Macrodystrophia lipomatosa (MDL) is a rare congenital non-hereditary disorder that has significant impact on patient morbidity. This study provides a comprehensive review of the natural history, diagnosis, management, and outcomes of the disorder. A literature search in PubMed was conducted to identify cases of MDL from January 1950 to 14 February 2014. After ruling out articles without information related to the management of the disorder, a summary of 32 studies was performed. An additional three cases from the authors are also presented. Based on 57 journal articles and three additional cases from the authors, around 108 cases of MDL were reviewed. Most patients were males who were admitted to a treatment clinic in the first four years of life. The lower extremities were more frequently affected, with unilateral presentation being most common. They commonly underwent a single-staged surgical procedure with follow-up periods ranging from more than one year up to 21 years. Out of 43 cases that underwent surgical procedures, 13 reported no complications, and there were seven cases of esthetic satisfaction and 15 cases of significant functional improvement. Depending on the severity of a patient's condition, the use of non-invasive diagnostic tools should be carefully considered. Surgery might be a better choice of management than observation, taking into account possible future complications in the absence of surgery and the beneficial outcomes of surgical procedures.
BackgroundA pneumatic tourniquet is generally used to achieve a bloodless operation field in hand surgery. However, this has changed with tumescent solution-based wide-awake surgery. This study is a preliminary prospective case series study to elaborate the formula and indications of the tumescent technique in hand surgery without a tourniquet.MethodsSeven patients (age range, 4 months to 37 years) underwent hand or upper extremity surgery for conditions such as nerve palsy, electric burn defect, fingertip injury, contracture, constriction ring syndrome, or acrosyndactyly. A "one-per-mil" tumescent solution (epinephrine 1:1,000,000+20 mg lidocaine/50 mL saline) was used to create a bloodless operating field without a tourniquet. Observation was performed to document the amount of solution injected, the operation field clarity, and the postoperative pain.ResultsThe "one per mil" epinephrine solution showed an effective hemostatic effect. The tumescent technique resulted in an almost bloodless operation field in the tendon and in the constriction ring syndrome surgeries, minimal bleeding in the flap and contracture release surgeries, and acceptable bleeding in acrosyndactyly surgery. The amount of solution injected ranged from 5.3 to 60 mL. No patient expressed significant postoperative pain. Flap surgeries showed mixed results. One flap was lost, while the others survived.ConclusionsEpinephrine 1:1,000,000 in saline solution is a potential replacement for a tourniquet in hand surgery. Further studies are needed to delineate its safety for flap survival.
Wound healing is a transition of processes which is also recognized as one of the most complex processes in human physiology. Complex series of reactions and interactions among cells and mediators take place in the healing process of wound involving cellular and molecular events. The inflammatory phase is naturally intended to remove devitalized tissue and prevent invasive infection. The proliferative phase is characterized by the formation of granulation tissue within the wound bed, composed of new capillary network, fibroblast, and macrophages in a loose arrangement of supporting structure. This second phase lasts from day 8 to 21 after the injury is also the phase for epithelialisation. The natural period of proliferative phase is a reflection for us in treating wound to reach the goal which ultimately defines as closed wound. The final maturation phase is also characterized by the balancing between deposition of collagen and its degradation. There are at least three prerequisites which are ideal local conditions for the nature of wound to go on a normal process of healing i.e. 1) all tissue involved in the wound and surrounding should be vital, 2) no foreign bodies in the wound, and 3) free from excessive contamination/infection. The author formulated a step ladder of thinking in regards of healing intentions covering all acute and chronic wounds. Regarding the "hierarchy" of healing intention, the first and ideal choice to heal wounds is by primary intention followed by tertiary intention and lastly the secondary intention.
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