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

Anthony J Piscopo Department of Neurosurgery, University of Iowa Hospital and Clinics, Iowa City, Iowa

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Nahom Teferi Department of Neurosurgery, University of Iowa Hospital and Clinics, Iowa City, Iowa

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Anthony Marincovich Department of Neurosurgery, University of Iowa Hospital and Clinics, Iowa City, Iowa

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Meron Challa University of Iowa, Carver College of Medicine, Iowa City, Iowa; and

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Brian J Dlouhy Department of Neurosurgery, University of Iowa Hospital and Clinics, Iowa City, Iowa
University of Iowa, Carver College of Medicine, Iowa City, Iowa; and
Iowa Neuroscience Institute, Iowa City, Iowa

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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.

ABBREVIATIONS

BiPAP = bilevel positive airway pressure; CM-1 = Chiari malformation type I; COVID-19 = coronavirus disease 2019; CSF = cerebrospinal fluid; ICU = intensive care unit; MRI = magnetic resonance imaging; SDB = sleep-disordered breathing

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.

ABBREVIATIONS

BiPAP = bilevel positive airway pressure; CM-1 = Chiari malformation type I; COVID-19 = coronavirus disease 2019; CSF = cerebrospinal fluid; ICU = intensive care unit; MRI = magnetic resonance imaging; SDB = sleep-disordered breathing

Chiari malformation type I (CM-I) is characterized by herniation of the cerebellar tonsils >5 mm through the foramen magnum.1 Recent studies have estimated its prevalence radiologically between 0.24% and 3.6% of the population.2–4 This condition is often asymptomatic but can result in a host of clinical manifestations due to the obstruction of cerebrospinal fluid (CSF) outflow, syringomyelia, syringobulbia, and/or brainstem compression.5,6 Suboccipital headache is the most common symptom, which can be further exacerbated by Valsalva maneuver or cough, further displacing the tonsils and obstructing CSF flow.6,7

A close association has been described between CM-I and sleep-disordered breathing (SDB), with reports ranging from 24% to 75% of patients with symptomatic CM-I developing SDB over their lifetime.8,9 This intermittent respiratory distress is hypothesized to be due to brainstem dysfunction, which disrupts afferent and efferent ventilatory signals, respiratory drive chemoreceptors in the medulla, and the cranial nerves that control upper airway patency. These structures are disrupted in CM-I due to the obstruction of CSF outflow in the fourth ventricle, syringobulbia/syringomyelia, and cerebellar tonsil compression of the medulla.10,11

Various stressors that result in repeated Valsvala maneuvers, including exercise and cough-inducing upper respiratory infections, have been reported to exacerbate symptoms and precede respiratory arrest in patients with CM-I.12–16 This acute presentation often consists of progressively worsening occipital headaches, hypoxemia, hypercapnia, neurological deficits, and imaging studies demonstrating an enlarged fourth ventricle, cervical cord syrinx, and/or signs of posterior fossa crowding.17–20 Surgical decompression is considered the gold standard treatment with the goal of reversing the respiratory symptomatology, which has been reported in a number of cases.12,16,20–22

Although SDB and other stressors have been associated with respiratory distress in CM-I, respiratory failure as the initial presentation of CM-I has seldom been described, with only 4 known cases reported in the literature.19,23,24 To date, coronavirus disease 2019 (COVID-19) infection has numbered over 768 million cases worldwide and 6.9 million deaths primarily due to severe hypoxemia and acute respiratory distress syndrome.25,26 Infection with COVID-19 as the inciting event in a patient with CM-I, leading to SDB and loss of the CO2 drive to breathe during sleep, causing acute respiratory failure, has not been reported in the literature. We describe the case of a 44-year-old female patient with no known history of CM-I presenting several months after contracting and recovering from COVID-19 with worsening SDB and occipital Valsalva-induced headaches, which eventually resulted in acute respiratory failure. Magnetic resonance imaging (MRI) demonstrated CM-I, severe posterior fossa crowding, medullary compression, and syringobulbia, for which she underwent surgical decompression with near-complete resolution of symptoms at the 6-month follow-up.

Illustrative Case

History and Examination

A 44-year-old female presented with a past medical history significant for asthma, type II diabetes mellitus, hyperlipidemia, and several years of headaches controlled with over-the-counter analgesics. She contracted COVID-19 with severe coughing episodes, which she described as “the worst and most violent coughing” she had ever had. Two weeks later, she began intermittently awakening several times per night with room-spinning vertigo, loud snoring, and episodes of bowel and bladder incontinence. During these episodes, as witnessed by her husband, she also appeared to be gasping for breath and was unresponsive to stimulation, which were initially concerning for seizure-like activity. These awakenings gradually worsened, and over 5 months, episodes increased to three to four times per night and required two trips to the emergency department with symptoms of blurry vision, throbbing occipital headache, gait imbalance, upper- and lower-extremity paresthesias, facial numbness, confusion, and nocturnal apneic episodes. In the emergency department, physical examination revealed frequent coughing, coarse lung sounds, tachycardia, and tachypnea. Her white blood cell count, bicarbonate, blood urea nitrogen, and creatinine were 10.7, 35, 19, and 1.63, respectively. The patient was treated with ceftriaxone and azithromycin and was given 1 mg of atropine out of concern for bradycardia and potential community-acquired pneumonia. Electroencephalography and electrocardiography recordings were normal at this time.

Six months after her COVID-19 infection, her symptoms worsened significantly, and she was taken emergently to the emergency department. She appeared somnolent and was found to be profoundly hypercapnic (pCO2 90 mm Hg) and acidotic (pH 7.23) with episodes of hypoxia and bradycardia when she would intermittently fall asleep. She was subsequently placed on bilevel positive airway pressure (BiPAP), but her respiratory condition failed to improve, and she later required intubation. MRI revealed 12 mm of tonsillar descent below the foramen magnum consistent with CM-I as well as a cervicomedullary buckle and fluid-filled cavity in the lower brainstem suggestive of syringobulbia (Fig. 1A and B). After 2 days, her respiratory status improved and arterial blood gas values returned to normal limits, and she was extubated and weaned onto BiPAP; the patient spent a total of 11 days in the hospital before discharge.

FIG. 1
FIG. 1

Preoperative MRI revealing CM-I and syringobulbia. A: Sagittal T2-weighted MRI of the cervical spine showing 12 mm of tonsillar descent below the foramen magnum with dorsal brainstem compression and a syrinx (white arrow) within the dorsal aspect of the caudal medulla consistent with CM-I and syringobulbia. There is a dilated fourth ventricle and enlarged fourth ventricle roof angle (83°), findings seen in patients with CM-I with fourth ventricle CSF obstruction and brainstem dysfunction. Notice the small posterior fossa with an upward hook in the opisthion of the occipital bone (asterisk) contributing to posterior fossa crowding. B: Axial T2-weighted MRI of the cervical spine (level of dashed white line in A) showing a small syrinx in the dorsal caudal medulla (white arrow) consistent with syringobulbia. The location of the syrinx is close to the medullary respiratory centers, which is responsible for the generation of inspiratory movements, basic rhythm of breathing, and CO2 drive to breathe. C: Axial T2-weighted MRI demonstrating no hydrocephalus.

Six weeks after hospital discharge, she followed up in the neurosurgery clinic, again demonstrating occipital headaches worse with Valsalva maneuver (pre-COVID-19 headaches were not associated with Valsalva), swallowing dysfunction, upper-extremity paresthesias, absent gag response, positive Romberg sign, and gait imbalance. She reported that snoring and nighttime apneic episodes had decreased since regularly using BiPAP. The decision was then made to proceed with intradural Chiari decompression, followed by duraplasty after completing a workup with MRI of the neural axis to assess for a syrinx.

Procedure

The patient was placed prone with her head flexed. A midline linear incision was made craniocaudally from the external occipital protuberance to the C6 protuberance. A craniectomy was planned from the inferior nuchal line inferiorly to the foramen magnum as wide as the occipital condyles (Fig. 2A). The occipital-atlantal membrane was excised. Next, a diagonally shaped linear dural opening was made to the right of the midline, and the dura was pulled laterally to expose the tonsils bilaterally and fourth ventricle (Fig. 2B). The arachnoid veil appeared pathologically semiopaque (Fig. 2C). This was gently dissected, identifying the medulla and tonsils, which appeared flattened with adhesions and scar tissue (Fig. 2D and E). The adhesions were gently dissected and cut to mobilize the tonsils to open the fourth ventricle. Overlying the foramen of Magendie was an arachnoid veil, which was opened with sharp dissection and Rhoton microdissectors (Fig. 2E, F, and G). The tonsils were reduced in size and mobilized superiorly and laterally using low-power pia arachnoid bipolar cautery (Fig. 2H). A duraplasty was performed using harvested cervical fascia with 4-0 Nurolon in an interrupted manner while using Vistaseal as an additive dural sealant for watertight closure (Fig. 2I). The patient was extubated and transported without incident to the intensive care unit (ICU) for postoperative neuromonitoring.

FIG. 2
FIG. 2

Intraoperative photographs from the extradural and intradural decompression for CM-I illustrating the intradural pathology in this case. A: Suboccipital craniectomy was performed from the inferior nuchal line to the foramen magnum along with a C1 laminectomy. B and C: On opening the dura, the arachnoid was semitranslucent, suggesting some arachnoid scarring. D: Both right (R) and left (L) tonsils descended to below the lamina of C1 and filled the cisterna magna, covering the foramen of Magendie. E: Separation of the tonsils revealed thick adhesions (black arrow) and an arachnoid veil overlying the foramen of Magendie. F and G: After pulling up the right tonsil, fine arachnoid adhesions were attached to the tonsil and floor of the fourth ventricle surrounding the foramen of Magendie, preventing complete opening of the foramen. The veil was opened with sharp dissection. H: Low-power pia arachnoid bipolar cautery was used to reduce the tonsils and further open up the foramen of Magendie. I: After expansile duraplasty and closure with a cervical fascia graft (CFG). Pf = posterior fossa.

Clinical Course

Six months after surgery, the patient demonstrated near-complete resolution of all preoperative symptoms. She did not have any residual Valsalva-induced headaches, her balance was improved (on examination, she had no sway with Romberg or tandem Romberg), her preoperative facial numbness was absent, her gag response returned, and she did not report any residual extremity paresthesias. MRI of the brain and cervical spine 6 months postoperatively revealed a significantly improved position of the cerebellar tonsils above the foramen magnum with surrounding CSF signal (Fig. 3A). There was no more brainstem compression, the fourth ventricle was smaller than preoperatively due to opening of the fourth ventricle, the fourth ventricle roof angle was smaller, and the syringobulbia was resolved (Fig. 3A and B).

FIG. 3
FIG. 3

A: Postoperative sagittal T2-weighted MRI of the cervical spine revealing tonsillar ascent, smaller fourth ventricle and fourth ventricular roof angle (52°), and resolution of the brainstem compression and syringobulbia. B: Axial T2-weighted MRI of the cervical spine (level of dashed white line in A) illustrating resolution of the syrinx in the dorsal caudal medulla postoperatively.

Patient Informed Consent

The necessary patient informed consent was obtained in this study.

Discussion

Observations

As a result of tonsillar descent through the foramen magnum, CM-I can obstruct CSF flow or compress the brainstem, resulting in a host of clinical symptoms including apnea, diplopia, dysphagia, weakness and paresthesias, vertigo, and other brainstem dysfunctions.27 Brainstem compression in CM-I and syringobulbia pose significant risks of death from the suppression of cardiorespiratory centers in the dorsal respiratory group and a subsequent inability to respond to homeostatic stressors including changes in CO2, O2, and pH.28 Such patients often demonstrate severe hypoxemia, hypercapnia, and acidosis on blood gas analysis.29 Interestingly, postmortem medullary lesion studies in patients with sudden unexpected death attributed to craniovertebral junction disorders have demonstrated gross preservation of both sensory and motor pathways.30–32 This suggests that a defective response in medullary cardiorespiratory centers likely played a central role in the etiology of sudden deterioration and death in these cases. A similar mechanism has been implicated in the high prevalence of SDB in patients with known CM-I.8,9 Several studies have reported significant polysomnographic disturbances in patients with CM-I, with one study demonstrating a significant increase in both the apnea hypopnea index (13 ± 15 vs 3 ± 6, respectively; p = 0.007) and central apneic episodes (22% ± 30% vs 4% ± 8%, respectively; p = 0.009) compared with values in healthy controls. Furthermore, both syringomyelia and basilar invagination were independently associated with a worsened apnea hypopnea index and increased central apneic episodes (p = 0.001 and p = 0.003, respectively).33 After decompressive surgery, one study showed a significant decrease in the central apnea index from 14.9 to 1.3 (p = 0.03), and another demonstrated a decrease in apnea hypopnea index from 82.2 to 0.2 at 15 months postoperatively.9,34

Although a large percentage of patients with CM-I are asymptomatic, a sudden unexpected deterioration and/or death has been reported in CM-I, most often after surgery, infection, or other significant stressors such as pregnancy.30,32,35 The pathology of CM-I leading to tonsillar herniation, CMI-associated syringomyelia, and subsequent symptoms is not completely understood. However, studies have suggested that a combination of both extradural and intradural pathology contributes to its pathogenesis, often showing signs of posterior fossa crowding, brainstem compression, an enlarged fourth ventricle, or mechanical obstructions of CSF outflow.17,36,37 Extradural pathology is believed to result largely from developmental anomalies, resulting in decreased posterior fossa volume and subsequent tonsillar herniation and obstruction of CSF flow, as measured by radiographic metrics including anterior-posterior atlas assimilation, Chamberlain’s line, and slope of the tentorium cerebelli.38,39 Intradural pathology, although less well described, consists of a group of conditions that may be congenital or acquired, obstructing CSF flow at the level of the foramen magnum or foramen of Magendie, including arachnoid adhesions, obstructive cerebellar tonsils, vessel loops, or arachnoid veils.36

Although a number of conditions have been associated with acute exacerbations of symptomatology in CM-I, acute respiratory failure has been described in the literature fewer than five times and never in association with COVID-19.19,23,24 The patient reported herein was otherwise asymptomatic until she contracted COVID-19 with constant, severe coughing fits. Several weeks later, she began waking up one to two times per night confused and gasping for breath, which worsened over the following months and progressed with additional symptoms of gait instability, blurry vision, dysphagia, and vertigo. Cough due to COVID-19 is among the most common symptoms and can be mild, moderate, or severe and associated with pneumonia and interstitial lung disease in select cases.40

Our patient’s radiographic and intraoperative findings revealed various findings associated with both extradural and intradural pathology associated with CM-I (Fig. 1A and B and Fig. 2C–G). This can be appreciated on MRI, for example, a pathologically upgoing angle of the opisthion of the occipital bone (Fig. 1A asterisk) decreasing the patency of the foramen magnum. Furthermore, cough is often among the first symptoms to unmask a new diagnosis of CM-I or to worsen its severity in the case of a previous diagnosis.41,42 Additionally, a longitudinal, fluid-filled collection in the medulla (syringobulbia) can be seen on MRI, which has long been associated with CM-I and the obstruction of CSF flow (Fig. 1A and B). Syringobulbia is most often present in the medulla, and there have been several documented cases of severe, acute-onset cough in the setting of syringobulbia and CM-I.43 The dilated fourth ventricle, enlarged fourth ventricular roof angle, and brainstem compression were evident on MRI and have been associated with brainstem dysfunction (Fig. 1A).17

Intraoperatively, further evidence of intradural pathology and chronic changes associated with CM-I were evident. A semiopaque arachnoid veil was observed overlying the opening of the fourth ventricle, and copious scar tissue and adhesions were associated with the medulla and cerebellar tonsils, all of which have been described in association with CM-I (Fig. 2C–G).36 Arachnoid veils due to inherent scar tissue as part of CM-I or due to chronic changes in CM-I can obstruct CSF outflow and lead to syringomyelia/syringobulbia formation in such patients.36,44 The fluid-filled cavity can cause brainstem dysfunction within the dorsal medulla, contributing to sleep apnea and CO2 narcosis as the result of decreased ventilatory drive from the dorsal medullary response centers. This was evident in our patient, with increased CO2 levels ultimately requiring intubation (Fig. 1A and B). After decompressive surgery, the patient experienced near-complete symptom improvement at 6 months and clear radiological improvement (Fig. 3A and B).

Lessons

CM-I consists of herniation of the cerebellar tonsils through the foramen magnum and is intimately associated with brainstem compression, obstruction of CSF flow at the foramen of Magendie, syringomyelia/syringobulbia, and SDB. Although many patients are asymptomatic, certain stressors can exacerbate and unmask a new diagnosis, including Valsalva-inducing infections such as COVID-19. Further cerebellar tonsil descent related to frequent cough/Valsalva maneuvers associated with arachnoid scarring and veils overlying the fourth ventricle lead to worsening CSF obstruction. These can obstruct CSF flow and lead to syringomyelia/syringobulbia and can compress the lower brainstem and dorsal medullary respiratory centers, suppressing the normal ventilatory response. In rare cases, an acute Valsalva exacerbation from an infection, including pneumonia or COVID-19, or other stressors can lead to a rapid decline and respiratory failure in patients with CM-I. Timely triage to an emergency department and, in severe cases, intubation and ventilatory support in an ICU setting are critical in such patients with a decreased respiratory drive and response to CM-I–induced CO2 narcosis. Surgical decompression is the standard of treatment and has been shown to result in improvements in respiratory symptoms, Valsalva-associated headaches, SDB, and other CM-I–associated symptoms.

Author Contributions

Conception and design: Dlouhy, Piscopo, Marincovich. Acquisition of data: Dlouhy, Piscopo, Teferi, Marincovich. Analysis and interpretation of data: Dlouhy, Piscopo. Drafting the article: Dlouhy, Piscopo, Teferi, Challa. Critically revising the article: all authors. Reviewed submitted version of manuscript: all authors. Approved the final version of the manuscript on behalf of all authors: Dlouhy. Study supervision: Dlouhy.

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    Alperin N, Loftus JR, Oliu CJ, et al. Magnetic resonance imaging measures of posterior cranial fossa morphology and cerebrospinal fluid physiology in Chiari malformation type I. Neurosurgery. 2014;75(5):515522, discussion 522.

    • PubMed
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  • 38

    Diniz JM, Botelho RV. The role of clivus length and cranial base flexion angle in basilar invagination and Chiari malformation pathophysiology. Neurol Sci. 2020;41(7):17511757.

    • PubMed
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    • Export Citation
  • 39

    Alkoç OA, Songur A, Eser O, et al. Stereological and morphometric analysis of MRI Chiari malformation type-1. J Korean Neurosurg Soc. 2015;58(5):454461.

  • 40

    Nguyen-Ho L, Nguyen-Nhu V, Tran-Thi TT, Solomon JJ. Severe chronic cough relating to post-COVID-19 interstitial lung disease: a case report. Asia Pac Allergy. 2022;12(4):e42.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 41

    Milhorat TH, Chou MW, Trinidad EM, et al. Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 1999;44(5):10051017.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 42

    Patel K, Wani K, Daneshvar A, Omar J. Unusual etiology of chronic cough and syncope as Chiari malformation type 1. Cureus. 2023;15(6):e40598.

  • 43

    Menezes AH, Greenlee JDW, Dlouhy BJ. Syringobulbia in pediatric patients with Chiari malformation type I. J Neurosurg Pediatr. 2018;22(1):5260.

  • 44

    Tubbs RS, Smyth MD, Wellons JC, 3rd, Oakes WJ. Arachnoid veils and the Chiari I malformation. J Neurosurg. 2004;100(5 Suppl Pediatrics):465467.

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  • FIG. 1

    Preoperative MRI revealing CM-I and syringobulbia. A: Sagittal T2-weighted MRI of the cervical spine showing 12 mm of tonsillar descent below the foramen magnum with dorsal brainstem compression and a syrinx (white arrow) within the dorsal aspect of the caudal medulla consistent with CM-I and syringobulbia. There is a dilated fourth ventricle and enlarged fourth ventricle roof angle (83°), findings seen in patients with CM-I with fourth ventricle CSF obstruction and brainstem dysfunction. Notice the small posterior fossa with an upward hook in the opisthion of the occipital bone (asterisk) contributing to posterior fossa crowding. B: Axial T2-weighted MRI of the cervical spine (level of dashed white line in A) showing a small syrinx in the dorsal caudal medulla (white arrow) consistent with syringobulbia. The location of the syrinx is close to the medullary respiratory centers, which is responsible for the generation of inspiratory movements, basic rhythm of breathing, and CO2 drive to breathe. C: Axial T2-weighted MRI demonstrating no hydrocephalus.

  • FIG. 2

    Intraoperative photographs from the extradural and intradural decompression for CM-I illustrating the intradural pathology in this case. A: Suboccipital craniectomy was performed from the inferior nuchal line to the foramen magnum along with a C1 laminectomy. B and C: On opening the dura, the arachnoid was semitranslucent, suggesting some arachnoid scarring. D: Both right (R) and left (L) tonsils descended to below the lamina of C1 and filled the cisterna magna, covering the foramen of Magendie. E: Separation of the tonsils revealed thick adhesions (black arrow) and an arachnoid veil overlying the foramen of Magendie. F and G: After pulling up the right tonsil, fine arachnoid adhesions were attached to the tonsil and floor of the fourth ventricle surrounding the foramen of Magendie, preventing complete opening of the foramen. The veil was opened with sharp dissection. H: Low-power pia arachnoid bipolar cautery was used to reduce the tonsils and further open up the foramen of Magendie. I: After expansile duraplasty and closure with a cervical fascia graft (CFG). Pf = posterior fossa.

  • FIG. 3

    A: Postoperative sagittal T2-weighted MRI of the cervical spine revealing tonsillar ascent, smaller fourth ventricle and fourth ventricular roof angle (52°), and resolution of the brainstem compression and syringobulbia. B: Axial T2-weighted MRI of the cervical spine (level of dashed white line in A) illustrating resolution of the syrinx in the dorsal caudal medulla postoperatively.

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    • Export Citation
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    • Export Citation
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    Milhorat TH, Chou MW, Trinidad EM, et al. Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 1999;44(5):10051017.

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    • Export Citation
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    Patel K, Wani K, Daneshvar A, Omar J. Unusual etiology of chronic cough and syncope as Chiari malformation type 1. Cureus. 2023;15(6):e40598.

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