Sublabial transseptal vs transnasal combined endoscopic microsurgery in patients with Cushing disease and MRI-depicted microadenomas.

OBJECTIVE: To assess whether the type of surgical approach to the sella (sublabial transseptal vs transnasal) affects surgical outcome among patients with Cushing disease. PARTICIPANTS AND METHODS: Both procedures were performed at our institution from January 1, 1995, through January 31, 2003. From a total of 106 patients with Cushing disease who had had surgery, we identified 42 adults undergoing an initial surgery, with microadenoma (<1 cm) determined by magnetic resonance imaging and a minimal follow-up of 3 months. RESULTS: We identified 21 patients (4 male, 17 female) for sublabial transseptal transsphenoidal microsurgery. Mean+/-SD age was 45.0+/-12.9 years (range, 27.0-69.0 years); median duration of symptoms was 2.5 years (range, 1.5-7.5 years). Median follow-up was 1.0 year (range, 0.3-11.0 years). We identified another 21 patients (5 male, 16 female) for endoscopic transsphenoidal microsurgery whose mean+/-SD age was 43.8+/-14.1 years (range, 19.0-70.0 years); median duration of symptoms was 2.4 years (range, 0.2-6.0 years). Median follow-up was 2.5 years (range, 0.3-8.7 years). Complications (cerebrospinal fluid leak and transient diabetes insipidus) and cure (86% initial cure; 76% relapse-included cure) were equivalent between the 2 procedures. However, the endoscopic approach was associated with shorter anesthesia time, less blood loss, and shorter hospital stays. CONCLUSION: The endoscopic transnasal surgical approach led to shorter total anesthesia time, less blood loss, and shorter hospital stay when compared with the sublabial transseptal approach. However, we found no difference between the 2 surgical procedures with respect to cure or complications, suggesting that outcome is more closely related to the experience of the operating team.

Selective peripheral denervation for the treatment of intractable spasmodic torticollis: experience with 168 patients at the Mayo Clinic.

OBJECT: Selective peripheral denervation is currently the primary surgical treatment for intractable cervical dystonia. The authors assessed preoperative factors to determine which, if any, correlated with outcomes in patients with torticollis who had undergone this procedure. METHODS: The records of 168 consecutive patients who had undergone selective peripheral denervation for cervical dystonia between 1988 and 1996 at the Mayo Clinic were reviewed. There were 89 women (53%) and 79 men (47%) with a mean age of 53.4 years. Selection of muscles for denervation was based on the patient's clinical presentation and electromyography mapping results. The most common torticollis vectors were rotational in 141 patients (84%) and laterocollis in 59 (35%). Seventy patients (42%) presented with combined vectors. The technique used to remedy both conditions involved denervation of the ipsilateral posterior cervical paraspinal and splenius capitis muscles. Denervation of the sternocleidomastoid muscle was performed on the contralateral side for rotational torticollis and on the ipsilateral side for laterocollis. A rigorous physical therapy program followed surgery. At the 3-month postoperative evaluation, 125 patients (77%) of the 162 who were available for follow up had moderate to excellent improvement in their head position, and pain was moderately to markedly improved in 131 patients (81%). The long-term follow up lasted a mean of 3.4 years and was undertaken in 130 patients. The original level of moderate to excellent improvement in head position and pain was retained in at least 71 patients (70%). Outcome was not predicted by preoperative head position, severity of abnormal posture of head, symptom duration, presence of tremor or phasic dystonic movements, or failure to respond to botulinum toxin treatment. Five patients recovered from postoperative complications including one myocardial infarction, one pulmonary embolism, and three respiratory failures. Three patients suffered from persistent C-2 distribution dysesthesias and three from slight shoulder weakness; one had a wound infection, and one died of respiratory arrest. CONCLUSIONS: Selective peripheral denervation is an effective method of achieving lasting improvement of dystonia in most patients with intractable torticollis.

The Pituitary in Gigantism.

To compare the pituitary pathology of gigantism to that of acromegaly, 19 surgically resected lesions were studied from 10 males and 9 females, ages 13-49 (mean, 19 yr) with excessive height (>/=95th percentile), onset of disease prior to puberty, elevated growth hormone (GH) levels despite glucose suppression, and a pathologically confirmed GH-producing pituitary mass. One patient had MEN-I. The lesions included 18 adenomas and 1 case of pure hyperplasia. The median, mean, and range of serum GH and prolactin (PRL) levels were 64, 235, 5-1000 ng/mL and 47, 146, 29-770 ng/mL, respectively. Of the 8 adenoma specimens accompanied by nontumoral pituitary (i.e., tissue wherein the presence of hyperplasia was assessable), 3 (37%) demonstrated both. Of the 18 tumors, 78% were macroadenomas and 22% were grossly invasive; their immunophenotypes included GH (5%), GH and PRL (19%), and GHPRL and a glycoprotein hormone, usually TSH and/or a-subunit (76%). Of the 10 adenoma-containing lesions subject to electron microscopy (EM), 2 consisted of GH cells alone; 2 of mammosomatotroph (MS) cells alone; 1 of GH and MS cells; 1 of GH and PRL cells; 2 of GH, PRL, and MS cells; 1 of GH, PRL, and glycoprotein cells; and 1 was a subtype 3 adenoma. Ultrastructurally, GH cells and/or MS cells predominated in these lesions. Immuno-EM of one CH and PRL cell and of one GH-PR-MS tumor showed GH and PRL to be present not only in single cells but within the same granules. Nine of 12 adenoma-associated lesions subject to combined in situ hybridization (ISH) and immunostaining showed double labeling for PRL (or GH) mRNA and for GH (or PRL), respectively, features indicating MS differentiation. In the 4 lesions exhibiting hyperplasia, either alone (1) or in association with adenoma (3), EM showed MS cells in 3, and immuno-EM as well as combined immunohistochemistry and ISH showed double labeling for GH and PRL in both of the 2 cases studied. In summary, although in terms of their tinctorial characteristics and tumor size, the lesions of giants resemble those of acromegalics, those of the former are less often invasive and glycoprotein hormone containing, and more often contain ultrastructurally distinctive MS cells. The high frequency of adenoma with hyperplasia (37%) and the occurrence of hyperplasia alone (6%) is of particular notice since this finding is rare in patients with acromegaly. Hyperplasia is, however, seen in ectopic GH-releasing hormone production and the McCune-Albright syndrome. We conclude that the presence of MS is not rare in the pituitary lesions of patients with gigantism. Their presence may be a reflection of either hypothalamic dysfunction or of an intrinsic abnormality of pituitary cells,