A case presentation of a patient of acromegaly who eventually developed panhypopituitarism and visual field defect along with subacute apoplexy.

1. Presented by
Dr. Lala Shourav Das
DEM Student
Department of Endocrinology
BIRDEM
Crisis in a acromegaly patient

2. Particulars of the patient:
 Name: Mr. Z
 Age: 40 years
 Sex: Male
 Marital status: Unmarried
 Occupation: Businessman
 Address: Golapgonj, Sylhet
 DOA: 29th July, 2017

3. Chief complaints:
 Progressive enlargement of the body for 1½ year.
 Generalized weakness for 6 months.
 Intermittent fever for 2 weeks.
 Headache and blurred vision for last 7 days.

4. According to the statement of patient, he was reasonably well 1½ year
back. Since then, he was experiencing slow and progressive
enlargement of body, mainly his head, hands, feet and face including
lower jaw, leading to chewing difficulty.
He was also suffering from generalized weakness associated with
increased thirst, increased frequency of micturition and excessive
sweating for last 6 months. It was not associated with any weight loss,
fever, cough, loss of appetite, altered bowel habit.
History of present illness:

5. He was then diagnosed as a case of Diabetes mellitus, treated by local
physician with oral anti-diabetic medication and was on irregular follow
up.
For last 2 weeks, he developed fever which was low grade, highest
recorded temperature was 101°F with afebrile periods in between. Fever
was associated with significant generalized weakness, decreased
appetite and limitation of daily physical activity.
History of present illness (Cont.):

6. Fever was not associated with cough, burning sensation during
micturition, joint pain, rash, palpitation, loss of consciousness,
convulsion, chills and rigor or any focal symptoms and was relieved by
taking antipyretic.
He was initially treated in a hospital outside BIRDEM as a case of viral
fever with uncontrolled DM along with suspicion of acromegaly. While
staying there, he developed headache with blurred vision, which was
associated with decreased appetite and few episodes of vomiting.
During that period, he was referred to BIRDEM.
History of present illness (Cont.):

7. History of present illness (Cont.):
Headache was diffuse, dull aching in nature, moderate in intensity, not
associated with aura, without any diurnal variation, not aggravated by
coughing, straining or change of posture.
Headache was associated with significant visual difficulty. On query
patient described visual difficulty was limiting his activities like reading,
watching televisions, walking through open doors etc. Vomitus contains
freshly ingested food particles, was not blood mixed and no association
to any relevant symptoms.

8.  History of past illness: Nothing significant.
 Personal history: Patient is non smoker, non alcoholic.
 Family history: Nothing contributory.
 Social economic history: Lower middle class socio-economic status.
 Travel history: Nothing significant.

9. Drug History:
 Inj. Regular Human Insulin (100u)
 Inj. Ondansetron (8mg)
 Cap. Omeprazole (20mg)
 Tab. Paracetamol (500mg)
 Tab. Bromazepam (3mg)

10. General Examination:
 Appearance: Large coarse face, protruded jaw, prominent supra-orbital
ridge. Nose, lips and ears were slightly enlarged.
 Hands and feet were large, warm, sweaty. Fingers were spade like.
 Skin was thick, greasy and sweaty. Acanthosis nigricans was present.
 Tongue was slightly enlarged.
 Voice was husky.
 Body Built: Average
 Co-operative
 BMI: 21.6 kg/m2

11. General Examination:
 Anemia: Absent
 Jaundice: Absent
 Cyanosis: Absent
 Clubbing: Absent
 Koilonychia: Absent
 Leukonychia: Absent
 Edema: Absent
 Dehydration: Moderately
dehydrated
 Pulse: 98 beats/min
 Blood pressure: 110/70 mmHg
 Respiratory Rate: 18 breaths/min
 Temperature: 100 °F
 Lymph node: Not palpable
 Hair Distribution: Coarse body hair,
Baldness present
 Thyroid Gland: Not enlarged
 Gynecomastia: Absent
 Bed side urine: Albumin: Nil,
Sugar: ++
Acetone: Nil

12. Patients face from front and side (taken with patient’s permission)

13. Patient’s hands and foot (taken with patient’s permission)

14. Systemic Examination:
Nervous System:
 Higher psychic function: Normal.
GCS: 15/15.
 Cranial nerve examination:
Optic nerve & other eye findings:
 Visual acuity: Impairment of
vision (Rt:6/36, Lt: 6/24).
 Color vision: Intact.
 Field of vision by confrontation
test: Loss of peripheral vision in
both eye.
 Anterior segment: Bilateral early
cataract.
 Post. segment: Fundoscopy
normal
 Rest of the cranial nerves were
intact.

15. Nervous System: (Continued)
 Motor function: Normal, no sign of proximal myopathy.
 Sensory function: Intact,
Phalen's maneuver: No paresthesia in median nerve distribution.
Tinel's sign: No tingling sensation in median nerve distribution.
 Ulnar nerve thickening: Absent.
 Cerebellar signs: Absent.
 Signs of meningeal irritation: Absent.

16. Systemic Examination:
Alimentary System:
Inspection: Tongue, lips and jaws were enlarged. Lower jaw was
slightly protruded with malocclusion of teeth.
Palpation: Liver and Spleen were not palpable. Kidney was not
ballotable. Rest of the findings are normal
Percussion: Normal Findings.
Auscultation: Normal Findings.

17. Systemic Examination:
Cardiovascular System:
Pulse: 98 beats/min
Blood pressure: 110/70mmHg
Apex beat on left 5th Intercostal space, medial to mid clavicular line.
Rest of the findings were within normal limit.
Respiratory System: No abnormality detected
Musculoskeletal System: No abnormality detected
Genitourinary System: No abnormality detected

18. Salient Feature
Mr. Z, 40 years old patient presented with complaints of slowly
progressive enlargement of body, especially his head, hands, feet and
face including lower jaw for 1½ year. He was diagnosed and treated as a
case of DM for last 6 months due to generalized weakness. For last 2
weeks, he was suffering from intermittent fever without any focal sign,
associated with more pronounced generalized weakness limiting daily
physical activity. Later, It became associated with diffuse, dull aching,
intermittent headache with progressive visual difficulty in both eyes,
few episodes of vomiting and decrease in appetite for last 7 days.

19. Salient Feature (Cont.)
Headache was moderate in intensity, without any diurnal variation or
relation with change of posture. Visual difficulty limited his visual
acuity and field of vision. On general examination, patient had features
suggestive of acromegaly. Patient was febrile (100°F), moderately
dehydrated, Pulse: 98b/m, BP: 110/70mmHg. On CNS examination,
patient had bitemporal hemianopia with decreased visual acuity and
early cataract (B/E) with normal fundus. Other systemic examination
revealed normal findings.

20. Provisional Diagnosis
 Acromegaly due to pituitary adenoma
 Enteric Fever
 Diabetes Mellitus

21. Differential Diagnosis
 Pituitary apoplexy (Subacute presentation)

22. Investigations

23. X-ray Skull lateral view (from outside)
Findings:
• Sella appears deeper.
• ? Pituitary adenoma
• Impression: ? Acromegaly

24. Hormonal Investigations:
Investigation (30.7.17) Value
Basal growth hormone 40 ng/ml (0.05-5.0ng/ml)
Basal cortisol (9am)
(Before starting replacement)
86.79 nmol/L (101.2 – 690nmol/L)
ACTH 8.60 pg/ml (8.3-57.8 pg/ml)
TSH 0.41 uIU/ml (0.47-5.01uIU/ml)
FT4 11.47pmol/l (9.14-23.18pmol/l)
Testosterone 1.04 ng/ml (2.8-14.0 ng/ml)
LH 0.58 mIU/ml (0.57-12.07 mIU/ml)
Prolactin 30 mIU/L (< 425 mIU/L)

25. Investigations
MRI of Sella and Perisellar region

26. MRI of Sella and Perisellar region (with contrast)

27. MRI Findings:
Pituitary Region:
A) On precontrast scan shows, large lobulated mass measuring about 28x25 mm in Sella and
supra sellar region with both Parasellar and subsellar extension abutting the optic chiasma.
B) The lesion shows heterogeneously iso to hyper intense in T1WI and hyper intense on
T2WI/FLAIR.
C) Post contrast shows ring enhancement of the lesion
D) Cystic changes maybe tumor necrosis, hemorrhage or cystic degeneration.
E) Skull: Sella appears to be enlarged on size with erosion of the floor and anterior wall.
3rd and both lateral ventricles are mildly dilated.
No focal abnormality is seen in brainstem or cerebellum.
Impression: Sellar and supra sellar mass with both parasellar and subsellar extension,
most likely pituitary macroadenoma with intratumoral hemorrhage and necrosis.

28. Routine Investigations (29.07.17)
CBC 29.07.17
Hb 11.5 gm/dl
Total Count 5960 /cmm
Differential
Count
N: 76.4 %, L: 16.6 %
Platelet Count 150000 / cmm
S. Creatinine Blood Urea
29.07.17 1.4 mg/dl 36 mg/dl
31.07.17 0.9 mg/dl

29. S. Electrolyte (mmol/l)-
Date Na K Cl TCO2 Ca2+ PO4
29.07.17 130 3.7 101 18 8.5mg/dl 4 mg/dl
31.07.17 135 3.5 99 24
Glucose Profile
(mmol/L)
FBS 2HABF AL AD HbA1C
30.07.17 17.4 23.7 23.7 26.6 18.1 %

30.  Urine R/E (30.07.17): Normal
 CXR-P/A (29.07.17): Normal findings
 Blood C/S (31.07.17): No growth
 Urine C/S (31.07.17): No growth
 Triple Antigen (30.07.17): Normal Titre
 ECG (29.07.17): Within normal limit
 USG of W/A (31.07.17): Normal findings

31. Investigations (29.07.17) value
AST 26 U/l
ALT 16 U/l
S. bilirubin 1.2 mg/dl
Total protein 64 gm/l
S. albumin 35 gm/l

32. Hospital Course
 On 2nd day of admission, patient suddenly developed hypotension (BP:
80/60 mmHg) with mild alteration of consciousness (GCS: 14/15).
Hydrocortisone IV replacement was started.
 Patient’s general condition improved and became afebrile after starting
hydrocortisone replacement.
 Headache relieved slightly but visual disturbance persisted.

33. Ophthalmological Consultation
 Posterior sub capsular cataract on both eye, Fundus looks normal.
 Perimetry couldn’t not done as patient was not able to clinically stable
& due to photophobia.
 Humphrey’s visual field analysis (HVFA) of both eye was advised after
improvement of patient’s condition.

34. Final Diagnosis
 Pituitary macroadenoma (Acromegaly) with apoplexy leading to
panhypopituitarism (Hypogonadotropic hypogonadism,
secondary adrenal insufficiency, secondary hypothyroidism)
 AKI and electrolyte imbalance
 DM
 PSC Cataract (B/E)

35. Treatment:
 MNT
 Inj. Regular + Intermediate acting human
insulin regimen
 Inj. Hydrocortisone (100mg) 6 hourly initially,
for replacement.
 Tab. Cabergoline (0.5mg)
 Tab. Thyroxine (50mcg)
 Inj. Ceftriaxone (2gm)
 Inj. Ondansetron (8mg)
 Inj. Omeprazole (40mg)
 Tab. Paracetamol (500mg)

36. Referral
 For timely management of progressive visual difficulty, patient was
shifted to Neurosurgery department of BSMMU for neurosurgical
intervention.

37. Follow up
Patient has been advised to follow up after 1 week after surgery in
Endocrinology OPD with:
 FBS, 2HABF, 2HAL, 2HAD
 S. Creatinine
 S. Electrolyte
 S. IGF-1 Level
 S. FT4
 DEXA Scan
 Colonoscopy and Testosterone replacement (Planned)

38. Follow Up (Cont.)
 To know further update, we contacted with patient over phone.
 We came to know that, transphenoidal surgery was done on BSMMU
neurosurgery department resulting in significant improvement of vision.
 His urine volume was normal in post-operative period and it was
uneventful. Later, he was discharged on request with proper
medication.
 Patient is irregular in follow up due to financial constrains & travel
issues. We have advised him to take regular glucocorticoid replacement
and encouraged him to attend follow up in our OPD.

39. Take Home Message
 Patient with vision threatening pituitary adenoma should
undergo early neurosurgical management to save the vision.
 Before any surgery in pituitary adenoma, adrenal insufficiency
should be evaluated and managed properly to prevent adverse
outcome.

40. Acknowledgement
 Department of Ophthalmology, BIRDEM
 Department of Neurosurgery, BSMMU