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Open access

Successful treatment of unilateral facial nerve palsy in a pediatric patient with syringobulbia and Chiari malformation type I: illustrative case

Daniel Sherlock, Nolan J Brown, Alvin Y Chan, Jessica K Campos, and Joffre Olaya

BACKGROUND

Unlike syringomyelia, syringobulbia is not commonly observed in pediatric patients with Chiari malformation type I (CMI). Previous series have reported the incidence of syringobulbia as between 3% and 4% in these patients. Presentation is typically chronic, with the slow onset of neurological symptoms and cranial nerve (CN) palsies resulting from lower brainstem involvement. The authors report the first case of a pediatric patient with simultaneous CMI, syringobulbia, and unilateral CN VII palsy.

OBSERVATIONS

A 7-year-old male presented with right facial weakness in addition to headaches, ataxia, urinary incontinence, and falls. Magnetic resonance imaging revealed CMI with a syrinx of the cervicothoracic spine and syringobulbia. Posterior fossa decompression with duraplasty was performed without complications, and the patient was discharged home on postoperation day 5. At the 3-week follow-up, the patient’s neurological deficits had largely subsided. At the 3-month follow-up, his CN VII palsy and syringobulbia had completely resolved.

LESSONS

Pediatric CMI patients with syringomyelia are at risk for developing syringobulbia and brainstem deficits, including unilateral facial palsy. However, craniocervical decompression can prove successful in treating such deficits.

Open access

Coronavirus disease 2019–associated persistent cough and Chiari malformation type I resulting in acute respiratory failure: illustrative case

Anthony J Piscopo, Nahom Teferi, Anthony Marincovich, Meron Challa, and Brian J Dlouhy

BACKGROUND

Chiari malformation type I (CM-I) is the herniation of cerebellar tonsils through the foramen magnum, potentially resulting in the obstruction of cerebrospinal fluid flow and brainstem compression. Sleep-disordered breathing (SDB) is common in patients with CM-I, and symptomatic exacerbations have been described after Valsalva-inducing stressors. Acute decompensation in the setting of coronavirus disease 2019 (COVID-19) has not been described.

OBSERVATIONS

After violent coughing episodes associated with COVID-19 infection, a 44-year-old female developed several months of Valsalva-induced occipital headaches, episodic bulbar symptoms, and worsening SDB, which led to acute respiratory failure requiring mechanical ventilation. Imaging demonstrated 12 mm of cerebellar tonsillar descent below the foramen magnum, dorsal brainstem compression, and syringobulbia within the dorsal medulla. She underwent posterior fossa and intradural decompression with near-complete resolution of her symptoms 6 months postoperatively.

LESSONS

Although CM-I can remain asymptomatic, Valsalva-inducing stressors, including COVID-19 infection, can initiate or acutely exacerbate symptoms, placing patients at risk for CM-I–associated brainstem dysfunction and, in rare cases, acute respiratory failure. Worsening Valsalva maneuvers can contribute to further cerebellar tonsil impaction, brainstem compression, syringomyelia/syringobulbia, and worsening CM-I intradural pathology. Ventilator support and timely decompressive surgery are paramount, as brainstem compression can reduce central respiratory drive, placing patients at risk for coma, neurological deficits, and/or death.

Open access

Protracted respiratory failure in a case of global spinal syringomyelia and Chiari malformation following administration of diazepam: illustrative case

Luke Bauerle, Brandon Rogowski, Aakash Shingala, Habib Emil Rafka, Timothy Webb, Brian F Saway, Edward F Kilb, Julio A Chalela, and Nathan C Rowland

BACKGROUND

Syringomyelia is defined as dilation of the spinal cord’s central canal and is often precipitated by skull base herniation disorders. Although respiratory failure (RF) can be associated with skull base abnormalities due to brainstem compression, most cases occur in pediatric patients and quickly resolve. The authors report the case of an adult patient with global spinal syringomyelia and Chiari malformation who developed refractory RF after routine administration of diazepam.

OBSERVATIONS

A 31-year-old female presented with malnutrition, a 1-month history of right-sided weakness, and normal respiratory dynamics. After administration of diazepam prior to magnetic resonance imaging (MRI), she suddenly developed hypercapnic RF followed MRI and required intubation. MRI disclosed a Chiari malformation type I and syrinx extending from C1 to the conus medullaris. After decompressive surgery, her respiratory function progressively returned to baseline status, although 22 months after initial benzodiazepine administration, the patient continues to require nocturnal ventilation.

LESSONS

Administration of central nervous system depressants should be closely monitored in patients with extensive syrinx formation given the potential to exacerbate diminished central respiratory drive. Early identification of syrinx in the context of Chiari malformation and hemiplegia should prompt clinical suspicion of underlying respiratory compromise and early involvement of intensive care consultants.

Open access

Fully endoscopic posterior fossa decompression for Chiari malformation type I: illustrative case

Daniel Staribacher, Guenther C Feigl, Gavin Britz, and Dzmitry Kuzmin

BACKGROUND

Surgery for symptomatic Arnold-Chiari malformation type I involves posterior fossa decompression. There are various approaches, including endoscope-assisted ones. New possibilities and fields of application of fully endoscopic techniques are currently being developed since new and advanced endoscopic equipment and instrumentation are available.

OBSERVATIONS

The authors describe the case of a fully endoscopic microsurgical procedure in a 30-year-old female patient with progressive vertigo who was diagnosed with Chiari malformation type I. Neuronavigation and neuromonitoring were used during the surgery.

LESSONS

Fully endoscopic surgery can be successfully performed in patients with Chiari malformation I. Intraoperative neuromonitoring and neuronavigation increase safety during this procedure.

Open access

Spontaneous improvement in syringomyelia in a patient with Chiari 1 malformation: illustrative case

Oyku Ozturk, Emetullah Cındıl, Hakan Emmez, Pelin Kuzucu, and Emrah Celtıkcı

BACKGROUND

“Chiari malformation” refers to a spectrum of hindbrain abnormalities characterized by impaired cerebrospinal fluid circulation through the foramen magnum. Syringomyelia is frequently found in patients with Chiari malformation type 1. Although many theories have suggested how cerebrospinal fluid enters and makes the cystic cavity in the spinal cord, the pathogenesis of syringomyelia remains controversial. This report documents a case with spontaneous resolution of syringomyelia followed up by 3-year serial magnetic resonance imaging (MRI). These kinds of cases support a more conservative approach.

OBSERVATIONS

A 59-year-old female presented to the authors’ clinic in June 2019 with a history of Chiari malformation type 1. This symptomatic patient has been followed up with serial MRI. When the last MRI was performed in August 2022, compared with previous imaging, resolution of the syringomyelia was recognized.

LESSONS

Because the natural evolution of mildly symptomatic/asymptomatic patients with syringes is unclear, these patients pose a treatment dilemma. Although surgical intervention is a widely accepted therapeutic method, a more conservative approach can be considered in cases with spontaneous resolution. Especially for patients without progressive symptoms, the surgical approach should not be considered as the first step. In view of relapses, follow-up with periodic neurological examinations and radiological imaging is preferrable.

Open access

Spinal cord stimulator for the treatment of central neuropathic pain secondary to cervical syringomyelia: illustrative case

Bryan A. Schatmeyer, Rakan Dodin, Michael Kinsman, and David Garcia

BACKGROUND

Central neuropathic pain (CNP) of the cervical and/or thoracic spinal cord has many etiologies, both natural and iatrogenic. Frequently, CNP is medically refractory and requires surgical treatment to modulate the perception of pain. Spinal cord stimulation is a modality commonly used in adults to treat this type of refractory pain; however, it is rarely used in the pediatric population.

OBSERVATIONS

The authors reported a case involving a common pediatric condition, Chiari malformation type I with syrinx, that led to a debilitating complex regional pain syndrome. The associated life-altering pain was successfully alleviated following placement of a spinal cord stimulator.

LESSONS

CNP, or the syndromic manifestations of the pain (complex regional pain syndrome), can alter an individual’s life in dramatic ways. Spinal cord stimulator placement in carefully selected pediatric patients should be considered in these difficult pain treatment paradigms.

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

Increase in clivo-axial angle is associated with clinical improvement in children undergoing occipitocervical fusion for complex Chiari malformation: patient series

Neelan J. Marianayagam, John K. Chae, Ibrahim Hussain, Amanda Cruz, Ali A. Baaj, Roger Härtl, and Jeffrey P. Greenfield

BACKGROUND

The authors analyzed the pre- and postoperative morphometric properties of pediatric patients with complex Chiari malformation undergoing occipitocervical fusion (OCF) to assess clinical outcomes and morphometric properties that might influence postoperative outcomes.

OBSERVATIONS

The authors retrospectively reviewed 35 patients younger than 22 years with Chiari malformation who underwent posterior fossa decompression and OCF with or without endoscopic endonasal odontoidectomy at their institution (13 with and 22 without odontoidectomy). Clivo-axial angle (CXA), pB-C2, atlantodental interval, basion-dens interval, basion-axial interval, and canal diameter at the level of C1 were measured on preoperative and approximately 3-month postoperative computed tomography or magnetic resonance imaging. The authors further stratified the patient cohort into three age groups and compared the three cohorts. The most common presenting symptoms were headache, neck/shoulder pain, and dysphagia; 80% of the cohort had improved clinical outcomes. CXA increased significantly after surgery. When stratified into those who showed postoperative improvement and those who did not, only the former showed a significant increase in CXA. After age stratification, the significant changes in CXA were observed in the 7- to 13-year-old and 14- to 21-year-old cohorts.

LESSONS

CXA may be the most important morphometric predictor of clinical outcomes after OCF in pediatric patients with complex Chiari malformation.

Open access

Predicting clinical outcomes using morphometric changes in adults with complex Chiari malformation undergoing occipitocervical fusion with or without ventral decompression: patient series

John K. Chae, Neelan J. Marianayagam, Ibrahim Hussain, Amanda Cruz, Ali A. Baaj, Roger Härtl, and Jeffrey P. Greenfield

BACKGROUND

The authors assessed the connection between clinical outcomes and morphometrics in patients with complex Chiari malformation (CM) who have undergone posterior fossa decompression (PFD) and subsequent occipitocervical fusion (OCF) with or without ventral decompression (VD).

OBSERVATIONS

The authors retrospectively reviewed 33 patients with CM aged over 21 years who underwent PFD and OCF with or without endoscopic endonasal odontoidectomy at the authors’ institution (21 OCF only and 12 OCF + VD). Clivoaxial angle (CXA), pB-C2 (perpendicular line to the line between the basion and C2), atlantodental interval (ADI), basion-dens interval (BDI), basion-axial interval (BAI), and C1 canal diameter were measured on preoperative and approximately 3-month postoperative computed tomography or magnetic resonance imaging scans. Common symptoms included headache, paresthesia, and bulbar symptoms. Clinical improvement after surgery was observed in 78.8% of patients. CXA, ADI, and BDI all significantly increased after surgery, whereas pB-C2 and BAI significantly decreased. OCF + VD had a significantly more acute CXA and longer pB-C2 preoperatively than OCF only. Patients who clinically improved postoperatively showed the same significant morphometric changes, but those who did not improve showed no significant morphometric changes.

LESSONS

Patients showing improvement had greater corrections in skull base morphometrics than those who did not. Although there are various mutually nonexclusive reasons why certain patients do not improve after surgery, smaller degrees of morphometric correction could play a role.

Open access

Delayed chemical meningitis after Chiari decompression with duraplasty: illustrative cases

Orgest Lajthia, Mithun G. Sattur, and Abhay K. Varma

BACKGROUND

Dural reconstruction to achieve expansion duraplasty is important in suboccipital decompression for Chiari malformation type 1 (CM1). Although various dural substitutes are available, including synthetic collagen matrix grafts and dural xenografts, they have the potential to induce an inflammatory response. In this case series, the authors present their experience and discuss the incidence and possible mechanism of aseptic meningitis after the use of bovine collagen matrix graft as a dural substitute in patients with CM1 after suboccipital decompression.

OBSERVATIONS

Three consecutive adult female patients who underwent suboccipital decompression at a single institution by a single neurosurgeon were retrospectively reviewed. They all presented with signs of aseptic meningitis in a delayed fashion, responded well to steroid administration, but had recurrence of their symptoms. Bovine collagen dural substitutes are resorbed in a process that induces an inflammatory response manifesting with signs of aseptic meningitis and is only alleviated with removal of the dural substitute.

LESSONS

DuraMatrix Suturable, a dural xenograft derived from bovine dermis, though a viable choice for dural repair, is a potential cause of chemical meningitis after duraplasty in Chiari decompression surgery. In patients presenting with delayed and persistent aseptic meningitis after intervention, removal of this dural substitute led to improved symptomatology.