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Transient Achalasia-Like Esophageal Motility Disorder After Candida Esophagitis in a Boy with Chronic Granulomatous Disease

Bode, C. P.; Schroten, H.; Koletzko, S.; Lübke, H.*; Wahnn, V.

Journal of Pediatric Gastroenterology & Nutrition: October 1996 - Volume 23 - Issue 3 - p 320-323
Case Report
Free

University Children's Hospital and *University Medical Hospital, Düsseldorf, Germany

Address correspondence and reprint requests to Prof. Dr. V. Wahn, Heinrich-Heine-Universität, Kinderklinik, Postfach 10 10 07, 40001 Düsseldorf, Germany.

Manuscript received December 28, 1993; revision received June 30, 1994; accepted June 26, 1995.

The term “chronic granulomatous disease” (CGD) describes a group of inherited disorders characterized by failure of granulocytes to generate antimicrobial oxygen metabolities. Although phagocytosis is unaffected, ingested microorganisms are not killed. Four genetically different molecular defects in the expression of the multicompoundenzyme NADPH-ase have been identified in the X-linked or autosomal-recessive forms of CGD (1).

Clinically the patients have recurrent bacterial and fungal infections primarily involving lymph nodes, liver, skin, and lungs. Chronic inflammation leads to formation of granuloma, probably associated with a cell-mediated immune response to persisting bacterial or fungal antigens (1). Granulomatous alterations have been reported to cause esophageal (2) and ureteral stenosis (3), adding further sources of morbidity and mortality to the clinical spectrum of manifestations as vital structures are obstructed.

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CASE REPORT

A 14-year-old boy with CGD (X-linked cytochrome b deficiency) was admitted in May 1992 with severe dysphagia. Candida esophagitis was confirmed by esophagoscopy and biopsy. He was treated with fluconazole (5 mg/kg) for 12 weeks with excellent clinical response, as the boy was able to swallow all types of food. Four months later, severe dysphagia recurred, requiring nasogastric tubefeeding. Esophagogram (barium swallow) showed a fixed stenosis reaching from the middle to the distal third of the esophagus. On endoscopy, the stenosis could not be passed with a pediatric endoscope (Olympus PQ 20). A computed tomography (CT) scan raised the suspicion for compression of the esophageal wall due to granuloma formation. Treatment was initiated with methylprednisolone (2mg/kg/day). After 2 weeks of treatment, the esophagogram still showed stenosis with rippled contrast, forcing us to extend therapy. In October 1992, esophagogram and esophagoscopy after 4 weeks of therapy showed resolution of the stenosis, absence of visible granuloma formation or signs of gastroesophageal reflux, but incoordinated motility, varying from a cork-screw-like pattern to marked dilatation (see Figs. 1 and 2). With a stationary eight-channel perfusion system, esophageal manometry revealed achalasia-like motility disorder with incoordinated, partly unpropagated and reduced esophageal peristalsis (in mm Hg), increased pressure of the lower esophageal sphincter (LES), and incomplete relaxation of the LES after swallowing (Fig. 3). Clinically, the patient tolerated liquid nutrition; solid food, however, continued to cause dysphagic symptoms. Barium-marked bread displayed significantly delayed passage for solid food of 150 s (normal, 30-90 s). Other gastrointestinal motility disorders were absent, and the family history with respect to achalasia was unremarkable. Several therapeutic attempts using cisaprid, carbachol, and nifedipine did not lead to clinical improvement. A second manometric examination in March 1993 confirmed a persisting disturbance of esophageal motility with partly repetitive simultaneous contractions of high amplitude in the mid and lower third of the esophagus and almost complete but further incoordinated relaxation of the LES (Fig. 4). After clinical improvement with normal swallowing, a third manometric examination in October 1993 confirmed completely restored esophageal motility.

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DISCUSSION

A presumably granuloma-induced esophageal stenosis in a boy with CGD was successfully treated with oral steroids. Several authors have reported similar positive effects of steroids on granuloma-related obstructions (4,5), whereas other therapeutic approaches including antibiotics (6), surgery (7), and balloon dilatation (2) did not yield satisfactory results.

However, a severe disturbance of esophageal motility remained, with uncoordinated and reduced esophageal peristalsis, increased LES pressure, incomplete relaxation of the LES, and significantly delayed passage. These motility abnormalities are the major findings in achalasia (8-10), in which they should be observed constantly. As our patient displayed more heterogeneous alterations, the term achalasia-like motility disorder is more appropriate.

As there is poor insight into the etiopathogenesis of achalasia or achalasia-like motility disorders, and full-thickness biopsies for histologic examination are not available in patients with CGD because of increased risk of infection, we can only speculate on the pathogenesis of these findings. To our knowledge, no case of such alteration in patients with CGD has been reported so far. Sutcliff and Chrispin (11) described changes in esophageal contractility in four of eight patients studied by barium swallow; however, these patients were asymptomatic. Esophageal infection with Trypanosoma cruzi in Chagas' disease is known to cause achalasia by ganglionary lesions of the myenteric plexus (12,13). Recently, Robertson (14) reported an association of persisting infection with varicella-zoster virus and achalasia (14). Because the symptoms in our patient developed after esophageal candida infection, it seems reasonable to assume that esophageal ganglionary structures have been reversibly damaged by this infection. However, we cannot exclude granulomatous alterations as a contributing or major cause of this motility disorder, even if there was no evidence for granuloma formation when performing esophagoscopy. Differential diagnosis includes gastroesophageal reflux and chronic idiopathic intestinal pseudoobstruction, but signs of reflux or other gastrointestinal motility disorders were absent.

In summary, this case illustrates that dysphagic symptoms in CGD may not always be related to stenosis due to granuloma formation but may also be based on achalasia-like motility disorders caused by neuronal dysfunction, most likely the result of esophageal infections.

FIG. 1.

FIG. 1.

FIG. 2.

FIG. 2.

FIG. 3.

FIG. 3.

FIG. 4.

FIG. 4.

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