Chiari Malformation (CM) type III surgical management in infant: a case report

Yusuf Baktir; Wihasto Suryaningtyas; Muhammad Arifin Parenrengi

doi:10.15562/bmj.v12i3.4525

Chiari Malformation (CM) type III surgical management in infant: a case report

Yusuf Baktir ,

Wihasto Suryaningtyas ,

Muhammad Arifin Parenrengi ,

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DOI: https://doi.org/10.15562/bmj.v12i3.4525 |
Published: 2023-09-16

Abstract

Background: Chiari Malformations (CM) is a collection of hindbrain abnormalities ranging from simple to complete agenesis of the cerebellum. Type III CM (CM III) is the rarest CM, with herniation of the cerebellum and brainstem into a posterior encephalocele and other intracranial anomalies. Reduction of the viable herniated brain, along with providing intracranial space and inadequate closure, often presents as the main problem. This case study aims to evaluate the Chiari malformation type III surgical management in infants.

Case Presentation: A 16-day-old male infant with an intact congenital mass in the occipital, with no neurological deficit. Radiological examination shows a defect in the occipital skull consisting of cystic non-contrast enhancing lesion and parenchymal tissue herniated through the defect. The cele excision and defect closure were performed, and viable neural tissue was found inside the sac. Blossom flower-like trim expanded the fossa posterior space and put the viable neural tissue inside the intracranial room. The defect was then closed with a dura, periosteal patch, and tension-free two-layered scalp closure. Post-operative follow-up presented a good outcome, and 1-year follow-up showed good recovery, with developmental delay, following developmental rehabilitation.

Conclusion: CM III is rare and has poor outcomes because neurological deficits and respiratory problems usually follow it. Operative management patient with CM III was presented with occipital encephalocele with no post-operative complication. Occipital bone reconstruction using a blossomed-flower-like technique could be considered to create adequate room for viable neural tissue.

References

Fons K, Jnah AJ. Arnold-Chiari Malformation: Core Concepts. Neonatal Netw. 2021;40(5):313-320.
Chapman PH, Swearingen B, Caviness VS. Subtorcular occipital encephaloceles. Anatomical considerations relevant to operative management. J Neurosurg. 1989;71(3):375–81.
Işik N, Elmaci I, Silav G, Celik M, Kalelioğlu M. Chiari malformation type III and results of surgery: a clinical study: report of eight surgically treated cases and review of the literature. Pediatr Neurosurg. 2009;45(1):19-28.
Cama A, Tortori-Donati P, Piatelli GL, Fondelli MP, Andreussi L. Chiari complex in children--neuroradiological diagnosis, neurosurgical treatment and proposal of a new classification (312 cases). Eur J Pediatr Surg. 1995;5 Suppl 1:35-38.
Jeong DH, Kim CH, Kim MO, Chung H, Kim TH, Jung HY. Arnold-Chiari malformation type III with meningoencephalocele: A case report. Ann Rehabil Med. 2014;38(3):401–404.
Winston KR. Pediatric neurosurgery: pride and prejudice. Pediatr Neurosurg. 2000;32(2):58-68.
Agrawal A, Mittal A, Kohali GB, Sampley S, Gupta A. Chiari III malformation. Pediatr Neurosurg. 2012;47(4):309–310.
Rani H, Kulkarni AV, Rao RV, Patil P. Chiari III malformation: A rare case with review of literature. Fetal Pediatr Pathol. 2013;32(3):169–174.
Mohanty A, Biswas A, Reddy M, Kolluri S. Expansile cranioplasty for massive occipital encephalocele. Child’s Nerv Syst. 2006;22(9):1170–1176.
Malkowicz DE, Myers G, Leisman G. Rehabilitation of cortical visual impairment in children. Int J Neurosci. 2006;116(9):1015-1033.
Hoyt CS. Visual function in the brain-damaged child. Eye (Lond). 2003;17(3):369-384.
Caesarita DP, Bakhtiar Y, Prihatningtias R. Visual function outcome post intracranial surgery in sellar region brain tumor. Bali Medical Journal. 2021;10(1):309-313.
Aritonang C, Putri SA, Utomo B. The effectiveness of using peek acuity in vision screening for the admission of elementary school students. Bali Medical Journal. 2022;11(2):671-675.
Hafizhan, Bakhtiar Y, Prihatningtias R. Visual function recovery post decompression surgery in meningioma: a case report. Bali Medical Journal. 2021;10(1):429-432.

[1] Fons K, Jnah AJ. Arnold-Chiari Malformation: Core Concepts. Neonatal Netw. 2021;40(5):313-320.

[2] Chapman PH, Swearingen B, Caviness VS. Subtorcular occipital encephaloceles. Anatomical considerations relevant to operative management. J Neurosurg. 1989;71(3):375–81.

[3] Işik N, Elmaci I, Silav G, Celik M, Kalelioğlu M. Chiari malformation type III and results of surgery: a clinical study: report of eight surgically treated cases and review of the literature. Pediatr Neurosurg. 2009;45(1):19-28.

[4] Cama A, Tortori-Donati P, Piatelli GL, Fondelli MP, Andreussi L. Chiari complex in children--neuroradiological diagnosis, neurosurgical treatment and proposal of a new classification (312 cases). Eur J Pediatr Surg. 1995;5 Suppl 1:35-38.

[5] Jeong DH, Kim CH, Kim MO, Chung H, Kim TH, Jung HY. Arnold-Chiari malformation type III with meningoencephalocele: A case report. Ann Rehabil Med. 2014;38(3):401–404.

[6] Winston KR. Pediatric neurosurgery: pride and prejudice. Pediatr Neurosurg. 2000;32(2):58-68.

[7] Agrawal A, Mittal A, Kohali GB, Sampley S, Gupta A. Chiari III malformation. Pediatr Neurosurg. 2012;47(4):309–310.

[8] Rani H, Kulkarni AV, Rao RV, Patil P. Chiari III malformation: A rare case with review of literature. Fetal Pediatr Pathol. 2013;32(3):169–174.

[9] Mohanty A, Biswas A, Reddy M, Kolluri S. Expansile cranioplasty for massive occipital encephalocele. Child’s Nerv Syst. 2006;22(9):1170–1176.

[10] Malkowicz DE, Myers G, Leisman G. Rehabilitation of cortical visual impairment in children. Int J Neurosci. 2006;116(9):1015-1033.

[11] Hoyt CS. Visual function in the brain-damaged child. Eye (Lond). 2003;17(3):369-384.

[12] Caesarita DP, Bakhtiar Y, Prihatningtias R. Visual function outcome post intracranial surgery in sellar region brain tumor. Bali Medical Journal. 2021;10(1):309-313.

[13] Aritonang C, Putri SA, Utomo B. The effectiveness of using peek acuity in vision screening for the admission of elementary school students. Bali Medical Journal. 2022;11(2):671-675.

[14] Hafizhan, Bakhtiar Y, Prihatningtias R. Visual function recovery post decompression surgery in meningioma: a case report. Bali Medical Journal. 2021;10(1):429-432.

Chiari Malformation (CM) type III surgical management in infant: a case report

Abstract

References

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