Substernal goiter. Report of 212 cases

AIM OF THE STUDY: The aim of this retrospective study was to report the results of the surgical treatment in a series of 210 patients operated on for substernal goiters. PATIENTS AND METHOD: From 1982 to 1996, 210 patients with substernal goiters, including 80% of women, were operated on via a cervical approach in 208 cases, via a sternotomy in two cases. Two patients with operative contra-indications were not operated on. Twenty-five were operated on for a substernal recurrence of a goiter. In 160 cases, extraction of the substernal portion was easy. In 48 cases, removal of the substernal portion was facilitated by the discovery of the recurrent nerve at its entering into the larynx and a downward dissection of the tracheal attachments of the lobe. The complete dissection of the cervical portion made easier the ascension of the substernal portion even in very large substernal components. RESULTS: Three papillary carcinomas were diagnosed. A transient laryngeal nerve palsy occurred in 7.2% of the patients and a transient hypoparathyroidism in 13.4%, A definitive laryngeal nerve palsy occurred in 1.2% of the patients, and a persistent hypoparathyroidism in 2.1%. Of the 25 patients who underwent surgery for recurrence of a goiter, three (12%) developed a transient laryngeal nerve palsy, one (4%) a permanent nerve palsy, four (20%) a transient hypoparathyroidism and one (4%) a persistent hypocalcemia. CONCLUSION: CT scan and MRI are the best explorations to evaluate intrathoracic extension of substernal goiters. Thyroidectomy was performed via a cervical incision in 208 patients and via a sternotomy in two patients only. The complete dissection of the cervical portion with discovery of the recurrent nerve at its entering into the larynx, facilitates the ascension of the substernal portion even in very large substernal goiters.     

Turning Data Into Information: Opportunities to Advance Rehabilitation Quality,Research, and Policy

Attention to health care quality and safety has increased dramatically. The internal focus of an organization is not without influence from external policy and research findings. Compared with other specialties, efforts to align and advance rehabilitation research, practice, and policy using electronic health record data are in the early stages. This special communication defines quality, applies the dimensions of quality to rehabilitation, and illustrates the feasibility and utility of electronic health record data for research on rehabilitation care quality and outcomes. Using data generated at the point of care provides the greatest opportunity for improving the quality of health care, producing generalizable evidence to inform policy and practice, and ultimately benefiting the health of the populations served.     

Prolonged administration of secretin to normal rats increases biliary proliferation and secretin-induced ductal secretory activity

Background and aim

Cholangiocyte proliferation is coordinately regulated by a number of gastrointestinal hormones/peptides, some of which display stimulatory effects and some have inhibitory actions on cholangiocyte proliferation. Enhanced biliary proliferation [for example after bile duct ligation (BDL) and partial hepatectomy] is associated with increased expression of secretin receptor (SR), cystic fibrosis transmembrane conductance regulator (CFTR) and Cl^–/HCO₃^– anion exchanger 2 and secretin-stimulated ductal secretion, whereas loss/damage of bile ducts [for example after acute carbon tetrachloride (CCl₄) administration] is associated with reduced secretin-stimulated ductal secretory activity. There is growing information regarding the role of gastrointestinal hormones the regulation of biliary growth. For example, while gastrin, somatostatin and serotonin inhibit bile duct hyperplasia of cholestatic rats by downregulation of cAMP signaling, secretin has been shown to stimulate the proliferation of normal mice by activation of cyclic adenosine 3'',5''-monophosphate (cAMP)-dependent signaling. However, no information exists regarding the stimulatory effects of secretin on biliary proliferation of normal rats. Thus, we evaluated the in vivo and in vitro effect of secretin on biliary proliferation, the expression of markers key of ductal secretion and secretin-stimulated ductal secretion.

Methods

Normal male rats were treated with saline or secretin (2.5 nmoles/kg BW/day by osmotic minipumps for one week). We evaluated: (I) intrahepatic bile duct mass (IBDM) in liver sections and PCNA expression in purified cholangiocytes; (II) SR and CFTR mRNA expression and secretin-stimulated cAMP levels in purified cholangiocytes; and (III) secretin-stimulated bile and bicarbonate secretion in bile fistula rats. In vitro, normal rat intrahepatic cholangiocyte lines (NRIC) were treated with BSA (basal) or secretin (100 nM) for 24 to 72 hours in the absence/presence of a PKA or a MEK inhibitor before evaluating proliferation by MTS assays.

Results

Prolonged administration of secretin to normal rats increased IBDM and PCNA expression in purified cholangiocytes compared to saline-treated normal rats. Also, secretin increased the expression of proteins (SR and CFTR) that are key in the regulating ductal secretion and enhanced secretin-stimulated cAMP levels and bile and bicarbonate secretion. In vitro, secretin increased the proliferation of NRIC, increase that was prevented by PKA and MAPK inhibitors.

Conclusions

We have demonstrated that secretin stimulates both in vivo and in vitro biliary proliferation and secretin-stimulated ductal secretory activity in normal rats. We suggest that the stimulatory effect of secretin on biliary proliferation and secretion may be important for preventing biliary dysfunction during ductopenic disorders.     

The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: clinical description and genetics

The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is characterized by congenital aplasia of the uterus and the upper part (two-third) of the vagina. It may be isolated (type I) or associated with other malformations (type II or MURCS association). These latter involve the upper urinary tract, the skeleton and, to a lesser extent, the otologic sphere or the heart. The incidence of MRKH syndrome has been estimated as 1 in 4500 women. The prime feature is a primary amenorrhea in women presenting otherwise with normal development of secondary sexual characteristics and normal external genitalia. However, the vagina is reduced to a vaginal dimple with variable depth. The ovaries are normal and functional as well as the endocrine status. Karyotype is 46,XX, with no visible chromosome modification. The phenotypic manifestations of MRKH syndrome overlap with various other syndromes or malformations and thus require accurate delineation as well as differential diagnosis. For a long time, the syndrome has been considered as a sporadic anomaly, but increasing familial cases now support the hypothesis of a genetic cause currently under investigation. The syndrome appears to be transmitted as an autosomal dominant trait with incomplete penetrance and variable expressivity.     

Surgical Management of Carotid Body Tumors

Background  Carotid body tumors (CBT) should be considered when evaluating every lateral neck mass. Methods  A retrospective study was conducted of 52 patients with 57 CBT. The surgical approach and complications were reviewed. All patients were operated on without preoperative embolization. Results  Multifocal paraganglioma (PG) were detected in six cases. A succinate dehydrogenase subunit D (SDHD) mutation was discovered in four patients. Vascular peroperative complication occurred in one case. Vascular reconstruction was decided peroperatively in five cases (8.8%). Vascular reconstruction was 0% for Shamblin 1 or 2 tumors, but 28.5% for Shamblin 3. A postoperative nerve paresis was reported in 24 patients (42.1%) and vagal nerve paralysis persisted in four cases (7.01%). The rate of serious complications, e.g., permanent nerve palsy, preoperative and postoperative complications, was 14.03%; it was 2.3% for Shamblin 1 or 2 tumors and 35.7% for Shamblin 3. One patient had malignant PG with node metastasis and was not referred for radiotherapy. No recurrence or metastasis was reported after 6-year follow-up. Conclusion  Early surgical treatment is recommended in almost all patients after preoperative evaluation and detection of multifocal tumors. Surgical excision of small tumors was safe and without complication, but resection of Shamblin 3 tumors can be challenging. Routine preoperative embolization of carotid body paragangliomas is not required.     

The anatomic bases of the surgical treatment of pharyngo-laryngeal cancers

It is essential to recognize certain laryngeal and hypopharyngeal landmarks in order to choose the most appropriate surgical approach for excision of a tumor in any given site: that the larynx has two embryological origins, branchial and non-branchial, separated by the glottis, that the cartilaginous skeleton and the ossification of the thyroid cartilage provide a means for the spread of endolaryngeal cancer, that the pre-epiglottic region is divided in two by a medial wall and is closely connected to the endolarynx through the hollow vascular channels of the fibro-cartilage of the epiglottis, that the connective intralaryngeal tissue - conus elasticus, anterior commissure ligament, conoid ligament, and the articular capsule of the crico-arytenoid joint - must be taken into consideration to explain the spread of the tumor, that there exists the concept of arytenoid unity, that element of the branchial larynx which must be conserved in any functional oncologic surgery to the larynx and pharynx.