Prenatal diagnosis and the pediatric surgeon: The impact of prenatal consultation on perinatal management

doi:10.1016/S0022-3468(96)90340-1

Journal of Pediatric Surgery

Volume 31, Issue 1, January 1996, Pages 156-163

https://doi.org/10.1016/S0022-3468(96)90340-1 Get rights and content

Abstract

Purpose: Pediatric surgeons are increasingly called on by obstetrical colleagues to counsel parents about the implications of a prenatal ultrasound finding. Our understanding of the natural history of many prenatally diagnosed surgical conditions has grown significantly in recent years. Whether prenatal surgical consultation can influence perinatal course had not been investigated. Methods: During an 21-month period, 12,865 prenatal ultrasound studies were performed on a total of 4,551 patients, and 221 prenatal surgical consultations were obtained through a newly established fetal treatment program at a tertiary care prenatal diagnostic center. To evaluate the impact of prenatal pediatric surgical consultation on perinatal course, the authors reviewed changes in management including termination of pregnancy, in utero intervention, and altered site, mode, or timing of delivery. Results: Two hundred twenty-one fetuses were referred for consultation; their 234 congenital anomalies included genitourinary (36%), thoracic (16%), intraabdominal (14.5%), abdominal wall (10.6%), neurological (9%), skeletal (6%), and head and neck (2.5%) defects; 2.5% had tumors and 2.5% were twin pregnancies. Pregnancy was terminated in 9.5% of cases, because of patient request, chromosomal abnormality, or dismal prognosis. In 3.6%, the decision to terminate was changed as a result of consultation. Site of delivery was changed as a result of consultation in 37% to facilitate postnatal evaluation and initiate immediate treatment. Mode of delivery was changed in 6.8% to prevent dystocia, hemorrhage into a tumor, as in sacrococcygeal teratoma, or to provide an emergency airway, as in cervical teratoma. The timing of delivery was changed in 4.5% to avoid further damage to fetal organs in cases of obstructive uropathy, gastroschisis, sacrococcygeal teratoma with high-output failure, and hydrocephalus. Five percent (11) underwent treatment in utero for fetal hydrothorax, obstructive uropathy, twin-twin transfusion syndrome, or lymphangioma. The overall perinatal mortality rate was 2.5%. Conclusion: Prenatal pediatric surgical consultation may have a significant impact on the perinatal management of the fetus with a surgically correctable congenital anomaly. Providing obstetric colleagues and families with valuable insight into the surgical management of anomalies allows fetal intervention when appropriate, and delivery in an appropriate setting, by the safest mode of delivery, and at the gestational age appropriate to minimize effects of the anomaly.

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The involvement of pediatric surgeons during multi-disciplinary prenatal meetings has been an important part of the evolution of comprehensive fetal care, characterized by detailed prenatal evaluation, diagnosis, prognosis, and planned perinatal and post-natal care. For certain diagnoses, such as congenital diaphragmatic hernia (CDH), protocol-based prenatal and post-natal care has led to improved neonatal outcomes, even in cases once considered non-survivable.1,2 Fetal diagnostics and treatments have always been both medically and ethically complex.
Pediatric surgeons play an essential role in prenatal consultation for congenital anomalies likely to require surgery in the newborn period. The involvement of pediatric surgeons during multi-disciplinary prenatal meetings has been an important part of the evolution of comprehensive fetal care, characterized by detailed prenatal evaluation, diagnosis, prognosis, and planned perinatal and post-natal care. Advances in fetal diagnostics and treatments, as well as complex postnatal medical care and decision-making create a broad range of care options for pregnant women with fetal surgical anomalies. Ethical challenges involve the availability and risks/benefits of maternal-fetal surgery, and diagnostic and prognostic uncertainty for the newborn. Clinical scenarios illustrate cases that pediatric surgeons may encounter in practice, with discussions highlighting the ethical principles involved as well as considerations for management.
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Knowledge of an intra-abdominal cyst's likely evolution can inform plans for antenatal follow-up and postnatal management, optimising the site of delivery and ensuring prompt perinatal review. Furthermore, it is important that prospective parents are counselled with regard to the expected outcome and the possibility of surgical intervention after birth [11]. Further research detailing the predictive value of antenatal USS may provide valuable data to inform prognostic counselling.
The outcome of antenatally detected fetal intra-abdominal cysts is highly variable and challenging to predict. Antenatal ultrasound scans may be of value in predicting postnatal outcome.
To report antenatal and postnatal course of fetal intra-abdominal cysts identified on antenatal ultrasound and establish the value of cyst dimensions for predicting outcome.
Retrospective observational study.
All intra-abdominal cysts diagnosed in a single centre between 2013 and 2019.
Cyst characteristics were recorded from radiological reports and postnatal diagnosis and outcomes documented. Growth characteristics were identified to distinguish different diagnosis. The maximum antenatal diameter of all cysts was identified and the best cut-off diameter to predict whether a cyst would persist postnatally or require surgery was identified. Best cut-off values were identified using Youden index.
Of the 38 cysts identified on antenatal ultrasound, 24 (63%) persisted postnatally, 8 required surgery (21%) and 4 (11%) were not considered an intra-abdominal cyst postnatally. Ovarian cyst and duplication cyst may have different growth characteristics. In the prediction of cysts persisting postnatally, the area under the ROC curve (AUC) was 0.81 (95% CI, 0.66–0.95). Two cut off values were identified, 37.5 mm (50% sensitivity, specificity 100%, Youden's index 0.50) and 28.5 mm (sensitivity 65%, specificity 85%, Youden's index 0.50). In the prediction of surgery, no optimal diameter was found; AUC was 0.57 (95% CI, 0.34–0.81).
Maximum antenatal cyst diameter is useful for the prediction of whether a cyst will persist postnatally but not for the prediction of surgery.
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This allows obstetric, neonatal and subspecialty teams to better anticipate the potential interventions that may be required and customize approaches to resuscitation. If DR emergencies are anticipated, transfer of care and delivery at a hospital with high-risk MFM providers, advanced neonatal providers and subspecialty availability is recommended [30–32]. Inclusion of pediatric sub-specialists in the discussion of potential impact on the fetus can provide a holistic view of interventions required, risk of mortality, lifetime morbidities, and potential impact on the family [33].
Effective communication is the keystone in the management of delivery room emergencies. A clearly defined framework and “shared mental model” optimize team collaboration and are essential when anticipating and preparing for high-stakes emergent cases. This review defines a framework to build shared mental models using dialogue, pre-briefing, checklists, simulation, debriefing and structured feedback to maintain a cohesive high functioning team. Strategies to approach different emergency lead-times will be discussed, and case examples will be used to illustrate concepts outlined.
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Pediatric surgeons frequently offer prenatal consultation for congenital pulmonary airway malformation (CPAM) and congenital diaphragmatic hernia (CDH); however, there is no evidence-based consensus to guide prenatal decision making and counseling for these conditions. Eliciting feedback from experts is integral to defining best practice regarding prenatal counseling and intervention.
A Delphi consensus process was undertaken using a panel of pediatric surgeons identified as experts in fetal therapy to address current limitations. Areas of discrepancy in the literature on CPAM and CDH were identified and used to generate a list of content and intervention questions. Experts were invited to participate in an online Delphi survey. Items that did not reach first-round consensus were broken down into additional questions, and consensus was achieved in the second round.
Fifty-four surgeons (69%) responded to at least one of the two survey rounds. During round one, consensus was reached on 54 of 89 survey questions (61%), and 45 new questions were developed. During round two, consensus was reached on 53 of 60 survey questions (88%).
We determined expert consensus to establish guidelines regarding perinatal management of CPAM and CDH. Our results can help educate pediatric surgeons participating in perinatal care of these patients.
V.
Assessing quality of life in pediatric gastroschisis patients using the Pediatric Quality of Life Inventory survey: An institutional study
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The purpose of this study was to quantify quality of life (QOL) outcomes in gastroschisis children using a validated QOL inventory survey.
A chart review and prospective survey (2012 Pediatric Quality of Life Inventory™ (PedsQL™)) were performed for gastroschisis patients from 2005 to 2011. Demographics and scores were compared between patents with simple versus complicated gastroschisis and patients with and without bowel resection.
One hundred nineteen patients (> 2 years of age) were identified. Twenty-eight families participated in the prospective survey with an average patient age of 5.8 ± 2.3 years. There were 11 complicated and 17 simple cases. Children with simple gastroschisis had lower rates of reoperation (0.06% versus 90%, p < 0.001) and bowel resection (12% and 64%, p = 0.004) than complicated patients. Average QOL scores for children with simple and complicated gastroschisis were 81.69/100 ± 19.50 and 78.7/100 ± 20.14 (p = 0.70), respectively. Scores were also similar in children with and without bowel resection (74.72/100 ± 19.94 and 83.29/100 ± 19.10, p = 0.171). Cronbach's alpha correlation was 0.912 for the overall survey.
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^☆: Presented at the 26th Annual Meeting of the American Pediatric Surgical Association, Boca Raton, Florida, May 20–23, 1995.

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Paper presented at the 26th Annual Meeting of the American Pediatric Surgical Association Boca Raton, Florida, May 20–23, 1995Prenatal diagnosis and the pediatric surgeon: The impact of prenatal consultation on perinatal management☆

Abstract

J Pediatr Surg

Am J Surg

Lancet

J Pediatr Surg

J Pediatr Surg

Prenatal diagnosis

N Engl J Med

Diagnostic ultrasound in pregnancy: An overview

Semin Perinatol

Contribution of birth defects to infant mortality—United States, 1986

MMWR Morb Mort Wkly Rep

Frequency and load of congenital anomalies in a neonatal intensive care unit, prenatal diagnosis, and perinatal management

Semin Perinatol

Invasive fetal therapy: Current status and future directions

Semin Perinatol

Paper presented at the 26th Annual Meeting of the American Pediatric Surgical Association Boca Raton, Florida, May 20–23, 1995
Prenatal diagnosis and the pediatric surgeon: The impact of prenatal consultation on perinatal management☆