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Raynaud's phenomenon

In medicine, Raynaud's phenomenon is discoloration of the fingers or toes due to emotion or cold in a characteristic pattern in time: white, blue and red. more...

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Medicines

It is associated with a large number of diseases (mainly forms of vasculitis and hence belonging to rheumatology), but is idiopathic in most sufferers and is then called Raynaud's disease. Raynaud's phenomenon has also been observed in patients after receiving chemotherapy, particularly after regimens that included bleomycin.

The condition is painful and potentially dangerous for those who suffer from it. The primary cause of the pain and discoloration of the affected extremeties is vasospasm.

In pregnancy, this sign normally disappears due to increased surface blood flow.

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Raynaud's phenomenon in radial forearm free-tissue transfer - Original Article
From Ear, Nose & Throat Journal, 7/1/02 by Theodoros N. Teknos

Abstract

During 1998, 69 patients underwent either fasciocutaneous or osteofasciocutaneous radial forearm free-tissue transfers at our institution. Three of these patients (4.3%), who had undergone a total of four transfers, had Raynaud's phenomenon. Three of the four transfers survived without perioperative incident. The one flap that failed had been well vascularized until postoperative day 3. Then, coincident with a decrease in ambient room temperature, the patient's extremities and his flap became cyanotic. A salvage procedure was unsuccessful. In this article, we discuss the perioperative course, proposed pathophysiology, and treatment strategies aimed at optimizing hand and flap outcomes in patients with Raynaud's phenomenon.

Introduction

Raynaud's phenomenon is characterized by episodic digital blanching, cyanosis, and rubor of the fingers and/or toes following exposure to cold and subsequent rewarming. Its overall incidence among the general population in the colder regions of the United States and northern Europe is reported to be 10 to 15%. (1) Most patients have a mild form of the disorder; severe forms are rare.

The radial forearm fasciocutaneous free-tissue transfer was popularized by Soutar et al in 1983 for oral cavity reconstruction. (2) Free-tissue transfers have since taken on a prominent role in a variety of head and neck reconstructions because of their high success rate, their malleability in conforming to complex three-dimensional defects, their ease of harvest, and the length of the pedicle.

All patients who are about to undergo a radial forearm free-tissue transfer should be investigated for the possibility of Raynaud's phenomenon. Also, a clearly positive Allen's test is important. The risk of an episode of Raynaud's phenomenon increases with the use of a tourniquet and in the event of a reperfusion injury, which is not uncommon during a revascularized free-tissue transfer. The purpose of this article is to discuss the effect that Raynaud' s phenomenon has on outcomes following radial forearm free-tissue transfer.

Patients and methods

From January through December 1998, we performed 69 fasciocutaneous or osteofasciocutaneous radial forearm free-tissue transfers during a variety of head and neck reconstructions at the University of Michigan Medical Center and the Ann Arbor Veterans Administration Hospital. A careful review of their medical records showed that three of these patients (4.3%), all white men, had clearly documented Raynaud' s phenomenon.

Among the three patients, a total of four microvascular free-tissue transfers had been performed: one patient underwent two radial fasciocutaneous free-flap procedures (patient 1), one patient underwent a single radial fasciocutaneous free-flap procedure (patient 2), and the other underwent a single radial osteofasciocutaneous free-flap procedure (patient 3). Preoperatively, only one of these three patients (patient 3) reported a history of Raynaud' s phenomenon.

Results

Two of the three patients who had Raynaud's phenomenon experienced adverse events. Patient 1 experienced a flap failure, and patient 2 experienced a delay in hand reperfusion following deflation of the tourniquet.

Patient 1. A 36-year-old man underwent surgery for a T3N2M0 squamous cell carcinoma of the left tonsil. His medical and surgical histories provided no indication that he had Raynaud' s phenomenon. His preoperative Allen's test was positive.

The surgeon performed a left modified radical neck dissection with a wide local excision of the left tonsil, base of the tongue, pharynx, and soft palate. The operation created a 4 x 6-cm defect. The flap was planned on his left forearm, and the tourniquet was inflated to a pressure of 250 mm Hg for 60 minutes. The flap was elevated in the standard fashion, and the vascular pedicle was meticulously dissected. When the tourniquet was deflated, the left hand and the attached flap demonstrated excellent reperfusion. The flap was detached and handed off to the head and neck for inset.

The flap remained well vascularized until postoperative day 3, when it became cyanotic and the capillary refill was noted to be poor; these events coincided with the temporary lowering of the temperature in the man's room to 68[degrees] F. During this period of low temperature, the patient also experienced bilateral hand cyanosis. The flap was punctured with a needle, but no bleeding was seen.

The patient was taken back to the operating room emergently for a neck exploration, which revealed that the entire arterial pedicle was thrombosed, suggesting that the problem might have originated in the microvasculature. The venous anastomoses were patent, and there was no evidence of thrombus formation. The next day, the patient was returned to the operating room, where the surgical defect was successfully closed with a free-tissue transfer taken from the right radial forearm.

Postoperatively, care was taken to maintain the ambient room temperature at more than 78[degrees] F, and the patient experienced no further complications. He was discharged home on postoperative day 7 with a viable flap. At the 24-month follow-up, he had experienced no further adverse events in his hand or in the revascularized free-tissue transfer.

Patient 2. A 41-year-old man underwent surgery for a T4N3M0 squamous cell carcinoma of the right base of the tongue. No suggestion of Raynaud's phenomenon was discerned from his medical and surgical histories. His Allen's test was positive.

The surgeon performed a bilateral neck dissection, subtotal glossectomy, total laryngectomy, and partial pharyngectomy. The operation created a 6 x 10-cm defect at the base of the tongue and the pharynx. The flap was planned on the left forearm, and the tourniquet was inflated to 250 mm Hg for 50 minutes. The flap was elevated in the same manner as in patient 1. When the tourniquet was released, the patient's hand experienced a rapid vascular refill. The flap was detached and handed off to the head and neck region.

The donor site was closed approximately 45 minutes after die tourniquet was released. Upon closure, the appearance of the patient's left hand progressed from pale to cyanotic to mottled. The forearm incision was immediately reopened, but no abnormality was seen. Next, the patient's hand was warmed. The patient maintained Doppler signals at the level of the distal ulnar artery, but not at the level of the digital arteries. After approximately 30 minutes, the cyanosis resolved and the digital artery Doppler signals returned to normal.

It was not until after the operation that the patient reported a history of digital and hand cyanosis associated with exposure to cold. His flap was routinely monitored by clinical inspection and Doppler checks, and the room temperature was maintained at a minimum of 78[degrees] F. During his postoperative stay, the flap exhibited no further cyanosis or other complication. At the 18-month follow-up, the patient had experienced no further adverse events in his hand or in the revascularized tissue.

Patient 3. No intra- or postoperative complications occurred in patient 3 (age: 73 yr), whose history of Raynaud's phenomenon was known before he underwent surgery. During surgery, we took care to follow standard protocol by maintaining this patient's core temperature at 37.8[degrees] C intraoperatively and by keeping his room temperature higher than 78[degrees] F postoperatively.

Discussion

Raynaud's phenomenon is common among patients who have systemic vasospastic disorders. For example, patients with Raynaud's phenomenon have a higher incidence of migraine and angina. (3) In addition, Raynaud's phenomenon can affect many areas of the skin, including the surface of the nose, ears, tongue, and nipples. (3) Moreover, Raynaud's phenomenon has been implicated in rhytidectomy flap necrosis in a report of two patients. (4)

Our purpose in studying this small cohort was to identify some of the potential problems that can be encountered while performing radial forearm free-tissue transfers in patients with Raynaud's phenomenon. The history of this phenomenon had not been elicited preoperatively in two of our three patients. In patient 1, Raynaud's phenomenon might have been the reason for the flap failure, although a causal relationship cannot be confirmed. In patient 2, Raynaud's phenomenon caused a delay in the reperfusion of the hand. By contrast, the case of patient 3 illustrates the importance of knowing the preoperative history of this condition so that the surgeon can anticipate and possibly prevent an unfavorable outcome.

Pathophysiologic mechanisms. To appreciate the impact Raynaud' s phenomenon can have on a revascularized free-tissue transfer, it is helpful to understand the disease process. Its pathophysiology is thought to be multifactorial--most likely caused by both vascular and neural mechanisms.

The vascular mechanism is related to vasospasm that is triggered by cold. (3) Repeated attacks of vasospasm lead to the release of damaging endothelial products, such as factor VIII and von Willebrand's factor (which hinder fibrinolysis) and endothelins (which are vasoconstrictors). (3) The cool ambient room temperature to which patient 1 was exposed coincided with the episode of Raynaud's phenomenon in his hands and in the revascularized free tissue. This temporal relationship suggests that there was an association between Raynaud's phe- nomenon and ischemia in the revascularized free tissue.

The neural mechanism involved in Raynaud's phenomenon is thought to be related to calcitonin gene-related peptide (CGRP), a 37-amino-acid peptide that is present in the neurons around blood vessels, including those of the skin and the mesenteric and cerebral vasculature. (5) CGRP acts on endothelial receptors and activates a series of intracellular processes that ultimately lead to an increase in cyclic guanosine monophosphate levels and resultant vasorelaxation. (5) These patients have a specific deficit of CGRP neurons in the digital skin. (5)

Treatment. In general, conservative and supportive measures are all that are necessary to treat digital Raynaud's phenomenon. When cold elicits Raynaud's phenomenon, simply warming the hand is usually adequate. If another stressor elicits the disorder, the solution is to eliminate the stressor. If these measures prove to be ineffective, a calcium channel blocker can be prescribed. The beneficial effect of calcium channel blockers is mediated via their peripheral vasodilatory, antiplatelet, and antileukocytic effects. (3) Another agent, the prostacyclin analog iloprost, has potent vasodilatory and antiplatelet effects. Iloprost can be used in cases of refractory Raynaud's phenomenon, such as occurs in patients with digital ischemia or infarction. If either of these drug classes are contraindicated, a topical vasodilator such as 1 to 2% hexyl nicotinate in aqueous cream can be used. (5) Initial clinical studies with CGRP and defibrotide (a poly-DNA compound that has profibrinolytic and antithrombotic activity) have shown that these agents might be useful in treating Raynaud's phenomenon. Although drug therapy was not necessary for either of our two patients, it should at least be considered as an adjunct to standard postoperative care.

In light of the prevalence of Raynaud's phenomenon and its potential deleterious effects, a preoperative history relating to vascular diseases of the extremities should be obtained from every patient who is a potential candidate for a radial forearm free-tissue transfer. In a severe case of Raynaud's phenomenon, the reconstructive surgeon should consider choosing an alternate donor site. Even so, Raynaud's phenomenon is not an absolute contraindication to radial forearm free-tissue transfer, and the forearm can be safely used if no other site is suitable. In these cases, maintaining an ambient room temperature of at least 78[degrees] F both intra- and postoperatively is absolutely essential.

References

(1.) Bartelink ML, Wollersheim H, van de Lisdonk E, et al. Prevalence of Raynaud's phenomenon. Neth J Med 1992;41:149-52.

(2.) Soutar DS, Scheker LR, Tanner NS, McGregor IA. The radial forearm flap: A versatile method for intra-oral reconstruction. Br J Plast Surg 1983;36:1-8.

(3.) Ho M, Belch JJ. Raynaud's phenomenon: State of the art 1998. Scand J Rheumatol 1998;27:319-22.

(4.) Vecchione TR. Rhytidectomy flap necrosis in Raynaud's disease. Plast Reconstr Surg 1983;72:713-9.

(5.) No authors listed. Raynaud's phenomenon. Lancet 1995;346:283-90.

From the Department of Otolaryngology--Head and Neck Surgery, University of Michigan, Ann Arbor.

Reprint requests: Theodoros N. Teknos, MD, Department of Otolaryngology--Head and Neck Surgery, University of Michigan Medical Center, 1904 Taubman Center, 1500 E. Medical Center Dr., Ann Arbor, MI 48109-0312. Phone: (734) 936-3172; fax: (734) 936-9625; e-mail: teknos@umich.edu

Originally presented as a poster during the annual meeting of the American Academy of Facial Plastic and Reconstructive Surgery; New Orleans; Sept. 23-25, 1999.

COPYRIGHT 2002 Medquest Communications, LLC
COPYRIGHT 2002 Gale Group

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