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At a glance

Complete absence of tears.

Incidence

Unknown.

Genetic inheritance

There are two forms, one with most likely autosomal dominant and the other with autosomal recessive inheritance and consanguineous parents. Both sexes are equally affected.

Clinical aspects

Absent or severely decreased lacrimation since birth can result in punctate corneal abrasions and corneal perforation. The parents may report that the child cries without tears. The patients may complain about a burning or foreign body sensation in both eyes and photophobia. Hypoplasia of lacrimal glands, absence of lacrimal acini, or the goblet cells has been documented in some patients. Corneal sensation is intact. This disorder refers to isolated lacrimal gland pathology with no other abnormal findings neither in the rest of the eye adnexa nor any other organ systems. This is in contrast to systemic disorders with alacrimia being one of the symptoms (see “Other conditions to be considered”). Ophthalmological examination can reveal considerable mucoid debris, a tiny tear meniscus, and an incomplete and broken tear film with blotchy and diffuse punctate staining of the cornea with fluorescein. The Schirmer test was pathologic for basal and reflex tear volume assessment with no or only minimal wetting of the filter paper and tear production could not or barely be elicited with neither edrophonium nor neostigmine.

Anesthetic considerations

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

Other conditions to be considered

  • Achalasia-Alacrima Syndrome (Allgrove Syndrome; Addisonian-Achalasia Syndrome; Double-A Syndrome; Alacrima-Achalasia-Adrenal Insufficiency; Triple-A Syndrome; AAA Syndrome; Hypoadrenalism with Achalasia; Alacrima-Achalasia-Addisonianism Syndrome; Adrenocorticotropic hormone [ACTH]-resistant Adrenal Insufficiency Syndrome; Achalasia and Alacrima; Adrenocorticotropic Insensitivity Syndrome): This ACTH insensitivity syndrome is an inherited disorder, but in a small minority it is an acquired abnormality induced by the formation of antibodies that block ACTH receptors. In contrast to ☞Addison Syndrome, the renin-angiotensin-aldosterone axis functions normally.

  • Allgrove Syndrome (Achalasia-Addisonianism-Alacrima Syndrome [AAA]; Triple A Syndrome; Hypoadrenalism with Achalasia): Autosomal recessive inherited disease characterized by the triad of ACTH-resistant adrenal insufficiency, achalasia, and alacrima. It presents in the first decade of life with severe hypoglycemic episodes, which can potentially result in death. A mixed pattern of upper and lower motor neuropathy, sensory impairment, autonomic neuropathy, and mental retardation is common.

  • Christ-Siemens-Touraine Syndrome (Anhidrotic Ectodermal Dysplasia; Hypohidrotic Ectodermal Dysplasia): Heterogeneous genetic disorder caused by abnormal development of the ectodermal tissue and characterized by the association of hypohidrosis, hypotrichosis, defective dentition, peculiar facies, abnormal thermoregulation, and exocrine glandular insufficiency.

  • Hereditary Sensory and Autonomic Neuropathy III (HSAN III); Familial Dysautonomia; Riley-Day Syndrome; Hereditary Sensory Neuropathy Type III (HSN III): Inherited neurodegenerative disorder characterized by the absence of overflow tears with emotional crying in infants, hypotonia, feeding difficulties, inappropriate ...

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