Arnold chiari malformation Type II – A case report

Type II Arnold-Chiari malformation (ACM) is an abnormality in which the cerebellum, pons, and medulla oblongata are displaced downward into the spinal cord. Type II ACM is often complicated by respiratory depression, sleep-disordered breathing, and deglutition disorder as a result of medullary dysfunction and impairment of the lower cranial nerves. Bending and stretching of the neck is restricted, and anesthetic management is problematic in patients with the disorder. We performed dental treatment twice under intravenous sedation in a patient with intellectual disability with type II ACM complicated by hypercapnic respiratory failure. Propofol was used for the first sedation procedure. Repeated bouts of respiratory depression occurred on that occasion, so the airway was managed manually by lifting the jaw. However, aspiration pneumonitis occurred postoperatively. A combination of dexmedetomidine and midazolam was used for sedation on the second occasion, and the intervention was completed uneventfully without any respiratory depression. Our experience with this patient highlights the need for selection of an agent for intravenous sedation that does not require neck extension and has minimal effect on respiration in patients with type II ACM, who are at high risk of respiratory depression and pulmonary aspiration.

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Mesencephalic spur (beaking deformity of the tectum) in Arnold-Chiari malformation

Journal of Neurosurgery ◽

10.3171/jns.1976.45.3.0315 ◽

1976 ◽

Vol 45 (3) ◽

pp. 315-320 ◽

Cited By ~ 11

Author(s):

Adelola Adeloye

Keyword(s):

Chiari Malformation ◽

Type Ii ◽

Content Type ◽

Arnold Chiari Malformation ◽

Degrees Of Severity ◽

Fine Print

✓ The beaking deformity of the tectum, referred to in this paper as the mesencephalic spur, is regularly present in the Type II variety of the Arnold-Chiari malformation in varying degrees of severity. When the mesencephalic spur is related to associated myelomeningocele, it was found that the more extensive the myelomeningocele, and the older the affected children, the more prominent was the mesencephalic spur.

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Arnold-Chiari malformation type II

10.32388/ro4vjx ◽

2020 ◽

Author(s):

Keyword(s):

Chiari Malformation ◽

Type Ii ◽

Arnold Chiari Malformation

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Arnold-Chiari Malformation Type II and CYP1B1 Congenital Glaucoma: A Possible Association

Case Reports in Ophthalmological Medicine ◽

10.1155/2021/4808346 ◽

2021 ◽

Vol 2021 ◽

pp. 1-3

Author(s):

Shaikha Aldossari ◽

Amani Al Bakri ◽

Yumna Kamal

Keyword(s):

Genetic Testing ◽

Chiari Malformation ◽

Primary Diagnosis ◽

Congenital Glaucoma ◽

Type Ii ◽

Nasal Bridge ◽

Arnold Chiari Malformation ◽

Ocular Symptoms ◽

Peripheral Hospital ◽

Eye Examination

Background. We describe a case of an infant with Arnold-Chiari Malformation Type II (ACM-II) who was born with lumbosacral myelomeningocele, hydrocephalus, and primary congenital glaucoma (PCG) together with dysmorphic features (scaphocephaly, frontal bossing, hypotelorism, entropion, and flat nasal bridge), which according to our knowledge, is a combination that has yet to be described in literature. Primary diagnosis. A 2-year-old female who is known to have ACM-II was referred due to abnormal eye examination done in a peripheral hospital that suggested infantile glaucoma in both eyes. Findings. During her last physical exam (postop), she was vitally stable, conscious with good feeding. Ophthalmic assessment revealed buphthalmia, superior paracentral scar, deep anterior chambers (AC), and round pupils with positive red reflex, clear lens, and an IOP of 16, 14 mm Hg, respectively. Neurological exam showed paraparesis and moving upper extremities and has axial hypotonia. Genetic testing showed CYP1B1 gene mutation. Conclusion. The aim of reporting this case is to share the findings in this infant as it may be a new association. The main learning message here is that ACM-II patients may present with certain ocular symptoms, including glaucoma-related ones that may mimic neurological disorders. This report brings information that could alert general practitioners, neurologists, and neurosurgeons. A deeper understanding of this rare disorder may aid the diagnosis of cases with similar characteristic physical findings by referring them to an ophthalmology clinic for further evaluation. Case presentation. A 2-year-old female who is known to have Arnold Chiari Malformation Type II (ACM- II) was referred due to abnormal eye examination done in a peripheral hospital that suggested infantile glaucoma in both eyes. MRI at 3 months of age showed lumbosacral myelomeningocele and hydrocephalus. Genetic testing confirmed a CYP1B1 mutation. These combinations of symptoms were never described in the literature before.

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