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Open access

A child with unilateral abducens nerve palsy and neurovascular compression in Chiari malformation type 1 resolved with posterior fossa decompression: illustrative case

Olivia A Kozel, Belinda Shao, Cody A Doberstein, Natalie Amaral-Nieves, Matthew N Anderson, Gita V Harappanahally, Michael A Langue, and Konstantina A Svokos

BACKGROUND

Unilateral cranial nerve (CN) VI, or abducens nerve, palsy is rare in children and has not been reported in association with Chiari malformation type 1 (CM1) in the absence of other classic CM1 symptoms.

OBSERVATIONS

A 3-year-old male presented with acute incomitant esotropia consistent with a unilateral, left CN VI palsy and no additional neurological symptoms. Imaging demonstrated CM1 without hydrocephalus or papilledema, as well as an anterior inferior cerebellar artery (AICA) vessel loop in the immediate vicinity of the left abducens nerve. Given the high risk of a skull base approach for direct microvascular decompression of the abducens nerve and the absence of other classic Chiari symptoms, the patient was initially observed. However, as his palsy progressed, he underwent posterior fossa decompression with duraplasty (PFDD), with the aim of restoring global cerebrospinal fluid dynamics and decreasing possible AICA compression of the left abducens nerve. Postoperatively, his symptoms completely resolved.

LESSONS

In this first reported case of CM1 presenting as a unilateral abducens palsy in a young child, possibly caused by neurovascular compression, the patient’s symptoms resolved after indirect surgical decompression via PFDD.

Open access

Management of failed Chiari decompression and intrasyringeal hemorrhage in Noonan syndrome: illustrative cases

Cody J. Falls, Paul S. Page, Garret P. Greeneway, Daniel K. Resnick, and James A. Stadler III

BACKGROUND

Noonan syndrome (NS) is a rare genetic RASopathy with multisystem implications. The disorder is typically characterized by short stature, distinctive facial features, intellectual disability, developmental delay, chest deformity, and congenital heart disease. NS may be inherited or arise secondary to spontaneous mutations of genes in the Ras/mitogen activated protein kinase signaling pathways.

OBSERVATIONS

Numerous case reports exist detailing the association between NS and Chiari I malformation (CM-I), although this relationship has not been fully established. Patients with NS who present with CM-I requiring operation have shown high rates reoperation for failed decompression. The authors reported two patients with NS, CM-I, and syringomyelia who had prior posterior fossa decompressions without syrinx improvement. Both patients received reoperation with successful outcomes.

LESSONS

The authors highlighted the association between NS and CM-I and raised awareness that patients with these disorders may be at higher risk for failed posterior fossa decompression, necessitating reoperation.

Open access

Spontaneous intracranial hypotension secondary to congenital spinal dural ectasia and genetic mosaicism for tetrasomy 10p: illustrative case

Peyton L. Nisson, Rhona Schreck, John M. Graham Jr., Marcel M. Maya, and Wouter I. Schievink

BACKGROUND

Spontaneous intracranial hypotension has historically been a poorly understood pathology that is often unrecognized and undertreated. Even more rarely has it been described in pediatric patients with an otherwise benign past medical history.

OBSERVATIONS

Herein the authors describe one of the youngest patients ever reported, a 2-year-old girl who developed severe headaches, nausea, and vomiting and experienced headache relief after lying down. Imaging revealed tonsillar herniation 14 mm below the foramen magnum, presumed to be a Chiari malformation, along with extensive dural cysts starting from thoracic level T2 down to the sacrum. She was found to have streaky skin pigmentary variation starting from the trunk down to her feet. Genetic analysis of skin biopsies revealed mosaicism for an isodicentric marker chromosome (10p15.3–10q11.2 tetrasomy) in 27%–50% of cells. After undergoing a suboccipital and cervical decompression at an outside institution, she continued to be symptomatic. She was referred to the authors’ hospital, where she was diagnosed with spontaneous intracranial hypotension.

LESSONS

After receiving a series of epidural blood patches, the patient experienced almost complete relief of her symptoms. To the authors’ knowledge, this is the first time this chromosomal anomaly has ever been reported in a living child, and this may represent a new genetic association with dural ectasia.

Open access

Noninvasive evaluation of craniovertebral junction instability in 2 patients following Chiari decompression with rigid C-collar immobilization: illustrative cases

Kerrin S. Sunshine, Theresa A. Elder, and Krystal L. Tomei

BACKGROUND

The complex Chiari malformation has been identified in a subset of Chiari patients at higher risk for worsening symptoms following Chiari decompression. Although parameters such as the clivoaxial angle and the perpendicular distance of the dens to the line from the basion to the inferoposterior part of the C2 body (pBC2) have been evaluated to help with the prediction of risk, the decision to pursue an occipitocervical fusion in lower-risk patients does not come without inherent risk.

OBSERVATIONS

The authors present 2 patients who had symptoms of worsening ventral brainstem compression following Chiari decompression, neither of whom was categorized in the highest risk category for occipitocervical instability. In addition, neither patient had gross instability on radiographic imaging. A trial with rigid C-collar immobilization provided relief of symptoms in both patients and allowed reassurance of the likelihood of success of occipitocervical fusion.

LESSONS

In patients without clear radiographic instability following Chiari decompression, a C-collar trial may provide a noninvasive option for assessing the potential success of occipitocervical fusion.