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1.
J Pediatr Surg ; 59(5): 969-974, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38042733

RESUMO

BACKGROUND: Open fetal resection for large lung lesions has virtually been replaced by maternal steroid administration. Despite this paradigm shift, little is known about the effects steroids have on lung lesion growth in utero. METHODS: A 10-year retrospective review of all prenatally diagnosed lung lesions cared for at our fetal care center was performed. We evaluated the effects of prenatal steroids on congenital pulmonary airway malformation (CPAM)-volume-ratio (CVR), distinguishing change in CVR among CPAMs, bronchopulmonary sequestrations (BPS), and bronchial atresias. We also correlated fetal ultrasound and MRI findings with pathology to determine the accuracy of prenatal diagnosis. RESULTS: We evaluated 199 fetuses with a prenatal lung lesion. Fifty-four (27 %) were treated with prenatal steroids with a subsequent 21 % mean reduction in the CVR (2.1 ± 1.4 to 1.1 ± 0.4, p = 0.003). Fetuses with hydrops and mediastinal shift who were treated with steroids rarely had resolution of these radiographic findings. Postnatal pathology was available for 91/199 patients (45.7 %). The most common diagnosis was CPAM (42/91, 46 %), followed by BPS (30/91, 33 %), and bronchial atresia (14/91, 15 %). Fetuses who received steroids and had pathology consistent with CPAM were more likely to have a reduction in their CVR (p = 0.02). Fetal ultrasound correctly diagnosed the type of lung lesion in 75 % of cases and fetal MRI in 81 % of cases. CONCLUSIONS: Prenatally diagnosed CPAMs are more likely to respond to maternal steroids than BPS or bronchial atresias. Knowing the diagnosis in utero could aid to steward steroid usage, however, fetal imagining modalities are not perfect in distinguishing subtype. LEVEL OF EVIDENCE: III.

2.
Obstet Gynecol ; 143(3): 440-448, 2024 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-38128107

RESUMO

OBJECTIVE: To report the outcomes of fetoscopic endoluminal tracheal occlusion in a multicenter North American cohort of patients with isolated, left-sided congenital diaphragmatic hernia (CDH) and to compare neonatal mortality and morbidity in patients with severe left-sided congenital diaphragmatic hernia who underwent fetoscopic endoluminal tracheal occlusion with those expectantly managed. METHODS: We analyzed data from 10 centers in the NAFTNet (North American Fetal Therapy Network) FETO (Fetoscopic Endoluminal Tracheal Occlusion) Consortium registry, collected between November 1, 2008, and December 31, 2020. In addition to reporting procedure-related surgical outcomes of fetoscopic endoluminal tracheal occlusion, we performed a comparative analysis of fetoscopic endoluminal tracheal occlusion compared with contemporaneous expectantly managed patients. RESULTS: Fetoscopic endoluminal tracheal occlusion was successfully performed in 87 of 89 patients (97.8%). Six-month survival in patients with severe left-sided congenital diaphragmatic hernia did not differ significantly between patients who underwent fetoscopic endoluminal tracheal occlusion and those managed expectantly (69.8% vs 58.1%, P =.30). Patients who underwent fetoscopic endoluminal tracheal occlusion had higher rates of preterm prelabor rupture of membranes (54.0% vs 14.3%, P <.001), earlier gestational age at delivery (median 35.0 weeks vs 38.3 weeks, P <.001), and lower birth weights (mean 2,487 g vs 2,857 g, P =.001). On subanalysis, in patients for whom all recorded observed-to-expected lung/head ratio measurements were below 25%, patients with fetoscopic endoluminal tracheal occlusion required fewer days of extracorporeal membrane oxygenation (ECMO) (median 9.0 days vs 17.0 days, P =.014). CONCLUSION: In this cohort, fetoscopic endoluminal tracheal occlusion was successfully implemented across several North American fetal therapy centers. Although survival was similar among patients undergoing fetoscopic endoluminal tracheal occlusion and those expectantly managed, fetoscopic endoluminal tracheal occlusion in North American centers may reduce morbidity, as suggested by fewer days of ECMO in those patients with persistently reduced lung volumes (observed-to-expected lung/head ratio below 25%).


Assuntos
Obstrução das Vias Respiratórias , Oclusão com Balão , Hérnias Diafragmáticas Congênitas , Gravidez , Recém-Nascido , Feminino , Humanos , Lactente , Hérnias Diafragmáticas Congênitas/cirurgia , Fetoscopia/efeitos adversos , Pulmão , Feto , Obstrução das Vias Respiratórias/etiologia , América do Norte , Traqueia/cirurgia , Oclusão com Balão/efeitos adversos
3.
Prenat Diagn ; 43(12): 1514-1519, 2023 11.
Artigo em Inglês | MEDLINE | ID: mdl-37807606

RESUMO

OBJECTIVE: Giant omphaloceles (GO) have associated pulmonary hypoplasia and respiratory complications. Total lung volumes (TLV) on fetal MRI can prognosticate congenital diaphragmatic hernia outcomes; however, its applicability to GO is unknown. We hypothesize that late gestation TLV and observed-to-expected TLV (O/E TLV) on fetal MRI correlate with postnatal pulmonary morbidity in GO. METHOD: A single-institution retrospective review of GO evaluated between 2012 and 2022 was performed. Fetal MRI TLV between 32 and 36 weeks' gestation and O/E TLV throughout gestation were calculated and correlated with postnatal outcomes. RESULTS: 86 fetuses with omphaloceles were evaluated; however, only 26 met strict inclusion criteria. MRIs occurred between 18 and 36 weeks' gestation. Those requiring delivery room intubation had significantly lower late gestation TLV and O/E TLV. O/E TLV predicted tracheostomy placement and survival. Neither TLV nor O/E TLV predicted the length of hospitalization or supplemental oxygen after discharge. Three fetuses had a TLV less than 35 mL: one died of respiratory failure, and the other two required tracheostomy. CONCLUSIONS: Fetal MRI TLV measured between 32 and 36 weeks' gestation and O/E TLV predict the need for delivery room intubation and tracheostomy. O/E TLV correlated with survival. These data support fetal MRI as a prognostic tool to predict GO associated pulmonary morbidity.


Assuntos
Hérnia Umbilical , Hérnias Diafragmáticas Congênitas , Lactente , Feminino , Gravidez , Humanos , Hérnia Umbilical/complicações , Pulmão/diagnóstico por imagem , Medidas de Volume Pulmonar , Hérnias Diafragmáticas Congênitas/complicações , Hérnias Diafragmáticas Congênitas/diagnóstico por imagem , Feto , Estudos Retrospectivos , Imageamento por Ressonância Magnética , Morbidade
4.
Fetal Diagn Ther ; 49(3): 117-124, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-34915495

RESUMO

INTRODUCTION: Uterine incision based on the placental location in open maternal-fetal surgery (OMFS) has never been evaluated in regard to maternal or fetal outcomes. OBJECTIVE: The aim of this study was to investigate whether an anterior placenta was associated with increased rates of intraoperative, perioperative, antepartum, obstetric, or neonatal complications in mothers and babies who underwent OMFS for fetal myelomeningocele (fMMC) closure. METHODS: Data from the international multicenter prospective registry of patients who underwent OMFS for fMMC closure (fMMC Consortium Registry, December 15, 2010-June 31, 2019) was used to compare fetal and maternal outcomes between anterior and posterior placental locations. RESULTS: The placental location for 623 patients was evenly distributed between anterior (51%) and posterior (49%) locations. Intraoperative fetal bradycardia (8.3% vs. 3.0%, p = 0.005) and performance of fetal resuscitation (3.6% vs. 1.0%, p = 0.034) occurred more frequently in cases with an anterior placenta when compared to those with a posterior placenta. Obstetric outcomes including membrane separation, placental abruption, and spontaneous rupture of membranes were not different among the 2 groups. However, thinning of the hysterotomy site (27.7% vs. 17.7%, p = 0.008) occurred more frequently in cases of an anterior placenta. Gestational age (GA) at delivery (p = 0.583) and length of stay in the neonatal intensive care unit (p = 0.655) were similar between the 2 groups. Fetal incision dehiscence and wound revision were not significantly different between groups. Critical clinical outcomes including fetal demise, perinatal death, and neonatal death were all infrequent occurrences and not associated with the placental location. CONCLUSIONS: An anterior placental location is associated with increased risk of intraoperative fetal resuscitation and increased thinning at the hysterotomy closure site. Individual institutional experiences may have varied, but the aggregate data from the fMMC Consortium did not show a significant impact on the GA at delivery or maternal or fetal clinical outcomes.


Assuntos
Terapias Fetais , Meningomielocele , Feminino , Terapias Fetais/efeitos adversos , Idade Gestacional , Humanos , Histerotomia/efeitos adversos , Recém-Nascido , Meningomielocele/etiologia , Meningomielocele/cirurgia , Placenta/cirurgia , Gravidez
5.
Am J Obstet Gynecol MFM ; 3(2): 100296, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-33485023

RESUMO

BACKGROUND: For pregnancies complicated by fetal myelomeningocele who meet the established criteria, prenatal closure is a viable management option. Prenatal closure is an open procedure, with some techniques requiring greater dissection of maternal tissue than cesarean delivery; pain control is an important postoperative goal. Given the rising rates of opioid dependence and concerns regarding the fetal and neonatal effects of opioid use, our practice has turned to nonopioid pain management techniques. OBJECTIVE: This study aimed to compare postoperative opioid use and pain scores in women undergoing open fetal myelomeningocele repair with and without continuous local bupivacaine wound infusion. STUDY DESIGN: This was a retrospective, single-center chart review of all consecutive patients who underwent open myelomeningocele repair from March 2013 to December 2019. Women were enrolled at the time of referral and locally followed for 2 weeks postoperatively. The control group received patient-controlled epidural analgesia for 48 hours with acetaminophen and oral and intravenous opioids as needed. The treatment group received patient-controlled epidural analgesia for 24 hours with acetaminophen, oral and intravenous opioids, and continuous local bupivacaine infusion. Pain scores, medication use, and postoperative milestones and complications through discharge were abstracted from the chart and compared. RESULTS: Of 72 subjects, 51 were in the control group and 21 in the treatment group. Total opioid use, including intravenous doses (165 vs 52.5 mg; P=.001) and daily average oral opioid use (30 vs 10.5 mg; P=.002) were lower in the treatment group. In addition, 24% of women in the treatment group used no opioid postoperatively, compared with 4% in the control group. There was no difference in postoperative day 1 to 4 pain scores, antiemetic use, or bowel function; the treatment group was discharged significantly earlier. CONCLUSION: Postoperative opioid use was reduced in women who received continuous local wound infusion of bupivacaine for incisional pain control after prenatal myelomeningocele repair. Pain control is paramount following open myelomeningocele repair; local bupivacaine wound infusion is an important adjunct to reduce opioid use postoperatively.


Assuntos
Meningomielocele , Transtornos Relacionados ao Uso de Opioides , Analgésicos Opioides/uso terapêutico , Anestésicos Locais , Bupivacaína , Feminino , Humanos , Recém-Nascido , Meningomielocele/cirurgia , Transtornos Relacionados ao Uso de Opioides/tratamento farmacológico , Dor Pós-Operatória/tratamento farmacológico , Gravidez , Estudos Retrospectivos
6.
Pediatr Surg Int ; 37(4): 425-430, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33399927

RESUMO

PURPOSE: The goal of this study was to determine the feasibility of identifying the anal dimple (AD) on routine prenatal ultrasound. Using the presence, absence, appearance, and location of the anal dimple as an indirect sign for possible underlying anorectal malformations (ARM), we hypothesize that evaluation of the anal dimple as part of the fetal anatomic survey may increase the sensitivity in detecting less severe ARMs. METHODS: In a prospective longitudinal observational study, pregnant women who underwent prenatal ultrasound (US) at the Colorado Fetal Care Center between January 2019 and 2020 were enrolled. The variables recorded included gestational age, singleton versus multiple pregnancy, gender of the fetus, visualization of the AD, and reason for non-visualization of the AD. RESULTS: A total of 900 ultrasounds were performed, evaluating 1044 fetuses, in 372 different pregnant women. Gestational ages ranged from 16 to 38 weeks. The AD was visualized in 612 fetuses (58.6%) and not seen in 432 (41.4%). The two most common reasons for non-visualization were extremes in gestational age (n = 155; 36%) and fetal position (n = 152; 35.3%). The optimal gestational age range for AD visualization was 28-33 weeks + 6 days, with 78.1% visualization rate. CONCLUSION: Visualization of the anal dimple by ultrasound is feasible and may aid in the detection of less severe ARMs, ultimately impacting pregnancy management and family counseling. The optimal timing for anal dimple visualization is late second and third trimester.


Assuntos
Malformações Anorretais , Diagnóstico Pré-Natal/métodos , Ultrassonografia Pré-Natal , Adulto , Canal Anal/anormalidades , Malformações Anorretais/diagnóstico por imagem , Colorado , Feminino , Idade Gestacional , Humanos , Lactente , Gravidez , Cuidado Pré-Natal , Estudos Prospectivos , Ultrassonografia
7.
Anesth Analg ; 132(4): 1164-1173, 2021 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-33048913

RESUMO

Maternal-fetal surgery is a rapidly evolving specialty, and significant progress has been made over the last 3 decades. A wide range of maternal-fetal interventions are being performed at different stages of pregnancy across multiple fetal therapy centers worldwide, and the anesthetic technique has evolved over the years. The American Society of Anesthesiologists (ASA) recognizes the important role of the anesthesiologist in the multidisciplinary approach to these maternal-fetal interventions and convened a collaborative workgroup with representatives from the ASA Committees of Obstetric and Pediatric Anesthesia and the Board of Directors of the North American Fetal Therapy Network. This consensus statement describes the comprehensive preoperative evaluation, intraoperative anesthetic management, and postoperative care for the different types of maternal-fetal interventions.


Assuntos
Analgesia Obstétrica , Anestesia Obstétrica , Doenças Fetais/cirurgia , Terapias Fetais , Procedimentos Cirúrgicos Obstétricos , Complicações na Gravidez/cirurgia , Analgesia Obstétrica/efeitos adversos , Anestesia Obstétrica/efeitos adversos , Consenso , Feminino , Terapias Fetais/efeitos adversos , Humanos , Procedimentos Cirúrgicos Minimamente Invasivos , Procedimentos Cirúrgicos Obstétricos/efeitos adversos , Complicações Pós-Operatórias/etiologia , Gravidez , Medição de Risco , Fatores de Risco , Resultado do Tratamento
8.
J Pediatr Surg ; 55(6): 1002-1005, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32173119

RESUMO

AIM OF STUDY: Gastroschisis is a congenital abdominal wall defect which results in herniation of abdominal contents. The objective of this study was to determine the maternal risk factors for gastroschisis in Colorado. METHODS: A case-control study was performed using the Birth Registry database from 2007 to 2016. The outcome was gastroschisis, and the main variable was maternal age which was divided into <21, 21-30, and >30 years of age. Descriptive analysis, bivariate analysis, and logistic regression was performed. RESULTS: There were 236 cases of gastroschisis compared to 944 controls. Maternal age did vary significantly between groups (23.4 ±â€¯5 years (cases) vs. 28.7 ±â€¯5.9 years (controls); p < 0.0001). Unadjusted analysis demonstrated that those with young maternal age (<21 years of age) had a 14.14 (95% CI 8.44-23.67) higher odds of gastroschisis compared to those >30 years of age. Independent risk factors for gastroschisis were exposure to prenatal and first trimester cigarettes, prenatal and first trimester alcohol, and chlamydia infection. The odds (4.41, 95% CI 1.36-14.26) of gastroschisis were highest in those with first trimester cigarette exposure and young maternal age (p = 0.03). CONCLUSIONS: Young maternal age, cigarette exposure, alcohol exposure, and chlamydia infection increase the odds of gastroschisis. The interaction between young maternal age and first trimester cigarette exposure significantly increases the odds of gastroschisis. TYPE OF STUDY: Prognosis Study. LEVEL OF EVIDENCE: Level III.


Assuntos
Gastrosquise/epidemiologia , Exposição Materna/estatística & dados numéricos , Adulto , Estudos de Casos e Controles , Fumar Cigarros , Colorado/epidemiologia , Feminino , Humanos , Recém-Nascido , Idade Materna , Sistema de Registros , Fatores de Risco , Adulto Jovem
9.
Clin Case Rep ; 8(1): 18-23, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31998479

RESUMO

A foregut duplication cyst occurring together with both a congenital cystic pulmonary airway malformation and extralobar pulmonary sequestration is an unusual combination. Prenatal ultrasound, MRI, and postnatal CT are helpful for operative planning. Surgical resection is the definitive management for all three anomalies.

10.
Fetal Diagn Ther ; 46(6): 411-414, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31048584

RESUMO

BACKGROUND: In utero repair has become an accepted therapy to decrease the rate of ventriculoperitoneal shunting and improve neurologic function in select cases of myelomeningocele. The Management of Myelomeningocele Study (MOMS) trial excluded patients with a BMI >35 due to concerns for increased maternal complications and preterm delivery, limiting the population that may benefit from this intervention. OBJECTIVES: The aim of this study was to evaluate outcomes associated with extending the maternal BMI criteria to 40 in open fetal repair of myelomeningocele. METHOD: Retrospective review of fetal closure of myelomeningocele at a quaternary referral center between 2013 and 2016 with maternal BMI ranging from 35 to 40. RESULTS: Eleven patients with a BMI >35 were identified. The average BMI was 37. The average maternal age at the time of evaluation was 27 years. The average gestational age at fetal surgery was 24 weeks. Gestational age at birth was an average of 32 weeks. There was one perinatal death immediately following the fetal intervention. The shunt rate at 1 year was 45% (5/11 patients). CONCLUSIONS: In this single-institution review of expanded BMI criteria for fetal repair of myelomeningocele, we did not observe any adverse maternal outcomes associated with maternal obesity; however, the gestational age at delivery was 2 weeks earlier compared to the MOMS trial.


Assuntos
Índice de Massa Corporal , Terapias Fetais/métodos , Saúde Materna , Meningomielocele/cirurgia , Obesidade/diagnóstico , Procedimentos Cirúrgicos Obstétricos , Adulto , Colorado , Feminino , Terapias Fetais/efeitos adversos , Terapias Fetais/mortalidade , Idade Gestacional , Nível de Saúde , Humanos , Meningomielocele/diagnóstico por imagem , Meningomielocele/mortalidade , Obesidade/complicações , Procedimentos Cirúrgicos Obstétricos/efeitos adversos , Procedimentos Cirúrgicos Obstétricos/mortalidade , Morte Perinatal , Complicações Pós-Operatórias/mortalidade , Complicações Pós-Operatórias/cirurgia , Gravidez , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Derivação Ventriculoperitoneal
11.
Fetal Diagn Ther ; 45(5): 339-344, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30157479

RESUMO

INTRODUCTION: The use of perioperative tocolytic agents in fetal surgery is imperative to prevent preterm labor. Indomethacin, a well-known tocolytic agent, can cause ductus arteriosus (DA) constriction. We sought to determine whether a relationship exists between preoperative indomethacin dosing and fetal DA constriction. MATERIALS AND METHODS: This is an IRB-approved, single-center retrospective observational case series of 42 pregnant mothers who underwent open fetal myelomeningocele repair. Preoperatively, mothers received either 1 (QD) or 2 (BID) indomethacin doses. Maternal anesthetic drug exposures and fetal cardiac dysfunction measures were collected from surgical and anesthesia records and intraoperative fetal echocardiography. Pulsatility Index was used to calculate DA constriction severity. Comparative testing between groups was performed using t- and chi-square testing. RESULTS: DA constriction was observed in all fetuses receiving BID indomethacin and in 71.4% of those receiving QD dosing (p = 0.0002). Severe DA constriction was observed only in the BID group (35.7%). QD indomethacin group received more intraoperative magnesium sulfate (p < 0.0001). Minimal fetal cardiac dysfunction (9.5%) and bradycardia (9.5%) were observed in all groups independent of indomethacin dosing. CONCLUSIONS: DA constriction was the most frequent and severe in the BID indomethacin group. QD indomethacin and greater magnesium sulfate dosing was associated with reduced DA constriction.


Assuntos
Anti-Inflamatórios não Esteroides/administração & dosagem , Canal Arterial/cirurgia , Terapias Fetais/métodos , Indometacina/administração & dosagem , Meningomielocele/cirurgia , Tocolíticos/administração & dosagem , Constrição , Relação Dose-Resposta a Droga , Canal Arterial/diagnóstico por imagem , Canal Arterial/efeitos dos fármacos , Feminino , Humanos , Meningomielocele/diagnóstico por imagem , Meningomielocele/tratamento farmacológico , Gravidez , Estudos Retrospectivos , Ultrassonografia Pré-Natal/métodos
12.
Am J Obstet Gynecol MFM ; 1(1): 74-81, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-32832884

RESUMO

Background: Despite improvements in fetal survival for pregnancies affected by twin-twin transfusion syndrome since the introduction of laser photocoagulation, prematurity remains a major source of neonatal morbidity and mortality. Objective: To investigate the indications and factors influencing the timing of delivery following laser treatment, we collected delivery information regarding twin-twin transfusion syndrome cases in a large multicenter cohort. Study Design: Eleven North American Fetal Therapy Network (NAFTNet) centers conducted a retrospective review of twin-twin transfusion syndrome patients who underwent laser photocoagulation. Clinical, demographic and ultrasound variables including twin-twin transfusion syndrome stage, and gestational age at treatment and delivery were recorded. Primary and secondary maternal and fetal indications for delivery were identified. Univariate analysis was used to select candidate variables with significant correlation with latency and GA at delivery. Multivariable Cox regression with competing risk analysis was utilized to determine the independent associations. Results: A total of 847 pregnancies were analyzed. After laser, the average latency to delivery was 10.11 ± 4.8 weeks and the mean gestational age at delivery was 30.7 ± 4.5 weeks. Primary maternal indications for delivery comprised 79% of cases. The leading indications included spontaneous labor (46.8%), premature rupture of membranes (17.1%), and placental abruption (8.4%). Primary fetal indications accounted for 21% of cases and the most frequent indications included donor non-reassuring status (20.5%), abnormal donor Dopplers (15.1%), and donor growth restriction (14.5%). The most common secondary indications for delivery were premature rupture of membranes, spontaneous labor and donor growth restriction. Multivariate modeling found gestational age at diagnosis, stage, history of prior amnioreduction, cerclage, interwin membrane disruption, procedure complications and chorioamniotic membrane separation as predictors for both gestational age at delivery and latency. Conclusion: Premature delivery after laser therapy for twin-twin transfusion syndrome is primarily due to spontaneous labor, preterm premature rupture of membranes and non-reassuring status of the donor fetus. Placental abruption was found to be a frequent complication resulting in early delivery. Future research should be directed toward the goal of prolonging gestation after laser photocoagulation to further reduce morbidity and mortality associated with twin-twin transfusion syndrome.


Assuntos
Terapias Fetais , Transfusão Feto-Fetal , Terapia a Laser , Feminino , Transfusão Feto-Fetal/cirurgia , Fetoscopia , Humanos , Recém-Nascido , Terapia a Laser/efeitos adversos , Placenta , Gravidez , Estudos Retrospectivos , Estados Unidos
13.
BMJ Case Rep ; 20182018 09 15.
Artigo em Inglês | MEDLINE | ID: mdl-30219776

RESUMO

Haemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal disorder. It is challenging to diagnose due to its rarity and variation in clinical presentation, laboratory abnormalities and underlying aetiologies. A reproductive-aged woman, gravida 2 para 1001 at 27 weeks gestation presented with fever, hypotension and subacute upper respiratory infection. She delivered a male infant by caesarean section secondary to fetal distress. Subsequently, she was diagnosed with T-cell lymphoma and secondary HLH. Despite management with supportive care and multiple chemotherapeutic agents, she ultimately died of multiorgan failure. Patients with HLH secondary to malignancy have a particularly poor prognosis. This case highlights the importance of considering secondary HLH in the differential diagnosis of a patient with fever, pancytopenia and systemic symptoms of unclear aetiology in pregnancy.


Assuntos
Linfo-Histiocitose Hemofagocítica/patologia , Linfoma de Células T/patologia , Células T Matadoras Naturais , Complicações Neoplásicas na Gravidez/patologia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Cesárea , Evolução Fatal , Feminino , Idade Gestacional , Humanos , Recém-Nascido , Linfo-Histiocitose Hemofagocítica/complicações , Linfo-Histiocitose Hemofagocítica/diagnóstico por imagem , Linfoma de Células T/complicações , Linfoma de Células T/tratamento farmacológico , Masculino , Gravidez , Falha de Tratamento
14.
Fetal Diagn Ther ; 44(2): 105-111, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-28873371

RESUMO

OBJECTIVE: We reviewed our experience with open fetal surgical myelomeningocele repair to assess the efficacy of a new modification of the hysterotomy closure technique regarding hysterotomy complication rates at the time of cesarean delivery. METHODS: A modification of the standard hysterotomy closure was performed on all patients undergoing prenatal myelomeningocele repair. The closure consisted of an interrupted full-thickness #0 polydioxanone (PDS) retention suture as well as a running #0 PDS suture to re-approximate the myometrial edges, and the modification was a third imbricating layer resulting in serosal-to-serosal apposition. A standard omental patch was placed per our routine. Both operative reports and verbal descriptions of hysterotomy from delivering obstetricians were reviewed. RESULTS: A total of 49 patients underwent prenatal repair of myelomeningocele, 43 having adequate follow-up for evaluation. Of those, 95.4% had completely intact hysterotomy closures, with only 1 partial dehiscence (2.3%) and 1 thinned scar (2.3%). There were no instances of uterine rupture. DISCUSSION: In patients undergoing this modified hysterotomy closure technique, a much lower than expected complication rate was observed. This simple modified closure technique may improve hysterotomy healing and reduce obstetric morbidity.


Assuntos
Fetoscopia/métodos , Histerotomia/métodos , Meningomielocele/diagnóstico , Meningomielocele/cirurgia , Cuidado Pré-Natal/métodos , Adulto , Feminino , Seguimentos , Humanos , Gravidez , Estudos Retrospectivos
15.
Fetal Diagn Ther ; 44(1): 10-17, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-28700992

RESUMO

OBJECTIVE: The aim of this study was to define the natural history of lower urinary tract obstruction (LUTO) with normal midgestational amniotic fluid volumes. MATERIALS AND METHODS: We performed a retrospective review of 32 consecutive patients with LUTO with normal midgestational amniotic fluid volume followed at 11 North American Fetal Therapy Network (NAFTNet) centers from August 2007 to May 2012. Normal amniotic fluid volume was defined as an amniotic fluid index (AFI) of ≥9 cm. RESULTS: The mean gestational age (GA) and AFI at enrollment were 23.1 ± 2.1 weeks and 15.8 ± 3.9 cm, respectively. The mean GA at delivery was 37.3 ± 2.8 weeks. The mean creatinine level at discharge was 1.2 ± 0.8 mg/dL. Perinatal survival was 97%. Twenty-five patients returned for serial postnatal assessment. Renal replacement therapy (RRT) was required in 32%. Development of oligohydramnios and/or anhydramnios, development of cortical renal cysts, posterior urethral valves, prematurity, and prolonged neonatal intensive care unit stay were associated with need for RRT (p < 0.05) by univariate analysis. By multivariate analysis, preterm delivery remained predictive of need for RRT (p = 0.004). CONCLUSION: Prenatal diagnosis of LUTO with normal midgestational amniotic fluid volumes is associated with acceptable renal function in the majority of patients. Approximately one-third of these children require RRT. Surrogate markers of disease severity appear to be predictive of need for RRT.


Assuntos
Obstrução Uretral/epidemiologia , Líquido Amniótico , Feminino , Humanos , Recém-Nascido , Masculino , América do Norte/epidemiologia , Gravidez , Sistema de Registros , Estudos Retrospectivos , Ultrassonografia Pré-Natal , Obstrução Uretral/diagnóstico por imagem
16.
Prenat Diagn ; 35(6): 564-70, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-25663164

RESUMO

OBJECTIVE: The objective of this study is to perform a longitudinal evaluation of blood flow patterns in the ductus arteriosus (DA) during the perioperative period in fetal myelomeningocele (MMC) surgical patients. METHOD: Serial fetal echocardiograms were reviewed in 10 MMC cases where mothers received indomethacin and intravenous and inhaled anesthesia. One-way analysis of variance was utilized to evaluate for differences in peak systolic velocity, end-diastolic velocity (EDV), time-averaged mean velocity (TAMV), and Pulsatility Index (PI) throughout the monitoring period. Regression analysis was performed to evaluate the relationship between PI and maternal hemodynamics and medications. RESULTS: The DA TAMV and EDV increased between baseline and inhaled anesthesia and decreased between inhaled anesthesia and postoperative day 2. PI decreased to a nadir during inhaled anesthesia and then increased through postoperative day 2. Three distinct ductal flow patterns, characterizing degree of ductal constriction, were observed. Two fetuses exhibited a severely constricted ductal flow pattern with concurrent moderate tricuspid insufficiency and right ventricular dysfunction during inhaled anesthesia. CONCLUSION: Abnormal DA flow patterns culminating in significant DA constriction occurred during fetal MMC repair. Limiting maternal exposure to indomethacin, supplemental oxygen, and inhaled anesthesia may reduce the incidence and severity of DA constriction and perhaps reduce fetal cardiac dysfunction during open fetal surgery.


Assuntos
Anestesia Geral , Anti-Inflamatórios não Esteroides/uso terapêutico , Canal Arterial/diagnóstico por imagem , Terapias Fetais , Indometacina/uso terapêutico , Meningomielocele/cirurgia , Período Perioperatório , Fluxo Pulsátil , Anestesia por Inalação , Anestesia Intravenosa , Velocidade do Fluxo Sanguíneo , Estudos de Coortes , Ecocardiografia , Feminino , Hemodinâmica , Humanos , Fluxometria por Laser-Doppler , Estudos Longitudinais , Oxigenoterapia , Gravidez , Estudos Retrospectivos , Fatores de Risco
17.
Am J Perinatol ; 31 Suppl 1: S39-46, 2014 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-25077476

RESUMO

The underlying etiology of twin-twin transfusion syndrome (TTTS) is unknown, but our growing understanding of the cardiovascular features of TTTS suggests this may be a disease that could respond to transplacental medical therapy. Adjunctive medical therapy in TTTS with the calcium channel blocker nifedipine has been shown to improve recipient survival while having no effect on the donor. There is no significant difference in recipient survival from postoperative day 5 to birth suggesting that the survival benefit is confined to the effects of nifedipine in the perioperative period. Also, there is no significant effect of nifedipine on gestational age at delivery suggesting the survival benefit was unrelated to the tocolytic effects of nifedipine and more likely a result of hemodynamic effects in the recipient twins' cardiovascular system during the perioperative period. TTTS remains poorly understood but there appears to be good evidence suggesting twin-twin hypertensive cardiomyopathy is a large component of the pathophysiology in recipient twins. The initial findings of nifedipine's effectiveness as a targeted medical therapy to address TTTS cardiomyopathy and improve survival of recipient twins opens the door for further research for adjunctive medical therapies in TTTS.


Assuntos
Transfusão Feto-Fetal/cirurgia , Fetoscopia , Bloqueadores dos Canais de Cálcio/uso terapêutico , Cardiomiopatias/complicações , Cardiomiopatias/fisiopatologia , Feminino , Transfusão Feto-Fetal/complicações , Transfusão Feto-Fetal/tratamento farmacológico , Transfusão Feto-Fetal/fisiopatologia , Humanos , Nifedipino/uso terapêutico , Gravidez , Disfunção Ventricular
18.
Am J Obstet Gynecol ; 191(6): 2148-53, 2004 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-15592305

RESUMO

OBJECTIVE: The purpose of this study was to compare the relationship between the actual amnionic fluid volume that was measured at delivery and magnetic resonance amnionic fluid volume determination, largest vertical pocket, and amnionic fluid index. STUDY DESIGN: Three hours before cesarean delivery, 80 women had sonographic measurement of the amnionic fluid index and the largest vertical pocket. Magnetic resonance imaging was then completed, and the magnetic resonance amnionic fluid volume was determined. At surgery, the amnionic fluid was collected. Pearson correlations were determined. Receiver operating characteristic curves were developed for each method as a measure of predictability for oligohydramnios. RESULTS: The correlations for the magnetic resonance amnionic fluid volume, amnionic fluid index, and largest vertical pocket to amnionic fluid volume was 0.84, 0.77, and 0.71, respectively. Magnetic resonance amnionic fluid volume has a statistically higher correlation than the largest vertical pocket ( P = .046). The 3 methods, however, are statistically comparable for identifying oligohydramnios. CONCLUSION: Magnetic resonance imaging is comparable with ultrasound evaluation for the prediction of oligohydramnios. Correlations to actual amnionic fluid volume are also comparable.


Assuntos
Imageamento por Ressonância Magnética , Oligo-Hidrâmnio/diagnóstico , Ultrassonografia Pré-Natal , Adulto , Líquido Amniótico/metabolismo , Cesárea , Intervalos de Confiança , Feminino , Sofrimento Fetal/diagnóstico , Idade Gestacional , Humanos , Gravidez , Resultado da Gravidez , Cuidados Pré-Operatórios/métodos , Curva ROC , Estudos de Amostragem , Sensibilidade e Especificidade
19.
Obstet Gynecol ; 103(3): 546-50, 2004 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-14990420

RESUMO

OBJECTIVE: The purpose of this study was to determine whether the placental transfer of interleukin (IL)-1alpha, IL-6, and tumor necrosis factor-alpha (TNF-alpha) occurs. METHODS: Four normal-term placentas were perfused for maternal-fetal transfer of the cytokines, 2 placentas for fetal-maternal transfer, and 4 additional placentas were used for an endogenous control. The ex vivo isolated cotyledon human placental perfusion model was used. The reference compound antipyrine was used to determine the transport fraction and clearance index of the cytokines. The cytokines were added to either the maternal or fetal circulations, and samples were collected for 1 hour in a constant-flow open circulation. Cytokine levels were compared between the study and control placentas. Concentrations of the cytokines were measured by sandwich enzyme immunoassay. RESULTS: The clearance index for the maternal-fetal transfer of IL-1alpha and TNF-alpha was 0.001, suggesting minimal transfer to the fetal circulation. The clearance index for IL-6 was 0.30, indicating transfer to the fetal circulation. When the cytokines were added to the fetal circulation, the clearance index for IL-1alpha was 0.001, again indicating minimal transfer. The clearance index for TNF-alpha in the fetal-maternal study was not determined. IL-6 had a clearance index of 0.23, which was similar to that observed with maternal-fetal transfer. IL-6 concentrations in the study placentas were higher than the concentrations found in the controls. CONCLUSION: There appears to be bidirectional transfer of IL-6 in the healthy-term human placental perfusion model. LEVEL OF EVIDENCE: II-2


Assuntos
Interleucina-1/metabolismo , Interleucina-6/metabolismo , Troca Materno-Fetal/fisiologia , Placenta/metabolismo , Circulação Placentária/fisiologia , Fator de Necrose Tumoral alfa/metabolismo , Técnicas de Cultura , Feminino , Humanos , Gravidez , Valores de Referência , Fatores de Tempo
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