Novel Finding of an Excess Bone in Postaxial Polydactyly of the Foot.

Background: Postaxial polydactyly is a common congenital foot anomaly. However, the severity of the anomaly varies from simple cases with only soft tissue duplication to complex cases with bone and joint disorders. In our clinical practice, we found a new morphological anomaly of postaxial polydactyly. We encountered several cases of postaxial polydactyly with bone fragments located between the fourth and fifth toes. The bone fragments were independent of the joint cavity. The mechanisms underlying its development remain unknown because it is a novel disorder. In the present study, we investigated the characteristics of the excess bone to formulate an embryological hypothesis. Methods: We examined the frequency and trends in the occurrence of excess bone using data from photographs and radiographs of these cases. An example of a disorder similar to excess bone is mosaic-like alignment, as reported by Iba et al. We also compared the characteristics of the mosaic-like alignment with those of the excess bone. Based on these data and existing embryological knowledge, we hypothesized the origin of the excess bone. Results: Excess bone and mosaic-like alignments showed different characteristics. Therefore, both were considered completely different disorders. We hypothesized that excess bone was caused by damage to the interdigital ectoderm immediately before interdigital programmed cell death. Conclusions: We encountered a new form of postaxial polydactyly. This can be a factor influencing the treatment strategy because it can affect alignment and stability.

Bronchogenic cysts in rare sites (retroperitoneum, skin, spinal cord and pericardial cavity): A case series and characterization of epithelial phenotypes.

Bronchogenic cysts are congenital malformations of the bronchial tree, detected as a cystic and/or mass lesion in the thoracic cavity. Although it occurs in distant locations, such as skin and retroperitoneum, to the best of our knowledge, little is known about the components and phenotypes of the epithelium that line a bronchogenic cyst in rare sites. The present study reviewed 34 bronchogenic cysts that were surgically resected at Osaka Medical and Pharmaceutical University Hospital (Osaka, Japan) from January 1998 to December 2020. Bronchogenic cysts in rare sites were detected and diagnosis was confirmed based on the presence of pseudostratified, ciliated and/or columnar epithelium together with at least one of the following: Cartilage, smooth muscle or seromucous glands. The phenotypes of epithelium lining the cyst were characterized using immunohistochemical analysis. A total of six bronchogenic cysts in rare sites (two cases each in the retroperitoneum and skin and one case each in the cervical spinal cord and pericardial cavity) met the criteria for confirmation of the diagnoses. The epithelium lining the cyst stained positive for cytokeratin CK7 and thyroid transcription factor 1 (a marker expressed in thyroid follicles and bronchial epithelium) and negative for CK20, indicating that the phenotypes were similar to those of the respiratory epithelium. The present study demonstrated that a bronchogenic cyst can occur in rare sites, such as the retroperitoneum, skin, spinal cord and pericardial cavity, suggesting that it should be considered as a differential diagnosis before surgical approach to implement relevant management modalities such as follow-up, simple or radical resection.

Congenital Nostril Stenosis with a Horseshoe-like Shape.

We report a rare case of congenital nostril stenosis because it is very interesting from the perspective of human embryo development. As we were not able to find a similar congenital case in the literature, we would like to describe it here. The patient is a 36-year-old woman who had bilateral congenital stenotic nostrils with horseshoe-like shape. Particularly, the bilateral medial crura protruded prominently. When she inhaled strongly, she could not breathe further because the action was analogous to the closure of a valve. Her elder sister had similar stenosis in the right nostril only, and parents did not have any stenotic nostrils. During the surgery, the prominent webs of the bilateral medial crura were resected, and Y-V advancement flap was inserted bilaterally to avoid restenosis. Histopathological examination revealed that the resected tissue contained cartilaginous and muscular tissue. A Koken retainer was worn throughout each entire day without washing the face or taking a bath for 3 months after surgery and was washed for keeping hygiene many times every day. After surgery, the patient can breathe well when she inhales strongly and is very satisfied with the operative results. Stenosis has not recurred 6 months after operation. Pathogenesis of this case is thought to occur at the early phase of embryo development when the medial and lateral nasal placodes form a downward-facing "horseshoe."

Orbital Roof Decompression and Transposition of Orbital Contents for Treatment of Orbital Deviation Associated With Fibrous Dysplasia.

A 25-year-old male was admitted to our institute complaining of caudal deviation of orbit. Surgery was performed through a coronal incision, a subtarsal incision, and a lynch incision. The periosteum of the orbital roof was raised, and the supraorbital wall was shaved cephalad in reference to the nonaffected side. Then the periosteum was incised from the inferior margin of the orbit to the medial and lateral palpebral ligaments, and the periosteum of the orbital floor was raised until the inferior orbital fissure. After the circumferential intraorbital periosteal detachment, the orbital contents could be readily moved cephalad ward. The outer table of the calvarial bone was transplanted to the orbital floor, and the palpebral ligaments were fixed 5 mm cranially. In the 5-year follow-up, the patient's visual function was normal, and no recurrence of fibrous dysplasia was observed.

Tryptase-Positive Mast Cells Promote Adipose Fibrosis in Secondary Lymphedema through PDGF.

Lymphedema is a chronic and progressive condition that causes physical disfigurement and psychological trauma due to the accumulation of lymphatic fluid in the interstitial space. Once it develops, lymphedema is difficult to treat because it leads to the fibrosis of adipose tissue. However, the mechanism behind this remains unclear. The purpose of this study was to investigate the involvement of mast cells (MCs) in the adipose tissues of patients with lymphedema. We found that fibrosis spread through blood vessels in the adipose tissues of lymphedema patients, and the expression of the collagen I and III genes was significantly increased compared to that of those in normal adipose tissue. Immunostaining of vimentin and α-smooth muscle actin showed that fibroblasts were the main cellular components in severely fibrotic regions. Toluidine blue staining confirmed a significant increase in the number of MCs in the adipose tissues of lymphedema patients, and immunostaining of serial sections of adipose tissue showed a significant increase in the number of tryptase-positive cells in lymphedema tissues compared with those in normal adipose tissues. Linear regression analyses revealed significant positive correlations between tryptase and the expressions of the TNF-α, platelet-derived growth factor (PDGF)-A, and PDGFR-α genes. PDGF-A-positive staining was observed in both fibroblasts and granules of tryptase-positive MCs. These results suggest that MC-derived tryptase plays a role in the fibrosis of adipose tissue due to lymphedema directly or in cooperation with other mediators.

Modified Preparatory Intravascular Stenting Technique in Super-microsurgical Lymphaticovenular Anastomosis for the Treatment of Lymphedema.

Clinical studies have reported that lymphaticovenular anastomosis (LVA) is more effective for early-stage lymphedema. However, the diameter of lymphatic vessels in early-stage lymphedema is thin (only about 0.3 mm). In this article, we report a modified preparatory intravascular stenting technique (PIST) for LVA with smaller lymphatic vessels and present the results of its application for the treatment of secondary lymphedema. In this technique, a 9-0 nylon thread is inserted into the target lymphatic vessel. Then, the thread is pulled until its tip has entered the lymphatic vessel. After that, the thread is allowed to proceed into the lumen by pushing it. Finally, with the nylon in place, the lymphatic vessel is transected and the lumen is secured. In this report, we investigated the surgical time for LVA between 10 patients who underwent LVA with modified PIST (group A) and another group of 10 patients who underwent LVA without the technique (group B). Lymphatic lumen was secured at all sites where indocyanine green lymphangiography confirmed lymphatic flow. The average outer diameter of lymphatic vessels in group A and B were 0.36 mm and 0.53 mm, respectively. The average surgical time for LVA in group A was 136 minutes, which was significantly shorter than the 187 minutes in group B. Our results indicated that modified PIST can help secure the lymphatic lumen even when the lymphatic vessels are thin. As a result, this technique can significantly reduce the surgical time for this procedure.

Experimental Study of Warburg Effect in Keloid Nodules: Implication for Downregulation of miR-133b.

Background: A keloid is composed of several nodules, which are divided into two zones: the central zone (CZ; a hypoxic region) and the marginal zone (MZ; a normoxic region). Keloid nodules play a key role in energy metabolic activity for continuous growth by increasing in number and total area. In this study, we aimed to investigate the roles of the zones in the execution of the Warburg effect and identify which microRNAs regulate this phenomenon in keloid tissue. Methods: Eleven keloids from patients were used. Using immunohistochemical analysis, 179 nodules were randomly chosen from these keloids to identify glycolytic enzymes, autophagic markers, pyruvate kinase M (PKM) 1/2, and polypyrimidine tract binding protein 1 (PTBP1). Western blot and qRT-PCR tests were also performed for PKM, PTBP1, and microRNAs (miR-133b and miR-200b, c). Results: Immunohistochemical analysis showed that the expression of the autophagic (LC3, p62) and glycolytic (GLUT1, HK2) were significantly higher in the CZ than in the MZ. PKM2 expression was significantly higher than PKM1 expression in keloid nodules. Furthermore, PKM2 expression was higher in the CZ than in the MZ. However, PKM1 and PTBP1 expression levels were higher in the MZ than in the CZ. The qRT-PCR analysis showed that miR-133b-3p was moderately downregulated in the keloids compared with its expression in the normal skin tissue. Conclusions: The Warburg effect occurred individually in nodules. The MZ presented PKM2-positive fibroblasts produced by activated PTBP1. In the CZ, PKM2-positive fibroblasts produced lactate. MiR-133b-3p was predicted to control the Warburg effect in keloids.

Secondary Reconstruction of Late Deformities of the Orbitozygomaticomaxillary Complex using a Halo Device.

Reconstructing late deformities of the orbitozygomaticomaxillary complex after inadequate treatment of facial fractures requires zygomatic osteotomy to reposition the bony fragments to their anatomical position. However, confirming the position of the bone fragments can be challenging due to the loss of anatomic landmarks caused by bone remodeling, and swelling resulting from surgery, which might hinder locating the bone from the body surface. Here, the authors describe fixation of the halo of the Blue Device multi-vector distraction system to the patient's ear canal, with alignment of the position of the zygoma by measuring the distances between the halo and zygoma using several reference points. This technique allows for measurement not only from the body surface using a K-wire but also directly to the bone using a needle. The authors applied this technique in 2 cases of post-traumatic deformities after complex zygomatic fractures. Both cases achieved an almost symmetrical appearance of the infraorbital region.

Abdominal Wall Incisional Hernia Repair with the Anterior Component Separation Technique and Reinforcement with an Anterior Rectus Abdominis Sheath Flap.

Mesh repair is currently the mainstay of treatment for abdominal wall incisional hernias and is considered the standard of care. However, if radiotherapy is used, the possibility of complications such as exposure or infection of the prosthesis after the surgery as a complication of the radiotherapy is a concern. The patient was a 51-year-old woman who underwent laparotomy by a mid-abdominal incision for ovarian tumors. Approximately 2 years later, the patient presented with a hypertrophic scar of the wound and mild pain in the scar. The hypertrophic scar was improved gradually by corticosteroid injection. However, she had a bulge on the left side of the umbilicus just below the hypertrophic scar. Computed tomography showed a 65 × 69 mm2 hernial orifice on the left side of the umbilical abdominal wall, and an abdominal wall incisional hernia was diagnosed. The patient underwent closure by the ACS technique and reinforcement by unilateral inversion of the anterior rectus abdominis sheath for the abdominal wall incisional hernia. No recurrence of the hypertrophic scar or abdominal wall incisional hernia was observed during the follow-up period. In the present case, the hernial orifice was closed by a modified ACS technique that was combined with the anterior rectus abdominis sheath turnover flap. This technique is likely a less invasive and relatively simple method resulting in a tighter repair of the abdominal hernia than the ACS method alone, without prostheses.

Ocular and extraocular sebaceous carcinomas: A retrospective study with emphasis on the presence of in situ lesion and discussion and review of the histogenesis of extraocular sebaceous carcinoma.

Sebaceous carcinoma (SC) is a rare carcinoma classified as ocular or extraocular. Ocular SC is believed to arise from the meibomian glands or the glands of Zeis. However, the origin of extraocular SC is controversial because there is no evidence of carcinoma arising from pre-existing sebaceous glands. Several hypotheses about the origin of extraocular SC have been proposed, including one suggesting an origin from intraepidermal neoplastic cells. Although extraocular SCs have been shown to occasionally comprise intraepidermal neoplastic cells, no study has investigated whether intraepidermal neoplastic cells possess sebaceous differentiation. The present study analyzed the clinicopathological features of ocular and extraocular SC, with an emphasis on the presence of in situ (intraepithelial) lesions. It retrospectively reviewed the clinicopathological features of eight patients with ocular and three patients with extraocular SC (eight women and three men; median age, 72 years), respectively. In situ (intraepithelial) lesions were observed in four of the eight ocular SC cases and one of the three extraocular SC cases and an apocrine component was noted in one patient with ocular SC (seboapocrine carcinoma). In addition, immunohistochemical analyses showed that the androgen receptor (AR) was expressed in all ocular SCs and two of the three extraocular SC cases. Adipophilin expression was observed in all ocular and extraocular SC. In situ lesions of extraocular SC showed positive immunoreactivity for both AR and adipophilin. The present study is the first to demonstrate sebaceous differentiation in in situ lesions of extraocular SC. The possible origin of extraocular SC is speculated to be the progenitor cells present in the sebaceous duct or interfollicular epidermis. The results of the present study and reported cases of SC in situ indicate that extraocular SC also arises from intraepidermal neoplastic cells.

Clinical Application to Improve the "Depth Perception Problem" by Combining Augmented Reality and a 3D Printing Model.

In our experience with intraoperative evaluation and educational application of augmented reality technology, an illusion of depth has been a major problem. To improve this depth perception problem, we conducted two experiments combining various three-dimensional models and holograms and the observation angles using an augmented reality device. Methods: In experiment 1, when observing holograms projected on the surface layer of the model (bone model) or holograms projected on a layer deeper than the model (body surface model), the observer's first impression regarding which model made it easier to understand positional relationships was investigated. In experiment 2, to achieve a more quantitative evaluation, the observer was asked to measure the distance between two specific points on the surface and deep layers from two angles in each of the above combinations. Statistical analysis was performed on the measurement error for this distance. Results: In experiment 1, the three-dimensional positional relationships were easier to understand in the bone than in the body surface model. In experiment 2, there was not much difference in the measurement error under either condition, which was not large enough to cause a misunderstanding of the depth relationship between the surface and deep layers. Conclusions: Any combination can be used for preoperative examinations and anatomical study purposes. In particular, projecting holograms on a deep model or observing positional relationships from not only the operator's viewpoint, but also multiple other angles is more desirable because it reduces confusion caused by the depth perception problem and improves understanding of anatomy.

Axillary accessory breast cancer reconstructed by a thoracodorsal artery perforator flap: A case report.

INTRODUCTION: Primary accessory breast cancer is rare and most commonly occurs in the axilla. Due to its low incidence, few studies have discussed axillary reconstruction after accessory breast cancer resection. In the present report, we describe a patient who underwent axillary reconstruction with a thoracodorsal artery perforator (TAP) flap after resection, and reconstruction methods after resection of axillary accessory breast cancer are discussed based on current and previous reports. PATIENT CONCERNS: A 60-year-old woman presented with a 7-year history of a gradually growing lump in the left axilla. DIAGNOSIS: The patient was diagnosed with latent breast cancer, axillary lymph node metastasis, or carcinoma of the accessory axillary breast with axillary lymph node metastasis. INTERVENTIONS: After preoperative chemotherapy, tumor resection and axillary lymph node dissection were performed, followed by immediate axillary reconstruction using a TAP flap. The patient received postoperative adjuvant endocrine and radiation therapy (50 Gy). OUTCOMES: No recurrence or metastasis was observed for 5 years postoperatively. The reconstructed axilla was not bulky, and scar contracture was not observed, with a full range of motion of the shoulder joint. CONCLUSION: We described a patient who underwent immediate TAP flap reconstruction after resection of accessory breast cancer and axillary lymph node dissection, followed by postoperative radiation, which could cause scar contracture. The patient was followed up for more than 5 years after the operation and radiation therapy, and the appearance of the axilla and range of motion of the shoulder were good despite postoperative radiation.

Modified trilobed transposition flap for a distal nasal defect.

Reconstruction of a distal nasal defect is challenging due to poor skin mobility and the potential for nasal alar retraction. A trilobed flap can utilize more mobile proximal skin, increase the total rotational arc, and decrease the tension associated with flap transposition. However, the trilobed flap may not be ideal for distal nasal defects because each flap is designed using immobile skin, which may lead to flap immobility and free margin distortion. To overcome these problems, the base and tip of each flap were extended further from the pivot point than those of the conventional trilobed flap. Herein, we report the use of the modified trilobed flap to treat 15 consecutive cases of distal nasal defects that occurred from January 2013 to December 2019. The mean duration of follow-up was 15.6 months. All flaps survived completely, and satisfactory aesthetic outcomes were achieved. No complications such as wound dehiscence, nasal asymmetry, or hypertrophic scarring were observed. The modified trilobed flap is a simple and reliable treatment for distal nasal defects.

Giant Density of States Enhancement Driven by a Zero-Mode Landau Level in Semimetallic Black Phosphorus under Pressure.

Dirac fermion systems form a unique Landau level at the Fermi level-the so-called zero mode-whose observation itself will provide strong evidence of the presence of Dirac dispersions. Here, we report the study of semimetallic black phosphorus under pressure by ^{31}P-nuclear magnetic resonance measurements in a wide range of magnetic field up to 24.0 T. We have found a field-induced giant enhancement of 1/T_{1}T, where 1/T_{1} is the nuclear spin lattice relaxation rate: 1/T_{1}T at 24.0 T reaches more than 20 times larger than that at 2.0 T. The increase in 1/T_{1}T above 6.5 T is approximately proportional to the squared field, implying a linear relationship between the density of states and the field. We also found that, while 1/T_{1}T at a constant field is independent of temperature in the low-temperature region, it steeply increases with temperature above 100 K. All these phenomena are well explained by considering the effect of Landau quantization on three-dimensional Dirac fermions. The present study demonstrates that 1/T_{1} is an excellent quantity to probe the zero-mode Landau level and to identify the dimensionality of the Dirac fermion system.

Useful Genioplasty for Repeated Recurrent Sleep Apnea of Congenital Anomalies and Its Evaluation.

Congenital facial anomalies with hypoplasia of the midface or lower face are associated with obstructive apnea syndrome. Although such patients underwent bone advancement surgery and their sleep apnea improved in the short term, it often recurred several years after surgery. It is difficult to perform another major osteotomy because of impairment of the facial contour or prior orthodontic treatment. Genioplasty was performed for genioglossus muscle advancement in patients with congenital anomalies and repeated sleep apnea. In this study, we evaluated the usefulness of this procedure and the mechanism for the improvement of sleep apnea. Methods: Six patients were included: three with syndromic craniosynostosis, two with Treacher-Collins syndrome, and one with micrognathia by Goldenhar syndrome. Patients who had recurrence of sleep apnea after previous maxillomandibular osteotomies, or advancement and orthodontic treatment, received genioplasty for genioglossus muscle advancement. The patients were evaluated by body mass index, simple polysomnography, hyoid bone position on cephalogram, and the airway area on computed tomography images pre- and postoperatively. Results: Polysomnography showed a significant improvement in the apnea-hypopnea index. Cephalometric measurement showed significant results of the hyoid bone position from point B and the ramus plane. However, no significant results were obtained in the airway area assessment. Conclusions: Genioplasty for genioglossus muscle advancement can improve apnea-hypopnea index by moving the hyoid bone forward. Genioplasty was useful in patients with congenital anomalies who had a recurrence of sleep apnea after several procedures.

Molding Helmet Therapy for Severe Deformational Brachycephaly: Position of Eurion and Therapeutic Effect.

BACKGROUND: Molding helmet therapy is used routinely for moderate to severe deformational plagiocephaly. However, there have been few reports of its use for deformational brachycephaly (DB). The incidence and severity of DB are high in the Asian population, including Japanese people, but there are no reports of treatment for severe cases. The current study showed significant improvement even in severe DB with various deformities. The difference in improvement according to the morphologic classification was investigated. METHODS: The study included 47 patients treated with helmet therapy for DB with a cephalic index of greater than 100%. Three-dimensional head scans were used for classification and measurement. DB was classified into four types according to the position of the eurion and the presence of deformational plagiocephaly. The therapeutic effect was determined by changes in cephalic index and cranial asymmetry. RESULTS: Patients exhibited a mean change of 8.0% in cephalic index and 6.4 mm in cranial asymmetry. An older age at initiation was associated with less change in both cephalic index and cranial asymmetry. A significant difference in the amount of cephalic index change was observed depending on the position of the eurion when the starting age was 6 months or older ( P < 0.05). CONCLUSIONS: Molding helmet therapy for severe DB results in significant morphological improvement. However, depending on the position of the eurion, the effect of treatment may be decreased if it is started too late. It is necessary to understand the difference in improvement depending on the morphology and to ensure early intervention. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

Glove-shaped Foam with Negative Pressure Wound Therapy for Skin Graft Fixation on the Hand.

Tie-over bolster dressing has been the gold standard for skin graft immobilization. However, skin grafting onto the hand remains challenging. To prevent shearing of the skin, joint fixation with Kirschner wire and casting is often required. However, wire fixation through the joint can disrupt finger growth and cause joint contracture, especially in pediatric patients. So, we performed graft fixation with negative pressure wound therapy (NPWT). The use of NPWT with skin grafting has recently been reported. Previous studies have reported that NPWT can provide even pressure on irregular wounds and in highly mobile areas. However, application of NPWT in the digital region often results in air leaks. This report includes four patients who required skin grafting on the hand. All patients received skin grafts in the affected area. Graft fixation was performed with NPWT. A glove-shaped form was designed. The hand was encased on the ventral and dorsal sides and small pieces of foam were placed between the fingers. The fixation was maintained for 7 days at a pressure of -50 to -80 mmHg. None of the cases had air leak requiring reattachment of the system and graft take was successful in all cases without any complications. The NPWT "glove-shape" technique enabled maintenance of average negative pressure for all skin grafts on the hand. This technique does not require joint fixation and may help to prevent growth disturbance and joint contracture.