Polyhydramnios Secondary to Esophageal Atresia – Cervical Cerclage and Serial Amnioreduction to Prolong Gestational Age in Select Cases (2024)

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  • J Indian Assoc Pediatr Surg
  • v.29(3); May-Jun 2024
  • PMC11192265

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Polyhydramnios Secondary to Esophageal Atresia – Cervical Cerclage and Serial Amnioreduction to Prolong Gestational Age in Select Cases (1)

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J Indian Assoc Pediatr Surg. 2024 May-Jun; 29(3): 292–294.

Published online 2024 May 8. doi:10.4103/jiaps.jiaps_259_23

PMCID: PMC11192265

Samantha Baey, Qi Toh, Li Wei Chiang, George S. H. Yeo,1 Bin Huey Quek,2 and Narasimhan Kannan Laksmi

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ABSTRACT

Esophageal atresia (OA) with or without tracheoesophageal fistula affects approximately 1 in 4000 births and commonly presents with polyhydramnios. This appears to be the first report regarding the utility of cervical cerclage with serial amnioreduction to prolong the gestational age of a neonate with OA, thereby improving outcomes for reconstructive surgery.

KEYWORDS: Amnioreduction, cervical cerclage, esophageal atresia, polyhydramnios

INTRODUCTION

Esophageal atresia (OA) with or without tracheoesophageal fistula (TOF) affects approximately 1 in 4000 births. Polyhydramnios has been observed in approximately 95% of neonates with pure OA and 35% of neonates with distal TOF. It is associated with preterm labor among other complications. This case study reviews the utility of cervical cerclage with serial amnioreduction to prolong the gestational age (GA) of a neonate with OA by 8 weeks and 1.37 kg weight increment. This translated into good neonatal outcomes after successful reconstructive surgery. This seems to be the first such effort.

CASE REPORT

A well 35-year-old primiparous female presented with ultrasound findings of polyhydramnios at GA of 29 weeks. Her pregnancy was booked early and noninvasive prenatal testing was low risk. Her fetal anomaly scan at GA 20 weeks was reported normal; however, at GA 29 weeks, transabdominal ultrasound detected polyhydramnios and an absent fetal stomach bubble raising suspicion for OA. The estimated fetal weight then was 1196 g and cervical length was shortened to 1.78 cm. The mother was asymptomatic aside from abdominal fullness with no other symptoms of threatened pregnancy. Amniocentesis was performed and DNA extraction results had no clinical significance. The consulting obstetrician recommended cervical cerclage with amnioreduction to prolong gestation. The mother was counseled regarding associated risks and underwent an operation uneventfully. Intraoperatively, cervical length was 1 cm, confirming initial ultrasound findings. The mother subsequently underwent serial ultrasonography and a total of four amnioreductions at two weekly intervals with fluid removal of 2L–2.5 L each time [Table 1]. The case was discussed with a multidisciplinary board and consensus was made for term delivery with pediatric surgery and neonatal team on standby. She underwent elective cesarean section and cerclage removal at GA 37 weeks and 3 days.

Table 1

Serial amnioreduction and interval ultrasound imaging

GA (weeks)AmnioreductionPreprocedural ultrasoundPostprocedural ultrasound
292.5 L fluid removedBreech, placenta anterior and high with an AFI of 18.7 cm, an EFW of 1196 g and normal Dopplers. The fetus stomach bubble was not seen. The cervical length was shorted to 1.78 cm with a Y-shaped proximal endAt GA 30 weeks, the fetus EFW 1378 g, AFI 10.9 cm with a cervical length of 3.3 cm
322.2 L fluid removedAFI 20 cm, DVP 10 cm, cervical length 3 cm
342 L fluid removedAFI 20.5 cm, DVP 9.4 cm, cervical length 2.7 cmPostprocedure AFI 11.3 cm, EFW 2113 g
362.5 L fluid removedEFW 2404 g, AFI 21.7 cm, DVP 9.8, cervical length 2.4 cmPostprocedure DVP 7.3 cm

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GA: Gestational age, AFI: Amniotic fluid index, EFW: Estimated fetal weight, DVP: Deepest vertical pocket, GA: Gestational age

The neonate was born at 2.66 kg with an Apgar score of 9 and 9 at the 1st and 5th min, respectively. A nasogastric tube was unable to advance beyond the T2 vertebral level and an X-ray showed distal bowel gas, indicative of Type C TOF [Figure 1]. A Replogle tube was inserted for upper pouch suction and the neonate was transferred to the intensive care unit (ICU). VACTERL screening detected a large patent ductus arteriosus, and moderate fenestrated atrial septal defect but no other fetal abnormalities.

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Figure 1

X-ray depicting nasogastric tube at T2 vertebral level

On day 2 of life, the neonate underwent primary repair of the Type C TOF. Bronchoscopy with size-8 Parsons laryngoscope discovered a large fistula opening at the carina [Figure 2], mild tracheomalacia and no laryngeal cleft. On thoracotomy, the distal TOF was identified, ligated, and the tracheal defect closed. The initial gap between the upper pouch and distal TOF was 4 cm. Careful mobilization of the above structures was performed and primary single anastomosis was done. Postoperatively, the neonate was monitored in ICU and an esophagogram 6 days later showed a patent anastomosis with no leak. The patient was discharged well on day 17 of life after establishing oral feeds.

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Figure 2

Bronchoscopy depicting large fistula opening at the carina

DISCUSSION

Antenatal diagnosis and timely treatment for polyhydramnios are crucial to optimize outcomes for neonates with OA. About 77.8% of neonates with OA present with polyhydramnios on antenatal ultrasound. This is occasionally associated with a small or absent stomach bubble (22.2% and 25.9%, respectively).[1] Polyhydramnios can result from maternal diabetes, infection, and fetal anomalies among others.[2] Fetal gut atresia, specifically OA, has been linked to polyhydramnios due to impaired fetal swallowing and fluid absorption affecting the regulation of amniotic fluid volume. If severe, polyhydramnios can lead to premature rupture of membranes, abnormal fetal lie, and increased rates of prematurity and perinatal mortality.[2]

On presentation, the mother although asymptomatic was at high risk for preterm delivery. Based on the consulting obstetrician’s clinical judgment and counseling with parents, a decision was made for a two-step operation; cervical cerclage and amnioreduction. In view of a fetal anomaly of OA, the aim was to optimize fetal growth and lung maturity for definitive surgery as prematurity is associated with increased cardiovascular and respiratory risks while very low birth weight is associated with higher postoperative complication rates as well as making surgery technically difficult.

In the literature, there is no consensus for the ideal timing of cerclage and it is usually performed between 12 and 28 weeks. Meta-analysis has not revealed a significant reduction of preterm birth for cerclage done after 24 weeks.[3] In addition, risks of cervical cerclage include infection, rupture of membranes, surgical site lacerations, and anesthesia-related complications. However, given the high risk of neonatal morbidity if born premature, after careful consideration of risks and benefits, the team together with the parents, decided to proceed with cerclage to optimize the likelihood of prolonging GA.

Amnioreduction is classically performed for symptomatic severe polyhydramnios with amniotic fluid index ≥35 cm or single deepest pocket ≥16 cm and has shown benefits in providing maternal symptomatic relief.[2] Some studies have also demonstrated benefits in prolonging gestation.[4] Associated complications include preterm rupture of membranes, preterm delivery, placenta abruption, and chorioamnionitis; however, these risks are low in singleton pregnancies.

Amniocentesis should be considered as neonates with OA are associated with VACTERL, CHARGE, DiGeorge syndrome, and trisomies 13, 18, and 21 among others. The diagnosis of the above syndromes should be considered when counseling parents for invasive procedures given a guarded prognosis.

A 2019 case series by Zhang et al. documented eight patients who underwent amnioreduction as an adjunct to cervical cerclage for cervical incompetence with benefits in prolonging GA.[5] With fetal OA, polyhydramnios will likely to recur hence close monitoring with repeated amnioreductions may prove beneficial. In our experience, both interventions were integral in prolonging gestation, thereby optimizing conditions for successful reconstructive surgery.

CONCLUSIONS

Antenatal management of OA is not often discussed as antenatal diagnosis is difficult. However, in selected cases of neonates with OA presenting with polyhydramnios and threatened preterm delivery, cervical cerclage with repeated amnioreduction may be a safe and efficacious treatment in prolonging GA, improving outcomes for postnatal reconstructive surgery. More research should be done to establish efficacy.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Acknowledgement

The contribution of the following individuals is sincerely appreciated. Dr June Tan Vic Khi and Dr Anju Bhatia were responsible for assisting fetal procedures, collecting antenatal data and the antenatal follow up. They provided valuable data points for the above write up and are gratefully acknowledged.

REFERENCES

1. Chang CS, Choi Y, Kim SY, Yee C, Kim M, Sung JH, et al. Prenatal ultrasonographic findings of esophageal atresia: Potential diagnostic role of the stomach shape. Obstet Gynecol Sci. 2021;64:42–51. [PMC free article] [PubMed] [Google Scholar]

2. Hamza A, Herr D, Solomayer EF, Meyberg-Solomayer G. Polyhydramnios: Causes, diagnosis and therapy. Geburtshilfe Frauenheilkd. 2013;73:1241–6. [PMC free article] [PubMed] [Google Scholar]

3. Gulersen M, Lenchner E, Nicolaides KH, Otsuki K, Rust OA, Althuisius S, et al. Cervical cerclage for short cervix at 24 to 26 weeks of gestation: Systematic review and meta-analysis of randomized controlled trials using individual patient-level data. Am J Obstet Gynecol MFM. 2023;5:100930. [PubMed] [Google Scholar]

4. Dickinson JE, Tjioe YY, Jude E, Kirk D, Franke M, Nathan E. Amnioreduction in the management of polyhydramnios complicating singleton pregnancies. Am J Obstet Gynecol. 2014;211:e1–7. [PubMed] [Google Scholar]

5. Zhang Y, Han Z, Gao Q, Bai X, Hou H. Amnioreduction in emergency cervical cerclage: A series of eight cases. Int J Gynaecol Obstet. 2020;150:416–7. [PubMed] [Google Scholar]

Articles from Journal of Indian Association of Pediatric Surgeons are provided here courtesy of Wolters Kluwer -- Medknow Publications

Polyhydramnios Secondary to Esophageal Atresia – Cervical Cerclage and Serial Amnioreduction to Prolong Gestational Age in Select Cases (2024)

FAQs

What is the relationship between polyhydramnios and esophageal atresia? ›

Polyhydramnios is present in approximately one third of pregnant women carrying fetuses with esophageal atresia because the fetus is unable to swallow amniotic fluid. Within hours after birth, these infants accumulate large amounts of oral secretions, which may precipitate coughing, choking, and respiratory distress.

How does TEF cause polyhydramnios? ›

Prenatal Diagnosis of TEF/EA:

Normally, a baby in the womb will swallow and breathe in the amniotic fluid. If the fistula is so narrow or restrictied in flow that it does not allow for this, the amniotic fluid will build up. This excessive amount of amniotic fluid is called polyhydramnios.

What is the cause of esophageal atresia? ›

The exact causes of esophageal atresia and tracheoesophageal fistula are unknown. There could be a genetic component, but it is not necessarily inheritable. Up to half of all babies with esophageal atresia or tracheoesophageal fistula have at least one other birth defect, and often several.

What happens if a baby is not swallowing the amniotic fluid? ›

Polyhydramnios can occur if the baby does not swallow and absorb amniotic fluid in normal amounts. This can happen if the baby has certain health problems, including: Gastrointestinal disorders, such as duodenal atresia, esophageal atresia, gastroschisis, and diaphragmatic hernia.

What birth defects are caused by polyhydramnios? ›

Gastrointestinal disorders, such as duodenal atresia, esophageal atresia, gastroschisis, and diaphragmatic hernia. Brain and nervous system problems, such as anencephaly and myotonic dystrophy. Achondroplasia. Beckwith-Wiedemann syndrome.

Which fetal abnormality is most likely responsible for the polyhydramnios? ›

What is the most common cause of polyhydramnios?
  • The fetus can't swallow amniotic fluid due to a congenital disorder.
  • High blood glucose levels (caused by diabetes before or after becoming pregnant).
  • Carrying identical twins with twin to twin transfusion syndrome (TTTS).

What are the three C's of esophageal atresia? ›

Healthcare providers learn to recognize esophageal atresia by the “three Cs”:
  • Coughing.
  • Choking.
  • Cyanosis (a bluish tint to the skin, a sign of low oxygen).

What is the survival rate for TEF? ›

The survival rate in healthy infants who undergo surgical repair for a congenital tracheoesophageal fistula (TEF) may be 100%. In groups of infants who have comorbidities or who are not fit enough for early repair, the survival rate is 80-95%.

What are the long term effects of esophageal atresia? ›

Late complications of EA/TEF include tracheomalacia, a recurrence of the TEF, esophageal stricture, and gastroesophageal reflux. These complications may lead to a brassy or honking-type cough, dysphagia, recurrent pneumonia, obstructive and restrictive ventilatory defects, and airway hyperreactivity.

What happens if esophageal atresia is not corrected? ›

Without a working esophagus, it's impossible to receive enough nutrition by mouth. Babies with EA are also more prone to infections like pneumonia and conditions such as acid reflux. Luckily, EA is usually treatable.

Is esophageal atresia an emergency? ›

Esophageal atresia is considered a surgical emergency. Surgery to repair the esophagus should be done quickly after the baby is stabilized so that the lungs are not damaged and the baby can be fed. Before the surgery, the baby is not fed by mouth.

What is the difference between TEF and esophageal atresia? ›

TE fistula is an abnormal connection between the esophagus and the trachea. Esophageal atresia happens when the esophagus has 2 segments. These parts don't connect to each other. Your child's healthcare provider will often spot symptoms of these issues soon after your baby is born.

What is considered severe polyhydramnios? ›

In cases with multiple gestation, a range of 3–8 cm is defined as normal. With this method, polyhydramnios is classified as mild, moderate or severe. Mild polyhydramnios is characterized by a value of 8–11 cm, moderate polyhydramnios by a value between 12–15 cm and severe polyhydramnios by values above 16 cm 86.

How long can a baby stay in the womb without amniotic fluid? ›

In cases where your baby would be premature, they may survive just fine for weeks with proper monitoring and treatment, usually in a hospital setting. In cases where your baby is at least 37 weeks, current research suggests that it may be safe to wait 48 hours (and sometimes longer) for labor to start on its own.

Is polyhydramnios considered high risk? ›

Polyhydramnios may increase the risk of these problems during pregnancy: Preterm birth – Birth before 37 weeks of pregnancy. Premature rupture of the membranes (PROM) – When the amniotic sac breaks after 37 weeks of pregnancy but before labor starts.

How does polyhydramnios cause atresia? ›

In utero, duodenal atresia, a proximal gastrointestinal tract obstruction, causes polyhydramnios by interfering with the gastrointestinal absorption of amniotic fluid swallowed by the fetus distal to the level of intestinal obstruction.

What anomalies are associated with esophageal atresia? ›

Approximately 19% of infants with esophageal atresia meet criteria for VACTERL. This x-ray shows anomalies associated with VACTERL (vertebral anomalies, anal atresia, cardiac malformations, tracheoesophageal fistula, esophageal atresia, renal anomalies and radial aplasia, and limb anomalies) syndrome.

What is polyhydramnios often associated with? ›

Typical symptoms of polyhydramnios include maternal dyspnea, preterm labor, premature rupture of membranes (PPROM), abnormal fetal presentation, cord prolapse and postpartum hemorrhage. Due to its common etiology with gestational diabetes, polyhydramnios is often associated with fetal macrosomia.

What trisomy is associated with polyhydramnios? ›

Polyhydramnios is also associated with various genetic disorders, including Down syndrome (Trisomy 21) and Edward's syndrome (Trisomy 18), but only when the baby also has a duodenal atresia or other blockage in the gastrointestinal tract.

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