Citations

Objective This study aimed to compare longitudinal cephalometric growth, speech outcomes, and secondary surgeries after 1-stage versus 2-stage palatoplasty in patients with non-syndromic unilateral cleft lip and palate (UCLP). Design Retrospective single-institution cohort study. Setting Tertiary academic craniofacial center. Patients/Participants Sixty-eight patients with non-syndromic UCLP treated between 1992 and 2015 who underwent primary palatal repair and had long-term clinical and radiographic follow-up. Forty-eight underwent 1-stage palatoplasty, and 20 underwent 2-stage palatoplasty. Interventions One-stage palatoplasty versus staged palatoplasty with initial soft palate repair followed by delayed hard palate closure. Main Outcome Measures Longitudinal cephalometric measurements at approximately 10 and 16 years, speech outcomes at 5, 10, 13, and 16 years, oronasal fistula, velopharyngeal insufficiency surgery, and need for continued speech follow-up after age 10. Results Mean follow-up was 15.2 ± 4.4 years. Linear fixed-effects modeling showed significant age-related worsening in sagittal skeletal relationships in both groups, including decreases in sella-nasion-A point angle (SNA), A point-nasion-B point angle (ANB), and Wits appraisal, without significant effects of palatoplasty strategy or age-by-strategy interaction for key sagittal outcomes. Speech outcomes were comparable between groups across all timepoints, including hypernasality, nasal emission, compensatory articulation, intelligibility, and Cleft Audit Protocol for Speech-Augmented (CAPS-A) scores. Rates of secondary velopharyngeal surgery and continued speech follow-up after age 10 did not differ significantly. Oronasal fistula occurred more frequently after 2-stage palatoplasty than 1-stage palatoplasty (30% vs 9%, P = .02). Conclusion One-stage and 2-stage palatoplasty demonstrated similar long-term growth and speech outcomes through adolescence. Two-stage repair was associated with a higher oronasal fistula rate but not greater secondary velopharyngeal or speech burden.

Background: Polyetheretherketone (PEEK) is a commonly used material for cranioplasties due to its advantages over other materials and autologous bone grafts. PEEK has an increased malleability, which increases postoperative aesthetics and adapts well to the cranial contours; it is also associated with a lower rate of infection when compared with autologous cranioplasties. Despite these benefits, there is limited information on the practical aspects of modifying PEEK cranioplasties after their initial placement. Case Series: We present 2 cases of patients who had previously undergone PEEK cranioplasty being treated with deep-brain stimulation (DBS), which was placed through their PEEK implant. The first was an 18-year-old male who had previously undergone cranial vault closure with bone grafts following a craniotomy. Later, when a deep-brain stimulator was planned, it was discovered that the bone grafts had significant resorption and a lack of structural integrity. Therefore, bone grafts were replaced with a custom PEEK implant cranioplasty before the DBS electrodes were placed via drilling through the PEEK implant to access the brain at a later surgery. The second patient was a 39-year-old male who developed epilepsy after a stroke, requiring craniotomy. DBS was unable to be performed initially due to significant resorption of the patient’s native bone. Therefore, a PEEK cranioplasty was performed in preparation for a staged, subsequent placement of DBS electrodes through the implant. Both patients recovered uneventfully following surgery, with noticeable improvement in functional (reduction in seizure frequency) and aesthetic outcomes. Systematic Review: A literature review was conducted using PubMed with keywords related to PEEK cranioplasties. After screening titles, abstracts, and full-text papers, it was found that 3 previously documented cases have involved drilling through PEEK implants: one by Mallela et al, involving SEEG electrode placement; one by Marrone et al, concerning a burr hole for evacuating a chronic subdural hematoma; and one by Ledesma et al, regarding the placement of subdural grid electrodes. Conclusions: This case series highlights how PEEK cranioplasties can be drilled through for therapeutic procedures, such as DBS electrode implantation, without compromising their structure. These cases illustrate the versatility, safety, and adaptability of PEEK implants for additional necessary interventions.

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.

Although complications of autologous bone flap cranioplasty are well described, data on long-term bone flap survival and the comorbidities associated with secondary reconstruction are limited. This study aimed to quantify the need for secondary alloplastic cranioplasty within a standardized patient cohort. Patients who underwent delayed cranioplasty following craniectomy with banked, frozen autologous bone between 2012 and 2025 were identified and analyzed using Cox regression, spline analysis, and Kaplan–Meier survival analysis. A total of 367 patients with an average age of 39.2±19.7 years (range: 0.3–84.4 years) at the time of cranioplasty were included. A secondary cranioplasty was required in 13.6% of patients (n=50 of 367), with resorption (52%) and infection (42%) as the most common indications. In adjusted analyses, patients who underwent cranioplasty following a traumatic injury (HR: 2.37; 95% CI: 1.07–5.26; P =0.03) and patients younger than 18 years (HR: 2.99; 95% CI: 1.35–6.62; P =0.01) were more likely to require a secondary cranioplasty due to bone flap resorption. Increasing defect size was also associated with a higher risk of secondary cranioplasty due to bone flap resorption, becoming significant at roughly 95 cm² (HR: 2.54; 95% CI: 1.04–6.20; P =0.04). The estimated cumulative incidence of secondary cranioplasty was 37% at ten years after autologous reconstruction. These results indicate that although autologous bone flap cranioplasty is largely effective, select patients face a substantially higher risk of secondary cranioplasty, and alternative reconstructive options may be warranted.

Biparietal osteodystrophy, also known as bilateral parietal calvarium bone thinning, is a rare calvarial condition characterized by loss of diploic bone within the parietal region. Although historically described in anthropologic and radiologic literature, current understanding of its epidemiology, pathogenesis, clinical relevance, and management remains fragmented. This review synthesizes current evidence and highlights emerging studies on biparietal osteodystrophy. Epidemiologic reports indicate that biparietal osteodystrophy affects a broad age range but appears most frequently in older adults, with several series suggesting a slight female predominance. Prevalence is difficult to estimate due to incidental detection, though modern imaging has increased recognition of the condition. Histologically and anatomically, biparietal osteodystrophy is marked by thinning of the outer table and loss of the diploic space, with little osteoblastic or osteoclastic remodeling. Proposed pathogenic mechanisms include chronic degenerative change, impaired vascular supply to the diploë, and other theories, although no single explanation fully accounts for its complete presentation. Clinically, most patients remain asymptomatic, with complications such as calvarial fractures and traumatic brain injury being rare but highlighting the structural vulnerability associated with severe thinning. Management strategies are not standardized, and the use of anti-osteoporotic drugs and cranioplasty has been reported to offer benefit in select cases, although no treatment has been formally validated for biparietal osteodystrophy. As the understanding of biparietal osteodystrophy continues to evolve, future research is needed to clarify its natural history, identify potential etiologic pathways, and establish evidence-based management guidelines.

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 We sought to characterize factors associated with postoperative complications in patients undergoing primary cleft lip repair (CLR). Design The National Surgical Quality Improvement Program-Pediatric database was queried to identify patients under 12 months old undergoing CLR using CPT codes. Multivariable analysis was conducted to characterize complications and associated comorbidities. Setting A multicenter sample. Patients/Participants A total of 11 957 cases (81.9% unilateral CLR and 18.1% bilateral CLR) were identified. Interventions None. Main Outcomes/Measures The postoperative outcomes of significance were surgical site infection (SSI), wound dehiscence, and unplanned reintubation within 30 days of CLR. Results The cohort had a mean age of 4.7 ± 2.0 months and a median American Society of Anesthesiologists classification of 2. SSIs occurred in 0.6% of cases and were independently associated with bilateral cleft anatomy (OR 2.16, P = .003) and longer anesthesia time (OR per minute: 1.004, P = .009). Wound dehiscence occurred in 0.4% of patients and was independently associated with bilateral cleft anatomy (OR 4.03, P < .001) but was associated with shorter anesthesia duration (OR per minute: 0.991, P < .001). Unplanned reintubation occurred in 0.2% of cases, with these patients having significantly higher rates of multiple comorbidities. Conclusion Wound dehiscence, SSI, and unplanned reintubation were rare complications following CLR. Patients with a bilateral cleft lip had increased odds of both SSI and wound dehiscence.

Introduction: Congenital preauricular lesions, including skin tags, accessory tragi, and pits, have long been associated with renal anomalies due to their shared embryologic origin. Despite the common practice of recommending routine renal ultrasound screening for these patients, the necessity of this protocol has not been conclusively validated. Methods: A retrospective review was conducted at a single institution from 1996 to 2025. A total of 109 patients presenting with congenital preauricular lesions were identified. Data extracted included demographics, comorbidities, syndromic status, hearing assessments, external ear abnormalities, and renal imaging findings. Statistical analyses (chi-square, Fisher's exact test, and one-sample proportion z-tests) were employed to evaluate the prevalence of renal anomalies compared to the general population and explore associations with other clinical factors. Results: Of the 109 patients with congenital preauricular lesions, 69 underwent renal imaging, and 34.8% (24/69) were found to have abnormal findings, significantly higher ( P = .0011) than the estimated 1.5% prevalence among the general US population. Hydronephrosis was the most frequent abnormality, while no significant differences emerged across syndromic versus nonsyndromic patients, external ear anomalies, or documented hearing loss. Conclusion: These findings indicate a higher-than-expected rate of renal pathology in patients with congenital preauricular lesions and support a low threshold for imaging regardless of syndromic status or other risk factors. Prospective research is needed to further refine screening recommendations.

Objective This study aimed to identify specific causes, clinical features, and contributing factors associated with early childhood mortality in a vulnerable subset of patients with cleft lip and/or palate (CL/P) who did not survive to undergo surgical repair. Design This was a retrospective chart review study. Setting The study was conducted at a single tertiary medical center. Patients/Participants We identified 26 liveborn CL/P patients who died without cleft repair from 1970 to 2024. Their data was compared to a control group of 2070 surgically repaired CL/P patients from the same institution. Main Outcome Measures Our primary goal was to characterize the clinical profile of patients experiencing prerepair mortality. Secondary outcomes included identifying causes of death, maternal and prenatal risk factors, and comparing these findings with the surgically repaired patients. Results All 26 unrepaired patients (100%) had genetic syndromes or isolated severe congenital anomalies, a significant difference compared to 24% in the surgically repaired group ( P < .0001). Trisomy 13 (30.8%), holoprosencephaly (23.1%), congenital cardiac defects (11.5%), and trisomy 18 (11.5%) were the leading causes of death. The median age at death for unrepaired patients was 25 days (interquartile range 4-125 days), significantly lower than the 61.9 years in the control group ( P < .0001). Multivariable logistic regression showed that genetic syndromes or severe congenital anomalies were strongly associated with early mortality ( P < .0001). Notably, all unrepaired patients who were born in the United States after 2005 had accurate prenatal diagnoses, which allowed clinical planning before the patients were born. Conclusions Prerepair mortality in CL/P is significantly driven by underlying genetic syndromes or congenital anomalies. For surgeons, this highlights the importance of genetic testing and prenatal care to guide appropriate surgical management. Considering a wait in surgical intervention until patients are approximately 4 months old may allow for better survival assessment and informed decisions for these high-risk individuals.

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.