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At least 235 records · Page 13Linked to original sources
PubMed1994

Metopic synostosis: evaluation of aesthetic results.

Analysis of intermediate- and long-term results of surgical treatment of metopic synostosis is lacking. We therefore retrospectively studied 23 patients with metopic synostosis (14 males, 9 females) who have been followed from 3 months to 8.1 years (mean 42.5 months) after operation. Age at first operation ranged from 2 to 56 months (mean 8.2 months), with 15 patients operated on before 6 months and 8 after 7 months. Fronto-orbital remodeling and calvarial vault reshaping with floating forehead techniques were carried out in all patients. Stabilization of bony segments was accomplished with microplates and screws in 7 patients (30 percent), wires in 15 (65 percent), and absorbable sutures in 1. Complications included minor wound dehiscence (n = 1), seizures (n = 1), and increased intracranial pressure (n = 1). Postoperative photographic documentation of surgical results was available in 17 of the 23 patients. Aesthetic outcome in these 17 patients was graded (I = none or minor contour irregularities; II = moderate; and III = severe) by one of the authors (Cohen) and by a lay panel (n = 3) according to the degree of residual cranio-orbital deformity. Judged by the surgeon, grade I results were present in 53 percent, grade II in 35 percent, and grade III in 12 percent. To date, total reoperation (reoperative fronto-orbital remodeling and calvarial vault reshaping) was necessary in 2 patients (9 percent), one of whom had signs of increased intracranial pressure 3 years after the original craniofacial procedure, while partial reoperation (temporal cranioplasty) was carried out (n = 2) or recommended (n = 3) in another 5 patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗
PubMed1994

[Pancraniofacial synostosis--indications for early craniofacial operation].

Premature synostosis of all major cranial sutures represents a rare craniofacial anomaly often leading to serious consequences. It is regularly associated with Kleeblattschädel-syndrome (clover-leaf skull) but can also be observed in Crouzon's craniofacial dysostosis, Apert's acrocephalosyndactyly, Pfeiffer's-syndrome and severe plagiocephaly. Since the disease not only affects the cranial vault but also the facial skeleton, we simply termed it pancraniofacial synostosis (PCS). Radical surgical therapy is frequently necessary after birth for vital indication. This study presents results and complications of 12 children with PCS. Eleven children were operated on by subtotal craniectomy and advancement of the fronto-orbito-facial skeleton. Concomitant hydrocephalus was shunted preoperatively. Four children with impending loss of vision and intracranial hypertension had to be operated on under emergency conditions only a few weeks after birth. One child with normal intracranial pressure has been closely followed for over ten years without surgery. Lethal complications occurred in three of four children that had been operated on under emergency conditions. Craniofacial surgery significantly improved intracranial hypertension, exorbitism, nasopharyngeal obstruction, and aesthetic appearance in all of the remaining children. Total craniofacial correction in PCS can safely be achieved at an age of three to twelve months. Emergency surgery carries a much higher risk. Simultaneous mid-face advancement should be avoided in those cases if at all possible.

Cephalometry↗
PubMed1994

Metopic synostosis: quantitative assessment of presenting deformity and surgical results based on CT scans.

Our study applied a method of using 14 clinically relevant measurements of CT scan images to document skeletal dysmorphology in patients with metopic synostosis to determine the effectiveness of their surgical procedure after a minimum of 1 year. Ten consecutive patients with metopic synostosis who underwent a standard surgical procedure were reviewed. Preoperative and postoperative (> 1 year) CT scans were compared with those of age-matched controls. Percentages of normal were then compared for significant differences. Preoperative cranial vault measurements revealed a narrowed anterior intercoronal distance at 92 percent of normal. Orbital measurements showed a narrowed anteromedial wall interorbital distance at 79 percent of normal, a narrowed lateral orbital wall distance at 94 percent, and an elevated medial orbital wall protrusion beyond the plane of the lateral orbital walls at 115 percent. After surgery, the intercoronal distance was significantly corrected to 101 percent, the anteromedial wall interorbital distance was improved but remained undercorrected at 90 percent, and the medial wall protrusion was significantly decreased to 98 percent of normal. Quantitative measurements of CT scan images confirmed clinically observed physical findings in unoperated trigonocephaly to be orbital hypotelorism, retruded lateral orbital rims, and a narrowed bitemporal width. The surgical technique corrected the lateral orbital wall aspects of the deformity as well as the narrowed anterior cranial vault width and improved the orbital hypotelorism.

Cephalometry↗
PubMed1996

Observations on a recent increase in plagiocephaly without synostosis.

OBJECTIVE: To verify and determine the cause of an increase in the referral of infants with plagiocephaly without synostosis (PWS) to a single tertiary craniofacial center. DESIGN: A chart review was performed for 269 infants with a diagnosis of PWS who presented to a single tertiary craniofacial center between 1979 and 1994. The pattern of referral for PWS was analyzed using both simple linear regression and time series regression analyses. In addition, the referral pattern for PWS was compared with that for infants seen at the same center who received a diagnosis of synostotic plagiocephaly. Changes in the distribution of several demographic, perinatal, and clinical variables during the study period were also assessed. Finally, in an effort to identify correlates of the risk of PWS developing, characteristics of patients who were Missouri residents and presented between 1992 and 1994 were evaluated and compared with those of the 1993 Missouri live birth cohort. SETTING: The Cleft Palate and Craniofacial Deformities Institute, St Louis Children's Hospital, Washington University Medical Center. RESULTS: The average annual number of referrals to our center for PWS in the period 1992 to 1994 was more than sixfold greater than that for the preceding 13 years. There was a statistically significant increase in the annual number of referrals to our center during the 16-year study period. Moreover, there was evidence that the average annual increase in referrals was significantly greater during the last 3 years (1992 through 1994) of the study than in the first 13 years. This shift in the referral patterns is roughly contemporaneous with the American Academy of Pediatrics recommendations regarding infant sleep position. There was no evidence that either the mean number of referrals or the average annual increase in referrals for patients with synostosis changed during the study period. Among patients with PWS, the average age at presentation did not change during the study period. There were also no significant changes in the distribution of other demographic, perinatal, and clinical variables. When compared with the Missouri birth cohort, infants with PWS were significantly more likely to be boys and to have been delivered by forceps. There was also some evidence that patients with PWS were more likely to be born prematurely and to be products of multiple-gestation pregnancies. These associations were, however, of only borderline statistical significance. CONCLUSION: Referrals to our center for PWS increased markedly in 1992 relative to previous years. The temporal coincidence of this increase with the American Academy of Pediatrics recommendation to avoid the prone sleeping position, to reduce the risk of sudden infant death syndrome, suggests a possible causal relationship. If this association is causal, education regarding the need for head position rotation coupled with that for sudden infant death syndrome should obviate positional PWS.

Craniosynostoses↗
PubMed1998

Three new cases of spondylocarpotarsal synostosis syndrome: clinical and radiographic studies.

Spondylocarpotarsal synostosis syndrome (SSS) or congenital synspondylism is a recently delineated clinical entity. At least 15 patients have been reported. We present 3 new patients, 2 of whom were sibs born to first-cousin parents. All of our patients had multiple synostoses involving cervical, thoracic and/or lumbar vertebral bodies and carpal/tarsal bones, scoliosis/lordosis, and short stature. Sensorineural deafness was found in 2 of the 3 patients. Analysis of clinical manifestations suggests clinical variability and genetic heterogeneity in SSS. Of a total of 18 SSS patients, 10 were five pairs of sibs from five families, with first-cousin consanguinity of parents in 3, indicating that at least one type of SS is an autosomal-recessive disorder.

Abnormalities, Multiple↗
PubMed1989

Humero-radio-ulnar synostosis: a new case and review.

We report on a patient with humero-radio-ulnar synostosis and upper limb oligoectrosyndactyly. All cases have been sporadic including discordance in monozygotic twins, and similar findings have occurred in thalidomide embryopathy. Further observations of similarly affected patients are needed to elucidate the nature of this upper limb defect and its cause.

Abnormalities, Multiple↗
PubMed1995

46,XY/47,XYY/48,XYYY karyotype in a 3-year-old boy ascertained because of radioulnar synostosis.

Chromosome analysis was performed on a 3-year-old boy because of bilateral radioulnar synostosis and demonstrated a mosaic karyotype 46,XY/47,XYY/48,XYYY. He had minor facial anomalies and mild intellectual delay. He appears to be the youngest patient reported with this rare chromosome complement. His father, mother, and brother had normal chromosomes. Fluorescence in situ hybridization (FISH) was performed on the propositus and his father with the Y chromosome heterochromatic probe (pHY3.4) to add to the evaluation of mosaicism.

Aneuploidy↗
PubMed1988

Sagittal synostosis--its clinical significance and the results of three different methods of craniectomy.

From 1973 to 1986, 50 infants with sagittal synostosis have been operated by three different methods of craniectomy (linear craniectomy and extended craniectomies, as proposed by Schut and Epstein et al.). Preoperatively, the mean cephalic index was 67 +/- 4, 35.5% had clinical findings as cerebral palsy, psychomotor retardation and/or neurological signs, and intraoperatively the epidural pressure was more than 200 mm H2O in 60% (recorded in the last 20 patients). The mean follow-up time was 4.7 (1-10.6) years. Postoperatively, only 14.5% had minor clinical signs, which were mostly not in relation to the former scaphocephaly. Half of the patients with increased ICP had clinical signs preoperatively, and none of the 20 patients had distinct findings postoperatively. Out of the 20 children operated on by linear craniectomy or by Schut's method up to 1980, two-thirds had no school problems and one-third some school problems; one-third had occasionally headaches and one-quarter ametropia. Concerning the aesthetic results, Epstein's method and, somewhat less Schut's method, were superior to linear craniectomy, as verified by craniometry and by the tracings of the outlines of the neurocranium 0.4-0.7 and 1.6-2.0 years postoperatively: mean cephalic indices 73 +/- 5 (normal in one-fourth), 74 +/- 7 (normal in half) and 79 +/- 4 (normal in nearly all patients). Epstein's method is superior to the other two methods because it renders it possible to increase the breadth the greatest during the period of greatest postnatal brain growth. In addition to the effect on the neurocranium, the extended craniectomies add to normalization of the base of the skull (in contrast to the natural history of scaphocephaly). In the long run, the results obtained remain the same. The disadvantage of residual skull defects (approximately 11% of the patients with extended craniectomies) can be avoided by performing surgery prior to 4-6 months of age or by preserving the removed bone in a deep-freeze for a limited time.

Cerebral Palsy↗
PubMed1980

Multiple synostosis syndrome.

A 2-year-old deeply mentally handicapped girl is reported with a multiple synostosis syndrome, as delineated by Maroteaux et al. (1972). Besides the multiple synostoses the peculiar facial dysmorphism and the hearing deficit allow, the clinical diagnosis of this apparently rare syndrome.

Child, Preschool↗
PubMed1989

Keyhole craniectomy for correction of sagittal synostosis.

A modification of the technique combining sagittal strip, circular occipital and biparietal wedge craniectomies (Albright's procedure) for the treatment of sagittal synostosis, with prominent occipital bossing, is described. "Keyhole," as opposed to wedge, parietal craniectomies allow improvement in the shape of the skull, beginning intraoperatively, while eliminating the outbending of the parietal bone that occurs at the apex of a wedge craniectomy.

Cranial Sutures↗
PubMed1991

Radio-ulnar synostosis in Williams syndrome. A frequently associated anomaly.

Limitation of supination and pronation of the forearm has been occasionally described in individuals with Williams syndrome. It was found in 26% of 23 subjects examined, and was associated with a spectrum of severity of radio-ulnar synostosis on radiography. The occurrence of this functional motor impairment in a substantial subset of persons with Williams syndrome is relevant, because its evaluation is important in habilitation planning.

Abnormalities, Multiple↗
PubMed1997

[Proximal radioulnar synostosis within the scope of fetal alcohol syndrome].

The extremely rare finding of a proximal radio-ulnar synostosis in a 5 years-old girl with a fetal alcohol syndrome is presented. Despite the elbow's being fixed at 30 degrees pronation, the patient exhibits good ipsilateral arm function. The difficulties in differential diagnosis and treatment modalities are discussed.

Child, Preschool↗
PubMed2006

Spondylocarpotarsal synostosis syndrome: MRI evaluation of vertebral and disk malformation.

Spondylocarpotarsal synostosis syndrome (SSS) is a rare autosomal recessive condition characterized primarily by vertebral malsegmentation, carpal/tarsal coalition, and a dysmorphic appearance. Differentiating SSS from other congenital scoliosis syndromes requires evaluation of the vertebrae, ribs, soft tissues, and spinal cord. The enhanced resolution over plain radiographs seen with MRI allows more detailed assessment of vertebral malformation and surrounding anatomy. Diagnosis of the underlying cause of congenital scoliosis might be enhanced using this technology. We report on a 12-year-old girl of unaffected parents with SSS who was evaluated with MRI sequences of the spine to show various types of malsegmentation. Additionally, there is the new finding of fusion of teeth, with developmental failure of a canine incisor.

Child↗