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PubMed · 11043322

[Oxycephaly, acrocephaly].

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H Nishimoto. 2000. [Oxycephaly, acrocephaly].. https://pubmed.ncbi.nlm.nih.gov/11043322/

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Prenatal ultrasound diagnosis of fetal craniosynostosis.

OBJECTIVE: Craniosynostosis is defined as the premature closure of the calvarial sutures. The prevalence of this heterogeneous condition is 1 in 2000 and approximately 100 different forms have been described with an established genetic transmission in half of them. Prenatal diagnosis of craniosynostosis relies mainly on identification of associated anomalies and molecular analysis of fetal DNA, which is only feasible in some syndromic forms and in well-documented families. The objective of this study was to investigate the value of prenatal ultrasound examination of cranial sutures in fetuses at risk for craniosynostosis. METHODS: Forty fetuses at risk for craniosynostosis on the basis of either a family history (Group 1, n = 16) or skull deformity suspected on a first-level fetal ultrasound examination (Group 2, n = 24) were retrospectively investigated. Craniosynostosis was suspected on the basis of skull deformities when present, however the diagnosis was only made in cases where there was a loss of hypoechogenicity of the normal sutures. All infants had both clinical and radiological investigations performed postnatally. RESULTS: In Group 1, serial ultrasound examination from 12 weeks' gestation onwards led to accurate prenatal diagnosis in all 16 cases. Dysmorphism and skull deformity preceded closure of the sutures by 4 to 16 weeks. In Group 2, prenatal diagnosis was correct in 23/24 cases. There were no false-negative results in either group. CONCLUSIONS: This series questions further the uncertain genetic determinism of craniosynostosis and seems to rule out the hypothesis of a deformation sequence following primary closure of the cranial sutures. It also suggests that ultrasound examination is useful to demonstrate closure of the sutures in the third trimester of pregnancy in most affected cases.

Craniosynostoses↗

Endoscopic strip craniectomy: a minimally invasive treatment for early correction of craniosynostosis.

Traditionally, surgical correction of craniosynostosis involves calvarial remodeling, large blood losses necessitating transfusions, hospital stays of several days, and less-than-satisfactory results. In this study, outcomes from a minimally invasive technique called endoscopic strip craniectomy, along with a postoperative molding helmet, to correct craniosynostosis in young infants were evaluated. The endoscopic strip craniectomy was performed on 185 patients with clinical signs of craniosynostosis, with the following distribution: 107 sagittal, 42 coronal, 37 metopic, and 7 lambdoid, for a total of 198 sutures. The mean blood loss was 29.4 cc, and only two patients underwent intraoperative blood transfusion. Fourteen patients underwent postoperative blood transfusion; none was life-threatening. There were no deaths, complications, neurological injuries, or infections. All but six patients were discharged on the first postoperative day. A majority of the patients achieved or approached normocephaly, and there were no complications. Neuroscience nurses need to be aware of this technique when they discuss treatment options with the families of infants with craniosynostosis.

Craniosynostoses↗

[Positional plagiocephaly].

INTRODUCTION: To prevent early sudden infant death syndrome, the health authorities recommend that newborn infants sleep on their back. This has led to an increase of positional plagiocephaly. The aim of this study was to describe this condition and to recommend a preventive treatment. MATERIAL AND METHODS: This is a retrospective registration of 133 children with positional plagiocephaly seen in the period from 1994 to 2000. RESULTS: The number of children with positional plagiocephaly increased from two in 1994 to a maximum of 43 in 1999. 83 were males and 50 were females. 84 were dextral and 49 were sinistral. In seven (14%) of the sinistral and three (4%) of the dextral, we found a physical explanation of the head turning. In 51 children, X-rays of the skull were performed, but no synostosis was found. DISCUSSION: The back-sleeping position of infants is a promotive factor to positional plagiocephaly which may be prevented by simple alternating head positioning. Otherwise early (< 6 months of age) corrective physiotherapy and positioning or an orthoplastic helmet must be considered. The diagnosis of positional plagiocephaly is based on clinical observations. There is a favourite head turning, an ipsilateral occipital flattening, an ipsilateral frontal bossing, and the ipsilateral ear is moved forward.

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