Spigelian hernia and cryptorchidism syndrome: open spigelian hernia repair and laparoscopic one-stage orchiopexy for ectopic testis

Summary

Spigelian hernia and cryptorchidism syndrome in children is increasingly reported in the literature. A variety of phenotypes have been reported, so diagnostic approaches and operative techniques remain poorly defined. The case of an infant male who presented with a left spigelian hernia and ipsilateral cryptorchidism who was initially misdiagnosed with an ectopic inguinal testis is presented. He underwent diagnostic laparoscopy which revealed an ectopic left testis within the spigelian hernia defect with a gubernaculum present. Collaborative decision making between paediatric surgery and paediatric urology resulted in the final operative repair entailing laparoscopic orchiopexy, open left spigelian and inguinal hernia repair, right inguinal hernia repair and umbilical hernia repair. We conclude that a laparoscopic orchiopexy of an ectopic testis within a spigelian hernia is a safe method to address this rare phenomenon, but collaborative multidisciplinary discussion is ideal to weigh operative strategies and management.

Background

Spigelian hernia-cryptorchidism syndrome is a rare but increasingly recognised phenomenon in the literature. Spigelian hernias are classically described to occur through the transversus abdominis aponeurosis at the junction of the linea semilunaris (arcuate line of Douglas) and the lateral border of the rectus abdominis—the so-called spigelian fascia. Although the reported incidence is low, recognition of this condition is important in guiding diagnosis and operative management. The rarity of spigelian hernias in childhood compounded by premature diagnostic closure can lead to misdiagnosis. Additionally, no clear management guidelines exist for treatment. Patients may have differing presentations that do not fit all syndromic criteria, and indeed, the criteria remain vague, leading to varying operative approaches. The authors present this case to share the preoperative and intraoperative decision making to guide future surgeons when confronted with this rare diagnosis.

Case presentation

The patient was an infant full-term male with no significant past medical history who presented with a left ‘groin’ bulge noted at 2 weeks of age. He was born via spontaneous vaginal delivery at full term with no significant abnormalities in his prenatal course. He had no prior surgical history, and family history was notable for a maternal sister who had a bad reaction to nitrous oxide. The patient had a bulge noted in the left lower quadrant by his mother during circumcision by a Jewish mohel. The patient’s mother contacted their primary care physician, and they were told to go to urgent care, where it was reduced spontaneously. One week later the patient had the bulge stick out and get stuck, prompting the patient’s parents to take the patient to the emergency department (ED). An ultrasound was performed which revealed a left inguinal hernia with bowel present and identified the testicle in the inguinal canal, and the ED physician was able to successfully reduce the inguinal hernia. The family was referred to paediatric surgery.

It was in this setting that the patient presented to the senior author (MAE) for paediatric surgical consultation. Exam was notable for an umbilical hernia, a descended right testis with a possible silk glove sign and an undescended left testis with no obvious reducible left groin bulge, but again with a silk glove sign, possible ectopic left thigh testicle and circumcised penis. The surgeon palpated a fold of left thigh skin that was felt to be a possible location for the ectopic testicle but could not feel a testicle in the inguinal canal as noted on the ultrasound obtained in the ED. Importantly, no bulge was noted on the abdominal wall. Given the preponderance of evidence, an undescended inguinal versus ectopic testicle with a left, and likely right, inguinal hernia was diagnosed. However, the measurements of the hernia sac on ultrasound (see below) seemed incongruent with the patient’s presentation during that first visit.

Collaborative decision making with the patient’s parents was then undertaken. The patient’s parents chose not to vaccinate the patient at birth, nor give the patient vitamin K. A respectful conversation was held about the risks of not vaccinating in the setting of surgery in an infant, and the family was very appreciative of the respectful approach to their decisions. A discussion was had regarding obtaining laboratory studies to assess the risk of bleeding prior to surgery as well. The risk of spontaneous haemorrhage appeared to diminish at 6 months of age, so the decision was made jointly between the surgeon and the family to proceed with surgery after 6 months of age during that first visit.

Investigations

The patient had a preoperative ultrasound performed during the ED visit. The ultrasound noted a left inguinal hernia containing fluid and nonobstructive bowel. The fascial defect measured up to 1.3 cm. The hernia sac measured approximately 4.0×1.4×5.3 cm. Vascularity was noted within the herniated bowel on Doppler imaging, and no bowel wall thickening was visible. The hernia was not reducible during the study. The left testicle was visible inferior to the hernia sac within the inguinal canal (figure 1). No mass, fluid collection or adenopathy was seen. This study impacted the initial evaluation of the primary surgeon by introducing an element of premature diagnostic closure. No alternatives to a typical indirect left inguinal hernia with an undescended testicle were considered. The findings on the original exam were supported by the ultrasound, but the findings on the second exam were not, prompting a reconsideration of the differential diagnosis of the patient. The patient had a normal complete blood count including normal platelets, prothrombin time and activated partial thromboplastin time.

Figure 1. Ultrasound images depicting (A) fascial defect with protruding bowel and (B) testicle interpreted as findings present in the inguinal canal.

Differential diagnosis

The original differential diagnosis included possible bilateral inguinal hernias and a left ectopic versus inguinal testis. Four days after the paediatric surgical consultation, the patient’s mother called the surgeon’s office with concern that the left abdominal wall bulge had returned. The consulting surgeon’s partner (SC) evaluated the patient and contacted the primary surgeon (MAE) to discuss the patient’s physical exam findings (figure 2). The surgeons discussed the findings, and the diagnosis of a ventral hernia, or very specifically, a spigelian hernia, was considered. Given the hernia was reducible, a follow-up evaluation was scheduled with the primary surgeon 3 days later. The primary surgeon was initially sceptical of the diagnosis given the extremely low incidence reported in the literature of spigelian hernias in children.¹ However, in preparing for the follow-up, the primary surgeon researched spigelian hernias and discovered reports of spigelian hernia-cryptorchidism syndrome.¹,⁷ The literature suggested a variety of presentations including a missing inguinal canal. On repeat examination, an easily reducible hernia was noted alternatively at the left internal ring and, confusingly, at the arcuate line of Douglas along the rectus sheath border. It was at this time the primary surgeon expanded the differential diagnosis to include spigelian hernia and to consider the location of the ectopic testicle to be elsewhere rather than the thigh or inguinal canal. Given the surgeon’s concern for an increased risk of incarceration, the family agreed to proceed urgently to surgery within 1 week. Paediatric anaesthesia was notified of the patient’s family history, and no special considerations were deemed necessary.

Figure 2. Clinical photograph by patient’s parents depicting a left lower quadrant lateral abdominal wall hernia.

Treatment

The patient underwent an urgent diagnostic laparoscopy with open umbilical hernia repair, laparoscopic left orchiopexy, open ventral hernia repair (combined spigelian hernia repair and left inguinal hernia repair) and open right inguinal hernia repair. On entry into the abdomen, the vas deferens and testicular vessels were noted to be entering a hernial defect in the transversus abdominis aponeurosis between the lateral edge of the rectus sheath and the semilunar line. There was a clear demarcation between the spigelian hernia and adjacent left indirect inguinal hernia by a fascial bridge (figure 3). A complex multidisciplinary discussion ensued between the primary surgeon, two of his partners (MB, LB) and a paediatric urologist (KG) to finalise the operative strategy. After weighing the risks and benefits of one-stage versus two-stage orchiopexy, the decision was made to perform a laparoscopic one-stage orchiopexy. The spigelian hernia sac was explored laparoscopically revealing a left undescended testicle and its associated gubernaculum attached anteriorly to the abdominal wall fascia (figures4 5). The testicle was dissected free from the hernia sac, preserving the vas deferens and testicular vessels. Once the testicle was free from its attachments to the hernia sac, it was apparent that the spigelian hernia and inguinal hernia defects had become a common defect, as the fascial bridge between the two had been obliterated (figure 6), and in fact, the respective hernia sacs had been completely reduced into the abdomen extirpating the inguinal canal (figure 7). This left a common anterior abdominal wall fascial defect comprised of the now combined spigelian and inguinal hernias (figure 8). The left testis was placed through a neo-inguinal ring medial to the obliterated spigelian and inguinal hernia defects (figure 9). The testis was then secured to the dartos layer of the left hemiscrotum. The common ventral hernia was then repaired with monofilament absorbable suture in a simple interrupted fashion, and the right inguinal hernia (figure 10) was repaired in a standard open fashion with high ligation of the hernia sac (Ferguson and Gross herniotomy).

Figure 3. Initial intraoperative finding of two separate hernial openings: a spiegliean hernia and an indirect inguinal hernia, separated by a fascial bridge.

Figure 4. Testicle reduced from the spigelian hernia.

Figure 5. Gubernaculum attached to the fundus of the spigelian hernia sac.

Figure 6. Common wall ventral hernia comprised of reduced spigelian hernia sac and indirect inguinal hernia sac (posterior view).

Figure 7. Common wall ventral hernia demonstrating complete reduction of spigelian hernia sac and indirect inguinal hernia sac (posterior view). Site of neo-internal ring for planned orchiopexy noted.

Figure 8. Common wall ventral hernia demonstrating anterior fascial defect (anterior view).

Figure 9. Completed laparoscopic orchiopexy demonstrating testicular vessels, vas deferens, neo-internal ring and proper orientation of anatomic structures.

Figure 10. Right indirect inguinal hernia.

Outcome and follow-up

The patient remained in the inpatient unit for 1 day postoperatively given the extensive dissection and need for close monitoring for postoperative bleeding. He was discharged home the day after surgery in stable condition. Follow-up occurred in the outpatient surgery clinic at 2 weeks and 6 weeks after surgery. The patient thrived following surgery. No recurrence of the hernias was identified on postoperative examinations, and the bilateral testes remained in descended position (figure 11).

Figure 11. Patient at 6 weeks postop demonstrating healed inguinal incision, ventral abdominal wall incision, umbilical incision and left scrotal incision. The testicle lay nicely in the left scrotum without any tension.

Discussion

Seventy-eight paediatric patients with 88 spigelian hernias have been reported in the literature as of 2015.⁸ Rushfeldt et al named this rare phenomenon ‘spigelian hernia-undescended testes syndrome,’ which involves a spigelian hernia defect, a hernia sac containing the testis, absence of a gubernaculum and absence of the inguinal canal, and Patoulias et al also argued these constellation of findings constitute a syndrome.^{3 9} An ipsilateral inguinal canal was present in this patient, and the gubernaculum was identified attached to the abdominal wall fascia with the testicle located within the spigelian hernial defect. The presentation in the neonatal period indicates a congenital hernia and may suggest a variation of the syndrome.

During development, the gubernaculum classically arises from the inferior poles of the testicles and migrates to the sites of the future scrotum. A peritoneal sac herniates ventral to the gubernaculum that eventually becomes the processus vaginalis. The migration of the testicles into the scrotum occurs along the processus vaginalis, and the testicles are fixed in the scrotum by the gubernaculum due to a differential growth rate between it and the abdominal wall. The proximal portion of the processus vaginalis normally obliterates, and the distal portion becomes attached to the testicle as the tunica vaginalis. When the processus vaginalis remains open, it gives rise to either an inguinal hernia or communicating hydrocele (figure 12). Figure 13 demonstrates the anatomy of the abdominal wall indicating the location of a spigelian hernia in relationship to a normally descended testicle. Although a spigelian hernia is thought to be a congenital weakness at the junction of the semilunar and semicircular lines of the anterior abdominal wall in children, it is not clear why these arise. The error in testicular descent in this case is even more interesting when considered in the context of normal embryology.

Figure 12. Original illustration of variations in testicular anatomy by the first author. (A) Normal testicle with obliterated processus vaginalis. (B) Patent processus vaginalis with communicating hydrocele. (C) Indirect hernia caused by patent processus vaginalis. This hernia travels through both the deep and superficial inguinal rings. (D) Direct hernia caused by weakness in the transversalis fascia. This hernia travels through the superficial ring only and lies medial to the inferior epigastric vessels, a key differentiation between indirect and direct hernias.

Figure 13. Original illustration of spigelian hernia by the first author. The spigelian hernia is identified at the weakness of the fascia located at the semilunar line. The transversus abdominis and external oblique are resected in this illustration.

The diagnosis proved challenging, as the primary surgeon did palpate a silk glove sign, a classic finding associated with an inguinal hernia in an infant (figure 14). On palpation of the skin over the pubic tubercle, the processus vaginalis feels like two pieces of silk being rubbed together by the examiner’s index finger. Furthermore, the ultrasound suggested an inguinal hernia with bowel present and erroneously confirmed the testicle in the inguinal canal. During the first examination, there was no evidence of a ventral hernia. The re-presentation with an abdominal wall hernia led the primary surgeon to research paediatric spigelian hernias and discovered the reports of a possible spigelian hernia-cryptorchidism syndrome in children. The lesson to the surgeon was to always be on guard for premature diagnostic closure and maintain a questioning mindset when examining a patient, even in the setting of such a routine presumed diagnosis. Collaborative, shared decision making with the family was essential to address the risks, benefits and alternatives of surgery.

Figure 14. Original illustration of the silk glove sign by the first author. The hernia sac is palpated by placing a finger lateral to medial over the pubic tubercle. This reproduces the sensation of rubbing two pieces of silk together.

Orchiopexy with spigelian hernia repair has been described using either an open or a laparoscopic technique.^{4 7 10 11} The decision to perform a one-stage orchiopexy involved complex multidisciplinary discussion and decision making. The intraoperative findings were not known prior to the operation, requiring expedient analytical reasoning to ensure safety and effective treatment for the diagnosis. A collaborative discussion among the paediatric surgical team and the paediatric urologist supported a laparoscopic one-stage orchiopexy. Factors in this decision included length of the testicular vessels and vas deferens, tension on the testis, anatomic feasibility of orchiopexy and surgeon experience. The procedure was performed by completely reducing the testicle out of the spigelian hernia sac and mobilising the gubernaculum at the insertion (figure 5). This obliterated the fascial bridge between the spigelian hernia and the inguinal hernia and completely reduced both hernia sacs (extirpating the inguinal canal), creating a common hernial defect between the two (figure 6). A neo-internal ring was created medial to the internal inguinal ring through which the testicle was placed into the scrotum, and an orchiopexy was performed in the left scrotum (figure 7). The operation was completed by repairing the newly created common-wall ventral hernia, right inguinal hernia and umbilical hernia in an open fashion.

Consideration was given on the best approach to repair the common ventral hernia comprised of the original spigelian hernia and left inguinal hernia as well as the right inguinal hernia. The basic tenet of any inguinal hernia repair in an infant or child is high ligation of the hernia sac. Paediatric laparoscopic inguinal hernia repairs are well described, and an excellent summary of the various techniques can be found on the Society of American Gastrointestinal and Endoscopic Surgeons website (https://www.sages.org/wiki/pediatric-laparoscopic-inguinal-hernia-repair-a-review-of-techniques/). Outcomes are generally similar between open and laparoscopic techniques (https://www.facs.org/for-patients/the-day-of-your-surgery/pediatric-inguinal-and-femoral-groin-hernia-repair/) (table 1).

Table 1. Outcomes of management of inguinal and spigelian hernia.

Hernia type	Risk of incarceration	Surgery type	Risk of recurrence	Risk of reoperation	Chronic pain
Inguinal hernia	4%–7%12	Open	0.8%–5%13,15	2.1%–3.3%13,15	Higher incidence of chronic pain
		Laparoscopic	0%–2.1%13,15	2.8%–4.0%13,15	Less incidence of chronic pain
Spigelian hernia	17%–30%16	Open	<1%17	No significant references in current literature available	Higher incidence of pain
		Laparoscopic	<1%17		Less incidence of pain

The creation of the common ventral hernia during the mobilisation of the testicle from the spigelian hernia made a laparoscopic repair via a posterior approach extremely challenging. Infants classically have an abdominal domain that is as wide as it is long. This is one reason a paediatric surgeon will classically make a transverse abdominal incision for a laparotomy. The extirpation of the hernia sac resulted in a weak posterior abdominal wall fascia, and a laparoscopic posterior repair was felt to be too high risk for a recurrence. The decision to repair the hernia anteriorly via an open approach ultimately had an excellent result. At that point, the decision to repair the contralateral inguinal hernia in an open fashion was determined by the comfort level and expertise of the operating surgeon.

The case of this patient demonstrates a variant to the spigelian hernia-undescended testis syndrome. Surgeons must maintain intellectual curiosity even when evaluating commonplace diagnoses and guard against premature diagnostic closure. When encountering this rare syndrome, multidisciplinary discussion is essential in choosing an effective operative strategy and should be considered early in the operation if anatomic identification is challenging. Laparoscopic one-stage orchiopexy is a safe and feasible operation for patients with similar intraoperative findings. By investigating operative approaches prior to proceeding, the surgeon can obtain peace of mind as they treat this rare phenomenon.

Patient’s perspective.

The family politely declined the invitation to provide a perspective; however, they were intimately involved in the review of the manuscript.

Learning points.

• A spigelian hernia may be present in children presenting with cryptorchidism and should be evaluated during physical examination, especially in the setting of a suspected inguinal hernia.

• Guard against premature diagnostic closure, even when evaluating common diagnoses.

• Laparoscopic one-stage orchiopexy can be safely used for paediatric spigelian hernia and ectopic testis.

• Intraoperative multidisciplinary discussion and shared decision making are prudent to ensure safety and efficacy of the operative approach.