Familial recurrence of gastroschisis: literature review and data from the population-based birth registry "Mainz Model".
BACKGROUND
Familial forms of gastroschisis are considered rare. A search for these forms in a population-based birth registry in 1993 found a recurrence risk of 3.5% among first-degree relatives. Since then, similar investigations in population-based registries have led to contradictory results.


METHODS
A search of the population-based birth registry "Mainz Model" for familial cases of gastroschisis and a systematic review of the literature were performed.


RESULTS
The Mainz Model database yielded 1 familial recurrence out of 27 gastroschisis cases. From the literature, 37 affected families could be retrieved. Among 412 gastroschises from population-based registries, 10 familial recurrences have been found. These translate into a recurrence risk of 2.4%, with a strong tendency toward underestimation.


CONCLUSIONS
The existing data support the hypothesis that familial recurrence of gastroschisis is much more likely than previously thought.
Kohl
Michael
M
Department of Pediatric Surgery, University Hospitals, Johannes Gutenberg University, 55101 Mainz, Germany. kohl-michael@web.de
Wiesel
Avi
A
Schier
Felix
F
D005190:Family; D020139:Gastroschisis / Q000453:epidemiology*; D006801:Humans; D012042:Registries