Dacryocystorhinostomy Precipitating Keratoconjunctivitis Sicca in Aplasia of Lacrimal and Major Salivary Glands (ALSG)

Abstract

We report a 16-month-old girl referred for bilateral epiphora and sticky eyes since birth. Examination revealed a refluxible left lacrimal sac mucocele, agenesis of the left lower punctum, and agenesis of both puncta on the right side. Complete bony obstruction was noted on probing of the left nasolacrimal duct. At 4 years of age, she underwent left external dacryocystorhinostomy (DCR) with silicone intubation because of chronic dacryocystitis. Her epiphora and stickiness improved significantly in the first postoperative year, but she subsequently developed dryness of the left eye, dry mouth, and dental caries. CT and MRI scans revealed the absence of the lacrimal and salivary glands. The clinical signs and symptoms improved with plugging the left upper punctum and topical lubricants. Aplasia of the lacrimal and salivary glands may present with symptoms of congenital lacrimal obstruction, and failure to make an early diagnosis will result in inappropriate lacrimal surgery and dry eye.

Bilateral Sequential Dacryocystitis in a Patient With Graft-Versus-Host Disease

“…Should dacryocystitis occur, after resolution of infection with medical treatment, there are several alternatives to standard DCR. These include careful observation with early antibiotic treatment in case of re-infection, DCR surgery with secondary punctal plug placement, 15 prosthetic replacement of the ocular surface ecosystem lens use, or dacryocystectomy. A multidisciplinary team experienced in the diagnosis and treatment of GVHD is necessary for optimal outcomes.…”

Aplasia of the lacrimal and major salivary glands (ALSG). First case report in spanish population and review of the literature

“…Alteration of lacrimal glands it’s related with irritable eyes symptoms, infections and even epiphora if the duct or puncta is involved (1). Nevertheless, the patients with ASLG syndrome usually don’t present any symptoms because the tear secretion is reduced but at the same time, balanced by the diminished drainage caused by alteration of the duct and/or puncta (17). This fact can be confirmed by a Schirimer test (12).…”

“…Also it is suggested that in ASLG the symptoms are presented earlier (1). Serum test must be realized to reject a systemic syndrome (1,17,18).…”

“…A mutation of FGF10 has been proposed as the responsible of the syndrome (1,8-11), but its low availability difficult the genetic confirmation (1,13,17).…”

“…The management of the lacrimal alteration depends of the clinical findings and can include artificial tears and sometimes dacryocystectomy or occasional puncta (17,19). Although the tear drainage can be balanced by the alteration of duct, the secretion of the gland could be abnormal (17).…”