Case Report

Congenital Eventration of Diaphragm Presenting As Diaphragmatic

Hernia: A Case Report
*Das JC1, Hasan SH2, Bhuiyan AH3, Paul N4, Faisal MA5, Roy DK6, Chowdhury N7, Ghosh
N8, Rashid MH9
Eventration of diaphragm is an abnormal elevation of diaphragmatic musculature while retaining
normal attachments to the sternum, ribs and dorsolumber spine. It is a rare anomaly where the
continuity of diaphragm remains intact. Pathological process can affect either all or only a portion of
hemidiaphragm. Symptoms vary according to size of the defect. Large defect may mimic
diaphragmatic hernia. The present case represents a full term female newborn that developed
respiratory distress, cyanosis and feeding difficulties since 1 st day of life. Clinical features and chest
imaging of this case was assumed to be left sided diaphragmatic hernia which was found wrong in the
operation theater. She was found to have left sided eventration of diaphragm to create symptom.
Successful plication of ipsilateral diaphragm was done on 13 th day of life. Supervised post operative
ventilatory support along with other postoperative care improved the condition of this neonate. A large
defect with eventration of diaphragm may be life threatening but a timely good management can save
neonate from such condition.
[Mymensingh Med J 2019 Oct; 28 (4): ]
Key words: Congenital, Eventration, Diaphragm, Hernia, Neonate

E
Introduction
ventration of the diaphragm is an abnormal
elevation; consisting of thin diaphragmatic
muscle resulting from failure of muscular
development of part or whole of the
hemidiaphragm1. The normal attachments to
sternum, ribs, and dorsolumbar spines are
maintained2. The unbroken continuity of
diaphragm differentiates it from diaphragmatic
hernia. It is a rare anomaly, occurring in about 1 in
10,000 live births with a male sex and left
hemidiaphragmatic preponderance3. Abnormal
development of phrenic nerve and phrenic nerve
injury during breech delivery may also cause
eventration1,4. Eventration can be associated with
other congenital anomalies like congenital heart
disease, tracheomalacia, cerebral agenesis, renal
ectopia, malrotation, Meckel’s diverticulum, and
Werdnig Hoffmann disease5. It is frequently not
possible to tell whether the defect is due to failure
of fusion (hernia) or due to focal muscular
deficiency (eventration)6. The important aspect of
the present case was that the neonate was clinically
diagnosed as diaphragmatic hernia but eventually
was confirmed as eventration of diaphragm during
operative procedure. The case is presented here
with a view to orient health personals particularly
pediatricians, neonatologists and pediatric surgeons
regarding diagnostic dilemma and management of
such life threatening condition to save life of
affected newborn infant.
1. *Professor Jagadish C Das, Professor, Department
of Neonatology, Chattogram Medical College,
Chattogram, Bangladesh; E-mail:jagadishcdas@
yahoo.com
2. Dr Syeda Humaida Hasan, Consultant, Department
of Neonatology, Chattogram Medical College,
Chattogram, Bangladesh
3. Professor Md Akbar Husain Bhuiyan, Professor
(Rtd), Department of Pediatric Surgery, Chattogram
Medical College, Chattogram, Bangladesh
4. Dr Nibedita Paul, Associate Professor, Department
of Obstetrics & Gynecology, BGC Trust Medical
College, Chattogram, Bangladesh
5. Dr Mohammad Abu Faisal, Lecturer, Department
of Forensic Medicine, Cox’s Bazar Medical College,
Coxe’s Bazar, Bangladesh
6. Dr Debashish Kumar Roy, Assistant Registrar,
Department of Neonatology, Chattogram Medical
College, Chattogram, Bangladesh
7. Dr Nita Chowdhury, Assistant Registrar,
Department of Neonatology, Chattogram Medical
College, Chattogram, Bangladesh
8. Dr Newton Ghosh, Assistant Registrar, Department
of Neonatology, Chattogram Medical College,
Chattogram, Bangladesh
9. Dr AKM Harunur Rashid, Assistant Professor,
Department of Pediatrics, Comilla Medical College,
Comilla, Bangladesh
*for correspondence

Mymensingh Med J 2019 Oct; 28 (4)

 Case Report
Case Report
A 7 days old full term female baby weighing
3000gm was referred to a tertiary care hospital with
the complaints of respiratory distress, cyanosis and
feeding difficulties since her 1st day of age. She was
delivered per vaginally with cephalic presentation
and had no history of perinatal asphyxia or risk
factors for developing sepsis. She was the third issue
of her poor parents. Mother’s antenatal period was
uneventful except polyhydramnios. On examination,
she was dyspneic, cyanosed with poor tone and
activities. Recorded oxygen saturation (SpO2) was
78%. Her chest was bulged on left side and
abdomen was scaphoid. Trachea and apex beat was
shifted to right side and assumed to be dull on
percussion with decreased air entry on the left side.
The 1st and 2nd heart sound was audible and there
was no murmur. There was neither organomegaly Figure 1: Patient is on artificial ventilation
nor other congenital anomaly. Chest and abdominal
radiograph showed patchy inhomogenous opacities
with tubular lucencies in the left lung field that
continued with abdominal bowel lucencies. The left
hemidiaphragm was not defined (Figure 4a). The
neonate was initially diagnosed as left sided
diaphragmatic hernia. Management was started with
temperature control, nasogastric suction, intravenous
fluid administration, high flow oxygen
supplementation and prophylactic antibiotics
administration. She underwent operative procedure
on her thirteenth postnatal day. Diaphragm was
explored by left subcostal incision. Thin, intact left
hemidiaphragm was found at the level of 2 nd
intercostal space. Diaphragmatic hernia was not
found. Stomach, spleen, intestinal coils, part of liver Figure 2: After weaning from ventilator and CPAP
was herniated in the left thoracic cavity with
compressed left lung. The abdominal viscera were
placed back in peritoneal cavity and plication of the
diaphragm was done with 1/0 prolene. Immediately
after operation, the patient was shifted to neonatal
intensive care unit for subsequent management. She
was on intermittent positive pressure ventilation
(IPPV) (Figure 1) and continuous positive airway
pressure (CPAP) for 7 days. Gradually feeding was
started through nasogastric tube on 7th post operative
day (POD). Breast feeding was allowed on 16 th POD
when her general condition was stable and
respiratory distresses abate. The newborn infant had
anemia and thrombocytopenia. Serum electrolyte,
liver and renal functions were within normal limit.

Mymensingh Med J 2019 Oct; 28 (4)

 Case Report
Figure 3: Healing scar at incision site
Follow up x-rays showed high up diaphragm and
gradual expansion of left lung field (Figure 4b &
4c).
Preoperative and postoperative echocardiography
did not reveal any abnormality. Blood culture
revealed Staphylococcus aureas which was
sensitive to ciprofloxacin, levofloxacin and
nitrofurantoin but resistant to meropenum and
gentamycin. She was treated with sensitive
Figure 4: Serial roentgenograms from left to right: a) Preoperative Chest and abdominal x-ray resembling
hernia; b) In immediate postoperative period with high up diaphragm and c) At the age of 45 days (at 32th
POD) with expansion of left lung
Discussion
Diaphragm develops from four components-
septum transversum, pleuroperitoneal membranes,
dorsal mesentery of the esophagus and muscular
components of the body wall7. Two types of
embryologic failure may be responsible for
eventration, failure of myoblasts to migrate
between the two leaves of the pleuroperitoneal
membrane on the affected side and non innervation
of myoblasts4,8. Pathological process can affect
either all or only a portion of the hemidiaphragm 2.
The degree of symptoms varies and correlates with
the size of the defect. Patients are asymptomatic
with small localized defects, whereas the
symptoms of significant eventration include:
recurrent chest infections, severe respiratory
distress with cyanosis, intestinal obstruction
Mymensingh Med J 2019 Oct; 28 (4)
antibiotic for appropriate duration. Blood and
blood products were given for several times.
Patient was on oxygen support till 1 month of her
age with gradual weaning. Her postoperative
course was uneventful and there was no wound
infection (Figure 2 & 3). On 42th postnatal day
when her condition was reasonably well without
supplemental oxygen, the newborn infant was
discharged from hospital.
following gastric volvulus, palpitations due to
mediastinal shift4,9. This patient presented with
dyspnea in neonatal period which is the principal
symptom of both diaphragmatic hernia and
eventration. Less effective caudal movement of the
diaphragm than normal individual, alveolar
hypoxia induced impaired perfusion to the basal
portion of ipsilateral lung, compression from
abdominal organs on the lung are the major
contributing factors though the paradoxical
respiratory movement is limited in eventration 2,9,11.
The condition, if severe, can simulate congenital
diaphragmatic hernia in a newborn and may be
complicated with pulmonary hypoplasia and/or
pulmonary hypertension3. Other symptoms we
found in this neonate are cyanosis and feeding
difficulties. Total eventration occurs almost
 Case Report
exclusively on the left side as seen in this case
though sex predilection does not match with
previous data3,4,8. Imaging of the diaphragm can be
used to confirm the diagnosis. Several radiologic
modalities, such as chest radiography, barium
studies of upper gastrointestinal tract, ultrasound,
CT scan, or magnetic resonance imaging (MRI)
may be used to evaluate the anatomy of the
diaphragm. The function of the diaphragm can be
evaluated with the use of fluoroscopy, ultrasound
or MR fluoroscopy9. Prenatal diagnosis of the
condition is also possible with ultrasound scan at
18-20 weeks gestation3 where there is elevation of
the affected dome of diaphragm on chest
imaging4,10. But in this case, chest X-ray showed
loss of demarcation of left hemidiaphragm and
bowel loops in the left thoracic cavity which
mimicked hernia. Mediastinum was markedly
displaced to right which was in favour of
eventration than diaphragmatic paralysis5.
Ultrasound of chest and barium studies could not
performed due to grave condition of the neonate
and furthermore, there was no provision of
fluoroscopy in our center. Final diagnosis was
done during operative procedure. Literature review
showed that even with the aid of MRI, sometimes
it is difficult to distinguish hernia from
eventration9. The management of eventration
depends upon the extent of respiratory distress. If
respiratory distress is mild, the management is
supportive. Support may include supplemental
oxygen and/ or pressure support 9. Surgical
intervention was required in this newborn as she
had severe respiratory distress with central
cyanosis demanding mechanical ventilation. The
established surgical treatment for eventration is
diaphragmatic plication which was first described
in 1923. It can be achieved by various techniques
and through various approaches: open transthoracic
approaches, thoracoscopic approaches, minimally
invasive approaches and open trans abdominal or
laparoscopic approaches2. Whatever the approach
and technique is used, the basic principle is to
make the diaphragm flat and taut in position of full
inspiration avoiding abdominal visceral injury 13.
Open transabdominal plication through left
subcostal incision was done in this patient which
revealed unilateral (left sided) complete
eventration. There are following advantages of an
Mymensingh Med J 2019 Oct; 28 (4)
open transabdominal approach: i) easily performed
through an upper abdominal incision; ii) less
morbid than a thoracotomy; iii) single-lung
ventilation is not necessary; and iv) it allows access
to both sides of the diaphragm with one
incision12,14. After operation, she was on
ventilatory support for about 5 days then gradual
weaning was done. Post operative chest imaging
showed gradual expansion of left lung. At her 45 th
day of life, the left dome of diaphragm was at the
level of 6th rib posteriorly with compensatory
hyperplasia of ipsilateral lung. The reduced
numbers of alveoli compensated by this
overdistension15. Reported complications of
plication such as pneumonia, pleural effusions,
abdominal compartment syndrome, abdominal
viscus injury, deep venous thrombosis, stroke,
upper gastrointestinal hemorrhage, pulmonary
emboli, arrhythmia and acute myocardial infarction
were not present in this patient 2. Incision site was
healthy but she had suffered from sepsis with
Staphylococcus aureas which was treated
accordingly. Her long term outcome will depend
on further pulmonary development. Some patients
of eventration and hernia may develop lower lobe
emphysema in the long run15.
Conclusion
Eventration of diaphragm is a rare but may be life
threatening condition in newborn infant. Clinically,
this condition present either in neonatal period or
beyond this period. During neonatal period, it may
mimic with congenital diaphragmatic hernia.
Overall mortality of eventration of diaphragm is
high even after plication. Though there is no
individual data in this center, only a few patients of
congenital eventration and hernia of diaphragm
survived here. In present case, the newborn infant
is presented in first day of life with respiratory
distress and cyanosis. Here, the neonate is saved
probably due to absence of associated congenital
anomaly, timely referral, prompt operative decision
and good postoperative supportive care.
Eventration of diaphragm should be kept in mind
as a differential diagnosis of respiratory distress
and cyanosis in a newborn infant beside
diaphragmatic hernia.
Reference
 Case Report
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