Complications after bladder augmentation in children

PURPOSE : Bladder augmentation is an effective surgical procedure for increasing bladder capacity and reducing pressure on the urinary system. It is indicated for patients with anomalies such as spina bifida, myelomeningocele, urethral valve and bladder exstrophy, who progress with low tolerance of medication. CASES : This was a retrospective study conducted on pediatric patients submitted to bladder augmentation from 2000 to 2011. RESULTS : 34 patients aged 4 to 17 years were submitted to bladder augmentation, 30 of them with an ileal loop and 4 with a ureter.A continent urinary shunt was performed in 16 patients, the Mitrofanoff conduit was associated in 15, and the Macedo technique was used in one. Mean follow-up was 34.35 months (1 to 122 months). Mean creatinine was 1.5 ng/ml (0.4 to 7.5 ng/ml) preoperatively and 1.78 ng/ml postoperatively. Three patients required a renal transplant during follow-up. There was improvement or resolution of vesicoureteral reflux in 83.5% of the kidneys on the right and in 75% on the left. Bladder capacity increased, on average, from 152.5 ml to 410 ml. The main complications were vesical lithiasis in 3 patients and conduit perforation in one. CONCLUSION : Bladder augmentation showed good results in this series, preserving renal function in most of the patients.(AU)

Espinha bífida oculta em um sagui-de-tufos-pretos (Callithrix penicillata) / Spina bifida occulta in a black-tufted-ear marmoset (Callithrix penicillata)

Background: The black-tufted ear marmoset, Callithrix penicillata, is an endemic primate from Brazil, distributed among Cerrado and Atlantic Forest vegetation. Most of the congenital malformations from the spinal cord arises from failures in the neural tube closure. Those failures result in an opened dorsal arch of one or more vertebrae are referred as spina bifida. In the occulta form, the condition is asymptomatic because spinal cord and meninges remain in the normal position. When the meninges become distended with fluid (myelomeningocele), the phenomenon is called spina bifida cystica. In these cases, the spinal cord protrudes into the subarachnoid space and the nerve roots may become displaced, causing neurological defi cits. When this defect allows leakage of cerebrospinal fluid through the skin, it is characterized as the open form. This article reports the findings of the first case of spina bifida occulta in an adult male of Callithrix penicillata collected dead in a brazilian highway. Case: A cadaver of a young-adult, male, black-tufted ear marmoset (Callithrix penicillata) was founded at km 103 of BR-116 highway in the city of Guapimirim, Rio de Janeiro State, Brazil. The cadaver was necropsied at the Laboratory of Animal Anatomy from Universidade Castelo Branco, in the city of Rio de Janeiro, Brazil. During macroscopic evaluation it was possible to identify a normal body score, healthy skin, and the presence of multiple fractures in the skull. Encephalic injury was indicated as the cause of his death. After the necropsy, the body was subjected to maceration technique to prepare his skeleton to the museum of the laboratory. Once soft tissues were removed, the skeleton was placed in the plastic box that contained a colony of necrophagous beetles (Dermestes maculatus), which completed the process of cleaning the bones. At this time, a failure to close the vertebral arches of T2, T3 and T4 was observed which characterized a case of spina bifida oculta. Discussion: As long as spina bifida is one of the most common malformations in humans, it has also been reported in several species of animals. Besides being one of the most common malformations in humans, spina bifida has been reported in several species of animals. However, there is only one report of spina bifida (cystic) of spontaneous occurrence in primates (in a female of Macaca niger with neurological defi cits). According to current literature, this is the first report of spina bifida in Callithrix penicillata, as well as in a wild animal in Brazil. Spina bifida affects most commonly lumbar or sacral vertebrae. In this case, although, malformation occurred at the level of thoracic vertebrae T2, T3 and T4, an uncommon location. The fact that the cause of death of the marmosets have been a traumatic brain injury, coupled with the appearance of changes of the vertebral arches, absence of deformation in the skin and the apparent good body condition, allowed us to deduce that this was a case of spina bifida occulta. It would be unlikely that a wild animal with myelomeningocele could survive until adulthood. The possibility of a coexisting meningocele could be compatible with the survival, despite some degree of motor impairment would make it proned to be hit by cars, falls or predation. It was not possible to determine the cause of malformation in this case, but one can imagine that some factors deficiencies (eg.: folic acid or zinc), genetic factors and effects of teratogenic substances from the environment would be some of the speculated causes for the appearance of spina bifida in this marmoset. Information about congenital malformations from other animals in the same geographic region could eventually reinforce this hypothesis.

Anesthetic management in intrauterine surgery to evaluate an experimental model of myelomeningocele in non human primates (Macaca mulatta) / Anestesia em cirurgia intra-uterina para avaliar um modelo experimental de mielomeningocele em primatas não humanos (Macaca mulatta)

PURPOSE: Evaluate the anesthetic management in intrauterine surgery to induce myelomeningocele in non human primates Macaca mulatta. METHODS: A total of nine fetuses had intrauterine surgery; laminectomy was performed on them in L5 and L6. The studied variables were: maternal death, fetus death, cardiac frequency, respiratory frequency, arterial pressure, temperature, and oxygen saturation. RESULTS: No maternal or fetal deaths occurred; the only variable that was reported below the normal ranges was temperature. CONCLUSION: No maternal or fetal deaths occurred; the only variable that was reported below the normal ranges was temperature.(AU)

OBJETIVO: Avaliar o manejo anestésico em cirurgia intra-uterina para induzir mielomeningocelo em primatas não humanos, Macaca mulatta. MÉTODOS: Operaram-se um total de nove fetos in útero que foram submetidos à laminectomia em L5 e L6. As variáveis a estudar foram mortes maternas ou fetais, freqüência cardíaca e respiratória, pressão arterial, temperatura e saturação de oxigênio. RESULTADOS: Não se apresentaram mortes maternas ou fetais, a temperatura se manteve abaixo dos 36ºC, não tendo repercussões no bem-estar dos macacos. CONCLUSÃO: Não ocorreu nenhum óbito materno ou fetal, sendo que a única variável abaixo do normal foi a temperatura.(AU)

Simplified correction of a meningomyelocele-like defect in the ovine fetus

PURPOSE: To develop a simplified technique for antenatal correction of a meningomyelocele -like defect in fetal sheep to allow direct skin closure. METHODS: A spinal defect was surgically created at 75 days of gestation in the fetuses of 36 pregnant sheep, 23 survived the surgery. At 102 days gestation, the defect was corrected in 14 cases (9 were left untreated). Skin surrounding the defect was dissected below the dermis to permit its edges to be approximated and sutured, without interposing any material to its edges. An interface material intended to protect the neural tissue from skin adhesion was used and the skin defect was completely closed over it. Pregnancy was allowed to continue up to 138 days gestation, the fetuses were submitted to macroscopic and microscopic analysis. RESULTS: The defect was successfully corrected in 90.9% in the experimental group, and spontaneous closure occurred in 22.3% in the control group (p 0.05). The survival rate after the creation and correction of the defect was 63.4% and 78% respectively. CONCLUSION: This simplified technique was successful in the correction a meningomyelocele-like defect, in the fetal sheep.

OBJETIVO: Desenvolver uma técnica simplificada de correção pré-natal de defeito semelhante à mielomeningocele em fetos de ovelha permitindo um fechamento direto da pele. MÉTODOS: Um defeito espinhal foi cirurgicamente criado com 75 dias de gestação, em 36 fetos de ovelha, 23 sobreviveram à cirurgia. Após 102 dias de gestação, o defeito foi corrigido em 14 casos (9 não foram tratados). A pele em volta do defeito foi dissecada abaixo da derme para permitir a aproximação direta das bordas através de sutura, sem a interposição de nenhum material entre a pele. Um material de interface foi colocado entre o tecido neural exposto e a pele, com o objetivo de evitar a adesão da medula à pele, que foi completamente fechada sobre o defeito. A gravidez foi mantida até 138 dias, os fetos foram submetidos a análises macroscópicas e microscópicas. RESULTADOS: O defeito foi corrigido em 90.9% no grupo experimental, e o fechamento espontâneo ocorreu em 22.3% no grupo controle (p 0.05). A taxa de sobrevivência após a criação do defeito e posteriormente a sua correção foi de 63,4% e 78%, respectivamente. CONCLUSÃO: Esta técnica simplificada teve sucesso na correção do defeito semelhante à mielomeningocele em feto de ovelha.

Simplified correction of a meningomyelocele-like defect in the ovine fetus / Correção simplificada de um defeito semelhante à meningomielocele em fetos de ovelha

PURPOSE: To develop a simplified technique for antenatal correction of a meningomyelocele -like defect in fetal sheep to allow direct skin closure. METHODS: A spinal defect was surgically created at 75 days of gestation in the fetuses of 36 pregnant sheep, 23 survived the surgery. At 102 days gestation, the defect was corrected in 14 cases (9 were left untreated). Skin surrounding the defect was dissected below the dermis to permit its edges to be approximated and sutured, without interposing any material to its edges. An interface material intended to protect the neural tissue from skin adhesion was used and the skin defect was completely closed over it. Pregnancy was allowed to continue up to 138 days gestation, the fetuses were submitted to macroscopic and microscopic analysis. RESULTS: The defect was successfully corrected in 90.9 percent in the experimental group, and spontaneous closure occurred in 22.3 percent in the control group (p < 0.05). The survival rate after the creation and correction of the defect was 63.4 percent and 78 percent respectively. CONCLUSION: This simplified technique was successful in the correction a meningomyelocele-like defect, in the fetal sheep.(AU)

OBJETIVO: Desenvolver uma técnica simplificada de correção pré-natal de defeito semelhante à mielomeningocele em fetos de ovelha permitindo um fechamento direto da pele. MÉTODOS: Um defeito espinhal foi cirurgicamente criado com 75 dias de gestação, em 36 fetos de ovelha, 23 sobreviveram à cirurgia. Após 102 dias de gestação, o defeito foi corrigido em 14 casos (9 não foram tratados). A pele em volta do defeito foi dissecada abaixo da derme para permitir a aproximação direta das bordas através de sutura, sem a interposição de nenhum material entre a pele. Um material de interface foi colocado entre o tecido neural exposto e a pele, com o objetivo de evitar a adesão da medula à pele, que foi completamente fechada sobre o defeito. A gravidez foi mantida até 138 dias, os fetos foram submetidos a análises macroscópicas e microscópicas. RESULTADOS: O defeito foi corrigido em 90.9 por cento no grupo experimental, e o fechamento espontâneo ocorreu em 22.3 por cento no grupo controle (p < 0.05). A taxa de sobrevivência após a criação do defeito e posteriormente a sua correção foi de 63,4 por cento e 78 por cento, respectivamente. CONCLUSÃO: Esta técnica simplificada teve sucesso na correção do defeito semelhante à mielomeningocele em feto de ovelha.(AU)

Validation of the ovine fetus as an experimental model for the human myelomeningocele defect / Validação do feto de ovino como modelo experimental de meningomielocele

PURPOSE: To produce a myelomeningocele-like human defect in the ovine fetus and validate this experimental model in our population. METHODS: A prospective study on 12 pregnant sheep of a crossed Hampshire/Down breed where a spinal defect was surgically created between Day 75 and Day 77 after conception. The technique consisted of a hysterotomy with exposure of fetal hind limbs and tail up to the mid spine. Fetal skin, paravertebral muscles, and 4 posterior spinal arches were excised, exposing the spinal cord. Duramater was opened and the medulla was incised until the medullar canal. Animals were euthanized at 139 days of gestation for fetal evaluation. The central nervous system was submitted to post-mortem magnetic resonance imaging (MRI) and the spine was submitted to pathological examination. RESULTS: The defect was created in 13 fetuses and 5 survived. Mean gestational age at necropsy was 121.6 days (varying from 93 to 145 days). Macroscopically, the defect was present in 4 cases. Microscopy revealed a flattened medulla with disappearance of the medullar canal and disruption of normal medullar architecture with neuronal apoptosis and/or fusion of the piamater and duramater. The MRI showed herniation of the cerebellum into the cervical canal and syringomyelia. CONCLUSIONS: The surgically produced defect mimics the defect found in the human fetus, including the Arnold-Chiari malformation. Post-mortem MRI was used for the first time in our study and proved an excellent alternative for demonstrating the cerebellar herniation. We standardized the technique for creating the defect in our population.(AU)

OBJETIVO: Produzir um defeito semelhante a meningomielocele humana em feto de ovinos, validando este modelo experimental, em nosso meio. MÉTODOS: Estudo prospectivo com 12 ovelhas de cruzamento das raças Hampshire e Down, onde um defeito na coluna foi criado cirurgicamente com 75 a 77 dias de gestação. A técnica consistiu em histerotomia com exposição das patas traseiras e cauda até a metade da coluna fetal. Foram retirados pele, musculatura paravertebral e 4 arcos vertebrais posteriores, expondo a medula. A dura foi aberta e uma incisão até o canal medular foi realizada. O sacrifício foi realizado com 139 dias de gestação para avaliação fetal. O sistema nervoso central foi submetido a ressonância magnética (REMA) post-mortem e a coluna foi submetida a exame anátomo-patológico. RESULTADOS: O defeito foi criado em 13 fetos e 5 sobreviveram. A idade gestacional média da necropsia foi de 121,6 dias (variando de 93 a 145 dias). Macroscopicamente o defeito estava presente em 4 casos. A microscopia revelou achatamento da medula, com desaparecimento do canal medular e desarranjo da citoarquitetura medular com apoptose neuronal e/ou fusão da pia e dura-máter. A REMA demonstrou herniação do cerebelo para o canal cervical e siringomelia. CONCLUSÕES: O defeito produzido foi muito semelhante à meningomielocele em fetos humanos, levando inclusive à mal-formação de Arnold-Chiari. A REMA post-mortem, utilizada de forma inédita neste estudo, demonstrou ser uma excelente alternativa para demonstrar a presença da herniação do cerebelo. A técnica para produzir o defeito foi padronizada em nosso meio.(AU)

Biosynthetic cellulose induces the formation of a neoduramater following pre-natal correction of meningomyelocele in fetal sheep / A celulose biossintética induz a formação de uma neoduramáter na correção antenatal da meningomielocele em fetos de ovelhas

PURPOSE: The aim of this study was to compare the effectiveness of two dura-mater substitutes, namely human acellular dermal matrix (HADM) and biosynthetic cellulose (BC), in repairing, in utero, surgically-induced meningomyelocele (MMC) in fetal sheep. METHODS: A neural tube defect was created at 74-77 days gestation in 36 fetal sheep. They were divided into 3 groups, the control group that did not receive pre-natal corrective surgery, and the other two groups that received corrective surgery using HADM (Group A) or BC (Group B). Both materials were used as a dura-mater substitutes between the neural tissue and the sutured skin. Correction was performed at gestation day 100 and the fetuses were maintained in utero until term. Sheep were sacrificed on gestation day 140. The fetal spine was submitted to macro and microscopic analysis. At microscopy, adherence of the material to the skin and neural tissue was analyzed. RESULTS: In the initial phase (pilot), experimentally-induced MMC was performed on 11 fetuses and 4 survived (37 percent). In the second phase (study), 25 fetuses received surgery and 17 survived (68 percent). In the study group, 6 fetuses did not undergo repair (control group), 11 cases were submitted to corrective surgery (experimental group) and one fetal loss occurred. Of the surviving cases in the experimental group, 4 constituted Group A and 6 in Group B. Macroscopically, skin and underlying tissues where easily displaced from the BC in all cases it was used; in contrast, HADM adhered to these tissues. To compare the adherence, 4 cases from Group A and 4 in Group B were studied. We observed adherence, host cell migration and vessel proliferation into the HADM all sections from Group A and this aspect was not present in any cases in Group B (p < 0.05). In Group B, we also observed that a new fibroblast layer formed around the BC thus protecting the medulla and constituting a "neoduramater". CONCLUSION: The use of BC...(AU)

OBJETIVO: Estudar os efeitos do emprego de dois materiais consideravelmente diferentes quanto à origem e custo na correção intra-uterina da meningomielocele criada experimentalmente em feto de ovino. MÉTODOS: Em 36 fetos de ovinos foi criado um defeito aberto de tubo neural, com 75 de dias de gestação. Os casos foram divididos em três grupos: o controle onde o defeito não foi corrigido, grupo corrigido A onde o material utilizado para cobrir a medula exposta foi a matriz dérmica humana acelular (MDHA) e o grupo corrigido B onde o material foi a celulose biossintética (CB). Após a correção realizada com 100 dias, os fetos eram mantidos intra-útero até o termo da gestação. Os sacrifícios foram realizados com 140 dias e a coluna fetal era submetida à análise macro e microscópica onde foi observada a aderência dos materiais à pele, medula ou tecido nervoso remanescente. RESULTADOS: Na fase inicial (piloto), 11 fetos foram operados e 4 sobreviveram (37 por cento). Na segunda fase (estudo) 25 fetos foram operados e 17 sobreviveram (68 por cento). No grupo de estudo, 6 fetos não foram submetidos à correção (grupo controle), 11 casos foram corrigidos e ocorreu 1 perda fetal. Do total de 10 casos, 4 constituíram o grupo A e 6, o grupo B. A macroscopia observou-se deslizamento da pele e tecidos subjacentes sobre a CB em todos os casos onde ela foi empregada e isto não ocorreu em nenhum dos casos onde a MDHA foi utilizada. Para comparar a aderência, foram considerados 4 casos do grupo A e 4 do grupo B. A aderência, caracterizada pela migração de células do hospedeiro e proliferação de vasos para dentro da MDHA, foi observada em 100 por cento dos casos do grupo A e em nenhum caso no grupo B (p < 0,05). No grupo B observou-se formação de uma camada de fibroblastos ao redor do material, protegendo a medula, caracterizando a formação de uma "neoduramater". CONCLUSÃO: A utilização da película de celulose biossintética parece ser mais adequada como substituto...(AU)

Teratogenic effect of retinoic acid in swiss mice / Efeito teratogênico do ácido retinóico em camundongo swiss

PURPOSE: To identify the types of malformations resulting from the administration of retinoic acid (RA) to Swiss mice on different days of pregnancy. METHODS: Twenty-four pregnant Swiss mice were divided into 4 groups of 6 animals each. The experimental groups received a single intraperitoneal injection of RA (70 mg/kg) on gestational days 7, 8 and 9 (D7, D8 and D9), while control animals (C) received only saline solution. RESULTS: Were obtained: exencephaly (C:0; D7:16.1 percent; D8:25.4 percent; D9:0), myelomeningocele (C:0; D7:25.8 percent, D8:30.9 percent, D9:0), spina bifida occulta (C:0, D7:29 percent, D8:41.8 percent, D90), gastroschisis (C:0, D7:6.4 percent D8:5.4 percent, D9:0), omphalocele (C:0, D7:6.4 percent, D8:14.5 percent, D9:0), lower limb alterations (C:0, D7:74.1 percent, D8:80 percent, D9:0), imperforated anus (C:0, D7:100 percent, D8:100 percent, D9:100 percent), and tail agenesis/alteration (C: D7:100 percent, D8:100 percent, D9:100 percent). CONCLUSION: The experimental model using Swiss mice proved to be efficient in the induction of the different types of defects, with the eighth gestational day being the one that most favored the induction of neural tube defect, omphalocele, gastroschisis, lower limb defects, imperforated anus and tail agenesis/alteration. On this basis, this is a useful model for future investigation of neural development and of the formation of the appendicular skeleton(AU)

OBJETIVO: Identificar os tipos de malformação resultantes da administração do ácido retinóico (AR) a camundongos Swiss em diferentes dias gestacionais. MÉTODOS: Foram utilizados 24 camundongos fêmeas, linhagem Swiss, prenhes, divididos em 4 grupos com 6 animais cada. Os grupos experimentais receberam uma única injeção intraperitoneal de AR (70mg/Kg) nos dias gestacionais 7, 8 e 9 (D7, D8 e D9), enquanto que os animais do grupo controle (C) receberam apenas solução salina. RESULTADOS: Foram encontrados: exencefalia (C:0; D7:16.1 por cento; D8:25.4 por cento; D9:0); mielomeningocele (C:0; D7:25.8 por cento; D8:30.9 por cento; D9:0); Espina Bífida Oculta (C:0; D7:29 por cento; D8:41.8 por cento; D90); gastrosquise (C:0; D7:6.4 por cento D8:5.4 por cento; D9:0); onfalocele (C:0; D7:6.4 por cento; D8:14.5 por cento; D9:0); alterações do membro inferior (C:0; D7:74.1 por cento; D8:80 por cento; D9:0); imperfuração anal (C:0; D7:100 por cento; D8:100 por cento; D9:100 por cento) e agenesia/alteração de cauda (C: D7:100 por cento; D8:100 por cento; D9:100 por cento). CONCLUSÕES: O modelo experimental utilizando camundongo Swiss mostrou-se eficiente na indução dos diferentes tipos de defeitos, sendo o oitavo dia gestacional o mais propicio na indução de DFTN, onfalocele, gastrosquise, defeitos de membro inferior, imperfuração anal e agenesia/alteração de cauda, tornando este um modelo útil para futuras investigações do desenvolvimento neural e no processo de formação do esqueleto apendicular(AU)

Estudo de uma nova técnica para criação cirúrgica de um defeito semelhante a meningomielocele em fetos de coelhos

PURPOSE: To evaluate fetal survival rate using a modified technique to surgically create a "myelomeningocele-like" defect in the rabbit model. METHODS: Six white New Zealand rabbits had a spinal defect created in their fetuses at 23 days of gestation. The defect was created in 19 fetuses and an attempt to correct it was made in 15 cases (group I), 4 fetuses where left without correction (group II)At 30 days of gestation, fetuses were harvested for anathomo-pathologic evaluation. RESULTS: At 30 days, a "myelomeningocele-like" defect was present in all surviving fetuses. The total survival rate was 73,68% (14/19), 11 fetus survived in group I and 3 in group II. CONCLUSION: The technique modifications including fetal positioning and exposure of its back prior to the incision of the lamina associated to a different technique for miometrium closure offers an alternative and probably safer method to surgically create a spinal defect in the fetal rabbit.

OBJETIVO: Avaliar a sobrevida fetal usando uma nova técnica cirúrgica para criação de um defeito semelhante a meningomielocele em fetos de coelho. MÉTODOS: Foram utilizadas seis coelhas da linhagem Nova Zelândia que tiveram um defeito espinhal criado em seus fetos no 23° dia de gestação. O defeito foi criado em 19 fetos e a tentativa de correção foi feita em 15 casos (grupo I), 4 fetos permaneceram sem correção (grupo II). No 30° dia de gestação, os fetos foram submetidos a avaliação anátomo-patológica. RESULTADOS: No 30° dia a meningomielocele estava presente em todos os fetos sobreviventes. A sobrevida total foi de 73,68% (14/19), 11 fetos sobreviveram no grupo I, e 3 no grupo II. CONCLUSÃO: A técnica modificada apresenta sobrevida fetal satisfatória, no modelo experimental de criação de defeito espinhal em fetos.