The patient came to the clinic 20-November-2010 complaining of loss of vision left eye for three weeks with decreased vision right eye. She is complaining of headache  and amenorrhea for 8 months. The patient was operated in Saudi Arabia 06-August-2010 with any benefit. Transsphenoidal approach was used and biopsy revealed craniopharyngioma as the histological result.

On examination: The patient  is almost blind in left eye and decreased vision right eye with hemianopia temporal field. The patient has no sensory, nor motor deficit. She is amenorrheic  and feel thirst all the time.

MRI brain done 13-November-2010 showing huge tumor with intra-supra- parasellar extension with invasion of the III ventricle. It was clear to see the previously performed approach and to see the small bone defect at the pituitary floor. The patient was sent for neuroophthalmological and endocrine evaluation which confirmed the almost blind left eye and the the residual of the visual field of the right eye. The cortisol ACTH where very low. Prolactin was 51 ng/dL.

Bifrontal approach was achieved with reflection of the bone flap to the right. The dura was opened parallel to the inferior edge of the bone defect. The olfactory bulbs and tracts were mobilized from the mediobasal frontal lobes. The right olfactory tract was dissected down to the trigone. The tumor was attacked between the optic nerves and piece-meal resection was performed. The tumor was rubbery in consistency, but it had good cleavage. That part compressing the left optic nerve was removed totally. The posterior right parasellar extension was removed in second stage. The suprasellar tumor which was invading the III ventricle was removed totally, trying to avoid traction with force. The pituitary stalk was the origin of the craniopharyngioma, which was attacked at the last stage, trying to minimize the remaining dust at the pituitary stalk, without violating its anatomical integrity.

Routine closure of the wound. Smooth postoperative recovery and improvement of the vision in both eyes. The patient was happy with her postoperative improvement.

This case and similar 4 cases over 30 years of experience with solid component of the craniopharyngioma pushing up far the III ventricle with blindness in one or both eyes, means that tumor is not only distorting the optic nerves, chiasm, A1 segments and the floor of the III ventricle, it is compressing them with force.  The one stage total removal in these cases must be avoided so as not to end with such catastrophe.  The flow of surgery in this case was smooth and the solid part came with ease from the floor of the III ventricle.  In the next case I will divide such surgery for 2 stages: The first will be the removal of the intrasellar, antesellar and parasellar parts and to leave the suprasellar part intentionally, to avoid such catastrophe. After that, with in 1-2 months later according to MRI data, to see the downward migration of the suprasellar part over the days by the cardio-pulmonary pulsation to the empty cavity created by the first surgery. If the tumor is separable from the floor of the III ventricle, then to proceed to the second stage of removal. If it is stuck with the floor, then better to leave it and think about radiotherapy.

Minirin in some patients is not effective, and there is a notice, that the previous trans-sphenoidal approach could participate with this no response.

With the experience with DI, Minirin is effective and the DI usually is responding well to medication. This hidden DI which showed itself one month ago and now, did not reflect itself by the specific gravity of the urine. The specific gravity was 1.010 before surgery and 1.005  during the peak of hypernatremia.

The hypothalamus is still a mystery and there must be unknown to us functions, that need to clarify and special management for them.

The cause of DI and hypernatremia, by no means could be explained by the reaction of the pituitary stalk. It is due to reversal of the floor of the III ventricle and the infendibulum down to its normal position over 3-4 hours of decompression and resection of the solid part of the tumor. 

The patient underwent first transsphenoidal surgery for craniopharyngioma, which was originating from the pituitary stalk and expanding to the III ventricle and compressing the chiasm and both optic nerves and the right ICA. This mission is impossible, even with advent of the most robust technologies.

With bifrontal approach with my own modification, patented 1985, it is possible to have these findings and make it possible to practically remove the whole tumor, preserving during that, the involved pituitary stalk and the olfactory function.

The patient the morning of 25-November-2010 showed somnolence and status epilepticus, for what, CT-scan urgently was performed, which revealed no hematoma and no residual of the tumor. The patient urine output was more than 6 liters during night with Na 275mmole/L. Blood sugar was over 400 and the patient was urgently put in ventilator to control the status epilepticus with propofol and depakine I/V was started and the Epanutin was stopped and Tegretol was started in NGT. Minirin was started but she showed minimal response to Minirin.  Trying over the next hours to control the dangerous level of hypernatremia was impossible despite all precautions to slow the slope of regression. It became 178mmole/L  the next day.

The morning of 27-November-2010 showed clinically nearly brain stem areflexia, for what aggressive measures where taken to decrease the brain edema. Another CT-scan was performed and the patient showed return of her corneal, gag reflexes and she could breath spontaneously in the SIMV mode. With all efforts to avoid rapid regression of the hypernatremia, it became 145 during this day. She showed improvement of brain stem functions the night of this day.

The morning of 28-November-2010 the patient showed clinical picture of brain stem areflexia and the BP started to decrease, which was corrected with dopamine infusion. Propofol was stopped and all antiepileptic drugs. Inomed ISIS IOM was connected to the patient over 24 hours, which confirmed the absence of cortical activity and profound decrease in AEP and VEP, but with good response to SEP and MEP. Clinical brain death was established in mid day of 29-November-2010. The patient still in the ventilator.