Oscar A. Cruz

Treatment of Periocular Capillary Hemangloma with Topical Clobetasol Propionate

BACKGROUND Hemangiomas pose a therapeutic challenge because they can threaten vision in infancy and early childhood. Intralesional injection of corticosteroid is widely regarded as the treatment of choice for hemangiomas which induce strabismus or significant refractive error, or occlude the visual axis. Ocular and systemic complications such as eyelid necrosis, central retinal artery occlusion, and adrenal suppression have been reported rarely after corticosteroid injection. METHODS Three infants were treated with clobetasol propionate (Temovate) cream for vision-threatening eyelid hemangiomas. RESULTS Treatment with this topical fluorinated corticosteroid produced a measurable reduction in the size of the hemangiomas, which permitted clearing of the visual axis. No regional side effects were noted. In addition, the patients did not demonstrate evidence of hypothalamic-pituitary-adrenal axis suppression. CONCLUSIONS This treatment modality appears to provide an additional alternative for managing superficial periocular hemangiomas which threaten vision.

BOTULINUM TOXIN: NOVEL TREATMENT FOR DRAMATIC URETHRAL DILATATION ASSOCIATED WITH DYSFUNCTIONAL VOIDING

A 7-year-old girl with previously diagnosed fetal alcohol syndrome presented with a 3-year history of lower urinary tract infections and daytime wetting. There was no history of febrile urinary tract infections, constipation or soiling. Renal ultrasound showed normal kidneys with mild bladder wall thickening. Voiding cystourethrography revealed no reflux but dramatic circumferential urethral dilatation and decompensation (part A of figure). Urodynamics demonstrated uninhibited contractions with detrusor-sphincter dyssynergia, which is typical of the pediatric uninhibited bladder. Therapy with behavioral modification, prophylactic antibiotics and anticholinergics was unsuccessful in controlling wetting andor urinary tract infections. Therefore, cystoscopy and periurethral injection of botulinum-A toxin were performed. Endoscopy revealed the urethra to be dilated circumferentially. The bladder neck was normal and there was tight coaptation of the external sphincter. This area was injected

Pseudotumor cerebri associated with cyclosporine use.

PURPOSE An 11-year-old boy had a one-month history of horizontal diplopia. Three years earlier, he had undergone allogeneic bone marrow transplantation complicated by graft versus host disease. METHODS The patient had esotropia and bilateral optic disk edema. A magnetic resonance imaging scan disclosed no intracranial lesion. The opening pressure of the lumbar puncture was 500 mm of water. Pseudotumor cerebri secondary to cyclosporine was diagnosed. RESULTS The patient improved with resolution of his esotropia and diplopia within five days of discontinuing his cyclosporine. The optic disk edema resolved within three months. CONCLUSION Cyclosporine must be added to the list of medications with a known association with pseudotumor cerebri.

Brief Report: Incidence of Ophthalmologic Disorders in Children with Autism.

Purpose To determine the incidence of ophthalmologic disorders in children with autism and related disorders. Design Retrospective chart review. Four hundred and seven children diagnosed with autism or a related disorder between 1998 and 2006. one hundred and fifty-four of these children completed a comprehensive ophthalmology exam by a pediatric ophthalmologist. Results Ophthalmologic pathology was found in 40% of patients with autism or a related disorder with 29% having significant refractive errors, 21% demonstrating strabismus, and 10% having amblyopia. Conclusions Children with autism or a related disorder will frequently have an ophthalmologic abnormality. Since cooperation with vision screening is understandably limited in these children, a comprehensive eye examination by a pediatric ophthalmologist is recommended for all such children.

Age and racial variation in central corneal thickness of preschool and school-aged children.

PURPOSE The purpose of this study was to determine variations in central corneal thickness (CCT) of preschool and school-aged African American and white children. Secondary aims were to assess possible correlations between CCT measurements and gender, axial length, intraocular pressure (IOP), family history of glaucoma, or history of prematurity. METHODS Contact ultrasound was used to measure CCT and axial length in 76 white and 60 African American children between the ages of 7 months and 18 years. A questionnaire was completed by the parents or guardians, including medical and family history. Statistically significant associations and differences were assessed using the independent t test, analysis of variance, and linear regression. All associations were defined as significant when the alpha value was less than 0.05 (two-tailed). RESULTS Mean CCT was thinner in African American children (535 +/- 35 microm) compared to white children (559 +/- 38 microm) (P < .001). The corneal thickness in children ages 10 to 18 years was significantly higher than in all other age groups in both African American (P = .03) and white (P < .005) children. No association was found between CCT and gender, axial length, IOP, or family history of glaucoma. Premature children had thinner CCT (536 +/- 40 pm) than full-term children (552 +/- 38 microm) (P = .009). CONCLUSIONS African American children have a thinner CCT compared to white children at all ages. Children of both racial groups have an increasing value of CCT with increasing age after approximately age 10 years. Children born prematurely have a thinner CCT than full-term children.

Bilateral inferior rectus muscle recession for correction of hypotropia in dysthyroid ophthalmopathy.

PURPOSE The restrictive myopathy of dysthyroid ophthalmopathy frequently results in strabismus and diplopia. The most common deviation is hypotropia. Ipsilateral inferior rectus muscle recession, the generally accepted treatment, may lead to a progressive marked overcorrection. The purpose of this study is to evaluate the results of bilateral asymmetric inferior rectus muscle operations with regard to late progressive overcorrection. METHODS A retrospective review of all patients undergoing bilateral inferior rectus muscle recession for dysthyroid ophthalmopathy between 1993 and 1997 found 8 patients with hypotropia resulting from dysthyroid ophthalmopathy. Alignment and motility were assessed preoperatively and postoperatively in all patients. Bilateral asymmetric inferior rectus muscle recession was performed on all patients with an adjustable suture performed on the hypotropic eye. A successful, long-term, postoperative result was defined as orthophoria or a vertical misalignment of less than or equal to 5 PD in primary gaze. RESULTS Data were collected from 8 patients at 4 to 6 weeks postoperatively. Six patients had successful alignment, and 2 patients remained undercorrected. Seven patients were successfully aligned, and 1 patient was undercorrected at the latest postoperative examination (mean, 18 months). CONCLUSION In our series, bilateral asymmetric inferior rectus muscle recession resulted in successful correction of hypotropia without late postoperative overcorrections in patients with dysthyroid ophthalmopathy.

Sensory Strabismus-Eso or Exo?

PURPOSE The type of horizontal strabismus from loss or impairment of vision is thought to depend on patient age at the time of vision loss. Association between the age at onset of vision loss and development of esotropia vs exotropia will be determined. METHODS Patients with a diagnosis of sensory strabismus and visual acuity of 20/40 or poorer were reviewed as well as patients with diagnoses consistent with the development of sensory strabismus. Parameters considered were age at onset of vision loss and type of strabismus. Patients were excluded if the age at onset was not clear. RESULTS Of 123 patients with sensory strabismus reviewed: 82 (67%) had unilateral vision loss; 41 (33%) had bilateral vision loss; 75 (61%) had congenital vision loss; 50 (67%) developed esotropia; 25 (33%) developed exotropia; 48 (39%) had acquired vision loss; 5 (10%) developed esotropia; and 43 (90%) developed exotropia. A significant difference was noted between age at onset and type of horizontal strabismus (X2= 37.44; P <.0001). CONCLUSION Of patients with congenital vision loss, 67% developed sensory esotropia and 33% developed sensory exotropia. Of those with acquired vision loss, 10% developed sensory esotropia and 90% developed sensory exotropia. Patients with congenital vision loss are significantly more likely to develop esotropia, P <.005, and those with acquired vision loss are significantly more likely to develop exotropia, P <.001.

Ocular neuromyotonia: differential diagnosis and treatment

Abstract Ocular neuromyotonia (ONM) is a rare but distinctive clinical entity characterized by involuntary episodic contraction of one or more muscles supplied by the ocular motor nerves. A retrospective review was conducted on all patients with ONM seen by the neuroophthalmology service in the past 20 years. Ten patients were identified with ONM; six affecting vertical muscles (superior oblique; inferior rectus; superior rectus) and four affecting lateral rectus muscles. Case 1 has been reported previously. Most episodes occurred every 10–40 min, lasted a few seconds to several minutes, and were repeated throughout the day. Only two patients had previously undergone cranial radiation. Two had thyroid eye disease. One patient presented with superior oblique myokymia and subsequently developed ONM. Membrane stabilizing medications were prescribed in 7 of the 10 patients with varied success. ONM episodes ceased after extraocular muscle surgery in one patient with thyroid eye disease.

Skin conductance responses in paediatric Harlequin syndrome

We report a novel and simple application of skin conductance response (SCR) testing for diagnosis of a new‐onset iatrogenic Harlequin syndrome in an infant. Isolated ipsilateral facial pallor, complicated by thermally induced systemic sympathetic vasodilatation, and preferential lateral decubitus positioning, mimics harlequin colour change. Correct diagnosis as Harlequin syndrome with facial sympathetic interruption was demonstrated by diminution of SCR.

Comparison of methods for determining the AC/A ratio in accommodative esotropia.

PURPOSE The two most commonly used clinical methods of determining a high AC/A ratio are: 1) the gradient method, and 2) the distance/near disparity method. Significant differences can be found not only between these two methods, but also in the different techniques for the gradient method alone. The purpose of our study is to compare the gradient method using +3.00 lenses for near and -3.00 lenses for distance, and the distance/near disparity method. METHODS Patients examined over a 2-year period (1995-1997) with a high AC/A ratio esotropia according to the distance/near disparity were grouped according to level of high AC/A ratio, then prospectively measured by the gradient method (using plus lenses for near and minus lenses for distance). All measurements were performed with full spectacle correction in place according to cycloplegic refraction, and with fixation on an accommodative target. RESULTS Forty-five patients were included. Using both plus and minus lenses, a high AC/A ratio by the gradient method was found in 16 (36%) patients, (2 [12%] with a grade 1; 7 [44%] with a grade 2; and 7 [44%] with a grade 3, by the distance/near disparity method), a normal ratio was found in 6 (13%), and no patient had a low ratio. Twenty-three patients fell into a different category of AC/A ratio using plus lenses for near compared with minus lenses for distance. CONCLUSIONS The distance/near disparity method appears to diagnose a high AC/A ratio much more frequently than the gradient method. There was some variability in the AC/A ratio with plus versus minus lenses when the gradient method was used. Further study using the gradient method in patients without a significant distance/near disparity is required.