Isolated scrotal lymphedema in a 43-year old male patient: A case report

Abstract

Introduction

Lymphedema of the external genitalia is a rare condition characterized by swelling of the scrotal skin and subcutaneous tissue, resulting from a pathology in lymphatic drainage. Over time, the development of fibrosis leads to a considerable impairment in the patient's quality of life. While conservative management is generally the first-line approach, surgical cases may necessitate surgical intervention to achieve comprehensive and lasting improvements.

Case presentation

We present the case of a 43-year-old obese male patient who presented to the clinic with a complaint of persistent bilateral scrotal swelling for three months. Clinical examination revealed a pressure-indolent, soft, and massively enlarged swelling of the scrotum on both sides. Ultrasound findings confirmed a diffusely thickened edematous scrotal wall. The patient was advised to start physiotherapy and adhere to conservative management. Due to the debilitating size of the mass, the patient opted for excision of the scrotal swelling followed by scrotoplasty.

Clinical discussion

This case report explores the presentation, signs and symptoms, impact on patients' lives, and various management options for scrotal lymphedema. It underscores the intricacies involved in the diagnosis and treatment decision-making process, emphasizing the need for a tailored and multidisciplinary approach.

Conclusion

It is imperative to initially rule out life-threatening causes of scrotal lymphedema to ensure optimal patient care. The integration of surgical interventions should be carefully considered in the overall management strategy for optimal and comprehensive results. Scrotoplasty, in the context of scrotal lymphedema, not only improves the quality of life but also positively influences sexual function.

Competencies

Interpersonal and communication skills, Medical knowledge, Patient care, Practice-based learning and improvement.

Highlights

• •There are limited cases reported on the presence of isolated scrotal lymphedema.
• •The most common etiologies for scrotal lymphedema include idiopathic, localized lymphedema secondary to morbid obesity, lymphatic filariasis, and genital hidradenitis.
• •Conservative management encompasses lymphatic massage, while surgical management involves excision with scrotoplasty and reconstruction.

1. Introduction

Lymphedema, characterized by abnormal lymphatic fluid accumulation, presents as chronic swelling in one or multiple regions, accompanied by potential tissue changes. Swelling may occur in proximal regions or distal extremities. Manifestations include heaviness, pain, skin discoloration, restricted range of motion, and difficulty in ambulation [1,2].

Scrotal lymphedema, a rare condition, results in swelling of the scrotal skin and subcutaneous tissue due to lymphatic vessel dysplasia or obstruction. Over time, fibrosis develops, impacting the patient's Quality of Life (QOL) [2,3]. First-line management involves a conservative approach, with physiotherapy proving beneficial in the early and milder stages, improving the QOL. Conservative therapy, often referred to as Complex Decongestive Therapy (CDT), includes Manual Lymph Drainage (MLD), skin care management, therapeutic exercises, compression garments, bandaging treatment, and importantly, self-education [4]. In cases where ineffective conservative management, surgical intervention is considered. Early diagnosis is crucial for proactive lymphedema management, preventing and reducing recurrence risk [5]. This case report focuses on idiopathic scrotal lymphedema, adhering to the SCARE Criteria [6].

2. Case report

A 43-year-old male, classified as obese with a Body Mass Index (BMI) of 44.75 kg/m², presented to our institution with massively enlarged bilateral scrotal swelling. The swelling, painless and onset three months prior, extended to the knees, causing difficulty in walking. The patient denied scrotal trauma, difficulty in urination, recent weight changes, or recent travel. There was no history of previous radiation exposure, and past medical, family, and social history were unremarkable. The patient had undergone inguinal hernia repair, although he could not recall the year. Additionally, no previous lower urinary tract infections were reported.

In October 2022, the patient sought medical attention in Europe for the swelling. Methylene blue stain and scar release surgery were performed, but it was unsuccessful as the swelling persisted. Within one year, he returned to the outpatient clinic in Dubai, United Arab Emirates, complaining of recurrent swelling.

Physical examination revealed pressure-indolent, soft, massively enlarged swellings on both sides. The swellings were non-tender, non-pitting, and non-reducible, with fibrotic and indurated scrotal tissue texture. Non-irritant thickened scars were observed bilaterally in the groin, and the penis was retracted (Fig. 1, Fig. 2). Evaluation of the testicles was challenging. The patient had normal external genitalia development. Transillumination tests were negative, revealing no testicular swelling or hydrocele findings on either side. Ultrasound (US) findings of the testis showed significant diffuse edema involving the entire scrotum, with no evidence of hydrocele or varicocele. The left testicle appeared relatively small and hypoechoic, with a diffusely thickened edematous scrotal wall (Fig. 3). Doppler US of the scrotum demonstrated good vascularity to both testes. Lymphoscintigraphy-Tc99m-NNC scan (Fig. 4, Fig. 5), revealing persistent visualization of the right popliteal lymph node along with the formation of collaterals, indicative of lymph being rerouted through the system, potentially leading to future lymphatic obstruction.

Fig. 1.

Clinical scrotal appearance (standing view).

Fig. 2.

Clinical scrotal appearance (supine view).

Fig. 3.

Ultrasound of the testes.

Fig. 4.

Lymphoscintigraphy-T99m-NNC.

Fig. 5.

Lymphoscintigraphy-T99m-NNC.

Considering the clinical presentation, a differential diagnosis was formulated, including bilateral idiopathic scrotal lymphedema, massive localized lymphedema secondary to morbid obesity, lymphatic filariasis, lymphedema secondary to hernia repair surgery, and adult acquired buried penis. Polymerase chain reaction tests for Treponema pallidum, Chlamydia trachomatis, and filarial antibodies were all negative. Tumor markers, including total beta-human chorionic gonadotropin and alpha-fetoprotein, were also negative. After excluding other causes, a preliminary diagnosis of bilateral idiopathic scrotal lymphedema was established.

The patient was advised to attempt weight loss and initiate CDT sessions to enhance lymphatic fluid transport, yielding some improvement. However, challenges in weight loss and ongoing swelling prompted mutual agreement for surgical intervention. Post-operatively, CDT sessions continued. The surgical procedure only included scrotal mass excision, as per the patient's preference, and scrotoplasty with the plastic surgery team's assistance.

In July 2023, scrotal excision surgery was performed using a median raphe incision from the penis base to the perineum, preserving underlying structures—urethra, testicles, and inguinal cords. The excised tissue, weighing approximately 1.3 kg, was removed in two wedge-shaped parts for histopathology. The largest piece measured 11.5 cm × 13.0 cm × 9.0 cm, while the smallest piece measured 9.0 cm × 5.5 cm × 2.5 cm, displaying a pale brown cut surface. The specimen displayed edematous stroma, hypertrophied muscle bundles, lobular perivascular lymphocytes, plasma cell infiltrate, evidence of hemorrhage, and vascular congestion. Malignancy and granuloma were ruled out, with the histomorphological features confirming scrotal lymphedema. Scrotoplasty with an inverse T-incision and suturing the redundant skin achieved an acceptable scrotal contour (Fig. 6).

Fig. 6.

Post-operative appearance.

Post-operatively, the patient was advised on lifestyle modifications, emphasizing good personal hygiene, exercise, and healthy eating habits. A follow-up appointment was recommended after three weeks, but the patient returned six weeks later for personal reasons. During the visit, it was observed that the wound had healed without signs of infection, and there was no evidence of lower urinary tract symptoms. However, the patient still had an unresolved buried penis. Despite normal bladder habits, challenges in sexual life persisted, and dissatisfaction with QOL following the surgery was expressed.

3. Discussion

Scrotal lymphedema results from abnormal lymphatic fluid in the scrotal tissue due to compromised drainage. Lymphedema is broadly categorized into primary and secondary types [7]. Primary lymphedema, arising from lymphatic system malformation, includes three subtypes: Congenital Lymphedema, presenting at birth; Lymphedema Praecox, manifesting during adolescence; and Lymphedema Tarda, emerging after age 35 [8]. Secondary lymphedema, more prevalent and occurring at any age, develops due to damage to the lymphatic system and is associated with underlying medical conditions or procedures such as cancer treatment, trauma, obesity, chronic venous insufficiency, and infections like lymphogranuloma venereum and filarial infestation with Wuchereria bancrofti, prevalent in tropical countries [3,8].

The rising prevalence of lymphedema is closely associated with an increasing rate of morbid obesity, as demonstrated in two notable studies [9]. A 2014 study led by Mehrara BJ et al. highlighted the reciprocal relationship between obesity and secondary lymphedema, revealing that obesity impairs lymphatic transport capacity and results in adipose tissue accumulation [1]. Additionally, a 2022 study by Sudduth CL et al. found that obesity-induced lymphedema is more likely with a BMI exceeding 40, significantly increasing when BMI surpasses 60 [10]. Noteworthy risk factors also include congestive heart failure and diuretic use. Clinically, scrotal lymphedema presents as a large, painless, slow-growing soft tissue mass, causing discomfort, ambulation difficulties, and cosmetic concerns, with fibrosis potentially leading to genital malformations impacting daily activities [2,9].

Diagnosis is primarily clinical, although US scans and magnetic resonance imaging may be considered to rule out other potential etiologies. Untreated scrotal lymphedema, due to its chronic and slow-progressing nature, can significantly diminish the patient's standard of living. Lifelong care and psychosocial support are crucial [11]. The management of chronic scrotal lymphedema requires a comprehensive approach, integrating conservative and surgical methods. A precise and prompt diagnosis is imperative to proactively address lymphedema, preventing complications and reducing the risk of recurrence.

Implementing a multifaceted strategy combining conservative and surgical interventions optimizes patient outcomes and enhance the overall efficacy in scrotal lymphedema management. Regardless of the chosen approach, maintaining meticulous self-hygiene is crucial to prevent complications like infection. Patient education, along with daily exercises like walking and stair usage, is pivotal for successful treatment. Emphasizing adherence to recommended practices and fostering a comprehensive understanding of the chosen management strategy is paramount for optimal outcomes [12].

The primary approach to managing scrotal lymphedema typically involves conservative methods, especially physiotherapy techniques in the early stages and milder cases [4]. Conservative therapy follows a comprehensive approach known as CDT, consisting of two stages. The first stage includes skin care, MLD, muscle-pumping exercises, and compression therapy to improve lymphedema volume. The second stage focuses on optimizing stage one results and preventing complications through continued techniques from the first stage [12].

When conservative management proves inadequate, surgical intervention becomes a consideration [12]. Surgical management is classified into physiological or reductive methods. Physiological methods aim to improve lymphatic drainage and reduce swelling through reconstructive procedures like Vascularized Lymph Node Transfer (VLNT) and Lymphaticovenular Anastomosis (LVA). VLNT involves transplanting healthy lymph nodes and associated blood vessels to the affected area, while LVA connects lymphatic vessels to nearby venules. Reductive methods address complications of lymphedema such as lipodystrophy and fibrosis. In these cases, edematous and fibrotic tissue is either directly excised, ablated, or subjected to liposuction; note that lymph flow is not restored with this method. In advanced stages, physiological and reductive procedures can be combined for a more comprehensive and effective approach [5].

Patients with scrotal lymphedema are advised to seek psychosocial support, including psychotherapy or consultation with psychologists, to enhance their overall QOL.

4. Conclusion

Scrotal lymphedema, a rare condition stemming from primary or secondary lymphedema, necessitates prompt diagnosis and management due to its substantial impact on patients' QOL, restricting movements.

Consent

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Provenance and peer review

Not commissioned, externally peer-reviewed.

Ethical approval

Written consent was obtained from the patient for publication, and our study was deemed exempt from ethical approval at our institution.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Author contribution

Ghazal Talal Saeed: contributed to the conception and design of the study, drafted the article, and critically revised it for important intellectual content, final approval of the version to be submitted.

Donia Ahmad: contributed to drafting the article, and critically revised it for important intellectual content, final approval of the version to be submitted.

Montaser Nabeeh Al Smady: contributed to the conception and design of the study, and drafted the article.

Gunjan Awatramani: contributed to the conception and design of the study and acquisition of data of the study, and drafted the article and revised it.

Tariq Abdul Hamid: contributed to the acquisition of data of the study.

Farhad Janahi: critically revised the manuscript for important intellectual content, and gave the final approval for the study to be submitted.

Guarantor

Farhad Janahi.

Research registration number

Not applicable.

Conflict of interest statement

No conflict of interest.