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ORIGINAL ARTICLE
Year : 2019  |  Volume : 7  |  Issue : 4  |  Page : 154-157

Right congenital diaphragmatic hernia: Four cases and literature review


Department of Paediatric Surgery, TNMC and BYL Nair Hospital, Mumbai, Maharashtra, India

Date of Submission14-May-2017
Date of Acceptance29-Aug-2019
Date of Web Publication12-Dec-2019

Correspondence Address:
Hemanshi Shah
Department of Paediatric Surgery, TNMC and BYL Nair Hospital, Mumbai - 400 008, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ssj.ssj_42_17

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  Abstract 

Background: Left-sided Bochdalek congenital diaphragmatic hernia (CDH) is the most common type of CDH. The right-sided Bochdalek CDH is rare and usually contains only the liver as its content. We describe four children with right CDH managed at our institution over 3 years.
Materials and Methods: We present a retrospective analysis of four patients with right CDH managed at our institution from 2012 to 2015 with respect to age at presentation, sex, presenting symptoms, investigations, associated anomalies, management, and outcome.
Results: The average age at presentation was 1.5 years, with range being 5 days to 4 years. The three older children presented with a history of frequent respiratory tract infections. The neonate was a preterm low-birth weight baby and had respiratory distress since birth. A chest radiograph suggested right pneumothorax for which intercostal chest drain insertion was done. A repeat chest X-ray showed suspicion of right-sided CDH which was confirmed on computerized tomography. All four patients underwent right thoracotomy with repair of the CDH. The neonate expired on the 4th postoperative day. The other three children had an uneventful postoperative recovery.
Conclusion: Right-sided CDH is rare and has varied presentations and poorer outcome.

Keywords: Bochdalek, respiratory tract infection, right congenital diaphragmatic hernia


How to cite this article:
Tiwari C, Shah H, Kumbhar V, Makhija D, Bothra J, Sandlas G. Right congenital diaphragmatic hernia: Four cases and literature review. Saudi Surg J 2019;7:154-7

How to cite this URL:
Tiwari C, Shah H, Kumbhar V, Makhija D, Bothra J, Sandlas G. Right congenital diaphragmatic hernia: Four cases and literature review. Saudi Surg J [serial online] 2019 [cited 2020 Feb 26];7:154-7. Available from: http://www.saudisurgj.org/text.asp?2019/7/4/154/272849


  Introduction Top


The history of congenital diaphragmatic hernia (CDH) in medical literature dates back to 1679 when CDH was first incidentally noted by Lazarus Riverius during an autopsy of a 24-year-old person.[1] In 1761, Giovanni Battista Morgagni described the classic anterior diaphragmatic hernia which has been named after him.[1] In 1848, Victor Alexander Bochdalek described both left and right posterolateral CDH, which today is referred as Bochdalek hernia.[1],[2]

Thus, there are three types of CDH – posterolateral Bochdalek hernia, anterior Morgagni hernia, and hiatal hernia.[1] The left-sided Bochdalek hernia is the most common type occurring in approximately 85% of cases.[1] In this type of CDH, the large and the small bowel with or without intra-abdominal solid organ may be herniated into the thorax. The incidence of right-sided Bochdalek CDH is rare and occurs in approximately 13%–20%;[1],[3] it contains only the liver along with some portion of the small bowel.[1],[4] CDH can be isolated (nonsyndromic) or familial (in about 2% of cases) – which can be autosomal recessive, or X-linked.[1] The various syndromes which have CDH as a component are Fryns, Donnai–Barrow, Beckwith–Wiedemann, Simpson–Golabi–Behmel, Coffin–Siris, and Denys–Drash syndromes. Karyotype anomalies are seen in approximately 4% of cases – trisomy 13 and 18, tetrasomy 12p mosaicism, and deletions of 1q, 8p, and 15q.[1],[2] CDH may be complicated and associated with other anomalies such as gastric volvulus, rotational abnormalities and midgut volvulus (incidence rate 30%–62%), gastric or intestinal perforations, and left ventricular hypoplasia of heart.[1],[5]

Right CDH has a varied presentation and poorer prognosis. We present four children with right CDH managed at our institution over 3 years.


  Materials and Methods Top


Case records of four patients with right CDH managed at our institution from 2012 to 2015 were retrospectively analyzed with respect to age at presentation, sex, presenting symptoms, investigations, associated anomalies, management, and outcome.

All patients were admitted. Routine blood investigations were sent for all the patients. X-ray of the chest and abdomen; Ultrasonography (USG); two-dimensional (2D) echo; and computed tomography of the chest were done. After stabilization, the patients were taken for surgery.


  Results Top


Demographic profile

A total of four children with right CDH were treated over a 4-year period. The average age at presentation was 1.5 years, with the range being 5 days to 4 years. There were three males and one female.

Clinical presentation

The three older children presented with a history of frequent respiratory tract infections. They were investigated by the pediatricians and were diagnosed to have right CDH on X-rays and computed tomography of the thorax [Figure 1]. A 2D echo and abdominal ultrasound ruled out other associated anomalies in these patients. The neonate was a preterm low-birth weight baby delivered by the cesarean section and had respiratory distress since birth. He was given bag and mask ventilation after birth. A chest radiograph suggested right pneumothorax, for which intercostal chest drain insertion was done. However, there was no improvement in respiratory distress. A repeat chest X-ray showed suspicion of right-sided CDH which was confirmed on computerized tomography.
Figure 1: Coronal section of computed tomography of the thorax showing right congenital diaphragmatic hernia

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Management and outcome

All four patients underwent right thoracotomy with repair of the CDH [Figure 2] and [Figure 3]. All four patients had the right lobe of the liver as the content. The neonate required postoperative ventilator support but succumbed on the 4th postoperative day. The other three children had an uneventful postoperative recovery. They are asymptomatic on follow-up.
Figure 2: Intraoperative image showing right congenital diaphragmatic hernia with the liver (arrow) herniating into the right hemithorax

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Figure 3: Intraoperative image showing the right-sided diaphragmatic defect with edges of the defined

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  Discussion Top


Right CDH is rare and accounts for about 13%–20% of all CDHs.[3] The embryogenesis of CDH has been postulated to be the failure in the closure of pericardioperitoneal canal by pleuroperitoneal membranes, which occurs during the 8th gestational week.[6] It is hypothesized that an early return of the foregut into the coelomic cavity results in wide posterolateral spaces and dysfunctional pleuroperitoneal membranes.[6] The abdominal viscera consequently herniate into the thorax. As a result, this causes pulmonary hypoplasia by compression of the growing lung.[6] The left CDH is more common than right CDH because of the early closure of right pleuroperitoneal opening; however, there are no postulated theories to explain this.[6]

Most of the patients present in neonatal age group with respiratory distress.[7] However, a delayed clinical presentation has been reported in approximately 5%–30% of the right CDH patients.[3] Late-onset right CDH can present itself in various ways in the form of respiratory distress, intestinal obstruction, jaundice, or failure to thrive.[3] It has been postulated that partial liver displacement, which occurs in most right-sided CDH patients, may block the further herniation of hollow viscera.[7] That is the reason why in the right CDH group most children have respiratory symptoms only.[7]

When the respiratory distress occurs in a baby with right CDH and group B streptococcal (GBS) sepsis, the radiological features represent GBS pulmonary inflammatory conditions and may be incorrectly interpreted as right-sided pneumonia, pleural effusion, or even pneumothorax.[3]

More than half of patients present with right-sided pleural effusion. Its cause has been speculated to be due to hepatic venous outflow obstruction which results in vascular congestion and a transudate exiting through the liver surface.[3] When liver is the only herniated organ, there would be no intestinal gas shadow in the right chest to give a clue to the underlying pathology. Erroneous chest tube drainage may be done for an apparent pleural effusion which may actually be a right CDH, with the liver being the reason for the clinical percussion dullness or radiographic opacification.[3] Deviation of the esophageal portion of the nasogastric tube to the left side along with the vertical orientation of the intra-abdominal portion of the nasogastric tube may provide the only clue to the diagnosis of right CDH in such cases.[3]

The most common radiological finding of right CDH is the opaqueness of the right hemithorax usually associated with mediastinal shift to the contralateral side on plain chest radiograph.[7] Computed tomography has been considered the ideal noninvasive technique for diagnosis – the presence of diaphragmatic defect, size, exact location, and contents of the various types of diaphragmatic hernia can be very well evaluated.[7]

Antenatal diagnosis of right CDH is difficult. The liver is the most common herniated into the thoracic cavity, and by analogy in the ultrasonographic echogenicity between the liver and lung, it becomes difficult to diagnose the right CDH.[8] Portal blood flow, ascites, or positioning of the gallbladder in the thoracic cavity may provide important diagnostic clues to the antenatal diagnosis of right CDH.[8] An intrathoracic kidney could also be a diagnostic clue for the diagnosis of right CDH.[8]

Right CDH, being rare, there are difficulties in evaluating its prognosis.[8] The current literature comparing the outcome of right CDH when compared to left CDH is inconsistent.[9] There are several studies in the literature which have described poorer postnatal outcomes in right CDH when compared with left CDH;[8],[10],[11],[12] however, others have found no difference between the two types of CDH.[8],[13],[14] However, right CDH has been stated in the literature to carry disproportionately high mortality and morbidity.[15]

Herniation of the liver which is almost always present in right CDH has been perceived as an indicator of poor prognosis; however, this significance has only been shown for left CDH.[8] Liver herniation causes caval compression, leading to reduced preload and impaired cardiac output.[10] Moreover, the right lung normally accounts for 57% of the total functional lung volume; hence, any developmental abnormality of the right lung may have a bigger clinical impact.[8] Third, the size of defect necessary to permit herniation of the liver on the right side is likely to be substantially larger than that of a left-sided CDH. This explains the higher rates of patch repair required to manage right CDH.[8]

The significance of liver herniation and lung-to-head ratio have been documented as prognostic factors only for left CDH.[8] As most fetuses with right CDH have a liver herniation, this finding cannot be a significant antenatal prognostic factor in right CDH.[8] Similarly, there is controversy regarding the utility of lung-to-head ratio measurements for predicting survival in the right CDH.[8]


  Conclusion Top


Right CDH is rare and carries high rates of morbidity and requirement of extracorporeal membrane oxygenation and patch repair. The outcomes reported in the literature are mixed. The clinical presentation can be delayed and confused with pleural effusion and pneumonia. The outcome for patients with delayed presentation has been reported to be good.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Sarkar A, Hajra DS. Right sided congenital diaphragmatic hernia – A case report with a brief review. J Anat Soc India 2012;61:53-6.  Back to cited text no. 1
    
2.
Hekmantia A, McHugh K. Congenital diaphragmatic hernia: eMedicine radiology. Card Dis Crit Care 2008;3:5-6.  Back to cited text no. 2
    
3.
Parida L. Delayed onset of right congenital diaphragmatic hernia associated with group B streptococcal sepsis in a neonate. J Indian Assoc Pediatr Surg 2016;21:87-9.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Jani JC, Nicolaides KH, Gratacós E, Vandecruys H, Deprest JA; FETO Task Group. et al. Fetal lung-to-head ratio in the prediction of survival in severe left-sided diaphragmatic hernia treated by fetal endoscopic tracheal occlusion (FETO). Am J Obstet Gynecol 2006;195:1646-50.  Back to cited text no. 4
    
5.
Steinhorn RH. Congenital diaphragmatic hernia. Cardiac disease and 5 critical care medicine. Neonatology 2009;3:1-5.  Back to cited text no. 5
    
6.
Romeo C, Turiaco N, Gitto E, Borruto FA, Santoro G. Right Bochdalek hernia associated with Kartagener syndrome: Developmental and clinical observations. European J Pediatr Surg Rep 2013;1:15-7.  Back to cited text no. 6
    
7.
De Marco EA, Merli L, Taddei A, Pulitanò SM, Manzoni C, Nanni L. Late-presenting right congenital diaphragmatic hernia with severe hypotrophy of the right lobe of the liver. Int J Surg Case Rep 2015;17:28-30.  Back to cited text no. 7
    
8.
Jeong BD, Ahn SH, Song JW, Shim JY, Lee MY, Won HS, et al. Impaction of an intrathoracic kidney acted as a shield against herniation of the abdominal viscera in a case of right congenital diaphragmatic hernia. Obstet Gynecol Sci 2016;59:58-61.  Back to cited text no. 8
    
9.
Duess JW, Zani-Ruttenstock EM, Garriboli M, Puri P, Pierro A, Hoellwarth ME, et al. Outcome of right-sided diaphragmatic hernia repair: A multicentre study. Pediatr Surg Int 2015;31:465-71.  Back to cited text no. 9
    
10.
DeKoninck P, Gomez O, Sandaite I, Richter J, Nawapun K, Eerdekens A, et al. Right-sided congenital diaphragmatic hernia in a decade of fetal surgery. BJOG 2015;122:940-6.  Back to cited text no. 10
    
11.
Fisher JC, Jefferson RA, Arkovitz MS, Stolar CJ. Redefining outcomes in right congenital diaphragmatic hernia. J Pediatr Surg 2008;43:373-9.  Back to cited text no. 11
    
12.
Skari H, Bjornland K, Haugen G, Egeland T, Emblem R. Congenital diaphragmatic hernia: A meta-analysis of mortality factors. J Pediatr Surg 2000;35:1187-97.  Back to cited text no. 12
    
13.
Wilson JM, Lund DP, Lillehei CW, Vacanti JP. Congenital diaphragmatic hernia – A tale of two cities: The Boston experience. J Pediatr Surg 1997;32:401-5.  Back to cited text no. 13
    
14.
Harrison MR, Adzick NS, Estes JM, Howell LJ. A prospective study of the outcome for fetuses with diaphragmatic hernia. JAMA 1994;271:382-4.  Back to cited text no. 14
    
15.
Parate LH, Geetha CR, Vig S. Right sided congenital diaphragmatic hernia: A rare neonatal emergency. Saudi J Anaesth 2015;9:227-9.  Back to cited text no. 15
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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