Dr.SreeLekshmy.S, Dr.C.Jayakumar, Dr.Jayasree, Dr.Sheela Namboothiri
Pretem (36+3weeks) born to a 34 year old G2A1 mother with antenatal scans showing fetus with vein of galen malformation, dextrocardia, bilateral SVC, lower placed and malrotated right kidney, single umbilical artery.
AFI- 31.
Microarray sent was normal.
Ba was born at 36+3 weeks of gestation on 20/09/24 at 10:36am elective LSCS due to Breech)m. Mother has history of GDM and was on Insulin and OHA.
H/o polyhydraminos- Post amino reduction on 24/8/24 & 06/09/24.
Ba was born dusky, floppy, with no spontaneous respiration.
IPPV via Tpiece resuscitator was initiated at pressures of 20/6 on 100% Fio2.
There was no response to this PIP which was increased due to absent chest rise. Lungs appeared stiff and IPPV was continued on 100% Fio2.
Nasogastric tube (Fr 6) insertion was attempted for stomach decompression. Due to no increase in Heart rate and no spontaneous breathing ba was intubated with ET tube 3.5mm.
Minimal chest rise noted with high pressures. Due to no increase in heart rate, chest compressions were begun. Adrenaline was given through ET tube initially and then through UVC as per NRP protocol. NS bolus was also administered. The abdomen was noted to have a steady distention and NGT Fr 8 size insertion was attempted for abdominal decompression. However there was difficulty in passing the NGtube which was coiled in the mouth.ET continued to be in position with equal air entry. Despite all measures ba could not be resuscitated, HR deteriorated and was declared dead at 10:46am. APGAR-1/10 at 1 minute and 5 minutes of life. Cord gas pH/ pCO2/ pO2/ HCO3/ BE- 7.32/ 35.6/ 32.7/ 17.9/-7.0
Due to the suspected airway abnormality in addition to the antenatally diagnosed multiple anomalies, a Genetics consultation was availed and was advised infantogram, WES and fetal autopsy. Infantogram revealed 11 thoracic ribs, bilateral pneumothorax and the nasogasatric tube (6Fr inserted at 20cm) coiled in the high airway. The option of autopsy was offered to the parents but was declined.
Cause of death could be Birth asphyxia suspected cardiacfailure with antenataly detected vein of galen malformation and suspected tracheo oesophageal anomalies.
Discussion:
Anatomy and Function
Location: The vein of Galen is located deep in the brain, formed the convergence of the great cerebral vein and other smaller veins.
Function: It plays a critical role in draining blood from the deep structures of the brain (e.g., thalamus, basal ganglia) into the straight sinus, which leads to the venous system.
Vein of Galen Malformation (VOGM)
Definition: A vascular anomaly characterized abnormal connections between the cerebral arteries and the vein of Galen.
Pathophysiology: Increased blood flow and pressure can lead to congestive heart failure, hydrocephalus, and neurological issues.
Symptoms: May include increased head size, developmental delays, seizures, and signs of heart failure.
Diagnosis: Typically diagnosed via imaging techniques such as ultrasound, MRI, or CT scans, often in infants.
Treatment: Options may include endovascular embolization to reduce blood flow to the malformation and surgical interventions for associated complications.