Long-term surgical results for congenital aural atresia

Thirty-nine primary surgical cases for correction of congenital aural atresia were reviewed for complications and long-term hearing results. Hearing averages of 25 dB for mild atresia, 40 dB for moderate atresia, and 46 dB for severe atresia were obtained. Serviceable hearing was achieved in 64% of the cases. The two most frequent complications were stenosis and recurrent infections of the cavity and canal skin, with an incidence of 33% and 31%, respectively. Use of split-thickness instead of full-thickness skin graft was associated with fewer complications. The goal of this review is also to share the experience of the senior author in the management of this complex problem.     

Onset, progression, and plateau of skeletal lesions in fibrous dysplasia and the relationship to functional outcome.

Most lesions in FD and their attendant functional disability occur within the first decade; 90% of lesions are present by 15 years, and the median age when assistive devices are needed is 7 years. These findings have implications for prognosis and determining the timing and type of therapy. INTRODUCTION: Fibrous dysplasia of bone (FD) is an uncommon skeletal disorder in which normal bone is replaced by abnormal fibro-osseous tissue. Variable amounts of skeletal involvement and disability occur. The age at which lesions are established, the pace at which the disease progresses, if (or when) the disease plateaus, and how these parameters relate to the onset of disability are unknown. To answer these questions, we performed a retrospective analysis of a group of subjects with FD. MATERIALS AND METHODS: One hundred nine subjects with a spectrum of FD were studied for up to 32 years. Disease progression was assessed in serial (99)Tc-MDP bone scans by determining the location and extent of FD lesions using a validated bone scan scoring tool. Physical function and the need for ambulatory aids were assessed. RESULTS: Ninety percent of the total body disease skeletal burden was established by age 15. Disease was established in a region-specific pattern; in the craniofacial region, 90% of the lesions were present by 3.4 yr, in the extremities, 90% were present by 13.7 yr, and in the axial skeleton, 90% were present by 15.5 yr. Twenty-five of 103 subjects eventually needed ambulatory aids. The median age at which assistance was needed was 7 yr (range, 1-43 yr). The median bone scan score for subjects needing assistance was 64.3 (range, 18.6-75) compared with 23.1 (range, 0.5-63.5) in the unassisted subjects (p < 0.0001). Among subjects needing assistance with ambulation, 92% showed this need by 17 yr. CONCLUSIONS: The majority of skeletal lesions and the associated functional disability occur within the first decade of life. The implication is that the window of time for preventative therapies is narrow. Likewise, therapeutic interventions must be tailored to where the patient is in the natural history of the disease (i.e., progressive disease [young] versus established disease [older subjects]). These findings have implications for prognosis, the timing and type of therapy, and the development of trials of new therapies and their interpretation.     

Temporal Changes in Micturition and Bladder Contractility after Sucrose Diuresis and Streptozotocin-induced Diabetes Mellitus in Rats

Studies were done to compare the acute effects of streptozotocin-induced diabetes and sucrose consumption on micturition, bladder mass and contractile responses of bladder strips to field stimulation and contractile agonists. Micturition changes occurred gradually in diabetic rats, reached maximal values within 7 to 14 days, and were accompanied by significant increases in bladder mass after 7 days. Bladder strips from diabetics responded to field stimulation, carbachol and KCl with significantly greater contractions than did those from controls within 7 days. Sucrose-drinking rats had maximal increases in fluid consumption and micturition frequency on the first night after starting treatment. Increases in micturition volumes were slower to develop than in diabetics. Bladder mass was significantly increased 30 and 60 days after starting sucrose treatment. Bladder strips from sucrose-drinking rats responded to field stimulation and carbachol with significantly greater contractions than did those from controls only after 60 days. Monitoring of drinking and micturition patterns established that diabetic rats drink and urinate during both the dark and light cycles. In contrast, control and sucrose-drinking rats drink and urinate principally at night. The results demonstrate that differences in bladder function between diabetic and sucrose drinking rats are apparent during the first month after treatment begins. The data suggest that the effects of diabetes and sucrose consumption on contractile bladder function are related to the diuresis-induced increases in bladder mass.     

Transcranial doppler ultrasonography for the evaluation of shunt malfunction in pediatric patients

Previous studies have demonstrated a high correlation between hydrocephalus and the resistive index (RI), as determined by transcranial Doppler ultrasonography. Measurements of RI, calculated by dividing the difference between the peak systolic velocity and the enddiastolic velocity by the peak systolic velocity, were attempted in 55 pediatric patients during evaluations for ventriculoperitoneal shunt malfunctions; values were obtained in 52. Indications of shunt malfunction included both clinical and radiographic evidence of increased intracranial pressure. Eleven patients, determined to have functional shunts both by clincal criteria and subsequent outcome, had RIs of 47±5 (average±1 standard deviation). Shunt malfunctions were confirmed in 41 patients. Prior to shunt revisions, these 41 patients has RIs of 71±10%; following revision, the RIs fell to 53±12%. Nine patients had had pre-malfunction RIs of 48±11% obtained during routine follow-ups; when they subsequently had shunt malfunctions, their RIs had significantly increased. Four of the 41 patients with shunt malfunctions had essentially normal RIs (52±7%), but had fluid tracking along the shunt; in these, RIs were essentially unaffected by shunt revision. For comparison, 119 pediatric patients with clinically functional ventriculoperitoneal shunts had RIs of 50±9%. The data, statistically significant with a P value of <0.001, showed a correlation between elevated RIs and shunt malfunction; thus, transcranial Doppler ultrasonography is a practical, non-invasive technique useful in the diagnosis of ventriculoperitoneal shunt malfunction.Presented at the Annual Meeting of the Pediatric Section of the American Association of Neurological Surgeons, Washington D.C., November 1989