Vascular "tumors" and the rules of their surgical management

After defining vascular tumors and malformations, formerly called angiomas, the authors adopt the classification of the International Society for the Study of vascular Anomalies. The various groups of malformations, after definition of the subject and the problems raised, are illustrated by a number of clinical cases and the rules of surgical management are discussed. Capillary malformations, formerly called portwine stains, can be treated by dye laser, but sometimes tissue and orthognathic reconstructive surgery in the presence of a skeletal malformation. Lymphatic malformations (lymphangiomas), optimally assessed by CT or MRI, can be treated by a variety of approaches: sclerotherapy, surgery. Venous malformations raise very different problems in the cervicocephalic region and on the limbs and trunk, but always require a multidisciplinary approach with, according to the site, size and repercussions, Ethibloc percutaneous sclerotherapy, embolization, surgery. Arteriovenous malformations require complete surgical treatment, usually preceded by embolization; reconstruction consists of local flaps or skin expansion in simple cases, and revascularized free flaps in difficult cases. If the malformation is cosmetically and functionally acceptable, the authors propose conservative management. The first-line treatment of haemangiomas is pharmacological (corticosteroids, interferon), but surgery may be indicated in two situations: early and late. There is a renewed interest in early surgery, subtended by several factors including cosmetic concerns and the development of new technologies, including the Cavitron. Late surgery retains its classical cosmetic and functional indications. Two key-words dominate the rules of therapeutic management of all types of vascular malformations: a multidisciplinary approach and modesty.     

Pathological changes in surgically resected angiographically occult vascular malformations after radiation

OBJECTIVE: The goal of this study was to evaluate the pathological changes associated with radiation treatment (stereotactic radiosurgery or conventional irradiation) of angiographically occult vascular malformations (AOVMs). METHODS: Eleven patients underwent surgical resection of an AOVM in the mesial temporal lobe, brain stem, thalamus, or basal ganglia after previous radiation treatment. The indications for surgery were recurrent symptomatic bleeding from the lesion in 10 patients and recurrent intractable seizures in 1 patient. Radiation was used as the initial therapy because the risk of surgical resection was deemed too high. Three patients received conventional radiation therapy of 3000 to 5400 rads at an outside institution. One patient received radiosurgery with the gamma knife at another institution using a dose of 15 Gy to the margin. The remaining 7 patients received stereotactic radiosurgery with a helium-ion particle beam. The dose range was from 18 to 26 Gy equivalents. The interval from radiation to surgical resection ranged from 1 to 10 years, with a mean of 3.5 years. These lesions were compared with 10 nonirradiated cavernous malformations. RESULTS: One irradiated lesion was identified pathologically as a true arteriovenous malformation despite being angiographically occult. This lesion did not demonstrate significant changes in the vasculature but did have radiation necrosis of the surrounding brain 5 years after 25 Gy equivalents of helium-ion radiosurgery. Two other specimens were too small to identify the type of vascular malformation adequately. Of the remaining eight malformations identified as cavernous malformations, six showed a combination of marked fibrosis of the vascular channels, fibrinoid necrosis, and ferrugination. However, the fibrinoid necrosis was the only finding unique to the irradiated lesions compared with nonirradiated controls. All the irradiated lesions still had patent vascular channels; none were completely thrombosed. CONCLUSION: Radiosurgery or conventional radiation therapy did not cause histologic vascular obliteration in intracranial AOVMs evaluated 1 to 10 years (mean 3.5 yr) after radiation delivery. It should be recognized that these patients are irradiation failures who may not be representative of all irradiated patients. However, recurrent bleeding from AOVMs may relate to poor radiation response in some patients.     

Posttraumatic syringomyelia. A report of an atypical case

Isolated congenital lymphoedema of the external genitalia in boys is extremely rare. It can have major physical and emotional consequences for the children. Three male patients with primary lymphoedema of the penis and scrotum are described. The first case presented with lymphoedema of the prepuce and the phallic skin that persisted after the age of 1 year. Circumcision and excision of the subcutaneous tissues of the phallic skin were successful in producing the appearance of a normal circumcised penis. The second case presented with oedema of both phallic and scrotal skin. At age 1 year only a pastous thickening of the prepuce remained, scrotal and phallic skin normalised spontaneously. Routine circumcision was successful with a 5-year follow up. The third case presented with persistent oedema of both the scrotum and the phallus. He suffered leakage of lymph requiring incontinence pads, with complicating skin infections recurring every 4-6 weeks at age 8 years. After complete peeling of the scrotal and phallic skin from subcutaneous tissue some leakage persisted, infections subsided. The cause of this disorder remains unknown, although hypoplasia of the lymphatic vessels is reported in most cases. In the literature, several congenital malformations have been associated with primary lymphoedema. These were not noted in our patients. Rarely, the lymphoedema regresses spontaneously. The treatment of persistent lymphoedema is surgical and consists of meticulous excision of all subcutaneous layers of the affected skin, combined with reconstruction of the penis and/or scrotum. CONCLUSION: Primary lymphoedema of the male external genitalia is an extremely rare malformation of the lymphatic vessels of unknown origin. If persistent, surgical treatment is necessary.     

Treatment of scalp arteriovenous malformation

We report seven patients with scalp arteriovenous malformations, including two patients with lesions > 10 cm in diameter, who were successfully treated. The principal complaint of each patient was a deforming mass. Each of four patients had a history of blunt traumatic injury. The lesions, each consisting of the nidus, feeders, and draining veins, evolved in all patients. The nidus consisted of fistulae, which exhibited various angioarchitectures as revealed by angiography. A hemangiomatous component was histologically recognized in one patient. In five patients, in whom the lesions were relatively small and whose nidi included only large fistulae, the malformations were remedied by surgical intervention alone or were cured with embolization alone using liquid adhesives. In the two patients with lesions > 10 cm, the nidi consisted of numerous large fistulae and plexiform fistulae in one patient and plexiform fistulae and a hemangiomatous component in the other patient. These patients were treated with a combination of transarterial embolization and surgical intervention. Preoperative embolization greatly reduced blood loss during resection. Total excision and scalp reconstruction using a soft tissue expander were performed in both patients. The cosmetic results were excellent in all of the patients, and no recurrence has been recognized during the follow-up period, which ranges from 31 to 99 months. The treatment of scalp arteriovenous malformations should strive to improve deforming features and to attain a permanent cure. Because each nidus includes a variety of anomalous angioarchitectural features, there should be different means and a combination of treatments for each patient. Embolization alone could be adequate treatment in relatively small lesions, the nidi of which consist only of several large fistulae. For malformations with more extensive, large fistulae or with anomalous components other than large fistulae, a combined endovascular and surgical approach and scalp reconstruction seems to be the best treatment.     

Large arteriovenous malformations of the face: aesthetic results with recurrence control

Large facial arteriovenous malformations are problematic for patients because of grotesque disfigurement, risk of rapid enlargement, and life-threatening rupture. Successful treatment of these relentless complex lesions is one of the most difficult challenges facing plastic surgeons. From a series of 300 large facial arteriovenous malformations, 85 patients were treated with embolization and excision; six of these cases (representing six separate anatomic regions: labial, auricular, eyelid, cheek, chin, and occipitoparietal) were selected for review. The purpose of this article was to look critically at the management of these six facial arteriovenous malformations, including patient presentation, angiographic procedures, surgical planning and technique, and postoperative long-term follow-up care. Lessons learned from the six representative cases provide clues for the management of large facial arteriovenous malformations and demonstrate the possibilities of recurrence and their occasionally relentless behavior. The cases show that long-term control of these lesions with acceptable aesthetic results can be achieved. The mainstay of treatment includes the following: (1) selective intra-arterial embolization with fine catheters and direct lesional embolization; (2) judicious resection and reconstruction with local or expanded tissue flaps; and (3) careful follow-up with serial examinations, duplex, and arteriography.     

Detection and preliminary characterization of matrix metalloproteinase activity in temporomandibular joint lavage fluid

MATERIALS AND METHODS: In the period 1986-1994, 2950 patients with cardiovascular diseases were surgically treated. In 2104 cases we placed biological or synthetic grafts to maintain vascular continuity. The most common has turned out to be abdominal aortic aneurysm. We treated 783 cases in emergency conditions. Staging and localization of infection has been the first aim in patients with synthetic vascular grafts. We studied signs and symptoms related to infections. In all cases we discovered the microorganism responsible of infection we started antibiotic therapy. RESULTS: Surgical infection incidence is 4.9% (154 cases). Series analysis has evidenced a decrease in infection incidence in the period 1986-1994. The most frequent infections are: the urinary tract infection (59 cases, 38.5%) followed by surgical wound infection (37 cases, 24.1%), respiratory tract infection (27 cases, 17.5%), vascular graft infection (23 cases, 14.4%). All patients underwent a preoperative antibiotic prophylaxis with 2 degrees-3 degrees generation cephalosporines. We noted a higher graft infection incidence in patients treated with aortobifemoral reconstruction. We handled surgical infection following two main directions: 1-antibiotic therapy, 2-surgical treatment and antibiotic therapy. CONCLUSIONS: We noted surgical technique improvement and correct application of an antibiotic prophylaxis form has turned out to be the "gold standard" in order to reduce cardiovascular surgical infections. To reduce sepsis or graft infection we can work on either of the following: 1) antibiotic therapy; 2) operative time reduction; 3) try to limit vascular surgery in case of concomitant gastrointestinal surgical disease; 4) using alloplastic vascular grafts with high biological compliance; 5) patency time reduction of invasive diagnostic technique.     

Reliability of hemorrhagic transformation diagnosis in acute ischemic stroke

Vascular malformations of the upper extremity continue to be a therapeutic challenge to the hand surgeon. In the past 22 years, the authors treated 17 patients with major vascular malformations of the upper extremity. The lesions were evaluated with the aid of plain radiographs, angiography, and histology. Thirteen of the patients were female and 4 were male. The average age at treatment was 18 years, ranging from birth to 64 years. The average follow-up period was 12 years, ranging from 4 months to 36 years. The main indications for surgery were pain, progressive enlargement, neurologic compromise, diminished function, or a combination of these. The progression of these lesions under observation led to surgical management in all cases. A total of 36 excisions, 3 embolizations, and 4 amputations were done. Postoperative angiograms were obtained in selected individuals. Diffuse lesions defied total surgical removal. Recurrence and persistence was evident in 12 of the 17 patients. A knowledge of the progressive nature of these lesions and the need for long-term observation are the keystones to the successful management of these challenging yet unresolved problems.     

Fibrolamellar hepatocellular carcinoma: results of partial liver resection

AIM: To assess the results of partial hepatic resection in the treatment of fibrolamellar hepatocellular carcinoma. PATIENTS AND RESULTS: We present six cases of Fibrolamellar hepatocellular carcinoma treated by partial hepatic resection. There were five females and one male with a mean age of 20 (2.6) yr. Five patients were stage IVA and 1 IVB based on the Union International Against Cancer Classification. Two patients, due to recurrent disease, needed further pulmonary resection and a hilar lymphadenectomy. All six cases underwent major resection, three with vascular reconstruction. No operative mortality was recorded; half of the patients displayed some degree of morbidity. One patient died six months after the operation and the rest are alive at 78, 41, 24, 12 and 9 months. We believe that even in advanced cases, an aggressive surgical policy even with vascular reconstruction is justified in patients with fibrolamellar hepatocellular carcinoma.     

Covering extensive soft tissue defects in infected knee endoprostheses by gastrocnemius flap

Total knee arthroplasty has become a routine procedure in surgery. Deep infections have an incidence of 2-5%. Major risk factors are large prostheses, rheumatoid arthritis, diabetes mellitus and postoperative wound-healing complications. In large soft-tissue defects with skin necrosis, local wound care shows poor results, especially if loosening of the prosthesis and necrosis of the patellar ligament are evident. In these cases, no standard surgical therapy has been developed yet. Thus, we consider meticulous débridement with synovialectomy to be mandatory. Exchange of the prosthesis may be necessary. Soft-tissue coverage ought to be performed with a gastrocnemius muscle flap covered with a split-thickness skin graft. In the last three years, 11 patients with large soft-tissue defects and necrosis of the ligament were treated according to this concept. In all cases the muscle flap healed primarily and soft tissue coverage was excellent. Two patients who underwent single-stage removal and reimplantation of the prosthesis showed reinfections of the prosthesis. Reconstruction of the ligament was performed with the flap tendon. The patients with two-stage removal and reimplantation of the prosthesis and those who retained their implants had a good functional outcome. The gastrocnemius muscle flap provides easy and reliable soft-tissue reconstruction in large defects. In our patients a two-stage operation for reimplantation of the prosthesis was superior to a single-stage procedure. The reconstructed ligament should be reinforced with autologous material to prevent a secondary rupture. Early reconstruction with sufficient soft-tissue coverage and reconstruction of the ligament offers the patient the best chances of obtaining a good functional result and prevents arthrodesis or amputation. In addition, reconstructive surgery reduces the length of hospital stay and costs.     

Dexamethasone stimulation of glutaminase expression in mesenteric lymph nodes

Cystic lymphatic malformations (CLM) are superficial vascular hemodynamically inactive malformations of the lymphatic compartment. We propose a new approach which uses a sclerosing agent as an alternative to surgical resection. In the past nine years we treated 70 patients with CLM. Fifty-five percent were younger than five years of age with a male preponderance and most (80%) of the CLM were located in the maxillofacial region. They usually presented with functional impairment from the mass effect; others had infections, bleeding, or inflammation. The CLM were injected under fluoroscopic control with a sclerosing agent, Ethibloc, which dries up the pockets and reduces the mass. On follow-up the results were good in 62%, unchanged in 5%, and continued progression in 20%. Fifteen percent underwent surgery failures (24%) occurred in mixed forms of cystic and cellular lymphangiomas. Complications were minors. Percutaneous embolization is useful for CLM, with minimal risk, absence of scar, and it avoids surgery. It should be the first line of treatment for these lesions.     

Surgical outcome in patients with epilepsy with occult vascular malformations treated with lesionectomy

PURPOSE: This retrospective study reports the long-term surgical outcome of patients with medically refractory epilepsy and vascular malformations who were treated with lesionectomy. A detailed analysis of surgical failures had been performed in an attempt to define predictors of surgical success and failure. METHODS: Fifteen patients with medically intractable epilepsy and angiographically occult vascular malformations (AOVMs) were treated surgically with lesionectomy at Duke University Medical Center. Lesionectomy consisted of removal of the AOVM and surrounding hemosiderin-stained brain only, without the use of electrocorticography (ECoG) to guide resection. RESULTS: Eleven (73%) patients are seizure free after lesionectomy. Three showed no significant improvement, and one patient died, presumably after a seizure. Age of onset, duration of seizures, age at resection, and gender did not affect outcome. All patients with neocortical AOVMs in whom EEG findings correlated with the site of the lesion were seizure free after lesional resection. Treatment failures were associated with the presence of multiple intracranial lesions, poorly localized or diffuse EEG findings, discordant positron emission tomography (PET) imaging, or with a lesion in close proximity to the limbic system. CONCLUSIONS: Lesionectomy, with removal of surrounding hemosiderin-stained brain, can be considered the procedure of choice in carefully selected patients with epilepsy with occult vascular malformations.     

The effects of endometrial ablation

Heparin-induced skin necrosis at the injection site is a rare adverse effect, more commonly associated with standard heparins than with low-molecular-weight heparins (LMWH) and its mechanism remains unclear. We report a case of LMWH-induced skin necrosis in a female during prophylactic treatment with LMWH after a surgical procedure. Determination of heparin-platelet-factor-4(PF4)-induced antibodies was positive. This case describes the occurrence of LMWH-induced skin necrosis and antibodies to heparin-PF4 complex, suggesting that this effect is more frequent than previously suspected.     

Surgical management after doxorubicin and epirubicin extravasation

Doxorubicin and epirubicin are strong antineoplastic agents widely used in chemotherapy. One major complication of their use is skin sloughing after subcutaneous extravasation, the degree of which is often underestimated. Both drugs have a tendency to produce liquefying necrosis in soft tissue and chronic ulcers if extravasation occurs. Three cases of extravasation, their surgical treatment and final results are presented. In cases of doxorubicin and epirubicin extravasation it is very important to perform an early extensive surgical débridement with delayed closure to avoid long hospitalization and disabling results.     

Pilomatrixoma of the head and neck in children: a study of 38 cases and a review of the literature

OBJECTIVES: To describe the clinical presentations and discuss the guidelines for surgical management of pilomatrixoma involving the head and neck in children. DESIGN: Retrospective study. SETTING: A tertiary care center. PATIENTS: Thirty-three patients, with a mean age of 4.5 years, underwent surgical treatment for pilomatrixoma (n = 38) between 1989 and 1997. INTERVENTION: All patients were treated surgically. In 34 cases, a direct approach was used to achieve complete removal of the lesion with (n = 11) or without (n = 23) skin resection. In the remaining 4 cases, an indirect approach via a parotidectomylike incision was used. RESULTS: In 88% of cases, the presenting symptom was a hard, slow-growing, subcutaneous tumor. The lesion was associated with pain and inflammation in 7 cases (18%) and abscess or ulceration in 4 cases (11%). Twenty-nine patients presented with single nodules and 4 presented with multiple occurrences. The lesions were located on the face (cheek, eyelid, or forehead) in 20 cases (53%), on the neck in 8 cases (21%), in the parotid region in 8 cases (21%), and on the scalp in 2 cases (5%). CONCLUSIONS: Pilomatrixoma is a rare, benign skin tumor, but practitioners should be aware of its clinical features. Diagnosis is usually easy based on clinical findings, but computed tomographic scan is helpful, especially in cases involving tumors located in the parotid region. Spontaneous regression is never observed. Complete surgical excision, including the overlying skin, is the treatment of choice.     

Vein of Galen vascular malformations in infants: clinical, radiological and therapeutic aspect

A series of 14 vein of Galen vascular malformations diagnosed in the pediatric populations and treated at the Hospital for Sick Children-Necker, Paris, between 1988 and 1994 is presented. Five of the patients were diagnosed in the neonatal period, of whom 4 presented with life-threatening, intractable cardiac decompensation and high-flow arteriovenous fistulae. Embolization was performed on vital indications in 4 patients during the first week after birth. One embolization failed with fatal outcome. Of the 3 who were embolized, 2 succumbed within 1 week and 1 survived with marked improvement of cardiac symptoms. The older children presented with hydrocephalus and neurologic symptoms. The 10 patients older than 1 year were embolized. These procedures were successful in 90 %, with hemodynamic stabilization and improvement of clinical symptoms. In this group the mortality rate was 10 %. The total mortality rate was 29 %. Hydrocephalus was secondary to a compression of the Sylvian aqueduct in 44 % of cases. Five patients had ventricular drainage before embolization followed by a staged elective embolization. Transarterial embolizations were performed in 11 patients, whereas 2 patients were embolized via the transvenous route.     

Overview of quality measurement and improvement

BACKGROUND: Oro-mandibular reconstruction using vascularized bone-containing free-flaps can be accomplished with flap survival rates in the range of 95%. Primary reconstruction offers the best opportunity to achieve the optimal aesthetic and functional results. Patients presenting for secondary oro-mandibular reconstruction have a unique set of problems; these include the presence of soft tissue contracture displacing the mandibular segments in malposition and soft tissue deficiencies, that makes surgical correction more difficult and potentially more hazardous. Vascularized bone-containing free-flaps are indicated in secondary oro-mandibular reconstruction where both hard and soft tissues replacement is needed or when the recipient bed is unfavourable due to previous surgery and/or radiation. METHODS: Authors presents personal experience in ten cases of secondary oro-mandibular reconstruction treated at Maxillofacial Department of Parma from September 1995 to September 1996 with secondary oro-mandibular reconstruction using bone containing free flaps. Two different donor sites were used to harvest bone-containing free flaps: iliac crest in 2 cases and fibula in the others. In 4 cases the flap was only osseous while in the other 6 cases it was osteocutaneous. RESULTS: All flaps were transplanted successfully; in 1 case necrosis of the skin component of the flap was observed. CONCLUSIONS: The introduction of vascularized bone containing free flaps transferred from distant sites by microvascular techniques has changed mandibular reconstruction. Vascularized bone transferred into tissue beds compromised by salivary contamination and previous irradiation and the rational use of the soft tissutal components of the flap permit also the restoration of articulation, deglutition and mastication with quality of life better than non-vascularized alternatives.     

Short- and long-term treatment with indometacin causes renal phospholipid alteration: a possible explanation for indomethacin nephrotoxicity

The authors report their experience of the use of a latissimus dorsi myocutaneous flap in reparative surgery after total or partial mastectomy for invasive breast cancer. The series, including 101 cases, was separated into four groups on the basis of the context: I--thoracic radionecrosis (5 cases); II--chest wall cover (24 cases); III--immediate breast reconstruction (57 cases); deferred breast reconstruction (15 cases). The well-known vascular reliability of this pediculated flap was confirmed here by the low incidence of necrosis (3%), always marginal. Other complications were not attributable to the flap itself but to concomitant radiodystrophic skin lesions and the presence of the prosthetic implant in the case of breast reconstruction.     

Paracoccidioidoma: case record and review

The ulcer resulting from snake-bite injury was characterized by deep and wide tissue necrosis and secondary infection. The patient was at high risk of loss of function of his extremity. From 1989 to 1996, 16 cases with deep ulcer of the upper extremity resulting from snake-bite injury were treated with different types of axial thoraco-abdominal skin flaps, depending on the location of the ulcer. Thoraco-umbilical skin flap was used in 2 cases, lateral thoracoabdominal skin flap in 1 case, iliolumbar skin flaps in 5 cases, lower abdominal skin flaps in 6 cases, lower abdominal divided foliated skin flap in 1 case and Y-shaped hypogastric skin flap in 1 case. Reparative operation was carried out within 3 weeks after injury and primary repair were undertaken in 6 of them. The pedicles were divided in 17 to 24 days after operation. Twelve flaps survived. Three of the 6 flaps had mild signs of inflammation which disappeared after administration of antibiotics. One had necrosis of the distal part of the flap, and was healed with split skin graft. This type of skin flap was an ideal method for the treatment of snake-bite injury of the upper extremity.     

Management of severe infection complicating aortofemoral grafts

OBJECTIVE: Infection of the retroperitoneum after implantation of an abdomino-femoral aortic graft remains one of the main problems in vascular surgery. On basis of a critical review of own experiences we evaluated the management of this difficult clinical situation. PATIENTS: From 1970-1996 1500 aortofemoral graft operations (aneurysmal disease: 512, aortoiliacal occlusive disease: 988) were performed. Abdominal infection occurred in 12 patients (0.8%) (12 men, median age 60.5 [48-80] years). RESULTS: The median interval between operation and infection was 17.7 (0.5-108) months. The port of infection was in 50% the groin, 25% suffered from abdominal infection, in 3 cases it was not to identify. Clinical manifestation of infection was aortoduodenal fistula in 2 patients, false aneurysms in 2 cases, and a paraprosthetic abscess in another 4 patients. Operative therapy comprised (partial) removal of infected material in 10 patients with consecutive extraanatomical reconstruction in 8 of these. Mortality of graft infection was 50%. Causes of death were untreatable sepsis in 4 patients, another 2 died from hemorrhagic shock. 3 out of 6 surviving patients finally lost their limbs following multiple vascular procedures. CONCLUSION: Adequate surgical therapy of infected aortofemoral grafts remains an unsolved problem. Lack of knowledge of suitable parameters for the best treatment leaves the outcome of prosthetic infection unpredictable. Removal of the infected graft with extraanatomic reconstruction seems to be the standard of surgical treatment, which is recommended in these cases.     

Study and planning of the surgical procedure for the orbital district in patients affected by craniofacial malformations

Orbital surgery is of critical importance within the framework of craniofacial surgery. The conical conformation of orbits requires analysis and surgical procedure planning methods involving all three dimensions. We present our protocol for the analysis and our three-dimensional surgical procedure plan to treat orbital malformations using teleradiography and two- and three-dimensional computed tomographic imaging. A number of clinical cases treated according to this approach are also presented.