Sanjeeb Kumar Sarma (Down Town Hospital / Guwahati / India), BJ Sarmah (Down Town Hospital / Guwahati / India), Prakritish Bora (International Hospital / Guwahati / India)  

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Ein Neugeborenes zeigte bei der Untersuchung eine kleine Schwellung im unteren zentralen Rueckenbereich. Ultraschall-Screening des Gehrins zeigte Hydrozephalus mit einer kleinen hinteren Schaedelgrube.  

Dieser Beitrag wurde original in Englisch geschrieben. Dieser Teil wurde noch nicht ins Deutsche uebersetzt.


In 1891, the German pathologist Hans Chiari described congenital hindbrain anomalies in which cerebellar tissue descends into cervical canal, now designated commonly as Chiari type I. According to the name of his professor Dr. Arnold and his name, this hind brain disorder is named as Arnold Chiari malformation, which is divided into four types depending upon its association with other anomalies. Aetiology is unknown so far.
Chiari I malformation, also known as congenital tonsillar ectopia, is a relatively simple anomaly and infrequently associated with other anomalies. In contrast to Chiari II malformation, in this disorder the vermis, 4th ventricle and medulla are normal or minimally deformed. Elongated, “peglike” cerebellar tonsils are displaced inferiorly through the foramen magnum into the upper cervical spinal canal. In general it is not associated with any brain lesion. Mild to moderate hydrocephalus is seen in 20-25% cases. Syringomyelia is seen in 30-60% of all cases. Skeletal anomalies like basilar invagination (25-50%), Klippel-Feil (5-10%) and atlantooccipital assimilation (1-5%) are seen.
Chiari II malformation is a complex anomaly with skull, dura, brain, spine and cord manifestations. Skull and dura abnormalities include calvarial defects (lacunar skull), small posterior fossa with low lying transverse sinuses, fenestrated falx, heart shaped incisura with hypoplastic tentorium, gaping foramen magnum, concave clivus/petrous ridges. Brain anomalies include inferiorly displaced vermis, medullary spur and kink, beaked tectum, interedigitated gyri, cerebellum “creeps” through the brainstem and “towers” through wide tentorial incisura, corpus callosal agenesis, heterotopias, polymicrogyria, stenogyria etc. Hydrocephalus is seen in 90% cases. The 4th ventricle is elongated, tube like, and inferiorly displaced. Large massa intermedia, colpocephaly, scalloped pointed walls. Myelomeningocele is seen in all cases. Syringohydromyelia is seen in 50-90% cases. Diastematomyelia and segmentation anomalies are seen in <10% cases.
Chiari III malformation is a herniation of hind brain into a low occipital and high cervical region in combination with Chiari II malformations. Cerebellum, occipital cortex are common contents of encephalocele. Occasionally medulla and pons are also herniated. The herniated tissue is strikingly abnormal and often non-functioning because of necrosis, gliosis, fibrosis and the presence of heterotopias. On imaging it is particularly important to recognize position of brain stem, medulla and potential anomalies of venous drainage associated with encephalocele. Herniation of 4th and lateral ventricles is also seen.
Chiari IV is severe cerebellar hypoplasia. The main abnormality includes absent or severely hypoplastic cerebellum, small brain stem, and large posterior fossa CSF spaces. Interestingly there is no hydrocephalus in this anomaly. Other CNS anomalies are also usually absent.
Imaging is the mainstay of diagnosis in this malformation.  

<- MRT 1 betrachten

MRT 1: Axial T1 gewichtetes MRT zeigt dilatierte Hinterhoerner der Seitenventrikel (Kolpozephaly). Milde interdigitationen der parafalzinen gyri.





<- MRT 2 betrachten

MRT 2: Axial T2 gewichtetes MRT zeigt die gleichen Befunde wie in MRT 1.





<- MRT 3 betrachten

MRT 3: Axial T2 gewichtetes MRT zeigt symmetrisch dilatierte Ventrikel.





<- MRT 4 betrachten

MRT 4: Sagittal T1 gewichtetes MRT zeigt grosse Massa intermedia. Dilatierte Seitenventrikel.





<- MRT 5 betrachten

MRT 5: Sagittal T1 gewichtetes MRT zeigt grosse Massa intermedia, Kleinhirn-Herniation, Rohr-artige vierte Ventrikel und schnabelfoermiges Tectum. Tiefligender Sinus transversus und Syrinx im Rueckenmark.





<- MRT 6 betrachten

MRT 6: Sagittal T1 gewichtetes MRT zeigt showing grosse Massa intermedia, Kleinhirn-Herniation, Rohr-artige vierte Ventrikel und schnabelfoermiges Tectum, medullaerer Knick und Sporn, Syrinx und Myelomeningozele in der sakralen Region.





<- MRT 7 betrachten

MRT 7: Sagittal T2 gewichtetes MRT zeigt showing Herniation mit kleiner hinteren Schaedelgrube. Kaudale Myelomeningozele in der sacralen Region.





<- MRT 8 betrachten

MRT 8:Sagittal T2 gewichtetes MRT zeigt gleiche Befunde wie MRT 6..





<- MRT 9 betrachten

MRT 9: Axial T2 gewichtetes MRT. Sektion durch die untere Thorax-Region zeigt eine Syrinx.



 

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Den Ausgang dieser Kondition wissend verliesen die Eltern das Krankenhaus mit dem Neugeborenen und erschienen nicht mehr wieder zur Folgeuntersuchung.  

1891 beschrieb der Deutsche Pathologe Hans Chiari eine kongenitale Rautenhirn-Anomalie in welcher die Kleinhirn-Tonsillen in den Zervikalkanal deszendieren - heutzutage als Chiari Typ I bezeichnet.  

1. Anne G. Osborn. Diagnostic Neuroradiology by (Mosby)- Chapter 2. Disorders of neural tube closure.1995,P 15-24.

2. Stevenson KL (2004) Chiari Type II malformation: past, present, and future. Neurosurg Focus 16(2):E5.  

Arnold chiari, meningomyelozele, Kleinhirn herniation, syringomyelia, Kind, Kindheit, Pädiatrie, Kinderheilkunde  

Sanjeeb Kumar, BJ Sarmah, Prakritish Bora. Arnold Chiari Malformation Typ II. PedRad [serial online] vol 9, no. 10.
URL: www.Kinderradiologie-online.de/?search=20091018134917