1. Chronic Bilateral Slipped Capital Femoral Epiphysis as an Unusual Presentation of Congenital Panhypopituitarism due to Pituitary Hypoplasia in a 17 year-old female Sasigarn A. Bowden1, Kevin E. Klingele2 1Division of Endocrinology, 2Division of Orthopedics, Department of Pediatrics, Nationwide Children’s Hospital The Ohio State University College of Medicine, Columbus, Ohio, USA
Background: Slipped capital femoral epiphysis (SCFE) occurs primarily during the pubertal growth spurt and is most frequently seen in obese children. Only small percentage of SCFE (8%) is associated with endocrinopathies such as hypothyroidism or growth hormone deficiency. Short stature has been suggested as a screening test for endocrinopathy in SCFE.
Objective: To report an interesting case of a 17 year-old normal-statured female who was diagnosed with congenital panhypopituitarism due to pituitary hypoplasia at the presentation of SCFE. Design/Methods: Case report
Results: A 17.5 year-old Caucasian female presented with 3 month history of hip pain, difficulty walking, and found to have bilateral SCFE on hip X-ray. She was admitted to Orthopedic service for hip surgery. Endocrine service was consulted due to her delayed puberty and primary amenorrhea. Her mid parental height was 165 cm (75th%). Weight was 65 kg (75th%), height cm (25th%). Breast and pubic hair development were Tanner stage 1. Labs revealed low free T4 (0.5 ng/dL; normal ), slightly elevated TSH (7.97 uIU/mL; normal ), FSH 1.3 mIU/mL, LH <0.5 mIU/mL, estradiol <0.1 ng/dL. Pelvic ultrasound revealed no visualized uterus or ovaries. The finding of hypogonadotropic hypogonadism prompted the work up for hypopituitarism. ACTH stimulation test showed low peak cortisol (5.9 ug/dL). She was given stress dose of hydrocortisone prior to her surgery on hospital day 3. Subsequently, complete pituitary evaluation showed undetectable IGF-1 level (<25 ng/mL), low IGF-BP3 (1.7 mg/dL; normal ), low peak growth hormone (<0.1 ng/mL) on growth hormone stimulation test, delayed bone age at 13 years. Brain MRI showed pituitary hypoplasia, with no identifiable pituitary stalk and ectopic posterior bright spot. Her pelvic MRI showed no identifiable uterus and streak ovaries. She was treated with levothyroxine, hydrocortisone, growth hormone, and subsequent estrogen replacement therapy. Repeat pelvic ultrasound after 9 months of estrogen therapy revealed a small uterus and ovaries.
Conclusions: This case illustrates that the work up for hypopituitarism, especially adrenal insufficiency should be performed in patients with SCFE that have signs of endocrinopathy, to prevent potential complication of adrenal crisis during surgery. This case also demonstrates growth without growth hormone which resulted in a delay in diagnosis of congenital panhypopituitarism in this patient.
ABSTRACT
HPI: A 17.5 year-old Caucasian female presented with 3-4 month history of hip pain, difficulty walking, inability to bend down. Her hips were always rotated out. Walking was painful for her.
Mother reported that the family traveled significantly because they were crafters. Patient did not have a regular medical care until this illness.
Past medical history: Normal birth history and development. She was home-schooled because of her learning difficulties.
Family history: Father’s height 5’10”. Mother’s height 5’7”. Mid parental height is 66” (75th%)
Mother started menarche at age 12.
Grandmother started menarche at age 16.
Patient was the only child.
Positive family history of diabetes, thyroid disorders, heart disease.
Physical Exam: Wt 65 kg (75th%), Ht cm (25th%). BMI 26 kg/m2 (90th%).
General appearance: alert, oriented, adolescent female in no acute distress, except having pain when standing. No dysmorphic features.
Musculoskeletal exam: bilateral hip pain with hips held in external rotation. Internal rotation of hip causes increased pain. Pain with walking.
Shuffling gait secondary to tightness on hip girdle with hips continually in external rotation.
Patient was unable to lay on her back due to gravity pushing further on her hip causing more pain.
Tanner stage 1 for breast and pubic hair development.
CASE REPORT
Pelvic ultrasound revealed no visualized uterus or ovaries.
Free T4 was low at 0.5 ng/dL (normal )
TSH was slightly elevated at 7.97 uIU/mL (normal )
FSH 1.3 mIU/mL
LH < 0.5 mIU/mL
Estradiol < 0.1 ng/dL
The finding of hypogonadotropic hypogonadism prompted the work up for hypopituitarism.
ACTH stimulation test showed low peak cortisol of 5.9 ug/dL.
Undetectable IGF-1 level < 25 ng/mL
Low IGF-BP3 at 1.7 mg/dL (normal )
Growth hormone stimulation test (Arginine/Clonidine): growth hormone levels were all <0.1 ng/mL, consistent with complete growth hormone deficiency
Bone age was delayed at 13 years.
Brain MRI scan showed pituitary hypoplasia with no identifiable pituitary stalk and ectopic posterior bright spot.
Pelvic MRI scan showed no identifiable uterus and tiny cystic structures bilaterally in the lower pelvis representing streak ovaries.
DEXA scan: Lumbar spine BMD gm/cm2.
Z score of -2.2.
ADDITIONAL LAB INVESTIGATIONS AND IMAGING STUDIES
Patient was found to have panhypopituitarism secondary to pituitary hypoplasia.
She was given stress dose of hydrocortisone prior to her hip surgery.
She was treated with levothyroxine, hydrocortisone and growth hormone, and subsequent estrogen replacement therapy.
Repeat pelvic ultrasound after 9 months of estrogen therapy showed a small uterus and ovaries.
CLINICAL COURSE
Patient had normal stature for age.
Her brain MRI scan showed pituitary hypoplasia which is a congenital condition.
Her nonvisualized uterus and ovaries at diagnosis of panhypopituitarism suggests that this is a congenital condition.
Her growth hormone levels were undetectable on the GH stimulation test, indicating complete GH deficiency.
This case illustrates growth without growth hormone syndrome, suggesting that other growth factors have effects on growth.
Elevated insulin level seen in obesity with insulin resistance may play a role in growth.
This patient’s fasting insulin level was normal at 15 uU/mL.
INTERESTING POINTS
Bone age
This case illustrates that the work up for hypopituitarism, especially adrenal insufficiency should be performed in patients with SCFE that have signs of endocrinopathy, to prevent potential complication of adrenal crisis during surgery.
This case also demonstrates growth without growth hormone which resulted in a delay in diagnosis of congenital panhypopituitarism in this patient.
SUMMARY AND CONCLUSION
Slipped capital femoral epiphysis (SCFE), a potentially disabling hip disorder, occurs during the adolescent growth spurt.
The etiology of SCFE is unknown but obesity and subtle hormone imbalances have been postulated as major factors.
Most cases of SCFE are idiopathic in nature and frequently associated with obesity.
A small percentage ( %) of SCFE is associated with endocrinopathies, primarily hypothyroidism (40%) and growth hormone deficiency (25%).
All growth hormone deficient children with SCFE had been diagnosed with endocrine disorder before the development of SCFE.
Short stature has been suggested as a screening test for endocrinopathy in SCFE.
BACKGROUND
Brain MRI scan
Loder RT, Wittenberg B, DeSilva G. Slipped capital femoral epiphysis associated with endocrine disorders. J Pediatr Orthop. 1995;15(3):
Burrow SR, Alman B, Wright JG. Short stature as a screening test for endocrinopathy in slipped capital femoral epiphysis. J Bone Joint Surg Br. 2001; 83(2):
Geffner ME. The growth without growth hormone syndrome. Endocrinol Metab Clin North Am. 1996; 25(3):
4. Phillip M, Moran O, Lazar L. Growth without growth hormone. J Pediatr Endocrinol Metab 2002; 15 Suppl 5:
REFERENCES
Hip AP/LAT-BILAT: bilateral slipped capital femoral epiphyses. There is atypical sclerosis to the proximal metaphyses of both femoral heads which suggests chronic or subacute condition.
Patient was admitted to Orthopedic Service for hip surgery.
Due to her delayed puberty and primary amenorrhea, Endocrine Service was consulted.
INITIAL LABORATORY
Hip X ray after pinning
DEXA scan showed low bone density