Pediatr Radiol (1992) 22:187-191

Pediatric Radiology 9 Springer-Verlag 1992

Delayed presentation of congenital diaphragmatichernia R. Fotter t, G. Schimpi 2, E. Sormitin 1, K. Fritz 1, and U. Landler t Department of Radiology, University Hospital Graz, A-8036 Graz, Austria 2Department of Pediatric Surgery, University Hospital Graz, A-8010 Graz, Austria Received: 11 February 1992; accepted: 5 March 1992

Abstract. Eight patients aged i m o n t h up to nine years with congenital diaphragmatic hernias (seven left sided postero-lateral, one right-sided antero-medial), who presented outside of the neonatal period, are reported, four are described in detail. Radiographic presentation was obvious in four patients, simulated inflammatory lung disease in one and p n e u m o t h o r a x in two. In one patient a Morgagni hernia was primarily missed. Two had previous normal chest X-rays. All cases with herniated bowel showed "connecting" bowel segements passing through the diaphragmatic deflect. Primarily the lack of awareness of delayed presentation of congenital diaphragmatic hernia in children with uncharacteristic thoracic and/or abdominal symptoms led to an undesirable time delay between first chest X-ray and surgery up to 16 months in four of our cases. Life threatening complications can be the consequence of delayed diagnosis as in one of our cases.

The severity of pulmonary hypoplasia is the main prognostic factor in newborns with congenital diaphragmatic hernia [1, 2]. In this age group congenital diaphragmatic hernias usually have few diagnostic problems. Congenital diaphragmatic hernias presenting outside of the neonatal period are called congenital diaphragmatic hernias with delayed presentation [3-5]. In contrast, the prognosis in delayed presentation is not determined by pulmonary hypoplasia but by complications as strangulation or incarceration of the herniated bowel or stomach, mediastinal shift and even cardiorespiratory arrest [6-8]. Misinterpretation of the clinical picture and the given radiographic pattern may lead to fatal consequences [8]. Clinical presentation of the late presenting diaphragmatic hernia is different from the newborn form and it is very important to keep in mind that a diaphragmatic hernia in a previous healthy child can be the cause of various abdominal and thoracic symptoms [4, 8]. The clinical presentation can vary from a longstanding and intermittent unspecific course to a life threatening acute event. O u r retrospective study consists of seven patients with left sided postero-lateral diaphragmatic hernia and one

with a right sided antero-medial diaphragmatic hernia, aged one m o n t h up to nine years. Two of our patients had previous "normal" chest X-rays. Four showed obvious radiographic features, one had findings on chest-radiography simulating pneumonic consolidation with pneumatoceles, two cases with a herniated stomach were primarily misinterpreted as p n e u m o t h o r a x and one anteromedial hernia was primarily missed. We report four cases as examples of the variable radiographic and clinical appearance of congenital diaphragmatic hernias with delayed presentation.

Case reports Case 1 A 9-month-old female had been in good health until one day before admission when she developed a cough and had a cyanotic spell. A chest radiograph showed a homogenous opacification in the left lung base with peripheral translucencies and a mediastinal shift to the right. The lower translucencies were "looplike" and in close relation to bowel gas in the left upper abdomen. The contour of the left hemidiaphragm could not be delineated (Fig. 1 a). The primary diagnosis was staphylococcal pneumonia with pneumatoceles although there was only a slight leucocytosis (12,700) and the body temperature was normal. After eight days of antibiotic treatment a repeat chest X-ray showed an increasing size of the opacification and an increase in number of translucencies, which extended up to the upper lung field. In the lower area there was still a "looplike" translucency and discrete airbubbles. With the suspected diagnosis of a late presenting congenital diaphragmatic hernia an upper GI series was performed. That showed a herniation of small and large bowel into the thorax and confirmed, that the lower looplike translucency and airbubbles represented partially airfilled "connecting" bowel segments passing through a a postero-lateral diaphragmatic defect (Fig. 1 b). The patient went to surgery and a congenital postero-lateral diaphragmatic hernia was found, which contained several small bowel loops, omentum, parts of transverse colon and the spleen. The left lung was normal. Postoperatively the patient did well and followup chest X-rays were normal. Delay in diagnosis eight days. Case 2 An 8-month-old boy was healthy until two days before admission to an outside hospital when he developed vomiting and anorexia. The first standard chest X-ray was normal. A second chest X-ray two

188 days later showed a large air-filled structure in the left hemithorax deviating the heart to the right and compressing the left lung in the upper field. This was initially interpreted as pneumothorax and an aspiration of the left thoracic cavity was unsuccessful. A pneumothorax resulted and the patient was transferred to our institution. Nasogastric tube placement and instillation of nonionic contrast confirmed, that the air collection in the left thorax was the stomach (Fig. 2). An immediate operation was performed which showed a left sided postero-lateral diaphragmatic hernia with the stomach in the left thorax. The left lung was normal in appearance. The patient recovered quickly and the posteroperative chest radiograph returned to normal.

Case 3 This 9-year-old boy was admitted for acute onset of abdominal pain, fever and vomiting with a pyrexia of 38.2~ and a leucocytosis of 20,600. There was a history of upper respiratory-tract infections. A chest X-ray in another hospital 16 months before had been interpreted as normal, but in retrospect it was apparent that the left sinus contour was rounded and opacified (Fig.3). With the clinical diagnosis of enteritis and appendicitis an appendectomy was performed. During operation the anesthesiologist noted an absence of breath sounds over the left hermithorax and a decrease in blood-oxygen saturation. An intraoperative chest X-ray showed opacification of the whole left hemithorax with large translucencies and a mediastinal shift (Fig.4). Nasogastric tube placement confirmed that the whole stomach was in the thorax and 2,400 ml of fluid was aspirated. A postero-lateral diaphragmatic hernia with herniation of the whole stomach, the left colonic flexure and the spleen was found at surgery. Since the first misinterpreted chest X-ray there was a delay in diagnosis of 16 months.

Case 4 A 13-months-old boy with a dysmorphia-syndrome with atrial-septal defect, hypospadia grade III and a trigonocephalus was admitted because of cough and vomiting after each meal. The primary chest X-rays four months earlier in another hospital were interpreted as "spastic bronchiolitis". The primary chest X-rays were reevaluated and showed a fight sided antero-medial herniation of parts of the transverse colon (Fig.5). At operation there was a typical anteromedial defect with herniation of transverse colon and liver into the thorax. The time delay was four months. Two of the four remaining patients (one boy with two years and one girl with four years) had respiratory and abdominal symptoms for some days, one boy aged 11 months had anorexia and came to admission in a severe cyanotic state and one one month old boy was admitted in a severe shock state with cyanosis and dyspnea. In three cases the hernial content was small and large bowel, in one case stomach and parts of the colon. In all of these cases a "connecting" bowel segment could be identified and the radiographic presentation was obvious for postero-lateral diaphragmatic hernia. There was no time delay between first chest X-ray and surgery.

Fig./a,b. Case 1. Female, 9 months, a Opacification of left lung base, peripheral translucencies with close relation to bowel gas in left upper abdomen. "Connecting" bowel segment (arrow). Misinterpretation as staphylococcal pneumonia, b Upper GI series 8 days later shows postero-lateral diaphragmatic hernia. Mediastinal shift Fig.2. Case 2. Male, 8 months. Primary misinterpretation as pneumothorax, unsuccessful needle aspiration. Resulting pneumothorax with collapsed left lung. Large air-filled structure with feeding tube and some contrast material respresents stomach. Slight mediastial shift. Posterolateral diaphragmatic hernia with herniated stomach

Discussion C o n g e n i t a l d i a p h r a g m a t i c h e r n i a s m a y v a r y in size a n d o c c u r in v a r i o u s p o r t i o n s o f t h e d i a p h r a g m . B a f f e s [9] reported the following frequency of congenital diaphragm a t i c h e r n i a s : P o s t e r o - l a t e r a l ( B o c h d a l e k ) 59.5 % , A n t e ro-medial (Morgagni) 2.6%, hiatal hernias 23.3%, eventration 14.6%. Postero-lateral diaphragmatic hernias r e s u l t f r o m a n a b s e n c e o r d e f e c t i v e f u s i o n of t h e sep-


Fig.3. Case3. Boy, 9years. Chest X-rays outside initially interpreted as normal. Retrospectively left sinus contour rounded and opacified (arrow) turn transversum dorsally and pleuroperitoneal membrane posteroqaterally. Developmental failure of the retrosternal portion of the septum transversum results in an antero-medial diaphragmatic hernia. Most Morgagni hernias are on the right side [10], postero-lateral diaphragmatic hernias are more frequent on the left side [4, 8]. Congenital diaphragmatic hernias can be differentiated from traumatic hernias by the lack of a history of trauma. Late herniations through the congenital defect have been postulated to occur when there is increased abdominal pressure from causes as surgery, vomiting or bowel distention (e.g. from enteritis) [11]. The spleen might protect left sided defects [11]. In case 3 enteritis and/or surgery might have been responsible for herniation after a nearly normal previous chest X-ray. Between 5 and 25 % of congenital postero-lateral diaphragmatic hernias present beyond the neonatal period [6, 8, 12]. The seven cases of pos tero-lateral hernia in our series constitute about 10.3 % of all congenital postero-lateral diaphragmatic hernias seen in our hospital. Congenital antero-medial diaphragmatic hernias usually present later in life [13]. The prognosis in late presenting congenital diaphragmatic hernias is good [5]. But an accurate diagnosis and immediate operative correction in symptomatic cases is mandatory because of (sometimes life threatening) com-

plications as: Cardio-respiratory arrest (possibly due to mediastinal compression by the herniated viscera) [7, 8], failure to thrive [3], incarceration or strangulation of herniated stomach or bowel with hypoxemic or ischemic injury resulting in necrosis, perforation or acute bleeding [6, 14, 15]. Mechanical bowel obstruction is another possible complication [16]. On the one hand large pleural effusions occur occasionally especially with hernias on the right side [17], on the other hand diaphragmatic hernias may also simulate pleural effusions or a loculated pyopneumothorax [14, 18]. In order to avoid a time delay in diagnosis it is mandatory to be aware of delayed presentation of congenital diaphragmatic hernia and to be familiar with the most common clinical presentations and roentgenographic patterns. Clinical symptoms usually are referable to the respiratory and/or gastrointestinal tract, sometimes recurrent but sometimes with a sudden onset in a previously healthy child [4, 5, 8]. Two of our patients had vomiting, three had vomiting and cough, two had cyanotic spells, one together with cough and one was admitted in a severe shock-state. In four children there was a sudden onset, four showed longer lasting symptoms. One case showed initially a completely normal chest X-ray followed by herniation of the stomach, one child had only minor radiographic changes in the costophrenic sinus, which were primarily incorrectly interpreted as normal and were followed 16 months later by herniation of stomach and massive mediastinal shift during surgery as a life threatening complication. If predominantly bowel is herniated in a left sided hernia the pattern with thinwalled translucencies and a mediastinal shift is well known. But if the spleen and/or the omentum is also herniated a mass lesion with only sparse translucencies might be observed, leading to the misdiagnosis of pneumonic consolidation with pneumatoceles. An important diagnostic clue for the correct roentgeno-

Fig.& Case3. 16 months later. Intraoperative chest X-ray. Large opacification of left hemithorax with large translucencies. Massive mediastinal shift. Postero-lateral diaphragmatic hernia with herniated stomach

190 niated liver and/or herniated bowel loops in the retrosternal or parasternal region. This was a retrospective study going back until 1976 without describing the role of ultrasound in this field. But it should be stressed that nowadays in cases of suspected diaphragmatic hernia ultrasound plays a major complementary role to roentgenographic technique in determining the diaphragmatic defect, the hernial content and for detection of pleural effusion [13, 17]. In four of our eight cases there was an undesirable time delay between first chest X-ray and surgery from two days up to 16 months, which could have been avoided if careful analysis of the chest X-rays was performed together with a knowledge of the potential clinical pictures of late presenting congenital diaphragmatic hernia. Two cases of our series confirm the statement that the diagnosis of congenital diaphragmatic hernia cannot be excluded if previous chest X-rays were largely normal [5, 8, 19, 20]. One of our cases (case 2) seems to confirm the statement that diaphragmatic hernia might be intermittent [5], since after 2 days of vomiting and anorexia the first standard chest X-ray was normal. Not before 2 days later a herniation of the stomach was detected. It seems that after a first episode of herniation with vomiting and anorexia the stomach returned to its abdominal location and in a second event was reherniated again being detected on the second chest X-ray 2 days later. Fig.5. Case 4. Male, 13 months. Primary diagnosis outside: Broncholitis. Reevaluation shows antero-medial herniation of parts of transverse colon (arrow). Antero-medial hernia of Morgagni. Associated atrial septal defect

graphic diagnosis in those cases are bubbly or looplike gas-accumulations in the lower part of the opacification, sometimes in close relation to bowel gas in the upper abdomen. They represent "connecting" bowel segments between abdominal and herniated bowel loops, passing through the diaphragmatic defect. According to the localization of the congenital diaphragmatic defect these translucencies are located either antero-medial or postero-lateral. Therefore in cases of doubt one should always search for translucencies in this region. We found this helpful in all our cases with herniated bowel. Furthermore in most of the cases there is no fever or cough and the lack of any laboratory changes should help to rule out pneumonia with pneumatoceles or loculated p y o p n e u m o t h o r ~ . If the stomach is herniated and airfilled a large translucency can result which may simulate pneumothorax or a congenital cystic lesion of the lung. On the other hand if the stomach is fluid filled because of intraoperative duodeno-gastric reflux, as in one of our cases, a large opacification may contain a large translucency representing some air in a dilated and predominantly fluid filled stomach. In such cases diagnosis can be difficult. But in a previous healthy child one should think at diaphragmatic hernia and insert a feeding tube for achieving the correct diagnosis. If one is not sure of the diagnosis some contrast material can be instillated. The antero-medial hernias commonly show a more characteristic pattern with her-

Conclusion Up to 25 % of congenital postero-lateral diaphragmatic hernias present later in life (10.3 % in on our own series). Clinical presentation and radiographic pattern are variable. The Prognosis does not depend on lung hypoplasia as in neonatal hernias, but relates to an accurate diagnosis of the condition and to the complications which include incarceration, strangulation and cardio-respiratory arrest. Prognosis is good if immediate surgery is performed in symptomatic children. A previous normal chest X-ray does not exclude diaphragmatic hernia. In cases of herniated bowel together with omentum and/or spleen larger opacities with sparse translucencies can lead to misinterpretation. Careful analysis of the chest film and searching for "connecting" bowel segments passing through the diaphragmatic defect may help to avoid incorrect diagnosis and an undesirable time delay. In cases of herniated stomach in previous healthy children a confusion with pneumothorax can be avoided by placing a feeding tube and diagnosis may sometimes be accomplished by instillation of contrast material. Ultrasound should be the complementary imaging modality of choice in all suspected diseases of the diaphragm in children, including congenital diaphragmatic hernias with delayed presentation.

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Professor Dr. R. Fotter Department of Radiology University Hospital Graz Auenbruggerplatz 9 A-8036 Graz, LKH Austria

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Delayed presentation of congenital diaphragmatic hernia.

Eight patients aged 1 month up to nine years with congenital diaphragmatic hernias (seven left sided postero-lateral, one right-sided antero-medial), ...
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