Isolated intestinal neuronal dysplasia Type B (IND-B) in Japan: results from a nationwide survey.

PURPOSE: Intestinal neuronal dysplasia Type B (IND-B) has been proposed to be an allied disorder of Hirschsprung's disease (ADHD). The original histological criteria included hyperganglionosis, giant ganglia, ectopic ganglion cells and an increased AChE activity in the lamina propria. The criteria for IND-B have been gradually revised. The present diagnostic criteria are [1] more than 20 % of the submucosal ganglia contain nine or more ganglion cells and [2] the patient is older than 1 year. To clarify the current status of IND-B in Japan, a nationwide retrospective cohort study was performed. METHODS: Questionnaires were sent to 161 major institutes of pediatric surgery and gastroenterology in Japan. RESULTS: A total of 355 cases of ADHD were collected, including 18 cases of IND-B (5 %). Based on original criteria, 13 out of 18 cases were diagnosed as IND-B. However, only four cases met the current criteria. Three of the four patients (75 %) required pull-through operation. All of the patients exhibited giant ganglia and ganglioneuromatosis-like hyperplasia of the myenteric plexus. CONCLUSIONS: IND-B cases matching the current criteria are thought to be quite rare and they are associated with marked hyperplasia of the myenteric plexus. "True" IND-B is a rare and intractable disease.

[Combined operation for congenital cardiovascular and other anomalies in children].

Combination of congenital cardiovascular and other anomalies are often found. Combined operation is needed for some of these cases. We report 3 cases who underwent combined operations by combined team of cardiovascular and general surgical units. Case 1: A 10-month-old boy with VATER association was complicated with tetralogy of Fallot, double aortic arch and so on. He underwent division of left aortic arch for vascular ring by cardiovascular surgical unit and esophagogastrostomy for esophageal atresia by general surgical unit. Case 2: A 15-day-old girl with patent ductus arteriosus and eventration of the diaphragm underwent ligation of ductus arteriosus by cardiovascular surgical unit and plication of left diaphragm by general surgical unit. Case 3: An 8-month-old boy with tracheobronchomalacia and atrial septal defect underwent atrial septal defect (ASD) closure by cardiovascular, and extra tracheobronchial stenting on cardiopulmonary bypass by cardiovascular and general surgical unit. These 3 operations were successful, and we consider that combined operation is useful for certain surgical congenital multiple anomalies.

Should all hepatic arterial branches be reconstructed in living-related liver transplantation?

BACKGROUND: Because graft arteries are smaller and shorter in living-related liver transplantation (LRLT) than in whole or reduced-size liver transplantation from cadavers, arterial reconstruction is thought to be one of the critical points for success. METHODS: Thirty LRLT patients were classified into two groups: those in whom all graft hepatic arteries were reconstructed (group A), and those whom only had some were reconstructed (group B). In group A 17 patients had a single hepatic artery and three had two hepatic arteries. In group B the thickest one of several arteries was reconstructed, but the others were ligated after pulsatile back-bleeding from their cut stumps had been confirmed. The clinical results were compared between the two groups. RESULTS: Neither arterial thrombosis nor liver dysfunction related to the arterial blood supply was observed during the postoperative course. One case of bile leakage and two cases of bile duct stenosis occurred in group A. No significant difference was noted in the postoperative values of aspartate aminotransferase, alanine aminotransferase, and lactate dehydrogenase between the two groups. Overall patient and graft survival was 90%. CONCLUSIONS: Although several hepatic arteries may supply the potential allograft in LRLT, it is not always necessary to reconstruct all of them.

Evaluation of operative stress and peritoneal macrophage function in minimally invasive operations.

BACKGROUND: Laparoscopic operative procedures have decreased postoperative pain and the length of hospitalization. In addition, evidence supports a physiologic benefit from laparoscopic surgery. By analyzing several parameters of peritoneal macrophage function, we report a comparison of the magnitude of postoperative stress between two types of minimally invasive access techniques contrasted with an open laparotomy, in a murine model. STUDY DESIGN: Immature male A/J mice were exposed to pneumoperitoneum using carbon dioxide, gasless suspension, or laparotomy. Peritoneal macrophages were then harvested, and the number and viability of the macrophages from each group of mice were compared. Last, as a marker of postoperative stress, the in vitro production of nitric oxide and tumor necrosis factor alpha by these macrophages was determined. RESULTS: The number of peritoneal macrophages and the viability of the macrophages in the laparotomy group were significantly decreased 4 hours after operation compared with the minimally invasive and control groups. In addition, macrophage production of tumor necrosis factor alpha and nitric oxide, two markers of macrophage stress, 24 hours after operation was significantly increased in the laparotomy group compared with animals serving as controls. Gasless suspension and pneumoperitoneum decreased the number of macrophages to a lesser degree than did open laparotomy and did not affect macrophage viability. Moreover, gasless suspension and pneumoperitoneum did not lead to an increase in tumor necrosis factor alpha or nitric oxide production by peritoneal macrophages. CONCLUSIONS: Postoperative stress, assessed by a decrease in macrophage viability and an increase in cytotoxic cytokine production, is maximized after laparotomy compared with stress in murine hosts that underwent minimally invasive treatment. These data provide basic scientific evidence for the possible physiologic benefit of minimally invasive techniques.

Surgical treatment for abdominal neuroblastoma in the laparoscopic era.

BACKGROUND: The role of laparoscopy in children with cancer has not been fully defined. The aims of this study were to develop an optimal surgical procedure for the treatment of abdominal neuroblastoma in the laparoscopic era and to evaluate the advantages and disadvantages of laparoscopy in the pediatric population. METHODS: Since July 1997, 37 children were diagnosed with abdominal neuroblastoma at our center, and 44 surgical procedures were performed on them. Patients with advanced neuroblastoma underwent laparoscopic biopsy, open biopsy, and delayed primary or second-look excision, whereas early neuroblastoma cases had either laparoscopic or open excision. We compared the length of the operation, intraoperative blood loss, length of hospital stay, complications, and time to start postoperative feeding and chemotherapy for the laparoscopic and open surgery groups. RESULTS: Length of stay and time to postoperative feeding and chemotherapy were significantly lower in the laparoscopic group than the open surgery group. However, there were no significant differences between the two groups in length of operation and intraoperative blood loss. CONCLUSION: Laparoscopic biopsy and excision of abdominal neuroblastoma are effective and efficient surgical procedures in children.

Challenges of laparoscopic resection of abdominal neuroblastoma with lymphadenectomy. A preliminary report.

BACKGROUND: The laparoscopic procedure involving total resection of abdominal neuroblastoma combined with lymphadenectomy has not been reviewed in English literature. The aim of this study was to evaluate the significance and accuracy of laparoscopic resection of abdominal neuroblastoma. METHODS: Since July 1997, five patients with abdominal neuroblastoma underwent laparoscopic resection combined with lymphadenectomy or sampling of the lymph nodes. The length of operation, intraoperative blood loss, resectability, and complications were retrospectively reviewed and evaluated. RESULTS: Four cases were managed laparoscopically, but one case was converted to open procedure because of poor visualization around large vessels. The mean operation time was 135 min and the intraoperative blood loss 52 ml. CONCLUSIONS: Good visualization of the primary tumor and large vessels is, arguably, the most important factor for successful completion of this procedure laparoscopically. Precise indicators for laparoscopic resection of abdominal neuroblastoma provide a better prognosis and a good quality of life for children with neuroblastoma.

Findings of pelvic musculature and efficacy of laparoscopic muscle stimulator in laparoscopy-assisted anorectal pull-through for high imperforate anus.

BACKGROUND: Laparoscopic findings of levator muscle and the efficacy of laparoscopic muscle stimulator (LMS) in infants with high imperforate anus have not been reported. METHODS: Twelve patients underwent laparoscopically assisted anorectoplasty for high imperforate anus. Following laparoscopic dissection of the distal rectum and division of the fistula, levator muscles in the pelvic floor were stimulated with a 5-mm-diameter LMS. Dilatation was done by inserting a guidewire and balloon catheter through the center of the levator muscle sling and muscle complex. Rectal pull-through and anastomosis between the rectum and anus were successfully completed. RESULTS: LMS showed good contraction of levator muscles and enhanced accurate midline placement of pull-through rectum. LMS was particularly useful in observing weak muscles in infants with rectovesical fistula. CONCLUSIONS: Laparoscopy and LMS offer excellent visualization of the pelvic musculature and precise tract of rectal pull-through. Fecal continence will be assessed by long-term follow-up.

Surgical management of piriform sinus fistula.

Four cases of acute cervical abscess resulting from infection through the left piriform sinus fistula are described. The fistula seems to be related to the cause of infection in many of previously reported cases of acute suppurative thyroiditis or "recurrent lateral cervical fistula." Complete removal of the fistula is essential to a permanent cure, and the guide of a Fogarty catheter through the fistula is useful at operation for patients with repeated infection.

Partial liver transplantation from living related donors.

Living related liver transplantation was performed in five cases between June 1989 and July 1991 at Shinshu University Hospital. All of the donors were fathers of the patients and blood type was identical in each case. All of them were discharged from the hospital 2 weeks after hepatectomy without any complications. They started to work 2 months after surgery. Four recipients are surviving but one died. Three are enjoying daily life 17 months after LT in case 1, 5 months after LT in case 4, and 4 months after LT in case 5. Case 2 is still in the hospital 14 months after LT. Advantages of LRLT we noted were (1) cases can be performed totally electively and allow full preparation for the family and the transplant team, (2) primary graft nonfunction has not been observed to date, and (3) 38 patients received the chance of liver transplantation in their own country, which under current legislation would not otherwise have been possible. Disadvantages of LRLT were (1) partial hepatectomy was performed in healthy persons, and (2) retransplantation is difficult.

Left hepatic trisegmentectomy for interlobar hepatoblastoma located close to the hepatic hilum.

A 10-month-old boy was noted to have hepatomegaly, and hepatic angiography showed a huge tumor of the medial segment of the left lobe, located close to the hepatic hilum and involving the anterior segment of the right lobe in contiguity. We considered at first that resection would not be possible, but at operation the anterior segmental branch of the right hepatic artery was successfully ligated and divided, which made left trisegmentectomy possible in this case. Postoperatively, the patient was treated with cisplatin and tetrahydropyranyl Adriamycin he was doing well without any evidence of disease 24 months after the hepatectomy.

Mechanism and prevention of port-site tumor recurrence after laparoscopy in a murine model.

BACKGROUND/PURPOSE: Although minimally invasive surgery (MIS) has been broadly applied in patients with cancer of the gastrointestinal tract, the etiology of port-site tumor recurrence (PSR) after laparoscopic cancer surgery remains unclear. The authors report here an analysis of PSR in a model of murine neuroblastoma after laparoscopic tumor biopsy and propose a mechanism for this complication as well as a potential treatment. METHODS: Immature 5- to 7-week old male A/J mice (18-23 g) were subcutaneously inoculated with the minimally immunogenic TBJ-neuroblastoma (TBJ-NB) in the left flank and divided into three treatment groups. The following operations were performed 14 days after tumor inoculation: group 1, additional intraperitoneal or intravenous injection of TBJ-NB during CO2 pneumoperitoneum; group 2, simulated transperitoneal tumor biopsy using MIS techniques during either CO2 pneumoperitoneum or gasless suspension; Group 3, intraperitoneal (IP) or intravenous (IV) administration of cyclophosphamide on postoperative days 0 and 3 to prevent PSR after simulated tumor biopsy during CO2 pneumoperitoneum. RESULTS: In group 1, the incidence of PSR was 0% in the intravenously injected mice versus 63% in mice injected intraperitoneally with TBJ-NB. In group 2, no significant difference in the incidence of PSR was seen between simulated tumor biopsy (89%) animals with CO2 pneumoperitoneum versus animals with gasless suspension (81%). In group 3, mice that did not receive any chemotherapy had an 89% incidence of PSR. Administration of cyclophosphamide via either the IP or IV route effectively prevented PSR, although there was no difference in the incidence of PSR between the two routes (IP 12% versus IV 13%). CONCLUSIONS: The data suggest that PSR in tumor-bearing hosts may be caused by direct seeding of exfoliated tumor cell, and not by hematogenous metastases. Contrary to the other reports, CO2 pneumoperitoneum was not found to be essential for the development of PSR. Furthermore, the authors conclude that postoperative chemotherapy may be useful in preventing PSR after MIS in patients bearing chemotherapy-sensitive tumors such as neuroblastoma.

Esophageal duplication cyst associated with pulmonary cystic malformations.

Two cases of esophageal duplication cyst associated with pulmonary cystic malformations (cystic bronchiectasis with pneumonia in one, intrapulmonary bronchogenic cysts with bronchial atresia in the other) are reported. The coexistence of these complex anomalies supports the recognition that esophageal duplication cyst also is an entity of a broad spectrum of developmental abnormalities caused by abnormal budding of the primitive foregut. Nine cases of similar complex anomalies in the lung and esophagus have been reported. Although rare, this malformation complex should be borne in mind in the treatment of pediatric mediastinal and pulmonary malformations.

Nude mouse xenograft study for treatment of neuroblastoma: effects of chemotherapeutic agents and surgery on tumor growth and cell kinetics.

Four human neuroblastomas transplanted into nude mice were used for experimental chemotherapy and surgery, and the following results were obtained. Cyclophosphamide was the most effective for human neuroblastoma, cis-platinum being the second, among several chemotherapeutic drugs examined. Aclacinomycin A is more effective than Adriamycin. VM26 should be administered 48 to 72 hours after injection of cis-platinum, according to flow cytometric analysis. Flow cytometric analysis also disclosed that residual tumor grows most rapidly seven days after subtotal excision. However, chemotherapy is more effective in the postoperative period than it is in the preoperative period.

Elective surgery for cystic meconium peritonitis: report of two cases.

The cases of two infants with prenatally diagnosed cystic meconium peritonitis, who underwent minimal surgical procedure (drainage) first and elective surgery later, are reported. Just after birth tube drains were inserted under local anesthesia with ultrasonographic guidance. They underwent laparotomy, at 16 days in case 1 and at 20 days in case 2. The adhesions were quite easily dissected, ileal atresia was found, and the intestines were safely primarily anastomosed. In cystic meconium peritonitis, radical surgery in the early neonatal period is usually difficult, so many patients require second or third operations. We recommend cyst drainage just after birth and elective surgery later.

Intralesional corticosteroid injection with short-term oral prednisolone for infantile hemangiomas of the eyelid and orbit.

Infants with hemangiomas of the eyelid and orbit are at risk for amblyopia and refractive errors. Several methods of treatment for these tumors have been associated with complications and limitations. Five infants with these hemangiomas were treated by intralesional corticosteroid injection combined with short-term oral prednisolone. In an attempt to eliminate complications, corticosteroid injections were administered. In the cases of orbital hemangioma, ultrasonography guidance was used to assist the injection. This treatment is safe, simple, and effective for infants. In addition, complications are minimized.

Familial multiple mesothelial cysts of the spleen.

A 5-month-old boy who was diagnosed as having hydrops fetalis at 25 weeks' gestation had severe ascites of unknown origin. At the age 12 months, ultrasonography and computed tomography showed multiple cysts in the spleen that were increasing in size rapidly. Splenectomy resulted in complete disappearance of the ascites. These cysts were diagnosed as mesothelial cysts because the cell lining of the splenic cysts stained positively with alcian blue and cytokeratin. The boy's mother had undergone splenectomy for splenic and retroperitoneal lymphangiomas at 4 years of age. Histological reevaluation showed that the lining of her splenic cysts had the same mesothelial components as her son's. Their chromosomal assay showed normal karyotypes. Mesothelial cyst of the spleen appears similar to splenic lymphangioma morphologically; however, bleomycin and OK-432 were not effective. Familial splenic mesothelial (epidermoid) cysts have been reported in three sets of siblings, but this is the first report of their occurrence in mother and son.

Antenatal diagnosis of biliary atresia (type I cyst) at 19 weeks' gestation: differential diagnosis and etiologic implications.

At 19 weeks' gestation, two cystic structures were first identified in the abdomen of a fetus. A repeat ultrasonography at 34 weeks confirmed a definite cyst communicating with the liver. The baby was born at 39 weeks, and serum direct bilirubin started to rise to 4.1 mg/dL. An operative cholangiogram at 23 days of life showed a cystically dilated choledochus with distal atresia and a relatively smooth yet hypoplastic intrahepatic biliary tree. Complete obliteration of the cystic duct was also noted. After excision of the cystic common bile duct, hepatico-jejunal anastomosis was performed, and the patient did well for 8 months postoperatively. Liver biopsy showed proliferation of the bile ductules, but no interlobular bile ducts were observed in any portal triad. A diagnosis of biliary atresia was established. Including the present case, five cases of antenatally diagnosed biliary atresia have been reported. All of them had type I cyst, and antenatal diagnosis was made at 19 to 32 weeks' gestation. Differential diagnosis between biliary atresia of type I cyst and choledochal cyst with complete distal obstruction has been a matter of discussion, and recognition of the entity of antenatally diagnosed biliary atresia is of significant importance from an etiological point of view.

Piston-pump-type high frequency oscillatory ventilation for neonates with congenital diaphragmatic hernia: a new protocol.

High frequency ventilation and extracorporeal membrane oxygenation (ECMO) are devices that are expected to save the lives of newborn infants whose pulmonary conditions have deteriorated. A piston-pump-type high-frequency oscillator (HFO), developed by Bryan and Miyasaka called "Hummingbird," is considered to be superior to high frequency "jet" ventilators or those of the flow-interrupter type, and was used successfully in two neonates with congenital diaphragmatic hernia (CDH) in a high-risk group. The first baby was on a conventional ventilator with pharmacologic support for the first 54 hours and then operated on. Postoperative deterioration necessitated the use of HFO for the next eight days. The infant then recovered uneventfully. For the second baby, HFO was necessary both preoperatively and postoperatively. This baby had a major diaphragmatic defect and her case was complicated with pneumothorax. There was a long stormy course on HFO (total, 70 days), but the patient was successfully extubated on the 75th day postoperatively and is now doing well. We believe active long preoperative stabilization with pharmacologic support and preoperative and postoperative hyperventilation with a piston-pump-type HFO may be a new innovative strategy for the management of severe CDH patients.

Partial liver transplantation from a living donor: experimental research and clinical experience.

Partial liver transplantation (PLTR) was studied experimentally, using 60 monkeys (20 recipients, 20 donors, 20 blood donors). The left lobe of the donors was transplanted orthotopically, using a veno-venous bypass catheter that was inserted in the portal vein and the other side passed through the hepatic portion of the inferior vena cava. The donor survival rate at 1 week was 70%. Seven recipients survived for more than 58 hours (58, 60, 64, 68, 72, 110, and 252 hours), and 13 died within 48 hours of surgery because of postoperative complications. Clinical living related liver transplantation (LRLT) was performed between June 1990 and March 1992 on six patients with biliary atresia and on one with liver cirrhosis and hepatocellular carcinoma. In all, the father's left lobe was transplanted orthotopically. Cyclosporine, azathioprine, and methyl prednisolone were administered. In addition, FK-506 was given to two patients in whom rejection was observed; one died 37 days after surgery because of acute rejection followed by systemic cytomegalovirus infection. The other six patients have survived for 8 to 29 months since transplantation. All six have been discharged from the hospital and are enjoying normal daily life. The postoperative course of all donors was uneventful. They were discharged 2 weeks after the operation and returned to their jobs in 2 months. The authors conclude that PLTR from a living donor is a promising therapeutic alternative to liver transplantation from a cadaver.

Effect of granulocyte colony-stimulating factor on neutropenia in liver transplant recipients with hypersplenism.

The authors present details of their initial experience with use of recombinant human granulocyte colony-stimulating factor (rhG-CSF) for preventing neutropenia caused by hypersplenism, and, possibly, for reducing the risk of postoperative infections in pediatric liver transplant recipients. Seven patients with end-stage liver disease, three of whom had severe hypersplenism, underwent living related liver transplantation (LRLT). The rhG-CSF was administered to the latter three patients. Peripheral neutrophil counts decreased immediately after reperfusion (to 1500 +/- 300/microL) in the three patients, and returned to normal with use of rhG-CSF 3 to 10 days after transplantation. The dosage was adjusted to maintain peripheral leukocyte and granulocyte counts above 5,000/microL and 2,000/microL, respectively. This initial clinical trial showed that rhG-CSF administration restores the leukocyte counts of patients who have hypersplenism, without any significant adverse effects, and that rhG-CSF holds promise for reducing the risk of infections after liver transplantation.