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Congenital Lumbar Hernia:Two Case Reports and a Review of the Literature

Esposito C. , Settimi A. , De Marco M., Alicchio F., Giurin I., Perricone F., De Fazio C., Esposito G.
Department of Pediatric Surgery University “Federico II” ,Naples, Italy

 

 

Correspondence

Ciro Esposito, MD, PhD
Associate Professor of Pediatric Surgery
Department. of Pediatrics, Chair of Pediatric Surgery
“ Federico II” University of Naples
Via Pansini 5, 80131, Naples, Italy
tel: +39-081-7463378;
fax: +39-081- 7463361
email: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

Congenital lumbar hernias are very rare, with no more than 50 cases reported in the literature so far. This may be the reason why pediatric surgery textbooks seldom mention this affection and why we decided to describe two patients with congenital lumbar hernia, along with a complete review of the literature.

Key words: congenital lumbar hernia, space of grynfeltt-lesshaft, space of Petit

 

Introduction

Congenital lumbar hernia is a compressible soft mass that may occur in the lumbar region especially through two weak points: the superior lumbar space of Grynfeltt- Lesshaft [1-2] and the inferior lumbar space of Petit ( fig. 1) [3-5]. It may be present at birth or may develop in older children [6-9]. Two cases of lumbar hernia – the first in a 5 year old child presenting also with pseudocholinesterase deficiency, the second in a 6-month infant in whom the hernia was diagnosed at birth – prompted us to perform a complete review of the literature on this pathology.

lumbar hernia 1

Figure 1. Representation of the anatomy of the Grynfelt-Lessaft's quadrilater and of the Petit’s triangle (drawings modified by Testut-Jacob and Gentile)

Case reports: 

Case 1: A 5 year old boy was admitted to our paediatric ward with a left superior lumbar hernia, noticed for the first time one year before. At the examination a 5x6 cm soft, swollen and reducible mass was evident below the left costal margin laterally to the dorsolumbar spine. The mass was painless, even when it protruded during effort. Routine laboratory investigations evidenced a total pseudocholinestherase deficiency, probably of familial origin, which given the partial deficiency in an older brother. X-rays of the chest and dorso-lumbar spine did not show any skeletal alterations. The patient underwent surgical repair of the hernia under general anaesthesia without any administration of depolarizing agents. Repair surgery consisted in the reduction of the hernia, preparing the edge of the hernia and creating a double muscolofascial layer using interrupted non absorbable suture. Recovery was uneventful and the patient was discharged on the 6th day postoperatively. At the follow up examinations 6 months and 2 years later, there was no relapse of the hernia.

Case 2: A 6 month old child, born after an uncomplicated pregnancy, was admitted to our paediatric ward for a very large mass that completely occupied the left lumbar region. The mass, noticed soon after birth, had increased gradually until admission. At the examination, there was a calotte-shaped mass measuring 7x8 cm, which was very soft and easily reducible by pressure of the hand, but increased enormously in volume when the child cried. It extended from the 12th left rib to the iliac crest and laterally to the extension of the posterior axillary line. The parents refused to have the infant undergo surgery, which was nevertheless performed one year later in another institution.

Discussion

Congenital lumbar hernias usually appear as large compressible soft-tissue mass in the lumbar region. The lumbar region is delimited above by the 12th rib, below by the iliac crest, medially by the vertebral spine and its musculature, and laterally by the extreme oblique muscle. Lumbar hernias are classified based on their anatomic localization: diffuse hernias occupy the whole lumbar region and localized hernias may appear in two weak points of the lumbar region – the superior space of Grynfeltt-Lesshaft (GL) [1-2] and the inferior space of Petit [3-5].

The superior space may be quadrilateral or triangular. In the first case it is delimited posteriorly by the sacrolumbar musculature, above and behind by the serratus postero-inferior muscle, above and in front by the 12th rib, in front and below by the posterior fibres of the internal oblique muscle. When the space of GL is triangular it is delimited above by the 12th rib, medially by the erectus spinal muscle and internally by the internal oblique muscle. The inferior triangle of Petit is delimited below by the iliac crest, in front by the rear margin of the external oblique muscle and behind by the anterior margin of the great dorsal muscle. The hernial sac may be empty, may contain various organs as the spleen, omentum, small bowel, appendix and colon or, may also contain, in order of frequency, retroperitoneal fat, the bowel, kidney, omentum, colon, stomach, ovary, spleen, appendix and kidney.

From the etiopathogenic point of view, lumbar hernias may be congenital – presenting soon after birth or in the first years of life, or acquired (spontaneous, posttraumatic, postoperative, a post-regional suppurative process) [6-11]. The causes of congenital hernias have not been completely defined: according Touloukian, a somatic mutation in the first weeks of embryogenesis, caused by transitory anoxia leading to an alteration of muscles and aponeuroses of the lumbar region may be the underlying cause. This theory is supported by the fact that lumbar hernias are associated with the lumbar-costo-vertebral syndrome in 75% of cases [12, 13]. As to isolated lumbar hernias, in 1870 Lesshaft suggested that they may be due to weakness of the lumbar region, where the 12th neurovascular bundle perforates the transversalis fascia. The congenital form may be isolated, although it often occurs in association with the lumbo-costo-vertebral syndrome – involving the costal, vertebral and lumbar musculature, or with many other abnormalities (Table1).

Table I: Anomalies associated with lumbar hernia.

Lumbocostovertebral syndrome, which includes one or more of following anomalies:
Hemivertebra, rib abnormalities (absence, hypoplasia, fusion, gap), aplasia of dorsolumbar muscles, scoliosis eventually with the
convex curve on the site of hernia.
Other isolated or associated abnormalities, in alphabetic order:
1. absence of the gallbladder and cystic duct
2. absence of the right kidney
3. absence of the tibia
4. bilateral dislocation of the radial head
5. pseudocholinesterase deficiency
6. crossed-fused renal ectopia
7. eventration of the right hemidiaphragm
8. focal nodular hyperplasia of the liver
9. hydrometrocolpos
10. hydronephrosis secondary to ureteropelvic obstruction
11. inguinal hernia
12. malrotation of the gut
13. ARM
14. micrognathia
15. microophtalmia
16. monolateral anorchia
17. monolateral criptorchidism
18. posterior meningomyelocele
19. sciatic hernia
20. 7th nerve paralysis

The association with pseudocolinestherase deficiency is a rare finding: in fact, to the best of our knowledge, it is the first case reported in the literature. The causes of acquired hernias are very different and may follow trauma, regional surgery for posterior meningocele or neuroblastoma and lastly, an infection involving the kidney area [12-24]. A Roentgenogram of the lumbar region may detect air filled bowel loops in the sac, whereas CT and US are most useful diagnostic tools especially to detect solid organs in the sac [25-27]. Generally unilateral and exceptionally bilateral – as in the case described by Touloukian and Karamani [8, 13] – congenital lumbar hernia occur in twice as much in males than in females. The treatment of lumbar hernia is surgical and should be performed in the first year of life [28-34] or as soon as the diagnosis is made to prevent incarceration; this is nevertheless a very rare event, the case reported by Hancock and Wiseman is the only one in the literature so far [32].

A large defect also requires early repair, to avoid worsening until the time of surgery. Surgical repair consists in a simple closure of the fascial defects or in a double layer musculo-fascial repair using interrupted non absorbable suture without tension. In the presence of a large defect, a patch of Goretex or Dacron of muscle mesh prosthesis may be used between the internal oblique muscle, the transverse muscle and the periosteum of the iliac crest. With the increased availability of and experience with videosurgery, lumbar hernias may also be repaired with the laparoscopic approach [35-38]. The laparoscopic approach is very simple. The patient is placed in a semilateral position with a 45 degree elevation corresponding to the side of the hernia. An open laparoscopy is made using a 5-10 mm trocar according the age of the patient. After inducing pneumoperitoneum and inserting the laparoscope, two trocars are placed to introduce the dissection instruments – one in the infraumbilical midline and the other in the supraumbilical midline. After defining the edges of the hernia, a single suture of fascial defects or a double layer of the musculo-fascial defects is performed. In case of a large defect, a polypropylene mesh is inserted and anchored to the 12th rib in case of superior lumbar hernia, or to the iliac crest in case of inferior lumbar hernia. Prognosis of the lumbar hernia is good and only one case of recurrence has been reported in the literature [32].

 

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