Charles D. Rice

ORBITAL INTRAMUSCULAR SCHWANNOMA

In an 8-year-old girl with asymptomatic proptosis, computed tomographic scans showed a large medial orbital mass that contoured the globe anteriorly, bowed the optic nerve laterally, and extended posteriorly to the orbital apex. T1-weighted coronal magnetic resonance images showed the mass to be a diffusely enlarged medial rectus muscle. Histopathologic examination of a medial rectus muscle biopsy specimen disclosed a multinodular, intramuscular schwannoma, separating and infiltrating normal skeletal muscle fibers. The intramuscular location and multinodular configuration of this tumor, together with its occurrence in a child, distinguish it from previous orbital schwannomas.

Subperiosteal Orbital Hematoma: Visual Recovery Following Delayed Drainage

Subperiosteal orbital hematomas are rare sequelae of blunt periorbital trauma. Visual loss in such cases is very infrequent, but reportedly irreversible, unless treated with early decompression. A 17-year-old boy developed marked visual loss secondary to an acutely developing traumatic subperiosteal orbital hematoma. Despite delayed referral surgical evacuation of the hematoma remarkably restored his vision. Postoperative clinical examination, fluorescein angiography, and electrophysiologic testing confirmed that a compressive optic neuropathy had caused the visual dysfunction. Although immediate surgical decompression remains the treatment of choice for subperiosteal hematomas producing visual loss, this case demonstrates that the optic nerve may recover dramatically even after a prolonged period of dysfunction from compression.

Ocular and Systemic Findings in the Aarskog (Facial-digital-genital) Syndrome

The Aarskog (facial-digital-genital) syndrome is an X-linked disorder in which short stature is accompanied by hypertelorism, digital anomalies, and shawl scrotum. Except for hypertelorism and blepharoptosis, ophthalmic abnormalities have been rarely noted in this condition. We examined four patients who had Aarskog syndrome and unilaterally or bilaterally decreased vision on initial examination. Three family members had V-pattern esotropia, latent nystagmus, inferior oblique overaction, and amblyopia. A fourth patient had bilateral blepharoptosis and severe astigmatism. Other ocular features included hyperopia, anisometropia, deficient ocular elevation, blue sclerae, and posterior embryotoxon. These findings underscore the need for ophthalmic examination in asymptomatic patients with Aarskog syndrome to rule out treatable causes of visual loss.

Absence of Chlamydia in Trachomatous Lacrimal Sacs

We performed lacrimal sac biopsies in 35 patients who underwent dacryocystohinostomies in Saudi Arabia. The nasolacrimal duct obstructions were presumably secondary to trachoma in 22 cases and unknown in the remaining 13 cases. Lacrimal sac specimens were submitted for routine histopathologic examination and the direct-smear fluorescent antibody test. All specimens were negative for chlamydia. Possible reasons for the low detection rate of chlamydia in the trachomatous group were the frequent finding of clinically inactive disease, common canalicular obstruction, and loss of the epithelial lining in the lacrimal sac.

Delayed Tarsal Eversion Following Periorbital Trauma

Delayed onset of upper lid edema with exuberant chemosis developed in a 3-year-old girl following blunt periorbital trauma. Examination under anesthesia demonstrated a tightly everted upper tarsus that focally compressed the underlying conjunctiva at the superior tarsal border. Injection of subconjunctival hyaluronidase followed by local compression and temporary tarsorrhaphy resulted in rapid resolution of the chemosis and restoration of the normal lid position.

Congenital upper lid retraction: A histological and ultrastructural study

Congenital upper lid retraction is a rare anomaly in which one or both eyelids are abnormally elevated. Unilateral involvement in males is the most frequent presentation. An intrinsic disorder of the levator or Muller muscle is the most likely etiology, since neurological, systemic, or orbital abnormalities have not been described. The authors describe two cases of congenital upper lid retraction. The levator muscle from one patient and an age matched control were examined histologically and ultrastructurally. The lid retraction and control specimens demonstrated similar fiber diameters, similar amount of connective tissue between fascicles, and absence of inflammatory cells or intercellular edema. Electron microscopy of the lid retraction specimen showed small areas of Z-line streaming and myofibrillar disarray which were not demonstrated in the control specimen. The significance of these ultrastructural findings is unclear, since they have been reported in some healthy young subjects, as well as in seve...