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Child's Nervous System

, Volume 34, Issue 8, pp 1549–1555 | Cite as

Early analysis of operative management of Chiari I malformation in pediatric cystic fibrosis patients

  • Derek C. Samples
  • Dewey J. Thoms
  • Izabela Tarasiewicz
Original Paper

Abstract

Introduction

Chiari I malformation, defined as herniation of the cerebellar tonsils at least 5 mm below the foramen magnum, can result from congenital or acquired pathology. While the mechanism is not well understood, an association between Chiari I and cystic fibrosis has been described in the literature. The lifelong respiratory status management necessitated by cystic fibrosis creates a greater risk of Chiari symptomatology as well as post-operative CSF-related complications in the setting of duraplasty secondary to recurrent transient increases in intracranial pressure. We will review the literature, describe our experience with these patients, and propose bony decompression as an approach to treatment.

Methods

A retrospective review of pediatric patients treated at our institution with both cystic fibrosis and Chiari I was performed. Since our first case in 2016, our department has evaluated four patients carrying that dual diagnosis. All four underwent posterior fossa decompression surgery. Two patients had incidental pathology. Two symptomatic patients exhibited headaches and/or coordination difficulty. Half of the patients had associated syringomyelia. All patients were offered posterior fossa decompression utilizing intraoperative ultrasound.

Results

All four patients underwent posterior fossa decompression without duraplasty. Average operative time was 128 min. There were no complications post-operatively. Average hospital stay was 3.8 days. Average surgical length of stay was 2.3 days. Morbidity and mortality were 0%. The longest follow-up to date is 20 months. The two asymptomatic patients remained so post-operatively. The child with headaches and imbalance had complete resolution of his symptoms after surgery, as did the toddler with headaches. Both patients with syringomyelia demonstrated significant decrease in the size of their syrinxes on imaging performed at least 3 months post-operatively.

Conclusion

Based on the literature and our experience, we recommend considering posterior fossa decompression without duraplasty as treatment for pediatric cystic fibrosis patients with Chiari I malformation. This approach can be effective for symptomatic and prophylactic cases in this particular patient demographic because their comorbidities predispose them to Chiari pathology and symptomatology as well as certain post-operative complications.

Keywords

Pediatric neurosurgery Chiari malformation Cystic fibrosis Posterior fossa decompression 

Notes

Acknowledgements

Thank you to David Garza for his editing contributions to the manuscript.

Compliance with ethical standards

With approval from our Institutional Review Board, we performed a retrospective analysis of our internal patient database starting from January 2016.

Conflict of interest

The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

References

  1. 1.
    Anderson RC (2003) Improvement in brainstem auditory evoked potentials after suboccipital decompression in patients with Chiari I malformations. J Neurosurg 98:459–464CrossRefPubMedGoogle Scholar
  2. 2.
    Caldarelli M, Novegno F, Vassimi L, Romani R, Tamburrini G, di Rocco C (2007) The role of limited posterior fossa craniectomy in the surgical treatment of Chiari malformation type I: experience with a pediatric series. J Neurosurg 106:187–195PubMedGoogle Scholar
  3. 3.
    Durham S, Fjeld-Olenec K (2008) Comparison of posterior fossa decompression with and without duraplasty for the surgical treatment of Chiari malformation Type I in pediatric patients: a meta-analysis. J Neurosurg Pediatr 2:42–49CrossRefPubMedGoogle Scholar
  4. 4.
    Genitori et al (2000) Chiari type I anomalies in children and adolescents; minimally invasive management in a series of 53 cases. Child Nerv Syst 16:707–718CrossRefGoogle Scholar
  5. 5.
    Hankison T, Tubbs R, Oakes W (2012) Chapter 58: Surgical decision-making and treatment options for Chiari malformations in children. Schmidek & Sweet operative neurosurgical techniques indicated, methods, and results. 6th Ed. Alfredo Quiñones-Hinojosa. Elsevier. 695–701. PrintGoogle Scholar
  6. 6.
    Kennedy B et al (2015) Delayed resolution of syrinx after posterior fossa decompression without dural opening in children with Chiari malformation type I. J Neurosurg Pediatr 16:599–606CrossRefGoogle Scholar
  7. 7.
    Kennedy B et al (2015) Outcomes after suboccipital decompression without dural opening in children with Chiari malformation type I. J Neurosurg Pediatr 16:150–158CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Khanna A, Coumans JV (2016) Spontaneous improvement of Chiari I malformation and syringomyelia in a patient with cystic fibrosis. Neurosurgery 78(2):E305–E308CrossRefPubMedGoogle Scholar
  9. 9.
    McGirt MJ, Attenello FJ, Atiba A, Garces-Ambrossi G, Datoo G, Weingart JD et al (2008) Symptom recurrence after suboccipital decompression for pediatric Chiari I malformation: analysis of 256 consecutive cases. Childs Nerv Syst 24:1333–1339CrossRefPubMedGoogle Scholar
  10. 10.
    McGirt M et al (2008) Intraoperative ultrasonography as a guide to patient selection for duraplasty after suboccipital decompression in children with Chiari malformation type I. J Neurosurg Pediatr 2:52–57CrossRefPubMedGoogle Scholar
  11. 11.
    Meadows J, Kraut M, Guarnieri M, Haroun RI, Carson BS (2000) Asymptomatic Chiari type I malformations identified on magnetic resonance imaging. J Neurosurg 92:920–926CrossRefPubMedGoogle Scholar
  12. 12.
    Munshi I, Frim D, Stine-Reyes R, Weir BKA, Hekmatpanah J, Brown F (2000) Effects of posterior fossa decompression with and without duraplasty on Chiari malformation-associated hydromyelia. Neurosurgery 46(6):1384–1390CrossRefPubMedGoogle Scholar
  13. 13.
    Mutchnick I et al (2010) Decompression of Chiari malformation with and without duraplasty: morbidity versus recurrence. J Neurosurg Pediatr 5:474–478CrossRefPubMedGoogle Scholar
  14. 14.
    Narasimahaiah D et al (2014) Chiari type I malformation in two adult patients with cystic fibrosis. J Cyst Fibros 13(2):S125CrossRefGoogle Scholar
  15. 15.
    Needleman J et al (2000) Chiair type I malformation in children and adolescents with cystic fibrosis. Pediatr Pulmonol 30:490–492CrossRefPubMedGoogle Scholar
  16. 16.
    Patel A, Raol V, Jea A (2011) Rare association between cystic fibrosis, Chiari I malformation, and hydrocephalus in a baby: a case report and review of the literature. J Med Case Rep 5:366CrossRefPubMedPubMedCentralGoogle Scholar
  17. 17.
    Patient-Centered Outcomes Research I, Johns Hopskins All Children’s H, Yale U et al (2017) Posterior fossa decompression with or without duraplasty for Chiari type I malformation with syringomyelia. [serial online]. Available from: ClinicalTrials.gov, Ipswich, MA
  18. 18.
    Rekheja et al (2002) Cystic fibrosis and Chiari type I malformation: autopsy study of two infants with a rare association. Pediatr Dev Pathol 6:88–93CrossRefGoogle Scholar
  19. 19.
    Rusakow LS, Guarin M, Lyon RM, Splaingard ML (1995) Syringomelia and Chiari malformation presenting as scoliosis in cystic fibrosis. Pediatr Pulmonol 19:317–318CrossRefPubMedGoogle Scholar
  20. 20.
    Siri Sahib S et al (2017) Comparison of posterior fossa volumes and clinical outcomes after decompression of Chiari malformation type I. J Neurosurg Pediatr 19:511–517CrossRefGoogle Scholar
  21. 21.
    Shweikeh F, Sunjaya D, Nuno M, Drazin D, Adamo MA (2015) National trends, complications, and hospital charges in pediatric patients with Chiari malformation type I treated with posterior fossa decompression with and without duraplasty. Pediatr Neurosurg 50:31–37CrossRefPubMedGoogle Scholar
  22. 22.
    Ventureyra et al (2003) The role of cine flow MRI in children with Chiari 1 malformation. Childs Nerv System 19:109–113Google Scholar
  23. 23.
    Yeh et al (2006) IOU to determine the extent of surgery necessary during PFD in children with Chiari malformation type I. J Neurosurg 105(1 Supp):26–32PubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Derek C. Samples
    • 1
  • Dewey J. Thoms
    • 1
  • Izabela Tarasiewicz
    • 1
  1. 1.Department of Neurological SurgeryUniversity of Texas Health San AntonioSan AntonioUSA

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