A 9-month-old boy presents with two episodes of vomiting as well as episodes of colicky pain. His abdominal examination is notable for upper abdominal tenderness, and his stools are guaiac-positive. US demonstrates a target sign, and he is then taken for an air-contrast enema. What is the likelihood that he will need operative intervention?
Patients with intussusception should be assessed for the presence of peritonitis and for the severity of systemic illness. In the absence of peritonitis, the child should undergo radiographic reduction. The air enema is diagnostic and may also be curative, and it is the preferred method of diagnosis and treatment of intussusception. Air is introduced with a manometer, and the pressure that is administered is carefully monitored. Under most instances, this should not exceed 120 mm Hg. Successful reduction is marked by free reflux of air into multiple loops of small bowel and symptomatic improvement as the infant suddenly becomes pain-free. Unless both of these signs are observed, it cannot be assumed that the intussusception is reduced. If reduction is unsuccessful and the infant remains stable, the infant should be brought back to the radiology suite for a repeat attempt at reduction after a few hours. This strategy has improved the success rate of nonoperative reduction in many centers. In addition, hydrostatic reduction with barium may be useful if pneumatic reduction is unsuccessful. The overall success rate of radiographic reduction varies based on the experience of the center and is typically between 60 and 90%.
A newborn is found to have a blind rectal pouch on examination. Which of the following are not associated with this condition?
The patient is described to have an imperforate anus, the embryologic basis of which involves failure of descent of the urorectal septum, resulting in a blind rectal pouch that often has a fistulous tract. Approximately 60% of patients have an associated malformation, the most common of which is a urinary tract defect (approximately 50% of patients). Skeletal defects are also seen, with the sacrum most commonly involved. Spinal cord anomalies, especially tethered cord, are common, particularly in children with high lesions. Gastrointestinal anomalies occur, most commonly EA. Cardiac anomalies may be noted, and occasionally patients present with a constellation of defects as part of the VACTERRL syndrome (vertebral anomalies, anorectal anomalies, cardiac defects, tracheoesophageal fistula, renal anomalies, and radial limb hyperplasia).
Which type of choledochal cyst arises from the intraduodenal portion of the common bile duct?
Choledochal cysts refer to a spectrum of congenital biliary tract disorders, with a cyst wall composed of fibrous tissue and devoid of mucosal lining. Type I cysts are characterized by fusiform dilatation of the bile duct, type II by isolated diverticula protruding from the wall of the common bile duct, and type III cysts arise from the intraduodenal portion of the common bile duct (also known as choledochoceles). Type IVA consists of multiple dilatations of the intrahepatic and extrahepatic bile ducts, whereas Type IVB involves only the extrahepatic bile ducts. Lastly, type V (Caroli disease) cysts are all intrahepatic, and usually consist of multiple dilatations. Cysts can lead to abdominal pain, cholangitis, pancreatitis, as well as a biliary tract malignancy.
Which of the following is not characteristic of prunebelly syndrome?
Prune-belly syndrome refers to a disorder that is characterized by extremely lax lower abdominal musculature, dilated urinary tract including the bladder, and bilateral undescended testes. The term prune-belly syndrome appropriately describes the wrinkled appearance of the anterior abdominal wall that characterizes these patients. The incidence is significantly higher in men, and patients manifest a variety of comorbidities, the most significant of which is pulmonary hypoplasia, which is not survivable in the most severe cases. Skeletal abnormalities include dislocation or dysplasia of the hip and pectus excavatum. Approximately 80% of these patients will have some degree of vesicoureteral reflux, which can predispose to urinary tract infection. Despite the marked dilatation of the urinary tract, most children with prune-belly syndrome have adequate renal parenchyma for growth and development.
Undescended testes are usually repaired by what age?
Men with bilateral undescended testicles are often infertile. When the testicle is not within the scrotum, it is subjected to a higher temperature, resulting in decreased spermatogenesis. Mengel and coworkers studied 515 undescended testicles by histology and demonstrated a decreasing presence of spermatogonia after 2 years of age. Despite orchidopexy, the incidence of infertility is approximately two times higher in men with unilateral orchidopexy compared with men with normal testicular descent. Consequently, it is now recommended that the undescended testicle be surgically repositioned by 1 year of age.
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