Orientador: Edmur Franco Carelli, Helder Jose Lessa Zambelli / Dissertação (mestrado) - Universidade Estadual de Campinas, Faculdade de Ciencias Medicas / Made available in DSpace on 2018-08-12T10:27:48Z (GMT). No. of bitstreams: 1 DalFabbro_Mateus_M.pdf: 627253 bytes, checksum: e7dc6279817a6c66308fc4d45f8d1a59 (MD5) Previous issue date: 2008 / Resumo: Introdução: A hidrocefalia congênita é uma condição freqüente, estando associada a um terço de todas as malformações congênitas do sistema nervoso. O conhecimento clínico e epidemiológico da hidrocefalia congênita e dos variados fatores etiológicos e prognósticos a ela relacionados são até o momento insuficientes para a compreensão global e otimização do tratamento desta complexa patologia. Objetivos: analisar o prognóstico comparativo de hidrocéfalos com e sem mielomeningocele. Métodos: trata-se de um estudo retrospectivo descritivo, através da revisão dos prontuários de 168 neonatos nascidos no Centro de Assistência Integral à Saúde da Mulher da Universidade Estadual de Campinas (CAISM - UNICAMP), Campinas - SP - Brasil, divididos em duas amostras: grupo 1 (G1), composto de 98 neonatos com hidrocefalia congênita não associada a mielomeningocele; grupo 2 (G2), composto por 70 neonatos com hidrocefalia congênita associada a mielomeningocele. Foram estudadas as comparativamente as seguintes características: idade materna, número de gestações, realização de pré-natal, apresentação fetal, tipo de parto, idade gestacional ao diagnóstico, idade gestacional ao nascimento, índice de Apgar ao 1o e 5o minutos, incidência de baixo peso, graduação ecográfica da hidrocefalia, tratamento cirúrgico de hidrocefalia, complicações dos shunts, duração da primeira internação, mortalidade na primeira internação, mortalidade no seguimento, incidência de retardo neuropsicomotor (RDNPM). Resultados: seguimento médio: G1 42 meses, G2 60 meses; parto cesáreo: G1 69,4%, G2 91,2% (p<0,05); baixo peso: G1 32,7%, G2 10% (p<0,005); Apgar 1o minuto < 8: G1 58,2%, G2 30% (p<0,025); hidrocefalia acentuada: G1 59,2%, G2 28,6% (p<0,005); realização de derivação ventrículo-peritoneal (DVP) na 1a internação: G1 34,7%, G2 71,4% (p<0,005); mortalidade na 1a internação G1 24,4%, G2 10% (p<0,05); internação menor que 5 dias G1 46,9%, G2 12,8% (p<0,005); RDNPM no seguimento G1 70,8%, G2 42,8% (p<0,05). Conclusão: os resultados revelam um prognóstico ruim para a hidrocefalia congênita, caracterizado por alto índice de mortalidade e alta incidência de RDNPM. Os pacientes com hidrocefalia acentuada (grave) pela ecografia apresentam pior prognóstico. A maior incidência de baixo peso, de hidrocefalias acentuadas, de óbitos no período neonatal e de RDNPM no grupo 1 revelam um pior prognóstico dos neonatos com hidrocefalia não associada a mielomeningocele quando comparada aos neonatos com hidrocefalia associada a mielomeningocele. / Abstract: Introduction: Congenital hydrocephalus is a frequent condition, and it is associated with one third of all Central Nervous System (CNS) malformations. The clinical and epidemiological knowledge about congenital hydrocephalus and the related risk and outcome predicting factors are still insufficient for the thorough comprehension of such a complex condition and for the optimization of its treatment. Objectives: to study comparatively the prognosis of hydrocephalic newborns with and without meningomyelocele (MMC). Methods: this is a retrospective study, based on the review of newborn and/or their mother's medical files, comprising 168 patients born at the State University of Campinas, Campinas - SP - Brazil, divided in two groups: group 1 (G1), consisting of 98 hydrocephalic newborns without MMC; group 2 (G2), consisting of 70 hydrocephalic newborns with MMC. These characteristics were comparatively analyzed between the groups: maternal age, number of pregnancies, prenatal diagnosis, fetal presentation, delivery type, gestational age at diagnosis, gestational age at birth, Apgar score at the 1st and 5th minute, low-weight incidence, ecographic severity of hydrocephalus, surgical treatment of hydrocephalus, shunt malfunctions, length of first hospital stay after birth, mortality on 1st hospital stay, mortality on follow-up, incidence of neurodevelopmental delay. Results: mean follow-up: G1 42 months, G2 60 months; cesarean section: G1 69,4%, G2 91,2% (p<0,05); low weight: G1 32,7%, G2 10% (p<0,005); Apgar score at 1st minute < 8: G1 58,2%, G2 30% (p<0,025); severe hydrocephalus: G1 59,2%, G2 28,6% (p<0,005); ventriculoperitoneal (VP) shunt insertion at 1st hospital stay: G1 34,7%, G2 71,4% (p<0,005); mortality at 1st hospital stay: G1 24,4%, G2 10% (p<0,05); length of 1st hospital stay up to 5 days: G1 46,9%, G2 12,8% (p<0,005); neurodevelopmental delay on follow-up: G1 70,8%, G2 42,8% (p<0,05). Conclusion: the results depict a bad outcome related to congenital hydrocephalus, characterized by high mortality and high neurodevelopmental delay rates. Patients harboring severe hydrocephalus on ultrasound present worse outcome. The higher low weight incidence, as well as higher rates of severe hydrocephalus, neonatal deaths and neurodevelopmental impairments on group 1 reveal a worse outcome in newborns with congenital hydrocephalus not related to MMC compared to MMC related hydrocephalic newborns. / Mestrado / Neurologia / Mestre em Ciências Médicas

The purpose of this study was to investigate further the characteristics of hearing loss in ventriculoperitoneal (VP) shunted hydrocephalus. Twelve (VP) shunt treated hydrocephalus children participated in this study. The etiology of the hydrocephalus was either intraventricular hemorrhage or spina bifida. A recent neurological examination reported the shunt to be patent in each child. Audiometric examination included pure tone air conduction thresholds, tympanometry, contralateral and ipsilateral acoustic reflex thresholds and distortion product otoacoustic emissions (DPOAEʹs). A unilateral, high frequency, sensorineural hearing loss was found in the ear ipsilateral to shunt placement in 10 (83%) of the 12 shunt treated hydrocephalic children. No hearing loss was observed the ear contralateral to shunt placement. Based on the pure tone findings coupled with the decrease in DPOAE amplitude in the shunt ear, the hearing loss appears to be cochlear in nature. It is hypothesized that the cochlear hydrodynamics are disrupted as the result of fluid pressure reduction within the perilymph being transmitted via a patent cochlear aqueduct as a reaction to the reduction of CSF via a patent shunt. In addition, a concomitant brainstem involvement is evidenced in the ART pattern possibly produced by the paten shunt draining CSF from the subdural space resulting in cranial base hypoplasia.

O objetivo do estudo foi medir a acuidade visual (AV) em crianças com diagnóstico de hidrocefalia, apresentando ou não a válvula de derivação ventricular (DVP). Participaram da pesquisa um total de 55 crianças (34 F e 21M) com diagnóstico de hidrocefalia (45 com DVP e 10 sem DVP), com idade entre zero a 291 semanas (média 74 semanas). A AV foi medida pela técnica do Potencial Visual Evocado de Varredura e os resultados foram comparados a valores de referência (Norcia, 1985b). Os diagnósticos etiológicos que levaram à hidrocefalia foram: hemorragia intracraniana (25 crianças), mielomeningocele (20 crianças) e hidrocefalia congênita (10 crianças). O diagnóstico de hidrocefalia foi estabelecido em média no 16° dia de vida. Verificamos que, 31 crianças tiveram o procedimento realizado com menos de 15 dias do diagnóstico de hidrocefalia enquanto que em 14 a válvula foi instalada após 15 dias. Das 55 crianças avaliadas no 1° exame, 18 fizeram o 2° exame, 13 fizeram o 3° exame, 10 completaram quatro exames e apenas cinco completaram as cinco avaliações. Os resultados mostram que dentre as 101 avaliações de AV realizadas em todas as crianças, 95 exames (94%) se mostraram alterados e seis normais. Não há diferença estatística entre a AV das crianças sem derivação em comparação com aquelas crianças cuja DVP foi realizada com tempo de derivação maior que 15 dias (p = 0.699). Houve, todavia, uma diferença estatística na AV, quando comparamos as crianças, cujas derivações foram realizadas em até 15 dias do diagnóstico da hidrocefalia, com aquelas cuja derivação foi realizada após 15 dias (p = 0.038) ou com aquelas que não foram derivadas (p = 0.031). Crianças que não tiveram complicações na DVP tiveram um melhor resultado de AV quando comparado com o grupo com complicação (p = 0.0001). No grupo de crianças com complicação, novamente aquelas que foram derivadas com tempo inferior a 15 dias do diagnóstico da hidrocefalia apresentou melhores resultados de AV quando comparado com aquelas crianças com complicação cuja derivação foi realizada com mais de 15 dias (p = 0.029). Nós concluímos que é possível a medida da acuidade visual de resolução pelos PVEs de varredura em crianças com diagnóstico de hidrocefalia e os valores geralmente são piores comparando aos valores normativos. O tempo da derivação e o número de complicações decorrentes da DVP parecem interferir nesta diferença. Em nosso conhecimento, este foi o primeiro trabalho a avaliar progressivamente, a acuidade visual de crianças, nos primeiros anos de vida, com hidrocefalia, com ou sem DVP e correlacionar o resultado com o tempo de derivações e número de complicações. Apesar da grande evolução tecnológica alcançada pela oftalmologia, ainda não somos capazes de prevenir as alterações visuais detectáveis pelo simples exame de AV nas crianças com hidrocefalia. O tempo para a inserção da válvula de derivação e o tratamento para as complicações ainda são medidas retardadas devido à prematuridade, instabilidade hemodinâmica e a não autorização do familiar. Estes motivos podem comprometer o desenvolvimento visual destas crianças. / The objective was to measure the visual acuity (VA) of children with the diagnosis of hydrocephalus with or without peritoneal-ventricular shunt (PVS). A total of 55 children with the diagnosis of hydrocephalus (45 with PVS and 10 without PVS) were included in the study (34F and 21M), with an age range of 0 to 291 weeks (mean=74 weeks). The VA was measured by the sweep visual evoked potential technique and the results were compared with reference values proposed by Norcia (1985b). Etiological diagnosis of the hydrocephalus was as follows: intracranial hemorrhage (25 children), meningomyelocele (20 children) and congenital hydrocephalus (10 children). The diagnosis of hydrocephalus was made in an average time of 16 days of life. After the diagnosis, the insertion of the PVS, when made, was accomplished in average on the 16th day. Of those with a PVS, in 31 the ventricular valve was inserted before 15 days after the diagnosis whereas in 14 in shunt was inserted after 15 days. Of the 55 children evaluated in the first exam, 18 were evaluated in a second exam, 13 did the third exam, 10 completed 4 exams and only 5 completed the 5 evaluations. The results of the 101 sweep visual evoked potential performed in all children, 95 exams (94%) were abnormal and only 6 were normal. There was no statistical difference in the VA of children without a ventricular shunt in comparison with those in which the shunt was inserted after 15 days of the diagnosis of hydrocephalus (p=0.699). There was, however, a statistical difference in the VA between children with a ventricular shunt inserted before 15 days of the diagnosis and children with a ventricular shunt after 15 days (p=0.038) or those without a shunt (p=0.031). Children with no complications of the ventricular shunt had a better VA as compared to those with shunt complications (p= 0.0001). In the group of children with complications, again those who had a shunt inserted before 15 days bad better VA results in comparison to those in whom the shunt was inserted after 15days (p=0.029). We concluded that measuring visual acuity by sweep visual evoked potential is feasible in children with the diagnosis of hydrocephalus and that results are usually worse comparing with reference values. The timing for insertion of a ventricular shunt and the occurrence of complications of this procedure are factors that may influence the VA. To our knowledge, this is the first study that progressively evaluated the VA in children, of early age, with hydrocephalus, with or without a ventricular shunt and correlated the VA with the timing and complications of the ventricular shunt. Despite the great technological advance in ophthalmology, we are still unable to prevent visual impairments, detectable by simple visual acuity tests, in children with hydrocephalus. The timing of the insertion of the ventricular shunt and the adequate treatment of the potential complications may be delayed due to prematurity, hemodynamic instability and family refusal. This delay may compromise the visual development of these children.

O objetivo do estudo foi medir a acuidade visual (AV) em crianças com diagnóstico de hidrocefalia, apresentando ou não a válvula de derivação ventricular (DVP). Participaram da pesquisa um total de 55 crianças (34 F e 21M) com diagnóstico de hidrocefalia (45 com DVP e 10 sem DVP), com idade entre zero a 291 semanas (média 74 semanas). A AV foi medida pela técnica do Potencial Visual Evocado de Varredura e os resultados foram comparados a valores de referência (Norcia, 1985b). Os diagnósticos etiológicos que levaram à hidrocefalia foram: hemorragia intracraniana (25 crianças), mielomeningocele (20 crianças) e hidrocefalia congênita (10 crianças). O diagnóstico de hidrocefalia foi estabelecido em média no 16° dia de vida. Verificamos que, 31 crianças tiveram o procedimento realizado com menos de 15 dias do diagnóstico de hidrocefalia enquanto que em 14 a válvula foi instalada após 15 dias. Das 55 crianças avaliadas no 1° exame, 18 fizeram o 2° exame, 13 fizeram o 3° exame, 10 completaram quatro exames e apenas cinco completaram as cinco avaliações. Os resultados mostram que dentre as 101 avaliações de AV realizadas em todas as crianças, 95 exames (94%) se mostraram alterados e seis normais. Não há diferença estatística entre a AV das crianças sem derivação em comparação com aquelas crianças cuja DVP foi realizada com tempo de derivação maior que 15 dias (p = 0.699). Houve, todavia, uma diferença estatística na AV, quando comparamos as crianças, cujas derivações foram realizadas em até 15 dias do diagnóstico da hidrocefalia, com aquelas cuja derivação foi realizada após 15 dias (p = 0.038) ou com aquelas que não foram derivadas (p = 0.031). Crianças que não tiveram complicações na DVP tiveram um melhor resultado de AV quando comparado com o grupo com complicação (p = 0.0001). No grupo de crianças com complicação, novamente aquelas que foram derivadas com tempo inferior a 15 dias do diagnóstico da hidrocefalia apresentou melhores resultados de AV quando comparado com aquelas crianças com complicação cuja derivação foi realizada com mais de 15 dias (p = 0.029). Nós concluímos que é possível a medida da acuidade visual de resolução pelos PVEs de varredura em crianças com diagnóstico de hidrocefalia e os valores geralmente são piores comparando aos valores normativos. O tempo da derivação e o número de complicações decorrentes da DVP parecem interferir nesta diferença. Em nosso conhecimento, este foi o primeiro trabalho a avaliar progressivamente, a acuidade visual de crianças, nos primeiros anos de vida, com hidrocefalia, com ou sem DVP e correlacionar o resultado com o tempo de derivações e número de complicações. Apesar da grande evolução tecnológica alcançada pela oftalmologia, ainda não somos capazes de prevenir as alterações visuais detectáveis pelo simples exame de AV nas crianças com hidrocefalia. O tempo para a inserção da válvula de derivação e o tratamento para as complicações ainda são medidas retardadas devido à prematuridade, instabilidade hemodinâmica e a não autorização do familiar. Estes motivos podem comprometer o desenvolvimento visual destas crianças. / The objective was to measure the visual acuity (VA) of children with the diagnosis of hydrocephalus with or without peritoneal-ventricular shunt (PVS). A total of 55 children with the diagnosis of hydrocephalus (45 with PVS and 10 without PVS) were included in the study (34F and 21M), with an age range of 0 to 291 weeks (mean=74 weeks). The VA was measured by the sweep visual evoked potential technique and the results were compared with reference values proposed by Norcia (1985b). Etiological diagnosis of the hydrocephalus was as follows: intracranial hemorrhage (25 children), meningomyelocele (20 children) and congenital hydrocephalus (10 children). The diagnosis of hydrocephalus was made in an average time of 16 days of life. After the diagnosis, the insertion of the PVS, when made, was accomplished in average on the 16th day. Of those with a PVS, in 31 the ventricular valve was inserted before 15 days after the diagnosis whereas in 14 in shunt was inserted after 15 days. Of the 55 children evaluated in the first exam, 18 were evaluated in a second exam, 13 did the third exam, 10 completed 4 exams and only 5 completed the 5 evaluations. The results of the 101 sweep visual evoked potential performed in all children, 95 exams (94%) were abnormal and only 6 were normal. There was no statistical difference in the VA of children without a ventricular shunt in comparison with those in which the shunt was inserted after 15 days of the diagnosis of hydrocephalus (p=0.699). There was, however, a statistical difference in the VA between children with a ventricular shunt inserted before 15 days of the diagnosis and children with a ventricular shunt after 15 days (p=0.038) or those without a shunt (p=0.031). Children with no complications of the ventricular shunt had a better VA as compared to those with shunt complications (p= 0.0001). In the group of children with complications, again those who had a shunt inserted before 15 days bad better VA results in comparison to those in whom the shunt was inserted after 15days (p=0.029). We concluded that measuring visual acuity by sweep visual evoked potential is feasible in children with the diagnosis of hydrocephalus and that results are usually worse comparing with reference values. The timing for insertion of a ventricular shunt and the occurrence of complications of this procedure are factors that may influence the VA. To our knowledge, this is the first study that progressively evaluated the VA in children, of early age, with hydrocephalus, with or without a ventricular shunt and correlated the VA with the timing and complications of the ventricular shunt. Despite the great technological advance in ophthalmology, we are still unable to prevent visual impairments, detectable by simple visual acuity tests, in children with hydrocephalus. The timing of the insertion of the ventricular shunt and the adequate treatment of the potential complications may be delayed due to prematurity, hemodynamic instability and family refusal. This delay may compromise the visual development of these children.

Σποραδικές μελέτες περιγράφουν την προσωρινή εξωτερική οσφυϊκή παροχέτευση του ΕΝΥ, ως μία δοκιμασία υψηλής ακριβείας όσον αφορά στην πρόβλεψη του κλινικού αποτελέσματος μετά από κοιλιοπεριτοναϊκή παροχέτευση στους ασθενείς με ΥΦΠ. Παρόλα αυτά, σε μία πιο πρόσφατη μελέτη από τους Walchenbach και συνεργάτες, εκτιμήθηκε ότι η θετική προγνωστική αξία της ΕΟΠ ήταν υψηλή, ενώ η αρνητική χαμηλή. Δια ταύτα, πραγματοποιήσαμε μία προοπτική μελέτη με σκοπό να εκτιμήσουμε την προγνωστική αξία μιάς προσωρινής εξωτερικής οσφυικής παροχέτευσης (ΕΟΠ), η οποία ρυθμίζει την εκροή ΕΝΥ μέσω μιάς βαλβίδας μέσης πίεσης μονόδρομης ροής, σε ασθενείς με υδροκέφαλο φυσιολογικής πίεσης (ΥΦΠ). Είκοσι επτά 27 ασθενείς με ΥΦΠ εισήχθησαν στη Νευροχειρουργική Κλινική του ΠΓΝΠ και υπεβλήθησαν σε τοποθέτηση προσωρινής εξωτερικής οσφυικής παροχέτευσης (ΕΟΠ), η οποία ρυθμίζει την εκροή ΕΝΥ μέσω μιάς βαλβίδας μέσης πίεσης, για 5 ημέρες. 22 ασθενείς υπεβλήθησαν τελικά σε κοιλιοπεριτοναϊκή παροχέτευση με βαλβίδα μέσης πίεσης, βασιζόμενοι σε κλινικά και ακτινολογικά κριτήρια, ανεξαρτήτως του αποτελέσματος της ΕΟΠ. Κλινική εκτίμηση των διαταραχών της βάδισης, της ούρησης και της νόησης, όπως επίσης και ακτινολογική εκτίμηση, πραγματοποιήθηκε προ και μετά την δοκιμασία της ΕΟΠ καθώς και 3 μήνες μετά την ΚΠΠ. Είκοσι δύο 22 ασθενείς υπεβλήθησαν τελικά σε κοιλιοπεριτοναϊκή παροχέτευση και πληρούσαν τα κριτήρια για να συμπεριληφθούν τελικά στη μελέτη. Χρησιμοποιώντας ένα εγκεκριμένο σύστημα βαθμολόγησης, η συνολική βελτίωση μετά τη δοκιμασία ΕΟΠ συσχετιζόταν καλά με τη βελτίωση 3 μήνες μετά την μόνιμη ΚΠΠ. (Spearman’ s rho = 0,462, p=0,03). Όταν θεωρήσαμε κάθε βαθμό βελτίωσης ως θετική ανταπόκριση, η δοκιμασία ΕΟΠ-βαλβίδας μέσης πίεσης είχε υψηλή θετική προγνωστική αξία για κάθε μία ανεξάρτητη κλινική παράμετρο (διαταραχές της βάδισης 94%, 95% CI 71%-100%, ακράτεια στην ούρηση 100%, 95% CI 66%-100%, διαταραχές της νόησης 100%, 95% CI 66-100%), ενώ η αρνητική προγνωστική αξία ήταν χαμηλή (<50%) εκτός από τις διαταραχές της νόησης (85%, 95% CI 55%-98%). Η ανωτέρω μελέτη συμπεραίνει ότι η δοκιμασία της ΕΟΠ-βαλβίδας μέσης πίεσης, θα πρέπει να θεωρείται ένα αξιόπιστο κριτήριο για την προεγχειρητική επιλογή υδροκεφαλικών ασθενών φυσιολογικής πίεσης που βελτιώνονται κλινικά μετά την τοποθέτηση ΚΠΠ. Σε περίπτωση αρνητικής δοκιμασίας ΕΟΠ-βαλβίδας μέσης πίεσης, θα πρέπει να διενεργείται περαιτέρω διερεύνηση με επιπρόσθετες διαγνωστικές δοκιμασίες. / Although sporadic studies have described temporary external cerebrospinal fluid (CSF) lumbar drainage as a highly accurate test in predicting the outcome after ventricular shunting in normal pressure hydrocephalus (NPH) patients, a more recent study reports that the positive predictive value of external lumbar drainage (ELD) is high but the negative predictive value is deceptively low. Therefore, we conducted a prospective study in order to evaluate the predictive value of a continuous ELD, with CSF outflow controlled by medium pressure valve, in NPH patients. Twenty-seven patients with presumed NPH were admitted to our department and CSF drainage was carried out by a temporary (ELD), with CSF outflow controlled by medium pressure valve for five days. Twenty-two patients received a ventriculo¬peritoneal shunt with medium pressure valve based upon preoperative clinical and radiographic criteria of NPH, regardless of ELD outcome. Clinical evaluation of gait disturbances, urinary incontinence and mental status, and radiological evaluation with brain CT was performed prior to and after ELD test, as well as three months after shunting. Twenty-two patients were finally shunted and included in this study. In a three-month follow-up, using a previously validated score system, overall improvement after permanent shunting correlated well with improvement after ELD test (Spearman’s rho=0.462, p=0.03). When considering any degree of improvement as a positive response, ELD test yielded high positive predictive values for all individual parameters (gait disturbances 94%, 95% CI 71%-100%, urinary incontinence 100%, 95% CI 66%-100%, and mental status 100%, 95% CI 66%-100%) but negative predictive values were low (<50%) except for cognitive impairment (85%, 95% CI 55%-98%). This study suggests that a positive ELD-valve system test should be considered a reliable criterion for preoperative selection of shunt-responsive NPH patients. In case of a negative ELD-valve system test, further investigation of the presumed NPH patients with additional tests should be performed.