A 3-year-old girl was given a diagnosis of congenital receptive deafness and chronic mucocutaneous candidiasis resulting from repeated C. albicans infections. Her disease course started with progressive dysphagia that worsened in coincidence with mucocutaneous infections that occurred when she was 5 years old. Esophageal stenosis was shown by barium meal testing at that time, and she subsequently underwent a large number of pneumatic dilatations and eventually percutaneous gastrostomy at the hospital to which she was referred. At the time of admission to our institution, she was 8 years old and had many Candida lesions scattered on her skin and mucosae. Complete immunologic assessment showed normal humoral immunity and basically normal cellular immunity with minimal decrease of CD4/CD8 ratio. Skin reactivity to Candida antigen was absent, in vitro lymphocyte responses to Candida mitogen at 1% and 10% were negative, and responses to phytohemagluttinen, concanavalin A, pokeweed mitogens, and OKT3 mitogen were normal. Endoscopy and barium meal testing showed that she had a tight, irregular esophageal stricture with various sinuses or pseudodiverticula caused by persistent infection, previous dilatations, and possibly at least one perforation during one of the endoscopies performed elsewhere. The esophagitis was grade IV, and this led us to perform the last endoscopy through the gastrostomy. There were many Candida lesions, with reddish mucosa and easily bleeding surface. The stricture was deemed intractable (at this time it was impossible to perform endoscopic dilatation); therefore, esophageal replacement was proposed. It was performed after 3 weeks of energetic treatment with amphoterycin B (1 mg · kg −1 · 24 h −1 ) and fluconazol (2 mg · kg −1 · 48 h −1 ). Transhiatal, intramediastinal, transverse left colonic grafting based on a left colic artery pedicle was performed without complications, and the patient has been swallowing normally for the ensuing 3 years, although she always has mucocutaneous lesions.
The resected esophagus had transmural severe fibrosis and scar tissue with grayish mucosa and a lumen of less than 2 mm in diameter at the upper third. Below this level, the entire esophagus was narrowed, although to a lesser degree. Histologic studies showed diffuse lymphocyte transmural infiltration and fibrosis only.
Herpes virus infection is common, and many individuals have type I antibodies after experiencing unspecific pseudo-flu events of fever, myalgia and eventually vesicular lesions in the pharynx. Esophageal involvement may cause dysphagia or odynophagia. In otherwise healthy patients, herpetic esophagitis can be considered to be an acute, benign, self-limited illness evolving toward complete resolution of the symptoms after 10 to 12 days (9). Herpes esophagitis has been reported occasionally in previously healthy children with intact humoral and cellular immunity (10–15). Most cases in patients of this age evolve favorably and without sequelae with solely supportive treatment. Ulceration and hemorrhage are rare in this type of infection. Rattner et al. (16) reported in 1985 one case of herpetic esophagitis in an adult with chronic leukemia and extensive bleeding. The lesions were consistent with the diagnosis of reflux esophagitis and were managed accordingly with secretory inhibitors. However, the correct diagnosis of herpetic esophagitis was possible after finding intranuclear inclusions and multinucleated cells in the biopsy samples. Bleeding persisted despite the antireflux regimen, but acyclovir treatment led to dramatic results. Spontaneous perforation of the esophagus as a complication of an intrinsic esophageal infection can occur at relatively low pressures in the presence of esophageal disease (17). Strictures seem to be exceedingly rare after herpes esophagitis (18), and we believe that no previous case of documented herpetic esophagitis leading to stricture necessitating esophageal replacement has been reported to date in a child.
The definitive diagnosis of herpes esophagitis is based on biopsy findings consisting of the presence of epithelial cells with spherical eosinophilic intranuclear bodies surrounded by a pale zone in addition to enlarged, apparently multinucleated cells and some cytoplasmic changes (19). However, the presence of multinucleated cells does not always reflect the viral origin of the disease (20), and immunohistochemical and electromicroscopic investigation may be necessary (21–23).
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