1/2439. Solitary hepatic hemangioma in a newborn infant complicated by cardiac failure, consumption coagulopathy, microangiopathic hemolytic anemia, and obstructive jaundice. Case report and review of the literature. A newborn infant with a large hepatic hemangioma developed congestive heart failure, consumption coagulopathy, microangiopathic hemolytic anemia, and obstructive jaundice. The patient was mildly heparinized (250 units per kg and day) and underwent successful resection of the tumor without lobectomy at the age of 3 days. blood volume increased from 93.9 ml/kg at the age of 5 h to 124.2 ml/kg prior to surgery. Red-cell mass simultaneously decreased from 53.8 to 39.4 ml/kg. The increase of blood volume is explained by congestive heart failure, the decrease of red-cell mass by intravascular coagulation within the tumor resulting in formation of thrombi and microangiopathic hemolytic anemia. A review of the literature on infants with symptoms caused by an intrahepatic hemangioma during the first month of life confirms that surgical intervention is the treatment of choice for infants with giant solitary hemangioma of the liver. ( info) |
2/2439. Phakomatosis pigmentovascularis: A new case with renal angiomas and some considerations about the classification. We report phakomatosis pigmentovascularis detected in a Caucasian child characterized by the presence of a nevus flammeus and nevus anemicus on the face, a telangiectatic linear nevus of the right leg, and a very extensive blue spot covering 60% of the body surface, with ocular melanosis. Multiple angiomatous lesions of the kidney are associated without alterations of the central nervous system (CNS). This association has not been reported before; it could be a further expression of the complex of developmental defects. Our case corresponds exactly to type IIb in the classification of phakomatosis pigmentovascularis proposed by Hasegawa. As this classification seems very extensive, the higher incidence of cases corresponding to the second subtype suggests that we should identify it by the term phakomatosis pigmentovascularis, while the others could be considered as only very uncommon variants. ( info) |
3/2439. The rebound effect in the treatment of complex hemangioma with interferon alpha 2A. The authors report the case of an infant with an extensive face hemangioma with subglottic airway obstruction which had been successfully treated with interferon alpha 2A but then reoccurred with the same dimensions and airway blockage after treatment was abruptly interrupted. The authors suggest the implementation of a standard procedure for the interruption of interferon alpha 2A treatment in order to avoid this rebound effect and advise on the need for further studies to properly evaluate dosage and administration parameters for interferon alpha 2A in the treatment of difficult hemangioma. ( info) |
| Four cases of renal angiomyolipoma are presented, 3 of which were diagnosed in non-tuberous sclerosis patients. In 1 case diagnosis was made preoperatively and in another case it was made intraoperatively, allowing for preservation of functioning renal parenchyma. The second successful kidney transplant in a patient with tuberous sclerosis and renal failure is reported. One cannot always differentiate renal angiomyolipomas from adenocarcinoma. However, if the classical angiographic findings of sacculated pseduo-aneurysms supplied by the interlobular and interlobar arteries are present non-operative observation or limited surgery with preservation of renal tissue is possible. Also, knowledge of the gross pathologic appearance and the syndrome of tuberous sclerosis will allow one to make a preoperative or intraoperative diagnosis with confidence. ( info) |
5/2439. Benign renal angiomyolipoma with regional lymph node involvement. The 2 cases reported herein involve benign renal angiomyolipomas, showing the same angiomyolipoma changes in the regional lymph nodes. It is concluded that these lymph node changes are caused by a multicentric origin of the angiomyolipoma rather than true metastasis. A plea is made not to over treat these tumors since all evidence points to the fact that they are indeed benign. ( info) |
| We report an unusual case of arteriovenous (AV) malformation (localized form of soft tissue angiomatosis): a reaction of angiolymphoid hyperplasia with eosinophilia (ALHE) developed over the lesion of this pre-existing AV malformation. To the best of our knowledge, there is no other report of ALHE in association with a pre-existing bona fide AV malformation. A 71-year-old woman consulted our clinic because a reddish, dome-shaped tumor on her preauricular region, present as a slightly elevated, skin-colored tumor since her childhood, had rapidly enlarged and become more elevated with color change during the preceding two months. The histological features of most of the lesion corresponded to those of soft tissue angiomatosis (localized form). The proliferative vessels underlying the soft tissue angiomatosis streamed out and welled into the dermis with an accompanying prominent inflammatory infiltrate composed of lymphoid cells and eosinophils: these histological features were characteristic of ALHE. The recent abrupt clinical changes in the tumor were considered to represent a reaction to the angiomatosis with development of ALHE. ( info) |
7/2439. Successful treatment of obstructive sleep apnea with use of nasal continuous positive airway pressure in three patients with mucosal hemangiomas of the oral cavity. cysts and benign tumors are uncommon causes of obstructive sleep apnea (OSA), and surgical removal is usually favored. In patients in whom an operation poses a high risk, however, nasal continuous positive airway pressure (CPAP) may prove beneficial. We describe three patients with hemangiomas of the oral cavity in whom polysomnography revealed moderate to severe OSA. In all three patients, nasal CPAP effectively decreased sleep-related disordered breathing events and dramatically improved their sleep. To our knowledge, this is the first report of OSA associated with hemangiomas involving the upper airway. Our experience suggests that nasal CPAP therapy is effective and well tolerated in such patients. ( info) |
8/2439. Anterior approach to intramedullary hemangioblastoma: case report. OBJECTIVE AND IMPORTANCE: Intramedullary spinal cord tumors are generally operated on by using the posterior approach. However, the posterior approach may not be suitable for a tumor in the anterior part of the spinal cord. In this report, we describe a case of a cervical intramedullary tumor that was successfully removed by using the anterior approach. CLINICAL PRESENTATION: A 48-year-old woman presented with lower cranial nerve disturbance and motor weakness of the upper extremities. magnetic resonance imaging revealed a large extensive syrinx and an intramedullary enhanced tumor at the C6 level. The tumor was located at the left of the anterior part of the spinal cord. INTERVENTION: Based on these findings, the anterior approach was used in performing a corpectomy of C5 and C6. The tumor was highly vascular and was resected without resulting in any operative deficits. The pathological diagnosis was hemangioblastoma. CONCLUSION: The present case suggests that the anterior approach is an important option among surgical approaches to the intramedullary tumor in cases in which the tumors are small in size and are located in the anterior part of the cervical cord. ( info) |
9/2439. Focal aneurysmal dilatation of subchorionic vessels simulating chorioangioma. Subchorionic vascular aneurysms of the placenta are rare lesions and may present confusion with chorioangioma or focal mesenchymal dysplasia on sonography. To our knowledge, the findings of placental aneurysms have not been reported in the ultrasound literature. We present a case with detailed sonographic evaluation, including spectral and color Doppler and pathological analysis, that was mistaken for chorioangioma prenatally. knowledge of this benign entity may allow the sonologist to recommend conservative management in similar cases. ( info) |
10/2439. Childhood lipoma arborescens presenting as bilateral suprapatellar masses. Synovial lipomatous proliferations are uncommon idiopathic lesions. Suprapatellar synovial plicae commonly are diagnosed with mechanical knee problems. However, it is not widely known that these plicae can isolate the suprapatellar pouch from the rest of the knee joint. We describe a case of complete bilateral compartmentalization of the suprapatellar pouch (plica synovialis suprapatellaris) in which a 10-year-old boy developed articular tumors isolated in this area. Arthroscopic synovectomy specimens revealed the lesions to represent lipoma arborescens. Rheumatologists should be aware of these two findings when examining a child with swollen knee. ( info) |