Introduction And Importance: Dandy-Walker malformation is a rare congenital anomaly of the brain that mainly affects the cerebellum region. It is characterised by abnormal dilatation of the fourth ventricle of the brain and partial or total absence of the cerebellar vermis. This malformation may also be accompanied by other anomalies of the brain. Ante-natal diagnosis is becoming increasingly frequent given the performance of medical imaging, in particular ante-natal ultrasound and MRI. The object of this article is to clarify the possible causes of rare cystic malformations of the posterior cerebral fossa, which are very rare congenital malformations.
Case Presentation: a 30 year old patient, second gesture, mother of a live child by caesarean section, referred to us at 32 weeks of amenorrhoea at the university hospital centre for management of a cystic malformation of the posterior cerebral malformation detected on 2nd trimester ultrasound and confirmed as a Dandy Walker malformation on 3rd trimester fetal MRI.
Clinical Discussion: The Dandy-Walker malformation can be described on prenatal MRI as vermian hypoplasia and can be detected as early as the 1st trimester of pregnancy using ultrasound, This cystic malformation poses a problem of differential diagnosis with other pathologies which also result in a cystic image of the posterior cerebral fossa, in particular Black's pouch cyst, arachnoid cyst and mega magna cistern, which requires careful interpretation of cerebral MRI of the foetus.
Conclusion: Imaging techniques play a fundamental role in diagnosis. Prenatal ultrasound and MRI can reveal a Dandy-Walker malformation as early as the 2nd month of pregnancy. MRI is ideal for differentiating differential diagnoses.
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http://dx.doi.org/10.1016/j.ijscr.2023.109037 | DOI Listing |
Ann Med Surg (Lond)
December 2024
Department of Neurosurgery, Hannover Medical School, Hannover, Germany.
Introduction: Dandy-Walker syndrome (DWS), a complex neurodevelopmental disorder, has intrigued clinicians and researchers since its description by physicians Walter Dandy and Arthur Walker. Despite its recognition for nearly a century, understanding its etiology, pathogenesis, and clinical manifestations remains elusive. This bibliometric analysis aims to elucidate influential academic works on DWS.
View Article and Find Full Text PDFCureus
October 2024
Department of Radiodiagnosis, Sawai Man Singh (SMS) Medical College, Jaipur, IND.
Goldston syndrome (GS) is an extremely rare syndrome involving the central nervous system and kidneys. It is believed to have a familial association and an autosomal recessive inheritance and is characterized by the concomitant occurrence of cystic dysplastic kidneys and Dandy-Walker malformation. We report a case of antenatally detected GS at 22 weeks of gestation in a female with a consanguineous marriage.
View Article and Find Full Text PDFCardiol Young
November 2024
Department of Pediatric & Congenital Cardiovascular Surgery, St Joseph's Children's Hospital, Tampa, FL, USA.
Objectives: We sought to examine the relative importance of surgical lesion complexity versus the presence of genetic/syndromic/extracardiac anomalies (GSAs) in determining survival, morbidity or need for reinterventions following repair for aortic arch hypoplasia.
Methods: A single-centre, retrospective cohort study of infants undergoing biventricular aortic arch repair sternotomy from 2010 to 2021 was conducted. Survival analysis was performed using Kaplan-Meier methods, with additional Bayesian survival modelling for subgroups.
Brain Commun
November 2024
CHU Sainte-Justine Azrieli Research Centre, Montreal H3T 1C5, Canada.
Hydrocephalus and Dandy-Walker malformation are amongst the most common congenital brain anomalies. We identified three consanguineous families with both obstructive hydrocephalus and Dandy-Walker malformation. To understand the molecular basis of these anomalies, we conducted genome-wide sequencing in these families.
View Article and Find Full Text PDFUltrasound Obstet Gynecol
December 2024
Department of Ultrasound, Beijing Obstetrics and Gynecology Hospital, Capital Medical University, Beijing Maternal and Child Health Care Hospital, Beijing, China.
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