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Pediatric Pulmonology 31:306±310 (2001)

Late Presentation of Bochdalek Hernia:


Clinical and Radiological Aspects
Haluk O _
È ztuÈrk, MD,* Ibrahim Karnak, MD, Mehmet Turgay Sakarya, MD,
and Salih C
Ë etinkurs,un, MD
Summary. Three infants with late presentation of Bochdalek hernia are presented. The
presenting symptoms were cough, intermittent vomiting, dyspnea, and cyanosis. Initial diagnoses
of isolated paravertebral mass and foreign material aspiration were made in two infants, based on
plain chest x-ray ®ndings and history of the patients. Further radiological investigations, such as
contrast upper gastrointestinal series or enema, computerized tomography, and magnetic
resonance imaging of the chest, suggested the diagnosis of Bochdalek hernia. The hernia was
found on the left side in two patients and on the right side in one. At operation, the stomach, small
intestine, and spleen were found as herniated organs in one patient, ascending colon in one, and
all of the small intestine together with ascending colon in the other.
A congenital diaphragmatic defect should be suspected in every child presenting with unusual
respiratory or gastrointestinal symptoms and with abnormal chest x-ray ®ndings. The radiological
®ndings vary greatly from one case to another, and even in the same case at different times
because of differences in herniated organs and intermittent spontaneous reduction. The
possibility of congenital diaphragmatic hernia should be kept in mind to avoid a wrong diagnosis,
undue delay in diagnosis, and inappropriate treatment. Pediatr Pulmonol. 2001; 31:306±310.
ß 2001 Wiley-Liss, Inc.

Key words: congenital diaphragmatic hernia; Bochdalek hernia; late presentation;


diagnosis; children.

INTRODUCTION examination revealed decreased breath sounds over the


left lower chest wall.
Although the usual form of presentation of congenital
Laboratory investigations, including complete blood
diaphragmatic hernia (CDH) is severe neonatal asphyxia,
count (CBC), peripheral blood smear, urinalysis, and
late presentation has also been reported.1±3 It has been
blood biochemistry, were within normal limits. Plain
proposed that herniation is often postnatal in such cases,
chest x-ray revealed a well circumscribed paravertebral
and consequently these patients do not have hypoplastic
opacity, possibly behind the heart, in the left lower hemi-
lungs.1 These patients usually present with respiratory
thorax (Fig. 1). Computerized tomography (CT) of the
and gastrointestinal symptoms, and emergency operation
chest without oral contrast material showed a round
is rarely required.
paravertebral mass of 3  4 cm in dimension in the
We report on 3 infants with late presentation of CDH, 2
posterior inferior part of the left hemithorax. An air-¯uid
of which were initially misdiagnosed as paravertebral
level was also seen in front of the mass. An upper gas-
mass and foreign material aspiration. Discussion of the
trointestinal (GI) series revealed herniation of the fundus
clinical and radiological features of this entity follows.
of the stomach (Fig. 2). No intestinal segments were
observed above the diaphragm in the remaining GI series.
Abdominal ultrasonography (USG) did not reveal any
CASE REPORTS
abnormality. Magnetic resonance imaging (MRI) of the
Case 1 thorax with oral contrast material showed elevation of the
A 9-month-old girl was admitted with the complaints
of cough and vomiting for 3 weeks. The cough was Department of Pediatric Surgery, GuÈlhane Military Medical Academy,
nonproductive. The vomiting was nonbilious and was Ankara, Turkey.
usually observed after feeding. Past medical history did
*Correspondence to: Haluk OÈ ztuÈrk, M.D., Department of Pediatric Surgery,
not reveal any previous respiratory tract infections or
GuÈlhane Military Medical Academy, 06018 Etlik, Ankara, Turkey.
trauma. Her height and weight were at the 60th percentile E-mail: hozturk@obs.gata.edu.tr
for age. Vital signs, including axillary temperature, pulse,
and blood pressure, were within normal limits. Physical Received 2 December 1998; Accepted 7 January 2000.
ß 2001 Wiley-Liss, Inc.
Bochdalek Hernia 307

Fig. 1. Plain chest x-ray of a diaphragmatic hernia shows a well-


circumscribed paravertebral density (Case 1).

posterior part of the left diaphragm. Both the stomach and


the spleen were elevated into the left hemithorax (Fig. 3).
The results of imaging studies were considered to be
highly suggestive for a left diaphragmatic hernia.
The patient was operated on through a left subcostal
laparotomy incision. The fundus of the stomach, small
intestinal segments, and the spleen were found to be
herniated into the thorax through a left posterolateral
diaphragmatic defect of 3  4 cm in size. The hernia was
easily reduced into the abdominal cavity. There was a thin
hernia sac, and the defect was repaired by using 2.0 silk
Fig. 2. Upper gastrointestinal series shows partial herniation of
sutures. The postoperative course was uneventful, and the the stomach through a diaphragmatic defect and a round
patient was discharged on postoperative day 10. The paravertebral mass (Case 1).
patient has been free of signs and symptoms during a
6-month follow-up period.
Case 2
A 45-day-old girl was admitted with the complaint of
dyspnea of 1 day's duration. Past medical history did not
reveal any previous respiratory problems or trauma. Her
weight and height were at the 50th percentile for age. She
had an axillary temperature of 36.8 C; pulse of 120/min;
respiratory rate of 50/min; and blood pressure of 90/50
mmHg. Physical examination revealed decreased respira-
tory sounds over the left hemithorax; cardiac sounds were
displaced to the right hemithorax.
Laboratory investigations including CBC, urinalysis,
and blood biochemistry were within normal limits. Plain

ABBREVIATIONS
CBC Complete blood count
CDH Congenital diaphragmatic hernia
CT Computerized tomography
GI Gastrointestinal
Fig. 3. Magnetic resonance imaging demonstrates a left dia-
MRI Magnetic resonance imaging
phragmatic hernia through which the stomach and spleen have
USG Ultrasonography
herniated (Case 1).
308 È ztuÈrk et al.
O

Fig. 4. Contrast enema shows colonic segments in the left Fig. 5. Plain chest x-ray reveals air-®lled bowel segments in
hemithorax, in a patient with a diaphragmatic hernia (Case 2). the right hemithorax, in a patient with a diaphragmatic hernia
(Case 3).

chest x-ray showed air-®lled bowel segments in the left material aspiration. However, bronchoscopy and bron-
hemithorax. Contrast enema with barium revealed colonic chial lavage did not reveal any foreign material or food
segments extending into the left hemithorax (Fig. 4). particles in the airways. The symptoms persisted after
These ®ndings were indicative of left diaphragmatic bronchoscopy, and a chest x-ray raised the possibility of a
hernia. diaphragmatic hernia. The patient was transferred to the
The patient underwent laparotomy through a left upper pediatric surgery unit.
transverse incision. Ascending colon was found to be The infant was dyspneic and cyanotic on admission.
herniating into the left hemithorax, and it was reduced Pulse rate was 160/min, and respiratory rate was 52/min.
easily into the abdomen. The diaphragmatic defect was in There was perioral cyanosis. Auscultation of the chest
a posterolateral location and 3.5  4 cm in size. No hernia revealed decreased breath sounds on the right hemi-
sac was present, and the defect was repaired by using 2.0 thorax, and occasional bowel sounds. Laboratory ®ndings
silk sutures. An appendectomy was also performed. The including CBC, urinalysis, and blood chemistry were
postoperative course was uneventful, and the patient was within normal limits. Plain chest x-ray revealed air-®lled
discharged on postoperative day 6. bowel segments in the right hemithorax (Fig. 5). The
patient underwent emergency laparotomy through a right
subcostal incision. All of the small intestine and the
Case 3
ascending colon were found herniating into the right
An 8-month-old boy was admitted to our Pediatrics hemithorax. Following reduction, the diaphragmatic
Unit because of recent complaints of dyspnea and defect was found located posterolaterally and 4  5 cm
cyanosis following oral feeding. The infant was admitted in size. It was repaired by using interrupted O silk sutures.
to the pediatric intensive care unit and underwent Postoperative course was uneventful until postoperative
bronchoscopy because of an initial diagnosis of foreign day 6. The patient suffered from marked abdominal
Bochdalek Hernia 309

distention and bilious vomiting. Abdominal x-ray sounds or bowel sounds on the involved side of the
revealed complete intestinal obstruction with multiple thorax. Rarely, these patients may present with acute
air ¯uid levels and no gas shadow in the pelvis. The infant picture such as intestinal obstruction,3 with or without
underwent another laparotomy; an ileoileal intussuscep- gangrene or perforation of the obstructed bowel or torsion
tion was found 50 cm proximal to the ileocecal valve. The of the spleen.5±7
intussuscepted segment was viable and reduced easily by The contents of the hernias vary considerably. The
manual manipulation. No lead point was encountered. stomach, colon, small intestine, spleen, liver, kidney,
The second postoperative period was uneventful, and the omentum, and tail of the pancreas may herniate through
patient was discharged on postoperative day 7. the diaphragmatic defect. Stomach and colon are the
organs that most frequently herniate into the thorax.
Roentgenographic evaluation is extremely helpful in
DISCUSSION
evaluating these patients. Chest x-ray is frequently abnor-
Congenital diaphragmatic hernia occurs in approxi- mal but not always diagnostic. In typical cases, the chest
mately 1/5,000 live births. The most common defect is ®lm shows gas-®lled bowel segments on the affected side
the posterolateral Bochdalek type. The defect is left-sided and sometimes a gas-¯uid level above the diaphragm.
in about 90% of cases, and only 20% of cases have a The plain chest ®lm may mimic lower lobe pneumonia,
hernial sac.4 The symptoms and age at presentation are diaphragmatic eventration, pneumothorax, pleural effu-
very varied. The usual forms of presentation of CDH are sion, and diaphragmatic mass, and may lead to mis-
severe neonatal asphyxia and pulmonary hypertension. diagnosis.8,9 In suspected cases, plain chest x-ray after
The presence and severity of pulmonary hypoplasia are insertion of a nasogastric tube may help in making the
the ultimate prognostic determinant of these patients. On diagnosis.
this basis, three types of CDH have been described.1 The normal spleen is a partially mobile organ, lying
Infants with Type 1 are born with severe bilateral pul- free within the peritoneal cavity except for its attach-
monary hypoplasia which may prove to be lethal in the ments to the stomach by the gastrosplenic ligament, to the
early neonatal period. Type 2 is characterized by uni- colon by the splenocolic ligament, and to the kidney by
lateral (ipsilateral) hypoplasia. In Type 3 CDH, pulmon- the lienorenal ligament. If the stomach moves up into the
ary hypoplasia is insigni®cant, and survival with normal thorax, through a large diaphragmatic hernia, it is con-
pulmonary function is the rule. ceivable that the spleen, attached to the greater curvature
Five to 30% of CDH may appear well during the of the stomach by the gastrosplenic ligament, might
newborn period, but manifest the abnormality in later life. accompany the stomach into the intrathoracic space. The
This group of infants constitutes a fourth group. This last herniated spleen may cause hypertension8 and undergo
group is refered to as having acquired diaphragmatic intraabdominal or intrathoracic torsion, resulting in an
hernia, and is de®ned as having delayed or late appe- emergency situation.5±7
arance of a diaphragmatic hernia after a documented time Case 1 presented as a paravertebral mass on chest
period of postnatal life with no evidence of herniation.1 x-ray. To the best of our knowledge, the appearance of
Despite its high incidence rates, CDH which presents late herniated spleen as a paravertebral mass on chest radio-
is not often recognized.2,3 Our patients did not present any graph has not been mentioned previously. Hence, splenic
respiratory or gastrointestinal symptoms in the neonatal herniation through a diaphragmatic hernia should be
period. considered in the differential diagnosis of paravertebral
Two clinical groups with CDH can be identi®ed after masses; the exact location of the spleen can be detected
the neonatal period: the younger children with mainly by radionucleid scanning.7,10
respiratory symptoms, and the older children with gastro- Contrast roentgenograms of the upper gastrointestinal
intestinal complaints. The common respiratory symptoms tract, barium enemas, or both are necessary to con®rm
are dyspnea, wheezing, repeated respiratory infections, the diagnosis of CDH. Demonstration of any part of the
and rarely cough. The children whose initial symptoms gastrointestinal tract in the thoracic cavity supports the
are gastrointestinal in origin present with abdominal pain diagnosis. Upper GI series showed only partial herniation
and vomiting. Of course none of these symptoms is sug- of the stomach in case 1, but we could not see any part of
gestive of CDH, and CDH is not considered in the light of the bowel above the diaphragm. It has been reported that
these signs at initial evaluation. However, these children herniated abdominal contents can reduce spontaneously
usually give a history of intermittent vomiting.2 Vomiting through the defect, and that the presence of a normal
was one of the presenting symptoms in our ®rst case. It chest radiograph and even normal contrast roentgen-
was intermittent and progressively increased in frequency ograms do not rule out the diagnosis.11 Another
during the last 3-week period before diagnosis. Dyspnea interesting point was that abdominal ultrasonogram
was the main symptom in the remaining two infants. (USG) revealed no abnormality in case 1. This could
Physical examination may reveal diminished respiratory only have occurred if the hernia contents spontaneously
310 È ztuÈrk et al.
O

reduced into the abdominal cavity. Therefore, an radiological ®ndings vary greatly from case to case, and
abdominal USG may not reveal any abnormality in even in the same case at different times, because of the
diaphragmatic hernia. Contrast enema con®rmed the different herniated organs and intermittent spontaneous
diagnosis in case 2. reduction. The possibility of CDH should be kept in mind
In case 1, a chest CT gave us an important clue that to avoid misdiagnosis, undue delay in treatment, and
there was also an air-¯uid level accompanying the mass. inappropriate treatment.
In fact, we could have demonstrated that this appearance
was due to a herniated stomach if we had used oral REFERENCES
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