Craniosynostosis is a birth defect. In this segment of “In the kNOW”, we will delve into the different types of craniosynostosis and the FY2024 expansion of codes that cover this condition.
As initially noted, craniosynostosis is a birth defect affecting the sutures or the space between skull bones that is normally flexible at birth. This flexibility allows for brain growth. Normally, sutures will remain flexible until a child is about two years of age, which permits the brain to grow to its normal size. Upon closure of the sutures, the skull bones become fused together, and the sutures become bone. In craniosynostosis, the sutures fuse or close too soon meaning before the brain in fully formed. This will lead to deformity(ies) of the head as growth will continue in the areas where suture closure has not yet taken place.
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Source: https://www.cdc.gov/ncbddd/birthdefects/craniosynostosis.html |
Source: https://www.hopkinsmedicine.org/health/conditions-and-diseases/craniosynostosis
Treatment depends on the type of craniosynostosis. If the suture closure(s) did not impact brain growth, the skull deformity may not be very noticeable or hidden by hair growth. In some instances, helmet therapy is used to remodel the skull. In more severe cases, the fusion(s) may prevent brain growth leading to pressure building in the skull which could lead to seizures, or brain damage. Surgery can be done to relieve the pressure and attempt to correct the craniosynostosis. These surgeries are usually performed in the first year of a child’s life. While most children with craniosynostosis are healthy aside from this condition, again, in severe cases, children may experience brain damage, and/or intellectual difficulties. Each case of craniosynostosis is different ranging from mild to severe and the treatment regime will be tailored accordingly.
FY2024 ICD-10-CM code updates expanded the
coding of craniosynostosis in order to identify the particular suture affected
and, when applicable, the laterality of the suture closure.
Q75.001 Craniosynostosis unspecified, unilateral
Q75.002 Craniosynostosis unspecified, bilateral
Q75.009 Craniosynostosis unspecified
Imperfect fusion of skull
Q75.01 Sagittal craniosynostosis
Non-deformational dolichocephaly
Non-deformational scaphocephaly
Sagittal craniosynostosis is
the most common type and runs across the top of the head, from the fontanelle
(soft spot) to the back of the head. Scaphocephaly describes the early fusion of the sagittal suture.
Q75.02 Coronal craniosynostosis
Non-deformational anterior plagiocephaly
Excludes1: dolichocephaly (Q67.2)
Q75.021 Coronal craniosynostosis unilateral
Non-deformational anterior plagiocephaly
Q75.022 Coronal craniosynostosis bilateral
Non-deformational brachycephaly
Q75.029 Coronal craniosynostosis unspecified
Coronal craniosynostosis
affects the suture that goes from the back of the ear to the top of the head
where it meets the sagittal suture. This
is the second most common type of craniosynostosis. Brachycephaly is another term for the bilateral
coronal suture premature closure.
Q75.03 Metopic craniosynostosis
Trigonocephaly
Metopic craniosynostosis affects the suture
that runs from the baby’s nose to the sagittal suture on top of the head. It is also known as trigonocephaly.
Q75.04 Lambdoid craniosynostosis
Non-deformational posterior plagiocephaly
Excludes1:
dolichocephaly (Q67.2)
Q75.041 Lambdoid craniosynostosis, unilateral
Q75.042 Lambdoid craniosynostosis, bilateral
Q75.049 Lambdoid craniosynostosis, unspecified
Lambdoid
craniosynostosis impacts the suture that goes along the back of the head.
Q75.051 Cloverleaf skull
Kleeblattschaedel skull
Q75.052 Pansynostosis
Q75.058 Other multi-suture craniosynostosis
Excludes1: coronal craniosynostosis, bilateral (Q75.022)
lambdoid craniosynostosis, bilateral
(Q75.042)
Q75.08 Other single-suture craniosynostosis
Lambdoid and metopic are rarer types of
craniosynostosis.
Now you are In the kNOW!!
About the Author
Dianna Foley, RHIA, CHPS, CCS, CDIP, is an HIM professional with over 25 years of experience. She earned her bachelor’s degree from the University of Cincinnati and holds RHIA, CHPS, CDIP, and CCS certifications from AHIMA, along with being an AHIMA-approved ICD-10-CM/PCS trainer. Dianna has held many positions in HIM and is now an independent coding consultant. She previously served as the program director for Medical Coding and HIT at Eastern Gateway Community College. Dianna is an AHIMA-published author and has volunteered with AHIMA on projects including certification item writing, certification exam development, coding rapid design, and most recently has served on AHIMA’s nominating committee. She is a presenter on coding topics at the national, state, and regional levels and serves as OHIMA’s Education Coordinator. Dianna mentors new AHIMA members and also provides monthly educational lectures to coders and clinical documentation specialists.
