ICD-10-CM Q87: Other specified congenital malformation syndromes affecting multiple systems

ICD-10-CM Diagnosis Code (2025)

Official Description for Q87

Other specified congenital malformation syndromes affecting multiple systems

Coding Notes & Guidelines for Q87

Excludes1: Not Coded Here
  • Ellis-van Creveld syndrome (Q77.6)
  • Smith-Lemli-Opitz syndrome (E78.72)
Excludes1: Not Coded Here

Zellweger syndrome (E71.510)

Use Additional Code

code(s) to identify all associated manifestations

Use Additional Code

code to identify stage of chronic kidney disease (N18.1-N18.6)

Inclusion Terms
  • Acrocephalopolysyndactyly
  • Acrocephalosyndactyly [Apert]
  • Cryptophthalmos syndrome
  • Cyclopia
  • Goldenhar syndrome
  • Moebius syndrome
  • Oro-facial-digital syndrome
  • Robin syndrome
  • Whistling face
Inclusion Terms
  • Aarskog syndrome
  • Cockayne syndrome
  • De Lange syndrome
  • Dubowitz syndrome
  • Noonan syndrome
  • Robinow-Silverman-Smith syndrome
  • Russell-Silver syndrome
  • Seckel syndrome
Inclusion Terms
  • Holt-Oram syndrome
  • Klippel-Trenaunay-Weber syndrome
  • Nail patella syndrome
  • Rubinstein-Taybi syndrome
  • Sirenomelia syndrome
  • Thrombocytopenia with absent radius [TAR] syndrome
  • VATER syndrome
Inclusion Terms
  • Beckwith-Wiedemann syndrome
  • Sotos syndrome
  • Weaver syndrome
Inclusion Terms
  • Asadollahi-Rauch syndrome
  • Mediator complex subunit 13L syndrome
Code Also
  • , if applicable, any associated manifestations such as:
  • autism spectrum disorder (F84.0-)
  • congenital malformations of cardiac septa (Q21.-)
  • epilepsy and recurrent seizures (G40.-)
  • intellectual disability (F70-F79)

Child Codes (More Specific Codes for Q87):

Q87.0 - Congenital malformation syndromes predominantly affecting facial appearance Q87.1 - Congenital malformation syndromes predominantly associated with short stature Q87.11 - Prader-Willi syndrome Q87.19 - Other congenital malformation syndromes predominantly associated with short stature Q87.2 - Congenital malformation syndromes predominantly involving limbs Q87.3 - Congenital malformation syndromes involving early overgrowth Q87.4 - Marfan syndrome Q87.40 - Marfan syndrome, unspecified Q87.41 - Marfan syndrome with cardiovascular manifestations Q87.410 - Marfan syndrome with aortic dilation Q87.418 - Marfan syndrome with other cardiovascular manifestations Q87.42 - Marfan syndrome with ocular manifestations Q87.43 - Marfan syndrome with skeletal manifestation Q87.5 - Other congenital malformation syndromes with other skeletal changes Q87.8 - Other specified congenital malformation syndromes, not elsewhere classified Q87.81 - Alport syndrome Q87.82 - Arterial tortuosity syndrome Q87.83 - Bardet-Biedl syndrome Q87.84 - Laurence-Moon syndrome Q87.85 - MED13L syndrome Q87.86 - Kleefstra syndrome Q87.89 - Other specified congenital malformation syndromes, not elsewhere classified

About ICD-10-CM Code Q87 (Other specified congenital malformation syndromes affecting multiple systems)

ICD-10-CM code Q87 is a billable or specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. The 2025 edition of ICD-10-CM Q87 became effective on October 1, 2024.

This page provides detailed information about Q87 - Other specified congenital malformation syndromes affecting multiple systems. It includes common terminology, coding guidelines such as "Includes," "Excludes1," and "Excludes2" notes, and information on required 7th characters. Utilizing this information correctly is essential for accurate medical coding and claims submission for conditions like Other specified congenital malformation syndromes affecting multiple systems.