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CLINICAL IMAGE Table of Contents  
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Gross fetal hydrocephalus due to aqueductal stenosis: Prognostic significance of prenatal ultrasound


 Department of Radiology, St. John's Hospital, Bengaluru, Karnataka, India

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Date of Submission02-Mar-2022
Date of Decision05-Jun-2022
Date of Acceptance06-Aug-2022
Date of Web Publication07-Sep-2022
 


How to cite this URL:
Reddy R. Gross fetal hydrocephalus due to aqueductal stenosis: Prognostic significance of prenatal ultrasound. Apollo Med [Epub ahead of print] [cited 2022 Sep 27]. Available from: https://apollomedicine.org/preprintarticle.asp?id=355692





  Description Top


A 28-year-old primigravida was referred for the third trimester ultrasound scan for fetal growth and well-being. She had no prior antenatal ultrasound scan performed. An ultrasound scan of a fetus at 35 weeks gestation suggested abnormal dilatation of both lateral ventricles. Biparietal diameter (BPD) and Head circumference (HC) fetal parameters were >97 percentile range as compared to AC and FL. Furthermore, ultrasound revealed findings such as secondary macrocephaly with thinning of the cortex mantle [Figure 1]. Posterior fossa was relatively normal with no dilatation of the 4th ventricle. A diagnosis of gross hydrocephalus secondary to aqueductal stenosis was made based on the ultrasound findings. Postmortem examination on the still born fetus confirmed the diagnosis of gross hydrocephalus. However, differentials included were ventricular obstruction secondary to intraventricular hemorrhage, aqueduct stenosis, and TORCH infection.
Figure 1: Antenatal ultrasound image of the fetal head demonstrating grossly dilated lateral cerebral ventricles with mantle of cortical brain parenchyma noted at the periphery of the calvarium suggesting gross hydrocephalus

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  Discussion Top


Prenatal hydrocephalus commonly persists in the postnatal period and is associated with dilatation of lateral ventricles, volume loss of periventricular white matter, thinning of gray matter, and delayed or abnormal myelination. Studies have suggested a strong correlation between ventricular volumes on prenatal ultrasound scans and delayed maturation of cerebral brain parenchyma.[1] On routine antenatal ultrasound scans, hydrocephalus can be assessed by measuring the width of atrium of the lateral ventricles. Progressive hydrocephalus is associated with macrocephaly, fetal trisomies, agenesis of corpus collosum, and gray matter heterotopia due to abnormal migration of neurons from the periventricular germinal matrix to cerebral cortex.[2] Although commonly encountered as a congenital form, aqueductal stenosis may rarely be inherited in a X-linked recessive manner (Bickers-Adams-Edwards syndrome), and hence, the patient should be subjected to prenatal genetic testing. Mild dilatation of lateral ventricles with width of atrium of the lateral ventricle measuring 1.0–1.5 cm with no additional central nervous system abnormalities has been termed as “mild ventriculomegaly” which is an early structural marker and predictor of prognosis for neuropsychiatric impairment.[3] The relative dilatation of lateral ventricles diagnosed in fetuses on the 2nd and 3rd trimester ultrasound scans appears to be well preserved into early postnatal period and beyond and therefore requires strict monitoring and follow-up. Prenatal diagnosis of aqueductal stenosis carries a grave prognosis with features such as gross dilatation of lateral, third ventricles and a near-normal posterior fossa, with secondary macrocephaly and thinning of cortical mantle.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients have given their consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgement(s)

I wish to thank Mrs. Mani Sabbavarapu for her assistance in proofreading and native English editing of the manuscript.

Conflicts of interest

There are no conflicts of interest.

Financial support and sponsorship

Nil.



 
  References Top

1.
Spennato P, Saccone G, Fratta A, Scala MR, Sarno L, Gragnano E, et al. Prenatal diagnosis and postnatal management of congenital unilateral hydrocephalus for stenosis of the foramen of Monro. Radiol Case Rep 2021;16:2530-3.  Back to cited text no. 1
    
2.
Cinalli G, Spennato P, Nastro A, Aliberti F, Trischitta V, Ruggiero C, et al. Hydrocephalus in aqueductal stenosis. Childs Nerv Syst 2011;27:1621-42.  Back to cited text no. 2
    
3.
Senat MV, Bernard JP, Delezoide A, Saugier-Veber P, Hillion Y, Roume J, et al. Prenatal diagnosis of hydrocephalus-stenosis of the aqueduct of Sylvius by ultrasound in the first trimester of pregnancy. Report of two cases. Prenat Diagn 2001;21:1129-32.  Back to cited text no. 3
    

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Correspondence Address:
Ravikanth Reddy,
Department of Radiology, St. John's Hospital, Bengaluru - 560 034, Karnataka
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/am.am_34_22



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