Complications After Treatment of Head and Neck Venous Malformations With Sodium Tetradecyl Sulfate Foam.

PURPOSE: The aim of this study was to evaluate complications in patients with head and neck venous malformations (VMs) treated with foam sclerotherapy using sodium tetradecyl sulfate (STS). METHODS: The authors retrospectively evaluated the complications, pain. and degree of satisfaction in 69 consecutive patients affected by cervicofacial VM managed with STS using the Tessari method in a single institution. RESULTS: The average number of procedures for each patient was 2.1. The most frequent complication was blistering. We observed 1 patient of temporary weakness of a facial nerve branch, 1 paradoxical embolism, and 1 orbital compartment syndrome.The average pain score was 0 (no pain at all) (51.5%). There was no statistically significant correlation between patient satisfaction and the presence of complications or the degree of pain. CONCLUSIONS: Sclerotherapy with STS is an effective treatment that yields to very high patient satisfaction. This procedure has an overall low complication rate and is usually effective within a few sessions. However, severe complications may occur; these must be pointed out in the informed consent and the surgeon must be aware of and ready to quickly treat them to prevent long-term sequelae.

Sixteen years of experience with persistent chylothorax in children.

BACKGROUND: Persistent chylothorax in children is rare. Conservative management represents the gold standard but, in case of failure (persistent effusion or relapse), surgery must be considered. This paper aimed at presenting our series of patients who underwent surgical treatment of persistent idiopathic chylothorax and at discussing the role of thoracic duct ligation in its management. METHODS: We included all patients who underwent surgery for persistent chylothorax in the period between January 1994 and January 2010. RESULTS: Nine patients were included (median age 25 months). Five patients had primitive or idiopathic chylothorax. Five patients had right-sided chylothorax, 3 left-sided, and 1 bilateral. Pleurodesis was applied to 8 patients (bilateral in one) and thoracic duct ligation to 4 patients for a total of 12 procedures in 9 patients. Complete cessation occurred within a median of 5 days (range 2 to 10) after thoracic duct ligation and 10 days (range 4 to 25) after pleurodesis. In 3 patients (all with right sided effusion and a median daily output higher than 20 ml/kg) pleurodesis failed and thoracic duct ligation was subsequently required to definitively treat chylothorax. Conversely, 5 patients were effectively treated with pleurodesis and 1 with thoracic duct ligation alone. Regardless of previous procedures, none of the patients who underwent thoracic duct ligation experienced relapses. CONCLUSIONS: Although based on a small number of patients, our experience confirmed that thoracic duct ligation represents an effective therapeutic option for persistent unresponsive chylothorax. In cases of right sided effusion with high output rate (>20 mL/kg) thoracic duct ligation might be considered as first choice treatment.

A case of congenital hypothyroidism in PHACE syndrome.

Although hemangiomas, benign tumors of vascular origin, are very common among children and represent the most frequent benign tumor at that age, their association with other malformations constitutes a rare neurocutaneous disorder called PHACE syndrome. This condition is characterized by posterior fossa anomalies, hemangioma of the face, arterial alterations, cardiac defects, and eye anomalies (as represented by the acronym PHACE); sternum defects, endocrinopathies, and thyreopathies may be present as well. In this report, we describe a case of congenital hypothyroidism due to an empty thyroid site, as demonstrated by ultrasound, in an Italian child.

Intra-articular venous malformations of the knee.

BACKGROUND: Intra-articular venous malformations (IAVM) of the knee represent a rare group of low-flow vascular malformations, mainly reported in the literature as synovial hemangiomas, usually with an onset in early childhood. The main symptoms and signs are knee pain, swelling, and hemarthrosis. These lesions are slowly progressive and can lead to chronic synovitis and joint damage. Confusing nomenclature and classification of these lesions have often led to misdiagnosis and inappropriate treatment. We report our experience in the treatment of 14 consecutive patients affected by knee IAVM. METHODS: We carried out a retrospective study based on the review of the medical records and diagnostic imaging of the patients admitted to our department from October 1999 to June 2009, and discharged with the final diagnosis of IAVM of the knee. RESULTS: We observed 14 consecutive patients (8 boys, 6 girls); the median age at symptom onset was 5 years (range, 2.5 to 13 y). Magnetic resonance imaging was diagnostic in all cases. Surgical resection associated with a wide synoviectomy was the therapeutic procedure of choice in all cases. Within 6 months after surgery and physiotherapy, all the patients were symptom free with a full or at least acceptable knee range of motion. CD34 staining carried out in the last 7 cases of our series showed labeling of the endothelium of the thick-walled vessels, whereas the endothelium of the thin-walled vascular spaces was not stained. This finding of unstained vascular spaces suggests that a component of lymphatic vessels mixed with dysplastic blood vessels may be frequently present in these abnormalities. CONCLUSIONS: Magnetic resonance imaging plays a pivotal role in identifying the lesion. Surgical excision is always indicated and should be performed as early as possible to avoid lesion progression and to reduce the risk of chondral degeneration. According to the microscopic features of our resected specimens, we suggest that these lesions of the knee should be more properly named as IAVM instead of hemangiomas. LEVEL OF EVIDENCE: Level IV.

An angiomatoid fibrous histiocytoma over the left pre auricular region in a 13-year-old boy.

A 13-year-old boy presented to us for the evaluation of a slowly growing mass over the left preauricular region. Physical examination showed a nodular swelling, firm on palpation. The patient had no lymphadenopathy. The findings of magnetic resonance imaging (MRI) and vascular MRI led us to suspect a vascular growth. An incisional biopsy was performed and revealed an angiomatoid fibrous histiocytoma (AFH). Angiomatoid fibrous histiocytoma (AFH) is a rare tumor of the soft tissue with fibrohistiocytic and vascular differentiation that shows an intermediate malignancy grade, mainly occurs in patients younger than 20 years of age, and is usually localized on the extremities. Angiomatoid fibrous histiocytoma is considered to be a tumor of intermediate malignancy because of its less aggressive course in contrast to conventional malignant fibrous histiocytoma (MFH). Our case of AFH is peculiar because of its localization in the pre auricular region and because it appeared in a young patient. Surgical excision with maxillo-facial, chest-abdomen, and neck CT and prolonged follow-up is recommended because rare cases of metastasizing AFH have been reported.

Safety and effectiveness of oral propranolol for infantile hemangiomas started before 5 weeks and after 5 months of age: an Italian multicenter experience.

BACKGROUND: Despite not being licensed for the treatment of infantile hemangiomas (IH) in infants younger than 5 weeks or older than 5 months, propranolol is often used in these age groups to prevent or to treat potentially severe complications. The objective of the present study was to review the experience of 8 Italian pediatric and dermatologic centers regarding propranolol treatment for IH started before 5 weeks or after 5 months of age. METHODS: We retrospectively reviewed the records of patients followed up for IH, on propranolol treatment started before 5 weeks or after 5 months of age, and collected information on sociodemographic data, treatment indications, IH involution, IH relapse, and treatment side effects. RESULTS: A total of 343 patients were enrolled; 15 were started on propranolol before 5 weeks (group 1), 328 were started after 5 months of age (group 2). The most frequent indications were permanent aesthetical disfigurement (91.8%) and function threatening complications (42.6%). In most cases, the treatment was effective. The involution was partial in 67.7% of patients. In 11.8% of cases a relapse was observed. No relapse was observed in group 1. Treatment complications were reported in 15.8% of children, most frequently sleep disorders (6.6%), followed by irritability (5.1%) and diarrhea (2.2%). Only a case of mild constipation was observed in group 1. CONCLUSION: The safety and effectiveness profile of propranolol in infants younger than 5 weeks or older than 5 months may be acceptable. Taking in account propranolol's potential in preventing severe complications, further studies should assess the acceptability of propranolol treatment, especially in the <5-week age group .

Adjuvant role of anti-angiogenic drugs in the management of head and neck arteriovenous malformations.

Arteriovenous malformations (AVMs) are high-flow vascular malformations characterised by a complex vessel network directly connecting feeding arteries and draining veins, typically featured by a natural history of progression, while spontaneous regressions are purely anecdotal. AVMs are very aggressive entities that possess a locally infiltrative behaviour like neoplasms. Complete "radical" surgical excision presents the highest chance of cure, but nowadays there is still considerable controversy on how to treat large AVMs that are not amenable of "radical" excision. The aim of this paper is to propose a different approach to treat vast AVMs that cannot be removed radically. The association of an antiangiogenic drug (to be initiated before surgery and to be continued in the post-operative period), could prevent the feared "explosive" growth of the remaining nidus after its partial removal. This could make recontouring and other "aesthetically" focused procedures feasible in these patients, with an obvious leap in their quality of life. The most promising antiangiogenic drug seems to be Thalidomide, but other drugs such as Sirolimus, VEGF pathway inhibitors, Interferon or Matrix Metalloproteinase (MMP) Inhibitors could serve the purpose just as well. Even Propranolol could prove useful in this sense as suggested by some recent researches on retinopathy of prematurity and tumour biology.

The use of propranolol for complicated infantile hemangiomas.

OBJECTIVE: To assess propranolol efficacy and safety in complicated infantile hemangiomas in two different age groups. PATIENTS AND METHODS: We report on 68 infants with infantile hemangiomas treated with oral propranolol at the lowest effective dose at different ages for a period of six months. Inclusion criteria were life-threatening hemangiomas, function-threatening hemangiomas, facial hemangiomas with risk for disfigurement, and extensive and ulcerated hemangiomas. A previously designed safety protocol was applied to all patients. The evolution of all hemangiomas since baseline (pre-therapy) until the end of follow-up was assessed on the basis of clinical features (color, palpable softening, size, and volume) and taken at follow-up visits. RESULTS: Our results showed that propranolol was effective in arresting the proliferative phase and in accelerating the involution of infantile hemangiomas in 92.6% of cases. Propranolol efficacy was clear even when it was started after 12 months of life at low dose; after discontinuation of therapy there was a moderate-to-severe regrowth in 9.3% of cases and a mild regrowth in 22.5%. No adverse events were observed. CONCLUSIONS: Propranolol should be used as first-line medical treatment in all cases of complicated infantile hemangiomas.

Nasal tip haemangiomas: guidelines for an early surgical approach.

The treatment of Cyrano nose haemangioma (CNH) is difficult because of its location and possible complications: psychological impact, severe skin infiltration and consequences on nasal growth. We suggest that the best treatment for nasal tip haemangiomas is an early surgery to remove the affected tissues and preserve the anatomy. A total of 39 children (32 females and seven males) underwent early surgery for the treatment of CNH. Mean age was 35 months. Skin infiltration was present in 15 cases. Cartilage lack or distortion was observed in 29 cases. Each patient was evaluated for global cosmetic appearance, reduction in volume of the tumour, improvement of skin texture and quality of the scar. Multiple surgical procedures were performed in 14 cases. The average postoperative follow-up was 48 months. Patients with low-volume tumours had only one surgery, whereas patients with large tumours underwent a mean of 1.9 surgeries. In 29 cases, distortion or lack of cartilaginous structures required dissection and approximation of the alar cartilages in their anatomical position. We could identify three types of CNH that lead to three distinct surgical approaches: type A (mild cases) is characterised by no cutaneous involvement, no misalignment of the cartilages and mild nasal volume increase; type B (moderate cases) entails partial cutaneous infiltration, misalignment of the cartilages and moderate nasal volume increase; and type C (severe cases) is characterised by cutaneous infiltration, misalignment of the cartilages and severe nasal volume increase.