Laparoscopic management of gastric volvulus, diaphragmatic eventration and wandering spleen in a child

Discussion

GV is a rare condition causing a pathological rotation of the stomach, greater than 180° along its longitudinal (organoaxial) axis or along an axis joining its mid-lesser and greater curvatures (mesenteroaxial). This rotation may lead to gastric obstruction and strangulation that can further manifest as gangrene, perforation and necrosis.4 This condition has been found to be predominant in the older age group, that is, above 50 years of age, with a high mortality rate of 42%–56%.5 If this condition manifests, it remains potentially fatal in children. GV can be either primary or secondary, the latter being more common. Primary GV is associated with congenital defects such as absence or laxity of the supporting structures of the stomach (gastrocolic, gastrohepatic, gastrosplenic, gastrophrenic ligaments) or with abnormal gastric distention. Alternatively, secondary volvulus may arise as a result of disorders of normal gastric anatomy or function or abnormalities of adjacent organs, particularly the diaphragm and spleen.6 This suggests an expected association of diaphragmatic eventration and GV in our patient.

GV classically presents as an acute abdominal emergency or symptoms of chronic upper gastrointestinal discomfort. The diagnostic clinical features of Borchardt’s triad include vomiting, epigastric pain and an inability to pass an NGT.7 However, some reports suggest that dysphagia, gastric reflux and chest pain remain common.8 The insertion of NGT or endoscopic decompression can help in relieving these symptoms.

The diagnosis of GV remains a challenge since no one laboratory value or a set of values is sensitive enough to exclude the diagnosis.5 A chest radiograph demonstrates a retrocardiac, air-filled mass, whereas abdominal films show an increased soft-tissue density in the upper abdomen consistent with a distended fluid-filled stomach.6 The abdominal X-ray showing a spherical stomach and double air-fluid level may indicate GV; however, these findings may be absent in some cases and further studies may be employed to confirm the diagnosis. Recent studies advocate using barium swallow and abdominal CT scans that provide accurate information about the anatomy and position necessary to hasten surgical interventions.9 GV was traditionally managed by laparotomy and anterior gastropexy.

In our case, the patient also presented with a WS. WS is a rare condition where the spleen is displaced from its anatomical position, that is, from the left hypochondrium of the abdomen to an unusual site in the abdominal cavity. Although it is seen commonly in women, typically in the reproductive age group (as a result of acquired ligamentous laxity), it has also been described in the paediatric population, especially in males, in the first year of life. Reports suggest that this may be due to failure in the development of splenic ligaments, which help anchor it to the stomach, diaphragm, colon and retroperitoneum. As a result, the spleen is suspended entirely with the help of its vascular pedicle, increasing the risk of torsion.2 Additionally, an association with other congenital malformations such as Prune-Belly syndrome, renal agenesis, eventration of the diaphragm and GV has been described.2 In cases of diaphragmatic defects, the spleen is typically expected to herniate intrathoracically through the defect, in contrast with the caudally situated spleen seen in our patient. The clinical presentation of a WS can be very ambiguous in young patients. The diagnosis is usually incidental, but in acute cases, the patient may present with an acute abdomen secondary to an acute splenic torsion, with or without any evidence of organomegaly. Abdominal Doppler ultrasonography is the first step in diagnosing a WS and demonstrating the abnormal position.10 Although patients with mild symptoms and minimal complications can be managed conservatively, studies have shown that they usually develop splenic torsion, splenic injury and injury to the other organs, making non-surgical therapy inadvisable.11

Although GV and WS are usually single and rare malformations, a few authors consider them to be frequently reported individually in association with diaphragmatic defects.10 To the best of our knowledge, only four cases have been reported that associated all three malformations.1 3 10 12 Very rarely the three aforementioned have been further complemented with an ectopic kidney.1 Currently, the mechanism of an ectopic kidney remains unclear. The association between an ascended kidney and diaphragmatic eventration varies among reports where the patient may remain asymptomatic and the diagnosis is purely incidental in most cases.13

In the index case, there was an ascended kidney in combination with GV and WS with eventration of the left hemidiaphragm, making it a unique presentation, which makes this case noteworthy. Such cases are traditionally managed by laparotomy in the paediatric age group. On the contrary, our case was managed laparoscopically with minimal scarring and faster recovery which is currently the accepted method of treatment. Endoscopic derotation and percutaneous endoscopic gastrostomy have been described earlier in patients with isolated GV.14 15 There are case reports of adult patients managed laparoscopically; however, our patient appears to be the youngest one to be treated similarly with this unusual combination.16 17 The authors advocate laparoscopic reduction and repair for such a presentation for reduced morbidity and enhanced recovery.