Dandy–Walker syndrome associated with syringomyelia in an adult: a case report and literature review

Zhang, Nan; Qi, Zhenyu; Zhang, Xuewen; Zhong, Fangping; Yao, Hui; Xiang, Xu; Liu, Jiangang; Huang, Yung-Sung

doi:10.1177/0300060518808961

Cited by 10 publications

(5 citation statements)

References 15 publications

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“…DWS is normally presented in childhood, however, rare cases are also reported in adulthood. 6 This is the first report of DWS in a sixteen-year-old female from Afghanistan. The condition is linked to congenital cardiac anomalies, spina bifida or anencephaly, cleft lip, cleft palate and Hirschsprung’s disease in some cases.…”

Section: Discussionmentioning

confidence: 77%

“…The condition is linked to congenital cardiac anomalies, spina bifida or anencephaly, cleft lip, cleft palate and Hirschsprung’s disease in some cases. 6 Predisposing factors of DWS are common congenital infections such as toxoplasmosis, cytomegalovirus and rubella, and drugs like, warfarin, retinol derivative (isotretinoin) and ethanol. 7 , 8 However, we are not certain whether the mother of this patient had faced these factors or not.…”

Section: Discussionmentioning

confidence: 99%

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A Rare Case of Dandy-Walker Syndrome

Oria¹,

Rasib²,

Pirzad³

et al. 2022

IMCRJ

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Dandy-Walker syndrome (DWS) is a rare congenital malformation characterized by hypoplasia of the cerebellar vermis and its upward rotation and cystic enlargement of the fourth ventricle. The clinical manifestations include psychomotor retardation, ataxia and hydrocephalus. We report a case of 16-year-old female patient in Ali Abad Teaching Hospital who was suffering from unsteady gait, memory deterioration and urinary incontinence. A brain magnetic resonance imaging revealed enlarged cystic posterior fossa, dilated fourth ventricles and upward rotated cerebellar vermis which were indicating DWS. The patient prepared for planned surgical operation and a written informed consent was obtained from her parents for surgery and general anesthesia. A cystoperitoneal (CP) shunt was placed and then the patient transferred to recovery room. After recovery and hospital stay, the patient discharged with improved clinical symptoms.

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Section: Discussionmentioning

confidence: 77%

Section: Discussionmentioning

confidence: 99%

A Rare Case of Dandy-Walker Syndrome

Oria¹,

Rasib²,

Pirzad³

et al. 2022

IMCRJ

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“…3). As well, some authors report the association between DWM and syringomyelia that probably occur due to the herniation of the cyst through the foramen magno changing the CSF circulation, similar to what happen in the Chiari I malformation [2,36,37].…”

Section: Epidemiology and Clinical Featuresmentioning

confidence: 68%

Hydrocephalus and Dandy- Walker Malformation: a review

Dastoli

Nicácio

Costa

et al. 2020

Arch Pediat Neurosurg

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Introduction: Dandy Walker malformation (DWM) is characterized by agenesis or hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle leading to an increase of the posterior fossa and superior dislocation of the lateral sinus, tentorium and torcula. Although it is the commonest posterior fossa malformation, its pathogenesis is still not fully understood, making the differential diagnosis with other posterior fossa malformations difficult and as a result the choice of therapeutic strategy. Material and methods: An extensive review of the literature relating to Dandy Walker malformation was performed. Historical, genetic, embryologic, epidemiologic, clinical and radiological presentation, treatment and prognosis were revised. Conclusion: The correct diagnosis of Dandy Walker malformation can be made through careful interpretation of magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) flow studies. The choice of hydrocephalus treatment depends on whether there is aqueduct stenosis. And, although ventriculoperitoneal (VP) shunts have been the treatment of choice for many years, neuroendoscopic techniques such as endoscopic thirdventriculostomy and stent placement are being frequently performed lately.

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“…During The prognosis of NCM is dismal, especially when presented in adults and associated with DWS [7]. associated with ganglioglioma [8]; with Associated Neurofibromatosis [9]; Refractory psychiatric symptoms and seizure [10]; Hemorrhagic Stroke in a Young Adult [11]. Syringomyelia in an Adult [12].…”

Section: Clinical Casementioning

confidence: 99%

Hydrocephalus and Dandy-Walker syndrome. Clinical case

Villalobos-Rodríguez

2023

AJMCRR

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Introduction. Dandy-Walker syndrome (DWS) is a developmental abnormality characterized by dilated posterior fossa, cystic enlargement of the fourth ventricle, hypoplasia of cerebellar vermis, and its upward rotation. The affected person may suffer from psychomotor retardation, ataxia, apnea attacks, muscle weakness, occasional muscle spasm, seizures, nystagmus, and macrocephaly. However, half of the cases have average intelligence. Clinical Case. Male newborn at 36 weeks gestation obtained by cesarean section, with a history in the current pregnancy of poor prenatal control. During the fifth month of pregnancy, an ultrasound established a diagnostic suspicion of massive ventriculomegaly. At the sixth month, thinning of the cerebral cortex compression of the cerebellum in the posterior fossa was detected, and a possible ruling out of chromosomopathy related to Dandy-Walker Syndrome was suggested. Discussion. Neurologically, this disease causes multiple anomalies, such as Hemorrhagic Stroke in a Young Adult with Undiagnosed Asymptomatic Dandy–Walker Malformation (5). The embryonic development of the cerebellum and fourth ventricle causes the tentorium to be displaced upwards, and the posterior fossa enlarges. The association of hydrocephalus with DWS is due to blockage of normal cerebrospinal flow, leading to an excessive amount of fluid accumulating in and around the brain and causing an increase in intracranial pressure and head circumference, which ultimately causes neurological impairment. Conclusions. The prognosis of patients with this syndrome depends on the association with intra/extracranial malformations. Early diagnosis of central nervous system abnormalities is considered one of the first objectives of obstetric ultrasound.

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Dandy–Walker syndrome associated with syringomyelia in an adult: a case report and literature review

Cited by 10 publications

References 15 publications

A Rare Case of Dandy-Walker Syndrome

A Rare Case of Dandy-Walker Syndrome

Hydrocephalus and Dandy- Walker Malformation: a review

Hydrocephalus and Dandy-Walker syndrome. Clinical case

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