To determine if surfactant replacement treatment is associated with an increase in the prevalence of retinopathy of prematurity (ROP) we studied 76 preterm babies who were treated with porcine surfactant (Curosurf) for severe respiratory distress syndrome from 1985 to 1990. Babies were first examined by indirect ophthalmoscopy at the equivalent of 32 weeks post-menstrual age and subsequently at 2-week intervals until discharge from hospital. Findings were documented according to the International Classification of ROP. Sixty-two (82%) babies survived to discharge, 7 survivors were not examined due to transfer elsewhere. Acute ROP developed in 14 (29%) of the 49 babies examined (7 stage I, 4 stage II, 2 stage III, and 1 stage IV); one baby required cryotherapy. No baby of birthweight > 1,500 g developed ROP. The prevalence of ROP was similar to that reported for non-surfactant-treated very-low-birthweight babies. We conclude that Curosurf treatment does not increase the risk of acute ROP in surviving very-low-birthweight babies.
A 10-month-old infant was referred to the ophthalmic department of the Royal Victoria Hospital for assessment. Pregnancy and delivery had been normal. At birth, cleft lip, cleft palate, hypertelorism and a soft tissue mass in the mouth were noted. At two days of age he developed meningitis, axial computerized tomography showed a sphenoethmoidal encephalocele, and agenesis of the corpus callosum. The basal encephalocele was surgically repaired. On examination in the ophthalmic department he was able to follow a light with the left eye. He perceived light with the right eye but did not follow. There was a manifest right divergent squint, measuring -10 degrees, and a right afferent nerve defect. Examination under anaesthetic was performed. The right fundus showed a Morning Glory syndrome. The disc was pink and deeply excavated, and surrounded by a ring of chorioretinal pigmentary disturbance. There was a central tuft of glial tissue. The left fundus was normal. The association of Morning Glory syndrome and basal encephalocele is rare; four previous cases have been reported. In all patients there were associated mid-facial congenital anomalies such as cleft lip and cleft palate. The presence of a basal encephalocele should be suspected if the Morning Glory syndrome occurs in association with mid-facial congenital anomalies.
A baby with unilateral cleft lip, midline cleft palate and hypertelorism developed meningitis in the first 48 h of life. Examination of the nasopharynx showed a soft tissue mass, which was confirmed as a basal encephalocele by computed tomography. There was also congenital hydrocephalus and the corpus callosum was absent. Surgical treatment included repair of the anterior basal skull defect, repair of the lip and palate, and ventriculo-peritoneal shunt. There is currently evidence of developmental delay and right-sided visual impairment due to Morning Glory syndrome. This case demonstrates that basal encephalocele should be considered in any baby with midline facial deformity who develops meningitis.
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