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Br J Ophthalmol: first published as 10.1136/bjo.64.6.417 on 1 June 1980. Downloaded from

British Journal of , 1980, 64, 417-421

Retinal detachment in congenital

R. J. COOLING, N. S. C. RICE, AND D. MCLEOD From Moorfields Hospital, City Road, London ECJ V 2PD

SUMMARY This paper reports the occurrence of in 18 patients suffering from congenital glaucoma. Difficulty in establishing control of the glaucoma and the presence of high were common findings. The poor results of detachment surgery were related to opacities in the media preventing evaluation of the retinal details and the development of massive preretinal retraction.

Detachment of the complicating congenital cases which do not have any other developmental glaucoma was first described by Axenfeld,l who anomaly, either ocular or systemic, and show considered that the detachment was often the typical gonioscopic appearances.6 Complicated con- determining factor in the production of blindness in genital glaucoma refers to cases in which glaucoma this condition. Knapp2 also reported this develop- is related to other developmental defects of the eye ment in an 11-year-old child with advanced anterior with or without extraocular abnormalities. segment changes of congenital glaucoma; he con- A detailed profile of each patient was constructed sidered that degenerative changes within the vitreous from the clinical records, and particular attention were implicit in the development of retinal detach- was directed to the following factors: age of onset ment. of glaucoma; family history of glaucoma or retinal Cases of congenital glaucoma complicated by detachment; undertaken and the retinal detachment have also been mentioned within control of achieved; the maxi- surveys of juvenile detachment.3-5 In these series a mum corneal diameter recorded and the refractive total of 11 cases of congenital glaucoma were en- error; the age at which the patient developed retinal

countered among 672 cases of retinal detachment detachment, the type and extent of the detachment, http://bjo.bmj.com/ within the first 2 decades of life. and the nature of retinal breaks and other retinal This report concerns 18 patients with retinal abnormalities encountered; the retinal status of the detachment associated with congenital glaucoma. fellow eye; retinal surgery undertaken and the The clinical characteristics of these detachments results achieved. and the salient features of the associated glaucoma are described together with the results of retinal Results detachment surgery. The incidence of retinal detach- ment in a closely studied series of patients with Retinal detachment occurred in 21 of 18 patients on September 28, 2021 by guest. Protected copyright. simple congenital glaucoma is also reported. with congenital glaucoma of varying aetiology. There were 13 males and 5 females. The details of Patients and methods the glaucoma and the principal features of the retinal detachments are presented in Table 1. We reviewed the clinical records of 189 patients Congenital glaucoma was classified as simple in with congenital glaucoma involving 290 eyes who 16 and complicated in 2 of our patients. The age of were treated at Moorfields Eye Hospital between onset of glaucoma ranged from 3 weeks to 12 1960 and 1979. Thirteen eyes of 11 patients suffered months and was bilateral in 16 and unilateral in a retinal detachment. In addition 7 patients presented 2 cases. Glaucoma surgery was undertaken in 18 during this same period with retinal detachment in eyes of 16 patients, including filtering procedures in association with congenital glaucoma which had 10 eyes. Surgery was not performed on 1 eye with been managed elsewhere. simple glaucoma which had undergone spontaneous The glaucoma was classified as either simple or arrest and both eyes of a patient with bilateral complicated. Simple congenital glaucoma refers to Sturge-Weber syndrome. Satisfactory control of the glaucoma was achieved in 10 eyes of 11 patients. Correspondence to Mr R. J. Cooling, FRCS. The interval between the onset of glaucoma and 417 Br J Ophthalmol: first published as 10.1136/bjo.64.6.417 on 1 June 1980. Downloaded from

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Retilial detachment ini congenital glaucoma 419

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_ _ _ _ _ Br J Ophthalmol: first published as 10.1136/bjo.64.6.417 on 1 June 1980. Downloaded from

420 R. J. Cooling, N. S. C. Rice, and D. McLeod the development of retinal detachment ranged from 7 months to 33 years. The presenting features of retinal detachment included ocular with or without subluxation in 9 eyes, subjective visual loss in 8 eyes, vitreous haemorrhage in 3 Fig. I Central eyes, and the observation of retinal detachment horizontal B-scan shortly after filtering surgery in 1 eye. A wide range section of right eye of refractive errors was encountered. Of a total showing retinal of 18 eyes in which the error could be measured detachment and 15 eyes were found to have myopia in excess of ..*..-choroidal haemangioma 2 50 and in 8 eyes to be 10 or more dioptres (arrow). as expressed in spherical equivalents. Retinal detachment was unilateral in 15 and bilateral in 3 cases. With the exception of serous detachments in both eyes of the patient with bilateral Sturge-Weber syndrome the detachment was con- sidered to be rhegmatogenous in all cases. However, retinal breaks were identified in only 9 eyes and wherever possible. In one eye a flat u-tear in tem- included u-tears in 4 eyes, round holes in the tem- poral retina was identified for which prophylactic poral periphery with multiple incomplete dialyses was undertaken. In a further case the in I eye, giant pre-equatorial tears in 2 eyes, and fellow eye of a patient with a giant retinal dialysis giant dialyses in 2 eyes. showed marked cystoid degeneration of the ora Retinal detachment surgery was undertaken in a serrata in all quadrants, which was treated by 360° total of 13 eyes of 12 patients. The limited potential cryotherapy. for useful visual recovery together with the presence Twelve eyes of 10 patients in this study belonged of significant corneal and lens opacities were the to a closely studied series of 158 cases of simple major factors precluding any attempt at surgical congenital glaucoma involving 246 eyes followed by management in the remaining 8 eyes. Encirclement the late A. Lister and one of the authors (N.S.C.R.). with or without additional silicone sponge explants The results of surgery and the overall complications was performed in 8 eyes and local procedures alone within this series have been the subject of a previous in 3 eyes. Silicone oil injection was used in 3 eyes report.7 Over a follow-up period ranging from 2 to

with massive preretinal retraction. Pars plana 19 years this represents an incidence of retinal http://bjo.bmj.com/ for massive preretinal retraction was detachment of 6 3%. performed on 2 eyes. In 3 eyes detachment surgery was successful as Discussion defined by retinal reattachment for 6 months or longer following surgery. In 2 of the 5 eyes in which Early reports of retinal detachment in congenital silicone oil injection was used anatomical reattach- glaucoma concerned eyes severely compromised by ment with visual improvement was achieved. The the effects of uncontrolled glaucoma.' 2 An increased causes of failure of detachment surgery included risk of retinal detachment was thought to accompany on September 28, 2021 by guest. Protected copyright. severe intraocular haemorrhage in one eye and surgical intervention in cases of uncontrolled massive preretinal retraction in 8 eyes. glaucoma.2 However, there have been few reports Cases classified as complicated congenital glau- of this in patients observed over a coma included a patient with Axenfeld's syndrome prolonged period following the introduction of which proved extremely resistant to all forms of more effective methods of glaucoma control. attempted glaucoma control and the case of bilateral Myopia is the most common in Sturge-Weber syndrome. The latter patient de- congenital glaucoma and is usually between 1 and 7 veloped bilateral serous retinal detachments in dioptres.8 The axial length and the degree of myopia association with diffuse choroidal angiomata are not constantly related, since the anticipated (Fig. 1). The only treatment considered likely to myopia is mitigated by changes in corneal curvature achieve retinal reattachment was the use of silicone and posterior displacement of the lens. Myopic oil injection. In the left eye a successful result was fundus changes in hydrophthalmia were described achieved whilst surgery of the right eye was compli- by Parsons8 and included retinal pigment atrophy cated by the development of severe intraocular and disc crescents. Myopia was an almost constant haemorrhage. finding in our patients, exceeding 10 dioptres in 8 The retinal status of the fellow eye was assessed of the eyes. This excess of highly myopic eyes Br J Ophthalmol: first published as 10.1136/bjo.64.6.417 on 1 June 1980. Downloaded from

Retinal detachment in congenital glaucoma 421 implies significant accelerated axial growth, though sidered likely to achieve retinal reattachment was we did not determine the axial length of the eyes. silicone oil injection, and a successful result, that is, With a single exception all patients received more than half of the retina reattached accompanied miotic therapy at some stage in their clinical course. by visual improvement, was achieved by this method No significant association between the use of in only 2 eyes. miotics and the development of retinal detachment Prolonged follow-up of patients suffering from was recognised. None of the patients were known to congenital glaucoma has identified a variety of have received irreversible inhibitors, factors which may limit the visual outcome or which might be considered inappropriate in cases of prejudice continued visual function. These include advanced uncontrolled glaucoma with the attendant ,'1 delayed recurrence of glaucoma, and risk of retinal detachment. late endothelial decompensation necessitating kera- The features of congenital glaucoma commonly toplasty.12 Retinal detachment is a further compli- encountered in our patients included bilateral cation which may develop in buphthalmic eyes at involvement and the necessity for repeated surgical varying intervals after the onset of glaucoma and intervention. These patients are commonly found would appear to carry an extremely poor prognosis. within the estimated 15% of all patients with simple congenital glaucoma who are not effectively con- We thank Mr L. G. Fison and Mr Redmond J. H. Smith for trolled by goniotomy alone.7 Serious impairment of allowing us to study patients under their care. The surgical vision was often present before the onset of retinal management of case 13 was undertaken by Mr J. D. Scott, to whom we are indebted for supplying the surgical details. detachment as a result of anterior segment changes We also thank Miss Marie Restori, who performed the and glaucomatous damage. However, in ultrasound. and Miss Heather Lucas for typing the manu- several cases the fellow eye also had poor visual script. function attributed to severe corneal scarring, dense amblyopia, or glaucomatous optic atrophy. References In 19 of 21 eyes the retinal detachment was con- sidered to be of rhegmatogenous origin, though 'Axenfeld T. Uber das Vorkommen von Netzhautablosung und uber die Bedeutung allgemeiner vasomotorischer retinal breaks were only identified in 9 eyes. The Storungen beim Hydrophthalmus. Klin Monatsbl Augen- presence of corneal scarring or precluded heilkd 1903; 41: Beilagehaft 1-16. visualisation of retinal details in many cases. Of the 2Knapp A. Retinal detachment in hydrophthalmia. J AMA retinal breaks that were identified, vitreoretinal 1916; 67: 356-7. 3Muinos A, Sellyei L. Juvenile retinal detachment at the traction played a predominant role in their origin. Barraquer Clinic. Mod Prob Ophthalmol 1969; 8: 284-93. Giant retinal breaks were located at varying distan- 4Gailloud C, Dufour R, von Barsewisch B, Meyer Schwick- http://bjo.bmj.com/ ces from the ora serrata but in all instances appeared erath G. Juvenile retinal detachment. Mod Probl Ophthal- to be pre-equatorial. Giant retinal breaks have been mol 1969; 8: 152-82. 5Daniel R, Kanski JJ, Glasspool MG. Retinal detachment described in association with various developmental in children. Trans Ophthalmol Soc UK 1974; 94: 325-34. anomalies.5 9 10 6Worst JGF. The Pathogenesis of Congenital Glaucoma. The results of detachment surgery in cases of Springfield, Illinois: Thomas, 1966. congenital glaucoma are frequently disappointing 'Rice NSC. The surgical management of the congenital . Aust J Ophthalmol 1978; 5: 174-9. in our experience. Of a total of 13 eyes considered 8Parsons JH. Refraction in Buphthalmia. Br J Ophthalmol on September 28, 2021 by guest. Protected copyright. likely to benefit from detachment surgery a success- 1920; 4: 211-6. ful outcome was achieved in only 3 eyes of 3 patients. 9Hovland KR, Schepens CL, Freeman HM. Developmental The major factors contributing to these poor results giant retinal tears associated with lens . Arch of peripheral Ophthalmol 1968; 80: 325-31. were the difficulty in the evaluation °0Foos RY, Kiechler RJ, Allen RA. Congenital nonattach- retinal details owing to opacities in the ocular media ment of the retina. Am J Ophthalmol 1968; 65: 202-10. and the tendency to develop massive preretinal "Rice NSC. Management of infantile glaucoma. Br J retraction. Evidence of the latter was often detected Ophthalmol 1972; 56: 294-8. at the time of presentation, and postoperatively 2Spencer WH, Ferguson WJ, Shaffer RN, Fine M. Late degenerative changes in the following breaks in several eyes proceeded rapidly to an inoperable Descemet's membrane. Trans Am Acad Ophthalmol Oto state. In 5 eyes the only surgical technique con- 1966; 70: 973-83.