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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

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

A multilevel posterior tension band–sparing laminectomy for intraspinal lesions: patient series

Ignacio J Barrenechea, Luis Márquez, Sabrina Miralles, Héctor P Rojas, Julián Pastore, Pablo Vincenti, and Telmo Nicola

BACKGROUND

Minimally invasive spine surgery (MISS) represents a major development in spinal tumor surgery. However, considering that many intradural lesions compromise multiple spinal segments, MISS has certain limitations. Thus, some intraspinal lesions still require traditional approaches. Because laminectomy has been shown to predispose patients to kyphosis, laminoplasty and hemilaminectomy are the most widely used approaches to preserve the posterior tension band (PTB). However, these techniques are not devoid of complications. To overcome these issues, the authors modified a previously described technique to preserve the PTB while removing various types of intradural lesions. This procedure was originally designed to treat lumbar stenosis and was modified to avoid muscle ischemia during long procedures.

OBSERVATIONS

Between 2014 and 2021, the authors found 17 cases of spinal lesions with a minimum of 2 years of follow-up after surgical treatment using their approach. No significant postoperative changes in the paraspinal Goutallier grade or spinal angles were observed. The cross-sectional area of the measured paraspinal muscles decreased 6% postoperatively. By performing certain technical modifications in this PTB-sparing (PBS) laminectomy, the authors avoided ipsilateral muscle ischemia.

LESSONS

In this initial series, PBS laminectomy proved to be a safe, versatile, inexpensive, and reliable technique to remove intraspinal lesions.

Open access

Single-level ossified ligamentum flavum causing a holocord syrinx: illustrative case

Prashant Punia, Ashish Chugh, Sarang Gotecha, and Apurva Lachake

BACKGROUND

Syringomyelia is a neurological disorder that is caused by abnormal cerebrospinal fluid flow or circulation. It is an incidental finding in most cases, predominantly presenting with sensory symptoms of insensitivity to pain and temperature. Spinal ossified ligamentum flavum (OLF) leading to syringomyelia is one of the rare causes. The authors report an unusual case of syringomyelia due to a thoracic OLF.

OBSERVATIONS

A 54-year-old female presented with backache, difficulty walking, spasticity in the bilateral lower limbs, tingling sensation in the bilateral lower limbs, and paraparesis for 5 years. Her radiological investigations were suggestive of an OLF causing a syrinx. She underwent laminectomy, and her syrinx resolved on subsequent follow-up.

LESSONS

A syrinx due to a single-level OLF is rare, and this uncommon cause should be kept in mind while formulating treatment plans.

Open access

Symptomatic thoracic epidural lipomatosis with syringomyelia: illustrative case

Lukasz Przepiorka, Katarzyna Wójtowicz, and Przemysław Kunert

BACKGROUND

Spinal epidural lipomatosis is a rare but well-described disease. Here the authors present an extraordinary co-occurrence of spinal epidural lipomatosis with syringomyelia that did not improve after laminoplasty but required duroplasty as a definitive treatment.

OBSERVATIONS

This is the seventh case in the literature describing a co-occurrence of spinal epidural lipomatosis and syringomyelia. The pragmatic argument for a causative relationship between 2 phenomena is syrinxes reduction after surgery for spinal epidural lipomatosis. Additionally, duroplasty for spinal epidural lipomatosis in the setting of syringomyelia has not previously been reported. Duroplasty offers much better reconstruction of the subarachnoid space as compared with extradural decompression surgeries. It is one of the treatment options for spinal epidural lipomatosis. Last, most of the patients described in the literature with secondary syringomyelia were women with diabetes mellitus.

LESSONS

The co-occurrence of spinal epidural lipomatosis and syringomyelia is extremely rare. Neurological deficits in such cases respond well to surgical treatment. Accordingly, each case described in the literature resulted in syringomyelia reduction or resolution after surgery. Duroplasty may be necessary in selected cases of spinal epidural lipomatosis. Close follow-up and ongoing management of patients with epidural lipomatosis is therefore important.

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

Coexisting ossification of the posterior longitudinal ligament, intramedullary hemangioblastoma, and syringomyelia of the cervical spine: illustrative case

Chi-Ruei Li, Cheng-Ying Lee, Wen-Yu Cheng, Hsin-Ni Li, Chih-Hsiang Liao, and Chiung-Chyi Shen

BACKGROUND

Ossification of the posterior longitudinal ligament (OPLL) is a rare but potentially devastating cause of severe spinal cord compression and degenerative cervical myelopathy. Because OPLL is rarely accompanied by prominent syringomyelia, when both are observed, other causes of syringomyelia should be considered. Simultaneous presentation of OPLL and hemangioblastoma of the cervical spine is a rare encounter and has never been reported in the English-language literature.

OBSERVATIONS

The authors present a case of a 64-year-old man with muscle weakness of the right upper limb and worsening dysesthesia of the right thumb and index finger. Noncontrast magnetic resonance imaging (MRI) of the cervical spine from another institution revealed OPLL from the C2 to C6 levels with severe spinal cord compression and prominent syringomyelia. Repeated MRI with contrast showed an intramedullary tumor, about 11 mm in diameter, at the right posterior aspect of the C4 level. The authors performed laminectomies from C1 to C6 with posterolateral fusion and removed the C4 tumor. Pathohistological examination of the tumor demonstrated hemangioblastoma.

LESSONS

Careful evaluation of the preoperative imaging study is extremely important in surgical decision making. Although rare, concomitant cervical hemangioblastoma should be listed in the differential diagnosis when OPLL is accompanied with prominent syringomyelia.

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

Cervical cerebrospinal fluid venous fistula with syringomyelia treated with suboccipital decompression: illustrative case

Chia-Jung Hsieh, Lu-Ting Kuo, Dar-Ming Lai, and Abel Po-Hao Huang

BACKGROUND

Cerebrospinal fluid (CSF) venous fistulas are a recently discovered and underdiagnosed cause of spontaneous spinal CSF leak, which may lead to spontaneous intracranial hypotension. Most cases occur in the thoracic spine, and only 2 cases were reported in the cervical spine. Treatments include the epidural blood patch, fibrin glue injection, and surgical ligation of the fistula.

OBSERVATIONS

The authors report the treatment of a C6–7 CSF venous fistula, for which direct ligation was not feasible, with suboccipital decompression, leading to the complete resolution of the symptoms. Based on the clinical course and outcome in our patient, the authors summarize the previous theory and propose a hypothesis for the pathophysiology of headache and other symptoms in patients with CSF venous fistulas.

LESSONS

The symptoms of CSF venous fistulas may be linked not only to intracranial hypotension but also to the altered CSF dynamics induced by tonsillar herniation. Suboccipital decompression should be considered as a potential treatment option, especially in patients with Valsalva-induced headache who show a poor response to surgical ligation, patients in whom surgical ligation is not feasible, and patients with foramen magnum obstruction. Further investigation of the pathophysiology of CSF venous fistulas is warranted and should be performed in the future.

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.