Blue rubber bleb nevus syndrome is a rare disorder that is characterized by multiple recurrent vascular malformations, such as hemangioma, and these primarily involve the skin and the gastrointestinal tract. It may also involve the brain, liver, lungs, and skeletal muscles. A 14-year-old female visited our hospital with a chief complaint of dizziness; upon examination, we found multiple recurrent hemangiomas on the skin and gastrointestinal tract. We were able to diagnose her as suffering from blue rubber bleb nevus syndrome and we treated her with methylprednisolone (2 mg/kg/day for 1 month and 1 mg/kg/day for additional 3 months). We report on this case along with a review of the literature.
Adolescent ; Female ; Gastrointestinal Neoplasms/*diagnosis/therapy ; Hemangioma/*diagnosis/therapy ; Humans ; Nevus, Blue/*diagnosis/therapy ; Skin Neoplasms/*diagnosis/therapy ; Syndrome

Infant hemangioma, the most common benign tumor in children, is characterized by rapid proliferation, followed by slower spontaneous involution. However, some patients with facial segmental hemangioma are associated with PHACE syndrome. PHACE syndrome is characterized by vascular nerve and vascular cutaneous lesions of multiple systemic systems, often resulting in structural and functional impairments. Recent studies have demonstrated that the possible pathogeneses of PHACE syndrome mainly include hypoxia, abnormality of mesodermal vascular endothelial cells, genetic abnormality, and abnormality of interstitial mesenchymal stem cells. The current medications for hemangioma with PHACE syndrome include beta blockers, glucocorticoids, and mTOR inhibitors. This review article mainly describes the pathogenesis, diagnoses and treatments of PHACE syndrome, in order to provide directions for diagnosis and treatment of this disorder.
Abnormalities, Multiple ; diagnosis ; etiology ; therapy ; Eye Abnormalities ; diagnosis ; etiology ; therapy ; Heart Defects, Congenital ; diagnosis ; etiology ; therapy ; Hemangioma ; diagnosis ; etiology ; therapy ; Humans ; Infant

Child ; Child, Preschool ; Face ; blood supply ; Female ; Hemangioma, Capillary ; congenital ; diagnosis ; therapy ; Humans ; Infant ; Lymphatic Abnormalities ; diagnosis ; therapy ; Male ; Mouth ; blood supply ; Vascular Malformations ; diagnosis ; therapy

Face ; blood supply ; Hemangioma ; classification ; diagnosis ; therapy ; Humans ; Jaw ; blood supply ; Lymphatic Abnormalities ; classification ; diagnosis ; therapy ; Mouth ; blood supply ; Vascular Malformations ; classification ; diagnosis ; therapy

OBJECTIVE: To discuss the diagnosis and treatment method in children with mass in subglottic. METHOD: There were totally 35 patients with subglottic mass who complained with dyspnea in our department. All the patients got electronic laryngoscopy examination in order to get the preliminary judgment of the mass' property. Then they accepted ultrasound and CT scan to make the definite diagnosis. The patients with subglottic hemangioma accepted oral propranolol or intralesional Pingyangmycin injection. The other type of masses got the laryngoscope CO2 laser tumor resection. RESULT: There were 31 patients with subglottic hemangiomas, 2 patients with subglottic cysts, 1 patient with subglottic fibroma, 1 patient with subglottic granuloma. The substantial follow-up time was from 1 month to 3 years. 25 patients with subglottic hemangiomas were cured, 6 patients got improvement. The patients with non-hemangiomas were all cured. CONCLUSION There was a high incidence of hemangioma in children with subglottic mass. We suggested making a diagnosis by doing electronic laryngoscopy, ultrasound and CT scan step by step. Oral propranolol was a safe and effective method in treating subglottic hemangioma. For the patients with non-hemangiomas, we considered the laryngoscope CO2 laser tumor resection as the first choice of treatment.
Child ; Glottis ; pathology ; Hemangioma ; diagnosis ; therapy ; Humans ; Laryngeal Diseases ; diagnosis ; therapy ; Laryngeal Neoplasms ; diagnosis ; therapy ; Laryngoscopy ; Larynx ; pathology ; Tomography, X-Ray Computed

We can classify the vasular anomalies into hemangiomas and vascular malformations in accordance with Mulliken's classification. But most surgeons and physicians have not yet been used to these terminologies and so incoherent terms have frequently caused problematic confusion in the interdepartment discussion, many trial and errors and uncontrollable patients' distrust with the waste of time in spite of various kinds of therapeutic practices. It is primarily important that the selection of therapeutic methods must be decided, assuming the usage of an accurate diagnosis and academic terms. The differntial diagnosis between hemangiomas and superficial vascular malfomations (capillary or venous malformations) is especially bewared and then the surgical resection is always considered as a method of treatment modalities, especially at any time whenever other practices end in failure or ineffective. Sometimes, patient's descriptions and clinical features of lesions during follow up periods are more helpful in the diagnosis of lesions than other studies especially in long-term or recurred, complicated vascular lesions. In primay venous malformations, we have tried surgical resections and in recurred cases radical resection and immediate reconstruction with normally vascularized flap have been applied. Such normally vascularized island and free flaps can prevent the postoperative ischemic bed from stimulating the dormant or inactivated shunts and causing new pathologic potentials of recurrence. Over a 2-year period, 16 patients underwent surgery. Of the 16 patients, 8 superficial vascular anomalies had previous malpractices such as inappropriate laser therapy or steroid. They had all surgical resection and reconstructive procedure if needed. The another 8 patients of venous malformations involving the face, neck and hand had radical resection and reconstruction with a normally vascularized island or free flap for prevention of recurrence. The mean follow-up period was 11 months and no recurrence was observed.
Classification ; Diagnosis ; Follow-Up Studies ; Free Tissue Flaps ; Hand ; Hemangioma ; Humans ; Laser Therapy ; Neck ; Recurrence ; Vascular Malformations

OBJECTIVE: To discuss the diagnosis and treatment of infantile congenital subglottic hemangioma. METHOD: Nine patients with recurrent stridor, feeding difficulties and laryngeal obstruction were diagnosed as congenital subglottic hemangioma with bronchial endoscopy. The lesions were unilateral in 7 cases and bilateral in 2 cases. Eight cases were treated by intralesional Pingyangmycin injection after tracheotomy. Patients were discharged with metal trachea and were followed up endoscopically 2 weeks thereafter. A second treatment would be required if the tumor reduced in size but not disappeared completely. Plugging tubes and extubation would be done when the tumor disappeared completely. One case with unilateral lesion underwent power-assisted tumor resection without tracheotomy. RESULT: Hemangioma vanished completely in 6 patients with unilateral subglottic hemangioma who were treated with single stage procedure. Tumor size was found reduced significantly after one procedure and vanished completely after a second procedure in 2 cases with bilateral subglottic hemangioma. All of the 8 cases were extubated successfully. The patient recovered thoroughly in two weeks after power-assisted tumor resection. CONCLUSION Intralesional Pingyangmycin injection after tracheotomy can relief the airway obstruction quickly, shorten the nature course of hemangioma, shorten the period of wearing trachea, has no complication such as subglottic stenosis and is thought to be a safe and effective therapy for large size hemangioma. Power-assisted tumor resection under good total anesthesia without tracheotomy combined with well hemostasis is effective, less injure, and suitable for small size hemangioma.
Antibiotics, Antineoplastic ; therapeutic use ; Bleomycin ; analogs & derivatives ; therapeutic use ; Female ; Glottis ; Hemangioma ; congenital ; diagnosis ; therapy ; Humans ; Infant ; Laryngeal Neoplasms ; congenital ; diagnosis ; therapy ; Male ; Treatment Outcome

OBJECTIVETo observe the management results of hemangioma in infants and children.

METHODSForty-nine cases of hemangioma in infants and children managed from 1986 to 2004 were reviewed. During the management period, the incidence and first visit age and sex were recorded. The location and volume of the lesions were photographed and followed up at an interval of 3 to 12 months until the complete involution of the tumor. The treatment modalities used included local injection of prednisone in 16 cases, surgery in 6 cases, and the remaining 33 cases were observed without treatment.

RESULTSThirty-three cases receiving no treatment involuted naturally. The complete involution age was found from 9 months to 10 years with average age of 5.4 years. The cosmetic results after involution were good.

CONCLUSIONSMost hemangiomas do not need treatment and can involute naturally. However, in cases with severe complications such as expansion, destruction and obstructive or congestive heart failure, treatment is indicated.

Child ; Child, Preschool ; Facial Neoplasms ; diagnosis ; physiopathology ; therapy ; Female ; Follow-Up Studies ; Hemangioma ; diagnosis ; physiopathology ; therapy ; Humans ; Infant ; Lip Neoplasms ; diagnosis ; physiopathology ; therapy ; Male ; Neoplasm Regression, Spontaneous ; physiopathology ; Parotid Neoplasms ; diagnosis ; physiopathology ; therapy ; Retrospective Studies

OBJECTIVE: To review the application of medical imaging in diagnosis and management of fetal hepatic hemangioma. METHODS: Clinical data and imaging findings of 14 cases of fetal hepatic hemangioma, who were diagnosed prenatally and followed up in Women's Hospital of Zhejiang University School of Medicine from February 2014 to September 2018 were retrospective reviewed. RESULTS: The fetal hepatic hemangiomas were single lesions in all 14 cases, and most of them were located in the right lobe of the liver (13/14). Ultrasound images were mainly hypoechoic with heterogeneity, the honeycomb-like or grid-like anechoic regions were presented in 9 lesions and circumferential blood flow was observed with low to moderate blood flow resistance index. MRI findings showed well-defined lesions with low signal intensity on T1WI, and high or slightly high signal intensity on T2WI. Among 14 cases, there were 8 cases of induced labor and 6 cases of continuing pregnancy. In 6 cases of successful delivery, 2 were treated with propranolol, 4 cases were followed-up without treatment. The growth and development of 6 children were normal. The lesions of hepatic hemangioma showed no significant changes in 3 children and were reduced in the other 3 children, of whom the lesion was complete disappeared in 1 case. CONCLUSIONS Fetal hepatic hemangiomas present relatively typical imaging characteristics, and prenatal diagnosis can be made with ultrasound and MRI. If there are no complications, the fetus with hepatic hemangioma can be delivered at full term with a good outcome.
Female ; Fetus ; Hemangioma ; diagnostic imaging ; therapy ; Humans ; Liver Neoplasms ; diagnostic imaging ; therapy ; Magnetic Resonance Imaging ; Pregnancy ; Pregnancy Outcome ; Prenatal Diagnosis ; Retrospective Studies

Herein, we report a neonatal case of an extra-renal non-cranial malignant rhabdoid tumor with simultaneous tumor in the adrenal gland, which was initially diagnosed with hemangioma. The patient showed a soft tissue mass on the chin at birth which grew rapidly with invasion of lower lip. Despite multimodal treatment including surgery, chemotherapy and high dose chemotherapy followed by autologous stem cell transplantation, the patient died of disease progression at the age of 20 months finally. The clinical presentation, diagnosis, pathology, treatment, and prognosis for this case are discussed.
Adrenal Glands ; Chin ; Combined Modality Therapy ; Diagnosis ; Disease Progression ; Drug Therapy ; Hemangioma ; Humans ; Infant, Newborn ; Lip ; Neoplasm Metastasis ; Parturition ; Pathology ; Prognosis ; Rhabdoid Tumor ; Stem Cell Transplantation