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Venous anomalies in hypoplastic posterior fossa: unsolved questions

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Abstract

Introduction

Anomalous intracranial venous anatomy is described in patients with syndromic craniosynostosis and is of significant importance when it comes to surgical morbidity. However, it is still controversial its origin, type of circulation in each syndrome, how it behaves over time, when it can be interrupted and wether it needs to be studied. The purpose of this paper is to discuss these issues by reviewing the literature.

Methods

A literature search was performed using the PubMed database with a focus on papers including detailed descriptions of the venous outflow in complex and syndromic craniosynostosis. Search details used were the following: ("veins"[MeSH Terms] OR "veins"[All Fields] OR "venous"[All Fields]) AND ("abnormalities"[Subheading] OR "abnormalities"[All Fields] OR "anomalies"[All Fields]) AND syndromic[All Fields] AND ("craniosynostoses" [MeSH Terms] OR "craniosynostoses"[All Fields] OR "craniosynostosis"[All Fields]). Studies that exposed details of venous anomalies found in syndromic or complex craniosynostosis were selected.

Results

Of a total of 211 articles found, 11 were selected for this review. Of these, 5 were case reports, 5 retrospective studies, and only 1 prospective study. From the 6 series of cases presented, 5 discussed the relationship between jugular foramen stenosis (JFS) and collateral venous drainage. The authors discuss data from the literature for each leading question presented: 1—collateral circulation: is it an intrinsic trouble, a consequence of stenosis of the cranial base foramina or related to raised intracranial pressure (ICP)?; 2—what venous anomalies should we search for, and what is the best exam to study them?; 3—collateral circulation changes with time?; 4—can neurosurgeons interrupt the collateral circulation?; 5—should we study all complex types of craniosynostosis?

Conclusion

The importance of the study of the venous outflow in patients with complex craniosynostosis is evident in the literature. The real relationship between intracranial hypertension, hypoplastic skull base foramen, Chiari I malformation, hydrocephalus, and venous collateral circulation remains unknown. Prospective studies focusing on molecular biology analysis will possibly solve all of these leading questions.

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Abbreviations

ICP:

Intracranial pressure

JFS:

Jugular foramen stenosis

CSF:

Cerebral spinal fluid

CT:

Computed tomography

MR:

Magnetic resonance

References

  1. Copeland AE, Hoffman CE, Tsitouras V et al (2018) Clinical significance of venous anomalies in syndromic craniosynostosis: plastic and reconstructive surgery - Global Open 6:e1613. https://doi.org/10.1097/GOX.0000000000001613

    Article  PubMed  PubMed Central  Google Scholar 

  2. Jeevan DS, Anlsow P, Jayamohan J (2008) Abnormal venous drainage in syndromic craniosynostosis and the role of CT venography. Child’s Nervous System 24:1413–1420. https://doi.org/10.1007/s00381-008-0667-8

    Article  PubMed  Google Scholar 

  3. Florisson JMG, Barmpalios G, Lequin M et al (2015) Venous hypertension in syndromic and complex craniosynostosis: the abnormal anatomy of the jugular foramen and collaterals. J Craniomaxillofac Surg 43:312–318. https://doi.org/10.1016/j.jcms.2014.11.023

    Article  PubMed  Google Scholar 

  4. Wang JC, Nagy L, Demke JC (2016) Syndromic craniosynostosis. Facial Plast Surg Clin North Am 24:531–543. https://doi.org/10.1016/j.fsc.2016.06.008

    Article  PubMed  Google Scholar 

  5. Coll G, Sakka L, Botella C et al (2018) Pattern of closure of skull base synchondroses in crouzon syndrome. World Neurosurg 109:e460–e467. https://doi.org/10.1016/j.wneu.2017.09.208

    Article  PubMed  Google Scholar 

  6. Tahiri Y, Paliga JT, Vossough A et al (2014) The spheno-occipital synchondrosis fuses prematurely in patients with Crouzon syndrome and midface hypoplasia compared with age- and gender-matched controls. J Oral Maxillofac Surg 72:1173–1179. https://doi.org/10.1016/j.joms.2013.11.015

    Article  PubMed  Google Scholar 

  7. Calandrelli R, D’Apolito G, Gaudino S et al (2014) Identification of skull base sutures and craniofacial anomalies in children with craniosynostosis: utility of multidetector CT. La Radiologia Medica 119:694–704. https://doi.org/10.1007/s11547-014-0387-y

    Article  PubMed  Google Scholar 

  8. Hoffman HJ, Tucker WS (1976) Cephalocranial disproportion. A complication of the treatment of hydrocephalus in children. Childs Brain 2:167–176

    CAS  PubMed  Google Scholar 

  9. Saldino RM, Steinbach HL, Epstein CJ (1972) FAMILIAL ACROCEPHALOSYNDACTYLY (PFEIFFER SYNDROME). Am J Roentgenol 116:609–622. https://doi.org/10.2214/ajr.116.3.609

    Article  CAS  Google Scholar 

  10. Cinalli G, Renier D, Sebag G et al (1995) Chronic tonsillar herniation in Crouzon’s and Apert’s syndromes: the role of premature synostosis of the lambdoid suture. J Neurosurg 83:575–582. https://doi.org/10.3171/jns.1995.83.4.0575

    Article  CAS  PubMed  Google Scholar 

  11. Coll G, El Ouadih Y, Abed Rabbo F et al (2019) Hydrocephalus and Chiari malformation pathophysiology in FGFR2-related faciocraniosynostosis: a review. Neurochirurgie 65:264–268. https://doi.org/10.1016/j.neuchi.2019.09.001

    Article  CAS  PubMed  Google Scholar 

  12. Cinalli G, Sainte-Rose C, Kollar EM et al (1998) Hydrocephalus and craniosynostosis. J Neurosurg 88:209–214. https://doi.org/10.3171/jns.1998.88.2.0209

    Article  CAS  PubMed  Google Scholar 

  13. Martinez-Perez D, Vander Woude DL, Barnes PD et al (1996) Jugular foraminal stenosis in Crouzon syndrome. Pediatric Neurosurg 25:252–255. https://doi.org/10.1159/000121134

    Article  CAS  PubMed  Google Scholar 

  14. Rollins N, Booth T, Shapiro K (2000) MR venography in children with complex craniosynostosis. Pediatr Neurosurg 32:308–315. https://doi.org/10.1159/000028959

    Article  CAS  PubMed  Google Scholar 

  15. Sanchez R, Ward K, St. Hilaire H, Mussell J, (2013) Basicranial venous anomalies associated with complex nonsyndromic craniosynostosis in a child. J Craniofac Surg 24:1970–1973. https://doi.org/10.1097/SCS.0b013e3182a24891

    Article  Google Scholar 

  16. Taylor WJ, Hayward RD, Lasjaunias P et al (2001) Enigma of raised intracranial pressure in patients with complex craniosynostosis: the role of abnormal intracranial venous drainage. J Neurosurg 94:377–385. https://doi.org/10.3171/jns.2001.94.3.0377

    Article  CAS  PubMed  Google Scholar 

  17. Coll G, Arnaud E, Collet C et al (2015) Skull base morphology in fibroblast growth factor receptor type 2-related faciocraniosynostosis. Neurosurgery 76:571–583. https://doi.org/10.1227/NEU.0000000000000676

    Article  PubMed  Google Scholar 

  18. Gray JL, Kang SS, Zenni GC et al (1994) FGF-1 Affixation stimulates ePTFE endothelialization without intimal hyperplasia. J Surg Res 57:596–612. https://doi.org/10.1006/jsre.1994.1189

    Article  CAS  Google Scholar 

  19. Ignelzi MA, Wang W, Young AT (2003) Fibroblast growth factors lead to increased Msx2 expression and fusion in calvarial sutures. J Bone Miner Res 18:751–759. https://doi.org/10.1359/jbmr.2003.18.4.751

    Article  CAS  PubMed  Google Scholar 

  20. Tischfield MA, Robson CD, Gilette NM et al (2017) Cerebral Vein malformations result from loss of twist1 expression and BMP signaling from skull progenitor cells and dura. Dev Cell 42:445-461.e5. https://doi.org/10.1016/j.devcel.2017.07.027

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Lopes L, Palma T, Cravo I et al (2012) The development of the cerebral venous system–from the embryo to the adult definitive structure. Acta Med Port 25(Suppl 1):30–33

    PubMed  Google Scholar 

  22. Okudera T, Huang YP, Ohta T et al (1994) Development of posterior fossa dural sinuses, emissary veins, and jugular bulb: morphological and radiologic study. AJNR Am J Neuroradiol 15:1871–1883

    CAS  PubMed  PubMed Central  Google Scholar 

  23. Thompson DNP, Hayward RD, Harkness WJ et al (1995) Lessons from a case of kleeblattschädel. J Neurosurg 82:1071–1074. https://doi.org/10.3171/jns.1995.82.6.1071

    Article  CAS  PubMed  Google Scholar 

  24. Anderson PJ, Harkness WJ, Taylor W et al (1997) Anomalous venous drainage in a case of non-syndromic craniosynostosis. Child’s Nervous Sys 13:97–100. https://doi.org/10.1007/s003810050052

    Article  CAS  PubMed  Google Scholar 

  25. Al-Otibi M, Jea A, Kulkarni AV (2007) Detection of important venous collaterals by computed tomography venogram in multisutural synostosis: case report and review of the literature. J Neurosurg: Pediatrics 107:508–510. https://doi.org/10.3171/PED-07/12/508

    Article  Google Scholar 

  26. Robson CD, Mulliken JB, Robertson RL et al (2000) Prominent basal emissary foramina in syndromic craniosynostosis: correlation with phenotypic and molecular diagnoses. AJNR Am J Neuroradiol 21:1707–1717

    CAS  PubMed  PubMed Central  Google Scholar 

  27. Sandberg DI, Navarro R, Blanch J, Ragheb J (2007) Anomalous venous drainage preventing safe posterior fossa decompression in patients with Chiari malformation type I and multisutural craniosynostosis: report of two cases and review of the literature. J Neurosurg: Pediatrics 106:490–494. https://doi.org/10.3171/ped.2007.106.6.490

    Article  Google Scholar 

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Authors and Affiliations

  1. Department of Pediatric Surgery, Division of Pediatric Neurosurgery, Fernandes Figueira National Institute of Health for Women,Children and Adolescents, Oswaldo Cruz Foundation (IFF-Fiocruz), Rio de Janeiro, Brazil

    T. Protzenko & A. Bellas

  2. Radiological Department, Fernandes Figueira National Institute of Health for Women, Children and Adolescents, Oswaldo Cruz Foundation (IFF-Fiocruz), Rio de Janeiro, Brazil

    C. Maria Cunha Ribeiro

  3. Laboratory of Genomic Medicine, Department of Medical Genetics, Fernandes Figueira National Institute of Health for Women, Children and Adolescents, Oswaldo Cruz Foundation (IFF-Fiocruz), Rio de Janeiro, Brazil

    S. Maria de Carvalho Gonzalez

  4. Department of Medical Genetics, Fernandes Figueira National Institute of Health for Women, Children and Adolescents , Oswaldo Cruz Foundation (IFFFiocruz), Rio de Janeiro, Brazil

    D. Dain Gandelman Horovitz

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  1. T. Protzenko
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Correspondence to T. Protzenko.

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Protzenko, T., Bellas, A., Ribeiro, C.M.C. et al. Venous anomalies in hypoplastic posterior fossa: unsolved questions. Childs Nerv Syst 37, 3177–3187 (2021). https://doi.org/10.1007/s00381-021-05315-4

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