Is laparoscopic approach for wandering spleen in children an option?

AIM: Wandering spleen present generally as an acute abdomen after twisting of the splenic vascular pedicle. This study aimed to review the literature with regard to the management and outcomes of the laparoscopy in children with wandering spleen.
METHODS: The literature was reviewed for articles on PubMed with regard to the following search terms 'laparoscopy', 'wandering', 'spleen' and 'children'. The inclusion criteria included article only in the paediatric age group of 0-16. Articles that did not meet the inclusion criteria were excluded from the study.
RESULTS: The PubMed search from 1998 to 2016 identified 15 articles. There were 20 children with an age range from 2 to 16 years who underwent the laparoscopic procedure for wandering spleen. The median age was 8 years. Associated conditions were present in 45% of patients: gastric volvulus (n = 3), torsion of the distal pancreas (n = 3), splenic cyst (n = 2), mental retardation and myotonic dystrophy (n = 1). In two cases, the spleen was twisted around the pedicle and was non-viable, and therefore, a splenectomy was performed. Other 18 cases were managed by splenopexy using a 3-5-port technique. An extraperitoneal pocket was created using a balloon device in five patients. Fixation of the spleen was performed using a mesh in 10 cases and omentum in three cases. In one case, additional support was created by plicating the phrenicocolic ligament. Simultaneous gastropexy was performed in four patients. There were no post-operative complications.
CONCLUSIONS: Wandering spleen is a rare entity and in the paediatric age group 10% cannot be salvaged for which splenectomy is the only option. Of the 90% that can be pexied, the literature has favoured the application of meshes followed by the extraperitoneal pockets and omental pouch. Laparoscopic splenopexy is feasible, with no reported conversions or complications.

INTRODUCTION

Wandering spleen is a rare condition in which the spleen has an abnormal mobility. This derives from the absence or laxity of the lineal ligaments that hold the organ in its normal position in the upper left abdomen. The spleen is suspended by only its hilar vessels and can migrate from its anatomical location, which is why the condition is also known as floating spleen or splenoptosis. The incidence of wandering spleen has not been precisely determined. It has been reported in multiparous females of the childbearing age group[1] and only in a few cases in the paediatric population. The pathophysiology of wandering spleen consists of a failure in the development of the primary ligamentous attachment of the spleen or a laxity of these ligaments due to an underdeveloped dorsal mesentery. There are no genetic factors reported for a congenital wandering spleen. An acquired condition is described in the adult literature after traumatic events or underlying conditions that may weaken the ligaments that hold the spleen.

The movement of the spleen may lead to twisting of the vascular pedicle which compromises its vascularisation and results in ischaemia and ultimately infarction. Torsion of the spleen may also draw the torsion of the distal pancreas or the stomach. Children with wandering spleen have elusive symptoms and usually present in an acute episode. Non-acute cases usually present with recurrent abdominal pain, occasionally positional probably due to spontaneous twisting and untwisting of the vascular pedicle of the spleen. Obstruction of venous return may lead to enlargement of the spleen, which presents as a palpable abdominal mass. Other presentations include bowel obstruction, pancreatitis and urinary symptoms. However, the majority of patients present with an acute abdomen after torsion of the spleen. Diagnosis is usually imagistic (supine and standing ultrasonography, Doppler studies and computed tomography scans) or during the laparoscopy. Laboratory findings may indicate the affected organs (thrombocytopenia, anaemia and high amylase and lipase levels).

Although the traditional surgical treatment of wandering spleen implied splenectomy, the current management aims at derotation and fixation of the spleen in an anatomic position. An infarcted spleen or one which does not revascularise after detorsion is deemed unviable, and therefore, requires splenectomy. A viable spleen can be repositioned in its normal location and fixated to prevent movement. Various techniques were described for splenopexy either intra or retroperitoneal. The spleen can be fixed to the abdominal wall, the diaphragm, directly or using prosthetic materials. In the rare association of a wandering spleen with gastric volvulus, simultaneous gastropexy and splenopexy can be conducted. Conventional laparotomy is challenged in recent years by the growing interest of minimal access surgery.

The first splenic laparoscopic procedure in children was reported by Tulman et al. in 1993[2] and the first laparoscopic splenopexy for wandering spleen by Hirose et al. in 1998.[3] The advantages of minimal access surgery include decreased pain medication, shorter length of stay, decreased complications and improved cosmesis, while missed accessory spleens, splenomegaly and haemorrhage raised concerns.[4] This study aimed to review the literature with regard to the management and outcomes of the laparoscopy in children with wandering spleen.

MATERIALS AND METHODS

The literature was reviewed for articles on PubMed with regard to the search terms ‘laparoscopy’, ‘wandering’, 'spleen’ and ‘children’. The inclusion criteria included articles in English only in the paediatric age group of 0–16. Articles that did not meet the inclusion criteria were excluded from the study. The primary endpoints of the analysis were the level of reporting/evidence, type of procedural options in MAS and outcomes (complications and morbidity). Data were collected in the following areas: (a) number of patients, (b) age at surgery, (c) spleen location, (d) type of procedure, (e) specific laparoscopic technique, (f) number of ports used and port placement, (g) method of fixation, (h) prosthetics material utilisation, (i) special instruments, (j) intraoperative time, (k) associated conditions, (l) post-operative morbidity and complications and (m) follow-up interval.

RESULTS

The literature search from 1998 to 2015 revealed 21 articles, of which 15 met the inclusion criteria as mentioned above. There were 13 case reports and 2 series published with the first paediatric report published in 1998. A total of 20 children were analysed for the laparoscopic procedures in wandering spleen. The study population included 11 girls and 6 boys, while the gender of the remaining 3 children was not identified. The age ranged from 2.5 to 16 years, the median age being 8 years.

Associated conditions were identified in 45% of the patients. There were three patients who presented with gastric outlet obstruction symptoms. Intraoperatively, they were found to have gastric volvulus. A nasogastric tube was inserted to decompress the stomach and gastropexy was performed. In other three cases, due to the torsion of the distal pancreas, patients presented with an acute pancreatitis signs. Splenic cysts were found in two girls: one measuring 8 cm and the other 2 cm × 4.6 cm. Both cysts were non-parasitic. One patient in this cohort had neurological pathology and the other myotonic dystrophy.

Regarding the types of laparoscopic procedures, there were 18 splenopexies and 2 splenectomies. Splenectomy was performed when the spleen was twisted around the pedicle and was non-viable after detorsion. Splenopexies were performed using a 3–5 port technique. Nearly 80% of surgeons used only three ports. The preferred setup was in 90% of cases a 10 mm optic port and 5 mm instruments. Port placement varied with the position of the spleen either in the upper or lower quadrants.

The spleen was found in the right flank (n = 1), in the mesogastrum (n = 2), in the left upper quadrant (n = 1), below the left kidney (n = 1) and in the pelvis (n = 5). The spleen location was not described for the other 10 patients. After derotation and positioning the spleen, an extraperitoneal pocket was created using a balloon device in five patients. In three patients, a special device was used and in two patients a glove finger attached to a nasogastric tube was fashioned and filled with saline. Fixation was performed using a Vicryl (polyglactin-910) mesh in 10 patients, omentum in three patients, peritoneal flaps in one patient. For additional support, in one patient the phrenicocolic ligament was plicated and in one patient absorbable spiral tackers used in adult hernia repair were used. Gastropexy was performed in four patients by suturing the anterior wall of the stomach to the peritoneum using 3/0 sutures. In two patients, derotation of the pancreas tail was warranted. In the two cases where splenic cysts were observed, the content of the cysts was first aspirated and then, the cysts were unroofed. The operative time ranged from 55 to 195 min, with a 139 min mean. There were no reported conversions. No drains were placed. The hospital stay varied from 36 h postoperatively to 7 days. There were no reported complications. The patients were reportedly followed up for at least to 2 months and for up to 4 years.

DISCUSSION

The first description of wandering spleen appeared in 1667 by Van Horne.[5] In a normal child the spleen is fixed in its normal position by the gastrosplenic ligament to the greater curvature of the stomach and the splenorenal ligament to the left kidney and posterior abdominal wall. Abnormal development of these attachments results in a spleen that can change its position in the abdomen. The spleen is either fixed only to the stomach or is only attached at the hilum by a long vascular pedicle. The cause of this is not fully determined, but two mechanisms are incriminated: a failure of fusion of the dorsal mesogastrium in the 5th or 6th week of pregnancy or a failure of foregut rotation and fusion of the dorsal mesogastrium as seen in Prune-Belly syndrome.[6]

Wandering spleen is a rare condition both in the adult and the paediatric population. The literature shows mostly isolated cases and very few series. Less than 500 cases were reported starting with 1895,[7] and these do not account for asymptomatic cases; hence, the prevalence of this condition might be higher. With regard to demographics, the population of this study is similar to the paediatric cases of wandering spleen reported by Fiquet-Francois et al.[8] regarding age. However, the laparoscopic procedures prevailed in girls, probably due to cosmetic reasons.

The reports of laparoscopic procedures for wandering spleen date almost 20 years back, which is relatively recent for minimal access surgery. Considering the acute nature of the presentation, the variable location of the spleen and the risk for injuries and bleeding, this is concordance with the development of emergency laparoscopy. Surgeons prefer an open approach when important comorbidities are present. Therefore, even though in the literature, a various conditions are associated with wandering spleen, there is little such pathology present in children who underwent minimal access surgery. One report[8] shows diaphragmatic hernia is a frequent concomitant pathologic condition and considers this and other congenital anomalies as indication for open surgery.

In the patients who were treated laparoscopically, the associated conditions are mostly connected to the physiopathology of wandering spleen itself. The association with gastric volvulus is described in various reports; although, no exact incidence was determined. The poor development of gastrosplenic ligament or lack thereof along with the suspension of the spleen by a long vascular pedicle is the suspected mechanism behind gastric rotation.[9] Not all patients with gastric outlet obstruction required gastropexies. A study by Magno et al.[10] described the symptomatology subsided after nasogastric tube placement and no gastropexy was performed.

Distal pancreas volvulus can be explained in the same manner as the gastric one. Although it is rarely described, it complicates the diagnosis of wandering spleen. After derotation and splenopexy, there is no reported evidence of pancreatic injury or complication.[1011]

Splenic cysts are also rare. They cause enlargement of the spleen and thus expedite the finding of an abdominal mass. Splenic torsion due to enlargement might be a concern. There is no increased incidence of wandering spleen in regions with splenomegaly.[12] In both cases reported in paediatric literature, the cysts were non-parasitic, and therefore, excision of the wall of the cyst was sufficient and the intact spleen was pexied. The management of wandering spleen has long been debated. Due to the risk of torsion and infarction, surgical treatment is considered even for asymptomatic cases.[7] Initially, splenectomy was preferred, but in recent years, fixation of the spleen was recommended, especially in children to prevent post-splenectomy sepsis.[5] The decision to perform splenopexy or splenectomy ultimately depends on the viability of the spleen after detorsion. Splenectomy is performed when spleen appears viable and the splenic vein is not thrombosed.[13]

In the conservative setting, minimal access surgery can flourish. As described above, the spleen can take various positions, but it is mainly found in the pelvis. Laparoscopy allows for a thorough inspection of the intraperitoneal cavity, finding and repositioning the spleen in its anatomical position without a major abdominal incision. To do this most surgeons only use a three-port access.

Various fixation techniques were imagined throughout the years. The goal was to house and anchor the spleen in the most anatomical way. Most surgeons preferred a prosthetic rather than a natural pouch. Vicryl bags were used or, where unavailable, two sheets of Vicryl mesh were tailored to cover the spleen,[4] leaving enough space for the vascular pedicle. To provide more support, Falchetti et al.[14] covered the mesh with omentum and made small serosal sacrifices of the adjacent abdominal wall. Kleiner et al.[15] describes placating the phrenicocolic ligament and stitching it to the lateral abdominal wall and to the lower margin of the mesh. Torri et al.[7] used 5 mm absorbable spirals as employed in adult hernia repair instead of conventional stitch placement making fixation extremely quick and safe. Upadhyaya et al.[1] used traction and countertraction and blunt dissection to create an extraperitoneal pouch and then pulled up the peritoneal flaps to keep the spleen in place. Extraperitoneal fixation was also favoured either by simple dissection, or by inserting a special balloon device or a glove finger and filling it with saline, and thus, mechanically creating a pouch to house the spleen. All the fixation methods used in paediatric laparoscopy for wandering spleen are presented in Table 1.

Table 1

Fixation methods in paediatric laparoscopy for wandering spleen

Author, year	Number of cases	Fixation technique
Torri et al., 2015[7]	1	Mesh pexy with metal clips and absorbable spirals to the posterolateral abdominal wall and the diaphragm
Magno et al., 2011[10]	1	Extraperitoneal mesh fixation
Fiquet-Francois et al., 2010[8]	5	three cases mesh pexy to the abdominal wall and diaphragm, one case wrapped with omentum
Okazaki et al., 2010[9]	1	Extraperitoneal fixation
François-Fiquet et al., 2009[16]	1	Extraperitoneal fixation
Rescorla et al., 2007[4]	2	Mesh pexy to the diaphragm
Falchetti et al., 2007[14]	1	Mesh pexy covered with omentum
Upadhyaya et al., 2007[1]	1	Extraperitoneal fixation with peritoneal flaps
Lacreuse et al., 2007[11]	1	Fixation to the parietal peritoneum with endostitches covered with omentum
Fukuzawa et al., 2006[17]	1	Extraperitoneal fixation
Kleiner et al., 2006[15]	1	Mesh pexy to the diaphragm using stapler, plication of phrenicocolic ligament
Martínez-Ferro et al., 2005[18]	1	Extraperitoneal fixation
Schaarschmidt et al., 2005[19]	1	Extraperitoneal fixation
Hirose et al., 1998[3]	1	Mesh pexy to the diaphragm using extracorporeal knots

Some authors[916] consider simultaneous gastropexy is required for the surgical care of wandering spleen to avoid gastric volvulus. Since their common pathophysiology lies in the failure of development of the dorsal mesentery, this may be taken into consideration. However, the number of patients who present with torsion of both the spleen and stomach is too little to allow for a generalisation. The absence of conversions and complications in children who were treated laparoscopically, and a hospital stay as short as 36 h are encouraging factors for minimal access surgery. Even though, a 60% rate of secondary splenic ischaemia was reported in children who underwent open splenopexy and required subsequent surgery,[8] there were no reinterventions in the laparoscopic managed population.

CONCLUSIONS

Wandering spleen is a rare entity and in the paediatric age group of 10% cannot be salvaged for which splenectomy is the only option. Of the 90% that can be pexied, the literature has favoured the application of meshes followed by extraperitoneal pockets and omental pouch. Laparoscopic splenopexy is feasible, with no reported conversions or complications.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.