A closer look at the eye in homocystinuria: a screened population.
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Biomedical subjects
Publications and source records attributed to M O'Keefe.
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A 7-month-old infant referred with suspected bilateral congenital glaucoma was noted under general anaesthesia to have simple megalocornea in association with contralateral glaucoma in an eye suspected of harbouring an intraocular tumour. Following enucleation, histopathology demonstrated persistent hyperplastic primary vitreous. The presence of simple megalocornea of autosomal recessive inheritance in association with contralateral persistent hyperplastic primary vitreous in a buphthalmic eye is of ophthalmic interest, and appears to be unique.
We report on 15 patients (10 boys, 5 girls) with clinical anophthalmos. Two of them had bilateral anophthalmos, 10 had systemic anomalies, and six had abnormalities of their remaining eye. Only two appeared to have an associated underlying aetiology. Fourteen patients underwent orbital reconstruction or socket enlargement with varying degrees of success. Our own experience suggests that unnecessary lid procedures should be avoided, but we recommend early prosthetic fittings. We feel that a multidisciplinary approach is necessary to attain useful rehabilitation.
Ocular abnormalities were present in five of 11 patients with Freeman-Sheldon syndrome. The predominant ocular findings were strabismus and blepharoptosis. Stiffness of the extraocular muscles, demonstrated in three patients by forced duction testing, made surgery difficult. Two patients underwent frontalis muscle sling surgery and a third underwent levator palpebrae superioris muscle resection. Although all three remained exotropic, the cosmetic results were good.
Orbital mucormycosis is reported in a healthy patient with maturity onset diabetes who was treated with orbital exenteration, amphotericin B, and ketoconazole. A six-year follow-up shows no evidence of recurrence.
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A 21-year-old soldier sustained a schrapnel blast injury during an accident with a mortar fuse. Surgical repair included the use of one of his reconstructed metacarpal bones to repair the orbital floor. The remainder of the orbital floor was repaired using maxillary bone. It proved an excellent method of repairing this patient's orbital floor.
Two patients with rheumatoid arthritis (two women, 58 and 62 years old) developed reversible penicillamine-induced ocular myasthenia gravis. Both had the HLA-DR1 antigen, unlike most patients with idiopathic myasthenia gravis who show HLA-DR3, suggesting that penicillamine-induced myasthenia gravis and its idiopathic counterpart occur in patients with different genetic backgrounds. In both cases, cessation of drug treatment led to resolution of the symptoms.
The first reported case of a patient with left internal mammary artery bypass graft dissection secondary to selective coronary and graft arteriography is presented. The intimal tear and false lumen could not be seen with flouroscopy during catheterization. This later led to antero-septal myocardial infarction.
The septum orbitale is an important landmark in ptosis surgery. It is structure that is difficult to identify and very easy to damage. In a series of 34 ptosis operations using a modified transcutaneous approach, we have shown that the septum orbitale can be readily identified, dissected and re-sutured after resection of the aponeurosis of the levator palpebrae superioris muscle.
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BACKGROUND: Lymphoepithelial-like carcinoma is a rare tumour type. The optimal treatment for this disease is not known. No effective therapies are described in the literature. AIMS: This report describes a case of lymphoepithelial-like carcinoma and documents a therapeutic strategy which has proved effective. RESULTS: The patient was initially treated with a common platinum-based chemotherapy regimen incorporating a taxane (Carbplatin and Docetaxel). Disease stabilization initially occurred but the patient soon progressed. The patient was then treated with VIP chemotherapy and had a complete response. CONCLUSION: VIP chemotherapy appears to be an effective therapeutic strategy in lymphoepithelial-like carcinoma.
PURPOSE: To assess prospectively the complications and changes in refraction, axial length, and keratometry after intraocular lens (IOL) implantation in children with congenital, developmental, and traumatic cataracts. SETTING: The Children's Hospital, Dublin, Ireland. METHODS: The study comprised 24 eyes of 20 patients, aged 4 weeks to 12 years, who had extracapsular cataract extraction and posterior chamber IOL implantation. Mean follow-up was 103 weeks (range 34 to 270 weeks). RESULTS: Six eyes with congenital cataracts operated on between 4 and 28 weeks of age had central, steady, maintained fixation postoperatively. In the developmental cataract group, 64% achieved a visual acuity of 6/24 or better and 43%, 6/12 or better. In the three traumatic cases, visual acuities were 6/6, 6/9, and 6/24. Posterior capsule opacification occurred in 95.8% of eyes and was treated with a neodymium: YAG or Zeigler knife posterior capsulotomy as a secondary procedure. Other postoperative complications (membranous uveitis, iris capture, posterior synechias, iris prolapse) occurred in 29.2% of eyes. CONCLUSION: With careful management and patient selection, the use of IOLs in children can produce good visual results with a minimum of complications. Further follow-up is needed to assess the long-term visual outcome, complications, and changes in refraction.
A 36-year-old woman had uneventful astigmatic myopic laser in situ keratomileusis (LASIK) to correct -12.00 -1.50 x 70. Three days later, she developed a corneal abscess, hypopyon, and an intense vitreous cellular reaction-endophthalmitis. The patient was immediately given intravenous ciprofloxacin and topical vancomycin and ceftazidime. The infecting organism was Streptococcus pneumoniae. One day after therapy was instituted, the hypopyon resolved. Seven months later, best corrected visual acuity was 20/25 and refractive error, -4.00 diopters. A stromal scar (grade 2 haze) was causing a slight reduction in acuity. Endophthalmitis after LASIK, if treated promptly, need not lead to a permanent reduction in visual acuity.
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