Study Questions for Chapter 21
1.  A congenital diaphragmatic hernia may re-
sult from failure of the
(A) septum transversum to develop
(B) pleuroperitoneal membranes to fuse in a
normal fashion
(C) pleuropericardial membrane to develop
completely
(D) dorsal mesentery of the esophagus to
develop
(E) body wall to form the peripheral part of the
diaphragm
2.  A congenital diaphragmatic hernia most
commonly occurs
(A) on the right anteromedial side
(B) on the right posterolateral side
(C) on the left anteromedial side
(D) on the left posterolateral side
(E) anywhere on the left side
3.  A congenital diaphragmatic hernia is usually
life threatening because it is associated with
(A) pulmonary hypoplasia
(B) pulmonary hyperplasia
(C) physiological umbilical hernia
(D) liver hypoplasia
(E) liver agenesis
4.  An 8-day-old boy presents with a history of
complete loss of breath at times and of turn-
ing blue on a number of occasions. If the baby
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is placed in an upright or sitting position, his
breathing improves. Physical examination re-
veals an unusually flat stomach when the new-
born is lying down; auscultation demonstrates
no breath sounds on the left side of the thorax.
What is the diagnosis?
(A) Physiological umbilical herniation
(B) Esophageal hiatal hernia
(C) Tetralogy of Fallot
(D) Congenital diaphragmatic hernia
(E) Tricuspid atresia
5.  During week 4, the developing diaphragm is
located at
(A) C3, C4, C5
(B) T3, T4, T5
(C) T8, T9, T10
(D) L1, L2, L3
(E) L4, L5, L6
6.  An apparently healthy newborn with a hardy
appetite has begun feedings with formula.
When she is laid down in the crib after feeding,
she experiences projectile vomiting. Which of
the following conditions is a probable cause of
this vomiting?
(A) Physiological umbilical herniation
(B) Esophageal hiatal hernia
(C) Tetralogy of Fallot
(D) Congenital diaphragmatic hernia
(E) Tracheoesophageal fistula
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 Answers and Explanations

1. B.  The formation of the diaphragm occurs through the fusion of tissue from four different
sources. The pleuroperitoneal membranes normally fuse with the three other components
during week 6 of development. Abnormal development or fusion of one or both of the
pleuroperitoneal membranes causes a patent opening between the thorax and abdomen
through which abdominal viscera may herniate.
2. D.  Congenital diaphragmatic hernias occur most commonly on the left posterolateral side.
The pleuroperitoneal membrane on the right side closes before the left for reasons that are
not clear. Consequently, the patency on the left side remains unclosed for a longer time. The
portion of the diaphragm formed by the pleuroperitoneal membrane in the newborn is located
posterolateral.
3. A.  The herniation of abdominal contents into the pleural cavity compresses the developing
lung bud, resulting in pulmonary hypoplasia. Lung development on the ipsilateral (left) side
of the herniation is most commonly affected, but lung development on the contralateral
(right) side can also be compromised. The lungs may achieve normal size and function after
surgical reduction of the hernia and repair of the diaphragm. However, mortality is high due to
pulmonary hypoplasia.
4. D.  Loss of breath and cyanosis result from pulmonary hypoplasia associated with congenital
diaphragmatic hernia. Placing the baby in an upright position will reduce the hernia somewhat
and ease the pressure on the lungs, thereby increasing the baby’s comfort. The baby’s stomach
is flat (instead of the plump belly of a normal newborn) because the abdominal viscera have
herniated into the thorax. Auscultation reveals no breath sounds on the left side because of
pulmonary hypoplasia.
5. A.  Although it may seem unusual, the adult diaphragm has its embryological beginning at the
cervical level (C3, C4, C5). Nerve roots from C3, C4, and C5 enter the developing diaphragm,
bringing both motor and sensory innervation. With the subsequent rapid growth of the neural
tube, there is an apparent descent of the diaphragm to its adult levels (thoracic and lumbar).
However, the diaphragm retains its innervation from C3, C4, and C5, which explains the
unusually long phrenic nerves.
6. B.  An esophageal hiatal hernia is a herniation of the stomach through the esophageal hiatus into
the pleural cavity. This compromises the esophagogastric sphincter so that stomach contents can
easily reflux into the esophagus. The combination of a full stomach after feeding and lying down
in the crib will cause vomiting in this newborn.

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