Prenatal diagnosis of Chiari malformation with syringomyelia in the second trimester

Iruretagoyena, J. Igor; Trampe, Barbara; Shah, Dinesh

doi:10.3109/14767050903061769

Cited by 18 publications

(11 citation statements)

References 2 publications

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“…The cerebel-lar hemisphere and the vermis, which form the roof of the fourth ventricle, grow rapidly (Kapur et al 2009), and during the second trimester the tentorium of the cerebellum is being shaped. Some abnormalities may arise during this process (Iruretagoyena et al 2010), such as COACH syndrome and Dandy-Walker syndrome (Alexiou et al 2010).…”

Section: Introductionmentioning

confidence: 99%

“…Ultrasound examination is the main technique used to evaluate the fetal brain, having the advantages of reliability, low cost, portability and real-time visualization. Some abnormalities in the posterior cranial fossa, including cerebellar tonsillar hernia (Iruretagoyena et al 2010), can be identified using ultrasound from the second trimester. Rutten et al (2009) measured the volume of the fetal cerebellum with 3D-ultrasound from 20 weeks of gestational age (GA) to determine the asymmetry between the left and right sides.…”

Section: Introductionmentioning

confidence: 99%

“…2009), and during the second trimester the tentorium of the cerebellum is being shaped. Some abnormalities may arise during this process (Iruretagoyena et al. 2010), such as COACH syndrome and Dandy–Walker syndrome (Alexiou et al.…”

Section: Introductionmentioning

confidence: 99%

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Development of the human fetal cerebellum in the second trimester: a post mortem magnetic resonance imaging evaluation

Liu¹,

Zhang²,

Lin³

et al. 2011

Journal of Anatomy

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The cerebellum is one of the most important structures in the posterior cranial fossa, but the characterization of its development by magnetic resonance imaging (MRI) is incomplete. We scanned 40 fetuses that had no morphological brain disorder at 14-22 weeks of gestation using 7.0 T MRI. Amira 4.1 software was used to determine morphological parameters of the fetal cerebellum, which included the cerebellar volume (CV), transverse cerebellar diameter (TCD), and the length and width of the vermis. The relationship between these measurements and gestational age (GA) was analysed. We found that the primary fissure was visible at week 14 of gestation. From week 16, the prepyramidal fissure, the secondary fissure and the dentate nucleus could be identified. The posterolateral fissure and the fourth ventricle were recognized at week 17, whereas the tentorium of the cerebellum was visible at week 20. The relationships between GA and CV, TCD, and the width and length of the vermis were described adequately by second-order polynomial regression curves. The ratios between TCD and vermis length and between TCD and vermis width decreased with GA. These results show that 7.0 T MRI can show the trajectory of cerebellar development clearly. They increase our understanding of normal cerebellar development in the fetus, and will facilitate the diagnosis of pathological intrauterine changes in the cerebellum.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Development of the human fetal cerebellum in the second trimester: a post mortem magnetic resonance imaging evaluation

Liu¹,

Zhang²,

Lin³

et al. 2011

Journal of Anatomy

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“…The central nervous system abnormalities are one of the most common ones detected. Chiari malformation is one among the CNS abnormalities diagnosed by prenatal sonography [6]. The obvious sonographic findings are the frontal bone scalloping (lemon sign) and absent cerebellum or abnormal anterior curvature of the cerebellar hemispheres (banana sign) [7].…”

Section: Discussionmentioning

confidence: 99%

Prenatal sonographic evaluation of Arnold Chiari II Malformation

T¹,

B²,

K³

et al. 2017

Int J Med Res Rev

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Arnold Chiari malformations are a broad group of malformations with distinctive imaging findings. They are named after an Austrian pathologist Hans Chiari who first identified types I-III in 1891. It is a spectrum of congenital abnormalities of CNS, characterized by downward displacement of the parts of the cerebellum, fourth ventricle, pons and medulla oblongata into the spinal canal. Type II is the most common subtype and is invariably associated with open neural tube defects like myelomeningocele. They constitute an important cause of neonatal morbidity and mortality and hence the need for prenatal evaluation with ultrasound to detect the anomalies. In this article we describe the features of Arnold chiari malformation, illustrate type II cases with sonological parameters for assessment, discuss the findings for diagnosis and conclude with prognosis and management.

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“…[4,5,13-15], there is only a scant literature on the pathology or mechanisms of syrinx formation in the human fetus. This is perhaps surprising, as large post-mortem series on the subject mention syrinx formation as not infrequent in fetal, neonatal and paediatric Chiari II malformations [14,15] and there is an ultrasound report of detection of syringomyelia in a pre-term fetus [16], although a large radiological series demonstrates detection by MRI be very unusual and late in gestation [17]. …”

Section: Introductionmentioning

confidence: 99%

Fetal syringomyelia

Guo

Chitayat

Blaser

et al. 2014

acta neuropathol commun

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We explored the prevalence of syringomyelia in a series of 113 cases of fetal dysraphism and hindbrain crowding, of gestational age ranging from 17.5 to 34 weeks with the vast majority less than 26 weeks gestational age. We found syringomyelia in 13 cases of Chiari II malformations, 5 cases of Omphalocele/Exostrophy/Imperforate anus/Spinal abnormality (OEIS), 2 cases of Meckel Gruber syndrome and in a single pair of pyopagus conjoined twins. Secondary injury was not uncommon, with vernicomyelia in Chiari malformations, infarct like histology, or old hemorrhage in 8 cases of syringomyelia. Vernicomyelia did not occur in the absence of syrinx formation. The syringes extended from the sites of dysraphism, in ascending or descending patterns. The syringes were usually in a major proportion anatomically distinct from a dilated or denuded central canal and tended to be dorsal and paramedian or median. We suggest that fetal syringomyelia in Chiari II malformation and other dysraphic states is often established prior to midgestation, has contributions from the primary malformation as well as from secondary in utero injury and is anatomically and pathophysiologically distinct from post natal syringomyelia secondary to hindbrain crowding.

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Prenatal diagnosis of Chiari malformation with syringomyelia in the second trimester

Cited by 18 publications

References 2 publications

Development of the human fetal cerebellum in the second trimester: a post mortem magnetic resonance imaging evaluation

Development of the human fetal cerebellum in the second trimester: a post mortem magnetic resonance imaging evaluation

Prenatal sonographic evaluation of Arnold Chiari II Malformation

Fetal syringomyelia

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