Asplenia (congenital)
ICD-10 Q89.01 is a billable code used to indicate a diagnosis of asplenia (congenital).
Asplenia, or the congenital absence of the spleen, is a rare condition that can occur as an isolated anomaly or as part of a syndrome involving multiple congenital malformations. The spleen plays a crucial role in the immune system, particularly in filtering blood and fighting infections. Children with congenital asplenia are at increased risk for infections, particularly from encapsulated bacteria such as Streptococcus pneumoniae and Haemophilus influenzae. Clinical management often includes prophylactic antibiotics and vaccinations to mitigate infection risk. Asplenia can be associated with other congenital malformations, including cardiac defects, gastrointestinal anomalies, and other organ system abnormalities. Diagnosis is typically made through imaging studies, such as ultrasound or MRI, which can visualize the absence of the spleen. Genetic counseling may be indicated, especially if asplenia is part of a syndrome. Understanding the implications of asplenia is essential for pediatric care, as these patients require careful monitoring and preventive measures to ensure their health and well-being.
Detailed history of congenital anomalies, vaccination status, and infection prophylaxis measures.
Management of a child with asplenia presenting with recurrent infections or during routine check-ups.
Ensure documentation reflects the multidisciplinary approach to care, including immunology and infectious disease consultations.
Genetic testing results, family history of congenital conditions, and any syndromic associations.
Counseling families about the implications of asplenia and associated genetic syndromes.
Consider the need for genetic testing in cases of syndromic asplenia and document findings thoroughly.
Administered to children with asplenia to prevent infections.
Document vaccination date and type.
Pediatricians should ensure that vaccination schedules are followed.
Patients with congenital asplenia require careful monitoring for infections, vaccination against encapsulated organisms, and possibly prophylactic antibiotics. Regular follow-up with pediatricians and specialists is essential to manage their health effectively.
