Genetics and Genetic Counselling in Neonatal Hydrocephalus

Habib, Zacharias

doi:10.1097/00006254-198110010-00001

Cited by 37 publications

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“…In most cases this is due to a complete or partial obstruction to the out flow of ventricular fluid from the ventricular system [1][2][3][4], In children and adults, such obstruction can occur either within the ventricular system or in the subarachnoid space at the level of the arachnoid granula tions as in postmeningitis communicating hydrocephalus. Congenital hydrocephalus is usually due to an obstruction within the ven tricular system, most commonly at the level of the cerebral aqueduct.…”

mentioning

confidence: 99%

“…While it is usually sporadic in its occurrence and there fore there is rarely a positive family history, a significant minority (approximately 2%) of the cases are inherited as an X-linked reces sive trait. In most cases, however, the etiol ogy is unknown [2], This disorder poses dif ficult practical and ethical problems for the family and physicians. It is relatively com mon, rarely lethal and often severely handi capping.…”

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confidence: 99%

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Congenital Hydrocephalus: Treatment in utero

Clewell¹

1988

Fetal Diagn Ther

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Congenital hydrocephalus occurs in about 1 in every 1,000 live births. Is is rarely lethal but frequently severely handicapping. It can be diagnosed by ultrasound as early as 20 weeks of gestation. When not accompanied by other serious malformations, it may be amenable to treatment in utero. Experimental treatment by shunting the lateral ventricle to the amnion has been shown to control ventricular enlargement during fetal life. The mental development of treated babies has, however, been disappointing. At this time no in utero treatment of fetal hydrocephalus is being done.

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confidence: 99%

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confidence: 99%

Congenital Hydrocephalus: Treatment in utero

Clewell¹

1988

Fetal Diagn Ther

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“…Hydrocephalus usually is caused by an obstruction of the fluid pathways, but it can also occur due to excessive production of cerebrospinal fluid as seen in patients with choroid plexus papilloma 6,7 . Clinically, hydrocephalus can affect individuals at an early age; it can be fetal‐ or infantile‐congenital or acquired as in adults following trauma 1 …”

Section: General Considerations On Hydrocephalusmentioning

confidence: 99%

Characteristics of individuals with hydrocephalus and their dental care needs

Gallarreta

Bernardotti

Freitas

et al. 2010

Special Care in Dentistry

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Hydrocephalus is considered a congenital disease affecting the central nervous system, and is characterized by accumulation of cerebrospinal fluid in the lateral ventricles of the brain causing progressive ventricular dilatation. This review paper reports on the oral characteristics of persons with hydrocephalus and discusses their dental care needs. It has been reported that patients with hydrocephalus can present chronological changes in the eruption of their teeth, changes in the occlusion, greater accumulation of plaque, and have a higher caries prevalence. Regular dental visits are needed to control caries as well as to educate the caretakers about oral hygiene for children with hydrocephalus. In addition, the dental practitioner should be careful not to put pressure on the ventricular-peritoneal drainage tube during dental treatment. Special devices are available to support the patient's head if they have macrocephally.

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“…The pyramids are generally absent. 40 The hands in affected infants are said to show a characteristic clenching with adduction deformity of the thumbs.40 41 Occipital encephalocoele and nuchal cystic hygroma are sometimes confused on ultrasound examination. Most encephalocoeles are isolated clefting, which are associted with a syndrome that only results in growth disproportion in late pregnancy or infancy.…”

Section: Skeletal Dysplasias and Other Causes Of Limb Reductionmentioning

confidence: 99%

Examination of products of conception terminated after prenatal investigation.

Knowles¹

1986

Journal of Clinical Pathology

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SUMMARY A large number of district general hospitals have access to diagnostic ultrasonography and other methods of prenatal diagnosis, resulting in an increased supply of freshly terminated malformed fetuses to general histopathology departments, and there is now more open discussion of malformation and greater concern over fetal wastage. General pathologists are therefore under greater pressure to produce complete and detailed descriptions of a wide range of often complex anomalies. The dismissal of specimens as "multiple congenital anomalies" is becoming increasingly unacceptable to couples who wish to embark on further pregnancies and to their medical attendants. As in other fields an understanding of the methods and terminology in clinical use and a consistent diagnostic approach should help pathologists to extract sufficient information for accurate counselling.

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Genetics and Genetic Counselling in Neonatal Hydrocephalus

Cited by 37 publications

References 0 publications

Congenital Hydrocephalus: Treatment in utero

Congenital Hydrocephalus: Treatment in utero

Characteristics of individuals with hydrocephalus and their dental care needs

Examination of products of conception terminated after prenatal investigation.

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