Hemangioma infantil e propranolol oral – recomendações atuais

O hemangioma infantil é um tumor endotelial benigno caracterizado por um rápido crescimento nos primeiros meses de vida e regressão espontânea nos anos subsequentes. Desde 2008, o uso do propranolol oral tem sido crescente com eficácia superior à corticoterapia sistémica e ótimo perfil de segurança, assumindo uma posição de primeira linha no tratamento do hemangioma infantil em idade pediátrica. Na presente revisão, os autores propõem uma atualização sobre as mais recentes recomendações para a abordagem dos doentes com hemangioma infantil e indicação para tratamento com propranolol oral.

Submucosal Hemangioma of the Trachea in an Infant: Diagnosis and Follow-Up with 3D-CT/Bronchoscopy

Introduction: Infantile hemangiomas of the airway are diagnosed at bronchoscopy as part of the investigation of stridor or other respiratory symptoms. Here, we present three-dimensional computed tomography (3D-CT)/bronchoscopy findings of submucosal subglottic hemangioma missed at bronchoscopy. Case Presentation: We report on the clinical usefulness of 3D-CT/bronchoscopy as the primary diagnostic tool and follow-up method in the evaluation of suspected airway infantile hemangiomas, especially when the hemangioma is the submucosal type. Conclusions: 3D-CT/bronchoscopy will reduce the need for invasive laryngoscopic studies and help to diagnose submucosal hemangiomas undetected on laryngoscope. Additionally, 3D-CT/bronchoscopy will help evaluating the extent of the lesion, degree of airway narrowing, and treatment response.

Management of Chronic Unilateral Hematuria by Ureterorenoscopy

Background and Purpose: Chronic unilateral hematuria is characterized by intermittent or continuous gross hematuria that cannot be diagnosed using standard radiology and hematology methods. In the past, it was managed with partial or total nephrectomy. In the age of minimally invasive procedures, however, endoscopy has enabled more accurate diagnosis and management. We analyzed our experience with transurethral ureterorenoscopy using a flexible ureteroscope to determine the feasibility and success of endoscopic management of renal hematuria. Patients and Methods: We reviewed the records of 13 patients who presented with chronic unilateral hematuria, in whom radiologic and laboratory tests failed to reveal the source of bleeding. In the cases in which the lesion was identified, after complete inspection of the collecting systems, the bleeding site was treated ureteroscopically with a holmium: yttrium-aluminum-garnet ( YAG) laser. Results: Follow-up ranged from 4 to 60 months ( mean 26 mos). During the follow-up of the 13 patients, 11 remained symptom-free, with only one session of flexible ureterorenoscopy necessary. Relapse occurred in two patients after 4 months and 6 months, respectively; during a second session of flexible ureteroscopy, the bleeding site was successfully identified and cauterized with a holmium: YAG laser. No surgical complications occurred. Conclusions: Conservative treatment of patients with chronic unilateral hematuria should always be considered. Laser ureteroscopic treatment is an excellent method and should be considered as the first option for the management of chronic unilateral hematuria.

Peritumoral brain edema in benign meningiomas: correlation with clinical, radiologic, and surgical factors and possible role on recurrence

Background: Approximately 60% of meningiomas are associated with peritumoral edema. Various causative factors have been discussed in the literature. The objective of this study was to investigate the correlation of PTBE with clinical, radiologic, and surgical aspects and recurrence of meningiomas. Methods: Sixty-one patients with benign meningiomas were chosen for surgical treatment by the Group of Brain Tumors and Metastasis of the Department of Neurosurgery. All patients underwent complete surgical resection (Simpson grades I and 2), and those with atypical and malignant histopathologic grades were excluded. Tumors located in the cavernous sinus, tuberculum sellae, foramen magnum, ventricles, and petroclival region were excluded. Results: Edema extension had a positive correlation with the higher recurrence rates (P=.042) and with the presence of irregular margins (P<.011) on bivariate analysis. Meningiomas with larger edema sizes also showed correlation with large meningiomas (P=.035), and the ones with smaller edema sizes correlated with the tentorial location (P=.032). Multivariate analysis showed an association between PTBE and the presence of seizures (odds ratio, 3.469), large meningiomas (odds ratio, 15.977), and for each cubic centimeter added to its size, the risk of edema increased 1.082 times (odds ratio). Conclusion: Peritumoral brain edema may be related to the invading potential of meningiomas and may play a role in the recurrence potential of the tumor. As a consequence, it is reasonable to consider the presence of edema as an additional factor to be taken into account when mapping out strategies for the treatment of meningiomas. (C) 2008 Elsevier Inc. All rights reserved.

The implications of microsurgical anatomy for surgical approaches to the sellar region

The knowledge of the normal anatomy and variations regarding the management of tumors of the sellar region is paramount to perform safe surgical procedures. The sellar region is located in the center of the middle cranial fossa; it contains complex anatomical structures, and is the site of various pathological processes: tumor, vascular, developmental, and neuroendocrine. We review the microsurgical anatomy (microscopic and endoscopic) of this region and discuss the surgical nuances regarding this topic, based on anatomical concepts.

Erection induced by Tx2-6 toxin of Phoneutria nigriventer spider: Expression profile of genes in the nitric oxide pathway of penile tissue of mice

The peptides Tx2-5 and Tx2-6, isolated from the whole venom of ""armed-spider"" Phoneutria nigniventer venom, are directly linked with the induction of persistent and painful erection in the penis of mammals. The erection induced by Tx2-6 has been associated with the activation of nitric oxide synthases. There is a scarcity of studies focusing on the outcome of Tx2-6 at the molecular level, by this reason we evaluated the gene profile activity of this toxin at the nitric oxide (NO) pathway. After microarray analyses on cavernous tissue of mice inoculated with Tx2-6 we found that only 10.4% (10/96) of these genes were differentially expressed, showing a limited effect of the toxin on the NO pathway. We found the genes sparc, ednrb, junb, cdkn1a, bcl2, ccl5, abcc1 over-expressed and the genes sod1, s100a10 and fth1 under-expressed after inoculation of Tx2-6. The differential expressions of sparc and ednrb genes were further confirmed using real-time PCR. Interestingly, ednrb activates the L-arginine/NO/cGMP pathway that is involved in the relaxation of the cavernous body. Therefore the priapism induced by Tx2-6 is a consequence of a highly specific interference of this neurotoxin with the NO pathway. (C) 2009 Elsevier Ltd. All rights reserved.

Preliminary findings from a new animal model for Peyronie`s disease involving extracorporeal shock waves

To develop an experimental model in rabbits to analyse the efficiency of extracorporeal shock wave therapy (ESWT) for Peyronie`s disease. We used 15 adult male rabbits divided into three equal groups. In group 1 (no penile ESWT) rabbits had three sessions of ESWT with 2000 shocks each (15 kV), but a rubber mat was placed between the shock head and rabbit to protect the penis; the rabbits were killed at 7 days after the last session of ESWT. In group 2 the rabbits had three sessions of ESWT using the same parameters, and were killed immediately after the last session to analyse the penis. In group 3 the rabbits had three sessions of ESWT as before but were killed at 7 days after the last session, and the penile tissue analysed macroscopically and histologically. The results showed clearly that the model was efficient, creating a similar situation to that when applying ESWT in the human penis. All of the rabbits in groups 2 and 3 had haematomas and diffuse petechiae after ESWT, and only four had urethral and penile bleeding. Almost all macroscopic changes disappeared after 48 h and only one rabbit in group 3 after 7 days had a haematoma on the dorsal penile surface. The histology (assessed using haematoxylin and eosin staining) of the cavernous body of the penis showed: unchanged histology in group 1; in group 2 there was a dilated and congested vascular space in the cavernous body, with interstitial extensive bleeding in the dermis; and in group 3 there was an increase in interstitial fibrous tissue in the cavernous septum, with deposition of collagen fibres and thickening of the tunica albuginea. The present model was efficient in producing tissue injury in the normal penis when treated with ESWT, suggesting that this promising model should be considered for use future studies of Peyronie`s disease.

The development of a rat model of erectile dysfunction after radical prostatectomy: preliminary findings

To develop a rat model of erectile dysfunction (ED) after cavernous nerve injury. Given the great similarity between the anatomical structure of the cavernous nerve in rats and humans, 24 rats underwent dissections and the cavernous nerves were identified with the aid of an operating microscope. Then the rats were randomized into two groups: sham-operated controls and a bilateral cavernous nerve section group. At 3 months after surgery, the rats were evaluated for their response to an apomorphine challenge. The erectile response after an apomorphine challenge was normal in all the control rats, while there were no erections in the bilateral injured group. The rat major autonomic ganglion and its cavernous nerve can be identified with the aid of a microscope. Rats are inexpensive and easy to handle, thus a good animal for developing an ED model of cavernous nerve injury. In the present study, the rats with cavernous nerve injury lost erectile capacity in a reliable and reproducible fashion. Because of the great similarity between the cavernous nerve of rats and humans, one may consider this technique as a reliable experimental model for studying ED after radical prostatectomy.

Penile erection induced in vivo by a purified toxin from the Brazilian spider Phoneutria nigriventer

OBJECTIVES To verify the effect on erectile tissue of mice of two neuropeptides extracted from the poison of a spider, Phoneutric nigriventer, (Tx2-5 and -6. termed `eretina`) after direct injection into the corpus cavernosum, to assess the minimum dosage necessary for effect. the time for initiation of action, the local duration of the erection, histological effects and the presence of local and systemic side-effects. MATERIALS AND METHODS When applied intraperitoneally, eretina promotes the relaxation of cavernous smooth muscle, thus causing penile erection. Thirty-five mice were divided in two groups; 10 control mice were injected 20 mu L of saline solution, and in the treated group, 25 mice were divided into groups of five and each subgroup received eretina in decreasing doses (0.024, 0.012, 0.006, 0.003 and 0.0015 mu g/kg) until the minimum dose that produced an erection was determined. After treatment 211 mice were monitored to determine the response and any collateral effects. RESULTS The minimum dose producing an erection was 0.006 mu g/kg, the five mice in this group having evidence of an erection at 35-45 min after injection. The histology of the cavernosum of mice treated with eretina showed dilatation and congestion of the vascular spaces with more blood than in controls. With the minimum dose there were no local or systemic collateral effects and the erection was lost after 120-140 min. CONCLUSION The minimum dose of eretina producing an erection in mice was determined. and the agent was safe for this use as it did not produce any collateral toxic effects. These studies indicate a possible means of determining the mechanism of action of eretina.

Cystic hygroma: characterization by computerized tomography

Lymphangiomas are benign nonencapsulated lesions composed of sequestered noncommunicating lymphoid tissue lined by lymphatic endothelium and are thought to be caused by congenital obstruction of lymphatic drainage. They are subclassified by vessel size, such as the capillary, which is rare and located in subcutaneous tissue, cavernous (located about the mouth and tongue), and cystic (cystic hygromas). The cystic hygromas show a predilection for the neck (75%) and maxilla (20%), and the remaining 5% arise in rare locations such as the mediastinum, retroperitoneum, bone, kidney, colon, liver, spleen and scrotum. Only 3%-10% of neck lesions extend into the mediastinum. In this paper, we report a rare case of cystic hygroma with a huge dimension discussing the use of computed tomography scanning for diagnosis.

“Papel dos recetores purinérgicos no crescimento e temodelação do tecido cavernoso”

A adenosina é um nucleósido ubíquo envolvido na regulação de controlo do tónus vascular do tecido cavernoso, desempenhando um papel importante na fisiopatologia da Disfunção Erétil (DE) resistente aos fármacos relaxantes musculares clássicos. Apesar da importância comprovada dos recetores da adenosina na fisiopatologia da DE no homem, pouca informação é conhecida no que diz respeito à expressão e localização dos recetores purinérgicos no Tecido Cavernoso de Ratazana (TCR). Neste trabalho avaliou-se o fenótipo dos recetores purinérgicos responsáveis pela regulação do tónus do tecido erétil de ratazana por imunofluorescência indireta aplicada à microscopia confocal em co-culturas de células endoteliais e musculares lisas do TCR. Para além da caracterização imunofenotípica, desenvolveu-se uma técnica que permite diferenciar funcionalmente em tempo real (por microscopia confocal funcional) células musculares lisas e células endoteliais isoladas de TCR em co-cultura marcadas com a sonda fluorescente Fluo-4NW. Esta técnica permite distinguir cada um dos subtipos celulares mediante o padrão e a magnitude das oscilações dos níveis intracelulares de Ca2+ ([Ca2+]i) em resposta ao ATP (agonista P2) e à fenilefrina (PE, agonista α-adrenérgico). Nas células musculares lisas, observou-se uma resposta mais acentuada ao agonista α-adrenérgico, PE, e uma resposta menos significativa ao ATP. O contrário foi observado relativamente às células endoteliais. A incubação das células musculares lisas e endoteliais com ATP (300 μM) causou um aumento dos níveis de [Ca2+]i. O efeito do ATP (300 μM) parece envolver a ativação de recetores dos subtipos P2X1 e P2X3 sensíveis ao bloqueio com NF023 (3μM) e A317491 (100 nM), respetivamente. Já o aumento dos níveis [Ca2+]i produzido pelo ADP (300 μM) parece envolver a ativação de recetores P2Y1, P2Y12 e P2Y13 mediante o antagonismo produzido pelos antagonistas MRS 2179 (0,3μM), AR-C66096 (0,1 μM) e MRS 2211 (10μM), respetivamente. Os dois tipos celulares expressam imunorreatividade contra recetores A2A, A2B, P2X1, P2X3, P2Y1, P2Y12 e P2Y13.

Miosite Orbitária

Orbital myositis is a subgroup of the nonspecific inflammatory syndrome or orbital pseudotumor and is characterized by a primary inflammation of extraocular muscles. The authors describe a 70-year-old patient with acute proptosis, ocular pain and right ophthalmoplegia, whose orbital computed tomographic scan showed enlargement of the homolateral extraocular muscles. Clinical presentation and complementary tests were compatible with the diagnosis of orbital myositis however, because of the particular aspects, which included retinal central vein occlusion, optic nerve lesion, distension of the superior ophthalmic vein and the homolateral cavernous sinus, the differential diagnosis with cavernous sinus pathology and thyroid ophthalmopathy was considered. The importance of a rapid diagnosis and treatment is stressed.

Fístulas Carótido-Cavernosas. Perspectivas de Diagnóstico e Terapêutica

Even though in clinical practice carotid cavernous fistulas (CCF) are not a frequent pathology, it should be a diagnostic hypothesis in face of a suggestive clinical presentation. We intended to review the diagnosis and the therapeutics, comparing them with the actual perspectives. Files of 25 patients with the diagnostic hypothesis of CCF, confirmed by conventional angiography in the HSAC Neuroradiology Department, were reviewed. In this group of patients, (5 males and 20 females), the fistulous aetiology was spontaneous in 10 and traumatic in 15. Clinically the symptom most often presented was diplopia (23 cases) and the most frequent encountered sign was ophthalmoplegia (20 cases). Of the Neuroradiologic investigation, CT scan (done to all patients) have shown an prominent superior ophthalmic vein as the most frequent abnormality. Angiographic study was based on Lasjaunias et al protocol (Surgical Neuroangiography, Vol. 2, Springer-Verlag). Surgery was the therapeutic approach for the traumatic fistulas; only 2 of the spontaneous were treated by endovascular route. Three patients are still under observation. In all the others cases there was a fistula exclusion.

Nefrectomia Radical e Nefroureterectomia Laparoscópica "Hand-Assited": a Experiência dos Primeiros 21 Casos

Objectivos: Os autores apresentam a sua casuística com os primeiros 21 casos submetidos a nefrectomia radical e nefroureterectomia laparoscópica "hand- assisted" e laparoscópica pura. Material e Métodos: Entre o período de Janeiro 2003 a Junho de 2004 procedeu-se à realização de nefrectomia radical e nefroureterectomia laparoscópica em 21 doentes com o diagnóstico clínico de carcinoma de células renais e de carcinoma de células de transição do trato urinário alto. Optámos pela assistência da mão apenas quando útil ou necessário. Realizamos 16 nefrectomias radicais laparoscopicas "hand-assisted", 2 nefroureterectomias radicais laparoscopicas "hand- assisted" com desincer- ção endoscópica do meato ureteral e 3 nefrectomias radicais laparoscopicas "puras". A idade média deste grupo foi de 62 anos.O diâmetro médio da massa renal foi de 4,8 cm. O diagnóstico anatomo-patológico revelou a existência de 17 carcinomas de células renais, 2 carcinomas de células de transição, 1 hemangioma e 1 quisto renal complicado. Tivemos uma taxa de conversão de 5%, e a duração da cirurgia foi de 1,46 horas. O follow- up destes doentes variou entre 1 e 2 anos, não se tendo verificado quaisquer recidivas. Nesta série houve um re-internamento. Conclusão: a nefrectomia radical laparoscópica "hand-assisted" e a nefroureterectomia laparoscópica "hand-assisted" são exequíveis sendo a curva de aprendizagem relativamente curta. A taxa de complicações é baixa.

Intraorbital Abducens Nerve Schwannoma

BACKGROUND: Schwannomas of the abducens nerve are extremely rare tumors. The tumor may be located within the cavernous sinus or more often at the prepontine region. However, literature research has identified only one case of isolated schwannoma of the orbit, arising from the terminal branches of the abducens nerve to the lateral rectus muscle. This is only the second report of an abducens nerve schwannoma located entirely intraconal. CASE DESCRIPTION: We report a case of an intraorbital abducens nerve schwannoma in a 42-year-old man with no signs of neurofibromatosis. The lesion resulted in progressive diplopia and focal abducens palsy. The clinical, radiologic, and pathologic features are presented. RESULTS: We point the particular aspects and discuss the possible treatments and approaches to preserve nerve function. CONCLUSIONS: Being a benign lesion, one of the goals has always been total removal. The knowledge of the correct anatomic features made us believe that the VI nerve function could be preserved. Our case is the first example of a total removal with eye abduction preserved. Because of that, we believe that it is reasonable to aim for these goals in future cases.