Esophageal Atresia and Tracheoesophageal Fistula

Gastroenterology 4 Responses »
May 152010

INTRODUCTION

Esophageal atresia (EA) is the most frequent congenital anomaly of the esophagus, affecting ?1/4,000 neonates. Of these, >90% have an associated tracheoesophageal fistula (TEF). In the most common form of EA, the upper esophagus ends in a blind pouch and the TEF is connected to the distal esophagus.

Fifty percent of infants are nonsyndromic without other anomalies while the rest have associated anomalies, most often associated with the VATER/VACTERL (vertebral, anorectal, [cardiac], tracheal, esophageal, renal, radial, [limb]) syndrome

PRESENTATION.

The neonate with EA typically has frothing and bubbling at the mouth and nose after birth as well as episodes of coughing, cyanosis, and respiratory distress.

Feeding exacerbates these symptoms, causes regurgitation, and may precipitate aspiration. Aspiration of gastric contents via a distal fistula causes more damaging pneumonitis than aspiration of pharyngeal secretions from the blind upper pouch.

The infant with an isolated TEF in the absence of EA (“H-type” fistula) may come to medical attention later in life with Continue reading »

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Treatment of Celiac Disease

Gastroenterology No Responses »
May 142010
A diagnosis of celiac disease mandates lifelong dietary exclusion of gluten from wheat and related products from barley and rye.
Strict adherence to such a diet is difficult and requires constant vigilance on the part of the patient. For this reason it is essential to confirm the diagnosis by means of an intestinal biopsy before starting an exclusionary diet.
In the short term, dietary modification will result in rapid remission of symptoms and correction of growth and nutritional deficiencies. Long-term benefits of adhering to the diet include preventing such complications as osteoporosis and reducing the risk for intestinal malignancies.
Repeating the serologic tests at intervals can help the physician and patient monitor whether gluten and related products are being adequately excluded from the diet.
In those who do not have selective IgA deficiency, the antiendomysium antibody is the best test for this purpose.
Persistent elevation of this antibody, or recurrence of a positive test that had previously been negative, is indicative that the patient is knowingly, or unknowingly, continuing to ingest gluten. Elevation of antiendomysium antibody levels and recurrence of significant small intestinal mucosal damage after initial recovery can precede symptoms by several years in those who do not strictly adhere to the diet.
Adherence to the diet can be particularly difficult in children, so that consultation with an experienced pediatric dietician is useful. Cookbooks and various gluten-free specialty items are available for patients with celiac disease.
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Clinical features of gasroesophegeal reflux disease

Gastroenterology No Responses »
May 042010

GERD is the most common esophageal disorder in children of all ages. Gastroesophageal reflux (GER) signifies the retrograde movement of gastric contents across the lower esophageal sphincter (LES) into the esophagus. Although occasional episodes of reflux are physiologic, exemplified by the regurgitation of normal infants, the phenomenon becomes pathologic (GERD) in children who have episodes that are more frequent or persistent, and thus produce esophagitis or esophageal symptoms, or in those who have respiratory sequelae.
CLINICAL MANIFESTATIONS.
Most of the common clinical manifestations of esophageal disease can signify the presence of GERD.
Infantile reflux manifests more often with regurgitation (especially postprandially), signs of esophagitis (irritability, arching, choking, gagging, feeding aversion), and resulting failure to thrive; symptoms resolve spontaneously in the majority by 12–24 mo.
Older children, in contrast, may have regurgitation during the preschool years; complaints of abdominal and chest pain supervene in later childhood and adolescence.
Occasional children present with neck contortions (arching, turning of head) designated Sandifer syndrome.
The respiratory presentations are also age dependent: GERD in infants may manifest as obstructive apnea or as stridor or lower airway disease in which reflux complicates primary airway disease such as laryngomalacia or bronchopulmonary dysplasia. Otitis media, sinusitis, lymphoid hyperplasia, hoarseness, vocal cord nodules, and laryngeal edema have all been associated with GERD. In contrast, airway manifestations in older children are more frequently related to asthma or to otolaryngologic disease such as laryngitis or sinusitis.
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