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PubMed1977

Cephalometric observations in premature craniosynostosis.

Forty patients with premature craniosynostosis of variable extent and severity were subjected to cephalometry. The majority of the children and adolescents involved had undergone bilateral cranial fenestration at age 6 months to 11 years. Lateral cephalometric radiographs were used for analysis. The measurements obtained were compared with normal values of comparable age groups. In two thirds of the patients examined facial skull growth was found to be impaired, the clinical appearance ranging from pronounced faciostenosis to abortive forms. In some cases the clinical appearance was normal. One of the conclusions derived from the cephalometric data is that inhibited growth primarily affects the anterior portions of the skull base, which are found to be shorter than normal. The defect appears to involve the spheno-ethmoidal, intersphenoidal and sphenofrontal synochondroses.

Cephalometry↗

PubMed1979

Syndromology's message for craniofacial biology.

Syndromology is a misunderstood specialty that has much to contribute to the understanding of cranio-facial biology in general and the study of craniofacial anomalies in particular. An introduction to the practice of syndromology and the rudiments of syndrome delineation is presented. The aetiology and pathogenesis of selected craniofacial anomalies (Robin complex, hemifacial microsomia, and craniosynostosis) are considered from the perspective of syndromology.

Chromosomes↗

PubMed1980

Methylmethacrylate onlay implants in the treatment of forehead deformities secondary to craniosynostosis.

Craniosynostosis (Cruzon's, Pfeiffer's and Apert's Syndrome, etc.) can cause forehead deformities that frequently need reconstruction. In general, osteotomies and/or bone grafting are the preferred methods for obtaining proper orbital protection from above and a good aesthetic appearance. In certain cases however, osteotomies and/or bone grafting are not possible or a patient may refuse to have such an extensive procedure. Preformed methylmethacrylate prostheses have in such cases been implanted in patients with significant skull deformities with only minimal morbidity. The ease of this technique coupled with the shortened operative time, minimal hospitalization, and few, minor complications offer the reconstructive surgeon another and relatively simple method for treatment of extensive forehead and supraorbital deformities.

Adolescent↗

PubMed1984

Oculo-motor disorders in craniofacial malformations.

Based on 77 observations including telorbitism, craniofacial stenosis (C.F.S.), and plagiocephalies, oculo-motor disorders involving horizontal and vertical imbalance occur very frequently. Abnormalities in the transverse plane (telorbitisms, craniofacial stenoses) give orbital divergence and exotropia. Orbital extorsion with a significant antimongoloid palpebral fissure, may be the cause of rectus muscle extorsion. Abnormalities in the vertical plane (plagiocephalies) induce hypertropia. Abnormalities in the sagittal plane (C.F.S.) lead to exotropia with exorbitism, the V syndrome with double up-shoot. These oculo-motor disorders seem to be the consequence of these orbital structure abnormalities, sometimes combined with sensory factors.

Acrocephalosyndactylia↗

PubMed1977

Craniosynostosis: a new operative technique.

In the treatment of craniosynostosis several methods have been proposed to prevent craniectomies from early reclosure. New bone is formed at the edge of the craniectomy, but particularly at the outer surface of the dura. The author has developed a method to separate the edges from each other and to stop osteogenesis inside the craniectomy. The outer layer of the dura is dissected free from the inner layer folded over the edge of the groove and sutured to the outer periosteum. The operative technique and the results in 40 patients with a follow up of 17 years are described.

Child↗

PubMed1997

A 19-week-old fetus with craniosynostosis, renal agenesis and gastroschisis: case report and differential diagnosis.

A case of a fetus affected with craniosynostosis, unilateral renal agenesis and gastroschisis is reported. The propositus was delivered on the 19th week of gestation for premature rupture of the membranes. Macroscopy showed turricephaly, shallow orbits, exophthalmos, hypertelorism, hypoplastic maxilla with relative mandibular prognathism and gastroschisis. Additional autopsy findings included a premature bilateral closure of the lambdoid suture and a unilateral renal agenesis. The nosological aspects of this fetus and the differential diagnosis of well-described craniosynostosis syndromes with characteristic craniofacial growth patterns (Crouzon syndrome, Jackson-Weiss syndrome, Apert syndrome, Saethre-Chotzen syndrome, Pfeiffer syndrome) are discussed.

Abdominal Muscles↗

PubMed2001

Standard variant venous dysplasia of the cerebellum in a patient suffering from Muenke's syndrome and tinnitus.

BACKGROUND: Muenke's syndrome is a rarely seen craniosynostosis syndrome. Bilateral or unilateral coronal craniosynostosis, brachydactyly, tarsal and carpal coalitions are the typical clinical findings. Sensorineural hearing loss is reported in more than 30% of the affected patients. In patients with Muenke's syndrome intracranial vascular dysplasia has not been reported yet. PATIENT AND METHODS: A 13-year-old boy suffering from Muenke's syndrome reported a pulsating left-sided light tinnitus. Clinically a bluish formation was found at the lower part of the left-sided ear drum. In order to clarify the cause of the tinnitus and the bluish formation, extensive diagnostics (e.g. audiometry, magnet resonance tomography, magnet resonance phlebography, angiography) were carried out. RESULTS: Audiometry found a left sensorineural hearing loss with a conductive component. Radiological investigations found a standard variant venous dysplasia of the left petrous bone to be the reason for the tinnitus. CONCLUSION: Awareness of intracranial vascular dysplasia should be noted in individuals with Muenke's syndrome especially in cases of clinically suggestive symptoms like pulsating tinnitus.

Adolescent↗

PubMed2000

[Evaluation of transfusion strategies during craniosynostosis repairs].

OBJECTIVE: To evaluate the changes in transfusion practices during craniosynostosis surgery in children, with consideration of the transfusion-associated risks. STUDY DESIGN: Retrospective study. PATIENTS: The study included 64 consecutive craniosynostosis repairs by the same neurosurgical and anaesthetic team, over a period of 17 years. METHODS: The children were allocated into two groups. In group I (1980-1991), blood loss was compensated precisely from scalp incision on. In group II (1992-1996), transfusion was only started when blood loss crossed a calculated limit considered as acceptable. The mean criterion of judgment was the quantity of transfused blood in comparison with the theoretical blood volume. Mean preoperative and postoperative haemoglobin concentrations, estimated blood losses, volume of replaced blood and number of transfused patients were compared between the two groups with a two tailed Student's t test. RESULTS: The rate of non transfused children increased from 6% before 1991 to 39% after 1992%; conservely the rate of postoperative transfusions increased from 3 to 39%. CONCLUSION: The risks of blood transfusion favoured the development of new of more restrictive transfusion practices, even in haemorrhagic surgery. To limit blood transfusion in craniosynostosis surgery, a harmonious cooperation between surgeons and anaesthetists is essential and blood replacement must be based on a definite determination of the acceptable blood losses.

Blood Loss, Surgical↗

PubMed2001

[Acute hemolysis following perioperative blood salvage in chldren operated for the surgical correction of craniosynostosis].

We report the case of a 6-month-old child, who suffered from acute haemolysis following transfusion of salvaged blood. This complication, of favourable outcome, was related to the accidental aspiration of benzalkonium chloride into the cell saver. This case emphasizes that any adjunction of antiseptic solution is contraindicated during blood saving. The use of a cell saver must be associated with written protocols, describing clearly the contraindications, precautions of use, and the different steps of use of this method of autologous blood transfusion.

Acute Disease↗

PubMed2002

[Perioperative management of blood loss during surgical treatment for craniosynostosis].

Blood saving is the major challenge during the surgical repair of craniofacial deformities. Treated patients have a low reserve volume and the techniques available to lower homologous blood transfusions are limited or insufficiently evaluated in this particular case. The most important factor determining blood loss is the quality of the surgical haemostasis. Blood saving begins with early preoperative evaluation of the patient's bleeding risk, which is a function of the type of surgery, of the surgical technique, of the number of sutures involved, of the length of surgery, and of the patients age, weight and physical status. Elaborated blood saving techniques such as preoperative autologous blood donation, erythropoietin administration, normovolaemic haemodilution, and peroperative autologous blood saving and reinfusion have revealed disappointing where used alone. These techniques require a heavy setup and still need to be evaluated extensively. They should be used in selected cases such as in patients with a very high risk of bleeding or face to Jehovah Witnesses. Monitoring during surgery should include precise evaluation of blood losses and haematocrit measurements at regular intervals. The haematocrit threshold allowing homologous blood transfusion should be set at 21%, provided that any other source of autologous blood is exhausted. Postoperative monitoring should also include precise evaluation of blood losses and haematocrit measurements. The 21% threshold should remain the reference during that period.

Blood Loss, Surgical↗

PubMed2002

[Anesthetic management for craniosynostosis].

Surgical procedures for correction of craniosynostosis are performed in young infants with a small blood volume and represent major surgery with extensive blood loss. An accurate determination and a precise restoration of blood losses represent the major concern for the anaesthetist during this surgery. The preoperative assessment of these patients is usually simple, except in the cases where the craniosynostosis is associated with other congenital malformations. The anaesthetist should keep in mind that intracranial hypertension may be associated with craniosysnostosis, which modify the anaesthetic management, especially the induction of anaesthesia. Even though the psychological impact of a craniosynostosis should be taken into consideration, surgery is most often indicated for functional considerations, therefore parents should be informed of the risks related to the procedure. During the postoperative period the major concerns are related to the possibility of a persistent bleeding, which usually decreases and disappears over the first 12 hours.

Anesthesia↗

PubMed1998

Rapid palatal expansion. Part 2: Dentoskeletal changes in cats with patent versus synostosed midpalatal suture.

Intercanine expansion (C-C) following rapid palatal expansion is made up of sutural displacement (Sd-Sd), tooth tip (Tt-Tt), tooth displacement (Td-Td), and alveolar process tipping and bending (At+b-At+b). The involvement of these four components was studied on 10 rapid palatal expansion treated and two control cats during an active phase (25 days), a retention phase (60 days), and a relapse phase (60 days). The midpalatal suture was analyzed for linear measurements, radiopaque versus radiolucent zones and optical density from occlusal radiographs. Nine treated cats exhibited sutural split and one treated cat showed no split as a result of synostosis of the suture. The contribution of the four constituents [(Sd-Sd):(Tt-Tt):(Td-Td):(At+b-At+b)] to the C-C expansion changed from active to relapse phase from [45%:15%:25%:15%] to [50%:25%:25%:0%] in the animals with sutural split and from [0%:40%:60%:0%] to [0%:0%:100%:0%] in the cat without sutural split, implying the major role of sutural displacement in patent suture, and tooth displacement in synostosed suture. The latter indicates the potential buccal corticalis fenestration, dehiscence or perforation in synostosed suture undergoing RPE. In patent suture (animals with sutural split), optical density increased during rapid palatal expansion (soft tissue build-up) and decreased during retention (remineralization) and relapse phases (medial convergence of the palatal processes). In the animal without sutural split, a continuous decrease in the optical density (predetermined ossification) was found. The progressive six-fold surge in coefficient of variation of C-C expansion during the relapse phase indicates limitation in predicting rapid palatal expansion stability. Clinically, the use of serial occlusal radiographs during rapid palatal expansion is recommended to evaluate patency and extent of retention period.

Animals↗

PubMedDate not supplied

Rapid protyping technology in medicine--basics and applications.

Using medical models built with Rapid Prototyping (RP) technologies represents a new approach for surgical planning and simulation. These techniques allow one to reproduce anatomical objects as 3D physical models, which give the surgeon a realistic impression of complex structures before a surgical intervention. The shift from the visual to the visual-tactile representation of anatomical objects introduces a new kind of interaction called 'touch to comprehend'. As can be seen, from the presented case studies of maxillo-cranio-facial surgery, the RP models are very well suited for use in the diagnosis and the precise preoperative simulation of skeleton modifying interventions.

Acrocephalosyndactylia↗

PubMed1992

New indirect method for estimating the birth prevalence of the Apert syndrome.

An indirect method for estimating the birth prevalence of the Apert syndrome is presented. The fraction of Apert syndrome patients in large clinical surveys of all cases of craniosynostosis is calculated and the fractional component obtained is multiplied by the known birth prevalence of craniosynostosis in general. Apert syndrome makes up approximately 4% of all cases of craniosynostosis. Using a weighted average estimate, birth prevalence was calculated to be 13.7/1,000,000. The results of the indirect method compare favorably with those obtained by the direct method. Nevertheless, because the indirect method is based on a number of assumptions that are easily violated, we cannot recommend its general use except under special conditions.

Acrocephalosyndactylia↗

PubMed1993

Facial scoliosis--a diagnostic dilemma.

Facial scoliosis may be caused by frontal plagiocephaly or muscular torticollis. Determining the correct cause has implications for patient management. Past confusion in the literature has led to difficulty in determining appropriate treatment plans. The correct diagnosis may be readily obtained by using a series of clinical and radiological features peculiar to each condition. Four cases are shown demonstrating these diagnostic features.

Adolescent↗

PubMed2001

[Metopic craniosynostosis, probable effect of intrauterine exposure to maternal valproate treatment].

UNLABELLED: Metopic craniosynostosis may be an adverse effect of valproic acid exposed fetus. CASES: We report two infants with metopic craniosynostosis, born to mothers who were treated with valproic acid. In one case, a prenatal diagnosis was made. In the other case, only the male dizygotic twin was affected. CONCLUSION: Trigonocephaly may be a symptom of valproate embryofoetopathy detectable by antenatal ultrasound examination.

Abnormalities, Drug-Induced↗

PubMed2003

[Clinico-radiological study of the skull deformation in the plagiocephaly without synostosis].

UNLABELLED: The aim of this study was to analyze the mechanism of the skull deformation observed in frontal and occipital plagiocephaly without synostosis. POPULATION AND METHODS: From 1987 to 2001, 96 plagiocephalies without synostosis, 72 males and 24 females, were examined. There were 72 Frontal Plagiocephalies (FP) and 24 Occipital Plagiocephalies (OP). The clinical examination of the neck muscles was performed to find out a torticolis with or without retraction. All patients underwent a 3D CT scan of the skull to exclude a craniostenosis. Cephalic Index (CI) was also calculated. RESULTS: The FP had a torticollis in 89.3% of cases associated with a muscle retraction (54.8%), a tonus asymmetry (16.6%), or in association (28.6%). The skull had a parallelepiped shape and the greater axis was oblique. The CI was 0.85. The OP had a torticollis in 50% of case associated with a retraction (28.6%), a tonus asymmetry (57.1%), or the association (14.3%). The skull had a square shape with an occipital flat and the CI was 0.95. DISCUSSION: The FP and OP are two deformations whose mechanism is different. The FP corresponds to a three plans skull asymmetry secondary to muscle traction on the base of the skull, and the OP corresponds to a posterior localized asymmetry of the skull secondary to compression. CONCLUSION: The traction and/or compression deformation start in utero or in the newborn period and the supine position is an aggravating positional factor.

Anthropometry↗

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↗