围产期婴儿with Congenital Abnormalities

在美国的33个婴儿中大约是一个出生的缺陷。其中最常见的是天空管间隔缺损，脊柱裂等缺陷和肠闭锁或狭窄。¹在常规超声成像期间怀孕早期检测到许多重大缺陷。“对于具有复杂怀孕的女性，通常需要多学科团队来管理母婴的需求，以管理在整个怀孕，交付和产后时期的母婴，”解释Jaimey M. Pauli, M.D.，母形医学专家，Penn State Hershey Obstetrics and Gynecology。Dr. Pauli andThomas Chin, M.D., chief, pediatric cardiology, are co-directors of the Penn State Hershey Perinatal Program atPenn State Hershey Medical Centerand theChildren’s Hospital, an active outreach program for pregnant women at high risk or patients who have newborns with birth defects or abnormalities.

“Expectant parents are often overwhelmed when they learn about these types of serious fetal abnormalities. Aside from help coping with the obvious emotional impact, they need help obtaining the complex care their baby requires. With our program, a team of specialists handles everything and provides highly coordinated care at a single center, which reduces a lot of stress and supports the parents,” adds Dr. Pauli. Through the perinatal program, Drs. Pauli and Chin assemble a team of maternal-fetal medicine specialists and pediatric specialists. This team can include pediatric cardiologists, cardiothoracic surgeons, neonatologists, neurosurgeons, pediatric surgeons, nephrologists, urologists, orthopaedic surgeons, radiologists and social workers, based on the needs of each individual infant and aimed at achieving the best possible outcomes.

当常规从另一个机构诊断患有胎儿异常的患者在常规解剖超声期间妊娠期妊娠的18-20周诊断患者时，围一的围困计划(see overview of how one case has been handled from referral to discharge)。Perinatal计划协调员Laurie Reesman，RN，BSN，安排宾夕法尼亚州Hershey母性胎儿医学和儿科专家的约会，以提供必要的诊断评估，家长咨询，产前护理，交付和产后护理。

Dr. Pauli reports that there are typically 10 new cases presented to the program each month. The perinatal program team of specialists meets on a monthly basis to discuss each case and ensure the patient has been seen by all of the necessary specialists in one location. Dr. Pauli meets weekly with the coordinators to review each patient’s plan of care.

Postpartum, mothers receive care with the maternal-fetal medicine service while the neonate becomes the focus of medical interventions. Most newborns in the program require surgery, such as complex congenital heart disease repair, closure of open neural tube defects and repair of abdominal wall defects, such as gastroschisis and omphalocele. These patients require the level of care provided in the neonatal intensive care unit (NICU) and the pediatric intensive care unit (PICU) of Penn State Hershey Children’s Hospital.

围产期计划还安排了非医疗保健和支持。在宾夕法尼亚州邦特科的Isabel Cate Rineer纪念捐赠在复杂的怀孕期间支持家庭的身体和情感需求。此外，由于这些家庭希望通过这次接近他们的新生儿，因此家庭成员在罗纳德麦克唐纳州的街对面，从宾夕法尼亚州Hershey Campus的街道上直接提供住宿。对于在住院住宿后需要复杂和持续支持的新生儿，家庭与社会工作者合作，帮助在当地社区中协调持续的医疗，并在宾夕法尼亚州立州立州立州儿童医院进行后续支持。对于患有生命危及生命情况的婴儿，没有预期存活，患者和家庭都得到了姑息，终身保健的支持Penn State Hershey’s Hummingbird Program。

“我们为孕妇母亲和婴儿提供了最高的家庭中心提供了一个家庭为中心的环境，”博士博士。“我们的家庭有助于整个经验都非常受欢迎和知情。他们保证，宾夕法尼亚州立州立州立州立州立州立州立州立州立州立州立州立州的高风险怀孕以及婴儿的医疗和手术需求的专业知识。“

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Jaimey Maureen Pauli, M.D.
母胎医学专家，宾夕法尼亚州Hershey妇产科和妇科
PHONE:717-531-7373
电子邮件：jpauli@hmc.psu.edu
FELLOWSHIP:Maternal-fetal medicine, Penn State Milton S. Hershey
Medical Center, Hershey, Pa.
RESIDENCY:OBGYN, Penn State Milton S. Hershey Medical Center, Hershey, Pa.
医学院：Penn State College of Medicine, Hershey, Pa.

与jaimey pauli，m.d. on doximity联系

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REFERENCE:

1. United States Centers for Disease Control:cdc.gov/ncbddd/birthdefects/data.html; accessed 8/5/2015.

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案例摘要：Penn State Hershey PerinataL计划处理的Hypoplastic左心综合征案例概述

1. 检测和转诊：A 32-year-old woman is referred to the perinatal program following anatomic ultrasound at 19 weeks gestation revealing complex cardiac defects in the fetus.
2. Initial evaluation:随访的胎儿超声心动图和额外的评估揭示了假血管左心综合征;母亲身体健康;三年前，一个先前健康，全年，阴道出生的历史。
3. 护理协调：Perinatal program coordinator arranges parent consultations and evaluations at Penn State Hershey Medical Center on a one- or two-day schedule with the following specialists and care team members: Neonatologist, Pediatric cardiothoracic surgeon, Maternal-fetal medicine specialist, Social services, Genetics consult.
4. Routine visits:Prenatal care visits with maternal-fetal medicine; serial fetal ultrasound evaluation and fetal monitoring.
5. 送货：Successful full-term vaginal delivery at 37 weeks; infant is successfully stabilized in the NICU.
6. Perinatal evaluation:Infant undergoes echocardiography and is evaluated by pediatric cardiac surgery team.
7. Perinatal intervention:Infant undergoes the Norwood procedure as the first step in surgical cardiac repair two days after birth; parents stay at Ronald McDonald House; infant is in PICU for 36 days.
8. Discharge and ongoing support:Infant is discharged to home, with continuing supportive care from local providers and followed by Penn State Hershey pediatric cardiothoracic surgeons. The infant then undergoes a series of staged pediatric cardiac surgeries in the first several years of life.