Unexpected postoperative atlantoaxial rotatory subluxation after excision of melanocytic nevi of the head and neck in older children: two case reports and literature review.

Postoperative atlantoaxial rotatory subluxation (AARS) is a rare complication that develops almost exclusively in children following oropharyngeal and otologic surgeries, proposing that oropharyngeal inflammatory responses and excessive head rotation are responsible factors. However, there have been no reports of AARS after excision of a nevus on the head and neck. Here, we present two cases of AARS following limited head rotation during simple nevus excision. Patient 1, a 9-year-old girl, complained of neck pain and limited range of motion after excision of the nevus on the neck. After 2 months, computed tomography and magnetic resonance imaging finally revealed AARS with a ruptured transverse atlantal ligament. A month of halo traction was required for the treatment. Patient 2, an 11-year-old girl, presented with immediate pain and limited neck extension after tissue expander insertion under the upper chest and excision of the nevus on her left cheek. The diagnosis was promptly made using cervical spine radiography. A cervical collar was applied for 1 month. Both patients recovered without any complications after treatment. This report highlights the importance of suspicion for AARS after surgery regardless of surgical duration or amount of head rotation.

Preoperative Intrathecal Morphine is Associated With Reduced Postoperative Pain, Agitation, and Delirium In Living Donor Kidney Transplantation Recipients.

BACKGROUND: Postoperative delirium after organ transplantation can lead to increased length of hospital stay and mortality. Because pain is an important risk factor for delirium, perioperative analgesia with intrathecal morphine (ITM) may mitigate postoperative delirium development. We evaluated if ITM reduces postoperative delirium incidence in living donor kidney transplant (LDKT) recipients. METHODS: Two hundred ninety-six patients who received LDKT between 2014 and 2018 at our hospital were retrospectively analyzed. Recipients who received preoperative ITM (ITM group) were compared with those who did not (control group). The primary outcome was postoperative delirium based on the Confusion Assessment Method for Intensive Care Unit results during the first 4 postoperative days. RESULTS: Delirium occurred in 2.6% (4/154) and 7.0% (10/142) of the ITM and control groups, respectively. Multivariable analysis showed age (odds ratio [OR]: 1.07, 95% CI: 1.01-1.14; P = .031), recent smoking (OR: 7.87, 95% CI: 1.43-43.31; P = .018), preoperative psychotropics (OR: 23.01, 95% CI: 3.22-164.66; P = .002) were risk factors, whereas ITM was a protective factor (OR: 0.23, 95% CI: 0.06-0.89; P = .033). CONCLUSIONS: Preoperative ITM showed an independent association with reduced post-LDKT delirium. Further studies and the development of regional analgesia for delirium prevention may enhance the postoperative recovery of transplant recipients.

Thickness of melanocytes in giant congenital melanocytic nevus for complete surgical excision: clinicopathological evaluation of 117 lesions according to the area and size.

BACKGROUND: Giant congenital melanocytic nevi (GCMN) are usually defined as nevi that exceed 20 cm in maximal diameter or 15% of the total body surface area. There have been reports of life-long malignant change risks arising from GCMN, leading to surgical excision of GCMN. This study aims to evaluate the thickness of melanocytes based on clinical factors in order to provide objective information for the complete resection of the lesion. METHODS: Overall, 75 patients diagnosed with GCMN between 2000 and 2021 were included, and their clinical records were collected retrospectively. 117 pathologic slides obtained during excision were reviewed to measure nevus thickness. Clinical factors were assessed with a generalized estimated equation model for association with nevus thickness. RESULTS: The thickness of nevus was significantly associated with the location and size. Nevus thickness was more superficial in the distal extremity than in the head and trunk (P = 0.003 [head]; P < 0.001 [trunk]; P = 0.091 [Proximal extremity]). Nevi sized 60 cm or more were significantly deeper than those measuring 20-29.9 cm (P = 0.035). An interaction between size and location existed (P < 0.001). Trunk and distal extremity lesions consistently exhibited uniform thickness regardless of lesion size, whereas head and proximal extremity lesions showed variations in thickness based on lesion size. CONCLUSION: GCMNs have differences in thickness according to location and size. Therefore, it is necessary to devise an approach optimized for each patient to treat GCMN. In the study, it was emphasized that the thickness of GCMN is correlated with clinical factors, specifically the location and size of the nevus. Consequently, these findings underscore the need for individualized treatment plans for effective surgical intervention.

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.

A remote-controlled automatic chest compression device capable of moving compression position during CPR: A pilot study in a mannequin and a swine model of cardiac arrest.

BACKGROUND: Recently, we developed a chest compression device that can move the chest compression position without interruption during CPR and be remotely controlled to minimize rescuer exposure to infectious diseases. The purpose of this study was to compare its performance with conventional mechanical CPR device in a mannequin and a swine model of cardiac arrest. MATERIALS AND METHODS: A prototype of a remote-controlled automatic chest compression device (ROSCER) that can change the chest compression position without interruption during CPR was developed, and its performance was compared with LUCAS 3 in a mannequin and a swine model of cardiac arrest. In a swine model of cardiac arrest, 16 male pigs were randomly assigned into the two groups, ROSCER CPR (n = 8) and LUCAS 3 CPR (n = 8), respectively. During 5 minutes of CPR, hemodynamic parameters including aortic pressure, right atrial pressure, coronary perfusion pressure, common carotid blood flow, and end-tidal carbon dioxide partial pressure were measured. RESULTS: In the compression performance test using a mannequin, compression depth, compression time, decompression time, and plateau time were almost equal between ROSCER and LUCAS 3. In a swine model of cardiac arrest, coronary perfusion pressure showed no difference between the two groups (p = 0.409). Systolic aortic pressure and carotid blood flow were higher in the LUCAS 3 group than in the ROSCER group during 5 minutes of CPR (p < 0.001, p = 0.008, respectively). End-tidal CO2 level of the ROSCER group was initially lower than that of the LUCAS 3 group, but was higher over time (p = 0.022). A Kaplan-Meier survival analysis for ROSC also showed no difference between the two groups (p = 0.46). CONCLUSION: The prototype of a remote-controlled automated chest compression device can move the chest compression position without interruption during CPR. In a mannequin and a swine model of cardiac arrest, the device showed no inferior performance to a conventional mechanical CPR device.

Improving Facial Fat Graft Survival Using Stromal Vascular Fraction-Enriched Lipotransfer: A Multicenter Randomized Controlled Study.

BACKGROUND: Although previous clinical studies have reported that cell-assisted lipotransfer increases the fat survival rate in facial fat transplants, most were case studies without quantitative evaluation. A multicenter randomized controlled study was performed to evaluate the safety and efficacy of the stromal vascular fraction (SVF) in facial fat grafts. METHODS: Twenty-three participants were enrolled for autologous fat transfer in the face, and assigned randomly to the experimental ( n = 11) or control ( n = 12) group. Fat survival was assessed using magnetic resonance imaging at 6 and 24 weeks postoperatively. Subjective evaluations were performed by the patients and surgeons. To address safety concerns, results of an SVF culture and the postoperative complications were recorded. RESULTS: The overall fat survival rate was significantly higher in the experimental group than in the control group (6 weeks, 74.5% ± 9.99% versus 66.55% ± 13.77%, P < 0.025; 24 weeks, 71.27% ± 10.43% versus 61.98% ± 13.46%, P < 0.012). Specifically, graft survival in the forehead was 12.82% higher in the experimental group when compared with that in the control group at 6 weeks ( P < 0.023). Furthermore, graft survival in the forehead ( P < 0.021) and cheeks ( P < 0.035) was superior in the experimental group at 24 weeks. At 24 weeks, the aesthetic scores given by the surgeons were higher in the experimental group than in the control group ( P < 0.03); however, no significant intergroup differences were noted in the patient-evaluated scores. Neither bacterial growth from SVF cultures nor postoperative complications were noted. CONCLUSION: SVF enrichment for autologous fat grafting can be a safe and effective technique for increasing the fat retention rate. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, II.

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.

The Outcomes of Endoscopic Suturectomy in Syndromic Craniosynostosis.

OBJECTIVE: Endoscopic suturectomy is a widely practiced surgical option for infants with craniosynostosis. But the efficacy and safety of the procedure remain unclear in syndromic patients. This study aims to evaluate the efficacy and safety of endoscopic suturectomy for patients with syndromic craniosynostosis. METHODS: From January 2013 to December 2020, 242 patients underwent endoscopic suturectomy at our institution. The surgical outcome was determined to be favorable or unfavorable based upon the necessity of an additional cranial surgery upon the last follow-up. First, we analyzed the outcomes of 26 syndromic craniosynostosis patients who have followed up for over a year. Second, we compared the outcomes between the syndromic (N=12) and nonsyndromic (N=11) patients with bilateral coronal synostosis who have followed up for over a year. RESULTS: Twenty-three out of 26 syndromic craniosynostosis patients (88%) showed favorable outcomes without significant complications. In the analysis for bilateral coronal synostosis patients, 11 of 12 syndromic patients (92%) presented favorable outcomes, and all nonsyndromic patients showed favorable outcomes. No significant differences were observed in various anthropometric indices (cranial index, intracranial volume, anterior cranial height, anterior cranial base length, and cranial height-length index) and surgical outcomes between syndromic and nonsyndromic groups. CONCLUSIONS: Endoscopic suturectomy has the potential to be a surgical option for syndromic craniosynostosis. Even for patients with unfavorable outcomes, endoscopic suturectomy could serve as a bridge treatment for infants to counter cranial deformation before additional extensive surgery.

Aneurysmal bone cyst arising from the surgically removed craniofacial fibrous dysplasia in the long-term follow-up: a case report.

Fibrous dysplasia (FD) is a rare skeletal disorder characterized by abnormal fibro-osseous connective tissue replacing normal bone. Despite its benign behavior, craniofacial FD can cause morphological disfigurement, headache, and even blindness as a result of the produced mass effect. Surgical resection is recommended when the patient shows apparent clinical symptoms or aggravating facial asymmetry. Postoperative complications have been reported, such as hematoma, surgical site infection, abscess formation, resorption of the bone graft used for reconstruction, and recurrence. An aneurysmal bone cyst (ABC) is a rare benign bony lesion that can occur secondary to preexisting bone tumor. Secondary ABCs in craniofacial FD are extremely rare in the literature, accounting for less than 30, all of which are either case reports or series. We report an extremely rare case of symptomatic secondary ABC arising from craniofacial FD that had been misdiagnosed with abscess formation or recurrence and was surgically removed. Notably, 17 years elapsed between the primary surgery and the complication of secondary ABC. The patient underwent total removal of secondary ABC. After surgery, symptoms were relieved, with no recurrence observed during a 6-month follow-up.

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.

Impact of a selective lens dose reduction protocol in 3D rotational angiography on radiation exposure to the eye lens during cerebral angiography: a randomized controlled trial.

BACKGROUND: We aimed to investigate the radiation dose to the eye lens (lens dose) during cerebral angiography and to evaluate the effectiveness of the lens dose reduction protocol for 3-dimensional rotational angiography (3D-RA) in reducing overall lens dose exposure. METHODS: We conducted a randomized, controlled clinical trial at a tertiary hospital with patients undergoing cerebral angiography. The lens dose reduction protocol in 3D-RA involved raising the table to position the patient's eye lens away from the rotation axis. The lens dose was estimated by measuring the entrance surface air kerma using a photoluminescent glass dosimeter. The lens doses of 3D-RA, overall examination, and image quality were analyzed and compared between the two groups. RESULTS: A total of 20 participants (mean age, 58±9.4 years; including 12 men [60%]) were enrolled and randomly assigned to either the conventional group or the dose reduction group. The median lens dose in 3D-RA was significantly lower in the dose reduction group compared with the conventional group (1.1 mGy vs 4.5 mGy, p<0.001). The total dose was significantly lower in the dose reduction group (median of 7.5 mGy vs 10.2 mGy, p=0.003). In the conventional group, 3D-RA accounted for 46% of the total lens dose, while in the dose reduction group, its proportion decreased to 16%. No significant differences were observed in the image quality between the groups. CONCLUSION: The lens dose reduction protocol resulted in a significant reduction in the lens dose of the 3D-RA as well as entire cerebral angiography, while maintaining the image quality.

Joint semiparametric kernel network regression.

Variable selection and graphical modeling play essential roles in highly correlated and high-dimensional (HCHD) data analysis. Variable selection methods have been developed under both parametric and nonparametric model settings. However, variable selection for nonadditive, nonparametric regression with high-dimensional variables is challenging due to complications in modeling unknown dependence structures among HCHD variables. Gaussian graphical models are a popular and useful tool for investigating the conditional dependence between variables via estimating sparse precision matrices. For a given class of interest, the estimated precision matrices can be mapped onto networks for visualization. However, the limitation of Gaussian graphical models is that they are only applicable to discretized response variables and for the case when p log ( p ) ≪ n $$ p\log (p)\ll n $$ , where p $$ p $$ is the number of variables and n $$ n $$ is the sample size. They are necessary to develop a joint method for variable selection and graphical modeling. To the best of our knowledge, the methods for simultaneously selecting variable selection and estimating networks among variables in the semiparametric regression settings are quite limited. Hence, in this paper, we develop a joint semiparametric kernel network regression method to solve this limitation and to provide a connection between them. Our approach is a unified and integrated method that can simultaneously identify important variables and build a network among those variables. We developed our approach under a semiparametric kernel machine regression framework, which can allow for nonlinear or nonadditive associations and complicated interactions among the variables. The advantages of our approach are that it can (1) simultaneously select variables and build a network among HCHD variables under a regression setting; (2) model unknown and complicated interactions among the variables and estimate the network among these variables; (3) allow for any form of semiparametric model, including non-additive, nonparametric model; and (4) provide an interpretable network that considers important variables and a response variable. We demonstrate our approach using a simulation study and real application on genetic pathway-based analysis.

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.

Reduction of Radiation Dose to Eye Lens in Cerebral 3D Rotational Angiography Using Head Off-Centering by Table Height Adjustment: A Prospective Study.

OBJECTIVE: Three-dimensional rotational angiography (3D-RA) is increasingly used for the evaluation of intracranial aneurysms (IAs); however, radiation exposure to the lens is a concern. We investigated the effect of head off-centering by adjusting table height on the lens dose during 3D-RA and its feasibility in patient examination. MATERIALS AND METHODS: The effect of head off-centering during 3D-RA on the lens radiation dose at various table heights was investigated using a RANDO head phantom (Alderson Research Labs). We prospectively enrolled 20 patients (58.0 ± 9.4 years) with IAs who were scheduled to undergo bilateral 3D-RA. In all patients' 3D-RA, the lens dose-reduction protocol involving elevation of the examination table was applied to one internal carotid artery, and the conventional protocol was applied to the other. The lens dose was measured using photoluminescent glass dosimeters (GD-352M, AGC Techno Glass Co., LTD), and radiation dose metrics were compared between the two protocols. Image quality was quantitatively analyzed using source images for image noise, signal-to-noise ratio, and contrast-to-noise ratio. Additionally, three reviewers qualitatively assessed the image quality using a five-point Likert scale. RESULTS: The phantom study showed that the lens dose was reduced by an average of 38% per 1 cm increase in table height. In the patient study, the dose-reduction protocol (elevating the table height by an average of 2.3 cm) led to an 83% reduction in the median dose from 4.65 mGy to 0.79 mGy (P < 0.001). There were no significant differences between dose-reduction and conventional protocols in the kerma area product (7.34 vs. 7.40 Gy·cm², P = 0.892), air kerma (75.7 vs. 75.1 mGy, P = 0.872), and image quality. CONCLUSION: The lens radiation dose was significantly affected by table height adjustment during 3D-RA. Intentional head off-centering by elevation of the table is a simple and effective way to reduce the lens dose in clinical practice.

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.

Refinements in the pre-expanded distant flap for giant melanocytic nevi of the upper extremity in pediatric patients.

BACKGROUND: Surgical treatment of large and giant congenital melanocytic nevi (GCMN) of the upper extremity is challenging due to limited options for reconstruction. A pre-expanded distant flap is considered an important option in upper extremity reconstruction, where the soft tissue available for use is limited. This study aimed to refine the pre-expanded distant flap after excision of the GCMN in the upper extremity. METHODS: Large (>10 cm) and giant (>20 cm) congenital melanocytic nevi of the upper extremities treated with tissue expansion and distant flaps over 10 years were retrospectively reviewed, and the authors describe in detail the surgical strategies for reconstruction of the upper extremity with distant flap. RESULTS: From March 2010 to February 2020, 13 patients (mean age: 2.87 years) treated with 17 pre-extended distant flaps were included. The mean flap dimension was 154.87 cm 2, ranging from 15×5 cm to 26.5×11 cm. All surgeries were successfully completed except for one patient with partial flap necrosis. Preconditioning was performed before flap transfer in five patients with larger rotation arcs and flap dimensions. The mean duration of postoperative follow-up was 51.85 months. A new reconstructive protocol was proposed that combined the three elements, including a distant flap, tissue expander, and preconditioning. CONCLUSION: In the treatment of GCMN in the upper extremities, careful planning and multiple stages are required. For pediatric patients, the pre-extended distant flap with preconditioning is useful and effective for reconstruction.

The Effect of Epiphysiodesis on the Longitudinal Bone Growth of Hands or Feet in Children With Macrodactyly Based on Long-term Quantitative Analysis.

BACKGROUND: Treatment protocols for macrodactyly have not been elucidated due to its rarity and variety of clinical manifestations. This study aims to share our long-term clinical results of epiphysiodesis in children with macrodactyly. METHODS: A retrospective chart review was performed for 17 patients with isolated macrodactyly treated with epiphysiodesis over 20 years. Length and width of each phalanx in both the affected finger and the corresponding unaffected finger in the contralateral hand were measured. Results were presented in ratios of the affected to unaffected side for each phalanx. Measuring of length and width of phalanx was performed preoperatively and postoperatively at 6, 12, and 24 months, and the last follow-up session. Postoperative satisfaction scoring was done with visual analogue scale. RESULTS: The mean follow-up period was 7 years and 2 months. In the proximal phalanx, length ratio significantly decreased compared with preoperative state at after more than 24 months, in the middle phalanx after 6 months, in the distal phalanx after 12 months. When classified by the growth patterns, the progressive type showed significant decrease in length ratio at after 6 months, and the static type after 12 months. Patients were overall satisfied with the results. CONCLUSION: Epiphysiodesis effectively regulated longitudinal growth with different degree of control for different phalanges in the long-term follow-up.

Psammomatoid Juvenile Ossifying Fibroma of the Maxilla Misdiagnosed as Fibrous Dysplasia: A Clinicopathologic Case Report.

Juvenile ossifying fibroma (JOF) is a variant of the ossifying fibroma and includes two histopathological subtypes: trabecular and psammomatoid. Psammomatoid JOF (PJOF) in craniofacial structures should be distinguished from other fibro-osseous lesions, such as fibrous dysplasia (FD), considering the difference in the treatment protocols. Here, we present a rare case of PJOF that was initially misdiagnosed as a case of FD and emphasize the importance of considering JOF in the differential diagnosis of patients with craniofacial fibro-osseous lesions. A 4-year-old boy demonstrated progressive enlargement of the zygomaticomaxillary area on his left side for the last 6 months. The patient was diagnosed as a case of FD based on the clinical features and radiographic findings, and was operated considering the rapid progression. To achieve facial symmetry, contouring of the zygomatic bone and arch was performed. However, the patient demonstrated rapid enlargement at the 3-month postoperative follow-up. The decision was made to surgically remove the tumor due to visual field impairment. Intraoperatively, a rubbery mass, which was separated from the surrounding cortical bone, was identified and excised. The lesion was confirmed as PJOF by histopathological examination. The possibility of PJOF should not be ruled out in the differential diagnosis of patients with fibrous-osseous lesions. In the event of suspected PJOF, accurate diagnosis should be made through definitive biopsy.

Single Center Experience of Autologous Stem Cell Transplantation in Patients with Systemic Light Chain Amyloidosis in Korea.

BACKGROUND: Systemic light chains is the most common systemic amyloidosis. In patients with AL amyloidosis, the prognosis is influenced by the extent of organ damage, especially cardiac involvement. Autologous stem cell transplantation (ASCT) is a highly effective treatment for AL amyloidosis for selective patient METHODS: One hundred patients treated with ASCT for AL amyloidosis were reviewed in the Samsung Medical Center amyloidosis cohort. The cardiac, renal, and hematologic response was analyzed, and survival results compared based on organ involvement and hematologic response. RESULTS: The most common involved organ was kidney (n = 62) followed by heart (n = 50). The organ response rate was 44.0% and 37.1% in the patients with cardiac and renal involvement, respectively. In hematologic response, overall response rate (ORR) was 79.0%, including 48.0% complete response (CR). Median overall survival (OS) in patients with and without hematologic CR were not reached and 64.2 months (95% CI, 19.5 to 109.0), respectively (P < .001). The survival rate was not significantly different between patients with or without cardiac or renal involvement. Treatment-related mortality (TRM) in 30 days and 100 days was 2.0% and 3.0%, respectively. CONCLUSIONS: ASCT is an effective treatment option for eligible patients with AL amyloidosis. Achieving hematologic CR is essential for long-term survival.