Portal hypertension is increased intravascular pressure of the portal vein. The prevalence of causes in children is different from adults ones. The commonest cause of pediatric portal hypertension is the extra-hepatic portal hypertension, comparing with an adult where liver cirrhosis is the comments cause. Also, taking into consideration, the fundamental physiological differences between the two age groups. These elements are making the attempt to extrapolate the adult guidelines to the pediatric age group unpractical. On the other hand, the limitation of well-designed studies in the pediatric age group makes reaching a consensus about the safety and efficiency of primary prophylaxis of variceal bleeding difficult. In contrast, there were enough data to recommend the secondary prophylaxis of variceal bleeding and the safety and efficiency of Meso-Rex shunt for portal hypertension have been confirmed. These indicate the necessity of further studies to reach a complete algorithm of guidelines for pediatric portal hypertension.

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Hirschsprung disease is the most common cause of low functional bowel obstruction in the pediatric age group, which caused by a complex genetic mutations which interfere with normal development of the enteric nervous system, about 2/3 of the cases presented as isolated disease, the other cases could present with congenital anomalies or within syndromic context, the surgical intervention consider curable for most of the cases provided it the surgical technique applied meticulously.