• Archives of Plastic Surgery
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• v.33 no.5
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• pp.669-671
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• 2006

Purpose: Congenital insensitivity to pain with anhidrosis(CIPA) is a rare form of autosomal recessive peripheral sensory neuropathy. Patients with CIPA show loss of pain sensation, which leads to corneal ulcers and opacities, self-mutilation of the tongue and fingertips, as well as fractures with subsequent joint deformities and chronic osteomyelitis. The purpose of this report is to highlight the fact that pressure sores also are a potential complication of CIPA. Methods: This case report describes a patient presenting with pressure sores resulting from CIPA. A 5-year-old boy was referred to our department for the treatment of a $5{\times}5cm$ sacral pressure sore as a result of a hip spica cast applied for the treatment of a left hip joint dislocation. He had a history suggesting CIPA such as multiple bony fractures, mental retardation, recurrent hyperpyrexia, anhidrosis, and clubbing fingers due to oral mutilation. A microscopic examination of the sural nerve showed mainly large myelinated fibers, a few small myelinated fibers and an almost complete loss of unmyelinated fibers. After wound preparation for two weeks, the exposed bone was covered with two local advancement flaps. Results: Two weeks later, complete wound healing was achieved. A 16-month follow-up showed no recurrence. However, the patient presented with a new pressure sore on the left knee due to orthosis for the treatment of the left hip joint dislocation. Conclusion: The early diagnosis of CIPA and special care of pressure sores are important for preventing and treating pressure sores resulting from CIPA.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.16 no.2
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• pp.113-121
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• 1994

Large oral defects following tumor resection pose formidable challenge for the reconstructive surgeon. Ideally, wound closure should utilize like tissue in providing expedient, single-stage closure, returning maximum function while minimizing deformity. Recent methods have reported and utilize variable mucocutaneous flaps. However, the ideal reconstruction has yet to defined. The small bowel serves as a readily available donor site for satisfying reconstructive needs in oropharyngeoesophageal defects. Segments of jejunum may be opened along the antimesenteric border and transferred to oral defect as free tissue transfers. Some of the benefits of this technique have included a one-stage procedure, abundant donor tissue with characteristics similar to oral mucosa, near normal facial appearance, preservation of maximum tongue function and relief of annoying xerostomia by jejunal mucous secretion. Three cases re presented in which two cases show successful use of this flap. The other one patient developed total necrosis of this flap. We report cases of reconstruction using free jejunal flap transfer in oral reconstruction.

• Archives of Reconstructive Microsurgery
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• v.15 no.2
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• pp.92-100
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• 2006

Radial forearm free flap (RFFF) has been established itself as a versatile and widely used method for reconstruction of the head and neck, although it is still criticized for high mortality of donor site. Delayed wound healing, cosmetic deformity, vascular compromise and potentially reduced wrist function have many plastic surgeons hesitate to adapt it as a first choice in micro-reconstruction. To overcome these drawbacks, some techniques for donor-site repair such as V-Y advancement with full thickness skin graft (FTSG), application of artificial dermis ($Terudermis^{(R)}$) or acellular dermal matrix ($AlloDerm^{(R)}$), and double-opposing rhomboid transposition flap have been reported. Authors performed 4 cases of RFFF in old-aged patients of the head and neck cancer from April 2005 to February 2006. We compared the outcomes of donor site of RFFF which were resurfaced with split thickness skin graft (STSG) only and STSG overlying an $AlloDerm^{(R)}$. Patients were all males ranging from 59 to 74 years old (mean, 67.5). Three of them had tongue cancers, and the other showed hypopharyngeal cancer. All cases were pathologically confirmed as squamous cell carcinomas. We included the deep fascia into the flap, so called subfascially elevated RFFF in three cases, and in the other one, we dissected the RFFF suprafascially leaving the fascia intact. The donor site of the suprafascially elevated RFFF was resurfaced with STSG only. Among three of subfascially elevated RFFFs, donor-sites were covered with thin STSG only in one case, and STSG overlying $AlloDerm^{(R)}$ in two cases. All RFFFs were survived completely without any complication. The donor site of the suprafascially elevated RFFF was taken well with STSG only. But, the partial graft loss exposing brachioradialis and flexor carpi radialis muscle was unavoidable in all the subfascially elevated RFFFs irregardless of $AlloDerm^{(R)}$ application. Considering that many patients of the head and neck cancer are in old ages, we believe the RFFF is still a useful and versatile choice for resurfacing the head and neck region after cancer ablation. Its reliability and functional characteristics could override its criticism for donor site in old-aged cancer patients.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.14 no.2
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• pp.97-101
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• 2018

Lesch-Nyhan syndrome is a rare X-linked recessive genetic disorder. During purine metabolism, the hypoxanthine guanine phosphribosyl transferase (HGPRT) enzyme is deficient causing phosphoribosyl transferase to accumulate and resulting in excessive uric acid. Clinical symptoms include hypercalcemia, choreoathetosis, spasticity, mental retardation, and self-injury to lips, tongue or fingers. This results not only in pain caused by the self-injury but also secondary infection of the wound site and the esthetic damage of the soft tissue defect. Dental treatments include conservative methods using intraoral appliances such as soft mouthguards, fixed lip bumpers, and occlusal guards, and invasive methods such as extraction of all teeth or forming an artificial anterior open bite. We report two cases of Lesch-Nyhan syndrome patients with self-mutilation behavior; one was treated with a preservative method using a soft mouthguard, and the other was treated with extraction of all teeth.