Orbicularis myectomy with levator advancement in Schwartz-Jampel syndrome.

PURPOSE: Schwartz-Jampel syndrome is a disorder of continuous myotonia causing blepharospasm, acquired ptosis, and blepharophimosis. We report the management of the associated eyelid anomalies with orbicularis oculi myectomy, levator aponeurosis resection, and lateral canthopexy. METHODS: Interventional case reports. Two patients with Schwartz-Jampel syndrome presented with blepharospasm, acquired ptosis, and blepharophimosis. Orbicularis myectomy, levator aponeurosis resection, and lateral canthopexy were performed to relieve the blepharospasm and to correct the ptosis and blepharophimosis. RESULTS: Significant functional and cosmetic improvements were achieved by increasing the palpebral fissure height and length while greatly decreasing the blepharospasm of the patients. No recurrence of the blepharospasm or eyelid anomalies has been noted after 1 and 15 years of follow-up, respectively. CONCLUSION: The blepharospasm and eyelid alterations caused by Schwartz-Jampel syndrome should be treated to provide functional and cosmetic improvements. Our technique of myectomy, levator resection, and lateral canthopexy provides an excellent, long-lasting result.

Solitary fibrous tumor of the orbit in a child.

PURPOSE: Solitary fibrous tumor is a rare spindle-cell tumor that usually is seen in the pleura. The orbit is one of the most common extrapleural sites. It is frequently misdiagnosed as hemangiopericytoma and is seen in older patients. We present the youngest case of this tumor, which was apparent in family photographs by age 10 and removed at age 15. The first reported echography findings are presented. METHODS: Case report and literature review. RESULTS: Solitary fibrous tumor was diagnosed by microscopy and immunohistochemical study that showed cells reactive with vimentin and CD34. CONCLUSIONS: Solitary fibrous tumor of the orbit has been diagnosed with increasing frequency in recent years as the result of improved methods of pathologic examination. It is important to be aware of this tumor and recognize that it must be included in the differential diagnosis of highly vascular spindle-cell tumors even in young children.

A new groove director for simplified nasolacrimal intubation.

Silicone intubation is commonly performed to maintain patency of the lacrimal drainage system. We describe a new lacrimal groove director, designed to simplify retrieval of the metal Crawford probe with minimal trauma to the nasal mucosa. This instrument can also be used to infracture or medialize the inferior turbinate in both pediatric and adult populations during nasolacrimal duct intubation or external or endoscopic dacryocystorhinostomy.

Management of eyelid anomalies associated with Blepharo-cheilo-dontic syndrome.

PURPOSE: To describe the previously unreported management of the eyelid anomalies associated with blepharocheilo-dontic syndrome. Blepharo-cheilo-dontic syndrome is a syndrome of congenital cleft lip/palate, oligodontia, euryblepharon, eyelid ectropion, and lagophthalmos. METHODS: Case report. A 3-month-old male presented with bilateral upper and lower eyelid ectropion with eyelid retraction, marked euryblepharon, severe lagophthalmos, and a right side cleft lip/palate. Lateral tarsal strips, lower eyelid retractor disinsertion, myocutaneous advancement of the cheek and eyelids, and lateral tarsorrhaphy were performed. RESULTS: Correction of the ectropion, eyelid retraction, euryblepharon, and marked improvement in his lagophthalmos were achieved postoperatively. No recurrence of the ectropion or euryblepharon has been noted after 6 months of follow-up. CONCLUSION: Appropriate reconstructive surgery of the eyelids reduces the morbidity associated with the eyelid anomalies and provides an excellent cosmetic result for patients with blepharo-cheilo-dontic syndrome.

Decontamination of human sclera: an in vitro study.

PURPOSE: The human sclera is frequently used in ophthalmic surgeries and must be preserved in disinfectants that prevent its contamination. In this study the efficiency of glycerin, absolute alcohol (ethanol), and benzalkonium chloride (1:5,000) as human sclera disinfectants were compared. METHODS: Fresh human scleras were trephined, the scleral disks divided into three groups and contaminated with Staphylococcus aureus (ATCC 29213), Pseudomonas aeruginosa (ATCC 27853), or Bacillus cereus (ATCC 11778) for 24 h. Thereafter they were transferred to preservation vials each containing glycerin, absolute alcohol, benzalkonium chloride diluted in 70% alcohol (1:5,000) or Trypticase Soy Broth (control), respectively, and stored at room temperature. From each vial, two scleral disks were removed after 1, 2, 3, 4, 7, 10, and 14 days of immersion. Both were plated on blood agar, one being macerated, and both incubated at 37 degrees C for 48 h. RESULTS: Pseudomonas aeruginosa, S. aureus, and B. cereus were recovered from the glycerin-immersed scleral disks until the second, fourth, and fourteenth days, respectively. Bacillus cereus was recovered from those immersed in absolute alcohol until the fourteenth day, whereas disks infected with the other microorganisms and immersed in absolute alcohol presented no growth since the very first day of immersion. Bacillus cereus was recovered from scleral disks immersed in benzalkonium chloride diluted in 70% alcohol (1:5,000) only on the first day. CONCLUSION: Resistant microorganisms can survive in scleral tissue preserved in glycerin and absolute alcohol. We conclude that benzalkonium chloride diluted in 70% alcohol (1:5,000) in vitro is the best disinfectant for human sclera after 24 h.