Partial Penectomy for Severe Penile Calciphylaxis: A Case Study
- 1. Department of Urology B, Mohamed V University, Morocco
Abstract
Penile calciphylaxis, a rare occurrence of calcific uremic arteriolopathy, is not frequently addressed in the literature. Conservative and surgical management have been found to have similar outcomes regarding mean survival time, leading to controversy around the advantages of surgery for this illness. This report showcases an ESRD patient dependent on hemodialysis who experienced severe penile calciphylaxis that made conservative treatment unfeasible. The case required immediate intervention through penectomy as a means of stopping disease advancement and enhancing quality of life for the patient.
Keywords
• Penile calciphylaxis
• Penectomy
• Glans necrosis
• Doppler ultrasound
CITATION
Maachi Y, Boustani A, Lalaoui AS, Slaoui A, Karmouni T, et al. (2024) Partial Penectomy for Severe Penile Calciphylaxis: A Case Study. J Urol Res 11(3): 1153.
INTRODUCTION
Calcific uremic arteriolopathy (CUA), otherwise known as systemic calciphylaxis, is a severe and uncommon disease that carries high risks of morbidity and mortality. It mainly affects patients with end-stage renal disease (ESRD) who undergo chronic hemodialysis, with an incidence rate ranging from 1% to 5% [1]. Factors like diabetes mellitus, obesity, calcium and vitamin D supplementation use of warfarin medication may also increase the risk for CUA in this patient population. Additionally, hypercoagulable states characterized by protein C/S deficiency can further heighten the probability of contracting this condition. Currently not fully comprehended lies within its pathophysiology; it seems linked to metabolic dysregulation involving phosphorouscalcium imbalances attributed to ESRD-induced secondary hyperparathyroidism leading up medial arterial calcification culminating thrombosis followed by small arteriole obliteration towards complete blockages via blood clots or vessel constriction [1]. Penile involvement has been identified as poor prognostic indicator but rarely reported due rarity observation among other medical cases complications occurring during progression stages such advanced penile calciphylaxis emphasize their severity when found co-occurring wit ERSR on onset at diagnosis state receiving various dialysis treatments options personalized care plans tailored needs each individual’s case are mandatory subduing chances associated ischemia too lengthy curfews stymie healing processes caused erectile dysfunction ultimately causing paralysis if untreated acting fast immediate treatment intervention important adopting healthy lifestyle change dietary restrictions urged achieving optimum wellness levels mitigate penalties incurred long terms negative health consequences arising hospitalization seeking medical advice early detection aids better management outcomes successfully reducing undesirable experiences without need undergoingj complex surgical procedures exacerbating situations earlier predictions approximate timeframes reemerging symptoms could illustrate predicting responding diverse stimuli guiding regulation dosage scheduling medications requiring constant attention update monitoring dosed personalised approach targeting cause source laying foundation successful recovery rates reaching beyond expectations without supporting instruments eyebrows raising hopes unprecedented breakthroughs redefine possible realms .
CASE PRESENTATION
A 60-year-old man who has been suffering from type 2 diabetes for a long time, chronic kidney disease that necessitated hemodialysis for five years; and hypertension with previous peripheral vascular ailment is being described. He went to the emergency department due to severe penile pain accompanied by glans necrosis [Figure 1].
Figure 1: Intraoperative picture showing the distal glans entirely necrotic, with the proximal shaft remaining viable.
The person’s key indicators revealed a temperature of 37.2°C, heart rate registering at 90 bpm, and blood pressure measuring in at 150/85 mmHg. Results from blood tests indicated heightened levels of calcium and phosphate as well as elevated inflammatory markers alongside high serum urea and creatinine counts.
Penile calciphylaxis was diagnosed as a result of chronic kidney disease and diabetes after reduced blood flow consistent with vascular calcification was documented through Doppler ultrasound [Figure 2].
Figure 2: Doppler ultrasound revealed evidence of vascular calcification. (Arrow).
To manage the condition, pain was managed using opioids and wound care measures were taken. Additionally, phosphate binders were administered along with low-calcium dialysate during hemodialysis to further aid in treatment. Moreover, necrotic tissue was removed through surgical debridement procedures. Because the condition was severe, a partial penectomy was necessary to eliminate the necrotic tissue and halt any further advancement of gangrene. After being admitted to the hospital for careful observation, he received surgery and medical treatment that stabilized his condition. To manage this long term, it’s crucial to diligently regulate calcium-phosphate levels and receive regular dialysis while prioritizing timely wound care. Recognizing symptoms early on and treating aggressively is key in avoiding serious complications or even death related to such cases.
DISCUSSION
Calciphylaxis, or calcifying uremic arteriolopathy (CUA), is a rare and serious condition that mainly affects patients with end-stage renal disease (ESRD) on dialysis. It is characterized by calcification of small and medium-sized blood vessels, leading to ischemia and necrosis of cutaneous and subcutaneous tissues. Calciphylaxis has a high mortality rate, often exceeding 50% within a year of diagnosis, mainly due to sepsis and complications associated with extensive skin ulcers.
The pathogenesis of calciphylaxis is complex and multifactorial. It involves disturbances in calcium and phosphate metabolism, endothelial dysfunction and pro-inflammatory states. Key factors include hyperphosphatemia and hypercalcemia due to chronic kidney disease (CKD), secondary hyperparathyroidism, reduced matrix Gla protein (MGP) which is a potent inhibitor of vascular calcification, warfarin use which inhibits MGP activation, and chronic inflammation accompanied by frequent endothelial damage in CKD.
Penile calciphylaxis is an uncommon but life-threatening complication of systemic calciphylaxis, which can lead to tissue death, gangrene and potentially fatal sepsis. In patients with end-stage renal disease (ESRD) or diabetes, the prognosis for penile ischemic gangrene is particularly poor, with a mortality rate exceeding 60% within six months [2]. Symptoms that may be present during clinical examination include painful lesions in blue-purple coloring; hardened knots underneath the skin; ulcers; as well as necrotic changes to either shaft or tip of penis [3]. Although laboratory investigations sometimes reveal higher levels of parathyroid hormone along with abnormalities related calcium and/or phosphate concentration in blood serum among affected individuals- our patient failed showed these particular irregularities [4].
The main risk factors for calciphylaxis are ESRD, diabetes mellitus, obesity, female gender and long-term warfarin therapy. Clinically, calciphylaxis presents as painful purpuric lesions progressing to non-healing ulcers, mainly located on the lower limbs, abdomen and thighs. These lesions can become necrotic and gangrenous, leading to significant morbidity.
The diagnosis of calciphylaxis is based primarily on clinical examination, supported by skin biopsies revealing calcifications in the media of small arteries and arterioles, intimal hyperplasia and thrombosis. Imaging techniques can also reveal vascular calcifications.
The treatment approach for penile calciphylaxis, a rare condition affecting the penis due to calcium deposition in blood vessels, is similar to that of non-penile cutaneous CUA and less frequently observed noncutaneous CUA with accompanying skin lesions. In milder cases of penile calciphylaxis, conservative management involves local wound cleaning procedures alongside pain relief measures and regulation of metabolic dysfunctions. Sodium thiosulfate (STS) possessing antioxidant properties along with chelation therapy as well hyperbaric oxygen therapy aiding skin recovery have shown promise in treating patients presenting with other types of CUA resulting from the accumulation of calcium deposits; hence their application extends towards instances involving penile tissue. However further exploration is essential regarding its effectiveness on this particular ailment manifestation. For those encountering severe hyperparathyroidism contributing significantly toward excessive depositing processions within body systems — parathyroidectomy serves as an alternative option although it’s overall mortality benefits do not conform likewise amongst medical experts universally [5].
Surgery is an option for treating penile lesions, and can involve either a partial or total removal of the penis with urinary diversion. However, because such involvement typically suggests advanced systemic calciphylaxis and has a high risk of mortality, whether surgery provides significant benefits remains contentious in scientific writing. While some studies have examined this issue extensively, they show no clear advantages to penectomy as opposed to more conservative options when it comes to extending lifespan before death occurs [3,5]. Additionally, patients who are immunocompromised or have multiple health problems may face greater danger from both operative complications and post-surgical risks related to any type of operation on their genitals.
Despite this, there is a proposal to consider penectomy as a potential life-saving measure for patients with high risk of progression towards gangrene and sepsis. Furthermore, it has been recommended in order to enhance the quality of life by alleviating physical and psychological symptoms associated with gangrene such as constant pain and spontaneous penis amputation. As such, decision-making regarding whether or not penectomy is an appropriate treatment option should be made on a case-by-case basis.
Upon assessing our patient, we determined that the severity of his pain, necrosis and gas foci on imaging necessitated surgical intervention. Conservative management was not a viable option due to the extent and seriousness of his condition - identifiable intraoperatively by pus-filled corpora revealing partial penectomy as an imperative measure. It should be noted that this decision path took into consideration the patient’s full code status; he desired definitive treatment which would halt infection spread and alleviate discomfort hence prioritizing surgical rather than conservative methods for symptom relief. Unfortunately data is limited concerning recurrence rates in proximal areas following either method partly due to high disease mortality and lack of follow-up protocols beyond discharge timeframes.
The prognosis of calciphylaxis is generally poor, with high mortality rates due to complications such as sepsis and cardiovascular events. Early recognition and a comprehensive treatment strategy are essential to improve patient outcomes.
CONCLUSION
The severity of penile calciphylaxis demands urgent attention as it can lead to fatalities. In cases with a high likelihood of disease progression, surgical intervention such as penectomy should be considered for infection control and betterment of patient’s well-being. Our conclusion after careful evaluation was that partial penectomy served as an appropriate management option in this critical scenario.
Authors contribution Maachi
Youssef analyzed and interpreted the patient data regarding the subject and were major contributors in writing the manuscript, amine slaoui, tariq karmouni , Khalid El Khader, Abdellatif Koutani and Ahmed Ibn Attya Andaloussi read and approved the final manuscript.
Ethics approval and consent to participate
The ethics committee of the Faculty of Medicine of Rabat has given us its agreement. Informed and verbal consent to participate in the study was provided by our patients. The reference number is not applicable.