Short answer · Medically reviewed summary · Last updated: 2026-05-08
The Pentalogy of Cantrell is classified under ICD-10 code Q79.5 (Other congenital malformations of abdominal wall) and ICD-9 code 756.79 (Other congenital anomalies of abdominal wall). Because Pentalogy of Cantrell is an extremely rare and complex condition, it does not have its own unique, specific code in either system, but rather falls under broader categories of abdominal wall defects. What exactly is Pentalogy of Cantrell? The Pentalogy of Cantrell is a rare congenital disorder characterized by a combination of five specific defects: a midline supraumbilical abdominal wall defect, a defect of the lower sternum, a deficiency of the anterior diaphragm, a defect of the pericardium, and congenital heart anomalies.
ICD10 code of Pentalogy Of Cantrell and ICD9 code
ICD-10 and ICD-9 codes for Pentalogy Of Cantrell, with classification details for clinicians, coders and patients.
The Pentalogy of Cantrell is classified under ICD-10 code Q79.5 (Other congenital malformations of abdominal wall) and ICD-9 code 756.79 (Other congenital anomalies of abdominal wall). Because Pentalogy of Cantrell is an extremely rare and complex condition, it does not have its own unique, specific code in either system, but rather falls under broader categories of abdominal wall defects.
What exactly is Pentalogy of Cantrell?
The Pentalogy of Cantrell is a rare congenital disorder characterized by a combination of five specific defects: a midline supraumbilical abdominal wall defect, a defect of the lower sternum, a deficiency of the anterior diaphragm, a defect of the pericardium, and congenital heart anomalies. Due to the complexity of the Pentalogy of Cantrell, clinical management requires a multidisciplinary team approach, as the severity of these defects can vary significantly between individuals.
What are the diagnostic criteria for Pentalogy of Cantrell?
Diagnosis of Pentalogy of Cantrell is typically made via prenatal ultrasound or fetal echocardiography. Because the condition involves multiple organ systems, clinicians look for the following hallmark features:
- Ectopia cordis (the heart is located partially or completely outside the chest).
- Omphalocele (abdominal organs protruding through the umbilical cord).
- Sternal cleft or absence of the lower sternum.
- Diaphragmatic hernia or aplasia.
- Intracardiac defects, most commonly ventricular septal defects (VSD) or diverticulum of the left ventricle.
Is Pentalogy of Cantrell hereditary?
Most cases of Pentalogy of Cantrell are sporadic, meaning they occur randomly without a family history. While the exact cause remains unknown, researchers believe Pentalogy of Cantrell arises from a failure of the lateral mesoderm to develop properly during the first few weeks of embryonic development. Currently, there is no evidence that it is an inherited genetic condition, though genetic counseling is recommended for families seeking to understand their specific situation.
Next steps
- Consult with a pediatric cardiothoracic surgeon and a neonatologist to coordinate complex surgical planning.
- Connect with the Pentalogy of Cantrell community at DiseaseMaps.org to share experiences with other families.
- Request a referral to a high-risk maternal-fetal medicine specialist if the diagnosis is made prenatally.
Medical disclaimer: This information is for educational purposes only and does not replace professional medical advice, diagnosis, or treatment; always seek the advice of your physician regarding any medical condition.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Pentalogy of Cantrell.
- Orphanet: Pentalogy of Cantrell (ORPHA:1367).
- OMIM (Online Mendelian Inheritance in Man): Cantrell Pentalogy (Entry #313850).
