CPC #7: Tuesday, March 12, 2002
Hurd Hall, The Johns Hopkins Hospital
12:00 Noon

Chief Complaint:
A 31-year-old male with fatigue, headaches, weakness, weight gain, and hypertension 2 months after pituitary surgery.

History of Present Illness:
For approximately 8 years, the patient has had blurred vision and headaches. Within the past year he developed episodes of double vision.  His headaches are usually daytime and localize behind the eyes.  He also developed weight gain, hypertension, fatigue, proximal muscle weakness, and hyperglycemia.  These symptoms worsened over the past six months and prompted a complete evaluation. 

He was found to have an AM cortisol= 23.4 mcg/ml, a 24 hr urine cortisol= 333 mcg/24hrs, and an ACTH= 169 pcg/ml (normal= 9-52).  The results of a dexamethasone test were not considered reliable because of inaccurate urine collection.  An MRI (without gadalinium) demonstrated no adenoma, but some rightward deviation of the pituitary stalk. 

A transphenoidal pituitary resection was performed.  Post-operatively the patient developed diabetes insipidus and was placed on ddAVP. 

A post-operative MRI was consistent with a small surgical defect in the left pituitary gland with persistent mild deviation of the infundibulum to the right.  One day post-operatively, the AM cortisol was 18.6 mcg/dl. 

The final pathology report read:

1. PITUITARY TUMOR (RESECTION): ANTERIOR PITUITARY TISSUE (ADENO-HYPOPHYSIS). SEE NOTE.

2.  PITUITARY TUMOR (BX): CONSISTENT WITH NEURO-HYPOPHYSIS, NEGATIVE FOR TUMOR.

3,4.   PITUITARY TUMOR (BX.):  HEMORRHAGE AND MINUTE FRAGMENT OF TISSUE SUGGESTIVE OF NEURO-HYPOPHYSIS.

Note: Although an immunohistochemical stain for ACTH is diffusely positive, reticulin stains performed on the frozen section control failed to show a pattern indicative of an adenoma.  Smears performed on this specimen at the time of surgery showed a mixed cell population. However, numerous Crooke's cells were noted.  These cells are often noted in non-neoplastic pituitary tissue adjacent to ACTH producing adenomas/microadenomas.

Two months after surgery, the patient reports that he feels no better.  He still has fatigue, weakness, and a difficulty controlling weight.  He also has hypertension similar to pre-op.  He reports difficulty sleeping, loud snoring, headaches, easy bruising, and blurred vision.  He is still using ddAVP to control frequent urination.

Past Medical History:
Hypertension for three years, wrist surgery for ruptured ligament 6 years ago.

Family History:
No family history of endocrine, neoplastic, cardiac diseases.  Both parents are alive and well.  Two children in good health. 

Social History
The patient works for a package delivery company splitting time between office and field.  No significant occupational exposures. He is married with two healthy children (4 and 2 years old).  He denies drug, alcohol, or tobacco abuse. 

Allergies
No known drug allergies.

Medications
Verapamil 240 milligrams, bid for 1 year

Review of Systems
Patient notes that he is frequently fatigued, and suffers from insomnia.  He denies chest pain, shortness of breath or abdominal pain.  He has noted that he bruises easily.  He feels as if his legs are sometimes swollen, feels a tingling sensation in his legs, and complains of a slight tremor in his hands.  The patient has a good appetite and normal bowel movements, but complains of polyuria.  He complains that his sleep is less than optimal.

Physical Exam and Hospital Course
The patient is in no acute distress.  He is 70 inches tall and weighs 194 lbs.

Vital Signs: blood pressure of 150/100, pulse regular at 72 bpm.  His face is symmetrical, but with mild swelling or moon facies and periorbital edema.  His pupils are equal, round and react to light in accommodation.  Visual fields have no defects. Extra-ocular movements are intact.  Sclera are white and conjunctiva are pink.  He does not have cervical or supraclavicular lymphadenopathy.  He has a humped back and supraclavicular fullness.  His cardiac exam reveals a normal S1 and S2 with a regular rate and rhythm.  His lungs are bilaterally clear to auscultation without wheezing or ronchi.  His abdomen is not distended and he does not demonstrate hepatosplenomegaly or lymphadenopathy.  Skin examination demonstrated multiple small bruises and violaceous striae, and 1+  peripheral edema.  Neurologically, cranial nerves 2 –12 are intact with movement in all extremities.  He has normal distal strength but mild decreased strength in the deltoids, psoas, and quadriceps.  There is decreased muscle mass in both calves.  Reflexes are normal.

Radiology:

Chest x-ray is normal except for minimal obesity.

Laboratory Studies:

Basic Chemistries were unremarkable except for Glucose= 135.  Extended chemistries including liver function tests were normal. CBC demonstrated:  WBC =16,400 with a normal differential, Hct= 37%, Platelets= 277, 000

Urine free cortisol: 283 mcg/24 hrs
ACTH: 146 pcg/ml (9-52)

Long (low and high dose) Dexamethasone Suppression:

Questions to consider:

1. Why may the patient still be hyper-cortisolemic after pituitary surgery?
   
   
2. Would any additional pre-operative information have been useful to determine the etiology of Cushing’s syndrome?
   
   
3. What further evaluation may elucidate the reason for the persistent clinical condition and laboratory abnormalities?
   
   
4. What further therapy should be considered?

Return to Top