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Aqueductal developmental venous anomaly as an unusual cause of congenital hydrocephalus: a case report and review of the literature

  • David Paulson1,
  • Steven W Hwang1,
  • William E Whitehead1,
  • Daniel J Curry1,
  • Thomas G Luerssen1 and
  • Andrew Jea1Email author
Journal of Medical Case Reports20126:7

DOI: 10.1186/1752-1947-6-7

Received: 19 July 2011

Accepted: 11 January 2012

Published: 11 January 2012

Abstract

Introduction

Aqueductal stenosis may be caused by a number of etiologies including congenital stenosis, tumor, inflammation, and, very rarely, vascular malformation. However, aqueductal stenosis caused by a developmental venous anomaly presenting as congenital hydrocephalus is even more rare, and, to the best of our knowledge, has not yet been reported in the literature. In this study, we review the literature and report the first case of congenital hydrocephalus associated with aqueductal stenosis from a developmental venous anomaly.

Case presentation

The patient is a three-day-old, African-American baby girl with a prenatal diagnosis of hydrocephalus. She presented with a full fontanelle, splayed sutures, and macrocephaly. Postnatal magnetic resonance imaging showed triventricular hydrocephalus, suggesting aqueductal stenosis. Examination of the T1-weighted sagittal magnetic resonance imaging enhanced with gadolinium revealed a developmental venous anomaly passing through the orifice of the aqueduct. We treated the patient with a ventriculoperitoneal shunt.

Conclusions

Ten cases of aqueductal stenosis due to venous lesions have been reported and, although these venous angiomas and developmental venous anomalies are usually considered congenital lesions, all 10 cases became symptomatic as older children and adults. Our case is the first in which aqueductal stenosis caused by a developmental venous anomaly presents as congenital hydrocephalus. We hope adding to the literature will improve understanding of this very uncommon cause of hydrocephalus and, therefore, will aid in treatment.

Introduction

It is common for intraventricular cerebrospinal fluid (CSF) flow to become obstructed at the aqueduct of Sylvius [1]. The obstruction may be caused by a tumor, congenital etiology, or post-inflammatory gliotic atresia, among other conditions [13]. Obstruction by a vascular malformation at the aqueduct is a very rare cause [2, 47], and aqueductal stenosis attributable to a developmental venous anomaly (DVA) is perhaps the most uncommon [8].

Although DVAs are thought of as congenital findings, all 10 previously reported cases became symptomatic as older children or adults. To the best of our knowledge, we present the first case of symptomatic congenital hydrocephalus from aqueductal obstruction due to a DVA.

Case presentation

The patient transferred to our institution was a three-day-old, African-American baby girl with a diagnosis of congenital hydrocephalus. Prenatal ultrasonographic screening at 20 weeks was unremarkable. At 37 weeks, the maternal abdominal girth exceeded the expected range, and a second ultrasound revealed interval development of congenital hydrocephalus (Figure 1). A Caesarian section was planned, but the mother went into spontaneous labor, and the baby was delivered vaginally before the procedure could be performed.
https://static-content.springer.com/image/art%3A10.1186%2F1752-1947-6-7/MediaObjects/13256_2011_Article_1959_Fig1_HTML.jpg
Figure 1

Prenatal ultrasound at 37 weeks gestation shows triventricular hydrocephalus, suggesting obstruction at the aqueduct of Sylvius.

The baby was born healthy with no obviously anomalous anatomy aside from macrocephaly, splaying of her cranial sutures, and a full anterior fontanelle. She opened her eyes spontaneously and cried to stimulation. Her pupils were equal and reactive to light; she had normal muscle tone and was moving all extremities symmetrically. Her head circumference was 35.5 cm, which correlated to the 50th percentile.

An MRI of the brain corroborated hydrocephalus with the presence of a DVA within the third ventricle (Figure 2). Even after re-reviewing the MRI of the brain with contrast with our neuroradiologist, we found that the DVA was intraventricular (inside the posterior third ventricle) in the region of the aqueduct rather than the quadrigeminal cistern. Additionally, the vein of Galen was normal size for the patient's age, and we did not see evidence of venous congestion in other regions of the brain which goes against the notion that the dilated vein seen in the third ventricle was secondary to congestion from hydrocephalus. We also closely examined the MRI of the brain with contrast with our neuroradiologist and found that the MRI did not suggest any vascular malformation. T2-weighted images did not demonstrate any abnormal signal flow voids. We did not feel that more invasive investigation with cerebral angiography and all its attendant risks was warranted in this case.
https://static-content.springer.com/image/art%3A10.1186%2F1752-1947-6-7/MediaObjects/13256_2011_Article_1959_Fig2_HTML.jpg
Figure 2

Postnatal T1-weighted axial and sagittal MRI with gadolinium at three days of life demonstrates a patent aqueduct of Sylvius, but a developmental venous anomaly (white arrow) extending from the left thalamus converging on a central draining vein bridges across the proximal orifice of the aqueduct of Sylvius and obstructs the aqueduct.

The baby's head circumference gradually increased over several days, and serial ultrasound examination showed she had progressively worsening ventriculomegaly, so she underwent placement of a ventriculoperitoneal shunt on her fifth day of life.

She was positioned supine with the head turned left, exposing the right occipital scalp. A curvilinear skin incision over the occipital scalp centered on the lambdoid suture was made for ventricular access. A linear skin incision in the midline periumbilical area was made to access the peritoneum for the distal end of the shunt catheter. A PS Medical low-pressure valve and distal catheter was then tunneled and passed in a typical fashion.

Ultrasound was used to define the trajectory of the ventricular catheter [9]. Xanthochromic CSF was visualized from the ventricular catheter corroborating old blood that was identified on the preoperative MRI. The shunt was then connected and placed in the peritoneum. The baby tolerated the procedure without incident.

She recovered well from the surgery and did not experience any post-operative complications. Routine post-operative imaging identified proper positioning of the shunt components and interval improvement of the hydrocephalus. Clinically, the patient recovered from the procedure and was subsequently discharged home in good condition.

Discussion

A number of congenital and acquired factors are known to cause aqueductal stenosis and subsequent obstructive hydrocephalus [13]. Vascular lesions causing aqueductal stenosis are rare but, when present, can be attributed to a DVA (or venous angioma), vein of Galen malformation, abnormal draining vein, ectatic basilar artery, dural arteriovenous fistula, arteriovenous malformation, or cavernoma [2, 3, 58, 1015].

Aqueductal stenosis caused by a vein or DVA is extremely rare [35, 10, 1517]. Only 10 cases have been reported (Table 1) in our review of the literature. There were 6 females and 4 males. Most patients were older children or young adults, with a mean age of 29 years (range 7 to 58 years). Symptoms were present for one month to several years prior to diagnosis and treatment. Headache was the most common presenting symptom. In our opinion, what makes our case unusual and worthy of publication is that our patient would be the youngest reported case in the literature with hydrocephalus occurring in the perinatal period from DVA.
Table 1

Patients with hydrocephalus from aqueductal stenosis from venous lesions (modified from Giannetti et al.)

Author, Year

[Reference]

Age/Sex

Symptoms (duration)

Imaging

Venous lesion

Treatment

Rosenheck, 1937 [16]

58 yo/F

Mental deterioration (5 years)

Postmortem

DVA

None

Avman and Dincer, 1980 [10]

35 yo/F

Headache (7 years)

Ventriculography, CT, and angiography

Venous element

Stent

Watanabe et al., 1991 [3]

39 yo/M

Headache (1 year)

CT, MRI, and angiography

Venous malformation

Shunt

Oka et al., 1993 [7]

43 yo/F

Seizures (2 months)

CT, MRI, and angiography

Venous malformation

ETV

Blackmore and Mamourian, 1996 [5]

16 yo/F

Headache, behavior changes (2 months)

MRI

DVA

None

Bannur et al., 2002 [4]

11 yo/M

Headache (5 months)

CT and MRI

DVA

Shunt

Sato et al., 2004 [2]

28 yo/F

Headache, diplopia (N/A)

CT, MRI, and angiography

DVA

ETV

Yagmurlu et al., 2005 [15]

7 yo/F

Headache (1 month)

MRI

DVA

None

Giannetti et al., 2008 [8]

42 yo/M

Headache, behavior changes (1 year)

CT and MRI

DVA

ETV

 

18 yo/M

Headache (6 years)

CT and MRI

Venous loop

ETV

Present case

Birth/F

Full fontanelle, splayed sutures, increased head circumference

MRI

DVA

Shunt

CT, computed tomography; DVA, developmental venous anomaly; ETV, endoscopic third ventriculostomy

In our case and a recent report of two other cases, an MRI based on a gadolinium-enhanced T1-weighted sequence revealed the aqueductal obstruction and its causes [8]. Aqueductal stenosis may be confirmed during endoscopic surgery [8], but direct visualization is not necessary. Likewise, invasive angiography is not necessary for the diagnosis of these venous lesions [4, 8, 18, 19]. Unfortunately, we do not have an magnetic resonance angiography (MRA) or magnetic resonance ventriculography (MRV) to add to our case report. We did not initially suspect a venous anomaly as a possible cause for hydrocephalus, so we did not order an MRA or MRV upfront. Moreover, we do not feel that the risks justify putting the baby through general anesthesia again to obtain an imaging study that would confirm what is already seen on her MRI of the brain with contrast - a DVA causing obstructive hydrocephalus at the level of the origin of the cerebral aqueduct.

DVAs are usually benign lesions and considered normal variants that drain healthy brain tissue and do not need treatment because there is no risk of rupture and bleeding. Attempts to remove or occlude them may cause venous infarction or edema of normal parenchyma [4, 5, 15]. Therefore, primary treatment should be targeted towards addressing hydrocephalus, if the DVAs are symptomatic. Treatment options in these cases include close observation, aqueductal stenting, shunt placement, and endoscopic third ventriculostomy (Table 1).

A discussion of the pros and cons of endoscopic third ventriculostomy (ETV) versus ventriculoperitoneal shunt (VPS) to address hydrocephalus is beyond the scope of this report. In brief, we chose to treat our patient with a VPS because of ETV's high rate of failure in the infant age group [20].

Conclusions

Aqueductal stenosis and consequent obstructive hydrocephalus by a DVA is extremely rare. Congenital hydrocephalus can present in this way. Enhanced T1-weighted sagittal MRI sequences may be informative for examination of the aqueductal region. No treatment is recommended for the DVA; however, CSF diversion may be necessary for the treatment of hydrocephalus. ETV should be considered in older children with this pathology or later shunt malfunction in this population.

Consent

Written informed consent was obtained from the patient's next of kin for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Declarations

Acknowledgements

The authors would like to recognize Lily Chun for her editorial assistance in the production of this manuscript.

Authors’ Affiliations

(1)
Division of Pediatric Neurosurgery, Texas Children's Hospital, Department of Neurosurgery, Baylor College of Medicine

References

  1. Jellinger G: Anatomopathology of non-tumoral aqueductal stenosis. J Neurosurg Sci. 1986, 30: 1-16.PubMedGoogle Scholar
  2. Sato S, Sonoda Y, Kuroki R, Kayama T: [A rare case of aqueductal stenosis due to venous angioma]. No To Shinkei. 2004, 56: 1042-1046.PubMedGoogle Scholar
  3. Watanabe A, Ishii R, Kamada M, Suzuki Y, Hirano K, Okamura H: Obstructive hydrocephalus caused by an abnormal vein in the aqueduct. Case report. J Neurosurg. 1991, 75: 960-962. 10.3171/jns.1991.75.6.0960.View ArticlePubMedGoogle Scholar
  4. Bannur U, Korah I, Chandy MJ: Midbrain venous angioma with obstructive hydrocephalus. Neurol India. 2002, 50: 207-209.PubMedGoogle Scholar
  5. Blackmore CC, Mamourian AC: Aqueduct compression from venous angioma: MR findings. AJNR Am J Neuroradiol. 1996, 17: 458-460.PubMedGoogle Scholar
  6. Brugieres P, Combes C, el-Khoury C, Decq P, Heine P, Meyrignac C, Gaston A: Aqueduct stenosis due to venous ectasia with a dural arteriovenous fistula. Neuroradiology. 2000, 42: 267-271. 10.1007/s002340050883.View ArticlePubMedGoogle Scholar
  7. Oka K, Kumate S, Kibe M, Tomonaga M, Maehara F, Higashi Y: Aqueductal stenosis due to mesencephalic venous malformation: case report. Surg Neurol. 1993, 40: 230-235. 10.1016/0090-3019(93)90072-9.View ArticlePubMedGoogle Scholar
  8. Giannetti AV, Rodrigues RB, Trivelato FP: Venous lesions as a cause of sylvian aqueductal obstruction: case report. Neurosurgery. 2008, 62: E1167-1168. 10.1227/01.neu.0000325882.21118.7d. discussion E1168View ArticlePubMedGoogle Scholar
  9. Whitehead WE, Jea A, Vachhrajani S, Kulkarni AV, Drake JM: Accurate placement of cerebrospinal fluid shunt ventricular catheters with real-time ultrasound guidance in older children without patent fontanelles. J Neurosurg. 2007, 107: 406-410.PubMedGoogle Scholar
  10. Avman N, Dincer C: Venous malformation of the aqueduct of Sylvius treated by interventriculostomy: 15 years follow-up. Acta Neurochir (Wien). 1980, 52: 219-224. 10.1007/BF01402077.View ArticleGoogle Scholar
  11. Branco G, Goulao A, Ferro JM: MRI in aqueduct compression and obstructive hydrocephalus due to an ecstatic basilar artery. Neuroradiology. 1993, 35: 447-448. 10.1007/BF00602826.View ArticlePubMedGoogle Scholar
  12. Esparza J, Lobato RD, Munoz MJ, Chillon D, Portillo JM, Lamas E: Giant cerebral arteriovenous malformation producing a noncommunicating hydrocephalus. Surg Neurol. 1981, 15: 76-80. 10.1016/S0090-3019(81)80096-1.View ArticlePubMedGoogle Scholar
  13. Hoi SU, Kerber C: Ventricular obstruction secondary to vascular malformations. Neurosurgery. 1983, 12: 572-575. 10.1227/00006123-198305000-00018.View ArticleGoogle Scholar
  14. Pribil S, Boone SC, Waley R: Obstructive hydrocephalus at the anterior third ventricle caused by dilated veins from an arteriovenous malformation. Surg Neurol. 1983, 20: 487-492. 10.1016/0090-3019(83)90032-0.View ArticlePubMedGoogle Scholar
  15. Yagmurlu B, Fitoz S, Atasoy C, Erden I, Deda G, Unal O: An unusual cause of hydrocephalus: aqueductal developmental venous anomaly. Eur Radiol. 2005, 15: 1159-1162. 10.1007/s00330-004-2356-7.View ArticlePubMedGoogle Scholar
  16. Rosenheck C: Venous angioma of the sylvian aqueduct and the fourth ventricle associated with internal hydrocephalus and mental deterioration. Arch Neurol Psychiatry. 1937, 38: 428-438.View ArticleGoogle Scholar
  17. Tien R, Harsh GRt, Dillon WP, Wilson CB: Unilateral hydrocephalus caused by an intraventricular venous malformation obstructing the foramen of Monro. Neurosurgery. 1990, 26: 664-666. 10.1227/00006123-199004000-00017.View ArticlePubMedGoogle Scholar
  18. Lee C, Pennington MA, Kenney CM: MR evaluation of developmental venous anomalies: medullary venous anatomy of venous angiomas. AJNR Am J Neuroradiol. 1996, 17: 61-70.PubMedGoogle Scholar
  19. Ostertun B, Solymosi L: Magnetic resonance angiography of cerebral developmental venous anomalies: its role in differential diagnosis. Neuroradiology. 1993, 35: 97-104. 10.1007/BF00593962.View ArticlePubMedGoogle Scholar
  20. Drake JM, Kulkarni AV, Kestle J: Endoscopic third ventriculostomy versus ventriculoperitoneal shunt in pediatric patients: a decision analysis. Childs Nerv Syst. 2009, 25: 467-472. 10.1007/s00381-008-0761-y.View ArticlePubMedGoogle Scholar

Copyright

© Paulson et al; licensee BioMed Central Ltd. 2012

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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