INTRODUCTION: Frontofacial monobloc advancement (FFMA), known as a high risk procedure, was evaluated in combination with distraction. PATIENTS AND METHOD: Twenty patients with faciocraniosynostosis, were treated with FFMA and quadruple internal distraction. Mean age at surgery was 3.2 Years and mean follow-up was 14 Months (3-32 Months). Four distractors were used per patient in combination with complete osteotomy FFMA. Rate of distraction was classical (0.5 mm to 1mm per day) starting on day 7. The rate of short-term complications was evaluated. Correction of exorbitism and the dental occlusion relationship were assessed to evaluate achievement of advancement. Rate of relapse was evaluated by measuring the orbital bony gap on horizontal CT slices, before and 6 Months after removal of the distractor. Respiratory impairment when present was also evaluated. RESULTS: Distraction was completed in 18/20 patients. Fifteen patients completed their distraction uneventfully during the initial period. In three patients with CSF leaks, distraction was delayed at day 14 and 16, but was resumed and completed normally. Second operations were required for two patients during the early phase: the distractor failed in one requiring immediate reoperation to change the device; distraction was completed successfully. Device infection developed in two patients requiring removal of the device and abortion of distraction. One patient died the day after surgery from acute tonsillar herniation before distraction could be initiated. Exorbitism was clinically corrected in all cases in whom distraction was completed (n=18). Class I occlusion was obtained in 16/18 patients. When present, respiratory impairment was corrected in all cases. Septic frontal necrosis occurred in one patient two Months after distraction was completed and necessitated removal of the forehead flap. Distractors were easily removed through a coronal approach at 6 Months, but reossification was limited. Relapse was observed in three patients whose retaining phase was less than five Months. Rate of relapse was much lower when distraction was maintained more than six Months. DISCUSSION: Internal distraction can provide early correction of respiratory impairment due to faciocraniosynostosis in infancy and can reduce the major risks of frontofacial monobloc advancement. Morbidity is higher in patients with prior surgery before FFMA. Further evaluation is necessary to determine whether the two stage treatment strategy for faciocraniosynostosis (fronto-orbital advancement before one Year of age, and later facial Lefort III type advancement) could be replaced by a routine FFMA procedure.

Adolescent↗

PubMed2003

[Is there a boundary between reconstructive and aesthetic surgery?].

Reconstructive and aesthetic surgery are the two closely bond facets of plastic surgery. The training, the techniques are the same, and limits are often difficult to define in many cases, intermixing indications. One should well explain to the lay public and to other doctors the complementarity of the two aspects of plastic surgery. One should also help the young plastic surgeons to be the best in reconstruction and embellishment.

Humans↗

PubMed2001

[Distraction osteogenesis with double internal devices combined with early frontal facial advancement for the correction of facial craniosynostosis. Report of clinical cases].

Frontofacial monobloc advancement is a surgical procedure which corrects at the same time both frontal and facial retrusions in faciocraniosynostosis. The high risk morbidity limited its use to major exorbitism or severe breathing impairment. We report the results of a preliminary experience in five children with a mean age of 3.8 years. All patients presented with a Crouzon or Pfeiffer syndrome. The procedure performed was a frontofacial monobloc osteotomy with peroperative mobilization. Two pairs of distractors (Martin-Medizin) were implanted. The upper pair of frontal distractors was located behind the supraorbital bar. The pair of temporo-zygomatic distractors (a modification of the MicroFrance prototypes) was implanted behind the zygomatic bone. In one patient a transfacial pin was connected to the anterior part the lower distractors, to avoid facial bipartition. Distraction was performed according to classical protocols with a 1 mm advancement per day, until sufficient advancement was obtained. The correction of exorbitism and facial retrusion was obtained in all cases. An open-bite was created by the advancement in two out of the five patients. Minor infection occurred in one patient. This combination of distractors with frontofacial advancement seemed to increase the efficacy and reduce the morbidity. This might lead to a one stage strategy for faciocraniosynostosis treatment, even if minor additive procedures will be necessary when children get older and that insufficient facial growth still reoccurs.

Child, Preschool↗

PubMed2001

[Diagnosis of facial and craniofacial asymmetry].

Craniofacial asymmetry is caused by various aetiologies but clinical examination remains the most important criteria since minor asymmetry is always present. The diagnosis can be confirmed by anthropometric measurements and radiological examinations but only severe asymmetries or asymmetries with an associated functional impairment should be treated. The treatment depends on the cause, and on the time of appearance. Congenital asymmetries might be treated early, during the first year of life if a craniosynostosis is present. Hemifacial microsomia are treated later if there is no breathing impairment. Since the pediatricians have recommended the dorsal position for infant sleeping, an increasing number of posterior flattening of the skull has been appearing, and could be prevented by adequate nursing. Other causes of craniofacial asymmetries are rare and should be adapted to the cause (tumors, atrophies, neurological paralysis, hypertrophies) by a specialized multidisciplinar team.

Craniofacial Dysostosis↗

PubMed2000

Management of craniosynostoses.

Although it is currently thought that surgery is indicated mainly for cosmetic reasons in isolated craniosynostoses, the functional aspects of the treatment must not be underestimated. Prospective studies on intracranial pressure and mental evolution of these children have shown that there were functional consequences in a significant proportion of cases even of single suture fusion. The frequency of increased intracranial hypertension and the risk of mental impairment depend on the age of the child and the type of craniosynostosis. In nonsyndromic cases, the higher risks are observed in multisutural craniosynostoses (brachycephaly, oxycephaly). In syndromic cases, the risk of intracranial hypertension is higher in Crouzon syndrome, and Apert syndrome carries the higher risk of mental retardation. The study of a personal series of 2,137 craniosynostoses shows that the functional and the cosmetic results are better after early surgery, and that the operative risks are not higher in infants than in older children.

Craniosynostoses↗

PubMed2000

[Musculoskeletal tissue engineering with resorbable polymers].

Musculoskeletal tissues can present congenital or acquired defects as a result of disease, accidental trauma or iatrogenous causes. This loss of bony substance is traditionally treated by the replacement of bony tissue (grafts or flaps), or by synthetic materials. Each of these methods of treatment, however, entails its specific disadvantages, limitations and complications. The recent approach for treatment of musculoskeletal defects has been the development of the growing of neotissues derived from autogenous cells, and artificial biodegradable matrixes. This method assumed the name "tissue engineering" in the late 1980s. Tissue Engineering, or TE, has employed advances made in the area of cellular culture, intercellular matrix biology, and also, in the area of biomaterial science. TE is an multi-disciplinary approach. Musculoskeletal TE, although in its preliminary stages, should allow access to treatments of the future.

Biocompatible Materials↗

PubMed1999

Midface surgery from Tessier to distraction.

The wall separating the face and the cranium was broken by Paul Tessier and Gérard Guiot in the 1960s, making it possible to perform a combined operation around the orbits and forehead, and opening up close cooperation between plastic surgeons and neurosurgeons, especially for treatment of the major malformations such as hypertelorism of major facial retrusions. The principles of mobilization of the orbits to correct teleorbitism or orbital dystopia are recalled with reference to the different variations and with clinical examples. Facial advancement to correct the retrusions created by faciocraniosynostosis is explained with the many possible variants, combined with a intracranial approach or not, with or without a bipartition. The indications are discussed as is the risk linked to combined advancement of face and forehead. The progressive bone elongation principle introduced by Ilizarov for the limbs has been applied to the face at the mandibular level by McCarthy, with great success. The distraction of bone structures is now also applied at the level of the midface and makes it possible to overcome the retraction of soft tissues and lower the risk of relapse of facial retrusion. Many technical problems have still to be solved, but the results are already very promising. Many other applications of the distraction principle will be developed for the midface level, with other technical improvements such as the use of absorbable mini-plates and screws.

Craniofacial Abnormalities↗

PubMed1998

Syndromal and nonsyndromal primary trigonocephaly: analysis of a series of 237 patients.

From a series of 1,713 patients with craniosynostosis hospitalized between 1976 and 1996, 237 propositi with metopic synostosis were analyzed. The prevalence of metopic synostosis was estimated in the order of 1 in 15,000 children. Family information was obtained from 184 propositi from 179 families. The male-to-female ratio was 3.3:1. There was no maternal or paternal age effect. A family history was obtained in 10 of the 179 families, giving a 5.6% figure of familial cases. The frequency of twinning was 7.8% with two concordances for metopic synostosis in two monozygotic twin pairs. The male-to-female ratio, the twinning frequency, and the proportion of familial cases in trigonocephaly are very similar to those observed in scaphocephaly, which also involves the longitudinal sutural system. Fetal exposure to valproic acid was noticed in eight cases. The series was divided into two groups: nonsyndromal trigonocephaly (n = 184) and trigonocephaly associated with other malformations (n = 53). The second group included 13 cases of well-delineated syndromes and 40 cases of trigonocephaly associated with one or more malformations, but without any known syndrome, that could be undelineated syndromes. These groups differed significantly in their mental prognosis.

Birth Weight↗

PubMed1998

[Trigonocephaly: isolated, associated and syndromic forms. Genetic study in a series of 278 patients].

UNLABELLED: From a series of 1,833 patients with craniosynostosis, 278 cases with metopic synostosis were analysed. The prevalence of metopic synostosis was estimated in the region of 1 in 15,000 children. PATIENTS AND METHODS: All patients with metopic suture fusion were selected, excluding cases where additional sutures were involved. The age at diagnosis was between 15 days and 15 years. The diagnosis was based on clinical and radiological evaluation. The search for associated malformations was based on clinical evaluation, CT or MRI scans, bone X-rays and ultrasounds. If possible, a study of the karyotype was performed in case of associated malformation. Family information was obtained through contact with the families, generally in person or sometimes by telephone. The series was divided into three groups: isolated trigonocephaly (group 1), trigonocephaly associated with other malformations without any known syndrome (group 2) and well delineated syndromes (group 3). RESULTS: There were 213 males and 65 females, a sex ratio of 3.3. Family information was obtained from 222 probands distributed among 216 families. There was no maternal or paternal age effect. The frequency of twinning was 7.9%, with three concordances for metopic synostosis in three monozygotic twin pairs. A positive family history was obtained in 13 of the 216 pedigrees, giving a 6% figure of familial cases. A vertical transmission was observed in only one case; in all other cases, there were two affected children with normal parents. Eleven familial cases were isolated trigonocephalies, and two were syndromic. Nine of the 53 available karyotypes were abnormal, involving the chromosomes 7, 9, 11 or 13. There were 208 observations in the group 1. In 53 cases (group 2), associated malformations involved mainly the heart (12 cases), the limbs (six cases), the brain (five cases) and the genito-urinary tract (four cases). These malformations were unique in 32 cases and multiple in 21 cases. Some: of the observations could represent new syndromes. Seventeen syndromes represented group 3. Nine were chromosomal syndromes. Eleven presented with multiple malformations. An in utero exposure to valproic acid was observed in two cases of the group 1, five cases of the group 2 and one case of the group 3.

Abnormalities, Multiple↗

PubMed1998

Hydrocephalus and craniosynostosis.

OBJECT: A retrospective study of 1727 cases of craniosynostosis was undertaken to determine the interrelationship between abnormal cerebrospinal fluid (CSF) hydrodynamics and craniosynostosis. METHODS: The patients were divided into two groups: nonsyndromic craniosynostosis and syndromic craniosynostosis. Cases of occipital plagiocephaly without suture synostosis and cases of shunt-induced craniosynostosis were excluded from the study. The majority of patients (1297) were treated surgically for their cranial deformity; 95% of these patients had a postoperative follow-up review period lasting 5 years. Clinical and radiographic charts covering the time from presentation through the follow-up period were reviewed. CONCLUSIONS: Abnormal intracranial CSF hydrodynamics was found in 8.1% of the patients (3.4% of whom had received shunts and 4.5% of whom had not). Three types of CSF hydrodynamic disturbance were observed: progressive hydrocephalus with ventricular dilation, nonprogressive ventriculomegaly, and dilation of the subarachnoid spaces. Hydrocephalus occurred much more frequently in patients with syndromic craniosynostosis (12.1%) than in those with isolated craniosynostosis (0.3%). In fact, patients with kleeblattschädel exhibited hydrocephalus as a constant feature and patients with Crouzon's syndrome were far more likely to have hydrocephalus than those with other syndromes. In Apert's syndrome, ventricular dilation occurred very frequently, but it was almost always nonprogressive in nature. In most cases of syndromic craniosynostosis, venous sinus obstruction and/or chronic tonsillar herniation were found. Their role in the pathophysiology of hydrocephalus in craniosynostosis is discussed.

Acrocephalosyndactylia↗