Table 4 Achondroplasia-related surgical categories in primary achondroplasia cohort (PAC) (n = 1,374).

≥1 Achondroplasia-related surgery	Procedures	Subjects, n (%)
Yes (ever)	4,552	1,094 (79.6)
No (never)		167 (12.2)
Unknown		113 (8.2)
Total		1,374
Surgical category^a
Ear, nose, and throat	2,803	893 (65.0)
Brain	326	137 (10.0)
Foramen magnum + C1/C2	314	281 (20.5)
Spine^b	425	175 (12.7)
Extremity	684	291 (21.2)

One thousand ninety-four (79.6%) of the primary achondroplasia cohort (PAC) had at least one achondroplasia-related surgery in one of the categories and 167 (12.2%) subjects were confirmed to never have had a surgical procedure in one of the categories. The surgical status of the remaining 113 subjects (8.2%) is unknown. One hundred one subjects of 113 with an unknown surgical history were from the oldest three birth cohorts (before 1980, n = 37; 1980–1990, n = 30; 1990–2000, n = 34) for whom clinical records were limited and/or subjects were lost to follow-up. Thus, there are only 12 subjects from the last two birth cohorts for whom a validated surgical history is unknown. A total of 460 non-achondroplasia-related surgeries (e.g., appendectomy, Cesarean section, exploratory laparotomy) in 302 PAC subjects were extracted from the medical records. Further analysis of these procedures is not feasible because of incomplete ascertainment of all members of the PAC. Among the 302 individuals who had a non-achondroplasia-related surgery, 271 also had a least one achondroplasia-related surgery, leaving 31 subjects (31/1,374 = 2.3%) who had exclusively non-achondroplasia-related surgeries documented in their medical history.
^aSubjects may have surgeries in more than one surgical category.
^bSpine category includes procedures from C3 through the sacrum.

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