks old, and adult pigs). Small and large bowel demonstrated identical innervation patterns. Myenteric and submucosal plexuses were stained with PGP 9.5 at 60 days gestation. However, the mucosal staining was first noted clearly at the newborn period. By 4 weeks, PGP 9.5 staining was noted in small amounts within the mucosa. Inner proprial and villous fibres were seen ahead in time to the subepithelial fibres. Both inner proprial and villous staining became quiet prominent by 12 weeks of age and remained unchanged into adulthood. However, the subepithelial fibres appear to increase in adulthood. This study demonstrates for the first time that enteric mucosal innervation first appears only at birth. The immaturity of the mucosa generated reflex activity, and secretory functions may have implication in the management of functional intestinal obstruction in the premature infant.