Long-term follow-up of biliary atresia using liver transient elastography.

OBJECTIVE: Liver transient elastography (TE) using FibroScan® has gained popularity as a non-invasive technique to assess hepatic fibrosis by measuring liver stiffness. This study focused on biliary atresia patients post Kasai operation for more than 10 years to prospectively correlate the hepatic fibrosis score to the biochemical changes of liver fibrosis and clinical development of portal hypertensive complications. METHODS: TE was performed in 37 patients who had biliary atresia post Kasai operation done at median age of 60 days. Biochemical indices of liver fibrosis including aspartate aminotransferase/platelet ratio index (APRI) and Fibrosis-4 (FIB-4) score based on age, platelet count, alanine aminotransferase and aspartate aminotransferase level were calculated at the time of TE. Platelet count, spleen size, varices, ascites and hepatic encephalopathy were evaluated as clinical markers of portal hypertension. RESULTS: There were 22 female and 15 male with TE done at median age of 17.0 years. Median FibroScan® fibrosis score was 11.4. Fibrosis score of 6.8 kilopascal (kPa) was taken as the upper reference limit of normal. Nine patients (24%) had normal fibrosis score. Score above or equal to 6.8 kPa was significantly associated with lower platelet level (p = 0.001), higher INR (p = 0.043), higher APRI (p = 0.021), higher FIB-4 score (p = 0.013), and larger splenic diameter (p = 0.004). Higher FibroScan® fibrosis score was also significantly associated with portal hypertensive complications (p = 0.001). CONCLUSIONS: The FibroScan® fibrosis score correlated well with the biochemical changes of liver fibrosis and development of portal hypertensive complications clinically. Screening of portal hypertensive complications such as varices is recommended for patients with raised fibrosis score upon long-term follow-up. LEVEL OF EVIDENCE: Level III, retrospective comparative study.

Biliary Atresia - emerging diagnostic and therapy opportunities.

Biliary Atresia is a devastating pediatric cholangiopathy affecting the bile ducts of the liver. In this review, we describe recent progress in the understanding of liver development with a focus on cholangiocyte differentiation and how use of technical platforms, including rodent, zebrafish and organoid models, advances our understanding of Biliary Atresia. This is followed by a description of potential pathomechanisms, such as autoimmune responses, inflammation, disturbed apical-basal cell polarity, primary cilia dysfunction as well as beta-amyloid accumulation. Finally, we describe current and emerging diagnostic opportunities and recent translation breakthroughs for Biliary Atresia in the area of emerging therapy development, including immunomodulation and organoid-based systems for liver and bile duct repair.

Early Development of Colonic Adenocarcinoma With Minimal Polyposis in a Young Child With Metastatic Hepatoblastoma and Germline APC Mutation.

Germline adenomatous polyposis coli (APC) gene mutation is a cancer-predisposing condition commonly presenting as familial adenomatous polyposis. We describe a patient first diagnosed at the age of 3 years with metastatic hepatoblastoma. With a positive family history, germline testing confirmed maternally inherited APC mutation (p.Thr899Ansfs*13). The patient was subsequently diagnosed at 8 years with colonic adenocarcinoma in the absence of macroscopic polyposis. Total colectomy with adjuvant chemotherapy was delivered and the patient remained disease-free for 5 years since the second diagnosis. This report demonstrates the importance of considering germline APC mutation in children with hepatoblastoma, who may benefit from the early institution of colonoscopic surveillance.

Hepatitis B Virus Seropositivity Is a Poor Prognostic Factor of Pediatric Hepatocellular Carcinoma: a Population-Based Study in Hong Kong and Singapore.

BACKGROUND: Hepatocellular carcinoma (HCC) is a rare hepatic malignancy in children. Hepatitis B virus (HBV) infection is a key predisposing factor in endemic regions but its impact on outcome has not been studied. We aim to evaluate the prognostic implication of HBV seropositivity and role of cancer surveillance in children with HCC from East Asian populations with national HBV vaccination. METHODS: Review of population-based databases for patients (< 18 years old) diagnosed with HCC from 1993 to 2017 in two Southeast Asian regions with universal HBV vaccination (instituted since 1988 and 1987 in Hong Kong and Singapore, respectively). RESULTS: Thirty-nine patients were identified (Hong Kong, 28; Singapore, 11). Thirty were male; median age at diagnosis was 10.8 years (range, 0.98-16.6). Abdominal pain was the commonest presentation while five patients were diagnosed through surveillance for underlying condition. Alpha-fetoprotein was raised in 36 patients (mean, 500,598 ng/ml). Nineteen had bilobar involvement, among the patients in whom pretreatment extent of disease (PRETEXT) staging could retrospectively be assigned, 3 had stage I, 13 had stage II, 4 had stage III, and 11 had stage IV disease. Seventeen had distant metastasis. HBsAg was positive in 19 of 38 patients. Two patients had fibrolamellar HCC. Upfront management involved tumor resection in 16 (liver transplantation, 2), systemic chemotherapy in 21, interventional procedures in 6 [transarterial chemoembolization (TACE), 5, radiofrequency ablation (RFA), 1], and radiotherapy in 4 (selective internal radiation, 3, external beam radiation, 1). Five-year event-free survival (EFS) and overall survival (OS) were 15.4 ± 6.0 and 26.1 ± 7.2%, respectively. Patient's HBsAg positivity, metastatic disease and inability to undergo definitive resection represent poor prognostic factors in univariate and multivariable analyses. Patients diagnosed by surveillance had significantly better outcome. CONCLUSION: Pediatric HCC has poor outcome. HBV status remains relevant in the era of universal HBV vaccination. HBV carrier has inferior outcome and use of surveillance may mitigate disease course.

Thoracoscopic removal of a displaced thoracoamniotic shunt in a newborn with antenatal pleural effusion-a case report.

Thoracoamniotic shunt has been considered as a treatment for antenatal pleural effusion and complication is rare. In majority of cases, the shunt can be removed uneventfully. In this article, we reported a rare complication of shunt migration resulting in the need of thoracoscopic removal at newborn period. The patient born at 39+3 weeks of gestation suffered from antenatal chylothorax detected at 28 weeks and was managed by intrauterine thoracoamniotic shunt insertion. This was complicated by shunt displacement, which caused respiratory distress after birth requiring ventilatory support and progressive pleural effusion in this patient. To prevent further neonatal compromise, thoracoscopic removal of the retained shunt was done on day 7 of life followed by post-op chest drain insertion. Post-op condition was stable with resolution of respiratory distress, and the patient was discharge on post-op day 16. We would like to remind clinicians about this potential complication of thoracoaminotic shunt, which can pose a potential risk of severe neonatal compromise, and that it can be managed by minimal invasive surgery even in the newborn period.

Haematological and immunological data of Chinese children infected with coronavirus disease 2019.

Haematological and immunological data of children with COVID-19 infection is lacking. Between 21st January and 20th March 2020, 244 children who were confirmed to have COVID-19 infection and admitted to the Wuhan Children's Hospital, China were retrospectively reviewed. 193 children were considered as symptomatic, which was defined as having either the presence of clinical symptoms or the presence of CT thorax abnormalities. Their haematological and immunological profiles, including complete blood counts, lymphocyte subsets (T, B and NK cell counts), immunoglobulin (Ig) profiles (IgG, IgA and IgM) and cytokine profiles were analysed and compared between the symptomatic and asymptomatic groups. The median values and the interquartile ranges were calculated. Comparison was made using the Mann-Whitney U test. Children with symptomatic COVID-19 infection had significantly lower haemoglobin levels, but higher absolute lymphocyte and monocyte counts, IgG and IgA levels, as well as interleukin 6 (IL-6), IL-10, tumour necrosis factor alpha and interferon gamma levels. The obtained data will be utilized for further studies in comparing children and adults with COVID-19 infections in other parts of the world and with different severity .

Short-term and long-term outcomes after Roux-en-Y hepaticojejunostomy versus hepaticoduodenostomy following laparoscopic excision of choledochal cyst in children.

BACKGROUND: Choledochal cysts are congenital dilations of the biliary tree. Complete cyst excision and biliary-enteric reconstruction have been the standard operations. In our center, more than 95% of choledochal cyst excision is now performed laparoscopically. Majority of current studies describe laparoscopic-assisted reconstruction using Roux-en-Y hepaticojejunostomy (HJ). However, only a few have studied laparoscopic hepaticoduodenostomy (HD) as an alternative method of biliary-enteric reconstruction. In this study, we focused on comparing longer-term outcomes between laparoscopic HJ and HD reconstruction following choledochal cyst excision. METHODS: We performed retrospective analysis of 54 children who had undergone laparoscopic choledochal cyst excision and biliary-enteric reconstruction between October 2004 and April 2018. Short-term outcomes including operative time, complications such as anastomotic leakage and bleeding, and hospital stays were included. Long-term outcomes including contrast reflux into biliary tree, cholangitis, anastomotic strictures, and need of reoperation were analyzed. RESULTS: Of the 54 patients, 21 of them underwent laparoscopic HD and 33 underwent laparoscopic Roux-en-Y HJ anastomosis reconstruction. There were no significant differences in gestation, gender, age at operation, antenatal diagnosis, and Todani type of choledochal cyst between HD and HJ group. Operative time was significantly shortened in HD group (p = 0.001). Median time to enteral feeding was 3 days in both groups. Median intensive care unit (p = 0.001) and hospital stay (p = 0.019) were significantly shorter in HD group. There was no perioperative mortality. There was no significant difference in anastomotic leakage requiring reoperation (p = 0.743). There were no significant differences in long-term outcomes including anastomotic stricture (p = 0.097), cholangitis (p = 0.061), symptoms of recurrent abdominal pain or gastritis (p = 0.071), or need of reoperation (p = 0.326). All patients had normal postoperative serum bilirubin level. CONCLUSIONS: Laparoscopic excision of choledochal cyst with HD reconstruction is safe and feasible with better short-term outcomes and comparable long-term outcomes compared to Roux-en-Y HJ reconstruction.

Determining the optimal timing of liver transplant for pediatric patients after Kasai portoenterostomy based on disease severity scores.

BACKGROUND: The objective of this study was to determine the most optimal timing of liver transplant (LT) for post-Kasai portoenterostomy (KPE) patients based on disease severity scores. METHODS: This was a retrospective study and the clinical data of all LT recipients aged <18 years (n = 89) with a history of KPE were analyzed. They were divided into three groups according to their PELD/MELD scores at the time of LT (A: <15; B: 15-25; C: >25). The effects of LT on the clinical outcomes and hospitalization status were analyzed. RESULTS: There were 33, 34 and 22 patients in group A, B and C, respectively. There was no significant difference in 3-year graft survival rate between the three groups but group C patients had the highest incidence of vascular or biliary complications (p = 0.022). Group C patients had a significantly lower hospital admission frequency (p = 0.036) and shorter hospital stay (p = 0.041) after LT when compared with their pre-LT status and with non-LT patients with similar disease severity scores. On the other hand, the hospitalization frequency and duration were similar in patients with the lowest disease severity score (group A) before, after and without LT. CONCLUSIONS: The benefit of LT was less obvious when the disease severity score is <15. A high complication rate was reported when LT was performed at a score > 25. Donor availability, the patient's general condition and parental wish should be considered during individual assessment. TYPE OF STUDY: Clinical research paper. LEVEL OF EVIDENCE: Level III.

Treatment outcome and pattern of failure in hepatoblastoma treated with a consensus protocol in Hong Kong.

BACKGROUND AND AIM: We reviewed the results and pattern of failure of the consensus HB/HCC 1996 treatment protocol for pediatric hepatoblastoma (HB) in Hong Kong. The role of SIOPEL and Children's Hepatic tumors International Collaboration (CHIC) risk stratification was evaluated. METHODS: Patients enrolled on the protocol from 1996 to 2014 were included. PRETEXT staging, SIOPEL, and CHIC risk groups were retrospectively assigned. RESULTS: Sixty patients were enrolled with median age at diagnosis of 1.1 years and median follow-up time of 6.8 years. Alpha-fetoprotein (AFP) was raised (>100 ng/mL) in 58 (97%) patients. Five (8%) had metastases at presentation and 7 (12%) experienced tumor rupture prior to or during treatment. Twenty-nine patients (48%) received a first-line cisplatin, 5-fluorouracil, and vincristine regimen only while 23 (38%) also had alternative chemotherapeutic agents. Hepatic resection could be performed in 48 (80%) patients. Three (5%) patients underwent upfront liver transplantation. Five-year event-free survival and overall survival rates were 69.2% ± 6.1% and 77.6% ± 5.5% respectively. Among the 16 patients with relapse/progression, 9 had intrahepatic failure only, 5 had distant failure only, and 2 had combined local and distant failure. Predictors of inferior outcome included advanced Evans staging, disease involving both lobes, rupture, low AFP, and suboptimal response to first-line chemotherapy. Assigned in 44 patients, PRETEXT staging, SIOPEL, and CHIC risk groups significantly predicted EFS and OS. CONCLUSIONS: Although the consensus HB/HCC 1996 protocol led to cure in three-quarters of pediatric HB patients, an upfront risk stratification system is required to identify and improve the outcome of high-risk patients.

Recent trends in the prevalence of Helicobacter Pylori in symptomatic children: A 12-year retrospective study in a tertiary centre.

OBJECTIVE: Helicobacter pylori infection is common among Asians. However, evidence in the recent years has demonstrated a decrease in the prevalence of H. pylori infection among children and adults worldwide. Our aim was to update its prevalence in symptomatic children in our locality in the recent 12 years and compared to the results of our previous review published in 2005. METHODS: A retrospective review was carried out between 2005 and 2017. All children who presented with dyspepsia or gastrointestinal bleeding and underwent oesophagogastroduodenoscopy with antral biopsy taken were included. Patient demographics, endoscopic, or histological diagnosis and the H. pylori status were recorded. MAIN RESULTS: A total of 602 patients were included. There was a statistically significant decreasing trend of H. pylori infection rate between 2005 and 2017 (p = 0.003). The overall infection rate from this study was 12.8%, compared to 25.6% from our previous review. Overall failure of eradication with first-line antibiotic therapy has increased to 29.3% from 10% in our previous review. CONCLUSION: There was a decrease in the prevalence of H. pylori infection among symptomatic children for the recent 12 years, comparing to our previous data from 2005. We hypothesize that the reduction in prevalence of H. pylori infection among adults and the decrease in the practice of sharing chopsticks during meals have led to a decrease in transmission of the bacteria among family members in Hong Kong. However, the failure of eradication with first line treatment was higher, possibly due to the increase in antibiotics usage and resistance. LEVEL OF EVIDENCE: III.

Peroral Endoscopic Myotomy with EndoFLIP and Double-Endoscope: Novel Techniques for Achalasia in Pediatric Population.

BACKGROUND: Experience of peroral endoscopic myotomy (POEM) for treatment of achalasia in pediatric population is limited with varying techniques in different centers. The accurate extent of submucosal tunneling into the gastric cardia and the adequacy of myotomy are the important determining factors to success of POEM. A majority of studies in pediatric population have described using submucosal dye injection for assessing the adequacy of myotomy, however, this is a rather crude and inaccurate method. We herein describe the first case of pediatric achalasia managed with POEM incorporated with novel combined techniques using EndoFLIP® (Endoluminal Functional Lumen Imaging Probe) and double endoscope. METHODS: Esophagogastric junction (EGJ) was identified with a gastroscope. Before POEM, EndoFLIP showed EGJ distensibility index of 1.7 mm2/mmHg. Submucosal tunnel was created from the mucosal entry site at midesophagus down and ∼3 cm beyond the EGJ. Anterior myotomy cutting the circular muscle layer while preserving the longitudinal muscle was performed for 8 cm. Double-endoscope technique was used to confirm the adequacy of myotomy by inserting a smaller endoscope through nostril into stomach and observing the transillumination of the first endoscope at the end of submucosal tunnel. After POEM, repeat EndoFLIP measurements revealed increased distensibility index to 6.0 mm2/mmHg. Endoscopic examination at the end of the procedure showed widely patent EGJ. RESULTS: Eckardt symptoms score improved from 9 to 0. At 7 month after POEM, esophagoscopy showed widely open EGJ with no esophagitis, and high-resolution esophageal manometry revealed normalized lower esophageal sphincter pressure and resting tone. CONCLUSIONS: We introduced the intraoperative use of EndoFLIP system that allows real-time assessment of EGJ distensibility and immediate treatment effect evaluation. Incorporation of double-endoscope POEM was also first described in our pediatric patient for ensuring complete gastric myotomy. From our experiences, POEM for achalasia in pediatric population appears to have encouraging results similar to adult patients.

Advances in paediatric gastroenterology.

Recent developments in paediatric gastrointestinal surgery have focused on minimally invasive surgery, the accumulation of high-quality clinical evidence, and scientific research. The benefits of minimally invasive surgery for common disorders like appendicitis and hypertrophic pyloric stenosis are all supported by good clinical evidence. Although minimally invasive surgery has been extended to neonatal surgery, it is difficult to establish its role for neonatal disorders such as oesophageal atresia and biliary atresia through clinical trials because of the rarity of these disorders. Advances in treatments for biliary atresia and necrotising enterocolitis have been achieved through specialisation, multidisciplinary management, and multicentre collaboration in research; similarly robust clinical evidence for other rare gastrointestinal disorders is needed. As more neonates with gastrointestinal diseases survive into adulthood, their long-term sequelae will also need evidence-based multidisciplinary care. Identifying cures for long-term problems of a complex developmental anomaly such as Hirschsprung's disease will rely on unravelling its pathogenesis through genetics and the development of stem-cell therapy.

Surgical Intervention for Primary Spontaneous Pneumothorax in Pediatric Population: When and Why?

INTRODUCTION: Spontaneous pneumothorax in pediatric patients is relatively uncommon. The management strategy varies in different centers due to dearth of evidence-based pediatric guidelines. In this study, we reviewed our experience of thoracoscopic management of primary spontaneous pneumothorax (PSP) in children and identified risk factors associated with postoperative air leakage and recurrence. MATERIALS AND METHODS: We performed a retrospective analysis of pediatric patients who had PSP and underwent surgical management in our institution between April 2008 and March 2015. Demographic data, radiological findings, interventions, and surgical outcomes were analyzed. RESULTS: A total of 92 patients with 110 thoracoscopic surgery for PSP were identified. The indications for surgery were failed nonoperative management with persistent air leakage in 32.7%, recurrent ipsilateral pneumothorax in 36.4%, first contralateral pneumothorax in 14.5%, bilateral pneumothorax in 10%, and significant hemopneumothorax in 5.5%. Bulla was identified in 101 thoracoscopy (91.8%) with stapled bullectomy performed. 14.5% patients had persistent postoperative air leakage and treated with reinsertion of thoracostomy tube and chemical pleurodesis. 17.3% patients had postoperative recurrence occurred at mean time of 11 months. Operation within 7 days of symptoms onset was associated with less postoperative air leakage (P = .04). Bilateral pneumothorax and those with abnormal radiographic features had significantly more postoperative air leakage (P = .002, P < .01 respectively) and recurrence (P < .01, P = .007). CONCLUSION: Early thoracoscopic mechanical pleurodesis and stapled bullectomy after thoracostomy tube insertion could be offered as a primary option for management of large PSP in pediatric population, since most of these patients had bulla identified as the culprit of the disease.

Genetic study of congenital bile-duct dilatation identifies de novo and inherited variants in functionally related genes.

BACKGROUND: Congenital dilatation of the bile-duct (CDD) is a rare, mostly sporadic, disorder that results in bile retention with severe associated complications. CDD affects mainly Asians. To our knowledge, no genetic study has ever been conducted. METHODS: We aim to identify genetic risk factors by a "trio-based" exome-sequencing approach, whereby 31 CDD probands and their unaffected parents were exome-sequenced. Seven-hundred controls from the local population were used to detect gene-sets significantly enriched with rare variants in CDD patients. RESULTS: Twenty-one predicted damaging de novo variants (DNVs; 4 protein truncating and 17 missense) were identified in several evolutionarily constrained genes (p < 0.01). Six genes carrying DNVs were associated with human developmental disorders involving epithelial, connective or bone morphologies (PXDN, RTEL1, ANKRD11, MAP2K1, CYLD, ACAN) and four linked with cholangio- and hepatocellular carcinomas (PIK3CA, TLN1 CYLD, MAP2K1). Importantly, CDD patients have an excess of DNVs in cancer-related genes (p < 0.025). Thirteen genes were recurrently mutated at different sites, forming compound heterozygotes or functionally related complexes within patients. CONCLUSIONS: Our data supports a strong genetic basis for CDD and show that CDD is not only genetically heterogeneous but also non-monogenic, requiring mutations in more than one genes for the disease to develop. The data is consistent with the rarity and sporadic presentation of CDD.

Application of anchoring stitch prevents rectal prolapse in laparoscopic assisted anorectal pullthrough.

BACKGROUND: Rectal prolapse has been reported after laparoscopic assisted anorectal pullthrough in children with anorectal malformation. We report our clinical outcome and study the application of an anchoring stitch to tack the rectum to the presacral fascia and the occurrence of rectal prolapse. MATERIAL AND METHODS: A retrospective review of all children who had undergone laparoscopic assisted anorectal pullthrough for anorectal malformation from 2000 to 2015 was performed. Patients were divided into two groups (group I: with anchoring stitch, group II: without anchoring stitch). Outcome measures including rectal prolapse, soiling, voluntary bowel control, and constipation, and Kelly Score were analyzed. RESULTS: There were thirty-four patients (group I, n=20; group II, n=14) undergoing laparoscopic assisted anorectal pullthrough during the study period. The median follow up duration for group I and group II was 60months and 168months, respectively. All patients had stoma performed prior to the operation. Both groups consisted of patients with high type (30% vs 57%, p=0.12) and intermediate type (70% vs 43%, p=0.12) anorectal malformation. Seven (35%) patients in group I and 3 (21%) in group II had concomitant vertebral and spinal cord pathologies (p=0.408). The mean operative time was significantly shorter in group I (193±63min vs 242±49min, p=0.048). Rectal prolapse occurred less in group I, 4 (20%) vs 9 (64%) patients in group II and was statistically significant (p=0.008). Median time to development of rectal prolapse was 7months in group I and 5months in group II (p=0.767). Mucosectomy was performed in 15% of group I and 36% of group II (p=0.171). Soiling occurred less in group I (55% vs 79%, p=0.167). Voluntary bowel control (85% vs 93%, p=0.499) and constipation (55% vs 64%, p=0.601) were comparable in both groups. 75% in group I and 71% in group II achieved a Kelly score of 5 or above (p=0.823). CONCLUSIONS: Our study showed application of anchoring stitch reduces rectal prolapse and soiling in laparoscopic assisted anorectal pullthrough. Treatment Study-Level III.

Childhood intussusception: 17-year experience at a tertiary referral centre in Hong Kong.

OBJECTIVES: To review all paediatric patients with intussusception over the last 17 years. DESIGN: Retrospective case series. SETTING: A tertiary centre in Hong Kong. PATIENTS: Children who presented with intussusception from January 1997 to December 2014 were reviewed. MAIN OUTCOME MEASURES: The duration of symptoms, successful treatment modalities, complication rate, and length of hospital stay were studied. RESULTS: A total of 173 children (108 male, 65 female) presented to our hospital with intussusception during the study period. Their median age at presentation was 12.5 months (range, 2 months to 16 years) and the mean duration of symptoms was 2.3 (standard deviation, 1.8) days. Vomiting was the most common symptom (76.3%) followed by abdominal pain (46.2%), per rectal bleeding or red currant jelly stool (40.5%), and a palpable abdominal mass (39.3%). Overall, 160 patients proceeded to pneumatic or hydrostatic reduction, among whom 127 (79.4%) were successful. Three (1.9%) patients had bowel perforation during the procedure. Early recurrence of intussusception occurred in four (3.1%) patients with non-operative reduction. No recurrence was reported in the operative group. The presence of a palpable abdominal mass was a risk factor for operative treatment (relative risk=2.0; 95% confidence interval, 1.8-2.2). Analysis of our results suggested that duration of symptoms did not affect the success rate of non-operative reduction. CONCLUSIONS: Non-operative reduction has a high success rate and low complication rate, but the presence of a palpable abdominal mass is a risk factor for failure. Operative intervention should not be delayed in those patients who encounter difficult or doubtful non-operative reduction.

Laparoscopic versus open operation for perforated peptic ulcer in pediatric patients: A 10-year experience.

BACKGROUND: Perforated peptic ulcer (PPU) is a relatively uncommon condition in children. We aim to evaluate and compare the outcomes of laparoscopic omental patch repair versus open repair for PPU in pediatric patients. METHODS: Children who underwent omental patch repair for PPU from 2004 to 2014 in our hospital were reviewed retrospectively. Patient demographics, perioperative as well as intraoperative details and surgical outcomes, were analyzed. RESULTS: Thirteen patients were identified, and all presented with abdominal pain. The median age of the study group was 14.9years (range 6.3 to 18.4years). Radiological evidence of pneumoperitoneum on erect chest x-ray (CXR) was found only in five patients (38.5%). None of the patients had a known history of peptic ulcer disease. Diagnosis other than PPU was made in five patients preoperatively. Laparoscopic repair was attempted in eight patients with one of them requiring conversion. There was no significant difference in patient demographics when compared with the open repair group. The perforation site was in the duodenum in 11 patients and in the antrum in two patients. The mean size of perforation was larger in the open repair group (p=0.005). Although the operating time was longer in the laparoscopic group (p=0.51), the length of hospital stay was significantly shorter (p=0.048). Only two patient diseases were Helicobacter pylori related. CONCLUSION: Clinical features of perforated peptic ulcer in children are different from adults. Risk factors are less frequently identified. Laparoscopic omental patch repair is a feasible surgical option and is associated with satisfactory outcomes in pediatric practice.

Biliary-enteric reconstruction with hepaticoduodenostomy following laparoscopic excision of choledochal cyst is associated with better postoperative outcomes: a single-centre experience.

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.

Little girl who conquered the "ALPPS''.

An insufficient future liver remnant (FLR) is associated with post-hepatectomy liver failure. Associating liver partition and portal vein ligation for stage hepatectomy (ALPPS) has been shown to be effective for the induction of rapid FLR hypertrophy so as to improve the resectability in patients with insufficient FLR. We hereby report our experience of this novel approach for a 6-year-old patient with hepatoblastoma. Computed tomography showed a hepatoblastoma measuring 12.5 cm × 9.9 cm × 11.7 cm in the right liver (Couinaud segment IV, V and VIII). Volumetric assessment of the FLR i.e., left lateral section was 112.6 mL i.e., 21.2% of the estimated total liver volume. In view of the small-for-size FLR, ALPPS was contemplated. An anterior approach was adopted for the in-situ parenchymal split without mobilisation of the right liver. FLR volumetry on the seventh postoperative day was 160.7 mL, which represented a 46.1% gain in volume, and a FLR/ESLV ratio of 30.2%. A right trisectionectomy was performed on the eighth postoperative day. Postoperative recovery was uneventful. Patient was discharged on day 16 after the first operation. To our knowledge, this was the first report that showed the applicability of ALPPS to a paediatric patient.

Thoracoscopic bullectomy for primary spontaneous pneumothorax in pediatric patients.

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.