From the Department of Pediatric Surgery, Maulana Azad Medical College, New Delhi 110 002, India.

Correspondence to: Dr. Yogesh Kumar Sarin, Professor & Head, Department of Pediatric Surgery, Maulana Azad Medical College, New Delhi 110 002, India. Email: [email protected]

Manuscript received: March 14, 2006; Initial review completed: May 9, 2006;
Revision accepted: August 21, 2006.

Abstract:

We report eventration of right hemi-diaphragm resulting in gastric volvulus of the right-sided stomach in an infant. The diagnosis of this rare association was made with contrast CT scan. Patient was initially managed with reduction of stomach, plication of right hemi-diaphragm, anterior gastropexy and Ladd’s procedure, but required re-laparotomy after two months for recurrent volvulus.

Keywords: Dextrogastria, Infant, Volvulus.

Congenital anomalies in the position or attachment of proximal portion of alimentary tube are exceedingly rare and usually occur as apart of general transposition of the viscera. Isolated dextrogastria is the rarest of all visceral transpositions and usually coexists with eventration of right hemi diaphragm. Another gastric condition known to coexist with eventration of diaphragm is gastric volvulus. But eventration of right hemi diaphragm resulting in volvulus of the right-sided stomach has never been reported hitherto. We report this association in an infant here. Another peculiarity of this case lies in the fact that rotational abnormalities of both the foregut and the midgut coexisted.

An 8-month-old male child was admitted with recurrent chest infections and non-bilious vomiting since birth, for which he was treated with antibiotics elsewhere. There was no history of cyanosis. Antenatal history was not significant. Examination revealed a chronically malnourished baby weighing 6.4 kg. Although there was no apparent distress, auscultation of the chest revealed absence of air entry in inframammary and infraaxillary region in the right side. The breath sounds on the left side and heart sounds were normal. Liver was palpable three-finger breadth below the lower coastal margin. Rest of the abdominal examination was unremarkable.

The hematological and biochemical investigations including blood gas studies were normal. Chest roentgenogram showed elevated right hemi-diaphragm with haziness and a gas shadow below it. Upper GI contrast study showed right-sided stomach with normally positioned duodenum. Contrast enhanced CT scan of abdomen showed right-sided stomach lying above the liver and below the markedly elevated right hemi-diaphragm.

Exploratory laparotomy performed through right sub-costal incision revealed the right-sided stomach going behind the normally located liver under the membranous, elevated right hemi-diaphragm; it was normally rotated about its sagittal plane. Malrotation of gut was evident because of the Ladd’s bands and the cecum positioned in the left lumbar region. Reduction of stomach in its normal position, plication of right hemidiaphragm, anterior gastropexy and Ladd’s procedure was done. Patient was discharged on the 8th post-operative-day.

Patient remained asymptomatic for next two months. He turned later with episodic postprandial non-bilious vomiting of two-day duration. The upper GI contrast study was repeated, which revealed gastric volvulus. The patient was re-explored; gastric volvulus was corrected, revision anterior gastropexy and Nissen’s fundoplication were performed. The postoperative period was uneventful.

The child had been asymptomatic on two and a half years follow up.

Total situs inversus is moderately uncommon occurring in 1:6000 to 1:8000 cases. Partial or isolated visceral transposition is also rare and most often involves the heart. Isolated dextrogastria is rare of all visceral transpositions and can occur in two distinct forms(1), type-1 stomach may lie completely behind the liver, but the chest appears normal and type-2 stomach lie above the liver in association with eventration of diaphragm and may simulate abscess, hydropneumothorax, right-sided hiatus hernia or other pathology of right lung base. Only isolated cases of either type are reported. Type-1 is apparently even rarer than type-2 (incidence <1:100000).

Generally, the dextrogastria produces no symptoms and is first noted as incidental finding on chest roentgenogram, liver scans, during abdominal surgeries, on autopsies. Most of the cases in existing literature have been reported in adulthood(1-6). There had been only three cases of dextrogastria in English literature reported to present in infancy hitherto(7-9). One of the these cases was of duodenal web where dextrogastria was incidentally detected on upper GI contrast study(9); in other two cases, the symptoms were that of recurrent vomiting and developmental retardation. In other patient, recurrent chest infections were also a predominant feature.

An underlying eventration of hemidiaphragm is known to occur in about one-sixth of all the children presenting with gastric volvulus(10). The stomach usually is relatively fixed at the esophageal hiatus at pylorus and is prevented from abnormal rotation by its ligamentous attachment. Absence or attenuation of these anatomical anchors results in the mobility of stomach within the wide sub diaphragmatic space under the eventrated diaphragm and makes it potential for gastric volvulus. Mesentero-axial is the most common type of gastric volvulus observed in the pediatric age group; the same was the finding in our case.

The coexistence of rotational abnormalities of both foregut and midgut was another peculiarity in this case. Similar coexistence has been reported only twice before in the English literature(3,7).

Eventration of the diaphragm associated with gastric volvulus always warrants surgery. Plication of the diaphragm with anterior gastric fixation via gastropexy or gatsrostomy and abdominal exploration for associated gastrointestinal anomalies is the most appropriate approach. Though the recurrences following surgical correction by gatsrostomy fixation for volvulus are not recorded, the recurrences have been known to occur gastropexy. We have treated the recurrence by gastropexy with Nissen’s fundoplication as we keeping the possibility of GERD in mind.

Contributors: YKS managed the patient, redrafted the paper, and shall stand as guarantor. NGN reviewed the literature wrote the initial draft of the manuscript.

Funding: None.

Competing interests: None.