When Altia Atkinson, a 34-year-old housewife from Reliance, Essequibo, found out that she was pregnant earlier this year, she and her husband Satesh Persaud, a 41-year-old Carpenter, were elated.
Already a mother of five healthy children, Atkinson anticipated an uncomplicated pregnancy, and delivery was inevitable.
As Atkinson sought prenatal care at her community Health Centre, her ultrasounds appeared normal and showed no cause for concern.
It was not until she was admitted for the delivery of her 6th baby that her ultrasound showed some irregularity.
However, physicians at the Suddie Hospital could not determine if the mass they were detecting was growing on Atkinson or her unborn child.
Following a caesarean section, Shailah Ariya Persaud was born on September 19, 2022, at Suddie Hospital and was immediately referred to the Georgetown Public Hospital on account of a malformation that presented as a second head.
Just a few days old, Shailah was reviewed by Chief Neurosurgeon Dr Amarnauth Dukhi, who quickly diagnosed little Shailah with a neural tube defect.
After MRI scans of the brain and cervical spine was done, Dr Dukhi confirmed a rare Cranio-Cervical Junction malformation, a combination of the neural tube defects of the brain and spine as one, a Myelomeningocele-encephalocele occurring at the back of the head where the brain joins the spinal cord.
While neural tube defects are quite a common birth defect, they usually occur separately on the head or the spine.
In fact, the occurrence of encephalocele and myelomeningocele together as one defect in the same patient is rarely described in the medical literature.
In these uncommon cases, the neural tube defect presents as sac-like protrusions on the brain and spine and are caused by the failure of the neural tube to close completely during foetal development, which may be attributed to several internal and external factors, mainly lacking the use of Folic Acid during pregnancy.
The management of encephalocele and myelomeningocele is often associated with numerous challenges, including blood loss, cardio-respiratory disturbances, and hypothermia.
Surgical intervention is critical for preventing hemodynamic fluctuations and excessive pressure on the sac, which may result in premature rupture and eventual death.
On September 30, Chief Neurosurgeon Dr Dukhi led a multi-disciplinary team at GPHC to separate this large malformation and repair the defect at the junction where the brain and spinal cord are joined.
This tedious and complicated medical intervention for Shailah commenced with placing a VP shunt for the developing Hydrocephalus that was also diagnosed.
The large malformation/mass, the size of the normal head, was then carefully separated from the head and upper cervical spine. This was followed by the reconstruction of the craniospinal junction’s defect to prevent the leakage of cerebrospinal fluid and allow for normal development of the brain and spinal structures. After the more than six-hour procedure, Dr Dukhi and the team deemed it successful.
Following seven days of neonatal post-operative care, little Shailah was discharged into her mother’s arms without any apparent neurological deficits and is expected to lead a normal childhood.
Close monitoring will be done to determine any difficulty in the child’s developmental curve.