Biomedical subjects
Publications and source records attributed to D Marchac.
The effectiveness of the "floating forehead" operation for treating brachycephaly in infants has been assessed. All children who had undergone this procedure at the Hôpital des Enfants Malades between 1977 and 1984 were reviewed retrospectively by an independent observer. Particular attention was paid to the effects on cranial growth, facial growth and morphology.
The functional and morphological aspects of craniosynostoses are reviewed, based on a series of 462 operations, 350 intra-cranial pressure recordings and 300 I.Q. assessments. Increased intracranial pressure and mental impairment are linked. The frequency of intracranial hypertension is higher when more than one suture is involved, but even monosutural craniosynostoses can be affected. Increased intracranial pressure had mental deficiency are more frequent after one year of age than before. Surgery lowers the intracranial pressure and halts the mental regression. The younger the patient is at the time of surgery, the better the results. Surgery does not improve the I.Q. once it is already impaired. Morphological results of frontocranial remodeling are good and remain so with growth. Facial anomalies associated with craniosynostoses improve after early frontocranial remodeling, except for the severe midface retrusion of Crouzon's disease and Apert's syndrome that need in most cases a supplementary midface advancement.
Result of a family study based on 584 patients with craniostenosis brings some answers useful for genetic counselling. For 98 patients (15%) a syndrome is associated. Third part of them has Apert syndrome, an other third part has Crouzon syndrome, and for the last third more exceptional acrocephalosyndactyly syndrome (Saethre-Chotzen, Pfeiffer) or others atypical associations, sometimes not yet described, but with an autosomal dominant inheritance. Non syndromic craniostenosis involves differently according to the type of join, but the localization is the same if recurrence will be happen. Coronal craniostenosis seems to be a dominant autosomal character, when scaphocephaly is more often sporadic; for both, an autosomal dominant inheritance is not excluded for some pedigrees. If the recurrence risk exist in some cases, it is generally well accepted by parents on account of the good neurosurgeon prognosis.
To obtain a shorter horizontal branch of the inverted T used in mammaplasties, the senior author has developed a technique based on a vertical skin resection stopping above the inframammary line. An evaluation of the results after 9 years of experience has been carried out in 176 patients. Results were satisfactory overall because the shape obtained was pleasing and stable in most cases; the horizontal scar remained hidden beneath the breast in the standing position. Modifications and refinements of the technique since its first publication in 1980 are discussed.
Craniosynotosis affects approximately one infant out of 1000. The increase of intracranial pressure and the risks of functional problems are more frequent than usually estimated, especially in monosutural synostosis. Frontocranial remodeling will correct both functional and aesthetic consequences of craniosynostosis. The best operative period is the first year of life, 2 to 3 months of age for the brachycephalies, and 6 to 9 months of age for the other craniosynostoses. Not only does growth not adversely affect the results of the forehead remodeling, but the adjacent orbitonasal areas improve with time. In Crouzon's disease and Apert's syndrome, early frontal advancement does not prevent the midface retrusion, and a radical frontofacial advancement may be indicated in very severe cases.
In early 1983 we developed a frontal pattern to aid in remodeling the forehead in craniosynostosis. We also designed a rigid wire to assist in checking the proper curvature of the supraorbital bar. In approximately 150 frontocranial remodelings the pattern has proved very useful and has simplified the procedure.
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The authors present a review of the most commonly observed ophthalmological troubles in 244 cases of children affected by cranio-facio stenoses and hospitalized for a first examination. The fundus examination shows that nearly all types of cranio-facio stenoses can be complicated by intracranial hypertension with potential damage on the optic nerve. The hyper and hypotelorisms, particularly frequent, carry along aesthetic prejudice and effect on the ocular motility. In the most severe forms of the diseases (Alport), pseudo-exophthalmy by retrusion of the face can justify preventive surgical procedures in emergency. Squint is frequent (36.5%). All types can be found. Finally, the authors certify the frequency and the seriousness of ocular repercussion, even in so called benign types of cranio-stenoses, such as plagiocephaly, trigonocephaly, and scaphocephaly.
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The capacity of the skin to stretch and expand, as shown in pregnancy, has been used by Radovan who, in 1976, introduced skin expanders made of silicone. These are empty balloons of different sizes and shapes which are surgically placed under the skin and gradually filled with saline. The saline solution is introduced through a valve with a syringe and a 21 G needle. Skin expansion usually requires 6 to 8 weeks to be completed. Transient redness of the skin is frequently observed. A thin capsule can be found around the expander but does not need to be removed. The excess skin can be utilized as advancement flap, rotation flap or even two-stage distant flap. Our experience is based on 33 expansions performed in 26 patients (table I). Giant naevi were the most frequent lesions treated (20 cases), and face, scalp and neck were the most frequent localizations (27/33 cases) (table II). Complications occurred in 21 p. 100 of the cases, and 5 expanders had to be removed. Nevertheless, expansion could be completed in most cases, sometimes after replacement of the prosthesis. The results of repair surgery using expanded skin have always been satisfactory: in colour and texture the expanded skin was similar to that of adjacent areas, and sensation was preserved. Skin expansion in fact made it possible to perform local flaps--which usually represent the best solution for skin replacement--by creating hyperlaxity in areas adjacent to the pathological areas. There has been no retraction of the expanded skin, and the scars have shown no tendency to hypertrophic reaction or distension.(ABSTRACT TRUNCATED AT 250 WORDS)
An evaluation of 220 cases of frontocranial remodeling for craniosynostosis is reported. The follow-up period was 10 years for children, 7 years for infants. The principles of the craniofacial techniques described in 1974 [4] and 1978 [3] were not modified; only variations were added. The floating forehead [5] concept has also remained basically unchanged. Intracranial pressure recordings have been used continually to help evaluate borderline indications and to assess results [9]. Our results show that 14% of cases of craniosynostosis affecting only one suture, usually considered as merely aesthetic problems, in fact were accompanied by increased intracranial pressure. The postoperative recordings have shown a return to normal pressures. Growth has not deteriorated the initially satisfactory results at the level of the forehead. At the midface level, improvement has been observed after surgery, especially for plagiocephalics. Their orbitonasal asymmetry has nearly completely disappeared. Patients with bilateral faciocraniosynostosis show an improvement of midface development compared with non-operated patients, but in severe cases an inverted bite still develops and further facial osteotomies remain necessary. The Apert's patients remain the most difficult to improve.
The abdominal wall of the infant presents a significant skin excess. Applying techniques of aesthetic abdominoplasty, it is possible to excise extensive abdominal wall lesions, as often seen in children with congenital hairy nevi. Three cases of congenital nevi were handled in this manner and are presented. One case involved the upper abdomen, while the other two involved the lower abdomen.
After 15 years of experience and 50 cases, we think that the axial frontonasal flap is of great value for the repair of large skin defects of the nose. This flap mobilizes all the skin cover of the nose located above the defect and the adjacent frontal skin and rotates it on a vascular pedicle existing at the level of the inner canthi. The excess of skin of the glabella is then transferred to the nose, and this large flap allows coverage of the defect without tension or distortion. The long-term results are very good, with a hardly visible repair in 26 of 50 patients, the long scar being very well hidden at the periphery of the nose.
Total removal of giant naevi with a minimal residual scar is of course ideal for prevention of malignancy and obvious psychological reasons. One can take advantage of the excess of skin existing in infants to be able to perform such wide excisions with immediate closure. Three cases of abdominal wall excisions in infants are presented, one of the upper abdomen at 9 months, two of the lower abdomen, at 3 months and 6 months. In all cases, a wide undermining was performed to allow the immediate closure. For the two lower abdominal excisions, preservation and transposition of the umbilicus were performed, like on an aesthetic abdominoplasty in adults. The blood loss was minimized by infiltration with epinephrine and meticulous hemostasis. Healing was uneventful. The scars, with a one to two years follow-up, are smooth and fine, and there are no change of contour. Progress in pediatric anesthesiology has resulted in the fact that a large excision and repair in infants presents no more potential dangers than operation performed later. Feasibility of such early wide excisions has been explored in other areas of the body; however, the abdominal wall is the area of choice.
Exophthalmos with lowering of the globe, eyelid enlargement, and canthal displacement are the usual consequences of orbitopalpebral neurofibromatosis. A cerebral hernia into the orbit may be seen on CT scan. An intracranial approach allows for pushing back the brain with a bone graft to create an enlarged orbital cavity, lifting the globe with a graft on the orbital floor, and fixing the canthi in proper position. An eyelid radical resection is performed in the same stage or later. This approach is discussed in the treatment of 17 cases of orbitopalpebral neurofibromatosis.
Exophthalmos with lowering of the globe, eyelid enlargement and canthal displacement are the usual consequences of orbitopalpebral neurofibromatosis. A cerebral hernia into the orbit may be seen on the CT scan. An intracranial approach allows for pushing back the brain with a bone graft to create an enlarged orbital cavity, lifting the globe with a graft on the orbital floor, and fixing the canthi in proper position. An eyelid radical resection is performed in the same stage or later. This approach is discussed in the treatment of 17 cases of orbito-palpebral neuro-fibromatosis.
A new technique in craniofacial surgery to separate the cranial and nasal cavities from each other is described. This can be achieved by preserving the complete anterior cranial fossa while simultaneously correcting the forehead, orbit, and face in craniofacial dysostosis. With this procedure, the risk of infection deriving from nasal sinuses and cavities should be minimized.
Our experience with a full-thickness running strip of abdominal skin for the treatment of large midline abdominal hernias has been very positive. The mechanical properties of the skin strip allow one to reestablish a normal anatomy by bringing the rectus muscle in contact. Clinical and histologic studies have shown that the full-thickness skin rapidly loses its epidermal components and appears to transform itself into a dense connective tissue very similar to normal aponeurosis.