A case of ‘an upside down stomach’
M. Bawa, S. Garge, S. Khanna, R. P. Kanojia & K. L. N. Rao
Hernia The World Journal of Hernia and Abdominal Wall Surgery ISSN 1265-4906 Volume 16 Number 4 Hernia (2012) 16:489-492 DOI 10.1007/s10029-010-0763-z
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Author's personal copy Hernia (2012) 16:489–492 DOI 10.1007/s10029-010-0763-z
CASE REPORT
A case of ‘an upside down stomach’ M. Bawa • S. Garge • S. Khanna • R. P. Kanojia K. L. N. Rao
•
Received: 6 September 2010 / Accepted: 21 November 2010 / Published online: 14 December 2010 Ó Springer-Verlag 2010
Abstract Neonatal paraesophageal hernia with intrathoracic gastric volvulus is very rare in the newborn period. We report a case of a 2-day-old term neonate who presented with an antenatal diagnosis of paraesophageal hernia. We discuss the diagnosis and management with classical radiological features including an antenatal diagnosis of this extremely rare surgical cause of neonatal nonbilious emesis. Keywords Neonatal Paraoesophageal hernia Gastric volvulus Prenatal
Introduction Congenital paraesophageal hiatal hernia is a rare condition in infants, and more so when associated with volvulus of the intrathoracic stomach. An early diagnosis and intervention are imperative for the successful management of this disease [1]. We report a case diagnosed antenatally with classical postnatal radiographic presentation along with anatomical interpretation of the clinical and radiographic features. A prenatal diagnosis helped us to intervene early and was the key to patient survival.
M. Bawa S. Garge S. Khanna R. P. Kanojia (&) K. L. N. Rao Department of Pediatric Surgery, Advanced Pediatric Center, Post Graduate Institute of Medical Education and Research (PGIMER), Sector 12, Chandigarh 160012, India e-mail:
[email protected]
Case report A 2.4 kg neonate, born at full term by normal vaginal delivery to a primigravida was referred to us within a few hours of birth with an antenatal suspicion of congenital diaphragmatic hernia or a hiatal hernia. An antenatal ultrasonography performed at 32 weeks of gestation showed a cystic structure in the right hemithorax (Fig. 1). There was moderate polyhydramnios. On physical examination, the baby was active with significant frothing and excessive salivation. Chest auscultation was normal and no added sounds could be appreciated. The abdomen was scaphoid and soft. The nasogastric tube could not be passed beyond 14 cm. Hemogram, renal function tests, liver function tests and serum electrolytes were within normal limits. Plain chest X-ray, anteroposterior (AP) and lateral view, revealed a large, almost spherical, radiolucent shadow in the right posterior mediastinum. The nasogastric tube was seen coiled in the mediastinum, which made us suspect a diagnosis of gastric volvulus along with hiatal hernia (Fig. 2). An upper gastro-intestinal (GI) contrast series confirmed a paraesophageal hernia with intra-thoracic gastric volvulus (Fig. 3). The patient was taken up for surgery after adequate hydration. Exploratory laparotomy revealed a wide esophageal hiatus (Fig. 4) through which the entire stomach had herniated into the thorax along with an organo-axial volvulus. Both the hemidiaphragms were well developed. The stomach was reduced into the abdominal cavity and the hiatal defect was repaired by approximating the crura. No antireflux procedure was performed. An anterior gastropexy using a gastrostomy tube was carried out to avoid recurrence. The patient was kept on nasogastric tube drainage, intra-venous fluids and antibiotics for 2 days. The baby had an uneventful recovery and was discharged on the 6th postoperative day with gastrostomy in situ. One week
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Fig. 1 Antenatal ultrasound picture showing cystic structure in the right hemithorax
Fig. 3 Upper gastrointestinal (GI) contrast showing herniated cardia of stomach with confirmation of volvulus
Fig. 2 Plain X-ray with nasogastric tube showing the alfa loop configuration typical of stomach volvulus
later the gastrostomy was removed. At follow up after 1 month, the baby is thriving well without any history of regurgitation of feeds. A follow up esophagogram and milk scan is planned at 6 months of age.
Discussion Congenital paraesophageal hiatus hernia in the neonatal period is a rare condition, which may or may not be symptomatic [1–4].The true incidence in the pediatric population is unclear. Neonatal primary intrathoracic gastric volvulus is extremely rare, with fewer than 20 cases reported in the literature [2, 3]. It has been reported
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Fig. 4 Intraoperative picture showing large esophageal hiatus through which stomach was pulled down
sporadically with general hiatal diaphragmatic hernia. A review of the literature by Cribbs et al. revealed a total of 581 cases, of which 252 (43%) children presented in an acute fashion. Of the acute cases, 7% were associated with paraesophageal hernia. Out of all these cases, about 21% presented in the 1st month of life [4]. Embryologically, paraesophageal hernia in the neonate is secondary to the failure of the dorsal musculature to form the posterior mesoderm, during the development of the
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lumbar part of the future diaphragm, which takes its origin from the mesodermal cells around the aorta—the area to which the gastro-esophageal junction is attached [3, 5, 6]. The most common structure to herniate is the fundus of the stomach [1, 2, 4]. At all stages, there is a complete parietal peritoneal sac in which the herniated viscera reside behind the heart, in front of the esophagus and in the right posteroinferior mediastinum [2, 4]. Although a paraesophageal hiatal hernia is rare in childhood, it has potential complications, including incarceration, strangulation, a complete gastric herniation with organoaxial volvulus (upside-down stomach), and perforation of the herniated viscera, both in children and adults [7–11]. Gastric volvulus is the most common and serious complication of intrathoracic upside-down stomach (huge paraesophageal hiatal hernia) [1]. This results from rotation of the fundus into the right pleural cavity along the organoaxial axis defined by the phreno-esophageal membrane at the hiatus and the retroperitoneal attachment of the first part of the duodenum [4]. This entity may result in a misdiagnosis of a right-sided congenital diaphragmatic hernia, eventration of the diaphragm, lobar pneumonia, pneumatocele, pneumothorax, or pleural effusion [7]. The insertion of a nasogatric tube may be helpful in confirming the diagnosis. In the index case, the inability to pass the nasogastric tube beyond 14 cm, and the aspirates being gastric with no bilious tinge raised a suspicion of volvulus. A plain chest X-ray showing a retrocardiac mass with a horizontal air–fluid level can be diagnostic. If complicated by volvulus of the stomach, each loculus of the stomach may be apparent as a gas-distended viscous, one above the diaphragm and to the right, and the other below and to the left. Upper GI contrast studies can act as a clincher for the diagnosis and also to rule out any malrotation, which is also common in these cases [7, 9]. The index case was prenatally diagnosed to have diaphragmatic hernia because of a retro cardiac cystic structure that had varying dimensions during ultrasound. Also, the gastric bubble was not visible in the abdomen. After in utero identification of an intrathoracic stomach, a diagnosis of a congenital Bochdalek diaphragmatic hernia was first considered. This condition has substantial morbidity and mortality due to associated pulmonary hypoplasia. In contrast, a congenital hiatal hernia is less severe and seems to be very rare, with only four published cases involving prenatal sonographic detection [12–15]. Sonographic criteria for the diagnosis of a congenital hiatal hernia were first proposed by Bahado-Singh et al. [12] and refined by Ruano et al. [15] in 2004 as a hypoechoic image in the posterior mediastinum just behind the heart and anterior to the vertebral body; absence of a mediastinal shift or pleural or pericardial effusion; an abnormal location of the stomach, which can be identified in the abdomen but in a
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median position; and up-and-down movements of the herniated stomach through the enlarged hiatus into the thoracic cavity. In infancy and childhood, the current surgical treatment in congenital para oesophageal hernia consists of abdominal exploration, proper inspection of the GI tract, reduction of all hernial contents, careful dissection of the gastro-esophageal junction, narrowing of the diaphragmatic crura, partial or complete excision of the hernial sac, and an anti-reflux procedure. During the procedure, care should be taken not to injure the vagus nerve; complete hernial sac excision should be attempted since a residual sac may lead to fluid accumulation and perhaps to infection. Some authors recommend an anterior gastropexy to lessen the possibility of recurrence [16]. A similar procedure was performed in this patient along with an anterior gastropexy. The report describes a case of an antenatally diagnosed case of paraesophageal hernia complicated by volvulus of the herniated stomach diagnosed by an upper GI series, managed by laparotomy with reduction of intra thoracic contents along with closure of hiatus with a gastropexy. The prenatal diagnosis led us to admit the patient early, which helped in successful treatment of the case without any complications. Conflict of interest
None.
References 1. Stolar CJ (2003) Congenital diaphragmatic hernia. In: O’Neill JA Jr (ed) Principles of pediatric surgery, 6th edn. Mosby, St Louis, pp 931–954 2. Kane TD, Harmon CM, Georgeson KE (2003) Laparoscopic reduction of gastric volvulus and repair of giant hiatal hernia in a neonate. J Laparoendosc Adv Surg Tech 7:323–326 3. Mutabagani KH, Teich S, Long FR (1999) Primary intrathoracic gastric volvulus in a newborn. J Pediatr Surg 34:1869–1871 4. Cribbs RK, Gow KW, Wulkan ML (2008) Gastric volvulus in infants and children. Pediatrics 122:752–762 5. Kleitsch WP (1958) Embryology of congenital diaphragmatic hernia, I. esophageal hiatus hernia. Arch Surg 76:868–873 6. Wells LJ (1954) Development of the human diaphragm and pleural sacs. Contrib Embryol Carneg Inst 35:107–137 7. Senocak ME, Bu¨yu¨kpamukc¸u N, Hic¸so¨nmez A (1990) Massive paraesophageal hiatus hernia containing colon and stomach with organo-axial volvulus in a child. Turk J Pediatr 32:53–58 8. Al-Arfaj AL, Khwaja MS, Upadhyaya P (1991) Massive hiatal hernia in children. Eur J Surg 157:465–468 9. Lister J (1972) Hiatus hernia in children. Postgrad Med J 48:501–506 10. Stiefel D, Willi UV, Sacher P et al (2000) Pitfalls in therapy of upside-down stomach. Eur J Pediatr Surg 10:162–166 11. Maruyama T, Fukue M, Imamura F et al (2001) Incarcerated paraesophageal hernia associated with perforation of the fundus of the stomach: report of a case. Surg Today 31:454–457
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Author's personal copy 492 12. Bahado-Singh RO, Romero R, Vecchio M, Hobbins JC et al (1992) Prenatal diagnosis of congenital hiatal hernia. J Ultrasound Med 11:297–300 13. Chacko J, Ford WD, Furness ME (1998) Antenatal detection of hiatus hernia. Pediatr Surg Int 13:163–164 14. Ogunyemi D (2001) Serial sonographic findings in a fetus with congenital hiatal hernia. Ultrasound Obstet Gynecol 17:350–353
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Hernia (2012) 16:489–492 15. Ruano R, Benachi A, Aubry MC et al (2004) Prenatal sonographic diagnosis of congenital hiatal hernia. Prenat Diagn 24:26–30 16. Allen B, Thompkins R, Mulder DG (1991) Repair of large paraesophageal hernia with complete intra-thoracic stomach. Am Surg 57:642–647
