Neonatal Bilateral Spigelian Hernia Associated with Undescended Testes and Scalp Aplasia Cutis

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

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• 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
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Correspondence