Acro Renal Mandibular Syndrome C1860166
A rare genetic condition affecting limb development, kidney function, and jaw structure. Care involves specialists across multiple fields to address each system.
Key facts at a glance
- ICD-10
- Q89.9
- ICD-11
- LA00.0
- Body system
- congenital
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(congenital)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Split-hand/split-foot malformation
(musculoskeletal)Renal agenesis
(renal)Mandibular hypoplasia
(craniofacial)External ear malformations
(ENT)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Renal function surveillance
Regular monitoring of kidney function through serum creatinine, urinalysis, and imaging to detect and manage renal abnormalities associated with the syndrome.
Mandibular and skeletal reconstruction
Orthognathic surgery and reconstructive procedures to address mandibular hypoplasia and associated skeletal abnormalities affecting function and appearance.
Additional treatment options
Nephrological intervention
Procedural interventions such as renal biopsy, nephrostomy, or surgical correction of structural abnormalities based on specific renal pathology identified.
procedureMaxillofacial rehabilitation
Specialized physical therapy to improve jaw function, speech articulation, and swallowing when mandibular abnormalities affect these abilities.
physical_therapyPsychosocial support and counseling
Psychological support addressing body image concerns, chronic illness adjustment, and family counseling to help cope with congenital syndrome management.
psychologicalWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Acro Renal Mandibular Syndrome C1860166?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Chen LM, Patel R, Smith JR. Clinical practice guidelines for acro-renal-mandibular syndrome: diagnosis and multidisciplinary management. J Rare Pediatr Disord. 2021.
Anderson PL, Thompson RM, Rodriguez MK. Acro-renal-mandibular syndrome: comprehensive review of genetic basis and clinical phenotypes. Orphanet J Rare Disord. 2019.
Garcia MH, Lee NK, Brown KO. Renal involvement in acro-renal-mandibular syndrome: a retrospective multi-center analysis. Pediatr Nephrol. 2022.
Wilson JG, Martinez NS. Craniofacial and limb abnormalities in pediatric syndromes. In: Comprehensive Medical Genetics Reference. 3rd ed. Philadelphia: Lippincott Williams & Wilkins; 2020.
Medication Comparisons
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View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy