Posters

Alacrimia revealing the triple syndrome A: about a family

Poster Details

First Author: M.Achargui MOROCCO

Co Author(s): H. Boutahar   C. Abdi   R. Azahrai   C. Siham   M. Asmae   R. Sekhsoukh     

Abstract Details

Purpose:

Allgrove syndrome or triple syndrome A is a rare inherited disease associating alacrimia, achalasia and Addison's disease, neurological signs are observed in a third of cases, we report the observation of a family whose 3 children had congenital alacrimia as part of the Allgrove syndrome.

Setting:

Chu Mohammed VI oujda

Methods:

We report 2 cases of a family with triple AAA sydrome, including two male brothers from a consanguineous marriage at the age of 10 and 4 years,consult for a congenital absence of tears, associated with dysphagia.

Results:

The anamnesis of the parents found the notion of alacrimia in a third brother who died after acute adrenal insufficiency. Ophthalmologic examination revealed superficial punctate keratitis with an altered Schirmer test in both brothers. The somatic examination found melanoderma in both children, with amyotrophy of the leg and hollow foot in the big child  The explorations performed showed Addison syndrome in children associated with achalasia                                   Artificial tear replacement therapy was instituted,oesophageal dilatation for achalasia and hormone replacement therapy for renal insufficiency.

Conclusions:

Congenital alacrymia is the earliest and most frequently reported sign, It may be asymptomatic or responsible for dry eye with superficial punctate keratitis Adrenocortical insufficiency is manifested during the first decade of life by severe hypoglycaemic attacks that can be lethal. Achalasia is classically frequently the initial sign that leads the patient to consult. The characteristic triad of Allgrove syndrome is frequently associated with other signs, particularly neurological signs. Before any alacrimia of the child or the young adult must evoke a syndrome of Allgrove. Careful investigation of adrenocortical insufficiency, achalasia and potentially life-threatening complications can be observed.

Financial Disclosure:

... is employed by a competing company