Primary hereditary oxalosis retinopathy.

A female infant had progressive atypical pigmentary retinopathy with type 1 hereditary oxalosis. At the age of 3 months she had a flecked retina type of retinopathy and six months later she exhibited a unique type of atypical pigmentary retinopathy. This latter abnormality was characterized by a dense parafoveal hyperpigmented ring five disc diameters in size, and composed of a confluence of small rings of hyperpigmented retinal pigment epithelium surrounding whitish highly refractile calcium oxalate crystalline deposits.

Early surgery for infantile esotropia: results and influence of age upon results.

In 105 patients with infantile esotropia who initially underwent corrective surgery between 5 and 24 months of age successful alignment of the eyes (to within 10 prism diopters of orthophoria) was associated with a higher prevalence of fusion and stereopsis and a lower prevalence of dissociated vertical deviation (DVD). Fusion was most frequent in the patients whose eyes were successfully aligned by 9 months of age, occurring in 92%. Inferior oblique overaction and amblyopia were less frequent when the initial operation had been performed before 12 months of age. Latent nystagmus, DVD, inferior oblique overaction and amblyopia were interrelated in a complex fashion.

Results of large single medial rectus recession.

Fifty-three patients with esotropia of 14 to 20 prism diopters received a 6-mm recession of one medial rectus muscle. All patients were three to ten years of age, had normal, equal vision, no vertical muscle imbalance, normal neurological status, and were followed for a minimum of two years. Twenty patients had high AC/A ratios, 22 were partially accommodative, four were deteriorated esophorias, four were anisometropes, and three were second operations. The average correction measured 16 prism diopters, ranging from 13 to 22 prism diopters, and 37 patients were aligned to 4 prism diopters or less of strabismus, 10 were microtropes, and all 53 had less than 10 prism diopters deviation. No persistent diplopia or consecutive exotropia was encountered. Stereopsis of 200 seconds or better was found in 75% of patients postoperatively. Large single medial rectus recessions appear to be a safe, effective, and predictable means of restoring ocular alignment and binocular vision in a select group of patients.

Benign episodic bilateral juvenile internal ophthalmoplegia.

A case of benign episodic bilateral juvenile internal ophthalmoplegia in a nine-year-old female is presented. Two separate episodes of sudden onset of bilateral mydriasis and accommodative loss accompanied by headache occurred, without any other physical, neuroophthalmic, or third nerve involvement. Complete resolution occurred over a period of months in both episodes. The differential diagnosis of isolated internal ophthalmoplegia is reviewed, and the episodic unilateral mydriasis syndrome of Hallett and Cogan appears to be similar to the bilateral involvement in the case presented.

Congenital primary upper eyelid entropion.

A case of primary congenital upper eyelid entropion ( PCUEE ) is reviewed. The author proposes a congenital shortness of the levator muscle with anomalous insertion onto the tarsal plate as the etiology, and a new simplified surgical correction is described. The classification of PCUEE as a syndrome with multiple congenital anomalies is supported, and the need for a complete systemic evaluation is stressed. Congenital primary upper eyelid entropion is an exceedingly rare condition. In 1969, Hiles and Wilder reviewed the 13 known cases in the ophthalmic literature, and presented a new case. They suggested the possibility that congenital upper eyelid entropion was part of a syndrome involving multiple systemic anomalies. Surgical correction of the ectropion is usually necessitated by corneal erosion secondary to trichiasis, and a multifarious assortment of surgical procedures has been described. The following is a case report of primary congenital upper eyelid entropion which corroborates the categorization as a syndrome, suggests a pathophysiologic abnormality of the eyelid as the etiology, and describes a new, simplified technique of surgical repair.

Surgery of infantile esotropia: a critical evaluation of marginal myotomy of the medial recti as a primary and secondary procedure.

Seventeen cases of marginal myotomy were compared to 88 conventional procedures in treatment of infantile esotropia. Successful alignment (within 10 prism diopters of orthophoria) was less frequently attained in the marginal myotomy group than in the conventional group, both after one surgery (18% versus 40%, respectively) and finally (24% versus 65%, respectively). Motor and sensory findings were similar in each group, although fusion and stereopsis were less frequent with marginal myotomy. Even though nearly all patients receiving marginal myotomy were successfully aligned immediately following surgery, a gradual return of esotropia was noted in 12 of 17 patients. The significantly inferior ability of marginal myotomy to produce stable successful alignment in infantile esotropia should incontrovertibly lead to the abandonment of marginal myotomy in favor of the vastly superior recession and/or resection procedures. Twenty-one cases of marginal myotomies of the medial recti performed as secondary procedures were also analyzed. The group receiving myotomy with resection had much better results (4 of 5 successfully aligned, average exo-shift 27.6 P.D.) than the myotomy only group (2 of 17 successfully aligned, average exo-shift 6.6 P.D.). Three of 17 were actually made more esotropic by the myotomy procedure alone. It is concluded that isolated myotomy is an ineffective reoperative procedure, and that by combining myotomy with resection, a fair rate of successful alignment may be achieved.

Early local steroid therapy of infantile eyelid hemangiomas (local steroid therapy of lid hemangiomas).

Two cases of upper eyelid capillary hemangiomas were successfully treated by local injection of steroids. Both cases required two injections, performed between 11 and 20 weeks of age. No untoward side effects occurred. This treatment would appear to be valuable in preventing the development of ophthalmic sequelae commonly associated with benign observation.

Infantile non-African Burkitt's lymphoma presenting as bilateral fulminant exophthalmos.

A case of non-african Burkitt's lymphoma presenting an acute bilateral fulminant exophthalmos in an infantile male is reviewed. The rapid progression of proptosis, and the dramatic response to chemotherapy is documented photographically. A brief description on non-African Burkitt's lymphoma and a differential diagnosis of bilateral infantile fulminant exophthalmos is included.

Nutritional nystagmus in infants.

Two infants presented with acquired infantile nystagmus. On examination, they manifested peculiar facies, growth and developmental delay, failure to thrive, and a Wernicke's type of nystagmus. Suspicion of a B vitamin deficiency resulted in correct diagnosis of dietary deficiency and/or enzymatic defect. In both instances, dietary treatment with B vitamins resulted in resolution of the nystagmus and improved growth and development of both individuals. Physicians, especially pediatricians and ophthalmologists, should be alert to the possibility of a Wernicke's type nystagmus in such infants, since the diagnosis may be critical to accurate diagnosis and treatment.

Infantile convergence-evoked nystagmus.

A case of convergence-evoked nystagmus in a healthy 15-month-old boy is presented. This is clinically demonstrated to be induced by fusional convergence, and not related to accommodative, tonic, or proximal convergence. The lack of neurological or ophthalmic abnormalities supports the classification of a "congenital" type of this rare phenomenon. A differential diagnosis is also discussed.

Ewing's sarcoma of the scapula with metastases to the lung and eye.

A small, round cell tumor of the left scapula was found in a 3-month-old female Caucasian child. The histology was consistent with Ewing's sarcoma. Subsequently, a solitary pulmonary metastasis was excised. Thirty-one months after diagnosis, the child presented with leukokoria and a solitary pleural metastasis. The histology of both was identical to that of the original scapular tumor. G-banded karyotypes of the pulmonary metastasis and the ocular tumor revealed the presence of a balanced translocation, 46,XX, t(11;22)(q24;q12), which supported the original diagnosis of Ewing's sarcoma.