Craniofacial surgery unit at Necker-Enfants malades hospital is an internationally renowned reference center. The work of the founders of the center, professors Renier and Marchac, have allowed to build the bases of current clinical and surgical management of these pathologies and to develop the research to understand their genetic causes.
Since its certification in 2006, the team has paid particular attention to multidisciplinary craniofacial care, associating in particular the craniofacial plastic surgery and pediatric neurosurgery teams.
The craniofacial surgery activity of the Necker-Enfants malades hospital is located within the pediatric neurosurgery department. It is a reconstructive surgery activity for neurosurgical complications.
One of the largest cohorts in the world (4,500 craniostenoses operated on) is followed in the unit, with more than 200 operations per year on an active file of 1,270 patients, with approximately 370 new patients. Approximately 1,200 consultations for these patients are provided each year, with approximately 35% of patients from outside the region and 15% of foreign patients. The number of annual interventions is constantly increasing, due to recruitment and iterative interventions on certain patients.
In case of emergency
The missions of the center are:
- to establish a diagnosis with certainty thanks to a specialized team;
- to offer personalized treatment based on multidisciplinary care;
- to train the parents of patients and their families;
- to prevent complications;
- to ensure continuity of care until adulthood;
- to develop clinical and fundamental research programs at national and international level;
- to study the pathophysiological and etiological mechanisms underlying these malformations;
- to develop new surgical techniques and tools;
- to disseminate knowledge and good medical practices;
- to develop a care network in collaboration with patient associations.
What is craniostenosis?
Craniostenosis is a primary growth defect in the cranial skeleton associated with the premature closure of one or more cranial sutures. There are various forms of craniostenoses without and with involvement of the facial mass or extremities (Crouzon, Pfeiffer, Saethre-Chotzen, Apert syndromes, etc.).
Craniostenoses pose a problem:
- morphological: cranial and facial dysmorphia
- functional: a growth conflict between skull and brain.
This can have mental, visual and psychological repercussions if the treatment is not early enough.
Sagittal craniostenosis (scaphocephaly)
Isolated scaphocephaly is a form of nonsyndromic craniosynostosis characterized by premature fusion of the sagittal suture.
Management and treatment
Metopic craniostenosis (trigonocephaly)
Isolated trigonocephaly is a nonsyndromic form of craniosynostosis characterized by the premature fusion of the metopic suture.
Management and treatment
Coronal craniostenosis (plagiocephaly)
Isolated synostotic plagiocephaly (SP) is a form of nonsyndromic craniosynostosis characterized by premature fusion of one coronal suture leading to skull deformity and facial asymmetry.
Management and treatment
Coronal craniostenosis (brachycephaly)
Isolated brachycephaly is a relatively frequent nonsyndromic craniosynostosis consisting of premature fusion of both coronal sutures leading to skull deformity with a broad flat forehead and palpable coronal ridges.
Management and treatment
A syndrome characterized by unilateral or bilateral coronal synostosis, facial asymmetry, ptosis, strabismus and small ears with prominent superior and/or inferior crus, among other less common manifestations.
Management and treatment
Crouzon disease is characterized by craniosynostosis and facial hypoplasia.
Management and treatment
An acrocephalosyndactyly associated with craniosynostosis, midfacial hypoplasia, hand and foot malformation with a wide range of clinical expression and severity. Most of the affected patients show various other associated manifestations.
Management and treatment
A frequent form of acrocephalosyndactyly, a group of inherited congenital malformation disorders, characterized by craniosynostosis, midface hypoplasia, and finger and toe anomalies and/or syndactyly.
Management and treatment
Multiple craniostenosis (Oxycephaly)
Isolated oxycephaly is a late-appearing form of nonsyndromic craniosynostosis characterized by premature fusion of both the coronal and sagittal sutures, and, in some cases, of the lambdoid sutures. Compensatory growth in the region of the anterior fontanel results in a pointed or cone-shaped skull.
Management and treatment
Non-syndromic unclassifiable craniostenoses
Various associations can be observed: scaphocephaly for example can be associated with plagiocephaly, trigonocephaly or lambdoid synostosis. This is not frequent.
Cloverleaf skull syndrome
A form of craniosynostosis involving multiple sutures (coronal, lambdoidal, sagittal and metopic) characterized by a trilobular skull of varying severity (frontal towering and bossing, temporal bulging and a flat posterior skull), dysmorphic features (downslanting palpebral fissures, midface hypoplasia, and extreme proptosis) and that is complicated by hydrocephalus, cerebral venous hypertension, developmental delay/intellectual disability and hind brain herniation.
Craniofacial clefts with hypertelorism
Craniofacial fibrous dysplasia
Nasal encephalocele is an extracranial herniation of intracranial contents (that maintain a connection to the subarachnoid space) into the fonticulus frontalis, presenting with nasal broadening and/or as a compressible, blue, pulsatile mass near the nasal bridge (that enlarges on crying or with jugular vein compression) or as an intranasal mass originating in the cribiform plate and that can cause nasal obstruction or respiratory distress. Hydrocephalus and increased intracranial pressure are also reported in some cases.
Dr Giovanna Paternoster
Dr Eric Arnaud
Dr Syril James
Dr Samer Haber
Dr Marie Bourgeois
Dr Hossein Khonsari
Ludivine de Freitas
The team of the craniostenoses reference center is based in the pediatric neurosurgery department.
There is a close collaboration with the specialists of the Necker-Enfants malades hospital. You will find here a non-exhaustive list.
- Dr Matthieu Robert
- Dr Abdelkader Belahda
- Dr Marie-Paule Morrisseau-Durand
- Pr Vincent Couloigner
- Pr Nicolas Leboulanger
- Dr Romain Luscan
Non-invasive ventilation and sleep unit for children
- Pr Brigitte Fauroux
- Dr Lucie Griffon
- Pr Arnaud Picard
- Dr Eva Galliani
- Dr Catherine Tomat
- Dr Sophie Eche
- Pr Valérie Cormier-Daire
- Dr Geneviève Baujat
- Dr Caroline Michot
- Dr Pauline Marzin
- Pr Nathalie Boddaert
- Dr Raphael Levy
- Delphine Crépin
Faciocraniostenosis is a group of rare diseases corresponding to a growth disorder of the skull associated with a facial growth deficiency. The care is complex and must take into account different functional problems, essentially neurosurgical during the first years of life, then visual, orbital, respiratory, and occlusal-dental with growth. Neurosurgical treatment is done in several sequences, with several cranial expansions at different ages.
The pediatric neurosurgery team now wishes to develop a therapeutic patient education (TPE) program to complete the care offer for patients followed in its reference center for faciocraniostenosis. For this reason, it has responded to a national call for projects launched by the Direction Générale de l’Offre de Soins (DGOS) of the Ministry of solidarities and health in October 2019 to develop educational actions in rare diseases.
The leader of the project entitled « accompanying craniofacial distraction in faciocraniostenosis » is Dr Giovanna Paternoster, pediatric neurosurgeon and deputy coordinator of the CRANIOST reference center
This TPE program will :
- improve patient and family knowledge about their disease and its care,
- explain the different steps of the surgery, the complications, the hospitalization and the return home,
- strengthen the autonomy of the patient and his family,
- prepare the child and his or her family for the significant physical changes caused by the surgery,
- reinforce concerted multidisciplinary support for the patient.
MDPH, AJPP, hospitalization, what should I do?
Taking care of the baby (Parents)
What is the Monobloc? (Parents)
Monobloc, what an ordeal? (Parents)
To explain is to reassure (Parents)
What about me? (Siblings-children)
What about me? (Siblings-teenagers)
What is the "Fort III"? (Parents)
The "Fort III", what an ordeal? (Parents)
What is the "Fort III"? (Children)
The "Fort III", what an adventure! (Children)
Everyone at home! (Parents)
Everyone at home! (Children)
- Simple midline craniosynostosis PNDS (under validation)
- Creation in November 2019 of the « Skull shape and growth » laboratory, affiliated with the Imagine Institute, with 1 M2, 3 PhD and a post-doc.
Modeling of craniofacial growth and evaluation of surgical results, thanks to a 350,000 euro grant from the Gueules Cassées foundation.
- Launch of a 3D printing platform to produce anatomical models and medical devices for craniofacial surgery at Necker hospital, based on the same funding.
- Coordination of the 3D printing policy for the AP-HP (mission led by C. Paugam), in conjunction with the agency for health equipment and products.
- Medical directorate of the Health Data Hub (national centralized medical database).
- « Emergence 2019 » obtained funding (Paris City Hall), 350,000 euros for the « Skull shape and growth » laboratory.
- Industrial study
- Evaluation of Kolibree (connected toothbrush) and Lunii (story box) on oral hygiene and perioperative anxiety in craniofacial malformation surgery – #2018A00758-47
- 3 PhD thesis projects in progress (starting Nov. 2019)
- Bone consolidation in FGFR mutants using mouse models, in collaboration with the Imagine Institute (A. Morice)
- Optimization of the shape of the forehead in fronto-orbital advances, in collaboration with Arts et Métiers (M. Geoffroy)
- Recognition of craniofacial syndromes by artificial intelligence, in collaboration with the Imagine Institute (Q. Hennocq)
- 1 M2 project in progress (starting Nov. 2020)
- Morphogenesis of the external ear and ear morphometry in craniofacial malformations, in collaboration with the Institut de la Vision (M. Cheval)
- 1 M2 project completed
- Modeling of frontal growth in trigonocephaly, in collaboration with Arts et Métiers (K. Bloch)
- Visiting professor at University College London (mechanical engineering laboratory): projects on biomechanical modeling of craniofacial growth in simple craniostenosis (Pr Moazen)
- Collaboration with Great Ormond St. Hospital (craniofacial surgery unit): projects on syndrome recognition by artificial intelligence
- Funding from the International Relations Department for a collaboration with the craniofacial surgery team of the Burdenko Institute (Moscow): 4 stays planned, 2 in Paris and 2 in Moscow (project postponed to 2021 because of COVID)
- Course for interns in maxillofacial surgery and neurosurgery in Île-de-France: management of faciocraniostenosis
- Courses at the IBODE School
- Craniofacial anatomy course at the Estienne school (technical and scientific drawing school)
- Participation in the teaching of the National Superior School of Beaux Arts: craniofacial malformations
- Participation in the teaching of the medicine and society course of the Ecole Normale Supérieure (Paris): craniofacial malformations
- Organization of the optional teaching unit « Representation of the body » (University of Paris) with a course on craniofacial malformations
- Organization of the optional teaching unit « Experimental Embryology » (University of Paris) with two courses on craniofacial development and its anomalies
- Organization of the optional teaching unit ‘Surgical Planning’ (University of Paris) with a course on 3D planning in craniofacial surgery
- Organization of the UD ‘Surgical planning and personalized medicine’ with several courses on 3D planning in craniofacial surgery
- Participation in the IUD of fetal medicine (University of Paris): midline abnormalities
- Development of 3D printed models for teaching craniofacial surgery to medical students (University of Paris) and interns (Île-de-France and national level): project financed by the ARS Île de France (72,000 euros) in collaboration with iLumens (simulation platform, University of Paris) and BONE 3D (start-up specialized in 3D printing)
- Development of an interface for teaching craniofacial anatomy and craniofacial surgery by virtual reality for medical students (University of Paris) and interns (Île-de-France and national level): in collaboration with iLumens (simulation platform, University of Paris) and Avatar Médical (start-up specialized in virtual reality)
- Forehead widening in nonsyndromic scaphocephaly operated after 12 months of age.
Paternoster G, Jing XL, Haber SE, James S, Legros C, Liu XX, Khonsari HR, Zerah M, Meyer P, Arnaud E.
J Craniofac Surg. 2021 Jan-Feb 01, PMID: 32804821 DOI: 10.1097/SCS.0000000000006860
- Sleep-disordered breathing in children with pycnodysostosis.
Khirani S, Amaddeo A, Baujat G, Michot C, Couloigner V, Pinto G, Arnaud E, Picard A, Cormier-Daire V, Fauroux B.
Am J Med Genet A. 2020 Jan, PMID: 31680459 DOI: 10.1002/ajmg.a.61393
- Extraocular muscle positions in anterior plagiocephaly: V-pattern strabismus explained using geometric mophometrics.
Touzé R, Heuzé Y, Robert MP, Brémond-Gignac D, Roux CJ, James S, Paternoster G, Arnaud E, Khonsari RH.
Br J Ophthalmol. 2020 Aug, PMID: 31694836 DOI: 10.1136/bjophthalmol-2019-314989
- Excessive ossification of the bandeau in Crouzon and Apert syndromes.
Bouaoud J, Hennocq Q, Paternoster G, James S, Arnaud E, Khonsari RH.
J Craniomaxillofac Surg. 2020 Apr, PMID: 32178948 DOI: 10.1016/j.jcms.2020.02.022
- The influence of fronto-facial monobloc advancement on obstructive sleep apnea: An assessment of 109 syndromic craniosynostoses cases.
Khonsari RH, Haber S, Paternoster G, Fauroux B, Morisseau-Durand MP, Cormier-Daire V, Legeai-Mallet L, James S, Hennocq Q, Arnaud E.
J Craniomaxillofac Surg. 2020 Jun, PMID: 32354613 DOI: 10.1016/j.jcms.2020.04.001
- Improvement of periorbital appearance in Apert syndrome after subcranial Le Fort III with bipartition and distraction
Chetty V, Haber SE, Khonsari RH, Arnaud E.
J Craniofac Surg. 2020 May/Jun, PMID: 32011541 DOI: 10.1097/SCS.0000000000006233
- Highlights of the proceedings from the 18th international congress of the international society of craniofacial surgery : ISCFS 2019, Paris, France.
Chen K, Bradley JP, Arnaud E, Mathijssen I.
J Craniofac Surg. 2020 May/Jun, PMID: 32102029 DOI: 10.1097/SCS.0000000000006309
- Strategy for bone conservation in the two-stage correction of hypertelorism in craniofrontonasal dysplasia.
Paternoster G, Haber SE, Britto JA, Benderbous D, James S, Legros C, Khonsari RH, Arnaud É.
J Craniofac Surg. 2020 Sep, PMID: 32833831 DOI: 10.1097/SCS.0000000000006585
- Secondary facial advancement in syndromic craniosynostoses: a prospective assessment of 109 cases.
Haber S, Khonsari RH, Leikola, J, Fauroux, B, Morisseau-Durand MP, Nowinski D, Arnaud E..
ISCFS 2019 Paris Abstract Supplement. Plast Reconstr Surg -GO Aug 2019, doi: 10.1097/01.GOX.0000582960.50147.f4
- Necker Experience of frontofacial monobloc advancement with distraction (FFMBA) about 145 cases in faciocraniosynostotic children
Arnaud E, Paternoster G, Khonsari RH, Haber S, Hennocq Q, James S, Morisseau-Durand MP, Fauroux B, Amaddeo A, Cormier-Daire V, Couloigner V, Diner P, Tomat C, Robert M, Meyer Ph.
ISCFS 2019 Paris Abstract Supplement, Plast Reconstr Surg -GO Aug 2019, doi: 10.1097/01.GOX.0000582748.92042.f6
- Defining critical ages for orbital shape changes after fronto-facial advancement in Crouzon syndrome
Khonsari RH, Hennocq Q, Nysjo J, Sandy R, Haber S, James S, Britto JA, Paternoster G, Arnaud E.
ISCFS 2019 Paris Abstract Supplement. Plast Reconstr Surg -GO Aug 2019, PMID: 31688760 DOI: 10.1097/PRS.0000000000006162
- Optic nerve stretching during fronto-facial Monobloc advancement for Crouzon syndrome.
Khonsari RH, Kogane N, Haber S, Paternoster G, James S, Arnaud E.
ISCFS 2019 Paris Abstract Supplement. Plast Reconstr Surg -GO Aug 2019, doi: 10.1097/01.GOX.0000583684.96300.fd
- 10 years review of “H Craniectomy” technique for sagittal synostosis in 436 patients, evaluation of surgical aspects, aesthetic and cognitive outcomes.
Paternoster G, Haber S, James S, Khonsari RH, Di Rocco F, Renier, D, Arnaud E.
ISCFS 2019 Paris Abstract Supplement, Plast Reconstr Surg -GO Aug 2019, doi: 10.1097/01.GOX.0000583180.22812.0c
- Fronto-facial monobloc advancement with distraction reduces severe obstructive sleep apnea in faciocraniosynostoses: prospective assessment of 109 cases.
Khonsari RH, Haber S, Paternoster G, Fauroux B, Morisseau-Durand MP, Leikola J, Hennocq Q, Viot-Blanc V, Guerin P, Baujat G, Collet C, Cormier-Daire V, Legeai-Mallet L, James S, Couloigner V, Tomat C, Diner P, Legros C, Meyer Ph, Arnaud E.
Plast Reconstr Surg -GO Aug 2019, doi: 10.1097/01.GOX.0000582956.73018.1a
- Effectiveness of distractor-mediated posterior vault expansion in complex craniosynostosis
Black J, Paternoster G, Haber S, Khonsari H, James S, Taylor D, Arnaud E.
ISCFS 2019 Paris Abstract Supplement. Plast Reconstr Surg -GO Aug 2019, doi: 10.1097/01.GOX.0000582860.91149.b7
- Cerebral perfusion in simple craniosynostosis using arterial spin labeling
Caudron Y, Paternoster G, Levy R, Haber S, Khonsari RH, Grevent D, Arnaud E, Boddaert N. MRI.
ISCFS 2019 Paris Abstract Supplement. Plast Reconstr Surg -GO Aug 2019, doi: 10.1097/01.GOX.0000582800.39570.17
- Transfacial external traction and internal distraction for young and severe faciocraniosynostoses: the very early monobloc
Arnaud E, Antunez S, Khonsari RH, Haber S, Paternoster G, James S, Meyer Ph.
ISCFS 2019 Paris Abstract Supplement. Plast Reconstr Surg -GO Aug 2019; doi: 10.1097/01.GOX.0000582752.99666.87
- Multi-stage preparation for the repair of complicated skull defects.
Sakamoto Y, Arnaud E.
Neurol Med Chir (Tokyo). 2019 May 15, PMID: 30867358 PMCID: PMC6527967 DOI: 10.2176/nmc.oa.2018-0283
- Defining critical ages for orbital shape changes after frontofacial advancement in Crouzon syndrome.
Khonsari RH, Hennocq Q, Nysjö J, Sandy R, Haber S, James S, Britto JA, Paternoster G, Arnaud É.
Plast Reconstr Surg, 2019 Nov, PMID: 31688760 DOI: 10.1097/PRS.0000000000006162
- Anterior skull base and pericranial flap ossification after frontofacial monobloc advancement.
Morice A, Paternoster G, Ostertag A, James S, Cohen-Solal M, Khonsari RH, Arnaud E.
Plast Reconstr Surg. 2018 Feb, PMID: 29036029 DOI: 10.1097/PRS.0000000000004040
- Dental consequences of pterygomaxillary dysjunction during fronto-facial monobloc advancement with internal distraction for Crouzon syndrome.
Sicard L, Hounkpevi M, Tomat C, James S, Paternoster G, Khonsari RH, Arnaud E.
J Craniomaxillofac Surg. 2018 Sep, PMID: 29970285 DOI: 10.1016/j.jcms.2018.06.003
- Bilambdoid and sagittal synostosis: Report of 39 cases.
Chivoret N, Arnaud E, Giraudat K, O’Brien F, Pamphile L, Meyer P, Renier D, Collet C, Di Rocco F.
Surg Neurol Int. 2018 Oct 11, PMID: 30386676 PMCID: PMC6194734 DOI: 10.4103/sni.sni_454_17
- Impact of extra-axial cerebrospinal fluid collection in frontal morphology after surgical treatment of scaphocephaly.
Nicolini F, Arnaud E, Usami K, Vecchione A, Brunelle F, Di Rocco F.
Surg Neurol Int. 2018 Oct 30, PMID: 30505617 PMCID: PMC6219275 DOI: 10.4103/sni.sni_13_18
- Results and limits of posterior cranial vault expansion by osteotomy and internal distractors.
Di Rocco F, Usami K, Protzenko T, Collet C, Giraudat K, Arnaud E.
Surg Neurol Int. 2018 Oct 30, PMID: 30505619 PMCID: PMC6219288 DOI: 10.4103/sni.sni_465_17
- Pattern of closure of skull base synchondroses in Crouzon syndrome.
Coll G, Sakka L, Botella C, Pham-Dang N, Collet C, Zerah M, Arnaud E, Di Rocco F.
World Neurosurg. 2018 Jan, PMID: 29024761 DOI: 10.1016/j.wneu.2017.09.208
- Multi-polarization reconfigurable antenna for wireless biomedical system.
Wong H, Lin W, Huitema L, Arnaud E.
IEEE Trans Biomed Circuits Syst. 2017 Jun, PMID: 28129184 DOI: 10.1109/TBCAS.2016.2636872
- Clinical delineation of a subtype of frontonasal dysplasia with creased nasal ridge and upper limb anomalies: Report of six unrelated patients.
Lehalle D, Altunoglu U, Bruel AL, Arnaud E, Blanchet P, Choi JW, Désir J, Kiliç E, Lederer D, Pinson L, Thauvin-Robinet C, Singer A, Thevenon J, Callier P, Kayserili H, Faivre L.
Am J Med Genet A. 2017 Dec, PMID: 29136349 DOI: 10.1002/ajmg.a.38490
- Clinical and neuroradiological features of the 9p deletion syndrome.
Spazzapan P, Arnaud E, Baujat G, Nizon M, Malan V, Brunelle F, Di Rocco F.
Childs Nerv Syst. 2016 Feb, PMID: 26597681 DOI: 10.1007/s00381-015-2957-2
- Unilateral coronal synostosis: can we trust the sagittal suture as a landmark for the underlying superior sagittal sinus?
Protzenko Cervante T, Arnaud E, Brunelle F, Di Rocco F.
J Neurosurg Pediatr. 2016 May, PMID: 26745648 DOI: 10.3171/2015.8.PEDS15117
- Cerebrospinal fluid collections in sagittal suture synostosis.
Usami K, Nicolini F, Arnaud E, Di Rocco F.
Childs Nerv Syst. 2016 Mar, PMID: 26767840 DOI: 10.1007/s00381-015-3011-0
- Multiple delayed scalp reconstruction for complicated cranial defects.
Sakamoto Y, Arnaud E.
Plast Reconstr Surg Glob Open. 2016 Aug 11, PMID: 27622104 PMCID: PMC5010327 DOI: 10.1097/GOX.0000000000000862
- The « Les p’tits courageux » association brings together parents and patients with monosutural and syndromic craniostenoses (Crouzon, Pfeiffer, Apert, Saethre-Chotzen …).
- The « Epi Têtes » association supports families and children with non-syndromic craniostenoses (scaphocephaly, trigonocephaly, plagiocephaly, brachycephaly, oxycephaly …).
- The « Tête en l’air » association is mobilizing for families whose children have been operated and followed in neurosurgery.
In Necker, the reference center for craniostenoses and craniofacial malformations in brief …
* data valid for 2019