Asian Cardiovasc Thorac Ann 2006;14:525-527
© 2006 Asia Publishing EXchange Ltd
Late Presentation of Congenital Bochdalek Hernia: A Case Report
Pankaj K Mishra, MCh,
Michael Shackcloth, MD,
Elizabeth Andrew, MD,
Richard D Page, FRCS (CTh)
Department of Cardiothoracic Surgery, Liverpool Cardiothoracic Center, Liverpool, United Kingdom
For reprint information contact: Pankaj K Mishra, MCh Tel: 44 20 7188 7883 Fax: 44 20 7188 0073 Email: mishrapk_25{at}yahoo.com, Department of Cardiothoracic Surgery, St. Thomas Hospital, Lambeth Palace Road, London SE1 7EH, United Kingdom.
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ABSTRACT
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A 23-year-old man presented with a long history of dyspnea and wheezing thought to be due to asthma. Abnormal appearance of the left hemithorax was an incidental finding on a chest X-Ray. On further investigations he was found to have congenital Bochdalek hernia which was repaired surgically. All his respiratory symptoms resolved and he was able to discontinue treatment for asthma. We want to emphasise that late presentations can be misleading even to an astute clinician.
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INTRODUCTION
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Congenital Bochdalek hernia usually presents in neonates. It results from a developmental defect in the posterior part of the diaphragm, which allows a free communication between the thoracic and abdominal cavities. The pleuroperitoneal canals usually close by the 8th week of gestation. Failure of closure leads to a diaphragmatic defect, which is usually located in the region of the 10–11th ribs. A Bochdalek hernia presenting in adult life is rare.1,2 Late presentations can be misleading even to an astute clinician.
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CASE REPORT
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A 23-year-old man presented with a long history of shortness of breath, wheeze, and intermittent episodes of chest tightness. He was prescribed regular inhalers as well as oral bronchodilator therapy. He denied any history of chest wall injury, and had no bowel symptoms. He also suffered from severe atopic eczema. As a consequence of the need to commence systemic treatment with Azathioprine for his eczema, a chest X-Ray was performed (Figure 1
). This showed marked elevation of the left hemidiaphragm with pleural thickening and blunting of the left costophrenic angle. The right lung appeared hyperinflated while there was volume loss within the left lower lobe. A computed tomography (CT) scan revealed an unusual appearance on the left side of the chest with fluid and feces-filled bowel loops in the left hemithorax suggesting a defect in the left hemidiaphragm (Figure 2). The most likely diagnosis was felt to be a congenital diaphragmatic hernia, although eventration and traumatic rupture of the diaphragm were other possibilities. Magnetic resonance image (MRI) scanning could not identify diaphragmatic muscle on the left side. Forced vital capacity and forced expiratory volume per second were 1.7 L (34.6% predicted) and 1.29 L (30.7% predicted) respectively.
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Figure 1. Preoperative chest X-Ray showing marked elevation of the left hemidiaphragm with blunting of the left costophrenic angle. The right lung appears hyperinflated.
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Video assisted thoracoscopic surgery (VATS) revealed almost complete herniation of the bowel contents into the left hemithorax. Left thoracotomy through the bed of the 9th rib revealed that the whole bowel from the duodenojejunal flexure to the mid-descending colon had herniated through a 6 x 6 cm defect in the posterolateral diaphragm. The lower lobe of the left lung was hypoplastic. The rest of the diaphragmatic muscle was unremarkable. There was no hernia sac. The herniated contents were free of any adhesions and were reduced without problem into the abdominal cavity. The defect in the diaphragm was closed with Marlex mesh sutured to the free edge of the diaphragmatic defect and the adjacent ribs. The patient’s recovery from surgery was uneventful with routine postoperative management. Postoperative chest X-Ray showed fully expanded lungs.
On follow-up at five months the patient was asymptomatic and no longer required bronchodilators. Forced vital capacity and forced expiratory volume per second were 3.5 L (70.3% predicted) and 2.58 L (61.1% predicted) respectively.
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DISCUSSION
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Bochdalek’s hernia is the most common form of congenital diaphragmatic hernia in neonates. It usually presents as life-threatening respiratory distress. It was described by Victor Alexander Bochdalek, an anatomist in 1848.3 Although it can occur on both sides as originally described by Bochdalek, it is five times more common on the left side due to the fact that the pleuroperitoneal canal closes earlier on the right side. The frequency of congenital diaphragmatic hernias has been variously described as 1 in every 3000–4000 live births.3 There is a predilection for male sex with a male to female ratio of 3:2 for left-sided hernias and 3:1 for right-sided hernias.4 The condition usually presents in infancy but rarely can present in late childhood.1 Delayed presentations can cause diagnostic difficulties, as symptoms are usually vague and non-specific. Our patient had no abdominal complaints at all. He only had respiratory symptoms which were attributed to asthma, which in retrospect he did not suffer from.
The diagnosis may be suspected clinically by the presence of bowel sounds in the lower hemithorax or by a thoracic position of a nasogastric tube on a plain chest X-Ray.5 Siegel et al reported that ultrasonography of the thorax can be helpful in establishing the diagnosis as it can show an air-containing mass in the thorax.6 Giving a patient oral contrast and then visualizing bowel loops above the diaphragm on a plain film 24 hours later can also establish diagnosis with a reasonable amount of certainty. The diagnosis can also be established prior to birth by ultrasonography.
In adults the condition is easily missed unless there is a high index of suspicion.5,7 It has been reported that nearly 40% of adults are misdiagnosed as having a pleural effusion for which attempted needle aspiration could be disastrous.5,7 Once the diagnosis is established, operative repair should be undertaken to prevent life-threatening complications as well as to lead to an improvement in symptoms.
Surgical access is good through the chest although an abdominal approach can be useful if associated malrotation of the gut is present. The hernia does not usually have a sac. The defect in the diaphragm can be closed with nonabsorbable sutures, or if the defect is large a prosthetic mesh can be used. Repair of a small Bochdalek hernia can be accomplished by VATS with a shorter recovery period and less postoperative pain.8
In conclusion, although the vast majority of Bochdalek hernias present in infancy they may also present in adults. A high index of suspicion is required as there are no classical symptoms. The case we presented was misdiagnosed for several years as asthma. Once correct diagnosis was made, the condition was easily repaired surgically with great benefit to the patient.
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REFERENCES
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- Berman L, Stringer D, Ein SH, Shandling B. The late-presenting pediatric Bochdalek hernia: a 20-year review. J Pediatr Surg 1988;23:735–9.[Medline]
- Naunheim KS. Adult presentation of unusual diaphragmatic hernias. Chest Surg Clin N Am 1998;8:359–69.[Medline]
- Gale ME. Bochdalek hernia: prevalence and CT characteristics. Radiology 1985;156:449–52.[Abstract/Free Full Text]
- Tibboel D, Gaag AV. Etiologic and genetic factors in congenital diaphragmatic hernia. Clin Perinatol 1996;23:689–99.[Medline]
- Baglaj M, Dorobisz U. Late-presenting congenital diaphragmatic hernia in children: a literature review. Pediatr Radiol 2005;35:478–88.[Medline]
- Siegel MJ, Shackelford GD, McAlister WH. Left-sided congenital diaphragmatic hernia: delayed presentation. AJR Am J Roentgenol 1981;137:43–6.[Abstract/Free Full Text]
- Thomas S, Kapur B. Adult Bochdalek hernia—clinical features, management and results of treatment. Jpn J Surg 1991;21:114–9.[Medline]
- Silen ML, Canvasser DA, Kurkchubasche AG, Andrus CH, Naunheim KS.Video-assisted thoracic surgical repair of a foramen of Bochdalek hernia. Ann Thorac Surg 1995;60:448–50.[Abstract/Free Full Text]
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ABSTRACT
INTRODUCTION
