Establishing disability weights for congenital pediatric surgical conditions: a multi-modal approach.

BACKGROUND: Burden of disease (BoD) as measured by Disability-Adjusted Life Years (DALYs) is one of the criteria for priority-setting in health care resource allocation. DALYs incorporate disability weights (DWs), which are currently expert-derived estimates or non-existent for most pediatric surgical conditions. The objective of this study is to establish DWs for a subset of key pediatric congenital anomalies using a range of health valuation metrics with caregivers in both high- and low-resource settings. METHOD: We described 15 health states to health professionals (physicians, nurses, social workers, and therapists) and community caregivers in Kenya and Canada. The health states summaries were expert- and community-derived, consisting of a narrated description of the disease and a functional profile described in EQ-5D-5 L style. DWs for each health state were elicited using four health valuation exercises (preference ranking, visual analogue scale (VAS), paired comparison (PC), and time trade-off (TTO)). The PC data were anchored internally to the TTO and externally to existing data to yield DWs for each health state on a scale from 0 (health) to 1 (dead). Any differences in DWs between the two countries were analyzed. RESULTS: In total, 154 participants, matched by profession, were recruited from Kijabe, Kenya (n = 78) and Hamilton, Canada (n = 76). Overall calculated DWs for 15 health states ranged from 0.13 to 0.77, with little difference between countries (intra-class coefficient 0.97). However, DWs generated in Kenya for severe hypospadias and undescended testes were higher than Canadian-derived DWs (p = 0.04 and p < 0.003, respectively). CONCLUSIONS: We have derived country-specific DWs for pediatric congenital anomalies using several low-cost methods and inter-professional and community caregivers. The TTO-anchored PC method appears best suited for future use. The majority of DWs do not appear to differ significantly between the two cultural contexts and could be used to inform further work of estimating the burden of global pediatric surgical disease. Care should be taken in comparing the DWs obtained in the current study to the existent list of DWs because methodological differences may impact on their compatibility.

Quantifying the Disability from Congenital Anomalies Averted Through Pediatric Surgery: A Cross-Sectional Comparison of a Pediatric Surgical Unit in Kenya and Canada.

BACKGROUND: Pediatric surgical practice is different in low- and middle-income countries as compared to North America. While resources are limited, the impact of pediatric surgical procedures is significant. The objective of this study was to calculate and compare disability-adjusted life years (DALYs) averted in a Kenyan and Canadian surgical unit for a subset of pediatric congenital anomalies. METHODS: Medical records of children having undergone surgical procedures for 13 congenital conditions in both surgical units were collected over 12 months. DALYs for each condition were calculated using previously obtained disability weights derived in each country. Age-adjusted life expectancy rates from the WHO were used to determine years of life lost. Risk of permanent disability without surgery and probability of successful treatment values were obtained from the literature and included in the DALY calculation. RESULTS: The conditions accounting for the largest total number of averted DALYs in Kenya were hydrocephalus (60.8%) and spina bifida (18.1%), whereas in Canada they were hydrocephalus (24.2%) and undescended testes (19.2%). A total of 23,169 DALYs were averted through 1042 surgical procedures (22.2 DALYs per procedure) during the study period in Kenya, compared to 5497 DALYs through 373 procedures (14.7 DALYs per procedure) in Canada. CONCLUSIONS: Using recent developments in burden of disease measurement, the results point to the significant impact of pediatric surgical centers in addressing the global burden of congenital surgical disease. The study carries significant implications for resource allocation and training.

Timing of video-assisted thoracoscopic debridement for pediatric empyema.

BACKGROUND: Video-assisted thoracoscopic debridement (VATD) is a new method of managing pediatric empyema. The purpose of this retrospective study was to determine the relation between the timing of VATD and its success in avoiding the need for open decortication. STUDY DESIGN: Twenty-one children aged 3 to 16 years (mean, 8 years) with symptomatic, loculated, parapneumonic empyema were treated with VATD at two tertiary pediatric centers between 1994 and 1997. The preoperative duration of symptoms, hospitalization, and previous need for thoracostomy drainage were compared between patients having VATD only and those who subsequently required a thoracotomy and decortication. Statistical analysis used the Wald chi-square test or Fisher's exact test with p < 0.05 considered significant. RESULTS: Video-assisted thoracoscopic debridement was successful in 15 patients (group 1) and unsuccessful in six patients (group 2), who required a thoracotomy and decortication. Group 1 had a shorter mean duration of preoperative symptoms (13 versus 27 days; p=0.03), a shorter median duration of preoperative hospitalization (6 versus 18 days; p=0.04), and a lower incidence of previous thoracostomy drainage (4/15 versus 5/6; p=0.05). CONCLUSIONS: The technique of VATD is most likely to be successful when used within one week of diagnosis of a loculated parapneumonic empyema. A prospective trial comparing VATD with intrapleural fibrinolytic agents for the initial treatment of pediatric empyema is needed.

The uncut Collis-Nissen fundoplication: results for 79 consecutively treated high-risk children.

PURPOSE: The Nissen fundoplication fails to control gastroesophageal reflux (GER) in up to 25% of children with neurological impairment or chronic lung disease. The uncut Collis modification lengthens the intraabdominal esophagus, improving the antireflux function without opening the stomach. This study reviews the results of the uncut Collis-Nissen fundoplication in a pediatric series. METHODS: Seventy-nine children had an uncut Collis-Nissen fundoplication performed over a 5-year period. The median age was 1.4 years. Associated problems included neurological impairment (77%), chronic lung disease (38%), and esophageal atresia (3%). Surgery was undertaken only in children with objective documentation of pathological GER, who had GER complications unresponsive to medical treatment. The usual complications that led to surgery were pulmonary (73%), esophagitis (67%), or failure to thrive (35%). Liquid gastric emptying was assessed routinely preoperatively, and was delayed in 42% patients who then had concomitant pyloroplasty. RESULTS: GER was controlled in 97% of patients after a median follow-up of 1.8 years. All children with recurrent symptoms were restudied, and only two children had documented recurrent GER. One of these required a repeat fundoplication. Thirty-three percent were on promotility medication for feeding difficulties, gagging, or retching. There were postoperative complications in 26% (minor 23%, major 3%) and one postoperative mortality. Eleven late deaths were unrelated to surgery or GER. CONCLUSION: The uncut Collis-Nissen fundoplication provides excellent control of GER in children and is associated with acceptable morbidity and low mortality. It should be particularly considered in children with neurological impairment or chronic lung disease.

Pediatric laparoscopic splenectomy using the lateral approach.

Laparoscopic splenectomy in children has been shown to be safe, to reduce postoperative pain and hospital stay, and to accelerate return to full activities. We describe our experience with a four-port "lateral" approach in 18 patients. Patients were placed in the lateral decubitus position and the table was flexed to separate the left subcostal margin and iliac crest. The camera port was inserted at the umbilicus and additional ports were placed in the epigastrium and left lower quadrant. After mobilization of the splenic flexure a port was inserted in the left flank below the 12th rib for elevation of the spleen. A 30 degrees laparoscope was used and the splenic vessels were controlled with an endo-GIA and/or clips. The spleens were placed in a bag, morcellated, and extracted through a port site. Eight females and 10 males with a median age of 12.5 years (5-17 years) and weight of 55.5 kg (17-124 kg) underwent splenectomy of idiopathic thrombocytopenia purpora (10), spherocytosis (6), elliptocytosis (1), and Hodgkin's disease (1). The median operating time was 160 min (90-300 min) and median blood loss was 105 ml (5-350 ml). Accessory spleens were removed in four cases. Three patients required extensions of a port site to remove large spleens which could not be placed in a bag. The sole complication was a transient pancreatitis with associated pleural effusion. The median postoperative hospital stay was 2 days (1-11 days) and time to full activities was 8 days (3-25 days). The lateral approach affords excellent visualization of the splenic vessels, pancreas, and accessory spleens. This approach is safe and reliable and is our preferred approach for laparoscopic splenectomy in children.

Colon patch esophagoplasty for caustic esophageal stricture.

Severe caustic injury with stricture may require esophageal reconstruction. The available methods of esophageal substitution do not satisfactorily replace the peristaltic and antireflux properties of the native esophagus. The authors report the results of two children treated with colon patch esophagoplasty and preservation of the injured esophagus. Both were 2 years of age when they accidently swallowed lye. Long esophageal strictures developed, which were resistant to repeated dilatation. Eleven and 13 months after the injury, the patients underwent colon patch esophagoplasty. A segment of the left colon was tailored to the length and shape of the opened stricture and was anastomosed side-to-side to the esophagotomy. One child later had a short recurrent stricture and diverticulum, which required revision of the distal end of the anastomosis. Follow-up endoscopic examination has shown healing of the esophagus and resolution of the strictures; both children are eating normally 3 years after surgery, without symptoms of gastroesophageal reflux. Colon patch esophagoplasty is an alternative to esophageal replacement for long caustic esophageal strictures. Its advantage is preservation of the peristaltic and antireflux mechanisms of the conserved esophagus.

Morbidity in neurologically impaired children after percutaneous endoscopic versus Stamm gastrostomy.

Neurologically impaired children frequently require a feeding gastrostomy. Few reports are available comparing the incidence of postoperative complications and symptomatic gastroesophageal reflux after endoscopic versus operative Stamm gastrostomy in this group of children. We undertook a retrospective study of 63 consecutive neurologically impaired children requiring a feeding gastrostomy, with an average of 23 months of follow-up. No child had symptomatic gastroesophageal reflux. Thirty children had a percutaneous endoscopic gastrostomy and 33 had a Stamm gastrostomy, depending on the preference of the surgeon. The two groups were comparable in age range, cause of neurologic impairment, and indication for gastrostomy. Minor complications occurred in 30%. All three major complications occurred after Stamm gastrostomy, including two postoperative deaths. Symptomatic gastroesophageal reflux developed in 60%. The incidence of fundoplication after gastrostomy was 10% in the percutaneous endoscopic gastrostomy group and 39% after Stamm gastrostomy (p < .025). Morbidity was lower after percutaneous endoscopic gastrostomy than after Stamm gastrostomy in this group of neurologically impaired children. Fundoplication for symptomatic gastroesophageal reflux was infrequent after percutaneous endoscopic gastrostomy and significantly more common after Stamm gastrostomy. Percutaneous endoscopic gastrostomy is recommended as the initial procedure in neurologically impaired children without symptomatic gastroesophageal reflux who require a feeding gastrostomy.

Obstructive jaundice caused by neuroblastoma managed with temporary cholecystostomy tube.

Neuroblastoma presenting as obstructive jaundice is very rare. The authors present two cases of neuroblastoma, one primary and one recurrent, manifesting as a malignant obstruction of the extrahepatic biliary system. Various methods of biliary decompression were considered in these children including transhepatic or retrograde biliary stenting and internal cholecystoenteric bypass. An attempt at percutaneous transhepatic stent placement failed in one case. In each patient, a simple insertion of a cholecystostomy tube proved effective. Immediately postoperatively, both patients had rapid resolution in symptoms and a decrease in bilirubin levels. Transient mild cholangitis in both children was successfully treated with antibiotics. Chemotherapy reduced the tumor size in each case, and the cholecystostomy tubes were removed within 3 weeks, after cholangiography showed patency of the distal common bile ducts. Temporary cholecystostomy tube drainage and systemic chemotherapy proved to be a safe, simple, and effective method for managing obstructive jaundice caused by neuroblastoma in these two cases.

A pig model for advanced laparoscopic biliary procedures.

Advanced laparoscopic biliary procedures can be undertaken in a pig model, but the small size of the normal common bile duct makes learning difficult. We have developed a prepared pig model of common bile duct ligation on which to practice advanced laparoscopic biliary surgery. The pig's distal common bile duct was occluded using several different methods via a minilaparotomy. Laparoscopic biliary procedures were undertaken 6-21 days later. The common bile duct became dilated to between 2 and 3 cm in diameter in all cases, and this was well tolerated by the animals. All advanced laparoscopic biliary procedures were possible, including choledochoscopy, exploration of the bile duct, and cholecystojejunostomy. Short-term common bile duct ligation is well tolerated in pigs and can be used to create a model for practicing advanced laparoscopic biliary procedures.

Laparoscopic-guided gastropexy for intermittent gastric volvulus.

A 12-year-old girl with acute gastric volvulus associated with severe scoliosis and neurological impairment did well initially with nasogastric tube decompression. To prevent recurrence, anterior gastropexy was performed using percutaneous sutures through the abdominal wall and stomach with the gastroscope in place. Simultaneous laparoscopic guidance was used to ensure that the stomach was fixed in a nonrotated position. One year later, volvulus has not recurred. This method of gastropexy is indicated only in children with intermittent gastric volvulus who do not have an underlying abnormality such as diaphragmatic hernia.

Giant hydatid lung cysts in the Canadian northwest: outcome of conservative treatment in three children.

Hydatid lung disease due to Echinococcus granulosus in the Canadian northwest and Alaska is often asymptomatic and usually benign. We reviewed the course and outcome of three children with giant hydatid lung cyst seen over a 2-year period. All were North American Indian children aged 9 to 12 years who presented with cough, fever, and chest pain. One had a rash. There was a history of exposure to domestic dogs who had been fed moose entrails in each case. Chest x-rays showed solitary lung cysts with air-fluid levels, from 6 cm to 12 cm in diameter. Aspiration of each cyst demonstrated Echinococcus hooklets and protoscolices. Serology was unhelpful, being negative in two cases. Transient pneumonitis and pneumothorax were seen as complications of needle aspiration. Two cysts gradually resolved over the following 6 months. One child returned after 9 months with a lung abscess due to superimposed infection of the cyst remnant with Haemophilus influenzae, and eventually required lobectomy. The existence of an endemic benign variant of E granulosus in Canada is not widely known, and it is important to distinguish it from the more aggressive pastoral form of the disease seen in immigrants from sheep-rearing countries. The native Canadian disease usually resolves spontaneously, does not cause anaphylaxis, and does not implant daughter cysts if spilled. Surgical treatment should be avoided except for complications such as secondary bacterial infection.

Appendicitis presenting with dysuria in a 2-year-old: ultrasound-aided diagnosis--a case report.

A 2-year-old Fijian boy presented with a week's history of fever and dysuria. On ultrasound scan of the abdomen, he was found to have an appendicular mass. The role of ultrasound in the diagnosis is emphasized as well as the need for consideration of appendicitis in any young child with abdominal pain.

Hereditary site-specific colon cancer in a Canadian kindred.

In summary, we have presented a large Canadian kindred exhibiting hereditary large bowel cancer, without polyposis coli, transmitted in an autosomal dominant fashion (Hereditary Site-Specific Colon Cancer). This series serves to emphasize the heritable nature of this and other malignant conditions and the importance of so fundamental a concept as the taking of a complete family history in the identification and management of these conditions. Looking to the future, the reduction of morbidity and mortality from Hereditary Site-Specific Colon Cancer lies in the education of the family and genetic counselling, both commencing in the mid teens the education of physicians and surgeons in the very considerable risk of malignancy in this condition the surveillance of asymptomatic family members including such measures as stool testing for occult blood six monthly augmented by air contrast barium enema and/or colonoscopy at two yearly intervals, commencing at age 25 the creation of national and international registries the identification of reliable biomarkers. We are indeed fortunate to live in a age when technology holds promise for the identification of the oncogenes operative in this and other heritable malignancies. This is the subject of ongoing collaboration between us and our molecular biology colleagues at Memorial University in St. John's and exemplifies, I believe, the very best in the cooperative spirit which may exist between a community hospital and a larger teaching centre.

Hereditary site-specific colon cancer in a Canadian kindred.

A large kindred with colorectal cancer unaccompanied by polyposis coli and characterized by autosomal dominant inheritance has been identified in eastern Canada. Ten family members from three successive generations have presented 17 documented colorectal cancers. The clinical features of the kindred are characteristic of hereditary site-specific colon cancer (HSSCC) (Lynch syndrome I): absence of multiple polyposis, autosomal dominant inheritance, onset of colorectal cancer at an early age and a high incidence of synchronous and metachronous colorectal cancers. A unique feature of this family is the high incidence of sporadic adenomatous polyps in affected members and their relatives. Patients with HSSCC have been managed by means of segmental colectomy followed by annual colonoscopic surveillance. All five patients with localized (Dukes' stage A or B) cancer at initial diagnosis were alive and free of disease after 2 to 12 years of follow-up, although three had required further colonic resection for metachronous carcinomas. Five young family members without cancer have had sporadic adenomatous polyps removed and are being followed with annual colonoscopy. It is not known whether polypectomy will alter the subsequent incidence of colon cancer. Subtotal colectomy is recommended for patients with HSSCC because of the high incidence of multiple lesions. An aggressive screening protocol, including colonoscopy, is recommended for all adult first- and second-degree relatives of patients with HSSCC. Identification of a biomarker, which is currently being sought in this kindred, would help identify those at greatest risk of development of cancer and allow earlier intervention.