Summary: A 3-day-old newborn boy presented with respiratory distress and
vomiting. An urgent chest X-ray and ultrasound of the abdomen were
performed, and the prenatal ultrasound scans were reviewed. Multiple
congenital anomalies were suspected, and a postnatal CT was done to
delineate all anomalies before attempting surgery.
Congenital right-sided Bochdalek hernia
CT scans of the thorax and abdomen showed multiple air-ﬁlled bowel
loops with mesentery in the right hemithorax causing signiﬁcant
mediastinal shift to the left (Figure 1). The right kidney was seen in a
paraspinal location in the right hemithorax, and mesenteric fat with
vessels was seen in the ipsilateral renal bed (Figure 1). The left
kidney showed features of obstruction at the pelviureteric junction:
gross hydronephrotic changes and delayed contrast excretion with
nonvisualization of the left ureter (Figure 2). The stomach appeared
grossly distended with an altered axis likely due to the associated
malrotation and volvulus (Figure 2).
Further review of the CT
scans also revealed the presence of 2 venous channels on either side of
the aorta in the infrarenal segment with a retroaortic left renal vein
draining into the right-sided channel (Figure 3). The intrahepatic
inferior vena cava (IVC) and hepatic veins were well seen. These ﬁndings
suggested duplication of the IVC (with 2 similarly sized vena cavae),
and the left IVC was seen draining into the right IVC via the
retroaortic left renal vein.
Corrective surgery was attempted using a subcostal abdominal approach
(which is usually preferred over a transthoracic approach). Although no
hernia sac was noted, the diaphragmatic defect was repaired after
reduction of the herniated contents. The stomach volvulus was untwisted,
and no gastropexy was done. Correction of the left pelviureteric
junction obstruction (pyleoplasty) was reserved for a later stage. At 3-
and 6-month follow-up examinations, the infant was asymptomatic and
Congenital diaphragmatic hernia (CDH) is a complex developmental
anomaly associated with substantial morbidity and mortality despite
advances in postnatal care. The major prognostic factors are the
presence of associated structural or chromosomal anomalies and the
herniation of liver into the thorax. Similarly, the complicated
embryogenesis of the IVC provides numerous opportunities for
developmental errors. Anomalies may involve the infrarenal, renal or
suprarenal segment of the IVC. Here, we present a case of right-sided
CDH with concurrent anomaly of the infrarenal IVC causing left
pelviureteric junction obstruction.
Bochdalek hernia is a
posterolateral diaphragmatic defect resulting from failure of the
retroperitoneal canal membrane to fuse with the dorsal esophageal
mesentery and the body wall. The location of the foramina of Bochdalek
is deﬁned by the location of the diaphragmatic coronary ligaments
bilaterally. The herniation is termed “congenital” if present at birth
and is termed “acquired” if the herniation forms later, because of
extension of intra-abdominal or perirenal fat into the thorax. Most
neonatal Bochdalek’s hernias are left-sided. Left-sided hernias are more
common (70% to 90% of cases), presumably owing to the protective
effects of the liver.1
These defects can be classiﬁed
as small, medium, and large on CT examinations (the large ones are
identiﬁable on ≥5 contiguous axial scans consisting of 1-cm-thick
sections).2 It presents as an echogenic lung mass during the
prenatal period, and other differential diagnoses include cystic
adenomatoid malformation (CAM), sequestrated lung, and tracheal or
bronchial atresia.3 At prenatal ultrasound, differentiation
of the liver and bowel loops from the lung is sometimes difﬁcult;
however, at MR imaging, the liver, small intestine, and colon are
differentiated from the lung by their characteristic signal intensities,
and, therefore, it can differentiate CDH from other chest masses. Also,
since the presence of liver herniation and the size of the hernia are
associated with the degree of pulmonary hypoplasia, MR ﬁndings are
important for counseling parents, selecting fetal surgical candidates,
and estimating prognosis.4
Color Doppler can help to
deﬁne which organs have herniated in fetuses with diaphragmatic
hernia—it can depict an abnormal course of the right renal artery,
arising from the aorta and feeding the intrathoracic right kidney or can
delineate portal or hepatic veins within the herniated liver.
Right-sided CDHs seem to cause less severe symptoms than those on the left.5 They
may manifest beyond the neonatal period with respiratory or
gastrointestinal symptoms or may be detected in adulthood incidentally.
Rarer presentations of right-sided CDH include superior vena cava
syndrome and congenital bronchobiliary ﬁstula.
Duplication of the
IVC is a rare anomaly of the infrarenal segment (due to persistence of
the supracardinal veins), with an incidence of 0.2% to 3%. It is known
to be associated with various urogenital tract anomalies,6 as
seen in this case in which the child had a left pelviureteric junction
obstruction associated with duplicated IVC. This rare association has
been reported in the literature.7 It is postulated that the
subcardinal and supracardinal system form extensive anastomotic channels
at the level of the renal vein and that the persistence of one of these
midline anastomoses could result in pelviureteric junction obstruction;
however, the occurrence of these anomalies in a case of CDH has not
been reported before.
Congenital posterolateral diaphragmatic hernia is widely regarded as an
isolated defect, but this view is incomplete. Defects in morphogenesis
are widespread and heterogeneous. Most often, the abnormalities involve
the heart, brain, genitourinary system, craniofacial region, or limbs.
Patients with CDH should be evaluated carefully for additional defects,
as their presence has a signiﬁcant impact on management and prognosis.
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RN, Tugran C, Yorulmaz I, et al. Bilateral large Bochdalek hernias in
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N, Venkataramu NK, Singh P, Suri S. Duplication of IVC and associated
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