Srikumar, Jainaha K.

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 To evaluate cancer risk in individuals with cleft lip and/or cleft palate (CL/P). Design Retrospective chart review of patients with CL/P, including abstraction of information on cancer diagnoses was conducted. Cancer risk was compared with national population-based estimates using standardized incidence ratios (SIRs) and exact binomial tests. Subgroup analyses compared demographic and clinical characteristics between patients with and without cancer. Setting Study was performed at a single tertiary academic center with multidisciplinary surgical, pediatric, and genetic care. Patients/Participants Total of 2121 patients with CL/P from 1970 to 2024 with complete medical documentation were included. Main Outcome Measures Main outcomes included prevalence of cancer diagnoses, childhood cancer incidence, and differences in cancer occurrence by age, cleft type, syndromic status, and family history. Results Total of 144 patients (6.8%) had pathologically confirmed cancer diagnoses. Twelve childhood cancers were identified, with 10 cases presenting before age 15. Children younger than 15 with CL/P had a significantly elevated cancer risk (SIR 4.7; 95% confidence interval: 1.8-7.6; P < .00001). Pediatric cancers occurred mainly in syndromic cases. Cancer prevalence was higher in cleft palate only and cleft lip/palate cases compared with cleft lip only ( P < .05). Cancer occurrence was not higher in adults with CL/P compared to the general population. Conclusions Cancer risk is not uniformly elevated over the lifespan in individuals with CL/P. Increased cancer risk is observed in our pediatric cohort. Clinical care should include careful assessment of childhood cancers, syndromic features, and family history, with consideration for genetic counseling and multidisciplinary follow-up.

Objective: To characterize pediatric patients experiencing unplanned intraoperative extubation (UIE) during cleft lip (CL) and cleft palate (CP) repair and assess postoperative outcomes. Design: Retrospective cohort study using the 2019–2023 National Surgical Quality Improvement Program–Pediatric database. Setting: Multicenter surgical registry of pediatric operations. Patients: Children undergoing CL repair (<1 year) or CP repair (<3 years). Interventions: Not applicable. Main Outcome Measure(s): Incidence of UIE, demographic and operative characteristics, and 30-day postoperative complications. Results: Among 17 047 cases, UIE occurred in 0.37% of CL repairs and 0.20% of CP repairs. Patients who experienced UIE had similar baseline characteristics to those without UIE but demonstrated significantly longer operative and anesthesia times for both procedures. Despite these intraoperative events, no cases of death, pneumonia, or unplanned reintubation occurred postoperatively, and overall 30-day complication rates remained low. Conclusions: UIE during CL and CP repair is rare and not associated with major postoperative complications.

Objective We sought to characterize the frequency of postoperative opioid prescriptions in patients undergoing cleft lip repair (CLR). Design The NSQIP-P 2023 database was queried to identify patients undergoing CLR using CPT codes. Univariable analysis was conducted to identify factors associated with postoperative opioid prescription. Setting A multicenter sample from 157 participating sites. Patients/participants A total of 1497 cases (1215 unilateral CLR and 282 bilateral CLR) were identified. Main outcomes/measures The postoperative outcomes of interest were whether an opioid was prescribed to a patient at discharge and what factors were independently associated with opioid prescribing at discharge. Results A total of 44.6% of patients with a mean age of 5.1 ± 1.9 months were prescribed opioids at discharge, most commonly oxycodone (76.3%). Univariable analysis identified an increase in weight (kg) (OR: 1.09, 95% CI: 1.02-1.17, P = .03), American Society of Anesthesiologists (ASA) classification (ordinal) (OR: 1.25, 95% CI: 1.06-1.47, P = .01), anesthesia time (10 min) (OR: 1.02, 95% CI: 1.01-1.04, P = .03), and age (months) (OR: 1.06, 95% CI: 1.00-1.12, P = .04) as being associated with opioid prescription at discharge. Bilateral versus unilateral CLR ( P = .49) and outpatient versus inpatient operation ( P = .38) were not associated with opioid prescribing. Conclusion Opioid stewardship is important in pediatric care, with our results showing age, weight, anesthesia time, and ASA classifications are predictors of opioid prescription. Further research examining contributors to patient pain following CLR is necessary to optimize opioid prescribing without negatively impacting the patient.

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.

Background The management and use of antibiotics in pediatric ear lacerations lacks standardization. This study sought to provide a comprehensive review of these injuries and assess the impact of administering perioperative antibiotics on the incidence of infections after repair. Methods Retrospective review of all pediatric ear lacerations treated at our institution's emergency department (ED) between 1998 and 2024. Results A total of 246 patients (177 boys) were included. Patients aged 0–6 years were most affected (n = 117, 47.6%), with the peak between 2–5 years old (n = 78, 31.7%). Most lacerations were superficial (n = 145, 58.9%), followed by intermediate (n = 62, 25.2%), full thickness (n = 34, 13.9%), and split earlobe defects (n = 5, 2%). Most of the patients (n = 110, 54.7%) were managed by emergency medicine providers. Plastic and reconstructive surgery (n = 34, 16.9%) and otorhinolaryngology (n = 32, 15.9%) consultations were obtained when the lacerations affected cartilage (P < 0.001). Surgical repair included primary closure in 203 (82.5%), with 179 (88.2%) performed under local/topical anesthesia in the ED. Of patients with cartilage lacerations (n = 61), 48 had cartilage repair. Eight patients had preoperative intravenous antibiotics, while 105 (42.7%) received postoperative prophylactic oral antibiotics. Among those with intermediate and full-thickness injuries (n = 96), 67.7% were discharged on prophylactic oral antibiotics. Only 1 case of surgical site infection occurred in a patient with a superficial laceration. Conclusions Pediatric ear lacerations mostly affect boys aged 2–5 years and are usually repaired under local anesthesia in the ED. The incidence of infection is low, and the routine prescription of prophylactic oral antibiotics appears to have a limited impact on the overall incidence of surgical site infection. Further studies are needed to validate these findings.