Alacrima (Congenital)

Complete absence of tears.

Autosomal dominant.

Deficient lacrimation and punctate corneal abrasions from infancy. Hypoplasia of lacrimal glands.

Examine the eyes for preexisting corneal lesions and obtain a history of corneal abrasions. The eyes should be well lubricated during anesthesia to prevent corneal abrasions.

Alacrima can refer to a disorder in association with achalasia, addisonism, hyperuricemia, and ectodermal dysplasia.

Achalasia-Alacrima Syndrome: Inherited disorder but in a small minority can be an acquired abnormality induced by the formation of antibodies that block ACTH receptors. Fluid and electrolyte disorders. In contrast to Addison disease, the renin-angiotensin-aldosterone axis functions normally. Regurgitation and aspiration occur upon induction.

Allgrove Syndrome: Triple A syndrome is a genetic autosomal recessive disease characterized by the triad of ACTH-resistant insufficiency, achalasia, and alacrima. It appears in the first decade of life with severe hypoglycemic episodes, which can cause death. Mixed pattern of upper and lower motor neuropathy, sensory impairment, autonomic neuropathy, and mental retardation. Regurgitation and aspiration occur upon induction.

Christ-Siemens-Touraine Syndrome: Genetic disorder characterized by the association of hypohidrosis, hypotrichosis, and defective dentition with peculiar facies. Consequences are related to thermoregulation and exocrine gland insufficiency.

Schinzel-Giedion Syndrome: Distinct dysmorphic syndrome of congenital hydronephrosis, skeletal dysplasia (open cranial sutures, steep short skull, wide occipital synchondrosis), and severe developmental retardation. Coarse facies characterized by midface retraction, bulbing forehead, facial hemangiomas, short nose with anteverted nostrils, protruding large tongue, and hypertelorism. Death usually occurs during infancy.