926
Bladder function and
dysfunction epispadias
Bladder exstrophy and epispadias are congenital abnormalities of the urinary bladder. Evaluation of bladder function before and after bladder-neck reconstruction has not been done in patients with these conditions. We report the preliminary results of a prospective study of lower-urinary-tract function in 36 children (10 girls, 26 boys; age range 1·5-16 years) with bladder exstrophy and epispadias. Children’s bladder function was measured by cystometry and cystography, and their upper urinary tracts were examined by ultrasound imaging. Normal bladder function was seen in children with primary epispadias but this was severely impaired in children who had had conventional bladder-neck surgery for continence, implying that this may not be the optimal treatment. Contrary to current assumption, children with closed exstrophy bladders are not capable of normal function, and the abnormalities we identified may be a major cause of upper-urinary-tract damage and may impair development of bladder capacity. Although unrecognised in the past, involuntary bladder contractions are a primary cause of urine leakage persisting in children with exstrophy and epispadias after continence surgery. Detailed functional testing should become a routine part of the evaluation of children with exstrophy and epispadias. Introduction Bladder exstrophy is a severe congenital abnormality characterised by a very small bladder turned inside out through a defect in the lower abdominal wall with no urethra or sphincter for urinary control. Epispadias is a less severe variant in which the bladder is small but not everted and the degree of urethral development and urinary control is variable. Historically, children with exstrophy and severe epispadias underwent urinary diversion to an ileal or colonic conduit. Over the past 15-20 years improvements in surgical techniques have allowed bladder closure and urethral construction, which have resulted in a greatly improved cosmetic outcome for most children.l3 However, because the subsequent surgery required to achieve urinary control lacks predictability, multiple major operations can result. In addition, although most affected children are born with normal kidneys, 25-50% have subsequent upper-urinarytract damage after reconstructive procedures. Thus, achieving continence with preservation of renal function in exstrophy and epispadias remains one of the most daunting of challenges in paediatric surgery. The first step of exstrophy reconstruction is turning in the everted bladder--done during the neonatal period-with no
in
exstrophy and
to create resistance to leakage. Serial evaluations after closure reveal that most patients have vesicoureteric reflux, and some degree of hydroureteronephrosis is common. Development of bladder capacity at this stage is variable. Initial surgery for continence (at 3-5 years of age) in exstrophy and epispadias is tubularisation of the inferior portion of the bladder to create a tight bladder neck that will provide resistance to leakage.4 This approach to management is based on the assumption that: (1) the closed exstrophy bladder leaks continuously, without providing storage function ; (2) bladder capacity will expand and eventually continence will develop in response to increased outflow resistance; and (3) the bladder (detrusor) muscle is capable of normal function-ie, relaxation during filling to enable urine storage at low pressure and volitional contraction for voiding. Failure to achieve capacity and continence with this protocol leads to additional surgical procedures including revision of the bladder-neck reconstruction, placement of an artificial urinary sphincter,S and enlargement of the bladder with a segment of bowe1.6,7 Often, these procedures also fail to achieve urinary control and result in further upper-urinary-
attempt
damage. Surprisingly, evaluation of bladder function during filling, voiding, and leaking before and after bladder-neck reconstruction has not been done for patients with exstrophy and epispadias. As a consequence, abnormalities in lower-urinary-tract function, which are potential causes of upper-urinary-tract damage and which may impair development of bladder capacity and continence, have gone unrecognised. We are doing a prospective study to answer several questions regarding bladder function in patients with exstropy and epispadias. This preliminary report is concerned with the unexpected findings that conventional surgery may be based on false assumptions and that the surgery itself may adversely affect bladder function. tract
Patients and methods For the purposes of our study children have been classified into three groups. Group A are 5 children (1girl, 4 boys; age range 3-13 years) with primary severe epispadias after urethral closure but before planned bladder-neck reconstruction. Group B are 15 children (6 girls, 9 boys; age range 1 -5-9 years) with exstrophy after bladder and urethral closure but before surgery for continence. Group C are 16 children (9 with exstrophy, 7 with epispadias; 3 girls, 14 boys; age range 6-16 years) who have had bladder-neck reconstruction(s) (without augmentation) but remain incontinent (secure dry intervals less than 3 h).
ADDRESSES: Department of Paediatric Urology, St Peter’s Hospitals and The Hospital for Sick Children, Great Ormond Street, London, UK (J. G Hollowell, MD, P G. Duffy, FRCS, P G. Ransley, FRCS), and Department of Medical Physics, St Peter’s Hospitals, London (P. D. Hill, MSc). Correspondence to Mr Philip G Ransley, Department of Urology, Hospital for Sick Children, London
WC1N 3JL, UK.
927
All children had their bladder pressure recorded during natural filling (in the sitting and supine positions) with a catheter (’Cystofix minipaed CH5’, B. Braun, Melsungen, Germany) placed suprapubically into the bladder under general anaesthesia at least 2 days before the bladder-function study. Intra-abdominal pressure (measured via a rectal catheter), bladder pressure, and subtracted (detrusor) pressure were recorded simultaneously throughout filling and voiding/leaking with the ’Ormed UIS 5000’ system (Lectromed, Welwyn Garden City, UK). All studies were done by the same individual. Bladder function during filling was defined as active when phasic contractions greater than 10 cm H2O occurred that were clearly not under the patient’s volitional control. Otherwise bladder function was considered stable. Leak pressure, a variable related to bladder-neck resistance, was defmed as the minimum total bladder pressure recorded by cystometry that resulted in leakage. Cystograms were taken under general anaesthesia of all children in groups A and B at the time of placement of the suprapubic catheter. The bladder was filled with contrast solution through a urethral catheter until bladder pressure was 25-30 cm H2O. Cystogram capacity was defined as the total volume of contrast solution instilled with an adjustment made for an estimate of upper-urinary-tract volume in those with reflux. Except in the case of 4 children, where the cystogram revealed reflux into a normal undilated upper-urinary-tract, renal ultrasound images were obtained.
Results
Group A All 5 children in this group were able to initiate a detrusor contraction for voiding and none had involuntary detrusor contractions during filling. The upper urinary tracts of these patients were normal.
NUMBER OF PATIENTS WITH EXSTROPHY OR EPISPADIAS AND BLADDER-NECK RECONSTRUCTION WITHIN EACH CATEGORY OF LOWER-URINARY-TRACT FUNCTION
*15 of 16 patients were emptying without catheterisation and evaluated m 14 of them
voiding could be
Group C Bladder capacity, bladder function during filling and voiding, and bladder-neck function (as measured by leak pressure) of the 16 children in group C are summarised in the table. Of these 16 children with persisting incontinence, 4 leaked only with involuntary detrusor contractions. 5 others had minor leakage at low pressures (10-20 cm H2O), but their major difficulty was high-pressure (55-165 cm H2O) detrusor contractions. The cause of incontinence in the other 7 children
was
low leak pressure.
Discussion
Group B the 15 children with exstrophy in this group had not had bladder-neck reconstruction to create outflow resistance, most did not leak urine continuously and had some storage function. Bladder-filling cycles in 10 children showed intermittent involuntary detrusor contractions (maximum pressure up to 90 cm H2O) that were responsible for most of the leakage of urine. The frequency of these contractions was variable, occurring once per 20 min in some but only once in 2-3 h in others. Leak pressure was 10-30 cm H2O in 8 patients and greater than 30 cm H2O in 4. 3 patients leaked urine with no increase in bladder pressure. Only 2 of the 11 patients in whom voiding could be evaluated were able to initiate a detrusor contraction for voiding. The other 9 voided by abdominal straining. Bladder capacities and upper-urinary-tract status of this group of patients were analysed with respect to bladder activity and leak pressure to determine if these variables of lower-urinary-tract function influence progression of urinary-tract development. Bladder capacity was poor (mean 45 ml) in the 3 children with leak pressure less than 10 cm H20. Of the 8 patients with a leak pressure of 10-30 cm H2O, capacity was better in the 2 without bladder activity (mean =120 ml) than in the 6 with involuntary contractions (mean = 80 ml). The 4 children with a leak pressure greater than 30 cm H20 all had involuntary contractions with variable capacity (40-260 ml). Upperurinary-tract dilatation was not observed by ultrasound even in the presence of involuntary detrusor contractions when leak pressure was less than 30 cm H2O. Of the 4 children with a leak pressure greater than 30 cm H2O, all of whom showed involuntary contractions, 2 had upperurinary-tract dilatation.
Although
=
Although this is a preliminary study with relatively few patients in each group, the data clearly refute several assumptions regarding lower-urinary-tract function on which current management of patients with exstrophy and epispadias is based. The closed exstrophy bladder (group B) does not continuously drip urine as thought previously. In most patients in this group leakage was intermittent and caused by involuntary detrusor contractions. It appears that development of bladder capacity at this stage is related to bladder activity as well as outflow resistance. A leak pressure of at least 10 cm H2O is required to allow the bladder to expand, but leakage due to involuntary contractions may limit achievement of capacity in many of those with a leak pressure greater than 10 cm H2O. This finding opens up the possibility that pharmacological control of contractions may allow capacity to develop, and a trial of this is in progress. The primary risk factor for upper-urinary-tract dilatation is a leak pressure greater than 30 cm H2O. In the group of children with incontinence after bladderneck reconstruction (group C) bladder function was not normal, and the abnormalities that we found may inhibit an increase in bladder capacity and the development of continence in response to bladder-neck reconstruction. The inability to initiate and sustain a detrusor contraction for voiding impairs bladder emptying and thereby decreases functional bladder capacity and thus potential dry intervals. Some of these children achieve adequate emptying by abdominal straining but others require intermittent catheterisation as the next step of management rather than further surgery. Involuntary detrusor contractions are the primary cause of leakage in many patients in this group. Thus, bladder-neck revision or placement of an artificial
928
urinary sphincter, to create a higher leak pressure, without concomitant treatment of the detrusor dysfunction may still not achieve continence or further development of capacity. These children with involuntary contractions are also undergoing a trial of anticholinergic therapy before further surgery.
This study raises an important question regarding management of continence in primary epispadias. The 5 children who had not had bladder-neck surgery (group A) showed normal bladder function during filling and voiding. However, evaluation of the 7 children with primary epispadias after bladder-neck reconstruction (part of group C) revealed a high incidence of involuntary contractions during filling and most could not initiate contractile voiding. These findings suggest that a formal bladder-neck reconstruction may severely damage detrusor function. If this proves to be the case, other management options will need to be evaluated. Possible alternatives are endoscopic submucosal injection of collagen in the bladder neck area8 or the implantation of an artificial urinary sphincter without first reconstructing the bladder neck. Further investigation of lower-urinary-tract function in children with exstrophy and epispadias is needed. However, this study provides sufficient evidence for several preliminary conclusions. Contrary to current assumptions, bladder function is not normal in most children with exstrophy and in those with epispadias after bladder-neck surgery. These functional abnormalities may be a cause of upper-urinary-tract damage and may impair development of bladder capacity and continence at all stages.
Clinical
respiratory
≽40/min in children over 12 months old and ≽ 50/min in infants, as
a
showed little additional
We thank Sister Brid Carr for her invaluable assistance and Mr Larry Watkinson for help with information technology support. The support for J. G. H. from The St Peter’s Research Trust and Action Research is
gratefully acknowledged. REFERENCES
JS. Surgical treatment of exstrophy of the bladder with emphasis neonatal primary closure: personal experience with 28 consecutive cases treated at the University of Washington Hospitals from 1962 to 1977: techniques and results. J Urol 1979; 121: 650-53. Osterling JE, Jeffs RD. The importance of a successful initial bladder closure in the surgical management of classical bladder exstrophy: analysis of 144 patients treated at the Johns Hopkins Hospital between 1975 and 1985. J Urol 1987; 137: 258-62. Ransley PG, Duffy PG, Wollin M. Bladder exstrophy closure and epispadias repair. In: Spitz L, Nixon HH, eds. Paediatric Surgery. London: Butterworths, 1988: 620-32. Jeffs RD, Lepor HL. Management of the exstrophy-epispadias complex and urachal anomalies. In: Walsh PC, Gittes RF, Perlmutter AD, Stamey TA, eds. Campbell’s Urology, 5th ed. Philadelphia: WB Saunders, 1986: 1882-921. Decter RM, Roth DR, Fishman IJ, Shabigh R, Scott FB, Gonzales ET Jr. Use of the AS800 device in exstrophy and epispadias. J Urol 1988; 140: 1202-03. Gearhart JP, Jeffs RD. Augmentation cystoplasty in the failed exstrophy reconstruction. J Urol 1988; 139: 790-93. Hollowell JG, Ransley PG. The surgical management of incontinence in bladder exstrophy. Br J Urol (in press). Bloom DA, McGuire EJ. Collagen injection therapy in children with myelodysplasia. Dialogues Paed Urol 1991; 14: 2-3.
1. Ansell on
2.
3.
4.
5.
6.
7. 8.
signs of pneumonia
Clinical and chest radiographic findings were recorded prospectively in 185 children with cough who attended an outpatient clinic in Papua New Guinea. Children were studied if they were between 8 weeks and 6 years of age; patients with wheeze, stridor, measles, or pertussis were excluded. 56 children (30%) had radiological evidence of pneumonia. The presence of either a respiratory rate ≽50/min or chest indrawing, or of both signs, was a good indication of pneumonia, with a predictive power of 46% for a positive test and 83% for a negative test. A more complex definition of
tachypnoea,
Detailed functional evaluation is necessary to identify abnormalities and plan management in children with exstrophy and epispadias and should become a routine part of their evaluation in the future.
rate
diagnostic benefit.
Introduction 4 million children annually die from acute respiratory infections world wide,l most as a result of bacterial pneumonia.2 The World Health Organisation (WHO) has suggested that mortality from pneumonia could be substantially reduced by early antibiotic therapy. Although most children with pneumonia present with cough, most children with a cough do not have pneumonia, so it is
in children
important to find clinical signs that will help primary health workers to decide which children with cough have pneumonia and require early treatment with antibiotics. There have been several attempts to identify reliable clinical signs of pneumonia in children: in some, pneumonia was occasionally or invariably diagnosed on clinical rather than radiological evidence;3-5 in another, radiographs were used to diagnose pneumonia but respiratory rate was not recorded;6 another only included 18 children with pneumonia;’ and yet another looked at children who already satisfied WHO criteria for pneumonia.8 We prospectively recorded clinical signs among children with cough before they had a chest X-ray, to determine which clinical signs predicted radiographic evidence of pneumonia. Patients and methods We studied 185 children who attended the paediatric outpatient department of Goroka Hospital, Papua New Guinea. Most of the children who attend this clinic come from rural villages in the area around Goroka, a small Highlands town. Children were admitted to ADDRESSES Goroka Base Hospital, Goroka, Papua New Guinea (M Harari, FRACP, V Spooner, MRCGP, S. Meisner, MB, M Carney, MB) and Royal Children’s Hospital, Melbourne, Australia (F. Shann, FRACP, J de Campo, FRACR). Correspondence to Dr F. Shann, Intensive Care Unit, Royal Children’s Hospital, Flemington Road, Parkville, Victoria 3052, Australia
Bladder function and
dysfunction epispadias
Bladder exstrophy and epispadias are congenital abnormalities of the urinary bladder. Evaluation of bladder function before and after bladder-neck reconstruction has not been done in patients with these conditions. We report the preliminary results of a prospective study of lower-urinary-tract function in 36 children (10 girls, 26 boys; age range 1·5-16 years) with bladder exstrophy and epispadias. Children’s bladder function was measured by cystometry and cystography, and their upper urinary tracts were examined by ultrasound imaging. Normal bladder function was seen in children with primary epispadias but this was severely impaired in children who had had conventional bladder-neck surgery for continence, implying that this may not be the optimal treatment. Contrary to current assumption, children with closed exstrophy bladders are not capable of normal function, and the abnormalities we identified may be a major cause of upper-urinary-tract damage and may impair development of bladder capacity. Although unrecognised in the past, involuntary bladder contractions are a primary cause of urine leakage persisting in children with exstrophy and epispadias after continence surgery. Detailed functional testing should become a routine part of the evaluation of children with exstrophy and epispadias. Introduction Bladder exstrophy is a severe congenital abnormality characterised by a very small bladder turned inside out through a defect in the lower abdominal wall with no urethra or sphincter for urinary control. Epispadias is a less severe variant in which the bladder is small but not everted and the degree of urethral development and urinary control is variable. Historically, children with exstrophy and severe epispadias underwent urinary diversion to an ileal or colonic conduit. Over the past 15-20 years improvements in surgical techniques have allowed bladder closure and urethral construction, which have resulted in a greatly improved cosmetic outcome for most children.l3 However, because the subsequent surgery required to achieve urinary control lacks predictability, multiple major operations can result. In addition, although most affected children are born with normal kidneys, 25-50% have subsequent upper-urinarytract damage after reconstructive procedures. Thus, achieving continence with preservation of renal function in exstrophy and epispadias remains one of the most daunting of challenges in paediatric surgery. The first step of exstrophy reconstruction is turning in the everted bladder--done during the neonatal period-with no
in
exstrophy and
to create resistance to leakage. Serial evaluations after closure reveal that most patients have vesicoureteric reflux, and some degree of hydroureteronephrosis is common. Development of bladder capacity at this stage is variable. Initial surgery for continence (at 3-5 years of age) in exstrophy and epispadias is tubularisation of the inferior portion of the bladder to create a tight bladder neck that will provide resistance to leakage.4 This approach to management is based on the assumption that: (1) the closed exstrophy bladder leaks continuously, without providing storage function ; (2) bladder capacity will expand and eventually continence will develop in response to increased outflow resistance; and (3) the bladder (detrusor) muscle is capable of normal function-ie, relaxation during filling to enable urine storage at low pressure and volitional contraction for voiding. Failure to achieve capacity and continence with this protocol leads to additional surgical procedures including revision of the bladder-neck reconstruction, placement of an artificial urinary sphincter,S and enlargement of the bladder with a segment of bowe1.6,7 Often, these procedures also fail to achieve urinary control and result in further upper-urinary-
attempt
damage. Surprisingly, evaluation of bladder function during filling, voiding, and leaking before and after bladder-neck reconstruction has not been done for patients with exstrophy and epispadias. As a consequence, abnormalities in lower-urinary-tract function, which are potential causes of upper-urinary-tract damage and which may impair development of bladder capacity and continence, have gone unrecognised. We are doing a prospective study to answer several questions regarding bladder function in patients with exstropy and epispadias. This preliminary report is concerned with the unexpected findings that conventional surgery may be based on false assumptions and that the surgery itself may adversely affect bladder function. tract
Patients and methods For the purposes of our study children have been classified into three groups. Group A are 5 children (1girl, 4 boys; age range 3-13 years) with primary severe epispadias after urethral closure but before planned bladder-neck reconstruction. Group B are 15 children (6 girls, 9 boys; age range 1 -5-9 years) with exstrophy after bladder and urethral closure but before surgery for continence. Group C are 16 children (9 with exstrophy, 7 with epispadias; 3 girls, 14 boys; age range 6-16 years) who have had bladder-neck reconstruction(s) (without augmentation) but remain incontinent (secure dry intervals less than 3 h).
ADDRESSES: Department of Paediatric Urology, St Peter’s Hospitals and The Hospital for Sick Children, Great Ormond Street, London, UK (J. G Hollowell, MD, P G. Duffy, FRCS, P G. Ransley, FRCS), and Department of Medical Physics, St Peter’s Hospitals, London (P. D. Hill, MSc). Correspondence to Mr Philip G Ransley, Department of Urology, Hospital for Sick Children, London
WC1N 3JL, UK.
927
All children had their bladder pressure recorded during natural filling (in the sitting and supine positions) with a catheter (’Cystofix minipaed CH5’, B. Braun, Melsungen, Germany) placed suprapubically into the bladder under general anaesthesia at least 2 days before the bladder-function study. Intra-abdominal pressure (measured via a rectal catheter), bladder pressure, and subtracted (detrusor) pressure were recorded simultaneously throughout filling and voiding/leaking with the ’Ormed UIS 5000’ system (Lectromed, Welwyn Garden City, UK). All studies were done by the same individual. Bladder function during filling was defined as active when phasic contractions greater than 10 cm H2O occurred that were clearly not under the patient’s volitional control. Otherwise bladder function was considered stable. Leak pressure, a variable related to bladder-neck resistance, was defmed as the minimum total bladder pressure recorded by cystometry that resulted in leakage. Cystograms were taken under general anaesthesia of all children in groups A and B at the time of placement of the suprapubic catheter. The bladder was filled with contrast solution through a urethral catheter until bladder pressure was 25-30 cm H2O. Cystogram capacity was defined as the total volume of contrast solution instilled with an adjustment made for an estimate of upper-urinary-tract volume in those with reflux. Except in the case of 4 children, where the cystogram revealed reflux into a normal undilated upper-urinary-tract, renal ultrasound images were obtained.
Results
Group A All 5 children in this group were able to initiate a detrusor contraction for voiding and none had involuntary detrusor contractions during filling. The upper urinary tracts of these patients were normal.
NUMBER OF PATIENTS WITH EXSTROPHY OR EPISPADIAS AND BLADDER-NECK RECONSTRUCTION WITHIN EACH CATEGORY OF LOWER-URINARY-TRACT FUNCTION
*15 of 16 patients were emptying without catheterisation and evaluated m 14 of them
voiding could be
Group C Bladder capacity, bladder function during filling and voiding, and bladder-neck function (as measured by leak pressure) of the 16 children in group C are summarised in the table. Of these 16 children with persisting incontinence, 4 leaked only with involuntary detrusor contractions. 5 others had minor leakage at low pressures (10-20 cm H2O), but their major difficulty was high-pressure (55-165 cm H2O) detrusor contractions. The cause of incontinence in the other 7 children
was
low leak pressure.
Discussion
Group B the 15 children with exstrophy in this group had not had bladder-neck reconstruction to create outflow resistance, most did not leak urine continuously and had some storage function. Bladder-filling cycles in 10 children showed intermittent involuntary detrusor contractions (maximum pressure up to 90 cm H2O) that were responsible for most of the leakage of urine. The frequency of these contractions was variable, occurring once per 20 min in some but only once in 2-3 h in others. Leak pressure was 10-30 cm H2O in 8 patients and greater than 30 cm H2O in 4. 3 patients leaked urine with no increase in bladder pressure. Only 2 of the 11 patients in whom voiding could be evaluated were able to initiate a detrusor contraction for voiding. The other 9 voided by abdominal straining. Bladder capacities and upper-urinary-tract status of this group of patients were analysed with respect to bladder activity and leak pressure to determine if these variables of lower-urinary-tract function influence progression of urinary-tract development. Bladder capacity was poor (mean 45 ml) in the 3 children with leak pressure less than 10 cm H20. Of the 8 patients with a leak pressure of 10-30 cm H2O, capacity was better in the 2 without bladder activity (mean =120 ml) than in the 6 with involuntary contractions (mean = 80 ml). The 4 children with a leak pressure greater than 30 cm H20 all had involuntary contractions with variable capacity (40-260 ml). Upperurinary-tract dilatation was not observed by ultrasound even in the presence of involuntary detrusor contractions when leak pressure was less than 30 cm H2O. Of the 4 children with a leak pressure greater than 30 cm H2O, all of whom showed involuntary contractions, 2 had upperurinary-tract dilatation.
Although
=
Although this is a preliminary study with relatively few patients in each group, the data clearly refute several assumptions regarding lower-urinary-tract function on which current management of patients with exstrophy and epispadias is based. The closed exstrophy bladder (group B) does not continuously drip urine as thought previously. In most patients in this group leakage was intermittent and caused by involuntary detrusor contractions. It appears that development of bladder capacity at this stage is related to bladder activity as well as outflow resistance. A leak pressure of at least 10 cm H2O is required to allow the bladder to expand, but leakage due to involuntary contractions may limit achievement of capacity in many of those with a leak pressure greater than 10 cm H2O. This finding opens up the possibility that pharmacological control of contractions may allow capacity to develop, and a trial of this is in progress. The primary risk factor for upper-urinary-tract dilatation is a leak pressure greater than 30 cm H2O. In the group of children with incontinence after bladderneck reconstruction (group C) bladder function was not normal, and the abnormalities that we found may inhibit an increase in bladder capacity and the development of continence in response to bladder-neck reconstruction. The inability to initiate and sustain a detrusor contraction for voiding impairs bladder emptying and thereby decreases functional bladder capacity and thus potential dry intervals. Some of these children achieve adequate emptying by abdominal straining but others require intermittent catheterisation as the next step of management rather than further surgery. Involuntary detrusor contractions are the primary cause of leakage in many patients in this group. Thus, bladder-neck revision or placement of an artificial
928
urinary sphincter, to create a higher leak pressure, without concomitant treatment of the detrusor dysfunction may still not achieve continence or further development of capacity. These children with involuntary contractions are also undergoing a trial of anticholinergic therapy before further surgery.
This study raises an important question regarding management of continence in primary epispadias. The 5 children who had not had bladder-neck surgery (group A) showed normal bladder function during filling and voiding. However, evaluation of the 7 children with primary epispadias after bladder-neck reconstruction (part of group C) revealed a high incidence of involuntary contractions during filling and most could not initiate contractile voiding. These findings suggest that a formal bladder-neck reconstruction may severely damage detrusor function. If this proves to be the case, other management options will need to be evaluated. Possible alternatives are endoscopic submucosal injection of collagen in the bladder neck area8 or the implantation of an artificial urinary sphincter without first reconstructing the bladder neck. Further investigation of lower-urinary-tract function in children with exstrophy and epispadias is needed. However, this study provides sufficient evidence for several preliminary conclusions. Contrary to current assumptions, bladder function is not normal in most children with exstrophy and in those with epispadias after bladder-neck surgery. These functional abnormalities may be a cause of upper-urinary-tract damage and may impair development of bladder capacity and continence at all stages.
Clinical
respiratory
≽40/min in children over 12 months old and ≽ 50/min in infants, as
a
showed little additional
We thank Sister Brid Carr for her invaluable assistance and Mr Larry Watkinson for help with information technology support. The support for J. G. H. from The St Peter’s Research Trust and Action Research is
gratefully acknowledged. REFERENCES
JS. Surgical treatment of exstrophy of the bladder with emphasis neonatal primary closure: personal experience with 28 consecutive cases treated at the University of Washington Hospitals from 1962 to 1977: techniques and results. J Urol 1979; 121: 650-53. Osterling JE, Jeffs RD. The importance of a successful initial bladder closure in the surgical management of classical bladder exstrophy: analysis of 144 patients treated at the Johns Hopkins Hospital between 1975 and 1985. J Urol 1987; 137: 258-62. Ransley PG, Duffy PG, Wollin M. Bladder exstrophy closure and epispadias repair. In: Spitz L, Nixon HH, eds. Paediatric Surgery. London: Butterworths, 1988: 620-32. Jeffs RD, Lepor HL. Management of the exstrophy-epispadias complex and urachal anomalies. In: Walsh PC, Gittes RF, Perlmutter AD, Stamey TA, eds. Campbell’s Urology, 5th ed. Philadelphia: WB Saunders, 1986: 1882-921. Decter RM, Roth DR, Fishman IJ, Shabigh R, Scott FB, Gonzales ET Jr. Use of the AS800 device in exstrophy and epispadias. J Urol 1988; 140: 1202-03. Gearhart JP, Jeffs RD. Augmentation cystoplasty in the failed exstrophy reconstruction. J Urol 1988; 139: 790-93. Hollowell JG, Ransley PG. The surgical management of incontinence in bladder exstrophy. Br J Urol (in press). Bloom DA, McGuire EJ. Collagen injection therapy in children with myelodysplasia. Dialogues Paed Urol 1991; 14: 2-3.
1. Ansell on
2.
3.
4.
5.
6.
7. 8.
signs of pneumonia
Clinical and chest radiographic findings were recorded prospectively in 185 children with cough who attended an outpatient clinic in Papua New Guinea. Children were studied if they were between 8 weeks and 6 years of age; patients with wheeze, stridor, measles, or pertussis were excluded. 56 children (30%) had radiological evidence of pneumonia. The presence of either a respiratory rate ≽50/min or chest indrawing, or of both signs, was a good indication of pneumonia, with a predictive power of 46% for a positive test and 83% for a negative test. A more complex definition of
tachypnoea,
Detailed functional evaluation is necessary to identify abnormalities and plan management in children with exstrophy and epispadias and should become a routine part of their evaluation in the future.
rate
diagnostic benefit.
Introduction 4 million children annually die from acute respiratory infections world wide,l most as a result of bacterial pneumonia.2 The World Health Organisation (WHO) has suggested that mortality from pneumonia could be substantially reduced by early antibiotic therapy. Although most children with pneumonia present with cough, most children with a cough do not have pneumonia, so it is
in children
important to find clinical signs that will help primary health workers to decide which children with cough have pneumonia and require early treatment with antibiotics. There have been several attempts to identify reliable clinical signs of pneumonia in children: in some, pneumonia was occasionally or invariably diagnosed on clinical rather than radiological evidence;3-5 in another, radiographs were used to diagnose pneumonia but respiratory rate was not recorded;6 another only included 18 children with pneumonia;’ and yet another looked at children who already satisfied WHO criteria for pneumonia.8 We prospectively recorded clinical signs among children with cough before they had a chest X-ray, to determine which clinical signs predicted radiographic evidence of pneumonia. Patients and methods We studied 185 children who attended the paediatric outpatient department of Goroka Hospital, Papua New Guinea. Most of the children who attend this clinic come from rural villages in the area around Goroka, a small Highlands town. Children were admitted to ADDRESSES Goroka Base Hospital, Goroka, Papua New Guinea (M Harari, FRACP, V Spooner, MRCGP, S. Meisner, MB, M Carney, MB) and Royal Children’s Hospital, Melbourne, Australia (F. Shann, FRACP, J de Campo, FRACR). Correspondence to Dr F. Shann, Intensive Care Unit, Royal Children’s Hospital, Flemington Road, Parkville, Victoria 3052, Australia
