A two-center comparative study of gastric pull-up and jejunal interposition for long gap esophageal atresia.

Purpose: When restoration of the anatomical continuity in case of long gap esophageal atresia (LGEA) is not feasible, esophageal replacement surgery becomes mandatory. The aim of this paper is to critically compare the experience of two tertiary referral centers in The Netherlands performing either gastric pull-up (GPU) or jejunal interposition (JI).

Methods: Retrospective chart review of all the patients with LGEA who underwent GPU in the University Medical Center Groningen and JI in the University Medical Center Utrecht.

Course of life into adulthood of patients with biliary atresia: the achievement of developmental milestones in a nationwide cohort.

Purpose: To investigate the course of life of young adults diagnosed with biliary atresia (BA) in infancy by comparing patients who did and did not underwent transplantation with an age-matched Dutch reference group.

Methods: All patients from the Dutch BA registry, aged >18 years, were invited to complete the course of life questionnaire.

Results: Forty patients participated (response = 74%).

Biliary atresia in the Netherlands: outcome of patients diagnosed between 1987 and 2008.

Objective: To examine the outcome of biliary atresia (BA) and to identify prognostic factors using a national database.

Study Design: All children born between January 1987 and December 2008 who underwent the Kasai surgical procedure for BA were retrieved from the Netherlands Study Group on Biliary Atresia Registry database. Outcomes were measured in terms of clearance of jaundice (bilirubin <1.

Cervical cystic swelling in an adolescent: unusual association of a cervical mature teratoma with vertebral anomalies and a history of gastric duplication cyst.

A 14-year-old girl presented with a cervical cystic swelling in association with deformity of cervical vertebrae. As a child, she had been treated for gastric duplication. Pathologic examination of the resected cervical swelling revealed a mature teratoma.

Mortality of biliary atresia in children not undergoing liver transplantation in the Netherlands.

In order to further improve the outcome of BA, we characterized the mortality of BA patients who did not undergo OLT in the Netherlands, and compared our results with international data. For this purpose, we analyzed the causes of mortality of non-transplanted BA patients before the age of five yr, using the NeSBAR database. To evaluate trends in mortality, we compared the cohort 1987-1996 (n=99) with 1997-2008 (n=111).

Partial external biliary diversion in children with progressive familial intrahepatic cholestasis and Alagille disease.

Background: Partial external biliary diversion (PEBD) is a promising treatment for children with progressive familial intrahepatic cholestasis (PFIC) and Alagille disease. Little is known about long-term outcomes.

Patients And Methods: A retrospective chart review of all patients undergoing PEBD in the University Medical Centre of Groningen (UMCG).

Factors affecting quality of life of children and adolescents with anorectal malformations or Hirschsprung disease.

Objectives: First, to compare the quality of life (QL) and perceived self-competence of children and adolescents with anorectal malformations or Hirschsprung disease with that of reference groups. Second, to identify predictors of QL.

Patients And Methods: A total of 491 patients with anorectal malformations or Hirschsprung disease were sent a questionnaire, which assessed QL (mental, physical), disease-specific functioning (defecation-related), perceived self-competence (self-esteem, athletic competencies, school attitude), and demographic characteristics (sex, age).

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.

Neurenteric cyst--a case report of this rare disorder.

Neurenteric cysts are rare congenital lesions that are believed to be the result of the split notochord syndrome. We report the clinical case of a 5-year-old boy presenting with vague gastrointestinal symptoms and fatigue, who had undergone resection of a small intestine duplication cyst as a newborn. Computed tomography revealed a mediastinal neurenteric cyst with partial destruction of several thoracic vertebrae.

Explaining change in quality of life of children and adolescents with anorectal malformations or Hirschsprung disease.

Objectives: The purpose of this work was to examine changes in quality of life, disease-specific functioning, and psychosocial competencies of children and adolescents (8-16 years of age) with anorectal malformations or Hirschsprung disease and to identify predictors of change in quality of life by testing an explanatory model in which background variables explained changes in quality of life via changes in disease-specific functioning and psychosocial competencies.

Methods: Questionnaires were administered to 129 patients with anorectal malformations and 121 patients with Hirschsprung disease within a 3-year interval. Clinical and sociodemographic background variables were measured on the first occasion.

Factors associated with recurrence and metastasis in sacrococcygeal teratoma.

Background: Sacrococcygeal teratoma (SCT) is a relatively uncommon tumour, with a high risk of recurrence and metastasis. The factors associated with recurrence and metastatic disease were studied.

Methods: A retrospective review was conducted of 173 children with SCT treated between January 1970 and February 2003 at the paediatric surgical centres in the Netherlands.

Hirschsprung's disease: healthcare meets the needs.

Objective: The objective of this study was to examine the types of healthcare services used by children, adolescents, and adults with Hirschsprung Disease (HD) in relation to the severity of the initial defect (mild, severe), whether additional care was needed, the provision of information, transfer to adult care, and satisfaction with the care provided.

Methods: Three hundred twenty (71%) HD patients, ages 6 to 54, completed a questionnaire that assessed the use of healthcare services, the need for more healthcare, the provision of information, the transfer to adult care and satisfaction with the provided care.

Results: In 6 months, 45% of the children, 14% of the adolescents, and 15% of the adults consulted a medical specialist.

[Recurrent abdominal pain caused by left mesocolic hernia].

A 37-year-old man presented with symptoms of intestinal obstruction. Laparotomy revealed a congenital left mesocolic hernia. Partial removal ofthe hernial sac that largely enclosed the small intestine resulted in a speedy recovery.

[The treatment of oesophageal atresia in The Netherlands].

The first description of oesophageal atresia dates back to 1670. Oesophageal atresia used to be regarded as a lethal disease, but since the successes of Leven and Ladd in 1939 and Haight in 1941 surgical correction is possible. The Dutch history ofoesophageal atresia begins in 1946.

Development of an adenocarcinoma of the esophagus 22 years after primary repair of a congenital atresia.

Esophageal cancer development after previous atresia repair is extremely rare in young patients. We present the clinical course of a patient who developed an adenocarcinoma of the esophagus at the age of 22 years, after repair of a tracheoesophageal fistula with esophageal atresia in the neonatal period. She developed a stricture of the esophageal anastomosis requiring frequent dilatations.

Explaining change over time in quality of life of adult patients with anorectal malformations or Hirschsprung's disease.

Purpose: The aim of this study was to examine changes in the quality of life of adult patients with anorectal malformations or Hirschsprung's disease over a three-year interval and to identify demographic, clinical, and psychosocial variables that explain possible quality-of-life changes. Understanding the factors that affect changes in quality of life over time is particularly important to provide adequate care.

Methods: Questionnaires were administered to 261 patients (77 percent), with a three-year interval.

Anorectal malformations: does healthcare meet the needs?

Objectives: The first aim was to identify the types of healthcare services used by children, adolescents, and adults with anorectal malformation (ARM) in relation to the severity of their disease and to examine whether additional care was needed. The second aim was to evaluate specific areas in the healthcare system, including provided information, transfer from pediatric to adult care, and satisfaction with the provided care.

Methods: Three hundred eighty-six (61%) patients with ARM, ages 6 to 52, completed a questionnaire that assessed their use of healthcare services and the need for additional services.

[Icterus prolongatus is a reason for laboratory investigation, also in breastfed neonates].

Three infants, a boy aged 4 months and two girls aged 3 months and 6 weeks, respectively, had jaundice while they were breastfed. Until then, the jaundice had been interpreted as an innocent consequence of the breastfeeding. In the two eldest patients, however, biliary atresia was diagnosed.

Perforation of a congenital umbilical hernia in a patient with Hurler's syndrome.

Congenital umbilical hernias in Hurler's syndrome (mucopolysaccharidosis I) are generally treated conservatively, because complications such as incarceration are, rare, and risks involved in surgical correction are high. This case report describes the surgical management of a ruptured umbilical hernia in a 3-year-old child with Hurler's syndrome. Emergency repair of the hernia was performed with primary closure of the fascia; hernia recurrence 6 months later was treated laparoscopically using a PTFE mesh graft with no evidence of re-recurrence.

[A cavernous haemangioma of the colon as the cause of rectal bleeding in childhood].

A 13-year-old girl presented with rectal bleeding, abdominal pain and a palpable mass in the lower right abdomen. The history mentioned a chronic anaemia since she was 2 years old. Further examination did not give any indications for inflammation as etiological factor and showed a thickening of (apparently) the small intestine.

Critical factors affecting quality of life of adult patients with anorectal malformations or Hirschsprung's disease.

Objectives: The first objective was to compare the quality of life of adult patients with anorectal malformations (ARM) or Hirschsprung's disease (HD) and to compare both groups with healthy people. And the second objective was to examine the factors that affect the quality of life of patients with ARM or HD, using a theoretical model in which patients' background characteristics explain quality of life via mediating disease-specific functioning and psychosocial functioning.

Methods: Three hundred and forty-one patients completed a questionnaire, which assessed sociodemographic characteristics, disease-specific and psychosocial functioning, and quality of life.

Excellent long-term outcome for survivors of apple peel atresia.

Background: Apple peel atresia is the rarest type of small bowel atresia. Because of its rare occurrence and high mortality rate, little is known about the long-term outcome of these children.

Methods: The patient charts, operative reports, and office notes of 15 children with apple peel atresia from 6 pediatric surgical centers in the Netherlands were reviewed.

Quality of life in patients with anorectal malformation or Hirschsprung's disease: development of a disease-specific questionnaire.

Purpose: Hirschsprung's disease and anorectal malformation are congenital diseases of the digestive tract with sequelae into adulthood. The quality of life of patients with these diseases is largely unknown. The aim of the study was 1) to construct a self-report disease-specific instrument to assess the quality of life in these patients and 2) to evaluate its psychometric performance.