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Congenital colonic atresia develops in 1 in 20,000 to 66,000 births, accounting for 1.8–15% of all intestinal atresias. Approximately 8.9% of patients with colonic atresia have multiple colonic atresias. A female infant with abdominal distention and bilious vomiting did not have a bowel movement until day 3 of life. Plain radiography showed significant dilation of the intestinal tract and no gas in the pelvic cavity. A contrast enema at the age of 3 days showed microcolon and obstruction at the level of the upper rectum. Operative findings included 3 membranous atresias (Type Ⅲ). For the 2 proximal membranous atresias, membranectomy and colostomy in the sigmoid colon were performed. Preoperative contrast enema at 10 months showed a gap between the distal side of the stoma and the blind end of the rectum. The sigmoid colon and rectum were completely resected 6 cm distal to the stoma. Colostomy closure was performed. A total of 4 membranous atresias were present in the sigmoid colon and the upper rectum. In a patient with multiple atresias in the sigmoid colon and upper rectum, multiple-stage surgery could be safe after searching for complicating malformations and evaluating the intestinal tract preoperatively. [ABSTRACT FROM AUTHOR]

The therapeutic strategies for the proximal transection of pancreas in children, consequently accompanying main pancreatic duct ( MPD) damages, remain controversial for its rare incidence. We experienced one case of proximal MPD injury in a child and performed end-to-end anastomotic repair easily without major postoperative complications using intraoperative ultrasonography. Such patients could be discharged from hospital after a short period; however, the long-term result must be clarified. [ABSTRACT FROM AUTHOR]

A trichobezoar is a rare mass formed by the ingestion and accumulation of hair within the gastrointestinal tract, especially the stomach. Cases of an isolated gastric trichobezoar with extension into the duodenum or the jejunum have been reported; however, synchronous gastric and intestinal trichobezoars causing a small-bowel obstruction is very unusual. We report our experience of two such cases to demonstrate the efficiency of preoperative multidetector computed tomography in locating the double bezoars and assisting us in surgical decision making. Open surgery is inevitable for symptomatic bezoars, because the masses are too hard and large to break up with endoscopic devices. [ABSTRACT FROM AUTHOR]

Abstract: Aims of Study: Retinoid-mediated signal transduction plays a crucial role in the embryogenesis of various organs. We previously reported the successful induction of anorectal malformations in mice using retinoic acid (RA). Retinoic acid controls the expression of essential target genes for cell differentiation, morphogenesis, and apoptosis through a complicated interaction in which RA receptors form heterodimers with retinoid X receptors. In the present study, we investigated whether the retinoid antagonist, LE135, could prevent the induction of anorectal malformations (ARMs) in mice. Methods: Retinoic acid was intraperitoneally administered as 100 mg/kg of all-trans RA on E9; and then the retinoid antagonist, LE135, was intraperitoneally administered to pregnant ICR strain mice on the eighth gestational day (E8), 1 day before administration of RA (group B) or on E9, simultaneously (group C) with RA administration. All of the embryos were obtained from the uteri on E18. Frozen sections were evaluated for concentric layers around the endodermal epithelium by hematoxylin and eosin staining. Results: In group A, all of the embryos demonstrated ARM with rectoprostatic urethral fistula, or rectocloacal fistula, and all of the embryos showed the absence of a tail. In group B, 36% of the embryos could be rescued from ARM. However, all of the rescued embryos had a short tail that was shorter than their hind limb. The ARM rescue rates in group B were significantly improved compared to those in group A (P < .01). In group C, 45% of the embryos were rescued from ARM, but all of the rescued embryos had short tail. The ARM rescue rate in group C was significantly improved compared to that in group A (P < .01). However, there was no significant difference in the ARM rescue rate between group B and Group C. Conclusion: The present study provides evidence that in the hindgut region, RAR selective retinoid antagonist, LE135, could rescue embryos from ARM. However, the disturbance of all-trans RA acid was limited to the caudal region. Further study to establish an appropriate rescue program for ARM in a mouse model might suggest a step toward protection against human ARM in the future. [Copyright &y& Elsevier]

Abstract: Background/Purpose: There has not been any study comparing laparoscopic abdominoperineal rectoplasty (ARP) with open ARP. This study investigated the true benefits of the laparoscopic approach in infants with high anorectal malformation. Patients and methods: A retrospective analysis was performed in 28 infants with high anorectal malformation treated between 1990 and 2007. Fifteen were treated by open ARP, and 13 were treated by laparoscopic ARP. Surgical durations, amount of bleeding, complications, anorectal pressure measurements, barium enema study, and clinical assessment were compared between the 2 groups. Results: The amount of intraoperative bleeding was significantly less in laparoscopic ARP (12 ± 11 g) than in open ARP (65 ± 44 g) (P = .003). Anal resting pressure was 34 ± 9 cm H2O after laparoscopic ARP and 31 ± 14 cm H2O after open ARP. Anorectal reflex was positive in 1 (7%) of 15 after open ARP and 3 (23%) of 13 after laparoscopic ARP. There was no significant difference in barium enema study and clinical assessment between the 2 groups. With regard to postoperative complications, mucosal prolapse occurred in 10 (67%) of 15 after open ARP and in none of 13 after laparoscopic ARP (P = .003). Conclusion: Benefits of the laparoscopic approach were reduced intraoperative bleeding and a lower incidence of postoperative anal mucosal prolapse. These results indicate that minimal dissection of the mesorectum in laparoscopic ARP may provide those better outcomes. [ABSTRACT FROM AUTHOR]

Abstract: Background/Purpose: Intraluminal meconium calculi are a rare cause of neonatal abdominal calcifications in patients with anorectal malformations. To investigate their pathogenesis, we performed infrared spectroscopic analysis of meconium-calcified lesions. Methods: Meconium calculi were collected from the colostomy in a newborn patient with imperforate anus and rectourethral fistula. The potassium bromide method was employed to obtain the infrared absorption spectrum of the meconium calculi. Results: The wavelength pattern of the meconium calculi exhibited 4 specific peaks at 1570, 1390, 1105, and 1005 cm−1 between 22% and 45% transmittance values. The unique absorption spectrum exclusively indicated ammonium hydrogen urate (C5N5O3H7), having the combined constituents of ammonium and uric acid. Conclusions: These results suggest that the intraluminal meconium calculi were originally derived from meconium and fetal urine. The stasis of meconium passage and fetal urine mixing through the rectourethral fistula in a low-pH condition was deduced to be the main cause of this rare stone formation. [Copyright &y& Elsevier]

Abstract: Background/Purpose: Tethered spinal cord is frequently associated with anorectal malformations (ARMs). However, it remains unknown how the tethered spinal cord develops and relates to the severity of ARM. We studied the development of the spinal cord in ARM mouse embryos induced by all-trans retinoic acid (ATRA). Methods: Pregnant ICR-Slc mice were administered 100 mg/kg of ATRA on the ninth embryonic day (E9.0). Embryonic specimens were obtained from the uteri between E11.0 and E18.5. Midsagittal histologic sections focusing on the spinal cord and pelvis were prepared for immuonhistochemistry specific for neurofilament and Protein Gene Product 9.5 molecules. Results: More than 98% of ATRA-treated embryos demonstrated ARM with rectourethral or rectocloacal fistula. Normal embryos exhibited progressive ascent of the spinal cord from E14.5. However, in ARM embryos, the distal spinal cord ended with meningomyelocelelike or atypical hamartomatous lesions at E11.5 to E13.5, which later caused stretch force that damaged the spinal cord, resulting in tethered cord between E16.0 and E16.5. Conclusions: In ATRA-induced ARM mouse embryos, tethered spinal cord was mostly established, accompanied by caudal neural maldevelopment, during early fetal development. This experimental model may be useful for researching detailed neuropathologic conditions in ARM children accompanied with tethered spinal cord. [Copyright &y& Elsevier]

Abstract: Multiple endocrine neoplasia type 2B (MEN 2B) is an inherited cancerous syndrome characterized by medullary thyroid carcinoma (MTC), adrenal pheochromocytoma, marfanoid habitus, and enteric ganglioneuromatosis. In this syndrome, a high frequency of persistent elevation of the serum calcitonin level, a sensitive marker for MTC, after total thyroidectomy has been reported, and the prognosis of such patients depends upon complete resection of recurrent MTC by repeated surgery. The authors performed iodine 123 metaiodobenzylguanidine (123I-MIBG) radio-guided navigation surgery for recurrent MTC in a 14-year-old girl with MEN 2B. She had undergone 4 neck operations, including total thyroidectomy at the age of 7 years. An intravenous injection of 100 MBq 123I-MIBG was followed by the fifth surgery. At surgery, the cervical and upper mediastinal areas were filled with adhesional scar tissue, in which a γ-scintillation probe conducted hot spots of isotope uptake by cancerous cells. Histopathology of resected specimens showed scattered nests of MTC cells corresponding to γ-scintillation counts. Intraoperative 123I-MIBG scanning is of substantial benefit for children with MEN 2B undergoing surgery for recurrent MTC. [Copyright &y& Elsevier]

Abstract: Background/Purpose: Recent biological studies have elucidated the molecular mechanism of muscle development, in which various regulatory factors (myogenic regulatory factors [MRFs]) play key roles during embryogenesis. To investigate the development of anorectal malformations (ARMs), we studied MRF expressions in myogenic cells in the pelvic floor using murine embryos affected with ARM. Methods: Anorectal malformation embryos were obtained from the 10.5th embryonal day (E10.5) to the 7.0th postnatal day (D7.0) in a natural mutant strain (Sd/+, RSV/Le). Serial frozen sections were prepared for immunohistochemistry using specific antibodies to M-cadherin, myoD, Myogenin, myosin heavy chain, and alfa-actin molecule. Results: In normal mice, embryonal caudal somites differentiated into myogenic stem cells and migrated to the pelvic floor between E11.0 and E14.0. In the ARM mice, however, caudal somites were irregularly arranged and MRF expressions in myogenic cells were markedly decreased in the dorsocaudal region at E11.5 to E13.0, leading to hypoplastic pelvic floor muscles. Conclusions: The maldevelopment of pelvic floor muscles in ARM is derived from a deficient supply of myogenic stem cells, with impaired MRF expression. These results suggest that myogenic stem cells, available from bone marrow contents, may be used for postnatal muscle regeneration to reinforce the pelvic floor muscle function in children with ARM. [ABSTRACT FROM AUTHOR]

INTRODUCTION: Some children with fecal incontinence respond to biofeedback therapy. However, whether they can achieve fecal continence posttherapeutically has not been clarified. We studied the serial results of biofeedback therapy and discuss the necessity' of providing repeated biofeedback therapy at home. METHODS: Nineteen children with encopresis underwent one session of biofeedback therapy. Seven of 15 children with fecal incontinence that developed after surgery for anorectal malformations underwent three to eight sessions of biofeedback therapy; the remaining 8 underwent one (mean, 2.9) session only. The patients were hospitalized for one session of biofeedback therapy. To monitor the clinical outcome of intervention, we used serial score assessments from three months to two years posttherapeutically. RESULTS: Seventeen of 19 (90 percent) patients with encopresis showed clinical improvement after one session of therapy (P < 0.0001). Six months after treatment, however, six of ten (60 percent) patients with encopresis reported recurrent fecal incontinence after one therapeutic session. Clinical improvement was noted in 5 of 15 (35 percent) patients who had fecal incontinence after surgery for anorectal malformations. All five patients showed clinical improvement from six months to two years after several sessions of biofeedback therapy (P < 0.05). CONCLUSIONS: Biofeedback therapy is effective in most children with encopresis and in some children with anorectal malformations. However, some patients need repeated sessions of biofeedback therapy to achieve fecal continence. Therefore, a new portable biofeedback apparatus for the treatment of fecal incontinence at home may be helpful. [ABSTRACT FROM AUTHOR]

Abstract: Hirschsprung''s disease is an inherited disorder characterized by the absence of ganglion cells in the distal bowel. Neurocutaneous melanosis is a rare congenital syndrome characterized by proliferation of melanin-producing cells in the skin and leptomeninges. The authors described a newborn patient with neurocutaneous melanosis associated with Hirschsprung''s disease. This male baby had congenital hydrocephalus, large and multiple pigmented skin nevi, and severe abdominal distension. He showed marked hydrocephalus at birth and underwent a ventriculo-peritoneal shunt at the age of 5 days. Investigations for gut motility disorders revealed typical findings consistent with Hirschsprung''s disease involving the rectosigmoid colon. He was surgically treated for Hirschsprung''s disease after transanal endorectal pull-through at the age of 7 months. After settlement of the ventriculo-peritoneal shunt, the transanal approach was of significant value for keeping the intraperitoneal catheter clean. The association of developmental disorders of melanocytes and enteric ganglia, both of which originated from the neural crest, suggested the presence of mutual pathogenetic factors in the patient. [Copyright &y& Elsevier]

The authors describe a newborn patient with mesenchymal hamartoma of the chest wall associated with pulmonary hypoplasia. A massive thoracic tumor was diagnosed by prenatal ultrasonography and magnetic resonance imaging at the 28th week of gestation. She was delivered through cesarean delivery at the 36th gestational week. Respiratory distress because of pulmonary hypoplasia necessitated neonatal intensive care. The tumor extensively involved the left hemithorax including all 12 ribs and the first 10 thoracic vertebrae, resulting in marked deformity of the thorax. At 5 days of age, she underwent the incisional biopsy through a left thoracotomy. Histopathology of biopsy specimens showed multiple components of mesenchymal origin including premature cartilage, bone, and cystic lesions resembling aneurysmal bone cyst. The tumor then showed a rapid overgrowth, but subsequently exhibited a self-limited growth for months, in which her respiratory condition gradually improved to spontaneous breathing without oxygenation support. The present case advocates perinatal preparations for associated pulmonary hypoplasia and conservative management for the neoplasm in fetuses prenatally diagnosed as having this unique pathological entity. [ABSTRACT FROM AUTHOR]