675:
53:
92:
1548:, and instead it is mostly the cisterna magna that is enlarged (sometimes diagnosed as "mega cisterna magna"). In 1999, Calabró et al. first used the phrase Dandy–Walker continuum when referring to proposals that a condition known as Blake's pouch cyst falls under the umbrella of the Dandy–Walker complex proposed by Barkovich. These additional terms are mostly discouraged by modern authors due to additional confusion and complexity to the diagnosis of DWM.
441:. Individuals with these features tend to have developmental delay or seizures. Those without any other central nervous system abnormalities tend to have normal or close-to-normal intellectual development. A 2003 review found that moderate-to-severe intellectual disability and non-DWM brain abnormalities were only present in those with the most severe cerebellar vermis malformations (less than two
938:
3476:
341:(ETV) is a less invasive option for patients older than 1 year. Posterior fossa shunts are most effective (80% of the time) but carry the highest risk of complications, while ETV is least effective but has the least risk of complications. The mortality rate is roughly 15%, mostly due to complications from hydrocephalus or its treatment, which can include
1038:(narrowing of the passage between the third and fourth ventricles) does not seem to be a factor in DWM. It is usually open, and shunts placed in the posterior fossa cyst almost always drain all above ventricles. When it is present, it may be the result of compression from a herniated vermis or cyst or an associated developmental abnormality.
1090:(the space behind the cerebellum), though the specific degree of hypoplasia or cystic enlargement for diagnosis of DWM is not agreed upon. Additionally, there are several similar conditions which have at various times been grouped with DWM on a continuum by some authors and separated as distinct by others, further complicating diagnosis.
701:. A large number of genetic conditions can result in the anomaly. In a large portion of DWM cases, the condition is identified in the person affected, however in most cases the cause is not identified. At least 21% of those with DWM have a sibling with the malformation, and at least 16% have a parent with the malformation.
1543:
once. In 1976, Harwood-Nash and Fitz proposed the term Dandy–Walker variant (DWV) for a malformation in which the posterior fossa is not enlarged but the cerebellar vermis is hypoplastic. In 1989, Barkovich et al. proposed the term Dandy–Walker complex (DWC) to include classic DWM and DWV (under type
1455:
Mortality rates from DWM are roughly 15%. In a study of Dandy–Walker variant (DWV), a mortality rate of 12.5% was observed. The most common cause of death is complications from hydrocephalus or its treatment. Untreated hydrocephalus can lead to increased intracranial pressure and brain damage. Shunts
1113:
beneath it, and instead it is mostly the cisterna magna that is enlarged. In this type, the hypoplasia of the cerebellar vermis does not reach past the horizontal midline of the fourth ventricle, and the posterior fossa is also not as large. The authors noted that this form would previously have been
1464:
are also possible and can lead to mortality. Shunts in the fourth ventricle (cystoperitoneal shunts, or CP shunts) have a generally high rate of successful cyst and ventricle size reduction, especially in the cyst (at least 80%). With a shunt in the lateral ventricles (ventriculoperitoneal shunt, or
1393:
by the malformation. However, a CP shunt almost always drains both the fourth and lateral ventricles in DWM, and according to strict definitions of the malformation, the aqueduct should be assumed open, though imaging is important to confirm this. Many authors therefore recommend the CP shunt as the
1228:
may also be used if MRI is unavailable, but it provides less detail. Klein et al. (2003) suggested that a suspected diagnosis based on CT or ultrasound should not be confirmed until an MRI is performed, due to the large number of conditions that can present highly similarly and confound diagnosis.
411:
Despite the hypoplastic cerebellar vermis, just over half of individuals with DWM (between 27% and 84%) do not appear to have significant intellectual disability or developmental delay. However, many of the genetic conditions associated with DWM can present with developmental delay and other brain
1181:
Due to the inconsistency of the presence of hydrocephalus in DWM, Spennato and Klein suggested that it should not be considered a criterion for DWM. Klein's criteria differed from
Spennato's mainly in that it required no apparent cerebellar hemisphere hypoplasia, but it may also have required the
1425:
is not a contraindication, however. ETV has a more modest success rate than shunts, as the hole often closes over. It is more likely to fail in younger patients (below one year), and its effects on the developing brain are not yet known. Cysts posterior to the cerebellum, presenting in children
1369:
are the mainstay of treatment. However, those with DWM have a higher rate of shunt-related complications than other patients with hydrocephalus (mainly due to the unconventional anatomy). One explanation for a failure of a shunt to reduce intracranial pressure in DWM has been that the cyst may
1122:
when referring to proposals that a condition known as Blake's pouch cyst falls under the umbrella of the Dandy–Walker complex proposed by
Barkovich. Later authors would put these terms and systems under intense scrutiny and state that they added considerable confusion to the diagnosis of DWM.
390:
in the postnatal period. Hydrocephalus occurs in an estimated 80% of patients with classic DWM. This usually presents within the first year of life (85% of the time), most often within the first three months. Signs of hydrocephalus in infants include increasing head size, vomiting, excessive
349:
The prevalence of DWM is estimated at between 1 in 25,000 to 1 in 50,000. DWM is the cause of around 4.3% of cases of congenital hydrocephalus and 2.5% of all cases of hydrocephalus. At least 21% of those with DWM have a sibling with the malformation, and at least 16% have a parent with the
2103:
Sasaki-Adams, Deanna; Elbabaa, Samer K.; Jewells, Valerie; Carter, Lori; Campbell, Jeffrey W.; Ritter, Ann M. (September 2008). "The Dandy-Walker variant: a case series of 24 pediatric patients and evaluation of associated anomalies, incidence of hydrocephalus, and developmental outcomes".
981:
on the forward surface of the fourth ventricle, which expand and roll over to fuse in the midline to form the cerebellar vermis by the 15th week. If this process does not complete, the cerebellar vermis will not form fully. This long period of development of the cerebellar vermis makes it
1406:. Spennato et al. therefore recommend a flow-regulating or anti-syphon valve. On the other hand, VP shunts have a lower rate of complications than CP shunts and are recommended initially by some. However, they are less effective in DWM, and the elevated position of the
1433:
of the posterior fossa and excision of the cystic membrane was used, which was often unsuccessful in preventing cyst reformation and carried a degree of mortality. This may still be reserved for patients with repeated shunt failures/infections.
1442:
Treatments for any other symptoms are generally focussed on the specific condition involved and may include supported education, physical therapy or other services. Genetic counselling may be offered to parents for future conceptions.
1012:(CSF) to escape into the subarachnoid space of the posterior fossa. However, later studies found that these foramina are usually open in DWM. Hydrocephalus is also usually (80% of the time) not present at birth in those with DWM.
345:
or infection. The prognosis after successful hydrocephalus treatment is usually good but depends on any associated condition and its symptoms. Those without hydrocephalus are treated based on any associated symptoms or condition.
1451:
The prognosis is first and foremost dependent on the early and successful treatment of hydrocephalus, if present. The other significant factor affecting prognosis is the presence of a comorbid genetic condition or brain anomaly.
3661:
2040:
Spennato, Pietro; Mirone, Giuseppe; Nastro, Anna; Buonocore, Maria
Consiglio; Ruggiero, Claudio; Trischitta, Vincenzo; Aliberti, Ferdinando; Cinalli, Giuseppe (October 2011). "Hydrocephalus in Dandy-Walker malformation".
1479:
A 2017 review found that most patients (65%) were diagnosed with either "Dandy–Walker malformation" or "Dandy–Walker syndrome", while 20% were diagnosed with "Dandy–Walker variant" and 1.1% with "mega cisterna magna".
3561:
3546:
1512:
described the malformation as partial or complete absence of the cerebellar vermis, an enlarged fourth ventricle and hydrocephalus. In 1942, American physician John K. Taggart and
Canadian–American neurosurgeon
1077:
The precise diagnostic criteria and classification systems of DWM are not agreed upon, and significant dispute exists as to which terms or criteria should be used. The core criteria of DWM are hypoplasia of the
1031:
and circulation. Excisions of the cyst in DWM have not been able to show whether impaired arachnoid absorption is involved, since the subarachnoid space always takes days to weeks to fill up following excision.
416:
has been found in between 5% and 17% of those with DWM. This does not seem to result in intellectual disability on its own, however. Other brain abnormalities known to be sometimes associated with DWM include
723:, a condition involving neurological and sometimes eye and kidney abnormalities. Anywhere from 21% to 81% of those with PHACE syndrome have DWM. Other comorbid genetic conditions that were found included
1624:
Barkovich, A. J.; Kjos, B. O.; Norman, D.; Edwards, M. S. (December 1989). "Revised classification of posterior fossa cysts and cystlike malformations based on the results of multiplanar MR imaging".
1421:
to allow CSF to bypass any obstruction. It cannot be used on those with brain abnormalities such as agenesis of the corpus callosum, due to the risk of CSF escaping to other brain areas. A compressed
1245:
Blake's pouch cyst (BPC), or persistent Blake's pouch, is a condition that arises when Blake's pouch, an invagination in the fourth ventricle that ruptures at around 4 months of gestation to form the
3654:
1202:
scan is the most useful method for diagnosis. MRI can delineate the shape and extent of the malformation as well as assessing additional areas for malformations such as the cerebellar hemispheres,
3480:
1456:
used to treat DWM have a moderate-to-good success rate, but they have a higher-than-average failure rate, which can result in failure to reduce the intracranial pressure or infection, such as
310:
genetic condition, though the causative condition is only identified in around half of those diagnosed before birth and a third of those diagnosed after birth. The mechanism involves impaired
647:
occasionally occurs with DWM, though it is not certain how often. One review reported an occurrence of 4.3% in a sample. This may be due to herniation of the bottom of the cyst through the
3647:
882:(trisomy 18), at roughly 26% of prenatal DWM cases. 6.5% of those diagnosed with DWM after birth also have Edwards syndrome. Other chromosomal abnormalities that can lead to DWM include
1222:
of the heart. In true DWM, this will find a flow from the cerebral aqueduct to the posterior fossa and no flow between the cisterna magna and the space behind the cervical spinal cord.
1109:) to include classic DWM and DWV (under type A) plus a third malformation (under type B) in which the cerebellar vermis remains large enough to sit between the fourth ventricle and the
1041:
It is known that once hydrocephalus has started, the compression by the posterior fossa cyst against the venous passages in the arachnoid mater is involved in the worsening pathology.
1476:
The prevalence of DWM is estimated at between 1 in 25,000 to 1 in 50,000. DWM is the cause of around 4.3% of cases of congenital hydrocephalus and 2.5% of all cases of hydrocephalus.
2880:
Hammond, Christopher J.; Chitnavis, Bhupal; Penny, Christopher C.; Strong, Anthony J. (2002-01-01). "Dandy-Walker
Complex and Syringomyelia in an Adult: Case Report and Discussion".
2829:
Forzano, F.; Mansour, S.; Ierullo, A.; Homfray, T.; Thilaganathan, B. (2007). "Posterior fossa malformation in fetuses: a report of 56 further cases and a review of the literature".
800:
641:
may occur in DWM. This has generally been found at rates of 6–8%. It has been suggested to occur to compensate for the increased pressure in the posterior fossa during foetal life.
318:
affecting the long period of development of the cerebellar vermis. The mechanism by which hydrocephalus occurs in DWM is not yet fully understood. The condition is diagnosed by
3504:
2294:
Kollias, S. S.; Ball, W. S.; Prenger, E. C. (November 1993). "Cystic malformations of the posterior fossa: differential diagnosis clarified through embryologic analysis".
1374:
and form a scarring adhesion at the cervical junction, preventing it from shrinking again. If this occurs, a suboccipital decompression with duraplasty may be attempted.
693:
DWM is caused by any disruption to embryonic development that affects the formation of the cerebellar vermis. This is usually a genetic mutation that results in impaired
2743:
Pandurangi, Swapna; Pandurangi, Aditya; Matkar, Abhay; Shetty, Nithin; Patil, Preetam (January 2014). "Psychiatric
Manifestations Associated With Mega Cisterna Magna".
1465:
VP shunt), studies have generally found a roughly 50% successful cyst size reduction rate, with successful ventricle size reduction roughly two thirds of the time.
1426:
younger than five years, have been labeled developmental retrocerebellar cysts under a new classification in relation to the proposed neuroendoscopic management.
3491:
3173:
Asai, Akio; Hoffman, Harold J.; Hendrick, Bruce; Humphreys, Robin P. (1989). "Dandy-Walker
Syndrome: Experience at the Hospital for Sick Children, Toronto".
326:. There are other malformations that can strongly resemble DWM, and disagreement exists around the criteria and classifications used for the malformation.
550:. Slightly more of these were found in Dandy–Walker variant (DWV) than in classic DWM, despite DWV being less common, at only around 20% of DWS diagnoses.
3122:
Pascual-Castroviejo, I.; Velez, A.; Pascual-Pascual, S. I.; Roche, M. C.; Villarejo, F. (1991-04-01). "Dandy-Walker malformation: analysis of 38 cases".
1101:) for a malformation in which the posterior fossa is not enlarged but the cerebellar vermis is hypoplastic. In 1989, Barkovich et al. proposed the term
3517:
3340:
Kaplan, Lawrence C. (1985). "Congenital Dandy Walker malformation associated with first trimester warfarin: A case report and literature review".
2381:
Klein, O.; Pierre-Kahn, A.; Boddaert, N.; Parisot, D.; Brunelle, F. (August 2003). "Dandy-Walker malformation: prenatal diagnosis and prognosis".
448:
In Dandy–Walker variant (DWV) and mega cisterna magna specifically, which are less severe malformations, there appears to be an increased rate of
3950:
3935:
1529:
355:
1000:
The reason why hydrocephalus occurs in DWM is not yet fully understood. The earliest authors had put it down it to blockage or narrowing of the
2692:
Kumar, Raj; Jain, Manoj; Chhabra, Devendra (2001-05-01). "Dandy-Walker syndrome: different modalities of treatment and outcome in 42 cases".
1544:
A) plus a third malformation (under type B) in which the cerebellar vermis remains large enough to sit between the fourth ventricle and the
1289:
below the fourth ventricle, is enlarged. It has been proposed to be due to a delayed rupture of Blake's pouch rather than a failed rupture.
2520:"The Lateral Recesses of the Fourth Ventricle; Their Relation to Certain Cysts and Tumours of the Cerebellum, and to Occipital Meningocele"
1394:
logical option. However, it is associated with a high rate of complications, including shifting and overdrainage. Overdrainage can lead to
1357:
and brain damage. A minority of those affected do not develop hydrocephalus and are treated based on any associated symptoms or condition.
1353:
The main immediate goal of treatment is the control of hydrocephalus and the enlarged posterior fossa cyst, as these can lead to increased
1126:
In 2011, Spennato et al. came up with a set of criteria based on Klein et al. (2003) that they considered necessary for diagnosis of DWM:
3945:
1732:
1249:(medial aperture), fails to rupture. This can lead to a dilated fourth ventricle and subsequent hydrocephalus of all four ventricles.
1211:
17:
715:
A genetic condition is identified in around 33% of those diagnosed with DWM after birth. In a 2017 review, 4.3% were found to have
538:
16% of patients were diagnosed with a mental or behavioural disorder, with 6.4% also having a learning disability. 5.3% had either
476:
A 2017 review found the following associations in patients with DWS (usually from an associated genetic condition or abnormality):
1830:
3846:
2234:
Bosemani, Thangamadhan; Orman, Gunes; Boltshauser, Eugen; Tekes, Aylin; Huisman, Thierry A. G. M.; Poretti, Andrea (2015-01-01).
1148:
The rest of the cerebellar vermis is hypoplastic and is pushed upwards and rotated forwards due to the enlarged posterior fossa.
2464:
Lumenta, Christianto B.; Skotarczak, Ulrich (1995-03-01). "Long-term follow-up in 233 patients with congenital hydrocephalus".
834:
772:
1829:
Stambolliu, Emelina; Ioakeim-Ioannidou, Myrsini; Kontokostas, Kimonas; Dakoutrou, Maria; Kousoulis, Antonis A. (2017-09-01).
2166:
Azab, Waleed A.; Shohoud, Sherien A.; Elmansoury, Tamer M.; Salaheddin, Waleed; Nasim, Khurram; Parwez, Aslam (2014-07-24).
1049:
Dandy–Walker malformation is diagnosed based on the characteristic neuroimaging findings. It can be diagnosed prenatally on
445:/three lobules in the vermis), and these comprised 16% of their sample. Hydrocephalus also affected all of these patients.
299:
or underdeveloped genitalia or kidneys. It is sometimes discovered in adolescents or adults due to mental health problems.
1468:
Other systemic or genetic conditions are often present with DWM, and each have their own significant effect on prognosis.
3576:
1676:
Calabrò, F.; Arcuri, T.; Jinkins, J. R. (2000-04-01). "Blake's pouch cyst: an entity within the Dandy-Walker continuum".
275:
within the first year of life, which can present as increasing head size, vomiting, excessive sleepiness, irritability,
3297:
Badano JL, Mitsuma N, Beales PL, Katsanis N (2006). "The ciliopathies: an emerging class of human genetic disorders".
1417:(ETV) may be considered as the first-line treatment. This less invasive procedure creates an artificial hole in the
1306:
There is debate as to whether this malformation is distinct from DWM or forms part of the "Dandy–Walker continuum".
1414:
1162:) is large, giving a flattened appearance to the bottom of the vermis, or the fastigial nucleus is absent entirely.
1015:
The impairment to CSF flow may lie beyond the outlets of the fourth ventricle. Theories of abnormal development or
655:). Alternatively, it may be a result of hydrocephalus, in which it forms as a "fifth ventricle" due to an enlarged
338:
205:
553:
Around 12% of patients had cancers or tumours arising from congenital genetic abnormalities. The most common were
3798:
1225:
922:
626:
5.9% of patients had underdeveloped reproductive organs, such as hypoplastic genitalia or undescended testicles (
413:
292:
119:
3385:"Developmental Retrocerebellar Cysts: A New Classification for Neuroendoscopic Management and Systematic Review"
1273:
Some authors, however, consider Blake's pouch cyst part of a continuum with DWM (the "Dandy–Walker continuum").
776:
724:
3639:
3009:
Poetke, M.; Frommeld, T.; Berlien, H. P. (December 2002). "PHACE syndrome: new views on diagnostic criteria".
2931:
Golden, Jeffrey A.; Rorke, Lucy B.; Bruce, Derek A. (1987). "Dandy-Walker
Syndrome and Associated Anomalies".
2602:
Taggart, John K.; Walker, A. Earl (1942-10-01). "Congenital
Atresia of the Foramens of Luschka and Magendie".
760:
748:
728:
449:
1237:
DWM has a large number of conditions that can present highly similarly on imaging and confound diagnosis.
1061:, but 28% of the time it is discovered in adolescence or adulthood due to mental health problems, such as
3821:
768:
570:
543:
2646:
Bindal, Ajay K.; Storrs, Bruce B.; McLone, David G. (1990). "Management of the Dandy-Walker
Syndrome".
788:
752:
736:
1831:"The Most Common Comorbidities in Dandy-Walker Syndrome Patients: A Systematic Review of Case Reports"
3755:
1195:
1057:. It is diagnosed within the first year of life 41% of the time, normally due to increasing signs of
871:
792:
756:
732:
493:
442:
399:. In contrast to classic DWM, only around 30% of those with Dandy–Walker variant (DWV), in which the
2786:
Bindal, A. K.; Storrs, B. B.; McLone, D. G. (1990–1991). "Management of the Dandy-Walker syndrome".
2437:
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489:
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288:
115:
91:
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1497:
1138:
1087:
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914:
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577:. The melanocytic tumours in these cases are thought to relate to the same genetic errors in the
481:
418:
400:
264:
127:
1331:
The cyst is clearly localised in a specific location separate from the fourth ventricle outlets.
1256:
The cerebellum is not hypoplastic, though it may be compressed by the enlarged posterior fossa (
1534:
1385:(a ventriculoperitoneal shunt, or VP shunt) or both, due to conflicting studies on whether the
1340:
Hydrocephalus, if it occurs, is due to the cyst pressing on the cerebellum and compressing the
1257:
1152:
974:
961:
begins forming at the fifth week of embryonic development. It differentiates at the top of the
611:
9% of patients (almost all with classic DWM) had musculoskeletal abnormalities, which included
360:
307:
276:
184:
156:
1517:
detailed the phenomenon extensively, ascribing the potential cause as underdevelopment of the
291:, eye abnormalities, intellectual disability, congenital tumours, other brain defects such as
3803:
2974:
Metry DW, Dowd CF, Barkovich AJ, Frieden IJ (July 2001). "The many faces of PHACE syndrome".
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The cerebellum is not hypoplastic, though it may be compressed by the cyst (mass effect).
1246:
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1035:
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has also been described in a handful of cases. Many of these disorders are classified as
652:
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2078:
1873:
1733:"Hydrocephalus Fact Sheet | National Institute of Neurological Disorders and Stroke"
1713:
985:
In DWM, the fourth ventricle opens up into and is continuous with almost the entire
719:, a condition involving brain, cardiovascular and eye abnormalities, while 2.3% had
3905:
3463:
3443:
3396:
3349:
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1378:
1158:
The angle at the centre of the cerebellar vermis (representing the location of the
1083:
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as early as 14 weeks of gestation, although it is usually diagnosed postnatally by
1005:
970:
946:
879:
784:
720:
678:
566:
539:
457:
260:
105:
Hydrocephalus: increasing head size, vomiting, excessive sleepiness, irritability,
85:
3789:
3592:
1522:
1418:
1321:
1207:
1020:
1001:
780:
666:
has been found with DWM. When it is present, it is usually spina bifida occulta.
392:
2562:"AN EXPERIMENTAL, CLINICAL AND PATHOLOGICAL STUDY: Part 1.—Experimental Studies"
941:
Diagram of the cerebellum, fourth ventricle and pons. The white arrow shows the
823:. DWM is one of the single largest predictors of a ciliopathic genetic disease.
333:
in infancy. This is usually inserted in the posterior fossa, but a shunt in the
3841:
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3808:
3674:
3669:
3431:
3400:
1828:
1545:
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is absent to varying degrees (three quarters, one half or one quarter missing).
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627:
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512:
438:
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46:
Dandy–Walker syndrome (DWS), Dandy–Walker complex (DWC), Dandy–Walker continuum
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2125:
2062:
1857:
1849:
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1337:
The CSF flow in the cyst is not continuous with that of the fourth ventricle.
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1016:
962:
926:
816:
808:
698:
656:
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638:
516:
501:
453:
426:
387:
315:
296:
272:
247:), is a rare congenital brain malformation in which the part joining the two
3353:
3121:
2987:
2535:
1572:"The Dandy-Walker Syndrome or The So-Called Atresia of the Foramen Magendie"
3884:
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1605:
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The term Dandy–Walker syndrome (DWS) was introduced by German psychiatrist
1505:
1023:
in the posterior fossa have been put forward. The arachnoid mater contains
978:
918:
812:
804:
663:
586:
366:
3369:
3202:
3151:
2960:
2815:
2705:
2675:
2493:
2323:
1689:
1653:
386:
The most frequent and prominent symptoms of DWM are those associated with
3862:
3699:
3682:
3258:
3246:
American Journal of Medical Genetics Part C: Seminars in Medical Genetics
3241:
3022:
2252:
2235:
744:
590:
582:
578:
558:
520:
303:
152:
60:
3432:"Surgical treatment of 13 pediatric patients with Dandy–Walker syndrome"
3784:
3135:
2477:
1457:
1430:
958:
710:
682:
422:
252:
3538:
3447:
3186:
2944:
2799:
2659:
1320:
An arachnoid cyst is a collection of cerebrospinal fluid (CSF) in the
350:
malformation. The malformation was first described by English surgeon
3712:
3622:
1422:
1183:
1062:
894:
and partial 3q deletion or duplication. The 3q24 region contains the
891:
883:
612:
574:
508:
465:
283:. Other, less common symptoms are generally associated with comorbid
213:
3216:
2842:
2561:
1155:
are pushed forwards and to the side by the enlarged posterior fossa.
507:
24% of patients had at least one ocular abnormality. These included
391:
sleepiness, irritability, downward deviation of the eyes (known as "
1219:
910:
532:
396:
280:
110:
969:(in the brainstem) differentiates at the bottom, separated by the
189:
Blake's pouch cyst (BPC), mega cisterna magna(?), posterior fossa
3707:
1377:
In DWM, it is not agreed whether a shunt should be placed in the
1215:
1173:, is elevated due to the enlarged posterior fossa. (The adjacent
801:
Craniosynostosis-Dandy-Walker malformation-hydrocephalus syndrome
177:
3581:
2742:
2102:
1488:
The malformation was first described in 1887 by English surgeon
3550:
2380:
2165:
528:
527:(microcornea) or corneal opacity (leukoma), short-sightedness (
524:
2233:
2039:
1266:
The cerebellar tentorium/confluence of sinuses is not raised.
1169:, part of the drainage system located at the far rear of the
852:
846:
840:
461:
218:
15% risk of death, mostly from hydrocephalus or its treatment
3172:
2828:
1269:
Hydrocephalus, when it occurs, involves all four ventricles.
1027:
necessary to return CSF from the subarachnoid spaces to the
3296:
3065:
Imataka, George; Yamanouchi, Hideo; Arisaka, Osamu (2007).
2879:
966:
858:
828:
686:
547:
68:
3242:"Making sense of cilia in disease: The human ciliopathies"
1623:
937:
633:
5.3% of patients had underdeveloped or polycystic kidneys.
2973:
2745:
The Journal of Neuropsychiatry and Clinical Neurosciences
1214:
can observe the flow of cerebrospinal fluid (CSF) during
1199:
1054:
319:
169:
64:
3064:
815:
that are believed to be crucial in signalling embryonic
3492:
National Institute of Neurological Disorders and Stroke
2785:
1141:(the space behind the cerebellum) is enlarged, and its
1008:, the two apertures in the fourth ventricle that allow
743:, among many others. DWM has also been associated with
1576:
Journal of Neuropathology & Experimental Neurology
3217:"OMIM Entry - # 200990 - ACROCALLOSAL SYNDROME; ACLS"
3067:"Dandy–Walker syndrome and chromosomal abnormalities"
3008:
2560:
Dandy, Walter E.; Blackfan, Kenneth D. (1914-12-01).
1145:
flow is continuous with that of the fourth ventricle.
585:
that lead to the DWM, since the subsequent embryonic
3528:
1675:
1327:
In a posterior fossa arachnoid cyst, unlike in DWM:
870:
In those who are diagnosed with DWM before birth on
354:
in 1887, though it was named by German psychiatrist
67:
of Dandy–Walker variant (DWV) with dysplasia of the
1410:should be considered before installing a VP shunt.
1299:
The fourth ventricle is of relatively normal shape.
995:
373:, who described it in 1914 and 1942, respectively.
2645:
2293:
329:Treatment for most involves the implantation of a
144:Dandy–Walker variant (DWV), mega cisterna magna(?)
2463:
2236:"Congenital Abnormalities of the Posterior Fossa"
1402:, due to scarring, or downward herniation of the
1309:
1093:In 1976, Harwood-Nash and Fitz proposed the term
826:Other genes that have been linked to DWM include
704:
3927:
3429:
2930:
2691:
2687:
2685:
1281:Mega cisterna magna is a condition in which the
1118:. In 1999, Calabró et al. first used the phrase
27:Congenital malformation of the cerebellar vermis
3382:
2442:NORD (National Organization for Rare Disorders)
1365:For hydrocephalus or the posterior fossa cyst,
1182:vermis to touch the tentorium or an absence of
913:use during pregnancy has been known to lead to
604:disorders, and 2.7% had excessive hair growth (
2343:"Orphanet: Isolated Dandy Walker malformation"
1072:
118:, eye abnormalities, intellectual disability,
3655:
3423:
2967:
2682:
2601:
2559:
1324:. 10% of these occur in the posterior fossa.
1198:as early as 14 weeks of gestation, though an
807:, genetic disorders that affect the cellular
1525:, now no longer believed to be significant.
1381:(a cystoperitoneal shunt, or CP shunt), the
865:
3239:
3662:
3648:
3290:
1460:. Complications from overdrainage such as
1296:The cerebellum is not usually hypoplastic.
90:
51:
3257:
3082:
2251:
2201:
2183:
1587:
1232:
2616:10.1001/archneurpsyc.1942.02290100083008
2566:American Journal of Diseases of Children
1252:In a Blake's pouch cyst, unlike in DWM:
982:particularly vulnerable to disruptions.
936:
902:genes, known to be associated with DWM.
673:
159:genetic conditions, often not identified
132:Shunt failure (shifting, overdrainage),
3847:Bilateral frontoparietal polymicrogyria
3509:in the Journal of Pediatrics, July 2001
3240:Baker, Kate; Beales, Philip L. (2009).
1292:In mega cisterna magna, unlike in DWM:
681:(E) location shown in red, between the
471:
337:may be used instead or in conjunction.
295:, skeletal abnormalities, an occipital
14:
3951:Syndromes affecting the nervous system
3936:Congenital disorders of nervous system
3928:
3487:Dandy-Walker Syndrome Information Page
3339:
3060:
3058:
3056:
2604:Archives of Neurology & Psychiatry
2517:
2513:
2511:
2459:
2457:
2432:
2430:
2428:
1626:AJR. American Journal of Roentgenology
1276:
953:(3). This usually remains open in DWM.
929:. In 1985, it was also linked to DWM.
773:Fukuyama congenital muscular dystrophy
3643:
3520:from Pan Arab Journal of Neurosurgery
3383:Dhandapani, S; Sahoo, SK (Dec 2019).
3311:10.1146/annurev.genom.7.080505.115610
3011:European Journal of Pediatric Surgery
2641:
2639:
2637:
2635:
2633:
2597:
2595:
2555:
2553:
2376:
2374:
2372:
2370:
2368:
2366:
2364:
2362:
2337:
2335:
2333:
2289:
2287:
2285:
2283:
2281:
2279:
2229:
2227:
2225:
2223:
2221:
2161:
2159:
2157:
2155:
2153:
2151:
2098:
2096:
2094:
2092:
2090:
2088:
2035:
2033:
2031:
2029:
2027:
2025:
2023:
2021:
2019:
2017:
2015:
2013:
2011:
2009:
2007:
2005:
2003:
2001:
1999:
1997:
1995:
1993:
1991:
1989:
1987:
1985:
1983:
1981:
1979:
1977:
1975:
1973:
1971:
1969:
1967:
1965:
1963:
1961:
1959:
1957:
1955:
1953:
1951:
1949:
1947:
1945:
1943:
1941:
1939:
1937:
1935:
1933:
1931:
1929:
1927:
1925:
1923:
1921:
1919:
1917:
1915:
1913:
1911:
1909:
1907:
1905:
1903:
1824:
1822:
1820:
1818:
1816:
1814:
1812:
1810:
1808:
1806:
1804:
1802:
1800:
1798:
1796:
1794:
1792:
1790:
1788:
1786:
1784:
1782:
1780:
1778:
1776:
1774:
1772:
1727:
1725:
1723:
1671:
1669:
1667:
1665:
1663:
1569:
1565:
1563:
1561:
1360:
1240:
403:is not enlarged, have hydrocephalus.
376:
365:in 1954 after American neurosurgeons
263:and space behind the cerebellum (the
2578:10.1001/archpedi.1914.02180010416002
1901:
1899:
1897:
1895:
1893:
1891:
1889:
1887:
1885:
1883:
1770:
1768:
1766:
1764:
1762:
1760:
1758:
1756:
1754:
1752:
1619:
1617:
1615:
1263:The posterior fossa is not enlarged.
1123:However, they remain commonly used.
3430:Yüceer N, Mertol T, Arda N (2007).
3053:
2508:
2454:
2425:
2106:Journal of Neurosurgery. Pediatrics
24:
3946:Syndromes affecting the cerebellum
3673:malformations and deformations of
3498:
3233:
2630:
2592:
2550:
2359:
2330:
2308:10.1148/radiographics.13.6.8031352
2276:
2218:
2148:
2085:
1720:
1660:
1558:
1194:DWM can be observed prenatally on
932:
905:
811:, thin cell projections made from
25:
3962:
3524:
3518:Dandy–Walker Malformation article
1880:
1749:
1612:
1504:. In 1914, American neurosurgeon
1413:In patients older than one year,
945:(medial aperture) connecting the
874:, up to half are found to have a
271:. Most of those affected develop
3479: This article incorporates
3474:
3084:10.1111/j.1741-4520.2007.00158.x
2894:10.1097/00006123-200201000-00028
2757:10.1176/appi.neuropsych.13040097
2172:Surgical Neurology International
1570:Benda, Clemens E. (1954-01-01).
1415:endoscopic third ventriculostomy
996:Pathophysiology of hydrocephalus
381:
339:Endoscopic third ventriculostomy
206:endoscopic third ventriculostomy
3799:Agenesis of the corpus callosum
3376:
3333:
3209:
3166:
3115:
3002:
2924:
2873:
2822:
2779:
2736:
2518:Sutton, J. Bland (1886-10-01).
1539:in 1954; he also used the term
1471:
923:agenesis of the corpus callosum
917:, including ocular dysgenesis,
414:Agenesis of the corpus callosum
406:
293:agenesis of the corpus callosum
259:) does not fully form, and the
120:agenesis of the corpus callosum
114:Associated genetic conditions:
1310:Posterior fossa arachnoid cyst
725:oculocerebrocutaneous syndrome
705:Ciliopathic genetic conditions
277:downward deviation of the eyes
204:, ventriculoperitoneal shunt,
122:, skeletal abnormalities, etc.
13:
1:
3513:E-medicine webpage definition
1551:
925:, skeletal abnormalities and
915:systemic defects in the fetus
878:, with the most common being
569:(4.4%). 3.2% of patients had
535:(a hole in an eye structure).
425:(fewer ridges in the brain),
1446:
1348:
1344:or fourth ventricle outlets.
1044:
729:oral-facial-digital syndrome
561:(4.8%, including those with
450:psychotic spectrum disorders
7:
3765:other reduction deformities
2438:"Dandy Walker Malformation"
1508:and American paediatrician
1073:Criteria and classification
571:congenital melanocytic nevi
544:psychotic spectrum disorder
523:dysplasia/atrophy, a small
302:DWM is usually caused by a
10:
3967:
3401:10.1016/j.wneu.2019.08.052
1838:Journal of Child Neurology
1483:
1313:
1302:Hydrocephalus is uncommon.
1189:
789:Cornelia de Lange syndrome
777:Ellis–Van Creveld syndrome
708:
226:1 in 25,000 to 1 in 50,000
3893:
3870:
3861:
3738:
3690:
3681:
3613:
3532:
2395:10.1007/s00381-003-0782-5
2055:10.1007/s00381-011-1544-4
1541:Dandy–Walker malformation
1130:The lower portion of the
866:Chromosomal abnormalities
761:Shah–Waardenburg syndrome
749:Rubinstein–Taybi syndrome
494:ventricular septal defect
233:Dandy–Walker malformation
222:
212:
196:
183:
163:
148:
140:
126:
99:
79:
59:
50:
42:
38:Dandy–Walker malformation
37:
3751:Congenital hydrocephalus
3299:Annu Rev Genom Hum Genet
2185:10.4103/2152-7806.137533
2118:10.3171/PED/2008/2/9/194
1850:10.1177/0883073817712589
1437:
669:
651:(a similar mechanism to
555:neurocutaneous melanosis
490:coarctation of the aorta
486:patent ductus arteriosus
289:congenital heart defects
116:congenital heart defects
3354:10.1002/tera.1420320302
2988:10.1067/mpd.2001.114880
876:chromosomal abnormality
769:Walker–Warburg syndrome
500:. In 2.7% of patients,
482:congenital heart defect
419:grey matter heterotopia
3481:public domain material
3175:Pediatric Neurosurgery
3124:Child's Nervous System
2933:Pediatric Neurosurgery
2788:Pediatric Neurosurgery
2694:Child's Nervous System
2648:Pediatric Neurosurgery
2466:Child's Nervous System
2383:Child's Nervous System
2043:Child's Nervous System
1638:10.2214/ajr.153.6.1289
1233:Differential diagnosis
1153:cerebellar hemispheres
1120:Dandy–Walker continuum
975:cerebellar hemispheres
954:
753:Marden–Walker syndrome
737:Meckel–Gruber syndrome
690:
573:, and 2.1% had tongue
480:27% of patients had a
185:Differential diagnosis
3804:Septo-optic dysplasia
3756:Dandy–Walker syndrome
2706:10.1007/s003810000425
2536:10.1093/brain/9.3.352
1690:10.1007/s002340050888
1492:as hypoplasia of the
1355:intracranial pressure
1167:confluence of sinuses
1114:classified as simply
940:
797:acrocallosal syndrome
793:Klippel–Feil syndrome
757:Sheldon–Hall syndrome
733:Coffin–Siris syndrome
677:
241:Dandy–Walker syndrome
18:Dandy–Walker syndrome
3506:Metry Phaces article
3259:10.1002/ajmg.c.30231
3071:Congenital Anomalies
3023:10.1055/s-2002-36849
2253:10.1148/rg.351140038
2168:"Blake's pouch cyst"
1589:10.1093/jnen/13.1.14
1404:cerebral hemispheres
1400:tethered spinal cord
1287:subarachnoid cistern
1175:cerebellar tentorium
1103:Dandy–Walker complex
1095:Dandy–Walker variant
1002:foramina of Magendie
596:10% of patients had
593:, among other cells.
498:atrial septal defect
472:Associated anomalies
429:(shallower ridges),
267:) are enlarged with
3730:Chiari malformation
1530:Clemens Ernst Benda
1519:foramina of Luschka
1462:subdural haematomas
1396:subdural haematomas
1277:Mega cisterna magna
1247:foramen of Magendie
1143:cerebrospinal fluid
1116:mega–cisterna magna
1036:Aqueductal stenosis
1010:cerebrospinal fluid
943:foramen of Magendie
653:Chiari malformation
519:dysplasia/atrophy,
356:Clemens Ernst Benda
343:subdural haematomas
324:prenatal ultrasound
322:or, less commonly,
269:cerebrospinal fluid
174:prenatal ultrasound
107:vertical gaze palsy
3901:Currarino syndrome
3872:Neural tube defect
3814:Hemimegalencephaly
3780:Microlissencephaly
3692:Neural tube defect
3614:External resources
3136:10.1007/BF00247863
2831:Prenatal Diagnosis
2478:10.1007/BF00570260
1515:Arthur Earl Walker
1383:lateral ventricles
1370:herniate into the
1361:Hydrocephalus/cyst
1241:Blake's pouch cyst
1212:phase-contrast MRI
1177:is also elevated.)
990:subarachnoid space
955:
691:
377:Signs and symptoms
371:Arthur Earl Walker
335:lateral ventricles
285:genetic conditions
134:subdural haematoma
3923:
3922:
3919:
3918:
3857:
3856:
3770:Holoprosencephaly
3637:
3636:
3448:10.1159/000106383
3436:Pediatr Neurosurg
3187:10.1159/000120445
2945:10.1159/000120299
2800:10.1159/000120518
2660:10.1159/000120518
2049:(10): 1665–1681.
1737:www.ninds.nih.gov
1494:cerebellar vermis
1490:John Bland-Sutton
1387:cerebral aqueduct
1342:cerebral aqueduct
1204:cerebral aqueduct
1160:fastigial nucleus
1132:cerebellar vermis
1080:cerebellar vermis
741:Kallmann syndrome
581:of the embryonic
484:. These included
435:holoprosencephaly
352:John Bland-Sutton
257:cerebellar vermis
239:), also known as
230:
229:
165:Diagnostic method
73:cerebellar vermis
32:Medical condition
16:(Redirected from
3958:
3906:Diastomatomyelia
3868:
3867:
3688:
3687:
3664:
3657:
3650:
3641:
3640:
3530:
3529:
3495:
3478:
3477:
3468:
3467:
3427:
3421:
3420:
3380:
3374:
3373:
3337:
3331:
3330:
3294:
3288:
3287:
3261:
3237:
3231:
3230:
3228:
3227:
3213:
3207:
3206:
3170:
3164:
3163:
3119:
3113:
3112:
3086:
3062:
3051:
3050:
3006:
3000:
2999:
2971:
2965:
2964:
2928:
2922:
2921:
2877:
2871:
2870:
2826:
2820:
2819:
2783:
2777:
2776:
2740:
2734:
2733:
2689:
2680:
2679:
2643:
2628:
2627:
2599:
2590:
2589:
2557:
2548:
2547:
2515:
2506:
2505:
2461:
2452:
2451:
2449:
2448:
2434:
2423:
2422:
2389:(7–8): 484–489.
2378:
2357:
2356:
2354:
2353:
2339:
2328:
2327:
2302:(6): 1211–1231.
2291:
2274:
2273:
2255:
2231:
2216:
2215:
2205:
2187:
2163:
2146:
2145:
2100:
2083:
2082:
2037:
1878:
1877:
1835:
1826:
1747:
1746:
1744:
1743:
1729:
1718:
1717:
1673:
1658:
1657:
1632:(6): 1289–1300.
1621:
1610:
1609:
1591:
1567:
1538:
1510:Kenneth Blackfan
1379:fourth ventricle
1210:. Cardiac-gated
1084:fourth ventricle
1082:and an enlarged
971:fourth ventricle
947:fourth ventricle
880:Edwards syndrome
799:, among others.
785:Aicardi syndrome
721:Joubert syndrome
679:Fourth ventricle
540:bipolar disorder
458:bipolar disorder
364:
287:and can include
261:fourth ventricle
200:Cystoperitoneal
95:
94:
86:Medical genetics
75:in an 8-year old
55:
35:
34:
21:
3966:
3965:
3961:
3960:
3959:
3957:
3956:
3955:
3926:
3925:
3924:
3915:
3889:
3853:
3790:Hydranencephaly
3734:
3677:
3668:
3638:
3633:
3632:
3609:
3608:
3541:
3527:
3501:
3499:Further reading
3484:
3475:
3472:
3471:
3428:
3424:
3389:World Neurosurg
3381:
3377:
3338:
3334:
3295:
3291:
3238:
3234:
3225:
3223:
3215:
3214:
3210:
3171:
3167:
3120:
3116:
3063:
3054:
3007:
3003:
2972:
2968:
2929:
2925:
2878:
2874:
2843:10.1002/pd.1722
2827:
2823:
2784:
2780:
2741:
2737:
2690:
2683:
2644:
2631:
2600:
2593:
2558:
2551:
2516:
2509:
2462:
2455:
2446:
2444:
2436:
2435:
2426:
2379:
2360:
2351:
2349:
2341:
2340:
2331:
2292:
2277:
2232:
2219:
2164:
2149:
2101:
2086:
2038:
1881:
1844:(10): 886–902.
1833:
1827:
1750:
1741:
1739:
1731:
1730:
1721:
1674:
1661:
1622:
1613:
1568:
1559:
1554:
1532:
1498:posterior fossa
1486:
1474:
1449:
1440:
1419:third ventricle
1363:
1351:
1322:arachnoid mater
1318:
1312:
1279:
1243:
1235:
1208:corpus callosum
1192:
1186:abnormalities.
1139:posterior fossa
1088:posterior fossa
1075:
1047:
1021:arachnoid mater
998:
987:posterior fossa
935:
933:Pathophysiology
908:
906:External toxins
868:
781:Fraser syndrome
713:
707:
672:
546:, and 2.1% had
474:
409:
401:posterior fossa
393:sunsetting eyes
384:
379:
358:
265:posterior fossa
113:
89:
33:
28:
23:
22:
15:
12:
11:
5:
3964:
3954:
3953:
3948:
3943:
3941:Rare syndromes
3938:
3921:
3920:
3917:
3916:
3914:
3913:
3908:
3903:
3897:
3895:
3891:
3890:
3888:
3887:
3882:
3876:
3874:
3865:
3859:
3858:
3855:
3854:
3852:
3851:
3850:
3849:
3842:Polymicrogyria
3838:
3837:
3836:
3835:
3833:Schizencephaly
3830:
3817:
3816:
3811:
3809:Megalencephaly
3806:
3801:
3795:
3794:
3793:
3792:
3787:
3782:
3777:
3772:
3761:
3760:
3759:
3758:
3748:
3742:
3740:
3736:
3735:
3733:
3732:
3727:
3722:
3721:
3720:
3715:
3710:
3705:
3696:
3694:
3685:
3679:
3678:
3675:nervous system
3667:
3666:
3659:
3652:
3644:
3635:
3634:
3631:
3630:
3618:
3617:
3615:
3611:
3610:
3607:
3606:
3595:
3584:
3573:
3558:
3542:
3537:
3536:
3534:
3533:Classification
3526:
3525:External links
3523:
3522:
3521:
3515:
3510:
3500:
3497:
3470:
3469:
3422:
3375:
3348:(3): 333–337.
3332:
3289:
3252:(4): 281–295.
3232:
3208:
3165:
3114:
3077:(4): 113–118.
3052:
3017:(6): 366–374.
3001:
2966:
2923:
2888:(1): 191–194.
2872:
2837:(6): 495–501.
2821:
2794:(3): 163–169.
2778:
2751:(2): 169–171.
2735:
2700:(6): 348–352.
2681:
2654:(3): 163–169.
2629:
2610:(4): 583–612.
2591:
2572:(6): 406–482.
2549:
2530:(3): 352–361.
2507:
2472:(3): 173–175.
2453:
2424:
2358:
2329:
2275:
2246:(1): 200–220.
2217:
2147:
2112:(3): 194–199.
2084:
1879:
1748:
1719:
1684:(4): 290–295.
1678:Neuroradiology
1659:
1611:
1556:
1555:
1553:
1550:
1546:cisterna magna
1496:, an enlarged
1485:
1482:
1473:
1470:
1448:
1445:
1439:
1436:
1372:foramen magnum
1362:
1359:
1350:
1347:
1346:
1345:
1338:
1335:
1332:
1316:Arachnoid cyst
1314:Main article:
1311:
1308:
1304:
1303:
1300:
1297:
1283:cisterna magna
1278:
1275:
1271:
1270:
1267:
1264:
1261:
1242:
1239:
1234:
1231:
1191:
1188:
1179:
1178:
1171:occipital lobe
1163:
1156:
1149:
1146:
1135:
1111:cisterna magna
1074:
1071:
1046:
1043:
997:
994:
977:form from the
951:cisterna magna
934:
931:
907:
904:
890:(trisomy 13),
888:Patau syndrome
867:
864:
765:Fryns syndrome
717:PHACE syndrome
709:Main article:
706:
703:
695:cell migration
671:
668:
649:foramen magnum
635:
634:
631:
628:cryptorchidism
624:
621:arthrogryposis
617:kyphoscoliosis
609:
606:hypertrichosis
594:
589:gives rise to
563:PHACE syndrome
551:
536:
513:microphthalmia
511:, small eyes (
505:
473:
470:
439:schizencephaly
431:polymicrogyria
408:
405:
383:
380:
378:
375:
331:cerebral shunt
312:cell migration
228:
227:
224:
220:
219:
216:
210:
209:
198:
194:
193:
191:arachnoid cyst
187:
181:
180:
167:
161:
160:
150:
146:
145:
142:
138:
137:
130:
124:
123:
103:
97:
96:
83:
77:
76:
57:
56:
48:
47:
44:
40:
39:
31:
26:
9:
6:
4:
3:
2:
3963:
3952:
3949:
3947:
3944:
3942:
3939:
3937:
3934:
3933:
3931:
3912:
3911:Syringomyelia
3909:
3907:
3904:
3902:
3899:
3898:
3896:
3892:
3886:
3883:
3881:
3878:
3877:
3875:
3873:
3869:
3866:
3864:
3860:
3848:
3845:
3844:
3843:
3840:
3839:
3834:
3831:
3829:
3826:
3825:
3824:
3823:
3819:
3818:
3815:
3812:
3810:
3807:
3805:
3802:
3800:
3797:
3796:
3791:
3788:
3786:
3783:
3781:
3778:
3776:
3775:Lissencephaly
3773:
3771:
3768:
3767:
3766:
3763:
3762:
3757:
3754:
3753:
3752:
3749:
3747:
3744:
3743:
3741:
3737:
3731:
3728:
3726:
3725:Encephalocele
3723:
3719:
3716:
3714:
3711:
3709:
3706:
3703:
3702:
3701:
3698:
3697:
3695:
3693:
3689:
3686:
3684:
3680:
3676:
3672:
3665:
3660:
3658:
3653:
3651:
3646:
3645:
3642:
3629:
3625:
3624:
3620:
3619:
3616:
3612:
3605:
3601:
3600:
3596:
3594:
3590:
3589:
3585:
3583:
3579:
3578:
3574:
3572:
3568:
3567:
3563:
3559:
3557:
3553:
3552:
3548:
3544:
3543:
3540:
3535:
3531:
3519:
3516:
3514:
3511:
3508:
3507:
3503:
3502:
3496:
3493:
3489:
3488:
3482:
3465:
3461:
3457:
3453:
3449:
3445:
3442:(5): 358–63.
3441:
3437:
3433:
3426:
3418:
3414:
3410:
3406:
3402:
3398:
3395:: e654–e664.
3394:
3390:
3386:
3379:
3371:
3367:
3363:
3359:
3355:
3351:
3347:
3343:
3336:
3328:
3324:
3320:
3316:
3312:
3308:
3304:
3300:
3293:
3285:
3281:
3277:
3273:
3269:
3265:
3260:
3255:
3251:
3247:
3243:
3236:
3222:
3218:
3212:
3204:
3200:
3196:
3192:
3188:
3184:
3180:
3176:
3169:
3161:
3157:
3153:
3149:
3145:
3141:
3137:
3133:
3129:
3125:
3118:
3110:
3106:
3102:
3098:
3094:
3090:
3085:
3080:
3076:
3072:
3068:
3061:
3059:
3057:
3048:
3044:
3040:
3036:
3032:
3028:
3024:
3020:
3016:
3012:
3005:
2997:
2993:
2989:
2985:
2982:(1): 117–23.
2981:
2977:
2970:
2962:
2958:
2954:
2950:
2946:
2942:
2938:
2934:
2927:
2919:
2915:
2911:
2907:
2903:
2899:
2895:
2891:
2887:
2883:
2876:
2868:
2864:
2860:
2856:
2852:
2848:
2844:
2840:
2836:
2832:
2825:
2817:
2813:
2809:
2805:
2801:
2797:
2793:
2789:
2782:
2774:
2770:
2766:
2762:
2758:
2754:
2750:
2746:
2739:
2731:
2727:
2723:
2719:
2715:
2711:
2707:
2703:
2699:
2695:
2688:
2686:
2677:
2673:
2669:
2665:
2661:
2657:
2653:
2649:
2642:
2640:
2638:
2636:
2634:
2625:
2621:
2617:
2613:
2609:
2605:
2598:
2596:
2587:
2583:
2579:
2575:
2571:
2567:
2563:
2556:
2554:
2545:
2541:
2537:
2533:
2529:
2525:
2521:
2514:
2512:
2503:
2499:
2495:
2491:
2487:
2483:
2479:
2475:
2471:
2467:
2460:
2458:
2443:
2439:
2433:
2431:
2429:
2420:
2416:
2412:
2408:
2404:
2400:
2396:
2392:
2388:
2384:
2377:
2375:
2373:
2371:
2369:
2367:
2365:
2363:
2348:
2347:www.orpha.net
2344:
2338:
2336:
2334:
2325:
2321:
2317:
2313:
2309:
2305:
2301:
2297:
2296:Radiographics
2290:
2288:
2286:
2284:
2282:
2280:
2271:
2267:
2263:
2259:
2254:
2249:
2245:
2241:
2240:RadioGraphics
2237:
2230:
2228:
2226:
2224:
2222:
2213:
2209:
2204:
2199:
2195:
2191:
2186:
2181:
2177:
2173:
2169:
2162:
2160:
2158:
2156:
2154:
2152:
2143:
2139:
2135:
2131:
2127:
2123:
2119:
2115:
2111:
2107:
2099:
2097:
2095:
2093:
2091:
2089:
2080:
2076:
2072:
2068:
2064:
2060:
2056:
2052:
2048:
2044:
2036:
2034:
2032:
2030:
2028:
2026:
2024:
2022:
2020:
2018:
2016:
2014:
2012:
2010:
2008:
2006:
2004:
2002:
2000:
1998:
1996:
1994:
1992:
1990:
1988:
1986:
1984:
1982:
1980:
1978:
1976:
1974:
1972:
1970:
1968:
1966:
1964:
1962:
1960:
1958:
1956:
1954:
1952:
1950:
1948:
1946:
1944:
1942:
1940:
1938:
1936:
1934:
1932:
1930:
1928:
1926:
1924:
1922:
1920:
1918:
1916:
1914:
1912:
1910:
1908:
1906:
1904:
1902:
1900:
1898:
1896:
1894:
1892:
1890:
1888:
1886:
1884:
1875:
1871:
1867:
1863:
1859:
1855:
1851:
1847:
1843:
1839:
1832:
1825:
1823:
1821:
1819:
1817:
1815:
1813:
1811:
1809:
1807:
1805:
1803:
1801:
1799:
1797:
1795:
1793:
1791:
1789:
1787:
1785:
1783:
1781:
1779:
1777:
1775:
1773:
1771:
1769:
1767:
1765:
1763:
1761:
1759:
1757:
1755:
1753:
1738:
1734:
1728:
1726:
1724:
1715:
1711:
1707:
1703:
1699:
1695:
1691:
1687:
1683:
1679:
1672:
1670:
1668:
1666:
1664:
1655:
1651:
1647:
1643:
1639:
1635:
1631:
1627:
1620:
1618:
1616:
1607:
1603:
1599:
1595:
1590:
1585:
1581:
1577:
1573:
1566:
1564:
1562:
1557:
1549:
1547:
1542:
1536:
1531:
1526:
1524:
1520:
1516:
1511:
1507:
1503:
1502:hydrocephalus
1499:
1495:
1491:
1481:
1477:
1469:
1466:
1463:
1459:
1453:
1444:
1435:
1432:
1427:
1424:
1420:
1416:
1411:
1409:
1405:
1401:
1397:
1392:
1388:
1384:
1380:
1375:
1373:
1368:
1358:
1356:
1343:
1339:
1336:
1333:
1330:
1329:
1328:
1325:
1323:
1317:
1307:
1301:
1298:
1295:
1294:
1293:
1290:
1288:
1284:
1274:
1268:
1265:
1262:
1259:
1255:
1254:
1253:
1250:
1248:
1238:
1230:
1227:
1223:
1221:
1217:
1213:
1209:
1205:
1201:
1197:
1187:
1185:
1176:
1172:
1168:
1164:
1161:
1157:
1154:
1150:
1147:
1144:
1140:
1136:
1133:
1129:
1128:
1127:
1124:
1121:
1117:
1112:
1108:
1104:
1100:
1096:
1091:
1089:
1085:
1081:
1070:
1068:
1067:mood disorder
1064:
1060:
1059:hydrocephalus
1056:
1052:
1042:
1039:
1037:
1033:
1030:
1026:
1022:
1018:
1013:
1011:
1007:
1003:
993:
991:
988:
983:
980:
976:
972:
968:
964:
963:metencephalon
960:
952:
948:
944:
939:
930:
928:
927:heart defects
924:
920:
916:
912:
903:
901:
897:
893:
889:
885:
881:
877:
873:
863:
861:
860:
855:
854:
849:
848:
843:
842:
837:
836:
831:
830:
824:
822:
818:
817:cell division
814:
810:
809:primary cilia
806:
802:
798:
794:
790:
786:
782:
778:
774:
770:
766:
762:
758:
754:
750:
746:
742:
738:
734:
730:
726:
722:
718:
712:
702:
700:
696:
688:
684:
680:
676:
667:
665:
660:
658:
657:central canal
654:
650:
646:
645:Syringomyelia
642:
640:
639:encephalocele
632:
629:
625:
622:
618:
614:
610:
607:
603:
599:
595:
592:
588:
584:
580:
576:
572:
568:
567:Wilms' tumour
564:
560:
556:
552:
549:
545:
541:
537:
534:
530:
526:
522:
518:
517:chorioretinal
514:
510:
506:
504:was reported.
503:
502:heart failure
499:
495:
491:
487:
483:
479:
478:
477:
469:
467:
463:
459:
455:
454:schizophrenia
451:
446:
444:
440:
436:
432:
428:
427:lissencephaly
424:
420:
415:
404:
402:
398:
394:
389:
388:hydrocephalus
382:Hydrocephalus
374:
372:
368:
362:
357:
353:
347:
344:
340:
336:
332:
327:
325:
321:
317:
313:
309:
305:
300:
298:
297:encephalocele
294:
290:
286:
282:
278:
274:
273:hydrocephalus
270:
266:
262:
258:
254:
250:
246:
242:
238:
234:
225:
221:
217:
215:
211:
207:
203:
199:
195:
192:
188:
186:
182:
179:
175:
171:
168:
166:
162:
158:
154:
151:
147:
143:
139:
135:
131:
129:
128:Complications
125:
121:
117:
112:
108:
104:
102:
98:
93:
87:
84:
82:
78:
74:
70:
66:
62:
58:
54:
49:
45:
41:
36:
30:
19:
3885:Rachischisis
3880:Spina bifida
3828:Porencephaly
3820:
3764:
3746:Microcephaly
3718:Iniencephaly
3621:
3597:
3586:
3575:
3560:
3545:
3505:
3486:
3473:
3439:
3435:
3425:
3392:
3388:
3378:
3345:
3341:
3335:
3302:
3298:
3292:
3249:
3245:
3235:
3224:. Retrieved
3221:www.omim.org
3220:
3211:
3181:(2): 66–73.
3178:
3174:
3168:
3130:(2): 88–97.
3127:
3123:
3117:
3074:
3070:
3014:
3010:
3004:
2979:
2975:
2969:
2939:(1): 38–44.
2936:
2932:
2926:
2885:
2882:Neurosurgery
2881:
2875:
2834:
2830:
2824:
2791:
2787:
2781:
2748:
2744:
2738:
2697:
2693:
2651:
2647:
2607:
2603:
2569:
2565:
2527:
2523:
2469:
2465:
2445:. Retrieved
2441:
2386:
2382:
2350:. Retrieved
2346:
2299:
2295:
2243:
2239:
2175:
2171:
2109:
2105:
2046:
2042:
1841:
1837:
1740:. Retrieved
1736:
1681:
1677:
1629:
1625:
1582:(1): 14–29.
1579:
1575:
1540:
1527:
1506:Walter Dandy
1487:
1478:
1475:
1472:Epidemiology
1467:
1454:
1450:
1441:
1429:Previously,
1428:
1412:
1376:
1364:
1352:
1326:
1319:
1305:
1291:
1280:
1272:
1251:
1244:
1236:
1224:
1193:
1180:
1125:
1119:
1115:
1106:
1102:
1098:
1094:
1092:
1076:
1048:
1040:
1034:
1025:granulations
1017:inflammation
1014:
999:
984:
979:rhombic lips
965:, while the
956:
919:microcephaly
909:
899:
895:
869:
857:
851:
845:
839:
833:
827:
825:
813:microtubules
805:ciliopathies
714:
692:
664:spina bifida
661:
643:
636:
587:neural crest
475:
447:
410:
407:Neurological
385:
367:Walter Dandy
348:
328:
301:
244:
240:
236:
232:
231:
29:
3863:Spinal cord
3700:Anencephaly
1533: [
1258:mass effect
1029:dural veins
745:3C syndrome
739:type 7 and
591:melanocytes
583:neural tube
579:development
559:hemangiomas
521:optic nerve
412:anomalies.
359: [
308:chromosomal
304:ciliopathic
249:hemispheres
157:chromosomal
153:Ciliopathic
136:, infection
61:T2-weighted
43:Other names
3930:Categories
3785:Pachygyria
3671:Congenital
3599:DiseasesDB
3342:Teratology
3305:: 125–48.
3226:2020-01-06
2976:J. Pediatr
2447:2020-01-06
2352:2019-12-30
1742:2019-12-31
1552:References
1458:meningitis
1431:craniotomy
1196:ultrasound
1051:ultrasound
959:cerebellum
872:ultrasound
711:Ciliopathy
683:cerebellum
637:Occipital
423:pachygyria
253:cerebellum
3713:Acalvaria
3628:radio/206
3623:eMedicine
3417:201632112
3362:1096-9926
3268:1552-4876
3195:1423-0305
3144:1433-0350
3093:1741-4520
3031:0939-7248
2953:1016-2291
2902:0148-396X
2851:1097-0223
2808:1016-2291
2765:0895-0172
2714:1433-0350
2668:1016-2291
2624:0096-6754
2586:0096-8994
2544:0006-8950
2486:1433-0350
2403:0256-7040
2316:0271-5333
2262:0271-5333
2194:2229-5097
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