Potential of Three-Dimensional Portable Document Format for Article Publication in Craniofacial Surgery.

The portable document format (PDF) is a globally recognized standard file format of Word documents. The PDF supports the embedding of three-dimensional objects. This three-dimensional PDF can contribute to new ways of presenting and disseminating medical information in future medical journals.

Pitfall of Monobloc Advancement in Beare-Stevenson Syndrome.

Beare-Stevenson syndrome (BSS) is an extremely rare form of craniosynostosis. Half of patients with BSS died within the first year of life due to cardiorespiratory arrest or unexpected sudden death. In addition, there have only been fewer than 30 cases, which limits prognostication for clinicians. We currently have an 8-year-old patient who is being followed up. She underwent tracheostomy, cranioplasty, and shunting. After waiting for growth, for improvement of ocular herniation, monobloc advancement was performed at the age of 5 years. The temporal fossa was quite narrow due to severe midface hypoplasia. In addition, even though midface advancement, it was difficult to remove tracheostomy. This was because midfacial hypoplasia was so severe that it was hard to notice, but there was also micrognathia. We report our experience with this midface treatment.

Is Postoperative Helmet Molding Therapy Necessary for Craniectomy for Sagittal Suture Synostosis?

Endoscopic strip craniectomy followed by postoperative remodeling helmet therapy for scaphocephaly is widely accepted, but the requirement of several helmets might burden patients or their families. This study examined whether craniectomy via a bicoronal approach without molding helmet therapy is worth considering. The authors retrospectively reviewed patients with nonsyndromic sagittal suture synostosis who underwent the modified Renier H technique between 2016 and 2021. We collected data on preoperative and postoperative cephalic index (CI), operative time, estimated blood loss, and blood transfusion rates. The authors also examined all related literature in English describing endoscopic craniectomy with postoperative helmet molding therapy for sagittal suture craniosynostosis. Among 17 patients, the average operative time was 92.2±15.6 minutes, the mean estimated blood loss was 35.8±22.4 mL, and a blood transfusion was needed for 9 patients (52.9%). The mean preoperative and postoperative CIs were 69.1±2.6 and 78.4±2.2, respectively. A comparison of the literature review with the authors' results showed that their procedures required a slightly longer operative time and a higher frequency of blood transfusions. There are no significant differences in blood loss or preoperative and postoperative CIs reported in the literature. The open procedure required a higher operative time than endoscopic suturectomy. However, dealing with bleeding is more challenging with endoscopic suturectomy. The cost of the helmets and the duration patients have to wear them, which might be burdensome for the patients or their families when determining the optimal minimally invasive treatment, should also be considered.

Hemostatic Efficacy of Oxidized Regenerated Cellulose Powder in Le Fort 1 Osteotomy.

A challenging aspect of Le Fort I osteotomy is bleeding control. Osteotomy techniques, devices, drugs, and anesthetic management have been reported to reduce bleeding; however, there are no reports on the use of hemostatic agents. We aimed to evaluate the hemostatic efficacy of a new topical absorbent hemostatic agent, Surgicel Powder, consisting of oxidized regenerated cellulose (ORC). We reviewed the records of 40 patients who underwent Le Fort I surgery for jaw deformities, with or without cleft lip and palate. Twenty of the 40 patients did not have cleft lips or cleft palates (CLCP); the remaining 20 had CLCP. In each group, an absorbent hemostatic agent was used in 10 patients but not in the other 10. Total blood loss and operative time for each group were evaluated. In the jaw deformity without CLCP group, the amount of bleeding with or without ORC was 112.0±33.8 and 158.6±75.3 mL, respectively, with a significant difference between groups ( P <0.05). Operative time with or without ORC was 206.4±31.3 and 238.3±42.5 minutes, respectively, with a significant difference observed between groups ( P <0.05). In the jaw deformity with CLCP group, the amount of bleeding with or without ORC was 199.7±64.6 and 476.8±104.8 mL, respectively, with a significant difference between groups ( P <0.05). Operative time with or without ORC was 213.7±27.6 and 220.8±41.5 minutes, respectively, with no significant difference between groups ( P =0.329). In conclusion, oxidized regenerated cellulose powder may be a beneficial hemostatic agent for reducing blood loss during Le Fort I osteotomy.

The Long-Term Natural History of Fibrous Dysplasia.

Fibrous dysplasia (FD), a developmental, nonfamilial, benign anomaly of bone development, is characterized by the replacement of normal bone by proliferating fibro-osseous tissue. Marked craniofacial deformities, functional disturbances, and emotional stress are major indications for treatment, and various surgical procedures have been performed; however, excision and regrowth issues have also been reported. While several treatment options are available, no studies have reported the natural history of untreated FD. Here, we report 2 patients, aged 73 and 50 years, respectively, who had not received treatment. Both patients presented to the hospital complaining of noise when moving their heads. Computed tomography scans showed niveau with honeycomb cavities in both patients, indicating abscess formation, and resection was performed. Relatively large cranial FD leads to the development of central necrosis over time. In such cases, surgical intervention should be performed at an early disease stage.

Three-dimensional adipofascial and dermal structures involved in forehead crease formation.

Deeply etched forehead creases indicate aging. Various treatments such as filler injections, fat grafting, and facelift surgery are used to remove them. However, knowledge of the anatomical structures associated with subcutaneous tissue changes and the superficial musculoaponeurotic system is lacking, and there is no consensus about the appropriate treatment. We have investigated the subcutaneous structures involved in forehead creases; this will help to establish selection criteria for improved treatment. The forehead sections of five unfixed adult Asian cadavers were obtained. Tissues containing forehead creases were removed from the periosteum and were examined using gross observation, radiography, histology, and nano-computed tomography. All methods revealed that the dermis in the skin crease area, namely the fold visible from the body surface, was bound to the frontalis muscle by a three-dimensional fibrous structure between the fatty septa. This structure was dense near the skin folds and sparse and thin in other areas. In particular, it was tightly bound to the dermis immediately below the crease, with collagen fibers traversing toward the epidermis. In addition, there were fewer skin appendages near the crease than in the normal area, or they were absent altogether; the epidermis was thicker, and the dermal papillae were more developed. It is thought that the density and firmness of the fibrous fatty septal structures between the dermis-frontalis muscle and the specific structures of the epidermis and dermis immediately below the crease account for the characteristic plastic forehead creases.

The Necessity of Dural Resection for Nasal Dermal Sinus Cyst With Intracranial Extension.

Nasal dermal sinus cysts are characterized by an intracranial-extradural extension. Complete extirpation of nasofrontal dermoid sinus cysts is essential for effective treatment to minimize recurrence. The authors revealed the pathologic findings of the cranial end connected to the dura. In our case, the cranial end and dura were fibrous connective tissues that were difficult to separate. For complete extirpation of the nasal dermal sinus cyst with intracranial extension, the cranial ends of the nasofrontal dermoid sinus cyst and dura should be resected en bloc.

Influence of the Number of Revision Surgeries and Nasal Symmetry After Final Rhinoplasty for Patients With Cleft Lip.

The effects of operative intervention on vertical nasal growth in patients with unilateral cleft lips (CLs) are well described. However, the factors influencing nasal symmetry have not been sufficiently evaluated. Therefore, this study aimed to study the factors that cause difficulties in obtaining nasal symmetry postoperatively in patients with CLs. We conducted a retrospective analysis using data from patients with CLs who underwent a series of treatments at Keio University Hospital from 1990 to 2000. We collected data on the patients' sex, cleft type, number and time of revision surgery, palatal fistula incidence, and history of the pharyngeal flap and orthognathic surgery. Nasal symmetry was analyzed as the symmetrical ratio after the final touch-up surgery, and multivariate analysis was conducted using binary logistic regression to determine the factors affecting nasal symmetry. This study included 89 patients with unilateral CL. Multivariate analysis revealed that complete cleft lip and palate ( P < 0.05, odds ratio = 4.37) and repeated revision surgery ( P < 0.05, odds ratio = 9.28) were significant predictors of the final nasal symmetry. Our study showed that cleft type and the number of revision surgeries were identified as important factors for obtaining nasal symmetry after final touch-up rhinoplasty. Revision surgery may be necessary to relieve patients' psychological stress due to nasal deformity, suggesting that this dilemma needs to be overcome.

Incidence of Otalgia After Orthognathic Surgery.

BACKGROUND: In orthognathic surgery, it is well known that maxillary osteotomies and displacements sometimes affect auditory function. Thus, this study examined the relationship between the direction of maxillary displacement and postoperative otalgia. METHODS: Twenty consecutive patients underwent Le Fort I maxillary osteotomy using advancement, impaction, setback, or a combination of these procedures. The direction of movement and incidence of otalgia were investigated. Patients provided informed consent preoperatively, and postoperative reassurance was prudent. RESULTS: Pure-tone average evaluation based on horizontal or vertical movements did not show significant differences, although vertical movements resulted in fewer changes in the hearing threshold. Specifically, no significant changes were observed in the hearing thresholds of patients after surgery. No significant difference was also observed between horizontal and vertical movements in the tympanometry results. Negative changes were found in the results of the Eustachian tube dysfunction test in vertical movements, which returned to preoperative values in the final test. CONCLUSIONS: The risk of minor changes in hearing function is probable during the first week after orthognathic surgery; however, these negative changes either completely disappear or remain negligible.

Influence of Lip Revision Surgery on Facial Growth in Patients With A Cleft Lip.

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.

The Learning Curve of a Craniofacial Surgeon: A Retrospective Study of 100 Consecutive Cases.

ABSTRACT: 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.

Factors Affecting Optic Nerve Damage in Le Fort III Osteotomy: A Retrospective Study.

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.

Application of Deep Learning Techniques for Automated Diagnosis of Non-Syndromic Craniosynostosis Using Skull.

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.

An Elderly Patient With Crouzon Syndrome Treated With Monobloc Distraction.

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.

Gorham-Stout disease with parietal bone osteolysis: a case series and review of literature.

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.

Early Fat Grafting for Augmentation in Craniofacial Microsomia.

ABSTRACT: 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.

Bioabsorbable System-Related Subcutaneous Swelling After Craniofacial Surgery.

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.

Reconstruction of Combined Orbital Floor and Medial Wall Fractures Using Custom-Made Titanium Alloy Implant.

ABSTRACT: 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.

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.

Delayed-Onset Familial Sagittal Suture Synostosis.

Sagittal craniosynostosis is the most common of all craniosynostoses. Patients with sagittal craniosynostosis exhibit a typical cranial appearance, including scaphocephaly, and an elongated head, with a fused and ridged sagittal suture. Moreover, some recent reports described atypical sagittal craniosynostosis accompanied by autism, speech delay, and hyperactivity. This condition, known as delayed-onset craniosynostosis, is rarely reported, given that it is difficult to determine if the condition is congenital or has a delayed-onset.This report describes the clinical course and treatment of 2 brothers with atypical sagittal synostosis. The shapes of their heads were not indicative of scaphocephaly and the younger brother exhibited delayed-onset sagittal synostosis. Their father and paternal grandmother exhibited similar cranial morphologies. Therefore, we hypothesized the involvement of a familial factor in the etiology of atypical sagittal synostosis in these patients.