Neurocognitive outcomes and associated clinical factors 5 years after surgery in children with craniosynostosis.

OBJECTIVE: Craniosynostosis involves early closure of one or more sutures, which is known to limit normal cranium growth and interfere with normal brain development. Various surgical methods are used, ranging from minimally invasive strip craniectomy to more extensive whole-vault cranioplasty. This study aimed to evaluate neurocognitive outcomes 5 years after surgical treatment in children with craniosynostosis and to evaluate relevant clinical factors. METHODS: After exclusion of genetically confirmed syndromic craniosynostosis patients, a retrospective review was conducted on 112 nonsyndromic craniosynostosis patients who underwent surgical treatment and follow-up neurocognitive assessment. Ninety-seven patients underwent strip craniectomy with postoperative orthotic helmet therapy, and 15 received other surgical treatment: 4 with distraction osteotomy and 11 with craniofacial reconstruction. Neurocognitive assessment using the Korean Wechsler Preschool and Primary Scale of Intelligence, Fourth Edition (K-WPPSI-IV), was performed 5 years postoperatively. Clinical factors were assessed regarding neurocognitive outcomes. RESULTS: The mean age at surgery was significantly younger in the strip craniectomy group (strip craniectomy 4.6 months vs other surgical treatment 18.6 months, p < 0.01). Patients with 2 or more sutures involved were more likely to receive more extensive surgical treatment (16.5% in the strip craniectomy group vs 53.8% in the other group, p < 0.01). Four (3.5%) patients who showed evidence of increased intracranial pressure (ICP) also underwent more extensive surgical treatment. Multivariable linear regression revealed a significant correlation of age at neurocognitive testing (-3.18, 95% CI -5.95 to -0.40, p = 0.02), increased ICP (-34.73, 95% CI -51.04 to -18.41, p < 0.01), and the level of maternal education (6.11, 95% CI 1.01-11.20, p = 0.02) with the Full-Scale Intelligence Quotient (FSIQ). Age at surgery, involvement of 2 or more sutures, and type of operation demonstrated no correlation with FSIQ. Among the 97 patients who underwent strip craniectomy, the FSIQ ranged from mean ± SD 100.2 ± 10.2 (bicoronal) to 110.1 ± 12.7 (lambdoid), and there was no significant difference between the suture groups (p = 0.41). The 5 index scores were all within average ranges based on their age norms. CONCLUSIONS: Age at neurocognitive assessment, increased ICP, and maternal education level showed significant correlations with the neurocognitive function of craniosynostosis patients. Although children with craniosynostosis exhibited favorable 5-year postoperative neurocognitive outcomes across various synostosis sutures, longer follow-up is needed to reveal the incidence of neurocognitive dysfunction in these patients.

Characterization of cranial growth patterns using craniometric parameters and best-fit logarithmic growth curves.

Few studies have reported a complete quantitative database of cranial growth, from infancy to adulthood, as a reference through three-dimensional analysis. Our study aimed to characterize cranial growth patterns using craniometric parameters by establishing sex- and age-specific norms. In total, 1009 Korean patients (male-to-female ratio, 2:1; age range, 0-18 years) who underwent thin-slice computed tomography (CT) scans for head trauma were divided into 20 age groups, with a 6-month interval for those under 2 years and a 1-year interval for those over 2 years. After four reference planes [Frankfurt horizontal (FH), midsagittal, and two coronal planes passing the sella (S) and basion (B)] had been established, intracranial volume (ICV), anteroposterior diameter (APD), biparietal diameter (BPD), cranial heights (CHs), cephalic index (CI, BPD/APD), and height index (HI, CH-B/APD) were measured using Mimics software. Best-fit logarithmic curves were derived using a linear regression model. The best-fit curves for ICV (cm3) were y = 785.6 + 157*ln(age) for males (R2 = 0.5752) and y = 702 + 150.5*ln(age) for females (R2 = 0.6517). After adjustment for age, males had higher values of ICV, APD, BPD, and CHs than females (all p < 0.0001). ICV, APD, BPD, and CHs demonstrated a rapid increase during the first few months of life, reaching 90-95% of the adult size by 5-6 years of age, while CI and HI showed a continuous decline by 4%, regardless of sex. This study presented cranial growth references for more than 1000 of the Korean population aged up to 18 years. This might help to provide guidelines for diagnosis and treatment (including timing, amount, and direction) for cranial reconstruction in pediatric patients with craniosynostosis.

Bilateral late hematomas after breast augmentation mimicking anaplastic large cell lymphoma: A case report.

RATIONALE: Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is rare, but its incidence has recently increased. It is characterized by a sudden onset of seroma collection after implantation of textured breast implants. However, BIA-ALCL may be confused with late hematoma, which is also a rare finding in aesthetic breast surgery. The cause of late hematoma is mostly unknown, and patients rarely present with specific symptoms. PATIENT CONCERNS: We presented a case of late hematoma that occurred in a patient who underwent augmentation mammoplasty 25 years ago and was on anticoagulants for 7 years. DIAGNOSES: Ultrasonography and magnetic resonance imaging could not rule out the possibility of BIA-ALCL. INTERVENTIONS: Bilateral implant removal was performed, and massive amounts of late hematoma and organizing tissues were removed. OUTCOMES: The pathologists confirmed the biopsy results as late hematoma with organizing tissues. Capsules from both sides were confirmed as fibrous capsules with chronic inflammation and foamy macrophage infiltration. LESSONS: Although malignancy needs to be primarily ruled out, late hematoma can occur beyond expectations, especially in anticoagulated patients, and must be included in the differential diagnosis.

Characterization of Spheno-occipital Synchondrosis Fusion From Preadolescents to Young Adults Using Age and Cervical Vertebrae Maturation Index.

The purpose of this study was to characterize the spheno-occipital synchondrosis fusion (SOSF) from preadolescents to young adults. A total of 630 Korean subjects (308 men, 322 women; age range, 6-18 y) were divided into 26 groups according to sex and age. After 3-dimensional computed tomography (CT) images were reoriented using the Frankfort horizontal (FH) plane, mid-sagittal plane, and frontal plane via ON3D software (3DONS), the cervical vertebrae maturation index (CVMI) and SOSF stages were identified using 6-stage and 5-stage scoring systems, respectively. The distributions of stage in each group were statistically investigated. Women showed early appearance and a short range of onset (CVMI stage 2, SOSF stage 2), middle (CVMI stage 4, SOSF stage 3 and stage 4), and completion (CVMI stage 6, SOSF stage 5), indicating rapid skeletal maturation compared with men. In both males and females, there were strong positive correlations between age and CVMI stage (rs=0.902, rs=0.890), between age and SOSF stage (rs=0.887, rs=0.885), and between CVMI and SOSF stages (rs=0.955, rs=0.964) (all P<0.001). The mean ages at SOSF stage 3 and stage 4 (12.7~13.9 y in males and 11.0~12.5 y in females) could be used as indicators of the pubertal growth peak. Regression equations for SOSF stage (y), age (a), and CVMI stage (b) were as follows: y=1.355-(0.133×a)+(0.29007×b)+(0.041×a×b) for males (r2=0.9496); y=1.305-(0.158×a)+(0.455×b)+(0.036×a×b) for females (r2=0.9606). Ordinal logistic regression analyses with the proportional odds model showed that females had more advanced SOSF stages than males (odds ratio: 1.972; 95% CI: 1.063-3.658, P<0.05). Our findings may provide basic references for CVMI and SOSF from preadolescents to young adults.

Deep Learning-Based Diagnostic System for Velopharyngeal Insufficiency Based on Videofluoroscopy in Patients With Repaired Cleft Palates.

Velopharyngeal insufficiency (VPI), which is the incomplete closure of the velopharyngeal valve during speech, is a typical poor outcome that should be evaluated after cleft palate repair. The interpretation of VPI considering both imaging analysis and perceptual evaluation is essential for further management. The authors retrospectively reviewed patients with repaired cleft palates who underwent assessment for velopharyngeal function, including both videofluoroscopic imaging and perceptual speech evaluation. The final diagnosis of VPI was made by plastic surgeons based on both assessment modalities. Deep learning techniques were applied for the diagnosis of VPI and compared with the human experts' diagnostic results of videofluoroscopic imaging. In addition, the results of the deep learning techniques were compared with a speech pathologist's diagnosis of perceptual evaluation to assess consistency with clinical symptoms. A total of 714 cases from January 2010 to June 2019 were reviewed. Six deep learning algorithms (VGGNet, ResNet, Xception, ResNext, DenseNet, and SENet) were trained using the obtained dataset. The area under the receiver operating characteristic curve of the algorithms ranged between 0.8758 and 0.9468 in the hold-out method and between 0.7992 and 0.8574 in the 5-fold cross-validation. Our findings demonstrated the deep learning algorithms performed comparable to experienced plastic surgeons in the diagnosis of VPI based on videofluoroscopic velopharyngeal imaging.

Spontaneous recovery of underlying bony erosion following surgical removal of craniofacial dermoid cysts in periatric patients: A prospective study.

This study aimed to evaluate the spontaneous recovery of bone deformity after surgical excision of craniofacial dermoid cysts in pediatrics. Pediatric patients who underwent excision of a dermoid cyst were included in the study. A prospective analysis was conducted to evaluate the amount of bone recovery by comparing the depth of bony concavity in the preoperative and postoperative (6 months) ultrasonography. In 145 of 187 patients with preoperative imaging available, the mean size of dermoid cysts was 1.4 cm3 (range, 0.1 to 9.5), and 41.4% (60/145 cases) showed cranial bone depression. In the comparison of preoperative and postoperative ultrasonography of 30 patients, the mean depth of bony cavity decreased significantly from 4.0 to 0.9 mm (p<0.001) after a mean of 6.7 months postoperatively. There was 13.3% (4/30) of mild (≤2.0 mm), 40.0% (12/30) of moderate (>2.0 to ≤4.0 mm), and 46.7% (14/30) of severe (>4.0 mm) depression, and the concavity depth significantly decreased in all groups (p = 0.028, mild; p<0.001, moderate; p<0.001 severe). Within the limitations of the study it seems that significant recovery of cranial bone depression does take place within 6 months after excision of craniofacial dermoid cysts in pediatric patients, saving the need for immediate reconstruction.

Catch-Up Growth Pattern in Cleft Palate: A Longitudinal Study from Infancy to Adolescence.

OBJECTIVE: To characterize the patterns of somatic catch-up growth from infancy to adolescence in patients with cleft palate (CP). STUDY DESIGN: We assessed 474 nonsyndromic patients with isolated cleft palate (n = 69) and unilateral and bilateral cleft lip and palate (n = 271; n = 134) who underwent palatoplasty between 1988 and 2017 and had longitudinal physical growth data at birth (T0), cheiloplasty (T1), palatoplasty (T2), childhood (T3), and adolescence (T4). The z scores of weight (ZWT), height (ZHT), and body mass index (ZBMI) were compared among the CP types (isolated cleft palate, unilateral cleft lip and palate, and bilateral cleft lip and palate) and time points (T1, T2, T3, and T4). Subgroup analyses were performed to investigate the growth of patients with malnourishment (z score < -1) at T1 or T2. A generalized linear model was used to investigate the effects of gestational age and cardiac anomalies on the longitudinal changes in ZHT and ZBMI. RESULTS: Regardless of the time point, the overall ZHT, ZWT, and ZBMI approximated 0 in all CP types, indicating few differences from the mean values of noncleft children. Significant catch-up growth occurred in ZHT and ZWT from T1 to T4 for all CP types (all P < .05). Despite the recovery of ZHT and ZBMI in most patients with malnourishment, these values remain relatively low until adolescence. Patients who were born at preterm stage or had surgically repaired cardiac anomalies grew well. CONCLUSIONS: Even in infants with CP and malnutrition, preterm birth, or cardiac anomalies, rapid catch-up growth can occur prior to palatoplasty with the help of comprehensive cleft care.

Multiple Injections of Adipose-Derived Stem Cells Improve Graft Survival in Human-to-Rat Skin Xenotransplantation through Immune Modulation.

BACKGROUND: Adipose-derived stem cells (ADSCs) exert immunomodulatory effects in the treatment of transplant rejection. This study aimed to evaluate the effects of ADSCs on the skin graft survival in a human-to-rat xenograft transplantation model and to compare single and multiple injections of ADSCs. METHODS: Full-thickness human skin xenografts were transplanted into the backs of Sprague-Dawley rats. The rats were injected subcutaneously on postoperative days 0, 3, and 5. The injections were as follows: triple injections of phosphate-buffered saline (PBS group), a single injection of ADSCs and double injections of PBS (ADSC × 1 group), and triple injections of ADSCs (ADSC × 3 group). The immunomodulatory effects of ADSCs on human skin xenografts were assessed. RESULTS: Triple injections of ADSCs considerably delayed cell-mediated xenograft rejection compared with the PBS and ADSC × 1 groups. The vascularization and collagen type 1-3 ratios in the ADSC × 3 group were significantly higher than those in the other groups. In addition, intragraft infiltration of CD3-, CD4-, CD8-, and CD68-positive cells was reduced in the ADSC × 3 group. Furthermore, in the ADSC × 3 group, the expression levels of proinflammatory cytokine interferon-gamma (IFN-γ) were decreased and immunosuppressive prostaglandin E synthase (PGES) was increased in the xenograft and lymph node samples. CONCLUSION: This study presented that triple injections of ADSCs appeared to be superior to a single injection in suppressing cell-mediated xenograft rejection. The immunomodulatory effects of ADSCs are associated with the downregulation of IFN-γ and upregulation of PGES in skin xenografts and lymph nodes.

Classification of Skeletal Phenotypes of Preadolescent Patients With Isolated Cleft Palate Using Principal Component Analysis and Cluster Analysis.

This study aimed to classify the skeletal phenotypes of preadolescent patients with isolated cleft palate using principal component analysis and cluster analysis. Sixty-four preadolescent female patients with isolated cleft palate (incomplete hard palate and complete soft palate cleft group, n=51; complete cleft of the hard and soft palate group, n=13; the mean age when lateral cephalograms were taken, 7.08±0.76 y) were included. Ten angular and 2 ratio cephalometric variables were measured on a lateral cephalogram. Cluster analysis was performed using 3 representative variables obtained from principal component analysis (SN-GoMe, SNA, and SNB). The differences in the variables among the clusters were characterized using the Kruskal-Wallis test. As a result of the analysis, 6 clusters were obtained from 3 groups: the retrusive maxilla and mandible group: cluster 3 (14.1%, moderately hyperdivergent pattern), cluster 5 (17.2%, severely hyperdivergent pattern); the normal maxilla and mandible group: cluster 1 (23.4%, normodivergent pattern), cluster 4 (12.5%, moderately hyperdivergent pattern), cluster 6 (20.3%, severely hyperdivergent pattern); the normal maxilla and protrusive mandible group: cluster 2 (12.5%, normodivergent pattern). The distribution of isolated cleft palate types did not differ among the 6 clusters ( P >0.05). Two thirds of the patients (68.7%, clusters 1, 2, 4, and 6) had a normal anteroposterior position of the maxilla, while one third of the patients (31.3%, clusters 3 and 5) showed a retrusive mandible. These results indicate that isolated cleft palate patients have diverse maxillo-mandibular growth patterns compared with patients with cleft lip and palate.

Classification of Skeletal Phenotypes of Adult Patients With Cleft Skeletal Class III Malocclusion Using Principal Component Analysis and Cluster Analysis.

The purpose of this study was to classify the skeletal phenotypes of adult patients with skeletal class III (C-III) malocclusion and unilateral or bilateral cleft lip and palate using principal component analysis and cluster analysis. The samples consisted of 81 adult C-III patients with cleft lip and palate (CLP) who underwent orthognathic surgery (OGS) or distraction osteogenesis (59 males and 22 females; 50 unilateral cleft lip and palate and 31 bilateral cleft lip and palate; mean age when lateral cephalograms were taken, 22.2±4.6 y). Thirteen angular and one ratio cephalometric variables were measured. Using 4 representative variables obtained from principal component analysis (SNA, SNB, Gonial angle, and Bjork sum), K-means cluster analysis was performed to classify the phenotypes. Then, statistical analysis was conducted to characterize the differences in the variables among the clusters. Five clusters were obtained from 3 groups: severely retrusive maxilla and moderately retrusive mandible group: cluster-1 (23.5%, severely hyperdivergent pattern), cluster-4 (27.2%, moderately hyperdivergent pattern), and cluster-5 (11.1%, normodivergent pattern); moderately retrusive maxilla and normal mandible group: cluster-2 (30.9%, normodivergent pattern); normal maxilla and moderately protrusive mandible group: cluster-3 (7.4%, normodivergent pattern). Although skeletal phenotypes were diverse, distribution of sex and cleft type did not differ among 5 clusters ( P >0.05). Sixty-two percent of cleft patients showed a severely retrusive maxilla and moderately retrusive mandible (cluster-1, cluster-4, and cluster-5), which indicated that these are the main cause of skeletal C-III malocclusion in CLP patients who were treated with OGS. Therefore, it is necessary to consider presurgical orthodontic treatment and surgical planning based on the skeletal phenotypes of CLP patients.

Distribution and Phenotype of Goldenhar Syndrome and Its Association With Other Anomalies.

The purpose of this study was to investigate the distribution and phenotype of Goldenhar syndrome (GS) and its association with other anomalies. The samples consisted of 18 GS patients (6 males and 12 females; mean age at investigation, 7.4 ± 4.8 y) who were treated or followed up at the Department of Orthodontics, Seoul National University Dental Hospital between 1999 and 2021. The prevalence of side involvement and degree of mandibular deformity (MD), midface anomalies, and association with other anomalies were evaluated using statistical analysis. The prevalence of unilateral and bilateral MD did not differ (55.6% versus 44.4%). In unilateral MD cases, there was a tendency for higher prevalence of more severe Pruzansky-Kaban types than mild ones (type I, 10%; type IIa, 10%; type IIb, 50%; type III, 30%). Despite hypoplasia of the condyle/ramus complex, compensatory mandibular body growth occurred in 33.3% of GS patients (more severe side in bilateral MD cases, 37.5%, and ipsilateral side in unilateral MD cases, 30%). Class II molar relation was more prevalent than class I and class III molar relations (72.2% versus 11.1% versus 16.7%, P <0.01). Al total of 38.9% of patients had congenitally missing tooth. #7 facial cleft was found in 44.4% of patients. In midface anomalies, ear problem was the most common anomaly, followed by hypoplasia/absence of zygomatic arch and eye problem (88.9% versus 64.3% versus 61.1%, P <0.01). Association with the midface, spine, cardiovascular, and limb anomalies did not differ between unilateral and bilateral MD cases. These results might provide a basic guideline for diagnosis and treatment planning for GS patients.

The Effectiveness of Releasing the Lower Lateral Cartilage Through Intranasal Z-plasty Incision on the Vestibular Web During Secondary Correction of Nasal Deformities in Complete Unilateral Cleft Lip and Palate.

The primary goal in the secondary correction of unilateral cleft lip nose deformity is to achieve symmetry of the nose and nostril. This study aimed to investigate the efficacy of freeing the lower lateral cartilage from the pyriform ligament through an intranasal Z-plasty incision on the vestibular web in adult patients with complete unilateral cleft lip and palate. Thirty-six patients with complete unilateral cleft lip and palate, who underwent open rhinoplasty between August 2014 and December 2021, were identified retrospectively. Five parameters for nose form and nostril symmetry were measured on basal views through 2-dimensional photographic analysis. The patients were divided into subgroups with or without septoplasty. Cleft-to-non-cleft ratios between the Z (13 patients) and non-Z groups (23 patients) were compared using the Mann-Whitney U test. The mean follow-up was 12.9 months (6-31 mo). In the Z group, there were significant differences between the preoperative and postoperative values for nostril angulation, regardless of septoplasty (all P <0.05). Despite septoplasty, significant differences in the postoperative changes in nostril angulation were found between the Z and non-Z groups (all P <0.05). Intranasal Z-plasty on the plica vestibularis is an effective technique for releasing the lower lateral cartilage, improving the nostril asymmetry in cleft lip nose deformity.

The Impact of Lateral Relaxing Incision on Middle Ear Function in Cleft Palate Repair.

Children with cleft palate are susceptible to otitis media with effusion. This study aimed to investigate the effect of lateral relaxing incision (RI) on middle ear function in cleft palate patients who underwent palatoplasty using double-opposing Z-plasty (DOZ). This is a retrospective study of patients who underwent bilateral ventilation tube insertion concurrently with DOZ, wherein RI was selectively performed on the right side of the palate (Rt-RI group) or not (No-RI group). The frequency of VTI, duration of the first ventilation tube retention, and hearing outcomes at the last follow-up were reviewed. Outcomes were compared using the χ 2 test and t test. A total of 126 treated ears from 63 non-syndromic children (18 male, 45 female) with cleft palate were reviewed. The mean age at surgery was 15.8±6.17 months. There were no significant differences in the frequency of ventilation tube insertion between the right and left ears within the Rt-RI group or between the Rt-RI and no-RI groups in the right ear. Subgroup analysis for ventilation tube retention time, auditory brainstem response thresholds, and air-conduction pure tone averages showed no significant differences. In the DOZ, the use of RI had no significant effects on middle ear outcomes during 3 years of follow-up. Relaxing incision seems to be safe without concern for middle ear function in children with cleft palate.

Facial Asymmetry Phenotypes in Adult Patients With Unilateral Cleft Lip and Palate and Skeletal Class III Malocclusion Using Principal Component Analysis and Cluster Analysis.

The purpose of this study was to classify and characterize facial asymmetry (FA) phenotypes in adult patients with unilateral cleft lip and palate (UCLP) and skeletal class III malocclusion. The samples comprised 52 adult UCLP patients (36 men and 16 women; mean age, 22.43 y) who had undergone orthognathic surgery for correction of class III malocclusion. After measurement of 22 cephalometric parameters in posteroanterior cephalograms taken 1 month before orthognathic surgery, principal component analysis was performed to obtain 5 representative parameters [deviation (mm) of ANS (ANS-dev), maxillary central incisor contact point (Mx1-dev), and menton (Me-dev); cant (degree) of the maxillary anterior occlusal plane (MxAntOP-cant) and mandibular border (MnBorder-cant)]. K-means cluster analysis was conducted using these representative parameters. The differences in cephalometric parameters among the clusters were statistically analyzed. The FA phenotypes were classified into 4 types: No-cant-and-No-deviation type (cluster-4, n=16, 30.8%); MxMn-cant-MxMn-dev to the cleft-side type (cluster-3, n=4, 7.7%); Mx-cant-Mn-shift to the cleft-side type (cluster-2, n=15, 28.8%); and Mn-cant-Mn-dev to the noncleft-side type (cluster-1, n=17, 32.7%). Asymmetry in the maxilla and/or mandible were observed in 70% of patients. One third of patients (cluster-2 and cluster-3; sum, 36.5%) exhibited significant cant of MxAntOP induced by cleft and cant or shift of the mandible to the cleft side. Another one third of patients (cluster-1, 32.7%) demonstrated significant deviation and cant of the mandible to the noncleft-side despite cleft in the maxilla. This FA phenotype classification might be a basic guideline for diagnosis and treatment planning for UCLP patients.

Is There a Relationship between Palatal Asymmetry and Speech in Children with Isolated Cleft Palate after Double-Opposing Z-Plasty?

BACKGROUND: Although some cleft palates show asymmetric palatal shelf length and/or width intraoperatively, the relationship between palate asymmetry and speech outcomes has not been fully investigated. METHODS: This study retrospectively reviewed 234 patients who underwent double-opposing Z-plasty (DOZ) for isolated cleft palate (Veau class I and II). Speech outcomes were analyzed to evaluate the association with width and length discrepancy of the palatal shelves using multiple logistic regression adjusting for patient age. RESULTS: The mean age at repair was 14.2 ± 5.26 months. The mean palatal shelf width and length differences were 0.87 ± 0.97 mm and 1.63 ± 1.61 mm, respectively. The mean age at initial and follow-up assessment was 37.6 ± 5.70 months ( n = 234) and 66.2 ± 8.81 months ( n = 120), respectively. Multivariate logistic regression analysis of initial speech outcomes showed odds ratios in width discrepancy of 1.67 ( P = 0.0703), 1.59 ( P = 0.0104), and 2.01 ( P = 0.0051) for nasal emission, hypernasality, and compensatory articulation, respectively. Additional analysis including follow-up outcomes also revealed that width discrepancy of the palatal shelves had higher odds ratios for nasal emission, hypernasality, and compensatory articulation (OR, 1.49, P = 0.0406; OR, 1.36; P = 0.0660; and OR, 1.65; P = 0.0170, respectively). There was no association between length discrepancy and all speech abnormalities. CONCLUSIONS: Greater discrepancies in palatal shelf width, rather than in length, were associated with poorer speech outcomes after DOZ. The authors suggest that DOZ is effective for longitudinally asymmetric cleft palates. CLIINCAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

Single Surgeon's Experience in Repair of Median Cleft Lip: A Case Series of 5 Median Craniofacial Dysplasia Patients.

Median craniofacial dysplasia is a rare congenital anomaly with a broad spectrum of severity, which can be classified as hypoplasia, dysraphia, and hyperplasia, depending on the involved tissue amount. A retrospective chart review was performed of patients with median craniofacial dysplasia who underwent repair of the upper lip median cleft between January 2013 and February 2020. The median cleft of the upper lip was present in 5 cases. The average age at operation was 11 months. Two patients had a median notch in the vermilion, 2 patients had an incomplete median cleft lip, and 1 patient had a complete median cleft lip with the absence of columella, prolabium, and premaxilla. A variety of surgical correction was performed for each case, including simple rhombus-shaped excision, modified version of straight-line repair, and columella reconstruction using an intranasal dorsal flap and bilateral cleft margin flaps. Each case needs to be carefully assessed with individualization for appropriate surgical treatment.

Long-Term Effect of Intermediate Rhinoplasty in Unilateral Complete Cleft Lip Nasal Deformity.

BACKGROUND: Intermediate rhinoplasties are performed during preschool to reduce the patients' psychosocial burden. At our institution, limited dissection of the cartilages followed by suspension and interdomal sutures was performed through an alar rim incision on the cleft side to minimize the risk of iatrogenic nasal growth restriction. However, the long-term outcomes of "limited intermediate rhinoplasty" through skeletal growth are uncertain. MATERIALS AND METHODS: A retrospective review of all unilateral complete cleft lip and/or palate patients who underwent definitive rhinoplasty was performed. To avoid the confounding effect of primary rhinoplasty, only the patients who did not receive primary rhinoplasty were included in the analysis. The maneuvers performed during definitive rhinoplasty were analyzed and compared between patients who underwent intermediate rhinoplasty and those who did not. RESULTS: A total of 60 Korean patients (27 female and 33 male) underwent definitive rhinoplasty at the average age of 20.6 years old (17.1-25.5). Forty-three (71.6%) patients previously underwent intermediate rhinoplasty. A combination of 6 maneuvers was performed based on the deformity of each subunit (alar medialization, interdomal with suspension sutures, nostril sill depression correction, septoplasty, osteotomy, and hump rasping). The average number of maneuvers performed during definitive rhinoplasty was significantly higher in the intermediate group (3.31 versus 2.1, P=0.012). Alar medialization and nostril sill depression correction were more frequently performed in the intermediate group, while the frequencies of other maneuvers were not statistically different. CONCLUSION: While intermediate rhinoplasty improves the patients' psychosocial well-being, the effects of "limited intermediate rhinoplasty" manipulating only the cartilages do not seem to last until skeletal maturity. A more comprehensive dissection allowing the release of the lower lateral cartilage in the hinge area along with septoplasty may be more effective in providing longer-lasting effects.

Early cranial vault distraction for a more balanced and enhanced expansion: a 3D craniometric analysis of anterior versus posterior distraction osteogenesis in patients with craniosynostosis.

OBJECTIVE: Posterior distraction osteogenesis (DO) is widely accepted for the treatment of craniosynostosis. The aim of this study was to quantitatively compare the effect of DO on the cranial vault according to the age of the patient and direction of distraction. METHODS: This was a retrospective study of patients with craniosynostosis who underwent DO in the anteroposterior direction. Postdistraction changes in intracranial volume (ICV), anteroposterior distance, biparietal distance, cranial height, and frontal bossing angle were measured using Mimics software on CT scans. Craniometric data were analyzed using a multivariate regression model. RESULTS: Thirty-two patients (16 anterior and 16 posterior DOs) were included in the study. The mean ICV increase in the anterior and posterior DO group was 211 cm3 (range 142-281 cm3) and 214 cm3 (range 150-279 cm3), respectively. Patients who were aged 1 year or younger showed a greater percentage increase in ICV than patients older than 1 year. In the anterior DO group, a more balanced increase in both anterior and posterior anteroposterior distance was observed in patients aged 1 year or younger when compared to patients older than 1 year. In the posterior DO group, a bigger expansion and smoother contour in the posterior cranial fossa was observed in patients aged 1 year or younger. CONCLUSIONS: Both anterior and posterior DO are effective surgical options for expanding the cranial vault in patients with craniosynostosis. Early distraction appeared to show greater morphological changes in the growing cranial vault than those predicted with the vector of distraction.

Comparison of safety outcomes between bi-coronal and direct approaches for device removal in patients treated with distraction osteogenesis for craniosynostosis.

The study aimed at comparing the safety outcomes between conventional bicoronal and direct method in device removal after cranial distraction in the treatment of craniosynostosis. This was a retrospective cohort study of patients treated with distraction osteogenesis to expand intracranial volume in the anteroposterior direction. Preoperative patient demographics, distraction protocols, and perioperative outcomes (operative time, estimated blood loss, intraoperative fluid volume, lowest hematocrit during surgery, transfusion, hospital stay, drain, postoperative complication) were collected and analyzed with the independent samples t-test. Twenty-four patients were included in the study (15 in the conventional and 9 in the direct approach group). The mean duration of surgery and intraoperative fluid volume were significantly shorter (114.56 ± 36.91 min vs. 177.20 ± 47.00 min, p = 0.003) and less (241.88 ± 194.07 ml vs. 624.00 ± 524.92 ml, p = 0.026), respectively in the direct group than in the conventional. No patients were transfused intraoperatively and admitted to the intensive care unit (ICU) postoperatively in the direct group. The mean intraoperative and total transfusion volumes were significantly greater in the conventional group (p = 0.004, 0.045, respectively). There were no significant differences in other safety outcomes. No postoperative wound complications were reported. The findings of this study indicated that the direct approach for distraction device removal in children with craniosynostosis appears to be safer than the conventional approach owing to the reduced operation time, intraoperative transfusion requirements, and ICU stay.

Effect of Early Spheno-Occipital Synchondrosis Fusion in Preadolescent Patients With Syndromic Craniosynostosis on Craniofacial Skeletal Patterns: A Preliminary Study Using Cephalometric Analysis.

ABSTRACT: The purpose of this study was to investigate the effects of early spheno-occipital synchondrosis (SOS) fusion in preadolescent patients with syndromic craniosynostosis (SC) on the craniofacial skeletal patterns. Twenty preadolescent SC patients were divided into the fused SOS (FS, n = 10; 8 Crouzon and 2 Apert) and not-fused SOS groups (NFS, n = 10; 9 Crouzon and 1 Apert). Lateral cephalograms (mean age: 9.60 years, cervical vertebral maturation index: stage I and II) were used to investigate the skeletal sagittal (ANB) and vertical patterns (SN-GoMe), upward inclination of the anterior cranial base (ACB; SN-FH), degree of midface hypoplasia (MH, SNA), retrusive position of orbitale (SNO), and forward position of the condyle in relation to sella (saddle angle). Using the ordinal values calculated by ethnic norm (criteria: moderate, over ±1 standard deviation, severe, over ±2 standard deviation), statistical analysis was performed. The FS group showed a higher percentage of severe MH than the NFS group (70% versus 10%, P < 0.05). Although the 2 groups did not differ in the distribution of ANB, SN-GoMe, saddle angle, and SN-FH (all P > 0.05), the FS group showed relatively higher percentages of severe Class III (100% versus 70%), severe hyper-divergent pattern (40% versus 10%), severely forward condyle position (30% versus 0%), and moderate and severe upward anterior cranial base inclination (90% versus 50%) than the NFS group. However, the 2 groups exhibited the same distribution of moderately and severely retrusive orbitale position ([50%, 20%], P > 0.05). Early SOS fusion in preadolescent SC patients might not be related to retrusive orbitale position, but to severe MH.