China
Africa
Explore chapters and articles related to this topic
Familial Dysautonomia
Published in David Robertson, Italo Biaggioni, Disorders of the Autonomic Nervous System, 2019
Alacrima or lack of overflow tears, which is very unusual in the normal infant after the first few months of life, is almost always present in the child with FD. Thus lack of overflow tears with emotional crying is one of the diagnostic criteria. History is usually sufficient to document this finding but semiquantitation of the deficiency can be obtained by the Schirmer filter paper test or by trying to provoke tearing emotionally or with an irritant.
Lacrimal Disorders in Children
Published in John C Watkinson, Raymond W Clarke, Christopher P Aldren, Doris-Eva Bamiou, Raymond W Clarke, Richard M Irving, Haytham Kubba, Shakeel R Saeed, Paediatrics, The Ear, Skull Base, 2018
Caroline J. MacEwen, Paul S. White
Congenital alacrima, or hyposecretion of tears, is rare. This may be due to absence of the lacrimal gland or to the lacrimal gland being ectopic. Alacrima may be associated with systemic conditions such as Allgrove syndrome (familial alacrima, achalasia of the cardia and glucocorticoid deï¬ciency), anhydrotic ectodermal dysplasia and Riley–Day syndrome (familial dysautonomia),
AAMR syndrome in a 22-month-old and literature review
Published in Ophthalmic Genetics, 2022
Mark A. Oet, Venkatesh Brahma, James McGrath, Jennifer A. Galvin
Alacrima is characterized by severely decreased or deficient tear production. It is caused by hypoplasia or aplasia of the lacrimal glands and can present unilaterally or bilaterally. Patients who present with alacrima can be completely asymptomatic or fully symptomatic. Usually, symptoms include photophobia, foreign body sensation, eye pain, chronic eye redness, and decreased vision. Alacrima is usually a congenital disorder—most infants older than 3 months old with alacrima are noted to have no tear production when crying—but it can also be acquired (1). Acquired causes of alacrima include damage of lacrimal gland tissue from Epstein—Barr virus infections, sequelae of graft-versus-host disease in patients who have undergone bone marrow transplants, burn injuries, and autoimmune destruction of gland tissues seen in Sjogren’s syndrome (1,2). Evaluation for the presence of keratopathy from dry eyes as well as objectively measuring tear production with a Schirmer’s test is helpful for diagnosis. Treatment for alacrima depends on the severity of the symptoms and ranges from treatment with lubricant tears and ointment to moisture chambers.
Congenital alacrima
Published in Orbit, 2022
Zhenyang Zhao, Richard C. Allen
Congenital alacrima, or historically ‘alacrimia,’ is an exceedingly rare condition characterized by decreased or absent tear production. It was first reported in 1848 by Thurnam,1 who described two cases in cousins exhibiting the inability to shed tears and sweat along with defects in skin, bone and teeth, currently recognized as hypohidrotic ectodermal dysplasia. As more cases were published, congenital alacrima was found to be associated with multiple genetic mutations, with various degrees of systemic involvement. The intention of this review is to provide a practical reference for oculoplastic surgeons focused on ocular manifestations, systemic characteristics, and genetic association of patients with congenital alacrima.
Lacrimal Gland Insufficiency in Aqueous Deficiency Dry Eye Disease: Recent Advances in Pathogenesis, Diagnosis, and Treatment
Published in Seminars in Ophthalmology, 2022
This can further be classified into congenital and acquired types (Figure 1). Congenital alacrima is a rare condition of absence of tear production from birth. There is abnormal or deficient development of the lacrimal gland or its ductal tissues. It is associated with a multitude of systemic disorders and is an important and rare cause of paediatric dry eye disease.13 Presentation can vary from congenital absence or hyposecretion of tears, developmental delay, to severe multi-systemic involvement.14–16