Skip to main content
SpringerLink
Log in

Management of lateral meningocele syndrome in a child without neurological symptoms and literature review

  • Case-Based Review
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Purpose

Lateral meningocele syndrome (LMS) is a rare genetic connective tissue disorder which is associated with meningocele-related neurologic dysfunction. Several patients with LMS have been reported. But, guidelines for screening and treatment of LMS have not been established.

Method and results

We review the current knowledge of LMS in the article. Then, we describe a boy for whom a genomic analysis which allowed us to make a diagnosis of LMS and to begin monitoring of his condition for possible neurological complications.

Conclusion

It would be difficult to make a diagnosis of LMS on the basis of clinical manifestations alone. The natural history of dural ectasia in patients with LMS needs to be better defined to establish surgical indications. Based upon the current literature, ventriculoperitoneal shunting (V-Ps) has been recommended as the first-line surgical treatment option for patients with symptomatic thoracolumbar meningoceles.

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

Similar content being viewed by others

Data availability

Not applicable.

References

  1. Gripp KW, Robbins KM, Sobreira NL et al (2015) Truncating mutations in the last exon of NOTCH3 cause lateral meningocele syndrome. Am J Med Genet A 167A(2):271–281. https://doi.org/10.1002/ajmg.a.36863

    Article  CAS  PubMed  Google Scholar 

  2. Ejaz R, Carter M, Gripp K (2016) Lateral meningocele syndrome. In: Adam MP, Ardinger HH, Pagon RA, et al., eds. GeneReviews®. Seattle (WA): University of Washington, Seattle; June 23

  3. Brown EC, Gupta K, Sayama C (2017) Neurosurgical management in lateral meningocele syndrome: case report. J Neurosurg Pediatr 19(2):232–238. https://doi.org/10.3171/2016.9.PEDS16311

    Article  PubMed  Google Scholar 

  4. Yamada M, Arimitsu T, Suzuki H, Miwa T, Kosaki K (2021) Early diagnosis of lateral meningocele syndrome in an infant without neurological symptoms based on genomic analysis [published online ahead of print, 2021 Jun 13]. Childs Nerv Syst.https://doi.org/10.1007/s00381-021-05232-6

  5. Cuoco JA, Klein BJ, Busch CM et al (2020) Neurosurgical management of lateral meningocele syndrome: a clinical update for the pediatric neurosurgeon. Pediatr Neurosurg 55(1):2–11. https://doi.org/10.1159/000504060

    Article  PubMed  Google Scholar 

  6. Katz SG, Grünebaum M, Strand RD (1978) Thoracic and lumbar dural ectasia in a two-year-old boy. Pediatr Radiol 6(4):238–240. https://doi.org/10.1007/BF00975545

    Article  CAS  PubMed  Google Scholar 

  7. Chen KM, Bird L, Barnes P, Barth R, Hudgins L (2005) Lateral meningocele syndrome: vertical transmission and expansion of the phenotype. Am J Med Genet A 133A(2):115–121. https://doi.org/10.1002/ajmg.a.30526

    Article  PubMed  Google Scholar 

  8. Lehman RA, Stears JC, Wesenberg RL, Nusbaum ED (1977) Familial osteosclerosis with abnormalities of the nervous system and meninges. J Pediatr 90(1):49–54. https://doi.org/10.1016/s0022-3476(77)80763-4

    Article  CAS  PubMed  Google Scholar 

  9. Ejaz R, Qin W, Huang L et al (2016) Lateral meningocele (Lehman) syndrome: a child with a novel NOTCH3 mutation. Am J Med Genet A 170A(4):1070–1075. https://doi.org/10.1002/ajmg.a.37541

    Article  CAS  PubMed  Google Scholar 

  10. Castori M, Morlino S, Ritelli M et al (2014) Late diagnosis of lateral meningocele syndrome in a 55-year-old woman with symptoms of joint instability and chronic musculoskeletal pain. Am J Med Genet A 164A(2):528–534. https://doi.org/10.1002/ajmg.a.36301

    Article  CAS  PubMed  Google Scholar 

  11. Cappuccio G, Apuzzo D, Alagia M et al (2020) Expansion of the phenotype of lateral meningocele syndrome. Am J Med Genet A 182(5):1259–1262. https://doi.org/10.1002/ajmg.a.61536

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  12. Gripp KW, Scott CI Jr, Hughes HE et al (1997) Lateral meningocele syndrome: three new patients and review of the literature. Am J Med Genet 70(3):229–239

    Article  CAS  Google Scholar 

  13. Pinna G, Alessandrini F, Alfieri A, Rossi M, Bricolo A (2000) Cerebrospinal fluid flow dynamics study in Chiari I malformation: implications for syrinx formation. Neurosurg Focus 8(3):E3. Published 2000 Mar 15. https://doi.org/10.3171/foc.2000.8.3.3

  14. Kim YJ, Cho HM, Yoon CS, Lee CK, Lee TY, Seok JP (2011) Surgical treatment of thoracic menigocele associated with neurofibromatosis and kyphoscoliosis. Korean J Thorac Cardiovasc Surg 44(5):383–386. https://doi.org/10.5090/kjtcs.2011.44.5.383

    Article  PubMed  PubMed Central  Google Scholar 

  15. Rainov NG, Heidecke V, Burkert W(1995) Thoracic and lumbar meningocele in neurofibromatosis type 1. Report of two cases and review of the literature. Neurosurg Rev 18(2):127–134. https://doi.org/10.1007/BF00417670

  16. Philip N, Andrac L, Moncla A et al (1995) Multiple lateral meningoceles, distinctive facies and skeletal anomalies: a new case of Lehman syndrome. Clin Dysmorphol 4(4):347–351. https://doi.org/10.1097/00019605-199510000-00011

    Article  CAS  PubMed  Google Scholar 

  17. Piper RJ, Pike M, Harrington R, Magdum SA (2019) Chiari malformations: principles of diagnosis and management. BMJ 365:l1159. Published 2019 Apr 8. https://doi.org/10.1136/bmj.l1159

  18. Davidson KA, Rogers JM, Stoodley MA (2018) Syrinx to subarachnoid shunting for syringomyelia. World Neurosurg 110:e53–e59. https://doi.org/10.1016/j.wneu.2017.09.205

    Article  PubMed  Google Scholar 

Download references

Funding

The study was supported by the University natural science research project of Jiangsu province (21KJB320019).

Author information

Authors and Affiliations

  1. Department of Neurosurgery, Children’s Hospital of Soochow University, 92 Zhongnan Street, Suzhou, Jiangsu, 215006, People’s Republic of China

    Yong Han, Min Chen & Hangzhou Wang

Authors
  1. Yong Han
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Min Chen
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Hangzhou Wang
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Hangzhou Wang.

Ethics declarations

Ethics approval and consent to participate

Not applicable.

Consent for publication

Written informed consent was obtained from the patient for the publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Conflict of interest

The authors declare no competing interests.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Yong Han is the first author.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Han, Y., Chen, M. & Wang, H. Management of lateral meningocele syndrome in a child without neurological symptoms and literature review. Childs Nerv Syst 38, 903–907 (2022). https://doi.org/10.1007/s00381-022-05466-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-022-05466-y

Keywords

Search

Navigation