Improving eye care follow-up adherence in diabetic patients with ocular abnormalities: the effectiveness of patient contracts in a free, pharmacy-based eye screening.

OBJECTIVES: Patient contracts are increasingly utilized in medical practice and have the potential to improve health outcomes in high-risk populations. However, as a relatively new tool, there has been limited research regarding the efficacy of patient contracts. Diabetic retinopathy (DR) is one of the leading causes of vision impairment in adults in the US and only 50-60% of adults with diabetes adhere to annual dilated fundus exam recommendations. This study aimed to evaluate the impact of patient contracts on follow-up adherence in diabetic patients with ocular abnormalities after a free, pharmacy-based eye screening. STUDY DESIGN: This prospective study implemented a non-invasive, non-mydriatic fundus camera in an urban, community-based pharmacy setting to screen for ocular diseases in patients with diabetes. Patients were assigned to the contract or non-contract group. Patients who signed a contract agreed to: 1) review their results with their primary care doctor, 2) follow-up with an ophthalmologist if their results were abnormal, and 3) inform research staff if/when they completed an eye care appointment. All study participants and their primary care doctors were notified of their results via mail. Follow-up questionnaires were administered to all patients by telephone three months after the screening results. RESULTS: 500 patients were screened and 113 (22.6%) had abnormal results. Of the patients who had abnormal results, 83 (74.3%) were able to be contacted. Of the 83 patients who were able to be contacted, the majority of patients were African American (73.5%) and female (56.6%). The mean age was 54.7 years. Of those, 34 (41.0%) adhered to follow-up recommendations. There was no significant difference in follow-up adherence between the contract (38.1%) and non-contract group (43.9%) (P = 0.59). In addition, 70.4% of patients did not comply with at least one measure of the contract agreement. CONCLUSION: Contracts did not increase follow-up adherence to eye appointments in diabetic patients with ocular abnormalities. The majority of patients did not comply with their contract and follow-up adherence was low in both groups. Most research has yielded mixed results regarding the efficacy of contracts in improving health outcomes. Therefore, different types of contracts or other patient-centered tools should be evaluated in order to increase follow-up adherence in patients at high risk for DR.

Clinical protocol. An open-label, phase I, single administration, dose-escalation study of ADGVPEDF.11D (ADPEDF) in neovascular age-related macular degeneration (AMD).

Age-Related Macular Degeneration (AMD) is, together with Diabetic Retinopathy, the most common cause of vision loss among adults in the U.S. and other developed countries. In the U.S., at least 1.7 million people have impaired vision due to AMD. Every year, more than 165,000 people contract AMD and 16,000 go blind from it, predominantly from a rapidly progressing form of the disease called "wet" AMD. Wet AMD is characterized by serious or hemorrhagic detachment of the retinal pigment epithelium and choroidal neovascularization. The macula has the highest concentration of photoreceptors facilitating central vision and permitting high-resolution visual acuity. The damage caused by the leakage and fibrovascular scarring in wet AMD leads to profound loss of central vision and severe loss of visual acuity (usually 20/200 or worse). People with wet AMD have several limitations, including inability to read, inability to recognize faces or drive, and the disease often leads to blindness. The onset of severe visual changes in wet AMD can occur suddenly. More than 400,000 Americans are currently affected by this form of the disease, and the incidence is rising rapidly with the aging of the population. Therefore, the serious consequences of this disease along with the limited treatment options and their effectiveness make this a very good candidate for a gene transfer treatment approach. The investigational agent, Ad(GV)PEDF.11D, is an E1-, partial E3-, E4- deleted replication-deficient, adenovirus serotype 5, gene transfer vector. The transgene in this vector is the cDNA for human pigment epithelium-derived factor (PEDF). PEDF is one of the most potent known antiangiogenic proteins found in humans. While Ad(GV)PEDF.11D is able to transduce many somatic cell types, the natural barrier to other tissues created by the retina limits the ability of Ad(GV)PEDF.11D to affect tissues other than in the eye. Intravitreal administration of Ad(GV)PEDF.11D provides a convenient means of delivering PEDF to the relevant cells within the eye likely to result in a more prolonged duration of effect versus administration of the PEDF protein alone. In three murine disease models (the laser-induced choroidal neovascularization model, the VEGF transgenic model, and the retinopathy of prematurity model) significant inhibition of neovascularization (up to 85%) was demonstrated with doses of Ad(GV)PEDF vectors ranging from 1 x 10(8) to 1 x 10(9) pu. In toxicology studies performed in Cynomolgus monkeys, a dose-related inflammatory response was observed. A dose of 1 x 10(8) pu caused no adverse effects, while the inflammatory response observed at 1 x 10(9) pu was minimal and fully reversible. The observed inflammatory response at doses of 1 x 10(10) and 5 x 10(10) pu were increasingly severe. The proposed clinical trial is an open-label, dose-escalation, phase I study to investigate the safety, tolerability and potential activity of intravitreal injection of Ad(GV)PEDF.11D in patients with wet AMD. Ad(GV)PEDF.11D will be injected once via intravitreal injection into the eye with the most advanced AMD based on visual acuity. Subjects will be age 50 or over and have severe wet AMD in at least one eye defined by a best-corrected vision of 20/200 or worse. The primary objectives of this investigation are: (1) to assess the safety, tolerability and feasibility of intravitreal injection of Ad(GV)PEDF.11D in patients with severe, neovascular AMD, (2) to identify the maximum tolerated dose (MTD) of Ad(GV)PEDF.11D, and (3) to get some indication of potential activity of Ad(GV)PEDF.11D.

Custom-made tracheostomy tube to relieve lower tracheal obstruction in infants and young children.

Two case histories are presented in which special difficulties were encountered in the management of lower tracheal obstruction in children. These problems were successfully overcome by the use of a tracheostomy tube which was custom-made from a standard thin wall polyvinyl chloride endotracheal tube sufficiently long to relieve this obstruction.

Hospitalizations for traumatic injuries among children in Maryland: trends in incidence and severity: 1979 through 1988.

Trends in incidence and severity of hospitalized injury among children aged 0 through 13 years in the state of Maryland from 1979 through 1988 (n = 35,746) were examined using routinely reported hospital discharge data. Hospital discharge rates declined over the study period from 509 per 100,000 population in 1979 to 320 in 1988. There was a decline in incidence trends for both races. However, the decrease in the nonwhite population was smaller than in whites. Analysis of incidence rates for specific Injury Severity Score groups revealed a declining trend in all Injury Severity Score groups, although the mildest group (Injury Severity Score 1 through 4) had the most notable decline of 44% compared with an average decline of 20% in the other severity groups. These data suggest a change in admission practices of mildly injured children as a major cause for the observed overall decline in hospitalization rates. The smaller decrease in the hospitalization rates of non-white children compared with white children requires further study to determine the cause.

Long-term outcome of children with severe head trauma and prolonged coma.

Forty-six children with significant head trauma who remained in coma more than 24 hours were the subjects of this long-term outcome study. Twelve (38%) died. The average length of coma in the 34 survivors was 15.5 days. Follow-up ranged from 9 months to 4 years (mean 21 months). Twenty-nine percent of the survivors were normal at follow-up. An additional 53% had mild cognitive or behavioral problems, but 61% of these had evidence of similar problems prior to the injury. Nine percent of survivors had motor residua but normal intellect. Nine percent had severe intellectual and motor problems. Children less than 2 years of age had a worse outcome. Despite severe injury and prolonged coma after head trauma, most children do well. Intensive medical and surgical care increases the rate of survival and does not result in a large number of severely disabled survivors.

The natural history of bronchial atresia. Serial observations of a case from birth to operative correction.

A 10-year-old child presented with a history of intermittent respiratory symptoms since birth. Progressive shift of the mediastinum away from a hyperexpanded left upper lobe to the right side was evident on serial chest films, since birth. Bronchograms demonstrated atresia of the bronchus to the hyperexpanded segment. Xenon washout demonstrated prolonged half-time in the left upper lobe resulting from collateral ventilatory channels. Resection of the apical posterior segment of the left upper lobe was performed without complication. Bronchial atresia with collateral ventilation caused "lobar emphysema" in this patient.

Central serous chorioretinopathy and pregnancy.

BACKGROUND AND OBJECTIVE: Fourteen cases of central serous chorioretinopathy in pregnancy had been reported before this study was conducted. These cases have suggested a nonwhite predominance. Subretinal fibrinous exudates have been seen in 90% of the patients, compared with fewer than 20% of patients in typical (nonpregnant) central serous chorioretinopathy. No case has recurred outside of pregnancy, to our knowledge, and there have been no reports of subsequent pregnancies uninvolved by this disorder. These findings led us to collect our cases of central serous retinopathy in pregnancy because our experience differed from that of previous reports and provides additional new information. DESIGN: Case series. SETTING: The Wilmer Institute Retinal Vascular Center, Baltimore, Md. PATIENTS: Questionnaires sent to retinal faculty and fellows and a review of files revealed four patients, all included herein, with central serous chorioretinopathy presenting during pregnancy. RESULTS: All four patients were white. Three patients had subretinal fibrinous exudates and/or precipitates. All experienced resolution of the serous detachment near the end of the pregnancy or within the first few months after delivery. Only one patient had a subsequent pregnancy, and this was not complicated by the presence of central serous chorioretinopathy. One other patient experienced a recurrence 2 1/2 years after her last pregnancy. CONCLUSIONS: There may be no racial predominance in the development of central serous chorioretinopathy in pregnancy. Subretinal fibrinous exudates are quite common, independent of race. The uninvolved subsequent pregnancy suggests that recurrence in the context of pregnancy is not inevitable. This disorder may recur outside of pregnancy.

Visual field loss following vitreous surgery.

OBJECTIVE: To assess possible causes of visual field loss following vitreous surgery. DESIGN: Charts of 8 patients prospectively identified, who developed visual field loss following vitreous surgery, were reviewed to characterize this newly recognized syndrome and assess possible causes. RESULTS: Two patients had preexisting chronic open-angle glaucoma and 1 had ocular hypertension. Indications for surgery included 4 eyes with macular holes, 1 eye with epiretinal membrane, 2 eyes with rhegmatogenous retinal detachment, and 1 eye with retinal detachment and giant retinal tear. All patients received retrobulbar anesthesia. Seven of 8 patients had fluid/gas exchange with installation of long-acting bubbles. In 1 patient with a macular hole, a small hemorrhage was noted along a vessel coming off the nerve superotemporally while attempting to engage the posterior cortical vitreous intraoperatively. This patient developed an inferior visual field defect. No intraocular pressure (IOP) measurements greater than 26 mm Hg were recorded in any eye perioperatively. Visual field defects included 4 eyes with inferotemporal defects, 2 eyes with inferior altitudinal defects, 1 eye with a cecocentral scotoma, and 1 eye with a superonasal defect. Only 1 patient had worsened visual acuity. A relative afferent pupillary defect was observed in 4 eyes and disc pallor in 5 eyes. CONCLUSIONS: Central or peripheral visual field loss can now be recognized as a possible complication of vitreous surgery. In some cases, a relative afferent pupillary defect and optic disc pallor are present, suggesting that the optic nerve is the site of injury. Possible mechanisms include ischemia due to elevated IOP or fluctuations in IOP, optic nerve damage from retrobulbar injection, direct intraoperative mechanical trauma to the optic nerve, indirect injury from vigorous suction near the optic nerve leading to shearing of peripapillary axons or vessels, or a combination of these. Certain optic nerves may be more susceptible to injury because of preexisting compromise from glaucoma or vascular disease.

Pilot trial of transscleral diode laser retinopexy in retinal detachment surgery.

OBJECTIVE: To evaluate the safety and efficacy of the transscleral diode laser for retinopexy in rhegmatogenous retinal detachments. DESIGN: Ten consecutive patients with primary rhegmatogenous retinal detachments underwent scleral buckling surgery, using the transscleral diode laser for retinopexy. Patients were followed up for at least 6 months. STUDY PARTICIPANTS: Ten patients with primary rhegmatogenous retinal detachments were enrolled. Patients with chronic detachments, a retinal break greater than 90 degrees, history of uveitis or infectious retinopathy, or proliferative vitreoretinopathy were excluded. MAIN OUTCOME MEASURE: Retinal reattachment. Secondary measures: visual acuity and complications, including choroidal, retinal, and vitreous hemorrhage, inflammation, and scleral damage. RESULTS: By 6 months, nine of 10 retinas were successfully repaired following only one operation. The retina of the 10th patient redetached at 6 1/2 weeks owing to proliferative vitreoretinopathy. Following a vitrectomy and retinal reattachment procedure, the retina of the 10th patient has remained attached for 1 year. No significant complications were encountered with the laser retinopexy, although small presumed breaks in Bruch's membrane occurred in three eyes and a moderate scleral thermal effect occurred in two. Visual results in the postoperative course were comparable to those achieved previously with cryotherapy. CONCLUSION: In this pilot series, transscleral diode laser retinopexy served as a safe and effective means of obtaining chorioretinal adhesion in retinal detachment surgery.

A new syndrome of myelinated nerve fibers, vitreoretinopathy, and skeletal malformations.

OBJECTIVE: To report a new vitreoretinopathy with high myopia, myelinated nerve fibers, and skeletal abnormalities. PATIENTS: A mother and daughter with a previously undescribed constellation of severe vitreoretinal degeneration with high myopia and extensive myelination of the retinal nerve fiber layer, and in addition, the daughter has limb reduction deformities. Vision was severely impaired in both patients and results of electroretinography indicated the presence of cone and rod dysfunction. CONCLUSION: This is a previously undescribed autosomal dominant vitreoretinopathy with congenital poor vision, vitreoretinal degeneration, myelinated nerve fibers, and skeletal deformities.

Histopathological features of vitreous removed at macular hole surgery.

OBJECTIVE: To describe the histopathological features of the vitreous removed at surgery for macular holes in 200 consecutive cases. METHODS: The complete vitrectomy specimen in each case was concentrated by means of cellulose membrane filters and stained for light microscopy. The cases were organized into 5 categories: (1) all cases (N = 200), (2) eyes without previous vitrectomy (n = 174), (3) eyes with previous vitrectomy (n = 26), (4) idiopathic cases (n = 143), and (5) traumatic (accidental or surgical) cases (n = 31). The type and frequency of tissue fragments present in the vitreous were determined for each case. RESULTS: Fibrocellular and cellular membrane fragments were found in a minority of cases in all categories. Retinal fragments were a rare finding, present in only 4 cases. Inflammation was present in 57 (28.5%) of all cases. CONCLUSIONS: The absence of fibrocellular and cellular membrane fragments in the majority of cases suggests that mechanisms other than cellular proliferation are important in the pathogenesis of macular holes. These fragments are, however, the likely histopathological correlate of the opercula that are often observed clinically in patients with macular hole. Opercula rarely if ever contain retinal fragments, and thus are better termed pseudo-opercula, as has been previously suggested. The cellular proliferation and inflammation that are observed in some of the cases are likely a secondary or reactive process.

Dietary fat and risk for advanced age-related macular degeneration.

OBJECTIVE: To evaluate the relationship between intake of total and specific types of fat and risk for advanced age-related macular degeneration (AMD), the leading cause of irreversible blindness in adults. DESIGN: A multicenter eye disease case-control study. SETTING: Five US clinical ophthalmology centers. PATIENTS: Case subjects included 349 individuals (age range, 55-80 years) with the advanced, neovascular stage of AMD diagnosed within 1 year of their enrollment into the study who resided near a participating clinical center. Control subjects included 504 individuals without AMD but with other ocular diseases. Controls were from the same geographic areas as cases and were frequency-matched to cases by age and sex. MAIN OUTCOME MEASURES: Relative risk for AMD according to level of fat intake, controlling for cigarette smoking and other risk factors. RESULTS: Higher vegetable fat consumption was associated with an elevated risk for AMD. After adjusting for age, sex, education, cigarette smoking, and other risk factors, the odds ratio (OR) was 2.22 (95% confidence interval [CI], 1.32-3.74) for persons in the highest vs those in the lowest quintiles of intake (P for trend,.007). The risk for AMD was also significantly elevated for the highest vs lowest quintiles of intake of monounsaturated (OR, 1.71) and polyunsaturated (OR, 1.86) fats (Ps for trend,.03 and.03, respectively). Higher consumption of linoleic acid was also associated with a higher risk for AMD (P for trend,.02). Higher intake of omega-3 fatty acids was associated with a lower risk for AMD among individuals consuming diets low in linoleic acid, an omega-6 fatty acid (P for trend,.05; P for continuous variable,.03). Similarly, higher frequency of fish intake tended to reduce risk for AMD when the diet was low in linoleic acid (P for trend,.05). Conversely, neither omega-3 fatty acids nor fish intake were related to risk for AMD among people with high levels of linoleic acid intake. CONCLUSION: Higher intake of specific types of fat--including vegetable, monounsaturated, and polyunsaturated fats and linoleic acid--rather than total fat intake may be associated with a greater risk for advanced AMD. Diets high in omega-3 fatty acids and fish were inversely associated with risk for AMD when intake of linoleic acid was low.

The eye in bone marrow transplantation. VI. Retinal complications.

OBJECTIVE: To evaluate the posterior segment ocular complications of patients undergoing bone marrow transplantation (BMT). DESIGN: Retrospective analysis. SETTING: Academic ophthalmology department at a tertiary care hospital with a BMT unit. PATIENTS: Patients undergoing BMT were seen by an ophthalmologist for clinical care and enrolled in a long-term follow-up study, during which they were seen 6 and 12 months after the transplantation and annually thereafter. RESULTS: Of 397 patients undergoing BMT, 51 (12.8%) developed posterior segment complications. Fourteen patients (3.5%) developed hemorrhagic complications with either intraretinal and/or vitreous hemorrhages and 17 patients (4.3%) developed cotton-wool spots in the fundus of both eyes. Eleven patients (2.8%) had bilateral optic disc edema, with eight cases attributed to the toxic effects of cyclosporine and three to other causes. Two patients (0.5%) developed serious retinal detachments. Eight patients (2.0%) developed infectious retinitis and/or endophthalmitis. Fungal infections with Candida or Aspergillus usually occurred within 120 days after BMT, while viral infections with herpes zoster or cytomegalovirus and parasitic infections with Toxoplasma occurred later. Intraocular lymphoma occurred in one patient (0.2%). CONCLUSION: Severe, potentially vision-threatening, posterior segment complications following BMT occur due to a variety of causes.

Clinical course of macular holes: the Eye Disease Case-Control Study.

OBJECTIVE: To describe the clinical course of affected and unaffected eyes in patients with idiopathic macular holes. PATIENTS: Prospective study of patients with macular holes enrolled in the Eye Disease Case-Control Study. MAIN OUTCOME MEASURES: The best-corrected visual acuity at follow-up was compared with that at baseline. Changes in the macular holes, including increases in size or spontaneous regression, were assessed. The rates of development of new macular holes in fellow unaffected eyes were estimated. RESULTS: Of the 198 patients examined at baseline, 28 (14.1%) died before reevaluation. Of those who survived, 122 (71.8%) had a follow-up examination. Approximately 34% (34.4%) of all eyes with macular holes had an increase in the size of the macular hole. Forty-five percent of eyes had a decrease in visual acuity of 2 or more lines and 27.8%, of 3 or more lines; 40.9% remained stable, with a gain or loss of fewer than 2 lines. The rate of development of a new macular hole during follow-up in fellow eyes that were unaffected at baseline was 4.3% for 3 or fewer years of follow-up, 6.5% for 4 to 5 years of follow-up, and 7.1% for 6 or more years of follow-up. Spontaneous regression of the macular hole occurred in 3 (8.6%) of 35 patients with a follow-up interval of 6 or more years, whereas no regression occurred in patients with a shorter follow-up. CONCLUSIONS: The visual acuity of 45.0% of eyes with macular holes deteriorated by 2 or more lines during follow-up. The rate of development of macular holes in unaffected fellow eyes was low.

Pediatric lipoblastomatosis: two unusual cases.

Lipoblastomatosis is a rare, fatty tumor of childhood that is usually located in the limbs. Two pediatric patients were seen with tumors that originated in the mediastinum and retroperitoneal area. The mediastinal tumor was the infiltrating form of lipoblastomatosis while the retroperitoneal tumor was the noninfiltrating type. Neither form is malignant but local recurrences are common. Both were surgically excised without sacrifice of important contiguous structures that were surrounded by tumor. There has been no recurrence 21/2 and 6 years after operation. Conservative excision of this malignant-appearing but histologically benign tumor is emphasized.

A model: systems management of life threatening injuries in children for the state of Maryland, USA.

In the past two years several guidelines for suggested components of emergency medical systems for children have been suggested, and for the first time, specific standards of pediatric trauma care have also been formulated. The American Academy of Pediatrics new Provisional Committee on Emergency Medicine has been charged with the responsibility of developing national standards of emergency care for children and are currently at work on such a landmark document. We have had a functioning statewide system for the management of life threatening injuries in children for the past 10 years. In 1985, 320 children were admitted to our trauma center. The evolution, organization, and current status of the Maryland system is described in this report. Hopefully it may serve as one successful model which could be modified for use in other regions of the country.

Tracheostomy in neonates and small infants: problems and pitfalls.

Newborn babies and small infants who require tracheostomy often follow a complicated clinical course characterized by frequent sepsis, altered ventilatory dynamics, and eventual respiratory decompensation. Many of these problems are avoidable with use of a properly placed endotracheal tube during tracheostomy, a special surgical technique in the performance of the tracheostomy, and silicone rubber tracheostomy tubes. Seventy-four babies underwent tracheostomy at our Children's Center between 1963 and 1976. A review of this experience demonstrates the benefits of unhurried, standardized technique and management. In the 48 newborns requiring tracheostomy, no technical difficulties were encountered, and no complications occurred as a result of the tracheostomy. In the group of 26 older infants, however, there were significant complications, especially in children undergoing emergency tracheostomy without a previously placed endotracheal tube. Although there were no deaths directly related to tracheostomy, one case of pirulent tracheitis and one case of interstitial thyroid hemorrhage were noted at autopsy. Among survivors, there was one case of bilateral pneumothoraces, two cases of severe subcutaneous emphysema, and three cases of postoperative bleeding. Review of the long-term complications in this series demonstrates the benefits of the silicone rubber polymer tube. Since its routine use, problems with stomal granulation have almost disappeared. There have been no problems in extubating the very young babies. Our operative technique and intensive care management of these babies will be emphasized as the keys to the improved outcome.

Endorectal pull-through operation in adults after colectomy and excision of rectal mucosa.

Ileal endorectal pull-through can be performed in adults after abdominal colectomy and excision of rectal mucosa. The procedure requires a painstaking dissection of the mucous membrane of the rectum to ensure cure of the polyposis or ulcerative colitis for which it is carried out. Some cases of ulcerative colitis are inappropriate for this dissection, and Crohn's disease prohibits the use of this operation. Fifteen operations in adults are described herein. Certain technical maneuvers have been of help to us and these are described. We have favored a diverting ileostomy maintained for 3 months. A pouch, positioned in the rectum, has hastened the return toward normal control. Sphincter control and responses have been uniformly good. Dilatations and sphincter exercises are routinely employed. Strictures are not rare but are amenable to correction by digital stretching.

Vulvar congenital dysplastic angiopathy.

Congenital dysplastic angiopathy is a syndrome consisting of vascular angiomata, congenital varicosities, and trophic changes of the soft tissue and the skeleton. Frequently referred to as Klippel-Trenaunay or Klippel-Trenaunay-Weber syndrome, it rarely affects the female genitalia. An 18-year-old woman underwent evaluation and treatment for Klippel-Trenaunay syndrome with vulvar involvement. Preoperative evaluation included consultation with pediatric surgeons, gynecologic surgeons, and an interventional radiologist as well as individual and family psychological counseling. Attention to intraoperative detail resulted in minimal operative blood loss and preservation of normal anatomy. A postoperative hematoma was treated aggressively with surgical evacuation and drainage. Six-month follow-up revealed functionally and cosmetically normal vulvar anatomy.

Diagnosis and management of congenital vascular rings: a 22-year experience.

Between 1968 and 1990, we operatively treated 39 patients (19 boys, 20 girls) with congenital aortic arch anomalies. Median age was 7 months (range, 1.5 months to 23 years). Thirty-seven patients (95%) had respiratory symptoms. Barium swallow was diagnostic in 95%. Right arch with aberrant left subclavian artery and double aortic arch were the most common types (11 each). Treatment of an aortic diverticulum was documented in 19 patients; the aortic diverticulum was excised (9), managed by aortopexy (7), or left in situ (3). Postoperative recovery was rapid, with a median intensive care unit stay of 2 days, time to oral feeding of 1 day, and postoperative time to discharge of 7 days. Two deaths occurred: 1 infant had undergone emergent operation for control of hemorrhage from an aortotracheal fistula due to tracheostomy tube erosion, and the other had multiple associated congenital heart defects. Postoperative complications included bleeding (1), pneumonia (5), and chylothorax (4). One boy had persistent severe symptoms due to an untreated aortic diverticulum and underwent subsequent excision of the aortic diverticulum with complete relief of symptoms. Median length of follow-up was 12.5 months, with at least 97% of survivors completely or nearly completely free of symptoms from the vascular ring. These results suggest that early repair of congenital aortic vascular rings, including fixating or excising an associated serious aortic diverticulum, is safe and effective and allows for normal tracheal growth.