Congenital Spigelian hernia and ipsilateral cryptorchidism: a new syndrome?

Spigelian hernia (1-2% of all hernias) is the protrusion of preperitoneal fat, peritoneal sac, or organ(s) through a congenital or acquired defect in the spigelian aponeurosis (i.e., the aponeurosis of the transverse abdominal muscle limited by the linea semilunaris laterally and the lateral edge of the rectus muscle medially). Mostly, these hernias lie in the "spigelian hernia belt," a transverse 6-cm-wide zone above the interspinal plane; lower hernias are rare and should be differentiated from direct inguinal or supravescical hernias. Although named after Adriaan van der Spieghel, he only described the semilunar line (linea Spigeli) in 1645. Josef Klinkosch in 1764 first defined the spigelian hernia as a defect in the semilunar line. Defects in the aponeurosis of transverse abdominal muscle (mainly under the arcuate line and more often in obese individuals) have been considered as the principal etiologic factor. Pediatric cases, especially neonates and infants, are mostly congenital. Embryologically, spigelian hernias may represent the clinical outcome of weak areas in the continuation of aponeuroses of layered abdominal muscles as they develop separately in the mesenchyme of the somatopleura, originating from the invading and fusing myotomes. Traditionally, repair consists of open anterior herniorraphy, using direct muscle approximation, mesh, and prostheses. 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.

Dear Editor, Spigelian hernia can be described as a protrusion of a peritoneal sac, organ, or preperitoneal fat through a congenital or acquired defect in the Spigelian fascia, which is the part transeversus abdominis aponeurosis that lies between the semilunar (Spigelian) line and the lateral edge of the rectus muscle, often above the inferior epigastric vessels, at the level of the arcuate line where the fascia is widest and weakest.[1] Some authors have suggested that perforating vessels can weaken the fascia, permitting the entrance of a lipoma and leading to hernia formation.[2] Because of their small neck size, approximately one third of Spigelian hernias in adults appear incarcerated during surgery. Spigelian hernia is in itself very rare and is difficult to diagnose clinically. It has been estimated that it constitutes 0.12% of abdominal wall hernias.[3] Despite the fact that cases of Spigelian hernia have also been reported in children or even infants, it is a relatively rare hernia that occurs usually in females between 40 and 70 years of age, while the etiologic factors classically associated with this defect are claimed to be obesity, chronic obstructive pulmonary disease, prior surgery, and abdominal trauma.[4] Patients with Spigelian hernia usually complain of pain or lump or both at the site of herniation.[5] The pain is sharp and constant or intermittent, or there is a dragging and uncomfortable feeling.[5] It has been estimated that Spigelian hernias are approximately 2% of the abdominal wall hernias that require emergency operation due to incarceration.[6] When strangulation or incarceration of the herniated contents is present, the pain at the hernia site will be severe and constant. A correct preoperative diagnosis is made in only 53 to 75% of patients, and a significant percentage of incarcerated Spigelian hernias are diagnosed during an emergency laparotomy.[4][7] Spangen reported that 24.1% of Spigelian hernias reach the surgeon incarcerated and that the hernial sac contents are usually found to be small bowel, colon, or omentum.[5] This is supported by our patient in whom hernia sac content was omentum. This report emphasizes possibility of a hernia while a surgeon is dealing with an abdominal wall abscess and the surgeon has to be totally sure about the appropriate treatment to prevent devastating complications. A 60-year old obese female presented to our hospital with history of constant pain and lump in the right lower abdominal wall for the last three days. She also gave history of fever for the last two days. Her bowel habits were normal and did not have any other complaint. On examination, a soft lump of about 4x4x1.5 cm was found on right lower abdomen at the margin of the right semilunar line with inflamed overlying skin (Figure 1 and Figure 2). Fig. 1 Spigelian hernia as abdominal wall abscess. Fig. 2 CT scan showing abdominal wall abscess and adjacent bowel walls. There was no cough impulse and lump would not decrease on lying down position. Bowel sounds were normal. Aspiration of swelling revealed purulent materials. Digital rectal examination was normal and x-ray of abdomen revealed nothing significant. Ultrasound (USG) abdomen showed parietal wall abscess with gut loop adherent to it. Computed tomography (CT) scan was advised which confirmed Spigelian hernia with strangulation or incarceration of the omentum and abscess formation in abdominal wall. The patient was taken up for exploratory laparotomy. On exploration, about 25 ml of pus was drained. The defect was seen in peritoneum and gangrenous omentum was seen herniating (Figure 3). Fig. 3 Gangrenous omentum. The strangulated omentum was excised and the defect was closed with herniorrhaphy. Pus culture sent grew Klebsiella sensitive to imipenam. The post-operative course of the patient was uneventful. The patient was followed up for over six months without any local recurrence.

BACKGROUNDSpigelian hernia (SH), a protrusion of intra-abdominal contents through a defect in the semilunar line (Spigelian fascia) of the abdominal wall, is extremely rare in the pediatric population. Fewer than 100 cases of pediatric SH have been reported in the literature since the first description in 1939. Pediatric SH is often congenital and may present with non-specific symptoms, making diagnosis challenging. Notably, about one-quarter to one-third of reported pediatric cases are associated with ipsilateral undescended testis, an association sometimes termed the "Spigelian-cryptorchidism syndrome".AIMTo systematically review all reported cases of Spigelian hernia in children and identify its diagnostic and surgical features.METHODSA comprehensive literature search was performed (1939 through 2023) using PubMed and other databases for all publications on Spigelian (semilunar line) hernias in children. Both English and non-English articles were included. Case reports, case series, and relevant reviews were analyzed. Data extracted included patient demographics, hernia side/location, clinical features, imaging and intraoperative findings, coexisting conditions (particularly cryptorchidism), management (open vs laparoscopic repair), and outcomes.RESULTSA total of approximately 90 pediatric SH cases from 44 publications were identified. The median age at presentation was around 3-4 years (range: Neonate to 17 years). Slight male predominance was observed, although SH also occurs in females. About 30% of cases involved an ipsilateral undescended testis within the hernia sac or in the Spigelian region. Rare associations with other anomalies were noted, including concurrent inguinal hernias, umbilical hernia, and even complex syndromic presentations (for example, limb defects, neuroblastoma) in a few cases. Clinical presentation often included a reducible lump or intermittent bulge along the lateral aspect of the lower abdomen, sometimes misdiagnosed as an inguinal hernia. Recurrent localized abdominal pain was a frequent symptom, and some infants presented with acute intestinal obstruction from incarcerated SH. Diagnosis was most commonly established by ultrasound, which is considered the gold standard imaging modality for SH in children due to its ability to detect fascial defects and herniated tissue dynamically. A subset of cases (approximately 10%) were attributed to blunt abdominal trauma (for example, bicycle handlebar injury), causing an acquired SH. Management: All reported pediatric SHs were treated surgically, given the high risk of incarceration. Open hernia repair through a small transverse incision over the defect was the traditional approach, and no recurrences have been reported after adequate fascial closure. Over the last decade, laparoscopic repair has become the method of choice, especially for cases combined with an undescended testis, allowing simultaneous orchiopexy. Laparoscopic techniques have been successful in safely reducing the hernia and closing the defect, with excellent outcomes and minimal morbidity.CONCLUSIONSpigelian hernia in children is a rare but clinically important entity that should be considered in cases of unexplained recurrent abdominal pain, lateral abdominal wall swelling, or cryptorchidism. Early recognition using high-resolution ultrasound and prompt surgical intervention are critical to prevent life-threatening incarceration. Congenital defect of the Spigelian fascia underlies most pediatric cases, and the frequent association with ipsilateral undescended testis suggests a developmental interplay. Surgical repair has excellent outcomes. Increased awareness of pediatric SH among clinicians is necessary to ensure timely diagnosis and treatment, thereby avoiding complications and improving patient outcomes.

Lateral ventral (spigelian) hernias in infants and children.

Pediatric cases of Spigelian hernias are rare. Only a few reports on this condition, in combination with ipsilateral cryptorchidism and testis in the hernia sac, have been published. We report on Spigelian hernia in a 3-week-old boy containing both the ipsilateral testis, without a gubernaculum and an incarcerated loop of the small intestine. It has been suggested that the combination of Spigelian hernia and ipsilateral cryptorchidism is part of a new syndrome. We discuss whether the lack of a gubernaculum and an inguinal canal reported in other similar cases may be additional elements of this new syndrome. We present a comprehensive overview of pediatric patients with Spigelian-cryptorchidism syndrome reported in the English language literature. In 75% of male infants with Spigelian hernia, there is an associated ipsilateral cryptorchidism, and in 87% of these patients, the testis is 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.

Frequent reporting of cases of the coexistence of a Spigelian hernia (SH) with an undescended testis (UT) suggests that this phenomenon may be a syndrome. In this article, four pediatric cases in which an UT accompanies a congenital SH have been discussed in light of the literature. In this study, four cases aged between 6 months and 5 years who had a SH accompanied by an UT were evaluated and underwent surgery. The patient's ages were 6 months, 1 year, 2 years, and 5 years old. The testis was observed in the opened hernia sac. The patients did not have a gubernaculum or an inguinal canal on the side of the hernia. Neither the theories suggesting that SH leads to an UT nor those suggesting that an UT leads to a SH are satisfactory. We believe that this coexistence may be the congenital Spigelian-cryptorchidism syndrome seen in boys. As in the four cases presented here, elements of this syndrome are defects in the Spigelian fascia and the hernia sac enveloping the testis and an absence of the gubernaculum and the inguinal canal.

Congenital Spigelian Hernia and Ipsilateral Cryptorchidism: Raising Awareness Among Urologists

Spigelian hernia (SH) is rarely seen in pediatric age group and is usually associated with cryptorchidism on the same side; termed as a syndromic association of the defect in the Spigelian fascia and absence of gubernaculum and inguinal canal. The absence of the inguinal canal has surgical implication as to placement of the undescended testis into the scrotum. A 3-month-old baby presented with spigelian hernia and ipsilateral impalpable testis. The spigelian hernia was repaired and undescended testis which was present in abdominal wall layers was brought to scrotum with cord structures anterior to external oblique muscle.

Nearly 28% of pediatric Spigelian hernias reported in the literature are associated with ipsilateral cryptorchidism. However, the pathogenetic relationship between the two has not been satisfactorily explained in the past. This paper describes a male neonate born with cryptorchidism and imperforate anus. Anal stenosis following the treatment of imperforate anus had let to the development of multiple hernias including Spigelian hernia on the right side. Surgical exploration revealed the right testis being located within the Spigelian hernia. Based on the sequence of events, it is hypothesized that Spigelian hernia in this case is a sequela of maldescended testis and raised intraabdominal pressure. As this explanation is also applicable to all of the previously reported cases, the author suggests that the combination of Spigelian hernia and ipsilateral cryptorchidism could probably form a hitherto unrecognized new syndrome.

Strangulation and necrosis of right hemicolon as an extremely rare complication of Spigelian hernia.

Spigelian hernias are an uncommon protrusion defect noted between the rectus abdominis and the transversus abdominis muscles, at the semilunar line, with a low incidence of approximately 0.12% to 2% of all ventral hernias. Furthermore, the incidence of bilateral cases is noted to be even lower in the general population. They are associated with bowel incarceration and strangulation; hence surgical repair is indicated. Therapeutic alternatives for Spigelian hernias include open repair, however laparoscopic repair stands as the standard of care in these cases. Feasible and successful robotic repair has been reported, with associated advantages in terms of visualization and surgical instrument dexterity. We present the first ever reported cases of bilateral Spigelian hernias repaired using robotic approach. Two female Puerto Rican patients referred to our institution complaining of abdominal pain, where imaging studies found bilateral Spigelian hernias. In both cases, a transabdominal preperitoneal repair was performed using the Da Vinci Surgical System. Both patients were discharged home tolerating oral intake with adequate wound healing. On follow up visits, patients denied abdominal discomfort and had adequate wound healing. Robotic surgery for Spigelian hernias poses an advantage over laparoscopic repair as improved visualization, mobility, and precision in movements allow for more gentle tissue manipulation. Furthermore, this is the first evidence of safe and effective repair in the uncommon entity of bilateral cases, providing a newer alternative in the setting of such presentation.

INTRODUCTION Lateral ventral hernia (Spigelian hernia) is a rare surgical condition in children. The cases are commonly seen in adult population. It arises because of weakness in fascia. Preperitoneal fatty tissue, intraabdominal organs may herniate through hernial sac. Strangulation or incarceration may rarely occur. Frequency of undescended testis was found much higher in cases with Spigelian hernia than normal population. A one-month old boy who had testis in Spigelian hernial sac was found to have gubernaculum and inguinal canal opposing previously described “Spigelian-cryptorchidism syndrome”. The aim of this study is to describe details of this case.

Background: Spigelian hernia (SH) is a rare entity characterized by a defect of the anterior abdominal wall located along the Spigelian line, it may be congenital or acquired.Association with other anomalies is worth reporting. Case Presentation: A 22-day-old male newborn was admitted with strangulated right inguinal hernia and operated on emergently. Clinical examination also revealed a Spigelian hernia in the left lower abdominal quadrant with bilateral cryptorchidism, associated with polydactyly of the small right finger and anal stenosis. At surgery, the SH contained a part of the small intestine and the ipsilateral undescended testis. Conclusion: Pediatric SH is rare, but its association with undescended ipsilateral testis is frequent. Other abnormalities can be concomitant to this association.

Background. Today, a special place in surgical practice is the treatment of patients with spigelian hernias, which can be subcutaneous, interstitial, prepperitoneal, and make up 1% of all external hernias of the abdomen. The difficulty in diagnosing of spigelian hernias can lead to a serious complication – infringement of the hernia, which will require emergency surgical treatment. Therefore, the study of the typical, sexual and variant anatomy of the Spigelian line region is important in improving the diagnosis and treatment of such patients.The aim. To study the features of typical, sexual and variant anatomy of the Spigelian line region.Materials and methods. We examined 42 non-fixed corpses of persons of both sexes without signs of pathology of the anterior abdominal wall. Among them were 26 (54.2%) men and 22 (45.8%) women. At autopsy, we performed anatomical dissection of the semilunar line region. We measured the width of the aponeurotic stretch from the end of the transverse abdominal muscle fibers to the lateral edge of the rectus abdominis muscle at level corresponding to d. bicostarum, umbilical ring and d. bispinarum.Results. We identified four clusters corresponding to the variations in the shape of the semilunar line: tapering down (9.5%), uniform wide (19.0%), tapering up (28.6%) and wide in the middle (42.9%). We found that the semilunar line, tapering up, was significantly more often observed in women (83.3%), uniformly wide – in brachymorphic body type (75.0%), wide in the middle – in mesomorphic body type (66.07%), and the semilunar line, tapering down, was found only in men. Variants of the shape of the semilunar line, tapering up or down, were absent in persons of the brachymorphic body type. Semilunar line, tapering up, was found (without significant differences) in persons of the mesomorphic body type in 41.6%, in the persons of the brachymorphic body type – in 58.4%, and semilunar line, tapering down, was noted in persons with a dolichomorphic body type in 75.0%.Conclusion. New data may allow to predict the location, type of spigelian hernia, and also improve the diagnosis and treatment of spigelian hernia.

Spigelian hernia (SH) is a rare type of lateral ventral hernia occurring through the Spigelian fascia, accounting for only 0.1-2% of all ventral hernias. Though commonly considered spontaneous, an increasing number of iatrogenic cases have been reported following laparoscopic surgery, particularly when 10-mm trocar ports are used. Due to the anatomical location between the rectus abdominis and semilunar line, SH can be challenging to detect clinically, often presenting with nonspecific symptoms. This is especially true in gynaecologic laparoscopy, where SH remains an underrecognized complication.We present the case of a 70-year-old woman who underwent laparoscopic total hysterectomy with bilateral salpingo-oophorectomy for a benign adnexal mass. Two days postoperatively, the patient developed a tender swelling at the trocar site after straining during defecation. CT imaging revealed a loop of small bowel herniated between abdominal wall muscles, with the fascial stitch still visible. During emergency laparoscopy, as we mobilized the small bowel out of the hernia defect, a bowel rupture was identified in the involved segment, leading to fecal peritonitis and necessitating conversion to open surgery. The patient's postoperative course was complicated by severe aspiration pneumonia requiring ICU admission and prolonged mechanical ventilation, as well as a surgical site infection requiring debridement.This case highlights the diagnostic difficulty and potentially severe complications of trocar-site Spigelian hernias. Even with proper fascial closure, factors such as early postoperative straining, advanced age, and predisposing anatomical defects may contribute to hernia formation. Surgeons should maintain a high level of suspicion in any postoperative patient presenting with localized pain or swelling near trocar sites, particularly lateral to the rectus muscle. Recognizing this uncommon yet potentially serious complication early can ensure early diagnosis and treatment, reducing the risk of morbidity.This report underscores the need for vigilance in gynaecologic laparoscopic practice and serves as a reminder that even well-closed trocar sites can lead to Spiegel hernia and thus culminate in severe complications.