Congenital Glaucoma in Cutis marmorata teleangiectatica congenita

Abstract

A case of congenital glaucoma in cutis marmorata teleangiectatica congenita (CMTC, van Lohuizen syndrome) is described. The cutaneous anomaly and heterochromia iridium were noticed at birth. Brown discoloration of one iris was due to iris anterior layer dysplasia, resulting in unilateral glaucoma. Two trabeculotomies were performed until persistent normalization of intraocular pressure could be achieved. The possibility of a genetic basis and hereditary condition of CMTC and its association with congenital glaucoma is discussed. Patients with CMTC should regularly undergo ophthalmological follow-up to rule out development of glaucoma.

Cutis Marmorata Telangiectatica Congenita in a Preterm Female Newborn: Case Report and Review of the Literature

“…29,30 Most frequent disorder is congenital glaucoma: up to 2007, 15 cases have been published. 10,[30][31][32][33][34][35][36][37][38] Petrozzi and Rahn first described congenital glaucoma in a CMTC patient with nevus flammeus overlying the involved eye and mental retardation. 18 In 1989 Picascia described two patients (of 22 with CMTC) with ocular anomalies: left cataract and glaucoma in a three month old infant and another patient with bilateral glaucoma and nevus flammeus involving the whole face.…”

“…19 Also in other cases, congenital glaucoma was detected in patients with vascular anomalies in the periocular skin, indeed it seems to affect the area of distribution of the ophthalmic branch of trigeminal nerve. 18,19,31,33,34,36,37 Congenital glaucoma is usually unilateral: on 15 reported cases 11 had unilateral forms, 2 bilateral ones and in the remaining 2 cases laterality was unspecified. The time of diagnosis is normally soon after birth or in infancy, though Murphy discovered a congenital glaucoma in a nine years old patient with CMTC during his follow-up.…”

Late-onset pediatric glaucoma associated with cutis marmorata telangiectatica congenita managed with Molteno implant surgery: Case report and review of the literature

“…[1][2][3][4][5][6][7][8][9] In 13 of these cases the diagnosis of glaucoma was made soon after birth and in the remaining two patients it was discovered at the first ophthalmic examination at 3 and 4 years of age. The glaucoma was unilateral in 11 of the patients and bilateral in 2 patients; in the remaining two cases laterality was unspecified.…”

“…1,[2][3][4]6,7,9 Trabeculotomy was carried out in 6 of these and was associated with a high rate of failure; 4 of the eyes went on to have trabeculectomy, 2 required repeat trabeculotomy, with a suprachoroidal hemorrhage occurring in one of those 2 cases. In one case, where no drainage angle anomaly had been detected, trabeculectomy successfully controlled the intraocular pressure.…”

Developmental and childhood glaucoma

“…221 It has also been associated with infantile trabeculodysgenic glaucoma [222][223][224][225] and, in one report, with intraoperative suprachoroidal hemorrhage. 194…”