The syndrome of Spigelian hernia and cryptorchidism: A review of paediatric literature

doi:10.1016/j.jpedsurg.2014.10.059

Journal of Pediatric Surgery

Volume 50, Issue 2, February 2015, Pages 325-330

https://doi.org/10.1016/j.jpedsurg.2014.10.059 Get rights and content

Abstract

Background/Purpose

The purpose of this study is to present a summary of paediatric Spigelian hernia (SH) reported to date, and discuss possible aetiology of SH associated with ipsilateral ectopic testis (SH-ET).

Methods

Search of PubMed, Medline, Embase, and CINAHL was performed using keywords “Spigelian hernia”. The following were extracted from articles describing paediatric SH: demographics, site and contents of SH, comorbidities, proposed aetiology, presence of ipsilateral inguinal canal (IC) and gubernaculum (G).

Results

There were 78 patients with 88 hernias (69 male, 19 female), including 55 male (19 left, 22 right, 7 bilateral) and 16 female (5 left, 5 right, 3 bilateral) nontraumatic SHs. In nontraumatic male SH, 29 hernias contained testis (10 left, 11 right, 4 bilateral), 15 did not, 10 had no data. Of 29 SH-EH, 15 were lacking IC and G, 3 were missing IC (no G data) and 2 had absent G (no IC data).

The combination of SH and cryptorchidism is increasingly recognised as a distinct syndrome. However, there is controversy as to the pathogenic mechanism of this association. We hypothesise SH-ET develops because the G, and therefore IC and line of descent, become cranially ‘mislocated’ along the mammary line, which overlies the Spigelian fascia.

Conclusion

SH is rare in children. SH-ET may result by testicular descent to an ectopic site along the embryological mammary line.

Section snippets

Material and methods

A search of PubMed, Medline, Embase, and CINAHL databases (1900 to present, January 2014) was conducted using the keyword “Spigelian hernia”. Abstracts, full text articles, conference proceedings, and letters to the editor in English were then reviewed to determine cases of SH occurring in the paediatric population (defined as age < 18 years). The bibliographies of articles describing paediatric SHs were then examined for additional cases that may not have appeared in the original search.

The

Results

A total of 57 English language articles were found containing reference to paediatric SH. Four articles included a total of 5 paediatric patients in adult cases series and did not describe the number, laterality, or other details of the hernias in the paediatric patients. These were excluded from further analysis [12], [13], [14], [15].

The remaining 53 papers described 78 patients (62 male, 16 female) with 88 hernias (69 male, 19 female) (Table 1). Traumatic aetiology for the SH was apparent in

Discussion

Paediatric SH is a rare entity, with only 71 cases of nontraumatic aetiology reported in the English language literature since 1946. Males account for 77% of nontraumatic hernias and 82% of traumatic hernias, and right-sided hernias are the most common.

Traumatic disruption of the Spigelian fascia (iatrogenic or community acquired) is frequently described as a cause of paediatric SH [17], [18], [19], [20], [21], [22], [23], [24], [25], [26], [27], [28], [29], [30], [31], [32]. Whilst this

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Cited by (20)

An unusual presentation of cryptorchidism: A case report of Spigelian-cryptorchidism syndrome
2023, International Journal of Surgery Case Reports
Spigelian hernia is an uncommon hernia presenting as a protrusion of abdominal contents through the spigelian fascia, lateral to the rectus abdominis. In some rare cases, Spigelian hernia can occur alongside cryptorchidism, which forms a recognized syndrome found in male infants with Spigelian hernia. This is a relatively unreported syndrome with very limited literature available regarding it, none of which is reported in Pakistan in adults.
We report a case of a 65-year-old male with right sided obstructed spigelian hernia along with the rare finding of testis in the hernial sac. The patient was successfully managed by transperitoneal primary repair (herniotomy) with orchiectomy. The patient recovered uneventfully and was discharged 5 days after the surgery.
The exact pathophysiology of this syndrome remains unclear. Three theories have been proposed to explain this syndrome, including the primary defect being Spigelian hernia leading to undescended testes (Al-Salem), testicular maldescent preceding the formation of the hernia (Raveenthiran), or the absence of the inguinal canal leading to the development of a rescue canal due to the undescended testes (Rushfeldt et al.).
In this case, the absence of gubernaculum was confirmed suggesting the findings to be consistent with Rushfeldt's theory. The surgical team proceeded with hernial repair and orchiectomy.
In conclusion, Spigelian-Cryptorchidism syndrome is a rare syndrome in adult male, with an unclear pathophysiology.
Management of this condition involves repair of the hernia along with either orchiopexy or orchiectomy, depending upon the risk factors involved.
Testicular descent: A review of a complex, multistaged process to identify potential hidden causes of UDT
2022, Journal of Pediatric Surgery
Citation Excerpt :
Mutations in the Hoxa-10 gene lead to abnormally long gubernacular cords and intra-abdominal testes in mice [24, 25], that are morphologically similar to PMDS in the human. There are also defects in the location of the end of the gubernaculum to the abdominal wall, such as those seen in Spigelian hernia, where the gubernaculum may be attached above the normal site of the inguinal canal, but the external oblique muscle has no gap to form the external inguinal ring, leading to the testis being caught inside the abdominal wall muscles within a blind-ending ectopic “inguinal” canal. [26]. Intra-abdominal testes theoretically also may be secondary to agenesis of the gubernaculum, although clinical cases of this have not been reported. (
What causes normal descent of the testis in a fetus, and what goes wrong with this complex process to cause undescended testes (UDT), or cryptorchidism? Over the last 2 decades, most authors searching for the cause(s) of UDT have looked at the 2 main hormones involved, insulin-like hormone 3 (Insl3) and testosterone (T)/ dihydrotestosterone (DHT), and their known upstream (hypothalamic-pituitary axis) and intracellular ‘downstream’ pathways. Despite these detailed searches, the genetic causes of UDT remain elusive, which suggest the aetiology is multifactorial, and/or we are looking in the wrong place.
In this review we highlight the intricate morphological steps involved in testicular descent, which we propose may contain the currently ‘idiopathic’ causes of UDT. By integrating decades of research, we have underlined many areas that have been overlooked in the search for causes of UDT.
It is quite likely that the common causes of UDT are still hidden in these areas, and we suggest examining these processes is worthwhile in the hope of finding the common genetic anomalies that lead to cryptorchidism. Given the fact that a fibrous barrier preventing descent is often described at orchidopexy, examination of the extracellular matrix enzymes needed to allow gubernacular migration may be a fruitful place to start.
This review of the complex anatomical steps and hormonal regulation of testicular descent highlights many areas of morphology and signalling pathways that have been overlooked in the search for causes of UDT.
Spigelian hernia in children: low versus classical
2018, Journal of Pediatric Surgery
Citation Excerpt :
Spigelian hernia and cryptorchidism association is a well-recognized entity [1–3, 9, 10]. Jones and Hudson found this association in 53% of cases in their extensive review of all available pediatric SH cases [1]. Bilici et al. had found its incidence as high as 60% in their review of cases younger than 15 years from 1950 to 2011 [3].
Pediatric spigelian hernias are very rare. They are often missed or misdiagnosed. A series of cases with spigelian hernia, presented to a tertiary care center are presented here with emphasis on different anatomy of spigelian hernias with cryptorchidism and those without associated cryptorchidism.
Over a period of seven years, nine cases of spigelian hernia presented to our tertiary care center. Male:female ratio was 3:1.There was a preponderance of right sided hernias. Three patients had associated cryptorchidism. One patient had associated lumbar hernia. All three patients with cryptorchidism had low spigelian hernia while others had classical spigelian hernia.
There is a likelihood of anatomical variation in SH associated with UDT and those without UDT. Understanding this anatomy may help in correct scrotal placement of testis.
Prospective Observational.
4.
Testicular ectopia: Why does it happen and what do we do?
2017, Journal of Pediatric Surgery
Citation Excerpt :
If the gubernaculum, or genitoinguinal ligament, is not attached to the correct site of the anterior abdominal wall, ectopic testes in the femoral canal or in a prevascular femoral hernia can be explained by the gubernaculum initially being attached to the future femoral canal or slightly anterior to the femoral vessels. A Spigelian hernia containing an ectopic testis is also explained by cranial displacement of the original gubernacular attachment to the anterior abdominal wall, at the site of the Spigelian triangle rather than the future internal inguinal ring [2]. Rarely there are some ectopic testes that can be explained by absence of a normal attachment of the gubernaculum to the future internal inguinal ring.
Testicular ectopia is rare, but the large range of anatomical locations described in the literature has spawned an abundance of possible theories to explain etiology. However, as the anatomical characteristics of normal testicular descent have only been elucidated recently, many of the theories of testicular ectopia do not incorporate this new perspective. In this study we aimed to determine what was in the literature about ectopic testis since 1980, and then try to explain the different anatomical variants in the light of current knowledge about testicular descent.
A literature search was performed and all articles in English published since 1980 about testicular ectopia using several key words were identified.
A total of 271 articles in English were found, of which 31 addressed the pathophysiology and are the primary focus of this study. Case reports and reviews described perineal ectopia (× 4), transverse testicular ectopia (× 11), and abdominal ectopia (× 2), along with 3 reviews/case reports addressing diagnosis and management. A range of proposed causes were found, including obstructed ‘third inguinal ring’ at neck of scrotum, abnormal CGRP function, aberrant distal gubernacular attachment, mechanical hindrance from retained Müllerian ducts, defective gubernacular formation or disruption of the gubernacular attachment to the testis.
After reviewing the proposed theories, we propose a unifying theory, based on current knowledge of testicular descent, where testicular ectopia can be explained by a) anomalous attachment of the gubernaculum to the anterior abdominal wall during transabdominal descent, or b) aberrant migration of the gubernaculum during the inguinoscrotal phase of testicular descent.
Pediatric Spigelian hernia: A case report and review of the literature
2017, Journal of Pediatric Surgery Case Reports
Citation Excerpt :
In young boys, a clinical “syndrome” has been described with a Spigelian hernia containing an ipsilateral undescended testis. The theorized pathologic mechanism of this condition is the cranial mislocation of the inguinal canal along the mammary line, which overlies the Spigelian fascia [7,8]. Although the patient in this report did not have a hernia containing an undescended testis, a certain index of suspicion should be raised for a pediatric patient with an undescended testis and palpable abdominal mass.
An inguinal hernia with cryptorchidism with a Leydig cell tumor in an elderly man: A case report
2017, International Journal of Surgery Case Reports
Cryptorchidism is common in children but is rare in the elderly. It often presents with a constellation of signs and symptoms similar to routine inguinal hernias. We present the case of an elderly man with cryptorchidism containing a Leydig cell tumor and provide clinical insights.
An-84-year old man was admitted with an incarcerated right lower quadrant hernia. Both testes were absent on palpation of the scrotum. After reduction of the hernia, computed tomography scan revealed a round lesion in the hernia sac, which was suspected to be the ectopic testis. Laparoscopic exploration was performed in combination with an open anterior approach. The hernia orifice was the right internal inguinal ring, and the inguinal canal was obliterated by adhesions because the spermatic cord did not pass through it. The ectopic testis was resected with the hernia sac, and the hernia repaired with a KUGEL™ patch (Bard, USA).
Laparoscopic exploration was useful to delineate the anatomy of this unusual inguinal hernia. The open anterior approach was necessary to dissect the ectopic testis and the hernia sac. Pathological findings revealed tumor cells with clear cytoplasm in the resected testis, diagnosed as a Leydig cell tumor.
The combination of laparoscopic and anterior approaches facilitated the surgical treatment of an unusual inguinal hernia with cryptorchidism. The resected ectopic testis should undergo thorough histopathologic examination.

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Review ArticleThe syndrome of Spigelian hernia and cryptorchidism: A review of paediatric literature

Abstract

Background/Purpose

Methods

Results

Conclusion

Section snippets

Material and methods

Results

Discussion

Am J Surg

J Pediatr Surg

Surg Clin North Am

Am J Surg

Am J Surg

J Pediatr Surg

Am J Surg

Ann Emerg Med

J Pediatr Surg

J Urol

J Pediatr Surg

J Urol

J Pediatr Surg

Lancet

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

Urology

J Pediatr Surg

Lateral ventral (Spigelian) hernias in infants and children

Surgery

Pediatric lateral ventral (Spigelian) hernias

South Med J

Spigelian hernia: surgical anatomy, embryology, and technique of repair

Am Surg

Congenital Spigelian hernia with cryptorchidism: probably a new syndrome

Hernia

The surgical treatment of Spigelian hernia

Surg Gynecol Obstet

Lasts Anatomy Regional and Applied

Ventral hernia due to normal banding of the abdominal muscles

Surg Gynecol Obstet

Spigelian hernia

Acta Chir Scand Suppl

Spigelian hernia and a “redundant” sigmoid colon

Med J Aust

Spigelian hernias: repair and outcome for 81 patients

World J Surg

Incidence and outcome of surgical repair of Spigelian hernia

Br J Surg

Spigelian hernia in childhood

Pediatr Surg Int

Spigelian hernia in children, two cases of unusual etiology

Pediatr Surg Int

Pediatric Spigelian hernia: reports of three cases

Surg Today

Spigelian hernias in infants: report of two cases

Can J Surg

Spigelian hernia in a child

South Med J

Trauma-related acute Spigelian hernia in a child

Pediatr Emerg Care

Review Article
The syndrome of Spigelian hernia and cryptorchidism: A review of paediatric literature