International Journal of Surgery Case Reports 114 (2024) 109036

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International Journal of Surgery Case Reports

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

Case report

Two cases of right congenital diaphragmatic hernia with stable respiratory

condition: Two case reports
Seiichiro Inoue *, Yuki Muta, Yuta Takeuchi, Akio Odaka
Department of Hepato-Biliary-Pancreatic Surgery and Pediatric Surgery, Saitama Medical Center, Saitama Medical University, 1981 Kamoda, Kawagoe, Saitama 350-
8550, Japan

A R T I C L E I N F O A B S T R A C T

Keywords: Introduction: Right congenital diaphragmatic defect (CDH) has been reported poor prognosis. However, laterality
Right congenital diaphragmatic hernia of the defect as the prognostic factor is recent controversial topic. We experienced two cases of right CDH with
relatively stable respiratory condition and good clinical course.
Presentation of cases: Case 1 was a girl diagnosed with right CDH by fetal ultrasonography and delivered by
planned caesarian section at 37 weeks, 3 days. Fetal MRI showed liver herniation into the right thoracic cavity.
High frequency oscillatory ventilation with nitric oxide gas was administered until day 5. At surgery on day 8, we
found defects in the right posterolateral diaphragm and sac herniation of the right side of the liver into the right
thoracic cavity. The postsurgical course was uneventful, and she was discharged on day 41. Case 2 was a girl with
suspected congenital jejunal atresia after fetal ultrasonography detected polyhydramnios and dilatation. She was
delivered by normal vaginal delivery at 38 weeks, 5 days, and thoraco-abdominal X ray showed right CDH but no
small intestinal atresia. Surgery performed on day 3 found liver herniation into the diaphragmatic defect.
Subsequently, bacterial infection occurred and was treated with the antibiotics, but her respiratory condition
remained stable. She was discharged on day 49.
Discussion: The volume of herniated abdominal organs is affected by the presence of a hernia sac or the size of the
diaphragmatic defect.
Conclusion: The size of diaphragmatic defect, but not the laterality of the diaphragmatic defect, may be an
important prognostic factor in right CDH.

1. Introduction some recent studies found no significant difference in mortality or

Congenital diaphragmatic hernia (CDH), a congenital defect of dia­
phragm, is defined as an abnormality in the integrity of, a discontinuity
in, or an under-muscularization of the diaphragm. Herniation of
abdominal viscera into the thoracic cavity induce sever damage to the
respiratory and cardiovascular system of neonates. About 85 % of CDH
were observed in the left side, and right congenital diaphragmatic hernia
(rCDH) has an incidence ratio of only 10 % to 20 % compared with left
CDH (lCHD) [1–7]. It was reported to have a poor prognosis [3,8] and
high mortality [1]. In 2008 Fisher et al. reported the survival rate of
rCDH 55 %, which were less compared to lCDH 85 % [1]. Recent
nationwide cohort study performed in France also reported the survival
rate was 57 % for rCDH and 77 % for lCDH with statistical difference (P
< .01) [6]. However, late-onset rCDH has also been reported [9], and
morbidity if lung volume was sufficient and appropriate treatment was
performed [4,7,10].
Here, we present two cases of rCDH with mild clinical courses and
relatively stable respiratory condition. The work is reported in line with
the SCARE criteria [11].
2. Presentation of cases
2.1. Case 1
Case 1 was a girl delivered by planned caesarian section at 37 weeks
and 3 days. Her birth weight was estimated as 3400 g, and her Apgar
scores were 4 at 1 min and 6 at 5 min. At 32 weeks of gestation, fetal
ultrasonography (US) showed polyhydramnios and a mass in the right

* Corresponding author at: Department of Pediatric Surgery, Saitama Medical Center, Saitama Medical University, 1981 Kamoda, Kawagoe, Saitama 350-8550,
Japan.
E-mail address: sei_khsr@saitama-med.ac.jp (S. Inoue).

https://doi.org/10.1016/j.ijscr.2023.109036
Received 3 October 2023; Received in revised form 7 November 2023; Accepted 8 November 2023
Available online 18 November 2023
2210-2612/© 2023 The Authors. Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license
(http://creativecommons.org/licenses/by-nc-nd/4.0/).
S. Inoue et al. International Journal of Surgery Case Reports 114 (2024) 109036

thoracic cavity. Congenital pulmonary airway malformation of the fetal
right lung was suspected, and the mother was referred to our center. At
32 weeks and 1 day, the fetus was diagnosed with rCDH by US. At 34
weeks and 1-day, magnetic resonance imaging (MRI) suggested rCDH
with liver herniation (Fig. 1a). Findings of fetal hydrops was not
observed, and fetal upper lobe of right lung was observed in MRI, Pe­
diatric surgeons, neonatologists and obstetricians discussed and judged
that maternal administration of glucocorticoids was not essential.
Observed/expected lung-to-head ratio (e/o LHR) was estimated as 59.6
% at 35 weeks and 6-day.
After birth, fentanyl and rocuronium were administered through the
umbilical cord vessels, and the patient was orally intubated. High fre­
quency oscillatory ventilation (HOFV) was administered (mean airway
pressure, 15; spontaneous ventilation, 16; fraction of inspired oxygen,
1.0) with nitric oxide gas (NO gas: maximum concentration, 20 ppm).
Chest X-ray revealed herniation of the abdominal organs through the
right diaphragm, deviation of the mediastinum to the left, and aeration
of the apex of the right lung (Fig. 1b). Respiratory stabilization was
performed, and HFOV with NO gas were discontinued on day 5.
Direct closure of the diaphragmatic defect with laparotomy was
performed on day 8. During surgery, a defect was seen in the right
posterolateral diaphragm, with herniation of the right side of the liver
into the right thoracic cavity through the defect (Fig. 1c). The dia­
phragmatic defect was 4.5 × 2.0 cm in size, and a hernia sac was
observed (Fig. 1d). After resection of the hernia sac, the residual
muscular parts were sutured together, and the sutured diaphragm was
covered with a dual mesh patch. The postoperative course was un­
eventful. Five days after surgery, the tracheal tube was extubated, and
the patient was discharged on day 41 after delivery. The patient is still
being followed up, but no signs of recurrence have been observed.
Pathological examination of the resected sac showed that it was
composed of membranous fibrous tissue covered with mesothelial cells,
which is compatible with CDH with a sac.
2.2. Case 2
Case 2 was a girl delivered by normal vaginal delivery at 38 weeks
and 5 days. Her birth weight was 2981 g, and her Apgar scores were 8 at
1 min and 9 at 5 min. At 34 weeks of gestation, fetal US showed poly­
hydramnios and dilatation of the upper gastrointestinal tract, and
congenital jejunal atresia was suspected.
Chest X-ray after birth revealed herniation of the abdominal organs
through the right diaphragm (Fig. 2a), and US showed that the herniated
organs included the liver. The patient was diagnosed with rCDH and
orally intubated, and on day 1 after birth, HOFV was started (mean
airway pressure, 13; spontaneous ventilation, 25; fraction of inspired
oxygen, 0.21). Abdominal X ray on day 2 showed that intestinal gas was
reaching the small intestine (Fig. 2b), and on day 3, it revealed that
intestinal gas was reaching the rectum and that no intestinal obstruction
was present. Thoraco-abdominal enhanced computed tomography on
day 2 (Fig. 2c) showed that the small intestine and right posterior upper
part of the liver were herniated into the right thoracic cavity. However,
the mediastinal shift to the left side of the thorax was not severe, and
aeration of the upper lobe of the right lung was observed (Fig. 2d). The
upper jejunal wall appeared to be pressing between the herniated liver
and the ridge of diaphragmatic defect.
On day 3 after delivery, direct closure of the diaphragmatic defect
with laparotomy was performed. During surgery, a defect was seen in
the right posterolateral diaphragm (Fig. 2e), through which almost the
whole small intestine, ascending colon, right upper posterior side of the

Fig. 1. Findings in Case 1.

(a) Fetal magnetic resonance image obtained at 34 weeks and 1 day of gestation detected herniation of the liver into the thoracic cavity (→) and decreased volume of
the right upper lobe of the lung (➡). (b) Chest-abdominal X-ray at birth detected abdominal visceral herniation into the intra-thoracic cavity (↑), a mediastinal shift to
the left, and aeration of the apex of the right lung (➡). (c) The right side of the liver was elevated into the right thoracic cavity through the defect in the right
diaphragm. (d) The upper lobe of the right lung was visible after resection of the hernia sac.

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S. Inoue et al. International Journal of Surgery Case Reports 114 (2024) 109036

Fig. 2. Findings in Case 2.

(a) Although jejunal atresia was suspected by the fetal ultrasonography, chest X-ray after birth showed herniation of the abdominal organs through the right dia­
phragm. (b) X-ray on day 2 showed that intestinal gas was reaching the lower intestine, confirming the absence of jejunal atresia. (c, d) Thoraco-abdominal computed
tomography performed on day 2 after birth revealed that the mid-part of the liver and the small intestine were herniated into the right thoracic cavity and showed the
matured upper lobe of the right lung (▴). (e) At surgery, the right upper posterior side of the liver was found to be impacted into the posterolateral defect of the right
diaphragm. (f) No hernia sac was present, and the small intestine, small part of the colon, part of the liver, and the gall bladder were herniated into the right
thoracic cavity.

liver, and gall bladder had herniated into the right thoracic cavity
(Fig. 2f). The diaphragmatic defect was 3.5 × 2.0 cm in size. No hernia
sac was observed.
Two days after surgery, the patient developed sepsis due to an
extended-spectrum beta-lactamases–producing bacterial infection, and
the antibiotic meropenem was administered. The patient recovered, and
the tracheal tube was extubated on day 7 after surgery. The patient was
discharged on day 49 after delivery. The patient is still being followed
up, but no signs of recurrence have been observed.
3. Discussion
An earlier study found that in fetuses with CDH, the diaphragmatic
defect was on the left in 85 % of cases, on the right in 15 %, and bilateral
in less than 1 % [3]. In data from 189 patients with CDH, 20.1 % had
rCDH (37 of 189 CDH patients) [4]. The clinical prognosis of rCDH is
worse than that of lCDH [1,3,8] and related to delayed diagnosis and
liver herniation, which are associated with worsening pulmonary hy­
pertension [8]. The prognosis may also be worse because a higher pro­
portion of patients have a large diaphragmatic defect [5]. Dekoninck
et al. reported that even after fetal surgery by fetal endoscopic tracheal
occlusion, rCDH has a worse outcome than lCDH in patients with a
similar lung size before birth [3]. Thus, rCDH might be a separate entity
and not simply a variation of typical left lateral CDH with a different
outcome. Cases of late-onset rCDH with mild respiratory symptoms were
reported [9]. Although rCDH has some unique risk factors, such as less
frequent prenatal diagnosis [2,4,8], more liver herniation [2,4,8], larger
diaphragmatic defect [5] that often requires patch closure [2,12], and
the severe complication of hepato-pulmonary fusion [13], some studies
found no significant differences in survival between rCDH and lCDH
with proper treatment [2,4,7,12], and one group suggested that later­
ality of the CDH defect might be not significantly associated with CDH
mortality [6].
The existence of a hernia sac in CDH may indicate a better prognosis
because it limits herniation of the abdominal organs, allowing the fetal
lung to grow more and resulting in a better postnatal outcome [14–16].
From fetal magnetic resonance imaging and ultrasonography data,
Oliver et al. showed that in CDH, a hernia sac is associated with a higher
fetal lung volume in the preterm period, shorter postnatal stay in the
neonatal intensive care unit, decreased need for mechanical ventilation
and extracorporeal membrane oxygenation, lower risk of pulmonary
hypertension, and lower rates of patch repair at surgical treatment;
however, they did not provide the details of the diaphragmatic defects
[16]. In Case 1 presented here, rCDH was accompanied by a sac, which
may explain the good clinical course after birth because the sac
appeared to have limited the herniation of abdominal organs and
allowed development of the upper lobe of the right lung.
The size of the diaphragmatic defect [17,18] and liver herniation
[15,19] are also important prognostic factors for CDH, and liver herni­
ation occurs frequently in rCDH [2,4,8]. A large diaphragmatic defect
and massive herniation of abdominal viscera compresses the fetal lung,
affecting its development. Additional prognostic factors in CDH are fetal
development and lung volume [10], especially in case of liver herniation
[20]. Liver herniation into the right chest cavity was observed in both
our cases. As mentioned above, in Case 1 the hernia sac prevented
massive herniation of the liver, allowing better development of the

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S. Inoue et al.
upper lobe of the right lung. In Case 2, the right diaphragmatic defect
was small, so the central part of the liver plugged the defect and pre­
vented herniation of the rest of the liver and almost all of the small in­
testine, enabling development of the upper lobe of the right lung. In both
cases, the matured upper lobes of the right lung are considered to have
contributed to the stable respiratory condition after birth. In both cases,
the gestation week was also satisfactory, which were also considered to
affect to their good clinical course.
4. Conclusion
We experienced two cases of rCHD with a good clinical course.
Massive herniation of abdominal organs was prevented in one case by
the presence of a hernia sac and in the other by impaction of part of the
herniated liver into the diaphragmatic defect, allowing growth of the
upper lobe of the right lung. The volume of the herniated abdominal
organs, but not the laterality of the diaphragmatic defect, may be an
important prognostic factor in rCDH.
Ethics approval
Approval for this study was obtained from the ethics committee of
Saitama Medical Center, Saitama Medical University, Japan (Research
registry number 2505).
Funding
This research did not receive any specific grant from funding
agencies in the public, commercial, or not-for-profit sectors.
Author contributions and authorship
Seiichiro Inoue: Conceptualization, Methodology, Investigation,
Writing-Original Draft, and Project administration. Yuki Muta: Investi­
gation. Yuta Takeuchi: Investigation. Akio Odaka: Investigation,
Writing-Reviewing and Editing, and Supervision.
All authors revised the manuscript for intellectual content and gave
final approval of the version to be published. All authors attest that they
meet the current ICMJE criteria for Authorship.
Guarantor
Seiichiro Inoue.
Patient consent
Written informed consent to publish this case report and any
accompanying images was obtained from the patients' legal guardians.
This report does not contain any personal information that could lead to
identification of the patients.
Declaration of competing interest
None.
Acknowledgments
None.
International Journal of Surgery Case Reports 114 (2024) 109036
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