Find best premium and Free Joomla templates at GetJoomlaTemplatesFree.com

Large Congenital Paraesophageal Hernia and the Risk of Intrathoracic Gastric Volvulus

Ahmed H. Al-Salem¹, Hilal Matta²

¹Department Of Pediatric Surgery, Dar Alafia Medical Company, Dammam, Saudi Arabia

²Division Of Pediatric Surgery, Department of Surgery, Tawam Hospital, Al-Ain, Abu Dhabi, United Arab Emirates

 

Correspondence:

Ahmed H. Al-Salem

P. O. Box 61015 Qatif 31911

Saudi Arabia

E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

Phone: 966546102999

 

Abstract

Aim: Congenital paraesophageal (CPH) is rare in infants and children. It can unusually be large with herniation of most of the stomach which can lead to intrathoracic gastric volvulus. This report describes our experience with infants and children with large CPH.

Patients and Methods: Twelve infants and children with large CPH, three of them presented with intrathoracic gastric volvulus were evaluated. Their medical records were reviewed for age at diagnosis, sex, clinical features, diagnosis and management. Results: Between 1992 and 2012, twelve infants and children with large CPH were treated. There were 7 males and 5 females. Their age at diagnosis ranged from 2 days to 2.5 years (mean 16.3 months). Two presented acutely, immediately after birth with respiratory distress secondary to a large CPH. One of them had intrathoracic gastric volvulus. Four presented with recurrent chest infection and failure to thrive while the remaining three had repeated attacks of vomiting. Another patient was a case of repaired esophageal atresia and tracheoesophageal fistula. He presented at the age of 2.5 years with recurrent attacks of cough and vomiting for one-year duration and was found to have a large paraesophageal hernia. Two patients were sisters and both of them presented with recurrent chest infection and failure to thrive. At the time of presentation, both had large CPH with intrathoracic gastric volvulus.

Conclusions: CPH is rare in the pediatric age group and sometimes it is unusually large with herniation of most of the stomach which may result in intrathoracic gastric volvulus. Awareness of this and early diagnosis and treatment are important to avoid subsequent morbidity and mortality.

Keywords: paraesophageal hernia, infants and children, familial, fundoplication

doi: 10.21614/jpss.11.1.6

Citation: J Pediatr Surg Spec. 2017;11(1):6-10

 

Introduction

Paraesophageal hernias are rare in infants and children and most of the reported cases are acquired following Nissen's fundoplication for gastroesophageal reflux [1]. This is seen more commonly in neurologically impaired children and in those where repair of the crura was not done at the time of fundoplication. Congenital paraesophageal hernia on the other hand is very rare in the pediatric age group and unusually can be large resulting in herniation of most of the stomach with the potential risk of intathoracic gastric volvulus [2, 3, 4]. This report describes our experience with twelve infants and children with unusually large pararesophageal hernia with herniation of most of the stomach in to the chest and intrathoracic gastric volvulus in three of them.

Patients and Methods

The medical records of all patients admitted to our hospital with the diagnosis of large paraesophageal hernia were retrospectively reviewed and the following information was obtained: age at diagnosis, sex, presenting symptoms, method of diagnosis, treatment and outcome.

Results

Between 1992 and 2012, twelve infants and children with the diagnosis of large paraesophageal hernia were treated at our hospital. There were 7 males and 5 females. Their age at diagnosis ranged from 2 days to 2.5 years (mean 16.3 months). Two presented acutely, immediately after birth with respiratory distress and repeated attacks of vomiting secondary to a large CPH. One of them had an upper contrast study which showed a large paraesophageal hernia with intrathoracic gastric volvulus (Fig. 1A and 1B). He underwent an emergency laparotomy which showed a large CPH with herniation of most of the stomach into the chest and intrathoracic gastric volvulus. The stomach was reduced and was found to be congested but no evidence of necrosis. Repair of the crura was done and gastropexy. The second patient had an upper contrast study which showed a large paraesophageal hernia with herniation of most of the stomach into the chest. He was operated on and repair of the crura was done as well as gastropexy. Four patients presented with recurrent chest infection and failure to thrive while the remaining three had repeated attacks of vomiting with fullness and pain in the epigastrium in one of them. Three of them had repair of the paraesophageal hernia and Nissen’s fundoplication while the remaining four had repair of the paraesohageal hernia and gastropexy. One of them had an associated extralobar pulmonary sequestration which was diagnosed during CT-scan evaluation (Fig. 2). He underwent repair of the paraesophageal hernia and gastropexy. The extralobar sequestration was excised at the same time through a transabdominal approach. Two of them had CT-scan which showed a large paraesophagaeal hernia (Fig. 3A and 3B). Another patient was a case of esophageal atresia and tracheoesophageal fistula who had repair when he was two days old. He presented at the age of 2.5 years with recurrent attacks of cough and vomiting of one-year duration. He was found to have a large paraesophageal hernia on barium meal. He underwent upper gastrointestinal endoscopy which showed para-esophageal hernia and mild esophagitis with no stricture or stenosis. He had repair of the para-esophageal hernia and Nissen’s fundoplication.

Figure 1A and 1B. Barium meal showing a large paraesophageal hernia with herniation of the stomach into the chest and intrathoracic gastric volvolus.

11.1 1 1

Figure 2. CT-scan showing a large paraesophageal hernia with herniation of stomach and bowel loops. There is also an associated pulmonary sequestration.

11.1 1 2

Figure 3A and 3B. CT-scan showing a large congenital paraesophageal hernia with herniation of the stomach and bowel loops into the chest.

11.1 1 3

Two of our patients were sisters and both of them presented with recurrent chest infection and failure to thrive. A 3-month-old female was referred to our hospital with a history of repeated attacks of vomiting of one-week duration. Prior to this, she was complaining of recurrent chest infection and failure to thrive. Two days prior to presentation, the vomiting was coffee ground. Her chest X-ray and barium meal showed a large paraesophageal hernia with an intrathoracic gastric volvulus. She underwent an emergency surgery and was found to have a large right paraesophageal hernia with a sac containing almost the whole stomach. The contents were reduced and the stomach was found congested but viable. The defect was repaired and gastropexy was also added. The second patient was 1.5-year-old female who was admitted to the hospital with chest infection and failure to thrive. Her chest X-ray prior to presentation showed herniation of bowel loops into the chest and current chest X-ray showed stomach herniation with double bubble appearance suggestive of intrathoracic gastric volvulus (Fig. 4A and 4B). Her barium meal showed herniation of most of the stomach into the chest with intrathoracic gastric volvulus. She was operated on and found to have a large para-esophageal hernia with herniation of most of the stomach as well as part of the transverse colon, small bowel loops and part of the left lobe of the liver. The contents were reduced, and the stomach was found to be congested but viable. The defect was repaired and Nissen's fundoplication was also added.

Figure 4. (A,B) Chest X-rays showing large paraesophageal hernia with stomach and bowel herniation into the chest in one and herniation of stomach with double bubble appearance suggestive of intrathoracic gastric volvulus.

11.1 1 4

Discussion

Hiatal hernias are classified into two types: a sliding hiatal hernia and a paraesophageal hernia. Paraesophageal hernia occurs when the stomach protrudes laterally through the esophageal hiatus into the thoracic cavity while the gastroesophageal junction remains in its normal intra-abdominal anatomic position. This however may not be the case in the pediatric age group where in some cases of paraesophageal hernia, most of the stomach herniates into the thoracic cavity with the gastroesophageal junction lying in the chest [2-5]. These cases may represent a combined type of sliding and paraesophageal hernias or in the pediatric age group congenital paraesophageal hernia is a distinct entity, different from their adult counterpart. Another distinguishing feature of pediatric paraesophageal hernia is that sometimes the defect is large allowing other intra-abdominal organs such as small and large intestines to herniate into the chest together with the stomach through the esophageal hiatal opening.

Paraesophageal hernias are relatively rare and constitutes about 3.5%-5% of all hiatal hernias with a female preponderance (M: F 1:4) [6]. One of the largest series of paraesophageal hernia in children was that reported by Karpelowsky et al. from the Red Cross War Memorial Children's Hospital in South Africa [5]. They reported 59 children treated over a 42-year period. Over a period of 20 years, we treated 12 infants and children with large paraesophageal hernia. Congenital paraesophageal hernia must also be differentiated from the purely intrathoracic stomach, an entity that is known to be associated with a short esophagus [7, 8, 9].

Paraesophageal hernias in the pediatric age group are divided into congenital and acquired and irrespective of the type whether congenital or acquired they are relatively rare. The vast majority of paraesophageal hernias are however acquired commonly seen following Nissen's fundoplication for the treatment of gastroesophageal reflux. The exact etiology of congenital paraesophageal hernia is not known. It is postulated that congenital paraesophageal hernia is secondary to embryonal developmental defects in the lumbar component of the diaphragm leading to defective right crus of the diaphragm [6]. A familial occurrence of hiatal hernia was first suggested in 1939 [9]. Since then there have been several reports documenting the occurrence of hiatal hernia among siblings and an autosomal dominant mode of inheritance was suggested [10]. This was not the case for congenital paraesophageal hernia where most of the reported cases occur sporadically. There is however a very limited number of reports describing familial paraesophageal hernias including three pairs of affected siblings [6]. Two of our patients were siblings and represent the fourth pair to be reported with familial paraesophageal hernias. This unusual familial occurrence supports a genetic predisposition to the development of congenital paraesophageal hernia. One of our patients had repair of esophageal atresia and tracheoesophageal fistula and this may have subsequently contributed to the development of paraesophageal hernia.

The presentation of children with congenital paraesophageal hernia is variable but usually they present with recurrent chest infection or vague gastrointestinal symptoms. The severity of these symptoms depend on the size of the hernia defect and the extent of stomach herniation into the chest. Awareness of this is important as congenital paraesophageal hernias are known to be associated with potentially lethal complications such as intrathoracic gastric volvulus with partial or complete gastric obstruction, strangulation and perforation [3]. It is also of importance to note that large congenital paraesophageal hernia can present at or soon after birth with respiratory distress that can be confused with the more common congenital posterolateral diaphragmatic hernia. Two of our patients presented immediately after birth with respiratory distress and vomiting and were found to have large paraesophageal hernias. One of them had an upper contrast study which showed a large paraesophageal hernia with intrathoracic gastric volvulus. Two of our patients were sisters who were suffering from repeated attacks of chest infection and failure to thrive presented acutely with large congenital paraesophageal hernia and intrathoracic gastric volvulus. Awareness of this and early diagnosis and treatment are important to avoid subsequent morbidity and mortality.

Gastric volvulus is an abnormal rotation of the stomach leading to partial or total obstruction and according to the axis of rotation it is classified into organoaxial, mesentericoaxial and mixed [11-14]. In mesenterico-axial volvulus, the stomach rotates around an imaginary axis passing through the greater and lesser curvatures, while in organo-axial, the stomach rotates around an imaginary axis passing between the esophago-gastric junction and the pylorus [11]. Organo-axial volvulus is the commonest. The mixed variety is extremely rare and difficult to diagnose both radiologically and intra-operatively. Gastric volvulus is rare in the pediatric age group, and depending on its presentation, it is also classified into acute where urgent surgical treatment is mandatory and chronic. Based on its location, gastric volvulus is classified into intraabdominal and intrathoracic [11, 14]. Intrathoracic gastric volvulus is extremely rare and seen in children with diaphragmatic hernia and intrathoracic herniation of the stomach. Three of our patients were found to have large paraesophageal hernias with herniation of the stomach and intrathoracic gastric volvulus. Gastric volvulus is also classified according to the etiology into idiopathic where no precipitating cause could be found and secondary to other anatomical defects such as diaphragmatic hernia, eventration of diaphragm, Morgagni's hernia, paraesophageal hernia and congenital asplenia [11].

The treatment of paraesophageal hernia is surgical repair. This is even for asymptomatic, incidentally discovered cases. This is to obviate the associated morbidity and mortality including the risk of gastric volvulus, strangulation and perforation in spite of its low frequency. The main operative procedure is a properly performed hiatoplasty which must be performed carefully and not too tight to avoid narrowing and obstruction of the esophagus. The addition of an antireflux procedure to the repair remains controversial. The rarity of paraesophageal hernia in the pediatric age group makes it difficult to evaluate the true necessity of adding an antireflux procedure for these patients. There are those who advocate adding an antireflux procedure while others do not. This is supported by the fact that 12 (60%) of the 20 patients in Karpelowsky et al. series who did not have fundoplication at the time of hernia repair developed recurrent reflux symptoms [5]. In our initial 5 cases we added a Nissen’s fundoplication as an antireflux procedure but subsequently we preferred gastropexy as an additional procedure. The stomach in these patients is mobile and although a Nissen’s fundoplication will limit this mobility we feel gastropexy is a more simple procedure and once hiatoplasty was done, there is no need to add an antireflux procedure. We perform gastropexy to the diaphragm and anterior abdominal wall. The recent advances in minimally invasive surgery have made it feasible and safe to repair paraesophageal hernias laparoscopically in children and adults. This is even in the presence of intrathoracic gastric volvulus [15,16,17].

In conclusion, congenital paraesophageal hernia is rare in the pediatric age group and sometimes the hernia defect is large with herniation of most of the stomach into the chest with the risk of intrathoracic gastric volvulus. This calls for early diagnosis and treatment. Congenital paraesophageal hernia should be treated surgically once the diagnosis is made. The addition of an antireflux procedure is still controversial. We feel adding a gastropexy to the repair is advantageous since the stomach is mobile in these patients and this should eliminate the possible risk of intra-abdominal gastric volvulus.

 

 

References:

1. Alrabeeah A, Giacomantonio M, Gillis DA: Paraesophageal hernia after Nissen fundoplication: a real complication in pediatric patients. J Pediatr Surg 23(8):766-8, 1988.

2. Al-Arfaj AL, Khwaja MS, Upadhyaya P. Massive hiatal hernia in children. Eur J Surg 1991; 157(8):465-8.

3. Salameh B, Pallati PK, Mittal SK. Incarcerated intrathoracic stomach with antral ischemia resulting in gastric outlet obstruction: a case report. Dis Esophagus 2008; 21(2):189-91.

4. Jawad AJ, al-Samarrai AI, al-Mofada S, al-Howasi M, Hawass NE, al-Beiruti Z. Congenital para-oesophageal hiatal hernia in infancy. Pediatr Surg Int 1998; 13(2-3):91-4.

5. Karpelowsky JS, Wieselthaler N, Rode H. Primary paraesophageal hernia in children. J Pediatr Surg 2006; 41(9):1588-93.

6. Baglaj SM, Noblett HR. Paraoesophageal hernia in children: familial occurrence and review of the literature. Pediatr Surg Int 1999; 15(2):85-7.

7. Petersons A, Liepina M, Spitz L. Neonatal intrathoracic stomach in Marfan's syndrome: report of two cases. J Pediatr Surg 2003; 38(11):1663-4.

8. Al-Assiri A, Wiseman N, Bunge M. Prenatal diagnosis of intrathoracic stomach (gastric herniation). J Pediatr Surg 2005; 40(2):E15-7.

9. Myles RB. Familial short oesophagus. Br J Radiol 1939; 12:645- 647.

10. Carré IJ, Johnston BT, Thomas PS, Morrison PJ. Familial hiatal hernia in a large five generation family confirming true autosomal dominant inheritance. Gut 1999; 45(5):649-52.

11. Al-Salem AH. Acute and chronic gastric volvolus in infants and children: who should be treated surgically? Pediatr Surg Int 2007; 23(11):1095-9.

12. Samuel M, Burge DM, Griffiths DM: Gastric volvolus and associated gastroesophageal reflux. Arch Dis Child 1995; 73:462-464.

13. McCallion WA, Potts SR, Wallace EC: Chronic organoaxial malrotation of the stomach in childhood. Eur J Pediatr Surg 1992; 2:365-367.

14. Del Rossi C, Cerasoli G, Tosi C, et al: Intrathoracic gastric volvolus in an infant. Pediatr Surg Int 1993; 8:146-148.

15. Iannelli A, Fabiani P, Karimdjee BS, Habre J, Lopez S, Gugenheim J. Laparoscopic repair of intrathoracic mesentericoaxial volvolus of the stomach in an adult: report of a case. Surg Today 2003; 33(10): 761-3.

16. Katkhouda N, Mavor E, Achanta K, Friedlander MH, Grant SW, Essani R, Mason RJ, Foster M, Mouiel J. Laparoscopic repair of chronic intrathoracic gastric volvolus. Surgery 2000; 128(5):784-90.

17. Pierre AF, Luketich JD, Fernando HC. Results of laparoscopic repair of giant paraesophageal hernia, 200 consecutive patients. Ann Thorac Surg 2002, 4 (6):1909-1915.