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Biomedical subjects

P B Mullaney

Publications and source records attributed to P B Mullaney.

34 records · Page 2Linked to original sources
PubMed1997

Retinal detachment associated with type III retinoblastoma regression after cryotherapy and external-beam radiotherapy.

PURPOSE: To report a child with rhegmatogenous retinal detachment originating from a focus of type III retinoblastoma regression after cryotherapy and external-beam radiation therapy. METHOD: Retinal detachment and multiple retinal holes, which were closely associated with the calcified mass of the regressed retinoblastoma, were treated with cryotherapy and scleral buckle. RESULTS: Positioning the regressed tumor and the retinal holes on the scleral buckle closed the holes. Subretinal fluid gradually reabsorbed after surgery, and complete retinal reattachment resulted. CONCLUSION: Rhegmatogenous retinal detachment is rare in patients with retinoblastoma, and scleral buckle can successfully manage such cases. In our patient, the detachment probably resulted from retinal necrosis secondary to cryotherapy and external-beam radiation therapy.

Calcinosis↗
PubMed1997

Workup for metastatic retinoblastoma. A review of 261 patients.

PURPOSE: The means available to screen for retinoblastoma metastases, including bone marrow aspiration, lumbar puncture, and radionuclide scans, offer variable usefulness at different stages of the disease. In this study, the authors attempted to assess the value of these tests as part of the initial workup for metastases. METHODS: Medical files of 261 patients with retinoblastoma were reviewed, and the results of bone marrow, lumbar puncture, and bone and liver radionuclide scans were correlated with the laterality, clinical staging, and histopathologic findings. The presence or absence of tumor in the choroid and the optic nerve also were correlated with the results of diagnostic tests. RESULTS: Of 261 patients with retinoblastoma, 147 (56.3%) and 114 (43.7%) had unilateral and bilateral disease, respectively; 11.6% of unilateral cases and 14.9% of bilateral cases had distant metastasis (P = 0.2). Bone marrow aspirations were performed on 101 patients (38.7%), and 10 (9.9%), of these specimens showed the presence of retinoblastoma cells in the aspirate. Ninety-four (36%) of the patients were examined with spinal tap cytology, and the results of four (4.3%) of these were positive for tumor cells. Radionuclide bone and liver scans were performed on 49 (18.8%) and 48 (18.4%) patients, respectively; 5 (10.2%) had abnormal bone scans and 3 (6.2%) had abnormal liver scans. The average age in the metastatic group was 3.1 years, whereas the average age in the nonmetastatic group was 2.3 years. All of our abnormal test results were found in patients with stages III and IV disease, with the exception of one abnormal lumbar puncture in a patient with stage II disease. The correlation of diagnostic tests with the histopathologic findings showed that with no choroidal involvement, no positive diagnostic tests were encountered. Correlation between positive diagnostic test results and the level of optic nerve involvement failed to indicate any trends. CONCLUSION: Our study indicated a good interrelation between the positivity of diagnostic tests (bone marrow and bone scan) and higher stages of the disease (stages III and IV) and choroidal involvement in enucleated eyes, with statistically significant correlations; correlations with lumbar puncture and liver scan were not significant.

Adolescent↗
PubMed1997

Presentation of retinoblastoma as phthisis bulbi.

PURPOSE: We sought to determine the incidence of retinoblastoma patients who presented with phthisis bulbi. METHODS: The medical records of 272 patients in the King Khaled Eye Specialist Hospital Retinoblastoma Registry were retrospectively studied. Clinical records, radiological investigations and histopathological slides were reviewed. RESULTS: We found that 2.7% of patients had retinoblastoma coincident with phthisis bulbi. Five of 10 patients had bilateral retinoblastoma; in the others it was unilateral. Radiologically, intraocular calcification was present in all except one case. All enucleated phthisical globes had residual viable tumour cells; optic nerve extension was found in 2 patients who had long-standing phthisis bulbi. CONCLUSION: Phthisis bulbi is an uncommon presenting sign of retinoblastoma which often occurs after an ocular inflammatory episode possibly related to intraocular tumour infarction. In most cases the tumour is not visible because of intraocular disruption. That every enucleated eye in this series harboured well-differentiated tumour cells underlies the seriousness with which phthisis bulbi of unknown origin in children should be investigated for retinoblastoma.

Adolescent↗
PubMed1996

Dissolution of pseudophakic fibrinous exudate with intraocular streptokinase.

A fibrinous exudate developed in 8 children after cataract extraction. Blunt trauma was the cause of cataract formation in 6 children; the other 2 presented with congenital cataract. In 4 of 6 children with traumatic cataract, iris damage was evident. Seven children had an intraocular lens implanted. The development of fibrinous exudate in the anterior chamber was noted despite routine post-operative topically administered steroids. It typically formed 1-4 days after surgery. In all cases topical fortified steroids were unsuccessful in hastening dissolution. Intracameral injection of streptokinase (500-1000 IU) caused dissolution of fibrin in all cases. No adverse effects were apparent clinically with intraocular administration of streptokinase.

Adolescent↗
PubMed1996

Retinoblastoma referral patterns in Saudi Arabia.

The purpose of this study was to review the patterns of referral and presentation of patients with retinoblastoma in Saudi Arabia from 1983-1994. Retinoblastoma represents a particularly high volume at the King Khaled Eye Specialist Hospital and the Hospital's Retinoblastoma Registry provided relevant data. Records of 257 registered patients were analyzed. Of these, 112 were bilateral whereas 145 were unilateral, resulting in a total of 369 eyes. Referral diagnosis accuracy was high, 75% of referring physicians ranking retinoblastoma at the top of their differential diagnosis. Delay in referral was found to average 9.4 weeks. However, 49.4% of eyes were staged at Reese-Ellsworth Stage V at presentation. Significantly, the number of patients presenting with extraocular disease fell from 22.8% (1983-1988) to 12.3% (1989-1994), highlighting increasing awareness of retinoblastoma and availability of services in Saudi Arabia.

Adolescent↗
PubMed1995

Congenital hereditary endothelial dystrophy associated with glaucoma.

BACKGROUND: Three children, ranging in age from 2 to 6 months, had diffuse and homogeneously opaque corneas, clinically consistent with congenital hereditary endothelial dystrophy. Bilateral elevated intraocular pressure (IOP) was a feature in all three children. METHODS: Initially, all patients underwent glaucoma surgery to reduce IOP. Subsequently, a penetrating keratoplasty was performed in one eye of each patient to clear the visual axis. The excised corneal button was examined by light microscopy and by transmission and scanning electron microscopy. RESULTS: Postoperatively, all patients maintained clear corneal grafts. Results of histopathologic examination showed an absence of the endothelial cell layer in all patients. The presence of a variably thick collagenous layer posterior to the anterior banded zone of Descemet's membrane and the absence of endothelial cells were noted on transmission electron microscopy. Scanning electron microscopy confirmed absent, or scanty, and abnormal endothelial cells. CONCLUSION: The authors describe three patients with a clear association between congenital glaucoma and congenital hereditary endothelial dystrophy. This combination should be suspected where persistent and total corneal opacification fails to resolve after normalization of IOP.

Cataract↗
PubMedDate not supplied

Diagnosis and management of iris juvenile xanthogranuloma.

BACKGROUND: Juvenile xanthogranuloma is a benign, self-limiting cutaneous disorder most commonly encountered during infancy. Approximately 10% of cases may develop ocular or adnexal involvement, most commonly in the iris. METHODS: We review clinical and morphological features of four cases of iris juvenile xanthogranuloma that reflect the diagnostic and therapeutic spectrum. RESULTS: Tissue diagnosis was confirmed in all cases; in one case, the disease was diagnosed with a skin biopsy and treated with local and systemic steroids, and its persistence in the iris was confirmed with a second tissue specimen obtained five months after systemic steroid treatment. CONCLUSION: The diagnosis and treatment of juvenile xanthogranuloma may be straightforward, particularly in cases when the ocular lesion receives early attention and responds well to topical steroids, and when there is no hyphema. However, in other instances, this entity may be difficult to manage and may necessitate iris biopsy for diagnosis and radiation therapy for treatment.

Administration, Topical↗
PubMedDate not supplied

Pediatric IOL implantation: the KKESH experience.

BACKGROUND: Intraocular lens (IOL) implantation is increasingly accepted as a means of correcting pediatric aphakia. However, few large series have been reported. We present our experience over the past 6 years. METHODS: Charts were reviewed of 84 patients (88 eyes) 8 years old or younger who underwent IOL implantation at King Khaled Eye Specialist Hospital between January 1989 and March 1995. An attempt was made to correlate visual outcome with patient variables, and refractive outcome was assessed. RESULTS: Despite a high percentage of patients with penetrating trauma (24%) and a significant level of poor compliance with amblyopia treatment (33%), one third of patients saw 20/40 or better, and 60% had at least 20/80 acuity. Improved visual outcome correlated with later onset and shorter duration of opacity and compliance with occlusion therapy. The posterior capsule was left intact in the majority of cases; opacification occurred in 47 eyes, 25 of which required Nd:YAG capsulotomy. No unexpected complications occurred. Prediction of postoperative pseudophakic refraction was within 2 diopters (D) in 70% of patients. CONCLUSION: IOL implantation in appropriate children can be used as a safe alternative to other methods of correcting pediatric aphakia. Visual outcome often depends on amblyopia management. These patients are appropriately followed by ophthalmologists who are experienced in managing amblyopia and pediatric pseudophakia.

Aphakia, Postcataract↗
PubMedDate not supplied

Tuberous sclerosis in infancy.

PURPOSE: To report two infants with tuberous sclerosis who initially were considered to have retinoblastoma. PATIENTS AND METHODS: An 8-day-old infant was referred with small tumors in the posterior poles of both eyes. A left microphthalmos with ciliochoroidal coloboma was present. Computed tomographic (CT) scanning of the brain showed scattered high-density subependymal foci in the lateral ventricle thought to be consistent with calcification resulting from intrauterine viral infection. Argon laser photocoagulation was applied to lesions in the right eye. Because one tumor was situated on the retina straddling the coloboma in the left eye, external beam radiotherapy was administered. A 5-month-old girl presented with a large mass in a left microphthalmic eye. Calcification was present on B-scan ultrasonography and CT scanning. Vitreous seeding was noted to originate from the tumor. The contralateral eye manifested four small gray translucent retinal tumors in the posterior pole. CT scan and magnetic resonance imaging (MRI) revealed multiple periventricular subependymal lesions, including one at the foramen of Monro. RESULTS: Repeated examinations in the younger child under anesthesia revealed small new retinal lesions that appeared to enlarge gradually. She developed intractable seizures and her electroencephalogram revealed a modified hypsarrhythmia recording. A careful review of available CT scans and MRI displays suggested the diagnosis of tuberous sclerosis. The child's most recent examination under anesthesia revealed multiple newly developed hamartomas. In the older child, prompt diagnosis was made on the basis of the intracranial radiologic findings. CONCLUSION: Retinal hamartoma presentation may vary in infancy. Small, initially fleck-like gliotic lesions appear to enlarge gradually and eventually may form gray, translucent tumors. Large astrocytic hamartomas of the retina associated with tuberous sclerosis may resemble retinoblastoma, particularly if the mass is large, calcified, and associated with vitreous seeding. Although ophthalmic presentation was reminiscent of retinoblastoma in both patients, radiologic evidence of subependymal hamartomas pathognomic for tuberous sclerosis helped establish the correct diagnosis. We stress the importance of intracranial radiologic findings in this regard.

Diagnosis, Differential↗