86
CASE REPORT
Right sided congenital diaphragmatic hernia, an operative
challenge
Abdul Rahman Almaawi, Rama Krishnaprasad Davuluri, Zakaullah Waqasi, Ghassan Alkouder,
Abdulla Alsharani, Ahmed Aref, Ahmed Safwat
Abstract
A diaphragmatic hernia is an abnormal communication between abdominal and thoracic
cavities, with or without contents in the thorax. The incidence of congenital diaphragmatic
hernia is 1 in 2000 to 1 in 5000 live births. The right sided diaphragmatic hernia (20%) is rare,
compared to Left sided diaphragmatic hernia (80%).Congenital diaphragmatic hernia Usually
refers to a posteriolateral defect of Bochdalek and other forms are rare. The usual Presentation
is with respiratory distress in the neonatal period (90%) and delayed presentations are also
reported. Ante-natal diagnosis, fetosopic intervention, ECMO, and intensive neonatal ventilator management techniques has improved the survival rates in these neonates.
The case under discussion has a unique combination of severe pulmonary hypoplasia, herniation of the entire liver into right hemithorax, and near total absence of the entire Right hemidiaphragm.
Keywords: Congenital diaphragmatic hernia, defect of Bochdalek, pulmonary hypoplasia,
herniation of liver into right hemithorax
Department of Pediatric
Surgery, King Abdullah
Hospital, Bisha, Saudi
Arabia
AR Almaawi
RK Davuluri
Z Waqasi
G Alkouder
Neonatal Intensive Care
Unit, King Abdullah
Hospital, Bisha, Saudi
Arabia
A Alsharani
A Aref
A Safwat
Correspondence:
Dr. Abdul Rahman
Almaawi
PO Box 60, King Abdullah
Hospital, Bisha, Saudi
Arabia
email: almaawi@yahoo.
com
Introduction:
Embryologically the diaphragm develops during
the 8th and 12th week of gestation. Septum transversum separates the thoracic from abdominal
cavity, later muscle fibres migrate in to this membrane. Left side defects are more common due to
the late closure of this membrane. The loss of lung
development can be explained by loss of space to
develop, but other structural deficiencies are added bronchial, vessel and alveolar membrane deficiencies. Because the abdominal contents have
migrated, the abdominal cavity will not grow as
much. Then after surgical correction it may be
difficult to reduce all organs in to abdominal cavity without pressure. Prenatal imaging provides
valuable information. The diagnosis of CDH can
be made prenatally by ultrasonography in 90% of
cases.1 Typically this is diagnosed at the 24th week
of gestation, but some have reported diagnosing it
as early as 11th week.2 Fetal ultrasound findings include polyhydramnios, bowel loops in the chest,
echogenic chest mass.
Two distinct features have been used to risk
stratification; low lung to head ratio (LHR) and
liver herniation in to the chest.
LHR in accordance with age is well defined.
LHR more than 1.35 is considered as borderline or high risk group at 28 weeks of
gestation.3Survival based on liver herniation
alone is 43% as compared to 93% survival without herniation.4Foetal MRI has been used as an
adjunct to evaluation of foetal lung volume and
liver herniation. Prenatal diagnosis of CDH provides an opportunity to optimize the prenatal
and post natal care. Newborns with CDH present with respiratory distress. These infants will
have a scaphoid abdomen and increased chest
diameter. Bowel sounds can be heard in the
chest. The diagnosis of CDH is made with ease
by chest radiograph.
Initial postnatal therapy is targeted at resuscitation and stabilization of infant in cardiopulmonary distress. In severe cases prompt
Pak J Surg 2015; 31(1):86-88
87
Right sided congenital diaphragmatic hernia, an operative challenge
Figure 1: Xray chest showing Right Figure 2: CT Scan of chest showing Figure 3: Chest Xray on first post op- Figure 4: Chest Xray before dishcarge
Congenital Diaphragmatic Hernia
liver at the level of heart
erative day with no intercostal drain
endotrachial intubation is warranted. Initial mechanical ventilation should be utilised for stabilisation. Pulmonary hypertension and associated
cardiac anomalies are evaluated with echocardiography. The timing of operative procedure is
based on clinical judgment and surgeon’s discretion
Case report:
Respiratory distress in the new born is a life
threatening condition and if not treated promptly can be fatal. We present a case of a newborn
male child born by caesarean section (weight
-3.1 kg, Apgar Score 6 at 5 mins.). The neonate
was promptly resuscitated, and x ray chest and
CT scan revealed a right sided congenital diaphragmatic hernia. 2D echo showed Pulmonary
Hypertension.New born was taken up for surgery after stabilization.
Surgery was performed with abdominal approach. The entire liver was intra-thoracic along
with intestinal loops. Stomach and spleen were
found in the abdomen. Only a thin rim of diaphragm on the anterior aspect was present, and
the posterior rim was absent. The right lung was
very small in size. The liver could be brought
down to the abdomen with difficulty, and the
intestinal loops were reduced. Repair of the
diaphragm was achieved by placing sequential
sutures through the anterior rim and the pleural
flap. Onlay gortex patch was kept. The abdomen
was closed primarily without much tension.
Post operatively baby was returned to NICU
and kept on High Frequency Oscillatory Ventilation. The baby was started on nasogastric feed
on the fourth post operative day, initially half
strength, and later full feeds. The chest x ray on
the seventh post operative day has shown partial
Pak J Surg 2015; 31(1): 86-88
showing good right lung expansion
expansion of right lung, and was weaned of from
ventilation after two weeks. The baby had a remarkable recovery and was discharged on twentieth post operative day, and is under follow-up.
Discussion:
Historically CDH was regarded a surgical emergency and the new born was rushed to the Operating Room for surgical correction. In 1987
Bohnetal5proposed a period of medical stabilization and delayed surgical repair in an attempt
to improve the overall condition of the neonate.
Published data on CDH largely reflect the experience of left CDH, which comprises 80%6.
Three prenatal features (liver herniation in to
thorax, low lung to head ratio, and diagnosis before 25 weeks gestation) are likely to have a poor
outcome with conventional treatment. In some
centres these patients were offered antenatal
intervention (tracheal occlusion) by foetoscpic
methods. The antenatal diagnosis of right side
defect were lower compared to left side, as this
will lead to delivery outside the institute, and
lack of early referral. The surgical repair is more
challenging in the right sided CDH, because of
universal liver herniation, as well as potential
Table 1: Repair Options for large defects13
Options
Prosthesis
Polytetrafluroethylene ( PTFE) (GORTEX)
Polypropylene (MARLEX)
Dacron
Muscle Flap
Abdominal 4
Latismus dorsi 5
Latismus dorsi and serratus anterior 10
Bioactive Material
Surgisis 6
88
AR Almaawi, RKDavuluri, Z Waqasi et.al
anatomic anamolies such as hepatopulmonary
fusion7, anamolous venous drainage uniquely
associated with right sided defects. The series
published by Fischer et al has shown the survival
rate(right CDH 55% to left CDH77%) ECMO
requirement (right CDH 40%vs left CDH 15%)
prosthetic material in R CDH vs L CDH ( 76%
vs 41%). And abdominal wall (38% vs 19%)
repairs.6These data support that right side CDH
carries a high mortality and morbidity. The repair of a CDH may be as variable as clinical
management.A subcoastal incision on the ipsilateral side is the traditional approach, less than
10% surgeons prefer a thoracic approach. The
type of repair is dependent on the size of the defect. If the defect is small, a tension free primary
surgical closure should be performed with non
absorbable sutures. If the defect is wide primary
closure may be attempted by one of the patch
methods described below.
Outcomes regarding the prosthetic patch material are mixed. Currently the most popular
material is small intestine submucosa (Surgisis
Cook Inc.) or human a cellular dermal matrix
(alloderm, life cell). The bioactive material may
act as a matrix for permanent tissue ingrowth.
Long term outcomes of biosynthetic patches for
CDH remain to be seen.post operatively tube
thoracostomy is probably not indicated.13An exception may be CDH repair on ECMO because
bleeding complication should be expected.
Even with advances in neonatal critical care, the
treatment of infants with CDH remains a challenge to paediatric surgeons, neonatologist and
intensivist. Improvement in the foetal ultrasonography revealed the true natural history of
CDH and the hidden mortality during gestation
and soon after birth. Foetal CDH interventions
evolved from open foetal surgery to current state
of endoscopic endoluminal tracheal occlusion.
Currently the technique involves the placement
of occlusion balloon, without maternal laparotomy or general anaesthesia.14 Tracheal balloons
are placed between 24 and 28 weeks of gestation
and deflated at 34 weeks.14Tracheal occlusion
continues to be investigated owing to the significant mortality of infants with LHR less than1.0
and liver herniation. The combination of entire
liver herniation in to thorax, hypoplastic right
lung, and near total absence of hemidiaphragm
on the right side posed a difficult situation for
the surgical team. Inspite of these challenges this
neonate could be salvaged because of the efforts
of neonatal Intensivists and timely intervention
of the paediatric surgeons.
Conclusion:
Congenital diaphragmatic hernia (CDH) is a
complex disease. Prenatal predictors, postnatal predictors, and the surgical techniques have
been established. Not only the survival rate with
risk of high morbidity, but good quality of life
has to be the goal in the future.
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