Anthony R. Caputo

Duane's retraction syndrome☆

Duanes retraction syndrome (DRS) has been a recognized clinical entity for nearly a century. It is a clinically well described ocular disorder consisting of retraction of the globe with narrowing of the lid fissure in attempted adduction, frequent abduction deficiency with variable limitation to adduction, and upshoot and/or downshoot of the affected eye on adduction. Among strabismus patients the incidence of DRS is probably not more than 5%. Most cases are sporadic, but familial cases have been estimated at 10% by most authors. Numerous theories concerning the etiology and pathogenesis of DRS have been proposed, including agenesis of the abducens nucleus, but the majority of investigators concur that the characteristic findings are best explained by a paradoxical innervation of the lateral rectus muscle, which subsequently causes a cocontraction of the horizontal rectus muscles. The frequent association of DRS with other congenital anomalies suggests a teratogenic event occurring between the fourth to eighth week of gestation as an etiological factor. In this review historical aspects and theories of the syndrome are studied and statistical data are compiled and analyzed. Clinical features, differential diagnoses and variants of the syndrome are examined. Testing and treatment objectives are discussed.

Down Syndrome Clinical Review of Ocular Features

A total of 187 medical records of Down syndrome individuals over a 10-year period were reviewed retrospectively for strabismus, myopia, hyperopia, astigmatism, nystagmus, cataract, glaucoma, and other significant eye findings. This study showed that a higher proportion of these individuals than reported in previous studies had strabismus (57%). Refractive errors of myopia (22.5%), hyperopia (20.9%) and astigmatism (22%) were common. The primary care physician needs to be aware of the specific eye problems of Down syndrome individuals so that he or she may initiate or refer the patient for appropriate ophthalmologic care, because most of the eye findings in Down syndrome are treatable. Significant visual loss, a usually avoidable event in Down syndrome, should occur rarely.

Keratoconjunctivitis Sicca in Male Patients Infected with Human Immunodeficiency Virus Type 1

Keratoconjunctivitis sicca (KCS) has not been reported as occurring as a single entity in the acquired immune deficiency syndrome (AIDS) population. In a survey of human immunodeficiency virus type 1 (HIV-1) infected male patients, the authors found that 21% (9/42) had signs and symptoms compatible with KCS with positive Schirmer test results. Tear osmolarity determinations were obtained from this group and from an age- and sex-matched group of HIV-infected patients without symptoms of KCS and with negative Schirmer test results. Eighty-nine percent of the suspect group had increased tear osmolarity, whereas none of the control patients had a hyperosmolar tear film (P less than 0.0001). Results strongly suggest that KCS occurs at a significantly greater rate in male individuals infected with HIV-1 than in the general population.

Nystagmus in Down Syndrome

The incidence and characteristics of nystagmus in Downs syndrome are unclear. In 188 consecutive patients, 56 had nystagmus. Most had no clinically recognizable ocular pathology to account for the nystagmus. Twenty-nine had fine rapid horizontal nystagmus, 14 had a dissociated nystagmus which appeared pendular, whereas 9 had a form of latent or manifest latent nystagmus. Of the total patients with nystagmus, 41 had esotropia. Our findings suggest that fine rapid horizontal nystagmus, sometimes dissociated, occurs frequently in patients with Downs syndrome.

Treatment of Congenital Ptosis by Maximal Levator Resection

The treatment of severe congenital ptosis may include frontalis suspension or maximal levator resection. A previous study of 145 consecutive frontalis suspensions for congenital ptosis showed a fairly high recurrence rate, frequent granuloma formation, and significant brow scars. In 28 patients undergoing 32 maximal levator resections utilizing our technique, 28 patients had excellent results. Two required reoperations and two additional patients had undercorrections that did not warrant reoperation. There were no significant complications. We believe that maximal levator resection is a better surgical alternative than frontalis suspension in the treatment of severe congenital ptosis whether unilateral or bilateral. In our experience, maximal levator resection provides a better cosmetic result and the recurrence rate is probably less than with frontalis suspension.

Management of the Anterior and Posterior Lens Capsules and Vitreous in Pediatric Cataract Surgery

PURPOSE To review and discuss the advantages and disadvantages of various methods of managing anterior and posterior lens capsules and anterior vitreous in pediatric cataract surgery. METHODS We reviewed the literature related to pediatric cataract surgery on PubMed and subclassified the subject into subtopics for managing the anterior lens capsule, posterior lens capsule, and anterior vitreous. RESULTS After a review of the literature, we summarized the advantages and disadvantages of various approaches related to surgical managements of the anterior lens capsule, posterior lens capsule, and anterior vitreous in pediatric cataract surgery. We discussed recommendations from the literature and commented on our experiences related to the above subtopics. CONCLUSION In the surgical management of pediatric cataract, anterior continuous curvilinear capsulorhexis provides the most reliable and tear-resistant capsular opening. Vitrectorhexis and radiofrequency diathermy, on the other hand, are alternative approaches (depending on the surgeons personal preference). Primary posterior continuous curvilinear capsulorhexis may delay the onset of posterior capsule opacification. Anterior vitrectomy may be necessary to prevent or eliminate the onset of posterior capsule opacification in young children.

Epibulbar Choristomas Containing Lacrimal Tissue: Clinical Distinction from Dermoids and Histologic Evidence of an Origin from the Palpebral Love

Three new cases of epibulbar choristomas, all containing lacrimal tissue, are presented and compared with those reported previously in the literature. The first and second cases were unilateral, complex choristomas, consisting of ectopic lacrimal tissue, smooth muscle, and cartilage; the first case was also remarkable for the presence of lesional tissue in the peripheral cornea and evidence of progressive growth during puberty. The third case was a bilateral, complex choristoma, manifesting epibulbar lacrimal tissue, cartilage, and smooth muscle; also associated were bilateral optic nervehead colobomas and a uniocular focus of posterior scleral cartilage demonstrated by computed tomography (CT) scanning. On clinical examination, all three lesions displayed highly vascularized thickenings of the conjunctiva with diagnostically useful gelatinous elevations, which corresponded microscopically to lobules of lacrimal tissue. In addition, all three cases had the interesting diagnostic feature of superficial corneal scarring or sclerosis, with a fine vascularity adjacent to the conjunctival lesional tissue. Electron microscopy of the third case demonstrated completely normal cytoarchitecture of the lacrimal tissue. Because of the frequent presence of Müllers smooth muscle in epibulbar lacrimal choristomas, the authors propose that these lesions represent embryologic ectopias or overly extensive field effects of the palpebral lobe of the lacrimal gland.

Systemic response to mydriatic eyedrops in neonates: mydriatics in neonates.

During routine dilation of 48 newborns, systemic responses and pupil dilation were monitored. Both 10 percent aqueous and viscous phenylephrine caused blanching around the eyes and produced considerable rise in blood pressure. Dilatation average 4.7 mm. In a double blind study, a 2.5 percent solution caused no skin blanching and no change in pressure or heart rate. Average dilation was 4.5 mm. No blood pressure changes were observed with either one percent cyclopentolate or one percent tropicamide. Average dilatations were 5.0 mm and 5.3 mm respectively. The above agents, used individually for a total dosage of three drops in each eye did not provide adequate dilation for a thorough funduscopic examination. Our protocol at United Hospitals Medical Center is a safe combination of drugs and provides excellent dilatation averaging greater th an 7 mm. No skin blanching or change in heart rate was observed.

Diplopia and Strabismus Following Ocular Surgeries

Postoperative diplopia and strabismus may result from a variety of ocular surgical procedures. Common underlying mechanisms include sensory disturbance, scarring, direct extraocular muscle injury, myotoxicity from injections of local anesthesia or antibiotics, and malpositioning of extraocular muscles by implant materials. The most common patterns are vertical and horizontal motility disturbance. Treatment options include prisms, botulinum, occlusion, or surgery.

Congenital Unilateral Adduction Deficit with Simultaneous Abduction: A Variant of Duane's Retraction Syndrome

Congenital adduction deficit not associated with other signs of oculomotor nerve weakness occurs infrequently. Type 2 Duanes retraction syndrome accounts for some of these cases. The authors identified three children with unilateral adduction deficits and simultaneous abduction of the eyes on attempted lateral gaze into the field of action of the apparently paretic medial rectus muscle. This is the first report of a series of patients with this condition previously termed simultaneous or synergistic divergence. All patients had a large exotropia, and two had a horizontal face turn away from the involved eye. A large (14 mm) lateral rectus muscle recession done on one of these children reduced the face turn but did not eliminate the simultaneous abduction. Clinical, intraoperative, and electromyographic data suggest that this condition is an unusual variant of type 2 Duanes syndrome.