Eur J Plast Surg DOI 10.1007/s00238-011-0657-z
IDEAS AND INNOVATIONS
A safe method for excision of a giant neurofibroma on both buttocks using a loop-shaped suture Stamatis Sapountzis & Ji Hoon Kim & Jin Sik Burm & Pham Minh & Chan Yeong Heo
Received: 24 June 2011 / Accepted: 18 October 2011 # Springer-Verlag 2011
Abstract Neurofibromatosis(NF) is an autosomal dominant systemic disease. Up to 50% of patients with NF are reported to have concomitant vascular abnormalities. In the resection of a larger NF, the risk of uncontrolled hemorrhage is much higher due to the difficulty of hemostasis of large vessels within the tumor. We ligated the base of the giant NF with a simple loop-shaped ligation before removal of the giant NF in both buttocks, following this, we could successfully reduce the amount of hemorrhage during the operation. A 46-yearold female patient presented with giant masses of both gluteal areas, which had been growing slowly for the last 10 years. Each mass was about 35×25 cm in size. After designing the elliptical resection margin, we tightened the tumor base by using continuous loop-shaped suture ligation (weaving the thread up and down in a loop-shaped pattern, leaving a space of 2 cm between each loop) a straight needle and prolene 2–0 was used after skin incision. We proceeded with the dissection towards the central and inferior side of the mass obliquely while we avoided opening large vascular sinuses. We resected the tumor in a wedge shape. Subcutaneous tissue was sutured layer by layer, and skin was closed by vertical mattress and interrupted sutures. The loop-shaped ligation of the base was S. Sapountzis : J. H. Kim (*) : P. Minh : C. Y. Heo Department of Plastic and Reconstructive Surgery, Seoul National University Bundang Hospital, 166 Gumiro, Bundang, Seongnam, Gyeonggi 463-707, Republic of Korea e-mail:
[email protected] S. Sapountzis e-mail:
[email protected] J. S. Burm Department of Plastic and Reconstructive Surgery, School of Medicine, Kyung Hee University, Seoul, South Korea
removed, and compressive dressing was done with gauze and elastic bandages. Postoperative complications such as infection, hemorrhage, hematoma, and dehiscence did not occur. Perioperatively, the patient was sufficiently transfused with only two units of blood. During the subsequent 1 year followup, the functional and cosmetic results were excellent. A continuous loop-shaped suture ligation procedure along the base of the giant NF effectively reduced the amount of hemorrhage during the operation, made dissection and ligation of vessels easy and quick, shortened the operating time and postoperative recovery time. Keywords Giant neurofibroma . Buttock . Suture ligation . Excision
Introduction Multiple neurofibromatosis is an autosomal dominant disorder with an incidence of approximately one in 3,000 live births [1]. The gene locus of neurofibromatosis is characteristically localized to a chromosome 17. Patients with this disorder develop Schwann cell tumors called neurofibromas and skin abnormalities. The most characteristic features are the café au lait pigmented skin spots, Lisch nodules (iris hamartomas), and multiple neural tumors [2]. In a case of multiple neurofibromatosis, malignant transformation should be suspected if there is progressive enlargement and pain related to a neurofibroma. In this case, the surgical excision was absolutely indicated which can also give the best cosmetic results. Even if hemorrhage following trauma or spontaneous is a rare complication of neurofibromatosis, vascular abnormalities have found in almost half of neurofibromatosis patients [3]. Macroscopically, these can be vascular stenoses, aneurysms, and
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arteriovenous fistulae. Microscopically, the most vulnerable are the small- and the medium-sized vessels, in which the intima becomes thicker and the media thinner and fibrotic. Abnormal vascular structures are also observed in the neurofibromatous tissue. There are thin-walled ecstatic blood vessels lying in a loose neural stroma which replace the normal adipose tissue. For this reason, the risk for severe bleeding during the surgical excision is high, especially in giant neurofibromas [4]. Here, we present a case of a huge neurofibroma of both gluteal areas. Because the risk for intra-operative bleeding was very high, we used a loop-shaped suture in order to tighten the tumor base. This way we could reduce the amount of bleeding during the operation. The postoperative period was uneventful, and the cosmetic result was excellent.
Case report A 46-year-old female patient presented in our department with giant masses in bilateral hip region, which had been growing slowly for the last 10 years. The patient had a history of von Recklinghausen’s disease with many café au lait spots throughout the body, freckling in the axillary regions and subcutaneous nodules of various sizes in the trunk, the limbs, and the face. In the past, she had three surgical excisions of subcutaneous nodules in different anatomical regions for cosmetic purposes but never in the gluteal region because of the high tendency of intra-operative bleeding. The patient’s main complaint was the difficulty in simple daily activities such as sitting on a chair. The clinical examination of the patient revealed a symmetrical giant mass bout 35×25 cm in both sides of the hips, which had slowly grown during a period of 10 years (Fig. 1). Multiple subcutaneous nodules were also observed around and above the giant mass. We performed magnetic resonance imaging, and the finding was a
Fig. 1 Giant neurofibroma on both buttocks in a 46-year-old woman
widespread mass in the subcutaneous layer, with neurofibroma characteristics. There was no evidence for malignant transformation or hematoma in the mass. The operation was performed under general anesthesia with the patient in the prone position with the hips bent about 15°. After designing the elliptical resection margin, we tightened the tumor base by using continuous loopshaped suture ligation (Fig. 2) (weaving the thread up and down in a loop-shaped pattern, leaving a space of 2 cm between each loop) with a straight needle and prolene 2–0. After skin incision, we proceeded with the dissection toward the central and inferior sides of the mass obliquely using Metzenbaum scissors while we avoided breaking into large vascular sinuses; we resected the tumor in a wedge shape. The small vessels which were visualized during the dissection were coagulated, and the small bleeding sites were also controlled with the electrocautery. Subcutaneous tissue was sutured layer by layer using vicryl 2–0, 3–0, and 4–0 using a technique to eliminate the dead space, and the skin was closed by vertical mattress and interrupted sutures using nylon 2–0. Two suction drains were placed on both sides. The loop-shaped ligation of the base was removed, and compressive dressing was performed with gauzes and elastic bandages. The size of the resected tumor was 26×32×21 cm and 24×30×19 cm on the left and right sides, respectively. After the operation, the patient was transfused with two units of blood. The pressure dressing was maintained until the seventh post-operative day. The sciatic nerve was also evaluated during this period, with no evidence of nerve damage. The post-operative period was uneventful with no signs of infection, bleeding, or hematoma (Fig. 3). The sutures were removed at the 13th post-operative day, and the patient was discharged the next day. The histological examination confirmed the diagnosis of neurofibroma with nodular proliferation of small vessels with thinned media and irregular wall thickening and aneurysmal dilation. In addition, fibrosis was observed around the blood vessels (Fig. 4). At the annual follow-up, there was no sign of
Eur J Plast Surg Fig. 2 The schematic view of a continuous loop-shaped suture ligation
recurrence and the patient was satisfied with the functional and the cosmetic result (Fig. 5).
Discussion Neurofibromatosis type I (NF1) or von Recklinghausen disease is an autosomal dominant disorder affecting one in 3,000 individuals [1]. Typically, the disease is diagnosed clinically during childhood, as the first sign in 80% of NF1 individuals is usually café-au-lait macules by 1 year of age. The neurofibromatosis is a progressive and unpredictable disease that is associated with a variety of clinical outcomes and complications; prognosis is dependent on age, severity of the disease, and on which organ may be affected by the growth of neurofibromas. The average life expectancy of individuals with NF1 is reduced by 10 to 15 years. Malignant peripheral nerve sheath tumors, soft tissue
Fig. 3 Postoperative view 2 days after surgery
sarcoma, and vasculopathy are the most common causes of early death in individuals with NF1 [5, 6]. In addition, about the half of the patients with neurofibromatosis type I also have vascular lesions; however, the pathogenesis of these lesions are not, until now, well defined. The vascular lesions have been described in the entire arterial tree, but involvement of the renal arteries is most common. NF1 vasculopathy of the cerebrum, endocrine system, gastrointestinal tract, and heart have also been reported. Frequently, multiple vessels are involved [7]. Vascular manifestations are classified into three types based on vessel size: pure intimal, intimal aneurismal, and nodular. Intimal proliferation involves breakdown of muscle and elastic layers and adventitial nodular thickening. Many arterial lesions are often overlapped, and it is difficult to classify an individual lesion as a single “pure type”. Proliferation of Schwann cells in the arterial walls with secondary degenerative change is the basic pathogenesis of the vascular lesions in neurofibromatosis. In larger vessels (aorta, carotid, and proximal renal artery), direct invasion by Schwann cells, intimal thickening, and destruction of the media and elastic tissue lead to either stenosis or aneurysm. In smaller vessels, mesodermal dysplasia causes the proliferation of smooth muscle in the intima leading to stenotic lesions and, occasionally, poststenotic aneurysms [8]. Salyer and Salyer [9] suggested that intimal thickening in NF1 vasculopathy is the result of proliferation of Schwann cells within the arteries. This implies a pathogenic relationship between these lesions and the neurofibromas that characterize this disease. Riccardi has suggested that NF1 vasculopathy results from a dysplastic process in which abnormal function of neurofibromin alters vascular histogenesis.
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Fig. 4 Histological findings of neurofibroma. (Left) A small-sized vessel shows nodular proliferation of intima with thinned media (hematoxylin and eosin stain, ×400). (Center) Irregular wall thickening
and aneurysmal dilation of a vessel was shown (hematoxylin and eosin stain, ×100). (Right) Marked perivascular fibrosis (Masson–Trichrome stain, ×400)
Macroscopically, these lesions can take a variety of forms including vascular stenosis, aneurysms, and arteiovenous fistulae. In histological examination, the smalland medium-sized vessels present with thickening of the intima and a thin but fibrotic media. The vascular structure in nerufibromatous tissue has also pathologic alterations with thin-walled ecstatic blood vessels lying in a loose neural stroma which replace the normal adipose tissue [3]. Coexisting coagulopathies may increase the complication rates. These may be inherited or secondary to vascular anomalies causing platelet trapping and consumption of clotting factors. Hemostasis can be difficult to obtain following injury to these lesions. Diathermy is of limited use as the tissue is very friable [10–12]. Preoperative angiography and superselective embolization have been used in elective excision of vascular neurofibromas; the results of such treatment without associated surgery have been disappointing because the tumors tend to revascularize quickly. It has been suggested that embolization therapy by a skilled interventional radiologist should be considered prior to elective excision of neurofibromas to reduce intraoperative blood loss. The most effective way to control the bleeding seems to be
ligation of the neurofibromata’s vascular pedicle under direct vision of the feeding vessels together with external compression [4]. In our case, using a continuous loop-shaped suture, we ligated the base of the giant neurofibromatosis tissue. The advantage of this technique was less bleeding during the surgery; the operation field was also relatively bloodless making the ligation of the feeding vessels easier. This method is totally safe and simple compared with other more complex procedures such as embolization. Postoperative complications such as infection, hemorrhage, and dehiscence did not occur. Perioperatively, the patient was sufficiently transfused with two units of blood. During the subsequent 1 year follow-up, the functional and cosmetic results were excellent.
Fig. 5 Postoperative result 12 months after surgery
Conclusion A continuous loop-shaped suture ligation procedure along the base of the giant NF effectively reduced the amount of hemorrhage during the operation, made dissection and ligation of vessels easy and quick, and shortened the operating time and postoperative recovery time.
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