Right sided congenital diaphragmatic defect with liver hernia: A case report

Right sided congenital diaphragmatic defect with liver hernia

International Archives of Integrated Medicine, Vol.

Copy right © 2014, IAIM, All Rights Reserved.

Case Report

Right sided congenital diaphragmatic defect

with liver hernia: A case report

Saket Jain1, Setu Satani

1Resident, Department of Radio diagnosis, MGM Hospital

2Resident, Department of Pediatrics, MGM Hospital

*Corresponding author email:

How to cite this article: Saket Jain, Setu Satani, Sanyukta Khandelwal, Sakshi Dandona, Akshay

Gursale. Right sided congenital diaphragmatic defect with liver hernia: A case report

1(4): 75-79.

Available online at

Received on: 01-12-2014

Abstract

Congenital diaphragmatic hernia is a rare entity occurring in 1 in 2000

for 8% of all major congenital anomalies. Congenital diaphragmatic hernia (CDH) is a major surgical

emergency in new-borns because the key to survival depends on the prompt diagnosis and

treatments. We reported here

hernia in a 7 month old baby, who came to seek medical care at a tertiary care centre in Navi

Mumbai for on and off cold and cough with breathlessness after feeds. The baby was tachypneic

examination. Immediately chest X

symptomatically and clinically suspected case of congenital diaphragmatic hernia. It was a case of

right sided congenital diaphragmatic hernia.

Key words

Congenital diaphragmatic hernia,

Introduction

Congenital diaphragmatic hernia is definitely a

matter of concern due to its high incidence of

morbidity and mortality. There

predominance with the ratio of 3:2

are three types and these are posterolateral

genital diaphragmatic defect with liver hernia

International Archives of Integrated Medicine, Vol. 1, Issue. 4, December, 2014.




, Setu Satani1*

, Sanyukta Khandelwal2, Sakshi Dandona

Akshay Gursale1

, Department of Radio diagnosis, MGM Hospital, Navi Mumbai

, Department of Pediatrics, MGM Hospital, Navi Mumbai

*Corresponding author email: [email protected]

Saket Jain, Setu Satani, Sanyukta Khandelwal, Sakshi Dandona, Akshay

congenital diaphragmatic defect with liver hernia: A case report

Available online at www.iaimjournal.com

2014 Accepted on:

Congenital diaphragmatic hernia is a rare entity occurring in 1 in 2000-4000 live


borns because the key to survival depends on the prompt diagnosis and

ed here a case of right sided congenital diaphragmatic

, who came to seek medical care at a tertiary care centre in Navi

off cold and cough with breathlessness after feeds. The baby was tachypneic

examination. Immediately chest X-ray and CT scan of thorax and abdomen was done to assess


right sided congenital diaphragmatic hernia.

Congenital diaphragmatic hernia, Right side, Rare, Liver herniation, 7 month female.

Congenital diaphragmatic hernia is definitely a

matter of concern due to its high incidence of

morbidity and mortality. There is a male

predominance with the ratio of 3:2 [1]. There

are three types and these are posterolateral

Bockdalek hernia (usually occurring at

approximately 6 weeks of gestation), the

anterior Morgagni hernia and the hiatal hernia.

Most common type of CDH is

Bockdalek hernia (85%) through posterolateral

defect in diaphragm (Foramen of Bockdalek). In

left sided hernia the large and the small bowel

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Page 75



, Sakshi Dandona1,

, Navi Mumbai, India

, Navi Mumbai, India

Saket Jain, Setu Satani, Sanyukta Khandelwal, Sakshi Dandona, Akshay

congenital diaphragmatic defect with liver hernia: A case report. IAIM, 2014;

Accepted on: 11-12-2014

4000 live births and accounts


borns because the key to survival depends on the prompt diagnosis and

iaphragmatic defect with liver

, who came to seek medical care at a tertiary care centre in Navi

off cold and cough with breathlessness after feeds. The baby was tachypneic on

ray and CT scan of thorax and abdomen was done to assess


iver herniation, 7 month female.

Bockdalek hernia (usually occurring at

approximately 6 weeks of gestation), the

anterior Morgagni hernia and the hiatal hernia.

Most common type of CDH is left-sided

Bockdalek hernia (85%) through posterolateral

defect in diaphragm (Foramen of Bockdalek). In

left sided hernia the large and the small bowel




with or without intra abdominal solid organ may

be herniated into the thorax. In right

hernia (incidence 13%) only the liver and portion

of small bowel tend to be herniated into the

thorax [2]. Chest x-ray and CT are considered as

best diagnostic methods [3].

Case report

A 7 month old female, full term, normal vaginal

delivery with no previous antenatal

ultrasonography done presented to MGM

Hospital, Kamothe, Navi Mumbai with chief

complaints of on and off episodes of cough and

cold, breathlessness especially after feeds s

two months. Usually child presents with

malnutrition after 1 month of birth. This is unlike

the classic presentation of CDH as this patient

was well developed and presented late i.e. 5

months of age. There were no gastrointestinal

symptoms in this case. On examination, the child

was found to be tachypneic with a respiratory

rate of 60 breaths per min with mild intercostal

retractions and decreased air entry on the right

side. The most severely affected events develop

respiratory distress at birth where

majority present respiratory symptoms within

24 hours. Of birth, only 2.6-10% of the cases

may present after this period. It has been seen

that the proportion of right sided CDH in late

presenting cases is higher. In retrospective view

of patients with right CDH, the mean age for

diagnosis to be six months. Usually right sided

CDH presents with gastrointestinal problems

and left sided CDH presents with respiratory

symptoms. Immediately chest x

ill defined non- homogenous soft tissue

occupying lower half of the right hemi

with shift of mediastinum and heart towards the

left side. The right hemi-diaphragm silhouette is

obscured with this opacity (Photo

the left hemi-diaphragm appears at its normal

level without any diaphragmatic defect.




abdominal solid organ may

be herniated into the thorax. In right-sided

cidence 13%) only the liver and portion

of small bowel tend to be herniated into the

ray and CT are considered as

A 7 month old female, full term, normal vaginal

delivery with no previous antenatal

ultrasonography done presented to MGM

Hospital, Kamothe, Navi Mumbai with chief

complaints of on and off episodes of cough and

cold, breathlessness especially after feeds since

two months. Usually child presents with

malnutrition after 1 month of birth. This is unlike

the classic presentation of CDH as this patient

was well developed and presented late i.e. 5

no gastrointestinal

e. On examination, the child

was found to be tachypneic with a respiratory

rate of 60 breaths per min with mild intercostal

retractions and decreased air entry on the right

side. The most severely affected events develop

respiratory distress at birth whereas large

majority present respiratory symptoms within

10% of the cases

may present after this period. It has been seen

the proportion of right sided CDH in late

presenting cases is higher. In retrospective view

with right CDH, the mean age for

diagnosis to be six months. Usually right sided

CDH presents with gastrointestinal problems

and left sided CDH presents with respiratory

Immediately chest x -ray showed an

homogenous soft tissue opacity

occupying lower half of the right hemi-thorax

with shift of mediastinum and heart towards the

diaphragm silhouette is

(Photo - 1) whereas

diaphragm appears at its normal

ut any diaphragmatic defect.

Photo - 1: Frontal chest radiograph: R

diaphragm is obscured with the well

opacity in right lower zone.

Tomogram showed soft tissue attenuation

occupying lower half of right hemi thorax with

non-visualization of right hemi diaphragm. There

is evidence of mediastinal and cardiac shif

towards the left side. (Photo

showed soft tissue attenuation oc

lower hemi thorax part of the right lobe of liver.

It showed at the level of origin of left lower lobe

bronchus the liver can be seen, herniation of

part of right lobe of liver in the right hemi

with non-visualization of right dome of

diaphragm throughout its entire extent.

evidence of shift of mediastinum and heart in

left hemi thorax. (Photo -

contrast enhanced CT was not done on the view

of increased radiation exposure to the child.

Right diaphragmatic crura

discontinuous on its progression.

Kidney was found in normal position whereas

liver was herniating into right hemi thorax.

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Frontal chest radiograph: Right hemi-

diaphragm is obscured with the well-defined

Tomogram showed soft tissue attenuation


ation of right hemi diaphragm. There

is evidence of mediastinal and cardiac shift

(Photo - 2) NECT of thorax

showed soft tissue attenuation occupying right

part of the right lobe of liver.

at the level of origin of left lower lobe

bronchus the liver can be seen, herniation of

part of right lobe of liver in the right hemi thorax

visualization of right dome of

diaphragm throughout its entire extent. There is

stinum and heart in

- 3, Photo - 4) The

contrast enhanced CT was not done on the view

of increased radiation exposure to the child.

Right diaphragmatic crura were found to be

uous on its progression. (Photo - 5)

found in normal position whereas

into right hemi thorax.




(Photo - 6) Post-operatively X-ray showed repair

of the hernia with absence of the opacity in the

right lower zone, the right lun

expanded. (Photo - 7)

Photo - 2: Tomogram: Soft tissue attenuation


non-visualization of right hemi diaphragm.

Photo - 3: NECT of thorax: S

attenuation occupying right lower hemi thorax





ray showed repair

of the hernia with absence of the opacity in the

right lower zone, the right lung appears well

oft tissue attenuation


visualization of right hemi diaphragm.

of thorax: Soft tissue

cupying right lower hemi thorax

Photo - 4: NECT of thorax

attenuation occupying right lower hemi thorax


Photo - 5: Arrow showing left crus of diaphragm

(normal) and right and arrowhead showing right

diaphragmatic crura which is discontinu

its progression.

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NECT of thorax: Soft tissue

upying right lower hemi thorax


Arrow showing left crus of diaphragm

(normal) and right and arrowhead showing right

diaphragmatic crura which is discontinuous on




Photo - 6: Coronal image: Kidney in normal

position and liver herniated into right hemi

thorax.

Photo - 7: Post-operative X-ray

hernia with absence of the opacity in the right

lower zone, the right lung

expanded.




idney in normal

position and liver herniated into right hemi

ray: Repair of the

hernia with absence of the opacity in the right

lower zone, the right lung appears well

Discussion

The diaphragm is the mesodermal partition in

between thorax and abdomen develops at 3 to 8

week of intrauterine life from the following

sources:

1) Septum transversum forms the central

tendon of diaphragm.

mesoderm lying caudal to the

pericardial sac and

ventral body wall to the oesophageal

segment of the foregut.

2) Dorsal mesentery of the oesophagus

forms the crura.

3) Peripheral part is developed from the

shelf-like projection of the body

4) Pleuroperitoneal membranes: The

openings are situated dorsal to the

septum transversum and on each side of

the dorsal mesentery of the

oesophagus. Each opening is closed

pleuroperitoneal membrane which is

dissected off from the body wall by the

caudal growth of the lung

membrane fuses with septum

transversum and with the dorsal

mesentery of the oesophagus

Here the defect is in the failure of the right sided

pleuroperitoneal membrane to close the same

sided pleuroperitoneal canal

Pleuroperitoneal membranes are located

dorsolateral to the pleuroperitoneal canals. The

pleuroperitoneal canal is closed by the fusion of

its edges.

The hernia most commonly occurs in the left

side as it closes later but here in this reported

case the hernia is in the right side which is very

uncommon. The diaphragmatic defect allows

the abdominal viscera to enter the thoracic

cavity. The herniated mass prevents the growth

of the right lung causing pulmonary hypoplasia

[7].

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Page 78

The diaphragm is the mesodermal partition in

between thorax and abdomen develops at 3 to 8

week of intrauterine life from the following

Septum transversum forms the central

tendon of diaphragm. It is a sheet of

mesoderm lying caudal to the

pericardial sac and extends from the

ventral body wall to the oesophageal

segment of the foregut.

sal mesentery of the oesophagus

Peripheral part is developed from the

like projection of the body wall.

peritoneal membranes: The

openings are situated dorsal to the

septum transversum and on each side of

dorsal mesentery of the

oesophagus. Each opening is closed by a

peritoneal membrane which is

dissected off from the body wall by the

of the lung-bud. The

membrane fuses with septum

transversum and with the dorsal

oesophagus [4, 5].

ilure of the right sided

peritoneal membrane to close the same

sided pleuroperitoneal canal [6].

Pleuroperitoneal membranes are located

dorsolateral to the pleuroperitoneal canals. The

pleuroperitoneal canal is closed by the fusion of

The hernia most commonly occurs in the left

side as it closes later but here in this reported

he hernia is in the right side which is very

uncommon. The diaphragmatic defect allows

the abdominal viscera to enter the thoracic

cavity. The herniated mass prevents the growth

of the right lung causing pulmonary hypoplasia




Even though majority of such cases occur as

isolated entity, it is important to look for other

congenital malformations like cardiac anomalies,

pulmonary agenesis or hypoplasia, renal

agenesis, hydronephrosis, spina bifida and

chromosomal anomalies such as Trisomy 21, 18

or 13 which can coexist along with CDH. Familial

inheritance is reported to occur in 2% of the

cases. The key to survival of these patients

depends on the prompt diagnosis, prenatal

management, delivery, stabilization and

treatment. The site, size, contents of the

and associated anomalies also greatly influence

the outcome of the disease.

CDH is also reported to occur in babies of the

mothers who are exposed to quinine,

thalidomide and antiepileptic drugs during

pregnancy [8]. Liver herniation might be

associated with poorer prognosis detected by

radiological investigations in

diaphragmatic hernia. Plain radiography

sometimes may be helpful for presumptive

diagnosis and contrast CT may help to arrive at a

precise diagnosis.

References

1. Torfs CP, Curry CJR, Bateson TF, et al. A

population based study of congenital

diaphragmatic hernia. Teratology

46: 555-65.

Source of support: Nil




ch cases occur as

isolated entity, it is important to look for other

congenital malformations like cardiac anomalies,

pulmonary agenesis or hypoplasia, renal

agenesis, hydronephrosis, spina bifida and

chromosomal anomalies such as Trisomy 21, 18

h can coexist along with CDH. Familial

inheritance is reported to occur in 2% of the

cases. The key to survival of these patients

depends on the prompt diagnosis, prenatal

management, delivery, stabilization and

The site, size, contents of the hernia

and associated anomalies also greatly influence

CDH is also reported to occur in babies of the

mothers who are exposed to quinine,

thalidomide and antiepileptic drugs during

. Liver herniation might be

iated with poorer prognosis detected by

radiological investigations in fetal congenital

diaphragmatic hernia. Plain radiography

sometimes may be helpful for presumptive

help to arrive at a

CP, Curry CJR, Bateson TF, et al. A

population based study of congenital

diaphragmatic hernia. Teratology, 1992;

2. Snow RW, Armstrong JRM, Forster D,

Winstanley MT, Marsh VM, Newton CR

et al. Childhood deaths in Africa:

and limitations of

Lancet, 1992; 340: 351

3. Khawahur H, Kattan A, Al

Saidy K. Congenital diaphragmatic

hernia: A local experience. Annal Saudi

Med, 1999; 19(6): 501

4. Dutta A.K. Essential Human Embryology.

3rd

edition. Kolkata: Current Books

International; 1995, p

5. Saddler TW. Langman's Medical

Embryology in Body Cavities. 11

edition. New Delhi: Lippincott Williams

and Wilkins, Wolters Kluwer Health

India Pvt Ltd; 2006, p

6. Moore KL, Persaud TVN.

mesenteries and diaphragm, 7

USA: Elsevier Publishers; 2003, p

97.

7. Standring S, Collins P, Heely LC, Johnson

D, Shah P, Wigley C. Gray's Anatomy in

Development of trachea, lungs and

diaphragm. 39th

London, New York, Oxford, Philadelphia,

St. Louis, Sydney, Toronto: Elsevier

Churchill Livingstones Publication; 2005,

p. 1092-93.

8. Hoboth N. Drugs and congenital

anomalies. Lancet, 1962; 2: 1444.

Nil Conflict of interest:

ISSN: 2394-0026 (P)

ISSN: 2394-0034 (O)

Page 79

Snow RW, Armstrong JRM, Forster D,

Winstanley MT, Marsh VM, Newton CR,

et al. Childhood deaths in Africa: Uses

and limitations of verbal autopsies.

351–5.

Khawahur H, Kattan A, Al-Alaiyan S,

Saidy K. Congenital diaphragmatic

hernia: A local experience. Annal Saudi

501-4.

Essential Human Embryology.

. Kolkata: Current Books

International; 1995, p. 157-59.

Saddler TW. Langman's Medical

Embryology in Body Cavities. 11th

. New Delhi: Lippincott Williams

and Wilkins, Wolters Kluwer Health

India Pvt Ltd; 2006, p. 159-162.

Persaud TVN. Body cavities,

nd diaphragm, 7th

edition.

USA: Elsevier Publishers; 2003, p. 192-

Standring S, Collins P, Heely LC, Johnson

D, Shah P, Wigley C. Gray's Anatomy in

Development of trachea, lungs and

edition. Edinburg,

London, New York, Oxford, Philadelphia,

St. Louis, Sydney, Toronto: Elsevier-

Churchill Livingstones Publication; 2005,

Hoboth N. Drugs and congenital

1962; 2: 1444.

Conflict of interest: None declared.

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Right sided congenital diaphragmatic defect with liver hernia: A case report