Edentulous child with Allgrove syndrome: a rare case report

Abstract

Triple-A syndrome, also known as Allgrove syndrome, is a rare autosomal recessive disorder. The 3 features of this syndrome are achalasia, adrenal insufficiency, and alacrima. Achalasia could be the first manifestation of the triple-A syndrome; however, its etiology is unclear. Alacrima is generally asymptomatic but can be detected by obtaining patient history. Although adrenal insufficiency could have manifestations such as asthenia, it might be wrongly diagnosed as muscle fatigue. Vitamin D and calcium supplements are usually prescribed for the prevention of osteoporosis. Neurologic manifestations could be present in adults. In some individuals with this disorder, genetic examination indicates mutations in both alleles of the AAAS gene, which encodes a special 546-amino-acid protein designated ALADIN, and in chromosome 12q13. The genetic cause of the triple A syndrome in some patients who do not have an identified mutation is unknown. While very few such cases have been reported till date, one such case was presented to us as an edentulous child.

Keywords: Achalasia; Allgrove; Child; Syndrome.

Fig. 1. A 15-year-old boy with Allgrove syndrome.

Fig. 2. Oral examination showed severe caries.

Fig. 3. Radiographic examination after barium ingestion revealed very slow barium passage through the esophagus along with severe constriction in the distal esophagus.

Fig. 4. Chest radiography demonstrated a normal heart shadow with fairly uniform opacity based on the upper half of the right lung.

Fig. 5. (A) Patient with complete maxillary and mandibular dentures. (B) Complete maxillary and mandibular dentures.