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Congenital Spigelian hernia-cryptorchidism syndrome: two cases report and review of literature

Mohamed Shahin, Gamal Al-Saied, Medhat Ibrahim, Ali Al-Zhrani, Mohamed Al-Saeed, Tharwat Hussien, Amin Alansary

Departments of Pediatric Surgery, Al-Azhar University and King Abdul Aziz Specialist Hospital, Taif, Kingdom of Saudi Arabia


Correspondence

Gamal Al-Saied
Housing area of King Abdul Aziz Specialist Hospital,
Block 3F4, Flat,103, P.O.Box:10127,
Taif, Saudi Arabia
Mobile: 0096653919449
Fax: 0096627310801
Email: This email address is being protected from spambots. You need JavaScript enabled to view it.


Abstract

Congenital Spigelian hernia (SH) is a very rare condition in children. Only a few reports on this condition, in combination with ipsilateral cryptorchidism and testis in the hernia sac, have been published. It has been suggested that the coexistence of Spigelian hernia and ipsilateral cryptorchidism is part of a new syndrome. We report on two cases of SH in 8 month and 2 day-old male infants. Both cases had Spigelian hernia containing the ipsilateral testis, without a gubernaculum. The first case had no other congenital anomalies, while the second one was associated with multiple congenital anomalies. The two cases are discussed in the light of published literature.

Key-words: Spigelian hernia, cryptorchidism, testis, gubernaculums, inguinal canal

 

Introduction

Spigelian hernia is a lateral ventral hernia that occurs in the Spigelian fascia lateral to the junction of the arcuate and semilunar lines formed by the fibrous union of the rectus sheath and the anterior abdominal wall muscles and below the level of the umbilicus. The sac of SH is usually containing small bowel segments, omental fat or both. Spigelian hernias are rare in adults and exceedingly rare in children. A few case reports describing (SH) in the paediatric population have been published and an association with ipsilateral cryptorchidism has been very rarely reported [1, 2].

Some authors suggested that a defect in the Spigelian fascia, combined with ipsilateral cryptorchidism, may be part of a new syndrome [3]. We report here two cases of SH in 8-month and 2 day-old male infants. Both cases had Spigelian hernia containing the ipsilateral testis, with no gubernaculums attachment or inguinal canal. The etiopathogenesis, surgical anatomy, diagnostic methods, and treatment for this rare condition are discussed with a review of the literature.

Case I:

An 8 month-old full-term boy was presented in outpatient clinic with a huge swelling occupying left lumbar and iliac regions of the abdomen with undeveloped left hemiscrotum dating shortly after birth and increasing gradually with time. It increased in size with crying and decreased on sleeping. On examination; the boy was generally good, vitally stable, and the swelling was reducible in downward and medial direction and non tender. The left testis was impalpable neither in the left hemiscrotum nor in the left inguinal region. An ultrasound showed a hernia sac containing a normal sized left testis and a loop of small intestine with good peristaltic movements and protruded through a 12 mm wide hernia opening and located between the external oblique and internal oblique muscles (fig. 1a).

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Figure 1a: An 8 month-old boy with a bulge in the left lower quadrant of the abdominal wall (Spigelian hernia).

He underwent elective hernia repair under general anesthesia. Following reduction of hernia sac and left lower abdominal crease incision, the sac was dissected off the position under the external oblique muscle with its port close to the lateral edge of the rectus muscle. The sac was opened and contained a normal sized left testis connected directly to the wall of the hernia via its mesorchium without gubernacular cord attachment to the lower pole of the testis (fig. 1b).

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Figure 1b: A left lower abdominal crease incision. The spigelian hernia sac containing a normal sized left testis without gubernacular attachment

The long hernia sac was dissected from the testis and its spermatic cord. After transfixation and excision of hernia sac, closure of fascial defect were performed around the cord using Vicryl 3-0 suture. Then, the testis, with a sufficient long spermatic cord, was tunneled subcutaneously and fixed into the ipsilateral subdartos pouch (fig. 1c). The child was followed up for 6 months and no complications occurred.

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Figure 1c: Hernia sac is transfixed and excised. Then, the testis, with a sufficient long spermatic cord, is tunneled subcutaneously and fixed into the ipsilateral subdartos pouch

Case II:

A 2 day-old preterm boy, 1.5 kg was admitted to our neonatal intensive care unit for evaluation and management of a huge bulge in the left side of the anterior abdominal wall associated with other gross multiple congenital anomalies. The neonate was born to a Gravida 2, Para 2 mother via cesarean section. On examination: he was vitally stable, crying and pink on room air. There was a huge swelling occupying the left lower lumbar and iliac regions of the anterior abdominal wall. The swelling was partially compressible and non tender associated with ipsilateral cryptorchidism and other gross multiple congenital anomalies (cleft lip and palate, dextrocardia, high imperforate anus, and absent left hip bone and femur, and absent toes) (fig. 2a). Lab work-up was within normal limits.

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Figure 2 a: A 2 day-old preterm boy with a bulge in the left lower quadrant of the abdominal wall (Spigelian hernia) associated with ipsilateral cryptorchidism and other gross multiple congenital anomalies

In supine position a left lower abdominal crease incision was performed under general anesthesia. The sac was dissected off the position under the external oblique muscle and opened. The sac contained a normal sized left testis connected directly to the wall of the hernia via its mesorchium without gubernacular cord attachment to the lower pole of the testis as well as descending and sigmoid colon (fig.2b). Diverting colostomy was performed through the Spigelian defect as an emergency procedure and the left testis was left in the superficial inguinal pouch. We preferred to postpone the classical orchidopexy to 1 year of age to avoid the possible catastrophic ischemic problems during the orchidopexy in this young age group.

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Figure 2 b: A normal size left testis (arrow) is connected directly to the wall of the hernia sac. The descending and sigmoid colon are seen inside the hernia sac as well.

Discussion

Spigelian hernia occurs as result of weakness in the Spigelian hernia belt arising from a transversus abdominis aponeurosis between the lateral edge of the rectus sheath and the semilunar line [4]. The usual content of the hernial sac are pre-peritoneal fat, small bowel, colon or omentum [5]. Most Spigelian hernias occur cranially and laterally to the epigastric inferior vessels, but with occasional medial and caudal location they are named “low Spigelian hernias” [4]. Whereas adult Spigelian hernias are considered to be acquired because of trauma or increased intra-abdominal pressure, pediatric cases are suspected to be congenital [6, 7]. Spigelian hernias are rare in adults and exceedingly rare in children. Up to 15% of these are bilateral in nature, and there is no gender predilection [5]. Among male infants with Spigelian hernias, 75% are associated with cryptorchidism [6]. Raveenthiran suggested that a defect in the Spigelian fascia, combined with ipsilateral cryptorchidism, may be part of a new syndrome [3]. In our two cases we encountered a well formed testis with sufficient length of spermatic cord within the hernia sac of spigelian hernia with empty ipsilateral hemiscrotum. Most Spigelian hernias associated with a cryptorchidism are recognized at birth or shortly indicating a congenital condition [2]. In our patients, the first case is recognized at the eighth months of age in outpatient clinic, while the second one is diagnosed immediately after birth in Neonatal Intensive Care Unit.

In 2010, Rushfeldt [2], presented a comprehensive overview of pediatric patients with Spigelian cryptorchidism syndrome reported in the English language literature. He mentioned that about 75% of male infants with Spigelian hernia was associated with ipsilateral cryptorchidism and in 87% of these patients, the testis was 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. Furthermore, he should be prepared to perform an orchidopexy via a subcutaneous route or subaponeurotic route with an artificial external ring since an inguinal canal may very well not be found [2]. In our patients, both testicles were found inside the hernia sacs.

No gubernaculum was identified during the operation, in 8 out of the 15 patients reported by Durham and Ricketts [6]. Similarly, we did not identify gubernaculum in our patients.

Other surgeons have reported a concomitant ipsilateral lack of both the gubernaculum and inguinal canal in 6 patients with 7 hernias and the lack of an inguinal canal, with no information on the gubernaculum in 2 additional patients. It is evident from the embryology that the gubernaculum is a prerequisite for the development of an inguinal canal [8-10]. There was no ipsilateral inguinal canal in our two patients.

We and others [2] noted that the spermatic cord was of sufficient length to bring the testes into the scrotum. The testis was dissected from the hernia sac and tunneled subcutaneously into the ipsilateral subdartos pouch. The hernia sac was then resected, and the opening was closed around the cord. Another approach, the testes could be brought down transabdominally and a new ring, where an external ring would normally be located, was created medial to the inferior epigastric vessels and just lateral to the pubic tubercle [6].

Surgical repair of congenital SH may be performed in the early period and orchidopexy could be postponed to 1 year of age. In the meantime, the risk of incarceration is decreased to a limited degree. The testis and spermatic cord are enlarged, and the infant’s immune response is getting stronger. After a year, the abdominal testis may be anchored by the Fowler-Stephens procedure or another surgical technique. The other speculated therapy is to wait until the end of the first year of life before performing both orchidopexy and surgical repair of congenital SH and conducting a strict follow-up because of the extremely rare risk of incarceration [11].

In 2005, Raveenthiran [3] suggested that ectopic location of the testis is the primary abnormality, and it leads to formation of Spigelian hernia by dragging a peritoneal sac along with it.

In 2007, Kumar and Singal support the mechanical theory accounts for the presence of the testis in the sac of Spigelian hernia rather than the testis causing the hernia [5].

Raveenthiran’s theory [3] is criticized by Rushfeldt et al. [2]. They mentioned that the most of testes were found inside the hernia sac which does not support the Raveenthiran’s theory of an ectopic processus vaginalis. In addition, Raveenthiran’s hypothesis that the testis drags down a processus vaginalis analogous to a normal scrotal descent is not in accordance with general embryologic knowledge [8, 10-12]. In 2010, Rushfeldt et al. attributed the occurrence of cryptorchidism, as a first step in the Spigeliancryptorchidism syndrome, to a failure in the development of a gubernaculum. As a consequence, the inguinal canal will not develop and the testis will not be able to descend from their intra-abdominal position to the scrotum. But they did not mention the causes of developmental failure of the gubernaculums. Also, they failed to explain the coexistence of Spigelian hernia with ipsilateral cryptorchidism. Is it due to a congenital ipsilateral simultaneous defect in the Spigelian fascia or may be due to the arrest of the testis that induces a sort of rescue canal through a weak area in the abdominal wall in the absence of an inguinal canal [2]?

In 2012, Inan et al. [14] support the idea that SH occurs secondary to an ectopically descended testis, as reported by Raveenthiran [3]. We think that the exact mechanism behind this syndrome remains unknown and more cases are needed to be investigated at the level of genes.

Conclusion

Congenital Spigelian hernia containing testis is a very rare entity in infants. This association may be a distinct clinical syndrome. It includes four congenital, ipsilateral components: a defect in the Spigelian fascia, a hernia sac containing undescended testis, absence of gubernaculum and inguinal canal. Other multiple congenital anomalies may associate the syndrome. The exact mechanism behind this syndrome remains unknown and more cases are needed to be studied.

 

 

 

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