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Alimentary tract duplications in children.

This report reviews the experience with 78 alimentary tract duplications found in 64 patients over a 40 year period at the Children's Hospital of Pittsburgh. Ten patients had the duplication discovered at autopsy. Multiple duplications were found in 15% of patients. The symptoms and physical findings of a duplication varied with location, size and mucosal pattern. About one-fifth of the duplications contained ectopic mucosa, usually gastric. Two-thirds of the patients were diagnosed prior to one year of age. Vertebral anomalies, as a clue to the presence of the lesion, were present in 15% of the patients. The most common indications for surgery included a mediastinal or abdominal mass, intestinal obstruction, and gastrointestinal bleeding. The results of surgery were favorable, with a mortality of 20%. Surgical complications accounted for six deaths, while four children died of severe associated anomalies. Three others died without surgical treatment, but with symptoms from the duplication.

Digestive System Abnormalities↗

Computed tomography and ultrasonography of carcinoma in duplication cysts.

The CT and ultrasound findings of malignancy arising in walls of alimentary tract duplication cysts are previously unreported. We describe two cases where identification of both cyst and tumor nodule and separation of the mass from other visceral organs was achieved. We review the English literature reporting this unusual condition and suggest that CT and ultrasound are of great value in accurate preoperative diagnosis.

Adenocarcinoma↗

Agenesis of the gallbladder: difficulties in management.

Gallbladder agenesis is a rare congenital biliary anomaly that may be associated with other biliary and extrabiliary congenital anomalies. Awareness of this entity by clinicians and radiologists is essential because many of these patients present with biliary symptoms and have unnecessary operations. In the present article, the relative epidemiological, etiological (embryology and development), pathophysiological, diagnostic tools and pitfalls and management aspects of this rare anatomic anomaly are briefly discussed through review of the literature. Particular reference to the difficulty in preoperative diagnosis is highlighted. The importance of the possibility of preoperative diagnosis to avoid unnecessary surgery is stressed.

Digestive System Abnormalities↗

Inappropriate use of contrast studies prior to referral of neonatal gastrointestinal anomalies and bowel obstruction.

Ten cases of neonatal gastrointestinal anomalies and bowel obstruction are described, in which contrast studies were carried out prior to transfer. Nine of the 10 infants had bile-stained vomiting, indicating the need for immediate surgical referral. The contrast studies resulted in delayed referral, deferment of appropriate resuscitation, and complications associated with the study. In several cases the wrong type of study, or the wrong contrast medium was used. These 10 infants required urgent surgical referral, not a sophisticated radiological procedure in geographic and surgical isolation.

Barium Sulfate↗

Perforation of jejunal duplication lined with ectopic gastric mucosa.

Alimentary tract duplications are rare congenital anomalies that occasionally contain ectopic gastric mucosa that secretes acid peptic juice and can cause pain, ulceration, bleeding and rarely perforation. We report a case of a 2-year-old girl who developed perforation of a jejunal duplication lined with ectopic gastric mucosa who was managed surgically.

Abdominal Pain↗

Fetal surgery for congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) continues to account for significant mortality in neonates. Advances in postnatal therapies have reduced mortality rates in the less severely affected infants, though surviving infants continue to display significant comorbid conditions. The history of fetal treatment for CDH is a microcosm for the development of fetal therapy in general. This paper traces this history from early clinical observations and imaging advances to experimental animal studies and finally to safe human application and development of clinical trails. Encouraging recent results and improved outcomes lend credence to the idea than prenatal interventions may give clinicians and families more hope.

Digestive System Abnormalities↗

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. Clinical factors were extracted from the medical records. RESULTS: Patients with ARM or Hirschsprung's disease did not differ in their quality of life. Compared to healthy people, both patient groups reported more limitations in their "overall" physical quality of life, but only patients with ARM reported impaired quality of life on several specific domains (e.g., physical role-functioning, pain). The model was largely accepted. Most striking were the strong effects of the psychosocial functioning factors in contrast to weak effects of the disease-specific "constipation" and "fecal continence" factors. CONCLUSIONS: The quality of life of patients with ARM or Hirschsprung's disease was found to be comparable. Compared to healthy people, both patient groups encountered "overall" physical health problems, but only patients with ARM reported additional pain and limitations in role functioning due to physical problems. It appeared that psychosocial functioning had the most important effect on the quality of life of patients with ARM or Hirschsprung's disease, while fecal incontinence and constipation had almost no effect on their quality of life.

Adaptation, Psychological↗

Prenatal diagnosis of gastrointestinal anomalies with ultrasound. What have we learned?

The use of routine obstetric ultrasound has been shown to accurately diagnose fetal gastrointestinal anomalies, both during and after the midtrimester. These are among the most accurately diagnosed of all anomalies, comprising 5-7% of all fetal anomalies. From a review of the literature it is clear that the use of routine ultrasound allows: (1) the detection of multiple anomalies that are often present and affect outcome significantly, (2) preparation for delivery at a tertiary center where neonatal surgical experience will allow optimal outcome, and (3) decisions about mode and timing of delivery in cases where this is important, i.e., cases of omphalocele and gastroschisis.

Delivery, Obstetric↗

Total parenteral feeding in infants using peripheral veins.

Fifteen infants with life-threatening gastrointestinal anomalies received total parenteral feeding for periods ranging from 10 to 54 days; their ages ranged from one day to 5 months. The solutions were administered through peripheral veins. All patients gained weight during the period of observation and no cases of septicaemia were encountered.

Amino Acids↗