Trans-nasal endoscopic holmium: YAG laser correction of choanal atresia

Five patients with choanal atresia were treated, using a trans-nasal endoscopic Holmium:YAG laser. The age of patients ranged from 15 months to 53 years. The nasal endoscope provides excellent visualization. The advantage of the Holmium:YAG laser is its fibre-optic delivery, allowing the laser to be hand-held, giving greater precision. Good nasal airways were achieved in four out of five cases with a single treatment. The procedure was found to be safe, quick, with minimal morbidity and a shorter in-patient stay.     

Pulmonary atresia with intact ventricular septum: long-term results of "one and a half ventricular repair"

BACKGROUND: Between 1982 and 1984, we successfully performed "one and a half ventricular repair" using a Glenn shunt for 3 patients with pulmonary atresia with intact ventricular septum. Here we review the 10-year follow-up results. METHODS: In these patients, the preoperative Z scores of the tricuspid valve diameters ranged from -5.2 to -6.5. Right ventricular outflow tract reconstruction combined with a Glenn shunt were performed in all patients. Cardiac catheterization was done at least 10 years post-operatively. RESULTS: All 3 patients have maintained New York Heart Association functional class I status for more than 10 years. Angiography in 2 patients confirms sufficient left pulmonary artery pressure with pulsatile blood flow and good right ventricular contraction. A pulmonary arteriovenous fistula has developed in 1 patient. CONCLUSIONS: Although the lower limits of the tricuspid valve diameter for "one and a half ventricular repair" using a cavopulmonary shunt have not yet been determined, we successfully performed this procedure in 3 patients with severely hypoplastic right ventricles and tricuspid valve diameter Z scores of less than -5.0. The results up to 10 years postoperatively are acceptable.     

Potential pitfalls in the work-up and diagnosis of choanal atresia

To increase an awareness of the developmental anatomy of the nasal cavity as it applies to the radiologic work-up of choanal atresia and frontoethmoidal cephaloceles, we report two cases that demonstrate potentially serious imaging pitfalls. Two neonates with nasopharyngeal obstruction were imaged with CT and MR. Both patients had surgically proved bilateral bony choanal atresia. In addition to choanal atresia, CT showed a radiolucent, or nonossified cribriform plate and mucoid secretions within the nasal fossa, adjacent to the cribriform plate, which approximated the attenuation of brain parenchyma. In one of the patients, a preoperative diagnosis of nasopharyngeal encephalocele resulted in surgical exploration. At surgery, however, the cartilaginous cribriform plate was found to be intact.     

Repair of left ventricular aneurysm: long-term results of linear repair versus endoaneurysmorrhaphy

BACKGROUND: Recently, endoaneurysomorrhaphy has been proposed as a more physiologic repair of postinfarction left ventricular aneurysm than is linear repair. There are only a few studies comparing the short-term and long-term results of the two techniques. METHODS: Clinical outcomes and echocardiographic measurements of left ventricular volume and sphericity in 27 patients who underwent endoaneurysmorrhaphy were compared with those in 20 patients who had linear repair. RESULTS: The two groups were matched with respect to age, gender, comorbid risk factors, functional class, urgency of the operation, and concomitant procedures. Preoperatively, left ventricular ejection fraction was lower in the endoaneurysmorrhaphy group (0.25 +/- 0.08 versus 0.30 +/- 0.09; p = 0.03). Follow-up was available in 44 patients (94%) and ranged from 2 to 86 months (mean, 41.0 +/- 26.5 months). Thirty-day operative mortality, perioperative complications, 5-year survival, and freedom from cardiac death were similar. Early postoperative percentage increase in left ventricular ejection fraction was greater after endoaneurysmorrhaphy (0.51 +/- 0.64 versus 0.18 +/- 0.48; p = 0.036). Long-term functional improvement was significantly better in the endoaneurysmorrhaphy group: At the time of last follow-up, 88% of patients were in New York Heart Association class I/II, compared with 53% after linear repair (p = 0.01). There were no measurable differences between the groups with respect to left ventricular ejection fraction (0.28 +/- 0.11 versus 0.27 +/- 0.11; p = 0.90), left ventricular volume (171.6 +/- 59.1 versus 169.9 +/- 54.4 mL; p = 0.94), and sphericity index (0.61 +/- 0.09 versus 0.61 +/- 0.12; p = 1.0). CONCLUSIONS: Despite having a similar effect on left ventricular geometry, endoaneurysmorrhaphy resulted in a greater increase in postoperative left ventricular ejection fraction and a substantially improved long-term clinical outcome.     

Third-degree obstetric perineal tear: long-term clinical and functional results after primary repair

BACKGROUND: This study was designed to investigate the long-term clinical and anorectal functional results after primary repair of a third-degree obstetrical perineal rupture. METHODS: One hundred and fifty-six consecutive women who had a primary repair of a third-degree perineal rupture were sent a questionnaire and asked to undergo anorectal function testing (anal manometry, anorectal sensitivity, anal endosonography and pudendal nerve terminal motor latency (PNTML)) RESULTS: Some 117 women (75 per cent) responded. Anal incontinence was present in 47 women (40 per cent); however, in most cases only mild symptoms were present. In 40 women additional anorectal function tests were performed and compared with findings in normal controls. Mean(s.d.) maximum squeeze pressure (31(15) versus 63(17) mmHg, P< 0.001) was decreased and first sensation to filling of the rectum (88(47) versus 66(33) ml, P=0.03) and anal mucosal electrosensitivity (4.7(1.7) versus 2.5(0.8) mA, P=0.003) were increased compared with values in normal controls. In 35 women (88 per cent) a sphincter defect was found with anal endosonography. Factors related to anal incontinence were the presence of a combined anal sphincter defect (relative risk (RR) 1.7 (95 per cent confidence interval (c.i.) 1.1-2.8)) or subsequent vaginal delivery (RR 1.6 (95 per cent c.i. 1.1-2.5)). CONCLUSION: Anal incontinence prevails in 40 per cent of women 5 years after primary repair of a third-degree perineal rupture. The presence of a combined sphincter defect or subsequent vaginal delivery increase the risk of anal incontinence.     

Mitral valve repair vs replacement. Current recommendations and long-term results

Techniques now exist to correct abnormalities of all components of the mitral valvular apparatus except extensive loss of pliable leaflet area. Thus, paradoxically, myxomatous valves with redundant leaflets represent the ideal candidates for mitral valve repair. Repair for mitral insufficiency can be performed for some rheumatic valves, but patient selection is critical. Loss of leaflet area, leaflet thickening, and extensive calcification of the leaflets or commissures are contraindications to repair. The abnormalities of the subvalvular apparatus are less important because a complete set of new chordae can be reconstructed using PTFE suture material. Some cases of endocarditis are ideal for repair using localized débridement and pericardial patch repair with or without PTFE chordal replacement. True ischemic mitral regurgitation of the Carpentier type I category is still something of a surgical enigma. Because it is a restrictive leaflet motion problem, annuloplasty alone is not always effective, and the outcome of any given repair attempt is less predictable. Repairs in patients with small annuli and multiple leaflet defects requiring complex series of maneuvers have a low probability of success. Furthermore, such patients with small left ventricular cavities are more prone to experience SAM. Several factors contributing to which therapy is chosen for mitral valve disease are summarized in Table 1. Patient selection, accurate evaluation of the cause or causes of mitral regurgitation, and well-executed application of the appropriate techniques for repair are all critical factors in the early and late success of mitral valve repair.     

Surgical management of congenital high vaginal atresia

In the past 15 years five neonates and children have been treated for high vaginal atresia at the Surgical Unit of Department of Pediatrics, University Medical School, Pécs, Hungary. In three of the six patients distal atresia of the vagina was found (Type III). Two of the three were neonates and had a large hydrometrocolpos and the third, a 13-year-old girl, hematometrocolpos. In the fourth patient the vaginal atresia was associated with cloacal and anorectal malformation (Type V). In the fifth there was a duplication of uterus and vagina; however, only one of the two vaginas was atretic. In three patients a pull-through of the vagina was carried out, in one of them according to Pena, in another a transvesical approach, as suggested by Monfort, was used. Following repeated surgeries in the patient with cloacal malformation the vagina was replaced with large intestine. In the case of duplication of vagina and uterus one half of the duplication was removed.     

Perinatal management of unanticipated congenital laryngeal atresia

Unless ventilation is achieved within minutes of delivery, patients with congenital laryngeal atresia will not survive. There are 2 settings in which survival is more likely: a tracheotomy may be immediately performed in the delivery room, or a communication may exist between the airway and the pharynx, allowing for air exchange. In the latter case, there are no characteristic findings on prenatal sonography to suggest the diagnosis and to ensure that preparations for immediate tracheotomy are made. We describe a neonate with unanticipated laryngeal atresia and a high tracheoesophageal fistula. Ventilation was maintained first by face mask and then by esophageal intubation until a tracheotomy could be performed. This report provides detailed photodocumentation of the anomaly, discusses the mechanism of air exchange, reviews the relevant embryological development, and outlines a protocol for perinatal management of unanticipated laryngeal atresia.     

Transnasal endoscopic excision of midline nasal dermoid from the anterior cranial base

We recommend that every patient with a midline nasal dermoid be evaluated preoperatively by fine-cut CT scan, in the axial and coronal planes, using both bone and soft-tissue algorithms. Because of CT limitations for assessing soft tissue at the cranial base, a complementary thin-section, high-resolution, multiplanar MRI scan should be considered. Transnasal endoscopic excision of midline nasal dermoid should be used if the dermoid is located within the nasal cavity, and there is minimal or no cutaneous involvement. This approach can be combined with a short vertical midline lenticular excision of a cutaneous punctum. Vertical cutaneous excision should be limited; the best location is the nasal tip. Vertical nasal scars over the bony pyramid invariably widen no matter what intradermal suture is used. Endoscopic removal should be considered even if preoperative radiologic studies show extension to the anterior cranial fossa. Endoscopic excision could also be used to resect a dermoid extending between the dural leaves of anterior fossa. However, a combined intra-extracranial approach is necessary if the dermoid is in the falx cerebri. We agree with other authors that frozen sectioning the superior margin of the specimen should be done to ensure that there is no intracranial extension. Valsalva maneuver during endoscopic visualization of the cranial base is a valuable means of ruling out a cerebral spinal fluid leak.     

COPD: results of long-term treatment

During the last decades the life expectation of patients with COPD increased continually. Today the life expectation of these patients reach that of the normal general population. Most important for this effect is the improvement of the therapeutical possibilities. Following the experience over nearly 3 decades the treatment regime is described. Glucocorticosteroids are at first line as much as necessary and as less as possible, starting with the inhalative forms adjusted at the clinical course stabilizes the situation. Bronchodilatation around the clock and as good as possible is a further important step. These effects have to be functionally-analytically controlled. It is not enough to follow only the clinical impression. beta 2-sympathicomimetics are the overall dilators with strongest effects and increase even the clearing mechanisms of the bronchopulmonary system. Short- as long-acting medicaments are available, but the duration of the action at each of the different forms is sometimes clearly shorter as the mean values describe. Anticholinergics and theophyllines have weaker bronchodilatation power, but they show additive effects, which could be used at special situations. Combinations as Berodual (beta 2-sympathicomimetics and anticholinergics) can decrease the dosage of the beta 2-sympathicomimetics and therefore increase the therapeutic range. At the situation of exacerbation antibiotics can be very helpful, but sometimes an increase of the dosage of glucocorticosteroids is necessary and sometimes the increase of this dosage alone can control the situation. It is very important to detect the deterioration which can end with a severe life threatening exacerbation as early as possible and to control the situation already at this stage. It is shown at examples of 56 patients functionally-analytically controlled over 5 years that the function values do not deteriorate during the constant treatment regime any more. Even in spite of the constant treatment with beta 2-sympathicomimetics no signs of any kind of down regulation or of tachyphylaxis of the bronchodilatation power could be detected. A bronchodilatation test after 4.2 years of constant bronchodilatation treatment together with beta 2-sympathicomimetics showed at 25 patients the same effect as normally at acute tests without pre-treatment seen. Because after the start of this treatment regime to-day a further deterioration does not take place, it is important to detect signs of the disease as early as possible. This can be achieved as the basis of lung function values measure over a long time. The best basis for this is the individual norm known from measurement at healthy days.     

Lung abscess: an unusual presentation of congenital tracheoesophageal fistula without atresia

Tracheoesophageal fistula (TEF) without atresia is rare and usually presents with symptoms from birth. In this report, a 9-year-old boy presented with productive cough of 4 month's duration and was shown to have a right lung abscess seen on chest radiograph. His parents denied earlier respiratory symptoms or illnesses. Rigid bronchoscopy showed a fistulous opening of about 1 mm in diameter in the posterior wall of the trachea about 16 cm from the upper incisor teeth. Cannulation with a ureteral catheter demonstrated that the fistulous opening communicated with the esophageal lumen. The tracheoesophageal fistula was 1 cm long and was divided through a right supraclavicular incision. The postoperative period was uneventful, and the patient was discharged on the third postoperative day. This case demonstrated that TEF should be considered in any patient presenting with chronic respiratory problems even after a prolonged symptom-free period.     

Surgical management of the congenital atresia of the ear

This study presents a 15 year experience of surgical treatment in 47 patients with congenital atresia of the ear. Emphasizing on the importance of pre-surgical evaluation of the patients for a better prognostic indicator. The ages of 40 patients were between 5-15 years. 24 patients (61%) had complete atresia and 23 (49%) incomplete. 27 patients (57%) with bilateral disease and 20 (43%) unilateral. Auditory test before surgery showed a conductive hearing loss of approximately 60 decibels (dB) and one case with total sensorineural hearing loss. There was an auditory average gain of 28.3 dB. 3 patients (6.4%) had associated cholesteatoma. Complications presented: Closure of the external auditory canal in 6 patients (12.7%), and facial nerve paralysis in 2 patients with recovery. The different surgical technics are described. In conclusion, for this type of surgery, combination between the otologist and plastic surgeon should be done for better results.     

Whipple's disease: results of long-term follow-up

AIM: To analyze clinical, diagnostic and therapeutic aspects of Whipple's disease. MATERIALS AND METHODS: Diagnostic and therapeutic data are available for 7 patients registered in 1990-1997. The diagnosis was made using intestinoscope SIF-10L ("Olympus"). Biopsies were obtained from the jejunal, duodenal and gastric mucosa. The patients received tetracycline, erythromycin, biceptol, on demand--prednisolone. RESULTS: Whipple's disease was diagnosed 6 years on the average following the first clinical symptoms. In most patients the intestinal symptoms were preceded or accompanied by such extraintestinal symptoms as enlargement of the lymph nodes, lowering of hemoglobin, hypoproteinemia, ESR increase to 40-60 mm/h. To study biopsies from the distal duodenum is the only measure needed for diagnosis of Whipple's disease. In untreated patients PAS-positive macrophages are detectable also in gastric body mucosa. The immediate treatment outcome is favorable. The recurrence was observed only in one patient who had given up taking tetracycline. CONCLUSION: Long-term antibacterial therapy brings the recovery. Primary disorder of the cellular immunity responsible for the disease onset necessitates long-term follow-up of the patients. Control biopsy should be examined once a year.     

Reconstructive surgery for congenital atresia of the uterine cervix

Reconstructive surgery for congenital atresia of the uterine cervix, utilizing the atretic cervix as a conduit for the permanent fistulous communication between the endometrial cavity and vagina, has resulted in the establishment of cyclic menses in four patients followed for up to 27 months postoperatively. A detailed review of maternal obstetric histories failed to reveal a common factor causally related to this extremely unusual and formerly hopeless congenital anomaly of the Müllerian ducts.     

Postanal repair for faecal incontinence: long-term follow-up

BACKGROUND: To determine the long-term outcome of postanal repair, and to assess whether the preoperative and physiological tests had any bearing on this outcome. Review included an opportunity for assessment with anal manometry and anal ultrasound. METHOD: Review of all patients (n = 22) over a 10 year period from 1986 to 1996. Comparison was of pre-operative symptoms to symptoms at review. Correlation of outcome with pre-operative manometry and the results of manometry and ultrasound at review (n = 6) was determined. RESULTS: Assessment was possible in 19 of the 22 patients. Follow-up ranged from 2 to 10 years (median, 8 years). Two had stomas created at 6 and 9 months and are considered failures. Seven patients considered the operation a success, in four it improved their symptoms and in six it was considered a failure. Comparison of pre- and postoperative symptoms scores found a statistically significant improvement (P = 0.0093; two-tailed Wilcoxon signed rank sum test). The outcome was not influenced by the results of pre-operative anal manometry. Anal ultrasound found five sphincter defects in six patients. Such defects did not preclude improvement from postanal repair. CONCLUSIONS: Although the results showed improvement or success in only 11 (58%) of the patients this was felt to be important given that these patients may have few alternatives other than complicated procedures or a stoma. Postanal repair has a place in the management of faecal incontinence.     

Mitral valve repair: Intermediate to long-term echocardiographic follow-up

PURPOSE: To study the changes in clear cornea incision size after phacoemulsification and implantation of a three-piece, silicone, foldable intraocular lens (IOL). SETTING: Departamento de Oftalmologia, Clinica Universitaria de Navarra, Pamplona, Spain. METHODS: In this prospective study, phacoemulsification with implantation of a foldable IOL (AMO SI-30NB) was performed in 133 eyes. Surgery was carried out by four surgeons who implanted the IOL with the same forceps. The external incision size was measured before and after phacoemulsification and irrigation/aspiration and before and after IOL implantation. RESULTS: Before phacoemulsification the mean incision size was 3.07 mm; after phacoemulsification and irrigation/aspiration, it enlarged to 3.09 mm (P = .001). The mean incision size before foldable IOL implantation was 3.16 mm, and after implantation it increased to 3.32 mm (P = .0001). When the IOL was less than 22.0 diopters (D), the final incision size was 3.31 mm +/- 0.11 (SD); when the IOL was 22.0 to 26.0 D, the final size was 3.32 +/- 0.01 mm; and when the IOL was 26.0 D or more, the final size was 3.39 +/- 0.08 mm (P = .01). CONCLUSIONS: Phacoemulsification and foldable IOL implantation enlarged the size of the clear cornea incision in this study. The IOL power may be a significant factor in determining the minimal incision size prior to IOL insertion and the final incision size.     

Analysis of 309 cases of esophageal atresia for associated congenital malformations

We describe a 50-year-old man with adult T-cell leukemia complicated by laryngeal tuberculosis whose tumor cells proliferate in response to IL-2 in a paracrine manner. On admission, the patient's white blood cell count was 17,900/mm3; 73% were abnormal lymphocytes with convoluted nuclei. FACS analysis showed that the tumor cells were CD4-negative, CD8-positive T cells. Southern blot analysis of tumor cells revealed integration of a defective HTLV-I genome lacking gag and pol genes. He was diagnosed with chronic ATL complicated by laryngeal tuberculosis. The primary leukemic cells expressed IL-2R alpha and IL-2R beta detected by FACS and Northern blot analysis and showed marked growth in response to exogenously added recombinant IL-2 in short-term cultures. Northern blot analysis did not show any IL-2 mRNA. We have previously demonstrated that primary leukemic cells from some ATL patients grow in response to IL-2 in an autocrine or paracrine manner. These results suggest that in CD8 ATL, IL-2 may be involved in a paracrine manner.     

Cartilage-sparing otoplasty: a review with long-term results

Prominent ears are the most frequent congenital deformity in the head and neck region. Anatomy of normal and prominent ears as well as the psychological aspects of prominent ears are reviewed. Two types of surgical technique are described with emphasis on the cartilage-sparing technique. A sound pre-operative analysis, focusing on all parts of the deformity, and surgical techniques which are gradually applied to these deformities should result in pleasing, permanent changes for the vast majority of patients. In our opinion, a combination of cartilage-sparing techniques augmented with cartilage-weakening procedures give predictable long-term results with a natural appearing ear and concomitant few, easily treated complications.     

Percutaneous alcohol administration for hepatocarcinoma: long-term results

We investigated the long-term outcome of percutaneous ethanol injection (PEI) in the treatment of hepatocellular carcinoma (HCC). A series of 184 HCC patients received PEI as the sole anticancer treatment over an 8-year period, December, 1987, to December, 1995. Ninety-four patients had a single tumor < or = 3 cm, 50 patients had a single lesion of 3.1-5 cm, and 40 patients had multiple nodules (up to four) < or = 3 cm each. All patients had liver cirrhosis, classified as Child-Pugh class A in 127 cases and as Child-Pugh class B in 57 cases. The treatment schedule included, for each lesion, 6-14 therapeutic sessions performed once or twice weekly. The total amount of alcohol administered ranged 10 to 110 ml (mean: 36 ml). All patients were followed after therapy with clinical examinations, laboratory tests, and imaging studies performed at regular time intervals. The follow-up period ranged 2 to 94 months (mean: 23.6 months). Overall survival rates by the Kaplan-Meier method were 67% at 3 years, 41% at 5 years, and 19% at 7 years. The survival rates of patients with single lesion < or = 3 cm (78% at 3 years, 54% at 5 years, and 28% at 7 years, respectively) were significantly higher (p < .01) than those of the patients with a single lesion of 3.1-5 cm (61% at 3 years, 32% at 5 years, and 16% at 7 years) or multiple lesions (51% at 3 years, 21% at 5 years, and 0% at 7 years). The survival of Child-Pugh A patients (79% at 3 years, 53% at 5 years, and 32% at 7 years) was significantly longer (p < .01) than that of Child-Pugh B patients (50% at 3 years, 28% at 5 years, and 8% at 7 years). A selected group of 70 patients with Child-Pugh A cirrhosis and a single lesion < or = 3 cm had a 3-, 5-, and 7-year survival of 89%, 63% and 42%, respectively. During the follow-up, new lesions appeared in 93 patients. The recurrence rates by the Kaplan-Meier method were 15% at 1 year, 34% at 2 years, 51% at 3 years, 67% at 4 years, 78% at 5 years, 88% at 6 years, and 94% at 7 years. The analysis of the survival curves of the treated patients confirms the effectiveness of PEI in the treatment of HCC. This therapeutic approach is particularly indicated for patients with a single lesion 3 cm or less in greatest diameter, as in these cases the long-term results of PEI are comparable to those reported in the best surgical series published in the literature.