ILLUSTRATIVE CASE

Diaphragmatic Hernia in a Pediatric Emergency Department Pasquale Comberiati, MD,* Luca Giacomello, MD,† Francesco S. Camoglio, MD,† and Diego G. Peroni, MD‡ Abstract: Diaphragmatic hernia in pediatric emergency department poses a diagnostic challenge because of the acute or subtle timing of onset and the wide variety of clinical features. We describe 3 different late presentations of Bochdalek diaphragmatic hernia in a pediatric emergency department. These reports may help physicians avoid delayed diagnosis of late-presenting congenital diaphragmatic hernia, thereby reducing the risk of inappropriate treatments and life-threatening conditions in children. Key Words: congenital diaphragmatic hernia, CT scan, radiography, wheezing (Pediatr Emer Care 2015;31: 354–356)

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ongenital diaphragmatic hernia (CDH) is a congenital anomaly of uncertain etiology, which occurs around 1 in 3000 live births. In most cases, CDH is diagnosed during antenatal ultrasound screening or within a few hours or days after birth presenting with severe respiratory distress and causing life-threatening conditions.1 However, in 5% to 25% of patients, CDH may manifests beyond the neonatal period or infancy.2 This late-presenting CDH can have acute or insidious onset and be characterized by nonspecific symptoms, which may lead to delayed diagnosis and inappropriate treatments.3 Here we described 3 different cases of late-presenting CDH in a pediatric emergency department.

CASES Case 1 A 3-month-old male infant exclusively fed with cow's milk formula was admitted to our emergency department because of repetitive vomiting since the last 2 days. These episodes mainly occurred a few hours after being fed. Previously, the baby boy had been well, except for a mild episode of vomiting that occurred 2 weeks before. No abnormalities in bowel movements or habit were reported. On admission, the infant was restless, but conscious, with no dyspnea, wheezing, or abnormalities in the vital signs (respiratory rate, 25 breaths per minute; heart rate, 105 beats per minute; normal blood pressure; no fever; and oxygen saturation, 100%). The abdominal, respiratory, and cardiovascular examinations were unremarkable, except for a slightly reduction of breath sounds in the left hemithorax. An intravenous saline rehydration solution partially improved his situation. A food protein-induced enterocolitis syndrome was suspected, and the patient was kept under observation. A few hours after, the child started to vomit without attempts at oral intake. Hematologic tests, venous blood gases, and inflammatory indexes were carried out, which came out all negative. Because of the persistence of reduction of breath sounds in the left hemithorax, a chest radiography From the *Pediatric Clinic, Department of Life and Reproduction Sciences, †Pediatric Surgery Unit, Department of Surgical Sciences, University of Verona, G.B. Rossi Polyclinic, Verona; and ‡Section of Paediatrics, University of Ferrara, Ferrara, Italy. Disclosure: The authors declare no conflict of interest. Reprints: Diego G. Peroni, MD, Section of Paediatrics, University of Ferrara, Via A. Moro 8, Cona 44124, Ferrara, Italy (e‐mail: [email protected]). Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. ISSN: 0749-5161

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(CXR) was carried out, which showed a left-sided diaphragmatic hernia. A chest computed tomography (CT) confirmed this finding the day after the child underwent surgery. At laparotomy, a Bochdalek hernia was seen, with herniation of the small bowel and left colon, but not of the stomach, into the left hemithorax. No signs of ischemia of the bowel were seen. The herniated viscera were carefully reduced into the abdomen, and the diaphragmatic defect closed with interrupted nonabsorbable (Ti-Cron 4-0) suture, without leaving an intercostal tube. The infant was discharged 1 week after the surgery without complications.

Case 2 A 16-month-old female thriving infant was admitted to our emergency department with a 2-day history of fever and cough. Previously, the child had been well, except for a reported history of poor intake and intermittent vomiting in the last 4 months. On admission, the patient was conscious, but tachypneic (respiratory rate, 35 breaths per minutes), tachycardic (heart rate, 186 beats per minute), febrile (temperature, 37.2°C), and in marked distress. Blood pressure and oxygen saturation were both in the reference range. The abdominal examination was unremarkable. No abnormalities in bowel movements or habit were reported. The respiratory system examination revealed a slightly reduction of breath sounds in the left hemithorax. A respiratory infection with a left pleural effusion was suspected. Biochemical and hematologic investigations were carried out, which revealed a white blood cell count of 11.440 cells/mm3 and a C-reactive protein level of 10 mg/L. A CXR was also carried out, which showed the presence of the small bowel in the left hemithorax without pleural effusion. Finally, a chest CT revealed a left posterolateral diaphragmatic hernia. At emergency surgery, which was performed within a few hours, a Bochdalek hernia was found. Part of the small bowel, transverse and left colon, but not the stomach, had herniated into the left hemithorax. No signs of ischemia of the bowel were seen. The herniated viscera were carefully reduced into the abdomen, and the diaphragmatic defect closed with interrupted nonabsorbable (Ti-Cron 4-0) suture without leaving

FIGURE 1. CXR showing left-sided diaphragmatic hernia, with mediastinal shift to the right side. Pediatric Emergency Care • Volume 31, Number 5, May 2015

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Pediatric Emergency Care • Volume 31, Number 5, May 2015

FIGURE 2. CT scan showing herniation of the small bowel and transverse colon in the left hemithorax, consistent with left-sided diaphragmatic hernia.

an intercostal tube. The patient was discharged 1 week after the surgery without complications.

Case 3 A 14-month-old male thriving infant was admitted to our emergency department with acute respiratory distress and wheezing. A history of recurrent wheezing in the last few months, requiring inhaled short-acting β2-agonist, was reported. On admission, the child was conscious and apyretic, but restless and tachypneic with a respiratory rate of 40 breaths per minute and an oxygen saturation of 96%. The cardiovascular examination showed a heart rate of 150 beats per minute and a normal blood pressure. The abdominal examination was unremarkable, and no abnormalities in bowel movements were reported. The respiratory system examination revealed a markedly reduction of breath sounds on the left hemithorax. Therefore, a CXR was carried out, which showed much of the small bowel in the left chest, with a mediastinal shift to the right side (Fig. 1). Computed tomography scan confirmed the presence of a left posterolateral diaphragmatic hernia (Fig. 2). An emergency surgery was performed within a few hours. At operation, a Bochdalek hernia was identified. The small bowel and transverse colon, but not the stomach, were seen in the thoracic cavity with no signs of ischemia and carefully reduced into the abdomen. The diaphragmatic defect was closed with interrupted nonabsorbable (Ti-Cron 4-0) suture without leaving an intercostal tube. The postoperative recovery was uneventful, and the patient was discharged 6 days after without complications and in good conditions.

DISCUSSION Late-presenting CDH is a significant diagnostic challenge for emergency department physicians because of its low incidence, variable timing of onset (age range, 5 weeks to 14 years; median age of presentation in our cases, 11 ± 7 months), and various clinical presentations with nonspecific respiratory and/or gastrointestinal symptoms, such as dyspnea, tachypnea, cough, wheezing, abdominal pain, vomiting, and diarrhea.2–6 The onset can be either insidious with intermittent/recurring symptoms or acute with respiratory distress or gastrointestinal complications in a child who has been well until that day.7 In addition, there is evidence that late-presenting CDH can have normal CXR before the clinical onset because the spleen or liver may temporarily occlude the diaphragmatic defect.7,8

CDH in a Pediatric Emergency Department

Our article describes 3 different late presentations of Bochdalek diaphragmatic hernia in a pediatric emergency department. The great variability in timing of clinical onset (corresponding to herniation) and the nonspecificity of symptoms associated with late-presenting CDH may cause misdiagnosis and inappropriate treatments.9 In case 1, a food protein-induced enterocolitis syndrome was suspected at first because of repetitive vomiting occurring a few hours after the ingestion of cow's milk formula, which is characteristic of this non–IgE-mediated food allergy.10 In case 2, pneumonia was the initial diagnosis because of the 2-day history of fever and cough and the respiratory distress on admission. Finally, in case 3, the initial suspect was of an acute asthma exacerbation because of the reported history of recurrent wheezing in the last few months. Interestingly, in all our cases, the respiratory system examination revealed a reduction of breath sounds in the left hemithorax, which helped us make the right diagnosis in a few hours in case 1 and avoid a delayed diagnosis in case 2 and 3. Indeed, in our opinion, the most important clinical feature that can help emergency department physicians focus the differential diagnosis of a late-presenting CDH is the examination finding of asymmetric decreased breath sounds. A prompt diagnosis of late-presenting CDH is crucial to prevent impaired development of children and life-threatening conditions, such as small bowel strangulation and cardiorespiratory arrest.11 Imaging studies are essential in confirming the diagnosis.8 Chest radiography is recommended as the first diagnostic study. However, initial radiographic features of late-presenting CDH can be misinterpreted in about 25% of cases. Tension pneumothorax and pleural effusion are the most common initial incorrect diagnosis according to CXR, resulting in inappropriate procedures, such as chest tube insertion, and subsequent risk of gastrointestinal perforation.3,12 Computed tomography scan is recommended to confirm the diagnosis and avoid inappropriate chest drain placement when clinical features raise the suspect of CDH, but CXR findings are incomplete or confusing. Chest radiography after the passage of a nasogastric tube placement or upper gastrointestinal series are alternative methods of confirming the diagnosis.3 Surgical correction of the diaphragmatic defect is the appropriate treatment. The prognosis of late-presenting CDH is usually favorable when a prompt surgical repair is made.11

CONCLUSIONS Late-presenting CDH should always be considered in the differential diagnosis of nonspecific respiratory and gastrointestinal symptoms in pediatric emergency department, especially when clinical examination reveals asymmetrical signs, such as decreased breath sounds in 1 hemithorax.

REFERENCES 1. de Buys Roessingh AS, Dinh-Xuan AT. Congenital diaphragmatic hernia: current status and review of the literature. Eur J Pediatr. 2009;168: 393–406. 2. Cigdem MK, Onen A, Otcu S, et al. Late presentation of Bochdalek-type congenital diaphragmatic hernia in children: a 23-year experience at a single center. Surg Today. 2007;37:642–645. 3. Bagłaj M, Dorobisz U. Late-presenting congenital diaphragmatic hernia in children: a literature review. Pediatr Radiol. 2005;35:478–488. 4. Waseem M, Quee F. A wheezing child: breath sounds or bowel sounds? Pediatr Emerg Care. 2008;24:304–306.

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5. Blackstone MM, Mistry RD. Late-presenting congenital diaphragmatic hernia mimicking bronchiolitis. Pediatr Emerg Care. 2007;23:653–656.

9. Chang SW, Lee HC, Yeung CY, et al. A twenty-year review of early and late-presenting congenital Bochdalek diaphragmatic hernia: are they different clinical spectra? Pediatr Neonatol. 2010;51:26–30.

6. Wu CT, Huang JL, Hsia SH, et al. Late-presenting congenital diaphragmatic hernia in pediatric emergency room: two case reports. Eur J Pediatr. 2009;168:1013–1015.

10. Banzato C, Piacentini GL, Comberiati P, et al. Unusual shift from IgE-mediated milk allergy to food protein-induced enterocolitis syndrome. Eur Ann Allergy Clin Immunol. 2013;45:209–211.

7. Chao PH, Chuang JH, Lee SY, et al. Late-presenting congenital diaphragmatic hernia in childhood. Acta Paediatr. 2011;100:425–428.

11. Baerg J, Kanthimathinathan V, Gollin G. Late-presenting congenital diaphragmatic hernia: diagnostic pitfalls and outcome. Hernia. 2012;16: 461–466.

8. Zaleska-Dorobisz U, Bagłaj M, Sokołowska B, et al. Late presenting diaphragmatic hernia: clinical and diagnostic aspects. Med Sci Monit. 2007; 13:137–146.

12. Muzzafar S, Swischuk LE, Jadhav SP. Radiographic findings in late-presenting congenital diaphragmatic hernia: helpful imaging findings. Pediatr Radiol. 2012;42:337–342.

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© 2015 Wolters Kluwer Health, Inc. All rights reserved.

Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.

Diaphragmatic hernia in a pediatric emergency department.

Diaphragmatic hernia in pediatric emergency department poses a diagnostic challenge because of the acute or subtle timing of onset and the wide variet...
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