Retrolental fibroplasia and blood transfusion in very low-birth-weight infants.

The relative contribution of transfusions of adult blood to the development of retrolental fibroplasia (RLF) in very low-birth-weight infants was examined. Five years of experience with the expanded use of replacement and exchange transfusions in 90 infants with birth weight less than or equal to 1,250 gm was reviewed. Twenty percent of the infants developed cicatricial RLF. Exchange transfusion was not related to development of cicatricial RLF. The incidence of RLF in infants receiving greater or equal to 130 ml of packed red blood cells per kilogram of birth weight as replacement blood transfusion (RBT) was significantly higher (42.9%) than that in infants receiving 61 to 131 ml of packed red blood cells per kilogram (15.4%) and infants receiving less than or equal to 60 ml of packed red blood cells per kilogram (0%), P less than .001. The need for RBT, however, was strongly correlated (r = .85, P less than .001) with increasing duration of O2 therapy. When O2 therapy was controlled for, the association between RBT and RLF did not achieve statistical significance (P = .07). The association between RBT and RLF remained significant when adjusted for duration of therapy in fractional inspired oxygen (FIO2) greater than 0.4. Further detailed studies of large numbers of susceptible infants are warranted to assess the magnitude of the contribution of transfusions of adult blood to development of RLF.

Intrascleral nerve loop mistakenly identified as a foreign body.

A 5-year-old boy had an excisional biopsy of a pigmented scleral lesion thought clinically to be a foreign body, probably graphite from a pencil. Histological study demonstrated that the pigmented lesion was an intrascleral nerve loop (Axenfeld).

Sequelae of arrested mild retinopathy of prematurity.

Twenty-six infants with totally resolved low-grade retinopathy of prematurity (ROP) were compared with a similar group of 38 premature infants in whom no retinopathy had ever developed in the nursery. At the examination performed at a physiologic age of 1 year, the two groups were almost indistinguishable with respect to their refractive errors, strabismus, and amblyopia. The incidence and severity of mild to moderate anisometropia was increased in the resolved ROP group. It appears that there is a group of infants in whom absolute resolution occurs, with few residua of active ROP. This is especially true when there are no anatomical retinal findings consistent with low-grade cicatricial retrolental fibroplasia at 1 year of age.

Measurement of Goldmann visual fields in older children who received cryotherapy as infants for threshold retinopathy of prematurity.

BACKGROUND: Cryotherapy administered to eyes with severe acute-phase (threshold) retinopathy of prematurity benefits retinal structure and visual acuity compared with the natural course of the retinopathy. OBJECTIVES: To determine the extent of peripheral field abnormalities in eyes with threshold retinopathy of prematurity that had retinal structure preserved by cryotherapy. METHODS: Kinetic perimetry was performed with a Goldmann perimeter by masked testers on patients in whom bilateral threshold retinopathy of prematurity developed and who had been randomly assigned to undergo cryotherapy in one eye and no cryotherapy in the fellow eye. With the V-4-e and the II-4-e targets, eight meridians were tested: 0 degrees, 45 degrees, 90 degrees, 135 degrees, 180 degrees, 225 degrees, 270 degrees, and 315 degrees. The median value of three presentations in each meridian was accepted as the extent in that meridian. RESULTS: Fourteen eyes (eight treated and six control) of eight patients (mean age, 9.9 years; range, 6 to 11 years) had adequate vision to undergo fields testing. Mean (+/-SE) extent of visual field for treated vs control eyes was 36 degrees +/- 3 degrees vs 46 degrees +/- 6 degrees for the II-4-e target and 49 degrees +/- 4 degrees vs 59 degrees +/- 6 degrees for the V-4-e target. This difference was consistent across all eight meridians for either target, and repeated-measures analysis of variance showed that cryotherapy was associated with smaller visual field extent for both target sizes (P=.08). CONCLUSION: The results of this small pilot study suggest that eyes that have retinal structure and acuity preserved by cryotherapy for severe acute-phase retinopathy of prematurity have slightly smaller visual fields than untreated eyes with severe acute-phase retinopathy of prematurity that had vision preserved.

A revised classification of retinopathy of prematurity.

We have developed a classification system for the acute phases of retinopathy of prematurity based on more than 13,000 ophthalmoscopic examinations of more than 3,400 premature infants between 1968 and 1982. Two forms of the active disease exist. Retinopathy of prematurity is a relatively common mild disease and retinopathy of prematurity plus is characterized by rapid progression and posterior pole vascular tortuosity and dilation. The five grades progress from peripheral vascular abnormalities (Grade 1) through a demarcation line (Grade 2) and extraretinal neovascularization (Grade 3) to partial (Grade 4) or total (Grade 5) retinal detachment. The persistence of abnormal retinal vessels during the first year of life is considered "transitional" retinopathy of prematurity unless unequivocal cicatricial changes with macular distortion develop.

Ocular alignment after craniofacial reconstruction.

We undertook a retrospective study of 160 patients to ascertain if craniofacial reconstruction affected ocular alignment. In most patientspre-existing alignment and binocularity were unaffected by craniofacial surgery; thus, correction of any strabismus can be performed on a child without concern for disruption of muscle tissue by later craniofacial surgery.

Variations in extraocular muscle number and structure in craniofacial dysostosis.

Five of 12 patients with craniofacial dysostosis who needed strabismus surgery had anomalies of extraocular muscle structure and number. Two cul-de-sac incisions per eye are sufficient to investigate all muscles for anomalies and add little time or risk to the planned strabismus procedure. The origin of these anomalies and their frequency in craniofacial stenosis are unknown.

Severe traumatic oculo-orbital displacement. Diagnosis and secondary treatment.

We have treated 12 patients with severe oculo-orbital trauma during the past 3 years. The structural problems, produced by disruption or displacement of the orbital cone, were treated effectively (and, on occasion, preferentially) with onlay bone grafts. For an effective correction, we advise radical mobilization of the soft tissue and simultaneous correction on the ocular adnexal deformities. Ocular muscle problems are produced by direct injury to the extraocular muscles, or oculomotor nerve, and were possible these should be corrected early. The structural damage to the eye and orbit falls into certain patterns, related to weak points about the orbit. These have been described.

Extracorporeal membrane oxygenation causing asymmetric vasculopathy in neonatal infants.

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) is a form of cardiopulmonary bypass therapy used in term or near-term infants with severe cardiorespiratory disorders not responsive to conventional intensive care interventions. An ECMO-associated retinal vasculopathy has been described with little reference to the specific condition of the patient. We examined the eyes of 91 infants who underwent ECMO treatment. An assessment was made of the following: (1) when retinal changes occurred, (2) whether there was a particular systemic disorder or ECMO approach associated with these retinal findings, and (3) whether there may be ocular sequelae from this development. METHODS: Ninety-one neonates were treated with ECMO for meconium aspiration syndrome (MAS), primary persistent pulmonary hypertension of the newborn, sepsis, congenital diaphragmatic hernia (CDH), respiratory distress syndrome (RDS), and blood aspiration. Venoarterial bypass was performed in 73 patients. The remaining 18 patients underwent venovenous bypass. Ophthalmologic examinations were performed during bypass in 6 infants and within 3 weeks of ECMO in the remainder. RESULTS: Asymmetric retinopathy (left eye > right eye) was discovered in six infants with CDH and in one infant with RDS within a 2-week period after bypass, demonstrating venous tortuosity with or without intraretinal hemorrhages. One infant treated for MAS had a left eye intraretinal hemorrhage only. All patients with the noted retinal changes underwent venoarterial cannulation. Six of 9 patients with CDH had retinal findings noted compared with 1 of 10 patients with RDS and 1 of 35 patients with MAS. CONCLUSION: Because we were able to examine infants while they were receiving ECMO or shortly after termination of bypass, asymmetric vasculopathy was found in a greater percentage of our patients compared with a similar large case series. ECMO-associated retinal vasculopathy appeared to disproportionately occur in those patients with CDH who underwent venoarterial bypass. Further study of retinal vascular changes in patients with CDH should be performed to assess long-term effects.

Comitant esodeviation resulting from neurologic insult in children.

PURPOSE: Our purpose was to establish whether comitance was a common or uncommon finding in children with esodeviation associated with a neurologic insult. METHODS: A retrospective chart review was performed of children with acquired esodeviation associated with an identifiable neurologic insult. RESULTS: Examinations of 30 children seen over a 2-year period were analyzed. Twenty-two (73%) had brain tumors. Twelve (40%) had comitant esodeviation, and the other 18 (60%) had incomitant measurements. Of the patients with comitant esodeviation, 6 had normal abduction OU (two after recovery from bilateral sixth nerve palsies), and 6 had mild or minimal abduction deficits. Moderate or severe abduction deficits were associated with incomitant measurements. CONCLUSIONS: Comitant esodeviation can be common in children with identifiable neurologic insults.

Outcome-based management of retinopathy of prematurity. Multicenter Trial of Cryotherapy for Retinopathy of prematurity Cooperative Group.

PURPOSE: A system is presented for sequentially computing the risk of progression of retinopathy of prematurity (ROP) for infants born weighing not more than 1250 gm. A personal computer program is used to monitor infants' risk of threshold ROP from first appearance of ROP, and the progression in severity is tracked with multiple logistic risk models developed from data in the Multicenter Trial of Cryotherapy for Retinopathy of Prematurity. METHODS: After entry of the infant's birth weight, gestational age, ethnicity, birth in the current hospital or elsewhere, single or multiple birth, and maturity zone of retinal vessels, risk of progression to threshold severity is calculated. New estimates of risk are computed at onset of ROP and prethreshold ROP (any zone I ROP, zone II stage 2+ or 3) according to the extent of retinal vascularization when ROP first appears, how rapidly ROP progresses, and how severe it is. When threshold ROP (8 total or 5 contiguous clock hours of stage 3+ in zone I or II) is reached,the system provides separate estimates of risk that the eye will have an unfavorable 3-month outcome if treated or not. RESULTS: Estimates of risk of progression to threshold disease among the 4099 patients in the Multicenter Trial of Cryotherapy for Retinopathy of Prematurity natural history study varied from less than 1% to more than 70%. For eyes with threshold disease, the risk of an unfavorable outcome at 3 months without treatment varied from less than 10% to more than 90%. CONCLUSION: This method of tracking identifies infants at high risk for severe ROP and poor structural outcome. It provides information about prognosis with a specificity heretofore impossible.

The ophthalmologic manifestations of the cardio-facio-cutaneous syndrome.

The cardio-facio-cutaneous (CFC) syndrome is an uncommon multiple congenital anomalies/mental retardation syndrome whose major manifestations are congenital heart defects, relative macrocephaly, stunted growth, ectodermal dysplasia, characteristic facial appearance, and psychomotor developmental delay. All described cases were sporadic and cytogenetically normal. We report three additional patients with this diagnosis. All three patients developed strabismus, requiring extraocular muscle surgery. Two of our patients also had nystagmus. Combining the 18 previously reported cases with our additional 3, 9 of 21 had strabismus, 10 of 21 had ptosis, and 6 of 21 had nystagmus. Ophthalmic craniofacial abnormalities of hypoplastic supraorbital ridges, prominent epicanthal folds, and antimongoloid slant of the palpebral fissures were also common features contributing to the characteristic facies described for this syndrome. The ophthalmologist could aid in the diagnosis and treatment of these patients because of the prominent ophthalmologic symptomatology of the CFC syndrome.

Infantile glaucoma associated with the Diamond-Blackfan syndrome.

Diamond-Blackfan syndrome is a rare congenital hematologic disorder characterized by isolated erythroid hypoplasia. Physical abnormalities such as short stature, thumb deformities, and ophthalmic findings including strabismus, hypertelorism, and microphthalmos have been reported to occur with this disease entity. We describe two patients with this blood dyscrasia and infantile glaucoma with trabeculodysgenesis. Both patients underwent multiple surgical procedures to control their glaucoma. The patients were on chronic systemic adrenal corticosteroid therapy for their anemia, which caused some confusion regarding the etiology of their glaucoma. To our knowledge, this is the first report of an association of infantile glaucoma with Diamond-Blackfan syndrome. We hope this report will encourage early recognition and treatment of glaucoma in patients with this disorder.

Kansas Regional Medical Program Library Services.

The Kansas Regional Medical Program Office for Library Services was developed to link the medical library resources and to make them available to health-related personnel throughout Kansas. Library offices have been established at the Central Kansas Medical Center, Great Bend, Stormont Medical Library, Topeka, and at the Wichita State University, Wichita. The main office, located at the Clendening Medical Library, University of Kansas Medical Center, Kansas City, provides medical library services to those three offices, the other medical libraries in Kansas, and to the health professionals in the state who do not have medical library resources available. Reference requests are accepted via a twenty-four-hour telephone service from medical librarians and from individual health professionals.

Oculocutaneous albinism associated with congenital glaucoma.

An infant with oculocutaneous albinism and congenital glaucoma is described. The association of these two uncommon congenital disorders might suggest a common defective factor. Theoretically this patient broadens the spectrum of disorders of neural crest cell migration associated with albinism.