Morphological consequences of lateral outfracture of the inferior turbinate.

OBJECTIVE: We report three cases of lateral outfracture of the inferior turbinate, which demonstrate a range of changes in the size, position and shape of the inferior turbinate. METHOD: During a study of the validity of computer modelling of nasal airflow, computed tomography scans of the noses of patients who had undergone lateral outfracture of the inferior turbinate were collected. The pre-operative scan was compared with the post-operative scan six weeks later. RESULTS: In one patient, there was only a small lateral displacement of the inferior turbinate. In the other two cases, appreciable reduction in the volume of one inferior turbinate was noted, in addition to minor changes in the shape. CONCLUSION: Lateral outfracture of the inferior turbinate produces varied and inconsistent changes in morphology which may affect the shape, size and position of the turbinate.

Bilateral hearing results of 751 unilateral stapedotomies evaluated with the Glasgow benefit plot.

AIMS: We aimed to evaluate bilateral hearing function in patients undergoing primary unilateral stapedotomy, according to the 1995 American Academy of Otolaryngology, Head and Neck Surgery guidelines and the Glasgow benefit plot. We also aimed to analyse the effect of pre-operative hearing impairment type on post-stapedotomy hearing. STUDY DESIGN: Retrospective chart review. METHODS: Medical records relating to 1369 stapedotomies performed by the senior author (JJH) from 1991 to 2006 were reviewed. Seven hundred and fifty-one patients undergoing primary unilateral stapedotomy were included. Hearing results for these patients were evaluated according to the criteria of the 1995 American Academy of Otolaryngology, Head and Neck Surgery Committee on Hearing and Equilibrium guidelines, and the Glasgow benefit plot. Subgroups of patients with pre-operative unilateral, bilateral symmetrical and bilateral asymmetrical hearing loss were separately analysed. RESULTS: The most successful results, as regards the achievement of bilateral, socially serviceable hearing, were demonstrated in patients with unilateral hearing loss; 78 per cent of these patients had normal hearing post-operatively. Overall, patients' achievement of bilateral, socially serviceable hearing correlated highly with their type of pre-operative hearing impairment (r = 0.74). Normal post-operative hearing levels also correlated with pre-operative bone conduction (r = 0.61). CONCLUSION: This study represents the largest reported series of primary stapedotomy cases evaluated with the Glasgow benefit plot. Patients' bilateral post-operative hearing function depended on their type of pre-operative hearing impairment. Pre-operative bone conduction thresholds, corrected for Carhart's effect, were useful in predicting achievable post-operative air conduction.

Hearing results of 1145 stapedotomies evaluated with Amsterdam hearing evaluation plots.

AIMS: To evaluate the hearing results of a large series of primary stapedotomies, according to American Academy of Otolaryngology, Head and Neck Surgery guidelines and Amsterdam hearing evaluation plots. STUDY DESIGN: Retrospective chart review. METHODS: The charts for 1369 consecutive stapedotomy cases were reviewed; 1145 cases of primary stapedotomy were included. Raw data from the audiometric database were evaluated using Amsterdam hearing evaluation plots. The effect on outcomes of using different audiological parameters was analysed. RESULTS: A significant improvement was demonstrated in mean post-operative air conduction and speech reception thresholds, with no change in bone conduction. Air-bone gap closure of 10 dB or more was achieved in 82 per cent of cases. A 'dead ear' occurred in one patient (0.1 per cent). CONCLUSION: This study reports the largest series of primary stapedotomies evaluated with Amsterdam hearing evaluation plots. This method enables visual identification of successful and unfavourable results, providing more accurate and detailed presentation of surgical outcomes.

Pharyngo-laryngo-oesophagectomy and gastric pull-up for post-cricoid and cervical oesophageal squamous cell carcinoma.

Pharyngo-laryngo-oesophagectomy and gastric pull-up (PLOGP) is a complex and relatively uncommon procedure. The aim of this study is to analyse the results of PLOGP in patients with post-cricoid and cervical oesophageal squamous cell carcinomas. This study was a retrospective review of 26 patients (11 males + 15 females, mean age 63.5 years) who underwent PLOGP from 1988 to 1997. Eighteen (69 per cent) patients were staged as T(3) and eight (31 per cent) T(4). Eighteen (69 per cent) patients had N(0), seven (27 per cent) N(1) and one (four per cent) N(2) disease. Multiple primary tumours were recorded in three (11.5 per cent) patients. Four (15 per cent) patients had pre-operative radiotherapy with poor response and two (eight per cent) required emergency tracheotomy prior to surgery. Feeding jejunostomy was performed on 19 (73 per cent) and neck lymph node dissection in eight (31 per cent) patients. The mean duration of surgery was five hours (range 3.5 to 7.5) with a mean blood loss of 840 ml (range 160 to 1800), a mean stay in ICU of 4.2 days and hospital stay ranged from nine to 84 days (mean 34). Three (11.5 per cent) patients died (pneumonia - one, congestive heart failure - one, pulmonary embolus - one) in the early post-operative period. Eight (31 per cent) patients remain alive from 30 to 136 months (mean 58 months). Two (eight per cent) patients died with no evidence of disease. Thirteen (50 per cent) patients died of their disease between two to 51 months (mean 17.3 months) post-operatively. Kaplan-Meier survival estimates for one year was 65 per cent, for three years 35 per cent and for five years 26 per cent (see Figure 1). Median survival in the whole series was 18 months. Post-operative speech was with an electrolarynx in 16 (62 per cent). One patient (four per cent) used gastric speech and one patient (four per cent) used a Blom-Singer valve effectively. Five (19 per cent) patients had no speech post-operatively. All patients maintained oral feeding. Gastric transposition constitutes a safe and reliable method of restoring the continuity of the upper digestive tract following pharyngo-laryngo-oesophagectomy.

Bilateral subdural collections--an unusual feature of possible Kawasaki disease.

A case of possible Kawasaki disease presenting with bilateral subdural collections is reported. A 6-month-old boy was admitted to hospital following a prolonged partial seizure. He showed signs of worsening encephalopathy and was treated for presumed infective meningoencephalitis. Imaging revealed bilateral subdural collections. Pyrexia and irritability continued despite appropriate antibiotic and antiviral therapy suggesting a vasculitic process. The mucocutaneous signs normally associated with Kawasaki disease were subtle and fleeting in this infant. There was a dramatic clinical response to intravenous immunoglobulin and high dose aspirin. At 2-year follow-up the boy has made a complete recovery with no echocardiographic evidence of coronary artery aneurysm and resolution of subdural collections. Kawasaki disease or other vasculitides should be considered in the differential diagnosis of acute subdural collections in infancy.

Deficiency of dolichol-phosphate-mannose synthase-1 causes congenital disorder of glycosylation type Ie.

Congenital disorders of glycosylation (CDG), formerly known as carbohydrate-deficient glycoprotein syndromes, lead to diseases with variable clinical pictures. We report the delineation of a novel type of CDG identified in 2 children presenting with severe developmental delay, seizures, and dysmorphic features. We detected hypoglycosylation on serum transferrin and cerebrospinal fluid beta-trace protein. Lipid-linked oligosaccharides in the endoplasmic reticulum of patient fibroblasts showed an accumulation of the dolichyl pyrophosphate Man(5)GlcNAc(2) structure, compatible with the reduced dolichol-phosphate-mannose synthase (DolP-Man synthase) activity detected in these patients. Accordingly, 2 mutant alleles of the DolP-Man synthase DPM1 gene, 1 with a 274C>G transversion, the other with a 628delC deletion, were detected in both siblings. Complementation analysis using DPM1-null murine Thy1-deficient cells confirmed the detrimental effect of both mutations on the enzymatic activity. Furthermore, mannose supplementation failed to improve the glycosylation status of DPM1-deficient fibroblast cells, thus precluding a possible therapeutic application of mannose in the patients. Because DPM1 deficiency, like other subtypes of CDG-I, impairs the assembly of N-glycans, this novel glycosylation defect was named CDG-Ie.

Diphallia in a mixed-breed dog with multiple anomalies.

Complete diphallia was detected in a six-month-old Poodle cross dog. Duplication of the urinary bladder, right renal hypoplasia, bifurcation of the descending colon and bilateral cryptorchidism were also present. The left urethra was patent, and emptied the left urinary bladder; the right urethra ended blindly at a point 2.5 cm from the tip of the right penis. The left urinary bladder was joined medially to a right urinary bladder with no interbladder communication; however, the right bladder opened caudally into a blind sac which drained into the left prostatic urethra. Pyelonephritis of the left kidney was present, and was suspected to be due to ascending bacterial infection of the right urinary bladder and the right segment of the bifurcated descending colon, which terminated at the apex of the right urinary bladder. Diphallia and associated urogenital anomalies are reviewed.

Teratogenic effect of acetohydroxamic acid in clinically normal beagles.

Acetohydroxamic acid (AHA), a potent urease inhibitor used for treatment of infection-induced struvite urolithiasis, was teratogenic after administration of 25 mg of AHA/kg of body weight/day orally to 5 clinically normal Beagles from the onset of proestrus until parturition. Thirty pups exposed to AHA in utero developed anomalies of the skeletal system, heart, and ventral midline. Cardiac anomalies included atrial septal defects (20%), ventricular septal defects (3%), and atrial and ventricular septal defects (3%). Skeletal anomalies included coccygeal hemivertebrae and fused coccygeal vertebrae (50%), supernumerary vertebrae (67%), supernumerary ribs (50%), duplicated sternebrae (3%), and lumbar hemivertebrae (3%). Defects of the ventral midline of the abdominal wall occurred in 20% of AHA-exposed pups. Other abnormalities included retarded growth, high neonatal mortality, and a decreased number of circulating RBC, compared with those in 30 control pups born to 5 Beagles given a placebo. Adverse effects of AHA in pregnant Beagles were limited to morphologic alterations (Howell-Jolly bodies, spherocytes, and target cells) in a small number of circulating RBC. Slight neutrophilic leukocytosis and monocytosis occurred between 0 and 30 days of pregnancy in dogs given AHA, compared with those in controls. Seemingly, AHA did not influence fertility, conception rate, or length of gestation.

Comparison of qualitative and quantitative analyses of canine uroliths.

A study was performed to compare qualitative test results of canine uroliths with quantitative test results. Qualitative test results were in agreement with quantitative test results in only 96 of 223 specimens (43 per cent). Lack of agreement was caused by false-negative qualitative test results (85 of 223), false-positive qualitative test results (15 of 233), and a combination of false-negative and false-positive qualitative test results (27 of 233). This study indicates that the veterinary profession should abandon exclusive use of qualitative chemical test kits for uroliths.