Congenital Spigelian hernia and ipsilateral cryptorchidism: a new syndrome?

Spigelian hernia (SH) is a rare ventral interstitial hernia occurring through a defect in the transversus abdominis aponeurosis (Spigelian fascia). Spigelian fascia is found between the lateral border of the rectus abdominis muscle and the semilunar line, which extends from the costal cartilage to the pubic tubercle. In other words, Spigelian line is where the transversus abdominis muscle ends in an aponeurosis characterized by a congenital or acquired defect in the Spigelian aponeurosis. Pediatric cases of SH are either congenital or acquired due to trauma, previous surgery or increased intra-abdominal pressure. SH in combination with ipsilateral cryptorchidism may constitute a new syndrome, as such cases are extremely rare in the literature is new syndrome is characterized by the following congenital, ipsilateral disturbances: SH, absence of inguinal canal and gubernaculum and the homolateral testis found within the Spigelian hernia sac (a hernia sac containing undescended testis). The aim of this study is to emphasize some typical findings of this specific entity, and, hence, the necessity for a thorough investigation of the origin of the SH.

Congenital lateral abdominal wall hernia.

Congenital abdominal wall defects that are located outside of the anterior wall are extremely rare and difficult to classify because there are no well accepted guidelines. There are two regions outside of the anterior wall: the flank or lateral wall; and the lumbar region. We report the case of a patient with an oval 3 cm-diameter hernia defect located above the anterior axillary line, which affects all layers of the muscular wall. An anorectal malformation consisting of a recto-vestibular fistula was also identified, and chest X-ray showed dextrocardia. The suggested treatment is repair of the defect before 1 year of age. Given that the anomalies described may accompany lateral abdominal wall hernia, it is important to diagnose and treat the associated defects.

The syndrome of Spigelian hernia and cryptorchidism: a review of paediatric literature.

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.

Undescended testis accompanying congenital Spigelian hernia: is it a reason, a result, or a new syndrome?

INTRODUCTION: 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. METHODS: 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. RESULT: 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. CONCLUSIONS: 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 associated with undescended testis.

BACKGROUND: A Spigelian hernia (SH) is a ventral interstitial hernia through a defect in the Spigelian fascia; an undescended testis is sometimes associated with this clinical entity in male newborns. The etiopathogenesis, surgical anatomy, diagnostic methods, and treatment for this rare condition are discussed with a review of the literature. METHODS: A 20-day-old newborn was admitted to our hospital for a swelling in the right lower abdomen and undescended testis. Physical examination of the abdomen and scrotum revealed a congenital SH associated with cryptorchidism. RESULTS: Herniotomy, herniorrhaphy, and orchidopexy were performed. In the post-operative period, scrotal abscess occurred and was drained. After drainage, the fixed testis was found to be atrophic. CONCLUSIONS: This association may be a distinct clinical syndrome. The operation time in cases of neonatal SH with undescended testis should be well planned because of probable surgical complications such as vascular damage, tension, or compression.

Challenges of giant ventral hernia repair in children in an African tertiary care center with limited resources.

OBJECTIVE: The objective of this study is to report the challenges and outcome of giant ventral abdominal hernia repair in infants/children in a tertiary care center in Africa. DESIGN: A retrospective analysis of infants/children who had a repair of giant ventral abdominal hernia between January 1998 and December 2007 at the University of Benin Teaching Hospital (UBTH), Benin City, Nigeria, was carried out. RESULTS: A total of 41 children aged between 9 months and 12 years (mean 2.6 +/- 1.8 years) comprising 25 males and 16 females with a male:female ratio of 1.6:1 had a repair of giant ventral abdominal hernias which were due to healed omphalocele in 37 (90.3%), incisional hernia in three (7.3%), and following initial skin closure of gastroschisis in one (2.4%). They had an average hernia defect of size 7.1 x 8.3 cm, which contained both solid and hollow viscera in all of the patients, along with 11 (29.7%) syndromic omphalocele. The major challenge was inadequate intraabdominal volume needed to accommodate the herniated viscera in nine children, which was compounded by the nonavailability of silastic materials, a pediatric ventilator, facilities required for intraabdominal pressure monitoring during closure, as well as undiagnosed associated cardiac anomaly. Overall, 39 (95.1%) children survived, while two (4.9%) mortalities were recorded due to postoperative aspiration in one child and cardiopulmonary failure in the other. Of those who survived, 29 (74.4%) had a smooth postoperative course, while ten (25.6%) had morbidity, with a mean hospitalization duration of 10 +/- 2.5 days. There was no recurrence recorded on follow-up for 5 years at the surgical outpatient clinic. CONCLUSION: Despite the challenges, better results were achieved with repairs in infants/children compared to repairs performed in neonates.

Large congenital transmesenteric hernia: a missed small-bowel atresia?

Internal hernias are uncommon diagnoses and represent rare causes of intestinal obstruction. Diagnoses are frequently made perioperatively. We present herein an illustrated case of transmesenteric hernia diagnosed in a pregnant woman who consulted for severe abdominal and dorsal pains. As CT scan was not possible because of the obstetric history, a decision to perform surgery was made because of acute pain, no history of previous surgery, and a plain erect X-ray disclosing early signs of intestinal obstruction. Perioperative findings were an ileal volvulus through a transmesenteric fossa circled by a unique ileocolic branch. This particular vascular disposition suggests transmesenteric hernias may be the first step in the constitution of congenital small-bowel atresia.

Treves' field congenital hernias in children: an unsuspected rare cause of acute small bowel obstruction.

The Treves' field congenital hernias (TFCH) are an unusual cause of bowel obstruction that may result in irreversible damage of the small bowel or a fatal outcome, unless treated timely and properly. We analyzed retrospectively the medical records of four patients who underwent surgery for TFCHs over a period of 9 years. The types of TFCHs, only diagnosed at surgery, were identified as transmesenteric through a defect in Treves' field in three patients, and as hernia into Treves' field pouch in one patient. Patients had non-specific clinical manifestations, and imaging findings demonstrated only small bowel obstruction. Delay in accurate preoperative diagnosis, and especially patient-related delay, led to a primary enterectomy-anastomosis for necrotic small bowel in the patients with transmesenteric hernias. One infant with necrotic bowel died postoperatively. It may be difficult to diagnose TFCHs in children preoperatively. Misdiagnosis of bowel obstruction caused by this type of internal hernia might lead to small bowel necrosis or death. The risk of this complication seems to be more influenced by pre-hospital delay. Awareness of the severe consequences of delay in diagnosis, high clinical suspicion, early recognition, and proper surgical intervention are essential in the successful management of TFCHs.

Hypospadias associated undescended testis in a Spigelian hernia.

BACKGROUND: First described in 1764, Spigelian hernias are rare. The patient profile is of an overweight, 50-year-old with associated respiratory disease. However we report the case of a four-month-old, with an associated hypospadias and cryptorchidism, where the testis was lying in a spigelian hernia. AIMS: To highlight this unusual presentation of cryptorchidism. CONCLUSION: Embryological development is a complex event that relies on the sequencing of hormonal surges for anatomical development. In this case report we postulate that the findings are secondary to hormonal disruption.

Spigelian hernia: a case report and review of the literature.

Spigelian hernia is a rare abdominal hernia that occurs through Spigelian aponeurosis. The Authors present a case of Spigelian hernia associated with narrowing of sigmoid colon and diverticular pathology. They also described historical background, surgical anatomy and etiopathogenesis of this hernia. By a remarkable revision of literature, they sum up epidemiology and clinical features of Spigelian hernia. Furthermore, they discuss diagnostic and therapeutic principles.

Congenital Spigelian hernia with cryptorchidism: probably a new syndrome.

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.

[Prenatal diagnosis and management of laparoschisis. Case report]. / Diagnosticul si conduita prenatala în laparoschizis.

The prenatal diagnosis of laparoschisis may be established by ultrasound early in the end of the first pregnancy trimester. The visualisation of the abdominal wall defect may predict a good fetal prognosis in the absence of the other anomalies and may establish the most favorable moment of birth. Identification of several risk factors for this malformation could lead to the decrease of its incidence.

[How is an abdominal hernia formed?]. / Comment se forme une hernie abdominale?

Abdomen can be compared in broad outline with an irregular cylinder, limited at the top by the diaphragm and below by the pond. The walls of this cylinder are musculo-aponevrotic and present "zones of weakness" seats of the hernias of the abdominal wall. We propose a topographic anatomical approach of abdominal hernias.

Congenital lumbar hernia in association with carpus equina varus.

A rare case of congenital lumbar hernia associated with carpus equina varus is described in a week old baby. The treatment is described with limited review of the literature.

Congenital varicella syndrome.

The fetal consequences of chickenpox complicating pregnancy depends on the period of gestation at which the infection is contracted. The extremely rare classical form of congenital varicella syndrome, resulting from maternal varicella infection in the first trimester of pregnancy, is being reported here. The unusual features in this baby are bilateral hypoplastic lower extremities, fracture of bones, a normal electroencephalogram and phantom hernias of the anterior abdominal wall.

Planned totally extraperitoneal laparoscopic Spigelian hernia repair.

A Spigelian hernia is a congenital defect in the tranversus aponeurosis fascia. Traditionally, an open anterior hernioplasty was used to repair these defects. Recently, laparoscopic approaches have been described. This report describes the first application of the totally extraperitoneal laparoscopic approach to a planned repair of a Spigelian hernia. The patient was a 62-year-old white female with a reducible left lower quadrant anterior abdominal wall bulge consistent with a Spigelian hernia. At the time of surgery, we exposed the posterior rectus fascia and modified our extraperitoneal inguinal hernia technique by passing the balloon dissector in a more lateral orientation. This created a unilateral preperitoneal space with adequate room for dissection and mesh fixation. The Spigelian defect was easily identified. Its preperitoneal fat contents were reduced, and a 5-mm laparoscopic tacking device was used to secure a piece of prolene mesh. The patient was discharged home with no complications. Placement of the mesh in the preperitoneal space avoids direct interaction of the mesh prosthesis and the intraperitoneal viscera. In conclusion, we find that a laparoscopic totally extraperitoneal approach is technically feasible and advantageous when a Spigelian hernia is diagnosed preoperatively.

Internal hernia with triple hiatus of congenital origin: report of a case.

We report herein the case of a 65-year-old woman who was referred to our department with prolonged ileus symptoms despite conservative therapy. A plain abdominal radiograph showed intestinal gas shadows with an air-fluid level in the lesser curvature of the stomach. As no improvement was achieved by the insertion of a short tube, a long tube was inserted. A loop formation of the long tube in the subphrenic region was detected on an abdominal radiograph, and an enterogram showed an interruption in the ileum in the lower abdomen. The patient was diagnosed as having an adhesional ileus and a strangulated ileus due to a lesser sac hernia. A laparotomy was performed which revealed that the small intestine had herniated into the lesser sac space through a hiatus of Treitz' fossa and a hiatus in the transverse mesocolon. Furthermore, part of the small intestine had herniated through an omental hiatus. The herniated intestine was manually reduced and the hiatus was closed. However, as the right ovary was found to have adhered to the ileum and stenosis was seen, we were forced to perform partial resection of the ileum. Considering that this patient had no history of laparotomy in the upper abdomen, abdominal injury, or acute abdomen, it was surmised that the three abnormal hiatuses were congenital.

Congenital spigelian hernia and cryptorchidism: cause or coincidence?

Congenital spigelian hernia (SH) is very rare in the pediatric age group. This is a report of two cases of SH in 1-week and 3-month-old male infants. A review of the literature revealed only 35 cases of SH in children younger than 17 years of age, bringing the total including our 2 cases to 37. There were 25 males and 12 females, a ratio of 2.1:1. Their ages ranged from 6 days to 17 years (mean 4.52 years). The hernia was situated on the right side in 13, the left side in 19, and was bilateral in 4. In one case the side of the hernia was not mentioned. In 29 cases the hernia was spontaneous while in 5 it was caused by trauma. In 3 children the hernia developed postoperatively, in 2 following repair of a congenital diaphragmatic hernia and in 1 following excision of a mediastinal neuroblastoma. Two children presented with a strangulated SH. Eleven of the 35 previously reported children had associated conditions; in 5 there was an ipsilateral undescended testis (UDT). Our two infants with SH also had an ipsilateral UDT. The significance of this association is discussed.