Biomedical subjects

D Marchac

Publications and source records attributed to D Marchac.

At least 37 records · Page 2Linked to original sources

PubMed1998

Functional outcome after surgery for trigonocephaly.

The long-term mental outcome of 76 children operated on for trigonocephaly was assessed, and the factors influencing the prognosis were studied. Final assessment of mental development was made on children who were more than 3 years old and was based on the occurrence of behavioral disturbances, learning disability, and school difficulties, and on intellectual efficiency. Children were graded into three groups: no abnormality, mild abnormalities but with normal social function, and grossly abnormal. Preoperative computed tomography scans were used to measure the severity of the frontal stenosis and to identify associated intracranial abnormalities, such as agenesis of the corpus callosum, dilatation of the subdural spaces, or hydrocephalus. Associated extracranial malformations and associated family cases were also noted. Lastly, the family setting was studied. Overall, 31.6 percent of patients had evidence of some degree of trouble. Several correlations were identified: mental development was worse when the frontal stenosis was severe, when cranial reconstruction was performed after 1 year of age, and when there were associated extracranial malformations. In addition, the family environment was found to have a major influence, but the presence of intracranial abnormalities did not correlate with mental development.

Adolescent↗

PubMed1998

[Aplasia of the skull without scalp anomalies. A case report].

The authors report an extremely rare case of cranio-facial anomaly, which, to our knowledge has previously been reported only once (1993, Chakraborty and al.). This male infant presented with a giant congenital bone defect of the skull in the vertex region (10 x 20 cm) with no scalp deficiency. Minimal turricephaly and moderate telorbitism were associated with minor limb anomalies, but psychometric assessment appeared normal. Non-surgical follow-up was initially decided, but spontaneous reossification was so moderate that skull reconstruction was decided at 28 months of age, because of traumatological risks. A full-size resin cephalic skeletal reconstruction was obtained by 3D computerized tomography utilizing stereolithography techniques. A titanium plate was customized on the resin model for ideal adaptation to the convex skull defect (8 x 16 cm). Surgery was simply performed, consisting of preliminary undermining between the dura mater and scalp, and screwing of the custom titanium plate. The initial follow-up was uneventful.

Bone Plates↗

PubMed1997

[Oxycephaly, a severe craniosynostosis. Apropos of a series of 129 cases].

AIMS: The authors analyse a series of patients with oxycephaly in order to detail the definition of this craniosynostosis and its functional prognosis. PATIENTS AND METHODS: The medical records of 129 oxycephalic patients were reviewed. Skull X-rays, ophthalmologic examination, mental level assessment, intracranial pressure monitoring and CT scan were analysed. The more recent patients were also analysed by MRI. Operated on or not, the patients were followed-up, particularly as far as the mental evolution is concerned. Mean follow-up was 3 years 7 months. RESULTS: One third of the patients came from North Africa, where oxycephaly seems predominant. Mean age at diagnosis was 6 years. Past history of rickets was found in 15% of the patients. On X-rays, the vast majority of the patients presented with multisutural synostosis involving both coronal and sagittal sutures, and diffuse digital prints. At the first mental assessment, one third of the patients had an IQ below 80. Papilledema was found in 17%. The monitoring of intracranial pressure showed an increased pressure in almost two thirds of the patients. Sixty-four percent of the patients with increased intracranial pressure had a normal fundoscopy. Out of 16 patients explored by MRI, 12 had a Chiari I malformation. Postoperatively, all papilledemas disappeared, and the intracranial pressure returned to normal in all cases with preoperative increased intracranial pressure. The mental level seemed to stabilize, the mean postoperative IQ being strongly correlated with the preoperative level. In non-operated patients, the mental level worsened significantly. CONCLUSIONS: Oxycephaly is a late-appearing craniosynostosis, with a high risk of ophthalmologic and mental complications. Based on the present series, the operation seemed effective in preventing these complications.

Adolescent↗

PubMed1997

Aplasia of the vertex without scalp defect.

We report a case of an extremely rare craniofacial condition, which, to our knowledge, has previously been reported once only. A male infant presented with a giant congenital bone defect of the skull, in the vertex region (10 x 20 cm) and without scalp deficiency. Minimal turricephaly and moderate orbital hypertelorism were associated with slight limb abnormalities, but psychometrical assessments appeared normal. Nonsurgical treatment was initially decided upon, but spontaneous reossification was so moderate that skull reconstruction was carried out at 28 months of age because of the risk of trauma. A full-size resin cephalic skeletal reconstruction was obtained according to three-dimensional computed tomography using stereolithographic techniques. A titanium plate was customized on the resin model for ideal adaptation to the convex skull defect (8 x 16 cm). Surgery was simply performed, consisting of a preliminary undermining between the dura mater and the scalp and screwing of the custom titanium plate. The initial follow-up was uneventful.

Bone Plates↗

PubMed1997

Fibrin glue fixation in forehead endoscopy: evaluation of our experience with 206 cases.

The endoscopic approach has totally replaced the bicoronal approach for the treatment of forehead aging in our practice since November of 1993. The fixation in proper position is obtained with fibrin glue. Results of a series of 206 procedures performed in 196 patients are presented. Eyebrow positioning and muscle weakening were rated on a scale of 1 to 4. The evaluation has been made at a minimal follow-up time of 6 months. The comparison of the results between the first series of 28 patients during the first 6 months of our experience and the second series of 69 patients during the following period showed a significant increase in the number of excellent results (from 47 to 78 percent). Altogether, the satisfactory results (rated excellent or good) increased from 71 to 86 percent. Fibrin glue provides a stable fixation with the possibility of displacement in several directions, including transversal expansion. Complications have been minimal. Secondary procedures and association with other facial operations have been analyzed.

Aged↗

PubMed1997

Classification of previously unclassified cases of craniosynostosis.

Cases of craniosynostosis usually fall into well-demarcated categories: those related to a syndrome or those identified by a combination of suture involvement and morphological appearance. Between 1976 and 1995, 53 (3.6%) of 1474 cases in the craniofacial databank were assessed and designated as nonsyndromic but unclassifiable. The records and radiological studies obtained in these patients were retrospectively analyzed and comparisons were made with patients classified in the databank as having simple craniosynostoses. It proved possible to divide the formerly unclassifiable cases into two groups: those with "two-suture disease" (Group A) and a "complex" group (Group B) in which more than two sutures were affected. Group A consisted of 36 cases (68%) of patients presenting with clear evidence of simultaneous involvement of two sutures but with no progression over time to suggest a more diffuse pansynostosis. Suture involvement was as follows: 17 of 36 sagittal plus one coronal; seven of 36 sagittal and metopic; six of 36 sagittal plus one lambdoid; and six of 36 metopic plus one coronal. The only significant difference between the Group A cases and the cases of simple craniosynostoses was in the percentage requiring a second operation (24% vs. 5%, p < 0.0001). Group B consisted of 17 cases in which the patients presented at a slightly earlier age (mean 1 year) with severe morphological changes and multiple suture involvement. At the time of surgery, six of 17 patients showed large areas of lacunae within the cranial vault, making craniectomy the only option. In Group B, 10 of 17 patients displayed bilateral lambdoid plus sagittal suture involvement resulting in marked occipital recession posteriorly, whereas anteriorly in six of these 10 patients there was a massive frontal bone associated with posteriorly located coronal sutures. In contrast, there were also four patients in Group B with bilateral coronal plus metopic involvement resulting in a small frontal bone. There was a trend toward a lower intelligence quotient and a worse morphological outcome in the patients in Group B, but again the only result attaining statistical significance when compared to the databank was the rate of second operation (37.5 vs. 5%, p < 0.0001). "Two-suture synostosis" is a relatively straightforward condition and is treatable with standard craniosynostosis techniques. However, possibly as a result of surgical compromise when two sutures are involved, the rate of reoperation is far higher than in simple suture cases. In contrast, patients in the "complex" group presenting with severe multisuture involvement require a more tailor-made approach to their management that often entails a second procedure.

Analysis of Variance↗

PubMed1997

[Craniosynostosis and faciocraniosynostosis].

The authors present a review of the aetiopathogenesis and treatment, based on a series of 1321 craniostenoses operated by the Enfants Malades team. After briefly reviewing the embryology of craniofacial growth, the authors describe the morphological classification of craniostenoses and their morphological and functional consequences. The main neurosurgical problems related to craniofacial surgery are described. The surgical techniques currently used by the unit are described for each type of craniostenosis, according to age: H or flap transposition craniectomies for scaphocephaly, unilateral advancement of a bilateral head-band for plagiocephaly, anterior transposition for oxycephaly, and fronto-orbital adbancement for brachycephaly. The results are presented with a follow-up of several years. The principles of one-stage or two-stage surgical treatment for the main types of faciocraniostenosis are recalled: initial fronto-orbital advancement then secondary treatment of maxillary recession. The prospects of one-stage combined treatment with early maxillary distraction are proposed. Surgical indications are described. The complications, morbidity and mortality are indicated for the series. It must be remembered that craniostenosis surgery is a form of plastic surgery with neurosurgical complications. To obtain optimal results with the lowest risk, craniofacial must be performed by multidisciplinary teams in specialized centres.

Child↗

PubMed1997

[Distraction of the maxilla].

The distraction of the maxilla provides very useful possibilities in young children with unstable articulation and in infants in order to avoid excessively radical operations. The principles of the distraction are reviewed. The authors report 13 clinical cases and complications. The material needs to be perfected, as numerous incidents are still observed.

Child↗

PubMed1997

[Mental prognosis of trigonocephaly and therapeutic implications].

The authors assessed the long-term mental prognosis of trigonocephaly, in a series of 76 operated cases. Mental prognosis factors were studied, showing that early cranial release and reconstruction were effective. Final assessment of mental development was performed on children of school age, and was based on the development of behavioral disturbances, learning disability, school difficulties, and intellectual efficiency. Children were graded into 3 groups: no abnormality, mild abnormalities with normal socialization, major abnormalities; 31.6% presented disorders. Preoperative C-T scans assessed the severity of the cranial deformity and identified associated intracranial abnormalities, such as agenesis of the corpus callosum, dilatation of the subdural spaces, or hydrocephalus. Associated extracranial malformations, and associated family cases were also noted. Finally, the quality of the family context was studied. Several correlations were identified; mental development was correlated with the severity of frontal stenosis, the age at surgery and the associated extracranial malformations. Family environment also had a major influence. Intracranial abnormalities were not correlated with mental development.

Child, Preschool↗

PubMed1997

[Antley-Bixler syndrome. Description of two new cases and review of the literature. Prognostic and therapeutic aspects].

Antley-Bixler syndrome was first described in 1975, and to date, 20 cases have been reported. In addition to brachycephaly, the syndrome is associated with midface hypoplasia often with choanal stenosis or atresia, bilateral radiohumeral synostosis, multiple joint contractures, femoral bowing and long bone fractures, "pear-shaped nose", dysplasic ears, and occasionally urogenital or cardiac defects. Survival is closely linked to upper airway obstruction, which also affects (with craniosynostosis) mental prognosis. Association and severity of malformations are variable, and while numerous children have died early from respiratory distress, one third of them are alive, and have had quite satisfactory development. With early and effective prevention of respiratory complications, and early treatment of craniosynostosis, overall prognosis can be favorable. The mode of inheritance is probably autosomal recessive and midtrimester prenatal diagnosis is feasible; genetic counseling depends on accurate prognostic and therapeutic data. We describe 2 further cases. The first a 4 years old male, with unilateral coronal synostosis and radiohumeral synostosis predominating on the same side. The second an 18 months old female, with brachycephaly and an imperforate anus.

Abnormalities, Multiple↗

PubMed1997

[The history of cranioplasty].

Cranioplasties were first performed at the dawn of the history of medicine, as they usually constitute the repair phase of trephination. In preColumbian civilizations, they usually consisted of simple interposition of metal sheets under the scalp. Hippocrates and especially Galien prohibited this surgery and their principles were respected until the 18th century, although a remarkable surgeon, Van Meekeren, succeeded in performing a heterologous cranial bone graft from dog to man in 1668. The discovery of the osteogenic role of periosteum by Duhamel in 1742 opened the way to new research. During the 19th century, there was an extraordinary growth of science, during which all of the bases of the modern medical approach were established. For example, the studies by Ollier in 1859 allowed the first cranial reconstructions by heterologous, homologous and autologous bone transfers. The large number of head injuries left by the First World War promoted the growth of bone cranioplasties, as shown by Delagénière. The discovery of antibiotics allowed the reintroduction of cranioplasties using inert materials such as acrylic resins. However, their excessive use was complicated by numerous cases of infectious rejection. At the end of the 20th century, microsurgery and molecular biology have provided solutions, but have still not resolved the dilemma between reconstructions by autologous or foreign materials.

Animals↗

PubMed1996

Genetic study of scaphocephaly.

From a series of 1,408 patients with craniosynostosis hospitalized between 1976 and 1994, 561 probands with non-syndromal isolated sagittal synostosis were analyzed. The prevalence of sagittal synostosis was estimated in the order of 1 in 5,000 children. Family information was obtained from 373 probands distributed among 366 families. The male:female ratio was 3.5:1. There was no maternal or paternal age effect. In 22 of the 366 pedigrees, a high degree of familial aggregation was observed, giving a 6% figure of familial cases. Segregation analysis of 253 families indicates that sagittal synostosis is transmitted as a dominant disorder with 38% penetrance and 72% of sporadic cases. The frequency of twinning was 4.8% with only 1 concordance for sagittal synostosis in a monozygotic twin pair. The possibility of a mechanical pathogenesis in sporadic cases is discussed.

Adolescent↗

PubMed1996

Faciocraniosynostosis: from infancy to adulthood.

Faciocraniosynostosis patients require continuous care from early infancy to adolescence, the problem being first cranial, then facial, and finally facial harmony. In this lecture the author's personal experience with patients affected by Crouzon and Apert syndromes is described. Advantages and disadvantages of the different surgical procedures are described, and early and late results are discussed in terms of cosmetic and functional correction.

Acrocephalosyndactylia↗

PubMed1996

[Mental prognosis of Apert syndrome].

BACKGROUND: Mental retardation, considered as common in Apert syndrome could be in part due to associated brain abnormalities. POPULATION AND METHODS: Sixty patients (32 males, 28 females) were included in the study. Patient age at the last examination was over 3 years (mean 10 years, range 3-28 years) in 38 patients. IQ was assessed from psychometric tests adapted for age. Brain anatomy was studied by MRI. Age at operation and quality of familial environment were also evaluated. RESULTS: The IQ was over 70 in 12 patients (32%), over 90 in five (13%) and the mean IQ was 62 (10-114). Thirty percent of patients had abnormalities of the corpus callosum, 43% of the cerebral ventricles and 55% of the septum pellucidum. There was no anomaly in 28% of the patients. One or more operations were performed in 53 patients, before one year of age in 37. Ten children were institutionalized or in deleterious family situation. The main factor influencing the mental prognosis was the age at operation: the final IQ was over 70 in 50% of the children operated on before one year of age versus 8% in those operated on later (P = 0.01). Only the anomalies of the septum pellucidum seemed to play a role: 50% of the patients with normal septum had an IQ > 70 compared to 18% in those with septum anomalies (P < 0.04). The quality of the familial environment also influenced the mental development: 12.5% of the patients who were institutionalized or in difficult familial situation had an IQ > 70 compared to 39% of those who live in a normal family. CONCLUSIONS: Careful investigation including MRI is necessary for detecting associated brain abnormalities. The patients must be operated on early, if possible before the age of nine months. Attention has also to be paid to quality of the sociofamilial environment.

Acrocephalosyndactylia↗

PubMed1996

[Mental prognosis in scaphocephaly].

BACKGROUND: The mental prognosis of scaphocephaly remains a controversial issue, and surgery is performed for functional or aesthetical reasons without clear evidence in the literature of which is the most important. PATIENTS AND METHODS: Three hundred and ninety six children with scaphocephaly were prospectively studied to analyse the correlation between age, intracranial pressure (ICP) and mental outcome. Before any treatment, the intracranial pressure was recorded (systematically during the first period of the study); the mental level was evaluated at first consultation and after a mean five-year follow-up. The mental evolution was compared whether the child was operated or not. RESULTS: The mental outcome of the patients was good in most of the cases whether or not they had been operated. There were significantly more normal patients in the scaphocephalies seen before one year of age at first consultation (P < 0.001) than in those seen after one year of age. There were significantly more abnormally high intracranial pressure cases in the group of patients who were seen later than one year of age (P = 0.0015). There were more retarded patients in the group with increased ICP, but the difference was not significant (P = 0.17). There was no correlations between ICP and final IQ neither in operated nor in non-operated patients. Conversely, a correlation was found between the early and late psychometric assessments in all patients. CONCLUSIONS: The main predictive factor of mental outcome appears to be the initial developmental level. Since the mental level was worse in the older children, and since the surgery does not influence the functional outcome when the initial mental level is low, we can conclude that the indication to perform surgery in scaphocephaly is sometimes not only a cosmetic problem.

Child Development↗

PubMed1996

Prognosis for mental function in Apert's syndrome.

The factors involved in the mental development of patients with Apert's syndrome were studied by the authors, focusing on the age of the patient at operation, associated brain malformations, and the quality of the family environment. Overall, 32% of patients with significant follow-up review had an intelligence quotient (IQ) greater than 70. Age at operation appeared to be the main factor associated with changes in mental development: final IQ was greater than 70 in 50% of patients operated on before 1 year of age versus only 7.1% in patients operated on later in life (p = 0.01). Malformations of the corpus callosum and size of the ventricles played no role in the final IQ, whereas anomalies of the septum pellucidum had a significant effect, with the proportion of patients with an IQ over 70 increasing more than twofold in patients with a normal septum compared with patients with septal anomalies (p < 0.04). Quality of the family environment was the third factor involved in intellectual achievement: only 12.5% of institutionalized children reached a normal IQ level compared to 39.3% of children from a normal family background.

Acrocephalosyndactylia↗

PubMed1996

[Lack of ossification of the skull after surgery for craniosynostosis. A study of risk factors apropos of 592 cases].

The Center for Craniofacial Anomalies of Necker-Enfants-Malades Hospital presents a retrospective study of the outcome of 592 patients who were operated for craniosynostosis between 1976 and 1991. The quality of ossification one year after operation is reported with a focus on influencing factors. The lack of ossification rate is 5% (30/592). 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. On the contrary, repaired tears of the dura mater do not seem to pose a risk. Seventy five per cent of patients with local infection and 12.4% of 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 to avoid it.

Child, Preschool↗

PubMed1996

[Outcome of nasal deviation in plgiocaphaly after bilateral frontocranial modeling in childhood].

The deviation of the nasal root is one of the major deformities in unilateral coronal synostosis. The objectives of this study are to evaluate the results of bilateral frontocranial remodeling on nasal deviation, focusing on the patient's age at the time of the operation. All the patients undergoing bilateral frontocranial remodeling for plagiocephaly and followed for a minimum of 5 years postoperatively, without any other craniofacial surgery were included in the study. Based on photographic data, 42 patients were assigned to one of 4 morphologic categories: M1 = absence of nasal deviation; M2 = minor nasal deviation with no or minor revision required; M3 = severe nasal deviation with major alternative osteotomies required or performed; M4 = major nasal deviation with major craniofacial procedure and bone-grafting required or performed. Thirty-nine patients presented with significant nasal deviation before surgery. Ninety-two percent had a morphologic improvement and 62% had no residual nasal deviation (M1) more than 5 years after the procedure. The best results were obtained in patients operated between the ages of 12 and 24 months. All patients who underwent surgery during this period obtained improvement of the deformity, and 90% had no residual deviation. In contrast, when patients were operated before the age of 12 months, 91% obtained improvement of the nasal deformity, and 57% were assigned to the M1 category. Additionally, surgery performed between the ages of 2 and 4 resulted in 67% of partial correction and 33% of ideal correction. The most difficult age group to correct were children 4 years and older. Of the 4 patients in this group, one required major secondary reconstruction (M3), and only one patient achieved complete correction. Although the endpoint for correction is an aesthetic assessment, cephalometric analysis was also performed in 29 cases. Comparison of the evaluations gave equivalent results.

Child↗