Srikumar, Jainaha

Fibrous dysplasia (FD) is a benign bone neoplasm often considered a disease of childhood, with existing literature suggesting lesions typically become quiescent by young adulthood. Consequently, the presentations and surgical outcomes of adult patients remain poorly characterized. This retrospective review analyzed 50 adult patients treated surgically for craniofacial FD at a single institution between 1988 and 2024 to evaluate patterns of presentation, operative indications, and long-term outcomes. The cohort (54.0% male) had a mean follow-up of 15.8 years. Polyostotic involvement was present in 60.0% of patients, and the frontal bone was most affected (51.2%). The median age at presentation was 31 years, and the median age at initial surgery was 34.5 years. The most common surgical indications were visible growth (30.5%) and pain or headache (27.1%). Of the 59 surgeries performed to directly treat FD, bone contouring only was the most common initial operative approach (n=42), followed by en bloc resection and reconstruction (n=7). However, 12% of patients undergoing initial contouring required additional operations for continued growth. All secondary deformities requiring corrective surgery were ocular. In addition, several patients demonstrated delayed recurrence or progression decades after primary surgical intervention. These findings suggest that craniofacial FD may remain symptomatic or continue to progress into adulthood, challenging the traditional paradigm that these lesions necessarily become quiescent after skeletal maturity and highlighting the importance of long-term surveillance in the adult population.

Objective We aimed to understand the impact of Cranial Remolding Orthotic therapy (CRO) on the quality of life of both infants with positional deformational head shape abnormalities (PDHSAs), including plagiocephaly and brachycephaly, and their caregivers. Design Survey study. Setting A single, tertiary academic center. Patients, Participants Families of infants with PDHSAs who underwent CRO between 2007 and 2022. Interventions CRO. Main Outcome Measure(s) Subjective assessment of caregiver perspectives. Results We obtained 242 responses (72% male) from 1013 patients contacted (response rate 23.9%). CRO was initiated at a median age of 6 months (IQR 5-7 months), with a median treatment duration of 4 months (IQR 3-5 months). Insurance covered costs fully for 9.8% of respondents and partially for 47.0%; 43.2% paid completely out-of-pocket. Most were “very satisfied” or “satisfied” with head shape (89.2%) and ear position (93.3%). CRO did not impact children's sleep (75.2%), “never” or “rarely” impacted children's play (90.5%), and never impacted most families’ attendance of social events (91.3%). Hair loss was temporary in 29.9% and permanent in 0.8%. Most families did not find CRO burdensome (73.1%) and “never” or “rarely” felt fatigued (68.5%). Conclusions The study sheds light on the mental and social effects of CRO on patients and caregivers, as well as physical adverse effects associated with treatment. Overall, patients’ families expressed satisfaction with results and felt that administering CRO had minimal negative impacts on themselves and their children.

Surgical treatment and outcomes of fibrous dysplasia (FD) in pediatric patients are areas that have not been fully studied. The authors completed a retrospective review of 20 patients who had at least one surgery for craniofacial fibrous dysplasia before 18 years of age. Surgeries were categorized into 3 groups: bone contouring only; removal of the involved bone, contouring, and replacement of the contoured bone; or en bloc resection of the involved bone and reconstruction with an alloplastic material, bone allograft, or bone autograft obtained from non-involved sites. A total of 36 distinct surgical procedures were performed in 20 patients, with a mean number of 1.9 surgeries/patient (range 1–5). The maxilla was both the bone that was most commonly affected and most commonly operated on. The most common surgical indication was visible or palpable growth in 63.9% of cases. Bone contouring only was the initial surgery in 15 patients, of whom 9 patients (55.6%) had continued bone growth requiring subsequent surgeries. Removal, contouring, and replacement wasthe initial surgery in 1 patient, who required a secondary surgery. Four patients, all with monostotic calvarial disease, initially had an en bloc resection and reconstruction; none had recurrence. Patients with subsequent surgeries had a mean time to operation of 3.7 years (range 0.24–9.8). The recurrence rate and need for secondary surgical procedures were relatively high in patients who had bone contouring only. These patients tended to be patients with facial bone involvement more than calvarial involvement.