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DOI: 10.1055/s-0029-1220674
© Georg Thieme Verlag KG Stuttgart · New York
Neonatal Bilateral Spigelian Hernia Associated with Undescended Testes and Scalp Aplasia Cutis
Publikationsverlauf
Publikationsdatum:
09. Juni 2009 (online)
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Introduction
Spigelian hernia (SH) is a very rare entity in children and infants. A recent report found only 30 pediatric patients described in the English literature [3]. In males (the M:F ratio is 2:1), the association rate with cryptorchidism is 80%. The site of the defect in the lateral abdominal wall is well defined: the intersection between the semilunar and the lateral edge of the rectus abdominis muscle is always the weakness point of the abdominal wall in these patients. A cause-and-effect relation between cryptorchidism and the abdominal defect has been suggested; however the pathogenic pathway is still far from clear.
To contribute to the knowledge of this entity and its possible malformative associations we present a patient from our Department in whom bilateral Spigelian hernias were found at birth. In addition to bilateral cryptorchidism, the newborn had aplasia cutis congenita (ACC) of the scalp: this malformation is a rare entity, localized in the scalp in around 75% of cases [4], and previously described in association with other abdominal wall defects [2].
References
- 1 Al-Salem AH. Congenital Spigelian hernia and cryptorchidism: cause or coincidence?. Pediatr Surg Int. 2000; 16 433-436
- 2 Boothroyd AE, Hall CM. The radiological features of Goltz syndrome: focal dermal hypoplasia. A report of two cases. Skeletal Radiol. 1988; 17 505-508
- 3 Durham MM, Ricketts RR. Congenital Spigelian hernias and cryptorchidism. J Pediatr Surg. 2006; 41 1814-1817
- 4 Rodrigues RG. Aplasia cutis congenita, congenital heart lesions, and frontonasal cysts in four successive generations. Clin Genet. 2007; 71 558-560
- 5 Verdyck P, Holder-Espinasse M, Hul WV. et al . Clinical and molecular analysis of nine families with Adams-Oliver syndrome. Eur J Hum Genet. 2003; 11 457-463
- 6 Zenker M, Mayerle J, Reis A. et al . Genetic basis and pancreatic biology of Johanson-Blizzard syndrome. Endocrinol Metab Clin North Am. 2006; 35 243-253
Correspondence
Dr. F. Fascetti-LeonMD
Pediatric Surgery
Pediatrics
via Giustiniani 3 Padova
35100 Padova
Italy
Telefon: +39/49/821 36 80
Fax: +39/49/821 17 81
eMail: francesco.fascettileon@unipd.it