Alacrima Clinical Presentation

  • Author: Dan D DeAngelis, MD, FRCS(C); Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Apr 26, 2012
 

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 may be either ocular or systemic.

  • 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
    • 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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Contributor Information and Disclosures
Author

Dan D DeAngelis, MD, FRCS(C)  Lecturer, Ophthalmic Plastic and Reconstructive Surgery, Assistant Professor, Department of Ophthalmology and Vision Sciences, University of Toronto Faculty of Medicine; Ophthalmologist, Department of Ophthalmology and Vision Sciences, Hospital for Sick Children

Dan D DeAngelis, MD, FRCS(C) is a member of the following medical societies: American Academy of Ophthalmology, American Society of Ophthalmic Plastic and Reconstructive Surgery, California Medical Association, Canadian Medical Association, Canadian Ophthalmological Society, Ontario Medical Association, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Coauthor(s)

Jeff Hurwitz, MD, FRCS(C)  Director of Ophthalmic Plastic Surgery, Ophthalmologist-in-Chief, Mount Sinai Hospital; Chairman, Professor, Department of Ophthalmology, University of Toronto, Canada

Disclosure: Nothing to disclose.

Specialty Editor Board

Jorge G Camara, MD  Professor of Ophthalmology, Department of Surgery and Director of Fellowship Training Program in Ophthalmic Plastic and Reconstructive Surgery for Countries Served by the Aloha Medical Mission, University of Hawaii John A Burns School of Medicine

Jorge G Camara, MD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, and American Society of Ophthalmic Plastic and Reconstructive Surgery

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Mark T Duffy, MD, PhD  Consulting Staff, Division of Oculoplastic, Orbito-facial, Lacrimal and Reconstructive Surgery, Green Bay Eye Clinic, BayCare Clinic; Medical Director, Advanced Cosmetic Solutions, A BayCare Clinic

Mark T Duffy, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Ophthalmic Plastic and Reconstructive Surgery, Sigma Xi, and Society for Neuroscience

Disclosure: Allergan - Botox Cosmetic Honoraria Speaking and teaching

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

References

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  17. Weber A, Wienker TF, Jung M, Easton D, Dean HJ, Heinrichs C, et al. Linkage of the gene for the triple A syndrome to chromosome 12q13 near the type II keratin gene cluster. Hum Mol Genet. Dec 1996;5(12):2061-6. [Medline].

  18. Yoshita T, Kobayashi A, Sugiyama K. Bilateral corneal perforation in an infant with congenital alacrima. J Pediatr Ophthalmol Strabismus. Jul-Aug 2006;43(4):236-8. [Medline].

 
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