Extraocular muscle responses to orbital cooling (ice test) for ocular myasthenia gravis diagnosis.

BACKGROUND: As a result of clinical and laboratory investigations of temperature correlates of myasthenia gravis, orbital cooling (ice test) has been developed as a reliable test for ocular myasthenia diagnosis through blepharoptosis response. The test has not been utilized in a prospective manner for myasthenia diagnosis through extraocular muscle responses. METHODS: Fifteen patients with acquired motility disorders were studied with the use of orbital cooling and other tests for myasthenia gravis. Orbital cooling was performed in a standard fashion for all patients. In 14 of 15 patients, the diagnosis of myasthenia was not established at the time the ice test was performed. Fifteen non-myasthenic patients with acquired motility disorders were also studied with use of the ice test. Temperatures during orbital cooling were measured in the superior cul-de-sac of one patient and between the lateral rectus muscle and globe in 3 patients. RESULTS: All patients subsequently proven to have myasthenia gravis by other tests and by response to myasthenia therapy had a positive (diagnostic of myasthenia) response to the ice test. No patient had a false-positive or a paradoxical response to the ice test. No control patient had a positive blepharoptosis or motility response to orbital cooling. Temperature measurements demonstrated significant cooling effects in the superotemporal cul-de-sac and beneath the lateral rectus muscles after orbital cooling for 5 minutes. CONCLUSIONS: Orbital cooling, within certain parameters, can be a useful clinical test for myasthenia diagnosis through motility response, as well as blepharoptosis response.

Posterior scleral choristoma in the organoid nevus syndrome (linear nevus sebaceus of Jadassohn).

PURPOSE: To highlight the association of posterior osseous and/or cartilaginous ocular choristomas with epibulbar choristomas and the nevus sebaceus of Jadassohn. DESIGN: Small case series. PARTICIPANTS: Four patients with the organoid nevus syndrome. METHODS: Clinical and histopathologic studies in four patients with epibulbar lesions and nevus sebaceus of Jadassohn. MAIN OUTCOME MEASURES: Ophthalmoscopic findings of peripapillary lesions. Computed tomographic and ultrasonographic characteristic of posterior scleral lesions. Ocular histopathologic findings in one globe from one of the study subjects. RESULTS: Three patients had the triad of posterior osseous/cartilaginous ocular choristomas, anterior epibulbar choristomas, and nevus sebaceus of Jadassohn and one patient had anterior epibulbar choristomas and posterior osseous/cartilaginous ocular choristomas. Ultrasonography and computed tomography were valuable in detecting scleral ossification or epibulbar cartilage or both. The ophthalmoscopic findings were similar to those of a choroidal osteoma. CONCLUSIONS: The presence of posterior osseous/cartilaginous ocular choristomas in a patient with epilepsy or epibulbar lesions or both suggests the diagnosis of nevus sebaceus of Jadassohn. Osseous/cartilaginous ocular choristomas should be suspected in patients with nevus sebaceus of Jadassohn and peripapillary hypopigmented fundus lesions.

Flexor tendon suture methods: a quantitative analysis of suture material within the repair site.

This study compared the cross-sectional area and volume occupied by suture material at the repair site in three common methods of flexor tendon repair. A total of 51 human cadaveric tendons were studied. Zone II flexor digitorum profundus tendon lacerations were created and then repaired using the techniques described by Kessler, Tajima, and Savage. Quantitative cross-sectional area and volumetric measurements of suture material within each repair site were determined using a digital image analysis system. The Tajima repair occupied 27% of the tendon area at the repair site, while the Savage and Kessler repairs occupied 18% and 2%, respectively.

Surgical results of secondary glaucomas in childhood.

OBJECTIVE: This study aimed to describe results of glaucoma surgeries performed at one institution over the past 20 years in children with aphakia, aniridia, anterior segment dysgenesis, and other secondary glaucomas. DESIGN: The study design was a retrospective review. PARTICIPANTS: Fifty-eight eyes of 40 patients were studied. INTERVENTION: Trabeculectomy with or without mitomycin C, Molteno implantation, goniotomy, sclerostomy, endolaser cyclophotocoagulation, and cyclocryotherapy were performed. MAIN OUTCOME MEASURES: Intraocular pressure (IOP) control, defined as complete success (IOP < or = 21 without medications) or qualified success (IOP < or = 25 without medications or IOP < or = 21 with medications) and postoperative visual acuity stability were assessed. RESULTS: One hundred thirty surgical procedures were performed on 58 eyes of 40 patients; follow-up averaged 7.3 years. Intraocular pressure control was achieved in 40 (70%) of 57 eyes after 1 or more procedures. Intraocular pressure control and stabilization of visual acuity and optic disc appearance were achieved in 28 (51%) of 55 eyes. Five eyes had significant postoperative complications. Trabeculectomy with mitomycin C controlled IOP on last visit in 8 of 13 eyes with aphakic glaucoma. CONCLUSIONS: Surgical intervention can control IOP and prevent visual loss in children with secondary glaucomas. In the authors' experience, a filtering procedure is the most effective treatment in aphakic glaucoma and anterior segment dysgenesis.

Capsular management and refractive error in pediatric intraocular lenses.

BACKGROUND: Guidelines for intraocular lens (IOL) implantation in children regarding patient selection, age limitations, operative techniques, including management of the posterior capsule, and refractive goals are not universally agreed on. METHODS: The authors placed posterior chamber IOLs in the capsular bag of 79 eyes in 57 children. Patient age ranged from 10 months to 17 years. Follow-up averaged 2 years. Patients were selected on the basis of age, cataract morphology, laterality, and lack of potential complicating factors. In general, postoperative refractions were intended to be mildly hyperopic with the magnitude dependent on patient age. RESULTS: Seventy-nine percent of patients able to report a postoperative visual acuity showed 20/40 or better visual acuity. Vision was limited by amblyopia in the remaining patients. There were no significant complications. The posterior capsule opacified on average 2 years after surgery regardless of patient age. CONCLUSIONS: Implantation of posterior chamber IOLs in carefully selected children appears to be effective and safe. Consideration should be given to primary posterior capsulectomy-anterior vitrectomy at the time of lens implant in children who are not expected to be candidates for yttrium aluminum garnet (YAG) capsulotomy within 18 months of surgery.

Surgical management of strabismus associated with chronic progressive external ophthalmoplegia.

PURPOSE: The purpose of the study is to describe the clinical characteristics and surgical management of strabismus associated with chronic progressive external ophthalmoplegia. METHODS: The authors present four patients with chronic progressive external ophthalmoplegia and strabismus requiring extraocular muscle surgery, with attention to presenting symptoms, patterns of misalignment, results of surgical and nonsurgical therapies, and associated ocular or systemic conditions or both. RESULTS: Three patients reported diplopia before surgery. One patient presented with an esotropia, one with an exotropia, and two with hypertropia. Three patients required only one strabismus surgery, and one patient required multiple surgeries. CONCLUSIONS: Chronic progressive external ophthalmoplegia may have clinical characteristics similar to those of myasthenia gravis or thyroid ophthalmopathy. Patients with chronic progressive external ophthalmoplegia and strabismus frequently have diplopia in primary position and may benefit from extraocular muscle surgery to improve alignment. In addition, because these patients typically have poor motor fusion, prisms often are useful adjuncts to surgery.

Surgical treatment of superior oblique palsy.

Reports of several large series of patients with superior oblique palsy (SOP) published in 1986 or before set forth important guidelines for both diagnosis and treatment of this condition. Newer information about the anatomy, physiology, and pathophysiology of the superior oblique has accrued over the past decade. This paper reviews our experience with diagnosis and treatment of SOP over the past 5 years in light of this new information. Charts of patients treated for SOP over 5 year (1990 to 1995) were reviewed for male or female sex, age, symptoms, refraction, vision, stereo acuity, head posture, facial asymmetry, intraoperative superior oblique traction test, diagnostic position prism and cover test, torsion, surgery performed, and results of treatment. The charts of 190 patients were reviewed. In 181, postoperative examinations were performed by us. The etiology of the SOP was congenital in 137 and acquired in 53. Twenty-nine acquired cases were due to trauma and 24 arose from other causes. Fifty-six patients had facial asymmetry, 51 of whom had congenital SOP. Ninety-five had a lax tendon, 83 (87%) of whom had congenital SOP. Sixty-six had a normal tendon, 29 (44%) of whom had acquired SOP. Seventy-seven percent of patients had Knapp class I, III, or IV palsy. An average of 1.26 surgeries was performed per patient. Inferior oblique weakening was performed in 177 (93%), while 68 vertical rectus recessions were done. Thirty-five patients had superior oblique tuck or resection, all on lax tendons, and 15 had Harada Ito procedures for torsion. Six patients had mild Brown syndrome postoperatively, none of which required a takedown. A cure, defined as relief of symptoms or elimination of strabismus and head tilt, was achieved in 166 of 181 (92%) of patients. Successful treatment of SOP can be accomplished in the majority of cases by selective surgery usually beginning with inferior oblique weakening plus additional vertical rectus and horizontal surgery as needed, with superior oblique strengthening used only for lax tendons or when torsion is the main problem.

The second annular pulley: a histologic examination.

The second annular pulley and underlying flexor tendons from 15 fresh frozen fingers were prepared, sectioned transversely and longitudinally and then stained with hematoxylin-eosin, Verhoeff-Van Gieson (Elastin), Masson's trichrome and for the presence of hyaluronic acid. Three discrete layers of the second annular pulley were identified (1) an outer layer, continuous with the membranous sheath, that was richly vascularized; (2) a middle layer, characterized by radially oriented collagen fibrils and significant elastin fibrils; (3) an inner gliding surface, characterized by longitudinally oriented collagen fibrils and modified fibroblasts secreting hyaluronic acid. The three layers of the pulley seem specialized and have developed based on the functional requirements of the pulley apparatus. The outer layer serves as a source of vascular supply and nutrition, the middle layer maintains structural integrity and is organized to efficiently resist flexor tendon bowstringing, and the innermost layer facilitates efficient excursion of the underlying flexor tendons both by the orientation of the collagen fibers and by the secretion of substances that are thought to minimize friction.

Familial remitting chorea, nystagmus, and cataracts.

We report on 2 brothers with chorea and monocular horizontal nystagmus beginning in early infancy, both of which remit during the first decade of life, and peripheral cataracts. While this condition shares manifestations with benign hereditary chorea and several other familial movement disorders, the slowly remitting course of the chorea combined with the visual abnormalities appears to be unique.

Adenoid cystic carcinoma of the lacrimal gland in a six-year-old girl.

A 6-year-old white female was found to have an adenoid cystic carcinoma originating in a lacrimal gland. Eighteen months following diagnosis, the tumor recurred. Conservative surgery has been the sole mode of therapy. To date, after four operations and quadrimenstral imaging surveillance, there is no sign of disease progression. Our purpose is to record the unusual occurrence of an adenoid cystic carcinoma of the lacrimal gland in a child. An interim report, 32 months after diagnosis, is presented.

A new classification of superior oblique palsy based on congenital variations in the tendon.

BACKGROUND: Superior oblique palsy is the most frequent isolated cranial nerve palsy seen in strabismus practice. It is traditionally diagnosed according to etiology as acquired, congenital, or idiopathic, but surgical treatment is based on deviation not etiology. Observations at surgery led to speculation that the superior oblique tendon is different in congenital compared with acquired superior oblique palsy and that this difference should be considered in surgical treatment. METHODS: The authors reviewed the charts of 82 patients (89 eyes) undergoing surgery on the superior oblique tendon for superior oblique palsy. In each case, the palsy had been diagnosed preoperatively as acquired, congenital, or idiopathic, and, at surgery, characteristics of the tendon anatomy were described. RESULTS: Thirty-eight superior oblique tendons (36 patients), diagnosed as congenital superior oblique palsy, included 33 abnormal tendons and 5 normal tendons. Twenty-four tendons (21 patients), diagnosed as traumatic superior oblique palsy, included 22 normal and 2 abnormal tendons. Twenty-seven tendons (25 patients), diagnosed as idiopathic, included 19 normal and 8 abnormal tendons. Abnormal tendons were divided into 4 categories: (1) redundant, (2) misdirected, (3) inserted in posterior Tenon's capsule, and (4) absent. CONCLUSIONS: The authors conclude that congenital superior oblique palsy is usually associated with a structural abnormality of the superior oblique tendon (87%). Whereas acquired superior oblique palsy usually has a normal tendon (92%). Superior oblique underaction in acquired superior oblique palsy results from a neural deficit. Potential variance in anatomy of the superior oblique tendon should be considered when undertaking surgery for superior oblique palsy.

X-linked recessive familial exudative vitreoretinopathy.

Familial exudative vitreoretinopathy is an inherited disorder characterized by retinal traction, peripheral vitreous opacities, and subretinal and intraretinal exudates. We observed a family in which four boys (the children of three sisters) were affected with this disorder and an X-linked recessive inheritance was apparent. The differential diagnosis includes retinopathy of prematurity, primary hyperplastic primary vitreous, Coats' disease, peripheral uveitis, retinoblastoma, and Norrie's disease, but this differentiation can usually be made on the basis of clinical findings alone. Knowledge of X-linked recessive transmission is important for correct diagnosis and for genetic counseling.

Surgical treatment of subluxated lenses in children.

BACKGROUND: Surgical removal of subluxated lenses had traditionally been discouraged because of concerns about poor surgical results and unacceptably high complication rates. METHODS: The authors reviewed the surgical results of 29 eyes in 15 consecutive patients who were operated on for subluxated lenses. RESULTS: Best-corrected visual acuity improved in all 29 eyes and was limited only by amblyopia. There were no significant complications in follow-up ranging from 5 months to 12 years. CONCLUSION: Modern surgical techniques using vitrectomy instruments allow the vitreous to be handled more effectively and have led to improved results and lower complication rates. Surgery for subluxated lenses can be done effectively and safely when indicated.

Orbital teratoma associated with trigonocephaly.

A rare case of congenital orbital teratoma that developed concurrently with the clinical findings of metopic sutural synostosis in an infant is presented. A cause-and-effect and/or embryologic relationship between these two cephalic malformations is possible, although coincidental occurrence is equally probable. The proximity of these congenital anomalies permitted complete resolution in a one-stage craniofacial procedure without alteration in the surgical technique.

Large recession of the horizontal recti for treatment of nystagmus.

Ten patients had large recession of four horizontal recti at one procedure for treatment of nystagmus. Six patients had congenital motor nystagmus, two had oculocutaneous albinism, and two had optic nerve hypoplasia. Anomalous head posture with null point was also present in five patients. The rectus muscles were placed at or behind the equator in all but one case. Three patients with both esotropia and nystagmus had the medial recti placed 1 mm behind the equator and the lateral recti or at 1 mm anterior to the equator. Visual acuity improved an average of 1 line at distance and/or near in 8 patients who cooperated for testing. Although nystagmus was not eliminated in any patient, its its amplitude decreased in eight of ten patients, and anomalous head posture improved in three of five patients. In all patients, near vision was better than distance vision both preoperatively and postoperatively. Ductions were diminished minimally after the large recession and there were no other complications from surgery.

A technique for arthroscopic anterior cruciate ligament repair.

Treatment options for anterior cruciate ligament (ACL) tears include rehabilitation therapy, derotation therapy, and surgical repair or reconstruction. This article presents an arthroscopic technique for reattaching the ACL that offers some advantages over other procedures. Advantages include avoiding exposure of the knee joint, improved cosmetic results, and reduced recuperation time.