We report for the first time a unique case of VACTERL vertebral anomalies, anal atresia or imperforate anus, cardiac anomalies, tracheoesophageal fistula, renal and limb defect spectrum associated with portal hypertension. She had caput medusae , ascites, splenomegaly, gastric and esophageal varices. Her liver function tests were within normal limits. Magnetic resonance imaging of the liver with contrast showed a thready portal vein with collateral vessels involving both right and left portal veins without intrahepatic duct dilation. Understandably, clinicians should give low priority to looking for portal hypertension in VACTERL spectrum patients presenting with gastrointestinal bleeding. The clinical manifestation of VACTERL association includes vertebral anomalies, anal atresia, congenital heart disease, tracheoesophageal fistula, renal dysplasia and limb abnormalities [ 1 ].
Anorectal Malformations / Imperforate Anus
Types of Anal Malformations
Clinically there is no anal opening. Subtypes can be classified into two broad categories: high supralevator or low infralevator , depending on the location of the atretic portion. Please Note: You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. Updating… Please wait.
Imperforate pronounced im-PURR-for-ate anus means there is no opening for poop stool or feces to leave the body at the end of the digestive tract. This problem can happen in these ways:. Why they happen is not known. Each letter stands for a possible problem:.
An imperforate anus or anorectal malformations ARMs are birth defects in which the rectum is malformed. ARMs are a spectrum of different congenital anomalies in males and females which vary from fairly minor lesions to complex anomalies. There are several forms of imperforate anus and anorectal malformations. The new classification is in relation of the type of associated fistula. Imperforate anus is usually present along with other birth defects— spinal problems, heart problems, tracheoesophageal fistula , esophageal atresia , renal anomalies and limb anomalies are among the possibilities, collectively being called the VACTERL association.