We have managed 23 infants and children with severe tracheal stenosis due to congenital complete tracheal rings producing a long-segment stenosis of the trachea. Nineteen (83%) have survived this ...life-threatening cause of airway obstruction, 7 of whom also had pulmonary artery slings. Pericardial patch tracheoplasty facilitated by partial cardiopulmonary bypass is currently our preferred technique for surgical repair. Eighteen patients (78%) underwent operative intervention, 3 of whom (17%) have died since surgery. The mean follow-up is 4.5 years. Bronchoscopy is essential for preoperative diagnosis and accurate intraoperative incision of the trachea, and is critical for long-term postoperative airway management. The more distal lesions are associated with increased complications and a higher mortality rate.
Fifty-three patients with stage III (eight patients, 15%), stage IV (36 patients, 68%), or recurrent disease (nine patients, 17%) entered a study of simultaneous cisplatin, 60 mg/m2 day 1, ...fluorouracil (5-FU) infusion, 800 mg/m2 days 1 to 5, and radiation, 2 Gy days 1 to 5, every other week for a total of seven cycles (70 Gy in 13 weeks). Patient acceptance was high, with only two patients (4%) refusing to complete therapy. The median actual dose delivered was 88% of the planned dose for cisplatin, 78% for 5-FU, and 70 Gy for radiation. Weight loss of 10% or more and severe mucositis were the most common side effects (53% and 48% incidence, respectively). All patients were followed at least 1 year (median, 51 months). While the complete response rate (55%) seemed no better than that reported in other series, freedom of progression of regional disease (73%), and the survival of all patients (median, 37 months) were substantially improved. Only 33% of partial responders have failed regionally, while 15% of complete responders have failed regionally (P greater than .10), which indicates that clinical assessment of response was unreliable. Stage, the presence of N3 disease, and delivery of less than the median actual dose received of 5-FU (but not cisplatin) were significantly associated with failure. This regimen is feasible and tolerable in this difficult patient population. It generally requires no special forced feeding techniques. Survival results from this limited institution study appear better than those using sequential multimodality therapies. With such favorable regional control, this approach may offer an alternative in the future to radical surgery and radiation in resectable disease. More definitive evaluation seems warranted.
Williams syndrome (WMS) is a rare, genetically based syndrome associated with a hemideletion in chromosome 7 (7q11.22-23) and characterized by a unique constellation of somatic, brain, and cognitive ...features. Individuals with WMS demonstrate an unusual and uneven neuropsychological profile showing cognitive and visual spatial deficits juxtaposed with relative language preservation and excellent facial recognition.
A neuroanatomical hypothesis for these behavioral findings suggests predominant involvement of the dorsal portions of the hemispheres relative to the ventral portions, including preferential involvement of peripheral visual field cortical representations over central representation. Predominant involvement of magnocellular visual pathways, as opposed to parvocellular pathways, is also suggested by this hypothesis.
We examined primary visual cortical area 17 in the right and left hemispheres in 6 age- and sex-matched autopsy specimens from 3 WMS-affected brains (1 male and 2 females; mean SD age, 44 14 years) and 3 control brains (1 male and 2 females; mean age, 43 11 years).
Neurons in layers II, III, IVA, IVB, IVCalpha, IVCbeta, V, and VI were measured using an optical dissector method to determine possible differences between WMS-affected and control brains in cell-packing density, neuronal size, and neuronal size distribution.
We found abnormalities in peripheral visual cortex in WMS-affected brains, but not in magnocellular subdivisions. There was a hemisphere by layer IV interaction and a layer IV left hemisphere and diagnosis interaction in cell-packing density. Williams syndrome-affected brains showed increased cell-packing density in left sublayer IVCbeta and an excess of small neurons in left layers IVA, IVCalpha, IVCbeta, V, and VI.
Cell measurements differ in peripheral visual cortical fields of WMS, with significantly smaller, more closely packed cells in some layers on the left side. These cell-packing density and neuronal size differences may be related to visuospatial deficits in this population.
Thirty-six patients with persistent tracheocutaneous fistula (TCF) after pediatric tracheotomy were managed at Children's Memorial Hospital in Chicago between June 1987 and July 1992. Persistent TCF ...was managed with surgical excision and primary closure. The mean patient age was 5 years 7 months, and the mean duration between decannulation and fistula closure was 21 months. There were no major complications and four minor complications. While most surgeons advocate other techniques, we feel that excision with primary closure is the preferred method for persistent TCF. The technique requires an airtight tracheal closure with loose closure of the peristomal soft tissue. Careful preoperative evaluation, postoperative monitoring, and wound drainage are stressed.
Between 1982 and 1990, 15 children have undergone pericardial patch tracheoplasty, 13 for complete tracheal rings and 2 for acquired tracheal stenosis. Eleven had complete tracheal rings from the ...thoracic inlet or the cricoid to the carina. Diagnosis was by bronchoscopy (15), computed tomography (10), and magnetic resonance imaging (3). As first described by Idriss in 1984, the surgical technique uses a median sternotomy approach, extracorporeal circulation, bronchoscopic guidance, and the use of pericardium as a tracheal patch. There was one perioperative death of mediastinitis (operative survival, 93%). There were two late deaths caused by complications of tracheostomy (1) and direct laryngoscopy and bronchoscopy (DLB) (1). Follow-up is complete in 12 intermediate-term survivors and ranges from 0.4 to 8.2 years (mean, 4.2 +/- 2.9 years). Five children required multiple DLBs with dilation and excision of granulation tissue; one of these patients underwent tracheoplasty revision 5 months after the initial procedure for residual tracheal rings, and four of these children have had tracheostomies, two temporary and two currently (4 months, 7 years). Seven children had widely patent tracheas observed at follow-up DLB with little or no residual stenosis after tracheoplasty. All survivors are currently asymptomatic, except for the two children with tracheostomies. Pericardial patch tracheoplasty offers effective therapy with relatively low operative mortality for infants and children with severe long segment tracheal stenosis. On intermediate-term follow-up, nearly all survivors are essentially free of symptoms of residual tracheal stenosis.
Ventilating tracheal resectoscope Dunham, M E; Holinger, L D
Annals of otology, rhinology & laryngology,
09/1994, Letnik:
103, Številka:
9
Journal Article
Recenzirano
The ventilating tracheal resectoscope (Karl Storz) combines the advantages of a ventilating bronchoscope and the resecting cytoscope for removal of soft tissue from the airway. The resectoscope ...offers several distinct advantages over other methods of granulation tissue removal. The procedure is done under direct vision with magnification and illumination of a rod-lens optical system. Suprastomal granulation tissue is resected cleanly, without tearing or stripping adjacent mucosa. Hemorrhage is readily controlled with the coagulating mode. The resectoscope can also be used for removing benign tumors (such as recurrent respiratory papillomatosis) from the trachea and bronchi. Its use in the efficient removal of malignant tumors and subglottic soft tissue has also been described.
Five infants with long tracheal stenosis were operated upon by means of a pericardial patch tracheoplasty. The approach was through a median sternotomy with extracorporeal circulation for respiratory ...support. In four, the obstruction was due to complete rings; in the other, there was an associated tracheal trauma which had occurred during resuscitation. This patient requires prolonged stenting with a tracheostomy tube. All others are asymptomatic postoperatively, with the longest follow-up being 22 months. There were no deaths or infections. We conclude from this experience that median sternotomy provides an excellent approach to the trachea, that autogenous pericardium is advantageous, and that there is no need for prolonged tracheal stenting in most patients.
Ninety-five patients with squamous cell carcinoma of the head and neck were entered into a randomized study testing a two-week course of induction chemotherapy with methotrexate and leucovorin given ...prior to regional therapy. In addition, following regional therapy, patients randomized to chemotherapy were to receive similar methotrexate courses every three months for one year. Poor tolerance to this regimen after radiation and surgery led to a change in the chemotherapy following regional therapy to a combination of Adriamycin (Adria Laboratories, Columbus, Ohio) and cisplatin every three weeks for four cycles after the first 35 patients had been entered. Nine cases were ineligible and four lacked any follow-up data, leaving 82 analyzable cases. Using Cox regression analysis, no differences in the percentage of patients achieving disease control, the relapse-free survival, or the overall survival were identified between any treatment group. As has been described in many pilot studies of induction chemotherapy of head and neck cancer, chemotherapy responders had a more favorable disease-free survival than chemotherapy nonresponders in the total group of patients receiving adjuvant chemotherapy. However, correcting for imbalances in the expected three year disease-free survival of these patients, based on their disease site and stage, erased this difference, indicating tumor response to this regimen of chemotherapy is not an independent factor affecting disease outcome. The division of patients into arbitrary prognostic categories based on the expected outcome for each specific tumor site and stage proved to be a useful method for balancing treatment groups, given the multiple site-stage combinations within the upper aerodigestive tract. The defined prognostic categories were the single most sensitive predictors of relapse-free and overall survival.
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