A 75-year-old woman had tumors in her pituitary, thyroid and left adrenal gland. Plasma ACTH and cortisol levels were both mildly elevated. Both plasma ACTH and cortisol concentrations were partially suppressed by 1 mg of overnight dexamethasone suppression test, while both were inhibited with a dosage of 8 mg dexamethasone. Plasma ACTH and cortisol levels were increased in response to human CRH and desmopressin. Together with the observation of pituitary microadenoma, the patient had a pituitary ACTH-producing tumor. The patient, however, had no typical Cushingoid features, hypertension, or impaired glucose tolerance, suggesting that the tumor had an autonomic ACTH secretion that was insufficient for expressing clinical symptoms, the so-called preclinical Cushing's disease. A case of preclinical Cushing's disease is extremely rare. Further, the patient had thyroid papillary carcinoma and non-functioning adrenal tumor. Molecular genetic analysis demonstrated a polymorphism of the menin gene in the patient. Even without Cushingoid features in pituitary incidentaloma, we concluded that the elevated ACTH and cortisol levels should be followed up by CRH, desmopressin and dexamethasone suppression tests. This patient with preclinical Cushing's disease would be observed whether the physical conditions in the patient develop to overt Cushing's disease.
The most popular techniques for repairing large diaphragmatic defects involve the use of synthetic patches. We present an alternative approach using living tissue. We reviewed our cases of congenital diaphragmatic hernia (CDH) diagnosed within the first 24 h of life from 1991 to 2003. Toldt's fascia (TF) flap was used to repair defects that were too large to repair primarily even though the anterior rim of the diaphragm was present. After confirming that a small medial muscle remnant of the diaphragm was present, its mesothelial covering was incised, and the incision was extended to the TF far enough to create a flap sufficiently large to repair the defect. The TF flap, consisting of the small medial muscle remnant, TF, peritoneum, and retroperitoneal connective tissue, was mobilized carefully from the ipsilateral kidney and adrenal gland, and the repair completed with interrupted sutures using nonabsorbable material. We used this TF flap approach in seven of 43 patients with CDH. Two had right-sided CDH. Six survived. The mean size of the diaphragmatic defects in the seven TF flap cases was 5.43+/-0.53 x 3.86+/-1.07 cm, which was significantly larger than the defects in direct primary repair cases (3.40+/-0.77 x 2.03+/-0.59 cm) (p<0.01). The six survivors had good outcomes, and none of them have had recurrence of herniation or required any additional surgical intervention (mean follow-up period: 4.7 years). To the best of our knowledge, this is the first report of TF being used to repair large diaphragmatic hernias. Our technique is simple and has proven to be reliable for durable restoration of the diaphragm, suggesting that it could reduce the dependence on synthetic patch repair, which is associated with certain long-term complications.
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