Follow up investigation brief August 14, 2006
Haya Al Yassin geb. 09.06.2003,
Robert-Koch-Str. 1, 79106 Freiburg
we report about the child Haya Al Yassin who was seen for a follow-up investigation in our department on 23.01.2006.
Diagnoses: 1) Status after operation of a Pilozytic Astrozytoma WHO-Grade I in the right cerebellar hemisphere, in continuing complete remission (C71.9)
2) Occlusive hydrocephalus (G91.9), insertion of a ventriculo-peritoneal shunt on 11.01.06 (Shunt Valve: PAEDI GAV, Pressure horizontal 04 cm H2O, Pressure vertikal 24 cm H2O)
3) Proof of normal function of the shunt
4) Residual neurological syndrome with ataxia and severe delay of psychomotor development after protracted intracranial hypertension in summer and fall 2005
History: For the longer history see our earlier reports. Since the insertion of the shunt in January 2006 the child has shown no more signs of increased ICP. She receives physiotherapy 5x per week and speech therapy 2x per week. She follows simple commands but has not yet developed active speech. Her ability to concentrate is poor. She takes up good personal contact and smiles. She sees and grasps small objects. She grasps with both hands using all fingers and thumb opposition. She is able to sit, kneel and to sit up. She moves with rolling and crawling on her stomach. She is able to drink and to feed herself. She has a normal sleep-waking behaviour. She is very interested in her surroundings.
Clinical: 3 year old girl in a good clinical state. Normal pupillar reaction. Haya fixates and looks at small toys. She shows a palmar grasp and has some problems with thumb opposition. Stable head control. Free and very stable sitting position with extended legs, good balance to the sides. Can rise from prone to a 4-foot-position and to kneeling. Able to stand and bear weight when held at two hands, but with recurvated knee joints and instable position in the hip joints. Inverted position of the right foot. Decreased muscular tone. Deep tendon reflexes symmetrical and not exaggerated. Babinski negative. No walking. Speaks only a few words.
Puncture and Function testing of the shunt: In Kuwait a CCT was performed in April and a MRI on August 1st, 2006. Both had shown distinctly dilated ventricles (I-IV), however without signs of increasd ICP such as CSF exsudation or compression of the external CSF spaces.
To make sure that the shunt is really functioning well we performed a puncture of the Rickham capsule and measured the CSF pressure. It was found to be 3 cm H2O and thus completely normal.
Epicrisis: Nearly one year after the exstirpation of the cerebellar tumor and 6 months after the insertion of the well functioning shunt Haya is still severely retarded without meaningfull speach, ataxia, inability to walk and very bad coordination of her hand function. However, the child is slowly improving and I am sure that she will make constant developmental progress in the future. However, I had to tell the parents that their daughter will never be healthy with completely normal neurological function.
The neuroradiological changes that are persisiting up to now have to be understood as residual damage to the brain tissue, as a result of the long-standing increased intracranial pressure before the operation of the tumor. I want to remind that the child came to our hospital unconscious and in an extremely bad state and that she surely would have died without an immediate operation. We found a cystic and very slowly growing tumor that with certainty had developed over many months, if not from birth onwards. A proper diagnosis at the age of 12 or 18 months would have been able to save most if not all of the neurological function.
Ø Continue physiotherapeutic and rehabilitative treatment
Ø I have prescribed a standing frame to make a standing position easier.
Ø Repeat MRI with contrast medium all 6 months to exclude tumor recurrence.
Ø After 2 years and continuing remission repeat MRI once per year.
With kind regards
Prof. Dr. med. R. Korinthenberg