Radial Periosteal Distraction as a Novel Intervention for Raynaud Syndrome with Gangrene: A Case Report

Introduction

Raynaud syndrome, also known as Raynaud phenomenon, occurs more frequently in women than in men, affecting about 20% to 30% of women, particularly in younger age populations [1]. Raynaud syndrome typically affect the fingers (distal areas). Patients with Raynaud syndrome experience vasoconstriction, pin and needles sensation, numbness, finger aches, or pain, which are common symptoms when the patients are stimulated by hypothermia or emotional stress. In severe cases, digital ulcers and gangrene commonly occur in secondary Raynaud syndrome, accompanied by vascular structural changes [2]. Nailfold capillary microscopy is used as a diagnostic method for Raynaud syndrome. Conservative treatment and pharmacological and surgical therapies can slow the progression of ischemic tissue damage and improve the quality of life to some extent [3–5]. However, there is still a lack of reliable methods to cure the disease.

Periosteal distraction technology is based on the periosteum’s mechanism in distraction osteogenesis. Mesenchymal stem cells, osteoblasts, microvessels, and growth factors are found in great abundance in the periosteum. The periosteum, in the process of distraction osteogenesis, can significantly stimulate angiogenesis and provide rich nutrients for the healing of fractures [6,7]. Several animal studies have also demonstrated that the distraction of the periosteum facilitates peripheral vascularization and promotes the growth of bone formation [8–11]. Based on this theory, researchers have applied the periosteal distraction technique in the treatment of diabetic foot and achieved better therapeutic effects [12,13]. This technique is feasible for diabetic foot. The slow and continuous low mechanical distraction stress promotes the expression of vascular endothelial growth factor, basic fibroblast growth factor, epidermal growth factor, and chemokine stromal cell-derived factor-1, which may be one of the mechanisms that promote diabetic foot wound healing [14,15]. In reported cases, radial cortex transverse distraction has been used for the management of Raynaud syndrome. However, the creation of a rectangular osteotomy was needed and took more recovery time [16]. This report describes a 67-year-old woman with a 10-year history of Raynaud syndrome presenting with ischemia and gangrene of the right index and middle fingers that was managed with periosteal distraction osteogenesis.

Case Report

A 67-year-old female patient was admitted to the hospital with a more than 10-year history of pain in both hands after exposure to cold water. The pain was aggravated by gangrene of the right finger for 1 week prior to presentation. Ten years earlier, the patient had no obvious cause for numbness and pain in both hands after touching cold water. The numbness and pain was accompanied by pallid and blue skin, lasting about several hours, and gradually returning to normal. Then, she received a diagnosis of Raynaud syndrome and was given oral nifedipine extended-release tablets 10 mg once a day. However, the above symptoms still occurred. Two weeks before presentation, the patient was admitted to our Intensive Care Unit for resuscitation of infectious shock. After active anti-infection treatment, fluid replacement, and maintenance of a water-electrolyte balance, the patient was awake, and the shock was corrected. One week prior to presentation, the patient felt a worsening of the pain in both fingers, which was accompanied by darkening of the distal ends of the right index and middle fingers. Therefore, she came to the Department of Orthopedics of our hospital. The patient had no history of smoking, alcohol consumption, substance abuse, hypertension, diabetes mellitus, coronary artery disease, or other chronic diseases. There were no obvious abnormalities in laboratory test results, including complete blood count, liver function, renal function, electrolytes, coagulation function, thyroid function, and C-reactive protein. Immune-related indicators, such as antinuclear antibody, anti-extractable nuclear antigen antibodies, and anti-cyclic citrullinated peptide antibody, were all within the reference range. The transcutaneous oxygen pressure in the right hand was 13.3 mmHg (normal value >40 mmHg). The results of her cardiac ultrasound, electrocardiogram, and chest computed tomography (CT) imaging were essentially normal.

Physical examination revealed that the patient had pale skin on both hands and purple-black skin on the ends of the right index and middle fingers, with pronounced pressure pains (Figure 1). Ulnar and radial artery pulsations were accessible and weak. CT angiography of the upper limb showed no obvious small vessels in the distal part of the right fingers. Rapid progression of gangrene and obvious pain in the right finger revealed that drug treatment was ineffective; therefore, the patient requested surgical treatment. The gangrene boundary was unclear; therefore, radial osteotomy was considered first to improve microcirculation, and then, necrotic finger extirpation was planned for once the necrosis boundary was established. The right radial periosteal distraction surgery was performed after the patient and her family signed the informed consent.

The patient was scheduled for a right radial periosteal distraction technique under regional anesthesia. After disinfection and surgical towel laying, a Henry incision was made on the palmar side of the right forearm. From the radial artery and radial wrist flexor muscle gap, the distal radius flat part of the lateral incision of the anterior rotator muscle and the periosteum was selected. The periosteum was gently peeled to avoid tearing, and a plate was inserted and fixed with screws. Decompression of the medullary cavity by drilling around the steel plate was performed (Figure 2). A C-arm fluoroscopy confirmed that the retractor plate was well positioned; then, the periosteal muscles were sutured, and the incision was closed (Figure 3). The finger ends were ill-defined and had dry gangrene, without any signs of active infection; therefore, no debridement was performed.

During the postoperative period, the distraction of the periosteum was initiated on the second postoperative day by twisting the knob clockwise at a rate of 0.25 mm/12 h (half a turn/turn) per day. On the third postoperative day, the patient felt that the distal pain was relieved, and the skin temperature became warmer. The patient was discharged from the hospital and distracted the periosteum on her own. The dressing at the distraction site was changed every 3 days, and the screws were disinfected with 75% alcohol to prevent infection. Continuous microcirculation recordings of the right hand were made at 1 month, 3 months, 6 months, and 1 year after surgery (Table 1). The patient returned to the hospital after 1 month of distraction, with no progression of gangrene and clear boundaries at the ends of the right fingers. CT angiography revealed a large number of small blood vessels at the ends of both fingers 1 month after the surgery (Figure 4), and the retractor screws and plates were removed. Three months after surgery, most of the necrotic tissue in the affected finger was shed, and normal tissue grew. At 6 months after surgery, the patient occasionally felt a little numbness at the end of the finger but had no pain, and no activity limitation. The gangrene at the end of the right hand finger recovered, and a little scab hyperplasia remained in the finger. One year after surgery, the affected finger was mildly shortened in shape. However, the skin was intact and warm, the joint could move freely, and the finger numbness was significantly reduced while touching cold water. In addition, the numbness and pain in the patient’s left fingers in cold water significantly improved (Figure 5).

Discussion

In this case, radial periosteal distraction was first applied to the treatment of Raynaud syndrome with gangrene. It effectively promoted the healing of ulcers, improved the microcirculation of fingers, and significantly alleviated the pain in both hands. This suggests that this technique can provide a new surgical treatment strategy for severe Raynaud syndrome of the digits. Raynaud syndrome is a vasospastic disorder primarily affecting the fingers and toes. It is usually associated with cold environments, emotional distress, or stress. This results in a reduced blood supply to the affected area, causing localized skin color changes. The condition comes in 2 types: primary Raynaud syndrome, which is a sensation of cold and pain caused by vasoconstriction, and secondary Raynaud syndrome, which usually occurs in connection with autoimmune disease [17].

Generally, primary Raynaud syndrome is a benign condition. Due to the relatively mild distal ischemia, it usually does not cause irreversible tissue injury. However, chronic misuse or overuse of vasoconstrictive medications, exposure to extreme cold, or engagement in occupations involving operating vibrating tools can exacerbate vascular spasm and induce severe ischemia in these patients. If Raynaud syndrome progresses, calcium channel antagonists, alpha-blockers, and other vasodilators can be used. However, some patients with unsatisfactory drug therapy need surgical interventions, including a thoracolumbar sympathectomy, nerve blocks, arterial epithelial stripping, or spinal cord stimulation [18–20]. It is true that surgical treatment can improve symptoms to a certain extent, but the surgery is challenging for the surgeon, highly individualized, and not curative. Once the limbs have entered the necrotic stage, the treatment is ineffective. Therefore, we need to find more effective ways to treat Raynaud syndrome.

Based on our patient’s medical history, typical symptoms and signs, and the results of laboratory and imaging examinations, primary Raynaud syndrome was considered in the present case. The patient developed ischemia and gangrene in her fingertips 1 week before presentation, which progressed rapidly. Further analysis revealed that the patient had been hospitalized 2 weeks before presentation, in an infectious shock coma. The patient’s medication history at that time showed that high-dose dopamine therapy was administered; therefore, it was hypothesized that the vasoconstrictor effect of dopamine caused the Raynaud syndrome to worsen in a short period, eventually resulting in gangrene of the fingertips. Although gangrene rarely occurs in primary Raynaud syndrome, the patient’s experience of high-dose vasoconstrictive medications might have been the reason. It is believed that small vessel lesions are responsible for Raynaud syndrome’s pathogenesis. The condition could then be delayed by finding a surgical procedure that effectively improves circulation to the fingers. Our team has been working on the periosteal distraction procedure for treating chronic wounds in diabetic foot [21,22]. The method of periosteal distraction is to implant a designed distractor on the appropriate bone surface. The method is divided into 3 stages: a latency period, distraction period, and consolidation period. The distraction period is to separate the periosteum from the bone surface at a speed of 0.2 to 1.0 mm/day. The consolidation period (from 1 week to 3 months) is for the new bone to mineralize and mature [23]. Periosteal distraction is a modification of transverse tibial bone transfer surgery that promotes wound healing and can treat ischemic wounds in the foot [24–26]. Since this technique does not require tibial drilling and bone transfer, the treatment time is significantly shorter than that of bone transfer surgery. Ma et al reported that radial cortex transverse distraction facilitated finger ulcer healing, salvaged the hand, and improved Raynaud symptoms [16]. Radial cortex transverse distraction and radial periosteal distraction are based on the principle of distraction osteogenesis and induce the regeneration of bone and soft tissues through mechanical stimulation. However, radial cortex transverse distraction requires creating a rectangular osteotomy, leading to more bone and tissue injuries than with periosteal distraction. Patients require about 6 weeks after surgery until removal of the external fixator, which causes inconvenience in their daily life. The procedure of periosteal distraction is simpler, and we remove the retractor screws and plates within 4 weeks. It has better effects in patients than radial cortex transverse distraction. Furthermore, we have successfully used tibial periosteal distraction for the limb-sparing treatment of diabetic foot patients with Wanger stage III or higher. Through sustained periosteal distraction, vascular endothelial growth factor, basic fibroblast growth factor, epidermal growth factor, and platelet-derived growth factor levels in peripheral blood increased significantly, compared with before the operation. After removing the distraction support, basic fibroblast growth factor, epidermal growth factor, and platelet-derived growth factor levels decreased, suggesting that sustained low-stress periosteal distraction can stimulate the expression of wound healing-promoting cellular growth factors [21]. The results suggest that continuous low-stress retraction of the periosteum facilitates wound healing and can enhance the expression of cell growth factors. Also, we observed that the periosteal distraction technique can stimulate vascular regeneration and improve peripheral microcirculation through distraction of the periosteum [12,27].

Currently, periosteal distraction techniques are mainly reported to treat lower limb ischemic disease; however, research on upper limb ischemic disease is lacking [28]. We attempted to use this surgical approach for the first time for a patient with Raynaud syndrome. According to the morphology of the ulnar radius, the ulna is thin and prone to fracture when plates are implanted, affecting the rotational function of the forearm. Therefore, the radial periosteal distraction technique was used in this case. The superficial and gentle palmar surface of the distal radius was selected as the basis of distraction as much as possible during the operation. Since there is no special radial periosteal distraction steel plate, we trimmed and shortened the plate and then inserted it to minimize intraoperative injury and complications. During follow-up, the patient’s symptoms improved, gangrene at the fingertips was significantly controlled, and the wound healed gradually. Amputation was avoided due to prominently improved microcirculation in the fingers, compared with the preoperative period. The periosteal distraction technique has shown a desired effect in the treatment of Raynaud syndrome with gangrene. This means that this technique is no longer limited to the treatment of chronic vascular lesions of the lower limbs, as it also achieves favorable outcomes in ischemic diseases of the upper limbs. This can restore patients’ ischemic limbs and improve their quality of life.

The lack of a control group is a limitation in our case. Consequently, a larger sample size, the establishment of a control group, and a longer follow-up period are necessary to further validate the exact effect of the periosteal distraction technique on Raynaud syndrome, providing a new treatment strategy for this disease.

Conclusions

Patients with Raynaud syndrome should exercise caution in the use of vasoconstrictor medications, which can aggravate the condition. This report supports recent studies that have shown the potential for periosteal distraction osteogenesis in the management of cases of severe and irreversible Raynaud disease of the digits.