ANOMALIES ASSOCIATED WITH

CONGENITAL HYDROCEPHALUS

Associated anomalies are important to detect, as the prognosis is dependent on their presence or absence.

• Anomalies are present in 70-83% of fetuses (1-3).
• 40% are intracranial
• microcephaly.
• encephalocele.
• 60% are extracranial
• meningocele.
• cardiac or renal anomalies (20%).
• polyhydramnios (30%).
• oligohydramnios (25-33%).
• chromosomal anomalies
• Trisomies 13, 18 and 21.
• Mortality = 60-74% (1-3).

PROGNOSIS OF UNILATERAL / ASYMMETRICAL VENTRICULOMEGALY

Lipitz et.al. (4) reviewed 27 cases and suggested that isolated unilateral ventriculomegaly, and concluded that isolated causes are not associated with neurological sequelae.

Patten et.al. (5) reported that in 3 of 6 cases there were associated motor and developmental disabilities postnatally.

Senat et.al. (6) reported that all isolated cases of unilateral ventriculomegaly have favorable neurological outcomes.

Durfee et.al. (7) describe 14 fetuses with asymmetrical ventriculomegaly (13 were unilateral). 58% of their fetuses had abnormal outcomes, which included several syndromes, developmental delays and intracranial hemorrhages.

Kinzler et.al. (8) describe 15 fetuses with unilateral ventriculomegaly. As an isolated finding developmental outcome tends to be normal.

REFERENCES

1. Pretorius DH, Davis K, Manco-Johnson ML et.al. Clinical course of fetal hydrocephalus: 40 cases. AJR 1985;144:827-831.
2. Nyberg AD, Mack LA, Hirsch J et.al. Fetal hydrocephalus: Sonographic detection and clinical significance of associated anomalies. Radiology 1987;163:187-191.
3. Hudgins RJ, Edwards MSB, Goldstein R et.al. Natural history of fetal ventriculomegaly. Pediatrics 1988;82:692-697.
4. Lipitz S, Yagel S, Malinger G et.al. Outcome of fetuses with isolated borderline unilateral ventriculomegaly diagnosed at mid gestation. Ultrasound Obstet Gynecol 1998;12:23-26.
5. Patten RM, Mack LA, Finberg HJ. Unilateral hydrocephalus: prenatal sonographic diagnosis. Am J Roentgenol 1991;156:359-363.
6. Senat MV, Bernard JP, Schwarzler P et.al. Prenatal diagnosis and follow-up of 14 cases of unilateral ventriculomegaly. Ultrasound Obstet Gynecol 1999;14:327-332.
7. Durfee SM, Kim FM, Benson CB. Postnatal outcome of fetuses with prenatal diagnosis of asymmetric hydrocephalus. J Ultrasound Med 2001;20:263-268.
8. Kinzler WL, Smulian JC, McLean DA et. Al. Outcome of prenatally diagnosed mild unilateral cerebral ventriculomegaly. J Ultrasound Med 2001;20:257-262.