Suruga lecture: The History of JSPS, AAPS, WOFAPS, and IPEG.

The history of Japanese Society of Pediatric Surgoens (JSPS), Asian Association of Pediatric Surgeons (AAPS), World Federation of Associations of Pediatric Srugeons (WOFAPS) and International Pediatric Endosurgery Group (IPEG) was presented focusing on a brilliant pediatric surgeon, Dr Keijiro Suruga, who contributed to all these associations as one of the leading founding members.

History of the Pacific Association of Pediatric Surgeons (PAPS) 1984-1998.

This is a history of the second 15 years of the Pacific Association of Pediatric Surgeons (PAPS) from 1984 to 1998.

Guidelines for managing conscientious objection to blood transfusion.

Parents sometimes deny their children blood transfusion because of their religious beliefs. The Japanese Joint Committee on the Refusal of Blood Transfusion on Religious Grounds asserts that the health and life of every child younger than 15 years should be guarded by the collective efforts of health, welfare, and advocacy institutions when a parent or guardian seeks to withhold transfusion therapy. Patients 18 years or older should receive treatment without transfusion after signing and submitting a "Certificate of Refusal Blood Transfusion and Exemption from Liability." For a patient younger than 18 years, but 15 years or older, essential transfusion can be performed if the patient or at least one guardian consents. Without patient's or guardian's consent, guidelines for patients 18 years or older shall apply. Health care providers should offer the best possible care that is consistent with a patient's age and competency.

Immunological investigation of the hepatic tissue from infants with biliary atresia.

PURPOSE: Matrix metalloproteinases (MMPs) and their endogenous tissue inhibitors [tissue inhibitors of metalloproteinases (TIMPs)] have been implicated in tissue injury and remodeling in many organs. The objective of this study was to evaluate the expression of MMP-3 and -9, and TIMP-1, -2, and -3 and their relationship to liver fibrosis in infants with biliary atresia. METHODS: The expression of MMP-3 and-9 and TIMP-1, -2 and -3 was investigated in liver tissue samples of nine patients with biliary atresia. In addition, the expression of CCR-4 and CCR-5 was analyzed to investigate the activation of Th1 and Th2 cells. The mRNA levels were measured by semiquantitative reverse transcriptase polymerase chain reaction. RESULTS: The expression of MMP-3 was higher than that of MMP-9 in all samples (P < 0.01). The expression of TIMP-1 was higher than that of TIMP-2 and -3 in all samples (P < 0.01). The expression of CCR-5 was higher than that of CCR-4 (P < 0.05), which implied higher activation of Th1 cells relative to Th2 cells. CONCLUSION: Our findings suggest that MMP-3, possibly induced by Th1 cytokines, and its balance with TIMP-1, may be one of the factors involved in the pathogenesis of biliary atresia.

Congenital diaphragamatic hernia associated with aortic coarctation: a case report.

Congenital diaphragmatic hernia associated with cardiac anomalies is a major therapeutic challenge. We report a case of Congenital diaphragmatic hernia associated with coarctation of the aorta.

Laparoscopy-assisted gastropexy for gastric volvulus in a child with situs inversus, asplenia, and major cardiac anomaly.

AIM: The aim of this study was to report on laparoscopy-assisted gastropexy in a child with situs inversus, asplenia, and major cardiac anomaly. CASE: A 15-month-old boy presented with a sudden onset of epigastralgia, nonbilious vomiting, and severe abdominal distention. After a nasogastric tube decompression of the stomach, symptoms resolved and an upper gastrointestinal contrast study confirmed situs inversus and asplenia. Computed tomography showed hepatic symmetry. Major cardiac anomalies (e.g., single atrium, single ventricle, common atrioventricular valve, and pulmonary atresia) were also present and had been treated elsewhere by a Blalock-Taussig shunt operation, the Glenn procedure, and pulmonary artery plasty. To prevent recurrent gastric volvulus, an anterior gastropexy procedure was performed laparoscopically. The patient's weight at the time of surgery was 8.1 kg, and the operating time was 65 minutes. Cardiopulmonary status was stable during insufflation and throughout the laparoscopic procedure. Postoperative recovery was uneventful, and the patient was allowed oral fluids 1 day after surgery and an unrestricted diet on day 2. A Fontan procedure was performed 18 months later, and our patient is now 6 years old and well--with no recurrence of gastrointestinal symptoms. CONCLUSION: This is the first report about the successful application of laparoscopy for performing a gastropexy procedure in a child with gastric volvulus, situs inversus, major cardiac anomaly, and asplenia.

Inchin-ko-to prevents medium-term liver fibrosis in postoperative biliary atresia patients.

The oriental herbal formulation inchin-ko-to (ICKT) inhibits liver cell apoptosis induced by transforming growth factor-beta 1 (TGF-beta1). This study evaluated the effect of ICKT on serum markers of liver function and liver fibrosis in postoperative biliary atresia (BA) patients. Twenty-one postoperative BA patients with elevated GOT, GPT and gamma-GTP, but normal serum total bilirubin levels, were divided into two groups arbitrarily; an ICKT group (n = 12), and a no-ICKT group (n = 9). Serum markers of liver function [GOT, GPT, gamma-GTP, total bile acids (TBA)], and serum markers of liver fibrosis [hyaluronic acid (HA), type IV collagen (C-IV)], were measured in both groups at the beginning of the study, and at 1, and 3 years after the beginning of the study and the results compared statistically. All patients tolerated ICKT well, and there were no side effects. In the ICKT group, mean serum HA levels were significantly decreased at 1 year (P < 0.012), and at 3 years, both mean serum HA and C-IV were significantly decreased (P < 0.001 and P < 0.003, respectively). However, mean serum levels of GOT, GPT, gamma-GTP, and TBA did not change significantly following ICKT use for any length of time (all P > 0.05). Administration of ICKT in postoperative BA patients appears to lower the serum levels of markers of fibrosis in the medium-term. Whether this in fact correlates with prevention cannot be determined from this paper, but ICKT would appear to protect against liver fibrosis. Long-term studies are required to determine the exact role ICKT plays in prognosis of BA patients.

A new rapid acetylcholinesterase staining kit for diagnosing Hirschsprung's disease.

Conventional acetylcholinesterase (AChE) histochemistry is both time consuming and complicated and requires the mixing of reagents that are toxic to the human body. We developed a rapid technique for performing AChE histochemistry, which has already been published, and now present a kit for performing AChE histochemistry that is a further improvement. Rectal suction biopsy specimens taken from 20 constipated patients and three full thickness biopsy specimens taken from 4 Hirschsprung's disease (HD) patients during pull-through surgery from aganglionic, transitional, and ganglionic bowel segments were tested using our rapid technique and the new kit. Each specimen was incubated for only 6 min. All ganglion cells stained clearly for AchE in just 6 min using both techniques. However, the kit was able to stain AchE positive nerve fibers more clearly and did not detect endogenous peroxidase-containing histiocytes, as did the earlier rapid technique. The kit could also detect AchE positive nerve fibers in the circular and longitudinal muscle layers, unlike the earlier rapid technique. The kit allows AChE histochemistry to be performed rapidly with complete accuracy, without any risk for toxicity. Moreover, the kit provides more focused information on AchE distribution in the bowel itself without any extraneous staining and can be used for diagnosing HD and allied disorders as well as establishing the exact level of innervation for pull-through resection.

The role of monocyte chemoattractant protein-1 in biliary atresia.

BACKGROUND: The aim of this study was to explain the role of monocyte chemoattractant protein-1 (MCP-1) in biliary atresia (BA). METHODS: Concentrations of serum MCP-1 and collagen type IV were measured in 38 patients with BA by using commercially available kits. MCP-1 was also assessed in liver biopsy specimens by using immunohistochemistry. Subjects were classified into groups. Group 1 comprised BA patients with normal liver function (n = 13), group II comprised BA patients with moderate liver dysfunction (n = 18), group III comprised BA patients older than 20 years awaiting liver transplantation (n = 7), and the control group comprised age-matched patients without evidence of liver disease (n = 23). RESULTS: Serum MCP-1 levels were significantly increased in group II compared with group I (P < .0001) and the control group (P < .0001). Serum MCP-1 levels in group III were lower than in the control group (P < .0001). There was a significant linear correlation between serum MCP-1 levels and type IV collagen levels in group II. Group II subjects with portal hypertension (PH) had higher MCP-1 levels than those without PH (P = .0009). Biopsy specimens showed MCP-1 was expressed mainly on biliary epithelial cells, vascular endothelial cells, and hepatocytes in group II. CONCLUSIONS: These findings suggest that MCP-1 probably plays a significant role in the development of progressive liver fibrosis in BA.

Efficacy of protocolized management for congenital diaphragmatic hernia. a review of 100 cases.

A review of 100 consecutive cases of congenital diaphragmatic hernia (CDH) treated at our institute focusing on the efficacy of protocolized management (PM) was conducted. Of the 100 cases, 14 who became symptomatic more than 24 h after birth, and seven with fatal anomalies (four cardiac and three chromosomal) were excluded, leaving 79 subjects for this study. Of these, 41 were diagnosed prenatally (PD). Subjects were divided into four groups. Group I: No PD, no PM (n = 34), Group II: No PD, PM (n = 4), Group III: PD, no PM (n = 21), and Group IV: PD, PM (n = 20). PM includes criteria for planned delivery, use of high frequency oxygenation, nitric oxide, echocardiography (EC), and a medication schedule. Overall survival rates for Groups I, II, III, and IV were 73.5% (25/34), 75% (3/4), 38.1% (8/21), and 70.0% (14/20), respectively. Survival rates were higher when PM was used: 70.8% (Groups II, IV) versus 60.0% (Groups I, III). Survival rates were significantly lower if diagnosed prenatally (PD+): 53.7% (Groups III, IV) versus 73.7% (Groups I, II) (P < 0.01). However, in PD+ groups, survival was significantly higher if PM was used (P < 0.05). PM significantly reduced length of hospital stay (35.5 vs. 52.0 days: P < 0.05). EC was found to be a predictor for survival while post-ductal AaDO(2) was not. In 17 cases with cardiac anomalies, PM did not affect survival. Our study suggests that use of PM for prenatally diagnosed CDH cases is associated with improved outcome, although the components of PM need to be tested in prospective trials to determine their true value.

Magnetic resonance urographic parameters for predicting the need for pyeloplasty in infants with prenatally diagnosed severe hydronephrosis.

PURPOSE: Treatment in infants with prenatally diagnosed, severe hydronephrosis remains controversial. We measured anatomical parameters using magnetic resonance urography to create a pelvic index ratio, which we correlated with outcome to assess its value. MATERIALS AND METHODS: We reviewed 35 cases of prenatally diagnosed severe hydronephrosis, including grade III in 19 and grade IV in 16. By 6 weeks after birth all patients had undergone repeat ultrasonography, diuretic renography and magnetic resonance urography. Magnetic resonance urography was used to measure the distance (D) from the bottom of the lowest renal calyx to the ureteropelvic junction and the total longitudinal length (L) of the caliceal system. The pelvic index ratio, defined as D/L, assesses the level at which the ureter inserts into the renal pelvis. RESULTS: All cases were initially managed conservatively. Pyeloplasty was required in 16 kidneys (mean patient age +/- SD 7.5 +/- 3.9 months) because of deterioration in renal function. All patients were well at a mean followup of 3.8 years. The remaining 19 cases continued to be managed conservatively. By a mean of 5.4 years spontaneous resolution of prenatally diagnosed severe hydronephrosis had occurred in 7 of 19 cases, while 12 were downgraded to grades I-II. The mean pelvic index ratio in surgical cases was 0.26 +/- 0.13 and in nonsurgical cases it was 0.12 +/- 0.11, indicating that ureters inserted significantly higher in surgical cases (p = 0.0012). Of 9 kidneys with a pelvic index ratio of greater than 0.3 pyeloplasty was required in 8, while 10 of 12 with a pelvic index ratio of less than 0.1 were managed conservatively. CONCLUSIONS: Pyeloplasty is likely to be required if the pelvic index ratio is greater than 0.3 and conservative management is likely to succeed if the ratio is less than 0.1. The pelvic index ratio can be calculated easily even in the neonatal period. It appears to be prognostic for pyeloplasty in cases of prenatally diagnosed, severe hydronephrosis.

Review of 86 patients with myelodysplasia and neurogenic bladder who underwent sigmoidocolocystoplasty and were followed more than 10 years.

PURPOSE: We reviewed the long-term outcome of sigmoidocolocystoplasty. MATERIALS AND METHODS: The records of 86 patients who underwent sigmoidocolocystoplasty with a followup of more than 10 years were reviewed, focusing on histopathology, renal function, vesicoureteral reflux, bladder stones and urinary continence. We developed a urinary continence evaluation score, calculated as the postoperative minus the preoperative urinary continence score, including 0-incontinence, 1-severe leakage, 2-moderate leakage, 3-mild leakage, 4-almost dry and 5-dry, for patient self-assessment of urinary continence. A score of 2 or more meant improvement and a score of less than 2 meant no improvement. RESULTS: Mean followup was 13.1 years (range 10 to 21). Histopathology showed inflammation in all cases, hyperplasia in 9 and metaplasia in 4. In 80 of 86 cases creatinine clearance was normal. Ureteral reimplantation had been performed before sigmoidocolocystoplasty in 9 cases (13 kidneys) for grades III to V vesicoureteral reflux, which recurred as grade I in 1 (1 kidney). Ureteral reimplantation was performed during sigmoidocolocystoplasty in 31 cases (58 kidneys) and grades I to II vesicoureteral recurred in 5 (6 kidneys). Of the remaining 44 sigmoidocolocystoplasty cases there were 2 (2 kidneys) of grade I vesicoureteral reflux. On (99m)technetium-dimercapto-succinic acid scintigraphy there was increased renal scarring in 8 cases. Bladder stones were identified in 18 of 86 cases during followup but they were absent at review. The urinary continence evaluation score showed improvement in 61 cases and no improvement in 25. CONCLUSIONS: Our results suggest that sigmoidocolocystoplasty is safe and effective in the long term.

Obstruction due to rectal cuff after laparoscopy-assisted transanal endorectal pull-through for Hirschsprung's disease.

We report a case of persistent obstruction after laparoscopy-assisted transanal endorectal pullthrough for Hirschsprung's disease in a 4-week-old boy with biopsy-proven HD. Before pull-through, the posterior rectal cuff was split along its entire length cranially, starting from the dentate line. Initial recovery was uneventful; however, signs of obstruction developed 3 weeks postoperatively. Reoperation through a posterior sagittal approach confirmed a residual rectal cuff surrounding the neo-rectum circumferentially. The dorsal side of the residual rectal cuff was removed completely. At follow-up 5 years later, he defecates 2 to 4 times a day with occasional staining. We hypothesize that the persistent postoperative obstruction was caused by a long residual rectal cuff that spontaneously reapproximated and/or became folded during pull-through. Therefore, a shorter cuff with near-total posterior excision should be strongly considered during laparoscopy-assisted transanal endorectal pull-through for Hirschsprung's disease.

Magnetic resonance imaging in lipoblastoma: can it be a diagnostic modality?

Magnetic resonance imaging (MRI) is generally an efficient tool for establishing the differential diagnosis of soft tissue tumours. We simultaneously encountered two patients with adipose tumours in the neck. Both had similar clinical features and MRI findings: lobular high intensity tumours on both T1-and T2-weighted images equivalent to subcutaneous fat with septal formation. The provisional diagnosis in both cases was lipoblastoma and both had complete excisions. Histopathological diagnoses, however, were different (lipoblastoma and fibrolipoma), indicating that while MRI is excellent for demonstrating the anatomical detail of soft tissue masses, it is not specific enough to be used alone for differentiating between adipose tumours. In this report, we assess the value of MRI and recommend a more appropriate and patient-friendly protocol for the evaluation for adipose tumours in children.

Management of a prenatally diagnosed huge teratoma arising from the soft palate.

Teratomas arising from the oral cavity are relatively rare and can cause life-threatening airway obstruction immediately after birth. We report a case of a huge teratoma arising from the soft palate detected prenatally. To save the patient, a caesarean section and ex utero intrapartum treatment (EXIT) were planned at 29 weeks of gestation. However, during EXIT, the patient's cardiopulmonary status deteriorated due to unstable cord blood flow secondary to uterine contractions. EXIT was abandoned, the patient was delivered and an emergency tracheotomy performed. The tumour was successfully excised 4 hours after tracheotomy. The tumour weighed 1,591 g and was 20 x 22 x 12 cm. The patient, a female, weighed 715 g. Histopathology showed Grade II teratoma. The postoperative course was uneventful and she is now 5 years old with no neurological sequelae. EXIT is indicated in patients who have a high risk for airway obstruction immediately after birth. However, if EXIT must be abandoned, as in this case, urgent tracheotomy is mandatory. Planned prevention of airway obstruction at delivery is indispensable for successful outcome and requires excellent coordination among obstetricians, neonatologists, anaesthesiologists and paediatric surgeons.

Reduction of intussusception in infants by a pediatric surgical team: improvement in safety and outcome.

Commonly, reduction of intussusception is performed by experienced radiologists. We review the performance of a pediatric surgical team for treating intussusception according to a standard protocol and present our findings. Three hundred and seventy eight patients with signs and symptoms of intussusception we treated from 1980 to 2005 were reviewed. Hydrostatic reduction (HR) was performed using a water-soluble contrast agent under fluoroscopy unless there was a serious condition clinically. Before 1998, HR was performed exclusively by pediatric surgical trainees (period A). In 1998, a standard protocol (double-balloon tube, maximum pressure of 120 cm H2O, repeated a maximum of five times, and HR performed by a pediatric surgical trainee under the supervision of a consultant pediatric surgeon) was adopted (period B). As part of the protocol, the operating room was notified of the HR procedure and placed on call for emergency surgery. Of the 378 patients, 21 required immediate laparotomy due to serious general condition, leaving 138 during period A and 219 during period B who had HR. Patient age, sex, and duration of symptoms (period A, 14.5 +/- 7.8 h; period B, 13.1 +/- 9.9 h) were not statistically significant. Success of HR during period A was 64.5%, and significantly improved for period B at 94.5% (P < 0.01). During period B, 128 of our patients had been referred from elsewhere for failed reduction attempted by radiologists or pediatricians. We were able to perform HR successfully in 118 of these (92.2%). During period A, it was significantly less at 54.0% (P < 0.01). Bowel perforation during HR occurred in two patients during period A (1.4%) and two patients during period B (0.9%), but the latter cases were transferred immediately for emergency surgery with good outcome. Reduction of intussusception by a pediatric surgical team would appear to be significantly safer with better outcome, and is thus more efficient.