Journal of Pediatric Surgery 50 (2015) 1088–1092

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Journal of Pediatric Surgery

journal homepage: www.elsevier.com/locate/jpedsurg

Congenital diaphragmatic eventration in children: 12 years' experience

with 177 cases in a single institution☆
Shengde Wu a,b,c,d, Na Zang a,b,c,e, Jin Zhu a,b,c,f, Zhengxia Pan a,b,c,d, Chun Wu a,b,c,d,⁎
a
Ministry of Education Key Laboratory of Child Development and Disorders
b
Key Laboratory of Pediatrics in Chongqing, CSTC2009CA5002
c
Chongqing International Science and Technology Cooperation Center for Child Development and Disorders
d
Department of Pediatric Surgery, Children's Hospital of Chongqing Medical University, Chongqing, China
e
Department of Respiratory Medicine, Children’s Hospital, Chongqing Medical University
f
Department of Pathology, Children's Hospital of Chongqing Medical University, Chongqing, China

a r t i c l e i n f o a b s t r a c t

Article history: Objective: This study sought to summarize the diagnostic and treatment aspects of congenital diaphragmatic
Received 17 June 2014 eventration (CDE) in children by retrospectively analyzing their medical records to identify and understand
Received in revised form 13 August 2014 the complications of CDE, its treatment, and to evaluate the long-term outcomes of diaphragmatic plication.
Accepted 3 September 2014 Methods: The medical records of children who received treatment for CDE from January 2000 to December
2011 at the Children's Hospital of Chongqing Medical University, China were analyzed. Data analyzed included
Key words:
the following: age, sex, symptom, location of eventration, associated anomalies, surgical procedures, complica-
Children
Congenital diaphragmatic eventration
tions, and survival and follow up details after diaphragmatic plication.
Plication Results: The medical records of 177 children (boys: 128, girls: 49, mean age: 10.28 ± 2.35 months) with CDE
Respiratory distress were included in this study. Specific symptoms of eventration of the diaphragm were reported for 86 cases;
and the typical symptoms included rapid breathing, vomiting, and recurrent respiratory infections. Except for
a bilateral case, all the other patients had unilateral CDE. Associated malformations were observed in 31 cases
(17.5%), hypoplastic lung (10 cases) was the most common followed by congenital heart disease (9 cases), and
cryptorchidism (3 cases). Interestingly, 91 patients were asymptomatic. Diaphragmatic plication was performed
in all symptomatic patients (86 cases, 48.5%) and none had recurrence.
Conclusions: Clinical symptoms of CDE varied in severity, ranging from asymptomatic conditions to life-
threatening respiratory distress. Timely accurate diagnosis and treatment of symptomatic CDE could effectively
resolve respiratory morbidity and reduce complications. The diaphragm plication surgery provided good results
among the study population with no recurrence.
© 2015 Elsevier Inc. All rights reserved.

Eventration of the diaphragm was first described by Jean Louis
Petit in 1774 [1]. The reported incidence is 1 per 1400 patients who
had chest radiographs and it was more common in males than fe-
males [2]. Clinical manifestations of congenital diaphragmatic
eventration (CDE) in infants and children are diverse, ranging from
asymptomatic conditions to mild gastrointestinal disorders such as
nausea and vomiting to sometimes life-threatening respiratory dis-
tress [3]. The CDE may easily be missed or misdiagnosed. Early diag-
nosis and repair of the diaphragm can prevent gastrointestinal
disorders, reduce recurrent respiratory tract infections, and improve
quality of life [4,5]. The purpose of this study was to summarize
the clinical manifestations, physical examination findings, and radio-
☆ Supported by National Natural Science Foundation of China (No. 81070475, No.
81100414, No. 30872706) and Chongqing Science and Technology Commission
(CSTC, 2011BA5036).
⁎ Corresponding author at: Room 806, Kejiao Building (NO.6 Building), No.136, 2nd
Zhongshan Road, Yuzhong District, Chongqing City, China. Tel.: +86 23 63633264;
fax: +86 23 63622754.
logical features of children with CDE in order to identify the opportu-
nities to improve the diagnostic and treatment aspects and to
evaluate the long-term outcomes of diaphragmatic plication.
1. Patients and methods
1.1. Study design
This retrospective analysis involved the review of medical re-
cords of children who received treatments for CDE from January
2000 to December 2011 at the Children's Hospital of Chongqing Medical
University (Department of Cardiothoracic Surgery), the largest child-
care hospitals in southwestern China.
The diagnosis of CDE was established in all cases by routine chest ra-
diographs and fluoroscopy. Patients were excluded from the study if
eventration was related to phrenic nerve injury at delivery or hypother-
mia for cardiac surgery. Initial treatment of patients included upright
positioning, supplemental oxygen therapy, treatment of pneumonia if
present, and nutritional support in symptomatic children.

http://dx.doi.org/10.1016/j.jpedsurg.2014.09.055
0022-3468/© 2015 Elsevier Inc. All rights reserved.
 S. Wu et al. / Journal of Pediatric Surgery 50 (2015) 1088–1092 1089

Fig. 1. Age distribution of children at the time of diagnosis of congenital diaphragmatic eventration.

Patients who failed to respond to conservative treatment were sub-
jected to surgery. Diaphragmatic plication was performed through a
posterolateral thoracotomy using a contralateral single lung ventilation
in right CDE, and through an abdominal approach in left CDE. The
hemidiaphragm was transected approximately 5 cm initially to avoid
intra-abdominal organ injury and then plicated from medial to lateral
with a series of six to eight parallel U sutures (2-0 polypropylene)
until it became taut and flat.
The medical history, physical examination findings, chest X-ray,
flouroscopy or ultrasonography and thorax spiral computed tomography
(CT) or magnetic resonance imaging (MRI) in the preoperative period
and during follow up period after the surgical plication were reviewed.
We also assessed with standard pulmonary function tests (PFT), in-
cluding: percent predicted forced vital capacity (FVC%), percent predicted
forced expiratory volume in 1 s (FEV1%) and percent predicted maximum
forced inspiratory flow (FIFmax%). All PFT results were collected in the
upright position at the following intervals: preoperatively, 1 month
after diaphragmatic plication, and 2 years after diaphragmatic plication.
1.2. Ethics
41 min to 12.7 years with a mean age of 10.28 ± 2.35 months and
peak age was 0–3 months (71/177). The age distribution of children
at the time of diagnosis of CDE is shown in Fig. 1.
2.2. Symptoms of eventration of the diaphragm
Specific symptoms of eventration of the diaphragm were reported
for 86 of 177 cases. The main symptoms of CDE in infants included the
following: rapid breathing, vomiting, difficult breathing, tachycardia or
arrhythmia, and cyanosis. The main symptoms of CDE in children aged
N12 months included the following: chest tightness, recurrent respira-
tory tract infections, and cough or expectoration. Few patients reported-
ly had two or three symptoms simultaneously. In the present study, the
most common symptom of CDE across all age groups of children was
rapid breathing (69 cases, 38.9%), which was followed by vomiting
(42 cases, 23.7%), recurrent respiratory infections (39 cases, 22.0%),
and difficulty breathing (21 cases, 11.8%). About 91 cases were
found to asymptomatic or accidentally discovered on routine physical
examination (51.4%, Table 1).

The Human Research Ethics Committee of the hospital approved the
study protocol.
1.3. Data collection
The details such as age, sex, symptoms, location of eventration, asso-
ciated anomalies, surgical procedures, complications, survival and fol-
low up details including improvement in symptoms and pulmonary
function, complications of surgery, and imaging results of diaphragm
were collected, analyzed, and compared.
2.3. Imaging of eventration of the diaphragm
The chest x-ray with CDE showed unusual increase in basal seg-
ments on the lesion side of diaphragm (Fig. 2A). Defect in eventration,
which was clearly seen on the lesion side of diaphragm with fluorosco-
py, had resulted in the sudden changes in breathing. In addition, an
upper gastrointestinal contrast, chest computed tomography scan, mag-
netic resonance imaging scan, ultrasound scan, or isotope scan could
also help to identify diaphragmatic paralysis and diaphragmatic
hernia (Fig. 3).

1.4. Statistics 2.4. Localization of congenital diaphragmatic eventration

Statistical analyses were performed using SPSS software version
17.0 (SPSS Inc. Chicago, IL). Categorical data were compared using chi-
square test. Follow-up data are compared with preoperative values
using Student’s t-test for paired and unpaired data as appropriate. A
p value of b0.05 was considered statistically significant.
CDE was unilateral in 176 cases and bilateral in one case. Of the 176
unilateral CDE, 52 (29.55%) were found on the left side and 124
Table 1
Symptoms of congenital diaphragmatic eventration in children (n = 177).

Symptoms No. of cases Rate (%)

2. Results Rapid breathing 69 38.9
Vomiting 42 23.7
2.1. Sex distribution and age at diagnosis Recurrent respiratory infections 39 22.0
Difficulty breathing 21 11.8
Chest pain 10 5.6
During the study period of 12 years, medical records of 182 children
Tachycardia 8 4.5
were reviewed. Two patients in whom eventration was related to Arrhythmia 4 2.2
phrenic nerve injury at delivery and 3 children in whom eventration Cyanosis 3 1.6
was related to hypothermia for cardiac surgery were excluded from Other non-specific symptoms 13 7.3
the study. The remaining 177 patients (boys: 128, girls: 49) who re- Asymptomatic 91 51.4

ceived treatment for CDE were analyzed. Patients’ age ranged from Rate meant the probability of one specific symptom appearing in 177 patients.
1090 S. Wu et al. / Journal of Pediatric Surgery 50 (2015) 1088–1092
Fig. 2. Preoperative chest radiograph of a child with CDE shows a raised right hemidiaphragm (A). A radiograph taken in postoperative recovery period shows the right hemidiaphragm in
normal position (B).
(70.45%) were on the right side. The defect was focal in 102 cases and
affected the entire diaphragm in 75 cases (Table 2). Of the 102 focal
CDE, 91 were asymptomatic, and 11 had specific symptoms.
2.5. Treatment of congenital diaphragmatic eventration
Of the 177 patients with CDE, 91 were asymptomatic and all symp-
tomatic patients (86 cases) received treatment by diaphragmatic plica-
tion (age ranged from 1.5 days to 6.3 years with a mean age of 2.42 ±
0.17 months). Central portion of diaphragm in all patients under sur-
gery was biopsied for histopathological examination. Histopathological
examination revealed relatively thin and weak (like a semi-permeable
membrane) fibers suggesting diaphragmatic fibrous dysplasia. One pa-
tient had phrenic nerve lesions with thickened nerve (Fig. 4).
2.6. Associated malformations
About 31 cases (17.51%) had other associated malformations such as
hypoplastic lung, congenital heart disease, pectus excavatum, cleft pal-
ate, hypospadias, cryptorchidism, and congenital torticollis (Table 3).
The most common malformations associated with CDE was hypoplastic
lung (10 cases, 5.6%), which was followed by congenital heart disease
(9 cases, 5.08%) and cryptorchidism (3 cases, 1.6%).
2.7. Follow-up data
The follow-up data were documented for 166 patients, including 80
patients who were asymptomatic and 86 patients who underwent
Fig. 3. Upper gastrointestinal contrast study shows a raised right entire hemidiaphragm (A) and a raised left entire hemidiaphragm (B).
plication surgery. The other 11 patients were lost to follow-up. The me-
dian age at follow-up was 4.7 years (range: 0.3 to 11.5 years), and the
median duration of follow-up (from the day of surgery until the last
follow-up) was 5.7 years (range: 1 month to 9.2 years).
Complications of plication surgery identified during the follow-up
included pneumonia and pleural effusions. Most common postoperative
complication was pleural effusion (13/86, 15.1%), followed by pneumo-
nia (5/86, 5.8%).
Patients had been followed-up radiologically annually to demon-
strate the position of the diaphragm, and symptoms if any were also
evaluated. Almost all respiratory and digestive symptoms disappeared
within 1 month of operation, and none had any symptom 1 year after
surgery (Table 4).
Compared with preoperative scores, we found significant improve-
ments in all components of the PFT at 1 month after diaphragmatic
plication; this improvement was maintained at 2 years post
surgery (Table 5).
3. Discussion
CDE is a rare congenital developmental defect in all or part of the
muscular portion of the diaphragm in which the diaphragmatic muscle
is replaced by fibroelastic tissue and its attachment to the sternum, ribs,
and dorsolumbar spine is affected, leading to the displacement of weak-
ened hemidiaphragm into the thorax [6]. Embryologically, the etiology
of CDE is postulated as the abnormal migration of myoblasts from the
upper cervical somites into two of the four embryological structures
that contribute to diaphragm development such as septum transversum
 S. Wu et al. / Journal of Pediatric Surgery 50 (2015) 1088–1092 1091

Table 2 Table 3
Localization of congenital diaphragmatic eventration. Associated malformations with congenital diaphragmatic eventration (n = 177).

Partial CDE Diffuse CDE Malformation No. of cases Rate (%)

Left CDE 11 41 Hypoplastic lung 10 5.65

Right CDE 91 33
Bilateral CDE – 1
Total 102 75
(beginning at 4 weeks of gestation) and pleuroperitoneal membrane
(at 8 to 12 weeks of gestation) [7,8]. Thomas hypothesized the involve-
ment of impaired growth of myoblast into pleuroperitoneal membrane,
when the abdominal viscera return to the peritoneal cavity prematurely
[9]. In the present study, most symptomatic patients with CDE had dif-
fuse fibroelastic changes and paucity of muscle fibers. Only one patient
had thicker nerve with a normal (albeit atrophic) amount of muscle fi-
bers, and the characteristics of his diaphragmatic paresis or paralysis
were found to be consistent to adults. The most common causes of dia-
phragmatic paresis are idiopathic, tumor encroachment on the phrenic
nerve, and phrenic nerve trauma from cardiac surgery [10].
CDE is more common among males [2,11]. In this study, the male
predominance was observed. The diaphragmatic abnormality can be ei-
ther localized or diffuse. In the diffuse variety, the diaphragm consists of
a thin, diaphanous membrane that is attached peripherally to the nor-
mal muscle. Partial defects mostly affect the right hemidiaphragm
(65%) [9], while diffuse defects tend to be unilateral and more common-
ly affect the left side. But, there were more cases affecting the right side
than left (124 vs. 52, the left: right ratio of 0.42:1). This could be attrib-
uted to the reason that the majority of right CDE was partial.
Early clinical manifestations of CDE are not obvious and nonspecific,
and they could often lead to misdiagnosis in a considerable number of pa-
tients. In the current study, 51.4% cases were asymptomatic. Asymptom-
atic noniatrogenic diaphragmatic eventrations occur in 7 to 35 per
100,000 persons and were noted in 1 per 10,000 healthy military recruits.
The main symptoms in CDE are due to the elevated intra-abdominal
organs resulting in compression of the lower lobe of the lung and caus-
ing dyspnea, cyanosis, acute respiratory distress, vascular dysfunction,
and cardiac symptoms. Anorexia, nausea, and vomiting may also be as-
sociated. We hypothesized that CDE causes these problems in 5 ways:
(1) inability to ventilate the ipsilateral lung; (2) direct compression of
the ipsilateral lung; (3) pneumonia due to chronic atelectasis;
(4) pendelluft due to ventilation between the affected and the unaffect-
ed lung; and (5) paradoxical motion of the diaphragm to make the ven-
tilation of the contralateral lung inefficient. In infants, the mediastinum
is very mobile, and the paradoxical movement of the affected dia-
phragm causes a shift of the heart and mediastinum towards the contra-
lateral thorax. In unilateral CDE, the vital capacity and total lung
Congenital heart disease: 9 5.08
(1) Atrial septal defect 6 3.39
(2) Ventricular septal defect 2 1.13
(3) Dextrocardia 1 0.56
Pectus excavatum 2 1.13
Microtia 2 1.13
Cleft lip 2 1.13
Cleft palate 1 0.56
Congenital muscular torticollis 1 0.56
Hypospadias 1 0.56%
Cryptorchidism 3 1.69%
Total 31 17.51%
Associated malformation rate meant the probability of one specific malformation
appearing in 177 patients.
capacity are reduced by 20% to 30% resulting in chest tightness, recur-
rent respiratory tract infections, and cough with or without expectora-
tion. In the current study, 22.03% of patients had recurrent respiratory
infections. Bilateral diaphragmatic eventration reduces the lung func-
tions even more seriously, especially in the supine position [12]. Al-
though such children can tolerate bilateral diaphragmatic eventration,
they are prone to chronic respiratory failure.
CDE can be isolated or associated with other developmental defects.
The important associated findings with CDE include hypoplastic lung,
congenital heart disease, pectus excavatum, cleft palate, hypospadias,
cryptorchidism, and congenital torticollis [13,14]. The results revealed
hypoplastic lung (10, 5.65%), and congenital heart disease (9, 5.08%),
as the major associated anomalies in this study.
The typical clinical manifestations may lead to the diagnosis of CDE,
which is often confirmed by chest x-ray and fluoroscopy. The symptom-
atic CDE in children usually requires surgical treatment [5,15–18]. Indi-
cations for surgery include: (1) rapid breathing without improvement
under a conservative treatment; (2) two or more recurrent ipsilateral
pneumonias; (3) one life-threatening pneumonia; (4) inability to
wean from mechanical ventilation; (5) respiratory distress related to
paradoxical motion of the diaphragm. Diaphragm plication is the surgi-
cal option for CDE, which was first successfully done in an adult in 1923
by Jean Louis Petit and infant in 1946 by Bisgard. The goal of surgery is to
flatten the dome of the diaphragm and provide the lung with greater
volume for expansion. Since its original description, diaphragm plica-
tion has been performed with numerous modifications, including the
minimally invasive video-assisted thoracic surgery approach [19,20].
In this study, the patients who underwent diaphragmatic plication for
symptomatic CDE were followed up for an average period of 5.7 years,
and it was found that diaphragmatic plication re-expanded the

Fig. 4. Histopathological examination shows a relatively thin and weak (like semi-permeable membrane) diaphragmatic fibrous dysplasia.
1092 S. Wu et al. / Journal of Pediatric Surgery 50 (2015) 1088–1092

Table 4 Table 5
Symptoms of congenital diaphragmatic eventration pre- and post-operation (n = 86). PFT results before and after laparoscopic diaphragmatic plication (n = 6).

Symptoms Pre-operation Post-operation Post-operation PFT Preoperative Postoperative (1 month) Postoperative (3 years)
(N) (1 month) (N) (1 year) (N)
FVC% 45.4 ± 10.6 61.6 ± 8.3 ⁎ 65.1 ± 12.7 ⁎
Rapid breathing 69 4 0 FEV1% 48.7 ± 11.2 62.7 ± 12.8 ⁎ 64.0 ± 11.2 ⁎
Vomiting 42 2 0 FIFmax% 90.3 ± 37.2 110.8 ± 30.6 ⁎ 113.6 ± 32.3 ⁎
Recurrent respiratory infections 39 1
PFT, pulmonary function tests; FVC%, percent predicted forced vital capacity; FEV1%, per-
Difficulty breathing 21 0 0
cent predicted forced expiratory volume in 1 s; FIFmax%, percent predicted maximum
Chest pain 10 0 0
forced inspiratory flow.
Tachycardia 8 0 0
⁎ Significantly different (P b0.05) from preoperative results.
Arrhythmia 4 0 0
Cyanosis 3 0 0

References

atelectatic lung, improved respiratory and digestive symptoms, and im-
proved the quality of life.
4. Conclusions
The clinical symptoms of CDE varied in severity, ranging from
asymptomatic conditions to life-threatening respiratory distress. Timely
surgical treatment should be considered for most symptomatic patients,
especially for patients with recurrent pulmonary infections. The dia-
phragm plication surgery provided good results among the study popu-
lation with no recurrence.
What is already known on this topic
(1) Congenital diaphragmatic eventration (CDE) is a relatively rare
congenital malformation, in which symptoms vary in severity,
ranging from asymptomatic to life-threatening respiratory
distress.
(2) CDE may be easily missed or misdiagnosed.
What this study adds
(1) Timely diagnosis and repair of the diaphragm in CDE can prevent
gastrointestinal disorders, reduce recurrent respiratory tract in-
fections and improve quality of life.
(2) The long-term outcomes following diaphragmatic plication are
usually good.
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