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Biomedical subjects

D Marchac

Publications and source records attributed to D Marchac.

At least 55 records · Page 3Linked to original sources

Genetic study of nonsyndromic coronal craniosynostosis.

From a series of 1265 individuals with different craniosynostoses hospitalized between 1976 and 1993, 260 probands with nonsyndromic unilateral (181) or bilateral (79) coronal synostosis were analysed. The prevalence of craniosynostoses was estimated as 1 in 2100 children. In the group of coronal synostosis, family history was obtained on 192 probands in 180 pedigrees. The male:female ratio was 1:2. The average paternal age was 32.7 +/- 6.4 years, which is significantly higher than normal. In 26 of the 180 pedigrees, a high degree of familial aggregation was observed, giving a 14.4% figure of familial cases. The bicoronal synostoses were significantly more often familial than the unicoronal synostoses. Segregation analysis of these families leads to the conclusion that coronal synostosis is transmitted as a dominant disorder with 0.60 penetrance and 61% of sporadic cases.

Adult↗

Shave excision and phenol peeling of generalized verrucous epidermal nevus.

BACKGROUND: Generalized verrucous epidermal nevus is a disorder characterized by papulokeratotic and verrucous plaques involving a large area of the skin. This disease has been associated with a number of malformations including osseous, neurological, and ophthalmologic anomalies. OBJECTIVE: To report clinical and histologic features of verrucous epidermal nevus that developed in association with osseous anomalies, and the surgical technique employed. METHODS: Treatment consisted of razor blade shaving using a dermatome, followed by phenol peeling on the pigmented areas of the face. RESULTS: The described procedure resulted in a cure of the verrucous epidermal nevus with good cosmetic outcome. CONCLUSION: Generalized verrucous epidermal nevus can be successfully managed by surgical excision using a razor blade mounted on a dermatome.

Chemexfoliation↗

Prognosis for mental function in scaphocephaly.

Three hundred ninety-six children with scaphocephalies were prospectively studied to analyze the correlation between age, intracranial pressure (ICP), and mental function outcome. The ICP measurements and the early and late psychometric assessments were compared. The influence of surgery, when performed, was analyzed. In most cases, the mental function outcome of the patients was good whether or not they had undergone surgery. The mental level and the frequency of increased ICP both correlated with patient age. A correlation was found between the early and late psychometric assessments in all patients. Thus, the main predictive factor of mental function outcome appears to be the initial developmental level.

Brain↗

Evaluation of the effect of early mobilization of the supraorbital bar on the frontal sinus and frontal growth.

Consequences of early frontocranial remodeling are controversial. It has been said that secondary operations are more difficult and that the frontal sinus does not develop well, with an adverse effect on forehead aesthetics. Some illustrative cases are presented, among 820 operated craniosynostoses, to demonstrate that (1) an early, well-performed frontal advancement and/or remodeling is followed by satisfactory reossification and permits easy secondary surgery, (2) frontal sinus development, which is always impaired in anterior craniosynostosis, is only moderately diminished by early supraorbital bar remodeling (when the frontal bar is repositioned after remodeling, as with trigonocephaly, the frontal sinus develops in 83 percent of cases, whereas when the advancement is significant, as with brachycephaly, the development of the frontal sinus is observed in only 50 percent of the cases), and (3) forehead aesthetics are not linked closely to the development of the frontal sinus. After a significant advancement, even if the sinus does not develop, the frontal bar projection can remain satisfactory.

Child↗

Timing of treatment for craniosynostosis and facio-craniosynostosis: a 20-year experience.

The timing of surgery for craniosynostosis is still controversial. Having used the same basic techniques since 1973, and having done follow-up on the growth of our 983 operated patients, we thought it useful to report our protocol. Early frontocranial remodelling is performed between 2 and 4 months for brachycephalies, but the other craniosynostoses are operated on between 6 and 12 months of age. When diagnosis is made later, we perform the same operations until 4 years of age, with some modifications, such as a tongue in groove advancement for brachycephalies, and a complete closure of the bony defects after 2 years of age. Later on, facial distortion and frontal sinus development complicate the surgery. For syndromal craniofacial synostosis, we prefer to perform a two-step operation: forehead advancement first, facial advancement later, to avoid the risk of frontal osteitis. The frontofacial monobloc is indicated, in our opinion, for severe exorbitism in infancy but otherwise we prefer a two-stage procedure. Facial bipartition is necessary to narrow the upper face and widen the maxilla in Apert's syndrome.

Acrocephalosyndactylia↗

Face lifts and sprayed fibrin glue: an outcome analysis of 200 patients.

The use of aerosolised fibrin glue has been evaluated in a group of 100 consecutive patients undergoing face lift procedures and compared to a similar group of 100 consecutive patients who had face lifts but received no fibrin glue. There was a statistically significant decrease in the rate of major haematoma formation and presentation of ecchymosis associated with the use of fibrin glue. The incidence of total complications was unchanged between the two groups, despite the fact that there were no drains or postoperative dressings used in the fibrin glue treated group. Patients found the omission of drains and dressings to be most convenient.

Adult↗

Use of coral granules in the craniofacial skeleton.

Solid coral blocks have been used as a bone graft substitute in clinical orthopedics for more than 12 years. In this study, 36 patients with 54 craniofacial osseous contour defects received subperiosteal augmentations with natural coral granules made chiefly of calcium carbonate. The patients were followed for 12 to 36 months. The postoperative results were satisfactory; there were only 5 sites of clinically evident resorption. There were 2 incidences of wound irritation and 1 instance of overt infection. Technical aspects of the procedure are discussed.

Adolescent↗

[Development of the frontal sinus after frontocranial remodeling for craniostenosis in infancy].

General considerations about frontal sinus development are discussed. This retrospective radiological study concerns 90 craniosynostoses among 850 cases operated in the Cranio-Facial Unit (1976-88, Hôpital Necker des Enfants Malades, Paris, France). The incidence of frontal sinus development is compared between a control group and the craniosynostoses group with a mean age at surgery of 3 years and a mean follow-up of 6.5 years. Pneumatization of the frontal bone seemed to vary according to the type of surgery and the age at review, but was not linked to sex and age at surgery. A classification of fronto-cranial remodelling is suggested.

Adolescent↗

[Glabellar morphology after frontocranial remodeling for craniosynostosis in infancy].

This retrospective clinical and radiographic study concerns 75 cases of craniosynostoses among 850 cases operated in the Craniofacial Unit of Necker's Hospital for Sick Children (1976-1988, Paris, France). The correlation between frontal sinus development and glabellar morphology was analyzed. When there was a significant advancement of the supraorbital ridge the projection of the glabella was satisfactory without any frontal sinus. When no significant advancement was performed, the pneumatization of the frontal bone was as frequent as in the general population and was dependent upon the underlying frontal sinus.

Adolescent↗

Development of the frontal sinus and glabellar morphology after frontocranial remodeling for craniosynostosis in infancy.

General considerations about frontal sinus development are first discussed. We include retrospective clinical and radiographical studies dealing with 90 craniosynostoses among 850 patients operated in the Craniofacial Unit of Necker's Hospital for Sick Children (1976-1988; Paris, France). The incidence of frontal sinus development is analyzed: The pneumatization of the frontal bone seemed to develop according to the type of surgery and to the age at which review was realized, but was not linked to the age at which surgery was performed. The surgery consisted of fronto-orbital remodeling after supraorbital bar mobilization. The median age at surgery was approximately 3 years, and the median length of the postoperative follow-up was 6.5 years, depending on the type of pathology. The correlation between frontal sinus development and glabellar morphology are also analyzed: When there was a significant advancement of the supraorbital bar, the projection of the glabella was satisfactory without any frontal sinus. When no significant advancement was performed, pneumatization of the frontal bone was as frequent as in the general population and was dependent on the underlying frontal sinus. A classification of the different type of frontal advancement is proposed.

Adolescent↗

Lack of ossification after cranioplasty for craniosynostosis: a review of relevant factors in 592 consecutive patients.

The Center for Craniofacial Anomalies of Necker-Enfants-Malades Hospital presents a retrospective study of the outcome of 592 patients who were operated on for craniosynostosis between 1976 and 1991. The quality of ossification 1 year after operation is reported, with a focus on influencing factors. The lack of ossification rate was 5% (30 of 592). Three parameters are identified as increasing the risk of poor osseous wound healing: local postoperative infection; forehead advancement, especially when accomplished with resorbable osteosynthesis; and brachycephaly. In contrast, repaired tears of the dura mater do not seem to pose a risk. Seventy-five percent of patients with local infection and 12.4% with forehead advancement presented a lack of ossification, which is statistically significant (p < 0.001). Lack of ossification can compromise aesthetic and functional results. Decreasing postoperative infection and stable fixation may help promote ossification.

Adolescent↗

Intracranial pressure and intracranial volume in children with craniosynostosis.

Intracranial volume and intracranial pressure have been measured in 66 children with craniosynostosis, 48 boys and 18 girls. The premature fusion of skull sutures is assumed to restrict skull growth and predispose to elevated intracranial pressure. Thirteen children (20 percent) had raised intracranial pressure and demonstrated a significant restriction of skull growth. In this series, volume measurement alone, however, did not serve as a reliable predictor that the intracranial pressure was raised.

Adolescent↗

Intracranial volume in children with craniosynostosis.

The intracranial volume of 104 children with craniosynostosis was measured. Premature fusion of skull sutures is thought to deny the growing brain room to expand, but contrary to traditional teaching the intracranial volume of such children is often within normal limits.

Cephalometry↗

Fibrin glue in craniofacial surgery.

Fibrin glue has been utilized for the past 15 years in Europe in plastic surgery, especially at the beginning for nerve and skin grafts. For 4 years, we have utilized it in craniofacial surgery in over 72 cases. No adverse reaction has been observed, and the various applications are discussed.

Bone Transplantation↗

The operative treatment of isolated craniofacial dysostosis (plagiocephaly): a comparison of the unilateral and bilateral techniques.

Both the safety and efficacy of the treatment of isolated craniofacial dysostosis (plagiocephaly) in infancy have been demonstrated. Opinions remain divided, however, as to the optimal type of procedure to be undertaken. In an attempt to answer this question, we have retrospectively evaluated a study population of 48 children operated on in infancy by either a unilateral or bilateral approach. Results of treatment at a minimal follow-up of 3 years were assessed based on preoperative and postoperative photographs and direct patient examination. Based on this retrospective comparison of the unilateral and bilateral approaches to the treatment of isolated craniofacial dysostosis, we conclude that (1) either approach as specifically outlined will give excellent results in the majority of patients, (2) there is no statistically significant difference in the results obtained by using either procedure, (3) in the majority of instances, less than ideal correction was manifested by contour irregularities evident in the temporal and/or lateral forehead region, and strict attention should be given to these areas in an attempt to further improve results, and (4) in those cases where significant protrusion is observed on the "normal side," a bilateral approach is preferable.

Bone Transplantation↗

The calculation of intracranial volume using CT scans.

A method of calculating intracranial volume from horizontal computerized tomography scan slices is presented. The accuracy of this technique was confirmed by applying it to 10 dry skulls and comparing the values obtained with the true intracranial volumes, as determined by filling the skulls with water. Values ranging between 98.14% and 102.6% of the true values were obtained, the mean error being 1.13%. This technique is now being used to study intracranial volume changes in children with craniostenosis.

Adult↗