Sequential morphological change of Chiari malformation type II following surgical repair of myelomeningocele

Object Postnatal improvement in Chiari malformation type II (CM-II) following surgical repair of myelomeningocele was evaluated. Methods The authors reviewed data obtained in 20 cases in which patients underwent postnatal myelomeningocele repair within the first 48 hours after birth between October 2002 and September 2006. In 14 patients (Group 1), myelomeningocele was diagnosed in utero and the infants were delivered by cesarean section at 35–39 weeks' gestation (mean 36.4). The 6 infants in Group 2 were born after full-term gestation (39–41 weeks), and their myelomeningoceles were diagnosed postnatally. In all 20 patients, the myelomeningoceles were surgically repaired postnatally. Dynamic change of the herniated cerebellar tonsils in CM-II before and after the myelomeningocele repair, associated hydrocephalus, and symptomatic CM-II were analyzed. Results In Group 1, the CM-II was confirmed before myelomeningocele repair in 13 cases (93%). The spinal level of the caudal end of the cerebellar tonsils ranged from C-2 to C-7. Ascent of the cerebellar tonsils was observed in 11 patients (range 1–4 spinal levels, mean 2 levels) and continued even after ventriculoperitoneal (VP) shunt placement in most patients. A VP shunt was required for the treatment of hydrocephalus in 12 patients (86%). Symptomatic CM-II developed in 8 of 13 patients (61%), 3 of whom required surgical decompression. In Group 2, CM-II was confirmed in 5 infants (83%), with the cerebellar tonsils at a spinal level of C-2 to C-4 or C-5. Ascent of the cerebellar tonsils was observed in 4 patients (range 1–1.5 spinal levels, average 1.1 levels), and no patient had symptomatic CM-II. A VP shunt was placed in 5 patients (83%). No patient was lost to follow-up during the 18-month follow-up period. The only statistically significant difference between the 2 groups was the presence of symptomatic CM-II in Group 2 (p = 0.02). Conclusions Patients showed ascent of the cerebellar tonsils after postnatal myelomeningocele repair. Placement of a VP shunt helped promote the ascent. However, postnatal myelomeningocele repair in the patients in Group 1 failed to consistently prevent development of symptomatic CM-II. This limited experience suggests that postnatal repair of myelomeningocele can partially reverse the anatomical CM-II, but symptomatic CM-II cannot be prevented in some patients when the repair is performed after 36 weeks' gestation.

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Congenital stridor in the context of Chiari malformation Type II: the etiological role of vernix caseosa granulomatous meningitis

Journal of Neurosurgery Pediatrics ◽

10.3171/2011.7.peds11208 ◽

2011 ◽

Vol 8 (4) ◽

pp. 372-376 ◽

Cited By ~ 7

Author(s):

Amelie I. Stritzke ◽

Christopher P. Dunham ◽

John A. Smyth ◽

Paul Steinbok

Keyword(s):

Neural Tube ◽

Neural Tube Defect ◽

Chiari Malformation ◽

Surgical Repair ◽

Type Ii ◽

Late Preterm Infant ◽

Vernix Caseosa ◽

Decompression Procedure ◽

Infant Girl

The authors describe the case of a late preterm infant girl who presented prenatally with a low lumbar neural tube defect and features of Chiari malformation type II (CM-II). At birth, she exhibited stridor and underwent surgical repair of a lumbosacral myelomeningocele on Day 2 of life. The prognosis was deemed to be poor, and hence a “Chiari decompression” procedure was not undertaken. The patient was subsequently extubated and died on Day 10. Postmortem findings included a rarely described but characteristic granulomatous meningitic reaction to vernix caseosa, which presumably entered the subarachnoid space and spinal cord syrinx antenatally via the open neural tube defect. The significance of congenital stridor in the context of CM-II and in particular the role of vernix caseosa granulomatous meningitis are examined. The antenatal repair of myelomeningoceles, as championed by some, may prevent this ominous meningitic complication.

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Meconium staining of the brainstem with open myelomeningocele

Journal of Neurosurgery Pediatrics ◽

10.3171/2012.10.peds1293 ◽

2013 ◽

Vol 11 (2) ◽

pp. 150-153 ◽

Cited By ~ 1

Author(s):

Sandi Lam ◽

Ramesh Grandhi ◽

Stephanie Greene

Keyword(s):

Chiari Malformation ◽

Perinatal Care ◽

Type Ii ◽

Meconium Staining

Meconium staining of open myelomeningoceles has been reported to occur both prenatally and postnatally, but meconium staining of the brainstem has not been previously documented. The authors present a case of meconium staining of the brainstem in an infant with a meconium-stained myelomeningocele, Chiari malformation Type II, and hydrocephalus and discuss possible implications for prenatal and perinatal care.

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Sleep disorder: a possible cause of attention deficit in children and adolescents with Chiari malformation type II

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x2009000100008 ◽

2009 ◽

Vol 67 (1) ◽

pp. 29-34 ◽

Cited By ~ 9

Author(s):

Paulo Sérgio Azeredo Henriques Filho ◽

Riccardo Pratesi

Keyword(s):

Sleep Apnea ◽

Sleep Disorders ◽

Attention Deficit ◽

Chiari Malformation ◽

Sleep Apnea Syndrome ◽

Rem Sleep Behavior Disorder ◽

Behavior Disorder ◽

Type Ii ◽

Sleep Behavior ◽

Apnea Syndrome

BACKGROUND: Attention deficit may be related to sleep disorders in Chiari malformation type II (CMII). Our aim is identify sleep disorders and their specific contribution in attention deficit. METHOD: We selected 24 patients with CM II and 24 without CM II. DSM-IV criteria and a neuropsychological analysis were applied in all. All patients underwent full night polysomnography. RESULTS: 14 CM II patients presented sleep apnea syndrome, REM sleep behavior disorder and periodic limb movement in sleep; six patients without CM II presented sleep apnea syndrome. Among these patients, 12 (six with CM II and six without CM II) presented attention deficit related to the sleep disorders. CONCLUSION: Sleep disorders may impair cognitive functions, as attention, and contribute to poor quality of learning also in patients with CM II.

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Morphometric analysis of posterior fossa after in utero myelomeningocele repair

Journal of Neurosurgery Pediatrics ◽

10.3171/2011.1.peds10234 ◽

2011 ◽

Vol 7 (4) ◽

pp. 362-368 ◽

Cited By ~ 19

Author(s):

Ryan A. Grant ◽

Gregory G. Heuer ◽

Geneive M. Carrión ◽

N. Scott Adzick ◽

Erin S. Schwartz ◽

...

Keyword(s):

Posterior Fossa ◽

Surgical Repair ◽

Correlation Coefficients ◽

In Utero ◽

Clinical Effects ◽

Type Ii ◽

Tonsillar Herniation ◽

Myelomeningocele Repair ◽

Repair Group

Object Myelomeningocele (MMC) is characterized by a defect in caudal neurulation and appears at birth with a constellation of neuroanatomical abnormalities, including Chiari malformation Type II. The authors investigated the effects of antenatal versus postnatal repair of MMC through a quantitative analysis of morphometric changes in the posterior fossa (PF). Methods The authors retrospectively reviewed the records of 29 patients who underwent in utero MMC repair, 24 patients who underwent postnatal repair, and 114 fetal and pediatric controls. Tonsillar displacement, cerebellum length, pons length, clivus-supraocciput (CSO) angle, and PF area were compared in antenatal and postnatal MMC repair groups as well as in controls without neural tube defects by using t-tests and correlation coefficients. Results Initially, the in utero CSO angle was significantly more acute in all patients with MMC—prenatally and postnatally repaired—as compared with controls (57.8° vs 75.4°, p < 0.001); however, the angle rapidly changed and became similar to that in controls between 30 and 31 weeks' gestation to approximately 80°, with antenatal repair having little effect. Postnatally, the CSO angle decreased in controls (R = −0.58) and in the antenatal repair group (R = −0.17). The cerebellum and pons length demonstrated no significant differences in any group. Overall, tonsil descent was corrected in the antenatal repair group as compared with postnatal repair (p < 0.001), and the PF area increased in all 3 groups in utero. Growth was less rapid in patients with MMC compared with controls, but this was corrected by antenatal repair (p = 0.015). Conclusions Myelomeningocele was associated with tonsillar herniation and a smaller PF than in control fetuses. Antenatal surgical repair corrected both abnormalities. The CSO angle began significantly more acutely in patients with MMC, but normalized with development regardless of when surgery was performed. Determining the clinical effects of antenatal repair requires further follow-up.

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Intravenous Sedation in Arnold-Chiari Malformation With Respiratory Failure

Anesthesia Progress ◽

10.2344/anpr-66-01-06 ◽

2019 ◽

Vol 66 (1) ◽

pp. 37-41 ◽

Cited By ~ 2

Author(s):

Yoshiki Shionoya ◽

Eishi Nakamura ◽

Takahiro Goi ◽

Kiminari Nakamura ◽

Katsuhisa Sunada

Keyword(s):

Respiratory Failure ◽

Respiratory Depression ◽

Chiari Malformation ◽

Dental Treatment ◽

Anesthetic Management ◽

Cranial Nerves ◽

Intravenous Sedation ◽

Type Ii ◽

Neck Extension ◽

Arnold Chiari Malformation

Type II Arnold-Chiari malformation (ACM) is an abnormality in which the cerebellum, pons, and medulla oblongata are displaced downward into the spinal cord. Type II ACM is often complicated by respiratory depression, sleep-disordered breathing, and deglutition disorder as a result of medullary dysfunction and impairment of the lower cranial nerves. Bending and stretching of the neck is restricted, and anesthetic management is problematic in patients with the disorder. We performed dental treatment twice under intravenous sedation in a patient with intellectual disability with type II ACM complicated by hypercapnic respiratory failure. Propofol was used for the first sedation procedure. Repeated bouts of respiratory depression occurred on that occasion, so the airway was managed manually by lifting the jaw. However, aspiration pneumonitis occurred postoperatively. A combination of dexmedetomidine and midazolam was used for sedation on the second occasion, and the intervention was completed uneventfully without any respiratory depression. Our experience with this patient highlights the need for selection of an agent for intravenous sedation that does not require neck extension and has minimal effect on respiration in patients with type II ACM, who are at high risk of respiratory depression and pulmonary aspiration.

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Surgical management of Chiari malformation type II

Child s Nervous System ◽

10.1007/s00381-020-04675-7 ◽

2020 ◽

Vol 36 (8) ◽

pp. 1621-1634 ◽

Cited By ~ 1

Author(s):

Giuseppe Talamonti ◽

Eleonora Marcati ◽

Lara Mastino ◽

Giulia Meccariello ◽

Marco Picano ◽

...

Keyword(s):

Surgical Management ◽

Chiari Malformation ◽

Type Ii

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Sleep-Disordered Breathing in Patients With Chiari Malformation Type II

CHEST Journal ◽

10.1378/chest.10039 ◽

2010 ◽

Vol 138 (4) ◽

pp. 318A

Author(s):

Muslim M. Al Saadi ◽

Shaikh M. Iqbal

Keyword(s):

Chiari Malformation ◽

Sleep Disordered Breathing ◽

Type Ii ◽

Disordered Breathing

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Mesencephalic spur (beaking deformity of the tectum) in Arnold-Chiari malformation

Journal of Neurosurgery ◽

10.3171/jns.1976.45.3.0315 ◽

1976 ◽

Vol 45 (3) ◽

pp. 315-320 ◽

Cited By ~ 11

Author(s):

Adelola Adeloye

Keyword(s):

Chiari Malformation ◽

Type Ii ◽

Content Type ◽

Arnold Chiari Malformation ◽

Degrees Of Severity ◽

Fine Print

✓ The beaking deformity of the tectum, referred to in this paper as the mesencephalic spur, is regularly present in the Type II variety of the Arnold-Chiari malformation in varying degrees of severity. When the mesencephalic spur is related to associated myelomeningocele, it was found that the more extensive the myelomeningocele, and the older the affected children, the more prominent was the mesencephalic spur.

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Improvement of obstructive sleep apneas caused by hydrocephalus associated with Chiari malformation Type II following surgery

Journal of Neurosurgery Pediatrics ◽

10.3171/2010.7.peds10207 ◽

2010 ◽

Vol 6 (4) ◽

pp. 336-339 ◽

Cited By ~ 18

Author(s):

Marco Luigetti ◽

Anna Losurdo ◽

Serena Dittoni ◽

Elisa Testani ◽

Salvatore Colicchio ◽

...

Keyword(s):

Chiari Malformation ◽

Third Ventricle ◽

Sleep Apnea Syndrome ◽

Respiratory Control ◽

Upper Airway ◽

Respiratory Pattern ◽

Ventricular Catheter ◽

Type Ii ◽

Obstructive Sleep ◽

Severe Airway Obstruction

Chiari malformation (CM) is the downward herniation of the caudal part of the cerebellum and/or medulla oblongata into the spinal canal. It can alter several neurological functions, including respiratory control and upper airway motility, and can be the cause of sleep-disordered breathing (SDB). The authors describe a 6-year-old boy affected by CM Type II associated with myelomeningocele who showed symptoms indicative of severe airway obstruction during sleep. Polysomnography revealed severe obstructive sleep apnea syndrome (OSAS). Magnetic resonance imaging demonstrated herniation of the cerebellar tonsils and diffuse ventricular dilation with a large pseudocystic formation in the third ventricle. Surgical marsupialization of the cystic wall was performed, associated with ventriculocystostomy and endoscopic replacement of the ventricular catheter. Polysomnography repeated 2 months after surgery revealed a striking improvement in the sleep-related respiratory pattern. The pathogenesis of OSAS was probably referable to a combination of CM and elevated intracranial pressure. However, the striking improvement of symptoms after ventriculoatrial shunt placement suggested that hydrocephalus plays a major role in this condition. Assessment and effective treatment of SDB is crucial in the care of patients with CM.

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