Carlo Buonomo

Association of Schatzki Ring With Eosinophilic Esophagitis in Children

Objective: To describe the clinicopathologic characteristics of children with Schatzki ring and to determine if Schatzki ring is associated with eosinophilic esophagitis. Methods: The authors report 18 adolescents with radiographically diagnosed Schatzki ring (SR). Their clinical and histologic characteristics were reviewed in a blinded fashion. Results: The mean age of the patients was 15.8 ± 0.8 years and mean duration of symptoms was 2.6 ± 0.4 years. By histologic criteria, two groups of patients were defined. Eight had clinical and histologic criteria of eosinophilic esophagitis (EE) and 10 of peptic esophagitis. There were no differences in the symptoms or radiographic findings in the two groups. The SR was not identified by endoscopy in any EE patient and was identified in 70% of peptic esophagitis patients. Grossly apparent mucosal features associated with EE were significantly more common in those with EE. Those with peptic esophagitis had a significantly higher acid exposure than did those with EE (12.6 ± 2.9 v 2.0 ± 1.1%; P < 0.01) by esophageal pH probe. Patients with peptic esophagitis responded to proton pump inhibitors and/or dilatation, whereas those with EE did not have good response and required specific therapy for EE. Conclusions: EE may play a role in the pathogenesis of some patients with SR.

THE RADIOLOGY OF NECROTIZING ENTEROCOLITIS

This article reviews the radiology of necrotizing entercolitis (NEC), with an emphasis on the sensitivity and specificity of the typical plain film findings. An approach to radiologic diagnosis is suggested. The more recently described entity of perforation without NEC also is described.

Endoscopic repair of laryngeal cleft type I and type II: When and why?

To evaluate the clinical features of children with type I and type II laryngeal cleft and the role of conservative monitoring versus endoscopic repair in their management.

Intraduodenal hematoma complicating intestinal biopsy: Case reports and review of the literature

We report two cases of postbiopsy duodenal hematoma and review 14 additional cases. Duodenal hematoma predominantly occurs in children and presents with abdominal pain, vomiting, and pancreatitis. Upper gastrointestinal series, abdominal ultrasound, and CT scan are useful in visualizing the hematoma. No comparative studies of the usefulness of these techniques are available, but a CT is indicated if perforation is suspected. The treatment is conservative if no perforation is detected, and resolution of symptoms generally occurs within 2 wk.

Diagnosis and management of MNGIE syndrome in children: case report and review of the literature.

Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) syndrome is a rare disorder that presents in childhood; however, marked delay in diagnosis is common. We report a case and review the literature describing the typical features that should alert pediatricians to the diagnosis. We also describe a novel management strategy for providing symptomatic relief.

The barium enema in constipation: comparison with rectal manometry and biopsy to exclude Hirschsprung's disease after the neonatal period.

Background. The diagnosis of Hirschsprungs disease is usually made in neonates but often considered in older infants and children with constipation: these children may be referred for barium enema. Since it is widely accepted that a normal barium enema does not exclude Hirschsprungs disease, some children, after a normal enema, undergo more invasive procedures such as rectal manometry or biopsy. Our study asked how frequently a diagnosis of Hirschsprungs disease was made by biopsy or manometry in children who had normal barium enema.¶Materials and methods. We reviewed the medical records and barium enemas of 54 patients older than 28 days with constipation or difficulty passing stool who had a barium enema followed by manometry and/or biopsy.¶Results. Forty-eight patients had normal enemas: 24 of those patients had biopsies, 16 had manometry, and 8 both manometry and biopsy. Only 1 had manometry suggestive of Hirschsprungs disease, confirmed by biopsy. Six patients had abnormal enemas. Five had biopsy and manometry compatible with Hirschsprungs disease; one had a normal biopsy and manometry study.¶Conclusion. The barium enema is a good initial screening test for Hirschsprungs disease in severely constipated children since it correlates well with manometry and biopsy. The enema is particularly useful in centers without easy access to pediatric gastroenterology services, and a normal enema in this setting allows the continuation of medical therapy with further evaluation only if there is a lack of response. An abnormal enema, however, requires referral to a facility equipped to perform confirmatory manometry or biopsy.

Lack of Utility of Abdominal X-rays in the Evaluation of Children With Constipation: Comparison of Different Scoring Methods

Background and Aim: Abdominal x-rays are used diagnostically in the evaluation of children with constipation. However, their clinical utility has not been established. The aim of the study was to assess the accuracy of different methods in identifying children with functional constipation (FC) or nonretentive fecal incontinence (NRFI). Patients and Methods: Retrospective review of abdominal x-rays in which colonic transit (CT), Barr, Leech, and fecal loading (FL) scores were blindly measured by blinded pediatric gastroenterologists and a radiologist. Children were classified a priori as FC or NRFI. Results: One hundred sixty patients (125 FC, 35 NRFI) were studied. There were significant differences (P < 0.05) when comparing those with FC and those with NRFI: CT: 51 ± 18 vs 40 ± 21 hours; Barr: 14 ± 5 vs 11 ± 4; Leech: 10 ± 2 vs 8 ± 2; FL: 2 ± 0.5 vs 1.7 ± 0.4. More than 20% of FC had normal Barr and Leech scores, whereas >50% of NRFI had abnormal scores. CT discriminated better between FC and NRFI. There was a significant correlation (P < 0.05) between CT and Barr (0.45), Leech (0.41) and FL scores (0.36), and between Barr and Leech scores (r = 0.94). There was good intraobserver correlation between Barr, Leech, and FL scores but poor interobserver reproducibility. Conclusions: Although significant differences in overall FC and NRFI scores exist, the discriminative value is low for all scores. There is poor interobserver reproducibility of the Barr, Leech, and FL scores. These findings confirm the limited value of the plain abdominal x-ray in the evaluation of children with constipation.

Benign pneumatosis in children.

Background. In pediatrics, pneumatosis intestinalis (PI) is usually due to necrotizing enterocolitis in premature newborns. Beyond infancy, PI is uncommon. “Benign pneumatosis” is PI in patients with few or no symptoms that resolves with conservative management.¶Objective. Our goal was to better characterize benign PI in children. Our investigation focused on identifying underlying risk factors, symptoms at time of diagnosis, management and outcome.¶Materials and methods. Available medical records and radiographs of children with pneumatosis intestinalis from 1990 to 1998 were reviewed for underlying conditions, symptoms at time of radiographs, management and outcome.¶Results. Thirty-seven children (mean age 4 years) were included. Thirty-two children had identifiable risk factors. Twenty -five children were immunocompromised by their underlying conditions or therapeutic regimen. Thirty-five children were managed conservatively with resolution of PI. Two patients, however, required surgery and one patient died.¶Conclusion. Benign pneumatosis does occur in children. The majority have underlying risk factors, most commonly related to immunosuppression. Clinical deterioration is the most useful indicator for surgical intervention. In most patients PI resolves with conservative management.

Allergic colitis: a mimic of Hirschsprung disease

Background. Allergy to cow milk protein is a common cause of gastrointestinal symptoms in infancy. Milk allergy is usually a clinical diagnosis, and thus there have been few reports of the radiographic findings. Objective. To describe the barium enema findings of allergic colitis and differentiate them from Hirschsprung disease. Materials and methods. Four infants (age range 7 days–5 weeks) with constipation underwent barium enema to exclude Hirschsprung disease. Radiographic findings were correlated with the pathologic specimens from suction rectal biopsy. Results. All enemas revealed irregular narrowing of the rectum and a transition zone. Rectal biopsies in each case demonstrated ganglion cells and evidence of an allergic colitis, with inflammatory infiltrates in the lamina propria. A diagnosis of milk allergy colitis was made and symptoms resolved after removal of milk from the diet. Conclusions. Milk allergy is common in infancy. The rectum is a primary target organ, with allergic colitis often diagnosed on clinical grounds alone. However, a child with allergic colitis may be referred to radiology for barium enema, especially if constipation is present. The radiologist should be aware of the unique imaging findings of allergic colitis, so as to avoid confusion with Hirschsprung disease and perhaps an unnecessary rectal biopsy.

Isolated Neonatal Swallowing Dysfunction: A Case Series and Review of the Literature

Our purpose was to describe the natural history of isolated neonatal swallowing dysfunction (INSD). Nine infants with INSD are described. Eight presented within two weeks of birth. Symptoms included choking and cyanotic spells with feeds, recurrent aspiration, apnea, stridor, and vomiting. Three had nonspecific neurological abnormalities and were diagnosed later in life with underlying disorders (myotonic dystrophy, CHARGE association, velocardiofacial syndrome). All required tube feeding. Six tolerated nasogastric feedings and received a gastrostomy tube. Three failed nasogastric feeds and required jejunal feedings. The gastrostomy tube was removed in 7/9 at a mean age of 37 ± 9 months. In conclusion, INSD has a good long-term prognosis. The presence of minor neurological abnormalities at presentation suggests another underlying disorder. Nasogastric feeding followed by a gastrostomy is recommended in those without gastroesophageal reflux. Jejunal feedings are necessary in some. While most improve over time, they may need nutritional support for 3 years or more.