VACTER Pediatric Anaesthesia Case Presentation

Case presentation on VACTER case.

1. Case Presentation Dr. Samir Inayath Dr. Damodar Rao

2. Case History A new born was referred to paediatric emergency with multiple congenital anomalies Child was born at 32 weeks via LSCS in a private hospital, CIAB, weighing 2 kg Antenatal history is uneventful, oligohydramnios, prev. LSCS, regular ANCs, TIFFA not done Physical examination of the child revealed; Cleft lip and palate Heart murmur Abnormal thumbs Absent anus Sacral dimple

3. Pre-Anaesthetic Assessment Child was posted for emergency Colostomy O/E; Baby was active, warm CVS Pansystolic murmur, heard all over the chest fields SpO2 - preductal ~65%, postductal ~70% RS B/L air entry with adventitious sounds Cleft Lip and Palate grade 3 Sacral anomaly on palpation, ambiguous genitalia Abdomen was soft, and non distended

4. Investigations Hb - 12 g%, WBC - 14,800 /cumm, Plt - 2.8 L/cumm Na - 138, K - 4.2, Cl - 108, Urea - 14.8, Creat - 0.88 GRBS - 97 mg% Baby BGT - B pos, LRPRBC reserved Screening 2D-echo showed, Large subcentric VSD, Overriding of Aorta, PDA with bidirectional shunting ? TOF USG abdomen poor window, ?hydronephrosis USG spine, linear tract noted from coccyx to skin, without meninges or neural elements

5. Anaesthetic Management Difficult airway cart, emergency drugs were kept at ready All monitors were attached (pre/post ductal SpO2), shoulder roll, precordial stethoscope were placed. All lines were flushed for air bubbles. No premedication was given Appropriate endocarditis prophylaxis was given 30 mins before procedure. 2% Sevoflurane inhalation in 100% Oxygen.

6. Child was positioned on lateral side, and aseptic drape covered. 26 G short stilleted needle was used to perform procedure at L4 - L5 interspace. 0.3mL of 0.5% Bupivacaine Heavy was injected. Level of anaesthesia was verified by pinch, T6 level was noted at start of surgery. 100% O2 was delivered with mask during surgical procedure. 2% Dextrose RL was used as intraoperative fluid Surgery was performed by senior surgery Professor Hemodynamic changes (HR, NIBP) we limited to less than 10% from baseline values

7. Surgical duration was 30 mins. After completion child was shifted to post op warmer for observation. Child was active and maintaining vitals through out. Regression of spinal level was noted at 40 mins, T12 to pinch and complete regression at 60 mins. Following this baby was shifted to NICU for further care and management.

8. Case Discussion

9. VACTERL Our patient was included in VACTERL group as the patient had; Sacral anomaly Anorectal malformation Congenital cardiac disease Hydronephrosis Abnormal th umbs Cleft lip and palate To qualify as VACTERL, 3 of the 7 components must be present with or without other associated developmental defects

10. V - Vertebral and Vascular anomalies (60 - 70%) Small hypoplastic/dysplastic/missing/supernumerary vertebrae; Hemivertebrae 'butterfly' vertebrae, wedge vertebrae, vertebral clefts and fusion; Caudal regression; tethered cord; branchial arch/cleft abnormalities; rib anomalies; Sacral agenesis; dysplastic sacral vertebrae; Scoliosis or kyphoscoliosis secondary to costovertebral anomalies; C5-6 dislocation and severe stenosis with spinal cord impingement Early complications – minimal; Late complications – risk of developing scoliosis/back pain Single umbilical artery 20%

11. A - Anal Atresia / imperforate anus (upto 90%) Oesophageal atresia can occur as an isolated defect with an incidence of around 8%. C - Cardiovascular anomalies (40 - 80%) T - Tracheo-oesophageal fistula (15 - 30%) Involvement of anus relates to risk of genital abnormalities, recto-vaginal fistulas and urogenital complications. VSD +/- heart failure, ASD, TOF Less common defects – truncus arteriosus, transposition of the great arteries, hypoplastic left heart syndrome, PDA, co-arctation of aorta

12. R - Renal anomalies (50-80%) Can effect one or both kidneys Severe reflux or obstruction, Horseshoe kidneys, cystic, aplastic, dysplastic or ectopic kidneys, hydronephrosis, unilateral +/- bilateral agenesis. May require kidney transplant. L - Limb anomalies (50-80%) Includes displaced, absent or hypoplastic thumb/s, polydactyly, syndactyly and radial aplasia/ dyplasia / hypoplasia; radial deformities; radioulnar synostosis; club foot; hypoplasia of great toe/tibia; lower limb tibial deformities

13. Anticipated problems were difficult airway and abdominal distension, Baby was nil by mouth with a nasogastric tube in situ. Positive pressure ventilation had to be avoided due to risk of regurgitation. Extubation would be needed to be orchestrated as carefully as intubation. GA has been associated with high incidence of postoperative apnoea, bradycardia, desaturation and requirement of prolonged post op ventilation. Postoperative problems include post-extubation croup and obstruction.

14. SAB in infants has been associated with decreased incidence of hypotension, hypoxia, bradycardia, and postoperative apnea as compared to GA No reports of regional blocks being contraindicated in VACTERL patients. Care to be taken when performing neuraxial blocks if they have an imperforate anus, genitourinary abnormalities or sacral dimple, as they frequent occult spinal dysraphisms. Spine ultrasound can be performed to rule out abnormality.

15. Neonatal spines have one primary anterior concave curvature. The spinal cord ends between L2 and L3 vertebrae in 90% of premature infants and between L1 and L2 vertebrae in 92% of term infants. The dural sac is at the S4 level at birth and reaches the S2 level by the end of the first year. The intercristal line crosses at the L5–S1 interspace at birth and is a safe landmark to prevent cord injury. The neonate may be kept in the lateral decubitus or sitting position. The distance from skin to epidural space is about 6 mm at birth. Paediatric spinal needles range from 22 to 29 G. Quincke, Sprotte and Whitacre variations

16. Dose - Drugs - Hyper / Isobaric Bupivacaine, Ropivacaine, Levobupivacaine. Adjuvants - Fentanyl, Clonidine Effect of the block can be judged by lack of response to pinch/tetanic stimulus. 0 - 5 kg 0.1 mL / kg 0.5 mg / kg 5 - 15 kg 0.08 mL / kg 0.4 mg / kg > 15 kg 0.06 mL / kg 0.3 mg / kg

17. …Findings show that hemodynamic parameters in infants with CHD undergoing NCS under awake SA are not different from controls without CHD and that SA appears to be safe in infants with CHD.

18. To conclude Ultrasound spine is a quick, feasible, economical way to look for spinal dysraphisms. Spinal anaesthesia in neonates has clear advantages over general anaesthesia. Spinal anaesthesia can useful considered in CHD.

19. References Jain D, Sampley S, Kaur G. Association of difficult airway to VACTERL anomaly: An anesthetic challenge. Anaesthesia, Pain & Intensive Care. 2019 Jan 29:192-4. Subramaniam R. Anaesthetic concerns in preterm and term neonates. Indian J Anaesth 2019;63:771‐9. Oberlander TF, Berde CB, Lam KH, Rappaport LA, Saul JP. Infants tolerate spinal anesthesia with minimal overall autonomic changes: analysis of heart rate variability in former premature infants undergoing hernia repair. Anesth Analg. 1995 Jan;80(1):20-7. doi: 10.1097/00000539-199501000-00005. PMID: 7802281. Goyal R, Jirtjil K, Baj BB, Singh S, Kumar S. Paediatric spinal anesthesia. Indian journal of anesthesia. 2008 May 1;52(3):264. O'Neill BR, Yu AK, Tyler-Kabara EC. Prevalence of tethered spinal cord in infants with VACTERL. J Neurosurg Pediatr. 2010 Aug;6(2):177-82. doi: 10.3171/2010.5.PEDS09428. PMID: 20672940. Caliskan E. Spinal anaesthetic management in paediatric surgery. In Pediatric and Neonatal Surgery 2017 May 3. IntechOpen. López T, Sánchez FJ, Garzón JC, Muriel C. Spinal anesthesia in pediatric patients. Minerva anestesiologica. 2012 Jan 1;78(1):78.

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