Early developmental assessment of children with major non-cardiac congenital anomalies predicts development at the age of 5 years.

AIM: the aim of this study was to evaluate cognitive and motor development in children with major congenital anomalies and the predictability of development at age 5 years. METHOD: a prospective, longitudinal follow-up study was undertaken. The Dutch version of the Bayley Scales of Infant Development - Mental Developmental Index (MDI) and Psychomotor Developmental Index (PDI) - were administered at the ages of 6, 12, and 24 months. The Revised Amsterdam Children's Intelligence Test - IQ and the Movement Assessment Battery for Children - Total impairment score (TIS) were used at age 5 years. A total of 117 children participated in the study. After excluding 12 children who had a major chromosomal or syndromal abnormality, the analysis was limited to 105 children (50 females, 55 males). Seven groups of congenital anomalies were distinguished: (1) small intestinal anomalies; (2) abdominal wall defects, comprising gastroschisis and omphalocele; (3) oesophageal atresia; (4) congenital diaphragmatic hernia; (5) Hirschsprung disease; (6) anorectal malformations; and (7) miscellaneous diagnoses. Logistic regression analyses served to determine the ability of MDI and PDI to predict IQ and TIS at age 5 years. RESULTS: at age five, 83.7% of 104 children had an IQ of 85 or above and 16.3% an IQ of less than 85. TIS was normal in 71.3% of 87 children, while 17.2% demonstrated a borderline score and 11.5% a definite motor problem. MDI and PDI scores showed equal sensitivity to predict IQ (p=0.004 at 6 and 12mo, p=0.001 at 24mo) and TIS (p<0.001 at 6 and 12mo, p=0.002 at 24mo). MDI and PDI were positively correlated with IQ and TIS; TIS was positively correlated with IQ. INTERPRETATION: IQ scores at 5 years of age corresponded to Dutch population scores, but TIS scores differed significantly. Early development of children with major congenital anomalies is predictive of development at 5 years, which can guide individualized follow-up for this vulnerable group of children.

Endorectal pull-through for Hirschsprung's disease-a multicenter, long-term comparison of results: transanal vs transabdominal approach.

PURPOSE: Previous studies have reported decreased continence in patients undergoing transanal endorectal pull-through (TERP) for Hirschsprung's disease compared to the older transabdominal approach (TAA). To address this, we examined long-term stooling outcomes in a large, multicenter cohort of patients undergoing either TERP or TAA. METHODS: Data were collected from 5 large pediatric institutions. Patient families were surveyed using a stooling score system (0-40, best to worst total score). Inclusion criteria included patients older than 3 years and those who had more than 6 months of recovery after pull-through. Those with total colonic aganglionosis were excluded. Statistical analysis included univariate and multivariate linear regression (significance, P < .05). RESULTS: Two hundred eighty-one patients underwent TERP (192) or TAA (89). Interviews were completed in 149 (104 [52%] TERP vs 45 [52%] TAA). The TAA group had a significantly greater number of daily bowel movements for each respective postoperative year and experienced more early complications (3% vs 1% with >1 complication; P = .061) and late complications (19% vs 4% with >1 complication; P < .001). Although the TAA group had a higher mean enterocolitis score (3.3 +/- 0.4 vs 1.8 +/- 0.2; P < .001), this was not borne out by multivariate regression analysis (P = .276). Parental survey showed that there were no significant differences between procedures in mean total, continence, or stooling pattern scores. CONCLUSION: Transanal endorectal pull-through was associated with fewer complications and fewer episodes of enterocolitis. In contrast to prior studies, TERP patients did not have a higher rate of incontinence. These results support use of TERP as an excellent surgical approach for children with Hirschsprung's disease.

Long-term functional sequelae of sacrococcygeal teratoma: a national study in The Netherlands.

BACKGROUND: Long-term functional sequelae after resection of sacrococcygeal teratoma (SCT) are relatively common. This study determines the incidence of these sequelae associated clinical variables and its impact on quality of life (QoL). PATIENTS AND METHODS: Patients with SCT treated from 1980 to 2003 at the pediatric surgical centers in The Netherlands aged more than 3 years received age-specific questionnaires, which assessed parameters reflecting bowel function (involuntary bowel movements, soiling, constipation), urinary incontinence, subjective aspect of the scar, and QoL. These parameters were correlated with clinical variables, which were extracted from the medical records. Risk factors were identified using univariate analysis. RESULTS: Of the 99 posted questionnaires, 79 (80%) were completed. The median age of the patients was 9.7 years (range, 3.2-22.6 years). There were 46% who reported impaired bowel function and/or urinary incontinence (9% involuntary bowel movements, 13% soiling, 17% constipation), and 31% urinary incontinence. In 40%, the scar was cosmetically unacceptable. Age at completion of the questionnaire, Altman classification, sex, and histopathology were not risk factors for any long-term sequelae. Size of the tumor (>500 cm3) was a significant risk factor for cosmetically unacceptable scar (odds ration [OR], 4.73; confidence limit [CL], 1.21-18.47; P = .026). Long-term sequelae were correlated with diminished QoL. CONCLUSION: A large proportion of the patients with SCT have problems with defecation, urinary incontinence, or a cosmetically unacceptable scar that affects QoL. Patients who are at higher risk for the development of long-term sequelae cannot be clearly assessed using clinical variables.