Review Article

Syringobulbia in Patients with Chiari Malformation Type I: A Systematic Review

Table 1

Summary of CM-I patients with syringobulbia.

Author, yearAge,
sex
ManifestationsExamination findingsExtent of SBExtent of SMOperationOutcomeFollow-up

Feinberg M., 20163, MTrismusCompletely normal aside from isolated trismusMedulla, right pontine tegmentumC1-T8Suboccipital craniectomy, C-1 laminectomyResolved1yr
Shankar, B., 201422, MPersistent singultusAtaxia, dysesthesiaMedullaHolocordDecompressionResolvedNone
Massimi, L., 201138, MAcute respiratory failureConsciousness, normal ICPMedullaHydrocephalus, holocordETVResolved3yrs
1, MHemiparesis, Horner syndromeLeft flaccid hemiparesis, myosis, ptosis, Horner syndromeMedulla, ponsCervicothoracicPFD, C-1 laminectomy, and duraplastyImproved3yrs
2.5, MTetraparesis, dyspnea, neck painTetraplegia, limb and trunk hypesthesiaMedulla, ponsCervicothoracicNot mentionedImproved10m
Massey, S. L., 20114, FEye inward deviation, neck tiltingCN VI, VII palsies, ataxia, limb spasticityMedulla, ponsCervicothoracicDecompressionAlleviated1.5yrs
Viswanatha, B., 200936, FDysarthria, headache, neck painCN IX, X palsies, dysarthriaMedulla, ponsHolocordFMDImproved1yr
Robert E., 200916, MTetraparesis, headaches, urinary retentionCN VI palsies, tetraparetic, limb weaknessMedullaCervicalLarge-volume shunt, PFD, laminectomy, duraplastyAlleviatedNot mentioned
14, MVomiting, facial weakness, diplopia, hoarseness, ataxiaCN VI and VII palsiesMedulla, ponsCervicothoracicSuboccipital craniectomy, C-1 laminectomy, fenestration, duraplastyImproved4 yrs
Seki, T., 200427, MTrunk numbness, limb weaknessHyperreflexia, dysesthesia(R)MedullaC2-C7PFD, laminectomy, duraplastyAlleviated5m
Aryan, H, 200455, MDysphagia, diplopia, gait difficultyBulbar, sensory, motor, and coordination deficitsMedullaC1-T8PFD, laminectomy, duraplasty, VP-shuntNo relief3m
Lee, J., 200116, MDysphagia, drowsyQuadriparesisMedulla, ponsC1-T11PFD, laminectomy, duraplasty, syringoperitoneal shuntRemission6m
Penarrocha, M., 200145, MOrofacial painHypoesthesiaMedullaCervicalDecompression, shuntResolved2yrs
Galarza, M, 20019, MLethargy and respiratory arrestQuadriparesisMedullaCervicalVentricle external drainageImprovedNot mentioned
Nogues, M., 200058, FDysesthesiaIncreased toneMedullaCervicothoracicNoneNoneNone
Takahashi, Y., 199915, MGait disturbance, dysphagiaHorizontal nystagmus, ataxia, limb weaknessMedullaCervicothoracicFMD, laminectomyImproved8yrs
Afifi, A, 199711, FDiplopia, snoringCN VI, XII palsies, arm hyporeflexia, leg hyperreflexiaMedulla, ponsCervicalPFD, laminectomy, duraplasty, 4th-V shuntImproved6m
Anwer, U, 199647, FDysphagia, tough numbnessHemiparalysis, Horner’s syndromeMedullaCervicothoracicPFDImproved2m
Kanev, P, 199413, FDiplopiaDiplopiaMedulla, ponsCervicalPFD, laminectomyImproved2m
Rhoton, E. L., 199169, FLimbs weakness and numbnessHemiparesis, dysesthesia, hyperreflexiaBrain stem, syringocephalyC2-C6craniectomy, laminectomy, 4th V shuntResolvedNot mentioned
Weissman, J., 199046, MArm weakness and Dysesthesia, dysarthria and dysphagiaTorsional nystagmus, vocal cord paralysisMedullaC2-T8Not mentionedNot mentionedNot mentioned
Okada, S., 198910, FLimbs weakness, dysesthesiaHemiparesis, dysesthesia, hyperreflexia, CN V palsiesBrain stem, syringocephalyC1-T10Craniectomy, laminectomy, duraplastyImproved1m
Bresnan, M., 198717, Flimbs numbness, nausea, vomiting, diplopia, oscillopsia, dysarthriaRotatory nystagmus, CN V-VII palsiesMedullaC2-T4Decompressed, stent shunt, duraplastyImproved1m

C: cervical, CN: cranial nerve, ETV: endoscopic third ventriculostomy, F: female, FMD: foramen magnum decompression, ICP: intracranial pressure, M: male, m: month(s), PDF: posterior foramen decompression, SB: syringobulbia, SM: syringomyelia, T: thoracic, yrs: year(s), and VP: ventriculoperitoneal.