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C H A P T E R

8
Body Cavities, Mesenteries,
and Diaphragm
Embryonic Body Cavity 141 Muscular Ingrowth from Lateral
Mesenteries 144 Body Walls 148
Division of Embryonic Body Cavity 144 Positional Changes and Innervation
Development of Diaphragm 146 of Diaphragm 148
Septum Transversum 147 Summary of Development of Body Cavities,
Pleuroperitoneal Membranes 147 Mesenteries, and Diaphragm 151
Dorsal Mesentery of Esophagus 147 Clinically Oriented Problems 153

E arly in the fourth week of development, the intraembryonic coelom appears as a


horseshoe-shaped cavity (Fig. 8-1A). The bend in the cavity at the cranial end of the embryo
represents the future pericardial cavity, and its limbs (lateral extensions) indicate the future
pleural and peritoneal cavities. The distal part of each limb of the intraembryonic coelom
is continuous with the extraembryonic coelom at the lateral edges of the embryonic disc (see
Fig. 8-1B). The intraembryonic coelom provides room for the organs to develop and move.
For instance, it allows the normal herniation of the midgut into the umbilical cord (Fig.
8-2E; see Chapter 11, Fig. 11-14). During embryonic folding in the horizontal plane, the
limbs of the coelom are brought together on the ventral aspect of the embryo (see Fig. 8-2C).
The ventral mesentery degenerates in the region of the future peritoneal cavity (see Fig.
8-2F), resulting in a large embryonic peritoneal cavity extending from the heart to the pelvic
region.

EMBRYONIC BODY CAVITY


The intraembryonic coelom becomes the embryonic body cavity, which is divided into three 6
well-defined cavities during the fourth week (Fig. 8-3; see Figs. 8-1A and 8-2):

● A pericardial cavity
● Two pericardioperitoneal canals
● A peritoneal cavity
141
Neural folds

Future pericardial cavity


Amnion (cut edge) Neural tube Somatic mesoderm layer

Amniotic
Intraembryonic coelom cavity
Future pleural cavity

Extraembryonic coelom

Future peritoneal cavity

Level of section B Wall of umbilical


Umbilical vesicle Somite vesicle

Notochord Splanchnic mesoderm layer


A B
F I G U R E 8 – 1 A, Drawing of a dorsal view of a 22-day embryo shows the outline of the horseshoe-shaped intraembryonic coelom.
The amnion has been removed, and the coelom is shown as if the embryo were translucent. The continuity of the coelom and the
communication of its right and left limbs with the extraembryonic coelom are indicated by arrows. B, Transverse section through the
embryo at the level shown in A.

Heart prominence
Amnion Amnion Midgut
Amnion
old Aorta
f
Head

Ta

La
il fold

tera ld
Heart Intraembryonic

l fo
coelom

Connecting
Pericardial
Level of section C stalk
cavity
Extraembryonic coelom
A B C Umbilical
vesicle
Umbilical
vesicle

Midgut Neural tube Peritoneal


cavity
Foregut Hindgut Dorsal
mesentery

Septum
transversum
Splanchnic
mesoderm
layer

Level of section F Somatic


Umbilical cord
mesoderm
layer Ventral
D E F body wall
Ventral mesentery
disappearing
F I G U R E 8 – 2 Illustrations of embryonic folding and its effects on the intraembryonic coelom and other structures. A, Lateral
view of an embryo (approximately 26 days). B, Schematic sagittal section of the same embryo shows the head and tail folds. C, Trans-
verse section at the level shown in A indicates how fusion of the lateral folds gives the embryo a cylindrical form. D, Lateral view of
an embryo (at approximately 28 days). E, Schematic sagittal section of the same embryo shows the reduced communication between
the intraembryonic and extraembryonic coeloms (double-headed arrow). F, Transverse section at the level shown in D illustrates forma-
tion of the ventral body wall and disappearance of the ventral mesentery. The arrows indicate the junction of the somatic and splanchnic
layers of mesoderm. The somatic mesoderm will form the parietal peritoneum lining the abdominal wall, and the splanchnic mesoderm
will form the visceral peritoneum covering the organs (e.g., the stomach).
CHAPTER 8 | B o d y Cav i t i e s , M e s en ter i es , an d D i aphrag m 143

Notochord Neural tube


Dorsal aorta

Pericardioperitoneal canal
Esophageal part of
tracheoesophageal
Tracheal part of tube
tracheoesophageal tube
Dorsal mesocardium

B Heart

Pericardial cavity

Foregut artery (future celiac


arterial trunk)

Stomach

Ventral mesentery

Liver
Peritoneal cavity

C Ventral abdominal wall

Peritoneal cavity

Dorsal mesentery

Midgut

D Umbilical vesicle

Neural tube
A
Dorsal aorta
Peritoneal cavity

Splanchnic
Dorsal mesentery
mesoderm

Hindgut
Somatic
mesoderm

E
F I G U R E 8 – 3 Illustrations of the mesenteries and body cavities at the beginning of the fifth week of development. A, Schematic
sagittal section. Notice that the dorsal mesentery serves as a pathway for the arteries supplying the developing midgut. Nerves and
lymphatics also pass between the layers of this mesentery. B to E, Transverse sections through the embryo at the levels indicated in
A. The ventral mesentery disappears, except in the region of the terminal esophagus, stomach, and first part of the duodenum. Notice
that the right and left parts of the peritoneal cavity separate in C but are continuous in E.
144 THE DEVEL O P I N G H U M A N

These cavities have a parietal wall, lined by mesothe- Mesenteries


lium (future parietal layer of peritoneum), that is derived A mesentery is a double layer of peritoneum that begins
from somatic mesoderm and a visceral wall, also covered as an extension of the visceral peritoneum covering an
by mesothelium (future visceral layer of peritoneum), that organ. The mesentery connects the organ to the body wall
is derived from splanchnic mesoderm (see Fig. 8-3E). The and conveys vessels and nerves to it. Transiently, the
peritoneal cavity is connected with the extraembryonic dorsal and ventral mesenteries divide the peritoneal cavity
coelom at the umbilicus (Fig. 8-4A and D). The cavity into right and left halves (see Fig. 8-3C). The ventral
loses its connection with the extraembryonic coelom mesentery soon disappears (see Fig. 8-3E), except where
during the 11th week of gestation as the intestines return it is attached to the caudal part of the foregut (primor-
to the abdomen from the umbilical cord (see Chapter 11, dium of stomach and proximal part of duodenum). The
Fig. 11-13C). peritoneal cavity then becomes a continuous space (see
During formation of the head fold, the heart and peri- Fig. 8-4D). The arteries supplying the primordial gut—
cardial cavity are relocated ventrally, anterior to the celiac arterial trunk (foregut), superior mesenteric artery
foregut (see Fig. 8-2B). As a result, the pericardial cavity (midgut), and inferior mesenteric artery (hindgut)—pass
opens into pericardioperitoneal canals, which pass dorsal between the layers of the dorsal mesentery (see Fig. 8-3C).
to the foregut (see Fig. 8-4B and D). After embryonic
folding, the caudal part of the foregut, midgut, and
hindgut are suspended in the peritoneal cavity from the Division of Embryonic Body Cavity
dorsal abdominal wall by the dorsal mesentery (see Figs. Each pericardioperitoneal canal lies lateral to the proxi-
8-2F and 8-3B, D, and E). mal part of the foregut (future esophagus) and dorsal to

Level of section B Neural tube


Amnion (cut)
Heart Pericardioperitoneal canal

Peritoneal cavity
Foregut

Septum transversum
(primordium of central
tendon of diaphragm)

Allantois
Pericardial cavity
Omphaloenteric duct
(yolk stalk)
A B

Common cardinal vein Neural tube Pericardioperitoneal canal Peritoneal cavity

Foregut

Heart

Pericardial cavity

Septum transversum
Pericardial cavity
Communication of intraembryonic
coelom with extraembryonic coelom
C D
F I G U R E 8 – 4 Schematic drawings of an embryo (at approximately 24 days). A, The lateral wall of the pericardial cavity has been
removed to show the primordial heart. B, Transverse section of the embryo illustrates the relationship of the pericardioperitoneal canals
to the septum transversum (primordium of central tendon of diaphragm) and the foregut. C, Lateral view of the embryo with heart
removed. The embryo has also been sectioned transversely to show the continuity of the intraembryonic and extraembryonic coeloms
(arrow). D, Sketch shows the pericardioperitoneal canals arising from the dorsal wall of the pericardial cavity and passing on each side
of the foregut to join the peritoneal cavity. The arrow shows the communication of the extraembryonic coelom with the intraembryonic
coelom and the continuity of the intraembryonic coelom at this stage.
CHAPTER 8 | B o d y Cav i t i e s , M e s en ter i es , an d D i aphrag m 145

Notochord

Aorta
Bronchial bud Pericardioperitoneal canal

Pleural cavity Lung

Lateral thoracic wall


Phrenic nerve Left common cardinal vein

Pleuropericardial fold Pleuropericardial


Heart membrane
A Pericardial cavity B

Aorta Aorta

Mesoesophagus

Pleural cavity
Esophagus in Thoracic wall
primordial mediastinum

Inferior vena cava


Fibrous
Phrenic nerve
pericardium
C D
Pericardial cavity Pericardial cavity
F I G U R E 8 – 5 Drawings of transverse sections through embryos cranial to the septum transversum illustrate successive stages in
the separation of the pleural cavities from the pericardial cavity. Growth and development of the lungs, expansion of the pleural cavi-
ties, and formation of fibrous pericardium are also shown. A, At 5 weeks. The arrows indicate the communications between the peri-
cardioperitoneal canals and the pericardial cavity. B, At 6 weeks. The arrows indicate development of the pleural cavities as they expand
into the body wall. C, At 7 weeks. Expansion of the pleural cavities ventrally around the heart is shown. The pleuropericardial mem-
branes are now fused in the median plane and with the mesoderm ventral to the esophagus. D, At 8 weeks. Continued expansion of
the lungs and pleural cavities and formation of the fibrous pericardium and thoracic wall are illustrated.

the septum transversum—a plate of mesodermal tissue


that occupies the space between the thoracic cavity and CONGENITAL PERICARDIAL DEFECT
the omphaloenteric duct (see Fig. 8-4A and B).
The septum transversum is the primordium of the Defective formation and/or fusion of the pleuropericar-
central tendon of the diaphragm. Partitions form in each dial membranes separating the pericardial and pleural
pericardioperitoneal canal separating the pericardial cavities is uncommon. This anomaly results in a congeni-
cavity from the pleural cavities and the pleural cavities tal defect of the pericardium, usually asymptomatic and
from the peritoneal cavity. Because of the growth of the on the left side. Consequently, the pericardial cavity com-
bronchial buds (primordia of bronchi and lungs) into the municates with the pleural cavity. In very unusual cases,
pericardioperitoneal canals, a pair of membranous ridges a part of the left atrium of the heart herniates into the
is produced in the lateral wall of each canal (Fig. 8-5A pleural cavity at each heartbeat.
and B):
● The cranial ridges—pleuropericardial folds—are
located superior to the developing lungs.
● The caudal ridges—pleuroperitoneal folds—are located
pleural cavities. These partitions—the pleuropericardial
inferior to the lungs.
membranes—contain the common cardinal veins (see
Figs. 8-4C and 8-5A), which drain the venous system into
Pleuropericardial Membranes the sinus venosus of the heart. Initially, the bronchial
As the pleuropericardial folds enlarge, they form parti- buds are small relative to the heart and pericardial cavity
tions that separate the pericardial cavity from the (see Fig. 8-5A). They soon grow laterally from the caudal
146 THE DEVEL O P I N G H U M A N

end of the trachea into the pericardioperitoneal canals Level of


(future pleural canals). As the primordial pleural cavities Lung section C
Heart Pleural
expand ventrally around the heart, they extend into the cavity
body wall, splitting the mesenchyme into Pleuroperitoneal
membrane
● An outer layer that becomes the thoracic wall
● An inner layer that becomes the fibrous pericardium, Stomach
the outer layer of the pericardial sac enclosing the
heart (see Fig. 8-5C and D)
The pleuropericardial membranes project into the cranial
ends of the pericardioperitoneal canals (see Fig. 8-5B).
With subsequent growth of the common cardinal veins,
positional displacement of the heart, and expansion of
the pleural cavities, the membranes become mesentery-
like folds extending from the lateral thoracic wall. By the
seventh week, the membranes fuse with the mesenchyme
Peritoneal
ventral to the esophagus, separating the pericardial cavity cavity
Liver
from the pleural cavities (see Fig. 8-5C). This primordial
Pericardial Septum
mediastinum consists of a mass of mesenchyme that cavity transversum
extends from the sternum to the vertebral column, sepa-
rating the developing lungs (see Fig. 8-5D). The right A
pleuropericardial opening closes slightly earlier than
the left one and produces a larger pleuropericardial
membrane.
Pleuroperitoneal Membranes
As the pleuroperitoneal folds enlarge, they project into
the pericardioperitoneal canals. Gradually the folds
become membranous, forming the pleuroperitoneal mem-
branes (Fig. 8-6 and Fig. 8-7). Eventually, these mem-
branes separate the pleural cavities from the peritoneal
cavity. The pleuroperitoneal membranes are produced as
H
the developing lungs and pleural cavities expand and L
invade the body wall. They are attached dorsolaterally to
the abdominal wall, and initially their crescentic free
edges project into the caudal ends of the pericardioperi-
toneal canals.
0.5 mm
During the sixth week of gestation, the pleuroperito-
neal membranes extend ventromedially until their free
edges fuse with the dorsal mesentery of the esophagus B
Spinal cord
and septum transversum (see Fig. 8-7C). This separates
the pleural cavities from the peritoneal cavity. Closure of Mesoesophagus
Spinal ganglion
the pleuroperitoneal openings is completed by the migra-
tion of myoblasts (primordial muscle cells) into the Pleural cavity
Aorta
pleuroperitoneal membranes (see Fig. 8-7E). The pleuro-
peritoneal opening on the right side closes slightly before Lung Pleuroperitoneal opening
the left one. The reason for this is uncertain, but it may
be related to the relatively large size of the right lobe of
the liver at this stage of development. Liver

Septum transversum
DEVELOPMENT OF DIAPHRAGM
Heart
6 The diaphragm is a dome-shaped, musculotendinous par-
tition that separates the thoracic and abdominal cavities. C Pericardial cavity
It is a composite structure that develops from four embry-
F I G U R E 8 – 6 A, The primordial body cavities are viewed
onic components (see Fig. 8-7):
from the left side after removal of the lateral body wall. B, Pho-
tograph of a 5-week-old embryo shows the developing septum
● Septum transversum
transversum (arrow), heart tube (H), and liver (L). C, Transverse
● Pleuroperitoneal membranes
section through an embryo at the level shown in A.
● Dorsal mesentery of esophagus
● Muscular ingrowth from lateral body walls
CHAPTER 8 | B o d y Cav i t i e s , M e s en ter i es , an d D i aphrag m 146.e1

(B, Courtesy Dr. Bradley R. Smith, University of Michigan, Ann


Arbor, MI.)
CHAPTER 8 | B o d y Cav i t i e s , M e s en ter i es , an d D i aphrag m 147

Esophageal mesentery Central tendon


Esophagus

A
Inferior
vena cava

Pericardioperitoneal
canal
Pleuroperitoneal
Pleuroperitoneal fold membrane

B Aorta C D
Muscular ingrowth
from body wall

Septum transversum Central tendon

Inferior vena cava


Mesentery of esophagus
Esophagus

Pleuroperitoneal folds and membranes


Aorta

Muscular ingrowth from body wall E Crura of the diaphragm

F I G U R E 8 – 7 Development of the diaphragm. A, Lateral view of an embryo at the end of the fifth week (actual size) indicates
the level of sections B to D. B, Transverse section shows the unfused pleuroperitoneal membranes. C, Similar section at the end of
the sixth week after fusion of the pleuroperitoneal membranes with the other two diaphragmatic components. D, Transverse section
of a 12-week fetus after ingrowth of the fourth diaphragmatic component from the body wall. E, Inferior view of the diaphragm of a
neonate indicates the embryologic origin of its components.

Several candidate genes on the long arm of chromo- of the esophagus (see Fig. 8-7B). The septum expands and
some 15 (15q) play a critical role in the development of fuses with the dorsal mesentery of the esophagus and
the diaphragm. pleuroperitoneal membranes (see Fig. 8-7C).

Septum Transversum Pleuroperitoneal Membranes


The transverse septum grows dorsally from the ventro- The pleuroperitoneal membranes fuse with the dorsal
lateral body wall and forms a semicircular shelf that mesentery of the esophagus and the septum transversum
separates the heart from the liver (see Fig. 8-6A). This (see Fig. 8-7C). This completes the partition between the
septum, which is composed of mesodermal tissue, forms thoracic and abdominal cavities and forms the primordial
the central tendon of diaphragm (see Fig. 8-7D and E). diaphragm. Although the pleuroperitoneal membranes
After the head folds ventrally during the fourth week, the form large portions of the early fetal diaphragm, they
septum forms a thick, incomplete connective tissue parti- represent relatively small portions of the neonate’s dia-
tion between the pericardial and abdominal cavities (see phragm (see Fig. 8-7E).
Fig. 8-4). The septum does not completely separate the
thoracic and abdominal cavities.
During early development, a large part of the liver is Dorsal Mesentery of Esophagus
embedded in the septum transversum. There are large The septum transversum and pleuroperitoneal mem-
openings, the pericardioperitoneal canals, along the sides branes fuse with the dorsal mesentery of the esophagus.
148 THE DEVEL O P I N G H U M A N

This mesentery constitutes the median portion of the Lung Pleural cavity Esophagus Pericardial cavity
diaphragm. The crura of the diaphragm, a leg-like pair
of diverging muscle bundles that cross in the median
plane anterior to the aorta (see Fig. 8-7E), develop from
myoblasts that grow into the dorsal mesentery of the
esophagus.

Muscular Ingrowth from


Lateral Body Walls
During the 9th to 12th weeks, the lungs and pleural cavi- Body wall
ties enlarge, burrowing into the lateral body walls (see
Fig. 8-5). During this process, the body-wall tissue is split Diaphragm
Costodiaphragmatic
into two layers: A B
recess of pleura
● An external layer that becomes part of the definitive F I G U R E 8 – 8 A and B, Extensions of the pleural cavities
abdominal wall into the body walls form peripheral parts of the diaphragm and
● An internal layer that contributes to peripheral parts costodiaphragmatic recesses and establish the characteristic
of the diaphragm, external to the parts derived from dome-shaped configuration of the diaphragm. Notice that body-
the pleuroperitoneal membranes (see Fig. 8-7D and E) wall tissue is added peripherally to the diaphragm as the lungs
Further extension of the developing pleural cavities and pleural cavities enlarge.
into the lateral body walls forms the costodiaphragmatic
recesses (Fig. 8-8A and B), establishing the characteristic
dome-shaped configuration of the diaphragm. After
birth, the costodiaphragmatic recesses become alternately sixth week, the diaphragm is at the level of the thoracic
smaller and larger as the lungs move in and out during somites. The phrenic nerves now have a descending
inspiration and expiration. course. As the diaphragm appears relatively farther cau-
dally in the body, the nerves are correspondingly length-
Positional Changes and ened. By the beginning of the eighth week, the dorsal part
of the diaphragm lies at the level of the first lumbar ver-
Innervation of Diaphragm tebra. Because of the cervical origin of the phrenic nerves,
During the fourth week of gestation, the septum trans- they are approximately 30 cm long in adults.
versum, before relocation of the heart, lies opposite the The phrenic nerves in the embryo enter the diaphragm
third to fifth cervical somites. During the fifth week, by passing through the pleuropericardial membranes.
myoblasts from the somites migrate into the developing This explains why the phrenic nerves subsequently lie on
diaphragm, bringing their nerve fibers with them. Conse- the fibrous pericardium, the adult derivative of the pleu-
quently, the phrenic nerves that supply motor innervation ropericardial membranes (see Fig. 8-5C and D).
to the diaphragm arise from the ventral primary rami of As the four parts of the diaphragm fuse (see Fig. 8-7),
the third, fourth, and fifth cervical spinal nerves (see mesenchyme in the septum transversum extends into the
Fig. 8-5A and C). The three twigs on each side join to other three parts. It forms myoblasts that differentiate
form a phrenic nerve. The phrenic nerves also supply into the skeletal muscle of the diaphragm. The costal
sensory fibers to the superior and inferior surfaces of the border receives sensory fibers from the lower intercostal
right and left domes of the diaphragm. nerves because of the origin of the peripheral part of
Rapid growth of the dorsal part of the embryo’s body the diaphragm from the lateral body walls (see Fig. 8-7D
results in an apparent descent of the diaphragm. By the and E).

POSTEROLATERAL DEFECT OF DIAPHRAGM


The only relatively common birth defect of the diaphragm may also be present. CDH is the most common cause of
is a posterolateral defect (Fig. 8-9A and B and Fig. 8-10), pulmonary hypoplasia. The candidate gene region for CDH
which occurs in about 1 in 2200 neonates. It is associated has been reported to be a chromosome 15q26 gene muta-
with congenital diaphragmatic hernia (CDH), which leads tion that includes zinc finger formation (GATA6).
to herniation of abdominal contents into the thoracic CDH, usually unilateral, results from defective formation
cavity. and/or fusion of the pleuroperitoneal membranes with the
Life-threatening breathing difficulties may be associated other three parts of the diaphragm (see Fig. 8-7). This
with CDH because of inhibition of development and infla- results in a large opening in the posterolateral region of the
tion of the lungs (Fig. 8-11). Moreover, fetal lung maturation diaphragm. As a result, the peritoneal and pleural cavities
may be delayed. Polyhydramnios (excess amniotic fluid) are continuous with one another along the posterior body
CHAPTER 8 | B o d y Cav i t i e s , M e s en ter i es , an d D i aphrag m 149

POSTEROLATERAL DEFECT OF DIAPHRAGM—cont’d


wall. This birth defect (sometimes referred to as the foramen herniate into the thorax (i.e., the timing and degree of
of Bochdalek) occurs on the left side in 85% to 90% of compression of the fetal lungs). The effect on the ipsilateral
cases. The preponderance of left-sided defects may be (same-side) lung is greater, but the contralateral lung also
related to the earlier closure of the right pleuroperitoneal shows morphologic changes. If the abdominal viscera are
opening. Prenatal diagnosis of CDH depends on ultrasound in the thoracic cavity at birth, the initiation of respiration is
examination and magnetic resonance imaging of abdomi- likely to be impaired. The intestines dilate, and this com-
nal organs in the thorax. promises the functioning of the heart and lungs. Because
The pleuroperitoneal membranes normally fuse with the the abdominal organs are most often in the left side of the
other three diaphragmatic components by the end of the thorax, the heart and mediastinum are usually displaced to
sixth week of gestation (see Fig. 8-7C). If a pleuroperitoneal the right.
canal is still open when the intestines return to the abdomen The lungs in infants with CDH are often hypoplastic. The
from the physiologic hernia of the umbilical cord in the 10th growth retardation of the lungs results from the lack of
week, some of the intestines and other viscera may pass room for them to develop normally. Further complicating
into the thorax. The presence of abdominal viscera in the the neonatal course is the associated pulmonary hyperten-
thorax pushes the lungs and heart anteriorly and com- sion resulting from decreased vascular cross-sectional area.
presses the lungs. Often, the stomach, spleen, and most of Hypoxia may also trigger pulmonary vasoconstriction, which
the intestines herniate (see Fig. 8-11). Mortality in cases in some cases may be reversible with inhaled nitric oxide,
of CDH results not because there is a defect in the dia- a potent pulmonary vasodilator. The lungs often become
phragm or that abdominal viscera are in the chest but aerated and achieve their normal size after reduction (repo-
because the lungs are hypoplastic due to compression sitioning) of the herniated viscera and repair of the defect
during development. in the diaphragm. Prenatal detection of CDH occurs in
The severity of pulmonary developmental abnormalities about 50% of cases. Most infants with CDH now survive
depends on when and to what extent the abdominal viscera because of improvements in ventilator care.

EVENTRATION OF DIAPHRAGM
In eventration of the diaphragm, an uncommon condition, wall to extend into the pleuroperitoneal membrane on the
half of the diaphragm has defective musculature and bal- affected side.
loons into the thoracic cavity as an aponeurotic (membra- Eventration of the diaphragm is not a true diaphragmatic
nous) sheet, forming a diaphragmatic pouch (see Fig. 8-9C herniation; it is a superior displacement of viscera into a
and D). The abdominal viscera are displaced superiorly into sac-like part of the diaphragm. However, the clinical mani-
the pocket-like outpouching of the diaphragm. This defect festations of diaphragmatic eventration may simulate CDH.
results mainly from failure of muscular tissue from the body

GASTROSCHISIS AND CONGENITAL EPIGASTRIC HERNIA


Gastroschisis is a congenital fissure in the anterior abdomi- defect is usually detected prenatally with routine ultrasound
nal wall that occurs in approximately 1 in 3000 live births. examination.
Usually, there is protrusion of viscera. The site of the Congenital epigastric hernia, on the other hand, is found
abdominal defect is to the right of the umbilical cord rather in the midline as a bulging of the abdominal wall located
than truly in the midline. This defect differs from an umbili- between the xiphoid process and the umbilicus. The bowel
cal hernia (see Chapter 11) in that the bowel is uncovered is not exposed to the amniotic fluid because it remains
and floating in the amniotic fluid. Although it is not a true covered by skin and subcutaneous tissues.
covering, an inflammatory peel may form secondary to Gastroschisis and epigastric hernias result from failure of
exposure of the bowel to amniotic fluid. If this is present, the lateral body folds to fuse completely when the anterior
the bowel at birth appears to be covered in a membrane, abdominal wall forms during the fourth week of gestation
and the individual loops are not easily discernible. This (see Fig. 8-2C and F).
150 THE DEVEL O P I N G H U M A N

Esophagus Posterolateral
Compressed lung defect in diaphragm

Aorta
Intestine in
thorax
Heart

Diaphragm
Liver

Stomach
Pericardial sac
A B

Eventration
Intestine Compressed lung of diaphragm

Compressed
lung

Eventration
of diaphragm

Intestine

Liver

Diaphragm

C D

F I G U R E 8 – 9 A, Diagram shows herniation of the intestine into the thorax through a posterolateral defect in the left side of the
diaphragm. Notice that the left lung is compressed and hypoplastic. B, Drawing of a diaphragm with a large posterolateral defect on
the left side due to abnormal formation and/or abnormal fusion of the pleuroperitoneal membrane on the left side with the meso-
esophagus and septum transversum. C and D, Eventration of the diaphragm resulting from defective muscular development of the
diaphragm. The abdominal viscera are displaced in the thorax within a pouch of diaphragmatic tissue.
CHAPTER 8 | B o d y Cav i t i e s , M e s en ter i es , an d D i aphrag m 151

CONGENITAL HIATAL HERNIA


Herniation of part of the fetal stomach may occur through
an excessively large esophageal hiatus—the opening in
the diaphragm through which the esophagus and the
vagus nerves pass. A hiatal hernia is usually acquired
during adult life; a congenitally enlarged esophageal
hiatus may be the predisposing factor in some cases.

RETROSTERNAL (PARASTERNAL)
L
HERNIA
Herniations may occur through the sternocostal hiatus
(also called foramen of Morgagni)—the opening for the
superior epigastric vessels in the retrosternal area.
However, they are uncommon. This hiatus is located *
between the sternal and costal parts of the diaphragm.
Herniation of intestine into the pericardial sac may occur, *
or, conversely, part of the heart may descend into the
peritoneal cavity in the epigastric region. Large defects
are commonly associated with body-wall defects in the
umbilical region. Radiologists and pathologists often
observe fatty herniations through the sternocostal hiatus; F I G U R E 8 – 1 0 Coronal magnetic resonance image of a
however, they are usually of no clinical significance. fetus with right-sided congenital diaphragmatic hernia. Notice
the liver (L) and loops of small intestine (arrowheads) in the tho-
racic cavity. Ascites is present (asterisks), with accumulation of
serous fluid in the peritoneal cavity and extending into the tho-
racic cavity. Arrows indicate abnormal skin thickening.
ACCESSORY DIAPHRAGM
● As peritoneal parts of the intraembryonic coelom come
More than 30 cases of this rare anomaly known as acces-
together, the splanchnic layer of mesoderm encloses
sory diaphragm have been reported. It is most often on the primordial gut and suspends it from the dorsal
the right side and associated with lung hypoplasia and body wall by a double-layered peritoneal membrane,
other respiratory complications. An accessory diaphragm the dorsal mesentery.
can be diagnosed by magnetic resonance imaging ● The parts of the parietal layer of mesoderm lining the
or computed tomography. It is treated by surgical peritoneal, pleural, and pericardial cavities become the
excision. parietal peritoneum, parietal pleura, and serous peri-
cardium, respectively.
● By the seventh week, the embryonic pericardial cavity
communicates with the peritoneal cavity through
SUMMARY OF DEVELOPMENT OF paired pericardioperitoneal canals. During the fifth
BODY CAVITIES, MESENTERIES, and sixth weeks, folds (later to become membranes)
AND DIAPHRAGM form near the cranial and caudal ends of the canals.
● Fusion of the cranial pleuropericardial membranes
● The intraembryonic coelom begins to develop near the with mesoderm ventral to the esophagus separates the
end of the third week. By the fourth week, it is a pericardial cavity from the pleural cavities. Fusion of
horseshoe-shaped cavity in the cardiogenic and lateral the caudal pleuroperitoneal membranes during forma-
mesoderm. The bend in the cavity represents the future tion of the diaphragm separates the pleural cavities
pericardial cavity, and its lateral extensions represent from the peritoneal cavity.
the future pleural and peritoneal cavities. ● The diaphragm develops from the septum transver-
● During folding of the embryonic disc in the fourth sum, mesentery of the esophagus, pleuroperitoneal
week (see Chapter 5, Fig. 5-1B), lateral parts of the folds and membranes, and muscular outgrowth from
intraembryonic coelom move together on the ventral the body wall.
aspect of the embryo. When the caudal part of the ● The diaphragm divides the body cavity into thoracic
ventral mesentery disappears, the right and left parts and peritoneal cavities.
of the intraembryonic coelom merge to form the peri- ● A birth defect (opening) in the pleuroperitoneal mem-
toneal cavity. brane on the left side becomes a CDH.
CHAPTER 8 | B o d y Cav i t i e s , M e s en ter i es , an d D i aphrag m 151.e1

(Courtesy Deborah Levine, MD, Director of Obstetric and Gyne-


cologic Ultrasound, Beth Israel Deaconess Medical Center,
Boston, MA.)
152 THE DEVEL O P I N G H U M A N

A B

C
F I G U R E 8 – 1 1 Diaphragmatic hernia on the left side of a female fetus (19 to 20 weeks) with herniation of liver (A), stomach,
and bowel (B), underneath the liver into left thoracic cavity. Notice the pulmonary hypoplasia visible after liver removal. C, Diaphrag-
matic hernia (posterolateral defect). Chest radiograph of a neonate shows herniation of intestinal loops (I) into the left side of the
thorax. Notice that the heart (H) is displaced to the right side and that the stomach (S) is on the left side of the upper abdominal cavity.
CHAPTER 8 | B o d y Cav i t i e s , M e s en ter i es , an d D i aphrag m 152.e1

(A and B, Courtesy Dr. D. K. Kalousek, Department of Pathology,


University of British Columbia, Children’s Hospital, Vancouver,
British Columbia, Canada. C, Courtesy Dr. Prem S. Sahni, formerly
of the Department of Radiology, Children’s Hospital, Winnipeg,
Manitoba, Canada.)

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