Congenital diaphragmatic hernia presenting as massive gastrothorax

CASE REPORT diaphragmatic hernia, congenital

Congenital Diaphragmatic Hernia Presenting as Massive Gastrothorax Delayed herniation of abdominal contents through a congenital diaphragmatic hernia may occur beyond the neonatal period. The case of a 29month-old child with a Bochdalek hernia presenting as acute respiratory failure is presenced. Chest radiography showed a tension gastrothorax that was misread as a tension pneumothorax. Tube thoracostomy resulted in clinical improvement by perforating and decompressing the stomach. Nasogastric tube placement confirmed herniation of the stomach into the left chest and is the initial treatment of choice when a tension gastrothorax is identified. A congenital diaphragmatic hernia m u s t be recognized promptly so that rapid gastric decompression and surgical repair of the diaphragmatic defect can be performed. [Snyder HS, 8alo DF, Kelly PH: Congenital diaphragmatic hernia presenting as massive gastrothorax. Ann Emerg Med May 1990;19:562-564.] INTRODUCTION The majority of congenital diaphragmatic hernias (CDHs) present in the neonatal period with tachypnea, cyanosis, and acidosis. While delayed presentation of a CDH is less frequent and has a lower mortality rate,] 3 there are exceptions. The case of a 29-month-old child with a Bochdalek-type CDH presenting as a massive gastrothorax is presented. Our case illustrates that the diagnosis of a Bochdalek CDH may be difficult and that significant morbidity and mortality may result without proper treatment.

Howard S Snyder, MD David F Salo, MD Peter H Kelly, MD Albany, New York From the Department of Emergency Medicine, Albany Medical Center Hospital, Albany, New York. Received for publication August 11, 1989. Revision received November 28, 1989. Accepted for publication December 21, 1989. Address for reprints: Howard S Snyder, MD, Department of Emergency Medicine, Albany Medical Center Hospital, 47 New Scotland Avenue, Albany, New York 12208.

CASE REPORT A 29-month-old boy presented to our emergency department in severe respiratory distress. The patient had a one-week history of a persistent mild, croupy cough. On the evening of admission, the child developed increased respiratory secretions and complained of right ear pain. On arrival at the physician's office, he was found to be in severe respiratory distress with cyanosis. He arrived in our ED after tolerating approximately 40 minutes of progressive respiratory insufficiency. The patient's history was unremarkable for respiratory or gastrointestinal complaints. Immunizations were current, and the child was on no medications. Physical examination showed an irritable, intermittently crying, acutely ill child lying on his left side. There were grunting respirations, an irritative cough productive of clear mucous, and obvious perioral cyanosis. Vital signs revealed a 10-kg child with a rectal temperature of 38.6 C; pulse, 144; and respirations, 26. Head, eyes, ears, nose, and throat examination showed a greyish right tympanic membrane with diminished mobility and an abnormal light reflex. The left tympanic membrane was normal. The lung examination showed scattered rhonchi on the right with good air movement. The left lung showed audible breath sounds diffusely that appeared to be somewhat distant and diminished. Cardiac examination showed a normal S1 and $2 at a rate of 120 to 140. The abdomen was somewhat scaphoid but nontender with audible bowel sounds. The nails were cyanotic but had good capillary refill. The remainder of the examination was normal. The child was placed on 100% supplemental oxygen by face mask after pulse oximetry showed an arterial oxygen saturation of 88% on room air.

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DIAPHRAGMATIC HERNIA Snyder, Salo & Kelly

FIGURE. Anteroposterior (A) and lateral (B) chest radiographs showing dilated stomach with air-fluid level in ]eft hemithorax. Note superior rim f o r m e d by compressed lung and stomach wall in addition to mediastinal shift to the right. A chest radiograph was obtained and initially interpreted as showing a large tension pneumothorax on the left side with significant shift of the mediastinum to the right (Figure). No prior chest radiographs were available. A 20F chest tube was carefully inserted under finger guidance into the left fourth interspace in the midaxillary line with only a slight return of air. A follow-up chest film showed persistence of a large air-fluid level in the left chest. The chest tube was placed on low wall suction, and the child showed a rapid improvement in c l i n i c a l s t a t u s i n c l u d i n g disappearance of the cyanosis and decrease in respirations. He became calm to the point of falling asleep. An additional chest radiograph showed a significant reduction in the size of the air-filled structure in the left chest with a normalization of the mediastinal shift. However, a rim of compressed lung in the left apex was noted to be present on each film. On transfer to the pediatric ICU, the diagnosis of Bochdalek hernia was considered, and an inserted nasogastric tube was found to coil in 114/563

the left chest. At surgery, the stomach, spleen, and much of the transverse colon were found in the chest as a result of herniation through a 2-cm posterolateral defect in the diaphragm. The chest tube was found to have been inserted into the stomach, and this defect was repaired along with the hernia. The postoperative course was without complications, and the child was discharged on day 8.

DISCUSSION B o c h d a l e k - t y p e (posterolateral) CDH results from a lack of the fus i o n and c l o s u r e of t h e d o r s a l p l e u r o p e r i t o n e a l canals that normally occur about the eighth week of embryonic life. This entity was first described by Boner 4 in 1679 and later by Bochdalek 5 in 1848. The incidence of Bochdalek hernias has been reported as between one in 3,6006 and one in 7,2002 live births. Presentation of a CDH may be delayed in 5% to 30% of cases, depending on the study.U,3, 7-11 CDHs may present in childhood or adulthood with acute or chronic symptoms. It has been suggested that occlusion of the diaphragmatic defect by liver or spleen prevents herniation in many cases.t2,13 Acute h e r n i a t i o n occurs with decreased intrathoracic pressure or increased intra-abdominal pressure.14, is Cases of delayed herniation have been reported after trauma, t5 sexual intercourse, 16 diving, 17 sneezAnnals of Emergency Medicine

ing, is and inversion on an amusement park ride, i4 and during pregnancy, labor, and childbirth.19, ~° S y m p t o m s of a l a t e - p r e s e n t i n g Bochdalek hernia include recurrent pulmonary infections, dyspnea, wheezing, chest pain, a b d o m i n a l pain, failure to thrive, vomiting, diarrhea, and anorexia. Physical examination reveals diminished breath sounds, tachypnea, cyanosis, dextrocardia, bowel sounds in the chest, and a scaphoid or distended abdomen. R e s p i r a t o r y s y m p t o m s are more c o m m o n in younger children (median age, 14 months), whereas gastrointestinal symptoms predominate in older children (median age, 5 years). 7 Several cases of acute respiratory failure and cardiopulmonary arrest have been reported. 21 Delayed presentation of a CDH has been mistaken in children for status asthmaticus, pneumonia, pleural effusion, p n e u m o t h o r a x , and c y s t i c l u n g bullae.7,21 22 The radiographic appearance of a CDH can be quite varied. Chest radiographs are often misread as pneum o n i a w i t h o p a c i f i c a t i o n of the lower lung fields and loss of the diap h r a g m a t i c outline. Multiple airfluid levels in the chest simulate a necrotizing pneumonia (ie, staphylococcal) with pneumatocele or abscess formation. Incarceration of bowel produces a serosanguinous exudate mimicking an inflammatory pleural 19:5 May 1990

e f f u s i o n and m a y e v e n progress to empyema.~ 3 Haines and Collins have n o t e d t h a t o m e n t u m in t h e c h e s t produces a scalloped superior margin on a lateral decubitus film instead of the straight line seen with free fluid. 24 H e r n i a t i o n of a dilated stomach into the chest m a y be m i s t a k e n for a cystic lung malformation, gastric volvulus, or p n e u m o th o r a x . D i s p l a c e m e n t of m e d i a s t i n a l structures and hypotension can occur w i t h f o r m a t i o n of a t e n s i o n gastrothorax, s i m u l a t i n g a t e n s i o n p n e u m o t h o r a x . T h e r e m a y be d i l a t e d loops of bowel in the a b d o m e n w i t h m u l t i p l e afr-fluid'levels, r e s e m b l i n g bowel o b s t ru c ti o n or paralytic ileus. T h e c o m b i n a t i o n of c h e s t f i n d i n g s suggesting p n e u m o n i a and abdominal findings suggesting o b s t r u c t i o n in the absence of gas in the r e c t u m should arouse suspicion for the presence of a CDH. 23 O u r case d r a m a t i c a l l y i l l u s t r a t e s h o w a gastrothorax can be m i s t a k e n for a tension p n e u m o t h o r a x . The patient presenting in acute respiratory distress requires i m m e d i a t e diagnost i c and t h e r a p e u t i c i n t e r v e n t i o n . In other situations, w h e n t i m e permits, useful diagnostic modalities include b a r i u m s t u d i e s of t h e u p p e r a n d lower g a s t r o i n t e s t i n a l tract, liverspleen scanning, and c o m p u t e d tomography in an a t t e m p t to identify i n t r a - a b d o m i n a l c o n t e n t s in t h e chest cavity. However, these tests are n o t helpful in th e acutely ill patient, and proper interpretation of the chest radiograph becomes crucial. T h e d e v e l o p m e n t of a spontaneous t e n s i o n p n e u m o t h o r a x in an otherw i s e w e l l c h i l d is an u n c o m m o n event. Both entities share the following features: increased l u c e n c y of one h e m i t h o r a x , s h i f t of m e d i a s t i n a l structures, acute respiratory distress, and, o c c a s i o n a l l y , h y p o t e n s i o n resulting from mediastinal compress i o n and d e c r e a s e d v e n o u s r e t u r n . Radiographic findings suggestive of a gastrothorax include a large air-fluid level in the dilated stomach, a superior r i m formed by compressed lung a n d s t o m a c h w a l l , a n d l a c k of a s t o m a c h b u b b l e in t h e l e f t u p p e r quadrant. Bowel sounds in the chest and a scaphoid or d i s t e n d e d (if obs t r u c t i o n is p r e s e n t ) a b d o m e n are clinical findings that further support the diagnosis. T h e m a n a g e m e n t of a gastrothorax i n v o l v e s rapid p l a c e m e n t of a ha19:5 May 1990

sogastric tube to decompress the dilated s t o m a c h , a l t h o u g h i n t u b a t i o n of the intrathoracic s e g m e n t m ay be difficult. Passage of t h e n aso g ast r i c tube into the chest cavity on a chest radiograph confirms the diagnosis and is e s p e c i a l l y h e l p f u l w h e n t h e s t o m a c h is the only herniated organ. Immediate clinical improvement s h o u l d o c c u r after s t o m a c h d e c o m pression, although strangulation and n e c r o s i s of t h e b o w e l or s t o m a c h should be suspected if shock persists. T h e incorrect diagnosis of a tension p n e u m o t h o r a x , as i n o u r p a t i e n t , leads to needle or tube t h o r a c o s t o m y t h a t p u n c t u r e s t h e d i s t e n d e d st o m ach wall and spills t h e gastric contents into the pleural cavity. Although providing temporary clinical i m p r o v e m e n t , this m a y delay the diagnosis and r e s u l t in e m p y e m a formation. After the diagnosis is made, surgical i n t e r v e n t i o n is required to repair t h e a n a t o m i c defect and prev e n t further complications.

SUMMARY We report the case of a 29-monthold c h i l d w i t h a B o c h d a l e k h e r n i a presenting as a m assi v e gastrothorax. A c u t e r e s p i r a t o r y distress, t r a c h e a l shift, decreased b r e a t h sounds, and bowel sounds in the chest raise the s u s p i c i o n of t e n s i o n g a s t r o t h o r a x . Proper interpretation of the chest radiograph allows differentiation from tension p n e u m o t h o r a x . P l a c e m e n t of a nasogastric tube is both diagnostic and therapeutic. N e e d l e or tube thor a c o s t o m y m a y result in clinical imp r o v e m e n t despite perforation of the dilated s t o m a c h and c o n t a m i n a t i o n of the pleural space. Once a C D H has been identified, surgical repair is indicated. The authors thank Barbara Norton and Jaimie Miller for their assistance in preparation and Edward C Geehr, MD, for his review of this manuscript.

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