Indian Pediatr 2011;48: 649-650
Cogenital Intrapericardial Herniation of Liver
NB Mathur, Anuradha Bansal and *Satish Kumar Aggarwal
From Department of Pediatrics and *Pediatric Surgery,
Maulana Azad Medical College, New Delhi 110002, India.
Correspondence to: Dr NB Mathur, Director Professor of
Pediatrics, Maulana Azad Medical College,
New Delhi 110002, India.
Received: February 15, 2010;
Initial review: April 13, 2010;
Accepted : May 25, 2010.
Intrapericardial herniation of liver is a rare form of diaphragmatic
hernia. We report a 30-hour old baby with right congenital anterior
diaphragmatic hernia masquerading as congenital pneumonia with
cardiomegaly. It is prudent to consider congenial anterior diaphragmatic
hernia in any newborn with unexplained respiratory distress,
cardiomegaly and pericardial effusion.
Key words: Diaphragmatic hernia, Intrapericardial herniation,
Neonate, Respiratory distress.
herniation of liver is the rarest form of diaphragmatic hernia [1,2]
and only one such case has been reported from India . We report a
case of right congenital diaphragmatic hernia masquerading as
congenital pneumonia with cardiomegaly and discuss the relevant
A 30-hour old female born to 28 year old
primigravida mother at 34 weeks of gestation presented to us with
respiratory distress since half hour of life. The baby was delivered
through caesarean section for meconium stained amniotic fluid at a
private nursing home. Antenatal period was uneventful with normal
antenatal ultrasound. Birthweight was 1800 g and Apgar score was 7,
8, 9. In view of respiratory distress, the baby was managed as a
case of congenital pneumonia with oxygen, intravenous fluids and
antibiotics. The symptoms persisted beyond 24 hours of life and the
baby was referred to our hospital. At admission she had a
respiratory rate of 92 per minute with minimal intercostal and
sub-costal retractions. Heart rate was 140 per minute and capillary
refill time was normal. Oxygen saturation was 89% in room air and
there was no evidence of congestive cardiac failure. Arterial blood
gas revealed a pH of 7.42 with PaO2
of 61 mmHg, PaCO2 33 mm of Hg and bicarbonate 25 mEq/L.
On examination, the neonate had a cleft of soft
palate and low set ears. Systemic examination was not contributory.
She was active, alert and neonatal reflexes were present. She was
started on oxygen, intravenous fluids and antibiotics were added in
view of positive sepsis screen. Chest X-ray showed normal
lung fields with massive cardiomegaly. Ultrasound of the chest
revealed moderate pericardial effusion. A 2.4 × 1.8 cm fluid filled
area was seen in right lower hemithorax continuing with pericardial
cavity. There was a defect in the central tendon of diaphragm with
herniation of superior part of right lobe of liver in lower
hemithorax abutting the right atrium. Both the lungs were filled
with air and solid organs were normal in morphology. CT scan of the
chest confirmed these findings. Echocardiography also showed
protrusion of liver into thoracic cavity and compressing the right
atrium and right ventricle. There was a 3 mm atrial septal defect
and mild bilateral peripheral pulmonary artery stenosis. Chromosomal
analysis could not be done due to financial constraints.
The baby was operated on day 5 of life. A
laparoscopic repair was planned. A defect (3cm diameter) in the
central tendon of the diaphragm was visualized, through which a part
of the right lobe of liver was herniating into the mediastinum. Rest
of the viscera was normal. While dissecting the margins of the
defect laparoscopically there was some bleeding from the diaphragm.
For the sake of safety the procedure was converted into open
operation. The bleeding was from a diaphragmatic vessel and could be
controlled easily. The defect was defined and the liver delivered
into the abdomen. A gush of clear fluid came from the pericardium,
into which the liver was herniating. Pleura was not opened on either
side. The defect was repaired with non absorbable sutures. The
abdomen was closed.
The baby was shifted to NICU for elective
ventilation and supportive treatment. The pericardial fluid analysis
revealed 15 cells, all lymphocytes and 342 mg/dL protein suggestive
of transudate. The culture was sterile. The baby remained stable for
24 hours but further course could not be followed as she was taken
against medical advice due to some family reasons.
Anterior diaphragmatic hernia of Morgagni is an
uncommon entity believed to occur due to failure of development of
retrosternal portion of septum transversum during 8th week of
embryonic life . The central tendon of diaphragm is a thin but
strong aponeurosis situated near the centre of the vault formed by
the muscle, but somewhat closer to the front than to the back of the
thorax so that the posterior muscular fibres are longer. It is
situated immediately below the pericardium, with which it is
partially blended . Hence, herniation through a defect in the
central tendon of diaphragm results in intra pericardial herniation
of abdominal contents.
Till date, only 13 cases of congenital anterior
diaphragmatic hernia into the pericardium have been reported.
Pericardial effusion was not clearly described in three of these.
All of these cases showed pathological characteristics differing
from those of congenital diaphragmatic hernia detected after early
infancy. Congenital anterior diaphragmatic hernia is characterized
by: (i) development during fetal period (ii) frequent
complication by massive pericardial effusion and the absence of
cardiac tamponade; (iii) less frequent respiratory problems,
and (iv) hernial contents consisting of liver herniating into
the pericardium .
Herniation of liver into the pericardial cavity
usually leads to slowly developing pericardial effusion as described
above [1-3,5-6]. Some cases may even present beyond neonatal period
as persistent pneumonia [7,8]. Although the detailed pathophysiology
of pericardial effusion remains unclear, the following mechanisms
have been proposed; compression of the thoracic duct causing
accumulation of lymph; venous obstruction in the liver leading to
congestion and transudation, and, mechanical irritation . In our
case, the baby remained hemodynamically stable and did not require
any ventilatory support preoperatively. This might be because of the
absence of lung hypoplasia. Smaller hernial content as compared to
the posterior hernia, and slow formation of pericardial fluid with
compensatory progressive dilatation of fetal pericardium have been
proposed to be the reasons for the absence of cardiac tamponade .
To conclude, it is prudent to consider congenial
anterior diaphragmatic hernia as a possibility in any newborn with
unexplained respiratory distress, cardiomegaly and pericardial
effusion. Prompt diagnosis is crucial as this condition is amenable
to surgical treatment. Minimal invasive surgery is gaining
popularity for the treatment [9,10].
Contributors: NBM was involved in analysis of
findings, planning work up of the patient, finalization of draft and
will act as guarantor for the paper. AB was involved in drafting the
paper and primary care of the patient. SKA was involved in surgical
management of the patient and drafting the paper.
Competing interests: None stated.
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