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Edward’s syndrome and emergency neurosurgery: Anaesthetic concerns for leaking meningomyelocele repair in a neonate with edward’s syndrome

 Department of Anaesthesiology, Dr Ram Manohar Lohia Hospital & Atal Bihari Vajpayee Institute of Medical Sciences, Baba Kharak Singh Marg, New Delhi, India

Date of Submission04-Jan-2021
Date of Decision30-Jan-2021
Date of Acceptance16-Feb-2021
Date of Web Publication07-Jan-2022

Correspondence Address:
Uma Hariharan,
Department of Anaesthesiology, Dr Ram Manohar Lohia Hospital & Atal Bihari Vajpayee Institute of Medical Sciences, BH 41, East Shalimar Bagh, Delhi 110088.
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpn.JPN_5_21


How to cite this URL:
Hariharan U, Ohri R, Gupta V, Shivpriya P N. Edward’s syndrome and emergency neurosurgery: Anaesthetic concerns for leaking meningomyelocele repair in a neonate with edward’s syndrome. J Pediatr Neurosci [Epub ahead of print] [cited 2023 Sep 26]. Available from: https://www.pediatricneurosciences.com/preprintarticle.asp?id=335208

Dear Editor,

Anesthetic concerns for pediatric neurosurgery are multifold and become more challenging with syndromic children like in Edward syndrome (Trisomy 18), which include airway problems (micrognathia), congenital heart disease, craniofacial anomalies, visceral abnormalities, and mental retardation. A 4-day old, preterm neonate weighing 1.39 kg, was posted for emergency repair of leaking meningomyelocele. There was difficulty in suckling and feeding since birth. Antenatal maternal ultrasound revealed hydrocephalus, ventriculomegaly, and disorganized lumber and sacral vertebre. On physical examination, he had a lumbar meningomyelocele which was leaking since birth, along with skeletal dysplasia, micrognathia with proximal shortening of lower limbs, and bilateral “Rocker bottom foot.” Pre-operative vital parameters were: heart rate - 131/min, respiratory rate - 48/min, blood pressure -57/31 mmHg, Spo2 - 97% on room air, blood sugar (random) - 94 mg/dL, on blood gas analysis pH was 7.28, pCo2 - 41.3 mmHg, po2 - 40.32 mmHg. All other laboratory investigations were within normal limits. Cardiac 2-D echocardiogram revealed that he had a 4 mm of ostium secundum atrial septal defect (ASD) and 2 mm of patent ductus arteriosus (PDA) with mild tricuspid regurgitation (TR). On ultrasound examination of the cranium, there was gross dilatation of third and fourth ventricles. A diagnosis of Edward syndrome was made by the pediatric team.

After thorough pre-anesthetic evaluation, the child was shifted to a pre-warmed operating room. Precordial stethoscope was placed along with standard ASA monitoring and difficult airway cart was kept ready. For temperature monitoring, we placed temperature probe in axillary region. Following preoxygenation, modified rapid sequence induction was performed using 1.5 mcg/kg of fentanyl, etomidate 0.3 mg/kg, and 0.6 mg/kg rocuronium IV. A 3.5 mm uncuffed endotracheal tube was inserted and oral packing was done [Figure 1]. Anesthesia was maintained with sevoflurane + air + oxygen mixture. After intubation, cannulation of right femoral vein and right radial artery were done under strict aseptic precautions. Patient was made prone with adequate padding. Hypothermia prevention methods like warm intravenous fluids, forced air warmer, and covering of exposed body parts were used. Anesthestic considerations for congenital heart disease with left to right shunt were followed, including keeping all the lines free of air bubbles and stabilizing hemodynamics (maintenance of systemic vascular resistance (SVR), peripheral vascular resistance (PVR) and heart rate). The entire perioperative period was uneventful. Postoperatively, oral pack was removed and the neonate was shifted to NICU (neonatal intensive care unit) intubated for elective ventilation and monitoring in view of preoperative and intraoperative ABG findings (acidosis). The neonate was extubated the next day with normal vital parameters. He was later referred to cardiology/cardiac surgery department for definitive management of congenital heart disease.
Figure 1: Picture showing intubated child with Edward syndrome

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Edward syndrome is the second most common autosomal trisomy after down syndrome.[1] It consists of congenital heart disease, craniofacial anomalies, muscular hypotonia, mental retardation, and extremity deformities.[2] Congenital heart disease can present in the form of atrial septal defect (ASD), ventricular septal defect (VSD), and patent ductus arteriosus (PDA). There is a need for thorough pre-anesthesia planning in a case of child with Edwards syndrome with multiple defects in order to prevent any drastic hemodynamic changes.[3] There is also the possibility of difficult airway because of micrognathia.[4] There is concern regarding patient positioning to prevent rupture of meningomyelocele sac. Few previous reports show that succinylcholine should be avoided in cases of Edward syndrome, because it may cause muscle rigidity.[5] Hence, we used Rocuronium in place of succinylcholine. There is dearth of the literature available for the anesthesia management of a child with Edward syndrome with multiple systemic defects.

Declaration of patient consent

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

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Conflicts of interest

There are no conflicts of interest.

   References Top

Cereda A, Carey JC. The trisomy 18 syndrome. Orphanet J Rare Dis 2012;7:81.  Back to cited text no. 1
Tsukamoto M, Hitosugi T, Esaki K, Yokoyama T. The anesthetic management for a special needs patient with trisomy 18 accompanying untreated tetralogy of Fallot. Egypt J Anaesth 2017;33:213-15.  Back to cited text no. 2
Arun O, Oc B, Oc M, Duman A. Anesthetic management of newborn with trisomy 18 undergoing closure of patent ductus arteriosus and pulmonary artery banding. Cardiovasc J Afr 2014;25:e10-2.  Back to cited text no. 3
Bali Ç, Özmete Ö, Ergenoğlu P, Akın Ş, Arıboğan A. Anaesthesia management for edward’s syndrome (trisomy 18). Turk J Anaesthesiol Reanim 2016;44:157-8.  Back to cited text no. 4
Matsuda H, Kaseno S, Gotoh Y, Furukawa K, Imanaka K. Muscle rigidity caused by succinylcholine in edwards’ syndrome. Masui 1983;32:125-8.  Back to cited text no. 5


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