Meconium staining of the brainstem with open myelomeningocele

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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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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Arnold-Chiari malformation type II

10.32388/ro4vjx ◽

2020 ◽

Author(s):

Keyword(s):

Chiari Malformation ◽

Type Ii ◽

Arnold Chiari Malformation

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Arnold-Chiari Malformation Type II and CYP1B1 Congenital Glaucoma: A Possible Association

Case Reports in Ophthalmological Medicine ◽

10.1155/2021/4808346 ◽

2021 ◽

Vol 2021 ◽

pp. 1-3

Author(s):

Shaikha Aldossari ◽

Amani Al Bakri ◽

Yumna Kamal

Keyword(s):

Genetic Testing ◽

Chiari Malformation ◽

Primary Diagnosis ◽

Congenital Glaucoma ◽

Type Ii ◽

Nasal Bridge ◽

Arnold Chiari Malformation ◽

Ocular Symptoms ◽

Peripheral Hospital ◽

Eye Examination

Background. We describe a case of an infant with Arnold-Chiari Malformation Type II (ACM-II) who was born with lumbosacral myelomeningocele, hydrocephalus, and primary congenital glaucoma (PCG) together with dysmorphic features (scaphocephaly, frontal bossing, hypotelorism, entropion, and flat nasal bridge), which according to our knowledge, is a combination that has yet to be described in literature. Primary diagnosis. A 2-year-old female who is known to have ACM-II was referred due to abnormal eye examination done in a peripheral hospital that suggested infantile glaucoma in both eyes. Findings. During her last physical exam (postop), she was vitally stable, conscious with good feeding. Ophthalmic assessment revealed buphthalmia, superior paracentral scar, deep anterior chambers (AC), and round pupils with positive red reflex, clear lens, and an IOP of 16, 14 mm Hg, respectively. Neurological exam showed paraparesis and moving upper extremities and has axial hypotonia. Genetic testing showed CYP1B1 gene mutation. Conclusion. The aim of reporting this case is to share the findings in this infant as it may be a new association. The main learning message here is that ACM-II patients may present with certain ocular symptoms, including glaucoma-related ones that may mimic neurological disorders. This report brings information that could alert general practitioners, neurologists, and neurosurgeons. A deeper understanding of this rare disorder may aid the diagnosis of cases with similar characteristic physical findings by referring them to an ophthalmology clinic for further evaluation. Case presentation. A 2-year-old female who is known to have Arnold Chiari Malformation Type II (ACM- II) was referred due to abnormal eye examination done in a peripheral hospital that suggested infantile glaucoma in both eyes. MRI at 3 months of age showed lumbosacral myelomeningocele and hydrocephalus. Genetic testing confirmed a CYP1B1 mutation. These combinations of symptoms were never described in the literature before.

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Movement Disorder as Unusual Manifestation of Chiari Malformation Type II in a Newborn

Journal of Pediatric Neurology ◽

10.1055/s-0040-1718378 ◽

2020 ◽

Author(s):

Lara Mastino ◽

Roberto Mai ◽

Marco Cenzato ◽

Giuseppe D'Aliberti ◽

Giuseppe Talamonti

Keyword(s):

Head And Neck ◽

Movement Disorder ◽

Ventriculoperitoneal Shunt ◽

Movement Disorders ◽

Chiari Malformation ◽

Ventricular Dilatation ◽

Type Ii ◽

Unusual Manifestation ◽

Myelomeningocele Repair

AbstractChiari malformation type II (CM-II) is a hindbrain developmental malformation. Movement disorders are rarely described as associated with this condition. We describe the case of a newborn affected by CM-II presenting with head and neck tremors a few days after myelomeningocele repair surgery. Later, self-resolving episodes of expiratory apneas arose. Cranial ultrasounds showed progressive ventricular dilatation. She underwent ventriculoperitoneal shunt followed by craniocervical bony decompression. After surgery, both apnea and tremors disappeared. This particular presentation of CM-II is very rare and potentially difficult to diagnose.

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