Skip to main content

Advertisement

SpringerLink
Log in

Cloverleaf skull deformity and hydrocephalus

  • Special Annual Issue
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Objective

This article describes the clinical aspects for both operated and non-operated patients with a cloverleaf skull deformity treated in our service, focusing on hydrocephalus.

Methods

We describe 13 cases of cloverleaf skull deformity treated in our services between 1977 and 2008. Among them, ten were operated (9 out of 13 for the craniofacial stenosis and 7 out of 13 for hydrocephalus).

Results

Hydrocephalus was present in all patients with bilateral lambdoid stenosis. There was no case of hydrocephalus among the patients with unilateral or absent lambdoid stenosis. Associated malformations and severe faciostenosis were associated with higher mortality and morbidity.

Conclusion

The development of hydrocephalus seems to be closely related to a bilateral lambdoid stenosis. The optimal treatment must be tailored individually considering the degree of the malformation and the presence of complications and comorbidities.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. Cohen MM Jr (2005) Editorial: perspectives on craniosynostosis. Am J Med Genet A 136A:313–326

    Article  PubMed  Google Scholar 

  2. Holtermueller K, Wiedemann HR (1960) The clover-leaf skull syndrome. Med Monatsschr 14:439–446

    PubMed  CAS  Google Scholar 

  3. Cohen MM, MacLean RE (2000) Craniosynostosis: diagnosis, evaluation, and management. Oxford University Press, New York

    Google Scholar 

  4. Robin NH, Falk MJ, Haldeman-Englert CR (1993) FGFR-related craniosynostosis syndromes. In: Pagon RA, Bird TD, Dolan CR, Stephens K (eds) GeneReviews. University of Washington, Seattle

    Google Scholar 

  5. Bonucci E, Nardi F (1972) The cloverleaf skull syndrome. Histological, histochemical and ultrastructural findings. Virchows Arch A Pathol Pathol Anat 357:199–212

    Article  PubMed  CAS  Google Scholar 

  6. O’Broin ES, O’Keefe M, Allcutt D, Earley MJ (1997) Cloverleaf skull anomaly with extreme orbitostenosis. J Craniofac Surg 8:75–77

    Article  PubMed  Google Scholar 

  7. Oyamada MK, Ferreira HS, Hoff M (2003) Pfeiffer syndrome type 2—case report. Sao Paulo Med J 121:176–179

    Article  PubMed  Google Scholar 

  8. Goodrich JT (2005) Skull base growth in craniosynostosis. Childs Nerv Syst 21:871–879

    Article  PubMed  Google Scholar 

  9. Witt PD, Hardesty RA, Zuppan C, Rouse G, Hasso AN, Boyne P (1992) Fetal kleeblattschadel cranium: morphologic, radiographic, and histologic analysis. Cleft Palate Craniofac J 29:363–368

    Article  PubMed  CAS  Google Scholar 

  10. Lodge ML, Moore MH, Hanieh A, Trott JA, David DJ (1993) The cloverleaf skull anomaly: managing extreme cranio-orbitofaciostenosis. Plast Reconstr Surg 91:1–9, discussion 10–14

    Article  PubMed  CAS  Google Scholar 

  11. Hayward R (2005) Venous hypertension and craniosynostosis. Childs Nerv Syst 21:880–888

    Article  PubMed  CAS  Google Scholar 

  12. Taylor WJ, Hayward RD, Lasjaunias P, Britto JA, Thompson DN, Jones BM, Evans RD (2001) Enigma of raised intracranial pressure in patients with complex craniosynostosis: the role of abnormal intracranial venous drainage. J Neurosurg 94:377–385

    Article  PubMed  CAS  Google Scholar 

  13. Partington MW, Gonzales-Crussi F, Khakee SG, Wollin DG (1971) Cloverleaf skull and thanatophoric dwarfism. Report of four cases, two in the same sibship. Arch Dis Child 46:656–664

    Article  PubMed  CAS  Google Scholar 

  14. Zuccaro G, Dogliotti P, Bennum R, Monges J (1996) Treatment of cloverleaf skull syndrome. Childs Nerv Syst 12:695–698

    Article  PubMed  CAS  Google Scholar 

  15. Resnick DK, Pollack IF, Albright AL (1995) Surgical management of the cloverleaf skull deformity. Pediatr Neurosurg 22:29–37, discussion 238

    Article  PubMed  CAS  Google Scholar 

  16. Renier D, El-Ghouzzi V, Bonaventure J, Le Merrer M, Lajeunie E (2000) Fibroblast growth factor receptor 3 mutation in nonsyndromic coronal synostosis: clinical spectrum, prevalence, and surgical outcome. J Neurosurg 92:631–636

    Article  PubMed  CAS  Google Scholar 

  17. Esmer MC, Rodriguez-Soto G, Carrasco-Daza D, Iracheta ML, Del Castillo V (2000) Cloverleaf skull and multiple congenital anomalies in a girl exposed to cocaine in utero: case report and review of the literature. Childs Nerv Syst 16:176–179, discussion 180

    Article  PubMed  CAS  Google Scholar 

  18. Kalache KD, Lehmann K, Chaoui R, Kivelitz DE, Mundlos S, Bollmann R (2002) Prenatal diagnosis of partial agenesis of the corpus callosum in a fetus with thanatophoric dysplasia type 2. Prenat Diagn 22:404–407

    Article  PubMed  Google Scholar 

  19. Hayward R, Jones B, Dunaway D (2004) The clinical management of craniosynostosis. MacKeith, London

    Google Scholar 

  20. Gosain AK, Moore FO, Hemmy DC (1997) The kleeblattschadel anomaly in Apert syndrome: intracranial anatomy, surgical correction, and subsequent cranial vault development. Plast Reconstr Surg 100:1796–1802

    Article  PubMed  CAS  Google Scholar 

  21. Cinalli G, Sainte-Rose C, Kollar EM, Zerah M, Brunelle F, Chumas P, Arnaud E, Marchac D, Pierre-Kahn A, Renier D (1998) Hydrocephalus and craniosynostosis. J Neurosurg 88:209–214

    Article  PubMed  CAS  Google Scholar 

  22. Di Rocco F, Juca CE, Arnaud E, Renier D, Sainte-Rose C (2010) The role of endoscopic third ventriculostomy in the treatment of hydrocephalus associated with faciocraniosynostosis. J Neurosurg Pediatr 6:17–22

    Article  PubMed  Google Scholar 

  23. Marchac D, Renier D, Flandin-Bléty C (1982) Chirurgie cranio-faciale des craniosténoses. Medsi

  24. Sainte-Rose C, LaCombe J, Pierre-Kahn A, Renier D, Hirsch JF (1984) Intracranial venous sinus hypertension: cause or consequence of hydrocephalus in infants? J Neurosurg 60:727–736

    Article  PubMed  CAS  Google Scholar 

  25. Golabi M, Edwards MS, Ousterhout DK (1987) Craniosynostosis and hydrocephalus. Neurosurgery 21:63–67

    Article  PubMed  CAS  Google Scholar 

  26. Angle CR, McIntire MS, Moore RC (1967) Cloverleaf skull: Kleeblattschadel-deformity syndrome. Am J Dis Child 114:198–202

    PubMed  CAS  Google Scholar 

  27. Raybaud C, Di Rocco C (2007) Brain malformation in syndromic craniosynostoses, a primary disorder of white matter: a review. Childs Nerv Syst 23:1379–1388

    Article  PubMed  Google Scholar 

  28. Thompson DN, Hayward RD, Harkness WJ, Bingham RM, Jones BM (1995) Lessons from a case of kleeblattschadel. Case report. J Neurosurg 82:1071–1074

    Article  PubMed  CAS  Google Scholar 

  29. Shiroyama Y, Ito H, Yamashita T, Nakano S, Kurokawa Y (1991) The relationship of cloverleaf skull syndrome to hydrocephalus. Childs Nerv Syst 7:382–385

    Article  PubMed  CAS  Google Scholar 

  30. Cinalli G, Chumas P, Arnaud E, Sainte-Rose C, Renier D (1998) Occipital remodeling and suboccipital decompression in severe craniosynostosis associated with tonsillar herniation. Neurosurgery 42:66–71, discussion 71–63

    Article  PubMed  CAS  Google Scholar 

  31. Goodrich JT, Staffenberg DA (2004) Plastic techniques in neurosurgery. Thieme, New York

    Google Scholar 

  32. Kroczek RA, Muhlbauer W, Zimmermann I (1986) Cloverleaf skull associated with Pfeiffer syndrome: pathology and management. Eur J Pediatr 145:442–445

    Article  PubMed  CAS  Google Scholar 

  33. Moore MH, Cantrell SB, Trott JA, David DJ (1995) Pfeiffer syndrome: a clinical review. Cleft Palate Craniofac J 32:62–70

    Article  PubMed  CAS  Google Scholar 

  34. Venes JL (1988) Arnold-Chiari malformation in an infant with Kleeblattschadel: an acquired malformation? Neurosurgery 23:360–362

    Article  PubMed  CAS  Google Scholar 

  35. Jarrahy R, Kawamoto HK, Keagle J, Dickinson BP, Katchikian HV, Bradley JP (2009) Three tenets for staged correction of Kleeblattschadel or cloverleaf skull deformity. Plast Reconstr Surg 123:310–318

    Article  PubMed  CAS  Google Scholar 

  36. Preuss M, Stein M, Neubauer BA, Schaaf H, Howaldt HP, Nestler U, Christophis P (2010) About the operative management and post-operative neural development of patients with cloverleaf skull deformity. Childs Nerv Syst 26:1211–1218

    Article  PubMed  Google Scholar 

  37. Kozlowski K, Jequier S, Sillence D, Moir DH (1987) Cloverleaf skull and bone dysplasias (report of four cases). Australas Radiol 31:309–314

    Article  PubMed  CAS  Google Scholar 

  38. Arseni C, Horvath L, Ciurea V (1972) Cloverleaf skull. Acta Neurochir (Wien) 27:223–230

    Article  CAS  Google Scholar 

  39. Goh KY, Poon WS (1997) A case of adult kleeblattschadel. Acta Neurochir (Wien) 139:1088–1089

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pediatric Neurosurgery, Hôpital Necker-Enfants Malades, Paris, France

    Guilherme Machado, Federico Di Rocco, Christian Sainte-Rose, Philippe Meyer, Daniel Marchac, Gaëlle Macquet-Nouvion, Eric Arnaud & Dominique Renier

  2. Departement of Neurosurgery, Hospital de Sao Teotonio de Viseu, Viseu, Portugal

    Guilherme Machado

  3. Department of Anesthesia-Reanimation, Hôpital Necker-Enfants Malades, Paris, France

    Philippe Meyer

Authors
  1. Guilherme Machado
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Federico Di Rocco
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Christian Sainte-Rose
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Philippe Meyer
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Daniel Marchac
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Gaëlle Macquet-Nouvion
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Eric Arnaud
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Dominique Renier
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Federico Di Rocco.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Machado, G., Di Rocco, F., Sainte-Rose, C. et al. Cloverleaf skull deformity and hydrocephalus. Childs Nerv Syst 27, 1683–1691 (2011). https://doi.org/10.1007/s00381-011-1508-8

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-011-1508-8

Keywords

Search

Navigation