Introduction

Pressure sores remain a common problem in bedridden patients, 25–85% incidence.1 Chronic wounds and scar tissues are prone to an increased risk of skin cancer, known as Marjolin’s ulcer. The commonest type of carcinoma arising from these lesions is squamous cell carcinoma with a bad prognosis.2

Case report

Marjolin’s ulcer was observed in a 40-year-old patient with Von Hippel Lindau syndrome leading to permanent paraplegia due to hemangioblastomas.

At 25 years of age he developed a gait ataxia. A contrast-enhanced magnetic resonance imaging (MRI) scan of the brain and spinal cord evidenced lesions within the posteroinferior portion of the fourth ventricle and the second, third, sixth and twelfth dorsal segments, with hemangioblastoma characters. Ventricular lesion was removed and an improvement of the gait disturbance was achieved. After 1 year, he developed an acute paraplegia with sensitive T9 level and loss of sphincteric control. A contrast-enhanced MRI disclosed an increase in volume of the intramedullary lesion at T10 with cord swelling. The patient was treated in emergency by a T9–T11 decompressive laminectomy followed by the tumor removal.

Postoperative MRI did not show complications.

Nonetheless, he never experienced a neurological recovery from the admission deficit. He developed multiple, unstable healing ulcers after 2 years.

In January 2012 the patient was admitted in our Department.

He presented lesions over the left ischiatic, trochanteric, bilateral and sacral areas (III/IV grade, National Pressure Ulcer Advisory Panel) and fistula abscess in the posterior region of the left thigh (Figure 1).

Figure 1
figure 1

Marjolin’s ulcer over the left ischiatic region, trochanteric, bilateral and sacral areas (III/IV grade, National Pressure Ulcer Advisory Panel) and fistula abscess in the posterior region of the left thigh.

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Malignant degeneration features were noticed on the ischiatic ulcer and biopsies were performed.

The histological examination identified a squamous cell carcinoma only on the left ischiatic ulcer.

The contrast-enhanced computed tomography (CT) and MRI scans did not show any systemic involvement.

The complicating factors included severe malnutrition, poor physical state and infection by a multiresistant germ (Pseudomonas aeruginosa). A specific antibiotic therapy with ciprofloxacin was started.

A defunctioning colostomy was performed to minimize the fecal contamination.

We opted for a radical surgical treatment.

The lesions were radically excised until bone (with oncological radicality). A bone biopsy was sent for a bone culture to guide the antibiotic therapy for osteomyelitis (the examination of bone culture resulted negative).

We previously disarticulated the flap.

An incision was made along the posterior aspect of the left thigh, identifying the lateral intermuscular septum.

This incision was continued beyond the popliteal fossa until tibial peroneal trunk. The periosteum over the femur was raised and the flap was raised off the entire length of the femur.

The articular capsule was divided at its attachment to the pelvis and division of the ligamentum teres completed the disarticulation.

The periosteal and muscular elevation was performed to close the acetabular cavity.

The total thigh flap (TTF) was turned on itself, and the deep muscles sutured into place.

We preferred to use a musculocutaneous rotational flap from the left thigh (anterior, medial and lateral regions) until the proximal third of the leg to cover the ischiatic left ulcer and sacral area. A tubularized flap extension covered the contralateral trochanteric defect (Figures 2 and 3).

Figure 2
figure 2

Postoperative result. A musculocutaneous rotational flap from the left thigh (anterior, medial and lateral regions) until the proximal third of the leg covered the ischiatic left ulcer and sacral one.

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Figure 3
figure 3

Post-operative result. A tubularized flap extension covered the contralateral trochanteric defect.

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After 2 months of air-fluidized bed, the patient was discharged to the rehabilitation department.

The patient is disease-free yet (24 months of follow-up), CT scan and MRI with contrast medium did not show any systemic and local involvement.

Discussion

Pressure sores are one of the most common secondary conditions in paraplegic patients.

The incidence of chronic non-healing ulcers undergoing malignant transformation is a rare phenomenon, 0.5% incidence.3

Awareness of the malignant potential may help decrease the morbidity.

In the clinical history, it is important to analyze the morphological changes through the most appropriate diagnostic techniques, as in the advanced stages even an aggressive surgical procedure may prove inadequate.

First-line treatment consists of radical excision with a range of 2 cm from the margin of the ulcer and potential lymph node dissection (according to fine-needle aspiration cytology and CT scan).

Adjuvant radiation therapy may be used; however, the response to systemic chemotherapy is generally poor.4 In our case, according to radiotherapist we decided not to perform radiotherapy. Management of massive pelvic defects can be a challenging problem. The pedicled lower limb flap offers a last resort technique for extensive defects where other options are insufficient or not available anymore.

The TTF is a well-vascularized myocutaneous flap and provides valuable viable tissue, improving the status of the paraplegic patient with multiple pressure ulcers.5

We demonstrated the necessity of rigorous surgical treatment because the clinical course of Marjolin’s ulcer is rapid and fulminant.

Patients should be followed up for the rest of their life.

As a result of 24-month follow-up, our patient is disease-free.