Persistent Jaundice in a 1-Month-Old Infant (Case 43)

Chapter 62 Persistent Jaundice in a 1-Month-Old Infant (Case 43)



David A. Rogers, MD, MHPE



Differential Diagnosis











Biliary atresia Biliary hypoplasia
Choledochal cysts Inspissated bile syndrome



Speaking Intelligently


I am usually asked to see a jaundiced infant because there is a suspicion that the patient has a congenital anatomic defect of the biliary system that may be causing the jaundice. My goal is to make certain that the proper diagnostic tests are done and then prepare the patient and the family for the surgical procedure.



PATIENT CARE



Clinical Thinking



• Surgical causes of jaundice usually cause an elevation in conjugated bilirubin and so can be detected with a direct bilirubin serum level.

• For optimal outcome in biliary atresia, the Kasai procedure should be performed by 6 weeks of age, and so there is need for a timely workup and accurate dx.


History



• Determine when jaundice was first noted and its course.

• Review all labs done prior to surgical consultation. Conjugated hyperbilirubinemia from surgical causes tends to continuously progress until it is treated. There are a number of other nonsurgical causes that must be excluded (e.g., maternal-infant ABO incompatibility).

• A family hx of α1-antitrypson deficiency or cystic fibrosis will make these diagnoses more likely. Maternal pregnancy hx can provide clues about congenital infection causing jaundice.

• Stool color is quite important as obstruction of the biliary system will result in “acholic” stools, which lack bile pigment and are white or clay colored.


Physical Examination



• The main focus is the size and texture of the liver. Patients with biliary atresia develop hepatomegaly, and the liver is firm to palpation.

• The general physical examination may reveal diagnostic clues of an intrauterine infection.


Tests for Consideration
























• Hemoglobin level: Low levels may be indicative of a hematological disorder.
 $25

• Fractionated bilirubin: Will show whether the jaundice is due to conjugated or unconjugated bilirubin. Most causes that require surgical correction are related to conjugated bilirubin.
 $25

• Comprehensive metabolic profile: Will include most liver function tests.
 $45

• α1-Antitrypson levels: To exclude this specific deficiency.
 $70

• Torch titers: Allows for evaluation of intrauterine infections with toxoplasmosis, other infections (e.g., syphilis), rubella, herpes. Urine for cytomegalovirus should be sent by 3 weeks of age.
 $275

• Newborn screen: Checks for hypothyroidism, cystic fibrosis, and galactosemia that can cause direct hyperbilirubinemia.
 $250



IMAGING CONSIDERATIONS















→ Ultrasonography: First imaging tool in pediatrics. Fast, no radiation, and no need for sedation. Choledochal cyst easily seen, as is the remainder of the gallbladder/biliary tree.
 $150

→ Biliary scintography: Requires radioactive isotope to be taken up by the liver and excreted into biliary ducts. No excretion: biliary atresia. Accumulation in cyst: choledochal cyst.
 $450

→ Magnetic resonance cholangiopancreatography (MRCP): MRI technique creates 3D images of the biliary tree. Limited value in the neonate due to small anatomy and need for sedation.
 $900

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Mar 20, 2017 | Posted by in GENERAL SURGERY | Comments Off on Persistent Jaundice in a 1-Month-Old Infant (Case 43)

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