BJUI. The management of genital lymphoedema

Transcription

1 2008 The uthors Investigative Urology GRFF ET L. JUI JU INTERNTIONL The management of genital lymphoedema Giulio Garaffa, Nim hristopher and David J. Ralph St Peter s Department of ndrology, and The Institute of Urology, London, UK ccepted for publication 13 December 2007 Study Type Therapy (case series) Level of Evidence 4 OJETIVES To report our experience and management of genital lymphoedema, as this condition can be extremely debilitating and difficult to manage. PTIENTS ND METHODS The clinical records of 90 patients with genital lymphoedema who presented between 1998 and 2007 were retrospectively reviewed. The surgical management of 34 patients consisted of the excision of the affected skin and of the subcutaneous layers. This involved scrotal excision in 15 patients with primary closure. Skin grafts were required in seven patients for penile shaft cover. The results for cosmesis, recovery of sexual function, patient s satisfaction and complications are discussed. RESULTS In all, 56 patients were successfully managed conservatively by treating the underlying condition, antibiotic administration, compression and elevation of the genitalia. The remaining 34 patients required surgical management with an overall excellent cosmetic result and a significant improvement in sexual function. ONLUSIONS When surgery is necessary for genital lymphoedema, the new techniques described provide excellent cosmetic and functional results. KEYWORDS lymphoedema, male genitalia, cellulitis, skin grafting INTRODUTION Lymphoedema arises from the abnormal retention of lymphatic fluid in the subcutaneous tissues as a result of lymphatic obstruction. Genital lymphoedema can occur in isolation with the scrotum, penis or both being involved or it can be combined with generalized lower limb oedema (Figs 1,2; Table 1). Lymphoedema is classified as primary (idiopathic) due to an abnormal development of the subcutaneous lymphatic system or, more commonly, due to a secondary disorder [1]. s the lymphatic drainage of the penis and scrotum is predominately to the inguinal lymph nodes bilaterally, it usually presents only when both lymph nodes chains have been affected or when a local factor, e.g. genital surgery is involved. ommon causes of secondary genital lymphoedema are surgical interventions and trauma, radiotherapy, malignant infiltration, venereal diseases and parasitic infections all involving the inguinal lymph nodes [2,3]. Rarer causes include rohns disease, sarcoidosis, nonspecific vasculitis, rosacea, granulomatous lymphadenitis and following peritoneal dialysis [4 8]. Regardless of the cause, chronic genital lymphoedema can be extremely debilitating and difficult to manage. Recurrent episodes of cellulitis are common and are responsible for the loss of elastic fibres, hyperplasia of the collagenous connective tissue and the formation of fibrosis that renders the swelling permanent with progressive loss of function and of cosmesis [9]. In advanced cases, the skin is so thickened that the condition is known as elephantiasis (Fig. 3). The management of penile and scrotal lymphoedema is directed at correcting the underlying pathology, preserving sexual and voiding functions and providing an acceptable cosmetic result. Lymphangioplasty, the establishment of new lymphatic drainage patterns, is technically difficult and results have been disappointing [10 13]. When conservative management fails to either reduce the swelling or control the bouts of cellulitis, surgical intervention is often necessary [1]. This paper describes the surgical management of this condition. PTIENTS ND METHODS The clinical records of all patients who presented with genital lymphoedema between 1998 and 2007 were retrospectively reviewed. ll patients were assessed to obtain a diagnosis where possible. Most of the patients developed their lymphoedema after either surgery or radiotherapy and therefore did not need any further investigations. Other investigations on the remainder included haematological and serology tests for venereal and tropical diseases (the Venereal Disease Research Laboratory test, chlamydial antibodies, filariasis), tuberculosis and chronic inflammatory diseases markers (-reactive protein). ll excised tissue was sent for histological assessment to confirm the diagnosis. Urethral swabs were also taken where a sexually transmitted disease was suspected. Overall, 90 patients presented during this period with a mean (range) age of 53.9 (13 92) years. The aetiology of the lymphoedema is shown in Table 2. Where possible patients were managed conservatively with a penile compression bandage and elevation, a tight scrotal support, daily massage and the 480 JOURNL OMPILTION 2008 JU INTERNTIONL 102, doi: /j x x

2 FIG. 1. Idiopathic penile lymphoedema in isolation. (a) (b) (c) (d) rea affected Isolated penile Isolated scrotal ombined penoscrotal ssociated with lower limbs and infrapubic lymphoedema TLE 1 lassification of genital lymphoedema FIG. 2. ombined idiopathic penoscrotal lymphoedema. etiology Overall, n Surgical management, n Idiopathic fter penile surgery* 8 7 Infection 4 4 Inguinal lymphadenectomy 49 5 Radiation therapy 4 1 Inflammatory 7 5 Neoplastic infiltration** 1 1 Renal impairment 1 0 Total TLE 2 etiology of genital lymphoedema *Penile augmentation 3, venous ligation 1, Nesbit 1, circumcision 2, hypospadias repair 1; Filariasis 2, gonorrhoea 1, Fournier s 1; Penile cancer 48, urethral cancer 1; Penile cancer 3, seminoma testis 1; rohns 4, vasculitis 2, oeliac 1; **Non-Hodgkin lymphoma 1. FIG. 3. dvanced penoscrotal lymphoedema ( elephantiasis ), cause unknown. FIG. 4. dvanced genital lymphoedema, request was for genital reduction. TLE 3 Surgical procedures performed (some patients had combined procedures) Number of Procedure performed patients ircumcision 12 Partial scrotectomy 8 Total scrotectomy 7 Wide penile skin excision 12 Skin grafting to the shaft 7 FIG. 5. The inner preputial skin is never involved and therefore is preserved. administration of broad-spectrum antibiotics to prevent episodes of cellulitis. In 34 patients, surgical management was necessary, because either the local conservative management had failed or that they had presented with advanced disease with irreversible skin changes that made sexual and voiding function difficult. Others wished to have surgery to reduce the size of their genitalia and to improve cosmesis (Fig. 4). The type of surgery performed is shown in Table 3. To improve micturition 12 patients were circumcised. It was noted that the inner preputial skin was frequently not involved by the lymphoedema due to a separate lymphatic drainage and therefore it was preserved with more of the outer diseased skin being excised (Fig. 5). Penile lymphoedema was otherwise treated by local excision of the penile skin with undermining in areas to remove only the lymphoedematous subcutaneous tissue. In the advanced cases the penile skin had to be widely excised and the penis then covered by skin grafts (SGs), either full-thickness SGs (FTSGs) harvested from the nonhair-bearing skin of the axilla (two), the inner prepuce (one) or the flank [1], or split-thickness SGs (STSGs) harvested from the thigh (three). The scrotal lymphoedema was treated by partial JOURNL OMPILTION 2008 JU INTERNTIONL 481

3 GRFF ET L. scrotectomy in eight patients and by total scrotectomy in seven patients. With the increase in size of the scrotum, it was found that the unaffected lateral skin fold also became expanded. This allowed excision of the whole diseased scrotum with primary closure using the two lateral skin folds (Fig. 6 D). FIG. 6., The lateral scrotal skin folds are unaffected by the lymphoedema are marked., Scrotal inverted W incision., Scrotum totally excised. D, Primary closure of the scrotal folds. For the advanced pathology, these principles of total scrotal excision with primary closure with lateral scrotal folds is applied and where possible the inner preputial skin is utilized for penile shaft coverage. In patients where the inner preputial skin was either not available or insufficient additional SGs were necessary (Fig. 7 F). SGs are quilted on the penile shaft, a compressive dressing is applied and erections inhibited with cyproterone acetate for 1 week. The results of surgery were recorded to include patient satisfaction for cosmesis, functional outcome, complications, and revision surgery. D RESULTS In all, 34 patients had surgery with a mean (range) follow-up of 27 (1 68) months. The mean age of these patients was 46.6 (23 77) years; their aetiology is shown in Table 2. Of the 12 patients that had a circumcision, one developed a hypertrophic scar that has required surgical re-excision and another developed penile and scrotal swelling that settled conservatively with antibiotics and elevation. nother patient has subsequently developed penile lymphoedema that has been treated with the excision of the affected penile skin. The cosmetic result was improved when there was undermining and excision of the subcutaneous tissue adjacent to both sides of the incision line or when the inner preputial layer was spared. One patient, although happy with the results of surgery, has subsequently died. The remainder are happy with the cosmetic and functional result and 10 have resumed sexual activity with confidence. In the treatment of the penile lymphoedema by local excision, two patients developed ischaemic areas after extensive excision of the subcutaneous tissue (two of 10 patients). This was treated by delayed scar excision in one patient and by early skin grafting in the other. One patient had a planned two stage local excision to prevent this ischaemic complication. The remainder of the patients had an excellent cosmetic and functional result (Fig. 8,); only one of these patients is not sexually active. In the seven patients that had a primary excision and grafting, a FTSG has been used in four cases and a STSG in the remainder. There was an incomplete SG take in one patient who had a FTSG, a complete take occurred in the remainder. However, patients with a FTSG were more satisfied with the cosmetic appearance and function due to less graft contraction (Fig. 9). Keloid formation and SG contracture was common in patients that had STSGs (Fig. 9). The cosmetic result has been excellent in six of the seven patients with one patient being lost to follow-up. partial scrotal excision performed in eight patients did not eradicate the disease and in three of these patients a subsequent total scrotectomy was necessary. ll seven patients that had a total scrotal excision had an excellent cosmetic result with no complications. No recurrence of scrotal lymphoedema has occurred at follow-up in the latter group. It became apparent that when performing a total scrotectomy in patients with penoscrotal lymphoedema, there was spontaneous resolution of the penile lymphoedema (Fig. 10 ). Of the 15 patients that have had a scrotectomy, nine are now able to have sexual intercourse; of the remainder, one has had a total penectomy for penile cancer and therefore penetration is impossible and five are lost to follow-up. DISUSSION Genital lymphoedema is a rare condition but can be very debilitating for the patient. Every effort should be made to establish a diagnosis particularly if infection or an underlying malignancy is suspected. The management should be therefore primarily focused on treating the underlying diseases. In particular, venereal diseases should be treated with the administration of an adequate antibiotic [7], inflammatory diseases with systemic steroid therapy [12,13], electrolytic imbalances should be corrected promptly and parasitic infections managed with antifilarial drugs. The presence of neoplastic processes should be excluded. 482 JOURNL OMPILTION 2008 JU INTERNTIONL

4 FIG. 7., Extensive penoscrotal lymphoedema., Isolation of cords and shaft., Excision of all the affected penoscrotal tissue. D, Primary scrotal closure with lateral scrotal flaps. E, Penile shaft skin cover with inversion of the inner preputial layer and with FTSGs. F, efore and after surgery. FIG. 8. Penile lymphoedema before () and after undermining and excision (). D E F FIG. 9., FTSG to the shaft showing mobility and elasticity., STSG to the shaft showing scarring and contracture The initial management of the lymphoedema is conservative with compression and elevation of the genitalia and administration of broad-spectrum antibiotics to prevent recurrent bouts of cellulitis, which would eventually destroy the elasticity of the skin. onservative management in this series has been successful in 64% of patients. learly therefore the success of conservative management requires an early identification and treatment strategy of the condition. When considering surgical management of this condition, certain features have become apparent: (i) The inner preputial layer is never involved by the lymphoedematous process as the lymphatic drainage travels with the dorsal neurovascular bundle into the internal JOURNL OMPILTION 2008 JU INTERNTIONL 483

5 GRFF ET L. FIG. 10. Spontaneous resolution of penile lymphoedema after total scrotal excision (same patient as in Fig. 6)., efore surgery., 3 months after surgery., 6 months after surgery. efore surgery 3 months postoperatively 6 months postoperatively pudendal system whereas the remaining shaft skin drains to the inguinal lymph nodes. Therefore, the inner preputial layer should be spared if possible and surgery modified to allow this. (ii) Usually the outer lateral part of the scrotum is also not affected due to a separate lymphatic drainage, and therefore should be used for primary closure [14]. With time, this skin tends to stretch and resembles a normal scrotum. (iii) In penoscrotal lymphoedema the scrotum should be treated first as then there is often spontaneous resolution of the penile lymphoedema. (iv) When SGs are required to cover the penile shaft, these should be of thick type to minimize contracture and therefore preserve physiological girth and length expansion during erection. The use of STSG on the penile shaft should be avoided. (v) The lymphoedema only involves skin and subcutaneous tissue and does not involve uck fascia of the penis and the external spermatic fascia of the cord [15]. Total scrotectomy followed by reconstruction with lateral flaps yielded excellent cosmetic results and there were no recurrences during the follow-up. However, patients that had a partial scrotectomy have had recurrence of the lymphoedema requiring further surgery. In penile lymphoedema, the removal of lymphoedematous tissue has been followed by direct repair in the vast majority of patients. However, in seven patients the excision was so extensive that a SG was required for penile skin cover. The use of FTSG is superior to STSG but does require extensive wound surveillance to ensure an adequate successful outcome. These efforts are rewarded by the superior functional and cosmetic outcome. Erections are encouraged after hospital discharge to allow the tissue to stretch and become more pliable. In conclusion, genital lymphoedema, although rare, should be investigated to exclude significant pathology and treatment given early to preserve cosmesis and function. If treated early, most patients can be managed conservatively but those that need surgical intervention can be assured that an excellent cosmetic and functional result can be achieved with the techniques described. ONFLIT OF INTEREST None declared. REFERENES 1 olt RJ, Peelen W, Nikkels PG, de Jong TP. ongential lymphodema of the genitalia. Eur J Pediatr 1998; 157: Nelson R, lberts GL, King LE Jr. Penile and scrotal elephantisis caused by indolent hlamydia trachomatis infection. Urology 2003; 61: 224i iii 3 Porter W, Dineen M, unker. hronic penile lymphoedema: a report of 6 cases. rch Dermatol 2001; 137: Murphy MJ, Kogan, arlson J. Granulomatous lymphangitis of the scrotum and the penis. Report of a case and review of the literature of genital swelling with sarcoidal granulomatous inflammation. J utan Pathol 2001; 28: Silver HM, Tsangaris NT, Eaton OM. Lymphoedema and lymphography in sarcoidosis. rch Intern Med 1966; 117: Muhlemann MF, Walker NP, Tan L, hampion RH. Elephantine sarcoidosis presenting as ulcerating lymphoedema. J R Soc Med 1985; 78: braham G, lake PG, Mathews R, argman JM, Izatt S, Oreopoulos DG. Genital swelling as a surgical complication of contiuous ambulatory peritoneal dialysis. Surg Gynecol Obstet 1990; 170: Deshmukh N, Kjellberg SI, Shaw PM. Occult inguinal hernia, a cause of rapid onset of penile and scrotal edema in patients on chronic peritoneal dialysis. Mil Med 1995; 160: Morey F, Meng MV, Mcninch JW. Skin graft reconstruction of the chronic genital lymphoedema. Urology 1997; 50: Gillies MH, Fraser FR. Treatment of lymphoedema by plastic operation. MJ 1935; 96: Goldsmith HS, De Los Santos R. Omental transposition in primary lymphoedema. Surg Gynecol Obstet 1967; 125: McDonald DF, Huggins. Surgical treatment of elephantiasis. J Urol 1950; 63: Nielubowicz J, Olsewski W, Sokolowski J. Surgical lympho-venous shunts. J ardiovasc Surg (Torino) 1968; 9: Ketterings. Lymphoedema of the penis and scrotum. r J Plast Surg 1968; 21: Dandapat M, Mohapatro SK, Patro SK. Elephantiasis of the penis and scrotum. review of 350 cases. m J Surg 1985; 149: orrespondence: Mr David J. Ralph, onsultant Urologist, Institute of Urology, 145 Harley St, London W1G 6J, UK. dralph@andrology.co.uk bbreviation: (FT)(ST)SG, (full-thickness) (split-thickness) skin graft. 484 JOURNL OMPILTION 2008 JU INTERNTIONL