eMedicine Specialties > Radiology > Pediatrics

Chiari II Malformation: Follow-up

Author: Lutfi Incesu, MD, Professor, Department of Radiology, Ondokuz Mayis University School of Medicine; Chief, Neuroradiology and MR Unit, Department of Radiology, Ondokuz Mayis University Hospital, Turkey
Coauthor(s): Anil Khosla, MBBS, Assistant Professor, Department of Radiology, Section of Neuroradiology, Mallinckrodt Institute of Radiology, Washington University School of Medicine, Veterans Affairs Medical Center of St Louis; Michael R Aiello, MD, Radiologist, St Elizabeth Medical Center, Utica, NY
Contributor Information and Disclosures

Updated: Oct 15, 2009

Intervention

Knowledge of the potential life-threatening symptoms of Chiari II malformation has resulted in the advocacy for early surgical intervention, especially in infants. Conservative treatment may lead to irreversible changes. Early recognition of the Chiari malformation is important because it is the leading cause of death in patients treated for myelomeningocele.

Neonates do more poorly with surgery. In one study, 23% died and 16% had a poor outcome. The reason may have been a delay in surgery with resultant brainstem infarction and hemorrhage. However, if surgery is needed, earlier intervention may result in a better prognosis.23,24

Medicolegal Pitfalls

  • Failure to diagnose a Chiari II malformation and its associated anomalies in a neonate or a child can cause serious brain and spinal complications.
  • Failure to identify early cranial and spinal cord lesions may result in delayed intervention and permanent neurologic deficit.
  • Failure to make the correct diagnosis (eg, cerebellar tumor instead of Chiari II malformation) can cause morbidity and mortality resulting from surgery.

Special Concerns

  • US can be performed in high-risk pregnancies, and with a careful US examination, the Chiari II malformation can be diagnosed antenatally.
    • US is routinely used during the antenatal period as a screening method. With careful examination, cranial and spinal pathologic changes related to Chiari II malformations in the fetus can be seen. Reportedly, a lacunar skull can be detected by using US in the fetus.
    • To confirm the diagnosis and gain detailed information, MRI can be used antenatally.
  • MRI has been used as a diagnostic tool during the antenatal period, allowing the intrauterine diagnosis, and even treatment, of spina bifida.
 


More on Chiari II Malformation

Overview: Chiari II Malformation
Imaging: Chiari II Malformation
Follow-up: Chiari II Malformation
Multimedia: Chiari II Malformation
References
Further Reading

References

  1. Chiari H. Uber veranderungen des kleinhirns, des pons und der medulla oblongata in folge von congenitaler hydrocephalie des Grosshirns. Denschr Akad Wiss. 1896;63:71-116.

  2. Gardner WJ. The Dysraphic States: From Syringomyelia to Anencephaly. Amsterdam, The Netherlands: Excerpta Medica; 1973:5-14.

  3. Truivit CL, Backovich A. Disorders of brain development. In: Atlas SW, ed. Magnetic Resonance Imaging of the Brain and Spine. 2nd ed. Philadelphia, Pa: Lippincott-Raven; 1996.

  4. Weaver DD. Genetics of developmental defects. In: Wilkins RH, Rengachary SS, eds. Neurosurgery. 2nd ed. New York, NY: McGraw-Hill; 1996:3424-5.

  5. Stevenson KL. Chiari Type II malformation: past, present, and future. Neurosurg Focus. Feb 15 2004;16(2):E5. [Medline].

  6. Tubbs RS, Oakes WJ. Treatment and management of the Chiari II malformation: an evidence-based review of the literature. Childs Nerv Syst. Jun 2004;20(6):375-81. [Medline].

  7. Herweh C, Akbar M, Wengenroth M, Blatow M, Mair-Walther J, Rehbein N, et al. DTI of commissural fibers in patients with Chiari II-malformation. Neuroimage. Jan 15 2009;44(2):306-11. [Medline].

  8. Callen AL, Stengel JW, Filly RA. Supratentorial abnormalities in the Chiari II malformation, II: tectal morphologic changes. J Ultrasound Med. Jan 2009;28(1):29-35. [Medline].

  9. Wong SK, Barkovich AJ, Callen AL, Filly RA. Supratentorial abnormalities in the Chiari II malformation, III: The interhemispheric cyst. J Ultrasound Med. Aug 2009;28(8):999-1006. [Medline].

  10. McLone DG, Knepper PA. The cause of Chiari II malformation: a unified theory. Pediatr Neurosci. 1989;15(1):1-12. [Medline].

  11. Cleland J. Contribution to the study of spina bifida, encephalocele and anacephalus. J Anat Physiol. 1883;17:237-92.

  12. Daniel PM, Strich SJ. Some observations on the congenital deformity of the central nervous system known as the Arnold-Chiari malformation. J Neuropathol Exp Neurol. Apr 1958;17(2):255-66. [Medline].

  13. Peach B. The Arnold-Chiari malformation; morphogenesis. Arch Neurol. May 1965;12:527-35. [Medline].

  14. Vandertop WP, Asai A, Hoffman HJ, et al. Surgical decompression for symptomatic Chiari II malformation in neonates with myelomeningocele. J Neurosurg. Oct 1992;77(4):541-4. [Medline].

  15. Miller E, Widjaja E, Blaser S, Dennis M, Raybaud C. The old and the new: supratentorial MR findings in Chiari II malformation. Childs Nerv Syst. May 2008;24(5):563-75. [Medline].

  16. Goh S, Bottrell CL, Aiken AH, Dillon WP, Wu YW. Presyrinx in children with Chiari malformations. Neurology. Jul 29 2008;71(5):351-6. [Medline].

  17. Ando K, Ishikura R, Ogawa M, et al. MRI tight posterior fossa sign for prenatal diagnosis of Chiari type II malformation. Neuroradiology. Dec 2007;49(12):1033-9. [Medline].

  18. Wilberger JE Jr, Maroon JC, Prostko ER, et al. Magnetic resonance imaging and intraoperative neurosonography in syringomyelia. Neurosurgery. Apr 1987;20(4):599-605. [Medline].

  19. Hunter JV, Youl BD, Moseley IF. MRI demonstration of midbrain deformity in association with Chiari malformation. Neuroradiology. 1992;34(5):399-401. [Medline].

  20. Duffau H, Sahel M, Sichez JP, Marro B. Three-dimensional computerized tomography in the presurgical evaluation of Chiari malformations. Acta Neurochir (Wien). 1998;140(5):429-35; discussion 435-6. [Medline].

  21. Sattar TS, Bannister CM, Russell SA, Rimmer S. Pre-natal diagnosis of occult spinal dysraphism by ultrasonography and post-natal evaluation by MR scanning. Eur J Pediatr Surg. Dec 1998;8 Suppl 1:31-3. [Medline].

  22. Hori A. Tectocerebellar dysraphia with posterior encephalocele (Friede): report of the youngest case. Reappraisal of the condition uniting Cleland-Chiari (Arnold-Chiari) and Dandy-Walker syndromes. Clin Neuropathol. Jul-Aug 1994;13(4):216-20. [Medline].

  23. Hirose S, Farmer DL. Fetal surgery for myelomeningocele. Clin Perinatol. Jun 2009;36(2):431-8, xi. [Medline].

  24. Adzick NS. Fetal myelomeningocele: Natural history, pathophysiology, and in-utero intervention. Semin Fetal Neonatal Med. Jun 17 2009;[Medline].

  25. Coley BD. Ultrasound diagnosis of luckenschadel (lacunar skull). Pediatr Radiol. Feb 2000;30(2):82-4. [Medline].

  26. Curnes JT, Oakes WJ, Boyko OB. MR imaging of hindbrain deformity in Chiari II patients with and without symptoms of brainstem compression. AJNR Am J Neuroradiol. Mar-Apr 1989;10(2):293-302. [Medline].

  27. el Gammal T, Mark EK, Brooks BS. MR imaging of Chiari II malformation. AJR Am J Roentgenol. Jan 1988;150(1):163-70. [Medline][Full Text].

  28. Ergün R, Akdemir G, Gezici AR, et al. Surgical management of syringomyelia-Chiari complex. Eur Spine J. Dec 2000;9(6):553-7. [Medline].

  29. Haines SJ, Berger M. Current treatment of Chiari malformations types I and II: A survey of the Pediatric Section of the American Association of Neurological Surgeons. Neurosurgery. Mar 1991;28(3):353-7. [Medline].

  30. Huppert BJ, Brandt KR, Ramin KD, King BF. Single-shot fast spin-echo MR imaging of the fetus: a pictorial essay. Radiographics. Oct 1999;19 Spec no:S215-27. [Medline][Full Text].

  31. McLone DG. Images in pediatric neurosurgery. Chiari malformations. Pediatr Neurosurg. Mar 2000;32(3):164. [Medline].

  32. McLone DG, Naidich TP. Developmental morphology of the subarachnoid space, brain vasculature, and contiguous structures, and the cause of the Chiari II malformation. AJNR Am J Neuroradiol. Mar-Apr 1992;13(2):463-82. [Medline].

  33. Nadkarni TD, Rekate HL. Treatment of refractory intracranial hypertension in a spina bifida patient by a concurrent ventricular and cisterna magna-to-peritoneal shunt. Childs Nerv Syst. Jul 2005;21(7):579-82. [Medline].

  34. Naidich TP, McLone DG, Fulling KH. The Chiari II malformation: Part IV. The hindbrain deformity. Neuroradiology. 1983;25(4):179-97. [Medline].

  35. Nishikawa M, Sakamoto H, Hakuba A, Nakanishi N, Inoue Y. Pathogenesis of Chiari malformation: a morphometric study of the posterior cranial fossa. J Neurosurg. Jan 1997;86(1):40-7. [Medline].

  36. Pearce JM. Arnold chiari, or "Cruveilhier Cleland Chiari" malformation. J Neurol Neurosurg Psychiatry. Jan 2000;68(1):13. [Medline][Full Text].

  37. Rath GP, Bithal PK, Chaturvedi A. Atypical presentations in Chiari II malformation. Pediatr Neurosurg. 2006;42(6):379-82. [Medline].

  38. Rauzzino M, Oakes WJ. Chiari II malformation and syringomyelia. Neurosurg Clin N Am. Apr 1995;6(2):293-309. [Medline].

  39. Salman MS, Sharpe JA, Eizenman M, et al. Saccadic adaptation in Chiari type II malformation. Can J Neurol Sci. Nov 2006;33(4):372-8. [Medline].

  40. Yumer MH, Nachev SS, Dzhendov TY, Kalev OK. Chiari type II malformation: a case report and review of literature. Folia Med (Plovdiv). 2006;48(1):55-9. [Medline].

Keywords

Chiari II malformation, neural tube defects, Arnold-Chiari malformation, meningomyelocele, hydrocephalus, Cruveilhier-Cleland-Chiari malformation, Chiari I malformation, Chiari III malformation, Chiari IV malformation, hindbrain anomaly, posterior fossa, myelomeningocele

Contributor Information and Disclosures

Author

Lutfi Incesu, MD, Professor, Department of Radiology, Ondokuz Mayis University School of Medicine; Chief, Neuroradiology and MR Unit, Department of Radiology, Ondokuz Mayis University Hospital, Turkey
Lutfi Incesu, MD is a member of the following medical societies: American Society of Neuroradiology and Radiological Society of North America
Disclosure: Nothing to disclose.

Coauthor(s)

Anil Khosla, MBBS, Assistant Professor, Department of Radiology, Section of Neuroradiology, Mallinckrodt Institute of Radiology, Washington University School of Medicine, Veterans Affairs Medical Center of St Louis
Anil Khosla, MBBS is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, American Society of Neuroradiology, North American Spine Society, and Radiological Society of North America
Disclosure: Nothing to disclose.

Michael R Aiello, MD, Radiologist, St Elizabeth Medical Center, Utica, NY
Michael R Aiello, MD is a member of the following medical societies: American College of Radiology, American Institute of Ultrasound in Medicine, American Medical Association, Radiological Society of North America, Society of Breast Imaging, and Society of Cardiovascular and Interventional Radiology
Disclosure: Nothing to disclose.

Medical Editor

Charles M Glasier, MD, Professor, Departments of Radiology and Pediatrics, University of Arkansas for Medical Sciences; Chief, Magnetic Resonance Imaging, Vice-Chief, Pediatric Radiology, Arkansas Children's Hospital
Charles M Glasier, MD is a member of the following medical societies: American College of Radiology, American Society of Neuroradiology, Radiological Society of North America, and Society for Pediatric Radiology
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

Marta Hernanz-Schulman, MD, FAAP, Professor, Radiology, Radiological Sciences, and Pediatrics, Director, Department of Pediatric Radiology, Radiologist-in-Chief, Director, Department of Diagnostic Imaging, Vanderbilt University Medical Center, Vanderbilt Children's Hospital
Marta Hernanz-Schulman, MD, FAAP is a member of the following medical societies: American Institute of Ultrasound in Medicine and American Roentgen Ray Society
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Resolution Imaging Medical Corporation
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

James G Smirniotopoulos, MD, Professor of Radiology, Neurology, and Biomedical Informatics, Chairman, Department of Radiology and Radiological Sciences, Uniformed Services University of the Health Sciences
James G Smirniotopoulos, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, American Society of Head and Neck Radiology, American Society of Neuroradiology, American Society of Pediatric Neuroradiology, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.