Persistent biliary dilatation and stenosis in postoperative congenital choledochal cyst.

BACKGROUND/PURPOSE: Cholangitis and intrahepatic stones occur long after total cyst excision in patients with congenital choledochal cyst (CCC). Our study aimed to characterize morphological features of intrahepatic biliary dilatation and stenosis before and after total cyst excision, based on long-term follow-up data. METHODS: Pre- and postoperative morphological features of intrahepatic biliary dilatation were determined in 63 patients with CCC. RESULTS: Postoperatively, hepatic ductal dilatation persisted in 50 patients and hepatic ductal dilatation with stenosis in 35 patients. Hepatic duct stenosis was seen in 35 patients: unilateral hepatic duct stenosis in 21, and bilateral stenosis in 14. Stenosis at the confluence of the right and left hepatic ducts occurred more often in the cystic type of dilatation than in the cylindrical type and was seen more often on the left side than the right. Cases with postoperative cholangitis or intrahepatic stones featured stenosis at the confluence of left and both hepatic ducts (n = 2); and alternating dilatation and stenosis of left hepatic ducts and branches (n = 3). However, no statistical associations were observed between the hepatic ductal stenosis and cholangitis or stone formation (P = 0.153). CONCLUSIONS: Cystic-type biliary dilatations persist postoperatively, frequently accompanied by ductal stenosis. Alternating dilatation and stenosis is a common morphological feature for postoperative cholangitis and stones.

Pathophysiology of chronic constipation of the slow transit type from the aspect of the type of rectal movements.

BACKGROUND/AIMS: The aim of this study was to analyze the defecation function, in particular the development of the gastrocolic reflex arising in coordination with the upper gastrointestinal tract, in patients with chronic constipation of the slow transit type (STC). METHODOLOGY: The rectal movement types in adult patients with STC were compared with those in normal subjects as a control. A force transducer was inserted transanally into the rectum of 10 patients with STC (Group A), and the intrarectal pressure waveforms were recorded for 2 hours before and after the ingestion of test diets. Similar recordings were obtained from control subjects who had no abnormalities of defecation (Group B). RESULTS: On the basis of the recordings obtained pre- and postprandially, the waveforms in the Group B subjects were classified into 4 types (Type I, continuous waves of low amplitude; Type II, continuous waves of high amplitude or with elevation of basal tone; Type III, only elevation of the basal tone; Type IV, monophasic contraction waves). In fasting state, only Type I waves were recorded in both Group A and B subjects. Postprandially, all the subjects in both the groups showed Type I waveforms. In addition, the frequency of this type of waveform was significantly increased in Group B (p < 0.01). Type III and IV waveforms were also recognized only in Group B. There was no difference in the frequency of Type II waveforms between Groups A and B, but the time required for the appearance of Type II waveforms after ingestion of the test diet was significantly (p < 0.01) shorter in Group B than in Group A. Thus, there were no differences in the preprandial movement type between the 2 groups. However, postprandially, in Group A, the appearance of continuous waves of high amplitude was delayed, although their frequency was not significantly increased, as compared to those in Group B, and there were few giant contraction waves. CONCLUSIONS: Functional disturbances of the rectal movements were recognized after food ingestion in patients with STC, and this result was considered to be attributable to decreased strength of the gastrorectal reflex.

A rare case of multiple duodenal perforations in early infancy.

Duodenal perforation in early infancy is an uncommon condition. We describe a case of duodenal perforation from suspected ulcer. A premature boy was born at the gestational age of 26 weeks with a birth weight of 764 g. The Apgar score at 1 min was 3 and at 5 min had decreased to 2. He was given intermittent mandatory ventilation for one month after the birth. Ninety-eight days after birth, the infant's abdomen became distended. A supine and cross-table lateral radiograph of the abdomen revealed massive pneumoperitoneum. An exploratory laparotomy was performed, which revealed two perforations in the anterior wall of the first portion of the duodenum. The operation procedure was direct closure and intra-abdominal drainage. On the postoperative first day, he had central urorrhagia from hematencephalon. The patient's growth after surgery has been normal, with no recurrence of duodenal ulcer.

Effect of FTY720 and ex vivo graft irradiation in rat small bowel transplantation: expression of mucosal addressin cell adhesion molecule-1.

PURPOSE: We performed a semiquantitative analysis of the expression of Mucosal addressin cell adhesion molecule-1 (MAdCAM-1) and gut-associated tissues (GALT) during small bowel graft rejection in the rat to confirm the effect of FTY720 and ex vivo graft irradiation. METHODS: Small bowel transplantations (SBT) were performed from BN rats to LEW rats. Four groups of SBT animals were studied on days 3, 5, and 7 after operations (untreated, FTY720, ex vivo graft irradiation, FTY720+ex vivo graft irradiation). Indirect immunoperoxidase staining was performed against CD4 and MAdCAM-1. The number of CD4-positive cells in the allografts was also analyzed by flow cytometry. RESULTS: The graft survival was prolonged only in the FTY720-treated groups. The SBT allografts treated by FTY720 demonstrated less infiltration, but the ex vivo graft irradiation group did not show any effect on its expression. In the FTY720-treated groups, MAdCAM-1 expression on high endothelial venules (HEVs) in Peyer's patches (PPs) was upregulated and its expression on endothelial cells of vessels in the lamina propria was downregulated in comparison with the allograft group without FTY720. CONCLUSIONS: It is important to prevent the infiltration of CD4-positive cells, the downregulation of MAdCAM-1 expression on HEVs in PPs and the upregulation of MAdCAM-1 expression on endothelial cells of vessels in the lamina propria for the prolongation of graft survival.

Usefulness of color doppler sonography and 99m Tc-RBC scintigraphy for preoperative diagnosis of a venous malformation of the small intestine in a 2-year-old child.

Venous malformations of the small intestine are rare in children, and the preoperative diagnosis of a venous malformation in the small bowel can be very difficult. We report the case of a 2-year-old girl with a solitary cavernous venous malformation of the small intestine that caused gastrointestinal bleeding and anemia and review the usefulness of the combination of color Doppler sonography and 99m Tc-RBC scintigraphy for the diagnosis of venous malformation of the small intestine.

Intraoperative radiation therapy for advanced neuroblastoma: the problem of securing the IORT field.

The purpose of this study is to evaluate the efficacy of intraoperative radiation therapy (IORT) and the problem of securing the IORT field in advanced pediatric neuroblastoma. Between 1996 and 2005, 12 children received IORT for advanced pediatric neuroblastoma patients. Electron beam energies ranged from 10 to 12 MeV and median dose was 10 Gy (8-12 Gy). All of them had surgery with IORT against the primary tumor site and the abdominal aorta surroundings. A gross total resection (GTR) was achieved in 10 patients and subtotal resection (STR) was two patients. All of 12 patients were classified as high risk. Nine patients were alive 17-120 (mean 48 months) after diagnosis. Local tumor control was achieved in 100% of patients, of whom one experienced local recurrence outside the IORT field. At the operation, it was difficult to secure the IORT field because of the angle of the radiation cylinder in three patients. One of the three of these patients experienced local recurrence outside of the IORT field in the upper side of superior mesenteric artery and two of three patients had an external beam radiation after surgery, and there was no local recurrence. One patient had a postoperative ileus, and one patient had transient diarrhea and hydronephrosis. For advanced neuroblastoma patients, IORT produced excellent local control after surgery. However, there is a problem of securing the IORT field. For local control, it is necessary to add an external beam radiation after IORT when it is difficult to secure the IORT field.

Treatment of fistula-in-ano in infants with a seton.

Anal fistula in infants is a common disease. Although many are recovered by conventional treatment, there are some patients who are not repaired easily. We performed the seton method to an anal fistula and report the good result that we obtained.

Intussusception in children of school age.

BACKGROUND: There are only a few reports discussing the characteristics of intussusception developing in school-age children. The characteristics of these cases are discussed, with reference to previous literature. METHODS: The present study included eight cases of intussusception in school-age children among 143 intussusception patients treated on an inpatient basis at Nihon University Itabashi Hospital, during the 11 year period from 1993 to 2003. The remaining 135 patients were assigned to the infant group as controls. The clinical characteristics of intussusception in school-age children were compared with those of the condition developing in infants. RESULTS: The eight children of school age with intussusception ranged in age from 8 to 15 years (mean, 11.6 years), and consisted of five boys and three girls. The major symptom was abdominal pain, occurring in 100% (8/8). Bloody stools and vomiting were reported in two patients each (25%) from this group. The triad of abdominal pain, bloody stools and vomiting was recognized in only one child (12.5%) of this group. Two children (25.0%) had a palpable abdominal mass, and one child (12.5%) complained of diarrhea. None of the school-age children with intussusception had any antecedent infection; five, two and one patients had the ileo-colic type, ileo-ileo-colic type and ileo-ileal type of intussusception, respectively. Four underwent enema reduction and four underwent surgical reduction. One of the eight children (12.5%) had underlying organic abnormality; in the remaining children the condition was labeled idiopathic. One child developed recurrences. CONCLUSIONS: In school-age children intussusception is generally believed to be commonly secondary to underlying organic abnormality, but in the present study only one of eight school-age children had underlying organic abnormality; in the remaining children, the condition was labeled idiopathic. The major symptom in school-age intussusception was abdominal pain. Therefore this may need to be differentiated from appendicitis in children of school age. It is considered that abdominal ultrasonography (USG) is a simple and useful method for making the diagnosis of intussusception, and that diagnostic USG should be conducted in all school-age children presenting with acute abdominal pain.

Study of 24 cases with congenital esophageal atresia: what are the risk factors?

BACKGROUND: Recovery from esophageal atresia (EA) and tracheoesophageal fistula (TEF) has improved markedly over the years. But postoperative complications, however, have remained. This study evaluates recovery, preoperative, and postoperative status of patients with EA/TEF. METHODS: A retrospective study review was undertaken in 24 patients with EA/TEF after primary anastomosis (January 1975 through September 2003). RESULTS: There were no patients who had major cardiac anomalies or trisomy 18. In total, 17 of 24 (70.8%, group A) patients have survived and seven (29.2%, group B) have died. Birthweight and Apgar Scores in group A were significantly higher than in group B. The ratio of GAP (the distance of the location of the blind pouch from the ends of the upper and lower esophagus) to body length in group B was significantly higher than in group A. The birthweight and Apgar Scores in group A were significantly higher than in group B. When the authors compared their sample of cases by means of the Waterston classification, the Montreal classification and the Spitz classification, there were statistically significant differences between the results using the Waterston classification and the results using to the Spitz classification. CONCLUSION: For the cases of EA surgery that were examined, the authors concluded that bodyweight at birth and the existence of pre-surgery respiratory system complications have a significant effect on post-surgery recovery, and that results appear to indicate the importance of classification using the Waterston classification and Spitz classification as a means of assessing the degree of risk. Results also appeared to indicate that the control of Respiratory Distress Syndrome throughout both the pre-surgery and post-surgery periods is critical.

Spontaneous localized intestinal perforation and intestinal dilatation in very-low-birthweight infants.

AIM: To elucidate how spontaneous localized intestinal perforation (SLIP) is related to intestinal morphological features such as dilatation in very-low-birthweight (VLBW) infants. METHODS: The medical records of 13 VLBW infants (<1500 g) undergoing laparotomy between 1983 and 2003 for presumed SLIP were retrospectively reviewed. Clinical findings including maternal, prenatal and perinatal factors were analysed, and the clinical and surgical findings upon laparotomy were compared. RESULTS: Postnatal pathological conditions included patent ductus arteriosus (n=7), sepsis (n=2), respiratory distress syndrome (n=7), intraventricular haemorrhage (n=2), an indwelling catheter via the umbilical vein (n=1) and pneumonia (n=1). Indomethacin was used in seven neonates with patent ductus arteriosus, and dexamethasone preventive therapy was employed in one neonate for bronchopulmonary dysplasia. Operative findings revealed a localized small punched-out perforation in the ileum. Five patients had intestinal dilatation: two with a perforation in the middle of the dilated intestine, and three with a perforation proximal to the region of dilatation. The muscularis propria was absent in the dilated intestine of four patients. CONCLUSION: This study found no significant relationship between perforation and dilatation of the intestine. Perforation may occur in any portion of the ischaemic intestine when circulatory failure becomes severe, and is not necessarily restricted to the dilated intestine. We believe that SLIP and intestinal dilatation may occur on the same basis in low-birthweight infants; however, the disease process may be aetiologically different.

Pancreatitis after a primary and secondary excision of congenital choledochal cysts.

PURPOSE: Pancreatitis has been reported long after total choledochal cyst excision. The aim of this study was to determine if the disease process of postoperative pancreatitis differs between a primary and secondary cyst excision in a long-term follow-up. METHODS: Among 53 postoperative patients who underwent a total cyst excision and were followed up, 44 patients underwent a primary cyst excision (primary excision group), while 9 patients underwent a secondary cyst excision after a previous cyst-duodenostomy for internal drainage (secondary excision group). The long-term clinical course, including the pancreatographic findings after a total cyst excision, was compared. RESULTS: In the primary excision group, six patients had mild pancreatitis. Endoscopic retrograde pancreatography demonstrated ductal dilatation that was limited to the common channel in two patients, concurrent with the ventral duct in three, and extended the duct of Santorini in three. Conservative treatments were carried out in three patients, and endoscopic irrigation in one patient with protein plugs in the ventral duct. A resection of the choledochal remnant in the pancreas was performed in two patients with choledochal remnant-associated pancreatitis. From the secondary excision group, 5 of the 9 patients had chronic pancreatitis. Endoscopic retrograde pancreatography showed entire pancreatic ductal dilatation. Two of these patients underwent duodenal papilloplasty at the same time as secondary surgery; however, the disease progressively worsened. CONCLUSION: In patients undergoing a secondary total excision after internal drainage, it is difficult to half the ongoing aggravating process in pancreatitis.

Pancreatitis complicated with dilated choledochal remnant after congenital choledochal cyst excision.

We describe here three cases of pancreatitis after congenital choledochal cyst excision. In these three cases, the choledochal remnant in the pancreas head was markedly dilated, probably because of an incomplete resection of the cyst at the primary operation, and an increase in intraluminal pressure of the pancreatic duct caused by a dynamic obstruction by a protein plug or a pancreatic calculus. Complete cyst excision, including the choledochal wall in the pancreas, is therefore strongly recommended.

The effect of a novel immunosuppressant, FTY720, in mice without secondary lymphoid organs.

PURPOSE: FTY720 is a novel immunosuppressive agent that is thought to reduce the number of peripheral blood lymphocytes (PBL) by directing them toward secondary lymphoid organs such as the lymph nodes and Peyer's patches. We studied the effects of FTY720 on aly/aly mice that do not have either lymph nodes or Peyer's patches, as well as on splenectomized aly/aly mice. METHODS: FTY720 was orally administered by gavage (1 mg/kg) to aly/aly mice as well as to aly/+ mice with and without a splenectomy on 14 consecutive days. The number of lymphocytes was then counted using True Cell beads and flow cytometry. The number of B220-, CD3-, and CD4-positive cells was also determined. In addition, skin grafts from C3H donor mice were performed on these mice. RESULTS: FTY720 was effective in significantly reducing the total lymphocyte count as well as the B220-, CD3-, and CD4-positive subtypes in the peripheral blood of aly/+ mice as well as in aly/aly mice with and without a splenectomy. While we did observe allograft skin graft rejection in both the aly/+ mice as well as the aly/aly mice recipients and splenectomized aly/aly mice, the graft survival was prolonged in all groups. The skin allografts treated by FTY720 thus demonstrated fewer lymphocytic cells and less infiltration of CD4-positive cells. CONCLUSIONS: The administration of FTY720 to mice without lymph nodes, Peyer's patches, or spleens still results in peripheral lymphopenia. In all groups, FTY720 was found to prevent the infiltration of CD4-positive cells in skin allografts while also prolonging skin allograft survival. The fate of these lymphocytes, however, is unclear.