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Purpose: Laparoendoscopic single-site surgery has recently been described in children and regarded as an improved technology leading to less pain and better cosmetic outcome. Compared to the traditional three-port method, it is not known if the single-port method is less invasive. The aim of this study was thus to investigate the post-operative acute inflammatory response in order to evaluate surgical stress in the two surgical approaches in children.Methods: A prospective, single blinded, case-control study was carried out. Thirteen male patients who presented with unilateral varicocele were divided into two groups. Six children underwent single-port laparoscopic procedure, while the other seven children underwent three-port laparoscopic procedure. Pre-operative and post-operative blood samples were taken for the measurement of tumor necrosis factor-α (TNF-α) and interleukin 6 (IL-6) using ELISA. Demographics including the operation time, and complications were recorded. Data between the two groups were analyzed using unpaired t-test and a p value of <0.05 was taken as statistically significant.Results: The mean age of patients was 14.5 years (range 12-19 years). There was no significant difference between the two groups in terms of operative time, nor there was any complication recorded. The change in serum TNF-α and IL-6 concentrations pre- and post-operatively between the single-port group and three-port group was not statistically significant. Overall, patients in the two groups showed excellent satisfaction in terms of post-operative cosmesis.Conclusion: Single-port laparoscopic varicocelectomy is safe, effective and produces excellent cosmesis with minimal surgical stress. [ABSTRACT FROM AUTHOR]

Hirschsprung's disease (HSCR) is a classical model of enteric neuropathy, occurring in approximately 2-2.8 in 10,000 newborns. It is the commonest form of congenital bowel obstruction and is characterized by the absence of enteric ganglia in distal colon. Recent advances in genome-wide association analysis (GWAS) and next generation sequencing (NGS) studies have led to the discovery of a number of new HSCR candidate genes, thereby providing new insights into the genetic architecture and molecular mechanisms of the disease. Altogether, these findings indicated that genetic heterogeneity, variable penetrance and expressivity, and genetic interaction are the pervasive characteristics of HSCR genetics. In this review, we will provide an update on the genetic landscape of HSCR and discuss how the common and rare variants may act together to modulate the phenotypic manifestation. Translating the genetic findings to genetic risk prediction and to optimize clinical outcomes are undoubtedly the ultimate goals for genetic studies on HSCR. From this perspective, we will further discuss the major obstacles in the clinical translation of these latest genetic findings. Lastly, new measures to address these clinical challenges are suggested to advance precision medicine and to develop novel alternative therapies., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background and Methods: Iatrogenic diaphragmatic hernia in childhood is rare, with only scanty case reports available in the literature. It is a potentially life-threatening condition resulting in intestinal obstruction and bowel strangulation. In this study, we retrospectively reviewed four patients with iatrogenic diaphragmatic hernia following right hepatic trisectionectomy with caudate lobectomy and liver transplantation in a tertiary referral centre. The patient characteristics, clinical presentation and risk factors were assessed., Conclusions: Iatrogenic diaphragmatic hernia should be considered in differential diagnosis in paediatric patients presenting with intestinal obstruction or respiratory distress after hepatic resection or liver transplantation for early diagnosis and management.

Introduction: Tracheobronchial obstruction, although uncommon in the pediatric age group, remains a challenging problem. We review the long-term outcome of endoscopic metallic stenting in infants with tracheobronchial obstruction., Materials and Methods: Medical records of all pediatric surgical patients who underwent tracheobronchial metallic stenting in our center were reviewed retrospectively from 1996 to 2014. Patients' demographic data, including etiology, associated anomalies and nature of obstruction were reviewed. Outcome measures include complications such as re-stenosis, granulation tissue, stent migration, fractured stent, maximal tracheal diameter achieved, weaning of ventilator and growth at interval follow-up., Results: Twelve balloon-expandable metallic stents were placed in the trachea (n = 10) and/or bronchi (n = 2) of 5 patients with a median age of 13 months (range 5-30 months). Etiology of the airway obstruction included congenital tracheal stenosis (n = 4), giant cervical and superior mediastinal lymphatic malformation with tracheobronchomalacia (n = 1). Seven complications were reported (3 patients developed granulation tissue, 2 patients had re-stenosis, 1 stent migrated, 1 stent fractured). All patients survived and were in good condition with a median follow-up of 16 years (range 11-18 years). Three patients weaned off ventilator and oxygen., Conclusions: Endoscopic stenting with metallic stent has satisfactory long-term outcome in treating infants with tracheobronchial obstruction.

Introduction: Congenital diaphragmatic hernia is a potentially life-threatening neonatal condition which required surgical intervention. With the advances in endosurgical instruments and techniques, thoracoscopic approach is gaining popularity as a standard procedure in the treatment of this condition. In this study, we reviewed our two centres' experience with thoracoscopic repair of congenital diaphragmatic hernia in recent years., Methods: All patients who underwent thoracoscopic repair of congenital diaphragmatic hernia between 2010 and 2013 at the two tertiary referral centres were identified. Medical records were retrospectively reviewed. Data including patients' demographics, peri-operative outcomes, length of hospitalisation and post-operative complications were extracted and analysed., Results: 60 patients were identified over the study period, with 46 males and 14 females. 48 patients received operation within the first 7 days of life. There were seven patients with delayed presentation and were operated after 1 month old. The average body weight was 3.03 kg. Left-sided hernia was more prevalent (n = 50). The mean operative time was 88.5 min (range 31-194 min). No conversion to open thoracotomy or laparotomy was required in any of the patients. All patients except one were intubated and paralysed in neonatal intensive care units for at least 3 days after operation. Average hospital stay was 14.6 days. There was no mortality in this series. There were five recurrences, one being the patient without post-operative paralysis, and the others with deficient posterior muscle rim. No musculoskeletal deformity was noted on follow-up examination., Conclusion: Thoracoscopic repair of congenital diaphragmatic hernia can be performed safely in specialised centres. The post-operative recovery and cosmesis are excellent. Diaphragmatic hernia with large defect remains a challenge for surgeons.

Background: With the advent of laparoscopic surgery, more choledochal cysts are excised laparoscopically. In this study, we compared the outcomes from laparoscopic hepaticojejunostomy (HJ) and hepaticoduodenostomy (HD) for biliary-enteric reconstruction., Methods: A retrospective analysis of patients who had undergone laparoscopic choledochal cyst excision between February 2005 and January 2014 in a tertiary referral centre was performed. Demographics data, operative techniques and surgical outcomes were analysed according to the way of biliary-enteric reconstruction., Results: A total of 31 patients were identified, 20 of whom underwent HJ and 11 underwent HD. There were no significant differences in terms of demographics. Median operative time was significantly shorter in HD group (211.0 ± 96.4 vs. 386.0 ± 90.4 min, p = 0.001). Although postoperative enteral feeding was initiated later in HD group (5.0 ± 0.8 vs. 4.0 ± 3.6 days, p = 0.036), postoperative stay in intensive care unit (ICU) (0.7 ± 1.0 vs. 2.4 ± 1.7 days, p = 0.007) and overall hospital stay (9.1 ± 1.0 vs. 14.4 ± 12.2 days, p = 0.157) favoured HD group. There was no perioperative mortality. Median follow-up duration was 24.0 (±11.0) months in HD group and 67.5 (±23.7) months in HJ group. One patient in HJ group had postoperative cholangitis related to anastomotic stricture whereas no cholangitis noted in HD group. In total, five patients in HJ group required second operation for complications and residual diseases whereas none in HD group required reoperation., Conclusions: Laparoscopic excision of choledochal cyst with hepaticoduodenostomy reconstruction is safe and feasible with shorter operative time, ICU stay and overall hospital stay. It is not inferior to HJ in terms of short-term postoperative outcomes.

Aim of Study: The objective of this study is to determine the diagnostic value of the 24-h delayed film for Hirschsprung's disease (HD). Other features of the barium enema were also examined, in particular the correlation between the radiological transition zone (TZ) and the final pathology., Methods: All patients with suspected HD from 2003 to 2013 who had undergone barium enema and rectal biopsy were reviewed retrospectively to study the correlation between radiological features of barium enema with the final diagnosis as well as severity., Results: A total of 182 patients were admitted for suspected HD during the study period, of which 82 had both investigations done. 68 patients had radiological features suggestive of the disease and ultimately, 12 patients had the disease confirmed with rectal biopsy. Among those without radiological features of HD, 2 patients were found to have the disease. Thus, the sensitivity of the 24-h delayed film was 85.7 % and the specificity was 17.6 %. The positive predictive value (PPV) of this test was 20.6 % and the negative predictive value (NPV) was 85.7 %. Regarding the level of TZ, it was not detected in the barium enema in 7 (50 %) out of the 14 patients. For those with the presence of TZ, 6 (85.7 %) of them correlated well with the intra-operative findings and 4 (57.1 %) of them correlated well with the final histology., Conclusion: The 24-h delayed film of barium enema has a high NPV and is useful to rule out HD. However, rectal biopsy is still suggested for disease confirmation given its low PPV. Lastly, once present, the level of radiological TZ is also a useful predictor for the actual disease involvement.

Introduction: Data on laparoscopic Nissen fundoplication for gastro-oesophageal reflux disease (GERD) in infants remain limited. We describe our experience with this operation in children and in particular, infants younger than 12 months old., Materials and Methods: Medical records of all paediatric patients who had laparoscopic fundoplication done for GERD from 1998 to 2013 were reviewed. Patients were divided into two groups based on age: group I: 0-12 months, and group II >12 months. Data on indications, patient's demographics, operative time, blood loss, conversions, complications, recurrences and duration of hospitalization were studied., Results: A total of 86 patients were reviewed (group I, n = 21; group II, n = 65). While the mean age and body weight for group I were 8 ± 2.99 months and 6 ± 1.96 kg, the values for group II were 98 ± 65 months and 18 ± 9.6 kg, respectively. All patients had concurrent laparoscopic gastrostomy for feeding difficulties. The mean operative time was shorter in group I (157 ± 55 vs 169 ± 52 min, p = 0.66). Both groups had minimal blood loss only. The surgical outcomes in both groups were comparable in terms of recurrence (0 vs 3 %, p = 0.105) and complications (9.5 vs 6 %, p = 0.275). The median follow-up duration for group I and group II was 23 and 40 months, respectively. Sixteen (76 %) patients in group I and 45 (67 %) patients in group II did not develop pneumonia post fundoplication., Conclusion: Laparoscopic Nissen fundoplication can be safely performed in infants with outcomes comparable to older patients and a shorter operative duration. Low recurrence rate (up to 3 %) is shown by our long term follow-up data. We recommend laparoscopic Nissen fundoplication to be considered in managing infants with GERD.

Purpose: The Hong Kong model for collaboration in education in Asia is based on internationalization. Hong Kong benefits from being an international city combining an Eastern heritage and a Western society. The University of Hong Kong ranks among the world's top 25 universities (2012/2013 QS world university rankings), and its Division of Paediatric Surgery has an international reputation in research and training. In the past two decades, Hong Kong has leading roles in major international pediatric surgical organizations including Pacific Association of Pediatric Surgeons, International Pediatric Endosurgery Group, Asian Association of Pediatric Surgeons and World Federation of Associations of Pediatric Surgeons. While Hong Kong has close collaboration with Japan and other advanced economies, the talk will focus on our transfer of international experience to Mainland China., Methods and Results: (1) A Train-the-Trainer scheme consisting of a 1-year structured education program for next-generation leaders from selected centers enabled replication and proliferation of similar training nationwide. (2) A series of laparoscopic workshops resulted in training of over 1,300 surgeons in basic and advanced skills in minimally invasive surgery within 5 years and the establishment of a national specialist organization overseeing development and quality assurance. (3) A series of Cross-Strait Symposia on Pediatric Oncology established the foundation of multidisciplinary, multicenter collaboration in education and research in Mainland China, Taiwan and Hong Kong. (4) Initiation of a scientific writing course will enhance surgical research and training in China., Conclusion: The Hong Kong experience may serve as a model of collaboration with other developing economies in Asia.

Introduction: The incidence of congenital cystic lung lesions has been increasing in recent years due to better antenatal detection. With the introduction and maturation of thoracoscopy, the operative management for these lesions has seen advancement in the last decade. In this study, we aimed to compare the post-operative outcomes of patients who had thoracoscopic resection with those who underwent open resection., Methods: A retrospective review of all patients who underwent surgery for congenital cystic lung lesions between January 1996 and June 2012 in a tertiary referral center was conducted. Patients' demographics, operative procedures and post-operative outcomes were analyzed., Results: Sixty-seven patients were identified over the past 15 years. Thirty-nine patients had thoracoscopic resections and 28 had open resections. Thirteen patients in the thoracoscopic group required conversion. Both groups had similar demographics in terms of age, body weight and laterality of lesions. The mean operative time and blood loss in the two groups were comparable. Patients in the thoracoscopic group had significantly shorter duration of chest tube drainage (4.3 vs. 6.9 days, p = 0.004), shorter intensive care unit stay (2.5 vs. 5.9 days, p = 0.003) and shorter hospital stay (6.9 vs. 12.0 days, p < 0.001). Post-operative complication rate was similar between the two groups. Patients with body weight less than 5 kg showed a significantly higher conversion to open surgery as compared to those with body weight more than 5 kg (62.5 vs. 25.8 %, p = 0.049)., Conclusion: Successful thoracoscopic resection for congenital cystic lung lesions results in better post-operative outcomes. However, this technique remains technically challenging in patients with body weight less than 5 kg.

Objective: With advances in clinical medicine, many premature babies nowadays can have excellent survival outcomes. As the incidence of inguinal hernias in this group is high and there is scarce data in the literature regarding the optimal timing for repair, this study aims to review our experience in laparoscopic repair in premature infants., Methods: In our centre, premature neonates with inguinal hernia noted during hospitalization were offered laparoscopic repair when the body weights reached 2.5 kg unless there is contraindication for laparoscopy. A retrospective review was carried out for all premature neonates who underwent laparoscopic inguinal hernia repair from 2001 to 2011. The operative results, complications, incarceration risk and postoperative apnea risk were recorded., Result: A total of 79 premature neonates received laparoscopic inguinal hernia repair during this period. The mean gestational age at birth was 31.9 weeks (27-36 weeks) and the mean gestational age at operation was 46.5 weeks (33-92 weeks). One patient had incarceration and required emergency operation while waiting for the elective repair. The mean operative time was 44.9 min (25-93 min). One patient (1.3 %) had recurrence. No postoperative apnea was noted in any patient., Conclusion: Laparoscopic hernia repair is safe and feasible in premature neonates when they attain reasonable body size, as long as there is excellent anaesthesia support. Low risk of incarceration was noted in this study and it is worth waiting for the body weight to build up and hence facilitate laparoscopic repair.

Introduction: Primary spontaneous pneumothorax is a condition that carries significant morbidities and mortalities if not managed properly. Thoracotomy with bullectomy has been the treatment of choice for persistent air leak or recurrence after initial chest drain insertion. With the advancement in minimal invasive surgery, the thoracoscopic approach can dramatically reduce the complications of open thoracotomy. We review our experience in managing spontaneous pneumothorax in children using thoracoscopy., Materials and Methods: The medical records of all patients who were discharged with the diagnosis of spontaneous pneumothorax from 1997 to 2007 were reviewed. The demographic data and management were noted. For those patients who underwent thoracoscopic surgery, the intra-operative findings, post-operative outcomes and complications were compared., Results: A total of 15 patients with spontaneous pneumothorax were identified. They all received chest drain insertion as the primary treatment modality. Nine patients, with mean age 16.1 +/- 0.9 years, failed the initial management and subsequently received thoracoscopic surgery with a mean interval of 7.6 +/- 2.5 days after initial chest drain insertion. Among these patients, bullae were found in seven patients (two patients had more than one bulla). The bullae were excised with mean operative time being 63.9 +/- 25.2 min. No conversion to thoracotomy was reported. All patients received paracetamol as pain control and there was no associated complication. No recurrence was found at follow-up till now., Conclusion: This study confirms that the presence of bulla is commonly seen in patients with spontaneous pneumothorax who fail initial management. Thoracoscopic bullectomy, a safe and effective operation, should be offered to this group of patients.

Background: Inguinal hernias are commonly seen in the paediatric population. Controversies still exist regarding the need for contralateral groin exploration when an unilateral inguinal hernia is presented, since the true incidence of contralateral patent processus vaginalis is not known. With the advent of laparoscopic hernioplasty, the status of the contralateral side can be evaluated at the same setting. Here, we describe our experience in this issue after the introduction of laparoscopic hernioplasty in our unit., Methods: A retrospective review was carried out between October 2002 and January 2008. All patients presented with unilateral inguinal hernias were included. The demographics of the patients and the operative findings at laparoscopy were recorded. Statistics were performed using Student t-test or chi(2) test as appropriate and p < 0.05 was taken as statistically significant., Results: During the study period, 363 children were included in our study, of which there were 292 males and 71 females. 212 patients presented with right-sided hernias and 151 were left-sided. The mean age of patients at presentation was 48.8 months. The incidence of contralateral PPV overall was 39.7%. There was no decrease in incidence of having a contralateral inguinal hernia in relation to age., Conclusion: Laparoscopy can accurately diagnose contralateral PPV in children who undergo unilateral inguinal hernia repair and thus holds an advantage over open herniotomy. Furthermore, there should not be an age criteria for contralateral exploration for surgeons who perform open herniotomy.

Hirschsprung's disease (HSCR) is a developmental disorder characterized by the absence of ganglion cells in the lower digestive tract. Aganglionosis is attributed to a disorder of the enteric nervous system (ENS) whereby ganglion cells fail to innervate the lower gastrointestinal tract during embryonic development. HSCR is a complex disease that results from the interaction of several genes and manifests with low, sex-dependent penetrance and variability in the length of the aganglionic segment. The genetic complexity observed in HSCR can be conceptually understood in light of the molecular and cellular events that take place during the ENS development. DNA alterations in any of the genes involved in the ENS development may interfere with the colonization process, and represent a primary etiology for HSCR. This review will focus on the genes known to be involved in HSCR pathology, how they interact, and on how technology advances are being employed to uncover the pathological processes underlying this disease.

Kasai portoenterostomy has been the treatment of choice for neonates with biliary atresia since its introduction. With the advance in laparoscopic techniques, a few centers have reported the feasibility of performing laparoscopic Kasai portoenterostomy. However, the outcome of this new technique is not known. Here, we aim to evaluate, as the only referral center for liver transplantation, our experience with patients referred for transplantation after failed Kasai portoenterostomy. A retrospective study was carried out between October 1996 and September 2005. The records of all patients with the diagnosis of biliary atresia were retrieved. The type of procedure and clinical outcome of the patients were noted. Early failure of Kasai enterostomy was defined as the need for liver transplantation within 1-year post-Kasai operation. For the period studied, a total of 72 patients with biliary atresia were identified. Sixty-three of the 72 patients had their Kasai portoenterostomies performed openly while nine patients underwent laparoscopic Kasai portoenterostomy in a center experienced in laparoscopic surgery. Six of these patients were referred for transplantation within 1 year, giving the early failure rate of 66.6%. In comparison, the early failure rate for open Kasai procedure was 38.5%. Regarding post-operative complications, one patient who underwent laparoscopic Kasai procedure also suffered intestinal volvulus after initial surgery and another was found to have internal herniation of the Roux loop. Laparoscopic Kasai portoenterostomy seems to be associated with more post-operative complications and worse early clinical outcome. As a result, we remain guarded about the present-day technique of laparoscopy for biliary atresia.

The pathogenesis of neonatal necrotizing enterocolitis (NEC) is unknown. Intestinal dilatation and preferred occurrence of NEC at sites of bacterial overgrowth (colon and ileum) are common findings. The study attempted to produce NEC with increasing intraluminal pressures and bacterial concentrations in two different aged groups of rats. First, 10-cm terminal ileum segments were isolated with intact vascular pedicles in 1-and 3-month-old rats, and a dose of 10(11) E. coli in 1 ml was injected into each segment. Intraluminal pressure was sustained for 1 h at 150, 100, 50 and 0 cmH(2)0, respectively, in four experimental groups ( n=6). The isolated loop was then returned to the abdominal cavity and assessed grossly for NEC after 24 h. Histological examination was performed by a pathologist (KWC) who was blinded to the procedures. Second, the procedure was repeated with doses of 10(8), 10(5) and 0 bacteria/ml ( n=6) at intraluminal pressure of 100 cmH(2)0 in 1-month-old rats. Third, in another experimental group, oxygenation of the pedicled loop was assessed by oximetry as the intraluminal pressure increased and the findings were correlated with aortic blood pressure. The blood pressures (mean+/-SD) for 3- and 1-month-old rats were 110+/-6 and 72+/-4 mmHg, respectively. Hypoxia (<50% oxygen saturation) of the bowel was detected when the intraluminal pressure exceeded the mean blood pressure. The relative incidences of NEC in the bowel with intraluminal pressure above and below mean blood pressure were 100% (6/6) vs. 4% (1/24; P<0.05) in 3-month-old rats, and 100% (12/12) vs. 11% (2/18; P<0.05) in 1-month-old rats. There was no occurrence of NEC in bowel injected with 10(5) E. coli/ml and less at 100 cm intraluminal pressure. Increased intraluminal pressure results in bowel hypoxia and in the presence of adequate bacterial concentration predisposes to the development of NEC. Young age is associated with a lower threshold for increased intraluminal pressure leading to NEC.

To determine if and when intussusception results in endotoxemia and to evaluate whether diagnostic and surgical interventions of intussusception aggravate endotoxemia, intussusception was created in seven pigs during general anesthesia (ileocolic n = 3, ileo-ileocolic n = 2, ileocolic with silicone ring as leadpoint n = 1, ileoileal with silicone ring n = 1). After a period of observation and before progression to bowel gangrene, a diagnostic ultrasound study with a saline enema was undertaken, followed by a laparotomy and resection of the intussusception with a primary bowel anastomosis. Serial blood samples were obtained before and at regular intervals after: (1) formation of the intussusception; (2) the enema; and (3) resection for endotoxin measurement by limulus amebocyte lysate assay. The mean level of endotoxin increased from a pre-intussusception value of 0.750 EU/ml (range: 0.215-1.281) to a post-intussusception maximum of 1.482 EU/ml (0.997-2.882, P = 0.009), peaking mostly at 2 h after the intussusception. The mean pre-saline-enema level was 1.547 EU/ml (0.869-2.677) while the post-enema level was 1.41 EU/ml (0.84-2.468, P = 0.655). The mean pre-resection level was 1.470 EU/ml (0.784-2.468) while the post-resection maximum was (2.130 EU/ml) (0.850-4.381, P = 0.09). It is concluded that: (1) even in the absence of bowel gangrene intussusception results in significant endotoxemia within 2 h of its formation; (2) gentle saline enemas are not associated with endotoxemia; and (3) surgical resection results in a further upward surge of endotoxemia. These findings provide a scientific basis for improved understanding and management of intussusception.

Oesophageal atresia (OA) is often associated with anomalies of other systems. The genetic contribution to the formation of the VACTERL association is not clear. The objective of this study was to evaluate the incidence of associated anomalies in two different racial populations. The associated anomalies in neonates with OA managed in an Asian and a European paediatric surgical centre from 1982 to 1998 were reviewed. Non-Asian and non-European patients were excluded from the respective centres. The incidence of anomalies was compared using Fisher's exact test, taking #E5/E5# below 0.05 as statistically significant. Forty-eight consecutive Asian (25 boys and 23 girls) and 34 consecutive European patients (20 boys and 14 girls) were included in the analysis. The percentage of patients with at least one associated anomaly was 50% and 74% in the Asian and European populations, respectively, which was significantly different (#E5/E5#=0. 04). There was no statistically significant difference in the incidence of associated cardiovascular (29% vs 39%), anorectal (11% vs 18%), and musculoskeletal (16% vs 22%) anomalies, duodenal atresia (4% vs 3%), or Down's syndrome (3% vs 6%) between the two populations. However, the European patients had a significantly higher incidence of urogenital (UG) anomalies (26% vs 4%, #E5/E5#=0.006), the most common being agenesis (n=4) and dysplasia (n=3) of one or both kidneys. Hereditary factors may influence the incidence of associated anomalies in children with OA, particularly of the UG system. However, environmental factors cannot be excluded.

H-type tracheo-oesophageal fistula (TOF) is a rare type of fistula without oesophageal atresia. The symptoms are usually present at birth, but the diagnosis is often delayed. Traditionally, contrast oesophagography and/or fiberoptic bronchoscopy are used to confirm the diagnosis. We describe the imaging features of a case of H-type TOF seen on three-dimensional computed tomography and virtual bronchoscopy and the usefulness of these techniques in pre-operative evaluation.

Solid-cystic papillary tumor (SCPT) of the pancreas is a rare neoplasm in children. The current study attempted to estimate the incidence and possible pathological origin of the tumor. Clinicopathologic features of the children under the age of 16 years with pancreatic tumors managed in a single institution between January 1993 and November 1999 were reviewed. Representative blocks of SCPT specimens were immunostained for neuron-specific enolase (NSE) chromogrannin, synaptophysin, insulin, glucagon, somatostatin, and gastrin. There were three SCPTs, two pancreatic endocrine tumors, and one acinar cell carcinoma during the study period. The estimated yearly incidence in the referral area of 2 million population is about 0.01 pediatric SCPT per year per 100,000 population. The children underwent surgical removal of the tumors. Postoperatively, they were followed up for 6 months to 4 years and were well. Immunostaining for NSE, chromogranin, synaptophysin, insulin, somatostatin, and gastrin was negative in all cases. SCPT may thus be the most common pancreatic tumor in the Asian pediatric population. The pathological origin of the tumor remains unclear and requires further investigations.

Duodenum is thought to go through a solid-core stage followed by recanalization during its development. This study investigates the role of apoptosis in normal duodenal development, especially during widening of the lumen, and hence, the possible role of apoptosis in duodenal atresia (DA). Twenty-four time-mated Sprague-Dawley rats were killed from day 13 to day 20 of gestation. Duodenums of 3 fetuses were chosen randomly from each rat and processed. Apoptosis was determined by the terminal deoxytransferase-mediated biotin dUTP nick-end labeling (TUNEL) technique (ApopTag). Apoptosis count and cross-sectional areas were measured with an image analyzer (MetaMorph). The number of apoptotic cells per unit area duodenum peaked on day 15 for the mucosal/submucosal layer and on day 14 for the muscular/mesenchymal layer. The maximal number of apoptotic cells per cross-section of duodenum was between 7 and 8. The cross-sectional areas of the duodenal wall and lumen increased exponentially between day 17 and day 19 while duodenal-wall thickness remained relatively constant throughout duodenal development. The localization, timing, and intensity of apoptosis do not suggest that apoptosis is responsible for the widening of the duodenal lumen; enlargement of the lumen is related to the increase in duodenal circumference. Apoptosis thus may not be involved in the pathogenesis of DA.

The commonly accepted indication for surgical intervention in necrotizing enterocolitis (NEC) is perforation of the bowel. In this study, the indication and role of surgery was assessed in neonates born with symptomatic congenital heart disease (CHD). Records of neonates admitted to a single institution in Hong Kong between January 1981 and December 1997 with symptomatic CHD who subsequently developed NEC were reviewed. The patients were categorized into cyanotic and acyanotic groups. Of 850 neonates with CHD admitted during the period, 30 developed NEC (3.5%); 17 had cyanotic and 13 had acyanotic heart disease. The average Apgar scores at 1 and 5 min were 7.5 and 8.6, respectively. The mean gestational age was 37.7 weeks and the mean birth weight was 2.5 kg. The mean age at which NEC developed was 16 days. The overall mortality in the proven cases of NEC was 57%. After excluding the suspected NEC cases (stage I), it was found that surgery in the proven NEC cases without perforation, i.e., stages II and IIIA, resulted in higher survival than in those managed medically (75% vs 44%). The cyanotic patients had higher mortality than the acyanotic group (71% vs 39%). Neonates with CHD who develop NEC belong to a unique group of mature babies with reasonable birth weights and Apgar scores, unlike the common NEC patient population. The mortality of these patients is extremely high, and a modified management approach is required. Surgical intervention may be indicated at a much earlier stage of proven NEC before gut perforation occurs.

To assess the prevalence of antenatal diagnosis of small-intestinal atresias (SIA) in the modern era and determine its effect on management and outcome, the records of neonates admitted to a single institution in 1991-1996 with a diagnosis of SIA were reviewed. Duodenal atresia, atresias complicating meconium ileus, and those associated with gastroschisis were excluded. Of 14 neonates with SIA, 10 had jejunal atresia (JA) (Grosfeld type I, n = 1; type II, n = 2; type IIIa, n = 3; type IIIb [apple peel], n = 3; type IV, n = 1) and 4 had ileal atresia (IA) (type II, n = 1; type IIIa, n = 3). Antenatal diagnosis was made in 4 neonates (overall rate = 28%), including all 3 type IIIb JA, and 1 type II JA. None of the IAs were diagnosed antenatally. There were 2 deaths, both in patients with high JAs with less than 10 cm viable bowel. Only 1 of these was antenatally diagnosed. The median (range) times to full enteral feeding were 20 days (17-22) for antenatally-diagnosed JA, 14 days (11-26) for other JAs, and 8 days (6-15) for IAs. Antenatal diagnosis of SIA thus remains relatively infrequent (less than one-third of cases). When an antenatal diagnosis is made, the atresia is more likely to be proximal in location, requiring intensive and prolonged postnatal treatment. Provided a reasonable length of bowel remains, the outcome of SIA, whether antenatally diagnosed or not, is favorable.

Recurrent tracheo-oesophageal fistula (RTOF) occurs in 5%-15% of patients following oesophageal atresia repair. Re-thoracotomy is technically challenging and associated with significant morbidity, including a re-fistulation rate of 10%-22%. Endoscopic occlusion of the RTOF with tissue adhesives (fibrin glue, Histoacryl) is reported to be safe and highly effective. However, long-term results of such therapy are absent from the literature. A postal survey of 13 institutions reporting the use of such treatment regimes for RTOF in the literature between 1974 and 1995 was performed, and data collected concerning the long-term outcomes of their reported patients. Eleven institutions responded to the request for data, providing 22 patients (age range 1 month to 12 years) for review. All had undergone initially successful RTOF closure by endoscopic methods and had been followed up for a median of 107 months (range 3-264 months). There was no morbidity or mortality directly related to the procedure. Overall, only 55% of these endoscopically treated fistulas remained closed long-term. Fistula recurrence invariably occurred within 12 months of successful therapy (median 46 days, range 9-335). Most patients required multiple endoscopic procedures to achieve successful RTOF closure (median 2.0, range 1-4 attempts), although significantly fewer attempts were required with fibrin glue therapy. Surgical re-exploration remains the treatment of choice in the fit child. Endoscopic therapy offers a safe and elegant alternative to high-risk surgery in the sick child, although repeated treatments may be required for successful RTOF closure.

A prospective study of 25 boys who underwent circumcision for medical reason was performed. Specimens of periurethral bacterial flora were taken before operation as well as 3 weeks after surgery, so that each boy acted as his own control. Before circumcision, 13 (52%) harboured uropathogenic organisms (Escherichia coli and other coliforms, Enterococcus spp, Proteus spp, Pseudomonas spp, and Klebsiella spp); after circumcision, none of the boys had uropathogens, the only organisms cultured from the periurethral region being skin commensals. We postulate that circumcision converts a 'cul-de-sac' that is a reservoir of organisms capable of causing ascending urinary tract infection into a surface colonised by natural skin organisms. This study provides circumstantial evidence supporting the idea that circumcision in well-selected patients may confer protection from urine infection.

The combination of neonatal coarctation of the aorta (CoA) and oesophageal atresia (oA) is rare and the optimum approach to surgical correction uncertain. Of 84 consecutive neonates with CoA over an 8-year period, 2 had coexisting oA. We consider that CoA repair by subclavian flap should be performed first to improve the environment for healing of the oesophageal anastomosis. A carefully co-ordinated approach between paediatric and cardiac surgical teams is recommended.