Spigelian hernia in children: A systematic review

Abstract

BACKGROUND

Spigelian hernia (SH), a protrusion of intra-abdominal contents through a defect in the semilunar line (Spigelian fascia) of the abdominal wall, is extremely rare in the pediatric population. Fewer than 100 cases of pediatric SH have been reported in the literature since the first description in 1939. Pediatric SH is often congenital and may present with non-specific symptoms, making diagnosis challenging. Notably, about one-quarter to one-third of reported pediatric cases are associated with ipsilateral undescended testis, an association sometimes termed the "Spigelian-cryptorchidism syndrome".

AIM

To systematically review all reported cases of Spigelian hernia in children and identify its diagnostic and surgical features.

METHODS

A comprehensive literature search was performed (1939 through 2023) using PubMed and other databases for all publications on Spigelian (semilunar line) hernias in children. Both English and non-English articles were included. Case reports, case series, and relevant reviews were analyzed. Data extracted included patient demographics, hernia side/location, clinical features, imaging and intraoperative findings, coexisting conditions (particularly cryptorchidism), management (open vs laparoscopic repair), and outcomes.

RESULTS

A total of approximately 90 pediatric SH cases from 44 publications were identified. The median age at presentation was around 3-4 years (range: Neonate to 17 years). Slight male predominance was observed, although SH also occurs in females. About 30% of cases involved an ipsilateral undescended testis within the hernia sac or in the Spigelian region. Rare associations with other anomalies were noted, including concurrent inguinal hernias, umbilical hernia, and even complex syndromic presentations (for example, limb defects, neuroblastoma) in a few cases. Clinical presentation often included a reducible lump or intermittent bulge along the lateral aspect of the lower abdomen, sometimes misdiagnosed as an inguinal hernia. Recurrent localized abdominal pain was a frequent symptom, and some infants presented with acute intestinal obstruction from incarcerated SH. Diagnosis was most commonly established by ultrasound, which is considered the gold standard imaging modality for SH in children due to its ability to detect fascial defects and herniated tissue dynamically. A subset of cases (approximately 10%) were attributed to blunt abdominal trauma (for example, bicycle handlebar injury), causing an acquired SH. Management: All reported pediatric SHs were treated surgically, given the high risk of incarceration. Open hernia repair through a small transverse incision over the defect was the traditional approach, and no recurrences have been reported after adequate fascial closure. Over the last decade, laparoscopic repair has become the method of choice, especially for cases combined with an undescended testis, allowing simultaneous orchiopexy. Laparoscopic techniques have been successful in safely reducing the hernia and closing the defect, with excellent outcomes and minimal morbidity.

CONCLUSION

Spigelian hernia in children is a rare but clinically important entity that should be considered in cases of unexplained recurrent abdominal pain, lateral abdominal wall swelling, or cryptorchidism. Early recognition using high-resolution ultrasound and prompt surgical intervention are critical to prevent life-threatening incarceration. Congenital defect of the Spigelian fascia underlies most pediatric cases, and the frequent association with ipsilateral undescended testis suggests a developmental interplay. Surgical repair has excellent outcomes. Increased awareness of pediatric SH among clinicians is necessary to ensure timely diagnosis and treatment, thereby avoiding complications and improving patient outcomes.

Keywords: Spigelian hernia; Children; Laparoscopic hernia repair; Spigelian hernia - undescended testis syndrome; Traumatic hernia; Ultrasound signs of spigelian hernia

Core Tip: Spigelian hernia in children is an exceptionally rare and often misdiagnosed condition due to its transient nature and non-specific clinical presentation. This review provides the most comprehensive analysis to date, incorporating historical perspectives, anatomical insights, and modern diagnostic and surgical approaches. Ultrasound remains the primary diagnostic tool, while laparoscopy is emerging as an effective surgical option, particularly in cases with localization challenges. Increased awareness among pediatric surgeons and radiologists can enhance early detection, reduce complications, and improve patient outcomes.