Combination of Cultured Epidermal Autograft and Meshed Skin Graft Enables Full-thickness Excision of Giant Congenital Nevus.

Giant congenital melanocytic nevus (GCMN) is a skin condition characterized by an abnormally dark, noncancerous skin patch. Two main issues with GCMN are aesthetics and malignant transformation. Various methods of treatment are reported, but each method has its own disadvantages, such as risk of recurrence or restriction in the treatable area. We report three cases of GCMN treated with full-thickness excision and immediately covered with cultured epidermal autograft (CEA) combined with split-thickness skin graft (STSG). This is a single-center, single-arm, retrospective report of three cases. The nevus was excised at full skin thickness. Meshed STSG taken from scalp was grafted to the defect, and CEA was grafted over simultaneously. Two weeks later, CEA was applied again as a booster. The same procedures were performed until all nevi were excised. In all cases, nearly complete epithelialization was achieved at several weeks after operation. The reconstructed skin was elastic, and there was no persistent joint contracture. Vancouver Scar Scale score was 4-8. Mesh-like appearance was observed. A hypertrophic scar appeared in the area without meshed STSG. An intractable keloid was observed in one patient. No recurrence of the nevus was observed during the follow-up period. The donor site scar on the scalp was well hidden by the hair. Our method enables full-thickness resection and reconstruction of a wider area in a single operation while improving the take rate of CEA, with reasonable degree of scarring compared with conventional methods.

Platelet changes and bleeding symptoms in children, adolescents, and adults with 22q11.2 deletion syndrome.

BACKGROUND: The deletion region of 22q11.2 deletion syndrome (22q11.2DS) contains a gene encoding glycoprotein Ibß (GPIbß), which is required to express the GPIb/IX/V complex on the platelet surface. Therefore, patients with 22q11.2DS may have congenital platelet disorders. However, information is limited on platelets and bleeding symptoms. In this study, we investigated clinical information, including bleeding symptoms, platelet counts, and GPIb expression levels in children and adolescents/adults with 22q11.2DS. PROCEDURE: Thirty-two patients with 22q11.2DS were enrolled in a prospective cohort study between 2022 and 2023 at outpatient clinics within our institute. RESULTS: The median platelet counts in adolescents/adults with 22q11.2DS were significantly lower than those in children (p < .0001). A gradual decrease was found along with increasing age (p = .0006). Values of median GPIb expression on platelet surfaces (66% in children and 70% in adolescents/adults) were significantly lower than those in healthy controls (p < .0001 and p = .0002). Bleeding symptoms included surgery-related bleeding (52%), purpura (31%), and epistaxis (22%); most of them were minor. The median International Society on Thrombosis and Hemostasis bleeding assessment tool score was not significantly different between children and adolescents/adults (p = .2311). CONCLUSION: Although there was an age-related decrease in platelet count and a disease-related decrease in GPIb expression, no difference in bleeding symptoms was found between children and adolescents/adults. 22q11.2DS overall had minor bleeding symptoms in daily life, and the disease had little effect on spontaneous bleeding. However, some patients had major bleeding events; further accumulation of data on hemostasis during surgery and trauma is required.

Geometric Morphometric Study on Distinguishing Metopic Craniosynostosis from Metopic Ridging.

Background: Craniosynostosis, a common congenital anomaly, results from premature fusion of the cranial sutures. One of the forms of craniosynostosis is premature fusion of the metopic suture, referred to as trigonocephaly, but the diagnosis of metopic suture synostosis remains controversial. The purpose of this study was to clarify, using geometric morphometric analysis, if a metopic ridge alone observed in cases of mild trigonocephaly represents a pathological phenomenon. Methods: Three different cranial morphologies were compared among patients up to 2 years old who were categorized into the true group, the mild group, and the normal group, based on the presence or absence of specific symptoms, history of cranioplasty for trigonocephaly, or lack of any abnormality on computed tomography. Using the obtained computed tomography images, 235 anatomical landmarks and semi-landmarks were plotted on the entire cranial surface for analysis of neurocranial morphology, and the cranial shapes represented by landmarks were analyzed using geometric morphometrics. Principal components of shape variations among specimens were then computed, based on the variance-covariance matrix of the Procrustes residuals of all specimens, and statistically analyzed. Results: The principal component analyses of the variations in endocranial shape, frontal bone shape, and occipital bone shape did not show any significant differences in cranial morphology between mild trigonocephaly and normal skulls; however, true trigonocephaly was found to differ significantly from mild trigonocephaly and normal skulls. Conclusions: These findings suggest that in assessments of cranial morphology, the presence of a ridge alone cannot be diagnosed as fundamentally pathological, and may represent normal morphology.

Modified Urethral Graciloplasty Cross-Innervated by the Pudendal Nerve for Postprostatectomy Urinary Incontinence: Cadaveric Simulation Surgery and a Clinical Case Report.

An artificial sphincter implanted in the bulbous urethra to treat severe postprostatectomy urinary incontinence is effective, but embedding-associated complications can occur. We assessed the feasibility, efficacy, and safety of urethral graciloplasty cross-innervated by the pudendal nerve. A simulation surgery on three male fresh cadavers was performed. Both ends of the gracilis muscle were isolated only on its vascular pedicle with proximal end of the obturator nerve severed and transferred to the perineum. We examined whether the gracilis muscle could be wrapped around the bulbous urethra and whether the obturator nerve was long enough to suture with the pudendal nerve. In addition, surgery was performed on a 71-year-old male patient with severe urinary incontinence. The postoperative 12-month outcomes were assessed using a 24-hour pad test and urodynamic study. In all cadaveric simulations, the gracilis muscles could be wrapped around the bulbous urethra in a γ-loop configuration. The length of the obturator nerve was sufficient for neurorrhaphy with the pudendal nerve. In the clinical case, the postoperative course was uneventful. The mean maximum urethral closure pressure and functional profile length increased from 40.7 to 70 cm H 2 O and from 40.1 to 45.3 mm, respectively. Although urinary incontinence was not completely cured, the patient was able to maintain urinary continence at night. Urethral graciloplasty cross-innervated by the pudendal nerve is effective in raising the urethral pressure and reducing urinary incontinence.

Necrotizing fasciitis following rapidly deteriorating neonatal omphalitis with good initial presentation.

Neonatal omphalitis is a postpartum infection of periumbilical superficial soft tissues that usually has a good prognosis in developed countries. In rare cases, it could progress to periumbilical necrotizing fasciitis (NF), which is an infection of the deep soft tissues, including muscle fascia, and has a high mortality rate. However, the signs and timing of developing NF secondary to omphalitis are unclear. We encountered a neonatal case of NF following omphalitis. In the initial days of the clinical course, general symptoms and condition of the patient were good, and abdominal physical findings were mild; however, the patient rapidly developed NF. The patient was successfully treated by emergent surgical debridement, broad-spectrum antibiotics, and intensive care. To determine the area of blood perfusion, we intravenously injected indocyanine green by intraoperative angiography, and then extensively removed necrotic and hypoperfused tissues. In neonatal omphalitis, the deterioration can suddenly occur despite good initial conditions; intensive monitoring should be required during the first few days of the clinical course.

Fate of Costochondral Grafts for Micrognathia: 3D Analysis of Postoperative Length, Position, and Graft Take.

PURPOSE: Costochondral grafting is performed in the reconstruction of severe micrognathia. In this study, we report our experience with graft take, as well as unique findings that have not been focused on in the past literature such as postoperative position and changes in the shape of the graft and thinning of the skull base in contact with the graft. METHODS: Between 2002 and 2019, costochondral grafting was performed on 15 sides of 10 patients with micrognathia. Graft take and other detailed findings such as growth, position, and changes in the shape of the grafts and skull base in contact with the grafts were evaluated using computed tomography images. RESULTS: Graft take was obtained in all cases, and subsequent distraction could be performed in 8 of 10 graft sites as planned. Six of 15 grafts were displaced anterior to the preoperative planned position. However, there were no cases with complications due to deviation such as trismus. Concave deformity of the skull base in contact with the graft was observed in 9 graft sites. The thinning and bending deformation were observed in 2 grafts. CONCLUSIONS: Costochondral grafting was considered a reasonable method for first-line treatment of severe micrognathia. Several unique findings that have not been focused on in the past literature were observed. These should be kept in mind when performing costochondral grafting. Several modifications to the present protocol are suggested to improve the quality of graft take.

Clinical Features of Bilambdoid and Sagittal Synostosis (BLSS): A Retrospective Multicenter Study in Japan.

ABSTRACT: Bilambdoid and sagittal synostosis (BLSS), a rare form of multisutural craniosynostosis, is sometimes known as the Mercedes-Benz syndrome due to the appearance of the fused sagittal and bilateral lambdoid sutures. Although previous studies have described some of its clinical features, the pathology of this disease is not yet fully understood. Moreover, it has been pointed out that BLSS is more common among individuals of Hispanic ethnicity, but its incidence in Asia remains unclear. In the present study, BLSS cases in Japan were analyzed to determine the characteristics of the condition in Japan. Three hospitals in Tokyo participated in the present study. Patients with BLSS who underwent cranial remodeling were included. Data on patient demographics, clinical symptoms, status of the cranial sutures, morphological subclassification, surgical procedures, developmental status, and genetic mutations were analyzed. In total, 22 patients met the enrollment criteria and were included, indicating a higher incidence of BLSS in Japan than in other nations reported in previous studies. In terms of morphological subclassification, there were 15 brachycephalic, 4 dolichocephalic, and 3 normocephalic. For the initial cranial procedure, 7 patients underwent a single-stage cranioplasty, 13 underwent a posterior distraction, and 2 underwent lateral expansion. Patients with a normocephalic cranial morphology tended to undergo surgery at an older age than patients with the other two types. Appropriate timing for surgery is important for healthy development; hence, surgeons should remember that BLSS can lead to "balanced dysmorphism" that may have led to a delay in diagnosis due to its normal-looking morphology.

Protocol for a multicentre, prospective, cohort study to investigate patient satisfaction and quality of life after immediate breast reconstruction in Japan: the SAQLA study.

INTRODUCTION: The aim of breast reconstruction (BR) is to improve patients' health-related quality of life (HRQOL). Therefore, measuring patient-reported outcomes (PROs) would clarify the value and impact of BR on a patient's life and thus would provide evidence-based information to help decision-making. The Satisfaction and Quality of Life After Immediate Breast Reconstruction study aimed to investigate satisfaction and HRQOL in Japanese patients with breast cancer who undergo immediate breast reconstruction (IBR). METHODS AND ANALYSIS: This ongoing prospective, observational multicentre study will assess 406 patients who had unilateral breast cancer and underwent mastectomy and IBR, and were recruited from April 2018 to July 2019. All participants were recruited from seven hospitals: Okayama University Hospital, Iwate Medical University Hospital, The Cancer Institute Hospital of Japanese Foundation for Cancer Research, Showa University Hospital, University of Tsukuba Hospital, Osaka University Hospital and Yokohama City University Medical Center. The patients will be followed up for 36 months postoperatively. The primary endpoint of this study will be the time-dependent changes in BREAST-Q satisfaction with breast subscale scores for 12 months after reconstructive surgery, which will be collected via an electronic PRO system. ETHICS AND DISSEMINATION: This study will be performed in accordance with the Ethical Guidelines for Medical and Health Research Involving Human Subjects published by Japan's Ministry of Education, Science and Technology and the Ministry of Health, Labour and Welfare, the modified Act on the Protection of Personal Information and the Declaration of Helsinki. This study protocol was approved by the institutional ethics committee at the Okayama University Graduate School of Medicine, Dentistry, on 2 February 2018 (1801-039) and all other participating sites. The findings of this trial will be submitted to an international peer-reviewed journal. TRIAL REGISTRATION NUMBER: UMIN000032177.

Evaluation of the Molding Helmet Therapy for Japanese Infants with Deformational Plagiocephaly.

INTRODUCTION: Deformational plagiocephaly (DP) is cranial flattening on one side of the back of the skull produced by an extrinsic force on the intrinsically normal skull. When the flattening is symmetrical, the deformity is called deformational brachycephaly (DB). In the US, its prevalence has increased since the "Back to Sleep" campaign by the American Association of Pediatrics. Helmet therapy is reported to be effective in improving head deformity by multiple studies, but there are few evidences from Japan. The purpose of this study is to investigate the safety and efficacy of helmet therapy for DP, and the feasibility of introducing this treatment to the clinical setting in Japan. METHODS: This was a single-arm, retrospective, nonrandomized study. Data were collected on infants who visited the "Clinic for Baby's Head Shape" in the National Center for Child Health and Development, Tokyo, Japan, between 2011 and 2014. Improvements in Argenta classification, cranial asymmetry (CA), and cranial vault asymmetry index (CVAI) were evaluated. The relationships between CA and influencing factors were evaluated using a linear mixed-effects model. RESULTS: Three hundred eighty-seven infants (273 boys and 114 girls; average age, 4.7 months) visited the clinic during the period, and 159 patients who completed the helmet therapy were analyzed. There were statistically significant improvements in Argenta classification, CA, and CVAI. Almost all of the parents reported increased sweating and mild skin irritation, but no adverse events necessitated the cessation of helmet therapy, except for one patient with increased sweating. CONCLUSIONS: Helmet therapy is safe and effective in treating DP and is feasible to introduce to the clinical setting in Japan. Through the distribution of knowledge regarding the etiology and treatment of head deformity, earlier detection and an evidence-based approach to head deformity are expected in the future.

Average Models and 3-dimensional Growth Patterns of the Healthy Infant Cranium.

Treatment of cranial deformity is often performed during infancy in cases such as craniosynostosis and deformational plagiocephaly. To acquire morphologic standards for the treatment goals of these conditions, we created cranial average models and elucidated the growth patterns of the cranium of healthy infants in 3-dimension (3D) using homologous modeling. METHODS: Homologous modeling is a technique that enables mathematical analysis of different 3D objects by converting the objects into homologous models that share the same number of vertices with the same spatial relationships. Craniofacial computed tomographic data of 120 healthy infants ranging in age from 1 to 17 months were collected. Based on the computed tomographic data, we created 120 homologous models. Six average 3D models (20 individuals each for 6 different age groups) were created by averaging the vertices of the models. Three-dimensional growth patterns of the cranium were clarified by comparing the 6 average models. RESULTS: We successfully created 6 average models and visualized the growth patterns of the cranium. From 1-month-old to 5-month-old infants, the entire cranium except for the occipital region grows, and the cranium tended to be brachycephalic (cephalic index at 4-5 months: 87.1-97.3), but the growth was thereafter localized to specific areas. CONCLUSIONS: Three-dimensional growth patterns of the cranium of healthy infants were clarified. These findings will support the understanding and treatment of the conditions that cause cranial deformity. To our knowledge, this is the first report to visualize the growth patterns of the entire cranium of healthy infants in 3D.

The Incidence of Sleep Disordered Breathing One Week After Primary Palatoplasty: Evaluation With Overnight Pulse Oximetry.

BACKGROUND: Sleep disordered breathing (SDB) is defined as a series of disorders including snoring, obstructive sleep apnea, and hypopnea. Few studies investigated the incidence of SDB following primary palatoplasty with objective testing. The aims of this study were to elucidate the prevalence and degree of SDB approximately 1 week following primary palatoplasty with objective testing and to clarify the risk factors. METHOD: A retrospective review was performed on children who underwent primary palatoplasty between April 2013 and July 2017 at National Center for Child Health and Development, Tokyo, Japan. As a national center, the authors accept many syndromic patients. The authors keep all patients after palatoplasty intubated and observe them overnight in intensive care unit to reduce the risks of respiratory events. Patients were evaluated with overnight pulse oximetry on 5 to 7 days postoperatively. RESULTS: Forty-four patients were included, and 30% of the patients were associated with congenital anomaly. Thirteen patients (30%) were diagnosed with SDB. None of the patients required additional treatment after the evaluation. Laryngomalacia and postoperative oxygen requirement significantly correlated with postoperative SDB. CONCLUSION: Approximately one-third of the patients may be at the risk of SDB 1 week after primary palatoplasty. Patients with history of laryngomalacia or those who required oxygen support for prolonged time after primary palatoplasty should be cared for significantly high risk of postoperative SDB.

Ecthyma gangrenosum in a 3-year-old boy post-heart transplantation.

Ecthyma gangrenosum (EG) is a serious bacterial infection in immunocompromised patients. EG in transplant recipients is rarely reported and may go unrecognized, which may delay initiation of appropriate treatment. We report a case of EG in a pediatric heart transplant recipient who was treated successfully with antibiotics and surgical debridement.

Analysis of Cranial Morphology of Healthy Infants Using Homologous Modeling.

OBJECTIVE: Data on cranial morphology of healthy individuals can be used as the guide in the treatment of cranial deformity. There are many reports analyzing the cranial morphology of healthy children in the past. But most of them focus on 2-dimensional values, and there are only a few reports, which analyzed the cranial morphology of Japanese healthy infants. We report a novel method that enables the comprehensive analysis of cranial morphology of Japanese healthy infants in 3D. METHODS: Craniofacial CT data of 20 healthy infants (9 males, 11 females) ranging in age from 1 to 11 months were collected. Based on the CT data, we created 20 homologous models of cranium using software specifically designed to support homologous modeling. We averaged vertex coordinates of the homologous models to create average model. We further performed principal component analysis, and created virtual models based on each principal component. The contribution rate was calculated, and the features described by each principal component were interpreted. RESULTS: We created the average cranial model of Japanese healthy infants. Seven principal components (cumulative contribution rate: 89.218%) were interpreted as to which part of the cranial shape each component was related to. The elements were extracted that may characterize the cranial morphology of some of the clinical conditions such as dolico/brachycephaly and deformational plagiocephaly. Some of these elements have not been mentioned in the past literature. CONCLUSION: Homologous modeling was considered to be valid and strong tool for comprehensive analysis of cranial morphology.

Reconstruction of the Urethral Sphincter with Dynamic Graciloplasty in a Male Rabbit Model.

INTRODUCTION: The use of artificial urinary sphincters can improve urinary incontinence after radical prostatectomies; however, complications can arise. We hypothesized that dynamic graciloplasty improves urethral sphincter reconstruction. MATERIALS AND METHODS: Models of urethral sphincter muscle reconstruction were developed in 5 adult rabbits by wrapping the gracilis muscle flap around the urethra. Intra-urethral pressure was measured in each of the models before reconstruction (control), after reconstruction, and after electrical stimulation of the flap in reconstructed models (stimulated models). RESULTS: The mean maximum urethral closure pressure was significantly greater in the reconstruction model (69.7 (66.5-115.8) mm Hg) than in the control model (39.2 (33.7-49.6) mm Hg). The mean integral of the urethral pressure and urethral length was also significantly greater in the reconstruction model than in the control model. Furthermore, sphincter tightening was enhanced by the electrical stimulation of the flap. CONCLUSIONS: Our results support our hypothesis that the functional reconstruction of urethral sphincters using muscle flaps is promising for the treatment of urinary incontinence.

Anatomic basis of anorectal reconstruction by dynamic graciloplasty with pudendal nerve anastomosis.

BACKGROUND: Dynamic graciloplasty has been proposed for anal reconstruction, but this method has 2 major drawbacks. First, an electrical device is required for control of the gracilis. The anastomosis with the pudendal nerve will provide more physiological control. Second, the limitation in the mobility of the muscle flap results in wrapping the anal canal with the muscle's distal portion, which is tendonlike and inelastic. Enhancing the mobility of the muscle flap will enable wrapping with the proximal, muscle-like, and extensible portion, possibly providing better sphincteric function. However, the basis for such an operative method is lacking. OBJECTIVE: The aim of this study is to provide the basis for the refined method of anal sphincter reconstruction by dynamic graciloplasty with pudendal nerve anastomosis and to verify the feasibility of lengthening the nerve to the gracilis muscle flap by dissecting into the muscle belly, detaching the gracilis muscle from its origin, and enhancing the mobility of the muscle flap. STUDY DESIGN: This is a retrospective, descriptive study. METHODS: The results from the anatomical study on 9 cadavers are reported. RESULTS: Tension-free anastomosis of the pudendal nerve and nerve to the gracilis was successfully performed in all the 9 cases: in 2 cases, by lengthening the nerve. The detachment of the muscle origin improved the mobility of the muscle flap, and the more proximal portion could be used for wrapping the anal canal, as confirmed in 4 cases. LIMITATIONS: The limited number of cases was a shortcoming of this study. CONCLUSIONS: By lengthening the nerve to the muscle, the gracilis can be used for anal sphincter reconstruction with pudendal nerve anastomosis, negating the need for an electrical device. By detaching the origin of the gracilis muscle, its proximal portion can be used to wrap the anal canal, possibly enabling a longer functional canal with stronger constricting force and better vascularity. These modifications to past methods may improve fecal continence after the operation.

Evaluation of maxillary sinus volume in cleft alveolus patients using 3-dimensional computed tomography.

OBJECTIVE: The aim of the study was to evaluate the volume of the maxillary sinus in patients with cleft alveolus. STUDY DESIGN: This is a retrospective, descriptive study. PATIENTS AND METHODS: The 3-dimensional computed tomographic data of 218 maxillary sinuses of 109 patients with cleft alveolus were compared with those of 100 sinuses of 50 healthy individuals. RESULTS: No significant difference in the maxillary sinus volume was found between the patients with cleft alveolus and the noncleft individuals. In the patients with cleft palate and alveolus, the maxillary sinus volume was significantly larger on the right side, but no significant difference was found between the cleft and noncleft sides. CONCLUSIONS: The volume of the maxillary sinus in the patients with cleft alveolus is not different from that of the noncleft individuals. The information about the maxillary sinus is clinically important in executing such operations as endoscopic sinus surgery.

QOL after head and neck reconstruction: evaluation of Japanese patients using SF-36 and GOHAI.

OBJECTIVE: To evaluate the health-related quality of life (HRQOL) of Japanese patients with head and neck cancer after treatment. STUDY DESIGN: Cross-sectional, descriptive study. PATIENTS AND METHODS: Twenty-nine patients who underwent resection and reconstruction of the head and neck cancer between September 2001 and January 2008 at the National Hospital Organization Tokyo Medical Center completed the Short Form 36 (generic QOL measure) and the General Oral Health Assessment Index (oral-specific QOL measure). RESULTS: The generic QOL of the patients was relatively maintained while oral-specific QOL was impaired compared to the Japanese norms. The patients with musculo-cutaneous flaps and 1y or longer after operation reported significantly lower QOL. CONCLUSION: This is a unique study on Japanese patients with relatively longer time after operation. Further evaluation with increased number of cases and disease-specific QOL scale is required to better understand the QOL of the patients.

Refined simple line closure for macrostomia repair: designing a mucosal triangular flap on the commissure region.

Congenital macrostomia is a relatively rare deformity. A number of different methods for its correction have been reported in the past. Here, we report our refined method of correcting macrostomia. Our method is characterized by creation of a small triangular mucosal flap on the cleft region of the lower red lip where the tissue appears identical to the normal commissure. Adequate advancement of this flap into the mouth along with proper reconstruction of muscle continuity and simple line closure of the skin enable correction of macrostomia with a natural-looking commissure in a symmetric position with an acceptable scar.

Advantages of open treatment for syndactyly of the foot: defining its indications.

For the repair of syndactyly of the foot, skin grafting is often used to close the skin defect, but open treatment is not common. However, with grafting, an additional scar at the donor site and patchwork-like scar at the recipient site are inevitable. Our aim was to describe the process of epithelialisation and define the indications for open treatment of syndactyly of the foot. The open treatment was used on 16 webs. The texture of epithelialised surface resembled volar skin; the visible scar was mainly at the dorsal edge; and web creep occurred predominantly on the volar side and resembled the natural slope of the commissure. Open treatment is better than skin grafting because of better match of texture without a patchwork-like scar, and it is indicated in cases of simple incomplete syndactyly of the foot that extends proximal to the distal interphalangeal joint.