Shiokawa Ichiro, Oshima Naoya, Mizumura Nao, Momosawa Akira
Department of Plastic Surgery, Yamanashi University Hospital, Yamanashi Prefecture, Japan.
Plast Reconstr Surg Glob Open. 2021 Jan 11;9(1):e3303. doi: 10.1097/GOX.0000000000003303. eCollection 2021 Jan.
Gorham-Stout disease (GSD) is a rare musculoskeletal disorder characterized by progressive bone resorption and overgrowth of lymphatic vessels. The mechanism of GSD is still largely unknown. Negative-pressure wound therapy (NPWT) is known to accelerate wound healing and is used worldwide. Herein, we report a successful treatment of a patient with GSD having a sacral pressure ulcer, using NPWT. An 18-year-old female GSD patient was referred to our department for treating a sacral wound. The wound was disinfected by pocket incision, cleansing, and administration of antibiotics; however, the lesion remain unhealed. Histopathology of the debrided sacral wound revealed fibrous granulation tissue, with no sign of lymphatic anomalies. NPWT was started with -75 mm Hg of pressure, and neither lymphorrhea nor growth of lymphangioma was noted. Negative pressure was gradually increased to -125 mm Hg. The ulcer size decreased to 2 × 2 cm, which healed 3 months after hospital discharge, with no recurrence for 8 months. For progressive diseases such as GSD, NPWT may cause the regrowth of lymphangioma or other neoplasms due to an increase in vessel endothelial growth factor. NPWT appears to be one of the safest and most effective wound therapies even for this rare and difficult disease, provided the use of the following treatment protocol: Pathohistological assessment before application of NPWT, and negative pressure initially set at a low level; then, gradually increased, with careful observation to avoid lymphorrhea. When changing the foam dressing, careful checking is important to determine whether the wound is necrotic, or if there is tumor-like tissue accumulation rather than healthy granulation.
戈勒姆-斯托特病（GSD）是一种罕见的肌肉骨骼疾病，其特征为进行性骨吸收和淋巴管过度生长。GSD的发病机制在很大程度上仍不清楚。负压伤口治疗（NPWT）已知可加速伤口愈合，且在全球范围内得到应用。在此，我们报告了一例使用NPWT成功治疗患有骶骨压疮的GSD患者的病例。一名18岁的女性GSD患者因骶骨伤口被转诊至我科。伤口通过袋状切开、清创和使用抗生素进行消毒；然而，病变仍未愈合。清创后的骶骨伤口组织病理学检查显示为纤维肉芽组织，未发现淋巴管异常迹象。NPWT以-75 mmHg的压力开始，未观察到淋巴漏或淋巴管瘤生长。负压逐渐增加至-125 mmHg。溃疡大小缩小至2×2 cm，出院3个月后愈合，8个月内无复发。对于GSD等进行性疾病，NPWT可能会因血管内皮生长因子增加而导致淋巴管瘤或其他肿瘤再生。NPWT似乎是治疗这种罕见且棘手疾病最安全、最有效的伤口治疗方法之一，前提是采用以下治疗方案：在应用NPWT前进行病理组织学评估，负压初始设定为低水平；然后逐渐增加，并仔细观察以避免淋巴漏。更换泡沫敷料时，仔细检查对于确定伤口是否坏死或是否存在肿瘤样组织堆积而非健康肉芽组织很重要。
