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PubMed1998

[Cranial development after corrective skull surgery in craniosynostosis].

Craniofacial development may be potentially implicated after vault corrections in cases of craniosynostosis. The aim of this prospective study was the investigation of the surgical effect on calvaria growth, correlating the percentiles of a group of patients before and after craniosynostosis correction in relation to the normal percentiles of growth. The patient population consisted of 71 children; the postoperative follow-up time in 57 patients was more than 12 months. Of the children followed up, 36 were male and 21 female. Thirty-six cases involved untreated non-syndromic craniosynostosis; the remaining 21 were syndrome cases. The patient ages ranged from 16 to 27 months. In 11 cases correction of an occipital craniosynostosis was carried out. The fronto-occipital circumference was registered in accordance with Prader's percentile table. The operative method included a fronto-parietal craniotomy, the temporary removal and shaping of the fronto-orbital band and a vault cranioplasty. During follow-up 13 patients treated with fronto-orbital advancement in cases of isolated craniosynostosis remained in the registered percentile curve. In 13 patients a change to a lower percentile curve was confirmed; 1 patient changed to a higher curve. In cases of syndromic craniosynostosis 10 patients remained in the post-operatively registered percentile curve; 8 patients changed to a lower and 1 patient to a higher percentile curve. No growth restriction of the reshaped calvaria after surgical correction of craniosynostosis was observed. No significant difference was found in the potential calvaria growth between syndromic and isolated craniosynostosis.

Child, Preschool↗
PubMed1998

[Occipital correction in scapho- and plagiocephaly].

The operation technique and the results after occipital correction in children with plagiocephaly and scaphocephaly are reported. The indication for the intervention is provided by aesthetic and functional considerations. The outcome is very good; the growth of the skull follows the same range of percentiles as preoperatively and normal mental development is seen.

Cephalometry↗
PubMed2003

Ultrasound screening of the lambdoid suture in the child with posterior plagiocephaly.

BACKGROUND: The child with posterior plagiocephaly may have positional molding or unilateral lambdoid synostosis. Molding responds to conservative treatment, lambdoid synostosis requires surgical reconstruction. CT is diagnostic, but uses ionizing radiation, may need sedation, and the incidence of lambdoid fusion is only 2-3%. OBJECTIVE: The purpose of this prospective study was to evaluate ultrasound as a screening test of lambdoid sutural patency using CT as the reference standard. MATERIALS AND METHODS: In total, 41 children having head CT examinations were enrolled over 6 months. Of those, 29 were referred for abnormal head shape and suspected synostosis, of whom two had lambdoid fusion; 12 were referred for indications not related to head shape and found to have a normal study. Ultrasound scanning and interpretation of the lambdoid sutures was performed blinded to the CT reference standard. The lambdoid suture was read as patent or fused if a hypoechoic gap could or could not be seen between the hyperechoic calvarial bones, respectively. RESULTS: The mean sensitivity and specificity of ultrasound in distinguishing a patent from fused lambdoid suture by three blinded pediatric radiologists was 100% and 89%, respectively. CONCLUSIONS: Sonography of the lambdoid sutures shows excellent preliminary promise as a screening test of lambdoid sutural patency.

Cranial Sutures↗
PubMed2003

Antley-Bixler syndrome with radioulnar synostosis.

This report describes a newborn boy with Antley-Bixler (AB) syndrome. AB syndrome is a rare disorder characterized mainly by craniosynostosis and multiple joint contractures including radiohumeral synostosis. Our patient differs from the usual presentation by the presence of radioulnar rather than a radiohumeral synostosis. In addition, the child had a FGFR1 1300T mutation, which has not previously been associated with AB syndrome. Thus, our patient presents unique features and represents another argument in favour of an AB-like spectrum rather than a single syndrome.

Abnormalities, Multiple↗
PubMed2004

Cranial suture simulator for ultrasound diagnosis of craniosynostosis.

BACKGROUND: In evaluating the effectiveness of ultrasound as a screening tool for craniosynostosis it was discovered that sonologists and sonographers needed more experience scanning and visualizing cranial sutures on ultrasound. OBJECTIVE: To create an ultrasound simulator to train radiologists and technologists to locate and recognize patent and fused cranial sutures in children. MATERIALS AND METHODS: The hypoechoic appearance of patent sutures was simulated by cutting lines into life-sized plastic doll heads and filling them with a commercial hypoechogenic material. Fused hyperechoic sutures were simulated by not cutting into the hard plastic region of a suture. The simulator's teaching value was evaluated on three radiology residents and three fellows. Subjects performed pre-training scans on unknown simulators, received feedback and an opportunity to scan a training simulator, and then performed post-training scans on random unknown simulators. Accuracy was recorded as percentage of correctly demonstrated sutures. RESULTS: The suture simulator reproduces the sonographic appearance of patent and fused cranial sutures. Accuracy of acquisition, interpretation, and overall diagnosis increased from 64 to 91%, 79 to 91%, 61 to 97%, respectively, between pre and post training scans. CONCLUSION: An ultrasound simulator can reproduce the appearance of patent and fused cranial sutures in children and can be used to train radiologists and technologists in the performance of a screening protocol.

Cranial Sutures↗
PubMed2000

Three-dimensional spiral CT of craniofacial malformations in children.

OBJECTIVE: To assess the value of three-dimensional CT (3D CT) in the diagnosis and management of suspected paediatric craniofacial malformations. MATERIALS AND METHODS: Twenty-eight children (12 girls, 16 boys) with a mean age of 4 years, suffering from craniofacial or cervical malformations, underwent craniofacial spiral CT. 3D reformatting was performed using an independent work-station. RESULTS: 3D CT allowed the preoperative evaluation of 16 patients with craniosynostosis and the post-surgical management of 2 patients. 3D CT clearly depicted malformations of the skull base involving the petrous bone in seven patients (four cases of Goldenhar-Gorlin syndrome, one case of Treacher-Collins syndrome and two cases of Crouzon's disease). Four patients with craniofacial clefts were also evaluated. Radiological findings were confirmed by the clinical and intraoperative findings in all patients that underwent surgical treatment. Movement artefacts and "Lego effect" related to abrupt change of cranial vault border were encountered and are discussed. CONCLUSIONS: 3D CT of the skull can safely and reliably identify paediatric craniofacial malformations involving bone, and it should be used as morphological mapping to help the surgeon in planning surgical treatment.

Adolescent↗
PubMed1997

Short-limb skeletal dysplasias and craniosynostosis: what do they have in common?

Fibroblast growth factor receptor mutations cause some of the main short-limb skeletal dysplasias and craniosynostosis syndromes, including achondroplasia, hypochondroplasia, thanatophoric dysplasia, Apert syndrome, Crouzon syndrome, Pfeiffer syndrome, and Jackson-Weiss syndrome. Much work remains to be done in unraveling the pathogenesis of these phenotypes.

Bone Diseases, Developmental↗
PubMed1998

High-resolution sonography of the abnormal cranial suture.

OBJECTIVE: The purpose of this investigation is to elucidate the sonographic features of abnormal major cranial sutures. MATERIALS AND METHODS: Eight excised synostosed suture specimens were evaluated. The high-resolution sonographic appearance was correlated with the histological section, plain radiographs, CT and MRI. Diastatic and molded sutures were also evaluated with sonography and compared with the normal cranial suture appearance. RESULTS: Synostosed sutures demonstrated one or more of the following features: (a) loss of echo-poor fibrous gap between bony plates (five sagittal and coronal synostoses); (b) irregular thickened inner sutural margin (three lambdoid synostoses); (c) loss of bevelled edge (one lambdoid synostosis); (d) asymmetric anterior fontanelle (one coronal synostosis). Cranial molding results in an overlap of echogenic bony plates. Sutural width (the distance between bony plates) is increased in cases of elevated intracranial pressure. CONCLUSION: Sonography is an inexpensive, radiation-free modality which can confirm synostosis versus molding versus an underlying intracranial lesion as a cause of plagiocephaly. The high-resolution sonographic images also provide a relatively easy means to assess sutural width and may provide information in regard to increased intracranial pressure.

Cranial Sutures↗
PubMed1998

Autosomal recessive omodysplasia: report of three additional cases.

Three new cases of autosomal recessive omodysplasia (ARO) are reported. One shows a new finding of craniosynostosis. One is related to a patient previously reported in 1991. Another is the first report of a patient living in Australia. The clinical and radiological findings further consolidate the condition as a distinct and readily diagnosable autosomal recessive bone dysplasia with marked limb shortening and facial abnormalities. These cases bring the total reported to 17. Names used in earlier publications include rhizomelic bone dysplasia with club-like femora, familial generalised micromelia with dislocated radius and congenital micromelic dysplasia (Borochowitz type).

Abnormalities, Multiple↗
PubMed1998

Craniosynostosis 1998: concepts and controversies.

The diagnosis of craniosynostosis requires attention to radiologic technique, familiarity with the signs of closure at each suture, and correlation with the clinical setting. Misdiagnosis may result in unwarranted calvarial or craniofacial surgery or a delay of surgery, necessitating a more extensive procedure.

Child↗
PubMed2003

In utero models of craniofacial surgery.

Fetal surgery is finding ever-increasing applications. At present, fetal surgical techniques are used only to treat diseases associated with high mortality risk or severe morbidity if left untreated prior to birth. Yet as a discipline, in utero therapy is attractive to plastic surgeons because it affords the potential to (1) provide a scarless repair, (2) correct the primary deformity, (3) prevent secondary deformities, and (4) give the parents a "normal"-appearing child at birth. Each of these potential benefits is particularly important in patients with craniofacial anomalies. This article will both review the animal models that have been used to study the potential for intrauterine plastic surgery and provide a synopsis of the benefits that fetal therapy may provide in the treatment of selected craniofacial anomalies.

Animals↗
PubMed2002

A case of I-cell disease (mucolipidosis II) presenting with craniosynostosis.

CASE REPORT: In this paper, a patient with mucolipidosis II (I-cell disease) is described. The initial findings were microcephaly and metopic craniosynostosis. He had coarse facial features and dysostosis multiplex. The first child in his family had died with severe bone pathology at 5 months of age. CONCLUSION: The case is presented to emphasize that craniosynostosis may be the first symptom in mucolipidosis II.

Abnormalities, Multiple↗
PubMed2002

Sagittal synostosis in twin girls.

INTRODUCTION: Sagittal synostosis has been found to occur in families at a low frequency. All previous reports of sagittal synostosis in twins have been of males. CASE REPORT: We now report a case of female twins concordant for sagittal synostosis. CONCLUSION: Our hopes are that this information will further our understanding of the genetics of sagittal synostosis not only in single births but also in that of twins.

Cranial Sutures↗
PubMed2002

True 3D reconstruction for planning of surgery on malformed skulls.

OBJECTIVES: Since the difficulty associated with surgical planning of craniosynostosis is mostly due to the inadequate possibilities for simulation of surgery, a new technique for constructing a precise reproduction of a patient's malformed skull has been developed. MATERIALS AND METHODS: CT scans of ten malformed skulls on a scale of 1:1 and with 1-mm slices have been used to reconstruct the skulls in a special resin using a special lathe used in the automobile construction industry for formula one engines. This lathe works in the opposite way to other lathes: by apposition of material instead of subtraction. RESULTS: The anatomical detail of the phantom is of a very high degree. The surgical planning it allows has proved highly consistent with reality in all cases in which it has been used in the planning before the operation. DISCUSSION AND CONCLUSIONS: This technique has made it possible to plan the surgical treatment of all patients with craniosynostosis in a highly satisfactory way. It has reduced operating times, in addition to which it provides information on materials needed, avoiding waste, and is also an excellent teaching method for residents.

Craniosynostoses↗
PubMed2002

How to decrease the impact of surgical scar in the correction of sagittal synostosis.

INTRODUCTION. Sagittal synostosis can be surgically corrected by a variety of surgical procedures, all of them, however, burdened by a significant cosmetic impact owing to the visibility of the surgical scar. TECHNICAL NOTE. In this note, the author describes a surgical procedure that utilizes six strategically placed short scalp incisions instead of the traditional S-shaped or biparietal scalp incisions. Though still allowing effective correction of the malformative condition, the new procedure has only a minor cosmetic impact and leads to easier intraoperative and postoperative management of the patient. The procedure can be carried out using both standard or endoscopic techniques.

Cranial Sutures↗
PubMed2003

Fronto-orbital remodeling without orbito-naso-frontal bandeau.

INTRODUCTION: Fronto-orbital bilateral advance is the procedure of choice for the treatment of craniosynostosis affecting most of the anterior area of the skull and orbitomalar regions. The aim of the technique is to achieve a supra-orbital bilateral bar and a frontal bone. We have introduced a modification in order to simplify the technique. PATIENTS AND METHODS: From November 1998 to January 2002, 18 patients with craniosynostosis have been surgically treated using our technique. The mean age when the treatment was performed was 6.93 months (range 3 to 22 months). Brain computed tomography (CT) scans and three-dimensional (3-D) reconstruction of CT scans were performed before and after treatment. SURGICAL TECHNIQUE: A bifrontal craniotomy was performed taking the osteotomy up to the supraorbital rim. A new frontal bone was obtained from another region of the cranium creating new orbital edges. The osteosynthesis was conducted using absorbable materials. RESULTS: The follow-up of the patients ranged from 3 months to 3 years. All patients were studied using CT scans and 3-D reconstruction of CT after treatment, which demonstrated the persistence of the fronto-orbital advance. No secondary complications related to the new technique were found in any of the patients. CONCLUSIONS: The frontal-orbital advance obtained was stable. The technique was simplified by not creating a supraorbital bar and by reducing the bone fixation points. The manipulation of both frontal lobes and orbital globes was negligible. The aesthetic results were excellent.

Bone Remodeling↗
PubMed2004

Muenke syndrome.

BACKGROUND: Muenke syndrome is a genetically determined craniosynostosis that involves one or both coronal sutures. In some patients it is associated with skeletal abnormalities such as thimble-like middle phalanges, coned epiphysis, and/or neurological impairment, namely sensorineural hearing loss or mental retardation. In spite of a variable phenotype, Muenke syndrome has been related to a unique mutation on the FGFR3 gene, Pro 250 to Arg, which is characteristic of this disease. Because of the incomplete penetrance of this anomaly, the suspicion of Muenke syndrome must be raised in any child with uni- or bilateral coronal craniosynostosis, and the genetic analysis propounded even in the absence of extracranial features. ILLUSTRATIVE CASES: We report the cases of two sisters who presented with Muenke syndrome and whose affected mother did not have any form of craniosynostosis.

Arginine↗