Alacrima Clinical Presentation

Updated: Jun 28, 2016
  • Author: Dan D DeAngelis, MD, FRCSC; Chief Editor: Hampton Roy, Sr, MD  more...
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Presentation

History

Patients with alacrima can have a wide spectrum of clinical presentations, from entirely asymptomatic and comfortable to presenting with debilitating symptoms of foreign body sensation, photophobia, ocular pain, and decreased visual acuity.

Parents may notice that there is a history of crying without tears since birth.

Information obtained from the history may be significant when alacrima is syndromic and associated with other findings.

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Physical

Physical findings of alacrima may be either ocular or systemic, as follows:

  • Eyelids - Chronic blepharoconjunctivitis
  • Tear film - Decreased/absent tear production
  • Conjunctiva - Hyperemia, thick mucoid discharge, keratinization
  • Cornea - Hypesthesia, interstitial keratitis, pannus, subepithelial opacities, infectious ulcers, corneal perforations
  • Extraocular muscles - Palsies of cranial nerves III, IV, and VI
  • Pupils - Anisocoria, tonic pupils
  • Optic nerve - Optic atrophy
  • Orbit - Absence of the orbital and/or palpebral lobe of the lacrimal gland
  • Systemic findings include the following:
    • Gastrointestinal - Xerostomia, achalasia, decreased salivation
    • Neurologic - Peripheral and autonomic neuropathy
    • Musculoskeletal - Osteoporosis, short stature
    • Metabolic - Adrenocortical insufficiency
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Causes

Etiologies can be separated into pathological mechanisms or syndromic associations.

  • Mechanism
    • Nuclear aplasia
    • Failure of central nervous system/peripheral nervous system (CNS/PNS) innervation
    • Lacrimal gland aplasia/hypoplasia
  • Syndromic
    • Isolated congenital alacrima
    • Riley-Day syndrome - Familial dysautonomia
    • Anhidrotic ectodermal dysplasia - Decreased sweating and salivation, heat intolerance, hypotrichosis
    • Sjögren syndrome - Xerostomia, salivary gland enlargement, collagen-vascular disease association
    • Allgrove (or triple-A) syndrome - Achalasia, alacrima, adrenocorticotropic hormone (ACTH) insensitivity, late neurologic manifestations
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