Patient Presentation
A 3-year-old male came to clinic with a history of pale stools off and on for 1 week. He was toilet training and so his mother was very aware of his elimination patterns. He had daily bowel movements which she said they were “pale” for a couple of days, then brown, then “pale” again. The color was uniform within soft, toothpaste consistency stools. She denied seeing any foreign material within the stool. He was otherwise acting normally and appeared well and was eating normally, and had clear “light-colored” urine multiple times a day. He had no rashes, fevers, chills, emesis or pain. She didn’t believe there were any weight changes. She denied any medications. The past medical and family history were non-contributory.

The pertinent physical exam showed a well-appearing male with growth parameters in the 50% range and was tracking from a visit 1 month previous. HEENT showed no scleral icterus. His skin had no jaundice or abnormal vein patterns. His abdomen was soft with normal size liver and spleen. No masses were palpated. He had some shotty anterior cervical and inguinal nodes. His examination was otherwise unremarkable.

The diagnosis of potentially abnormal colored stools was made.
Pictures provided by the mother within the toilet showed what appeared to be pale yellow stools that were uniform in color. The child otherwise appeared well so the physician told the mother that these appeared normal but to continue monitoring the patient for white or grey stools, or any signs that the patient was unwell.

Discussion
Color can be difficult to discern and communicate. Stooling is an important part of overall health and an important harbinger of potential illness. Stooling consistency and color changes with food, environment, medication, and health conditions.

For neonates and young infants, some hepatobiliary problems may not be seen at birth but in the next few days to weeks. This includes Alagille syndrome, biliary atresia and cystic fibrosis. It is not uncommon for these patients to have prolonged jaundice and therefore the diagnosis may be delayed with some presenting with increased jaundice, acholic stool and poor feeding. In older children causes of white/pale/clay-colored/grey stool may be easier to determine. Patients with underlying health problems usually have a different history (i.e. potential infectious hepatitis or medication exposure, increasing scleral icterus, poor eating, fever, etc.) and usually different physical examination. For a well-appearing child without concerning history or physical examination findings, the most common cause is a misperception or reporting of the change.

The differential diagnosis of acholic stools includes:

• Biliary disease
  • Liver
    • Alagille syndrome
    • Biliary atresia
    • Cystic fibrosis
    • Cirrhosis, end-stage liver disease
    • Infectious hepatitis
    • Drug-induced hepatitis
    • Tumors
  • Gallbladder
    • Anatomical problems
    • Biliary stricture
    • Gallstones
    • Sclerosing cholangitis
    • Tumors
  • Pancreas
    • Pancreatitis
    • Tumor
• Foreign bodies – crayons, paint, toys, worms
• Malabsorption and undigested food
• Medications – barium, antacids
• Misperception of color
• Mucus
• Tumors, abdominal

A review of different color stools can be found here.

Learning Point
True acholic stools should be appropriately evaluated usually with a gastroenterologist. As colors are difficult to discern, neonatal stool color cards have been used to help parents. There is also a mobile phone application which can help with classification of stool color. Images of neonatal stool color cards and acholic stools can be reviewed in To Learn More below.

Questions for Further Discussion
1. What is the most common reason for “pale or white” stool in your practice?
2. How do you approach the concern of “blood in stool?”
3. What would you include in your evaluation for acholic stools?

Related Cases

Disease: Acholic Stool | Digestive Diseases

Symptom/Presentation: Colors of Stools

Specialty: Gastroenterology | General Pediatrics

Age: Preschooler

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Bowel Movement and Digestive Diseases.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Chen SM, Chang MH, Du JC, et al. Screening for biliary atresia by infant stool color card in Taiwan. Pediatrics. 2006;117(4):1147-1154. doi:10.1542/peds.2005-1267

Gu YH, Yokoyama K, Mizuta K, et al. Stool color card screening for early detection of biliary atresia and long-term native liver survival: a 19-year cohort study in Japan. J Pediatr. 2015;166(4):897-902.e1. doi:10.1016/j.jpeds.2014.12.063

Shneider BL, Moore J, Kerkar N, et al. Initial assessment of the infant with neonatal cholestasis – Is this biliary atresia? PLoS One. 2017;12(5):e0176275. doi:10.1371/journal.pone.0176275

Madadi-Sanjani O, Blaser J, Voigt G, Kuebler JF, Petersen C. Home-based color card screening for biliary atresia: the first steps for implementation of a nationwide newborn screening in Germany. Pediatr Surg Int. 2019;35(11):1217-1222. doi:10.1007/s00383-019-04526-w

Singh H, Hong MH, Hinds R. Acholic stools and a small gallbladder: Not always a case of biliary atresia. Journal of Paediatrics and Child Health. 2020;56(11):1812-1813. doi:10.1111/jpc.14694

Angelico R, Liccardo D, Paoletti M, et al. A novel mobile phone application for infant stool color recognition: An easy and effective tool to identify acholic stools in newborns. J Med Screen. 2021;28(3):230-237. doi:10.1177/0969141320974413

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa