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Biomedical subjects

G T Lueder

Publications and source records attributed to G T Lueder.

At least 37 records · Page 2Linked to original sources

Retinoma associated with vitreous seeding.

PURPOSE/METHODS: Malignant transformation of retinoma to retinoblastoma associated with vitreous seeding has been reported in three patients. We examined two patients with retinomas associated with vitreous seeding who have been followed up for eight years and 33 years. RESULTS/CONCLUSION: The lesions in both patients have remained stable without signs of malignant transformation. Vitreous seeding alone does not signify malignant transformation of a retinoma.

Adult↗

Corneal abnormalities in a mother and daughter with focal dermal hypoplasia (Goltz-Gorlin syndrome).

PURPOSE/METHODS: Focal dermal hypoplasia is an inherited dermatologic disorder commonly associated with skeletal and dental abnormalities. Ocular abnormalities frequently found in patients with focal dermal hypoplasia include microphthalmos, anophthalmos, and colobomas. Corneal abnormalities rarely have been described in patients with focal dermal hypoplasia. We examined a mother and daughter with focal dermal hypoplasia with distinctive corneal lesions. RESULTS/CONCLUSION: Several discrete vascularized peripheral subepithelial corneal opacifications were present bilaterally in both patients with focal dermal hypoplasia. No ocular abnormalities that would predispose to these abnormalities were found. These corneal lesions appear to represent an unusual manifestation of focal dermal hypoplasia.

Adolescent↗

Duane retraction syndrome associated with Rubinstein-Taybi syndrome.

The authors present a case report of Duane retraction syndrome (DRS) associated with Rubinstein-Taybi syndrome (RTS). RTS is a multisystem condition characterized by psychomotor developmental delay, broad thumbs and great toes, short stature, and characteristic facies with a beaked nose. DRS is an ocular disorder characterized by retraction of the globe with narrowing of the palpebral fissure in attempted adduction, with abduction and/or adduction deficiency, and, frequently, upshoot or downshoot of the affected eye on adduction. The case report described represents the first reported case of DRS associated with RTS, an association not previously recognized. This association raises the possibility that the abnormal CNS development in RTS may occasionally involve the ocular motor nerves.

Child↗

Retinoblastoma.

Retinoblastoma (RB) is the most common intraocular malignancy of childhood, occurring in approximately 1 in 20,000 live births. It arises from pluripotential retinal progenitor cells. Mutations of the RB1 gene interfere with regulation of the cell cycle. Patients who harbor a germinal mutation of the RB1 gene are at risk for the development of second, non-ocular tumors.

Child, Preschool↗

Long-term results of adjustable suture surgery for strabismus secondary to thyroid ophthalmopathy.

PURPOSE: To review the results of adjustable suture surgery in patients with strabismus secondary to thyroid ophthalmopathy to determine long-term outcome and to identify postoperative complications. METHODS: The records of 1524 patients with thyroid ophthalmopathy were retrospectively reviewed to identify those who required treatment for strabismus in the ocular motility clinic. Treatment consisted of adjustable suture surgery, prisms, or both. Elimination of diplopia in primary and reading positions was used at the criterion for success. RESULTS: Forty-seven patients were treated with adjustable suture surgery, with an average follow-up of 41 months. Results after 1 or more surgeries were: 47% excellent, 26% good, 19% fair, and 9% poor. Significant postoperative complications included eyelid retraction and A-pattern exodeviation. Sixteen of 18 patients with fair or poor outcomes after the initial surgery were recognized within 6 months. Postoperative changes in vertical deviation from primary position to downgaze were predictive of postoperative diplopia in downgaze. Analysis of multiple preoperative characteristics showed no statistically significant associations with outcome. Eight additional patients had adequate relief of diplopia using prisms alone, with an average follow-up of 49 months. CONCLUSIONS: Long-term symptomatic relief of diplopia was obtained in the majority of patients using adjustable suture strabismus surgery, combined occasionally with small amounts of prism postoperatively. Prisms alone provided effective long-term relief in patients with small-to-moderate deviations.

Fluorescein Angiography↗

Heritable retinoblastoma and pinealoma.

A fourth case of bilateral retinoblastoma (RB) associated with pinealoma was identified among 56 patients with heritable RB. Using life-table analysis, the association was 5.6% 2 years after RB diagnosis. Retinoblastoma-pinealoma (RB-P) was uniformly fatal in this series and in 40 additional patients described in the literature. Ninety-five percent of patients with RB-P had bilateral RB, a positive family history of RB, or both. All patients with pinealoma presented with symptoms of increased intracranial pressure due to obstructive hydrocephalus. The advanced stage of the tumors at diagnosis may contribute to their uniform lethality. The incidence of a positive family history of RB in patients with pinealoma was higher than expected (62% of patients), which may be related to earlier surveillance and treatment of patients with positive family histories of RB.

Brain Neoplasms↗

Second nonocular tumors in survivors of heritable retinoblastoma.

We reviewed the records of 50 patients with heritable retinoblastoma to study the cause of death, if deceased, or the incidence of second nonocular malignant neoplasms. The incidence of second tumors in these patients was 6% at ten years, 14% at 20 years, and 14% at 30 years. These findings are lower than previously reported results. Pinealoma was the probable cause of death in three of the five patients who died of second nonocular tumors.

Adult↗

Synthetic granuloma.

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Eye Foreign Bodies↗

Alveolar capillary dysplasia with misalignment of pulmonary veins and anterior segment dysgenesis of the eye: a report of a new association and review of the literature.

The association of alveolar capillary dysplasia with misalignment of pulmonary veins (ACD-MPV) and ocular abnormalities has not been previously reported. We present a case of ACD-MPV and anterior segment dysgenesis of the eye in a full-term infant as well as a review of the relevant literature.

Abnormalities, Multiple↗

Neonatal dacryocystitis associated with nasolacrimal duct cysts.

Neonatal dacryocystitis is a rare complication of congenital nasolacrimal duct (NLD) obstruction. Recent reports suggest that the association of neonatal dacryocystitis and NLD cysts may be more common than previously believed. The purpose of this study is to describe the nasal endoscopic findings and treatment of three patients with this disorder. In this prospective study, three consecutive patients who presented at 2 weeks of age or less with dacryocystitis were treated with NLD probing and rigid nasal endoscopy. All patients were found to have congenital NLD cysts at the time of NLD probing. The cysts were marsupialized under endoscopic visualization. The treatment was successful in all patients. Dacryocystitis in the early newborn period is commonly associated with NLD cysts. Nasal endoscopic marsupialization of the cysts is an effective adjunct to treatment of these patients.

Cysts↗