Congenital Dislocation of the Knee in the Delivery Room.

BACKGROUND Congenital dislocation of the knee (CDK) is rare and can cause significant distress in the delivery room to parents and to healthcare providers, especially if the latter are unaware of this condition. It may not be detected by prenatal ultrasound and can be either an isolated finding or associated with other anomalies such as developmental hip dysplasia and genetic syndromes such as Larsen syndrome. Because of the risk of development of contractures, immediate referral to a specialized provider is needed. Poor prognostic factors include an association with a genetic syndrome, limited knee flexion related to severe quadriceps retraction, and absence of anterior skin grooves. A satisfactory outcome can be anticipated in isolated cases with easy reducibility of the knee. CASE REPORT A term baby presented unexpectedly with left knee dislocation after delivery. The providers, unaware of the condition, immediately consulted the orthopedic service, who assisted in the diagnosis, and appropriate management was initiated. The baby had serial casting of the leg, which was applied for almost 3 months, with excellent results on the clinical examination. CONCLUSIONS CDK is a rare finding. The diagnosis is primarily clinical and radiographs are used to confirm and assess the degree of the dislocation. The degree of dislocation is important for management and prognosis. Interventions ranging from serial casting to surgery are required as soon as possible. As the CDK can be associated with genetic syndromes or other dysplasias such as developmental dysplasia of the hip and talipes equinovarus, further evaluation for these conditions is warranted.

Severe congenital toxoplasmosis in the United States: clinical and serologic findings in untreated infants.

BACKGROUND: Congenital toxoplasmosis can cause significant neurologic manifestations and other untoward sequelae. METHODS: The Palo Alto Medical Foundation Toxoplasma Serology Laboratory database was searched for data on infants 0 to 180 days old, in whom congenital toxoplasmosis had been confirmed and who had been tested for Toxoplasma gondii-specific immunoglobulin G (IgG), IgM, and IgA antibodies, between 1991 and 2005. Their clinical findings were confirmed at the National Collaborative Chicago-based Congenital Toxoplasmosis Study center. We reviewed available clinical data and laboratory profiles of 164 infants with congenital toxoplasmosis whose mothers had not been treated for the parasite during gestation. RESULTS: One or more severe clinical manifestations of congenital toxoplasmosis were reported in 84% of the infants and included eye disease (92.2%), brain calcifications (79.6%), and hydrocephalus (67.7%). In 61.6% of the infants, eye disease, brain calcifications, and hydrocephalus were present concurrently. T. gondii-specific IgM, IgA, and IgE antibodies were demonstrable in 86.6%, 77.4%, and 40.2% of the infants, respectively. Testing for IgM and IgA antibodies increased the sensitivity of making the diagnosis of congenital toxoplasmosis to 93% compared with testing for IgM or IgA individually. IgM and IgA antibodies were still present in 43.9% of infants diagnosed between 1 and 6 months of life. CONCLUSIONS: Our study reveals that severe clinical signs of congenital toxoplasmosis including hydrocephalus, eye disease, or intracranial calcifications occurred in 85% infants whose sera were referred to our reference Toxoplasma Serology Laboratory during a period of 15 years. Laboratory tests, including serologic and polymerase chain reaction tests, were critical for diagnosis in the infants. Our results contrast remarkably with those of European investigators who rarely observe severe clinical signs in infants with congenital toxoplasmosis.

Cataracts in congenital toxoplasmosis.

PURPOSE: To determine the incidence and natural history of cataracts in children with congenital toxoplasmosis. METHODS: Children referred to the National Collaborative Chicago-based Congenital Toxoplasmosis Study (NCCCTS) between 1981 and 2005 were examined by ophthalmologists at predetermined times according to a specific protocol. The clinical course and treatment of patients who developed cataracts were reviewed. RESULTS: In the first year of life, 134 of 173 children examined were treated with pyrimethamine, sulfadiazine, and leukovorin, while the remaining 39 were not treated. Cataracts occurred in 27 eyes of 20 patients (11.6%, 95% confidence interval [7.2%, 17.3%]). Fourteen cataracts were present at birth and 13 developed postnatally. Locations of the cataracts included anterior polar (three eyes), anterior subcapsular (six eyes), nuclear (five eyes), posterior subcapsular (seven eyes), and unknown (six eyes). Thirteen cataracts were partial, nine total, and five with unknown complexity. Twelve cataracts remained stable, 12 progressed, and progression was not known for 3. Five of 27 eyes had cataract surgery, with 2 of these developing glaucoma. Sixteen eyes of 11 patients had retinal detachment and cataract. All eyes with cataracts had additional ocular lesions. CONCLUSIONS: In the NCCCTS cohort, 11.6% of patients were diagnosed with cataracts. There was considerable variability in the presentation, morphology, and progression of the cataracts. Associated intraocular pathology was an important cause of morbidity.