An atypical lateral hernia and concomitant inguinal and umbilical hernias in a patient with polycystic kidney disease and an intracranial aneurysm – a combined approach of clinical and radiological investigation, endoscopic hernia repair, and anatomical cadaver model documentation and a systematic review of the literature
© Veréb-Amolini; licensee Springer. 2015
Received: 22 August 2014
Accepted: 27 January 2015
Published: 14 February 2015
Atypical hernias are difficult to diagnose due to their rarity and often unspecific symptoms. In the literature there exist hints to peri-inguinal hernias, i.e. direct lateral hernia, but most of them are forms of Spigelian hernias. Since the majority were described during the first half of the past century or even earlier, only very few cases have been documented using modern diagnostic techniques. We report a unique case of a 51 year old patient presenting with an atypical inguinal hernia with concomitant inguinal and umbilical hernias in combination with cystic kidney disease and intracranial aneurysm. The atypical position of the hernia was assumed from clinical inspection, ultrasound and CT scan and verified during pre-peritoneoscopy. Using an anatomical cadaver dissection approach, we followed the unusual position of the hernia through the abdominal wall below the aponeurosis of the external oblique muscle. After a thorough literature search, we assume that the present hernia containing a hernial sac has not been documented before, especially not in such a multidisciplinary approach comprising radiological, surgical and anatomical localisation and endoscopic treatment in a patient with a clinical situation being aggravated by large cystic kidneys leading to dialysis-dependency. Rare hernias have been described as being often associated with concomitant inguinal or other hernias, a predisposition for the male gender and a pathogenic mechanism related to other soft tissue defects such as cystic kidney disease or cranial aneurysm. Thus, we consider this a unique case that has not been documented in this constellation previously, which may increase the awareness for these rare hernias.
Summary of the reported hernias from the literature
Description of intraoperativ finding
Classification by author
Classification by Gallese
Charles Greene Cumston
9 year old girl
Origin in the internal ring but aside in abnormal position containing 3 small lipomas, plus a direct hernia
45 year old woman
8 cm above and lateral to the internal ring containing appendix which was removed
Lateral ventral Hernia
50 year old man
interstitial between transveralis and internal oblique muscle
Lateral ventral Hernia
William E.Lower, N.Fred Hicken
54 year old man
Opening just above the internal inguinal ring (containing omentum), no communication with the inguinal canal, no inguinal hernia demonstrable
Parainguinal interstitial Hernia
William E.Lower, N.Fred Hicken
41 year old woman
Orifice just lateral and above the internal inguinal ring, Lig. Rotundum through the internal ring
Parainguinal interstitial Hernia
John Grierson, Aubrey Leacock
59 year old woman
Neck about one inch above the internal ring no inguinal hernia
John Grierson, Aubrey Leacock
35 year old man
Direct hernia and 2nd orifice 1 inch above the internal ring (lliohypogastric nerve)
Sten Ulbak Jørgen Ørnsholt
36 year old man
Internal Aperture in the inguinal canal, Fundus lateral and above the internal ring containing sigmoid loop
maschio 55 anni
Hernia inguinale diretta, obliqua esterna, voluminoso lipoma preerniario e piccola hernia periinguinale
Hernia Periinguinale (adiposo)
maschio 50 anni
Hernia inguinale diretta e difetto circulare del muscoli piccolo obliquo e trasverso
Hernia Periinguinale (francia epiploica)
Giuseppe Cavallaro et al.
maschio 79 anni
ansa ileale al di sopra del canale inguinale
Takahide Yokohama et al.
81 old woman
Bilateral femoral, left indirect and right direct hernia and a hernia with an orifice lateral and cranial ot the internal ring
Lateral ventral Hernia: preaperitoneal fat with concomittant (right illiac) vessel
51 year old man
Atypical Hernia laterally to the non enlarged inner inguinal ring and direct hernia, recurrent combined hernia (direct hernia and praeperitoneal Lipoma) on the opposite side plus umbilical hernia
A 51 year old patient with dialysis-dependent cystic kidney disease was presented by the nephrology unit to the emergency unit of our hospital due to his primarily non-repositionable umbilical hernia. In his history, a bleeding aneurysm of the anterior communicating artery had been clipped, three years ago.
Clinical investigation revealed an umbilical hernia of 1 cm × 1 cm in size, which could be repositioned without difficulty. In addition, with the patient in an upright position, on the left side, a discrete tumour was detected of 3 cm × 3 cm in size with protrusion into the inguinal canal and with an enlargement of the superficial inguinal ring to 1.5 cm. Several decades ago the patient had a surgical repair of an inguinal hernia at this site. Clinically, we also found a protrusion of 7.5 cm × 7.5 cm in size on the right side, however, not presenting as typical inguinal hernia. Written informed consent for publication of this case was obtained from the patient whom the authors herewith gratefully acknowledge.
Native CT scan (multislice-helical CT, 0.6 mm collimation, axial and coronal reconstructions, 2 mm slice thickness) confirmed a hernial sac containing adipose tissue and small intestine without signs of complications. The hernial sac was localised directly lateral-dorsal to the oblique and transversus abdominalis muscles and the inguinal ligament and cranial to the spermatic cord. The hernial sac protruded in a latero-cranial direction below the aponeurosis of the external oblique muscle. Distinct to this, indirect lipoma (left) and direct hernias were discriminated coated by transversal fascia. As a secondary finding, enlarged polycystic kidneys of 25 cm and 27 cm, respectively, were visible (Figure 1b,c).
Endoscopic hernioplasty and postoperative follow-up
In order to track the interstitial localisation of the hernia, we reconstructed the intraoperative situation by a cadaver dissection (Figure 2b) in a routinely formalin-fixed male corpse obtained from the institutional body donation programme (http://www.anatom.uzh.ch/Bodydonation.html) using anatomical landmarks, e.g. according to Netter (2010). The institutional body donation program follows the ethical guidelines “On the use of cadavers and parts of cadavers in medical research and for pre-, postgrad and continued education” (2008) and “Research with human subjects” (2010) by the Swiss Academy of Medical Sciences. The study was conducted in the year 2013.
A literature search using PubMed was performed for the key words parainguinal hernia, lateral ventral hernia, Spigelian hernia, Spieghel hernia and interstitial hernia complemented by a search for polycystic kidney disease, ADPKD, cranial aneurysm and hernia. We further searched the reference lists of defined articles for further publications. Additionally, hernia surgery and anatomy books were compared for the respective landmarks. The cases most similar to our case were collected and reviewed for a systematic nomenclature.
whether endoscopic treatment would be possible with respect to the large cystic kidneys,
whether the diverse and atypical hernias could be treated endoscopically
what would be the distinct origin and localisation of the atypical hernia, in particular the inner hernia entry point.
Endoscopic treatment was successfully performed in a single session. Since the atypical hernia does not fit into the common pattern of inguinal hernias, we at first considered whether this was a so-called low Spigelian hernia. As described above, the respective hernias were situated along the semilunar line defined as the lateral edge of the rectus abdominalis muscle. Thus, Spigelian hernias are located laterally to the rectus abdominalis muscle aponeurosis and, in particular, below the arcuate (semi-circular) line of Douglas, mainly within the “spigelian hernia belt”, a transverse 6-cm-wide zone above the interspinal plane (Skandalakis et al. 2006). Typically, they are localised above the triangle of Hesselbach, whereby the so-called low Spigelian hernias are sometimes also located within that triangle. Thus, discrimination between these Spigelian hernias and direct hernias is not possible. Peri-inguinal and para-inguinal hernias are classified as lateral ventral hernias (Gallese 1991), which need to be discriminated from Spigelian hernias as well as from direct medial hernias. According to the definition by Gallese (1991), both peri-inguinal and para-inguinal hernias originate directly adjacent to the inguinal canal, however, below the semilunar line and remain below the aponeurosis of the external oblique muscle. Whereby, the para-inguinal hernias penetrate into the inguinal canal, while the peri-inguinal hernias cannot be followed into the inguinal canal.
In our case, the hernia was located laterally to the inferior epigastric vessels, even lateral to the inner inguinal ring, and ended in the abdominal wall above the inguinal ligament. Thus it can be classified among the lateral abdominal wall hernias according to the actual classification for primary abdominal wall hernias, where 2 types of midline hernias (epigastric and umbilical) and 2 types of lateral hernias (Spigelian and lumbar) are defined (Muysoms et al. 2009). According to this classification, we classify the present hernia among the L3 hernias with a size smaller than 4 cm as W1 hernia.
According to La Chausse (1756), a ventral hernia includes any hernia except a femoral, inguinal or umbilical, amongst which he also included para-inguinal, medial inguinal, supravesical and one reported hernia at the semilunar line (for references see: Skandalakis et al. 2006). Molliere (1877) stated that ventral hernias nearly always occur in the linea alba or just outside it or in the semilunar line of Spiegel, and on a level with a line from the anterior superior iliac spine to the umbilicus as outlined by Holloway (1922). He suggested to define a spontaneous ventral hernia as one that appears at an abnormal opening in the abdominal wall, apparently without explicable reason, but usually presenting in or near the linea alba, or the semilunar line of Spiegel. Whether our present case corresponds to the lateral abdominal hernia of Graser (1913) who defined a ventral hernia outside the Spigelian line as a “lateraler Bauchbruch” and attributed their pathogenesis to defects in muscle constitution (for reference see: Holloway 1922), is not easy to define due to the lack of radiological imaging in that work. Among the several hernias collected by Holloway (1922), a hernia described by Steimker (1912) in a male with several concomitant hernias might best correspond to our finding. Steimker’s hernia was observed 6 cm horizontally and medially from the left anterior superior spine with a deep hernial sac protruding into the muscles of the abdominal wall.
Another description was suggested by Grierson and Leacock (1949), who presented 2 cases of para-inguinal hernias whereby they defined them as originating near the inguinal canal, but without entering into it. In both cases, the origin was 1 inch, i.e. approximately 2.5 cm, above the inner inguinal ring with the hernial sac penetrating below the aponeurosis of the external oblique muscle towards the superior anterior iliac spine. A para-inguinal hernia was described by Ulbak and Ørnsholt (1983) as an atypical Spiegelian variant with preoperative radiological verification of a herniation into the abdominal wall using a barium enema. Intraoperatively, a hernia was found with the fundus in a similar position to the present one, namely above and lateral to the internal inguinal ring, but a loop of the spermatic cord was found to adhere to the hernial sac, which penetrated the abdominal wall through the internal aperture of the inguinal canal, which is clearly different from our case. Among the 2 cases reported by Lower and Hicken (1931), one hernia lying between the 2 oblique muscles was found in direct apposition to the lateral walls of the inguinal canal, piercing the internal oblique and transversus abdominalis muscles and the transversal fascia and opening into the peritoneal cavity by its own orifice lateral to and above the inner inguinal ring. According to the nomenclature by Kröhnlein, this would be termed a para-inguinal interstitial hernia to be differentiated from pro-peritoneal and superficial hernia (for references see: Lower and Hicken 1931). Cavallaro et al. (2007) reported another hernia superior to the inguinal region in a male patient that penetrated between the transversus abdominalis and internal oblique muscles. The preferred presence of interstitial hernias of the interparietal type in males and concomitant with other hernias is in agreement with early descriptions published in 1905 by Greene Cumston in the discussion of potential developmental predispositions.
What was already suspected at that time has been confirmed in the meantime. Indeed, congenital malformations such as cystic kidneys and intracranial aneurysms have been found to be more frequently present in patients with hernias, and defects of the extracellular matrix have been recognized to be involved in the biology of hernia formation (Morris-Stiff et al. 1997; Lynen Jansen et al. 2004).
Significantly greater numbers of inguinal, incisional, and paraumbilical hernias were found in autosomal dominant polycystic kidney disease ADPKD (Morris-Stiff et al. 1997). Our present case is further characterised by a history of bleeding intracranial aneurysm and kidney transplantation. Asymptomatic intracranial aneurysms were found by screening in 8% of ADPKD patients, a prevalence two to three times higher than that in the general population (Pirson et al. 2002; for further reading: Kanaan et al. 2014). Vascular abnormalities in ADPKD such as intracranial aneurysms are probably linked to mutations in PKD1 or PKD2 (Bichet et al. 2006), an integral membrane protein involved in cell-cell and cell-matrix interactions.
The so-called lateral ventral inguinal hernias are not clearly differentiated so far due to their high variability and rarity. Furthermore, they are very sparsely documented, not alone because most of the cases described in the literature date from the first half of the last century. Thus, para-inguinal and peri-inguinal hernias are not clearly defined and mainly lack a clear radiological and photographical documentation. From the case presented here with a combined radiological, surgical/endoscopic and anatomical approach, we propose to term the peri-inguinal hernias as lateral direct hernias including also intraparietal, but not Spigelian hernias. Only via an endoscopic approach can the inner origin of the hernial sac be distinctly defined and only modern imaging methods can fully reveal the entire dimension of the hernia. Even combined modern radiological and surgical techniques were not sufficient to fully unravel the nature of the hernia, while anatomical reconstruction of the endoscopic site finally confirmed its interstitial situation. Extensive literature research that went beyond the key words in PubMed, but referred to older literature, finally clarified that these rare hernias were a feared issue among surgeons and were associated with a high mortality rate since they were difficult to predict without diagnostic imaging and enigmatic due to the lack of a systematic definition and their rarity. From this, we feel that the older literature as well as clinical hints such as congenital malformations, male gender and concomitant hernias at more conventional sites might be helpful for a timely diagnosis of this rare entity (see: Learning points).
Preoperatively difficult to localize, by endoscopy also difficult since not all abdominal wall sheaths are separate and only viewed from “inward”.
Often several hernias are observed in patients with an interstitial hernia.
The vast majority are male patients.
Congenital malformations may pre-dispose to interstitial hernias due to obstruction of the inguinal canal.
The presence of cystic kidneys and other malformations such as intracranial aneurysms may point to inguinal malformations due to extracellular matrix deficiencies.
Endoscopic treatment of this type of hernia is a suitable method since this hernia can also be easily closed with a mesh without modification of the method, in our case even in a patient with dialysis-dependent renal failure due to cystic kidney disease.
The authors gratefully acknowledge Karl Link, Mirjana Manestar, and Magdalena Vich, Division of Gross Anatomy, Institute of Anatomy, University of Zurich, for helpful support of this study.
- Bichet D, Peters D, Patel AJ, Delmas P, Honoré E (2006) Cardiovascular polycystins: insights from autosomal dominant polycystic kidney disease and transgenic animal models. Trends Cardiovasc Med 16:292–8View ArticleGoogle Scholar
- Cavallaro G, Fornari F, Polistena A, Orlando G, De Toma G (2007) Un caso di ernia peri-inguinale strozzata. G Chir 28:142–4Google Scholar
- Gallese N (1991) Peri-inguinal hernia: classification and casuistry. Considerations on the rarity of the occurrence, personal case reports. Minerva Chir 46:393–5Google Scholar
- Ghosh SK, Sharma S, Biswas S, Chakraborty S (2014) Adriaan van den Spiegel (1578–1625): anatomist, physician, and botanist. Clin Anat. Published online doi:10.1002/ca.22414Google Scholar
- Graser (1913) Handb. d. prakt. Chir. (v. Bruns, Garre. u. Kuttner). Bd. V, S, Stuttgart, p 572Google Scholar
- Greene Cumston C (1905) Interstitial hernia. Ann Surg 41:427–36View ArticleGoogle Scholar
- Grierson J, Leacock A (1949) Spontaneous ventral hernia. Two cases of para-inguinal hernia. Br J Surg 36:327–8View ArticleGoogle Scholar
- Holloway JK (1922) Spontaneous lateral ventral hernia. Ann Surg 75:677–85View ArticleGoogle Scholar
- Kanaan N, Devuyst O, Pirson Y (2014) Renal transplantation in autosomal dominant polycystic kidney disease. Nat Rev Nephrol 10:455–65View ArticleGoogle Scholar
- La Chausse BI (1756) Dissertatio chirurgica de hernia ventrali. Gessare, NaplesGoogle Scholar
- Light D, Chattopadhyay D, Bawa S (2013) Radiological and clinical examination in the diagnosis of Spigelian hernias. Ann R Coll Surg Engl 95:98–100View ArticleGoogle Scholar
- Lower WE, Hicken F (1931) Interparietal hernias. Ann Surg 94:1070–87View ArticleGoogle Scholar
- Lynen Jansen P, Mertens PR, Klinge U, Schumpelick V (2004) The biology of hernia formation. Surgery 136:1–4View ArticleGoogle Scholar
- McGlannan A (1927) Lateral ventral hernia. Ann Surg 85:284–7View ArticleGoogle Scholar
- Molliere D (1877) Bull. et mem. Soc. de Chir., Paris, vol iii., pp 278–284Google Scholar
- Moreno-Egea A, Carrasco L, Girela E, Martin JG, Aguayo JL, Canteras M (2002) Open vs laparoscopic repair of Spigelian hernia: a prospective randomized trial. Arch Surg 137:1266–8View ArticleGoogle Scholar
- Morris-Stiff G, Coles G, Moore R, Jurewicz A, Lord R (1997) Abdominal wall hernia in autosomal dominant polycystic kidney disease. Br J Surg 84:615–7View ArticleGoogle Scholar
- Muysoms FE, Miserez M, Berrevoet F, Campanelli G, Champault GG, Chelala E, Dietz UA, Eker HH, El Nakadi I, Hauters P, Hidalgo Pascual M, Hoeferlin A, Klinge U, Montgomery A, Simmermacher RK, Simons MP, Smietański M, Sommeling C, Tollens T, Vierendeels T, Kingsnorth A (2009) Classification of primary and incisional abdominal wall hernias. Hernia 13:407–14View ArticleGoogle Scholar
- Netter FH (2010) Atlas of human anatomy. Elsevier, LondonGoogle Scholar
- Pirson Y, Chauveau D, Torres V (2002) Management of cerebral aneurysms in autosomal dominant polycystic kidney disease. J Am Soc Nephrol 13:269–76Google Scholar
- Rath A, Bhatia P, Kalhan S, John S, Khetan M, Bindal V, Ali A, Singh R, Wadhera S, Bansal N (2013) Laparoscopic management of Spigelian hernias. Asian J Endosc Surg 6:253–6View ArticleGoogle Scholar
- Salameh JR (2008) Primary and unusual abdominal wall hernias. Surg Clin N Am 88:45–60View ArticleGoogle Scholar
- Skandalakis P, Zoras O, Skandalakis JE, Mirilas P (2006) Spigelian hernia: surgical anatomy, embryology, and technic of repair. Am Surg 72:42–8Google Scholar
- Steimker (1912) Beitr. z. klin. Chir, vol bcxxii., p 633Google Scholar
- Swiss Academy of Medical Sciences (2008) Verwendung von Leichen und Leichenteilen in der medizinischen Forschung sowie Aus-, Weiter- und Fortbildung., http://www.samw.ch/de/Ethik/Richtlinien/Aktuellgueltige-Richtlinien.html. Published 27 Nov 2008Google Scholar
- Swiss Academy of Medical Sciences (2010) Research with human subjects; a manual for general practice., http://www.samw.ch/en/News/News.html. Published 1 Apr 2010Google Scholar
- Ulbak S, Ørnsholt J (1983) Para-inguinal hernia. An atypical Spigelian variant. Acta Chir Scand 149:335–6Google Scholar
- Yokoyama T, Kobayashi A, Shimizu A, Motoyama H, Miyagawa S (2013) Laparoscopic repair for a previously unreported form of ventral hernia on the right iliac fossa in an elderly emaciated woman., Hernia 2013, Nov 12 [Epub ahead of print]Google Scholar
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