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Prognosis for mental function in scaphocephaly

Eric Arnaud, Dominique Renier, and Daniel Marchac

✓ Three hundred ninety-six children with scaphocephalies were prospectively studied to analyze the correlation between age, intracranial pressure (ICP), and mental function outcome. The ICP measurements and the early and late psychometric assessments were compared. The influence of surgery, when performed, was analyzed. In most cases, the mental function outcome of the patients was good whether or not they had undergone surgery. The mental level and the frequency of increased ICP both correlated with patient age. A correlation was found between the early and late psychometric assessments in all patients. Thus, the main predictive factor of mental function outcome appears to be the initial developmental level.

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Evolution in the frequency of nonsyndromic craniosynostosis

Clinical article

Federico Di Rocco, Eric Arnaud, and Dominique Renier

Object

The prevalence of the different subtypes of craniosynostosis varies greatly. The aim of this study was to analyze the prevalences of the different subtypes of craniosynostosis at a single major craniofacial center and their changes during a 20-year period.

Methods

The medical charts of 2808 children hospitalized between 1988 and 2007 for a true craniosynostosis were retrospectively reviewed. Patients were divided according to their subtype of craniosynostosis (sagittal, plagiocephaly, brachycephaly, metopic, oxycephaly, syndromic cases, or unclassifiable).

Results

The total number of patients according to their craniosynostosis subtype were as follows: 1224 cases of scaphocephaly, 315 cases of plagiocephaly, 598 cases of trigonocephaly, 105 cases of brachycephaly, 69 cases of oxycephaly, 396 syndromic cases, and 101 unclassifiable cases. The prevalences of these craniosynostoses evolved differently over time. The number of children hospitalized each year increased over time from 106 to 181 cases, but this increase varied according to the subtype of craniosynostosis. Cases of scaphocephaly, plagiocephaly, brachycephaly, and syndromic cases increased similarly (170% increase on average), whereas trigonocephalies dramatically increased (420% increase).

Conclusions

The evolution observed in the prevalence of craniosynostosis might result from several factors. An increased recruitment of patients at the center and an improvement in the diagnosis of craniosynostosis might explain the overall increase. However, other mechanisms should be examined to explain the great increase in the number of cases of trigonocephaly (environmental or pharmacological).

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Fibroblast growth factor receptor 3 mutation in nonsyndromic coronal synostosis: clinical spectrum, prevalence, and surgical outcome

Dominique Renier, Vincent El Ghouzzi, Jacky Bonaventure, Martine Le Merrer, and Elizabeth Lajeunie

Object. A recurrent point mutation in the fibroblast growth factor receptor 3 gene that converts proline 250 into arginine has been reported recently in cases of apparently nonsyndromic coronal craniosynostosis. The goal of the present study was to examine the phenotype of patients in whom this mutation was present, to determine the prevalence of the condition, and to assess the functional and the morphological outcome of the surgically treated patients.

Methods. A DNA analysis was performed in 103 children suffering from apparently isolated coronal synostosis, 41 of whom had bilateral and 62 of whom had unilateral disease. There were 31 boys and 72 girls in the study group. Sixty cases were sporadic and 43 were familial; the 43 familial cases arose in 33 unrelated families. The mutation was found in seven (12%) of 60 sporadic cases and in 24 (73%) of the 33 families. The functional and morphological results were assessed in all surgically treated patients who had at least 1 year of follow up and who were at least 3 years of age at the time of assessment. A comparison was made between patients with the mutation and those without.

Conclusions. The most typical presentation was seen in girls and consisted of a bicoronal synostosis resulting in a severe brachycephaly associated with mild hypertelorism and marked bulging of the temporal fossae, which resulted in a huge enlargement of the upper part of the face. The most frequently associated extracranial anomaly was brachydactyly, identified either clinically or radiologically. Based on the proportion of bilateral and unilateral coronal synostoses, the present data indicate that the mutation is associated with more severe cases and that girls with the mutation are more severely affected than boys. The functional and morphological results were worse in patients in whom the mutation was present as compared with those in whom it was not.

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Dandy—Walker malformation associated with syringomyelia

Case illustration

Giuseppe Cinalli, Laurent Vinikoff, Michel Zerah, Dominique Renier, and Alain Pierre-Kahn

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Intracranial pressure in craniostenosis

Dominique Renier, Christian Sainte-Rose, Daniel Marchac, and Jean-François Hirsch

✓ In this study, intracranial pressure (ICP) was recorded with an epidural ensor for periods of 12 to 24 hours in 92 cases of craniosynostosis. Pre- and postoperative recordings were performed in 23 patients, and 55 children underwent preoperative psychometric testing. The ICP was found to be normal in one-third of the cases, was obviously elevated in one-third, and was borderline in one-third. Waves of increased ICP were recorded during rapid eye movement (REM) sleep. After surgery, ICP decreased progressively and returned to normal in several weeks. A significant statistical relationship was found between the patients' ICP and their mental level: the higher the ICP the lower the mental level. The regression curve of ICP as a function of age shows that ICP is maximal at the age of 6 years and decreases later. The significance of these results is discussed. The authors recommend that ICP be recorded in cases of craniosynostosis since it is of some help in deciding whether patients should undergo surgery.

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Brain abscesses in neonates

A study of 30 cases

Dominique Renier, Cécile Flandin, Elizabeth Hirsch, and Jean-François Hirsch

✓ Since the introduction of ultrasonography and computerized tomography (CT) scanning, brain abscesses are found more frequently in cases of neonatal meningitis and septicemia, particularly when the offending pathogen is Proteus. Thirty cases of brain abscess in neonates are reported, 27 of which were caused by Proteus species infections. Twenty infants had meningitis and 13 had septicemia. Most of the abscesses were enormous, and multiple abscesses were observed in 17 cases. The frontal region was involved in 22 cases (12 unilaterally and 10 bilaterally). The ventricles were enlarged on the first CT scan in 13 cases.

The abscesses were treated by aspiration and antibiotics in 25 cases, and by antibiotics alone in five. A shunt for hydrocephalus was necessary in 14 infants. Four infants died, three from the initial illness and one from a shunt complication. Sixteen children have seizures. Subsequent intelligence quotient (IQ) testing was performed in 22 children: eight (36%) have an IQ at or above 80 and eight have an IQ of less than 60. In the 17 children followed for more than 2 years, the proportion with an IQ at or above 80 fell to 24% (four cases). The absence of initial seizures, sterile cerebrospinal fluid, normal ventricles on CT scans, and early aspiration of the abscess seem to be factors portending a better prognosis in terms of epilepsy and mental sequelae.

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Genetic considerations in nonsyndromic midline craniosynostoses: a study of twins and their families

Elizabeth Lajeunie, Darach William Crimmins, Eric Arnaud, and Dominique Renier

Object

The origins of both sagittal synostosis (scaphocephaly) and metopic synostosis (trigonocephaly) remain unclear. Genetic and environmental factors probably play a role. Twin and family data of patients with these conditions are presented.

Methods

Records of 860 patients with scaphocephaly and 394 with trigonocephaly were examined for prevalence of twin birth and family history. Concordance rates of disease in monozygotic (MZ) and dizygotic (DZ) twins were compared.

A family history of disease was present in 5.7% of children with scaphocephaly and in 6.8% with trigonocephaly. The frequency of twins was 4.2% in sagittal synostosis and 6.8% in metopic synostosis, which is higher than in the normal population. Twenty-eight of 38 twin pairs with a scaphocephalic proband were identified to be DZ and 10 were confirmed to be MZ (MZ/DZ ratio 0.36). Twenty of 27 twin pairs with trigonocephaly were DZ and seven were MZ (MZ/DZ ratio 0.35). Concordance rates were higher in the MZ group, 30% for scaphocephaly and 43% for trigonocephaly (χ2 = 14.4091, p = 0.0001). There was a strong prevalence of boys, with a frequency of 79.1% with scaphocephaly and 75.8% with trigonocephaly. Twinning was more frequent among affected boys in both groups.

Conclusions

A midline craniosynostosis is more likely to develop in twins compared with singletons. A genetic component is supported by the higher risk in MZ twins. The presence of an environmental component is reinforced by the high rate of twinning, the normal MZ/DZ ratio, and a less than 100% concordance rate in MZ twins.

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Postoperative cognitive outcome for synostotic frontal plagiocephaly

Irene Mathijssen, Eric Arnaud, Elizabeth Lajeunie, Daniel Marchac, and Dominique Renier

Object

The goals of this study were to evaluate the cognitive outcome after surgery for synostotic frontal plagiocephaly and to compare the effects of early (< 1 year of age) and late (≥ 1 year of age) surgical correction on intellectual development.

Methods

In a prospective study involving 220 patients, both preoperative and postoperative cognitive function were measured using developmental quotient (DQ) or intelligence quotient (IQ) determination. Preoperative intracranial pressure (ICP) was also measured, and the presence of the fibroblast growth factor receptor 3 (FGFR3) P250R mutation was assessed.

Conclusions

Preoperative DQ/IQ was not affected by patient age at the time of surgery. There was no statistically significant difference in mean postoperative IQ between patients who underwent surgery before 1 year of age and those who underwent surgery at 1 year of age or older. In the latter group, there was a statistically significant increase in the incidence of elevated ICP (≥ 15 mm Hg in 16%), but no effect on cognitive outcome was observed. Likewise, there was no statistically significant correlation between the presence of the FGFR3 mutation and postoperative IQ. The indication for surgical correction appears to be largely cosmetic.

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Factors influencing the complication rate of subduroperitoneal shunt placement for the treatment of subdural hematomas in infants

Senta Kurschel, Stéphanie Puget, Marie Bourgeois, Michel Zerah, Petra Ofner, and Dominique Renier

Object

In this study the authors' goal was to identify the complication rate of subduroperitoneal (SDP) shunts for the treatment of subdural hematomas (SDHs) in infants and to determine the influences on and predictive factors for these complications.

Methods

The authors present a case series spanning the years 1994 to 2003 and include a statistical analysis of 161 children 2 years of age or younger with SDH who were treated using a unilateral valveless SDP shunt. The patient history, characteristics, and treatment methods including prior therapies, neuroimaging findings, and clinical outcomes were measures of evaluation.

Thirty-six children (22.4%) suffered complications related to SDP shunts: obstruction in 27 (16.8%), infections in eight (5%), disconnection in four (2.5%), migration in three (1.9%), wound complications (leakage and skin ulceration) in two (1.2%), and symptomatic subdural rebleeding in one (0.6%) necessitating bur hole evacuation. Seventeen children (10.6%) underwent placement of a second SDP shunt because of ipsilateral or contralateral persistent fluid collections, or premature shunt removal. With the exception of 12 patients (7.4%), shunt removal was performed systematically and resulted in the following minor complications in 30 children (18.6%): an adherent proximal catheter in 16 (9.9%), transient symptoms of intracranial hypertension in six (3.7%), subcutaneous cerebrospinal fluid accumulation in four (2.5%), local infections in three (1.9%), and hydrocephalus requiring placement of a ventriculoperitoneal shunt in one (0.6%).

Status epilepticus at presentation and neuroimaging findings such as areas of hyperdensity on computed tomography (CT) scans representing fresh blood in the subdural fluid collections before shunt insertion and at follow up were predictors of shunt-related complications. Correlations were also discovered for the following CT findings: ischemic lesions before shunt treatment, cerebral atrophy and ventricular dilation during the last follow up, and residual medium to large collections before shunt removal. Children who attained a good outcome were less affected by shunt-related complications, unlike those who presented with focal deficits and/or visual impairment.

Conclusions

Subduroperitoneal shunt placement for the treatment of SDH in infants is—despite the complication rate—an effective and often inevitable treatment option, especially for most large and symptomatic SDHs; a certain number of complications could be reduced with careful and precise surgical techniques. Close observation for detection of risks is mandatory, and seizure control is essential to prevent further brain injury that may result in large subdural fluid collections that are difficult to treat.

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Ependymoma in childhood: prognostic factors, extent of surgery, and adjuvant therapy

Marie-Lise C. van Veelen-Vincent, Alain Pierre-Kahn, Chantale Kalifa, Christian Sainte-Rose, Michel Zerah, John Thorne, and Dominique Renier

Object. The aim of this study was to investigate the effect of patient-related factors, extent of surgery, and adjuvant therapy on survival in children presenting with intracranial ependymoma.

Methods. Between 1980 and 1999, 83 children (mean age 36 months) underwent surgery for intracranial ependymomas. Complete resection, verified on postoperative computerized tomography scans, was achieved in 73%. Adjuvant therapy modalities have changed over the years: before 1990 all patients received radiotherapy, whereas after 1990 the children younger than 3 years of age and later those younger than 5 years of age were treated first with chemotherapy and received radiotherapy only after their first tumor recurrence. The follow-up period averaged 70 x 49 months.

Conclusions. Overall survival, intraoperative deaths excluded, was 73 ± 11% and 51 ± 14% at 5 and 10 years, respectively. The event-free survival rate at 5 and 10 years was 48 ± 12% and 46 ± 12%, respectively. Most of the events were local recurrences. Despite multiple reinterventions, the overall survival rate in this group dropped to 14%. On univariate analysis, the only significant prognostic factors were complete resection and radiotherapy. Both of these factors combined increased the 5- and 10-year survival rates to 93 and 75%, respectively. Age of the patients was not a statistically independent prognostic factor. The patients in the chemotherapy group did not fare as well as those in the radiotherapy group. A subgroup (36%) within the chemotherapy group, however, survived tumor free after a mean follow-up period of 67 months. It is not clear whether this subgroup either responded well to chemotherapy or needed no adjuvant therapy. Further research is warranted to answer this question.