Congenital lacrimal fistula associated with Down's syndrome.
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Child, Preschool↗
Biomedical subjects
M P Clarke
Publications and source records attributed to M P Clarke.
Iris sector heterochromia as a marker for neural crest disease.
A 6-month-old female infant with biopsy-proved Hirschsprung's disease had associated sector heterochromia of the irides. The association between sector heterochromia and Hirschsprung's disease has been previously reported and both conditions have been ascribed to neural crest defects. Histologic characteristics of the ocular involvement have not previously been reported, to our knowledge. Histopathologic examination of the globes revealed decreased iris stroma, decreased pigmentation in the anterior stroma, and reduced numbers of pigment-producing cells in the affected areas. Both the ocular and gastrointestinal findings reflect abnormalities in tissues of neural crest origin.
Female↗
Eyelid and fornix reconstruction in bilateral abortive cryptophthalmos (Fraser syndrome).
Cryptophthalmos refers to a group of uncommon congenital anomalies of eyelid formation that can occur alone or in combination with multiple congenital anomalies as part of the Fraser syndrome. We present a case of bilateral abortive cryptophthalmos in a child with Fraser syndrome and discuss the problems of surgical management. A brief discussion of isolated and syndromic cryptophthalmos, including normal eyelid development, the pathogenesis of cryptophthalmos, and the management options follows.
Abnormalities, Multiple↗
Management of congenital dacryocystocoele.
Congenital dacryocystocoele occurs when the nasolacrimal drainage apparatus in the newborn has concomitant blocks at the level of the junction of the common canaliculus with the lacrimal sac and at the distal end of the nasolacrimal duct. This results in a typical pink or blue swelling in the region of the medial canthus. Spontaneous resolution is common, although dacryocystitis may supervene. Treatment should be conservative unless dacryocystitis occurs, or intranasal extension coexists. A series of seven consecutive cases is presented and a management plan for the neonate with congenital dacryocystocoele is proposed.
Cysts↗
The surgical management of congenital lacrimal fistulae.
Congenital lacrimal fistulae are developmental anomalies of the lacrimal apparatus that are usually symptomatic, frequently causing epiphora and occasionally causing fistulitis or dacryocystitis. They may be associated with other abnormalities of the lacrimal system or with systemic anomalies. Complete excision alone, or in combination with nasolacrimal intubation and/or dacryocystorhinostomy is recommended for treatment.
Dacryocystorhinostomy↗
Familial congenital corneal anaesthesia.
Congenital corneal anaesthesia is a cause of severe corneal ulceration and scarring in childhood. Although uncommon, it may be underdiagnosed when present as an isolated entity. Measures such as the use of elbow splints and tarsorrhaphy may be necessary to prevent visual loss. In rare instances, the condition may be inherited. A family is presented with autosomal dominant isolated congenital corneal anaesthesia, and the systemic associations and treatment of the condition are reviewed.
Adult↗
Senior-Loken syndrome. Case reports of two siblings and association with sensorineural deafness.
Two siblings with Senior-Loken syndrome are described. The need for a full evaluation of renal function and hearing in children with a retinal dystrophy is emphasised.
Child, Preschool↗
MR angiography of the liver.
Recently, several versatile techniques have been developed for performing magnetic resonance (MR) angiography in the body. Relative to MR angiography in the head and neck, these methods require more interactive involvement by a physician to derive their full benefit, but they show great promise in the clinical workup of several vascular problems relating to the liver. This article outlines practical clinical applications for MR angiography of the liver, based on the experience of the authors and others working in the field.
Blood Vessels↗
Ophthalmic manifestations of metastatic carcinoma of the breast.
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Adenocarcinoma↗
The development of cataract following trabeculectomy.
OBJECTIVE: To determine the risk of cataract development following trabeculectomy. DESIGN: A prospective follow-up study of patients seen pre-operatively and at one, three, six and 12 months following surgery and subsequently at yearly intervals. SETTING: Ophthalmology Department of Nottingham University Hospital. PATIENTS: Fifty-seven patients with primary open angle glaucoma, including pseudoexfoliation, undergoing trabeculectomy. MAIN OUTCOME MEASURES: Increases in lens opacity measured by the Lens Opacity Meter 701, the Keeler Projectoscopy and clinical grading. RESULTS: Of 35 patients seen at six months post-operatively, none had lost two Snellen lines of visual acuity due to lens opacity, although three patients showed evidence of increasing lens opacity on all three parameters. CONCLUSION: There is no evidence of visually significant lens opacity occurring at six months post-operative follow-up.
Adult↗
Influence of pupil size on measurements made with the Lens Opacity Meter 701.
The Lens Opacity Meter 701 (Interzeag, Switzerland) is a recently developed instrument for assessing cataract. It measures the degree of scatter of a red light beam by the lens. Fifty patients prior to cataract surgery had measurements made before and after dilatation of the pupil. Inconsistent results were found at pupil diameters of less than 4 mm. It is postulated that this was due to absorption of scattered light by the iris.
Cataract↗
Do intraocular lenses with ultraviolet absorbing chromophores protect against macular oedema?
In a prospective study, designed to test the effect of intraocular lenses containing an ultraviolet absorbing chromophore on the incidence of post-operative cystoid macular oedema, 55 patients undergoing extracapsular cataract extraction were randomly allocated to receive intraocular lenses with or without an ultra-violet absorbing chromophore. Post-operative fluorescein angiography showed no difference in the incidence of macular oedema in those patients receiving an intraocular lens with an ultraviolet absorbing chromophore compared with patients receiving a standard posterior chamber intraocular lens.
Aged↗
Prospective comparison of preoperative imaging and intraoperative ultrasonography in the detection of liver tumors.
A prospective evaluation of the accuracy of preoperative computed tomography (CT), ultrasonography (US), and angiography was performed in 54 patients undergoing resection of hepatic neoplasms. The results were compared with surgical findings and intraoperative ultrasonography (IOUS). A total of 167 lesions was seen by means of IOUS, of which preoperative US enabled detection of 127 (76%). In 48 patients CT allowed detection of 91 of 150 lesions (61%), and in 35 patients angiography showed 56 of 107 lesions (52%). When the detection rate is analyzed according to hepatic segment, the greater overall accuracy of preoperative US may be attributed to a markedly better detection rate in lateral segment of the left lobe of the liver. Lesion size also represented a factor, with preoperative US allowing detection of a greater number of small (less than 2 cm) lesions compared with CT. In patients studied with both CT and US, the combined lesion-detection rate increased to 81% in the right lobe and 76% in the left lobe. Because of this we recommend that preoperative assessment include both CT and US evaluation of the liver. IOUS showed 25% to 35% additional lesions compared with preoperative US and CT. More importantly, 40% of the lesions demonstrated by IOUS were neither visible nor palpable at surgery. We recommend that IOUS be considered in all patients in whom resection of hepatic neoplasm is planned.
Angiography↗
Contrast sensitivity and pattern ERG in Waldenstrom's macroglobulinaemia.
Patients with Waldenstrom's macroglobulinaemia (WM) and age matched controls have been examined using tests of contrast sensitivity and pattern electroretinography. The patients with WM were in remission on maintenance treatment and had only moderately elevated plasma viscosity. They showed evidence of abnormal contrast sensitivity but there was no significant difference in amplitude of the pattern ERG between patients and controls.
Blood Viscosity↗
Oculomotor nerve palsies in children.
Fifty-four patients with oculomotor nerve palsy who presented over a 21-year period at our institution were reviewed retrospectively. There were 38 isolated third nerve lesions, and 16 with additional cranial nerve involvement. Eleven cases were congenital in origin, and 43 were acquired. Of the acquired group, 31 were traumatic, 7 infection-related, 3 attributed to migraine or other vascular causes, and 2 neoplastic. Average follow up was 36 months. The congenital lesions were predominantly right-sided; amblyopia, although common, responded well to treatment. Trauma and bacterial meningitis accounted for more cases of isolated oculomotor nerve palsy than seen in the previous literature. In distinct contrast to the adult population, no cases of diabetes, posterior communicating artery aneurysms, metastatic tumors, or pituitary lesions were found.
Adolescent↗
The ocular manifestations of the Sturge-Weber syndrome.
Sturge-Weber syndrome (SWS) is a rare neuro-oculocutaneous disorder. The ocular component manifests as glaucoma and vascular malformations of the conjunctiva, episclera, choroid, and retina. The authors reviewed the records of and recalled for examination 51 consecutive patients with a diagnosis of SWS seen at The Hospital for Sick Children, Toronto, Ontario, Canada, from 1980 to 1991. Of the 51 patients, 36 (71%) had glaucoma, 26 experienced onset before 24 months of age and the remainder after 66 months of age, 35 (69%) had conjunctival or episcleral hemangiomas, and 28 (55%) had choroidal hemangiomas, which were bilateral in 12 patients. Other ocular manifestations (retinal vascular tortuosity, iris heterochromia, retinal detachment, and strabismus) also occurred. Thirty-four of 51 (67%) glaucomatous eyes had a final visual acuity of 20/40 or better, or central, steady, and maintained fixation in each eye.
Child↗
Multiple congenital contractures (arthrogryposis) in association with Peters' anomaly and chorioretinal colobomata.
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Abnormalities, Multiple↗