Surgical management of a dermal lymphatic malformation of the lower extremity

Dermal lymphatic malformations are rare congenital hamartomas of superficial lymphatics characterized by high recurrence rates after excision. The standard therapy for a single lesion is surgical excision with wide margins, which reduces recurrence but can have a potentially unacceptable aesthetic outcome. A case of a 24-year-old woman with a 6 cm × 5 cm dermal lymphatic malformation on her right thigh, diagnosed by clinical history, physical examination, magnetic resonance imaging and pathological findings, is reported. The patient underwent wide local excision with split-thickness skin grafting. After pathological examination revealed negative margins, the patient underwent tissue expander placement and excision of the skin graft with primary closure. The lesion did not recur, and the patient achieved a satisfactory aesthetic result. The present case represents the first report of the use of tissue expanders to treat dermal lymphatic malformations in the lower extremity and demonstrates a safe, staged approach to successful treatment.     

浅表血管瘤和脉管畸形的手术治疗

目的：评估手术切除方法对浅表血管瘤和脉管畸形的治疗效果。方法：对各部位位置表浅、界限清楚的血管瘤和静脉畸形、淋巴管畸形行手术切除,继发软组织缺损行直接缝合、局部皮瓣转移或植皮修复。结果：本组病例共91例,血管瘤56例,静脉畸形30例,淋巴管畸形5例;55例血管瘤1次手术切除,1例经2次手术切除;25例浅表静脉畸形1次手术切除,2例经2次手术切除,2例残余部分给予注射平阳霉素治愈;5例淋巴管畸形均1次手术切除。直接缝合63例,行局部皮瓣转移修复20例,植皮修复18例。随访时间分别为3个月～7年,所有病例均无复发,修复部位外形恢复良好。结论：对于诊断确切、界限清楚的浅表血管瘤和脉管畸形,手术切除是一种有效的治疗方法。     

An arteriovenous malformation of the external ear in the pediatric population: A case report and review of the literature

The literature regarding arteriovenous malformations of the external ear is sparse. A case of a patient clinically diagnosed with an arteriovenous malformation of the external ear that was managed empirically with surgical excision, without recurrence, is presented. The pathogenesis, clinical presentation, radiological work up and management options regarding arteriovenous malformations are reviewed.     

Lymphatic malformation: Predictive factors for recurrence

OBJECTIVE: Although lymphatic malformations are often found to be well circumscribed when surgery is undertaken in early childhood, complete surgical excision can be difficult when the lesion is infiltrative. This study retrospectively evaluates these patients in an attempt to identify prognostic factors that may predict recurrence. STUDY DESIGN AND SETTING: A retrospective chart review was conducted covering the years 1991 to 1998. Seventeen patients were identified having undergone 32 surgical resections of tumors described as lymphatic malformations. Data abstracted from the charts included the site of the lesion, surgical and histologic assessment of encapsulation, and status at follow-up examination. RESULTS: Six of 17 patients developed a recurrence after surgery. Correlation between recurrence and histologic or operative impressions of encapsulation was significant by chi(2) analysis (P<0.01). CONCLUSION: On the basis of the findings of this case series, lymphatic malformations that are found to be nonencapsulated and infiltrative by intraoperative or histologic assessment are more likely to recur.     

Spontaneous nontraumatic hemoperitoneum due to a bleeding arteriovenous malformation on the serosal surface of the transverse colon: a case report

Arteriovenous malformations of the gastrointestinal tract are a very common entity and a frequently cited cause of lower gastrointestinal bleeding in adults. They are characteristically found in the mucosa or submucosa of the bowel wall, and the vast majority do not cause any symptoms. We discuss the rare case of an extraluminal arteriovenous malformation of the transverse colon in an elderly woman who presented to the emergency department in hypovolemic shock after collapsing at home. An exploratory laparotomy was performed after a diagnostic peritoneal lavage yielded gross blood. The patient was found to have a spontaneously bleeding lesion confined to the serosal surface of the right transverse colon, which histological examination revealed to be an arteriovenous malformation. The lesion was excised and the patient recovered without sequelae. To date there have been no other documented cases of an arteriovenous malformation on the serosal surface of a visceral abdominal organ.     

Venous malformations: clinical characteristics and differential diagnosis

Venous malformations (VM) are localized defects of blood vessels that are due to vascular dysmorphogenesis. These slow-flow lesions can affect any tissue or organ. Clinically, a cutaneous VM is characterized by a bluish mass that is compressible on palpation. Phleboliths are commonly present. Symptoms depend on location and size. VM are often sporadic and isolated, however, they can be associated with other malformations and be part of a syndrome; Klippel-Trenaunay (capillary-lymphatico-venous malformation with limb hypertrophy) is the most common. Glomuvenous malformation (GVM) is another type of venous anomaly. In contrast to VM, GVM is often painful on palpation and not compressible. Clinical diagnosis of VM is often made in the presence of a bluish cutaneous lesion: however, other lesions can mimick VM. The most frequent anomalies are a blue naevus, a hemorrhagic lymphatic malformation, a sub-cutaneous hemangioma or even the presence of dilated superficial normal veins due to underlying venous stenoses. This chapter will detail the clinical characteristics of venous anomalies and their differential diagnosis.     

Cutaneous lymphatic malformation with secondary fat hypertrophy.

Lymphatic malformations are a type of vascular anomaly. They are uncommon and can present in various ways. Associated hypertrophy of the soft tissue and bone have been described in association with deep lymphatic connections, but late-onset localised fat hypertrophy in the absence of deep connections has not been previously described. We present a case of cutaneous lymphatic malformation of the thigh and buttock with secondary fat hypertrophy. The lesion had been present since childhood but enlarged rapidly during adolescence. MRI excluded deep connections and lipoma. Liposuction was used to debulk the area with a satisfactory result.     

Surgical management of cavernous malformations of the brain stem

Different surgical approaches to the brain stem with results of excision of brain stem cavernous malformations are reported. Nine patients with brain stem cavernous malformations were operated with six different approaches. The patients were evaluated neurologically, and by magnetic resonance imaging (MRI) examination. All patients had at least one and often more episodes of haemorrhage. Seven patients underwent the total removal of the malformation, without recurrence. One patient with a large medullary cavernoma had recurrence, but without further growth. One patient with a large pontomesencephalic malformation and partial resection, suffered rebleeding due to residual cavernoma. The mean follow-up period was 49 months (range 3-112). The results of surgery were excellent or good in eight patients, and poor (worsening) in one. It is concluded that surgical resection is recommended for the cavernous malformations of the brain stem which are symptomatic, have bled or are growing, and are approachable through one of the pial surfaces of the brain stem. Different skull base approaches are useful for this procedure. Radiosurgery is not recommended in such cases.     

Neoplastic growth of cerebral cavernous malformation presenting with impending cerebral herniation: a case report and review of the literature on de novo growth of cavernomas

BACKGROUND: Cerebral cavernomas are rare vascular anomalies and their expansile growth has been considered to be mainly due to recurrent hemorrhages. They are not generally reported to show aggressive behavior.CASE DESCRIPTION: A 27-year-old male presented with headache, visual disturbances, and a 17-year history of seizures. He was known to have a temporal lobe lesion on CT scan, consistent with a diagnosis of cavernous malformation but with no relevant family history. Serial clinical and radiological follow-up revealed a progressive increase in the size of the lesion with formation of a growing cyst of 7 cm in diameter, which produced mass effect, resulting in the clinical picture of cerebral herniation. The patient underwent emergency surgical resection with symptomatic relief. There was no evidence of significant hemorrhage at surgery.CONCLUSION: Cavernous malformations can show expansile growth without any evidence of a presaging hemorrhagic event and, in the manner of a neoplastic lesion, present with raised intracranial pressure and cerebral herniation.     

Extensive cervical-mediastinal cystic lymphatic malformation treated with sclerotherapy in a child with Klippel-Trenaunay syndrome

Klippel-Trenaunay syndrome is a rare congenital disorder characterized by combined vascular malformations, varicosities, and limb enlargement. A child case of Klippel-Trenaunay syndrome with a large cervicomediastinal cystic lymphatic malformation and a vascular mass on the chest wall has been presented to demonstrate combined use of conservative and surgical approaches in this syndrome. Cystic lymphatic malformation was treated by sclerotherapies including ultrasound-guided transthoracic bleomycin injection into mediastinal cyst, which was tried for the first time in the literature, and vascular chest wall tumor necessitated surgical excision.     

Imaging in diagnosis and treatment of vascular lesions of the hand

Vascular lesions of the hand are challenging for diagnosis and treatment. These lesions are far more common than malignant tumors. Clinical presentation may be confusing and correct identification by imaging is sometimes mandatory. Standard X-rays and doppler ultrasound examination are the first modalities to be used. Doppler ultrasound help to characterize the lesion or/and to assess the flow velocity within the lesion. It allows to separate the high flow lesions (hemangioma, arteriovenous malformation) from the low or no flow lesions (venous malformation, cystic lymphangioma). MR imaging is the gold examination to define the size and the extension of the lesion to adjacent structures. Careful clinical and imaging evaluation and multidisciplinary staff decision are crucial before to propose embolotherapy and/or surgical treatment. In case of persistent doubt, a biopsy must be required. AVM must be excluded before to propose this biopsy sample. Interventional radiology can be used alone or in combination with surgery to treat vascular malformation (arteriovenous, lymphatic, venous, or mixed). Embolisation may either be done by percutaneous approach (all malformations) or by intravascular catheterization (arteriovenous malformation). Hemangioma is a benign tumor which most often spontaneously involutes with age. Vascular malformations of the hand are difficult to eradicate completely either with embolization or surgery or both. Recurrences are frequent. Management in multidisciplinary staff is mandatory and long follow-up is needed.     

Approche diagnostique et thérapeutique en imagerie des anomalies vasculaires de la main

Vascular lesions of the hand are challenging for diagnosis and treatment. These lesions are far more common than malignant tumors. Clinical presentation may be confusing and correct identification by imaging is sometimes mandatory. Standard X-rays and doppler ultrasound examination are the first modalities to be used. Doppler ultrasound help to characterize the lesion or/and to assess the flow velocity within the lesion. It allows to separate the high flow lesions (hemangioma, arteriovenous malformation) from the low or no flow lesions (venous malformation, cystic lymphangioma). MR imaging is the gold examination to define the size and the extension of the lesion to adjacent structures. Careful clinical and imaging evaluation and multidisciplinary staff decision are crucial before to propose embolotherapy and/or surgical treatment. In case of persistent doubt, a biopsy must be required. AVM must be excluded before to propose this biopsy sample. Interventional radiology can be used alone or in combination with surgery to treat vascular malformation (arteriovenous, lymphatic, venous, or mixed). Embolisation may either be done by percutaneous approach (all malformations) or by intravascular catheterization (arteriovenous malformation). Hemangioma is a benign tumor which most often spontaneously involutes with age. Vascular malformations of the hand are difficult to eradicate completely either with embolization or surgery or both. Recurrences are frequent. Management in multidisciplinary staff is mandatory and long follow-up is needed.     

Arteriovenous malformation of ureter.

Congenital arteriovenous malformations of the genitourinary tract are rare. We report the first case of an arteriovenous malformation of the ureter. The patient presented with recurrent hematuria and left flank pain. Evaluation included an intravenous urogram, cystoscopy, and retrograde pyelography. A pedunculated lesion thought to be a fibroepithelial polyp was seen at the time of ureteroscopy, and the lesion treated by excisional biopsy and fulguration. Pathologic examination demonstrated an arteriovenous malformation. After thirty-month follow-up, the patient remains asymptomatic and free of recurrence.     

Sclerotherapy for cervical cystic lymphatic malformations in children. Our experience with computed tomography-guided 98% sterile ethanol insertion and a review of the literature

Purpose

The treatment of cystic lymphatic malformations of the neck and mediastinum is controversial. Surgical management may be limited by the invasiveness of the procedure, the complex anatomy of this region, and the high recurrence rate. An alternative therapeutic method is sclerotherapy. We report our experience in the treatment of cystic lymphatic malformations of the neck by computed tomography (CT)-guided instillation of 98% sterile ethanol in children.

Methods

Eight children with clinical suspicion of cervical cystic lymphatic malformation were assessed by ultrasonography (US) and magnetic resonance imaging (MRI) to define the location, size, and number of cystic cavities. The CT-guided instillation of 98% sterile ethanol was performed. Cystic fluid was analyzed by fine-needle aspiration cytology. Clinical and US or MRI follow-up was performed after 1 and 3 months and at 1 and 2 years.

Results

The results were excellent with complete disappearance of the lesion in 7 (87.5%) of 8 patients. One patient (12.5%) with satisfactory results required a second alcohol injection with an excellent outcome. No allergic reactions or complications were observed.

Conclusions

The CT-guided 98% sterile ethanol sclerotherapy is a good alternative to surgical therapy. This procedure seems accurate, minimally invasive, safe, low cost, and reliable without untoward complications. Moreover, it does not exclude later surgical treatment.     

Sclerotherapy to a large cervicofacial vascular malformation: a case report with 24 years' follow-up

BACKGROUND: Large craniofacial venous malformations frequently cause significant cosmetic and functional problems. Treatment of the lesions early in life helps to avoid these problems. We present a case of a large cervicofacial venous malformation. METHODS: The patient was treated with a 5% benzyl alcohol solution of sodium morrhuate. The treatment was begun when the patient was 3 months of age, with a total of 23 injections. RESULTS: The lesion had completely disappeared by age 10. No major complications were observed except a superficial tissue loss on the temporal region. It was healed by conservative treatment. There were no recurrences during the 14-year follow-up. CONCLUSIONS: It is difficult to conclude that this type of treatment is the best choice for patients with venous malformation on the basis of a single case. However, intralesional sclerotherapy should be kept in mind as a saving treatment option in cases of large venous malformations in anatomic regions that present challenges to both the surgeon and the patient. Intralesional sclerotherapy can be performed without serious complications if the sclerosing agent is selected and injected cautiously.     

Complex right hemisphere developmental venous anomaly associated with multiple facial hemangiomas. Case report

Complex developmental venous anomalies (DVAs) represent variations of normal cerebral venous drainage and consist of dilation of the superficial and/or deep venous system. These rare anomalies can occur unilaterally or bilaterally, supratentorially or infratentorially, focally or they can affect the entire hemisphere. Some DVAs are associated with cervicofacial venous malformations or facial lymphatic malformations. Anomalies of this type are generally clinically silent, and cerebral dysfunction is usually absent. Symptoms, when they occur, are most commonly headache or mild seizure disorders. The angiographic findings are striking, with well-formed but enlarged transcerebral medullary and deep and/or superficial cortical veins. Opacification of these venous structures occurs within the same time frame as a normal angiographic venous phase. The authors report the case of a 33-year-old man in whom a large inoperable arteriovenous malformation had been previously diagnosed and who presented with seizures. Repeated magnetic resonance imaging and angiography demonstrated abnormally dilated transcerebral, superficial, and deep venous structures involving the entire right hemisphere with no identifiable nidus. Additionally, multiple bilateral benign facial hemangiomas were present in this patient. It is important to recognize this rare venous appearance as a developmental variant and not mistake it for an arteriovenous malformation or a partially thrombosed vein of Galen malformation. Because these venous anomalies are extreme variants of the normal venous system, hemorrhage rarely, if ever, occurs and the patient can be reassured that no interventional or surgical therapy is necessary or warranted.     

Cavernous malformations of the brain stem

Once they become symptomatic, cavernous malformations of the brain stem appear to cause progressive morbidity from repetitive hemorrhage, and can even be fatal. Twenty-four patients with long-tract and/or cranial nerve findings from their cavernous malformations of the brain stem were seen for initial evaluation or surgical consultation and thereafter received either surgical or continued conservative treatment. The decision to operate was based on the proximity of the cavernous malformation to the pial surface of the brain stem, the patient's neurological status, and the number of symptomatic episodes. Sixteen patients were treated by definitive surgery directed at excision of their malformation. In four patients, associated venous malformations influenced the surgical approach and their recognition avoided the risk of inappropriate excision of the venous malformation. Although some of the 16 patients had transient, immediate, postoperative worsening of their neurological deficits, the outcome of all except one was the same or improved. Only one patient developed recurrent symptoms: a new deficit 2 1/2 years after surgery required reoperation after regrowth of the cavernous malformation. She has been neurologically stable since the second surgery. One patient died 6 months postoperatively from a shunt infection and sepsis. The eight conservatively treated patients are followed with annual magnetic resonance imaging studies. One has a dramatic associated venous malformation. Seven patients have either minor intermittent or no symptoms, and the eighth died from a hemorrhage 1 year after his initial presentation. Based on these results, surgical extirpation of symptomatic cavernous malformations of the brain stem appears to be the treatment of choice when a patient is symptomatic, the lesion is located superficially, and an operative approach can spare eloquent tissue. When cavernous malformations of the brain stem are completely excised, cure appears permanent.     

Proteus syndrome in adulthood

Proteus syndrome is a very rare congenital condition comprising malformations and overgrowth of multiple sorts of tissue. It was described for the first time in 1979 and was termed Proteus syndrome in 1983. The authors describe a 37-year-old patient who was diagnosed initially as having Klippel-Trenaunay-Weber syndrome at the age of 10 years. The patient was operated for a major thoracic lymphatic malformation, which caused functional problems. The operation at older age for this large lymphatic malformation proved to be complex. In addition, the authors address the difficulties in diagnosing Proteus syndrome. Vascular malformations causing functional problems in adulthood require major surgical procedures with a high risk of postoperative complications.     

Use of the deep inferior epigastric perforator flap in a 15-month-old boy after resection of lymphatic malformation

Lymphatic malformations are benign tumors that arise from congenital defects of the lymphatic system. Despite the fact that all lymphatic malformations develop embryologically, their presence may not be detected until the tumor enlarges from hemorrhage or infection. Typical of these tumors is a slow-growing, asymptomatic phase with the potential for rapid and massive expansion and a tendency to infiltrate into surrounding tissues. These tumors are associated with a high rate of recurrence, especially with incomplete excisions. To minimize the chance of recurrence, a complete resection of a groin microcystic lymphatic malformation was performed on this 15-month-old boy, creating a significant defect in the upper thigh. Immediate coverage was felt to be the best option for reducing any associated morbidity. We used a skin-soft tissue flap supplied by perforators of the deep inferior epigastric artery to repair the wound. Using this technique, the musculofascial structures in the abdominal wall were completely spared, and the skin and fat were transferred on a vascular leash to the desired location. The donor site was closed by approximating the fascial edges without tension. The defect on the thigh was filled with tissue of similar thickness and characteristics, avoiding potential contour irregularities and color mismatch. The deep inferior epigastric perforator (DIEP) flap is a useful option for groin defects and can be applied safely in the pediatric population.     

Cavernous-venous malformation of brain stem--report of a case and review of literature.

BACKGROUND: Because of improved imaging techniques, the association of cavernous and venous malformations is now being seen more often in neurosurgical practice. However, only a few cases have been subjected to surgery. Surgical excision of the cavernous malformation, sparing the venous component, has been the method of choice advocated by most authors. This, however, is not possible when the components are intimately associated with each other. It thus becomes important to predict the surgical outcome in such lesions, especially when they are located in an eloquent area. A surgically excised case of cavernous-venous malformation in the dorsal pontomedullary region is presented here; the histology confirmed the intimate association of both components. The relevant literature is also discussed. CASE DESCRIPTION: This 20-year-old male presented with episodic vomiting, giddiness, and persistent cerebellar signs over a six-year period. Neuroimaging studies-computed tomography scan/magnetic resonance imaging (CT/MRI) were suggestive of a cavernous malformation. MRI also showed a draining vein close to the lesion. Following total excision, histopathology confirmed the intimate association of both cavernous and venous malformations. The patient made an uneventful recovery. MRI at follow up confirmed total excision. CONCLUSIONS: The presence of venous channels draining into a lesion that otherwise appears to be a cavernous malformation may indicate an intimately associated venous component. Selective surgical excision of the cavernous component may be difficult in such cases. Surgical outcome, though favorable in this case, will be difficult to comment upon unless more such cases are reported. This may be of greater significance in lesions located in eloquent areas.