Double and synchronous trichobezoars causing small-bowel obstruction and detected by multidetector computed tomography: report of two cases.

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.

Establishment of a rescue program for anorectal malformations induced by retinoic acid in mice.

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.

Laparoscopic versus open abdominoperineal rectoplasty for infants with high-type anorectal malformation.

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 H(2)O after laparoscopic ARP and 31 ± 14 cm H(2)O 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.

Bowel function after surgery for anorectal malformations in patients with tethered spinal cord.

Tethered spinal cord (TC) is an anomaly frequently recognized in association with anorectal malformations (ARM). However, the influence of TC on bowel function in children with ARM remains unknown. Furthermore, there are few studies that have assessed anorectal function in children with ARM and TC. The aim of this study was to evaluate anorectal function in ARM patients with TC using clinical assessment and anorectal manometry. Among 258 patients with ARM, this retrospective investigation included 35 patients who underwent spinal magnetic resonance imaging (MRI) after surgery for ARM. The patients were divided into two groups based on the presence or absence of TC, and bowel function was assessed by Kelly's clinical score and anorectal manometry. Tethered cord was found in nine of the 35 patients (26%) with ARM. Of the ARM patients, TC was noted in four of 11 (36%) with high type anomalies, one of 8 (13%) with intermediate type anomalies, two of 14 (14%) with low type anomalies, and two of two patients (100%) with cloacal anomalies. Kelly's clinical score did not significantly differ between the two groups. However, two of the nine patients with TC had poor bowel function (Kelly's score; 2-0 points). On the contrary, patients without TC did not have poor bowel function. Anorectal manometry did not show a significant difference between patients with and without TC. However, the two patients with TC who had poor bowel function by Kelly's score had low anal resting pressure, which was essential for achieving fecal continence. In conclusion, the present study showed that tethered cord was more frequently found in patients with more severe anorectal anomalies. Patients with TC were more likely to have poor bowel function, but this did not reach statistical significance.

Infrared spectrophotometry of intraluminal meconium calculi in a neonate with imperforate anus and rectourethral fistula.

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 (C(5)N(5)O(3)H(7)), 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.

Developmental study of tethered spinal cord in murine embryos with anorectal malformations.

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.

Iodine 123 metaiodobenzylguanidine radio-guided navigation surgery for recurrent medullary thyroid carcinoma in a girl with multiple endocrine neoplasia type 2B.

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 ((123)I-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 (123)I-MIBG was followed by the fifth surgery. At surgery, the cervical and upper mediastinal areas were filled with adhesional scar tissue, in which a gamma-scintillation probe conducted hot spots of isotope uptake by cancerous cells. Histopathology of resected specimens showed scattered nests of MTC cells corresponding to gamma-scintillation counts. Intraoperative (123)I-MIBG scanning is of substantial benefit for children with MEN 2B undergoing surgery for recurrent MTC.

Neurocutaneous melanosis associated with Hirschsprung's disease in a male neonate.

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.

Impaired expression of myogenic regulatory molecules in the pelvic floor muscles of murine embryos with anorectal malformations.

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.

Respiratory insufficiency in a newborn with mesenchymal hamartoma of the chest wall occupying the thoracic cavity.

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.

Surgical treatment for anomalous arrangement of the pancreaticobiliary duct with nondilatation of the common bile duct.

BACKGROUND/PURPOSE: For anomalous arrangement of the pancreaticobiliary duct (AAPBD) with nondilatation of the common bile duct (CBD), the optimal surgical procedure remains controversial. The authors investigated which procedure would be most effective for AAPBD with nondilatation of the CBD. METHODS: The authors encountered 60 children with AAPBD in our institution between 1979 and 2002. Six of the 60 were classified as the nondilated type (CBD diameter; less than 8 mm), whereas the other 54 were classified as the dilated type (CBD diameter; more than 9 mm). Amylase levels in serum, CBD, and gallbladder were examined. Cellular activity of the resected gallbladder was examined for the incidence of hyperplasia and Ki-67 labeling index (Ki-67 LI). RESULTS: The amylase level in the nondilated type was elevated as in the dilated type. Epithelial hyperplasia of the gallbladder was present in 4 of the 6 with the nondilated type (67%). 10 of the 20 with the dilated type (50%), and none of the 6 controls (0%). The Ki-67 LI of the dilated type was significantly higher than that of control. CONCLUSIONS: A free reflux of pancreatic juice into the biliary system was found regardless of dilatation, and cellular proliferative activity of the gallbladder mucosa was increased in both the nondilated and dilated type. Therefore, excision of the extrahepatic bile duct including cholecystectomy is recommended for AAPBD with nondilatation of the CBD.

[A case of hepatocellular carcinoma with bone metastasis responding to concurrent TS-1/low-dose cisplatin (CDDP) therapy and radiotherapy].

A standard treatment for hepatocellular carcinoma with extrahepatic metastasis is not established and chemotherapy is ineffective. We experienced a case of hepatocellular carcinoma with bone metastasis that responded to concurrent TS-1/low-dose cisplatin (CDDP) therapy and radiotherapy. A 58-year-old male patient with left iliac bone metastasis after 2 hepatectomies was admitted to our hospital. The titer of serum AFP and PIVKA-II showed an extremely high levels, 12,350.5 ng/ml and 993 mAU/ml, respectively. The uptake area was found at the left iliac bone by scintigraphy with 99mTc-HMDP. Treatment with TS-1/low-dose CDDP therapy and radiotherapy (36 Gy) was started concurrently. The chemotherapy regimen comprised daily oral administration of 100 mg of TS-1 for 21 days and CDDP 10 mg/body infusion (day 1-5, 8-12). An additional 2 courses of TS-1/low-dose CDDP therapy were repeated. After that, severe pain diminished and the titer of serum showed AFP and PIVKA-II had improved to within normal ranges. The uptake at the left iliac bone was found to have decreased by scintigraphy. Adverse events were grade 1 nausea and leucopenia. TS-1/low-dose CDDP therapy seems to be applicable for the treatment of hepatocellular carcinoma with bone metastasis.

[A case of colon carcinoma with unresectable multiple liver metastases responding to various combined chemotherapies centering on hepatic arterial infusion therapy].

We have experienced a case of colon carcinoma with unresectable multiple liver metastases responding to various combined chemotherapies centering on hepatic arterial infusion therapy. A 42-year-old female with descending colon carcinoma and synchronous unresectable multiple liver metastases underwent left hemicolectomy in September 2001. She was treated with chemotherapy centering on hepatic arterial infusion (HAI) following implantation of a reservoir in the outpatient setting. Pharmacokinetic modulating therapy (PMC), and HAI with Leucovorin/CDDP/5-FU and Levoforinate/CDDP/5-FU were conducted sequentially. Alternative HAI of 5-FU with intravenous infusion of Levoforinate and systemic irinotecan/CDDP were continued biweekly after release of the hepatic artery occlusion. Liver metastases shrunk after 6 months of treatment and were remarkably decreased in size in May 2003. Although 3 metastatic lesions existed in both lungs, good quality of life has been maintained in the 2 years following surgery.

Sonic hedgehog and bone morphogenetic protein 4 expressions in the hindgut region of murine embryos with anorectal malformations.

PURPOSE: The aim of this study was to determine the possible role of the retinoid-mediated signaling pathway in the pathogenesis of anorectal malformations (ARM). The authors investigated whether all-trans retinoic acid (ATRA) affects the expression pattern of Sonic hedgehog (Shh) and Bone morphogenetic protein 4 (BMP4), which play important roles in anorectal morphogenesis in vertebrates. METHODS: Pregnant ICR strain mice were fed 100 mg/kg of ATRA on the ninth gestational day (E9). Embryos with or without administration of ATRA were obtained from the uteri between E12 and E16 and were fixed immediately in a 4% paraformaldehyde solution. Frozen sections were evaluated for concentric layers around the endodermal epithelium by H&E and immunohistochemistry using antibodies created specifically to act against Shh and BMP4. RESULTS: More than 95% of the embryos administered ATRA had ARM; rectoprostatic urethral fistula, rectocloacal fistula, and short tail were the most frequent anomalies in the mouse embryos. On E14, normal mouse embryos had normal rectum and anus in which the epithelium of the anorectum was positive for Shh, and the mesenchyme was positive for BMP4. In the ARM embryos, however, the epithelium of the anorectum was negative for Shh, and the mesenchyme was also negative for BMP4. CONCLUSIONS: In normal hindgut development, Shh from the epithelium induces BMP4 expression in the mesenchyme, which differentiates into the lamina propria and the submucosa. In ARM embryos, expressions of Shh and BMP4 could not be found in those regions of the hindgut. Therefore, these findings indicate that Shh and BMP4, which appear to play a crucial role in organogenesis of the hindgut, were disturbed in the cell signaling pathway between the epithelium and the mesenchyme layers.

Results of biofeedback therapy for fecal incontinence in children with encopresis and following surgery for anorectal malformations.

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 (33 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.

Giant cystic lymphangioma of the small bowel mesentery: report of a case.

We herein describe the case of a 48-year-old man who presented to our hospital with abdominal distension and pain. Preoperative studies including abdominal ultrasonography and computed tomography failed to determine the cause of the pain. At laparotomy, a giant cystic tumor of the small bowel mesentery was found. Histologically, the tumor was diagnosed as a cystic lymphangioma. Although mesenteric lymphangiomas are rare, especially in adults, they should be considered as a possible cause of acute abdomen.