Diaphragmatic hernia masquerading as pneumothorax in two toddlers

Diaphragmatic hernia masquerading as pneumothorax in two toddlers

CASE REPORT pneumothorax Diaphragmatic Hernia Masquerading as Pneumothorax in Two Toddlers From the Division of General Pediatrics, Emergency Medic...

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CASE REPORT

pneumothorax

Diaphragmatic Hernia Masquerading as Pneumothorax in Two Toddlers

From the Division of General Pediatrics, Emergency Medicine, The Children's Hospital of Philadelphia;* and the Division of Emergency Medicine, St Christopher's Hospital for Children,* Philadelphia, Pennsylvania. Receivedfor publication May 19, I992. Revision received November 25, 1992. Accepted for publication December 17, 1992.

Joel A Fein, MD* John Loiselle, MD* Samuel Eberlein, MD* James F Wiley, MD t Louis M Bell, MD*

In two separate incidents, two toddlers with no previous history of respiratory ailments presented to the emergency department of a children's hospital with progressive respiratory distress. Both children had unilateral lung findings on auscultation and initial chest radiographs that were consistent with a pneumothorax. Thoracostomy and chest tube insertion were performed during initial resuscitation efforts. In both cases, subsequent radiographs revealed that the stomach was located in the left hemithorax, suggestive of a diaphragmatic hernia. Nasogastric tube insertion relieved the respiratory distress of these two children. Recognition of the "acquired" congenital diaphragmatic hernia in the setting of extreme aerophagia or mild abdominal trauma may prevent unnecessary procedures during the resuscitation of children with acute respiratory distress and unilateral lung findings. [Fein JA, Loiselle J, Eberlein S, Wiley JF, Bell LM: Diaphragmatic hernia masquerading as pneumothorax in two toddlers. Ann EmergMedJuly 1993;22:1221-1224.] INTRODUCTION Congenital diaphragmatic hernia typically occurs soon after birth in the form of a critically ill newborn. Presentation in the older child is less obvious, ranging from failure to thrive to acute respiratory distress. We report two cases of a late-onset diaphragmatic hernia simulating a pneumothorax.

CASE REPORTS Patient 1 A 27-month-old boy was brought to a local children's hospital emergency department by fire rescue personnel for evaluation of respiratory distress. The grandmother of the child reported that the child had been crying for several hours. On her return, the child's mother noted that the child was having some difficulty breathing and appeared to be blue around his lips. She called her pediatrician, who obtained the history that the child had

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been playing with a felt-tipped pen that morning and that the pen cap was missing. The child was referred to the ED with the concern that his symptoms were related to the aspiration of a foreign body. No history of trauma could be elicited. The patient's birth history, dietary history, and developmental progress were considered normal. He had been receiving routine pediatric care with a workup in progress for multiple episodes of loose boweI movements. On arrival at the ED the child was anxious and in moderate respiratory distress. His face and mouth were covered with marks from the felt-tipped pen. He was alert and responsive to verbal commands. Initial vital signs revealed a blood pressure of 120/90 mm Hg; temperature, 36.1 C axillary; respirations, 36; and pulse, 120. The physical examination was remarkable for central cyanosis, moderate intercostal and suprasternal retractions, and decreased breath sounds in the left hemithorax. A pectus excavatum was noted. No inspiratory stridor, tales, rhonchi, or wheezes were noted on chest examination. Pulse oximetry in room air revealed an oxygen saturation of 86%, which improved to 96% with supplemental oxygen administration. Back blows were administered without any change in the patient's clinical status. A portable chest radiograph was obtained (Figure 1A). The radiograph and clinical presentation were believed to be consistent with a left-sided pneumothorax. At that time, the patient's respiratory distress appeared to be increasing, and a 14-gauge needle catheter was inserted in the second intercostal space along the midclavicular line without any significant release of air or change in the patient's condition. This maneuver was followed by the insertion of a 16F chest'tube catheter into the fourth intercostal space at the anterior axillary line. A 14F nasogastric tube then was inserted, and a volume of air was aspirated. A follow-up radiograph was obtained (Figure 1B). The child's color and respiratory status improved dramatically. The patient subsequently underwent surgical repair of a moderate-sized Bochdalek hernia. Surgical findings revealed the presence of the stomach, splenic flexure of the colon, and a significant amount of small bowel displaced into the left hemithorax. The left lung was easily re-expanded to its normal size, and the patient was discharged without complications after five days. The child subsequently has done well without further episodes of respiratory distress. Patient 2 A 3-year-old boy developed a sudden, sharp pain in the left upper quadrant of his abdomen and in his left chest while throwing a football. Over the next few hours, he experienced intermittent episodes of sharp pain in the same areas associated with pallor and difficulty

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breathing. Six hours later, he presented to his private physician with labored breathing. A chest radiograph (Figure 2A) was reported to show a left-sided pneumothotax, and he was referred to a local children's hospital for treatment. His medical history was unremarkable. The patient arrived at the ED two hours later in marked respiratory distress with retractions and cyanosis. Blood pressure was 106/50 mm Hg; temperature, 36.0 C; respirations, 36; and pulse, 137. The trachea was deviated rightward, 3-cm jugular venous distention was noted, and only faint breath sounds were auscultated in the left hemithorax. After the chest radiograph was reviewed briefly, an emergency thoracocentesis was performed using a 14-gauge angiocatheter at the second intercostal space, midclavicular line. A large rush of air returned from the catheter, and the child improved immediately. A 20F drainage catheter then was inserted at the fourth intercostal space, anterior axfllary line, and breath sounds Figure 1. A: Initial chest radiograph of patient 1, obtained in the ED. B: Superiorly located chest tube catheter placement and the presence of a nasogastric catheter in the left hemithorax

Figure 2. A: Initial chest radiograph that accompanied patient 2 to the ED. B: Inferiorly located chest tube catheter and the presence of a nasogastric catheter in the left hemithorax

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in the left hemithorax improved. Over the next several hours, the chest tube was noted to drain a small amount of yellowish fluid, and the child's respiratory distress slowly returned. The original chest radiograph was reviewed, and a nasogastric tube was placed. A follow-up chest radiograph (Figure 213) showed the tip of the nasogastric tube located in the left hemithorax, strongly suggesting the presence of a diaphragmatic hernia. The patient subsequently underwent surgical repair of a congenital posterolateral defect, with an acute tear apparently extending the original opening to 8 x 5 cm. The childg stomach and spleen and two thirds of his small bowel were removed from the left hemithorax. The chest tube had not violated any viscera and was draining free serosanguinous fluid. The child did well after surgery and was discharged home from the hospital after five days. DISCUSSION

Congenital diaphragmatic hernia presents in newborns in approximately one of 2,500 live births 1 and is not a subtle diagnosis. In contrast, between 5°/0 and 30% of patients with congenital posterolateral diaphragmatic defects present with herniation after the age of 5 months. 2,3 The signs and symptoms of the cases in the literature range from wheezing, 2,4 frequent respiratory infections, 5 and mild gastroenteritis 3 to acute dyspnea, 6 pleural effusions, r and cardiorespiratory arrest, s Although the defect is present during fetal life, the o c c u r r e n c e of postnatal herniation through the defect is evidenced by cases reported months to years after previously normal chest radiographs were noted.9, lo In these cases, it has been suggested that the extrusion of abdominal contents is delayed or "protected" by the presence of the liver on the right side and the spleen on the left. 11 The abdominal contents can be restricted further by the presence of a thin hernial sac composed of pleura and peritoneum. 9 A significant mechanical pressure change in the thoracoabdominal cavity therefore is required to force abdominal contents through the defect. 12 In case 1, it is possible that the prolonged episode of crying precipitated these changes in thoracoabdominal pressures. In case 2, some trauma to the abdominal cavity probably occurred while the child played football. In the absence of prenatal herniation through the defect, the lungs of these patients developed normally. Both patients' ipsilateral lung volumes apparently were normal after repair of the defect. The diagnosis ,of congenital diaphragmatic hernia should be considered in any patient with an abnormal chest roentgenogram and respiratory or gastrointestina[

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symptoms. Because the majority of congenital diaphragmatic hernias are left sided (Bochdalek or Morgagni), a large proportion of these defects allow the stomach into the thoracic space. 5 Judicious placement of a nasogastric tube before radiographic evaluation therefore can aid in diagnosis. A barium contrast study carl be performed for confirmation and evaluation of severity. It should be noted, however, that the positioning and weight of the contrast material could inadvertently reduce the hernia contents and thereby underestimate the extent of the defect. 13 Nevertheless, in cases of traumatic diaphragmatic hernia, contrast studies probably represent the most useful confirmatory method. 14 Most children with late-presenting congenital diaphragmatic hernia do well after surgical repair. The morbidity associated with the lesion is related to the cardiorespiratory compromise incurred by the compression of normal thoracic structures as well as the disruption of the vascular supply of the herniated viscera, leading to gangrenous bowel. 15 Because the presentation of these patients can mimic acute pneumothorax, additional sequelae can be seen secondary to the perforation of a viscus by a thoracotomy tube. In one series, this occurred in 20% of patients with late-presenting left-sided congenital diaphragmatic hernia. 5 In both figures, a closer analysis of the abnormality reveals a radiopaque "membrane" surrounding a radiolucent area in the affected lung field. This represents the gastric wall surrounding the stomach contents. In addition to tension pneumothorax and diaphragmatic hernia, the differential diagnosis of this radiograph includes congenital lobar emphysema, pneumatocele, and cystic adenomatoid malformation. Although the discomfort experienced by a newborn infant associated with air swallowing is well known to parents, it often is not considered in the emergency situation. Small volumes of air in the stomach of a crying infant can result in what first appears to be painful abdominal distention and may distract the examinerg attention from the true areas of illness or inju~ Patient 1 demonstrates the extreme of aerophagia, in which air swallowing from prolonged crying presumably raised the intra-abdominal pressure, resulting in a herniation through an existing defect in the diaphragm. After upper airway obstruction was ruled out, this diagnosis was acted on as a potentially life-threatening cause of this childg respiratory distress. SUMMARY

We present two cases of congenital diaphragmatic hernia that became clinically apparent in the third or fourth year

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of life. Although the initial chest radiographs of both patients were misread as pneumothorax, the placement of a nasogasmc tube provided symptomatic relief and suggested the correct diagnosis. Both patients did well after surgical management. A substantial proportion of children with "acquired" congenital diaphragmatic hernia present with mild gastrointestinal or respiratory complaints. The diagnosis can remain elusive until an acute exacerbation or other circumstances force radiologic evaluation. The diagnosis might be suspected earlier when an acute respiratory &compensation is preceded by increased abdominothoracic pressure, such as bowel distention, Valsalva from vomiting or crying, or blunt abdominal trauma. 14 These cases demonstrate the importance of gastric decompression in the evaluation of the young child. We recommend that if the airway is stable, nasogastric tube placement be considered as an early adjunct in the infant or young child with an unusual presentation of respiratory distress.

Address for reprints: Louis M Bell, MD The Children's Hospital of Philadelphia 34th Street and Civic Center Boulevard Philadelphia, Pennsylvania 19104

REFERENCES 1. Seigel M, Shackford G, McAlister W: Left-sided congenital diaphragmatic hernia: Delayed presentation. AJR1981;137:43-46. 2. Osebold W, Seper R: Congenital pesterolateral hernia past infancy. AmJSurg 1976;131:748-754. 3. Newman B, Afshani E, Karp M, et al: Presentation of congenital diaphragmatic hernia past the neonatal period. Arch Surg1986;121:813-816. 4. Singer J: Herniation of abdominal contents simulating status asthmaticus. PodiatrEmergCare 1987;3:250-252. 5. Berman L, Stringer D, Ein S, et al: The late-presenting pediatric Bochdalek hernia: A 20-year review. J PediatrSorg1988;23:735-739. 6. Reynolds V, May R: Delayed-onset Bochdalek hernia in a 3-year-old child. Am J EmergMed 1988;6:594-595. 7. Hurdiss L, Taybi H, Johnson L: Delayed appearance of left-sided diaphragmatic hernia in infancy. J Pediatr1976;88:990-992. 8. Byard R, Bohn D, Wilson G, et al: Unsuspected diaphragmatic hernia: A potential cause of sudden and unexpected death in infancy and early childhood. J PediatrSurg1990;25:1166-1168. 9. Glasson M, Barter W, Cohen D, et al: Congenital left posterelateral diaphragmatic hernia with previously normal chest x-ray. PediatrRadio/1975;3:201-205. 10. Heij H, Dos AP, Hasebroek W: Acquired congenital diaphragmatic hernia. EurJPediatr 1987;146:440-441. 11. Wiseman NI MacPherson R: Acquired congenital diaphragmatic hernia. JPediatrSurg 1977;12:657-665. 12. Day B: Late appearance of Bochdalek hernia. BrMedJ1972;1:786. 13. Kirkland J: Cengenital posterior diaphragmatic hernia in the adult. BrJSurg 1959;47:16. 14. McHugh K, Ogilvfe B, Drunton F: Delayed presentation of traumatic diaphragmatic hernia. ClinRadiol1991 ;43:246-250. 15. Mishalany HG, Nakada K, Wooley M: Congenital diaphragmatic hernias: Eleven years' experience. Arch Surg1979;114:1118-1123.

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