Anaesthetic Management of a 4-Day-Old Neonate with Congenital Diaphragmatic Hernia: A Case Report

1. Professor, Department of Anaesthesiology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Dr. D. Y. Patil Vidyapeeth (Deemed to be University), Pimpri, Pune, Maharashtra, India. 2. Assistant Professor, Department of Anaesthesiology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Dr. D. Y. Patil Vidyapeeth (Deemed to be University), Pimpri, Pune, Maharashtra, India. 3. Resident, Department of Anaesthesiology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Dr. D. Y. Patil Vidyapeeth (Deemed to be University), Pimpri, Pune, Maharashtra, India.

Abstract

Congenital Diaphragmatic Hernia (CDH) is a life-threatening condition characterised by the herniation of abdominal organs into the thoracic cavity through a diaphragmatic defect, leading to pulmonary hypoplasia and Persistent Pulmonary Hypertension (PPHN). This case report highlights the perioperative anaesthetic management of a 4-day-old, 2.5 kg neonate with left-sided CDH and severe pulmonary hypertension undergoing surgical repair. The neonate was intubated immediately after birth due to respiratory distress and was managed with Synchronised Intermittent Mandatory Ventilation (SIMV). Cardiopulmonary support was provided using infusions of milrinone, sildenafil, and dobutamine. After stabilisation, surgical repair was performed on day four of life. Anaesthesia was induced with sevoflurane and oxygen under spontaneous respiration to minimise barotrauma, transitioning to controlled anaesthesia with atracurium following bowel exteriorisation. A caudal epidural catheter was placed for intraoperative and postoperative analgesia using 0.1% ropivacaine. Following surgery, the neonate remained on SIMV with gradual weaning from respiratory support, achieving successful extubation by postoperative day three. Regular monitoring and a multidisciplinary approach ensured stabilisation and optimal recovery. This case underscores the importance of comprehensive perioperative strategies in managing neonates with CDH. Key measures include careful preoperative stabilisation, use of spontaneous ventilation during anaesthesia induction to reduce lung trauma, and effective analgesia using caudal epidural techniques. Combining these interventions with cardiopulmonary support can significantly improve outcomes in this high-risk population.

Anaesthesia, Epidural anaesthesia, Infant, Intermittent positive-pressure ventilation, Newborn, Paediatric, Pulmonary hypertension

Case Report

We report a case of a four-day-old neonate weighing 2.5 kg with a left-sided CDH complicated by severe PPHN. Antenatal ultrasound at 33.5 weeks of gestation showed a left-sided diaphragmatic defect with herniation of the bowel loops, stomach, and spleen into the left hemithorax, along with dextrocardia. The foetal Lung-to-Head Circumference Ratio (LHR) was 0.26, corresponding to an observed-to-expected LHR of less than 25%, indicating a poor prognosis.

The neonate was born at term via caesarean section and displayed signs of respiratory distress and bradycardia immediately after birth. The patient was promptly intubated, sedated, paralysed, and transferred to the Neonatal Intensive Care Unit (NICU), where they were placed on SIMV with an FiO₂ of 35%.

Routine investigations, including complete blood count, serum electrolytes, and Arterial Blood Gas (ABG) analysis, were conducted, revealing severe respiratory and metabolic acidosis. A chest X-ray suggested the presence of bowel loops in the left hemithorax with displacement of the heart to the right (Table/Fig 1). A Two-Dimensional Echocardiogram (2D ECHO) revealed severe pulmonary hypertension, significant tricuspid regurgitation, a dilated right atrium, a small patent foramen ovale, and a persistent ductus arteriosus with a left-to-right shunt. Consequently, the patient was started on infusions of milrinone (0.5 mcg/kg/min) and sildenafil (1.6 mg/kg/dose) to reduce pulmonary vascular resistance, along with dobutamine (10 mcg/kg/min) for inotropic support, to optimise cardiopulmonary function. Following stabilisation and correction of acidosis, surgical repair of the diaphragmatic defect was planned for the fourth day of life. Informed consent was obtained from the guardians after detailed counselling regarding the neonate’s condition and prognosis.

Intraoperatively, Heart Rate (HR), Blood Pressure (BP), Electrocardiogram (ECG), temperature, and pre- and post-ductal oxygen saturation were monitored. A 24 G intravenous line and an umbilical venous catheter were secured preoperatively. The patient was managed on a Jackson-Rees (JR) circuit at an FiO₂ of 50%, maintaining pre-ductal oxygen saturation at 90% and post-ductal at 87% (Table/Fig 2). Preoperative infusions were continued throughout the procedure. A caudal epidural catheter was placed for analgesia. Anaesthesia was induced under spontaneous respiration with oxygen and sevoflurane until bowel exteriorisation (Table/Fig 3). Subsequently, neuromuscular blockade with atracurium was initiated, and a caudal epidural infusion of 0.1% ropivacaine at 0.2 mg/kg/hour was maintained throughout the procedure.

Following the reduction of hernia contents and defect repair (Table/Fig 3), (Table/Fig 4), oxygen saturation improved to 94% pre-ductal and 92% post-ductal (Table/Fig 5). The patient was subsequently returned to the NICU, still intubated and sedated with a left intercostal drain in situ, and was reinitiated on SIMV, with ongoing preoperative infusions to promote lung expansion and function (Table/Fig 6). Extubation was achieved uneventfully on postoperative day three. The perioperative management strategy is comprehensively outlined in (Table/Fig 7).

Discussion

CDH is a life-threatening condition characterised by the herniation of abdominal contents into the thoracic cavity through a developmental defect of the diaphragm, which impairs lung development. This leads to pulmonary hypoplasia, aberrant pulmonary vascular architecture, and PPHN (1),(2). Paoletti M et al., report a global prevalence of approximately 2.3 per 10,000 births, with left-sided defects accounting for 90% of cases, typically involving herniation of bowel loops, the stomach, spleen, and liver (3).

CDH can further be classified anatomically as a posterolateral (Bochdalek) or anteromedial (Morgagni) defect, with Bochdalek hernias being the most common (4). The prognosis is influenced by multiple factors, including gestational age at diagnosis, defect size, liver herniation, associated anomalies, and birth weight (1),(4). In the present case, prenatal imaging demonstrated significant visceral herniation and mediastinal shift, consistent with a large left-sided defect and observed-to-expected LHR of <25%—features linked to a poor prognosis (5).

CDH is often associated with congenital anomalies and syndromes such as CHARGE, Beckwith-Wiedemann, Cornelia de Lange, and Denys-Drash, as well as chromosomal abnormalities including trisomies 13, 18, 21, and Turner syndrome (45,X) (6). Compression of the developing lungs by herniated organs leads to pulmonary hypoplasia and a reduction in functional surface area for gas exchange. As a result, the neonate exhibited respiratory distress immediately after birth, necessitating prompt intubation to ensure adequate oxygenation.

Effective ventilatory management in such cases requires balancing adequate oxygenation with the avoidance of barotrauma to the hypoplastic lungs. In this case, SIMV was chosen to support spontaneous respiratory efforts while maintaining controlled airway pressures, consistent with lung-protective strategies recommended in recent literature (1),(7). A similar approach was used by Parate LH et al., who successfully managed a case of right-sided CDH with permissive hypercapnia and low peak inspiratory pressures to reduce barotrauma (8).

Pharmacological infusions were initiated to manage severe pulmonary hypertension and right heart strain. Milrinone, a phosphodiesterase-3 inhibitor, enhanced myocardial contractility and reduced pulmonary vascular resistance. Sildenafil, a phosphodiesterase-5 inhibitor, was co-administered to further decrease pulmonary vascular resistance via the nitric oxide pathway, as noted by Nakwan N et al., (9). Dobutamine provided inotropic support, improving myocardial output while avoiding increased systemic vascular resistance that could hinder right ventricular ejection (7). Omar MA et al., described a similar protocol in their case study, utilising milrinone, inotropes, and vasopressors to stabilise a neonate with bilateral CDH prior to surgery (10).

Surgical repair was delayed until the fourth day of life to allow for the stabilisation of the neonate’s cardiopulmonary function and the correction of metabolic acidosis, thereby optimising conditions for safer intraoperative and postoperative management (2),(11). In their case report, Parate LH et al., likewise deferred surgery until the fourth day of life to facilitate preoperative cardiopulmonary optimisation (8). Tiwari C et al., also espoused the importance of preoperative optimisation in improving postoperative outcomes (12).

Intraoperatively, the patient was initially maintained on spontaneous respiration until the reduction of hernia contents to minimise sudden changes in thoracic pressure and facilitate better ventilation and lung expansion (1),(7). Caudal epidural analgesia was used to minimise opioid-related respiratory depression and support stable ventilation, as recommended in CDH management (7),(13). Its safety and efficacy in neonatal surgery are well established, particularly with ropivacaine, as demonstrated by Khalil SN et al., and supported by Gupta A et al., (14),(15). Kulkarni K et al., also utilised caudal epidural analgesia to facilitate perioperative pain control and maintain stable haemodynamics intraoperatively (16). Similarly, Omar MA et al., employed regional anaesthesia to provide effective analgesia while minimising systemic opioid requirements (10).

Postoperatively, SIMV and pharmacological infusions were continued to further support lung expansion and optimise gas exchange in the previously hypoplastic lungs [1,7]. Adequate pain control was maintained through caudal epidural and gradual weaning from ventilatory support was prioritised to reduce the risk of pulmonary hypertensive crises (9),(13). Long-term outcomes in CDH survivors commonly include chronic lung disease, gastroesophageal reflux, feeding difficulties, and neurodevelopmental delays. Studies report that up to 30% of infants require supplemental oxygen at discharge, and a significant proportion experience growth or cognitive delays, highlighting the importance of coordinated multidisciplinary follow-up (13).

Conclusion

In neonates with CDH, preoperative optimisation is crucial for successful management. Achieving cardiopulmonary stabilisation prior to surgery not only minimises intraoperative complications but also establishes a foundation for improved postoperative outcomes. Additionally, initiating spontaneous ventilation during the early phases of anaesthesia—prior to bowel exteriorisation—helps reduce the risk of barotrauma by preserving the neonate’s natural respiratory efforts. Finally, the effective use of epidural analgesia, both intraoperatively and postoperatively, significantly enhances pain control, thereby promoting optimal respiratory efforts and smoother recovery.

These integrated strategies collectively contribute to improved overall outcomes in this high-risk neonatal population.

DOI and Others

DOI: 10.7860/JCDR/2025/79428.21320

Date of Submission: Mar 19, 2025
Date of Peer Review: Apr 10, 2025
Date of Acceptance: Jul 02, 2025
Date of Publishing: Aug 01, 2025

AUTHOR DECLARATION:
• Financial or Other Competing Interests: None
• Was informed consent obtained from the subjects involved in the study? Yes
• For any images presented appropriate consent has been obtained from the subjects. Yes

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EMENDATIONS: 6