Miwako Nakano

Long-term results of jejunal replacement of the esophagus

Since 1969, jejunal interpositions have been carried out in 19 patients for esophageal replacement. A segment of the upper jejunum was used to bridge the gap in the right thoracic cavity. The distal esophagus and its sphincteric mechanism was preserved in all but four patients, who had peptic strictures. Early postoperative complications such as total necrosis of the graft, perforation of the graft, and anastomotic leak developed in three patients (16%). There were no operative deaths but there were two later deaths (11%). We were able to follow 12 patients over a long term. Among these 12 patients there were two anastomotic strictures, one of which was dilated successfully by bouginage, and one marked redundancy of the jejunum which necessitated surgical correction. Both height and weight were lower than -2 SD on a Japanese standard growth curve in two patients who had anastomotic strictures. Transient stagnation of swallowed barium at the lower esophagus was the common finding; it was observed in seven cases (58%). Only three patients (25%) complained of occasional feelings of delay in swallowing. Stagnation with a mildly redundant jejunum was the common radiologic finding in these three patients. Reflux of the gastric content into the esophagus did not occur. All the patients, except one who still has dysphagia due to anastomotic stricture, can eat anything they wish at almost normal speed. These long-term results indicate that jejunal interposition with preservation of the lower esophagus is a recommendable procedure for esophageal replacement.

Extended dissection of the portahepatis and creation of an intussuscepted ileocolic conduit for biliary atresia.

This paper introduces a new operation for biliary atresia that establishes successful biliary drainage by extending the portahepatic dissection, and decreases ascending cholangitis by incorporating a nonrefluxing intussuscepted draining conduit. An analysis of the postoperative results is presented. The usual dissection of the portahepatis is extended to include the area between and beneath the branches of the right portal vein to incorporate all potentially usable remnants of the intrahepatic ducts. Ascending cholangitis is decreased by interposing an intussuscepted ileocolic segment between the portahepatis and the abdominal wall. This extended dissection of the portahepatis was performed since 1978 in 15 infants with noncorrectable biliary atresia, and bile drainage was achieved in all. In ten infants an intussuscepted ileocolic conduit was constructed. Ascending cholangitis in these ten infants has been either completely absent or easily controlled by antibiotics. The draining bile was highly concentrated due to the water-absorbing capacity of the interposed colonic segment; therefore, fluid and electrolyte disturbances, which develop frequently in patients having jejunal conduits, have never been encountered.

Congenital absence of the portal vein in a boy.

Abstract Congenital absence of the portal vein (CAPV) is a malformation that is generally thought to be limited to females. We encountered an 11-year-old boy with this malformation. In 17 previously reported cases of CAPV, 2 were male. Three male patients, including our case, were Abernethy type Ib malformation. They had no associated liver tumour and two had no additional congenital abnormality. Conversely, 13 of the 15 female patients had congenital abnormalities and 6 had liver mass lesions.

Clinical application of minimal residual neuroblastoma cell detection by reverse transcriptase-polymerase chain reaction

The highly sensitive method to detect neuroblastoma (NB) cells using reverse transcriptase polymerase chain reaction (RT-PCR) was applied in the practical clinics, and its efficacy was assessed in the present study. Human tyrosine hydroxylase (TH), a rate-limiting enzyme in the catecholamine biosynthesis, was used as the marker for NB cells, and the expression of THmRNA was examined in 13 samples (four peripheral blood and nine bone marrow) harvested from seven patients (four with stage IV, one with stage III, two with stage II) using RT-PCR with our original primers. The positive signals for NB cells were detected in four samples (one peripheral blood and three bone marrow) by the PCR method, but were undetectable by the conventional histological examinations. In the present series, a case that showed a positive signal for NB cells in the peripheral blood showed a remarkably unfavorable clinical course, indicating that the circulating NB cells detected by the PCR method can be a sign of the progressively advanced NB, and may define a new prognostic factor suggesting higher risk. In another case, the PCR detection for the residual NB cells in the bone marrow provided important supporting evidence to determine the necessity of the additional chemotherapy and the suitable timing for bone marrow transplantation. This detection also guaranteed the safety of the bone marrow for transplantation. The PCR method was considered to be very beneficial in the selected cases. However, some problems such as the false-negative results in the negative urinary vanillylmandelic acid secretor were also highlighted in the present study.

An investigation on the mechanism of contralateral manifestations after unilateral herniorrhaphy in children based on laparoscopic evaluation.

BACKGROUND/PURPOSE No straightforward theoretical descriptions explaining the discrepant incidences of metachronous contralateral hernia and positive contralateral patent processus vaginalis (cPPV) exist. The indications for contralateral groin repair are still not clearly defined. METHODS During laparoscopic herniorrhaphy, the intraoperative appearances of the internal inguinal rings (IIRs) were characterized and classified in 211 children with symptomatic unilateral inguinal hernias. A total of 125 boys and 86 girls with 130 right-sided and 81 left-sided indirect inguinal hernias were examined. The morphological appearances of the IIRs in patients with cPPV were compared before and after the ligation of the symptomatic IIRs. RESULTS Of 211 children, 108 (51%) had positive cPPV findings. The cPPVs had significantly smaller orifices and shorter lengths than those on the side of the symptomatic hernia. Among these children, 24 (22%) patients exhibited morphological changes in their cPPVs, characterized by an upward movement of the overlying peritoneal veil after the closure of the symptomatic hernia. CONCLUSION Metachronous contralateral hernia might occur because of morphological changes to the cPPV resulting from closure of the symptomatic IIRs. The predicted incidence of contralateral hernia in this series was 11.2%.

Effectiveness of an intussusceptive antireflux valve to prevent ascending cholangitis after hepatic portojejunostomy in biliary atresia

An intussusceptive antireflux valve was created in the Roux-en-Y loop at the same time as the hepatic portojejunostomy (Kasai procedure) for the purpose of preventing ascending cholangitis in 14 new cases of biliary atresia and in 6 long-term survivors of the Kasai operation after episodes of ascending cholangitis. Ascending cholangitis did not develop in any of the 13 new patients who showed bile outflow following a modified Kasai procedure with antireflux valve nor in 5 of 6 patients who had secondary valve implementation. Postmortem examination of a patient with a valve who died 2 years 3 months after surgery showed that the structure of the valve was intact and that the valve still was quite effective as an antireflux mechanism.

Delayed puberty in girls having biliary atresia

Biliary atresia patients have several problems even after undergoing successful Kasai operation. Fourteen female patients have been followed for over 12 years after successful Kasai original portoenterostomy procedures. The oldest patient is 21 years of age. All patients are jaundice-free, but 10 patients have a history of esophageal varices and/or hypersplenism. Five patients complain of menstrual disorders. The average age of menarche was delayed 1 year, 9 months, compared with Japanese controls. Two cases of 11 (18.2%) presented primary amenorrhea after 14 years, 6 months, which is mean + 2SD of Japanese controls. Four cases of 11 (36.4%) showed secondary amenorrhea. Unestablished menstrual cycles after 2 years of menarche were observed in two patients of nine (22.2%). Four cases had regular menstruation. Five patients showed delay in the development of pubic hair and breasts. Six patients showed atypical body height velocity pattern that showed no peaking, and one patient showed no growth spurt. All the patients with amenorrhea had portal hypertension. In hormonal evaluation, the patients with menstruation showed normal or slightly good reaction of luteinizing hormone and follicle stimulating hormone in the luteinizing hormone-releasing hormone test, having normal to high estradiol levels. The patients with menstrual abnormality showed overreaction of LH, having normal estradiol levels.

Diagnosing native liver fibrosis and esophageal varices using liver and spleen stiffness measurements in biliary atresia: a pilot study

BackgroundBiliary atresia commonly leads to liver fibrosis and cirrhotic complications, including esophageal varices.ObjectiveTo evaluate liver and spleen stiffness measurements using acoustic radiation force impulse (ARFI) imaging for diagnosing grade of liver fibrosis and predicting the presence of esophageal varices in patients treated for biliary atresia.Materials and methodsARFI imaging of the spleen and native liver was performed in 28 patients with biliary atresia. We studied the relation between ARFI imaging values and liver histology findings (n=22), upper gastrointestinal endoscopy findings (n=16) and several noninvasive test results. Diagnostic accuracy was assessed using receiver operating characteristic curve analyses.ResultsLiver stiffness measurements exhibited a significant difference among the different grades of liver fibrosis (P=0.009), and showed higher values in patients with high-risk esophageal varices than in the other patients (P=0.04). The areas under the receiver operating characteristic curves of liver stiffness measurements for liver fibrosis grades  ≥ F2, ≥F3 and = F4 were 0.83, 0.93 and 0.94, respectively. Patients with high-risk esophageal varices were preferentially diagnosed by the combined liver and spleen stiffness measurements (area under the curve, 0.92).ConclusionLiver and spleen stiffness measurements using ARFI imaging are potential noninvasive markers for liver fibrosis and esophageal varices in patients treated for biliary atresia.

Sacroperineal anorectoplasty using intraoperative ultrasonography: Evaluation by computed tomography

A new operative technique that uses ultrasonography during sacroperineal or sacroabdominoperineal anorectoplasty was performed on seven patients with an intermediate or high anorectal malformation. Ultrasonography was used for the purpose of making a pull-through tunnel under visual guidance. The results of this technique were evaluated by computed tomography scanning to find the anatomic position of the pulled-through rectum in relation to the anal sphincter muscle. Postoperative computed tomography scans showed that the rectum was correctly placed in the center of the sphincter muscle in all patients.

Neonatal intestinal volvulus and preduodenal portal vein associated with situs ambiguus: Report of a case

Situs ambiguus is a rare lateralization anomaly that is frequently associated with other malformations, including preduodenal portal vein (PDPV), intestinal malrotation, and cardiovascular anomalies. This is a case report on a newborn that was clinically diagnosed with situs ambiguus and midgut volvulus. During surgery the patient was found to have intestinal malrotation, Meckel’s diverticulum, and PDPV that was not a direct cause of duodenal obstruction. The patient was treated with Ladd’s procedure and resection of Meckel’s diverticulum. It is important to be familiar with the spectrum of situs anomalies to prevent trauma to the portal vein with serious complications during surgery.