Power Doppler imaging of the fetal upper aerodigestive tract using a 4-point standardized evaluation: preliminary report.
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OBJECTIVE: Our investigation of fetal swallowing has identified potential limitations in the use of color Doppler imaging for detection of amniotic fluid flow and discrimination of respiratory from ingestive activity. The objective of this study was to evaluate an alternative imaging modality, power Doppler sonography, as a technique to enhance detection of amniotic fluid flow in the upper aerodigestive tract. METHODS: We applied a standardized 4-axis sonographic examination of upper aerodigestive structures and used power Doppler imaging to document amniotic fluid flow. Normal aerodigestive activities from 62 healthy control subjects were compared with 4 abnormal cases. RESULTS: Our longitudinal experience with 66 subjects showed that a directed evaluation of the fetal upper aerodigestive tract with power Doppler imaging provided a systematic approach for studying the physiologic development of this region in both healthy and at-risk fetuses. CONCLUSIONS: A standardized 4-axis examination with power Doppler imaging is a useful adjunct in addressing ingestive and respiratory functions in the developing fetus. (+info)
Dramatic subperiosteal bone formation following physeal injury in patients with myelomeningocele.
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We present five cases of dramatic subperiosteal bone formation following fracture of the distal femoral physis in children with myelomeningocele. These fractures often go unrecognized due to lack of displacement and/or pain. Children present with a swollen, warm extremity and often systemic signs of inflammation, but rarely any pain or history of trauma. The dramatic radiographic appearance can cause a diagnostic dilemma. We present these cases in order to familiarize the clinician with the oftentimes dramatic appearance of this injury. (+info)
Fetal therapy: state of the art.
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OBJECTIVE: To review our experience with the use of sonography in evaluating potential candidates for in utero fetal therapy performed at The Center for Fetal Diagnosis and Treatment at The Children's Hospital of Philadelphia. METHODS: This review article was designed to discuss open hysterotomy for the 4 fetal surgical procedures that have been performed at our institution. The procedures included surgical repair of myelomeningocele, resection of sacrococcygeal teratoma in fetuses with nonimmune hydrops, resection of an enlarging congenital cystic adenomatoid malformation that is not amenable to thoracoamniotic shunting, and tracheal clip occlusion for severe left congenital diaphragmatic hernia. RESULTS: For each surgical procedure, the use of sonography in the prenatal diagnosis of the congenital anomaly was detailed, as were indications for surgery and surgical procedures, postoperative monitoring and finally delivery, postnatal treatment, and long-term follow-up. Three of the procedures have been reasonably successful with rather dramatic results in some cases such that these techniques are still being performed. The 1 exception was open hysterotomy for the tracheal clip procedure for congenital diaphragmatic hernia, which has been abandoned. CONCLUSIONS: Fetal therapy is a rapidly evolving specialty, which is being practiced at several centers in this country. Sonography is an integral part of this specialty practice and has been used extensively in the diagnosis of some congenital anomalies that have debilitating or lethal consequences for the fetus. Technologic improvements in both sonography and magnetic resonance imaging have assisted tremendously in the many advances herein reported in the diagnosis and treatment of the above-described 4 congenital anomalies. (+info)
Some sources of stress found in mothers of spina bifida children.
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The scores on a malaise inventory for 51 mothers of spina bifida children born between 1956 and 1962 in South Wales revealed significantly higher scores when the child was incontinent, had a severe locomotor disability, had an IQ less than 80, and was attending a special school rather than a normal school. No difference in scores was found between the sexes or for size of family, and only a slight difference occurred between the malaise scores of mothers of mildly and moderately handicapped children. (+info)
A comparison of visual function scores in hydrocephalic infants with and without lumbosacral myelomeningocoele.
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PURPOSE: The cerebrum is frequently malformed in children with myelomeningocoele. This anomaly renders them potentially susceptible to cerebral visual impairment. In these patients, hydrocephalus is an important and frequent complicating lesion which compromises intellectual function and may also cause cerebral visual impairment. In this study, we determined whether hydrocephalic patients with lumbar myelomeningocoele (HLM) are at a greater risk of visual impairment than hydrocephalic patients without this lesion (H). METHODS: In this prospective study, we assessed five parameters of visual function in 20 hydrocephalic children with lumbar myelomeningocoele and compared the total visual function scores (TVFS) obtained with those from hydrocephalic children without overt spinal dysraphism, but similar in age, sex and ventricular size. The parameters, which were assessed with the aid of a quantitative grading scale, were pupillary size and reaction, optic atrophy, visual fixation and tracking. RESULTS: The age and sex distributions of the patients in the two groups were similar. The anterior and posterior dimensions of the lateral ventricles were also similar. The mean (SD) of the TVFS were 24.25 (3.63) and 24.20 (3.47) respectively for the two groups (P = 0.90). CONCLUSIONS: The results suggest that, in hydrocephalic infants with lumbar myelomeningocoele, visual function is not further diminished by the associated dysraphism and that ventricular dilatation is the major determinant of visual impairment. (+info)
Preventing the birth of infants with Down's syndrome: a cost-benefit analysis.
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The costs and economical benefits of providing routine prenatal diagnosis of Down's syndrome with termination of affected pregnancies in older pregnant women in the west of Scotland were examined. The potential economic benefits would be greater than the costs for women aged 40 and over, probably about equal to costs for those aged 35 and over, but less than costs if the service were extended to women under 35. (+info)
Prenatal diagnosis: a directive approach to genetic counseling using decision analysis.
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The decision which prospective parents face concerning mid-trimester amniocentesis for prenatal diagnosis was examined by decision analysis. The prospective parents' decision depends on the likelihood of the birth of a child affected by a genetic disorder, the risk of amniocentesis, and the probability that the diagnoses provided by the amniocentesis will be correct. The couple's decision must also depend on their attitudes toward each possible outcome. The likelihoods of the outcomes can be obtained from appropriate medical consultation, while the relative costs or burdens of the outcomes should be obtained from the prospective parents. A truly informed decision for this couple can then be formulated from these probabilities and values, thus allowing genetic counseling to be more directive. The technique is illustrated for the prenatal diagnosis of Down's syndrome, meningomyelocele, and Duchenne muscular dystrophy. (+info)
Kyphectomy in myelomeningocele with a modified Dunn-McCarthy technique followed by an anterior inlayed strut graft.
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Rigid congenital kyphosis in myelomeningocele is associated with an important morbidity with skin breakdown, recurrent infection, and decreased function. Kyphectomy is the classic treatment to restore spinal alignment; however, surgery is associated with an important morbidity and long-term correction is uncertain. The authors retrospectively reviewed 9 patients with a mean age of 8.8 years who underwent a two stage surgical procedure: first a posterior kyphectomy with a modified Dunn-McCarthy fixation consisting of lumbar pedicle screws and long S-shape rods buttressing the anterior sacrum. Then a second stage done several weeks later consisting of a thoraco-abdominal approach to the spine with an inlay strut graft classically from T10-S1. The mean follow-up was 34 months (range 1-5 years). The kyphosis was corrected from a mean of 110 degrees of Cobb angle (range 70-130 degrees) to 15 degrees after surgery (45-0 degrees). There was no instrumentation failure, no loss of correction and no pseudarthrosis. Complications consisted of one intra-operative cardiac arrest fortunately reversible, a wound necrosis, one deep venous thrombosis and one late aseptic bursitis on the posterior hardware. Congenital kyphosis in myelomeningocele can be treated successfully with an initial posterior approach correction and instrumentation followed by an anterior approach allowing for anterior inlay impacted structural graft. The authors believe that this technique improves biomechanical and biological fusion mass anteriorly and will prevent late instrumentation failure and loss of correction. (+info)