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Pediatric cases of Spigelian hernias are rare. Only a few reports on this condition, in combination with ipsilateral cryptorchidism and testis in the hernia sac, have been published. We report on Spigelian hernia in a 3-week-old boy containing both the ipsilateral testis, without a gubernaculum and an incarcerated loop of the small intestine. It has been suggested that the combination of Spigelian hernia and ipsilateral cryptorchidism is part of a new syndrome. We discuss whether the lack of a gubernaculum and an inguinal canal reported in other similar cases may be additional elements of this new syndrome. We present a comprehensive overview of pediatric patients with Spigelian-cryptorchidism syndrome reported in the English language literature. In 75% of male infants with Spigelian hernia, there is an associated ipsilateral cryptorchidism, and in 87% of these patients, the testis is found inside the hernia sac. Thus, the surgeon dealing with a congenital Spigelian hernia should look for an undescended testis and be prepared to find it in the hernia sac.

Low Spigelian hernia (LSH) in children is considered an extreme surgical rarity. This clinically deceptive hernia is difficult to diagnose preoperatively and has a real risk of strangulation. Strangulated LSH may be misdiagnosed as strangulated inguinal hernia. Early recognition and timely surgical repair are important to avoid strangulation.

Congenital spigelian hernia (SH) is very rare in the pediatric age group. This is a report of two cases of SH in 1-week and 3-month-old male infants. A review of the literature revealed only 35 cases of SH in children younger than 17 years of age, bringing the total including our 2 cases to 37. There were 25 males and 12 females, a ratio of 2.1:1. Their ages ranged from 6 days to 17 years (mean 4.52 years). The hernia was situated on the right side in 13, the left side in 19, and was bilateral in 4. In one case the side of the hernia was not mentioned. In 29 cases the hernia was spontaneous while in 5 it was caused by trauma. In 3 children the hernia developed postoperatively, in 2 following repair of a congenital diaphragmatic hernia and in 1 following excision of a mediastinal neuroblastoma. Two children presented with a strangulated SH. Eleven of the 35 previously reported children had associated conditions; in 5 there was an ipsilateral undescended testis (UDT). Our two infants with SH also had an ipsilateral UDT. The significance of this association is discussed.

Spigelian hernia is rare at any age, and exceedingly so in childhood. Three cases encountered in a paediatric surgical practice in 16 years are reported. A Spigelian hernia is a ventral interstitial herniation through a defect in the aponeurosis of the transversus abdominis muscle (the Spigelian fascia) adjacent to the rectus abdominis. It presents as a mass, usually tender, in the semilunar line, most commonly at the level of the arcuate line below the umbilicus. Strangulation can occur and repair is advised. The site of the hernia is difficult to recognise under anaesthesia. Pre-operative localisation and marking is advised.

An 11-year-old girl with a Spigelian, i.e., spontaneous lateral ventral hernia, is reported. This rare entity has been previously described in only nine children. Five of them had true Spigelian hernias, in which surgery was the treatment of choice. Four hernias were the consequence of trauma, in which cases surgery could be delayed because spontaneous closure eventually occurred.

A 5-year-old girl with blunt trauma to the abdomen and a 3-month-old boy after abdominal surgery were found to have a spigelian hernia. The pathogenesis of these rare hernias is discussed. Our first case is the third recorded traumatic spigelian hernia occuring in a child, and the second is the first postoperative case described after abdominal repair of a Bochdalek hernia.