Ilana Nissenkorn

Long-term Results of Cryotherapy for Active Stages of Retinopathy of Prematurity

Between 1976 and 1980, retinopathy of prematurity (ROP) stage III (moderate) (fibrovascular proliferation) developed in 29 eyes (confluent fibrovascular proliferation of 3 clock hours in extension) of 17 preterm babies. The eyes were treated by cryopexy to the avascular retina. Analysis of the visual function and anatomic results in these eyes, five to eight years postoperatively, revealed very good visual function and only a few anatomic abnormalities. Comparison of these eyes with those of two groups of age- and birth-weight-matched preterms (one with less severe ROP, not treated by cryopexy, and the other with no ROP) showed that the degree of myopia was significantly higher in the cryotreated group. Although not statistically significant, there was a higher incidence and a greater degree of astigmatism in this study group.

A clinicopathological case of retinopathy of prematurity (ROP) treated by peripheral cryopexy.

A baby born at 25 weeks gestation with a birth weight of 800 g who suffered from severe respiratory distress syndrome with metabolic acidosis is described. Active retinopathy of prematurity, stages II and III, was found in the right eye at the age of 1 month and in the left eye a month later. Both eyes were treated by peripheral cryopexy. The pathological examination showed that cryopexy was effective in preventing progression of the retinopathy to cause severe cicatrisation.

Congenital Orbital Vascular Malformation Complicated by Massive Retrobulbar Hemorrhage

A 2-year-old female infant with a presumed diagnosis of infantile hemangioma of the left eyebrow developed acute proptosis of the left eye several days after a mild orbital trauma. Computerized axial tomography (CAT) and carotid angiography confirmed the presence of a vascularized mass in the retrobulbar space, spreading anteriorly to the orbital rim and the frontal area. Due to progression of the proptosis and the development of exposure keratitis, surgical intervention was necessary. A large vascular tumor and a massive hemorrhage occupying the orbit were removed. The pathological examination confirmed the diagnosis of a vascular malformation of the orbit, most probably orbital varices. A relatively good visual acuity was achieved after surgery by occlusion therapy.

Effect of partial cryoablation on retinopathy of prematurity.

Cryotherapy has been shown to be an effective treatment for retinopathy of prematurity stage 3. It is said to reduce the unfavourable outcome of the disease by 50%. The accepted method of therapy includes ablation of the whole avascular retina from the ridge to the ora serrata. This is achieved by two to three rows of cryoapplications. When 360 degrees cryo treatment is performed, it requires 52 cryo applications. We present our method of cryotherapy which involves only one row of cryoapplications in the avascular retina anterior to the fibrovascular ridge. An average of 25-30 cryoapplications was required for a 360 degrees treatment. The anatomical results in 23 babies are presented. Complete regression of active retinopathy was found in all.

Muscle involvement in mucolipidosis IV

A 15-month-old boy, thought to have a congenital myopathy, was subsequently diagnosed as having mucolipidosis type IV, with typical membranous inclusions in muscle fibers. Involvement of skeletal muscle in this lysosomal storage disease may explain the motor delay and hypotonia that are its most common presenting signs.

Nasal versus temporal preretinal vasoproliferation in retinopathy of prematurity.

Nasal preretinal neovascularisations have been observed to develop at least two weeks earlier than those on the temporal side in more than a third of premature babies with retinopathy of prematurity (ROP) stage 3, weighing between 690 and 1030 g. All these babies were assigned for cryotherapy. This interesting observation is discussed in relation to retinal vascular development and the pathogenesis of ROP.

Aicardi Syndrome: A Cause of Infantile Seizures

Received for publication June 1983, revised September 1983, and accepted December 1983. IN 1965, AICARDI described a syndrome consisting of infantile spasms, chorioretinopathy, agenesis of corpus callosum, costovertebral anomalies, and mental subnormality.’ By 1979, about 60 cases had been reported in the medical literature. 1.3 All the described patients were females. In this report, we wish to highlight some of the problems raised in the differential diagnosis and to stress the unusual mode of inheritance of this syndrome.

Retinopathy of Prematurity: Incidence and Risk Factors

A high incidence of retinopathy of prematurity (ROP) was found in the very low-birth-weight infants discharged from the neonatal intensive care unit during the years 1977 to 1980, in spite of frequent monitoring of oxygen use. Although the yearly incidence of ROP in infants weighing less than 1,500 g varied between 35% to 36%, none were blind. The medical records of 65 infants with a birth weight between 501 and 1,250 g; surviving in 1979 to 1980, were reviewed in order to find risk factors for ROP. There were no significant differences between the 34 infants with ROP and the 31 infants who did not have ROP in mean birth weight or mean gestational age. Of 32 possible risk factors examined, the factors significantly associated with ROP were: apnea with mask and bag ventilation; prolonged parenteral nutrition; number of blood transfusions; and episodes of hypoxemia, hypercarbia, and hypocarbia. A highly significant association between hypocarbia and the development of severe ROP was found.

Gestational age assessment by the lens examination method

ical effects. Within 2 months she was able to walk alone, became more alert and began to say a few words. Af te r 8 months on a gluten-free diet she showed a weight gain of 1800 g, a height increase of 6 cm and the mucosa of the small intestine was normal on biopsy. She was challenged with a normal gluten-containing diet. A further biopsy after 1 year on a gluten-containing diet showing a definite mucosal relapsed. Thus, the diagnosis of coeliac disease was fully established according to current criteria formulated by the European Society for Paediatric Gastroenterology and Nutri t ion [3], and a gluten-free diet was reinstituted. Subsequent biopsies on a gluten-free diet have shown a normal mucosa. The patient s mother , father and four older siblings had a normal small intestine mucosa on biopsy. To our knowledge this is the first report of coeliac disease in a child with FAS. With this letter we want to stress the possibility of coexisting coeliac disease in patients with FAS who show abnormally slow growth. However , only further studies will reveal if the concurrence of the two disorders is a syntropy or sheer coincidence. 75