Treatment and outcomes of high-risk neuroblastoma in Southeast Asia: a single-institution experience and review of the literature

INTRODUCTION@#In Europe and North America, the majority of children with high-risk neuroblastoma survive the disease. Elsewhere, the treatment outcomes are poor.@*METHODS@#A retrospective review of children treated for high-risk neuroblastoma in a single institution in Singapore from 2007 to 2019 was carried out. Treatment consisted of intensive chemotherapy, surgery aimed at gross total resection of residual disease after chemotherapy, consolidation with high-dose therapy followed by autologous stem cell rescue, and radiotherapy to the primary and metastatic sites followed by maintenance treatment with either cis-retinoic acid or anti-disialoganglioside monoclonal antibody therapy. Survival data were examined on certain clinical and laboratory factors.@*RESULTS@#There were 57 children (32 male) treated for high-risk neuroblastoma. Their mean age was 3.9 (range 0.7-14.9) years. The median follow-up time was 5.5 (range 1.8-13.0) years for the surviving patients. There were 31 survivors, with 27 patients surviving in first remission, and the five-year overall survival and event-free survival rates were 52.5% and 47.4%, respectively. On log-rank testing, only the group of 17 patients who were exclusively treated at our centre had a survival advantage. Their five-year overall survival rate compared to patients whose initial chemotherapy was done elsewhere was 81.6% versus 41.1% (P = 0.011), and that of event-free survival was 69.7% versus 36.1% (P = 0.032). Published treatment results were obtained from four countries in Southeast Asia with five-year overall survival rates from 13.5% to 28.2%.@*CONCLUSION@#Intensified medical and surgical treatment for high-risk neuroblastoma proved to be effective, with superior survival rates compared to previous data from Southeast Asia.

Abdominal neuroblastoma in a child with inferior vena cava anomaly

Congenital anomalies of the inferior vena cava are rare. Such anomalies pose great challenges to the surgeon in neuroblastoma surgery, especially when unrecognised preoperatively. We report the first case of an abdominal neuroblastoma detected in a child with a developmental anomaly of the left-sided inferior vena cava. The patient underwent surgical resection after good response to preoperative chemotherapy.

Perforated appendicitis in children: benefits of early laparoscopic surgery

<p><b>INTRODUCTION</b>The aim of this study was to analyse the feasibility, safety and benefits of laparoscopic appendicectomy (LA) in comparison with open appendicectomy (OA) for perforated appendicitis (PA) in children.</p><p><b>MATERIALS AND METHODS</b>A retrospective analysis of all consecutive cases of PA who underwent OA or LA between July 2001 and April 2004 was done. The patient demographics, duration of symptoms and operative findings were noted and the feasibility, safety and benefits of LA were analysed with respect to postoperative recovery and complications.</p><p><b>RESULTS</b>One hundred and thirty-seven consecutive patients with PA underwent either OA (n = 46) or LA (n = 91). Both groups were comparable with respect to patient demographics, duration of symptoms and operative findings. The mean operative time was 106.5 min (95% CI, 100.2 - 112.8) in the LA group and 92.8 min (95% CI, 82.9-102.7) in the OA group (P = 0.02). The return to afebrile status after surgery was significantly faster in the LA group [mean, 45.4 hours (95% CI, 36.8-54)] than the OA group [mean, 77 hours (95% CI 56.7-97.3)] (P = 0.007). The mean duration for postoperative opioid analgesia was 2.5 days (95% CI, 2.2-2.7) for LA and 3.2 days (95% CI, 2.9- 3.6) for OA (P = 0.001). The resumption of oral feeds after surgery was at 3.1 days (95% CI, 2.8-3.3) for LA and 3.7 days (3.4-4.1) for OA (P = 0.005). The length of the hospital stay was shorter in the LA group [mean, 6.5 days (95% CI, 6.1-6.8)] as compared to that of the OA group [mean, 8.2 days (95% CI, 7.1-9.3)] (P = 0.006). Postoperative complications included wound infection, adhesive intestinal obstruction and pelvic abscess formation. The incidence of these complications was 5.6% in the LA group and 19.6% in the OA group (P = 0.01). Nine patients (9.8%) needed conversion to open surgery in the LA group. None of the LA patients had wound infection.</p><p><b>CONCLUSION</b>LA is feasible, safe and beneficial in children with PA.</p>

Laparoscopic appendicectomy in children: A trainee's perspective

<p><b>INTRODUCTION</b>The emergence of operative laparoscopy has brought a great demand for training surgical residents, but there are no clear guidelines regarding choice of procedure for training, proficiency assessment and accreditation. We aim to examine from a trainee's point of view, the safety and efficacy of laparoscopic appendicectomy (LA) as a laparoscopic training procedure.</p><p><b>MATERIALS AND METHODS</b>A retrospective analysis of all the consecutive cases of LA done by a trainee in paediatric laparoscopic surgery from September 2003 to February 2005 was done.</p><p><b>RESULTS</b>A total of 70 consecutive patients (37 males and 33 females) aged 3 to 15 years (mean, 10.5 +/- 2.5) with suspected appendicitis underwent LA. Twelve (17.1%) patients had acute appendicitis, 25 (35.7%) had acute suppurative appendicitis adherent to the caecum with localised and/or pelvic pus pocketing, 29 (41.4%) had perforated appendicitis with generalised peritonitis and 4 (5.7%) had normal appendix [mesenteric adenitis (1), omental infarct (1), torted Meckel's diverticulum (1) and primary peritonitis (1)]. There were no operative complications and none required conversion to open surgery. The operative duration ranged from 25 to 110 minutes (mean, 55.6 +/- 23.4). There were two complications; one had adhesive intestinal obstruction and underwent successful laparoscopic adhesiolysis and one had umbilical wound infection.</p><p><b>CONCLUSIONS</b>LA is a safe and effective laparoscopic training procedure, in addition to being effective for all stages of appendicitis, as well as concurrent lesions encountered in children with suspected appendicitis.</p>

Bile duct perforation in children: is it truly spontaneous?

<p><b>INTRODUCTION</b>Bile duct perforation (BDP) with resultant biliary ascites in children is a rare clinical condition. The aetiopathogenesis is still an enigma, with increasing evidence suggesting anomalous union of pancreaticobiliary ductal (AUPBD) system as the prime causative factor.</p><p><b>CLINICAL PICTURE</b>We report 2 cases of spontaneous perforation of the bile duct confirmed on histopathological examination as choledochal cyst, in a 6-month-old female child and a 4-year-old boy who presented with subtle clinical symptoms.</p><p><b>TREATMENT AND OUTCOME</b>Both patients were successfully managed by excision of the gall bladder and common bile duct and Roux-en- Y hepaticojejunostomy. This procedure was performed following initial cholecystostomy drainage in the second case.</p><p><b>CONCLUSIONS</b>From the available literature and experience with our patients, BDP is not merely spontaneous but may be related to AUPBD and choledochal cyst.</p>