The impact of a biocellulose-based repair of fetal open spina bifida on the need to untether the cord: is it time to unify techniques for prenatal repair?

OBJECTIVE: To report the need for cord untethering after prenatal repair of open spina bifida using a unique biocellulose-based technique performed at a later gestational age. METHODS: An observational cohort study was conducted to determine the incidence of tethered cord syndrome. Between May 2013 and May 2022, we performed 172 procedures using the percutaneous fetoscopic approach in fetuses at 26-28 weeks of gestation. After placode dissection, a biocellulose patch was placed to cover the placode, a myofascial flap (when possible) was dissected, and the skin was closed. Owing to death or loss to follow-up, 23 cases were excluded. Cord tethering syndrome was defined as symptoms of medullary stretching, and the infants were evaluated and operated on by local neurosurgeons after an magnetic resonance imaging examination. Infants over 30-month had ambulation and neurodevelopment evaluations (PEDI scale). RESULTS: Among 172 cases operated at a median gestational age of 26.7 weeks and delivered at 33.2 weeks, 149 cases were available for postnatal follow-up, and cord untethering was needed in 4.4% of cases (6/136; excluding 13 cases younger than 12 months). Cerebrospinal fluid diversion and bladder catheterization were needed in 38% and 36% of cases, respectively. Of the 78 cases evaluated at 30 months, 49% were ambulating independently, and 94% had normal social function. CONCLUSION: The biocellulose-based technique was associated with a low rate of cord tethering, wich may be attributed to the lack of the duramater suture during prenatal repair, the formation of a neoduramater and/or later gestational age of surgery.

The impact of a biocellulose-based repair of fetal open spina bifida on the need to untether the cord: is it time to unify techniques for prenatal repair?

ABSTRACT Objective: To report the need for cord untethering after prenatal repair of open spina bifida using a unique biocellulose-based technique performed at a later gestational age. Methods: An observational cohort study was conducted to determine the incidence of tethered cord syndrome. Between May 2013 and May 2022, we performed 172 procedures using the percutaneous fetoscopic approach in fetuses at 26-28 weeks of gestation. After placode dissection, a biocellulose patch was placed to cover the placode, a myofascial flap (when possible) was dissected, and the skin was closed. Owing to death or loss to follow-up, 23 cases were excluded. Cord tethering syndrome was defined as symptoms of medullary stretching, and the infants were evaluated and operated on by local neurosurgeons after an magnetic resonance imaging examination. Infants over 30-month had ambulation and neurodevelopment evaluations (PEDI scale). Results: Among 172 cases operated at a median gestational age of 26.7 weeks and delivered at 33.2 weeks, 149 cases were available for postnatal follow-up, and cord untethering was needed in 4.4% of cases (6/136; excluding 13 cases younger than 12 months). Cerebrospinal fluid diversion and bladder catheterization were needed in 38% and 36% of cases, respectively. Of the 78 cases evaluated at 30 months, 49% were ambulating independently, and 94% had normal social function. Conclusion: The biocellulose-based technique was associated with a low rate of cord tethering, wich may be attributed to the lack of the duramater suture during prenatal repair, the formation of a neoduramater and/or later gestational age of surgery.

Ultrasound diagnosis of microcephaly: a comparison of three reference curves and postnatal diagnosis.

PURPOSE: To evaluate which reference curve (RC)-Snijders, Intergrowth 21st (IG21) and World Health Organization (WHO)-is more accurate for microcephaly diagnosis. METHODS: Retrospective cohort study with more than 30,000 exams in more than 11,000 women. Microcephaly was confirmed by a neonatologist at birth and positive predictive values (PPVs) and misdiagnosis were assessed. RESULTS: A total of 71 cases were confirmed as microcephaly at birth. IG21 and Snijders PPVs showed to be more significant over WHO's (p < 0.001), without difference between them (p = 0.39). All RC were superimposed and did not show significant difference. When evaluated in different fragments, three trends were observed (until 30 weeks, between 30 and 36 and after 36 weeks of gestational age), with the latter interval showing a significant difference between IG21 and WHO (p = 0.0079). Conversely, WHO exhibited only one misdiagnosis, a much lower rate than Snijders, who missed eight cases and IG21, nine. CONCLUSION: WHO's RC appears to misdiagnose fewer cases, which could be useful for a population screening, while IG21's RC presented a more significant PPV, being more useful for a more precise final diagnosis in reference centers.

Evaluation of Cases of Abdominal Wall Endometriosis at Universidade Estadual de Campinas in a period of 10 Years / Avaliação dos casos de endometriose de parede abdominal na Universidade Estadual de Campinas em um período de 10 anos

Abstract Purpose To determine the clinical and epidemiological characteristics of abdominal wall endometriosis (AWE), as well as the rate and recurrence factors for the disease. Methods A retrospective study of 52 women with AWE was performed at Universidade Estadual de Campinas from 2004 to 2014. Of the 231 surgeries performed for the diagnosis of endometriosis, 52 women were found to have abdominal wall endometriosis (AWE). The frequencies, means and standard deviations of the clinical characteristics of these women were calculated, as well as the recurrence rate of AWE. To determine the risk factors for disease recurrence, Fisher's exact test was used. Results The mean age of the patients was 30.71 ± 5.91 years. The main clinical manifestations were pain (98%) and sensation of a mass (36.5%).We observed that 94% of these women had undergone at least 1 cesarean section, and 73% had used medication for the postoperative control of endometriosis. The lesion was most commonly located in the cesarean section scar (65%). The recurrence rate of the disease was of 26.9%. All 14 women who had relapsed had surgical margins compromised in the previous surgery. There was no correlation between recurrent AWE and a previous cesarean section (p = 0.18), previous laparotomy (p = 0.11), previous laparoscopy (p = 0.12) and postoperative hormone therapy (p = 0.51). Conclusion Women with previous cesarean sections with local pain or lumps should be investigated for AWE. The recurrence of AWE is high, especially when the first surgery is not appropriate and leaves compromised surgical margins.

Resumo Objetivos Determinar as características clínicas e epidemiológicas da endometriose de parede, bem como sua taxa de recorrência e os fatores que levam a ela. Métodos Estudo retrospectivo, em que se avaliaram 52 mulheres com endometriose de parede na Universidade Estadual de Campinas no período de 2004 a 2014. Entre as 231 cirurgias para diagnosticar endometriose, foram encontradas 52 mulheres que apresentavam endometriose de parede. Foram calculadas as frequências, a média e o desvio padrão das características clínicas destas mulheres, bem como a taxa de recorrência da endometriose de parede. Para determinar os fatores de risco de recorrência, foi utilizado o teste exato de Fisher. Resultados A idade média das mulheres foi de 30.71 ± 5,91 anos. As principais manifestações clínicas foram dor (98%) e sensação do nódulo (36,5%). Foi observado que 94% dessas mulheres tinham pelo menos uma cesárea, e 73% destas fizeram uso de medicação para controle da endometriose no pós-operatório. A localizaçãomais frequente da lesão foi na cicatriz da cesárea (65%). A taxa de recorrência da doença foi de 26,9%. Todas as 14mulheres que tiveramrecidiva tinhammargens cirúrgicas comprometidas na cirurgia prévia. Não houve correlação entre a endometriose de parede recorrente e a cesariana prévia (p = 0,18), a laparotomia prévia (p = 0,11), a laparoscopia prévia (p = 0,12) e receber terapia hormonal no pós-operatório (p = 0,51). Conclusão Mulheres com antecedente de cesárea anterior com dor local ou nódulo devem ser investigadas com relação à endometriose de parede. A recorrência do endometrioma de parede é alta, principalmente quando a primeira cirurgia não é adequada, e deixa margens cirúrgicas comprometidas.

Evaluation of Cases of Abdominal Wall Endometriosis at Universidade Estadual de Campinas in a period of 10 Years.

Purpose To determine the clinical and epidemiological characteristics of abdominal wall endometriosis (AWE), as well as the rate and recurrence factors for the disease. Methods A retrospective study of 52 women with AWE was performed at Universidade Estadual de Campinas from 2004 to 2014. Of the 231 surgeries performed for the diagnosis of endometriosis, 52 women were found to have abdominal wall endometriosis (AWE). The frequencies, means and standard deviations of the clinical characteristics of these women were calculated, as well as the recurrence rate of AWE. To determine the risk factors for disease recurrence, Fisher's exact test was used. Results The mean age of the patients was 30.71 ± 5.91 years. The main clinical manifestations were pain (98%) and sensation of a mass (36.5%). We observed that 94% of these women had undergone at least 1 cesarean section, and 73% had used medication for the postoperative control of endometriosis. The lesion was most commonly located in the cesarean section scar (65%). The recurrence rate of the disease was of 26.9%. All 14 women who had relapsed had surgical margins compromised in the previous surgery. There was no correlation between recurrent AWE and a previous cesarean section (p = 0.18), previous laparotomy (p = 0.11), previous laparoscopy (p = 0.12) and postoperative hormone therapy (p = 0.51). Conclusion Women with previous cesarean sections with local pain or lumps should be investigated for AWE. The recurrence of AWE is high, especially when the first surgery is not appropriate and leaves compromised surgical margins.

Objetivos Determinar as características clínicas e epidemiológicas da endometriose de parede, bem como sua taxa de recorrência e os fatores que levam a ela. Métodos Estudo retrospectivo, em que se avaliaram 52 mulheres com endometriose de parede na Universidade Estadual de Campinas no período de 2004 a 2014. Entre as 231 cirurgias para diagnosticar endometriose, foram encontradas 52 mulheres que apresentavam endometriose de parede. Foram calculadas as frequências, a média e o desvio padrão das características clínicas destas mulheres, bem como a taxa de recorrência da endometriose de parede. Para determinar os fatores de risco de recorrência, foi utilizado o teste exato de Fisher. Resultados A idade média das mulheres foi de 30.71 ± 5,91 anos. As principais manifestações clínicas foram dor (98%) e sensação do nódulo (36,5%). Foi observado que 94% dessas mulheres tinham pelo menos uma cesárea, e 73% destas fizeram uso de medicação para controle da endometriose no pós-operatório. A localização mais frequente da lesão foi na cicatriz da cesárea (65%). A taxa de recorrência da doença foi de 26,9%. Todas as 14 mulheres que tiveram recidiva tinham margens cirúrgicas comprometidas na cirurgia prévia. Não houve correlação entre a endometriose de parede recorrente e a cesariana prévia (p = 0,18), a laparotomia prévia (p = 0,11), a laparoscopia prévia (p = 0,12) e receber terapia hormonal no pós-operatório (p = 0,51). Conclusão Mulheres com antecedente de cesárea anterior com dor local ou nódulo devem ser investigadas com relação à endometriose de parede. A recorrência do endometrioma de parede é alta, principalmente quando a primeira cirurgia não é adequada, e deixa margens cirúrgicas comprometidas.

[Beta thalassemia major and pregnancy during adolescence: report of two cases]. / Talassemia beta maior e gestação na adolescência: relato de dois casos.

Beta thalassemia major is a rare hereditary blood disease in which impaired synthesis of beta globin chains causes severe anemia. Medical treatment consists of chronic blood transfusions and iron chelation. We describe two cases of adolescents with beta thalassemia major with unplanned pregnancies and late onset of prenatal care. One had worsening of anemia with increased transfusional requirement, fetal growth restriction, and placental senescence. The other was also diagnosed with hypothyroidism and low maternal weight, and was admitted twice during pregnancy due to dengue shock syndrome and influenza H1N1-associated respiratory infection. She also developed fetal growth restriction and underwent vaginal delivery at term complicated by uterine hypotonia. Both patients required blood transfusions after birth and chose medroxyprogesterone as a contraceptive method afterwards. This report highlights the importance of medical advice on contraceptive methods for these women and the role of a specialized prenatal follow-up in association with a hematologist.

Talassemia beta maior e gestação na adolescência: relato de dois casos / Beta thalassemia major and pregnancy during adolescence: report of two cases

A talassemia beta maior é uma doença hematológica hereditária rara em que deficiência na síntese de cadeias globínicas beta causa anemia grave. O tratamento consiste de transfusão sanguínea e quelação de ferro. Descrevemos dois casos de adolescentes com talassemia beta maior, com gestação não planejada e início tardio de pré-natal. Uma delas apresentou piora da anemia, necessidade transfusional aumentada, restrição de crescimento fetal e senescência placentária. A outra apresentava também hipotireoidismo e baixo peso materno, e foi internada por duas ocasiões durante a gestação, por choque hemorrágico do dengue e por infecção respiratória associada a vírus influenza H1N1. Uma delas apresentou restrição de crescimento fetal e teve parto vaginal no termo complicado com hipotonia uterina. Ambas necessitaram de transfusão sanguínea no pós-parto e optaram por medroxiprogesterona como método contraceptivo subsequentemente. Esse relato ressalta a importância de orientação contraceptiva para essas mulheres e o papel do cuidado pré-natal especializado em conjunto com hematologista.

Beta thalassemia major is a rare hereditary blood disease in which impaired synthesis of beta globin chains causes severe anemia. Medical treatment consists of chronic blood transfusions and iron chelation. We describe two cases of adolescents with beta thalassemia major with unplanned pregnancies and late onset of prenatal care. One had worsening of anemia with increased transfusional requirement, fetal growth restriction, and placental senescence. The other was also diagnosed with hypothyroidism and low maternal weight, and was admitted twice during pregnancy due to dengue shock syndrome and influenza H1N1-associated respiratory infection. She also developed fetal growth restriction and underwent vaginal delivery at term complicated by uterine hypotonia. Both patients required blood transfusions after birth and chose medroxyprogesterone as a contraceptive method afterwards. This report highlights the importance of medical advice on contraceptive methods for these women and the role of a specialized prenatal follow-up in association with a hematologist.