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Introduction: The objective of our research was to design and develop a novel needle driver with multiple degrees of freedom (DOFs) for pediatric laparoscopic surgery. Pediatric laparoscopic surgery has many advantages for patients, but the difficulty of the operation is increased due to many restrictions. For example, the motion of the needle driver is restricted by the insertion points, and the operation workspace is smaller in children than in adults., Material and Methods: A needle driver with 3 DOFs and a 3.5-mm diameter is proposed and implemented in this study. Grasping DOF is achieved using a piston mechanism actuated by a wire. Deflection and rotation DOFs are actuated by gears., Results: Experiments were conducted to evaluate the workspace and ligation force, and the results confirmed that the needle driver meets all the necessary requirements. Finally, a first reaction of a pediatric surgeon on the suturing and ligaturing capabilities of the prototype is reported., Conclusion: A multi-DOF needle driver with a new mechanism was proposed for pediatric laparoscopic surgery and a first prototype was developed. It is expected that further elaboration of the developed first prototype of the needle driver may contribute to the advancement of pediatric laparoscopic surgery.

Background: Plasma citrulline has been reported to be a good indicator of intestinal functional volume in patients with intestinal dysfunction. We reconfirmed the facts and also investigated the dynamic changes of plasma citrulline in acute-phase patients with intestinal dysfunction., Methods: We measured plasma citrulline in six patients with intestinal dysfunction who were in the acute and chronic phase for more than 6 months., Results: Four patients out of six could be withdrawn from total parenteral nutrition, and their plasma citrulline level dynamically changed according to their intestinal states and finally increased up to 15 nmol/mL. Two patients, who could not be withdrawn from parenteral nutrition, showed very low levels of plasma citrulline throughout the treatment course (under 15 nmol/mL)., Conclusion: The cut-off level of plasma citrulline indicating permanent intestinal dysfunction may be 15 nmol/mL in our data. In the acute phase, plasma citrulline changed dynamically according to the intestinal state and may be a good indicator of fluctuating intestinal functions. Thus, although only a few patients were enrolled in this study, plasma citrulline may be a good indicator of stable-state as well as acute-unstable-state intestinal functions., (© 2012 The Authors. Pediatrics International © 2012 Japan Pediatric Society.)

Aim: This study aimed to develop a novel procedure for esophagoesophageal anastomosis for natural orifice translumenal endoscopic surgery (NOTES)., Materials and Methods: An ex vivo feasibility study was performed in eight porcine models. The procedure was as follows: (1) A BraceBar™ (Olympus Medical Systems Corp., Tokyo, Japan), a double T-bar suturing device, was placed endoscopically at the blind end of the upper esophagus (UE). (2) The blind end was incised, and the scope was advanced out of the esophagus. (3) A balloon catheter was inserted into the lower esophagus (LE). (4) The catheter and a thread on the BraceBar were withdrawn so that the end of the UE was inverted, and the LE was pulled into the UE. (5) After the catheter was removed, a short tube was placed inside the duplicated part of the esophagus via the transgastric route. (6) A double ligature was performed using a ligating device over the tube. A liquid leak test was performed after the procedure., Results: All steps in this procedure were technically successful under the endoscopic visualization without any assistance from outside of the esophagus. The median time of this procedure was 31 (23-66) minutes. The median internal pressure of the UE was 122 (82-142) mm Hg when the anastomosed esophagus was separated into two specimens during the leak test., Conclusions: Translumenal esophagoesophageal anastomosis was feasible. The duration of the procedure was short, and the anastomoses appear to have sufficient strength for use in clinical practice. An in vivo survival study is needed to confirm the safety and reliability of this NOTES procedure.

Purpose: Meconium peritonitis is caused by an intestinal perforation that may occur in the fetus, followed by severe chemical peritonitis, resulting in high morbidity., Methods: We have experienced six patients with meconium peritonitis. Cystic drainage was performed soon after birth for all patients. We investigated the concentrations of several cytokines and a chemokine (interleukin 8) in the ascites from the six patients with meconium peritonitis. In two patients we also measured the serum cytokines and chemokine level just after birth., Results: Interleukin 6 and interleukin 8 concentrations were very high in the cyst or ascites just after birth. In the serum taken from two patients, the levels of interleukin 6 and interleukin 8 were also high. In five patients who underwent drainage of cysts after birth, systemic inflammation could not be completely suppressed before curative surgery., Conclusions: Interleukin 6 and interleukin 8 play important roles in the inflammatory response syndrome associated with meconium peritonitis, and drainage of cystic fluid did not completely suppress this inflammation. To lessen the high morbidity of meconium peritonitis, efforts should be made to suppress the inflammatory response using new treatment strategies, such as administration of steroids or anti-cytokine therapy to supplement cystic drainage.

We report a case of malignant steroidogenic tumor arising from a sacrococcygeal teratoma in a 5-year-old girl. A congenital gluteal mass and a 7-month history of precocious puberty had been noted, and a large estrogen-producing tumor in the sacrococcygeal area was found. After a biopsy, chemotherapy and tumor resection were performed, and no recurrence has been observed. The biopsy specimen showed small clusters of atypical round cells adjacent to a mature teratoma. They had large round nuclei with prominent nucleoli and abundant eosinophilic cytoplasms and were positive for vimentin, steroidogenic factor-1, inhibin α, and melan-A. Increased mitoses, vascular invasion, and necrosis were noted. The tumor was diagnosed as sacrococcygeal mature teratoma, with malignant steroidogenic tumor as somatic malignant transformation. Although several kinds of somatic malignant transformation of sacrococcygeal teratoma have been reported, to the best of our knowledge, this is the first case of malignant steroidogenic tumor arising from sacrococcygeal teratoma., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Purpose: In 2006, The Japanese Society of Pediatric Endoscopic Surgeons devised a plan to develop a pediatric endoscopic surgical skill qualification (ESSQ) system. This system is controlled by The Japan Society for Endoscopic Surgery. The standard requirement for skills qualification is the ability of each applicant to complete common types of laparoscopic surgery. The main goal of the system is to decrease complications of laparoscopic surgery by evaluating the surgical skills of each applicant and subsequently certify surgeons with adequate skills to perform laparoscopic operations safely., Methods: A committee of pediatric ESSQ created a checklist to assess the applicant's laparoscopic surgical skills. Skills are assessed in a double-blinded fashion by evaluating an unedited video recording of a fundoplication for pediatric gastroesophageal reflux disease., Results: The initial pediatric ESSQ system was started in 2008. In 2008 and 2009, respectively, 9 out of 17 (53%) and 6 out of 12 (50%) applicants were certified as expert pediatric laparoscopic surgeons., Conclusions: Our ultimate goal is to provide safe and appropriate pediatric minimally invasive procedures and to avoid severe complications. To prove the predictive validity of this system, a survey of the outcomes of operations performed by certified pediatric surgeons is required.

Background/purpose: There are numerous techniques of minimally invasive surgery for pediatric inguinal hernia. However, laparoscopic percutaneous extraperitoneal closure (LPEC) is one of the most simple and reliable methods. Single-incision laparoscopic surgery has recently been developed to reduce the invasiveness of traditional laparoscopy. This study aimed to assess the safety and feasibility of the single-incision approach for LPEC., Materials and Methods: Between June 2009 and March 2010, 117 conventional LPEC and 60 single-incision LPEC (SILPEC) procedures were performed. A laparoscope was placed through a transumbilical incision. A 3-mm grasping forceps was inserted in the lower abdomen in LPEC and through the same transumbilical incision with a different entrance in SILPEC. Using the LPEC needle, the hernia sac was closed extraperitoneally. We compared the short-term outcomes of 177 children operated on using either technique., Results: Visualization and tissue manipulation were good in both methods. There were no complications or evidence of early recurrence. Cosmetically, SILPEC proved to be superior to LPEC because the scar resulting from surgical incision is hidden within the umbilicus., Conclusion: Single-incision LPEC proved to be as successful an operative procedure as LPEC and produced excellent cosmetic results. Single-incision LPEC for inguinal hernia in children is safe and feasible., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Introduction: An engineered trachea with an absorbable scaffold should be used to augment the repair of a stenotic tracheal section in infants and children because this type of engineered airway structure can grow as the child grows. Our strategy for relief of tracheal stenosis is tracheoplasty by engineered cartilage implantation in accordance with the concept of costal cartilage grafting to enlarge the lumen. This study investigated the mechanical properties of regenerative cartilage with a biodegradable scaffold, Neoveil®, to aid in design of a composite scaffold that maintained semi-rigid properties until cartilage could be generated., Materials and Methods: New Zealand White rabbit (n=3) chondrocytes were isolated from auricular cartilage with collagenase type 2 digestion. Then 10x10(6)/cm3 chondrocytes in atelocollagen solution were seeded onto polyglycolic acid (PGA) mesh. A total of 36 constructs, 12 from each rabbit, were implanted into athymic mice (3 constructs/mouse). Constructs were retrieved after 8 weeks and evaluated by measurements of mechanical and biochemical properties as well as histological examination. Thirty-six PGA mesh sheets of the same size but without cells were implanted in control mice., Results: After 6 weeks of implantation, staining of sections with Safranin O revealed cartilage accumulation. Glycosaminoglycan was gradually produced from chondrocytes in the engineered constructs, correlating with the duration of implantation. Mechanical parameters had the same values as those for rabbit tracheal cartilage 8 weeks after implantation., Conclusions: Biodegradable Neoveil® had good biocompatibility and was able to support extracellular matrix formation in engineered cartilage in an animal model.

Most patients with thymic cysts complain of a slowly enlarging, asymptomatic cervical mass. Only 6-10% suffer dysphagia, dyspnoea, stridor, cervical pain or vocal paralysis. In some rare cases sudden onset of severe dyspnoea or asphyxia is the first symptom, especially in neonates and small infants. We report a unique case of a 20-month-old child, who required emergency tracheal intubation due to asphyxia. Cervicomediastinal thymic cyst might need to be included in causes of life-threatening airway obstruction in young children.

Background: Infants with severe congenital anomaly often need to undergo operation followed by antibiotic therapy. As a result they inevitably acquire abnormal intestinal microbiota, which cause severe infections such as necrotizing enterocolitis. Also, intestinal function deteriorates and their nutritional state is very poor. In order to prevent these situations probiotic therapy is proposed as an effective supporting treatment. Probiotic therapy were therefore applied to infants with severe congenital anomaly as early as possible to ascertain its efficacy., Methods: As probiotics, two bacteria were used: Bifidobacterium breve Yakult and Lactobacillus casei Shirota. Probiotic therapy was used in four infants with severe congenital anomaly as early as possible after surgery. Their intestinal microbiota and physical growth were followed through the treatment course., Results: Two patients suffered from meconium peritonitis with ileal atresia. One patient was born with complex anomalies (omphalocele, bladder exstrophy, myelomeningocele). The fourth patient suffered from complete urorectal septum malformation. The intestinal microbiota of these four patients was first induced to be probiotic dominant and finally changed to commensal anaerobe dominant that was similar to normal intestinal microbiota. Pathogenic bacteria were seldom detected. The patients' physical growth was excellent despite short bowel and pulmonary hypoplasia., Conclusion: Probiotic therapy was effective in inducing probiotic dominant intestinal microbiota and normal intestinal microbiota in infants with severe congenital anomalies. As a result their intestinal absorptive functions were activated and severe infections were completely prevented. All of the infants grew well despite their physical disadvantages.

Choledochal cyst causes liver fibrosis, the extent of which varies with each case. Liver damage seldom progresses to cirrhosis, but when it does, it is generally irreversible. We report an infantile case of liver cirrhosis associated with choledochal cyst in which complete clinical resolution was achieved by surgery. Pancytopenia caused by splenomegaly, massive ascites, hypoalbuminemia, and coagulation abnormality that were observed during the early postoperative period had disappeared within 4 months after surgery. Needle liver biopsy performed at 1 year after surgery revealed marked improvement in liver fibrosis., (Copyright (c) 2010 Elsevier Inc. All rights reserved.)

The number and types of minimally invasive surgical procedures being performed in children have increased exponentially in the last 15 years. Laparoscopic fundoplication is commonly performed for gastroesophageal reflux disease (GERD), although the population of patients who undergo this procedure is different in adults and children. In Japan, laparoscopic fundoplication has become a standard procedure, even for children with neurological impairment; however, its indications remain controversial. In this article we review the status of laparoscopic antireflux surgery for infants and children, looking at its indications, the procedures available, the complications, and the training required to perform the procedure safely and effectively.

Ras guanyl nucleotide-releasing protein 2 (RASGRP2), one of the Ras guanine exchange factors, is implicated as a critical regulator of inside-out integrin activation in human lymphocytes, neutrophils and platelets. However, the activities of this protein in endothelial cells remain unclear. In the current study, we identify a physiological function in blood vessel formation for XRASGRP2, which is the Xenopus ortholog of mammalian RASGRP2. XRASGRP2 over-expression induced ectopic vascular formation, and XRASGRP2-knockdown embryos showed delayed vascular development. We also investigated the upstream signaling of XRASGRP2 in endothelium formation. XRASGRP2 expression was up-regulated in the presence of VEGF-A and down-regulated following VEGF-A depletion. XRASGRP2 knockdown abolished the ectopic induction of endothelial cells by VEGF-A in the posterior ventral blood island. These results suggest that XRASGRP2 is essential for vascular formation during Xenopus development.

We report 2 cases of growth spurt-related recurrence after Nuss procedure. Each of the 2 cases underwent bar insertion at the age of 6 and 11 years, respectively. The support bar was removed 2 years later followed by severe redepression during the growth spurt. One patient underwent redo Nuss procedure elsewhere. The other patient was diagnosed as idiopathic precocious puberty and is in treatment. The possibility of growth spurt-related recurrence must be explained to those who undergo early correction before surgery, and follow-up is mandatory at least until patients' puberty is over.

Board-certified pediatric surgeons and instructors less than 65 years old in Japan total 244 and 211, respectively. Board-certified general surgeons wishing to become board-certified pediatric surgeons must be able to perform an additional 150 pediatric surgical procedures. In addition, board-certified pediatric surgeons wishing to maintain board certification must perform 100 pediatric surgeries in each 5-year period. While the number of children is decreasing gradually, people living in both metropolitan and rural areas require increasing numbers of pediatricians and pediatric surgeons, necessitating a reform of the system for pediatric emergent care. Because of the low number of pediatric surgeries performed, young pediatric surgeons need to share surgical opportunities. Board-certified instructors should discuss how to teach young pediatric surgeons in university and children's hospitals, dispatch them to suburban and rural clinical hospitals, and help them maintain a good quality of life in busy hospitals. Postgraduate training and specialty certification systems in pediatric surgery for the near future are described.

Purpose: Although various urologic complications have been reported after abdominoperineal pull-through and posterior sagittal anorectoplasty for the treatment of high-type imperforate anus, reports regarding complications after laparoscopically assisted anorectoplasty (LAARP) are surprisingly rare. Here, we discuss the potential complications of LAARP., Methods: A retrospective study was conducted of 24 patients treated with LAARP from 2000 to 2006. The clinical and operative records were reviewed. Of the 24 patients, 18 were evaluated postoperatively by screening magnetic resonance imaging (MRI)., Results: The 24 participants are composed of patients with rectoprostatic urethral fistula (n = 15), rectal agenesis (n = 3), rectovesical fistula (n = 2), rectovaginal fistula (n = 2), and cloaca (n = 2). Defecatory function after LAARP was satisfactory. None of the patients had dysuria or urinary infection postoperatively. Cystic formations posterior to the urethra were demonstrated in 9 of the 18 cases examined by MRI. Postoperative voiding cystourethrography failed to demonstrate the lesion in 6 of 9 patients. The types of imperforate anus in this subgroup were rectoprostatic urethral fistula (n = 7), rectovesical fistula (n = 1), and rectal agenesis without fistula (n = 1). Average cyst diameter was 22 +/- 19 mm. Two patients with large cysts (62 and 42 mm) underwent surgical resection., Conclusions: Although satisfactory fecal continence could be achieved by LAARP, we experienced 2 cases with a large residual fistula that required surgical resection. In addition, screening MRI demonstrated the presence of cystic formations in 9 of 18 patients. We recommend that MRI be performed routinely during follow-up of patients treated with LAARP.

The ERas gene promotes the proliferation of and formation of teratomas by mouse embryonic stem (ES) cells. However, its human orthologue is not expressed in human ES cells. This implies that the behavior of transplanted mouse ES cells would not accurately reflect the behavior of transplanted human ES cells and that the use of nonhuman primate models might be more appropriate to demonstrate the safety of human ES cell-based therapies. However, the expression of the ERas gene has not been examined in nonhuman primate ES cells. In this study, we cloned the cynomolgus homologue and showed that the ERas gene is expressed in cynomolgus ES cells. Notably, it is also expressed in cynomolgus ES cell-derived differentiated progeny as well as cynomolgus adult tissues. The ERas protein is detectable in various cynomolgus tissues as assessed by immunohistochemisty. Cynomolgus ES cell-derived teratoma cells, which also expressed the ERas gene at higher levels than the undifferentiated cynomolgus ES cells, did not develop tumors in NOD/Shi-scid, IL-2Rgamma(null) (NOG) mice. Even when the ERas gene was overexpressed in cynomolgus stromal cells, only the plating efficiency was improved and the proliferation was not promoted. Thus, it is unlikely that ERas contributes to the tumorigenicity of cynomolgus cells. Therefore, cynomolgus ES cells are more similar to human than mouse ES cells despite that ERas is expressed in cynomolgus and mouse ES cells but not in human ES cells.

Introduction: We have designed an engineered graft fabricated from a biodegradable scaffold using chondrocytes and applied this construct to augment repair of tracheal stenosis. This study investigated the feasibility of using such tissue-engineered airways with autologous chondrocytes in a rabbit model., Material and Methods: Chondrocytes were isolated and expanded from the auricular cartilage of New Zealand white rabbits, then seeded onto composite 3-layer scaffolds consisting of a collagen sheet, a polyglycolic acid mesh, and a copolymer (l-lactide/epsilon-caprolactone) coarse mesh. The engineered grafts were implanted into a 0.5 x 0.8-cm defect created in the midventral portion of the cervical trachea. Gelatin sponges that slowly released basic fibroblast growth factor (b-FGF) were then placed on the constructs, which were retrieved 1 or 3 months after implantation., Results: The biodegradable scaffold seeded with chondrocytes could maintain airway structure up to 3 months after implantation. Tracheal epithelial regeneration occurred in the internal lumen of this composite scaffold. Three months after implantation, staining of the sections showed cartilage accumulation in the engineered tracheal wall., Conclusion: This composite biodegradable scaffold may be useful for developing engineered trachea. A gelatin sponge slowly releasing b-FGF might enhance chondrogenesis.

At 24 weeks of gestation, a fetus was suspected of having a huge intraabdominal cyst by fetal ultrasound. Multicystic dysplastic kidney (MCDK) was the most probable diagnosis; however, because a solid area was visualized in the large cystic lesion, a neoplasm of the kidney could not be ruled out. A 3529-g boy was born at 35 weeks of gestation by cesarean delivery. Eight days after birth, the tumor was resected. Histopathologic examination confirmed MCDK. The cause of MCDK in this patient was assumed to be ureteral obstruction in early fetal life. These findings suggested that the affected kidney had experienced mesenchyme-to-epithelium transition followed by interaction between the metanephric blastema and ureteral bud.

Introduction: Construction of engineered respiratory tract using tissue-engineered cartilage has not yet been reported. In order to generate artificial trachea using human chondrocytes obtained from tracheal cartilage, we investigated whether human tracheal chondrocytes can act as a cell source to fabricate engineered airway patches to augment stenotic parts of the trachea., Materials and Methods: After informed consent, chondrocytes were obtained from five patients who needed tracheal surgery. A small piece of resected tracheal cartilage was digested by collagenase type 2 for 3-4 h. This yielded chondrocytes, which were expanded in vitro and seeded onto biodegradable scaffolds; these were then implanted subcutaneously in athymic mice. The implanted constructs were retrieved 8 weeks later for histologic and biochemical analysis., Results: In monolayer cultures, chondrocytes proliferated well, showing a 100- to 1,000-fold increase in 1 month. Once expanded, the cells lost their original morphological and biologic characteristics, but the engrafted scaffold showed histologic and biochemical characteristics of cartilage. Viable chondrocytes and extracellular matrix were detected, and glycosaminoglycan (GAG) in vivo was present., Conclusions: Here we show that a small piece of human tracheal cartilage can generate sufficient chondrocytes in vitro and form tracheal cartilage architecture in an in vivo environment.

Complete urorectal septum malformation sequence (URSMS) is usually a lethal anomaly that is characterized by urethral obstruction, imperforate anus, ambiguous genitalia, renal agenesis or dysplasia, and mullerian duct maldevelopment. This anomaly is thought to be caused by the cessation of urorectal septum migration toward the caudal cloacal membrane. Teratogenic factors or a genetic abnormality is postulated as the etiology. To date, only 4 patients with URSMS have survived the neonatal period; however, 2 of these infants died before the age of 1 year. We report the survival in a case with complete URSMS who had moderate pulmonary hypoplasia and preserved left renal function. The cloacal remnant was dilated more than expected because the wall of the muscle layer was torn, perhaps in early fetal life, and timely placement of vesico-amniotic shunts prevented severe pulmonary hypoplasia caused by oligohydramnios., (Copyright 2008 S. Karger AG, Basel.)

Neuroblastoma is one of the most common solid tumors, and the biopsy and excision of this tumor are often required as part of a planned multimodal treatment. In 1995, Holcomb and coworkers first reported endosurgical procedures for the diagnosis and treatment of pediatric malignancies; however, the usefulness of laparoscopic procedures for abdominal neuroblastoma is still unclear. Twenty-five laparoscopic biopsies for advanced abdominal neuroblastoma and nine laparoscopic excisions for localized abdominal neuroblastoma performed at Saitama Children's Medical Center were evaluated. The laparoscopic procedures significantly reduce the time to start postoperative feeding as well as the time to start postoperative chemotherapy and the duration of hospital stay. Also, the blood loss of laparoscopic excision of localized neuroblastoma was significantly little compared with that of open excision. Precise indications of laparoscopic procedure for the diagnosis and treatment of abdominal neuroblastoma provide better prognosis and quality of life for infants and children.

There has been steady improvement in the overall survival rate of patients with esophageal atresia during the past 30 years. With modern neonatal intensive care, very low birth-weight infants with esophageal atresia are also expected to survive. However, there are many questions in the treatment including delayed primary surgery and multistaged esophageal elongation for extremely low birth-weight infants and patients with long-gap esophageal atresia, respectively. On the other hand, reports of thoracoscopic repair of esophageal atresia are increasing, although there has been no report on long-term follow-up. This procedure has many advantages including wound cosmesis and little thoracic deformity, but it is technically difficult even for experienced endoscopic surgeons and is performed only in a few children's hospital in Japan. Easier thoracoscopic surgery using robotic technology is expected in the near future.

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.

In the present study we have successfully isolated neuroblastoma cells from primary human neuroblastoma tissues by using a magnetic bead-mediated purification system. Since primary neuroblastoma tissues contained CD3- and CD19-positive lymphocytes, total cell suspensions were prepared and incubated with magnetic beads coated with anti-CD3 or with anti-CD19 antibody. After magnetic separation, unbound materials were recovered and analyzed by immunohistochemical staining for NB84, one of the neuroblastoma markers. Immunohistochemical and FACS analyses demonstrated that NB84-positive cells were enriched in the unbound fraction. Subsequently, unbound materials were seeded on cell culture plates and maintained at 37 degrees C overnight. After incubation, non-adherent cells were collected and stained with anti-NB84 antibody. Under our experimental conditions, a significant increase in the number of NB84-positive cells was observed. Furthermore, our purified NB84-positive cells responded to all-trans retinoic acid and nerve growth factor better than the initial primary cells. Collectively, our present results suggest that magnetic bead-mediated purification enriches neuroblastoma cells which retain their biological properties.

Purpose: The aim of this study was to evaluate postoperative anal function of high-type imperforate anus after laparoscopically assisted anorectoplasty (LAARP)., Methods: Between 2000 and 2002, LAARP was performed in 13 patients with high-type imperforate anus. Clinical data of the LAARP group were compared with the posterior sagittal anorectoplasty (PSARP) group (n = 7) operated before 2000. All patients were treated with initial colostomy in the newborn period. After anorectoplasty, daily glycerin enemas were given for postoperative bowel management. Anorectal function of these patients was evaluated using the Kelly score and manometry at the age of 3 to 5 years (LAARP) and 5 to 6 years (PSARP)., Results: Age at evaluation in the LAARP group was younger than that in the PSARP group (51 +/- 10 vs 73 +/- 12 months, P < .01). Kelly score was 3.8 +/- 1.3 vs 3.4 +/- 0.8, respectively. Resting pressure of internal sphincter was 31 +/- 11 vs 33 +/- 10 cm H(2)O, respectively. Relaxation reflex of the internal anal sphincter was observed in 62% (8/13) and 29% (2/7), respectively., Conclusions: Midterm follow-up study revealed that satisfactory fecal continence can be achieved in patients with high-type imperforate anus after LAARP. Laparoscopically assisted anorectoplasty may be a good alternative in this patient population. However, long-term follow-up is necessary to compare the benefits of LAARP against PSARP.

Atypical coarctation of the lower descending or the abdominal aorta is a relatively rare disease which occurs in about 0.5 to 2% of all coarctation cases. The majority of these diseases present with circumscribed narrowing of the abdominal aorta. However, we treated a 7-year-old boy with a rare form: a long, diffuse hypoplasia of the thoracoabdominal aorta.

A 2-month-old boy with biliary atresia (BA) with an extremely rare prenatal history is reported. A cystic lesion in the hepatic hilum was diagnosed during prenatal ultrasonography at 20 weeks' gestation. However, it disappeared during later prenatal observation. He was diagnosed with an uncorrectable type of BA after a massive subdural hemorrhage at the age of 66 days. A fetus suspected of having a cyst of the biliary tree should be followed even after the cyst has disappeared spontaneously.

Purpose: Members of the Japanese Biliary Atresia Society were surveyed to determine their current practice regarding early use of corticosteroids after Kasai's operation., Methods: Questions included the patient's background data, dosage, timing, complications, and outcome. Anicteric survival with the native liver was statistically compared between groups categorized by steroid dosage using Kaplan-Meier analysis., Results: Among 54 institutions surveyed, a total of 222 patients with uncorrectable BA were collected from 34 responders, including 208 patients who received steroid therapy and 14 without it. Prednisolone was started during the first postoperative week in 31% and during the second week in 64%. Perforation and peritonitis occurred in 1 patient given 3 mg/kg/d of prednisolone on postoperative day 1. The survival rates of the steroid and nonsteroid groups were 58.0% and 35.7%, respectively (P = .052). The initial dose of prednisolone was or=4.0 mg/kg/d in 108 patients. The survival rates of the group receiving >or=4.0 mg/kg/d and the nonsteroid group were 58.9% and 35.7%, respectively (P = .0494)., Conclusions: (1) Most surgeons use steroids. (2) Although the anicteric survival rate was higher in the steroid group, the number of patients in the nonsteroid group was too small to draw conclusions. (3) The recommended initial steroid dose is >or=4.0 mg/kg/d. (4) Complications are uncommon if administration is not started too early.

The aim of this study was to evaluate the advantages and complications of endosurgical procedures for benign and malignant pediatric solid tumors. Endosurgical techniques of biopsy and excision were used for diagnosis and treatment of solid tumors, respectively. Since July 1997, a total of 24 biopsies and 24 excisions have been performed laparoscopically for neuroblastoma ( n=24), ovarian solid tumors ( n=10) and other tumors. Seventeen biopsies and six excisions were performed for abdominal neuroblastoma, while ten excisions were performed for ovarian tumor. In these patients, the length of the operation, blood loss, time to start postoperative feeding, time to start postoperative chemotherapy and length of hospital stay were evaluated and compared to the those of the open surgery group. Furthermore, intra- and postoperative complications were analyzed in all patients of both groups. The length of the hospital stay and time to start postoperative feeding were significantly shorter in the group of patients who underwent endosurgical procedures for either abdominal neuroblastoma or ovarian tumor when compared to the open procedure group. The time to start postoperative chemotherapy was shorter only in the abdominal neuroblastoma group. The procedure for two patients undergoing endosurgical tumor excision had to be converted to open surgery due to large tumor size. Two weeks after thoracoscopic excision of a dumb bell-type neurofibroma, one patient underwent open repair of the dura mater because of leakage of cerebrospinal fluid. There were no port-site recurrences in any tumor types. Endosurgical procedures for solid tumors are effective and minimally invasive. However, better indicators are needed for their implementation in order to prevent complications and subsequent conversions to open procedures.

Port-site recurrence (PSR) following laparoscopic procedures has been an unpredictable complication in adult cancer patients; however, no data exist about this phenomenon in the pediatric field. The aim of this study was to determine whether PSR, following endosurgical procedure for malignancies, is a typical complication or a rare event in the pediatric population. Eighty-one questionnaires were mailed to members of The Japanese Society of Pediatric Endosurgeons. They were asked to provide a list of their institutions that had experience with PSR after endosurgical procedures for pediatric malignancies. Among 29 institutions, a total of 129 endosurgical procedures for pediatric malignancies were reported; these included 85 laparoscopic and 44 thoracoscopic procedures, performed on 104 neuroblastomas, 8 hepatoblastomas, 7 nephroblastomas, and 10 other tumors. Of the 104 neuroblastomas, 83 were found by mass screening using high levels of urinary vanillylmandelic acid and homovanillic acid. Sixty-five of the 83 patients had their tumor excised, and 18 had their tumor biopsied by endosurgical procedures. Additionally, 47 of these patients did not require any postoperative chemotherapy. No incidence of PSR was reported in any of the patients that underwent endosurgical procedures. The PSR following endosurgical procedure is a rare phenomenon in the pediatric population. Both, laparoscopic and thoracoscopic procedures, are safe and recommended for treating pediatric malignancies, especially mass-screened neuroblastomas.

A 1-month-old boy with tetralogy of Fallot, pulmonary atresia, right aortic arch, and right ductus arteriosus, exhibited progressive right upper lobar emphysema since his birth. The emphysema was caused by the right ductus arteriosus compressing the right upper bronchus. After division of the ductus arteriosus the emphysema completely regressed. We should explore the cause of lobar emphysema thoroughly before lobectomy especially when it is extrinsic. The emphysema may regress by eliminating the extrinsic factor.

Background/purpose: In infants, neuroblastoma has been known to spontaneously differentiate into a benign ganglioneuroma. Although several investigators have compared mass-screened with unscreened, disseminated with localized, and adrenal with retroperitoneal neuroblastoma, there are very few cross-comparisons of the above parameters. Herein, the authors report the maturation of mass-screened, localized adrenal neuroblastoma., Methods: Fifty-one mass-screened adrenal neuroblastomas were divided into 2 groups. In infants less than 1 year of age (Group A), 45 neuroblastomas were resected, whereas 6 neuroblastomas were resected after observation in 1- to 4-year-old children (group B). Histopathology of the tumors in the 2 groups was compared. Data were analyzed by X(2) test, and P <.05 was considered significant., Results: According to the International Neuroblastoma Pathological Classification, 41 of 45 tumors of group A were "differentiating neuroblastoma" and 4 of 6 tumors of group B were "maturing ganglioneuroma." Maturation toward ganglioneuroblastoma was observed in 16 neuroblastomas of group A (36%) and 6 neuroblastomas of group B (100%). In group A, 58% had low mitosis karyorrhexis index (MKI); all patients in group B had low MKI., Conclusions: If left untreated, maturation of mass-screened, localized adrenal neuroblastomas is a common phenomenon. These children do not need to undergo early operation., (Copyright 2001 by W.B. Saunders Company.)

Background: Laparoscopic surgery in small infants is still an uncommon procedure in Japan. The present study was conducted to evaluate the advantages and disadvantages of laparoscopic surgery in neonates and infants weighing less than 5 kg., Methods: Between July 1997 and November 1999, 54 infants underwent laparoscopic surgery. They were evaluated for length of operation, intra- and postoperative complications, changes in intra-operative body temperature, time to postoperative feeding, length of hospital stay and changes in serum levels of C-reactive protein (CRP), creatinine phosphokinase (CPK) and interleukin (IL)-6 on days 0, 1 and 4. These parameters in the laparoscopic pyloromyotomy (LP) and laparoscopic fundoplication groups were compared with those in the open pyloromyotomy (OP) and open fundoplication groups, respectively, which were performed during the same period., Results: Three laparoscopy cases were converted to open procedures. One case of fundoplication had panperitonitis due to failed gastrostomy and required long-term parenteral nutrition. Time to postoperative feeding and length of hospital stay in the LP group were significantly shorter than in the OP group. In LP group, intra-operative body temperature did not markedly decrease during CO, pneumoperitoneum. Although serum levels of CRP, CPK and IL-6 were elevated in all groups on postoperative day 1, there were no significant differences between the groups., Conclusions: Better quality of life after laparoscopy is a significant advantage over conventional surgical procedures. This advantage not only outweighs the incidence of intra- and postoperative complications in small infants, but further emphasizes the need to improve laparoscopic techniques to avoid complications.

The role of pre- and intraoperative procedures for the localization of insulinomas has been extensively debated. We report a case of successful treatment using preoperative selective intra-arterial calcium injection and intraoperative glucose monitoring. A 12-year-old boy with hypoglycemic attacks had a large insulinoma in the head of the pancreas on computed tomography. Preoperative selective angiography combined with arterial stimulation-venous sampling (ASVS) by intra-arterial injection of calcium revealed no other insulinomas in the body and tail of the pancreas. Elevation of serum glucose on intraoperative monitoring confirmed complete enucleation of the insulinoma. Preoperative ASVS can accurately localize an insulinoma, and may help to increase the success rate of surgery and avoid blind pancreatectomy.

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.

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.