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Journal of Indian Association of Pediatric Surgeons, Vol. 13, No. 1, January-March, 2008, pp. 18-21 Original Article Diaphragmatic crural eventration Sivakumar K Department of Pediatric Surgery, SAT Hospital, Medical College, Trivandrum; Medical College, Kottayam Code Number: ip08006 Abstract Aim: We evaluated patients with gastric volvulus secondary to diaphragmatic pathology. Keywords: Diaphragmatic defects, gastric volvulus, right crus of diaphragm Introduction Gastric volvulus is relatively rare in children. The incidence peaks in those in the age group of 40-50 years. Approximately, 20% occur in children and of this majority occur in infancy. [1] When one encounters a case of gastric volvulus, he/she should always look for associated the pathology of stomach, gastric ligaments, spleen, colon and for defects in diaphragm. [2] Two thirds of the cases are associated with diaphragmatic pathology. [1] The usual diaphragmatic pathologies are eventration diaphragm, congenital diaphragmatic hernia, rupture of diaphragm, hiatus hernia [2] Morgagni′s hernia [3] and to add one more type of diaphragmatic defect, the diaphragmatic crural eventration, through this paper. [4],[5] The objective of this study is to identity the diaphragmatic pathology observed in cases of gastric volvulus and to highlight the new terminology "diaphragmatic crural eventration" and its embryo pathogenic correlation with the gastric volvulus. Materials And Methods The cases of gastric volvulus the author had personally encountered during the period of 1997 to 2006 were preoperatively and peroperatively assessed and analyzed in terms of age, sex, clinical features, type of gastric volvulus and the associated pathology of diaphragm and ligaments of stomach.Results There were eight cases of gastric volvulus during this period. Male: Female ratio was 5:3. Five patients were infants. The mean age of presentation was 24.6 months (The youngest one was 14 days of age and oldest one was 9 years of age). Clinical features: The presentation was with acute symptoms in six cases. The acute symptoms and signs observed were as follows: epigastric pain, vomiting, retching, epigastric fullness with mass, respiratory distress, pneumoperitoneum, bleeding per rectally and shock. In rest of the two cases, the presentation was with chronic symptoms such as intermittent cry or irritability, recurrent non-bilious vomiting and failure to thrive. Investigations: Acute cases were diagnosed by plain X-ray abdomen. A distended stomach with fluid level associated with diaphragmatic pathology aroused the suspicion of volvulus and then confirmed by upper GI contrast study. Chronic cases were diagnosed by upper GI contrast study when done as part of the evaluation of recurrent non-bilious vomiting. None of them had CT scan or other imaging techniques. Operative findings: All the patients were operated except one patient who died preoperatively due to aspiration pneumonitis. This patient had chronic presentation as the failure to thrive. (Birth weight was 3.5 kg, and at 2 months of age, the weight was 2.3 kg.) Type of Gastric volvulus : It is determined by analyzing mainly the contrast pictures and peroperative findings. Organoaxial type volvulus is observed in six cases and mesenteroaxial type is observed in one case and not defined in one case. Stomach : It was gangrenous in two cases and both of these patients died postoperatively. The gastric ligaments were very much attenuated in five cases and absent in two cases. Diaphragmatic pathology : In all the six cases with acute presentation, there was associated diaphragmatic pathology. The diaphragm was normal in the two cases with chronic presentation. Classical left hemidiaphragm eventration was observed in two cases. Late-presenting congenital diaphragmatic hernia was observed in another case. In the rest of the three cases, the right crus of diaphragm were absent. Defect of right crus of diaphragm (diaphragmatic crural eventration ): This was noted in three patients. (age: 8 months, 1½ years and 4½ years; male/female ratio - 1:2.) All of them presented with acute symptoms. Case 1 Case 2 Case 3 Discussion Stomach is held in its normal position by its natural ligaments, namely, gastrohepatic, gastrophrenic, gastrosplenic and gastrocolic ligaments. Since these ligaments are related to diaphragm, spleen and colon, any problems of these ligaments as such or the adjacent diaphragm spleen or colon can produce volvulus. [2] Gastric volvulus is defined as abnormal rotation of all or part of stomach for more than 180° that may lead to closed-loop obstruction and possible strangulation. [1] Berti first described it in 1866. [2] In 1899, Oltmann first reported the pediatric case. [6] In 1904, Borchardt described the classical triad of severe epigastric pain, retching or vomiting and inability to pass nasogastric tube. [2] However, one need not find the classical triad in all the cases. Most of the studies show that males are affected more than females. [2],[7] This was same also in this study; however, the specific entity of diaphragmatic crural eventration was found to be greater in females. According to the axis of rotation, gastric volvulus is classified into the following: organoaxial (60%) mesenteroaxial (30%), mixed type (nearly 2%) and the rest are unclassified. [1],[2] Organoaxial is more common and usually associated with diaphragmatic pathology, and there is 25% chance of strangulation. Perforation of stomach can also occur. [8] Splenic pathology such as wandering spleen [9] or asplenia [10] may be associated with increased incidence of gastric volvulus. Although during this period we had a case of torsion of wandering spleen, he never presented with the features of gastric volvulus before splenopexy or even during the follow-up after 10 years. In situations where diaphragmatic pathology is absent, the absence or the laxity of the ligament of stomach was found to be responsible. Dalgaard first noted this in cadaver. [11] Eventration of diaphragm is the commonest associated diaphragmatic pathology, and it is followed by congenital diaphragmatic hernia, which were well described in literature. In this study, there were three cases, where gastric volvulus was associated with attenuated right crus of diaphragm. This association has got a good embryological backup [Figure - 4]. The dorsal mesoesophagus and mesogastrium play an important role in the proper development of the right crus of the diaphragm, gastrophrenic, gastrosplenic, spleenorenal, gastrocolic ligaments and lesser sac. [12] Therefore, a defect in the development of dorsal mesoesophagus and mesogastrium leads to defective right crus and gastric ligaments. The right crus will get attenuated to form a thin membrane to the right of esophageal hiatus from its level of vertebral origin and protrude to the right hemithorax, and this along with the defective gastric ligaments predisposes to the gastric volvulus. This entity can be clinically suspected when a plain X-ray chest and abdomen show right-sided partial eventration, where the partial eventration is located to the right of cardiac shadow in the anteroposterior film and posteriorly near vertebra in the right lateral film. The content is stomach and its status is better delineated by upper GI contrast study. While operating such cases, one has to plicate the defect and one must be careful about the pericardium and vagus nerve. The esophageal hiatus must be reconstructed. Pyloroplasty is not necessary, but it should be done if one damages the vagus nerve. The outcome is very good and depends on the viability of the stomach. Although it is embryologically well-established that the right crus of the diaphragm develops from dorsal mesoesophagus, a defective right crus of diaphragm is not reported thus far as a separate entity. The chart for all the diaphragmatic defects [13] does not mention the defects of the crus of diaphragm. In situation of paraesophageal hiatus hernia, the hiatus is found to be sufficiently wide for stomach to roll or slide in; however, there is no mention of the crural pathology. Congenital paraesophageal hiatus hernia by means is not well established. [13] The correlation of the right crus defect and gastric volvulus is important and should be grouped as a separate entity called diaphragmatic crural eventration. Paraesophageal hiatus hernia should be reserved for the acquired condition seen in adults since its congenital nature is not established. Hence, the author recommends a closer examination for this crural defect while operating a case of gastric volvulus or hiatus hernia in children. Acknowledgement I thank Prof. S. Hariharan, Prof. R. R. Varma, Prof. R. Hema, Prof. S. Rajendran and colleagues of SAT Hospital Medical College Trivandrum and Medical College Kottayam for guidance and support. References
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