I care for many children with septooptic dysplasia and a few with Rathke's cleft cyst. It is a developmental abnormality that occured during development in utero. Cause is unknown. When it affects things in the midline brain area this includes the pituitary-hence need for endocrine folks to assess the hormones. I am glad to hear that his visual pathways are not affected. There may be a need to also have consultation with a neurosurgeon. Although the cyst does not continue to develop, some folks want to be sure that the structure does not change over time. Also the endocrinologist needs to test first and foremost the pituitary adrenal axis, this is the ability of the pituitary to tell the adrenals when to put out important cortisol during stress, this is tested with an acth stim test-takes about 2 hours and is really important. The other things to monitor include thyroid as the pituitary tells the thyroid to get going. Also growth hormone and later in life puberty(but lets not worry about that yet!)
Vision needs to be assessed and seeing an opthalmologist is critical as you have already done. Before surgery for the cleft or anything, have his adrenal test done, cortisol is an important hormone and if surgery or other stressors are happening our body relies on good communication with the pituitary and adrenal glands.
Thank you for your response. We live rather small rural area in Florida with limited medical resources. Wes is followed by a ped endocrinologist, but she has no expererience with children with the Wes' conditions. She treats mainly diabetic patients.
He will follow with a neurosurgeon in Chicago every 6 months for a MRI to monitor the cyst. The Rathke's cleft cyst was found with MRI's that were looking at Nasoethmoidal encephalocele that was repaired August 07.
Although he presently has aspiration pneumonia from his last MRI(extubation), his palate surgery will be scheduled once he has a clear chest xray. He did have a growth hormone stimulation test at 10 months old. Would this stimulate cortisol production also or would it need to be specific for cortisol levels? He is 2 years old, would a test at 10 months olds be accurate?
Could cortisol levels affect his ability to recover from illness, which seems to be concern with him?
Again thank you for your time.
why do you go to chicago for the mri, is that where his surgeons are? Yes, cortisol is important in helping us with stress and illness, if they used insulin to stimulate low sugar then they measured cortisol, find out from the folks that did the stim test
Yes, Wes neurosurgeon is located in Chicago at Children's Memorial. She is experienced with basal enecphalocele, which was not the case with the other neurosurgeon Wes saw. Wes is actually in the hospital now, after 10 days of antibiotics at home, he was spiking fevers 105.5. Seems to be responding well to iv abs. His stim test was done at 7 months old, he turned 2 years old March 08. The past few months he had the high fevers and what seems to be a very low resistance. He is constantly sick. I feel like something is not right. Maybe I am worng, I just feel like my little guy is worth searching for the answers.
We are in the process of looking for a endocrinologist for a second opinion. Wes ped recommendation is here in Florida at Shands.
Any suggestions for ped endocrinologist here in Florida?
There are pediatric endocrinologists at Children's Memorial, while he is in the hospital ask neurosurg to explore his endocrine status by consulting them, they can get a random cortisol while he is sick and if it is high we know we make ample steroid response to stress.
If not he may need replacement during illnesses like this one.
Hope he recovers soon!
He is in a very small hospital in Florida near our home. He will need to see an endocringologist every 6 months, so I am also looking for one to follow him.
Is there any other reason than his cortisol level that might cause him to run high fevers?
I would really like to see him healthy. Hate that he is sick so often, just would like to see him grow and have fun, be a 2 year old.