Sandbox: diaphragmatic hernia: Difference between revisions
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===Age=== | ===Age=== | ||
90% of the congenital diaphragmatic hernias present in the neonatal period. | 90% of the congenital diaphragmatic hernias present in the neonatal period. | ||
Classification | |||
Congenital diaphragmatic hernia can be classified into three types according to the site of herniation. | |||
Posterolateral (Bochdalek) diaphragmatic hernia | |||
It is the most common subtype. | |||
Most commonly occurs on the left side and rarely occurs bilaterally. | |||
Anterior (Morgagni) diaphragmatic hernia | |||
In anterior diaphragmatic hernia, the intestine bulges into the thorax through the anterior midline. | |||
Most of the cases of anterior diaphragmatic hernia occur on the right side. | |||
Hiatal hernia | |||
The intestine finds its way to the thorax through the esophageal hiatus. | |||
More common in the adults than in the neonates. | |||
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Pathophysiology | |||
The herniated viscera decrease the area needed by the lung to develop appropriately. | |||
According to the degree of herniation, the degree of pulmonary hypoplasia is determined. | |||
The development of the pulmonary artery tree is halted too resulting in excessive masculinization of the arteries. | |||
As most of the cases of diaphragmatic hernia is unilateral, the pulmonary hypoplasia is also usually unilateral, but it can be bilateral if the mediastinum is pushed by a massive unilateral hernia. | |||
The abnormal alveolar development can lead to hypoxemia leading to pulmonary vasoconstriction which aggravates the condition. | |||
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Natural history, complications and prognosis | |||
Natural history | |||
90% of the cases of congenital diaphragmatic hernia presents in the neonatal period. | |||
If left untreated, congenital diaphragmatic hernia can be fatal due to pulmonary hypoplasia and severe pulmonary hypertension. | |||
Complications | |||
GI complications | |||
Pulmonary hypoplasia | |||
Abnormal developmental rotation of the midgut | |||
Midgut volvulus | |||
Gastric volvulus | |||
Cardiopulmonary complications | |||
Pulmonary hypoplasia | |||
Pulmonary artery hypertension | |||
In severe cases, ventricular hypoplasia | |||
Prognosis | |||
The prognosis is mainly dependent on the size of the defect and the degree of the herniation. | |||
The presence or absence of liver herniation on fetal MRI is the most reliable prediction of the postnatal survival. | |||
The estimation of fetal lung volume and lung area to head circumference is a useful indicator for the prognosis of the disease in the absence of liver herniation. | |||
The mortality ranges from 25-60% of the cases. | |||
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==References== | ==References== | ||
Revision as of 14:24, 7 December 2017
Historical perspective
Historical perspective:
In 1679, the first case of congenital diaphragmatic hernia was reported by Riverius. In 1888, the first procedure to repair congenital diaphragmatic hernia in an adult was done and in 1889, the first procedure on an infant was done. In 1905, the first successful operation was done but the success rate was only about 60% in 1925. In 1940, A set of diagnostic criteria for diaphragmatic hernia were suggested. In 1950, the transthoracic approach was suggested instead of the transabdominal approach.
Epidemiology
Incidence
The incidence of congenital diaphragmatic hernia ranges from 50 to 150 per 100.000 live births.[1]
Prevalence
The prevalence of congenital diaphragmatic hernia ranges from 10 to 40 per live births.[2]
Sex
Congenital diaphragmatic hernia has no sex predilection.[2]
Age
90% of the congenital diaphragmatic hernias present in the neonatal period.
Classification
Congenital diaphragmatic hernia can be classified into three types according to the site of herniation.
Posterolateral (Bochdalek) diaphragmatic hernia It is the most common subtype. Most commonly occurs on the left side and rarely occurs bilaterally.
Anterior (Morgagni) diaphragmatic hernia In anterior diaphragmatic hernia, the intestine bulges into the thorax through the anterior midline. Most of the cases of anterior diaphragmatic hernia occur on the right side.
Hiatal hernia The intestine finds its way to the thorax through the esophageal hiatus. More common in the adults than in the neonates.
Pathophysiology
The herniated viscera decrease the area needed by the lung to develop appropriately. According to the degree of herniation, the degree of pulmonary hypoplasia is determined. The development of the pulmonary artery tree is halted too resulting in excessive masculinization of the arteries. As most of the cases of diaphragmatic hernia is unilateral, the pulmonary hypoplasia is also usually unilateral, but it can be bilateral if the mediastinum is pushed by a massive unilateral hernia. The abnormal alveolar development can lead to hypoxemia leading to pulmonary vasoconstriction which aggravates the condition.
Natural history, complications and prognosis
Natural history 90% of the cases of congenital diaphragmatic hernia presents in the neonatal period. If left untreated, congenital diaphragmatic hernia can be fatal due to pulmonary hypoplasia and severe pulmonary hypertension. Complications GI complications Pulmonary hypoplasia Abnormal developmental rotation of the midgut Midgut volvulus Gastric volvulus Cardiopulmonary complications Pulmonary hypoplasia Pulmonary artery hypertension In severe cases, ventricular hypoplasia
Prognosis The prognosis is mainly dependent on the size of the defect and the degree of the herniation. The presence or absence of liver herniation on fetal MRI is the most reliable prediction of the postnatal survival. The estimation of fetal lung volume and lung area to head circumference is a useful indicator for the prognosis of the disease in the absence of liver herniation. The mortality ranges from 25-60% of the cases.
References
- ↑ Fisher JC, Haley MJ, Ruiz-Elizalde A, Stolar CJ, Arkovitz MS (2009). "Multivariate model for predicting recurrence in congenital diaphragmatic hernia". J. Pediatr. Surg. 44 (6): 1173–9, discussion 1179–80. doi:10.1016/j.jpedsurg.2009.02.043. PMC 3072822. PMID 19524735.
- ↑ 2.0 2.1 McGivern MR, Best KE, Rankin J, Wellesley D, Greenlees R, Addor MC, Arriola L, de Walle H, Barisic I, Beres J, Bianchi F, Calzolari E, Doray B, Draper ES, Garne E, Gatt M, Haeusler M, Khoshnood B, Klungsoyr K, Latos-Bielenska A, O'Mahony M, Braz P, McDonnell B, Mullaney C, Nelen V, Queisser-Luft A, Randrianaivo H, Rissmann A, Rounding C, Sipek A, Thompson R, Tucker D, Wertelecki W, Martos C (2015). "Epidemiology of congenital diaphragmatic hernia in Europe: a register-based study". Arch. Dis. Child. Fetal Neonatal Ed. 100 (2): F137–44. doi:10.1136/archdischild-2014-306174. PMID 25411443.