Madoka Sugiyama

Clinical presentation of epignathus teratoma with cleft palate; and duplication of cranial base, tongue, mandible, and pituitary gland.

A 2-day-old girl was diagnosed with an oral epignathus teratoma and an uncommon combination of orofacial malformations including cleft palate; tongue, mandible, cranial base, cervical vertebrae, lower lip, and pituitary gland duplications; and fistula of the glabella and lower lip. Computed tomography revealed that the mass within the nasal cavity had tooth-like calcifications and protruded into the nasopharynx and oral cavity. It was implanted on the anterior wall of the body of the sphenoid bone and was accompanied with mandibular duplication. Magnetic resonance imaging detected duplication of the pituitary gland and confirmed the absence of intracranial communication of the nasopharyngeal mass. The teratoma did not cause respiratory obstruction; however, the patient required continuous nasogastric tube feeding. Usually, an epignathus teratoma is associated with few midline defects and can be corrected with multiple interventions at different time points. The current study describes the surgical procedure comprising excision of the tumor along with reconstructive surgeries of the mandible, tongue, and fistulae undertaken when the infant reached 7 months of age. The cleft palate was repaired at 18 months of age using the Kaplan buccal flap method. Histopathologic examination confirmed a grade 0 teratoma covered with keratinized skin and containing pilosebaceous and sweat glands, adipose tissue, and smooth muscle. The long-term success of this intervention was determined at the follow-up examination conducted at 3 years of age, with no signs of the teratoma recurrence observed.

Preclinical and clinical research on bone and cartilage regenerative medicine in oral and maxillofacial region

Abstract Recently, there have been remarkable advances in regenerative medicine, and almost all disorders of the oral and maxillofacial region could be research targets of regenerative medicine. Meanwhile, treatment in this region has been well established using biomaterials, prostheses, and microsurgery. Therefore, to surpass such a conventional approach as an alternative, regenerative medicine should take an approach of being less invasive and/or more effective. In this report, we present our preclinical and clinical research on bone and cartilage regenerative medicine in the oral and maxillofacial region. Regarding bone regenerative medicine, we have tried to develop artificial bone that would maximize bone formation at the transplanted site, but would subsequently be replaced by autologous bone. We have made custom-made artificial bone (CT-Bone) using alpha-tricalcium phosphate (α-TCP) particles and an ink-jet printer, and have conducted clinical research and trials on 30 patients. To develop tissue-engineered cartilage with proper three-dimensional (3D) morphological form and mechanical strength, we have optimized the culture medium of chondrocytes and the scaffold. Following a preclinical study confirming efficacy and safety, we have conducted clinical research in three patients with nasal deformity associated with cleft lip and palate, and are now starting multicenter clinical research.

Implant-type Tissue-engineered Cartilage for Secondary Correction of Cleft Lip-nose Patients: An Exploratory First-in-human Trial

Objective: Secondary correction of cleft lip-nose presents a formidable challenge in cleft lip and palate surgery. Although numerous approaches have been proposed, suitable graft materials cannot be obtained from any part of body or the artificial biomaterials. We have established implant-type tissue-engineered cartilage using a porous scaffold comprised of poly L-lactic acid. The aim of this study was to primarily assess the safety of the autologous tissue-engineered cartilage when used in the cleft lip-nose patients as an exploratory first-in-human trial, and to explore the usefulness of the cartilage. Methods: After the acquisition of institutional and governmental permission, we used this implant-type tissueengineered cartilage for the treatment of three cleft lip-nose patients. We examined whether or not serious adverse events had occurred by which removal of the tissue-engineered cartilage was needed, 3 years after the transplantation. We also explored the usefulness of the cartilage, as aesthetic and functional outcomes. Results: Each tissue-engineered cartilage fulfilled the defined release criteria for transplantation. The transplantation of the tissue-engineered cartilage in all the patients was performed just as planned. After 3 years of transplantation, we did not experience any serious adverse events that were related to the tissue-engineered cartilage. As a non-serious adverse event, calcification of the tissue-engineered cartilage was found in one patient. Nose shapes improved in all the patients, and more than 2 mm of nose augmentation maintained for 3 years postsurgery, as measured in cephalogram. Although the dysfunction in facial expression or playing sports had rather increased immediately after the transplantation, the inconvenience generally recovered or improved during the postsurgical course. Conclusion: The implant-type tissue-engineered cartilage could safely reconstruct the nasal dorsum and apex of cleft lip-noses. This tissue-engineered cartilage possibly leads to effective correction of a severe cleft lip-nose deformity with aesthetic and functional improvement.

Three-dimensional changes of noses after transplantation of implant-type tissue-engineered cartilage for secondary correction of cleft lip–nose patients

Introduction We have developed an implant-type tissue-engineered cartilage using a poly-l-lactide scaffold. In a clinical study, it was inserted into subcutaneous areas of nasal dorsum in three patients, to correct cleft lip–nose deformity. The aim of this study was to helping evaluation on the efficacy of the regenerative cartilage. Methods 3D data of nasal shapes were compared between before and after surgery in computed tomography (CT) images. Morphological and qualitative changes of transplants in the body were also evaluated on MRI, for one year. Results The 3D data from CT images showed effective augmentation (>2 mm) of nasal dorsum in almost whole length, observed on the medial line of faces. It was maintained by 1 year post-surgery in all patients, while affected curves of nasal dorsum was not detected throughout the observation period. In magnetic resonance imaging (MRI), the images of transplanted cartilage had been observed until 1 year post-surgery. Those images were seemingly not straight when viewed from the longitudinal plain, and may have shown gentle adaptation to the surrounding nasal bones and alar cartilage tissues. Conclusion Those findings suggested the potential efficacy of this cartilage on improvement of cleft lip–nose deformity. A clinical trial is now being performed for industrialization.

Secondary repair of an oblique facial cleft with an absorbable mesh tray and particulate cancellous bone and marrow

Abstract Oblique facial cleft is an extremely rare disorder, accounting for only 0.24% of facial clefts. Even after initial surgery, depressions of the eyeball and facial surface are formed due to bone defects of the maxilla and orbital floor, and since performing bone reconstruction during infancy and early childhood is difficult, pronounced deformity tends to persist. We report our experience with using an absorbable mesh tray together with particulate cancellous bone and marrow for secondary repair by means of bone reconstruction in a 4-year-old girl, achieving good results at a young age.

Necrotising Ulcerative Stomatitis in a Neutropenic Patient with Malignant Lymphoma

Abstract Necrotising ulcerative stomatitis is a very rapid, destructive disease of the alveolar bone and gingiva. The rapid destruction leads to devastating facial defects and death, if it is not treated promptly. Candida albicans and Pseudomonas aeruginosa are frequent causes of disseminated infections among patients who are immunocompromised. This report is of a 72-year-old woman with dose large B-cell lymphoma, who developed acute necrotising ulcerative stomatitis and septic shock together with histopathological and microbiological evidence of C. albicans and P aeruginosa infections. Gingival necrosis was managed by debridement to remove slough and irrigation with povidone iodine. All oral symptoms subsided following the initiation of antimicrobial therapy and debridement. The patient completed chemotherapy and achieved prompt remission. This report highlights the importance of prompt recognition, debridement, teeth extraction, scrupulous oral hygiene, appropriate antibiotic therapy, and nutritional support for immunocompromised patients with necrotising ulcerative stomatitis.

A 2-Stage Reconstruction of the Jaw Using Vascularized Bone and Secondary Alveolar Ridge Augmentation With Particulate Cancellous Bone and Marrow.

Purpose:To present the novel technique for reconstruction of the jaw, that facilitates occlusal restoration using dental implants, in cases with wide bony defects from tumor resection. Materials and Methods:After alveolar ridge is augmented using titanium mesh tray and particulate cancellous bone and marrow (PCBM) from iliac bone on reconstructed bone, by way of improvement of maxillomandibular relationship for dental implants. Results:This 2-stage surgery underwent successfully in 3 cases. After 2-stage surgery and occlusal reconstruction using dental implant, the patients experienced no complications, and received satisfaction with results functionally and aesthetically. Conclusions:Our results suggest that, in cases where bone defect is over a wide area, in addition to vascularized bone grafts, secondary alveolar ridge augmentation using a titanium mesh tray and PCBM on grafted bone can provide satisfactory occlusion further to improvement of facial form.

Correction of Upturned Nasal Tip With a Costal Cartilage Graft in Bilateral Cleft Lip Patients

Abstract We used a piece of costal cartilage as a cartilaginous strut to correct the upturned nasal tip in patients with bilateral cleft lip. The grafted cartilage provides more definition of the tip and improves the obtuse nasolabial angle. Neither the septal cartilage nor the ear cartilage has enough strength to shape the tip. This method of correction has consistently produced favorable, long-lasting results in adults and has improved the contour of the nasal tip in younger patients.