Published papers

Limited data exist with regard to the outcome of the Nuss procedure for pectus excavatum repair in adults. Here, we analysed changes in lung capacity and thoracic morphology based on computed tomography (CT) imaging in adults with pectus excavatum before surgery, during bar insertion and after bar removal. Patients who underwent the Nuss procedure for pectus excavatum after the age of 20 were included in this study. Chest CT scans of the included participants were taken before the Nuss procedure, immediately before removal of the pectus bar and 6 months after removal of the pectus bar. Lung capacity and thoracic morphology measurements were made from the CT scans. Six patients aged 24–43 years were included in this study. After the Nuss procedure, lung capacity was decreased in all patients. Although the pectus bar was removed, lung capacity had not significantly increased and was almost the same volume as before the Nuss procedure. After the Nuss procedure, the funnel chest shape had improved in all cases, patients’ thoracic spine had also moved forward as the thorax moved forward and patients’ stoop had improved. Despite a lack of change in lung capacity, surgical modification should be considered to reduce stress on the thoracic spine and in turn, reduce pain in patients with pectus excavatum. However, further long-term observation seems necessary.

The reconstruction of combined orbital floor and medial wall fractures is challenging due to the surgical approach and implant stability. A 0.5 mm thickness rigidity custom made titanium alley implant was created by 3-dimensional printing with electron-beam additive manufacturing. The implant was designed to cover the entire surface of the inferomedial orbit based on the flipped the contralateral orbit images. Although the cost was expensive, the use of three-dimensional printed implants for extensive orbital wall fractures was useful for decreasing operative time, and can be accessed with a limited surgical approach with a precise fit.

The inframammary fold defines the shape and structure of the breast, especially in women. As the inframammary fold is placed between the fifth and sixth ribs, the ligaments or fascia are thought to attach from these ribs. However, the previous literature on what structures constitute the inframammary fold does not provide sufficient knowledge for reconstructing the natural form of the fold. This study aimed to clarify the structure that involves the inframammary fold. Ten sides of five formalin-fixed, adult Asian cadaveric breasts were studied. Upon dissection of the breast, including the ribs, the fat lobules were removed while preserving the septal structures under the microscope. The fascial structures were observed grossly and radiographically. A multilayered fascial structure was noted from the dermis near the inframammary fold, anchored to the deep fascia of the pectoralis major muscle mainly at the height of the fourth rib and partially of the fifth rib, from the outside of the nipple to the linea axillaris media, where the fold could be clearly observed. Additionally, the fat lobules around the inframammary fold were subdivided by thin septa closer to the dermis, and they fused posteriorly and upward to form this fascial structure. The inframammary fold was not formed by an adhesion directly under the sixth rib, but by the skin “hanging” from the height of the fourth and fifth ribs due to the multilayered fascial structure that repeatedly fused and dissociated and the changes in the size of the fat lobules. This new anatomical finding may help in inframammary fold reconstruction.

Mastering the techniques of craniofacial surgery is difficult owing to the small number of cases and the special skills required. To improve the outcomes of craniofacial surgery in Japan, the authors charted the progress of a craniofacial surgeon by reviewing the results for the 100 consecutive craniosynostosis cases, in which the authors performed fronto-orbital advancement (FOA). in this study, all FOA surgeries were performed by the first author beginning in 2012. The number of FOA surgeries previously performed by the surgeon at the time of the current surgery served as an indicator of his level of experience; the cases were grouped in 5 consecutive sets of 20. The preparation time before the operation, the operative time, and the amount of bleeding were retrospectively investigated. The operative and preparation times decreased as the surgeon's experience increased until a plateau was reached after 41 to 60 surgeries. Increases in operative time also corresponded to decreases in the amount of bleeding. Everyone has a learning curve, including surgeons performing craniofacial surgeries. Surgery is not performed by the surgeon alone. Reductions in preparation time, operative time, and the time needed to leave the operation room after the completion of the surgery were attributed to better collaborations with nurses and anesthesiologists. Hence, the growth of the team is very important to the success of the craniofacial surgeon and ensures safe and effective treatment of the patient.

Background and purpose: Teaching cleft repair in the operating room may not sufficiently prepare craniofacial residents to perform the procedure self-reliantly. This study aimed to assess the influence of surgeons’ experience level on the operative time and graft survival ratio after alveolar bone grafts for cleft repair. Subjects and methods: This was a retrospective review of 100 consecutive alveolar bone graft patients treated for unilateral alveolar cleft by one craniofacial surgeon between 2012 and 2019. An alveolar bone graft from the iliac bone was performed using the lateral sliding flap. Sex, age, cleft width, cleft defect volume, and operative time were recorded, and the 1-year postoperative graft survival ratio was calculated. Multiple regression analysis was performed to identify factors that affect the operative time and survival ratio. Results: Factors affecting the operative time were the experience level of the surgeon and the presence of a cleft palate. Factors affecting the graft survival ratio were the cleft defect volume and experience level of the surgeon. In cleft lip and alveolus, the survival ratio was constant, and the operative time stabilised after approximately 20 cases. In cleft lip and palate, more experience was required to master the technique, and the operative time and survival ratio were stabilised only after approximately 40 cases. Conclusion: Surgeons’ experience level influenced the outcomes of alveolar bone grafting. Although surgical training is currently undergoing a paradigm shift from on-the-job training to simulation-based training, clinical experience is critical to be self-sufficient.

Monobloc advancement by distraction osteogenesis is the treatment of choice in patients with syndromic craniosynostosis. This procedure is usually performed at 18 to 24 months/5 to 10 years of age. Herein, we present the case of a male patient with Crouzon syndrome who underwent monobloc advancement at the age of 62 years. Although the patient lived a normal life (employed, married, and being a father of a daughter), he visited our hospital for surgical improvement in facial esthetics. The patient underwent monobloc advancement by distraction osteogenesis. He was satisfied with the postoperative esthetic improvement and did not experience any major complications. This case highlights the fact that patients with syndromic craniosynostosis desire esthetic improvement and suggests that multidisciplinary treatment involving both the neuro and plastic surgeons is important in such cases.

Abstract Non-syndromic craniosynostosis (NSCS) is a disease, in which a single cranial bone suture is prematurely fused. The early intervention of the disease is associated with a favorable outcome at a later age, so appropriate screening of NSCS is essential for its clinical management. The present study aims to develop a classification and detection system of NSCS using skull X-ray images and a convolutional neural network (CNN) deep learning framework. A total of 56 NSCS cases (scaphocephaly [n = 17], trigonocephaly [n = 28], anterior plagiocephaly [n = 8], and posterior plagiocephaly [n = 3]) and 25 healthy control infants were included in the study. All the cases underwent skull X-rays and computed tomography scan for diagnosis in our institution. The lateral views obtained from the patients were retrospectively examined using a CNN framework. Our CNN model classified the 4 NSCS types and control with high accuracy (100%). All the cases were correctly classified. The proposed CNN model may offer a safe and high-sensitivity screening of NSCS and facilitate early diagnosis of the disease and better neurocognitive outcome for patients.

Several studies have analyzed the long-term stability of cranioplasty and midface distraction in patients with craniosynostosis; however, few studies have investigated long-term quality of life (QOL) and complications in adults with syndromic craniosynostosis. This study aimed to investigate the QOL (social, physical, and psychosocial) of patients with adult syndromic craniosynostosis. Patients aged ≥20 years with syndromic craniosynostosis, who were surgically treated at a single craniofacial institution, were included in this study. We investigated everyday inconvenience (using the World Health Organization Disability Assessment Schedule questionnaire), any ongoing treatment, marital status, and number of children. Totally, 18 patients aged 22–48 years (mean: 31.4 ± 9.2 years) answered the questionnaire (Crouzon syndrome, 9; Apert syndrome, 5; Pfeiffer syndrome, 4). Of these, only one Crouzon syndrome patient was married; she was also the only one with a child. Apert syndrome patients were found to have difficulty in understanding, communication, and self-care because of their mental retardation and hand and foot handicaps; however, their participation in society was the most aggressive. In contrast, Crouzon syndrome patients had especially poor participation in society. In all patients, any ongoing hospital treatment was due to ophthalmological conditions. Crouzon syndrome patients have extremely poor QOL; the absence of mental retardation and hand and foot handicaps forces them to live in mainstream society, for which they are emotionally ill-equipped. It is necessary to treat these patients without any residual deformity to provide psychological support and to create an accepting society.

Background: It is generally accepted that the alar base on the cleft side in the cleft lip nose is displaced outward and downward; therefore, it is rotated inward and upward in almost all procedures for cleft lip closure. However, nostril narrowing and collapse of the lower lateral cartilage on the cleft side are sometimes experienced. In this retrospective study, we investigated whether the preoperative alar base on the cleft side is displaced outward and downward. Methods: This was a retrospective evaluation using preoperative frontal images obtained from patients with unilateral cleft lip (n = 245). The midcolumellar and subalare points were placed, and each ratio was analyzed and compared to those of age-matched controls (n = 40). Results: The subalare on the noncleft side and subnasale were displaced upward and outward. In contrast, the subalare on the cleft side was displaced downward and inward. The displacement of the subalare was greater on the noncleft than on the cleft side. There were no significant differences between male and female patients and between the left and right sides. Among the complete cleft lip and palate, incomplete cleft lip and palate, complete cleft lip and alveolus, incomplete cleft lip and alveolus, and microform cleft lip groups, the complete cleft lip and palate group showed the greatest displacement. Conclusion: The cleft alar base appeared to shift outward and downward because of an optical illusion, but in fact, both alar bases shifted; the alar base on the cleft side was displaced inward and downward, and the alar base on the noncleft side was displaced outward and upward.

Background: Bioabsorbable systems have been commonly used in pediatric patients for primary cranioplasty and other related surgeries. However, subcutaneous swelling, a unique complication related to bioabsorbable osteosynthesis, is a concern. Differences in the incidence of subcutaneous swelling, depending on the bioabsorbable material used to construct the plate, are still unknown. Methods: The authors retrospectively reviewed all incidences of subcutaneous swelling related to resorbable systems used during primary cranioplasty for patients with craniosynostosis at their hospital between 2014 and 2018 during a 12-month follow-up period. Furthermore, the authors reviewed all published English-language articles (since 1995) on subcutaneous swelling in bioabsorbable systems used for craniosynostosis. Results: The most common resorbable systems used in the literature were divided into 2 groups: mixtures of poly D-lactic acid and polyglycolic acid, and mixtures of poly D- and L-lactic acid. In patients for whom poly D-lactic acid and polyglycolic acid were used, the incidence of subcutaneous swelling during resorption was 0% to 4.2% between 3 and 9 months of follow-up. In patients for whom poly D- and L-lactic acid was used, subcutaneous swelling during resorption occurred in 5% to 16.7% of these patients between 6 and 12 months of follow-up. All cases resolved spontaneously after complete absorption of the plate. Conclusions: It was difficult to determine which system had the lowest incidence of subcutaneous swelling. The thickness of each resorbable system and the thickness of the infants’ scalps were different in each study. However, subcutaneous swelling occurred in every resorbable system. Therefore, preoperative counseling and careful follow-up are necessary.

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.

Patients with craniofacial microsomia often require multiple surgical interventions to address both hard and soft tissue defects. For improvement of soft tissue defects, microvascular free tissue transfers have been widely performed after puberty. To camouflage facial asymmetry, early fat grafting was performed on five six-month-old patients, and acceptable outcomes were obtained without overcorrection. This result suggests that early fat grafting in craniofacial microsomia is useful to camouflage asymmetrical facial contours.

The causes of visual impairment following Le Fort osteotomy for syndromic craniosynostosis have not been completely elucidated. The authors investigated the potential causes and means of prevention of optic nerve damage, with particular emphasis on intraoperative blood transfusion volume and operating time. This retrospective study evaluated patients who underwent Le Fort III osteotomy for syndromic craniosynostosis between 2000 and 2020. Data on pupillary reflex, pupil size, operating time, blood transfusion, age at time of surgery, sex, and syndrome type were obtained from medical records. Univariate analysis and multivariate analysis with the level of statistical significance set at P<0.05. For the 86 patients included, the mean values of operating time, amount of blood transfusion based on body weight, amount of blood transfusion per body weight per hour, and age were 6.0 hours (range: 3.5-12.3 h), 30.5 mL/kg (range: 0-322 mL/kg), 5.14 mL/kg/h (range: 0-35.7 mL/kg/h), and 10.0 years (range: 4-38 y), respectively. Crouzon, Apert, and Pfeiffer syndromes were observed in 49, 29, and 8 patients, respectively. Abnormal pupillary findings were observed in 27 patients of whom 25 showed no abnormalities in subsequent visual function and 2 developed blindness. Abnormal pupillary findings correlated with the amount of blood transfused per body weight (P=0.0082) and amount of blood transfused per body weight per hour (P=0.0052). As demonstrated in this study, increased intraoperative bleeding and amount of blood transfused were associated with optic nerve damage, particularly during acute bleeding. Prompt inspection of the pupils following surgery is therefore warranted.

Background: Gorham-Stout disease (GSD) is a rare and idiopathic bone disorder, characterized by massive osteolysis. To date, there is no established treatment strategy for GSD. We empirically treated two patients, who had presented to us with cranial lesions of GSD. Here, we propose a novel algorithm for the management of Gorham’s disease based on our experience and review the literature published to date. Methods: We reviewed all existing literature on GSD describing the pathophysiology and suggested treatment methods, up to 2018. Results: We found 13 papers with 14 reported cases; an inclusion of our two cases brings the total count up to just 16 recorded cases of GSD involving the skull. Of these, the base of the skull was affected in eight cases, while the remaining eight cases showed cranial involvement. The patients with skull-base involvement were managed conservatively, using medications or radiotherapy. The patients with cranial osteolysis were managed surgically, with an excision of the osteolytic portion, followed by cranioplasty. Of the latter group, the pericranium was not removed in one patient, in whom a very slight progression of the osteolytic process was later observed. Conclusions: The pathogenesis of GSD remains poorly understood. Further study is required to determine an optimum management strategy. A long-term follow-up will also be necessary to establish the effectiveness of the treatment process. The untreated patients show a progressive resorption of the affected bones of the skull. A painful, vanishing skull deformity is an alarming sign of GSD. Early diagnosis and treatment are necessary to arrest disease progression and to prevent complications.

Although patients with cleft lip and palate often present with poor maxillary growth because of intrinsic and iatrogenic factors, the surgical influence of lip revision surgery, palatal fistula repair, and pharyngeal flap procedures remains uncertain in contrast to that of primary cleft lip repair and palatoplasty. Therefore, this study aimed to reveal factors inhibiting maxillary growth and inducing later orthognathic surgery. A retrospective analysis was conducted on the data of patients with cleft lip and palate who underwent a series of treatments at Keio University Hospital from 1990 to 2000. We collected data on patient sex, cleft type, number and timing of lip revision surgery, the incidence of palatal fistulae, history of pharyngeal flap procedures, and timing of a repeat bone graft, and reviewed whether these patients underwent orthognathic surgery later in life. Multivariate analysis was conducted using binary logistic regression to extract factors affecting later orthognathic surgery. A total of 52 patients were included in this study. Results showed that revision surgery conducted more than twice was the highest statistically significant predictor of later orthognathic surgery in patients with a cleft lip and palate (P<0.05, odds ratio=43.3), followed by palatal fistula occurrence after cleft palate repair (P<0.05, odds ratio=22.3). Therefore, primary surgical procedure is most important for these patients.

© 2019, American Cleft Palate-Craniofacial Association. Purpose: To evaluate the utility and efficacy of bioabsorbable hydroxyapatite and collagen complex (HA/Col) for secondary bone grafting in unilateral alveolar clefts. Patients and Methods: Between 2015 and 2016, 21 patients with unilateral cleft lips and alveolar clefts were enrolled. In group I, a cancellous iliac bone graft was placed at the alveolar cleft (6 males, 5 females). In group II, a bioabsorbable HA/Col was placed at the alveolar cleft (4 males, 7 females). Results: The groups did not differ in age, cleft volume, or surgical duration. There was a significant difference in intraoperative blood loss between the 2 groups (6.7 ± 1.89 mL in group II vs 38.8 ± 9.73 mL in group I [P <.01]). The use of patient-controlled intravenous analgesia was also significantly lower in group II than in group I (2.2 ± 1.9 times vs 12.2 ± 4.4 times [P <.01]). Only 1 female in group II who had maxillary sinusitis did not achieve osteosynthesis. With the exception of this patient, the 12-month bone volumes in groups I and II were 0.567 ± 0.066 and 0.596 ± 0.073 mL, respectively, without significant difference (P =.18). Conclusion: Compared with an autogenous bone, bioabsorbable HA/Col significantly reduces postoperative pain. In addition, the use of HA/Col alone produced the same result as an autologous bone and is effective in filling the alveolar cleft.

© 2019 Elsevier Ltd Several resorbable fixation systems are used for osteosynthesis in craniofacial surgery. Recently, ultrasonic-assisted pinned resorbable systems have been introduced; however, few studies have described the associated complications during the long-term follow-up until complete resorption. In this study, we investigated the complications of craniofacial surgery using the ultrasonic-assisted pinned resorbable system with a follow-up of at least 30 months. Among patients who underwent craniofacial surgery using a commercially available ultrasonic-assisted pinned resorbable system between 2014 and 2016, those with follow-up visits for at least 30 months were included in this study. We investigated the development of complications such as local infection, exposure of the device, and reoperation related to the implant. Twenty-four patients aged 6 months to 69.4 years (median: 3.5 years) were followed up for more than 30 months. None of the patients required reoperation regardless of implants. Further, no infection or device exposure was seen among all patients. However, two patients aged 6 and 22 months who underwent cranioplasty for craniosynostosis and another patient aged 148 months who underwent cranioplasty for cranial defect exhibited plate-related bulging in the scalp during the course of resorption between 7 and 12 months of follow-up. The bulges were characterized by swelling without pain or redness and resolved spontaneously within 18 months of follow-up, which was considered to occur after complete absorption of the plate. In conclusion, subcutaneous swelling is related to resorbable plates and has a benign clinical course. We recommend that patients be informed of this phenomenon preoperatively to relieve their anxiety.

© 2018 American Cleft Palate-Craniofacial Association. Craniofrontonasal syndrome (CFNS) is a very rare genetic disorder, the common physical malformations of which include coronal synostosis, widely spaced eyes, clefting of the nasal tip, and various skeletal anomalies. Mutations of EFNB1, which encodes a member of the ephrin family of transmembrane ligands for Eph receptor tyrosine kinases, is the cause of CFNS. Although familial CFNS cases have been reported, no studies in the literature describe familial cases of CFNS expressing bilateral cleft lip and palate. Here, we describe a Japanese family with three cases of CFNS expressing bilateral cleft lip and palate.

Copyright © 2017 by Mutaz B. Habal, MD. Osteomas are slow-growing, benign bony tumors. Most are asymptomatic. Paranasal sinus osteoma in the pediatric population is rare; hence, there are few reports of symptomatic osteomas. The authors report the case of a giant ethmoid sinus osteoma in an 11-year-old girl who presented with diplopia and proptosis. Total excision and reconstruction of the medial orbital wall were performed via a coronal approach. The postoperative period was uneventful, the child’s vision improved, and a good cosmetic result was obtained.