Congenital Spigelian Hernia in a Two-week-old Neonate
Spigelian hernia (SH), also called lateral ventral hernia, seen in neonates is one of the rarest types of abdominal hernias. It occurs through slit-like defects in the anterior abdominal wall adjacent to the semilunar line, the lateral line which joins the ninth rib to the pubic tubercle and protrudes between the muscular and aponeurotic portion of transversus abdominis muscle. The fibrous neck of the defect is more often narrow and prone for incarceration and intestinal obstruction. Multiple unilateral SH are more rare. In this article we present a case of a two-week-old neonate who presented with multiple Spigelian herniae on the left side since birth.
Spigelian hernia (SH) is a lateral ventral hernia that occurs through a slit-like defect in the anterior abdominal wall adjacent to the semilunar line. SH is rare in adults accounting for 1.5% of the abdominal herniae and exceedingly rare in children. In the review of literature, 78 cases with SH were reported in the pediatric age group from 1935 to 2015. With infrequent reports in neonates, a high index of suspicion is needed for prompt diagnosis especially when the interparietal hernia is small.
A two-week-old female neonate born by full term normal delivery and an unremarkable antenatal period, and weighing 3.5 kilograms at birth, presented with a left lateral abdominal swelling.
On examination, the swelling measured 8 x 10 centimetres (cm) in the left lumbar region, lateral to the left rectus abdominis and was more prominent on crying. There was an associated smaller swelling subcostally in the mid clavicular line on the same side measuring 2 x 3 cm with an impulse on crying (Figure 1).
Ultrasonography of the swelling revealed a defect in the anterior abdominal wall lateral to the rectus muscle with herniation of preperitoneal fat and bowel loops through it. Both the sites of herniae were marked prior to induction of anaesthesia (Figure 2), patient was operated under caudal anaesthesia with a left transverse crease incision made over the hernial sac at the umbilical level.
After the division of skin and subcutaneous tissue, the hernial sac was identified protruding out from a defect lateral to the left border of the rectus abdominis. The external oblique muscle was thinned out and covered the sac whereas the hernial sac was protruding through the internal oblique and transversus abdominis defect (Figure 3).
The contents were identified as preperitoneal fat and the bowel loops were reduced without opening the sac. The defect was repaired extra peritoneally by double breasting with 3 ‘0’ Vikryl sutures (Figure 4).
The rest of the layers were closed without any drain. The superior defect was repaired by a similar method. Postoperatively, oral feeding was started after 3 hours and the patient was discharged after a day. Upon follow up one year later the child is doing well with no recurrence.
Though Adriaan van der Spieghel described the semilunar line (Linea Spigeli) in 1645, it was Josef Klinkosch, in 1764, who first defined Spigelian hernia as a defect in the semilunar line. Scopinaro was the first to report a lateral ventral hernia or Spigelian hernia in a pediatric patient in 1935. SH account for 1.5% of abdominal herniae in adults, however there have been sparse case reports of the same in the pediatric age group and even rarer instances in neonates where the aetiology is mostly congenital. Only 78 cases have been reported in the pediatric age group in a review of the literature from 1935 to 2015.
Embryologically, Spigelian hernias may represent outcome of weak areas in the continuation of aponeurosis of layered abdominal muscles as they develop separately in the mesenchyme of the somatopleura, originating from the invading and fusing myotomes. Mostly, these hernias lie in the “Spigelian hernia belt,” a transverse 6-cm-wide zone above the interspinal plane. A congenital abnormality in the development of the abdominal wall secondary to a structural change of the internal oblique and transversus abdominis muscles, neurovascular openings in the fascia, infiltration of muscles layers with fat and muscle palsy has been hypothesized. Lateral ventral (Spigelian) hernias may not be recognized if the physician is unaware that they can occur in pediatric patients.
Diagnosis depends on finding a protrusion in the Spigelian fascia lateral to the rectus sheath at the junction of the arcuate and semilunar lines. Ultrasound is an indispensable diagnostic tool in Spigelian hernias. Ultrasonography is considered to be the gold standard for a Spigelian hernia.
SH in children differs from that in adults: 1) It commonly occurs on the left side 2) More common in males 3) aetiology is mostly congenital 4) In male children it may be associated with undescended testis. The combination of SH and cryptorchidism is increasingly recognised as a distinct syndrome.
Surgical repair involves a layered overlapping closure using interrupted sutures. Graivier et al emphasized the need for preoperative marking of the neck of the hernia defect as it is extremely difficult to outline the same in a relaxed patient.[3,5] Traditionally, repair consists of open anterior herniorrhaphy, using direct muscle approximation, with a mesh and prostheses. We used double breasting of the muscles with interrupted sutures as the defect in this neonate was very wide, closure was easily possible, and a mesh was not necessary. Laparoscopy, preferably a totally extraperitoneal procedure, or intraperitoneal when other surgical repairs are planned within the same procedure, is currently employed as an adjunct to diagnosis and treatment of Spigelian hernias. Care must be taken not to create iatrogenic Spigelian hernias when using laparoscopy trocars or classic drains in the Spigelian aponeurosis. In a large defect such as this case, we postulate that direct closure by double breasting preferably without opening the peritoneum is the ideal treatment of SH.
Being interparietal hernias the diagnosis requires a high level of clinical suspicion. Usually the lesion is single. Open extraperitoneal repairs done in this case, is the treatment of choice in large Spigelian hernias without any recurrence during the follow up for a year.
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- Jones BC, Hutson JM. The syndrome of Spigelian hernia and cryptorchidism: a review of paediatric literature. J Pediatr Surg. 2015; 50(2):325-30.
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- Al-Salem AH. Congenital Spigelian hernia and cryptorchidism: cause or coincidence? Pediatr Surg Int. 2000; 16(5-6):433-6.
- Vega Y, Zequeira J, Delgado A, Lugo-Vicente H. Spigelian hernia in children: case report and literature review. Bol Asoc Med P R. 2010; 102(4):62-4.